Cystic fibrosis of the pancreas: report of a Japanese autopsy case.
Cystic fibrosis of the pancreas is considered to be rare in the Japanese population. Forty-five cases from the Japanese literature (1951-1983) are reviewed and one autopsy case added. The patient was a 17-year-old Japanese male with recurrent episodes of pulmonary infection. His sweat test was positive (sodium 332 mg/l, chloride 316 mg/l). No particular hereditary trait was identified. Autopsy findings showed cystic dilatation of pancreatic ducts and ductules with mucous hypersecretion. Acini of the pancreas were replaced by fibrosis, while islands of Langerhans remained. These findings were compatible with cystic fibrosis of the pancreas (mucoviscidosis). The lungs showed chronic hypertrophic bronchitis and acute obliterating bronchiolitis. Cystic dilatation and hypersecretion were seen in the esophageal glands, Brenner's glands, and enteric glands. Goblet-cell hyperplasia was seen in the enteric glands. Central fatty metamorphosis and focal bile duct proliferation were seen in the liver. No obvious intralobular cholestasis was present. The epithelium of the gall bladder was highly columnar.
D000293:Adolescent; D001652:Bile Ducts / Q000473:pathology; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging / Q000473:pathology*; D004386:Duodenum / Q000473:pathology; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D010183:Pancreatic Ducts / Q000473:pathology; D011859:Radiography