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COVID-19 in a patient with sporadic lymphangioleiomyomatosis awaiting lung transplantation.

ABSTRACT: Coronavirus disease 2019 (COVID-19) is an emerging viral disease with a mortality that depends on the individual's condition. Underlying comorbidities are major risk factors for COVID-19-related morbidity and mortality. However, information regarding the clinical course of COVID-19 in patients with rare respiratory system diseases is lacking. Here, we present a case of severe COVID-19 in a patient with advanced sporadic lymphangioleiomyomatosis (LAM) who was awaiting lung transplantation. She experienced a marked worsening of her respiratory status despite the limited size of the infiltrations seen on chest computed tomography. She responded to treatment with dexamethasone and remdesivir, and did not require mechanical ventilation. She recovered her pre-COVID-19 respiratory function. This case illustrates that patients with severe lung parenchymal destruction due to advanced LAM are at risk of worsening hypoxemia, but may not have a bad outcome if managed appropriately. Prevention and early diagnosis of COVID-19 are crucial in patients with advanced LAM. Future studies are needed to improve understanding of the clinical features and optimal treatment of COVID-19 in patients with LAM.

SUBMITTER: Noda Y

PROVIDER: S-EPMC8413486 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Lung transplantation for patients with severe COVID-19

Project description:Lung transplantation can potentially be a life-saving treatment for patients with non-resolving COVID-19-associated respiratory failure. Concerns limiting transplant include recurrence of SARS-CoV-2 infection in the allograft, technical challenges imposed by viral-mediated injury to the native lung, and potential risk for allograft infection by pathogens associated with ventilator-associated pneumonia in the native lung. Most importantly, the native lung might recover, resulting in long-term outcomes preferable to transplant. Here, we report results of the first successful lung transplantation procedures in patients with non-resolving COVID-19-associated respiratory failure in the United States. We performed sm-FISH to detect both positive and negative strands of SARS-CoV-2 RNA in the explanted lung tissue, extracellular matrix imaging using SHIELD tissue clearance, and single cell RNA-Seq on explant and warm post-mortem lung biopsies from patients who died from severe COVID-19 pneumonia. Lungs from patients with prolonged COVID-19 were free of virus but pathology showed extensive evidence of injury and fibrosis which resembled end-stage pulmonary fibrosis. We used a machine learning approach to project single cell RNA-Seq data from patients with late stage COVID-19 onto a single cell atlas of pulmonary fibrosis, revealing similarities across cell lineages. There was no recurrence of SARS-CoV-2 or pathogens associated with pre-transplant ventilator associated pneumonias following transplantation. Our findings suggest that some patients with severe COVID-19 develop fibrotic lung disease for which lung transplantation is the only option for survival.

2020-10-28 | GSE158127 | GEO

Lung Transplantation for Lymphangioleiomyomatosis in Japan.

Project description:BACKGROUND:Lung transplantation has been established as the definitive treatment option for patients with advanced lymphangioleiomyomatosis (LAM). However, the prognosis after registration and the circumstances of lung transplantation with sirolimus therapy have never been reported. METHODS:In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network. RESULTS:Transplantation was performed in 57 patients as of March 2014. Survival rate was 86.7% at 1 year, 82.5% at 3 years, 73.7% at 5 years, and 73.7% at 10 years. Of the 98 patients, 21 had an inactive status and received sirolimus more frequently than those with an active history (67% vs. 5%, p<0.001). Nine of twelve patients who remained inactive as of March 2014 initiated sirolimus before or while on a waiting list, and remained on sirolimus thereafter. Although the statistical analysis showed no statistically significant difference, the survival rate after registration tended to be better for lung transplant recipients than for those who awaited transplantation (p = 0.053). CONCLUSIONS:Lung transplantation is a satisfactory therapeutic option for advanced LAM, but the circumstances for pre-transplantation LAM patients are likely to alter with the use of sirolimus.

| S-EPMC4714890 | biostudies-literature

Hemodynamic effects of COVID-19 vaccination in hospitalized patients awaiting heart transplantation.

