Favorable Outcomes of Liver Transplantation for Hepatopulmonary Syndrome.

BACKGROUND: Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of chronic liver disease, which develops insidiously as a result of chronic liver disease. The prognosis for untreated patients with HPS is extremely poor, and liver transplantation (LT) serves as the only effective means for treating this condition. Here, we performed a retrospective analysis to evaluate the efficacy of LT on the survival and long-term prognosis of patients with HPS. METHODS: Clinical data, including survival and postoperative efficacy, from patients with HPS from records as obtained over the period from January 1 to December 31, 2022. All records were from a waiting list for LT at the Beijing Friendship Hospital Affiliated with Capital Medical University. RESULTS: Among the 274 patients on the LT waiting list, 37 were diagnosed with HPS (13.50%) and were enrolled. Survival rates of patients with HPS receiving an LT were greater, whereas a statistically significant difference was obtained between patients with LT vs non-LT with moderate to severe HPS (P = .003). The overall time until death without LT was 4-72 days after their initial HPS diagnosis. Patients with HPS receiving an LT showed a significant improvement in the state of oxygenation after surgery (P = .001). CONCLUSION: Comprehensive preoperative screening of patients on the waiting list for LT is critical to identify those patients with HPS who would maximally benefit from LT. Survival rates of patients with moderate to severe HPS are significantly increased after LT, a procedure that should be performed as soon as possible in these patients with HPS.

Coexistence of Primary Sjögren's Syndrome and Autoimmune Gastritis With Pernicious Anemia and Subacute Combined Degeneration of the Spinal Cord: Case Report and Literature Review.

Background: Autoimmune gastritis (AIG) and Primary Sjögren's syndrome (pSS) are both autoimmune diseases with low prevalence in China. Subacute combined degeneration (SCD) of the spinal cord is the most common neurological manifestation of vitamin B12 deficiency. Until now, a patient with pSS and complications of AIG including SCD has not been reported. Case Presentation: A 69-year-old woman presented with palpitations and symmetrical and progressive numbness in her hands and feet. The patient had a sense of stepping on cotton and could not write or walk without help. We reviewed the patient's history and analyzed her blood tests, imaging, gastroscopic findings, and pathological results. The patient fulfilled the criteria of AIG, pSS, spinal cord SCD and early pernicious anemia (PA) simultaneously. Although pSS can lead to reduction of vitamin B12, this is the first overlapping case of pSS with spinal cord SCD. After symptomatic treatment, the patient returned to a normal life. Conclusions: This first report about the coexistence of pSS and complications of AIG including SCD and PA will promote a better understanding of the relationship between these diseases.

EBV-specific cytotoxic T lymphocytes for refractory EBV-associated post-transplant lymphoproliferative disorder in solid organ transplant recipients: a systematic review.

The use of Epstein-Barr virus-specific cytotoxic T lymphocytes (EBV-CTLs) in adoptive immunotherapy in hematopoietic stem cell transplantation (HSCT) patients with post-transplantation lymphoproliferative disorder (PTLD) has demonstrated safety and effectiveness. EBV-CTLs might also be the effective treatment of refractory PTLD of solid organ transplantation (SOT) recipients. Two independent assessors searched Pubmed, Embase, Cochrane Library, and Web of Science from their inception to November 2020. Eleven studies with 76 patients (42, 55% male) were included. We extracted the data and completed the quality assessments. Most of the studies were from Europe and the USA. Liver and kidney transplantation accounted for most of the transplant types. Thirty-five (46.1%) patients were diagnosed with monomorphic PTLD, and B lymphocyte type was the most common. All the patients received primary treatment for PTLD while it was ineffective. CTLs included autologous EBV-CTLs (15/76, 22%) and HLA-matched third-party EBV-CTLs (61/76, 78%). The response rate for EBV-CTL treatment of refractory PTLD was 66%. Of 50 patients, 36 achieved complete remission and 14 achieved partial remission. EBV-DNA level decreased in 39 patients. Adverse reactions were rare and mild. We conclude that adoptive therapy with EBV-specific CTLs is safe, well-tolerated, and effective in PTLD.

