Doctors can monitor hepatomegaly and hypotonia and place patients on a diet high in protein and raw corn starch.
Patients can also go to physiotherapy to learn how to use their muscles properly.
Genetics - genetic counselling, prenatal diagnosis (cultured amniotic fluid cells display a decrease in amylo-1,6-glucosidase activity)
Eventually, some signs and symtoms will disappear after puberty.
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Glycogen Storage Disease Type 3
Patient
Glycogen in the Liver (left stained to show glycogen, right normal)
Glycogen in Muscle Cells
Glucose Breakdown in muscle Cells
Vocabulary
-Glycogen:used for storage form of glucose
-Transferase: It takes the last three glucose residues beyond a branch point and transfers them to another branch
-amylo-1,6-glucosidase: an enzyme in the glycogenolytic path-way which helps convert glycogen to lactate by disrupting the 1,6 linkages between glycosyl units ("debrancher enzyme")
-Hypoglycemia: low blood pressure
-Transferase: It takes the last three glucose residues beyond a branch point and transfers them to another branch
-amylo-1,6-glucosidase: an enzyme in the glycogenolytic path-way which helps convert glycogen to lactate by disrupting the 1,6 linkages between glycosyl units ("debrancher enzyme")
-Hypoglycemia: low blood pressure
Blog Archive
Sources
Music
I made this music player at MyFlashFetish.com.
Symptoms
Symptoms and complications:
Irritability
Tantrums
Tremulousness
Poor feeding
Respiratory distress
Apnea
Bradycardia
Lethargy
Confusion or apathy
Twitching or shakiness
Seizures
Inappropriate behavior (eg, laughter, crying)
Poor concentration
Hypothermia
Headache
Pallor
Palpitations
Hypotonia
Sweating
Coma
Sudden death
Long-term complications of hypoglycemia and/or storage of abnormal glycogen include the following:
Short stature
Cirrhosis
Liver failure
Hepatic adenomas
Hepatocellular carcinomas
Exercise intolerance
Muscle wasting and weakness
Cardiomegaly
Dilated hypertrophic cardiomyopathy with ventricular dysfunction
Renal failure secondary to myoglobinuria (rare)
Irritability
Tantrums
Tremulousness
Poor feeding
Respiratory distress
Apnea
Bradycardia
Lethargy
Confusion or apathy
Twitching or shakiness
Seizures
Inappropriate behavior (eg, laughter, crying)
Poor concentration
Hypothermia
Headache
Pallor
Palpitations
Hypotonia
Sweating
Coma
Sudden death
Long-term complications of hypoglycemia and/or storage of abnormal glycogen include the following:
Short stature
Cirrhosis
Liver failure
Hepatic adenomas
Hepatocellular carcinomas
Exercise intolerance
Muscle wasting and weakness
Cardiomegaly
Dilated hypertrophic cardiomyopathy with ventricular dysfunction
Renal failure secondary to myoglobinuria (rare)