Hereditary Vascular Disorders

Question 1

thin-walled blood vessels with a discontinuous endothelium, inadequate smooth muscle, and inadequate or missing elastin in the surrounding stroma

Answer 1

Hereditary Hemorrhagic Telangiectasia
(Rendu-Osler-Weber Syndrome)

Question 2

Telangiectasias are fragile and prone to rupture. _ is an almost universal finding, and symptoms almost always worsen with age.

Answer 2

Epistaxis

Question 3

described the association of a giant cavernous hemangioma (vascular tumor), thrombocytopenia, and a bleeding diathesis. The hemangiomas are visceral or subcutaneous, but rarely both

Answer 3

Hemangioma-Thrombocytopenia Syndrome
(Kasabach- Merritt Syndrome)

Question 4

hyperextensible skin, hypermobile joints, joint laxity, fragile tissues, and a bleeding tendency, primarily subcutaneous he- matoma formation

bleeding ranges: from easy bruising to arterial rupture

Answer 4

Ehlers-Danlos Syndrome and Other Genetic Disorders