Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption

doi:10.1016/S0022-3476(82)80501-5

The Journal of Pediatrics

Volume 100, Issue 6, June 1982, Pages 857-862

https://doi.org/10.1016/S0022-3476(82)80501-5 Get rights and content

The clinical presentation, courses, and sweat chloride values of 72 CF patients with normal fat absorption are described. In general, these patients had milder clinical symptoms and a lower mean sweat chloride value than their counterparts with steatorrhoea. Pulmonary function tests, including FEV₁, FVC, FEF_25%–75%, Pa_O₂, and RV/TLC%, were significantly better in patients with normal fat absorption compared with both male and female patients who had steatorrhoea. The maintenance of better pulmonary function, coupled with the low mortality, suggests that patients without steatorrhoea have a better prognosis. This difference remains unexplained, but may be contributed to by nutritional, genetic, or pancreatic factors.

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Citation Excerpt :
Before knowledge of a specific genetic defect, patients with CF were noted to have a wide spectrum of survival as well as nutritional and pulmonary outcomes.20,21 Patients with CF and normal fat absorption had milder clinical symptoms, lower mean sweat chloride values, and higher lung function compared with those with steatorrhea.21 It was speculated that variation in disease severity was the result of genetic and environmental factors.22
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^**: Supported by the Canadian Cystic Fibrosis Foundation.

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Original articleImproved respiratory prognosis in patients with cystic fibrosis with normal fat absorption**

J Pediatr

Pediatr Clin North Am

Am J Med

Lancet

Clin Chim Acta

Cystic fibrosis of the pancreas

JAMA

Fibrocystic disease of the pancreas with normal or partial pancreatic function

Pediatrics

Cystic fibrosis of the pancreas with varying degrees of pancreatic insufficiency

Am J Dis Child

Pancreozymin secretin test of exocrine pancreatic function in cystic fibrosis and the significance of the result for the pathogenesis of the disease

Can Med Assoc J

Improved pulmonary function in cystic fibrosis patients without pancreatic insufficiency

A simple method for collection and analysis of sweat for chloride

Am J Dis Child

Statistical methods

Original article
Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption**