Cases reported "Dysgammaglobulinemia"

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1/156. severe combined immunodeficiency with B lymphocytes: in vitro correction of defective immunoglobulin production by addition of normal T lymphocytes.

    A 6 1/2-month-old male with severe combined immunodeficiency (SCID) had a low percentage and number of T cells (11%; 241/mm3) and a high percentage and number of B cells (52%; 1187/mm3) and null cells (37%; 868/mm3). in vitro studies were performed to determine if this child's primary defect involved differentiation of both T and B lymphocytes or if failure of B lymphocytes to differentiate into immunoglobulin producing cells was secondary to T lymphocyte abnormalities. Immunoglobulin production by lymphocytes in response to polyclonal mitogens (pokeweed mitogen and foetal calf serum) was measured by pulse-labelling cells with 3H-leucine and then precipitating cytoplasmic and secreted immunoglobulins with polyvalent anti-human immunoglobulin and S. aureus (Cowan strain I) protein A. The patient's lymphocytes did not synthesize immunoglobulins in vitro in response to mitogens. They did not suppress synthesis of immunoglobulins by normal lymphocytes. However, addition of normal purified T cells, which themselves did not synthesize immunoglobulins, enabled the patient's B lymphocytes to become immunoglobulin synthesizing and secreting cells. Gamma, mu, and light chains were secreted. This suggests that the primary abnormality was in the T-cell axis at the level of lymphoid stem cells or prothymocytes and that failure of B lymphocytes to become immunoglobulin-producing cells was secondary to this defect.
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2/156. 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.
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3/156. secretory component deficiency. A disorder of the IgA immune system.

    We studied a 15-year-old boy with chronic intestinal candidiasis who had normal serum IgA levels without IgA in his secretions. There was an elevated number of peripheral blood lymphocytes bearing surface IgA. In addition, the lymphocytes cultured in vitro with pokeweed mitogen produced IgA as well as other immunoglubulins. Despite this evidence of normal IgA synthetic capacity, the patient had greatly diminished levels of IgA in the saliva and jejunal fluid, and, as estimated by 14C-L-leucine incorporation, could not synthesize IgA locally at intestinal-mucosal sites. Finally, the patient had no detectable free secretory component in saliva or jejunal fluid in contrast to normal persons and to patients with iga deficiency. The basis of this disorder is probably a defect in the homing of IgA precursor cells to secretory sites or in the selective proliferation/differentiation of IgA cells at such sites.
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4/156. Multiple recurrent hordeola associated with selective IgM deficiency.

    An external hordeolum is an acute, suppurative inflammation of the glands of Zeis and sweat glands or hair follicles most commonly caused by staphylococci, usually in the setting of a chronic blepharitis.(1) We report a case of a boy with unilateral multiple recurrent hordeola in association with selective IgM deficiency.
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5/156. An unusual presentation of immunodeficiency with hyper-IgM.

    Hyper-IgM syndrome is a rare immunodeficiency disease characterized by markedly decreased serum IgG, IgA, and IgE levels but normal or elevated IgM levels. The most common clinical signs are infections, cirrhosis, arthritis, malignancies, and mucosal ulcers. Approximately two-thirds of patients have chronic neutropenia associated with oral and perirectal ulcers. We report a 14-month-old girl with hyper-IgM syndrome who has recurrent cutaneous ulcers restricted to the diaper area.
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6/156. Effects of sulfasalazine treatment on serum immunoglobulin levels in children with juvenile chronic arthritis.

    This article describes the effects of sulfasalazine (SSZ) treatment on serum immunoglobulin (Ig) levels in 6 children with oligoarticular- or polyarticular onset juvenile chronic arthritis (JCA). None of the children who developed dysimmunoglobulinemia during treatment showed clinical symptoms of this adverse event, in particular none developed severe infections. All patients regained normal immunoglobulin levels after discontinuing SSZ treatment. One patient with a partial iga deficiency at the start of SSZ treatment showed a slow increase in the IgA level during treatment. During follow-up (4-6 years), one patient spontaneously developed a dysimmunoglobulinemia and one patient developed diabetes mellitus. Based on these case reports and review of the literature we advocate monitoring of serum immunoglobulin levels while on SSZ treatment.
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7/156. X-linked hyper IgM syndrome: a report of the first case in thailand with a confirmed mutation of cd40 ligand gene.

