Madaliński K, Sabbouh K, Imielska D, Chorazykiewicz M
Department of Immunology, Children's Memorial Hospital, Warsaw.
Arch Immunol Ther Exp (Warsz). 1989;37(5-6):669-74.
Seventy one persons (67 children and 4 of their mothers) who came to the out-patient department of our hospital because of recurrent edemas of subcutaneous tissue and mucous membranes were the subjects of study. Serum haemolytic activity of complement and concentration of C1-esterase inhibitor, C1q, C4 and C3 were measured. Twenty six persons were related: altogether 12 families were studied, mostly sisters and brothers. In six patients (k 8.5% of the group studied) inherited type I defect of C1-inhibitor was detected and the diagnosis of angioneurotic edema confirmed. The results of complement determination in these patients (5 girls and 1 mother) were compared to the rest of the group and to healthy blood donors and showed clear differences in concentrations of C1-inhibitor, C1q, C4 and in haemolytic activity. Treatment consisted of fibrinolysis inhibitor, epsilon-aminocaproic acid and of antihistamine drugs. The value of this kind of therapy in the treatment of a very well responding child is discussed.
71名因皮下组织和粘膜反复水肿前来我院门诊的患者（67名儿童及其4名母亲）作为研究对象。检测了补体的血清溶血活性以及C1酯酶抑制剂、C1q、C4和C3的浓度。26人有亲属关系：共研究了12个家庭，大多为兄弟姐妹。在6名患者（占研究组的8.5%）中检测到遗传性I型C1抑制剂缺陷，并确诊为血管性水肿。将这些患者（5名女孩和1名母亲）的补体测定结果与研究组其他患者以及健康献血者进行比较，结果显示C1抑制剂、C1q、C4的浓度以及溶血活性存在明显差异。治疗包括使用纤溶抑制剂ε-氨基己酸和抗组胺药物。讨论了这种治疗方法对一名反应良好的儿童的治疗价值。
