Advertisement

Inborn Errors of Oxygen-Dependent Microbial Killing by Neutrophils

  • R. Seger
Conference paper
Part of the Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics book series (KINDERHEILK. NF, volume 51)

Abstract

The neutrophil granulocyte is a wandering blood and tissue cell which protects the host by ingestion and subsequent killing of invading microorganisms. It is extremely well equipped for this function. Its antimicrobial activity consists of three stages: chemotaxis, phagocytosis, and microbial destruction. The neutrophil first responds to the concentration gradient of chemical mediators activated in the vicinity of invading microorganisms and migrates toward their source (chemotaxis). Microorganisms coated with serum proteins are then identified as targets. These proteins, also called opsonins, consist of immunoglobulins and complement factors that are recognized by specific receptors on the phagocyte membrane. The neutrophil flows around the opsonized microorganisms. Invagination of the plasma membrane occurs with the incorporation of the organisms into intracellular vacuoles (phagocytosis, Fig. I a, b). Then neutrophil granules discharge their hydrolytic enzymes into the phagocytic vacuoles (degranulation) and microbicidal hydrogen peroxide is produced in an oxygen-consuming metabolic pathway. The end result is the death of the ingested organism, which is finally degraded.

Key words

Neutrophil Granulocytes Respiratory Burst Microbial Killing Auto-oxidation Chronic Granulomatous Disease Myeloperoxidase Deficiency Glutathione Deficiency Acatalasemia 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Abe E, Belohradsky BH, Fink U, Wüst I (1978) Das vorgetäuschte Bild einer exogenen allergischen Alveolitis ( Farmerlunge) bei chronischer septischer Granulomatose. Fortschr Med 96: 1910Google Scholar
  2. 2.
    Adinoff AD, Johnston RB, Dolen J, South MA (1982) Chronic granulomatous disease and Pneumocystis carinii pneumonia. Pediatrics 69: 133 (letter)Google Scholar
  3. 3.
    Agner K (1941) Verdoperoxidase. A ferment isolated from leukocytes. Acta Physiol Scand 2 [Suppl 8]: 1Google Scholar
  4. 4.
    Alexander JW, Good RA (1968) Effect of antibiotics on the bactericidal activity of human leukocytes. J Lab Clin Med 71: 971PubMedGoogle Scholar
  5. 5.
    Allen RC, Mills EL, McNitt TR, Quie PG (1981) Role of myeloperoxidase and bacterial metabolism in chemiluminescence of granulocytes from patients with chronic granulomatous disease. J Infect Dis 144: 344PubMedGoogle Scholar
  6. 6.
    Allen RC, Stjernholm RL, Reed MA, Harper TB, Gupta S, Steele RH, Waring WW (1977) Correlation of metabolic and chemiluminescent responses of granulocytes from three female siblings with chronic granulomatous disease. J Infect Dis 136: 510PubMedGoogle Scholar
  7. 7.
    Ament ME, Ochs HD (1973) Gastrointestinal manifestations of chronic granulomatous disease. N Engl J Med 288: 382PubMedGoogle Scholar
  8. 8.
    Anderson R (1981) Assessment of oral ascorbate in three children with chronic granulomatous disease and defective neutrophil motility over a 2-year period. Clin Exp Immunol 43: 180PubMedGoogle Scholar
  9. 9.
    Anderson R, Grabow G, Oosthuizen R, Theron A, Van Rensburg AJ (1980) Effects of Sulfamethoxazole and trimethoprim on human neutrophil and lympho- cyte functions in vitro: in vivo effects of co-trimoxazole. Antimicrob Agents Chemother 17: 322PubMedGoogle Scholar
  10. 10.
    Arnaout MA, Pitt J, Cohen HJ, Melamed J, Rosen FS, Colten HR (1982) Defi- ciency of a granulocyte-membrane glycoprotein (gp 150) in a boy with recurrent bacterial infections. N Engl J Med 306: 693PubMedGoogle Scholar
  11. 11.
    Arroyo J, Medoff G, Kobayashi GS (1977) Therapy of murine aspergillosis with amphotericin B in combination with rifampin or 5-fluorocytosine. Antimicrob Agents Chemother 11: 21PubMedGoogle Scholar
  12. 12.
    Azimi PH, Bodenbender JG, Hintz RL, Kontras SB (1968) Chronic granulomatous disease in three female siblings. JAMA 206: 2865PubMedGoogle Scholar
  13. 13.
    Babior BM, Kipnes RS (1977) Superoxide-forming enzyme from human neutrophils: evidence for a flavin requirement. Blood 50: 517PubMedGoogle Scholar
  14. 14.
    Babior BM, Curnutte JT, McMurrich BJ (1976) The particulate superoxide-form- ing system from human neutrophils. Properties of the system and further evidence supporting its participation in the respiratory burst. J Clin Invest 58: 989Google Scholar
  15. 15.
    Babior BM, Kipnes RS, Curnutte JT (1973) Biological defense mechanisms. The production by leukocytes of Superoxide, a potential bactericidal agent. J Clin Invest 52: 741Google Scholar
  16. 16.
    Baehner RL (1975) Subcellular distribution of nitroblue tetrazolium reductase (NBT-R) in human polymorphonuclear leukocytes (PMN). J Lab Clin Med 86: 785PubMedGoogle Scholar
  17. 17.
    Baehner RL, Nathan DG (1967) Leukocyte oxidase: Defective activity in chronic granulomatous disease. Science 155: 835Google Scholar
  18. 18.
    Baehner RL, Nathan DG (1968) Quantitative nitroblue tetrazolium test in chronic granulomatous disease. N Engl J Med 278: 971PubMedGoogle Scholar
  19. 19.
    Baehner RL, Johnston RB, Nathan DG (1972) Comparative study of the metabolic and bactericidal characteristics of severely glucose-6-phosphate dehydrogenase- deficient polymorphonuclear leukocytes and leukocytes from children with chronic granulomatous disease. J Reticuloendothel Soc 12: 150PubMedGoogle Scholar
  20. 20.
    Baehner RL, Karnovsky MJ, Karnovsky ML (1969) Degranulation of leukocytes in chronic granulomatous disease. J Clin Invest 48: 187PubMedGoogle Scholar
  21. 21.
    Baehner RL, Nathan DG, Karnovsky ML (1970) Correction of metabolic deficien- cies in the leukocytes of patients with chronic granulomatous disease. J Clin Invest 49: 865PubMedGoogle Scholar
  22. 22.
    Baldridge CW, Gerrard RW (1933) The extra-respiration of phagocytosis. Am J Physiol 103: 235Google Scholar
  23. 23.
    Balfour HH, Shehan JJ, Speicher CE, Kauder E (1971) Chronic granulomatous disease of childhood in a 23-year-old man. JAMA 217: 960PubMedGoogle Scholar
  24. 24.
    Bannatyne RM, Skowron PN, Weber JL (1969) Job’s Syndrome - a variant of chronic granulomatous disease. J Pediatr 75: 236PubMedGoogle Scholar
  25. 25.
    Bartman J, Van de Velde RL, Friedman F (1967) Pigmented lipid histiocytosis and susceptibility to infection: Ultrastructure of splenic histiocytes. Pediatrics 40: 1000Google Scholar
  26. 26.
    Bass LJ, Voorhees JJ, Dubin HV, Tanner MH (1972) Chronic granulomatous disease of childhood. Superficial pyoderma as a major dermatologic manifestation. Arch Dermatol 106: 68Google Scholar
  27. 27.
    Beam TR (1979) Sequestration of staphylococci at an inaccessible focus. Lancet 11: 227Google Scholar
  28. 28.
    Beiding ME, Klebanoff SJ, Ray CG (1970) Peroxidase-mediated virucidal systems. Science 167: 195Google Scholar
  29. 29.
    Berendes H, Bridges RA, Good RA (1957) A fatal granulomatosis of childhood. The clinical study of a new Syndrome. Minn Med 40: 309Google Scholar
  30. 30.
    Berman JD, Wyler DJ (1980) An in vitro model for investigation of chemothera- peutic agents in leishmaniasis. J Infect Dis 142: 83PubMedGoogle Scholar
  31. 31.
    Beutler E (1971) Abnormalities of the hexose monophosphate shunt. Semin Hematol 8: 311PubMedGoogle Scholar
  32. 32.
    Beyer P, Talon P, Undreiner F, Simeoni (1982) Becegite et granulomatose sep- tique. A propos d’une nouvelle Observation. Pediatrie 37: 53Google Scholar
  33. 33.
    Biggar WD, Büro S, Holmes B (1976) Chronic granulomatous disease in an adult male: A proposed X-linked defect. J Pediatr 88: 63Google Scholar
  34. 34.
    Bigley RH, Stankova L (1974) Uptake and reduction of oxidized and reduced ascorbate by human leukocytes. J Exp Med 139: 1084PubMedGoogle Scholar
  35. 35.
    Björksten B, Lundmark KM (1972) Abnormal nitroblue tetrazolium test in relatives of a female with chronic granulomatous disease. Scand J Infect Dis 4: 167PubMedGoogle Scholar
  36. 36.
    Bloomberg SD, Neu HC, Ehrlich RM, Blanc WA (1974) Chronic granulomatous disease of childhood with renal involvement. Urology 4: 193PubMedGoogle Scholar
  37. 37.
    Boivin P, Galand C (1965) La synthese du glutathion au cours de Fanemie hemo- lytique congenitale avec deficit en glutathion reduit. Deficit congenital en gluta- thion-synthetase erythrocytaire. Nouv Rev Fr Hematol 5: 707Google Scholar
  38. 38.
    Borregaard N, Johansen KS, Taudorff E, Wandall JH (1979) Cytochrome b is present in neutrophils from patients with chronic granulomatous disease. Lancet 1: 949PubMedGoogle Scholar
  39. 39.
    Borregaard N, Bang J, Berthelsen JG, Johansen KS, Koch C, Philip J, Rasmussen K, Schwartz M, Therkelsen AJ, Valerius NH (1982) Prenatal diagnosis of chronic granulomatous disease. Lancet 1: 114 (letter)Google Scholar
  40. 40.
    Bowell PJ, Hill FGH (1978) Autoanalyser determination of red cell Kell pheno- types with chronic granulomatous disease ( CGD) and heterozygous carriers. Br J Haematol 39: 351Google Scholar
  41. 41.
    Bowen A, Gibson MD (1980) Chronic granulomatous disease with gastric antral narrowing. Pediatr Radiol 10: 119PubMedGoogle Scholar
  42. 42.
    Boxer LA, Oliver JM, Spielberg SP, Allen JM, Schulman JD (1979) Protection of granulocytes by vitamin E in glutathione synthetase deficiency. N Engl J Med 301: 901Google Scholar
  43. 43.
