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European protocols for the diagnosis and initial treatment of interstitial lung disease in children.Thorax Nov 2015Interstitial lung disease in children (chILD) is rare, and most centres will only see a few cases/year. There are numerous possible underlying diagnoses, with specific... (Review)
Review
Interstitial lung disease in children (chILD) is rare, and most centres will only see a few cases/year. There are numerous possible underlying diagnoses, with specific and non-specific treatment possibilities. The chILD-EU collaboration has brought together centres from across Europe to advance understanding of these considerations, and as part of this process, has created standard operating procedures and protocols for the investigation of chILD. Where established consensus documents exist already, for example, for the performance of bronchoalveolar lavage and processing of lung biopsies, these have been adopted. This manuscript reports our proposals for a staged investigation of chILD, starting from when the condition is suspected to defining the diagnosis, using pathways dependent on the clinical condition and the degree of illness of the child. These include the performance of genetic testing, echocardiography, high-resolution CT, bronchoscopy when appropriate and the definitive investigation of lung biopsy, in order to establish a precise diagnosis. Since no randomised controlled trials of treatment have ever been performed, we also report a Delphi consensus process to try to harmonise treatment protocols such as the use of intravenous and oral corticosteroids, and add-on therapies such as hydroxychloroquine and azithromycin. The aim is not to dictate to clinicians when a therapeutic trial should be performed, but to offer the possibility to collaborators of having a unified approach when a decision to treat has been made.
Topics: Bronchoalveolar Lavage; Bronchoscopy; Child; Clinical Protocols; Diagnosis, Differential; Disease Management; Europe; Humans; Lung Diseases, Interstitial; Morbidity; Tomography, X-Ray Computed
PubMed: 26135832
DOI: 10.1136/thoraxjnl-2015-207349 -
Clinical Reviews in Allergy 1990The technique of BAL performed through the fiberoptic bronchoscope has, in two decades, provided clinicians and researchers with the ability to safely sample the... (Review)
Review
The technique of BAL performed through the fiberoptic bronchoscope has, in two decades, provided clinicians and researchers with the ability to safely sample the inflammatory-immune cell milieu of the human lung. Standardized BAL and processing of the lavage constituents provides assistance in determining the optimal care of patients with a variety of lung diseases, and renders diagnosis in selected cases. It has become indispensable in the diagnosis of pulmonary infiltrates in immunocompromised patients, and plays an important role in improving clinical management. Finally, it continues to yield an ever increasing amount of data for the researchers studying the mechanisms and pathogenesis of lung disease. It is likely that BAL will become an even more valuable tool with increasing relevance to the practice of chest medicine in the 1990s.
Topics: Bronchoalveolar Lavage Fluid; Humans; Lung Diseases; Therapeutic Irrigation
PubMed: 2292101
DOI: 10.1007/BF02914451 -
Prostaglandins & Other Lipid Mediators Jun 2022Inflammatory signaling pathways involving eicosanoids and other regulatory lipid mediators are a subject of intensive study, and a role for these in acute lung injury is...
Inflammatory signaling pathways involving eicosanoids and other regulatory lipid mediators are a subject of intensive study, and a role for these in acute lung injury is not yet well understood. We hypothesized that oxylipin release from lung injury could be detected in bronchoalveolar lavage fluid and in plasma. In a porcine model of surfactant depletion, ventilation with hyperinflation was assessed. Bronchoalveolar lavage and plasma samples were analyzed for 37 different fatty acid metabolites (oxylipins). Over time, hyperinflation altered concentrations of 4 oxylipins in plasma (TXB, PGE, 15-HETE and 11-HETE), and 9 oxylipins in bronchoalveolar lavage fluid (PGF, PGE, PGD, 12,13-DiHOME, 11,12-DiHETrE, 13-HODE, 9-HODE, 15-HETE, 11-HETE). Acute lung injury caused by high tidal volume ventilation in this porcine model was associated with rapid changes in some elements of the oxylipin profile, detectable in lavage fluid, and plasma. These oxylipins may be relevant in the pathogenesis of acute lung injury by hyperinflation.
