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博茨瓦纳玛丽娜公主医院肾病综合征患儿的临床特征、病理特征及治疗结果

Clinical Characteristics, Pathological Features and Treatment Outcomes of Children With Nephrotic Syndrome at Princess Marina Hospital, Botswana.

作者信息

Mosalakatane Thembisile Dintle, Gottlich Errol, Mazhani Loeto, Joel Dipesalema, Mogotsi Thabiso Vivien, Arscott-Mills Tonya

机构信息

Department of Paediatrics and Adolescent Health, Faculty of Medicine, University of Botswana, Gaborone, Botswana.

University of Pretoria, Morningside Hospital, Johannesburg, South Africa.

出版信息

Glob Pediatr Health. 2024 Oct 5;11:2333794X241285272. doi: 10.1177/2333794X241285272. eCollection 2024.

DOI:10.1177/2333794X241285272
PMID:39376930
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11457277/
Abstract

Despite the remarkable progress made in the understanding and treatment of childhood nephrotic syndrome (NS), a lot is still unknown about its epidemiology in many African countries. This study sought to determine the clinicopathological features and treatment outcomes of children with NS at the largest tertiary hospital in Botswana. A retrospective study of 26 children with NS treated from 2009 to 2014 was conducted. Mean age at presentation was 5.96 ± 3.06. Hematuria was found in 92.3%, low C3 in 26.7%, high creatinine in 19.2% and hypertension in 46.2% of the patients. 92.3% had primary NS out of which 23.1% had familial NS. HIV, tuberculosis and hepatitis B infections were diagnosed in 3.85%, 9.09% and 4.16% respectively. 69.2% had steroid-resistant nephrotic syndrome, focal segmental glomerulosclerosis (SRNS-FSGS). The frequency of primary SRNS-FSGS and familial SRNS appears to be much higher in Botswana highlighting the possibility of genetic causes.

摘要

尽管在儿童肾病综合征(NS)的认识和治疗方面取得了显著进展,但在许多非洲国家,其流行病学仍有很多未知之处。本研究旨在确定博茨瓦纳最大的三级医院中NS患儿的临床病理特征和治疗结果。对2009年至2014年治疗的26例NS患儿进行了回顾性研究。就诊时的平均年龄为5.96±3.06岁。92.3%的患者出现血尿,26.7%的患者C3降低,19.2%的患者肌酐升高,46.2%的患者患有高血压。92.3%的患者患有原发性NS,其中23.1%患有家族性NS。分别有3.85%、9.09%和4.16%的患者被诊断出感染艾滋病毒、结核病和乙型肝炎。69.2%的患者患有类固醇抵抗性肾病综合征,局灶节段性肾小球硬化(SRNS-FSGS)。在博茨瓦纳,原发性SRNS-FSGS和家族性SRNS的发生率似乎要高得多,这突出了遗传因素的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab3/11457277/2120820cabbe/10.1177_2333794X241285272-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab3/11457277/2120820cabbe/10.1177_2333794X241285272-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab3/11457277/2120820cabbe/10.1177_2333794X241285272-fig1.jpg

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本文引用的文献

1
Hiding in plain sight: genetics of childhood steroid-resistant nephrotic syndrome in Sub-Saharan Africa.隐匿于众目睽睽之下:撒哈拉以南非洲儿童类固醇耐药性肾病综合征的遗传学。
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New Clinical Practice Guideline for the Management of High Blood Pressure in Children and Adolescents.
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Evidence-based clinical practice guidelines for nephrotic syndrome 2014.2014年肾病综合征循证临床实践指南
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Trends in the histopathology of childhood nephrotic syndrome in Ibadan Nigeria: preponderance of idiopathic focal segmental glomerulosclerosis.尼日利亚伊巴丹儿童肾病综合征的组织病理学趋势:特发性局灶节段性肾小球硬化症占优势
BMC Nephrol. 2015 Dec 15;16:213. doi: 10.1186/s12882-015-0208-0.
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Distribution of pathological finding in the children with nephrotic syndrome from Guangxi.广西肾病综合征患儿的病理 findings 分布。 (注:这里“findings”结合语境推测可能是“检查结果”之类的意思,原英文表述不太完整准确)
Saudi J Kidney Dis Transpl. 2014 May;25(3):684-8. doi: 10.4103/1319-2442.132240.
7
Pathological profile of biopsied Egyptian children with primary nephrotic syndrome: 15-year single center experience.埃及原发性肾病综合征活检患儿的病理特征:15年单中心经验
J Nephrol. 2014 Aug;27(4):419-23. doi: 10.1007/s40620-013-0032-1. Epub 2014 Jan 16.
8
Patterns of childhood nephrotic syndrome in Aljouf region, Saudi Arabia.沙特阿拉伯阿尔朱夫地区儿童肾病综合征的模式。
Saudi J Kidney Dis Transpl. 2013 Sep;24(5):1050-4. doi: 10.4103/1319-2442.118096.
9
50 years of nephrotic syndrome in children, and hereafter.儿童肾病综合征的50年及未来。
Indian Pediatr. 2013 Jan 8;50(1):107-10. doi: 10.1007/s13312-013-0024-1.
10
Two-year outcome of the ISKDC regimen and frequent-relapsing risk in children with idiopathic nephrotic syndrome.特发性肾病综合征患儿 ISKDC 方案的两年结局和频繁复发风险。
Clin J Am Soc Nephrol. 2013 May;8(5):756-62. doi: 10.2215/CJN.09010912. Epub 2013 Jan 31.