Cystic Fibrosis

  • U. Stephan
  • M. Götz
  • K. Stephan
  • S. Bender
Conference paper
Part of the Ergebnisse der Inneren Medizin und Kinderheilkunde / Advances in Internal Medicine and Pediatrics book series (KINDERHEILK. NF, volume 44)

Abstract

When, 44 years ago, Fanconi et al. (228) published their paper on “Das Coeliakie-Syndrom bei angeborener cystischer Pankreasfibromatose und Bronchiektasien”, they were of the opinion that they were reporting on a very rare syndrome that in most cases led to death at an early age. In 1957 Bachmann (31), in Vol. 8 of this series, summarized the available knowledge on “the so-called cystic fibrosis of the pancreas”; he expressed the hope that an improvement in our knowledge of the etiology of the disease could lead to an improvement in the results of its treatment.

Key words

Cystic Fibrosis Pathogenesis Pathology Pathophysiology Genetics Screening Microbiology Immunology Diagnosis Psychosocial Problems Treatment Prognosis 
This is a preview of subscription content, log in to check access.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Alderson PO, Secker-Walker RH, Strominger CB, Markham J, Hill RL (1974a) Pulmonary deposition of aerosols in children with cystic fibrosis. J Pediatr 84: 479– 484Google Scholar
  2. 2.
    Alderson PO, Secker-Walker RH, Strominger DB, McAlister WH, Hill RL, Markham J (1974b) Quantitative assessment of regional ventilation and perfusion in children with cystic fibrosis. Radiology 111: 151–155PubMedGoogle Scholar
  3. 3.
    Allan JD, Mason A, Moss AD (1973) Nutritional supplementation in treatment of cystic fibrosis of the pancreas. Am J Dis Child 126: 22–26PubMedGoogle Scholar
  4. 4.
    Allan JD, Townley RRW, Phelan PD, (1974) Family response to cystic fibrosis. Aust Paediatr J 10: 136–146PubMedGoogle Scholar
  5. 5.
    Allan JD, Moss AD, Wallwork JC, McFarlane H (1975) Immediate hypersensitivity in patients with cystic fibrosis. Clin Allergy 5: 255–261PubMedGoogle Scholar
  6. 6.
    Allars HM, Blomfield J, Rush AR, Brown JM (1976a) Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. I. Physicochemistry. Pediatr Res 10: 578–584Google Scholar
  7. 7.
    Allars HM, Cockayne DJH, Blomfield J, Rush AR, Lennep EW von, Brown JM (1976b) Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. II. Electron microscopy and electrophoresis. Pediatr Res 10: 584–594PubMedGoogle Scholar
  8. 8.
    Allen HD, Taussig LM, Goldberg SJ, Sahn DJ, Gaines J (1976) Echocardiographic definition of progressive cardiac changes in cystic fibrosis (abstr.). Pediatr Res 10: 310Google Scholar
  9. 9.
    Altland L, Schmidt SR, Kaiser G, Knoche W (1975) Demonstration of a factor in the serum of homozygotes and heterozygotes for cystic fibrosis by a non-biological technique. Hum Genet 28: 207–216Google Scholar
  10. 10.
    Altmann RP, Kulczycki LL, Randolph JG, McClenathan JE (1973) Bronchoscopy and bronchial lavage (BBL) in children with cystic fibrosis. J Pediatr Surg 8: 809– 814Google Scholar
  11. 11.
    Alvarez WC (1947) Do enteric coatings of pills do what they are supposed to do? Gastroenterology 9: 219–221Google Scholar
  12. 12.
    Andersen DH (1938) Cystic fibrosis of the pancreas and its relation to celiac disease, a clinical pathology study. Am J Dis Child 56: 344–399Google Scholar
  13. 13.
    Andersen DH (1960) Cystic fibrosis and social stress. Children 7: 9Google Scholar
  14. 14.
    Andersen DH (1962) Pathology of cystic fibrosis. Ann NY Acad Sci 93: 500–517Google Scholar
  15. 15.
    Andersen DH, Gareis FJ (1957) The possible detection of cystic fibrosis by means of the neutron-activation analysis of fingernails. Trans Am Nucl Soc 10: 57–61Google Scholar
  16. 16.
    Andersen DH, Hodges RG (1946) Celiac syndrome. V. Genetics of cystic fibrosis of pancreas with consideration of etiology. Am J Dis Child 72: 62–80PubMedGoogle Scholar
  17. 17.
    Anderson CM, Goodchild MC (1976) Cystic fibrosis — manual of diagnosis and management. Blackwell, OxfordGoogle Scholar
  18. 18.
    Antonelli M, Donfrancesco (1970) Indagine clinico-statistica sulla epidemiologica della fibrosi cystica (F.C.) in Italia nel quadrienno 1966–1969. Fracastoro 63: 207– 216Google Scholar
  19. 18a.
    Antonelli M, Ballati G, Annibaldi L (1969) Simplified nail clipping test for diagnosis of cystic fibrosis. Arch Dis Child 44: 218–220PubMedGoogle Scholar
  20. 19.
    Antonowicz I, Reddy V, Khaw, K-T, Shwachman H (1968) Lactase deficiency in patients with cystic fibrosis. Pediatrics 42: 492–500PubMedGoogle Scholar
  21. 20.
    Antonowicz I, Ishida S, Shwachman H (1975) Studies in meconium: disaccharidase activities in meconium from cystic fibrosis patients and controls. Pediatrics 56: 782– 787Google Scholar
  22. 21.
    Antonowicz I, Ishida S, Shwachman H (1976) Screening for cystic fibrosis. Lancet I: 746–747Google Scholar
  23. 22.
    Antonowicz I, Ishida S, Shwachman H (1978) Studies in meconium in cystic fibrosis: the activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase. Biol Neonate 34: 225–230PubMedGoogle Scholar
  24. 23.
    Araki H, Field M, Shwachman H (1975) A new assay for cystic fibrosis factor: effects of sera from patients with cystic fibrosis: in vitro electrical properties of rat jejunum. Pediatr Res 9: 932–934PubMedGoogle Scholar
  25. 24.
    Arvanitakis C, Greenberger NJ (1976) Diagnosis of pancreatic disease by a synthetic peptide. A new test of exocrine pancreatic function. Lancet I: 663PubMedGoogle Scholar
  26. 24a.
    Arvanitakis SN, Mangos JA, McSherry NR, Rennert OM, Laponite D (1973) Polyamin metabolism in cystic fibrosis. Pediatr Res 7: 336Google Scholar
  27. 25.
    As A van (1977) Pulmonary airway clearance mechanisms: a reappraisal. Am Rev Respir Dis 115: 721–726PubMedGoogle Scholar
  28. 26.
    Asano T (1964) Metabolic disturbances and short circuit current across intestinal wall of rat. Am J Physiol 207: 415–422PubMedGoogle Scholar
  29. 27.
    Aspin N, Wong JW, Yeates DB, Levison H (1977) Mucociliary clearance in cystic fibrosis. Mod Probl Paediatr 19: 199–206Google Scholar
  30. 28.
    Astley R (1975) Radiology of the gastrointestinal tract. In: Anderson CM, Burke V (eds) Paediatric gastroenterology. Blackwell, OxfordGoogle Scholar
  31. 29.
    Athreya BH, Borns P, Rosenlund ML (1975) Cystic fibrosis and hypertrophic ostearthopathy in children. Am J Dis Child 129: 634–637PubMedGoogle Scholar
  32. 30.
    Baar B, Deutsch J, Götz M (1977) Atemtherapie bei kindlichem Asthma bronchiale, Teil 2: Eutonustraining. Paediatr Paedol 12: 76–84Google Scholar
  33. 31.
    Bachmann KD (1957) Die sogenannte cystische Pankreasfibrose. Ergeb Inn Med Kinderheilkd 8: 316–366PubMedGoogle Scholar
  34. 32.
    Baig MM, Cetorelli JJ, Roberts RM (1975) Plasma membrane components of skin fibroblasts from normal individuals and patients with cystic fibrosis. J Pediatr 86: 72–76PubMedGoogle Scholar
  35. 33.
    Balfe JW, Cole C, Welt LG (1968) Red-cell transport defect in patients with cystic fibrosis and in their parents. Science 162: 689–690PubMedGoogle Scholar
  36. 34.
    Baran D, Bogaert E van (eds) (1977) Chest physical therapy in cystic fibrosis and chronic obstructive pulmonary disease. European Press, GhentGoogle Scholar
  37. 35.
    Baran D, Cordier N (1973) Usefulness of transtracheal puncture in the bacteriological diagnosis of lung infections in children. Helv Paediatr Acta 28: 391–399PubMedGoogle Scholar
  38. 36.
    Baran D, Englert M (1973) Pulmonary mechanics and diffusing capacity in cystic fibrosis. Helv Paediatr Acta 28: 175–183PubMedGoogle Scholar
  39. 37.
    Baran D, Dachy A, Klastersky J (1975) Concentration of gentamicin in bronchial secretions of children with cystic fibrosis or tracheostomy. Int J Clin Pharmacol Biopharm 12: 336–341PubMedGoogle Scholar
  40. 38.
    Barbero GJ, Sibinga MS (1962) Enlargement of the submaxillary salivary glands in cystic fibrosis. Pediatrics 29: 788–793PubMedGoogle Scholar
  41. 39.
    Barbero GJ, Sibinga MS, Marino JM, Seibel R (1966) Stool trypsin and chymotrypsin. Value in the diagnosis of pancreatic insufficiency in cystic fibrosis. Am J Dis Child 112: 536–540PubMedGoogle Scholar
  42. 40.
    Barclay RPC, Shannon RS (1964) Trial of artificial diet in treatment of cystic fibrosis of pancreas. Arch Dis Child 50: 490–493Google Scholar
  43. 41.
    Barker R, Levison H (1972) Effects of ultrasonically nebulized distilled water on airway dynamics in children with cystic fibrosis and asthma. Am Rev Respir Dis 80: 396–400Google Scholar
  44. 42.
    Barnett DR, Kurosky A, Bowman BH, Barranco SC (1973a) Loss of the ciliary inhibitory effect of the cystic fibrosis factor following proteolytic digestion and heat denaturation. Tex Rep Biol Med 31: 697–701PubMedGoogle Scholar
  45. 43.
    Barnett DR, Kurosky A, Bowman BH, Hutchison HT, Schmoyer I, Carson SD (1973b) Cystic fibrosis: molecular weight estimation of the ciliary inhibitor. Tex Rep Biol Med 31: 703–708PubMedGoogle Scholar
  46. 44.
    Barton AD, Ryder K, Laurenco RV, Dralle W, Weiss SG (1976) Inflammatory reaction and airway damage in cystic fibrosis. J Lab Clin Med 88: 423–426PubMedGoogle Scholar
  47. 45.
    Bateman JRM, Newman SP, Daunt K, Pavia D, Clarke SW (1979) Regional lung clearance of excessive bronchial secretions during chest physiotherapy in patients with stable airways obstruction. Lancet I: 294–297Google Scholar
  48. 46.
    Batten J,Muir D, Simon G, Carter CO (1963) The prevalence of respiratory disease in heterozygotes for the gene for fibrocystic disease of the pancreas. Lancet I: 1348–1350PubMedGoogle Scholar
  49. 47.
    Bau SK, Aspin N, Wood DE, Levison H (1971) The measurement of fluid deposition in humans following mist tent therapy. Pediatrics 48: 605–612PubMedGoogle Scholar
  50. 48.
    Baumann T (1958) Die Mucoviscidosis als recessives und irreguläres dominantes Erbleiden: eine klinische und genetische Studie. Helv Paediatr Acta (Suppl 8 ) 13: 1–79Google Scholar
  51. 49.
    Beam AG (1973) The relevance of biological markers to the pathogenesis of inherited disease. In: Mangos I A, Talamo RC (eds) Fundamental problems of cystic fibrosis and related diseases. Intercontinental Medical Book, New York, London, p 21Google Scholar
  52. 50.
    Bedrossian CWM, Greenberg SD, Singer DB, Hansen JJ, Rosenberg HS (1976) The lung in cystic fibrosis. Hum Pathol 7: 195–204PubMedGoogle Scholar
  53. 51.
    Beier FR, Renzetti AD, Mitchell M, Watanabe S (1966) Pulmonary pathophysiology in cystic fibrosis. Am Rev Respir Dis 94: 430–440PubMedGoogle Scholar
  54. 52.
    Bender SW, Stephan U (1977) Mucoviscidose, cystische Fibrose. Ein Leitfaden, 2nd edn. Deutsche Ges.z.Bek.d.Mucoviscidose e.V., Erlangen, p 12Google Scholar
  55. 53.
    Benke PJ, Erbstoeszer M, Pilot HC (1972) Transport of labelled compounds in control and cystic fibrosis cells in vitro. Lancet I: 182–184Google Scholar
  56. 54.
    Benn A, Cooke WT (1971) Intraluminal pH of duodenum and jejunum in fasting subjects with normal and abnormal gastric or pancreatic function. Scand J Gastroenterol 6: 313–317PubMedGoogle Scholar
  57. 55.
    Bennett MJ, Medwadowski BF (1967) Vitamin A, vitamin E and lipids in serum of children with cystic fibrosis or congenital heart defects compared with normal children. Am J Clin Nutr 20: 415–421PubMedGoogle Scholar
  58. 56.
    Beratis NG, Conover JH, Conod EJ, Bonforte RJ, Hirschhorn K (1973) Studies on ciliary dyskinesia factor in cystic fibrosis. III. Skin fibroblasts and cultured amniotic fluid cells. Pediatr Res 7: 958PubMedGoogle Scholar
  59. 57.
    Berdon WE, Baker DH, Becker J, DeSanctis P (1967) Scrotal masses in healed meconium peritonitis. N Engl J Med 277: 585–587PubMedGoogle Scholar
  60. 58.
    Bergner A, Bergner RK (1975) Pulmonary hypersensitivity associated with pancreatin powder exposure. Pediatrics 55: 814–817PubMedGoogle Scholar
  61. 59.
    Bernstein JG, Vawter G, Harries GB, Young V, Hillman LS (1960) The occurence of intestinal atresia in newborns with meconium ileus. Am J Dis Child 99: 804–818Google Scholar
  62. 60.
    Berry HG, Kellog EW, Hunt MM, Ingberg RL, Richter I, Gutjahr C (1975) Dietary supplement and nutrition in children with cystic fibrosis. Am J Dis Child 129: 165– 171Google Scholar
  63. 61.
    Bertrand A, Puchelle E (1977) Mesure de la clearance mucociliaire des bronches proximales chez le bronchitique chronique. Pathol Biol (Paris) 25: 623–627Google Scholar
  64. 62.
    Besley GT, Patrick AD, Norman AP (1969) Inhibition of the motility of gill cilia of dreissensia by plasma of cystic fibrosis patients and their parents. J Med Genet 6: 278–280PubMedGoogle Scholar
  65. 63.
    Biener G (1974) Sozialbetreuung des mukoviscidosekranken Kindes. Med Welt 25: 282–283PubMedGoogle Scholar
  66. 64.
    Bigger WD, Holmes B, Good RA (1971) Opsonic defect in patients with cystic fibrosis of the pancreas. Proc Natl Acad Sei USA 68: 1716–1719Google Scholar
  67. 65.
    Bishop HC, Koop CE (1957) Management of meconium ileus, resection, Roux-en-Y anastomosis and ileostomy, irrigation with pancreatic enzymes. Ann Surg 145: 410– 414Google Scholar
  68. 65a.
    Bitar J, Lightwood R (1967) The Wiskott-Aldrich syndrome associated with mucoviscidosis in the same patient. J Pediatr 71: 123–126PubMedGoogle Scholar
  69. 66.
    Blackfan KD, May CD (1938) Inspissation of secretion, dilatation of the ducts and acini, atrophy and fibrosis of the pancreas in infants. J Pediatr 13: 627–634Google Scholar
  70. 67.
    Blanc WA, Reid JD, Andersen DH (1958) Avitaminosis E in cystic fibrosis of the pancreas: a morphological study of gastrointestinal and striated muscle. Pediatrics 22: 494–506Google Scholar
  71. 68.
    Blanc WA, McGiluray ER, Miller OJ (1968) Chromosomes in cystic fibrosis. Lancet I: 1152Google Scholar
  72. 69.
    Blomfield J, Warton KL, Brown JM (1973a) Flow rate and inorganic components of submandibular saliva in cystic fibrosis. Arch Dis Child 48: 267–274PubMedGoogle Scholar
  73. 70.
    Blomfield J, Drascula J, Lennep EW van (1973b) Cystic fibrosis: a disorder of exocrine serous secretion. Aust Paediatr J 9: 232Google Scholar
  74. 71.
    Blomfield J, Drascalu J, Lennep EW van, Brown JM (1973c) Hypersecretion of zymogen granules in the pathogenesis of cystic fibrosis. Gut 14: 558–565PubMedGoogle Scholar
  75. 72.
    Blomfield J, Rush AR, Allars HM, Brown JM (1976) Parotid gland function in children with cystic fibrosis and child control subjects. Pediatr Res 6: 574–578Google Scholar
  76. 73.
    Boat TF, Cheng PW (1976) Mucuous glycoproteins. In: Mangos IA, Talamo RC (eds) Cystic fibrosis: projections into the future. Intercontinental Medical Book, New York, London, p 165Google Scholar
  77. 74.
    Boat TF, Kleinermann JI (1975) Human respiratory secretions. 2. Effects of cholinergic and adrenergic agents on in vitro release of protein and mucous glycoprotein. Chest 67: 325Google Scholar
  78. 75.
    Boat TF, Pallavicini UN (1974) Purification and properties of the calcium-precipitable proteins in submaxillary saliva of normal and cystic fibrosis subjects. Pediatr Res 8: 531–539PubMedGoogle Scholar
  79. 76.
    Boat TF, Petty TL (1977) Chronic bronchitis and cystic fibrosis: two chronic obstructive lung diseases of adults. Am Rev Respir Dis 116: 1–2PubMedGoogle Scholar
  80. 77.
    Boat TF, Sant’Agnese PA di, Warwick WJ, Handwerger S (1969) Pneumothorax in cystic fibrosis. JAMA 209: 1498–1504PubMedGoogle Scholar
  81. 78.
    Boat TF, Kleinermann JI, Carlson DM, Maloney WH, Matthews LW (1974a) Human respiratory tract secretions. I. Mucuous glycoproteins secreted by cultured nasal polyp epithelium from subjects with allergic rhinitis and with cystic fibrosis. Am Rev Respir Dis 110: 428–441PubMedGoogle Scholar
  82. 79.
    Boat TF, Doershuk CF, Stern RC, Matthews LW (1974b) Serum alkaline phosphatase in cystic fibrosis. Clin Pediatr (Phila) 13: 505–512Google Scholar
  83. 80.
    Boat TF, Cheng PW, Iyer RN, Carlson DM, Polony I (1976) Human respiratory tract secretions: mucuous glycoproteins of non-purulent tracheobronchial secretions and sputum. Arch Biochem Biophys 177: 95–104PubMedGoogle Scholar
  84. 81.
    Bock H, Koch E, Stephan U, Windorfer A, Sitzmann FC, Grosse H (1967) Investigations on electrolyte concentrations in the nails of cystic fibrosis patients and controls. Mod Probl Pediatr 10: 279–283Google Scholar
  85. 82.
    Bockova DN, Gromova RV, Eremeeva AS, Kuandykow EU, Nemerskaja LA, Reumova IA (1974) Frequency of mucoviscidosis in children in Moscow (in Russian). Pediatria 52: 24–26Google Scholar
  86. 83.
    Bodian M (1953) Fibrocystic disease of the pancreas. A congenital disorder of mucus production. (Mucosis). Grune & Stratton, New York, p 244Google Scholar
  87. 84.
    Böhme B (1972) Phagozytosedefekt der Granulozyten bei zystischer Fibrose. Helv Paediatr Acta 27: 607–612PubMedGoogle Scholar
  88. 85.
    Böhme G, Wässer S (1973) Phoniatrische Befunde bei Muco viscid ose. Folia Phoniatr (Basel) 25: 342–346Google Scholar
  89. 86.
    Boehme B, Sprössig C (1971) Modifikation eines Tests zur Erkennung heterozygoter Genträger der Mucoviscidose. Dtsch Gesundheitsw 26: 743–745Google Scholar
  90. 87.
    Bogart BI, Conod EJ, Conover JH (1977) The biologic activities of cystic fibrosis serum. I. The effects of cystic fibrosis sera and calcium iontophore A 23187 on rabbit tracheal explants. Pediatr Res 11: 131–134PubMedGoogle Scholar
  91. 88.
    Bogart BI, Conod EJ, Gaerlan PE, Conover J (1978) The biologic activities of cystic fibrosis serum. II. Ultrastructural aspects of the effect of cystic fibrosis sera and calcium iontophore A 23187 on rabbit tracheal explants. Pediatr Res 12: 15–24PubMedGoogle Scholar
  92. 89.
    Bolande RP, Towler WF (1973) Terminal autonomic nervous system in cystic fibrosis. Arch Pathol 95: 172–177PubMedGoogle Scholar
  93. 90.
    Bolton WE, Barranco SC (1975) Characterization of the cell kinetics and growth properties of cystic fibrosis diploid fibroblasts in vitro. Am J Hum Genet 27: 394–409PubMedGoogle Scholar
  94. 91.
    Bonin A, Roy C, Lasalle R, Weber A, Morin C (1973) Fecal chymotrypsin: a reliable index of pancreatic function in children. J Pediatr 83: 594–600PubMedGoogle Scholar
  95. 92.
    Boos D, Kolb H, Rehbein F (1971) Behandlung von Ösophagusvarizen bei prähepatischer portaler Hypertension mit der Dissektionsligatur nach Vossschulte. Z Kinderchir 10: 192–203Google Scholar
  96. 93.
    Botelho SY, Goldstein AM, Rosenlund ML (1973) Tear sodium, potassium, chloride and calcium at various flow rates: children with cystic fibrosis and unaffected siblings with and without corneal staining. J Pediatr 83: 601–606PubMedGoogle Scholar
  97. 94.
    Bowden DH, Fischer VW, Wyatt JP (1965) Cor pulmonale in cystic fibrosis. Am J Med 38: 226–232PubMedGoogle Scholar
  98. 95.
    Bowman B, Lockhart L, McCombs M (1969) Oyster ciliary inhibition by cystic fibrosis factor. Science 164: 325–326PubMedGoogle Scholar
  99. 96.
    Bowman BH, McCombs ML, Lockhart LH (1970) Cystic fibrosis: characterization of the inhibitor to ciliary action in oyster gills. Science 167: 871–873PubMedGoogle Scholar
  100. 97.
    Bowman BH, Barnett DR, Matalon R, Danes BS, Beam AG (1973) Cystic fibrosis: fractionation of fibroblast media demonstrating ciliary inhibition. Proc Natl Acad Sei USA 70: 548Google Scholar
  101. 98.
    Bowman B, Lankford B, Fuller G, Carson S, Kurosky A, Barnett D (1975) Cystic fibrosis: the ciliary inhibitor is a small polypetide associated with IgG. Biochem Biophys Res Commun 64: 1310–1315PubMedGoogle Scholar
  102. 99.
    Bowman BH, Lankford BJ, Fuller GM, Carson SD, Kurosky A, Barnett DR (1975) Cystic fibrosis: the ciliary inhibitor is a small polypetide associated with immunoglobin G. Biochem Biophys Res Commun 64: 1310–1315PubMedGoogle Scholar
  103. 100.
    Bowring AC, Kern IB, Jones RFC (1968) The use of tween 80 in meconium ileus. Australian Paediat. Ass., Canberra April 1968Google Scholar
  104. 101.
    Boxerbaum B, Doershuk DC, Matthews LW (1970) Use of carbenicillin in cystic fibrosis. J Infect Dis (Suppl) 122: 59–61Google Scholar
  105. 102.
    Boxerbaum B, Pittmann S, Doershuk CG, Stern RC, Matthews LW (1971) Use of gentamicin in children with cystic fibrosis. J Infect Dis (Suppl) 124: 293–295Google Scholar
  106. 103.
