AIM: To investigate the efficacy of angiotensinⅡreceptor antagonist on hepatic stellate cells (HSCs) activation in the patients with non-alcoholic steatohepatitis (NASH). METHODS: Seven patients with NASH were prescr...AIM: To investigate the efficacy of angiotensinⅡreceptor antagonist on hepatic stellate cells (HSCs) activation in the patients with non-alcoholic steatohepatitis (NASH). METHODS: Seven patients with NASH were prescribed losartan, a selective angiotensinⅡtype 1 receptor antagonist (50 mg/d) for 48 wk. Liver biopsies were performed both at the entry and end of the study in all patients. Quiescent and activated HSCs were identified by double immunostaining using anti-p75 andα-smooth muscle actin antibodies, and the number of each phenotype was counted. Similarly, the liver specimens obtained from the eight patients with non-alcoholic fatty liver (NAFL) were also examined as controls. RESULTS: In NASH hepatic tissues, activated HSCs were dominantly distributed as compared with those in NAFL. The 48-wk losartan treatment induced a remarkable decrease in activated HSCs and a mild increase in quiescent phenotypes. CONCLUSION: Our data suggest the crucial involvement of HSCs in anti-fibrotic effect of angiotensinⅡreceptor antagonist on patients with NASH.展开更多
AIM:To study possible decrease in prevalence of Helicobacter pylori(H.pylori) infection in the Czech Republic within a 10-year period.METHODS:A total of 22 centres entered the study.The catchment areas of these centre...AIM:To study possible decrease in prevalence of Helicobacter pylori(H.pylori) infection in the Czech Republic within a 10-year period.METHODS:A total of 22 centres entered the study.The catchment areas of these centres covered cities and towns with more than 20 000 inhabitants,smaller towns(≤ 20 000 inhabitants) with surrounding villages and rural areas,and were spread over the whole country,corresponding well to the geographical distribution of the Czech population.A total of 1 837 subjects(aged 5-98 years) took part in the study,randomly selected out of 38 147 people from the general population.H.pylori infection was investigated by means of a 13 C-urea breath test.Breath samples in duplicates were analysed using isotope ratio mass spectrometry.The cut-off point was 3.5.Social and demographic characteristics were based on data from self-completed questionnaires.RESULTS:The overall prevalence of H.pylori infection was 23.5%(430/1826),and 4.8%(20/420) in children aged 15 or less.There was no statistically significant difference in prevalence between males(24.3%;208/857) and females(22.9%,222/969,P = 0.494).H.pylori infection was strongly associated with higher age,among subjects aged 55+ years,prevalence of H.pylori infection was 39.8%(252/633,P < 0.001).The highest prevalence of H.pylori infection was found among persons aged 55-64 years(43.9%,97/221) and 75+ years(37.9%,58/153).Among study subjects aged 15+ years,prevalence of H.pylori infection was significantly increased in those with lowest education(odds risk 3.19,95% CI 1.87-5.47).Compared to never married(14.1%),the prevalence of H.pylori infection was statistically significantly higher among married(35.4%,246/694,P < 0.001),divorced(36.8%,49/133,P < 0.001) and widowed study subjects(40.2%,45/112,P < 0.001),both in minimally and fully adjusted analysis.There was no significant difference in the prevalence of H.pylori infection between married and widowed subjects(35.4%,246/694 vs 40.2%,45/112,P = 0.389).There was little variation in smoking prevalence across categories of smoking and there was no evidence of an increased risk ofH.pylori infection among current or past smokers in our data(odds risk 1.04 with 95% CI 0.78-1.40 for current smokers;odds ratio 0.83 with 95% CI 0.60-1.16 for former smokers).The current prevalence of H.pylori in 2011 was significantly lower compared to the prevalence reported from identical geographical areas in 2001(23.5%vs 41.7%,P < 0.001).CONCLUSION:The overall prevalence of H.pylori infection in the general population has fallen substantially in the Czech Republic over the past 10 years.展开更多
