Objective To analyse CT and DSA appearances of intrahepatic cholangiocarcinoma,and to improve understanding of intrahepatic cholangiocarcinoma.Methods Plain and enhanced CT scanning were performed in 17 patients with intrahepatic cholangiocarcinoma pathollogically proved,6 patients(6/17) performed dynamic CT scanning,5 performed DSA examinations.Results Pre-contrast CT scanning was a single low density lesions in all patients,post-contrast CT scanning showed slightly inhomogeneous enhancement in 17 patients,3 patients(3/17) showed low density unenhancement area with margin enhancement,2 patients delayed enhancement;Intrahepatic billary delatation was found in 15 patients and was within the lesion in 10 patients(10/15);On DSA,5 patients showed enlaragement increasment and rigidity of supplying arteries and tumour stain.Conclusion CT scanning and DSA were important,investigative methods for intrahepatic cholangiocarcinoma,the billary dilatations with slightly inhomogeneous enhancement lesions is important sign in diagnosising intrahepatic cholangiocarcinoma.

OBJECTIVETo study the relationship between the nonresponse to hepatitis B vaccine and HLA genotype/heplotype in Chinese population and provide the evidence for explaining the genetic mechanism of this nonresponse.

METHODSOur research focused on the relationship between nonresponse to Hepatitis B vaccine and HLA-DRB1, DRB3, DRB4, DRB5 and DQB1 genotype/haplotype in Chinese population, collected from a community in Guangxi Zhuang Autonomous Region. The group specific amplification was employed to characterize 107 individuals' genotype and haplotype of HLA clusters. Different models statistics such as relative risk test, correlation test and linkage disequilibrium analysis were used to analyze the data.

RESULTSThe results showed that there is a linkage disequilibrium between nonresponse to Hepatitis B vaccine and HLA haplotype DR4, 1122 (DRB1 * 0401- 22, 1122)-DR53 (DRB4 * 0101101, 0102/3)-DQB4 (DQB1 * 04).

CONCLUSIONIn Chinese population, nonresponse to hepatitis B vaccine is highly associated with special HLA haplotye.

Asian Continental Ancestry Group ; genetics ; China ; Genotype ; HLA-DQ Antigens ; classification ; genetics ; HLA-DQ beta-Chains ; HLA-DR Antigens ; classification ; genetics ; HLA-DRB1 Chains ; HLA-DRB3 Chains ; HLA-DRB4 Chains ; HLA-DRB5 Chains ; Haplotypes ; Hepatitis B ; genetics ; immunology ; prevention & control ; Hepatitis B Vaccines ; immunology ; Humans ; Linkage Disequilibrium

OBJECTIVEMucopolysaccharidosis(MPS) is a congenital hereditary disease. Only a few patients with this disease can be controlled by enzyme replacement therapy. Most of them are short of effective interference. To exploit the effect of treatment with allogenic hematopoietic stem cell transplantation, two children were treated with the transplantation.

METHODSThe two patients included a 23 month MPS-IH and an 18 month old MPS-VI at the time of transplantation. Busulfan of 20 mg/kg plus 200 mg of Cyclophosphamide were used as the conditioning regimen. Peripheral stem cells were collected from a 9/10 high resolution matched unrelated donor and a matched sibling carrier donor, respectively. The heart and lung were affected in the patient with MPS-IH. Medium obstructed pulmonary impairment was found by pulmonary function test at the time of transplantation. Medium mitral valve countercurrent and patent ductus arteriosis(PDA) were found by Doppla examination.

RESULTSThe number of hematopoietic stem cells was comparative between the two donors with total nucleated cells and CD34+ cells of 11 x 10(8)/kg and 17 x 10(8)/kg, and 7.6 x 10(6)/kg and 7.2x 10(6)/kg respectively. Neutrophil engrafted at day 11. The process of transplantation in the MPS-VI patient went smoothly with grade II graft versus host disease(GVHD) briefly and only 1 U RBC and 2 U platelet were transfused. For the MPS-IH patient, the process of transplantation was tough with platelet reaching to 20 x 10(9)/L till day 40 and 5 U RBC and 7 U platelet were transfused during transplantation. Grade III GVHD was resolved by steroid, mycophenolate mofetil (MMF) and CD25 antibody. Pneumonia recurred 3 times with 2 times rescued by trachea intubation and mechanical ventilation because of accompanying acute heart failure. At day 14 the lymphocytes in both patients were 100% from donors as evidenced by short tandem repeat-PCR(STR-PCR). MPS associated enzyme activity was increased to 70 nmol/h.mg and 66 nmol/h.mg at 3 month and still remained 50.9 nmol/h.mg and 44.5 nmol/h.mg at 2 years post transplantation. Till now the 2 patients have been followed up for 25 months and 28 months with good general condition. The cardiac and pulmonary functions have improved obviously in the MPS-IH patient. The cornea became clear in this patient.

CONCLUSIONAllogeneic hematopoietic stem cell transplantation is an effective measure to treat patient with MPS-IH and MPS-VI. Transplantation at earlier stage of age can decrease transplant related complications. It requires longer time follow up for observing the clinical effects for these patients.

