儿童肾病综合征型狼疮性肾炎30例临床、病理及近期疗效观察

doi:10.6040/j.issn.1671-7554.0.2019.1268

摘要/Abstract

摘要： 目的总结儿童肾病综合征(NS)型狼疮性肾炎(LN)的临床、病理特点,分析治疗方案与近期疗效,为NS型LN患儿的诊治提供参考。方法收集2009年1月1日至2018年12月31日确诊为NS型LN患儿的临床资料,进行回顾性分析。结果 107例LN患儿中,NS型共30例,占28.0%。发病年龄5~13岁,平均(9.7±2.3)岁。其中男6例,女24例,男女比例为1∶4。最常见的肾外表现为贫血。与非NS型LN患儿相比,NS型LN患儿合并急性肾损伤与高血压比例高、白细胞减少比例低(P<0.001;P<0.001; P=0.01)。行肾穿刺活检22例,最常见的病理类型为Ⅳ型,共12例(54.5%),其次为Ⅴ型5例(22.7%)、Ⅱ型3例(13.6%)、Ⅴ+Ⅱ型与Ⅴ+Ⅳ型各1例(4.5%)。30例患儿均给予泼尼松联合免疫抑制剂治疗,其中大剂量甲泼尼龙(MMP)联合环磷酰胺(CTX)冲击19例(63.3%),MMP冲击后序贯吗替麦考酚酯(MMF)1例(3.3%),仅用CTX冲击3例(10.0%),MMF联合他克莫司2例(6.7%),仅MMF 1例(3.3%),用药不依从4例(13.3%)。治疗6个月,完全缓解17例,部分缓解8例,未缓解4例,死亡1例,死亡原因为合并重症肺炎与严重脓毒症。结论 NS型LN患儿合并急性肾损伤与高血压比例高于非NS型,病理类型具有多样性,弥漫增生性Ⅳ型是主要的病理类型。MMP联合CTX冲击是临床表现为NS型或病理表现为Ⅳ型LN患儿主要的诱导治疗方案,可有效诱导缓解。

关键词: 狼疮性肾炎, 肾病综合征, 临床, 近期疗效, 儿童

Abstract: Objective To summarize the clinical and pathological characteristics of children with nephrotic syndrome(NS)in clinical trials of lupus nephritis(LN), analyze the treatment plan and prognosis, and provide reference for the diagnosis and treatment. Methods Clinical data of children diagnosed with NS-type LN were collected for retrospective analysis from January 1, 2009 to December 31, 2018. Results NS was found in 30 cases(28.0%)of 107 cases with LN. The ages of onset were 5-13 years, average(9.7±2.3)years. Among them, there were 6 males and 24 females with a male to female ratio of 1∶4. The most common extrarenal manifestation is anemia. Compared with non-NS-type LN patients, the proportion of acute kidney injury and hypertension was higher and the proportion of leukopenia was lower in NS-type LN patients(P<0.001;P<0.001; P=0.01). Renal biopsy showed the most common is class Ⅳ(54.5%), followed by class Ⅴ(22.7%), Ⅱ(13.6%), Ⅴ + Ⅱ(4.5%)and Ⅴ + Ⅳ(4.5%)in 22 cases. Thirty cases were given prednisone plus immunosuppressant, including intravenous methylprednisolone pulses(MMP)combined with cyclophosphamide(CTX)(63.3%), sequential usage of mycophenolate mofetil(MMF)after MMP(3.3%), only with CTX(10.0%), MMF combined with tacrolimus(6.7%), only with MMF(3.3%), and poor medication 山东大学学报 (医学版)58卷2期 -阚娜娜,等.儿童肾病综合征型狼疮性肾炎30例临床、病理及近期疗效观察 \=-compliance(13.3%). After 6 months of treatment, 17 cases had complete remission, 8 cases had partial remission, 4 cases had no remission, and 1 case had died of severe pneumonia and severe sepsis. Conclusion The proportion of acute kidney injury and high blood pressure in NS-type LN patients is high than those in non-NS-type LN patients. In NS-type LN patients, the renal pathology is complex, and class Ⅳ type is the main pathological type. Among the patients with NS-type LN or pathological type of Ⅳ, MMP joint CTX is the main induction therapy, which can effectively induce remission.

Key words: Lupus nephritis, Nephrotic syndrome, Clinical, Prognosis, Children

中图分类号:

R725

阚娜娜,余丽春,孙书珍,李倩,栾春丽. 儿童肾病综合征型狼疮性肾炎30例临床、病理及近期疗效观察[J]. 山东大学学报 (医学版), 2020, 58(2): 54-59.

KAN Nana, YU Lichun, SUN Shuzhen, LI Qian, LUAN Chunli. Clinics, pathology and prognosis of 30 children with nephrotic syndrome in clinical trials of lupus nephritis[J]. Journal of Shandong University (Health Sciences), 2020, 58(2): 54-59.