    Brandrup F, Koch C, Petri M, Schiodt M, Johansen KS (1981) Discoid lupus ery- thematosus-like lesions and Stomatitis in female carriers of X-linked chronic granulomatous disease. Br J Dermatol 104: 495PubMedGoogle Scholar
  44. 44.
    Breton-Gorius J, Coquin Y, Guichard J (1975) Aetivites peroxydasiques de cer- taines granulations des neutrophiles dans deux cas de deficit congenital en myelo- peroxydase. CR Acad Sc (Paris) 280: 1753Google Scholar
  45. 45.
    Bridges RA, Berendes H, Good RA (1959) A fatal granulomatous disease of child- hood. The clinical, pathological and laboratory features of a new Syndrome. J Dis Child 97: 387Google Scholar
  46. 46.
    Briggs RT, Drath DB, Karnovsky ML, Karnovsky MJ (1975) Localisation of NADH oxidase on the surface of human polymorphonuclear leukocytes by a new cytochemical method. J Cell Biol 67: 566PubMedGoogle Scholar
  47. 47.
    Brown KN, Percival A (1978) Penetration of antimicrobials into tissue culture cells and leukocytes. Scand J Infect Dis [Suppl] 14: 251Google Scholar
  48. 48.
    Brzica SM, Rhodes KH, Pineda AA, Taswell HF (1977) Chronic granulomatous disease and the McLeod phenotype. Successful treatment of infection with granulocyte transfusions resulting in subsequent hemolytic transfusion reaction. Mayo Clin Proc 52: 153Google Scholar
  49. 49.
    Bujak JS, Kwon-Chung KJ, Chusid MJ (1974) Osteomyelitis and pneumonia in a boy with chronic granulomatous disease of childhood caused by a mutant strain of Aspergillus nidulans. Am J Clin Pathol 61: 361PubMedGoogle Scholar
  50. 50.
    Bujak JS, Ottesen EA, Dinarello CA, Brenner VJ (1973) Nocardiosis in a child with chronic granulomatous disease. J Pediatr 83: 98PubMedGoogle Scholar
  51. 51.
    Burns DA, Sarkany I (1979) Chronic granulomatous disease in an adult. J R Soc Med 72: 139PubMedGoogle Scholar
  52. 52.
    Bushby SRM, Hitehings GH (1968) Trimethoprim, a sulphonamide potentiator. Br J Pharmacol Chemother 33: 72PubMedGoogle Scholar
  53. 53.
    Butler T, Rumans L, Arnold K (1982) Response of typhoid fever caused by chlor- amphenicol-susceptible and chloramphenicol-resistant strains of Salmonella typhi to treatment with trimethopriin-sulfamethoxazole. Rev Infect Dis 4: 551PubMedGoogle Scholar
  54. 54.
    Caldicott WJ, Baehner RL (1968) Chronic granulomatous disease of childhood. Am J Roentgenol 103: 133Google Scholar
  55. 55.
    Castranova V, Jones GS, Phillips RM, Peden D, Vandyke K (1981) Abnormal responses of granulocytes in chronic granulomatous disease. Biochim Biophys Acta 645: 49PubMedGoogle Scholar
  56. 56.
    Cech P, Stalder H, Widman JJ, Rohner A, Miescher P (1979) Leukocyte myeloperoxidase deficiency and diabetes mellitus associated with Candida albicans liver abscess. Am J Med 66: 149PubMedGoogle Scholar
  57. 57.
    Chan TK, Todd D, Tso SC (1976) Drug-induced haemolysis in glucose-6-phosphate dehydrogenase deficiency. Br Med J 11: 1227Google Scholar
  58. 58.
    Chisholm GD, Waterworth PM, Calnau JS, Garrod LP (1973) Concentration of antibacterial agents in interstitial tissue fluid. Br Med J 1: 569PubMedGoogle Scholar
  59. 59.
    Chusid MJ, Tomasulo PA (1978) Survival of transfused normal granulocytes in a patient with chronic granulomatous disease. Pediatrics 61: 556PubMedGoogle Scholar
  60. 60.
    Chusid MJ, Nutter C, Rosenwasser LJ (1976) Nocardia infections. Pediatrics 58: 294 (letter)Google Scholar
  61. 61.
    Chusid MJ, Parillo JE, Fauci AS (1975) Chronic granulomatous disease: Diagnosis in a 27-year-old man with Mycobacterium fortuitum. JAMA 233: 1295PubMedGoogle Scholar
  62. 62.
    Chusid MJ, Shea ML, Sarff LD (1980) Determination of posttransfusion granulocyte kinetics by chemiluminescence in chronic granulomatous disease. J Lab Clin Med 95: 168PubMedGoogle Scholar
  63. 63.
    Chusid MJ, Gelfand JA, Nutter C, Fauci AS (1975) Pulmonary aspergillosis, Inhalation of contaminated marijuana smoke, chronic granulomatous disease. Ann Intern Med 82: 682Google Scholar
  64. 64.
    Clark RA, Klebanoff SJ (1978) Chronic granulomatous disease. Studies of a family with impaired neutrophil chemotactic, metabolic and bactericidal function. Am J Med 65: 941Google Scholar
  65. 65.
    Clawson CC, Rodey GE, Good RA (1970) Ultrastructure of familial lipochrome histiocytosis. Lab Invest 22: 294PubMedGoogle Scholar
  66. 66.
    Clini V, Grassi C (1970) The action of new antituberculous drugs on intracellular tubercle bacilli. Antibiot Chemother 16: 20PubMedGoogle Scholar
  67. 67.
    Cohen JH, Newburger PE, Chovaniec ME, Whitin JC, Simons ER (1981) Opson- ized zymosan-stimulated granulocytes — activation and activity of the superoxide- generating system and membrane potential changes. Blood 58: 975PubMedGoogle Scholar
  68. 68.
    Cohen MS, Isturiz RE, Malech HL, Root RK, Wilfert CM, Gutman L, Buckley RH (1981) Fungal infection in chronic granulomatous disease. The importance of the phagocyte in defense against fungi. Am J Med 71: 59Google Scholar
  69. 69.
    Cole P, Brostoff J (1975) Intracellular killing of Listeria monocytogenes by acti- vated macrophages ( Mackaness system) is due to antibiotics. Nature(Lond) 256: 515Google Scholar
  70. 70.
    Cooper MR, De Chatelet LR, McCall CE,La Via MF,Spurr CL, Baehner RL (1972) Complete deficiency of leukocyte glucose-6-phosphate dehydrogenase with de- fective bactericidal activity. J Clin Invest 51: 769Google Scholar
  71. 71.
    Corrons JLV, Feliu E, Pujades MA, Cardellach F, Rozman C, Carreras A, Jou JM, Vallespi MT, Zuazu FJ (1982) Severe glucose-6-phosphate dehydrogenase (G6PD) deficiency associated with chronic hemolytic anemia, granulocyte dysfunction, and increased susceptibility to infections: Description of a new molecular variant ( G6PD Barcelona ). Blood 59: 428Google Scholar
  72. 72.
    Cross AR, Jones OTG, Harper AM, Segal AW (1981) Oxidation-reduction proper- ties of the cytochrome b found in the plasma-membrane fraction of human neutrophils. A possible oxidase in the respiratory burst. Biochem J 194: 599Google Scholar
  73. 73.
    Curnutte JT, Kipnes RS, Babior BM (1975) Defect in Pyridine nucleotide-depen- dent Superoxide production by a particulate fraction from the granulocytes of patients with chronic granulomatous disease. N Engl J Med 293: 628PubMedGoogle Scholar
  74. 74.
    Curnutte JT, Whitten DM, Babior BM (1974) Defective Superoxide production by granulocytes from patients with chronic granulomatous disease. N Engl J Med 290: 593PubMedGoogle Scholar
  75. 75.
    Cyr WL, Johnson H, Balfour JC (1973) Granulomatous cystitis as a manifestation of chronic granulomatous disease of childhood. J Urol 110: 357PubMedGoogle Scholar
  76. 76.
    Davis WC, Huber H, Douglas SD, Fudenberg HH (1968) A defect in circulating mononuclear phagocytes in chronic granulomatous disease of childhood. J Immunol 101: 1093PubMedGoogle Scholar
  77. 77.
    De Chatelet LR Shirley PS (1981) Evaluation of chronic granulomatous disease by a chemiluminescence assay of microliter quantities of whole blood. Clin Chem 27: 1739Google Scholar
  78. 78.
    De Chatelet LR, Shirley PS, McPhail LC (1976) Normal leukocyte glutathione peroxidase activity in patients with chronic granulomatous disease. J Pediatr 89: 598Google Scholar
  79. 79.
    De Chatelet LR, McPhail LC, Mullikin D, McCall CE (1975) An isotopic assay for NADPH oxidase activity and some characteristics of the enzyme from human polymorphonuclear leukocytes. J Clin Invest 55: 714Google Scholar
  80. 80.
    Dechelette E, Rossignol AM, Gout JP, Poirot P, Frappat P, Bost M (1977) Amylose renale et granulomatose septique chronique. Pediatrie 32: 573Google Scholar
  81. 81.
    Densen P, Wilkinson-Kroovands S, Mandell GL, Sullivan G, Øyen R, Marsh WL (1981) Kx: Its relationship to chronic granulomatous disease and genetic linkage with Xg. Blood 58: 34Google Scholar
  82. 82.
    Dewald B, Baggiolini M, Curnutte JT, Babior BM (1979) Subcellular localization of the Superoxide-forming enzyme in human neutrophils. J Clin Invest 63: 21PubMedGoogle Scholar
  83. 83.
    Diamond RD, Root RK, Bennett JE (1972) Factors influencing killing of Crypto- coccus neoformans by human leukocytes in vitro. J Infect Dis 125: 367PubMedGoogle Scholar
  84. 84.
    Dilworth JA, Mandell GL (1977) Adults with chronic granulomatous disease of “childhood”. Am J Med 63: 233PubMedGoogle Scholar
  85. 85.
    Douglas SD, Spicer SS (1971) Acid phosphatase cytochemistry of phagocytosing leukocytes from patients with chronic granulomatous disease. Infect Immun 3: 179PubMedGoogle Scholar
  86. 86.
    Douglas SD, Davis WC, Fudenberg HH (1969) Granulocytopathies: Pleomorphism of neutrophil dysfunction. Am J Med 46: 901PubMedGoogle Scholar
  87. 87.
    Easmon CSF (1979) The effect of antibiotics on the intracellular survival of Sta- phylococcus aureus in vitro. Br J Exp Pathol 60: 24PubMedGoogle Scholar
  88. 88.