    Boxerbaum B, Kagumba M, Matthews LW (1973) Selective inhibition of phagocytic activity of rabbit alveolar macrophages by cystic fibrosis serum. Am Rev Respir Dis 108: 777–781PubMedGoogle Scholar
  107. 104.
    Braddock LI, Nagelberg I, Margallo E, Barbero GJ (1969) Calcium binding in submaxillary saliva in cystic fibrosis. In: Cystic Fibrosis Club (abstr). Cystic Fibrosis Foundation, Atlanta, p 17Google Scholar
  108. 105.
    Brand-Auraban A, Kopito L, Shwachman H (1972) Chemical analysis of some inorganic elements in cerumen from patients with cystic fibrosis. J Invest Dermatol 58: 14–15PubMedGoogle Scholar
  109. 106.
    Brasfield D, Hicks G, Seng-jaw Soong H, Tiller RE (1979) The chest roentgenogram in cystic fibrosis: a new scoring system. Pediatrics 63: 24–29PubMedGoogle Scholar
  110. 107.
    Braunstein MS, Fleegler B (1974) Failure of bronchopulmonary lavage in cystic fibrosis. Chest 66: 96–99PubMedGoogle Scholar
  111. 108.
    Braunstein M, Ess JD van, Schwartz RH (1975) T-lymphocytes in cystic fibrosis. Cystic Fibrosis Club (abstr) 28. C.F. Foundation, AtlantaGoogle Scholar
  112. 109.
    Bray PT, Clark GCF, Moody GJ, Thomas JDR (1976) Studies on the Orion sweat chloride measuring system. Proc. 7th Int. Cyst. Fibrosis Congress, Paris, pp 165–170Google Scholar
  113. 110.
    Brogan TD, Ryley HC, Neale L, Yassa J (1975) Soluble proteins of bronchopulmonary secretions from patients with cystic fibrosis, asthma, and bronchitis. Thorax 30: 72–79PubMedGoogle Scholar
  114. 111.
    Brown PM, Wilson ND (1961) Tumour of the cecum with cystic fibrosis of the pancreas. Am J Surg 101: 236Google Scholar
  115. 111a.
    Brown PM, Hallenbeck GA, Soule EH, Burgert EO (1960) Cystic fibrosis with fecal retention and intussusception in late stages. N Engl J Med 263: 544–546Google Scholar
  116. 112.
    Brown GA, Oshin A, Goodchild MC, Anderson CM (1971) Inhibition of sugar transport by plasma from cystic fibrosis patients. Lancet II: 639–640Google Scholar
  117. 113.
    Buchanan D, Rapoport S (1952) Chemical comparisons of normal meconium and meconium from a patient with meconium ileus. Pediatrics 9: 304–310PubMedGoogle Scholar
  118. 114.
    Buchwald M (1975) Abnormal cyclic AMP levels in isoproterenol — stimulated fibroblasts from patients with cystic fibrosis. Am J Hum Henet (abstr) 27: 22Google Scholar
  119. 115.
    Buescher ES, Winkelstein JA (1978) The ability of bacteria to activate the terminal complement components in serum of patients with cystic fibrosis. J Pediatr 93: 530–531PubMedGoogle Scholar
  120. 116.
    Bureau M, McDougall DM, Beaudry PH, Belmonte MM (1978) Late effect of nocturnal mist tent therapy related to the severity of airway obstruction in children with cystic fibrosis. Pediatrics 61: 842–846PubMedGoogle Scholar
  121. 117.
    Burneil RH, Robertson EF (1974) Cystic fibrosis in a patient with Kartagener syndrome. Am J Dis Child 127: 746–747Google Scholar
  122. 118.
    Burns MW, May JR (1968) Bacterial precipitins in serum of patients with cystic fibrosis. Lancet I: 270–272Google Scholar
  123. 119.
    Busch R (1977) K. Landsteiner-first author of cystic fibrosis? Twelfth Annual Meeting ICF(M)A, Dresden, GDR, June 18Google Scholar
  124. 120.
    Busch R (1978) Zur Geschichte der Mukoviszidose. Dtsch Gesundheitsw 33: 316– 320Google Scholar
  125. 121.
    Busch R (1979) Zur Frühgeschichte der zystischen Fibrose (Pankresfibromatose). NTM Schriftenr Gesch Naturwiss Tech Med 16: 95–109Google Scholar
  126. 122.
    Butterworth J (1974) Properties of microsomal glycoprotein galactosyltransferase of cultured human fibroblasts in relation to cystic fibrosis. Clin Chim Acta 56: 159PubMedGoogle Scholar
  127. 123.
    Butterworth J, Scott F, McCrae WM, Brain AD (1972) Lyssomal enzymes of cultured fibroblasts of cystic fibrosis patients. Clin Chim Acta 40: 139PubMedGoogle Scholar
  128. 124.
    Camner P, Jarstrand C, Philpson K (1973) Tracheobronchial clearance in patients with influenza. Am Rev Respir Dis 108: 131–135PubMedGoogle Scholar
  129. 125.
    Campbell IM, Crozier DN, Caton RB (1976) Abnormal fatty acid composition and impaired oxygen supply in cystic fibrosis patients. Pediatrics 57: 480–486PubMedGoogle Scholar
  130. 126.
    Carson S, Godhammer R, Carpenter R (1966) Mucus transport in the respiratory tract. Am Rev Respir Dis 93: 86–92PubMedGoogle Scholar
  131. 127.
    Carter CO (1967) Genetical aspects of cystic fibrosis of the pancreas. Mod Probl Paediatr 10: 372–380Google Scholar
  132. 128.
    Cezeaux G Jr, Telford J, Harrison GM, Keats AS (1967) Bronchial lavage in cystic fibrosis. JAMA 199: 15–18PubMedGoogle Scholar
  133. 129.
    Chang N, Levison H (1972) The effect of a nebulized bronchodilator administered with or without intermittent positive pressure breathing on ventilatory function in children with cystic fibrosis and asthma. Am Rev Respir Dis 106: 867–872PubMedGoogle Scholar
  134. 130.
    Chang N, Levison H, Cunningham K, Crozier DN, Grossett O (1973) An evaluation of nightly mist tent therapy for patients with cystic fibrosis. Am Rev Respir Dis 107: 672–675PubMedGoogle Scholar
  135. 131.
    Changus JE, Quissel DO, Sukup MR, Pilot HC (1975) Studies on the synthesis of plasma membrane proteins of fibroblasts from patients with cystic fibrosis. Am J Pathol 80: 317–328PubMedGoogle Scholar
  136. 132.
    Charman J, Reid L (1972) Sputum viscosity in chronic bronchitis, bronchiectasis, asthma, and cystic fibrosis. Biorheology 9: 185–199PubMedGoogle Scholar
  137. 133.
    Chase HP (1976) Fatty acids, prostaglandins and cystic fibrosis. Pediatrics 57: 441–442PubMedGoogle Scholar
  138. 134.
    Chauncey HH, Levine DM, Kass G, Shwachman H, Henriques BL, Kulczycki LL (1962) Composition of human saliva. Arch Oral Biol 7: 707PubMedGoogle Scholar
  139. 135.
    Chernick WS, Barbero GJ (1959) Composition of tracheobronchial secretions in cystic fibrosis of the pancreas and bronchiectasis. Pediatrics 24: 739–745PubMedGoogle Scholar
  140. 136.
    Chernick WS, Barbero GJ, Parkins FM (1961) Studies on submaxillary saliva in cystic fibrosis. J Pediatr 59: 890–898PubMedGoogle Scholar
  141. 137.
    Chernick WS, Eichel HJ, Barbero GJ (1964) Submaxillary salivary enzymes as a measure of glandular activity in cystic fibrosis. J Pediatr 65: 694–700PubMedGoogle Scholar
  142. 138.
    Cherry J, Roden VJ, Rejent AJ, Dorner RW (1971) The inhibition of ciliary activity in tracheal organ cultures by serum from children with cystic fibrosis and control subjects. J Pediatr 79: 937–942PubMedGoogle Scholar
  143. 139.
    Chrispin AR, Norman AP (1974) The systematic evaluation of the chest radiograph in cystic fibrosis. Pediatr Radiol 2: 101–106PubMedGoogle Scholar
  144. 140.
    Cichocki T, Litwin JA, Szotowa W, Hanicka M, Gutkowski P, Zebrak J, Czeczotko E (1974) Histochemical observations on the pulmonary macrophages in cystic fibrosis. Z Kinderheilkd 116: 127–136PubMedGoogle Scholar
  145. 141.
    Cohen LF (1975) Problems in reproductive physiology and anatomy in young adults with cystic fibrosis. GAP Conf. report. Cystic Fibrosis Foundation, Atlanta, P6Google Scholar
  146. 142.
    Cohen LF, Sant’ Agnese PA di, Taylor A, Gill JR (1977) The syndrome of inappropriate antidiuretic hormone secretion as a cause of hyponatremia in cystic fibrosis. J Pediatr 90: 574–578PubMedGoogle Scholar
  147. 143.
    Cole CH, Dirks JH (1972) Changes in erythrocyte membrane ATP-ase in patients with cystic fibrosis of the pancreas. Pediatr Res 6: 616–621PubMedGoogle Scholar
  148. 144.
    Conn JW (1949) Electrolyt composition of sweat: clinical indication as an index of adrenal function. Arch Intern Med 83: 416–421Google Scholar
  149. 145.
    Conn HO (1974a) Therapeutic portocaval anastomosis: to shunt or not to shunt. Gastroenterology 67: 1065–1071PubMedGoogle Scholar
  150. 146.
    Conn HO (1974b) The rational evaluation and management of portal hypertension. In: Schaffner F, Sherlock S, Leevy CM (eds) The liver and its diseases. Thieme, StuttgartGoogle Scholar
  151. 147.
    Conneally PM, Merritt AD, Yu PL (1973) Cystic fibrosis: population genetics. Tex Rep Biol Med 31: 637–644Google Scholar
  152. 148.
    Conover JH, Beratis NG, Conod EJ, Ainbender E, Hirschkorn K (1973a) Studies on ciliary dyskinesia factor in cystic fibrosis. II. Short term leukocyte cultures and long term lymphoid lines. Pediatr Res 7: 224–228PubMedGoogle Scholar
  153. 149.
    Conover JH, Conod EJ, Hirschkorn K (1973b) Ciliary-dyskinesia factor in immunological and pulmonary disease. Lancet I: 1194PubMedGoogle Scholar
  154. 150.
    Conover JH, Conod EJ, Hirschkorn K (1973c) Complement components in cystic fibrosis. Lancet II: 1501PubMedGoogle Scholar
  155. 151.
    Conover JT, Bonforte RJ, Hathaway P, Pacinc S, Conod EJ, Hirschkorn K, Kobel KB (1973d) Studies on ciliary dyskinesia factor in cystic fibrosis. I. Bioassays and heterozygote detection in serum. Pediatr Res 7: 220–223PubMedGoogle Scholar
  156. 152.
    Conover JH, Conod EJ, Hirschkorn K (1974) Studies on ciliary dyskinesia factor in cystic fibrosis. Life Sci 14: 253–266PubMedGoogle Scholar
  157. 153.
    Cooper HS, Oppenheimer EH (1974) Cystic fibrosis manifested as focal biliary cirrhosis in a newborn infant with congenital heart disease. Johns Hopkins Med J 135: 268–273PubMedGoogle Scholar
  158. 154.
    Cooper DM, Doron J, Mansell AL, Bryan AC, Levison H (1974) The relative sensivity of closing volume in children with asthma and cystic fibrosis. Am Rev Respir Dis 109: 519–524PubMedGoogle Scholar
  159. 155.
    Coradello H, Scheibenreiter S (1973) Mukoviscidose unter dem Bild des infantilen Lobaremphysems beginnend. Klin Paediatr 185: 501–506Google Scholar
  160. 156.
    Corbet A, Ross J, Popkind J, Beaudry P (1975) Relationship of arterial-alveolar nitrogen tension to alveolar-arterial oxygen tension, lung volume, flow measurements, and diffusing capacity in cystic fibrosis. Am Rev Respir Dis 112: 513–519PubMedGoogle Scholar
  161. 157.
    Cordonnier JK, Izant RJ (1963) Meconium ileus equivalent. Surgery 54: 667–672PubMedGoogle Scholar
  162. 158.
    Corey M, Levison H, Crozier D (1976) Five — to seven — year course of pulmonary function in cystic fibrosis. Am Rev Respir Dis 114: 1085–1092PubMedGoogle Scholar
  163. 159.
    Counahan R, Mearns MB (1975) Prevalence of atopy and exercise induced bronchial lability in relatives of patients with cystic fibrosis. Arch Dis Child 50: 477–481PubMedGoogle Scholar
  164. 160.
    Cozzetto FJ (1963) Intestinal lactase deficiency in a patient with cystic fibrosis. Pediatrics 32: 228–233PubMedGoogle Scholar
  165. 161.
    Craig J, Haddad M, Shwachman H (1957) The pathological changes in the liver in cystic fibrosis of the pancreas. Am J Dis Child 93: 357–369Google Scholar
  166. 162.
    Crossley JR, Berryman CC, Elliot RB (1977) Cystic fibrosis screening in the new-born. Lancet II: 1093–1095Google Scholar
  167. 163.
    Crossley JR, Elliot RB, Smith PA (1979) Dried- blood spot screening for cystic fibrosis in the newborn. Lancet I: 472–474Google Scholar
  168. 164.
    Crozier DN (1974) Cystic fibrosis — a not-so-fatal disease. Pediatr Clin North Am 21: 935–950PubMedGoogle Scholar
  169. 165.
    Crozier DN (1976) Discussion remark. J Infect Dis (Suppl) 134: 198Google Scholar
  170. 166.
    Crozier DN, Khan SR (1976) Tobramycin in treatment of infections due to pseudomonas aeruginosa in patients with cystic fibrosis. J Infect Dis (Suppl) 134: 187–190Google Scholar
  171. 167.
    Czegledy-Nagy E, Sturgess JM (1976a) Cystic fibrosis — effects of serum factors on mucus secretion. Lab Invest 35: 588–595PubMedGoogle Scholar
  172. 168.
    Czegledy-Nagy E, Sturgess JM (1976b) Effects of serum factors in mucus secretion. Lab Invest 35: 6–13Google Scholar
  173. 169.
    Dahm LS, Ewing CW, Harrison GM, Rucker RW (1977) Comparison of three techniques of lung lavage in patients with cystic fibrosis. Chest 72: 593–596PubMedGoogle Scholar
  174. 170.
    Danes BS, Beam AG (1968) A genetic cell marker in cystic fibrosis of the pancreas. Lancet I: 1061–1063Google Scholar
  175. 171.
    Danes BS, Beam AG (1969a) Cystic fibrosis distribution of mucopolysaccharides in fibroblast cultures. Biochem Biophys Res Commun 36: 919–924PubMedGoogle Scholar
  176. 172.
    Danes BS, Beam AG (1969b) Cystic fibrosis of the pancreas. A study in cell culture. J Exp Med 129: 775–793PubMedGoogle Scholar
  177. 173.
    Danes BS, Beam AG (1972) Oyster ciliary inhibition by cystic fibrosis culture medium. J Exp Med 136: 1313–1317PubMedGoogle Scholar
  178. 174.
    Danes BS, Flensborg EW (1971) Cystic fibrosis. Cell culture studies on a Danish populations. Am J Hum Genet 23: 297–302PubMedGoogle Scholar
  179. 175.
    Danes BS, Litwin SD, Hutteroth TH, Cleve H, Beam AG (1973) Characterization of cystic fibrosis factor and its interaction with human immunoglobin. J Exp.Med 137: 1538–1543PubMedGoogle Scholar
  180. 176.
    Danielson GK, Kyle GC, Denton R (1966) Portal hypertension in cystic fibrosis. JAMA 195: 155–158Google Scholar
  181. 177.
    Danks DM, Allan J, Anderson CM (1965) A genetic study of fibrocystic disease of the pancreas. Ann Hum Genet 28: 323–356Google Scholar
  182. 178.
    Davidson GP, Hassel FM, Crozier D, Corey M, Forstener GG (1978) Iatrogenic hyperuricemia in children with cystic fibrosis. J Pediatr 93: 976–978PubMedGoogle Scholar
  183. 179.
    Davis PB, Braunstein M, Jay C (1978) Decreased adenosine 3’5’-monophosphate response to isoproterenol in cystic fibrosis leukocytes. Pediatr Res 12: 703–707PubMedGoogle Scholar
  184. 180.
    Davis PB, Sant’Agnese PA di (1978) Assisted ventilation for patients with cystic fibrosis. JAMA 239: 1851–1854PubMedGoogle Scholar
  185. 181.
    Dawes C (1969) The effects of flow rate and duration of stimulation on the concentrations of protein and the main electrolytes in human parotid saliva. Arch Oral Biol 14: 277PubMedGoogle Scholar
  186. 182.
    Day G, Mearns MB (1973) Bronchial lability in cystic fibrosis. Arch Dis Child 48: 355–359PubMedGoogle Scholar
  187. 183.
    Dearborn DG (1976) Water and electrolytes of exocrine secretions in cystic fibrosis. In: Mangos JA, Talamo RC (eds) Cystic fibrosis. Intercontinental Medical Book, New York, London, p 179Google Scholar
  188. 184.
    Denning CR, Wiele RL van de (1966) Sterility studies in the male patients with cystic fibrosis. In: Cystic Fibrosis Club (abstr). Cystic Fibrosis Foundation, Atlanta, p 18Google Scholar
  189. 185.
    Denning CR, Sommers SC, Quigley JJ Jr (1968) Infertility in male patients with cystic fibrosis. Pediatrics 41: 7–17PubMedGoogle Scholar
  190. 186.
    Denning CR, Gluckson MM, Mohr I (1976) Psychological and social aspects of cystic fibrosis. In: Mangos JA, Talamo RC (eds) Cystic fibrosis. Projections into the future. Intercontinental Medical Book, New York, London, pp 127–151Google Scholar
  191. 187.
    Denny FW (1974) Effect of a toxin produced by Haemophilus influenzae on ciliated respiratory epithelium. J Infect Dis 129: 93–100PubMedGoogle Scholar
  192. 188.
    Denton R, Kwart H, Litt M (1967) N-acetylcysteine in cystic fibrosis. Am Rev Respir Dis 95: 643–651PubMedGoogle Scholar
  193. 189.
    Deren JJ, Arora B, Toskes PP, Hansell J, Sibinga M (1973) Malabsorption of crystalline vitamin B12 in cystic fibrosis. N Engl J Med 288: 949–950PubMedGoogle Scholar
  194. 190.
    Deschamps J-P, Didier F, Floquet J (1971) Mucoviscidose reveleé et dominée par ses manifestations cardiaques. Arch Fr Pediatr 28: 417–424PubMedGoogle Scholar
  195. 191.
    Desor JA, Maller O (1975) Taste correlates of disease states: cystic fibrosis. J Pediatr 87: 93–96PubMedGoogle Scholar
  196. 192.
    Dickson JAS (1977) Surgical emergencies in the first few weeks of life. In: Harries JT (ed) Essentials of paediatric gastroenterology. Churchill, Edinburgh London New YorkGoogle Scholar
  197. 193.
    Dickson JAS, Mearns MB (1975) Meconium ileus. In: Wilkinson AW (ed) Recent advances in paediatric surgery. Churchill, Edinburgh London New YorkGoogle Scholar
  198. 194.
    Dietzsch HJ, Gottschalk B, Mittenzwey KW, Klemm E, Winkler E (1975) Untersuchungen zur pathogenetischen Bedeutung der Staphylokokken für die broncho- pulmonalen Veränderungen bei Kindern mit Mucoviscidose. Dtsch Gesundheitsw 30: 498–501Google Scholar
  199. 195.
    Dietzsch HJ, Gottschalk B, Machill G (1978) Zur Einführung und Organisation eines Neugeborenenscreenings für das Krankheitsbild der Mucoviscidose. Kinderaerztl Prax 46: 599–601PubMedGoogle Scholar
  200. 196.
    DiMagno EP, Go VLW, Summerskill WHJ (1973) Relations between pancreatic enzyme outputs and malabsorption in severe pancreatic insufficiency. N Engl J Med 288: 813–815PubMedGoogle Scholar
  201. 197.
    DiMagno EP, Malagelada JE, Go VLW, Moertel CG (1977) Fate of orally ingested enzymes in pancreatic insufficiency. N Engl J Med 296: 1318–1322PubMedGoogle Scholar
  202. 198.
    Dodge JA, Bouton L (1975) Heterozygote advantages in cystic fibrosis. Lancet I: 572Google Scholar
  203. 199.
    Doershunk CF, Matthews LW, Gillespie CT, Lough MD, Spector S (1968) Evaluation of jet-type and ultrasonic nebulizers in mist tent therapy for cystic fibrosis. Pediatrics 41: 723–732Google Scholar
  204. 200.
    Doggett RG, Harrison GM (1972) Pseudomonas aeruginosa: immune status in patients with cystic fibrosis. Infect Immun 6: 628–635PubMedGoogle Scholar
  205. 201.
    Doggett RG, Harrison GM (1973) Cystic fibrosis: in vivo reversal of the ciliostatic character of serum and parotid secretions by heparin. Cystic fibrosis: in vitro reversal of the ciliostatic character of serum and salivary secretions by heparin. Nature (New Biol) 243: 250–251Google Scholar
  206. 202.
    Doggett RG, Bentinck B, Harrison GM (1971) Structure and ultrastructure of the labial salivary glands in patients with cystic fibrosis. J Clin Pathol 24: 270–282PubMedGoogle Scholar
  207. 203.
    Dolan TF, Gibson LE (1971) Complications of iodide therapy in patients with cystic fibrosis. J Pediatr 79: 684–687PubMedGoogle Scholar
  208. 204.
    Dolan TF, Meyers A (1974) Bronchial asthma and allergic rhinitis associated with inhalation of pancreatic extracts. Am Rev Respir Dis 110: 812–813PubMedGoogle Scholar
  209. 205.
    Dolan TF, Rowe DS, Gibson LE (1970) Edema and hypoproteinemia in infants with cystic fibrosis. Clin Pediatr (Phila) 9: 295–297Google Scholar
  210. 206.
    Donnison AB, Shwachman H, Gross RE (1966) A review of 164 children with meconium ileus seen at the Children’s Hospital Medical Center, Boston. Pediatrics 37: 833–850Google Scholar
  211. 207.
    Dreiling DA, Janowitz HD (1962) The measurement of pancreatic function. In: Reuck AVS de, Cameron MP (eds) Ciba foundation symposium on the exocrine pancreas. Churchill, Edinburg London New YorkGoogle Scholar
  212. 208.
    Duffy MJ, Schwartz V (1973) Cyclic AMP stimulated phosphorylation of erythrocyte membranes from cystic fibrosis and control subjects. Clin Chim Acta 49: 379– 399Google Scholar
  213. 209.
    Duffy MJ, Comer D, Schwarz V (1973) Serum binding of Ca2+ and cystic fibrosis. Nature 246: 151Google Scholar
  214. 210.
    Dulfano MJ, Adler K, Wooten O (1973) Physical properties of sputum. IV. Effects of 100 percent humidity and water mist. Am Rev Respir Dis 107: 130–132PubMedGoogle Scholar
  215. 211.
    Dunnill MS (1959) Metaplastic changes in the visceral pleura in a case of fibrocystic disease of the pancreas. J Pathol Bacteriol 77: 299–302PubMedGoogle Scholar
  216. 212.
    Durand P, Borrone C, Delia Cella G, Liotta A (1969) Le mucopolysaccaridosi. Recent Prog Med (Roma) 44: 279–285Google Scholar
  217. 213.
    Dyck WP (1967) Titrimetric measurements of the fecal trypsin and chymotrypsin in cystic fibrosis patients with pancreatic exocrine insufficiency. Am J Dig Dis 12: 310–317PubMedGoogle Scholar
  218. 214.
    Eichenwald HF, McCracken GH (1978) Antimicrobial therapy in infants and children. J Pediatr 93: 337–377PubMedGoogle Scholar
  219. 215.
