Objective To investigate the clinical efficacy and prognosis of lung transplantation in the treatment of cystic fibrosis (CF). Methods Clinical data of one patient with end-stage CF undergoing allogeneic bilateral lung transplantation were retrospectively analyzed. Clinical characteristics, diagnostic methods and treatment strategies of the CF recipient were summarized. Results The recipient had suffered from relevant symptoms since childhood including repeated cough and purulent sputum for 30 years, complicated with recurrent pulmonary infection combined with acute exacerbation, chronic sinusitis and extremely severe malnutrition. Prior to lung transplantation, the patient had to depend upon the invasive ventilator due to respiratory muscle weakness, and admitted to intensive care unit (ICU) for a long time. Imaging examination revealed multiple cystic columnar bronchiectasis accompanied with infection in bilateral lungs. The diagnosis of CF was further confirmed by sweat test and gene detection. The recipient underwent bilateral lung transplantation on August 17, 2017 and received rehabilitation treatment. The lung function was gradually restored to normal. The recipient had obtained the same quality of life to the healthy counterparts since the date of manuscript submission (over 2 years). Conclusions Lung transplantation is an efficacious treatment for end-stage CF, which can not only save patients' lives, but also significantly improve the quality of life of patients.

Objective To explore the treatment strategies of pleuroparenchymal fibroelastosis (PPFE). Methods A 22-year-old male patient was complicated with PPFE after receiving chemotherapy in combination with stem cell transplantation for lymphoma. He underwent thoracoscopic left lung tongue wedge resection, bilateral pleurodesis followed by allogeneic left lung transplantation. Literature review was performed to analyze the etiology, pathogenesis, imaging features, pathological features and treatment of PPFE. Results The PPFE patient required the non-invasive ventilator for 24 h before lung transplantation. After lung transplantation, the shortness of breath and respiratory failure were cured and the quality of life was significantly improved. No eligible studies was found in the domestic database, and 26 literatures published in English were found in the international databases. Among them, 9 literatures (case reports) were finally included after screening. PPFE could be divided into the primary and secondary categories according to the etiology. The clinical manifestations of PPFE mainly included dry cough, dyspnea on exertion, chest pain, repeated pneumothorax and body weight loss. Chest CT scan demonstrated irregular thickening of the pleura in bilateral upper lungs. Pathological manifestations consisted of evident thickening of the visceral pleura, fibroelastosis and arrangement disorder in the pleura and the underlying pulmonary interstitium. PPFE could progress rapidly. Adrenocortical hormone and other immunosuppressive agents yielded low clinical efficacy and poor clinical prognosis. Lung transplantation was a necessary treatment for PPFE. Conclusions PPFE cannot be effectively treated by conservative therapy. It is recommended to deliver lung transplantation as early as possible.

Objective To analyze the dynamic changes and the influencing factors of T lymphocyte subsets in recipients with stable graft status within 1 year after lung transplantation. Methods Clinical data of 41 recipients with stable graft status after allogeneic lung transplantation were analyzed. The absolute value and ratio of T lymphocyte subsets in peripheral blood from recipients were measured by flow cytometry before operation, 2 weeks and each month (within 1 year) after operation, respectively. The effects of age, gender, body mass index (BMI), surgical method, incidence of primary graft dysfunction (PGD) after operation, and primary disease upon the absolute values of T lymphocytes were evaluated. Results Within 1 year after lung transplantation, the absolute values of CD3⁺, CD3⁺CD4⁺, CD3⁺CD8⁺T lymphocytes and CD4⁺/CD8⁺ ratio were changed over time (all P < 0.001). Compared with preoperative values, there was no statistical significance in the absolute values of CD3⁺ and CD3⁺CD4⁺T lymphocytes at 12 months after operation (P=0.659, 0.109), whereas the absolute value of CD3⁺CD8⁺T lymphocytes was increased (P=0.02) and the CD4⁺/CD8⁺ ratio was decreased (P < 0.001). Age, gender, BMI, surgical method and incidence of PGD after operation exerted no significant effect on the dynamic changes of absolute values of CD3⁺CD4⁺ and CD3⁺CD8⁺T lymphocytes (all P > 0.05). Primary disease before lung transplantation exerted no effect on the changes of CD3⁺CD4⁺T lymphocytes, whereas the postoperative absolute value of CD3⁺CD8⁺T lymphocytes was higher in recipients with infectious lung diseases (P < 0.05). Conclusions The absolute values of CD3⁺, CD3⁺CD4⁺, CD3⁺CD8⁺T lymphocytes in recipients with stable graft status after lung transplantation are relatively low in the early stage after lung transplantation, then gradually restore, and stabilize at 6 months after operation. Dynamic changes are not associated with age, gender, BMI, surgical method and incidence of PGD after operation of recipients.

