Objective To observe the efficacy of imatinib on the treatment of bcr-abl positive essential thrombocythemia (ET). Methods A case of bcr-abl positive ET resistant to hydroxyurea (HU) treating with imatinib (200～400 mg/d) was reported and related literatures were reviewed. Results A case of bcr-abl positive ET was initially treated with 1.5～2.0 g/d HU, the platelet count decreased to 562x109/L after 4 weeks; however, the platelet count increased to (1020～1330)×109/L treating with same dose of HU 16 months later. With the elevation of HU to 3.0 g/d, platelet count was still high(1290～1780)x109/L companied with the very low white blood cell count(0.3～0.9)×109/L. While treating with imatinib (400 mg/d) for 1 month,the platelet count decreased to 390×109/L and white blood cell count was 0.5×109/L; Furthermore, treating with 200×300 mg/d of imatinib, the platelet and white blood cell count recovered in normal after 1 month,and bcr-abl fusion gene negative 2 months later. Conclusion Imatinib may be the effective targeting drug for the bcr-abl positive ET, and the bcr-abl positive ET is sensitive to low dose imatinib.

Objective To compare and evaluate two type-specific primers PCR genotyping methods of hepatitis B virus ( HBV) which were established by Naito et al ( Naito method) and our lab (new method). Methods The two genotyping methods were applied for detecting the plasmids containing the HBV genomes of genotype A or D or subgenotype B1 or C2 and the plasmids mixed with different proportion of subgenotypes B1 and C2. In addition, the genotypes of 113 serum samples of patients with chronic HBV infection from Shenzhen, Handan and Urumqi cities of China were identified by the two methods, respectively. The results were verified by PCR product based sequencing. Results The sensitivity of the two methods showed no difference when they were applied to detect the plasmids containing the HBV genomes of genotype A or D or subgenotype B1 or C2. While detecting the plasmids mixed with different proportion of subgenotypes B1 and C2, the sensitivity of the new method was superior than that of Naito method. Meanwhile, the specificity of the new method was obviously superior than that of Naito method. Both of the two methods were highly sensitive in identification of HBV genotypes of serum samples with a single genotype. However, the new method showed more sensitive in identification of the B/C mix strains than that of Naito method. The total coincidence rate between the two methods was 83. 2% (94/113), with the discrepancy of 16. 8% (19/113). Fifteen of the 19 inconsistent genotypes by the two methods were selected and their PCR products were sequenced directly. The sequencing results were identical with that of the new methods, but not with that of the Naito method. Conclusion The new method is more sensitive, and its specificity is superior to the Naito method. It could be used for clinical and epidemiological studies on HBV genotype and subgenotype in China.

OBJECTIVETo investigate the changes of NF-kappa B binding activity, the expression of PPARr and their correlation in the liver of rats with fatty liver disease (FLD) induced by different pathogenic factors and to investigate the molecular mechanism of the inflammation in FLD.

METHODS40 Wistar rats were randomly divided into 4 groups of ten each: normal group, alcohol group, fat-rich diet group, alcohol adding fat-rich diet group. The rats were sacrificed at the end of the 16th week from the starting day of the experiment. Serum and liver specimens were collected. Histological specimens were stained with HE, SudanIV, and Masson and then studied microscopically. The ultrastructural changes were also checked under an electron microscope. NF-kappa B binding activity and the expression of PPARr mRNA were determined by electrophoretic mobility shift assay (EMSA) and RT-PCR respectively. The correlations between NF-kappa B binding activity and the expression of PPARr and the biochemical indexes were analyzed.

