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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
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Surveillance for individuals with a pathogenic or likely pathogenic variant in VHL

Surveillance for individuals with a pathogenic or likely pathogenic variant in VHL
Surveillance Age to initiate Interval Comments
Dilated retinal examination Before 1 year Every 6 to 12 months
  • Annually after age 30
History and physical examination by physician informed about VHL 1 year Every year  
Blood pressure and pulse 2 years Every year  
Metanephrines 5 years Every year
  • Plasma free metanephrines preferred but 24-hour urine fractionated metanephrines can be used
MRI of the brain and spine 11 years Every 2 years
  • Performed with and without contrast (without contrast if pregnant)
  • Can be coordinated with MRI of the abdomen
  • Include thin cuts through the posterior fossa and petrous temple bone
  • One-time MRI of the internal auditory canal at age 15 years
Audiology 11 years Every 2 years  
MRI of the abdomen 15 years Every 2 years
  • Performed with and without contrast (without contrast if pregnant)
  • Assess kidneys, pancreas, and adrenal glands
  • Can be coordinated with MRI of the neuroaxis
This table is based on a 2020 surveillance guideline from the VHL Alliance (VHLA). It only refers to surveillance of asymptomatic individuals who are not pregnant. Any symptoms or signs of disease should be investigated promptly. During pregnancy, regular retinal examinations and testing for pheochromocytoma are performed; brain and spine MRI without contrast are done during the fourth month to assist planning for delivery. Counseling of the affected individual and/or their family members should be made available to all individuals at any age. Refer to the source document on the VHLA website for additional important information. Refer to UpToDate for additional information about surveillance and evaluation of symptoms in VHL disease.
VHL: von Hippel-Lindau; MRI: magnetic resonance imaging; VHLA: VHL Alliance.
Adapted with permission from: VHLA Suggested Active Surveillance Guidelines 2020. Copyright © 2020 VHL Alliance. Available at: https://www.vhl.org/clinicians/surveillance/ (Accessed on April 29, 2020).
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