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1.
Zhongguo Dang Dai Er Ke Za Zhi ; 26(9): 914-920, 2024.
Artigo em Zh | MEDLINE | ID: mdl-39267505

RESUMO

OBJECTIVES: To study the risk factors and prognostic characteristics of pediatric silent lupus nephritis (SLN) with class Ⅲ to V. METHODS: A retrospective study was conducted to collect clinical data from 30 children diagnosed with SLN at the Department of Pediatrics, Second Xiangya Hospital, Central South University, from May 2007 to April 2023. Based on renal pathological classification, the patients were divided into a class Ⅱ group (12 cases) and a class Ⅲ to Ⅴ group (18 cases). The risk factors for the occurrence of class Ⅲ to Ⅴ SLN were analyzed, and the prognostic characteristics were summarized. RESULTS: Among the 30 SLN patients, the median follow-up time was 61.50 months. There were no statistically significant differences in the proportions of patients who discontinued glucocorticoids or achieved low disease activity status, nor in the annual decline rate of estimated glomerular filtration rate (eGFR) between the class Ⅱ and class Ⅲ to V groups (P>0.05). However, three patients in the class Ⅱ group progressed to stage 1 chronic kidney disease (CKD), while eight patients in the class III to V group reached stage 1 CKD, and four patients reached stage 2 CKD. Among the 26 female SLN patients, serum complement C3 levels in the class III to V group were lower than those in the class Ⅱ group (P<0.05). Serum C3 levels in SLN patients, as well as in female SLN patients, were negatively correlated with the fluorescence intensity of IgA, IgG, and C3 immune complexes in the kidneys (P<0.05). Additionally, serum C3 levels in female SLN patients were negatively correlated with the renal pathological activity index (P<0.05). Binary logistic regression analysis indicated that being female and having low serum complement C3 levels were risk factors for the occurrence of class Ⅲ to V SLN in children (P<0.05). CONCLUSIONS: Class Ⅲ to V SLN is not uncommon among SLN children, and there remains a risk of long-term renal function progression. Being female and having low serum complement C3 levels are identified as risk factors for class Ⅲ to V SLN in children.


Assuntos
Complemento C3 , Nefrite Lúpica , Humanos , Feminino , Masculino , Criança , Fatores de Risco , Estudos Retrospectivos , Prognóstico , Complemento C3/análise , Adolescente , Taxa de Filtração Glomerular , Pré-Escolar
2.
Artigo em Inglês | MEDLINE | ID: mdl-36549658

RESUMO

BACKGROUND: Congenital anomalies of the kidney and urinary tracts (CAKUT) are the leading cause of kidney failure in children with phenotypic and genotypic heterogeneity. Our objective was to describe the genetic spectrum and identify the risk factors for kidney failure in children with CAKUT. METHODS: Clinical and genetic data were derived from a multicenter network (Chinese Children Genetic Kidney Disease Database, CCGKDD) and the Chigene database. A total of 925 children with CAKUT who underwent genetic testing from 2014 to 2020 across China were studied. Data for a total of 584 children wereobtained from the CCGKDD, including longitudinal data regarding kidney function. The risk factors for kidney failure were determined by the Kaplan-Meier method and Cox proportional hazards models. RESULTS: A genetic diagnosis was established in 96 out of 925 (10.3%) children, including 72 (8%) with monogenic variants, 20 (2%) with copy number variants (CNVs), and 4 (0.4%)with major chromosomal anomalies. Patients with skeletal abnormalities were more likely to have large CNVs or abnormal karyotypes than monogenic variants. Eighty-two patients from the CCGKDD progressed to kidney failure at a median age of 13.0 (95% confidence interval, 12.4-13.6) years, and twenty-four were genetically diagnosed with variants of PAX2, TNXB, EYA1, HNF1B and GATA3 or the 48, XXYY karyotype. The multivariate analysis indicated that solitary kidney, posterior urethral valves, bilateral hypodysplasia, the presence of certain variants and premature birth were independent prognostic factors. CONCLUSIONS: The genetic spectrum of CAKUT varies among different subphenotypes. The identified factors indicate areas that require special attention.

