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.

OBJECTIVETo observe the therapeutic effects of steel plate fixation after the failure of arthrodesis of ankle by screw fixation.

METHODSFrom August 2001 to October 2011, 15 patients were with steel plate fixation after failure of arthrodesis of ankle by screw fixation. Among patients, 9 cases were males and 6 cases were females,ranging age from 40 to 65 years old with the average of 56 years old. Ten cases were in left and 5 cases were in right. Screws were removed and steel plate was fixed intraoperatively, and plaster external fixation for postoperation. Clincal effect were evaluated according to AOFAS scoring system from pain, waliking ability and aligment before and after operation, and X-ray was used to evaluate joint fusion after operation.

RESULTSAll patients were followed up, and the duration ranged from 4 months to 4 years with an average of 2 years. The incison were healed in stage I. No ankle pain,injury of blood vessel and nerve,infection and farilure of internal fixation occuerred. The AOFAS score increased from 36.86 +/- 8.32 preoperatively to 85.09 +/- 4.65 (t = -26.366, P = 0.000).

CONCLUSIONSteel plate fixation after the failure of ankle arthrodesis of screw fixation has the advantages of rigid stability, simple manipulation and high success rate, less pain, perfect recovery.

Adult ; Aged ; Ankle Joint ; diagnostic imaging ; surgery ; Arthrodesis ; Bone Plates ; Bone Screws ; Female ; Follow-Up Studies ; Fracture Fixation, Internal ; instrumentation ; Humans ; Male ; Middle Aged ; Steel ; Tomography, X-Ray Computed ; Treatment Failure

Objective To study the association of the expression of bone morphogenetic protein (BMP) antagonist gremlin and vascular calcification in radial arteries of patients with stage 5 of chronic kidney disease (CKD).Methods Radial arteries of 40 patients with stage 5 of CKD were collected as specimens of the study group,which were trimmed off during arterial venous fistula operations.Splenic trabecular arteries were collected as specimens of the control group,which were removed from 38 patients with simple traumatic splenic rupture.All the arteries were examined histologically for calcification with yon Kossa stain.Expressions of gremlin and BMP-2,-7were detected by immunohistochemistry and their serum concentrations were detected by ELISA.Images of histological sections were semi-quantitatively analyzed by Image-Pro Plus 6.0.SPSS 19.0software was used to perform statistical analysis.Results Significantly positive von Kossa stain was found in radial arteries from 12 of 40 patients (30％) in study group,which located in the layer of medial smooth muscle cells.However,there was no obvious positive stain in control group.Additionally,in study group,significant expressions of gremlin and BMP-2 were detected in those radial arteries of positive yon Kossa stain,which also located in the layer of medial smooth muscle cells.Positive correlations were found among gremlin expression level,BMP-2 expression level and yon Kossa stain intensity.However,the BMP-7 expression intensity in arteries of study group was much weaker as compared to control group.Conclusions Both gremlin and BMP-2 may be involved in the process that the smooth muscle cells of radial arteries in patients with stage 5 of CKD phenotypically transform into osteoblast-like cells.However,BMP-7 possibly prevents this process.

The finite element method (FEM) is a mathematical technique using modern computer technology for stress analysis, and has been gradually used in simulating human body structures in the biomechanical field, especially more widely used in the research of thoracolumbar spine traumatology. This paper reviews the establishment of the thoracolumbar spine FEM, the verification of the FEM, and the thoracolumbar spine FEM research status in different fields, and discusses its prospects and values in forensic thoracolumbar traumatology.
Biomechanical Phenomena ; Computer Simulation ; Finite Element Analysis ; Humans ; Lumbar Vertebrae/injuries* ; Models, Theoretical ; Stress, Mechanical ; Thoracic Vertebrae/injuries* ; Traumatology

Adrenal Gland Neoplasms ; surgery ; Adrenalectomy ; methods ; Adult ; Female ; Humans ; Kidney ; abnormalities ; Laparoscopy ; Multiple Endocrine Neoplasia Type 2a ; surgery ; Pheochromocytoma ; surgery

Objective To evaluate therapeutic effects and toxicity of advanced non-small cell lung cancer (NSCLC)treated by combining chemotherapy on ifosfamide(IFO)and vinorelbine(NVB).Methods 107 cases pa- tients with advanced NSCLC were enrolled.IFO was given in a dosage of 1.5g/m~2 on day 1 to 4.and NVB in a dosage of 25mg/m~2 on day 1 and 8.It was repeated every three or four weeks,up to two to four cycles.Results Two patients had complete response and 40 patients had partial response.The overall response rate was 47.7% ,the median survival time 10.3 months,1-year and 2-year survival rate was 42% and 12.3%,respectively.The main toxicity was bone marrow suppression.Conclusion The regimen is effective,sale and tolerable in advanced non- small cell lung cancer therapy.

