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Atypical presentation of cystic fibrosis--obese adolescent with hypertension and pseudo-Bartter's syndrome.
MedLine Citation:
PMID:  22624431     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
INTRODUCTION: Infants with cystic fibrosis may fail to thrive despite recommended caloric intake because of electrolyte disurbances caused by salt depletion resulting in hypochloremic metabolic alkalosis or pseudo-Bartter's syndrome. In most patients reported symptoms began in infancy, but it may be an initial presentation of disease in a previously healthy adolescent.
CASE REPORT: A 15-year-old boy was admitted for evaluation of recurrent episodes of malaise associated with dehydration and acute renal insufficiency. Laboratory analysis showed hypochloremic metabolic alkalosis with hyponatremia and hypokalemia. On admission the boy was obese, with body weight of 95.5 kg (> P97), height 174 cm (> P75), and body mass index of 31.2 kg/m2 (> P95). Physical examination was inconclusive. Blood pressure holter monitoring proved significant systolic hypertension. Routine urinalysis, protein and electrolyte levels in urine were normal. Plasma renin and aldosteron were normal. Sweat chloride concentration was 63 mmol/L. Genetic testing confirmed the diagnosis of cystic fibrosis.
CONCLUSION: To our knowledge, this is the first reported case of atypical presentation of cystic fibrosis in an adolescent presented with pseudo-Bartter's syndrome and signs of obesity and hypertension. We suggest that every patient with hypochloremic metabolic alkalosis should be evaluated for cystic fibrosis.
Authors:
Aleksandar Sovtić; Predrag Minić; Radovan Bogdanović; Natasa Stajić; Milan Rodić; Gordana Marković-Sovtić
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Vojnosanitetski pregled. Military-medical and pharmaceutical review     Volume:  69     ISSN:  0042-8450     ISO Abbreviation:  Vojnosanit Pregl     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-05-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  21530700R     Medline TA:  Vojnosanit Pregl     Country:  Serbia    
Other Details:
Languages:  eng     Pagination:  367-9     Citation Subset:  IM    
Affiliation:
Department for Pulmonology and Bronchology, Mother and Child Health Institute of Serbia "Dr Vukan Cupić", Belgrade, Serbia. asovtic@eunet.rs
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