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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six Polynesian children aged 8-14 years with recurrent lower respiratory infections and abnormal lung mucociliary clearance were assessed for immuno-deficiency. Measurements were made in these 6 children and in 21 healthy Polynesians of serum immunoglobulins (IgG, IgM, IgA) and complement components (C3, C4), leucocyte function tests, and T and B cell populations. A further 8 Polynesian bronchiectatics were tested for sputum secretory-IgA. There was no evidence of S-IgA deficiency and 4 had high levels. There was no evidence of immunodeficiency. Nasal epithelium was obtained from 6 children using a cytology brush and ciliated epithelium was obtained in only 4. The ultrastructure was normal although few cilia were examined.
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PMID:Polynesian bronchiectasis. 657 56

Immunological and genetic studies were performed in nine members from three generations of the family of a patient with common variable immunodeficiency (CVI). Two additional symptomatic members (mother and grandmother) had CVI. Among other six asymptomatic members, two had CVI and one had selective IgA deficiency. The proportions of monoclonal antibody defined total T cells (Leu 1+), helper phenotype (Leu 3+) suppressor phenotype (Leu 2+) T cells, natural killer cells (Leu 7+) and surface Ig+ B cells and proliferative response to phytohaemagglutinin (PHA), concanavalin A (Con A), pokeweed mitogen (PWM) and in mixed lymphocyte reaction (MLR) were comparable to controls. Addition of purified interleukin-2 (IL-2) resulted in augmentation of PHA-induced proliferation of T lymphocytes similar to that seen in the controls, however with IL-2 freshly isolated T cells in the absence of PHA demonstrated markedly increased proliferative response, suggesting the presence of in vivo activated T cells. Study of HLA phenotype did not reveal any linkage. This study demonstrates the genetic nature, possibly autosomal dominant inheritance, of common variable immunodeficiency; however the immunodeficiency is not linked to any specific HLA antigen.
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PMID:Immunological analysis in familial common variable immunodeficiency. 660 34

A receptor for IgA was observed on a subset of T cells (T alpha) that is distinct from other T lymphocyte subsets, T mu or T gamma cells. IgA receptor on T alpha cells is blocked by IgA from human serum through its cytophilic attachment. Neither T mu nor T gamma cells, following an in vitro interaction with insoluble immune complexes during the process of purification and further incubation at 37 degrees C, changed their phenotypes to T alpha cells. However, some T gamma cells demonstrated transition to T mu cells. The numbers and proportion of T alpha cells in patients with selective IgA deficiency were either normal, increased or decreased. The significance of T alpha cell analysis in thirty-one patients with primary immunodeficiency disorders including those with selective IgA deficiency is discussed.
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PMID:Subpopulations of human T lymphocytes. XV. T lymphocytes with receptors for IgA (T alpha), a distinct subpopulation of T lymphocytes. Studies in patients with primary immunodeficiency disorders. 696 55

Four of 18 patients with common variable immunodeficiency and one of eight patients with selective IgA deficiency had increased proportions of T epsilon cells. Three patients with common variable immunodeficiency and two with selective IgA deficiency had increased numbers of T epsilon cells. Three patients with common variable immunodeficiency had decreased numbers of T epsilon cells. Five of six patients with hyperimmunoglobulinaemia E had high proportions of T epsilon cells; this increase was not related to the elevated IgE levels.
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PMID:Subpopulations of human T lymphocytes. XVIII. T lymphocytes with receptors for IgE (T epsilon) in patients with primary immunodeficiency and hyperimmunoglobulinaemia E states. 697 77

Patients with selective IgA deficiency, the most common primary immunodeficiency, occasionally may undergo spontaneous recovery. In this paper we present three additional children whose IgA levels spontaneously returned to normal. All three presented with gastrointestinal symptoms, and one child also had frequent upper respiratory infections. We review other cases of spontaneous IgA deficiency, and the role of exogenous function in its etiology, and possible mechanisms of recovery.
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PMID:Spontaneous recovery of selective IgA deficiency. Additional case reports and a review. 705 10

Tracheobronchial mucociliary clearance was studied in six patients with immunoglobulin deficiency. They inhaled a test aerosol of 6 microns teflon particles tagged with 99mTc, after which external measurements of the retention of test particles in the lungs were made during 2 h. Four younger patients with common variable immunodeficiency and severe symptoms of airway infections all had a markedly slow clearance. In one of these cases clearance was measured again after 6 months of adequate medical treatment and was found much improved. Two older patients with less severe immunoglobulin deficiency (one with selective IgA deficiency and one with transient hypogammaglobulinemia), and with a history of less severe airway infections, had normal clearance. The results indicate that chronic infection of the airways, if severe enough, will damage the mucociliary transport system, but that this damage is not necessarily irreversible. Ciliary ultrastructure was investigated by electron microscopy in two of the younger patients with slow clearance, and was found to be normal.
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PMID:Mucociliary clearance in patients with immunoglobulin deficiency. 717 52

