TITLE

Ehlers-Danlos Syndrome, Classical Type: Case Management

AUTHOR(S)
Whitelaw, Sara E.
PUB. DATE
November 2003
SOURCE
Pediatric Nursing;Nov/Dec2003, Vol. 29 Issue 6, p423
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
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.
ACCESSION #
11839512

 

Related Articles

  • Ehlers-Danlos Syndrome, Classical Type: Case Management. Whitelaw, Sara E. // Dermatology Nursing;Oct2004, Vol. 16 Issue 5, p433 

    The article presents information on Ehlers-Danlos syndrome (EDS), 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...

  • Inter-examiner reproducibility of tests and criteria for generalized joint hypermobility and benign joint hypermobility syndrome. B. Juul-Kristensen; H. Røgind; D. V. Jensen; L. Remvig // Rheumatology;Dec2007, Vol. 46 Issue 12, p1835 

    Objective. To test the reproducibility of tests and criteria for generalized joint hypermobility (GJH) and benign joint hypermobility syndrome (BJHS). Methods. A standardized protocol for clinical reproducibility studies was followed using a three-phase study (with a training, an overall...

  • Ehlers-Danlos Syndrome: Recent Advances and Current Understanding of the Clinical and Genetic Heterogeneity. Byers, Peter H. // Journal of Investigative Dermatology;Nov94 Supplement, Vol. 103, p47S 

    The Ehlers-Danlos syndrome (EDS) is a heterogeneous group of generalized connective tissue disorders, the major manifestations of which are skin fragility, skin hyperextensibility, and joint hypermobility. The clinical and molecular definition of more than ten types of EDS has, more than ever,...

  • Ascorbate Action on Normal and Mutant Human Lysyl Hydroxylases from Cultured Dermal Fibroblasts. Miller, Robert L.; Elsas, Louis J.; Priest, Robert E. // Journal of Investigative Dermatology;May79, Vol. 72 Issue 5, p241 

    The mechanism of ascorbate action on lysyl hydroxylase (LH) was investigated using cultured dermal fibroblasts from a patient with hydroxylysine-deficient collagen disease and from appropriate control patients. Cells from this patient had moderately impaired LH and both parents had intermediate...

  • A Recessive Form of the Ehlers—Danlos Syndrome Caused by Tenascin-X Deficiency. Schalkwijk, Joost; Zweers, Manon C.; Steijlen, Peter M.; Dean, Willow B.; Taylor, Glen; van Vlijmen, Ivonne M.; van Haren, Brigitte; Miller, Walter L.; Bristow, James // New England Journal of Medicine;10/18/2001, Vol. 345 Issue 16, p1167 

    Background: The Ehlers–Danlos syndrome is a heritable connective-tissue disorder caused by defects in fibrillar-collagen metabolism. Mutations in the type V collagen genes account for up to 50 percent of cases of classic Ehlers–Danlos syndrome, but many other cases are unexplained....

  • Ehlers-Danlos Syndrome (EDS) type IV. Review of the literature. Macabu Badauy, Cristiano; Gomes, Sabrina S.; Sant'Ana Filho, Manoel; Bogo Chies, José Artur // Clinical Oral Investigations;Sep2007, Vol. 11 Issue 3, p183 

    Ehlers-Danlos syndrome (EDS) is a heterogeneous group of connective tissue heritable disorders. EDS type IV is a rare form that presents typical clinical signs, such as easy bruising and haematomas at sites of trauma, skin manifestations (translucent skin with visible veins), and joint...

  • Ehlers-Danlos syndrome type IV and recurrent carotid-cavernous fistula: review of the literature, endovascular approach, technique and difficulties. Desal, H. A.; Toulgoat, F.; Raoul, S.; Guillon, B.; Bommard, S.; Naudou-Giron, E.; Auffray-Calvier, E.; de Kersaint-Gilly, A. // Neuroradiology;Apr2005, Vol. 47 Issue 4, p300 

    We report the follow-up of a previously published case (Forlodou et al. Neuroradiology 38:595-597, 1996) of carotido-cavernous fistulas (CCFs) in a patient presenting with type IV Ehlers-Danlos syndrome (EDS 4) that were successfully treated twice by an endovascular approach. Initial treatment...

  • Ehlers-Danlos syndrome and multiple sclerosis: a possible association. Vilisaar, J.; Harikrishnan, S.; Suri, M.; Constantinescu, C. S. // Multiple Sclerosis (13524585);May2008, Vol. 14 Issue 4, p567 

    Ehlers-Danlos syndrome (EDS) has various neurological manifestations. Here we present an association of EDS with multiple sclerosis (MS). Four MS patients from a total of 1892 followed up at our MS outpatient clinics had EDS. This frequency suggests 10-11 times increased prevalence of EDS in MS...

  • Ehlers-Danlos syndrome type IV. Germain, Dominique P. // Orphanet Journal of Rare Diseases;2007, Vol. 2, p32 

    Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics