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April 1986

Portacaval Shunt as Treatment for Glycogenosis Ib

Author Affiliations

Department of Pediatric Hematology Hacettepe University Ankara, Turkey

Am J Dis Child. 1986;140(4):324. doi:10.1001/archpedi.1986.02140180058011

Sir.—I read with great interest the article by Dr Ambruso and colleagues1 "Infectious and Bleeding Complications in Patients With Glycogenosis lb." Granulocytes and platelets have no detectable glucose-6-phosphatase activity; therefore, the functional and quantitative impairments of these cells should not be related to this enzyme directly but as results of general metabolic derangements reflected in the serum. The correction of platelet aggregation, motility, chemotaxis, and the in vivo mobilization of neutrophils and leukopenia in the patient of Corbeel et al2 following a portacaval shunt operation would support this assumption.

Since "the infection was the most prominent complication sustained" by the patients of Dr Ambruso and colleagues1 as a general characteristic of this form of the disease for which "no specific therapy is available," portosystemic shunt operations should be suggested to correct these patients' granulocytopenia, and thus the infection. In addition, restudy of the granulocyte count, as

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