Skip to main content
Top
Published in: Pediatric Nephrology 6/2014

01-06-2014 | Original Article

Electrolyte abnormalities in cystic fibrosis: systematic review of the literature

Authors: Elisabetta Scurati-Manzoni, Emilio F. Fossali, Carlo Agostoni, Enrica Riva, Giacomo D. Simonetti, Maura Zanolari-Calderari, Mario G. Bianchetti, Sebastiano A. G. Lava

Published in: Pediatric Nephrology | Issue 6/2014

Login to get access

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.
Literature
1.
go back to reference Liamis G, Milionis H, Elisaf M (2008) A review of drug-induced hyponatremia. Am J Kidney Dis 52:144–153PubMed Liamis G, Milionis H, Elisaf M (2008) A review of drug-induced hyponatremia. Am J Kidney Dis 52:144–153PubMed
2.
go back to reference Greenlee M, Wingo CS, McDonough AA, Youn JH, Kone BC (2009) Narrative review: evolving concepts in potassium homeostasis and hypokalemia. Ann Intern Med 150:619–625PubMed Greenlee M, Wingo CS, McDonough AA, Youn JH, Kone BC (2009) Narrative review: evolving concepts in potassium homeostasis and hypokalemia. Ann Intern Med 150:619–625PubMed
3.
go back to reference Kerem E (2006) Atypical CF and CF related diseases. Paediatr Respir Rev 7[Suppl 1]:S144–S146PubMed Kerem E (2006) Atypical CF and CF related diseases. Paediatr Respir Rev 7[Suppl 1]:S144–S146PubMed
4.
go back to reference Kessler WR, Andersen DH (1951) Heat prostration in fibrocystic disease of the pancreas and other conditions. Pediatrics 8:648–656PubMed Kessler WR, Andersen DH (1951) Heat prostration in fibrocystic disease of the pancreas and other conditions. Pediatrics 8:648–656PubMed
5.
go back to reference 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 151:264–269PubMed 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 151:264–269PubMed
6.
go back to reference Anonymous (1964) Convulsions after therapy for alveolar hypoventilation. JAMA 189:1023 Anonymous (1964) Convulsions after therapy for alveolar hypoventilation. JAMA 189:1023
7.
go back to reference 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 153:825–828PubMed 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 153:825–828PubMed
8.
go back to reference Beckerman RC, Taussig LM (1979) Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. Pediatrics 63:580–583PubMed Beckerman RC, Taussig LM (1979) Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. Pediatrics 63:580–583PubMed
9.
go back to reference O'Sullivan BP, Freedman SD (2009) Cystic fibrosis. Lancet 373:1891–1904PubMed O'Sullivan BP, Freedman SD (2009) Cystic fibrosis. Lancet 373:1891–1904PubMed
10.
go back to reference 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 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
11.
go back to reference Al-Ghimlas F, Faughnan ME, Tullis E (2012) Metabolic alkalosis in adults with stable cystic fibrosis. Open Respir Med J 6:59–62PubMedCentralPubMed Al-Ghimlas F, Faughnan ME, Tullis E (2012) Metabolic alkalosis in adults with stable cystic fibrosis. Open Respir Med J 6:59–62PubMedCentralPubMed
12.
go back to reference Arvanitakis SN, Lobeck CC (1973) Metabolic alkalosis and salt depletion in cystic fibrosis. J Pediatr 82:535–545PubMed Arvanitakis SN, Lobeck CC (1973) Metabolic alkalosis and salt depletion in cystic fibrosis. J Pediatr 82:535–545PubMed
13.
go back to reference 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 69:302–305PubMed 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 69:302–305PubMed
14.
go back to reference Baird JS, Walker P, Urban A, Berdella M (2002) Metabolic alkalosis and cystic fibrosis. Chest 122:755–756PubMed Baird JS, Walker P, Urban A, Berdella M (2002) Metabolic alkalosis and cystic fibrosis. Chest 122:755–756PubMed
15.
go back to reference 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 22:725–727PubMed 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 22:725–727PubMed
16.
