儿童家族性播散性非典型分枝杆菌感染

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1981~1989年期间,4例马耳他儿童患了一种少见的播散性非典型分枝杆菌感染综合征,这种综合征与以往已知的免疫缺陷无关。这4例儿童居住在同一村庄,其中3例有亲戚关系,此后不久,在希腊也发现一对兄弟发生了同样的综合征。据推测,此病是由一种由分枝杆菌感染引起的遗传性免疫缺陷。 例1:男,1岁出现厌食、倦怠、发热、盗汗和脾肿大,并有贫血和低白蛋白血症,白细胞升高。反复细菌培养阴性,骨髓穿刺示反应性表现。结核菌素试验阴性,类风湿因子阴性。应用阿斯匹林、抗菌索和泼尼松 Between 1981 and 1989, four cases of Maltese children developed a rare syndrome of atypical mycobacterium disseminated infection that was not associated with previously known immunodeficiency. The four children living in the same village, of which three were relatives, were also found shortly afterwards in Greece with the same syndrome as a pair of brothers. It is speculated that the disease is caused by a mycobacterial infection caused by genetic immunodeficiency. Example 1: Male, 1 year old Anorexia, fatigue, fever, night sweats and splenomegaly, and anemia and hypoalbuminemia, leukocytosis. Repeated bacterial culture negative, bone marrow puncture showed reactivity. Tuberculin test negative, rheumatoid factor negative. Application aspirin, antibacterial cord and prednisone
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