Frequency of renal diseases and clinical indications for renal biopsy in children (report of the Italian National Registry of Renal Biopsies in Children). Group of Renal Immunopathology of the Italian Society of Pediatric Nephrology and Group of Renal Immunopathology of the Italian Society of Nephrology.

Coppo, Rosanna; Gianoglio, Bruno; Porcellini, Maria Gabriella; Maringhini, Silvio

doi:10.1093/oxfordjournals.ndt.a027821

Cited by 130 publications

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“…Our cohort was older than that described in other paediatric registries . This is likely because we are a tertiary centre, with a large referred patient population drawn from neighbouring states and countries.…”

Section: Discussionmentioning

confidence: 95%

Spectrum of biopsy proven renal disease in South Asian children: Two decades at a tropical tertiary care centre

et al. 2018

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Section: Discussionmentioning

confidence: 95%

Spectrum of biopsy proven renal disease in South Asian children: Two decades at a tropical tertiary care centre

et al. 2018

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“…Unfortunately, and until very recently, these young familial hematuric patients were not routinely submitted to an early renal biopsy to include electron microscopy. Instead, a "wait-and-see" approach until proteinuria developed was followed by many experts [20]. The recent recognition of the pure isolated C3 nephropathy and the additional possibility of TBMN may now help to shift the clinician’s decision towards an earlier renal biopsy in combination with molecular genetics studies.…”

Section: Introductionmentioning

confidence: 99%

The role of molecular genetics in diagnosing familial hematuria(s)

2011

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Familial microscopic hematuria (MH) of glomerular origin represents a heterogeneous group of monogenic conditions involving several genes, some of which remain unknown. Recent advances have increased our understanding and our ability to use molecular genetics for diagnosing such patients, enabling us to study their clinical characteristics over time. Three collagen IV genes, COL4A3, COL4A4, and COL4A5 explain the autosomal and X-linked forms of Alport syndrome (AS), and a subset of thin basement membrane nephropathy (TBMN). A number of X-linked AS patients follow a milder course reminiscent of that of patients with heterozygous COL4A3/COL4A4 mutations and TBMN, while at the same time a significant subset of patients with TBMN and familial MH progress to chronic kidney disease (CKD) or end-stage kidney disease (ESKD). A mutation in CFHR5, a member of the complement factor H family of genes that regulate complement activation, was recently shown to cause isolated C3 glomerulopathy, presenting with MH in childhood and demonstrating a significant risk for CKD/ESKD after 40 years old. Through these results molecular genetics emerges as a powerful tool for a definite diagnosis when all the above conditions enter the differential diagnosis, while in many at-risk related family members, a molecular diagnosis may obviate the need for another renal biopsy.

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“…The different criteria to perform renal biopsies in European children with oligo-symptomatic urinary abnormalities account for the variability in the reports on the clinical presentation at renal biopsy and the natural course of pediatric IgAN in Europe [1-3]. For this reason, to gain a general overview of this glomerular disease in children, a suitable observational approach is offered by the cohort gathered by the collaborative study to validate the Oxford Classification of IgAN, VALIGA [9].…”

Section: Clinical Features Of Children and Young Subjects With Igan Imentioning

confidence: 99%

“…Europe is the second continent with a frequency of this disease accounting for 20% of renal biopsies performed in pediatric age [1-3], while Asia has a 40% frequency [4, 5]. This different prevalence might be due to genetic or environmental factors, which play a greater role in Asia than in Europe, particularly favoring the early onset of IgAN [6].…”

Section: Introduction and Interest For European Pediatric Nephrologymentioning

confidence: 99%

“…However, most probably, the frequency increases where screening programs are active, like in Japan and Korea [5, 7]. In Europe, this policy has not been adopted, and IgAN is diagnosed in children undergoing renal biopsy because of persistent heavy isolated microscopic hematuria or hematuria associated with non-nephrotic proteinuria [1-3]. The prevalence of pediatric IgAN in Europe varies in different reports, due to the variable criteria for performing renal biopsy in young subjects shortly after the detection of urine anomalies or limiting renal biopsy to cases having developed proteinuria.…”

Section: Introduction and Interest For European Pediatric Nephrologymentioning

confidence: 99%

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Pediatric IgA Nephropathy in Europe

Coppo

2019

Kidney Dis

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Background: In Europe IgA nephropathy (IgAN) is detected in 20% of children with glomerular diseases diagnosed by renal biopsy. The outcome during childhood is generally good, but progression in the long-term follow-up may occur in about 20% of children after 20 years. Summary: In Europe, urine screening programs are not active, and there is variability in the policy to perform renal biopsies in oligo-symptomatic children. Hence, a suitable observational approach to pediatric IgAN is offered by the VALIGA study which included 174 children aged < 18 years from 13 European countries followed over a median of 4.4 (2.5–7.5) years. Renal pathology lesions were centrally scored according to the Oxford Classification of IgAN (mesangial hypercellularity, M; endocapillary hypercellularity, E; segmental glomerulosclerosis, S; tubular atrophy/interstitial fibrosis, T; crescents, C [MEST-C]). Children had renal biopsy mostly with normal estimated glomerular filtration rate (eGFR) and moderate proteinuria of a median of 0.84 g/day/1.73 m² (< 0.30 g/day/1.73 m² in 30% of the cases). Children showed M1 in 21.8%, E1 in 13.8%, S1 in 42.5%, T1–2 in 6.3%, and C1 in 14.9%. The survival at the combined endpoint of 50% eGFR decrease or end-stage renal disease at 15 years was 94%. The slow progression rate and the limited number of cases progressing to the combined endpoint (6.4%) did not allow the detection of a predictive value of the MEST-C score. Moreover, the predictive value of clinical and pathological features was likely blunted by the use of corticosteroid/immunosuppressive treatment (CS/IS) in 50% of the cases. The survival tree analysis also proved that children < 16 years old with IgAN without mesangial hypercellularity (M0) and well preserved eGFR (> 90 mL/min/1.73 m²) had a high probability of proteinuria remission during follow-up. Moreover, in this subgroup of children, the benefits of CS/IS therapy reached statistical significance. In Europe, the use of CS/IS treatment in IgAN is still a debated issue, but most children tend to be treated more commonly than adults with CS/IS. A recent uncontrolled study reports a favorable outcome in European children with IgAN and very active acute forms of IgAN with improvement in eGFR and reduction in proteinuria. Key Messages: In Europe, children with IgAN have a favorable prognosis in the short term, and this may be due also to the frequently adopted CS/IS therapy, particularly with acute and active pathological features. The risk of progression over decades of follow-up remains an unsolved problem which needs to be addressed by controlling subtle chronic pathogenetic factors which work in children as well as in adult cases of IgAN.

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Abstract: This National Registry provides data on the indications for performing renal biopsy in Italian children and on the frequency and annual incidence of histological lesions detected. IgAN, primary or related to HSP, was the most common nephritis in Italian children undergoing renal biopsy.

Cited by 130 publications

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Spectrum of biopsy proven renal disease in South Asian children: Two decades at a tropical tertiary care centre

Spectrum of biopsy proven renal disease in South Asian children: Two decades at a tropical tertiary care centre

The role of molecular genetics in diagnosing familial hematuria(s)

Pediatric IgA Nephropathy in Europe

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