go back to reference Bar-Or O, Blimkie CJ, Hay JA, MacDougall JD, Ward DS, Wilson WM (1992) Voluntary dehydration and heat intolerance in cystic fibrosis. Lancet 339:696–699PubMed Bar-Or O, Blimkie CJ, Hay JA, MacDougall JD, Ward DS, Wilson WM (1992) Voluntary dehydration and heat intolerance in cystic fibrosis. Lancet 339:696–699PubMed
17.
go back to reference Bates CM, Baum M, Quigley R (1997) Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent. J Am Soc Nephrol 8:352–355PubMed Bates CM, Baum M, Quigley R (1997) Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent. J Am Soc Nephrol 8:352–355PubMed
18.
go back to reference Beckerman RC (1979) Metabolic alkalosis in infants with cystic fibrosis. Pediatrics 64:389PubMed Beckerman RC (1979) Metabolic alkalosis in infants with cystic fibrosis. Pediatrics 64:389PubMed
19.
go back to reference 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 5:189–190PubMed 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 5:189–190PubMed
20.
go back to reference Bianchetti MG, Mauri S (1996) Hypotonic dehydration in cystic fibrosis: mild or severe disease phenotype? J Pediatr 128:723PubMed Bianchetti MG, Mauri S (1996) Hypotonic dehydration in cystic fibrosis: mild or severe disease phenotype? J Pediatr 128:723PubMed
21.
go back to reference Bijman J (1987) Transport processes in the eccrine sweat gland. Kidney Int Suppl 21:S109–S112PubMed Bijman J (1987) Transport processes in the eccrine sweat gland. Kidney Int Suppl 21:S109–S112PubMed
22.
go back to reference Carlile JR, Pafford DM, Waring WW (1983) Severe hypernatremia in cystic fibrosis. Am J Dis Child 137:702–703PubMed Carlile JR, Pafford DM, Waring WW (1983) Severe hypernatremia in cystic fibrosis. Am J Dis Child 137:702–703PubMed
23.
go back to reference 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 97:785–790PubMed 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 97:785–790PubMed
24.
go back to reference Coates AJ, Crofton PM, Marshall T (2009) Evaluation of salt supplementation in CF infants. J Cyst Fibros 8:382–385PubMed Coates AJ, Crofton PM, Marshall T (2009) Evaluation of salt supplementation in CF infants. J Cyst Fibros 8:382–385PubMed
25.
go back to reference 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 90:574–578PubMed 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 90:574–578PubMed
26.
go back to reference Corbeel L, Proesmans W (1994) Dehydration associated with hypochloraemic alkalosis and cystic fibrosis. Eur J Pediatr 153:792 Corbeel L, Proesmans W (1994) Dehydration associated with hypochloraemic alkalosis and cystic fibrosis. Eur J Pediatr 153:792
27.
go back to reference Davé S, Honney S, Raymond J, Flume PA (2005) An unusual presentation of cystic fibrosis in an adult. Am J Kidney Dis 45:e41–e44PubMed Davé S, Honney S, Raymond J, Flume PA (2005) An unusual presentation of cystic fibrosis in an adult. Am J Kidney Dis 45:e41–e44PubMed
28.
go back to reference Davison AG, Snodgrass GJ (1983) Cystic fibrosis mimicking Bartter's syndrome. Acta Paediatr Scand 72:781–783PubMed Davison AG, Snodgrass GJ (1983) Cystic fibrosis mimicking Bartter's syndrome. Acta Paediatr Scand 72:781–783PubMed
29.
go back to reference 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 82:38–43PubMedCentralPubMed 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 82:38–43PubMedCentralPubMed
30.
go back to reference 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 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
31.
go back to reference Di Sant'Agnese PA (1960) Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. JAMA 172:2014–2021 Di Sant'Agnese PA (1960) Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. JAMA 172:2014–2021
32.
go back to reference Eigenmann P, Délèze G, Kuchler H (1991) Chronic metabolic alkalosis in an infant with cystic fibrosis. Eur J Pediatr 150:669–670PubMed Eigenmann P, Délèze G, Kuchler H (1991) Chronic metabolic alkalosis in an infant with cystic fibrosis. Eur J Pediatr 150:669–670PubMed
33.
