Gerald Lewis, MD
Marfan Syndrome is an inherited disorder that affects the design and maintenance of the body's tissues making up the walls of arteries, heart valves, joints, skin and eyes. The result is unusual height, along with a few other observations that vary from individual to individual. In children, the diagnosis is most dependent upon genetic studies. The most important concern for people affected by Marfan Syndrome is the progressive enlargement of major arteries that become prone to tear or rupture.
In someone affected by Marfan, the largest artery (the aorta) is examined on a yearly basis. Should it enlarge rapidly or reach a specific size, surgical repair is necessary to prevent tear or rupture. Medical treatment can help people with Marfan Syndrome. Two types of medicine, typically used to lower blood pressure in adults, can be used as early as childhood to slow the growth of arteries, delay the need for surgery, and prevent rupture.
The medicines are called angiotensin receptor blockers (losartan and valsartan are two examples) and beta-type adrenalin receptor blockers (carvedilol, metoprolol and bisoprolol are examples). When and how these medicines are used in children is a matter best left to someone with special knowledge about Marfan Syndrome. Your nephew's pediatrician will be the best resource for finding such a person.