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"Natural medicine is really all about common sense."

Diana Mossop

3-methyl glutaconic aciduria

Definition

Aagenaes syndrome is a condition which is characterized by inborn hypoplasia of the lymph vessels. This eventually causes recurring cholestasis on the formative years lymphedema on the legs. This could develop into hepatic cirrhosis and giant cell hepatitis (which could occur with portal tract fibrosis). The name of Aagenaes syndrome was derived from a pediatrician in Norway named Oystein Aagenaes. The name was finalized in 1968 when the condition was first described by Aagenaes. It is sometimes called cholestasis-lymphedema syndro

Symptoms and Signs

Dysphagia lusoria is a type of dysphagia which is caused by an abnormal subclavian artery on the right. Ortner's syndrome is known as recurring laryngeal nerve palsy. The defective right subclavian artery often occurs from an open portion of the proximal descending aorta which is also termed as Kummerel or Diverticulum. In both cases of subclavian arteries, the vessel passes behind the esophagus. An impression is made on the back portion of the esophagus by the aberrant right subclavian artery while the left subclavian artery is often the one that causes anterior dislocation and acute compression on the esophagus. The left subclavian artery often causes compression of the airways. The right aberrant artery could be linked with adult dysphagia. The left aberrant artery is the one that usually causes symptoms on the obstruction of the esophagus and airw

Epidemiology

The mean age of the start of chorea-acanthocystosis or ChAc is approximately at age 35. There are a few cases, though, where it can occur as early as the initial decade or it can be as late as the 70th year of life. It runs a persistent progressive course and it may end up as a major disability in just a few years. Some patients become dependent on wheelchairs or become bedridden just on the third decade. It is certain that the individual's life expectancy will be reduced; there are even cases of unexplained or sudden deaths when the patient goes through an epileptic seizure. The range of the patients' ages at death is at 28-61 years.on or race.

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