Understanding vascular-type Ehlers-Danlos syndrome and avoiding vascular complications

Jocelyn Carter1, Andrew Z Fenves1

  • 1Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.

Proceedings (Baylor University. Medical Center)
|January 28, 2017
PubMed

Insights

Vascular-type Ehlers-Danlos syndrome (EDS), a rare connective tissue disorder, presents diagnostic and treatment challenges. This report details two cases of this severe inherited condition.

Area of Science:

  • Genetics
  • Medicine
  • Pathology

Background:

  • Vascular-type Ehlers-Danlos syndrome (EDS) is a rare, life-threatening inherited connective tissue disorder.
  • It stems from mutations in the gene encoding type III procollagen.
  • This subtype of EDS has the highest mortality rate among all EDS types.

Related Concept Videos

Peripheral Artery Disease V: Postoperative Nursing Management01:23

Peripheral Artery Disease V: Postoperative Nursing Management

During the postoperative period, it is crucial to focus on maintaining circulation, identifying and managing potential complications, and planning for discharge.Nursing AssessmentVital signs monitoring: Regularly monitor vital signs, including blood pressure, heart rate, respiratory rate, and temperature, to detect early signs of complications such as bleeding and infection.Circulation assessment: Monitor pulses, perform Doppler assessments, and check capillary refill, color, temperature, and...
490
Venous Thrombosis I: Introduction01:30

Venous Thrombosis I: Introduction

Venous thrombosis, the most common disorder of the veins, involves the formation of a thrombus or blood clot associated with vein inflammation. It can be classified as either superficial vein thrombosis or deep vein thrombosis.Superficial Vein Thrombosis: This involves the formation of a thrombus in a superficial vein, usually the greater or lesser saphenous vein. Though less severe than deep vein thrombosis (DVT), SVT can lead to complications if untreated.Deep Vein Thrombosis (DVT): This...
601
Venous Thrombosis IV: Nursing Management01:30

Venous Thrombosis IV: Nursing Management

Nursing management begins with a thorough assessment of the patient's health history. Key factors include trauma to veins, peripherally inserted central catheters, varicose veins, recent pregnancy or childbirth, surgery, bacteremia, prolonged bed rest, atrial fibrillation, COPD, heart failure, cancer, coagulation disorders, myocardial infarction, spinal cord injury, stroke, prolonged travel, recent bone fractures, and dehydration. Review medication intake, particularly oral contraceptives,...
341
Esophageal Varices-I: Introduction01:24

Esophageal Varices-I: Introduction

Esophageal varices are dilated, tortuous veins which are found mainly in the submucosa of the lower esophagus but which may also appear higher up or extend into the stomach. They develop due to increased pressure in the portal venous system, often as a result of liver cirrhosis. This condition scars and damages the liver, impeding normal blood flow through the portal vein. To compensate, blood seeks alternative pathways, forming fragile new vessels (varices) in the esophagus and stomach. These...
1.8K
Vascular Spasm01:16

Vascular Spasm

The vascular phase, also known as vasospasm, is the initial stage of hemostasis, crucial for preventing excessive bleeding when a blood vessel is injured. After a vessel is cut, nerves in the damaged area trigger pain and other sensory impulses. Simultaneously, the smooth muscles in the vessel wall contract, resulting in a vascular spasm. This contraction reduces the vessel's diameter at the injury site, slowing or stopping blood loss through the vessel wall. Vascular spasms typically last...
4.4K
Disorders of Hemostasis01:24

Disorders of Hemostasis

Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
2.6K