RESULTSSteatosis, inflammation, necrosis and fibrosis were present in livers of the rats of all the experimental groups, and were most severe in the alcohol adding fat-rich diet group. NF-kappa B binding activity was markedly increased in the livers of the alcohol group (142+/-16.32) and of the alcohol adding fat-rich diet group (238+/-19.14) in comparison to the livers of the normal (73+/-9.24, F = 6.36, 17.93) and those of the fat-rich diet group (84+/-10.38, F = 5.96, 16.20). Binding activity was higher in the alcohol adding fat-rich diet group than that in the simple alcohol group, but there was no difference between those of the fat-rich diet and normal groups. The level of PPARr mRNA was lower in the livers of the alcohol, fat-rich diet, alcohol adding fat-rich diet groups (0.2530+/-0.069, 0.3647+/-0.082, 0.1226+/-0.054) than that of the controls (0.8097+/-0.094) (F = 15.43, 7.24, 21.45). NF-kappa B binding activity was correlated positively with the level of serum TNF alpha (r = 0.527, 0.639) and the content of MDA in the liver homogenates (r = 0.723, 0.537), but negatively with the expression of PPARr in the livers of the alcohol and the alcohol adding fat-rich diet groups (r = -0.568, -0.891).

CONCLUSIONThe enhanced nuclear factors NF-kappa B binding activity and decreased expression of PPARr play a pivotal role in the inflammatory response of FLD induced by alcohol and fat-rich diet. It may provide a new idea for treating FLD effectively.

Animals ; Fatty Liver ; metabolism ; Liver ; metabolism ; Male ; NF-kappa B ; metabolism ; PPAR gamma ; biosynthesis ; genetics ; RNA, Messenger ; biosynthesis ; genetics ; Random Allocation ; Rats ; Rats, Wistar

Purpose To investigate the effect of EPCR on the proliferation and migration, and to explore the molecular mechanism of EPCR affecting the tumor growth and metastasis in human breast cancer cell line MCF-7. Methods MCF-7 cell was transfected with EPCR siRNA and treated with anti-PAR-1 antibody. Then CCK-8 assay was performed to determine the proliferation of MCF-7 cell. Transwell migration assay was employed to determine the cell's migration. Cell-ELISA was used to detect the activation of PAR-1 on the membranes of MCF-7. Result After EPCR siRNA transfection, the proliferation and migration ability of the MCF-7 in the interference of EPCR gene group was significantly decreased compared with the negative control and untreated control group. After treated with anti-PAR-1 antibody, the proliferation and migration of ability of MCF-7 were decreased significantly compared with the negative control group and the untreated control group. Cell-ELISA assay indicated that the activation of PAR-1 in the cells surface of MCF-7 cell in the EPCR gene interference group was mitigated versus the negative control and untreated control group. Conclusion EPCR may promote the proliferation and migration of MCF-7 cell by activating PAR-1.

Objective To evaluate the diagnostic value of rectal endoscopic ultrasonography for recto-vaginal endometriosis (RVEM).Methods The clinical data of 36 patients who met the criteria for RVEM between September 2009 and September 2016 in Shengjing Hospital were analyzed.Rectal endoscopic ultrasonography was compared with colonoscopy and pelvic magnetic resonance imaging (MRI) in terms of preoperative diagnosis and their conformance with surgical and postoperative pathological findings.Results Rectal endoscopic ultrasonography was superior to colonoscopy and pelvic MRI in diagnosing RVEM,invasive bowel disease,and the invasive level in the preoperative assessment (P ＜ 0.05),and in its conformance with surgical and postoperative pathological findings.Conclusion Rectal endoscopic ulwasonography is a reliable technique for diagnosing RVEM,with good accuracy in the preoperative diagnosis of invasive bowel disease and its level of invasiveness.

OBJECTIVETo study the value of measuring electrical discharge of external oblique in assessment of young rat model of visceral hypersensitivity.

METHODSEight-day-old neonatal Sprague-Dawley rats were randomly assigned to two groups: an experimental group and a control group (n=16 each). Rats in the experimental group were subjected to mechanical colorectal irritation daily for 7 consecutive days, while the rats in the control group did not received colorectal irritation treatment. On the 6th week of their lives, the spike amplitude of external oblique were measured to evaluate the bowel sensitivity.

RESULTSWhen the colorectal distention (CRD) pressure was 30 and 45 mmHg, the 95% confidence interval of the spike amplitude in the experimental group was significantly higher than that in the control group (P<0.01). When the CRD pressure were 60 and 75 mmHg, the 95% confidence interval of the spike amplitude in female rats was significantly higher than that in males (P<0.05).

