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Respiration; International Review of... 2020Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects approximately 1 in 2,500 individuals globally [Ashizawa et al.: Neurol Clin Pract... (Review)
Review
PURPOSE OF REVIEW
Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects approximately 1 in 2,500 individuals globally [Ashizawa et al.: Neurol Clin Pract 2018;8(6):507-20]. In patients with DM1, respiratory muscle weakness frequently evolves, leading to respiratory failure as the main cause of death in this patient population, followed by cardiac complications [de Die-Smulders et al.: Brain 1998;121(Pt 8):1557-63], [Mathieu et al.: Neurology 1999;52(8):1658-62], [Groh et al.: Muscle Nerve 2011;43(5):648-51]. This paper provides a more detailed outline on the diagnostic and management protocols, which can guide pulmonologists who may not have experience with DM1 or who are not part of a neuromuscular multidisciplinary clinic. A group of neuromuscular experts in DM1 including pulmonologists, respiratory physiotherapists and sleep specialists discussed respiratory testing and management at baseline and during follow-up visits, based on their clinical experience with patients with DM1. The details are presented in this report.
RECENT FINDINGS
Myotonic recruited 66 international clinicians experienced in the treatment of people living with DM1 to develop and publish consensus-based care recommendations targeting all body systems affected by this disease [Ashizawa et al.: Neurol Clin Pract. 2018;8(6):507-20]. Myotonic then worked with 12 international respiratory therapists, pulmonologists and neurologists with long-standing experience in DM respiratory care to develop consensus-based care recommendations for pulmonologists using a methodology called the Single Text Procedure. This process generated a 7-page document that provides detailed respiratory care recommendations for the management of patients living with DM1. This consensus is completely based on expert opinion and not backed up by empirical evidence due to limited clinical care data available for respiratory care management in DM patients. Nevertheless, we believe it is of relevance for professionals treating adults with myotonic dystrophy because it addresses practical issues related to respiratory management and care, which have been adapted to meet the specific issues in patients with DM1.
SUMMARY
The resulting recommendations are intended to improve respiratory care for the most vulnerable of DM1 patients and lower the risk of untoward respiratory complications and mortality by providing pulmonologist who are less experienced with DM1 with practical indications on which tests and when to perform them, adapting the general respiratory knowledge to specific issues related to this multiorgan disease.
Topics: Consensus Development Conferences as Topic; Disorders of Excessive Somnolence; Humans; Hypoventilation; Myotonic Dystrophy; Noninvasive Ventilation; Physical Therapy Modalities; Practice Guidelines as Topic; Pulmonary Medicine; Respiration Disorders; Respiratory Function Tests; Respiratory Insufficiency; Respiratory Paralysis; Sleep Apnea, Obstructive
PubMed: 32299079
DOI: 10.1159/000505634 -
Equipment and procedural setup for interventional pulmonology procedures in the intensive care unit.Journal of Thoracic Disease Aug 2021Procedural setup is an important aspect of any procedure. Interventional pulmonologists provide a procedural practice and have additional expertise in performing... (Review)
Review
Procedural setup is an important aspect of any procedure. Interventional pulmonologists provide a procedural practice and have additional expertise in performing high-risk procedures needed in the critically ill patients in intensive care. Taking the time to plan the procedure setup in advance and having all necessary equipment readily available at the patient's bedside is imperative for procedural services. This is especially essential to ensure patient safety, minimize risk of complications, and improve success for specialized procedures performed by interventional pulmonary in the intensive care unit. In this review we describe the equipment and procedural setup ideal for both pleural and airway procedures. These include flexible diagnostic and therapeutic bronchoscopy, ultrasound guided thoracentesis, chest tube insertion, difficult airway management, and bedside percutaneous dilatation tracheostomy. We provide a guide checklist for these procedures emphasizing the practical aspects of each procedure from selecting the appropriate size endotracheal tube to operator positioning to ensure efficiency and best access. The components of procedural setup are discussed in relation to patient factors that include patient positioning and anesthesia, personnel in the procedure team and the equipment itself. We further briefly describe the additional equipment needed for specialized techniques in therapeutic bronchoscopy used by interventional pulmonologists.
PubMed: 34527369
DOI: 10.21037/jtd-20-3595 -
BMC Pulmonary Medicine Jan 2022The usefulness and safety of transbronchial lung cryobiopsy (TBLC) for reassessment of diffuse parenchymal lung disease (DPLD) with progression is still unknown. Our... (Comparative Study)
Comparative Study
BACKGROUND
The usefulness and safety of transbronchial lung cryobiopsy (TBLC) for reassessment of diffuse parenchymal lung disease (DPLD) with progression is still unknown. Our purpose was to clarify the usefulness and safety of TBLC for reassessment of DPLD with progression.
