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• Clinical, hematologic, and immunologic findings were reviewed in 21 patients with glycogenosis lb. Fifteen of the patients suffered from moderate to severe bacterial infections. Ten patients had excessive epistaxis or bleeding from surgical sites, and eight suffered oral and anal mucosal ulceration. Sixteen of 21 patients exhibited chronic neutropenia associated with abnormalities In myelold maturation and decreases in the bone marrow storage and peripheral marginating pools. Diminished neutrophil motility was documented in 14 of 15 patients tested, and adherence was decreased in three patients studied. Neutrophil microbicidal activity, reduction of nitroblue tetrazolium, and ingestion were normal in all patients tested. Bleeding times were prolonged in five of eight patients, and results of platelet function studies were abnormal in five individuals. Excessive bleeding in patients with glycogenoses la and lb are similar and may be secondary to the functional deficiency of glucose-6-phosphatase. However, neutropenia, neutrophil dysfunction, and the resulting Infectious complications are specific for lb disease and may be related to abnormal glucose-6-phosphate transport. (AJDC1985;139:691-697)

In Reply .—Corbeel et al 1 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 infections 2 ; 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