    Eigel-Hanus G, Oberländer U, Dierschke R (1977) Schweisselektrolytuntersuchun gen unter intravenöser Kochsalzbelastung bei Patienten mit Mukoviszidose und chronischer Bronchitis. Paediatr Grenzgeb 16: 65–68Google Scholar
  220. 216.
    Elliott RB (1976) A therapeutic trial of fatty acid supplementation in cystic fibrosis. Pediatrics 57: 474–479PubMedGoogle Scholar
  221. 217.
    Elliott RB (1978) Cystic fibrosis screening in the newborn. Med J Aust 65 II: 95Google Scholar
  222. 218.
    Ellis CE, Hill DE (1975) Growth, intelligence, and school performance in children with cystic fibrosis who have had an episode of malnutrition during infancy. J Pediatr 87: 565–568PubMedGoogle Scholar
  223. 219.
    Emrich HM, Stoll E, Rossi E (1970) Aldosteronwirkung auf die Natrium- und Kaliumausscheidung im Schweiß von Pankreasfibrose-Patienten und Gesunden. Klin Wochenschr 48: 696–791Google Scholar
  224. 220.
    Esterley J, Oppenheimer E (1962) Observations in cystic fibrosis of pancreas. I. The gallbladder. Bull Johns Hopkins Hosp 110: 247–254Google Scholar
  225. 221.
    Esterley JR Oppenheimer EH (1968a) Cystic fibrosis of the pancreas: structural changes in peripheral airways. Thorax 23: 670–675Google Scholar
  226. 222.
    Esterley JR, Oppenheimer EH (1968b) Observations in cystic fibrosis of the pancreas. III. Pulmonary lesions. Johns Hopkins Med J 122: 94–101Google Scholar
  227. 223.
    Esterly NB, Cantolino SJ, Alter BP, Brusilow SE (1968) Pupillatonia, hyporeflexia and segmental hypohydrosis: autonomic dysfunction in a child. J Pediatr 73: 852– 855PubMedGoogle Scholar
  228. 224.
    Exss R, Mende S (1972) Hepatische Manifestation der zystischen Fibrose. Dtsch Med Wochenschr 97: 425–428PubMedGoogle Scholar
  229. 225.
    Falchuk ZM, Taussig LM (1973) IgA synthesis by jejunal biopsies from patients with cystic fibrosis and hereditary pancreatitis. Pediatrics 51: 49 — 54PubMedGoogle Scholar
  230. 226.
    Falkman C (1977) Cystic fibrosis — a psychological study of 52 children and their families. Acta Paediatr Scand (Suppl) 269Google Scholar
  231. 227.
    Fanconi G, Botsztejn A (1944) Die familiäre Pankreasfibrose mit Bronchiektasien. Schweiz Med Wochenschr 74: 85–89Google Scholar
  232. 228.
    Fanconi G, Uehlinger E, Knauer C (1936) Das Coeliakiesyndrom by angeborener cystischer Pankreasfibromatose und Bronchiektasien. Wien Med Wochenschr 86: 753–756Google Scholar
  233. 229.
    Farber S (1943) Pancreatic insufficiency and the celiac syndrome. N Engl J Med 229: 653–682Google Scholar
  234. 230.
    Farber S (1944a) The relation of pancreatic achylia to meconium ileus. J Pediatr 24: 387–392Google Scholar
  235. 231.
    Farber S (1944b) Pancreatic function and disease in early life. V. Pathologic changes associated with pancreatic insufficiency in early life. Arch Pathol 37: 238–250Google Scholar
  236. 232.
    Farber S, Shwachman H, Maddock CL (1943) Pancreatic function and disease in early life. I. Pancreatic enzyme activity and the celiac syndrome. J Clin Invest 20: 827–838Google Scholar
  237. 233.
    Farrell PM, Pallavicini JC, Ulane MM (1975) Growth characteristics and protein content of tissue-cultured fibroblasts from cystic fibrosis patients. Proc Soc Exp Biol Med 149: 340–343PubMedGoogle Scholar
  238. 234.
    Farrell PM, Fox GN, Spicer SS (1976) Determination and characterization of ciliary ATPase in the presence of serum from cystic fibrosis patients. Pediatr Res 10: 127–135PubMedGoogle Scholar
  239. 235.
    Featherby EA, Russell G (1970) Sputum viscosity and pulmonary function in cystic fibrosis. Arch Dis Child 45: 807–808Google Scholar
  240. 236.
    Featherby EA, Weng T-R, Crozier DN, Duic A, Reilly BJ, Levison H (1970a) Dynamic and static lung volumes, blood gas tensions, and diffusing capacity in patients with cystic fibrosis. Am Rev Respir Dis 102: 737–749PubMedGoogle Scholar
  241. 237.
    Featherby EA, Weng T-R, Levison H (1970b) The effect of isoproterenol on airway obstruction in cystic fibrosis. Can Med Assoc J 102: 835–838PubMedGoogle Scholar
  242. 238.
    Feig SA, Segel GB, Kern KA, Osher AB, Schwartz RH (1974) Erythrocyte transport function in cystic fibrosis. Pediatr Res 8: 594–597PubMedGoogle Scholar
  243. 239.
    Feigelson J, Pecau Y, Sauvegrain J (1970) Liver function studies and biliary tract investigations in mucoviscidosis. Acta Paediatr Scand 59: 539–544PubMedGoogle Scholar
  244. 240.
    Feigelson J, Pecau Y, Perez R (1972) Liver scanning and function in cystic fibrosis. Acta Paediatr Scand 61: 337–342PubMedGoogle Scholar
  245. 241.
    Feigelson J, Mareschal JL, Sauvergrain J (1975a) Les anomalies de la vesicule biliare dans la mucoviscidose. Med Chir Dig 4: 121–124Google Scholar
  246. 242.
    Feigelson J, Pecau Y, Cathelineau L, Navarro J (1975b) Additional data on hepatic function tests in cystic fibrosis. Acta Paediatr Scand 64: 337–344PubMedGoogle Scholar
  247. 243.
    Ferlinz R (1972) Aldosteron-Antagonisten bei chronisch respiratorischer Insuffizienz. In: Brendel W (ed) Extrarenal activity of aldosterone and its antagonists. Excerpta Medica, Amsterdam, pp 148–150Google Scholar
  248. 244.
    Fink RJ, Doershuk CF, Tucker AS, Stern RC, Boat TF, Matthews LW (1978) Pulmonary function and morbidity in 40 adult patients with cystic fibrosis. Chest 74: 643–647PubMedGoogle Scholar
  249. 245.
    Fisher OD (1954) Intestinal obstruction as a later complication of fibrocystic disease of the pancreas. Arch Dis Child 29: 262–264PubMedGoogle Scholar
  250. 246.
    Fitzpatrick PF, Landon EJ, James V (1972) Serum binding of calcium and the red cell membrane in cystic fibrosis. Nature 235: 173–174Google Scholar
  251. 247.
    Fletcher DS, Lin T-Y (1973) Incorporation of L-leucine and D-glucosamine into skin fibroblasts derived from cystic fibrosis and normal individuals. Clin Chim Acta 44: 5–19PubMedGoogle Scholar
  252. 248.
    Floersheim GL, Hopff WH, Gasser M, Bucher K (1971) Impariment of cell-mediated immune response by Pseudomonas aeruginosa. Clin Exp Immunol 9: 241–247PubMedGoogle Scholar
  253. 249.
    Fluge G, Aarskog D (1974) Silver-Russell dwarfism and cystic fibrosis. Am J Dis Child 127: 760PubMedGoogle Scholar
  254. 250.
    Forcucci RA, Stark EW (1972) Hearing loss, speech language and cystic fibrosis. Arch Otolaryngol 96: 361–364PubMedGoogle Scholar
  255. 251.
    Fox WW, Bureau MA, Taussig LM, Martin RR, Beaudry PH (1974) Helium flow curves in the detection of early small airway disease. Pediatrics 54: 293 - 299PubMedGoogle Scholar
  256. 252.
    Francis DEM (1975) Diets for sick children. Clackwell, OxfordGoogle Scholar
  257. 253.
    French RS, McAlister WH, Ternberg J, Strominger D (1970) Meconium ileus relieved by 40 per cent water-soluble contrast enemas. Radiology 94: 341–342Google Scholar
  258. 254.
    Freudenberg E (1952) Die kongenitale Pankreassklerose. Therapiewoche 3: 111–113Google Scholar
  259. 255.
    Freye HB, Kurtz SM, Spöck A, Capp P (1964) Light and electron microscopic examination of the small bowel of children with cystic fibrosis. J Pediatr 64: 575– 579Google Scholar
  260. 256.
    Fritze W, Götz M, Stur O, Zweymüller E (1973) Hearing defects in cystic fibrosis. Z Kinderheilkd 114: 111–118PubMedGoogle Scholar
  261. 257.
    Gairdner D (1975) Heterozygote advantages in cystic fibrosis. Lancet I: 279Google Scholar
  262. 258.
    Galant S, Well I, Rucker R, Novey H (1976) The presence of fungal hypersensitivity in cystic fibrosis. J Allergy Clin Immunol 57: 234Google Scholar
  263. 259.
    Gandevia B, Anderson C (1959) The effect of a bronchodilator aerosol on ventilatory capacity in fibrocystic disease of the pancreas. Arch Dis Child 34: 511–514PubMedGoogle Scholar
  264. 260.
    Garnham JR, Carter TRG (1964) Mechanical intestinal obstruction in a young adult male with mucoviscidosis. Gut 5: 256–259PubMedGoogle Scholar
  265. 261.
    Gatzimos CD, Jowitt RH (1955) Jaundice in mucoviscodosis. (Fibrocystic disease of the pancreas.) Am J Dis Child 89: 182–186Google Scholar
  266. 262.
    Gayton WF, Friedman SB (1973) Psychosocial aspects of cystic fibrosis. Am J Dis Child 126: 856–859PubMedGoogle Scholar
  267. 263.
    Gayton WF, Friedman SB, Tavormina JF, Tucker F (1977) Children with cystic fibrosis. I. Psychological test findings on patients, siblings, and parents. Pediatrics 59: 888–894Google Scholar
  268. 264.
    Gear EV, Dobbins WO (1968) Rectal biopsy: a review of its diagnostic usefulness. Gastroenterology 55: 522–544PubMedGoogle Scholar
  269. 265.
    Geisler M, Bender SW, Mohrmann C, Svecjar J, Degenhardt KH, Hoevels O (1972) Heterzygote testing in cystic fibrosis: metachromasia in cultured white blood cells. Z Kinderheilkd 112: 133–141PubMedGoogle Scholar
  270. 266.
    Genin C, Gilly R, Touraine J-L, Souillet G, Veysseyre C, Frobert Y, Berthoux FC, Manel AM (1977) L’immunité dans la mucoviscidose. Arch Fr Pediatr 34: 717–729PubMedGoogle Scholar
  271. 267.
    George L, Norman AP (1971) Life tables for cystic fibrosis. Arch Dis Child 46: 139–143PubMedGoogle Scholar
  272. 268.
    Gewitz M, Eshagpour E, Holsclaw DS, Miller HA, Kawai N (1977) Echocardiography in cystic fibrosis. Am J Dis Child 131: 275–280PubMedGoogle Scholar
  273. 269.
    Gharib R, Allen RP, Joos HA, Bravo LR (1964) Paranasal sinuses in cystic fibrosis. Am J Dis Child 108: 499–502PubMedGoogle Scholar
  274. 270.
    Gibbons ISE (1969) Disaccharides and cystic fibrosis of the pancreas. Arch Dis Child 44: 65–68Google Scholar
  275. 271.
    Gibbons ISE, Seakin JWT, Ersser RS (1967) Tyrosine metabolism and faecal amino- acids in cystic fibrosis of the pancreas. Lancet I: 877–878Google Scholar
  276. 272.
    Gibbons A, Allan JD, Holzel A, McFarlane H (1976) Cell-mediated immunity in patients with cystic fibrosis. Br Med J 1: 120–122PubMedGoogle Scholar
  277. 273.
    Gibbs GE (1950) Secretin tests with biluminal gastroduodenal drainage in infants and children. Pediatrics 5: 941–947PubMedGoogle Scholar
  278. 274.
    Gibbs GE, Griffin G, Reimer K (1967) Quantitative microdetermination of enzymes in sweat gland. V. Ouabain — sensitive sodium-potassium activated adenosintri- phosphatase. Pediatr Res 1: 24–26Google Scholar
  279. 275.
    Gibson LE, Cooke RE (1959) A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics 23: 545–549PubMedGoogle Scholar
  280. 276.
    Gibson LE, Mathews WJ, Minihan PT (1970) Hyperpermeable mucus in cystic fibrosis. Lancet II: 189–191Google Scholar
  281. 277.
    Gibson LE, Matthews WJ Jr, Minihan PT, Patti JA (1971) Relating mucus, calcium, and sweat in a new concept of cystic fibrosis. Pediatrics 48: 695–710Google Scholar
  282. 278.
    Gillard BK, Feig SA, Harrison GM, Nelson TE (1976) Cystic fibrosis: enzymatic detection of a ciliostatic factor. Pediatr Res 10: 907–910Google Scholar
  283. 279.
    Gillard BK, Markman HC, Feig SA (1978) Differences between cystic fibrosis and normal saliva a-amylase as a function of age and sex. Pediatr Res 12: 868–872PubMedGoogle Scholar
  284. 280.
    Gilly R, Hermier M, Robert JM, Challamel MJ, Gery G (1971) Etude genetique de la mucoviscidose. Arch Fr Pediatr 28: 49–63PubMedGoogle Scholar
  285. 281.
    Glanzmann E (1946) Dysporia entero-broncho-pancreatica congenita familiaris, cystische Pankreasfibrose. (Syndrom von Landsteiner-Fanconi-Andersen.) Eine klinische Vorlesung. Ann Paediatr (Basel) 166: 289–313Google Scholar
  286. 282.
    Glotova AV, Fadeeva MA, Zaslavskaya KA (1973) Sodium content in sweat and nail plates of healthy children and those suffering from mucoviscidosis and also in the relatives of such patients. Vopr Okhr materin Det 18: 35–40PubMedGoogle Scholar
  287. 283.
    Godfrey S, Mearns MB (1971) Pulmonary function and response to exercise in cystic fibrosis. Arch Dis Child 40: 144–151Google Scholar
  288. 284.
    Godfrey S, Mearns MB, Howlett G (1978) Serial lung function studies in cystic fibrosis in the first five years of life. Arch Dis Child 53: 83–85PubMedGoogle Scholar
  289. 285.
    Götz M, Eibl M (1979) Immunity of patients with cystic fibrosis and Pseudomonas aeruginosa infection. Pädiatr Fortbildkurse Prax 48: 37–46Google Scholar
  290. 286.
    Götz M, Lubec G (1978) Complement in cystic fibrosis. Eur J Pediatr 127: 133– 139Google Scholar
  291. 287.
    Götz M, Ludwig H, Polymenidis Z (1974) HLA antigens in cystic fibrosis. Z Kinderheilkd 117: 183–186PubMedGoogle Scholar
  292. 288.
    Goldberg PS, Lourenpo RV (1973) Deposition of aerosol in pulmonary disease. Arch Intern Med 131: 88PubMedGoogle Scholar
  293. 289.
    Goldbloom RB, Sekelj P (1963) Cystic fibrosis of the pancreas. Diagnosis by application of a sodium electrode to the skin. N Engl J Med 269: 1349–1352PubMedGoogle Scholar
  294. 290.
    Goldring RM, Fishman AP, Turino GM, Cohen HI, Denning CR, Andersen DH (1964) Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas. J Pediatr 65: 501–524PubMedGoogle Scholar
  295. 291.
    Goodchild MC, Nelson R, Anderson CM (1973) Cystic fibrosis and celiac disease: co-existence in two children. Arch Dis Child 48: 684–691PubMedGoogle Scholar
  296. 292.
    Goodchild MC, Insley J, Rushton DI, Gaze H (1974a) Cystic fibrosis in 3 Pakistani children. Arch Dis Child 49: 739–743PubMedGoogle Scholar
  297. 293.
    Goodchild MC, Sagaro E, Brown GA, Cruchley PM, Jukes HR, Anderson CM (1974b) Comparative trial of Pancrex V forte and Nutrizym in treatment of malabsorption in cystic fibrosis. Br Med J 3: 712–714PubMedGoogle Scholar
  298. 294.
    Goodchild MC, Murphy GM, Howell AM, Nutter SA, Anderson CM (1975) Aspects of bile acid metabolism in cystic fibrosis. Arch Dis child 50: 769–778PubMedGoogle Scholar
  299. 295.
    Goodman HO, Reed CS (1952) Heredity of fibrosis of the pancreas: possible mutation rate of the gene. Am J Hum Genet 4: 59–71PubMedGoogle Scholar
  300. 296.
    Gordon DS, Hunter RG, O’Reilly RJ, Conway BP (1973) Pseudomonas aeruginosa allergy and humoral antibody-mediated hypersensitivity pneumonia. Am Rev Respir Dis 108: 127–131PubMedGoogle Scholar
  301. 297.
    Gottlieb RP (1971) Metabolic alkalosis in cystic fibrosis. J Pediatr 79: 930–936PubMedGoogle Scholar
  302. 298.
    Gottschalk B (1970) Muco viscid ose: Diagnostik und Therapie. Z Erkr Atmungs-organe 133: 358–366Google Scholar
  303. 299.
    Gottschalk B (1976) Four methods of mass screening in cystic fibrosis and results of 10 000 newborns. Proc 7th Int. Cyst. Fibrosis Congr. 31.5–3.6.76, Paris, pp 162–164Google Scholar
  304. 300.
    Govan JRW, Fyfe JAM (1978) Mucoid Pseudomonas aeruginosa and cystic fibrosis: resistance of the mucoid form to carbenicillin, flucloxacillin and tobramycin and the isolation of mucoid variants in vitro. J Antimicrob Chemother 4: 233–240PubMedGoogle Scholar
  305. 301.
    Gracey M (1975) Cystic fibrosis. In: Anderson CM, Burke V (eds) Paediatric gastroenterology. Blackwell, OxfordGoogle Scholar
  306. 302.
    Gradey M, Anderson CM (1969) Cystic fibrosis of the pancreas in adolescence and adulthood. Aust Ann Med 18: 91–101Google Scholar
  307. 303.
    Gracey M, Burke V, Anderson CM (1969a) Treatment of abdominal pain in cystic fibrosis by oral administration of N-acetylcysteine. Arch Dis Child 44: 404–405PubMedGoogle Scholar
  308. 304.
    Gracey M, Burke V, Anderson CM (1969b) Assessment of medium chain triglyceride feeding in infants with cystic fibrosis. Arch Dis Child 44: 401–405PubMedGoogle Scholar
  309. 305.
    Gracey M, Burke V, Anderson CM (1970) Medium chain triglycerides in paediatric practice. Arch Dis Child 45: 445–452PubMedGoogle Scholar
  310. 306.
    Graham DY (1977) Enzyme replacement therapy of exocrine pancreatic insufficiency in man. N Engl J Med 296: 1314–1317PubMedGoogle Scholar
  311. 307.
    Grand RJ (1970) Changing patterns of gastrointestinal manifestations of cystic fibrosis. Clin Pediatr (Phila) 9: 588–593Google Scholar
  312. 308.
    Grand RJ, Lebenthal E, Jacobsen M, Kevy S, Shwachman H (1976) Predictive value of BSP kinetics for early liver involvement in cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Cong., ParisGoogle Scholar
  313. 309.
    Green MN, Clarke JT, Shwachman H (1958) Studies in cystic fibrosis of the pancreas. Protein pattern in meconium ileus. Pediatrics 21: 635–641Google Scholar
  314. 310.
    Green OC, Fefferman R, Nair S (1967) Plasma growth hormone levels in children with cystic fibrosis and short stature. Unresponsiveness to hypoglycemia. J Clin Endocrinol 27: 1059–1061Google Scholar
  315. 311.
    Griffiths AD, Bull FE (1972) Anomalous sweat chloride levels in cystic fibrosis during antibiotic therapy. Arch Dis Child 47: 132–134PubMedGoogle Scholar
  316. 312.
    Grinwald PM, Segal MB (1978) Effect of saliva from cystic fibrosis patients and from normal subjects on red blood cell sodium transport. Pediatr Res 12: 1–3PubMedGoogle Scholar
  317. 313.
    Grosse KP, Stephan U, Sitzmann FC (1967) Untersuchungen über den Natrium- und Kaliumgehalt in Finger-und Zehennägeln. Z Kinderheilkd 100: 87–100PubMedGoogle Scholar
  318. 314.
    Grove SS (1959) Fibrocystic disease in the Bantu. S Afr J Lab Clin Med 5:113– 119Google Scholar
  319. 315.
    Gürson CT, Sertel H, Gürkan M, Pala S (1973) Newborn screening for cystic fibrosis with the chloride electrode and neutron activation analysis. Helv Paediatr Acta 28: 165–174PubMedGoogle Scholar
  320. 316.
    Guggenbichler JP, Kienel G (1979) Bioavailability of oral antibiotics in cystic fibrosis. Monogr Paediatr 10: 34–40PubMedGoogle Scholar
  321. 317.
    Gugler E, Pallavicini JC, Swerdlow H, Zipkin I, Sant’Agnese PA di (1967a) Immunological studies of submaxillary saliva from patients with cystic fibrosis and from normal children. J Pediatr 73: 548–549Google Scholar
  322. 318.
    Gugler E, Pallavicini CL, Swerdlow H, Sant’Agnese PA di (1967b) The role of calcium in submaxillary saliva of patients with cystic fibrosis. J Pediatr 71: 585–591PubMedGoogle Scholar
  323. 319.
    Gyr K, Stalder GA, Schiffmann I, Felir C, Vonderschmitt D, Fahrlaender H (1976) Oral administration of a chymotrypsinlabile peptide. A new test of exocrine pancreatic function in man ( PFT ). Gut 17: 27Google Scholar
  324. 320.
    Gyrkovits, Markus V, Bittera I (1977) Cystic fibrosis heterozygosity in childhood bronchial asthma. Lancet I: 203Google Scholar
  325. 321.
    Habboushe C, Iacocca V, Braddock L, Barbero G (1971) Pseudomonas agglutinins in patients with cystic fibrosis. Pediatrics 48: 973–974PubMedGoogle Scholar
  326. 322.
    Habboushe C, Iacocca V, Braddock LR, Barbero G (1971) Pseudomonas agglutinins in patients with cystic fibrosis. Pediatrics 48: 973–974PubMedGoogle Scholar
  327. 323.
    Hackett PR, Reas HW (1965) A radical approach to therapy for the pulmonary complications of cystic fibrosis. Anesthesiology 26: 248Google Scholar
  328. 324.
    Hadden JW, Hansen LG, Shaprio BL, Warwick WJ (1973) Erythrocyte enigmas in cystic fibrosis. Proc Soc Exp Biol Med 142: 577PubMedGoogle Scholar
  329. 325.
    Hadorn B (1975) The exocrine pancreas. In: Anderson CM, Burke V (eds) Paediatric gastroenterology. Blackwell, OxfordGoogle Scholar
  330. 326.
    Hadorn B, Johansen PG, Anderson CM (1968a) Pancreozymin-secretion test of excrine pancreatic function in cystic fibrosis and the significance of the results for the pathogenesis of the disease. Can Med Assoc J 98: 377–386PubMedGoogle Scholar
  331. 327.
    Hadorn B, Zoppi G, Shmerling DH, Prader A, Mclntyre I, Anderson CM (1968b) Quantitative assessment of exocrine pancreatic function in infants and children. J Pediatr 73: 39–50PubMedGoogle Scholar
  332. 328.
    Hänggeli C-A (1973) Die Kalziumkonzentration im Schweiß bei Patienten mit zystischer Pankreasfibrose und bei gesunden Kindern. Helv Paediatr Acta 28: 611– 615Google Scholar
  333. 329.
    Hage E, Anderson FV (1972) Light and electron microscopical studies of rectal biopsies in cystic fibrosis. Acta Pathol Microbiol Scand (A) 80: 345 — 350Google Scholar
  334. 330.
