Tyler Shared "HODI - Week 5 - metabolism" - 26 Picmonics
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HODI - Week 5 - metabolism
Cystinuria
- Pathophysiology
- Defect of Renal Tubular Amino Acid Transporter
- C-O-L-A Acronym
- Cystine
- Ornithine
- Lysine
- Arginine
- Signs and Symptoms
- Excess Cystine in Urine
- Hexagonal Crystals
- Staghorn Kidney Stones
- Diagnosis
- Cyanide Nitroprusside Test
- Treatment
- Acetazolamide to Alkalinize the Urine
3 mins
Maple Syrup Urine Disease
- Pathophysiology
- Defect in alpha ketoacid dehydrogenase
- Blocked degradation of branched chain amino acids
- Leucine
- Isoleucine
- Valine
- Signs and Symptoms
- Seizures
- Intellectual Disability
1 min
Hemoglobin A1c Lab Value
- 4-6%
1 min
Diabetic Ketoacidosis (DKA) Signs and Symptoms
- Kussmaul respirations
- Dehydration
- Abdominal Pain
- Nausea/vomiting
- Psychosis
- Fruity breath Odor
1 min
Diabetic Ketoacidosis (DKA) Diagnosis and Labs
- Diagnosis
- Blood glucose Increased > 250 mg/dL
- Anion gap metabolic acidosis
- Decreased pH < 7.3
- Decreased serum bicarbonate < 18
- Plasma ketones
- Other Labs
- Hyperkalemia
1 min
Diabetic Ketoacidosis (DKA) Treatment
- Initial Management
- IV Normal Saline
- Regular Insulin Drip
- Subsequent Management (correction of electrolytes)
- Potassium (K+)
- Glucose
- Treat Underlying Cause
- Monitoring
- Venous pH
- Serum Bicarbonate (HCO3)
1 min
Phenylketonuria (PKU)
- Pathophysiology
- Decreased Phenylalanine Hydroxylase
- Decreased Tetrahydrobiopterin Cofactor
- Autosomal Recessive
- Tyrosine Becomes Essential
- Phenylalanine Found in Nutrasweet
- Symptoms
- Musty or Mousy Body Odor
- Growth Retardation
- Seizures
- Intellectual Disability
- Hypopigmentation
2 mins
Metformin
- Indications
- Diabetes Mellitus Type 2
- Polycystic Ovary Syndrome (PCOS)
- Mechanism of Action
- Decreased Gluconeogenesis
- Increased Insulin Sensitivity
- Side Effects
- Lactic Acidosis
- GI Distress
- Weight Loss
- B12 Deficiency
- Contraindication
- GFR < 30 ml/min
2 mins
Insulin
- Rapid Acting
- Insulin Lispro (Humalog)
- Insulin Aspart (Novolog)
- Insulin Glulisine (Apidra)
- Short Acting
- Regular Insulin (Humulin R)
- Intermediate Acting
- Isophane NPH (Humulin N)
- Long Acting
- Detemir (Levemir)
- Glargine (Lantus)
2 mins
Metachromatic Leukodystrophy
- Pathophysiology
- Autosomal Recessive
- Arylsulfatase A Deficiency
- Increased Cerebroside Sulfate
- Signs and Symptoms
- Demyelination
- Ataxia
- Dementia
1 min
Krabbe Disease
- Pathophysiology
- Autosomal Recessive
- Galactocerebrosidase Deficiency
- Increased Psychosine
- Signs and Symptoms
- Developmental Delay
- Peripheral Neuropathy
- Optic Atrophy
- Diagnosis
- Globoid Cells
1 min
Fabry's Disease
- Pathophysiology
- X-linked Recessive
- Alpha-galactosidase A Deficiency
- Increased Ceramide trihexoside
- Early Signs and Symptoms
- Decreased Sweating (Hypohidrosis)
- Angiokeratoma
- Episodic Peripheral Neuropathy
- Late Signs and Symptoms
- Cardiovascular Disease
- Renal Failure
1 min
Hunter Syndrome
- Pathophysiology
- X-linked Recessive
- Mucopolysaccharidoses
- Iduronate Sulfatase Deficiency
- Increased Dermatan Sulfate
- Increased Heparan Sulfate
- Signs and Symptoms
- No Corneal Clouding
- Aggressive Behavior
1 min
Hurler Syndrome
- Pathophysiology
- Autosomal Recessive
- Mucopolysaccharidoses
- Alpha-L-Iduronidase Deficiency
- Increased Heparan Sulfate
- Increased Dermatan Sulfate
- Signs and Symptoms
- Hepatosplenomegaly
- Developmental Delay
- Gargoylism
- Corneal Clouding
- Airway Obstruction
2 mins
Ornithine Transcarbamylase Deficiency
- Pathophysiology
- Most Common Urea Cycle Disorder
- X-Linked Recessive
- Signs and Symptoms
- Hyperammonemia
- Decreased BUN
- Carbamoyl Phosphate is Converted to Orotic Acid
- Pyrimidine Synthesis Pathway
- Evident in Babies
3 mins
Marasmus
- Deficiency of all Nutrients
- Body Weight < 60% of Normal
- Tissue and Muscle Wasting
- Loss of Subcutaneous Fat (Buttocks and Thighs)
- Variable Edema
2 mins
Orotic Aciduria
- Pathophysiology
- Autosomal Recessive
- Defect in UMP Synthase
- De Novo Pyrimidine Synthesis Pathway
- Symptoms
- Increased Orotic Acid in Urine
- Megaloblastic Anemia
- No Response to B12 or Folate
- Non-Hyperammonemic
- Failure to Thrive
- Treatment
- Oral Uridine Monophosphate
2 mins
Kwashiorkor
- Protein Deficiency
- Swollen Belly
- MEALS
- Malnutrition
- Edema
- Anemia
- Liver Malfunction
- Skin Lesions
2 mins
Chlorpropamide and Tolbutamide (Orinase) (1st Generation Sulfonylureas)
- Mechanisms
- 1st Generation Sulfonylureas
- Stimulate Release of Insulin
- Indications
- Type 2 Diabetes
- Side Effects
- Hypoglycemia
- Possible Cardiovascular Toxicity
- Weight Gain
- Considerations
- Exacerbated Hypoglycemia with Beta-blockers
- Avoid During Pregnancy and Breast Feeding
- Avoid Alcohol
2 mins
Glyburide and Glipizide (2nd Generation Sulfonylureas)
- Mechanisms
- 2nd Generation Sulfonylureas
- Blocks Potassium Channels in Beta Cells
- Stimulate Release of Insulin
- Indications
- Type 2 Diabetes
- Side Effects
- Hypoglycemia
- Considerations
- More Potent
- Beta Blockers Reduce Effects
- Avoid Alcohol
- Avoid Breastfeeding
2 mins
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