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8 Cards in this Set
- Front
- Back
Von Gierkes
type 1 |
(LIVER)
deficiency: glucose 6 phosphatase (liver, kidney, intestine) symptoms: hepatomegaly, acidosis, ketosis, HYPOGLYCEMIA, hypolipidemia |
|
Pompes
type 2 |
(MUSCLE)
deficiency: lysosomal glucosidase (all organs) symptoms: cardiomegaly, hepatomegaly, lysosome can't break glycogen; DEATH BY ONE YEAR OLD |
|
Coris or Forbes
type 3 |
(LIVER)
deficiency:glycogen debranching enzyme (liver, heart muscle) symptoms: hepatomegaly, partial degradation of glycogen, normal blood glucose and lipids |
|
Andersen
type 4 |
(LIVER)
deficiency: glycogen branching enzyme (generalized) symptoms: hepatomegaly, cirrhosis, liver failure |
|
McArdle
type 5 |
(MUSCLE)
deficiency: muscle glycogen phosphorylase symptoms: muscle weakness and cramping, normal lactate |
|
Hers
type 6 |
(LIVER)
deficiency:liver glycogen phosphorylase symptoms: hepatomegaly, less severe hypoglycemia than type 1, lipidemia or acidosis |
|
Taruis
type 7 |
(MUSCLE)
deficiency: muscle PFK1 (muscle and erythrocytes symptoms: muscle weakness and cramping during exercise |
|
type 0
|
deficiency: glycogen synthase
symptoms: no glycogenesis, hypoglycemia LARGE FLUCTUATIONS (hyperglycemic after a huge meal, hypoglycemia while fasting) |