阿拉吉耶综合征患儿胆汁淤积性瘙痒的管理：病例报告及文献综述
Management of cholestatic pruritus in children with Alagille syndrome: Case report and literature review.
作者信息
Ben Ameur S, Chabchoub I, Telmoudi J, Belfitouri Y, Rebah O, Lacaille F, Aloulou H, Mehrzi A, Hachicha M
机构信息
Pediatric department, Hedi Chaker Hospital, El ain street km 0,5, 3029 Sfax, Tunisia; Faculty of medicine, university of Sfax, Tunisia.
Pediatric department, Hedi Chaker Hospital, El ain street km 0,5, 3029 Sfax, Tunisia.
出版信息
Arch Pediatr. 2016 Dec;23(12):1247-1250. doi: 10.1016/j.arcped.2016.09.004. Epub 2016 Oct 27.
Alagille syndrome causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol. Drug therapy in addition to surgical intervention may be effective in many patients in reducing serum bile acids, cholesterol levels, pruritus, and skin xanthomas. In this report, we describe a child with Alagille syndrome who presented with severe pruritus and xanthomas as a consequence of severe hypercholesterolemia and discuss the treatment modalities.
阿拉吉尔综合征由于胆汁酸和胆固醇潴留，可导致顽固性瘙痒和毁容性黄瘤。除手术干预外，药物治疗可能对许多患者有效，可降低血清胆汁酸、胆固醇水平、瘙痒和皮肤黄瘤。在本报告中，我们描述了一名患有阿拉吉尔综合征的儿童，该患儿因严重高胆固醇血症出现严重瘙痒和黄瘤，并讨论了治疗方式。
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