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MMD Test 2

Quiz yourself by thinking what should be in each of the black spaces below before clicking on it to display the answer.
        Help!  

Question
Answer
Von Gierke's disease symptoms   Severe hypoglycemia, increased glycogen stores, lactic acidemia, large liver and kidney, dwarfism  
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Von Gierke's deficiency   glucose-6-phosphatase  
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Von Gierke's organ involved   Liver  
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Von Gierke   Type I  
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Pompe   Type II  
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Pompe's deficiency   Lysosomal alpha 1-4 glucosidase  
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Pompe's organ involved   All organs  
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Pompe's symptoms   Cardiomegaly, hepatomegaly  
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Forbes/Cori   Type III  
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Cori's deficiency   debranching enzyme  
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Cori's organ involved   Liver, muscle, heart  
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Cori symptoms   Hepatomegaly, normal ECG, lipids and glucose, normal kidney, muscle weakness, hepatic fibrosis but not cirrhosis; mild gypoglycemia  
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Andersen   Type IV  
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Andersen deficiency   Branching enxyme  
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Andersen organ involbed   Generalized  
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Andersen symptoms   Hepatosplenomegaly, ascites, cirrhosis, liver failure, death within 2-4 years  
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McArdle   Type V  
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McArdle deficiency   muscle glycogen phosphorylase  
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McArdle organ involved   skeletal muscle  
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McArdle symptoms   weakness, cramps on exercise with no blood lactate rise, can tolerate moderate exercise, myoglobin excreted in urea; normal liver phosphorylase  
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Hers   Type VI  
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Hers deficiency   liver glycogen phosphorylase  
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Hers organ affected   liver  
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Hers symptoms   hepatomegaly, normal spleen and lipids, no acidosis; mild form of glycogen storage; hypoglycemia  
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Early onset Pompe symptoms   cardiac/resp infection/failure; floppiness, inability to gain weight; death within 1 yr  
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Late onset Pompe symptoms   skeletal and joint muscles, difficulty breathing  
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Infantile Pompe   hypotonia, cardiomegaly (abnormal ECG), respiratory difficulties  
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Early childhood Pompe   progress much more slowly, skeletal muscle weakness, death due to resp failure  
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Adult form of Pompe   no organomegaly, weak muscles, disease slowly progresses  
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Cori's disease similar to   Type I  
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Cori's disease similar to   Von Gierke's  
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Cori's disease   shows presence of creatine kinase (usually in muscles)  
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Andersen's disease   shows increase in transaminase activity in blood (shows liver damage)  
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Childhood/Adolescence McArdle's   increase in fatigability  
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20-40 yrs McArdle's   severe muscle cramps and pain  
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>40 years McArdle's   muscle wasting and fatigue  
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Her's Disease similar to   mild Type I  
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Her's Disease similar to   Von Gierke's  
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Lactase   breaks down lactose into galactose and glucose  
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