糖皮质激素治疗儿童非腹泻相关溶血尿毒综合征的疗效评价

doi:10.13481/j.1671-587x.20190532

摘要/Abstract

摘要： 目的：探讨应用糖皮质激素治疗儿童非腹泻相关溶血尿毒综合征（HUS）临床症状改善的时间，评估其疗效。方法：选择22例非腹泻相关HUS患儿作为研究对象。根据患儿急性期在使用血浆的同时是否联合使用糖皮质激素，将患儿分为单纯血浆治疗组（n=11）和血浆联合糖皮质激素治疗组（n=11），分析2组患儿平均住院时间、尿色转清时间、血小板及血肌酐恢复正常水平的时间，并收集2组患儿出院后的临床资料，根据随访结果评估2组患儿的预后。结果：与单纯血浆治疗组比较，血浆联合糖皮质激素治疗组患儿平均住院时间、尿色转清时间和血肌酐恢复正常水平时间均缩短，但差异无统计学意义（P>0.05）。2组患儿血小板升至正常水平时间比较差异无统计学意义（P>0.05）。单纯血浆治疗组有10例患儿顺利完成随访，其中1例患儿遗留高血压后遗症，1例患儿在出院1年后病情复发；血浆联合糖皮质激素治疗组9例患儿顺利完成随访，其中1例患儿持续尿检异常，并遗留听力受损，生长发育较同龄儿缓慢，其余随访患儿达到临床治愈标准。单纯血浆治疗组临床治愈率为80.0%，血浆联合糖皮质激素治疗组临床治愈率为88.9%，组间临床治愈率比较差异无统计学意义（P>0.05）。结论：在儿童非腹泻相关HUS的急性期使用糖皮质激素并不能缩短患儿的急性期病程，也不能改善患儿的预后。

关键词: 溶血尿毒综合征, 糖皮质激素, 血栓性微血管病变, 血浆, 儿童

Abstract: Objective:To investigate the clinical symptom improvement time of the children with non-diarrhea-related hemolytic uremic syndrome(HUS) treated with glucocorticoids,and to evaluate its efficacy. Methods:A total of 22 children with non-diarrhea-related HUS were selected as the subjects. According to whether glucocorticoid was used in combination of plasma in the actue stage of the children, the children were divided into simple plasma group (n=11) and plasma combined with glucocorticoid treatment group (n=11). The average hospital stays, the time of hematuria, the time of platelets and serum creatinine to recover to the normal levels of the children in two groups were analyzed. The clinical material of children in two groups after hospital discharge were collected,and the prognosis of the children in two groups was evaluated according to the follow-up results. Results:Compared with simple plasma group, the average hospital stays,and the time of hematuria and the time of serum creatinine to recover to the normal level of the children in plasma combined with glucocorticoid treatment group were shortened,but there were no significant differences(P>0.05). The time of platelets rising to the normal level was similar had no significant difference between two groups(P>0.05). Ten cases completed the follow-up in simple plasma group;one children suffered hypertension during follow-up,and one case replapsed 1 year after operation.In plasma combined with glucocorticoid treatment group, nine cases completed the follow-up;one children persistented urinalysis abnormalities and hearing loss,with the growth and development being slower than their peers;the remaining children all achieved the clinical cure standards. The clinical cure rate of the chidren in plasma combined with glucocorticoid treatment group was 88.9%,while it was 80.0% in simple plasma group; there was no significant difference in the clinical cure rate between two groups(P>0.05). Conclusion:Using glucocorticoids in the acute stage of non-diarrhea-related hemolytic uremic syndrome in the children could not shorten the acute course and improve the prognosis of the children.

Key words: hemolytic uremic syndrome, glucocorticoids, thrombotic microangiopathy, plasma, children

中图分类号:

R725.9

盛爱芹, 傅海东, 张晨美, 叶盛. 糖皮质激素治疗儿童非腹泻相关溶血尿毒综合征的疗效评价[J]. 吉林大学学报(医学版), 2019, 45(05): 1163-1167.

