Objective To observe the dynamic changes of hepatic ischemia reperfusion injury ( IRI) by contrast enhanced ultrasound(CEUS) in rabbit model and investigate its clinical value . Methods Sixty rabbits were randomly assigned into seven groups :sham operation group and 6 hepatic IRI groups ( 0 .5 h ,2 h ,6 h ,12 h ,24 h ,72 h) . The rabbits of hepatic IRI group established as follows :the hepatic artery , portalvein and bile duct in the portal triad to the left liver lobes were interrupted for 60 min with a vascular clip and then reflow the blood . The perfusion imaging of left liver lobe was observed and then the peak signal Intensinty ( PI) ,time to peak intensinty ( Tp) ,area under the curve ( AUC) ,and mean transit time ( MTT ) were calculated . The rabbits were sacrificed and blood samples were withdrawn for the measurement of liver enzymes aspartate aminotransferase ( AST) and alanine aminotransferase ( ALT) ,liver samples from the left lateral were collected for the measurement of myeloperoxidase ( MPO) and histologic examination . The correlation between parameters of CEUS and the test results above were analyzed . Results There were significant differences in the parameters of CEUS between hepatic IRI group and sham group ( P < 0 .001) . PI and AUC showed a decreased tendency and the lowest values were measured in the 24 h group .Tp and MTT showed a upward tendency and the maximum values were measured in the 24 h group . The parameters of CEUS showed correlation with ALT ,AST and MPO ( P < 0 .001 ) . AUC showed significant correlation with ALT ,AST and MPO ( r = - 0 .702 , - 0 .802 , - 0 .846 ;all P < 0 .001 ) . Conclusions CEUS can assess liver functional and pathological changes in the process of hepatic IRI in real-time ,dynamically ,rapidly ,noninvasively and objectively . It is expected to replace the invasive pathological inspection . AUC is the best index for evaluating hepatic IRI in parameters of CEUS .

Neonatal chylothorax is a common cause of neonatal congenital pleural effusion and is often caused by the accumulation of chylous fluid in the thoracic cavity due to the rupture of the thoracic duct and its branched lymphatic vessels for a variety of reasons. Neonatal chylothorax caused by malignant tumors is extremely rare, and this is the first case of neonatal mediastinal neuroblastoma with chylothorax in China. The boy was found to have pleural effusion in the left thoracic cavity in the uterus, and experienced apnea at birth, as well as dyspnea and cyanosis as the main manifestations after birth. He was diagnosed with left chylothorax based on conventional biochemical analysis of pleural effusion. After the treatment including persistent chest drainage and symptomatic and supportive treatment, the drainage of the left thoracic cavity reached a volume of 90-180 mL per day. Neonatal refractory chylothorax was considered. Chest radiograph on day 13 after birth showed lesions in the upper left lung field, and contrast-enhanced plain CT scan of the chest suggested the possibility of posterior mediastinal neuroblastoma. The autopsy confirmed giant posterior mediastinal neuroblastoma (poorly differentiated), which involved the C7-T6 spinal canal and the nearby erector spinae, with a small amount of tumor tissue in the liver and both adrenal glands. Mediastinal tumor is considered the underlying cause of chylothorax in this case.
China ; Chylothorax ; Dyspnea ; Female ; Humans ; Infant, Newborn ; Male ; Pleural Effusion ; Uterus

BACKGROUNDPatent ductus arteriosus (PDA) is one of the most common congenital heart diseases and began to get treated by transcatheter occlusion since 1997 in China. Since then, several devices have been invented for occluding PDA. This study aimed to evaluate the technical feasibility, safety, and efficacy of transcatheter occlusion of PDA with different devices.

METHODSOne thousand five hundred and twenty-six patients (537 boys, 989 girls) with PDA from January 1997 to September 2014 underwent descending aortogram and transcatheter occlusion procedure. We retrospectively analyzed data of these patients, including gender, age, weight, size and morphology of PDA, and devices used in transcatheter occlusion, outcomes, and postoperational complications.

RESULTSMedian age and median weight were 4.0 years (range: 0.3-52.0 years old) and 15.3 kg (range: 4.5-91.0 kg), respectively. Mean ductal diameter, aortic ductal diameter, ductal length, and pulmonary artery pressure were 3.50 ± 2.15 mm, 10.08 ± 2.46 mm, 7.49 ± 3.02 mm, and 30.21 ± 17.28 mmHg, respectively. Morphology of PDA assessed by descending aortogram was of type A in 1428 patients, type B in 6 patients, type C in 79 patients, type D in 4 patients, and type E in 9 patients according to the classification of Krichenko. Of all the 1526 patients, 1497 patients underwent transcatheter PDA closure, among which 1492 were successful. Devices used were Amplatzer duct occluder I (ADO I, 1280, 85.8%), Cook detachable coils (116, 7.8%), ADO II (ADO II, 68, 4.6%), muscular VSD occluder (12, 0.8%), and Amplatzer vascular plug (16, 1.0%).

