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儿童肾病综合征的组织病理学诊断与预后

Histopathological diagnosis and outcome of paediatric nephrotic syndrome.

作者信息

Ejaz Iftikhar, Khan Humayun Iqbal, Javaid Bushra Karim, Rasool Ghulam, Bhatti Mohammad Tariq

机构信息

Department of Paediatrics, King Edward Medical College, Lahore.

出版信息

J Coll Physicians Surg Pak. 2004 Apr;14(4):229-33.

Abstract

OBJECTIVE

To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy.

DESIGN

An analytical study.

PLACE AND DURATION OF STUDY

Department of Paediatrics, King Edward Medical College, and Mayo Hospital, Lahore from 1st June 1996 to 31st December, 2001 over a period of 5-1/2 years.

SUBJECTS AND METHODS

Children suffering from nephortic syndrome who had atypical features at presentation were initially or late non-responders; frequent relapsers on < 1mg kg/day and were steroid dependent or frequently relapsed on < 1 mg kg/day but developed steroid toxicity were included. Renal biopsy was performed in these patients. Treatment was administered according to the histopathology reports. Prednisolone 60 mg /m2/day followed after response by 40 mg /m2 on alternate days (AD) which was later tapered off. In minimal change nephrotic syndrome (MCNS) with frequent relapses cyclophosphamide, cyclosporine and levimisole were used. For steroid resistant focal segmental glomerulosclerosis (FSGS) intravenous pulses of methyl-prednisolone and cyclosporine were also given. These patients were followed to see the response of the therapy.

RESULTS

The commonest diagnosis was focal segmental glomerulosclerosis (FSGS) (42%) followed by minimal change disease (MCNS) (22%), membranoproliferative or mesangiocapillary glomerulonephritis (MPGN) (14%) and Mesangioproliferative glomerulonephritis (Mes PGN) (12%). There were 6% cases of membranous nephropathy and 4% of diffuse proliferative glomerulonephritis. On presentation, 40% had hematuria, 20% were found to be hypertensive, 12% patients had renal insufficiency and in 4% C3 level was low. Majority of the patients with MPGN and FSGS had atypical features whereas none of the patients with membranous nephropathy had any of these features. Thirty percent cases each of FSGS and MCNS were responders. Among non-responders there were 4 cases of FSGS and one of MPGN.

CONCLUSION

FSGS was the commonest histology in cases of childhood nephrotic syndrome that needed renal biopsy. Highest frequency of atypical features was seen in MPGN and FSGS.

摘要

目的

确定需要进行肾活检的儿童肾病综合征病例的组织学表现及治疗结果。

设计

一项分析性研究。

研究地点及时间

1996年6月1日至2001年12月31日,在拉合尔爱德华国王医学院儿科学系及梅奥医院,为期5年半。

研究对象及方法

纳入那些在初诊时具有非典型特征、初始或后期治疗无反应、每日每千克体重<1毫克时频繁复发且依赖类固醇治疗,或每日每千克体重<1毫克时频繁复发但出现类固醇毒性的肾病综合征患儿。对这些患者进行肾活检。根据组织病理学报告进行治疗。初始给予泼尼松龙60毫克/平方米/天,出现反应后改为隔日40毫克/平方米,随后逐渐减量。对于频繁复发的微小病变肾病综合征(MCNS),使用环磷酰胺、环孢素和左旋咪唑。对于类固醇抵抗的局灶节段性肾小球硬化(FSGS),还给予甲泼尼龙静脉冲击和环孢素治疗。对这些患者进行随访以观察治疗反应。

结果

最常见的诊断是局灶节段性肾小球硬化(FSGS)(42%),其次是微小病变病(MCNS)(22%)、膜增生性或系膜毛细血管性肾小球肾炎(MPGN)(14%)和系膜增生性肾小球肾炎(Mes PGN)(12%)。有6%的膜性肾病病例和4%的弥漫性增生性肾小球肾炎病例。初诊时,40%有血尿,20%被发现有高血压,12%的患者有肾功能不全,4%的患者C3水平低。大多数MPGN和FSGS患者有非典型特征,而膜性肾病患者均无这些特征。FSGS和MCNS各有30%的病例有反应。在无反应者中,有4例FSGS和1例MPGN。

结论

FSGS是需要进行肾活检的儿童肾病综合征病例中最常见的组织学类型。MPGN和FSGS中非典型特征的发生率最高。

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