3.
Cell Physiol Biochem ; 40(3-4): 743-756, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27915349

RESUMO

BACKGROUND/AIMS: The study aims to elucidate the roles of 1,25(OH)2D3 and vitamin D receptor (VDR) in the pathogenesis of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) by regulating the activation of CD4+ T cells and the PKCδ/ERK signaling pathway. METHODS: From January 2013 to December 2015, a total of 130 SLE patients, 137 RA patients and 130 healthy controls were selected in this study. Serum levels of 1,25(OH)2D3 and VDR mRNA expression were detected by ELISA and real-time fluorescence quantitative PCR (RT-qPCR). Density gradient centrifugation was performed to separate peripheral blood mononuclear cells (PBMCs). CD4+ T cells were separated using magnetic activated cell sorting (MACS). CD4+T cells in logarithmic growth phase were collected and assigned into 9 groups: the normal control group, the normal negative control (NC) group, the VDR siRNA group, the RA control group, the RA NC group, the VDR over-expressed RA group, the SLE control group, the SLE NC group, and the VDR over-expressed SLE group. The mRNA and protein expressions of VDR, PKCδ, ERK1/2, CD11a, CD70 and CD40L were detected by RT-qPCR and Western blotting. Bisulfite genomic sequencing was conducted to monitor the methylation status of CD11a, CD70 and CD40L. RESULTS: Compared with healthy controls, serum 1,25(OH)2D3 level and VDR mRNA expression in peripheral blood were decreased in SLE patients and RA patients. With the increase of concentrations of 1,25(OH)2D3 treatment, the VDR mRNA expression and DNA methylation levels of CD11a, CD70 and CD40L were declined, while the expressions of PKCδ, ERK1/2, CD11a, CD70 and CD40L were elevated in SLE, RA and normal CD4+T cells. Compared with the SLE contro, RA control, SLE NC and RA NC groups, the expressions of PKCδ, ERK1/2, CD11a, CD70 and CD40L decreased but DNA methylation levels of CD11a, CD70 and CD40L increased in the VDR over-expressed SLE group and VDR over-expressed RA group. However, compared with the normal control and normal NC groups, the expressions of PKCδ, ERK1/2, CD11a, CD70 and CD40L increased, but DNA methylation levels of CD11a, CD70 and CD40L decreased in the VDR siRNA group. Compared with the normal control group, the expressions of PKCδ, ERK1/2, CD11a, CD70 and CD40L increased, but DNA methylation levels of CD11a, CD70 and CD40L decreased in the SLE control and RA control groups. CONCLUSION: Our study provide evidence that 1,25(OH)2D3 and VDR could inhibit the activation of CD4+ T cells and suppress the immune response of SLE and RA through inhibiting PKCδ/ERK pathway and promoting DNA methylation of CD11a, CD70 and CD40L.


Assuntos
Artrite Reumatoide/imunologia , Linfócitos T CD4-Positivos/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Ativação Linfocitária/imunologia , Sistema de Sinalização das MAP Quinases , Proteína Quinase C-delta/metabolismo , Receptores de Calcitriol/metabolismo , Adulto , Antígenos CD/metabolismo , Artrite Reumatoide/sangue , Artrite Reumatoide/genética , Calcitriol/sangue , Calcitriol/farmacologia , Estudos de Casos e Controles , Metilação de DNA/genética , Feminino , Regulação da Expressão Gênica , Vetores Genéticos/metabolismo , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/genética , Masculino , Proteína Quinase C-delta/genética , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , RNA Interferente Pequeno/metabolismo , Receptores de Calcitriol/genética , Transfecção
4.
BMC Urol ; 13: 41, 2013 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-23964797

RESUMO

BACKGROUND: The incidence of AKI appears to have increasing trend. Up to now, prospective, multi-center, large-sample epidemiological study done on pediatric AKI on aspects of epidemiological characteristics, causes and outcomes have not reported. It is necessary to develop prospective, multi-center, large-sample epidemiological study in our country on pediatric AKI. The aim of this study was to determine the clinical features, etiology, and outcomes of acute kidney injury (AKI) in Chinese children. METHOD: Paediatric patients (≤18 years old) admitted to 27 hospitals (14 children's hospitals and 13 general hospitals) affiliated with the Medical University were investigated. AKI was defined using the 2005 Acute Kidney Injury Network criteria. RESULTS: During the study period, 388,736 paediatric patients were admitted. From this total, AKI was diagnosed in 1,257 patients, 43 of whom died. The incidence and mortality of AKI was 0.32% and 3.4% respectively. The mean (± SD) age of patients was 48.4 ± 50.4 months. Among the 1,257 AKI paediatric patients, 632 were less than one year old. Among the AKI paediatric patients, 615 (48.9%) were in stage 1, 277 (22.0%) in stage 2, and 365 (29.0%) in stage 3. The most common causes of AKI were renal causes (57.52%), whereas postrenal (25.69%) and prerenal (14.96%) causes were the least common. The three most common causes of AKI according to individual etiological disease were urolithiasis (22.35%), of which exposure to melamine-contaminated milk accounted for the highest incidence (63.7%); acute glomerulonephritis (10.10%); and severe dehydration (7.48%). A total of 43 AKI patients (3.4%) died during their hospital stay; 15 (34.9%) of the 43 died as a result of sepsis. CONCLUSION: Primary renal diseases are a major risk factor for paediatric AKI in China. In terms of specific etiological disease, urolithiasis (postrenal disease) was the leading cause of paediatric AKI in 2008, when the disease was linked to exposure to melamine-contaminated milk. Sepsis is the leading cause of death in Chinese paediatric AKI patients. Future studies should focus on effective ways of controlling renal disorders and sepsis to improve the clinical management of paediatric AKI in China.


Assuntos
Injúria Renal Aguda/mortalidade , Doenças Transmitidas por Alimentos/mortalidade , Nefrite/mortalidade , Sepse/mortalidade , Triazinas/intoxicação , Urolitíase/mortalidade , Injúria Renal Aguda/diagnóstico , Injúria Renal Aguda/terapia , Adolescente , Distribuição por Idade , Causalidade , Criança , Pré-Escolar , China/epidemiologia , Comorbidade , Feminino , Doenças Transmitidas por Alimentos/diagnóstico , Doenças Transmitidas por Alimentos/terapia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Nefrite/diagnóstico , Nefrite/terapia , Estudos Prospectivos , Sepse/diagnóstico , Sepse/terapia , Taxa de Sobrevida , Urolitíase/diagnóstico , Urolitíase/terapia
5.
Zhongguo Dang Dai Er Ke Za Zhi ; 13(4): 273-7, 2011 Apr.
Artigo em Zh | MEDLINE | ID: mdl-21507292

