1/26. Influenza pneumonia in a paediatric lung transplant recipient.Although a common cause of morbidity and mortality in the general population, influenza infections are uncommon in lung transplant recipients and, to date, have only been associated with transient declines in pulmonary function and a relatively benign clinical course. This paper describes severe influenza pneumonia in a 13-year-old paediatric lung transplant recipient (5 months after double lung transplantation). Influenza pneumonia was diagnosed by direct fluorescent antibody testing and viral culture of bronchoalveolar lavage fluid. The patient required mechanical ventilation for 2 days due to respiratory failure and fatigue. Since his recovery from this pneumonia, he has developed obliterative bronchiolitis and currently awaits re-transplantation.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/26. Constrictive bronchiolitis obliterans. Characterisation of fibrogenesis and lysyl oxidase expression patterns.The process leading to irreversible fibrotic constriction of the bronchioles was studied in two cases of bronchiolitis obliterans (BO) after bone marrow transplantation. Because lysyl oxidase (LOX) is the main collagen cross-linking enzyme that might account for irreversible fibrosis, its expression was studied together with expression of extracellular matrix (ECM) proteins. Characteristic types of lesions could be distinguished on the basis of histological and immunohistological criteria. An inflammatory stage was characterised by infiltration restricted to the bronchioles by lymphocytes and dendritic cells. A fibro-inflammatory stage was characterised by the coexistence of a persistent immune cellular lesion pattern with further focal modelling of a sub-epithelial neo-synthesised connective matrix. LOX expression was observed at the tips of intra-luminal fibrotic protrusions, together with tenascin and cellular fibronectin. A fibrotic stage was characterised by dense ECM deposits spreading throughout the peri-bronchiolar connective tissue, resulting in bronchiole obliteration and final disappearance. In contrast to reversible cases of fibrosis, persistence of long-term LOX expression reflecting continuing fibrosing activity might account for the irreversible status of BO. Our two cases illustrated that, at inflammatory and fibro-inflammatory stages, BO may be stabilised by immunosuppressive treatment, while the persistence of LOX expression in the fibrotic stage might correspond to a disease that becomes irreversible and fatal.- - - - - - - - - - ranking = 0.012063856197857keywords = process (Clic here for more details about this article) |
3/26. Morphologic changes leading to bronchiolitis obliterans in a patient with delayed non-infectious lung disease after allogeneic bone marrow transplantation.A 37-year-old man developed delayed non-infectious lung disease after undergoing bone marrow transplantation (BMT) for acute myeloid leukaemia. Over a 15-month period, the progression of morphologic changes from cellular interstitial pneumonia to bronchiolitis obliterans organizing pneumonia and cicatricial bronchiolitis obliterans was documented. Pulmonary function tests, high-resolution CT, bronchoalveolar lavage, lung biopsy and extensive microbiological studies were used as diagnostic tools either at onset and during the follow-up. This represents the first reported case in which a model--supported by longitudinal biopsy results--for the evolution of histologic lesions toward bronchiolitis obliterans after BMT is suggested; therapeutic implications are discussed.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
4/26. Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with Macleod/Swyer-James syndrome and bronchiectasis.Macleod/Swyer-James syndrome is an uncommon and complex disease characterized by roentgenographic hyperlucency of one lung or lobe due to loss of the pulmonary vascular structure and to alveolar overdistension. This syndrome seems to be an acquired disease that follows viral bronchiolitis and pneumonitis in childhood. It must be differentiated from many other causes of unilateral lung "transradiancy" on the chest roentgenogram, such as those related to congenital bronchial and/or vascular abnormalities. We here describe an 11-year-old patient with Macleod/Swyer-James syndrome and bronchiectasis resulting in severe recurrent bronchopulmonary infections. Despite the severe impairment of pulmonary function, the patient underwent resection of the right lung with progressive improvement of clinical and physiologic parameters.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
5/26. bronchiolitis obliterans organizing pneumonia associated with systemic lupus erythematosus.bronchiolitis obliterans organizing pneumonia (BOOP) is a pathologic entity characterized by the formation of plugs of fibrous tissue in bronchioles and alveolar ducts. It has been described in association with several connective tissue diseases including rheumatoid arthritis, polymyositis-dermatomyositis, and mixed connective tissue disease. Well-documented reports of BOOP in patients with systemic lupus erythematosus (SLE) are limited. We report two patients with SLE who presented with subacute respiratory illnesses due to BOOP, adding further strength to the association of this entity with SLE.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
