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RATIONALE: Many lung transplant recipients with cystic fibrosis (CF) have low preoperative body mass index (BMI); however, post-transplant BMI recovery is not well understood. OBJECTIVES: To evaluate BMI recovery (⩾18.5 kg/m(2)) among CF lung transplant recipients with low preoperative BMI and to investigate the association of survival with BMI recovery. METHODS: The United Network for Organ Sharing and CF Foundation patient registries (June 2005–December 2016) were used to identify CF lung transplant recipients. Among recipients surviving ⩾1 year, Cox modeling compared post-transplant 1-year conditional survival between recipients with low (

In 2017, Nkam et al. published a prognostic score to predict death or lung transplant within 3 years among adult cystic fibrosis (CF) patients. Their model was developed using French CF registry data and was subsequently validated in the Canadian CF registry. We evaluated this prognostic score using data from adult patients with CF in the United States (US) CF Foundation Patient registry, combined with lung transplant records from the United Network for Organ Sharing (UNOS) Registry (2013 to 2016) (n=11,542). We found that the prognostic score had a very good discriminative index predicting death or lung transplant in the US CF population (AUC 0.88, 95% CI 0.88-0.89) with an odds ratio (OR) of 2.83 (95% CI 2.69 – 2.97) for each unit increase in the score. However, it did not provide significant additional utility over an FEV1 ≤30% of predicted as a predictor of death or lung transplant.

RATIONALE: Low body mass index (BMI) may influence lung transplant decisions for patients with advanced cystic fibrosis (CF) lung disease. OBJECTIVE: Determine whether patients with advanced CF lung disease and BMI ≤17 kg/m(2) are less likely to be listed for lung transplant or have a higher risk of death without listing compared to those with higher BMI. METHODS: Using merged United Network for Organ Sharing and CF Foundation Patient Registries, we identified adults with onset of advanced lung disease (FEV(1) ≤ 40% predicted) between May-2005 and December-2016. We analyzed survival using competing risks regression with cause-specific risks of listing for lung transplant and death without listing. BMI ≤ 17 kg/m(2) was our predictor. MEASUREMENTS AND MAIN RESULTS: Among 5,121 CF patients with advanced lung disease, 23% were listed for lung transplant (n=1,201), 23% died without listing (n=1,190), and 44% were alive without listing (n=2,730) as of December-2016. Patients with BMI ≤ 17 kg/m(2) were less likely to be listed for transplant (HR 0.69; 95% CI 0.57, 0.83) and more likely to die without listing (HR 1.63; 95% CI 1.41, 1.88). We identified important regional variations in the likelihood of referral and listing, based on BMI. CONCLUSIONS: Patients with advanced CF lung disease and BMI ≤ 17 kg/m(2) are less likely to be listed for lung transplant and have a higher risk of dying without listing, compared to those with higher BMI. Regional differences suggest access to transplant for malnourished CF patients may be limited by location.

Background Low muscle mass is common in patients approaching lung transplantation and may be linked to worse post-transplant outcomes. Existing studies assessing muscle mass and post-transplant outcomes include few patients with cystic fibrosis (CF). Methods Between May 1993 and December 2018, 152 adults with CF received lung transplants at our institution. Of these, 83 met inclusion criteria and had usable computed tomography (CT) scans. Using Cox proportional hazards regression, we evaluated the association between pre-transplant thoracic skeletal muscle index (SMI) and our primary outcome of death after lung transplantation. Secondary outcomes, including days to post-transplant extubation and post-transplant hospital and intensive care unit (ICU) length of stay, were assessed using linear regression. We also examined associations between thoracic SMI and pre-transplant pulmonary function and 6-min walk distance. Results Median thoracic SMI was 26.95 cm2/m2 (IQR 23.97, 31.32) for men and 22.83 cm2/m2 (IQR 21.27, 26.92) for women. There was no association between pre-transplant thoracic SMI and death after transplant (HR 1.03; 95% CI 0.95, 1.11), days to post-transplant extubation, or post-transplant hospital or ICU length of stay. There was an association between pre-transplant thoracic SMI and pre-transplant FEV1% predicted (b = 0.39; 95% CI 0.14, 0.63), with higher SMI associated with higher FEV1% predicted. Conclusions Skeletal muscle index was low for men and women. We did not identify a significant relationship between pre-transplant thoracic SMI and post-transplant outcomes. There was an association between thoracic SMI and pre-transplant pulmonary function, confirming the potential value of sarcopenia as a marker of disease severity.

