1.Clinicopathological features and prognosis of 128 children with idiopathic membranous nephropathy
Junmei LIU ; Peipei SHI ; Limin JIA ; Lu CAO ; Huating ZHANG ; Qin WANG ; Jianjiang ZHANG
Chinese Journal of Applied Clinical Pediatrics 2023;38(6):452-456
Objective:To analyze the clinicopathological features and prognosis of idiopathic membranous nephropathy (IMN) in children, and to investigate the factors influencing their prognosis.Methods:The clinical and pathological data of 128 children with IMN hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019 were retrospectively analyzed.They were divided into 2 groups according to the pathological manifestations: group A[typical membranous nephropathy(MN) group] and group B (atypical MN group), and the clinicopathological characteristics of the 2 groups were compared.Different treatment regimens and their efficacy were summarized, and the prognosis and its influencing factors were analyzed.The primary endpoint event at follow-up was the occurrence of end stage renal disease (ESRD), and the secondary endpoint event was the occurrence of renal insufficiency.Children with IMN were further divided into endpoint event group and non-endpoint event group according to the presence or absence of endpoint events at the last follow-up.Survival analysis was performed using the Kaplan-Meier survival curve method.The Cox proportional risk model method was used to analyze the factors influencing the prognosis of poor kidney outcomes in children with IMN. Results:(1)A total of 128 children were included, with the male-to-female ratio of 1.13∶1.00.The median age of onset and peak age of onset were 13.0 (10.3, 15.0) years, and 12-16 years (68.8%), respectively.Massive proteinuria was detected in 119 cases (93.0%), including 103 cases (80.5%) with massive proteinuria and hematuria, 4 cases(3.1%) with simple hematuria, and 5 cases (3.9%) with non-renal proteinuria.There were 29 cases (22.7%) in group A and 99 cases (77.3%) in group B. (2)Blood triacylglycerol level was significantly higher in group B than that of group A[2.1 (1.5, 3.0) mmol/L vs.1.7(1.1, 2.5) mmol/L], while high-density lipoprotein[1.5(1.1, 1.8) mmol/L vs.1.8(1.4, 2.1) mmol/L], serum albumin[22.0(17.0, 27.3) g/L vs.25.5 (21.0, 32.5) g/L] and complement C3[(1.1±0.2) g/L vs.(1.2±0.2) g/L] were significantly lower in group B than those of group A (all P<0.05). (3)Complete clinical data during hospitalization and follow-up data were obtained from 91 children with IMN, with a median follow-up time of 87.0 (49.0, 104.5) months.Among them, 5 cases (5.5%) progressed to ESRD, involving 3 cases received renal transplantation, and 9 cases (9.9%) had secondary endpoints.Cumulative renal survival rate for ESRD at 5 and 10 years were 96.2% and 92.9%, respectively, which, for the secondary endpoints at 5 and 10 years were 95.2% and 84.8%, respectively.(4)Kaplan-Meier survival analysis showed no significant difference in the cumulative renal survival between group A and group B ( P>0.05). Multifactorial Cox regression analysis showed that tubular atrophy/interstitial fibrosis was an independent risk factor for renal insufficiency in children with IMN ( HR=0.102, 95% CI: 0.011-0.940, P<0.05). Conclusions:Massive proteinuria combined with hematuria is the major clinical manifestation of IMN in children, and atypical MN is the major pathological manifestation.Tubular atrophy/interstitial fibrosis is an independent risk factor for renal insufficiency in children with IMN.
