He síMarfan syndrome is an inherited disorder that causesñor connective tissues. Connective tissues are found everywhere in the body and playñstill play an important role in supporting various body structures and óorgans.
in this disorder, connective tissues in blood vesselsíneos, the heartón, the eyes and skeleton are generally affected.
This disorder is caused by a mutationón in a gene that is responsible for the elasticity and strength of connective tissues.
Treatment is given to maintain blood pressureón normal blood pressure and to reduce blood pressureón in the aorta. In some cases, the surgeryía is necessary to repair the affected parts.
Since he síMarfan syndrome is a genetic diseaseética, having a parent with this conditionón is an important risk factor. This conditionón affects men and women and all races equally.
Index
Síntomas
The síntomas varífrom person to person. Some people experience sótheímild symptoms, while some experience severe sísymptoms that threaten life. This conditionón may worsen with age. The signs and sísymptoms include:
- Flat feet
- tall and slim body
- Body disproportion in the arms, legs and fingers
- Esternómalformed n
- heart murmursón
- shortsightedía
- Abnormal curvatures in the spine or teeth
Complications
He síMarfan syndrome can cause complications in any part of the body:
cardiovascular complications
It can cause dañyou are serious in the heartón and the blood vesselsíneos. May cause weakening of the connective tissues that support the aorta. He isñor caused to the aorta can cause many complications.
1. Aneurisma aóarctic: This conditionón is characterized by the formationón of protrusions in the wall aóArctic. It is caused by increased pressureón de sanguípumped by the heartón.
2. dissectón aóArctic: The wall of the aorta is made up of layers. When I dissected heródoes not happen, blood seeps between the layers más internas y máexternal s of the aorta. Can cause severe chest or back pain. Can weaken vessels and increase the chance of rupture. It can be a conditionónot life threatening.
3. malformations of váwash: people with síMarfan syndrome may have a vámalformed valve or excessivelyástica. This can do to the heartón work from meás, and cause heart failureíaca.
eye complications:
1. Displace usóno of the Crystalline: La Ectopia Lentis o dislocación of the lens in the eye affects at least the 50% of the people who have the síndrome de Marfan. It occurs due to the weakening of the muscular structures that support the lens to stay in place.
2. retinal complications: Retinal complications may occur, such as retinal detachment or tear, this can occur in some people with éthis condition.
3. Other eye problems: People who have the síMarfan syndrome may have other related eye problems, like glaucoma or cataracts. Although they are usually age-related problems, mayíeven come to present at an early age if the síMarfan's ndrome isá gift.
skeletal complicationsétics
He síMarfan syndrome can lead to abnormal spinal curvatures such as the scoliosis. He sísyndrome can lead to abnormal development of the ribs, pneumotórax manifesting as a sunken or protruding chest. Spaghettién may experience back pain and foot pain.
pregnancy complications
The SíMarfan syndrome can causeñor to the aorta by the weakening of its césquid. During pregnancy, the aorta isá low strés additional and the heartón of a woman pumped más blood than normal. This can increase the risk of dissection.ón of the aorta or rupture.
