Marfan sentence example

marfan
  • Marfan syndrome is an inherited disorder of the connective tissue that causes abnormalities of a child's eyes, cardiovascular system, and musculoskeletal system.
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  • It is named for the French pediatrician, Antoine Marfan (1858-1942), who first described it in 1896.
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  • The genetic mutation responsible for Marfan was discovered in 1991.
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  • The most common external signs associated with Marfan syndrome include excessively long arms and legs, with the child's arm span being greater than his or her height.
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  • It is important to note, however, that children with Marfan vary widely in the external signs of their disorder and in their severity; even two children from the same family may look quite different.
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  • Most of the external features of Marfan syndrome become more pronounced as the child gets older, so that diagnosis of the disorder is often easier in adults than in children.
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  • Marfan syndrome by itself does not affect a child's intelligence or ability to learn.
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  • There is, however, some clinical evidence that children with Marfan have a slightly higher rate of hyperactivity and attention-deficit disorder (ADD) than the general population.
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  • In addition, a child with undiagnosed nearsightedness related to Marfan may have difficulty seeing the blackboard or reading printed materials, and thus do poorly in school.
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  • Marfan syndrome affects males and females equally, and appears to be distributed equally among all races and ethnic groups.
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  • Marfan syndrome is sometimes called arachnodactyly, which means "spider-like fingers" in Greek, since one of the characteristic signs of the disease is disproportionately long fingers and toes.
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  • It is estimated that one person in every 3000-5000 has Marfan syndrome, or about 50,000 people in the United States.
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  • Marfan syndrome is one of the more common inheritable disorders.
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  • Marfan syndrome is caused by a single gene for fibrillin on chromosome 15, which is inherited in most cases from an affected parent.
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  • Mutations of the fibrillin gene (FBNI) are unique to each family affected by Marfan, which makes rapid genetic diagnosis impossible, given present technology.
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  • Another important genetic characteristic of Marfan syndrome is variable expression.
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  • The most important complications of Marfan are those affecting the heart and major blood vessels; some are potentially life-threatening.
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  • This is the most serious potential complication of Marfan syndrome.
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  • Between 75 and 85% of children with Marfan have loose or "floppy" mitral valves, which are the valves that separate the chambers of the heart.
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  • In children with Marfan, it is the abnormal mitral valve that is most likely to become infected.
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  • Some children with Marfan develop cystic disease of the lungs or recurrent spontaneous pneumothorax, which is a condition in which air accumulates in the space around the lungs.
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  • Marfan syndrome causes an increase in the length of the child's bones, with decreased support from the ligaments that hold the bones together.
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  • It is caused by a combination of the rapid growth of children with Marfan, and the looseness of the ligaments that help the spine to keep its shape.
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  • Children with Marfan may develop kyphosis either in the upper (thoracic) spine or the lower (lumbar) spine.
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  • It can cause difficulties in breathing, especially if the heart, spine, and lung have been affected by Marfan.
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  • In other children with Marfan, the sternum is pushed outward and narrowed.
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  • A few children with Marfan may have a pectus excavatum on one side of their chest and a pectus carinatum on the other.
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  • Children with Marfan are more likely to develop pes planus (flat feet) or so-called "claw" or "hammer" toes than people in the general population.
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  • In a child with Marfan, the acetabulum becomes deeper than normal during growth, for reasons that are not yet understood.
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  • Although the visual problems that are related to Marfan syndrome are rarely life-threatening, they are important in that they may be the child's first indication of the disorder.
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  • Most children with Marfan develop nearsightedness, usually in childhood.
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  • Between 65 and 75 percent of children with Marfan have dislocated lenses.
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  • Glaucoma. This condition is much more prevalent in children with Marfan syndrome than in the general population.
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  • Cataracts. Children with Marfan are more likely to develop cataracts, and to develop them much earlier in life, sometimes as early as 40 years of age.
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  • Children with Marfan are more vulnerable to this disorder because of the weakness of their connective tissues.
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  • Children with Marfan sometimes develop dental problems related to crowding of the teeth caused by a high-arched palate and a narrow jaw.
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  • Children with Marfan often develop striae over the shoulders, hips, and lower back at an early age because of rapid bone growth.