RESUMO

OBJECTIVE: To explore possible correlations between renal Th1/Th2 ratio and renal microvascular injury in children with Henoch-Sch-nlein purpura nephritis (HSPN). METHODS: Thirty-two children with HSPN were enrolled. They were classified into four groups by renal pathology: HSPN class II (n=8), HSPN class IIIa (n=7), HSPN class IIIb (n=10) and HSPN class IV/V (n=7). Five patients undergoing nephrectomy due to trauma were used as the controls. INFγ, IL-4 and CD34 in the renal tissues were measured by immunohistochemical analysis. INFγ was used as a marker of Th1, IL-4 was used as a marker of Th2 and CD34 was used as a marker of microvessel. The renal microvessel density was evaluated according to the Weidner standard. The relationships among the local Th1/Th2 ratio, renal pathological grade, microvessel score and microvessel density were studied. RESULTS: Immunohistochemical analysis showed a lower expression of INFγ and a higher expression of IL-4 in the HSPN groups than in the control group. The local Th1/Th2 ratio in the HSPN groups decreased and correlated significantly with the renal pathological grade. There were significant differences among four HSPN subgroups (P<0.05). Compared with the control group, the renal microvessel density in the HSPN class II and class IIIa groups increased significantly (P<0.05), but it decreased in the HSPN class IV/V group (P<0.05). The renal microvessel scores in the HSPN class IIIa, class IIIb and class IV/V groups increased significantly compared with those in the control and the HSPN classⅡ. The increased renal microvessel scores were associated with more severe renal pathological changes. A negative correlation was found between the local Th1/Th2 ratio and the microvessel density in kidneys (r=-0.921, P<0.01). CONCLUSIONS: The decrease of Th1/Th2 ratio in kidneys might be responsible for renal microvascular injury in children with HSPN.


Assuntos
Vasculite por IgA/imunologia , Rim/irrigação sanguínea , Nefrite/imunologia , Células Th1/imunologia , Células Th2/imunologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Vasculite por IgA/patologia , Rim/patologia , Masculino , Microvasos/patologia , Nefrite/patologia
6.
Zhongguo Dang Dai Er Ke Za Zhi ; 12(10): 788-92, 2010 Oct.
Artigo em Zh | MEDLINE | ID: mdl-20959043

RESUMO

OBJECTIVE: To investigate the changes of blood pressure by 24-hour ambulatory blood pressure (ABP) monitoring in children with primary nephrotic syndrome (PNS) and explore the relationship of the changes in blood pressure with rennin-angiotensin-aldosterone system (RAAS) in these children. METHODS: ABP and casual blood pressure (CBP) monitoring were performed in 114 children with PNS. Plasma levels of rennin activity (PRA), angiotensin II (AngII) and aldosterone (ALD) were measured. The correlation of plasma levels of PRA, AngII and ALD with ABP was evaluated. RESULTS: Of the 114 children with PNS, 101 (88.6%) presented elevated blood pressure. Mild or severe masked hypertension was found in 45 children (39.5%). Eighty (70.2%) children showed non-dipper blood pressure. The index and load of systolic blood pressure were higher than those of diastolic blood pressure. The blood pressure index and blood pressure load during sleep were higher than those during wakefulness. The boy presented higher diastolic blood pressure index and load than girls. Decubitus blood PRA, AngII and ALD levels in children with PNS were significantly higher than normal controls. The group with elevated blood pressure presented significantly higher decubitus blood PRA, AngII and ALD levels than the group with normal blood pressure. AngII level was significantly positively correlated with the index and load of both systolic blood pressure and diastolic blood pressure. CONCLUSIONS: The children with PNS present a high incidence of hypertension, with a large percentage of masked hypertension and non-dipper blood pressure. Systolic blood pressure increases more significantly than diastolic blood pressure. Blood pressure during sleep increases more significantly than that during wakefulness. Diastolic blood pressure increases more significantly in boys than in girls. RAAS activity is elevated and the elevated RAAS activity might increase the blood pressure mainly by AngII in children with PNS.


Assuntos
Monitorização Ambulatorial da Pressão Arterial , Pressão Sanguínea , Síndrome Nefrótica/fisiopatologia , Sistema Renina-Angiotensina/fisiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino
7.
Zhongguo Dang Dai Er Ke Za Zhi ; 12(1): 35-9, 2010 Jan.
Artigo em Zh | MEDLINE | ID: mdl-20113632

RESUMO

OBJECTIVE: To evaluate the efficacy and safety of ganciclovir therapy for congenital cytomegalovirus (CMV) infection in newborn infants. METHODS: The randomized controlled trials (RCTs) and quasi-RCTs on ganciclovir therapy for congenital CMV were reviewed in the following electronic databases: PubMed (January 1988 to January 2009), EMbase (January 1988 to January 2009), the Cochrane library (Issue 3, 2003 and Issue 1, 2009), the Chinese Journals Full-text Database (January 1994 to January 2009), the Chinese Biological Medical Disc (January 1994 to January 2009) and the Chinese Medical Current Contents (January 1994 to January 2009). Quality assessment, data extraction, and meta analysis were performed. RESULTS: Ten papers were included. Meta analysis showed that the ganciclovir therapy increased the improvement rate (91.4% vs 34.0%; p<0.01) and led CMV infection indexes to become negative in more patients (87.6% vs 15.3%; p<0.01) and decreased incidence of hearing disturbance (4.7% vs 37.2%; p<0.01) as compared with the non-ganciclovir therapy control group. The incidence of the ganciclovir-therapy-related side effects was low. CONCLUSIONS: Ganciclovir treatment may increase the improvement rate and the rate of CMV infection indexes becoming negative, and decrease incidence of hearing disturbance, with few side effects, in newborn infants with CMV infection. However the supporting evidence is not strong due to few trials and more high-quality research is needed.


