Woodruff Samantha A, Sontag Marci K, Accurso Frank J, Sokol Ronald J, Narkewicz Michael R
Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition and Digestive Health Institute, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO, United States.
Department of Epidemiology, Colorado School of Public Health, University of Colorado School of Medicine and Children's Hospital Colorado Aurora, CO, United States.
J Cyst Fibros. 2017 Jan;16(1):139-145. doi: 10.1016/j.jcf.2016.08.002. Epub 2016 Aug 20.
Prevalence and risks for elevated liver enzymes have not been studied systematically in children with CF identified by newborn screen.
298 CF children identified by newborn screen since 1982. AST, ALT and GGT tested at annual visits. Percent of children with 1 or ≥2 values of elevated AST, ALT and GGT determined. Relationship of liver enzymes to clinical factors or subsequent liver disease was analyzed RESULTS: At least one abnormal value for AST (63%), ALT (93%) and ALT ≥1.5× ULN (52%) occurred by 21years of age. Liver enzyme elevations were not correlated with CFTR mutation, meconium ileus or ethnicity. AST and GGT ≥1.5× ULN were associated with later advanced liver disease HR (CI) 6.53 (2.02-21.1) and 4.03 (1.15-13.45), respectively.
Elevated liver enzymes are common during childhood in CF patients identified by newborn screen. Elevated AST and GGT may be markers for risk of advanced liver disease.
通过新生儿筛查确诊的囊性纤维化（CF）患儿中，肝酶升高的患病率及风险尚未得到系统研究。
自1982年以来通过新生儿筛查确诊的298例CF患儿。每年随访时检测谷草转氨酶（AST）、谷丙转氨酶（ALT）和γ-谷氨酰转肽酶（GGT）。确定AST、ALT和GGT一项或多项值升高的患儿百分比。分析肝酶与临床因素或后续肝病的关系。结果：到21岁时，AST（63%）、ALT（93%）以及ALT≥1.5倍正常上限（ULN）（52%）至少有一项出现异常值。肝酶升高与CF跨膜传导调节因子（CFTR）突变、胎粪性肠梗阻或种族无关。AST和GGT≥1.5倍ULN与后期进展性肝病相关，风险比（HR）（置信区间）分别为6.53（2.02 - 21.1）和4.03（1.15 - 13.45）。
通过新生儿筛查确诊的CF患儿在儿童期肝酶升高很常见。AST和GGT升高可能是进展性肝病风险的标志物。
