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28.01.2020 | Original Article

Crescentic IgA nephropathy in children

verfasst von: Yuko Shima, Koichi Nakanishi, Taketsugu Hama, Hironobu Mukaiyama, Masashi Sato, Yu Tanaka, Ryojiro Tanaka, Hiroshi Kaito, Kandai Nozu, Mayumi Sako, Kazumoto Iijima, Norishige Yoshikawa

Erschienen in: Pediatric Nephrology | Ausgabe 6/2020

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Abstract

Objectives

Crescentic IgA nephropathy (C-IgAN) is defined as IgAN with more than 50% of glomeruli showing crescents. C-IgAN in children is rare; we investigate in detail for the first time.

Methods

We retrospectively analyzed the 515 consecutive children who were newly diagnosed with biopsy-proven IgAN between June 1976 and May 2010. We compared clinical and pathological findings between C-IgAN and non-C-IgAN.

Results

Among 515 cases of childhood IgAN, 25 children (4.9%) had C-IgAN. Of these 25, 16 children (64%) were referred to hospitals by annual school screening. Clinical findings showed significant differences in gross hematuria (76 vs. 50%, p = .03), excretion of proteinuria (1.9 vs. 0.5 g/day/m², p < .0001), eGFR (102 vs. 108 ml/min/1.73 m², p = .03), and duration from onset to renal biopsy (4.0 vs. 8.0 months, p = .04) between groups. Pathological findings showed significant differences in M1 (88 vs. 55%, p = .02), E1 (83 vs. 53%, p = .008), and presence of tubular atrophy/interstitial fibrosis (88 vs. 53%, p < .0001) between groups. The 16 children with C-IgAN were treated with prednisolone and immunosuppressant. Four cases (16%) reached chronic renal failure (eGFR < 60) at the latest observation (mean observation period: 6.0 ± 3.6 years). Patients with C-IgAN had significantly lower renal survival curve than non-C-IgAN patients according to Kaplan-Meier analysis (77.1% vs. 92.6% at 13 years, p < .0001). Compared with previous reports, however, they had better renal outcome.

Conclusions

We confirmed the importance of school screening to find C-IgAN. Although most crescents (mean: 98.1%) of C-IgAN were cellular/fibrocellular, and acute lesions were well modified with combination therapy, the presence of tubular atrophy in C-IgAN may be the reason for poorer prognosis.

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Titel: Crescentic IgA nephropathy in children
verfasst von: Yuko Shima
Koichi Nakanishi
Taketsugu Hama
Hironobu Mukaiyama
Masashi Sato
Yu Tanaka
Ryojiro Tanaka
Hiroshi Kaito
Kandai Nozu
Mayumi Sako
Kazumoto Iijima
Norishige Yoshikawa
Publikationsdatum: 28.01.2020
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 6/2020
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-020-04483-w

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Crescentic IgA nephropathy in children

Abstract

Objectives

Methods

Results

Conclusions

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Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

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Stillende Frauen haben geringeres kardiovaskuläres Risiko

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Live-Webinar: Aktuelle Leitlinien bei Herz-Kreislauf-Erkrankungen

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Abstract

Objectives

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Results

Conclusions

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