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Kidney Week

Abstract: TH-PO806

Correlation Between Endocapillary Proliferative and Nephrotic-Range Proteinuria in Children with Henoch-Schönlein Purpura Nephritis

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Huang, Yanjie, The First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Zhengzhou, China
  • Yang, Xiaoqing, The First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Zhengzhou, China
Background

The endocapillary proliferative (EP) lesion is not included in ISKDC pathological classification of 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. 24-hour proteinuria was measured by pyrogallol red-molybdate. Urinary IgG, transferrin and albumin levels were measured by immunonephelometry. The correlations between EP lesion and 24-hour proteinuria, urinary IgG, urinary transferrin and urinary albumin were analyzed. Renal biopsy specimens were immunohistochemically stained for nephrin and podocalyxin.

Results

Of 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 EP, IVb, and pure EP type. Among these types, pure EP type accounted for 7.4%. The levels of 24-hour proteinuria and urinary albumin were the highest in EP type among all pathological types, and the percentage of EP correlated with 24-hour proteinuria and urinary albumin levels. 24-hour proteinuria was significantly higher in pure 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.

Conclusion

In addition to mesangial proliferation and crescent formation, 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.