Atanasova P
Vutr Boles. 1985;24(5):93-6.
A patient is described, with genetic form of amyloidosis with family Mediterranean fever. The secondary amyloidosis, that developed as a complication, has been preceded by attacks of acute articular and abdominal pains of several years. Elevated level of ethicholanolon in urine was established as well as extremely high values of serum fibrinogen, admitted to be pathognomonic signs of family Mediterranean fever. The eposition of amyloidosis is confirmed via rectal and renal punch biopsy. In spite of the diffuse character of the deposited amyloid in the renal tissue, there were still no signs of glomerular sclerosis and clinicalf--normal depuration renal function was observed, with normal creatinine clearance and normal nitrogenous bodies in serum. A favourable effect of colchicine therapy was observed in the patients both as regards the acute attacks of the disease and as regards the renal involvement.
本文描述了一名患有遗传性地中海热型淀粉样变性病的患者。继发性淀粉样变性作为一种并发症出现,在此之前患者已遭受数年的急性关节痛和腹痛发作。尿中乙基胆甾醇水平升高,血清纤维蛋白原值极高,这些被认为是家族性地中海热的特征性体征。通过直肠和肾脏穿刺活检证实了淀粉样变性的沉积。尽管肾脏组织中淀粉样蛋白沉积具有弥漫性,但仍无肾小球硬化迹象,且观察到临床肾功能正常——肌酐清除率正常,血清含氮物质正常。观察到秋水仙碱治疗对患者的疾病急性发作和肾脏受累均有良好效果。