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European Journal of Pediatrics Jun 2024In 2016, the Spanish Research Group on Bronchopulmonary Dysplasia (BPD) (GEIDIS) established a national registry with participation of 66 hospitals to collect...
In 2016, the Spanish Research Group on Bronchopulmonary Dysplasia (BPD) (GEIDIS) established a national registry with participation of 66 hospitals to collect information on clinical characteristics and long-term outcomes of BPD infants into adulthood. The aim of this observational study is to examine forced spirometry data in early childhood and to assess their correlation with the respiratory support required at 36 weeks postmenstrual age (PMA). The study analyzed data from preterm infants with BPD born between January 2016 and December 2017 who underwent forced spirometry at 5-7 years of age. Statistical analyses were conducted to investigate the relationships between spirometry results, perinatal factors, and the required respiratory support at 36 weeks PMA. The study involved 143 patients with a median gestational age (GA) of 27.3 weeks (range 25.7-28.7) and a median weight of 880 g (range 740-1135). Abnormal spirometry results were observed in 39.2% (56) of the patients. Among patients diagnosed with BPD type 3, those requiring over 30% oxygen at 36 weeks PMA exhibited an increased risk of abnormal spirometry results (OR 4.48; 95% CI 1.11-18.13) compared to those requiring positive pressure with less than 30% oxygen. In addition, this subgroup had a higher risk of developing a restrictive-mixed pattern compared to those with BPD type 1 (OR 10.65; 95% CI 2.06-54.98) and BPD type 2 (OR 6.76; 95% CI 1.09-42.06). No significant differences were found in the incidence of an obstructive pattern between BPD types. Conclusion: The requirement of more than 30% oxygen at 36 weeks PMA serves as a risk indicator for pulmonary function impairment in school-aged children with BPD. These findings suggest persistent airway and parenchymal injury in this specific patient population, and highlight the importance of careful monitoring to evaluate their long-term effects on lung function. What is Known: • Premature patients with bronchopulmonary dysplasia (BPD) may present abnormalities in pulmonary function tests during school age. However, the predictive accuracy of consensus BPD severity classification remains uncertain. What is New: • The requirement of more than 30% oxygen at 36 weeks postmenstrual age (PMA) indicates a potential risk of pulmonary function impairment in school-aged children with BPD. Additionally, a significant correlation has been observed between a restrictive-mixed pattern with exposure to mechanical ventilation and the development of severe forms of BPD.
PubMed: 38858227
DOI: 10.1007/s00431-024-05629-w -
Acta Medica Philippina 2024Cystic Fibrosis (CF) is a rare condition among Asians and has not been reported in the Philippines as of this time. The inclusion of this disease in the Philippines'...
Cystic Fibrosis (CF) is a rare condition among Asians and has not been reported in the Philippines as of this time. The inclusion of this disease in the Philippines' Expanded Newborn Screening Program (ENBS) has provided this Filipino family the opportunity of early detection and appropriate management of this condition that could ensure the survival of the proband and his other surviving siblings. Here we present a case of a 24-month-old male who had a positive Expanded Newborn Screening (ENBS) test for cystic fibrosis and eventually underwent further tests to confirm a homozygous deletion of exons 1 - 2 of the CFTR gene. He subsequently had recurrent pneumonia but is being managed by a team consisting of a pulmonologist, gastroenterologist, and a metabolic dietitian. The proband had an older sibling whose Newborn Screening (NBS) test was normal and who eventually expired from recurrent bouts of pneumonia. This sibling was never managed as a case of cystic fibrosis. Implications on the diagnosis and management of CF in the local setting is also discussed. The importance of an appropriate CF panel customized to the local population should be reiterated and carrier testing should be encouraged to help with proper family counseling for future pregnancies for the family involved.
PubMed: 38846171
DOI: 10.47895/amp.vi0.7570 -
American Journal of Respiratory and... Jun 2024Ground glass opacities (GGO) in the absence of interstitial lung disease are understudied.
RATIONAL
Ground glass opacities (GGO) in the absence of interstitial lung disease are understudied.
OBJECTIVE
To assess the association of GGO with white blood cells (WBCs) and progression of quantified chest CT emphysema.
METHODS
We analyzed data of participants in the Subpopulations and Intermediate Outcome Measures In COPD Study (SPIROMICS). Chest radiologists and pulmonologists labeled regions of the lung as GGO and adaptive multiple feature method (AMFM) trained the computer to assign those labels to image voxels and quantify the volume of the lung with GGO (%GGO). We used multivariable linear regression, zero-inflated negative binomial, and proportional hazards regression models to assess the association of %GGO with WBC, changes in %emphysema, and clinical outcomes.
