title: Glycemic management in living donor liver transplantation for patients with glycogen storage disease type 1b.
abstract: GSD type 1b is an autosomal recessive inborn error of carbohydrate metabolism caused by defects of the G6Pase translocase (G6PT). Patients with GSD1b have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll-like face, and neutropenia. LT has been indicated for severe glucose intolerance. This study retrospectively reviewed glycemic management of eight children with a diagnosis of GSD1b who underwent liver transplantation (LDLT). Between November 2005 and September 2011, 172 children underwent LDLT, of which eight (4.7%) were indicated for GSD1b. Glucose-rich solution was placed in all children when preoperative fasting started to prevent preoperative hypoglycemia. During the reperfusion of graft, the glucose administration could significantly be reduced to maintain the proper blood glucose level, while the dosage of glucose administration prior to reperfusion of graft was significantly higher in the patients with GSD1b in comparison with patients with BA. The current series also showed significantly high incidence of infectious complications in the patients with GSD1b owing to persistent neutropenia after LDLT. All patients are doing well with an excellent quality of life owing to the stabilization of glucose intolerance. This current study clearly documented drastic change in glycemic management in LDLT. Cautious perioperative management to prevent hypoglycemia and infection is crucial for successful LT.
authors.affiliation: Department of Anesthetic and Intensive Care, National Center for Child Health and Development, Tokyo, Japan. karaki-c@ncchd.go.jp
authors.forename: Chiaki
authors.initials: C
authors.lastname: Karaki
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Takanobu
authors.initials: T
authors.lastname: Shigeta
authors.forename: Hajime
authors.initials: H
authors.lastname: Uchida
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kanazawa
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Kakiuchi
authors.forename: Akinari
authors.initials: A
authors.lastname: Fukuda
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Suzuki
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005947:Glucose / Q000627:therapeutic use*; D018149:Glucose Intolerance / Q000209:etiology; D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000601:surgery*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology / Q000517:prevention & control*; D007223:Infant; D007431:Intraoperative Complications / Q000517:prevention & control*; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D011183:Postoperative Complications / Q000517:prevention & control*; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D005947:Glucose