2.Spondyloenchondrodysplasia with immune dysregulation caused by ACP5 gene mutation: a case report and literature review
Peipei SHI ; Hua WANG ; Jianjiang ZHANG ; Zhen LIU ; Huiqin ZENG ; Miao WANG ; Huating ZHANG
Chinese Journal of Applied Clinical Pediatrics 2022;37(1):50-53
Objective:To summarize the clinical features and gene phenotype of children with spondyloenchondrodysplasia with immune dysregulation (SPENCDI) caused by ACP5 gene mutation. Methods:The medical data and genetic phenotype of a child diagnosed with SPENCDI in the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University in February 23, 2017 were analyzed retrospectively.Besides, " spondyloenchondrodysplasia" were taken as the search terms to perform the retrieval in CNKI, Wanfang Data, and PubMed, in an attempt to conduct the literature review.χ 2 test was used to compare the factors among children with different mutations. Results:The 4.5-year-old girl was admitted to hospital for complaint of " fever and chilblain-like rash" when she was 2 years old.She was diagnosed with systemic lupus erythematosus (SLE) concomitant with lupus nephritis.Methylprednisolone combined with cyclophosphamide, mycophenolate mofetil was used for the treatment.However, she experienced multiple infections, thrombocytopenia, limp, and growth retardation during the treatment.Genetic detection identified ACP5 gene compound hybrid mutation: c.779C>A and c. 770T>C.She was diagnosed with SPENCDI, and was subjected to follow-up.A total of 78 SPENCDI patients were retrieved from the databases, with various clinical manifestations of SPENCDI, commonly with skeletal involvement and immune phenotypes; 73.08% of the cases were positive for antinuclear antibodies, 57.69% of cases were positive for anti-double stranded-DNA antibodies and 34.62% of cases had neurological symptoms.In 58 cases, ACP5 gene mutations were detected, including 44 homozygous mutations and 14 compound heterozygous mutations.Patients with ACP5 gene homozygous mutation had a higher probability of consanguineous marriage in parents [56.82% (25/44 cases) vs.14.29% (2/14 cases)]; patients with ACP5 gene heterozygous mutation were more likely to develop SLE [64.29% (9/14 cases) vs.34.09% (15/44 cases)]( χ2=7.722, 3.992; all P<0.05). Conclusions:The majority of the ACP5 gene mutations are homozygous mutations in patients with SPENCDI, and heterozygous mutations are rare.The clinical manifestations of SPENCDI are various and complex, it is prone to develop autoimmune diseases, and there was no clear correlation between clinical features and gene phenotype in SPENCDI patients.
3.Epidemic characteristics of the coexistence of common chronic diseases among community residents in Jiading District , Shanghai
An-le LI ; Gen-ming ZHAO ; Feng JIANG ; Ji-long WANG ; Yi-feng WANG ; Wei-feng ZHANG ; Ying JI
Journal of Public Health and Preventive Medicine 2022;33(5):141-143
Objective To explore epidemic characteristics of the coexistence of common chronic diseases among community residents in Jiading District, Shanghai, and to provide a basis for comprehensive prevention and control of chronic diseases in the community. Methods A multi-stage stratified cluster random sampling method was adopted in the present study, and residents over 20 years old in three selected streets (towns) were investigated. SPSS statistical software was used to analyze the data, and χ2 test was used to compare the difference between groups. Results Among the respondents, 24.80% had no chronic diseases and 75.20% had various chronic diseases. The top ten most common diseases were hypertension (37.82%), fatty liver (30.10%), chronic gastritis (18.11%), hyperlipidemia (14.51%), thyroid disease (8.85%), diabetes mellitus (8.65%), renal cyst (8.12%), chronic bronchitis (7.62%), kidney stone (6.97%) and gout (5.75%). The prevalence increased with age. Chronic diseases existed in the form of multiple diseases, and the prevalence rates of two, three, four, five, six and more chronic diseases at the same time were respectively 19.78%, 13.07%, 7.90%, 4.11% and 4.09%. The most common comorbid diseases were hypertension, hyperlipidemia and diabetes combined with other chronic diseases. Conclusion The prevalence of chronic diseases in residents in Jiading District was very high. Most of the residents suffered from multiple chronic diseases in which hypertension, hyperlipidemia and diabetes were the common basis. The comprehensive prevention and control of chronic diseases in the community should be strengthened.
4.Isolation of Growth-Promoting Bacteria and Effect of Compound Bacteria on Yield of Fritillaria przewalskii
Jiang ZHAO ; Shi-jun LIANG ; Tao YANG ; Xiao-long WU ; Peng-nian QI ; Shi-wei WANG ; Zhi-ye WANG
Chinese Journal of Experimental Traditional Medical Formulae 2021;27(24):163-170
Objective:To explore the effects of anti-microbial compound (T1) from
5. A case of coronavirus disease 2019 with tuberculous meningitis
Liang WANG ; Jia CAI ; Huating LUO ; Hongzhi GUAN ; Hongzhi WANG ; Cheng HUANG ; Fachun ZHOU
Chinese Journal of Neurology 2020;53(0):E004-E004
Novel coronavirus pneumonia, also known as coronavirus disease 2019 (COVID-19), is caused by a new coronavirus that infects the lungs. Although some patients with COVID-19 may be combined with neurological symptoms, there is no direct evidence that this new coronavirus can directly invade nerve system. A case of COVID-19 with tuberculous meningitis is reported to remind that when patients with COVID-19 present symptom of encephalitis or meningitis, a comprehensive pathogen examination is recommended.
