Received Lung Transplantation Research Articles

Postoperative foot drop in patients receiving lung transplantation: increasing awareness and preventing risks

Postoperative foot drop in patients receiving lung transplantation: increasing awareness and preventing risks

Epidemiology and Prognosis of Invasive Fungal Disease in Chinese Lung Transplant Recipients.

This study explored the epidemiology, risk factors, and prognosis of invasive fungal disease (IFD) in Chinese lung transplant recipients (LTRs). This retrospective cohort study included patients who received lung transplants at four hospitals in South China between January 2015 and June 2019. The participants were divided into IFD and non-IFD (NIFD) groups. The final analysis included 226 LTRs (83.2% males) aged 55.0 ± 14.2 years old. Eighty-two LTRs (36.3%) developed IFD (proven or probable diagnosis). The most common pathogens were Aspergillus (57.3%), Candida (19.5%), and Pneumocystis jiroveci (13.4%). Multivariate logistic regression revealed that anastomotic disease [odds ratio (OR): 11.86; 95% confidence interval (95%CI): 4.76–29.54; P < 0.001], cytomegalovirus (CMV) pneumonia (OR: 3.85; 95%CI: 1.88–7.91; P = 0.018), and pre-transplantation IFD (OR: 7.65; 95%CI: 2.55–22.96; P < 0.001) were associated with higher odds of IFD, while double-lung transplantation (OR: 0.40; 95%CI: 0.19–0.79; P = 0.009) was associated with lower odds of IFD. Logistic regression analysis showed that anastomotic disease was associated with higher odds of death (OR: 5.01; 95%CI: 1.24–20.20; P = 0.02) and that PJP prophylaxis was associated with lower odds of death (OR: 0.01; 95%CI: 0.001–0.11; P < 0.001). Invasive fungal disease is prevalent among LTRs in southern China, with Aspergillus the most common pathogen. Prophylaxis should be optimized based on likely pathogens.

Mortality after Lung Transplantation for Children Bridged with Extracorporeal Membrane Oxygenation

Rationale: Extracorporeal membrane oxygenation (ECMO) is increasingly used to bridge children who are wait-listed and failing conventional respiratory support for lung transplantation. Objectives: To compare in-hospital mortality and a composite outcome of 1-year mortality or retransplantation in children bridged with ECMO, supported with mechanical ventilation (MV), and given neither support. Methods: The United Network for Organ Sharing was used to analyze lung transplant recipients aged ⩽20 years from January 2004 to August 2019. Recipients were categorized according to their degree of respiratory support at the time of transplant, including ECMO, MV, or neither. Multivariable analysis was used to evaluate support type and in-hospital mortality. Results: Of 1,014 children undergoing a lung transplant, 68 (6.7%) required ECMO as a bridge to transplant, 144 (14.2%) required MV, and 802 (79.1%) required neither. Primary diagnosis in the ECMO cohort included cystic fibrosis (43%), pneumonia and/or acute respiratory distress syndrome (10.3%), interstitial pulmonary fibrosis (7.4%), and pulmonary hypertension (5.9%). The number of patients bridged with ECMO increased throughout the study period from 0% in 2004 to 16.7% in 2018. Multivariable analysis showed bridging with both ECMO (adjusted odds ratio, 3.57; 95% confidence interval, 1.42-8.97) and MV (adjusted odds ratio, 2.67; 95% confidence interval, 1.26-5.57) increased in-hospital mortality after lung transplantation. However, there was no difference in composite outcome of mortality and retransplantation at 1 year between the three groups. Conclusions: ECMO to bridge children receiving lung transplantation has increased. Despite this, ECMO is a high-risk bridge strategy for children awaiting lung transplantation. Future research should target interventions that can be focused on improving survival in these patients.

