Bronchiolitis Obliterans ; diagnosis ; etiology ; therapy ; Child ; Humans

OBJECTIVEBronchiolitis obliterans (BO) is a chronic airflow obstruction syndrome associated with inflammatory lesions of the small airways. The etiology, pathogenesis, effective treatment and prognosis of this disorder remain uncertain by now. There has been no large scale clinical research on BO in China. This study aimed to analyze the clinical characteristics of BO in children.

METHODSClinical analysis was done on data of 42 patients (31 boys and 11 girls, aged from 7 months to 12 years and 2 months) with bronchiolitis obliterans diagnosed and treated in Beijing Children's Hospital from April 2001 to August 2007. The diagnostic criteria included typical clinical manifestations, specific pulmonary CT imaging and lung function tests.

RESULTSAll the patients presented with persistent cough and wheezing. Crackles and wheezing were heard in most cases. Thirty-two (76.2%) cases were post-infectious BO, among whom 8 (25%) were supposed to be adenovirus pneumonia, 7 (21.9%) measles pneumonia and 2 (6.2%) respiratory syncytial virus infection. In 4 (9.5%) cases BO occurred after Steven-Johnson syndrome, and 1 (2.4%) after bone marrow transplantation. The constrictive obstruction in small airway occurred in 35 cases (89.7%), while mixed pattern in 4 (10.3%). Pulmonary CT revealed mosaic perfusion in 34 cases (81.0%), bronchiectasis in 14 cases (33.3%), bronchial wall thickening in 14 cases (33.3%), atelectasis in 4 cases (9.5%) and Swyer-James syndrome in 2 cases (4.8%). All the cases received oral corticosteroid and low doses of erythromycin or azithromycin, with corticosteroid and bronchodilator inhalation or oral montelukast. Follow up time was from 1 month to 5 years. Besides 2 cases in whom the disease was ameliorated in clinical presentation, pulmonary imaging and function, the rest experienced deterioration and one died.

CONCLUSIONIn most of the patients BO occurred following infections. The specific clinical presentations, imaging and pulmonary function are sufficiently informative for diagnosis. Most of the cases enrolled in this study had a poor outcome.

Bronchiolitis Obliterans ; diagnosis ; etiology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Prognosis

OBJECTIVETo analyze the clinical characteristics, image findings, laboratory examination, the therapeutic methods and clinical outcomes of bronchiolitis obliterans (BO) in pediatric patients.

METHODTwenty-six pediatric patients with BO were reported. All data were collected from cases who were hospitalized in the Department of Pediatrics, First Affiliated Hospital of Guangzhou Medical College from June 1(st), 2009 to the April 30(th), 2011, and infectious agents, clinical manifestations, risk factors, changes in imageology, laboratory examination, therapeutic methods and treatment responses were analyzed.

RESULTThe ranges of age at onset was 4.5 months-8 years in 26 cases (18 boys and 8 girls). The course of disease was (6.2 ± 3.5) months. The period of followed-up ranged from 2 to 24 months. The common clinical characteristics were persistent wheezing of different severity (26 cases, 100%), cough (24 cases, 92%), intolerance to exercise (22 cases, 85%), short of breath (21 cases, 81%), retraction (20 cases, 77%), wheezy phlegm (16 cases, 62%), keeping with crackles (10 cases, 38%), cyanosis around the mouth (3 cases, 12%) and no clubbed fingers (toes). In 18 cases the etiology was detected, mycoplasma (11 cases, 42%), respiratory syncytial virus (4 cases, 15%), parainfluenza virus (2 cases, 8%), influenza virus A (2 cases, 8%) and influenza virus B (2 cases, 8%), human bocavirus (HBoV) (1 case, 4%). There were 8 cases (31%) with combined infection. Chest X-ray in 10 cases indicated changes suggestive of bronchopneumonia (38%), in only 1 case there was an image of interstitial pneumonia disease (4%). All the patients were diagnosed by high-resolution computerized tomography (HRCT). All cases were demonstrated to have air retention, poor blood perfusion in lung, just like "Westemark sign" with HRCT. In 19 cases antineutrophil cytoplasmic antibody (ANCA) was determined and 10 patients (53%) were positive for P-ANCA, and 8 cases (42%) were positive for C-ANCA. All patients received oral corticosteroid and low doses azithromycin. In 13 cases (50%) the treatment effectively reduced the severity of disease and the frequency of cough and wheezing. The average number of days for symptom improvement was (7.1 ± 4.8) days.

