Children with pancreatic insufficiency and neutropenia syndrome

Children with pancreatic insufficiency and neutropenia syndrome were reported by Shwachman in 1964, so they are also called Shwachman syndrome, Shwachman-Diamond syndrome. Essence is congenital pancreatic insufficiency, accompanied by hypoplasia of the bone marrow hematopoietic system. Most infants and young children develop disease in 2 to 10 months, have a familial tendency, may belong to autosomal recessive inheritance.