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With the increasing age of patients with pulmonary arterial hypertension (PAH) at diagnosis, multiple comorbidities are more and more common in these patients, especially cardiopulmonary diseases. This article introduces the incidence of PAH combined with cardiovascular disease and/or chronic lung disease, elaborates on the impact of cardiopulmonary comorbidities on the diagnosis, prognosis, and targeted drug treatment strategies of PAH, and proposes management recommendations for PAH patients with comorbidities, aiming to further improve the diagnosis and treatment level of pulmonary hypertension.

As medical science has advanced, the incidence of central airway lesions, traditionally the primary focus of interventional pulmonology, has decreased significantly. However, the large population suffering from chronic airway diseases, coupled with unmet treatment needs and a high demand for new technologies and methods, has greatly driven the innovation and application of interventional respiratory techniques. The development and clinical application of techniques such as lung volume reduction, targeted lung denervation, pulsed electric field technology, bronchial thermoplasty, and regenerative medicine for chronic airway diseases are providing new treatment options. Chronic airway diseases are becoming a new frontier in interventional pulmonology.

There is a legal entitlement to participate in outpatient exercise groups in accordance with the German Social Code (Book IX) which regulates and facilitates prescriptions for patients with chronic respiratory diseases. A medical examination with specific inclusion and exclusion criteria prior to admission to an exercise group ensures safe participation.Traditional outpatient exercise groups are conducted in face-to-face groups for 60 to 90 minutes, once a week, with structured warm-up, main and cool-down phases. In addition, since the coronavirus pandemic, the introduction of online exercise groups via videoconferencing has enabled flexible participation (even without a prescription). To date, more than 11,000 German patients have participated in online exercise groups since 2021.Scientific evidence confirms the significant benefits of regular exercise, such as improved physical performance and reduced breathlessness. The psychosocial benefits and the promotion of self-efficacy are additionally supported by the supervision of a specialized trainer. Regular exercise (e.g. in outpatient exercise groups) is an inexpensive and very effective form of therapy to improve the quality of life of people with chronic respiratory diseases., Competing Interests: RG ist Vorstandsmitglied der AG Lungensport in Deutschland e.V.; IJ und RK haben keine Interessenskonflikte angegeben., (Thieme. All rights reserved.)

Pulmonary complications are very common after noncardiac surgery and can be easily overlooked. If not properly screened for or evaluated these can in many instances lead to postoperative respiratory failure or even death. Decisions regarding ambulatory versus inpatient surgery, modality of anesthesia, protective ventilation and method of weaning, type of analgesia, and postoperative monitoring can be crucial to avoid such complications., Competing Interests: Disclosures R. Kaw serves as consultant for Medtronic., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Diffuse alveolar hemorrhage (DAH) is a morbid syndrome that occurs after autologous and allogeneic hematopoietic cell transplantation in children and adults. DAH manifests most often in the first few weeks following transplantation. It presents with pneumonia-like symptoms and acute respiratory failure, often requiring high levels of oxygen supplementation or mechanical ventilatory support. Hemoptysis is variably present. Chest radiographs typically feature widespread alveolar filling, sometimes with peripheral sparing and pleural effusions. The diagnosis is suspected when serial bronchoalveolar lavages return increasingly bloody fluid. DAH is differentiated from infectious causes of alveolar hemorrhage when extensive microbiological testing reveals no pulmonary pathogens. The cause is poorly understood, though preclinical and clinical studies implicate pretransplant conditioning regimens, particularly those using high doses of total-body-irradiation, acute graft-versus-host disease (GVHD), medications used to prevent GVHD, and other factors. Treatment consists of supportive care, systemic corticosteroids, platelet transfusions, and sometimes includes antifibrinolytic drugs and topical procoagulant factors. Therapeutic blockade of tumor necrosis factor-α showed promise in observational studies, but its benefit for DAH remains uncertain after small clinical trials. Even with these treatments, mortality from progression and relapse is high. Future investigational therapies could target the vascular endothelial cell biology theorized to contribute to alveolar bleeding and pathways that contribute to susceptibility, inflammation, cellular resilience, and tissue repair. This review will help clinicians navigate through the limited evidence to diagnose and treat DAH, counsel patients and families, and plan for future research., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE., Methods: Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed., Results: 4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died., Conclusions: DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pediatric pulmonary critical care literature has continued to grow in recent years. Our aim in this review is to narrowly focus on publications providing clinically-relevant advances in pediatric pulmonary critical care in 2023., (© 2024 Wiley Periodicals LLC.)

Bakgrunn: Legeforeningen har utarbeidd retningslinjer for lindrande sedering i livets sluttfase, sist revidert i 2014. Vi ville undersøke lungelegar sin kjennskap til retningslinjene og bruk av lindrande sedering ved lungesjukdomar., Materiale Og Metode: Eit elektronisk spørjeskjema blei sendt til alle medlemmer av Norsk forening for lungemedisin (529 legar) i oktober 2022. Svara blei analysert med deskriptiv statistikk, og fritekstkommentarane blei analysert ved systematisk tekstkondensering., Resultat: Totalt 50 legar svarte (9,5 %). 22 hadde kjennskap til retningslinjene, og eit mindretal meinte desse var tydelege og gav god støtte. 37 oppgav at lindrande sedering blei gjennomført der dei jobba, men det var stor variasjon i kor ofte. 25 av deltakarane hadde vore med på å gjennomføre lindrande sedering ein eller fleire gonger, og 19 hadde opplevd etiske utfordringar relatert til dette. Sederinga vart oftast utført hjå pasientar med lungekreft ved intraktabel dyspné og smerter, men også hjå andre sjukdomsgrupper. Avgjerda vart stort sett tatt i tverrfagleg fellesskap, og fritekstkommentarane utdjupa at dette gav støtte og tryggleik. Det vart påpeikt ein krevjande grenseoppgang mot dødshjelp, og ønskje om at gråsonene kunne utdjupast meir i retningslinjene., Tolking: Definisjonen av lindrande sedering blir framleis oppfatta ulikt og kan framstå uklar. Det kan moglegvis vere til hjelp å legge til diagnosespesifikke råd ved revisjon av gjeldande retningslinjer.