    Elgefors B, Olling S, Peterson H (1978) Chronic granulomatous disease in three siblings. Scand J Infect Dis 10: 79PubMedGoogle Scholar
  89. 89.
    Esterly JR, Sturner WQ, Esterly NB, Windhorst DB (1971) Disseminated BCG in twin boys with presumed chronic granulomatous disease of childhood. Pediatrics 48: 141PubMedGoogle Scholar
  90. 90.
    Ezer G, Soothill JF (1974) Intracellular bactericidal effects of rifampicin in both normal and chronic granulomatous disease polymorphs. Arch Dis Child 49: 463PubMedGoogle Scholar
  91. 91.
    Feiberg NT, Schultz J (1972) Evidence that myeloperoxidase is composed of isozymes. Arch Biochem Biophys 148: 407Google Scholar
  92. 92.
    Fikrig SM, Smithwick EM, Suntharalingam K, Good RA (1980) Fibroblast nitro- blue tetrazolium test and the in-utero diagnosis of chronic granulomatous disease. Lancet 1: 18PubMedGoogle Scholar
  93. 93.
    Fikrig SM, Phillipp JCD, Smithwick EM, Øyenyen R, Marsh WLC (1980) Chronic granulomatous disease and McLeod Syndrome in a black child. Pediatrics 66: 403PubMedGoogle Scholar
  94. 94.
    Fleming GM, Kleinerman J, Doershuk CF, Perrin EV (1975) Chronic granulomatous disease of childhood. An unusual cause of honeycomb lung. Chest 68: 834Google Scholar
  95. 95.
    Forbes GS, Hartmann GW, Burke EC, Segura JW (1976) Genitourinary involve- ment in chronic granulomatous disease of childhood. Am J Roentgenol 127: 683Google Scholar
  96. 96.
    Ford DK, Price GE, Culling CFA, Vassar P (1962) Familial lipochrome pigmenta- tion of histiocytes with hyperglobulinemia, pulmonary Infiltration, splenomegaly, arthritis and susceptibility to infection. Am J Med 33: 478PubMedGoogle Scholar
  97. 97.
    Forsgren A, Schmeling D (1977) Effect of antibiotics on Chemotaxis of human leukocytes. Antimicrob Agents Chemother 11: 580PubMedGoogle Scholar
  98. 98.
    Frater-Schröder M, Seger R, Hitzig WH, Linnell JC, Huser HJ, Galle J, Wüdfeuer A (1981) Abnormal biochemistry and morphology of erythrocytes in a case of transcobalamin II deficiency during treatment. J Inher Metab Dis 4: 143Google Scholar
  99. 99.
    Frayha H, Gold R, Prober CG, Biggar WD (1982) Trimethoprim-sulphamethoxa- zole (TMP-SMX) Prophylaxis in nine patients with chronic granulomatous disease ( CGD ). Pediatr Res 16: 240AGoogle Scholar
  100. 100.
    Frazer JP (1973) Chronic granulomatous disease involving the larynx and bron- chus. Ann Otol Rhinol Laryngol 82: 816PubMedGoogle Scholar
  101. 101.
    Gabig TG, Kipnes RS, Babior BM (1978) Solubilization of the 02~ forming activity responsible for the respiratory burst in human neutrophils. J Biol Chem 253: 6663PubMedGoogle Scholar
  102. 102.
    Gabig TG, Schervish EW, Sautinga JT (1982) Functional relationship of the cyto- chrome b to the superoxide-generating oxidase of human neutrophils. J Biol Chem 257: 4114PubMedGoogle Scholar
  103. 103.
    Giblett ER, Klebanoff SJ, Pincus SH, Swanson J, Park BH, McCullough J (1971) Kell phenotypes in chronic granulomatous disease: A Potential transfusion hazard. Lancet 1: 1235Google Scholar
  104. 104.
    Gmünder FK, Seger RA (1981) Chronic granulomatous disease: Mode of action of sulfamethoxazole/trimethoprim. Pediatr Res 15: 1533Google Scholar
  105. 105.
    Gold RH, Douglas SD, Preger L, Steinbach HL, Fudenberg HH (1969) Roent- genographic features of the neutrophil dysfunction syndromes. Radiology 92: 1045PubMedGoogle Scholar
  106. 106.
    Gold SB, Hanes DM, Stites DP, Fudenberg HH (1974) Abnormal kinetics of de- granulation in chronic granulomatous disease. N Engl J Med 291: 332PubMedGoogle Scholar
  107. 107.
    Good RA, Quie PG, Windhorst DB, Page AR, Rodey GE, White J, Wolfson JJ, Holmes BH (1968) Fatal (chronic) granulomatous disease of childhood: A heredi- tary defect of leukocyte function. Semin Hematol 5: 215Google Scholar
  108. 108.
    Goudemand J, Anssens R, Delmas-Marsalet Y, Farriaux JP, Fontaine G (1976) Essai de traitement d’un cas de granulomatose familiale chronique par greffe de moelle osseuse allogenique. Arch Fr Pediatr 33: 121PubMedGoogle Scholar
  109. 109.
    Gray GD, Knight KA, Talley CA (1980) Rifampin (R) has paradoxical effects on leukotaxis ( LTX ). Fed Proc 39: 878AGoogle Scholar
  110. 110.
    Gray GR, Klebanoff SJ, Stamatoyannopoulos G, Austin T, Naimann SC, Yoshida A, Kliman MR, Robinson GCF (1973) Neutrophil dysfunction, chronic granulomatous disease and non-spherocytic haemolytic anemia caused by complete deficiency of glucose-6-phosphate dehydrogenase. Lancet 11: 530Google Scholar
  111. 111.
    Grignaschi VI, Sperperato AM, Etcheverry MJ, Macario AJL (1963) Un nuevo cuadro citoquimico: Negatividad espontanea de las reacciones de peroxidasas, oxidasas y lipido en la progenie neutrofila y en los moncitos de dos hermanos. Rev Asoc Med Argent 77: 218Google Scholar
  112. 112.
    Griscom NT, Kirkpatrick JA, Girdany BR, Berdon WE, Grand RJ, Macki GG (1974) Gastric antral narrowing in chronic granulomatous disease of childhood. Pediatrics 54: 456PubMedGoogle Scholar
  113. 113.
    Grüneberg RN (1980) Antibiotic sensitivities of urinary pathogens, 1971-8. J Clin Pathol 33: 853PubMedGoogle Scholar
  114. 114.
    Haddad HL, Beatty DW, Dowdle EB (1976) Chronic granulomatous disease of childhood. S Afr Med J 50: 2068PubMedGoogle Scholar
  115. 115.
    Halayun JF, Anast CS, Lukens JN(1972)Thyrotoxicosis associated with asper- gillus thyroiditis in chronic granulomatous disease. J Pediatr 80: 106Google Scholar
  116. 116.
    Hamers MN, Wever R, Van Schalk MLJ, Weening RS, Roos D (1981) Cytochrome b and the superoxide-generating system in human neutrophils. In: Bannister WH, Bannister JV (eds) Biological and clinical aspects of Superoxide and Superoxide dismutase. Elsevier/North-Holland, Amsterdam, p 242Google Scholar
  117. 117.
    Hansen I, Nielsen ML, Heerfordt L, Henriksen B, Bertelsen S (1973) Trimethoprim in normal and pathological human lung tissue. Chemotherapy 19: 221PubMedGoogle Scholar
  118. 118.
    Harada T, Sugihara H, Tsuchiyama H, Nöda H (1977) Histochemical and ultra- structural Observation of the pigment in pigmented lipid histiocytes of chronic granulomatous disease. Acta Histochem Cytochem 10: 500Google Scholar
  119. 119.
    Harris BH, Boles ET (1973) Intestinal lesions in chronic granulomatous disease of childhood. J Pediatr Surg 8: 955PubMedGoogle Scholar
  120. 120.
    Harvath L, Andersen BR (1979) Defective initiation of oxidative metabolism in polymorphonuclear leukocytes. N Engl J Med 300: 1130PubMedGoogle Scholar
  121. 121.
    Hassan M, Douillet P, Mage K (1976) Une cause rare de stenose antro-pylorique de Fenfant: la granulomatose septique. J Radiol Electrol 57: 93PubMedGoogle Scholar
  122. 122.
    Hattori H (1961) Studies on the labile, stable NADI oxidase and peroxidase staining reactions in the isolated particles of horse granulocytes. Nagoya J Med Sei 23: 362Google Scholar
  123. 123.
    Herbert V (1973) Metabolism of folic acid in man. J Infect Dis 128: S601Google Scholar
  124. 124.
    Heyne K, Gahr M (1980) Differentiation between glycogenosis type la and Ib by measurement of extra respiration during phagocytosis by polymorphonuclear leukocytes? Eur J Pediatr 133: 69PubMedGoogle Scholar
  125. 125.
    Heyne K, Lorenz M (1971) Zur Lungenerkrankung bei progressiver Granulomatose des Kindes. Dtsch Gesundheitswesen 26: 1745Google Scholar
  126. 126.
    Hitzig WH, Weber C (1980) Die progressive septische Granulomatose. In: Frick et al. (eds) Ergebnisse der inneren Medizin und Kinderheilkunde, vol 44. Springer, Berlin Heidelberg New York, p 37Google Scholar
  127. 127.
    Hitzig WH, Döhmann U, Plüss HJ, Vischer D (1974) Hereditary transcobalamin II deficiency: clinical findings in a new family. J Pediatr 85: 622PubMedGoogle Scholar
  128. 128.
    Hitzig WH, Molz G, Plüss HJ, Renner R (1969) Progressive septische Granulomatose. Helv Paediatr Acta 24: 246PubMedGoogle Scholar
  129. 129.
    Hoeprich PD, Martin CH (1970) Effect of tetracycline, polymyxin B, and rifam- pin on phagocytosis. Clin Pharmacol Ther 11: 418PubMedGoogle Scholar
  130. 130.
    Hohn DC, Lehrer RI (1975) NADPH oxidase deficiency in X-linked chronic granulomatous disease. J Clin Invest 55: 707PubMedGoogle Scholar
  131. 131.
    Hoidal JR, Fox RB, Repine JE (1979) Defective oxidative metabolic responses in vitro of alveolar macrophages in chronic granulomatous disease. Am Rev Respir Dis 120: 613PubMedGoogle Scholar
  132. 132.
    Holmes B, Good RA (1972) Laboratory models of chronic granulomatous disease. J Reticuloendothel Soc 12: 216PubMedGoogle Scholar
  133. 133.
