Pancreatic achylia and glycosuria due to cystic diseaseof the pancreas in a 9-year-old child

Abstract

Summary The case of a 9-year-old, previously healthy boy is described; he developedintestinal obstruction and at operation presented an abdominal mass, which on two separate biopsies proved to be pancreas studded with multiple small cysts. Microscopic examination revealed a different lesion from that seen in congenital cystic fibrosis of the pancreas, but closely resembling that found in polycystic disease as described by Lindau. Extreme emaciation and evidence of vitamin A deficiency were present. These were accompanied by pancreatic achylia, failure to absorb vitamin A, flat gelatine tolerance test, and the presence of glycosuria. Clinical, chemical, and metabolic studies of the case are reported. In a follow-up after one year, the patient's general condition is good although his height and weight are retarded and he is prone to develop upper respiratory infections with rapid loss of weight. His mild glycosuria persists. Cystic disease of the pancreas and tests for pancreatic function are discussed.