CONCLUSIONRespiratory infection plays an important role in BO in children. The chronic and persistent wheezing, cough, intolerance to exercises, short breath, retraction were the main clinical manifestations. But these symptoms are non-specific. Chest X-ray can not provide enough information for diagnosis. Classical "Westemark sign" with HRCT is an important sign. ANCA with a high positive rate (approximately 50%) suppose immuno-lesion in BO. Oral corticosteroid and methotrexate may relieve clinical symptoms.

Bronchiolitis Obliterans ; diagnosis ; etiology ; therapy ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Prognosis ; Treatment Outcome

Accidents, Occupational ; Adult ; Ammonia ; poisoning ; Bronchiolitis Obliterans ; etiology ; surgery ; Female ; Humans ; Lung Transplantation

Adult ; Aged ; Asthma ; epidemiology ; etiology ; Bronchiolitis Obliterans ; epidemiology ; etiology ; China ; epidemiology ; Female ; Humans ; Male ; Middle Aged ; Prevalence ; Smoking ; adverse effects

OBJECTIVETo explore the feasibility and efficiency of lung transplantation in the treatment of bronchiolitis obliterans (BO) after allogeneic bone marrow transplantation (allo-BMT).

METHODSWe reported one case of bilateral lung transplantation for BO after allo-BMT and reviewed the related literatures.

RESULTSA 23 year-old man diagnosed as BO after allo-BMT underwent a sequential bilateral lung transplantation through bilateral anterolateral thoracotomy without sternal division. The patient suffered from acute rejection on post-operation day (POD) 2, and cured by mechanical ventilation, large dose of methylprednisolone and gamma globulin. The patient was transferred out of the intensive care unit on POD 14 and discharged from the hospital on POD 43. Chest CT scans and pulmonary function tests showed good performance in 3 and 6 months follow-up period.

CONCLUSIONBO is one of the late common non-infectious pulmonary complication after allo-BMT. For patients who have no response to medication, lung transplantation is the only efficient treatment choice so far, which can prolong survival and improve the quality of life. However, limited by small samples, optimal surgery time and appropriate care of postoperative complications still need accumulation of experience by multicenter and large samples studies.

Bone Marrow Transplantation ; adverse effects ; Bronchiolitis Obliterans ; etiology ; surgery ; Humans ; Lung Transplantation ; methods ; Male ; Transplantation, Homologous ; Young Adult

Objective: To investigate the incidence, risk factors and survival of bronchiolitis obliterans syndrome (BOS) in patients who had undergone haplo-hematopoietic stem cell transplantation (haplo-HSCT) . Methods: This study retrospectively analyzed clinical data of 444 consecutive patients who underwent haplo-HSCT and survived at least 100 days after transplantation in the First Affiliated Hospital of Soochow University between January 2013 and December 2015. Results: By the end of follow-up on January 1, 2018, 25 patients (5.63%) had BOS (BOS group) . The median onset time of BOS was 448 (165-845) d post transplantation, the 1-year, 2-year and 3-year cumulative incidence of BOS was 1.6% (95%CI 1.5%-1.6%) , 4.8% (95%CI 4.7%-4.8%) and 5.8% (95%CI 5.7%-5.8%) , respectively. Among patients with chronic graft-versus-host disease (cGVHD) , the cumulative incidence at the same intervals was 2.8% (95%CI 2.7%-2.8%) , 9.5% (95%CI 9.4%-9.5%) and 11.5% (95%CI 11.4%-11.6%) , respectively. In the multivariate analysis, the risk factors for BOS were high-risk primary disease, Ⅱ-Ⅳ aGVHD and preceding cGVHD with other organs. The 3-year overall survival (OS) was lower among patients with than those without BOS, but the difference was not significant [71.8% (95%CI 53.9%-89.6%) vs 72.4% (95%CI 68.1%-76.7%) , P=0.400]. Overall 1-year, 3-year survival of patients with BOS from the time of diagnosis was 78.4% (95%CI 61.5%-95.3%) and 37.0% (95%CI 2.5%-71.5%) , respectively, significantly less than those without (93.9% and 89.3%, from day 448 after transplantation, respectively, P<0.001) . Furthermore, we found a significantly higher incidence of transplantation-related mortality (TRM) in patients with compared with patients without BOS (28.2% vs 10.9%, P<0.001) . The main risk factor for OS of BOS patients was the severity of pulmonary impairment at the time of diagnosis. Patients who developed severe BOS had a worse OS than those with moderate and mild BOS (P=0.049) . Conclusion: BOS is a severe pulmonary complication of haplo-HSCT. High-risk primary disease, Ⅱ-Ⅳ aGVHD and preceding cGVHD were independent risk factors for BOS. Patients who developed BOS had a worse OS than those without BOS. The main risk factor for OS of BOS patients was the severity of pulmonary impairment.
Bronchiolitis Obliterans/etiology* ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Humans ; Lung ; Retrospective Studies

