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Low sweat electrolytes in a patient with cystic fibrosis.
Am J Med 1980; 69(4):643-6AJ

Abstract

A patient with the clinical syndrome of cystic fibrosis characterized by chronic pulmonary disease, infection with mucoid Pseudomonas aeruginosa, sinusitis, nasal polyposis, abnormal pancreatic bicarbonate response to secretin stimulation, but normal levels of trypsin and chymotrypsin in the duodenal drainage, and a sibling with autopsy-documented cystic fibrosis, is described. Sweat chloride ranged from 20 to 44 meq/liter and sweat sodium from 36 to 55 meq/liter. Immunoglobulin deficiency, alpha 1-antitrypsin deficiency, tuberculosis and abnormalities of ciliary ultrastructure were excluded. Review of sweat electrolytes in 213 patients with cystic fibrosis revealed that patients with normal pancreatic enzyme release have significantly lower sweat sodium and chloride concentrations (p < 0.0005) than do patients with pancreatic insufficiency. Chronic pulmonary disease, pancreatic insufficiency and elevated levels of sweat electrolytes comprise the classic diagnostic triad for cystic fibrosis. The expression of these features may be variable, but the sweat test remains the cardinal laboratory confirmation of the diagnosis. Over 98 percent of patients with cystic fibrosis have sweat chloride values greater than 60 meq/liter, 1 to 2 percent between 50 and 60 meq/liter, and only about one in 1,000, like our patient, less than 50 meq/liter. Patients with cystic fibrosis with borderline sweat chloride values frequently have chronic pulmonary disease but intact pancreatic enzyme release. In such patients, family history, ancillary clinical features and systemic exclusion of other syndromes assume special diagnostic importance.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

7424954

Citation

Davis, P B., et al. "Low Sweat Electrolytes in a Patient With Cystic Fibrosis." The American Journal of Medicine, vol. 69, no. 4, 1980, pp. 643-6.
Davis PB, Hubbard VS, Di Sant'Agnese PA. Low sweat electrolytes in a patient with cystic fibrosis. Am J Med. 1980;69(4):643-6.
Davis, P. B., Hubbard, V. S., & Di Sant'Agnese, P. A. (1980). Low sweat electrolytes in a patient with cystic fibrosis. The American Journal of Medicine, 69(4), pp. 643-6.
Davis PB, Hubbard VS, Di Sant'Agnese PA. Low Sweat Electrolytes in a Patient With Cystic Fibrosis. Am J Med. 1980;69(4):643-6. PubMed PMID: 7424954.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Low sweat electrolytes in a patient with cystic fibrosis. AU - Davis,P B, AU - Hubbard,V S, AU - Di Sant'Agnese,P A, PY - 1980/10/1/pubmed PY - 1980/10/1/medline PY - 1980/10/1/entrez SP - 643 EP - 6 JF - The American journal of medicine JO - Am. J. Med. VL - 69 IS - 4 N2 - A patient with the clinical syndrome of cystic fibrosis characterized by chronic pulmonary disease, infection with mucoid Pseudomonas aeruginosa, sinusitis, nasal polyposis, abnormal pancreatic bicarbonate response to secretin stimulation, but normal levels of trypsin and chymotrypsin in the duodenal drainage, and a sibling with autopsy-documented cystic fibrosis, is described. Sweat chloride ranged from 20 to 44 meq/liter and sweat sodium from 36 to 55 meq/liter. Immunoglobulin deficiency, alpha 1-antitrypsin deficiency, tuberculosis and abnormalities of ciliary ultrastructure were excluded. Review of sweat electrolytes in 213 patients with cystic fibrosis revealed that patients with normal pancreatic enzyme release have significantly lower sweat sodium and chloride concentrations (p < 0.0005) than do patients with pancreatic insufficiency. Chronic pulmonary disease, pancreatic insufficiency and elevated levels of sweat electrolytes comprise the classic diagnostic triad for cystic fibrosis. The expression of these features may be variable, but the sweat test remains the cardinal laboratory confirmation of the diagnosis. Over 98 percent of patients with cystic fibrosis have sweat chloride values greater than 60 meq/liter, 1 to 2 percent between 50 and 60 meq/liter, and only about one in 1,000, like our patient, less than 50 meq/liter. Patients with cystic fibrosis with borderline sweat chloride values frequently have chronic pulmonary disease but intact pancreatic enzyme release. In such patients, family history, ancillary clinical features and systemic exclusion of other syndromes assume special diagnostic importance. SN - 0002-9343 UR - https://www.unboundmedicine.com/medline/citation/7424954/Low_sweat_electrolytes_in_a_patient_with_cystic_fibrosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0002-9343(80)90482-9 DB - PRIME DP - Unbound Medicine ER -