High incidence of idiopathic nephrotic syndrome in East Asian children: a nationwide survey in Japan (JP-SHINE study)

Kikunaga, Kaori; Ishikura, Kenji; Terano, Chikako; Mai, Sabine; Komaki, Fumiyo; Hamasaki, Yuko; Sasaki, Satoshi; Iijima, Kazumoto; Yoshikawa, Norishige; Nakanishi, Koichi; Nakazato, Hitoshi; Matsuyama, Tomohiro; Ashihara, Takashi; Ito, Shuichi; Honda, Masashi

doi:10.1007/s10157-016-1319-z

Cited by 49 publications

(27 citation statements)

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“…2 In Japan, the estimated incidence of INS is 6.49 cases per 100,000 children. 3 Approximately 3% of children with steroid-sensitive nephrotic syndrome (SSNS) have a family history of SSNS. 4 The pathogenesis of INS is still unclear, although more than 50 causative single genes for steroid-resistant nephrotic syndrome have been found, and causative mutations in these genes were identified in approximately 30% of childhood and young adult patients with steroid-resistant nephrotic syndrome.…”

mentioning

confidence: 99%

Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome

Jia

McNulty

Song

et al. 2020

Kidney International

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confidence: 99%

Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome

Jia

McNulty

Song

et al. 2020

Kidney International

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“…The incidence of T1DM in Japanese children < 14 years of age is 1.4–2.25/100,000 [ 1 ], which is much lower than that in other countries, especially Finland (45.0–64.2/100,000 children < 15 years of age) [ 2 ]. Conversely, the estimated incidence of pediatric idiopathic nephrotic syndrome in Japan is 6.49 cases/100,000 [ 3 ], which is slightly higher than the average incidence in previous studies worldwide (4.7/100,000) [ 4 ]. Most cases of pediatric idiopathic nephrotic syndrome manifest as minimal change nephrotic syndrome (MCNS) in Japan, which is similar to that in other countries.…”

Section: Introductionmentioning

confidence: 99%

Concurrent minimal change nephrotic syndrome and type 1 diabetes mellitus in an adult Japanese woman: a case report

et al. 2020

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Background Concurrent type 1 diabetes mellitus (T1DM) and idiopathic nephrotic syndrome is rare, and most previously reported cases were in children. We report the case of an adult woman who developed T1DM and minimal change nephrotic syndrome (MCNS) nearly simultaneously. Case presentation A 24-year-old woman had first presented to another hospital with nausea, vomiting, and fatigue. She was diagnosed with diabetic ketoacidosis and T1DM on the basis of her hyperglycemia, ketoacidosis, and positive anti-glutamic acid decarboxylase antibody test result. Rapid infusion of normal saline and insulin administration alleviated hyperglycemia and ketoacidosis. Two weeks after admission, however, she developed nephrotic syndrome (NS) with rapidly decreasing urine volume. She was referred to our hospital with a diagnosis of acute kidney injury. Although she temporarily required dialysis and high doses of insulin, within 1 month NS and acute kidney injury had been alleviated by oral prednisolone and low-density lipoprotein apheresis. Renal biopsy showed minor glomerular abnormalities without diabetic nephropathy, so we diagnosed her with MCNS. Seven weeks after the discharge, NS relapsed, and cyclosporine was added to prednisolone. However, NS relapsed twice within the next 4 months, so we started her on rituximab. At 6 months after initiating rituximab therapy, she remained in complete remission. Her mother also had T1DM but not MCNS. The patient had HLA-DRB1*09:01/09:01, DQB1*03:03/03:03, and her mother had HLA-DRB1*04:05/09:01, DQB1*03:03/04:01. Conclusions Concurrent T1DM and MCNS is rare and their coexistence might be coincidental. Alternatively, they might have been caused by an underlying, unidentified genetic predisposition. Previous reports and our patient’s findings suggest that specific HLA alleles and haplotypes or a Th1/Th2 imbalance might be associated with T1DM and MCNS that occurred nearly simultaneously.

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“…Primary nephrotic syndrome (PNS) is one of the most common kidney diseases in children with an incidence of 2.0 to 7/100,000 and a prevalence of 16/100,000 [1][2]. The incidence of PNS in East Asian children is approximately three to four times higher than that in Caucasians [3] [4]. The major clinical features of PNS are proteinuria, hypoalbuminemia, hyperlipidemia, and varying degrees of edema.…”

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confidence: 99%

Changes in clinicopathological features of primary nephrotic syndrome in children over a 10 years period: Research Paper

Guo

et al. 2020

Preprint

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Background Primary nephrotic syndrome (PNS) is a common chronic disease in childhood and results in heavy illness burden. The aim of this study is to investigate the change in the clinicopathological features of children with NS worldwide. Methods Children with PNS were divided into two groups according to the date of admission. Group A was admitted from January 1, 2006 to December 31, 2010 and group B from January 1, 2011 to December 31, 2015. Clinical manifestations and pathological features of the children in both groups were retrospectively analyzed. Results This study includes 1072 patients. There were 783 cases of steroid-sensitive nephrotic syndrome (SSNS), 241 cases of steroid-resistant nephrotic syndrome (SRNS), and 48 cases of unknown steroid effects. The incidence of complications in group B was significantly higher than that in group A, especially for infections. A total of 136 (12.96%) patients underwent renal biopsy. The most common lesions were minimal change disease (44.12%). The rate of mesangial proliferative glomerulonephritis (MsPGN) significantly decreased and that of membranous nephropathy (MN) increased over the years. A total of 292 (27.24%) patients were administered immunosuppressants. The administration of Tripterygium was decreased, whereas the using of Tacrolimus increased over the years. Conclusion There have been changes in the incidence of complications, pathological features, and immunosuppressants in children with PNS over 10 years.

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High incidence of idiopathic nephrotic syndrome in East Asian children: a nationwide survey in Japan (JP-SHINE study)

Cited by 49 publications

References 28 publications

Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome

Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome

Concurrent minimal change nephrotic syndrome and type 1 diabetes mellitus in an adult Japanese woman: a case report

Changes in clinicopathological features of primary nephrotic syndrome in children over a 10 years period: Research Paper

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