Cystic Fibrosis and Hypoelectrolytemia with Metabolic Alkalosis
By:
Michael Emmett Department of Medicine, Baylor University Medical Center, Dallas, Texas

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  • 1.

    Quinton PM: Physiological basis of cystic fibrosis: A historical perspective. Physiol Rev 79[Suppl]: S3S22, 1999 PubMed

  • 2.

    Marx JL. The CF gene hits the news. Science 245: 924, 1989 PubMed

  • 3.

    Kessler WR, Andersen DH: Heat prostration in fibrocystic disease of the pancreas and other conditions. Pediatrics 8: 648656, 1951 PubMed

  • 4.

    Darling RC, Disant’Agnese PA, Perera GA, Andersen DH: Electrolyte abnormalities of the sweat in fibrocystic disease of the pancreas. Am J Med Sci 225: 6770, 1953 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5.

    Di Sant’Agnese PA, Darling RC, Perera GA, Shea E: Sweat electrolyte disturbances associated with childhood pancreatic disease. Am J Med 15: 777784, 1953 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6.

    Rendle-Short J; Archives Disease of Childhood: Fibrocystic disease of the pancreas presenting with acute salt depletion. Arch Dis Child 31: 2830, 1956 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7.

    Di Sant’Agnese PA: Salt depletion in cold weather in infants with cystic fibrosis of the pancreas. JAMA 172: 20142021, 1960 PubMed

  • 8.

    Gottlieb RP: Metabolic alkalosis in cystic fibrosis. J Pediatr 79: 930936, 1971 PubMed

  • 9.

    Scurati-Manzoni E, Fossali EF, Agostoni C, Riva E, Simonetti GD, Zanolari-Calderari M, et al.: Electrolyte abnormalities in cystic fibrosis: Systematic review of the literature. Pediatr Nephrol 29: 10151023, 2014 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10.

    Terlizzi V, Padoan R, Leonetti G, Vitullo P, Tosco A, Taccetti G, et al.: Cystic fibrosis and CFTR-related disorder with electrolyte imbalance at diagnosis: clinical features and outcome in an Italian cohort. Eur J Pediatr 182: 52755283, 2023 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11.

    Sismanlar Eyuboglu T, Dogru D, Çakır E, Cobanoglu N, Pekcan S, Cinel G, et al.: Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis. Pediatr Pulmonol 55: 20112016, 2020 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12.

    Smith HR, Dhatt GS, Melia WM, Dickinson JG: Cystic fibrosis presenting as hyponatraemic heat exhaustion. BMJ 310: 579580, 1995 PubMed

  • 13.

    Bates CM, Baum M, Quigley R: Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent. J Am Soc Nephrol 8: 352355, 1997 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14.

    Baird JS, Walker P, Urban A, Berdella M: Metabolic alkalosis and cystic fibrosis. Chest 122: 755756, 2002 PubMed

  • 15.

    Davé S, Honney S, Raymond J, Flume PA: An unusual presentation of cystic fibrosis in an adult. Am J Kidney Dis 45: e41e44, 2005 PubMed

  • 16.

    Sweetser LJ, Douglas JA, Riha RL, Bell SC: Clinical presentation of metabolic alkalosis in an adult patient with cystic fibrosis. Respirology 10: 254256, 2005 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17.

    Augusto JF, Sayegh J, Malinge MC, Illouz F, Subra JF, Ducluzeau PH: Severe episodes of extra cellular dehydration: An atypical adult presentation of cystic fibrosis. Clin Nephrol 69: 302305, 2008 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18.

    Priou-Guesdon M, Malinge MC, Augusto JF, Rodien P, Subra JF, Bonneau D, et al.: Hypochloremia and hyponatremia as the initial presentation of cystic fibrosis in three adults. Ann Endocrinol (Paris) 71: 4650, 2010 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19.

    Pavone MA, Solís Padrones A, Muratore DG, Saiz M, Puig C: Hiponatremia, hipopotasemia e insuficiencia renal aguda prerrenal como presentación de fibrosis quística. [Hyponatraemia, hypopotassaemia and prerenal acute renal failure as a presentation of cystic fibrosis] Nefrologia 30: 481482, 2010 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20.

    Vertolli U, Ruffatti A, Loreta De Giorgi M, Scapin V, Naso A, Calò LA: A very unusual case of hypokalaemia. Clin Kidney J 6: 8789, 2013 PubMed

  • 21.

    Raya CM, Zubillaga IP, Schneider P: Alcalosis metabólica con hiponatremia, hipopotasemia e hipocloremia como forma de presentación de fibrosis quística en un adulto. [Metabolic alkalosis with hyponatremia, hypokalemia and hypochloremia as the initial presentation of cystic fibrosis in an adult patient] Med Clin (Barc) 143: 137138, 2014 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22.

    Cao Y, Donaldson R, Lee D: “Summer hypokalemia” as an initial presentation of cystic fibrosis in a morbidly obese African American adult: Case report. BMC Nephrol 21: 462, 2020 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23.

    Ghimire S, Yerneni H, Oyadomari TA, Sedlacek M: Metabolic alkalosis and cystic fibrosis: A case report. Ann Intern Med 173: 315, 2020 PubMed

  • 24.

    Raya Cruz M, Zubillaga IP, Schneider P: Metabolic alkalosis with hyponatremia, hypokalemia and hypochloremia as the initial presentation of cystic fibrosis in an adult patient. Med Clin (Barc) 143: 137138, 2014 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25.

    Choi JY, Muallem D, Kiselyov K, Lee MG, Thomas PJ, Muallem S: Aberrant CFTR-dependent HCO3 transport in mutations associated with cystic fibrosis. Nature 410: 9497, 2001 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26.