Background: Long term oxygen therapy (LTOT) is prescribed for hypoxemia in pulmonary disease. Like other medical therapies, LTOT requires a prescription documenting the dosage (flow rate) and directions (at rest, with activity) which goes to a supplier. Communication with patients regarding oxygen prescription (flow rate, frequency, directions), monitoring (pulse oximetry) and dosage adjustment (oxygen titration) differs in comparison with medication prescriptions. We examined the communication of oxygen management plans in the electronic health record (EHR), and their consistency with patient-reported LTOT use., Study Design and Methods: A cross-sectional study was conducted in 71 adults with chronic lung disease on LTOT. Physician communication regarding oxygen management was obtained from the EHR. Participants were interviewed on their LTOT management plan. The information from each source was compared., Results: The study population was, on average, 64 years, two-thirds women, and most used oxygen for over 3 years. Only 45% of both at-rest and with-activity oxygen prescriptions were documented in the Electronic Health Record (EHR). Less than 20% of prescriptions were relayed to the patient in the after-visit summary. Of those with EHR-documented oxygen prescriptions, 44% of patients adhered to prescribed oxygen flow rates. Nearly all patients used a pulse oximeter (96%)., Interpretation: We identified significant gaps in communication of oxygen management plans from provider to patient. Even when the oxygen prescription was clearly documented, there were differences in patient-reported oxygen management. Critical gaps in oxygen therapy result from the lack of consistent documentation of oxygen prescriptions in the EHR and patient-facing documents. Addressing these issues systematically may improve home oxygen management., (© 2024. The Author(s).)

Chronic lung disease is the third leading cause of death globally, imposing huge burden of death, disability and healthcare costs. However, traditional pharmacotherapy has relatively limited effects in improving the cure rate and reducing the mortality of chronic lung disease. Thus, new treatments are urgently needed for the prevention and treatment of chronic lung disease. It is particularly noteworthy that, multiple aging-related phenotypes were involved in the occurrence and development of chronic lung disease, such as blocked proliferation, telomere attrition, mitochondrial dysfunction, epigenetic alterations, altered nutrient perception, stem cell exhaustion, chronic inflammation, etc. Consequently, senescent cells induce a series of pathological changes in the lung, such as immune dysfunction, airway remodeling, oxidative stress and regenerative dysfunction, which is a critical issue that needs special attention in chronic lung diseases. Therefore, anti-aging interventions may bring new insights into the treatment of chronic lung diseases. In this review, we elaborate the involvement of aging in chronic lung disease and further discuss the application and prospects of anti-aging therapy., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Treatment outcomes and long-term survival of non-tuberculous mycobacterial pulmonary disease (NTM-PD) in a real-world setting are difficult to assess, especially for species other than Mycobacterium avium complex (MAC).This was a retrospective cohort study on all Croatian residents with respiratory NTM isolates from 2006 to 2015, with follow-up to 2020.Therapy was started in 98/137 (71.5%) of patients, significantly more often in patients with fibrocavitary disease and/or sputum smear positivity. Unsuccessful treatment outcomes were recorded in 39/98 (39.8%) patients (14 deaths and 25 treatment failures). One-year and 5-year all-cause mortality were respectively 18.2% and 37.6%. Guideline-based treatment (GBT) was started in 50/98 (51%) of treated patients and followed for the recommended duration in 35.7% (35/98). This resulted in a higher chance of cure (OR 3.79, 95% CI 1.29 to 11.1; P = 0.012) than inadequately treated/untreated patients. For Mycobacterium xenopi disease, high cure rates (>80%) were achieved both with GBT and non-GBT treatment regimens.Guideline-based therapy resulted in a four-time higher chance of being cured. The impact of GBT on treatment outcomes was clear for MAC disease, but no apparent effect was observed for patients with M. xenopi disease..

Purpose of Review: Although lung transplantation stands as the gold standard curative therapy option for end-stage lung disease, the scarcity of available organs poses a significant challenge in meeting the escalating demand. This review provides an overview of recent advancements in ambulatory respiratory assist systems, selective anticoagulation therapies that target the intrinsic pathway, and innovative surface coatings to enable permanent respiratory support as a viable alternative to lung transplantation., Recent Findings: Several emerging ambulatory respiratory assist systems have shown promise in both preclinical and clinical trials. These systems aim to create more biocompatible, compact, and portable forms of extracorporeal membrane oxygenation that can provide long-term respiratory support. Additionally, innovative selective anticoagulation strategies, currently in various stages of preclinical or clinical development, present a promising alternative to currently utilized nonselective anticoagulants. Moreover, novel surface coatings hold the potential to locally prevent artificial surface-induced thrombosis and minimize bleeding risks., Summary: This review of recent advancements toward permanent respiratory support summarizes the development of ambulatory respiratory assist systems, selective anticoagulation therapies, and novel surface coatings. The integration of these evolving device technologies with targeted anticoagulation strategies may allow a safe and effective mode of permanent respiratory support for patients with chronic lung disease., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

The field of pediatric rare and diffuse lung disease continues its maturation as research advances the understanding of diagnosis and treatment of children's interstitial lung disease, noncystic fibrosis bronchiectasis, and primary ciliary dyskinesia. The rarity and breadth of these conditions make them challenging to study, yet we continue to make progress in our understanding of pathophysiology, genotype/phenotype relationships, and treatment. Papers published on these topics in Pediatric Pulmonology and other journals in 2023 describe the power of multicenter cooperation and patient registries, enhance our understanding of pathophysiology and genotype/phenotype relationships, and report progress in treatments. In this review, we hope to increase awareness and knowledge of these conditions and to inspire future research., (© 2024 The Author(s). Pediatric Pulmonology published by Wiley Periodicals LLC.)

Chronic lung diseases are debilitating illnesses ranking among the top causes of death globally. Currently, clinically available therapeutic options capable of curing chronic lung diseases are limited to lung transplantation, which is hindered by donor organ shortage. This highlights the urgent need for alternative strategies to repair damaged lung tissues. Stem cell transplantation has emerged as a promising avenue for regenerative treatment of the lung, which involves delivery of healthy lung epithelial progenitor cells that subsequently engraft in the injured tissue and further differentiate to reconstitute the functional respiratory epithelium. These transplanted progenitor cells possess the remarkable ability to self-renew, thereby offering the potential for sustained long-term treatment effects. Notably, the transplantation of basal cells, the airway stem cells, holds the promise for rehabilitating airway injuries resulting from environmental factors or genetic conditions such as cystic fibrosis. Similarly, for diseases affecting the alveoli, alveolar type II cells have garnered interest as a viable alveolar stem cell source for restoring the lung parenchyma from genetic or environmentally induced dysfunctions. Expanding upon these advancements, the use of induced pluripotent stem cells to derive lung progenitor cells for transplantation offers advantages such as scalability and patient specificity. In this review, we comprehensively explore the progress made in lung stem cell transplantation, providing insights into the current state of the field and its future prospects., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

It is increasingly recognised that diverse genetic respiratory disorders present as severe pulmonary hypertension (PH) in the neonate and young infant, but many controversies and uncertainties persist regarding optimal strategies for diagnosis and management to maximise long-term outcomes. To better define the nature of PH in the setting of developmental lung disease (DEVLD), in addition to the common diagnoses of bronchopulmonary dysplasia and congenital diaphragmatic hernia, we established a multidisciplinary group of expert clinicians from stakeholder paediatric specialties to highlight current challenges and recommendations for clinical approaches, as well as counselling and support of families. In this review, we characterise clinical features of infants with DEVLD/DEVLD-PH and identify decision-making challenges including genetic evaluations, the role of lung biopsies, the use of imaging modalities and treatment approaches. The importance of working with team members from multiple disciplines, enhancing communication and providing sufficient counselling services for families is emphasised to create an interdisciplinary consensus., Competing Interests: Conflict of interest: E.D. Austin reports grants from the NIH and a leadership role with TBX4Life. C. Galambos reports leadership roles with PPHNet and TBX4Life. D. Yung reports grants from Merck, Janssen and the NIH. S.O. Vargas reports grants from the Chan Zuckerberg Initiative, consultancy fees from Vertex Pharmaceuticals, lecture fees from the American Academy of Allergy, Asthma & Immunology, participation on a data safety monitoring board or advisory board with Millipore Sigma, and a leadership role with the Society for Pediatric Pathology. E.O. Jackson reports support for attending meetings from Seattle Children's Hospital. E.C. Whalen reports consultancy fees from the Pulmonary Hypertension Association Care Center and a leadership role with PPHNet. N.M. Villafranco reports support for attending meetings from the Children's Hospital of Philadelphia. S.H. Abman reports grants from the NHLBI (U01 HL12118), consultancy fees from Chiesi, and participation on a data safety monitoring board or advisory board with Bayer Pharmaceuticals and the NHLBI. The remaining authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2024.)

Pulmonary mucociliary clearance (MCC) is an important defence mechanism of the respiratory system and clears pathogens and foreign particles from the airways. Understanding the effect of disease states, drugs, toxins and airway manipulations on MCC could be beneficial in preventing early pulmonary disease and developing new pulmonary therapeutics. This review summarises the current methods and future efforts to detect pulmonary MCC in vivo ., Competing Interests: Conflict of interest: All authors have nothing to disclose., (Copyright ©The authors 2024.)

The emerging field of gut-lung axis research has revealed a complex interplay between the gut microbiota and respiratory health, particularly in asthma. This review comprehensively explored the intricate relationship between these two systems, focusing on their influence on immune responses, inflammation, and the pathogenesis of respiratory diseases. Recent studies have demonstrated that gut microbiota dysbiosis can contribute to asthma onset and exacerbation, prompting investigations into therapeutic strategies to correct this imbalance. Probiotics and prebiotics, known for their ability to modulate gut microbial compositions, were discussed as potential interventions to restore immune homeostasis. The impact of antibiotics and metabolites, including short-chain fatty acids produced by the gut microbiota, on immune regulation was examined. Fecal microbiota transplantation has shown promise in various diseases, but its role in respiratory disorders is not established. Innovative approaches, including mucus transplants, inhaled probiotics, and microencapsulation strategies, have been proposed as novel therapeutic avenues. Despite challenges, including the sophisticated adaptability of microbial communities and the need for mechanistic clarity, the potential for microbiota-based interventions is considerable. Collaboration between researchers, clinicians, and other experts is essential to unravel the complexities of the gut-lung axis, paving a way for innovative strategies that could transform the management of respiratory diseases.

Continued improvements in the treatment of pulmonary infections have paradoxically resulted in a growing challenge of individuals with postinfectious pulmonary complications (PIPCs). PIPCs have been long recognized after tuberculosis, but recent experiences such as the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic have underscored the importance of PIPCs following other lower respiratory tract infections. Independent of the causative pathogen, most available studies of pulmonary infections focus on short-term outcomes rather than long-term morbidity among survivors. In this document, we establish a conceptual scope for PIPCs with discussion of globally significant pulmonary pathogens and an examination of how these pathogens can damage different components of the lung, resulting in a spectrum of PIPCs. We also review potential mechanisms for the transition from acute infection to PIPC, including the interplay between pathogen-mediated injury and aberrant host responses, which together result in PIPCs. Finally, we identify cross-cutting research priorities for the field to facilitate future studies to establish the incidence of PIPCs, define common mechanisms, identify therapeutic strategies, and ultimately reduce the burden of morbidity in survivors of pulmonary infections.

Children's interstitial and diffuse lung diseases (chILDs) are a heterogenous and diverse group of lung disorders presenting during childhood. Infants and children with chILD disorders present with respiratory signs and symptoms as well as diffuse lung imaging abnormalities. ChILD disorders are associated with significant health care resource utilization and high morbidity and mortality. The care of patients with chILD has been improved through multidisciplinary care, multicenter collaboration, and the establishment of patient research networks in the United Stated and abroad. This review details past and current innovations in the diagnosis and clinical care of children with chILD., Competing Interests: Disclosure Dr A. Casey has no disclosures. Drs E.K. Fiorino and J. Wambach developed continuing medical education in childhood interstitial lung disease for Simumetrix. Support: National Institutes of Health, United States (R01-149853- JAW), Washington University School of Medicine, United States/St. Louis Children’s Hospital Children’s Discovery Institute (JAW)., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Newer medications and devices, as well as greater understanding of the benefits and limitations of existing treatments, have led to expanded treatment options for patients with lung disease. Treatment advances have led to improved outcomes for patients with asthma, chronic obstructive pulmonary disease, interstitial lung disease, pulmonary hypertension, and cystic fibrosis. The risks and benefits of available treatments are substantially variable within these heterogeneous disease groups. Defining the role of newer therapies mandates both an understanding of these disorders and overall treatment approaches. This section will review general treatment approaches in addition to focusing on newer therapies for these conditions.., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Repair and regeneration of a diseased lung using stem cells or bioengineered tissues is an exciting therapeutic approach for a variety of lung diseases and critical illnesses. Over the past decade, increasing evidence from preclinical models suggests that mesenchymal stromal cells, which are not normally resident in the lung, can be used to modulate immune responses after injury, but there have been challenges in translating these promising findings to the clinic. In parallel, there has been a surge in bioengineering studies investigating the use of artificial and acellular lung matrices as scaffolds for three-dimensional lung or airway regeneration, with some recent attempts of transplantation in large animal models. The combination of these studies with those involving stem cells, induced pluripotent stem cell derivatives, and/or cell therapies is a promising and rapidly developing research area. These studies have been further paralleled by significant increases in our understanding of the molecular and cellular events by which endogenous lung stem and/or progenitor cells arise during lung development and participate in normal and pathological remodeling after lung injury. For the 2023 Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Diseases Conference, scientific symposia were chosen to reflect the most cutting-edge advances in these fields. Sessions focused on the integration of "omics" technologies with function, the influence of immune cells on regeneration, and the role of the extracellular matrix in regeneration. The necessity for basic science studies to enhance fundamental understanding of lung regeneration and to design innovative translational studies was reinforced throughout the conference.

Advanced diagnostic and therapeutic flexible bronchoscopy in children is a rapidly evolving field. Recent advances in technology and awareness of indications, risks, and benefits by pediatric providers have greatly increased the use of advanced techniques in children. This review highlights advanced diagnostic procedures including assessment of endobronchial lesions, mediastinal/hilar masses, and peripheral lung nodules as well as therapeutic techniques for restoring airway lumen patency, managing persistent air leaks, and treating tracheoesophageal fistulas in children as well as the potential to spare these patients more invasive procedures., Competing Interests: Disclosure The authors have no conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Sickle cell lung disease presents a challenging care paradigm involving acute and chronic lower airway disease, sleep-disordered breathing, pulmonary vascular disease, and modification by environmental factors. Understanding the presentation, pathophysiology, and diagnostic approaches is essential for accurate identification and management. While significant progress has been made, there remains a need for research to develop effective treatments and interventions to decrease disease burden in these children. Additionally, the long-term impact of interventions on cardiopulmonary outcomes is unknown. Collaborative efforts among health care providers, researchers, advocacy groups, and policy makers are crucial to improving the lives of children with SCD., Competing Interests: Disclosure The authors have no relevant disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Gender-specific differences in the diagnostics and treatment must be considered for various lung diseases. In the case of pneumothorax, in addition to differences in etiology there are also relevant differences in treatment and recurrence rates between men and women. For example, to achieve low recurrence rates catamenial pneumothorax requires interdisciplinary collaboration with gynecology. The incidence of lung cancer has equalized in recent years and in addition, various gender-specific prognostic factors have become relevant. Several meta-analyses have identified female gender as a positive prognostic factor for lung cancer, in addition to the higher prevalence of various driver mutations in women. In current trials of multimodal treatment for lung cancer, gender differences in tolerability and patient outcome are already apparent. In subgroup analyses better event-free survival was observed in women, although immune-mediated adverse events were more common in women., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that affects exocrine glands, mainly the salivary and lacrimal glands, leading to the development of sicca symptoms. Patients with pSS may also present with extraglandular manifestations, including lung involvement, estimated to occur in 9-24% of cases. Shrinking lung syndrome (SLS) is an uncommon respiratory complication primarily associated with systemic lupus erythematosus, with a prevalence of approximately 1% in these patients. It typically manifests as dyspnea, pleuritic chest pain, lung volume reduction, and a restrictive pattern on respiratory function tests. Cases reporting SLS with other connective tissue diseases, including pSS, are even rarer. Herein, we describe a case of a 57-year-old woman with a 10-year history of pSS who presented with dyspnea and pleuritic chest pain. After evaluation, the patient was diagnosed with SLS based on clinical, radiologic, laboratorial, and electrophysiologic characteristics. In addition, we identified and analyzed previously published cases of SLS in pSS. Treatment includes corticosteroids, immunosuppressants, and respiratory muscle training. This study highlights the importance of considering SLS in the differential diagnosis of patients with pSS and respiratory symptoms., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The endothelium is a cell monolayer that lines vessels and separates tissues from blood flow. Endothelial cells (ECs) have a multitude of functions, including regulating blood flow and systemic perfusion through changes in vessel diameter. When an injury occurs, the endothelium is affected by altering its functions and structure, which leads to endothelial dysfunction, a characteristic of many vascular diseases. Understanding the role that the endothelium plays in pulmonary vascular and cardiopulmonary diseases, and exploring new therapeutic strategies is of utmost importance to advance clinically. Currently, there are several treatments able to improve patients' quality of life, however, none are effective nor curative. This review examines the critical role of the endothelium in the pulmonary vasculature, investigating the alterations that occur in ECs and their consequences for blood vessels and potential molecular targets to regulate its alterations. Additionally, we delve into promising non-pharmacological therapeutic strategies, such as exercise and diet. The significance of the endothelium in cardiopulmonary disorders is increasingly being recognized, making ECs a relevant target for novel therapies aimed at preserving their functional and structural integrity.

