x连锁慢性肉芽肿病1例报告

N. Prashanti, B. Subrahmanyam, G. Rachana, A. Sushma, B. Sharvanabhava
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摘要

慢性肉芽肿病是一种罕见的原发性免疫缺陷疾病,主要攻击白细胞,其特点是随着组织中肉芽肿的发生,对复发性细菌和真菌感染的易感性增加。葡萄球菌和曲霉病是CGD中大多数感染的主要病原体。我们在此报告一个5岁的男孩,他的主诉是发烧,咳嗽,右腿,右脚踝和左手多发脓肿,并有脓。对慢性肉芽肿病进行了DHR试验,结果呈阳性。3个月前无症状,后发展为肺炎,需通气5天。儿童有反复发作的下呼吸道感染,静脉抗生素处方。家族中未见免疫缺陷的类似症状。儿童按年龄进行了免疫接种,但已推迟了里程碑。免疫学研究显示IgA-192(100-490)、IgM-90(50-320)浓度正常,IgG-797(800-1700)轻度下降。全血图显示小细胞性低色素贫血。进行抗生素敏感性试验,发现患儿对左氧氟沙星、亚胺培南、美罗培南敏感。
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A Case Report on X-Linked Chronic Granulomatous Disease (CGD)
The Chronic Granulomatous Disease is a rare primary immunodeficiency disease, principally attacking white blood cells, characterized by an increased susceptibility to the recurrent bacterial and fungal infections along with the genesis of granulomas in tissue. Staphylococcus and aspergillosis are the main pathogens responsible for majority of infections in CGD. Here we report a case of 5yrs old boy who came with the complaints of fever, cough, and multiple abscess over the right leg, right ankle and left hand with puss discharge. DHR test was performed and have shown the positive results for chronic granulomatous disease. asymptomatic till 3 months of life, then developed pneumonia for which he was ventilated for 5 days. Child has recurrent episodes of LRTI for which IV antibiotics were prescribed. There were no similar complaints in family relating to immune deficiency. Child was immunized as per age profile but has delayed milestones. Immunological studies show normal concentrations of IgA-192 (100-490), IgM-90 (50-320) with mild decrease of IgG-797 (800-1700). Complete blood picture reveals microcytic hypochromic anaemia. Antibiotic sensitivity test was done, and child was found to be sensitive to levofloxacin, imipenem and meropenem.
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