Cases reported "IgA Deficiency"

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1/14. Reversible total iga deficiency associated with phenytoin treatment.

    A 55-y-old male developed long-standing upper respiratory symptoms during phenytoin treatment of epileptic fits. Complete lack of serum IgA was noted repeatedly. Following replacement of phenytoin, normal IgA levels were found and a slow improvement in respiratory symptoms occurred. Immunoglobulin deficiency, in particular low levels of IgA, are not uncommon among phenytoin treated subjects. The occurrence of frequent respiratory infections in such patients justifies the investigation of immunoglobulin levels.
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2/14. Recurrent parotitis in selective iga deficiency.

    Recurrent parotitis is an uncommon condition in children. In most cases the etiology is unknown, although the disease is occasionally associated with viral infections, autoimmune disorders and immunodeficiency. We describe, for the first time, a child with recurrent parotitis and isolated immunoglobulin a (IgA) deficiency, without autoimmune disease. As IgA is the main immunoglobulin secreted into the mucosal surfaces, including that of the respiratory and gastrointestinal tracts, and into the saliva, the lack of IgA may be involved in the pathogenesis of recurrent parotitis. We recommend that IgA and other immunoglobulins be tested in all cases of recurrent parotitis.
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keywords = tract
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3/14. Protracted enteric cryptosporidial infection in selective immunoglobulin a and saccharomyces opsonin deficiencies.

    Chronic cryptosporidial infection in man usually occurs in those who are immunocompromised. We report a patient with a one year history of bowel symptoms resulting from persistent cryptosporidial infection of the colon. Investigations showed underlying selective IgA and saccharomyces opsonin deficiencies but no evidence of cell mediated immune dysfunction. Both selective immunoglobulin a and opsonin deficiencies are relatively common in the general population and may be a cause of susceptibility to persistent cryptosporidial infection.
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ranking = 151.12412173958
keywords = tract
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4/14. Selective iga deficiency and spondyloarthropathy: a distinct disease?

    A woman with selective iga deficiency and severe ankylosing spondylitis (AS), complicated by intractable peripheral arthritis, is described. Three previous cases of selective iga deficiency and AS have been reported, all of whom had severe AS. It is suggested that selective iga deficiency is a poor prognostic factor in AS and therefore warrants further investigation to determine the clinical course of such patients.
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5/14. Bacterial overgrowth after high-dose corticosteroid treatment.

    A 63-year-old man with systemic lupus erythematosus and selective iga deficiency developed intractable diarrhoea the day after treatment with prednisone, 50 mg daily, was started. The diarrhoea was considered to be caused by bacterial overgrowth and was later successfully treated with doxycycline. Although iga deficiency is a risk factor for bacterial overgrowth, a further predisposing condition is necessary for development of this disorder but was not present in this case. We therefore suppose that high-dose treatment with corticosteroids might be a hitherto undescribed risk factor for bacterial overgrowth in vulnerable patients.
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keywords = tract
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6/14. Serial immunologic and histopathologic studies in the treatment of necrotizing fasciitis with combined immunodeficiency by a bovine thymic extract (thymostimulin).

    A 25-day-old Chinese female baby developed necrotizing fasciitis (NF) with indurated erythematous patches with superficial necrosis over two thirds of the back. skin cultures of the lesions yielded streptococcus pyogenes, enterococcus, pseudomonas aeruginosa, and candida species. The patient had lymphopenia with low T cells and T cell subsets. The lymphoproliferative response to mitogen and macrophage migration (MIF) production were also poor. Her lymphocytes were unable to produce IgG in vitro. Also, IgG bearing cells were not demonstrable. The initial lymph node biopsy demonstrated an absence of follicular formation and depletion of lymphocytes in both thymic-dependent and thymic-independent areas. Five weeks after bovine thymic extract (Thymostimulin) treatment, a second lymph node biopsy demonstrated germinal centers containing IgG bearing cells. Three weeks later, a third lymph node biopsy showed germinal centers with cuffs of lymphocytes. Differentiation of the cortex and medulla of the node was demonstrated. IgG was also detectable in an in vitro Ig synthesis study. The total T cells, T cell subsets, lymphoproliferative response MIF production also increased gradually after Thymostimulin treatment. This investigation demonstrated the therapeutic effectiveness of Thymostimulin in NF with underlying combined immuno-deficiency, both serious and frequently fetal diseases, by the histologic and immunologic reconstitution of T and B cell function.
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ranking = 188.90515217447
keywords = tract
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7/14. A rare case of variable immune deficiency with type II dysgammaglobulinaemia, light chain defect, gut associated iga deficiency and progressive neutropenia.

    This report describes in detail an unusual variant of a common variable immunodeficiency disease in a seven-year-old boy. The unique features were progressive neutropenia due to defective myelopoiesis, serum IgG and IgA deficiencies, defective immunoglobulin light-chain synthesis, absence of secretory IgA and IgM gammopathy. He had been born healthy, but following a thermal injury at the age of 1 1/2 years, he suffered recurrent attacks of sinopulmonary and urinary tract infections, enteritis due to enteropathogenic E. coli, giardia lamblia and E. histolytica, developed pulmonary tuberculosis and died of deep mycotic infection of the oral cavity and obstruction of the bronchial tree. The cause of the defective myelopoiesis could not be determined, but it might have been due to prolonged sulphomamide therapy administered for controlling his persistent urinary tract infection due to paraphymosis.
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ranking = 5856.997652239
keywords = tract infection, tract
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8/14. malacoplakia associated with vesicoureteral reflux and selective immunoglobulin a deficiency.

    A case of malacoplakia involving the lower urinary tract of a young black boy, with associated bilateral vesicoureteral reflux, hydronephrosis and selective immunoglobulin a deficiency is reported. Reflux was caused by the malacoplakia. Reflux and hydronephrosis persisted despite elimination of bacterial infection and malacoplakia by drug therapy. These abnormalities were corrected by a conventional antireflux operation. malacoplakia appears to be related to immunologic incompetence and diminished levels of intracellular cyclic 3',5' guanine monophosphate. cholinergic agonists reverse or prevent the pathological changes of malacoplakia.
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keywords = tract
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9/14. blood transfusion reaction in a patient with immunoglobulin a deficiency.

    Selective deficiency of serum IgA is the most common immunodeficiency in humans; when immunodeficient individuals receive blood transfusions a severe anaphylactoid reaction can develop. The present report describes such a patient. After the transfusion reaction a hemagglutination inhibition assay revealed that her blood contained less than 1.0 micrograms/ml of IgA and an anti-IgA antibody that reacted with the 2 IgA proteins, isotypes IgA1 and IgA2. Immediately after the reaction the patient's serum anti-IgA antibody titer was 1:16,384, and when reevaluated 5 weeks later it was 1:8000. All 3 of her children were shown to be IgA deficient, and 2 of them had antibodies against IgA2. This type of anaphylactoid reaction can be avoided by transfusing blood from IgA-deficient donors, frozen deglycerolized red cells, or red cells that have been washed several times to extract all IgA proteins.
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keywords = tract
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10/14. Spontaneous recovery of selective iga deficiency. Additional case reports and a review.

    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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