34.
go back to reference 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 68:552–553PubMed 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 68:552–553PubMed
35.
go back to reference 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 154:868–869PubMed 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 154:868–869PubMed
36.
go back to reference Forsyth JS, Gillies DR, Wilson SG (1982) Cystic fibrosis presenting with chronic electrolyte depletion, metabolic alkalosis and hyperaldosteronism. Scott Med J 27:333–335PubMed Forsyth JS, Gillies DR, Wilson SG (1982) Cystic fibrosis presenting with chronic electrolyte depletion, metabolic alkalosis and hyperaldosteronism. Scott Med J 27:333–335PubMed
37.
go back to reference Fustik S, Pop-Jordanova N, Slaveska N, Koceva S, Efremov G (2002) Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis. Pediatr Int 44:289–292PubMed Fustik S, Pop-Jordanova N, Slaveska N, Koceva S, Efremov G (2002) Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis. Pediatr Int 44:289–292PubMed
38.
go back to reference Geara AS, Parikh A, Rekhtman Y, Rao MK (2012) The Case - Metabolic alkalosis in a patient with cystic fibrosis. Kidney Int 81:421–422PubMed Geara AS, Parikh A, Rekhtman Y, Rao MK (2012) The Case - Metabolic alkalosis in a patient with cystic fibrosis. Kidney Int 81:421–422PubMed
39.
go back to reference Gökçe S, Süoğlu OD, Celtik C, Aydoğan A, Sökücü S (2007) Cystic fibrosis and hypoelectrolytemia. Pediatr Emerg Care 23:760PubMed Gökçe S, Süoğlu OD, Celtik C, Aydoğan A, Sökücü S (2007) Cystic fibrosis and hypoelectrolytemia. Pediatr Emerg Care 23:760PubMed
40.
go back to reference Gottlieb RP (1971) Metabolic alkalosis in cystic fibrosis. J Pediatr 79:930–936PubMed Gottlieb RP (1971) Metabolic alkalosis in cystic fibrosis. J Pediatr 79:930–936PubMed
41.
go back to reference 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 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
42.
go back to reference 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 161:285–289PubMed 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 161:285–289PubMed
43.
go back to reference Hochman HI, Feins NR, Rubin R, Gould J (1976) Chloride losing diarrhoea and metabolic alkalosis in an infant with cystic fibrosis. Arch Dis Child 51:390–391PubMedCentralPubMed Hochman HI, Feins NR, Rubin R, Gould J (1976) Chloride losing diarrhoea and metabolic alkalosis in an infant with cystic fibrosis. Arch Dis Child 51:390–391PubMedCentralPubMed
44.
go back to reference Holland AE, Wilson JW, Kotsimbos TC, Naughton MT (2003) Metabolic alkalosis contributes to acute hypercapnic respiratory failure in adult cystic fibrosis. Chest 124:490–493PubMed Holland AE, Wilson JW, Kotsimbos TC, Naughton MT (2003) Metabolic alkalosis contributes to acute hypercapnic respiratory failure in adult cystic fibrosis. Chest 124:490–493PubMed
45.
go back to reference Holland AE, Wilson JW, Kotsimbos TC, Naughton MT (2004) Metabolic alkalosis and cystic fibrosis. Chest 125:1169–1170 Holland AE, Wilson JW, Kotsimbos TC, Naughton MT (2004) Metabolic alkalosis and cystic fibrosis. Chest 125:1169–1170
46.
go back to reference Hooman N, Jafari D, Jalali-Farahani S, Lahouti Harahdashti A (2012) An infant with alternating metabolic acidosis and alkalosis. Pediatr Nephrol 27:51–54PubMed Hooman N, Jafari D, Jalali-Farahani S, Lahouti Harahdashti A (2012) An infant with alternating metabolic acidosis and alkalosis. Pediatr Nephrol 27:51–54PubMed
47.