AIM:To investigate intestinal alkaline phosphatase(iAP) in the intestinal mucosa of children with inflammatory bowel disease(IBD).METHODS:Colonic biopsy samples were taken from 15 newly diagnosed IBD patients and from...AIM:To investigate intestinal alkaline phosphatase(iAP) in the intestinal mucosa of children with inflammatory bowel disease(IBD).METHODS:Colonic biopsy samples were taken from 15 newly diagnosed IBD patients and from 10 healthy controls.In IBD patients,specimens were obtainedboth from inflamed and non-inflamed areas.The iAP mRNA and protein expression was determined by reverse transcription-polymerase chain reaction and Western blotting analysis,respectively.Tissue localization of iAP and Toll-like receptor(TLR) 4 was investigated by immunofluorescent staining.RESULTS:The iAP protein level in the inflamed mucosa of children with Crohn's disease(CD) and ulcerative colitis(UC) was significantly decreased when compared with controls(both P < 0.05).Similarly,we found a significantly decreased level of iAP protein in the inflamed mucosa in CD compared with non-inflamed mucosa in CD(P < 0.05).In addition,the iAP protein level in inflamed colonic mucosa in patients with UC was decreased compared with non-inflamed mucosa in patients with CD(P < 0.05).iAP protein levels in the non-inflamed mucosa of patients with CD were similar to controls.iAP mRNA expression in inflamed colonic mucosa of children with CD and UC was not significantly different from that in non-inflamed colonic mucosa with CD.Expression of iAP mRNA in patients with noninflamed mucosa and in controls were similar.Co-localization of iAP with TLR4 showed intense staining with a dotted-like pattern.iAP was present in the inflamed and non-inflamed mucosa of patients with CD,UC,and in control biopsy specimens,irrespective of whether it was present in the terminal ileum or in the colon.However,the fluorescent signal of TLR4 was more pronounced in the colon compared with the terminal ileum in all groups studied.CONCLUSION:Lower than normal iAP protein levels in inflamed mucosa of IBD patients may indicate a role for iAP in inflammatory lesions in IBD.Based on our results,administration of exogenous iAP enzyme to patients with the active form of IBD may be a therapeutic option.展开更多
AIM: To investigate relapse predictors in chronic hepatitis C (CHC) patients with end-of-treatment response (ETR), after pegylated interferon-α (PegIFN-α) and ribavirin treatment. METHODS: In a retrospective study w...AIM: To investigate relapse predictors in chronic hepatitis C (CHC) patients with end-of-treatment response (ETR), after pegylated interferon-α (PegIFN-α) and ribavirin treatment. METHODS: In a retrospective study we evaluated a spectrum of predictors of relapse after PegIFN-α and ribavirin treatment in 86 CHC patients with ETR. Viral loads were determined with real-time reverse transcrip-tion polymerase chain reaction. Hepatitis C virus geno-typing was performed by sequencing analysis. Patients with genotype 1 were treated for 48 wk with 180 μg PegIFN-α2a or 1.5 μg/kg PegIFN-α2b once weekly plus ribavirin at a dosage of 1000 mg/d for those under 75 kg or 1200 mg/d for those over 75 kg. Patients with geno- types 2 and 3 were treated for 24 wk with 180 μgPegIFN-α2a or 1.5 μg/kg PegIFN-α2b once weekly plus ribavirin at a dosage of 800 mg/d. RESULTS: In all ETR patients, binary logistic regression analysis identif ied absence of complete early virological response (cEVR) (OR 27.07, 95% CI: 3.09-237.26, P < 0.005), serum alkaline phosphatase (ALP) levels prior to therapy < 75 U/L (OR: 6.16, 95% CI: 2.1-18.03, P < 0.001) and body mass index > 26 kg/m2 (OR: 8.27, 95% CI: 2.22-30.84, P < 0.005) as independent predictors of relapse. When cEVR patients were analyzed exclusively, ALP prior to therapy < 75 U/L remained the only predictor of relapse. CONCLUSION: Lower levels of ALP prior to, during and after therapy seem to be associated with a higher risk of relapse in CHC patients with ETR.展开更多