Assuntos
Antivirais/uso terapêutico , Infecções por Citomegalovirus/congênito , Infecções por Citomegalovirus/tratamento farmacológico , Ganciclovir/uso terapêutico , Infecções por Citomegalovirus/complicações , Seguimentos , Transtornos da Audição/etiologia , Humanos , Recém-Nascido
8.
Zhongguo Dang Dai Er Ke Za Zhi ; 11(6): 476-80, 2009 Jun.
Artigo em Zh | MEDLINE | ID: mdl-19558814

RESUMO

OBJECTIVE: To evaluate the effect of clearance of superoxide anion by catechin on the expression of nitrogen monoxidum (NO) and endothelial nitricoxide synthase (eNOS) and apoptosis in endothelial progenitor cells (EPCs) induced by angiotensin II (Ang II). METHODS: The marrow endothelial progenitor cells of Sprague-Dawley rats were isolated and assigned to control (no treatment), Ang II treatment and Ang II + catechin treatment groups. After 48 hrs of culture, the concentration of O2*- in the supernate was measured by the NBT method, and NO concentration in the supernate was measured by the nitrate reductase method; the apoptosis rate of EPCs was detected by the TUNEL method; the mRNA expression of eNOS was detected by RT-PCR; the protein expression of eNOS was detected by Western blot analysis. RESULTS: Ang II of 10-6 mol/L was determined as the suitable concentration for cell induction by the MTT test. Catechin of 400 mg/L was determined as an advisable intervention dosage. The apoptosis rate of EPCs in the control, the Ang II and the Ang II+catechin treatment groups were 2.48+/-0.12%, 54.18+/-0.77% and 16.87+/-0.35%, respectively, and there were significant differences among the three groups (P<0.01). The O2*- concentration in the Ang II and the Ang II+catechin treatment groups (81.7+/- 3.6 and 62.3+/- 2.2 U/L respectively) was significantly higher than that in the control group (33.7+/- 2.8 U/L) (P<0.01). An increased NO concentration was also found in the Ang II (189. 8+/- 9.0 micromol/L) and the Ang II+catechin treatment groups (276.4+/- 10.1 micromol/L) compared with that in the control group (105.8+/- 9.8 micromol/L) (P<0.01). There were significant differences in the concentrations of O2*- and NO between the Ang II and the Ang II+catechin treatment groups (P<0.05). The mRNA (P<0.05) and protein expression (P<0.01) of eNOS in the Ang II and the Ang II+catechin treatment groups increased significantly compared with those in the control group. The Ang II+catechin treatment group showed increased eNOS protein expression compared with the Ang II group (P<0.05). CONCLUSIONS: Ang II may induce the generation of O2*-, inactivate NO and increase gene and protein expression of eNOS in EPCs. Catechin might decrease the apoptosis of EPCs through the effective clearance of O2*-and the reduction of NO inactivation and of eNOS protein uncoupling.


Assuntos
Angiotensina II/farmacologia , Apoptose/efeitos dos fármacos , Catequina/farmacologia , Células Endoteliais/efeitos dos fármacos , Óxido Nítrico Sintase Tipo III/genética , Óxido Nítrico/biossíntese , Células-Tronco/efeitos dos fármacos , Superóxidos/metabolismo , Animais , Sobrevivência Celular/efeitos dos fármacos , Células Endoteliais/metabolismo , Feminino , Óxido Nítrico Sintase Tipo III/análise , RNA Mensageiro/análise , Ratos , Ratos Sprague-Dawley , Células-Tronco/metabolismo
9.
Zhongguo Dang Dai Er Ke Za Zhi ; 11(9): 717-21, 2009 Sep.
Artigo em Zh | MEDLINE | ID: mdl-19755018

RESUMO

OBJECTIVE: To investigate the relationship between vascular endothelial growth factor (VEGF) expression and microvessel injury of renal interstitium in children with Henoch-Schönlein purpura nephritis (HSPN). METHODS: Thirty-two children with HSPN and who had not received glucocorticoid or immunodepressants treatment before hospitalization were enrolled. Five children undergoing nephrectomy due to renal trauma were used as the control group. Renal samples were stained by hematoxylin and eosin and renal pathological changes were evaluated semi-quantitatively. CD34 and VEGF expression was detected by immunohistochemistry. CD34 was used as the marker for endothelial cells of renal microvessels. The microvessel density (MVD) was calculated by CD34 immunostaining. RESULTS: Compared with the control and the renal pathological grade II HSPN groups, MVD in the grade III and above HSPN groups decreased significantly, with an obvious reduction in MVD with the increased renal pathological grade (p<0.05). The renal microvessel score in the grades IIIa, IIIb, IV, and V HSPN groups decreased obviously compared with that in the control group. The renal microvessel score decreased with the increased renal pathological grade (p<0.05). VEGF expression in the grade II HSPN group was higher (p<0.05), while that in the grades IV and V HSPN group was lower than that in the control group (p<0.05). VEGF expression in the HSPN group showed a significant reduction with the increased renal pathological grade (p<0.05). There was a positive correlation between VEGF expression and MVD in renal tissue in the HSPN group (r=0.935, p<0.01). CONCLUSIONS: The decreased expression of VEGF may be responsible for the renal pathological damage and microvessel injury in HSPN.