go back to reference Issa ARA, Teebi AS, Issa MA, Shaabani IS, Ramadan DJ (1988) Metabolic alkalosis in cystic fibrosis: atypical presentation in Kuwait. Ann Trop Paediatr 8:271–272PubMed Issa ARA, Teebi AS, Issa MA, Shaabani IS, Ramadan DJ (1988) Metabolic alkalosis in cystic fibrosis: atypical presentation in Kuwait. Ann Trop Paediatr 8:271–272PubMed
48.
go back to reference 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 40:612–619PubMed 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 40:612–619PubMed
49.
go back to reference Kaskavage J, Sklansky D (2012) Hyponatremia-associated rhabdomyolysis following exercise in an adolescent with cystic fibrosis. Pediatrics 130:e220–e223PubMed Kaskavage J, Sklansky D (2012) Hyponatremia-associated rhabdomyolysis following exercise in an adolescent with cystic fibrosis. Pediatrics 130:e220–e223PubMed
50.
go back to reference Kennedy JD, Dinwiddie R, Daman-Willems C, Dillon MJ, Matthew DJ (1990) Pseudo-Bartter's syndrome in cystic fibrosis. Arch Dis Child 65:786–787PubMedCentralPubMed Kennedy JD, Dinwiddie R, Daman-Willems C, Dillon MJ, Matthew DJ (1990) Pseudo-Bartter's syndrome in cystic fibrosis. Arch Dis Child 65:786–787PubMedCentralPubMed
51.
go back to reference Khorasani E (2011) Case of Pseudo-Bartter’s Syndrome: an atypical presentation of cystic fibrosis. J Nepal Paediatr Soc 31:121–123 Khorasani E (2011) Case of Pseudo-Bartter’s Syndrome: an atypical presentation of cystic fibrosis. J Nepal Paediatr Soc 31:121–123
52.
go back to reference Kleta R, Brune T, Harms E (1999) Cystic fibrosis and metabolic alkalosis in children—revisited. Miner Electrolyte Metab 25:210PubMed Kleta R, Brune T, Harms E (1999) Cystic fibrosis and metabolic alkalosis in children—revisited. Miner Electrolyte Metab 25:210PubMed
53.
go back to reference 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 167:115–116PubMed 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 167:115–116PubMed
54.
go back to reference 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 31:774–779PubMed 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 31:774–779PubMed
55.
go back to reference Kurlandsky LE (2002) Failure to recognize the association of cystic fibrosis and metabolic alkalosis. Clin Pediatr (Phila) 41:715–719 Kurlandsky LE (2002) Failure to recognize the association of cystic fibrosis and metabolic alkalosis. Clin Pediatr (Phila) 41:715–719
56.
go back to reference Laughlin JL, Brady MS, Eigen H (1981) Changing feeding trends as a cause of electrolyte depletion in infants with cystic fibrosis. Pediatrics 68:203–207PubMed Laughlin JL, Brady MS, Eigen H (1981) Changing feeding trends as a cause of electrolyte depletion in infants with cystic fibrosis. Pediatrics 68:203–207PubMed
57.
go back to reference 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 101:48–56PubMed 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 101:48–56PubMed
58.
go back to reference 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 127:281–283PubMed 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 127:281–283PubMed
59.
go back to reference 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 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
60.
go back to reference Marah MA (2010) Pseudo-Bartter as an initial presentation of cystic fibrosis. A case report and review of the literature. East Mediterr Health J 16:699–701PubMed Marah MA (2010) Pseudo-Bartter as an initial presentation of cystic fibrosis. A case report and review of the literature. East Mediterr Health J 16:699–701PubMed
61.
go back to reference Mathew PM, Hamdan JA, Nazer H (1991) Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis. Eur J Pediatr 150:264–266PubMed Mathew PM, Hamdan JA, Nazer H (1991) Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis. Eur J Pediatr 150:264–266PubMed
62.
