Goldsmith D J, Roberts I S, Short C D, Mallick N P
Renal Unit, Royal Infirmary, Manchester, UK.
Nephron. 1996;74(3):572-6. doi: 10.1159/000189454.
Systemic amyloidosis normally has a dismal prognosis. However, there are several case reports of protracted survival, usually as a response to measures designed to retard the further deposition of amyloid fibrils. In AA amyloid, most commonly associated with inflammatory rheumatological, bowel, and chest diseases, such interventions have had some success, but the dramatic response of complete resolution of nephrotic syndrome as a result of the regular institution of postural chest drainage and antibiotic therapy, in the clinical context of bronchiectasis, has been previously reported only once. In both of our cases, after protracted remission, such therapy was abandoned by the patients, leading both to recurrence of nephrotic syndrome and also eventually to end-stage renal failure requiring dialysis.
系统性淀粉样变性通常预后不佳。然而，有几例关于长期存活的病例报告，通常是对旨在延缓淀粉样纤维进一步沉积的措施的反应。在AA型淀粉样变性中，最常与炎症性风湿病、肠道和胸部疾病相关，此类干预已取得了一些成功，但在支气管扩张的临床背景下，通过定期进行体位性胸腔引流和抗生素治疗使肾病综合征完全缓解的显著反应此前仅报道过一次。在我们的两个病例中，经过长期缓解后，患者放弃了这种治疗，导致肾病综合征复发，最终发展为需要透析的终末期肾衰竭。
