Shwachman syndrome. Report of three cases

ABSTRACT

Shwachman syndrome, next to cystic fibrosis, is the second cause of congenital exocrine pancreatic insufficiency in children. It appears as steatorrhea, recurrent infections and hematologic abnormalities such as neutropenia, skeletal dysplasia and short stature. In this study, we reviewed 3 patients' histories. All of them showed cellular chemotaxic defect. One of them had been affected by nephrotic syndrome and finally succumbed to septic shock. This patient suffered from skeletal disorders