[Congenital hypoplasia of the pancreas with lipomatosis and bone marrow dysfunction (Shwachman syndrome)].

The description is given of Shwachman syndrome in a male of 17, with a long anamnesis of malabsorption syndrome, granulocytopenia, repeated bronchopneumonia, somatogenic subnanism and hypogonadism. The death resulted from acute cardiovascular insufficiency. Post-mortem examination revealed exocrine pancreas hypoplasia with lipomatosis, Wirsung duct atresia, fibrocholangiocytosis and fat hepatosis, visceral anemia, delay in the maturation of granulocytes in the bone marrow, somatogenic polyglandular endocrinopathy, metabolic myocardium necrosis, calcinosis of the small arteries wall, lymphoid tissue atrophy, focal pneumonia of the upper lobe of the left lung.