    Holmes B, Page AR, Good RA (1967) Studies of the metabolic activity of leukocytes from patients with a genetic abnormality of phagocytic function. J Clin Invest 46: 1422PubMedGoogle Scholar
  134. 134.
    Holmes B, Quie PG, Windhorst DB, Good RA (1966) Fatal granulomatous disease of childhood. An inborn abnormality of phagocytic function. Lancet 1: 1225Google Scholar
  135. 135.
    Holmes B, Park BH, Malawista SE, Quie PG, Nelson DL, Good RA (1970) Chronic granulomatous disease in females. A deficiency of leukocyte glutathione per- oxidase. N Engl J Med 283: 217Google Scholar
  136. 136.
    Hughes DTD (1979) Antibiotic treatment of chronic bronchitis. J R Coli Physicians Lond 13: 26Google Scholar
  137. 137.
    Hughes WT, Kuhn S, Chaudhary S, (1977) Successful ehemoprophylaxis for Pneumocystis carinii Pneumonitis. N Engl J Med 297: 1419PubMedGoogle Scholar
  138. 138.
    Huhn D, Belohradsky BH, Haas R (1978) Familiärer Myeloperoxidasedefekt und akute myeloische Leukämie. Acta Haematol 59: 129Google Scholar
  139. 139.
    Humbert JR, Fishman CB, Weston WL, De Armey PA, Thoren CH (1976) Fre- quency of the carrier State for X-linked chronic granulomatous disease among females with lupus erythematosus. Clin Genet 10: 16PubMedGoogle Scholar
  140. 140.
    Idriss ZH, Cunningham RJ, Wilfert CM (1975) Nocardiosis in ehildren: Report of three cases and review of the literature. Pediatrics 55: 479Google Scholar
  141. 141.
    Ismail G, Boxer LA, Baehner RL (1979) Utilization of liposomes for correction of the metabolic and bactericidal deficiencies in chronic granulomatous disease. Pediatr Res 13: 769PubMedGoogle Scholar
  142. 140.
    Iyer GYN, Quastel JH (1963) NADPH and NADH oxidation by guinea pig poly- morphonuclear leucocytes. Can J Biochem 41: 427PubMedGoogle Scholar
  143. 143.
    Iyer GYN, Islam DMF, Quastel JH (1961) Biochemical aspects of phagocytosis. Nature 192: 535Google Scholar
  144. 144.
    Jacobs AA, Low IE, Paul BB, Strauss RR, Sbarra AJ (1972) Mycoplasmacidal activity of peroxidase-H202-halide systems. Infect Immun 5: 127PubMedGoogle Scholar
  145. 145.
    Jacobs RF, Wilson CB (1981) Antibiotics effective against intracellular staphylo- cocci in chronic granulomatous disease. 2Ist Interscientific Conference on Antimicrobial Agents Chemotherapy. American Society for Microbiology, Washington DC, abstract 725Google Scholar
  146. 146.
    Jacobs RF, Wilson CB, Laxton JG, Haas JE, Smith AL (1982) Cellular uptake and intracellular activity of antibiotics against Haemophilus influenzae type b. J Infect Dis 145: 152PubMedGoogle Scholar
  147. 147.
    Jandl RC, Andre-Schwartz J, Borges-Du Bois L, Kipnes RS, McMurrich BJ, Babior BM (1978) Termination of the respiratory burst in human neutrophils. J Clin Invest 61: 1176PubMedGoogle Scholar
  148. 148.
    Jensen MS, Bainton DF (1973) Temporal changes in pH within the phagocytic vacuole of the polymorphonuclear neutrophilic leukocyte. J Cell Biol 56: 379PubMedGoogle Scholar
  149. 149.
    Johansen KS, Borregaard N, Koch C, Taudorf E, Wandall JH, Repine JE (1982) Chronic granulomatous disease presenting as xanthogranulomatous Pyelonephritis in late childhood. J Pediatr 100: 98PubMedGoogle Scholar
  150. 150.
    Johnson FE, Humbert JR, Kuzela DC, Todd JK, Lilley JR (1975) Gastric outlet obstruction due to X-linked chronic granulomatous disease. Surgery 78: 217PubMedGoogle Scholar
  151. 151.
    Johnson JD, Hand WL, Francis JB, King-Thompson N, Corwin RW(1980) Anti- biotic uptake by alveolar macrophages. J Lab Clin Med 95: 429Google Scholar
  152. 152.
    Johnston RB, Baehner RL (1970) Improvement of leukocyte bactericidal activity in chronic granulomatous disease. Blood 35: 350PubMedGoogle Scholar
  153. 153.
    Johnston RB, McMurry JS (1967) Chronic familial granulomatosis. Report of five cases and review of the literature. Am J Dis Child 114: 370Google Scholar
  154. 154.
    Johnston RB, Newman SL (1977) Chronic granulomatous disease. Pediatr Clin North Am 24: 365PubMedGoogle Scholar
  155. 155.
    Johnston RB, Wilfert CM, Buckley RH, Webb LS, De Chatelet LR, McCall CE (1975) Enhanced bactericidal activity of phagocytes from patients with chronic granulomatous disease in the presence of sulphisoxazole. Lancet 1: 824PubMedGoogle Scholar
  156. 156.
    Kakinuma K, Yamaguchi T, Kaneda M, Shimada K, Tomita Y, Chance B (1979) A determination of H202 release by the treatment of human blood polymorphonuclear leukocytes with myristate. J Biochem 86: 87PubMedGoogle Scholar
  157. 157.
    Kaplan EL, Laxdal T, Quie PG (1968) Studies of polymorphonuclear leukocytes from patients with chronic granulomatous disease of childhood: Bactericidal capacity for Streptococci. Pediatrics 41: 591Google Scholar
  158. 158.
    Kaplow LS (1975) Substitute for benzidine in myeloperoxidase strains. Am J Clin Pathol 63: 451PubMedGoogle Scholar
  159. 159.
    Keller F (1976) Krankheitsbild einer generalisierten BCG Impftuberkulose als Ausdruck einer progressiven septischen Granulomatose. Z Erkr Atmungsorgane 146: 134PubMedGoogle Scholar
  160. 160.
    Kitahara M, Simonian Y, Eyre HJ (1979) Neutrophil myeloperoxidase: a simple, reproducible technique to determine activity. J Lab Clin Med 93: 232PubMedGoogle Scholar
  161. 161.
    Kitahara M, Eyre HJ, Simonian Y, Atkin CL, Hasstedt SJ (1981) Hereditary myeloperoxidase deficiency. Blood 57: 888PubMedGoogle Scholar
  162. 162.
    Klebanoff SJ (1968) Myeloperoxidase-halide-hydrogen peroxide antibacterial system. J Bacteriol 95: 2131PubMedGoogle Scholar
  163. 163.
    Klebanoff SJ (1970) Myeloperoxidase: Contribution to the microbicidal activity of intact leukocytes. Science 169: 1095PubMedGoogle Scholar
  164. 164.
    Klebanoff SJ (1977) Estrogen binding by leukocytes during phagocytosis. J Exp Med 145: 983PubMedGoogle Scholar
  165. 165.
    Klebanoff SJ, Clark RA (1978) The neutrophil: function and clinical disorders. North Holland, AmsterdamGoogle Scholar
  166. 166.
    Klebanoff SJ, Green WL (1973) Degradation of thyroid hormonesby phagocytos- ing human leukocytes. J Clin Invest 52: 60PubMedGoogle Scholar
  167. 167.
    Klebanoff SJ, HamonCB (1972) Role of myeloperoxidase-mediated antimicrobial systems in intact leukocytes. J Reticuloendothel Soc 12: 170PubMedGoogle Scholar
  168. 168.
    Klebanoff SJ, Pincus SH (1971) Hydrogen peroxide utilization in myeloper- oxidase-deficient leukocytes: a possible microbicidal control mechanism. J Clin Invest 50: 2226PubMedGoogle Scholar
  169. 169.
    Klebanoff SJ, White LR (1969) Iodination defect in the leukocytes of a patient with chronic granulomatous disease of childhood. N Engl J Med 280: 460PubMedGoogle Scholar
  170. 170.
    Klempner MS, Styrt B (1981) Clindamycin uptake by human neutrophils J Infect Dis 144: 472Google Scholar
  171. 171.
    Knothe H (1973) The effect of a combined preparation of trimethoprim and sulphamethoxazole following short-term and long-term administration of the flora of the human gut. Chemotherapy 18: 285PubMedGoogle Scholar
  172. 172.
    Kobayashi Y, Amano D, Ueda K, Kagosaki Y, Usui T (1978) Treatment of seven cases of chronic granulomatous disease with sulfamethoxazole-trimethoprim ( SMX-TMP ). Eur J Pediatr 127: 247Google Scholar
  173. 173.
    Koch C, Søgaard H, Christensen MF (1973) Inheritance of chronic granulomatous disease in females. Acta Paediatr Scand 62: 659PubMedGoogle Scholar
  174. 174.
    Kontras SB, Bodenbender JG, McClave CR, Smith JP (1971) Interstitial cystitis in chronic granulomatous disease. J Urol 105: 575PubMedGoogle Scholar
  175. 175.
    Koven NL, Clark MM, Cody CS, Stanley CA, Baker L, Douglas SP (1981) Im- paired Chemotaxis and neutrophil (PMN) function in glycogenosis (GSD) Ib. Pediatr Res 14:226 AGoogle Scholar
  176. 176.
    Kozlowski K (1976) Dactylitis secondary to chronic granulomatous disease (report of a case). Rad Diagn (Berl) 3: 347Google Scholar
  177. 177.
    Kragballe K, Borregaard N, Brandrup F, Koch C, Johansen KS (1981) Relation of monocyte and neutrophil oxidative metabolism to skin and oral lesions in carriers of chronic granulomatous disease. Clin Exp Immunol 43: 390PubMedGoogle Scholar
  178. 178.
    Kreutzer DL, Dreyfuss LA, Robertson DC (1979) Interaction of polymorpho- nuclear leukocytes with smooth and rough strains of Brucella abortus. Infect Immun 23: 737PubMedGoogle Scholar
  179. 179.
    Krinsky NI (1974) Singlet excited oxygen as a mediator of the antibacterial action of leukocytes. Science 186: 363PubMedGoogle Scholar
  180. 180.
    Kunin CM, Brandt D, Wood H (1969) Bacteriologic studies of rifampin, a new semisynthetic antibiotic. J Infect Dis 119: 132PubMedGoogle Scholar
  181. 181.
    Kvarstein B, Halvorsen KS (1970) Oxygen consumption during the initial stage of human leukocyte phagocytosis as a test for chronic granulomatous disease. Scand J Clin Lab Invest 26: 175PubMedGoogle Scholar
  182. 182.
