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Rachel Shared "SCIFOM EXAM 2" - 56 Picmonics

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SCIFOM EXAM 2

Hexose Monophosphate Shunt (Pentose Phosphate Pathway)
Oxidative Phase (Irreversible)
Glucose-6-Phosphate Dehydrogenase
Converts Glucose-6-Phosphate to Ribulose-5-Phosphate
Generates NADPH
Reduces Glutathione
Prevents Oxidative Damage
Non-Oxidative Phase (Reversible)
Ribulose-5-Phosphate to Ribose-5-Phosphate
Nucleotide Synthesis
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2 mins
G6PD Deficiency
X-linked Recessive
Hemolytic Anemia
Inflammatory Response
Fava Beans
Sulfonamides
Primaquine
Anti TB Drugs
Heinz Bodies
Bite Cells
Prevalent Among African Americans due to Increased Malarial Resistance
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2 mins
Gluconeogenesis
OVERVIEW
Produces Glucose from Non-Carbohydrate Substrates
Upregulated by Glucagon and Cortisol
PATHWAY
Pyruvate
Pyruvate Carboxylase
Oxaloacetate
Phosphoenolpyruvate (PEP) Carboxykinase
Phosphoenolpyruvate (PEP)
Fructose-1,6-bisphosphate
Fructose-1,6-bisphosphatase
Fructose-6-phosphate
Glucose-6-phosphate
Glucose 6-phosphatase
Glucose
Clinical Relevance
Von Gierke Disease
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4 mins
Cori Disease
Pathophysiology
Type III Glycogen Storage Disease
Deficiency of Alpha 1, 6 Glucosidase
Debranching Enzyme
Limit-Dextrin Accumulation (In Cytosolic Structures)
Signs and Symptoms
Hypoglycemia
Muscle Weakness
Hypotonia
Stunted Growth
Hepatomegaly
Diagnosis
Normal Blood Lactic Acid Levels
Gluconeogenesis Intact
Increased LFT's
Increased Creatine Kinase (CK/CPK)
Treatment
Continuous Feeding
High Protein Diet
Cornstarch (Uncooked)
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4 mins
Ethanol Metabolism
Characteristics
Zero Order Elimination
Pathway
Ethanol
Alcohol Dehydrogenase
Produces NADH
Acetaldehyde
Acetaldehyde Dehydrogenase
Produces NADH
Acetate
Thiokinase
Acetyl-CoA
Drugs
Fomepizole
Disulfiram
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3 mins
Lipid Metabolism and Ketogenesis
Lipid Metabolism
Acetyl-CoA
Biotin Cofactor
Malonyl-CoA
Fatty Acids
Triglycerides
Glycerol
DHAP
Acetoacetyl-CoA
HMG-CoA
HMG-CoA Reductase
Mevalonate
Cholesterol
Ketogenesis
Acetoacetate
Beta-Hydroxybutyrate
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4 mins
Urea Cycle
Mitochondria
N-Acetylglutamate
Carbamoyl Phosphate Synthetase I
NH3 + CO2
Carbamoyl Phosphate
Ornithine Transcarbamylase
Citrulline
Cytosol
Aspartate
Argininosuccinate
Fumarate
Arginine
H2O to Urea
Ornithine
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3 mins
Cholesterol Synthesis
Pathway
Acetyl-CoA
Acetoacetyl-CoA
Cytosolic HMG-CoA Synthase
HMG-CoA
HMG-CoA Reductase
Mevalonate
Cholesterol
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2 mins
Lipogenesis
Fatty Acid Synthesis
Acetyl-CoA
Citrate Shuttle to Cytoplasm
Acetyl-CoA Carboxylase
Biotin Cofactor
Malonyl-CoA
Fatty Acid Synthase
Triglyceride Synthesis
Glycerol
Glycerol-3-Phosphate Acyltransferase
Triglycerides
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2 mins
Lipolysis
Triglyceride Breakdown
Triglycerides
Hormone Sensitive Lipase
Glycerol
Fatty Acids
Fatty Acid Transport
Long-chain Fatty Acids
Carnitine Shuttle
Medium-chain and Short-chain Fatty Acids Diffuse Freely
Fatty Acid Breakdown
Beta Oxidation
Acetyl-CoA if Even Number of Carbon Atoms
Propionyl-CoA if Odd Number of Carbon Atoms
