Metabolic consequences of pancreatic insufficiency in cystic fibrosis of the pancreas: a statistical analysis

OVER the years opinion has varied on the nature of the dietary regimen which most effectively compensates for the specific digestive defect in cystic fibrosis of the pancreas. This paper comprises a review of the literature and a statistical analysis of available data to determine whether the accumulated evidence supports any dietary recommendations. HISTORICAL BACKGROUND All reviews of pancreatic function begin with the work of Claude Bernard. This is appropriate since he first correctly described in two papers, 1849 and 1856, the role of the pancreas in the digestive process in dogs. A series of confusing experiments, many of them faulty, led in the next 80 years to changing estimates of the importance of this gland in digestion. The first point at issue was whether the pancreas was involved in digestion. Some investigators questioned, while others substantiated, Bernard's observations. Abelmann, working in the laboratory of Minkowski, was principally responsible for confirming the work of Bernard. After the necessity of the pancreas for normal digestion had been established, the route of excretion of the pancreatic digestive ferments was disputed. Some claimed all excretion was via the blood, some were uncertain of the route; finally Hess and Pratt et al. showed that the secretion was directly into the bowel. Hess, by careful dissection, demonstrated that the dog has from two to five accessory pancreatic ducts, and Pratt and his associates showed that ligation of all ducts to exclude pancreatic juice from the intestinal tract always produced marked impairment in absorption of fat and nitrogen. These early reports have been critically reviewed by Handelsman. The observations of Pratt et al. have been confirmed repeatedly. Coffey et al. published extensive metabolic data which seem to have ended any confusion. Experimental achylia pancreatica was produced in dogs by either ligation of all pancreatic ducts or by extirpation of the pancreas. Both operations had the same effect on fat and nitrogen absorption.