    Landing BH, Shirkey HS (1957) A Syndrome of recurrent infection and infiltra- tion of viscera by pigmented lipid histiocytes. Pediatrics 20: 431PubMedGoogle Scholar
  183. 183.
    Lazarus GM, Neu HC (1975) Agents responsible for infection in chronic granulomatous disease of childhood. J Pediatr 86: 415PubMedGoogle Scholar
  184. 184.
    Lew PD, Southwick FS, Stossel TP, Whitin JC, Simons E, Cohen HJ (1981) A variant of chronic granulomatous disease: deficient oxidative metabolism due to a low-affinity NADPH oxidase. N Engl J Med 305: 1329PubMedGoogle Scholar
  185. 185.
    Lehrer RI (1969) Antifungal effects of peroxidase systems. J Bacteriol 99: 361PubMedGoogle Scholar
  186. 186.
    Lehrer RI (1970) Measurement of candidacidal activity of specific leukocyte types in mixed cell populations. I. Normal, myeloperoxidase-deficient and chronic granulomatous disease neutrophils. Infect Immun 2: 42PubMedGoogle Scholar
  187. 187.
    Lehrer RI (1971) Measurement of candidacidal activity of specific leukocyte types in mixed cell populations. II. Normal and chronic granulomatous disease eosinophils. Infect Immun 3: 800PubMedGoogle Scholar
  188. 188.
    Lehrer RI (1972) Functional aspect of a second mechanism of candidacidal activity by human neutrophils. J Clin Invest 51: 2566PubMedGoogle Scholar
  189. 189.
    Lehrer RI (1975) Fungicidal mechanisms of human monocytes. I Evidence for myeloperoxidase4inked and myeloperoxidase-independent candidacidal mechanisms. J Clin Invest 55: 338PubMedGoogle Scholar
  190. 190.
    Lehrer RI, Cline M (1969) Leukocyte myeloperoxidase deficiency and dis- seminated candidiasis: the role of myeloperoxidase in resistance to Candida infection. J Clin Invest 48: 1478PubMedGoogle Scholar
  191. 191.
    Lehrer RI, Cline MJ (1969) Interaction of Candida albicans with human leukocytes and serum. J Bacteriol 98: 996PubMedGoogle Scholar
  192. 192.
    Lehrer RI, Hanifin J, Cline MJ (1969) Defective bactericidal activity in myeloperoxidase-deficient human neutrophils. Nature 223: 78PubMedGoogle Scholar
  193. 193.
    Lewis VJ, Thacker WL, Shepard CC, McDade JE (1978) In vivo susceptibility of the Legionnaires Disease bacterium to ten antimicrobial agents. Antimicrob Agents Chemother 13: 419PubMedGoogle Scholar
  194. 194.
    Light DR, Walsh C, O’Callaghan AM, Goetzel EJ, Tauber AI (1981) Character- istics of the eofactor requirements for the superoxide-generating NADPH oxidase of human polymorphonuclear leukocytes. Biochemistry 20: 1468PubMedGoogle Scholar
  195. 195.
    Lobo MC, Mandell GL (1972) Treatment of experimental staphylococcal infec- tion with rifampin. Antimicrob Agents Chemother 2: 195PubMedGoogle Scholar
  196. 196.
    Lobo MC, Mandell GL (1973) The effect of antibiotics on Escherichia coli ingested by macrophages. Proc Soc Exp Biol Med 142: 1048PubMedGoogle Scholar
  197. 197.
    Loos H, Roos D, Weening R, Houwerzijl J (1976) Familial deficiency of gluta- thione reductase in human blood cells. Blood 48: 53PubMedGoogle Scholar
  198. 198.
    Lopez R, Ronda-Lain C, Tapia A, Waks SB, Tomasz A (1976) Suppression of the lytic and bactericidal effects of cell wall-inhibitory antibiotics. Antimicrob Agents Chemother 10: 697PubMedGoogle Scholar
  199. 199.
    Lorber B (1980) Rifampin in chronic granulomatous disease. N Engl J Med 303: 111 (letter)Google Scholar
  200. 200.
    Lowrie DB (1981) Survival of intracellular bacteria inside macrophages. Ann Immunol (Paris) 132D: 151Google Scholar
  201. 201.
    Lowrie DB, Aber VR, Carrol MEW (1979) Division and death rates of Salmonella typhimurium inside macrophages: use of penicillin as a probe. J Gen Microbiol 110: 409PubMedGoogle Scholar
  202. 202.
    Lyon MF (1972) X-chromosome inactivation and developmental patterns in mammals. Biol Rev 47: 1PubMedGoogle Scholar
  203. 203.
    MacFarlane PS, Speirs AL, Sommerville RG (1967) Fatal granulomatous disease of childhood and benign lymphocytic infiltration of the skin. Lancet 1: 408PubMedGoogle Scholar
  204. 204.
    Malawista SE, Gifford RH (1975) Chronic granulomatous disease of childhod (CGD) with leukocyte glutathione peroxidase(LGP) deficiency in a brother and sister: A likely autosomal recessive inheritance. Clin Res 23: 416AGoogle Scholar
  205. 205.
    Mandell GL (1973) Interaction of intraleukocytic bacteria and antibiotics. J Clin Invest 52: 1673PubMedGoogle Scholar
  206. 206.
    Mandell GL (1974) Bactericidal activity of aerobic and anaerobic polymorphonuclear neutrophils. Infect Immun 9: 337PubMedGoogle Scholar
  207. 207.
    Mandell GL, Hook EW (1969) Leukocyte function in chronic granulomatous disease of childhood. Studies on a 17-year-old boy. Am J Med 47: 473PubMedGoogle Scholar
  208. 208.
    Mandell GL, Hook EW (1969) Leukocyte bactericidal activity in chronic granulomatous disease: Correlation of bacterial hydrogen peroxide production and sus- ceptibility to inti-acellular killing. J Bacteriol 100: 531Google Scholar
  209. 209.
    Mandell GL, Vest TK (1972) Killing of intraleukocytic Staphylococcus aureus by rifampin: In vitro and in vivo studies. J Infect Dis 125: 486Google Scholar
  210. 210.
    Marcovitch H, Norman AP (1970) Treatment of nocardiosis. Lancet 11: 362 (letter)Google Scholar
  211. 211.
    Marsh WL, Øyen R, Nichols ME (1976) Kx antigen, the McLeod phenotype and chronic granulomatous disease: further studies. Vox Sang 31: 356PubMedGoogle Scholar
  212. 212.
    Martyn LJ, Lischner HW, Pileggi AJ, Harley RD (1972) Chorioretinal lesions in familial chronic granulomatous disease of childhood. Am J Ophthalmol 73: 403PubMedGoogle Scholar
  213. 213.
    Matamoros N, North ME, Ciria L, Webster ADB (1982) Chronic granulomatous disease with normal neutrophil glutathione peroxidase activity in a brother and sister. Acta Paediatr Scand 71: 327PubMedGoogle Scholar
  214. 214.
    Matsuda I, Oka Y, Taniguchi N, Furuyama M, Kodama S, Arashima S, Mitsuyama T (1976) Leukocyte glutathione peroxidase deficiency in a male patient with chronic granulomatous disease. J Pediatr 88: 581PubMedGoogle Scholar
  215. 215.
    McCallum JE, Brand E, Selker RG (1974) Osteomyelitis of the skull in chronic granulomatous disease of childhood. J Neurosurg 40: 764PubMedGoogle Scholar
  216. 216.
    McPhail LC, De Chatelet LR, Shirley PS, Wilfert C, Johnston RB, McCall CE (1977) Deficiency of NADPH oxidase activity in chronic granulomatous disease. J Pediatr 90: 213PubMedGoogle Scholar
  217. 217.
    McRipley RJ, Sbarra AJ (1967) Role of the phagocyte in host-parasite interac- tions. XII. Hydrogen peroxide-myeloperoxidase bactericidal system in the phagocyte. J Bacteriol 94: 1425Google Scholar
  218. 218.
    Medoff G, Kobayashi GS, Kwan CN, Schiessinger D, Venkov P (1972) Potentiation of rifampicin and 5-fluorocytosine as antifungal antibiotics by amphotericin B. Proc Natl Acad Sei USA 69: 196Google Scholar
  219. 219.
    Miller RM, Garbus J, Hornick RB (1972) Lack of enhanced oxygen consumption by polymorphonuclear leukocytes on phagocytosis of virulent Salmonella typhi. Science 175: 1010PubMedGoogle Scholar
  220. 220.
    Miller TE (1969) Killing and lysis of gram-negative bacteria through the syner- gistic effect of hydrogen-peroxide, ascorbic acid and lysozyme. J Bacteriol 98: 949PubMedGoogle Scholar
  221. 221.
    Mills EL, Rholl KS, Quie PG (1980) X-linked inheritance in females with CGD. J Clin Invest 66: 332PubMedGoogle Scholar
  222. 222.
    Miyazaki S, Shin H, Goya N, Nakagawara A (1976) Identification of a carrier mother of a female patient with chronic granulomatous disease. J Pediatr 89: 784PubMedGoogle Scholar
  223. 223.
    Moellering RC, Weinberg AN (1970) Persistent Salmonella infection in a female carrier for chronic granulomatous disease. Ann Intern Med 73: 595PubMedGoogle Scholar
  224. 224.
    Möhler DN, Majerus PW, Minnich V, Hess CE, Garrick MD (1970) Glutathione synthetase deficiency as a cause of hereditary hemolytic disease. N Engl J Med 283: 1253PubMedGoogle Scholar
  225. 225.
    Moosmann K, Bojanovsky A (1975) Rezidivierende Candidosis bei Myeloper- oxydasemangel. Monatsschr Kinderheilkd 123: 408PubMedGoogle Scholar
  226. 226.
    Musson RA, McPhail LC, Shafran H, Johnston RB (1982) Differences in the ability of human peripheral blood monocytes and in vitro monocyte-derived macrophages to produce Superoxide anion: Studies with cells from normals and patients with chronic granulomatous disease. J Reticuloendothel Soc 31: 261Google Scholar
  227. 227.
    Nakamura M, Nakamura MA, Yanai M (1981) Polarographic micromethods for the rapid assay of phagocytosis-connected oxygen consumption by leukocytes in diluted peripheral blood. J Lab Clin Med 97: 31PubMedGoogle Scholar
  228. 228.
    Nathan DG, Baehner RL, Weaver DK (1969) Failure of nitroblue tetrazolium reduetion in the phagocytic vacuoles of leukocytes in chronic granulomatous disease. J Clin Invest 48: 1895PubMedGoogle Scholar
  229. 229.