Assuntos
Vasculite por IgA/patologia , Rim/irrigação sanguínea , Fator A de Crescimento do Endotélio Vascular/análise , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Vasculite por IgA/metabolismo , Imuno-Histoquímica , Rim/química , Masculino , Microvasos/patologia , Nefrite
10.
Zhongguo Dang Dai Er Ke Za Zhi ; 11(1): 61-4, 2009 Jan.
Artigo em Zh | MEDLINE | ID: mdl-19149926

RESUMO

OBJECTIVE: To study the effect of H2O2 on the proliferation and apoptosis of endothelial progenitor cells (EPCs) and the antogonistic effects of catechin on the cell apoptosis induced by H2O2 in rats. METHODS: Immuno-fluoreascence assay was applied to detect CD34, CD133 and VEGFR-2 expression. EPCs of generation 2 were divided into control cells, H2O2-treated cells and catechin-H2O2-treated cells (H2O2: 100 mg/L; catechin: 10 mg/L). Genomic DNA was extracted by the conventional method after intervention for the analysis of apoptosis ladder pattern. The MTT assay was applied to detect proliferation rate of EPCs. RESULTS: The cultured cells at day 10 expressed CD34, CD133 and VEGFR-2. DNA apoptosis ladder pattern appeared in H2O2-treated cells 2 days after intervention. After 3 days of intervention DNA apoptosis ladder pattern appeared in both H2O2-treated cells and H2O2-catechinjtreated cells, with more ladders and grayer scale in H2O2 -treated cells. Compared with the controls, H2O2-treated cells and H2O2-catechin-treated cells showed significantly decreased proliferation rate (p<0.01), with the lowest proliferation rate at the 2nd day (p<0.05). The H2O2-catechin-treated cells showed increased proliferation rate than H2O2-treated cells at the 1st, 2nd and 3rd days. CONCLUSIONS: H2O2 may impair EPCs proliferation and induce EPCs apoptosis. Catechin may increase the capacity of EPCs for the resistance to apoptosis induced by H2O2.


Assuntos
Apoptose/efeitos dos fármacos , Catequina/farmacologia , Células Endoteliais/efeitos dos fármacos , Peróxido de Hidrogênio/toxicidade , Células-Tronco/efeitos dos fármacos , Antígeno AC133 , Animais , Antígenos CD/análise , Antígenos CD34/análise , Proliferação de Células/efeitos dos fármacos , Células Endoteliais/citologia , Feminino , Glicoproteínas/análise , Peptídeos/análise , Ratos , Ratos Sprague-Dawley , Células-Tronco/citologia , Receptor 2 de Fatores de Crescimento do Endotélio Vascular/análise
11.
World J Pediatr ; 15(3): 262-269, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30864060

RESUMO

BACKGROUND: Mizoribine (MZR) is an immunosuppressant used to treat adult nephropathy. There is little experience with the drug in treating Chinese children with frequently relapsing nephrotic syndrome (FRNS). We investigated the efficacy and safety for treating MZR with FRNS. Furthermore, the relationship between efficacy and serum concentration was investigated. METHODS: A prospective multicenter observational 12-month study was performed for evaluating the usefulness of MZR with FRNS. Serum MZR concentration was measured, and the relationships between pharmacokinetic parameters (Cmax, AUC), number of relapses, and urinary protein were evaluated. RESULTS: Eighty-two pediatric patients from four hospitals were treated with MZR and prednisone. MZR treatment significantly reduced the number of relapses and steroid doses. A correlation between pharmacokinetic parameters and relapses was observed, which fits well with the sigmoidal Emax model. Even in the relationship between pharmacokinetic parameters and urinary proteins, it was recognized that there was a threshold in the pharmacokinetic parameters for the therapeutic effect similar to the results obtained with the sigmoidal Emax model. Eleven patients (13.4%) experienced mild adverse events. CONCLUSIONS: MZR therapy was effective in reducing the number of relapses and steroid doses. No severe adverse reactions were observed. Therapeutically effective serum concentrations were estimated to be Cmax ≥ about 2 µg/mL or AUC ≥ about 10 µg h/mL. MZR and steroid treatment were effective and safe for pediatric FRNS.


Assuntos
Imunossupressores/farmacocinética , Imunossupressores/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , Ribonucleosídeos/farmacocinética , Ribonucleosídeos/uso terapêutico , Adolescente , Criança , Pré-Escolar , China , Feminino , Humanos , Masculino , Estudos Prospectivos , Recidiva
12.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 33(3): 227-32, 2008 Mar.
Artigo em Zh | MEDLINE | ID: mdl-18382057

