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Electrolyte abnormalities in cystic fibrosis: systematic review of the literature

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Abstract

Background

Cystic fibrosis per se can sometimes lead to hyponatremia, hypokalemia, hypochloremia or hyperbicarbonatemia. This tendency was first documented 60 years ago and has subsequently been confirmed in single case reports or small case series, most of which were retrospective. However, this issue has not been addressed analytically. We have therefore systematically reviewed and analyzed the available literature on this subject.

Methods

This was a systematic review of the literature.

Results

The reports included in this review cover 172 subacute and 90 chronic cases of electrolyte imbalances in patients with cystic fibrosis. The male:female ratio was 1.57. Electrolyte abnormalities were mostly associated with clinically inapparent fluid volume depletion, mainly affected patients aged ≤2.5 years, frequently tended to recur and often were found before the diagnosis of cystic fibrosis was established. Subacute presentation often included an history of heat exposure, vomiting, excessive sweating and pulmonary infection. History of chronic presentation, in contrast, was often inconspicuous. The tendency to hypochloremia, hypokalemia and metabolic alkalosis was similar between subacute and chronic patients, with hyponatremia being more pronounced (P < 0.02) in subacute compared to chronic presentations. Subacute cases were treated parenterally; chronic ones were usually managed with oral salt supplementation. Retention of urea and creatinine was documented in 38 % of subacute cases.

Conclusions

The findings of our review suggest that physicians should be aware that electrolyte abnormalities can occur both as a presenting and a recurring feature of cystic fibrosis.

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References

  1. Liamis G, Milionis H, Elisaf M (2008) A review of drug-induced hyponatremia. Am J Kidney Dis 52:144–153

    PubMed  CAS  Google Scholar 

  2. Greenlee M, Wingo CS, McDonough AA, Youn JH, Kone BC (2009) Narrative review: evolving concepts in potassium homeostasis and hypokalemia. Ann Intern Med 150:619–625

    PubMed  Google Scholar 

  3. Kerem E (2006) Atypical CF and CF related diseases. Paediatr Respir Rev 7[Suppl 1]:S144–S146

    PubMed  Google Scholar 

  4. Kessler WR, Andersen DH (1951) Heat prostration in fibrocystic disease of the pancreas and other conditions. Pediatrics 8:648–656

    PubMed  CAS  Google Scholar 

  5. Moher D, Liberati A, Tetzlaff J, Altman DG, PRISMA Group (2009) Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Ann Intern Med 151:264–269

    PubMed  Google Scholar 

  6. Anonymous (1964) Convulsions after therapy for alveolar hypoventilation. JAMA 189:1023

    Google Scholar 

  7. Sojo A, Rodríguez-Soriano J, Vitoria JC, Vazquez C, Ariceta G, Villate A (1994) Chloride deficiency as a presentation or complication of cystic fibrosis. Eur J Pediatr 153:825–828

    PubMed  CAS  Google Scholar 

  8. Beckerman RC, Taussig LM (1979) Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. Pediatrics 63:580–583

    PubMed  CAS  Google Scholar 

  9. O'Sullivan BP, Freedman SD (2009) Cystic fibrosis. Lancet 373:1891–1904

    PubMed  Google Scholar 

  10. Akgün C, Başaranoğlu M, Yuca SA, Akbayram S, Arslan Ş, Kirimi E (2009) Pseudo–Bartter’s syndrome associated with cystic fibrosis. Nobel Med 5:43–45

    Google Scholar 

  11. Al-Ghimlas F, Faughnan ME, Tullis E (2012) Metabolic alkalosis in adults with stable cystic fibrosis. Open Respir Med J 6:59–62

    PubMed Central  PubMed  Google Scholar 

  12. Arvanitakis SN, Lobeck CC (1973) Metabolic alkalosis and salt depletion in cystic fibrosis. J Pediatr 82:535–545

    PubMed  CAS  Google Scholar 

  13. Augusto JF, Sayegh J, Malinge MC, Illouz F, Subra JF, Ducluzeau PH (2008) Severe episodes of extra cellular dehydration: an atypical adult presentation of cystic fibrosis. Clin Nephrol 69:302–305

