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Clinical and histological differences between adults and children in new onset IgA nephropathy
Alexandra Cambier1, Marion Rabant2, Khalil El Karoui3
1Service de néphrologie pédiatrique, APHP, Hôpital Universitaire Robert-Debré, 48 Boulevard Serrurier, 75019, Paris, France. alexandra.cambier@aphp.fr.
Insights
Children with IgA nephropathy (IgAN) show higher eGFR and more proliferative lesions, while adults present with more chronic damage. Histological assessment is crucial for understanding disease presentation in IgAN patients.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- IgA nephropathy (IgAN) presentation differs between children and adults.
- No prior systematic comparison of pediatric vs. adult IgAN clinical, biological, and histological features exists.
Purpose of the Study:
- To systematically compare clinical and histological characteristics of childhood-onset IgAN with adult-onset IgAN.
- To identify differences in disease presentation and response to treatment between pediatric and adult IgAN patients.
Main Methods:
- Retrospective review of 211 IgAN patients (82 children, 129 adults) from two Paris centers.
- Kidney biopsies analyzed for Oxford classification and podocytopathic (P1) features.
- Comparison of clinical and histological data at diagnosis and after steroid treatment.
Main Results:
- Children had higher eGFR (89.5 vs. 64 ml/min/1.73 m²), while proteinuria levels were similar.
- Pediatric IgAN showed more mesangial (M1) and endocapillary (E1) hypercellularity.
- Adults had more focal glomerulosclerosis (S1), tubular atrophy/fibrosis (T1), and P1 features.
- Proteinuria associated with proliferative lesions (M1, E1, C1) in children, and chronic lesions (S1, P1, T1) in adults.
- Steroid treatment improved proteinuria in both groups, with eGFR increasing in children and remaining stable in adults.
Conclusions:
- Proteinuria in pediatric IgAN indicates glomerular proliferative lesions.
- Proteinuria in adult IgAN often signifies chronic lesions, necessitating histological evaluation.
- Distinct clinical and histological profiles of IgAN in children and adults underscore the need for tailored diagnostic and therapeutic approaches.
Background:
Previous reports suggest initial presentation of IgA nephropathy (IgAN) in children is different from adults. No systematic comparison of clinical, biological, and histological childhood- and adult-onset IgAN is currently available.
Methods:
We compared pediatric and adult clinical and histological characteristics at IgAN diagnosis. Data on 211 consecutive patients from two different centers in Paris (82 children, 129 adults) were reviewed. Kidney biopsies were scored for Oxford classification and podocytopathic (P1) features.
Results:
We report higher eGFR at diagnosis in children compared to adults (89.5 vs. 64 ml/min/1.73 m2; p = 0.0001) but no difference in proteinuria. Histological analysis of kidney biopsy found higher proportions of mesangial (M1) and endocapillary (E1) hypercellularity in children compared with adults (M1 [80.7% vs. 27.9%, p = 0.0001]; E1 [71.3% vs. 30%, p = 0.0001]). Focal glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis ≥ 25% (T1), and P1 were more frequent in adults (S1 [81.5% vs. 61.3%, p = 0.0012], T1 [49.5% vs. 1.35%, p = 0.0001], P1 [33.8% vs. 16.4%, p = 0.008). Proteinuria associated with M1, E1, and C1 in children (M1, p = 0.0001; E1, p = 0.0005; C1, p = 0.0014) but S1, P1, and T1 in adults (S1, p = 0.0001; P1, p = 0.0001; T1, p = 0.001). After steroid treatment (41 children and 28 adults), proteinuria decreased in children (p < 0.001, follow-up 38 months) and adults (p < 0.001, follow-up 76.9 months), whereas eGFR remained stable in adults but increased significantly in children (90.6 to 110 ml/min/1.73m2).
Conclusion:
Proteinuria in children with IgAN is a marker of glomerular proliferative lesions whereas its presence in adults often reflects the presence of chronic lesions. This suggests the need for histological assessment.
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