背景：在患有泄殖腔畸形的儿童中，肾功能障碍是一个持续关注的问题，报告的发病率高达50%。存在多种可能损害肾功能的因素。我们的机构在该人群中遵循严格的肾脏保护协议。这些患者肾功能不全的发生率未知。
目的：我们的目的是评估在泄殖腔畸形患儿队列中实施该方案时肾功能不全的发生率。
方法：我们回顾了一个前瞻性收集的数据库，该数据库是自实施肾脏保护方案以来在单一机构管理的泄殖腔畸形儿童。这包括定期的实验室评估，适当选择全泌尿生殖系统动员或泌尿生殖系统分离，对有即将发生的肾功能不全或尿潴留迹象的患者进行主动成像，并在必要时进行早期导尿教学和实施。使用Schwartz公式计算肾小球滤过率(GFR),并根据标准定义分配CKD等级。肾功能障碍定义为CKD3b级或更高，需要肾脏替代疗法（RRT）或移植。计算了描述性统计数据。
结果：共有105名儿童根据该方案接受治疗，中位随访时间为4.2年[IQR：2.0-5.9]。在最近的随访中，有6名儿童（5.7％）患有肾功能不全；这些儿童中，只有3例(2.9%)从初始评估时的肾功能正常发展为肾功能不全(表).到目前为止，尚无肾功能正常的儿童需要透析或移植。
结论：以前的文献估计泄殖腔患者肾功能不全的发生率高达50％；相反，我们证实,在采用严格的肾脏保护方案后,女孩的肾功能不全进展率为2.9%.大多数发生肾功能不全的儿童在表现出肾脏功能失调。这表明，在儿童早期可能有可能保留肾功能，多学科肾脏保护协议。
结论：在我们严格的肾脏保护方案后的泄殖腔畸形患者队列中，进行性肾功能不全的发生率较低,为2.9%.大多数继续肾功能障碍的人表现为肾功能受损。
BACKGROUND: In children with cloacal malformations, renal dysfunction is a constant concern, with reported incidence as high as 50%. Multiple factors exist that may impair renal function. Our institution follows a strict renal protection protocol in this population. Incidence of renal dysfunction in these patients is unknown.
OBJECTIVE: We aimed to evaluate incidence of renal dysfunction while implementing this protocol in a cohort of children with cloacal malformation.
METHODS: We reviewed a prospectively collected database of children with cloacal malformations managed at a single institution since implementation of a renal protection protocol. This involves regular laboratory evaluation, appropriate selection of total urogenital mobilization or urogenital separation, proactive imaging in patients with signs of impending renal dysfunction or urinary retention, and early catheterization teaching and implementation if necessary. Glomerular filtration rate (GFR) was calculated with the Schwartz formula and CKD grades assigned per standard definitions. Renal dysfunction was defined as CKD grade 3b or higher, need for renal replacement therapy (RRT) or transplantation. Descriptive statistics were computed.
RESULTS: A total of 105 children were managed under this protocol with a median follow-up of 4.2 years [IQR: 2.0-5.9]. Six children (5.7%) had renal dysfunction at most recent follow-up; of these children, only three (2.9%) progressed from normal renal function at initial evaluation to renal dysfunction (Table). No child with normal presenting renal function thus far has progressed to require dialysis or transplantation.
CONCLUSIONS: Previous literature estimated rates of renal dysfunction in cloaca patients as high as 50%; in contrast, we demonstrate a rate of progression to renal dysfunction of 2.9% in girls following a strict renal protection protocol. Most children who developed renal dysfunction had dysfunctional kidneys on presentation. This suggests that preservation of renal function may be possible in early childhood with a strict, multi-disciplinary renal protection protocol.
CONCLUSIONS: In our cohort of patients with cloacal malformations following a strict renal protection protocol, incidence of progressive renal dysfunction is low at 2.9%. Most who go on to renal dysfunction present with impaired renal function.
