CHEST pulmonaryPub Date : 2023-09-01DOI: 10.1016/j.chpulm.2023.100011
Xiao Li MD , Yu Tang MD , Chaohui Chen MD , Penggao Wang MD , Yuelin Shen MD, PhD
{"title":"A 7-Year-Old Boy With a Giant Mobile Mass in the Right Ventricle","authors":"Xiao Li MD , Yu Tang MD , Chaohui Chen MD , Penggao Wang MD , Yuelin Shen MD, PhD","doi":"10.1016/j.chpulm.2023.100011","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100011","url":null,"abstract":"<div><h3>Case Presentation</h3><p>A 7-year-old male patient sought treatment with a 7-day history of high-grade fever, nonproductive cough, and fatigue. He did not report any chest pain or hemoptysis. His perinatal history, growth, and development were unremarkable. Family history showed hereditary thromboembolism. His sister had sought treatment with similar symptoms 1 week previously. Upper airway samples taken for SARS-CoV-2 showed negative results. The patient received azithromycin for community-acquired pneumonia. He was transferred to our hospital because of persistent fever and worsening lobar pneumonia on chest radiograph.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49791519","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-09-01DOI: 10.1016/j.chpulm.2023.100008
Kelly M. Chin MD, MSCS , William R. Auger MD , Raymond L. Benza MD , Richard N. Channick MD , R. Duane Davis MD , C. Greg Elliott MD , Feng He MS , Sonia Jain PhD , Michael M. Madani MD , Vallerie V. McLaughlin MD , Sudarshan Rajagopal MD, PhD , Josanna Rodriguez-Lopez MD , Victor F. Tapson MD , Kim M. Kerr MD, FCCP , Andrea LaCroix PhD
{"title":"Long-term Survival and Quality of Life","authors":"Kelly M. Chin MD, MSCS , William R. Auger MD , Raymond L. Benza MD , Richard N. Channick MD , R. Duane Davis MD , C. Greg Elliott MD , Feng He MS , Sonia Jain PhD , Michael M. Madani MD , Vallerie V. McLaughlin MD , Sudarshan Rajagopal MD, PhD , Josanna Rodriguez-Lopez MD , Victor F. Tapson MD , Kim M. Kerr MD, FCCP , Andrea LaCroix PhD","doi":"10.1016/j.chpulm.2023.100008","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100008","url":null,"abstract":"<div><h3>Background</h3><p>Chronic thromboembolic pulmonary hypertension (CTEPH) causes significant morbidity and mortality, but long-term outcomes from contemporary multicenter studies are lacking.</p></div><div><h3>Research Question</h3><p>How are survival and health-related quality of life characterized in patients with CTEPH who are classified as inoperable, who are operable but have not undergone surgery, and who have undergone pulmonary thromboendarterectomy surgery?</p></div><div><h3>Study Design and Methods</h3><p>Patients with CTEPH recruited from 30 US sites from 2015 through 2018 completed the 36-item Short Form Health Survey (SF-36) and emPHasis-10 survey at baseline and 6-month intervals. Mixed model repeated measures analysis was used to compare between-group differences in score up to 2 years vs baseline. Multivariable Cox proportional hazards models were used to analyze survival by CTEPH group<em>.</em></p></div><div><h3>Results</h3><p>Seven hundred fifty patients with a median age of 59 years were enrolled; 566 patients, 88 patients, and 96 patients were in the operated, operable but no surgery, and inoperable groups, respectively. Survival at 1, 2, and 3 years was 93%, 91%, and 87%, respectively. Patients in the inoperable and the operable but no surgery groups showed higher mortality rates relative to the operated group (hazard ratios, 2.10 [95% CI, 1.17-3.77] and 2.19 [95% CI, 1.20-3.99], respectively). The EmPHasis-10 and both SF-36 component scores improved during follow-up, with larger increases for the operated group (<em>P</em> < .05, unadjusted and adjusted vs inoperable and operable but no surgery groups at all time points up to 2 years for the SF-36 physical component score and EmPHasis-10 and at some time points for the SF-36 mental component score).</p></div><div><h3>Interpretation</h3><p>Better survival and quality-of-life outcomes were observed in patients undergoing pulmonary thromboendarterectomy.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49819291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-09-01DOI: 10.1016/j.chpulm.2023.100009
