Portacaval Shunt as Treatment for Glycogenosis Ib-Reply

In Reply.—Corbeel et al¹ have suggested that portacaval anastomosis be considered as a primary mode of therapy to resolve neutropenia and neutrophil dysfunction on the basis of clinical experience with a patient previously described. However, in at least one other patient, a portacaval shunt operation did not reverse the neutropenia or infections²; and, indeed, the patient described by Corbeel et al had neutropenia on at least three occasions after the portacaval anastomosis.¹ Furthermore, the effect of portosystemic shunting in this patient could not be completely appreciated since granulocyte dysfunction prior to surgery could not be completely documented. Finally, platelet dysfunction in Corbeel and colleagues' patient normalized in response to continuous gastric feeding for three weeks, not the shunt procedure itself. Although increases in leukocyte counts are seen in other disorders after shunting, there is little experience in glycogenosis lb to support its use routinely as a