The Marfan and Ehlers-Danlos syndromes and pregnancy

The Marfan and Ehlers-Danlos syndromes have always received great public interest and fascination. Individuals with the Marfan syndrome impress with their tall statlls, long extremities, hands and feet, and patients affected with the EhlersDanlos syndrome are known for their agility in performing acts and tricks of hypermobility before theatre and circus audiences. From the medical point of view, many of these patients are at risk of an early death because of cardiovascular complications caused by aneurysms and cardiac insufficiency in the Marfan syndrome, and by aneurysms and vascular fragility in the Ewers-Danlos syndrome. Moreover, many cases are of autosomal dominant inheritance, with a risk of 50% for a child to inherit the disorder from an affected parent. The Marfan syndrome and the various types of the Ehlers-Danlos syndrome (EDS) are rare inheritable disorders of connective tissue resulting in a generalized weakness of the supporting tissues of the body. Affected women may experience significant health problems for themselves and their offspring during and after pregnancy. For this study the Marfan and Ehlers-Danlos syndromes were chosen from the group of genetic connective tissue disorders for three reasons. First, because they share some of the clinical features, which include severe complications; second, because the syndromes show mainly an autosomal dominant pattern of inheritance; third, because the disorders have a similar prevalence. The activities of the patient associations in the Netherlands were an incentive to collect families with affected individuals for the study.

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