Jenny Z. Yang MD , Nick H. Kim MD , Seth Kligerman MD , Timothy M. Fernandes MD, MPH , Demosthenes G. Papamatheakis MD , David S. Poch MD , Mona Alotaibi MD , Victor G. Pretorius MD , Michael M. Madani MD , Kim M. Kerr MD
{"title":"Outcomes Associated With Catheter-Directed Therapies in Chronic Thromboembolic Pulmonary Hypertension","authors":"Jenny Z. Yang MD , Nick H. Kim MD , Seth Kligerman MD , Timothy M. Fernandes MD, MPH , Demosthenes G. Papamatheakis MD , David S. Poch MD , Mona Alotaibi MD , Victor G. Pretorius MD , Michael M. Madani MD , Kim M. Kerr MD","doi":"10.1016/j.chpulm.2023.100009","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100009","url":null,"abstract":"<div><h3>Background</h3><p>Catheter-directed therapies (CDTs) for acute pulmonary embolism (PE) are becoming increasingly popular. Although potentially beneficial in acute PE, CDT is ineffective for chronic thromboembolic disease. Herein we present our experience of patients with subsequently confirmed chronic thromboembolic pulmonary hypertension (CTEPH) or pulmonary artery sarcoma who initially received CDT before referral for surgical intervention.</p></div><div><h3>Research Question</h3><p>How often is CDT being used in patients with CTEPH or pulmonary artery tumor, and what are the associated outcomes?</p></div><div><h3>Study Design and Methods</h3><p>Retrospective review of all pulmonary thromboendarterectomy surgeries performed at the University of California, San Diego, from January 1, 2020, through December 31, 2021.</p></div><div><h3>Results</h3><p>Three hundred fifty-four pulmonary thromboendarterectomy surgeries were performed during the study period. Fifty-two patients received CDT before referral (15%). Before CDT attempt, duration of dyspnea ranged from 3 days to 10 years and mean right ventricular systolic pressure measured by echocardiography was 75 ± 23 mm Hg. After CDT, mean right ventricular systolic pressure was 77 ± 23 mm Hg. Three patients reported full recovery of symptoms, 23 patients reported some improvement, and 26 patients reported no change in symptoms. Imaging at time of CDT was available for review for 32 patients; 23 patients showed radiologic evidence of CTEPH and three patients showed evidence suspicious of sarcoma. Complications associated with CDT occurred in seven patients (13%) and included one death.</p></div><div><h3>Interpretation</h3><p>Radiologic signs of CTEPH frequently were overlooked at the time of CDT. Most patients (94%) achieved minimal or no improvement in symptoms after CDT, and 13% experienced complications. It is important to assess for clinical and radiologic signs of CTEPH when considering CDT in presumed acute PE to minimize unnecessary risk and instead refer patients for CTEPH evaluation.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49819275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-09-01DOI: 10.1016/j.chpulm.2023.100007
Taiga Miyazaki MD, PhD , Katsuji Hirano MD , Kiyoshi Ichihara MD, PhD , Elisa Gonzalez MS , Bradford D. Gessner MD , Raul E. Isturiz MD , Pingping Zhang MS , Sharon Gray MS , Michael Pride PhD , Mark van der Linden PhD , Luis Jodar PhD , Takahiro Maeda MD, PhD , Shigeru Kohno MD, PhD , Adriano Arguedas MD , Goto City Pneumococcal Pneumonia Incidence Study Group
{"title":"Community-Acquired Pneumonia Incidence in Adults Aged 18 Years and Older in Goto City, Japan","authors":"Taiga Miyazaki MD, PhD , Katsuji Hirano MD , Kiyoshi Ichihara MD, PhD , Elisa Gonzalez MS , Bradford D. Gessner MD , Raul E. Isturiz MD , Pingping Zhang MS , Sharon Gray MS , Michael Pride PhD , Mark van der Linden PhD , Luis Jodar PhD , Takahiro Maeda MD, PhD , Shigeru Kohno MD, PhD , Adriano Arguedas MD , Goto City Pneumococcal Pneumonia Incidence Study Group","doi":"10.1016/j.chpulm.2023.100007","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100007","url":null,"abstract":"<div><h3>Background</h3><p>Few studies have measured the burden of community-acquired pneumonia (CAP) and pneumococcal vaccine-type CAP in Japan after the introduction of the 23-valent pneumococcal polysaccharide vaccine into the adult national immunization program for individuals aged ≥ 65 years in 2014. In this study, we estimated the incidences of CAP and <em>Streptococcus pneumoniae</em> CAP among Japanese adults between 2015 and 2020.