Objective To evaluate the significance and expression of the GDNF/GFRα1/RET genes in distal rectum with Congenital Anorectal Malformations(ARMs) Methods Specimens were collected from resected colon which is 3 cm away to the anus and the distal of rectum in 12 ARMs patients. Haematoxylin and Eosin (HE) staining, immunohistochemical (IHC) staining and reverse transcription PCR (RT-PCR) were used to test RET, GDNF and GFRα1 expression in the differ-ent site of samples with ARMs. Results Expression of ganglia and RET, GDNF and GFRα1 were all negative in distal rec-tum in 12 ARMs patients. Ganglionic cells were found in tissues 3 cm away from the anus where RET, GDNF and GFRα1 expression were also positive in those ARMs patients. Expression of RET, GDNF and GFRα1 were strong positive in middle and low imperforate anus indicated by brown color and week positive in high imperforate anus indicated by yellow color. One case of faeces contamination and one case of loose motion without anal incontinence were found in post-op follow up of high ARMs patients. By contrast, one case of rectal mucosa prolapse and one case of occasional faeces contamination which recov-ered with hip bath were found in post op follow up in middle or low ARMs. Conclusion The unsatisfactory anorectal func-tion is possibly related to the decrease or lost of neurotrophic factors (GDNF/GFRα1/RET) and ganglia in the myenteric plex-uses post plastic surgery in ARMs patients.

Objective To investigate the relationship between the methylation of RET (proto-oncogene, RET) and Hirschsprung disease (HD), and understand its significance in the development of intestinal wall ganglion cells. Methods Twenty-one surgical removal specimens, which were all dilation segment of HD in Tianjin Children’s Hospital, were used as experimental group, and 5 samples of non-HD normal colon tissues were used as control group. The bisulfite sequencing (BSP)-direct detecting method was used to detect RET CpG island methylation status. The expression of RET protein was detected by immunohistochemistry in experimental group and control group. Results In the experimental group 12 cases (57.14%) were found methylation, but no methylation was found in control group. The average optical density of methylated RET protein was 0.201±0.015 in 12 cases. The average optical density of un-methylated RET protein was 0.364±0.023 in 9 cases (P<0.05). Conclusion RET CpG island methylation reduced protein expression levels of RET. The corollary RET gene methylation may influence the expression levels of RET protein, thereby affecting the ganglion cell development, and thus participating in the occurrence of HD.

The early screening and early diagnosis of biliary atresia is the key to improve the 5-year native liver survival rate,jaundice-free-native-liver survival rate and overall survival rate.So far,the neonatal jaundice,especially that caused by surgical cholestasis exists the lack of awareness.Because of this reason,the delay depletion jaundice patients did not receive early diagnosis and treatment until the severe complications happened.So,it has often missed the best period of operation for the biliary atresia.Called for local medical health governments to formulate the effective early screening methods of biliary atresia and the reasonable schedule of postnatal follow-up,and improving the ability of basic unit medical staff in well understanding cholestasis disease in order to achieve early diagnosis and treatment.Consequently,it will improve the situation of diagnosis and treatment of biliary atresia in our country.

Objective To investigate the effects of apoptosis and related gene p21WAF1 and Bcl-2 in the tumorigenesis of primary gallbladder neoplasms. Methods p21WAF1 and Bcl-2 were detected by immunohistochemistry and apoptotic cells were stained in situ by terminal deoxynucleotidyl transferase mediated-dUTP nick end labeling(TUNAL) in 46 cases of primary gallbladder neoplasms with different histological grades. Results Apoptotic index and p21WAF1 were decreased,but the expression of Bcl-2 was increased with the increasing of the histological grades in primary gallbladder neoplasms (P

