The development of hepato cellular adenomas in the liver of patients with glycogen storage disease type I is a well-known complication of the disease. Surgical procedures and perioperative managements described so far have reported persistent and important morbidity. We report here a series of six patients (three males and three females) who under went hepatic resection, and we propose a new hemostatic management protocol comprising glucose infusion, corticosteroids, desmopressin, and antifibrinolytic drugs, used to prevent efficaciously hepatic hemorrhage due to glycogen storage disease (GSD) platelet dysfunction.
CITATION STYLE
Mollet-Boudjemline, A., Hubert-Buron, A., Trioche-Eberschweiller, P., Gajdos, V., Boyer-Neumann, C., Boyer-Neumann, C., … Labrune, P. (2011). Perioperative management of hemostasis for surgery of benign hepatic adenomas in patients with glycogen storage disease type Ia. Journal of Inherited Metabolic Disease, 1, 97–106. https://doi.org/10.1007/8904_2011_23
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