    Necheles TF, Maldonado N, Barquet-Chediak A, Allen DM (1969) Homozygous erythrocyte glutathione peroxidase deficiency: Clinical and biochemical studies. Blood 33: 164Google Scholar
  230. 230.
    Nelson JP (1970) Chronic granulomatous disease of childhood. Hematogenous Osteomyelitis due to nonpigmented Serratia marcescens. Rocky M Med J 67: 17Google Scholar
  231. 231.
    Newburger PE (1982) Superoxide generation by human fetal granulocytes. Pediatr Res 16: 373PubMedGoogle Scholar
  232. 232.
    Newburger PE, Latt SA (1979) Improved fluorescent staining of interphase nuclei for prenatal diagnosis. Lancet 1: 1144 (letter)Google Scholar
  233. 233.
    Newburger PE, Cohen HJ, Rothchild SB, Hobbins JC, Malawista SE, Mahoney MJ (1979) Prenatal diagnosis of chronic granulomatous disease. N Engl J Med 300: 178PubMedGoogle Scholar
  234. 234.
    Nezelof C, Vilde F, Ganter P (1972) Les lesions anatomiques de la granulomatose chronique. Etüde histopathologique et histochimique de cinq observations. Ann Anat Pathol 17: 101Google Scholar
  235. 235.
    Nguyen BT, Stadtsbaeder S (1978) In vitro effects of trimethoprim ± Sulfamethoxazole upon Toxoplasma gondii Rh within human monocytes (antitoxoplasma activity of trimethoprim-sulfamethoxazole). Drugs Exp Clin Res 4: 43Google Scholar
  236. 236.
    Nishida M, Mine Y, Nonoyama S (1978) Relationship between the effect of car- benicillin on the phagocytosis and killing of Proteus mirabilis by polymorphonuclear leukocytes and therapeutic efficacy. J Antibiot (Tokyo) 31: 719Google Scholar
  237. 237.
    Nishimura ET, Whest GM, Yang H (1976) Ultrastructural localization of per- oxidatic catalase in human peripheral blood leukocytes. Lab Invest 34: 60PubMedGoogle Scholar
  238. 238.
    Nishimura Y, Chida N, Hayashi T, Arakawa T (1972) Homozygous glutathione peroxidase deficiency of erythrocytes and leukocytes. Tohoku J Exp Med 108: 207PubMedGoogle Scholar
  239. 239.
    Noriega AR, Gutierrez A, Palenque E, Otero JR, Dorado MS (1981) Treatment of human brucellosis with prolonged therapy with sulfamethoxazole-trimethoprim. 12th International Congresss Chemotherapy (Florence), abstract 659Google Scholar
  240. 240.
    Ochs HD, Igo RP (1973) The NBT slide test: A simple Screening method for detecting chronic granulomatous disease and female carriers. J Pediatr 83: 77Google Scholar
  241. 241.
    Pagani A, Spalla R, Ferrari FA, Duse M, Lenzi L, Bretz U, Baggiolini M, Siccardi AG (1981) Defective aspergillus killing by neutrophil leucocytes in a case of systemic aspergillosis. Clin Exp Immunol 43: 201PubMedGoogle Scholar
  242. 242.
    Park BH, Holmes BM, Rodey GE, Good RA (1969) Nitroblue tetrazolium test in children with fatal granulomatous disease and newborn infants. Lancet 1: 157PubMedGoogle Scholar
  243. 243.
    Parry MF, Root RK, Metealf JA, Delaney KK, Kaplow LS, Richar WJ (1981) Myeloperoxidase deficiency. Prevalence and clinical significance. Ann Intern Med 95: 293Google Scholar
  244. 244.
    Paul BB, Jacobs AA, Strauss RR, Sbarra AJ (1970) Role of the phagocyte in host-parasite interactions. XXIV. Aldehyde generation by the myeloperoxidase- H202-chloride antimicrobial system: a possible in vivo mechanism of action. Infect Immun 2: 414PubMedGoogle Scholar
  245. 245.
    Pedersen FK, Johansen S, Rosenkvist J, Tygstrup I, Valerius NH (1979) Refrac- tory Pneumocystis carinii infection in chronic granulomatous disease: Successful treatment with granulocytes. Pediatrics 64: 935Google Scholar
  246. 246.
    Perry HB, Boulanger M, Pennoyer D (1980) Chronic granulomatous disease in an adult with recurrent abscesses. Arch Surg 115: 200PubMedGoogle Scholar
  247. 247.
    Philippart AI, Colodny AH, Baehner RL (1969) Chronic granulomatous disease of childhood. J Pediatr Surg 4: 85PubMedGoogle Scholar
  248. 248.
    Philippart AI, Colodny AH, Baehner RL (1972) Continuous antibiotic therapy in chronic granulomatous disease: preliminary communication. Pediatrics 50: 923PubMedGoogle Scholar
  249. 249.
    Pincus SH, Klebanoff SJ (1971) Quantitative leukocyte iodination. N Engl J Med 284: 744PubMedGoogle Scholar
  250. 250.
    Pitt J, Bernheimer HP (1974) Role of peroxide in phagocytic killing of pneumo- cocci. Infect Immun 9: 48PubMedGoogle Scholar
  251. 251.
    Preimesberger KF, Goldberg ME (1974) Acute liver abscess in chronic granulomatous disease of childhood. Radiology 110: 147PubMedGoogle Scholar
  252. 252.
    Preisig E, Hitzig HW (1971) Nitroblue-tetrazolium test for the detection of chronic granulomatous disease - technical modification. Eur J Clin Invest 1: 409PubMedGoogle Scholar
  253. 253.
    Prokesch RC, Hand WL (1982) Antibiotic entry into human polymorphonuclear leukocytes. Antimicrob Agents Chemother 21: 373PubMedGoogle Scholar
  254. 254.
    Pruul H, McDonald PJ (1979) Enhancement of leukocyte activity against Escherichia coli after brief exposure to chloramphenicol. Antimicrob Agents Chemother 16: 695PubMedGoogle Scholar
  255. 255.
    Quie PG, White JG, Holmes B, Good RA (1967) In vitro bactericidal capacity of human polymorphonuclear leukocytes. Diminished activity in chronic granulomatous disease in childhood. J Clin Invest 46: 668Google Scholar
  256. 256.
    Raubitschek AA,Levin AS, Stites DP, Shaw EB, Fudenberg HH (1973) Normal granulocyte infusion therapy for aspergillosis in chronic granulomatous disease. Pediatrics 51: 230Google Scholar
  257. 257.
    Reed PW (1969) Glutathione and the hexose monophosphate Shunt in phago- cytizing and hydrogen peroxide-treated rat leukocytes. J Biol Chem 244: 2459PubMedGoogle Scholar
  258. 258.
    Reeves DS, Wilkinson PJ (1979) The pharmacokinetics of trimethoprim and tri- methoprim/sulphonamide combinations, including penetration into body tissue. Infection 7: S330PubMedGoogle Scholar
  259. 259.
    Repine JE, Rasmussen B, White JG (1979) An improved nitroblue tetrazolium test using phorbol myristate acetate-coated coverslips. Am J Clin Pathol 71: 582PubMedGoogle Scholar
  260. 260.
    Repine JE, Clawson CC, White JG, Holmes B (1975) Spectrum of function of neutrophils from carriers of sex-linked chronic granulomatous disease. J Pediatr 87: 901PubMedGoogle Scholar
  261. 261.
    Repine JE, White JG, Clawson CC, Holmes BM (1974) Effects of phorbol myristate acetate on the metabolism and ultrastructure of neutrophils in chronic granulomatous disease. J Clin Invest 54: 83PubMedGoogle Scholar
  262. 262.
    Repine JE, White JG, Clawson CC, Holmes BM (1974) The influence of phorbol myristate acetate on oxygen consumption by polymorphonuclear leukocytes. J Lab Clin Med 83: 911PubMedGoogle Scholar
  263. 263.
    Rieder J, Schwartz DE, Zangaglia O (1974) Passage of Sulfamethoxazole and trimethoprim into the bile in man. Chemotherapy 20: 65PubMedGoogle Scholar
  264. 264.
    Rister M, Fröhlich G, Seiferth J (1974) Chronische septische Granulomatose bei zwei Brüdern. Dtsch Med Wochenschr 99: 2172PubMedGoogle Scholar
  265. 265.
    Roback SA, Weintraub WH, Good RA, Lindsay WG, Quie PG, Park B, Leonard AS (1971) Chronic granulomatous disease: surgical considerations. J Pediatr Surg 6: 601PubMedGoogle Scholar
  266. 266.
    Robertson CF, Thong YH, Hodge GL, Cheney K (1979) Primary myeloperoxidase deficiency associated with impaired neutrophil margination and Chemotaxis. Acta Paediatr Scand 68: 915PubMedGoogle Scholar
  267. 267.
    Rodey GE, Park BH, Windhorst DB, Good RA (1969) Defective bactericidal activity of monocytes in fatal granulomatous disease. Blood 33: 813PubMedGoogle Scholar
  268. 268.
    Rodey GE, Jacob HS, Holmes B, McArthur JR, Good RA (1970) Leukocyte glucose-6-phosphate dehydrogenase levels and bactericidal activity. Lancet 1: 355PubMedGoogle Scholar
  269. 269.
    Romeo D, Zabucchi G, Miani N, Rossi F (1975) Ion movement across leukocyte plasma membranes and excitation of their metabolism. Nature 253: 542PubMedGoogle Scholar
  270. 270.
    Roos D, Weening RS (1979) Defects in the oxidative killing of microorganisms by phagocytic leukocytes. In: Fitz Simons D (ed) Oxygen free radicals and tissue damage. Ciba Found Symp 65: 225Google Scholar
  271. 271.
    Roos D, Weening RS, Wyss SR, Aebi HE (1980) Protection of human neutrophils by endogenous catalase. Studies with cells from catalase-deficient individuals. J Clin Invest 65: 1515Google Scholar
  272. 272.
    Roos D, Weening RS, Voetman AA, van Schaik LJ, Bot AAM, Meerhof LJ, Loos JA (1979) Protection of phagocytic leukocytes by endogenous glutathione: Studies in a family with glutathione reductase deficiency. Blood 53: 851Google Scholar
  273. 273.
    Root RK (1974) Correction of the function of chronic granulomatous disease (CGD) granulocytes (PMN) with extracellular H202. Clin Res 22: 452AGoogle Scholar
  274. 274.
    Root RK (1981) Antibacterial properties of polymorphonuclear leukocytes and their interactions with antibiotics. In: Schlesinger D (ed) Microbiology — 1981. American Society for Microbiology, Washington, p 176Google Scholar
  275. 275.
