Judge Parminder K, Harper Charlie H S, Storey Benjamin C, Haynes Richard, Wilcock Martin J, Staplin Natalie, Goldacre Raph, Baigent Colin, Collier Jane, Goldacre Michael, Landray Martin J, Winearls Christopher G, Herrington William G
Medical Research Council-Population Health Research Unit.
Clinical Trial Service Unit and Epidemiological Studies Unit, and.
J Am Soc Nephrol. 2017 Sep;28(9):2738-2748. doi: 10.1681/ASN.2017010084. Epub 2017 May 2.
Polycystic liver disease is a well described manifestation of autosomal dominant polycystic kidney disease (ADPKD). Biliary tract complications are less well recognized. We report a 50-year single-center experience of 1007 patients, which raised a hypothesis that ADPKD is associated with biliary tract disease. We tested this hypothesis using all England Hospital Episode Statistics data (1998-2012), within which we identified 23,454 people with ADPKD and 6,412,754 hospital controls. Hospitalization rates for biliary tract disease, serious liver complications, and a range of other known ADPKD manifestations were adjusted for potential confounders. Compared with non-ADPKD hospital controls, those with ADPKD had higher rates of admission for biliary tract disease (rate ratio [RR], 2.24; 95% confidence interval [95% CI], 2.16 to 2.33) and serious liver complications (RR, 4.67; 95% CI, 4.35 to 5.02). In analyses restricted to those on maintenance dialysis or with a kidney transplant, RRs attenuated substantially, but ADPKD remained associated with biliary tract disease (RR, 1.19; 95% CI, 1.08 to 1.31) and perhaps with serious liver complications (RR, 1.15; 95% CI, 0.98 to 1.33). The ADPKD versus non-ADPKD RRs for biliary tract disease were larger for men than women (heterogeneity <0.001), but RRs for serious liver complications appeared higher in women (heterogeneity <0.001). Absolute excess risk of biliary tract disease associated with ADPKD was larger than that for serious liver disease, cerebral aneurysms, and inguinal hernias but less than that for urinary tract infections. Overall, biliary tract disease seems to be a distinct and important extrarenal complication of ADPKD.
多囊肝病是常染色体显性遗传性多囊肾病（ADPKD）的一种已被充分描述的表现形式。胆道并发症则较少被认识到。我们报告了一家中心对1007例患者长达50年的经验，由此提出了一个假说，即ADPKD与胆道疾病有关。我们使用全英格兰医院事件统计数据（1998 - 2012年）对这一假说进行了检验，在这些数据中我们识别出了23454例ADPKD患者以及6412754例医院对照。对胆道疾病、严重肝脏并发症以及一系列其他已知的ADPKD表现的住院率进行了潜在混杂因素的校正。与非ADPKD医院对照相比，ADPKD患者的胆道疾病住院率更高（率比[RR]，2.24；95%置信区间[95%CI]，2.16至2.33）以及严重肝脏并发症住院率更高（RR，4.67；95%CI，4.35至5.02）。在仅限于维持性透析患者或肾移植患者的分析中，RR大幅降低，但ADPKD仍与胆道疾病有关（RR，1.19；95%CI，1.08至1.31），并且可能与严重肝脏并发症有关（RR，1.15；95%CI，0.98至1.33）。ADPKD与非ADPKD患者的胆道疾病RR在男性中比女性更大（异质性<0.001），但严重肝脏并发症的RR在女性中似乎更高（异质性<0.001）。与ADPKD相关的胆道疾病的绝对超额风险大于严重肝脏疾病、脑动脉瘤和腹股沟疝，但小于尿路感染。总体而言，胆道疾病似乎是ADPKD一种独特且重要的肾外并发症。
