Medical Equipments Reviews

Archive for the ‘Adrenoleukodystrophy (ALD)’ Category

ENT meeting equipped with:

. Intake system / air
. Light source
. Irrigation system headset
. Vestibular stimulation system
. Microscope
. Coagulation Devices
. etc. …

Equipment:

. Diagnosis strobe
. Strobe and endoscopic diagnosis, incl. video documentation
. Measurement of nasal resistance (rhinomanometry)
. Irrigation ear / vestibular diagnosis

Glycogen Storage Disease VIII is a hereditary and congenital disease in which a change in the genetic material can not be broken glycogen by the body. Glycogen, the form of sugar stored in muscles and liver. The substance is broken down by certain enzymes enzymes accompany chemical reactions. If one of these enzymes is missing or malfunctioning, poorly, or glycogen will not be interrupted. This creates an accumulation of this substance in the cells. The effects of glycogen and vary markedly depending on the type of enzyme involved.

Glycogen Storage Disease VIII is caused by a lack of activity of the liver enzyme phosphorylase b kinase. The symptoms of this disorder are usually evident in infancy, and are very mild. Features include an enlarged liver and a slightly decreased blood sugar (hypoglycemia). Growth may be delayed. While sometimes inflammation of the liver can occur, the function of this organ is usually normal. The symptoms of glycogen storage disease VIII usually disappear gradually with age, so adults are usually free of symptoms. This is the condition sometimes not detected.

Adrenoleukodystrophy (ALD) is a hereditary congenital disorder, involving degradation of the white matter in the brains (leukodystrophy) both in children and adults. Moreover, the most common peroxisomal disorder. In peroxisomal disorders, there is a change in the genetic material a disorder in the peroxisomes . Peroxisomes are small organs (organelles) in a cell, using enzymes – enzymes-guiding chemical reactions. ensure the degradation of waste. This disease affects the degradation of waste disturbed, so they accumulate in the cells. The accumulation of waste is toxic and difficult operation of the cells. This finally causes damage to organs and tissues.

ALD is characterized by a progressive degradation of insulation of the nerves in the central nervous system (brains and spinal cord) that is associated with insufficient functioning of the adrenal glands. This causes various symptoms, including paralysis and impaired motor control (ataxia). Read the rest of this entry »