Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease
Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease
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DOI：
10.1111/j.1478-3231.2007.01595.x
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发表时间：
2008-02-01
影响因子：
6.7
通讯作者：
Everson, Gregory T.
中科院分区：
文献类型：
--
作者：
Hoevenaren, Inge A.;Wester, Ruth;Everson, Gregory T.
本研究的目的是比较孤立性多囊肝病（PCLD）患者与多囊肝和常染色体显性多囊肾病（ADPKD）患者的临床特征。研究方法：通过ICD-10编码从丹佛（美国）的科罗拉多大学医院和奈梅亨（荷兰）的Radboud大学医院的临床记录中识别病例。要纳入本分析，患者必须在到我们诊所就诊的六年内首次诊断为PCLD。对病历中的人口统计学信息、病史、体格检查、症状、并发症、实验室和影像学结果、治疗和结局进行了审查。结果：在总共94名患者中，53名患者符合我们进入本研究的标准，19名为PCLD，34名为ADPKD。从PCLD诊断到临床表现的平均时间间隔，PCLD为1.21年，ADPKD为2.76年（P=NS）。在PCLD和ADPKD中，PCLD与女性相关。与ADPKD患者相比，PCLD患者有更多的肝囊肿（P=0.031）和更大的肝囊肿（P=0.0051），但相关的发病率较低。PCLD患者行肝囊肿去顶减压术的频率更高（57.9% vs.23.5%，P=0.012）。然而，ADPKD患者中严重的肝脏并发症（足以考虑肝移植）更常见（0/19 vs. 6/34，P
The goal of this study was to compare the clinical features of patients with isolated polycystic liver disease (PCLD) with those of patients with polycystic liver and autosomal dominant polycystic kidney disease (ADPKD). Methods: Cases were identified from clinical records at the University of Colorado Hospital in Denver (USA) and at the Radboud University Hospital in Nijmegen (the Netherlands) by ICD-10 codes. To be included in this analysis, patients had to have an initial diagnosis of PCLD within six years of presentation to our clinics. Medical records were reviewed for demographic information, medical history, physical examination, symptoms, complications, laboratory and imaging results, therapy and outcomes. Results: Out of a total of 94, 53 patients met our criteria for entering this study, 19 with PCLD and 34 with ADPKD. The mean time interval from diagnosis of PCLD to presentation in our clinics was 1.21 years for PCLD and 2.76 years for ADPKD (P=NS). PCLD was associated with female gender in both PCLD and ADPKD. Patients with PCLD had greater numbers (P=0.031), and larger sizes of liver cysts (P=0.0051), but had less associated morbidities than patients with ADPKD. Liver cyst decompressions were performed more frequently in PCLD patients (57.9 vs. 23.5%, P=0.012). However, serious hepatic complications, sufficient to require consideration of liver transplantation, were more frequent in patients with ADPKD (0/19 vs. 6/34, P