Congenital fiber-type disproportion (CFTD) is a rare form of congenital myopathy,characterized by non-progressive generalized muscle weakness from early childhood.Diagnosis depends on crucial histological abnormality that type 1 muscle fibers are consistently smaller than type 2 fibers in the absence of other specific histological abnormalities.Whether CFTD should be regarded as a distinct diagnostic entity has always been a controversial issue.Many pathogenic genes have been identified in recent years.This article reviews clinical manifestation,pathology,genetic diagnosis and treatment progress of CFTD.

Objective To assess the validity of using fasting plasma glucose （FPG） and HbA1c for the screening of diabetes. Methods 1118 subjects （489 men, 629 women） in Beijing area underwent an oral glucose tolerance test （OGTT） for screening diabetes. HbA1c was examined at the same time. They have never undergone OGTT and controlled plasma glucose levels by any methods. Their average age was 48±12 years. Results Using 1999 WHO criteria, 510 had normal glucose tolerance （NGT）, 35 had impaired fasting glucose（IFG）, 155 had impaired glucose tolerance （IGT）, 52 had IGT and IFG, 366 had diabetes. Using a receiver operating characteristic curve （ROC curve）, the optimal cut-point of FPG related to diabetes diagnosed by OGTT was 6.2mmol/L that was associated with a sensitivity and specificity of 85.0% and 90.4% respectively. Area under the curve was 0.943 （95% CI 0.9270.959）, a positive likelihood ratio （LR） was 8.9, and a negative LR was 0.2. The optimal cut-point of HbA1c related to diabetes diagnosed by OGTT was 6.2%, which was associated with a sensitivity and specificity of 86.6% and 77.5% respectively. Area under the curve was 0.896 （95% CI 0.8760.916）, a positive LR was 3.9, and a negative LR was 0.2. The cut-point of FPG related to IGT diagnosed by OGTT was 5.1 mmol/L, which was associated with a sensitivity and specificity of 65.2% and 68.3% respectively. Area under the curve was 0.729 （95% CI 0.6890.769）, a positive LR was 2.1, and a negative LR was 0.5. The cut-point of HbA1c related to IGT diagnosed by OGTT was 5.7%, which was associated with a sensitivity and specificity of 63.3% and 56.5% respectively. Area under the curve was 0.634 （95% CI 0.5910.677）, a positive LR was 1.5, and a negative LR was 0.7. Conclusions When FPG<7.0 mmol/L and ≥6.2 mmol/L or HbA1c≥6.2%, OGTT was necessary to confirm the diagnosis of diabetes. FPG or HbA1c was not reliable to identify IGT.

Anti-angiogenesis therapies have emerged as new treatment strategies for several types of tumors. To date, the most studied programs are to find out effective medicines to block the vascular endothelial growth factor (VEGF) signaling pathway. However, VEGF inhibitors are not effective to all kinds of tumors.Thus there is a need to explore other angiogenesis signaling pathways. Recently, the Dll4-Notch pathway was identified as a new target in tumor angiogenesis. Moreover, an important feature in angiogenesis is the manifold ways in which the VEGF and Dll4-Notch pathways interact each other, which play critical roles in tumor angiogenesis. Therefore, the further exploration of these two pathways will provide more therapies for clinic to treat tumors.

With the treatment of lacunar cerebral infarction with pure motor hemiparesis (PMH) attach importance to the treatment effect, application benefit of Yihong Kangnaoshuan capsule in the treatment of further effect. Treatment of PMH is mainly depending on the thorough discussion on the cause, to determine the clinical value of treatment based on the principle of. Through the research of Zhejiang People's Hospital of Fenghua from 2013 March to 2014 September 178 cases of pure motor hemiparesis were the benefit of Yihong Kangnaoshuan capsule and vedrin capsule treatment, compared the indexes of efficacy after treatment and blood rheology. Finally found the benefits of Yihong Kangnaoshuan capsule in the application process with high safety, efficacy and greater proportion. And in favor of blood lipid and blood rheology indicators of stability, the repair of neurological function is more. Therefore, clinicians should be applied benefit of red brain thrombus capsule in the treatment of PMH. But the overall difference between drugs is need to further comparison, in order to ensure clinical curative effect.
Adult ; Aged ; Capsules ; Cerebral Infarction ; drug therapy ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Male ; Middle Aged ; Paresis ; drug therapy

[ABSTRACT]OBJECTIVETo assess the clinical effects of proton pump inhibitor(PPI) combined with traditional Chinese medicine for laryngopharyngeal reflux disease(LPRD).METHODSA total of 74 LPRD patients with laryngopharyngeal signs and symptoms confirmed by reflux symptom index(RSI) and reflux finding score(RFS) were enrolled. All patients received PPI combined with traditional Chinese medicine therapy. Effect evaluation was conducted at 2, 6, 12, and 18 months after the beginning of therapy.RESULTSThe relief rate was 74.3%, 90.5%, 87.8%, and 86.5% at the 2, 6, 12, and 18 months after the beginning of treatment respectively.CONCLUSIONPPI combined with traditional Chinese medicine therapy is safe and effective for LPRD and worthy of wide application.

