The incidence and severity of Henoch-Schönlein purpura nephritis over a 22-year period in Fukushima Prefecture, Japan

Kawasaki, Yukihiko; Suyama, Kazuhide; Yugeta, E; Katayose, Masahiko; Suzuki, Shigeo; Sakuma, Hitoshi; Nemoto, Kenji; Tsukagoshi, Akira; Nagasawa, Katsutoshi

doi:10.1007/s11255-009-9701-3

Cited by 26 publications

(27 citation statements)

References 19 publications

(21 reference statements)

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“…The incidence of ESRD in type 1 DM was nine times higher than that in the United States and warrants public attention on disease management (24). These findings are compatible with and also strengthened the current knowledge in pediatric ESRD (25,26).…”

Section: Discussionsupporting

confidence: 74%

Incidence and Renal Survival of ESRD in the Young Taiwanese Population

Tsai

Chen²,

et al. 2014

Clinical Journal of the American Society of Nephrology

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SummaryBackground and objectives ESRD in the young represents a heavy burden to patients, families, and health care systems. This nationwide retrospective study characterized the incidence of ESRD and analyzed diagnoses associated with renal survival in the young population in Taiwan.Design, setting, participants, & measurements Through use of Taiwan's National Health Insurance Research Database, the population of young patients (age,30 years, including children and young adults) with ESRD between January 1998 and December 2009 were enrolled. The medical claims were used to derive the date when the cause of ESRD was first determined. The medical data were reviewed and the renal survival time (time from first diagnosis of the cause to the start of ESRD) was calculated by experts, including clinical physicians and a large-database specialist.Results The incidence rate of ESRD in the young population was high compared with the worldwide rate at 21.1 per million person-years, whereas the incidence in the pediatric group was still similar to that in other countries at 10.3 per million person-years. A total of 2304 patients with new-onset ESRD and identified renal diseases during the study period were enrolled. All preschool-age patients (100%) began receiving peritoneal dialysis as their initial treatment for ESRD. The leading causes, which varied by sex and onset age, were glomerulonephropathy followed by hypertension for the young adult group and glomerulonephropathy followed by congenital anomalies of the kidney and urinary tract (CAKUT) for the pediatric group. Renal survival was cause-dependent. The median overall renal survival duration was 0.8 year (interquartile range [IQR], 0.7-3.5 years). CAKUT-related ESRD had the longest progression time (median renal survival, 16.0 years; IQR, 10.7-23.5 years); glomerulonephropathy progressed more rapidly into ESRD and had the shortest median renal survival of 0.5 year (IQR, 0.1-2.7 years).Conclusions The incidence and causes of ESRD greatly differ between pediatric patients and young adults. Moreover, renal survival in the young population markedly varies depending on the cause of renal disease.

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Section: Discussionsupporting

confidence: 74%

Incidence and Renal Survival of ESRD in the Young Taiwanese Population

Tsai

Chen²,

et al. 2014

Clinical Journal of the American Society of Nephrology

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“…Increased polymeric IgA production by the mucosal immune system in response to a mucosally presented antigen, such as bacteria, viruses, or fungi, has been hypothesized as a potential mechanism for the development of HSP 9) . Hyper -reactivity of both B and T cells in response to specific antigenic stimuli in vitro has been reported in patients with HSP, resulting in increased polymeric IgA production, including Gd -IgA in mucosal and tonsillar cells 18) .…”

Section: Pathogenesismentioning

confidence: 99%

HENOCH-SCH^|^Ouml;NLEIN PURPURA NEPHRITIS IN CHILDHOOD: PATHOGENESIS, PROGNOSTIC FACTORS AND TREATMENT

Kawasaki

Ono

Ohara

et al. 2013

FJMS

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: Henoch -Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. Thus, it is important to clarify the onset mechanism as well as the prognostic factors of Henoch -Schönlein purpura nephritis (HSPN) and to identify the most appropriate treatment. We herein review the pathogenesis, the prognostic factors and treatment of HSPN. As to the pathogenesis, several studies suggest that galactose -deficient IgA1 (Gd -IgA1) is recognized by anti -glycan antibodies, leading to the formation of circulating immune complexes and their mesangial deposition, thereby inducing renal injury. With regard to the prognostic factors, a number of factors have been suggested including nephrotic syndrome, decreased factor XIII activity, hypertension, severe renal injury, high renal accumulation of activated macrophage, alpha -smooth muscle actin, and high serum myeloid -related protein levels. For the treatment of severe HSPN, aggressive therapies including multiple drug combination therapy and plasmapheresis have been shown to be effective in ameliorating proteinuria and histological severity. Nevertheless, detailed investigation into the pathogenesis of HSPN and double -blind randomized control studies on children with HSPN are still necessary.

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“…The incidence of nephritis in patients with Henoch-Schönlein purpura has been reported to be 15-62% with an estimated annual incidence of 20.4 per 100,000 children (Gardner-Medwin et al, 2002;Shenoy, Bradbury, et al 2007;Bogdanovic, 2009). The overall incidence of Henoch-Schönlein purpura nephritis and the severity of HenochSchönlein purpura nephritis in patients between 1987 and 1997 were similar to those in children between 1998 and 2008 and the number of patients with severe Henoch-Schönlein purpura nephritis has not decreased (Kawasaki et al, 2010). The overall incidence of Henoch-Schönlein purpura nephritis is rather stable over time.…”

Section: Incidencementioning

confidence: 96%

“…Another clinical evaluation categorized the patients according to four stages. Stage (A) is considered normal: the patient was normal on physical examination, with normal urine and renal function; stage (B) had minor urinary abnormalities: the patient was normal on physical examination, with microscopic hematuria or proteinuria of less than 20 mg/m 2 /h; stage (C) had persistent nephropathy: the patient had proteinuria of 20 mg/m 2 /h or greater or hypertension and a 24-h creatinine clearance of 60 ml/ min/1.73 m 2 or greater; stage (D) had renal insufficiency: the patient had a 24-h creatinine clearance of less than 60 ml/min/1.73 m 2 , including dialysis/transplant or death (Kawasaki et al, 2010).…”

Section: Classificationmentioning

confidence: 99%

“…The renal biopsy is helpful in determining the need for treatment with immunosuppression in the acute phase (McCarthy & Tizard, 2010). The criteria for renal biopsy were defined as follows: (1) the patients had proteinuria of 20 mg/m 2 /hour or greater and haematuria or (2) the patients had proteinuria of less than 20 mg/m 2 /hour and recurrent macrohematuria (Kawasaki et al, 2010). According to another recent review a renal biopsy has been recommended in the following situations: (1) acute renal impairment/nephritic syndrome at presentation; (2) nephrotic syndrome with normal renal function persisting at 4 weeks; (3) nephrotic range proteinuria (urine protein/creatinine ratio, >250 mg/mmol) at 4-6 weeks (if not improving spontaneously); (4) persistent proteinuriaurine protein/creatinine ratio >100 mg/mmol for more than 3 months.…”

Section: Renal Biopsymentioning

confidence: 99%

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