Association of stress hyperglycemia with clinical outcomes in patients with ST-elevation myocardial infarction undergoing percutaneous coronary intervention: a cohort study.

BACKGROUND: In recent years, several studies have demonstrated that stress hyperglycemia is significantly associated with poor prognosis in patients diagnosed with acute coronary syndrome (ACS). In the present study, we aimed to investigate the potential associations between various markers of stress hyperglycemia, such as admission blood glucose (ABG), fasting blood sugar (FBS), and stress hyperglycemia ratio (SHR) with different definitions, and the occurrence of adverse cardiovascular events in patients diagnosed with ST-elevation myocardial infarction (STEMI) who have undergone percutaneous coronary intervention (PCI). METHODS: Our study enrolled a total of 1099 patients diagnosed with STEMI who underwent PCI from 2016 to 2021. The primary outcomes of this study were in-hospital death and all-cause mortality. RESULTS: Stress hyperglycemia was associated with a higher incidence of in-hospital death (ABG OR: 1.27 95% CI 1.19-1.36; FBS OR: 1.25 95% CI 1.16-1.35; SHR1 OR: 1.61 95% CI 1.21-2.14; SHR2 OR: 1.57, 95%CI 1.22-2.01; SHR3 OR: 1.59, 95%CI 1.24-2.05) and all-cause mortality (ABG HR: 1.10, 95% CI 1.07-1.14; FBS HR: 1.12, 95 CI 1.07-1.17; SHR1 HR: 1.19 95% CI 1.03-1.39; SHR2 HR: 1.28, 95%CI 1.14-1.44; SHR3 HR: 1.29, 95%CI 1.14-1.45) after adjusting for ischemic time, age, gender, BMI, hypertension, hyperlipidemia, diabetes mellitus (DM), current smoking history, chronic kidney disease (CKD), previous history of coronary artery disease (CAD), atrial fibrillation (AF), heart failure (HF), stroke, cancer, culprit vessel, multi-vessel disease. These associations exhibited a non-linear, J-shaped pattern, wherein the risk significantly increased when the ABG and FBS levels exceeded 5mmol/L. Moreover, the inflection point for SHR was estimated to be 1.2. CONCLUSIONS: Stress hyperglycemia was significantly associated with an increased risk of in-hospital death and all-cause mortality in STEMI patients treated with PCI. Stress hyperglycemia should be considered a high-risk prognostic marker in all STEMI patients, regardless of with or without diabetes.

Clinicopathological features of twenty four active aortitis cases / 中华风湿病学杂志

Objective:To explore the clinicaland pathological characteristics of aortitisin order to improve the understanding of this rare conditionand improve correct diagnostic rate.Methods:Twenty-four cases of active aortitis were identified from a total of 1 838 cases of ascending aorta specimens in the last 6 years at Wuhan Asia General Hospital. Clinical data including medical history, laboratory and imaging data were collected and the treatment with immunosuppressive and hormonal treatment as well as follow-up data were analyzed. Pathological data including gross findings, microscopic features of the aortic valve and aorta were analyzed. Continuous variables were expressed as mean±standard deviation. Frequencies were described as percentages. Results:Among the 24 cases of active aortitis, 1 case was clinically diagnosed as aortitis before operation, 7 cases were suspected aortitis before operation, and the other 16 cases were diagnosed as aortitis after pathological examination. Among those 16 cases, one case was Behcet′s syndrome, 2 cases were infectious aortitis, 3 cases were Takayasu aortitis, and 10 cases were clinically isolated aortitis. None case had aortic stenosis, while 21 cases had aortic valve insufficiency. Eleven cases of aortitis showed coagulation necrosis. In the 5 cases of Behcet′s syndrome, 3 had acute noninfectious endocarditis of aortic valve.Conclusions:Most of the aortitis in this study was found accidentally in pathological examination. All of the clinically isolated aortitiswere misdiagnosed before pathological examination. Most of the patients with aortitis had simple aortic valve insufficiency. Coagulation necrosis is an important clue for the diagnosis of aortitis. Acute noninfectious endocarditis is an important clue for the diagnosis of Behcet′s syndrome.

