Parfenov A I, Ekisenina N I, Gudkova R B, Chanturin Zh A
Klin Med (Mosk). 1990 May;68(5):90-4.
Twenty-two patients with general variable immunodeficiency (GVI) and malabsorption syndrome (MS) were followed up for 2-12 years. III degree MS was found in 17 cases. Serum immunoglobulins concentration and T-lymphocyte count were reduced, the latter at the expense of theophylline-resistant and active E-RFC. With casein and milk albumin as the antigens, lymphokine-producing capacity of the mononuclear cells appeared elevated. MS treatment with adjuvant gamma-globulin produced a positive trend in clinical manifestations of the disease, content of T lymphocytes and relevant subpopulations. Long-term results were less favourable: partial compensation with recurrences persisted in 15 patients only. Seven patients died: two of pneumonia, five of cardiac failure and visceral dystrophy. All MS patients are recommended to undergo serum immunoglobulins diagnosis of GVI and in case of its verification to receive life-time gamma-globulin replacement therapy.
对22例伴有吸收不良综合征(MS)的普通可变免疫缺陷(GVI)患者进行了2至12年的随访。发现17例为Ⅲ度MS。血清免疫球蛋白浓度和T淋巴细胞计数降低,后者以茶碱抵抗性和活性E-RFC为代价。以酪蛋白和乳白蛋白作为抗原时,单核细胞产生淋巴因子的能力似乎升高。用辅助性γ球蛋白治疗MS在疾病临床表现、T淋巴细胞及相关亚群含量方面产生了积极趋势。长期结果不太理想:仅15例患者出现部分代偿且复发。7例患者死亡:2例死于肺炎,5例死于心力衰竭和内脏营养不良。建议所有MS患者接受血清免疫球蛋白检测以诊断GVI,确诊后接受终身γ球蛋白替代治疗。