Long-term outcomes of sensitized lung transplant recipients after peri-operative desensitization

The Toronto Lung Transplant Program has been using a peri-operative desensitization regimen of plasma exchange, intravenous immune globulin, and antithymocyte globulin in order to accept donor-specific antibody (DSA)-positive lung transplants safely since 2008. There are no long-term data on the impact of this practice on allograft survival or the development of chronic lung allograft dysfunction (CLAD). We extended our prior study to include long-term follow-up of 340 patients who received lung transplants between January 1, 2008 and December 31, 2011. We compared allograft survival and CLAD-free survival among patients in three cohorts: DSA-positive, panel reactive antibody (PRA)-positive/DSA-negative, and unsensitized at the time of transplant. The median follow-up time in this extension study was 6.7years. Among DSA-positive, PRA-positive/DSA-negative, and unsensitized patients, the median allograft survival was 8.4, 7.9, and 5.8years, respectively (p=.5908), and the median CLAD-free survival was 6.8, 7.3, and 5.7years, respectively (p=.5448). This follow-up study confirms that long-term allograft survival and CLAD-free survival of patients who undergo DSA-positive lung transplants with the use of our protocol do not differ from other lung transplant recipients. Use of protocols such as ours, therefore, may improve access to transplant for sensitized candidates.

Impact of bone-active drugs and underlying disease on bone health after lung transplantation: A longitudinal study

Introductionthe effect of bone-active drugs on the risk of fragility fractures (Fx), bone mineral density (BMD) and trabecular bone score (TBS) changes in patients receiving lung transplantation (LTx) is largely unknown. This study assessed the bone-active drugs effect in patients undergoing LTx both with (CF) and without (nCF) cystic-fibrosis. MethodsWe evaluated incident Fx, both clinical and morphometric vertebral Fx by spinal X-ray, BMD and trabecular bone score (TBS) in 117 patients (CF=50, nCF n = 67) before and 24-months after LTx. A bone-active therapy was proposed to all LTx candidates. Results83.8% of patients started a bone-active drug. Lumbar-spine (LS) T-score improved significantly only in treated patients (-1.4 ± 1.0 vs -2.0±1.0, p = 0.0001), whereas femur BMD and TBS remained stable in treated and not treated subjects. The rate of incident Fx was 15.3%, with no difference between treated and not treated patients. After LTx, LS T-score improved significantly only in nCF group (-1.3 ± 1.0 vs -1.8 ± 1.1, p = 0.0001), while femur remained stable in both nCF and CF groups. Patients with CF showed a significant Z-TBS increase (-3.6 ± 1.7 vs -3.0 ± 1.7, p = 0.019) and a lower Fx incidence as compared with nCF patients (4.1% vs 24.2%, p =0.003). Incident Fx were associated with nCF diagnosis (OR 7.300, CI95% 1.385–38.461, p = 0.019) regardless of prevalent Fx, previous glucocorticoid therapy and bone-active therapy introduced at least 6 months before LTx. ConclusionsA prompt medical intervention helps in preventing BMD loss after LTx. As compared with nCF patients, CF patients show a TBS increase and a lower Fx risk after LTx.

Hemodynamic and prognostic impact of the diastolic pulmonary arterial pressure in children with pulmonary arterial hypertension-a registry-based analysis.

Diastolic pulmonary arterial pressure (dPAP) is regarded to be less sensitive to flow metrics as compared to mean PAP (mPAP), and was therefore proposed for the assessment of a precapillary component in patients with postcapillary pulmonary hypertension (PH). To analyze the diagnostic and prognostic impact of dPAP in patients with pure precapillary PH, we purposed to compare the correlation between dPAP and mPAP, as well as hemodynamically-derived calculations [ratio of PAP to systemic arterial pressure (PAP/SAP), pulmonary vascular resistance index (PVRI), transpulmonary gradient (TPG)], using both dPAP and mPAP, at rest and during acute vasoreactivity testing (AVT) in children with idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH). Furthermore, we aimed to assess the association of these metrics (at baseline and changes after AVT) with transplant-free survival. We conducted a retrospective analysis of the TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) registry including 246 IPAH/HPAH patients. Of these, 45 children (18.3%) died, and 13 (5.3%) received lung transplantation during the observation period. dPAP and mPAP-derived variables showed almost linear relationship. Higher mPAP/mSAP, and dPAP-/mPAP-derived PVRI at rest was associated with time to death/transplantation. At maximum AVT-response, the decrease of dPAP and mPAP, diastolic pulmonary gradient (DPG) and TPG, as well as dPAP/dSAP and mPAP/mSAP was associated with time to death/transplantation, showing higher significance than corresponding baseline values. Remarkably, no predictive value was found for PVRI-reduction during AVT, neither dPAP- nor mPAP-derived. There is a strong relationship between dPAP and mPAP-derived variables. According to our results, hemodynamics during AVT (irrespectively of dPAP- or mPAP-derived) may have more prognostic implications than resting hemodynamics in children with IPAH/HPAH, except for PVRI.

