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
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
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).