Objective To analyze genetic and clinical features of 14 children with ?-thalassemia intermedia in Guangxi area.Methods ?-thalassemia genes,?-thalassemia genes,single nucleotide polymorphism(SNP) at position-158 of()~G?-globin gene,AT repeats polymorphisms of DNase I-hypersensitive site 2 of the ?-globin gene cluster locus control region(?-LCR-HS2) were detected by PCR techniques.Clinical data were analyzed.Results Genotype:1.Seven cases were homozygous or compound heterozygous for nt-28(A→G).Among them,2 cases′ genotypes were nt-28/nt-28,1 case was ?~E/ nt-28,2 cases were ?~0/nt-28,1 case(?~0/nt-28) co-inherited()~G?158(T) and 1 case(?~0/nt-28) co-inherited simultaneously()~G?-158(T) and——SEA ?-thalassemia-1 genes.2.Three cases with ?~0/?~0 presented()~G?-158(T),and other 3 cases co-inherited——SEA ?-thalassemia-1 genes.3.One patient with ?~0/?~0 co-inherited()~G?-158(T) and——SEA ?-thalassemia-1 genes.4.Six cases carrying()~G?-158(T) had(AT)_9 N_(12)(AT)_(10) sequences in ?-LCR-HS2.Phenotype:The values of Hb,MCV,HbF of 14 patients were(75.9?9.7) g/L,(68.9?5.9) fL,66.9%?16.3%,respectively.Except for 2 cases with genotypes of nt-28/nt-28 and 1 case with ?~E/nt-28 who had never been transfused,the others had more severe symptoms and required irregularly transfusion.Conclusions In the 14 children with ?-thalassemia intermedia from Guangxi area,nt-28(A→G),()~G?-158(T) and——SEA ?-thalassemia-1 gene are main alleviating gene factors.Incidence of(AT)_9 N_(12)(AT)_(10) sequence in ?-LCR-HS2 in these patients is high.Patients who are homozygous for nt-28 or compound heterozygous for ?~E have milder phenotypes.

Objective To analyze the clinical and pathological characteristics of children with lupus nephritis(LN).Methods The data of 116 inpatients from Mar.2000 to Nov.2008 with LN were retrospectively analyzed.The clinical,immunochemical and pathological data were recorded.Renal tissue was observed by light microscopy and electron microscopy after HE,PAS,Masson and PASM staining according to WHO standards.Follow-up results showed complete remission,partial remission,disease activity,renal insufficiency and death.Results Of the 116 cases of LN,there were 27 male and 89 female with a ratio of male to female 1.03.3,and the mean age was(12.0?2.2) years.The incidence of nephrotic syndrome was 63.8 %(74 cases),and acute nephritis was 21.5%(25 cases).Class Ⅳ LN was the most frequent type(14 cases,50%) and classⅢ was next(25 cases,21.5%).In view of the outcome,rapidly progressive glomerulonephritis and class Ⅳ LN were the worst.LN was initially controlled in 96.5% of the patients.Relapses of LN were most caused by the intermittent treatment.Totally 32 cases showed different renal injury,and 6 cases progressed to death.Conclusions Renal biopsy is important to diagnosis,treatment and prognosis evaluation of LN.Long and regular treatment is important for children with LN.

Objective To study the effect of aquaporin 1 on intestinal capillary endothelial barrier in rats with experimental acute necrotizing pancreatitis (ANP).Methods In this study,160 male Sprague-Dawley rats were randomly divided into five groups:Control group ( n =32),ANP group (n =32),NS group,Dexamethasone group,and Acetazolamide group.Eight rats in each group were sacrificed at 3,6,12 and 18 h after induction of experimental models.Volume of ascites and levels of serum amylases were deternined at each time point.Pathological changes in intestine tissues were observed under electron microscope after HE staining.Capillary permeeabilities in intestine tissues were detected by Evans blue (EB) extravasation experiment.The mRNA and protein expressions of AQP1 in intestine tissue were determined by real-time PCR and Western blotting,respectively.Results Serum amylase level in ANP group was significantly higher than that in control group.Amylase level in dexamethasone group was lower than that in ANP group,and amylase level in acetazolamide group was higher than that in ANP group at 12 h (P ＜0.05 ) ; The concentration of EB in intestine tissues at each time point in ANP group was significantly higher than those in control group,and EB in dexamethasone group was lower than those in ANP group at 6,12 and 18 h.EB in acetazolamide group was higher than that in ANP group at 3 h ( P ＜ 0.05 ) ; The mRNA expression of AQPI in ANP group was significantly lower than that in control group.The expression of AQP1 in dexamethasone group was higher than those in ANP group at 6,12 and 18 h,and the expression of AQP1 in acetazolamide group was lower than that in ANP group at 3,6,12 h in intestine tissue ( P ＜ 0.05 ).Protein expression of AQPI in tissues in ANP group was significantly lower than that in control group.The expression of AQP1 in dexamethasone group increased more than that in ANP group at 3,6,12 h,and the expression of AQP1 in acetazolamide group was lower than that in ANP group at 3 h,6 h ( P ＜ 0.05 ).Conclusions The expression of AQP1 is down-regulated in intestine tissue in rats with acute necrotizing pancreatitis,and AQP1 could play an important role in the pathogenesis of capillary endothelial barrier dysfunction.

