Lupus nephritis in Egyptian children: a 16-year experience

Lupus nephritis in Egyptian children: a 16-year experience
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DOI:
10.1007/s40620-014-0157-x
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发表时间:
2015-10
影响因子:
3.4
通讯作者:
Atef Elmougy;A. Sarhan;A. Hammad;A. El-refaey;M. Zedan;R. Eid;Wafaa Limon;A. A. Elrahman-A.;Fatma Elhussieni;Enas El-Sherbeny;A. Bakr
Atef Elmougy;A. Sarhan;A. Hammad;A. El-refaey;M. Zedan;R. Eid;Wafaa Limon;A. A. Elrahman-A.;Fatma Elhussieni;Enas El-Sherbeny;A. Bakr
中科院分区:
医学3区
文献类型:
--
作者:
Atef Elmougy;A. Sarhan;A. Hammad;A. El-refaey;M. Zedan;R. Eid;Wafaa Limon;A. A. Elrahman-A.;Fatma Elhussieni;Enas El-Sherbeny;A. Bakr

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我们回顾了1997年1月至2012年12月在曼苏拉大学儿童医院儿科肾脏科狼疮诊所进行随访的儿童和青少年狼疮性肾炎(LN)的临床特征、组织病理学模式、治疗方式和结果。在194例确诊为系统性红斑狼疮(SLE)的患者中,报告LN 136例(70%),其中男性27例(20%),女性109例(80%)。平均发病年龄12.5±12.9岁,随访2个月~12个月,平均4.1个月。血尿79例(58%),蛋白尿126例(92.6%),其中肾病38例,肾损害20例(15%)。肾活检132例,II级23例,III级25例,IV级39例,V级2例。再次肾活检58例(首次活检不足2例,随诊45例,狼疮发作8例,治疗无效3例),第三次活检仅8例。类固醇是最常见的初始药物;128例患者使用了类固醇(63例单独使用,65例与其他药物一起使用),患者使用了环磷酰胺。最后一次随访时,失访的患者占19%,完全缓解的占45%,仍有活动性疾病的占21%,有终末期肾病的占1%,死亡的占11%。总之,在埃及,儿童系统性红斑狼疮在发病时与频繁和严重的肾炎有关,这可能归因于遗传、种族或环境因素。
We retrospectively evaluated the clinical features, histo-pathological patterns, treatment modalities, and outcome of children and adolescents with lupus nephritis (LN), followed-up in Lupus Clinic, Pediatric Nephrology Unit, Mansoura University Children’s Hospital between January 1997 and December 2012. Out of 194 patients diagnosed with systemic lupus erythematosus (SLE), LN was reported in 136 (70 %) patient, they were 27 males (20 %) and 109 females (80 %). The mean age at presentation was 12.5 ± 2.9 years, the mean duration of follow up was 4.1 years (range 2 months–12 years). Hematuria was present in 79 patients (58 %), proteinuria in 126 (92.6 %), 38 of them were in nephrotic range, while renal impairment was documented in 20 patients (15 %). Renal biopsy was done in 132 patients; diagnosis of class II, III, IV, V were 23, 25, 39, and 2 % respectively. Second renal biopsy was indicated in 58 patients (insufficient first biopsy 2, follow up in 45, lupus flare in 8, no response to therapy in 3 patients), while the third one was needed in only eight patients. Steroids were the commonest initial medications; used in 128 patients (63 alone and 65 with others) and cyclophosphamide was used in 64 patients. At the last follow-up visit; 19 % of patients lost follow-up, 45 % had complete remission, 21 % still had active disease, 1 % had end-stage renal disease, and 11 % died. In conclusion, in Egypt, childhood SLE is associated with frequent and severe nephritis at presentation, and this may be attributed to genetic, ethnic, or environmental factors.