CONCLUSIONSExcellent occlusion rates with low complication rates were achieved with all devices regardless of PDA types. With transcatheter occlusion technique and devices developing, more patients with PDA can be treated with transcatheter closure both safely and efficiently.

Adolescent ; Adult ; Cardiac Catheterization ; Child ; Child, Preschool ; Ductus Arteriosus, Patent ; surgery ; Female ; Heart Defects, Congenital ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Prosthesis Design ; Retrospective Studies ; Septal Occluder Device ; Treatment Outcome ; Young Adult

The structure of fibrauretin made by our lab was modified. Fibrauretin was demethylated at 9-site under high temperature pyrolysis at 160℃-180℃ and was reacted with a series of acid chlorides. Twele derivatives of fibrauretin were obtained. The structure of each derivative was determined by1H-NMR and13C-NMR. The derivatives were 9-O-benzoyl-fibrauretin, 9-O-( 2-methylbenzoyl)-fibrauretin, 9-O-( 4-methylbenzoyl)-fibrauretin, 9-O-(3, 5-dimethylbenzoyl)-fibrauretin, 9-O-(4-(chloromethyl) benzoyl)-fibrauretin and other derivatives. The 12 derivatives are all new chemical compounds. Taking ATCI as substrate,the inhibitory activity on acetylcholinesterase (AChE) from the head of flies of the fibrauretin and its derivatives were screened. The results showed that most of the derivatives had improved their inhibitory activity on AChE through esterification reaction. Compounds 9-O-(4-methylbenzoyl)-fibrauretin, 9-O-(3,5-dimethylbenzoyl)-fibrauretinand 9-O-(4-(chloromethyl)benzoyl)-fibrauretin had significant inhibitory effect on AChE,and the inhibitory activity was stronger than the that of donepezil.

Aspergillus fumigatus ; Cytokines ; Humans ; Interleukin-1beta ; Keratitis ; Receptors, IgE ; Tumor Necrosis Factor-alpha ; Tumor Necrosis Factors

Objective:To explore the influence factors of chromosomal aberration levels in radiation workers in hospitals.Methods:Two hundred and fourteen age- and sex- matched hospital radiation workers were recruited by stratified random sampling method. According to the job title, the individuals were divided into four groups including diagnostic radiology group ( n=57), radiotherapy group ( n=49), nuclear medicine group ( n=52) and interventional radiology group ( n=56). Chromosomal aberrations in peripheral blood lymphocytes from the subjects were measured using conventional cytogenetic analysis method, and the influence factors of chromosomal aberrations were analyzed. Results:There was significant difference in the frequencies of acentric fragment, translocation and total chromosome-type aberrations among the four groups ( χ2=9.906, 19.965, 32.824, P<0.05), and the rates of aberrations were significantly higher in the interventional radiology group and the nuclear medicine groups than those in the diagnostic radiology (interventional group: χ2=4.711, 10.798, 10.845, P<0.05; nuclear medicine group: χ2=3.853, 7.674, 7.708, P<0.05) and the radiotherapy groups (interventional group: χ2=9.209, 9.772, 21.330, P<0.05; nuclear medicine group: χ2=8.010, 6.969, 10.812, P<0.05). The rates of translocation and total aberrations ( χ2=7.706, 6.667, P<0.05) and the frequencies of acentric fragment, translocation and total aberrations ( χ2=12.263, 15.360, 21.478, P<0.01) were dependent on the length of service and the dose among different groups. The rates of translocation and total aberrations significantly increased along with exposure doses ( r=0.347, 0.263, P<0.01). Poisson regression analysis indicated that the job titles and annual effective dose partly affected the levels of chromosomal aberrations[ IRR=1.797 (nuclear medicine group), 2.136 (interventional group) and 1.422 (0.5-1 mSv group); P<0.05]. Conclusions:The frequencies of chromosomal aberrations in the radiation workers of interventional and nuclear medicine groups remain higher levels in hospital, thus it is necessary to strengthen the radiation protection on these radiation workers.

