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[巴特综合征患者亲属活体肾移植前后的内分泌学分析]

[Endocrinological analysis before and after living-related renal transplantation in a patient of Bartter's syndrome].

作者信息

Yokoyama T

机构信息

Department of Nephrology and Dialysis, Yubari Municipal General Hospital, Hokkaido, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1995 Oct;37(10):580-6.

PMID:7474511
Abstract

In order to clarify endocrinological changes before and after living-related renal transplantation in a patient of Bartter's syndrome involving chronic renal failure, serial quantitative determinations of the renin-angiotensin-aldosterone system and 24-hour urinary excretion of 6-keto-prostaglandin F1 alpha and kallikrein were performed. A male patient was admitted to hospital because of a pale face and short stature at the age of 13 years. He was 126 cm in height (M-3.8SD). Blood pressure was 110/60 mmHg and edema was not observed. Laboratory findings showed that his hematocrit was 22.1%, serum potassium 2.9 mEq/1, creatinine clearance was 30.7 ml/min/1.73m2 and beta 2-microgobulin was elevated to 39.9 mg/1 in urinalysis. Plasma renin activity and aldosterone were remarkably elevated to 24.23 ng/ml/hr and 738 pg/ml, respectively. The kidney biopsy specimen showed diffuse glomerulosclerosis and hypertrophic change of the juxtaglomerular apparatus was also demonstrated. He was diagnosed as Bartter's syndrome with short stature and chronic renal failure. At the age of 18, he was introduced on hemodialysis and the living-related renal transplantation was performed the next year. Two weeks after the transplantation, plasma renin activity, angiotensin, I,II and aldosterone were markedly changed from 37.8 to 2.3 ng/ml/hr, 2400 to 220 pg/ml, 256 to 17 pg/ml and 3700 to 110 pg/ml, respectively. Urine prostaglandin F 1 alpha was improved from 860 to 321 ng/day and kallikrein was also changed from 400 to 25.2 micrograms/day. These results indicated that abnormalities of several hormones in Bartter's syndrome could be normalized by living-related renal transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

为了阐明患有慢性肾衰竭的巴特综合征患者亲属活体肾移植前后的内分泌变化,对肾素 - 血管紧张素 - 醛固酮系统以及6 - 酮 - 前列腺素F1α和激肽释放酶的24小时尿排泄量进行了系列定量测定。一名男性患者13岁时因面色苍白和身材矮小入院。他身高126厘米(低于均值3.8个标准差)。血压为110/60 mmHg,未观察到水肿。实验室检查结果显示,其血细胞比容为22.1%,血清钾2.9 mEq/L,肌酐清除率为30.7 ml/min/1.73m²,尿液分析中β2 - 微球蛋白升高至39.9 mg/L。血浆肾素活性和醛固酮分别显著升高至24.23 ng/ml/hr和738 pg/ml。肾活检标本显示弥漫性肾小球硬化,同时也证实了肾小球旁器的肥大变化。他被诊断为患有身材矮小和慢性肾衰竭的巴特综合征。18岁时开始进行血液透析,次年接受了亲属活体肾移植。移植后两周,血浆肾素活性、血管紧张素I、II和醛固酮分别从37.8显著降至2.3 ng/ml/hr、从2400降至220 pg/ml、从256降至17 pg/ml以及从3700降至110 pg/ml。尿前列腺素F1α从860 ng/天改善至321 ng/天,激肽释放酶也从400降至25.2 μg/天。这些结果表明,巴特综合征中几种激素的异常可通过亲属活体肾移植恢复正常。(摘要截断于250字)

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