Acute kidney injury (AKI) is one of the common early complications after lung transplantation, which not only increases the short-term and long-term fatality of lung transplant recipients, but also significantly increases the incidence of long-term chronic renal insufficiency after surgery. In recent years, early AKI after lung transplantation has attracted high attention along with the rapid development of lung transplantation in China. In this article, research progresses on diagnosis, incidence, risk factors, prevention and treatment of early AKI after lung transplantation around the globe were reviewed, aiming to better identify the risk factors and poor prognosis of early AKI after lung transplantation, and provide theoretical and practical guidance for early clinical interventions.

Objective To analyze the risk factors and clinical prognosis of acute kidney injury (AKI) early after lung transplantation. Methods Clinical data of 155 recipients undergoing lung transplantation or combined heart-lung transplantation were retrospectively analyzed, and they were divided into the AKI group (n=104) and non-AKI group (n=51) according to the 2012 Kidney Disease: Improving Global Outcomes Clinical Practice Guideline. The incidence of AKI early after lung transplantation was summarized. The main indexes of recipients were collected. The risk factors of the occurrence of AKI early after lung transplantation were subjected to univariate and multivariate analysis. The clinical prognosis of lung transplant recipients was evaluated and the survival curve was delineated. Results The incidence of AKI early after lung transplantation was 67.1%(104/155), including 47 recipients with stage 1 AKI, 34 recipients with stage2 AKI and 23 recipients with stage 3 AKI, respectively. Sixteen recipients required continuous renal replacement therapy (CRRT) early after lung transplantation. Preoperative complication with diabetes mellitus, preoperative complication with pulmonary hypertension, intraoperative mean arterial pressure (MAP) < 60 mmHg, intraoperative massive blood transfusion, and treatment with excessive therapeutic concentration of tacrolimus (Tac) within postoperative 1 week were the independent risk factors for the occurrence of AKI early after lung transplantation. Up to the end of follow-up, 66 recipients (42.6%) died, including 50 recipients in the AKI group and 16 recipients in the non-AKI group. The cumulative survival rate in the AKI group was significantly lower than that in the non-AKI group (40% vs. 66%, P < 0.05). With the increase of AKI severity, the cumulative survival rate of lung transplant recipients was decreased. Conclusions AKI develops early after lung transplantation with high incidence and poor clinical prognosis. Preoperative complication with diabetes mellitus and pulmonary hypertension, intraoperative MAP < 60 mmHg and massive blood transfusion, and treatment with excessive therapeutic concentration of Tac within postoperative 1 week are the independent risk factors for the occurrence of AKI early after lung transplantation.

Acute cellular rejection (ACR) is a common complication after lung transplantation, which is mainly caused by the immune response of T lymphocytes recognizing the major histocompatibility complex on the cellular surface of grafts. It is currently considered as the main pattern of acute rejection. ACR is not only a direct cause of death of recipients, but also a high-risk factor for chronic rejection after lung transplantation. Nevertheless, it is a challenging task to deliver the diagnosis and treatment of ACR following lung transplantation. In this article, new progresses on the risk factors, pathogenesis, diagnosis and treatment of ACR in lung transplant recipients were summarized, aiming to improve the diagnostic and treatment efficiency of ACR and prolong the survival of recipients.

Objective To investigate the treatment on de novo donor specific antibody (dnDSA) mediated acute rejection after lung transplantation. Methods Clinical data of 1 recipient with antibody-mediated rejection (AMR) early after lung transplantation was retrospectively analyzed. The process of diagnosis and treatment were assessed. Results The recipient underwent right lung transplantation due to systemic sclerosis-associated end-stage interstitial lung disease. Preoperatively, classⅠ panel reactive antibody (PRA) was positive (11%). No pretreatment was given before transplantation. Antithymocyte globulin induction therapy was delivered on the day of transplantation and postoperatively. The recipient was properly recovered early after transplantation. Chest tightness and shortness of breath occurred at postoperative 13 d, which were progressively worsened and rapidly progressed into type Ⅰ respiratory failure. Class Ⅰ PRA was increased to 58%, and dnDSA was observed at the loci of A24: 02. The mean fluorescence intensity (MFI) was 2 110. According to the guidelines of International Society for Heart and Lung Transplantation, the recipient was diagnosed as possible AMR. After comprehensive treatment including plasmapheresis, protein A immunoadsorption, glucocorticoid pulse, rituximab and immunoglobulin intravenous drip, the PRA and DSA levels were gradually decreased, and the MFI of DSA was 0 at postoperative 20 d. Clinical condition of the recipient was gradually improved. The dyspnea was healed, shortness of breath was eased, respiratory failure was treated, and pulmonary effusion was gradually absorbed. At postoperative 45 d, the recipient was discharged after full recovery. During 1-year follow-up, the recipient was physically stable and obtained normal quality of life. Class Ⅰ PRA was 5%, and class Ⅱ PRA was negative. No DSA was noted. Conclusions Based on traditional drug therapy, supplement of protein A immunoadsorption therapy may effectively eliminate DSA from the circulating blood of the recipient and mitigate the damage of target organs. Ideal short- and long-term prognosis may be achieved. Traditional drug therapy combined with immunoadsorption may yield ideal efficacy in treating AMR after lung transplantation.

