Clinical characteristics of congenital renal anomalies in children
Fang Xiang , Gao Chunlin , Xia Zhengkun,et al
(1. Department of Pediatrics, Jinling Hospital, Medical School Of Nanjing University, Jiangsu, Nanjing 210002, China; 2. Department of Information, Jinling Hospital, Medical School Of Nanjing University, Jiangsu, Nanjing 210002, China)
Abstract: 【Abstract】 Objective To investigate the clinical characteristics, classification, ultrasound diagnosis, clinical management and pathological characteristics. Method A retrospective analysis was performed on children with congenital renal anomalies who were diagnosed by renal and ureteral ultrasonography in Jinling Hospital from January 2010 to December 2015. The clinical data of the cases were collected, including gender, age, diagnosis, initial symptoms, combined symptoms, ?rst and multiple ultrasound examination results, estimated glomerular ?ltrationrate (eGFR), chronic kidney diseases (CKD) staging, renal pathology, genetic testing, etc. Result A total of 470 cases were retrieved, 299 male and 171 female (male: female =1.75∶1), agedmedian 11 years (0-18 years ), 0-4 years (57 cases), 5-9 years (132 cases), 10-14 years (113 cases), 15-18 years (168 cases). Renal cysts (49.6%, 233/470) were the most common renal dysplasia which could be single or multiple. Single renal cysts were mostly asymptomatic. Multiple renal cysts were found in 48 cases (20.6%, 48/233). 106 cases (22.6%, 106/470) were small kidney, 32 cases were left kidney, 52 cases were right kidney, and 22 were bilateral kidney. 56 cases (11.9%, 56/470) with renal agenesis, 30 cases with duplex kidney (6.4%, 30/470), ectopic kidney, sponge kidney, horseshoe kidney, and renal fusion, accounted for 45 patients (9.5%, 45/470), 44 cases (44/470, 9.4%) with nephrolithiasis/hydronephrosis, and 5 cases with ureteral malformation (1.1%, 5/470). 97 hospitalized children were followed up for 6 months to 10 years. There were 49 cases (50.5%, 49/97) with CKD, including 17 cases (17.5%, 17/97) with eGFR<30 ml/min. The primary symptoms of the children were: CKD with/without anemia in 28 cases, nephrotic syndrome in 28 cases, and hematuria with/without proteinuria in 20 cases, isolated proteinuria in 17 cases. Renal biopsy was performed in 30 cases, 4 cases of Alport syndrome, 9 cases of focal segmental glomerulosclerosis, 8 cases of mesangial proliferative glomerulosclerosis, 3 cases of chronic interstitial nephritis, 4 cases of IgA, 1 case of IgM, and 1 case of glomerular sparse glomeruli. Conclusion Congenital renal Anomalies are the common cause of CKD and even end-stage renal disease in children. Renal cysts are the most common renal dysplasia, most of them are solitary and asymptomatic, which require long-term follow-up. Renal ultrasonography is often the most commonly used non-invasive method for the ?rst diagnosis of children. Renal pathology and genetic testing are helpful for the diagnosis.
2022, Vol. 10 