CONCLUSIONSThe electrical discharge of external oblique confirmed that chronic colorectal irritation in neonatal rats can result in a chronic visceral hypersensitivity in the juvenile stage, with gender differences. Electrophysiological assessment is a quantitative test, and can objectively reflect visceral sensibility of pain.

Animals ; Colon ; physiopathology ; Disease Models, Animal ; Female ; Irritable Bowel Syndrome ; physiopathology ; Male ; Rats ; Rats, Sprague-Dawley ; Rectum ; physiopathology ; Reflex ; physiology

Objective: To study the mechanism of Heshi-Gejiugao on the nerve endocrine immune network in the treatment of perimenopausal syndrome. Methods: A total of 100 patients with perimenopausal syndrome were randomly divided into treatment group and control group, 50 cases in each group. The control group was only given general treatment, while the treatment group was treated with Heshi-Gejiugao on the basis of general treatment for 30 days. The clinical efficacy, Kupperman score, nerve, endocrine and immune related indexes of the two groups before and after treatment were observed. Results: The total effective rate was 96.0% (48/50) in the treatment group and 50.0% (25/50) in the control group. There was significant difference between the two groups (χ²=37.639, P<0.01). The Kupperman score (17.52 ± 2.73 vs. 24.22 ± 6.87, t=6.409) in the treatment group was significantly lower than that in the control group (P<0.01). After treatment, NE (1 878.08 ± 931.57 ng/m vs. 1 278.43 ± 866.32 ng/ml, t=3.331), DA (1 568.56 ± 597.15 ng/ml vs. 1 183.62 ± 798.72 ng/ml, t=2.729) in the treatment group were significantly higher than those in the control group (P<0.05); E₂ (42.12 ± 9.77 pg/ml vs. 35.91 ± 12.55 pg/ml, t=2.761), FSH (62.70 ± 15.96 mIU/ml vs. 72.67 ± 30.18 mIU/ml, t=2.065), LH (33.88 ± 12.18 mIU/ml vs. 42.93 ± 9.83 mIU/ml, t=4.089) were significantly lower than those of the control group (P<0.05). CD3⁺ (1 087.34/μl ± 432.19/μl vs. 918.27/μl ± 199.72/μl, t=2.511), CD4⁺ (738.16/μl ± 326.75/μl vs. 588.43/μl ± 212.55/μl, t=2.716) and CD4/CD8 (1.87 ± 0.56 vs. 1.16 ± 0.55), t=6.483) were significantly higher than those of the control group (P<0.05); CD8⁺ (788.32/μl ± 214.56/μl vs. 976.37/μl ± 318.62/μl, t=3.462) was significantly lower than that of the control group (P<0.05). Conclusions Heshi-Gejiugao can reduce the symptoms and improve the quality of life by regulating the multi target and multi direction of the neuroendocrine immune network of perimenopausal patients.

OBJECTIVETo investigate the efficacy of high dose chemotherapy combined with autologous peripheral blood stem cell transplantation (APBSCT) for the treatment of neural ectodermal solid tumor originated from neural crest in children.

METHODSTwenty-three children at a medium age of 5.8 + or - 3.5 years with neural ectodermal solid tumor originated from neural crest were enrolled. Of the 23 children, 20 with stage IV neuroblastoma (9 were in complete remission, 7 were in partial remission and 4 were in progressive disease), 2 with stage IV primitive neuroectodermal tumor (PNET) in complete remission, and 1 with retinoblastoma in partial remission. Before APBSCT the children received 8.0 + or - 4.3 courses of chemotherapy. During chemotherapy the autologous peripheral blood stem cells were harvested and the tumor excision was performed. Then APBSCT was performed.

