Lu Chun-Ting, Yang Jing, Huang Si-Min, Feng Lie, Li Ze-Jian
The First Affiliated Hospital of Jinan University Department of Internal Medicine Department of Endocrinology and Metabolism Medical Centre of Stomatology, The First Affiliated Hospital of Jinan University, Guangdong, China.
Medicine (Baltimore). 2017 Nov;96(45):e8638. doi: 10.1097/MD.0000000000008638.
Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) primarily manifests in neonates or infants with hepatomegaly, liver dysfunction, and hypoglycemia. This study investigated the functions of islet beta cells and their correlations with liver dysfunction in NICCD patients.We retrospectively analyzed clinical data on liver function and islet beta cell functions for 36 patients diagnosed with NICCD and 50 subjects as the control group. The NICCD group had significantly higher total bilirubin (TBIL), direct bilirubin (DBIL), alanine aminotransferase (ALT), aspartate amino transferase (AST), gamma-glutamyl transpeptidase (GGT), alkaline phosphatase (ALP) and alpha-fetoprotein (AFP) levels and albumin/globulin ratio (A/G) (P < .05), and lower ALB and GLB levels than the control group (P < .05). The differences in fasting blood glucose (FBG), fasting insulin, C-peptide (C-P), the homeostasis model of assessment for the insulin resistance index (HOMA-IR), fasting beta cell function (FBCI), and the HOMA beta cell function index (HBCI) between the NICCD and control groups were not significant (P > .05). A linear correlation was found between FBG and fasting insulin (P < .001) and between FBG and C-P in the NICCD patients (P = .001). Fasting insulin (P = .023), HOMA-IR (P = .023), FBCI (P = .049), and HBCI (P = .048) were positively correlated with increases in the ALT level. There was no difference in islet beta cell functions between the NICCD and control groups. The liver dysfunction may be correlated with islet beta cell functions in NICCD patients.
瓜氨酸缺乏所致新生儿肝内胆汁淤积症（NICCD）主要表现为新生儿或婴儿出现肝肿大、肝功能障碍和低血糖。本研究调查了NICCD患者胰岛β细胞的功能及其与肝功能障碍的相关性。我们回顾性分析了36例确诊为NICCD的患者以及作为对照组的50名受试者的肝功能和胰岛β细胞功能的临床数据。NICCD组的总胆红素（TBIL）、直接胆红素（DBIL）、丙氨酸氨基转移酶（ALT）、天冬氨酸氨基转移酶（AST）、γ-谷氨酰转肽酶（GGT）、碱性磷酸酶（ALP）和甲胎蛋白（AFP）水平以及白蛋白/球蛋白比值（A/G）显著高于对照组（P<0.05），而白蛋白（ALB）和球蛋白（GLB）水平低于对照组（P<0.05）。NICCD组与对照组在空腹血糖（FBG）、空腹胰岛素、C肽（C-P）、胰岛素抵抗指数稳态模型评估（HOMA-IR）、空腹β细胞功能（FBCI）和HOMAβ细胞功能指数（HBCI）方面的差异不显著（P>0.05）。在NICCD患者中，发现FBG与空腹胰岛素之间存在线性相关性（P<0.001），FBG与C-P之间也存在线性相关性（P=0.001）。空腹胰岛素（P=0.023）、HOMA-IR（P=0.023）、FBCI（P=0.049）和HBCI（P=0.048）与ALT水平的升高呈正相关。NICCD组与对照组之间的胰岛β细胞功能无差异。肝功能障碍可能与NICCD患者的胰岛β细胞功能相关。
