Liver Diseases Flashcards
What you know about autoimmune hepatitis?
In young females, associated with other autoimmune diseases.
Progress to fibrosis and cirrhosis
Ix- ^ALT AST
hypergamaglobulinaemia
Mild normochromic normocytic anemia with thrombocytopenia and leucopenia
3 types - type 1 (ANA, anti smooth muscle ab)
Type 2 - (anti liver kidney microsomal ab, anti liver cytosole ab) affect children only.
Type 3- soluble liver kidney ag ab. Affects adults in middle age. Now a part of type 1
Mx- perdnisolone,azathioprine, liver transplant
NASH mechanism
Osmosis
NASH Mx and Ix?
Ix- USS , CT, MRI , Elastography, biopsy
Mx- weight loss, strict control of HTN DM and lipid, regular followup, vit E, pioglitazone, liver transplant in end stage
Etiology of hemachromatosis
- Primary - HFE gene mutation on chromosome 6. Enterocytes absorb iron even when there’s no need.
- Frequent blood transfusions
Clinical features of hemachromatosis
- triad- hepatomegaly, bronze skin, DM ( also malabsorption due to destruction of exocrine glands)
- Cardiomyopathy,arrhythmia
- hypogonadism and other pituitary hormones (amenorrhea,testicular atrophy)
- calcium pyrophosphate deposition of large and small joints, chondrocalcinosis
Ix and Mx of hemachromatosis
Ix- High serum iron High transferrin saturation Low transferrin production Low TIBC High ferritin LFT often normal Biopsy (diagnostic)
Mx- venesection
Chelation
Desferioxamine
Ix and Mx for alcoholic liver disease
Ix- High MCV AST>ALT High gamma GT elastography Low albumin ^PT
Mx- Stop drinking Tx complications Prednisolone (severity measure by maddrey score) Transportation in end stage
What is the pathophysiology of Wilson’s disease?
Osmosis
Clinical features of Wilson’s disease?
Children- often present with hepatic prob
Young adults- more neurological problems, tremors, dysarthria, involuntary movements, dementia,chorea
Acute/chronic hepatitis,cirrhosis
KF rings
Ix and Mx of Wilson’s disease?
Ix-
Reduced serum copper and caeruloplamin
Increased urinary copper
Tx-
Life time penicillamine/trientine
SE- skin rash, leucopenia, skin changes, renal damage.
Pathophysiology of cirrhosis?
Osmosis
Aetiological criteria of cirrhosis?
Alcohol Metabolic Infective Immune Biliary Genetic Venoocclusive disease
Complications of cirrhosis?
Portal HTN asites and SBP Congestive spleenomegaly Portosystemic shunt Hepatic encephalopathy Hepatorenal failure hepatopulmonary syndrome Decrease liver function Increase estrogen in blood Jaundice Hypoalbuminemia Decreased coagulation
Features of decompensated liver disease?
Hepatic encephalopathy Ascites Bleeding Portal HTN new onset jaundice
Prognostic indicators of cirrhosis?
Low albumin
Low serum sodium
Prolonged PT >_6s
Increased creatinine