go back to reference 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 23:33–37PubMed 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 23:33–37PubMed
63.
go back to reference Muñoz AI, Rodríguez A, Jiménez JF (1983) Cystic fibrosis of the pancreas presenting as metabolic alkalosis. Bol Asoc Med P R 75:230–231PubMed Muñoz AI, Rodríguez A, Jiménez JF (1983) Cystic fibrosis of the pancreas presenting as metabolic alkalosis. Bol Asoc Med P R 75:230–231PubMed
64.
go back to reference 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 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
65.
go back to reference Nussbaum E, Boat TF, Wood RE, Doershuk CF (1979) Cystic fibrosis with acute hypoelectrolytemia and metabolic alkalosis in infancy. Am J Dis Child 133:965–966PubMed Nussbaum E, Boat TF, Wood RE, Doershuk CF (1979) Cystic fibrosis with acute hypoelectrolytemia and metabolic alkalosis in infancy. Am J Dis Child 133:965–966PubMed
66.
go back to reference Omron EM (2004) Metabolic alkalosis and cystic fibrosis. Chest 125:1169PubMed Omron EM (2004) Metabolic alkalosis and cystic fibrosis. Chest 125:1169PubMed
67.
go back to reference Özçelik U, Göçmen A, Kiper N, Coşkun T, Yilmaz E, Özgüç M (1994) Sodium chloride deficiency in cystic fibrosis patients. Eur J Pediatr 153:829–831PubMed Özçelik U, Göçmen A, Kiper N, Coşkun T, Yilmaz E, Özgüç M (1994) Sodium chloride deficiency in cystic fibrosis patients. Eur J Pediatr 153:829–831PubMed
68.
go back to reference Öztürk Y, Soylu OB, Arslan N (2006) Prevalence and clinical features of cystic fibrosis with pseudo-Bartter syndrome. Ann Trop Paediatr 26:155PubMed Öztürk Y, Soylu OB, Arslan N (2006) Prevalence and clinical features of cystic fibrosis with pseudo-Bartter syndrome. Ann Trop Paediatr 26:155PubMed
69.
go back to reference 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 30:481–482PubMed 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 30:481–482PubMed
70.
go back to reference 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 15:245–250PubMed 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 15:245–250PubMed
71.
go back to reference 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 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
72.
go back to reference 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 98:768–771PubMed 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 98:768–771PubMed
73.
go back to reference Rendle-Short J (1956) Fibrocystic disease of the pancreas presenting with acute salt depletion. Arch Dis Child 31:28–30PubMedCentralPubMed Rendle-Short J (1956) Fibrocystic disease of the pancreas presenting with acute salt depletion. Arch Dis Child 31:28–30PubMedCentralPubMed
74.
go back to reference Rodríguez Portales JA, Delea CS (1986) Renal tubular reabsorption of chloride in Bartter's syndrome and other conditions with hypokalemia. Clin Nephrol 26:269–272PubMed Rodríguez Portales JA, Delea CS (1986) Renal tubular reabsorption of chloride in Bartter's syndrome and other conditions with hypokalemia. Clin Nephrol 26:269–272PubMed
75.
go back to reference Ruddy R, Anolik R, Scanlin TF (1981) Hypoelectrolytemia as a presentation and complication of cystic fibrosis. Clin Pediatr (Phila) 21:367–369 Ruddy R, Anolik R, Scanlin TF (1981) Hypoelectrolytemia as a presentation and complication of cystic fibrosis. Clin Pediatr (Phila) 21:367–369
76.
go back to reference 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 3:135–136PubMed 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 3:135–136PubMed
77.
go back to reference Schiffer M, Lynch R (1977) Inappropriate secretion of ADH as a cause of hyponatremia in cystic fibrosis. J Pediatr 91:850–851PubMed Schiffer M, Lynch R (1977) Inappropriate secretion of ADH as a cause of hyponatremia in cystic fibrosis. J Pediatr 91:850–851PubMed
78.
