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

The aim of this case report is to draw the attention to the occurrence of cystic fibrosis (C.F.) in a Korean infant and thus increase the awareness for the diagnosis. The male infant was presented with a history of recurrent bronchiolitis manifested by severe cough, wheeze and dyspnea from three weeks of age, in whom the diagnosis of C.F. was clinically suspected and was confirmed by demonstration of two elevated sweat chloride levels (97 mEq/L and 99 mEq/L) in the patient. The diagnosis was delayed because the main manifestations of C.F. were the same as the main symptoms of common diseases such as cough, diarrhea and failure to thrive. C.F. is probably underdiagnosed in Korean population both because the diagnosis is not considered since the disease is thought to be uncommon or even not to occur and because diagnostic facilities including the quantitative iontophoresis sweat test are lacking.
Bronchiolitis/diagnosis/*etiology ; Cystic Fibrosis/complications/*diagnosis/ethnology ; Humans ; Infant ; Male

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

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

OBJECTIVEDiffuse panbronchiolitis (DPB) is a chronic progressive disease of the lower respiratory tract, which is prevalent in Asian population. So far, many DPB cases have been found in adults in China. To our knowledge, no pediatric DPB case has ever been reported in China. We describe the first pediatric DPB case in Chinese literature and the second case in the English-language literature.

METHODThe clinical manifestations, characteristic imaging and histological features of this DPB case were summarized.

RESULTSThe patient was a 13-year old girl complained of chronic productive cough with wheezing. Chest auscultation revealed fine moist rales and wheezing over both lung fields. The chest X-ray showed small nodules and reticular opacities in left lower lobe. High-resolution thorax computerized tomography (HRCT) demonstrated bilateral diffuse small centrilobuler nodules and bronchial wall thickening or bronchiectasis in some parts of the lungs. Histopathological examination of transbronchial biopsy specimen revealed lymphocytes and foamy histocytes infiltrated in the walls of bronchi, respiratory bronchioles and adjacent alveoli. Lymphoid follicles were present around some bronchi. Sinus radiographs revealed sinusitis. Lung function studies showed obstruction and restriction. PaO2 was 65 mm Hg. The diagnosis of DPB was made according to the current diagnostic criteria. Low-dose erythromycin [5 - 10 mg/(kg.d)] was effective.

CONCLUSIONDPB could occur in children in China. The major diagnostic clues may include the following: (1) persistent cough, sputum, and dyspnea; (2) coexistent chronic sinusitis; (3) bilateral diffuse small nodular opacities on HRCT. Low-dose erythromycin was effective in treatment of the case with DPB.

Adult ; Bronchiolitis ; pathology ; China ; epidemiology ; Chronic Disease ; Cough ; blood ; diagnosis ; etiology ; Diagnosis, Differential ; Female ; Humans ; Lung ; pathology ; Pulmonary Disease, Chronic Obstructive ; diagnosis ; pathology ; Respiratory Function Tests ; methods ; Sputum ; microbiology

A case of inflammatory pseudotumor of the lung occurring in a six-year-old boy is reported with clinicopathologic findings, including its ultrastructure. The patient had had frequent upper respiratory tract infections, and one and half year before the discovery of the lung mass, he suffered from pneumonia of the right lung, which was serologically proven to be a mycoplasma pneumoniae infection. Exploratory thoracotomy revealed a large mediastinal mass that was removed together with the right middle and lower lobes of the lung. The mass arose from the lung with an endobronchial element. Microscopically, the mass was composed of a variety of inflammatory and mesenchymal cells, including plasma cells, histiocytes, lymphocytes, and fibroblast-like spindle cells. Ultrastructurally, the spindle-shaped mesenchymal cells were either fibroblasts or myofibroblasts. At the time of diagnosis of the inflammatory pseudotumor of the lung, the serum titer of antimycoplasma antibody rose again, and the lung parenchyma adjacent to the mass showed interstitial pneumonia with features of bronchiolitis obliterans. The present case suggests that the inflammatory pseudotumor of the lung could be a postinflammatory lesion associated with mycoplasma pneumoniae infection.
Antibodies, Bacterial/blood ; Bronchiolitis Obliterans/complications ; Child ; Diagnosis, Differential ; Fibroma/etiology/*pathology/surgery ; Humans ; Lung Neoplasms/etiology/*pathology/surgery ; Male ; Mycoplasma pneumoniae/immunology/*pathogenicity ; Pneumonia, Mycoplasma/complications/microbiology/*pathology ; Tomography, X-Ray Computed