Marandian M H, Saboury-Deilami M, Rakchan M, Lessani M, Behvad A, Grouhi M
Pediatrie. 1985 Dec;40(8):653-7.
Distal gangrene associated with arterial hypertension but without systemic manifestations or muco-cutaneous eruptions was noted in a 5 years old child. Gangrene first appeared in toes and then in fingers. Progression in a 7 months period led to bilateral midleg amputation and amputation of several fingers. Angiography revealed complete obliteration of popliteal and humeral arteries by thrombi and histologic examination of amputated material showed organized thrombi with phlebothrombosis and without vasculitis. Biopsies of liver, lung, kidneys and skin were unremarkable and immunofluorescence studies with various antisera were negative. Also no contributing factor or etiologic agents were found, similarities to adult type Buerger's disease are striking. Vasodilators were of no help and bilateral lumbar and cervical sympathectomy could only demarcate the already present gangrene.
一名5岁儿童出现与动脉高血压相关的远端坏疽,但无全身表现或黏膜皮肤疹。坏疽首先出现在脚趾,然后是手指。在7个月的时间里病情进展,导致双侧小腿中段截肢和多个手指截肢。血管造影显示腘动脉和肱动脉被血栓完全阻塞,截肢材料的组织学检查显示血栓机化伴静脉血栓形成且无血管炎。肝、肺、肾和皮肤活检无异常,用各种抗血清进行的免疫荧光研究均为阴性。此外,未发现促成因素或病原体,与成人型伯格病的相似性很显著。血管扩张剂无效,双侧腰交感神经切除术和颈交感神经切除术只能界定已有的坏疽范围。