    Root RK, Isturiz R, Molavi A, Metealf JA, Malech HL (1981) Interactions be- tween antibiotics and human neutrophils in the killing of staphylococci: Studies with normal and cytochalasin B-treated cells. J Clin Invest 67: 247Google Scholar
  276. 276.
    Rosen H, Klebanoff SJ (1976) Chemiluminescence and Superoxide produetion by myeloperoxidase-deficient leukocytes. J Clin Invest 58: 50PubMedGoogle Scholar
  277. 277.
    Rossi F, Zatti M (1964) Biochemical aspects of phagocytosis in polymorphonuclear leukocytes. NADH and NADPH oxidation by the granules of resting and phagocytizing cells. Experientia 20: 21Google Scholar
  278. 278.
    Sabath LD, Gerstein DA, Leaf CD, Finland M (1970) Increasing the usefulness of antibiotics: treatment of infections caused by gram negative bacilli. Clin Pharmacol Ther 11: 161PubMedGoogle Scholar
  279. 279.
    Salmon SE, Cline MJ, Schultz J, Lehrer RI (1970) Myeloperoxidase deficiency. Immunologic study of a genetic leukocyte defect. N Engl J Med 282: 250Google Scholar
  280. 280.
    Samuels LD (1971) Liver scans in chronic granulomatous disease of childhood. Pediatrics 48: 41PubMedGoogle Scholar
  281. 281.
    Sanders DY, Goodman HO, Cooper MR (1974) Chronic granulomatous disease in a child with Klinefelter’s Syndrome. Pediatrics 54: 373PubMedGoogle Scholar
  282. 282.
    Sbarra AJ, Karnovsky ML (1959) The biochemical basis of phagocytosis. I. Metabolic changes during the ingestion of particles by polymorphonuclear leukocytes. J Biol Chem 234: 1355Google Scholar
  283. 283.Scully C (1981) Orofacial manifestations of chronic granulomatous disease of childhood. Oral Surg 51: 148Google Scholar
  284. 284.
    Scully RE, Galdabini JJ, McNeely BU (1981) Case 26 - 1981. N Engl J Med 304: 1592Google Scholar
  285. 285.
    Segal AW, Jones OTG (1978) Novel cytochrome b system in phagocytic vacuoles of human granulocytes. Nature 276: 515PubMedGoogle Scholar
  286. 286.
    Segal AW, Jones OTG (1979) Reduction and subsequent oxidation of a cytochrome b of human neutrophils after Stimulation with phorbol myristate acetate. Biochem Biophys Res Commun 88: 130PubMedGoogle Scholar
  287. 287.
    Segal AW, Jones OTG (1979) The subcellular distribution and some properties of the cytochrome b component of the microbicidal oxidase system of human neutrophils. Biochem J 182: 181PubMedGoogle Scholar
  288. 288.
    Segal AW, Jones OTG (1980) Rapid incorporation of the human neutrophil plasma membrane cytochrome b into phagocytic vacuoles. Biochem Biophys Res Commun 92: 710PubMedGoogle Scholar
  289. 289.
    Segal AW, Levi AJ (1973) The mechanism of the entry of dye into neutrophils in the nitroblue tetrazolium ( NBT) test. Clin Sei Mol Med 45: 817Google Scholar
  290. 290.
    Segal AW, Jones OTG, Webster D, Allison AC (1978) Absence of a newly de- scribed cytochrome b from neutrophils of patients with chronic grnaulomatous disease. Lancet 11: 446Google Scholar
  291. 291.
    Segal AW, Geisow M, Garcia R, Harper A, Miller R (1981) The respiratory burst of phagocytic cells is associated with a rise in vacuolar pH. Nature 290: 406PubMedGoogle Scholar
  292. 292.
    Seger RA, Hitzig WH (1981) Chronic granulomatous disease. Further evidence for an abnormality in transmembrane potential. Eur J Pediatr 135: 332Google Scholar
  293. 293.
    Seger R, Steinmann B (1981) Prenatal diagnosis of chronic granulomatous disease: Unreliability of fibroblast nitroblue tetrazolium test. Lancet 1: 1216 (letter)Google Scholar
  294. 294.
    Seger RA, Baumgartner S, Tiefenauer LX, Gmünder FK (1981) Chronic granulomatous disease: effect of sulfamethoxazole/trimethoprim on neutrophil microbicidal funetion. Helv Paediatr Acta 36: 579PubMedGoogle Scholar
  295. 295.
    Seger RA, Hollis DG, Weaver RE, Hitzig WH (1982) Chronic granulomatous disease: Fatal septicemia due to an unnamed gram negative bacterium. J Clin Micro- biol 16: 821Google Scholar
  296. 296.
    Seger R, Frater-Schröder M, Hitzig WH, Wüdfeuer A, Linnell JC (1980) Granulo- cyte dysfunction in transcobalamin II-deficiency responding to leueovorin or hydroxocobalamin-plasma transfusion. J Inher Metabol Dis 3: 3Google Scholar
  297. 297.
    Seger R, Steinmann B, Tiefenauer L, Matsunaga T, Gitzelmann R (in press) Glycogenosis Ib: Neutrophil microbicidal defect due to impaired hexose mono- phosphate Shunt. Pediatr ResGoogle Scholar
  298. 298.
    Seger RA, Tiefenauer L, Matsunaga T, Wildfeuer A, NewburgerPE (1983) Chronic granulomatous disease due to granulocytes with abnormal NADPH oxidase activity and deficient cytochrome b. Blood 61: 423PubMedGoogle Scholar
  299. 299.
    Seger R, Galle J, Wildfeuer A, Frater-Schröder M, Linnell J, Hitzig WH (1980) Impaired funetions of lymphocytes and granulocytes in transcobalamin II deficiency and their response to treatment. In: Seligmann M, Hitzig WH (eds) Primary immunodeficiencies. Elsevier/North Holland, Amsterdam, p 353Google Scholar
  300. 300.
    Seligmann BE, Gallin JI (1980) Use of lipophilic probes of membrane potential to assess human neutrophil activation. Abnormality in chronic granulomatous disease. J Clin Invest 66: 493Google Scholar
  301. 284.
    Seligmann B, Chused TM, Gallin JI (1981) Human neutrophil heterogeneity iden- tified using flow microfluorometry to monitor membrane potential. J Clin Invest 68: 1125.PubMedGoogle Scholar
  302. 285.
    Selvaraj RJ, Sbarra AJ (1967) The role of the phagocyte in host-parasite interactions. VII. Di- and triphosphopyridine nucleotide kinetics during phagocytosis. Biochem Biophys Acta 141: 243Google Scholar
  303. 286.
    Shurin S, Cohen H, Newburger P (1981) Chronic granulomatous disease due to an abnormal NADPH-dependent oxidase. Blood 58: 77AGoogle Scholar
  304. 287.
    Silverglade D (1979) Oral lesions associated with chronic granulomatous disease. J Dent Child 46: 324Google Scholar
  305. 305.
    Sloan EP, Crawford DR, Schneider DI (1981) Isolation of plasma membrane from human neutrophils and determination of cytochrome b and quinone content. J Exp Med 153: 1316PubMedGoogle Scholar
  306. 306.
    Snyderman R, Pike MC, Altmann LC (1975) Abnormalities of leukocyte Chemotaxis in human disease. Ann NY Acad Sei 256: 386Google Scholar
  307. 307.
    Solberg CO (1972) Protection of phagocytized bacteria against antibiotics: a new method for the evaluation of neutrophil granulocyte funetions. Acta Med Scand 191: 383PubMedGoogle Scholar
  308. 308.
    Solberg CO, Helium KB (1978) Protection of phagocytosed bacteria against anti- microbial agents. Scand J Infect Dis [Suppl] 14: 246Google Scholar
  309. 309.
    Spielberg SP, Boxer LA, Oliver JM, Allen JM, Schulman JD (1979) Oxidative damage to neutrophils in glutathione synthetase deficiency. Br J Haematol 42: 215PubMedGoogle Scholar
  310. 310.
    Spielberg SP, Garrick MD, Corash LM, Butler JDB, Tietze F, Rogers LV, Schulman JD (1978) Biochemical heterogeneity in glutathione synthetase deficiency. J Clin Invest 61: 1417PubMedGoogle Scholar
  311. 311.
    Subbartnam S, Miller NG (1979) Comparative study of the effect of thiabenda- zole and fungizone on Histoplasma capsulatum in macrophages. Sabouraudia 17: 331Google Scholar
  312. 312.
    Sutcliffe J, Chrispin AR (1970) Chronic granulomatous disease. Br J Radiol 43: 110PubMedGoogle Scholar
  313. 313.
    Suzuki Y, Lehrer RI (1980) NAD(P)H oxidase activity in human neutrophils stimulated by phorbol myristate acetate. J Clin Invest 66: 1409PubMedGoogle Scholar
  314. 314.
    Svedhem A, Iwarson S (1979) Cerebrospinal fluid concentrations of trimetho- prim during oral and parenteral treatment. J Antimicrob Chemother 5: 717PubMedGoogle Scholar
  315. 315.
    Symchych PS, Wanstrup J, Andersen V (1968) Chronic granulomatous disease of childhood. A morphologic study. Acta Pathol Microbiol Scand 74: 179Google Scholar
  316. 316.
    Schaller J (1972) Illness resembling lupus erythematosus in mothers of boys with chronic granulomatous disease. Ann Intern Med 76: 747PubMedGoogle Scholar
  317. 317.
    Schultz J, Kaminker K (1962) Myeloperoxidase of the leucocyte of normal human blood. I. Content and localization. Arch Biochem Biophys 96: 465Google Scholar
  318. 318.
    Schultz J, Schmuckler HW (1964) Myeloperoxidase of leucocyte of normal human blood. II. Isolation, spectrophotometry and amino acid analysis. Bio- chemistry 3: 1234Google Scholar
  319. 319.
    Stendahl O, Lindgren S (1976) Function of granulocytes with deficient myelo- peroxidase-mediated iodination in a patient with generalized pustular Psoriasis. Scand J Haematol 16: 144PubMedGoogle Scholar
  320. 320.
    Stites DP, Glezen WP (1967) Pulmonary nocardiosis in childhood. A case report. Am J Dis Child 114: 101Google Scholar
  321. 321.
    Stjernholm RL, Allen RC, Steele RH, Waring WW, Harris JA (1973) Impaired chemiluminescence during phagocytosis of opsonized bacteria. Infect Immun 7: 313PubMedGoogle Scholar
  322. 322.
    Stossel TP, Mason RJ, Smith AL (1974) Lipid peroxidation by human blood phagocytes. J Clin Invest 54: 638PubMedGoogle Scholar
  323. 323.