Application value of single-port laparoscopic left lateral donor liver acquisition in pediatric living donor liver transplantation / 中华消化外科杂志

Objective:To investigate the application value of single-port laparoscopic left lateral donor liver acquisition in pediatric living donor liver transplantation (PLDLT).Methods:The retrospective and descriptive study was conducted. The clinical data of the donor and recipient who were admitted to Beijing Friendship Hospital of Capital Medical University for PLDLT in January 2020 were collected. The donor was a male, aged 28 years with body mass as 62 kg, height as 174 cm and body mass index (BMI) as 20.5 kg/m 2. The recipient was the daughter of the donor, aged 1 year with body mass as 9 kg, height as 75 cm and BMI as 16.0 kg/m 2. The donor underwent single-port laparoscopic left lateral donor liver acquisition. The recipient underwent living donor liver trans-plantation by the same operation team. Observation indicators: (1) intraoperative conditions; (2) postoperative conditions; (3) follow-up. Results:(1) Intraoperative conditions. The donor under-went single-port laparoscopic left lateral donor liver acquisition successfully, with the single-port access system being placed through a transumbilical incision. The operation time, the warm ischemia time of the donor liver and volume of intraoperative blood loss were 240 minutes, 3 minutes and 40 mL, respectively, of the donor. The weight of the donor liver was 233.6 g, and the corrected graft-to-recipient body weight ratio was 2.60%. The recipient underwent living donor liver transplantation successfully. (2) Postoperative conditions. The donor began to take liquid diet at postoperative day 1, and results of laboratory examination showed that the alanine aminotransferase (ALT), aspartate aminotransferase (AST), glutamyltransferase (GGT) and total bilirubin (TBil) of the donor was 239 U/L, 116 U/L, 53 U/L and 22.57 μmol/L. The donor began to take diet with high quality proteins at postoperative day 2, and to get for out-of-bed activities moderately. The donor′s peritoneal drainage fluid was light red at postoperative day 3, and no fluid accumulation was found in the operation area after abdominal B-ultrasound examination, so the peritoneal drainage tube was removed. The donor was discharged at postoperative day 4. The liver function of the recipient recovered to normal level 2 weeks after the operation. (3) Follow-up. The donor was followed up by outpatient examination 2 weeks after discharged, and results of laboratory examination showed that the ALT, AST, GGT and TBil was 44 U/L, 25 U/L, 53 U/L and 9.22 μmol/L, respectively. Neither the donor nor the recipient had complication ≥Ⅱ grade of the Clavien-Dino classification, such as biliary fistula and vascular complication during the 6 months after operation.Conclusion:Single-port laparoscopic left lateral donor liver acquisition can be used into the PLDLT.

Utilizing ultra-small volume graft in auxiliary liver transplantation for portal hypertension / 中华外科杂志

Objective: To examine the clinical effect of auxiliary liver transplantation with ultra-small volume graft in the treatment of portal hypertension. Methods: Twelve cases of portal hypertension treated by auxiliary liver transplantation with small volume graft at Liver Transplantation Center,Beijing Friendship Hospital, Capital Medical University between December 2014 and March 2022 were studied retrospectively. There were 8 males and 4 females,aged 14 to 66 years. Model for end-stage liver disease scores were 1 to 15 points and Child scores were 6 to 11 points. The grafts was derived from living donors in 9 cases,from split cadaveric donors in 2 cases,from whole cadaveric liver of child in 1 case. The graft recipient body weight ratios of 3 cadaveric donor livers were 0.79% to 0.90%, and of 9 living donor livers were 0.31% to 0.55%.In these cases, ultra-small volume grafts were implanted. The survivals of patient and graft, complications, portal vein blood flow of residual liver and graft, abdominal drainage and biochemical indexes of liver function were observed. Results: All the grafts and patients survived. Complications included outflow tract torsion in 2 cases, acute rejection in 1 case, bile leakage in 1 case, and thyroid cancer at the later stage of follow-up in 1 case, all of which were cured. The torsion of outflow tract was attributed to the change of anastomotic angle after the growth of donor liver. After the improvement of anastomotic method, the complication did not recur in the later stage. There was no complication of portal hypertension. The measurement of ultrasonic portal vein blood flow velocity showed that the blood flow of residual liver decreased significantly in the early stage after operation, and maintained a very low blood flow velocity or occlusion in the long term after operation, and the blood flow of transplanted liver was stable. Conclusions: Auxiliary liver transplantation can implant ultra-small donor liver through compensation of residual liver. This method may promote the development of living donor left lobe donation and split liver transplantation. However, the auxiliary liver transplantation is complex, and it is difficult to control the complications. Therefore, this method is currently limited to centers that are skilled in living related liver transplantation and that have complete ability to monitor and deal with complications.

Firm-level short selling and the local COVID-19 pandemic: Evidence from China.

