Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease.
Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease.
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DOI：
10.1097/mpg.0b013e31824711b7
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发表时间：
2012-05
影响因子：
2.9
通讯作者：
Shneider BL
中科院分区：
文献类型：
--
作者：
Srinath A;Shneider BL
对已发表的先天性肝纤维化 (CHF) 自然史进行了检查，为常染色体隐性多囊肾病 (ARPKD) 的临床决策提供信息。对CHF、ARPKD、Caroli病、Caroli综合征和V型胆总管囊肿的数据进行系统文献检索，提取与门脉高压、感染、恶性肿瘤、死亡率和移植相关的数据。从 155 篇文章中提取了与 1230 名 CHF 患者相关的信息。诊断时的中位年龄和平均年龄分别为 2 岁和 11.2 岁。诊断后随访的中位时间和平均时间分别为 5.0 和 7.5 年（范围 0-38 年）。注意到门脉高压 (n = 409)、胆管炎 (n = 152) 和恶性肿瘤 (n = 21) 的后遗症。门脉高压的性质与其他儿科疾病相似（164 例有静脉曲张，74 例静脉曲张出血，81 例接受门体分流术）。肾移植后感染的 23 名儿童中，有 3 名记录在案的胆管炎导致死亡。 21 名患者罹患肝胆癌，其中大多数患有胆管癌 (n = 19)。胆管癌 (CCA) 在 40 岁以上患有 Caroli 综合征或孤立性 CHF 的个体中占主导地位，而非 ARPKD（CCA 诊断时的中位年龄和平均年龄分别为 70.3 岁和 60.1 岁；范围为 33-75 岁）。肝肾联合移植的数据相对较少。 ARPKD 的临床决策应反映对 CHF 引起的潜在问题的理解。公认的针对门脉高压的儿科特定方法是必要的，但必须考虑肾功能不全的阶段和潜在的肾移植计划。胆管炎是一个重大问题，需要前瞻性指导和认识。 CCA 虽然是一种可怕的并发症，但似乎并不是儿童时期的主要问题。肝脏和肝肾联合移植的适应证存在争议，值得进一步分析。
The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD). A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V choledochal cyst was performed to extract data related to portal hypertension, infection, malignancy, mortality, and transplantation. Information related to 1230 patients with CHF was extracted from 155 articles. Median and mean age at diagnosis were 2 and 11.2 years, respectively. Median and mean time followed after diagnosis were 5.0 and 7.5 years, respectively (range 0–38 years). Sequelae of portal hypertension (n = 409), cholangitis (n = 152), and malignancy (n = 21) were noted. The nature of the portal hypertension was similar to that in other pediatric conditions (164 with varices, 74 bleeding varices, 81 underwent portosystemic shunting). Documented cholangitis was fatal in 3 of 23 children who were infected after renal transplantation. Twenty-one patients developed hepatobiliary cancer, with the majority having cholangiocarcinoma (n = 19). Cholangiocarcinoma (CCA) was predominant in individuals older than 40 years with either Caroli syndrome or isolated CHF, not ARPKD (median and mean age at CCA diagnosis were 70.3 and 60.1 years, respectively; range 33–75 years). There was a relative paucity of data on combined liver-kidney transplantation. Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF. Accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation. Cholangitis is a major issue and necessitates anticipatory guidance and awareness. CCA, although a dreaded complication, does not appear to be a major issue during childhood. The indications for liver and combined liver-kidney transplantation are controversial and warrant further analysis.