BACKGROUND:Transplantation of autologous stem cells for treatment of liver cirrhosis has been widely reported.But up to now,there exist some concerns for clinical physicians,including relationship between stem cells and post-transplantation prognosis/turnover of liver cirrhosis,directed differentiation of stem cells in the impaired liver,and malignant phenotype.OBJECTIVE:To dynamically monitor the serum levels of alpha-fetoprotein(AFP)and AFP variants(AFP-L3)in decompensated cirrhosis patients following intrahepatic transplantation of peripheral blood stem calls via portal vein and evaluate the safety of this treatment method.METHODS:A total of 44 decompensated cirrhosis patients who underwent intrahepatic transplantation of peripheral blood stem cells via portal vein in the 309 Hospital of Chinese PLA in April 2007 were included in this study.Prior to and after surgery,serum levels of AFP and AFP-L3 were detected by chemiluminescence.Through the use of a positive criterion for liver cirrhosis,i.e.,the proportion of AFP-L3 in AFP[AFP-L3(%)]≥10%,and the relationship between decompensated cirrhosis treatment using stem cells transplantation and the malignant phenotype of liver cancer were analyzed.RESULTS AND CONCLUSION:At 2 months after surgery,serum level of AFP showed a transient increase.There was no significant difference in AFP-L3(%)between prior to and after surgery(P>0.05).No significant difference in AFP-L3-positve rate,as well as AFP-L3(%),existed among patients with different serum level of AFP.These findings indicate that clinical symptoms and liver function of decompensated cirrhosis patients recovered to some extent after transplantation of peripheral blood stem cells via portal vein.Results regarding serum level of AFP-L3,a serological marker of liver cancer,did not demonstrate the appearance of malignant biological phenotype.

Objective To investigate the expression and significance of adaptor protein containing PH domain,PTB domain,and leucine zipper motif 1 (APPL1) in gastric cancers.Methods Expressions of APPL1 protein and mRNA were detected with immunohistochemistry and reverse transcription-polymerase chain reaction (RT-PCR) in 47 surgical specimens of gastric carcinomas (GC) and 27 normal gastric tissue specimens,respectively.Results The expression rate of APPL1 protein was 59.6％ (28/47) in GCs,and 26.1％ (6/23) in normal gastric tissues,with a statistically significant difference between two groups (P ＜0.05).The relative expression of mRNA was 0.821 ±0.141 in GCs,and 0.731 ±0.112 in normal gastric tissues,with a statistically significant difference between two groups (P ＜ 0.05).Conclusions The expressions of APPL1 protein and mRNA are increased in GCs,with a close relationship between GC and APPL1.

Child ; Graft vs Host Disease ; prevention & control ; Hematopoietic Stem Cell Transplantation ; methods ; Humans ; Immunosuppressive Agents ; therapeutic use ; Lymphohistiocytosis, Hemophagocytic ; genetics ; therapy ; Male ; Membrane Proteins ; genetics ; Mutation ; Prognosis ; Transplantation Conditioning ; methods ; Treatment Outcome ; Unrelated Donors

0.05. Conclusion The tumour chemosensitivity test in vitro gave some prediction and guidances for the clinic chemotherapy,and it could discover the drug resisting cases.The combined chemotherapy should be selected for gastric carcino- ma patients.

Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.
Adolescent ; Brain Diseases/diagnosis/*etiology/surgery ; Diagnosis, Differential ; *Hematopoiesis, Extramedullary ; Humans ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed ; beta-Thalassemia/*complications

