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.