Document Detail

Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent.
MedLine Citation:
PMID:  9048354     Owner:  NLM     Status:  MEDLINE    
Cystic fibrosis (CF) is an exocrine disease affecting multiple organ systems. Patients with CF usually present with respiratory or gastrointestinal abnormalities. This study presents a case of a previously healthy 17-yr-old man who was diagnosed with CF after presenting with metabolic alkalosis and hypokalemia. The defect associated with CF is in the cystic fibrosis transmembrane regulator (CFTR), which acts primarily as a chloride channel. Partially functional CFTR may be associated with less severe pulmonary and gastrointestinal manifestations, as in the case presented. Dysfunctional CFTR in the sweat ducts of CF patients are responsible for excessive chloride and sodium losses, especially in warm weather. Hypokalemia seen with heat stress is secondary to sweat as well as renal potassium wasting. Metabolic alkalosis is maintained by the excessive sweat sodium chloride losses which leads to extracellular fluid (ECF) volume contraction and chloride depletion. Generation of alkalosis may be related to dysfunctional CFTR in the kidney, but is most likely secondary to hypokalemia with ECF volume contraction. Finally, one must consider CF when confronted with hypokalemia and alkalosis in a previously healthy patient.
C M Bates; M Baum; R Quigley
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the American Society of Nephrology : JASN     Volume:  8     ISSN:  1046-6673     ISO Abbreviation:  J. Am. Soc. Nephrol.     Publication Date:  1997 Feb 
Date Detail:
Created Date:  1997-06-05     Completed Date:  1997-06-05     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  9013836     Medline TA:  J Am Soc Nephrol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  352-5     Citation Subset:  IM    
Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas 75235-9063, USA.
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MeSH Terms
Acidosis / etiology*
Cystic Fibrosis / complications*,  diagnosis*,  genetics
Cystic Fibrosis Transmembrane Conductance Regulator / genetics,  metabolism
Hyperaldosteronism / etiology
Hypokalemia / etiology*
Sweat / metabolism
Reg. No./Substance:
0/CFTR protein, human; 126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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