Cases reported "Bronchiolitis Obliterans"

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1/9. Bilateral pneumothoraces with multiple bullae in a patient with asymptomatic bronchiolitis obliterans 10 years after bone marrow transplantation.

    A 16-year-old boy developed bronchiolitis obliterans (BO) 10 years after BMT for myelodysplastic syndrome. Although the patient complained of almost no dyspnea on exertion, he had mild hypercapnea with a markedly reduced forced expiratory volume of 0.32 l. Chest x-rays showed occasional bilateral minimal pneumothoraces, which is in accordance with the existence of multiple small bullae found on the pleural surface at video-assisted thoracic surgery. Histologic examination of the biopsied lung revealed BO. This case indicates that BO in adolescence following BMT and possible chronic GVHD may be masked because of lung immaturity at BMT, and BO after BMT may be associated with multiple pleural bullae.
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keywords = forced expiratory volume, expiratory volume
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2/9. bronchiolitis obliterans organizing pneumonia and scleroderma.

    Pulmonary involvement in scleroderma is characterized by interstitial fibrosis and pulmonary hypertension. Although bronchiolitis obliterans organizing pneumonia (BOOP) may be associated with a variety of connective tissue diseases and their treatment, there are only rare reports of bronchiolitis associated with scleroderma. We describe 2 patients with scleroderma and rapidly evolving pulmonary infiltrates, which upon biopsy showed histologic findings of BOOP. Each patient had severe restrictive lung disease and markedly diminished diffusion capacity. Treatment with high dose prednisone showed a good response in one patient. The rapid course of pulmonary findings in these patients differs from the usual course of pulmonary fibrosis in scleroderma. Although BOOP is a rare finding in scleroderma, our findings suggest that rapid pulmonary decompensation or atypical findings for pulmonary fibrosis should prompt consideration for an open lung biopsy. Finding a potentially steroid responsive disorder in an otherwise steroid unresponsive disorder has important clinical implications.
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ranking = 0.0016823438736137
keywords = capacity
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3/9. Living-donor lobar lung transplantation for bronchiolitis obliterans after bone marrow transplantation.

    We report a successful case of living-donor lobar lung transplantation (LDLLT) for severe bronchiolitis obliterans (BO) after bone marrow transplantation (BMT). The patient is a 29-year-old woman who underwent BMT because of aplastic anemia in 1995. In 1996, BO developed in the patient because of chronic graft-versus-host disease. In May 2000, a LDLLT was performed. Pulmonary function tests showed improvement of both vital capacity and forced expiratory volume in 1 second. At present, 91 months after BMT and 38 months after lung transplantation, the patient is in good health. LDLLT may offer a therapeutic option for the treatment of BO after BMT.
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ranking = 1.3646365358261
keywords = forced expiratory volume, vital capacity, expiratory volume, capacity
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4/9. 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.
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ranking = 0.36463653582612
keywords = vital capacity, capacity
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5/9. Early results of single lung transplantation in patients with end-stage pulmonary fibrosis.

    Three patients underwent single left lung transplantation for end-stage pulmonary fibrosis between June and November 1987. Preoperatively all were housebound, receiving continuous, supplemental oxygen, and their pulmonary function had deteriorated despite corticosteroid and cyclophosphamide therapy. Pulmonary preservation was by means of pulmonary arterial perfusion with modified Euro-Collins solution, 60 ml/kg, at 4 degrees C with adjunctive iloprost (synthetic prostacyclin) infusion. The heart from each donor was used successfully for transplantation. Good early graft function enabled extubation 11, 46, and 96 hours after transplantation. An omental wrap was used around the bronchial anastomosis, and bronchial healing was satisfactory in all. All patients had episodes of pulmonary rejection diagnosed by a combination of symptoms, chest x-ray infiltrates, the exclusion of pneumonitis by bronchoalveolar lavage, and prompt response to "pulse" steroid therapy. Two of the three patients had three episodes of opportunistic pulmonary infections: herpes simplex pneumonitis, pneumocystis carinii infection, and aspergillus pneumonitis. The three patients were discharged from the hospital after 5, 6, and 7 1/2 weeks, respectively. The first and third patients remain alive and well, living essentially normal lives 24 and 19 months after transplantation with no evidence of arterial desaturation on exercise testing while breathing room air. The second patient had symptoms of deteriorating lung function with a progressive decline in forced expiratory volume in 1 second, vital capacity, and diffusion capacity despite repeated "pulse" therapy with combinations of methylprednisolone, antithymocyte globulin, and OKT3 (Ortho Diagnostic Systems Inc., Raritan, N.J.). An open lung biopsy specimen showed obliterative bronchiolitis, and this patient underwent orthotopic lung retransplantation, on the right side. Despite excellent early graft function and early extubation, he died of uncontrolled rejection and general debility after 3 weeks. This early experience in our center with two of three patients surviving 19 to 24 months, respectively, confirms the restoration of good pulmonary function and near normal life-style in patients with end-stage pulmonary fibrosis after single lung transplantation, as first reported by the Toronto Lung Transplant Group. We have used an alternative method of lung preservation (cold crystalloid pulmonary perfusion as opposed to topical cooling, used by the Toronto group), which provided excellent pulmonary preservation up to and beyond 4 hours' storage.(ABSTRACT TRUNCATED AT 400 WORDS)
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ranking = 1.3663188796997
keywords = forced expiratory volume, vital capacity, expiratory volume, capacity
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6/9. charcoal lung. bronchiolitis obliterans after aspiration of activated charcoal.

