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World J Pediatr. 2015 Nov;11(4):338-45. doi: 10.1007/s12519-014-0527-4. Epub 2014 Nov 20.

Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children.

Huang YJ1,2, Yang XQ3,4, Zhai WS3,4, Ren XQ3,4, Guo QY3,4, Zhang X3,4, Yang M3,4, Yamamoto T3,5, Sun Y3,6, Ding Y3,4.

Author information

1
, Zhengzhou, China. huangyanjie69@hotmail.com.
2
Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China. huangyanjie69@hotmail.com.
3
, Zhengzhou, China.
4
Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
5
Second Department of Medicine, Numazu City Hospital, Numazu, Japan.
6
Department of Anesthesia, Stanford University School of Medicine, California, USA.

Abstract

BACKGROUND:

The aim of this retrospective study was to define the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like 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 ± 2.03 years, mean ± SD). The clinical features, laboratory and pathological findings, 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.

KEYWORDS:

Henoch-Schönlein purpura nephritis; clinicopathological features; prognosis

PMID:
25410673
DOI:
10.1007/s12519-014-0527-4
[Indexed for MEDLINE]

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