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[先天性无丙种球蛋白血症:5例研究]

[Congenital agammaglobulinemia: study of 5 cases].

作者信息

Naspitz C, Solé D

出版信息

Bol Med Hosp Infant Mex. 1989 Jan;46(1):22-9.

PMID:2653356
Abstract

Five congenital agammaglobulinemic (CA) boys, started their recurrent bacterial infections between the ages of 3-18 months, presenting otitis (4), pneumonias (4), chronic diarrhea (4), meningitis (2), septicemia (2), septic arthritis (1) and urinary infection (1). The gamma globulin fraction was below 0.08 mg/dL in all patients. IgG, IgA, IgM and IgE levels were always below 50 mg/dL, 2 mg/dL, 35 mg/dL and 20 IU/mL, respectively. Secretory IgA was non-detectable in all patients. Total complement levels were normal (3) and the C3 fraction was elevated in 4 patients. The in vitro response of peripheral lymphocytes to PHA was normal in 4 patients, as well as the number of OKT3, OKT4 and OKT8 cells (2).

摘要

五名先天性无丙种球蛋白血症(CA)男孩在3至18个月大时开始反复出现细菌感染,表现为中耳炎(4例)、肺炎(4例)、慢性腹泻(4例)、脑膜炎(2例)、败血症(2例)、化脓性关节炎(1例)和尿路感染(1例)。所有患者的丙种球蛋白组分均低于0.08mg/dL。IgG、IgA、IgM和IgE水平始终分别低于50mg/dL、2mg/dL、35mg/dL和20IU/mL。所有患者均未检测到分泌型IgA。3例患者的总补体水平正常,4例患者的C3组分升高。4例患者外周淋巴细胞对PHA的体外反应正常,OKT3、OKT4和OKT8细胞数量也正常(2例)。

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