背景:儿童慢性肝病(CLD),通常导致肝硬化和终末期肝病(ESLD)。CLD在管理和预后方面提出了重大挑战。评估身体成分,包括少肌症,越来越被认为在理解这一人群的结果方面很重要。
方法:我们进行了一项前瞻性观察研究,涉及2至18岁的ESLD儿童等待肝移植。社会人口学,临床,收集了实验室数据,使用生物电阻抗分析(BIA)评估身体成分。使用阑尾骨骼肌质量(aSMM)和无脂质量(FFM)的年龄特异性截止点来定义肌肉减少症。
结果:该研究包括57名儿童(42.1%的女孩,57.9%的男孩;中位年龄:10.9岁)患有肝硬化。其中11人(19.3%)在研究期间死亡。在参与之前生活与终末期肝病的平均持续时间为5.43年[IQR:3.32,8.39]。最常见的病因是胆道闭锁(24.6%),其次是隐源性(22.8%)。死亡儿童表现出明显较高的肌少症患病率,与幸存者相比,基础代谢率和生长评分较低(P<0.05),(771.0与934.0,P=0.166)(65.0与80.5,P=0.005)。死亡儿童的全身和肢体指定的瘦体重较低,虽然没有统计学意义。同样,死亡儿童的总矿物质(90%正常)和骨矿物质含量较低,仅在水与FFM百分比中观察到显著差异(72.5vs.73.1,P=0.009)。
结论:本研究强调了ESLD患儿中肌肉减少症的高患病率及其与不良结局的关系。包括死亡率。生物阻抗分析作为一个有前途的,非侵入性方法评估小儿ESLD的身体成分,保证进一步调查并融入临床实践。
BACKGROUND: Chronic liver disease (CLD) in children, often leads to cirrhosis and end-stage liver disease (ESLD). CLD poses significant challenges in management and prognosis. Assessing body composition, including sarcopenia, is increasingly recognized as important in understanding outcomes in this population.
METHODS: We conducted a prospective observational study, involving children aged 2 to 18 years with ESLD awaiting liver transplantation. Socio-demographic, clinical, and laboratory data were collected, and body composition was assessed using Bioelectrical Impedance Analysis (BIA). Sarcopenia was defined using age-specific cut-off points for appendicular skeletal muscle mass (aSMM) and fat-free mass (FFM).
RESULTS: The study included 57 children (42.1% girls, 57.9% boys; median age: 10.9 years) with liver cirrhosis. Of them 11 (19.3%) died during the study. The mean duration of living with end-stage liver disease prior to participation was 5.43 years [IQR: 3.32, 8.39]. The most common etiology was biliary atresia (24.6%), followed by cryptogenic (22.8%). Deceased children exhibited significantly higher sarcopenia prevalence, lower basal metabolic rate and growth scores compared to survivors (P < 0.05), (771.0 vs. 934.0, P = 0.166) (65.0 vs. 80.5, P = 0.005). Total body and limb-specified lean mass were lower in deceased children, although not statistically significant. Similarly, total mineral (90% normal) and bone mineral content were lower in deceased children, with a significant difference observed only in water-to-FFM percentage (72.5 vs. 73.1, P = 0.009).
CONCLUSIONS: This study highlights the high prevalence of sarcopenia among children with ESLD and its association with adverse outcomes, including mortality. Bioimpedance analysis emerges as a promising, non-invasive method for assessing body composition in pediatric ESLD, warranting further investigation and integration into clinical practice.