小儿脑积水。

IF 76.9 1区 医学 Q1 MEDICINE, GENERAL & INTERNAL Nature Reviews Disease Primers Pub Date : 2024-05-16 DOI:10.1038/s41572-024-00519-9
Kristopher T Kahle, Petra M Klinge, Jenna E Koschnitzky, Abhaya V Kulkarni, Nanna MacAulay, Shenandoah Robinson, Steven J Schiff, Jennifer M Strahle
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引用次数: 0

摘要

脑积水通常被认为是脑脊液(CSF)平衡失调导致脑室主动扩张。患有脑积水的婴儿可表现为头围进行性增大,而年龄较大的儿童通常表现为颅内压升高的体征和症状。先天性脑积水在出生时或接近出生时出现,有些病例与基因突变有关,基因突变会破坏大脑的形态发生并改变 CSF-大脑界面的生物力学。后天性脑积水可在出生后任何时候发生,通常由中枢神经系统感染或出血引起,与 CSF 通路阻塞和炎症依赖性 CSF 分泌和清除失调有关。脑积水的治疗方法主要包括外科 CSF 分流术或内镜下第三脑室造口术,以及或不包括脉络丛烧灼术。胎儿脑积水可通过手术闭合相关神经管缺陷进行治疗。脑积水患儿的长期预后差异很大,取决于内在(遗传)和外在因素。基因组学、脑成像和其他技术的进步开始完善脑积水的定义,提高预后的精确度,并确定非手术治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Paediatric hydrocephalus.

Hydrocephalus is classically considered as a failure of cerebrospinal fluid (CSF) homeostasis that results in the active expansion of the cerebral ventricles. Infants with hydrocephalus can present with progressive increases in head circumference whereas older children often present with signs and symptoms of elevated intracranial pressure. Congenital hydrocephalus is present at or near birth and some cases have been linked to gene mutations that disrupt brain morphogenesis and alter the biomechanics of the CSF-brain interface. Acquired hydrocephalus can develop at any time after birth, is often caused by central nervous system infection or haemorrhage and has been associated with blockage of CSF pathways and inflammation-dependent dysregulation of CSF secretion and clearance. Treatments for hydrocephalus mainly include surgical CSF shunting or endoscopic third ventriculostomy with or without choroid plexus cauterization. In utero treatment of fetal hydrocephalus is possible via surgical closure of associated neural tube defects. Long-term outcomes for children with hydrocephalus vary widely and depend on intrinsic (genetic) and extrinsic factors. Advances in genomics, brain imaging and other technologies are beginning to refine the definition of hydrocephalus, increase precision of prognostication and identify nonsurgical treatment strategies.

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来源期刊
Nature Reviews Disease Primers
Nature Reviews Disease Primers Medicine-General Medicine
CiteScore
76.70
自引率
0.20%
发文量
75
期刊介绍: Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.
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