Cases reported "Bronchiectasis"

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1/8. Hilar adenopathy in allergic bronchopulmonary aspergillosis.

    BACKGROUND: A 20-year-old male student developed allergic bronchopulmonary aspergillosis (ABPA). Computed tomography (CT) of the thorax done to detect central bronchiectasis (CB) for confirmation of diagnosis revealed, in addition, right hilar lymphadenopathy. Hilar adenopathy is thought to be rare in ABPA and has been documented only once before. Because of the finding of hilar adenopathy, the earlier reported patient had to undergo an invasive surgical procedure. OBJECTIVE: To report a case of true hilar adenopathy in ABPA. methods: This is a single case report. Contrast enhanced CT of the thorax was done. serum precipitating antibodies against aspergillus fumigatus were tested using gel diffusion technique, and intradermal testing with antigens of Aspergillus species was performed. Specific IgG antibodies against A. fumigatus and total IgE levels were measured by ELISA. RESULTS: A review of serial chest radiographs over a period of 3 years demonstrated transient pulmonary infiltrates and right hilar prominence. Computed tomography of the thorax revealed right hilar lymphadenopathy along with bilateral central bronchiectasis and patchy infiltrates. Strong bands of precipitins were detected against A. fumigatus. Intradermal testing with antigens of Aspergillus species elicited strong type I (immediate) and type III (Arthus-type) hypersensitivity reactions to A. fumigatus and A. niger. Specific IgG antibodies against A. fumigatus was positive and total IgE level was significantly elevated. Peripheral blood eosinophilia was also detected. CONCLUSIONS: Although extremely rare, ABPA should be considered in the differential diagnosis of hilar adenopathy.
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2/8. common variable immunodeficiency with mosaic trisomy 8: report of one case.

    This case study reported a 17-year-old female of common variable immunodeficiency (CVID) associated with bronchiectasis, pernicious anemia and mosaic trisomy 8. Clinically this patient presented with recurrent sinopulmonary infections, intractable diarrhea, macrocytic anemia, and primary amenorrhea. Immunological tests showed pan-hypogammaglobulinemia and a decrease of peripheral blood B cells (4%) and CD4 cells (25%). Lymphoproliferative responses to mitogen (PHA) and specific antigen (BCG) were profoundly impaired in the patient in comparison to those in control. Production of interleukin 4 (IL-4) and gamma interferon (IFN-gamma) in the in vitro lymphoproliferation was also profoundly depressed. Pernicious anemia demonstrated by larger MCV (112.9 fl) and hyper-segmental granulocytes on peripheral blood smear responded to parental administration of vitamin B12. Interestingly, she had a mosaic trisomy 8 in peripheral blood mononuclear cells but normal 46XX karyotype in the bone marrow cells. To our knowledge, this is the first case of CVID associated with mosaic trisomy 8 reported in the literature. As the case exemplifies, CVID should be considered when the physicians evaluate the patient presenting with recurrent sinopulmonary infections, diarrhea, malnutrition, and pernicious anemia. It requires further study to explore whether the genes in the chromosome 8 are linked to CVID.
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3/8. Valaciclovir for chronic hepatitis b virus infection after lung transplantation.

    We report on a chronic asymptomatic hepatitis B surface antigen (HBsAg) carrier who developed an increase in aminotransferase and HBsAg levels 1 year after lung transplantation. During treatment for cutaneous herpes simplex virus (HSV) infection with oral valaciclovir there was a marked decrease in replicating hepatitis b virus (HBV)-dna and aminotransferase levels, which was sustained for 9 months by continuing low-dose valaciclovir. A second rise in aminotransferase levels again responded to a valaciclovir dose increase and the HBV-dna levels declined further. Although we cannot exclude a spontaneous variation of the serologic parameters, our observation suggests that valaciclovir may represent a valuable therapeutic option in the treatment of chronic hepatitis B after lung transplantation.
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4/8. Progressive sinobronchiectasis associated with the "bare lymphocyte syndrome" in an adult.

