Cases reported "Hypergammaglobulinemia"

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1/12. A novel case of immunodeficiency, centromeric instability, and facial anomalies (the ICF syndrome): immunologic and cytogenetic studies.

    The immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is characterized by hypogammaglobulinemia and recurrent bacterial infections. Here we report a novel case of ICF syndrome with hypogammaglobulinemia and an inverted CD4/CD8 ratio. Cytogenetically abnormal cells,that were identified in both CD4 and CD4- peripheral blood lymphocytes, retained their ability to proliferate in vitro following polyclonal stimulation. A primitive defect of B-cell differentiation was detected.
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ranking = 1
keywords = bacterial infection
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2/12. Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.

    Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.
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ranking = 5
keywords = bacterial infection
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3/12. An extremely basic monoclonal IgG in an aged apoplectic patient with prolonged bacterial infection.

    A case with prolonged bacterial infection accompanied by an abnormal serum protein which migrated in the post-gamma region on electrophoresis is presented. The abnormal protein was identified as IgG with gamma-type light chain moiety. The patient suffered from prolonged pneumonia and cholecystitis, Bone marrow aspiration and skeletal x-rays did not indicate multiple myeloma.
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ranking = 5
keywords = bacterial infection
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4/12. Hyper-IgM syndrome: a case report.

    Hyperimmunoglobulin M syndrome is a rare primary immunodeficiency disorder. We report a case of a 6-month-old boy who suffered from developmental delays, frequent respiratory tract infection, and unusual fungal and bacterial infection. X-linked hyperimmunoglobulin M syndrome was ultimately diagnosed with decreasing immunoglobulin-G, A, and E (immunoglobulin g = 51.3 mg/dL, immunoglobulin a = 8.32 mg/dL, immunoglobulin e <17.5 mg/dL), elevating immunoglobulin m (immunoglobulin m = 140 mg/dL), and decreasing T-cell expression of the cd40 ligand over flow cytometry. Seizure episodes and hypotonia developed with greater signal intensity at the putamen in a brain magnetic resonance imaging, which is compatible with hypoxic ischemic encephalopathy. This is the youngest proven case of hyper-IgM syndrome in taiwan ever reported.
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ranking = 1
keywords = bacterial infection
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5/12. De novo mutation causing X-linked hyper-IgM syndrome: a family study in taiwan.

    X-linked hyper-IgM syndrome (XHIM) is a rare primary immunodeficiency disorder caused by mutations of the gene encoding the cd40 ligand (CD40L). It is characterized by recurrent infections with markedly decreased serum IgG, IgA and IgE levels but normal or elevated IgM levels. We report the clinical manifestations and complete immune studies in the first family with molecularly proven XHIM in taiwan. A 5-month-old boy presented with rapidly progressive pneumonia which responded poorly to antibiotics. High levels of IgM and very low levels of IgG, IgA, and IgE were noted in his plasma specimen: IgM, 128 mg/dl; IgG, 18 mg/dl; IgA, 4 mg/dl); IgE, 1 IU/ml. Whole blood flow cytometry when he was 21 months old showed that only a small percentage (0.48%) of his in vitro-activated CD4 T cells expressed CD40L. When he was 3 years old, repeated flow cytometry showed essentially the same result (0.4%), compared with his father's CD40L expression of over 85%. The patient's mother had moderately decreased CD40L expression (74.4%). Hyper-IgM syndrome was confirmed by CD40L mutation analysis in the boy, which revealed a Lys 96 stop (nucleotide A307T) in exon 2 of CD40L, with a truncated protein resulting in the loss of the entire TNF domain. His mother was a carrier and apparently the individual in whom the mutation originated. Eleven other family members, including the patient's father, sister, and grandmother, and the mother's sisters and their children, all had normal results on CD40L mutation analysis. The patient has remained without significant bacterial infection on a regimen of monthly IVIG infusion and oral trimethoprim-sulfamethoxazole for pneumocystis carinii pneumonia (PCP) prophylaxis, although he has had recurrent oral ulcers and neutropenia. bone marrow transplantation is planned.
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ranking = 1
keywords = bacterial infection
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6/12. AIDS and antibodies to human immunodeficiency virus (hiv) in children and their families: clinical experience at Yale-New Haven Hospital.

