TESTED QUESTIONS AND ACCURATE
ANSWERS GRADED A+
●● Cholangitis.
Answer: Localized or diffuse inflammatory changes affecting the
intrahepatic and extrahepatic bile ducts.
●● Common causes for Cholangitis.
Answer: Condition caused by:
CBD (common bile duct) stones
tumors
strictures
complications from endoscopic retrograde cholangiopancreatography
●● Primary Sclerosing Cholangitis (PSC).
Answer: a chronic cholestatic liver disease of unknown etiology. This
condition is characterized by fibrosing inflammation of both the
intraheptatic and extrahepatic biliary tree resulting in irreversible
damage to the bile ducts and ultimately leads to cholestatis, cirrhosis and
liver failure. It is frequently associated with IBD - particularly UC and
Crohn's disease
, ●● Alpha 1-Antitrypsin Deficiency.
Answer: genetic disorder caused by a variety of mutations to the A1AT
gene, leading to decreased A1AT activity in the blood and lungs and
deposition of excessive abnormal A1AT proteins in the liver.
Deficiencies in normal A1AT production result in failure of this
protective mechanism, with the development of significant pulmonary
disease, primarily emphysema.
●● A1AT.
Answer: gene produced in the liver. function is to protect lungs from
enzymes that can damage connective tissue and destroy alveoli
●● Gaucher's Disease.
Answer: lipid storage disease as a result of mutations to the gene that
regulates lipid storage
●● Type 1 Gaucher's Disease.
Answer: this type of Gaucher's Disease is non-neuronopathic and onset
can occur early in age. Clinical features include hepatosplenomegaly,
thrombocytopenia and pathologic bone fractures. These individuals may
have a normal life expectancy.
●● Type 2 Gaucher's Disease.
Answer: this type of Gaucher's Disease is infantile or acute
neuronopathic commonly results in death within the first year. These