RESUMO

OBJECTIVE: To explore the relationship between pathological features and clinical manifestations in children with nephropathy under 6 years old. METHODS: Renal biopsy by rapid percutaneous puncturation was performed on 313 children under 6 who were all diagnosed clinically as kidney diseases of 14 different kinds. The specimens were divided into 3 parts for microscope, electron microscope and immuno fluorescence examination respectively and processed by HE, PAS, PASM, and Masson staining. Immunofluorescence was used to detect the deposition of IgG, IgM, IgA, C3, C4, C1q, and Fb in the renal tissues. Additional examinations were done to detect HBs-Ag, HBeAg and HBcAg deposition in some cases with positive serum HBs-Ag. Altogether 290 of the specimens (290/313, 92.65%) were examined by electron microscope. RESULTS: All the renal biopsy performances were successful. The clinical manifestations comprised of persistent haematuria (32.92%, 103/313), idiopathic nephritic syndrome (26.1%, 82/313), acute nephritic syndrome (20.14%, 63/313), Henoch Schonlein purpura nephritis (8.32%, 26/313), HBV-nephritis (4.79%, 15/313), and isolated proteinuria (2.56%, 8/313). The main pathological patterns of glomerular disease were identified as mesangial proliferation (51.75%, 162/313), IgM nephropathy (8.31%,26/313), minor and minimal change (7.99%, 25/313), IgA nephropathy (7.35%, 23/313), endocapillary proliferative glomerulonephritis (5.11%, 16/313), focus segmental glomerulosclerosis (4.47%, 14/313), thin basement membrane nephropathy (4.47%, 14/313), and membrane nephropathy (4.47%, 14/313). Alport syndrome, congenital nephrotic syndrome, and thin basement membrane nephropathy can be diagnosed by electron microscope, white IgA nephropathy, IgM nephropathy and C1q nephropathy by immunopathology. CONCLUSION: Similar clinical manifestations may differ in the pathology and the clinical features of one pathological diagnosis may vary greatly. Renal biopsy is of great help to the diagnosis, treatment and the prognosis evaluation for children with nephropathy under 6. Electron microscopes also play an important role in the diagnosis of nephropathy.


Assuntos
Nefropatias/patologia , Rim/ultraestrutura , Biópsia por Agulha , Criança , Pré-Escolar , Glomerulonefrite/diagnóstico , Glomerulonefrite/patologia , Humanos , Lactente , Rim/patologia , Nefropatias/diagnóstico , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/patologia
14.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 32(6): 938-40, 2007 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-18182707

RESUMO

OBJECTIVE: To investigate the efficacy and adverse effect of mycophenolate mofetil (MMF) in the treatment of frequently relapsing nephrotic syndrome in children. METHODS: The study population consisted of 37 children (24 simple nephrotic syndrome and 13 nephritis-type syndrome) suffering from frequently relapsing nephrotic syndrome. Patients received 20-30 mg/(kg d) of MMF in conjunction with 1 mg/(kg d) prednisone for 3-6 months. RESULTS: Out of 24 patients suffered from simple nephrotic syndrome, 17 patients (70.8%) with complete relief, 4 patients (16.7%) with partial relief and 3 patients (12.5%) with non-relief, whereas out of 13 patients suffered from nephritis-type syndrome 6 patients (46.2%) with complete relief, 3 patients (23.1%) with partial relief and 4 patients (30.7%) with non-relief. Eight patients with Minimal Change Disease (MCD) achieved complete relief. Of 23 patients with Mesangial Proliferative Glomerulonephritis (MsPGN) or Membranoproliferative Glomerulonephritis (MPGN), complete relief was observed in 17 patients (73.9%), partial relief in 4 patients (17.4%) and non-relief in 2 patients. CONCLUSION: These Results suggest that MMF has better efficacy against simple renal disease than against nephritis-type syndrome, and MMF may be more suitable for the treatment of frequently relapsing nephrotic syndrome characterized by proliferative lesions.


Assuntos
Ácido Micofenólico/análogos & derivados , Síndrome Nefrótica/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Masculino , Ácido Micofenólico/efeitos adversos , Ácido Micofenólico/uso terapêutico , Recidiva , Resultado do Tratamento
15.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 32(6): 964-72, 2007 Dec.
Artigo em Zh | MEDLINE | ID: mdl-18182711

RESUMO

OBJECTIVE: To evaluate the benefits and toxicities of different corticosteroid regimes in preventing relapse in children with steroid sensitive nephrotic syndrome (SSNS). METHODS: MEDLINE (Jan. 1963-Mar. 2007), elsevier (Jan. 1997-Aug. 2006), OVID databank (Jan. 1993-Aug. 2006), Springer databank (Jan. 1994-March 2007), the Cochrane Controlled Trials Register (Cochrane Library, Issue Feb. 2006), Cochrane Renal Group Specialised Register (Jul. 2006), EMBASE (Jan. 1980-Mar. 2007) and CNKI (Jan. 1994-Mar. 2007) etc, were searched by the terms primary nephrotic syndrome, glucocorticoid, corticosteroid, steroid, prednisone, methylprednisolone, dexamethasone and children etc for the human clinical trials about glucocorticoid (GC) administration in primary nephrotic syndrome (PNS) (aged 3 months to 18 years), controlled or semi-controlled ones, including unpublished documents from scientific meetings and theses, and similar documents listed in the references of the above documents were also included. All the studies were evaluated strictly according to Jadad Standard, and the Meta-analysis were adopted. Review manager 4.2 software was used to analyze the data. The odds ratio was calculated for the relapse rate and side effect from the initial episode to the end of follow-up between the patients treated with corticosteroids and the controls. RESULTS: Totally 12 trials with 868 subjects meeting the criteria were included in this review. A Meta-analysis of 7 trials, which compared between 2 months of prednisone and 3 months or more in the first episode, showed that longer treatment duration significantly reduced the risk of relapse at 12-24 months (RR=0.70,95% CI:0.60-0.89),without an increase of side effect. There was a negative linear relationship between the duration of treatment and risk of relapse (r2 =0.66, P=0.05). CONCLUSION: (1) Children in their first episode of SSNS should be treated for at least 3 months of GC. The therapeutic effect of patients in the primary nephrotic syndrome treated with GC for 12 weeks was prior to that for 8 weeks, compared with that in the controls. It could reduce the relapse rate of half year, the longer treatment duration in the NS patients at the first relapse was, the lower relapse risk was.(2) Compared with alternative GC administration, standard GC administration can reduce the side effects; Course more than 1 year of GC administration can reduce the 2-year relapse rate. Hence in children who relapse frequently, multicentre, well-designed experiments should be adopted.