Outcomes after lung transplantation among Chinese patients with connective tissue disease-associated interstitial lung disease and pulmonary hypertension: a retrospective cohort study.

The present study aimed to compare the post-lung transplant survival and complications of connective tissue disease (CTD)-related interstitial lung disease (ILD) and/or pulmonary arterial hypertension with idiopathic pulmonary fibrosis (IPF). The clinical data of patients with CTD-ILD or IPF who received lung transplantation between 2015 and 2020 were retrospectively reviewed. Cumulative survival rates after transplantation were estimated using the Kaplan-Meier method. The study included 31 patients with confirmed CTD-ILD and 98 with IPF. Patients with CTD-ILD were significantly younger (53.2 ± 13.7 vs. 62.3 ± 7.2 years, p=0.001) and more likely female (61.3% vs. 7.1%, p<0.001) than patients with IPF. No significant difference was noticed in the 1-year and 5-year survival rates between CTD-ILD and IPF patients (1-year, 73.2% vs 71.4%, p=0.76; 5-year, 69.1% vs. 39.5%, p=0.21). The incidence of primary graft dysfunction was significantly higher in CTD-ILD patients (90.3% vs. 70.4%, p=0.03), while there was no significant difference in primary graft dysfunction-related mortality (6.5% vs. 6.1%, p=0.95) between the two groups. There was no significant difference in post-lung transplant survival and complications between CTD-ILD and IPF.

Comparing outcomes in patients with end-stage chronic obstructive pulmonary disease: single versus bilateral lung transplants.

Debate continues on whether a bilateral (BLT) or a single lung transplantation (SLT) is preferred for patients with end-stage chronic obstructive pulmonary disease (COPD). The purpose of this study is to examine the interplay between patient age and transplant type on survival outcomes. We performed a retrospective study of lung transplants for COPD at our centre from February 2012 to March 2020 (n = 186). Demographics and clinical parameters were compared between patients based on their age (≤65 vs >65 years old) and type of transplant (single vs bilateral). Cox proportional hazards regression was also performed. P-values <0.05 were considered significant. Of the 186 patients with COPD who received lung transplants, 71 (38.2%) received BLTs and 115 (61.8%) received SLTs. There was no significant difference in survival outcomes when looking at patients with single versus BLTs (P = 0.870). There was also no difference in survival between the 2 age groups ≤65 versus > 65 years (P = 0.723). The Cox model itself also did not show a statistically significant improvement in survival outcomes (P = 0.126). Lung transplant outcomes in patients with end-stage COPD demonstrated non-inferior results in patients with an SLT compared to patients with a BLT. When we compared the age groups, neither transplant type showed superior survival benefits, suggesting there may be some utility in an SLT in younger recipients.

Racial/Ethnic Disparities in Receipt of Lung Transplant and In-Hospital Mortality among Pediatric and Adolescent Patients in the United States

The purpose of this study was to analyze racial/ethnic disparity in inpatient mortality among pediatric and adolescent lung transplant recipients (≤19 years old) in the United States (US). The Nationwide Inpatient Sample (NIS) dataset from 2003-2017 was used for this analysis. Survey logistic regression model was utilized to find the association between lung transplant and inpatient mortality among various racial/ethnic groups. The overall inpatient mortality rate was 3.92 per 100 lung transplant hospitalizations. Overall, when adjusted for covariates, those who received lung transplants were 8.49 times as likely to experience inpatient mortality (95% CI: 5.66-12.73) compared to those who did not receive it. The likelihood of inpatient mortality was highest among Hispanics (OR=10.11, 95% CI=4.93-14.95) relative to the findings among other racial/ethnic groups. Our findings may guide healthcare providers when caring for pediatric and adolescent populations from different racial/ethnic backgrounds who need a lung transplant. Copyright © 2021 Salihu, et al. Published by Global Health and Education Projects, Inc. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work, first published in this journal, is properly cited.