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  • In children with Marfan, obstructive sleep apnea is caused by the unusual flexibility of the tissues lining the child's airway.
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  • Prospective parents with a family history of Marfan syndrome should check with their doctor concerning genetic counseling.
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  • Also a doctor should be called if a child has symptoms suggestive of Marfan syndrome.
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  • The symptoms of Marfan syndrome in some children resemble the symptoms of homocystinuria, which is an inherited disorder marked by extremely high levels of homocystine in the child's blood and urine.
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  • The treatment and management of Marfan is tailored to the specific symptoms of each child.
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  • After a child has been diagnosed with Marfan, he or she should be monitored with an echocardiogram every six months until it is clear that the aorta is not growing larger.
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  • A child with Marfan may be given drugs called beta-blockers to slow down the rate of aortic enlargement and decrease the risk of dissection by lowering the blood pressure and decreasing the forcefulness of the heartbeat.
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  • The most commonly used beta-blockers in chidren with Marfan are propranolol (Inderal) and atenolol (Tenormin).
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  • Because children with Marfan are at increased risk for infective endocarditis, they must take a prophylactic dose of an antibiotic before having dental work or minor surgery, as these procedures may allow bacteria to enter the bloodstream.
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  • Children diagnosed with Marfan should be checked for scoliosis by their pediatricians at each annual physical examination.
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  • Children with Marfan should consider wearing shoes with low heels, special cushions, or orthotic inserts.
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  • Children with Marfan should have a thorough eye examination, including a slit-lamp examination, to test for dislocation of the lens as well as nearsightedness.
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  • Because children with Marfan are at increased risk of glaucoma, they should have the fluid pressure inside the eye measured every year as part of an eye examination.
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  • It is important, however, to seek treatment at medical centers with eye surgeons familiar with the possible complications of cataract surgery in children with Marfan syndrome.
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  • All children with Marfan should be taught to recognize the signs of retinal detachment (sudden blurring of vision in one eye becoming progressively worse without pain or redness) and ask their parents to seek professional help immediately.
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  • Children with Marfan should be evaluated by their dentist at each checkup for crowding of the teeth and possible misalignment and referred to an orthodontist if necessary.
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  • Children with Marfan should avoid sports or occupations that require heavy weightlifting, rough physical contact, or rapid changes in atmospheric pressure (e.g., scuba diving).
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  • Regular noncompetitive physical exercise, however, is beneficial for children with Marfan.
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  • It is one of the most important single indicators in diagnosing Marfan syndrome.
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  • In Marfan's syndrome, the gene responsible for fibrillin has mutated, causing the body to produce a defective protein.
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  • Smoking is particularly harmful for children and adolescents with Marfan because it increases their risk of emphysema.
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  • Children and adolescents with Marfan may benefit from supportive counseling regarding appearance, particularly if their symptoms are severe enough to cause them to withdraw from social activities.
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  • The prognosis for children with Marfan has improved markedly in recent years.
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  • Marfan syndrome that occurs because of spontaneous new mutations (15% to 25% of the cases) cannot be prevented.
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  • However, for prospective parents with a family history of Marfan syndrome, genetic counseling is recommended.
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  • Support groups are often good sources of information about Marfan; they can offer helpful suggestions about living with it as well as emotional support.
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  • Marfan syndrome: an inherited disorder of connective tissue (tissue that adds strength to the body's structures), affecting the skeletal system, cardiovascular system, eyes, and skin.
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  • Marfan syndrome is a connective tissue disorder that causes tears in the aorta.
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  • Since the disease also causes excessive bone growth, most Marfan syndrome patients are over 6 ft (1.8 m) tall.
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  • Researchers believe the defect responsible for Marfan's syndrome is found in gene FBN1 on chromosome 15.
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  • High myopia has been associated with various syndromes: Ehlers-Danlos, Marfan, Down, and Stickler syndromes.
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  • See also Diabetes mellitus; Ehlers-Danlos syndrome; Marfan's syndrome.
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  • He did not get to see the fruits of his labors - on January 25, 1996, just after the final dress rehearsal and before the off-Broadway premiere, Larson died of an aneurysm, due to undiagnosed Marfan Syndrome.
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