Goddard E A, Beatty D W
Department of Paediatrics and Child Health, University of Cape Town.
S Afr Med J. 1989 Dec 2;76(11):605-7.
X-linked agammaglobulinaemia (XLA) is a rare immunodeficiency disorder affecting only male subjects. There is an absence of all serum immunoglobulins and circulating B cells. T-cell function and numbers are normal. The clinical characteristics are recurrent pyogenic infections starting in infancy, hypoplasia of lymphoid tissue and a family history in about half the cases. Ten patients with XLA have been seen over the last 14 years at Red Cross War Memorial Children's Hospital, Cape Town. Chronic infections causing significant morbidity occurred in half our patients and 2 have died. Intravenous of gammaglobulin replacement therapy is superior to the intramuscular form. The recommended dose is 200-400 mg/kg/mo., although the optimal dose and frequency of the gammaglobulin infusion should be individualised for each patient.
X连锁无丙种球蛋白血症（XLA）是一种仅影响男性的罕见免疫缺陷疾病。所有血清免疫球蛋白和循环B细胞均缺失。T细胞功能和数量正常。临床特征为始于婴儿期的反复化脓性感染、淋巴组织发育不全，约半数病例有家族史。过去14年里，在开普敦红十字战争纪念儿童医院共诊治了10例XLA患者。我们的患者中有半数发生了导致严重发病的慢性感染，2例患者死亡。静脉注射免疫球蛋白替代疗法优于肌肉注射。推荐剂量为200 - 400mg/kg/月，不过免疫球蛋白输注的最佳剂量和频率应针对每位患者个体化制定。