Short seller trading behavior attracts much attention, especially when negative shocks occur. Recent literature has focused on the impact of the COVID-19 pandemic, an unprecedented shock, but evidence on short sellers' reactions is quite scarce. This paper investigates how short sellers responded to the local COVID-19 pandemic in China. Empirical results show that greater numbers of newly confirmed COVID-19 cases in listed firms' headquarters locations are associated with more subsequent short selling of those firms. The results hold after addressing other potential concerns. In addition, the impact of the local COVID-19 pandemic on short selling is stronger for firms with weaker financial conditions, in more vulnerable industries, and with higher risks of a stock price crash. The impact is alleviated after lifting the lockdown restrictions in Wuhan and becomes insignificant in later outbreaks. Overall, our findings support the informational role of short sellers within the context of the COVID-19 pandemic.

Pediatric liver transplantation for Alagille syndrome: a single-center experience / 器官移植

Objective To evaluate the clinical efficacy of liver transplantation in children with Alagille syndrome (ALGS). Methods Clinical data of 12 children with ALGS were collected and retrospectively analyzed. Clinical characteristics of children with ALGS, pathological characteristics of liver tissues, characteristics of liver transplantation, postoperative complications and follow-up of children with ALGS were analyzed. Results JAG1 gene mutation and typical facial features was present in all 12 children. Jaundice was the most common initial symptom, which occurred at 7 (3, 40) d after birth. Upon liver transplantation, the Z scores of height and body weight were calculated as -2.14 (-3.11, -1.83) and -2.32 (-3.12, -1.12). Five children developed severe growth retardation and 4 children with severe malnutrition. Eight of 12 children were diagnosed with cardiovascular abnormalities. Pathological examination showed that the lobular structure of the diseased livers of 4 children was basically maintained, and 8 cases of nodular liver cirrhosis in different sizes including 1 case of single early moderately-differentiated hepatocellular carcinoma. Three children were misdiagnosed with biliary atresia and underwent Kasai portoenterostomy. Eight children underwent living donor liver transplantation, three children underwent cadaveric donor liver transplantation (two cases of split liver transplantation and one case of cadaveric total liver transplantation), and one child underwent domino liver transplantation (donor liver was derived from a patient with maple syrup urine disease). during the follow-up of 30.0(24.5, 41.7) months, the survival rates of the children and liver grafts were both 100%. During postoperative follow-up, the Z scores of height and body weight were calculated as -1.24 (-2.11, 0.60) and -0.83 (-1.65, -0.43), indicating that the growth and development of the children were significantly improved after operation. Conclusions Liver transplantation is an efficacious treatment for children with ALGS complicated with decompensated cirrhosis, severe itching and poor quality of life. For children with ALGS complicated with cardiovascular abnormalities, explicit preoperative evaluation should be delivered, and consultation with pediatric cardiologists should be performed if necessary.

Clinical research of incomplete immune tolerance to donor specific blood group antigen in pediatric ABO incompatible living donor liver transplantation recipients / 中华器官移植杂志

Objective:To explore the potential immune mechanism of pediatric ABOi-LDLT presenting low humoral immune response to donor specific blood group antigen.Methods:From June 2013 to December 2020, clinical data were retrospectively reviewed for 29 patients of long-term surviving pediatric ABOi-LDLT.There were A to O ABOi-LDLT( n=10)and B to O ABOi-LDLT( n=19). Graft types included left lateral lobe( n=26)and left hemi-liver( n=3). The median age of liver transplantation was 10 months, the median weight 8.0 kg and the median follow-up time 41.9 months.The titers of donor specific blood group antibodies and non-donor specific blood group antibodies(IgG, IgM)were continuously monitored before transplantation and at 1, 3, 6, 12, 24, 36 months post-transplantation.Protocol or event-based liver biopsy was performed to determine whether or not there was antibody-mediated rejection. Results:The titer of donor specific blood group antibody in recipients was significantly lower than that of non-donor specific blood group antibody( P<0.001). Among 18 protocol liver pathological biopsies, two cases were C4d positive for vascular endothelium.Five abnormal event-based liver biopsies were completed and one was C4d positive in bile duct endothelium.No pathological sign of typical blood group antibody mediated antigen-antibody complex mediated cascade immune reaction was detected in liver pathological biopsy.Typical pathological signs of blood group antibody mediated rejection were absent in protocol liver biopsy. Conclusions:Donor specific blood group antibody is expressed at a low level in pediatric ABOi-LDLT recipients.It presents as incomplete immune tolerance to donor specific blood group antigen.