参考文献 30

[1]	Harry O, Yasin S, Brunner H. Childhood-onset systemic lupus erythematosus: a review and update[J]. J Pediatr, 2018, 196: 22-30. e2. doi: 10.1016/j.jpeds.2018.01.045.
[2]	Hiraki LT, Benseler SM, Tyrrell PN, et al. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study[J]. J Pediatr, 2008, 152(4): 550-556.
[3]	Zhang CX, Cai L, Zhou ZY, et al. Clinical manifestations, immunological features and prognosis of Chinese pediatric systemic lupus erythematosus: a single-center study[J]. Int J Rheum Dis, 2019, 22(6): 1070-1076.
[4]	Gomez Mendez LM, Cascino MD, Katsumoto TR, et al. Outcome of participants with nephrotic syndrome in combined clinical trials of lupus nephritis[J]. Lupus Sci Med, 2019, 6(1): e000308. doi: 10.1136/lupus-2018-000308.
[5]	成学琴,鲍华英,张爱华,等. 儿童狼疮性肾炎45例临床、病理及免疫学分析[J]. 临床儿科杂志, 2014, 32(3): 246-249. CHENG Xueqin, BAO Huaying, ZHANG Aihua, et al. Clinical and immunological features of 45 children with lupus nephritis[J]. J Clin Pediatr, 2014, 32(3): 246-249.
[6]	Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus[J]. Arthritis Rheum, 2012, 64(8): 2677-2686.
[7]	Gipson DS, Massengill SF, Yao L, et al. Management of childhood onset nephrotic syndrome[J]. Pediatrics, 2009, 124(2): 747-757.
[8]	Weening JJ, DAgati VD, Schwartz MM, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited[J]. J Am Soc Nephrol, 2004, 65(2): 521-530.
[9]	Radhakrishnan J, Cattran DC. The KDIGO practice guideline on glomerulonephritis: reading between the(guide)lines - application to the individual patient[J]. Kidney Int, 2012, 82(8): 840-856.
[10]	Abdel-Nabi HH, Abdel-Noor RA. Comparison between disease onset patterns of Egyptian juvenile and adult systemic lupus erythematosus(single centre experience)[J]. Lupus, 2018, 27(6): 1039-1044.
[11]	Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents[J]. Pediatr Clin North Am, 2012, 59(2): 345-364.
[12]	Lionaki S, Liapis G, Boletis JN. Pathogenesis and management of acute kidney injury in patients with nephrotic syndrome due to primary glomerulopathies[J]. Medicina(Kaunas), 2019, 55(7). pii: E365. doi: 10.3390/medicina55070365.
[13]	Jin SY, Huang DL, Dang XQ, et al. Lupus glomerulonephritis in 788 Chinese children: a multi-centre clinical and histopathological analysis based on 549 renal biopsies[J]. Paediatr Int Child Health, 2017, 37(4): 286-291.
[14]	Mahmoud SS, Bazaraa HM, Lotfy HM, et al. Renal involvement in childhood-onset systemic lupus erythematosus in Egypt[J]. Rheumatol Int, 2012, 32(1): 47-51.
[15]	Cabral M, Escobar C, Conde M, et al. Juvenile systemic lupus erythematosus in Portugal: clinical and immunological patterns of disease expression in a cohort of 56 patients[J]. Acta Reumatol Port, 2013, 38(4): 274-285.
[16]	Abdel-Hafez MA, Abdel-Nabi H. Juvenile systemic lupus erythematosus: onset patterns and short-term outcome in Egyptian children, a single-center experience[J]. Lupus, 2015, 24(13): 1455-1461.
[17]	Fayyaz A, Igoe A, Kurien BT, et al. Haematological manifestations of lupus[J]. Lupus Sci Med, 2015, 2(1): e000078. doi: 10.1136/lupus-2014-000078.
[18]	Gigante A, Barbano B, Sardo L, et al. Hypercoagulability and nephrotic syndrome[J]. Curr Vasc Pharmacol, 2014, 12(3): 512-517.
[19]	Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease[J]. Clin J Am Soc Nephrol, 2012, 7(3): 513-520.
[20]	Perico N, Remuzzi G. Edema of the nephrotic syndrome: the role of the atrial peptide system[J]. Am J Kidney Dis, 1993, 22(3): 355-366.
[21]	Appenzeller S, Cendes F, Costallat LT. Epileptic seizures in systemic lupus erythematosus[J]. Neurology, 2004, 63(10): 1808-1812.
[22]	Mikdashi J, Krumholz A, Handwerger B. Factors at diagnosis predict subsequent occurrence of seizures in systemic lupus erythematosus[J]. Neurology, 2005, 64(12): 2102-2107.
[23]	Hanly JG, Urowitz MB, Su L, et al. Seizure disorders in systemic lupus erythematosus results from an international, prospective, inception cohort study[J]. Ann Rheum Dis, 2012, 71(9): 1502-1509.
[24]	Copple SS, Sawitzke AD, Wilson AM, et al. Enzyme-linked immunosorbent assay screening then indirect immunofluorescence confirmation of antinuclear antibodies: a statistical analysis[J]. Am J Clin Pathol, 2011, 135(5): 678-684.
[25]	Katsuno T, Ozaki T, Ozeki T, et al. Investigation on the benefits of mycophenolate mofetil and therapeutic drug monitoring in the treatment of Japanese patients with lupus nephritis[J]. Clin Exp Nephrol, 2018, 22(6): 1341-1350.
[26]	Lau KK, Ault BH, Jones DP, et al. Induction therapy for pediatric focal proliferative lupus nephritis: cyclophosphamide versus mycophenolate mofetil[J]. J Pediatr Health Care, 2008, 22(5): 282-288.
[27]	Smith E, Al-Abadi E, Armon K, et al. Outcomes following mycophenolate mofetil versus cyclophosphamide induction treatment for proliferative juvenile-onset lupus nephritis[J]. Lupus, 2019, 28(5): 613-620.
[28]	Ginzler EM, Dooley MA, Aranow C, et al. Mycophenolate mofetil or intravenous cyclophosphamide for lupus nephritis[J]. N Engl J Med, 2005, 353(21): 2219-2228.
[29]	An Y, Zhou Y, Bi L, et al. Combined immunosuppressive treatment(CIST)in lupus nephritis: a multicenter, randomized controlled study[J]. Clin Rheumatol, 2019, 38(4): 1047-1054.
[30]	孙利,徐虹,刘海梅,等.上海单中心101例儿童狼疮性肾炎的长期随访分析[J].中华儿科杂志, 2011, 49(11): 819-824. SUN Li, XU Hong, LIU Haimei, et al. Long-term follow-up of 101 cases with pediatric lupus nephritis in a single center in Shanghai[J]. Chin J Pediatr, 2011, 49(11): 819-824.

