Uma doença pulmonar que está principalmente ligada ao mau funcionamento contínuo de um pulmão após um transplante. Ela se caracteriza por inflamação e cicatrização (fibrose) das paredes dos bronquíolos (pequenos tubos de ar dentro dos pulmões). Isso faz com que os bronquíolos se estreitem, resultando em uma obstrução progressiva e irreversível da passagem de ar.
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Uma doença pulmonar que está principalmente ligada ao mau funcionamento contínuo de um pulmão após um transplante. Ela se caracteriza por inflamação e cicatrização (fibrose) das paredes dos bronquíolos (pequenos tubos de ar dentro dos pulmões). Isso faz com que os bronquíolos se estreitem, resultando em uma obstrução progressiva e irreversível da passagem de ar.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.450
Ruxolitinib and Tocilizumab for Child Bronchiolitis Obliterans After Toxic Epidermal Necrolysis.
Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous disorder with rare but potentially fatal respiratory complications. We present an exceptional documented case of TEN with severe bronchial obstruction successfully treated with tocilizumab and ruxolitinib in an 11-year-old patient. Despite early methylprednisolone pulse therapy, a repeat bronchoscopy showed progressive obstruction of proximal bronchial lumen by synechiae and mucosal membranes. Following multidisciplinary discussion, treatment was intensified with tocilizumab and ruxolitinib, resulting in marked respiratory improvement and decreased proinflammatory cytokines in bronchoalveolar lavage. No adverse effects were observed during 8 months of follow-up. Recent research supports Janus kinase inhibitors as targeted therapy in TEN.
BET inhibition blunts antibody production and macrophage-mediated fibrosis to restore lung function in murine cGVHD.
Chronic graft-versus-host disease (cGVHD) significantly contributes to late mortality after allogeneic stem cell transplantation, with bronchiolitis obliterans syndrome (BOS) being a particularly lethal and treatment-resistant complication despite available therapies. Bromodomain and Extra terminal (BET) proteins are epigenetic readers driving inflammatory transcriptional programs across multiple cell types. We hypothesized that BET inhibition would suppress inflammatory T and B cells while also decreasing macrophage polarization to a profibrotic phenotype, alleviating disease. In an established BOS cGVHD model, BET inhibition reduced germinal center formation and response through a reduction of the CXCL13:CXCR5 axis, inflammatory Tfh/GC B cells in the spleen along with a reduction in plasma cell infiltration within the lung. Mice with cGVHD had elevated pathogenic IgG1 and IgM, both in circulation and deposited on lung tissue, which was attenuated under BET inhibition. Single-cell RNA sequencing analysis revealed distinct cell states in the BOS lung vs. control. In cGVHD mice, GSEA analysis revealed upregulation of profibrotic Arginase1 and Tgfb1 expression in alveolar macrophages (AM) and interstitial macrophages (IM), which was significantly reduced with BET inhibition. Furthermore, BET inhibition targeted lung-infiltrating M2 macrophages, through selective depletion of CD206+FcgR+ IMs and AMs, ultimately resulting in reduced collagen deposition and improved lung function. Our findings reveal a previously unrecognized mechanistic axis of BET regulation during cGVHD fibrosis and highlight BET inhibition as a promising therapeutic strategy.
Post-Infectious Bronchiolitis Obliterans as a Model of Immune-Mediated Airway Fibrosis: A Pediatric Case Report.
Post-infectious bronchiolitis obliterans (PIBO) is a rare but severe chronic lung disease of childhood, characterized by irreversible small-airway obstruction following severe lower respiratory tract infections early in life. The disease course is often progressive and associated with long-term respiratory morbidity, while effective disease-modifying therapies remain limited. We report the case of a young child who developed severe PIBO following adenovirus pneumonia complicated by prolonged respiratory failure and multisystem involvement. Diagnosis was based on persistent respiratory symptoms, characteristic radiologic findings, and poor response to conventional anti-inflammatory treatment. Given the severity of the clinical course and steroid-refractory disease, an individualized immunomodulatory strategy, including hydroxychloroquine, was initiated within a multidisciplinary framework. During follow-up, the patient showed progressive clinical improvement, with gradual weaning from continuous oxygen supplementation, fewer respiratory exacerbations, simplification of systemic therapies, and radiologic findings consistent with partial improvement. Although causal conclusions regarding treatment efficacy cannot be drawn from a single case, the overall disease trajectory appeared more favorable than typically reported in PIBO cohorts. This case supports the emerging view of PIBO as an immune-mediated airway fibrotic disorder and underscores the importance of integrating detailed clinical phenotyping with evolving molecular insights to inform future precision medicine in pediatric post-infectious airway disease.
Targeting integrin beta 4 in diacetyl-induced anoikis of the airway epithelium.
