[ceres]

In glycogen storage disease type I, why is it that the patient do not exhibit ketosis (do not have ketone bodies in their urine) and has high blood fatty acids and triglycerides?

[JGK]

In this case Google and Wikipedia isare your friends

http://en.wikipedia.org/wiki/Glycogen_storage_disease_type_I

[Suleman]

Dear!
     Before jumping into the topic I would like to tell you what is Ketosis, Ketonemia and ketonuria!

Ketonemia is elevated blood ketone bodies
ketonuria is elevated Urine ketone bodies
While ketosis = ketonemia + ketonuria

In glycogen type 1 Storage disease ketonemia occurs but ketonuria does not!

WHY KETONEMIA BUT NOT KETONURIA!

This can be well explained by compraing the Insulin Dependent Diabetic mellitus and your cited diease.

In Diabetic mellitus type 1 the WHOLE body does not depends solely on fats but most of the tissues except muscle can get GLUCOSE from blood! So ketone bodies are used by muscle etc (insulin depenednt tissues) and REST of the ketone bodies are remove by kidney resulting in ketonuria. In simple terms The WHOLE BODY is not using the ketone bodies but the insulin dependent tissues are usining ketone bodies from blood, So ketone bodies usage is less compartively

In Glycogen storage dieases the liver cannot provide carbohydare during fasting as it cannot spare free glucose from its phophorylated form! so the WHOLE body is TOTALY dependent on Ketolysis and TAGs. so the use of ketone bodies is more in this type of disease so it is secreted by kidney so no ketonuria occurss, but ketone bodies level in blood is elevated!

[YogaEarth]

We are new to the board and are looking forward to being a part of the discussions!
~YogaEarth