Marfan Syndrome Research - Genetics, Causes, Symptoms, Treatment

Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.


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Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome.

Selamet Tierney ES, Feingold B, Printz BF, Park SC, Graham D, Kleinman CS, Mahnke CB, Timchak DM, Neches WH, Gersony WM

Division of Pediatric Cardiology, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University, College of Physicians & Surgeons, New York, NY, USA. Seda.Tierney@cardio.chboston.org

OBJECTIVE: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients. STUDY DESIGN: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied. All clinical data and 213 serial echocardiograms were reviewed, and aortic root dimensions were measured. Patients were divided into 2 groups for comparison: untreated (n = 34) and treated (n = 29). RESULTS: At study entry, the 2 study groups were comparable in terms of age, sex, body surface area (BSA), aortic root measurements, heart rate, and corresponding z scores. Follow-up duration in each group was similar. At last follow-up, heart rates and heart rate z scores were lower in the treated group. Rates of change of aortic root measurements (P = .52) and the corresponding z scores were not statistically different between the 2 group at the study's end. CONCLUSIONS: This study suggests that that beta-blocker therapy does not significantly alter the rate of aortic root dilation in children with Marfan syndrome. Based on these data, the recommendation of lifetime beta-blocker therapy instituted during childhood should be reassessed.

Published 25 December 2006 in J Pediatr, 150(1): 77-82.
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Marfan Syndrome Research Today Archive:

Volume 1 (2005)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)
  Issue 9 (September)
  Issue 10 (October)
  Issue 11 (November)
  Issue 12 (December)

Volume 2 (2006)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)
  Issue 9 (September)
  Issue 10 (October)
  Issue 11 (November)
  Issue 12 (December)

Volume 3 (2007)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)
  Issue 9 (September)
  Issue 10 (October)
  Issue 11 (November)
  Issue 12 (December)

Volume 4 (2008)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)



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Marfan Syndrome: A Primer for Clinicians and Scientists (Medical Intelligence Unit)

Marfan Syndrome: A Primer for Clinicians and Scientists (Medical Intelligence Unit)