MEASUREMENTS AND MAIN RESULTS
Among 2,714 participants, 1,680 had COPD and 1,034 had normal spirometry. Among COPD participants, based on the multivariable analysis, current smoking and chronic productive cough was associated with higher %GGO. Higher %GGO was cross-sectionally associated with higher WBCs and neutrophils levels. Higher %GGO per interquartile range at visit 1 (baseline) was associated with an increase in emphysema at one-year follow visit by 11.7% (Relative increase; 95%CI 7.5-16.1%;P<0.001). We found no association between %GGO and one-year FEV decline but %GGO was associated with exacerbations and all-cause mortality during a median follow-up time of 1,544 days (Interquartile Interval=1,118-2,059). Among normal spirometry participants, we found similar results except that %GGO was associated with progression to COPD at one-year follow-up.
CONCLUSIONS
Our findings suggest that GGO is associated with increased systemic inflammation and emphysema progression.
PubMed: 38843116
DOI: 10.1164/rccm.202310-1825OC -
Pediatric Pulmonology Jun 2024Childhood interstitial lung disease (chILD) associated with connective tissue and immune mediated disorders is the second most common chILD diagnostic category. As...
Childhood interstitial lung disease (chILD) associated with connective tissue and immune mediated disorders is the second most common chILD diagnostic category. As knowledge of the molecular and genetic underpinnings of these rare disorders advances, the recognized clinical spectrum of associated pulmonary manifestations continues to expand. Pulmonary complications of these diseases, including ILD, confer increased risk for morbidity and mortality and contribute to increased complexity for providers tasked with managing the multiple organ systems that can be impacted in these systemic disorders. While pulmonologists play an important role in diagnosis and management of these conditions, thankfully they do not have to work alone. In collaboration with a multidisciplinary team of subspecialists, the pulmonary and other systemic manifestations of these conditions can be managed effectively together. The goal of this review is to familiarize the reader with the classic patterns of chILD and other pulmonary complications associated with primary immune-mediated disorders (monogenic inborn errors of immunity) and acquired systemic autoimmune and autoinflammatory diseases. In addition, this review will highlight current, emerging, and innovative therapeutic strategies and will underscore the important role of multidisciplinary management to improving outcomes for these patients.
PubMed: 38837875
DOI: 10.1002/ppul.27068 -
The Journal of Asthma : Official... Jun 2024Inappropriate use of short-acting beta2-agonists (SABA) in asthma has been associated with undesired outcomes. This national expert consensus was developed to spread...
OBJECTIVE
Inappropriate use of short-acting beta2-agonists (SABA) in asthma has been associated with undesired outcomes. This national expert consensus was developed to spread awareness of SABA overuse and provide recommendations on the ways to eliminate SABA overprescription and overreliance in asthma care in Malaysia.
DATA SOURCES
This expert consensus was developed by searching the PubMed database, using index terms to identify SABA overuse-related burden and recommendations made in asthma guidelines. Consensus recommendations were made via the Delphi method, involving a Malaysian expert committee comprising 13 healthcare professionals (five pulmonologists, four family medicine specialists, two emergency medicine physicians and two pharmacists).
STUDY SELECTIONS
The articles reviewed include randomized controlled trials, systematic reviews, meta-analyses, observational studies, guidelines, and surveys, with abstracts in English and published up until June 2023. Relevant recommendations were also sourced from the verified websites of medical organizations and societies.
RESULTS
Eleven consensus statements were developed, each statement achieving agreement level of at least 70%. The statements reflect SABA overreliance in asthma care, as well as recommendations to eliminate SABA overprescription and overreliance in Malaysia. Supporting evidence in literature as well as expert committee discussions leading to the development of the finalized statements were elaborated.
CONCLUSION
This national expert consensus discussed the burden of SABA overreliance and made specific recommendations to eliminate SABA overprescription and overreliance in the Malaysian context. This consensus document is anticipated to impart better awareness among Malaysian healthcare providers and contribute to the continuous improvement of asthma care in the country.
PubMed: 38832793
DOI: 10.1080/02770903.2024.2361780 -
Nuclear Medicine Communications Jun 2024This prospective study included 92 patients who underwent BLVR with quantitative SPECT/CT study pre- and post-procedure between November 2018 and June 2023. The mean age...
