Update: cystic fibrosis
Dolan, T.F.
Pediatric Annals 15(4): 296-7 300-1 304
1986
ISSN/ISBN: 0090-4481 PMID: 3714330 Document Number: 284215
A brief overview emphasizes the multiple presenting signs and symptoms, complications, and management of children with hereditary cystic fibrosis (CF). This disease causes chronic obstructive pulmonary disease and pancreatic insufficiency and currently limits life expectancy to about 21 years. Specific attention is given to pulmonary and gastrointestinal-hepatobiliary manifestations, and to the consequences of malabsorption and metabolic losses in the neonate, infant, and older child. These consequences include steatorrhea, hypoproteinemia, vitamin A and E deficiencies, hyperglycemia without ketosis, failure to thrive, and delayed maturation. Various tests for diagnosing CF are discussed.(wz)