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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The prevalence of bronchiectasis decreased due to the effective use of vaccines and advances of antibiotic therapy after 1970. However, it remains an important long-term morbidity of lower respiratory tract infection in developing country. To evaluate the clinical features of bronchiectasis in a tertiary hospital, we collected 21 patients with a diagnosis of bronchiectasis in Taipei Veterans General Hospital from May, 1984 to Dec, 2001 in pediatric outpatient with the admission of age below 18 years old. The diagnosis was based on the history of recurrent cough with fetid sputum, hemoptysis, or recurrent lobar pneumonia for months at least and radiological findings of lobar infiltration, tram-track like patterns, bronchiolar dilatation or honeycomb patterns. The diagnostic examinations included chest plain radiography, bronchography and chest computed tomography (CT) scans. Respiratory tract infections were the commonest cause predisposing to bronchiectasis in our study. Tuberculosis is not rare in this study. In recent years, immunodeficiency disorders have been recognized. Most patients suffered from recurrent cough and fetid sputum for years before diagnosis was established. Hemoptysis was the second common symptom in our study. The plain chest radiograph of bronchiectasis revealed dilatation of bronchial trees with honeycomb pattern or infiltration only. In recent years, chest CT became the most accurate and being noninvasive diagnostic tool. The initial treatment was primarily medical conservative therapy. Only five patients in our cases underwent pulmonary resection due to persistent hemoptysis, recurrent bacterial pneumonia or pulmonary parenchyma destruction. Most patients still suffered from recurrent pneumonia or occasional exacerbation in the long-term follow-up. In conclusion, bronchiectasis is not uncommon in pediatric population in northern Taiwan. The history of recurrent cough with fetid sputum, hemoptysis, or recurrent pneumonia were the most important clues to early diagnosis of this disease. Early diagnosis and appropriate treatment are effective in order to prevent lung abscess, empyema and pneumothorax, bronchopleural fistula, hemoptysis or cor pulmonale.
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PMID:Clinical spectrum of bronchiectasis in children. 1260 83

A 64-year-old man presented a nodular shadow in the right lower lobe on a chest computed tomography (CT) scan. The nodule had grown remarkably in 3 years and was suspected to be a lung cancer. An open lung biopsy showed a solid lesion adjacent to lung abscess, and histopathological examination of a biopsy specimen revealed follicular bronchiolitis. Follicular bronchiolitis is a rare condition and is reported to occur mainly in association with connective tissue disorders or immunodeficiency syndromes. In such conditions, it generally exhibits bilateral, diffuse, small nodular lesions or ground-glass opacities. The present case was not associated with such underlying diseases and was thought to have occurred in association with lung abscess. Few cases of follicular bronchiolitis caused by non-specific airway infection have been reported in Japan.
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PMID:[Solid follicular bronchiolitis]. 2106 43

Pulmonary disease, ranging from infectious pneumonia, lung abscess, and empyema to structural lung diseases to malignancy, significantly increase morbidity and mortality in primary immune deficiency. Treatment with supplemental immunoglobulin (intravenous or subcutaneous) and antimicrobials is beneficial in reducing infections but are largely ineffective in preventing noninfectious complications, including interstitial lung disease, malignancy, and autoimmune disease. A low threshold for suspecting pulmonary complications is necessary for the early diagnosis of pulmonary involvement in primary immunodeficiency disorders, before irreversible damage is done, to improve patient outcomes.
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PMID:Pulmonary Manifestations of Primary Immunodeficiency Disorders. 2645 17


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