4 glycogen strage disease

(hepatic)1→glucose-6-phosphatase def
(von gierke)
G6P is diverted to other pathways and subsequent ☆lactic acid production
★↑uric acid
★↑TG
☆steatosis⇔ cori

(muscular)2→α-1,4-glucosidase with α-1,6-glucosidase def (☆lysosomal strage disease)
☆serere cardiomegaly
☆positive PAS
(pompe)

(hepatic)3→debranching enzyme(α-1,6-glucosidase) def
(cori)
(normal lactic acid levels)
☆hypotonia, muscle weakness⇔ von, hers(type Ⅵ)

(muscular)5→glycogen phosphorylase def
(mcardle)

pediatrics biochemistry UWorld step2CK