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Ehlers-Danlos Syndrome, classical type: case management

Whitelaw SE

Pediatric nursing, 2003

Abstract

As the field of genetics expands, there will be more need for health care professionals to possess basic knowledge of genetic conditions and patterns of inheritance to assist their patients and to make the proper referrals. Ehlers-Danlos Syndrome (EDS) is a group of genetic connective tissue disorders that affects approximately 1 in 5,000 live births, including males and females of all racial and ethnic groups. The main characteristics of EDS are skin hyperextensibility, tissue fragility, and joint hypermobility. Diagnosis of EDS is often difficult due to the complexity of symptoms and lack of specific genetic tests. However, once a diagnosis is suspected or confirmed, nurses play a vital role in assisting the patient and family to manage the disorder. This article describes EDS symptoms and genetic basis, provides suggestions for management, and reviews resources available for health care providers, families, and patients with EDS.

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