Amasyali Akin S, Groegler Jason, Alsyouf Muhannad, Stokes Phillip, Baldwin D Duane, Abourbih Samuel
Department of Urology, Loma Linda University Health, Loma Linda, California.
J Endourol Case Rep. 2019 Aug 30;5(3):128-130. doi: 10.1089/cren.2019.0023. eCollection 2019.
Autosomal dominant polycystic kidney disease is the most prevalent hereditary renal disease, associated with progressive renal insufficiency, usually leading to dialysis. It is rarely diagnosed with other renal abnormalities. We present a case of a 35-year-old woman with a duplicated left polycystic kidney, who had recurrent pain and pyelonephritis because of ureteropelvic junction (UPJ) obstruction of the upper moiety. A 35-year-old female patient initially presented with left flank pain for 7 days. Evaluation demonstrated enlarged bilateral polycystic kidneys with the appearance of a duplicated system of the left kidney and UPJ obstruction of the upper moeity. She underwent endoscopic management, including balloon dilatation and stent placement. After stent removal she had no symptoms, and ultrasonography showed resolution of the upper pole hydronephrosis. Minimally invasive nephron sparing approaches for UPJ obstruction could delay the process of end-stage renal disease development in polycystic kidney disease patients who have additional congenital renal anomalies. Balloon dilatation should be considered as a feasible therapy for UPJ obstruction in polycystic kidney disease patients with duplicated systems.
常染色体显性多囊肾病是最常见的遗传性肾病，与进行性肾功能不全相关，通常会发展至需要透析。该病很少与其他肾脏异常同时被诊断出来。我们报告一例35岁女性，其左肾重复多囊肾，因上半部分肾盂输尿管连接部（UPJ）梗阻反复出现疼痛和肾盂肾炎。一名35岁女性患者最初因左侧胁腹疼痛7天前来就诊。评估显示双侧多囊肾增大，左肾呈重复系统表现且上半部分存在UPJ梗阻。她接受了内镜治疗，包括球囊扩张和支架置入。支架取出后她无症状，超声检查显示上极肾积水消退。对于合并其他先天性肾脏异常的多囊肾病患者，针对UPJ梗阻的微创保留肾单位方法可延缓终末期肾病的发展进程。对于存在重复系统的多囊肾病患者，球囊扩张应被视为治疗UPJ梗阻的一种可行方法。
