Objective To search for a way of treating chi1dren's idtopathic nephrotic syndrome (INS) hypoadrenocorticismand its significance. Meethods Thirty-nine patients (31 males and 8 females) with INS were randomly divided into twogroups: 21 patients in tera-peutic group (treated by prednisone, astragali and acanthopanax root) and 18patients in control group (Simply treated by prednisone). 1ml blood was respectively drawn from all thepatients at 8 o'clock (one time before treatment and one time after treatment), the serum cortisol wasdetermined by radioimmunoassay (RIA). Results In control group, the levels of serum cortisol still remained lower than the normal value aftertreatment and the levels after large dosages prednisone treatment were markedly lower than that beforetrea-tment. The values of serum cortisol returned to be normal after treatment in therapeutic group. Conclusion Astragali and acanthopanax root can restore the hypoadrenocorticism in children with INS.

Objective To explore the correlation of β2-adrenergic receptor (β2-AR)polymorphisms (Arg16Gly) with the prognosis of myasthenia gravis (MG) complicated with other autoimmune diseases.Methods Among the 75 MG patients in analysis,17 cases were complicated with other autoimmune diseases (AIDMG),58 cases without other autoimmune diseases (NAIDMG).MG patients,AIDMG patients,NAIDMG patients were separately divided into recurrence groups and nonrecurrence groups according to the progression at 2 years after onset.The genotypes of β2-AR in 75 MG patients were determined by gene sequecing.Results The frequencies of three genotypes (Arg/Arg,Arg/Gly and Gly/Gly) in position 16 were 30.8％,50.0％,19.2％ in recurrence MG group and 42.9％,38.8％,18.3％ in non-recurrence MG group respectively.The difference in distribution of the genotypes between recurrence MG group and non-recurrence MG group was not statistically significant (x2 =1.150,P=0.563).The frequencies of Arg and Gly allele were 55.8％ and 44.2％ in recurrence MG group,and 62.2％ and 37.8％ in non-recurrence MG group.The difference in distribution of the alleles between the two groups was not statistically significant.The frequencies of 3 genotypes in position 16 were 27.3％,63.6％ and 9.1％ in recurrence AIDMG group and 100.0％,0,0 in non-recurrence AIDMG group,respectively.The frequencies of Arg and Gly allele were 59.1％,40.9％ in recurrence AIDMG group,and 100.0％,0 in non-recurrence AIDMG group.The difference in distribution of the genotypes between recurrence AIDMG group and non-recurrence AIDMG group was statistically significant (P =0.009).There also was significant difference in distribution of alleles between recurrence and non-recurrence AIDMG groups (x2 =6.676,P =0.010).The frequencies of 3 genotypes in position 16 were 33.3％,40.0％ and 26.7％in recurrence NAIDMG group and 34.9％,44.2％,20.9％ in non-recurrence NAIDMG group,respectively.The frequencies of Arg and Gly allele were 53.3％,46.7％ in recurrence NAIDMG group,and 57.0％,43.0％ in non-recurrence NAIDMG group.There was no significant difference in distribution of genotypes or alleles between recurrence and non-recurrence NAIDMG groups.Conclusion β2-AR gene polymorphism in position 16 may predict the prognosis of AIDMG,and there is no correlation between the polymorphism in position 16 of β2-AR and the prognosis of MG and NAIDMG.

Objective To investigate the association of glucocorticoid receptor (GR) polymorphisms (BclI)with the prognosis of myasthenia gravis (MG).Methods We totally enrolled 74 patients diagnosed as MG from the Department of Neurology,Beijing Shijitan Hospital between 2002 and 2014.Of them,54 patients started with ocular MG and 20 patients started with general MG.MG patients were divided into recurrence group and non-recurrence group according to the progression at two years after onset.Patients with simple ocular symptom at disease onset were further divided into generalized MG (GMG) group and single ocular MG (OMG) group according to disease progression or not.The GMG group was divided into two groups (≤6 months,7-24 months) according to the progression time of generalization.The GMG group was further divided into three groups (limbs,throat,both limbs and throat) according to the first symptom of generalization.The genotypes of GR were determined by polymerase chain reaction and nucleotide sequence determination.Results The frequencies of three genotypes (GG,CG,CC) in BclI were 57.7％,34.6％,7.7％ in recurrence MG and 64.6％,31.3％,4.1％ in non-recurrence MG respectively.The difference in distribution of the genotypes between the two groups was not statistically significant (x2 =0.570,P =0.750).The frequencies of G and C allele were 75.0％ and 25.0％ in recurrence MG,and 80.2％ and 19.8％ in non-recurrence MG.The difference in distribution of the alleles between the two groups was not statistically significant (x2 =0.540,P =0.462).The frequencies of three genotypes GG,GC and CC were 55.9％,35.3％,8.8％ in GMG and 2/6,4/6,0/6 in OMG respectively.The frequencies of G and C allele were 73.5％ and 26.5 ％ in GMG,and 8/12,4/12 in OMG.The difference in distribution of the genotypes and alleles between the two groups was not statistically significant (x2 =2.278,P =0.320;x2 =0.241,P =0.624).The frequencies of three genotypes GG,GC,CC were respectively 61.9％,28.6％,9.5％ and 3/6,3/6,0/6 in ≤6 months,7-24 months of GMG group.The frequencies of G and C allele were 76.2％,23.8％ and 9/12,3/12 in the two groups.The difference in distribution of the genotypes and alleles between two of the three groups was not statistically significant (x2 =1.326,P =0.515;x2 =0.007,P =0.932).The frequencies of three genotypes GG,GC and CC were respectively 2/8,4/8,2/8;11/13,2/13,0/13 and 3/6,3/6,0/6 in limbs,throat,both limbs and throat of GMG group.The frequencies of G and C alleles were 8/16,8/16;92.3％,7.7％ and 9/12,3/12 in the three groups.The difference in distribution of the genotypes and alleles between two of the three groups was statistically significant (x2 =8.813,P =0.028;x2 =9.706,P =0.008).The genotype frequencies in every group were all in Hardy-Weinberg equilibrium.Conclusions BclI polymorphism may predict the first generalized symptom of OMG.BclI polymorphisms of GR might have no relationship with the recurrence of MG,generalization and generalized time of OMG during the first two years after MG onset.