Female ; Follow-Up Studies ; Graft vs Host Disease ; drug therapy ; etiology ; Hematopoietic Stem Cell Transplantation ; adverse effects ; methods ; Humans ; Infant ; Intraoperative Complications ; drug therapy ; etiology ; Male ; Mucopolysaccharidoses ; enzymology ; pathology ; physiopathology ; surgery ; Recovery of Function ; Transplantation, Homologous

Objective:To provide a reference for extracorporeal membrane oxygenation (ECMO) inter-hospital transport during coronavirus disease 2019 (COVID-19), based on the transport experience of 6 patients with severe H1N1 influenza virus pneumonia using ECMO.Methods:Clinical data of patients with severe H1N1 influenza virus pneumonia implemented by ECMO in the First Affiliated Hospital of Wannan Medical College from October 2018 to December 2019 were retrospective analyzed, including general information, ECMO transport distance, time, clinical parameters before and after ECMO, including the patients' oxygenation index (PaO 2/FiO 2), respiratory rate (RR), pulse blood oxygen saturation (SpO 2), arterial blood carbon dioxide partial pressure (PaCO 2), and pH value, various complications during transport, mechanical ventilation time, patients' prognosis and other indicators. Experience from the aspects of personal protection, transport process and equipment, team cooperation, mid-transit monitoring, quality control, etc., was summarized to provide suggestions for patients with severe COVID-19 using ECMO during inter-hospital transport and protection. Results:A total of 6 patients with severe H1N1 influenza virus pneumonia were transported on ECMO. All patients were transported to the intensive care unit (ICU) of the First Affiliated Hospital of Wannan Medical College by the ECMO transport team after the establishment of ECMO in the local hospital. The transfer distance was 11 to 197 km, with an average of (93.8±58.6) km; the transfer time was 30 to 150 minutes, with an average of (79.2±40.6) minutes. Two patients experienced a drop in ECMO flow and SpO 2 during the process, and the main reason was insufficient volume, which was improved after fluid resuscitation and posture adjustment. All patients maintained SpO 2 above 0.93. Six patients survived and were discharged. ECMO assisted time was 4-9 days, with an average of (6.5±1.5) days; mechanical ventilation time was 7-24 days, and median time was 10.0 (8.0, 14.5) days. No H1N1 transmission occurred in medical personnel. To achieve good therapeutic effect, the main experience was to choose the proper timing and mode of ECMO; intact transportation vehicles and equipment to reduce or avoid mechanical complications; the effective management of respiration and circulation during the transportation to avoid ventilation-associated lung injury (VALI) and serious hypoxemia; the appropriate space for the transfer team to quickly handle various critical situations; and personal protection to avoid infection. Conclusion:With an experienced ECMO transport team, good transport equipment, comprehensive protection measures, reasonable transport procedures, and a perfect emergency plan, it is safe to use ECMO transport for COVID-19 patients.

OBJECTIVE To analyze the current status and research hot spots of traditional Chinese medicine （TCM）in the prevention and treatment of myocardial ischemia -reperfusion injury （MIRI），and to provide reference for developing relevant studies. METHODS A bibliometric analysis was performed with the literature on TCM treatments for MIRI included in the Web of Science as the data source . RESULTS A total of 972 literatures were included in this study . The research on TCM for prevention and treatment of MIRI in recent 10 years showed an increasing trend year by year ，and the literature published in 2021 were cited most frequently ；most research scholars focused in China ；Peking Union Medical College ，Chinese Academy of Medical Sciences had the most papers in this field ，and a lot of collaboration with other institutions ，which had a large influence in the field ；Sun Guibo and Sun Xiaobo were high -yielding authors in the field ，but the global authorship was not very close and had not formed cooperative teams of some scale ；Molecular Medicine Reports was perhaps the most popular journal in the field . Co-citation analysis found that the research direction in this field was mostly focused on exploring the pathogenesis of MIRI and finding intervention targets . Key words analysis revealed that TCM regulated apoptosis ， pyroptosis， mitochondrial dysfunction ， miRNA and other pathways to improve MIRI research was a hot spot in the field ，and more studies had focused on the Nrf 2 pathway，NF-κB pathway ，NLRP3 inflammasomepathway， TNF pathway ， SIRT3 pathway， JNK pathway ，PI3K/Akt pathway and AMPK pathway . CONCLUSIONS Much attention has been paid to the prevention and treatment of MIRI by TCM in the past 10 years，and the current hot spots in this field are mostly focused on the studies about the intervention mechanism of TCM ’s active ingredients and Chinese patent medicines on MIRI . Moreover，the research about TCM in the treatment of MIRI is mostly focused on the field of basic experiments at present ，and it is recommended that many large -scale，high-quality， multicenter，randomized controlled clinical studies should be carried out in the future ，to provide high -grade evidence -based evidence for the application of TCM in clinic .

The widespread application of next generation sequencing (NGS) in clinical settings has enabled testing, diagnosis, treatment and prevention of genetic diseases. However, many issues have arisen in the meanwhile. One of the most pressing issues is the lack of standards for reporting genetic test results across different service providers. The First Forum on Standards and Specifications for Clinical Genetic Testing was held to address the issue in Shenzhen, China, on October 28, 2017. Participants, including geneticists, clinicians, and representatives of genetic testing service providers, discussed problems of clinical genetic testing services across in China and shared opinions on principles, challenges, and standards for reporting clinical genetic test results. Here we summarize expert opinions presented at the seminar and report the consensus, which will serve as a basis for the development of standards and guidelines for reporting of clinical genetic testing results, in order to promote the standardization and regulation of genetic testing services in China.