Topics: Acute Lung Injury; Animals; Bronchoalveolar Lavage Fluid; Dinoprostone; Eicosanoids; Oxylipins; Swine
PubMed: 35307566
DOI: 10.1016/j.prostaglandins.2022.106636 -
European Respiratory Review : An... Mar 2023Childhood interstitial lung diseases (chILDs) are rare and heterogeneous diseases with significant morbidity and mortality. An accurate and quick aetiological diagnosis... (Review)
Review
Childhood interstitial lung diseases (chILDs) are rare and heterogeneous diseases with significant morbidity and mortality. An accurate and quick aetiological diagnosis may contribute to better management and personalised treatment. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review summarises the roles of the general paediatrician, paediatric pulmonologists and expert centres in the complex diagnostic workup. Each patient's aetiological chILD diagnosis must be reached without prolonged delays in a stepwise approach from medical history, signs, symptoms, clinical tests and imaging, to advanced genetic analysis and specialised procedures including bronchoalveolar lavage and biopsy, if necessary. Finally, as medical progress is fast, the need to revisit a diagnosis of "undefined chILD" is stressed.
Topics: Child; Humans; Lung Diseases, Interstitial; Diagnostic Imaging; Morbidity; Bronchoalveolar Lavage; Biopsy; Lung
PubMed: 36813289
DOI: 10.1183/16000617.0188-2022 -
The Journal of Heart and Lung... Nov 2020Bronchoalveolar lavage (BAL) is a key clinical and research tool in lung transplantation (LTx). However, BAL collection and processing are not standardized across LTx...
Bronchoalveolar lavage (BAL) is a key clinical and research tool in lung transplantation (LTx). However, BAL collection and processing are not standardized across LTx centers. This International Society for Heart and Lung Transplantation-supported consensus document on BAL standardization aims to clarify definitions and propose common approaches to improve clinical and research practice standards. The following 9 areas are covered: (1) bronchoscopy procedure and BAL collection, (2) sample handling, (3) sample processing for microbiology, (4) cytology, (5) research, (6) microbiome, (7) sample inventory/tracking, (8) donor bronchoscopy, and (9) pediatric considerations. This consensus document aims to harmonize clinical and research practices for BAL collection and processing in LTx. The overarching goal is to enhance standardization and multicenter collaboration within the international LTx community and enable improvement and development of new BAL-based diagnostics.
Topics: Bronchoalveolar Lavage; Consensus; Heart Transplantation; Humans; Lung Transplantation
PubMed: 32773322
DOI: 10.1016/j.healun.2020.07.006 -
European Respiratory Review : An... Jun 2011Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant... (Review)
Review
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic "crazy paving" pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) or anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.
Topics: Autoimmunity; Biopsy; Bronchoalveolar Lavage Fluid; Genetic Predisposition to Disease; Granulocyte-Macrophage Colony-Stimulating Factor; Humans; Immunotherapy; Mutation; Plasmapheresis; Predictive Value of Tests; Pulmonary Alveolar Proteinosis; Pulmonary Surfactant-Associated Proteins; Rare Diseases; Respiratory Function Tests; Risk Factors; Therapeutic Irrigation; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 21632797
DOI: 10.1183/09059180.00001311 -
Revue Des Maladies Respiratoires Dec 2014Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three... (Review)
Review
Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90% of all AP); secondary AP, mainly occurring as a consequence of haematological diseases, or following on from toxic inhalation or infections, and genetic AP, which affects almost exclusively children. AP diagnosis is suspected where chest CT-scan demonstrates interstitial lung disease with a crazy paving aspect; and confirmed by bronchoalveolar lavage, which has a milky appearance and contains periodic acid Schiff positive proteinaceous alveolar deposits. The use of surgical lung biopsy to confirm AP is less frequent nowadays. In this context, positive antibodies against GM-CSF indicates an auto-immune etiology of the AP. Concerning management, whole lung lavage is the gold standard therapy. In refractory AP, new treatments are available such as subcutaneous or inhaled GM-CSF supplementation, or rituximab infusions. The clinical course is unpredictable. Spontaneous improvement or even cure can occur, and the 5-year actuarial survival is 95%. The most frequent complications are infectious etiology.
Topics: Animals; Antibodies, Monoclonal, Murine-Derived; Biopsy; Bronchoalveolar Lavage; Disease Progression; Granulocyte-Macrophage Colony-Stimulating Factor; Humans; Lung Transplantation; Pulmonary Alveolar Proteinosis; Radiography, Thoracic; Rare Diseases; Rituximab
PubMed: 25496792
DOI: 10.1016/j.rmr.2014.08.009 -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... Feb 2021Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the...
