Ehlers-Danlos syndrome
Definition:
Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls. Connective tissue is a complex mixture of proteins and other substances that provides strength and elasticity to the underlying structures in your body.
People who have Ehlers-Danlos syndrome usually have overly flexible joints and stretchy, fragile skin. This can become a problem if you have a wound that requires stitches, because the skin often isn't strong enough to hold them.
A more severe form of the disorder, called vascular Ehlers-Danlos syndrome, can cause the walls of your blood vessels, intestines or uterus to rupture. If you have vascular Ehlers-Danlos syndrome, you may want to talk to a genetic counselor before starting a family.
Symptoms:
Signs and symptoms of the most common form of Ehlers-Danlos syndrome include:
Vascular Ehlers-Danlos syndrome
People who have the vascular subtype of Ehlers-Danlos syndrome often share distinctive facial features of a thin nose, thin upper lip, small earlobes and prominent eyes. They also have thin, translucent skin that bruises very easily. In fair-skinned people, the underlying blood vessels are very visible through the skin.
One of the most severe forms of the disorder, vascular Ehlers-Danlos syndrome can weaken your heart's largest artery (aorta), as well as the arteries to your kidneys and spleen. A rupture of any of these blood vessels can be fatal. The vascular subtype also can weaken the walls of the uterus or large intestines — which may also rupture.
Causes:
Different subtypes of Ehlers-Danlos syndrome are associated with a variety of genetic causes, some of which are inherited and passed on from parent to child.
If you have some subtypes of Ehlers-Danlos syndrome, such as the vascular variety, there's a 50 percent chance that you'll pass the gene on to each of your children.
Complications:
Complications depend on the types of signs and symptoms you have. For example, overly flexible joints can result in joint dislocations and early-onset arthritis. Fragile skin may develop prominent scarring.
People who have vascular Ehlers-Danlos syndrome are at risk of often fatal ruptures of major blood vessels. Some organs, such as the uterus and intestines, also may rupture. Pregnancy can increase these risks.
Treatments and drugs:
There is no cure for Ehlers-Danlos syndrome, but treatment can help you manage your symptoms and prevent further complications.
Medications
Your doctor may prescribe drugs to help you control:
Joints with weak connective tissue are more likely to dislocate. Exercises to strengthen the muscles around a joint can help stabilize the joint. Your physical therapist might also recommend specific braces to help prevent joint dislocations.
Surgical and other procedures
In rare cases, surgery is recommended to repair joints damaged by repeated dislocations. However, your skin and the connective tissue of the affected joint may not heal properly after the surgery.
Definition:
Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls. Connective tissue is a complex mixture of proteins and other substances that provides strength and elasticity to the underlying structures in your body.
People who have Ehlers-Danlos syndrome usually have overly flexible joints and stretchy, fragile skin. This can become a problem if you have a wound that requires stitches, because the skin often isn't strong enough to hold them.
A more severe form of the disorder, called vascular Ehlers-Danlos syndrome, can cause the walls of your blood vessels, intestines or uterus to rupture. If you have vascular Ehlers-Danlos syndrome, you may want to talk to a genetic counselor before starting a family.
Symptoms:
Signs and symptoms of the most common form of Ehlers-Danlos syndrome include:
- Overly flexible joints. Because the connective
tissue that holds joints together is looser, your joints can move far
past the normal range of motion. Small joints are affected more than
large joints. You might also be able to touch the tip of your nose with
your tongue.
- Stretchy skin. Weakened connective tissue allows
your skin to stretch much more than usual. You may be able to pull a
pinch of skin up away from your flesh, but it will snap right back into
place when you let go. Your skin might also feel exceptionally soft and
velvety.
- Fragile skin. Damaged skin often doesn't heal well.
For example, the stitches used to close a wound often will tear out and
leave a gaping scar. These scars may look thin and crinkly.
- Fatty lumps at pressure points. These small, harmless growths can occur around the knees or elbows and may show up on X-rays.
Vascular Ehlers-Danlos syndrome
People who have the vascular subtype of Ehlers-Danlos syndrome often share distinctive facial features of a thin nose, thin upper lip, small earlobes and prominent eyes. They also have thin, translucent skin that bruises very easily. In fair-skinned people, the underlying blood vessels are very visible through the skin.
One of the most severe forms of the disorder, vascular Ehlers-Danlos syndrome can weaken your heart's largest artery (aorta), as well as the arteries to your kidneys and spleen. A rupture of any of these blood vessels can be fatal. The vascular subtype also can weaken the walls of the uterus or large intestines — which may also rupture.
Causes:
Different subtypes of Ehlers-Danlos syndrome are associated with a variety of genetic causes, some of which are inherited and passed on from parent to child.
If you have some subtypes of Ehlers-Danlos syndrome, such as the vascular variety, there's a 50 percent chance that you'll pass the gene on to each of your children.
Complications:
Complications depend on the types of signs and symptoms you have. For example, overly flexible joints can result in joint dislocations and early-onset arthritis. Fragile skin may develop prominent scarring.
People who have vascular Ehlers-Danlos syndrome are at risk of often fatal ruptures of major blood vessels. Some organs, such as the uterus and intestines, also may rupture. Pregnancy can increase these risks.
Treatments and drugs:
There is no cure for Ehlers-Danlos syndrome, but treatment can help you manage your symptoms and prevent further complications.
Medications
Your doctor may prescribe drugs to help you control:
- Pain. If over-the-counter pain relievers — such as ibuprofen (Advil, Motrin IB, others) and naproxen (Aleve) — aren't enough, your doctor may prescribe stronger medications for your joint or muscle pain.
- Blood pressure. Because blood vessels are more fragile in some types of Ehlers-Danlos syndrome, your doctor may want to reduce the stress on the vessels by keeping your blood pressure low.
Joints with weak connective tissue are more likely to dislocate. Exercises to strengthen the muscles around a joint can help stabilize the joint. Your physical therapist might also recommend specific braces to help prevent joint dislocations.
Surgical and other procedures
In rare cases, surgery is recommended to repair joints damaged by repeated dislocations. However, your skin and the connective tissue of the affected joint may not heal properly after the surgery.
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