METHODS
This retrospective study included 31 patients with DPLD diagnosed by surgical lung biopsy who progressed in the clinical course and underwent TBLC for reassessment between January 2017 and September 2019 at Kanagawa Cardiovascular & Respiratory Center. Two pulmonologists independently selected the clinical diagnosis, treatment strategy, and confidence level of the treatment strategy based on clinical and radiological information with and without pathological information from TBLC. A consensus was reached among the pulmonologists regarding the clinical diagnosis, treatment strategy, and confidence level of the treatment strategy. Complications of TBLC were also examined.
RESULTS
Seven (22.6%), 5 (16.1%), and 6 (19.4%) of clinical diagnosis was changed after TBLC for Pulmonologist A, for Pulmonologist B, and for consensus, respectively. The treatment strategy was changed in 7 (22.6%), 8 (25.9%), and 6 (19.4%) cases after TBLC for Pulmonologist A, for Pulmonologist B and for consensus, respectively. The definite or high confidence level of the consensus treatment strategy was 54.8% (17/31) without TBLC and 83.9% (26/31) with TBLC. There were 6 cases of moderate bleeding, but no other complications were noted.
CONCLUSIONS
Pathological information from TBLC may contribute to decision-making in treatment strategies for the progression of DPLD, and it may be safely performed.
Topics: Adrenal Cortex Hormones; Aged; Aged, 80 and over; Biopsy; Bronchoscopy; Decision Making; Disease Progression; Female; Humans; Japan; Lung; Lung Diseases, Interstitial; Male; Middle Aged; Pulmonologists; Retrospective Studies
PubMed: 35086515
DOI: 10.1186/s12890-022-01838-x -
Annual Review of Medicine Jan 2024Interventional pulmonary medicine has developed as a subspecialty focused on the management of patients with complex thoracic disease. Leveraging minimally invasive... (Review)
Review
Interventional pulmonary medicine has developed as a subspecialty focused on the management of patients with complex thoracic disease. Leveraging minimally invasive techniques, interventional pulmonologists diagnose and treat pathologies that previously required more invasive options such as surgery. By mitigating procedural risk, interventional pulmonologists have extended the reach of care to a wider pool of vulnerable patients who require therapy. Endoscopic innovations, including endobronchial ultrasound and robotic and electromagnetic bronchoscopy, have enhanced the ability to perform diagnostic procedures on an ambulatory basis. Therapeutic procedures for patients with symptomatic airway disease, pleural disease, and severe emphysema have provided the ability to palliate symptoms. The combination of medical and procedural expertise has made interventional pulmonologists an integral part of comprehensive care teams for patients with oncologic, airway, and pleural needs. This review surveys key areas in which interventional pulmonologists have impacted the care of thoracic disease through bronchoscopic intervention.
Topics: Humans; Pulmonary Medicine; Bronchoscopy; Thoracic Diseases
PubMed: 37827195
DOI: 10.1146/annurev-med-050922-060929 -
Monaldi Archives For Chest Disease =... Jun 2018Pneumomediastinum is defined as the presence of air or gas within the mediastinum and it rarely complicates bronchoscopy. We report, to our best knowledge, the first...
Pneumomediastinum is defined as the presence of air or gas within the mediastinum and it rarely complicates bronchoscopy. We report, to our best knowledge, the first case of pneumomediastinum following a transbronchial cryobiopsy (TBLC). TBLC is considered a safe procedure as compared with both transbronchial biopsy and surgical lung biopsy. Systematic reviews, metanalysis and a Pubmed research, revealed that in literature no pneumomediastinum has been mentioned after TBLC. We report this case for to make it known to interventional pulmonologists the possibility that a pneumomediastinum can follow a TBLC. In our case the spontaneous resolution in few days did not require any intervention.
PubMed: 29929353
DOI: 10.4081/monaldi.2018.909 -
La Radiologia Medica Apr 2018To apply the Delphi exercise with iterative involvement of radiologists and pulmonologists with the aim of defining a structured reporting template for high-resolution...
OBJECTIVES
To apply the Delphi exercise with iterative involvement of radiologists and pulmonologists with the aim of defining a structured reporting template for high-resolution computed tomography (HRCT) of patients with fibrosing lung disease (FLD).
METHODS
The writing committee selected the HRCT criteria-the Delphi items-for rating from both radiology panelists (RP) and pulmonology panelists (PP). The Delphi items were first rated by RPs as "essential", "optional", or "not relevant". The items rated "essential" by < 80% of the RP were selected for the PP rating. The format of reporting was rated by both RP and PP.
