Recognizing and treating Ehlers-Danlos syndrome(s): the need for a multidisciplinary approach.

被引:0
作者
Morlino, Silvia [1 ]
Piedimonte, Caterina [2 ]
Castori, Marco [2 ]
机构
[1] Univ Roma La Sapienza, Unidad Genet Med, Dept Med Mol, Hosp Gen San Camillo Forlanini,Gen Hosp, Rome, Italy
[2] Univ Roma La Sapienza, Dept Pediat Neurol Infantil & Psiquiatria, Policlin Hosp Univ Umberto 1, Rome, Italy
来源
CUADERNOS DE NEUROPSICOLOGIA-PANAMERICAN JOURNAL OF NEUROPSYCHOLOGY | 2016年 / 10卷
关键词
diagnosis; Ehlers-Danlos syndrome; fatigue; joint hypermobility; management; pain;
D O I
10.7714/CNPS/10.4.203
中图分类号
B84 [心理学];
学科分类号
04 ; 0402 ;
摘要
Ehlers-Danlos syndrome groups together different inherited disorders of the soft connective tissues with systemic manifestations. The most common features include chronic/recurrent musculoskeletal pain, headache, chronic fatigue and cardiovascular dysautonomia, arterial and visceral fragility with propensity to spontaneous ruptures, functional gastrointestinal disorders, pelvic and voiding dysfunction, minor neurodevelopmental disorders and some psychiatric comorbidities. Severity and extent of such complications are strongly influenced by the clinical-molecular subtype. Hence, accurate diagnosis is pivotal for appropriate management. The proteiform manifestations of Ehlers-Danlos syndromes often request a multidisciplinary approach in the pediatric as well as adult patients. The multisystem nature of many symptoms and their still incompletely understood pathogenesis make hard to treat the complex patient, who often needs the coordinated interventions of various professionals. Due to the lack of a consensus on the treatment of Ehlers-Danlos syndromes, here the authors report their experience and a proposal.
引用
收藏
页码:45 / 60
页数:16
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