Other Liver Disease Flashcards

1
Q

Budd Chiari

A
  • obstruction of hepatic venous outflow
  • sudden (acute), refractory ascites, hepatomegaly
  • imaging: US, MRA/MRV
  • biopsy: congestion around zone 3, dilatation of sinusoids around zone 3
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2
Q

Hemangioma/Hemangioendothelioma

A
  • Think about how outside/inside can reflect each other.
    Hemangioma is most common, can be benign and found in infancy
  • Associated skin lesions
  • High output heart failure
  • Associations: KM syndrome, Hypothyroidism
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3
Q

Hepatoblastoma

A

Hepatoblastoma is more common that Hepatocellular carcinoma in childhood.
-HB is a/w extreme premie (especially 1500g).
- Hep B virus
- Beckwith-Wiedemann syndrome
- FAP
- Glycogen storage diseases (mainly type 1)
- Hereditary tyrosinemia
- Cholestatic conditions
- BA
-PFIC 2
- Alagille syndrome.

Tx:
-chemo: cisplatin, 5-FU, Vincristine.
- surgery: resection/OLT
- post-oper chemo: +Doxorubicin, irinotecan, ifosfamide if relapse (increased risk for renal and hearing impairment post-transplant).

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