Pub Date : 1900-01-01DOI: 10.3165/jjpn.oa.2019.0156
Yuji Tomii, Chisato Umeda, T. Nishino, Yoshitaka Watanabe, Koji Sakuraya, S. Fujinaga
The frequency of rituximab (RTX) use increases in complicated nephrotic syndrome (NS), raising concerns about infections associated with low IgG due to combination therapy with RTX and immunosuppressants. Between February 2008 and June 2017, we enrolled 74 children with complicated steroid-dependent NS (178 total RTX administrations, 13.8 years average patient age at time of administration) and retrospectively assessed prevalence of hypogammaglobulinemia (IgG level < 500 mg/dl) and infection. Twenty doses (11%) were associated with hypogammaglobulinemia at 6 months after RTX administration. The average age (6.1 vs 14.4 years old, p < 0.01) and serum IgG levels before administration of RTX (426 vs 656 mg/dl, p < 0.01) were significantly lower in the hypogammaglobulinemia group. Among 15 patients we were able to observe for 1 more year, 8 patients had persistent hypogammaglobulinemia, and the use of mycophenolate mofetil (MMF) was significantly higher (78 vs 17%, p < 0.05. IgG replacement therapy was performed in two patients whose IgG levels declined over time. In this study, only one varicella infection was noted. IgG replacement therapy should be considered on a case by case basis.
利妥昔单抗(RTX)在复杂肾病综合征(NS)中使用的频率增加，引起了对RTX和免疫抑制剂联合治疗与低IgG相关感染的关注。在2008年2月至2017年6月期间，我们招募了74名患有复杂类固醇依赖性NS的儿童(总共178次RTX给药，给药时患者平均年龄为13.8岁)，并回顾性评估了低γ球蛋白血症(IgG水平< 500 mg/dl)和感染的患病率。在RTX给药后6个月，20个剂量(11%)与低γ -球蛋白血症相关。低γ球蛋白血症组的平均年龄(6.1 vs 14.4岁，p < 0.01)和RTX给药前血清IgG水平(426 vs 656 mg/dl, p < 0.01)显著降低。在我们能够观察1年以上的15例患者中，8例患者存在持续性低γ -球蛋白血症，霉酚酸酯(MMF)的使用率显著高于对照组(78 vs 17%， p < 0.05)。对两名IgG水平随时间下降的患者进行IgG替代治疗。在这项研究中，只注意到一例水痘感染。IgG替代疗法应视具体情况而定。
{"title":"Hypogammaglobulinemia and infection after administration of rituximab for steroid-dependent nephrotic syndrome","authors":"Yuji Tomii, Chisato Umeda, T. Nishino, Yoshitaka Watanabe, Koji Sakuraya, S. Fujinaga","doi":"10.3165/jjpn.oa.2019.0156","DOIUrl":"https://doi.org/10.3165/jjpn.oa.2019.0156","url":null,"abstract":"The frequency of rituximab (RTX) use increases in complicated nephrotic syndrome (NS), raising concerns about infections associated with low IgG due to combination therapy with RTX and immunosuppressants. Between February 2008 and June 2017, we enrolled 74 children with complicated steroid-dependent NS (178 total RTX administrations, 13.8 years average patient age at time of administration) and retrospectively assessed prevalence of hypogammaglobulinemia (IgG level < 500 mg/dl) and infection. Twenty doses (11%) were associated with hypogammaglobulinemia at 6 months after RTX administration. The average age (6.1 vs 14.4 years old, p < 0.01) and serum IgG levels before administration of RTX (426 vs 656 mg/dl, p < 0.01) were significantly lower in the hypogammaglobulinemia group. Among 15 patients we were able to observe for 1 more year, 8 patients had persistent hypogammaglobulinemia, and the use of mycophenolate mofetil (MMF) was significantly higher (78 vs 17%, p < 0.05. IgG replacement therapy was performed in two patients whose IgG levels declined over time. In this study, only one varicella infection was noted. IgG replacement therapy should be considered on a case by case basis.","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"140 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127386721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/JJPN.OA.2020.0187
Takahiro Kirisawa, T. Uehara, Azusa Kawaguchi, Yoshinori Araki, Kazuyuki Nishinaka
in children: multicentre randomised controlled non-inferiority trial. BMJ We conducted a retrospective study of the clinical feature of neonates with multicystic dysplastic kidney (MCDK). A total of 36 patients were analyzed, of whom 6 (16.7%) had vesicoureteral reflux (VUR). Among children with VUR, high-grade vesicoureteral reflux (grades 3 to 5) was observed in 5 (83%). Initially, all children with VUR were prescribed prophylactic antibiotics. Of the five children with high-grade VUR, VUR was resolved or downgraded to grade 1 in two. The remaining children showed no improvement, so endoscopic surgery (injection of Deflux at the ureteral orifice) was performed. All cases of reflux resolved after the first injection. No significant association was observed between anomaly of the contralateral kidney and overall VUR. Furthermore, the result remained the same even when considering the grade of VUR. In the present study, no prerequisite condition concerning the omission of VCUG (voiding cystourethrography) in neonates with MCDK was detected. In cases with only one normal kidney and a risk of developing reflux nephropathy, routine VCUG for neonates with MCDK is considered beneficial.