    PubMed  Google Scholar 

  14. Baird JS, Walker P, Urban A, Berdella M (2002) Metabolic alkalosis and cystic fibrosis. Chest 122:755–756

    PubMed  Google Scholar 

  15. Ballestero Y, Hernandez MI, Rojo P, Manzanares J, Nebreda V, Carbajosa H, Infante E, Baro M (2006) Hyponatremic dehydration as a presentation of cystic fibrosis. Pediatr Emerg Care 22:725–727

    PubMed  Google Scholar 

  16. Bar-Or O, Blimkie CJ, Hay JA, MacDougall JD, Ward DS, Wilson WM (1992) Voluntary dehydration and heat intolerance in cystic fibrosis. Lancet 339:696–699

    PubMed  CAS  Google Scholar 

  17. Bates CM, Baum M, Quigley R (1997) Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent. J Am Soc Nephrol 8:352–355

    PubMed  CAS  Google Scholar 

  18. Beckerman RC (1979) Metabolic alkalosis in infants with cystic fibrosis. Pediatrics 64:389

    PubMed  CAS  Google Scholar 

  19. Berezin S, Ruddy RM, Dozor AJ, Newman L (1989) Hypoelectrolytemia, hypovolemia, and alkalosis in cystic fibrosis with wood-burning stove in winter. Pediatr Emerg Care 5:189–190

    PubMed  CAS  Google Scholar 

  20. Bianchetti MG, Mauri S (1996) Hypotonic dehydration in cystic fibrosis: mild or severe disease phenotype? J Pediatr 128:723

    PubMed  CAS  Google Scholar 

  21. Bijman J (1987) Transport processes in the eccrine sweat gland. Kidney Int Suppl 21:S109–S112

    PubMed  CAS  Google Scholar 

  22. Carlile JR, Pafford DM, Waring WW (1983) Severe hypernatremia in cystic fibrosis. Am J Dis Child 137:702–703

    PubMed  CAS  Google Scholar 

  23. Chan LL, Osmond DH, Balfe JW, Halperin ML (1981) Plasma "prorenin"-renin in Bartter's syndrome, cystic fibrosis, and chloride deficiency, and the effect of prostaglandin synthetase inhibition. J Lab Clin Med 97:785–790

    PubMed  CAS  Google Scholar 

  24. Coates AJ, Crofton PM, Marshall T (2009) Evaluation of salt supplementation in CF infants. J Cyst Fibros 8:382–385

    PubMed  CAS  Google Scholar 

  25. Cohen LF, di Sant'Agnese PA, Taylor A, Gill JR Jr (1977) The syndrome of inappropriate antidiuretic hormone secretion as a cause of hyponatremia in cystic fibrosis. J Pediatr 90:574–578

    PubMed  CAS  Google Scholar 

  26. Corbeel L, Proesmans W (1994) Dehydration associated with hypochloraemic alkalosis and cystic fibrosis. Eur J Pediatr 153:792

    Google Scholar 

  27. Davé S, Honney S, Raymond J, Flume PA (2005) An unusual presentation of cystic fibrosis in an adult. Am J Kidney Dis 45:e41–e44

    PubMed  Google Scholar 

  28. Davison AG, Snodgrass GJ (1983) Cystic fibrosis mimicking Bartter's syndrome. Acta Paediatr Scand 72:781–783

    PubMed  CAS  Google Scholar 

  29. Devlin J, Beckett NS, David TJ (1989) Elevated sweat potassium, hyperaldosteronism and pseudo-Bartter's syndrome: a spectrum of disorders associated with cystic fibrosis. J R Soc Med 82:38–43

    PubMed Central  PubMed  Google Scholar 

  30. Di Sant'Agnese PA, Darling RC, Perera GA, Shea E (1953) Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 12:549–563

    Google Scholar 

  31. Di Sant'Agnese PA (1960) Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. JAMA 172:2014–2021

    Google Scholar 

  32. Eigenmann P, Délèze G, Kuchler H (1991) Chronic metabolic alkalosis in an infant with cystic fibrosis. Eur J Pediatr 150:669–670

