1/22. Selective antibody deficiency to bacterial polysaccharide antigens in patients with netherton syndrome.Three patients with netherton syndrome, recurrent sinopulmonary infections, and humoral immune deficiency are described. Although quantitative serum immunoglobulin levels were generally normal, two patients had selective antibody deficiency to bacterial polysaccharide antigens, one associated with IgA-IgG-2 deficiency. A third patient had an antibody deficiency to protein antigens. This is the first report, to our knowledge, that describes antibody deficiency in patients with netherton syndrome. This finding demonstrates the importance of evaluating functional antibody responses to both protein and bacterial polysaccharide antigens and not relying on IgG subclass determination.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/22. The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency.BACKGROUND: Most patients with chronic mucocutaneous candidiasis (CMC) have a selective defect of cell-mediated immunity against candida albicans (as demonstrated by cutaneous anergy and decreased lymphoproliferative responses to Candida antigen) and intact antibody responses. Many CMC patients also develop infections with other organisms, suggesting a more extensive immunologic defect. OBJECTIVES: The aim of this study was to describe a patient with CMC and selective antibody deficiency and identify eight similar previously reported patients. DATA SOURCES: Relevant articles in the English language derived from searching the medline database were used. RESULTS: We describe an 18-year-old male patient who was identified with CMC as an infant and later developed immunoglobulin (Ig)G2, IgG4, and iga deficiency at age 12 associated with poor antibody responses to vaccine antigens. We have identified eight other previously reported CMC patients with selective antibody deficiencies and bacterial infections. IgG2 deficiency was present in all nine patients, and was associated with IgG4 deficiency in 8 patients and iga deficiency in 3 patients. Six patients had poor or absent antibody responses to pneumococcal polysaccharide vaccine, and all nine patients developed severe recurrent lung infections. CONCLUSIONS: We suggest that these cases represent a distinct phenotype of CMC and should be studied for common histocompatibility leukocyte antigen types and molecular defects.- - - - - - - - - - ranking = 0.42857142857143keywords = antigen (Clic here for more details about this article) |
3/22. Extrinsic allergic alveolitis with iga deficiency.Up to now only 3 cases of extrinsic allergic alveolitis (hypersensitivity pneumonitis) with iga deficiency have been published worldwide. We had the opportunity to detect two additional cases which will be presented here. Summarizing all cases iga deficiency is a risk factor for a severe course of the disease and an increased susceptibility to acquire allergic alveolitis by low dose antigen exposure.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
4/22. Multicentric angiofollicular lymph node hyperplasia with peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis in women. A distinct syndrome.Four women with multicentric angiofollicular lymph node hyperplasia had a distinct clinical syndrome characterized by peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis. The nodes displayed typical morphologic changes of the plasma cell variant of multicentric angiofollicular lymph node hyperplasia. The pathologic changes are morphologically distinct from angioimmunoblastic lymphadenopathy with dysproteinemia although clinical similarities do exist. In these four cases, the lymphadenopathy was usually bulky and multicentric. There was frequent splenic involvement. The neuropathies were severe and disabling. Clinical courses have been variable with some responses to therapy with steroids and alkylating agents. No neoplastic transformations have occurred. Multicentric angiofollicular lymph node hyperplasia may represent a reactive lesion in which the antigenic stimulus is unknown but results in follicular hyperplasia, angiogenesis, and the systemic manifestations of hyperimmune stimulation. We believe this clinical syndrome may represent a distinct variant of multicentric angiofollicular lymph node hyperplasia, and it requires close observation for neoplastic transformation and other complications of its multisystem nature.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
5/22. Severe duodenal candidiasis in a patient with iga deficiency and T cell defects.candida albicans was found to repeatedly colonise and invade the duodenal ulcer base in a 45 years old otherwise healthy patient receiving H2 receptor antagonists for a prolonged period. He had no delayed hypersensitivity to Candida skin test, and had T cell deficiency, abnormality in T cell blast transformation, defective macrophage migration inhibition factor (MIF) and IgA hypogammaglobulinemia. When treated with ketoconazole alone his ulcer healed completely. Ulcer scar biopsy and aspirates revealed no Candida and anti candidal antibodies disappeared from his serum. His T cell blastoid transformation, MIF and skin DTH to Candida were restored to normal levels, but IgA levels remained unchanged. Thus H2 receptor antagonists probably caused abnormalities in T helper cells leading to lymphokine unresponsiveness and subsequently loss of cellular immunity to candidal antigen. This combined with prior IgA immunodeficiency resulted into severe invasive candidiasis.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