Outcomes of Lung Transplantation From Hepatitis C Viremic Donors

BackgroundDirect-acting antiviral (DAA) therapy for hepatitis C virus (HCV) has encouraged lung transplantation with HCV positive donors. Early trials have been promising; however, nationwide data have not been previously examined. MethodsThe United Network for Organ Sharing registry was queried for adult patients receiving lung transplants from 2016 to 2019. We excluded multiorgan transplants, incomplete data, and loss to follow-up. Nucleic acid testing (NAT) determined HCV status. Propensity matching was performed for comparison of outcomes. ResultsHepatitis C virus NAT-positive lungs were transplanted in 189 patients, compared with 9511 recipients of NAT-negative lungs. The HCV NAT-positive donors were younger (mean 33 vs 35 years, P = .017) with higher rates of Pao2/Fio2 greater than 300 (83.6% vs 76.5%, P = .029). Recipients of NAT-positive lungs had lower lung allocation scores (mean 39.3 vs 42.4, P = .009). Distance traveled was significantly further for HCV viremic donor lungs (mean 416 vs 206 miles, P < .001). Kaplan-Meier survival analysis demonstrated no difference in survival (P = .56). There were no differences in airway dehiscence (P = .629), acute rejection (P > .999), or reintubation (P = .304). At mean follow-up of 395 days, 63 recipients of NAT-positive lungs (40%) seroconverted, 14 with viremia. One-year mortality rates among seroconverted patients was 6% and did not differ significantly from 14% in nonseroconverted patients or 13.2% in recipients of HCV-negative lungs. ConclusionsShort-term outcomes of lung transplantation from HCV viremic donors are promising, with no difference in early complications or survival. The effects of seroconversion and long-term outcomes including chronic rejection and infection need to be further explored.

Epstein-Barr virus infection-related post-transplant lymphoproliferative disorders in transplanted lung: a clinicopathological analysis

Objective: To investigate the clinicpathological characteristics of post-transplant lymphoproliferative disorders (PTLD) in transplanted lung, and to improve its diagnosis and treatment. Methods: The clinicopathological characteristics of PTLD in three transplanted lungs were evaluated at Wuxi People's Hospital Affiliated to Nanjing Medical University from 2014 to 2019. HE, immunohistochemical staining and in situ hybridization were performed. The relevant literature of PTLD was reviewed. Results: All three patients had chronic obstructive pulmonary disease (COPD) before lung transplantation. After receiving both lung transplants, they were all treated with anti-rejection drugs tacrolimus or mycophenolate mofetil, and combined with antiviral and/or rituximab. The time from transplantation to diagnosis of PTLD was four years, seven months, and five months, respectively. Two patients died one month and five months after initial diagnosis, and one patient was alive with no disease after one year. Histologically, all cases were monomorphic B-cell PTLD (diffuse large B-cell lymphoma, unspecified), and the tumor cells were positive for Epstein-Barr virus by in situ hybridization; one of the late-onset patients had herpes simplex virus infection. Conclusions: PTLD in the post-transplant lung tissue shows unique morphology and clinical characteristics, and is closely related to Epstein-Barr virus infection. Patients who receive lung transplantation due to COPD are more susceptible to develop PTLD, while late-onset ones occur more commonly in the hilum of lungs, and the prognosis is relatively poor.