METHODS
This prospective study included 92 patients who underwent BLVR with quantitative SPECT/CT study pre- and post-procedure between November 2018 and June 2023. The mean age was 70 years (range 56-85). with 51 males and 41 females. SPECT/CT quantified perfusion for each lobe, and the lowest counts/volume ratio determined the procedural target. Postprocedure SPECT/CT assessed total atelectasis and perfusion shifts. The 6-minute walk test and pulmonary function tests were compared pre- and post-BLVR.
RESULTS
SPECT/CT-guided BLVR showed clinical benefits (decreased oxygen requirements) and physiological improvements in total lung capacity, forced expiratory volume, and forced vital capacity (P < 0.05). Significant perfusion shifts were observed away from the target lobe, with unique patterns noted for ipsilateral and contralateral nontarget lobes (P < 0.05).
CONCLUSION
Quantitative lobar SPECT/CT in preprocedural guidance for BLVR proved useful in identifying suitable targets in multi-lobe homogeneous emphysema, resulting in physiological and clinical improvements for this patient group. The perfusion shift information provided by SPECT/CT offers valuable insights for pulmonologists.
PubMed: 38832427
DOI: 10.1097/MNM.0000000000001868 -
International Journal of Chronic... 2024Chronic obstructive pulmonary disease (COPD) poses a significant global health burden despite being largely preventable and treatable. Despite the availability of...
PURPOSE
Chronic obstructive pulmonary disease (COPD) poses a significant global health burden despite being largely preventable and treatable. Despite the availability of guidelines, COPD care remains suboptimal in many settings, including high-income countries (HICs) and upper-middle-income countries (UMICs), with varied approaches to diagnosis and management. This study aimed to identify common and unique barriers to COPD care across six countries (Australia, Spain, Taiwan, Argentina, Mexico, and Russia) to inform global policy initiatives for improved care.
METHODS
COPD care pathways were mapped for each country and supplemented with epidemiological, health-economic, and clinical data from a targeted literature review. Semi-structured interviews with 17 respiratory care clinicians were used to further validate the pathways and identify key barriers. Thematic content analysis was used to generate the themes.
RESULTS
Six themes were common in most HICs and UMICs: "Challenges in COPD diagnosis", "Strengthening the role of primary care", "Fragmented healthcare systems and coordination challenges", "Inadequate management of COPD exacerbations", "Limited access to specialized care" and, "Impact of underfinanced and overloaded healthcare systems". One theme, "Insurance coverage and reimbursement challenges", was more relevant for UMICs. HICs and UMICs differ in patient and healthcare provider awareness, primary care involvement, spirometry access, and availability of specialized care. Both face issues with healthcare fragmentation, guideline adherence, and COPD exacerbation management. In addition, UMICs also grapple with resource limitations and healthcare infrastructure challenges.
CONCLUSION
Many challenges to COPD care are the same in both HICs and UMICs, underscoring the pervasive nature of these issues. While country-specific issues require customized solutions, there are untapped possibilities for implementing global respiratory strategies that support countries to manage COPD effectively. In addition to healthcare system-level initiatives, there is a crucial need for political prioritization of COPD to allocate the essential resources it requires.
Topics: Pulmonary Disease, Chronic Obstructive; Humans; Qualitative Research; Health Services Accessibility; Attitude of Health Personnel; Developing Countries; Primary Health Care; Developed Countries; Health Knowledge, Attitudes, Practice; Mexico; Healthcare Disparities; Interviews as Topic; Delivery of Health Care, Integrated; Practice Patterns, Physicians'; Pulmonologists; Argentina; Guideline Adherence; Taiwan
PubMed: 38831892
DOI: 10.2147/COPD.S449659 -
Chest Jun 2024Acute pulmonary embolism (PE) is a common disease encountered by pulmonologists, cardiologists, and critical care physicians throughout the world. For patients with... (Review)
Review
TOPIC IMPORTANCE
Acute pulmonary embolism (PE) is a common disease encountered by pulmonologists, cardiologists, and critical care physicians throughout the world. For patients with high-risk acute PE (defined by systemic hypotension) and intermediate high-risk acute PE (defined by the absence of systemic hypotension, but the presence of numerous other concerning clinical and imaging features), intensive care often is necessary. Initial management strategies should focus on optimization of right ventricle (RV) function while decisions about advanced interventions are being considered.
REVIEW FINDINGS
We reviewed the existing literature of various vasoactive agents, IV fluids and diuretics, and pulmonary vasodilators in both animal models and human trials of acute PE. We also reviewed the potential complications of endotracheal intubation and positive pressure ventilation in acute PE. Finally, we reviewed the data of venoarterial extracorporeal membrane oxygenation (ECMO) use in acute PE. The above interventions are discussed in the context of the underlying pathophysiologic features of acute RV failure in acute PE with corresponding illustrations.
SUMMARY
Norepinephrine is a reasonable first choice for hemodynamic support with vasopressin as an adjunct. IV loop diuretics may be useful if evidence of RV dysfunction or volume overload is present. Fluids should be given only if concern exists for hypovolemia and absence of RV dilatation. Supplemental oxygen administration should be considered even without hypoxemia. Positive pressure ventilation should be avoided if possible. venoarterial ECMO cannulation should be implemented early if ongoing deterioration occurs despite these interventions.
PubMed: 38830402
DOI: 10.1016/j.chest.2024.04.032 -
Journal of Managed Care & Specialty... Jun 2024Health plan coverage is central to patient access to care, especially for rare, chronic diseases. For specialty drugs, coverage varies, resulting in barriers to access....
BACKGROUND
Health plan coverage is central to patient access to care, especially for rare, chronic diseases. For specialty drugs, coverage varies, resulting in barriers to access. Pulmonary arterial hypertension (PAH) is a rare, progressive, and fatal disease. Guidelines suggest starting or rapidly escalating to combination therapy with drugs of differing classes (phosphodiesterase 5 inhibitors [PDE5is], soluble guanylate cyclase stimulators [sGC stimulators], endothelin receptor antagonists [ERAs], and prostacyclin pathway agents [PPAs]).
OBJECTIVE
To assess the variation in commercial health plan coverage for PAH treatments and how coverage has evolved. To examine the frequency of coverage updates and evidence cited in plan policies.
METHODS
We used the Tufts Medical Center Specialty Drug Evidence and Coverage database, which includes publicly available specialty drug coverage policies. Overall, and at the drug and treatment class level, we identified plan-imposed coverage restrictions beyond the drug's US Food and Drug Administration label, including step therapy protocols, clinical restrictions (eg, disease severity), and prescriber specialty requirements. We analyzed variation in coverage restrictiveness and how coverage has changed over time. We determined how often plans update their policies. Finally, we categorized the cited evidence into 6 different types.
RESULTS
Results reflected plan coverage policies for 13 PAH drugs active between August 2017 and August 2022 and issued by 17 large US commercial health plans, representing 70% of covered lives. Coverage restrictions varied mainly by step therapy protocols and prescriber restrictions. Seven plans had step therapy protocols for most drugs, 9 for at least one drug, and 1 had none. Ten plans required specialist (cardiologist or pulmonologist) prescribing for at least one drug, and 7 did not. Coverage restrictions increased over time: the proportion of policies with at least 1 restriction increased from 38% to 73%, and the proportion with step therapy protocols increased from 29% to 46%, with generics as the most common step. The proportion of policies with step therapy protocols increased for every therapy class with generic availability: 18% to 59% for ERAs, 33% to 77% for PDE5is, and 33% to 43% for PPAs. The proportion of policies with prescriber requirements increased from 24% to 48%. Plans updated their policies 58% of the time annually and most often cited the 2019 CHEST clinical guidelines, followed by randomized controlled trials.
CONCLUSIONS
Plan use of coverage restrictions for PAH therapies increased over time and varied across both drugs and plans. Inconsistency among health plans may complicate patient access and reduce the proportion who can persist on PAH treatments.
Topics: Humans; United States; Antihypertensive Agents; Pulmonary Arterial Hypertension; Insurance Coverage; Phosphodiesterase 5 Inhibitors; Hypertension, Pulmonary; Insurance, Pharmaceutical Services
PubMed: 38824632
DOI: 10.18553/jmcp.2024.30.6.541 -
Clinics in Chest Medicine Jun 2024The pericardium comprises a double-walled fibrous-serosal sac that encloses the heart. Reflections of the serosal layer form sinuses and recesses. With advances in... (Review)
Review
The pericardium comprises a double-walled fibrous-serosal sac that encloses the heart. Reflections of the serosal layer form sinuses and recesses. With advances in multidetector computed tomography (CT) technology, pericardial recesses are frequently detected with thin-section CT. Knowledge of pericardial anatomy on imaging is crucial to avoid misinterpretation of fluid-filled pericardial sinuses and recesses as adenopathy/pericardial metastasis or aortic dissection, which can impact patient management and treatment decisions. The authors offer a comprehensive review of pericardial anatomy and its variations observed on CT, potential pitfalls in image interpretation, and implications for the pulmonologist with respect to unnecessary diagnostic procedures or interventions.
Topics: Humans; Pericardium; Tomography, X-Ray Computed; Pulmonologists; Multidetector Computed Tomography
PubMed: 38816085
DOI: 10.1016/j.ccm.2024.02.002