6/22. Combined IgG2, IgG4 and iga deficiency: low C1q concentrations and the presence of excess C1r and C1s in an adult patient with recurrent pneumococcal infections.The complement (C) profile was investigated in an adult patient with combined IgG2, IgG4 and iga deficiency and recurrent pneumococcal infections. The analysis revealed no gross impairment of the classic and alternative pathways of C activation. However, the concentrations of circulating C1q were persistently decreased, and the sera contained an excess of C1r-C1s complexes, resembling the C1 aberrations previously found in children with recurrent acute otitis media. The concentrations of C4 in the patient were persistently low. This could be ascribed to partial C4 deficiency with lack of C4A variants. The patient's IgG and IgM antibody responses to pneumococcal capsular polysaccharides and to other bacterial carbohydrate antigens were very poor. Interestingly, pneumococcal C-polysaccharide (CPS) could be detected in serum obtained during infection-free periods. Since CPS has been shown to bind C1q without causing C1 activation, the possibility was considered that the C1 aberrations in serum were due to circulating CPS. After administration of intramuscular gammaglobulin to the patient, the serum C1q levels were observed to return to normal.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
7/22. Hypogammaglobulinemia in infants infected with human immunodeficiency virus.Two premature infants were infected with hiv via blood transfusions during the neonatal period. Although neither patient had serum antibody to hiv owing to severe hypogammaglobulinemia, hiv infection was confirmed by finding hiv antigen in the sera of both patients. These cases show that hiv infection can produce severe hypogammaglobulinemia, and illustrate the value of hiv antigen detection in the diagnosis of hiv infection in seronegative patients.- - - - - - - - - - ranking = 0.28571428571429keywords = antigen (Clic here for more details about this article) |
8/22. Pulmonary sarcoidosis associated with acquired humoral and cellular immunodeficiency.A previously healthy 27-year-old man with class II pulmonary sarcoidosis developed severe humoral immunodeficiency within the course of the disease with an IgG of less than 250 mg/ml and undetectable levels of IgA and IgM. Repeated skin tests were negative for seven common recall antigens. Cellular blood test demonstrated normal numbers of B cells and slight T-cell lymphopenia with a normal T-helper/suppressor subset distribution (ratio 1.6). In contrast, parallel examination of the bronchial alveolar lavage fluid (BAL) demonstrated highly elevated numbers of T cells with a subset ratio of 3.1 and significant numbers of activated T cells as revealed by the expression of Ia and Tac antigens. Functional in vitro assays showed a greatly decreased mitogenic response of blood T cells and diminished production of immunoglobulins. These data indicate that, despite a severely depressed systemic humoral and cellular immune system, T-cell activation can take place at the inflammatory site, potentially causing the lesions characteristic of sarcoidosis.- - - - - - - - - - ranking = 0.28571428571429keywords = antigen (Clic here for more details about this article) |
9/22. Evan's syndrome, chronic active hepatitis and focal glomerulonephritis in iga deficiency.A 10-year-old female with a complete selective iga deficiency and recurrent autoimmune disease (chronic active hepatitis, focal glomerulonephritis, hemolytic anemia and thrombopenic purpura) is presented. Both serum IgA and saliva secretory IgA were below the detection limit. The small bowel biopsy using a peroxidase-antiperoxidase technique showed absence of plasma cells secreting IgA. Circulating antibodies against mitochondria, microsomal thyroid antigen were detected as well as rheumatoid factor. Circulating immune complexes were present. A positive Coombs' test and a slightly positive reaction for cryoagglutinins were demonstrated. No alterations in cellular immunity were observed. Clinical and analytical improvement with prednisone and azathioprine was obtained.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
10/22. Improvement of cellular immunity and IgA production in immunodeficient children after treatment with synthetic serum thymic factor (FTS).Three children with IgA and IgE deficiency and T-cell defects (two related patients with ataxia telangiectasia and one with common variable immune deficiency) were treated with synthetic serum thymic factor (FTS) intravenously. A reduction in frequency and severity of infection was noted concomitantly with improvement in cell-mediated-immunity tests. serum IgA, which was absent in two patients, appeared within 4 weeks of treatment and increased significantly in the third patient. Specific antibodies against vaccination antigens appeared for the first time or increased to titres higher than ever before. In two patients, transient interruption of FTS administration was followed by a regression of the immunological improvement, but this disappeared after the treatment was started again.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
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