Traumatic brain injury causes gene expression changes in different brain regions. Occurrence and development of traumatic brain injury are closely related, involving expression of three factors, namely cyclooxygenase-...Traumatic brain injury causes gene expression changes in different brain regions. Occurrence and development of traumatic brain injury are closely related, involving expression of three factors, namely cyclooxygenase-2, glutamate receptor-2, and platelet activating factor receptor. However, little is known about the correlation of these three factors and brain neuronal injury. In this study, primary cultured rat hippocampal neurons were subjected to fluid percussion injury according to Scott's method, with some modifications. RT-PCR and semi-quantitative immunocytochemical staining was used to measure the expression levels of cyclooxygenase-2, glutamate receptor-2, and platelet activating factor receptor. Our results found that cyclooxygenase-2 expression were firstly increased post-injury, and then decreased. Both mRNA and protein expression levels reached peaks at 8 and 12 hours post-injury, respectively. Similar sequential changes in glutamate receptor 2 were observed, with highest levels mRNA and protein expression at 8 and 12 hours post-injury respectively. On the contrary, the expressions of platelet activating factor receptor were firstly decreased post-injury, and then increased. Both mRNA and protein expression levels reached the lowest levels at 8 and 12 hours post-injury, respectively. Totally, our findings suggest that these three factors are involved in occurrence and development of hippocampal neuronal injury.展开更多
A 44-year-old woman developed jaundice and was diagnosed as stage II of primary biliary cirrhosis (PBC). She showed a severely high total cholesterol level. This article focuses on atypical presentations of PBC and th...A 44-year-old woman developed jaundice and was diagnosed as stage II of primary biliary cirrhosis (PBC). She showed a severely high total cholesterol level. This article focuses on atypical presentations of PBC and the need to test the total cholesterol level of PBC patients.展开更多
慢性 B 细胞淋巴细胞白血病(CLL)用常规化疗不能治愈。虽然其常呈慢性过程,但对那些白细胞计数高、骨髓广泛浸润、淋巴细胞倍增时短和晚期患者,生存时间明显缩短。故很有必要寻找出能使其长期缓解或根治的治疗措施。初步研究结果提示,...慢性 B 细胞淋巴细胞白血病(CLL)用常规化疗不能治愈。虽然其常呈慢性过程,但对那些白细胞计数高、骨髓广泛浸润、淋巴细胞倍增时短和晚期患者,生存时间明显缩短。故很有必要寻找出能使其长期缓解或根治的治疗措施。初步研究结果提示,去髓性放疗和化疗后自体骨髓移植(ABMT)有可能将其治愈。但因自体骨髓中常有大量的白血病细胞污染。展开更多
Inflammatory bowel disease (IBD) is a chronic inflammatory state associated with increased risk of intestinal cancers. The aim of this study is to examine serum concentrations of growth-related oncogene (GRO)α , a cy...Inflammatory bowel disease (IBD) is a chronic inflammatory state associated with increased risk of intestinal cancers. The aim of this study is to examine serum concentrations of growth-related oncogene (GRO)α , a cytokine with inflammatory and growth-regulatory properties, in patients with IBD. We measured serum concentrations of GRO-α in 60 patients with ulcerative colitis, 42 patients with Crohn’ s disease, 16 patients with other colitides, 12 patients with colorectal cancer, and 40 normal subjects using an enzyme-linked immunosorbent assay. We then analyzed how the cytokine was related to clinical and laboratory variables. Serum GRO-α concentrations in patients with active IBD were significantly higher than those in patients with quiescent disease, which in turn were higher than those in normal controls. Concentrations in patients with active ulcerative colitis were higher than in patients with active Crohn’ s disease. Analysis of paired serum samples showed a decrease in GRO-α after initiation of therapy. Furthermore, serum GRO-α correlated well with laboratory markers of IBD activity. We conclude that GRO-α may have an important role in development of IBD, and might itself be used as a marker of activity. Manipulation of GRO-α function might prove therapeutically useful.展开更多
Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN...Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.展开更多
