Pancreatic Insufficiency and Neutropenia With Associated Immunoglobulin Deficit

Recent reports have called attention to the association of pancreatic insufficiency and bone marrow dysfunction in children.1-7This syndrome, although uncommon, is thought to represent the major disorder of pancreatic dysfunction in childhood when cystic fibrosis is excluded. We have recently evaluated such a patient in whom the significance of the association was not realized until adolescence and who was also found to have immunoglobulin deficiencies. Patient Summary A 16-year-old white boy was admitted to the hospital in 1966 for evaluation of previously diagnosed pancreatic insufficiency. He was born at eight months' gestation and weighed 3,281 gm (5 lb 4 oz). His first teeth appeared when he was 7 months old and he walked at 21 months of age. The patient was thought to have celiac disease during his first year when he was found to be anemic and to have bulky, foul-smelling stools. It was also reported that