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Ishikura K.,Tokyo Metropolitan Childrens Medical Center | Uemura O.,Aichi Childrens Health and Medical Center | Ito S.,National Center for Child Health and Development | Wada N.,Shizuoka Childrens Hospital | And 8 more authors.
Nephrology Dialysis Transplantation | Year: 2013

Background. Chronic kidney disease (CKD) in children is a progressive and intractable condition that may severely impair the child's growth, development and quality of life. Epidemiological information on pediatric CKD, particularly in Asians, is scant. Methods. We conducted a nationwide, population-based survey of Japanese children aged 3 months to 15 years with pre-dialysis CKD to examine the prevalence of pediatric CKD in Japan. CKD was classified according to newly established criteria derived from reference serum creatinine levels in Japanese children. Surveys were sent to 1190 institutions across Japan to report on cases of pediatric CKD managed as of 1 April 2010. Results. A total of 925 institutions (77.7%) responded. Information on 447 children was collected. When subdivided according to our diagnostic criteria, 70.5% of children had stage 3 CKD, 23.9% stage 4 and 5.6% stage 5. The estimated prevalence of Japanese children with CKD was 2.98 cases/100 000 children. Of 407 CKD cases with non-glomerular disease, 278 (68.3%) had congenital anomalies of the kidney and urinary tract (CAKUT). The newly established criteria showed good validity compared with existing criteria, including the abbreviated Schwartz equation. Conclusions. Findings from the first nationwide survey of predialysis CKD in Asian children indicate that the prevalence of stage 3-5 CKD in children in Japan aged 3 months to 15 years is 2.98 cases/100 000 children. Most children with CKD presented with non-glomerular disease, most frequently CAKUT. Improved management of CAKUT, including renoprotective treatment and urological intervention, is required. © The Author 2013. Published by Oxford University Press on behalf of ERA-EDTA.

Ishikura K.,Tokyo Metropolitan Childrens Medical Center | Uemura O.,Aichi Childrens Health and Medical Center | Hamasaki Y.,Toho University | Ito S.,National Center for Child Health and Development | And 6 more authors.
Nephrology Dialysis Transplantation | Year: 2014

BackgroundThe risk of progressing to end-stage kidney disease (ESKD) and factors associated with progression in children with chronic kidney disease (CKD) are unclear, especially in Asian children.MethodsWe started a nationwide, prospective cohort study of 447 Japanese children with pre-dialysis CKD in 2010, with follow-up in 2011. Progression to ESKD was analyzed by Kaplan-Meier analysis according to CKD stage. Cox regression analysis was used to identify risk factors for progression.ResultsData were analyzed for 429/447 children. Five patients died, of which four died before progression to ESKD. Fifty-two patients progressed to ESKD (median follow-up 1.49 years), including 9/315 patients with stage 3 CKD, 29/107 with Stage 4 CKD and 14/25 with Stage 5 CKD. One-year renal survival rates were 98.3, 80.0 and 40.9%, for Stages 3, 4 and 5 CKD, respectively. Risk factors for progression to ESKD included CKD stage [versus Stage 3; Stage 4: hazard ratio (HR) 11.12, 95% confidence interval (CI) 4.22-29.28, P < 0.001; Stage 5: HR 26.95, 95% CI 7.71-94.17, P < 0.001], heavy proteinuria (>2.0 g/g urine creatinine; HR 7.56, 95% CI 3.22-17.77, P < 0.001) and age (< 2 years: HR 9.06; 95% CI 2.29-35.84, P = 0.002; after starting puberty: HR 4.88; 95% CI 1.85-12.85, P = 0.001).ConclusionsIn this cohort, 12.5% of children with pre-dialysis CKD progressed to ESKD with a median-follow-up of 1.49 years. Children with advanced (Stage 4/5) CKD were particularly likely to progress. To our knowledge, this is the first, nationwide, prospective cohort study of children with pre-dialysis CKD in Asia. © The Author 2014.

