常染色体隐性遗传性多囊肾病患儿的肾脏浓缩能力与肾小球滤过率和高血压相关。
Kidney concentrating capacity in children with autosomal recessive polycystic kidney disease is linked to glomerular filtration and hypertension.
机构信息
Department of Pediatrics, Dr. v. Hauner Children's Hospital, University Hospital, Ludwig Maximilian University Munich, Lindwurmstraße 4, 80337, Munich, Germany.
Department of Pediatrics, 2nd Medical Faculty, Charles University, Prague, Czech Republic.
出版信息
Pediatr Nephrol. 2023 Jul;38(7):2093-2100. doi: 10.1007/s00467-022-05834-5. Epub 2022 Dec 20.
BACKGROUND
Impaired kidney concentration capacity is present in half of the patients with autosomal dominant polycystic kidney disease (ADPKD). The kidney concentrating capacity was further impaired within the animal model of autosomal recessive polycystic kidney disease (ARPKD). To date, only one small study has investigated it in children having ARPKD. Therefore, we aimed to study the kidney concentrating ability in a larger cohort of children with ARPKD.
METHODS
Eighteen children (median age 8.5 years, range 1.3-16.8) were retrospectively investigated. A standardized kidney concentrating capacity test was performed after the application of a nasal drop of desmopressin (urine osmolality > 900 mOsmol/kg). The glomerular filtration rate was estimated using the Schwartz formula (eGFR) and blood pressure (BP) was measured as office BP.
RESULTS
Kidney concentrating capacity was decreased (urine osmolality < 900 mOsmol/kg) in 100% of children with ARPKD. The median urine osmolality after desmopressin application was 389 (range 235-601) mOsmol/kg. Sixteen patients (89%) were defined as hypertensive based on their actual BP level or their use of antihypertensive drugs. The maximum amounts of urinary concentration correlated significantly with eGFR (r = 0.72, p < 0.0001) and hypertensive scores (r = 0.50, p < 0.05), but not with kidney size. Twelve patients (67%) were defined as having CKD stages 2-4. The median concentrating capacity was significantly lower in children within this group, when compared to children with CKD stage 1 possessing a normal eGFR (544 mOsmol/kg, range 413-600 mOsmol/kg vs. 327 mOsmol/kg, range 235-417 mOsmol/l, p < 0.001).
CONCLUSIONS
Impaired kidney concentrating capacity is present in most children with ARPKD and is associated with decreased eGFR and hypertension. A higher resolution version of the Graphical abstract is available as Supplementary information.
背景
常染色体显性多囊肾病（ADPKD）患者中有一半存在肾脏浓缩能力受损。常染色体隐性多囊肾病（ARPKD）的动物模型中，肾脏浓缩能力进一步受损。迄今为止，仅有一项小型研究调查了 ARPKD 患儿的肾脏浓缩能力。因此，我们旨在更大的 ARPKD 患儿队列中研究肾脏浓缩能力。
方法
回顾性调查了 18 名儿童（中位年龄 8.5 岁，范围 1.3-16.8 岁）。应用去氨加压素滴鼻剂后进行标准化的肾脏浓缩能力测试（尿渗透压>900mOsmol/kg）。肾小球滤过率使用 Schwartz 公式估算（eGFR），血压（BP）作为诊室 BP 进行测量。
结果
100%的 ARPKD 患儿的肾脏浓缩能力下降（尿渗透压<900mOsmol/kg）。去氨加压素应用后的中位尿渗透压为 389（范围 235-601）mOsmol/kg。根据实际 BP 水平或使用降压药物，16 名患者（89%）被定义为高血压。最大尿浓缩量与 eGFR（r=0.72，p<0.0001）和高血压评分（r=0.50，p<0.05）显著相关，但与肾脏大小无关。12 名患者（67%）被定义为 CKD 2-4 期。与 eGFR 正常的 CKD 1 期患儿（544mOsmol/kg，范围 413-600mOsmol/kg）相比，该组患儿的浓缩能力明显较低（327mOsmol/kg，范围 235-417mOsmol/kg，p<0.001）。
结论
大多数 ARPKD 患儿存在肾脏浓缩能力受损，与 eGFR 降低和高血压相关。高分辨率版本的图表摘要可在补充资料中查看。
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