Objective To explore the correlation of β2-adrenergic receptor (β2-AR) polymorphisms (Arg16Gly) with early onset Myasthenia Gravis (MG). Methods Forty-eight with age less than 40 years at disease onset were divided into three groups:normal thymus (13 cases), thymic hyperplasia (22 cases) and thymoma (7 cases) groups according to the thymus histology. These patients were further divided into different subgroups including female (31 cases) and male groups (17 cases) based on the gender, OMG (29 cases) and GMG (19 cases) groups according to the symptom of disease onset and groups associated with (10 cases) or without (33cases) other autoimmune diseases Or with unknown causes (5 cases). The genotypes ofβ2-AR in 48 early onset MG were determined by gene sequencing. Results Arg/Arg was more common in early MG patient with normal thymus ( 53.8%)and thymic hyperplasia(54.6%)whereas Arg/Gly was more common in thymus group(71.4%). The difference in distribution of the genotypes between the three groups was not statis?tically significant (χ2=5.657,P=0.226). Arg/Arg was more common in early female MG patient (58.1%) and Arg/Gly was more common in male MG patients (58.8%). The difference in distribution of the genotypes between the two groups was
statistically significant (χ2=6.064,P=0.048). Arg/Arg was more common in early OMG patient (48.3%). Arg/Arg(42.1%) and Arg/Gly(47.4%) were equal common in GMG patients. The difference in distribution of the genotypes between the two groups was statistically significant ( χ2=1.623,P=0.444). Arg/Arg was more common in early MG patient associated with other autoimmune diseases (80.0%). Arg/Gly was more common in MG patients without other autoimmune diseases (39.4%). The difference in distribution of the genotypes between the three groups was statistically significant (χ2=6.394, P=0.041). Conclusionβ2-AR gene polymorphism in position 16 is associated with gender and other autoimmune diseas?es in patients with early onset of MG.

Objective To study the liver damage induced by platelet activating factor (PAF) and the protective effects of the PAF antagonist WEB2170. Methods Experiments in vivo: after the administration of PAF to induce liver damage in rats, serum levels of aspartate transaminase (AST), alanine transaminase (ALT), lactic dehydrogenase (LDH) and tissue malonyldialdehyde (MDA) were determined by biochemical method, the succinate dehydrogenase (SDH) activity in mitochondrion was assessed by histochemical method, and the morphologic changes in hepatic structure were observed. In the treatment group, WEB2170 was used for intervention. Experiments in vitro: the fluidity of cellular velum of liver cells was detected with DPH probe, and the effect of TNF and MDA produced by Kupffer cells cultured by PAF stimulation was observed. Results PAF increased the expression of serum AST, ALT, LDH and tissue MDA (P

Objective To discuss the application of Germany Cooperation for Transparency and Quality in Health Care (KTQ) quality certification system in the field of nursing management. Methods The Plan-Do-Check-Act (PDCA) from KTQ was used in the management of ward nursing quality. The post-interventional quality of basic nursing, critical nursing, health education, ward management, nursing safety and disinfection and isolation were compared and contrasted with the pre-interventional one. Result The quality of basic nursing, critical nursing, health education, ward management, nursing safety and disinfection and isolation were significantly improved after intervention with PDCA from KTQ (P<0.01). Conclusion The KTQ quality certification system can be applied to improve the nursing quality in the process of nursing management.

