title: [Clinical aspects of Marfan syndrome].
abstract: Marfan syndrome (MFS) and MFS-related diseases are inherited connective tissue disorders involving several organ systems. The diagnosis of MFS is difficult as the many symptoms overlap with those of other systemic connective tissue diseases. The phenotype is progressive. Effective surgical therapy and standardized follow-up programs have led to an improved lifespan for the affected individuals. Selective angiotensin II, type 1 (AT1) blockers may improve several manifestations of MFS, but the outcome of clinical trials is presently unknown. This review describes the importance of a coordinated strategy for diagnosis, treatment and follow-up.
authors.affiliation: Kildegårdsvej 16 B, 2. tv., Hellerup, Denmark. t.z.belsing@dadlnet.dk
authors.forename: Tina Zimmermann
authors.initials: TZ
authors.lastname: Belsing
authors.forename: Allan Meldgaard
authors.initials: AM
authors.lastname: Lund
authors.forename: Lars
authors.initials: L
authors.lastname: Søndergaard
authors.forename: Lennart
authors.initials: L
authors.lastname: Friis-Hansen
authors.forename: Steen Zabell
authors.initials: SZ
authors.lastname: Abildstrøm
mesh_terms: D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D006348:Cardiac Surgical Procedures; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D005820:Genetic Testing; D006801:Humans; D008382:Marfan Syndrome / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D010641:Phenotype; D011379:Prognosis
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers