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Children (Basel, Switzerland) May 2024Pulmonologists may be involved in managing pulmonary diseases in children with complex clinical pictures without a diagnosis. Moreover, they are routinely involved in... (Review)
Review
Pulmonologists may be involved in managing pulmonary diseases in children with complex clinical pictures without a diagnosis. Moreover, they are routinely involved in the multidisciplinary care of children with rare diseases, at baseline and during follow-up, for lung function monitoring. Lysosomal storage diseases (LSDs) are a group of genetic diseases characterised by a specific lysosomal enzyme deficiency. Despite varying pathogen and organ involvement, they are linked by the pathological accumulation of exceeding substrates, leading to cellular toxicity and subsequent organ damage. Less severe forms of LSDs can manifest during childhood or later in life, sometimes being underdiagnosed. Respiratory impairment may stem from different pathogenetic mechanisms, depending on substrate storage in bones, with skeletal deformity and restrictive pattern, in bronchi, with obstructive pattern, in lung interstitium, with altered alveolar gas exchange, and in muscles, with hypotonia. This narrative review aims to outline different pulmonary clinical findings and a diagnostic approach based on key elements for differential diagnosis in some treatable LSDs like Gaucher disease, Acid Sphingomyelinase deficiency, Pompe disease and Mucopolysaccharidosis. Alongside their respiratory clinical aspects, which might overlap, we will describe radiological findings, lung functional patterns and associated symptoms to guide pediatric pulmonologists in differential diagnosis. The second part of the paper will address follow-up and management specifics. Recent evidence suggests that new therapeutic strategies play a substantial role in preventing lung involvement in early-treated patients and enhancing lung function and radiological signs in others. Timely diagnosis, driven by clinical suspicion and diagnostic workup, can help in treating LSDs effectively.
PubMed: 38929247
DOI: 10.3390/children11060668 -
Annals of the American Thoracic Society Mar 2024Indoor sources of air pollution worsen indoor and outdoor air quality. Thus, identifying and reducing indoor pollutant sources would decrease both indoor and outdoor air...
Indoor sources of air pollution worsen indoor and outdoor air quality. Thus, identifying and reducing indoor pollutant sources would decrease both indoor and outdoor air pollution, benefit public health, and help address the climate crisis. As outdoor sources come under regulatory control, unregulated indoor sources become a rising percentage of the problem. This American Thoracic Society workshop was convened in 2022 to evaluate this increasing proportion of indoor contributions to outdoor air quality. The workshop was conducted by physicians and scientists, including atmospheric and aerosol scientists, environmental engineers, toxicologists, epidemiologists, regulatory policy experts, and pediatric and adult pulmonologists. Presentations and discussion sessions were centered on ) the generation and migration of pollutants from indoors to outdoors, ) the sources and circumstances representing the greatest threat, and ) effective remedies to reduce the health burden of indoor sources of air pollution. The scope of the workshop was residential and commercial sources of indoor air pollution in the United States. Topics included wood burning, natural gas, cooking, evaporative volatile organic compounds, source apportionment, and regulatory policy. The workshop concluded that indoor sources of air pollution are significant contributors to outdoor air quality and that source control and filtration are the most effective measures to reduce indoor contributions to outdoor air. Interventions should prioritize environmental justice: Households of lower socioeconomic status have higher concentrations of indoor air pollutants from both indoor and outdoor sources. We identify research priorities, potential health benefits, and mitigation actions to consider (e.g., switching from natural gas to electric stoves and transitioning to scent-free consumer products). The workshop committee emphasizes the benefits of combustion-free homes and businesses and recommends economic, legislative, and education strategies aimed at achieving this goal.
Topics: Humans; Child; United States; Air Pollution, Indoor; Natural Gas; Environmental Monitoring; Air Pollution; Air Pollutants; Particulate Matter
PubMed: 38426826
DOI: 10.1513/AnnalsATS.202312-1067ST -
Cureus Aug 2023Asthma is a prevalent chronic respiratory condition affecting a significant portion of women of reproductive age. While the impact of asthma on general health and... (Review)
Review
Asthma is a prevalent chronic respiratory condition affecting a significant portion of women of reproductive age. While the impact of asthma on general health and well-being has been extensively studied, its association with fertility challenges in women remains an area of growing concern. This review article explores the complexities surrounding fertility challenges in asthmatic women, specifically focusing on pregnancy loss, infertility, and the utilization of assisted reproductive technologies (ARTs). Various factors contribute to the heightened risk of pregnancy loss in asthmatic women, including the systemic inflammation associated with asthma, suboptimal asthma control, medication usage, and comorbidities. The review highlights the need for multidisciplinary management approaches to optimize asthma control before and during pregnancy, reducing the risk of adverse pregnancy outcomes. Furthermore, the review investigates the potential impact of asthma on female fertility and the underlying mechanisms involved. Asthma-related factors, such as chronic inflammation, altered hormonal balance, and medication effects, may disrupt the delicate reproductive processes, leading to infertility. It emphasizes the importance of comprehensive fertility evaluations and personalized treatment strategies for asthmatic women experiencing difficulties conceiving. Additionally, the article explores the utilization of ARTs, including in vitro fertilization (IVF) and intracytoplasmic sperm injection (ICSI), in asthmatic women. It discusses the safety considerations and potential challenges associated with these techniques, such as the impact of asthma medications on oocyte quality, the effects of hormonal stimulation on asthma control, and the risk of exacerbations during the IVF process. The review underscores the importance of collaborative efforts among healthcare providers, including allergists, pulmonologists, obstetricians, and fertility specialists, to ensure optimal management of asthmatic women seeking to conceive. It emphasizes the significance of preconception counseling, meticulous asthma control, appropriate medication management, and individualized fertility treatments to enhance the reproductive outcomes in this population.
PubMed: 37692593
DOI: 10.7759/cureus.43104 -
Journal of Asthma and Allergy 2023It has been estimated that, in 2019, 54,000 patients in Germany had uncontrolled GINA step 4/5 asthma. In the current study we analyzed which health care providers were...
PURPOSE
It has been estimated that, in 2019, 54,000 patients in Germany had uncontrolled GINA step 4/5 asthma. In the current study we analyzed which health care providers were involved in the management of these patients and their role in disease phenotyping.
PATIENTS AND METHODS
The year 2019 was retrospectively analyzed using the IQVIA LRx, a longitudinal anonymized prescription database, and the electronic, anonymized medical records database, the IQVIA Disease Analyzer.
RESULTS
Of 54,000 uncontrolled GINA step 4/5 asthma patients in Germany, 52% had consulted both general practitioners (GPs) and pulmonologists, and 48% were seen exclusively by a GP. Of these 54,000 patients, 45% were being prescribed and were thus overusing short-acting β2-agonists (SABAs) and oral corticosteroids (OCS) for ≥2 years, 26% for ≥3 years, and 16% for ≥4 years. In most regions, pulmonologists saw one of their uncontrolled GINA step 4/5 asthma patients per week. Laboratory tests from consultations with a GP were available for only 10% of patients referred to a pulmonologist. In 50% of uncontrolled asthma patients treated according to GINA step 4/5, these were initiated by the pulmonologist, and 34% received laboratory testing within the first year (in GINA step 4/5 asthma, the numbers are 20% and 18%, respectively).
CONCLUSION
Fifty percent of uncontrolled asthma patients treated according to GINA step 4/5 were regularly seen by pulmonologists, who performed most of the phenotyping confirming their importance in the management of severe, uncontrolled asthma in Germany. To understand treatment pathways for these patients, further studies are needed.
PubMed: 37559894
DOI: 10.2147/JAA.S418658 -
Respiratory Medicine Case Reports 2024Urinothorax is a rare cause of pleural effusion. Infected urinothorax is even rarer. Here we present a case of infected urinothorax from renal mass causing obstructive...
Urinothorax is a rare cause of pleural effusion. Infected urinothorax is even rarer. Here we present a case of infected urinothorax from renal mass causing obstructive uropathy. Patient improved with pleural drainage and a multidisciplinary approach of treatment between team involving urologist and pulmonologist. This case highlights the complexity in the diagnosis and management of infected urinothorax.
PubMed: 38318225
DOI: 10.1016/j.rmcr.2024.101989 -
Medicina 2024Adult smokers, those with comorbidities, and the elderly, are at greater risk of contracting infections and their complications. Community acquired respiratory... (Review)
Review
Adult smokers, those with comorbidities, and the elderly, are at greater risk of contracting infections and their complications. Community acquired respiratory infections due to viruses, pneumococcus and other bacteria, affect both healthy and sick adults. There are vaccines that the pulmonologist must know and prescribe. The target strains of the influenza vaccine are defined by the WHO for the Southern hemisphere considering those involved in the previous influenza season in the Northern hemisphere. Its effectiveness depends on virulence, concordance between circulating and vaccine strains, and population coverage. The anti-pneumococcal polysaccharide vaccine available since 1983 is being replaced by more effective conjugate vaccines to prevent infections related to serotypes present in them. Immunization against SARS-CoV-2 reduced the contagion, severity, and lethality of COVID-19. The acellular vaccine against Bordetella pertussis for adults is present for specific situations in the adult calendar; vaccinating them strengthens the control of childhood contagion. The double (diphtheria + tetanus), and triple (double + pertussis) bacterial vaccines, and the vaccines against measles, chickenpox, rubella, human papillomavirus, Haemophilus influenzae, meningococcus, herpes zoster, Argentine hemorrhagic fever and yellow fever, are of a more limited use. Soon we will have new vaccines such as the one recently approved by the FDA against respiratory syncytial virus. Through a consensus of experts in respiratory infections, we review the new evidence regarding the immunization of adults who consult a pulmonologist, and thus update the recommendations on vaccination made eight years ago.
Topics: Adult; Humans; Infant; Aged; Pulmonary Medicine; Vaccination; Pneumococcal Vaccines; Influenza Vaccines; COVID-19
PubMed: 38271938
DOI: No ID Found -
Emergency Medicine Journal : EMJ Oct 2023Serial point-of-care ultrasound (PoCUS) can potentially improve acute patient care through treatment adjusted to the dynamic ultrasound findings. The objective was to... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Serial point-of-care ultrasound (PoCUS) can potentially improve acute patient care through treatment adjusted to the dynamic ultrasound findings. The objective was to investigate if treatment guided by monitoring patients with acute dyspnoea with serial cardiopulmonary PoCUS and usual care could reduce the severity of dyspnoea compared with usual care alone.
METHODS
This was a randomised, controlled, blinded-outcome trial conducted in three EDs in Denmark between 9 October 2019 and 26 May 2021. Patients aged ≥18 years admitted with a primary complaint of dyspnoea were allocated 1:1 with block randomisation to usual care, which included a single cardiopulmonary PoCUS within 1 hour of arrival (control group) or usual care (including a PoCUS within 1 hour of arrival) plus two additional PoCUS performed at 2 hours interval from the initial PoCUS (serial ultrasound group). The primary outcome was a reduction of dyspnoea measured on a verbal dyspnoea scale (VDS) from 0 to 10 recorded at inclusion and after 2, 4 and 5 hours.
RESULTS
There were 206 patients recruited, 102 in the serial ultrasound group and 104 in the control group, all of whom had complete follow-up. The mean difference in VDS between patients in the serial ultrasound and the control group was -1.09 (95% CI -1.51 to -0.66) and -1.66 (95% CI -2.09 to -1.23) after 4 and 5 hours, respectively. The effect was more pronounced in patients with a presumptive diagnosis of acute heart failure (AHF). A larger proportion of patients received diuretics in the serial ultrasound group.
CONCLUSION
Therapy guided by serial cardiopulmonary PoCUS may, together with usual care, facilitate greater improvement in the severity of dyspnoea, especially in patients with AHF compared with usual care with a single PoCUS in the ED. Serial PoCUS should therefore be considered for routine use to aid the physician in stabilising the patient faster.
TRIAL REGISTRATION NUMBER
NCT04091334.
Topics: Humans; Adolescent; Adult; Point-of-Care Systems; Point-of-Care Testing; Heart; Dyspnea; Heart Failure; Ultrasonography; Emergency Service, Hospital
PubMed: 37595984
DOI: 10.1136/emermed-2022-212694 -
JMIR Formative Research Dec 2023Asthma is one of the most common respiratory diseases, with an ever-growing health care burden. Remote patient monitoring (RPM) has gained increasing importance in the...
BACKGROUND
Asthma is one of the most common respiratory diseases, with an ever-growing health care burden. Remote patient monitoring (RPM) has gained increasing importance in the respiratory care area with the outbreak of the COVID-19 pandemic. In this pilot study, we introduced a novel platform that remotely monitors patients with chronic respiratory illnesses using Centers for Disease Control and Prevention guidelines to reduce hospitalizations and emergency department visits.
OBJECTIVE
This study aimed to understand patient and physician engagement with a new virtual care solution (KevaTalk app and Keva365 platform) and the value, for both patients and providers, of using an RPM tool. We assessed real-world use of the platform from both physician and patient perspectives and the impact of devices on engagement and monitoring.
METHODS
Participants with a history of moderate to severe persistent asthma, seen by a pulmonologist at a hospital, were included in this study. The inclusion criteria involved being aged ≥18 years and having access to an Android or iOS mobile device with internet. We provided patient questionnaires to assess the app's usefulness and evaluate its features. We monitored remote spirometry and oximetry data, app check-ins, alerts, and escalations during this study's time window. Data were reviewed daily and predetermined criteria were set to escalate for physician review based on the patient's symptoms and objective data.
RESULTS
Overall, 25 patients were included in this pilot. The mean age was 57 (SD 10.7) years and a majority (n=23, 92%) were female. A baseline questionnaire, which was used to rate the app, indicated that the ease of check-in and ease of modification to the patient's asthma plan were the 2 highest rated features. In total, 2066 check-ins (1550 green, 506 yellow, and 10 red check-ins) and 1155 spirometry sessions were recorded during this 3-month period. Further, 64% (14/22) and 91% (20/22) of patients were found to have peak flows in their red and yellow zones at least once, respectively. During the course of this study, 484 alerts were recorded and evaluated by the team, of which 37.2% (n=180) required an escalation to the physician; this included a transfer to a medical facility, change in respiratory medication, or further education.
CONCLUSIONS
In this pilot study, we demonstrated the feasibility of implementing a novel RPM platform in patients with asthma. Our platform showed high patient engagement and satisfaction and provided physicians with real-time subjective data to evaluate patients remotely that aids in clinical decision-making. The escalations prevented patients from having an exacerbation or flare up, which led to the prevention of an emergency department visit. Continuous monitoring of chronic disease has benefits over episodic monitoring. It allows for improved quality of life, better outcomes, and huge health care savings.
PubMed: 38153783
DOI: 10.2196/51065 -
Patient Preference and Adherence 2023Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and ultimately fatal lung disease that, while rare, has seen incidence rise over time. There is no cure...
I-PreFer Study: A Discrete Choice Experiment to Explore Patient, Caregiver and Pulmonologist Preferences of Idiopathic Pulmonary Fibrosis Pharmacological Treatment Options.
PURPOSE
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and ultimately fatal lung disease that, while rare, has seen incidence rise over time. There is no cure for IPF other than a lung transplant, though two antifibrotic (AF) drugs do exist to slow disease progression. While these drugs are efficacious, they are both associated with differing profiles of adverse events. This study aimed to elicit patient, caregiver and pulmonologist preferences on the treatment profiles of AFs via a discrete choice experiment (DCE).
PATIENTS AND METHODS
The DCE and associated survey were distributed across 7 European countries, and bespoke DCEs were developed for patients/caregivers and pulmonologists. After collaboration with European Pulmonary Fibrosis & Related Disorders Federation (EU-PFF) and expert pulmonologists, respectively, a patient/caregiver DCE with 5 attributes and a pulmonologist DCE with 6 attributes were finalized. The DCEs had a blocked approach to reduce participant burden and were distributed on an online survey platform. Preferences were estimated through conditional multinomial logit regression analysis.
RESULTS
Ninety-five patients, 22 caregivers and 115 pulmonologists fully completed their respective DCEs. Overall, patients and caregivers preferred management of treatment-related adverse events over both survival benefits and disease progression. Nearly all preference levels were found to be significantly different from their reference level. In contrast, pulmonologists showed a greater preference for control of lung function and exacerbations over adverse events. Although there were relative differences between the univariate subgroups in terms of the preference weights, most of these were not statistically significant.
CONCLUSION
The outcomes from this study suggest that while patients and caregivers had similar preferences for characteristics of IPF treatments, pulmonologists did not share those same preferences. Patients and caregivers preferred safety, while pulmonologists preferred efficacy. These differences should be considered by clinicians to better involve the patient in treatment decision-making for IPF.
PubMed: 37560147
DOI: 10.2147/PPA.S409767 -
Europace : European Pacing,... Jul 2023Chronic obstructive pulmonary disease (COPD) negatively impacts the efficacy of heart rhythm control treatments in patients with atrial fibrillation (AF). Although COPD...
AIMS
Chronic obstructive pulmonary disease (COPD) negatively impacts the efficacy of heart rhythm control treatments in patients with atrial fibrillation (AF). Although COPD is recognized as a risk factor for AF, practical guidance about how and when to screen for COPD is not available. Herein, we describe the implementation of an integrated screening and management pathway for COPD into the existing pre-ablation work-up in an AF outpatient clinic infrastructure.
METHODS AND RESULTS
Consecutive unselected patients accepted for AF catheter ablation in the Maastricht University Medical Center+ were prospectively screened for airflow limitation using handheld (micro)spirometry at the pre-ablation outpatient clinic supervised by an AF nurse. Patients with results suggestive of airflow limitation were offered referral to the pulmonologist. Handheld (micro)spirometry was performed in 232 AF patients, which provided interpretable results in 206 (88.8%) patients. Airflow limitation was observed in 47 patients (20.3%). Out of these 47 patients, 29 (62%) opted for referral to the pulmonologist. The primary reason for non-referral was low perceived symptom burden. Using this screening strategy 17 (out of 232; 7.3%) ultimately received a diagnosis of chronic respiratory disease, either COPD or asthma.
CONCLUSION
A COPD care pathway can successfully be embedded in an existing AF outpatient clinic infrastructure, using (micro)spirometry and remote analysis of results. Although one out of five patients had results suggestive of an underlying chronic respiratory disease, only 62% of these patients opted for a referral. Pre-selection of patients as well as patient education might increase the diagnostic yield and requires further research.
Topics: Humans; Atrial Fibrillation; Pulmonary Disease, Chronic Obstructive; Lung; Risk Factors
PubMed: 37421318
DOI: 10.1093/europace/euad193