Ksouri H, Mellouli F, Barbouch R, Ben Hassen A, Béjaoui M
Service des laboratoires, centre national de greffe de moelle osseuse, 2, rue Djebel-Lakdhar, 1007 Tunis, Tunisie.
Arch Pediatr. 2006 Jul;13(7):1034-9. doi: 10.1016/j.arcped.2006.03.152. Epub 2006 May 18.
Intravenous immunoglobulin (Ig IV) has been used for many years in the treatment of primary antibody deficiencies. We performed a retrospective study of the clinical features and outcome of agammaglobulinemia children who received prolonged Ig IV infusions.
Ten children, 9 male et 1 female, with agammaglobulinemia diagnosis were studied for the clinical manifestations before and during the Ig IV replacement therapy. Serum Ig levels were quantified by nephelometry. Circulating B ant T cells were counted by immunofluorescence labeling by monoclonal antibodies. T-cell functions were assessed by using mitogen and antigen -induced T-cell proliferation assays in vitro. Patients clinical status was evaluated respectively, before initiation and at every moment (when patients had an infection) of the replacement therapy.
Ig IV therapy was performed for 866 cumulated months, median 108 months. The median Ig IV doses administered to the 10 patients was 500 mg/kg/month. Residual serum IgG mean level was 3,9 g/L. All patients had 99 bacterial infections/year before Ig IV, mainly respiratory tract infections (48,5%), and 4 patients had bronchiectasis before Ig replacement therapy. The number of infection/year fall to 25 during IgIV replacement, and the infection/patient/year rate decreases significantly. One patient developed an Echovirus 27 meningoencephalitis during this treatment.
Ig IV therapy with residual IgG mean level of 3,9 g/l reduced significantly the rate of bacterial infections. The use of specific antibiotherapy and respiratory kinesitherapy led to a lower rate of respiratory tract infections, and the stabilisation of the bronchiectasis. However this intravenous replacement therapy does not protect against viral meningoencephalitis.
静脉注射免疫球蛋白（Ig IV）已用于原发性抗体缺陷的治疗多年。我们对接受长期Ig IV输注的无丙种球蛋白血症儿童的临床特征和结局进行了一项回顾性研究。
对10名诊断为无丙种球蛋白血症的儿童（9名男性和1名女性）在Ig IV替代治疗前和治疗期间的临床表现进行了研究。血清Ig水平通过散射比浊法进行定量。循环B细胞和T细胞通过单克隆抗体免疫荧光标记进行计数。通过体外使用丝裂原和抗原诱导的T细胞增殖试验评估T细胞功能。分别在替代治疗开始前和治疗期间的每个时刻（患者发生感染时）评估患者的临床状况。
Ig IV治疗累计进行了866个月，中位数为108个月。10名患者接受Ig IV的中位剂量为500mg/kg/月。残余血清IgG平均水平为3.9g/L。所有患者在接受Ig IV治疗前每年发生99次细菌感染，主要为呼吸道感染（48.5%），4名患者在Ig替代治疗前患有支气管扩张。在IgIV替代治疗期间，每年感染次数降至25次，患者每年感染率显著降低。1名患者在该治疗期间发生了埃可病毒27型脑膜脑炎。
残余IgG平均水平为3.9g/l的Ig IV治疗显著降低了细菌感染率。使用特异性抗生素治疗和呼吸运动疗法导致呼吸道感染率降低，支气管扩张得到稳定。然而，这种静脉替代治疗不能预防病毒性脑膜脑炎。