    Quinton PM: The neglected ion: HCO3 −. Nat Med 7: 292293, 2001 PubMed

  • 27.

    Quinton PM: Cystic fibrosis: Impaired bicarbonate secretion and mucoviscidosis. Lancet 372: 415417, 2008 PubMed

  • 28.

    Berg P, Svendsen SL, Sorensen MV, Larsen CK, Andersen JF, Jensen-Fangel S, et al.: Impaired renal HCO3 excretion in cystic fibrosis. J Am Soc Nephrol 31: 17111727, 2020 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29.

    Berg P, Jeppesen M, Leipziger J: Cystic fibrosis in the kidney: New lessons from impaired renal HCO3 excretion. Curr Opin Nephrol Hypertens 30: 437443, 2021 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30.

    Berg P, Svendsen SL, Hoang TTL, Praetorius HA, Sorensen MV, Leipziger J: Impaired renal HCO3 secretion in CFTR deficient mice causes metabolic alkalosis during chronic base-loading. Acta Physiol (Oxf) 231: e13591, 2021 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31.

    Berg P, Svendsen SL, Sorensen MV, Schreiber R, Kunzelmann K, Leipziger J: The molecular mechanism of CFTR- and secretin-dependent renal bicarbonate excretion. J Physiol 599: 30033011, 2021 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 32.

    Berg P, Andersen JF, Sørensen MV, Wang T, Malte H, Leipziger J: Alkalosis-induced hypoventilation in cystic fibrosis: The importance of efficient renal adaptation. Proc Natl Acad Sci U S A 119: e2116836119, 2022 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 33.

    Berg P, Sorensen MV, Rousing AQ, Vebert Olesen H, Jensen-Fangel S, Jeppesen M, et al.: Challenged urine bicarbonate excretion as a measure of cystic fibrosis transmembrane conductance regulator function in cystic fibrosis. Ann Intern Med 175: 15431551, 2022 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34.

    Berg P, Jensen T, Andersen JF, Svendsen SL, Modvig IM, Wang T, et al.: Loss of the secretin receptor impairs renal bicarbonate excretion and aggravates metabolic alkalosis in mice during acute base-loading. J Am Soc Nephrol 34: 13291342, 2023 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35.

    Berg P, Svendsen SL, Ayasse N, Sorensen MV, Leipziger J: Secretin: A hormone for HCO3 homeostasis [published online ahead of print Jan 25, 2024]. Pflugers Arch doi:10.1007/s00424-024-02906-3 2024 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36.

    Wall SM: Regulation of blood pressure and salt balance by pendrin-positive intercalated cells: Donald Seldin Lecture 2020. Hypertension 79: 706716, 2022 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37.

    Varasteh Kia M, Barone S, McDonough AA, Zahedi K, Xu J, Soleimani M: Downregulation of the Cl/HCO3 exchanger pendrin in kidneys of mice with cystic fibrosis: Role in the pathogenesis of metabolic alkalosis. Cell Physiol Biochem 45: 15511565, 2018 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38.

    Procino G, Milano S, Carmosino M, Barbieri C, Nicoletti MC, Li JH, et al.: Combination of secretin and fluvastatin ameliorates the polyuria associated with X-linked nephrogenic diabetes insipidus in mice. Kidney Int 86: 127138, 2014 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39.

    Azroyan A, Morla L, Crambert G, Laghmani K, Ramakrishnan S, Edwards A, et al.: Regulation of pendrin by cAMP: Possible involvement in β-adrenergic-dependent NaCl retention. Am J Physiol Renal Physiol 302: F1180F1187, 2012 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40.

    Vitzthum H, Meyer-Schwesinger C, Ehmke H: Novel functions of the anion exchanger AE4 (SLC4A9) [published online ahead of print Jan 29, 2024]. Pflugers Arch doi:10.1007/s00424-023-02899-5 2024 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41.

    Bijman J, Quinton PM: Lactate and bicarbonate uptake in the sweat duct of cystic fibrosis and normal subjects. Pediatr Res 21: 7982, 1987 PubMed

  • 42.

    Devlin J, Beckett NS, David TJ. Elevated sweat potassium, hyperaldosteronism and pseudo-Bartter's syndrome: a spectrum of disorders associated with cystic fibrosis. J R Soc Med 82: 3843, 1989 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43.

    Pedroli G, Liechti-Gallati S, Mauri S, Birrer P, Kraemer R, Foletti-Jäggi C, et al.: Chronic metabolic alkalosis: not uncommon in young children with severe cystic fibrosis. Am J Nephrol 15: 245250, 1995 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44.

    Holland AE, Wilson JW, Kotsimbos TC, Naughton MT: Metabolic alkalosis contributes to acute hypercapnic respiratory failure in adult cystic fibrosis. Chest 124: 490493, 2003 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 45.

    Al-Ghimlas F, Faughnan ME, Tullis E: Metabolic alkalosis in adults with stable cystic fibrosis. Open Respir Med J 6: 5962, 2012 PubMed

  • 46.

    Spoletini G, Fitch G, Gillgrass L, Etherington C, Clifton I, Peckham DG: Urinary bicarbonate and metabolic alkalosis during exacerbations in cystic fibrosis. ERJ Open Res 8: 0066902021, 2022 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 47.

    Bemand TJ, Chatoor R, Natale P, Strippoli G, Delaney A: Acetazolamide for metabolic alkalosis complicating respiratory failure with chronic obstructive pulmonary disease or obesity hypoventilation syndrome: A systematic review. Thorax 78: 10041010, 2023 PubMed

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 48.

    Stinebaugh BJ, Austin WH: Acid-base balance—A pattern-analysis diagram. N Engl J Med 275: 375, 1966 PubMed

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