6/26. World Trade Center dyspnea: bronchiolitis obliterans with functional improvement: a case report.BACKGROUND: bronchiolitis obliterans is a severe, often progressive, lung disease characterized by cough, exertional dyspnea, and airflow obstruction. It has been ascribed to specific causes such as lung or bone marrow transplant, medications for rheumatoid disease, and most recently in association with exposure to environmental agents. METHOD: A 42-year-old, previously healthy new york city Highway Patrol officer who arrived at the World Trade Center (WTC), "ground zero," early on September 11, 2001 was evaluated. He has been followed for over 2 years with serial chest radiographs, CT scans, and pulmonary function studies. He eventually underwent an open lung biopsy. RESULTS: His dyspnea started on September 12, 2001 and progressed despite aggressive therapy with inhaled bronchodilator as well as oral and inhaled corticosteroids. At no time did he have any radiographic evidence of pulmonary disease. His forced vital capacity (FVC) decreased from 5.32 L in October 2001 to 2.86 L in January 2003. He underwent an open lung biopsy because of the persistent exertional dyspnea coupled with the loss of over 2 L of lung volume. The pathological findings were chronic bronchiolitis with focal obliterative bronchiolitis and rare non-necrotizing granuloma. Symptoms and pulmonary function improved after therapy with azithromycin was added to his treatment. DISCUSSION: This process is believed to be secondary to his massive exposure to the cloud of dust that followed the collapse of the WTC. It is our conviction that many of those present at the WTC on September 11 who have persistent dyspnea and deterioration of pulmonary function may have a similar pathologic process despite absence of abnormalities on CT of the chest. CONCLUSION: In view of the many signs and symptoms seen in first responders we feel that these findings provide important information about the pathophysiology and treatment of progressive disease resulting from this exposure.- - - - - - - - - - ranking = 0.024127712395713keywords = process (Clic here for more details about this article) |
7/26. Successful treatment of bronchiolitis obliterans in a bone marrow transplant patient with tumor necrosis factor-alpha blockade.bronchiolitis obliterans (BO) in children is a rare, inflammatory/fibrosing process involving the small airways that often results in progressive, irreversible obstructive pulmonary disease. Because treatment has focused mainly on supportive care and generally unsuccessful immunosuppression, children with BO experience significant morbidity and mortality. We report a case of biopsy-proven BO after bone marrow transplantation in a child who, after failed corticosteroid therapy, was treated with infliximab, a monoclonal antibody with binding specificity for human tumor necrosis factor-alpha. With initiation of treatment, her pulmonary symptoms and radiographic and spirometric evidence of BO resolved. Nine months later, she remains asymptomatic and shows no evidence of pulmonary decompensation. This case illustrates a successful treatment of BO with selective tumor necrosis factor-alpha blockade.- - - - - - - - - - ranking = 0.012063856197857keywords = process (Clic here for more details about this article) |
8/26. A case of bronchiolitis obliterans organizing pneumonia associated with primary sjogren's syndrome who died of superimposed diffuse alveolar damage.bronchiolitis obliterans organizing pneumonia (BOOP) developed in association with primary sjogren's syndrome in a 69-year-old female. She died of diffuse alveolar damage superimposed on BOOP in spite of corticosteroid therapy.- - - - - - - - - - ranking = 5keywords = alveolar (Clic here for more details about this article) |
9/26. Acute fibrinous and organizing pneumonia: initial presentation as a solitary nodule.A 55-year-old man complained of cough and dyspnea. His chest radiograph and CT scan revealed a solitary nodule with a diameter of 2 cm, which showed an air bronchogram and had a hazy contour. After 3 weeks, the radiologic findings changed to diffuse infiltration with small centrilobular nodules, alveolar consolidation, and bronchial wall thickening. The alveoli contained numerous fibrin balls and organizing tissue, which are typical pathologic features of acute fibrinous and organizing pneumonia (AFOP), on histologic examination. We report the initial radiologic features of AFOP and suggest that there are some radiologic similarities between this condition and cryptogenic organizing pneumonia.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
10/26. Membranous obliterative bronchitis: a proposed unifying model.Three adults with cystic fibrosis (one after lung transplantation) presented with fever, chest pain, and acute radiographic changes. The changes included a cavitary lesion of the lung, acute dense infiltrates, and lobar collapse. After failing conventional antibiotic therapy, the patients underwent flexible bronchoscopy. All had bronchial obstruction by a membrane that had completely occluded the bronchial orifice at the bifurcation of the bronchi. Therapeutic interventions ranged from continuing intravenous antibiotics, bronchoscopy-assisted perforation of the membrane by sharp instrumentation, and transthoracic needle-guided perforation of the membrane with subsequent stenting of the orifice. The patients recovered, but the posttransplant patient had recurrent membranous obstructions with multiple interventions. The cause and triggers of the process are unknown. Based on repeated observations of the evolution of the membranes, and histologic material from bronchoscopies, we propose a putative paradigm of the natural history of the process. We suggest that local stimuli generate a richly vascularized granulation polyp, which progresses in a "shutter-like" motion to form partial or completely obstructive membranes. The subsequent course depends on the vascular supply to the membrane. We also propose that similar processes may be the underlying pathologic events in some cases of lung abscess and necrotizing pneumonia.- - - - - - - - - - ranking = 0.03619156859357keywords = process (Clic here for more details about this article) |
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