maple syrup urine disease
This was first described in 1954 the disease g. In English and American literature, it is more known as a disease maple syrup because of the smell of urine, which is similar to the smell of maple syrup from the tree.
This hereditary disease is associated withmetabolic disorders of amino acids such as leucine, isoleucine, valine: child's body can not assimilate the amino acids. The characteristic smell of urine is associated with the presence of a substance produced from leucine.
The disease begins to emerge in 3-14 days after birth: the baby refuses to eat, quietly crying, may be frequent vomiting, and even vomiting.
If you do not seek medical help, candevelop twitching of individual muscle groups, muscle tone increases, in connection with which the whole body is stretched and the lower limbs are crossed. The disease progresses rapidly and in most cases ends in death in the first year of life. If children live to a later age, the main manifestation of the disease is mental retardation.
In severe illness can occurdisordered breathing and consciousness, and if the first weeks of life, treatment is not carried out, poor prognosis. Such children are dramatically lagging behind in weight and psychomotor development.
Diagnosis is characterized by the symptoms of the disease, the smell of urine and the content of leucine, isoleucine, valine urine.
It consists in the reduction of leucine, isoleucine andvaline in blood plasma, which uses a special diet in which the protein is assigned instead of a special medicine, a mixture consisting of 18 amino acids in the same proportion as in human milk. Fats are present in such a mixture of corn oil and carbohydrates are replaced with maltose-dextrin. minerals and vitamins are also added. As soon as the general condition of the child improved appetite appears, normalizes muscle tone, is introduced into the diet of a small amount of cow's milk. Fruits and vegetables, which also contain the protein, but a small amount is allowed to give the child since 3 months. In 6-8 months ration administered gelatin which does not contain said amino acids. With 9.5 months of a daily serving of milk is 120 ml.
Leucine and isoleucine content in products (in grams per 100 g of product), respectively:
1. Milk female - 0.108 and 0.062.
2. Milk cow - 2.278 and 0.182.
3. Kefir - 0.263 and 0.173.
4. Cottage cheese - 0.924 and 0.548.
5. Eggs - 1.130 and 0.83.
6. Meat Beef - 1.730 and 1.06.
7. Meat Chicken - 1,620 and 1,117.
8. Beef Liver - 1,543 and 0,800.
9. Cod - 1.222 and 0.879.
10 Rice groats - 1.008 and 0.369.
11. Semolina - 0.364 and 0.258.
12 Buckwheat - 0.702 and 0.301.
13. oat groats - 0.672 and 0.302.
14. Millet - 1,040 and 0,244.
15. Pearl barley - 0.584 and 0.258.
16. Peas - 1.204 and 0.780.
17. Wheat flour - 0.567 and 0.29.
18. Pasta - 0.690 and 0.380.
19. Rye bread - 0.275 and 0.146.
20. Bread, wheat - 0.550 and 0.25.
21. Cookies - 0.357 and 0.171.
22. Potatoes - 0.107 and 0.083.
23. Carrots - 0.061 and 0.034.
24. Cabbage - 0,059 and 0,040.
25. Oranges - 0.019 and 0.019.
26. Lemons - 0,013 and 0,016.
27. Apple juice - 0.015 and 0.009.
28. Orange juice - 0.150 and 0.010.
29. Lemon Juice - 0.016 and 0.014.