Comparison of trends in congenital heart disease mortality from 1990 to 2017 between China and North America / 中华心血管病杂志

Objective: To compare trends in congenital heart disease (CHD) mortality between China and North America from 1990 to 2017. Methods: Using the data from the Global Burden Of Disease (GBD) study 2017, we analyzed the related indicators of CHD mortality in China and North America from 1990 to 2017, including standardized mortality, number of deaths, age distribution of death population and age-specific mortality of CHD in each birth cohort. Age-period-cohort model was used to calculate the annual percent change of age-standardized and age-specific mortality rates of CHD (% per year), period effect-adjusted age-specific mortality rates, and the relative risk of death among CHD population at different time periods (2000－2004 as reference period) and different birth cohorts (1970 as reference cohort). Results: In 2017, the age-standardized mortality rates for CHD in China and North America were 2.63/100 000 and 1.13/100 000 respectively, a decrease of 50.4% and 49.4% compared to 1990. Of all deaths from CHD in China, 76.8% were found in children under 5 years, which was higher than that in North America (51.7%). For population under 40 years, the period effect-adjusted age-specific mortality was higher in China (0.46-167.94 per 100 000 person-years) than in North America (0.68-22.47 per 100 000 person-years); whereas for population over 40 years, mortality was lower in China (0.13-0.34/100 000 person years) than in North America (0.43-0.72/100 000 person-years).From 1990 to 2017, CHD mortality in China decreased by 1.95% per year. The annual decrease of mortality ranged from 1.95% to 3.64% per year in population under 45 years, but the mortality showed increasing trends among those over 50 years. In 2015－2019, the relative risk of death from CHD decreased by 31% in China and 24% in North America. For 2015 birth cohort, the relative risk of death decreased by 84% in China and by 64% in North America. Conclusions: In the past 30 years, the risk of death from CHD in China has significantly decreased, and the survival gap with North America is dramatically narrowed. However, mortality is higher among younger populations in China than in North America, and the mortality in the elders shows increasing trends each year in China.

Therapeutic Effects of Transthoracic Pulmonary Artery Denervation in a Rat Model of Pulmonary Arterial Hypertension / 中国循环杂志

Objectives:The aim of this study was to observe whether transthoracic pulmonary artery denervation (TPADN) could reduce the pulmonary arterial pressure and attenuate pulmonary vascular remodeling in rats with pulmonary arterial hypertension induced by monocrotaline. Methods:Twenty-four healthy male SD rats were randomly divided into control group, sham operation group and operation group (n=8 per group). Rats in sham operation group and operation group received single subcutaneous injection of monocrotaline (MCT, 60 mg/kg). After four weeks, the mean pulmonary arterial pressure (mPAP) and other hemodynamic parameters were measured with the right heart catheter in rats of these two groups. Then, operation group received the surgery of TPADN, which included thoracotomy in left 2-3 rib, exposing pulmonary artery, and removing the near connective tissue of the pulmonary artery trunk. After two weeks of operation, the mPAP and other hemodynamic parameters were measured again by the right heart catheter. The microstructure changes of the heart and pulmonary vessels was observed by immunohistochemistry and immunofluorescence. Meanwhile, RV cardiomyocyte cross-sectional area (CSA) and the right hearthy pertrophy index (RVHI= RV/[LV+S]) were used to evaluate the degree of right ventricular hypertrophy. Results:After four weeks of injection of MCT, the mPAP was significantly higher in the operation group and the sham operation group than in control group (P＜0.01). Two weeks after the surgery of TPADN, the mPAP was significantly reduced in the operation group than compared in the sham group(P＜0.01). Meanwhile, the percentage of medial thickness to outer diameter of the small pulmonary arterioles, right ventricular myocardial cell cross-sectional area and RVHI were also significantly decreased in the operation group compared to sham operation group(all P＜0.01). Conclusions:Our results show that TPADN could reduce the mean pulmonary arterial pressure and attenuate the hypertrophy of medial thickness of small pulmonary arterioles and of right ventricle in PAH rats induced by monocrotaline.