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Polycythemia Vera
- Chronic myeloproliferative neoplasms characterized by clonal proliferation of myeloid cells.
- Presence of elevated RBC mass with low EPO
- Symptoms
- Hypertension
- Palpable Spleen
- Pruritus and erythromelalgia (burning of hands/feet with erythema), pallor or cyanosis
- Thrombosis and Hemorrhage
- 95-97% of patients have a JAK2 mutation
- Treat with phlebotomy, ASA, and hydroxyurea
Posted 01/19/17 09:51:25 AM by Ying Liu
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Budd Chiari
- Thombosis of the hepatic vein
- Can cause fulminant liver failure requiring transplant
- Can also be palliated by TIPS or TIVCS (IVC to portal vein)
- See Caudate Lobe hypertrophy on U/S
Posted 01/19/17 09:52:26 AM by Ying Liu
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- Some patients with ascites have high morbidity with ascites but are under-served by MELD
- MELD-Na helps adjust for this and is better predictor of mortality
- Now adopted for transplant
Posted 01/19/17 09:53:52 AM by Ying Liu
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Hepato-Renal Syndrome (HRS)
- Definition:
- Cirrhosis with ascites
- Cr > 1.5
- No improvement in Cr after discontinuing diuretics and volume expansion
- Albumin 1g/kg/day x 2 days
- No other etiology of renal dysfunction (i.e. shock, nephrotoxic agents, obstruction, intrinsic renal disease)
- Type 1: Cr > 2.5 or 1.5x baseline in <2weeks
- Associated with severe liver failure
- Median survival 2 weeks
- Type 2: Indolent course
Posted 01/19/17 09:56:09 AM by Ying Liu
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HRS Treatment --> AASLD Recommendations:
- Albumin infusion plus administration of vasoactive drugs such as octreotide and midodrine should be considered in the treatment of type I hepatorenal syndrome. (Class IIa, Level B)
- Dose: Midodrine 7.5mg TID, Ocretodie 50mcg/hr (vs SQ 100-200mcg TID), Albumin 1g/kg/day x 2 days, then 25-50g/day
- Albumin infusion plus administration of norepinephrine should also be considered in the treatment of type I hepatorenal syndrome, when the patient is in the intensive care unit. (Class IIa, Level A)
- Transplant is the only cure
Posted 01/19/17 09:56:44 AM by Ying Liu
Created by Christopher Kelly
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