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Cardiac ultrasonography or CT scan.
Marfan syndrome is an inherited connective tissue disorder in which a genetic mutation occurs on chromosome 15. People with Marfan syndrome can be tested to prevent extreme events, and it is recommended that people with a family history of Marfan syndrome should go to the cardiovascular surgery department for a check-up. Early detection through cardiac ultrasonography or CT examination to avoid heart failure, aortic rupture and myocardial infarction.
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Boys and girls.
First, Marfan syndrome is characterized by finger lengths longer than normal, and the length of the patient's fingers may be equal to or exceed the length of the palm.
The long bones are very long, which is manifested in the fact that the arms and legs are particularly long, which exceeds the normal proportionIn addition, the diagnosis is not a flat chest, but a chicken breast, that is, the sternum is a little lordosis.
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Abstract Hello, I am a professional consulting teacher, please wait, I am giving you the best answers and suggestions according to your questions, you just need to wait patiently for a while, and give you the answer within five minutes, thank you very much.
How long can you live with Marfan syndrome.
Hello, I am a professional consulting teacher, please wait, I am giving you the best answers and suggestions according to your questions, you just need to wait patiently for a while, and give you a reply within five minutes, thank you very much.
Hello, according to the situation you said, the survival time of Marfan syndrome is related to the time of detection, the timing and the extent of the disease. The natural lifespan of un** patients is about 30 years, and a few patients can be more than 40 years old; If early detection and aortic root surgery, mitral valve repair, etc.**, life can be prolonged.
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1. Skeletal abnormalities.
The most characteristic manifestation of Marfan syndrome is skeletal abnormalities, such as a tall stature and slender limbs. People with Marfan syndrome usually have longer limbs than ordinary people, and their palms, fingers, and toes resemble the limbs of spiders, and when the hands are drooping, they can be longer than the knees.
2. Muscle abnormalities.
After the occurrence of the disease, the patient has less subcutaneous fat, and the muscles are not developed enough, and the abdomen, chest and arms will show certain wrinkles. In addition, patients with Marfan syndrome have low muscle tone, elongation or relaxation of ligaments, tendons and joint capsules, and sometimes symptoms such as chicken breasts and pectus excavatum.
3. Ocular abnormalities.
In patients with Marfan syndrome, the lens of the eye may have subluxation or dislocation, high myopia, cataracts, iris tremor, and retinal detachment, which is more common in men than in women.
4. Cardiovascular abnormalities.
About 80% of patients with this disease are accompanied by congenital cardiovascular malformations, and important manifestations such as progressive arterial dilation, aortic regurgitation, mitral valve prolapse, mitral regurgitation, and tricuspid regurgitation are common.
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1 Marfan syndrome is an inherited connective tissue disease, which is inherited dominantly by autosomal dominance, characterized by uneven slenderness of limbs, fingers and toes, and a height significantly beyond that of ordinary people, accompanied by cardiovascular system abnormalities, especially combined heart valve abnormalities and aortic aneurysms. The disease may also affect other organs, including the lungs, eyes, dura, and hard palate.
2. Marfan syndrome is autosomal dominant inheritance, which is affected by genetic factors, and many tissues of the human body, such as endocardium, heart valves, large blood vessels, bones, etc., have mucopolysaccharide accumulation such as chondroitin sulfate A or C, which affects the structure and function of elastic fibers and other connective tissue fibers, and causes the corresponding organs to underdevelop and function abnormally.
3 mainly have slender limbs, spider fingers (toes), arms stretched out fingers greater than the length of the body, hands hanging down over the knees, lower body than the upper body. Longocedence, narrow face, high palatal arch, large and low ears. There is little subcutaneous fat, underdeveloped muscles, and wrinkles in the chest, abdomen, and arms.
Low muscle tone and weakness. Ligaments, tendons, and joint capsules are elongated and relaxed, and joints are hyperextended. Sometimes pectus excavatum, chicken breast, kyphosis, scoliosis, spina bifida, etc.
The main ones are lens dislocation or subluxation, high myopia, cataract, retinal detachment, iris tremor, etc. There are more males than females. Cardiovascular system.
About 80% of patients have congenital cardiovascular malformations. Progressive dilation of the aorta, aortic regurgitation, aortic sinus aneurysm, dissection aneurysm, and rupture due to cystic necrosis of the middle layer of the aorta are common. Mitral valve prolapse and mitral regurgitation are also important manifestations.
Congenital atrial septal defect, ventricular septal defect, Fallot's tetralogy, patent ductus arteriosus, coarctation of the aorta and so on may be combined. It can also be combined with various arrhythmias such as conduction block, pre-excitation syndrome, atrial fibrillation, atrial flutter, etc.
41. Cardiovascular is most likely to be complicated by idiopathic dilation of the aorta, aortic stenosis, aortic dissection aneurysm and mitral valve abnormality.
2. Ocular lesions can be complicated by lens dislocation or subluxation, high myopia, glaucoma, retinal detachment, iritis, etc.
3. Nervous system lesions can be complicated by subarachnoid hemorrhage, internal carotid aneurysm, and grand mal seizures. In addition, patients with Marfan syndrome can also develop spina bifida, myelocele, and syringomyelia.
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5. If Marfan syndrome is to be further diagnosed, an MRI is required.
Magnetic resonance imaging (MRI) refers to magnetic resonance imaging (MRI), which is a type of imaging equipment made by using the phenomenon of nuclear magnetic resonance for medical examination. Magnetic resonance imaging can not only examine the facial features, but also check for lesions such as cardiac agent macrovascular malformation, which is one of the main tests for judging Marfan syndrome.
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