Objective To explore the liver pathology from live related liver transplatation(LRLT) of Wilson′s disease(WD) in children,and evaluatethe indication of LRLT.Methods The sample of this study,including the donater and patient,came from the LRLT. It was observed with HE,MASSON,Timm′s and Rubeanic staining.Results With HE and MASSON stainning,hepatocyte showed degeneration,fiber hyperplasic and false lobule formed;Timm′s and Rubeanic stainning showed typical black deposit of granules and conglomerations.Liver pathology revealed that liver cirrhosis appeared,and it was diffrentent from Child Pugh.Conclusions It is of great significance that the liver pathology is useful for the treatment of WD.If liver pathology supportes,the indication of LRLT can be measurably broadened.

Acupuncture Therapy ; instrumentation ; Adult ; Breathing Exercises ; Combined Modality Therapy ; Dysmenorrhea ; therapy ; Exhalation ; Female ; Humans ; Needles

OBJECTIVE:To provide reference for regulating pediatric clinical drugs. METHODS:Package inserts of pediatric drugs used by the outpatient,inpatient and emergency pharmacies of our hospital during January and March in 2015 were collected. The information about pediatric drugs in the package inserts,including drug name,indications or functions,specification,adminis-tration and dosage,was calculated and analyzed. 815 kinds of drugs applicable to adults and children in our hospital were studied to summarize and analyze the dosage form,specification and dose of pediatric applicable drugs. RESULTS:438 package inserts were collected,including 327 related to chemicals and biologicals among which those imported or produced by sino-foreign joint venture were labeled with more complete information about drug use for children compared to those made in China,and 111 rele-vant to Chinese patent medicines and natural medicines for which there were significantly insufficient information about drug use for children. The applicable drugs with the dosage form for children and those with the specification therefor respectively accounted for 51.17% and 31.65% of the above-mentioned 815 kinds of drugs,and were mainly available as injections,tablets or capsules, granules and oral liquid. The drugs labeled with the dose based on the age group of children such as infant,preschooler,school child and adolescent(1-18 years old)accounted for 61.24% of those with the specification for children,the drugs labeled with the dose for babies (28 d-1 year old) accounted for 26.74% thereof,and the drugs labeled with the dose for neonatus (younger than 28 d)accounted for 12.02% thereof. CONCLUSIONS:The label information and variety of pediatric applicable drugs are marked-ly insufficient. It is suggested that our country should formulate relevant policies,strengthen the development of pediatric drugs and improve the data for clinical use thereof to ensure the safety of clinical use for children.

Objective To examine the role of c-Src activation in hepatitis B virus X (HBx) protein induced epithelial-mesenchymal transition (EMT) in liver cancer.Methods SMMC-7721 liver cancer cells were transfected with HBx gene to induce EMT and the activated c-Src expression was evaluated by Western blot.Both the morphological changes and the epithelial and mesenchymal markers expression (real-time PCR,western blot and immunocytochemistry) of HBx-transfected SMMC-7721 cell treated by c-Src kinase inhibitor PP2 and negative control PP3 were observed and compared,respectively.Results The activated c-Src expression in HBx gene transfected SMMC-7721 cells was significantly increased compared to that in mock transfected cells,c-Src kinase inhibitor PP2 could enable the HBx-transfected SMMC-7721 cells to transmit from spindle-like shape to original epithelial morphology.Western blot and immunocytochemistry confirmed that the expression of epithelial markers and mesenchymal markers almost returned to the levels of parental cells,indicating the mesenchymal-epithelial transition.Conclusions c-Src activation plays a key role in the process of EMT induced by HBx protein in SMMC-7721 cells.

