Cases reported "Dysgammaglobulinemia"

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21/156. Selective iga deficiency associated with glomerulonephritis and oligoarthritis.

    A 59 year old woman with selective iga deficiency associated with oligoarthritis and glomerulonephritis is described. She was seropositive for rheumatoid factor and renal histological examination showed a focal glomerulonephritis. High titre rheumatoid factor and a focal glomerulonephritis were also present in the only other well documented report of selective iga deficiency and renal disease. Histological examination of the kidney suggested that the glomerulonephritis was mediated by immune complexes.
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22/156. Dysgammaglobulinaemia in the elderly--a review and case studies.

    An understanding of the possible causes of dysgammaglobulinaemia in the elderly helps to direct further investigation to establish a diagnosis. In this review we provide brief case studies to illustrate some of the disorders associated with dysgammaglobulinaemia in the elderly. We consider both hypergammaglobulinaemia (polyclonal, characteristic of chronic inflammatory disorders or autoimmunity, and monoclonal, often with an associated malignant disorder) and hypogammaglobulinaemia (including immunodeficiency, immune paresis secondary to malignancy and protein loss). Where dysgammaglobulinaemia is noted in the elderly the most useful laboratory tools to help discern the pathogenesis are serum and urine electrophoresis, autoantibody investigations and measurement of liver and renal function.
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ranking = 0.87702775965226
keywords = iga, deficiency
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23/156. X-linked lymphoproliferative disease associated with hypogammaglobulinemia and growth-hormone deficiency.

    X-linked lymphoproliferative disease is a rare immunodeficiency disorder characterized by extreme vulnerability to Epstein-Barr virus, dysgammaglobulinemia, and very high incidence of lymphoma. growth-hormone deficiency has been described in rare cases to be associated with certain immunodeficiencies, such as X-linked agammaglobulinemia. We report a first case with X-linked lymphoproliferative disease associated with hypogammaglobulinemia and growth-hormone deficiency, which was confirmed by SAP gene mutation. The patient's mutation is novel. He is also the first patient with X-linked lymphoproliferative disease to be reported from saudi arabia. The patient's Btk expression and BTK gene were normal. patients with hypogammaglobulinemia and GH deficiency should be considered to have not only X-linked agammaglobulinemia, but also X-linked lymphoproliferative disease.
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keywords = deficiency
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24/156. Inflammatory and autoimmune complications of common variable immune deficiency.

    Common variable immune deficiency (CVID) is associated with autoimmune and inflammatory complications in addition to recurrent infections. The most common conditions are idiopathic thrombocytopenia purpura, autoimmune hemolytic anemia, sarcoid-like granulomatous disease and gastrointestinal inflammation. IVIG administration reduces the frequency of infections, but does not always prevent autoimmunity or inflammation. TNF antagonists and anti-CD20 immunomodulators have shown some efficacy in CVID in a few patients; further controlled studies are needed to determine the best management of these conditions in the setting of immunodeficiency.
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25/156. Crusted scabies in acquired selective iga deficiency.

    Crusted scabies, an unusual clinical variant of human scabies mite infestation, is usually reported in cases of gross debility, mental deficiency, or immunosuppression. We report here the occurrence of crusted scabies in a 40-year-old man with acquired selective iga deficiency suspected to be caused by long-term medication with phenytoin for epilepsy.
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26/156. Atypical adrenal insufficiency with failure of the pituitary feedback receptor. A case with associated diabetes mellitus and selective iga deficiency with steatorrhea.

    A 59 year old woman with insulin-dependent diabetes mellitus and chronic diarrhea was found to have mild steatorrhea, selective plasma iga deficiency and adrenal insufficiency. Significant adrenal secretion of corticosteroids resulted only after prolonged stimulation with large doses of exogenous ACTH. Plasma ACTH levels were not elevated during clinical adrenal insufficiency or after metyrapone administration but did respond normally to vasopressin and insulin-induced hypoglycemia. These studies were interpreted as showing both primary adrenal insufficiency and impaired pituitary reserve for ACTH secretion in response to the feedback stimulus. No deficiency was found in secretion of other pituitary tropic hormones. Jejunal biopsy showed a lack of IgA-containing plasma cells. With cortisone replacement, diarrhea subsided and a malabsorption pattern on a film of the small bowel was no longer seen. iga deficiency has been noted frequently with steatorrhea but rarely with diabetes and only once previously with adrenal insufficiency.
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ranking = 1.1666666666667
keywords = deficiency
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27/156. Nodular lymphoid hyperplasia of the colon associated with dysgammaglobulinemia.

    A case of dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the colon is reported. The patient had typical immunoglobulin deficiency, diarrhea, recurrent respiratory infections, giardia lamblia in the stool, and lymphoid hyperplasia of the small intestine. His barium enema showed diffuse submucosal nodules. Rectosigmoid biopsy confirmed nodular lymphoid hyperplasia. The similar findings on barium enema in this entity and in lymphosarcoma are stressed.
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28/156. Childhood eosinophilic fasciitis presenting as inflammatory polyarthritis and associated with selective iga deficiency.

    A 13 year old school boy presented with seronegative inflammatory polyarthritis after a flu-like illness. Four months later clinical features of eosinophilic fasciitis became apparent. After histological diagnosis treatment was started with prednisone 40 mg daily, with a good response. Routine investigations showed persistent selective iga deficiency.
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ranking = 1.1885138798261
keywords = iga, deficiency
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29/156. rhabdomyolysis after intramuscular iron-dextran in malabsorption.

    The case of a 59 year old white man who had chronic malabsorption and selective iga deficiency with severe iron deficiency is reported. In addition, he was deficient in vitamin e and selenium, important antioxidants which protect against lipid peroxidation. He was intolerant of oral iron and when treated with iron-dextran developed symptoms suggestive of polymyositis with evidence of rhabdomyolysis. It is suggested that free iron within iron-dextran activated free radicals, initiating lipid peroxidation and leading to polymyositis, rhabdomyolysis, and myoglobulinuria.
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ranking = 0.33333333333333
keywords = deficiency
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30/156. Changes in serum immunoglobulin patterns in adults with common variable immunodeficiency.

    Striking variations of serum immunoglobulin class and IgG subclass levels were observed in five patients with common variable immunodeficiency. They occurred mainly in untreated patients or, in those patients who received substitutive therapy, could not be merely due to replacement. They result in major changes in the immunoglobulin deficiency patterns, such as a shift from profound hypoimmunoglobulinaemia to IgA/IgG2/IgG4 deficiency or to isolated IgG2 deficiency. These findings have practical implications for the diagnosis and management of patients with primary humoral immunodeficiency.
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