Objective:To investigate the clinically curative effects of Liyuan Pure Lysothrombusase(LYPL，力源精纯溶栓酶) on unstable angina pectoris (UAP) and to observe its safety.Methods:One hundred and twenty cases with UAP were selected and in accordance with the proportion of one to one randomly divided into treated and control group.Based on conventional therapy the LYPL infused intravenously was added in treated group.The conditions of symptomatic convalescence,the changes in electrocardiography (ECG),and the parameters of blood rheology were observed for two groups.Results:The curative effects on symptoms and ECG were 93.33％ and 88.33％ respectively in treated group but in control group they were 70.00％ and 68.33％ respectively.The difference in comparison between two groups was significant (all P<0.01).In treated group the parameters of blood rheology were obviously improved meanwhile in control group they were not.Conclusions:LYPL possesses the actions on improving blood rheology.Its curative effects on UAP are marked and it is safe and easy to use.

A total of 258 cases of elderly patients with herpes zoster were divided into treatment group (n =128) and control group (n =130).The treatment group received a once daily dose of narrowband ultraviolet irradiation plus oral acyclovir 0.8 g,5 times a day.However,the control group received a once daily dose of infrared therapy plus the same oral acyclovir.At Day 9,the effective rates of treatment and control groups were 91.7％ (117/128) vs.74.6％ (97/130) (P＜0.05).Also,in terms of pain relief time (2.56 ± 1.51) vs.(5.44 ±4.06) days,crusting time (4.51 ±0.48) vs.(6.11 ± 1.81) days and healing time (5.65 ±0.56) vs.(9.28 ±0.21) days,the treatment group was better than those of the control group (P ＜0.01).

A potential pathological role of angiopoietins (Ang) in glomeruli following podocyte injury-induced progressive glomerulosclerosis was explored. Eighty male Wistar rats were randomly allocated into sham operation group (Sham, n = 25), Uninephrectomy group (UPHT, n = 25) and Uninephrectomy+Daunorubicin group (DRB, n = 30). In DRB group, daunorubicin (5 mg/kg) was injected via tail vein on the 7th and 14th day after uninephrectomy. At week 1, 2, 4, 6 and 8 respectively following establishment of the animal model, 5 rats in Sham group and UPHT group, and 6 in DRB group were taken respectively for determining 24-h urinary protein excretion rate (24hUPER), blood urea nitrogen (BUN) and serum creatinine (Scr). The sections of kidneys were examined by an electric microscope, PAS staining, immunohistochemical staining and in situ hybridization histochemistry. The results showed that 24hUPER, BUN and Scr in DRB group were more than those in Sham group and UPHT group at the same time points, and there was a trend towards an increase on level of GSI in DRB group from week 2 to week 8. Electric microscopy revealed that podocyte injury presented in DRB group. The expression of Ang1 mRNA and protein in glomeruli of DRB group was decreased, while the expression of Ang2 protein in glomeruli of DRB group increased. Meanwhile, the expression of Ang1 mRNA had a negative correlation with the expression of Ang2 mRNA, and the expression of Ang1 protein had a positive correlation with the expression of Ang1 mRNA, and had a negative correlation with 24hUPER, BUN, Scr, glomerular sclerotic index (GSI), the expression of Ang2 protein and CoIV protein. The expression of Ang2 protein had a positive correlation with the expression of Ang2 mRNA, and had a positive correlation with 24hUPER, BUN, Scr, GSI, the expression of CoIV protein. It was concluded that podocyte injury might lead to an alteration in the expression of Ang1 and Ang2 within glomeruli. Ang2 may get rid of inhibition from Ang1 for downregulation of the Ang1 expression, which facilitate upregulation of the Ang2 expression in glomeruli to promote progressive glomerulosclerosis in the rats.

This study was aimed to observe the effect of Bu-Shen Huo-Xue Tong-Lin (BSHXTL) formula on gene expression of Caspase-3 in benign prostatic hyperplasia (BPH) rats. A total of 72 rats were randomly divided into the sham operation group, model group, proscar group, low-dose BSHXTL group, middle-dose BSHXTL group and high-dose BSHXTL group. The rat model was established by injecting testosterone propionate for 30 days after removing testis except the sham operation group. The drugs were administered once a day at the same time of the model establishment. Immunohistochemical method was used to measure positive average gray. And RT-PCR was used to measure the gene expression of Caspase-3.The results showed that compared with the model group, the expression of Caspase-3 and mRNA in each treatment groups were increased (P < 0.05). And the high-dose BSHXTL group was higher than the proscar group and low-dose BSHXTL group (P< 0.05). It was concluded that BSHXTL formula may upregulate gene expression of Caspase-3, which may be the mechanism of BPH treatment.