Assuntos
Glucocorticoides/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , Criança , Resistência a Medicamentos , Glucocorticoides/farmacologia , Humanos
16.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 32(1): 82-7, 2007 Feb.
Artigo em Zh | MEDLINE | ID: mdl-17344593

RESUMO

OBJECTIVE: To explore the effect and possible mechanism of catechin microcapsulation on the repair of DNA damage in glumreular mesangial cells (GMCs) induced by H2O2. METHODS: According to H2O2 concentration, the experiment GMCs were divided into 6 groups: a control group, 50 micromol/L group, 100 micromol/L group, 150 micromol/L group, 200 micromol/L group and 250 micromol/L group. Each group was sub-divided into 3 groups: 6 h group, 12 h group and 24 h group, in order to determining the optimum dose and the best time of detecting the DNA damage in GMCs. The cultured cells were divided into 8 groups as follows: the NS control group, the H2O2 group, the catechin groups (the final concentrations were 10.0, 15.0, and 20.0 mg/L respectively) and the various catechin microcapsulation groups (the final concentrations were 10.0, 15.0, and 20.0 mg/L respectively). At the end of the experiment, hydroxy radical (OH), malonydialdehyde (MDA) and total superoxide dismutase (tSOD) concentration of supernadant in GMCs were determined by biochemistry assay, the repair of DNA damage in GMCs were detected by single cell gel electrophoresis assay. RESULTS: (1)At 6th h, H2O2 of 100 micromoL/L could cause the DNA damage of GMCs, and H2O2 of 150 micromol/L could result in DNA damage significantly. (2) No difference was found in the comet span of GMCs DNA in the catechin group and catechin microcapsulation group of different concentrations, while the DNA comet tail-long in the catechin microcapsulation group was shorter than that of the catechin group(all P(s)<0.05), and the fluorescence intensity of tail in the catechin microcapsulation group was lower than that of the catechin group(all P(s)<0.01). (3)When the concentration of catechin was 10.0 mg/L, no statistical significance was obtained in the concentration of dOH-, MDA and tSOD between the catechin microcapsulation group and the catechin group; while dOH- and MDA concentrations were lower, and the tSOD was higher in the catechin microcapsulation group than that in the catechin group when the concentration of catechin was 15.0 mg/L and 20.0 mg/L(all P(s)<0.05). CONCLUSION: Catechin microcapsulation can enhance the GMCs ability of repairing DNA damage,which may be due to elevating the capacity of its anti-oxidation by catechin microcapsulation.


Assuntos
Catequina/farmacologia , Dano ao DNA/efeitos dos fármacos , Reparo do DNA/efeitos dos fármacos , Peróxido de Hidrogênio/toxicidade , Células Mesangiais/efeitos dos fármacos , Animais , Cápsulas , Células Cultivadas , Ensaio Cometa , Relação Dose-Resposta a Droga , Radical Hidroxila/metabolismo , Malondialdeído/metabolismo , Células Mesangiais/metabolismo , Células Mesangiais/patologia , Ratos , Superóxido Dismutase/metabolismo
17.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 32(1): 174-8, 2007 Feb.
Artigo em Zh | MEDLINE | ID: mdl-17344613

RESUMO

OBJECTIVE: To evaluate the clinical and pathological features of 94 children suffering from IgA nephropathy (IgAN) while estimating the prevalent situation in Hunan province. METHODS: To summarize the annual number of hospitalized children, those with kidney diseases, those accepted biopsy, and those confirmed as IgAN in both Xiangya Hospital and Second Xiangya Hospital undertaking kidney biopsy in Hunan province during 1995 and 2004. RESULTS: In the past 10 years, as the hospitalized population in both hospitals accrued to 9.98% each year. The rate of 7.5% was seen in those with kidney diseases. Among whom 56.3% accepted kidney biopsy and 94 of them were confirmed as IgAN. Hematuria was the main clinical presentation, seen in 71 cases, accounting to 76%, and even to 98% after excluding those with nephrotic syndrome and isolating proteinuria type of IgAN. Inflammation infiltration (91%), renal tubule degeneration (81%), and renal interstitial fibrosis (31%) were the major pathological features of 94 children, especially in nephrotic syndrome IgAN. CONCLUSION: The number of children with IgAN synchronously accrues as hospitalized population, those with kidney diseases, and those by kidney biopsy. Hematuria is the major symptom. To routinely perform urine analysis and kidney biopsy in asymptomatic hematuria may improve the diagnosis. Inflammation infiltration, renal tubule degeneration, and renal interstitial fibrosis are the major pathological features in IgAN children, especially in nephrotic syndrome IgAN, probably relating to continuous proteinuria. Early control of proteinuria may delay or decrease renal tubule fibrosis.