Background: Currently, there is conflicting information and guidance on the effective management of Alpha 1 Antitrypsin Deficiency (AATD). Establishing a consensus of assessment and disease management specific to AATD is important for achieving a standardized treatment pathway and for improving patient outcomes. Here, we aim to utilize the Delphi method to establish a European consensus for the assessment and management of patients with severe AATD., Methods: Two rounds of a Delphi survey were completed online by members of the European Alpha-1 Research Collaboration (EARCO). Respondents were asked to indicate their agreement with proposed statements for patients with no respiratory symptoms, stable respiratory disease, and worsening respiratory disease using a Likert scale of 1-7. Levels of agreement between respondents were calculated using a weighted average., Results: Round 1 of the Delphi survey was sent to 103 members of EARCO and 38/103 (36.9%) pulmonologists from across 15 countries completed all 109 questions. Round 2 was sent to all who completed Round 1 and 36/38 (94.7%) completed all 79 questions. Responses regarding spirometry, body plethysmography, high-resolution computed tomography, and the initiation of augmentation therapy showed little variability among physicians, but there was discordance among other aspects, such as the use of low-dose computed tomography in both a research setting and routine clinical care., Conclusions: These results provide expert opinions for the assessment and monitoring of patients with severe AATD, which could be used to provide updated recommendations and standardized treatment pathways for patients across Europe., (© 2024. The Author(s).)

The SWI/SNF complex, also known as the BRG1/BRM-associated factor (BAF) complex, represents a critical regulator of chromatin remodeling mechanisms in mammals. It is alternatively referred to as mSWI/SNF and has been suggested to be imbalanced in human disease compared with human health. Three types of BAF assemblies associated with it have been described, including (1) canonical BAF (cBAF), (2) polybromo-associated BAF (PBAF), and (3) non-canonical BAF (ncBAF) complexes. Each of these BAF assemblies plays a role, either functional or dysfunctional, in governing gene expression patterns, cellular processes, epigenetic mechanisms, and biological processes. Recent evidence increasingly links the dysregulation of mSWI/SNF complexes to various human non-malignant lung chronic disorders and lung malignant diseases. This review aims to provide a comprehensive general state-of-the-art and a profound examination of the current understanding of mSWI/SNF assembly processes, as well as the structural and functional organization of mSWI/SNF complexes and their subunits. In addition, it explores their intricate functional connections with potentially dysregulated transcription factors, placing particular emphasis on molecular and cellular pathogenic processes in lung diseases. These processes are reflected in human epigenome aberrations that impact clinical and therapeutic levels, suggesting novel perspectives on the diagnosis and molecular therapies for human respiratory diseases., Competing Interests: Declaration of interests The authors declare no competing interests., (Copyright © 2024 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.)

Community-based palliative care (CBPC) models address the growing needs of patients and caregivers with chronic and serious illnesses. From pediatrics to geriatrics, individuals prefer to receive care within their local community and at home. Delivering care at the community level and within the home improves health outcomes, reduces disparities, and supports local economic activity. Various models of CBPC have developed through partnerships with existing services, such as home health agencies, but ongoing challenges and barriers exist for further expansion of specialty palliative care. Advanced practice registered nurses increasingly manage chronic and serious illnesses and are essential for all health care teams, particularly for CBPC. As trusted providers within the community, advanced practice registered nurses create lasting relationships that allow for meaningful exchanges with patients and caregivers. This article reviewed the concept and features of CBPC offered within the United States and describes an advanced practice registered nurse-led embedded palliative care program for advanced lung disease., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 by The Hospice and Palliative Nurses Association. All rights reserved.)

Patients with Mycobacterium avium complex pulmonary disease (MAC-PD) often suffer from chronic symptoms such as sputum production, which reduces quality of life. Oscillatory positive expiratory pressure (OPEP) devices are used in physiotherapy to promote the clearance of respiratory secretions. We report two cases of improved lung function and improved scores on the Leicester Cough Questionnaire (LCQ) and the Breathlessness, Cough and Sputum Scale (BCSS) after the use of OPEP in patients with MAC-PD where treatment with guideline-based therapy, including amikacin liposome inhalation suspension, had proved ineffective for symptoms. Use of OPEP might maximize the efficacy of therapy and thereby improves outcomes in patients with MAC-PD. It is important to use both guideline-based therapy and OPEP, especially in patients whose health-related quality of life is affected by sputum symptoms. Further prospective studies are warranted to assess the benefit of adding OPEP to guidelines concerning therapy for patients with MAC-PD and sputum symptoms., Competing Interests: Declarations of competing interest None., (Copyright © 2024 Japanese Society of Chemotherapy, Japanese Association for Infectious Diseases, and Japanese Society for Infection Prevention and Control. Published by Elsevier Ltd. All rights reserved.)

While the incidence and prevalence of non-tuberculous mycobacterial-pulmonary disease (NTM-PD) are increasing and microscopic polyangiitis (MPA) is common in East Asian countries, case reports of MPA associated with NTM-PD are limited. A 72-year-old male receiving treatment for NTM-PD with antibiotics was referred to our hospital with fever and arthralgia that developed a few months previously. A blood test revealed the presence of the myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) and renal impairment. Based on a pathological examination of renal tissue, which showed crescentic glomerulonephritis, the patient was diagnosed with MPA. Due to acute kidney injury and strongly positive MPO-ANCA, pulse steroid therapy was initiated followed by intravenous rituximab (RTX). The patient also received plasmapheresis (14 sessions). Renal dysfunction was reversed. MPA associated with NTM-PD is extremely rare and, thus, there is currently no established treatment. Our patient was diagnosed with MPA based on the findings of renal biopsy while receiving treatment for NTM-PD. RTX and plasmapheresis combined with systemic glucocorticoid therapy were initiated before these clinical conditions had fully recovered. Although MPA secondary to NTM-PD may be more refractory to treatment than primary MPA in the presence of a very low interferon-gamma (IFN-γ) level, this case was successfully treated with steroids, RTX, and plasmapheresis., (© 2023. The Author(s), under exclusive licence to Japanese Society of Nephrology.)