OBJECTIVE:To observe clinical efficacy and safety of recombinant human interleukin-11 (rhIL-11) in the treat-ment of chemotherapy-induced thrombocytopenia. METHODS:86 patients with thrombocytopenia induced by chemotherapy were selected and divided into control group and observation group according to random number table,with 43 cases in each group. Con-trol group was given platelet transfusion 10 IU,once every 2-3 d;observation group was given Recombinant human IL-11 for injec-tion 25-50 μg/kg,qd. Both groups received treatment for 14 d. Clinical efficacies of 2 groups were observed as well as platelet count before and after treatment. The duration of platelet decrease and recovery,the occurrence of ADR were compared between 2 groups. RESULTS:The total effective rate of observation group was 81.40%,which was significantly higher than 62.79% of con-trol group,with statistical significance (P<0.05). There was no statistical significance in platelet count between 2 groups before treatment(P>0.05);after treatment,platelet count of 2 groups increased significantly,and the observation group was significant-ly higher than the control group,with statistical significance(P<0.05). The duration of platelet count <50×109 L-1,the time of platelet count recovering to 70×109 L-1 and 100×109 L-1 in observation group were significantly shorter than in control group,with statistical significance(P<0.05). There was no statistical significance in the incidence of ADR between 2 groups(P>0.05). CON-CLUSIONS:rhIL-11 shows good therapeutic efficacy in the treatment of chemotherapy-induced thrombocytopenia,and can signifi-cantly improve platelet count,shorten the duratione of platelet decrease and recovery with good safety.

Adult ; Blood Glucose ; metabolism ; Female ; Herbicides ; poisoning ; Humans ; Liver ; physiopathology ; Male ; Middle Aged ; Paraquat ; poisoning

Phage display is a new technology developed in recent years. Random peptides displayed on the filamentous phage surface offer a rich source of molecular diversity in searching for ligands that bind an antibody, receptor and other proteins. The phage display system has been effective in the discovery of various ligands. Potential applications of the peptide library include investigation of the protein protein interactions and discovery of mimetic drug candidates.

OBJECTIVETo explore the effect of combination of Epimedii Folium and Ligustri Lucidi Fructus on osteoporosis rats induced by retinoic acid.

METHODSixty three-month-old male Wistar rats were randomly divided into the normal control group, the model group, the Epimedii Folium group, the Ligustri Lucidi Fructus group, the combination group of Epimedii Folium and Ligustri Lucidi Fructus and the raloxifene group. The osteoporosis model was established through oral administration with retinoic acid for two weeks. Meanwhile, all of treatment groups were administered with corresponding drugs for three weeks. The contents of serum calcium (Ca), phosphorus (P), alkaline phosphatase (AKP) and tartrate-resistant acid phosphatase (StrACP) were detected, and the pathomorphological changes of femurs were observed.

RESULTThe model control group showed much lower contents of serum Ca and P than the normal control group, but with significantly higher AKP and StrACP activity than the normal control group. The femoral head area showed reduced, narrow and sparse trabecular bones, with typical osteoporosis-like changes. Compared with the model control group, all of treated groups showed significant increase in Ca and P contents in serum, and down-regulate AKP and StrACP levels, while trabecular bones became more and wider, and densely interweaved as a reticular formation. Among them, the combination group showed the most significant effect.

CONCLUSIONEpimedii Folium and Ligustri Lucidi Fructus could effectively correct the abnormal bone metabolism and improve pathological conditions of bone tissues, so as to show the anti-osteoporosis effect. The combined application of the two drugs showed a better efficacy.

Acid Phosphatase ; blood ; Alkaline Phosphatase ; blood ; Animals ; Body Weight ; drug effects ; Calcium ; blood ; Drug Interactions ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Epimedium ; chemistry ; Femur ; drug effects ; pathology ; Isoenzymes ; blood ; Ligustrum ; chemistry ; Male ; Osteoporosis ; blood ; chemically induced ; drug therapy ; pathology ; Phosphorus ; blood ; Rats ; Rats, Wistar ; Tartrate-Resistant Acid Phosphatase ; Tretinoin ; adverse effects

