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)

We describe here one 8-year-old girl with an unusual form of immunodeficiency, characterized by hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and chronic lymphadenopathy. Patient's B cells failed to produce IgG or IgA in vitro following stimulation with either pokeweed mitogen or Epstein-Barr virus, suggesting an intrinsic B-cell defect. Abnormal T-cell function was demonstrated by impaired in vivo delayed type hypersensitivity, reduction of mitogen-induced proliferation and interleukin 2 production, reduction of interferon-gamma production, and marked decrease of circulating OKT4+ cells. The latter cells were found in normal proportion in the patient's lymph node tissue. This finding suggests that the decrease of OKT4+ cells in peripheral blood was due to the abnormal recirculation of these cells. The identity of this syndrome with the infantile form of the acquired immunodeficiency syndrome was apparently ruled out by the failure to demonstrate HTLV-III-related sequences in patient's lymphocytes or virus-specific serum antibodies.
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PMID:Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy. 307 86

A boy aged 6 months who presented with poor weight gain, diarrhoea, and infection with Pneumocystis carinii was found to have congenital hypogammaglobulinaemia, which did not improve despite monthly treatment with intravenous gammaglobulin. At the age of 3 years and 2 months he developed severe vomiting and diarrhoea due to cryptosporidiosis, which failed to respond to conventional treatment. Infusion of hyperimmune bovine colostrum produced against parasite antigen, given by nasogastric tube, was started after symptoms had persisted for three weeks. His vomiting and diarrhoea resolved within five days of treatment, and oocysts were no longer seen in the stools after eight days. Later, however, he developed a rare complication, and oocysts were found in the common bile duct. Hyperimmune bovine colostrum may be useful in the treatment of many patients with immunodeficiency disorders.
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PMID:Remission of diarrhoea due to cryptosporidiosis in an immunodeficient child treated with hyperimmune bovine colostrum. 309 62

Arthritis may be the first clinical manifestation of primary hypogammaglobulinaemia. In 16 years of 281 patients who had immunodeficiency, 30 had arthritis at presentation. It was more common in Bruton's disease (15 (22%) of 69 patients) than in other forms of immunodeficiency (15 (7%) of 212 patients). Non-septic arthritis was more prevalent than septic arthritis, particularly monoarticular arthritis in Bruton's disease and pauciarticular disease in common variable immunodeficiency. Boys in whom a diagnosis of Bruton's disease was delayed were likely to develop recurrent infections complicated by arthritis. The measurement of serum immunoglobulin concentrations readily differentiates immunodeficiency from conditions such as Still's disease and dictates subsequent management.
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PMID:Primary hypogammaglobulinaemia and arthritis. 311 63

Human immunoglobulin for intravenous (IV) use has an established safety record with regard to transmission of hepatitis B virus. The bulk of available evidence also suggests that the human immunodeficiency virus (HIV) is not transmitted by IV immunoglobulin. There has been one report, however, of isolation of HIV from two patients with hypogammaglobulinaemia who had been treated with several immunoglobulin products. Certain IV immunoglobulin products have transmitted non-A, non-B (NANB) hepatitis but careful clinical assessment of recipients of other products suggests that non-infective preparations can be made. Interpretation of available data most likely to be correct is that contamination with NANB is reduced but not eliminated by cold-ethanol fractionation and that the use of further virucidal procedures in the finishing of immunoglobulin products will confer a higher degree of safety.
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PMID:The viral safety of intravenous immunoglobulin. 311 93

Immune serum globulin has been available for approximately 40 years. Although this substance represented a major advance in the treatment of patients with agammaglobulinemia and hypogammaglobulinemia, it has a number of major limitations that restrict its clinical utility. These include the need for intramuscular administration, pain at the site of injection, loss of immunoglobulin G (IgG) extravascularly, limitations on the degree to which serum IgG can be increased, incomplete and delayed onset of absorption, and limitations on the volume of material administered. Intravenous forms of gamma globulin do not have these limitations and, therefore, have been preferred for therapeutic use. While studying the physical chemistry of IgG in solution, it was observed that lowering the pH to the range of 4.0 to 4.5 markedly enhanced its monomer content and stability, obviating the need for any chemical modification, enzymatic treatment, or lyophilization. A new IgG preparation suitable for intravenous administration, IGIV, pH 4.25, has been developed and subjected to extensive clinical testing. It is licensed in the United States (Gamimune N) for replacement therapy of IgG in immunodeficiency syndromes and for the treatment of idiopathic thrombocytopenic purpura. The biochemistry and safety of IGIV, pH 4.25, are reviewed.
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PMID:Overview of the biochemistry and safety of a new native intravenous gamma globulin, IGIV, pH 4.25. 311 8

