| 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. ( info) |
| Constrictive bronchiolitis obliterans is rare, and the pathogenesis of the disease often remains unknown. This study reports on the case of a 38 yr-old female with constrictive bronchiolitis obliterans and paraneoplastic pemphigus associated with malignant lymphoma. The patient developed progressive obstructive lung disease. The chest radiograph showed almost normal lungs. Paraneoplastic pemphigus is a newly described syndrome in which patients have autoantibodies binding to some epithelia, including in the respiratory tract. The disease develops in association with non-Hodgkin's lymphomas or other malignant neoplasms. The case presented here suggests that constrictive bronchiolitis obliterans associated with paraneoplastic pemphigus may be one of the facets of autoimmune responses in this context. ( info) |
| BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family. ( info) |
4/161. Treatment of cachexia with recombinant growth hormone in a patient before lung transplantation: a case report. OBJECTIVES: To describe the effects of recombinant human growth hormone (rhGH) in a severely malnourished patient before lung transplantation. DESIGN: Case study. SETTING: intensive care unit. patients: A 38-yr-old severely malnourished (body mass index, 15.1 kg/m2) woman (receiving prednisone) with bronchiolitis obliterans evolving during 10 yrs presented with end-stage lung disease and required continuous noninvasive mechanical ventilation. INTERVENTIONS: Two courses of 35 days of 16 IU/day (0.42 IU/kg/day) rhGH administered subcutaneously, with an interruption of 5 wks between the two courses of rhGH. MEASUREMENTS AND MAIN RESULTS: weight gain of 14.7% and 12.8% fat-free mass, as measured by 50-kHz bioelectrical impedance analysis, during treatment during a 3.5-month period. nitrogen excretion decreased from 23.7 g/day before treatment to 8.0 g/day while receiving rhGH. Improvement of pulmonary function was also noted and allowed discharge of the patient from the hospital after the second course of rhGH. She underwent successful lung transplantation 2 months later and reached 48.8 kg of body weight 6 months later. CONCLUSIONS: rhGH treatment is a possible strategy that could be used with malnourished patients who are awaiting lung transplantation to improve the nutritional status and respiratory muscle function to prevent recurring respiratory infection and postoperative complications favored by malnutrition and possibly to decrease the length of hospital stay. ( info) |
| Respiratory syncytial virus (RSV) lower respiratory tract infections are common, yet long-term sequelae in previously healthy infants and children are rarely reported. We report a child who developed bronchiectasis and bronchiolitis obliterans following an RSV lower respiratory tract infection but in whom adenovirus was detected on stored serum. Co-infection with adenovirus is the likely cause for the bronchiectasis and bronchiolitis obliterans. ( info) |
6/161. Severe pulmonary disease in association with Crohn's disease in a 13-year-old girl. Pulmonary manifestations of Crohn's disease are infrequent in adults and even less common in children. Our literature search found only a few cases of Crohn's disease causing pulmonary manifestations in children. We report on the case of a 13-year-old girl in whom severe pulmonary disease was found four years after the onset of Crohn's disease. Open lung biopsy uncovered bronchiolitis obliterans and granulomatous lung disease. Aggressive treatment has yielded gradual improvement. This case emphasizes the importance of recognizing the association, the differential diagnosis, and treatment implications. ( info) |
| Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. autoantibodies of PNP bind to the plakin family of cytoplasmic proteins and desmogleins of cell-surface target antigens. We describe a 36-year-old female patient with PNP who had non-Hodgkin's lymphoma, and who developed bronchiolitis obliterans and died of respiratory failure. autopsy findings confirmed luminal narrowing of bronchioles by scarring, which is a histopathologic features of bronchiolitis obliterans. After the onset of respiratory failure, the reaction of autoantibodies against the plakins detected by immunoprecipitation at the onset of PNP disappeared with negative immunofluorescence within the bronchial epithelium. It is thought that autoantibodies against some of these antigens play a role in causing acute inflammation of the respiratory epithelium. In treating PNP, the possibility of the patient developing the lethal complication bronchiolitis obliterans should be kept in mind. Furthermore, prevention of the initial autoantibody-mediated injury to the respiratory epithelium should be an important treatment goal. ( info) |
8/161. 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. ( info) |
9/161. Soluble donor HLA class I and beta 2m-free heavy chain in serum of lung transplant recipients: steady-state levels and increases in patients with recurrent CMV infection, acute rejection episodes, and poor outcome. We determined the concentration of donor sHLA/beta(2)m and total beta(2)m-free heavy chain (HC) in the serum of lung transplant recipients with ELISA assays. While we were unable to detect specific donor beta(2)m-free HCs due to a lack of available antibodies, we could determine if events that led to an increase in the release of beta(2)m-free HC also led to an increase in the release of donor sHLA/beta(2)m, particularly the 36 kDa, proteolytically cleaved form. We found that lung transplants constituitively release donor sHLA/beta(2)m at ng/ml levels. The levels (both of donor sHLA/beta(2)m and total beta(2)m-free HC) were significantly increased in CMV-sero-negative recipients (but not in CMV-sero-positive recipients) at the onset of post-transplant CMV disease. Acute rejection episodes were also associated with an increased release of donor sHLA/beta(2)m, but not of beta(2)m-free HC. However, in patients with particularly poor outcome (i.e., graft loss within 1 year) there was a significant release of beta(2)m-free HC. Analysis of one such patient showed a predominance of 36 kDa forms of donor-sHLA/beta(2)m. Our data are consistent with the hypothesis that the metalloproteinase that cleaves beta(2)m-free HC is active during uncontrolled CMV infection and acute rejection. However, recall responses to CMV and controlled immune responses to donor may result in little or no activation of sHLA class I release. ( info) |
10/161. Drug-induced methemoglobinemia during thoracoscopic lung biopsy. Acquired methemoglobinemia occurs rarely in clinical practice. We present a case of a 57-year-old man who developed severe drug-induced methemoglobinemia after exposure to benzocaine spray and lidocaine jelly during intubation for an elective thoracoscopic lung biopsy. Information regarding the classifications, pathophysiology, diagnosis, and treatment of this entity is reviewed. ( info) |