BACKGROUND: In recent years, the progression of transplantation of allografts has put forward to a new way to repair massive bone and joint defect caused by various reasons, which was a difficult subject in the past.OBJECTIVE: To probe into the effect of massive allograft and interlocking intramedullary nail on rebuilding bone and joint after bone tumor resection.DESIGN: Self-control observation SETTING: Department of Orthopaedics, Yunnan Provincial Tumor Hospital PARTICIPANTS: From April 1998 to January 2003, totally 18 patients with 15 cases of malignant bone tumor and 3 of osteoma-like disease, accepted bone tumor focus removal followed by intrmedullary nailing and allogenic bone transplantation, consented to join the study, were recruited.INTERVENTIONS: Allografts were taken from fresh cadaveric bone (Prepared and stored in Yunnan Provincial Tumor). The bone segment of osteoma was cut completely and the allografts were transplanted at the bone defects. The proximal and distal intrmedullary nailing were inserted in order. Auto cancellous bone particles were transplanted at the bone joint to promote the healing at bone end. The upper part of shoulder bone was transplanted to perform reconstruct of retotar and triangular muscle; half knee joint was transplanted to attach important muscle and ligament. After external fixation of 4 to 6 weeks following operation, the functional exercise was conducted in order sequence. Evaluating the curative effect of functional recovery of the limbs at postoperative 3,6 and 12 months as well as follow-up was carried out according to the criteria of Mankin(excellent ,good , moderate , unsatisfied).MAIN OUTCOME MEASURES: ①Functional recovery of limb of the patients. ② Adverse and side effectRESULTS: Date of totally 18 patients was entered result analysis. ①Functional recovery result of the limbs: According to evaluated criteria put forward by Mankin. In this group, 7 cases of excellent, 6 of good, 4 of moderate and 1 of unsatisfied. Bony callus and the concentration of nuclide were increased gradually in the examination at month 6 and 12. About 10-14 months after operation, the osteotylus would cover the end of allograft, substituted the allograft gradually. ② Adverse events and side effect:In this group, no tumor was survived in 16 cases, two cases died of primary lesion and pulmonary metastasis at postoperative 12 months and 18 months respectively. No infection, bone nonunion, flexible internal fixation or ruptures were found in the limbs of other patients. No one was found with shortened limbs. The patient with part joint transplantation has limitation in joint move, but the joint was stable.CONCLUSION: This operation has the highest stabilization .The patients can do exercise earlier to recover function, and injury arthritis could be prevented. It is suitable to rebuild the bone after bone tumor resection.

Objective To investigate the efficacy of sunitinib in the treatment of patients with imatinibresistant advanced gastrointestinal stromal tumor (GIST).Methods The clinical data of 45 patients with imatinib-resistant advanced GIST who received the treatment of sunitinib (37.5 mg/d) at the First Affiliated Hospital of Sun Yat-Sen University from March 2008 to June 2012 were retrospectively analyzed.The mutation of c-kit and platelet-derived growth factor receptor α (PDGFRα) was detected,and the efficacy of imatinib was assessed after the treatment for 3 months,and factors influencing the survival were analysed.The survival rate was calculated using the Kaplan-Meier method,survival analysis was done using the one-way analysis of variance,and multivariate analysis was done using the COX regression model.Results The median time of treatment with sunitinib for the 45 patients was 11.0 months (range,4-37 months).The complete remission rate,partial response rate,rate of stabilized condition and disease progression rate were 15.6％ (7/45),8.9％ (4/45),46.7％ (21/45) and 28.9％ (13/45) after the treatment with sunitinib for 3 months.All the patients with clinical (imaging) complete remission received surgery for metastatic lesions or B-ultrasound guided ablation for single liver metastasis before the treatment with sunitinib.The most common grade 3 or 4 adverse reactions of sunitinib were hand-foot syndrome and anemia.C-kit and PDGFRα mutational analysis were carried out.C-kit exon 9 mutation was detected in 9 patients,c-kit exon 11 mutation in 21 patients,and no mutation was detected in 12 patients.The median progression-free survival time was 8.0 months (range,4.1-11.9 months),and the median overall survival time was 25.0 months (range,13.4-36.6 months).The results of univariate analysis showed that the primary lesion sites and mutational status of primary lesions were factors influencing the progression-free survival and overall survival (x2=5.967,6.622 ; 7.965,8.765,P ＜ 0.05).The results of multivariate analysis showed that only the mutational status of c-kit of primary lesions was the independent factor influencing the progression-free survival and overall survival (Wald =6.540,7.205,P ＜ 0.05).The progression-free survival and overall survival of patients with c-kit exon 9 mutation and patients with no gene mutation were significantly longer than patients with c-kit exon 11 mutation (x2 =7.965,8.765,P ＜ 0.05).Conclusion Sunitinib with a dosage of 37.5 mg/d could effectively treat patients with imatinib-resistant advanced GIST.A better survival is observed in patients with c-kit exon 9 mutation or with no gene mutation when compared with patients with c-kit exon 11 mutation.