Other Hepatic Disorders Flashcards
What are the 3 major patterns of drug induced liver injury?
- Hepatocellular injury
- Cholestatic injury
- Mixed pattern
What is the second most common cause of acute drug induced liver injury?
- antibiotics
* Augmentin, INH, Rifampin
Drug induced liver injury is a result of what? (pathophysiology)`
- direct effects of drug or metabolites = dose dependent
* intrinsic hepatotoxicity
What are the 2 forms of drug induced idiosyncratic hepatotoxicity?
- hypersensitivity (allergy)
- metabolic
What is the enzymatic profile of hepatocellular type DILI?
- ALT/Alk Phos >5
What is the enzymatic profile of cholestatic type DILI?
- ALT/Alk Phos <2
What is the enzymatic profile of mixed type DILI?
- ALT/Alk Phos 2-5
Autoimmune hepatitis is characterized by what?
- circulating antibodies = hypergammaglobulinemia
What 2 antibodies are seen in Type 1 autoimmune hepatitis?
- ANA (Anti-nuclear antibody)
- ASMA (Anti-smooth muscle antibody)
What % of patients with autoimmune hepatitis will present with cirrhosis at time of diagnosis?
25%
What is the first line treatment of autoimmune hepatitis?
- prednisone (+ azathioprine)
Primary biliary cholangitis is characterized by what?
- granulomatous destruction of interlobular bile ducts
What antibodies are seen in primary biliary cholangitis?
- AMA (Anti mitochondrial antibodies)
In what population is primary biliary cholangitis most commonly seen?
- caucasian middle aged women
Xanthomata and Xanthelasma suggests what?
cholestasis (flow of bile from liver is blocked)
What is the treatment of primary biliary cholangitis?
- ursodeoxycholic acid (UDCA)
Primary sclerosing cholangitis is characterized by what?
- obliteration of bile ducts (both extra/intrahepatic)
“Beads on a string” seen on a cholangiogram is classic for what?
Primary sclerosing cholangitis
The majority of patients with Primary sclerosing cholangitis also have THIS additional GI disease.
- Ulcerative colitis (70%)
What antibody is seen in 80% of cases of primary sclerosing cholangitis?
- p-ANCA
What diagnostic procedure for PCS can also treat obstructions causing acute cholangitis?
- ERCP
What type of genetic inheritance is seen in Wilson’s Disease?
- autosomal recessive disorder
Wilson’s disease results in what?
- copper overload
Kayser-Fleischer rings seen on a slit lamp exam is consistent with disease?
- Wilson’s disease
What lab values are low in Wilson’s Disease?
- Ceruloplasmin levels
What is the most effective copper chelating agent used to treat Wilson’s Disease?
- Penicillamine
Hereditary Hemochromatosis results in what?
- iron overload
* increased iron absorption with toxic deposition into multiple organs
What type of genetic inheritance is seen in Hereditary Hemochromatosis?
- Autosomal recessive
What lab findings are consistent with Hemochromatosis?
- Fasting transferrin saturation > 45%
What is the most common chromosome mutation resulting in hemochromatosis?
- C282Y homozygous
What MRI findings are consistent with hemochromatosis?
- decrease signal intensity of liver compared to skeletal muscle
What is the treatment for hemochromatosis?
- Phlebotomy
If a patient presents with suspected hemochromatosis who needs to be screened?
- first degree relatives
* check fasting iron sat and transferrin
What allele pattern causes the disease of a1 antitrypsin deficiency.
- PiZZ
Premature emphysema is a common sign of this disease.
- a1 antitrypsin deficiency
Positive diastase resistant globules seen on acid Schiff staining is consistent with this disease.
- a1 antitrypsin deficiency
