Alamir Abdulrahman, Al Rasheed Soud A, Al Qahtani Abdullah T, Almosa Mohammad S, Aljehani Nawaf D, Alanazi Eid D, Almutairi Khalid A
Department of Pediatric Nephrology, King Abdullah Specialized Children's Hospital, Riyadh, SAU.
College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, SAU.
Cureus. 2023 Apr 22;15(4):e37994. doi: 10.7759/cureus.37994. eCollection 2023 Apr.
Background Multicystic dysplastic kidney (MCDK) is a type of kidney dysplasia consisting of many irregular, various-sized cysts divided by dysplastic renal tissue, which negatively impacts kidney function. MCDK is one of the most common renal congenital disorders seen in antenatal ultrasounds. The typical prognosis of MCDK is complete or partial involution that starts antenatally and continues postnatally. The aim of the study was to shed light on the overall outcome of patients with MCDK. Methods We retrospectively collected data on MCDK patients from 2016 until 2022 at King Abdulaziz Medical City, Ministry of National Guard Health Affairs in Saudi Arabia, Riyadh. The data included the recording of epidemiological data, radiological and laboratory reports, and the presence of urological or non-urologically associated anomalies. Results A total of 57 patients with MCDK were reviewed. Seven of them were excluded due to the diagnosis of bilateral MCDK, which was incompatible with life. Of the remaining 50 patients, the right kidney was affected in 52% of them. Most patients were diagnosed antenatally (98%). The mean duration of follow-up for the study was 48 months. Vesicoureteral reflux (VUR) was detected in 22% of the total sample. Overall, 90% of the patients underwent kidney involution. A small percentage had genitourinary anomalies (20%), while a larger percentage (48%) had extrarenal abnormalities. Conclusion Multicystic dysplastic kidney disease is relatively common in children. The prognosis is affected by the presence of genitourinary and non-genitourinary anomalies. Patients have an overall good prognosis with conservative management. Antenatal screening, diagnosis, and long-term nephrological follow-up are essential for the optimal management of patients.
背景 多囊性发育不良肾（MCDK）是一种肾发育不良，由许多不规则、大小各异的囊肿组成，其间被发育异常的肾组织分隔，对肾功能有负面影响。MCDK是产前超声检查中最常见的肾先天性疾病之一。MCDK的典型预后是在产前开始并在产后持续的完全或部分退化。本研究的目的是阐明MCDK患者的总体结局。方法 我们回顾性收集了2016年至2022年在沙特阿拉伯利雅得国民卫队卫生事务部阿卜杜勒阿齐兹国王医疗城的MCDK患者的数据。数据包括流行病学数据记录、放射学和实验室报告，以及是否存在泌尿系统或非泌尿系统相关异常。结果 共审查了57例MCDK患者。其中7例因诊断为双侧MCDK而被排除，双侧MCDK与生命不相容。在其余50例患者中，52%的患者右侧肾脏受累。大多数患者在产前被诊断（98%）。本研究的平均随访时间为48个月。在总样本中，22%检测到膀胱输尿管反流（VUR）。总体而言，90%的患者肾脏发生退化。一小部分患者有生殖泌尿系统异常（20%），而一大部分患者（48%）有肾外异常。结论 多囊性发育不良肾病在儿童中相对常见。预后受生殖泌尿系统和非生殖泌尿系统异常的影响。患者通过保守治疗总体预后良好。产前筛查、诊断和长期肾病随访对于患者的最佳管理至关重要。