Diacetyl (DA) is a flavoring chemical commonly found in food and beverages. When inhaled at occupationally relevant concentrations, DA can cause bronchiolitis obliterans (BO), yet the mechanisms remain poorly understood. Common to all forms of BO is airway epithelial injury, with failed epithelial cell survival contributing to BO development. The purpose of the current study was to target integrin beta 4 (ITGβ4) - one of the primary integrins that connect airway epithelial cells to the basement membrane - in DA-exposed airway epithelial cells to prevent adhesion-related cell apoptosis ('anoikis'). Sprague-Dawley rats were exposed to 200 parts-per-million DA vapor or filtered air for 6 hours per day for 5 consecutive days, then monitored for 5 weeks post-exposure and assessed for airway remodeling using Trichrome staining and the hydroxyproline assay. ITGβ4 protein expression was assessed via western blot as well as co-immunofluorescent staining using common airway epithelial cell markers. In parallel, primary human airway epithelial cells and human bronchial epithelial cells (16HBE14o-) were grown in vitro, exposed to DA, and treated with the pan-caspase inhibitor Z-VAD-FMK or transfected with ITGB4. End-points included viability staining, extracellular caspase 3/7 activity, and ITGβ4 protein expression. Rats exposed to DA vapors developed significant airway remodeling with increased total lung collagen content and sub-epithelial airway collagen deposition. Airway epithelial ITGβ4 expression remained decreased weeks after DA exposure with expansion of pan-cytokeratin positive epithelial cells, independent of ciliated and club cell markers. In parallel, DA-exposed human airway epithelial cells exposed in vitro developed significant anoikis. Treatment with Z-VAD-FMK reduced anoikis and improved ITGβ4 cytoplasmic surface expression but failed to improve total ITGβ4 protein expression. ITGB4 overexpression failed to suppress ITGβ4 cleavage or prevent anoikis. In summary, DA exposure in both rats and human airway epithelial cells results in caspase-mediated cleavage of ITGβ4. Future studies targeting post-translational modifications of ITGβ4 may prevent airway epithelial cell anoikis and fibrotic remodeling.
Longitudinal Study of Late Acute and Chronic Graft-Versus-Host Disease after Allogeneic Hematopoietic Cell Transplantation: A Long-Term Follow-up Study from the Chronic Graft-Versus-Host Disease Consortium.
Late acute and chronic graft-versus-host disease (GVHD) contribute to morbidity and death after allogeneic hematopoietic cell transplantation (HCT). A prior national Chronic GVHD Consortium longitudinal study enrolled patients pre- or early post-HCT and identified the incidence of late acute GVHD, chronic GVHD, and chronic GVHD subtypes of bronchiolitis obliterans syndrome (BOS) and cutaneous sclerosis. We now report 10-yr follow-up from that study in a long-term follow-up analysis (N = 911 subjects). Late acute GVHD occurred in 11% at a median of 5.5 mo. Chronic GVHD in total occurred in 54% with median onset of 7.4 mo. BOS (4% of subjects, median onset 12.6 mo) and cutaneous sclerosis (10% of subjects, median onset of 17.2 mo) were less frequent and had later-onset. The long-term analysis demonstrated additional GVHD events occurring beyond 1 to 2 yr post-HCT. Inter-conversion between GVHD types was common, and notably included new development of BOS and cutaneous sclerosis after initial chronic GVHD presentation without these manifestations. GVHD-free, relapse-free survival (GVHD-DFS, survival without relapse or development of late acute or chronic GVHD) was 22% at 2 yr, 18% at 5 yr, and 15% at 10 yr post-HCT. Non-relapse mortality continued to increase beyond 2 yr, with 10-yr estimates up to 35% (late acute), 31% (chronic GVHD), 62% (BOS), and 36% (cutaneous sclerosis). Durable complete discontinuation of immune suppression was uncommon for all GVHD types. These data suggest that extended surveillance for late acute and chronic GVHD is needed post-HCT due to late occurrences, and that associated mortality is high through 10 yr post-GVHD onset.
Publicações recentes
Risk factors and prediction models for bronchitis obliterans after severe Mycoplasma pneumoniae pneumonia with plastic bronchitis in children.
Distinct central and peripheral airway features in bronchiolitis obliterans and COPD: an observational study.
Pulmonary complications in pediatric hematopoietic stem cell transplantation: an overview for pediatricians.
Early BAL microRNA Signatures Delineate Biological Trajectories Towards CLAD After Lung Transplantation.
Pulmonary Function Deficit and Clinical Associations in Childhood Acute Lymphoblastic Leukaemia Survivors: A National Retrospective ALL-STAR Lungs Cohort Study.
📚 EuropePMC1.835 artigos no totalmostrando 197
Efficacy and safety of a new cladribine-based conditioning regimen for allogeneic hematopoietic stem cell transplantation in children with relapsed or refractory acute myeloid leukemia.
Frontiers in medicinePost-transplant Cyclophosphamide Reduces Bronchiolitis Obliterans Syndrome Risk Through Chronic Graft-versus-Host Disease Prevention: A Multicenter Cohort Study.
ChestMycoplasma pneumoniae-induced Postinfectious Bronchiolitis Obliterans in a Child: A Case Report From Taiwan.
The Pediatric infectious disease journalSwyer-James-MacLeod Syndrome: A Rare Cause of Unilateral Hyperlucent Lung in a Two-Year-Old Male Child.
CureusA Tale of Two Lungs: 99mTc-MAA Pulmonary Scintigraphy Reveals Unilateral Chronic Lung Allograft Dysfunction (CLAD) After Bilateral Living-Donor LobarLung Transplants (LDLLT).
Clinical nuclear medicineKnowledge structure and academic influence in lung transplantation: a systematically bibliometric analysis of highly cited documents and multi-perspective extensive review.
Journal of thoracic diseaseRuxolitinib and Tocilizumab for Child Bronchiolitis Obliterans After Toxic Epidermal Necrolysis.
PediatricsBET inhibition blunts antibody production and macrophage-mediated fibrosis to restore lung function in murine cGVHD.
BloodBronchiolitis Obliterans Syndrome Following High-Dose Chemotherapy and Autologous Hematopoietic Stem Cell Transplantation in a Pediatric Patient With Neuroblastoma.
CureusManagement of CMV Pneumonia, DAH, and BOS Following HSCT in a Child with β-Thalassemia: A Case Report.
Journal of inflammation researchEffects of Nutritional Support Combined With Respiratory Training on Pulmonary Function in Patients With Obliterative Bronchiolitis: A Propensity Score Matching Study.
British journal of hospital medicine (London, England : 2005)Lung retransplantation after initial single lung transplantation.
JHLT openUpdates, Management, and Future of Diagnosing and Managing Chronic Lung Allograft Dysfunction.
Journal of clinical medicineInterleukin-2 Receptor Antagonist Induction Therapy in Lung Transplantation-A Meta-Analysis of Reconstructed Time-to-Event Data.
Journal of clinical medicinePost-Infectious Bronchiolitis Obliterans as a Model of Immune-Mediated Airway Fibrosis: A Pediatric Case Report.
International journal of molecular sciencesNovel translational pulmonary MRI in pediatrics: A Review of the last 10 years.
The European respiratory journalTargeting integrin beta 4 in diacetyl-induced anoikis of the airway epithelium.
Cell death discoveryThoracic Surgery - notes for its history in Brazil (Part II): Lung transplantation.
Portuguese journal of cardiac thoracic and vascular surgeryLongitudinal Study of Late Acute and Chronic Graft-Versus-Host Disease after Allogeneic Hematopoietic Cell Transplantation: A Long-Term Follow-up Study from the Chronic Graft-Versus-Host Disease Consortium.
Transplantation and cellular therapyMachine learning and bioinformatics-based identification of mitophagy-related diagnostic biomarkers in bronchiolitis obliterans.
MedicineImmune gene correlation networks differentiate both chronic lung allograft dysfunction and survival.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant SurgeonsLong-term Outcome of Lung Retransplantation for Chronic Lung Allograft Dysfunction After En-bloc Heart and Lung Transplantation.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgerySuccessful Bilateral-Lung Transplantation With Long Survival Time for Multicentric Castleman Disease With Fatal Pulmonary Involvement.
Transplantation proceedingsBronchiolitis obliterans complicating follicular lymphoma: a case series and clinical insights.
International journal of hematologySurvival improvement with sirolimus plus tacrolimus immunosuppression for treatment of bronchiolitis obliterans syndrome after lung transplantation.
JHLT openAssociation between adjunctive corticosteroid therapy and clinical outcomes in children with severe viral community-acquired pneumonia: A retrospective cohort study.
MedicineSteroid-Refractory Cryptogenic Organising Pneumonia (COP) in a Patient With Mannose-Binding Lectin (MBL) Deficiency.
Respirology case reportsCharacteristics and significance of bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation at bronchoscopy.
Respiratory researchPrediction of Long-Term Mortality in Acute Hypercapnic Respiratory Failure with Use of Low-Flow Veno-Venous Extracorporeal CO2 Removal: A Retrospective Single-Center Study.
Blood purificationDeterminants of Asthma Control in Jordanian Children: The Role of Comorbidities and FeNO Levels.
Children (Basel, Switzerland)Passenger Lymphocyte Syndrome in Pediatric Lung Transplantation: A Recipient With Previous Hematopoietic Stem Cell Transplantation.
Pediatric transplantationBronchiolitis Obliterans as an Initial Presentation in an Adolescent With HIV: A Case Report.
Pediatric pulmonologyImmunoglobulin levels of children with bronchiolitis obliterans syndrome after hematopoietic cell transplantation.
Frontiers in medicineDiacetyl-induced occupational lung disease: insights from two cases.
Clinical toxicology (Philadelphia, Pa.)Autoantibody repertoire analysis in paraneoplastic pemphigus reveals novel targets linked to mucocutaneous blistering and bronchiolitis obliterans.
Communications medicineAntineutrophil Cytoplasmic Antibodies Contribute to Airway Inflammation via Induction of Neutrophil Extracellular Traps in Children With Bronchiolitis Obliterans.
The clinical respiratory journalFrom the archives of MD Anderson Cancer Center: Paraneoplastic autoimmune multiorgan syndrome (PAMS) associated with stroma-rich Castleman disease.