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

BackgroundWaitlist mortality due to donor shortage for lung transplantation is a serious problem worldwide. Currently, the selection of recipients in Japan is mainly based on the registration order. Hence, scientific evidence for risk stratification regarding waitlist mortality is urgently needed. We hypothesized that patient-reported dyspnea and health would predict mortality in patients waitlisted for lung transplantation.MethodsWe analyzed factors related to waitlist mortality using data of 203 patients who were registered as candidates for lung transplantation from deceased donors. Dyspnea was evaluated using the modified Medical Research Council (mMRC) dyspnea scale, and the health status was determined with St. George’s Respiratory Questionnaire (SGRQ).ResultsAmong 197 patients who met the inclusion criteria, the main underlying disease was interstitial lung disease (99 patients). During the median follow-up period of 572 days, 72 patients died and 96 received lung transplantation (69 from deceased donors). Univariable competing risk analyses revealed that both mMRC dyspnea and SGRQ Total score were significantly associated with waitlist mortality (p = 0.003 and p < 0.001, respectively) as well as age, interstitial lung disease, arterial partial pressure of carbon dioxide, and forced vital capacity. Multivariable competing risk analyses revealed that the mMRC and SGRQ score were associated with waitlist mortality in addition to age and interstitial lung disease.ConclusionsBoth mMRC dyspnea and SGRQ score were significantly associated with waitlist mortality, in addition to other clinical variables such as patients’ background, underlying disease, and pulmonary function. Patient-reported dyspnea and health may be measured through multi-dimensional analysis (including subjective perceptions) and for risk stratification regarding waitlist mortality.

Outcomes after Lung Re-Transplantation: A Single-Center Experience

<h3>Purpose</h3> Lung Re-transplantation (ReLTx) imposes an increased risk compared with a primary lung transplantatin (PLTx), and the median survival after ReLTx was reported only 2.5 years. Recently, some paper reported comparable result of ReLTx by adjustments for known transplantation risk factors. We investigated our results of ReLTx. <h3>Methods</h3> A retrospective data collection was performed for the 746 patients who received lung transplantation between Jan-2012 to Jun-2020 at our center. We compared variables between patients undergoing PLTx (Group P) and those undergoing ReLTx (Group R). Survival outcome was assessed using Kaplan-Meier curve and analyzed using log-rank test between the two groups. P-value <0.05 was considered significant (SAS Inc.). <h3>Results</h3> ReLTx was performed in 14 cases (1.9%). There was significant difference in age (p<0.001), lung allocation score (p=0.020) and hospital stay (p=0.025) (image 1). The diagnosis at the ReLTx were bronchiolitis obliterans syndrome in 9, bronchial stenosis in 2, emphysema in 2 and primary graft failure in 1. The types of 1^st transplantation and 2^nd transplantation were double (D) to single (S) in 5, D to D in 3, S to contralateral side S in 3, S to D in 2 and S to ipsilateral side S in 1. The mean duration between 1st transplantation and 2^nd transplantation was 2333.5 ± 2070.1 days (range: 31-6368). Kaplan-Meier curve showed no significant differences in survival between two groups (p=0.962) (image 2). <h3>Conclusion</h3> ReLTx showed non-inferior survival outcome compared with PLTx. In selected patients careful ReLTx is an acceptable option.

Impact of Pulmonary Artery Pressure on Survival Outcome of Single- and Double-Lung Transplantation in Chronic Obstructive Pulmonary Disease Patients

