The social capital and organizational performance of civil society organizations in the field of HIV/AIDS prevention and control / 中国卫生政策研究

To analyze the influence of external social capital on organizational performance of civil society organ-izations in HIV/AIDS prevention and control fields. Methods:Multi-variate stratified sampling and cluster sampling methods were used to investigate 212 civil society organizations in the field of AIDS prevention and control in eight prov-inces of China. The data were analyzed with the help of the multivariate logistic regression. Results:The findings showed that the shared vision,network will affect organizational institution performance(OR=3.23,95% CI,1.77-5.88),(OR=2.21,95% CI:1.21-4.02); Shared vision factors will affect the organization's performance of the organization's external social capital (OR=4.17,95% CI:2.23-7.78);Shared vision is closely relative to Shared vision and the organization's financial performance (OR=2.97,95% CI:1.60-5.51);Shared vision, network and support has positive correlation with organizational culture performance(OR=2.04,95% CI:1.09-3.85),(OR=2.02 ,95% CI:1.09 -3.76), (OR =3.34, 95% CI: 1.79 -6.23); Shared vision and the network will affect the organization's comprehensive performance (OR=14.56,95% CI:6.83-31.06), (OR=2.23,95% CI:1.11-4. 48). Conclusions:The external social capital of civil society organizations in the field of HIV/AIDS prevention and con-trol can affect organizational performance,and different organizations and the governments should develop the external social capital vigorously to improve organizational performance.

Clinical characteristics and WT1 genetic analysis of patients with steroid-resistant nephrotic syndrome accompanied with genitourinary malformations / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the clinical features and WT1 gene mutations in patients with steroid-resistant nephrotic syndrome (SRNS) accompanied with genitourinary malformations. The expression of podocyte molecules was also investigated in renal specimen of these WT1 mutated patients.</p><p><b>METHODS</b>From January 2005 to May 2007, 3 cases of SRNS accompanied with genitourinary malformations were involved in this study. The expression of podocyte molecules (nephrin, podocin, alpha-actinin 4, WT1 and CD2AP) in 2 cases was analyzed by immunofluorescence and immunohistochemistry; using PCR to amplify genomic DNA and RT-PCR to amplify WT1 cDNA. GeneScan and GeneScan software were used to quantify the ratio of +KTS/-KTS isoforms.</p><p><b>RESULTS</b>The age of onset of the 3 cases were 6 months, 1 year and 10 years, respectively. The age at diagnosis was 7 months, 9 years and 15 years, respectively. The phenotype of case 1 and case 3 was male accompanied with genitourinary malformations. Case 2 was phenotypic female. Karyotype analysis of the 3 cases revealed 46, XY. Each case was diagnosed as SRNS. Focal segmental glomerulosclerosis (FSGS) was confirmed in 2 cases. Podocyte molecular expression altered in renal tissues of 2 cases. WT1 staining was negative in case 1. WT1 expression in case 2 showed a diffuse nuclear staining with less obvious speckles compared with controls. WT1 IVS 9 + 5 G > A mutation was detected in case 2 and WT1 exon 9 1186 G > A mutation was detected in case 3. No WT1 mutation was detected in case 1.</p><p><b>CONCLUSIONS</b>Karyotype analysis and WT1 genetic analyzing should be performed for all female patients with early onset SRNS and in male patients with SRNS accompanied with genitourinary malformations. The abnormal ratio of +KTS/-KTS isoforms caused by WT1 mutations along with abnormal expression of podocyte molecules were involved in the pathogenesis of proteinuria.</p>

Prenatal diagnosis and genetic counseling of X-linked Alport syndrome in China / 中华儿科杂志

