[Myocardial fibrosis in Shwachman's syndrome (author's transl)].

A case of a child affected by Shwachman's syndrome with a chronic diarrhea, cyclic neutropenia and bone defects is described. The child died at the age of eight months from an acute cardiac failure which showed analytical alterations compatible to an acute myocardial infarction. Pathologic study showed a pancreatic lipomatous hypoplasia, myocardial fibrosis and chondrometaphyseal dysplasia. Main hypothesis that relate pancreatic pathology and development of myocardial fibrosis are discussed, although there is a lack of definitive evidence for confirming such relation.