Diffuse Liver Pathology- Glycogen Storage Disease

Glycogen storage disease is also called _________________.

Glycogen storage disease is an _______________ __________________ disorder of carbohydrate metabolism.

The underlying problem in all of the glycogen storage diseases is the __________ and _____________ of glycogen.

Glucose is a major source of energy for the body; it is stored in the form of ______________ and later released with the help of ____________.

People with Glycogen storage disease have an inherited absence or deficiency of any of the ______________ responsible for forming or releasing glycogen as it is needed by the body during exercise and/or between meals.

The enzyme defect leads to abnormal tissue concentrations of _____________ or to structurally _______________ forms of glycogen

There are about 12 known types of GSD but the most common type is ________ _____________ disease, or type _____(#).

Type 1 GSD results from a defect in the enzyme glucose 6- phosphatase allowing excessive _______________ of glycogen in the liver, intestinal tract and kidnets.

In GSD 1, the breaking down of _____________ into _______________ does not occur.

As a result of GSD 1, the liver is clogged with excess ____________ and becomes _______________ and _______________.

Von Gierke's disease usually occurs in ___________ and ______________ _________________.

Clinically, symptoms include __________________ and ____________ levels of glucose 6-phosphatase, and elevated levels of lactic acid, ______________, and uric acid in the blood.

____________ are stored in the liver along with the _____________ which leads to ________________.

Clinically, growth is ____________, puberty is often _________, and bones may be ______________ by __________________.

Glycogen storage disease- clinicallyBlood platelets are also affected and frequent ______________ and easy ________________ are common.

Glycogen storage disease- clinicallyPrimary symptoms ______________ with age, but after age 20-30, liver tumors, liver cancer, chronic renal disease, and gout may appear.

Glycogen storage disease- Sono appearanceType 1 GSD is indistinguishable from any other cause of diffuse fatty infiltration so there will be a diffuse ______________ in liver parenchyma echogenicity and ______________ penetration.

Glycogen storage disease- sono appearanceThere will be _________________.

GSD is also associated with hepatic _________________.

Types 3 and 4 GSD is mostoften associated with ________________ and ____________________ ____________________.

Treatment of Glycogen storage disease depends on the severity of the disease  and includes simple ____________ management, to more severe ____________ _____________. Glycogen storage disease can result in _____________.