AIM:To study the potential pathological role of abnormal expression of endogenous angiopoietins in progressive glomerulosclerosis.METHODS:80 male Wistar rats were randomly allocated into sham operation group(sham,n=25),unilateral nephrectomy group(UNx,n=25)and UNx+daunorubicin(DRB)group(n=30).The rats in DRB group were intravenously injected with DRB(5 mg/kg)on the seventh and the fourteenth day respectively after excising one kidney.Then,at week 1,2,4,6 and 8,5,male Wistar rats from each group were taken randomly for determining 24 h urinary protein quantitative measurement(24hUPQ),BUN,Scr,and the kidneys were examined by electronic microscope,PAS staining,immunohistochemical staining and in situ hybridization histochemistry.RESULTS:There was a trend towards an increase respectively in levels of 24hUPQ,Bun,Scr,GSI in DRB from week 2 to week 8.Electronic microscope revealed that podocyte injury presented in DRB group.Expression of Ang1 mRNA and protein in glomerulus in DRB group decreased,while expression of Ang2 protein in glomeruli in DRB group increased.In DRB group,expression of Ang1 protein had a negative correlation with 24hUPQ,BUN,Scr,GSI,expression of Ang2 protein and CoIV protein.Expression of Ang2 protein had a positive correlation with 24hUPQ,BUN,Scr,GSI,expression of CoIV protein.CONCLUSION:Podocyte injury may lead to glomeruli abnormally express angiopoietins.A decrease in expression of Ang1,and upregulation in expression of Ang2 may facilitate progressive glomerulosclerosis in the rat.

Objective: To summarize and review the clinical and genetic features of neonatal sclerosing cholangitis (NSC) caused by DCDC2 variations. Methods: Whole exome sequencing was performed to identify DCDC2 variants in two Chinese siblings with NSC who were diagnosed in Children's Hospital of Fudan University in May 2017. Clinical, laboratory and genetic data of the two cases were summarized. Key words of "DCDC2" "neonatal sclerosing cholangitis" were searched in Chinese databases and PubMed for articles published until April 2018, and all the relevant literature were reviewed. Results: Patient 1 was a 3-year-and-2-month-old boy. He was admitted to our hospital due to cholestasis for 3 years. Laboratory findings showed elevated levels of gamma-glutamyl transpeptidase (161-1 092 U/L) and total cholesterol (5.4-7.7 mmol/L). Magnetic resonance cholangiopancreatography showed multiple dilations of intrahepatic bile ducts and bilateral hydronephrosis. Patient 2, the older brother of patient 1, was a 9-year-and-9-month-old boy. He was admitted to our hospital due to "cholestasis for 9 years" . CT angiography showed hydrocephalus and left internal carotid artery aneurysms with vascular malformations. A homozygous variant c.529dupA (NM_001195610) in DCDC2 gene was identified in patient 1 by whole exome sequencing. Patient 2 was a homozygote and his parents were heterozygotes with the variation. There has been 2 relevant articles published (Chinese 0, English 2), which reported 11 cases of DCDC2-related NSC in total. All the 13 patients, including the 2 cases reported here, had an onset of symptoms at 0 to 6 months of age. The most common clinical manifestation was cholestasis with high gamma-glutamyl transpeptidase levels, acholic stool, and progression to portal hypertension. Renal and neurological abnormalities were also frequently present. Hypercholesterolemia was observed in one case. Radiological findings revealed the characteristic strictures and dilatations of the intrahepatic and (or) extrahepatic biliary tree. Liver histological examination showed peripheral ductopenia, ductal plate malformation, fibrosis, and cirrhosis. Among the 13 patients, 10 patients required liver transplantation. A total of 7 types of DCDC2 variants were detected in 13 patients. Conclusions DCDC2-related NSC is characterized by the onset of cholestasis with high gamma-glutamyl transpeptidase level and acholic stool in early infancy, which was likely to progress to cirrhosis in early childhood. Renal and neurological abnormalities are also frequently present.Cholangiography or magnetic resonance cholangiopancreatography show strictures and dilatations of the intrahepatic or (and) extrahepatic biliary tree. Identification of pathogenic DCDC2 variants would aid the diagnosis of NSC.

