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 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.

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.

Apoptosis ; Humans ; Periodontitis ; pathology

Objective To observe the ultrastructure of dimorphic Penicillium marneffeiisolates from wild bamboo rats (Rhizomys pruinosus) in Guangxi region as well as from a patient with penieilliosis marneffei,and to compare their biological characteristics and anti-oxidative mechanisms.Methods Two Penicillium marneffei strains,including one isolated from wild bamboo rats (Rhizomys pruinosus) in Guangxi region and one from a patient with penicilliosis marneffei,were cultured with or without the presence of 2.0 mmol/L hydrogen peroxide in potato dextrose agar (PDA) at 25 ℃ and in brain-heart infusion (BHI) broth at 37℃ for seven days.The shape of colony and growth of both strains were observed.Light microscopy was carried out to study the morphology,and transmission electron microscopy to observe the ultrastructure,of both isolates.Results Ater incubation with hydrogen peroxide,there was a slowdown in the growth of both Penicillium marneffei isolates at both mycelial phase and yeast phase,with an increase in the production of pigment at mycelial phase at 25℃.No obvious changes were observed at 37 ℃ in the morphology of either the clinical isolate or the bamboo rat isolate when cultured with hydrogen peroxide compared with those cultured without hydrogen peroxide.Light microscopy showed attenuated spore formation by the clinical isolate when cultured at 25 ℃ with hydrogen peroxide,crenation of both isolates when cultured at 37 ℃ with hydrogen peroxide.Under a transmission electron microscope,the mycelial cells of both isolates exhibited smooth cell walls,intact cell membranes,with nuclei,mitochondria,endoplasmic reticulum,lipid body,vacuoles of various sizes in the cytoplasm at 25 ℃,and even microbodies at 37 ℃,when cultured without the presence of hydrogen peroxide.After incubation with hydrogen peroxide,the cell wall of both isolates became incomplete with defects in some areas and uneven thickness,the cell membrane discontinuous with shrinkages and projections,and the cytoplasm was inhomogeneous with obvious phagocytosis and numerous phagocytic vacuoles.Conclusions The clinical and bamboo rat isolates of Penicillium marneffei experience different biological and morphological changes under oxidative stress,hinting differences in antioxidative mechanism between them.

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

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

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

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