    Stossel TP, Root RK, Vaughan M (1972) Phagoeytosis in chronic granulomatous disease and the Chediak-Higashi Syndrome. N Engl J Med 286: 120PubMedGoogle Scholar
  324. 324.
    Takeshige K, Matsumoto T, Shibata R, Minakami S (1979) Simple and rapid method for the diagnosis of chronic granulomatous disease, measuring hydrogen peroxide and Superoxide anions released from leukocytes in whole blood. Clin Chim Acta 92: 329PubMedGoogle Scholar
  325. 325.
    Tappel AL (1973) Lipid peroxidation damage to cell components. Fed Proc 32: 1870PubMedGoogle Scholar
  326. 326.
    Taswell HF, Lewis JC, Marsh WL, Wimer BM, Pineda AA, Brzica SM (1977) Erythrocyte morphology in genetic defects of the Rh and Kell blood group sys- tems. Mayo Clin Proc 52: 157PubMedGoogle Scholar
  327. 327.
    Tauber AI, Babior BM (1977) Evidence for hydroxyl radical production by human neutrophils. J Clin Invest 60: 374PubMedGoogle Scholar
  328. 328.
    Tauber AI, Goetzel EJ (1979) Structural and catalytic properties of the solubil- ized superoxide-generating activity of human polymorphonuclear leukocytes. Solubilization, stabilization in Solution, and partial characterization. Biochem- istry 18: 5576Google Scholar
  329. 329.
    Thompson EN, Soothill JF (1970) Chronic granulomatous disease: Quantitative clinicopathological relationships. Arch Dis Child 45: 24Google Scholar
  330. 330.
    Undritz E (1959) Leucocytes. Morphology and genetics in hereditary constitu- tional anomalies of leucocytes. 7th European Congress Haematology (abstract 102 )Google Scholar
  331. 331.
    Undritz E (1966) Die Alius-Grignaschi-Anomalie: Der erblich-konstitutionelle Peroxidasedefekt der Neutrophilen und Monozyten. Blut 14: 129PubMedGoogle Scholar
  332. 332.
    Urban C, Becker H, Mutz I, Fritsch G (1980) BCG-Impfkomplikation bei Septischer Granulomatose. Klin Paediatr 192: 13Google Scholar
  333. 333.
    Van der Hart M, Szaloky A, Van Loghem JJ (1968) A new antibody associated with the Kell blood group system. Vox Sang 15: 456PubMedGoogle Scholar
  334. 334.
    Van der Waaij D, Berghuis JM, Lekkerkerk JEC (1972) Colonization resistance of the digestive tract of mice during systemic antibiotic treatment. J Hyg (Camb) 70: 605Google Scholar
  335. 335.
    Van Rhenen DJ, Koolen MI, Feltkamp-Vroom TM, Weening RS (1979) Immune complex glomerulonephritis in chronic granulomatous disease. Acta Med Scand 206: 233PubMedGoogle Scholar
  336. 336.
    Van Zwieten R, Wever R, Hamers MN, Weening RS, Roos D (1981) Extracellular proton release by stimulated neutrophils. J Clin Invest 68: 310PubMedGoogle Scholar
  337. 337.
    Vaudaux P, Waldvogel FA (1979) Gentamicin antibacterial activity in the pres- ence of human polymorphonuclear leukocytes. Antimicrob Agents Chemother 16: 743PubMedGoogle Scholar
  338. 338.
    Vaudaux P, Waldvogel FA (1980) Gentamicin inactivation in purulent exudates: role of cell lysis. J Infect Dis 142: 586PubMedGoogle Scholar
  339. 339.
    Veale DR, Finch H, Smith H (1976) Penetration of penicillin into human phago- cytes containing Neisseria gonorrhoeae: intracellular survival and growth at Optimum concentrations of antibiotic. J Gen Microbiol 95: 353Google Scholar
  340. 340.
    Verronen PC (1974) Presumed disseminated BCG in a boy with chronic granulomatous disease of childhood. Acta Paediatr Scand 63: 627PubMedGoogle Scholar
  341. 341.
    Vilde F, Nezelof C (1976) La maladie granulomateuse familiale. Donnees histo- pathologiques et histogenetiques. Ann Anat Pathol 21: 59Google Scholar
  342. 342.
    Vilde JL (1971) Le deficit phagocytaire de la granulomatose chronique familiale. Etüde du pouvoir bactericide des leucocytes chez 8 malades. Ann Inst Pasteur 120: 382Google Scholar
  343. 343.
    Voetman AA, Loos JA, Roos D (1980) Changes in the level of glutathione in phagocytosing human neutrophils. Blood 55: 741PubMedGoogle Scholar
  344. 344.
    Voetman AA, Weening RS, Hamers MN, Meerhof LJ, Bot AA, Roos D (1981) Phagocytosing human neutrophils inactivate their own granular enzymes. J Clin Invest 67: 1541PubMedGoogle Scholar
  345. 345.
    Wade JC, Schimpff SC, HargadonMT, FortnerCL, Young VM, Wiernik PH (1981) A comparison of trimethoprim-sulfamethoxazole plus nystatin with gentamicin plus nystatin in the prevention of infections in acute leukemia. N Engl J Med 304: 1057Google Scholar
  346. 346.
    Wallace RJ, Wissk, Bushby MB, Hollowell DC (1982) In vitro activity of tri- methoprim and Sulfamethoxazole against the non-tuberculous mycobacteria. Rev Infect Dis 4: 326PubMedGoogle Scholar
  347. 347.
    Ward PA, Schlegel RJ (1969) Impaired leucotactic responsiveness in a child with reeurrent infections. Lancet 11: 344Google Scholar
  348. 348.
    Weemaes C, Leijh P, Blusse van Oud Alblas D, Van der Meer J, Van Furth R (1980) Normal microbicidal function of monocytes in a girl with chronic granulomatous disease. Acta Paediatr Scand 70: 421Google Scholar
  349. 349.
    Weening RS, Roos D, WeemaesCMR, Homan-Müller JWT, van Schaik MLJ (1976) Defective initiation of the metabolic Stimulation in phagocytizing granulocytes: a new congenital defect. J Lab Clin Med 88: 757Google Scholar
  350. 350.
    Werlin SL, Chusid MJ, Caya J, Oechler HW (1982) Colitis in chronic granulomatous disease. Gastroenterology 82: 328PubMedGoogle Scholar
  351. 351.
    Westminster Hospitals Bone-Marrow Transplant Team (1977) Bone-marrow transplant from an unrelated donor for chronic granulomatous disease. Lancet 1: 210Google Scholar
  352. 352.
    Whitin JC, Chapman CE, Simons ER, Chovaniec ME, Cohen HJ (1980) Correla- tion between membrane potential changes and Superoxide production in human granulocytes stimulated by phorbol myristate acetate. Evidence for defective activation in chronic granulomatous disease. J Biol Chem 255: 1874Google Scholar
  353. 353.
    Wildfeuer A, Krawinkel M, Lange CE, Voigt WH (1980) Leberabszess bei chronischer Granulomatose des Kindesalters. Klin Paediatr 192: 241Google Scholar
  354. 354.
    Wilson CB, Tsai V, Remington JS (1980) Failure to trigger the oxidative metabolic burst by normal macrophages. Possible mechanism for survival of intracellular pathogens. J Exp Med 151: 328Google Scholar
  355. 355.
    Wimer BM, Marsh WL, Taswell HF, Galey WR (1977) Haematological changes associated with the McLeod phenotype of the Kell blood group system. Br J Haematol 36: 219PubMedGoogle Scholar
  356. 356.
    Windhorst DB, Good RA (1971) Dermatologie manifestations of fatal granulomatous disease of childhood. Arch Dermatol 103: 351PubMedGoogle Scholar
  357. 357.
    Windhorst DB, Holmes B, Good RA (1967) A newly defined X-linked trait in man with demonstration of the Lyon effect in carrier females. Lancet 1: 737PubMedGoogle Scholar
  358. 358.
    Windhorst DB, Page AR, Holmes B, Quie PG, Good RA (1968) The pattern of genetic transmission of the leucocyte defect in fatal granulomatous disease of childhood. J Clin Invest 47: 1026PubMedGoogle Scholar
  359. 359.
    Woeber KA, Ingbar SH (1973) Metabolism of L-thyroxine by phagocytosing human leukocytes. J Clin Invest 52: 1796PubMedGoogle Scholar
  360. 360.
    Wolf JE, Ebel LK (1978) Chronic granulomatous disease: report of case and review of the literature. J Am Dent Assoc 96: 292PubMedGoogle Scholar
  361. 361.
    Wolfson JJ, Quie PG, Laxdal SD, Good RA (1968) Roentgenologic manifestations in children with a genetic defect of polymorphonuclear leukocyte function. Radiology 91: 37PubMedGoogle Scholar
  362. 362.
    Wolfson JJ, Kane WJ, Laxdal SD, Good RA, Quie PG (1969) Bone findings in chronic granulomatous disease of childhood. J Bone Joint Surg [Am] 51: 1573Google Scholar
  363. 363.
    Woodward TE, Smadel JE (1964) Management of typhoid fever and its com- plications. Ann Intern Med 60: 144PubMedGoogle Scholar
  364. 364.
    Wright DG, Robichaud KJ, Pizzo PA, Deisseroth AB (1981) Lethal pulmonary reactions associated with the combined use of amphotericin B and leukocyte transfusion. N Engl J Med 304: 1185PubMedGoogle Scholar
  365. 365.
    Wysocki GP, Brooke RI (1978) Oral manifestations in chronic granulomatous disease. Oral Surg 46: 815PubMedGoogle Scholar
  366. 366.
    Wyss SR, Aebi H (1975) Properties of leukocyte catalase in Swiss type acatalas- emia: A comparative study of normals, heterozygotes and homozygotes. Enzyme 20: 257Google Scholar
  367. 367.
    Yagawa K, Okamura J (1981) Role of adenosine deaminase in activation of macrophages. Infect Immun 32: 394PubMedGoogle Scholar
  368. 368.
    Yogman MS, Touloukian RJ, Gallagher R (1974) Intestinal granulomatosis in chronic granulomatous disease and in Crohn’s disease. N Engl J Med 290: 228 (letter)Google Scholar
  369. 369.
    Yomtovian R, Abramson J, Quie P, McCulloughJ (1981) Granulocyte transfusion therapy in chronic granulomatous disease: report of a patient and review of the literature. Transfusion 21: 739Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1984

Authors and Affiliations

  • R. Seger
    • 1
  1. 1.Universitäts-Kinderklinik ZürichSwitzerland

Personalised recommendations