[General variable immunologic deficiency with malabsorption syndrome].
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.
A I
AI
Parfenov
N I
NI
Ekisenina
R B
RB
Gudkova
Zh A
ZhA
Chanturin
D000293:Adolescent; D000328:Adult; D004406:Dysgammaglobulinemia / Q000209:etiology* / Q000628:therapy; D006801:Humans; D017098:IgA Deficiency; D017099:IgG Deficiency; D007116:Immunization, Passive; D007075:Immunoglobulin M / Q000172:deficiency*; D007958:Leukocyte Count; D008231:Lymphopenia / Q000209:etiology* / Q000628:therapy; D008286:Malabsorption Syndromes / Q000150:complications / Q000276:immunology*; D008875:Middle Aged; D013601:T-Lymphocytes / Q000276:immunology*
D007075:Immunoglobulin M
Obshchaia variabel'naia immunologicheskaia nedostatochnost' s sindromom narushennogo vsasyvaniia.