Annals of diagnostic pathologyImmunodeficiency-Associated Childhood Interstitial Lung Diseases: Data from the Türkiye chILD Registry.
Balkan medical journalOutcomes of Lung Transplantation for Bronchiolitis Obliterans after Hematopoietic Stem Cell Transplantation Compared with Those for Idiopathic Pulmonary Fibrosis.
Yonsei medical journalSpatial Transcriptomics Analysis of Macrophage-to-Myofibroblast Transition in Bronchiolitis Obliterans Following Hematopoietic Stem Cell Transplantation.
Transplantation and cellular therapyQuantitative CT and Artificial Intelligence in Chronic Lung Disease.
Journal of thoracic imagingClassical complement activation in light and heavy chain deposition disease with acquired cutis laxa and bronchiolitis obliterans: a case report of monoclonal gammopathy of clinical significance.
Frontiers in immunologyCystatin C prevents tissue injury after lung transplantation.
Life science allianceDiacetyl-induced bronchiolitis obliterans involves ubiquitin C upregulation and fibrosis-related gene activation in rats.
Frontiers in pharmacology[Therapy of follicular lymphoma associated bronchiolitis obliterans with lung transplantation: 2 cases and literature review].
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseasesFungal colonization and CLAD in lung transplant recipients: Implications for antifungal prophylaxis and graft survival.
JHLT openCardiorespiratory fitness in patients with post-infectious bronchiolitis obliterans: a cross-sectional study.
ERJ open researchPsittacosis chlamydia pneumonia complicated with organizing pneumonia: a case report and literature review.
Frontiers in medicineTo Respond or Not to Respond: That's the Question in Bronchiolitis Obliterans Syndrome Treatment.
TransplantationExtracorporeal Photopheresis for Chronic Graft-Versus-Host Disease According to 2014 NIH Consensus Criteria.
Journal of clinical apheresisRuxolitinib as second-line therapy for bronchiolitis obliterans syndrome after hematopoietic cell transplantation: a retrospective multicenter study.
Annals of hematologyHMGA1 drives EMT in obliterative bronchiolitis through epigenetic regulation of chromatin accessibility in pulmonary epithelial cells.
Clinical epigeneticsCurrent therapeutics for sclerotic and fibrotic manifestations of chronic graft-versus-host disease.
Bone marrow transplantationA European multi-center, randomized controlled trial of Pirfenidone in bronchiolitis obliterans syndrome after bilateral lung transplantation.
The European respiratory journal[A case of unicentric chest wall Castleman disease-associated paraneoplastic autoimmune multiorgan syndrome].
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseasesRisk for severe refractory Mycoplasma pneumoniae pneumonia complicated with bronchitis obliterans among children.
MedicineRegulatory T cell therapy in lung transplantation: bridging the gap from bench to bedside.
Frontiers in immunologyBeyond systemic immunity: the mucosal frontier in bronchiolitis obliterans syndrome pathogenesis.
The European respiratory journalPulmonary small lymphocytic lymphoma combined with paraneoplastic pemphigus and bronchiolitis obliterans: a case report.
BMC pulmonary medicineMycoplasma Pneumoniae bronchiolitis and hypoxemia: A retrospective cohort study on risk and prognosis.
PloS oneRisk factors for bronchiolitis obliterans in children with community-acquired pneumonia and analysis of CT findings and clinical manifestations of pneumonia after the diagnosis of bronchiolitis obliterans.
Annals of medicineDevelopment and validation of a deep learning-based algorithm for quantifying bronchiolitis obliterans in paediatric computed tomography.
The British journal of radiologyRetransplant- and Bronchiolitis Obliterans Syndrome-Free Survival among COVID Lung Transplant Recipients: A National Cohort Study.
Annals of the American Thoracic SocietyBronchiolitis Obliterans in Unicentric Castleman Disease Without Paraneoplastic Pemphigus Requiring Bilateral Lung Transplantation.
CureusBronchiectasis as a Distinct Entity in Patients With Bronchiolitis Obliterans Syndrome After Hematopoietic Cell Transplantation.
Transplantation and cellular therapyBetween hospital and home: Respiratory rehabilitation challenges and needs among caregivers of children with post-infectious bronchiolitis obliterans.
Journal of pediatric nursingNoninfectious Severe Pulmonary Complications in Immunocompromised Critically Ill Patients.
Seminars in respiratory and critical care medicineSpecific risk factors for heart-lung transplantation.
JHLT openPharmacological EZH2 inhibition attenuates post-transplant obliterative bronchiolitis by reprogramming macrophage polarization.
Biochemical pharmacologySerum belumosudil concentrations in a patient receiving a combination of 3 gastric acid-suppressing agents: A case report.
American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System PharmacistsBronchiolitis Obliterans in an Adult Male After Admission With Panton-Valentine Leucocidin Staphylococcal Pneumonia.