基金Supported by a Grant-in-Aid for Scientific Research (C) from the Japan Society for the Promotion of Science, No. 15590613,and a grant for Research on Intractable Disease from the Japanese Ministry of Public Welfare
文摘AIM: To investigate the efficacy of angiotensinⅡreceptor antagonist on hepatic stellate cells (HSCs) activation in the patients with non-alcoholic steatohepatitis (NASH). METHODS: Seven patients with NASH were prescribed losartan, a selective angiotensinⅡtype 1 receptor antagonist (50 mg/d) for 48 wk. Liver biopsies were performed both at the entry and end of the study in all patients. Quiescent and activated HSCs were identified by double immunostaining using anti-p75 andα-smooth muscle actin antibodies, and the number of each phenotype was counted. Similarly, the liver specimens obtained from the eight patients with non-alcoholic fatty liver (NAFL) were also examined as controls. RESULTS: In NASH hepatic tissues, activated HSCs were dominantly distributed as compared with those in NAFL. The 48-wk losartan treatment induced a remarkable decrease in activated HSCs and a mild increase in quiescent phenotypes. CONCLUSION: Our data suggest the crucial involvement of HSCs in anti-fibrotic effect of angiotensinⅡreceptor antagonist on patients with NASH.
基金Supported by Research Project PRVOUK P37-08 from Faculty of Medicine at Hradec Králové,Charles University in Praha,Czech Republic
文摘AIM:To study possible decrease in prevalence of Helicobacter pylori(H.pylori) infection in the Czech Republic within a 10-year period.METHODS:A total of 22 centres entered the study.The catchment areas of these centres covered cities and towns with more than 20 000 inhabitants,smaller towns(≤ 20 000 inhabitants) with surrounding villages and rural areas,and were spread over the whole country,corresponding well to the geographical distribution of the Czech population.A total of 1 837 subjects(aged 5-98 years) took part in the study,randomly selected out of 38 147 people from the general population.H.pylori infection was investigated by means of a 13 C-urea breath test.Breath samples in duplicates were analysed using isotope ratio mass spectrometry.The cut-off point was 3.5.Social and demographic characteristics were based on data from self-completed questionnaires.RESULTS:The overall prevalence of H.pylori infection was 23.5%(430/1826),and 4.8%(20/420) in children aged 15 or less.There was no statistically significant difference in prevalence between males(24.3%;208/857) and females(22.9%,222/969,P = 0.494).H.pylori infection was strongly associated with higher age,among subjects aged 55+ years,prevalence of H.pylori infection was 39.8%(252/633,P < 0.001).The highest prevalence of H.pylori infection was found among persons aged 55-64 years(43.9%,97/221) and 75+ years(37.9%,58/153).Among study subjects aged 15+ years,prevalence of H.pylori infection was significantly increased in those with lowest education(odds risk 3.19,95% CI 1.87-5.47).Compared to never married(14.1%),the prevalence of H.pylori infection was statistically significantly higher among married(35.4%,246/694,P < 0.001),divorced(36.8%,49/133,P < 0.001) and widowed study subjects(40.2%,45/112,P < 0.001),both in minimally and fully adjusted analysis.There was no significant difference in the prevalence of H.pylori infection between married and widowed subjects(35.4%,246/694 vs 40.2%,45/112,P = 0.389).There was little variation in smoking prevalence across categories of smoking and there was no evidence of an increased risk ofH.pylori infection among current or past smokers in our data(odds risk 1.04 with 95% CI 0.78-1.40 for current smokers;odds ratio 0.83 with 95% CI 0.60-1.16 for former smokers).The current prevalence of H.pylori in 2011 was significantly lower compared to the prevalence reported from identical geographical areas in 2001(23.5%vs 41.7%,P < 0.001).CONCLUSION:The overall prevalence of H.pylori infection in the general population has fallen substantially in the Czech Republic over the past 10 years.
