G.P.134: Liver functional impairment and glycolipid metabolic abnormality in myotonic dystrophy type 1

Abstract

Recently, non-alcoholic steatohepatitis complicated in myotonic dystrophy type 1 (DM1) has been reported. Glucose intolerance is one of well-known complications in DM1. Lipid metabolic abnormality such as increase of visceral fat or abnormal response to oral lipid absorption test is also described in DM1. The aim of this study was to elucidate the relation between liver functional impairment and glycolipid metabolic abnormality in DM1. Forty-two patients in DM1 without cholelithiasis (19 females and 23 males; the mean age, 46.7years; the mean number of CTG repeat, 1125) were participated. Subjects had computed tomography (CT) examination to measure area of visceral fat (Vfat) and liver spleen ratio of CT density (LSR). Routine blood test was performed. Linear regression analysis was used to assess the association among Vfat, LSR, the number of CTG repeat, age, and results of blood test. According to the oral glucose tolerance test, patients were classified into diabetes (12 cases), impaired glucose tolerance (IGT, 18), and normal (NGT, 12). Similar parameters in each group were compared using non-parametric test. Forty-five percent of patients showed V-fad above 100cm 2 . Nineteen percent had LSR less 0.9. High value of gamma GTP was observed in 81%, abnormal ALT was in 57%, and AST in 33%. The number of CTG repeat was significantly correlated to Vfat or gamma GTP. There was significant positive correlation between Vfat and gamma GTP, ALT or AST. Gamma GTP was significantly correlated to serum triglyceride or fasting plasma glucose. Vfat in diabetes group was significantly higher than that in IGT or NGT, although there was no significant difference for Vfat between IGT and NGT. Gamma GTP in IGT was significantly higher than that in NGT, and was lower than that in diabetes group. Liver functional impairment was relevant to glucose intolerance, visceral fat accumulation, and the number of CTG repeat in DM1.