Application of ICF-CY in Family Rehabilitation of Children with Duchenne Muscular Dystrophy / 中国康复理论与实践

Objective To explore the feasibility of assessments of functional and environmental factors in children with Duchenne mus-cular dystrophy (DMD) under the frame of International Classification of Functioning, Disability and Health-Children and Youth Version (ICF-CY). To execute family intervention and to evaluate its effects. Methods A 6-year-and-5-month-old boy with DMD was enrolled to the study. Functional and environmental factors were assessed with the North Star Ambulatory Assessment (NSAA), time tests, hand-held dyna-mometry assessment, body mass index and family interview. Plans of family intervention were settled and executed for one year and at the end of intervention, the boy received all the above assessments to compare the effects of intervention. Results After one-year family inter-vention, the muscle strength was improved or maintained in most muscles except abductors of hip and the body mass index did not change. For the activities, the scores of NSAA increased and maintained, and the result of time tests improved. Otherwise, attitude and execution of parents were improved. Conclusion It is feasible to execute family intervention under the frame of ICF-CY in children with DMD. Both children and their family may benefit from the intervention.

Quaternary Ammonium Compounds Resistant Gene of Multiple Drug Resistant Acinetobacter baumannii / 中华医院感染学杂志

OBJECTIVE To investigae the gene resistant to quaternary ammonium compounds of multiple drug resistant Acinetobacter baumannii(MDR-ABA).METHODS The quaternary ammonium compounds qacE?1 gene in 20 MDR-ABA strains were detected by PCR.RESULTS The 20 MDR-ABA strains showed multiple resistance,its sensitivity to imipenm was 65% only.Among twenty strains of MDR-ABA qacE?1 gene were all positive.CONCLUSIONS The results indicate that qacE?1 gene were all carred class 1 integron.The chlorhexidine usage in prevnting hospital infection after surgery should be reevaluated.

Expression of dystrophin associated glycoprotein complex in Duchenne muscular dystrophy at different age groups / 临床儿科杂志

Objective To analyze the expression of dystrophin associated glycoprotein complex (DGC) in Duchnne muscular dystrophy (DMD) in different age groups. Methods The confirmed 24 DMD patients were divided into 3 groups according to their chronological age (children whose age between 1 to 3 were assigned to early childhood group; children whose age between 3 to 6 were assigned to preschool group; children whose age between 6 to 13 were assigned to school-age group). Several proteins from sarcolemmal muscle membrane were analyzed by immunohistocbemistry. Results The primary loss of dystrophin may lead to a secondary or completely deficiency of α-SG, β-SG, γ-SG,δ-SG, β-DG and dysferlin from sarcolemmal muscle membrane in different age groups. The expression of nNOS is completely deficient in the 3 groups, while the expression of caveolin-3 increased. Conclusions The expression of dystrophin related DGC proteins from DMD patients in different age groups showed diversed degrees of deficiency or complete deficiency. The deficiency of these proteins may occur during the embryonic period, while the increased expression of eaveolin-3 may have a relation with the regeneration of skeletal muscle fibers.

Anti-osteoporotic effect of raloxifene on mice with osteoprotegerin gene deficiency / 中华内分泌代谢杂志

Objective To observe the effect of raloxifene, a selective estrogen receptor modulator, on osteoporosis in the osteoprotegerin (OPG) gene knock-out female and male mice. Methods Two groups of OPG gene deficient (OPG-/-) female and male mice, 20 mice in each group, were assigned to raloxifene-treated (3 mg The effect of raloxifene was evaluated by comparing the values of bone mineral density (BMD) , bone strength,histomorphometric measurement and osteoclast number between the raloxifene treated group and placebo group.Results As compared with placebo group osteoporotic manifestations were improved in OPG-/- female mice treated with raloxifene orally. BMD was increased both in lumbar vertebrae (P<0.05) and femurs (P<0.01).Bone strength was measured in femurs by three-point bending test and vertebrae by stress test. Results showed that ultimate load, ultimate stress and Young's modulus were increased both at lumbar and femur bone, suggesting decreased risk of fracture. Tartrate-resistant acid phosphatase, a marker enzyme of osteoclasts, was detected, and the number of osteoclasts declined significantly after the treatment of raloxifene. At the same time, results of histomorphometric measurements indicated that bone trabecular volume was increased and bone formation rate decreased from(8.05±4.02)mm3·mm-2·year-1 to (5.48±1.89)mm3·mm-2· year-1(P＜0.05).These findings were found in the group of OPG-/- female mice treated with reloxifene but not in male mice. Conclusions Raloxifene is effective in treating osteoporosis in female OPG-/- mice, indicating that its action is at least in part independent of OPG gene. But it is ineffective in male OPG-/- mice.

