Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The group of patients consisted of 4 pairs of male siblings with the family history suggesting X-linked immunodeficiency. The concentration of serum immunoglobulins (Ig) and the titer of anti-E. coli and anti-Candida albicans antibodies was extremely low in comparison to the control group. Only 2 siblings showed normal number of lymphocytes with surface Ig. Four out of 8 children had the increased number of Fc-IgG bearing T cells. Blast transformation of lymphocytes revealed the decreased response to PHA and Con A in 2 children. Cellular immunoglobulin (cIg) synthesis of in vitro stimulated peripheral blood lymphocytes (PBL) was decreased in all 8 children. Prednisolone, when added to the PBL cultures, increased cIg synthesis in 2 children. Thymosin (TFX) caused an enhancement of cIg production by lymphocytes of 4 children. Cowan I--elicited stimulation of PBL was low in all but 2 children. Our data further support the view on the heterogeneity of etiopathogenesis of hypogammaglobulinemia.
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PMID:Immunoglobulin synthesis by lymphocytes of eight immuno-deficient children. 390 93

Angio-immunoblastic lymphadenopathy is a systemic disease of unknown aetiology. It carries a high mortality mainly from infection which results both from the intrinsic immunodeficiency and its exacerbation by treatment with steroids and or cytotoxics. We report a case of angio-immunoblastic lymphadenopathy with the unusual feature of hypogammaglobulinaemia who died of miliary tuberculosis. We suggest that all patients with angio-immunoblastic lymphadenopathy who have inactive tuberculous foci should be given antituberculous prophylaxis.
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PMID:Angio-immunoblastic lymphadenopathy with hypogammaglobulinaemia complicated by miliary tuberculosis. 392 66

A case of hypogammaglobulinemia in a 4.5-year-old boy is presented. The radiologist was the first physician to suggest this diagnosis by noting the absence of adenoidal and tonsillar lymphoid tissue on computed tomography in a patient with sinusitis. A review of the radiographic features of the primary immunodeficiency states is discussed.
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PMID:Computed tomography findings in a case of hypogammaglobulinemia. 394 54

The authors report the case of a 17 year-old girl who presented with typical clinical features (pneumonia, sinusitis, otitis and diarrhoea) of Variable Primary Hypogammaglobulinemia for over 15 years. Immunodeficiency investigations showed a defective antibody response with a panhypogammaglobulinemia but normal cell-mediated immunity. Serum protein electrophoresis and detection of natural and acquired antibodies in the blood are the basic biological tests to run for a quick diagnosis. The main long term complication consists of the risk of neoplasia. The patient recovered a good clinical status after she was treated by IgG infusions every 2,5 weeks and antibiotics in case of superimposed infection.
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PMID:[Common variable hypogammaglobulinemia. Apropos of a new case]. 407 99

A severe combined immunodeficiency disorder was demonstrated in two Arabian foals which were full siblings. The defect in the B-lymphocyte system was shown by hypogammaglobulinemia, lymphopenia, and absence of germinal centers. The almost total absence of thymic tissue in one foal and the lack of thymic dependent lymphocytes in the spleens of both foals demonstrate a T-lymphocyte defect. In a retrospective study of total available Arabian foal cases, 4 of 15 had evidence of immunodeficiency.
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PMID:Hypogammaglobulinemia and thymic hypoplasia in horses: a primary combined immunodeficiency disorder. 419 58

We present data on 14 patients with chronic symptoms of disabling fatigue in association with serologic evidence of active Epstein-Barr virus (EBV) infection. Two thirds were women, and the average age at onset was 29.6 years. Forty-three percent were known to have had previous infectious mononucleosis, but the usual criteria for that diagnosis were not helpful with the present syndrome. Eighty-six percent had serologic evidence of cytomegalovirus (CMV) infection. Profound immunodeficiency was not present, but 71% had partial hypogammaglobulinemia, and minor abnormalities of T cell subsets were noted in six of seven patients studied. Fifty-seven percent achieved temporary serologic and symptomatic remission after an average duration of 33 months. Only one patient has a sustained remission. Comparison is made with other reported chronic, recurrent, and persistent EBV syndromes, and tentative diagnostic criteria for chronic mononucleosis syndrome are presented. Recently available EBV serologic techniques allow for identification of patients who have reactivated EBV infection, and this reactivation may be related to symptoms.
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PMID:Chronic mononucleosis syndrome. 609 68