    A Japanese adult lacking the expression of HLA-A, HLA-B, and hla-c antigens on the lymphocyte (the "bare lymphocyte syndrome") had recurrent sinopulmonary infections and diffusely progressive bronchiectasis. Class I antigen and beta 2-microglobulin on the lymphocytes of the patient, his siblings, his father, and healthy subjects were assayed by using monoclonal antibodies against these surface markers. Class I antigen and beta 2-microglobulin were not found on the lymphocytes of the patient and the Daudi cells. In addition, lymphocytes of his siblings showed low fluorescence intensity. The HLA molecule on the lymphocyte plays an important role at the stage of recognition of immunogenic antigens. These results might suggest a correlation between sinobronchiectasis and the deficiency of HLA molecules, and the importance of the HLA system in the immune defense mechanism.
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5/8. Improvement of cellular immunity and IgA production in immunodeficient children after treatment with synthetic serum thymic factor (FTS).

    Three children with IgA and IgE deficiency and T-cell defects (two related patients with ataxia telangiectasia and one with common variable immune deficiency) were treated with synthetic serum thymic factor (FTS) intravenously. A reduction in frequency and severity of infection was noted concomitantly with improvement in cell-mediated-immunity tests. serum IgA, which was absent in two patients, appeared within 4 weeks of treatment and increased significantly in the third patient. Specific antibodies against vaccination antigens appeared for the first time or increased to titres higher than ever before. In two patients, transient interruption of FTS administration was followed by a regression of the immunological improvement, but this disappeared after the treatment was started again.
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6/8. Increased suppressor cell activity in a patient with Mycobacterium avium-intracellulare pulmonary disease and hypogammaglobulinemia.

    Immunologic studies were done in a patient with common variable hypogammaglobulinemia, bronchiectasis and M. avium intracellulare pulmonary infection. Adherent cells from the patient were found to suppress the proliferative response of normal control cells to PPD antigen. The suppression seemed to be mediated by prostaglandin as it was reversed by indomethacin. Increased suppressor cell activity may play an important role in the pathogenesis of mycobacterium avium-intracellulare infection by permitting the transformation of a status of colonization by the organism in pre-existing pulmonary conditions into an invasive, progressive disease.
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7/8. association of HLA class I antigen deficiency related to a TAP2 gene mutation with familial bronchiectasis.

    Two siblings with pansinusitis, nasal polyps, and bronchiectasis were found to have histocompatibility lymphocyte antigen (HLA) class I antigen deficiency ("bare lymphocyte syndrome") and dysfunction of natural killer cells. Reduced class I cell surface expression resulted from a single mutation in the TAP2 gene, which is located in the class II region of the major histocompatibility complex and encodes subunit 2 of the class I peptide transporter. The defect was transmitted in an autosomal recessive manner. This deficiency did not lead to severe viral infections but was apparently associated with susceptibility to bacterial infections of the respiratory mucosae. We suggest that class I HLA typing should be systematically performed in children with unexplained bronchiectasis.
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8/8. vasculitis and bronchiectasis in a patient with antibodies to bactericidal/permeability-increasing protein and alpha1-antitrypsin deficiency.

    A patient with alpha1-antitrypsin deficiency is reported herein; this subject developed aggressive bronchial disease and recurrent cutaneous vasculitis after pulmonary infection with pseudomonas aeruginosa. autoantibodies to neutrophil cytoplasmic antigens were detected, which produced granular cytoplasmic staining by indirect immunofluorescence with specificity for a newly characterized antigen: bactericidal/permeability-increasing protein (BPI). The bronchial disease and vasculitis improved, and the IgA anti-BPI titer fell after antipseudomonal treatment. This raises the possibility that anti-BPI antibodies contributed to both the bronchial disease and vasculitis.
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