CureusClinical Characteristics, Treatment Response, and Outcomes of Bronchiolitis Obliterans Syndrome in Children with Hematologic Malignancies Undergoing Haploidentical Hematopoietic Cell Transplantation with Granulocyte Colony-Stimulating Factor/Antithymocyte Globulin-Based Protocol: A Single-Center Study.
Transplantation and cellular therapyRisk factors for bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation: a systematic review and meta-analysis.
BMC pulmonary medicineCharacterization of Spirometric Response to Standard-of-care Treatment in Lung Allograft Recipients With Bronchiolitis Obliterans and the Utility of Spirometric Criteria for Rescue Therapy: Implications for the Design of Risk-stratified Clinical Trials.
TransplantationRespiratory Virus Infections and Pulmonary Impairment After Allogeneic Hematopoietic Cell Transplantation.
The Journal of infectious diseasesIdentification of Surrogate Outcome Biomarkers in Lung Allograft Recipients With Bronchiolitis Obliterans: Implications for Randomized Controlled Trials.
Transplantation directPulmonary Graft-Versus-Host Disease After Hematopoietic Cell Transplantation.
Clinical journal of oncology nursingDevelopment and Validation of a Nomogram for Predicting Bronchiolitis Obliterans in Children with Severe Adenovirus Pneumonia: Identification of Key Risk Factors.
Pediatric health, medicine and therapeuticsLow-dose versus high-dose methylprednisolone for children with severe Mycoplasma pneumoniae pneumonia (MCMP): A randomized controlled trial.
Pediatric investigationCryptogenic Organizing Pneumonia.
Seminars in respiratory and critical care medicine[Consensus on early screening, prevention, diagnosis and treatment of post-infectious bronchiolitis obliterans (2025)].
Zhonghua er ke za zhi = Chinese journal of pediatricsClinical Implementation of Inspiratory-Expiratory Chest CT: Defining Quality Criteria for Diagnostic Quality and Detection of Concurrent FEV1 Decline following Lung Transplantation.
Radiology. Cardiothoracic imagingTaiwan consensus on pulmonary chronic graft-versus-host disease: A joint statement from the Taiwan Society of Blood and Marrow Transplantation (TBMT) and Taiwan Society of Pulmonary and Critical Care Medicine (TSPCCM).
British journal of haematology[Hyaline vascular Castleman disease with indolent T-lymphoblastic proliferation complicated by paraneoplastic autoimmune multiorgan syndrome: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyNoninfectious pulmonary complications after hematopoietic cell transplantation: a comprehensive review.
Respiratory medicineRespiratory viral infections in lung transplantation: Recent advances in epidemiology, clinical impact, and therapeutic approaches.
JHLT openIncreased reflux burden on pre-transplant reflux testing independently predicts significant pulmonary function decline after lung transplantation.
World journal of transplantationFour decades of heart-lung transplantation: Milestones and outcomes in advanced cardiorespiratory failure.
ESC heart failureNeurological manifestations of chronic graft versus host disease.
Internal medicine journalLung transplantation in a patient with severe bronchiolitis obliterans secondary to Castleman disease.
Respiratory medicine case reportsBronchiectasis in Bronchiolitis Obliterans Syndrome after Hematopoietic Cell Transplantation.
Transplantation and cellular therapyAnesthesia management for lung transplant in children with obliterated bronchiolitis after hematopoietic stem cell transplantation: a single-center experience.
Journal of thoracic diseaseInhibition of BRD4 prevents peribronchial fibrosis in mice with cutaneous lewisite exposure.
Frontiers in molecular biosciencesTreatment Options for Bronchiolitis Obliterans Secondary to Hematopoietic Cell Transplantation in Children: A Systematic Review.
Clinical transplantationSafe and effective antireflux surgery in lung transplant recipients: preliminary results.
Wideochirurgia i inne techniki maloinwazyjne = Videosurgery and other miniinvasive techniquesMachine Learning for 1-Year Graft Failure Prediction in Lung Transplant Recipients: The Korean Organ Transplantation Registry.
Clinical transplantation[Recommendations on the prevention and management of radiation-induced lung injury from the Association francophone pour les soins oncologiques de support and the Société française de radiothérapie oncologique].
Cancer radiotherapie : journal de la Societe francaise de radiotherapie oncologiquePulmonary outcomes of pediatric patients with postinfectious bronchiolitis obliterans treated with pulse methylprednisolone and intravenous immunoglobulin: a single-center cohort study.
European journal of pediatricsFrom Pulmonary Hyperlucency to Pulmonary Hypertension: A Case Report of Swyer-James-MacLeod Syndrome.
CureusThe usefulness of transbronchial lung cryobiopsy for chronic lung allograft dysfunction: A case report.
Respiratory investigationA Severe Infectious Hit to the Lungs With Poor Recovery? Time to Think of Post-Infectious Bronchiolitis Obliterans.
Acta paediatrica (Oslo, Norway : 1992)Pediatric Bronchiolitis Obliterans and Bronchiolitis Obliterans Syndrome: Nursing Considerations.
Critical care nurseCluster analysis for clinical, radiological, and histopathological profiling in chronic pulmonary graft-versus-host disease.
