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

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

Bone Neoplasms ; Female ; Humans ; Middle Aged ; Waldenstrom Macroglobulinemia

Objective To study the clinicopathological characteristics of complicated Kaposi's sarcoma after renal transplantation.Methods Five cases of complicated Kaposi's sarcoma after kidney transplantation were studied by histopathological immunohistochemical and ultrastructural observations.Results Of the 5 Kaposi's sarcoma patients,2 had multiple amaranthine dermal plaques starting on the legs,one had lymph node hypertrophy all over the body,and others had inguinal lymph node and adenoid hypertrophy. Histopathologically,these angiomatous lesions had characteristic areas of spindle cells within which erythrocytes were enmeshed.Admixed in these lesions were hyaline bodies and hemosider-laden,phagocytic cells.Immunohistochemical and ultrastructural studies revealed that this tumor was derived from vasofomative mesenchyme with multipotential capabilities.It was found that the epithelia and spindle cells were positive for Vimentin,CD-31,CD-34 and Ⅷ-RAg.Conclusion Complicated Kaposi's sarcoma after kidney transplantation can be diagnosed by combination of clinical manifestations and histopathological examinations with immunohistochemical and ultrastructural observations.

Insulin-mediated glucose metabolism of peripheral tissue and liver was assessed by euglycemic hyperinsulinemia clamp technique in lipid-infused rats. There was a significant increase in plasma free fatty acids and a significant reduction in glucose infusion rates in the lipid-infused group. The suppressive effect of insulin on hepatic glucose production (HGP) was significantly blunted and the rate of glucose disappearance showed a slight decrease in the lipid-infused rats, suggesting that lipid impaired the abilities of insulin to suppress lipolysis, HGP and insulin-mediated glucose utilization in peripheral tissues.

Objective:Human capillary lymphatic endothelial cells (LECs) were isolated and cultured to assist further investigation of the function of lymphatic vessels generation during cancer metastasis. Methods:Human skin was digested by type I collagenase. Cells were isolated using magnetic beads which were marked by monoclonal antibodies against the extracellular domain of VEGFR-3, and purified by cloning col-umn. The morphology and structure of cells were observed by microscopy. Immunophenotype was identified by immunofluorescence. Cell growth curve was recorded to measure the effect of VEGF-C protein. Results:LECs exhibited the typical cobblestone morphology as monolayer growth pattem under microscopy. Enlarged nucleus and rich cytoplasm with bubbles were found under electromicroscopy. LECs specific markers inclu-ding VEGFR-3, LYVE-1, Podoplaninand D2-40 all were positive. VIII factor as specific marker of blood ves-sel endothelium cells (BVECs) was negative. VEGF-C induced a marked increase of cell proliferation. Con-clusions:Human dermal LECs could be harvested successfully using collagenase digestion, immunomagnet-ic beads sorting and clonic column purification.