    Haibert SP, Sant’Agnese PA di, Kutek FR (1960) Staphylococcal antibodies in cystic fibrosis of the pancreas. Pediatrics 26: 792–799Google Scholar
  335. 331.
    Hall BD, Simpkiss M J (1968) Incidence of fibrocystic disease in Wessex. J Med Genet 5: 262–265PubMedGoogle Scholar
  336. 332.
    Hallett WY, Knudson AG Jr, Massey FJ Jr (1965) Absence of detrimental effect of the carrier state for the cystic fibrosis gene. Am Rev Respir Dis 92: 714–724PubMedGoogle Scholar
  337. 333.
    Handwerger S, Roth J? Gordon P, Sant’Agnese PA di, Charpenter DF, Peter G (1969) Glucose intolerance in cystic fibrosis. N Engl J Med 281: 451–461Google Scholar
  338. 334.
    Harries JT, Muller DPR (1971) Absorption of different doses of fat soluble and water miscible preparations of vitamin E in children with cystic fibrosis. Arch Dis Child 46: 341–344Google Scholar
  339. 335.
    Harries JT, Muller DPR, McCollum JPK, Lipson A, Roma E, Norman AP (1979) Intestinal bile salts in cystic fibrosis. Arch Dis Child 54: 19–24PubMedGoogle Scholar
  340. 336.
    Harris RC, Cohen HI (1963) Sweat electrolytes in glycogen storage disease, type 1. Pediatrics 31: 1044–1046PubMedGoogle Scholar
  341. 337.
    Harris RL, Riley HD Jr (1968) Cystic fibrosis in the American Indian. Pediatrics 41: 733–738PubMedGoogle Scholar
  342. 338.
    Harris R, Norman AP, Payne WW (1955) The effect of pancreatic therapy on fat absorption and nitrogen retention in children with fibrocystic disease of the pancreas. Arch Dis Child 30: 424–427PubMedGoogle Scholar
  343. 339.
    Harris GBC, Neuhauser EBC, Shwachman H (1963) Roentgenographic spectrum of cystic fibrosis. Postgrad Med 34: 251–265PubMedGoogle Scholar
  344. 340.
    Hawley HB, Lewis RM, Swartz DR, Gump DW (1974) Tobramycin therapy of pulmonary infections in patients with cystic fibrosis. Curr Ther Res 16: 414–423PubMedGoogle Scholar
  345. 341.
    Heizer WD, Cleaveland CR, Iber FL (1965) Gastric inactivation of pancreatic supplements. Bull Johns Hopkins Hosp 116: 261–270PubMedGoogle Scholar
  346. 342.
    Henkin RI, Powell GF (1962) Increased sensitivity of taste and smell in cystic fibrosis. Science 138: 1107–1108PubMedGoogle Scholar
  347. 343.
    Hennequet A (1971) Bronchorrhea in mucoviscidosis. Pédiatre 7: 202–207Google Scholar
  348. 344.
    Henning K, Woller P, Gottschalk B (1972) Lungenperfusions- und Ventilationsuntersuchungen bei Kindern mit Mucoviscidose. Z Erkr Atmungsorgane 137: 187– 200Google Scholar
  349. 345.
    Herold W (1969) Schweißwiderstandsmessung bei Mukoviszidose mit einer ein-fachen Capillarsonde. Monatschr Kinderheilkd 117: 626–628Google Scholar
  350. 346.
    Herrod HG, Spöck A (1977) Mother and daughter with cystic fibrosis. J Pediatr 91: 276–277PubMedGoogle Scholar
  351. 347.
    Herson RE (1957) Meconium ileus. Radiology 68: 568–571Google Scholar
  352. 348.
    Herzberg V, Calvert L, Bowman BH (1973) Evidence that the ciliary inhibitor of cystic fibrosis is not an antibody. J Clin Invest 52: 2732–2736PubMedGoogle Scholar
  353. 349.
    Hill HR, Warwick WJ, Dettloff J, Quie PG (1974) Neutrophil granulocyte function in patients with pulmonary infection. J Pediatr 84: 55–58PubMedGoogle Scholar
  354. 350.
    Hinden E (1950) Meconium ileus with no pancreatic abnormality. Arch Dis Child 25: 99–100PubMedGoogle Scholar
  355. 351.
    Hirsch SR, Zastrow JE, Kory RC (1969) Sputum liquefying agents: a comparative in vitro evaluation. J Lab Clin Med 74: 346–353PubMedGoogle Scholar
  356. 352.
    Hodes ME, Merrit AD, Glier JT (1974) An evaluation of differential methylation of ribonucleic acid in cystic fibrosis. Pediatr Res 8: 212–214PubMedGoogle Scholar
  357. 353.
    Hodson CJ, France NE (1962) Pulmonary changes in cystic fibrosis of the pancreas. Clin Radiol 13: 54–61PubMedGoogle Scholar
  358. 354.
    Hösli P, Erickson RP, Vogt E (1976) Prospects for prenatal diagnosis of cystic fibrosis: induction of biochemical abnormalities in fibroblasts from patients with cystic fibrosis by an urinary glycoprotein. Biochem Biophys Res Commun 73: 209–216PubMedGoogle Scholar
  359. 355.
    Hösli P, Kollberg H, Vogt E (1978) Reliable diagnosis of the major type of cystic fibrosis with fibroblast cultures. Acta Paediatr Scand 67: 617–620PubMedGoogle Scholar
  360. 356.
    Hoff GE, Schitz PO, Paulsen J (1974) Tobramycin treatment of Pseudomonas aeruginosa infections in cystic fibrosis. Scand J Infect Dis 6: 333–337PubMedGoogle Scholar
  361. 357.
    Hoffmann-Krayer F, Bächtold-Stäubli H (1932/33) Handwörterbuch des Deutschen Aberglaubens, vol V. Gruyter de, Berlin Leipzig, p 859Google Scholar
  362. 358.
    HØiby N (1977) Pseudomonas aeruginosa infection in cystic fibrosis. Acta Pathol Microbiol Scand (C) (Suppl) 262Google Scholar
  363. 359.
    HØiby N, Kilian M (1976) Haemophilus from the lower respiratory tract of patient with cystic fibrosis. Scand J Respir Dis 57: 103–107PubMedGoogle Scholar
  364. 360.
    HØiby N, Mathiesen L (1974) Pseudomonas aeruginosa infection in cystic fibrosis. Acta Pathol Microbiol Scand (B) 82: 559–566Google Scholar
  365. 361.
    HØiby N, Wiik A (1975) Antibacterial precipitins and autoantibodies in serum of patients with cystic fibrosis. Scand J Respir Dis 56: 38–46PubMedGoogle Scholar
  366. 362.
    HØiby N, Jacobsen L, Jorgensen BA, Lykkegaard E, Weeke B (1974) Pseudomonas aeruginosa infection in cystic fibrosis. Acta Paediatr Scand 63: 843–848PubMedGoogle Scholar
  367. 363.
    HØiby N, Flensborg EW, Beck B, Friis B, Jacobsen SV, Jacobsen L (1977) Pseudomonas aeruginosa infection in cystic fibrosis. Scand J Respir Dis 58: 65–79PubMedGoogle Scholar
  368. 364.
    Holsclaw DS (1975) Problems in reproductive physiology in the young adult with cystic fibrosis. GAP Conference 1975, p 1Google Scholar
  369. 365.
    Holsclaw DS, Shwachman H (1969) Genital function in cystic fibrosis. In: Proc. 5th Int. Cyst. Fibrosis Conf. Cystic Fibrosis Research Trust, pp 308–319Google Scholar
  370. 366.
    Holsclaw DS, Shwachman H (1971) Increased incidence of inguinal hernia, hydrocele and undescended testicle in males with cystic fibrosis. Pediatrics 48: 442–445PubMedGoogle Scholar
  371. 367.
    Holsclaw DS, Teckling JS (1977) The effectiveness of bronchial drainage and aerosol inhalation in cystic fibrosis. In: Baran D, Bogaert van (eds) Chest physical therapy in cystic fibrosis. European Press, Ghent, pp 230–238Google Scholar
  372. 368.
    Holsclaw DS, Topham AL (1977) Cystic fibrosis. A comprehensive bibliography of the medical literature, 1813–1972. National Library of Medicine, Bethesda, (DHEW Publication (NIH) 77–1132, p 201 )Google Scholar
  373. 369.
    Holsclaw DS, Eckstein HB, Nixon HH (1965) Meconium ileus: a 20-year review of 109 cases. Am J Dis Child 109: 101–113PubMedGoogle Scholar
  374. 370.
    Holsclaw DS, Grand RJ, Shwachman H (1970) Massive hemoptysis in cystic fibrosis. J Pediatr 76: 829–838PubMedGoogle Scholar
  375. 371.
    Holsclaw DS, Rocmans C, Shwachman H (1971) Intussusception in patients with cystic fibrosis. Pediatrics 48: 51–58PubMedGoogle Scholar
  376. 372.
    Holsclaw DS, Rocmans C, Shwachman H (1974) Abdominal complaint and appendiceal changes leading to the diagnosis of cystic fibrosis. J Pediatr Surg 9: 867–873PubMedGoogle Scholar
  377. 373.
    Holsclaw DS, Keith HH, Palmer J (1978) Meconium screening for cystic fibrosis. Pediatr Ann 7: 15–20Google Scholar
  378. 374.
    Horton CR, Cole WO, Bader H (1970) Depressed (Ca++)-transport ATPase in cystic fibrosis erythrocytes. Biochem Biophys Res Commun 40: 505–509PubMedGoogle Scholar
  379. 375.
    Houck JC, Sharma VK (1970) Functional failures of fibrocystic fibroblasts. Proc Soc Exp Biol Med 135: 369–372PubMedGoogle Scholar
  380. 276.
    Housteck J, Vavrova V (1967) Notre experience a propos de la mucoviscidose. Rev Med Liege 22: 421–426Google Scholar
  381. 377.
    Huang NN (1974) Antibiotic therapy. In: Guide to drug therapy in patients with with cystic fibrosis. Cystic Fibrosis Foundation, Atlanta, pp 7–24Google Scholar
  382. 378.
    Huang NN, Laraya-Cuasay LR (1978) The planning, analysis and evaluation of antibiotic program in patients with cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr Paris 1978, pp 503–506Google Scholar
  383. 379.
    Huang NN, Macri CN, Girone J, Sproul A (1970) Survival of patients with cystic fibrosis. Am J Dis Child 120: 289–295PubMedGoogle Scholar
  384. 380.
    Huang NN, Hiller EJ, Macri CN, Capitanio M, Cundy KR (1971) Carbenicillin in patients with cystic fibrosis: clinical pharmacology and therapeutic evaluation. J Pediatr 78: 338–345PubMedGoogle Scholar
  385. 381.
    Huff DS, Huang JB, Arey JB (1979) Atypical cystic fibrosis of the pancreas with normal levels of sweat chloride and minimal pancreatic lesions. J Pediatr 94: 237– 239Google Scholar
  386. 382.
    Hunton DB, Long WK, Tsumagari HY (1966) Meconium ileus equivalent: an adult complication of fibrocystic disease. Gastroenterology 50: 99–106PubMedGoogle Scholar
  387. 383.
    Iacocca VF, Sibinga MS, Barbero GJ (1963) Respiratory tract bacteriology in cystic fibrosis. Am J Dis Child 106: 315–324PubMedGoogle Scholar
  388. 384.
    Impero JE, Harrison GM, Nelson TE (1978) Cystic fibrosis: isolation and physical properties of a salivary cystic fibrosis factor. Pediatr Res 12: 108–114PubMedGoogle Scholar
  389. 385.
    Isenberg JN, L’Heureux P, Warwick WJ, Sharp H (1974) Detection of cirrhosis in cystic fibrosis: lack of correlation with gamma-glutamyltranspeptidase and biliary tract roentgenogram. Gastroenterology 66: 887 (A 233)Google Scholar
  390. 386.
    Jabro IH, Gibbs GE (1966) Rectal mucosal biopsies as a diagnostic aid in cystic fibrosis. Lancet 86: 385–388Google Scholar
  391. 387.
    Jahnke V, Theopold HM (1977) Fine structure of nasal mucosa in mucoviscidosis with special regard to polyposis. Arch Otorhinolaryngol (NY) 216: 630–631Google Scholar
  392. 388.
    Jean R, Bonnet H, Emberger JM, Bosc R, Rieu S (1970) Ictère cholostatique du nourrisson révélateur d’une mucoviscidose. Arch Fr Pediatr 27: 849–857PubMedGoogle Scholar
  393. 389.
    Jehanne M, Guivarch J, Guillerm J, Sorgniard R, Hennequet A (1974) L’aérosol therapie sous tente dans le traitment de la mucoviscidose. Ann Pediatr 21: 595–605Google Scholar
  394. 390.
    Jensen KG (1962) Meconium ileus equivalent in a 15 years old patient with mucoviscidosis. Acta Paediatr Scand 51: 344–348Google Scholar
  395. 391.
    Johansen PG, Anderson CM, Hadorn B (1968) Cystic fibrosis of the pancreas. A generalised disturbance of water and electrolyte movement in exocrine tissues. Landet I: 455–460Google Scholar
  396. 392.
    Johnson GG, Thompson MF, Fetteroff S, Fasano AN (1971) Neutron activation analysis technique for nail sodium concentration in cystic fibrosis. Pediatrics 47: 88–93PubMedGoogle Scholar
  397. 393.
    Jusko WJ, Mosovich LL, Gerbracht LM, Mattar ME, Yaffe SJ (1975) Enhanced renal excretion of dicloxacillin in patients with cystic fibrosis. Pediatrics 56: 1038– 1044Google Scholar
  398. 394.
    Kaiser (1973) Excretion of protons, bicarbonate and cations by the single sweat gland of patients with cystic fibrosis. In: Mangos JA, Talamo RC (eds) Fundamental problems of cystic fibrosis and related diseases. Intercontinental Medical Book, New York London, pp 247–256Google Scholar
  399. 395.
    Kaiser D, Drack E (1974) Diminished excretion of bicarbonate from the single sweat gland of patients with cystic fibrosis of the pancreas. Eur J Clin Invest 4: 261–265PubMedGoogle Scholar
  400. 396.
    Kaiser G, Zuppinger P, Joss E (1970) Mukoviscidose und Diabetes mellitus. Helv Paediatr Acta 25: 135–146PubMedGoogle Scholar
  401. 397.
    Kaiser D, Drack E, Rossi E (1971a) Effect of cystic fibrosis sweat on sodium re- absorption by the normal sweat gland. Lancet I: 1003Google Scholar
  402. 398.
    Kaiser D, Drack E, Rossie E (1971b) Inhibition of net sodium transport in single sweat glands by sweat of patients with cystic fibrosis of the pancreas. Pediatr Res 5: 167–172Google Scholar
  403. 399.
    Kaiser D, Schöni M, Drack E (1974) Anionen- und Kationenausscheidung der Parotis in Abhängigkeit von der Fließrate bei Mukoviscidosepatienten und Gesunden. Helv Paediatr Acta 29: 145–150PubMedGoogle Scholar
  404. 400.
    Kalayoglu M, Sieber W, Rodnan JB, Kiesewetter WB (1971) Meconium ileus: a critical review of treatment and eventual prognosis. J Pediatr Surg 6: 290–300PubMedGoogle Scholar
  405. 401.
    Kamdar HR (1961) Cystic fibrosis of the pancreas with respiratory allergy in children. Antiseptic 58: 863–870Google Scholar
  406. 402.
    Kaplan E, Shwachman H, Perlmitter AD, Rule A, Khaw T, Holsclaw DS (1968) Reproductive failure in males with cystic fibrosis. N Engl J Med 279: 65–69PubMedGoogle Scholar
  407. 403.
    Kate LP ten (1975) De natale frequentie van kystische fibrose. Verenigde Reproduktie Bedrivjen-Kleine der A 4, GroningenGoogle Scholar
  408. 404.
    Kate LP ten, Feenstra-de Gooyer I, Ploeg-de Groot G, Glouw WL, Anders GJPA (1978) Should we screen all newborns for cystic fibrosis? Int J Epidemiol 7: 323– 330Google Scholar
  409. 405.
    Kattwinkel J, Agus S, Taussig L, Sant’Agnese PA di, Laster L (1972) The use of L-arginine and sodium bicarbonate in the treatment of malabsorption due to cystic fibrosis. Pediatrics 50: 133–137PubMedGoogle Scholar
  410. 406.
    Kattwinkel J, Taussig LM, McIntosh CL, Sant’Agnese PA di, Boat TF, Wood RE (1973a) Intrapleural instillation of quinacrine for recurrent pneumothorax: use in a patient with cystic fibrosis. JAMA 226: 557–559Google Scholar
  411. 407.
    Kattwinkel J, Taussig LM, Statland BE, Verter JI (1973b) The effects of age on alkaline phosphatase and other serologic liver function tests in normal subjects and patients with cystic fibrosis. J Pediatr 82: 234–242PubMedGoogle Scholar
  412. 408.
    Katz S (1978) Calcium and sodium transport processes in patients with cystic fibrosis. I. A specific decrease in Mg2+ dependent, Ca2+-adenosine triphosphatase activity in erythrocyte membranes from cystic fibrosis patients. Pediatr Res 12: 1033– 1038Google Scholar
  413. 409.
    Katz AJ, Falchuk ZM, Shwachman H (1976) The coexistence of cystic fibrosis and celiac disease. Pediatrics 57: 715–722PubMedGoogle Scholar
  414. 410.
    Katznelson D (1978) Increased intracranial pressure in cystic fibrosis. Acta Paediatr Scand 67: 607–609PubMedGoogle Scholar
  415. 411.
    Keal EE (1970) The neuraminic acid content of sputum, its variation in disease and contribution to the physical properties. MD thesis, University of LondonGoogle Scholar
  416. 412.
    Keating JP, Feigin RD (1970) Increased intracranial pressure associated with probable vitamin A deficiency in cystic fibrosis. Pediatrics 46: 41 –46PubMedGoogle Scholar
  417. 413.
    Keens TG, Krastins IRB, Wannamaker EM, Levison H, Crozier DN, Bryan AC (1977) Ventilatory muscle endurance training in normal subjects and patients with cystic fibrosis. Am Rev Respir Dis 116: 853–860PubMedGoogle Scholar
  418. 414.
    Kessler WR, Andersen DH (1951) Heat prostration in fibrocystic disease of pancreas and other conditions. Pediatrics 8: 648–655PubMedGoogle Scholar
  419. 415.
    Kjellman N-IM, Larsson Y (1975) Insulin release in cystic fibrosis. Arch Dis Child 50: 205–209PubMedGoogle Scholar
  420. 416.
    Klagsbrun M, Farrell PM (1973) The methylation of RNA in fibroblasts of normal volunteeers and patients with cystic fibrosis. In: Mangos JA, Talamo RC (eds) Fundamental problems of cystic fibrosis and related diseases. Intercontinental Medical Book, New York London, pp 53–62Google Scholar
  421. 417.
    Klagsbrun M, Farrell PM (1974) The methylation of transfer and ribosomal ribonucleid acid in human fibroblasts: normal methylation of ribonucleic acid in cystic fibrosis. Pediatr Res 8: 205–211PubMedGoogle Scholar
  422. 418.
    Klastersky J, Geuning C, Monawad E, Daneau D (1972) Endotracheal gentamicin in bronchial infections in patients with tracheostomy. Chest 61: 117–120PubMedGoogle Scholar
  423. 419.
    Kleinerman J (1977) Pathology of the lung-cystic fibrosis. In: Chronic bronchitis and cystic fibrosis: two chronic obstructive lung diseases of adult, GAP Conference 1977. Cystic Fibrosis Foundation, AtlantaGoogle Scholar
  424. 420.
    Kluge RM, Standiford HC, Tatem B, Young VM, Greene WH, Schimpff SC, Calia FM, Hornick RB (1974a) Comparative activity of tobramycin, amikacin, and gentar micin, alone and with carbenicillin against Pseudomonas aeruginosa. Antimicrob Agents Chemother 6: 442–446PubMedGoogle Scholar
  425. 421.
    Kluge RM, Standiford HC, Tatem B, Young VM, Schimpff SC, Greene WH, Calia FM, Hornick RB (1974b) The carbenicillin, gentamicin combination against Pseudomonas aeruginosa. Ann Intern Med 81: 584–591PubMedGoogle Scholar
  426. 422.
    Knauff RE, Adams JA (1968) Meconium proteins and mucoproteins in meconium ileus. Proc Soc Exp Biol Med 127: 801–804PubMedGoogle Scholar
  427. 423.
    Knudson AG, Wayne L Jr, Hallett WY (1967) On the selective advantage of cystic fibrosis heterozygotes. Am J Hum Genet 19: 388–392PubMedGoogle Scholar
  428. 424.
    Koch G, Schwanitz G (1968) Neuere humangenetische Aspekte der Mucoviscidose beim Kind. In: Mukoviscidose. Cystische Fibrose. 2. Deutsches Symposium. Thieme, Stuttgart, S 30–34Google Scholar
  429. 425.
    Koch F, Schwick HG, Störiko K (1965) Alpha-1-Antitrypsinbestimmungen im Serum bei Patienten mit Mucoviscidose. Klin Wochenschr 43: 1120PubMedGoogle Scholar
  430. 426.
    Kollberg H, Ekbohm G (1974) A clinical study of the diagnosis of cystic fibrosis by instrumental neutron activation analysis of sodium in nail clippings. Acta Paediatr Scand 63: 411–417PubMedGoogle Scholar
  431. 427.
    Kollberg H, Hellsing K, Landström O (1973) Diagnosis of cystic fibrosis (CF): analysis of meconium and nail-clippings. Acta Paediatr Scand (Suppl) 236: 55–56Google Scholar
  432. 428.
    Komi N, Miyanaga T, Murakami K (1966) Meconium ileus and fibrocystic disease of the pancreas in Japan. Bull Tokyo Med Dent Univ 13: 1–8PubMedGoogle Scholar
  433. 429.
    Komp DM, Seiden RF (1970) Coagulation abnormalities in cystic fibrosis. Chest 58: 501–503PubMedGoogle Scholar
  434. 430.
    Kopel FB (1972) Gastrointestinal manifestations of cystic fibrosis. Gastroenterology 62: 483–491PubMedGoogle Scholar
  435. 431.
    Kopito L, Shwachman H (1966) Mineral composition of meconium. J Pediatr 68: 313–314Google Scholar
  436. 432.
    Kopito L, Shwachman H (1969) Studies in cystic fibrosis: determination of sweat electrolytes in situ with direct reading electrodes. Pediatrics 43: 794–798PubMedGoogle Scholar
  437. 433.
    Kopito L, Mahmoodian A, Townley RRW, Khaw KT, Shwachman H (1965) Studies in cystic fibrosis: analysis of nail clippings for sodium and potassium. N Engl J Med 272: 504–509PubMedGoogle Scholar
  438. 434.
    Kopito L, Brand-Auraban A, Vawter GF, Shwachman H (1972) Cerumen and ceruminous glands in cystic fibrosis. Pediatr Res 6: 427Google Scholar
  439. 435.
    Kopito LE, Kosasky HS, Shwachman H (1973) Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fértil Steril 24: 512–516PubMedGoogle Scholar
  440. 436.
    Kraemer R, Meier HP, Hammerschlag P, Kaiser D, Sidiropoulus D (1977) Neug borenen-Screening der cystischen Fibrose. Schweiz Med Wochenschr 107: 1105–1109PubMedGoogle Scholar
  441. 437.
    Kraemer R, Tschäppeler H, Rüdeberg A, Stoll E, Rossi E (1979) Verlauf und quantitative Erfassung des pulmonalen Befalls bei cystischer Fibtose. Schweiz Med Wochenschr 109: 39–45PubMedGoogle Scholar
  442. 439.
    Kraus I, Antonowicz I, Shah H, Lazarus H, Shwachman H (1971) Metachromasia and assay for lysosomal enzymes in skin fibroblasts cultured from patients with cystic fibrosis and controls. Pediatrics 47: 1010–1018PubMedGoogle Scholar
  443. 440.