<p><b>OBJECTIVE</b>Alport syndrome (AS) is a progressive renal disease characterized by hematuria and progressive renal failure. X-linked dominance is the major inheritance form of the syndrome, accounting for almost 80% of the cases, caused by mutations in COL4A5 genes. There is currently no effective treatment that has been shown to favorably affect the outcome of AS, so early diagnosis and even prenatal diagnosis is very important.</p><p><b>METHODS</b>In this study mutation of COL4A5 was detected by amplifying the entire coding sequence mRNA of peripheral blood lymphocytes using nested PCR in two Chinese X-linked dominant Alport syndrome (XLAS) families, then the first prenatal diagnosis of XLAS in China was performed. Mutation analysis of the fetus was performed on both cDNA-based level and DNA-based level of amniocytes. Fetus sex was determined by PCR amplification of SRY as well as karyotypes analysis. Maternal cells contamination was excluded by linkage analysis.</p><p><b>RESULTS</b>There was a deletion mutation in the proband of the first family, 2696 - 2705 del gtatgatggg in the 32 exon of COL4A5, but the mother did not carry the mutation (de novo). There was a G to A substitution at position 4271 in exon 46 of COL4A5 gene (c.G4271A) in the second family, the mother also carried this mutation. After genetic counselling, only the second family accepted prenatal diagnosis. Both amniocytes cDNA level and amniocytes genomic DNA level based prenatal diagnosis showed that the fetus did not carry the same mutation as the mother. PCR amplification of SRY and karyotypes analysis showed a male fetus. Linkage analysis of X chromosome polymorphic microsatellite markers showed that there was no MCC in amniocytes.</p><p><b>CONCLUSION</b>Both cDNA level and DNA level analysis could enhance the accuracy and reliability of prenatal diagnosis. PCR amplification of SRY was faster than karyotypes analysis in the fetal sex determination. Linkage analysis was useful in the detection of maternal cells contamination in amniocytes.</p>

Clinical characteristics and WT1 genetic analysis of patients with steroid resistant nephrotic syndrome accompanied with genitourinary malformations / 中南大学学报(医学版)

OBJECTIVE@#To understand WT1 mutations in patients with steroid resistant nephrotic syndrome (SRNS) accompanied with genitourinary malformations.@*METHODS@#Three cases of SRNS accompanied with genitourinary malformations were enrolled. The expression of podocyte molecules (nephrin, podocin, alpha-actinin-4, WT1, and CD2AP) in 2 cases was analyzed with the immunofluorescence and immunohistochemistry techniques. The genomic DNA and cDNA of WT1 were analyzed by using PCR and RT-PCR, respectively. GeneScan and GeneScan software were used to quantify the ratio of +KTS/-KTS isoforms.@*RESULTS@#The onset ages of 3 cases were 6 months, 1 year, and 10 years old, respectively. The diagnosis age was 7 months, 9 years, and 15 years old, respectively. The phenotype of Case 1 and Case 3 was male accompanied with genitourinary malformations. Case 2 was phenotypic female. Karyotype analysis of 3 cases revealed 46, XY. Three cases were diagnosed as SRNS. Focal segmental glomerulosclerosis (FSGS) was confirmed in 2 cases. Podocyte molecular expression altered in renal tissues of 2 cases. In addition, WT1 staining was negative in Case 1. WT1 expression in Case 2 showed diffuse nuclear staining with less obvious speckles compared with controls. WT1 IVS 9 +5 G>A mutation was detected in Case 2 and WT1 Exon 9 1186 G>A mutation was detected in Case 3. No WT1 mutation was detected in Case 1.@*CONCLUSION@#Karyotype analysis and WT1 genetic testing should be done in all female patients with early onset steroid resistant FSGS and in male patients with SRNS accompanied with genitourinary malformations. Abnormal podocyte molecular expression suggests that more podocyte molecules might be involved in the pathogenesis of proteinuria in WT1 mutational patients.

