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中枢神经系统曲霉菌病患者生存率的提高:一组接受曲霉菌瘤立体定向切除术的白血病免疫受损儿童。4例报告。

Improved survival in central nervous system aspergillosis: a series of immunocompromised children with leukemia undergoing stereotactic resection of aspergillomas. Report of four cases.

作者信息

Middelhof Charles A, Loudon William G, Muhonen Michael D, Xavier Christopher, Greene Clarence S

机构信息

Department of Neurological Surgery, University of California, Irvine School of Medicine, Irvine, USA.

出版信息

J Neurosurg. 2005 Oct;103(4 Suppl):374-8. doi: 10.3171/ped.2005.103.4.0374.

DOI:10.3171/ped.2005.103.4.0374
PMID:16270691
Abstract

Central nervous system (CNS) aspergillosis remains a daunting diagnosis. This opportunistic mycosis historically carries a mortality rate approaching 100% in immunocompromised patients, with death ensuing within days after the onset of neurological symptoms. From their literature review, the authors concluded that children contracting CNS aspergillosis while undergoing systemic chemotherapy for leukemias represent a particularly unfortunate prognostic group. Antifungal medications prove ineffective for treating CNS aspergillosis in patients immunocompromised because of their chemotherapy regimens. In contrast, withholding chemotherapy to reverse immunosuppression, thereby improving the efficacy of antifungal medications, allows for progression of the primary leukemic disease. The authors present a series of four immunosuppressed patients whose course of treatment for leukemia was complicated by CNS Aspergillus sp. abscesses. Multiple cerebral fungal abscesses developed in two patients and a single cerebral abscess developed in two. All four patients underwent frameless stereotactic resection of the aspergilloma. All children later experienced resolution of their CNS infections and full neurological recovery. At 2- to 4-year follow ups, one patient has died of leukemia and the other three continue to thrive without evidence of recurrent aspergillosis. Given the grave natural history cited in the literature for this disease when medical treatment is instituted alone, the authors stress the crucial role of stereotactic neurosurgery for the intelligent treatment of immunocompromised children suspected of harboring a CNS aspergilloma abscesses. The authors propose that the goal for successful treatment in these patients should be gross-total resection of the abscess, its wall, and its capsule.

摘要

中枢神经系统(CNS)曲霉病仍然是一个难以诊断的疾病。这种机会性真菌病在历史上,免疫功能低下患者的死亡率接近100%,在神经系统症状出现后的几天内就会死亡。通过文献回顾,作者得出结论,在接受白血病全身化疗期间感染CNS曲霉病的儿童是一个预后特别差的群体。由于化疗方案,抗真菌药物对免疫功能低下的CNS曲霉病患者治疗无效。相反,停止化疗以逆转免疫抑制,从而提高抗真菌药物的疗效,会导致原发性白血病病情进展。作者介绍了4例免疫抑制患者,他们白血病的治疗过程因CNS曲霉属脓肿而复杂化。2例患者出现多个脑真菌脓肿,2例出现单个脑脓肿。所有4例患者均接受了无框架立体定向切除曲霉菌瘤。所有儿童后来中枢神经系统感染均得到缓解,神经功能完全恢复。在2至4年的随访中,1例患者死于白血病,其他3例继续茁壮成长,无曲霉病复发迹象。鉴于文献中单独采用药物治疗时该病严重的自然病程,作者强调立体定向神经外科手术在明智治疗疑似患有CNS曲霉菌瘤脓肿的免疫功能低下儿童中的关键作用。作者提出,这些患者成功治疗的目标应该是脓肿、脓肿壁及其包膜的大体全切除。

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