{"title":"Examination of the necessity of voiding cystourethrography in neonates with multicystic dysplastic kidney","authors":"Takahiro Kirisawa, T. Uehara, Azusa Kawaguchi, Yoshinori Araki, Kazuyuki Nishinaka","doi":"10.3165/JJPN.OA.2020.0187","DOIUrl":"https://doi.org/10.3165/JJPN.OA.2020.0187","url":null,"abstract":"in children: multicentre randomised controlled non-inferiority trial. BMJ We conducted a retrospective study of the clinical feature of neonates with multicystic dysplastic kidney (MCDK). A total of 36 patients were analyzed, of whom 6 (16.7%) had vesicoureteral reflux (VUR). Among children with VUR, high-grade vesicoureteral reflux (grades 3 to 5) was observed in 5 (83%). Initially, all children with VUR were prescribed prophylactic antibiotics. Of the five children with high-grade VUR, VUR was resolved or downgraded to grade 1 in two. The remaining children showed no improvement, so endoscopic surgery (injection of Deflux at the ureteral orifice) was performed. All cases of reflux resolved after the first injection. No significant association was observed between anomaly of the contralateral kidney and overall VUR. Furthermore, the result remained the same even when considering the grade of VUR. In the present study, no prerequisite condition concerning the omission of VCUG (voiding cystourethrography) in neonates with MCDK was detected. In cases with only one normal kidney and a risk of developing reflux nephropathy, routine VCUG for neonates with MCDK is considered beneficial.","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130075443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/jjpn.cr.2020.0179
Hitoshi Irabu, Shuya Kaneko, N. Inoue, M. Mizuta, Kazuyuki Ueno, M. Shimizu, A. Yachie
{"title":"Clinical usefulness of rituximab for early remission in anti-neutrophil cytoplasmic antibody associated vasculitis: a case report","authors":"Hitoshi Irabu, Shuya Kaneko, N. Inoue, M. Mizuta, Kazuyuki Ueno, M. Shimizu, A. Yachie","doi":"10.3165/jjpn.cr.2020.0179","DOIUrl":"https://doi.org/10.3165/jjpn.cr.2020.0179","url":null,"abstract":"","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"BC-26 3","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"120989598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/jjpn.oa.2020.0190
Yuka Ezumi, Kazuna Yamamoto, Toshiki Masuda, S. Ichioka, T. Sakai, T. Sawai, K. Ogata, Y. Maruo
The long-term renal prognosis of Henoch-Schönlein purpura nephritis (HSPN) in children and the pathological changes before and after treatment has not yet been clarified, and the optimal treatment for HSPN remains unclear. In Japan, children with HSPN are treated according to the treatment guidelines for childhood immunoglobulin A nephropathy. Our study included 8 patients with HSPN who received multidrug combination therapy with azathioprine. All patients underwent renal biopsy at presentation, and 7 patients underwent follow-up biopsy after therapy. Four years after initiating treatment, no patients had decreased renal function compared to that pretreatment and 2 patients had proteinuria (urine protein-to-creatinine ratio: 0.15 – 0.5 g/gCr). Follow-up renal biopsy revealed chronic lesions in 3 patients, but there was no correlation between chronic lesions and remnant proteinuria. Multidrug combination therapy with azathioprine has certain therapeutic efficacy in alleviating HSPN in children.
{"title":"Four-year prognosis of multidrug combination therapy for Henoch-Schönlein purpura nephritis","authors":"Yuka Ezumi, Kazuna Yamamoto, Toshiki Masuda, S. Ichioka, T. Sakai, T. Sawai, K. Ogata, Y. Maruo","doi":"10.3165/jjpn.oa.2020.0190","DOIUrl":"https://doi.org/10.3165/jjpn.oa.2020.0190","url":null,"abstract":"The long-term renal prognosis of Henoch-Schönlein purpura nephritis (HSPN) in children and the pathological changes before and after treatment has not yet been clarified, and the optimal treatment for HSPN remains unclear. In Japan, children with HSPN are treated according to the treatment guidelines for childhood immunoglobulin A nephropathy. Our study included 8 patients with HSPN who received multidrug combination therapy with azathioprine. All patients underwent renal biopsy at presentation, and 7 patients underwent follow-up biopsy after therapy. Four years after initiating treatment, no patients had decreased renal function compared to that pretreatment and 2 patients had proteinuria (urine protein-to-creatinine ratio: 0.15 – 0.5 g/gCr). Follow-up renal biopsy revealed chronic lesions in 3 patients, but there was no correlation between chronic lesions and remnant proteinuria. Multidrug combination therapy with azathioprine has certain therapeutic efficacy in alleviating HSPN in children.","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133978776","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/jjpn.cr.2022.0208
Akinori Tabata, H. Yabe, Y. Mitake, T. Shirai, K. Kawamura
{"title":"Exercise therapy during hospitalization for a pediatric patient with active IgA nephropathy: A case report","authors":"Akinori Tabata, H. Yabe, Y. Mitake, T. Shirai, K. Kawamura","doi":"10.3165/jjpn.cr.2022.0208","DOIUrl":"https://doi.org/10.3165/jjpn.cr.2022.0208","url":null,"abstract":"","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114572682","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/jjpn.cr.2019.0154
Yuko Yamada, Yasuo Kawaba, Hiroki Yokoyama, K. Kitamoto, Shin’ichi Okada, S. Kanzaki
{"title":"Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome) with a multiple renal artery and essential hypertension","authors":"Yuko Yamada, Yasuo Kawaba, Hiroki Yokoyama, K. Kitamoto, Shin’ichi Okada, S. Kanzaki","doi":"10.3165/jjpn.cr.2019.0154","DOIUrl":"https://doi.org/10.3165/jjpn.cr.2019.0154","url":null,"abstract":"","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"26 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134262057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.3165/jjpn.rv.2022.2001
Y. Ikezumi
{"title":"Renal biopsy —The first step and another step—","authors":"Y. Ikezumi","doi":"10.3165/jjpn.rv.2022.2001","DOIUrl":"https://doi.org/10.3165/jjpn.rv.2022.2001","url":null,"abstract":"","PeriodicalId":205802,"journal":{"name":"Japanese journal of pediatric nephrology","volume":"542 ","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131657159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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