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Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children |
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Yan-Jie Huang, Xiao-Qing Yang, Wen-Sheng Zhai, Xian-Qing Ren, Qing-Yin Guo, Xia Zhang, Meng Yang, Tatsuo Yamamoto, Yuan Sun, Ying Ding |
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Background: The aim of this retrospective study was to define the clinical manifestations, pathological features and prognosis of children with membranoproliferativelike Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI.
Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5¡À2.03 years, mean¡ÀSD). The clinical features, laboratory and pathological fi ndings, treatment and outcome of the 9 patients were retrospectively analyzed.
Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients.
Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.
Key words: clinicopathological features; Henoch-Schönlein purpura nephritis; prognosis
World J Pediatr 2015;11(4):338-345
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