• 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

• 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

• MELD vs. MELD-Na
  • 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

• 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
    • Median survival 6 months
  
  

  Posted 01/19/17 09:56:09 AM by Ying Liu

• 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

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