Objective To investigate the effects of different duration hypotension thresholds on p-Tau-181 and Aβ-42 protein expression and cognition in rats. Methods Thirty-nine healthy male SD rats were randomly di-vided into 4 groups:the control group(group C,n=9),the hypotension group(groupA1、A2、A3 ,n=10). The blood pressure of groupA1、A2、A3 was measured in different time of 2 h、4 h、6 h ,for 5 days. The antihyperten-sive group of mean arterial pressure(MAP)were maintained in the 50~55 mmHg safe range. Morris water maze was used to detect the spatial learning and memory ability of rats. The levels of Aβ42 and p-Tau-181 were detected by ELISA. Results There was no significant difference in mortality of rats in each group (P > 0.05). Compared with the group C,the escape latency and swimming distance of A2 group and A3 group were increased(P<0.05). In 3~7 days after operation,the cerebrospinal fluid P-Tau-181 and Aβ42 protein expression increased in the A2 group and A3 group compared with the A1 group(P<0.05). The escape latency and swimming distance of the A2 group and the A3 group were significantly longer than those in the control group. Aβ42 and p-Tau-181 were signifi-cantly increased in A3 group(P < 0.05). Compared with the A2 group,the increase of Aβ42 and p-Tau-181 in the A3 group was not significant(P<0.05). Conclusion Long-term controlled hypotension may lead to postoper-ative cognitive dysfunction which may relate to the increase of Aβ42 and p-Tau-181 protein expression.

OBJECTIVETo investigate the protective effect of high density lipoprotein on the cardiac function of rats with severe burns.

METHODSOne hundred and thirty-five Wistar rats were employed in the study and were randomly divided into control (n = 15, without treatment), burn (n = 60, with 30% TBSA full-thickness burn on the back) and experimental (n = 60, with the injection of HDL (80 mg/kg) via the caudal vein immediately after burns) groups. The rats in the groups with burn injury were resuscitated with intraperitoneal isotonic saline (50 ml/kg) 30 minutes after burn (PBM). The serum contents of CK, ICAM-1 and TNF-alpha of the rats of all the three groups were determined with corresponding methods. The histological changes in the cardiac muscle tissue of the rats in all groups were observed under light microscope and electronic microscope.

RESULTSThe serum contents of CK, ICAM-1 and TNF-alpha in the control group were obviously lower than those in burn group (P < 0.01), while those in experimental group were also markedly lower than those in control group (P < 0.05 or 0.01). The average reduction rate was 36.5%, 32.0% and 12.6%, respectively. The size and the structure of the cardiac muscular fiber in the control group were even and normal. Compared with the burn group, degeneration, inflammatory infiltration and mitochondrial swelling were found to be less marked in the experimental group at 48 PBH, and no focal lysis and necrosis were found, which were observed in the burn group.

CONCLUSIONHigh density lipoprotein can be beneficial to the protection of cardiac tissue in protecting from secondary injury in rats with severe burns.

Animals ; Burns ; blood ; physiopathology ; Creatine Kinase ; blood ; Intercellular Adhesion Molecule-1 ; blood ; Lipoproteins, HDL ; blood ; Myocardium ; cytology ; metabolism ; Rats ; Rats, Wistar ; Tumor Necrosis Factor-alpha ; metabolism

Background Researches demonstrated that corneal dystrophy is associated with the mutation of transforming growth factor beta induced gene(TGFBI)located at chromosome 5q31 domine.Recent study showed that the gene mutation location is in R124H of TGFBI gene. Objective This study was to identify the mutation characteristics of TGFBI gene in a Chinese family with Avellino corneal dystrophy. Methods This Chinese family with Avellino corneal dystrophy were determined and surveyed in Peking University Third Hospital.Periphery blood from 8 patients with Avellino corneal dystrophy and 2 unaffected subjects were collected from a Chinese family with corneal dystrophy for the extraction of DNA.Exons 4,11,12 of the TGFBI gene were amplified by polymerase chain reaction(PCR),and the amplified products were sequenced directly and compared the gene sequence with that of TGFBI in GenBank.Written informed consent was obtained from each Subject prior to any medieal process. Results This family included 27 members of consecutive 4 generation.The hereditary pattern W88 in accordance with the autosomal dominant inheritance.Directly sequencing of 8 affected members revealed a G tO A transition at codon 124 (CGC to CAC),producing R124H mutation of TGFBI gene.Two synonymous single nucleotide polymorphism(SNP)of TGFBI gene occurred in the family.including a C to T transition at eodon 472(CTC to CTT)in 8 members,and a T to C transition at codon 540(TTT>TTC)in 9 members,which wag unrelated with disease. Conclusion R124H mutation of the TGFBI gene is found in this Chinese family with Avellino corneal dystrophy.