基金Supported by Grants OTKA-76316,OTKA-K81117,and ETT-028-02 (Veres G and Vannay á are holders of the János Bolyai Research grant)János Bolyai Research Scholarship of the Hungarian Academy of Sciences
文摘AIM:To investigate intestinal alkaline phosphatase(iAP) in the intestinal mucosa of children with inflammatory bowel disease(IBD).METHODS:Colonic biopsy samples were taken from 15 newly diagnosed IBD patients and from 10 healthy controls.In IBD patients,specimens were obtainedboth from inflamed and non-inflamed areas.The iAP mRNA and protein expression was determined by reverse transcription-polymerase chain reaction and Western blotting analysis,respectively.Tissue localization of iAP and Toll-like receptor(TLR) 4 was investigated by immunofluorescent staining.RESULTS:The iAP protein level in the inflamed mucosa of children with Crohn's disease(CD) and ulcerative colitis(UC) was significantly decreased when compared with controls(both P < 0.05).Similarly,we found a significantly decreased level of iAP protein in the inflamed mucosa in CD compared with non-inflamed mucosa in CD(P < 0.05).In addition,the iAP protein level in inflamed colonic mucosa in patients with UC was decreased compared with non-inflamed mucosa in patients with CD(P < 0.05).iAP protein levels in the non-inflamed mucosa of patients with CD were similar to controls.iAP mRNA expression in inflamed colonic mucosa of children with CD and UC was not significantly different from that in non-inflamed colonic mucosa with CD.Expression of iAP mRNA in patients with noninflamed mucosa and in controls were similar.Co-localization of iAP with TLR4 showed intense staining with a dotted-like pattern.iAP was present in the inflamed and non-inflamed mucosa of patients with CD,UC,and in control biopsy specimens,irrespective of whether it was present in the terminal ileum or in the colon.However,the fluorescent signal of TLR4 was more pronounced in the colon compared with the terminal ileum in all groups studied.CONCLUSION:Lower than normal iAP protein levels in inflamed mucosa of IBD patients may indicate a role for iAP in inflammatory lesions in IBD.Based on our results,administration of exogenous iAP enzyme to patients with the active form of IBD may be a therapeutic option.
文摘AIM: To investigate relapse predictors in chronic hepatitis C (CHC) patients with end-of-treatment response (ETR), after pegylated interferon-α (PegIFN-α) and ribavirin treatment. METHODS: In a retrospective study we evaluated a spectrum of predictors of relapse after PegIFN-α and ribavirin treatment in 86 CHC patients with ETR. Viral loads were determined with real-time reverse transcrip-tion polymerase chain reaction. Hepatitis C virus geno-typing was performed by sequencing analysis. Patients with genotype 1 were treated for 48 wk with 180 μg PegIFN-α2a or 1.5 μg/kg PegIFN-α2b once weekly plus ribavirin at a dosage of 1000 mg/d for those under 75 kg or 1200 mg/d for those over 75 kg. Patients with geno- types 2 and 3 were treated for 24 wk with 180 μgPegIFN-α2a or 1.5 μg/kg PegIFN-α2b once weekly plus ribavirin at a dosage of 800 mg/d. RESULTS: In all ETR patients, binary logistic regression analysis identif ied absence of complete early virological response (cEVR) (OR 27.07, 95% CI: 3.09-237.26, P < 0.005), serum alkaline phosphatase (ALP) levels prior to therapy < 75 U/L (OR: 6.16, 95% CI: 2.1-18.03, P < 0.001) and body mass index > 26 kg/m2 (OR: 8.27, 95% CI: 2.22-30.84, P < 0.005) as independent predictors of relapse. When cEVR patients were analyzed exclusively, ALP prior to therapy < 75 U/L remained the only predictor of relapse. CONCLUSION: Lower levels of ALP prior to, during and after therapy seem to be associated with a higher risk of relapse in CHC patients with ETR.
