Pediatric gastroenterology 1/1/69-12/31/75: a review. Part I. Hollow viscera and the pancreas.

Cumulative survival in cystic fibrosis (CF) for three successive 5-year periods between 
1952 and 1967 is 35%, 64%, and 77%, respectively. Operative mortality 
in meconium ileus is not reduced to 25% from 70%. N-Acetylcysteine 
effectively cleanses the bowel of tenacious meconium plugs when directly injected 
during laparotomy. However, Mucomist enema alone generally does not suffice. Selected 
cases have been treated successfully with hyperosmolar Gastrograffin enema. Several screening 
tests, based on the increased albumin concentrations present in the meconium, 
are available for CF. The Boehringer-Mannheim test strip is favored, and 
yielded no false positives in 69,000 tests of meconium. However, 4 
of 60 neonates who subsequently proved to have CF had false 
negative tests. A pathognomonic elevation of sodium and chloride concentrations in 
the sweat collected by pilocarpine iontophoresis remains the definitive diagnostic test. 
Bile acid malabsorption occurs in CF but not in celiac disease. 
The authors postulate that the presence of maldigested materials or the 
absence of pancreatic enzymes interferes with bile acid absorption. The defect 
disappears with pancreatic extracts. Substitution of medium-chain triglycerides for dietary long-chain 
fatty acids reduces steatorrhea in CF. However, the positive effect of 
MCT on weight gain is transitory. Growth retardation correlates better with 
the degree of pulmonary disease than with pancreatic insufficiency. 