RESULTSThe reconstruction of the hematopoietic system was noted in 19 of 20 children with stage IV neuroblastoma 16.5 + or - 0.9 days after transplantation. A follow-up (median 15.8 months) was done in these children. The follow-up showed that the survival rate in children in complete remission before transplantation was 100%, 57% in those in partial remission, and none of children in progressive disease survived (P<0.05). The total survival rate was 67% in children with neuroblastoma. The child with retinoblastoma had complete remission in a 6-months follow-up. The tumors recurred in children with PNET 5 to 8 months after transplantation and all died within one year after transplantation.

CONCLUSIONSHigh dose chemotherapy combined with APBSCT can result in a good outcome in children with neural ectodermal solid tumor originated from neural crest in complete remission before transplantation and can improve the outcome in patients in partial remission before transplantation. However, the children with PNET, even in complete remission before transplantation, do not respond to the therapy.

Antigens, CD34 ; analysis ; Antineoplastic Agents ; therapeutic use ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Neural Crest ; pathology ; Neuroblastoma ; therapy ; Neuroectodermal Tumors, Primitive ; therapy ; Peripheral Blood Stem Cell Transplantation ; Transplantation, Autologous

OBJECTIVETo map the candidate gene by linkage analysis in a Chinese family with autosomal dominant congenital retinaochoroidal coloboma.

METHODSA detailed clinical examination was performed for all patients in the family. The genomic DNA of all family members was extracted from peripheral blood leukocytes. Linkage analysis and genome-wide linkage screening was conducted using fluorescent detection of 398 microsatellite markers representing all autosomes at an average resolution of approximately 10 cM. Polymerase chain reaction was carried out to amplify all 398 microsatellite markers. The allele sizes were determined on ABI 3130-Avant genetic analyzer according to an internal size standard, and the results were analyzed using Genescan 3.1 and Genotyper 2.0 software.

RESULTSLinkage analysis showed the markers D2S2382-D2S301-D2S2244-D2S163 co-segregated with the disease locus in all affected members. The maximum Lod score was 3.01(D2S2382).

CONCLUSIONThe candidate region of the disease gene in the family was located in 2q34-2q35.

Asian Continental Ancestry Group ; Chromosome Mapping ; Coloboma ; genetics ; DNA Mutational Analysis ; Family ; Female ; Genetic Linkage ; Genotype ; Humans ; Lod Score ; Loss of Heterozygosity ; Male ; Microsatellite Repeats ; genetics ; Myopia ; genetics ; Pedigree ; Polymerase Chain Reaction

BACKGROUNDNatural disasters have been frequent in recent years. Effective treatment of patients with cardiovascular disease following natural disasters is an unsolved problem. We aimed to develop a novel miniature mobile cardiac catheterization laboratory (Mini Mobile Cath Lab) to provide emergency interventional services for patients with critical cardiovascular disease following natural disasters. A feasibility study was performed by testing the Mini Mobile Cath Lab on dogs with ST-elevation myocardial infarction (STEMI) model in a hypothetical natural-disaster-stricken area.

METHODSThe Mini Mobile Cath Lab was transported to the hypothetical natural-disaster-stricken area by truck. Coronary angiography and primary percutaneous coronary intervention (PCI) were performed on six dogs with STEMI model. The transportation and transformation of the Mini Mobile Cath Lab were monitored and its functioning was evaluated through the results of animal experiments.

RESULTSThe Mini Mobile Cath Lab could be transported by truck at an average speed of 80 km/h on mountain roads during daytime in the winter, under conditions of light snow (-15°C to -20°C/-68°F to -59°F). The average time required to prepare the Mini Mobile Cath Lab after transportation, in a wetland area, was 30 minutes. Coronary angiography, and primary PCI were performed successfully.

CONCLUSIONThis preliminary feasibility study of the use of the Mini Mobile Cath Lab for emergency interventional treatment of dogs with STEMI indicated that it may perform well in the rescue of critical cardiovascular disease following natural disasters.

Angioplasty, Balloon, Coronary ; Animals ; Cardiac Catheterization ; Cardiovascular Diseases ; therapy ; Coronary Angiography ; Disasters ; Dogs ; Electrocardiography ; Feasibility Studies ; Laboratories ; Myocardial Infarction ; therapy