Richard Shared "BCH E4" - 21 Picmonics

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BCH E4

Acute Intermittent Porphyria (AIP)
Pathophysiology
Autosomal Dominant
Inhibited Porphobilinogen Deaminase
Increased Serum Porphobilinogen
Increased Serum Delta-ALA
Increased Urinary Porphyrin Precursors
Signs & Symptoms
Precipitated By P450 Inducers
Painful Abdomen
Polyneuropathy
Psychological Disturbances
Port Wine-Colored Urine
Treatment
Glucose and Hemin
Considerations
Avoid Triggers
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3 mins
Porphyria Cutanea Tarda (PCT)
Pathophysiology
Inhibited Uroporphyrinogen Decarboxylase (UROD)
Most Common Porphyria
20% Autosomal Dominant
80% Sporadic Type
Hepatitis C Association
Signs & Symptoms
Photosensitivity
Blistering of Skin
Tea Colored Urine
Diagnosis
Increased Uroporphyrins (Urinary & Serum)
Treatment
Avoid Sunlight
Phlebotomy
Hydroxychloroquine (Low Dose)
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3 mins
Winter's Formula
Evaluates Respiratory Compensation
Used in Metabolic Acidosis
Equation
(HCO3 x 1.5 ) + 8 plus-minus 2 = PCO2
Steps
Bicarbonate (HCO3) x 1.5
+ 8
± 2
= Expected Value for PCO2
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1 min
Normal Gap Metabolic Acidosis
HARD-ASS
Hyperalimentation
Addison's Disease
Renal Tubular Acidosis
Diarrhea
Acetazolamide
Spironolactone
Saline Infusion
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2 mins
Respiratory Acidosis
Hypoventilation
Increased PaCO2 > 45
Barbiturates Depress Central Respiratory Center of Brain
Opioids Depress Central Respiratory Center of the Brain
Airway Obstruction
Respiratory Muscle Weakness/Paralysis
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3 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
Respiratory Alkalosis
Hyperventilation
High Altitude
Aspirin
Restrictive Lung Disease
Pulmonary Embolism
Pregnancy
Progesterone
Rib Fracture
Anxiety
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1 min
Metabolic Alkalosis
Diuretic use
Vomiting
Antacid
Hyperaldosteronism
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26 secs
Chloride (Cl-) Lab Value
95-105 mEq/L
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27 secs
Gilbert's Syndrome
Pathophysiology
Decreased UDP-Glucuronosyltransferase Activity
Decreased Bilirubin Conjugation
Signs and Symptoms
Often Asymptomatic
Jaundice (Mild)
Fasting
Stress
Alcohol Intake
Labs
Increased Unconjugated Bilirubin
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2 mins
Microcytic Anemia Causes
MCV < 80
Causes
Thalassemias
Chronic Disease or Illness
Lead Toxicity
Late Iron Deficiency
Sideroblastic Anemia
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1 min
Normocytic Hemolytic Anemia Causes
Intrinsic Hemolytic
Hereditary Spherocytosis
RBC Enzyme Deficiency
Hemoglobin C Defect
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Sickle Cell Anemia
Extrinsic Hemolytic
Autoimmune
Microangiopathic
Mechanical Destruction
Prosthetic Cardiac Valves
Infection
Snake Venom
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2 mins
Macrocytic Anemia Causes
MCV > 100
Megaloblastic
B12 Deficiency
Folate Deficiency
Orotic Aciduria
Non Megaloblastic
Alcoholism
Liver Disease
Reticulocytosis
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2 mins
Anemia Lab Values
Iron Deficiency Anemia
Serum Iron Decreased
Transferrin Increased
Ferritin Decreased
% Transferrin Saturation Decreased
Pregnancy and OCP Use
Serum Iron Normal
Transferrin Increased
Ferritin Normal
% Transferrin Saturation Decreased
Anemia of Chronic Disease
Serum Iron Decreased
Transferrin Decreased
Ferritin Increased
% Transferrin Saturation Decreased or Normal
Hemochromatosis
Serum Iron Increased
Transferrin Decreased
Ferritin Increased
% Transferrin Saturation Increased
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3 mins
Iron Deficiency Anemia
Causes
Malnutrition/Malabsorption
Hemorrhage
Signs
Microcytic, Hypochromic Anemia
Decreased Heme Synthesis
Labs
Decreased Reticulocytes
Decreased Ferritin
Increased Red Cell Distribution Width (RDW)
Poikilocytosis
Anisocytosis
Increased Central Pallor
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2 mins
Hereditary Spherocytosis Disease
Northern Europeans
Mechanisms
Autosomal Dominant
Spectrin/Ankyrin Deficiency
Spherocyte Formation
Hemolytic Anemia
Symptoms
Bilirubin Gallstones
Jaundice
Splenomegaly
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3 mins
Hereditary Spherocytosis Diagnosis & Treatment
Labs
Increased MCHC
Spherocytes
Normocytic Anemia
Diagnosis
Osmotic Fragility Test
Treatment
Splenectomy
Folic Acid
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1 min
Sickle Cell Anemia (Mechanism)
Intrinsic Normocytic Hemolytic Anemia
Point Mutation
Autosomal Recessive
African-American
Dehydration or Decreased O2
Newborns Asymptomatic
Heterozygote Malarial Resistance
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2 mins
Beta Thalassemia
Mechanism
Microcytic, Hypochromic Anemia
Mediterranean Populations
Decreased Beta-Globin
Beta-Thalassemia Minor
Increased HbA2
No Intervention
Beta-thalassemia Major
Blood Transfusions
Hemochromatosis
Crew-cut on Skull X-Ray
Diagnosis
Electrophoresis
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2 mins
Lesch-Nyhan Syndrome
X-linked Recessive
Absence of HGPRT
Hypoxanthine to IMP
Guanine to GMP
Excess uric acid
Gout
Retardation
Self-mutilation
Choreoathetosis
PRPP is increased
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2 mins

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