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Hepatology

Genetic Liver Disease   

Questions discussed in this category

What would you your approach to evaluation and monitoring of a patient with elevated AMA and increased immunoglobulins with a low alkaline phosphatase?
What kind of monitoring for and risk of PBC would this carry?
1 Answer available
In a PSC patient who has received liver transplant, what graft and/or patient factors predispose to a more rapid return of their disease and how do you manage these peri-operatively and post-transplant?
1 Answer available
Would you recommend phlebotomy for a patient with previously treated ALL and HBV reactivation both now in remission but with elevated liver enzymes and ferritin, and liver biopsy with widespread peri-canalicular moderate iron deposition and perisinusoidal fibrosis with focal periportal fibrosis?
1 Answer available
If a patient has a low ceruloplasmin with normal 24 hours urine copper excretion, how would you go about an approach to evaluation of other disorders of copper metabolism as a cause of liver disease?
1 Answer available
How would you approach the management of a patient with elevated iron saturation (60%) and ferritin (500s) with negative genetic testing for hemochromatosis?
What kind of further monitoring do you perform?
1 Answer available
How would you approach a referral for concern for hemochromatosis with ferritin elevation but otherwise normal iron studies?
1 Answer available
What is your preferred approach to a patient with incidentally found low ceruloplasmin?
1 Answer available
In what clinical scenario would you consider liver transplant evaluation for a patient with sickle cell hepatopathy?
And should this be considered in conjunction with or sequentially with a bone marrow transplant as the actual cure for underlying hematologic disease?
1 Answer available
How do you differentiate primary from secondary iron overload?
Any unique management features for each?
1 Answer available
How would you differentiate neurologic manifestations of a patient with Wilson's disease versus manifestations of hepatic encephalopathy due to end-stage liver disease?
1 Answer available
What variables do you weigh most heavily when choosing which copper chelation therapy you'll recommend for a patient?
1 Answer available
In Fontan-associated liver disease (FALD), what clinical scenario would lead you to pursue an evaluation for simultaneous liver-heart transplant as opposed to heart alone if there is evidence of fibrosis on a liver biopsy?
1 Answer available


Papers discussed in this category

Hepatology (Baltimore, Md.), 2022 Dec 07
A multidisciplinary approach to the diagnosis and management of Wilson disease: 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases.
Journal of hepatology, 2011-08
A diagnostic approach to hyperferritinemia with a non-elevated transferrin saturation.

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