</p></div><div><h3>Research Question</h3><p>What are the incidences of CAP and <em>S pneumoniae</em> CAP among Japanese adults? What are the common pneumococcal serotypes detected in patients with <em>S pneumoniae</em> CAP?</p></div><div><h3>Study Design and Methods</h3><p>This prospective population-based multicenter active surveillance study enrolled adults ≥ 18 years of age with clinically and radiologically confirmed CAP in Goto City, Japan. <em>S pneumoniae</em> was detected using standard-of-care blood and sputum cultures, BinaxNOW (Abbott), and serotype-specific urinary antigen detection assays.</p></div><div><h3>Results</h3><p>A total of 2,103 patients with CAP were enrolled; 84% were aged ≥ 65 years and 6.7% died during the study. The annual CAP, <em>S pneumoniae</em> CAP, 13-valent pneumococcal conjugate vaccine (PCV13) serotype CAP, and 20-valent pneumococcal conjugate vaccine (PCV20) serotype CAP incidences per 100,000 population were 1,280, 227, 63, and 110, respectively. <em>S pneumoniae</em> was detected in 17.8% of all patients with CAP by any detection method, with 4.9%, 5.5%, and 8.6% of cases of CAP resulting from PCV13, 15-valent pneumococcal conjugate vaccine, and PCV20 serotypes, respectively. Applying Goto’s incidence and case fatality rate to the Japanese population, assuming PCV20 has the same vaccine efficacy and duration of protection as PCV13 and if licensed in Japan for the prevention of CAP, the inclusion of PCV20 in the national immunization program for adults ≥ 65 years of age could prevent 29,036 cases of CAP and 2,275 CAP-related deaths per year.</p></div><div><h3>Interpretation</h3><p>Given the substantial burden of preventable pneumococcal disease, introduction of pneumococcal conjugate vaccines in Japanese adults may be of merit.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49899667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-07-18DOI: 10.1016/j.chpulm.2023.100010
Michael G. Risbano MD, MA, FCCP , Corrine R. Kliment MD, PhD , Daniel G. Dunlap MD , Carl Koch MD , Luiz Campedelli MD , Karla Yoney PA-C , S. Mehdi Nouraie MD, PhD , Frank Sciurba MD , Alison Morris MD
{"title":"Invasive Cardiopulmonary Exercise Testing Identifies Distinct Physiologic Endotypes in Postacute Sequelae of SARS-CoV-2 Infection","authors":"Michael G. Risbano MD, MA, FCCP , Corrine R. Kliment MD, PhD , Daniel G. Dunlap MD , Carl Koch MD , Luiz Campedelli MD , Karla Yoney PA-C , S. Mehdi Nouraie MD, PhD , Frank Sciurba MD , Alison Morris MD","doi":"10.1016/j.chpulm.2023.100010","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100010","url":null,"abstract":"<div><h3>Background</h3><p>Exercise intolerance and dyspnea on exertion are prominent symptoms in postacute sequelae of COVID-19 (PASC) that significantly impact quality of life, but underlying physiologic contributors are not well understood. Invasive cardiopulmonary exercise testing (iCPET) uses right heart catheterization, arterial access, and standard cardiopulmonary exercise testing to identify pathophysiology in undifferentiated exercise intolerance.</p></div><div><h3>Research Question</h3><p>What are the distinguishing clinical and physiologic features of exercise intolerant PASC and associated physiologic endotypes?</p></div><div><h3>Study Design and Methods</h3><p>We performed a cross-sectional observational study with prospective enrollment of consecutive patients with dyspnea and/or exercise-intolerant PASC who were referred for iCPET > 3 months after SARS-CoV-2 infection. All patients underwent history, physical examination, pulmonary function testing, echocardiogram, laboratory work, and chest imaging. We completed a detailed cardiopulmonary and hemodynamic analysis of 37 patients with iCPET to categorize PASC endotypes.