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持续性瘙痒是常染色体显性遗传性多囊肾病肝脏受累的一种罕见且可能严重的表现。

Persistent pruritus as a rare and potentially serious manifestation of liver involvement in autosomal dominant polycystic kidney disease.

作者信息

García-Prieto Ana, Torres Esther, Hernández Andrés, Barbieri Diego, Vega Almudena

机构信息

Nephrology Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

出版信息

Clin Kidney J. 2018 Jul 6;12(3):380-381. doi: 10.1093/ckj/sfy057. eCollection 2019 Jun.

Abstract

Polycystic liver disease (PCLD) is the most frequent extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD), usually asymptomatic. We present a patient with ADPKD who developed cholestatic pruritus due to bile duct compression of multiple liver cysts. Because of severity of pruritus she received treatment with lanreotide to reduce liver volume and she was studied to be included in the liver transplant waiting list. She evolved favourably with medical treatment and she is now asymptomatic. In conclusion, persistent pruritus is a rare but potentially serious manifestation of PCLD, so it should be taken into account in patients with ADPKD.

摘要

多囊肝病(PCLD)是常染色体显性多囊肾病(ADPKD)最常见的肾外表现,通常无症状。我们报告一名患有ADPKD的患者,其因多个肝囊肿压迫胆管而出现胆汁淤积性瘙痒。由于瘙痒严重,她接受了兰瑞肽治疗以减少肝脏体积,并接受评估以列入肝移植等待名单。她经药物治疗后病情好转,目前无症状。总之,持续性瘙痒是PCLD一种罕见但可能严重的表现,因此在ADPKD患者中应予以考虑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4cff/6543959/525fdcd73325/sfy057f1.jpg

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