1/25. A paraprotein in severe combined immunodefeciency disease detected by immunoelectrophoretic analysis of plasma.A qualitative study was made of the plasma immunoglobulins of a child with severe combined immunodeficiency. By immunoelectrophoresis an immunoglobulin with an abnormal electrophoretic mobility was detected. This protein possessed mu heavy chain determinants, gave no detectable reaction with antisera specific for light chains, was of a relatively small molecular size, and was probably not composed of subunits held together by easily reduced disulfide bonds. The light chains that were present in this patient's plasma had a homogeneous electrophoretic mobility. The patient's plasma also contained at least two other immunoglobulins whose antigenic identity could not be established. One of these was abnormal in its electrophoretic mobility. The presence of the abnormal protein with mu determinants in the plasma of the second unrelated child with a similar disease suggests that the detection of this protein may have implications for the diagnosis or classification of immunodeficiency diseases.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/25. Selective immunoglobulin m (IgM) deficiency in two immunodeficient adults with recurrent staphylococcal pyoderma.Two adult men with recurrent pyoderma due to staphylococcus aureus and a selective deficiency of immunoglobulin m (IgM) antibody synthesis are described. An analysis of each patient's polymorphonuclear leukocyte chemotaxis, phagocytosis and killing of Staph. aureus, serum opsonizaiton of Staph. aureus, and serum and lymphocyte-mediated responses to antigenic stimulation was performed. family studies revealed a possible autosomal dominant inheritance pattern with heterogenetic expression of various dysgammaglobulinemic states in each patient's first degree relatives. In vivo studies of delayed hypersensitivity and in vitro studies of polymorphonuclear leukocyte and lymphocyte function were normal. A defect in IgM, but not in IgG (immunoglobulin G), antibody synthesis to a number of antigens, and a mild decrease in serum opsonic activity to Staph. aureus correctable by heat inactivated normal human serum were found in each patient. In these patients, the recurrent staphulococcal pyoderma prompted an investigation of host defense mechanisms and revealed low to absent IgM levels and a defect in IgM antibody synthesis.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/25. association of autoimmunity with IgG2 and IgG4 subclass deficiency in a growth hormone-deficient child.An association between humoral immune deficiency and childhood autoimmune disease has been previously established. We describe a 7-year-old male with severe autoimmune disease, recurrent infections, a marked deficiency of IgG2 and IgG4, and an inability to respond to polysaccharide antigens. This child was also found to have isolated growth hormone (GH) deficiency. Laboratory results included a positive anti-smooth muscle antibody, a positive Raji-cell assay for immune complexes, and normal levels of IgG, IgM, and IgA. IgG subclasses revealed an IgG1 of 1225 (normal for age, 280-1120 mg/dl), IgG2 of less than 10 (30-630 mg/dl), IgG3 of 36 (40-250 mg/dl), and IgG4 of less than 4 (11-620 mg/dl). No increase in antibody titer was noted to either Pneumovax or unconjugated haemophilus influenzae vaccine. Numbers of circulating B cells (CD19) were markedly diminished (less than 0.5%). Liver biopsies have shown chronic active hepatitis. Somatomedin C was 0.28 U/ml (normal for age, 0.5-2.06 U/ml). Challenge with either L-dopa or clonidine produced a peak GH response of 2.3 ng/ml (normals = greater than 7 ng/ml). Children with autoimmune disorders should be evaluated for IgG subclass deficiencies and ability to make antibody in response to antigen challenge regardless of the serum immunoglobulin levels. Growth failure in immune-deficient children should not be assumed to be due to chronic illness or recurrent infections. Other etiologies for growth failure should be sought.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/25. 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 = 1keywords = antigen (Clic here for more details about this article) |
5/25. 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 = 1keywords = antigen (Clic here for more details about this article) |
6/25. 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 = 1keywords = antigen (Clic here for more details about this article) |
7/25. 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 = 2keywords = antigen (Clic here for more details about this article) |
8/25. 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 = 1keywords = antigen (Clic here for more details about this article) |
9/25. Selective iga deficiency and circulating immune complexes containing bovine proteins in a child with chronic graft versus host disease.We have previously shown that a selective absence of serum and secretory immunoglobulin a (IgA) may lead to the development of circulating immune complexes which appear to contain bovine milk antigens. We report here that high levels of circulating immune complexes were found in the serum of a child who was treated for severe combined immunodeficiency by bone marrow transplantation but in whom the IgA-producing cells subsequently failed. As increasing amounts of complexes appeared over a two year period, the child had a parallel progression of an apparent chronic graft versus host disease including a Sjogrens syndrome and scleroderma. Very large amounts of complexes were eventually formed but the level fell 77 per cent after milk was excluded from the diet. Chemical studies on the complexes showed that the majority of complexes did contain bovine milk proteins, and fluorescence antibody staining of skin biopsy samples showed the presence of dense deposits of bovine casein in the dermis. The relationship between bovine protein-antigen antibody complexes and the chronic graft reaction remains uncertain.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
10/25. Fibrosing alveolitis and hepatitis b surface antigen-associated chronic active hepatitis in a patient with immunoglobulin a deficiency.Fibrosing alveolitis is described in a 22 year old woman with immunoglobulin a (IgA) deficiency and hepatitis b surface antigen (HBsAg)-associated chronic active hepatitis. At lung biopsy HBsAg was detected by indirect immunofluorescence in the alveolar space but not in the septal fibrosis. We discuss the possible relationships between iga deficiency on the one hand, and HBsAg-associated lung and liver diseases on the other hand.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
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