Renoprotective effects of matrine on experimental glomerulosclerosis in rats / 中华儿科杂志

<p><b>OBJECTIVE</b>Matrine has an anti-fibrosis effect, such as hepatic cirrhosis and derma fibrosis, while its effect on glomerulosclerosis is unknown. The purpose of this study was to analyze the renoprotective effects of matrine on experimental glomerulosclerosis in rats and inquire into its mechanisms.</p><p><b>METHODS</b>The rats were randomly assigned to following groups: normal control group, model control group, benazepril treatment group, matrine 100 mg/kg treatment group and matrine 50 mg/kg treatment group. The rats of normal control group were subjected to sham operation and were injected with normal saline via the tail vein one week later. The rats of the other groups were uninephrectomized and injected with adriamycin (5 mg/kg) via the tail vein one week later. The dose of benazepril was 6 mg/kg. Both matrine and benazepril were given by gastric perfusion from the first day after the operation. The level of urinary protein was measured at the 2nd, 4th and 6th week after the operation. The serum total protein and albumin, serum creatinine, blood urea nitrogen (BUN) were tested only at the 6th week after operation. Renal pathology changes were evaluated at the 6th week as well. Immunohistochemistry was used to detect the expression of fibronectin (FN), laminin (LN), connective tissue growth factor (CTGF) and transforming growth factor-beta1 (TGF-beta1) in glomeruli.</p><p><b>RESULTS</b>Matrine and benazepril not only reduced the excretion of urinary protein and the level of serum creatinine and BUN, but also significantly ameliorated glomerular mesangial proliferation and glomerular sclerosis (P < 0.05, respectively). Immunohistochemical staining indicated that there was an increasing FN, LN, CTGF and TGF-beta1 expression in model control group as compared to the three treatment groups (P < 0.05). Matrine 100 mg/kg treatment group and benazepril treatment group showed much more advantages than matrine 50 mg/kg treatment group (P < 0.05), but there was no significant difference between the former two groups (P > 0.05).</p><p><b>CONCLUSION</b>Matrine has a renoprotective effect on experimental glomerulosclerosis in rats, the possible mechanism might relate to the reduction of the TGF-beta1 negative function via CTGF, which will inhibit the activation and proliferation of glomerular intrinsic cells, decrease the secretion of ECM accordingly.</p>

Study Progress of Henoch-Schonlein Purpura Associated with Antiphospholipid Syndrome / 实用儿科临床杂志

Henoch-Schonlein purpura(HSP)is the most common vasculitic disease affecting children,which is a multisystem immunoglobulin A mediated vasculitis with a self-limited course affecting the skin,joints,gastrointestinal tract and kidneys.Antiphospholipid syndrome(APS)is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss,accompanied by elevated titers of antiphospholipid antibodies.Both HSP and APS are multisytem diseases and are thought to result from immunologically mediated processes.It was known that APS can associated with HSP,but the relationship between the 2 diseases is unclear.The literature on HSP associated with APS was reviewed and the possible mechanism was analyzed.

Correlation between X-Inactivation of Different Tissues and Phenotypes in Female with X-Linked Alport Syndrome / 实用儿科临床杂志

Objective To explore the correlation between phenotypes in female with X-linked Alport syndrome(XLAS) and X-inactivation of different tissues.Methods Thirty-six female diagnosed as XLAS were studied,and proteinuria was taken as a marker of the severity of clinical phenotypes.X-inactivation patterns were analyzed in peripheral blood cells of 36 XLAS female and in skin fibroblasts of 12 XLAS female.The X-inactivation analysis was performed by using Hpa Ⅱ predigestion of DNA followed by polymerase chain reaction(PCR) of the highly polymorphic CAG repeat of the androgen receptor gene.Results The average X-inactivation levels of the mutant allele decreased while the degree of proteinuria increased,so there was a negative correlation between the degree of proteinuria and the X-inactivation ratios of the mutant allele in blood cells(r=-0.543,P=0.002).However,there was no correlation between the degree of proteinuria and the X-inactivation ratios of the mutant allele in skin fibroblasts(r=-0.131,P=0.701).Conclusions X-inactivation ratios might explain partially the diverse phenotypes in XLAS female patients,which suggested that the prognosis of XLAS female might be predicted via analysis of the X-inactivation in peripheral blood cells.