Glycogen storage disease type III


What is the outcome for children with glycogen storage disease type 3?

The outcome for children with glycogen storage disease III (GSD III) varies and usually depends on how a child is managed. Children with GSD III who follow a strict diet and have close care when ill usually have good growth and development. When treated well over time, GSD III does not affect your child’s intellectual or developmental abilities. However, children and babies who do not receive prompt treatment and control of the diet almost always have developmental disabilities and may have a shorter lifespan from the health problems.

People with GSD IIIa may also have heart problems that can start in childhood or adolescence. It will be important for a child with GSD IIIa to also be followed by a heart doctor also called a cardiologist.

The liver problems in GSD III typically get better over time. The most common liver problem in GSD III is an enlarged liver (hepatomegaly). However, in a small amount of people with GSD III the liver problems may worsen and may result in a liver transplant.

It is important that children with GSD III are followed closely throughout life by a genetic or metabolic doctor. Genetics and metabolic doctors in your area can be found by talking with your child's pediatrician or family doctor or by using the Find A Genetic Service tool at the American College of Medical Genetics.

"Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome."Sentner CP, Hoogeveen IJ, Weinstein DA, Santer R, Murphy E, McKiernan PJ5, Steuerwald U, Beauchamp NJ, Taybert J, Laforêt P, Petit FM, Hubert A, Labrune P, Smit GP, Derks TG. J Inherit Metab Dis. 2016 Apr 22.

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