In this paper, we studied the relationship between the prostaglandin F(2alpha) (PGF(2alpha))-induced cardiac hypertrophy and calcineurin (CaN) signal transduction pathway in vivo and in vitro. Male Sprague-Dawley rats were given a single i.p. injection with monocrotaline (MCT) (60 mg/kg) and then given orally with celecoxib (20 mg/kg) or vehicle once a day for 14 d before (from d 1 to d 14) or after (from d 15 to d 28) right ventricular hypertrophy (RVH) was formed. Body weight (BW), right ventricular weight (RV), left ventricular with septum weight (LV), as well as lung weight were determined. RVH index (RVHI=RV/LV), RV/BW, and lung weight/BW were calculated and histological changes were observed with transmission electron microscope. PGF(2alpha) level, atrial natriuretic peptide (ANP) and CaN mRNA expressions, expression of CaN and its downstream effectors, NFAT(3) and GATA(4) protein were assayed by EIA kit, RT-PCR, and Western blotting, respectively. The cardiomyocyte hypertrophy in primary culture induced by PGF(2alpha) (0.1 micromol/L) was evaluated by measuring the cell diameter, protein content, and ANP mRNA as well as CaN mRNA expressions. It was found that 14 d or 28 d after MCT was given, the RVHI, RV/BW, and lung weight/BW were significantly increased by 47%, 53% and 118%, and by 64%, 94% and 156%, respectively; at the same time PGF(2alpha) levels in RV tissue were increased by 44% and by 51% with increasing RVHI, and elevated expressions of ANP and CaN mRNA, as well as CaN, NFAT(3) and GATA(4) proteins in a positive correlation manner. Furthermore, some histological injuries were found in RV tissue. Celecoxib, a cyclooxygenase inhibitor, obviously blunted the elevation of RVHI, RV/BW, and lung weight/BW no matter it was given before or after RVH. In vitro experiments showed that 0.1 micromol/L PGF(2alpha) significantly increased the cardiomyocyte diameter and protein content, and promoted ANP and CaN mRNA expressions, which was blocked by cyclosporin A, a CaN inhibitor. Our results indicate that PGF(2alpha) may be involved in cardiac hypertrophy induced by MCT in rats through CaN signal transduction pathway.
Animals ; Calcineurin ; genetics ; metabolism ; physiology ; Cells, Cultured ; Dinoprost ; metabolism ; physiology ; Hypertrophy, Right Ventricular ; chemically induced ; metabolism ; physiopathology ; Male ; Monocrotaline ; Myocytes, Cardiac ; metabolism ; pathology ; RNA, Messenger ; genetics ; metabolism ; Rats ; Rats, Sprague-Dawley ; Signal Transduction ; physiology

OBJECTIVETo compare the physicians' lipid lowering drug prescribing behavior and knowledge on dyslipidemia before and at 8 months after new-issued blood-lipid reports in our hospital.

METHODBlood-lipid reports in our hospital is newly modified in that the classification of dyslipidemia and lipid-lowering guideline and target lipid level are listed on the back of lipid report besides the normal lipid value listed immediately after the measured lipid levels. Physicians' lipid lowering drug prescribing behavior and knowledge on dyslipidemia before and at 8 months after new-issued blood-lipid reports were examined in 143 doctors from various departments before and at 8 months after new-issued lipid reports.

RESULTSAt 8 months after the new issued lipid reports, doctors' cognition rate about the guideline was significantly increased [83.9% (120/143) vs. 67.1% (96/143), P < 0.001] and the guideline was considered more helpful on daily practice [75.3% (58/77) vs. 55.8% (43/77), P = 0.005] compared to baseline. However, the prescription rate of dyslipidemia therapy did not change significantly (69.2% vs. 63.2%, P = 0.117) at 8 months after the new issued lipid reports.

CONCLUSIONSThe modification of the blood-lipid reports improved doctors' knowledge on dyslipidemia and on the "Chinese guidelines on prevention and treatment of dyslipidemia in adults". However, the lipid lowering drug prescribing behavior remained unchanged at 8 months after the modification of the lipid reports. Further investigation is warranted to see if the lipid lowering drug prescribing behavior could be changed in the long-term.

Dyslipidemias ; blood ; drug therapy ; Guideline Adherence ; Health Knowledge, Attitudes, Practice ; Humans ; Hypolipidemic Agents ; administration & dosage ; therapeutic use ; Lipids ; blood ; Physicians ; Practice Patterns, Physicians' ; Prescriptions ; Research Report

Objective To evaluate the effects of portoenterostomy (Kasai surgery) on living donor liver transplantation (LDLT) for children with biliary atresia (BA). Methods A total of 150 children with BA, who were treated with LDLT in our center from September 2006 to September 2014, were retrospectively analysed. The children were categorized into Kasai group (90 cases, 60%) and non-Kasai (60 cases, 40%) group, based on whether they had previously undergone Kasai procedure pre-LDLT. Clinical data, incidence of complications and accumulated survival rates were compared between two groups. Results The ages of pediatric patients were 4.9-87.0 months. The patient age and height were significantly higher in Kasai group than those of non-Kasai group (P<0.05). The serum bilirubin level was lower before surgery in Kasai group than that of non-Kasai group (P<0.05). There were no significant differences in body weight, pediatric end stage of liver disease (PELD) score, graft to recipient body weight ratio (GRWR), operation time and blood loss between two groups ( P>0.05). Similarly, there were no significant differences in pulmonary infection, acute rejection, portal vein thrombosis, hepatic artery occlusion and biliary complications between the two groups (P>0.05). The overall complication rate of post-LDLT was 61.1%in Kasai group, which was higher than that in non-Kasai group (43.3%,χ2=4.580, P=0.032). Totally, there were 7 cases (4.7%) died on post-LDLT, in which there were 6 cases (4.0%) in Kasai group including 5 cases of multiple organ
failure and 1 case of severe pulmonary infection, and 1 case (0.7%) in non-Kasai group, who died of multiple organ failure due to preoperative gastrointestinal bleeding for emergency surgery. There were no serious complications and death in donors. The overall cumulative survival rates were 98.6%, 96.6%, 94.9%and 92.7%in 1 month, 1 year, 3 years and 5 years after LDLT, respectively. And there were no significant differences in survival rates in 1 month, 1 year, 3 years and 5 years between two groups (χ2=1.490, P=0.222) with the rates of 98.9%, 96.5%, 93.8%, 91.3%in Kasai group and 98.3%, 96.6%, 96.4%, 95.5% in non-Kasai group. Conclusion Performing Kasai procedure can acquire satisfied results to pediatric patients with BA pre-LDLT, without increasing the incidence of major complications and mortality post-LDLT. And the accumulated survival rate is not different in pediatric patients received Kasai surgery compared with that in non-Kasai patient. Besides that, Kasai surgery might postpone the time of receiving LDLT, benefit to the growth of children and reduce the jaundice of pre-LDLT.

