Belsing Tina Zimmermann, Lund Allan Meldgaard, Søndergaard Lars, Friis-Hansen Lennart, Abildstrøm Steen Zabell
Kildegårdsvej 16 B, 2. tv., Hellerup, Denmark.
Ugeskr Laeger. 2011 Jan 31;173(5):337-42.
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.
马凡综合征（MFS）及与MFS相关的疾病是累及多个器官系统的遗传性结缔组织疾病。由于MFS的许多症状与其他系统性结缔组织疾病的症状重叠，故其诊断困难。该疾病的表型呈进行性发展。有效的手术治疗和标准化的随访方案已使患者的寿命延长。选择性血管紧张素II 1型（AT1）受体阻滞剂可能会改善MFS的多种表现，但目前尚不清楚临床试验的结果。本综述阐述了诊断、治疗和随访协调策略的重要性。