Eight of 400 (2%) unselected dermatological patients showed an IgA deficiency in their serum. Two had a complete and four an isolated deficiency of serum IgA. In three patients the IgA deficiency was combined with a deficiency of another immunoglobulin (antibody deficiency syndrome), and twice with a cellular immunodeficiency. An elevated IgE level was found in the serum of one patient. Most patients with IgA deficiency had recurrent infections of the skin and mucous membranes, or the dermatoses were caused secondarily by an inflammatory process of the latter. In contrast to the deficiency of serum IgA the concentration of secretory IgA in the saliva was normal. The immunotherapy of serum IgA deficiency depended on the existence or absence of associated immunological disorders: in case of isolated IgA deficiency substitution with enriched IgA, in antibody deficiency syndrome with gamma globulin and in combined humoral and cellular immune deficiency with additional therapy with transfer factor.
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PMID:[IgA deficiency in the dermatologic clinic. Frequency, clinical relevance, diagnosis and therapy]. 729 30

In 1974 and 1975 7000 patients' sera were tested for levels of IgG, IgA and IgM. In 330 patients at least one of the three Ig classes was low. In most instances secondary immunodeficiency was present in association with myeloma, etc. However, 81 patients above 8 years of age fulfilled the criteria of idiopathic late onset Ig deficiency. In 44 of these patients clinical follow-up and repeated measurements of Ig levels were possible 1--8 years after the initial diagnosis. Selective IgA deficiency was present initially (15 patients) most frequently and persisted most often (14 patients). 4 patients had initially low IgG and 6 patients low IgM, findings which were only rarely confirmed later on. In 19 patients 2 or 3 Ig classes were initially low, with persistence of Ig deficiency in 12 individuals. In no instance had clinical symptoms appeared in the first two years of life. The following diseases were documented in the 44 patients studied (28 individuals with persistent and 16 with transitory Ig deficiency): recurrent infections (16 patients), atopic disease (8 times), rheumatoid arthritis (6 times), epilepsy (4 times), SLE (3 times) and enteropathies (twice). Seven patients also had a malignancy, 4 diabetes, and 2 hyperthyroidism.
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PMID:[Idiopathic immunoglobulin deficiency in juveniles and adults. Catamnestic studies]. 739 75

From the Immunodeficiency Cancer Registry, it has appeared that there is an increasing frequency of neoplasia in individuals who have a selective absence of serum IgA. Approaching this question from another point of view, we have found that of 4,120 sera drawn in this cancer-oriented hospital, 12 sera had a total absence of IgA and 3 additional sera had less than 10 mg/dl. The incidence of IgA deficiency in a cancer hospital is thus 1 : 342 or 1 : 273, which is statistically similar to that previously found for other patient groups studied in the USA (average of two studies, 1 : 418), but it is substatistically increased over the incidence of IgA deficiency found in normal blood donors (average of five studies 1 : 1,677). Analysis of these sera by diagnostic categories showed that of 1,517 sera of patients with lymphoproliferative disorders, 6 were IgA deficient (frequency 1 : 253), and of 249 sera of patients with gastrointestinal neoplasm, 2 were IgA deficient (frequency 1 : 125). We conclude that, in the absence of IgA, certain organ systems, the gastrointestinal and lymphoid tissue may be at increased risk for malignant change and that the protective, anti-neoplastic role of IgA requires investigation.
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PMID:Selective IgA deficiency and neoplasia. 740 56

Ataxia telangiectasia (AT) is an autosomal recessive disorder characterized by telangiectasia, progressive ataxia, sinopulmonary infections and a combined immunodeficiency (usually consisting of IgA deficiency, IgE deficiency, IgG2 and IgG4 deficiency and a disturbed T cell immunity). The alpha-fetoprotein level is elevated. Cytogenetic studies reveal a very specific chromosome instability with multiple chromosome 7 and/or 14 rearrangements (preferential breakpoints 14q32, 14q12, 7q35 and 7p12). X-ray hypersensitivity is one of the hallmarks of the disease. Nijmegen Breakage Syndrome (NBS), an autosomal recessive disorder with some features of AT, was first reported in 1981. At this moment at least 19 patients have been recognized. Clinical symptoms are microcephaly from birth, a peculiar face, growth retardation, repeated respiratory tract infections and renal abnormalities. Immunological, cytogenetic and cell-biological findings in NBS are identical to AT. However, alpha-fetoprotein levels are not increased. A tendency toward malignancy has been demonstrated in both syndromes. Recently, we encountered three patients with variants of these syndromes.
Immunodeficiency 1993
PMID:Variants of Nijmegen breakage syndrome and ataxia telangiectasia. 751 25


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