Significance of Epstein-Barr virus-infected lymphocyte cell types in peripheral blood mononuclear cells in post-transplant lymphoproliferative disorder after pediatric liver transplantation / 中华器官移植杂志

Objective:To explore the characteristics and significance of Epstein-Barr virus-infected lymphocyte cell types in peripheral blood mononuclear cells(PBMC)in post-transplant lymphoproliferative disorder(PTLD)after pediatric liver transplantation.Methods:From June 2013 to March 2021, retrospective data analysis was performed for 14 pediatric liver transplant recipients with PTLD.The determination of EBV-DNA in PBMC, plasma and TBNK lymphocyte cells was analyzed.Results:EBV-DNA in PBMC showed a high viral load(>10 4 copies/ml)and plasma EBV-DNA was >10 3 copies/ml( n=8). There were dominant B-cell-type infection( n=12)and T/NK-cell-type infection( n=2). After treatment, EBV-DNA in PBMC and plasma turned negative in 7 patients with a decline( n=6)and an increase( n=1). EBV-DNA in B lymphocyte became negative( n=10)with a decline( n=3). In one case, EBV-DNA increased in T, B and NK cells with a high viral load.The remainders recovered after treatment.One case of hemophagocytic syndrome died from a progression of PTLD. Conclusions:A large majority of EBV-related PTLD are dominated by B-cell-type infection and a few belong to T or NK-cell-type infection.Patients with T/NK-cell-type infection have a worse response to therapy and poorer prognosis than those with B-cell-type infection.Determination of EBV-infected lymphocyte cell types is of vital research value for treatment and prognosis.

Liver transplantation for acute liver failure in children: 8-year experience in a single center / 器官移植

Objective To evaluate the efficacy of liver transplantation for acute liver failure (ALF) in children. Methods Clinical data of 15 children with ALF who underwent liver transplantation were collected and retrospectively analyzed. The proportion of ALF among children undergoing liver transplantation during the same period was calculated. The characteristics, postoperative complications and clinical prognosis of ALF children receiving liver transplantation were analyzed. Results In the same period, the proportion of ALF was 2.0% (15/743) among pediatric recipients undergoing liver transplantation. All 15 children had acute onset of ALF, and most of them were accompanied by fever, diarrhea and progressive yellowing of skin and sclera. Thirteen children were complicated with hepatic encephalopathy before operation (6 cases of stage Ⅳ hepatic encephalopathy), and two children were complicated with myelosuppression and granulocytopenia before liver transplantation. Ten children underwent living donor liver transplantation with relative donor liver, 4 received liver transplantation from donation after cardiac death (DCD), and 1 underwent Domino donor-auxiliary liver transplantation. Of 15 children, 12 recipients had the same blood type with their donors, 1 recipient had compatible blood type with the donor and 2 cases had different blood type with their donors. Among 15 children, 10 cases developed postoperative complications. Postoperative cerebral edema occurred in 5 cases, of whom 4 cases died of diffuse cerebral edema, and the remaining case was in a persistent vegetative state (eyes-open coma). Postoperative cytomegalovirus (CMV) infection was seen in 5 cases. Two children presented with aplastic anemia and survived after bone marrow transplantation, 1 case died of CMV hepatitis and viral encephalitis, and 2 cases died of diffuse brain edema. One child developed graft-versus-host disease (GVHD) after liver transplantation, and died of septic shock after bone marrow transplantation. Nine children survived and obtained favorable liver function during postoperative follow-up. Conclusions Liver transplantation is an efficacious treatment for ALF in children, which may enhance the survival rate. Brain edema is the main cause of death in ALF children following liver transplantation, and treatment such as lowering intracranial pressure, improving brain metabolism and blood purification should be actively performed. Liver transplantation should be promptly performed prior to the incidence of irreversible neurological damage in ALF children, which might prolong the survival and enhance long-term prognosis.

Construction of a genome instability-derived lncRNA-based risk scoring system for the prognosis of hepatocellular carcinoma.

Emerging evidence revealed the critical roles of long non-coding RNAs (lncRNAs) in maintaining genomic instability. However, genome instability-associated lncRNAs (GILncRNAs) and their performance in clinical prognostic significance in hepatocellular carcinoma (HCC) are rarely reported. Our study constructed a computational framework integrating somatic mutation information and lncRNA expression profiles of HCC genome and we identified 88 GILncRNAs of HCC. Function enrichment analysis revealed that GILncRNAs were involved in various metabolism processes and genome instability of cancer. A genome instability-derived lncRNA-based gene signature (GILncSig) was constructed using training set data. The performance of GILncSig for outcome prediction was validated in testing set and The Cancer Genome Atlas (TCGA) set. The multivariate cox regression analysis and stratification analysis demonstrated GILncSig could serve as an independent prognostic factor for the overall survival of HCC patients. The time-dependent Receiver Operating Characteristic (ROC) curve illustrated GILncSig outperformed two recently published lncRNA signatures for overall survival prediction. The combination of GILncSig and tumor protein p53 (TP53) mutation status exhibited better prognostic performance in survival evaluation compared to TP53 mutation status alone. AC145343.1 was further validated to be a risk factor for HCC in vitro among GILncSig. Overall, our study provided a novel approach for identification of genome instability-associated lncRNAs and established an independent risk score system for outcome prediction of HCC patients, which provided a new insight for exploring in-depth mechanism and potential therapy strategy.