6.Suspected fetal congenital glaucoma identified by prenatal ultrasound: a case report
Yanpeng SONG ; Huating BI ; Haiyan YU ; Tiezhu WANG
Chinese Journal of Perinatal Medicine 2020;23(8):549-551
We report a case of suspected fetal congenital glaucoma detected by prenatal ultrasound. The mother had no history of cold, medication, or radiation exposure in the first trimester. Routine prenatal ultrasound at 23 +2 weeks of gestation found a 2.5 mm ventricular septum defect, and the sagittal and transverse diameters of the left and right eyeballs were all greater than the normal range of the same gestational weeks, which were noted at 18.57 mm and 17.26 mm, 18.21 mm and 17.22 mm, respectively. Dynamic observation revealed that the bilateral eyelids were unable to close with cornea being exposed to amniotic fluid. The pregnancy was terminated at 23 +6 weeks and a stillborn female weighing 650 g was delivered two days later. Congenital glaucoma was highly suspected by postnatal ophthalmic examination, accompanied by a deformity of the left thumb. No abnormality was detected on fetal chromosome karyotyping or whole-exon sequencing. When unilateral or bilateral megalophthalmos in the fetus is detected by prenatal ultrasound, congenital glaucoma should be considered.
7.Effects of mycophenolate mofetil and glucocorticoid on Henoch-Schonlein purpura nephritis in children
Jianjiang ZHANG ; Limin JIA ; Peipei SHI ; Miao WANG ; Yue HAN ; Huating ZHANG
Chinese Journal of Nephrology 2017;33(9):670-677
Objective To explore the effect and safety of mycophenolate mofetil (MMF) and glucocorticoid on Henoch-Schonlein purpura nephritis in children and compared with cyclophosphamide (CTX).Methods The data of 48 patients diagnosed as Henoch-Schonlein purpura by renal biopsy were retrospectively analyzed.Median follow-up time was 22(7,48) months.The subjects were divided into 2 groups.34 cases were in the MMF group:MMF (15-20 mg · kg-1 · d-1 or 800-1200 mg/m2)+prednisone,and 14 cases in the CTX group:CTX (8-12 mg · kg-1 · d-1) + prednisone.Clinical and laboratory data were collected at baseline and 1,3,6 months after treatment.During follow-up,cumulative retreatment rate and adverse reactions after treatment were recorded.Results In two groups after treatment for 1,3,6 months,24 hours urinary protein quantitative was significantly lower than the baseline value,serum albumin (sAlb) was significantly higher than the baseline value,and serum creatinine (Scr) indicated no statistically significant difference during the follow-up period.After the treatment of 1 month,the efficient rate of MMF group was higher than the CTX group (MMF 73.5 % vs 42.9%,P=0.046),the effective treatment of 3,6 months at the end of the follow-up,no statistically significant difference were observed in the accumulative remission rate.The total rate of retreatment was 10.4% (5/48),where MMF group was lower that of the than CTX group (3.0% vs 28.6%,P<0.001).The retreatment often occurred after discontinuation of prednisone and CTX,MMF reduction process.Eleven children received IMPDH2 gene polymorphisms test in MMF group,9 AA children had shorter time for drugs to be effective than that of the AG and GG children.The incidence of adverse reactions of MMF group was obviously lower than CTX group at the end of the follow-up (8.8% vs 35.7%,P=0.025),where two groups developed fungal infection.Conclusions The short-term effect of both groups are the same,but the recurrent rate and incidence of adverse reactions of MMF group are lower than those of the CTX group.The preliminary study shows that IMPDH2 gene polymorphisms is associated with MMF curative effect and adverse reactions.
8.Effect of microRNAs 224 and 21 on human glioblastoma stem cell survival and the possible molecular mechanisms
Jiaqing WANG ; Yunlong YU ; Huixing WANG ; Huating LIU
Chinese Journal of Biochemical Pharmaceutics 2016;36(11):30-36
Objective To explore the effect of microRNAs 224 and 21 on human glioma stem cells survival and the possible molecular mechanisms.Methods qPCR was used to detect the dysregulated expression of microRNAs in malignant glioma samples, human GBM stem cells, artificially established GBM stem cell lines and human tissues.Caspase 3/7 assay, Annexin V apoptosis/fluorescence assay were performed to determine the effect of miR-21 or miR-224 mimics and inhibitor on cell apoptosis.Living cells count was used to assess miR-21 or miR-224 mimics and inhibitor on cell growth.TargetScan was used to explore potential targets of miR-21 and miR-224, and dual luciferase reporter assay was used to identify whether the 3’UTR of Caspase 3, Caspase 9 and Bim mRNA was a binding target of miR-21 or miR-224.Western blot was used to detect the expression of Caspase 3, Caspase 9 and Bim protein after transfection of miR-21 or miR-224 mimics or inhibitors.Results miR-21 and miR-224 are strongly upregulated in GSC samples, multiple GBM human tumor specimens, and GBM neurosphere stem cell lines ( P<0.05 ) .Caspase 3/7 assay and Annexin V apoptosis/fluorescence assay results showed that miR-224 and miR-21 regulated GSC apoptosis.Living cells count results demonstrated that miR-224 and miR-21 regulated GSC growth.miR-224 and miR-21 regulate pro-apoptotic gene expression by directly targeting Caspase 3, Caspase 9, and Bim 3’-UTRs. Conclusion These results indicate that miR-224 and miR-21 are important physiologic drivers of GSC resistance to apoptosis, providing new points of therapeutic leverage against these treatment-resistant cells.
