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儿童多囊性发育不良肾——需要保守和长期治疗。

Multicystic dysplastic kidney in children--a need for conservative and long term approach.

机构信息

Department of Pediatric Surgery, Advanced Pediatric Center, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India.

出版信息

Indian J Pediatr. 2009 Aug;76(8):809-12. doi: 10.1007/s12098-009-0117-y. Epub 2009 Apr 16.

DOI:10.1007/s12098-009-0117-y
PMID:19381503
Abstract

OBJECTIVE

We retrospectively studied our cases of Multicystic Dysplastic Kidney (MCDK). The review was aimed at identifying the pattern of the disease in Indian Scenario and the required management thereof.

METHODS

We studied the clinical, radiological and nuclear scan findings of 22 patients with unilateral MCDK. They were diagnosed and/or treated in our unit from 1999 to 2007. The diagnosis was achieved by Ultrasound and further confirmed by DMSA scans. Other ancillary investigations like Micturating cystourethrogram were done if indicated. These patients were followed and followup investigations consisted of renal ultrasound, blood pressure measurement, and urinalysis and blood biochemistry

RESULTS

A total of 22 patients (18 boys and 4 girls) with unilateral MCDK were investigated and followed for a mean period of 41 months. MCDK was detected on antenatal ultrasound only in 12(55%) and postnatally in 10(45%) babies. Mean age for postnatal diagnosis was 20 months. Follow up ultrasound revealed complete involution of MCDK in 3 patients and partial regression in 11 patients. The size of dysplastic kidney was unchanged in 4 patients and a further 4 patients underwent nephrectomy. Indications of nephrectomy were parental anxiety in 2, hypertension in 1 and palpable mass in 1.

CONCLUSION

Large proportion (45%) of patients in presented series are diagnosed post natally contrary to western world where more than 80% are diagnosed antenatally. Uncomplicated isolated MCDK carry good prognosis with nephrectomy required in only a few patients. Association with other urological anomalies in ipsilateral/contralateral genitourinary tract is important to identify as they have worse outcome in terms of ultimate renal function. All patients with simple/complex unilateral MCDK should be advised long term follow up for the possible development of hypertension and/or hyper infiltration injury.

摘要

目的

我们回顾性研究了多房性囊性发育不良肾(MCDK)的病例。本研究旨在确定印度人群中该病的发病模式及其治疗方法。

方法

我们研究了 2007 年在我院经超声诊断并经 DMSA 扫描证实的 22 例单侧 MCDK 患者的临床、放射影像学和核扫描资料。如果需要,还进行了排尿性膀胱尿道造影等辅助检查。这些患者接受了随访,随访检查包括肾脏超声、血压测量、尿液分析和血液生化检查。

结果

单侧 MCDK 患者共 22 例(男 18 例,女 4 例),平均随访 41 个月。12 例(55%)患者在产前超声检查时发现 MCDK,10 例(45%)在产后发现。产后诊断的平均年龄为 20 个月。随访超声检查显示,3 例 MCDK 完全退化,11 例部分退化,4 例肾脏大小无变化,4 例患者接受了肾切除术。行肾切除术的指征分别为父母焦虑 2 例、高血压 1 例、可触及肿块 1 例。

结论

与西方国家相比,本研究系列中,45%的患者为产后诊断,而西方国家中,80%以上的患者为产前诊断。单纯性孤立性 MCDK 预后良好,仅少数患者需要行肾切除术。同侧/对侧泌尿道畸形的合并与患者的最终肾功能转归相关,应注意识别,因为它们的肾功能结局较差。所有单纯性/复杂性单侧 MCDK 患者均应建议长期随访,以预防高血压和/或高滤过性损伤的发生。

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Natural history of patients with multicystic dysplastic kidney-what followup is needed?多囊性发育不良肾患者的自然病史——需要怎样的随访?
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Unilateral multicystic dysplastic kidney: long term outcomes.单侧多囊性发育不良肾:长期预后
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Routine voiding cystourethrography is of no value in neonates with unilateral multicystic dysplastic kidney.常规排尿性膀胱尿道造影对患有单侧多囊性发育不良肾的新生儿没有价值。
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