Torregrosa E, Hernández-Jaras J, Calvo C, Ríus A, García-Pérez H, Maduell F, Vera J M
Servicio de Nefrología Hospital General de Castellón Avda. de Benicassim, s/n. 12004 Castellón de la Plana.
Nefrologia. 2003 Jul-Aug;23(4):321-6.
Amyloidosis is a disease resulting from extracellular deposition of fibrillar protein in various organs. AA amyloidosis may complicate chronic inflammatory diseases, chronic infections and another chronic diseases. We review 31 patients (13 males and 18 females) with biopsy proven renal or rectal AA amyloidosis, referred to out hospital between january 1999 and november 2002. Renal failure was defined as serum creatinine > or = 1.5 mg/dl. Mean age was 58.4 +/- 15.7 years. The causes of AA amyloidosis were an underlying chronic rheumatologic disease (51.6%), chronic infection (41.9%) and a chronic inflammatory intestinal disorder (6.5%). Renal failure (RF) was detected in 20 patients (61.2%) and proteinuria and hematuria were found in 90.3% and 45.5 respectively. Proteinuria at diagnosis was 5.2 +/- 3.9 g/24 h and mean serum creatinine 3.5 +/- 3.7 mg/dl. Survival of patients without dialysis was 66.8 (51.1% RF, 90.9% non-RF) and 53.4% (38.2 RF, 77.9% non-RF) at 12 and 24 months respectively (p = 0.017). End-stage renal disease developed in 13 patients (41.9%). Ten patients were maintained on hemodialysis and 3 on CAD. Survival in dialysis at 6 and 12 months was 68.3% and 42.7% respectively. Fifteen patients died and the main causes of death were: infections (46.6) haemorrhagic complications (33.3%), cardiovascular events (13.3%) and cachexia (6.6%).
淀粉样变性是一种由于纤维状蛋白在各器官细胞外沉积而导致的疾病。AA型淀粉样变性可能使慢性炎症性疾病、慢性感染及其他慢性疾病复杂化。我们回顾了1999年1月至2002年11月间转诊至我院的31例经活检证实为肾型或直肠型AA淀粉样变性的患者(13例男性,18例女性)。肾衰竭定义为血清肌酐≥1.5mg/dl。平均年龄为58.4±15.7岁。AA淀粉样变性的病因包括潜在的慢性风湿性疾病(51.6%)、慢性感染(41.9%)和慢性炎症性肠道疾病(6.5%)。20例患者(61.2%)检测到肾衰竭,90.3%的患者发现蛋白尿,45.5%的患者发现血尿。诊断时蛋白尿为5.2±3.9g/24小时,平均血清肌酐为3.5±3.7mg/dl。未进行透析的患者在12个月和24个月时的生存率分别为66.8%(肾衰竭患者为51.1%,非肾衰竭患者为90.9%)和53.4%(肾衰竭患者为38.2%,非肾衰竭患者为77.9%)(p = 0.017)。13例患者(41.9%)发展为终末期肾病。10例患者维持血液透析,3例患者进行连续性动静脉血液滤过。透析6个月和12个月时的生存率分别为68.3%和42.7%。15例患者死亡,主要死亡原因是:感染(46.6%)、出血并发症(33.3%)、心血管事件(13.3%)和恶病质(6.6%)。