    Kravetz RE, Spiro HM (1965) Gastric secretion in chronic pancreatitis. Ann Intern Med 63: 776–782PubMedGoogle Scholar
  444. 441.
    Kulczycki LL, Schauf V (1974) Cystic fibrosis in blacks in Washington, C.D. Am J Dis Child 127: 64–68Google Scholar
  445. 442.
    Kulczycki LL, MacLeod KI, Shwachman H (1960) Boston: a review of school children for cystic fibrosis. J Dis Child 100: 174–180Google Scholar
  446. 443.
    Kulczycki LL, Miiller H, Shwachman H (1961) Respiratory allergy in patients with cystic fibrosis. JAMA 175: 358–364PubMedGoogle Scholar
  447. 444.
    Kulczycki LL, Mutler JS, McCord-Dickmann D, Herer GH (1970a) The hearing of patients with cystic fibrosis. Arch Otolaryngol 92: 53–59Google Scholar
  448. 445.
    Kulczycki LL, Herer GR, Butler JS (1970b) Cystic fibrosis and hearing. Clin Pe- diatr (Phila) 9: 390–402Google Scholar
  449. 446.
    Kulczycki LL, Shwachman H, Langer E, Provost H, Ferguson BS, Good P (1970c) A medical care program for patients with cystic fibrosis in Maine-the first ten years. Am J Public Health 60: 880–890Google Scholar
  450. 447.
    Kulczycki LL, Bellanti JA, Millis RM, Zeligs B (1976) Significance of hyperglobulinemia in cystic fibrosis. Proc.7th Int. Cyst. Fibrosis Congr. Paris, pp 89–94Google Scholar
  451. 448.
    Kulczycki LL, Murphy TM, Bellanti JA (1978) Pseudomonas colonization in cystic fibrosis JAMA 240: 30–34Google Scholar
  452. 449.
    Kuo PT, Huang NN (1969) The effect of medium-chain triglyceride upon fat absorption and plasma lipid and depot fat of children with cystic fibrosis of the pancreas. J Clin Invest 44: 1924–1933Google Scholar
  453. 450.
    Kylstra JA, Rausch DC, Hall KD, Spock A (1971) Volume-controlled lung lavage in the treatment of asthma, bronchiectasis and mucoviscidosis. Am Rev Respir Dis 103: 651–665PubMedGoogle Scholar
  454. 451.
    Lake KB, Dyke JJ van, Rumsfeld JA (1975) Combined topical pulmonary and systemic gentamicin: the question of safety. Chest 68: 62–64PubMedGoogle Scholar
  455. 452.
    Lamarre A, Reilly BJ, Bryan AC, Levison H (1972) Early detection of pulmonary function abnormalities in cystic fibrosis. Pediatrics 50: 291–298PubMedGoogle Scholar
  456. 453.
    Lamb D, Reid L (1972) The tracheobronchial submucosal glands in cystic fibrosis: a qualitative and quantitative histochemical study. Br J Dis Chest 66: 239–247PubMedGoogle Scholar
  457. 454.
    Lamblin G, Degand P, Roussel P, Haves R, Hartemann R, Fillat M (1972) Les glyco- peptides du mucus bronchique fibrillaire dans la mucoviscidose. Clin Chim Acta 36: 329–340PubMedGoogle Scholar
  458. 455.
    Landau LI, Phelan PD (1973a) The spectrum of cystic fibrosis. A study of pulmonary mechanics in 46 patients. Am Rev Respir Dis 108: 593–602PubMedGoogle Scholar
  459. 456.
    Landau LI, Phelan PD (1973b) The variable effect of a bronchodilating agent on pulmonary function in cystic fibrosis. J Pediatr 82: 863–868PubMedGoogle Scholar
  460. 457.
    Landau LI, Taussig LM, Macklem PT, Beaudry PH (1975) Contribution of inhomo- geneity of lung units to the maximal flow volume curve in children with asthma and cystic fibrosis. Am Rev Respir Dis 111: 725–731PubMedGoogle Scholar
  461. 458.
    Landing BH (1962) Comments on pathologic aspects of fibrocystic disease. Ann NY Acad Sci93: 518–525Google Scholar
  462. 459.
    Landsteiner K (1905) Darmverschlufi durch eingedicktes Mekonium, “Pankreatitis”. Zentralbl Pathol 16: 903–907Google Scholar
  463. 460.
    Lange C de (1927) Cirrhosis of pancreas and liver in infant. Am J Dis Child 34: 372–379Google Scholar
  464. 461.
    Langgard H, Haase H, Flensborg EW (1968) Composition of the skin in mucoviscidosis. Acta Paediatr Scand 57: 255–256PubMedGoogle Scholar
  465. 462.
    Lapey A, Gardner JD (1971) Abnormal erythrocyte sodium transport in cystic fibrosis of the pancreas. Pediatr Res 5: 446–451Google Scholar
  466. 463.
    Lapey A, Kattwinkel J, Sant’Agnese PA di, Laster L (1974) Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis. J Pediatr 84: 328–334PubMedGoogle Scholar
  467. 464.
    Laraya-Cuasay LR, Cundy KR, Huang NN (1976) Pseudomonas carrier rates of patients with cystic fibrosis and of members of their families. J Pediatr 89: 23–26PubMedGoogle Scholar
  468. 465.
    Lau WK, Young LS, Osher AB (1977) Amikacin therapy of exacerbations of Pseudomonas aeruginosa infections in patients with cystic fibrosis. Pediatrics 60: 372–377PubMedGoogle Scholar
  469. 466.
    Lawson D (1970) Bacteriology of the respiratory tract in cystic fibrosis — a hypothesis. In: Watt PJ (ed) The control of chemotherapy. Churchill, Edinburgh, p 69Google Scholar
  470. 467.
    Lawson D (1972) Cystic fibrosis: assessing the effects of treatment. Arch Dis Child 47: 1–4PubMedGoogle Scholar
  471. 468.
    Lebenthal E, Antonowicz I, Shwachman H (1976) Enterokinase and trypsin activities in pancreatic insufficiency and diseases of the small intestine. Gastroenterology 70: 508–517PubMedGoogle Scholar
  472. 469.
    Lee PA, Roloff DW, Howatt WF (1974) Hypoproteinemia and anemia in infants with cystic fibrosis. JAMA 228: 585–588PubMedGoogle Scholar
  473. 470.
    Lelong M, Joseph R, Tan-Vinh, Bouvattier le (1950) Fibrocystic disease of the pancreas and massive steatosis of the liver. Arch Fr Pediatr 7: 234–240PubMedGoogle Scholar
  474. 471.
    Leonard PJ, Morris WP (1972) Sodium, calcium, and magnesium levels in nails of children with cystic fibrosis of the pancreas. Arch Dis Child 47: 495–498PubMedGoogle Scholar
  475. 472.
    Leonidas JC, Berdon WE, Baker DH, Santulli TW (1970) Meconium ileus and its complications. AJR 108: 598–609Google Scholar
  476. 473.
    Lev R, Spicer SS (1965) A histochemical comparison of human epithelial mucins in normal and in hypersecretory states including pancreatic cystic fibrosis. Am J Pathol 46: 23–37PubMedGoogle Scholar
  477. 474.
    Levin S (1963) Fibrocystic disease of the pancreas in Israel. In: Goldsmith R (ed) The genetics of migrant and isolated populations. Williams & Wilkins, Baltimore, p 294Google Scholar
  478. 475.
    Levison H (1977) The effects of gravity on tracheal mucus transport in patient with cystic fibrosis and normals. In: Baran D, Bogaert E van (eds) Chest physical therapy in cystic fibrosis and chronic obstructive pulmonary diseases. European Press, Ghent, pp 157–162Google Scholar
  479. 476.
    Levison H, Godfrey S (1976) Pulmonary aspects of cystic fibrosis. In: Mangos JA, Talamo RC (eds) Cystic fibrosis. Projections into the future. Intercontinental Medical Book, New York London, pp 3–24Google Scholar
  480. 477.
    Levitsky S, Lapey A, Sant’Agnese PA di (1970) Pulmonary resection for life threatening hemoptysis in cystic fibrosis. JAMA 213: 125–127PubMedGoogle Scholar
  481. 478.
    Lewis MB (1972) Rothmund-Thompson syndrome and fibrocystic disease. Austra- las J Dermatol 13: 105–106Google Scholar
  482. 479.
    Lieberman J (1975) Carboxypeptidase B-like activity and C3 in cystic fibrosis. Am Rev Respir Dis 111: 100–102PubMedGoogle Scholar
  483. 480.
    Lieberman J, Kaneshiro W (1977) Abnormal response of cultured lymphocytes to phytohemagglutinin and autologous serum in cystic fibrosis. Am Rev Respir Dis 116: 1047–1055PubMedGoogle Scholar
  484. 481.
    Liebman J, Doershuk CF, Rapp C, Matthews LW (1967) The vectorcardiogram in cystic fibrosis. Circulation 35: 552–569PubMedGoogle Scholar
  485. 482.
    Liebman J, Krause DA, Doershuk CF, Downs TD, Matthews LW (1973) Orthogonal vectorcardiogram in cystic fibrosis. Chest 63: 218–226PubMedGoogle Scholar
  486. 483.
    Liebman J, Lucas RV, Moss A, Rosenthal A (1976) Cor pulmonale and related cardiovascular effects of cystic fibrosis. In: Mangos JA, Talamo RC (eds) Cystic fibrosis. Projections into the future. Intercontinental Medical Book, New York London, pp 41–80Google Scholar
  487. 484.
    Lifschitz MI, Denning CR (1969) Assessment of bronchospasm in patients with cystic fibrosis. Am Rev Respir Dis 99: 399–405PubMedGoogle Scholar
  488. 485.
    Lifschitz MI, Denning CR (1970) Quantitative interaction of water and cystic fibrosis sputum. Am Rev Respir Dis 102: 456–458PubMedGoogle Scholar
  489. 486.
    Lifschitz ML, Bowman FO, Denning CR, Wylie RH (1968) Pneumothorax as a complication of cystic fibrosis. Am J Dis Child 116: 623–640Google Scholar
  490. 487.
    Lillebridge CB, Docter JM, Eidelman S (1967) Oral administration of N-acetyl- cysteine in the prophylaxis of “meconium ileus equivalent”. J Pediatr 71: 887–889Google Scholar
  491. 488.
    Lillie JG, Chrispin AR (1972) Investigations and management of neonatal obstruction by gastrografin enema. Ann Radiol (Paris) 15: 237–241Google Scholar
  492. 489.
    Lindenbaum RH, Blackwell NL,Sa DJ de (1972) A case of double aneuploidy, 47, XXY, 14-,t(13q14q) +, also probably homozygous for cystic fibrosis gene. J Med Genet 9: 232–235Google Scholar
  493. 490.
    Litt M (1977) Mucus rheology and mucociliary clearance. Mod Probl Paediatr 19: 175–181Google Scholar
  494. 491.
    Littman A, Hanscon DH (1969) Current concepts: pancreatic extracts. N Engl J Med 281: 201–204PubMedGoogle Scholar
  495. 492.
    Lloyd-Still JC, Khaw K-T, Shwachman H (1974) Severe respiratory disease in infants with cystic fibrosis. Pediatrics 53: 687–682Google Scholar
  496. 493.
    Lobeck CC, Barta RA, Mangos JA (1963) Study of sweat in pitressin-resistant diabetes insipidus. J Pediatr 62: 868–872PubMedGoogle Scholar
  497. 494.
    Lobeck CC (1972) Cystic fibrosis. In: Stanbury JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited disease. McGraw Hill, New York, p 1605Google Scholar
  498. 495.
    Lober C, Wood RE, Sant’Agnese PA di, Rourk MH, Spöck A (1974) Pattern of presentation of cystic fibrosis of the pancreas seen in patients over age 20. Chest 66: 332–341Google Scholar
  499. 496.
    Lober CW, Saltzman HA, Kylstra JA (1975) Volume-controlled lung lavage in a woman with cystic fibrosis. Chest 68: 382–383PubMedGoogle Scholar
  500. 497.
    Lockhart LH, Bowman BH, Peters D (1968) Effect of cystic fibrosis serum upon oyster cilia. South Med J 61: 1356–1357Google Scholar
  501. 498.
    Lopez-Vidriero MT, Reid L (1977) Pathophysiology of mucus secretion in cystic fibrosis. Mod Probl Paediatr 19: 120–128Google Scholar
  502. 499.
    Lopez-Vidriero MT, Reid L (1978) Bronchial mucus in health and disease. Br Med Bull 34: 63–74PubMedGoogle Scholar
  503. 500.
    Lorin MI, Denning CR (1971) Evaluation of postural drainage by measurement of sputum volume consistency. Am J Phys Med 50: 215–219PubMedGoogle Scholar
  504. 501.
    Lowe CU, May CD, Reed SC (1949) Fibrosis of pancreas in infants and children; a statistical study of clinical and hereditary features. Am J Dis Child 78: 349–374PubMedGoogle Scholar
  505. 502.
    Lox CD, Davis JR, Christian CD, Heine MW (1974) Anosomic hypogonadotropic hypogonadism: Kallman’s syndrome, a case history complicated by cystic fibrosis. Ariz Med 31: 508–512PubMedGoogle Scholar
  506. 503.
    Lundgren DW, Farrell PM, Sant’Agnese PA di (1975) Poly amine alterations in blood of male homozygotes and heterozygotes for cystic fibrosis. Clin Chim Acta 62: 357–362PubMedGoogle Scholar
  507. 504.
    Lundh G (1962) Pancreatic exocrine function in neoplastic and inflammatory disease; a simple and reliable new test. Gastroenterology 42: 275–280PubMedGoogle Scholar
  508. 505.
    Lyrene RK, Polhill RB Jr, Guthrie LA, Tiller RE (1977) Alternative complement pathway in cystic fibrosis. J Pediatr 91:681 –682PubMedGoogle Scholar
  509. 506.
    Macklem PT (1971) Airway obstruction and collateral ventilation. Physiol Rev 51: 368–436PubMedGoogle Scholar
  510. 507.
    Macklem PT (1974) Physiology of cough. Ann Otol Rhinol Laryngol 83: 761–768Google Scholar
  511. 508.
    MacLean WC, Tripp RW Jr (1973) Cystic fibrosis with edema and falsely negative sweat test. J Pediatr 83: 86–89PubMedGoogle Scholar
  512. 509.
    Maddock CL, Farber S, Shwachman H (1943) Effect of secretin on pancreatic function of infants and children. Am J Dis Child 66: 370Google Scholar
  513. 510.
    Magid SL, Smith CC, Dolowitz DA (1967) Nasal mucosa in pancreatic cystic fibrosis. Arch Otolaryngol 86: 212–216PubMedGoogle Scholar
  514. 511.
    Mandel ID, Thompson RH, Wotman S, Taubman M, Kutscher AH, Zegarelli E, Denning CR, Botwick JT, Fahn BS (1965) Parotid saliva in cystic fibrosis. II. Electrolytes and protein bound carbohydrates. Am J Dis Child 110: 646–651PubMedGoogle Scholar
  515. 512.
    Mandel ID, Kutscher A, Denning CR, Thompson RH, Zegarelli E (1967) Salivary studies in cystic fibrosis. Am J Dis Child 133: 431–438Google Scholar
  516. 513.
    Mangos JA, McSherry NR (1967) Sodium transport inhibitory factor in sweat of patients with cystic fibrosis. Science 158: 135–136PubMedGoogle Scholar
  517. 514.
    Mangos JA, McSherry NR (1968) Studies on the mechanism of inhibition of sodium transport in cystic fibrosis of the pancreas. Pediatr Res 2: 378–384PubMedGoogle Scholar
  518. 515.
    Mangos JA, McSherry NR, Benke PJ (1967) A sodium transport inhibitory factor in saliva of patients with cystic fibrosis. Pediatr Res 1: 436–442PubMedGoogle Scholar
  519. 516.
    Mangos JA, McSherry NR, Benke PJ, Spock A (1969a) Studies on the pathogenesis of cystic fibrosis: the isoproterenol treated rat as an experimental model. In: Lawson D (ed), Cambridge (UK) Proc. of the 5th Int. Cyst. Fibrosis Conf. Cystic Fibrosis Trust, London, pp 25–36Google Scholar
  520. 517.
    Mangos J A, Benke PJ, McSherry N (1969b) Salivary gland enlargement and functional changes during feeding of pancreatin to rats. Pediatr Res 3: 562–570PubMedGoogle Scholar
  521. 518.
    Mansell A, Dubrawsky C, Levison H, Bryan AC, Crozier DN (1974) Lung elastic recoil in cystic fibrosis. Am Rev Respir Dis 109: 190–197PubMedGoogle Scholar
  522. 519.
    Marchi AG, Marchi MAM, Mastella G, Nordio S (1973) Abnormal β-glucuronidase activity in cystic fibrosis. Helv Paediatr Acta 28: 427–435PubMedGoogle Scholar
  523. 520.
    Marks MI, Prentice R, Swarson R, Cotton EK, Eichoff TC (1971) Carbenicillin and gentamicin: pharmacologic studies in patients with cystic fibrosis and pseudomonas pulmonary infections. J Pediatr 79: 822–828PubMedGoogle Scholar
  524. 521.
    Martinez JR, Adelstein E, Quissel D, Barbero GJ (1975a) The chronically reserpinized rat as a possible model for cystic fibrosis. I. Submaxillary gland morphology and ultrastructure. Pediatr Res 9: 463–469PubMedGoogle Scholar
  525. 522.
    Martinez JR, Adshead PC, Quissel DO, Barbero GJ (1975b) The chronically reserpinized rat as a possible model for cystic fibrosis. II. Composition and cilioinhibitory effects of submaxillary saliva. Pediatr Res 9: 470–475PubMedGoogle Scholar
  526. 523.
    Martinez-Tello FJ, Braun DG, Blanc WA (1968) Immunoglobulin production in bronchial mucosa and bronchial lymph nodes, particularly in cystic fibrosis of the pancreas. J Immunol 101: 989–1003PubMedGoogle Scholar
  527. 524.
    Mastella G, Chiamenti GP, Gracco G, Olivieri D, Rossi FA, Tonon M, Trabucchi C, Mengoli V (1976) Insulin release and growth hormone release in cystic fibrosis — a new approach. Proc. 7th Int. Cyst. Fibrosis Congr, Paris 1976Google Scholar
  528. 525.
    Matalon R, Dorfmann A (1968) Acid mucopolysaccharides in cultured fibroblasts of cystic fibrosis of the pancreas. Biochem Biophys Res Commun 33: 954–958PubMedGoogle Scholar
  529. 526.
    Matthews LW, Spector S, Lemm J, Potter JL (1963) Studies on pulmonary secretions. I. The overall chemical composition of pulmonary secretions from patients with cystic fibrosis, bronchiectasis, and laryngectomy. Am Rev Respir Dis 88: 199– 204Google Scholar
  530. 527.
    Matthews LW, Doershuk CF, Wise M, Eddy GE, Nudelman H, Spector S (1964) A therapeutic regimen for patients with cystic fibrosis. J Pediatr 65: 558–575PubMedGoogle Scholar
  531. 528.
    Matthews LW, Doershuk CF, Spector S (1967) Mist tent therapy of the obstructive pulmonary lesion of cystic fibrosis. Pediatrics 39: 176–185PubMedGoogle Scholar
  532. 529.
    Mavromichalis J, Zannos-Mariolea L, Nicolaidou M, Georgatou P, Dentaki-Svolaki K, Matsoniatis N (1974) Hematological findings in Greek children with cystic fibrosis. Helv Paediatr Acta 29: 151–155PubMedGoogle Scholar
  533. 530.
    May CD, Lowe CU (1948) The treatment of fibrosis of the pancreas in infants and children. Pediatrics 1: 159–173PubMedGoogle Scholar
  534. 531.
    May JR, Herrick NC, Thompson D (1972) Bacterial infection in cystic fibrosis. Arch Dis Child 47: 908–913PubMedGoogle Scholar
  535. 532.
    McCollum JPK, Mathias PM, Sciberras D, Muller DPR, Newman C, Harries JT, Norman AP (1976) Factor influencing the serum concentrations of vitamin E and essential fatty acids in cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  536. 533.
    McCrae WM, Raeburn JA (1974) The pattern of infection in cystic fibrosis. Scott Med J 19: 187–190PubMedGoogle Scholar
  537. 534.
    McCrae WM, Cull AM, Burton L, Dodge J (1973) Cystic fibrosis: patients response to the genetic basis of the disease. Lancet II: 141–144Google Scholar
  538. 535.
    McCrae WM, Raeburn JA, Hanson EJ (1976) Tobramycin therapy in infections due to Pseudomonas aeruginosa in patients with cystic fibrosis: effect of dosage and concentration of antibiotic in sputum. J Infect Dis (Suppl) 134: 191–193Google Scholar
  539. 536.
    McEvoy FA, Davies RJ, Goodchild MC, Anderson CM (1974) Erythrocyte membrane properties in cystic fibrosis. Clin Chim Acta 54: 195–204PubMedGoogle Scholar
  540. 537.
    McFarlane H, Allan JD (1976) Cystic fibrosis heterozygosity in pathogenesis of allergy. Lancet I: 1241Google Scholar
  541. 538.
    McFarlane H, Holzel A, Brenchley P, Allan JD, Wallwork JC, Singer BE, Worsley B (1975)Immune complexes in cystic fibrosis. Br Med J 31: 423–428Google Scholar
  542. 539.
    McFarlane H, Allan JD, Zeilm P v d (1977) Passive cutaneous anaphylaxis ( PCA) and specific IgE in cystic fibrosis and their heterozygotes. Clin Allergy 7: 279–284Google Scholar
  543. 540.
    McManus SP, Masterson J (1974) Cellular metachromasia with toluidine blue O in cultured white cells of cystic fibrosis heterozygotes. J Med Genet 11: 216–217PubMedGoogle Scholar
  544. 541.
    McPartlin JF, Dickson JAS, Swain VAJ (1972) Meconium ileus: immediate and long term survival. Arch Dis Child 47: 107–210Google Scholar
  545. 542.
    Mearns MB (1970) Aerosol therapy in cystic fibrosis. Arch Dis Child 45: 605–607PubMedGoogle Scholar
  546. 543.
    Mearns MB (1972) Treatment and preventation of pulmonary complications of cystic fibrosis in infancy and early childhood. Arch Dis Child 47: 5–11PubMedGoogle Scholar
  547. 544.
    Mearns MB, Simon G (1973) Patterns of lung and heart growth as determined from serial radiographs of 76 children with cystic fibrosis. Thorax 28: 537–546PubMedGoogle Scholar
  548. 545.
    Mearns M, Young W, Batten J (1965) Transient pulmonary infiltrations in cystic fibrosis due to allergic aspergillosis. Thorax 20: 385–392Google Scholar
  549. 546.
    Mearns M, Longbottom J, Batten J (1968) Precipitating antibody to aspergillus fumigatus in cystic fibrosis. Lancet I: 538–539Google Scholar
  550. 547.
    Mearns MB, Hodson CJ, Jackson ADM, Haworth EM, Sellors TH, Sturridge M, France NE, Reid L (1972a) Pulmonary resection in cystic fibrosis: results in 23 cases 1957–1970. Arch Dis Child 47: 499–508PubMedGoogle Scholar
  551. 548.
    Mearns MB, Hunt GH, Rushworth R (1972b) Bacterial flora of respiratory tract in patients with cystic fibrosis 1950–1971. Arch Dis Child 47: 902–907PubMedGoogle Scholar
  552. 549.
    Meeker IA, Kincannon WN (1964) Acetylcysteine used to liquefy inspissated meconium causing intestinal obstruction in the newborn. Surgery 56: 419–425PubMedGoogle Scholar
  553. 550.
    Mellins RB (1969) The site of airway obstruction in cystic fibrosis. Pediatrics 44: 315–318PubMedGoogle Scholar
  554. 551.
    Mellins RB, Levine R, Ingram RH, Fishman AP (1968) Obstructive disease of the airways in cystic fibrosis. Pediatrics 41: 560–573PubMedGoogle Scholar
  555. 552.