Humans ; Bronchiolitis Obliterans Syndrome ; Chronic Disease ; Consensus ; East Asian People ; Graft vs Host Disease/etiology* ; Hematopoietic Stem Cell Transplantation ; Eye Diseases/etiology*

OBJECTIVE: To evaluate the high resolution computed tomography (HRCT) findings of bronchiolitis obliterans (BO) after bone marrow transplantation (BMT). MATERIALS AND METHODS: During the past three years, 11 patients were diagnosed as having BO after BMT when they developed irreversible air flow obstruction, with an FEV1 value of less than 80% of the baseline value, without any clinical evidence of infection. All 11 patients underwent HRCT, of whom eight also underwent follow-up HRCT. The HRCT images were assessed retrospectively for the presence of decreased lung attenuation, segmental or subsegmental bronchial dilatation, diminution of peripheral vascularity, centrilobular nodules, and branching linear structure on the inspiratory images. The lobar distribution of the decreased lung attenuation and bronchial dilatation was also examined. The presence of air trapping was investigated on the expiratory images. The interval changes of the HRCT findings were evaluated in those patients who had follow-up images. RESULTS: Abnormal HRCT findings were present in all cases; the most common abnormalities were decreased lung attenuation (n=11), subsegmental bronchial dilatation (n=6), diminution of peripheral vascularity (n=6), centrilobular nodules or branching linear structure (n=3), and segmental bronchial dilatation (n=3). Expiratory air trapping was noted in all patients. The decreased lung attenuation and bronchial dilatations were more frequent or extensive in the lower lobes. Interval changes were found in all patients with follow-up HRCT: increased extent of decreased lung attenuation (n=7) ; newly developed or progressed bronchial dilatation (n=4) ; and increased lung volume (n=3). CONCLUSION: HRCT scans are abnormal in patients with BO, with the most commonly observed finding being areas of decreased lung attenuation. While the HRCT findings are not specific, it is believed that their common features can assist in the diagnosis of BO in BMT recipients.
Adult ; Bone Marrow Transplantation/*adverse effects ; Bronchiolitis Obliterans/diagnosis/*etiology/*radiography ; Bronchography ; Female ; Human ; Lung/radiography ; Male ; *Tomography, X-Ray Computed ; Transplantation, Homologous

OBJECTIVETo study the clinical features of bronchiolitis obliterans (BO) in children.

METHODSThe clinical data of 28 children with BO between July 2007 and April 2012 was retrospectively reviewed.

RESULTSAll patients presented with persistent or repeated cough and wheezing. Twenty-three cases were post-infectious bronchiolitis obliterans (PIBO), among whom the etiology were adenovirus (12 cases), measles (2 cases), influenza virus A (2 cases), mycoplasma pneumoniae (1 case), mycoplasma pneumoniae coinfection with adenovirus (1 case), respiratory syncytial virus coinfection with Parainfluenza type 3 virus (1 case) and pulmonary tuberculosis (1 case). The etiology of 3 cases was not associated with infection. The etiology was unknown in 2 cases. Pulmonary HRCT revealed that decreased density in 25 cases, mosaic perfusion in 21 cases, bronchial wall thickening in 15 cases, bronchiectasis in 12 cases and air retention in 6 cases. Lung function test was performed on 21 cases and demonstrated that obstructive ventilation disorder in all 21 cases. Bronchodilation test was performed on 18 cases and 17 cases showed a negative result. All 28 cases received corticosteroid treatment, and 24 cases were orally administered with low doses of azithromycin. One case died during hospitalization. Eighteen cases were followed up for 4 months to 4 years and seven months. Clinical manifestations were improved in 12 cases and one case died.

CONCLUSIONSLow respiratory infection is the most common cause of pediatric BO and adenovirus is a major pathogen. Persistent wheezing and cough were main clinical manifestations. Pulmonary HRCT imaging is important for diagnosis and follow-up of BO. Lung function test can typically show obstructive ventilation disorder. Corticosteroid and methotrexate may be effective for treatment of BO. Prognosis of this disease is unsatisfactory. Early diagnosis and treatment, and avoidance of repeated respiratory tract infection may be helpful to improve the prognosis.

Bronchiolitis Obliterans ; diagnosis ; drug therapy ; etiology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Prognosis ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed