Health-related quality of life in Marfan syndrome: a 10-year follow-up.

Background: Marfan syndrome, a rare hereditary connective tissue disorder caused by mutations in fibrillin-1, can affect many organ systems, especially the cardiovascular system. Previous research has paid less attention to health-related quality of life and prospective studies on this topic are needed. The aim of this study was to assess changes in health-related quality of life after 10 years in a Norwegian Marfan syndrome cohort.

Features of Marfan syndrome not listed in the Ghent nosology - the dark side of the disease.

: The revised Ghent nosology presents the classical features of Marfan syndrome. However, behind its familiar face, Marfan syndrome hides less well-known features.: The German Marfan Organization listed unusual symptoms and clinical experts reviewed the literature on clinical features of Marfan syndrome not listed in the Ghent nosology.

Marfan syndrome: Evolving organ manifestations-A 10-year follow-up study.

The age-dependent penetrance of organ manifestations in Marfan syndrome (MFS) is not known. The aims of this follow-up study were to explore how clinical features change over a 10-year period in the same Norwegian MFS cohort. In 2003-2004, we investigated 105 adults for all manifestations in the 1996 Ghent nosology.

Dural ectasia in Marfan syndrome and other hereditary connective tissue disorders: a 10-year follow-up study.

Background Context: Dural ectasia is widening of the dural sac often seen in patients with Marfan syndrome and other hereditary connective tissue disorders. Dural ectasia can cause specific symptoms and is associated with surgical complications. The knowledge on how and at which age dural ectasia develops is incomplete.

Survival, causes of death, and cardiovascular events in patients with Marfan syndrome.

Background: To explore survival, causes of death, and the prevalence of cardiovascular events in a Norwegian Marfan syndrome (MFS) cohort. MFS is a heritable connective tissue disorder associated with reduced life expectancy-primarily due to aortic pathology.

Methods: A follow-up study of 84 MFS adults, initially investigated in 2003-2004.

Ten-year reinvestigation of ocular manifestations in Marfan syndrome.

Importance: Long-term follow-up of Marfan syndrome (MFS) patients.

Background: Investigate changes in ocular features in MFS patients fulfilling the Ghent-2 criteria following a period of 10 years.

Design: Repeated cross-sectional study with two observations.