Chiamaka Shared "Clin Med final " - 27 Picmonics

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Clin Med final

Hemochromatosis Pathophysiology
Pathophysiology
Hemosiderin Deposition
Autosomal Recessive
HLA-A3
Secondary to Transfusion Therapy
Increase Iron
Increase Ferritin
Increased Transferrin Saturation
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2 mins
Hemochromatosis Signs and Symptoms
Signs and Symptoms
Micronodular Cirrhosis
Hepatocellular Carcinoma
Diabetes
Bronze Skin Coloring
CHF
Arthropathy
Testicular Atrophy
Impotence
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2 mins
alpha-1-anti-trypsin
PAS-positive
Resist Diastase
Co-dominant
Cirrhosis
Emphysema
Polymerization of AAT
Misfolding
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Wilson's Disease Symptoms
Cirrhosis
Hepatocellular Carcinoma
Basal Ganglia Degeneration
Dementia
Dyskinesia
Asterixis
Kayser-Fleischer Rings
Slit Lamp Exam
Hemolytic Anemia
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2 mins
Wilson's Disease Pathophysiology
Autosomal Recessive
Mutation in ATP7B gene
Chromosome 13
Decrease in Ceruloplasmin
Inadequate copper excretion
Liver
Brain
Kidney
Cornea
Joints
Treat with Penicillamine
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2 mins
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
Crigler-Najjar Syndrome Type 1
Pathophysiology
Autosomal Recessive
Absent UDP-glucuronosyltransferase
Symptoms
Neonatal Jaundice
Kernicterus
Diagnosis
Normal Liver Function Tests
Elevated Unconjugated Bilirubin
Treatment
Phototherapy
Plasmapheresis
Calcium Phosphate and Orlistat
Death often by Age 2
Considerations
No Response to Phenobarbital
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3 mins
Dubin Johnson Syndrome (with Audio)
Pathophysiology
defective hepatocyte secretion in to proper canal
Autosomal recessive- "hereditary conjugated hyperbilirubinemia"
Absent MRP2 (multidrug resistant protein)
Error is inside the hepatocyte.... NOT due to a clogged bile duct
Signs/symptoms
Liver changes from Brown to Black
Jaundiced (otherwise asymptomatic)
labs
Conjugated hyperbilirubinemia
Treatment
NO excess urinary coproporphyrins
no treatment necessary (benign disease)
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6 mins
Dubin Johnson syndrome and Rotor syndrome
Autosomal recessive
Increased Conjugated Bilirubin
Dubin -johnson syndrome
defective liver excretion
deficiency of bilirubin canalicular transport protein
black liver
coarse pigmented granules
develop episodes of jaundice
in the context of a trigger (illness, prego, OCP)
Rotor syndrome
milder in presentation
without black liver
impaired hepatic uptake and excretion
Jaundice
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Rotor's Syndrome
Autosomal Recessive
Normal Liver
High Urine Coproporphyrin
Jaundice
Increased Conjugated Bilirubin
Dysfunction of SLCO1B Genes for Blood Bilirubin Uptake Protein
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Cirrhosis Assessment
Mechanism
Degeneration and Destruction of Liver Cells
Signs and Symptoms
Jaundice
Fatigue
Palmar Erythema
Spider Angiomas
Hepatosplenomegaly
Edema
Gynecomastia
Change in LOC
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2 mins
Cirrhosis Interventions
Interventions
Maintain Nutrition
Paracentesis
Esophageal and Gastric Varices
Drug Therapy
Endoscopic Band Ligation or Sclerotherapy
Balloon Tamponade
Transjugular Intrahepatic Portosystemic Shunt (TIPS)
Hepatic Encephalopathy
Lactulose
Antibiotics
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2 mins
Complications of Cirrhosis
Portal Hypertension
Ascites
Esophageal Varices
Coagulation Defects
Portal-Systemic Encephalopathy
Hepatorenal Syndrome
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1 min
Hepatitis A (HAV) Assessment
Transmission
Fecal-Oral
Ingestion of Contaminated Food or Water
Incubation
4 Weeks
Assessment
Fever
General Malaise
Hepatomegaly
Considerations
Self-Limiting
Proper Hand Washing
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2 mins
Hepatitis B (HBV) Assessment
Transmission
Bodily Fluids
Blood Transmission
IV Drug Abusers
Incubation
Variable 1-6 Months
Assessment
Fever
Nausea and Vomiting
Hepatomegaly
Chronic Disease
Cirrhosis
Hepatocellular Carcinoma
Considerations
Vaccination
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2 mins
Hepatitis B Virus Disease
Can turn chronic
< 10% of adults become chronic carriers
90% of newborns become chronic carriers
Cirrhosis
Hepatocellular carcinoma
Membranous Glomerulonephritis
Polyarteritis nodosa
Serum sickness like syndrome
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2 mins
Hepatitis C (HCV) Assessment
Transmission
Blood Transmission
High Risk Sex (rare)
IV Drug Abusers
Incubation
7 Weeks
Chronic Disease
Cirrhosis
Considerations
Barrier Protection
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2 mins
Budd-Chiari Syndrome
Causes
Pregnancy and Postpartum
Hematologic Disorders
Tumors
Chronic Infections
Mechanism
Thrombosis of Major Hepatic Veins
Centrilobular Congestion and Necrosis
Symptoms
Abdominal Pain
Ascites
Hepatomegaly
Nutmeg Liver
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3 mins
Hepatocellular carcinoma/hepatoma
Most common 1° malignant tumor of liver in adults
Associated with HBV (+/− cirrhosis) and all other causes of cirrhosis (including HCV, alcoholic and nonalcoholic fatty liver disease, autoimmune disease, hemochromatosis, α1-antitrypsin deficiency (A1AT deficiency), and Wilson Disease).
Related to specific carcinogens (eg, aflatoxin from Aspergillus). These toxins induce p53 mutations.
May lead to Budd-Chiari syndrome and Anemia of Chronic Disease.
Findings:
Jaundice, tender hepatomegaly, ascites, polycythemia, anorexia.
Spreads hematogenously.
Diagnosis
Increased α-fetoprotein (AFP); ultrasound or contrast CT/MRI, biopsy.
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Primary Sclerosing Cholangitis Mechanisms
Mechanism
Unknown Mechanism
"Onion Skin" Fibrosis
"Beading" of Bile Ducts
Symptoms
Pruritus
Hepatosplenomegaly
Jaundice
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2 mins

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