title: Perioperative management of hemostasis for surgery of benign hepatic adenomas in patients with glycogen storage disease type ia.
abstract: The development of hepatocellular 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 underwent 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.
authors: APHP, Centre de Référence Maladies Héréditaires du Métabolisme Hépatique, Service de Pédiatrie, Hôpital Antoine Béclère, 157 Rue de la Porte de Trivaux, 92141, Clamart cedex, France, philippe.labrune@abc.aphp.fr.
authors: Alix
authors: A
authors: Mollet-Boudjemline
authors: Aurélie
authors: A
authors: Hubert-Buron
authors: Catherine
authors: C
authors: Boyer-Neumann
authors: Roxana
authors: R
authors: Aldea
authors: Dominique
authors: D
authors: Franco
authors: Pascale
authors: P
authors: Trioche-Eberschweiller
authors: Anne-Elisabeth
authors: AE
authors: Mas
authors: Mylène
authors: M
authors: Mabille
authors: Philippe
authors: P
authors: Labrune
authors: Vincent
authors: V
authors: Gajdos