Alogliptin is a novel dipeptide peptidase-4 inhibitor approved for the treatment of type 2 diabetic mellitus.Numerous clinical studies showed that alogliptin alone or in combination with other oral antidiabetic drugs or insulin can substantially control the level of plasma glucose and glycated hemoglobin (HbA1c) in type 2 diabetic patients.12.5 to 25 mg alogliptin alone once daily reduced HbA1c by 0.56％ to 0.59％ ; while combination with other antidiabetic agents resulted additional HbA1c lowering of 0.4％ to 0.8％.Alogliptin was well tolerated,with low incidence of hypoglycemia and no weight gain.Furthermore,alogliptin displayed no extra cardiovascular risk in patients with cardiovascular diseases.

Objective To compare dexmedetomidine versus midazolam sedation for myocardial protection in the patients undergoing percutaneous coronary intervention (PCI).Methods Sixty patients with acute myocardial infarction who required for emergency PCI,were selected and randomly divided into 2 groups (n =30 each) using a random number table:dexmedetomidine group (group D) and midazolam group (group M).In group D,a loading dose of dexmedetomidine 1 μg/kg was infused intravenously for 10 min before surgery.In group M,midazolam 0.05 mg/kg was injected intravenously before surgery.Dexmedetomidine 0.2-1.4 μg· kg-1 · h-1 and midazolam 0.02-0.10 mg·kg-1 ·h-1 were given via a pump during surgery until the end of surgery in the two groups.The RASS score was maintained at-2-2 in two groups.Before administration and on 3rd day after surgery,cardiovascular color doppler ultrasound was performed to record left ventricular ejection fraction (LVEF),venous blood samples were collected for determination of the levels of serum myocardial enzyme and cardiac troponin I (cTnI),and the development of surgical complications,duration of ICU stay and length of hospital stay were recorded.Results Compared with group M,the activities of serum creatine kinase,creatine kinase isoenzyme-MB,aspartate amino transferase and lactic dehydrogenase and cTnI concentrations were significantly decreased,LVEF was increased,and duration of ICU stay and length of hospital stay were shortened in group D.There was no significant difference in the development of postoperative complications between the two groups.Conclusion Dexmedetomidine sedation provides better efficacy for myocardial protection and prognosis and is more suitable for sedation than midazolam in the patients undergoing PCI.

As a kind of polyphenolic phytoalexin,resveratrol exerts protective roles in anti-tumor,antiinflammatory and promoting metabolism.Resveratrol's anti-tumor mechanisms may be involved in upregulating tumor suppressor microRNAs and downregulating oncogenic microRNAs,and affect its relative pathways and target genes in different cancers such as colon cancers and breast cancers.This will provide a new theoretical basis for the further clarification of resveratrol-associated tumor suppressing mechanisms and its application to the clinical.

Objective To construct prokaryotic cell expression vector of human Eotaxin mutants.Methods By point mutation,eight amino acid residues in the N-terminal(residues of 3-7)and N-loop(residue 14)regions of Eotaxin were individually mutated to methionine and residue 14 was delleted or methiomine was inserted after the residue 14,and then cloned respectively into prokaryotic cell expression vector-PET30a+.Results Eight N-terminal and N-loop mutants of human Eotaxin and their prokaryotic cell expression vector-PET30a+ were gained.Conclusion The successful construction of prokaryotic cell expression vector of human Eotaxin mutants lays a foundation for their expression and biological activity and for filtering antagonists of CCR3.

Objective To study hepatotoxicity and superoxide dismutase(SOD) acitvity produced by mixture of Nickel Sulfate,Potassium Dichromate and Cobalt Chloride.Mehtods Hepatotoxicity and superoxide dismutase(SOD)activity of mixture of Nickel Sulfate,Potassium Dichromate and Cobalt Chloride were detected by ip in mice.Results The liver weights were increased consistently during three weeds treatment.It was showed in histology study that hepatocytes swelling,necrosis and congestion wigh blood in venae interlobares hepatis.The SOD activity of liver and blood decreased after one week treatment but increased the following two weeks treatment.Conclusions Mixture of Nickel Sulfate,Potassium Dichromate and Cobalt Chloride had obvious hepatotoxicity and a certain lipid peroxiodation effect in vivo.