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双丁域含蛋白2 (DCDC2) 相关的新生儿硬化胆道炎的门口高血压

Prabhsaran Kaur¹, Bikrant Bihari Lal¹, Deepa Janakiraman²

¹Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.

Journal of pediatric gastroenterology and nutrition

|November 18, 2024

概括

患有DCDC2基因突变的儿童会发展为新生儿硬化胆管炎和门性高血压. 许多人经历了早期的水出血,由于难以治疗的水,经常需要肝移植.

科学领域:

遗传学是一个遗传学.
儿科胃肠病学儿科胃肠病学
肝病学肝病学是一种肝病学.

背景情况:

双皮质蛋白域含蛋白2 (DCDC2) 基因的突变与新生儿硬化性脑膜炎 (NSC) 有关.
NSC经常与门高血压 (PHTN) 相关,这是一种涉及门静脉系统压力升高的情况.

研究的目的:

系统地评估PHTN的发病率,水出血,以及与DCDC2相关的NSC的儿科患者的治疗结果.
评估静脉动脉疗的疗效和肝脏移植的必要性在这个队列.

主要方法:

这项研究包括了在DCDC2基因中诊断出同卵性或复合异卵性变异的儿童.
数据收集的重点是PHTN的存在,水出血的发生,静脉动脉的内镜治疗和肝移植 (LT) 状态.

主要成果:

所有14名患有DCDC2相关NSC的儿童都呈现了PHTN.
很大一部分人 (57.1%) 在3岁的中位年龄经历了水出血.
78.6%的高风险静脉动脉患者进行了内疗,成功率可变;由于耐火静脉动脉和再出血,三名患者需要LT.

结论:

在DCDC2的变体与高发病率的PHTN和早期发病的水出血与NSC的儿童有关.
静脉的治疗可能具有挑战性,存在相当大的再次出血风险和经常需要进行肝移植.

关键词:

肝移植肝移植肝移植单一的胆固醇中毒.新生儿胆固醇的形成.流体变形性出血流体变形出血

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