go back to reference Sheth KJ, Heimler R (1977) Cystic fibrosis in an infant presenting with metabolic alkalosis. Wis Med J 76[Suppl]:47–49 Sheth KJ, Heimler R (1977) Cystic fibrosis in an infant presenting with metabolic alkalosis. Wis Med J 76[Suppl]:47–49
79.
go back to reference Smith HR, Dhatt GS, Melia WM, Dickinson JG (1995) Cystic fibrosis presenting as hyponatraemic heat exhaustion. BMJ 310:579–580PubMedCentralPubMed Smith HR, Dhatt GS, Melia WM, Dickinson JG (1995) Cystic fibrosis presenting as hyponatraemic heat exhaustion. BMJ 310:579–580PubMedCentralPubMed
80.
go back to reference 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 69:367–369PubMed 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 69:367–369PubMed
81.
go back to reference Stenvinkel P, Hjelte L, Alván G, Hedman A, Hultman E, Strandvik B (1991) Decreased renal clearance of sodium in cystic fibrosis. Acta Paediatr Scand 80:194–198PubMed Stenvinkel P, Hjelte L, Alván G, Hedman A, Hultman E, Strandvik B (1991) Decreased renal clearance of sodium in cystic fibrosis. Acta Paediatr Scand 80:194–198PubMed
82.
go back to reference Sweetser LJ, Douglas JA, Riha RL, Bell SC (2005) Clinical presentation of metabolic alkalosis in an adult patient with cystic fibrosis. Respirology 10:254–256PubMed Sweetser LJ, Douglas JA, Riha RL, Bell SC (2005) Clinical presentation of metabolic alkalosis in an adult patient with cystic fibrosis. Respirology 10:254–256PubMed
83.
go back to reference 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 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
84.
go back to reference 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 7:573–575PubMed 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 7:573–575PubMed
85.
go back to reference 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 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
86.
go back to reference 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 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
87.
go back to reference Wahab AA, Janahi IA, Marafia MM (2004) Pseudo-Bartter's syndrome in an Egyptian infant with cystic fibrosis mutation N1303K. J Trop Pediatr 50:242–244PubMed Wahab AA, Janahi IA, Marafia MM (2004) Pseudo-Bartter's syndrome in an Egyptian infant with cystic fibrosis mutation N1303K. J Trop Pediatr 50:242–244PubMed
88.
go back to reference Wang MC, Shu SG, Chang SM, Ho WL, Chi CS (1993) Cystic fibrosis in two Chinese infants in Taiwan. Acta Paediatr Sin 34:314–321PubMed Wang MC, Shu SG, Chang SM, Ho WL, Chi CS (1993) Cystic fibrosis in two Chinese infants in Taiwan. Acta Paediatr Sin 34:314–321PubMed
89.
go back to reference Williams AJ, McKiernan J, Harris F (1976) Heat prostration in children with cystic fibrosis. BMJ 273:297 Williams AJ, McKiernan J, Harris F (1976) Heat prostration in children with cystic fibrosis. BMJ 273:297
90.
go back to reference 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 25:119–124PubMed 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 25:119–124PubMed
91.
go back to reference 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 49:102–104PubMed 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 49:102–104PubMed
92.
go back to reference 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 71:290–292PubMed 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 71:290–292PubMed
93.
go back to reference 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 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
94.
go back to reference Dahabreh MM, Najada AS (2013) Pseudo-Bartter syndrome, pattern and correlation with other cystic fibrosis features. Saudi J Kidney Dis Transpl 24:292–296PubMed Dahabreh MM, Najada AS (2013) Pseudo-Bartter syndrome, pattern and correlation with other cystic fibrosis features. Saudi J Kidney Dis Transpl 24:292–296PubMed
95.
go back to reference Aguirre I (2010) Fibrosis quística en el Perú. Neumol Pediatr 20:52 Aguirre I (2010) Fibrosis quística en el Perú. Neumol Pediatr 20:52
96.
go back to reference 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 106:443–446PubMed 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 106:443–446PubMed
97.
go back to reference 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 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.