    PubMed  CAS  Google Scholar 

  33. English MC (1993) Clinical quiz. Pediatr Nephrol 7:119–120

    PubMed  CAS  Google Scholar 

  34. Epaud R, Girodon E, Corvol H, Niel F, Guigonis V, Clement A, Feldmann D, Bensman A, Ulinski T (2005) Mild cystic fibrosis revealed by persistent hyponatremia during the French 2003 heat wave, associated with the S1455X C-terminus CFTR mutation. Clin Genet 68:552–553

    PubMed  CAS  Google Scholar 

  35. Escobar Castro H, Medina E, Kirchschläger E, Camarero C, Suarez L (1995) Metabolic alkalosis with hypo-electrolytaemia or pseudo-Bartter syndrome as a presentation of cystic fibrosis in infancy. Description of three cases. Eur J Pediatr 154:868–869

    PubMed  CAS  Google Scholar 

  36. Forsyth JS, Gillies DR, Wilson SG (1982) Cystic fibrosis presenting with chronic electrolyte depletion, metabolic alkalosis and hyperaldosteronism. Scott Med J 27:333–335

    PubMed  CAS  Google Scholar 

  37. Fustik S, Pop-Jordanova N, Slaveska N, Koceva S, Efremov G (2002) Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis. Pediatr Int 44:289–292

    PubMed  Google Scholar 

  38. Geara AS, Parikh A, Rekhtman Y, Rao MK (2012) The Case - Metabolic alkalosis in a patient with cystic fibrosis. Kidney Int 81:421–422

    PubMed  Google Scholar 

  39. Gökçe S, Süoğlu OD, Celtik C, Aydoğan A, Sökücü S (2007) Cystic fibrosis and hypoelectrolytemia. Pediatr Emerg Care 23:760

    PubMed  Google Scholar 

  40. Gottlieb RP (1971) Metabolic alkalosis in cystic fibrosis. J Pediatr 79:930–936

    PubMed  CAS  Google Scholar 

  41. Greig F, Schoeneman MJ, Kandall SR, Bonforte RJ (1993) Neonatal hyponatremic dehydration as an initial presentation of cystic fibrosis. Clin Pediatr (Phila) 32:548–551

    CAS  Google Scholar 

  42. Guimarães EV, Schettino GC, Camargos PA, Penna FJ (2012) Prevalence of hyponatremia at diagnosis and factors associated with the longitudinal variation in serum sodium levels in infants with cystic fibrosis. J Pediatr 161:285–289

    PubMed  Google Scholar 

  43. Hochman HI, Feins NR, Rubin R, Gould J (1976) Chloride losing diarrhoea and metabolic alkalosis in an infant with cystic fibrosis. Arch Dis Child 51:390–391

    PubMed Central  PubMed  CAS  Google Scholar 

  44. Holland AE, Wilson JW, Kotsimbos TC, Naughton MT (2003) Metabolic alkalosis contributes to acute hypercapnic respiratory failure in adult cystic fibrosis. Chest 124:490–493

    PubMed  Google Scholar 

  45. Holland AE, Wilson JW, Kotsimbos TC, Naughton MT (2004) Metabolic alkalosis and cystic fibrosis. Chest 125:1169–1170

    Google Scholar 

  46. Hooman N, Jafari D, Jalali-Farahani S, Lahouti Harahdashti A (2012) An infant with alternating metabolic acidosis and alkalosis. Pediatr Nephrol 27:51–54

    PubMed  Google Scholar 

  47. Issa ARA, Teebi AS, Issa MA, Shaabani IS, Ramadan DJ (1988) Metabolic alkalosis in cystic fibrosis: atypical presentation in Kuwait. Ann Trop Paediatr 8:271–272

    PubMed  CAS  Google Scholar 

  48. Kabra SK, Kabra M, Lodha R, Shastri S, Ghosh M, Pandey RM, Kapil A, Aggarwal G, Kapoor V (2003) Clinical profile and frequency of delta f508 mutation in Indian children with cystic fibrosis. Indian Pediatr 40:612–619

    PubMed  CAS  Google Scholar 

  49. Kaskavage J, Sklansky D (2012) Hyponatremia-associated rhabdomyolysis following exercise in an adolescent with cystic fibrosis. Pediatrics 130:e220–e223

    PubMed  Google Scholar 

  50. Kennedy JD, Dinwiddie R, Daman-Willems C, Dillon MJ, Matthew DJ (1990) Pseudo-Bartter's syndrome in cystic fibrosis. Arch Dis Child 65:786–787

