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先天性中性粒细胞减少症中的自发性败血梭菌性肌坏死

Spontaneous Clostridium septicum myonecrosis in congenital neutropaenia.

作者信息

Keogh G, Unsworth I, Vowels M, Kern I B

机构信息

Department of Surgery, Prince of Wales Children's Hospital, Randwick, New South Wales, Australia.

出版信息

Aust N Z J Surg. 1994 Aug;64(8):574-5. doi: 10.1111/j.1445-2197.1994.tb02291.x.

DOI:10.1111/j.1445-2197.1994.tb02291.x
PMID:8048901
Abstract

Spontaneous Clostridium septicum myonecrosis is an uncommon disorder that has been described in association with malignancy, immunosuppression and neutropaenia. Typical clostridial myonecrosis develops without a visible portal of entry and mortality is high. The pathogenesis is not completely understood but the clostridia may gain access to the circulation via areas of ileo-caecal ulceration secondary to enterocolitis, antibiotics or neoplasms. A 5 year old boy with congenital neutropaenia presented with spontaneous Clostridium septicum myonecrosis in the thigh. Limb salvage was achieved using antibiotics, hyberbaric oxygenation and selective debridement. The portal of entry may have been the gastrointestinal tract as colonic ulceration may occur in neutropaenia, and pre-morbid clindamycin administration may have encouraged overgrowth of colonic clostridia.

摘要

自发性败血梭菌性肌坏死是一种罕见的疾病,已被描述与恶性肿瘤、免疫抑制和中性粒细胞减少有关。典型的梭菌性肌坏死在没有可见的侵入途径的情况下发生,死亡率很高。发病机制尚未完全了解,但梭菌可能通过继发于小肠结肠炎、抗生素或肿瘤的回盲部溃疡区域进入循环系统。一名患有先天性中性粒细胞减少症的5岁男孩出现大腿自发性败血梭菌性肌坏死。通过使用抗生素、高压氧疗和选择性清创术成功保住了肢体。侵入途径可能是胃肠道,因为中性粒细胞减少症可能会发生结肠溃疡,而发病前使用克林霉素可能会促使结肠梭菌过度生长。

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Radiol Case Rep. 2015 Nov 6;8(3):806. doi: 10.2484/rcr.v8i3.806. eCollection 2013.
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Pathology of fatal traumatic and nontraumatic clostridial gas gangrene: a histopathological, immunohistochemical, and ultrastructural study of six autopsy cases.致命性创伤性和非创伤性梭菌性气性坏疽的病理学:6例尸检病例的组织病理学、免疫组织化学和超微结构研究
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Clostridium septicum alpha-toxin is proteolytically activated by furin.
败血梭菌α毒素可被弗林蛋白酶进行蛋白水解激活。
Infect Immun. 1997 Oct;65(10):4130-4. doi: 10.1128/iai.65.10.4130-4134.1997.