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[伴有高钾血症、肾小管酸中毒及肾功能正常的动脉性高血压:戈登综合征和/或Ⅱ型假性醛固酮减少症?]

[Arterial hypertension with hyperkalemia, tubular acidosis and normal renal function: Gordon syndrome and/or pseudohypoaldosteronism type II?].

作者信息

Ader J L, Waeber B, Suc J M, Brunner H R, Tran-Van T, Durand D, Praddaude F

机构信息

Service de néphrologie, CHU de Toulouse-Rangueil, France.

出版信息

Arch Mal Coeur Vaiss. 1988 Jun;81 Spec No:193-7.

PMID:2973296
Abstract

Basic examination of Mr S., 45 years of age, short in stature and overweight (1.60 m, 76 kg), was carried out because of the mild hypertension (mean AP 125 mm Hg) from which he had suffered for 20 years. The results were as follows: (1) variable hyperkalemia: plasma potassium values were 5.3 to 6.9 mmol/l; (2) normal renal function: serum creatinine 91.5 mumol/l, clearance of inulin 136.6 ml/mn; (3) proximal tubular acidosis: plasma bicarbonate and chloride values were 18.4 and 109 mmol/l, respectively; urinary pH was 7.1 with negative H+ ions urinary excretion (-33 mumol/mn); when plasma bicarbonate level was raised to 26 mmol/l by acute loading, fractional excretion of bicarbonate increased to 19,5 p. 100 while plasma potassium value decreased to 4.2 mmol/l; (4) low PRA (0.29 ng/ml/h) and normal plasma aldosterone concentration (63 pg/ml) with a normal intake of sodium and in a recumbent position. Plasma atrial natriuretic factor (ANF) level was normal: 14 fmol/ml. Intravenous infusion of ANF for 2 h (1 microgram/mn) induced the expected increases in urinary flow rate, and sodium and potassium excretions (+226, +307 and +171 p. 100, respectively). Intravenous infusion of isotonic saline (2 l in 2 h) and oral administration of fludrocortisone acetate for 4 weeks (400 micrograms per day) resulted in a normal decrease in PRA and plasma aldosterone concentration, a normal rise in plasma ANF level (22 and 42 fmol/ml) while slightly increasing AP without improving bicarbonaturia and acidosis.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

对45岁的S先生进行了基础检查。他身材矮小且超重(身高1.60米,体重76千克),因患有20年的轻度高血压(平均动脉压125毫米汞柱)前来检查。检查结果如下:(1)血钾波动:血浆钾值为5.3至6.9毫摩尔/升;(2)肾功能正常:血清肌酐91.5微摩尔/升,菊粉清除率136.6毫升/分钟;(3)近端肾小管酸中毒:血浆碳酸氢盐和氯化物值分别为18.4和109毫摩尔/升;尿pH值为7.1,尿氢离子排泄为阴性(-33微摩尔/分钟);当通过急性负荷使血浆碳酸氢盐水平升至26毫摩尔/升时,碳酸氢盐排泄分数增至19.5%,而血浆钾值降至4.2毫摩尔/升;(4)肾素活性低(0.29纳克/毫升/小时),血浆醛固酮浓度正常(63皮克/毫升),钠摄入正常且处于卧位。血浆心钠素(ANF)水平正常:14飞摩尔/毫升。静脉输注ANF 2小时(1微克/分钟)导致尿流率、钠和钾排泄量预期增加(分别增加226%、307%和171%)。静脉输注等渗盐水(2小时内输注2升)和口服醋酸氟氢可的松4周(每天400微克)导致肾素活性和血浆醛固酮浓度正常下降,血浆ANF水平正常升高(分别为22和42飞摩尔/毫升),同时平均动脉压略有升高,但未改善尿液中碳酸氢盐含量和酸中毒情况。(摘要截选至250词)

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