The Glycogenosis Type 1a

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The Glycogenosis type 1a, also known as Von Gierke’s disease, is in a group of diseases caused by alteration of metabolism of glycogen in the organism. The glycogen is one of the most important energy sources and glucose reserve in the liver. The GSD is a genetic disease considered rare and affects about 1 in 50.000 people.

The metabolic complications of the disease include extreme hipoglicemy (low sugar in the blood), cholesterol, triglycerides and latici acid increased in the blood, increased liver size and low stature. The treatment requires a ruled and hard diet, eating cornstarch every 4-6 hours with restriction on consumption of fructose, maltose and glucose. Eating cornstarch stabalizes glucose levels, preventing hipoglicemy and normalizing levels of fat that could form due to the poor control of metabolism.
Click here to learn more about the disease and to meet the “Associação Brasileira de Glicogenose”.

text reviewed by Dra. Carolina Fishenger Souza