Objective Evaluate the effects of venous-arterial modified ultrafiltration on hemodynamics compared to arterial-venous in children undergoing cardiopulmonary bypass (CPB) for repair of congenital heart defects. Methods Forty patients underwent MUF randomly divided into two groups,group V-A MUF (n =20) and group A-V MUF (n =20) for 10 min after CPB. They were studied before CPB, after CPB, 10 min after CPB, and 30 min after CPB. Haemodynamic data including heart rate, blood pressure, central venous pressure and hematocrit were recorded. Transoesophaegeal echocardiography determined left ventricular posterior wall thickness in end-systole ( LVPWs) and end-diastole (LVPWd) , end diastolic volume (EDV) , end systolic volume (ESV) and ejection fraction (EF) were measured and compared in two groups. Results Patients in V-A MUF maintained better systolic arterial blood pressure at 10 min and 30 min compared with 0 min values after CPB. A significant decrease in EF were observed in both groups immediately after CPB ( P < 0.05 ). Significant increase in EF was observed at 10 min (60% ) and 30 min (46% ) after CPB compared with 0 min value after bypass in V-A MUF (P <0.001 ). In A-V MUF, no such increase in EF was observed. EF were significantly higher at 10 min and 30 min in V-A MUF as compared with A-V MUF (P < 0. 001). There was also significant improvement in posterior wall thickness in V-A MUF (P <0.05). Haematocrit values were not different in duration of postoperative between two groups. Conclusion Veno-arterial modified ultrafiltration is a safe and effective method of improving hemodynamics in children following cardiac surgery.

Objective To induce cord blood derived endothelial progenitor cells(EPCs)into endothelial cells,and investigate the feasibility of these cells as the seed cells of tissue engineering cardiovascular replacement.Methods Mononuclear cells were isolated from fresh cord blood by 6%HES and density gradient centrifugation.Isolated cells were cultured in medium supplemented with vascular endothelial growth factor(VEGF).Attached cells were identied by morphology,immunofluorescence staining and flow cytometry.Results The percentage of mononuclear cells isolated from fresh cord blood was(3.4?2.1)?10~7/mL.The morphology of attached cells changed while being cultured and inducted,from small-sized round cells to spindle-like cells,to a typical cobblestone morphology,and the total number of cells was 10~7.After 7 days of culture,immunofluorescence staining showed that the vWF and VEGFR-2 were expressed.Compared with the original,cell markers CD_133 decreased(3.11%?1.05%) to(0.09%?0.02%),P

Objective To explore the clinical feature,diagnosis and treatment of postoperative chylothorax after congenital cardiac surgery.Methods From Jan.2000 to Jan.2008,36 consecutive infants following cardiac surgery were diagnosed postoperative chylothorax.Age at operation ranged 6 days to 4 years.Weight ranged 3.5 kg to 17.0 kg.Indications for cardiac surgery were:tetralogy of Fallot 18 cases,complete atrioventricular septal defect 5 cases,double outlet of right ventricle 4 cases,complex heart defect for Fontan operation 4 cases,coarctation of the aorta 2 cases,total anomalous pulmonary venous drainage 2 cases,patent ductus arteriosus 1 case.Chylothorax occurred in right side was 32 cases,in left side 3 cases,bilateral side 1 case.Diagnostic time for chylothorax was postoperative 1-10 d.Results Thirty-four cases were treated conservatively with success(34/36,94.44%).Only 2 cases needed surgical treatment.The chest drainage volume before con-servative treatment was 22-60 mL/(kg?d)[mean 40.82 mL/(kg?d)].The mean duration of chylous drainage was 15 d(ranged 7 d to 42 d).There was no death in hospital.Two children had sepsis.The cases were followed up 2 weeks-7 years.One newborn was abandoned because of the other reason after discharge.There was no recurrence of chylothorax.Twenty-two cases were still in low-fat diet.Conclusions The chylothorax following operation of congenital heart disease should be noticed and early diagnosed.The initial treatment is conservative,but surgical treatment ought to be considered if the conservative management is unsuccessful.J Appl Clin Pediatr,2009,24(1):70-72