Cases reported "Bronchiectasis"

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1/48. 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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ranking = 1
keywords = iga
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2/48. Immune-deficient bronchiectasis associated with X-linked lymphoproliferative disease.

    bronchiectasis may occur with various congenital and acquired immunodeficiency diseases. The association of bronchiectasis and the X-linked lymphoproliferative disease (XLP), also known as Duncan's disease is unknown. We describe the case of a 39-year-old man with XLP, the oldest surviving, who developed chronic bronchiectasis with hemoptysis and required a pneumonectomy to control his symptoms.
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ranking = 1.2942485479969
keywords = deficiency
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3/48. bronchiectasis due to ciliary aplasia in Turner's syndrome.

    A seven-year-old girl with Turner's syndrome, who suffered from recurrent respiratory system infections since birth, was investigated to determine the etiology of bronchiectasis. Electron microscopy of recurrent nasal biopsy specimens revealed ciliary aplasia. Ciliary aplasia in Turner's syndrome, has not previously been reported.
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ranking = 0.2
keywords = iga
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4/48. bronchiectasis and homozygous alpha1-antitrypsin deficiency.

    A 34-year-old woman with homozygous a1-antitrypsin deficiency suffered from progressive, generalized cystic bronchiectasis. Although bronchiectasis was reported in the original monograph on the enzyme inhibitor deficiency, it has received minimal attention since then. Alpha1-antitrypsin levels should be measured in patients with severe bronchiectasis.
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ranking = 7.7654912879816
keywords = deficiency
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5/48. 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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ranking = 6.4712427399847
keywords = deficiency
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6/48. Living-donor lobar lung transplantation for primary ciliary dyskinesia.

    A ventilator-dependent patient with primary ciliary dyskinesia underwent successful living-donor lobar lung transplantation. The case was a 24-year-old woman who had developed recurrent lower respiratory infection and became ventilator-dependent due to severe bronchiectasis. Transmission electron microscopy of the resected bronchus demonstrated inner dynein arm deficiency.
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keywords = deficiency
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7/48. Lung scan in the diagnosis and management of patent foramen ovale pulmonary embolism, paradoxical embolism.

    This case illustrates the reopening of foramen ovale in a young patient with chronic pulmonary hypertension caused by bronchiectasis and chronic pulmonary fibrosis, which resulted in a prominent right-to-left shunt and severe hypoxia. Her clinically unsuspected right-to-left shunt was discovered during ventilation-perfusion scan, which was performed for the evaluation of pulmonary embolism. She had common variable immune deficiency, a primary immunodeficiency disease in which b-lymphocytes produce few or no antibodies. Most patients with this syndrome have an intrinsic defect in their b-lymphocytes that results in reduced immunoglobulin production. In these patients, recurrent respiratory tract infections are common and may result in chronic lung disease, fibrosis, particularly bronchiectasis (20-30%) and even cor pulmonale as happened in our patient [J. Clin. Immunol. 9 (1989) 22-33.].
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ranking = 2.5884970959939
keywords = deficiency
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8/48. lung transplantation in patients with x-linked agammaglobulinemia.

    lung transplantation is an established procedure to treat patients with end-stage lung disease. The criteria for recipient selection are broadening to include patients with congenital defects of the immune system, such as X-linked hypogammaglobulinemia (XLA). We report 2 cases of successful double lung transplantation in patients with XLA. The 2 men had developed bronchiectasis and end-stage lung disease despite early institution of intravenous immunoglobulin (IVIG) replacement therapy. Before transplantation, hypogammaglobulinemia was well controlled with IVIG in both patients. After transplantation, IVIG was administered every 48 hours during the first 10 days and then tapered slowly in the following weeks until returning to an every 3 weeks schedule. One patient has been followed up for 12 months and the other for 6 months. Lung function normalized in the first case and showed a restrictive pattern in the second one. lung transplantation may be considered as a therapeutic option for patients with XLA and end-stage lung disease. Regular administration of IVIG overcomes the high risk of infections due to the severe immunodeficiency and the intensive immunosuppressive therapy.
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ranking = 1.2942485479969
keywords = deficiency
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9/48. bronchiectasis in children with lymphocytic interstitial pneumonia and acquired immune deficiency syndrome. Plain film and CT observations.

    In a review of 77 hiv positive children seen between 1981 and 1990, 32 were diagnosed as having lymphocytic interstitial pneumonitis). Four of the lip group developed bronchiectasis, a finding not previously reported. The precise factors leading to the bronchiectasis are unclear. All patients had chronically consolidated lung with volume loss. A history of recurrent bacterial superinfection was not noted in any of the cases. With more cases of hiv positive children living longer, bronchiectasis, long known to occur in primary immunologic disorders, will probably be more frequently noted.
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ranking = 5.1769941919878
keywords = deficiency
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10/48. Progressive multifocal leucoencephalopathy, sclerosing cholangitis, bronchiectasis and disseminated warts in a patient with primary combined immune deficiency.

    A 24 year old man presented with an unusual primary combined immune deficiency syndrome characterised by a profound lymphopenia of CD4 cells, selective serum IgG2 subclass deficiency, poor polysaccharide antibody responses, disseminated warts, recurrent sinopulmonary infection and bronchiectasis. The developed progressive multifocal leucoencephalopathy (PML) in association with sclerosing cholangitis. Progressive multifocal leucoencephalopathy (PML) usually occurs as an opportunistic infection in patients with secondary defects in cellular immunity.
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ranking = 7.7654912879816
keywords = deficiency
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