    X-linked hyper IgM (XHIM) syndrome is a rare congenital immunodeficiency disease caused by failure of B cell to isotype switch from IgM to other classes of immunoglobulins in response to infections. Recently, a molecular cloning of the gene responsible for the syndrome, the CD40L gene has been accomplished and the gene was successfully mapped to the long arm of x chromosome at the position Xq26. We, herein, report the first case of molecular proven XHIM in a Thai boy with a classic presentation and with a confirmed mutation of the CD40L gene. Case Report: A.S. was a 1 year 7 month old boy referred from Buriram Provincial Hospital for a work up and treatment for his recurrent infections consisted of chronic respiratory tract infections with otitis media (since 6 months of age), chronic diarrhea (since 9 months of age) and malnutrition (marasmus) secondary to his longstanding illnesses. He was a product of a consanguineous marriage but without history of similar illness observed in his pedigree. Abnormal laboratory works up included IgG of 300 mg/dl, IgA 10 mg/dl, IgM 1,635 mg/dl, positive stool examinations for cryptosporidium, chronic colitis on radiographic gastrointestinal follow through study, a positive acid fast bacillus (AFB) stain of gastric aspirate and multiple positive bacterial cultures from various body sources. His anti-hiv serology was negative. His hospital course was significant for several bouts of infections of gastrointestinal, respiratory, and genitourinary systems. His treatment consisted of multiple courses of antibiotics, antituberculous drugs and IVIG administrations. His hospital course was complicated with feeding problem from an esophageal stricture requiring several esophageal dilatations. The analysis of CD40L gene revealed a point mutation of exon 5 (A619T) of the CD40L gene resulting in a stop codon confirming that indeed he had XHIM. He died with pseudomonas septicemia during the waiting period for a bone marrow transplantation from a cord-blood stem cell.
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keywords = iga, deficiency
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8/156. The progressive appearance of multiple urinary Bence-Jones proteins and serum paraproteins in a child with immune deficiency.

    Multiple urinary Bence-Jones proteins and serum paraproteins were found in a child with type I dysgammaglobulinaemia (Seligmann et al., 1968). These showed a continually evolving pattern over a period of 4 months in relation to systemic infections and with no evidence of underlying malignancy.
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9/156. Deficiency of secretory Ig-A and intestinal malabsorption.

    A patient under treatment with hemodialysis suffered increasing clinical and laboratory evidence of intestinal malabsorption. Jejunal aspirates revealed heavy bacterial and mycotic flora within the proximal jejunum. Secretory Ig-A and secretory component were present only in trace amounts. The deficiency of the generally ubiquitous secretory component is particularaly striking. Oral administration of 20-30 ml. colostrum daily reversed not only the clinical evidence but also laboratory findings of intestinal malabsorption.
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10/156. Thymus transplantation in patients with thymic hypoplasia and abnormal immunoglobulin synthesis.

    Fetal thymus transplantation was performed in three patients with thymic hypoplasia with abnormal immunoglobulin synthesis, one patient with ataxia telangiectasia, and one patient with immunodeficiency with eczema and thrombocytopenia. All patients received transfer factor before transplantation of a fetal thymus i.p. Reconstitution of cell-mediated immunity occurred in three of five patients. Two of the three patients with reconstitution of cell-mediated immunity also had evidence of improved antibody-mediated immunity. Reconstitution of cell-mediated immunity was characterized as occurring rapidly and being of varying duration, and was unassociated with HL-A chimerism. Successful reconstitution of immunity in these patients may have been related to several factors, including the use of fetal thymus less than 6 hr after abortion, i.p. transplantation, and a synergistic effect of transfer factor.
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