Introduction: The use of ultrasound in respiratory disease has evolved substantially over the past two decades. From a test done to confirm the safe site of pleural fluid drainage, thoracic ultrasound has become a point-of-care test that guides the management of patients on respiratory wards, in clinics and endoscopy., Areas Covered: This review overviews the process of ultrasound examination in the chest. It then delves into specific disease areas (pleural disease, lung disease, diaphragm disease, and invasive procedures) to highlight how thoracic ultrasound is being used to refine management. The review concludes with discussion on the training curricula and assessment tools for competency in thoracic ultrasound. Being a scoping review, literature searches were conducted on PubMed using relevant search terms., Expert Opinion: In addition to its current uses, there are many avenues where thoracic ultrasound will soon be beneficial. Recent studies show promising roles in areas such as patient-tailored guidance of pleurodesis and non-invasively predicting lung re-expansion after pleural fluid drainage. In addition, auxiliary tools such as contrast-enhanced ultrasound and elastography are proving useful in identifying the etiology and directing the successful sampling of pleural and lung lesions. Studies are also exploring the utility of sonographic biomarkers such as echogenicity and septations to predict outcomes in pleural disease.

Gene delivery therapy has emerged as a popular approach for the treatment of various diseases. However, it still poses the challenges of accumulation in target sites and reducing off-target effects. Aerosol gene delivery for the treatment of pulmonary diseases has the advantages of high lung accumulation, specific targeting and fewer systemic side effects. However, the key challenge is selecting the appropriate formulation for aerosol gene delivery that can overcome physiological barriers. There are numerous existing gene carriers under study, including viral vectors and non-viral vectors. With the development of biomaterials, more biocompatible substances have applied gene delivery via inhalation. Furthermore, many types of genes can be delivered through aerosol inhalation, such as DNA, mRNA, siRNA and CRISPR/Cas9. Aerosol delivery of different types of genes has proven to be efficient in the treatment of many diseases such as SARS-CoV-2, cystic fibrosis and lung cancer. In this paper, we provide a comprehensive review of the ongoing research on aerosol gene delivery therapy, including the basic respiratory system, different types of gene carriers, different types of carried genes and clinical applications.

Congenital tracheo-esophageal fistula/esophageal atresia (TEF/EA) with concomitant pulmonary agenesis is exceedingly rare and has a high mortality rate. While there are several reported cases of successful repair, all but one patient had right-sided pulmonary agenesis. In the case of left-sided pulmonary agenesis, the patient had incomplete agenesis and underwent repair through a left thoracotomy. We present the first successful repair of TEF/EA with complete left-sided pulmonary agenesis. This patient also underwent elective pre-operative veno-venous extracorporeal membrane oxygenation (ECMO) and subsequent repair of the TEF/EA. We discuss the management, anesthesia risks, and role of periprocedural ECMO in pediatric patients who are high anesthetic risk., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Lung transplantation is a lifesaving intervention for people with advanced lung disease, but it is costly and resource-intensive. To investigate the cost-effectiveness of lung transplantation as a treatment option in pulmonary disease, we must understand costs attributable to end-of-life hospitalizations for end-stage lung disease., Research Question: What are the costs associated with end-of-life hospitalizations for people with pulmonary disease, and how have these trends changed over time?, Study Design and Methods: Adults aged 18 to 74 years with hospitalization data in the Cost and Utilization Project National Inpatient Sample data from 2009 to 2019 with a pulmonary disease admission were included in this analysis. Those with a history of lung transplantation were excluded. International Classification of Diseases codes were used to identify pulmonary disease admissions, complications, and procedures and interventions. Total charges were calculated for hospitalizations and stratified by patient status at time of discharge. Trends in charges over time were assessed by demographic and hospital factors., Results: One hundred nine thousand nine hundred twenty-four (4.1%) hospital admissions for pulmonary disease resulted in in-hospital mortality. Those with obstructive lung disease accounted for 94.1% of hospitalizations and 88.1% cases of in-hospital mortality. Estimated costs for end-of-life hospitalizations were $29,981 on average with wide variation in cost by diagnosis and procedure utilization. Inpatient costs were highest for younger people who received more procedures. Among the most expensive admissions, mechanical ventilation accounted for the greatest proportion of interventions. Significant increases in the use of mechanical ventilation, extracorporeal membrane oxygenation, and dialysis occurred over the time period. The rate of hospital transfers increased with a proportionately greater increase across admissions resulting in in-hospital mortality., Interpretation: Costs accrued during end-of-life hospitalizations vary across people but represent a significant health care cost that can be averted for selected people who undergo lung transplantation. These costs should be considered in studies of cost-effectiveness in lung transplantation., Competing Interests: Financial/Nonfinancial Disclosures None declared., (Published by Elsevier Inc.)

The use of nanomaterials in gene editing and synthetic biology has emerged as a pivotal strategy in the pursuit of refined treatment methodologies for pulmonary disorders. This review discusses the utilization of nanomaterial-assisted gene editing tools and synthetic biology techniques to promote the development of more precise and efficient treatments for pulmonary diseases. First, we briefly outline the characterization of the respiratory system and succinctly describe the principal applications of diverse nanomaterials in lung ailment treatment. Second, we elaborate on gene-editing tools, their configurations, and assorted delivery methods, while delving into the present state of nanomaterial-facilitated gene-editing interventions for a spectrum of pulmonary diseases. Subsequently, we briefly expound on synthetic biology and its deployment in biomedicine, focusing on research advances in the diagnosis and treatment of pulmonary conditions against the backdrop of the coronavirus disease 2019 pandemic. Finally, we summarize the extant lacunae in current research and delineate prospects for advancement in this domain. This holistic approach augments the development of pioneering solutions in lung disease treatment, thereby endowing patients with more efficacious and personalized therapeutic alternatives., (© 2024. The Author(s).)