European journal of internal medicineCurrent Practices on Prescribing and Deprescribing for Patients on Long-Term Antibiotic Treatment for Chronic Pulmonary Conditions: An Umbrella Review by the European Society of Clinical Pharmacy (ESCP).
COPDLong-term outcomes of the international EXPAND trial of Organ Care System (OCS) Lung preservation for lung transplantation.
EClinicalMedicineIndications for pediatric lung transplantation in 2025: A new era.
JHLT openBronchodilator Response: Utility and implications in patients undergoing hematopoietic stem cell transplantation.
Respiratory medicinePostinfectious Bronchiolitis Obliterans in Children.
Canadian respiratory journalOne versus two: How much does it matter? A single-center retrospective study evaluating 1-day extracorporeal photopheresis schedule for treating patients with chronic lung allograft rejection.
TransfusionAtelectasis predicts poor prognosis in pediatric macrolides-unresponsive Mycoplasma pneumoniae pneumonia with A2063/2064G mutations treated with azithromycin.
Frontiers in cellular and infection microbiologyEn-Bloc Versus Bilateral Sequential Technique for Pediatric Lung Transplantation.
Pediatric transplantationDisease Classification of Pulmonary Xenon Ventilation MRI Using Artificial Intelligence.
Academic radiologyIt takes two: Aberrant repair and low-grade inflammation characterize bronchiolitis obliterans syndrome after lung transplantation in serum proteomic analysis.
JHLT openRespiratory Burden in Hematology-Related Bronchiectasis: Clinical Characteristics and Treatable Traits.
Respiration; international review of thoracic diseasesAddressing Knowledge Gaps in the Early Detection of Bronchiolitis Obliterans Syndrome after Hematopoietic Cell Transplantation: An Official American Thoracic Society Research Statement.
American journal of respiratory and critical care medicineClinical Impact of Vaping.
ToxicsA model developed by machine learning and principal component analysis for predicting bronchiolitis obliterans syndrome after allogeneic HSCT in a Chinese population with hematologic malignancies.
Computers in biology and medicinePirfenidone for the treatment of bronchiolitis obliterans syndrome related to chronic graft-versus-host disease.
Blood advancesYounger Age and Viral Triggers Were Associated With Post-Infectious Bronchiolitis Obliterans in French Children.
Acta paediatrica (Oslo, Norway : 1992)Inflammatory Markers and Microbiome Dysbiosis in Hematopoietic Cell Transplant Recipients with Lung Graft-versus-Host Disease.
Transplantation and cellular therapyPostoperative outcomes of antireflux surgery in lung transplant recipients.
Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary TractCritical care illness in allogeneic hematopoietic stem-cell transplantation recipients with chronic graft versus host disease.
Critical care (London, England)A new anchor point for gut microbiome to regulate complications of allogeneic hematopoietic stem cell transplantation: oxidative stress.
Experimental hematologyRespiratory management of pediatric patient with bronchiolitis obliterans syndrome during general anesthesia surgery: a case report.
Translational pediatrics[Risk factors for bronchiolitis obliterans after Mycoplasma pneumoniae bronchiolitis in children].
Zhonghua er ke za zhi = Chinese journal of pediatricsCorrigendum to Diagnosis of post-hematopoietic stem cell transplant bronchiolitis obliterans syndrome in children: time for a rethink? Transplantation and Cellular Therapy. 2024 Aug;30(8):760-769.
Transplantation and cellular therapyBronchiectasis after allogeneic hematopoietic cell transplantation - an underdiagnosed complication.
Respiratory medicineEfficacy of Intravenous Methylprednisolone Pulses in Children With Postinfectious Bronchiolitis Obliterans.
Pediatric pulmonologySurvival of patients with advanced chronic lung allograft dysfunction and the role of redo transplantation.
JHLT openExhaled breath particles as a diagnostic tool for bronchiolitis obliterans syndrome in lung transplant recipients: a longitudinal study.
Frontiers in transplantationFPPS Downregulation Attenuates Endoplasmic Reticulum Stress in Pulmonary Epithelial Cells.
The journal of gene medicinePediatric lung transplantation in China, 2019-2023.
World journal of pediatrics : WJPAssociation of Fc gamma-binding protein rs1464897604 polymorphism with bronchiolitis obliterans syndrome in lung transplant recipients.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationWhat's new in the management of pulmonary complications in allogeneic stem cell transplantation?
Expert review of respiratory medicineDiagnostic potential of MMP-9, IL-8, and MPO in bronchoalveolar lavage fluid as combined biomarkers for pediatric post-infectious bronchiolitis obliterans.
Journal of immunology (Baltimore, Md. : 1950)Impaired IgA mucosal immunity following lung transplantation: a potential trigger for bronchiolitis obliterans syndrome.
The European respiratory journalCase report: refractory atelectasis after infection of adenovirus and Mycoplasma Pneumoniae in an immunocompetent patient.
BMC pulmonary medicineUse of quantitative CT chest imaging to derive and assess a radiographic phenotype of deployment-related constrictive bronchiolitis.