    Mellis CM, Levison H (1978) Bronchial reactivity in cystic fibrosis. Pediatrics 61: 446–450PubMedGoogle Scholar
  556. 553.
    Mende S (1971) Hepatische Manifestation der cystischen Pankreasfibrose. Zentralbl Allg Pathol 114: 604Google Scholar
  557. 554.
    Merrit AD, Hanna BL, Todd CW Jr, Meyers TL (1962) Incidence and mode of inheritance of cystic fibrosis. J Lab Clin Med 60: 998–999Google Scholar
  558. 555.
    Metre VA van, Cooke RE, Gobosn LE, Winkenwerder WL (1960) Evidence of allergy in patients with cystic fibrosis of the pancreas. J Allergy 31: 141–150Google Scholar
  559. 556.
    Meyer H (1978) Therapie der pulmonalen Symptome der cystischen Fibrose. Monatschr Kinderheilkd 126: 168–171Google Scholar
  560. 557.
    Meyer H, Mansfeld J, Schnell L, Haller J de, Haller R de (1974) Infection mycotique de l’arbre respiratoire dans la fibrose kystique du pancreas. Helv Paediatr Acta (Suppl) 32: 32–33Google Scholar
  561. 558.
    Meyers A, Dolan TF, Mueller D (1975) Compliance and self-medication in cystic fibrosis. Am J Dis Child 129: 1011–1013PubMedGoogle Scholar
  562. 559.
    Milner AD (1969) Blood glucose and serum insulin levels in children with cystic fibrosis. Arch Dis Child 44: 351–355PubMedGoogle Scholar
  563. 560.
    Milunsky A (1968) Cystic fibrosis and Down’s syndrome. Pediatrics 42: 501–504PubMedGoogle Scholar
  564. 561.
    Milunsky A, Littlefield J (1969) Diagnostic limitations of metachromasia. N Engl J Med 281: 1128–1129PubMedGoogle Scholar
  565. 562.
    Milunsky A, Bray GA, Londono J, Lorida L (1971) Insulin, glucose, growth hormone and free fatty acids. Determination in patients with cystic fibrosis. Am J Dis Child 121: 15–19Google Scholar
  566. 563.
    Mitchell I, Corey M, Woenne R, Krastins IRB, Levison H (1978) Bronchial hyper-reactivity in cystic fibrosis and asthma. J Pediatr 93: 744–748PubMedGoogle Scholar
  567. 564.
    Mitchell-Heggs PF, Batten JC (1970) Pleurectomy for spontaneous pneumothorax in cystic fibrosis. Thorax 25: 165–171PubMedGoogle Scholar
  568. 565.
    Mitchell-Heggs PF, Palfrey AJ, Reid L (1974) The elasticity of sputum at low shear rates. Biorheology 11: 417–426PubMedGoogle Scholar
  569. 566.
    Mitchell-Heggs PF, Mearns M, Batten JC (1976) Cystic fibrosis in adolescents and adults. Q J Med 45: 479–504PubMedGoogle Scholar
  570. 567.
    Morin CL, Desjeux JF, Anthier L (1973) Effect of saliva and serum from patients with cystic fibrosis on intestinal uptake of amino acids in rat. Biomedicine 19: 133– 137Google Scholar
  571. 568.
    Morin CL, Roy CC, Lasalle R, Bonin A (1976) Small bowel mucosal dysfunction in cystic fibrosis. J Pediatr 88: 213–216PubMedGoogle Scholar
  572. 569.
    Morison A, Beatty DW, Bowie MD (1978) Cellular immune functions in marasmic and underweight infants with prolonged diarrhea. S Afr Med J 54: 7–9PubMedGoogle Scholar
  573. 570.
    Morse WI, Cochrane WA, Landrigan PL (1961) Familial hypoparathyreoidism with pernicious anemia, steatorrhea and adrenocortical insufficiency: a variant of mucoviscidosis. N Engl J Med 264: 1021–1026PubMedGoogle Scholar
  574. 571.
    Moss AJ, Harper WH, Dooley RR, Murray JF, Mack JF (1965) Cor pulmonale in cystic fibrosis of the pancreas. J Pediatr 67: 797–807PubMedGoogle Scholar
  575. 572.
    Motoyama EK, Gibson LE, Zigas CJ (1972) Evaluation of mist tent therapy in cystic fibrosis using maximum expiratory flow volume curve. Pediatrics 50: 299– 306Google Scholar
  576. 573.
    Mowat AP (1977) Chronic liver disease. In: Harries JT (ed) Essentials of paediatric gastroenterology. Churchill, EdinburghGoogle Scholar
  577. 574.
    Muller DPR, Harries JT (1969) Vitamin E studies in children with malabsorption. Biochem J 112: 28Google Scholar
  578. 575.
    Mullius F, Talamo R, Sant’Agnese PA di (1965) Late intestinal complications of cystic fibrosis. JAMA 192: 741–746Google Scholar
  579. 576.
    Nadler HL, Swae MA, Wodnicki JM, O’Flynn ME (1969) Cultivated amniotic- fluid cells and fibroblasts derived from families with cystic fibrosis. Lancet II: 84– 85Google Scholar
  580. 577.
    Nadler HL, Rao GJS, Taussig LM (1978) Cystic fibrosis. In: Stanbury JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited disease. McGraw-Hill, New York, p 1688Google Scholar
  581. 578.
    Neter E (1974) Pseudomonas aeruginosa infections and humoral antibody response of patients with cystic fibrosis. J Infect Dis (Suppl) 130: 132–133Google Scholar
  582. 579.
    Neuhauser EBD (1946) Roentgen changes associated with pancreatic insufficiency in early life. Radiology 46: 319–328PubMedGoogle Scholar
  583. 580.
    Neumann CG, Lawlor GJ, Stiehm ER, Swendseid ME, Newton C, Herbert J, Amman AJ, Jacob M (1975) Immunologic response in malnourished children. Am J Clin Nutr 28: 89–104PubMedGoogle Scholar
  584. 581.
    Neutra MR, Trier JS (1978) Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture. Gastroenterology 75: 701–720Google Scholar
  585. 582.
    Neutra MR, Grand RJ, Trier JS (1977) Glycoprotein synthesis, transport and secretion by epithelial cells of human rectal mucosa: normal and cystic fibrosis. Lab Invest 36: 535–546PubMedGoogle Scholar
  586. 583.
    Newhouse MT, Rossman CM, Dolovich J, Dolovich MB, Wilson WM (1977) Impairment of mucociliary transport in cystic fibrosis. Mod Probi Paediatr 19: 190–198Google Scholar
  587. 584.
    Nielsen EL, Jacobsen L (1973) Investigation of some clinical parameters in cystic fibrosis in relation to metachromasia of cultured fibroblasts. 4th Ann. Meeting EWGCF, WarszawaGoogle Scholar
  588. 585.
    Nielsen HE, Lundh S, Jacobsen SV, HØiby N (1978) Hyper-gamma-globulinemic purpura in cystic fibrosis. Acta Paediatr Scand 67: 443–447PubMedGoogle Scholar
  589. 586.
    Nielsen HE, Lundh S, Jacobsen SV, HØiby N (1978) Hypergammaglobulinemic purpura in cystic fibrosis. Acta Paediatr Scand 67: 443–447PubMedGoogle Scholar
  590. 587.
    Niznikowska-Marks J, Oknifiska A, Kaminski Z (1971) Fibrose myocardique au cours de la mucoviscidose. Helv Paediatr Acta 26: 56–62PubMedGoogle Scholar
  591. 588.
    Noblett HR (1969) Treatment of uncomplicated meconium ileus by gastrografin enema: a preliminary report. J Pediatr Surg 4: 190–197PubMedGoogle Scholar
  592. 589.
    Nolte D (1973) Spironolacton-Wirkung und Lunge. Dtsch Med Wochenschr 98: 2406–2407PubMedGoogle Scholar
  593. 590.
    Norman AP, Hall M (1971) Nocturnal mist therapy in cystic fibrosis. Practitioner 206: 786–790PubMedGoogle Scholar
  594. 591.
    Nousia-Arvanitakis S, Stapleton FB, Linshaw MA, Kennedy J (1977) Therapeutic approach to pancreatic extract-induced hyperuricosuria in cystic fibrosis. J Pediatr 90: 302–305Google Scholar
  595. 592.
    Nousia-Arvanitakis S, Arvanitakis C, Nila D, Greenberger NJ (1978) Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by the synthetic peptide N-benzoyl-L-tyrosyl-p-aminobenzoid acid. J Pediatr 92: 734–737PubMedGoogle Scholar
  596. 593.
    Oehlert W (1957) Die Leberzirrhose bei der cystischen Pancreasfibrose des Säuglings. Beitr Pathol 117: 253–265Google Scholar
  597. 594.
    Ogilvie D, McCollum JPK, Packer S, Manning J, Oyesiku J, Muller DPR, Harries JT (1976) Urinary outputs of oxalate, calcium and magnesium in children with intestinal disorders: a potential cause of renal calculi. Arch Dis Child 51: 790–795PubMedGoogle Scholar
  598. 595.
    Olim CB, Cutait A (1954) Meconium ileus: new method of relieving obstruction. Ann Surg 140: 736–740PubMedGoogle Scholar
  599. 596.
    Oppenheimer EH (1972) Glomerular lesions in cystic fibrosis: possible relation to diabetes mellitus, acquired cyanotic heart disease and cirrhosis of the liver. Johns Hopkins Med J 131: 351 –366Google Scholar
  600. 597.
    Oppenheimer EH, Esterly JR (1962) Observations in cystic fibrosis of the pancreas: II. Neonatal intestinal obstruction. Bull Johns Hopkins Hosp 111: 1 –13Google Scholar
  601. 598.
    Oppenheimer EH, Esterly JR (1970) Observations in cystic fibrosis of the pancreas. VI. The uterine cervix. J Pediatr 77: 991–995Google Scholar
  602. 599.
    Oppenheimer EH, Esterly JR (1973a) Myocardial lesions in patients with cystic fibrosis of the pancreas. Johns Hopkins Med J 133: 252–261PubMedGoogle Scholar
  603. 600.
    Oppenheimer EH, Esterly JR (1973b) Cystic fibrosis of the pancreas: morphologic findings in infants with and without diagnostic pancreatic lesions. Arch Pathol 96: 149–154PubMedGoogle Scholar
  604. 601.
    Oppenheimer EH, Esterly JR (1975a) Pathology of cystic fibrosis. Review of the literature and comparison with 146 autopsied cases. Perspect Pediatr Pathol 2: 241–278PubMedGoogle Scholar
  605. 602.
    Oppenheimer EH, Esterly JR (1975b) Hepatic changes in young infants with cystic fibrosis. Possible relation to focal biliary cirrhosis. J Pediatr 86: 683–689Google Scholar
  606. 603.
    Orenstein DM, Boat TF, Stern RC, Tucker AS, Charnock Le Roy EL, Matthews W, Doershuk CF (1977) The effect of early diagnosis and treatment in cystic fibrosis. Am J Dis Child 131: 973–975PubMedGoogle Scholar
  607. 604.
    Pallavicini J, Wiesmann U, Uhlendorf WB, Sant’Agnese PA di (1970) Glycogen content of tissue culture fibroblasts from patients with cystic fibrosis and other heritable disorders. J Pediatr 77: 280–284PubMedGoogle Scholar
  608. 605.
    Parkins RA, Eidelman S, Rubin CE, Dobbins WO, Phelps PC (1963) The diagnosis of cystic fibrosis by rectal suction biopsy. Lancet II: 851–856Google Scholar
  609. 606.
    Parks CR, Woodrum DE, Graham CB, Cheney FW, Hodson WA (1971) Effect of water nebulization on normal canine pulmonary mucociliary clearance. Am Rev Respir Dis 104: 99–106PubMedGoogle Scholar
  610. 607.
    Parks CR, Alden ER, Standaert TA, Woodrum DE, Graham CB, Hodson WA (1973) The effect of water nebulization on cough transport of pulmonary mucus in the mouth breathing dog. Am Rev Respir Dis 108: 513–519PubMedGoogle Scholar
  611. 608.
    Parry MF, Neu HC (1976) Tobramycin and ticarcillin therapy for exacerbations of pulmonary disease in patients with cystic fibrosis. J Infect Dis (Suppl) 134: 194–197Google Scholar
  612. 609.
    Parry MF, Neu HC, Merlino M, Gaerlan PF, Ores CN, Denning CR (1977) Treatment of pulmonary infections in patients with cystic fibrosis: a comparative study of ticarcillin and gentamicin. J Pediatr 90: 144–148PubMedGoogle Scholar
  613. 610.
    Passarge E (1978) Cystische Fibrose, Genetik und Erbberatung. Monatsschr Kinderheilkd 126: 172–173PubMedGoogle Scholar
  614. 611.
    Patterson PR, Denning C, Kutscher AH (eds) (1973) Psychosocial aspects of cystic fibrosis. Columbia University Press, New YorkGoogle Scholar
  615. 612.
    Pennington JE (1974) Preliminary investigations of Pseudomonas aeruginosa vaccine in patients with leukemia and cystic fibrosis. J Infect Dis (Suppl) 130: 159– 162Google Scholar
  616. 613.
    Pennington JE, Reynold HY (1973) Concentrations of gentamycin and carbenicillin in bronchial secretions. J Infect Dis 128: 63–68PubMedGoogle Scholar
  617. 614.
    Peter G, Whang-Peng J (1968) Chromosomes in cystic fibrosis. Lancet I: 978–979Google Scholar
  618. 615.
    Petersen RA, Petersen VS, Robb RM (1968) Vitamin A deficiency with xerophtalmia and night blindness in cystic fibrosis. Am J Dis Child 116: 662–663PubMedGoogle Scholar
  619. 616.
    Pfyffer JX (1848) Wörterbuch der schweizerdeutschen Sprache, vol 7, p 899Google Scholar
  620. 617.
    Phair JP, Tan JS, Watanakunakorn C, Schwab L, Sanders LW (1970) Carbenicillin treatment of Pseudomonas pulmonary infection. Use in children with cystic fibrosis. Am J Dis Child 120: 22–25Google Scholar
  621. 618.
    Phelan PD (1973) Management of children with cystic fibrosis. Aust Paediatr J 9: 302–306PubMedGoogle Scholar
  622. 619.
    Phelan PD, Gracey M, Williams HE, Anderson CM (1969) Ventilatory function in infants with cystic fibrosis. Physiological assessment of inhalation therapy. Arch Dis Child 44: 393–400Google Scholar
  623. 620.
    Piepsz A, Decastre P, Baran D (1973) Scintigraphic study of pulmonary blood flow distribution in cystic fibrosis. J Nucl Med 14: 326–330PubMedGoogle Scholar
  624. 621.
    Platt MW, Rao GJS, Nadler HL (1978) Reaction of 4-methylumbelliferylguanidino- benzoate with cultivated human skin fibroblasts derived from patients with cystic fibrosis. Pediatr Res 12: 874–877PubMedGoogle Scholar
  625. 622.
    Polley M (1974) Ciliary inhibitory factors. Cystic Fibrosis GAP Conference reportGoogle Scholar
  626. 623.
    Polley MJ, Beam AG (1974) Cystic fibrosis: current concepts. J Med Genet 11: 249–252PubMedGoogle Scholar
  627. 624.
    Porte D, Pupo AA (1969) Insulin response to glucose: evidence for a two pool system in man. J Clin Invest 48: 2309–2319PubMedGoogle Scholar
  628. 625.
    Porter RC, Cloutier MM, Brasfield DM, Mangos JA, Miale T, Tiller RE (1979) Cystic fibrosis in two black children with sickle cell anemia. J Pediatr 94: 239–240PubMedGoogle Scholar
  629. 626.
    Posselt HG, Bender SW (1971) Heterozygote testing in cystic fibrosis. Z Kinderheilkd 110: 93–98PubMedGoogle Scholar
  630. 627.
    Posselt HG, Bender SW (1978) Gastroenterologische Probleme der Mucoviscidose. Monatsschr Kinderheilkd 126: 161–164PubMedGoogle Scholar
  631. 628.
    Posselt HG, Bender SW (to be published) MeconiumIleus-Equivalent und Invagination: Spätkomplikationen intestinaler Obstruktionen bei MucoviscidoseGoogle Scholar
  632. 629.
    Potter JL, Spector S, Matthews LW, Lemm J (1969) Studies on pulmonary secretions. III. Am Rev Respir Dis 99: 909–916Google Scholar
  633. 630.
    Problems in sweat testing (1975). GAP Conference reports. Cystic Fibrosis Foundation, AtlantaGoogle Scholar
  634. 631.
    Prosser R (1979) Screening for cystic fibrosis in the newborn. Monogr Paediatr 10: 43–45PubMedGoogle Scholar
  635. 632.
    Quissel DO, Pitot HC (1974) Number of ouabain-binding sites in fibroblasts from normal subjects and patients with cystic fibrosis. Nature 247: 115–116Google Scholar
  636. 633.
    Rachelefsky GS, Osher A, Dooley RE, Ank B, Stiehm ER (1974) Coexistent respiratory allergy and cystic fibrosis. Am J Dis Child 128: 355–359PubMedGoogle Scholar
  637. 634.
    Rao GJS, Nadler HL (1972) Deficiency of trypsin-like activity in saliva of patients with cystic fibrosis. J Pediatr 80: 573–576PubMedGoogle Scholar
  638. 635.
    Rao GJS, Nadler HL (1974) Arginine esterase in cystic fibrosis of the pancreas. Pediatr Res 8: 684–686PubMedGoogle Scholar
  639. 636.
    Rauch S, Litvak AM, Steiner M (1939) Congenital familial steatorrhea with fibromatosis of the pancreas and bronchiolectasis. J Pediatr 14: 462–490Google Scholar
  640. 637.
    Raynaud EJ, Hennequet A, Menut G, Lavignon A, Malpuech G, Lagarade R (1971) Ictere retentional neonatal revelateur de la mucociscidose. Arch Fr Pediatr 28: 885–886PubMedGoogle Scholar
  641. 638.
    Reas HW (1963) The effect of N-acetylcysteine on the viscosity of tracheobronchial secretions in cystic fibrosis of the pancreas. J Pediatr 62: 31–35PubMedGoogle Scholar
  642. 639.
    Reas HW (1964) The use of N-acetylcysteine in the treatment of cystic fibrosis. J Pediatr 65: 542–557PubMedGoogle Scholar
  643. 640.
    Reddy CR, Devi C, Anees AM, Murthy DP, Reddy GE (1969) Cystic fibrosis of pancreas in India. J Pediatr 75: 522–523PubMedGoogle Scholar
  644. 641.
    Reed GB, Bain AD, McRae WM, Scott FM (1970) Cellular metachromasia in cystic fibrosis. J Pathol 101: 251–257PubMedGoogle Scholar
  645. 642.
    Reemtsma K, Sant’Agnese PA di, Malm JR, Barker HG (1958) Cystic fibrosis of the pancreas: intestinal absorption of fat and fatty acid labeled with J-131. Pediatrics 22: 525–532PubMedGoogle Scholar
  646. 643.
    Regan PT, Malagelada JF, Magno EP di, Glanzman SL, Go VLW (1977a) Comparative effects of antacids, Cimetidine and enteric coating on the therapeutic response of oral enzymes in severe pancreatic insufficiency. N Engl J Med 297: 854–858PubMedGoogle Scholar
  647. 644.
    Regan PT, Malagelada JR, Magno EP di, Go VLM (1977b) Fate and efficacy of oral enzymes in pancreatic insufficiency: effects of neutralizing antacids, Cimetidine and enteric coating (abstr A 95). Gastroenterology 72: 1118Google Scholar
  648. 645.
    Regenbrecht J, Hadid D (1969) Neuere Gesichtspunkte zur Behandlung der Mucoviscidose, insbesondere des MeconiumIleus. Z Kinderchir 7: 394–402Google Scholar
  649. 646.
    Rehbein F, Halsband H (1968) A double-tube technique for the treatment of meconium ileus and small bowel atresia. J Pediatr Surg 3: 723–726Google Scholar
  650. 647.
    Reid L (1970) Sputum viscosity in cystic fibrosis. Lancet II: 423–424Google Scholar
  651. 648.
    Reid L, Haller R de (1967) The bronchial mucous glands — their hypertrophy and change in intracellular mucus. Mod Probi Paediatr 10: 195–199Google Scholar
  652. 649.
    Reilly BJ, Featherby EA, Weng T-R, Crozier DN, Duic A, Levison H (1971) The correlation of radiological changes with pulmonary function in cystic fibrosis. Radiology 98: 281–285PubMedGoogle Scholar
  653. 650.
    Remy J, Arnaud H, Fardou H, Giraud R, Voisin C (1977) Treatment of hemoptysis by embolization of bronchial arteries. Radiology 122: 33–37PubMedGoogle Scholar
  654. 651.
    Rennert O, Frias J, LaPointe D (1977) Methylation of RNA and polyamine metabolism in cystic fibrosis. In: Mangos JA, Talamo RC (eds) Fundamental problems of cystic fibrosis and related diseases. International Medical Book, New York London, pp 45–52Google Scholar
  655. 652.
    Report (1976) of the committee for a study for evaluation of testing for cystic fibrosis. J Pediatr 88: 711–750Google Scholar
  656. 653.
    Report (1978) on survival studies of patients with cystic fibrosis. Cystic Fibrosis Foundation, AtlantaGoogle Scholar
  657. 654.
    Rich RH, Leonard AS (1978) Open thoracotomy and pleural abrasion in the treatment of spontaneous pneumothorax in cystic fibrosis. J Pediatr Surg 13: 237–242PubMedGoogle Scholar
  658. 655.
    Rickham PP, Boeckman CR (1965) Neonatal meconium obstruction in the absence of mucoviscidosis. Am J Surg 109: 173–177PubMedGoogle Scholar
  659. 656.
    Riddell RW (1969) Fungi associated with bronchial infections. Proc. 5th Int. Cyst. Fibrosis Conf., Lawson D (ed), Cambridge (UK). Cystic Fibrosis Research Trust, London, pp 189–193Google Scholar
  660. 657.
    Ristow SC, Condemi JJ, Stuard ID, Schwartz RH, Bryson MF (1977) Systemic amyloidosis in cystic fibrosis. Am J Dis Child 131: 886–888PubMedGoogle Scholar
  661. 658.
    Roberts WC (1962) The hepatic cirrhosis of cystic fibrosis of the pancreas. Am J Med 32: 324–328PubMedGoogle Scholar
  662. 659.
    Robinson PG, Elliott RB (1976) Cystic fibrosis screening in the newborn. Arch Dis Child 51: 301–304PubMedGoogle Scholar
  663. 660.
    Robinson GC, Miller JR, Bensimon JR (1962) Familiae ectodermal dysplasia with sensorineural deafness and other anomalies. Pediatrics 30: 797–800Google Scholar
  664. 661.
    Robinson ME, Davies V, Williams EM, Brown KM, Dodge J (1977) The short term changes seen in pulmonary function before and after physiotherapy in young patients with cystic fibrosis. In: Baran D, Bogaert E van (eds) Chest physical therapy in cystic fibrosis and obstructive pulmonary diseases. European Press, Ghent, pp 222–229Google Scholar
  665. 662.
    Robson AM, Tateishi S, Ingelfinger JR, Strominger DB, Klahr S (1971) Renal function in patients with cystic fibrosis. J Pediatr 79: 42–50PubMedGoogle Scholar
  666. 663.
    Rochholz EL (1857) Alemannisches Kinderlied und Kinderspiel aus der Schweiz. Weber Verlagsbuchhandlung, Leipzig, S 280Google Scholar
  667. 664.
    Roelfs RE, Gibbs GE, Griffin GD (1967) The composition of rectal mucus in cystic fibrosis. Am J Dis Child 113: 419–421PubMedGoogle Scholar
  668. 665.
    Röttger P (1976) Pathologische Anatomie. In: Forell MM (ed) Pankreas. Springer, Berlin Heidelberg New York (Handbuch der inneren Medizin, 5th edn, vol 3 /6, p 891 )Google Scholar
  669. 666.