The lung is regarded as having limited regenerative capacity, and there are few treatment options for refractory lung diseases, such as interstitial pneumonia. Lung transplantation is the final option available in some scenarios. Research in this area has been slow owing to the complex structure of the lung for efficient gas exchange between the alveolar spaces and capillaries as well as the difficulty in obtaining specimens from patients with progressive lung disease. However, basic research over the past decade in the field of mouse and human embryology using genetic lineage tracing techniques and stem cell biology using primary and pluripotent stem cell-derived alveolar organoids has begun to clarify the tissue response in various intractable lung diseases and the mechanisms underlying remodeling. Advancement in this area may expand potential therapeutic targets for alveolar regeneration, providing alternatives to lung transplantation, and contribute to the development of effective therapeutic methods that activate or repopulate stem cells in the lung. In this review, we cover research focused on alveolar epithelial cells and discuss methods expected to regenerate lungs that are damaged by diseases., (© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Adjustment for race is discouraged in lung-function testing, but the implications of adopting race-neutral equations have not been comprehensively quantified., Methods: We obtained longitudinal data from 369,077 participants in the National Health and Nutrition Examination Survey, U.K. Biobank, the Multi-Ethnic Study of Atherosclerosis, and the Organ Procurement and Transplantation Network. Using these data, we compared the race-based 2012 Global Lung Function Initiative (GLI-2012) equations with race-neutral equations introduced in 2022 (GLI-Global). Evaluated outcomes included national projections of clinical, occupational, and financial reclassifications; individual lung-allocation scores for transplantation priority; and concordance statistics (C statistics) for clinical prediction tasks., Results: Among the 249 million persons in the United States between 6 and 79 years of age who are able to produce high-quality spirometric results, the use of GLI-Global equations may reclassify ventilatory impairment for 12.5 million persons, medical impairment ratings for 8.16 million, occupational eligibility for 2.28 million, grading of chronic obstructive pulmonary disease for 2.05 million, and military disability compensation for 413,000. These potential changes differed according to race; for example, classifications of nonobstructive ventilatory impairment may change dramatically, increasing 141% (95% confidence interval [CI], 113 to 169) among Black persons and decreasing 69% (95% CI, 63 to 74) among White persons. Annual disability payments may increase by more than $1 billion among Black veterans and decrease by $0.5 billion among White veterans. GLI-2012 and GLI-Global equations had similar discriminative accuracy with regard to respiratory symptoms, health care utilization, new-onset disease, death from any cause, death related to respiratory disease, and death among persons on a transplant waiting list, with differences in C statistics ranging from -0.008 to 0.011., Conclusions: The use of race-based and race-neutral equations generated similarly accurate predictions of respiratory outcomes but assigned different disease classifications, occupational eligibility, and disability compensation for millions of persons, with effects diverging according to race. (Funded by the National Heart Lung and Blood Institute and the National Institute of Environmental Health Sciences.)., (Copyright © 2024 Massachusetts Medical Society.)

Background: Mesenchymal stem cells (MSCs) have undergone extensive investigation for their potential therapeutic applications, primarily attributed to their paracrine activity. Recently, researchers have been exploring the therapeutic potential of extracellular vesicles (EVs) released by MSCs., Methods: MEDLINE/PubMed and Google scholar databases were used for the selection of literature. The keywords used were mesenchymal stem cells, extracellular vesicles, clinical application of EVs and challenges EVs production., Results: These EVs have demonstrated robust capabilities in transporting intracellular cargo, playing a critical role in facilitating cell-to-cell communication by carrying functional molecules, including proteins, RNA species, DNAs, and lipids. Utilizing EVs as an alternative to stem cells offers several benefits, such as improved safety, reduced immunogenicity, and the ability to traverse biological barriers. Consequently, EVs have emerged as an increasingly attractive option for clinical use., Conclusion: From this perspective, this review delves into the advantages and challenges associated with employing MSC-EVs in clinical settings, with a specific focus on their potential in treating conditions like lung diseases, cancer, and autoimmune disorders., (© 2024. Korean Tissue Engineering and Regenerative Medicine Society.)

Systemic Lupus Erythematosus (SLE) is a multifaceted, multisystem autoimmune disorder with diverse clinical expressions. While prevalence reports vary widely, pulmonary involvement accounts for significant morbidity and mortality in SLE. This comprehensive review explores the spectrum of pulmonary disease in SLE, including upper airway manifestations (e.g., laryngeal affection), lower airway conditions (e.g., bronchitis, bronchiolitis, bronchiectasis), parenchymal diseases (e.g., interstitial lung disease, acute lupus pneumonitis, diffuse alveolar hemorrhage), pleural diseases (e.g., serositis, shrinking lung syndrome), and vascular diseases (e.g., pulmonary arterial hypertension, pulmonary embolism, acute reversible hypoxemia syndrome). We discuss diagnostic modalities, treatment strategies, and prognosis for each pulmonary manifestation. With diagnostics remaining a challenge and with the absence of standardized treatment guidelines, we emphasize the need for evidence-based guidelines to optimize patient care and improve outcomes in this complex disease., Competing Interests: None declared., (Thieme. All rights reserved.)

Performance: Congenital pulmonary malformations (CPM) are rare and can be associated with high morbidity. Clinical presentation, diagnostic procedures, imaging, and therapy of CPM are discussed., Achievements: Today, most CPM can be diagnosed prenatally by ultrasound. Postnatally, respiratory symptoms up to respiratory failure and recurrent lower respiratory tract infection are typical findings. Due to low diagnostic accuracy of chest x‑ray in CPM, all children with prenatal diagnosis of CPM or postnatally suspected CPM should undergo cross-sectional imaging., Practical Recommendations: Based on imaging alone, the various subtypes of CPM cannot be definitively differentiated, which is why histological confirmation remains the gold standard. Surgical resection is the standard of care with minimally invasive procedures increasingly being employed. In certain situations, a watch-and-wait approach is possible., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Rationale: Diffuse alveolar hemorrhage (DAH) is a life-threatening manifestation of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). The PEXIVAS (Plasma Exchange and Glucocorticoids in Severe Antineutrophil Cytoplasmic Antibody-Associated Vasculitis) (NCT00987389) trial was the largest in AAV and the first to enroll participants with DAH requiring mechanical ventilation. Objectives: Evaluate characteristics, treatment effects, and outcomes for patients with AAV with and without DAH. Methods: PEXIVAS randomized 704 participants to plasma exchange (PLEX) or no-PLEX and reduced or standard-dose glucocorticoids (GC). DAH status was defined at enrollment as no-DAH, nonsevere, or severe (room air oxygen saturation of ⩽ 85% as measured by pulse oximetry, or use of mechanical ventilation). Measurements and Main Results: At enrollment, 191 (27.1%) participants had DAH (61 severe, including 29 ventilated) and were younger, more frequently relapsing, PR3 (proteinase 3)-ANCA positive, and had lower serum creatinine but were more frequently dialyzed than participants without DAH ( n  = 513; 72.9%). Among those with DAH, 8/95 (8.4%) receiving PLEX died within 1 year versus 15/96 (15.6%) with no-PLEX (hazard ratio, 0.52; confidence interval [CI], 0.21-1.24), whereas 13/96 (13.5%) receiving reduced GC died versus 10/95 (10.5%) with standard GC (hazard ratio, 1.33; CI, 0.57-3.13). When ventilated, ventilator-free days were similar with PLEX versus no-PLEX (medians, 25; interquartile range [IQR], 22-26 vs. 22-27) and fewer with reduced GC (median, 23; IQR, 20-25) versus standard GC (median, 26; IQR, 25-28). Treatment effects on mortality did not vary by presence or severity of DAH. Overall, 23/191 (12.0%) with DAH died within 1 year versus 34/513 (6.6%) without DAH. End-stage kidney disease and serious infections did not differ by DAH status or treatments. Conclusions: Patients with AAV and DAH differ from those without DAH in multiple ways. Further data are required to confirm or refute a benefit of PLEX or GC dosing on mortality. Original clinical trial registered with www.clinicaltrials.gov (NCT00987389).

Pulmonary delivery of therapeutic agents has been considered the desirable administration route for local lung disease treatment. As the latest generation of therapeutic agents, nucleic acid has been gradually developed as gene therapy for local diseases such as asthma, chronic obstructive pulmonary diseases, and lung fibrosis. The features of nucleic acid, specific physiological structure, and pathophysiological barriers of the respiratory tract have strongly affected the delivery efficiency and pulmonary bioavailability of nucleic acid, directly related to the treatment outcomes. The development of pharmaceutics and material science provides the potential for highly effective pulmonary medicine delivery. In this review, the key factors and barriers are first introduced that affect the pulmonary delivery and bioavailability of nucleic acids. The advanced inhaled materials for nucleic acid delivery are further summarized. The recent progress of platform designs for improving the pulmonary delivery efficiency of nucleic acids and their therapeutic outcomes have been systematically analyzed, with the application and the perspectives of advanced vectors for pulmonary gene delivery., (© 2024 The Authors. Advanced Science published by Wiley‐VCH GmbH.)

Background: Chronic lung disease affects nearly 37 million Americans and often results in significant quality of life impairment and healthcare burden. Despite guidelines calling for palliative care (PC) integration into pulmonary care as a vital part of chronic lung disease management, existing PC models have limited access and lack scalability. Use of telehealth to provide PC offers a potential solution to these barriers. This study explored perceptions of patients with chronic lung disease regarding a telehealth integrated palliative care (TIPC) model, with plans to use findings to inform development of an intervention protocol for future testing., Methods: For this qualitative study, we conducted semi-structured interviews between June 2021- December 2021 with patients with advanced chronic lung disease. Interviews explored experiences with chronic lung disease, understanding of PC, and perceived acceptability of the proposed model along with anticipated facilitators and barriers of the TIPC model. We analyzed findings with a content analysis approach., Results: We completed 20 interviews, with two that included both a patient and caregiver together due to patient preference. Perceptions were primarily related to three categories: burden of chronic lung disease, pre-conceived understanding of PC, and perspective on the proposed TIPC model. Analysis revealed a high level of disease burden related to chronic lung disease and its impact on day-to-day functioning. Although PC was not well understood, the TIPC model using a shared care planning approach via telehealth was seen by most as an acceptable addition to their chronic lung disease care., Conclusions: These findings emphasize the need for a patient-centered, shared care planning approach in chronic lung disease. The TIPC model may be one option that may be acceptable to individuals with chronic lung disease. Future work includes using findings to refine our TIPC model and conducting pilot testing to assess acceptability and utility of the model., (© 2024. The Author(s).)

The articles on the history of Russian pulmonology presented in the historical, medical and therapeutic literature contain materials for this history, but their authors did not solve the problem of its consistent presentation, highlighting the stages of formation and founders. The authors of this study critically reviewed the literary and archival primary sources, for the first time proposed the identification of three stages in the development of Russian pulmonology and indicated eight of its founders at these stages. The abundance of material did not allow us to present it in one article. This article is devoted to the 1st stage of the history of pulmonology - the formation of the doctrine of lung diseases. The second (development of pulmonology as an independent scientific direction in internal diseases) and the third (organizational design of pulmonology as a new independent clinical scientific and educational discipline and medical specialty, i.e. its institutionalization) stages will be discussed in the next articles.

Background: An app providing material for education and entertaining is a possible way to support patients and healthcare providers in achieving person-centered care., Methods: An app tailored on the Fondazione Toscana Gabriele Monasterio (FTGM), a research hospital treating cardiac and lung disorders, was created. A pilot evaluation project was conducted on consecutive patients hospitalized for heart or lung disorders. Patients were asked to complete an assessment questionnaire., Results: The FTGM app provides information on diagnostic and therapeutic investigations, hospital and healthcare personnel, and includes content for entertainment and learning. It was tested on 215 consecutive patients (75% men, 66% aged >60 years, and 40% with a primary or middle school degree). Sixty-nine percentage of patients used the FTGM app, including 67% of patients aged >80 years and 65% of those with an elementary education (65%). Patients gave positive feedback on the app layout. Many (76%) looked for information on doctors and nurses in the 'People' section. Sixty-five percent of responders had used at least one of the sections called 'Music' and 'Museum visits'. The app helped many patients perceive the hospital as a more liveable place (68%), and to feel less anxious (76%), and more engaged in the diagnostic and therapeutic workup (65%). Overall, the majority of responders (87%) rated the app as 'excellent' or 'good', and almost all (95%) would have recommended other patients to use the app., Conclusions: The FTGM app is a possible tool to improve patient wellbeing during hospitalization., (Copyright © 2024 Italian Federation of Cardiology - I.F.C. All rights reserved.)