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巴基斯坦旁遮普省南部儿科人群中的局灶节段性肾小球硬化症:一家三级医疗医院的经验

Focal Segmental Glomerulosclerosis in Paediatric Population of South Punjab Pakistan: A Tertiary Care Hospital Experience.

作者信息

Safdar Rabia Saleem, Mehar M Faisal, Khan Afsheen Asghar, Buzdar Nusrat

机构信息

Rabia Saleem Safdar, FCPS (Pediatric Medicine), Department of Pediatrics, Ward Number 19, Nishtar Medical University Hospital, Multan, Pakistan.

M. Faisal Mehar, FCPS (Pediatric Medicine), Department of Pediatrics, Ward Number 19, Nishtar Medical University Hospital, Multan, Pakistan.

出版信息

Pak J Med Sci. 2021 Mar-Apr;37(2):510-514. doi: 10.12669/pjms.37.2.3535.

Abstract

OBJECTIVES

To find out frequency, clinicopathological features, response of treatment and outcome among children with primary focal segmental glomerulosclerosis (FSGS).

METHODS

This retrospective, non-interventional medical charts review study was conducted from a period of January 2011 to January 2020 at Pediatric Department of Nishtar Medical University Hospital, Multan, Pakistan. During the nine years study period, children of both genders, aged less than 16 years, with renal biopsies proven FSGS were included. Patient's demographic along with clinical and laboratory data, urine dipstick for proteinuria, renal functions, 24 hours urinary protein and ultrasonography findings of kidneys, ureters and bladder (KUB) were noted from case records. Response rates of various treatment options and their outcome like remission, partial remission, no remission with stable kidney disease & no remission with progression of kidney disease were noted.

RESULTS

During the study duration, out of 307 renal biopsies performed in glomerulonephritis cases, 124 (40.4%) had primary FSGS. In 124 primary FSGS cases, mean age was 8.83±3.05 years while most of the children, 70 (56.5%) were above 10 years of age. Majority of the cases, 64 (51.6%) were male. Mean follow up duration was noted to be 28.35+18.47 months. Most of the cases, 68 (54.8%) were found to have complete remission, 22 (17.7%) partial remission while 11 (8.9%) progressed to ESKD.

CONCLUSIONS

Among children, frequency of primary FSGS was high at our setting. Most of the cases achieved sustained remission rates with the help of immunosuppressive drugs. Cyclosporine and tacrolimus were found to be the most effective drugs.

摘要

目的

了解原发性局灶节段性肾小球硬化(FSGS)患儿的发病率、临床病理特征、治疗反应及预后。

方法

本回顾性、非干预性医学图表审查研究于2011年1月至2020年1月在巴基斯坦木尔坦尼什塔尔医科大学医院儿科进行。在9年的研究期间,纳入年龄小于16岁、经肾活检证实为FSGS的男女儿童。从病例记录中记录患者的人口统计学资料以及临床和实验室数据、蛋白尿尿试纸检测结果、肾功能、24小时尿蛋白以及肾脏、输尿管和膀胱(KUB)的超声检查结果。记录各种治疗方案的缓解率及其预后,如完全缓解、部分缓解、肾病稳定但未缓解以及肾病进展但未缓解。

结果

在研究期间,在307例肾小球肾炎病例中进行的肾活检中,124例(40.4%)患有原发性FSGS。在124例原发性FSGS病例中,平均年龄为8.83±3.05岁,而大多数儿童,70例(56.5%)年龄超过10岁。大多数病例,64例(51.6%)为男性。平均随访时间为28.35+18.47个月。大多数病例,68例(54.8%)实现完全缓解,22例(17.7%)部分缓解,而11例(8.9%)进展为终末期肾病(ESKD)。

结论

在我们的研究环境中,儿童原发性FSGS的发病率较高。大多数病例在免疫抑制药物的帮助下实现了持续缓解率。环孢素和他克莫司被发现是最有效的药物。

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Focal Segmental Glomerulosclerosis.局灶节段性肾小球硬化症
Clin J Am Soc Nephrol. 2017 Mar 7;12(3):502-517. doi: 10.2215/CJN.05960616. Epub 2017 Feb 27.
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Treatment of FSGS in Children.儿童局灶节段性肾小球硬化的治疗
Adv Chronic Kidney Dis. 2014 Mar;21(2):194-9. doi: 10.1053/j.ackd.2014.01.010.

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