Objective To use bioinformatics methods to analyze large amounts of data generated by gene chips and to screen common key genes in hepatocellular carcinoma in human and rat.Methods For search of the medical literature,3 sets of gene chip with data which met our predetermined criteria were downloaded from the GEO database.The data were standardized by using the bioconductor and R version of the 2.10.1 version.The original data of the affymetrix platform were normalized with background correction,standardized and transformed into log2 by using the algorithm of the affy packages RMA.The TTEST function of the excel was then used to calculate the significance of each gene.The DAVID was used for gene ID conversion and a table was established for samples and the corresponding gene expression data.A meta analysis was performed to calculate the common genes of human and rat.An enrichment regulation pathway was gained with the KEGG in the DAVID library. Results There were 26 common expression genes in the development process of hepatocellular carcinoma in human and rat.Five of these genes were up-regulation genes,while twenty-one were down-regulation genes.An enrichment pathway,which is a focal adhesion pathway,was found and this pathway has been reported to be associated with development of hepatocellular carcinoma.Conclusion With bioinformatics,we were able to screen common key genes and a pathway which were closely related to development of hepatocellular carcinoma in human and rat.

ObjectiveTo report clinical, pathological and molecular genetic features in a Chinese family with hereditary motor and sensory neuropathy type 6. MethodsThe index case is a 15 years old boy.He developed progressive distal limb weakness at the age of 5.The disease deteriorated slowly,accompanied with contracture of achilles' tendon. At the age of 11 years old he suffered from decrease of visual acuity. At the age of 12, he found the muscular atrophy of both hands without sensory disturbances.Visual evoked potential revealed prolonged latency of bilateral P100. Ophthalmological examination showed bilateral optic atrophy. His mother had the similar symptoms at the age of 7 and reduced visual acuity at the age of 10. Nerve conduction velocity revealed in both pat1ents no compound motor and sensory nerve action potentials in most nerves or slightly reduced nerve conduction velocities with severely reduced amplitudes of the compound motor and sensory nerve action potentials. Sural nerve biopsy was performed on the proband.The sequence of MFN2 gene was analyzed in DNA from the index, his mother and 100 healthy controls.ResultsSural nerve biopsy revealed severe loss of myelinated fibers with few regenerating clusters.Ultrapathological examination showed a few of atypical bulbs of myelinated fibers, occasionally regenerating clusters, mitochondrial swelling and aggregation in a few of axons. A new mutation of W740R mutation in MFN2 gene has been identified in the index case, her mother, but not in 50 healthy controls. Conclusions A novel MFN2 gene mutation result in hereditary motor and sensory neuropathy type 6.Mild visual loss appeares after the lesion of spinal nerves. Demyelination of peripheral nerve appears in the disease.

Objective To study the characteristics of child acute lymphocytic leukemia (ALL) immuno-phenotype and evaluate its diagnosis value. Methods Direct immunofluorescence staining and CD45/Side Scatter (SSC) gating of flow cytometry were used for immunophenotyping in 111 cases of child ALL. The relation of mor-phology and immunology classification was analyzed. Results Three categories could be identified,including 81 ca-ses (73.0%) of B lineage ALL, 16 cases (14.4%) of T lineage ALL and 14 cases (12.6% ) of B/T lineage ALL. There were 25 cases (22.5% ) of ALL expressing myeloid-associated antigens. According to the FAB Morphology classification,59 cases (53.2%) of L1 type and 47 cases (42.3%) of L2 type were diagnosed. The two cases (1.8%) of L3 type were classified as one case of null-ALL and one case of B-ALL. One case (0.9%)of acute my-eloblastic leukemia (AML-M2a) was identified as null-ALL. The two cases that could not be diagnosed by FAB clas-sification were c-ALL. Conclusion The immunophenotyping helps to identify the character of leukemia with an im-portant value in diagnosis of child ALL.

Objective To study the curative effects and adverse effects of the thalidomide combined with COMP chemotherapy in treating multiple myeloma(MM).Methods 42 patients were initially diagnosed as MM and 27 patients were refractory and relapsed MM.The small dose of thalidomide combined with COMP management and COMP management alone were used.The effective rate and adverse effects were analyzed.Changes of M-protein in serum,percentage of plasma cells in bone marrow and the level of hemoglobin were also analyzed in both pre-treatment and post-treatment periods.Results In 42 patients who were initially diagnosed as MM,the effective rate was 40.9% for 22 patients treated by chemotherapy alone and 70.0% for 20 patients treated by the thalidomide combined with chemotherapy.Statistic difference was observed between those two group.As to the 27 patients who were refractory and relapsed MM,the effective rate was 42.9% for 13 patients treated by chemotherapy alone and 84.6% for 14 patients treated by the thalidomide combined with chemotherapy.Statistic significance was present between them.Adverse effects were less and tolerated.Conclusion Treatment of small dose of thalidomide combined with COMP chemotherapy could significantly improve the effective rate with less adverse effects.