Loeys–Dietz syndrome
Medical condition
Loeys–Dietz syndrome
Loeys–Dietz syndrome (LDS) is an autosomal dominant genetic connective tissue disorder. It has features similar to Marfan syndrome and Ehlers–Danlos syndrome. The disorder is marked by aneurysms in the aorta, often in children, and the aorta may also undergo sudden dissection in the weakened layers of the wall of the aorta. Aneurysms and dissections also can occur in arteries other than the aorta. Because aneurysms in children tend to rupture early, children are at greater risk for dying if the syndrome is not identified. Surgery to repair aortic aneurysms is essential for treatment. It was previously believed that the life expectancy of an individual with this condition was around 30-40 years of age; however, with progressive treatments such as possibilities for surgery and medications like losartan, it is now proven that life expectancy can be normal with appropriate medical intervention. There are five types of the syndrome, designated types I through V, caused by mutations in TGFBR1...
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