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
Wilson's Disease Symptoms
- Cirrhosis
- Hepatocellular Carcinoma
- Basal Ganglia Degeneration
- Dementia
- Dyskinesia
- Asterixis
- Kayser-Fleischer Rings
- Slit Lamp Exam
- Hemolytic Anemia
Hemochromatosis Pathophysiology
- Hemosiderin Deposition
- Autosomal Recessive
- HLA-A3
- Secondary to Transfusion Therapy
- Increase Iron
- Increase Ferritin
- Increased Transferrin Saturation
Hemochromatosis Signs and Symptoms
- Micronodular Cirrhosis
- Hepatocellular Carcinoma
- Diabetes
- Bronze Skin Coloring
- CHF
- Arthropathy
- Testicular Atrophy
- Impotence
Primary Sclerosing Cholangitis Mechanisms
- Unknown Mechanism
- "Onion Skin" Fibrosis
- "Beading" of Bile Ducts
- Pruritus
- Hepatosplenomegaly
- Jaundice
Primary Sclerosing Cholangitis Labs and Treatment
- Increased Conjugated Bilirubin
- Increased ALK-P
- Increased IgM
- Ulcerative Colitis
- Cholangiocarcinoma
- Secondary Biliary Cirrhosis
- Liver Transplant
- Stent
Primary Biliary Cholangitis (Primary Biliary Cirrhosis)
- Autoimmune Destruction of Bile Ducts
- Intrahepatic bile ducts
- Cholestasis
- Occurs in Middle-Aged Females
- Fatigue
- Pruritus
- Skin Hyperpigmentation
- Hepatomegaly
Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Diagnosis and Treatment
- Positive Antimitochondrial Antibodies
- Increased Alkaline Phosphatase (ALK-P)
- Increased Cholesterol
- Ursodiol
- Liver Transplant