    PubMed Central  PubMed  CAS  Google Scholar 

  51. Khorasani E (2011) Case of Pseudo-Bartter’s Syndrome: an atypical presentation of cystic fibrosis. J Nepal Paediatr Soc 31:121–123

    Google Scholar 

  52. Kleta R, Brune T, Harms E (1999) Cystic fibrosis and metabolic alkalosis in children—revisited. Miner Electrolyte Metab 25:210

    PubMed  CAS  Google Scholar 

  53. Kose M, Pekcan S, Ozcelik U, Cobanoglu N, Yalcin E, Dogru D, Kiper N (2008) An epidemic of pseudo-Bartter syndrome in cystic fibrosis patients. Eur J Pediatr 167:115–116

    PubMed  Google Scholar 

  54. Kriemler S, Wilk B, Schurer W, Wilson WM, Bar-Or O (1999) Preventing dehydration in children with cystic fibrosis who exercise in the heat. Med Sci Sports Exerc 31:774–779

    PubMed  CAS  Google Scholar 

  55. Kurlandsky LE (2002) Failure to recognize the association of cystic fibrosis and metabolic alkalosis. Clin Pediatr (Phila) 41:715–719

    Google Scholar 

  56. Laughlin JL, Brady MS, Eigen H (1981) Changing feeding trends as a cause of electrolyte depletion in infants with cystic fibrosis. Pediatrics 68:203–207

    PubMed  CAS  Google Scholar 

  57. Legris GJ, Dearborn D, Stern RC, Geiss CL, Hopfer U, Douglas JG, Doershuk CF (1998) Sodium space and intravascular volume: dietary sodium effects in cystic fibrosis and healthy adolescent subjects. Pediatrics 101:48–56

    PubMed  CAS  Google Scholar 

  58. Leoni GB, Pitzalis S, Podda R, Zanda M, Silvetti M, Caocci L, Cao A, Rosatelli MC (1995) A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration. J Pediatr 127:281–283

    PubMed  CAS  Google Scholar 

  59. Lumpaopong A, Thirakhupt P, Srisuwan K, Chulamokha Y (2009) Rare F311L CFTR gene mutation in a child presented with recurrent electrolyte abnormalities and metabolic alkalosis: case report. J Med Assoc Thail 92:694–698

    Google Scholar 

  60. Marah MA (2010) Pseudo-Bartter as an initial presentation of cystic fibrosis. A case report and review of the literature. East Mediterr Health J 16:699–701

    PubMed  CAS  Google Scholar 

  61. Mathew PM, Hamdan JA, Nazer H (1991) Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis. Eur J Pediatr 150:264–266

    PubMed  CAS  Google Scholar 

  62. Mauri S, Pedroli G, Rüdeberg A, Laux-End R, Monotti R, Bianchetti MG (1997) Acute metabolic alkalosis in cystic fibrosis: prospective study and review of the literature. Miner Electrolyte Metab 23:33–37

    PubMed  CAS  Google Scholar 

  63. Muñoz AI, Rodríguez A, Jiménez JF (1983) Cystic fibrosis of the pancreas presenting as metabolic alkalosis. Bol Asoc Med P R 75:230–231

    PubMed  Google Scholar 

  64. Nahida e-R, Mohammed H, Guy L (2011) Pseudo-Bartter's syndrome revealing cystic fibrosis in an infant caused by 3849 + 1G>A and 4382delA compound heterozygosity. Acta Paediatr 100:e234–e235

    CAS  Google Scholar 

  65. Nussbaum E, Boat TF, Wood RE, Doershuk CF (1979) Cystic fibrosis with acute hypoelectrolytemia and metabolic alkalosis in infancy. Am J Dis Child 133:965–966

    PubMed  CAS  Google Scholar 

  66. Omron EM (2004) Metabolic alkalosis and cystic fibrosis. Chest 125:1169

    PubMed  Google Scholar 

  67. Özçelik U, Göçmen A, Kiper N, Coşkun T, Yilmaz E, Özgüç M (1994) Sodium chloride deficiency in cystic fibrosis patients. Eur J Pediatr 153:829–831

