IgA nephropathy with presentation of nephrotic syndrome at onset in children

Shima, Yuko; Nakanishi, Koichi; Sato, Masashi; Hama, Taketsugu; Mukaiyama, Hironobu; Togawa, Hiroko; Tanaka, Ryojiro; Nozu, Kandai; Sako, Mayumi; Iijima, Kazumoto; Suzuki, Hiroyuki; Yoshikawa, Norishige

doi:10.1007/s00467-016-3502-6

Cited by 23 publications

(18 citation statements)

References 30 publications

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“…Likewise, NS was found to be a poor prognostic factor for IgAN patients in previous studies [35,36]; none of the IgAN patients in this study who presented with NS and achieved remission progressed to CKD stage 3-5. Several recent studies reported that the most significant factor with respect to survival is responsiveness to treatment [18,35]; the results of the present study also support this notion (Figure 4, Table S3).…”

Section: Discussionsupporting

confidence: 89%

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Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy

Suh

Jang

Hyun

et al. 2020

JCM

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Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerulopathies diagnosed in children and adolescents. This study aimed to evaluate the clinical features in and outcomes of pediatric IgAN over the last 30 years. Patients who were diagnosed before age of 18 at 20 centers in Korea were evaluated retrospectively. Of the 1154 patients (768 males, 386 females) with a median follow-up of 5 years, 5.6% (n = 65) progressed to stage 3–5 chronic kidney disease (CKD). The 10- and 20-year CKD-free survival rates were 91.2% and 75.6%, respectively. Outcomes did not differ when comparing those in Korea who were diagnosed prior to versus after the year 2000. On multivariate analysis, combined asymptomatic hematuria and proteinuria as presenting symptoms and decreased renal function at the time of biopsy were associated with progression to CKD, while remission of proteinuria was negatively associated with this outcome. Patients who presented with gross hematuria or nephrotic syndrome tended toward positive outcomes, especially if they ultimately achieved remission. While remission of proteinuria might imply that the disease is inherently less aggressive, it also can be achieved by management. Therefore, more aggressive management might be required for pediatric-onset IgAN.

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Section: Discussionsupporting

confidence: 89%

“…No or minor abnormalities were considered as favorable overall outcomes; all other conditions were regarded as unfavorable outcomes. The primary endpoint was deterioration of renal functional to an eGFR < 60 mL/min/1.73 m 2 ; this was considered as a diagnosis of chronic kidney disease (CKD) in this study [18,19].…”

Section: Definitions Of Outcomesmentioning

confidence: 99%

Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy

Suh

Jang

Hyun

et al. 2020

JCM

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“…Nephrotic-range proteinuria is a relatively rare presentation but important risk factor for poor prognosis in IgAN patients. In our study, it showed that 33.8% of the children with primary IgAN have the clinical manifestation of nephrotic-range proteinuria, which is consistent with the result (30.6%) of a multi-center study in China, [9] but higher than that of other countries [8] . It may be partly because of racial or genetic differences, but still requires further study.…”

Section: Discussionsupporting

confidence: 91%

“…In most of the IgAN patients, the initial manifestations are recurrent episodes of asymptomatic microscopic hematuria or gross hematuria that usually concurrent with infections, with or without proteinuria. Nephrotic-range proteinuria is a relatively rare presentation in IgAN, with a prevalence rate of 5% to 20%, and 7% to 30.6% in adults, and children, [5–9] respectively. However, persistent proteinuria has been identified to be one of the most important clinical risk factors for poor prognosis, as well as hypertension and renal insufficiency, while diffuse mesangial proliferation, segmental sclerosis, crescents (C), interstitial fibrosis, and tubular atrophy (T) are considered to be pathological risk factors [10–13] .…”

Section: Introductionmentioning

confidence: 99%

Primary IgA nephropathy with nephrotic-range proteinuria in Chinese children

Zhao¹,

Wang²,

Huang³

et al. 2021

Medicine

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To investigate the clinicopathological features and outcomes of primary IgA nephropathy with nephrotic-range proteinuria in Chinese children. Patients with biopsy-proven IgA nephropathy and nephrotic-range proteinuria between January 2011 and December 2017 were included, and their proteinuria and renal function were followed up. A total of 90 patients were enrolled, and 21.1% (19/90) of them had decreased renal function at diagnosis. Complete remission, partial remission, and no response of proteinuria occurred in 88.6% (70/79), 10.1% (8/79), and 1.3% (1/79), respectively, of the 79 patients who were followed up for 6 to 104 months. 73.7% (14/19) of the patients with decreased renal function at diagnosis recovered to normal level while 26.3% (5/19) of them did not recover or progressed to end-stage renal disease. Two patients with normal renal function at diagnosis progressed to renal insufficiency during follow-up period. By multivariate analysis, the risk for renal function deterioration was significantly higher in the partial remission and no response groups than in the complete remission group. Remission of proteinuria was important for improving renal prognosis in children with IgA nephropathy and nephrotic-range proteinuria. The outcomes for pediatric patients appeared to be better than that reported in adults.

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“…Podocyte function may also be impaired in the course of lysosomal diseases, which should be considered in the diagnostic procedure, especially in the case of the development of nephropathy in young children [ 2 ]. In older children, nephrotic syndrome may develop in the course of autoimmune inflammatory diseases, such as lupus erythematosus, acute poststreptococcal glomerulonephritis, IgAN (IgA Nephropathy) or IgAVN (Immunoglobulin (Ig)A vasculitis nephritis) [ 3 , 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

Involvement of Hemopexin in the Pathogenesis of Proteinuria in Children with Idiopathic Nephrotic Syndrome

Pukajło-Marczyk

Zwołińska

2021

JCM

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Hemopexin (Hpx) is considered a factor in the pathogenesis of idiopathic nephrotic syndrome (INS). The aim of the study was to evaluate the serum and urine values of Hpx (sHpx and uHpx) in children with INS, analyze the role of Hpx, and assess its usefulness as a marker of the disease course. 51 children with INS and 18 age-matched controls were examined. Patients were divided into subgroups depending on the number of relapses (group IA—the first episode of INS, group IB—with relapses) and according to method of treatment (group IIA treated with gluco-corticosteroids (GCS), group IIB treated with GCS and other immunosuppressants). Hpx concentrations were determined by enzyme-linked immunosorbent assay (ELISA). sHpx and uHpx values in relapse were elevated in the whole INS group versus controls (p < 0.000). In remission their levels decreased, but still remained higher than in the control group (p < 0.000). In group IB uHpx levels were increased during remission as compared to group IA (p < 0.006). No significant impact of immuno-suppressants on sHpx was observed, but uHpx excretion in group IIA was higher in relapse (p < 0.026) and lower in remission (p < 0.0017) as compared to group IIB. The results suggest the role of Hpx in the pathogenesis of INS. Hpx may be a useful indicator for continuation of treatment, but it requires confirmation by further controlled studies.

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IgA nephropathy with presentation of nephrotic syndrome at onset in children

Abstract: The most significant factor for renal survival was responsiveness to treatment, not NS itself. As modifiable acute lesions are the dominant pathological findings in NS-IgAN, histological improvements achieved by appropriate treatments can result in a favorable prognosis.

Cited by 23 publications

References 30 publications

Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy

Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy

Primary IgA nephropathy with nephrotic-range proteinuria in Chinese children

Involvement of Hemopexin in the Pathogenesis of Proteinuria in Children with Idiopathic Nephrotic Syndrome

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