Respiratory researchGlomerular disease in chronic graft versus host disease.
NefrologiaThe impact of allogeneic hematopoietic stem cell transplantation on pulmonary complications in adults with inborn errors of immunity.
The Journal of allergy and clinical immunology[Immunological Aspects after Lung Transplantation].
Zentralblatt fur ChirurgieChanges in Chronic Graft-versus-Host Disease Treatment Over Time: A 15-Years Survey Within Allogeneic Hematopoietic Stem Cell Transplant Centers in Germany, Austria, and Switzerland.
Transplantation and cellular therapyRole of genetic modifiers on pulmonary consequences of cancer therapy in children.
Scientific reportscGAS/STING signalling in macrophages aggravates obliterative bronchiolitis via an IFN-α-dependent mechanism after orthotopic tracheal transplantation in mice.
Clinical and translational medicinePost-RSV Bronchial Web Formation in a Lung Transplant Recipient: A Case Report.
Respirology case reportsEffect of Artificial Food Additives on Lung Health-An Overview.
Medicina (Kaunas, Lithuania)Lung Transplantation From Donors With a History of Substance Use.
Clinical transplantationAn Autopsy Case of Bronchiolitis Obliterans Associated With Oral Lichen Planus and Non-Hodgkin Lymphoma.
CureusEvaluating Metabolic Signatures in the Serum of South Korean Patients with Humidifier Disinfectant-Associated Lung Injury Identified through Untargeted Metabolomics.
Environmental health perspectivesClinical features of HAdV-55 in children with respiratory tract infections: a retrospective case series and literature review.
BMC infectious diseasesPediatric Pulmonology 2024 Year in Review: Rare and Diffuse Lung Disease.
Pediatric pulmonologyClinical characteristics of Mycoplasma pneumoniae pneumonia with airway involvement in children.
Pediatric investigationBronchiolitis obliterans due to toxic epidermal necrolysis with structural evaluation using computed tomography bronchography: A case report.
Acute medicine & surgeryShared roles of immune and stromal cells in the pathogenesis of human bronchiolitis obliterans syndrome.
JCI insightChronic Lung Disease after Hospitalization with Adenovirus or Respiratory Syncytial Virus.
The Pediatric infectious disease journalClinical course of children with postinfectious bronchiolitis obliterans with versus without comorbid bronchopulmonary dysplasia.
Clinical and experimental pediatricsUnicentric Castleman disease complicated with bronchiolitis obliterans: A single-centre retrospective study from China.
British journal of haematologyAdenoviral Infections in Immunocompetent Children.
Indian journal of pediatricsThe Role of Pulmonary Genetic Variations in the Pathogenesis of Pediatric Postinfectious Bronchiolitis Obliterans.
Thoracic research and practiceComputed tomography-based radiomic features combined with clinical parameters for predicting post-infectious bronchiolitis obliterans in children with adenovirus pneumonia: a retrospective study.
PeerJOutbreak of Post-Infectious Bronchiolitis Obliterans (PIBO) After Adenovirus Infection: A Case Series and Review of the Literature.
Pediatric pulmonologyRisk factors for bronchiolitis obliterans development in children after Mycoplasma pneumoniae pneumonia: a retrospective study of 981 patients.
Italian journal of pediatricsPulmonary function testing in pediatric allogeneic stem cell transplant recipients to monitor for Bronchiolitis obliterans syndrome: a systematic review.
BMC pediatricsContribution of post-infectious bronchiolitis obliterans to non-cystic fibrosis bronchiectasis in children.
The international journal of tuberculosis and lung disease : the official journal of the International Union against Tuberculosis and Lung DiseaseAntireflux surgery for prevention of chronic lung allograft dysfunction (CLAD) onset after lung transplantation: The earlier the better before CLAD onset? A single-center series of 284 patients.
JHLT openConcomitant abdominal organ transplantation alongside lung transplantation: An ISHLT transplant database analysis.
JHLT openLung transplantation for pulmonary chronic graft-versus-host disease: a missed opportunity?
JHLT openDynamic 19F-MRI of pulmonary ventilation in lung transplant recipients with and without chronic lung allograft dysfunction.
JHLT openRadiological distribution patterns in restrictive chronic lung allograft dysfunction: Impact on survival across all phenotypes.
JHLT openA novel web-based tool for lung transplant patients undergoing extracorporeal photopheresis.
JHLT openAutomated CT Image Processing for the Diagnosis, Prediction, and Differentiation of Phenotypes in Chronic Lung Allograft Dysfunction After Lung Transplantation.
Clinical transplantationEmerging trends and hotspots in animal experimental research on lung transplantation from 2004 to 2023: a bibliometric analysis.
Journal of thoracic diseaseMultiple Myeloma Complicated With Bronchiolitis Obliterans Syndrome After Allogeneic Haematopoietic Stem Cell Transplantation and Subsequently Underwent Lung Transplantation Successfully: A Case Report.
Respirology case reportsDNAH9 variants in children with post-infectious bronchiolitis/bronchitis obliterans.
