慢性肉芽肿病中的侵袭性霉菌感染：一项 25 年回顾性调查。

Invasive mold infections in chronic granulomatous disease: a 25-year retrospective survey.

机构信息

Immunology and Haematology Unit, Hôpital Necker Enfants Malades, Paris, France.

出版信息

Clin Infect Dis. 2011 Dec;53(12):e159-69. doi: 10.1093/cid/cir731.

DOI:10.1093/cid/cir731

PMID:22080130

Abstract

BACKGROUND

Invasive fungal infection (IFI) represents a life-threatening condition for patients with chronic granulomatous disease (CGD) and causes one-third of deaths in this population. This study offers a descriptive review of invasive mold infection (mIFI) in children with CGD over an extended period of time.

METHODS

In a cohort of patients with CGD registered in the French National database for Primary Immunodeficiency, we performed a retrospective review of proven mIFI episodes (European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group 2008 criteria) occurring from 1984 through 2009.

RESULTS

Twenty-nine proven mIFIs were identified in 24 patients. Thirteen (54%) of 24 children were receiving itraconazole prophylaxis. Seven episodes were caused by Aspergillus fumigatus, 10 by Aspergillus nidulans, 2 by Aspergillus species, and 6 by other opportunistic molds (4 patients only had positive pathological examination findings). First proven mIFI occurred later in the group that received itraconazole than in the group without (median time to mIFI, 10 vs 4 years; P < .01), with a higher proportion of infections due to A. nidulans and other opportunistic molds (P < .05). Course of IFI was complex, with the median duration of therapy and hospitalization reaching 446 and 153 days, respectively. Combined antifungal therapy was commonly used. Four patients received geno-identical hematopoietic stem cell transplantation as salvage therapy. Global cure rate among the cohort reached 75%, but sequelae were frequent. Prognosis has improved over time (43% mortality during 1985-1990 vs 6% thereafter; P = .06). Mortality tended to be lower in the group that recieved itraconazole prophylaxis but at the cost of a longer duration of therapy among cured patients.

CONCLUSIONS

Management of mIFI remains challenging in patients with CGD, but significant improvements have been made over the past decade.

摘要

背景

侵袭性真菌感染（IFI）是慢性肉芽肿病（CGD）患者面临的危及生命的病症，也是该人群中三分之一死亡病例的病因。本研究对 CGD 患儿在较长时间内侵袭性霉菌感染（mIFI）进行了描述性回顾。

方法

在法国原发性免疫缺陷登记数据库中的 CGD 患者队列中，我们对 1984 年至 2009 年期间发生的已证实的 mIFI 发作（欧洲癌症研究与治疗组织/侵袭性真菌感染合作组和美国国家过敏与传染病研究所霉菌研究组 2008 标准）进行了回顾性分析。

结果

在 24 名患儿中，共发现 29 例已证实的 mIFI。13 名（54%）患儿正在接受伊曲康唑预防。7 例由烟曲霉引起，10 例由构巢曲霉引起，2 例由曲霉菌种引起，6 例由其他机会性霉菌引起（仅 4 例患儿仅有阳性病理检查结果）。接受伊曲康唑治疗的患儿首次确诊 mIFI 的时间晚于未接受治疗的患儿（mIFI 中位时间，10 年比 4 年；P<.01），且感染构巢曲霉和其他机会性霉菌的比例较高（P<.05）。IFI 病程复杂，治疗和住院中位时间分别为 446 天和 153 天。常联合使用抗真菌治疗。4 名患儿接受了亲缘相合造血干细胞移植作为挽救性治疗。该队列的总体治愈率为 75%，但后遗症频繁。随着时间的推移，预后有所改善（1985-1990 年死亡率为 43%，此后为 6%；P=.06）。在接受伊曲康唑预防治疗的患儿中，死亡率较低，但治愈患儿的治疗时间较长。