Post by Master Kim on Dec 18, 2014 19:03:39 GMT -5
Ehlers–Danlos syndrome (EDS) - en.wikipedia.org/wiki/Ehlers%E2%80%93Danlos_syndrome
Ehlers–Danlos syndrome (EDS) is an inherited connective tissue disorder with different presentations that have been classified into several primary types. EDS is caused by a defect in the structure, production, or processing of collagen or proteins that interact with collagen, such as mutations in the COL5A or COL3A genes. (This is the collagen of granulation tissue, and is produced quickly by young fibroblasts before the tougher type I collagen is synthesized. Commonly associated with keloid formation. Reticular fiber. Also found in artery walls, skin, intestines and the uterus.--COL3A1). The collagen in connective tissue helps tissues resist deformation. Collagen is an important contributor to the physical strength of skin, joints, muscles, ligaments, blood vessels and visceral organs; abnormal collagen renders these structures more elastic. In some cases, the severity of the mutation can be life-threatening.
There is no cure for EDS, and treatment is supportive, including close monitoring of the digestive, excretory and particularly the cardiovascular systems. Occupational and physical therapy, bracing, and corrective surgery may help with the frequent injuries and pain that tend to develop in certain types of EDS, although extra caution and special practices are advised to prevent permanent damage.
People who suffer from EDS are often misdiagnosed with hypochondriasis, depression, chronic fatigue syndrome and other conditions because EDS is considered an invisible disability and there is generally poor knowledge about EDS among practitioners. Many sufferers of EDS have psychological difficulties as a result of frustration with the medical system and the socially inconvenient combination of appearing normal while being in severe pain. To deal with these issues, interest groups have tried to raise awareness about EDS among medical professionals.
The features of EDS were first described by Hippocrates in 400 BC. The syndrome is named after two physicians, Edvard Ehlers from Denmark, and Henri-Alexandre Danlos from France, who described it at the turn of the 20th century.
Classification...
Not that important to KOSA
The collagen fibril and EDS.
(a) Normal collagen fibrils are of uniform size and spacing. Fibrils from a patient with dermatosparaxis
(b) show dramatic alterations in fibril morphology with severe effects on tensile strength of connective tissues. Fibrils from a patient with classical EDS
(c) may show composite fibrils (arrows). Fibrils from a TNX-deficient patient
(d) are uniform in size and no composite fibrils are seen. TNX-null
(e) fibrils are less densely packed and not as well aligned to neighboring fibrils.
Individual with EDS displaying hypermobile joints
Individual with EDS displaying skin hyperelasticity
Signs and symptoms
Signs vary widely based on which type of EDS the patient has. In each case, however, the signs are ultimately due to faulty or reduced amounts of collagen. EDS typically affects the joints, skin, and blood vessels. Following is a list of major signs and symptoms.
Ehlers–Danlos syndrome (EDS) is an inherited connective tissue disorder with different presentations that have been classified into several primary types. EDS is caused by a defect in the structure, production, or processing of collagen or proteins that interact with collagen, such as mutations in the COL5A or COL3A genes. (This is the collagen of granulation tissue, and is produced quickly by young fibroblasts before the tougher type I collagen is synthesized. Commonly associated with keloid formation. Reticular fiber. Also found in artery walls, skin, intestines and the uterus.--COL3A1). The collagen in connective tissue helps tissues resist deformation. Collagen is an important contributor to the physical strength of skin, joints, muscles, ligaments, blood vessels and visceral organs; abnormal collagen renders these structures more elastic. In some cases, the severity of the mutation can be life-threatening.
There is no cure for EDS, and treatment is supportive, including close monitoring of the digestive, excretory and particularly the cardiovascular systems. Occupational and physical therapy, bracing, and corrective surgery may help with the frequent injuries and pain that tend to develop in certain types of EDS, although extra caution and special practices are advised to prevent permanent damage.
People who suffer from EDS are often misdiagnosed with hypochondriasis, depression, chronic fatigue syndrome and other conditions because EDS is considered an invisible disability and there is generally poor knowledge about EDS among practitioners. Many sufferers of EDS have psychological difficulties as a result of frustration with the medical system and the socially inconvenient combination of appearing normal while being in severe pain. To deal with these issues, interest groups have tried to raise awareness about EDS among medical professionals.
The features of EDS were first described by Hippocrates in 400 BC. The syndrome is named after two physicians, Edvard Ehlers from Denmark, and Henri-Alexandre Danlos from France, who described it at the turn of the 20th century.
Classification...
Not that important to KOSA
The collagen fibril and EDS.
(a) Normal collagen fibrils are of uniform size and spacing. Fibrils from a patient with dermatosparaxis
(b) show dramatic alterations in fibril morphology with severe effects on tensile strength of connective tissues. Fibrils from a patient with classical EDS
(c) may show composite fibrils (arrows). Fibrils from a TNX-deficient patient
(d) are uniform in size and no composite fibrils are seen. TNX-null
(e) fibrils are less densely packed and not as well aligned to neighboring fibrils.
Individual with EDS displaying hypermobile joints
Individual with EDS displaying skin hyperelasticity
Signs and symptoms
Signs vary widely based on which type of EDS the patient has. In each case, however, the signs are ultimately due to faulty or reduced amounts of collagen. EDS typically affects the joints, skin, and blood vessels. Following is a list of major signs and symptoms.
- Musculoskeletal
- Thoracic outlet syndrome sprain, dislocation, subluxation and hyperextension
- Skin
- Cardiovascular
- Other manifestations or complications...
Treatment can start from either one of Ascetic Saahm's formula #1 or #3.
However, only #1 is described here.
Based on Ascetic Saahm's formula #1, fostering large intestine,
foster SP3 and LU9 and
subdue LU10, ST41, SP2, BL60, KI2, GB38 and LR2.