welcome to oneEDSvoice
- a positively charged Ehlers Danlos Syndrome community.- join today!
- login
Bullous Emphysema as First Presentation of Ehlers-Danlos Syndrome in Monozygotic Twins
source: Respiratory Medicine Case Reports
year: 2014
authors: Ruggeri P, Calcaterra S, Girbino G
summary/abstract:Ehlers-Danlos syndrome, characterized by hyperextensible skin, hypermobile joints, and fragile vessels, is the most common heritable disorder of connective tissue and has an estimated prevalence of 1 in 5000. Pulmonary involvement with signs of lung destruction (bullous emphysema) as first presentation is unusual. We report a case of monozygotic twins 37 years old men with occasional evidence of bullous emphysema with previously undiagnosed Ehlers-Danlos syndrome type IV. We emphasize the importance of considering uncommon genetic causes of emphysema in young adults, discuss underlining pathophysiological mechanisms and propose a conservative management and follow-up.
organization: Policlinico Universitario G.Martino MessinaDOI: 10.1016/j.rmcr.2014.12.002
read more full text source
expertly curated content related to this topic
-
Broadening the Spectrum of Ehlers Danlos Syndrome in Patients with Congenital Adrenal HyperplasiaCONTEXT: The contiguous gene deletion s...
-
The Effectiveness of Interventions Focused on Functional Stability on the Level of Pain in Patients with Joint Hyper...This review will consider both experimen...
-
Xiaoyan Wang, MDDr. Wang graduated from the Dalian Medic...
-
Hospital PrincessThese are the ramblings of my chronic, 2...
-
The aftermath of the Holidays with EDS and POTSFor many people the holidays are full of...
-
MidAtlantic Urology Associates, LLCMAUA (MidAtlantic Urology Associates, LL...
-
Classifications of EDS: Hypermobility Typehttps://www.youtube.com/watch?v=nSO1Sut1...