RESULTS
A total of 42 RPs and 12 PPs participated to the survey. In both Delphi round 1 and 2, 10/27 (37.7%) items were rated "essential" by more than 80% of RP. The remaining 17/27 (63.3%) items were rated by the PP in round 3, with 2/17 items (11.7%) rated "essential" by the PP. PP proposed additional items for conclusion domain, which were rated by RPs in the fourth round. Poor consensus was observed for the format of reporting.
CONCLUSIONS
This study provides a template for structured report of FLD that features essential items as agreed by expert thoracic radiologists and pulmonologists.
Topics: Adult; Aged; Delphi Technique; Female; Humans; Male; Middle Aged; Models, Theoretical; Prospective Studies; Pulmonary Fibrosis; Pulmonary Medicine; Radiology; Research Report; Tomography, X-Ray Computed
PubMed: 29230680
DOI: 10.1007/s11547-017-0835-6 -
Jornal Brasileiro de Pneumologia :... Sep 2020
Topics: Diaphragm; Humans; Pulmonologists; Ultrasonography
PubMed: 32965297
DOI: 10.36416/1806-3756/e20200367 -
Respiratory Medicine Sep 2020While pulmonary nodule guidelines provide follow-up recommendations based on nodule size and malignancy risk, these are inconsistently followed in clinical practice. In...
BACKGROUND
While pulmonary nodule guidelines provide follow-up recommendations based on nodule size and malignancy risk, these are inconsistently followed in clinical practice. In this study, we sought to identify patient characteristics associated with guideline-concordant nodule follow-up.
METHODS
We conducted a retrospective cohort study of patients diagnosed with a pulmonary nodule between 2011 and 2014 at Boston Medical Center. Appropriate nodule follow-up evaluation was based upon the 2005 Fleischner Society Guidelines. In primary analysis, we compared patients with guideline-concordant follow-up to those with delayed or absent follow-up. In secondary analysis, we compared those with any follow-up to those without follow-up as well as the rate of guideline-concordant follow-up in patients seen by a pulmonologist.
RESULTS
Of 3916 patients diagnosed with a pulmonary nodule, 1117 were included for analysis. Overall, 598 (53.5%) patients received guideline-concordant follow-up. Lower rates of guideline concordance were seen in patients of Hispanic ethnicity (OR 0.60, 95% CI 0.36-1.00), while higher rates were seen for nodules 7-8 mm (OR 1.55, 95% CI 1.02-2.35) and nodules >8 mm (OR 1.49, 95% CI 1.01-2.20). Having a history of COPD (OR 1.75, 95% CI 1.26-2.43), and being seen by a pulmonologist (OR 1.97, 95% CI 1.51-2.58) were also associated with guideline concordance. Among patients seen by a pulmonologist, 62.2% received guideline-concordant follow-up.
CONCLUSION
Overall rates of pulmonary nodule follow-up are low. Patient ethnicity, COPD history, nodule size and involvement of a pulmonologist may impact follow-up rates and are potential targets for implementation interventions to improve pulmonary nodule follow-up.
Topics: Aftercare; Aged; Female; Humans; Male; Middle Aged; Patient Compliance; Practice Guidelines as Topic; Pulmonary Disease, Chronic Obstructive; Pulmonologists; Retrospective Studies; Solitary Pulmonary Nodule
PubMed: 32658836
DOI: 10.1016/j.rmed.2020.106075 -
European Clinical Respiratory Journal 2019The evaluation of patients with lung lesions is challenging. The nature of the lesion can be determined by pathological evaluation of biopsies. The pulmonologists will... (Review)
Review
The evaluation of patients with lung lesions is challenging. The nature of the lesion can be determined by pathological evaluation of biopsies. The pulmonologists will be met by increasing demands with regard to biopsy techniques including ultrasound-guided transthoracic needle biopsy (US-TTNB). The aim of this paper is to present the pulmonologist to a systematic step-by-step guide for performing US-TTNB and to assess the evidence for this approach. : Indications, contraindications and a step-by-step guide for the techniques used when performing US-TTNB are presented, and major complications and handling of these are described. : US-TTNB performed by pulmonologists is a safe and feasible procedure.
PubMed: 30815241
DOI: 10.1080/20018525.2019.1579632 -
Breathe (Sheffield, England) Dec 2020Extrapulmonary tuberculosis (EPT) can affect all organs. Its diagnosis is often challenging, especially when the lung is not involved. Some EPT locations, such as when... (Review)
Review
Extrapulmonary tuberculosis (EPT) can affect all organs. Its diagnosis is often challenging, especially when the lung is not involved. Some EPT locations, such as when the central nervous system is involved, are a medical emergency, and some have implications for treatment options and length. This review describes clinical features of EPT, diagnostic tests and treatment regimens.
PubMed: 33664835
DOI: 10.1183/20734735.0216-2020