High-bone-turnover Osteoporosis and Aortic Calcification in Opg Knockout Mice / 生物化学与生物物理进展

Bone turnover is regulated by local concentrations of cytokines such as osteoprotegerin (OPG) and receptor activator of nuclear factor kappaB ligand (RANKL). To explore the in vivo biological function of Opg and the mechanism of osteoporosis due to deficiency of Opg, Opg knockout mice have been generated through homologous recombination. Opg-/- mice exhibit a sharply decrease in bone density and strength as expected. The number of osteoclasts in Opg-/- mice significantly increases. Morphologically, osteoclasts appear more cuboidal in shape in Opg-/- mice than those of wt mice, suggesting that active osteoclastogenesis occurs in the absence of Opg. In consistent with this finding, an increase of osteoblast activity was also observed with accelerated mineral accumulation rate by histomorphometric measurement and elevated serum alkaline phosphatase activity (ALP) in Opg-/- mice. Interestingly, more than 50% of 2-month-old Opg-/- mice manifest medial calcification of aorta with comparable serum concentrations of calcium and phosphorus to wt mice. In conclusion, Opg-/- mice have a high-bone-rurnover type osteoporosis. The aortic calcification in Opg-/- mice is not due to abnormality of calcium and phosphorus metabolism. The mechanism underlying aortic calcification in Opg-/- mice needs to be further investigated.

Aminoglycoside Modifying Enzymes Genes of Multi-drug Resistant Acinetobacter baumannii:A Study of 20 Strains / 中华医院感染学杂志

OBJECTIVE To investigate the aminoglycoside modifying enzymes genes of multi-drug resistant Acinetobacter baumannii(MDR-ABA).METHODS The four kinds aminoglycoside modifying enzymes genes(aac(6′)-Ⅰad,aac(6′)-Ⅰb,aac(3)-Ⅰ,ant(3″)-Ⅰ) of 20 strains MDR-ABA were detected by PCR.RESULTS Among 20 MDR-ABA strains,12 strains of aac(3)-Ⅰ,15 strains of aac(6′)-Ⅰb,18 strains of ant(3″)-Ⅰwere positive,and aac(6′)-Ⅰad was negative.The new subtype aac(6′)-Ⅰad gene has not been found.CONCLUSIONS Twenty strains MDR-ABA aminoglycoside modifying enzymes genes are found in all the 20 MDR-ABA strains.The genotype is in accordance with antibiotics resistance.It can induce clone transmitting hospital infection.It is first time to study the aac(6′)-Ⅰad gene of A.baumannii in China.

?-Lactamases Gene of Multi-drug Resistant Acinetobacter baumannii:An Analysis of 20 Strains / 中华医院感染学杂志

OBJECTIVE To investigate the ?-lactamases gene of multi-drug resistant Acinetobactor baumannii(MDR-ABA).METHODS The blaTEM,blaSHV,blaPER,blaGES,blaIMP,blaVIM,blaSIM,blaOXA-23,blaADC and blaDHA genes of 20 MDR-ABA strains were detected by PCR.RESULTS Among 20 MDR-ABA strains,18 strains were with positive blaADC,11 strains were with positive blaTEM;the others were negative.Totally 19 strains were with genes correlated to ?-lactam antibiotics.The results indicated that the blaADC DNA sequence was a new subtype type compared with the blaADC sequence which had registered on America GenBank.CONCLUSIONS blaADC Gene and blaTEM gene have spread in the MDR-ABA group and a new blaADC subtype has been found.

Generation of Adiponectin Gene Knock-out and LacZ Gene Knock-in Mouse Model / 生物化学与生物物理进展

Adiponectin is an adipocyte-derived secretory protein. It was found to be associated with insulin resistance, inflammation and arteriosclerosis. To further study the biological function and expression of adiponection in vivo, adipoenctin gene knock-out and LacZ gene knock-in mouse model was constructed. Gene targeting strategy was designed to replace part of exon 2 and exon 3 of adiponectin gene with full length LacZ gene in frame with remaining upstream ATG and signal peptide sequence of exon 2. The targeting vector (Adipo-LacZ-XpPNT) was constructed and verified by restriction enzyme digestion and sequencing. CJ7 ES cells were transfected with targeting vector linearized by NotⅠ digestion, selected in the medium containing both G418 and ganciclovoir. Resistant clones were screened by PCR and further confirmed by Southern blot for correct homologous recombinants. Chimera mice were obtained by routing microinjection of homologous recombined ES cells into blastocysts. After mating, mice heterozygous and further homozygous for adiponectin knockout and LacZ gene knock-in were established. Expression of both endogenous adiponectin and exogenous LacZ gene in mouse tissues and sera were detected by RT-PCR, Northern-blot, Western blot and ELISA. The results show that adiponectin was disrupted at both mRNA and protein levels. LacZ gene is expressed exclusively in adipose tissue of mutant mice. Its expression profile is identical to endogenous adiponection. Unexpectedly, LacZ activity could not be detected in both adipose tissue and serum although LacZ protein can be detected in adipose tissue but not in serum of mutant mice. In conclusion, mice homozygous for adiponectin knockout and LacZ gene knock-in have been successfully constructed. Mutant mice display LacZ expression profile identical to endogenous adiponectin albeit neither LacZ activity nor protein can be detected in serum of mutant mice.

New Drug-resistant Characteristics of Acinetobacter baumannii and Its Resistance to Imipenem / 中华医院感染学杂志

OBJECTIVE To test and analyze the new drug-resistant characteristics of Acinetobacter baumannii.METHODS The bacteria identification was determined by the routine method.Antimicrobial susceptibility was detected by K-B test.RESULTS In 95 A.baumannii strains,in addition to imipenem,the drug-resistant incidence was above 49%.There were two A.baumannii strains resistant to imipenem,and were seen panresistant strains The new characteristics of drug-resistance were found.CONCLUSIONS A new drug-resistant mode about A.baumannii to amikacin is found.The high drug-resistant incidence of A.baumannii becomes a very important problem.