Focal segmental glomerulosclerosis in a patient with isolated ACTH deficiency and reversible hypothyroidism
Focal segmental glomerulosclerosis in a patient with isolated ACTH deficiency and reversible hypothyroidism
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DOI：
10.1007/s10157-009-0228-9
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发表时间：
2010-04-01
影响因子：
2.3
通讯作者：
Iida, Mitsuo
中科院分区：
文献类型：
--
作者：
Yamada, Shunsuke;Bandai, Sachiko;Iida, Mitsuo
1例23岁男性患者因乏力、食欲减退、蛋白尿和外周水肿入院治疗。蛋白尿在15岁时首次被发现，但没有进行进一步的研究。在入院前六年，患者注意到变得很容易疲劳。入院时实验室检查显示外周血嗜酸性粒细胞增多(29.2%，计数：1,071/亩L)和蛋白尿。内分泌学研究显示单纯性促肾上腺皮质激素缺乏伴原发性甲状腺功能减退，但缺乏自身免疫性甲状腺炎。肾活检显示局灶性节段性肾小球硬化。氢化可的松治疗单纯性促肾上腺皮质激素缺乏症(30 mg/d)可显著改善肾上腺和甲状腺功能，并在两个月内改善蛋白尿(从2.8g/d到1.0g/d)。氢化可的松治疗开始五年后，肾功能仍保持正常。研究结果表明，氢化可的松治疗和甲状腺激素状态正常化在改善与局灶节段性肾小球硬化相关的蛋白尿方面发挥了关键作用。
A 23-year-old man was admitted to our hospital for fatigue, anorexia, proteinuria, and peripheral edema. Proteinuria was first pointed out at the age of 15, but no further studies were performed. Six years prior to admission, the patient noted becoming easily fatigued. Laboratory tests on admission showed marked peripheral eosinophilia (29.2%, count: 1,071/mu L) and proteinuria. Endocrinological studies revealed isolated adrenocorticotropic hormone deficiency with primary hypothyroidism, but a lack of autoimmune thyroiditis. Renal biopsy showed focal segmental glomerulosclerosis. Hydrocortisone therapy (30 mg/day) for isolated adrenocorticotropic hormone deficiency resulted in marked improvement of adrenal and thyroid functions, and amelioration of proteinuria (from 2.8 to 1.0 g/day) over a two-month period. Renal function remains normal at five years after the start of hydrocortisone treatment. The findings suggest that both hydrocortisone therapy and normalized thyroid hormone status played a pivotal role in the improvement of proteinuria associated with focal segmental glomerulosclerosis.