What condition is characterized by the presence of arteriovenous malformations (AVMs) in multiple organs?

Study for the USMLE Step 2 CK Exam. Prepare with flashcards and multiple choice questions, each question has hints and explanations. Get ready for your exam!

The condition characterized by the presence of arteriovenous malformations (AVMs) in multiple organs is indeed Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia (HHT). This genetic disorder is marked by the formation of AVMs, which are abnormal connections between arteries and veins that can lead to a variety of complications, including bleeding, especially in the lungs, liver, and brain.

Patients with Osler-Weber-Rendu syndrome often present with telangiectasias (small dilated blood vessels) on the skin and mucous membranes, frequent nosebleeds, and gastrointestinal bleeding. The genetic mutations involved often affect the vascular endothelial growth factor (VEGF) pathway, which plays a critical role in blood vessel formation and maintenance.

Other conditions listed, such as Von Willebrand disease, Ehlers-Danlos syndrome, and Marfan syndrome, do not primarily involve the development of AVMs as a central feature. Von Willebrand disease is a bleeding disorder resulting from defects in platelet function and coagulation. Ehlers-Danlos syndrome is a connective tissue disorder characterized by hyperelasticity of the skin and joint hypermobility, while Marfan syndrome is primarily associated with cardiovascular, skeletal

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