    Rogers RM, Braunstien MS, Shuman JF (1972) Role of bronchopulmonary lavage in the treatment of respiratory failure. A review. Chest (Suppl) 62: 95–106Google Scholar
  670. 667.
    Roomans GM, Afzelius BA, Kollberg H, Forslind B (1978) Electrolytes in nails analyzed by X-ray microanalysis in electron microscopy. Acta Paediatr Scand 67: 89–94PubMedGoogle Scholar
  671. 668.
    Rosan RC, Shwachman H, Kulczycki LL (1962) Diabetes and cystic fibrosis of the pancreas. Am J Dis Child 104: 625–633PubMedGoogle Scholar
  672. 669.
    Roscher A, Wiesman U, Hadorn B (1979) Relevance of second messenger in cystic fibrosis. Monogr Paediatr 10: 77–83PubMedGoogle Scholar
  673. 670.
    Roschlau G (1978) Leberbiopsie im Kindesalter. Fischer, JenaGoogle Scholar
  674. 671.
    Rosenbluth M, Chernick V (1974) Influence of mist tent therapy on sputum viscosity and water content in cystic fibrosis. Arch Dis Child 49: 606–610PubMedGoogle Scholar
  675. 672.
    Rosenfeld R, Spigelblatt L, Chicoine A (1969) False positive sweat test, malnutrition, and the Mauriac syndrome. J Pediatr 94: 240–242Google Scholar
  676. 673.
    Rosenlund ML, Kim HK, Kritchevsky D (1974) Essential fatty acids in cystic fibrosis. Nature 251: 719PubMedGoogle Scholar
  677. 674.
    Rosenstein BJ, Oppenheimer EH (1977) Prolonged obstructive jaundice and giant cell hepatitis in an infant with cystic fibrosis. J Pediatr 91: 1022–1023PubMedGoogle Scholar
  678. 675.
    Rosenthal A, Tucker CR, Williams RG, Khaw K-T, Strieder D, Shwachman H (1976) Echocardiographic assessment of cor pulmonale in cystic fibrosis. Pediatr Clin North Am 23: 327–344PubMedGoogle Scholar
  679. 676.
    Rossmann C, Dolovich J, Dolovich M, Wilson W, Newhouse M (1977) Cystic fibrosis — related inhibition of mucociliary clearance in vivo in man. J Pediatr 90: 579– 584Google Scholar
  680. 677.
    Rothstein RJ, Pinney MA, Buckley JM, Cotton EK (1974) Evaluation of reactive airway disease in atopic cystic fibrosis patients. J Allergy Clin Immunol 53: 100Google Scholar
  681. 678.
    Rotter J, Kaiser D (1975) Einfache Chloridaktivitätsmessung auf der Haut zur Diagnose der Mucoviscidose. Z Kinderheilkd 120: 135–139PubMedGoogle Scholar
  682. 679.
    Rousing H, Sloth K (1973) Microgallbladder and biliary calculi in mucoviscidosis. Acta Radiol (Stockh) 14: 588–592Google Scholar
  683. 680.
    Roy CC, Weber AM, Chartrand L, Lepage G, Morin CL, Lasalle R (1976) Büe acid metabolism in cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  684. 681.
    Rubin LS, Barbero GJ, Chernick WS, Sibinga MS (1963) Pupillary reactivity as a measure of anatomic balance in cystic fibrosis. J Pediatr 63: 1120–1129PubMedGoogle Scholar
  685. 682.
    Rucker RW, Harrison GM (1974) Outpatient intravenous medications in the management of cystic fibrosis. Pediatrics 54: 358–360PubMedGoogle Scholar
  686. 683.
    Rule AH, Kopito L, Shwachman H (1970) Chemical analysis of ejaculates from patients with cystic fibrosis. Fertil Steril 21: 515–520PubMedGoogle Scholar
  687. 684.
    Ryley HC, Neal LM, Brogan TA, Bray PT (1975) Albumin-alpha-1-trypsin inhibitor ratio. Clin Chim Acta 64: 117–125PubMedGoogle Scholar
  688. 685.
    Ryley HC, Neal LM, Lynne M, Brogan TA, Bray PT (1979) Screening for cystic fibrosis in the newborn by meconium analysis. Arch Dis Child 54: 92–97PubMedGoogle Scholar
  689. 686.
    Ryssing E (1977) Assessment of cor pulmonale in cystic fibrosis by echocardiography. Acta Paediatr Scand 66: 753–756PubMedGoogle Scholar
  690. 687.
    SadS J, Eliezer N, Silberberg A, Nevo AC (1970) The role of mucus in transport by cilia. Am Rev Respir Dis 102: 48–52Google Scholar
  691. 688.
    Salem M, Idresh H (1962) Cystic fibrosis of the pancreas in the Middle East. J Med Liban 15: 61–64Google Scholar
  692. 689.
    Samänek M, Houstek J, Vavrovä V, Ruth C, Snobl O (1971) Distribution of pulmonary blood flow in children with cystic fibrosis. Acta Paediatr Scand 60: 149– 157Google Scholar
  693. 690.
    Samuels CE, Elliott RB (1975) Cystic fibrosis: detection of heterozygotes. Lancet II: 607–608Google Scholar
  694. 691.
    Sanchis J, Dolovich M, Rossman C, Wilson W, Newhouse M (1973) Pulmonary mucociliary clearance in cystic fibrosis. N Engl J Med 288: 651–654PubMedGoogle Scholar
  695. 692.
    Sant’Agnese PA di (1953) Bronchial obstruction with lobar atelectasis and emphysema in cystic fibrosis of the pancreas. Pediatrics 12: 178–190Google Scholar
  696. 693.
    Sant’Agnese PA di (1955) Fibrocystic disease of the pancreas with normal or partial pancreatic function. Pediatrics 15: 683 — 696Google Scholar
  697. 694.
    Sant’Agnese PA di (1960) Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. JAMA 172: 2014–2021Google Scholar
  698. 695.
    Sant’Agnese PA di, Blanc WA (1956) A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas. Pediatrics 18: 387–409Google Scholar
  699. 696.
    Sant’Agnese PA di, Davis PB (1976) Research in cystic fibrosis. N Engl J Med 295: 481–485, 534–541, 597–602Google Scholar
  700. 697.
    Sant’Agnese PA di, Davis PA (1979) Cystic fibrosis in adults. Am J Med 66: 121–131Google Scholar
  701. 698.
    Sant’Agnese PA di, Lepore MJ (1961) Involvement of abdominal organs in cystic fibrosis of the pancreas. Gastroenterology 40: 64–74Google Scholar
  702. 699.
    Sant’Agnese PA di, Powell GF (1962) The eccrine sweat defect in cystic fibrosis of the pancreas. Ann NY Acad Sei 93: 555–599Google Scholar
  703. 700.
    Sant’Agnese PA di, Darling RC, Perera A, Shea E (1953) Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 12: 549–563Google Scholar
  704. 701.
    701.Sant’Agnese PA di, Grossman A, Darling RC, Denning CR (1958) Saliva, tears, and duodenal contents in cystic fibrosis of the pancreas. Pediatrics 22: 507–514Google Scholar
  705. 702.
    Santa Cruz R, Landa J, Hirsch J, Sackner MA (1974) Tracheal mucus velocity in normal man and patients with obstructive lung disease: effects of terbutaline. Am Rev Respir Dis 109: 458–463PubMedGoogle Scholar
  706. 703.
    Santulli TV (1969) Meconium ileus. In: Mustard WR, Ravitch MM, Synder WH, Welch KJ, Benson CD (eds) Paediatric surgery, 2nd edn, vol I I. Year Book Medical Publishers, ChicagoGoogle Scholar
  707. 704.
    Sarsfield JK, Davies JM (1975) Negative sweat tests and cystic fibrosis. Arch Dis Child 50: 463–466PubMedGoogle Scholar
  708. 705.
    Saunders JHB, Wormsley KG (1975) Pancreatic extracts in the treatment of pancreatic exocrine insufficiency. Gut 16: 157–162PubMedGoogle Scholar
  709. 706.
    Saunders JHB, Drummond S, Wormsley KG (1977) Inhibition of gastric secretion in treatment of pancreatic insufficiency. Br Med J 1: 418–419PubMedGoogle Scholar
  710. 707.
    Scanlin TF Jr, Norman ME, Rosenlund ML (1974) C3 in cystic fibrosis. Lancet I: 1382Google Scholar
  711. 708.
    Schaap T, Cohen MM (1976) A proposed model for the inheritance of cystic fibrosis. In: Mangos JA, Talamo RC (eds) Cystic fibrosis, projections into the future. Intercontinental Medical Book, New York London, pp 291–303Google Scholar
  712. 709.
    SchiØtz PU, HØiby N, Juhl F, Permin H, Nielsen H, Svehag SE (1977) Immune complexes in cystic fibrosis. Acta Pathol Microbiol Scand (C) 85: 57–64Google Scholar
  713. 710.
    Schi00D8tz PU, Nielsen H, HØiby N, Glikmann G, Svehag SE (1978) Immune complexes in the sputum of patients with cystic fibrosis suffering from chronic Pseudomonas aeruginosa lung infection. Acta Pathol Microbiol Scand (C) 86: 37–40Google Scholar
  714. 711.
    Schlesinger L, Stekel A (1974) Impaired cellular immunity in marasmic infants. Am J Clin Nutr 27: 615–620PubMedGoogle Scholar
  715. 712.
    Schmidt JG (1729) Die gestriegelte Rockenphilosophie, 4. Aufl. Christoph und Stößeln, ChemnitzGoogle Scholar
  716. 713.
    Schmoyer IR, Baglia FA (1974) Cystic fibrosis: effect of media from cultured cystic fibrosis fibroblast on ATPase activity. Biochem Biophys Res Commun 58: 1066PubMedGoogle Scholar
  717. 714.
    Schmoyer IR, Fischer JF, Brooks SP (1972a) Fractionation of oyster cilia inhibitor from cystic fibrosis heterozygote serum. Biochem Biophys Res Commun 46: 1923– 1927Google Scholar
  718. 715.
    Schmoyer IR, Brooks SP, Fischer JF (1972b) Isolation and characterization of a ciliary dyskinetic factor from cystic fibrosis heterozygous serum. Life Sei 11 II: 1037–1044Google Scholar
  719. 716.
    Schöni M, Kaiser D, Drack E (1975) Excretion of trypsin-like activity, electrolytes and protein in mixed and parotid saliva of patients with cystic fibrosis of the pancreas. Eur J Clin Invest 5: 153–158PubMedGoogle Scholar
  720. 717.
    Schulz J (1969) Micropuncture studies of sweat formation in cystic fibrosis patients. J Clin Invest 48: 1470–1477PubMedGoogle Scholar
  721. 718.
    Schulz J, Froemter E (1968) Mikropunktionsuntersuchungen an Schweißdrüsen von Mukoviscidosepatienten und gesunden Versuchspersonen. In: Windorf er A, Stephan U (Hrsg) Mucoviscidose. 2. Deutsches Symposium Erlangen. Thieme, Stuttgart, S 12–21Google Scholar
  722. 719.
    Schulz J, Ullrich KJ, Froemter E, Holzgreve H, Frick A, Hegel U (1965) Mikropunktion und elektrische Potentialmessung an Schweißdrüsen des Menschen. Pfluegers Arch 284: 360Google Scholar
  723. 720.
    Schuster SP (1972) Surgical aspects of therapy of pneumothorax. In: Pulmonary complications in cystic fibrosis. National Cystic Fibrosis Research Foundation, Atlanta, p 14Google Scholar
  724. 721.
    Schutt WH, Isles TE (1968) Protein in meconium from meconium ileus. Arch Dis Child 43: 178–181PubMedGoogle Scholar
  725. 722.
    Schwartz RH (1966) Serum immunoglobulin levels in cystic fibrosis. Am J Dis Child 111: 408–411PubMedGoogle Scholar
  726. 723.
    Schwartz EE, Holsclaw DS (1974) Pulmonary involvement in adults with cystic fibrosis. AJR 122: 708–718Google Scholar
  727. 724.
    Schwartz RH, Johnstone DE, Holsclaw DS, Dooley RR (1970) Serum precipitins to aspergillus in cystic fibrosis. Am J Dis Child 120: 432–433PubMedGoogle Scholar
  728. 725.
    Schwarz V, Sutcliffe CH, Style PP (1968) Some hazards of the sweat test. Arch Dis Child 43: 695–701PubMedGoogle Scholar
  729. 726.
    Seale TW, Tirkill H, Tarpay M, Flux M, Rennert OM (1979) Serotypes and antibiotic susceptibilities of Pseudomonas aeruginosa isolates from single sputa of cystic fibrosis patients. J Clin Microbiol 9: 72–78PubMedGoogle Scholar
  730. 727.
    Seidmon EJ, Mosovich LL, Neter E (1975) Colonization by enterobacteriaceae of the respiratory tract of children with cystic fibrosis of the pancreas and their antibody response. J Pediatr 87: 528–533PubMedGoogle Scholar
  731. 728.
    Seliger G, Firooznia H, Genieser NB (1972) Intestinal obstruction in an adult with cystic fibrosis. Am J Dig Dis 17: 934–938PubMedGoogle Scholar
  732. 729.
    Shapira E, Rao GJS, Wessel HU, Nadler HL (1976) Absence of an alpha-2-macro- globulin-protease complex in cystic fibrosis. Pediatr Res 10: 812–817PubMedGoogle Scholar
  733. 730.
    Shapiro BL, Lee SM, Warwick WJ (1970) The pentose phosphate pathway in cystic fibrosis erythrocytes. Biochem Biophys Res Commun 39: 816–821PubMedGoogle Scholar
  734. 731.
    Shaw A (1969) Safety of N-acetylcysteine in the treatment of meconium obstruction of the newborn. J Pediatr Surg 4: 119–125PubMedGoogle Scholar
  735. 732.
    Shaw A, Cooperman A, Fusco J (1967) Gas embolism produced by hydrogen peroxide. N Engl J Med 277: 238–341PubMedGoogle Scholar
  736. 733.
    Sheffner AL, Medler EM, Jacobs LW, Sarett HP (1964) The in vitro reduction in viscosity of human tracheobronchial secretions by acetylcysteine. Am Rev Respir Dis 90: 721–727PubMedGoogle Scholar
  737. 734.
    Sherr HP, Nair PP, White JJ, Banwell JG, Lockwood DH (1974) Bile acid metabolism and hepatic disease following small bowel bypass for obesity. Am J Clin Nutr 27: 1369–1379PubMedGoogle Scholar
  738. 735.
    Shmerling DH, Forrer JCW, Prader A (1970) Fecal fat and nitrogen in healthy children and in children with malabsorption or maldigestion. Pediatrics 46: 690– 695Google Scholar
  739. 736.
    Shwachman H (1974) Changing concept of cystic fibrosis. Hosp Pract 9: 143–150Google Scholar
  740. 737.
    Shwachman H, Holsclaw D (1969) Complications of cystic fibrosis. N Engl J Med 281: 500–501PubMedGoogle Scholar
  741. 738.
    Shwachman H, Holsclaw D (1972) Examination of the appendix at laparotomy as a diagnostic clue in cystic fibrosis. N Engl J Med 286: 1300–1301PubMedGoogle Scholar
  742. 739.
    Shwachman H, Kulczycki LL (1958) Long-term study of 105 patients with cystic fibrosis. Am J Dis Child 96: 6–15Google Scholar
  743. 740.
    Shwachman H, Farber S, Maddock CL (1943) Pancreatic function and disease in early life. III. Methods of analysing pancreatic enzyme activity. Am J Dis Child 66: 418–424Google Scholar
  744. 741.
    Shwachman H, Leubner H, Catzel P (1955) Mucoviscidosis. Adv Pediatr 7: 249– 323Google Scholar
  745. 742.
    Shwachman H, Dooley RR, Guilmette F, Patterson PR, Weil C, Leubner H (1956a) Cystic fibrosis of the pancreas with varying degrees of pancreatic insufficiency. Am J Dis Child 92: 347–368Google Scholar
  746. 743.
    Shwachman H, Pryles CF, Gross RE (1956b) Meconium ileus, a clinical study of twenty surviving patients. Am J Dis Child 91: 223–244.Google Scholar
  747. 744.
    Shwachman H, Kulczycki LL, Mueller HL, Flake CG (1962) Nasal polyposis in patients with cystic fibrosis. Pediatrics 30: 389–401PubMedGoogle Scholar
  748. 745.
    Shwachman H, Dunham R, Philips WR (1963) Electrical conductivity of sweat. Pediatrics 32: 85–88PubMedGoogle Scholar
  749. 746.
    Shwachman H, Redmond A, Khaw KT (1970) Studies in cystic fibrosis report of 130 patients diagnosed under 3 months of age over a 20-year period. Pediatrics 46: 335–342PubMedGoogle Scholar
  750. 747.
    Shwachman H, Stamm SJ, Doctor JM, Murphy TF, Mahmoodian A, Kennedy JL, Grimes G (1973) Screening tests for newborns for cystic fibrosis using the presence of albumin in meconium as an indicator. In: Mangos JA, Talamo CR (eds) Fundamental problems of cystic fibrosis and related diseases. Intercontinental Medical Book, New York London, p 279Google Scholar
  751. 748.
    Shwachman H, Lebenthal E, Khaw K-T (1975a) Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes. Pediatrics 55: 86–95PubMedGoogle Scholar
  752. 749.
    Shwachman H, Khaw K-T, Kowalski SM (1975b) The management of cystic fibrosis: observations on gentamicin when used with other antibiotics in pulmonary infections associated with cystic fibrosis. Clin Pediatr (Phila) 14: 1115–1118Google Scholar
  753. 750.
    Shwachman H, Kowalski M, Khaw K-T (1977) Cystic fibrosis: a new outlook. 70 patients above 25 years of age. Medicine (Baltimore) 56: 129–149Google Scholar
  754. 751.
    Shwachman H, Antonowicz I, Mahmoodian A, Ishida S (1978) Studies in meconium: an approach to screening tests to detect cystic fibrosis. Am J Dis Child 132: 1112– 1114Google Scholar
  755. 752.
    Siafarikas K, Glaubitt D (1975) Kinetik des Wachstumshormons unter Stimulation bei Kindern mit Mukoviscidose. Saarl Ärztblatt 562v565Google Scholar
  756. 753.
    Siassi B, Moss AJ, Dooley RR (1971) Clinical recognition of cor pulmonale in cystic fibrosis. J Pediatr 78: 794–805PubMedGoogle Scholar
  757. 754.
    Sibert JR (1976) Cystic fibrosis in summer. Lancet II: 696Google Scholar
  758. 755.
    Silverman M, Hobbs FDR, Gordon IRS, Carswell F (1978) Cystic fibrosis, atopy and airways lability. Arch Dis Child 53: 873–877PubMedGoogle Scholar
  759. 756.
    Simopoulos AP, Pak CYC, Kattwinkel J (1971) Calcium metabolism of parathyroid function in cystic fibrosis (abstr). American Pediatric Society and Society for Pediatric Research, Atlantic City, N.J., p 201Google Scholar
  760. 757.
    Sitzmann FC, Stephan U (1967) Untersuchungen zur Frage der Leberbeteiligung bei cystischer Pankreasfibrose im Kindesalter. Arch Kinderheilkd 175: 263–270PubMedGoogle Scholar
  761. 758.
    Skorecki K, Levison H, Crozier DN (1976) Bronchial lability in cystic fibrosis. Acta Paediatr Scand 65: 39–44PubMedGoogle Scholar
  762. 759.
    Sleigh MA (1977) The nature and action of respiratory tract cilia. In: Brain JD, Proctor DF, Reid LM (eds) Respiratory defense mechanisms. Dekker, New York Basel, pp 247–288Google Scholar
  763. 760.
    Sloth K (1972) Liver and biliary changes in cystic fibrosis. Acta Paediatr Scand 61: 380Google Scholar
  764. 761.
    Small DH (1975) The formation and treatment of gallstones. In: Schiff L (ed) Diseases of the liver. Lippincott, PhiladelphiaGoogle Scholar
  765. 762.
    Smalley CA, Brown GA, Parkes M, Tease H, Anderson CM (1976) Fat and bile acid metabolism in cystic fibrosis in relation to pancreatic extracts and medium chain triglycerides. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  766. 763.
    Smith DW, Doctor JM, Ferrier PE, Frias JL, Spöck A (1978) Possible localisation of the gene for cystic fibrosis of the pancreas to the short arm of chromosome 5. Lancet II: 309Google Scholar
  767. 764.
    Smith QT, Shapiro BL, Hamilton MJ (1972) Lack of differences in serum binding of calcium in cystic fibrosis. Nature 240: 56–57Google Scholar
  768. 765.
    Sorensen RU, Stern RC, Polmar SH (1977) Cellular immunity to bacteria: impairment of in vitro lymphocyte responses to Pseudomonas aeruginosa in cystic fibrosis patients. Infect Immun 18: 735–740PubMedGoogle Scholar
  769. 766.
    Sorensen RU, Stern RC, Polmar SH (1978) Lymphocyte responsiveness to Pseudomonas aeruginosa in cystic fibrosis — relationship to status of pulmonary disease in sibling pairs. J Pediatr 93: 201–205PubMedGoogle Scholar
  770. 767.
    South MA, Warwick WJ, Wollheim FA, Good RA (1967) The IgA system. IgA levels in the serum and saliva of pediatric patients — evidence for a local immunological system. J Pediatr 71: 645–653Google Scholar
  771. 768.
    Spencer H (1977) Pathology of the lung, 3rd edn. Pergamon Press, Oxford, p 146Google Scholar
  772. 769.
    Spöck A, Heick HM, Cress H, Logan WS (1967) Abnormal serum factor in patients with cystic fibrosis of the pancreas. Pediatr Res 1: 173–177PubMedGoogle Scholar
  773. 770.
    Stahl M, Girard J, Rutishauser M, Nars PW, Zuppinger K (1974) Endocrine function of the pancreas: evidence for an impaired glucagon and insulin response following arginine infusion. J Pediatr 84: 821–824PubMedGoogle Scholar
  774. 771.
    Stapleton FB, Kennedy J, Nousia-Arvanitakis S, Linshaw MA (1976) Hyperuricosuria due to high-dose pancreatic extract therapy in cystic fibrosis. N Engl J Med 295: 246–248PubMedGoogle Scholar
  775. 772.
    Steele WM, Rodnan JB, Wood OL (1973) Diminished response to phytohemagglutinin by peripheral lymphocytes cultured from patients with cystic fibrosis. Pediatr Res 7: 394Google Scholar
  776. 773.
    Steele WM, Rodnan JB, Wood OL (1974) Instability of the cell membrane of peripheral lymphocytes cultured from patients with cystic fibrosis. Int Res Commun Syst 2: 1614Google Scholar
  777. 774.
    Stein AA, Porta E, Powers S, Leather R, Linton P, Patterson PR (1963) Studies on surgical biopsies of pancreas and liver in four cases of cystic fibrosis. Ann Surg 157: 516–524PubMedGoogle Scholar
  778. 775.
    Stein AA, Manlapas FC, Soike KF, Patterson PR (1964) Specific isoantibodies in cystic fibrosis. J Pediatr 65: 495–500PubMedGoogle Scholar
  779. 776.
    Steinrud J, Winkel S, Flensborg EW (1974) Screening for cystic fibrosis with chloride electrode. Dan Med Bull 21: 251–255PubMedGoogle Scholar
  780. 777.
    Stephan U (1975) Psychosoziale Aspekte der Mukoviscidose. Gentner, Stuttgart (Schriftenreihe Arbeitsmedizin, Sozialmedizin, Präventivmedizin, Bd56,S 131–135 )Google Scholar
  781. 778.
    Stephan U (1979) Progress in screening. Monogr Paediatr 10: 41–42PubMedGoogle Scholar
  782. 779.
    Stephan U, Brehm G (1969) Zur Diagnose der Mukoviscidose (cystische Fibrose). Dtsch Med Wochenschr 94: 1677–1679PubMedGoogle Scholar
  783. 780.
    Stephan U, Busch EW, Kollberg H, Hellsing K (1975) Cystic fibrosis detection by means of a test-strip. Pediatrics 55: 35–38PubMedGoogle Scholar
  784. 781.
