Congenital adrenal hyperplasia(CAH) is a family of autosomal recessive disorders.The most frequent form of the disease is steroid 21-hydroxylase deficiency,which accounts for more than 90％ of all cases of CAH.In the pathogenesis of CAH,the cortisol synthesis impairement leads to excessive ACTH secretion of the adrenal glands,which ultimately results in adrenal hyperplasia and androgen overproduction.CAH causes signs of androgen excess including ambiguous genitalia in newborn females and rapid postnatal growth in both sexes.In the most severesalt wasting form of CAH,concomitant aldosterone deficiency may lead to salt wasting with life-threatening adrenal crises.Newborn screening,especially in males,reduces morbidity and mortality from adrenal crises.Glucocorticoid replacement remains the cornerstone of treatment.However,how to make a suitable therapeutic regimen of CAH is still a challenge to the pediatric clinician.

Objective:To explore the pretection of small dosage desamethasone to pulmonary function on acute lung injury model.Methods:54 male SD rats were divided randomly into control group,injury group and therapeutic group.Major observed marker consisted of count of cell sum,total protein level and TNF-? level of bronchoalveolar douche solution.Secondary marker consisted of lung coefficient,PAO2,TNF-? level in blood serum and histopathologic examination.Results: Alveolar texture in therapeutic group existed yet,but alveolar interval increased thickness,neutrophilic granulocyte infiltration relieved obviously,and a little bleeding in alveolar cavity.PAO2 in therapeutic group was higher than that of injured group(P

Objective To study the relationship between HLA-DRB1alleles and the systematic lupus erythematosus of the Zhuang Nationality in Guangxi Province.Methods Allele polymerase chain reaction-sequence specific primers(PCR-SSP)were used to study the HLA-DRB1allele genes in52patients with SLE and70healthy controls of the Zhuang Nationality in Guangxi Province.Results The allele frequencies of HLA-DRB1*1401and HLA-DRB1*16were significantly less in SLE patients than those of the controls(RR=0.28,? 2 =5.00,P=0.02and RR=0.39,? 2 =3.95,P=0.05).HLA-DRB1*08,DRB1*11and DRB1*13alleles were not found in these two groups.Conclusion Our data suggest that the HLA-DRB1*1401and HLA-DRB1*16might be protecting genes for SLE in Guangxi Zhuang nationality.No susceptible gene is found in our study.

Objective To investigate the effect of Sufentainil on myocardial ischemia-reperfusion injury and caspase-3 to rat heart. Methods Ninety Wistar rats weighing 200-300g were randomly divided into 3 groups(n=30):sham group(group S), ischemia-reperfusion group (group I/R)and Sufentainil group (group SF). Each group was divided into 3 subgroups according to the time phases at 30, 60 and 120min(n=10).In group I/R and group S the animals were received normal saline intravenously. In group SF sufentainil 5μg/kg NS 20ml was given. After 24 h, all animals were anesthetized with intraperitioneal 20% urethane 1 mg/kg, intubated and mechanically ventilated. The chest was opened and heart exposed via thoracotomy.In group S , left artery anterior descending artery(LAD) was not ocluded. While in I/R and SF group myocardial ischemia was introduced by clamping LAD for40 min. The animals were killed at 30, 60 and 120 min of reperfusion respectively(n=10each). Cardiac tropoin I(cTnI), expression of caspase-3 and apoptosis of cell in heart tissues were measured at the same time.Ultrastructure of myocardium taking the from apex was examined by electron microscopy at the end of 120 min reperfusion. Results Compared with group S, the concentration of cTnI was increased in the other 2 groups(P<0.05).cTnI was significantly lower in group SF than in group I/R(P<0.05). Expression of caspase-3 and apoptosis index induced by myocardial reperfusion were attenuatated in group SF(P<0.05). The damage of myocardial ultrastructure caused by myocardial I/R were also significantly improved in group SF. Conclusion Sufentainil may relieve the heart cellular apoptosis by decreasing caspase-3expressing.

Apoptosis ; Humans ; Periodontitis ; pathology

Objective: To explore the effect of KangXin oral solution on brain mitochondrial DNA(mtDNA) deletion mutation in aged Balb/c mice.Methods: The Balb/c mice were divided into the young group(6weeks),middle-aged group(6months) and aged group(14 months),each group has 10 mice.Brain mtDNA were obtained and polymerase chain reaction(PCR) technique was used to examine the fragment deletion of brain mtDNA,thus,to confi rm there was deletion of mtDNA in aged mice,s brain.The aged Balb/c mice were divided into two groups: the aged blank control group being given 0.9% normal sodium,KangXin oral solution group.After being treated for four months,the brain mtDNA were obtained and polymerase chain reaction(PCR) technique was used to amplify wild-type and deletion from of mtDNA.Gel imaging meter was used to detect optical density,then,to compare the optical density ratios of deletion from mtDNA/ wide type mtDNA in two groups.Results: There were 304bp mtDNA deletion in brain mitochondrial of aged Balb/c mice,but same mtDNA deletions were not detected in brain mitochondrial of young and middle-aged mice.Compared with aged blank control group,the mtDNA deletion of aged Balb/c mice in KangXin oral solution group decreased obviously(P〈0.001).Conclusion: mtDNA deletion mutation accumulates with the increase of age.KangXin oral solution can inhibit mtDNA deletion of aged mice.

Adult ; Humans ; Male ; Middle Aged ; Phosphines ; poisoning ; Young Adult

Adult ; Antithyroid Agents ; therapeutic use ; Autoantibodies ; blood ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Glycosides ; therapeutic use ; Graves Disease ; drug therapy ; immunology ; Humans ; Immunosuppressive Agents ; therapeutic use ; Male ; Methimazole ; therapeutic use ; Middle Aged ; Phytotherapy ; Tripterygium ; chemistry

Adolescent ; Adult ; Child ; Child, Preschool ; Heart Defects, Congenital ; surgery ; Humans ; Young Adult

Capsid Proteins ; metabolism ; Endocytosis ; physiology ; Virus Internalization ; Viruses ; metabolism