相关文章 15

[1]	山东省药学会. 药物临床试验用药品管理山东专家共识[J]. 山东大学学报 (医学版), 2026, 64(4): 8-13.
[2]	李东梅,边天帅,王晓华,张静,赵炎,孙素芬,牟鸿,许安廷. 儿童前庭神经炎的前庭诱发肌源性电位结果[J]. 山东大学学报 (医学版), 2026, 64(4): 38-43.
[3]	王兴山,张浩天,张云峰,周一新. 旋转铰链膝在复杂初次膝关节置换中的应用及临床疗效[J]. 山东大学学报 (医学版), 2026, 64(4): 51-57.
[4]	孙红林,管玉强,于培儒,李磊,杜庆霞,丁浩. 支架植入治疗儿童主动脉缩窄并高血压的可行性及近中期疗效[J]. 山东大学学报 (医学版), 2026, 64(4): 58-62.
[5]	况利,徐何雁. 从精准评估到整合干预:儿童青少年心理健康问题诊疗范式的革新与展望[J]. 山东大学学报 (医学版), 2026, 64(3): 10-16.
[6]	李芳华,刘娜,李晓宇,许殿花,王倩. 儿童青少年抑郁障碍患者服药依从性研究进展[J]. 山东大学学报 (医学版), 2026, 64(3): 24-34.
[7]	孙佳丽,王亮,王长凤,张杰,郭磊. Klippel-Trenaunay综合征儿童情绪与行为特征及其影响因素[J]. 山东大学学报 (医学版), 2026, 64(3): 78-82.
[8]	王倩,胡晓婧,王爱青,孙强,孟春燕,李芳华. 儿童青少年抑郁障碍患者非自杀性自伤文献的可视化分析[J]. 山东大学学报 (医学版), 2026, 64(3): 93-107.
[9]	黄佩文, 王旭东. 治疗非小细胞肺癌新药:靶向c-Met蛋白的抗体药物偶联物Telisotuzumab Vedotin[J]. 山东大学学报 (医学版), 2026, 64(3): 124-130.
[10]	机器人辅助经椎间孔腰椎椎体间融合术手术技术专家组. 机器人辅助经椎间孔腰椎椎体间融合术专家共识[J]. 山东大学学报 (医学版), 2026, 64(2): 11-21.
[11]	王悦祺,宋东玉,李玉丽,谢昊,张月蓉. 儿童困难问题和亲社会行为在家庭弹性与祖辈隔代教养压力间的中介作用[J]. 山东大学学报 (医学版), 2026, 64(1): 57-64.
[12]	李千,杨帆,薛付忠. 基于多模态数据融合的多癌种风险预测模型[J]. 山东大学学报 (医学版), 2025, 63(8): 79-85.
[13]	罗淇,王霞,姜孟. 脑功能网络分析在失语症诊疗中的应用:病理机制分析、临床诊断与疗效评价[J]. 山东大学学报 (医学版), 2025, 63(8): 111-126.
[14]	张政,王建伟,杨玉娟,张宇,宋西成. 哮喘儿童2008及2019年免疫球蛋白E变化及相关危险因素[J]. 山东大学学报 (医学版), 2025, 63(7): 32-36.
[15]	孙相洁,尹海燕,刘春兰,赵风娥. 基于潜在剖面分析的临床护理带教老师感知上级社会支持类型及与工作投入的关系[J]. 山东大学学报 (医学版), 2025, 63(7): 109-115.

多维度评价

Viewed

Full text

Abstract

Cited

Shared

Discussed