    Stephan U, Bray PF, Kollberg H (1977) Report of the screening committee of the EWGCF, concerning the international study using BM-Test Meconium. 7th Annual Meeting EWGCF, Dresden, GDR June 20 /21, 1977Google Scholar
  785. 782.
    Stephan U, Bauer G, Böwing B, Göring U (1977/78) Erfahrungen eines Behandlungszentrums für Mukoviscidose. Paediatr Prax 19: 391–400Google Scholar
  786. 783.
    Stern RC, Stevens DP, Boat TF, Doershuk CF, Izant RJ, Matthews LW (1976a) Symptomatic hepatic disease in cystic fibrosis: incidence, course and outcome of portal systemic shunting. Gastroenterology 60: 645–649Google Scholar
  787. 784.
    Stern RC, Boat TF, Doershuk CF, Tucker AS, Primiano FP, Matthews LW (1976b) Course of cystic fibrosis in 95 patients. J Pediatr 89: 406–411PubMedGoogle Scholar
  788. 785.
    Stern RC, Boat TF, Doershuk CF, Tucker AS, Miller RB, Matthews LW (1977) Cystic fibrosis diagnosed after age 13. Ann Intern Med 87: 188–191PubMedGoogle Scholar
  789. 786.
    Stern RC, Boat TF, Abramowsky CR, Matthews LW, Wood RE, Doershuk CF (1978a) Intermediate-range sweat chloride concentration and Pseudomonas bronchitis. JAMA 239: 2676–2680PubMedGoogle Scholar
  790. 787.
    Stern RC, Wood RE, Boat TF, Matthews LW, Tucker AS, Doershuk CF (1978b) Treatment and prognosis of massive hemoptysis in cystic fibrosis. Am Rev Respir Dis 117: 825–828PubMedGoogle Scholar
  791. 788.
    Stern RC, Boat TF, Orenstein DM, Wood WE, Matthews LW, Doershuk CF (1978c) Treatment and prognosis of lobar and segmental atelectasis in cystic fibrosis. Am Rev Respir Dis 118: 821–826PubMedGoogle Scholar
  792. 789.
    Stowe SM, Boat TF, Mendelsohn H, Stern RC, Tucker AS, Doershuk CF, Matthews LW (1975) Open thoracotomy for pneumothorax in cystic fibrosis. Am Rev Respir Dis 111: 611–617PubMedGoogle Scholar
  793. 790.
    Strickland AJ (1973) Sweat electrolytes in thyroid disorders. J Pediatr 82: 284– 287Google Scholar
  794. 791.
    Strober W, Peter G, Schwartz RH (1969) Albumin metabolism in cystic fibrosis. Pediatrics 43: 416–426PubMedGoogle Scholar
  795. 792.
    Stur O (1970) Bronchiektasien bei Mukoviscidose (cystische Fibrose). Pneumonologie 143: 174–175PubMedGoogle Scholar
  796. 793.
    Sturgess JM (1977) Bronchial mucus secretion in cystic fibrosis. Mod Probl Paediatr 19: 129–140Google Scholar
  797. 794.
    Sturgess J, Reid L (1973) The effect of isoprenaline and pilocarpine on: (a) bronchial mucussecretory tissue and (b) pancreas, salivary glands, heart, thymus, liver and spleen. Br J Exp Pathol 54: 388–403PubMedGoogle Scholar
  798. 795.
    Sturgess JM, Palfry AJ, Reid L (1970) The viscosity of bronchial secretion. Clin Sei 38: 145–156Google Scholar
  799. 796.
    Sturridge MF (1978) Pulmonary resection for cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1978, pp 355–357Google Scholar
  800. 797.
    Super M (1975) Cystic fibrosis in the south West African Afrikaner. S Afr Med J 49: 818–820PubMedGoogle Scholar
  801. 798.
    Susmano A, Carleton RA (1971) Prevention of hypoxic pulmonary hypertension by chlorpheniramine. J Appl Physiol 31: 531–535PubMedGoogle Scholar
  802. 799.
    Szczepanski Z (1972) Sweat bromide test — a diagnostic tool for mucoviscidosis. Z Kinderheilkd 113: 297–302PubMedGoogle Scholar
  803. 800.
    Szibor R, Koch R (1977) Cystische Fibrose — Screening durch Untersuchungen an Mekonien im zentralen Laboratorium — positive Erfahrungen einer Pilotstudie. Kinderaerztl Prax 45: 193–198PubMedGoogle Scholar
  804. 801.
    Szibor R, Thal W (1976) Schweißkonduktometrie in Zelluloseazetatfolie — ein vereinfachtes Verfahren zur Diagnostik der zystischen Fibrose. Kinderaerztl Prax 44: 209–214PubMedGoogle Scholar
  805. 802.
    Tabolin VA, Fadeeva MA, Dukarshii FG, Korneva TI, Shchegoleva GP, Glotova AV (1972) Functions of the adrenal cortex and C-21-steroid metabolism in children with mucoviscidosis. Vopr Okhr Materin Det 17: 41–44PubMedGoogle Scholar
  806. 803.
    Talamo RC, Hendren WH (1968) Prolonged obstructive jaundice. Report of a case in a neonate with meconium ileus and jejunal atresia. Am J Dis Child 115: 74–79Google Scholar
  807. 804.
    Talamo RC, Stiehm ER, Schwartz RH (1976) Immunologic aspects of cystic fibrosis. In: Mangos JA, Talamo RC (eds) Cystic fibrosis. Grune and Stratton, New YorkGoogle Scholar
  808. 805.
    Tarn CH, Mansell AL, Levison H, Reilly BJ, Aspin N (1973) Dynamic regional lung function studies in patients with asthma and cystic fibrosis. Am Rev Respir Dis 108: 283–293Google Scholar
  809. 806.
    Tataev ZB (1974) A case with mucoviscidosis with the celiac disease syndrom. Pediatria 96Google Scholar
  810. 807.
    Taussig LM (1974) Mist and aerosols: new studies, new thoughts. J Pediatr 84: 619–622Google Scholar
  811. 808.
    Taussig LM, Gardner JD (1972) Effects of saliva and plasma from cystic fibrosis patients on membrane transports. Lancet I: 1367–1368Google Scholar
  812. 809.
    Taussig LM, Kattwinkel J, Sant’Agnese PA di (1973a) A new prognostic score and clinical evaluation system for cystic fibrosis. J Pediatr 82: 380–390PubMedGoogle Scholar
  813. 810.
    Taussig LM, Braunstein GD, White BJ, Christiansen RL (1973b) Silver-Russel dwarfism and cystic fibrosis in a twin. Am J Dis Child 125: 495–499PubMedGoogle Scholar
  814. 811.
    Taussig LM, Belmonte MM, Beaudry PH (1974) Staphylococcus aureus empyema in cystic fibrosis. J Pediatr 84: 724–727PubMedGoogle Scholar
  815. 812.
    Taylor WF, Quaqundah BY (1972) Neonatal jaundice associated with cystic fibrosis. Am J Dis Child 123: 161–162PubMedGoogle Scholar
  816. 813.
    Taylor B, Sokol G (1973) Cystic fibrosis and coeliac disease. Arch Dis Child 48: 692–696PubMedGoogle Scholar
  817. 814.
    Taylor B, Evans JNG, Hope GA (1974a) Upper respiratory tract in cystic fibrosis. Arch Dis Chüd 49: 133–136Google Scholar
  818. 815.
    Taylor A, Mayo JW, Boat TF, Matthews LW (1974b) Standardized assay for the sodium reabsorption inhibitory effect and studies of its salivary gland distribution in patients with cystic fibrosis. Pediatr Res 8: 861–865PubMedGoogle Scholar
  819. 816.
    Taysi K, Kistenmacher ML, Punnett HH, Mellmann WJ (1969) Limitations of metachromasia as a diagnostic aid in pediatrics. N Engl J Med 281: 1108–1111PubMedGoogle Scholar
  820. 817.
    Thal W, Abu-Amna M, Klaer U (1970) Zur Häufigkeit und Diagnose der cystischen Fibrose ( Mucoviscidose) Erfahrungen mit der Nachtschweißgewinnung. Kinderaerztl Prax 38: 514–519Google Scholar
  821. 818.
    Thoenes F (1926) Über familiäre Pancreasinsuffizienz. Monatsschr Kinderheilkd 34: 398–400Google Scholar
  822. 819.
    Thomaidis TS, Arey JB (1963) The intestinal lesions in cystic fibrosis of the pancreas. J Pediatr 63: 444–453PubMedGoogle Scholar
  823. 820.
    Thomas JM, Merritt AD, Hodes ME (1977) Electrophoretic analysis of serum proteins in cystic fibrosis. Pediatr Res 11: 1148–1154PubMedGoogle Scholar
  824. 821.
    Thomson ML, Pavia D, Short MD, Norman AP (1973) Lung clearance in two patients with cystic fibrosis. N Engl J Med 289: 749–750PubMedGoogle Scholar
  825. 822.
    Tomashefski JF, Christoforidis AJ, Abdullah AK (1970) Cystic fibrosis in young adults. Chest 57: 28–36PubMedGoogle Scholar
  826. 823.
    Torriani R, Chassot J, Stocker F, Weber JW (1979) Two children with cystic fibrosis and myocardial fibrosis. Monogr Paediatr 10: 155–157PubMedGoogle Scholar
  827. 824.
    Torstenson OL, Humphrey GB, Edson JR, Warwick WJ (1970) Cystic fibrosis presenting with severe haemorrhage due to vitamin K malabsorption: a report of three cases. Pediatrics 45: 857–861PubMedGoogle Scholar
  828. 825.
    Toskes PP, Hansell J, Cerda J, Deren JJ (1971) Vitamin B12 malabsorption in chronic pancreatic insufficiency. N Engl J Med 284: 627–632PubMedGoogle Scholar
  829. 826.
    Turner MW, Warner OJ, Stokes CR, Norman AP (1978) Immunological studies in cystic fibrosis. Arch Dis Child 53: 631–638PubMedGoogle Scholar
  830. 827.
    Tyson KRT, Schuster SR, Shwachman H (1968) Portal hypertension in cystic fibrosis. J Pediatr Surg 3: 271–277PubMedGoogle Scholar
  831. 828.
    Underwood BA, Denning CR (1972) Blood and liver concentrations of vitamin A and E in children with cystic fibrosis of the pancreas. Pediatr Res 6: 26–31Google Scholar
  832. 829.
    Underwood BA, Denning CR, Navab M (1972) Polyunsaturated fatty acids and tocopherol levels in patients with cystic fibrosis. Ann NY Acad Sei 203: 237–247Google Scholar
  833. 830.
    Valman HB, Evans KE (1970) Serum unbound levels of cloxacillin and erythromycin in cystic fibrosis. Arch Dis Child 45: 686–689PubMedGoogle Scholar
  834. 831.
    Valman HB, France NE, Wallis PG (1971) Prolonged neonatal jaundice in cystic fibrosis. Arch Dis Child 46: 805–809PubMedGoogle Scholar
  835. 832.
    Vanfraechem J, Hostie A (1978) Evaluation de possibilités d’effort chez les enfants atteints de muco viscid ose. Rev Fr Mal Respir 6: 151–158Google Scholar
  836. 833.
    Vawter GF (1972) Pathology and pathogenesis of lesions of respiratory tract. In: Pathology of cystic fibrosis, GAP Conference 1972. National Cystic Fibrosis Research Foundation, AtlantaGoogle Scholar
  837. 834.
    Veeger W, Abels J, Hellemans N, Nieweg HO (1962) Effect of sodium bicarbonate and pancreatin on the absorption of vitamin Bi 2 and fat in pancreatic insufficiency. N Engl J Med 267: 1341–1344PubMedGoogle Scholar
  838. 835.
    Virtanen S (1966) Salivary secretion of ABO blood group substances in cystic fibrosis of the pancreas. J Pediatr 68: 139–141PubMedGoogle Scholar
  839. 836.
    Vlachos P, Liakakos D (1974) Cystic fibrosis with edema and falsely negative sweat test. J Pediatr 84: 926PubMedGoogle Scholar
  840. 837.
    Vossschulte K (1957) Dissektionsligatur des Oesophagus bei Varizen der Speise-röhre infolge Pfortaderhypertonie. Chirurg 28: 186–189PubMedGoogle Scholar
  841. 838.
    Wagget J, Johnson DG, Borns P, Bishop HC (1970) The non-operative treatment of meconium ileus by gastrografin enema. J Pediatr 77: 407–411PubMedGoogle Scholar
  842. 839.
    Wagner KD, Hein J (1976) Psychosoziale Probleme der Mukoviscidose (cystische Fibrose). Kinderaerztl Prax 44: 465–472PubMedGoogle Scholar
  843. 840.
    Wallwork JC, Brenchley P, McCarthy J, Allan JA, Moss D, Ward AM, Holzel A, Williams RR, McFarlane H (1974) Some aspects of immunity in patients with cystic fibrosis. Clin Exp Immunol 18: 303–320PubMedGoogle Scholar
  844. 841.
    Wang CI, Sumi WT, Healy MH, Rissmann EM, Kwok S, Yamazaki JN (1968) Cystic fibrosis in a pure oriental child. Cystic Fibrosis Club (abstr), p 57Google Scholar
  845. 842.
    Wang CL (1972) CF Club (abstr), p 51Google Scholar
  846. 843.
    Wanner A (1977) Clinical aspects of mucociliary transport. Am Rev Respir Dis 116: 73–125PubMedGoogle Scholar
  847. 844.
    Waring WW (1973) Cilia and cystic fibrosis. N Engl J Med 288: 681–682PubMedGoogle Scholar
  848. 845.
    Waring WW, Brunt CH, Hilman BC (1967) Mucoid impaction of the bronchi in cystic fibrosis. Pediatrics 39: 166–175PubMedGoogle Scholar
  849. 846.
    Warner JO, Norman AP, Soothill JF (1976a) Cystic fibrosis heterozygosity in the pathogenesis of allergy. Lancet I: 990–991Google Scholar
  850. 847.
    Warner JO, Taylor BW, Norman AP, Soothill JF (1976b) Association of cystic fibrosis with allergy. Arch Dis Child 51: 507–511PubMedGoogle Scholar
  851. 848.
    Warren CPW, Tai E, Batten JC, Hutchcroft BJ, Pepys J (1975) Cystic fibrosis — immunological reactions to aspergillus fumigatus and common allergeus. Clin Allergy 5: 1–12PubMedGoogle Scholar
  852. 849.
    Warwick WJ (1966) Cystic fibrosis sweat test for newborns. JAMA 198: 59–62Google Scholar
  853. 850.
    Warwick WJ (1969) Respiratory complications of cystic fibrosis. Minn Med 52: 1567–1569PubMedGoogle Scholar
  854. 851.
    Warwick WJ, Pogue RE (1977) Cystic fibrosis. An expanding challenge for internal medicine. JAMA 238: 2159–2162Google Scholar
  855. 852.
    Warwick WJ, Bernard B, Meskin LH (1964) The involvement of the labial mucous salivary gland in patients with cystic fibrosis. Pediatrics 34: 621–628PubMedGoogle Scholar
  856. 853.
    Warwick WJ, Philippe R, L’Heureux PR, Sharp HL, Isenberg JN (1976) Gallstones and cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  857. 854.
    Watkins JB, Tercyak AM, Szcepanik P, Klein PD (1974) Bile salt kinetics in cystic fibrosis: influence of pancreatic enzyme replacement. Gastroenterology 67: 835Google Scholar
  858. 855.
    Watts R, Taylor S, Postuma R, Smalley CA (1975) Essential fatty acid metabolism in cystic fibrosis. Lancet II: 938Google Scholar
  859. 856.
    Weber B (1974) Kohlenhydratstoffwechsel bei der Muko viscid ose. Med Welt 25: 246–248PubMedGoogle Scholar
  860. 857.
    Weber AM, Roy CC, Morin CL, Lasalle R (1973) Malabsorption of bile acids in children with cystic fibrosis. N Engl J Med 289: 1001–1005PubMedGoogle Scholar
  861. 858.
    Weber AM, Roy CC, Chartrand L, Dufour OL, Lasalle R (1976) Influence of a sodium bicarbonate supplement on fecal fat and bile acid in cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  862. 859.
    Webster R, Wilhams H (1953) Hepatic cirrhosis associated with fibrocystic disease of the pancreas: clinical and pathological reports of 5 patients. Arch Dis Child 28: 343–360PubMedGoogle Scholar
  863. 860.
    Webster L, Lochiin H (1977) Cystic fibrosis screening by sweat analysis. Med J Aust 641: 923–927Google Scholar
  864. 861.
    Weeke B, Flensborg EW, Jacobsen L, JØrgensen BA, Lykkegaard BE, HØiby N (1976) Immunochemical quantitation of 18 proteins from patients with cystic fibrosis. Dan Med Bull 23: 155–160PubMedGoogle Scholar
  865. 862.
    Weise J, Kobayashi Y, Tolckmitt W (1976) Untersuchungen über die Anwendbarkeit tryptischer und chymotryptischer Aktivitätsmessung im Stuhl in Diagnostik und Therapie der Muco viscid ose. Eur J Pediatr 122: 107–115PubMedGoogle Scholar
  866. 863.
    Weiß S (1966) Die Absorption von Eisensalzen bei Mukoviscidose. Arch Kinderheilkd 174: 254–262PubMedGoogle Scholar
  867. 864.
    Welch DW, Roberts RM (1975) Complex saccharide metabolism in cystic fibrosis fibroblasts. Pediatr Res 9: 698–702PubMedGoogle Scholar
  868. 865.
    Wentworth P, Gough J, Wentworth JE (1968) Pulmonary changes and cor pulmonale in mucoviscidosis. Thorax 23: 582–589PubMedGoogle Scholar
  869. 866.
    White H (1956) Meconium ileus new roentgen sign. Radiology 66: 567–571PubMedGoogle Scholar
  870. 867.
    Whitman V, Stern RC, Bellet P, Doershuk CF, Liebman J, Boat TF, Borkat G, Matthews LW (1975) Studies on cor pulmonale in cystic fibrosis. I. Effects of diuresis. Pediatrics 55: 83–85PubMedGoogle Scholar
  871. 868.
    Wiesman UN, Neufeld EF (1970) Metabolism of sulfated mucopolysaccharides in cultured fibroblasts from cystic fibrosis patients. J Pediatr 77: 685–690Google Scholar
  872. 869.
    Wiesman UN, Boat TF, Sant’Agnese PA di (1972) Flow-rates and electrolytes in minor-salivary-gland saliva in normal subjects and patients with cystic fibrosis. Lancet II: 510–512Google Scholar
  873. 870.
    Wilmshurst EG, Soeldner JS, Holsclaw DS, Kaufman R, Shwachman H, Aoki TT, Gelason RE (1975) Endogenous and exogenous insulin responses in cystic fibrosis. Pediatrics 55: 75–82PubMedGoogle Scholar
  874. 871.
    Wilroy RS, Crawford SW, Johnson WW (1966) Cystic fibrosis with extensive fatty replacement of the liver. J Pediatr 68: 67–73PubMedGoogle Scholar
  875. 872.
    Wilson GB, Fudenberg HH (1975) Studies on cystic fibrosis using isoelectric focusing. I. An essay for the detection of cystic fibrosis homozygote and heterozygote carriers from serum. Pediatr Res 9: 635–640PubMedGoogle Scholar
  876. 873.
    Wilson GB, Monsher MT, Fudenberg HH (1977) Studies on cystic fibrosis using isoelectric focusing. III. Correlation between cystic fibrosis protein and ciliary dyskinesia activity in serum shown by a modified rabbit tracheal bioassay. Pediatr Res 11: 143–146PubMedGoogle Scholar
  877. 874.
    Wissler H, Zollinger HU (1945) Die familiäre kongenitale cystische Pankreasfibrose mit Bronchiectasien. Helv Paediatr Acta (Suppl) I: 3–88Google Scholar
  878. 875.
    Wolfsdorf J, Swift DL, Avery ME (1969) Mist therapy reconsidered; an evaluation of the respiratory deposition of labelled water aerosols produced by jet and ultrasonic nebulizers. Pediatrics 43: 799–808PubMedGoogle Scholar
  879. 876.
    Wolfson JJ, Engel R (1969) Anticipating meconium peritonitis from metaphyseal bands. Radiology 92: 1055–1060PubMedGoogle Scholar
  880. 877.
    Wong JW, Keens TG, Wannamaker EM, Crozier DN, Levison H, Aspin N (1977) Effects of gravity in tracheal mucus transport rates in normal subjects and in patients with cystic fibrosis. Pediatrics 60: 146–152PubMedGoogle Scholar
  881. 878.
    Wood RE, Wanner A, Hirsch J, Farrell P (1975) Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. Am Rev Respir Dis 111: 733–737PubMedGoogle Scholar
  882. 879.
    Wood RE Boat TF, Doershuk CF (1976) Cystic fibrosis. Am Rev Respir Dis 113: 833–878PubMedGoogle Scholar
  883. 880.
    Wood RE, Inkley S, Ference M, Roth E, Horowitz JG, Primiano F, Doershuk CF (1977) Evaluation of the effect of postural drainage on regional ventilation in patients with cystic fibrosis. In: Baran D, Bogaert E van (eds) Chest physical therapy in cystic fibrosis and chronic obstructive pulmonary diseases. European Press, Ghent, pp 202–209Google Scholar
  884. 881.
    Wright SW, Morton NE (1968) The incidence of cystic fibrosis in Hawaii. Am J Hum Genet 20: 157–169PubMedGoogle Scholar
  885. 882.
    Yassa JG, Prosser R, Dodge JA (1976a) A controlled trial of an elemental diet for patients with cystic fibrosis. Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  886. 883.
    Yassa JG, Reynolds D, Castillo C, Prosser R, Dodge JA (1976b) Do essential fatty acids play an important role in cystic fibrosis? Proc. 7th Int. Cyst. Fibrosis Congr., Paris 1976Google Scholar
  887. 884.
    Yeates DB, Sturgess JM, Khan SR, Levison H, Aspin N (1976) Mucociliary transport in trachea of patients with cystic fibrosis. Arch Dis Child 51: 28–33PubMedGoogle Scholar
  888. 885.
    Yohe RM (1972) Allergy in patients with cystic fibrosis. Ann Allergy 30: 627– 631Google Scholar
  889. 886.
    Zapletal A, Motoyama EK, Gibson LE, Bouhuys A (1971) Pulmonary mechanics in asthma and cystic fibrosis. Pediatrics 48: 64–72PubMedGoogle Scholar
  890. 887.
    Zapletal A, Paul T, Samanek M (1977) Die Bedeutung heutiger Methoden der Lungenfunktionsdiagnostik zur Feststellung einer Obstruktion der Atemwege bei Kindern und Jugendlichen. Z Erk Atmungsorgane 149: 343–371Google Scholar
  891. 888.
    Zimmer J (1948) Microcolon. Acta Radiol (Stockh) 29: 228–236Google Scholar
  892. 889.
    Zöllner H (1977) Standardized technique of quantitative sweat test to diagnose cystic fibrosis. Seventh Annual Meeting EWGCF, Dresden 1977Google Scholar
  893. 890.
    Zoppi G, Shmerling DH, Gaburro D, Prader A (1970) The electrolyte and protein contents and outputs in duodenal juice after pancreozymin and secretin stimulation in normal children and children with cystic fibrosis. Acta Paediatr Scand 59: 692–696PubMedGoogle Scholar
  894. 891.
    Zuelzer WW, Newton WA Jr (1949) The pathogenesis of fibrocystic disease of the pancreas. Pediatrics 4: 53–69PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin · Heidelberg 1980

Authors and Affiliations

  • U. Stephan
    • 1
  • M. Götz
    • 2
  • K. Stephan
    • 3
  • S. Bender
    • 4
  1. 1.Univ.-KinderklinikEssenGermany
  2. 2.Univ.-KinderklinikWienAustria
  3. 3.Univ.-KinderklinikEssenGermany
  4. 4.Zentrum der KinderheilkundeFrankfurtGermany

Personalised recommendations