Okada K, Takahashi S, Horiuchi K, Nagura Y, Hatano M
2nd Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan.
Nihon Jinzo Gakkai Shi. 1991 May;33(5):529-31.
We present here a case of young adult-onset primary systemic amyloidosis with AA protein. A 29-year-old male presenting with macrohematuria was transferred to our hospital because of aggravation of renal function. Amyloid protein was detected in the bladder, stomach and thyroid. The amyloid protein proved to be AA type by potassium permanganate histochemical analysis. Since secondary amyloidosis was excluded by the laboratory data and there was no family history of amyloidosis, a diagnosis of primary systemic amyloidosis with AA protein was reached. Although the patient was prescribed dimethyl sulphoxide, his renal function worsened with gastrocolic symptoms and a bleeding tendency. Hemodialysis (HD) was then initiated. After starting the HD, the patient's general condition recovered and subsequently the patient on treatment with maintenance HD was discharged.
我们在此报告一例青年成人起病的伴有AA蛋白的原发性系统性淀粉样变性病例。一名29岁男性因肉眼血尿就诊,后因肾功能恶化转入我院。在膀胱、胃和甲状腺中检测到淀粉样蛋白。经高锰酸钾组织化学分析,该淀粉样蛋白被证实为AA型。由于实验室检查排除了继发性淀粉样变性,且无淀粉样变性家族史,故诊断为伴有AA蛋白的原发性系统性淀粉样变性。尽管给该患者开了二甲基亚砜,但他的肾功能仍因胃肠症状和出血倾向而恶化。随后开始进行血液透析(HD)。开始血液透析后,患者的一般状况有所恢复,随后接受维持性血液透析治疗出院。