go back to reference Frontera Izquierdo P, Cabezuelo Huerta G (1980) Alcalosis metabólica en un lactante come primera manfestación clínica de mucoviscidosis. An Esp Pediatr 13:731–732PubMed Frontera Izquierdo P, Cabezuelo Huerta G (1980) Alcalosis metabólica en un lactante come primera manfestación clínica de mucoviscidosis. An Esp Pediatr 13:731–732PubMed
99.
go back to reference Param T, Aldunate D (1988) Alcalosis metabólica en fibrosis quística del páncreas. Rev Chil Pediatr 59:322–325PubMed Param T, Aldunate D (1988) Alcalosis metabólica en fibrosis quística del páncreas. Rev Chil Pediatr 59:322–325PubMed
100.
go back to reference Saieh CA, Rodríguez JA, Paris EM, Fuentes AG, Letelier GG (1982) Alcalosis metabólica hipokalémica: importancia del cloro. Rev Chil Pediatr 53:233–236PubMed Saieh CA, Rodríguez JA, Paris EM, Fuentes AG, Letelier GG (1982) Alcalosis metabólica hipokalémica: importancia del cloro. Rev Chil Pediatr 53:233–236PubMed
101.
go back to reference Götz M, Parth K, Singer P, Weissenbacher G (1976) Hypochlorämische Alkalose und sekundärer Hyperaldosteronismus bei Mukoviszidose. Pädiatr Pädol 11:275–282PubMed Götz M, Parth K, Singer P, Weissenbacher G (1976) Hypochlorämische Alkalose und sekundärer Hyperaldosteronismus bei Mukoviszidose. Pädiatr Pädol 11:275–282PubMed
102.
go back to reference Sauter R, Will M, Helwig H (1997) Schwere Hyponatriämie als diagnoseweisendes Symptom der cystischen Fibrose. Klin Padiatr 209:361–363PubMed Sauter R, Will M, Helwig H (1997) Schwere Hyponatriämie als diagnoseweisendes Symptom der cystischen Fibrose. Klin Padiatr 209:361–363PubMed
103.
go back to reference 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 212:41–43PubMed 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 212:41–43PubMed
104.
go back to reference 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 1:42–45PubMed 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 1:42–45PubMed
105.
go back to reference 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 34:647–648PubMed 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 34:647–648PubMed
106.
go back to reference Sanfelice NFT, Zucchi L (1998) Síndrome de Bartter: relato de dois casos em crianças. J Pediatr (Rio J) 74:473–478 Sanfelice NFT, Zucchi L (1998) Síndrome de Bartter: relato de dois casos em crianças. J Pediatr (Rio J) 74:473–478
107.
go back to reference 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 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
108.
109.
go back to reference 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 36:78PubMedCentralPubMed 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 36:78PubMedCentralPubMed
110.
go back to reference Royer P (1975) Métabolisme du sodium et développement prénatal et postnatal. Arch Fr Pediatr 32:497–502PubMed Royer P (1975) Métabolisme du sodium et développement prénatal et postnatal. Arch Fr Pediatr 32:497–502PubMed
111.
go back to reference Miozzari HH, Tönz M, von Vigier RO, Bianchetti MG (2001) Fluid resuscitation in infantile hypertrophic pyloric stenosis. Acta Paediatr 90:511–514PubMed Miozzari HH, Tönz M, von Vigier RO, Bianchetti MG (2001) Fluid resuscitation in infantile hypertrophic pyloric stenosis. Acta Paediatr 90:511–514PubMed
Metadata
Title
Electrolyte abnormalities in cystic fibrosis: systematic review of the literature
Authors
Elisabetta Scurati-Manzoni
Emilio F. Fossali
Carlo Agostoni
Enrica Riva
Giacomo D. Simonetti
Maura Zanolari-Calderari
Mario G. Bianchetti
Sebastiano A. G. Lava
Publication date
01-06-2014
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 6/2014
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-013-2712-4

Other articles of this Issue 6/2014

Pediatric Nephrology 6/2014 Go to the issue