Orphanet journal of rare diseasesEffect of chronic lung allograft dysfunction phenotypes on the outcome after lung retransplantation: A retrospective single-center data analysis.
JTCVS openEfficacy of Pomalidomide on Motor Performance and Functional Abilities in Patients with Steroid Refractory Chronic Graft Versus Host Disease: A Randomized Clinical Study.
Transplantation and cellular therapyMissed Diagnosis of Cystic Fibrosis in Developing Countries-Need to Raise the Awareness!
Pediatric pulmonology[Post-infectious bronchiolitis obliterans in children: A rare and potentially devastating disease].
Revue des maladies respiratoiresCIP2A promotes bronchiolitis obliterans by activating the NF‑κB pathway.
Molecular medicine reportsMustard lung with a unique oxidative stress profile as an independent pulmonary disease.
International immunopharmacologyDeficiency of T follicular helper cell Tet3 DNA demethylation inhibits pathogenic IgG2c class switching and chronic GVHD.
BloodA case report of Castleman disease with paraneoplastic pemphigus and bronchiolitis obliterans: Challenges and key takeaways.
International journal of surgery case reportsSulfur dioxide exposure of mice induces peribronchiolar fibrosis-A defining feature of deployment-related constrictive bronchiolitis.
PloS oneBronchiolitis in Adults: Etiology, Diagnostic, and Therapeutic Approach.
Respiration; international review of thoracic diseasesHarnessing deep learning to detect bronchiolitis obliterans syndrome from chest CT.
Communications medicineEffectiveness of oral platelet lysate gel to treat oral mucosal manifestations associated with chronic graft versus host disease.
Blood transfusion = Trasfusione del sangueAirway associated inflammation in post-transplant cystic fibrosis patients as a predictor of chronic lung allograft dysfunction (CLAD).
Journal of clinical pathologyDonor-derived cell-free DNA in chronic lung allograft dysfunction phenotypes: a pilot study.
Frontiers in transplantationImmune dysregulation as a driver of bronchiolitis obliterans.
Frontiers in immunologyEfficacy of transbronchial biopsy and frequency of pneumothorax in lung transplant recipients: single center experience.
BMC pulmonary medicineClonal GZMK+CD8+ T cells are identified as a hallmark of the pathogenesis of cGVHD-induced bronchiolitis obliterans syndrome after allogeneic hematopoietic stem cell transplantation.
EBioMedicineEffects of paclitaxel and methotrexate associated with cholesterol-rich nanoemulsions on ischemia-reperfusion injury after unilateral lung transplantation in rats.
Scientific reportsConstruction and validation of a nomogram model to predict bronchiolitis Mycoplasma pneumoniae pneumonia in children.
Scientific reportsHigh-titer rheumatologic markers in serum of veterans with severe pulmonary complications 25-30 years after sulfur mustard exposure.
International immunopharmacologyDistinct inflammatory profiles in mustard lung: A study of sulfur mustard-exposed patients with serious pulmonary complications.
International immunopharmacologyAssociation of hormone profile with various respiratory disorders in sulfur mustard-chemical veterans.
International immunopharmacologyLow-dose heparin sodium as a protective factor against bronchiolitis obliterans formation after adenovirus infection.
Annals of medicineAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação

Comunidade internacional de pacientes, famílias e profissionais de saúde dedicada à Bronquiolite Obliterante (BO). Reúne testemunhos, informações sobre cuidados, medicamentos e profissionais especializados. Apoiada pela associação Hope for Gabriel.
Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Ruxolitinib and Tocilizumab for Child Bronchiolitis Obliterans After Toxic Epidermal Necrolysis.
- BET inhibition blunts antibody production and macrophage-mediated fibrosis to restore lung function in murine cGVHD.
- Post-Infectious Bronchiolitis Obliterans as a Model of Immune-Mediated Airway Fibrosis: A Pediatric Case Report.
- Targeting integrin beta 4 in diacetyl-induced anoikis of the airway epithelium.
- Longitudinal Study of Late Acute and Chronic Graft-Versus-Host Disease after Allogeneic Hematopoietic Cell Transplantation: A Long-Term Follow-up Study from the Chronic Graft-Versus-Host Disease Consortium.
- Risk factors and prediction models for bronchitis obliterans after severe Mycoplasma pneumoniae pneumonia with plastic bronchitis in children.
- Distinct central and peripheral airway features in bronchiolitis obliterans and COPD: an observational study.
- Pulmonary complications in pediatric hematopoietic stem cell transplantation: an overview for pediatricians.
- Early BAL microRNA Signatures Delineate Biological Trajectories Towards CLAD After Lung Transplantation.
- Pulmonary Function Deficit and Clinical Associations in Childhood Acute Lymphoblastic Leukaemia Survivors: A National Retrospective ALL-STAR Lungs Cohort Study.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1303(Orphanet)
- MONDO:0015265(MONDO)
- GARD:5961(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q56013703(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Bronquiolite obliterante
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Ensaios clínicos
- fonte: ClinicalTrials.gov