OBJECTIVETo investigate the application of three-dimensional CT(3D-CT) in the treatment of oblique facial clefts with mandibular outer cortex, including the surgical design and results assessment.

METHODSFrom Jan. 2003 to Dec. 2013, 22 cases with oblique facial cleft, who underwent mandibular outer cortex onlay bone graft were retrospectively studied. 3D images from CT data were reconstructed before operation for design. Then the mandibular outer cortex onlay bone transplant was performed to reconstruct the bone defect and cleft. 3D CT was performed 5-10 days postoperatively and 6- 12 months postoperatively to assess the facial symmetry.

RESULTSAccording to the results of CT measurement, the average volume of the orbital bone defects on the affected side decreased by(64. 6 ± 14. 4)% 5 to 10 days after operation. The average volume of the maxillary and zygomatic bone defects on the affected side decreased by(71.4 ± 15.7)% after surgery. After 6 to 12 months,the average recovery of the mandibular donor site was (57. 9 ± 13. 9)% of the removed mandibular outer cortex. The average absorption of grafted bones was(24.7 ± 25.6 )%. The average height difference between the centre of pupils on both sides before surgery was(3.76 ± 1.27) mm,which decreased to( 1. 15 ± 1.00) mm 5 to 10 days after surgery(P =0. 000) , and( 1.35 ± 1. 13) mm 6 to 12 months after surgery(P = 0. 003). The relapse may be caused by the absorption of the grafted bones.

CONCLUSIONS3D-CT can be used for preoperative design and postoperative assessment in the treatment of oblique facial cleft with mandibular outer cortex.

Bone Transplantation ; Cleft Palate ; surgery ; Craniofacial Dysostosis ; surgery ; Eye Abnormalities ; surgery ; Facial Bones ; abnormalities ; Humans ; Imaging, Three-Dimensional ; Mandible ; transplantation ; Maxillofacial Abnormalities ; surgery ; Retrospective Studies ; Tomography, X-Ray Computed ; methods ; Transplant Donor Site

Forest musk deer (Moschus berezovskii), a rare wild medicinal animal, is listed under the category of the state key protected wildlife list of China. Musk, secreted by the musk glands, is with high economic and medicinal value and used as precious traditional medicine in China. In order to meet the needs of musk in Chinese traditional medicine, forest musk deer farming was conducted in 1950s, but the research progress on musk secretion mechanism was slow. Therefore, by reviewing the histological and anatomical structure of forest musk deer musk gland, the relationship between sex hormones and the musk secretion process, and the molecular mechanism of the musk secretion, the existing problems in investigating the musk secretion mechanism were analyzed and the development trends in this field were also discussed, in order to provide a reference for further studies on the musk secretion mechanism and improve musk production of forest musk deer.
Animals ; Deer ; metabolism ; Exocrine Glands ; anatomy & histology ; chemistry ; secretion ; Fatty Acids, Monounsaturated ; chemistry ; metabolism ; Male

OBJECTIVETo study the effect of Qianyang Recipe (QYR) on the Gan-yang hyperactivity syndrome (GYHS), the blood pressure, and correlated vascular regulatory factors of hypertension rat.

METHODSThirty SD rats were randomly divided into the normal control group, the model group, and the QYR group, ten in each. Hypertension rat model of GYHS was prepared using Aconiti Praeparata Decoction plus ephedrine plus salt water. Rats in the QYR group orally took QYR physic liquor, while distilled water was given to rats in the normal control group and the model group. They were medicated for 28 successive days. The facial temperature, the grip strength, and the systolic pressure were determined once every 7 days. Rats' irritable degree and feather color were observed and recorded once every 14 days. After the last administration the plasma renin (PR), angiotensin II (Ang II), aldosterone (ALD), atrial natriuretic peptide (ANP), calcitonin gene-related peptide (cGRP) were determined.

RESULTSCompared with the model group of the same phase, the facial temperature of rats in the QYR group significantly decreased on the 14th, 21th and 28th day after administration. The systolic pressure obviously decreased on the 21st day after administration. On the 28th day after administration symptoms such as irritability, dry hair were improved, and the Ang II level decreased. There was significant difference in all these changes (P<0.05, P<0.01).

