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儿童先天性孤立功能性肾相关的异常情况。

The anomalies associated with congenital solitary functioning kidney in children.

作者信息

Akl Kamal

机构信息

College of Medicine, Jordan University Hospital, Amman, Jordan.

出版信息

Saudi J Kidney Dis Transpl. 2011 Jan;22(1):67-71.

Abstract

The aim of this study was to determine the incidence of associated urological and non-urological anomalies as well as the renal outcome in patients with a congenital solitary func-tioning kidney (CSFK). A retrospective review of 30 consecutive cases of CSFK seen at the pediatric renal service at the Jordan University Hospital between 2004 and 2008 was performed. There were 20 males and 10 females, whose ages ranged from five days to 14 years. In 20 patients (67%), the left kidney was absent. Associated anomalies were detected in 23 (77%) of the 30 patients; urological anomalies accounted for 47% (14/30) and non-urological anomalies were found in 19/30 (53%) patients. The latter included anomalies of the ear, nose and throat (ENT) in 9/30 (30%), musculoskeletal system (one with hypermobile joints) in 8/30 (27%), gastrointestinal (GI) in 7/30 (23%), cardiovascular (CV) in 4/30 (13%) and dermatological with epidermolysis bullosa, endocrine (euthyroid goiter) and gynecological (cervical cyst) in one patient each (3%). Proteinuria was seen in 6/30 (20%) and hypertension in 2/30 (7%) patients. Chronic renal failure (CRF) was seen in 6/30 (20%) patients, of whom three had end-stage renal failure (ESRF). CRF was seen mainly in patients with more than two associated urological anomalies. Idiopathic hyperuricosuria was found in five of the six tested patients (83%). In our study, the most common associated anomalies with CSFK were urological. The presence of more than two associated urological anomalies increased the risk of CRF.

摘要

本研究的目的是确定先天性孤立功能肾(CSFK)患者泌尿系统及非泌尿系统相关异常的发生率以及肾脏转归情况。对2004年至2008年期间约旦大学医院儿科肾脏科连续收治的30例CSFK患者进行了回顾性研究。其中男性20例,女性10例,年龄范围为5天至14岁。20例(67%)患者左肾缺如。30例患者中有23例(77%)检测到相关异常;泌尿系统异常占47%(14/30),19/30(53%)的患者存在非泌尿系统异常。后者包括耳鼻喉(ENT)异常9/30(30%)、肌肉骨骼系统异常(1例关节活动过度)8/30(27%)、胃肠道(GI)异常7/30(23%)、心血管(CV)异常4/30(13%)以及皮肤病(大疱性表皮松解症)、内分泌(甲状腺肿)和妇科(宫颈囊肿)各1例(3%)。6/30(20%)的患者出现蛋白尿,2/30(7%)的患者出现高血压。6/30(20%)的患者出现慢性肾衰竭(CRF),其中3例为终末期肾衰竭(ESRF)。CRF主要见于合并两种以上泌尿系统相关异常的患者。6例接受检测的患者中有5例(83%)发现特发性高尿酸尿症。在我们的研究中,CSFK最常见的相关异常是泌尿系统异常。合并两种以上泌尿系统相关异常会增加CRF的风险。

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