OBJECTIVES
Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.
METHODS
We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University.
RESULTS
Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up.
CONCLUSIONS
PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.
Topics: Biopsy; Bronchoalveolar Lavage; Cough; Dyspnea; Humans; Pulmonary Alveolar Proteinosis; Retrospective Studies
PubMed: 33678652
DOI: 10.11817/j.issn.1672-7347.2021.190792 -
Revista Espanola de Quimioterapia :... Apr 2022Patients with a compromised immune system suffer a wide variety of insults. Pulmonary complications remain a major cause of both morbidity and mortality in... (Review)
Review
Patients with a compromised immune system suffer a wide variety of insults. Pulmonary complications remain a major cause of both morbidity and mortality in immunocompromised patients. When such individuals present with radiographic infiltrates, the clinician faces a diagnostic challenge. The differential diagnosis in this setting is broad and includes both infectious and non-infectious conditions. Evaluation of the immunocompromised host with diffuse pulmonary infiltrates can be difficult, frustrating, and time-consuming. This common and serious problem results in significant morbidity and mortality, approaching 90%. Infections are the most common causes of both acute and chronic lung diseases leading to respiratory failure. Non-invasive diagnostic methods for evaluation are often of little value, and an invasive procedure (such as bronchoalveolar lavage, transbronchial biopsy or even open lung biopsy) is therefore performed to obtain a microbiologic and histologic diagnosis. Bronchoscopy allows certain identification of some aetiologies, and often allows the exclusion of infectious agents. Early use of computed tomography scanning is able to demonstrate lesions missed by conventional chest X-ray. However, even when a specific diagnosis is made, it might not impact patient's overall survival and outcomes.
Topics: Bronchoalveolar Lavage; Bronchoscopy; Humans; Immunocompromised Host; Lung Diseases; Pneumonia
PubMed: 35488835
DOI: 10.37201/req/s01.20.2022 -
The Journal of Allergy and Clinical... 2019Children with severe asthma have frequent exacerbations despite guidelines-based treatment with high-dose corticosteroids. The importance of refractory lung inflammation...
BACKGROUND
Children with severe asthma have frequent exacerbations despite guidelines-based treatment with high-dose corticosteroids. The importance of refractory lung inflammation and infectious species as factors contributing to poorly controlled asthma in children is poorly understood.
OBJECTIVE
To identify prevalent granulocyte patterns and potential pathogens as targets for revised treatment, 126 children with severe asthma underwent clinically indicated bronchoscopy.
METHODS
Diagnostic tests included bronchoalveolar lavage (BAL) for cell count and differential, bacterial and viral studies, spirometry, and measurements of blood eosinophils, total IgE, and allergen-specific IgE. Outcomes were compared among 4 BAL granulocyte patterns.
RESULTS
Pauci-granulocytic BAL was the most prevalent granulocyte category (52%), and children with pauci-granulocytic BAL had less postbronchodilator airflow limitation, less blood eosinophilia, and less detection of BAL enterovirus compared with children with mixed granulocytic BAL. Children with isolated neutrophilia BAL were differentiated by less blood eosinophilia than those with mixed granulocytic BAL, but greater prevalence of potential bacterial pathogens compared with those with pauci-granulocytic BAL. Children with isolated eosinophilia BAL had features similar to those with mixed granulocytic BAL. Children with mixed granulocytic BAL took more maintenance prednisone, and had greater blood eosinophilia and allergen sensitization compared with those with pauci-granulocytic BAL.
CONCLUSIONS
In children with severe, therapy-resistant asthma, BAL granulocyte patterns and infectious species are associated with novel phenotypic features that can inform pathway-specific revisions in treatment. In 32% of children evaluated, BAL revealed corticosteroid-refractory eosinophilic infiltration amenable to anti-T2 biological therapies, and in 12%, a treatable bacterial pathogen.
Topics: Adolescent; Anti-Asthmatic Agents; Asthma; Bronchoalveolar Lavage; Bronchoalveolar Lavage Fluid; Cell Count; Child; Drug Resistance; Eosinophilia; Eosinophils; Female; Humans; Male; Neutrophils; Phenotype; Spirometry
PubMed: 30654199
DOI: 10.1016/j.jaip.2018.12.027