Background Chronic pain is common among people living with cystic fibrosis (CF) and associated with worse clinical outcomes. Despite this, little is known about how pain is managed and how opioids are used to treat pain. The purpose of this convergent mixed methods study was to examine self-reported satisfaction and effectiveness of pain management strategies among a sample of adults with CF who are prescribed opioids. Methods We developed an online survey querying 4 domains - demographics, pain characteristics, pain communication, and management strategies. This was distributed nationally to adults with CF (n=48) via various online platforms. We obtained quantitative and qualitative responses regarding satisfaction and effectiveness of pain management. Emerged themes from qualitative data were compared with responses from quantitative survey domains. Results Participants reported high levels of satisfaction and effectiveness with their opioid pain management plans. However, qualitative themes emerged regarding fears of addiction, experiences of feeling stigmatized by the healthcare system and ineffectiveness and inefficiency of alternative therapies for adequate pain relief. Conclusions Adults with CF reported opioids as an important component of their current pain management plans despite risks associated with opioid use. CF-specific pain management guideline development is warranted as is further research exploring pain development.

Tens of thousands of patients with advanced lung diseases may be eligible to be considered as potential candidates for lung transplant around the world each year. The timing of referral, evaluation, determination of candidacy, and listing of candidates continues to pose challenges and even ethical dilemmas. To address these challenges, the International Society for Heart and Lung Transplantation appointed an international group of members to review the literature, to consider recent advances in the management of advanced lung diseases, and to update prior consensus documents on the selection of lung transplant candidates. The purpose of this updated consensus document is to assist providers throughout the world who are caring for patients with pulmonary disease to identify potential candidates for lung transplant, to optimize the timing of the referral of these patients to lung transplant centers, and to provide transplant centers with a framework for evaluating and selecting candidates. In addition to addressing general considerations and providing disease specific recommendations for referral and listing, this updated consensus document includes an ethical framework, a recognition of the variability in acceptance of risk between transplant centers, and establishes a system to account for how a combination of risk factors may be taken into consideration in candidate selection for lung transplantation.

Epstein-Barr virus (EBV) seronegative solid organ transplant recipients (SOTRs) are at increased risk for post-transplant lymphoproliferative disorder (PTLD). Assays for EBV serostatus assess antibody to both EBV viral capsid antigen (VCA) and Epstein-Barr nuclear antigen-1 (EBNA-1), but PTLD risk among SOT recipients with discordant VCA and EBNA-1 results is unknown.We performed a retrospective, single-center cohort study to determine the risk of PTLD among adult (≥ 18 years) SOTRs with discordant pre-transplant VCA and EBNA-1 IgG compared to that of SOTRs with concordantly negative or concordantly positive serology using univariable and multivariable Cox-proportional hazards models.Of 4106 SOTRs, the number (%) who were concordantly positive, concordantly negative, and discordant was 3787 (92.2%), 149 (3.6%), and 170 (4.2%), respectively. The adjusted hazard of PTLD was significantly higher among discordant SOTRs compared to concordantly positive SOTRs (aHR 2.6, 95% CI 1.04-6.6, p =.04) and lower compared to concordantly negative SOTRs (aHR 0.27, 95% CI 0.10-0.76, p.001). The adjusted hazard of EBV+ PTLD among those with discordant serology was also significantly higher compared to the concordantly positive cohort (aHR 3.53, 95% CI 1.04-12.0, p =.04) and significantly lower compared to the concordantly negative cohort (aHR 0.23, 95% CI 0.06-0.82, p =.02).Risk of PTLD among SOTRs with discordant VCA and EBNA-1 may be intermediate between those with concordantly positive and negative serology. If confirmed in future studies, revision of national EBV serology reporting to include both VCA and EBNA results may be needed to optimize PTLD risk stratification.

As a rare complication after lung transplant, cardiac constriction should not be missed. Physical exam, echocardiography, and catheterization are essential for diagnosis A 65-year-old man with previous coronary artery disease and idiopathic pulmonary fibrosis underwent bilateral lung transplant and subsequently presented for progressive dyspnea and volume overload. Cardiac imaging and cardiac catheterization confirmed constriction, and complete pericardiectomy was performed. The patient had rapid resolution of heart failure symptoms. Pericardial constriction is a rare complication following lung transplant, and we provide a review of the literature and discussion of potential contributing factors. (Level of Difficulty: Intermediate.), Graphical abstract, As a rare complication after lung transplant, cardiac constriction should not be missed. Physical exam, echocardiography, and catheterization are…

Background Reduced BMI is an absolute contraindication for lung transplantation (LTx) at most centers in the United States. The objective of this study was to quantify post-LTx survival of moderate to severely underweight patients with cystic fibrosis (CF) (BMI Methods Using United Network for Organ Sharing Registry data (undergoing transplant from June 2005-November 2015), Kaplan-Meier estimates of median posttransplant survival were calculated for all patients with CF, COPD, and idiopathic pulmonary fibrosis (IPF), as well as low and normal weight CF subgroups. Cox regression modeling stratified according to transplant center assessed risk of posttransplant mortality in recipients with CF and a BMI Results Median posttransplant survival (95% CI) for CF, COPD, and IPF was 7.9 (7.2-8.6), 5.9 (5.6-6.2), and 5.5 (5.2-5.8) years, respectively. Although an absolute decrease was noted in posttransplant survival for recipients with CF and a BMI Conclusions Transplant recipients with CF and a BMI

Objective Lung transplant (LTx) saves lives in cystic fibrosis (CF). However, many potential candidates express uncertainty about LTx and die before receiving this treatment. CF guidelines recommend LTx education and clinical discussions well before the need for LTx arises, but limited patient resources exist. Materials and Methods We engaged people with CF and CF physicians in human-centered design of “Take On Transplant” (TOT), a web-based education tool to prepare patients for LTx discussions. Across 3 phases, needs assessment, design groups, and iterative user testing of TOT, we refined TOT from wireframe prototypes, to an interactive website, to a fully functional intervention ready for clinical trials. Results Fifty-five people with CF and 105 physicians identified information needs to prepare for LTx discussions. Design groups (n = 14 participants) then established core requirements: didactic education (“Resource Library”), patient narratives (“CF Stories”), frequently asked questions (“FAQ”), and self-assessment to tailor content (“My CF Stage”). Iterative usability testing (n = 39) optimized the design of CF Stories and prototype layout. We then developed the TOT website and demonstrated feasibility and preliminary efficacy of use through 2-week field testing (n = 9). Discussion Our human-centered design process provided guidance for educational tools to serve the evolving needs of potential LTx candidates. Our findings support the process of patient deliberation as a foundation for shared decision-making in CF, and inform educational tools that could potentially translate beyond LTx. Conclusion TOT fills a critical gap in preparing people with CF for shared decision-making about LTx and may serve as a model for educational tools for other preference-sensitive decisions.

Improved predictive models are needed in lung transplantation in the setting of a proposed allocation system that incorporates longer-term post-transplant survival in the United States. Allocation systems require accurate mortality predictions to justly allocate organs.Utilizing the United Network for Organ Sharing database (2005-2017), we fit models to predict 1-year mortality based on the Lung Allocation Score (LAS), the Chan, et al, 2019 model, a novel "clinician" model (a priori clinician selection of pre-transplant covariates), and two machine learning models (Least Absolute Shrinkage and Selection Operator; LASSO and Random Forests) for predicting 1-year and 3-year post-transplant mortality. We compared predictive accuracy among models. We evaluated the calibration of models by comparing average predicted probability vs observed outcome per decile. We repeated analyses fit for 3-year mortality, disease category, including donor covariates, and LAS era.The area under the cure for all models was low, ranging from 0.55 to 0.62. All exhibited reasonable negative predictive values (0.87-0.90), but the positive predictive value for was poor (all0.25). Evaluating LAS calibration found 1-year post-transplant estimates consistently overestimated risk of mortality, with greater differences in higher deciles. LASSO, Random Forests, and clinician models showed no improvement when evaluated by disease category or with the addition of donor covariates and performed worse for 3-year outcomes.The LAS overestimated patients' risk of post-transplant death, thus underestimating transplant benefit in the sickest candidates. Novel models based on pre-transplant recipient covariates failed to improve prediction. There should be wariness in post-transplant survival predictions from available models.