<h3>Purpose</h3> It remains unclear whether double lung transplantation (DLT) or single lung transplantation (SLT) is preferred for end-stage chronic obstructive pulmonary disease (COPD) patients. The purpose of this study is to examine the interplay between patient age and pulmonary artery pressure on survival after SLT vs DLT for COPD. <h3>Methods</h3> We performed a single-center retrospective study of lung transplantations for COPD between February 2012 to March 2020 (n=186). Demographics and clinical parameters were compared between patients based on their pulmonary artery pressure (PAP; PAP<25 mmHg vs PAP≥25 mmHg) and type of transplantation (single vs. double). Additionally, survival based on age (≤65 vs. >65 years old) and PAP were analyzed using Kaplan-Meier curves and log-rank tests. Cox proportional-hazards regression was also performed. P-value less 0.05 was considered significant. <h3>Results</h3> Of the 186 COPD patients who received lung transplants, 71 (38.2%) received double-lung transplants and 115 (61.8%) received single-lung transplants. Demographics and clinical parameters between the two PAP groups showed significance in: lung allocation score (p=0.002) and BMI at time of transplant (p<0.001). There was no significant difference in survival outcomes between SLT vs. DLT patients (p=0.870). Patients with elevated pulmonary artery pressures (PAP≥25 mmHg) did not have significantly different survival outcomes from patients with normal pressures (PAP<25 mmHg; p=0.16). There was also no difference in survival between the two age groups, ≤65 vs. >65 years (p=0.723). The Cox model itself, also, did not show a statistically significant improvement in evaluating survival outcomes (p=0.126). <h3>Conclusion</h3> Lung transplantation outcomes in end-stage COPD patients demonstrated non-inferior results with SLT compared to DLT. When patients were differentiated based on PA pressure, adopting SLT or DLT did not show any survival differences.

Electrical impedance tomography in pulmonary arterial hypertension.

The characterization of pulmonary arterial hypertension (PAH) relies mainly on right heart catheterization (RHC). Electrical impedance tomography (EIT) provides a non-invasive estimation of lung perfusion that could complement the hemodynamic information from RHC. To assess the association between impedance variation of lung perfusion (ΔZQ) and hemodynamic profile, severity, and prognosis, suspected of PAH or worsening PAH patients were submitted simultaneously to RHC and EIT. Measurements of ΔZQ were obtained. Based on the results of the RHC, 35 patients composed the PAH group, and eight patients, the normopressoric (NP) group. PAH patients showed a significantly reduced ΔZQ compared to the NP group. There was a significant correlation between ΔZQ and hemodynamic parameters, particularly with stroke volume (SV) (r = 0.76; P < 0.001). At 60 months, 15 patients died (43%) and 1 received lung transplantation; at baseline they had worse hemodynamics, and reduced ΔZQ when compared to survivors. Patients with low ΔZQ (≤154.6%.Kg) presented significantly worse survival (P = 0.033). ΔZQ is associated with hemodynamic status of PAH patients, with disease severity and survival, demonstrating EIT as a promising tool for monitoring patients with pulmonary vascular disease.

Physical function after lung transplantation for late-onset noninfectious pulmonary complications after allogeneic hematopoietic stem cell transplantation.

Late-onset noninfectious pulmonary complications (LONIPCs) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) are fatal, and lung transplantation is the only curative treatment. Although lung transplantation for LONIPCs may confer good survival rates, it is unclear whether or how impaired physical functioning is restored. Thus, this study aimed to investigate the long-term course and associated changes in physical functions after lung transplantation in patients with LONIPCs after allo-HSCT. This prospective cohort study enrolled 15 patients who received lung transplantation for LONIPCs after allo-HSCT between 2012 and 2018. Dyspnea scores, performance status, physical function, and exercise tolerance were assessed before lung transplantation and up to 2 years after transplantation. Two years after lung transplantation, the dyspnea scores and performance status improved, but did not recover completely. Physical function was assessed using the knee extensor strength (KES) and 6-min walk test (6MWT); the results were poor until 3 months after transplantation but improved over 2 years. The 6MWT distance showed improvement to a nearly healthy level (562.7 m). Recovery of exercise tolerance was associated with recovery in % vital capacity (%VC; r=0.5) and KES (r=0.4) from 3 months to 2 years after lung transplantation. Furthermore, a flat thorax, which is a characteristic of patients with LONIPCs, affected the %VC at 2 years after transplantation (r=0.8). Lung transplantation for LONIPCs may restore impaired physical function. A multifaceted rehabilitation program should be considered, especially to improve muscle weakness and pulmonary function.

Skin cancers in Italian lung transplant recipients: Incidence and risk factors analysis.

Only a few studies reported the incidence and risk factors of skin cancers in lung transplant recipients. The aim of this study was to determine the cumulative incidence of skin cancers in a cohort of patients undergoing lung transplantation and to define predictors of their development. About 247 consecutive patients receiving lung transplantation at the Thoracic Surgery Unit of University Hospital of Padova between May 1995 and October 2016 were studied. Cumulative incidence of skin cancers was estimated considering death as a competing event. The effect of potential predictors was evaluated with univariate and multivariable Cox models for competing risks. About 37 (15.0%) patients developed skin tumors. The cumulative incidence of any skin cancer was 14.2% at 5 years, 21.4% at 10 years, and 24.3% at 15 years posttransplantation. Age at transplantation, male gender, phototype II, and voriconazole use were independent risk factors for development of squamous cell carcinoma. Only male gender and phototype II were independent risk factors for development of basal cell carcinoma. Since lung transplant recipients have a greater risk of developing skin cancers, the management of these patients needs a multidisciplinary approach, in which dermatologists and transplant physicians have a primary role.

Nursing practice of extracorporeal membrane oxygenation combined with continuous renal replacement therapy in a severe COVID-19 patient with lung transplant

Objective: To summarize the nursing path of extracorporeal membrane oxygenation (ECMO) combined with continuous renal replacement therapy (CRRT) after lung transplantation for severe COVID-19, and to provide clinical references for improving the specialized nursing process and strengthening the specialized nursing technology. Methods: We retrospectively analyzed a case of severe COVID-19 who received lung transplantation in Renmin Hospital of Wuhan University. Results: In this case, the cumulative duration of ECMO treatment was up to 64 days, in which ECMO combined with CRRT was used to treat acute kidney injury and dehydration after lung transplantation for 3 days, and ECMO and CRRT treatment was successfully withdrawn without embolism, infection, and other complications. Conclusion: Active nursing practices of monitoring on ECMO and CRRT procedures are vital for the success of lung transplantation. © 2021, Editorial Board of Medical Journal of Wuhan University. All right reserved.

Application of extracorporeal membrane oxygenation in the perioperative period of lung transplantation

Objective: To investigate the application of extracorporeal membrane oxygenation (ECMO) in the perioperative period of lung transplantation, and summarize the experience. Methods: The clinical data of 19 patients receiving lung transplantation operation in Renmin Hospital of Wuhan University from December 2016 to December 2020 were retrospectively analyzed. Patients were divided into groups according to whether ECMO support was used, and their clinical data were compared. Results: Among the 19 patients, there were 5 cases of chronic obstructive pulmonary disease, 3 cases of bronchiectasis, 4 cases of pneumoconiosis, 4 cases of idiopathic pulmonary fibrosis, 1 case of connective tissue disease interstitial pulmonary fibrosis, 1 case of Kartagener syndrome, and 1 case of COVID-19 with advanced pulmonary fibrosis. Among the 10 patients in the non-ECMO group, 1 died during the operation, 1 died after abandoning treatment, and 1 was discharged after abandoning treatment. There were 9 patients in the ECMO group, 2 patients received ECMO bridged treatment before surgery, and the remaining 7 patients received ECMO adjuvant treatment after anesthesia. In the ECMO group, 1 patient died, 1 patient was discharged after abandoning treatment, and the rest patients were all improved and discharged after successful weaning of ECMO. The two groups only differed in the way of preoperative respiratory support (P=0.033). There were no significant differences in gender, age, preoperative assessment, surgical process, length of hospital stay, ICU stay, postoperative multi-drug resistant organism infection, and outcome. Conclusion: ECMO is an important auxiliary tool during the perioperative period of lung transplantation. The use of ECMO did not increase the risk of postoperative infection with multidrug-resistant bacteria, nor did it lead to an increase in mortality. In the other hand, the choice of preoperative respiratory support method has reference for perioperative patients whether to use ECMO. © 2021, Editorial Board of Medical Journal of Wuhan University. All right reserved.