基金supported by the National Natural Science Foundation of China,No.30471934
文摘Traumatic brain injury causes gene expression changes in different brain regions. Occurrence and development of traumatic brain injury are closely related, involving expression of three factors, namely cyclooxygenase-2, glutamate receptor-2, and platelet activating factor receptor. However, little is known about the correlation of these three factors and brain neuronal injury. In this study, primary cultured rat hippocampal neurons were subjected to fluid percussion injury according to Scott's method, with some modifications. RT-PCR and semi-quantitative immunocytochemical staining was used to measure the expression levels of cyclooxygenase-2, glutamate receptor-2, and platelet activating factor receptor. Our results found that cyclooxygenase-2 expression were firstly increased post-injury, and then decreased. Both mRNA and protein expression levels reached peaks at 8 and 12 hours post-injury, respectively. Similar sequential changes in glutamate receptor 2 were observed, with highest levels mRNA and protein expression at 8 and 12 hours post-injury respectively. On the contrary, the expressions of platelet activating factor receptor were firstly decreased post-injury, and then increased. Both mRNA and protein expression levels reached the lowest levels at 8 and 12 hours post-injury, respectively. Totally, our findings suggest that these three factors are involved in occurrence and development of hippocampal neuronal injury.
文摘A 44-year-old woman developed jaundice and was diagnosed as stage II of primary biliary cirrhosis (PBC). She showed a severely high total cholesterol level. This article focuses on atypical presentations of PBC and the need to test the total cholesterol level of PBC patients.
文摘慢性 B 细胞淋巴细胞白血病(CLL)用常规化疗不能治愈。虽然其常呈慢性过程,但对那些白细胞计数高、骨髓广泛浸润、淋巴细胞倍增时短和晚期患者,生存时间明显缩短。故很有必要寻找出能使其长期缓解或根治的治疗措施。初步研究结果提示,去髓性放疗和化疗后自体骨髓移植(ABMT)有可能将其治愈。但因自体骨髓中常有大量的白血病细胞污染。
文摘Inflammatory bowel disease (IBD) is a chronic inflammatory state associated with increased risk of intestinal cancers. The aim of this study is to examine serum concentrations of growth-related oncogene (GRO)α , a cytokine with inflammatory and growth-regulatory properties, in patients with IBD. We measured serum concentrations of GRO-α in 60 patients with ulcerative colitis, 42 patients with Crohn’ s disease, 16 patients with other colitides, 12 patients with colorectal cancer, and 40 normal subjects using an enzyme-linked immunosorbent assay. We then analyzed how the cytokine was related to clinical and laboratory variables. Serum GRO-α concentrations in patients with active IBD were significantly higher than those in patients with quiescent disease, which in turn were higher than those in normal controls. Concentrations in patients with active ulcerative colitis were higher than in patients with active Crohn’ s disease. Analysis of paired serum samples showed a decrease in GRO-α after initiation of therapy. Furthermore, serum GRO-α correlated well with laboratory markers of IBD activity. We conclude that GRO-α may have an important role in development of IBD, and might itself be used as a marker of activity. Manipulation of GRO-α function might prove therapeutically useful.
基金supported by grants from the 12th Five-year Plan of National Science and Technology Support(No.2013BAI02B07)Talents Support Fund for Science and Technology Innovation in Colleges and Universities of Hennan Province in 2011(No.2012HASTIT019).
文摘Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.