Improved methods are needed to risk-stratify patients with cystic fibrosis (CF) and reduced FEVWhat are the predictors of death or lung transplantation (LTx) within 2 years among patients with CF whose FEVPatients with CF, age ≥ 6 years with FEVFEVAmong individuals with CF and FEV

Lung transplantation (LTx) can be a life-extending treatment option for patients with advanced and/or progressive fibrotic interstitial lung disease (ILD), especially idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis, sarcoidosis and connective tissue disease-associated ILD. IPF is now the most common indication for LTx worldwide. Several unique features in patients with ILD can impact optimal timing of referral or listing for LTx, pre- or post-transplant risks, candidacy and post-transplant management. As the epidemiology of LTx and community practices have evolved, recent literature describes outcomes and approaches in higher-risk candidates. In this review, we discuss the unique and important clinical findings, course, monitoring and management of patients with IPF and other progressive fibrotic ILDs during pre-LTx evaluation and up to the day of transplantation; the need for co-management with clinical experts in ILD and LTx is emphasised. Some post-LTx complications are unique in these patient cohorts, which require prompt detection and appropriate management by experts in multiple disciplines familiar with telomere biology disorders and infectious, haematological, oncological and cardiac complications to enhance the likelihood of improved outcomes and survival of LTx recipients with IPF and other ILDs.

Chronic lung allograft dysfunction (CLAD) is the most important long-term complication after lung transplant (LTx), and clinical experience suggests significant variability in its management. We sought to capture azithromycin practices among LTx providers internationally. A survey was distributed via the International Society for Heart and Lung Transplantation and completed by 103 respondents (15 countries). Azithromycin indications, timing, and dosing varied significantly, and 37 (36%) reported inconsistency even within their center. Thirty (29%) reported initiating azithromycin prophylactically (during initial transplant hospitalization). Of 73 others, only 10 (14%) reported waiting until CLAD diagnosis (with persistent ≥20% pulmonary function decline). Most initiated azithromycin after a CLAD risk-factor/event, including 59 (81%) for a persistent ≥10% decrement in FEV 1 , 32 (44%) for lymphocytic bronchiolitis, and 27 (37%) for bronchoalveolar lavage neutrophilia . Azithromycin prescribing patterns appear to vary significantly, and further study is needed to elucidate the optimal timing and indications for its initiation after LTx.

Bronchoalveolar lavage (BAL) is a key clinical and research tool in lung transplantation (LTx). However, BAL collection and processing are not standardized across LTx centers. This International Society for Heart and Lung Transplantation-supported consensus document on BAL standardization aims to clarify definitions and propose common approaches to improve clinical and research practice standards. The following 9 areas are covered: (1) bronchoscopy procedure and BAL collection, (2) sample handling, (3) sample processing for microbiology, (4) cytology, (5) research, (6) microbiome, (7) sample inventory/tracking, (8) donor bronchoscopy, and (9) pediatric considerations. This consensus document aims to harmonize clinical and research practices for BAL collection and processing in LTx. The overarching goal is to enhance standardization and multicenter collaboration within the international LTx community and enable improvement and development of new BAL-based diagnostics. ispartof: JOURNAL OF HEART AND LUNG TRANSPLANTATION vol:39 issue:11 pages:1171-1190 ispartof: location:United States status: published

Skin cancer is the most common malignancy affecting solid organ transplant recipients (SOTR), and SOTR experience increased skin cancer-associated morbidity and mortality. There are no formal multidisciplinary guidelines for skin cancer screening after transplant, and current practices are widely variable. We conducted three rounds of Delphi method surveys with a panel of 84 U.S. dermatologists and transplant physicians to establish skin cancer screening recommendations for SOTR. The transplant team should risk stratify SOTR for screening, and dermatologists should perform skin cancer screening by full-body skin examination. SOTR with a history of skin cancer should continue regular follow-up with dermatology for skin cancer surveillance. High-risk transplant patients include thoracic organ recipients, SOTR age 50 and above, and male SOTR. High-risk Caucasian patients should be screened within 2 years after transplant, all Caucasian, Asian, Hispanic, and high-risk African American patients should be screened within 5 years after transplant. No consensus was reached regarding screening for low-risk African American SOTR. We propose a standardized approach to skin cancer screening in SOTR based on multidisciplinary expert consensus. These guidelines prioritize and emphasize the need for screening for SOTR at greatest risk for skin cancer.