    As of December 1986, we have identified 23 symptomatic children with human immunodeficiency virus (hiv) infection in New Haven. Twelve developed AIDS as manifested by lymphocytic interstitial pneumonitis, pneumocystis carinii pneumonia (PCP), and/or disseminated mycobacterial infections; seven of them have died. The remainder have milder clinical syndromes, which include failure to thrive, diffuse lymphadenopathy, and parotid swelling. When compared to adults with AIDS, children often have hypergammaglobulinemia and normal numbers of T4 lymphocytes. Intravenous drug abuse by the mother or mother's consort is the risk factor in 87 percent of these children. Two families have now been identified with more than one symptomatic child, but in no family is there evidence of spread from symptomatic children to uninfected siblings. A prospective study was begun to attempt to assess the risk of developing symptomatic hiv infection when a child is born to a mother with antibodies to hiv.
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ranking = 1
keywords = bacterial infection
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7/12. Clinical syndrome of chemotaxis defect, infections, and hyperimmunoglobulinemia E. A case report.

    A 17-year-old girl had a clinical syndrome that consisted of neutrophil chemotaxis defect, recurrent bacterial infections, and hyperimmunoglobulinemia E. Neutrophil chemotaxis was quantitated by a radioassay technique that used 51Cr-labeled neutrophils and a double micropore filter chemotaxis chamber. A table of the features of other cases of this syndrome is presented. Since ten of the 13 patients who are described had a chronic eczematoid dermatitis, we suggest that this may be a fourth feature of this syndrome. Red hair and fair skin were not consistent findings in these patients. In the evaluation of patients with chronic, recurrent bacterial infections of the skin of undetermined origin, we recommend determination of serum IgE levels. If IgE is found to be elevated, one should consider the presence of this syndrome, and neutrophil chemotaxis should then be examined.
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ranking = 2
keywords = bacterial infection
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8/12. A new defect of neutrophil chemotaxis and random motility in a child with recurrent bacterial infections and hyperimmunoglobulinemia E.

    A new defect of chemotaxis and random motility of polymorphonuclear leukocytes is described in a patient with severe recurrent bacterial infections since early childhood. This defect seems to be intrinsic to the cells. The patient's plasma did not contain an inhibitor of chemotaxis. Addition of plasma to the patient's cells restored their chemotactic activity. High concentrations of immunoglobulin E were found in the patient's plasma. phagocytosis, bacterial killing, and reduction of nitroblue tetrazolium salt were normal. Histological examination of lung tissue revealed a pattern similar to that observed in chronic granulomatous disease.
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ranking = 5
keywords = bacterial infection
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9/12. Defective leukocytotaxia and recurrent staphylococcal infecion: deficiency of leukocytotaxia and abnormal granulocytes associated with increase serum IgE levels in an adult with recurrent staphylococcal infection.

    A man who was suffering from recurrent staphylococcal infection had antecedent symptoms of severe pruritus. Laboratory investigations showed leukocytosis with eosinophilia, hyperimmunoglobulinemia of all fractions, but particularly of IgE, and a deficiency of cell-mediated immunity on in vivo testing. phagocytosis and bactericidal activity of polymorphonuclear leukocytes were normal, but a cellular and serum-associated defect in leukocytotaxia was present. Ultrastructural changes were observed in polymorphonuclear leukocytes. association of impaired leukocytotaxia and elevated levels of IgE is not uncommon. Recurrent bacterial infections in the patient described are probably related to defective chemotaxis.
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ranking = 1
keywords = bacterial infection
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10/12. Ascorbate therapy in impaired neutrophil and monocyte chemotaxis. With atopy, hyperimmunoglobulinemia E, and recurrent infection.

    A candida albicans corneal ulcer developed in a 24-year-old man with a history of eczema, asthma, and multiple bacterial infections since childhood. The infection responded well to oral flucytosine (12 g/day for 15 days) and topical amphotericin b. Positive laboratory findings included eosinophilla, hyperimmunoglobulinemia E, and impaired neutrophil and monocyte spontaneous migration and chemotactic responses. ascorbic acid corrected the monocyte defect in vitro and in vivo, but had no effect on neutrophil function.
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ranking = 1
keywords = bacterial infection
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