Bassiri-Jahromi Shahindokht, Doostkam Aida
Medical Mycology Department, Pasteur Institute of Iran and Education Deceleopment Centre, Tehran, Iran.
J Glob Infect Dis. 2011 Oct;3(4):348-52. doi: 10.4103/0974-777X.91056.
Chronic granulomatous disease (CGD) is an inherited disorder of the Nicotinamide adenine dinucleotide phosphate reduced oxidase complex characterized by recurrent bacterial and fungal infections. Disseminated infection by combination of opportunistic agents is being increasingly reported in CGD patients. We presented in the retrospective review of medical records, the etiology, presentation, clinical characteristics the infections detected, predisposing condition and outcome of nocardiosis and actinomycosis involved in a group of pediatric patients diagnosed with CGD.
The clinical presentation of CGD-related infections was reviewed retrospectively from the medical records of all 12 patients with CGD. We studied respectively 12 patients between 2001 and 2008, and we analyzed two pediatric patients with CGD who acquired Nocardia and Actinomyces infections, and their clinical and microbiological characteristics were described. The material for investigations was collected from scrapings, crusts, pus from subcutaneous abscesses or exudation from sinus tracts, surgical debridement, and biopsy specimens. The microbiological diagnosis was determined by biochemical tests, histology, microscopy, and culture of clinical samples.
The medical records of 12 diagnosed CGD patients with suspected nocardiosis or actinomycosis were reviewed. One patient was diagnosed with actinomycosis and one patient with nocardiosis. Patients consisted of seven males and five females with ranging ages of 3 to 18 years. Nocardiosis and actinomycosis isolated in the two patients were confirmed by histology and culture methods. Neutrophil oxidative burst were absent (NBT=0) in both patients. The most common manifestations of CGD due to fungal infections, actinomycosis, and nocardiosis were osteomyelitis (42.8%), pulmonary infections (28.6%), lymphadenopathy (14.3%), and skin involvement (14.3%) during their illness.
Nocardiosis and actinomycosis in children indicate the need for evaluation for an underlying immunological deficiency. Early diagnosis remains critical for decreased morbidity and occasional mortality. Physicians caring for patients with CGD should maintain a high index of suspicion for nocardiosis and actinomycosis especially if work up for TB and fungal infections are negative.
慢性肉芽肿病(CGD)是一种遗传性烟酰胺腺嘌呤二核苷酸磷酸还原氧化酶复合物疾病,其特征为反复发生细菌和真菌感染。CGD患者中,机会性病原体联合引起的播散性感染报道日益增多。我们通过回顾病历,阐述了一组诊断为CGD的儿科患者中诺卡菌病和放线菌病的病因、表现、临床特征、检测到的感染、易感因素及结局。
回顾性分析12例CGD患者病历中与CGD相关感染的临床表现。我们研究了2001年至2008年间的12例患者,并分析了2例感染诺卡菌和放线菌的CGD儿科患者,描述了他们的临床和微生物学特征。调查材料取自刮片、痂皮、皮下脓肿脓液或窦道渗出物、手术清创物及活检标本。通过生化试验、组织学、显微镜检查及临床样本培养进行微生物学诊断。
回顾了12例诊断为CGD且疑似诺卡菌病或放线菌病患者的病历。1例患者诊断为放线菌病,1例患者诊断为诺卡菌病。患者包括7名男性和5名女性,年龄在3至18岁之间。通过组织学和培养方法确诊了2例患者的诺卡菌病和放线菌病。2例患者中性粒细胞氧化爆发均缺失(硝基四氮唑蓝试验=0)。在患病期间,真菌感染、放线菌病和诺卡菌病导致的CGD最常见表现为骨髓炎(42.8%)、肺部感染(28.6%)、淋巴结病(14.3%)和皮肤受累(14.3%)。
儿童诺卡菌病和放线菌病提示需要评估潜在的免疫缺陷。早期诊断对于降低发病率和偶尔出现的死亡率仍然至关重要。照顾CGD患者的医生应高度怀疑诺卡菌病和放线菌病,尤其是在结核病和真菌感染检查结果为阴性时。