Objective To analyze the influencing factors for intestinal colonization and secondary infection of car-bapenem-resistant Klebsiella pneumoniae(CRKP)in neonates,and provide a basis for formulating prevention and control strategies for CRKP infection.Methods Neonates who were admitted to the neonatal ward of a hospital from January 2021 to October 2022 were selected as the study subjects,and the first screening of CRKP was con-ducted within 48 hours after admission.In addition,active anal swab screening for carbapenem-resistant Ente-robacterales(CRE)was performed weekly during hospitalization,and the infection status of CRKP strains was mo-nitored.Clinical data of neonates in the colonization group,non-colonization group,and infection group were ana-lyzed.Intestinal colonized strains and the non-repetitive CRKP strains isolated from clinical specimens of neonates with secondary infection after colonization were performed carbapenemase gene detection,multilocus sequence ty-ping(MLST)and pulsed-field gel electrophoresis(PFGE)analysis.Results A total of 1 438 neonates were active-ly screened for CRE,174 were CRKP positive,CRKP colonization rate was 12.1％.Among 174 neonates,35 were with secondary infection,with the incidence of 20.1％.The independent risk factors for neonatal CRKP intestinal colonization were cesarean section(OR=2.050,95％CI:1.200-3.504,P=0.009),use of cephalosporins(OR=1.889,95％CI:1.086-3.288,P=0.024),nasogastric tube feeding(OR=2.317,95％CI:1.155-4.647,P=0.018).Protective factors were breast-feeding(OR=0.506,95％CI:0.284-0.901,P=0.021),oral probiotics(OR=0.307,95％CI:0.147-0.643,P=0.002),and enema(OR=0.334,95％CI:0.171-0.656,P=0.001).Independent risk factors for secondary infection after intestinal colonization of neonatal CRKP were carbapenem anti-biotic use(OR=19.869,95％CI:1.778-222.029,P=0.015)and prolonged hospital stay(OR=1.118,95％CI:1.082-1.157,P＜0.001).The detection results of drug resistance genes showed that carbapenemase-producing genes of CRKP strains were all blaKPC-2,all belonged to type ST11.Homologous analysis showed that intestinal CRKP colonization was highly homologous with the secondary infection strains after colonization.Conclusion CRKP intestinal colonization during neonatal hospitalization may increase the risk of CRKP infection.Risk and pro-tective factors of neonatal intestinal colonization and secondary infections after colonization should be paid attention,and corresponding preventive and control measures should be taken,so as to reduce the occurrence and transmission CRKP healthcare-associated infection.

The aim of this paper was to study the improvement effect of ethanol extract from Citri Reticulatae Pericarpium(CRP) on triglyceride of hyperlipidemia model rats, and to explore the possible mechanism. SD rats were randomly divided into normal group, model group, positive control group, and high, medium and low-dose CRP ethanol extract groups, with 10 rats in each group. During the experiment, except for the normal group that was fed with distilled water and ordinary feed, rats in the other groups were given different concentrations of alcohol and fed with high-sugar and fat diets. All rats were given free diets. While being modeled, each group was administered with 0.01 mL·g~(-1) by gavage once a day for six weeks. Blood samples were collected after two weeks, four weeks and six weeks of drug treatment. After the completion of the experiment, blood, liver and adipose tissue were collected. Triglyceride(TG), alanine aminotransferase(ALT), aspartate aminotransferase(AST), alkaline phosphatase(ALP) in serum, TG in liver tissue and TG in fecal were detected. Free fatty acid(FFA) and triglyceride-related hydrolase, such as adipose tiglyceride lipase(ATGL), lipoprotein lipase(LPL), hepatic lipase(HL), hormone-sensitive triglyceride lipase(HSL) were detected by ELISA. The mRNA expressions of peroxisome proliferators-activated receptors(PPARγ), sterol regulatory element binding protein 1 c(SREBP-1 c) and farnesoid X receptor(FXR) were determined by RT-PCR. Compared with the model group, each administration group could reduce TG levels in serum and liver to varying degrees, reduce serum ALT, AST, ALP activities, significantly reduce free fatty acid content in serum, significantly increase triglyceride metabolism-related enzymes, including fat ATGL, LPL and liver HL content, and significantly reduced the content of fat HSL. According to the study of transcriptional regulation genes relating to triglyceride metabolism, extract from CRP could significantly increase the mRNA expressions of PPARγ and FXR. In conclusion, ethanol extract from CRP could ob-viously reduce the TG level of hyperlipidemia model rats, and might reduce plasma TG content by increasing PPARγ-LPL/ATGL and FXR-HL triglyceride hydrolysis pathways.
Animals ; Ethanol ; Hyperlipidemias/genetics* ; Liver ; Plant Extracts ; Rats ; Rats, Sprague-Dawley ; Triglycerides

