Management of colonic complications of type IV Ehlers–Danlos syndrome: a systematic review and evidence‐based management strategy

2019 ◽  
Vol 22 (2) ◽  
pp. 129-135 ◽  
Author(s):  
D. Speake ◽  
L. Dvorkin ◽  
C. J. Vaizey ◽  
G. L. Carlson
Keyword(s):  
Systematic Review ◽  
Management Strategy ◽  
Evidence Based ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Bowel perforation in type IV vascular Ehlers–Danlos syndrome. A systematic review

2018 ◽  
Vol 22 (5) ◽  
pp. 333-341 ◽  
Author(s):  
H. El Masri ◽  
T.-H. Loong ◽  
G. Meurette ◽  
J. Podevin ◽  
F. Zinzindohoue ◽  
...  
Keyword(s):  
Systematic Review ◽  
Bowel Perforation ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

scholarly journals Multiexon deletion in the procollagen III gene is associated with mild Ehlers-Danlos syndrome type IV.

1991 ◽  
Vol 266 (8) ◽  
pp. 5244-5248
Author(s):  
H Vissing ◽  
M D'Alessio ◽  
B Lee ◽  
F Ramirez ◽  
P H Byers ◽  
...  
Keyword(s):  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome ◽  
Procollagen Iii

scholarly journals Hereditary Connective Tissue Diseases in Young Adult Stroke: A Comprehensive Synthesis

2011 ◽  
Vol 2011 ◽  
pp. 1-18 ◽  
Author(s):  
Olivier M. Vanakker ◽  
Dimitri Hemelsoet ◽  
Anne De Paepe
Keyword(s):  
Connective Tissue ◽  
Metabolic Disorders ◽  
Type Iv Collagen ◽  
Connective Tissue Diseases ◽  
Haemorrhagic Stroke ◽  
Monogenic Disease ◽  
Connective Tissue Disorders ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Though the genetic background of ischaemic and haemorrhagic stroke is often polygenetic or multifactorial, it can in some cases result from a monogenic disease, particularly in young adults. Besides arteriopathies and metabolic disorders, several connective tissue diseases can present with stroke. While some of these diseases have been recognized for decades as causes of stroke, such as the vascular Ehlers-Danlos syndrome, others only recently came to attention as being involved in stroke pathogenesis, such as those related to Type IV collagen. This paper discusses each of these connective tissue disorders and their relation with stroke briefly, emphasizing the main clinical features which can lead to their diagnosis.

scholarly journals Internal carotid artery dissection in a patient with Ehlers-Danlos syndrome type IV: diagnosis and management

2013 ◽  
Vol 12 (2) ◽  
pp. 174-179 ◽  
Author(s):  
Michel Nasser ◽  
Murilo Bucci Vega ◽  
Luca Giovani Antonio Pivetta ◽  
Ana Izabel Nasser ◽  
Debora Gusmao Melo
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Internal Carotid ◽  
Vascular Complications ◽  
Carotid Artery Dissection ◽  
Artery Dissection ◽  
Internal Carotid Artery Dissection ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Ehlers-Danlos syndrome (EDS) type IV, also known as vascular EDS, is an inherited connective tissue disorder with an estimated prevalence of 1/100,000 to 1/250,000. In EDS type IV, vascular complications may affect all anatomical areas, with a preference for large- and medium-sized arteries. Dissections of the vertebral and carotid arteries in their extra- and intra-cranial segments are typical. The authors report the case of a patient with EDS type IV for whom the diagnosis was established based on clinical signs and who developed internal carotid artery dissection at the age of 44 years. In the absence of a specific treatment for EDS type IV, medical interventions should focus on symptomatic relief, prophylactic measures, and genetic counseling. Invasive imaging techniques are contraindicated, and a conservative approach to vascular complications is usually recommended.

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