Objective:To explore the significance of US lung allocation score (LAS) in Chinese lung transplant recipients.Methods:The clinical data were analyzed for 173 lung recipients from May 2005 to March 2018. The LAS of each patient was calculated by an online LAS calculator of Organ Procurement and Transplantation Network (OPTN).Results:The mean age was (56.49±12.64) years and the mean LAS (56.63±18.39)(32.79-90.70). The underlying diseases were chronic obstructive pulmonary disease (COPD, n=62), interstitial lung disease (n=85), bronchiectasis (n=11), pulmonary arterial hypertension (n=8) and others (n=7). And the value of LAS was (47.85±15.22) vs. (61.89±18.63) vs. (56.58±18.91) vs. (55.23±10.74) vs. (72.45±16.41). LAS of COPD patients was significantly lower than that of interstitial lung disease ( P<0.001). Mean LAS was the highest in endotracheal intubation or ECMO group (79.15±7.95), then non-invasive ventilation group (48.42±11.58) and lowest in oxygen inhalation group (44.11±8.81)( P<0.001). Recipients were divided into three groups of LAS <50 for low-risk, 50-75 for moderate-risk and >75 for high-risk. Survivals at 90 days and 1 year were 90.5% vs. 81.8% vs. 71.1% and 85.4% vs. 74.4% vs. 57.8% ( P=0.002). Conclusions:LAS can not only reflect the urgency of recipients waiting for lung transplantation but also predict postoperative period. LAS score should be employed for selecting suitable lung transplant recipients in China and the optimal LAS lies between 30 and 75.

Objective:To explore the diagnosis and treatment strategies of primary ciliary dyskinesia (PCD).Methods:A 37-year-old male recipient who had repeated cough and sputum from childhood, with shortness of breath after activity and progressive development, the number of hospitalizations per year was ≥6 times, the MRC score was 2~3 diagnosed with primary ciliary dyskinesia through medical examination and multidisciplinary consultation, and received allogeneic double lung transplantation after medical treatment failure. Search related domestic and foreign literatures to explore and analyze the etiology, pathogenesis, clinical manifestations and imaging features, diagnosis and treatment of PCD.Results:PCD is an autosomal hereditary disease. Due to abnormal skeletal structure and/or functional development, clinical manifestations are mostly chronic sinusitis, bronchiectasis, otitis media and infertility. Chest imaging showed situs inversus totalis, right heart and diffuse cystic bronchiectasis infection with bronchial wall thickening, diagnosis depends on clinical manifestations, saccharin test, nasal nitric oxide test, high-speed video microscopy analysis, transmission electron microscopy, genetic testing and immunofluorescence.Conclusions:Lung transplantation is the only effective treatment for end-stage PCD. It not only saves patients' lives, but also significantly improves their quality of life.

Objective:To explore the clinical manifestations and imaging features of nocardia infection (NI) after lung transplantation and boost the diagnosis and treatment of NI.Methods:From January 2018 to December 2019, basic profiles, clinical manifestations, laboratory examinations, imaging features and treatment outcomes of 5 lung transplant recipients with a diagnosis of NF were retrospectively analyzed and summarized with the relevant literatures. There were 4 males and 1 female with a median age of 66(26-69) years. 3 patients were single-lung transplantation, 2 patients were bilateral-lung transplantation. The median time from an initial diagnosis of NI to lung transplant surgery was 6(5-19) months. Common symptoms included fever, cough with yellow phlegm and shortness of breath. Laboratory findings showed lymphopenia, significantly high C-reactive protein levels, a slight elevation of procalcitonin, hypoproteinemia and anemia. The major manifestations of high-resolution computed tomography (CT) included multiple nodules, consolidation, cavitation and pleural effusion.Results:Five strains of N. farcinica were identified from bloodstream infection ( n=2) and pulmonary infection ( n=3). After with a combined therapy of two sensitive agents, all patients improved and were discharged from hospital. During follow-ups, one patient died and the remainders were cured. Conclusions:Nocardia infection occurs in lung transplant recipients mostly within 1 year post-operation. There are non-specific symptoms and imaging features of multiple nodules and consolidation. Combination therapy of sensitive agents is indicated for lung transplant recipients with NI.