Assuntos
Glomerulonefrite por IGA/patologia , Rim/patologia , Adolescente , Biópsia por Agulha , Criança , Pré-Escolar , China/epidemiologia , Feminino , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/epidemiologia , Hematúria/diagnóstico , Hematúria/etiologia , Hospitalização/estatística & dados numéricos , Humanos , Masculino
19.
Zhongguo Dang Dai Er Ke Za Zhi ; 9(2): 144-8, 2007 Apr.
Artigo em Zh | MEDLINE | ID: mdl-17448311

RESUMO

OBJECTIVE: To study the mobilization effects of stem cell factor (SCF) along with granulocyte colony-stimulating factor (G-CSF) on bone marrow stem cells and endothelial progenitor cells in rats with unilateral ureteral obstruction (UUO). METHODS: Fifty-six healthy male Wistar rats were randomly divided into seven groups: control, SCF, G-CSF, SCF+G-SCF, Sham-operated, UUO and UUO+SCF+G-CSF groups (n=8 each). The rats from the control, SCF, G-CSF and SCF+G-CSF groups were hypodermically injected with normal saline (2 mL/kg), SCF (200 microg/kg), G-CSF (200 microg/kg) and SCF along with G-CSF respectively for 5 days. The rats from the UUO and UUO+SCF+G-CSF groups were subjected to the ligation of right ureter and then were hypodermically injected with normal saline (2 mL/kg) and SCF (200 microg/kg)+G-CSF (200 microg/kg) respectively for 5 days. The sham-operated group had the same operative approach as the UUO and the UUO+SCF+G-CSF groups but the right ureter was not ligated. After operation they received a hypodermical injection of 2 mL/kg normal saline for 5 days. Five days later blood samples were collected. The percentages of CD34+ and CD34+/CD133+ cells in intravenous blood mononuclear cells were detected by flow cytometry. Serum contents of glutamate-pyruvate transaminase (GPT), glutamic oxalacetic transaminase (GOT), urea nitrogen and creatinin were measured. RESULTS: Except for the sham-operated group, the other five groups (SCF, G-CSF, SCF+G-SCF, UUO and UUO+ SCF+G-CSF groups) had significantly higher percentage of CD34+ cells and CD34+/CD133+ cells in intravenous blood mononuclear cells than the control group (P < 0.05). There were significant differences in the percentage of CD34+ cells and CD34+/CD133+ cells among the five groups (P < 0.05). The UUO+SCF+G-CSF group showed the highest percentage of CD34+ cells and CD34+/CD133+ cells, followed by the SCF+G-CSF group. There were no significant differences in serum contents of GPT, urea nitrogen and creatinin among the seven groups. Except the UUO group showed higher GOT contents, there were no significant differences in the GOT contents among the other six groups. CONCLUSIONS: The mobilization effects of SCF and G-CSF on bone marrow stem cells and endothelial progenitor cells were not always in paraller. A combination of SCF and G-CSF can effectively mobilize stem cells and endothelial progenitor cells, and side effects were not found in the liver and the kidney.


Assuntos
Células da Medula Óssea/efeitos dos fármacos , Células Endoteliais/efeitos dos fármacos , Fator Estimulador de Colônias de Granulócitos/farmacologia , Mobilização de Células-Tronco Hematopoéticas , Fator de Células-Tronco/farmacologia , Obstrução Ureteral/fisiopatologia , Antígeno AC133 , Animais , Antígenos CD/análise , Antígenos CD34/análise , Células da Medula Óssea/citologia , Células Endoteliais/citologia , Glicoproteínas/análise , Masculino , Peptídeos/análise , Ratos , Ratos Wistar , Proteínas Recombinantes
20.
Zhongguo Dang Dai Er Ke Za Zhi ; 9(2): 117-21, 2007 Apr.
Artigo em Zh | MEDLINE | ID: mdl-17448305

RESUMO

OBJECTIVE: To investigate the clinicopathologic characteristics of childhood renal diseases. METHODS: A retrospective analysis of 1316 renal biopsies performed over the past 20 years was performed. RESULTS: Of the 1316 patients, 383 (29.09% ) were diagnosed as nephrotic syndrome, 291 (22.00%) as acute nephritis syndrome, 224 (17.21%) as isolated hematuria, 209(15.87%) as purpura nephritis, and 96 (7.30% ) as hepatitis B virus-associated nephritis . Mesangial proliferation was the most common pathological change (756 cases; 57.45%), followed by IgA nephropathy (113 cases; 8.59%), endothelial capillary proliferation(112 cases; 8.51%), membranous nephropathy (66 cases; 5.02%), and various minor and minimal changes (59 cases; 4.48%). Alport syndrome, congenital nephrotic syndrome, thin basement membrane nephropathy, fibrillary glomerulopathy disease, and Fabry disease were confirmed by electronic microscopy. IgA, IgM and C1q nephropathy were definitely diagnosed using immune histochemistry or immunofluorescent. A diagnosis of primary glomerular disease was made in 69.53% of the cases (915 cases); secondary glomerular disease was noted in 26.14% (344 cases). Of the 915 cases of primary glomerular disease, 375 (41.0%) had nephrotic syndrome. Secondary glomerular disease due to purura nephritis was common (209/344; 60.8%). CONCLUSIONS: Primiary glomerular disease predominates in children. Nephrotic syndrome is the most common clinical diagnosis. Mesangial proliferation is the most common pathological patterns in children with renal disease.


Assuntos
Nefropatias/patologia , Rim/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Glomérulos Renais/patologia , Masculino , Insuficiência Renal/patologia , Estudos Retrospectivos
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