Association of pancreatic insufficiency and chronic neutropenia in childhood

Abstract

Eleven patients with the syndrome of pancreatic insufficiency and neutropenia are described. Although this syndrome is rare, after excluding patients with cystic fibrosis, it appears to be the commonest cause of pancreatic insufficiency in childhood. The association of neutropenia and pancreatic insufficiency may be overlooked. Neutropenia is often variable and leucocytosis occurs with infections. If frequent white cell counts are not performed when the patient is free from infection, neutropenia may not be apparent. In these patients there is a tendency for steatorrhoea to diminish with time though pancreatic insufficiency is still demonstrable. Symptoms of malabsorption may, therefore, be minimal in older children. Thus, in children with idiopathic neutropenia, and perhaps in adults, pancreatic function should be investigated. Metaphysial dysostosis and Hirschsprung's disease were present in some of the patients, suggesting that the syndrome is more complex. A familial incidence was observed but, because of the small number of patients studied, suggestions regarding a mode of inheritance are not possible.