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Alagille’s syndrome

Introduction

Alagille’s syndrome is a syndrome in which the number of intrahepatic bile ducts is markedly reduced, bile stasis is present, and cardiovascular, skeletal, eye, facial, pancreatic and neurodevelopmental disorders may be accompanied.

The prevalence of the disease is reportedly one in 100,000, but the frequency is expected to be greater if the symptoms of the disease include milder…

简介

Alagille综合症是其中肝内胆管数量明显减少，存在胆汁淤积并且可能伴有心血管，骨骼，眼睛，面部，胰腺和神经发育障碍的综合征。

据报道该疾病的患病率为十万分之一，但如果该疾病的症状包括较轻的患者，则预计发病率会更高。

症状

Alagille综合征的临床症状通常在三个月内出现。黄疸和胆汁淤积的大多数病例在新生儿期持续存在，需要在胆道闭锁和无症状肝胆功能不全之间进行鉴别诊断。在儿童期后期，可以发现慢性肝病，在成年期以后。由于临床症状多种多样且可以不完全遗传，因此诊断可能很困难。

肝胆问题

大多数患者在婴儿期出现黄疸，胆汁淤积，瘙痒和进行性肝功能衰竭症状。但是，肝脏疾病的程度与其他器官的症状不成正比。可能发生肝肿大，血液检查会升高胆汁酸，直接胆红素，碱性磷酸酶和谷氨酰转肽酶，表明胆汁排泄受损。在大多数患者中观察到黄疸，超过一半的患者持续到儿童晚期。瘙痒是由胆汁淤积引起的，一些孩子在皮下…

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All of my photos are free for all to use any way you want (for non-profit uses of course).