Homocystinuria
Contents:
Description:
Homocystinuria (the Greek hom[oi]os similar, the same + cystine + the Greek uron wetting) — the hereditary disease caused by methionine exchange disturbance, which is characterized by defeat of connecting fabric, nervous, bone, muscular and cardiovascular systems.
Homocystinuria symptoms:
Are shown gradually within the first year of life of the child in the form of slight lag in the weight and growth. At the same time appetite of the child remains normal, functions of digestive tract do not suffer, however all attempts to improve the child's food additional administration of protein in the form of cottage cheese or kefir only aggravate situation.
Note late closing of a fontanel, a curvature of extremities, the child is irritable, whining, it interrupts a sleep, and deficit of body weight increases.
Outward a reenka with a homocystinuria
Homocystinuria reasons:
As a result of decrease of the activity of special enzyme of a liver (tsistationinsintetaza) in an organism of the child the methionine and homocystine having the damaging effect on a row of systems of an organism collect (bone and central nervous systems) that leads to development of a homocystinuria.
Treatment of the Homocystinuria:
There are two forms of this disease, one of which will respond to treatment high doses (50 — 500 mg a day) B6 vitamin, and another demands dietary food. The diet has to be low-proteinaceous with the low content of methionine and additional administration of calcium, iron and vitamins.
The amount of methionine in clinical nutrition makes 29 — 45 mg on 1 kg of body weight of the child that is reached by an exception (or decrease) from a diet of animal products.
The so-called gelatinous candies containing mix of amino acids, protein-free bread, sucrose, a dextrin maltose belong to special products.
The offered natural products: gelatin, milk, corn flakes, rice, lentil, peanut and corn oils, potatoes, other vegetables and fruit.
Products with the high content of methionine: cottage cheese, cheese, egg, meat beef, meat of a rabbit, chicken meat, herring, cod, beef liver, kidneys beef, soy, peas, wheat flour.
Products with the low content of methionine: milk cow, goat, women's, rice, carrots, beet, potatoes, fresh green peas and tinned, tomatoes, bananas, oranges, tangerines.