SHENG Aiqin, FU Haidong, ZHANG Chenmei, YE Sheng. Evaluation on efficacy of glucocorticoids in treatment of children with non-diarrhea-related hemolytic uremic syndrome[J]. Journal of Jilin University(Medicine Edition), 2019, 45(05): 1163-1167.

参考文献

[1] RUGGENENTI P, NORIS M, REMUZZI G.Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura[J].Kidney Int,2001,60(3):831-846.
[2] RIVAS M, MILIWEBSKY E, CHINEN I, et al. Characterization and epidemiologic subtyping of Shiga toxin-producing Escherichia coli strains isolated from hemolytic uremic syndrome and diarrhea cases in Argentina[J].Foodborne Pathog Dis,2006,3(1):88-96.
[3] NORIS M, REMUZZI G.Hemolytic uremic syndrome[J].J Am Soc Nephrol,2005,16(4):1035-1050.
[4] LICHT C, GREENBAUM L A, MUUS P, et al. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies[J].Kindey Int,2015,87(5):1061-1073.
[5] GREENBAUM L A, FILA M, ARDISSINO G, et al. Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome[J]. Kidney Int,2016,89(3):701-711.
[6] NAYER A, ASIF A. Atypical hemolytic-uremic syndrome:A clinical review[J]. Am J Ther,2016,23(1):E151-E158.
[7] 国家儿童医学中心,福棠儿童医学发展研究中心aHUS管理协作组.中国儿童非典型溶血尿毒综合征诊治规范专家共识[J]. 中国实用儿科杂志,2017,32(6):401-404.
[8] 葛伟,孙若鹏.小儿溶血尿毒综合征10例临床分析[J].临床儿科杂志, 2007, 25(10):844-847.
[9] WATT T, WARSHAW B, KATZENSTEIN H M, et al. Atypical hemolytic uremic syndrome responsive to steroids and intravenous immune globulin[J]. Pediatr Blood Cancer,2009, 53(1):90-91.
[10] RACHEL L,LOUISE W,NICHOLAS J W.Nephrotic syndrome in children[J].Paediatr Child Health,2010,20(1):36-42.
[11] 邓会英,高岩,李颖杰,等.血浆置换术后联合小剂量激素治疗溶血尿毒综合征[J].实用儿科临床杂志,2012,27(6):412-414,445.
[12] KDIGO AKI Work Group. KDIGO clinical practice guideline for acute kidney injury[J]. Kidney Int Suppl,2012,2(1):1-138.
[13] BESBAS N, KARPMAN D, LANDAU D, et al. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders[J]. Kidney Int,2006,70(3):423-431.
[14] SPINALE J M, RUEBNER R L, COPELOVITCH L, et al. Long-term outcomes of Shiga toxin hemolytic uremic syndrome[J]. Pediatr Nephrol, 2013, 28(11):2097-2105.
[15] JOHNSON S, STOJANOVIC J, ARICETA G, et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome[J]. Pediatr Nephrol,2014,29(10):1967-1978.
[16] CODY E M,DIXON B P. Hemolytic uremic syndrome[J]. Pediatr Clin North Am,2019,66(1):235-246.
[17] 杜悦,吴玉斌,韩梅.血浆置换治疗小儿溶血尿毒综合征疗效及随访研究[J].中国实用儿科杂志,2009,24(12):930-933.
[18] COPPO P, VEYRADIER A, DUREY M A, et al. Pathophysiology of thrombotic microangiopathies:Current understanding[J].Ann Med Interne(Paris),2002,153(3):153-166.
[19] KING A J. Acute inflammation in the pathogenesis of hemolytic-uremic syndrome[J]. Kidney Int,2002,61(4):1553-1564.
[20] 白晓玲,孟建中,王曼秋,等.血浆置换联合连续血液净化救治溶血尿毒综合征对炎症介质的影响[J].山东医药,2009, 49(51):62-63.
[21] SINHA A,GULATI A,SAINI S, et al. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children[J].Kidney Int,2014,85(5):1151-1160.