Shwachman-Diamond综合征肝脏病变研究进展
Journal of Rare Diseases（2022）
Abstract
Shwachman-Diamond综合征(SDS)是一种可累及胰腺、骨髓等全身多个脏器的常染色体隐性遗传病,肝脏受累也较常见.肝脏受累也可单独出现,主要表现为转氨酶升高、肝肿大.肝脏表现在婴幼儿期较明显,多数随年龄增长逐渐好转,但也有少数患儿并发肝硬化、肝衰竭,预后不良.本文主要总结SDS肝脏病变的流行病学、发病机制、临床特点及诊疗等方面的研究进展.
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Key words
shwachman-diamond syndrome,hepatopathy,clinical manifestations
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