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Glycogen Storage Disease      

Common names or abbreviations:

bulletGlycogen storage disease
bulletglycogenoses

Description or definition:

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Glycogen storage diseases are also called glycogenoses.   The conditions are very rare and involve an inherited absence or deficiency of enzymes responsible for releasing glycogen as it is needed by the body during exercise and/or between meals.  The body needs glucose in order to perform everyday functions.  The stored form of glucose is called glycogen.  When glucose is needed by the body, glycogen is released and broken down by an enzyme in the liver.  With glycogen storage disease, glycogen is formed, but not released.  Therefore, it continues to be stored in the liver, causing liver enlargement and other problems resulting from the body’s need for glucose.
 

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The three glycogen storage diseases (GSD) most often reported in dogs are: Type I. GSD; Type II GSD, and Type III GSD.

Symptoms:

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Types I and III GSD directly affect the liver.  Clinical signs for both include muscle tremors, lack of coordination, retarded growth, hepatomegaly (abnormal enlargement of the liver), progressive abdominal distention as a result of hepatomegaly, hypoglycemia (low blood sugar), seizures, and weakness and depression due to hypoglycemia.  Type I. GSD is most often seen in toy breed puppies, and symptoms are present between 6 and 12 weeks of age.  Type III GSD is most often seen in German Shepherds and Akitas.  Symptoms of muscular weakness and exercise intolerance has been noted at around 2 months of age.  Type II. symptoms include progressive muscle weakness, frequent vomiting and regurgitation, megaesophagus (abnormal enlargement of the lower portion of the esophagus), dysphonia (hoaresness, difficulty in barking or vocalizing), persistent panting, cardiac abnormalities.  The symptoms begin to appear at about 6 months of age, are progressive, and death usually occurs before two years of age.

Diagnosis:

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Diagnosis can be made by a series of biopsies.

Treatment:

Many aspects of the GSD diseases are still poorly understood, and there are no cures.  Treatment is aimed at alleviating symptoms through use of frequent small meals of high‑carbohydrate food, and administration of glucose therapy.  Although sometimes the condition can be maintained through careful, and very controlled nutrition, unfortunately, the prognosis for many animals with GSD is poor and most succumb to these diseases at a young age.

Links to sites about this disease:

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http://www.ivis.org/special_books/Braund/braund18/chapter_frm.asp?LA=1

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http://www.upei.ca/~cidd/Diseases/nervous%20system%20disorders/lysosomal%20storage%20diseases.htm

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http://www.merckvetmanual.com/mvm/index.jsp?cfile=htm/bc/25628.htm

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http://www.mesavet.com/library/gsd.htm

This summary provided by:

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Jessica S

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Wildfire Kennel

 

 

Dedicated to improving the health of ISSR Shiloh Shepherds.

 


Copyright © 1998 - 2009. Shiloh Shepherd Dog™ Club of America.
All rights reserved. Revised: January 2008

The information on this website was written by ISSR breeders and other concerned individuals, however we are are NOT veterinarians. This information is being provided as a general overview, from information we were able to find about each disease through our own research. These summaries are not intended to be relied upon as medical or veterinary advice, nor do we consider ourselves experts in the veterinary field or in any of these conditions. While we do our best to provide the most up to date information, new research is constantly being done on these diseases. We recommend that you do further study and talk to your veterinarian on any topics you see here, as we cannot guarantee that the information posted here is the most current information available.  This site was originally designed and maintained by Debbie Knatz.