Intraosseous schwannoma of the proximal humerus with pathologic fracture.

BACKGROUND: Intraosseous schwannomas are extremely rare in the humerus, and less than five cases have been reported previously in the literature. This is the first report of its origin in the proximal humerus with pathologic fracture. We herein present this case to discuss the reason for its rarity and share our experience of management. CASE PRESENTATION: A 55-year-old female patient presented with pain in the right shoulder, which was caused by tripping and falling over a board. Radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) showed considerable tumor in proximal humerus, which connected with a fracture. For this suspected tumor, we performed two operations. Pathological examination demonstrated typical picture of a schwannoma, showing whorls and interlacing fascicles of schwannoma spindle cells. Immunohistochemistry, the tumor cells were diffusely positive for S-100 protein, SOX-10 and CD68, while they were completely negative for desmin, DOG-1, AE1/AE3 and P63. The Ki-67 index was about 10%. No mitoses or features of malignancy were identified. The final diagnosis of intraosseous schwannoma was made. The treatment for intraosseous schwannoma with pathologic fracture includes excisional biopsy, curettage, bone allograft, and fracture fixation. The patient recovered well. After the surgery, the patient gradually regained mobility and the pain subsided. There was no recurrence after 6 months of follow-up by X-ray. CONCLUSIONS: Although very rare, intraosseous schwannoma should be taken under consideration in the differential diagnosis of benign-appearing osseous tumor in the proximal humerus with pathologic fracture.

Bioinformatics Analyses of Potential miRNA-mRNA Regulatory Axis in HBV-related Hepatocellular Carcinoma.

Aims: We aimed to explore the crucial miRNA-mRNA axis through bioinformatics analysis and provide evidences for the development of pathophysiological mechanisms and new therapies for HBV-related HCC. Methods: MiRNA (GSE76903) and mRNA (GSE77509) dataset were used to screen differentially expressed miRNAs (DE-miRNAs) and differentially expressed mRNAs (DE-mRNAs) using R software. Overlapping genes between DE-mRNAs and target genes of DE-miRNAs were identified as candidate genes. Hub genes were obtained via cytohubba analysis. The expression at protein and mRNA levels and prognostic value of hub genes were evaluated based on The Cancer Genome Atlas (TCGA) data. Key miRNA-mRNA axes were constructed according to predicted miRNA-mRNA pairs. MiRNA expression and prognostic role were respectively identified using starBase v3.0 and Kaplan-Meier plotter database. Real-time PCR was performed to verify the expression of crucial miRNAs and mRNAs. Coexpression of crucial miRNA and mRNA were analyzed using starBase v3.0. Results: CDK1, CCNB1, CKS2 and CCNE1 were screened as hub genes, which were significantly upregulated at protein and mRNA levels. These up-regulated hub genes were also significantly associated with poor prognosis. Hsa-mir-195-5p/CDK1, hsa-mir-5589-3p/CCNB1 and hsa-let-7c-3p/CKS2 were screened as critical miRNA-mRNA axes. Critical miRNAs were decreased in HCC, which indicates unfavourable prognosis. QPCR results showed that crucial miRNAs were decreased, whereas critical mRNAs were increased in HBV-related HCC. A reverse relationship between miRNA and mRNA in crucial axis was further verified. Conclusion: This study identified several miRNA-mRNA axes in HBV-related HCC. Hsa-mir-195-5p/CDK1, hsa-mir-5589-3p/CCNB1 and hsa-let-7c-3p/CKS2 might serve as potential prognostic biomarkers and therapeutic targets for HBV-related HCC.

