Assessment of Hypertension in Children with Autosomal Dominant Polycystic Kidney Disease; Single-Center Experience

Alper Uygun, H. Nalcacioglu, O. Aydog
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Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary renal cystic diseases. Althoughits clinical manifestations usually occur in adulthood, hypertension (HT) is known to develop in most patients before the decline inrenal function and it is associated with faster progression to end stage kidney disease (ESKD). We investigated ambulatory bloodpressure monitoring (ABPM) results of 23 patients with ADPKD, followed up in the Pediatric Nephrology Clinic of Ondokuz MayısUniversity Medical Faculty Hospital. Patients’ demographic characteristics, laboratory and ultrasonography (US) results, officeblood pressure, and ABPM measurements were evaluated. The parameters of gender, age, increased kidney size, proteinuria,glomerular filtration rate (GFR) was compared in hypertensive and non-hypertensive group. Twenty three patients (13 girls, tenboys) with a mean age of 11.94±4.01 (min-max: 4.6-18) years and a female/male ratio of 1.3/1 were examined. Ultrasound revealedincreased kidney sizes in 12 patients (52.2%) and multiple cysts in the bilateral kidneys in 20 patients (87%). Mild to moderateproteinuria was detected in 7 patients (30.4%). The HT ratio of patients was 52.2% and 39.1% when assessed with office bloodpressure (BP) measurement and ABPM respectively. A non-dipper pattern was established in 14 patients (60.9%). Gender, age,increased kidney size, proteinuria, GFR did not differ significantly between ADPKD patients with and without ambulatory HT. Thisstudy shows that nearly half of children with ADPKD have HT by ABPM. BP should be regularly screened by ABPM in all pediatricADPKD patients.
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常染色体显性多囊肾病患儿高血压的评价单中心经验
常染色体显性多囊肾病(ADPKD)是最常见的遗传性肾囊性疾病之一。虽然其临床表现通常发生在成年期,但已知高血压(HT)在大多数患者肾功能下降之前就已经出现,并且与终末期肾病(ESKD)的快速进展有关。我们调查了在Ondokuz MayısUniversity医学院医院儿科肾脏科门诊随访的23例ADPKD患者的动态血压监测(ABPM)结果。评估患者的人口统计学特征、实验室和超声检查(US)结果、办公室血压和ABPM测量。比较高血压组和非高血压组的性别、年龄、肾脏增大、蛋白尿、肾小球滤过率(GFR)等参数。23例患者(女孩13例,男孩10例),平均年龄11.94±4.01 (min-max: 4.6-18)岁,男女比例为1.3/1。超声显示12例(52.2%)患者肾脏增大,20例(87%)患者双侧肾脏多发囊肿。轻度至中度蛋白尿7例(30.4%)。以办公室血压(BP)和ABPM评估时,患者的HT比例分别为52.2%和39.1%。14例(60.9%)患者无侧翻模式。性别、年龄、肾脏体积增大、蛋白尿、GFR在伴有和不伴有动态HT的ADPKD患者之间没有显著差异。这项研究表明,近一半的ADPKD儿童在ABPM时发生了HT。所有儿科adpkd患者应定期通过ABPM筛查血压。
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