Cirrhosis, PHTN, Varices and management of LD Flashcards
What is cirrhosis?
- Pathological extensive fibrosis with regenerative nodules and is the end-result of many forms of liver disease.
- May be secondary to hepatocellular disease or to chronic bile duct obstruction (biliary cirrhosis).
Pathophysiology of cirrhosis?
- Diminished hepatic function and portal hypertension with splenomegaly, varies and ascites.
- Hepatocellular carcinoma may develop.
Presentation of cirrhosis?
1) Compensated children may be asymptomatic if liver function is adequate - no jaundice and normal LFTs are possible.
2) Distended abdomen due to ascites and hepatosplenomegaly
3) Dilated abdominal veins and splenomegaly due to PHTN
4) Palmar and plantar erythema and spider naevi
5) Malnutrition
Aetiology, Dx, and Tx of oesophageal varies?
Develop as a result of PHTN and may develop rapidly in children.
Dx: Upper GI endoscopy
Tx: Acute bleeding treated with blood transfusion and H2-blockers/PPI: Ranitidine/Omepraole
Persistant bleeding treated with vasopressin: IV Terlipressin (vasoconstriction stems the bleeding)
Management of liver disease?
Nutrition: - High protein, high carbohydrate diet with 50% more calories than recommended.
- Many children will require NG or parenteral nutrition
Pruritus: Many children with cholestasis will have severe pruritus. Help treat with: loose clothing, emollients, Phenobarbital to stimulate bile flow.
Fat-soluble vitamins (ADEK): - There is often malabsorption of fat-soluble vitamins due to cholestasis - bile is unstable to emulsify fats.
- Replace vitamins A, D (prevents rickets and fractures), E (prevents peripheral neuropathy and ataxia), K (important clotting factor).
Liver transplant indications for chronic liver failure?
1) Severe malnutrition unresponsive to intensive nutritional therapy
2) Recurrent complications - resistant ascites and bleeding varices
3) Failure of growth and development
4) Poor QOL