</p></div><div><h3>Results</h3><p>We evaluated 37 patients with PASC and dyspnea on exertion and/or exercise intolerance for a median of 323 days after COVID-19 diagnosis. We compared 12 patients (32.4%) who demonstrated a normal exercise capacity (normal oxygen consumption [<span><math><mrow><mover><mtext>V</mtext><mo>˙</mo></mover></mrow></math></span><span>o</span><sub>2</sub>]) with 25 patients (67.6%) that had a reduced peak <span><math><mrow><mover><mtext>V</mtext><mo>˙</mo></mover></mrow></math></span><span>o</span><sub>2</sub>. We then identified distinct PASC endotypes with iCPET including preload insufficiency, decreased oxygen extraction, mixed preload insufficiency with decreased oxygen extraction, exercise pulmonary hypertension, chronic pulmonary embolism, deconditioning, and ventilatory limitation, which included physiologic abnormalities in patients with PASC with normal exercise capacity. Nine of the 12 patients with normal <span><math><mrow><mover><mtext>V</mtext><mo>˙</mo></mover></mrow></math></span><span>o</span><sub>2</sub> had normal exercise physiology (one had evidence of exercise pulmonary hypertension and two had decreased oxygen extraction) yet still presented with symptoms of exercise intolerance.</p></div><div><h3>Interpretation</h3><p>iCPET identified heterogeneous physiologic endotypes in patients with PASC presenting with similar symptoms of exercise intolerance or dyspnea on exertion. Future studies are needed to define associated pathogenesis and target effective therapies based on these physiologic endotypes.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49703112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-06-01DOI: 10.1016/j.chpulm.2023.100006
Ramzi Ibrahim MD , Coco Victoria Gomez Tirambulo MPH , Rebecca Wig MBBS , Roger Y. Kim MD, MSCE , Bryan S. Benn MD, PhD , See-Wei Low MD
{"title":"Impact of the Social Vulnerability Index on Pulmonary Embolism Mortality","authors":"Ramzi Ibrahim MD , Coco Victoria Gomez Tirambulo MPH , Rebecca Wig MBBS , Roger Y. Kim MD, MSCE , Bryan S. Benn MD, PhD , See-Wei Low MD","doi":"10.1016/j.chpulm.2023.100006","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100006","url":null,"abstract":"","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49703045","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-06-01DOI: 10.1016/j.chpulm.2023.100005
Kierstin Hederstedt BS , Nathan Mesfin MD , Mikhail C.S.S. Higgins MD, MPH , Daniel J. Sturgeon MS , Westyn Branch-Elliman MD, MMSc , Marva V. Foster PhD, RN , Hillary J. Mull PhD, MPP
{"title":"Variation in Incidence of Pneumothorax Complications After Percutaneous Lung Biopsies Among Veterans Health Administration Hospitals","authors":"Kierstin Hederstedt BS , Nathan Mesfin MD , Mikhail C.S.S. Higgins MD, MPH , Daniel J. Sturgeon MS , Westyn Branch-Elliman MD, MMSc , Marva V. Foster PhD, RN , Hillary J. Mull PhD, MPP","doi":"10.1016/j.chpulm.2023.100005","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100005","url":null,"abstract":"","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49703051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-06-01DOI: 10.1016/j.chpulm.2023.100003
Amanda Grant-Orser MBBCh , Nicola A. Adderley MD , Katelyn Stuart , Charlene D. Fell MD , Kerri A. Johannson MD, MPH
{"title":"Patient and Physician Assessments of Clinical Status","authors":"Amanda Grant-Orser MBBCh , Nicola A. Adderley MD , Katelyn Stuart , Charlene D. Fell MD , Kerri A. Johannson MD, MPH","doi":"10.1016/j.chpulm.2023.100003","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100003","url":null,"abstract":"<div><h3>Background</h3><p>The SARS-CoV-2 pandemic necessitated novel health care delivery for patients with interstitial lung disease (ILD), including reduced in-person appointments and physiologic testing to minimize transmission. Clinicians often have been required to rely on patients’ subjective assessments of their clinical status during phone follow-up appointments. It is unknown how accurate a patient’s self-assessment is compared with that of their physician during an in-person evaluation.</p></div><div><h3>Research Question</h3><p>Are patients’ self-assessments of their clinical status in agreement with their physicians’ assessments, and are telemedicine vs in-person visits acceptable?</p></div><div><h3>Study Design and Methods</h3><p>Patients were enrolled prospectively from the University of Calgary ILD clinic. Participants were asked by phone before the in-person appointment and after the appointment to rate their clinical status on a five-point Likert scale. Physicians then rated the patient’s clinical status after the appointment on a similar five-point Likert scale, masked to patient responses. Patients and physicians were asked if an in-person appointment was necessary or if telemedicine would have sufficed. Clinical variables associated with physician assessments were assessed.