Evaluation of the performance of systems for whole blood C-reactive protein detection: a multi-center study / 中华检验医学杂志

Objective:To explore the performance of the commonly used whole blood C-reactive protein (CRP) detection systems and give related recommendation on the performance requirements of detection systems.Methods:A total of 7 540 venous blood samples from 26 maternal, child and children′s hospitals were collected to conduct this multi-center study on the analytical performance of 5 commonly used whole blood CRP detection systems from March to April in 2019. The blank check, carryover, repeatability, intermediate precision, linearity, sample stability, influence of hematocrit/triglyceride/bilirubin, comparison with SIEMENS specific protein analyzer and trueness were evaluated. The 5 systems included BC-5390CRP autohematology analyzer, AstepPLUS specific protein analyzer, Ottoman-1000 Automated Specific Protein POCT Workstation, i-CHROMA Immunofluorometer equipment Reader and Orion QuikRead go detecting instrument. The 5 systems were labeled as a, b, c, d and e randomly.Results:Within the 5 systems, all values of blank check were less than 1.00 mg/L, the carryovers were lower than 1.00%. The repeatability of different ranges of CRP concentrations including 3.00-10.00, 10.00-30.00 and>30.00 mg/L were less than 10.00%, 6.00% and 5.00%, respectively, and the intermediate precision was less than 10.00%. The linearity correlation coefficients of the 5 systems were all above 0.975, while the slope was within 0.950-1.050. Whole blood samples were stable within 72 hours both at room temperature (18-25 ℃) and refrigerated temperature (2-8 ℃). The CRP results were rarely influenced by high triglyceride or bilirubin, except for the immmunoturbidimetric test based on microparticles coated with anti-human CRP F(ab) 2 fragments. When triglyceride was less than 15.46 mmol/L, the deviation of CRP was less than 10.00%. When bilirubin was less than 345.47 μmol/L, the deviation of CRP was less than 10.00%. CRP was more susceptible to Hct on the systems without Hct correction. The deviation of CRP between different Hct dilution concentration and 40% dilution concentration can reach as high as 67.48%. The correlation coefficients ( r) of 5 systems were all more than 0.975 in the range of 0-300.00 mg/L compared with Siemens specific protein analyzer. All systems passed the trueness verification using the samples with specified values of 12.89 and 30.60 mg/L. Conclusion:The performance of 5 systems can basically meet the clinical needs, but it is suggested that the whole blood CRP detection system without automatic Hct correction should be modified manually.

Lowering the incidence of complications of portal vein through vascular graft interposition during pediatric transplantation / 中华器官移植杂志

Objective:To explore the feasibility of using vascular graft interposition for lowering the complications of portal vein during pediatric liver transplantation.Methods:From June 1, 2013 to May 31, 2018, clinical data were collected for 297 children undergoing liver transplantation, including basic demographics, general preoperative status, preoperative tests, imaging findings, graft related profiles, surgical procedures and postoperative follow-ups, etc. Then the authors analyzed the effect of using interposition vessels upon lowering postoperative complications of portal vein reconstruction.Results:With a median age of 12 months, there were 153 boys (51.5%) and 144 girls (48.5%). The primary disease was mostly biliary atresia ( n=222, 74.7%). The median diameter of portal vein was 5 mm. There were 19 cases (6.4%) using vascular interposition. Among 20 cases of portal vein complications, there were portal vein stenosis ( n=17, 5.7%) and portal vein thrombosis ( n=3, 1.0%). After univariate analysis, binary Logistic regression analysis revealed that diameter of recipient's portal vein was an independent risk factor for the occurrence of portal vein complications after liver transplantation. Statistical analysis of children with portal vein diameter <4 mm ( n=90) was carried on and the results showed that there was no inter-group statistical difference ( χ2=3.061, P=0.080)on the occurrence of portal vein complications. Conclusions:Diameter of portal vein is an important factor affecting the strategic choice of portal vein reconstruction during pediatric liver transplantation and an independent risk factor for portal vein complications after liver transplantation. When the diameter of portal vein is ≤4 mm, using interposition vascular anastomosis shows no significant difference with other conventional modes.

Case report of living donor liver transplantation for pediatric propionic acidemia combined with dilated cardiomyopathy / 中华实用儿科临床杂志

To analyzed a case of pediatric patient with propionic acidemia combined with dilated cardiomyopathy retrospectively, who underwent living donor liver transplantation at the Liver Transplantation Center, Beijing Friendship Hospital, Capital Medical University in March 2019.A 2 years and 6 months female child was admitted to hospital for propionic acidemia.The pretransplant echocardiogram showed left ventricular dilatation and systolic dysfunction, and thus dilated cardiomyopathy was considered.A living donor liver transplant was performed using her mother′s left latera-llobe.On the 14 months postoperatively, the child was on a liberated protein diet, but still required levocarnitine supplementation.Her hepatic and cardiac function returned normal, but growth retardation was still present.During the follow-up period, further propionic acidemia-related complications like metabolic decompensation, or any transplant-related complications were not reported.This case report suggested that liver transplantation is effective on pediatric propionic acidemia combined with cardiomyopathy, which reverses cardiomyopathy, improves cardiac function, relieves strict protein restriction, reduces the risk of metabolic decompensation, and significantly improves quality of life.