Patients with connective tissues disease (CTD) are often on immunomodulatory agents before lung transplantation (LTx). Till now, there's no consensus on the safety of using these agents perioperative and post-transplant. The International Society for Heart and Lung Transplantation-supported consensus document on LTx in patients with CTD addresses the risk and contraindications of perioperative and post-transplant management of the biologic disease-modifying antirheumatic drugs (bDMARD), kinase inhibitor DMARD, and biologic agents used for LTx candidates with underlying CTD, and the recommendations and management of non-gastrointestinal extrapulmonary manifestations, and esophageal disorders by medical and surgical approaches for CTD transplant recipients.

While remarkable advances in cystic fibrosis (CF) care have led to improvements in survival and quality of life, many individuals with CF are living with advanced cystic fibrosis lung disease (ACFLD) and others will face continued disease progression and its associated complex treatments and choices. This review will provide a summary of recently published guidelines for ACFLD care and lung transplant referral and highlight ongoing work to enhance the care of those with ACFLD through improvements in medical and psychosocial care, palliative care, and care around lung transplantation.

With the rapidly expanding pandemic of SARS-CoV-2, there is concern that solid organ transplant recipients will be particularly vulnerable to infection and may experience a more severe clinical course. We report four cases of COVID-19 in solid organ transplant recipients including recipients of kidney, liver, lung, and heart transplants. We describe each patient's medical history including transplantation history, their clinical presentation and workup, and their course from diagnosis to either hospital discharge or to improvement in symptoms. These reports demonstrate a range of symptoms, clinical severity, and disease course in solid organ transplant recipients with COVID-19, including two hospitalized patients and two patients managed entirely in the outpatient setting.

Background Cystic fibrosis (CF) is a disease in which nutritional barriers are diverse and common, with malnutrition greatly influencing pulmonary trajectory and overall outcomes. Despite this, the most effective methods to optimize CF nutrition are unknown, and literature describing patients’ perspectives on their specific nutritional needs is lacking, particularly in the modern era of CF care. This study aimed to identify the most important nutritional needs and desired health-improvement resources in a contemporary adult CF cohort. Methods A 14-question investigator-designed survey addressing nutrition concerns, preferred health-improvement resources, and dietary/exercise routines was administered to CF adults. Clinical characteristics and survey responses are presented with descriptive statistics, and responses compared by body mass index (BMI) category (

Idiopathic Pulmonary Fibrosis (IPF) is a unique type of interstitial pneumonia in which progressive fibrosis can ultimately result in respiratory failure and death. The median survival of IPF remains dismal despite newer anti-fibrotic therapies. Lung transplantation is the only modality currently known to enhance survival for patients with IPF. Areas covered: Since IPF is predominantly a disorder of the elderly, determination of the impact of co-morbidities is crucial for risk stratification of the individual patient. We review the potential effect of anti-fibrotic therapy in the pre and post-transplant period; and also discuss the factors that need to be considered in deciding between single lung and bilateral lung transplantation. Expert commentary: Early referral to a transplant center is recommended for patients with IPF due to the high waiting list mortality. Evaluation of the transplant candidate should also be directed specifically at identifying co-morbidities that portend higher risk. While there has been a universal trend favoring bilateral lung transplantation over single lung transplantation for IPF, there are inherent pros and cons for both strategies and decisions should be individualized. Further studies are required to deduce the efficacy and safety of anti-fibrotic drugs in the immediate pre-and post-lung transplantation period.

First described as a disease of the pancreas, cystic fibrosis is a genetically inherited progressive disease affecting multiple organ systems. Pulmonary and pancreatic involvement is common in individuals with cystic fibrosis, and the former is attributable to most of the mortality that occurs with the condition. This chapter provides an overview of a clinical approach to the pulmonary and pancreatic manifestations of cystic fibrosis.

Background Epstein-Barr virus (EBV) donor positive (D+), recipient negative (R-) serostatus is a major risk factor for post-transplant lymphoproliferative disorder (PTLD) in adult solid organ transplant recipients (SOTR). Few studies have systematically characterized absolute organ transplant type-specific incidence, timing, and outcomes of PTLD in adult EBV D+R- SOTR. Methods We retrospectively assessed the incidence, timing, and associated morbidity and mortality of biopsy-confirmed PTLD (WHO classification) among consecutive adult SOTR between Jan 1, 2000 and Apr 30, 2018 at a single university center, and who had a minimum 2 years of follow-up. Antibodies to EBV (viral capsid antigen and nuclear antigen) were assessed in candidates and donors by FDA-cleared ELISA assay. Donors with unknown serology were considered seropositive based on the known prevalence of >93% seropositivity in this donor population. Results Among 4,923 SOTR, prior to transplant, 4,770 (96.9%) were R+ (regardless of donor status), 144 (2.9%) were D+R-, and 9 (0.2%) were D-R-. PTLD incidence by last follow-up was higher among D+R- (15/144 [10.4%]) than R+ (61/4,770 [1.2 %]), P < 0.0001, and occurred earlier after transplant (median 9.6 months [IQR 6.1-34.2] versus 35.1 months [IQR 7.1-73.1]), P= 0.003, respectively. Among D+R-, incidence was higher among recipients of non-kidney versus kidney organs (13/89 [14.6%] vs. 2/55 [3.6%], P = 0.05, respectively). Incidence in rank order was: pancreas (2/9 [22.2%]), lung (6/29 [20.7 %]), heart (2/21 [9.5%]), and liver (3/30 [6.7%]). PTLD histopathology was monomorphic in 9/15 [60%] and EBV-encoded RNA-1 (EBER-1) positive in 12/12 evaluable cases. Outcomes among the 15 PTLD cases included: graft failure without transplant in 3 (20%), graft failure with re-transplant in 2 (13.3%), and death within 6 months in 3 (20%). Table 1: Characteristics of the EBV Donor seropositive, Recipient seronegative (D+R-) cohort Figure 1: Cumulative Incidence of PTLD among the EBV D+R- cohort (all organs) Figure 2: Incidence of PTLD among the D+R- cohort, stratified by organ transplant type Conclusion Although rare overall, we identified a specific subgroup of adult SOT patients (EBV D+R- non-kidney recipients) whose absolute PTLD incidence and associated morbidity and mortality are high, and who should be targeted for future mechanistic or therapeutic studies. Disclosures All Authors: No reported disclosures

Background Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD. Methods The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate. Results The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues. Conclusions These recommendations are intended to be paired with previously published management guidelines for the overall CF population, with the objective of reducing practice variability and improving overall care, quality of life, and survival in those with ACFLD.

While lung transplantation is a potentially life-saving therapeutic option for many patients with end-stage pulmonary disease, overall outcomes remain inferior to recipients of other solid organ transplants. The immediate post-operative period presents a particularly high risk time for this extremely vulnerable population with a wide array of possible early complications including infection, rejection, pulmonary edema, primary graft dysfunction, bleeding, wound and airway complications, and venous thromboembolism. This chapter reviews important diagnostic considerations for lung transplant recipients with pulmonary infiltrates in the immediate post-operative period, specifically pertaining to infection, rejection, and pulmonary edema.

Lung transplantation has become a viable treatment option for end-stage lung disease. Common indications for lung transplantation are chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis, cystic fibrosis, alpha-1 antitrypsin deficiency, and pulmonary arterial hypertension. Either single or bilateral lung transplantation can be performed, but bilateral lung recipients appear to have a better median survival than single lung recipients. Complications after lung transplantation are common and may have nonspecific clinical and radiologic manifestations. The time point at which these complications occur relative to the date of transplant is crucial in formulating a differential diagnosis and recognizing them accurately. Significant advances in imaging techniques and recognition of air trapping in exhalation images and other patterns /distribution of parenchymal abnormalities have led to routine use of HRCT for diagnostic evaluation in patients manifesting respiratory decline in the lung transplant recipient.

Background Tracheal diverticula (TD) are rare anomalies that may harbor infected secretions, posing potential risk to patients with lung disease. In an end-stage cystic fibrosis (CF) cohort, we describe the characteristics and associated post-lung transplant (LTx) outcomes of TD. Methods Pre-transplant computed tomography (CT)'s were reviewed in CF patients undergoing LTx. TD were characterized radiographically and on autopsy when available. Pre-transplant clinical variables and post-transplant outcomes were compared by TD status. Results Of 93 patients, 35 (37.6%) had TD. 58% of TD had fat-stranding, and post-mortem TD examinations revealed histology carrying intense submucosal inflammation, and purulent contents that cultured identical species to sputum. There was no difference in post-LTx survival [HR 1.77 (0.82–3.82), p =0.147], bacterial re-colonization, or rejection in patients with TD compared to those without. Patients with TD were more likely to die from infection, but the result was not statistically significant [HR 2.02 (0.62–6.63), p =0.245]. Conclusions We found a high prevalence of TD in end-stage CF, where diverticula may represent a large-airway bacterial reservoir. TD were not associated with differences in post-LTx outcomes, but given the infectious concerns further investigation is necessary.

Purpose Real-time polymerase chain reaction (RT-PCR) detection of severe acute respiratory syndrome coronavirus (SARS-CoV-2) is required for diagnosis of coronavirus disease 2019 (COVID-19). Sensitivity of RT-PCR nasopharyngeal (NP) testing is presumed to be high, but there is no gold standard against which this has been determined. The objective was to determine whether lower respiratory tract infection (LRTI), detected in bronchoalveolar lavage fluid (BALF), occurs in the absence of upper respiratory tract infection with clinical testing of both specimen types. Methods Between March 26, 2020 and April 17, 2020 at the University of Washington Medical Center all patients with BALF specimens clinically tested for SARS-CoV-2 were identified. We assessed the proportion of patients with positive RT-PCR for SARS-CoV-2 in BALF after negative NP testing. We describe 3 cases with positive testing in BALF. Results Among 16 patients with BALF samples, 3 cases (19%) had SARS-CoV-2 detected in BALF. In Case 1, negative NP testing occurred early in the infection and respiratory symptoms may have been missed due to neurologic injury. In Case 2, outpatient diagnosis was aspiration pneumonia, but clinical suspicion remained high for COVID-19 at hospitalization based on epidemiological and clinical features. All 3 cases involved older adults (age >65 years), one of whom was immunosuppressed in the setting of lung transplantation (Case 3). Conclusions These data demonstrate that SARS-CoV-2 LRTI occurs in the presence of negative NP testing. NP testing may underestimate the prevalence of COVID-19 and has implications for spread of SARS-CoV2 in the community and healthcare setting., Highlights • Negative nasopharyngeal testing may underestimate the true prevalence of COVID-19. • COVID-19 lower respiratory tract infection occurs despite negative NP testing. • False negative nasopharyngeal testing has implications for the spread of COVID-19.

Malignant melanoma is the most aggressive form of skin cancer and carries a predisposition for metastasis to many different organs. Pulmonary dissemination is common, most often presenting as multiple discrete pulmonary nodules. While a variety of other intrathoracic patterns can occur, diffuse parenchymal infiltration causing acute respiratory failure is an extremely rare manifestation of metastatic disease. We present a case of an otherwise healthy man who developed rapidly progressive respiratory failure mimicking acute respiratory distress syndrome due to melanomatous infiltration of the lung parenchyma and airways.

A man in his 20s with a history of classical Hodgkin's lymphoma was admitted with fever. His original lymphoma diagnosis was made 3 years prior, when he had presented with lymphadenopathy and a mediastinal mass. He had relapsed disease despite chemotherapy and radiation. As a result, he underwent autologous peripheral blood stem cell transplant (SCT) 6 months prior to current presentation and subsequently allogeneic SCT 2 months prior for added graft vs tumor effect.

