glycogen storage diseases

myopathic forms

deficiency of enzymes in the musclesmuscular weakness

myopathic

mcardle's is myopathic or hepatic?

hepatic forms

inherited deficiency of hepatic enzymereduction in blood glucose

hepatic

Von Gierke's: myopathic or hepatic?

pompe disease

lysosomal glycogen disease

Von Gierke's Disease

type 1 glycogen storage disease

pompe disease

Type II glycogen storage disease

type I

Von gierkes disease

type II

pompe disease

type III

cori disease

Cori disease

Type III glycogen storage disease

type IV

Andersen disease

Andersen Disease (Type IV)

type IV glycogen storage disease

type V

mcardle disease

McArdle's disease

Type V glycogen storage disease

type VI

Hers disease

Hers disease

type VI glycogen storage disease

type VII

PFK disease - tauri disease

PFK disease Tauri

Type VII glycogen storage disorder

type VIII

phosphorylase kinase - disease

PK disease

Type VIII glycogen storage disorder

Von Gierke disease (type I)

Glucose-6-phosphatase deficiencyliver and kidneyHYPOglycemia

Glucose-6-phosphatase deficiencyliver and kidneyHYPOglycemia

Von Gierke disease (type I)

Pompe disease (type II)

Lysosomal a-1,4-glucosidase deficiency. Sxs: cardiomyopathy heart failure

Lysosomal a-1,4-glucosidase deficiency. Sxs: cardiomyopathy heart failure

Pompe disease (type II)

Cori disease (type III)

Debranching enzyme (a-1,6,-glucosidase) deficiency. muscle and liver Sxs: milder form of Type 1 (fasting hypoglycemia, elevated liver glycogen, etc)

Debranching enzyme (a-1,6,-glucosidase) deficiency. muscle and liver Sxs: milder form of Type 1 (fasting hypoglycemia, elevated liver glycogen, etc)

Cori disease (type III)

Andersen Disease (Type IV)

branching enzyme deficiencyliver and spleen long outer branches - cirrhosis of liverGBE1 gene

branching enzyme deficiencyliver and spleen long outer branches - cirrhosis of liverGBE1 gene

Andersen Disease (Type IV)

McArdle disease (type V)

Skeletal muscle glycogen phosphorylase deficiency. MUSCLE ONLYcramps with strenuous exercise

Skeletal muscle glycogen phosphorylase deficiency. MUSCLE ONLYcramps with strenuous exercise

McArdle disease (type V)

Hers Disease (Type VI)

deficient Hepatic glycogen phosphorylase

deficient Hepatic glycogen phosphorylase

hers disease type VI

PFK type VII - tauri disease

MUSCLE PFK deficiencymuscle weakness and cramps

MUSCLE PFK deficiencymuscle weakness and cramps

PFK type VII - tauri disease

PK type VIII - hemolytic anemia

LIVER PK deficiencymild hypoglycemina

LIVER PK deficiencymild hypoglycemina

PK type VIII - hemolytic anemia

type I von gierke

liver and kidney

Cori disease (type III)

muscle and liver

Andersen Disease (Type IV)

liver and spleen

McArdle's diseasePFK

muscle only

hers diseasePK

liver only

HYPOglycemia

what is a symptom associated with liver glycogen storage disease?

nocturnal gastric infusion of glucoseeating uncooked cornstarch - slower release and complex carbsAVOID fructose, galactose, sucrose

what is a treatment for von gierke's disease?

phosphorylase

degrades glycogen from the nonreducing ends of the glycogen molecule

autosomal recessive

glycogen storage disorders mode of inheritance

McArdle's disease

mutation in PYGM gene