OBJECTIVETo observe the three-dimensional distribution of vessels, and to establish a new method for measurement of blood flow velocity in mice cerebral cortex using two-photon laser scanning microscopy and fluorescence probe labeling technique.

METHODSThe mouse was made cranial window surgery and injected Texas-Red through tail vein after anesthetized. The three-dimensional imaging of vessel was obtained through z-stack scanning, and blood flow velocity was quantified through line scanning.

RESULTSWe could detect vascular distribution for more than 500 µm depth using two-photon microscopy. The velocity of blood flow was (0.59 ± 0.12) mm/s in capillary.

CONCLUSIONThe method for observing the brain blood flow by two-photon microscopy was established, which could achieve quantification of single vascular blood flow velocity and provide experimental evidence for basic research and medical applications.

Animals ; Blood Flow Velocity ; Brain ; blood supply ; Capillaries ; Cerebrovascular Circulation ; Fluorescent Dyes ; Hemodynamics ; Mice ; Microscopy, Fluorescence

BACKGROUNDRetinoic acid (RA) is a potent signaling molecule that plays pleiotropic roles in patterning, morphogenesis, and organogenesis during embryonic development. The synthesis from retinol (vitamin A) to retinoic acid requires two sequential oxidative steps. The first step involves the oxidation of retinol to retinal through the action of retinol dehydrogenases. Retinol dehydrogenases1l (RDH1l) is a novel zebrafish retinol dehydrogenase. Herein we investigated the role of zebrafish RDH1l in heart development and cardiac performance in detail.

METHODSRDH1l specific morpholino was used to reduce the function of RDH1l in zebrafish. The gene expressions were observed by using whole mount in situ hybridization. Heart rates were observed and recorded under the microscope from 24 to 72 hours post fertilization (hpf). The cardiac performance was analyzed by measuring ventricular shortening fraction (VSF).

RESULTSThe knock-down of RDH1l led to abnormal neural crest cells migration and reduced numbers of neural crest cells in RDH1l morphant embryos. The reduced numbers of cardiac neural crest cells also can be seen in RDH1l morphant embryos. Furthermore, the morpholino-mediated knock-down of RDH1l resulted in the abnormal heart loop. The left-right determining genes expression pattern was altered in RDH1l morphant embryos. The impaired cardiac performance was observed in RDH1l morphant embryos. Taken together, these data demonstrate that RDH1l is essential for the heart development and cardiac performance in zebrafish.

CONCLUSIONSRDH1l plays a important role in the neural crest cells development, and then ultimately affects the heart loop and cardiac performance. These results show for the first time that an enzyme involved in the retinol to retinaldehyde conversion participate in the heart development and cardiac performance in zebrafish.

Alcohol Oxidoreductases ; genetics ; metabolism ; Animals ; Animals, Genetically Modified ; Heart ; embryology ; Zebrafish ; Zebrafish Proteins ; genetics ; metabolism

OBJECTIVETo develop a headspace gas chromatography method for determining dimethyl sulphate residual in granisetron hydrochloride.

METHODSAn Angilent INNOWAX capillary column with nitrogen gas as carrier and FID as detector was applied in this study. Dimethyl sulphate was tested under a constant column temperature.

RESULTDimethyl sulphate had different retention time from other organic solvents such as alcohol,acetoacetate, isopropanol, dichlormethane and chloroform, which might exist in granisetron hydrochloride. The detection limit of dimethyl sulphate;s was 0.0016%.

CONCLUSIONThe method can be used for the determination of dimethyl sulphate residual in granisetron hydrochloride.

Chromatography, Gas ; methods ; Drug Contamination ; prevention & control ; Granisetron ; analysis ; Pharmaceutical Preparations ; analysis ; Sulfuric Acid Esters ; analysis

Adult ; Bone Neoplasms ; diagnosis ; pathology ; Female ; Humans ; Lipoma ; diagnosis ; pathology ; Talus