Di Pinto Diana, Balestracci Alejandro, Dujovne Noelia, de Palma Isabel, Adragna Marta, Delgado Norma
Hospital Nacional de Pediatría "Prof. Dr. Juan P. Garrahan".
Arch Argent Pediatr. 2010 Aug;108(4):353-7. doi: 10.1590/S0325-00752010000400011.
Nephrourologic malformations in Turner syndrome are frequent, its diagnosis and follow-up is important in order to diminish the morbidity of this disease. The aim of this retrospective study was to analyze the nephrourologic pathology in 72 girls with Turner syndrome followed between 1989 and 2008 at Garrahan Hospital. The prevalence of nephrourologic involvement was 33% (24 patients). The most frequent findings were urinary system malformations, isolated (10 pacientes, 42%) or associated with renal malformations (9 patients, 37%); 5 patients (21%) had only renal malformations. Fifty percent of patients developed complications (8 urinary tract infection, 2 proteinuria and 2 arterial hypertension); however, none progressed to chronic renal failure. The prevalence of nephrourologic involvement was 33% and a half of these girls developed complications, our findings show the need of routine nephrological follow-up of girls with Turner syndrome and nephrourologic malformations.
特纳综合征中的肾泌尿系统畸形很常见，对其进行诊断和随访对于降低该疾病的发病率很重要。这项回顾性研究的目的是分析1989年至2008年期间在加拉汉医院随访的72例特纳综合征女孩的肾泌尿系统病理情况。肾泌尿系统受累的患病率为33%（24例患者）。最常见的发现是泌尿系统畸形，孤立存在（10例患者，42%）或与肾脏畸形相关（9例患者，37%）；5例患者（21%）仅有肾脏畸形。50%的患者出现了并发症（8例尿路感染、2例蛋白尿和2例动脉高血压）；然而，无人进展为慢性肾衰竭。肾泌尿系统受累的患病率为33%，其中一半女孩出现了并发症，我们的研究结果表明对患有特纳综合征和肾泌尿系统畸形的女孩进行常规肾脏随访的必要性。
