更新于：2024-11-01

Cakut

先天性肾脏及泌尿系统异常

更新于：2024-11-01

基本信息

别名

CAKUT、Cakut、Congenital Anomalies Of Kidney And Urinary Tract

+ [4]

简介-

关联

项与先天性肾脏及泌尿系统异常相关的药物

Rilparencel

靶点-

作用机制

细胞替代物

在研机构

原研机构

在研适应症

非在研适应症

最高研发阶段临床1期

首次获批国家/地区-

首次获批日期1800-01-20

项与先天性肾脏及泌尿系统异常相关的临床试验

NCT06440499 / Not yet recruitingN/AIIT

Prenatal Ultrasonographic Screening of Congenital Anomalies of Kidney and Urinary Tract During the Third Trimester of Pregnancy

To evaluate the role of ultrasound in prenatal diagnosis of congenital anomalies of kidney and urinary system in third trimester of pregnancy.
Primay outcomes:
To determine incidence of congenital anomalies in kidney and urinary system in Third Timerter by ultrasound.

开始日期2024-07-01

申办/合作机构

Assiut University

NCT06534749 / Active, not recruitingN/AIIT

Ultrasound Screening of Congenital Anomalies of Kidney and Urinary Tract in Neonates

This cross sectional study will be conducted to investigate the incidence of the congenital anomalies of kidney and urinary tracts in neonates born or admitted at Tanta University Hospital.

开始日期2023-10-28

申办/合作机构

Tanta University

NCT05694169 / Recruiting临床1期

A Phase 1, Open-label, Supplemental Dosing Study of Participants With Chronic Kidney Disease Previously Treated With REACT (REGEN-015)

The purpose of this study is to evaluate the safety of supplemental REACT injections in participants with chronic kidney disease (CKD) who have previously received REACT treatment.

开始日期2023-07-11

申办/合作机构

ProKidney LLC

100 项与先天性肾脏及泌尿系统异常相关的临床结果

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100 项与先天性肾脏及泌尿系统异常相关的转化医学

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0 项与先天性肾脏及泌尿系统异常相关的专利（医药）

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1,044

项与先天性肾脏及泌尿系统异常相关的文献（医药）

2024-12-01·Pediatric Nephrology

Risk factors and outcome of BK polyomavirus infection in pediatric kidney transplantation

Article

作者: Chen, Jing ; Fang, Xiaoyan ; Liu, Feng ; Shen, Qian ; Sun, Yubo ; Zhang, Zhiqing ; Wang, Chunyan ; Xu, Hong ; Lin, Fang ; Chen, Rui ; Zhai, Yihui

BACKGROUNDBK polyomavirus (BKV) infection is a critical complication hindering graft survival after kidney transplantation. We aimed to investigate the risk factors and outcome of BKV infection in pediatric kidney transplantation.METHODSThe clinical and follow-up data of pediatric kidney transplant recipients at the Children's Hospital of Fudan University from Jan 2015 to June 2023 were retrospectively analyzed.RESULTSA total of 217 patients were included in the study with mean follow-up time of 24.3 ± 19.9 months. The mean age at transplantation was 9.7 ± 4.2 years. The patient survival rate and graft survival rate were 98.2% and 96.8%, respectively. Twenty-nine patients (13.4%) developed BKV infection, which was detected at 5.8 ± 3.2 months after transplantation. Among these 29 patients with BKV infection, 8 patients (3.6%) developed BKV nephropathy (BKVN), which was diagnosed at 8.3 ± 2.9 months after transplantation, and 2 patients developed graft failure eventually. Compared with the non-BKV infection group (eGFR 76.7 ± 26.1 mL/min/1.73 m²) and BKV infection without BKVN group (eGFR 85.2 ± 23.8 mL/min/1.73 m²), BKVN group had lowest eGFR during follow-up (33.5 ± 11.0 ml/min/1.73 m², P < 0.001). Younger age at transplant (OR 0.850, 95%CI 0.762-0.948, P = 0.005), CAKUT disease of primary etiology (OR 2.890, 95%CI 1.200-6.961, P = 0.018), and CMV negative recipient serostatus before transplantation (OR 3.698, 95%CI 1.583-8.640, P = 0.003) were independent risk factors for BKV infection.CONCLUSIONSIncidence of BKV infection is quite high within 12 months after pediatric kidney transplantation and children with BKVN have poor graft function. Younger age at transplant, CAKUT disease, and CMV negative recipient serostatus before transplantation increase the risk of BKV infection after kidney transplantation.