    PubMed  Google Scholar 

  68. Öztürk Y, Soylu OB, Arslan N (2006) Prevalence and clinical features of cystic fibrosis with pseudo-Bartter syndrome. Ann Trop Paediatr 26:155

    PubMed  Google Scholar 

  69. Pavone MA, Solís Padrones A, Muratore DG, Saiz M, Puig C (2010) Hyponatraemia, hypopotassaemia and pre-renal acute renal failure as a presentation of cystic fibrosis. Nefrologia 30:481–482

    PubMed  CAS  Google Scholar 

  70. Pedroli G, Liechti-Gallati S, Mauri S, Birrer P, Kraemer R, Foletti-Jäggi C, Bianchetti MG (1995) Chronic metabolic alkalosis: not uncommon in young children with severe cystic fibrosis. Am J Nephrol 15:245–250

    PubMed  CAS  Google Scholar 

  71. Priou-Guesdon M, Malinge MC, Augusto JF, Rodien P, Subra JF, Bonneau D, Rohmer V (2010) Hypochloremia and hyponatremia as the initial presentation of cystic fibrosis in three adults. Ann Endocrinol (Paris) 71:46–50

    CAS  Google Scholar 

  72. Rapaport R, Levine LS, Petrovic M, Wilson T, Draznin M, Bejar RL, Johanson A, New MI (1981) The renin–aldosterone system in cystic fibrosis. J Pediatr 98:768–771

    PubMed  CAS  Google Scholar 

  73. Rendle-Short J (1956) Fibrocystic disease of the pancreas presenting with acute salt depletion. Arch Dis Child 31:28–30

    PubMed Central  PubMed  CAS  Google Scholar 

  74. Rodríguez Portales JA, Delea CS (1986) Renal tubular reabsorption of chloride in Bartter's syndrome and other conditions with hypokalemia. Clin Nephrol 26:269–272

    PubMed  Google Scholar 

  75. Ruddy R, Anolik R, Scanlin TF (1981) Hypoelectrolytemia as a presentation and complication of cystic fibrosis. Clin Pediatr (Phila) 21:367–369

    Google Scholar 

  76. Salvatore D, Tomaiuolo R, Abate R, Vanacore B, Manieri S, Mirauda MP, Scavone A, Schiavo MV, Castaldo G, Salvatore F (2004) Cystic fibrosis presenting as metabolic alkalosis in a boy with the rare D579G mutation. J Cyst Fibros 3:135–136

    PubMed  Google Scholar 

  77. Schiffer M, Lynch R (1977) Inappropriate secretion of ADH as a cause of hyponatremia in cystic fibrosis. J Pediatr 91:850–851

    PubMed  CAS  Google Scholar 

  78. Sheth KJ, Heimler R (1977) Cystic fibrosis in an infant presenting with metabolic alkalosis. Wis Med J 76[Suppl]:47–49

    Google Scholar 

  79. Smith HR, Dhatt GS, Melia WM, Dickinson JG (1995) Cystic fibrosis presenting as hyponatraemic heat exhaustion. BMJ 310:579–580

    PubMed Central  PubMed  CAS  Google Scholar 

  80. Sovtić A, Minić P, Bogdanović R, Stajić N, Rodić M, Marković-Sovtić G (2012) Atypical presentation of cystic fibrosis—obese adolescent with hypertension and pseudo-Bartter's syndrome. Vojnosanit Pregl 69:367–369

    PubMed  Google Scholar 

  81. Stenvinkel P, Hjelte L, Alván G, Hedman A, Hultman E, Strandvik B (1991) Decreased renal clearance of sodium in cystic fibrosis. Acta Paediatr Scand 80:194–198

    PubMed  CAS  Google Scholar 

  82. Sweetser LJ, Douglas JA, Riha RL, Bell SC (2005) Clinical presentation of metabolic alkalosis in an adult patient with cystic fibrosis. Respirology 10:254–256

    PubMed  Google Scholar 

  83. Teeratakulpisarn J, Kosuwon P, Srinakarin J, Panthongviriyakul C, Sutra S (2006) Cystic fibrosis in three northeast Thai infants is CF really a rare disease in the Thai population? J Med Assoc Thail 89:1756–1761

