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儿童狼疮性肾炎

Lupus nephritis in children.

作者信息

Hafeez Farkhanda, Tarar Abid Mahmood, Saleem Rashid

机构信息

Department of Nephrology, The Children's Hospital and Institute of Child Health, Lahore,Pakistan.

出版信息

J Coll Physicians Surg Pak. 2008 Jan;18(1):17-21.

Abstract

OBJECTIVE

To determine the clinicopathological pattern of lupus nephritis in paediatric nephrology patients.

DESIGN

Case series study.

PLACE AND DURATION OF STUDY

The department of paediatric nephrology at the Children's Hospital and Institute of Child Health, Lahore, Pakistan, over a period of five years from January 2001 to December 2005.

PATIENTS AND METHODS

Twenty six patients upto the age 16 years of either gender, with a mean age of 12.4 +/- 1.90 years having primary SLE with renal involvement in the form of oedema, hypertension, haematuria and proteinuria were included. Twenty one were females. Percutaneous renal biopsy was performed. Histological lesion was classified according to WHO classification. Patients were treated with immunosuppressive therapy and their clinical course was followed for at least one year. The mean duration of follow up was 1.77 years.

RESULTS

Renal involvement was seen in 92.30% within 2 years of the onset of primary disease. Diffuse proliferative glomerulonephritis was the commonest histological lesion (n=14) followed by membranous nephropathy (n=6). The commonest clinical manifestation was oedema (80.76%) followed by hypertension (46.15%). Proteinuria was present in 100% of cases, haematuria in 38.46% and azotemia in 19.33% of patients. Nephrotic range proteinuria was more common in class III and IV, while azotemia was observed only in class IV. The disease was well controlled in 73.07% , relapse was seen in 3.8% of patients, 15.38% died of infections and uremic encephalopathy while 7.69% were lost to follow-up.

CONCLUSION

Diffuse proliferative glomerulonephritis is the commonest histological lesion in our set-up. Renal involvement is mostly seen within first two years of the primary disease which can be controlled satisfactorily with immunosuppressive therapy.

摘要

目的

确定儿科肾脏病患者狼疮性肾炎的临床病理模式。

设计

病例系列研究。

研究地点和时间

巴基斯坦拉合尔儿童医院及儿童健康研究所儿科肾脏病科,从2001年1月至2005年12月的五年期间。

患者和方法

纳入26例年龄在16岁及以下的患者,男女不限,平均年龄为12.4±1.90岁,患有原发性系统性红斑狼疮且伴有以水肿、高血压、血尿和蛋白尿形式出现的肾脏受累。其中21例为女性。进行了经皮肾活检。组织学病变根据世界卫生组织分类进行分类。患者接受免疫抑制治疗,并对其临床病程进行了至少一年的随访。平均随访时间为1.77年。

结果

在原发性疾病发病后2年内,92.30%的患者出现肾脏受累。弥漫性增殖性肾小球肾炎是最常见的组织学病变(n = 14),其次是膜性肾病(n = 6)。最常见的临床表现是水肿(80.76%),其次是高血压(46.15%)。100%的病例存在蛋白尿,38.46%的患者有血尿,19.33%的患者有氮质血症。肾病范围蛋白尿在III级和IV级中更常见,而氮质血症仅在IV级中观察到。73.07%的疾病得到良好控制,3.8%的患者复发,15.38%的患者死于感染和尿毒症性脑病,7.69%的患者失访。

结论

在我们的研究中,弥漫性增殖性肾小球肾炎是最常见的组织学病变。肾脏受累大多在原发性疾病的头两年内出现,免疫抑制治疗可使其得到满意控制。

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