December 9, 2022

Andrea Aguilera

Hi my name is andrei lera my topic about is atenolol versus loser tan in children and young’s adults with marfan syndrome definition marfan syndrome is an autosomal dominant genetic disorder with a prevalence of one every five thousand and ten newborns so it is classified as an in common disease it affects multiple organs and system its prognosis is marked by the

Cardiovascular involvement multidisciplinary follow of the special love the timeline diagnosis of complication and improve their quality of life marfan syndrome happens because of an abnormality in one copy of angina that cause problem with the body production of the protein fibrilla this protein is an important part of connective tissue work and a connective tissue

Can lead the problem in many parts of the body especially the heart eyes and bones application in medicine it is antagonists to receptor antagonists uh it works by blocking specifically and highly selectively the bending of function tension two to taiwan and hyundance receptors present in the arterial wall and other diseases as a consequence of this block there

Is an individual of the vasopressor and aldosterone releasing effects by not blocking the synthesis of angiotensin ii it acts on type 2 and condensing receptors producing vasodilation and other beneficial effects such as angiogenesis naturalises calories and increased urine production unlike ancient since converting enzyma inhibitors they don’t know interfered

With the breakdown of bradykinin and since its accumulation does not occur it does not cause the adverse effects thereby from it such as gold and axidema pros and cons brush losers and therapy it means means with more fun synthetic preparing elastic fever ground pigmentation and their third four personal hour the war acting detector los certain also decreased

The radioartic growth meditation into a small series of children’s with murfrees syndrome cons among children and jones adults with marfan syndrome who were randomly attended to los arton or atenolo we found and we found no significant difference in the rate of already regulation between the two treatment growth over a three years period issue applying in

Ecuador in the country one hundred six cases are officially known but there could be up to four hundred do they in and constantinity there are some rare entities with a high percentage of affectation conclusion marfan syndrome is an entity that is transmitted in an autosomal dominant manner clinically characterized by atria that includes escalator ocular and

Cardiovascular manifestation weeding of the skeletal uh ones are are aromability joint lacility protrusion of the septums depression of the system the crest of the radius between the upper segment and the lower segment displaced as vertebrates and specifically in the spinal lumbar flat fluid acetabulum protrusion thank you so the nih marfan trial is going to be

The biggest trial for marfan syndrome ever done it’s a trial that’s sponsored by the nih so it’s funded by the federal government and it’s being run by the pediatric heart network which is a research network that was started in 2001 to try to do research on kids with heart disease with a long-term goal of improving outcome the marfan trial was started in 2007

And in the course of the trial we’ve recruited 608 young children and young adults half of the half of the subjects are getting a tennolol and the other half are getting lozartin we do know that children and young adults with marfan syndrome are tolerating both of these medicines very well in addition to trying to figure out which medicine is better at slowing

Your aortic enlargement we’re going to learn a lot about side effects of the medicines we’re going to learn whether these medicines are helping other parts of your heart your mitral valve your left ventricle we’re looking at the effect of these medications on growth on on your skeleton whether these medicines affect your pectus or your scoliosis so we’re going to

Learn a great deal not only about the effect of atenolol and losartan in your heart but also on the rest of your body we’ve all invested a lot of time money and talent trying to get this trial done and sometimes i’m afraid that we may end up at the end of the trial with results that we didn’t expect or results that don’t move the field but because we’re looking

At a lot of different things i think that we’re going to learn a lot even if the study shows no difference between lozartin and a tenno so that keeps me going marfan syndrome it is an autosomal dominant multi-system genetic disorder characterized by abnormal manifestations in the skeletal cardiovascular and ocular systems the typical facial features observed in

Marfan syndrome include an elongated face flat cheekbones a posteriorly positioned jaw deep set eyes and down slanting palpable fissures abraham lincoln had several of these features but did he have marfan syndrome well who knows increased linear growth of the long bones is a common finding and generally results in tall stature with long limbs the torso length

To leg length ratio is often reduced while the arm span to height ratio may be increased thoracolumbar scoliosis as seen in this image is also commonly present increased joint laxity and arachnodactyly are common and when both present the combination can result in a couple of signs the wrist sign is positive if the distal phalanges of the thumb and fifth finger

Overlap when the risk is grabbed with the opposite hand and the positive thumb sign is present when the entire distal phalanx of the inductive thumb is visible beyond the ulnar border of the hand some other common skeletal manifestations include pectus excavatum pectus carnatum hind foot valgus and flat feet a variety of ocular manifestations can occur such as

Subluxation of the corneal lens myopia cataracts and glaucoma ectopia lentis in which the lens is typically displaced upwards into poorly is a cardinal feature of marfan syndrome aortic dilation is a potentially life-threatening complication that can occur in these images we can see that the aortic root is abnormally wide aortic dissection is another serious

Complication in this echocardiography we can see a double lumen blood flow during ventricular systole indicates that the upper lumen is the true lumen two other cardiac manifestations that commonly occur in patients with marfan syndrome are mitral valve prolapse and the bicuspid aortic valve as well many other non-cardiac abnormalities may be present such as

Hernia and skin striae now although it is an autosomal dominant condition the phenotypic expression is highly variable affected parents may not even be aware that they have it and may negate a family history of the disease the novel gene mutations can also occur in which case no other family member will be affected the diagnosis can be made if two or more of the

Following are present family history of marfan syndrome ectopia lentis aortic dilation or the section or a causal mutation in the fbn1 gene it is important to note that a minority of patients with marfan syndrome do not have a defined fpn1 gene mutation if the patient only has the aortic criterion or a positive family history then a systemic score of seven or more

In the revised skin criteria is required screening with echocardiography is recommended at 6 to 12 month intervals when imaging patients with marfan syndrome it is important to consider therapy with beta blockers and to advise them to avoid caffeine stimulants weightlifting and high-intensity contact sports the addition of an angiotensin receptor blocker

Transcribed from video
Atenolol versus losartan in Children and Young adults with Marfan’s Syndrome By Andrea Aguilera