Common Severe Infections in Chronic Granulomatous Disease
Common Severe Infections in Chronic Granulomatous Disease
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DOI：
10.1093/cid/ciu1154
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发表时间：
2015-04-15
影响因子：
11.8
通讯作者：
Holland, Steven M.
中科院分区：
文献类型：
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作者：
Marciano, Beatriz E.;Spalding, Christine;Holland, Steven M.
背景。慢性肉芽肿病（CGD）是由于烟酰胺腺嘌呤二核苷酸磷酸氧化酶活性缺陷所致，其特征是有限范围的细菌和真菌的反复感染以及炎症并发症。为了了解 CGD 中常见严重感染的影响，我们检查了 4 年来在单个中心跟踪的 268 名患者的记录。方法。所有患者均确诊为 CGD，并尽可能确定基因型。医疗记录被摘录成标准格式。微生物分析仅限于葡萄球菌、伯克霍尔德氏菌、沙雷氏菌、诺卡氏菌和曲霉。结果。曲霉菌发病率估计为每 100 患者年 2.6 例；伯克霍尔德杆菌，每 100 患者年 1.06 个；诺卡氏菌，每 100 患者年 0.81 个；沙雷氏菌感染，每 100 患者年 0.98 例，严重葡萄球菌感染，每 100 患者年 1.44 例。 87%的患者发生肺部感染，32%的患者发生肝脓肿。在产生超氧化物的较低四分位数（四分位数 1 和 2）中，曲霉菌发病率为 55%，但在较高四分位数中，曲霉菌发病率仅为 41%（比率，
Background. Chronic granulomatous disease (CGD) is due to defective nicotinamide adenine dinucleotide phosphate oxidase activity and characterized by recurrent infections with a limited spectrum of bacteria and fungi as well as inflammatory complications. To understand the impact of common severe infections in CGD, we examined the records of 268 patients followed at a single center over 4 decades.Methods. All patients had confirmed diagnoses of CGD, and genotype was determined where possible. Medical records were excerpted into a standard format. Microbiologic analyses were restricted to Staphylococcus, Burkholderia, Serratia, Nocardia, and Aspergillus.Results. Aspergillus incidence was estimated at 2.6 cases per 100 patient-years; Burkholderia, 1.06 per 100 patient-years; Nocardia, 0.81 per 100 patient-years; Serratia, 0.98 per 100 patient-years, and severe Staphylococcus infection, 1.44 per 100 patient-years. Lung infection occurred in 87% of patients, whereas liver abscess occurred in 32%. Aspergillus incidence was 55% in the lower superoxide-producing quartiles (quartiles 1 and 2) but only 41% in the higher quartiles (rate ratio,