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Correlation between endocapillary proliferative and nephrotic-range proteinuria in children with Henoch-Schönlein purpura nephritis

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Abstract

Background

The endocapillary proliferative (EP) lesion is not included in the International Study of Kidney Disease in Children (ISKDC) pathological classification of Henoch-Schönlein purpura nephritis (HSPN). The main objective of the study was to determine the pathological importance of EP in the development of proteinuria in children with Henoch-Schönlein purpura nephritis (HSPN).

Methods

The pathological features of 148 HSPN children with nephrotic-range proteinuria were investigated retrospectively. Urinary IgG, transferrin, and albumin levels were measured by immunonephelometry. The correlations between EP lesion and 24-h proteinuria, urinary IgG, urinary transferrin, and urinary albumin were analyzed. Renal biopsy specimens were immunohistochemically stained for nephrin and podocalyxin.

Results

Of the total 581 cases of children with HSPN who underwent renal biopsy, 148 cases (25.5%) presented with nephrotic-range proteinuria. The pathological types of HSPN with nephrotic-range proteinuria were categorized as IIb, IIIa, IIIb, IIIb with diffuse EP, IVb, pure focal EP type, and pure diffuse EP type. Among these types, pure diffuse EP type accounted for 7.4%. The levels of 24-h proteinuria and urinary albumin were the highest in pure diffuse EP type among all pathological types, and the percentage of EP correlated with 24-h proteinuria and urinary albumin levels. 24-h proteinuria was significantly higher in pure diffuse EP type relative to HSPN IIb type, and significantly higher in IIIb with EP, compared with HSPN IIIb. Nephrin, but not podocalyxin, was downregulated in EP segment.

Conclusions

EP is an independent pathogenic factor in HSPN with nephrotic-range proteinuria. Downregulation of nephrin in EP segment is a potential molecular mechanism of nephrotic-range proteinuria. Albumin is the major urinary protein component in HSPN with EP.

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Funding

This study was funded by independent innovation and applied basic research project of university basic scientific research business in 2014 (No. 2014KYYWF-ZZCX2-03) and innovation team for treatment of Henoch-Schönlein purpura nephritis in children by Integrated Chinese and Western medicine methods (No. 18IRTSTHN028).

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Author notes

  1. Yanjie Huang and Xiaoqing Yang have contributed equally to the work and are both to be considered first authors

Authors and Affiliations

  1. Department of Pediatrics, The First Affiliated Hospital of Henan University of Traditional Chinese Medicine, 19 Renmin Road, Zhengzhou, 450000, Henan, China

    Xiao-qing Yang, Yan-jie Huang, Wen-sheng Zhai, Xian-qing Ren, Qing-yin Guo, Xia Zhang, Meng Yang, Jian Zhang & Ying Ding

  2. Department of Pediatrics, The Second Affiliated Hospital of Henan University of Traditional Chinese Medicine, 6 Dongfeng Road, Zhengzhou, 450000, Henan, China

    Shan Zhu

  3. Division of Nephrology, Fujieda Municipal General Hospital, Shizuoka, Japan

    Tatsuo Yamamoto

  4. Department of Anesthesia, Stanford University School of Medicine, Stanford, CA, USA

    Yuan Sun

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  1. Xiao-qing Yang
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  10. Shan Zhu
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Correspondence to Yan-jie Huang or Shan Zhu.

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Yang, Xq., Huang, Yj., Zhai, Ws. et al. Correlation between endocapillary proliferative and nephrotic-range proteinuria in children with Henoch-Schönlein purpura nephritis. Pediatr Nephrol 34, 663–670 (2019). https://doi.org/10.1007/s00467-018-4134-9

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  • DOI: https://doi.org/10.1007/s00467-018-4134-9

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