A Rare Disorder of Bleeding Tendency: Ehlers-Danlos Syndrome

نویسندگان

  • Alavi, Samin Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Molaei Tavana, Parastou Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
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Ehlers-Danlos syndrome: type VI A – kyphoscoliosis; a case report

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The Ehlers-Danlos syndrome, a disorder with many faces.

The Ehlers-Danlos syndromes (EDSs) comprise a heterogeneous group of diseases, characterized by fragility of the soft connective tissues and widespread manifestations in skin, ligaments, joints, blood vessels and internal organs. The clinical spectrum varies from mild skin and joint hyperlaxity to severe physical disability and life-threatening vascular complications. The current Villefranche c...

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Twenty patients with the Ehlers-Danlos syndrome, (10 type I, six type II, and four type IV) were studied to assess the frequency of respiratory abnormalities in this condition. Five patients (25%) had had at least one episode of haemoptysis, but none had any defect of coagulation. There was a high frequency of recurrent sinusitis, notably in those with the type I syndrome. Two patients had bull...

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Ehlers-Danlos syndrome.

448 BMJ | 1 SEPTEMBER 2007 | VoluME 335 hinder implementation of these recommendations. Recognising the condition and the impact it can have on the lives of patients and their families is therefore a necessary preliminary to improving care. • People with severe CFS/ME who are housebound or need prolonged bed rest require specialised care, but evidence is lacking about which treatments are most ...

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عنوان ژورنال

دوره 11  شماره 2

صفحات  75- 76

تاریخ انتشار 2019-06

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