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
What are the Ix of cirrhosis?
Uss abd FBC LFT serum bilirubin Serum albumin, PT/INR RFT Serum alpha feto protein
Aetiology- Hep B and C serology ANA, ASMA serum caeruloplasmin, 24hr urinary cu excretion, serum Fe studies Anti mitochondrial ab
Liver biopsy
Severity of cirrhosis-
Modified child Pugh classification
Model of endstage liver disease (MELD)
Mx of cirrhosis?
General Mx-
Treat causative agent
Adequate diet ( correct deficiencies, veg protein 1g/kg/day, adequate calorie 25-35 kcal/kg/day, vit and mineral supplements, salt restrictions)
Avoid physical exertion,dehydration
Vaccines
Avoid ppt factors of HE ( dehydration, constipation, hypokalemia, hyponatremia, drugs, infection, surgery, UGI bleeding, hypovolemia)
Mx of haematemesis?
Resuscitation
Left lateral
2 wide bore
Blood for FBC DT grouping
IV NS 20ml/kg
FFP or packed red cells if needed
IV omeprazole
IV vassopresin , octreotide, terlipressin
Prophylactic antibiotic (3rd gen cephalosporin)
Urgent endoscopic but doing injection sclerotherapy or banding
Balloon tamponade with a sengstaken-blakemore tube
Drugs to mx HE
prophylactic non selective beta blocker (o propranolol)
Follow up endoscopy
TIPS
Mx of ascites?
IP OP chart and daily weight chart Salt restricted diet (<2g/d) Diuretics Large volume paracentesis (with or without albumin 10g/L) Watch out for HE and pre renal ARF
Mx of spontaneous bacterial peritonitis?
Exclude the DDs
Empirical broad spectrum antibiotics
IV albumin (1.5 g/kg initially, 1g/kg 48hrs later)
Correct adrenal insufficiency
Mx of HE?
Grade Elevate the head end IP OP chart and proper fluid Mx Tx the underlying cause Nutrition- veg protein Maintain electrolyte balance Tx infection Reduce N load from gut (lactulose 30ml 3-4 times a day) Metronidazole 200mg tds /rifaximine Branched amino acid Mannitol in cerebral edema
Precipitating factors of HE
GI- haemorrhage,constipation,high protein diet
Infection-SBP,pneumonia,UTI
Electrolyte imbalance-hypokalemia,hyponatremia,dehydration, uraemia
Drugs- benzodiazepine,sedatives,K loosing diuretics
Surgery-TIPS
superimposed acute liver disease(acute hep), hep ca