Disease Progression and Outcomes in Patients with Telomere Biology Disorders

Dyskeratosis congenita and related telomere biology disorders (DC/TBDs) are multisystem illnesses caused by germline pathogenic variants (PV) in telomere biology genes resulting in very short telomeres. The inheritance varies and includes autosomal dominant (AD), autosomal recessive (AR) and X-linked (XLR) disease. Young patients may present with bone marrow failure (BMF) and the mucocutaneous triad of dysplastic nails, abnormal skin pigmentation, and oral leukoplakia, whereas adults may be identified with isolated BMF, pulmonary fibrosis (PF), or liver disease. In addition to BMF, DC/TBDs are associated with high risk of acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), head and neck squamous cell cancer (HNSCC) and many other medical problems. Hematopoietic cell transplant (HCT), lung, and/or liver transplantation may be required. A limited number of genotype-phenotype studies suggest the mode of inheritance to be associated with phenotypes. We studied 229 individuals (144 males, 85 females) with DC/TBDs enrolled in an IRB-approved longitudinal cohort study of inherited BMF syndromes (NCT00027274) between 01/2002 and 05/2019. Questionnaires and medical records were reviewed and a subset of 93 patients (40.6%) was systematically evaluated at the NIH Clinical Center (CC) during a 5-day visit. The median age at diagnosis for the entire cohort was 23.4 (0-82.2) years and median follow-up time was 4.2 (0-36.2) years. The PV was identified in 180 (78.6%) patients: 98 had AD disease (TERT, TERC, RTEL1, ACD, or PARN), 58 had XLR or AR (TERT, RTEL1, ACD, WRAP53, PARN, or DKC1) and 24 TINF2 (AD or de novo). In the entire cohort of 229 patients, 69 (30.1%) required HCT for BMF [median age 14.9 (0.9-63.1) years] and 58% of these had AR/XLR or TINF2-associated disease. Eight patients received lung transplant for PF [median age 51.4 (13.1-62.4) years]. Liver transplant was required in 5 patients [median age 25.8 (9.9-56.7) years]. Twenty-seven non-HCT patients developed cancer [median age of 42.1 (18.2-67.5) years]. In 5 patients, cancer occurred after HCT [median time since HCT=4.6 (1.8-14.6) years] and 3 developed post-HCT lymphoproliferative disease. Ninety-three patients were systematically phenotyped at the CC; 52 (55.9%) had &amp;gt;=1 triad feature and 33 (35.5%) had severe BMF. Three non-HCT patients had a history of cancer. PF was present in 6 patients and 3 had severe liver disease (portal hypertension and/or liver cirrhosis/fibrosis). Esophageal strictures were present in 7 and 5 had hip avascular necrosis (AVN, non-HCT). Median follow-up time after CC visit was 7.1 (0-17.1) years with median age at last follow-up of 29.6 (2.2-79.5) years . During follow-up, 9 patients progressed to severe BMF, and 8 developed cancer (4 post-HCT). Ten developed PF (5 post-HCT), and 9 advanced to pulmonary arteriovenous malformations (8 post-HCT). Eight patients progressed to severe liver disease (7 post-HCT). Gastrointestinal telangiectasias were seen in 6 individuals. Four patients developed esophageal stricture and 10 AVN (7 after radiotherapy and/or HCT). At last follow up of the entire cohort, 92 (40.2%) of 229 patients were deceased. The main causes of death were lung disease (28.3%), treatment-related complications (19.6%), cancer (16.3%), or BMF (14.1%). The median overall survival was 54.2 years [95% confidence interval (CI) 47.3-59.8] and HCT-free median survival was 46.9 years (95% CI 37.9-54.2). Overall survival was significantly better in patients with AD versus XLR/AR or TINF2 disease (p&amp;lt;0.0001, log-rank test, median survival 66.5 versus 33.2 years, respectively). Our study systematically quantified the presence and progression of DC/TBDs clinical manifestations and illustrates the significant morbidity faced by patients with these multisystem disorders. Long term survival is poor overall, but significantly better in patients with AD disease compared with other inheritance patterns. Although HCT has improved outcomes related to BMF, this study illustrates the pressing need to develop therapies for patients with DC/TBDs. These data also form the foundation for incorporating the mode of inheritance into evidence-based clinical care guidelines, risk stratification, and possibly treatment decisions in patients with DC/TBDs. Disclosures No relevant conflicts of interest to declare.