Lee S M, Lightner E, Witte M, Oberfield S, Levine L, New M I
Acta Endocrinol (Copenh). 1982 Feb;99(2):251-5. doi: 10.1530/acta.0.0990251.
A 9 year old Mexican boy presented with severe hypertension, hypokalaemia and features suggesting acute glomerulonephritis. Nephrosclerosis was present on renal biopsy. Aldosterone levels were unresponsive to variations in dietary salt intake and plasma renin activity was suppressed. Following oral dexamethasone therapy (2 mg/day), plasma aldosterone decreased to undetectable levels, serum potassium normalized and plasma renin activity gradually increased. Dexamethasone also restored the normal responsiveness of the renin-aldosterone system to postural stimuli. The patient exhibited a marked response to a single dose of ACTH with a rise in plasma aldosterone. Long-term blood pressure control and normal potassium levels have been achieved with oral prednisone therapy (5 mg/day) for a period of one year. This case of dexamethasone suppressible hyperaldosteronism (DSH) illustrates that the degree of hypertension in this syndrome may produce severe renal microvascular lesions. DSH should be considered in all children who present with low renin hypertension.
一名9岁墨西哥男孩出现严重高血压、低钾血症及提示急性肾小球肾炎的特征。肾活检显示存在肾硬化。醛固酮水平对饮食中盐摄入量的变化无反应,血浆肾素活性受抑制。口服地塞米松治疗(2毫克/天)后,血浆醛固酮降至无法检测水平,血钾恢复正常,血浆肾素活性逐渐升高。地塞米松还恢复了肾素-醛固酮系统对体位刺激的正常反应性。患者对单剂量促肾上腺皮质激素有明显反应,血浆醛固酮升高。口服泼尼松治疗(5毫克/天)一年来实现了长期血压控制和血钾正常。这例地塞米松可抑制性醛固酮增多症(DSH)表明,该综合征的高血压程度可能会导致严重的肾微血管病变。所有表现为低肾素性高血压的儿童都应考虑DSH。