2024-11-01·International Journal of Surgery Case Reports

Urethral duplication associated with epispadias: Case report

Article

作者: Mohammed, Mensur ; Gebreselassie, Hana Abebe ; Girma, Hiwote ; Ahmed, Mohamed ; Merga, Tafese Gudissa

INTRODUCTION AND IMPORTANCEUrethral duplication is a rare congenital urinary tract anomaly. Its association with male epispadias is extremely rare. Due to various anatomical types, patients with urethral duplication can have a wide range of clinical presentations. Managing epispadias associated with urethral duplication involves different techniques and one should identify the functional urethra before proceeding with the surgery. It is associated with better functional outcomes than bladder epispadias exstrophy.CASE PRESENTATIONWe report a case of a 4-year-old male child presented with a complaint of urinary incontinence. After evaluation, he was diagnosed with Urethral duplication associated with penopubic epispadias. Epispadias repair with urethral advancement was done and the child had a smooth postoperative course.CLINICAL DISCUSSIONBoth urethral duplication and epispadias are rare congenital urinary tract anomalies. Urethral duplication associated with epispadias is extremely rare. Patient clinical presentation varies from incontinence to incidental findings. The diagnosis of urethral duplication in epispadias patients is usually missed preoperatively and even intraoperatively. The type of procedure going to be done depends on the type of urethral duplication. Management of these patients is associated with good functional outcomes.CONCLUSIONEven though urethral duplication associated with epispadias is a rare congenital malformation, it should be carefully searched in male epispadias.

2024-10-01·Journal of Pediatric Urology

Review – Renal transplantation for congenital urological diseases

Review

作者: Lorenzo, Armando J ; Rickard, Mandy ; Kim, Jin Kyu ; Santos, Joana Dos

INTRODUCTIONCongenital Anomalies of the Kidney and Urinary Tract (CAKUT) are structural disorders originating prenatally and present at birth. Affecting 4.2 to 1000 per 10,000 births globally, CAKUT includes conditions like posterior urethral valves, cloacal anomalies, and reflux nephropathy. These anomalies can lead to chronic kidney disease (CKD) or end-stage kidney disease (ESKD) in children, necessitating renal replacement therapy or transplantation.OBJECTIVESThis article aims to provide an updated perspective on pediatric kidney transplantation for children with CAKUT, emphasizing pre-transplant evaluation and management to optimize long-term outcomes.METHODSA comprehensive urologic evaluation is essential for children with ESKD being considered for kidney transplantation. Key pre-transplant investigations include kidney/bladder ultrasound (US), voiding cystourethrogram (VCUG), uroflowmetry, and urodynamics (UDS)/video-urodynamics (VUDS). Non-operative interventions such as pharmacotherapy and clean intermittent catheterization (CIC) are also considered. Surgical interventions, like augmentation cystoplasty or continent catheterizable channels, are evaluated based on individual patient needs.RESULTSKidney/bladder US and VCUG provide essential anatomical information. UDS offers comprehensive functional data, identifying hostile bladders needing pre-transplant optimization. Non-surgical measures like anticholinergics and CIC improve bladder function, while surgical options enhance compliance and capacity. Concurrent interventions during transplantation are feasible but require careful consideration of risks and benefits.DISCUSSIONChildren with CAKUT undergoing kidney transplantation exhibit comparable or better graft survival rates than those without CAKUT. However, those with lower urinary tract obstructions (LUTO) may have poorer long-term outcomes. UDS is crucial for evaluating bladder function pre-transplant, guiding the need for interventions. Long-term monitoring for urinary tract infections (UTIs) and bladder dysfunction is essential.CONCLUSIONOptimal outcomes in pediatric kidney transplantation for CAKUT patients require thorough pre-transplant evaluation and management, particularly for those with LUTO. Multidisciplinary approaches ensure careful monitoring and timely interventions, improving graft survival and quality of life for these patients.