1/16. 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) |
2/16. rothmund-thomson syndrome with herpes encephalitis.A 4-year-old Japanese boy with rothmund-thomson syndrome suffered from severe herpes encephalitis at 5 months of age. The serum level of immunoglobulin g was low and the responsiveness of peripheral blood mononuclear cells to bacterial superantigens was poor. It was suggested that these immunological abnormalities, possibly associated with rothmund-thomson syndrome, led to severe infection with herpes simplex virus in our patient.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
3/16. Immunologic compensation in a patient with a large IgH constant region deletion.BACKGROUND: Deficiencies of serum Ig of the IgG isotype typically predispose individuals to recurrent infections in some but not all cases. patients with large deletions of the Ig heavy chain genes are free of recurrent and severe infections. OBJECTIVE: We sought to determine a mechanism of immunologic compensation that would possibly explain the reason for this patient's paucity of infection despite lacking several classes of serum Ig. methods: The patient is a 50-year-old white man. serum Ig levels and specific antibody titers were measured by using various methods, including nephelometry, enzyme immunoassay, and radial immunodiffusion. The status of the Ig heavy chain genes was examined by means of Southern blotting of genomic dna isolated from EBV-transformed B cells. RESULTS: The patient's serum lacked detectable IgG1, IgG2, IgG4, and IgA1 levels. Southern blot analysis demonstrated a large heavy chain constant (C) region gene deletion that included Cgamma1, Calpha1, psiCgamma, Cgamma2, and Cgamma4. Antibody responses to capsular pneumococcal and hemophilus polysaccharide antigens were essentially absent. However, IgG3 antibodies against the protein antigen tetanus toxoid were present. Relatively high antibody titers were found against pneumococcal surface proteins as well. CONCLUSION: We conclude that our patient's relative freedom from serious infection may be as a result of production of IgG3 antibodies to pneumococcal capsular proteins.- - - - - - - - - - ranking = 0.28571428571429keywords = antigen (Clic here for more details about this article) |
4/16. 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) |
5/16. Transverse sinus thrombosis and IVIg treatment: a case report and discussion of risk-benefit assessment for immunoglobulin treatment.A 54 year old woman presented with symptoms resulting from a thrombosis of the lateral transverse and sagittal sinuses the day after an infusion of intravenous immunoglobulin (IVIg) replacement treatment. She had previously suffered a milder episode after IVIg. Following recurrent bacterial chest infections and sinusitis for more than 40 years, a diagnosis of IgG1 deficiency had been made two years earlier, after exclusion of other causes. She made a good recovery from the thrombosis but high platelet counts were investigated and primary thrombocythaemia was diagnosed. Investigation of humoral immunity revealed protective amounts of IgG antibodies to pathogens, and because the previous IgG1 deficiency had resolved IVIg infusions were not restarted. She made a good response to treatment with hydroxyurea, with improvement of the headaches and lowering of the platelet counts. Prophylactic antibiotics reduced the number of bacterial chest infections and nasal corticosteroids improved the chronic sinusitis. This case is presented to highlight the need to look for other contributing factors for severe recurrent headaches after IVIg treatment, and to consider the risk of thrombosis even when replacement doses of IVIg are used. It is also important to emphasise the need to ensure that an isolated IgG subclass deficiency is not transient; that failure to produce specific IgG antibodies to immunisation and/or exposure antigens is confirmed, thus meeting the criteria for the diagnosis of primary antibody deficiency. A thorough risk-benefit assessment is essential before blood product treatment is started.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
6/16. Coexistent yellow nail syndrome and selective antibody deficiency.BACKGROUND: yellow nail syndrome (YNS) is a rare, often underdiagnosed condition of unknown origin. The clinical features of the syndrome include yellow nails, chronic sinusitis, bronchiectasis, pleural effusion, and lymphoedema. Despite the frequent occurrence of upper and lower respiratory tract infections in patients with YNS, comprehensive analysis of their humoral immunity has not been previously reported. OBJECTIVE: To present the case of a patient with YNS whose recurrent upper and lower respiratory tract infections may have been caused by an underlying selective antibody deficiency that manifests as impaired IgG antibody response to polysaccharide antigens. methods: The patient underwent cultures of purulent sputum for streptococcus pneumoniae and haemophilus influenzae, bronchial washings for H. influenzae, and nail scrapings for fungi. Her serum levels of IgG, IgA, IgM, IgG subclasses, and serum titers of IgG antitetanus toxoid, anti-H. influenzae, and anti-S. pneumoniae antibodies were measured. RESULTS: Cultures of purulent sputum were positive on multiple occasions for S. pneumoniae and H. influenzae and bronchial washings were positive for H. influenzae. Nail scrapings were consistently negative for fungi. She had no reductions in serum levels of IgG, IgA, IgM, or IgG subclasses and had normal serum titers of IgG antitetanus toxoid antibodies. However, she demonstrated impaired IgG antibody responses following immunization with Pneumovax and an H. influenza B vaccine. CONCLUSIONS: This case report describes the first comprehensive analysis of humoral immune function in a patient with YNS. The finding of a selective antibody deficiency in our patient provides a potential explanation for the occurrence of respiratory infections in YNS. Accordingly, we recommend that functional antibody determinations and quantitative serum immunoglobulins be evaluated in patients diagnosed as having this unusual, enigmatic syndrome.- - - - - - - - - - ranking = 0.14285714285714keywords = antigen (Clic here for more details about this article) |
7/16. 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 = 0.28571428571429keywords = antigen (Clic here for more details about this article) |
8/16. 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) |
9/16. 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) |
10/16. Disseminated cryptococcal infection in a patient with hypogammaglobulinemia and normal T cell functions.The immunologic status of a patient with disseminated cryptococcosis and no underlying predisposing factor was evaluated. The proportions and numbers of T cells, T cell subsets, B cells and proliferative responses to mitogens and soluble antigens, including cryptococcal antigen, were comparable to those in healthy control subjects; however, the patient had moderately severe hypogammaglobulinemia. The patient continues to do well clinically with intravenous gammaglobulin and antifungal therapy. This study suggests that antibodies might also play a role in the defense against cryptococcal antigen.- - - - - - - - - - ranking = 0.42857142857143keywords = antigen (Clic here for more details about this article) |
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