On the treatment of Marfan syndrome

Content

  • medication
  • Surgery


  • medication

    On the treatment of Marfan syndromeUnfortunately, not yet found means tonormalization of metabolism in the connective tissue in patients with Marfan syndrome. Although there is some improvement in long-term use of anabolic steroids, and large doses of vitamin C.

    The basis of drug treatment isappointment of b-blockers. In case of expansion of the aorta, and especially in the presence of regurgitation (reverse blood flow) to reduce emissions in the aorta and consequently the load on the wall it is corrected concomitant hypertension. propranolol dose may be from 40 to 200 mg / day. Experience shows advantageous use of long-b-blockers, such as atenolol 25 to 150 mg / day.

    Regurgitation with mitral valve prolapse,arrhythmia also requires the appointment of b-blockers. It is believed that b-blockers prevent the risk of sudden death in patients with Marfan syndrome. But it is also necessary to know that b-blockers may aggravate the human condition of the patient, which is manifested increased dizziness, weakness, decreased performance, especially in young individuals with hypotension.

    Pathology of the skeleton in Marfan syndrome are usuallyidentified by the age of 5 years and sometimes progressing at an incredible pace, although the size of elongated limbs and fingers - the so-called dolichostenomelia and arachnodactyly already noticeable in the newborn. It is believed that in such cases responsible deficit certain macronutrients (calcium, magnesium, zinc, copper), and proteins involved in the "construction" of connective tissue, so start to measure the effectiveness of dietary supplements containing the above macrocells, and hyaluronic acid, menadione, Kolekaltsiferol .

    In the blood of patients with Marfan syndrome oftenthere is an increased level of growth hormone, so for the "suppression of excessive growth" is recommended vysokozhirovye enpity in nutrition from an early age in order to reduce the secretion of growth hormone.

    The diet of patients with Marfan syndrome shouldcontain a sufficient amount of magnesium (studies on laboratory animals have shown that when the artificial aortic damage during subsequent diet with high magnesium content is more rapid restoration of defect than normal diets with low and its contents). Because the use of magnesium preparations leading to drug correction of magnesium deficiency with mitral valve prolapse.

    The approximate scheme of therapy (treatment) with exposureon the connective tissue in Marfan syndrome must include ascorbic acid (in the absence of pathology oxaluria and stone) in the form of drinks with milk, yogurt; Dose - from 1.0 to 4.0 g per day depending on the age. Recommended products containing Glucosamine; dose at the age of 12 and adults - 1.5 g 1 times a day with meals, drink plenty of water; Course - 1.5 months. The next course is possible to use preparations containing chondroitin sulfate. Dose: for children up to 1 year - 250 mg, from 1 to 5 years - 500 mg, from 6 to 12 years - 500-750 mg, for adults - 1.5-2.0 g during meals; drink plenty of water; course - 2 months. You can also include succinic acid treatment (100 mg per capsule); 1-2 capsules two times daily rate - 3 weeks. Widely used magnesium preparations.

    Another recommended drug - carnitinechloride, 20% solution; dose for children aged 1 year - 5-10 drops, from 1 to 6 years - 15 drops, from 6 to 12 years - 30-40 drops, over 12 years - 1 teaspoon 3 times a day, after food; course - 1 month. It is also often recommended vitamin-mineral complexes course - 1 month; supplements containing L-lysine; dose - depending on the age; the multiplicity of reception - 2 times a day; Course - 1 month; tocopherol; dose at the age of 12 years and adults from 400 to 800 IU per day .; Course - 3 weeks.


    Surgery

    When aortic aneurysm, dissecting aneurysm,vice aortic valve with symptoms of heart failure in patients with Marfan syndrome can help only surgical treatment. Surgical treatments have their own indications for prosthesis: aneurysmal aortic enlargement (greater than 6 cm) is replaced by the endo- or biological prosthetics. Mitral valve prolapse in the case of "stable", even severe regurgitation (reverse blood flow direction), prosthetic valve do not conduct because it has a relatively favorable prognosis and for the absence of complications. With the rapid progression to severe regurgitation, left ventricular failure or accession requires replacement of the mitral valve. All patients with pathology of the aortic and mitral valve infective endocarditis is a risk, therefore, in the case of small and large surgical procedures should be carried out primary prophylaxis with antibiotics.

    Surgical treatment of deformities of the chest andSpine is a traumatic procedure is often complicated by pleurisy, pericarditis, pneumonia in the early and late postoperative periods. The question of whether thoracoplasty at deformations of the chest repeatedly discussed at a symposium on the pathologies of the connective tissue, and to date, specialists from various regions took a position which denies the expediency thoracoplasty Marfan syndrome.

    According to recent data, postoperative mortalityin patients with Marfan syndrome has steadily decreased (which is good). Following the transactions using prosthetic patients are able to work well.

    And now a few words about the activity of the patients. Physical activity not isklyuchayutsya, but we must remember that everything is good in moderation. Definitely negatively resolved the question of contact, as well as the group, playing sports, working with weights. Encouraged to swim.

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