Project description:BackgroundThe hemodynamic effects of pre-transplant vaccination against COVID-19 among heart transplant candidates hospitalized for advanced heart failure remains unknown.MethodsA retrospective chart review was conducted at a high-volume transplant center from January through December 2021. 22 COVID-19 vaccination events occurred among patients hospitalized for decompensated heart failure while awaiting transplantation. Primary outcomes included inotrope and vasopressor dosages. Secondary outcomes included vital signs, pulmonary artery catheter measurements, diuretic dosages, and renal function. Data were extracted 24 h before through 72 h after vaccination.ResultsOne of 22 vaccination events was associated with hemodynamic changes requiring increased inotropic and vasopressor support post-vaccination. In all other cases, transient hemodynamic changes occurred without need for escalated therapy.ConclusionsCOVID-19 vaccination can be administered safely to most critically ill patients with advanced heart failure including those awaiting transplantation. All patients should be monitored closely as some may be susceptible to significant hemodynamic changes.

| S-EPMC9252923 | biostudies-literature

Sympatho-Vagal Dysfunction in Patients with End-Stage Lung Disease Awaiting Lung Transplantation.

Project description:Although the literature demonstrates that cardiac autonomic control (CAC) might be impaired in patients with chronic pulmonary diseases, the interplay between CAC and disease severity in end-stage lung disease has not been studied yet. We investigated the effects of end-stage lung disease on CAC through the analysis of heart rate variability (HRV) among patients awaiting lung transplantation. Forty-nine patients on the waiting list for lung transplantation (LTx; 19 men, age 38 ± 15 years) and 49 healthy non-smoking controls (HC; 22 men, age 40 ± 16 years) were enrolled in a case-control study at Policlinico Hospital in Milan, Italy. LTx patients were divided into two groups, according to disease severity evaluated by the Lung Allocation Score (LAS). To assess CAC, electrocardiogram (ECG) and respiration were recorded at rest for 10 min in supine position and for 10 min during active standing. Spectral analysis identified low and high frequencies (LF, sympathetic, and HF, vagal). Symbolic analysis identified three patterns, i.e., 0V% (sympathetic) and 2UV% and 2LV% (vagal). Compared to HCs, LTx patients showed higher markers of sympathetic modulation and lower markers of vagal modulation. However, more severely affected LTx patients, compared to less severely affected ones, showed an autonomic profile characterized by loss of sympathetic modulation and predominant vagal modulation. This pattern can be due to a loss of sympathetic rhythmic oscillation and a subsequent prevalent respiratory modulation of heart rate in severely affected patients.

| S-EPMC7230240 | biostudies-literature

Risk factors for death of patients with cystic fibrosis awaiting lung transplantation.

Project description:RationaleThe optimal timing for listing of cystic fibrosis patients for lung transplantation is controversial.ObjectivesWe conducted a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers to identify risk factors for death while awaiting transplantation.MethodsData on possible risk factors were abstracted from medical records.MeasurementsTime to death, patient demographic characteristics, and risk factors for death while awaiting transplantation were assessed. Univariate and multivariate survival analyses were performed using Cox regression.ResultsBy univariate analyses, FEV1 < or = 30% predicted (HR, 3.8; 95% CI, 2.0-7.5), Pa(CO2) > or = 50 mm Hg (HR, 1.85; 95% CI, 1.1-3.0), and shorter height (HR, 1.8; 95% CI, 1.1-3.0) were associated with a higher risk of death. Referral from an accredited cystic fibrosis center was associated with a lower risk (HR, 0.53; 95% CI, 0.30-0.92). The final multivariate model included referral from an accredited cystic fibrosis center (HR, 0.5; 95% CI, 0.3-1.0) and listing year after 1996 (HR, 0.4; 95% CI, 0.2-0.7); both were associated with a lower risk of death. FEV1 < or = 30% predicted (HR, 6.8; 95% CI, 2.4-19.3), Pa(CO2) > or = 50 mm Hg (HR, 6.9; 95% CI, 1.5-32.1), and use of a nutritional intervention (HR, 2.3; 95% CI, 1.3-4.1) were associated with increased risk. Patients with FEV1 > 30% predicted had a higher risk of death only when their Pa(CO2) was > or = 50 mm Hg (HR, 7.0; 95% CI, 1.5-32), while the increased risk of death with FEV1 < or = 30% was not further influenced by the presence of hypercapnia.ConclusionsWe identified risk factors for waiting list mortality that could impact on transplant listing and allocation guidelines.

| S-EPMC2662949 | biostudies-literature

Lung transplantation for patients with severe COVID-19

Project description:Lung transplantation for patients with severe COVID-19

| PRJNA664057 | ENA

Lung transplantation for patients with severe COVID-19.

Project description:Lung transplantation can potentially be a life-saving treatment for patients with nonresolving COVID-19-associated respiratory failure. Concerns limiting lung transplantation include recurrence of SARS-CoV-2 infection in the allograft, technical challenges imposed by viral-mediated injury to the native lung, and the potential risk for allograft infection by pathogens causing ventilator-associated pneumonia in the native lung. Additionally, the native lung might recover, resulting in long-term outcomes preferable to those of transplant. Here, we report the results of lung transplantation in three patients with nonresolving COVID-19-associated respiratory failure. We performed single-molecule fluorescence in situ hybridization (smFISH) to detect both positive and negative strands of SARS-CoV-2 RNA in explanted lung tissue from the three patients and in additional control lung tissue samples. We conducted extracellular matrix imaging and single-cell RNA sequencing on explanted lung tissue from the three patients who underwent transplantation and on warm postmortem lung biopsies from two patients who had died from COVID-19-associated pneumonia. Lungs from these five patients with prolonged COVID-19 disease were free of SARS-CoV-2 as detected by smFISH, but pathology showed extensive evidence of injury and fibrosis that resembled end-stage pulmonary fibrosis. Using machine learning, we compared single-cell RNA sequencing data from the lungs of patients with late-stage COVID-19 to that from the lungs of patients with pulmonary fibrosis and identified similarities in gene expression across cell lineages. Our findings suggest that some patients with severe COVID-19 develop fibrotic lung disease for which lung transplantation is their only option for survival.

| S-EPMC8050952 | biostudies-literature

A narrative review on the management of patients awaiting lung transplantation in Japan.

Project description:The number of lung transplantations performed in Japan is increasing, and post-transplant outcomes are relatively favorable. A major concern is the extremely long waiting time and the high mortality rate on the waitlist. The management of patients before transplantation is very important and essential for further improvement of outcomes. In this review, we summarize the management of patients awaiting lung transplantation in Japan. A literature search was conducted via PubMed in November 2022 using the following keywords: lung transplantation, waiting, management, recipient, and Japan. Peer-reviewed academic journal articles published in English were also included. A growing number of studies have evaluated the management of pre-transplant patients. This includes infection control, vaccination, respiratory disease-specific treatment, malignancy, nutrition, rehabilitation, psychosocial assessment, and health-related quality of life (HRQOL) for patients on the waitlist. Each is important, and various evaluation methods and strategies to improve outcomes have been reported. Proper and multifaceted management of pretransplant patients is extremely important to reduce the mortality of candidates for lung transplantation. Integrated management is crucial to the success and survival of lung transplant recipients.

| S-EPMC10636442 | biostudies-literature

COVID-19 patient samples collected by the PREDICT-19 consortium

Project description:To investigate the role of gene expression in patients with COVID-19. Full article available at DOI: 10.1136/bmjopen-2020-044497.

2023-01-30 | GSE217948 | GEO

Patient Perceptions of COVID-19-Related Surgical Delay: An Analysis of Patients Awaiting Total Hip and Knee Arthroplasty.

Project description:BackgroundCOVID-19 has caused unprecedented delays in elective orthopedic surgery. Understanding patients' perceptions of the disruptions in care and their willingness to reengage the healthcare system are crucial to planning the resumption of elective care.Questions/purposesThe purpose of this study was to elicit patient perceptions about delays in total joint arthroplasty during the COVID-19 pandemic.MethodsWe identified a consecutive series of patients who experienced COVID-19-driven delays to scheduled total hip or knee arthroplasty at an urban, academic medical center in the Southeastern United States. A 20-item survey was administered via telephone. Answers were recorded and descriptive statistics were performed. A post hoc χ-square analysis compared characteristics and outlooks of patients who did and did not immediately desire surgery.ResultsOf 111 patients (64% of those identified) who met inclusion criteria and completed the survey, 96% said they felt that they were treated fairly and 90% said that the surgical delay was in their best interest; 68% reported emotional distress from the delay, but 45% reported a desire to wait longer for the pandemic to subside. Lower joint-function scores, higher pain levels, higher pain catastrophizing scores, and longer latency from personally deciding to pursue surgery were associated with the reported need for immediate surgery.ConclusionOverall, patients reported that they understood the need for elective surgical delays during the COVID-19 pandemic. However, the psychological implications they reported were not negligible. Patient preference for immediate reengagement with the healthcare system was dichotomous, with many patients favoring precautionarily furthering the delay. Understanding these preferences will help optimize elective orthopedic care during unprecedented times.

| S-EPMC7491018 | biostudies-literature

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