Gmar-Bouraoui S, Achour A, Abbes M, Mahjoub S, Frih A, Skhiri H, Ben Dhia N, El May M
Service de Néphrologie et Médecine interne, CHU Fattouma Bourguiba, 5000, Monastir, Tunisie.
Ann Endocrinol (Paris). 2001 Feb;62(1 Pt 1):4-6.
Renal involvement in amyloidosis leads to chronic renal failure. Prognosis is poor. Although amyloid deposits are frequent in adrenal glands, symptomatic adrenal dysfunction is uncommon. We report the case of a 63-year-old man with chronic renal failure (serum creatinine: 202 micromol/L) subsequent to amyloidosis who was referred to our unit for vomiting, dehydration despite a persistent nephrotic syndrome, acidosis, hyponatremia (121 mmol/l) and hyperkaliemia (7.1 mmol/l). A synacthen test was performed and disclosed adrenal insufficiency. Despite the initiation of substitution therapy, the patient died one month later from Addisonian crisis. Features of adrenal insufficiency may be masked by those of chronic renal failure, emphasizing the importance of adrenal explorations in patients with chronic renal failure due to amyloidosis.
肾脏受累于淀粉样变性会导致慢性肾衰竭。预后较差。虽然淀粉样沉积物在肾上腺中很常见,但有症状的肾上腺功能不全并不常见。我们报告了一例63岁男性患者,其在淀粉样变性后出现慢性肾衰竭(血清肌酐:202微摩尔/升),因呕吐、尽管持续存在肾病综合征仍脱水、酸中毒、低钠血症(121毫摩尔/升)和高钾血症(7.1毫摩尔/升)而转诊至我院。进行了促肾上腺皮质激素刺激试验,结果显示肾上腺功能不全。尽管开始了替代治疗,但患者在一个月后死于肾上腺危象。肾上腺功能不全的特征可能被慢性肾衰竭的特征所掩盖,这凸显了对淀粉样变性所致慢性肾衰竭患者进行肾上腺检查的重要性。