Objective To explore the distribution of dendritic cells (DCs) and the expression of adhesion molecules in rat kidney with unilateral ureteral obstruction (UUO),as well as the regulatory effect of anti-P-selectin lectin-EGF domain monoelonal antibody (PsL-EGFmAb) on adhesion,maturation and function of human DCs cultured in vitro.Methods UUO rat models were established,which were divided into sham group (n=6),untreated group (n=18)and treated group with PsL-EGFmAb(n=18).DCs were analyzed with Axioplan 2 microscopy,while P-selectin being observed by immunohistochemistry.CD34~+ stem cells were isolated from cord blood and cultured in 20%IMDM medium with SCF,GM-CSF,TGF-?1,Flt-3L and TNF-?in vitro.During development, PsL-EGFmAb was added and IL-10 served as control.FACS was performed to detect the expression of HLA-DR,CD1a,CD11c,CD54,CD83,CD80,CD86,CD209 (DC-SIGN) and CD62-P,-E,-L (P-,E-,L-selectin) on DCs.RT-PCR was performed to detect the expression of NF-_KB P50, P65 mRNA.MLR was performed to detect the stimulatory effect of DCs on T cell proliferation and ELISA to determine IL-12p70 amount.Results Comparing with Sham group,the expression of P- selectin was up-regulated among tubulointerstitium mainly on renal tubular epithelial cell after unilateral ureteral obstruction on day 1,while CD1a~+CD80~+DCs being also found in renal interstitium. The expression of P-seleetin and CD1a~+CD80~+DCs was increased evidently on day 7,and correlated with the degree of renal tubulointerstitial fibrosis closely.However,these changes became less conspicuous in rat treated with PsL-EGFmAb.In vitro experiment showed on day 5 after cultured with the induction of TNF-?,immature DCs highly expressed C-type lectin DC-SIGN of pattern recognition receptors;the expression of co-stimulatory molecules such as CD11c,CD83,CD80 and CD86 on mature DCs was up-regulated in paralleling with the mRNA level of NF-_KB;the secretion of IL-12 was enhanced,as well as displaying the features of antigen-presenting cells with a higher ability to induce proliferation of T lymphocytes in vitro.In addition,L-selectin expressed highly on immature DCs,but lowly on mature DCs,neither of two DCs expressed P- and E-selectin.Compared with the IL-10 treated group,PsL-EGFmAb had an inhibitory effect on DC-SIGN of DCs with down- regulating the mRNA level of NF-_KB.PsL-EGFmAb could also inhibited CD11c,CD83,CD80, CD86 expression,reduced secretion of IL-12,and inhibited T cell proliferation stimulated by DCs in vitro.Conclusion DCs may play a critical role on initiating the inflammatory injury of renal tubulointerstitium,and the inhibitory effect of PsL-EGFmAb on DC maturation and function correlated with the inhibition of DC-SIGN,which is mainly mediated through NF-_KB signaling pathway.

OBJECTIVE: To explore the clinical and genetic characteristics of a child with Pallister-Hall syndrome (PHS). METHODS: DNA was extracted from peripheral blood sample from the child and subjected to whole exome sequencing. Suspected variants were verified by Sanger sequencing of his family members. RESULTS: Genetic testing revealed that the child has harbored a heterozygous c.3320_3330delGGTACGAGCAG (p.G1107Afs×18) variant of the GLI3 gene. Neither parent was found to carry the same variant. CONCLUSION The c.3320_3330delGGTACGAGCAG (p.G1107Afs×18) frameshift variant of the GLI3 gene probably underlay the pathogenesis of PHS in this child. Genetic testing should be considered for patients featuring hypothalamic hamartoma and central polydactyly.
Humans ; Child ; Pallister-Hall Syndrome/genetics* ; Kruppel-Like Transcription Factors/genetics* ; Zinc Finger Protein Gli3/genetics* ; Polydactyly/genetics* ; Hamartoma/pathology* ; Nerve Tissue Proteins/genetics*