Objective:To investigate the relationship between mutated genes and clinical features in patients with essential thrombocythemia(ET).Methods:The clinical data of 69 patients with ET from October 2018 to March 2022 were retrospectively analyzed.According to driver mutation type,patients were divided into JAK2 group,CALR group and triple-negative group.The sex,age,cardiovascular risk factors,thrombosis,splenomegaly,routine blood test and coagulation status of patients in three groups were analyzed.Results:Among 69 ET patients,46 cases were associated with JAK2 mutation,14 cases with CALR mutation,8 cases with triple-negative mutation,and one with MPL gene mutation.There were no significant differences in age and sex among the three groups(P＞0.05).The highest thrombotic rate was 26.09％(12/46)in JAK2 group,then 12.5％(1/8)in triple-negative group,while no thrombotic events occurred in CALR group.The incidence of splenomegaly was the highest in JAK2 group(34.78％),while no splenomegaly occurred in triple-negative group.The white blood cell(WBC)count in JAK2 group was(9.00±4.86)× 109/L,which was significantly higher than(6.03±2.32)× 109/L in CALR group(P＜0.05).The hemoglobin(Hb)and hematocrit(HCT)in JAK2 group were(148.42±18.79)g/L and(0.44±0.06)％,respectively,which were both significantly higher than(131.00±15.17)g/L and(0.39±0.05)％in triple-negative group(P＜0.05).The platelet(PLT)in JAK2 group was(584.17±175.77)× 109/L,which was significantly lower than(703.07±225.60)× 109/L in CALR group(P＜0.05).The fibrinogen(Fg)in JAK2 and triple-negative group were(2.64±0.69)g/L and(3.05±0.77)g/L,respectively,which were both significantly higher than(2.24±0.47)g/L in CALR group(P＜0.05,P＜0.01).The activated partial thromboplastin time(APTT)in triple-negative group was(28.61±1.99)s,which was significantly decreased compared with(31.45±3.35)s in CALR group(P＜0.05).Conclusions:There are differences in blood cell count and coagulation status among ET patients with different driver gene mutations.Among ET patients,JAK2 mutation is most common.Compared with CALR group,the thrombotic rate,WBC and Fg significantly increase in JAK2 group,while PLT decrease.Compared with triple-negative group,the incidence of splenomegaly and HCT significantly increase.Compared with CALR group,Fg significantly increases but APTT decreases in triple-negative group.

BACKGROUND: Osteogenesis imperfecta (OI), a heritable bone fragility disorder, is mainly caused by mutations in COL1A1 gene encoding α1 chain of type I collagen. This study aimed to investigate the COL1A1 mutation spectrum and quantitatively assess the genotype-phenotype relationship in a large cohort of Chinese patients with OI. METHODS: A total of 161 patients who were diagnosed as OI in Department of Endocrinology of Peking Union Medical College Hospital from January 2010 to December 2017 were included in the study. The COL1A1 mutation spectrum was identified by next generation sequencing and confirmed by Sanger sequencing. A new clinical scoring system was developed to quantitatively assess the clinical severity of OI and the genotype-phenotype relationship was analyzed. The independent sample t-test, analysis of variance, Mann-Whitney U-test, Chi-squared test, Pearson correlation, and multiple linear regression were applied for statistical analyses. RESULTS: Among 161 patients with OI, 32.9% missense mutations, 16.8% non-sense mutations, 24.2% splice-site mutations, 24.8% frameshift mutations, and 1.2% whole-gene deletions were identified, of which 38 variations were novel. These mutations led to 53 patients carrying qualitative defects and 67 patients carrying quantitative defects in type I collagen. Compared to patients with quantitative mutations, patients with qualitative mutations had lower alkaline phosphatase level (296 [132, 346] U/L vs. 218 [136, 284] U/L, P = 0.009) and higher clinical score (12.2 ± 5.3 vs. 7.4 ± 2.4, P < 0.001), denoting more severe phenotypes including shorter stature, lower bone mineral density, higher fracture frequency, more bone deformity, vertebral compressive fractures, limited movement, and dentinogenesis imperfecta (DI). Patients would not present with DI if the glycine substitutions happened before the 79th amino acid in triple helix of α1 chains. CONCLUSIONS This presented distinctive COL1A1 mutation spectrum in a large cohort of Chinese patients with OI. This new quantitative analysis of genotype-phenotype correlation would be helpful to predict the prognosis of OI and genetic counseling.
Adolescent ; Adult ; Child ; Child, Preschool ; Collagen Type I ; genetics ; Female ; Genetic Association Studies ; High-Throughput Nucleotide Sequencing ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Mutation ; genetics ; Osteogenesis Imperfecta ; genetics ; pathology ; Young Adult