从儿童和成人肾脏学的角度看肾小管疾病和结石。

IF 2.8 3区 医学 Q2 UROLOGY & NEPHROLOGY
Seminars in nephrology Pub Date : 2023-07-01 Epub Date: 2023-11-14 DOI:10.1016/j.semnephrol.2023.151437
Johannes Münch, Paul R Goodyer, Carsten A Wagner
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引用次数: 0

摘要

肾脏的管状系统是由一系列复杂的形态和功能单位组成的,当管状液体沿着肾元和收集管流动时,它会协调管状液体的内容。肾小管维持体内水分,调节电解质和酸碱平衡,重新吸收宝贵的有机溶质,并消除特定的代谢物、毒素和药物。此外,调节血压的决定性机制是由肾小管控制的。这些小管功能的遗传和获得性障碍可能导致儿童和成年期出现的严重疾病。这篇文章讨论了小管病变的选择和潜在的病理机制,同时强调了儿科和成人肾病护理的重要差异。这些疾病包括罕见的单基因疾病,如儿童时期出现的肾源性尿崩症、胱氨酸病和巴特综合征,以及在成人中更为普遍的遗传和获得性肾小管病变,导致高血压或肾结石。儿科和成人肾病学家都必须意识到这些情况和年龄依赖性的表现,保证两个亚专科之间的密切互动。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Tubular Diseases and Stones Seen From Pediatric and Adult Nephrology Perspectives.

The tubular system of the kidneys is a complex series of morphologic and functional units orchestrating the content of tubular fluid as it flows along the nephron and collecting ducts. Renal tubules maintain body water, regulate electrolytes and acid-base balance, reabsorb precious organic solutes, and eliminate specific metabolites, toxins, and drugs. In addition, decisive mechanisms to adjust blood pressure are governed by the renal tubules. Genetic as well as acquired disorders of these tubular functions may cause serious diseases that manifest both in childhood and adulthood. This article addresses a selection of tubulopathies and the underlying pathomechanisms, while highlighting the important differences in pediatric and adult nephrology care. These range from rare monogenic conditions such as nephrogenic diabetes insipidus, cystinosis, and Bartter syndrome that present in childhood, to the genetic and acquired tubular pathologies causing hypertension or nephrolithiasis that are more prevalent in adults. Both pediatric and adult nephrologists must be aware of these conditions and the age-dependent manifestations that warrant close interaction between the two subspecialties.

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来源期刊
Seminars in nephrology
Seminars in nephrology 医学-泌尿学与肾脏学
CiteScore
5.60
自引率
0.00%
发文量
27
审稿时长
6-12 weeks
期刊介绍: Seminars in Nephrology is a timely source for the publication of new concepts and research findings relevant to the clinical practice of nephrology. Each issue is an organized compendium of practical information that serves as a lasting reference for nephrologists, internists and physicians in training.
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