Haematological findings in type Ib glycogen storage disease before and after portacaval shunt.

Persistent neutropenia and repeated respiratory infections were documented in a girl with glycogen storage disease type Ib. A termino-lateral portacaval shunt resulted in normalisation of the granulocyte counts and disappearance of the recurrent infections. The platelet dysfunction that was apparent before surgery, was also corrected by the shunting procedure. A marked hypochromic anaemia, however, probably caused by a sequestration of iron in the spleen and resistant to therapy, remains a persistent feature in this patient.