9.Immune intervention effect of human-mouse chimeric antibody B7-1 against murine lupus nephritis model
Hui SHEN ; Yuqiang ZHU ; Yong KONG ; Jing WANG ; Huating ZHU ; Gehua YU ; Lei CAI ; Ying ZHU ; Zhiyao WANG ; Yuhua QIU
Chinese Journal of Immunology 2015;(9):1200-1205
Objective:On the basis of the use of chemical methods to establish mouse model of lupus nephritis and its biological identification , we investigate the reverse effect of pathological lesions of B 7-1 human-mouse chimeric antibody blockade against B7/D28 signaling pathway in mice with lupus nephritis model.Methods:Pristane was injected intraperitoneally to 6-week-old female C57BL/6J mice at dose of 0.5 ml per mouse in one go,and urine protein,ANA and renal pathological changes were detected on a monthly basis.Mice whose urine protein content reached ++and ANA fluorescence intensity reached ++were randomly devided into three groups ,five each.Antibody intervention group was sequentially injected with B 7-1-mouse chimeric antibody by orbital venous , positive control group was injected with immunosuppressant CTX , negative control group was injected with isotype control IgG.Urine protein and ANA were also detected on a monthly basis.Mice were sacrificed three months after intervention was executed.Kidney was used for H&E dying , IC detection and electric microscope observation.Results: After four-month Pristane induction , urine protein content of 80%mice reached +-+++,meanwhile,serum ANA fluorescence intensity reached ++-+++.Glomerulonephritis infiltrating cells were observed Mice with urine protein and ANA , glomerular inflammatory cell infiltration , tubular epithelial cell degeneration visible edema ,vascular congestion significantly ,fibrosis.After antibody intervention ,urine protein content in antibody intervention group gradually reduced from ++-+++to ±-+++,ANA ++-+++to +-++,and were significantly different from that in the negative control group ( P<0.01 ).Analysis of kidney H&E dying showed that antibody glomerular infiltration of inflammatory cells in the intervention group and tubular congestion and other symptoms were improved significantly.Immunofluorescence staining indicated that fluorescence intensity of IC was significantly reduced in the antibody intervention group.Electron dense deposits reduction and glomerular basement membrane uniformity were observed in antibody intervention group by electric microscope when compared with the negative control group.Conclusion:B7-1 antibodies could downregulate immune response through inhibiting B 7-1/CD28 signaling pathway , reducing the production of autoantibodies and reversing pathological damage caused by autoimmune response .
10.Protective effects of sindacon on myocardial ischemic reperfusion injury in rats
Tiantian YUN ; Huating WANG ; Shumei WANG ; Guohai SU ; Changling LI
Chinese Pharmacological Bulletin 2003;0(07):-
Aim To observe the effects of total flavones of hippophae rhamnoides L on myocardial ischemia-reperfusion injury and the MMP-9 and TIMP-1 expression in cardiac tissues of rats,and to explore the protective mechanism of total flavones of hippophae rhamnoides L on myocardial ischemia-reperfusion injury.Methods Fifty rats were classified into 4 groups by using the random grouping principle:model group,losartan group,sindacon group of different doses and sham operation group.The myocardial ischemia-reperfusion injury model was established by ligating left anterior descending coronary artery for 30 minutes and releasing then.The cardiac muscle tissue was stained by HE to observe its necrosis area and pathological changes as well as the expression of MMP-9 and TIMP-1by immunohistochemistry method.Results HE sections showed that necrosis of cardiac muscle in rats was significantly relieved in sindacon group by using different doses compared with model group,and immunohistochemistry sections showed that the sindacon group using different doses decreased the expression of MMP-9 compared with the model group and increased the expression of TIMP-1.Conclusion Sindacon has protective effect on cardiac muscle after the myocardial ischemia-reperfusion injury,and its mechanism may be related to its inhibition of the MMP-9 and increasing TMPM-1 expression in cardiac muscle.


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