Pancreatic lipomatous hypoplasia associated with skeleton anomalies. Shwachman's syndrome
Guerrero, J.; Gamallo, C.; Agustí, B.; Segurado, E.; Vázquez, C.
Anales Espanoles de Pediatria 11(10): 721-728
1978
ISSN/ISBN: 0302-4342 PMID: 727604 Document Number: 136057
A five month old girl with exocrine pancreatic insufficiency, skeletal deformities and a non constant neutropenia (Shwachman's syndrome) is presented. Infectious complications, mainly septic and pulmonary, which caused her death, were frequent. The pathologic findings showed a pancreatic lipomatous hypoplasia and absence of zymogen granulos, as well as anomalies of the growing cartilage where no radiological abnormalities could be seen.