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1096例患有代谢相关脂肪性肝病的儿童的长期死亡率和肝外结局:一项回顾性队列研究。

Long-term mortality and extrahepatic outcomes in 1096 children with MASLD: A retrospective cohort study.

作者信息

Schwimmer Jeffrey B, Thai Nhat Quang N, Noon Sheila L, Ugalde-Nicalo Patricia, Anderson Sabina R, Chun Lauren F, David Rhys S, Goyal Nidhi P, Newton Kimberly P, Hansen Eleanor G, Lin Bonnie, Shapiro Warren L, Wang Andrew, Yu Elizabeth L, Behling Cynthia A

机构信息

Division of Gastroenterology, Department of Pediatrics, Hepatology, and Nutrition, University of California San Diego School of Medicine, La Jolla, California, USA.

Department of Gastroenterology, Rady Children's Hospital, San Diego, California, USA.

出版信息

Hepatology. 2025 Apr 22. doi: 10.1097/HEP.0000000000001357.

DOI:10.1097/HEP.0000000000001357
PMID:40262118
Abstract

BACKGROUND AND AIMS

Metabolic dysfunction-associated steatotic liver disease (MASLD) is the most common chronic liver disease in children, but its long-term outcomes are poorly understood. This study aimed to quantify mortality rates, identify causes of death, and evaluate the incidence of cirrhosis and extrahepatic outcomes in children with pediatric-onset MASLD.

APPROACH AND RESULTS

The Longitudinal InVestigation Evaluating Results of Steatosis (LIVERS) study is a single-center, retrospective cohort study conducted at Rady Children's Hospital San Diego. We included 1096 children aged 2-18 years who were diagnosed with MASLD between 2000 and 2017 and followed for a mean of 8.5 years. Mortality was ascertained through the National Death Index, and comorbidities were assessed through follow-up research visits and medical records. Overall, 3.4% of children died, yielding a mortality rate of 398 per 100,000 person-years; nearly half of these deaths were liver-related. Male sex and lower HDL levels independently predicted increased mortality risk. The cumulative incidence of cirrhosis was 4.7%. High incidence rates of extrahepatic comorbidities were observed, including dyslipidemia (3664 per 100,000 person-years), hypertension (1901), obstructive sleep apnea (1185), and type 2 diabetes (911).

CONCLUSIONS

Pediatric MASLD is associated with significant premature mortality and a substantial burden of hepatic and extrahepatic comorbidities. These findings highlight the need for timely screening, early intervention, and long-term management strategies to improve outcomes for children with MASLD.

摘要

背景与目的

代谢功能障碍相关脂肪性肝病(MASLD)是儿童中最常见的慢性肝病,但其长期预后尚不清楚。本研究旨在量化死亡率、确定死亡原因,并评估儿童期发病的MASLD患儿肝硬化和肝外结局的发生率。

方法与结果

脂肪变性纵向评估结果(LIVERS)研究是在圣地亚哥拉迪儿童医院进行的一项单中心回顾性队列研究。我们纳入了2000年至2017年间被诊断为MASLD的1096名2至18岁儿童,平均随访8.5年。通过国家死亡指数确定死亡率,并通过随访研究访问和医疗记录评估合并症。总体而言,3.4%的儿童死亡,死亡率为每10万人年398例;其中近一半的死亡与肝脏有关。男性和较低的高密度脂蛋白水平独立预测死亡风险增加。肝硬化的累积发生率为4.7%。观察到肝外合并症的高发病率,包括血脂异常(每10万人年3664例)、高血压(1901例)、阻塞性睡眠呼吸暂停(1185例)和2型糖尿病(911例)。

结论

儿童MASLD与显著的过早死亡以及肝脏和肝外合并症的沉重负担相关。这些发现凸显了及时筛查、早期干预和长期管理策略对于改善MASLD患儿结局的必要性。

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Noninvasive scores are poorly predictive of histological fibrosis in paediatric fatty liver disease.
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J Pediatr Gastroenterol Nutr. 2024 Jan;78(1):27-35. doi: 10.1002/jpn3.12068. Epub 2023 Dec 29.
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A multisociety Delphi consensus statement on new fatty liver disease nomenclature.多学会专家共识:新的非酒精性脂肪性肝病命名。
Hepatology. 2023 Dec 1;78(6):1966-1986. doi: 10.1097/HEP.0000000000000520. Epub 2023 Jun 24.
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Cardiovascular disease risk in paediatric and young adult non-alcoholic fatty liver disease.儿童和青年非酒精性脂肪性肝病患者的心血管疾病风险
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