    Google Scholar 

  84. Trauer JM, Wrobel JP, Young AC (2008) The syndrome of inappropriate anti-diuretic hormone secretion concurrent with an acute exacerbation of cystic fibrosis. J Cyst Fibros 7:573–575

    PubMed  CAS  Google Scholar 

  85. vande Velde S, Verloo P, Van Biervliet S, Robberecht E (2007) Heroin withdrawal leads to metabolic alkalosis in an infant with cystic fibrosis. Eur J Pediatr 166:75–76

    Google Scholar 

  86. Vertolli U, Ruffatti AM, De Giorgi ML, Scapin V, Naso A, Calò LA (2013) A very unusual case of hypokalaemia. Clin Kidney J 6:87–89

    Google Scholar 

  87. Wahab AA, Janahi IA, Marafia MM (2004) Pseudo-Bartter's syndrome in an Egyptian infant with cystic fibrosis mutation N1303K. J Trop Pediatr 50:242–244

    PubMed  Google Scholar 

  88. Wang MC, Shu SG, Chang SM, Ho WL, Chi CS (1993) Cystic fibrosis in two Chinese infants in Taiwan. Acta Paediatr Sin 34:314–321

    PubMed  CAS  Google Scholar 

  89. Williams AJ, McKiernan J, Harris F (1976) Heat prostration in children with cystic fibrosis. BMJ 273:297

    Google Scholar 

  90. Yalçin E, Kiper N, Doğru D, Ozçelik U, Aslan AT (2005) Clinical features and treatment approaches in cystic fibrosis with pseudo-Bartter syndrome. Ann Trop Paediatr 25:119–124

    PubMed  Google Scholar 

  91. Yalçın SS, Akça T, Genç Ö, Çelik M, Doğru D, Özçelik U (2007) Modified oral rehydration therapy in a case with cystic fibrosis. Turk J Pediatr 49:102–104

    PubMed  Google Scholar 

  92. Yiallouros PK, Neocleous V, Zeniou M, Adamidou T, Costi C, Christophi C, Tzetis M, Kanavakis E, Deltas C (2007) Cystic fibrosis mutational spectrum and genotypic/phenotypic features in Greek-Cypriots, with emphasis on dehydration as presenting symptom. Clin Genet 71:290–292

    PubMed  CAS  Google Scholar 

  93. Živanović S, Šaranac L, Kostić G (2008) The case of Pseudo-Bartter’s syndrome: an atypical presentation of cystic fibrosis. Med Biol 15:33–36

    Google Scholar 

  94. Dahabreh MM, Najada AS (2013) Pseudo-Bartter syndrome, pattern and correlation with other cystic fibrosis features. Saudi J Kidney Dis Transpl 24:292–296

    PubMed  Google Scholar 

  95. Aguirre I (2010) Fibrosis quística en el Perú. Neumol Pediatr 20:52

    Google Scholar 

  96. Aranzamendi RJ, Breitman F, Asciutto C, Delgado N, Castaños C (2008) Deshidratación con alcalosis hipoclorémica: presentación inusual de fibrosis quística en un lactante. Arch Argent Pediatr 106:443–446

    PubMed  Google Scholar 

  97. Campaňá-Cobas NG, Razón Behar R, Álvarez SD, Mañalich Coma R, Valdés Mesa M, Hernández Hernández JS (2008) Fibrosis quística que simula un síndrome de Bartter. Rev Cubana Pediatr 80. Available at: www.scielo.sld.cu/scielo.php

  98. Frontera Izquierdo P, Cabezuelo Huerta G (1980) Alcalosis metabólica en un lactante come primera manfestación clínica de mucoviscidosis. An Esp Pediatr 13:731–732

    PubMed  CAS  Google Scholar 

  99. Param T, Aldunate D (1988) Alcalosis metabólica en fibrosis quística del páncreas. Rev Chil Pediatr 59:322–325

    PubMed  CAS  Google Scholar 

  100. Saieh CA, Rodríguez JA, Paris EM, Fuentes AG, Letelier GG (1982) Alcalosis metabólica hipokalémica: importancia del cloro. Rev Chil Pediatr 53:233–236

