Zambaiti Elisa, Sergio Maria, Baldanza Fabio, Corrado Ciro, Di Pace Maria Rita, Cimador Marcello
Pediatric Urological Unit, Department of Sciences for Health Promotion and Mother-Child Care "G. D'Alessandro", AOU Policlinico Paolo Giaccone, Via Giordano 3, 90127, Palermo, Italy.
Pediatric Nephrology Unit, Children's Hospital 'G. Di Cristina', A.R.N.A.S. 'Civico', Piazza Nicola Leotta 4, 90127, Palermo, Italy.
Pediatr Surg Int. 2019 Jan;35(1):167-174. doi: 10.1007/s00383-018-4389-z. Epub 2018 Oct 29.
Solitary functioning kidney (SFK) may be associated to hypertrophy, hypertension and chronic kidney disease. We evaluated blood pressure (BP) of children with congenital SFK comparing agenesis to multicystic dysplastic kidney (MCDK) and correlated BP profiles with renal dimensions of affected and contralateral kidney.
We compared 40 patients with MCDK, grouped for either treatment options (A: conservative vs B: nephrectomy) or involution time (A1: before 4 years-of-age vs A2: persistence-of-MCDK), to 10 unilateral agenesis (C). Patients were evaluated with ultrasound, scintigraphy, office-ambulatory BP monitoring.
Compensatory hypertrophy was demonstrated in most of the subjects, without differences between subgroups, with an increase over time (p < 0.001). A1-C showed an overall percentage of hypertrophy significantly higher than A2-B (83%-88% vs 70%-73%, respectively; p = 0.03); moreover, cumulative risk to develop hypertension in A1-C is significantly higher compared to A2-B in office and ambulatory BP monitoring (p = 0.03). Insufficient dipping in systolic and/or diastolic BP was found in 82% children, without differences between subtypes.
Patients with a small/absent dysplastic kidney have an increased risk to develop hypertrophy and hypertension compared to patients with a large residual, regardless of nephrectomy. ABPM revealed absent dipping in most patients with SFK, warning further investigations in apparently not symptomatic patients.
孤立功能肾(SFK)可能与肥大、高血压和慢性肾病相关。我们评估了先天性SFK患儿的血压(BP),比较了肾缺如与多囊性发育不良肾(MCDK),并将血压情况与患侧及对侧肾脏的尺寸相关联。
我们将40例MCDK患者根据治疗方案(A:保守治疗 vs B:肾切除术)或退化时间(A1:4岁前 vs A2:MCDK持续存在)分组,与10例单侧肾缺如患者(C组)进行比较。对患者进行超声、闪烁扫描、诊室-动态血压监测评估。
大多数受试者出现代偿性肥大,各亚组间无差异,且随时间增加(p < 0.001)。A1-C组的肥大总体百分比显著高于A2-B组(分别为83%-88% vs 70%-73%;p = 0.03);此外,在诊室和动态血压监测中,A1-C组发生高血压的累积风险显著高于A2-B组(p = 0.03)。82%的儿童存在收缩压和/或舒张压夜间谷值不足,各亚型间无差异。
与有较大残余肾脏的患者相比,发育不良的小肾脏/无肾脏患者发生肥大和高血压的风险增加,无论是否进行肾切除术。动态血压监测显示大多数SFK患者夜间谷值消失,提示应对看似无症状的患者进行进一步检查。