ObjectiveTo investigate the clinical and genetic features of patients with glycogen storage disease type Ⅸa (GSD Ⅸa), and to improve the clinical understanding of the disease. MethodsA retrospective analysis was performed for the clinical data of 20 patients who were hospitalized and genetically diagnosed with GSD Ⅸa in Children’s Hospital of Fudan University from January 2015 to December 2018, and their clinical and genetic features were summarized. ResultsAll 20 patients with GSD Ⅸa were male, with a median age of 2.5 years at the time of confirmed diagnosis. All patients had hepatomegaly and elevated aminotransferases; of all patients, there were 5 patients (250%) with growth retardation, 19 (95.0%) with fasting hypoglycemia, 14 (70.0%) with hyperlactatemia, 9 (45.0%) with hypertriglyceridemia, and 5 (25.0%) with hypercholesterolemia. Fasting blood ketone was measured for 8 patients and all of these patients had an increase in blood ketone; all patients had normal uric acid, and 5 patients (25.0%) had positive urine ketone. Liver biopsy was performed for 18 patients, among whom 15 had mild to moderate liver fibrosis. A total of 16 mutations were detected in the PHKA2 gene, among which 5 were known pathogenic mutations and 11 were novel mutations, and most of the mutations were detected in the c.3614 locus. All patients were treated with uncooked cornstarch, and most patients achieved an improvement in clinical manifestations. ConclusionGSD Ⅸa is more common in male patients. This disease should be considered for patients with hepatomegaly, elevated aminotransferases, growth retardation, fasting hypoglycemia, elevated fasting blood ketone, and normal uric acid. Liver biopsy may help with the diagnosis of this disease, and clinical biochemical parameters and gene detection can be used to confirm diagnosis and classification. Most patients have mild clinical manifestations, while some patients may have liver fibrosis, and treatment with uncooked cornstarch can improve the condition of this disease.

A potential pathological role of angiopoietins (Ang) in glomeruli following podocyte injury-induced progressive glomerulosclerosis was explored. Eighty male Wistar rats were randomly allocated into sham operation group (Sham, n = 25), Uninephrectomy group (UPHT, n = 25) and Uninephrectomy+Daunorubicin group (DRB, n= 30). In DRB group, daunorubicin (5 mg/kg)was injected via tail vein on the 7th and 14th day after uninephrectomy. At week 1, 2, 4, 6 and 8 respectively following establishment of the animal model, 5 rats in Sham group and UPHT group,and 6 in DRB group were taken respectively for determining 24-h urinary protein excretion rate (24hUPER), blood urea nitrogen (BUN) and serum creatinine (Scr). The sections of kidneys were examined by an electric microscope, PAS staining, immunohistochemical staining and in situ hybridization histochemistry. The results showed that 24hUPER, BUN and Scr in DRB group were more than those in Sham group and UPHT group at the same time points, and there was a trend towards an increase on level of GSI in DRB group from week 2 to week 8. Electric microscopy revealed that podocyte injury presented in DRB group. The expression of Ang1 mRNA and protein in glomeruli of DRB group was decreased, while the expression of Ang2 protein in glomeruli of DRB group increased. Meanwhile, the expression of Angl mRNA had a negative correlation with the expression of Ang2 mRNA, and the expression of Angl protein had a positive correlation with the expression of Angl mRNA, and had a negative correlation with 24hUPER, BUN, Scr, glomerular sclerotic index (GSI), the expression of Ang2 protein and CoIV protein. The expression of Ang2 protein had a positive correlation with the expression of Ang2 mRNA, and had a positive correlation with 24hUPER, BUN, Scr, GSI, the expression of CoIV protein. It was concluded that podocyte injury might lead to an alteration in the expression of Angl and Ang2 within glomeruli. Ang2 may get rid of inhibition from Ang1 for downregulation of the Angl expression, which facilitate upregulation of the Ang2 expression in glomeruli to promote progressive glomerulosclerosis in the rats.

Objective: To understand the results of tuberculin skin test (PPD) in preschool children after the vaccination of BCG, and to analyze the effect of BCG vaccination on latent tuberculosis infection in children. Methods: From January to November 2018, a total of 1 359 preschool children from 14 kindergartens in 8 districts and cities of Jiangsu Province were selected for tuberculin test(PPD), and chest X-ray examination was performed on children with strong PPD results. Results: The positive rate of PPD in preschool children in Jiangsu Province was 23.33%, of which strong positive and moderate positive (PPD≥10 mm) were totaled 6.47%. There were 149 boys (21.29%) with PPD positive reactions and 168 girls(25.50%) with PPD positive reactions, and differences of PPD positive reactions with different genders were of no statistical significance (χ2=3.36, P>0.05) And there were 201 children (25.35%) with PPD positive reactions in northern Jiangsu, 116 children (20.50%) with PPD positive reactions in southern Jiangsu. There were significant differences in the results of PPD positive and negative reactions between different regions(χ2=4.35, P<0.05). There was 1 case of PPD positive reactions among 3-year-old children(0.71%), 19 cases among 4-year-old children(3.89%), 31 cases among 5-year-old children(8.96%), and 37 cases among 6-year-old children(9.63%), and the differences of PPD positive reactions of different age groups were of statistical significance(χ2=21.69, P<0.01). Conclusion The positive rate of PPD in preschool children in Jiangsu Province is very low, indicating that PPD can be used as a detection method for latent infection in children. The overall positive rate of PPD in preschool children in Jiangsu Province is also low, and appropriate measures should be taken to protect susceptible children and effectively prevent and control childhood tuberculosis.