    PubMed  CAS  Google Scholar 

  101. Götz M, Parth K, Singer P, Weissenbacher G (1976) Hypochlorämische Alkalose und sekundärer Hyperaldosteronismus bei Mukoviszidose. Pädiatr Pädol 11:275–282

    PubMed  Google Scholar 

  102. Sauter R, Will M, Helwig H (1997) Schwere Hyponatriämie als diagnoseweisendes Symptom der cystischen Fibrose. Klin Padiatr 209:361–363

    PubMed  CAS  Google Scholar 

  103. Weller F, Wiebicke W, Tümmler B (2000) Türkischer Säugling mit Hypoelektrolytämie und metabolischer Alkalose als alleinige Manifestation einer milden Form einer zystischen Fibrose (Mutation D110H). Klin Padiatr 212:41–43

    PubMed  CAS  Google Scholar 

  104. Bérard E, Maillotte AM, Albertini M, Delalandre E, Boutté P, Mariani R (1994) Mucoviscidose révélée par une déshydratation avec alcalose hypochloronatrémique chez trois nourrissons et un nouveau-né. Arch Pediatr 1:42–45

    PubMed  Google Scholar 

  105. Desmazes-Dufeu N, Hubert D, Burgel PR, Kanaan R, Vélea V, Dusser D (2005) Déshydratation sévère, consequence de la canicule d’août 2003 sur une cohorte d’adultes atteints de mucoviscidose. Presse Med 34:647–648

    PubMed  Google Scholar 

  106. Sanfelice NFT, Zucchi L (1998) Síndrome de Bartter: relato de dois casos em crianças. J Pediatr (Rio J) 74:473–478

    Google Scholar 

  107. Santos GPC, Cecon MR, Riedi CA, Dias LM, Braga MC, Rosário NA (2007) Alcalose hipoclorêmica em pacientes com fibrose cística—relato de 6 casos. J Paran Pediatr 8:43–44

    Google Scholar 

  108. Luke RG, Galla JH (2012) It is chloride depletion alkalosis, not contraction alkalosis. J Am Soc Nephrol 23:204–207

    PubMed Central  PubMed  CAS  Google Scholar 

  109. Peruzzo M, Milani GP, Garzoni L, Longoni L, Simonetti GD, Bettinelli A, Fossali EF, Bianchetti MG (2010) Body fluids and salt metabolism—part II. Ital J Pediatr 36:78

    PubMed Central  PubMed  Google Scholar 

  110. Royer P (1975) Métabolisme du sodium et développement prénatal et postnatal. Arch Fr Pediatr 32:497–502

    PubMed  CAS  Google Scholar 

  111. Miozzari HH, Tönz M, von Vigier RO, Bianchetti MG (2001) Fluid resuscitation in infantile hypertrophic pyloric stenosis. Acta Paediatr 90:511–514

    PubMed  CAS  Google Scholar 

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The authors are very grateful to Mrs. Mary Coduri for linguistic consultation.

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Authors and Affiliations

  1. Pediatric Emergency Unit, De Marchi Hospital, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Elisabetta Scurati-Manzoni & Emilio F. Fossali

  2. Integrated Department of Pediatrics, Ente Ospedaliero Cantonale Ticinese, University of Berne, Bern, Switzerland

    Elisabetta Scurati-Manzoni, Maura Zanolari-Calderari, Mario G. Bianchetti & Sebastiano A. G. Lava

  3. Second Pediatric Clinic, Clinica De Marchi, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy

    Carlo Agostoni

  4. Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy

    Enrica Riva

  5. Pediatric Nephrology, University Children’s Hospital Berne, University of Berne, Bern, Switzerland

    Giacomo D. Simonetti & Sebastiano A. G. Lava

  6. San Giovanni Hospital, 6500, Bellinzona, Switzerland

    Mario G. Bianchetti

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  1. Elisabetta Scurati-Manzoni
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Correspondence to Mario G. Bianchetti.

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Scurati-Manzoni, E., Fossali, E.F., Agostoni, C. et al. Electrolyte abnormalities in cystic fibrosis: systematic review of the literature. Pediatr Nephrol 29, 1015–1023 (2014). https://doi.org/10.1007/s00467-013-2712-4

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  • DOI: https://doi.org/10.1007/s00467-013-2712-4

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