We report on a case of nodular lymphoid hyperplasia (NLH) of the small intestine in a patient with common variable immunodeficiency (CVID) syndrome. The CVID syndrome comprises a group of heterogeneous immunological disorders. It is characterised by hypogammaglobulinemia, recurrent sinopulmonary infections, gastrointestinal disorders (including diarrhea, infestation with Giardia lamblia, chronic-atrophic gastritis and nodular lymphoid hyperplasia (NLH), and an increased risk of malignancy. NLH is frequently associated with gastrointestinal lymphomas. It has also been found in the terminal ileum of children and in adult patients with Gardner's syndrome. NLH is found in about 20% of patients with the CVID syndrome. The diagnosis of NLH requires endoscopic and bioptic-histological examinations and the determination of the immunoglobulins.
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PMID:Common variable immunodeficiency syndrome and nodular lymphoid hyperplasia in the small intestine. 316 41

Patients with common varied immunodeficiency (CVI) are characterized by hypogammaglobulinemia. We investigated in vitro T cell-dependent B cell differentiation in CVI peripheral blood mononuclear cells by stimulating the T cells with an anti-CD3 (T3) monoclonal antibody. In cultures from CVI patients with no detectable circulating B cells, little immunoglobulin (Ig) was produced following anti-CD3 stimulation. In cultures from CVI patients with near-normal numbers of circulating B cells, anti-CD3 stimulation induced a normal percentage increase in Ig-secreting cells and appreciable (albeit subnormal) increases in IgG and IgM secretion. Cell-mixing experiments pointed to a quantitative, rather than qualitative, defect in B cell function in most of these CVI patients. Nevertheless, CVI T cells can induce substantial differentiation of autologous (and normal) B cells following anti-CD3 stimulation (which may mimic physiologic stimulation). This raises the possibility of correcting the hypogammaglobulinemia of CVI by in vivo or ex vivo administration of appropriate T cell stimuli.
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PMID:In vitro induction of T cell-dependent B cell differentiation in patients with common varied immunodeficiency. 326 71

In screening for defects in humoral immunity, as well as measuring serum immunoglobulins it is advisable to have some measure of antibody production, e.g., immunising with a test antigen and measuring the antibody response some days later. However, this delay may be inconvenient and requires a second blood sample. An alternative is to test for antibody to a widespread commensal organism to which the patient must have been repeatedly exposed. The measurement of antibody to E. coli by haemagglutination has been previously used for this purpose. We report here that an equally good and simpler alternative is to measure antibodies to the commensal Candida albicans by immunofluorescence. Using a polyvalent conjugate, all 114 blood donors tested had antibody titres greater than 8 to C. albicans; similar responses were noted in 20 children (aged 6 months-16 years) without recurrent infections. In contrast, anti-candida responses were low or absent as expected in patients with hypogammaglobulinaemia but also in some patients with other immunodeficiency diseases. Overall candida immunofluorescence closely paralleled E. coli haemagglutination.
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PMID:Measurement of antibodies to Candida albicans as a screening test for humoral immunodeficiency. 331 4

Currently recognized equine immune deficiency disorders include failure of passive colostral immunoglobulin transfer, combined immunodeficiency, selective IgM deficiency, transient hypogammaglobulinemia, and agammaglobulinemia. Additional equine immune deficiency disorders probably exist. Immune deficiency should be suspected in any horse experiencing persistent or recurrent infections that are not responsive to conventional therapy.
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PMID:Evaluation of foals for immune deficiency disorders. 332 24

Acquired immunodeficiency syndrome (AIDS) in infants has different clinical and immunological characteristics from adult AIDS because of immunological immaturity of the fetus and newborn when infection is produced. Differential diagnosis with primary immunodeficiency diseases, mainly with severe combined immunodeficiency and hypogammaglobulinemia is often difficult, but clinical, epidemiological and immunological data aid in establishing diagnosis. Repeated bacterial infections and abnormal antibody production are common in such children and gammaglobulin therapy is indicated to prevent them and avoid continuous immunological stimulation that viral replication and disease progression.
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PMID:[Diagnostic clinical and immunologic characteristics of infection by the human immunodeficiency virus in infants]. 335 37


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