[Endocrinological analysis before and after living-related renal transplantation in a patient of Bartter's syndrome].
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)
D015121:6-Ketoprostaglandin F1 alpha / Q000652:urine*; D000293:Adolescent; D001477:Bartter Syndrome / Q000378:metabolism* / Q000601:surgery*; D006801:Humans; D007610:Kallikreins / Q000378:metabolism; D016030:Kidney Transplantation; D008297:Male; D012084:Renin-Angiotensin System; D014019:Tissue Donors
D015121:6-Ketoprostaglandin F1 alpha; D007610:Kallikreins