LIMIT DEXTRINOSIS Meaning and
Definition
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Limit dextrinosis is a rare genetic disorder that affects an individual's ability to properly digest certain complex carbohydrates. It is a type of glycogen storage disease characterized by a deficiency of the enzyme amylo-1,6-glucosidase, also known as debranching enzyme. This enzyme plays a crucial role in breaking down glycogen, which is a form of stored glucose in the body.
In individuals with limit dextrinosis, the enzyme deficiency leads to the accumulation of a specific type of branched glycogen structure called limit dextrins. These limit dextrins cannot be completely broken down into glucose molecules, which results in an impairment of glycogen metabolism. As a result, affected individuals experience difficulty digesting and utilizing complex carbohydrates such as starch and maltose.
The symptoms of limit dextrinosis typically appear in infancy or early childhood and may vary in severity. Common symptoms include liver enlargement, hypoglycemia (low blood sugar), and growth retardation. Individuals may also experience recurrent episodes of metabolic disturbances, such as ketotic hypoglycemia, which can result in seizures, lethargy, and other neurological symptoms.
Treatment for limit dextrinosis is primarily focused on managing the symptoms. A specialized diet low in complex carbohydrates and high in simple sugars is often recommended to ensure adequate nutrition and prevent hypoglycemia. Regular monitoring of blood glucose levels and liver function is essential to gauge the effectiveness of dietary management and adjust treatment as needed. In some cases, liver transplantation may be considered as a treatment option.
Common Misspellings for LIMIT DEXTRINOSIS
- kimit dextrinosis
- pimit dextrinosis
- oimit dextrinosis
- lumit dextrinosis
- ljmit dextrinosis
- lkmit dextrinosis
- lomit dextrinosis
- l9mit dextrinosis
- l8mit dextrinosis
- linit dextrinosis
- likit dextrinosis
- lijit dextrinosis
- limut dextrinosis
- limjt dextrinosis
- limkt dextrinosis
- limot dextrinosis
- lim9t dextrinosis
- lim8t dextrinosis
- limir dextrinosis
- limif dextrinosis
