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      Cerebral neurovascular embryology, anatomic variations, and congenital brain arteriovenous lesions

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      Journal of NeuroInterventional Surgery
      BMJ

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          Abstract

          Cerebral neurovascular development is a complex and coordinated process driven by the changing spatial and temporal metabolic demands of the developing brain. Familiarity with the process is helpful in understanding neurovascular anatomic variants and congenital arteriovenous shunting lesions encountered in endovascular neuroradiological practice. Herein, the processes of vasculogenesis and angiogenesis are reviewed, followed by examination of the morphogenesis of the cerebral arterial and venous systems. Common arterial anatomic variants are reviewed with an emphasis on their development. Finally, endothelial genetic mutations affecting angiogenesis are examined to consider their probable role in the development of three types of congenital brain arteriovenous fistulas: vein of Galen malformations, pial arteriovenous fistulas, and dural sinus malformations.

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          Mechanisms and regulation of endothelial VEGF receptor signalling.

          Vascular endothelial growth factors (VEGFs) and their receptors (VEGFRs) are uniquely required to balance the formation of new blood vessels with the maintenance and remodelling of existing ones, during development and in adult tissues. Recent advances have greatly expanded our understanding of the tight and multi-level regulation of VEGFR2 signalling, which is the primary focus of this Review. Important insights have been gained into the regulatory roles of VEGFR-interacting proteins (such as neuropilins, proteoglycans, integrins and protein tyrosine phosphatases); the dynamics of VEGFR2 endocytosis, trafficking and signalling; and the crosstalk between VEGF-induced signalling and other endothelial signalling cascades. A clear understanding of this multifaceted signalling web is key to successful therapeutic suppression or stimulation of vascular growth.
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            International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.

            HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.
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              Vascular heterogeneity and specialization in development and disease

              Blood and lymphatic vessels have essential roles in physiology and disease. The endothelial cells that line these vessels specialize to fulfil the needs of the tissue that they pervade. Recent studies in animal models have provided insights into the mechanisms underlying vessel type- and organ-specific specialization, which is crucial for the understanding of several diseases.
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                Author and article information

                Journal
                Journal of NeuroInterventional Surgery
                J NeuroIntervent Surg
                BMJ
                1759-8478
                1759-8486
                August 12 2022
                September 2022
                September 2022
                February 15 2022
                : 14
                : 9
                : 910-919
                Article
                10.1136/neurintsurg-2021-018607
                35169032
                9e1368d6-6014-4ffa-8e2f-059c42920341
                © 2022
                History

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