CONCLUSIONSQYR could relieve GYHS rats' symptoms such as facial hotness, irritability, dry hair, and so on, and decrease the systolic pressure. Decreased Ang II level might be one of its mechanisms.

Aldosterone ; blood ; Angiotensin II ; blood ; Animals ; Atrial Natriuretic Factor ; blood ; Calcitonin Gene-Related Peptide ; blood ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Hypertension ; blood ; diagnosis ; Male ; Medicine, Chinese Traditional ; Rats ; Rats, Sprague-Dawley ; Renin ; blood

In order to investigate the anti-proliferative effects of triptolide (TP) on 4T1 mice breast cancer cell line in vitro and in mouse model, as well as the possible mechanisms, we detected the effect of TP on cell proliferation by MTT assay or Crystal Violet Staining in our research. Flowcytometry combined with FITC-Annexin V/PI staining were used for detecting TP induced 4T1 cell apoptosis. The protein expression of ERalpha, p-ERalpha, ERbeta, p-ERbeta, ERK, p-ERK, p38, p-p38, SAPK/JNK, and p-SAPK/JNK was tested by western blotting. We also compare TP with chemotherapy drug doxorubicin in 4T1 tumor bearing BLAB/c mice model, the Xenogen bioluminescence imaging, H&E, and IHC result indicated that TP exhibits an anticancer proliferation activity. As a result, TP in 100, 10, 1, 0.1 micromol x L(-1), all inhibited the proliferation of 4T1 cells by MTT assay and Crystal Violet Staining. TP which concentrations is 10, 1, 0.1 micromol x L(-1) could induce the apoptosis of 4T1 cells and reduce the cell proliferation. TP in 200 microg x kg(-1) could inhibit the tumor growth in vivo. The anticancer proliferation of TP was involved in its effect on reducing expression of ERalpha, p-ERalpha, ERbeta, and p-ERbeta, but nothing to do with the activation of MAPK signaling pathway.
Animals ; Apoptosis ; drug effects ; Cell Line, Tumor ; Cell Proliferation ; Diterpenes ; pharmacology ; therapeutic use ; Down-Regulation ; drug effects ; Epoxy Compounds ; pharmacology ; therapeutic use ; Female ; Lung Neoplasms ; secondary ; Mammary Neoplasms, Experimental ; drug therapy ; metabolism ; pathology ; Mice ; Mice, Inbred BALB C ; Phenanthrenes ; pharmacology ; therapeutic use ; Phosphorylation ; drug effects ; Receptors, Estrogen ; metabolism ; Tumor Burden ; drug effects

Objective To develop a laser positioning device for GE OEC850 and Wandong HMC-36 C-arm.Methods The device was composed of two cross-type laser tubes,timing circuit and power source.The optimal 30 cm projection point was determined with laser dual-crossed-line positioning method and considerations on the distance between the tube of GE OEC850 or Wandong HMC-36 C-arm and the image intensifier.Then the projection range was found around 30 cm to define the targeted area.Results The device eliminated the needs of multi times of projection due to improper positioning,decreased the exposure to medical personnel and patient during the operation,prolonged the service life of the tube and reduced the cost for hospital equipment.Conclusion The device behaves well in positioning and easy operation,and thus is worthy promoting clinically.

Objective To investigate the role and intracellular signal transduction mechanism in the injury of renal tubular cells induced by postasphyxial-serum in neonate.Methods Human renal proximal tubular cell(HK-2 cell) was used as target cell. The experiment was designed as:control group, asphyxia group ,and pyrrolidine dithiocarbamate (PDTC)blocking group. The attacking concentration of serum was 20%, and the apoptosis rate of HK-2 cells was detected by flow cytometer.Results Compared with controls[(13.3?1.70)%],after being stimulated with postasphyxial-serum, the apoptosis rate of HK-2 cells of asphyxia group [(46.73?3.68)%] and PDTC blocking group [(31.19?2.79)%]were significantly increased(P

Objective To investigate the role of postasphyxial-serum of neonate in inducing injury of human renal proximal tubular cells(HK-2 cells).Methods HK-2 cells were used as target cell.The neonatal different concentration postasphyxial-serum of 1,3,7 days after asphyxia were used as attacking means.The experimental groups were divided into 15 groups:the 2.5%,5.0%,10.0%,(20.0%) attacking concertration groups of 1,3,7 day after asphyxia and control group of each concertration.The culture medium and concertration of the control group and the experimental groups were the same.The changes of morphology were observed under inverted microscope,the cell viability was measured by 3-(4,5-dimethyl-2-thiazoly1)-2,5-diphenyl-2H-tetrazolium bromide(MTT) method and the leakage rate of lactate dehydrogenase(LDH) was determined by biochemical methods.Results Compared with control group,the changes in morphology of HK-2 were most serious and obvious,the cell viability were obviously decreased(all P