Digital Public Defence: Thy Thy Vanem
Cand.med. Thy Thy Vanem at Institute of Clinical Medicine will be defending the thesis Marfan syndrome in adults – re-investigations in a Norwegian cohort after 10 years for the degree of PhD (Philosophiae Doctor).
Photo: Phoi Phoi Diep
The public defence will be held as a video conference over Zoom.
The defence will follow regular procedure as far as possible, hence it will be open to the public and the audience can ask ex auditorio questions when invited to do so.
Due to copyright reasons, an electronic copy of the thesis must be ordered from the faculty. In order for the faculty to have time to process the order, it must be received by the faculty no later than 2 days prior to the public defence. Orders received later than 2 days before the defence will not be processed. Inquiries regarding the thesis after the public defence must be addressed to the candidate.
Digital Trial Lecture - time and place
- First opponent: Professor Anne de Paepe, Ghent University, Belgium
- Second opponent: Professor Truls Myrmel, UiT The Arctic University of Norway, Tromsø
- Third member and chair of the evaluation committee: Professor Olav Røise, Institute of Clinical Medicine, University of Oslo
Chair of defence
Associate Professor Grethe Månum, Institute of Clinical Medicine, University of Oslo
Svend Rand-Hendriksen, Sunnaas Rehabilitation Hospital, Nesodden
Marfan syndrome is a heritable connective tissue disorder, which can affect many organ systems. The syndrome is difficult to diagnose and the manifestations vary in individuals with Marfan syndrome, even within the same family. Changes over time in the reported manifestations of Marfan syndrome are not fully understood.
Life expectancy has been reduced in Marfan syndrome patients, mainly due to cardiovascular causes, especially aortic complications. Better diagnosis and treatment seem to improve life expectancy, but prior to this study, no one has documented how much life expectancy has increased. As life expectancy increases, health-related quality of life becomes more important.
The main aims of this study were, after 10 years, to explore survival and causes of death, describe the changes of all the relevant organ manifestations, to study the changes in health-related quality of life and to reassess the diagnosis of Marfan syndrome, in a Norwegian adult Marfan syndrome cohort.
After 10 years, 16 of 84 patients were deceased, mainly due to aortic complications. Compared to the general Norwegian population males and females with Marfan syndrome have eight and nearly four times higher mortality, respectively. New severe organ manifestations may occur in adulthood and progress throughout life. Although physical health-related quality of life is significantly reduced after 10 years, mental health-related quality of life remains unchanged. In addition, new organ findings after 10 years did not predict the changes in health-related quality of life.
The diagnosis is still difficult and dependent on the results of DNA sequencing, and due to new criteria, some patients were re-classified to other diagnoses.
Despite better diagnosis and better treatment, life expectancy is still reduced in this Marfan syndrome cohort compared to the general Norwegian population.
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