Morisada N.,Kobe University | Sekine T.,Toho University | Ishimori S.,Kakogawa West City Hospital | Tsuda M.,Tsuda Childrens Clinic | And 5 more authors.
Pediatrics International | Year: 2014

Microdeletion of 16q12 is a rare chromosomal abnormality. We present the cases of two Japanese patients with developmental and renal symptoms of differing clinical severity. Both patients had 16q12 interstitial microdeletions that included the entire SALL1 gene. Patient 1 was a 15-year-old Japanese boy clinically diagnosed with branchio-oto-renal syndrome with mild developmental delay, but with no imperforate anus or polydactyly. Array comparative genome hybridization (aCGH) indicated a 5.2 Mb deletion in 16q12, which included SALL1. Patient 2 was a 13-year-old Japanese boy diagnosed with Townes-Brocks syndrome and severe developmental delay, epilepsy, and renal insufficiency requiring renal replacement therapy. Fluorescence in situ hybridization indicated deletion of the entire SALL1 gene. Subsequent aCGH showed a 6 Mb deletion in 16q12q13, which included SALL1. Precise analysis of the present two cases will give us some clues to elucidate the pathogenic mechanisms of 16q12 microdeletion syndrome. © 2014 Japan Pediatric Society.

Imamura T.,Kyoto Prefectural University of Medicine | Iwamoto S.,Mie University | Kanai R.,The University of Shimane | Shimada A.,Nagoya University | And 10 more authors.
British Journal of Haematology | Year: 2012

The acute myeloid leukaemia (AML) 99 trial conducted previously in Japan for the treatment of de novo paediatric AML showed excellent results, with a 5-year overall survival (OS) and event-free survival (EFS) of 75·6% and 61·6%, respectively. To examine reproducibility of these results in another cohort, the outcome of 146 newly diagnosed AML paediatric patients prospectively registered in the Japan Association of Childhood Leukaemia Study (JACLS) from 2003 to 2006 was compared to that of 240 patients in the original AML 99 clinical trial. The 5-year EFS and OS achieved in the new cohort was 66·7 ± 4·0% and 77·7 ± 8·0% respectively, which were comparable to those obtained in the original AML 99 clinical trial, although less frequent core-binding factor (CBF) AML (29·5% vs. 37%) and an almost equal frequency of allogeneic haematopoietic stem cell transplantation (allo-HSCT) during first complete remission (16·5% vs. 19%) were observed. The 5-year EFS in patients with a normal karyotype (NK) (n = 35, 54·9 ± 15·1%) was inferior in the present cohort when compared to the original AML99 trial. This study confirmed the excellent outcome of the original AML99 protocol. © 2012 Blackwell Publishing Ltd.

Takahashi H.,Toho University | Watanabe T.,Aichi Gakuin University | Kinoshita A.,St. Marianna University School of Medicine | Yuza Y.,Tokyo Metropolitan Childrens Medical Center | And 21 more authors.
British Journal of Haematology | Year: 2016

We evaluated the efficacy of treatment using reduced cumulative doses of anthracyclines in children with acute promyelocytic leukaemia (APL) in the Japanese Paediatric Leukaemia/Lymphoma Study Group AML-P05 study. All patients received two and three subsequent courses of induction and consolidation chemotherapy respectively, consisting of all-trans retinoic acid (ATRA), cytarabine and anthracyclines, followed by maintenance therapy with ATRA. Notably, a single administration of anthracyclines was introduced in the second induction and all consolidation therapies to minimize total doses of anthracycline. The 3-year event-free (EFS) and overall survival rates for 43 eligible children were 83·6% [95% confidence interval (CI): 68·6–91·8%] and 90·7% (95% CI: 77·1–96·4%), respectively. Although two patients died of intracranial haemorrhage or infection during induction phases, no cardiac adverse events or treatment-related deaths were observed during subsequent phases. Patients not displaying M1 marrow after the first induction therapy, or those under 5 years of age at diagnosis, showed inferior outcomes (3-year EFS rate; 33·3% (95% CI: 19·3–67·6%) and 54·6% (95% CI: 22·9–78·0%), respectively). In conclusion, a single administration of anthracycline during each consolidation phase was sufficient for treating childhood APL. In younger children, however, conventional ATRA and chemotherapy may be insufficient so that alternative therapies should be considered. © 2016 John Wiley & Sons Ltd

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