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
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
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
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)
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
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
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
3 mins
Cholesterol Synthesis
- Pathway
- Acetyl-CoA
- Acetoacetyl-CoA
- Cytosolic HMG-CoA Synthase
- HMG-CoA
- HMG-CoA Reductase
- Mevalonate
- Cholesterol
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
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
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
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
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)
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
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
1 min
Diabetic Ketoacidosis (DKA) Signs and Symptoms
- Kussmaul respirations
- Dehydration
- Abdominal Pain
- Nausea/vomiting
- Psychosis
- Fruity breath Odor
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
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)
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
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
54 secs
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