    Activated charcoal usually provides effective and safe treatment for drug overdose. We describe a patient who developed bronchiolitis obliterans and respiratory failure following aspiration of activated charcoal. This patient had a markedly reduced vital capacity with roentgenographic evidence of airtrapping. Chest roentgenograms did not demonstrate the large amount of charcoal identified at postmortem examination.
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ranking = 0.36463653582612
keywords = vital capacity, capacity
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7/9. bronchiolitis obliterans organizing pneumonia. diagnosis by transbronchial biopsy.

    Transbronchial biopsy (TBB) has been considered to be inadequate for the diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP). We describe herein two patients with interstitial pulmonary disease in whom the diagnosis of BOOP was achieved by TBB. The two patients presented with progressive dyspnea, cough, tachypnea, and fine end-inspiratory crackles. The radiologic findings disclosed patchy alveolar infiltrates. Pulmonary function tests showed a restrictive pattern and decreased diffusing capacity. The pathologic findings disclosed bronchioles, alveolar ducts, and alveoli infiltrated with mononuclear cells. The lumina were obliterated with fibroblasts and loose granulation tissue. Corticosteroid treatment resulted in significant improvement. Transbronchial biopsy should be considered as a useful diagnostic tool for BOOP.
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ranking = 0.0016823438736137
keywords = capacity
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8/9. A case report of a double-blind, randomized trial of inhaled steroids in a patient with lung transplant bronchiolitis obliterans.

    Lung transplant bronchiolitis obliterans syndrome (BOS) is the most significant long-term cause of morbidity and mortality after lung transplantation. Although augmented immunosuppression is used by most centers, reported on treatment to reverse BOS are largely anecdotal. We performed a double-blind, randomized, controlled trial (RCT) with ten treatment pairs of 2 weeks duration each comparing inhaled fluticasone propionate (2 x 1,000 micrograms/day) with placebo in a patient with BOS grade 2 who previously showed an improvement in lung function after inhaled steroids. The Baseline dyspnea Index and the Modified Medical research Council dyspnea Scale showed a significant improvement during fluticasone treatment compared with the placebo period (2.7 /- 0.2 vs. 2.0 /- 0.3; p = 0.043; and 1.7 /- 0.2 vs. 2.4 /- 0.2; p = 0.043). The patient correctly identified fluticasone and placebo, respectively, in eight of ten trial pairs (p = 0.016). The values of forced expiratory volume in 1 s were significantly higher during the fluticasone period (1,207 /- 10 ml; 95% confidence interval, CI, 1,187-1,227 ml) compared to the placebo period (1,150 /- 6 ml; 95% CI 1,138-1,162 ml; p = 0.0012). In conclusion, this n-of-1 RCT suggests the efficacy of high-dose inhaled fluticasone in our patient with lung transplant BOS. We propose to conduct a multicenter RCT of high-dose inhaled steroids. Until further data are available, this treatment modality should be offered to patients with lung transplant BOS.
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keywords = forced expiratory volume, expiratory volume
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9/9. Lung retransplantation 3 yrs after heart-lung transplantation.

    The main long-term complication after lung transplantation is chronic rejection in terms of obliterative bronchiolitis; when therapy does not arrest the progression of the airflow obstruction, lung retransplantation seems to be. at present, the only strategy in the management of chronic rejection. We report the 12 month follow-up of a single lung retransplantation in a 21 yr old female who had received a heart-lung transplantation 35 months previously for Eisenmenger syndrome. The patient had excellent first allograft function and quality of life for 26 months, then progressively deteriorated due to the occurrence of obliterative bronchiolitis, and further worsened in the following 9 months. At that time, she underwent left lung retransplantation, based on her negative history of infection, low rate of acute rejection, ambulatory status, and young age. She is now doing well at 12 months after retransplantation and her forced expiratory volume in one second is still improving, p thus justifying both retransplantation and hopeful expectation.
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keywords = forced expiratory volume, expiratory volume
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