Propionyl-CoA Carboxylase
Biotin Cofactor
Methylmalonyl-CoA
Methylmalonyl-CoA Mutase
Vitamin B12 Cofactor
Succinyl-CoA
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5 mins
Ketogenesis and Ketogenolysis
Ketogenesis
2 Acetyl-CoA
Thiolase
Acetoacetyl-CoA
Mitochondrial HMG-CoA Synthase
HMG-CoA
HMG-CoA Lyase
Ketone Bodies
Acetoacetate
Beta Hydroxybutyrate
Acetone
Ketogenolysis
Succinyl-CoA and Acetoacetate
Acetoacetyl-CoA
Thiolase
2 Acetyl-CoA
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4 mins
Von Gierke Disease
Pathophysiology
Autosomal Recessive
Type I Glycogen Storage Disease
Glucose-6-Phosphatase Deficiency
Signs and Symptoms
Increased Glycogen in Liver
Hepatomegaly
Enlarged Kidneys
Severe Fasting Hypoglycemia
Increased Triglycerides
Increased Uric Acid (Gout)
Increased Lactic Acid
Doll-like Facies
Treatment
Continuous Oral Glucose (or Cornstarch)
Avoid Fructose and Galactose
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2 mins
Pompe Disease
Pathophysiology
Type II Glycogen Storage Disease
Autosomal Recessive
Deficiency of Lysosomal Alpha 1,4 Glucosidase
Acid Maltase
Signs and Symptoms
Hypertrophic Cardiomyopathy
Hepatomegaly
Macroglossia
Hypotonia
Diagnosis
Increased Glycogen within Lysosomes
Increased Lactate Dehydrogenase (LDH)
Increased Creatine Kinase (CK/CPK)
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2 mins
McArdles Disease
Pathophysiology
Type V Glycogen Storage Disease
Muscle Glycogen Phosphorylase Deficiency
Signs and Symptoms
Increased Glycogen in Muscle
Painful Muscle Cramps
Myoglobinuria
Normal Lactic Acid
Treatment
Vitamin B6
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2 mins
Anion Gap Metabolic Acidosis
Pathophysiology
Increased Anion Gap
MUDPILES
Methanol
Uremia
Diabetic Ketoacidosis (DKA)
Propylene Glycol
Isoniazid or Iron
Lactic Acid
Ethylene Glycol
Salicylates
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1 min
Diabetic Ketoacidosis (DKA) Signs and Symptoms
Kussmaul respirations
Dehydration
Abdominal Pain
Nausea/vomiting
Psychosis
Fruity breath Odor
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1 min
Pyruvate Kinase Deficiency
Pathophysiology
Autosomal Recessive
Defect in Pyruvate Kinase
Decreased ATP
Extravascular Hemolysis
Presentation / Symptoms
Hemolytic Anemia in a Newborn
Diagnosis
RBC Enzyme Assay
Echinocytes
Burr Cells
Small Uniform Projections
Normocytic Anemia
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2 mins
Pyruvate Dehydrogenase Deficiency
Characteristics
X-Linked
Acquired from Thiamine Deficiency
Backup of Alanine and Pyruvate
Symptoms and Complications
Neurologic Defects
Lactic Acidosis
Interventions
Ketogenic Nutrients
Lysine and Leucine (Ketogenic Nutrients)
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2 mins
Classic Galactosemia
Pathophysiology
Autosomal Recessive
Galactose-1-phosphate uridyltransferase is Absent (GALT)
Impaired Galactose-1-P to UDP-Galactose
Galactitol Accumulation in Lens
Signs and Symptoms
Infantile Cataracts
Failure to Thrive
Hepatomegaly
Jaundice
Intellectual Disability
Increased Risk E. Coli Sepsis
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2 mins
Essential Fructosuria
Autosomal Recessive
Defect in fructokinase
Fructose to fructose 1-P
Benign
Fructose appears in blood and urine
Fructose does not enter cells
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54 secs

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Rachel Shared SCIFOM EXAM 2 - 56 Picmonics