What caused hyperglycinemium disease? What are the symptoms of this disease? How to treat him? Read in this article.
Content
Causes
Violation of the transformation of glycine into serine due to the insufficient function of the liver enzymes is the cause of the disease. In the urine of such a child, high content of glycine, as well as oxalates and ketone bodies.
Manifestations
The manifestations of the disease directly depend on the number of by-products (ketone bodies) of the exchange of glycine. From the first days of the child's life, vomiting may occur against the background of drowsiness or convulsion. Vomiting can be stubborn, and therefore dehydration comes without special treatment, then — Coma and death.
Treatment
It begins with the elimination of a acetional crisis, the symptom of which is vomiting. As a rule, such treatment is possible only in hospital. In the future, the child is prescribed a special diet consisting of a protein substitute (casein hydrolyzate) at the rate of 0.5 g per 1 kg of body weight per day.
To date, it is known that leucine, isoleucine, valine, methionine are toxic. But at the same time, other studies are also carried out, since this form of the disease is often combined with other exchange violations.
The content of glycine in products (in grams per 100 g of product):
one. Women's milk — 0.042.
2. Milk cow — 0.03.
3. Kefir — 0.056.
4. Cottage cheese — 0.184.
five. Chicken egg — 0.37.
6. Meat beef — 1,447.
7. Chicken meat — 1,519.
eight. Beef liver — 0.903.
nine. Cod — 0.525.
10. Rice croups — 0,630.
eleven. Semolina — 0.263.
12. Buckwheat — 0,796.
13. Oatmeal cereals — 0,453.
fourteen. Croup-ray — 0.220.
fifteen. Pearl cereals — 0.308.
sixteen. Peas — 0.480.
17. Wheat flour — 0.149.
18. Pasta — 0.215.
nineteen. Rye bread — 0.217.
twenty. Wheat bread — 0.264.
21. Biscuit — 0.172.
22. Potato — 0,053.
23. Carrot — 0.041.
24. White cabbage — 0,048.
25. Tomatoes — 0,029.
26. Oranges — 0.020.
27. Lemons — 0.016.
28. Apple juice — 0.018.
29. Orange juice — 0.018.
thirty. Lemon juice — 0.013.