INTRODUCTION Mycobacterium abscessus infection in cystic fibrosis (CF) patients can lead to poor outcomes. Early diagnosis is important, but there are no studies outlining specific imaging features of M. abscessus in CF. OBJECTIVES To describe the computed tomography (CT) findings of early M. abscessus infection in our CF population. METHODS Thirteen CF patients with sputum cultures positive for M. abscessus from 2006 to 2013 were identified at our institution. Clinical characteristics including culture dates and lung function were reviewed. Positive cultures were classified as "disease" versus "colonization" based on published criteria. Chest CT scans were reviewed at times closest to initial infection, and features including bronchiectasis, mucous plugging, consolidation, ground glass opacities, nodules, and cavitation were evaluated. Brody scores were calculated to evaluate extent of CF lung disease. RESULTS All patients had bronchiectasis and mucous plugging, with 10 of 13 (76.9%) in an upper lobe distribution. Consolidation was seen in 12 of 13 (92.3%) patients, 8 (61.5%) patients had nodules, and 5 (38.5%) with cavitation. The average Brody score was 59.5, which was no different than previously described CF cohorts without M. abscessus. There were no significant differences between subjects with disease versus colonization. CONCLUSION The most common CT features of early M. abscessus in our CF population include bronchiectasis, mucus plugging, and consolidation, but the findings did not reveal a unique radiologic signature. CT at this initial time point may not distinguish early M. abscessus infection from background lung disease or mycobacterial colonization in CF patients.

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A 21-year-old woman with cystic fibrosis (CF) was seen in the pulmonary clinic complaining of abdominal pain. Her past medical history included bilateral lung transplantation for CF pulmonary disease 26 months previously, as well as gastroesophageal reflux disease and pancreatic insufficiency. Her baseline weight was 49.1 kg (BMI, 19.4 kg/m2).

Background Mycobacterium abscessus infection in patients with cystic fibrosis (CF) can result in accelerated clinical decline and the potential for direct or indirect transmission between patients has been recently demonstrated. Data on the outcomes of M abscessus outbreaks and the efficacy of specific infection control procedures in patients with CF remain limited. This study provides follow-up from an outbreak of pulmonary M abscessus in our center, highlighting outcomes and strategies that appear to have prevented further spread of the organism. Methods Data from our adult CF center (1989-2015) were analyzed, including chart reviews of all patients with positive mycobacterial sputum cultures, cultures from environment surfaces, and epidemiologic evaluation of infected patients. Following an M abscessus outbreak in 2009, infection control policies were intensified based on CF guidelines and surveillance data were collected and reviewed. Results Five cases of M abscessus were involved in the outbreak; 3 patients died during follow-up. An environment search failed to reveal an intermediary source of transmission between patients. After implementation of infection control measures composed of staff/patient education, environment sterilization, and patient isolation, no new cases were detected. Conclusions Direct or indirect patient-to-patient transmission of M abscessus is a threat in the CF population. A multifaceted infection control strategy based on CF guidelines was effective in halting transmission in our center.

The syndrome of systemic blastomycosis is well described, and has a predilection for involving the lungs, skin, bone, and genitourinary tract. We report a unique case of disseminated disease in an immunocompetent male, in which the presenting symptoms included focal intramuscular involvement. In this case, it was the discovery of the intramuscular abscess that led to the diagnosis of systemic blastomycosis. We provide a general discussion of this endemic mycosis, with focus on disseminated disease and the rare complication of skeletal muscle abscess.

Purpose Extubation failure is associated with adverse outcomes in mechanically ventilated patients, and it is believed that high rates of failed planned extubation (FPE) should be avoided. However, many believe that very low rates may also correlate with adverse outcomes if resulting from overly conservative weaning practices. We examined the relationship between the percentage of FPE (%FPE) and associated outcomes, with the aim of elucidating a favorable middle range. Methods A total of 1395 extubations were analyzed in mechanically ventilated subjects. Monthly %FPE values were separated into tertiles. Ventilator-free days (VFDs), intensive care unit–free days (IFDs), and mortality were compared among tertiles. Results Monthly %FPE tertiles were as follows: low, less than 7%; intermediate, 7% to 15%; and high, greater than 15%. There were significant differences in VFDs and IFDs by tertile from low to high (VFDs: low, 11.8; intermediate, 12.1; high, 9.9 [P = .003]; IFDs: low, 10.5; intermediate, 10.7; high, 9.0 [P = .033]). Post hoc comparisons demonstrated significant differences between the middle and high tertiles for both VFDs and IFDs. Conclusions Although exact rates may vary depending on setting, this suggests that a high %FPE (> 15) should be avoided in the intensive care unit and that there may be an intermediate range where ventilator outcomes are optimized.