Caldeira Filipa, Juvandes Carla, Pinto Margarida, Braga Manuela, Calhau Paulo
Serviço de Pediatria, Hospital Garcia de Orta, Almada, Portugal.
Acta Med Port. 2011 Dec;24 Suppl 2:549-56. Epub 2011 Dec 31.
Multicystic dysplastic kidney (MDK) is one of the most common congenital anomalies of the urinary tract and the main cystic renal disease in children. Our institution follow-up protocol of MDK uses, as has been performed more widely in the last two decades, a conservative attitude.
To characterize MDK cases followed in our Outpatient Clinic of Pediatric Nephrology, evaluate their progress and reflect on the protocol adopted.
36 MDK patients observed between January 1995 and December 2009 were included in this retrospective study and followed-up. In 35 children the diagnosis was the result of prenatal ultrasound. All children underwent a systematic protocol, including conservative treatment (no surgery) and periodic clinical, laboratory and ultrasound evaluation. All children underwent a MAG3 renogram or a 99mTc-DMSA renal scintigraphy and a voiding cystourethrography, only repeated in cases of vesicoureteral reflux (VUR).
Eighteen children (50%) are male. The median age of first visit was four weeks. The median of follow-up was 65 months. The MDK was in the left kidney in 20 children (56%). The median age of onset was 10 and six weeks Contralateral nephro-urologic pathology was identified in 10 cases (28%): seven children with VUR (grade ≥ IV in three), two with ureteropelvic junction obstruction (UPJO) and one with mild pelvic dilatation. There was involution of dysplastic kidney in 27 cases (75%), partial in 24 and total in three. The involution rate was higher in the first 36 months. There was a progressive compensatory hypertrophy of the contralateral kidney, with the highest rate in the first two years of life. There was resolution of VUR in five of the seven units reflux (three spontaneous and two after ureteral reimplantation). The two children with UPJO underwent surgery. There was no malignant degeneration and there was not carry out any nephrectomy of dysplastic kidney. Urinary infection occurred in nine children (25%), three of which have VUR and two with UPJO. There were no cases of hypertension or decreased glomerular filtration rate.
The results of this study are generally consistent with other casuistics and confirm the fairness of a conservative attitude in the approach of children with MDK. This clinical approach is safe, with a minimum incidence of complications, with tendency to involution of dysplasic kidney being the rule.
多囊性发育不良肾(MDK)是最常见的先天性泌尿道异常之一,也是儿童主要的囊性肾病。在过去二十年中,我们机构对MDK的随访方案采用了更为广泛实施的保守态度。
描述在我们儿科肾脏病门诊随访的MDK病例,评估其进展并反思所采用的方案。
本回顾性研究纳入了1995年1月至2009年12月期间观察到的36例MDK患者并进行随访。35名儿童的诊断是产前超声检查的结果。所有儿童均接受了系统方案,包括保守治疗(不手术)以及定期的临床、实验室和超声评估。所有儿童均接受了MAG3肾图或99mTc-DMSA肾闪烁显像以及排尿性膀胱尿道造影,仅在膀胱输尿管反流(VUR)病例中重复进行。
18名儿童(50%)为男性。首次就诊的中位年龄为4周。随访的中位时间为65个月。20名儿童(56%)的MDK位于左肾。发病的中位年龄为10周和6周。10例(28%)发现对侧肾泌尿生殖系统病变:7名儿童有VUR(3例为IV级及以上),2例有肾盂输尿管连接部梗阻(UPJO),1例有轻度肾盂扩张。27例(75%)发育不良肾发生退化,24例为部分退化,3例为完全退化。退化率在前36个月较高。对侧肾有进行性代偿性肥大,在生命的头两年发生率最高。7例反流单位中有5例VUR得到缓解(3例自发缓解,2例输尿管再植术后缓解)。2例UPJO患儿接受了手术。未发生恶性变,未对发育不良肾进行任何肾切除术。9名儿童(25%)发生尿路感染,其中3例有VUR,2例有UPJO。无高血压或肾小球滤过率降低的病例。
本研究结果与其他病例系列总体一致,证实了对MDK患儿采取保守态度的合理性。这种临床方法是安全的,并发症发生率最低,发育不良肾退化趋势是普遍规律。