Effect of liver transplantation on intestinal microflora in children with biliary atresia / 器官移植

Objective To evaluate the effect of liver transplantation on intestinal microflora in children with biliary atresia. Methods The fecal samples and liver function indexes of 16 children with biliary atresia before and 6 months after liver transplantation were collected, and 10 healthy children were selected as the healthy controls. DNA extraction and metagenome sequencing were carried out in the fecal samples. Statistical analysis was performed by software packages, such as R language. The changes of species structure and functional composition of intestinal microflora after liver transplantation were analyzed. The recovery of intestinal microflora in children with biliary atresia after liver transplantation was assessed. The relationship between intestinal microflora and liver function indexes was investigated. Results Following liver transplantation, the number of species of intestinal microflora in children with biliary atresia was increased. The opportunistic pathogens were the dominant species of intestinal microflora in children with biliary atresia before liver transplantation. The abundance of opportunistic pathogens was decreased and the abundance of short-chain fatty acid-producing bacteria was increased after liver transplantation (all P < 0.05). Following liver transplantation, lipid metabolism, amino acid metabolism, carbohydrate metabolism, energy metabolism, metabolism of cofactors and vitamins were enhanced, whereas infectious diseases of bacterial, immune diseases and drug resistance were weakened. Compared with the healthy control group, there were no statistically significant differences in the diversity and structure of intestinal microflora in the post-liver transplant group, but different species were observed between two groups. The liver function indexes of children with biliary atresia after liver transplantation tended to decline (all P < 0.000 1). The abundance of beneficial intestinal microflora was negatively correlated with liver function indexes, whereas the abundance of opportunistic pathogens was positively correlated with liver function indexes (all P < 0.05). Conclusions Liver transplantation may significantly improve the structure and functional composition of intestinal microflora in children with biliary atresia.

Diagnosis and treatment of hepatic sinusoidal obstruction syndrome after liver transplantation: a single-center experience / 器官移植

Objective To summarize the incidence, diagnostic and therapeutic experience of hepatic sinusoidal obstruction syndrome (HSOS) after liver transplantation. Methods Clinical data of 4 patients with HSOS after liver transplantation were retrospectively analyzed. The incidence, clinical manifestations, imaging and pathological characteristics of HSOS after liver transplantation were collected, and the treatment methods and clinical outcomes of patients with HSOS were analyzed. Results The incidence of HSOS after liver transplantation was 0.8%(2/239), and the median time of onset was 4.5(1.7, 9.0) months after liver transplantation. The clinical manifestations of HSOS mainly included abdominal distension, ascites, hepatomegaly, increased bilirubin, and renal insufficiency in partial cases. Enhanced abdominal CT scan of 4 patients with HSOS showed uneven spot-like enhancement and the liver histopathological examination mainly showed the signs of hepatic sinusoidal dilatation complicated with congestion. Four patients were administered with an adjusted regime of immunosuppressant by replacing tacrolimus (Tac) with ciclosporin and adding anticoagulant therapy with warfarin. One patient received transjugular intrahepatic portosystemic shunt (TIPS). After treatment, the symptoms of 3 patients were completely relieved, and 1 patient died. One of the 3 surviving patients died from pulmonary infection and gastrointestinal bleeding. Conclusions HSOS is a rare and fatal complication after liver transplantation. Timely diagnosis and treatment can avoid the incidence of graft failure and improve clinical prognosis of the patients.

Genetic analysis of three cases of acephalic spermatozoa syndrome caused by SUN5 mutation and the outcome of assisted reproductive technology / 北京大学学报(医学版)