The therapeutical use of gammaglobulin preparations in inborn immunodeficiency syndromes should be performed critically and only if an immunoglobulin lack exists which can be substituted. Immunodeficiency defects are listed according to the WHO-classification. For substitution of immunoglobulins plasma or different gammaglobulin preparations may be applied. The preparation of intravenous applicable preparations by different methods results in changes of half live times. The most important humoral immunodeficiency syndromes are the transitory hypogammaglobulinemia of infancy, the pathological hypogammaglobulinemia with delayed maturation of immunoglobulin-synthesis, the infantile X-linked agammaglobulinemia (Morbus Bruton) and the X-linked immunoglobulin deficiency syndrome with hyper-IgM. Intravenously applicable gammaglobulin preparations are preferred in the therapy the last two mentioned antibody deficiency syndromes which require large volumes. It has been demonstrated recently that these preparations are also suitable for continuous substitution.
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PMID:[Therapeutical application of gammaglobulin preparations in inborn immunodeficiency syndromes (author's transl)]. 617 May 72

Low levels of serum complement subcomponent C1q may accompany primary humoral immunodeficiency diseases such as sex-linked agammaglobulinemia, severe combined immunodeficiency, and common varied immunodeficiency. This selective depression of C1q is proportional to the degree of hypogammaglobulinemia, and is corrected in severe combined immunodeficiency by bone marrow transplantation or in hypogammaglobulinemia by immunoglobulin infusions, possibly because C1q is stabilized by IgG by reversible interactions which reduce extravascular degradation. In this study it is shown that a pH 4.0 treated intravenous gamma-globulin (ivGG) and a reduced and alkylated ivGG can equally increase levels of serum IgG, but that only the pH 4.0 preparation can raise C1q levels into the normal range. These findings show that some of the methods used to produce immunoglobulins suitable for intravenous use may hinder the ability of these molecules to stabilize Clq. The clinical implications of this observation remain unclear.
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PMID:Normalization of serum C1q after intravenous immunoglobulin infusions in hypogammaglobulinemia: dependence upon methods of immunoglobulin preparation. 620 68

Peripheral blood mononuclear cells from 16 patients with common variable immunodeficiency and 4 with selective IgA deficiency were studied for the quantitative analysis of T-cells and T-cell subsets with distinct immunoregulatory properties, using a battery of monoclonal antibodies and the fluorescence-activated cell-sorter. The proportions of OKT4+ cells were decreased and OKT8+ cells were increased in patient groups when compared to normal controls analyzed simultaneously. 14/20 (70%) patients demonstrated a lower OKT4+/OKT8+ cell ratio compared to controls. Imbalance of immunoregulatory T-cells may explain one of the mechanisms of hypogammaglobulinemia in a subgroup of patients with primary immunodeficiency.
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PMID:T-cell subpopulations defined with monoclonal antibodies in patients with primary immunodeficiency. 621 Jun 28

A panel of previously characterized monoclonal antibodies: OKT3, OKT4, OKT8, OKT10, OKT11, OKIa1, OKM2; 3A1, 4F2, UCTH1 and 5/9 were used to evaluate peripheral blood mononuclear cells in patients with severe primary immunodeficiencies: three patients with severe combined immunodeficiency, five with X-linked agammaglobulinemia, 20 with common variable hypogammaglobulinemia, 11 with IgA defect, and one with an unclassified form of T cell defect and hypogammaglobulinemia. Surface markers for T and B cells and in some cases functional assays, were also performed. Our results indicate a heterogeneous pattern in patients with severe combined immunodeficiency: one had peripheral blood mononuclear cells negative with all the monoclonal antibodies used; one had an increase in OKM2+ cells, whereas OKT3+ cells were absent; one had defect and imbalance of immunoregulatory T cell subpopulations. Major imbalances of T cell subsets were not detected in patients with X-linked agamma and IgA defect, whereas in some patients with common variable hypogammaglobulinemia an inversion of the physiological ratio between OKT4+ and OKT8+ cells was consistently detected. In an unclassified case of primary immunodeficiency, almost all peripheral blood mononuclear cells formed rosettes with sheep erythrocytes, but lacked antigens detected by monoclonal antibodies. Based on these observations, possible sites of defects in the T cell differentiation are discussed. We believe that monoclonal antibodies are useful for diagnosis, classification, and monitoring of therapy of primary immunodeficiencies.
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PMID:Monoclonal antibody analysis of T cell subsets in 40 patients with immunodeficiencies. 621 27


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