ehlersdanlos syndrome

Ehlers-Danlos Syndrome

EDS, Ehlers-Danlos-Meekeren syndrome, Van-Meekeren syndrome, fibrodysplasia elastica generalisata, dermatolysis, cutis hyperelastica, “rubber skin”, among others Franz. Laxité articulaire congénitale multipleEngl: Danlos’ syndrome, Meekeren-Ehlers-Danlos syndrome, Chernogubov’s syndrome, Sack’s syndrome, Sack-Barabas syndrome, Van Meekeren’s syndrome IRussian: Chernogubov syndrome Definition/Introduction Ehlers-Danlos Syndrome (EDS) is a group of heterogeneous, genetic connective tissue diseases caused by disorders in the synthesis … Ehlers-Danlos Syndrome

Causes | Ehlers-Danlos Syndrome

Causes The cause of the disease is a genetic defect. A change (mutation) in the genes that describe the structural protein collagen on the human genome, the DNA, occurs. The mutation leads to an altered structure and/or reduced synthesis of collagen, which results in a reduced strength of the entire connective tissue. Types I and … Causes | Ehlers-Danlos Syndrome

Diagnosis | Ehlers-Danlos Syndrome

Diagnosis The diagnosis is based on the clinical appearance, symptoms, and is supplemented by a family examination (family history). In addition, a skin biopsy can be performed, in which the removed skin tissue is examined with an electron microscope and its collagen structure is assessed. Differentiation into the different types of Ehlers-Danlos syndrome is done … Diagnosis | Ehlers-Danlos Syndrome

Prognosis | Ehlers-Danlos Syndrome

Prognosis Patients with Ehlers-Danlos syndrome generally have a normal life expectancy. However, the disease is progressive, i.e. it leads to an ever worsening of the health condition.Skin wounds and dislocations of the joints impair the patient’s quality of life, while ruptures of the large vessels can be life-threatening. Ehlers-Danlos syndrome is a chronic disease for … Prognosis | Ehlers-Danlos Syndrome

Diagnosis | Torn aorta

Diagnosis The diagnosis of aortic rupture is not easy to make. However, if a rupture is suspected, action must be taken very quickly, as the death rate is very high, depending on the size and location of the rupture. A rupture or dilatation of the aorta can be diagnosed by ultrasound, especially by swallowing ultrasound, … Diagnosis | Torn aorta

Prognosis of aortic rupture | Torn aorta

Prognosis of aortic rupture The forecast depends on many factors. The larger the crack is, the later it is detected and the more unfavourable the location, the death rate can be over 80%. If the aortic tear is treated early, the mortality rate can drop to 20%. The chances of survival in the event of … Prognosis of aortic rupture | Torn aorta

Torn aorta

Introduction The aorta is the main artery and runs from the heart to the legs, where it splits. A rupture of the aorta is life-threatening because even a small tear can lead to mass bleeding in seconds. The tear of the aorta is relatively rare, it is indicated in the literature with about 5/100. 000. … Torn aorta

Causes | Torn aorta

Causes There are two causes for a rupture of the aorta. In principle, accidents can cause the aorta to rupture, but this is extremely rare because the aorta is relatively protected inside the body. A much more common reason for a rupture of the aorta is an aortic aneurysm. An aneurysm is an enlargement of … Causes | Torn aorta