To explore the genetic causes of 3 male infertility patients with acephalospermia and the outcome of assisted reproductive technology. Clinical diagnosis, sperm morphology examination, sperm transmission electron microscopy examination were performed on 3 patients, and the whole exome sequencing technology was used for screening, Sanger sequencing verification, mutation pathogenicity analysis, and protein sequence homology comparison. Assisted reproductive technology was implemented to assist pregnancy treatment. The 3 patients were all sporadic infertile men, aged 25, 42 and 26 years, and there was no obvious abnormality in the general physical examination. Male external genitalia developed normally, bilateral testicles were normal in volume, and bilateral epididymis and spermatic vein were palpated without nodules, cysts, and tenderness. Repeated semen analysis showed that a large number of immature sperm could be seen, and they had the ability to move. The SUN5 gene of the 3 male infertile patients was a case of homozygous missense mutation c.7C>T (p.Arg3Trp), a case of compound heterozygous missense mutation c.1067G>A (p.Arg356His) and nonsense mutation c.216G>A (p.Trp72*) and a case of homozygous missense mutation c.1043A>T (p.Asn348Ile), of which c.7C>T (p.Arg3Trp) and c.1067G>A (p.Arg356His) were new variants that had not been reported. SIFT, Mutation Taster and PolyPhen-2 software function prediction results were all harmful, the nonsense mutation c.216G>A (p.Trp72*) led to the premature termination of peptide chain synthesis which might have a greater impact on protein function. The homology regions in the protein sequence homology alignment were all highly conserved.The 3 male patients and their spouses obtained 4 biological offspring through intracytoplasmic sperm injection, all of which were boys, and one of them was a twin.Three male infertile patients might be caused by SUN5 gene mutations. Such patients could obtain their biological offspring through assisted reproductive technology. It was still necessary to pay attention to the genetic risk of ASS, it was recommended that both men and women conduct genetic counseling and screening at the same time. In clinical diagnosis, whole exome sequencing technology could be used to perform auxiliary examinations to determine the treatment plan and assisted reproductive methods as soon as possible to reduce the burden on the family and society. The newly discovered mutation sites of SUN5 gene provided clues and directions for elucidating the pathogenic mechanism, and at the same time expanded the pathogenic mutation spectrum of ASS.

Clinical use of 'Full right-Full left’ split liver transplantation: a report of 4 cases / 中华肝胆外科杂志

Objective:To review our experience in the use of "Full right-Full left" split liver transplantation in adult-to adult or adult-to adult-size child.Methods:The clinical data of liver donors to 4 recipients of full right-full left split liver transplantation performed at Beijing Friendship Hospital of Capital Medical University from January to December 2019 were reviewed. The surgical methods of split liver transplantation, cold ischemia time, operation time, intraoperative blood transfusion, and postoperative complications and prognosis were analyzed.Results:The 4 recipients of complete right hepatic-left hepatic split liver transplantation included 3 adults and 1 heavy child (45 kg). Their ages ranged from 14 to 48 years, and body weight ranged from 45 to 61 kg. The end-stage liver disease model score were 21, 12, 41, and 30 points. The ratios of graft mass to recipient's body mass ranged from 0.85% to 1.35%. The cold ischemia time was 457-650 min, and the operation time was 460-575 min. Early liver function recovered smoothly in all the 4 patients after transplantation, and no small liver syndrome occurred. Patients were followed up to 6 months after operation. One patient developed anastomotic biliary leak, which was cured by endoscopic retrograde cholangiopancreatographic treatment. Another patient developed biliary stricture presenting with repeated biliary tract infection despite percutaneous transhepatic puncture biliary drainage. A third patient died six months from lung infection.Conclusion:In properly selected patients, using full right-full left hemiliver by split liver transplantation increased organ utilization and provided patients with increased treatment opportunities.

Hemodynamic monitoring of the liver after auxillary liver transplantation treated with a functional shunt for portal hypertension associated with a small-for-size graft / 中华肝胆外科杂志

Objective:To study the hepatic hemodynamics changes and pathophysiological mechanisms of the use of a functional shunt after auxillary liver transplantation to treat portal hypertension associated with a small-for-size graft.Methods:A retrospective analysis of the clinical data of patients with portal hypertension treated with functional shunting of small-volume grafts from a living donor liver at the Beijing Friendship Hospital, Capital Medical University from July 2014 to December 2018, and a total of 6 patients were included as the research objects, including 4 males and 2 females, with a median age of 35.5 (29.0-52.0) years old. Blood flow monitoring data were collected during and after operation, and the characteristics of liver hemodynamics were analyzed.Results:The portal venous blood flow of the remnant native liver gradually decreased to no flow. As a buffer response, the flow velocity of hepatic artery increased. The portal venous blood flow of the graft gradually increased in the early postoperative period and then gradually decreased from post-operation Day 5 to 10 due to gradual increase in portal venous resistance. However, the portal venous perfusion gradually increased from Day 10 after the operation, reached to a level and declined to a stable level about 1 month after the operation. The volume of abdominal drainage slowly decreased after the peak level at Day 5-10 after the operation, and disappeared completely at Day 30 after operation.Conclusions:When using auxiliary liver transplantation for functional shunting to treat portal hypertension, autologous residual liver can act as a guide buffer for the pressure gradient of portal vein hyperperfusion in liver transplantation, and reach a steady state of blood flow distribution about 1 month after surgery, while relying on autologous remnant liver hepatic artery buffer response prevents small liver syndrome.