Bronchiolitis obliterans is an infrequent disorder characterized by partial or complete obstruction of bronchi and bronchioles by fibrous tissue following an insult to the lower respiratory tract. In the pediatric population, Bronchiolitis obliterans has most frequently been preceded by respiratory tract infection by adenovirus, influenza, measles. But it may occur in young adults after mycoplasma infection. We experienced the case of Bronchiolitis obliterans, which developed after the onset of Stevens-Johnson syndrome due to Mycoplasma pneumoniae infection and present a brief review of the related literature.
Adenoviridae ; Bronchi ; Bronchioles ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Humans ; Influenza, Human ; Measles ; Mycoplasma Infections ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pneumonia, Mycoplasma* ; Respiratory System ; Respiratory Tract Infections ; Stevens-Johnson Syndrome* ; Young Adult

PURPOSE: Bronchiectasis in children is still one of the most common causes of childhood mortality in developing countries. The aim of this study was to investigate the epidemiological characteristics, clinical features, underlying etiologic factors, and distinct change in the management of patients with bronchiectasis at Asan Medical Center Children's Hospital of Seoul. METHODS: A retrospective study of children diagnosed with bronchiectasis was conducted between January 1999 and December 2008. All patients underwent a comprehensive examination to identify etiologic factors. Data analysis in terms of age at onset, initial presenting symptoms, underlying etiology, distinct change in treatment, distribution of pulmonary involvement on computed tomography (CT), and causative microbiological flora triggering secondary infections was performed. RESULTS: The median age at the time of the diagnosis of bronchiectasis was 7.6 years (range, 2 months to 18 years). Persistent coughing was the most common symptom. The underlying etiologies identified in 79 patients (85.8%) included bronchiolitis obliterans (32.6%), childhood respiratory infection (20.6%), interstitial lung disease (17.3%), immunodeficiency (8.6%), and primary ciliary dyskinesia (4.3%). In 53 children (67%), the identified cause led to a distinct and individualized change in management. The distribution of CT abnormalities had no correlation with the underlying cause of bronchiectasis. CONCLUSIONS: Selected Korean children with bronchiectasis were reviewed to identify diverse underlying etiologies. All children with bronchiectasis should be comprehensively investigated because identifying underlying causes may have a major impact on their management and prognosis.
Bronchiectasis ; Bronchiolitis Obliterans ; Child ; Coinfection ; Cough ; Developing Countries ; Humans ; Kartagener Syndrome ; Lung Diseases, Interstitial ; Prognosis ; Retrospective Studies ; Statistics as Topic

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

OBJECTIVETo recognize the clinical features of the bronchiolitis obliterans.

METHODClinical manifestation, chest X-ray, computed tomography (CT) and pulmonary function of 4 cases with bronchiolitis obliterans were retrospectively analyzed.

RESULTTwo cases were after Stevens-Johnson syndrome (SJS), the other 2 were after severe pneumonia, including one suffered from adenovirus pneumonia. Cough, tachypnea and wheezing persisted in all the 4 patients. The symptoms lasted for at least 6 weeks, in one case for over one year. Crackles and wheezing were present in all the 4 cases. Hyperinflation was seen in chest radiographs in all cases. On pulmonary CT/high-resolution CT (HRCT), patchy opacity and bronchial wall thickening were seen in each patient. Areas of air trapping were seen in three cases. Bronchiectasis was seen in 2 cases, atelectasis and mosaic perfusion were seen respectively in one case. PO(2) was low in all the four cases. Wheezing was not responsive to beta(2) agonist and other bronchodilating therapy. Prednisone was used at a dose of 1 mg/(kg.d) in 3 cases. Two cases were followed up for 3 months. The clinical condition of one case was improved, whose wheezing and bronchiolar constriction disappeared, cough and dyspnea were also relieved. However, the condition of one patient was not improved, although the wheezing disappeared. The HRCT of these two cases showed no improvement.

CONCLUSIONClinical symptoms of BO were cough, tachypnea, and wheezing after acute lung injury. Crackles and wheezing were the most common signs in the BO. Chest radiographs showed hyperinflation. Pulmonary CT showed bronchial wall thickening, bronchiectasis, atelectasis, and mosaic perfusion. Pulmonary function tests suggested obstruction of small airway.

Bronchiolitis Obliterans ; etiology ; pathology ; physiopathology ; Child ; Child, Preschool ; Humans ; Infant ; Male ; Pneumonia ; complications ; Pneumonia, Viral ; complications ; Prognosis ; Respiratory Function Tests ; Stevens-Johnson Syndrome ; complications ; Tomography, X-Ray Computed

Bronchiolitis obliterans organizing pneumonia(BOOP) is a pathologic entity characterized by the formation of plugs of fibrous tissue in bronchioles and alveolar ducts. It had been described in association with several connective tissue diseases, i.e. systemic lupus erythematosus, Behcet's disease, polymyositis-dermatomyositis, polymyalgia rheumatica and Sjogren's syndrome. Recently, there were few additional reports on BOOP, anteceded by rheumatoid arthritis. We had experienced a case of 60 years old female patient who had been admitted for acute respiratory difficulty and abnormal chest roentgenographic findings. She was diagnosed as having rhematoid arthritis 20 years ago. On the course of our treatment, she did not respond to antibiotics. Moreover, no microorganism grew in the sputum and blood. Thus, the bronchoscopic biopsy was done. The patient showed a dramatic response to steroid therapy, and the pathologic findings of the bronchoscopic biopsy confirmed as BOOP. To our knowledge, this is the first reported case of BOOP anteceded by rheumatoid arthritis in Korea.
Anti-Bacterial Agents ; Arthritis ; Arthritis, Rheumatoid* ; Biopsy ; Bronchioles ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia* ; Dermatomyositis ; Female ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Polymyalgia Rheumatica ; Sjogren's Syndrome ; Sputum ; Thorax

Bronchiolitis Obliterans(BO) is one of the rare inflammatory lung disease that primarily affects bronchi and bronchioles, followed by partial or complete obstruction. In children, the etiology of BO has been under consideration, but infections and toxic inhalation, connective tissue diseases, lung transplantation, congestive pulmonary edema, etc., may be causes of BO. Even though steroid therapy was reported as an effective treatment, there are few data regarding the epidemiology, pathophysiology, long term prognosis and therapy. Here we describe a 5-year-old boy who presented with intractable wheezing associated with Stevens-Johnson syndrome after Varicella-Zoster virus infection and was diagnosed as BO through chest high-resolution computed tomography.
Bronchi ; Bronchioles ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Child ; Child, Preschool ; Connective Tissue Diseases ; Epidemiology ; Estrogens, Conjugated (USP) ; Herpesvirus 3, Human ; Humans ; Inhalation ; Lung Diseases ; Lung Transplantation ; Male ; Prognosis ; Pulmonary Edema ; Respiratory Sounds ; Stevens-Johnson Syndrome* ; Thorax

PURPOSE: Adenoviral respiratory infection can develop a life threatening condition similar to severe bacterial pneumonia. Despite adequate intensive care, this infection progresses to acute respiratory distress syndrome and causes permanent lung damage in some patients. In this study, we analyzed clinical features and long-term follow-up clinical data of this infection in children. METHODS: Forty-seven cases of inpatients were diagnosed as adenoviral respiratory tract infection by viral culture of nasal aspirates or histopathological diagnosis at Samsung Medical Center during the period from February 1995 to July 1998. We reviewed medical records retrospectively. RESULTS: During this study, 1301 cases of acute respiratory tract infection were investigated, of which 47 cases were confirmed as adenoviral infection. Age ranged from 2 months to 6 years. Initial symptoms were productive cough(100%), fever(91%), dyspnea(62%), diarrhea(40%), and conjunctival injection(30%). Thirty-nine cases(83%) were pneumonia and 7 cases(15%) were bronchiolitis. The radiologic findings were pneumonic consolidation(60%), effusion(36%), infiltration(19 %), and atelectasis(13%). Five cases developed acute respiratory distress syndrome and 3 cases expired. In 31 cases of recovered patient, follow-up evaluation was done at a minimum of 3 months(mean duration 9.9+/-9.3 months). Under the follow-up evaluation, 13 patients(42%) showed physical and radiologic findings that consisted with bronchiolitis obliterans or bronchiectasis. CONCLUSION: Because of severe clinical manifestations and pulmonary complications, early diagnosis, adequate management, and long-term follow-up are needed for adenoviral respiratory tract infection.
Adenoviridae ; Bronchiectasis ; Bronchiolitis ; Bronchiolitis Obliterans ; Child* ; Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Humans ; Inpatients ; Critical Care ; Lung ; Medical Records ; Pneumonia ; Pneumonia, Bacterial ; Respiratory Distress Syndrome, Adult ; Respiratory Tract Infections ; Retrospective Studies

Anti-Inflammatory Agents ; therapeutic use ; Bronchi ; pathology ; Bronchiolitis Obliterans ; pathology ; Humans ; Lung ; pathology ; Male ; Methylprednisolone ; therapeutic use ; Middle Aged ; Pneumonia ; pathology ; Pulmonary Fibrosis ; complications ; drug therapy ; pathology ; Respiratory Distress Syndrome, Adult ; etiology

Objective: To evaluate the efficacy and safety of HLA-haploidentical hematopoietic stem cell transplantation (allo-HSCT) for hepatitis-related aplastic anemia (HRAA) patients. Methods: Retrospective analysis was performed on hepatitis-associated aplastic anemia patients who received haplo-HSCT at our center between January 2012 and June 2022. October 30, 2022 was the final date of follow-up. Results: This study included 28 HRAA patients receiving allo-HSCT, including 18 males (64.3% ) and 10 females (35.7% ), with a median age of 25.5 (9-44) years. About 17 cases of severe aplastic anemia (SAA), 10 cases of very severe aplastic anemia (VSAA), and 1 case of transfusion-dependent aplastic anemia (TD-NSAA) were identified. Among 28 patients, 15 patients received haplo-HSCT, and 13 received MSD-HSCT. The 2-year overall survival (OS) rate, the 2-year failure-free survival (FFS) rate, the 2-year transplant-related mortality (TRM) rate, the 100-day grade Ⅱ-Ⅳ acute graft-versus-host disease (aGVHD) cumulative incidence rate, and the 2-year chronic graft-versus-host disease (cGVHD) cumulative incidence rate were 81.4%, 81.4% (95% CI 10.5% -20.6% ), 14.6% (95% CI 5.7% -34.3% ), 25.0% (95% CI 12.8% -45.4% ), and 4.2% (95% CI 0.6% -25.4% ), respectively. After transplantation, all patients had no significant liver function damage. Compared with the MSD-HSCT group, only the incidence of cytomegaloviremia was significantly higher in the haplo-HSCT group [60.0% (95% CI 35.2% -84.8% ) vs 7.7% (95% CI 0-22.2% ), P=0.004]. No statistically significant difference in the Epstein-Barr virus was found in the 2-year OS, 2-year FFS, 2-year TRM, and 100-day grade Ⅱ-Ⅳ aGVHD cumulative incidence rates and 2-year cGVHD cumulative incidence rate. Conclusion: Allo-HSCT is safe and effective for HRAA, and haplo-HSCT can be used as a safe and effective alternative for newly diagnosed HRAA patients who cannot obtain HLA-matched sibling donors.
Male ; Female ; Humans ; Adult ; Treatment Outcome ; Anemia, Aplastic/therapy* ; Retrospective Studies ; Epstein-Barr Virus Infections ; Herpesvirus 4, Human ; Graft vs Host Disease/etiology* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Hepatitis/etiology* ; Bronchiolitis Obliterans Syndrome ; Transplantation Conditioning

Objective: To investigate the early effect and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with a 10-day decitabine-containing conditioning regimen in the treatment of acute myeloid leukemia (AML) /myelodysplastic syndrome (MDS) . Methods: From April 2021 to May 2022, 31 AML/MDS patients who received allo-HSCT with a 10-day decitabine-containing conditioning regimen were analyzed. Results: AML (n=10), MDS-AML (n=6), CMML-AML (n=1), and MDS (n=14) were identified in 31 patients, 16 males, and 15 females, with a median age of 41 (20-55) yr. Neutrophils and platelets were successfully implanted in 31 patients (100%), with a median implantation duration of 12 (9-30) and 14 (9-42) days, respectively. During the preconditioning period, 16 patients (51.6%) developed oral mucositis, with 15 cases of Ⅰ/Ⅱ grade (48.4%) and one case of Ⅲ grade (3.2%). After transplantation, 13 patients (41.9%) developed CMV viremia, six patients (19.4%) developed hemorrhagic cystitis, and four patients (12.9%) developed a local infection. The median time of acute graft versus host disease (aGVHD) following transplantation was 33 (12-111) days. The cumulative incidence of aGVHD and Ⅲ/Ⅳ grade aGVHD was 41.9% (95% CI 26.9%-61.0%) and 22.9% (95% CI 13.5%-47.5%), respectively. There was no severe cGVHD, and mild and moderate chronic GVHD (cGVHD) incidence was 23.5% (95% CI 12.1%-43.6%). As of November 30, 2022, only one of the 31 patients had relapsed, with a 1-yr cumulative relapse rate (CIR) of 3.2% (95% CI 0.5%-20.7%). There was only one relapse patient death and no non-relapse deaths. The 1-yr overall survival (OS) and disease-free survival (DFS) rates were 92.9% (95% CI 80.3%-100%) and 96.8% (95% CI 90.8%-100%), respectively. Conclusions: A 10-day decitabine-containing conditioning regimen for allo-HSCT reduced relapse and was safe and feasible in treating AML/MDS.
Male ; Female ; Humans ; Decitabine ; Myelodysplastic Syndromes/therapy* ; Leukemia, Myeloid, Acute/complications* ; Disease-Free Survival ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Recurrence ; Chronic Disease ; Graft vs Host Disease/etiology* ; Transplantation Conditioning/adverse effects* ; Bronchiolitis Obliterans Syndrome ; Retrospective Studies