Urisarri Adela, Gil Marta, Mandiá Natalia, Aldamiz-Echevarría Luís, Iria Roca, González-Lamuño Domingo, Couce María-Luz
Neonatal Service. Department of Pediatrics, Santiago de Compostela University Hospital, IDIS (Health Research Institute of Santiago de Compostela), CIBERER, Travesia Choupana Pediatric Nephrology Unit, Santiago de Compostela University Hospital, Santiago de Compostela Metabolism Unit, Clinical University Hospital of Cruces, Bilbao Department of Pediatrics, University Hospital Marqués de Valdecilla-Universidad de Cantabria, Santander, Spain.
Medicine (Baltimore). 2018 Aug;97(32):e11819. doi: 10.1097/MD.0000000000011819.
To evaluate the prognostic significance of factors frequently associated with a reduction in renal mass, such as prematurity, low birth weight, and congenital anomalies of kidney and urinary tract (CAKUT), in patients with solitary functioning kidney (SFK) and investigate signs of early renal injury due to glomerular hyperfiltration damage or dysplasia in the remaining kidney.Retrospective observational study of congenital SFK diagnosed and followed at a tertiary care hospital over a period of 10 years in which 32,900 newborns underwent routine neonatal abdominal ultrasound screening. We analyzed age at diagnosis, sex, gestational age, anthropometric measurements at birth and prenatal and neonatal ultrasound findings, in addition to follow-up data corresponding to imaging findings (ultrasound, micturating cystourethrography, dimercaptosuccinic acid renal, and scintigraphy), ipsilateral CAKUT, compensatory hypertrophy, and renal injury in the form of albuminuria, blood pressure, and estimated glomerular filtration rate (eGFR).In total, 128 congenital SFK cases were detected (1 per 257 live births). Of these, 117 (91.4%) were diagnosed by neonatal ultrasound screening and 44.5% of these had been previously identified by prenatal ultrasound. Neonatal ultrasound had a specificity of 100% and a sensitivity of 92.1%. Forty-five patients (35.2%) had ipsilateral CAKUT and the most common type was urinary collecting system anomalies (75.5%). Over a median follow-up of 6.3 years (1-10 years), compensatory renal hypertrophy was observed in 81 patients (63.7%), most of whom had ipsilateral CAKUT (76.1% vs 56.6% of patients without ipsilateral CAKUT). Albuminuria and hypertension were observed in 3.12% and 5% of patients, respectively, and both were associated with ipsilateral CAKUT (P < .05). In addition, 75% of albuminuria cases (P = .031), 83.3% of hypertension cases (P = .004), and 100% of decreased eGFRcases (P = .031) were significantly associated with CAKUT (renal parenchymal anomaly category), being the strongest predictor of GFR the presence or absence of CAKUT.Neonatal ultrasound screening is useful for the early diagnosis of SFK. The presence of ipsilateral CAKUT should be evaluated in all patients with SFK as congenital anomalies of the renal parenchyma are associated with a poorer prognosis. Because morbidity from CAKUTs may not develop until adulthood, patients should be closely followed throughout life.
评估早产、低出生体重以及肾和尿路先天性异常(CAKUT)等常与肾质量减少相关的因素在单功能肾(SFK)患者中的预后意义,并调查因肾小球超滤损伤或剩余肾脏发育异常导致的早期肾损伤迹象。对一家三级医院在10年期间诊断并随访的先天性SFK进行回顾性观察研究,其中32900例新生儿接受了常规新生儿腹部超声筛查。我们分析了诊断时的年龄、性别、胎龄、出生时的人体测量数据以及产前和新生儿超声检查结果,此外还分析了与影像学检查结果(超声、排尿性膀胱尿道造影、二巯基丁二酸肾显像和闪烁显像)、同侧CAKUT、代偿性肥大以及蛋白尿、血压和估计肾小球滤过率(eGFR)形式的肾损伤相关的随访数据。总共检测到128例先天性SFK病例(每257例活产中有1例)。其中,117例(91.4%)通过新生儿超声筛查诊断,其中44.5%此前已通过产前超声检查发现。新生儿超声的特异性为100%,敏感性为92.1%。45例患者(35.2%)有同侧CAKUT,最常见的类型是泌尿系统集合系统异常(75.5%)。在中位随访6.3年(1 - 10年)期间,81例患者(63.7%)观察到代偿性肾肥大,其中大多数有同侧CAKUT(有同侧CAKUT的患者为76.1%,无同侧CAKUT的患者为56.6%)。分别有3.12%和5%的患者观察到蛋白尿和高血压,两者均与同侧CAKUT相关(P < 0.05)。此外,75%的蛋白尿病例(P = 0.031)、83.3%的高血压病例(P = 0.004)和100%的eGFR降低病例(P = 0.031)与CAKUT(肾实质异常类别)显著相关,CAKUT的有无是GFR最强的预测因素。新生儿超声筛查对SFK的早期诊断有用。所有SFK患者均应评估同侧CAKUT的存在,因为肾实质先天性异常与较差的预后相关。由于CAKUT的发病可能直到成年才出现,因此患者应终身密切随访。