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Kaplan Qbank USMLE



Author4 Posts
  #1

I had 2 qs on it so & its not covered anywhere, so decided to share it

What is Von Hippel-Lindau Disease (VHL)?von Hippel-Lindau disease (VHL) is a rare, genetic multi-system disorder characterized by the abnormal growth of tumors in certain parts of the body (angiomatosis). The tumors of the central nervous system (CNS) are benign and are comprised of a nest of blood vessels and are called hemangioblastomas (or angiomas in the eye). Hemangioblastomas may develop in the brain, the retina of the eyes, and other areas of the nervous system. Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas. Symptoms of VHL vary among patients and depend on the size and location of the tumors. Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Cysts (fluid-filled sacs) and/or tumors (benign or cancerous) may develop around the hemangioblastomas and cause the symptoms listed above. Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer.

Don't forget it is sometimes associated with PHEOCROMOCYTOMA ( clue in the qs)


  #2

asociated with chromosome 3.
thanks for sharing

___________________
As a general rule, the better it felt when you said it, the more trouble it's going to get you into.

  #3

Yeah, thats all I remember from Step1: von Hippel Lindau (3 words, 3rd chromosome)
smiling face


___________________
Our greatest glory is not in never falling, but in rising every time we fall.

  #4

Ok, there is more: (and this is how I USED TO remember it) smiling face

VHL -3 words, 3rd chromosome
3 places in the nervous system:
Hemangiomas of the
1. cerebellum
2. brainstem
3. spinal cord
(these are called Lindau tumors)

von-Hippel: Retinal Hemangioblastomas.

And also Cysts of the:
1. liver
2. pancreas
3 Kidneys (MULTIPLE bilateral renal cell carcinomas)

(maybe an over simplification, but I can't fit any more into my brain about VHL) smiling face






___________________
Our greatest glory is not in never falling, but in rising every time we fall.







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