Argininosuccinic Aciduria
Argininosuccinic Aciduria is a heritage disorder that causes ammonia to accumulate in the blood. Ammonia, which is formed when the proteins are broken down in the body, if the level becomes too much then it is poisonous. The nervous system is particularly susceptible to the effects of extra ammonia.
Argininosuccinic Aciduria usually becomes apparent in the first few days of life. Argininosuccinic Aciduria may be reluctant to eat or eat an infant energy (sluggish) with, and controlled bad breath rate or body temperature. With this disorder some children experience seizures or abnormal body activities, or go into coma. Complications from Argininosuccinic Aciduria may include delay in development and intellectual inefficiency. Progressive liver damage, skin lesion, and brittle hair can also be seen.
Occasionally, a person may be the heir apparent to the mild form of the disorder, in which blood pressure is stored in blood flow only during the period of illness or other stress.
Argininosuccinic Aciduria is a heritage disorder that causes ammonia to accumulate in the blood. Ammonia, which is formed when the proteins are broken down in the body, if the level becomes too much then it is poisonous. The nervous system is particularly susceptible to the effects of extra ammonia. Argininosuccinic Aciduria usually becomes apparent in the first few days of life. Argininosuccinic Aciduria may be reluctant to eat or eat an infant energy (sluggish) with, and controlled bad breath rate or body temperature. With this disorder some children experience seizures or abnormal body activities, or go into coma. Complications from Argininosuccinic Aciduria may include development delays, intellectual inefficiency, progressive liver damage, skin lesion, and brittle hair. This condition is due to mutation in the ASL gene and it is inherited in an autosomal recessive pattern. Long-term management includes dietary restriction of protein and supplemented with arginine. Closing oral protein intake, supplementing oral intake with intravenous lipids and / or glucose, and acute crisis can be treated by using intravenous arginine and nitrogen scavenging therapy. If ammonia levels are not normal, then hemodialysis may be necessary. Read More
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