</p></div><div><h3>Results</h3><p>Fifty patients with mean age of 67 ± 11.8 years participated. Mean time since last follow-up was 5.0 ± 3.0 months. No correlation was found between the preclinical patient self-assessment and postclinical physician assessment (<em>P</em> = .18; κ = 0.28). Correlation of postclinical assessment was statistically significant (<em>P</em> < .001), with moderate agreement (κ = 0.49). Physicians thought telephone visits were acceptable for 58% of appointments, whereas only 12% of patients preferred telephone visits. Physician’s assessment of clinical status seemed to be driven by change in diffusion capacity of the lungs for carbon monoxide (<em>P</em> = .039).</p></div><div><h3>Interpretation</h3><p>Telemedicine may improve access to care for patients during pandemic management, in rural communities, and for those with impaired mobility. Despite these benefits, our data support that patients and physicians may not agree on determination of clinical status and that patients generally prefer in-person patient-physician interactions.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49703081","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-06-01DOI: 10.1016/j.chpulm.2023.100004
Michael Glenn O’Connor MD , Ricardo Mosquera MD , Hilda Metjian MD , Meghan Marmor MD , Kenneth N. Olivier MD, MPH , Adam J. Shapiro MD
{"title":"Primary Ciliary Dyskinesia","authors":"Michael Glenn O’Connor MD , Ricardo Mosquera MD , Hilda Metjian MD , Meghan Marmor MD , Kenneth N. Olivier MD, MPH , Adam J. Shapiro MD","doi":"10.1016/j.chpulm.2023.100004","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100004","url":null,"abstract":"<div><p>Primary ciliary dyskinesia (PCD) is a rare but underdiagnosed disorder that affects motile cilia function throughout the body. With increasing prevalence through ongoing genetic discovery, PCD underlies the disease process in a significant number of patients with chronic suppurative lung disease and bronchiectasis when properly investigated using current diagnostic standards. Classic PCD symptoms include chronic rhinosinusitis and otitis, organ laterality defects, infertility, year-round productive cough, and recurrent pneumonias with bronchiectasis. Clinical symptoms of PCD manifest very early in life (often at birth), although diagnosis frequently is delayed because of poor phenotypic recognition and limited access to specialized diagnostic testing. In the past decade, PCD research networks have established specific PCD phenotypes to increase clinical recognition, and the availability of PCD genetic panels in various commercial laboratories has expanded access to an accurate PCD diagnosis greatly. Clinical practice guidelines also were created to guide diagnosis and management of this rare but increasingly recognized suppurative respiratory disease. PCD is more common than previously thought and can be recognized through specific clinical phenotypes in both children and adults. Diagnostic PCD testing outside of highly specialized centers can be difficult, but increased availability of nasal nitric oxide measurement and commercial genetic panels now allows for noninvasive screening and definitive diagnosis regardless of center expertise. Identification of patients with accurately diagnosed PCD is needed worldwide to populate future clinical trials and to develop disease-specific therapies for PCD.</p></div>","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49703082","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
CHEST pulmonaryPub Date : 2023-06-01DOI: 10.1016/j.chpulm.2023.100001
Matthew C. Miles MD, MEd, FCCP
{"title":"Announcing the Launch of CHEST Pulmonary and the CHEST Portfolio of Journals","authors":"Matthew C. Miles MD, MEd, FCCP","doi":"10.1016/j.chpulm.2023.100001","DOIUrl":"https://doi.org/10.1016/j.chpulm.2023.100001","url":null,"abstract":"","PeriodicalId":94286,"journal":{"name":"CHEST pulmonary","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49703108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}