Glycogen Storage Dz Flashcards
Glycogen Storage Dz
Type I
Name: ___ ______
Enzyme dysfunctional= ____________
Organs Affected: ___ & _____
How much AND how does the Glycogen appear?
Clinical Conditions [7]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type I = Viagra = Von Gierke’s
Enzyme dysfunctional: Glucose 6-Phosphatase
Organs Affected: KIDNEY & LIVER
*Glycogen amount is INCREASED but Normal Structure
Clinical Conditions:
“Von G with [S H U G were MF (L)ame] “
ºS= SEVERE hypOglycemia ºH= Hepatomegaly ºU= hyperUricEmia (High metabolism of Alanine) ºG= Gouty Arthritis
ºM= Mental Retardation ºF= Failure to Thrive
º(L)ame= hyperLipidemia
Glycogen Storage Dz
Type 2
Name: ______
Enzyme dysfunctional= ____________
Organs Affected: _________
How much AND how does the Glycogen appear?
Clinical Conditions [3]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 2 = Pills = Pompe’s
Enzyme dysfunctional: 1,4-Glucosidase
Organs Affected: ALLLL ORGGANS DUDE!
*Glycogen amount is MASSIVELY INCREASED but normal structure
Clinical Conditions
ºhypOtonia Cardiomegaly
ºCardiorespiratory failure
ºDeath before 2
Glycogen Storage Dz
Type 3
Name: ______
Enzyme dysfunctional= ____________
Organs Affected: _____ and ____
How much AND how does the Glycogen appear?
Clinical Conditions [1]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 3 = Cause = Cori’s
Enzyme dysfunctional: [Amylo-1-6-DeBranching Enzyme]
Organs Affected: MUSCLE and LIVER
*Glycogen amount is INCREASED and SHORT OUTER Structure
Clinical Conditions
ºSimilar to [Type I Von G] but milder
Glycogen Storage Dz
Type 4
Name: ______
Enzyme dysfunctional= ____________
Organs Affected: _____ and ______
How much AND how does the Glycogen appear?
Clinical Conditions [2]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 4 = A = Anderson’s
Enzyme dysfunctional: Branching Enzyme
Organs Affected: SPLEEN and LIVER
*Glycogen amount is Normal but LONG BRANCH Structure
Clinical Conditions
ºProgressive Liver Cirrhosis
ºLiver failure—>Death before 2
Glycogen Storage Dz
Type 5
Name: ______ ______
Enzyme dysfunctional= ____________
Organs Affected: _________
How much AND how does the Glycogen appear?
Clinical Conditions [2]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 5 = Major = McArdle’s
Enzyme dysfunctional: [Glycogen Phosphorylase]
Organs Affected: MUSCLE
*Glycogen amount is MODERATE but normal structure
Clinical Conditions
ºUnable to perform strenuous exercise
ºpainful muscle cramps
Glycogen Storage Dz
Type 6
Name: ______ _____
Enzyme dysfunctional= ____________
Organs Affected: _________
How MUCH Glycogen appears?
Clinical Conditions [1]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 6 = Hardon = Hers’
Enzyme dysfunctional: [Glycogen Phosphorylase]
Organs Affected: LIVER
*Glycogen amount is INCREASED
Clinical Conditions
ºSimilar to [Type I Von G] but milder
Glycogen Storage Dz
Type 7
Name: ______
Enzyme dysfunctional= ____________
Organs Affected: _________
How MUCH Glycogen appears?
Clinical Conditions [1]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 7 = 7 = Type 7
Enzyme dysfunctional: PFK1
Organs Affected: MUSCLE
*Glycogen amount is INCREASED
Clinical Conditions
ºSimilar to Type 5
Glycogen Storage Dz
Type 8
Name: ______ ______
Enzyme dysfunctional= ____________
Organs Affected: _________
How MUCH Glycogen appears?
Clinical Conditions [1]
“Viagra Pills Cause A Major Hardon (x) 78 “
Type 8 = 8 = Type 8
Enzyme dysfunctional: [Phosphorylase KINASE]
Organs Affected: LIVER
*Glycogen amount is INCREASED
Clinical Conditions
ºMild hepatomegaly
ºMild hypOglycemia
