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特发性儿童期起病肾病综合征的成年幸存者

Adult survivors of idiopathic childhood onset nephrotic syndrome.

作者信息

Marchel Dorota M, Gipson Debbie S

机构信息

Division of Nephrology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.

出版信息

Pediatr Nephrol. 2021 Jul;36(7):1731-1737. doi: 10.1007/s00467-020-04773-3. Epub 2020 Nov 6.

Abstract

Like many pediatric chronic health conditions, idiopathic childhood onset nephrotic syndrome (iCONS) and late effects of iCONS medical management may continue to impact the affected population in adulthood. Approximately 15% of adult survivors of steroid-sensitive iCONS continue to relapse. Long-term kidney health is associated with steroid response patterns as well as pathology findings of FSGS, tubulointerstitial fibrosis, tubular atrophy, and global glomerulosclerosis. Long-term cardiovascular disease burden is largely unknown in adult survivors, but risk factors starting in childhood, including hypertension, dyslipidemia, and obesity, are common in iCONS. Reproductive health concerns, including azo-/oligospermia and successful pregnancies, are largely related to prior exposure to cytotoxic therapies. Additional investigations are needed to complete the assessment and initiate the mitigation of the late effects of treatment-sensitive and treatment-resistant iCONS.

摘要

与许多儿童慢性健康状况一样,特发性儿童期起病的肾病综合征(iCONS)及其医学管理的晚期影响可能会在成年期继续影响受影响人群。约15%对类固醇敏感的iCONS成年幸存者会继续复发。长期肾脏健康与类固醇反应模式以及局灶节段性肾小球硬化症(FSGS)、肾小管间质纤维化、肾小管萎缩和全球肾小球硬化的病理结果相关。成年幸存者的长期心血管疾病负担在很大程度上尚不清楚,但儿童期开始的危险因素,包括高血压、血脂异常和肥胖,在iCONS中很常见。生殖健康问题,包括无精子症/少精子症和成功怀孕,在很大程度上与先前接触细胞毒性疗法有关。需要进一步调查以完成评估并开始减轻治疗敏感型和治疗抵抗型iCONS的晚期影响。

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