Ch 20 - Liver disorders Flashcards
Prolonged neonatal jaundice: time frame (1) definition & features (3)
> 2 weeks (is usually a self resolving unconjugated bilirubinaemia) Liver disease - conjugated Br >20 umol/l
F - pale stools, dark urine, bleeding tendency, FTT
Biliary atresia: definition (1) Features (5) Ix (3) Management (2)
D - destruction/absence of extrahepatic biliary tree + intrahepatic bile ducts (leads to chronic liver failure & death)
F - PROLONGED CONJUGATED bilirubinaemia, FTT, mild jaundice, pale stools + dark urine, heptamegaly, splenomegaly (2ndary to portal HTN)
Ix - USS; may show absent/contracted GB, radioisotope scan - good uptake in liver > but no bowel excretion. Cholangiography - CONFIRMS diagnosis - fails to outline normal biliary tree
M - Hepatoportoenterostomy (KASAI procedure) - anastomse jejenum to liver - allows bile drainage. If fails liver transplant
Choledochal cysts: definition, features (4), Ix (2), maangement (2), complications (2)
D - cystic dilations of extrahepatic biliary system
F - cholestasis, abdo pain, palpable mass, cholangitis, jaundice
Ix - USS, radionuclide scan
M - surgical excision + Roux-en-Y formation (straight from upper biliary duct to distal jejenum) C - cholangitis/ malignancy
Neonatal heptatis syndrome: causes ( 5), contrast to biliary atresia - in terms of presentation(2)
Causes: congenital infection CF Galactosaemia inborn errors of metabolism alpha 1 anti trypsin def
Contrast - these babies present at birth with IUGR + hepatosplenomegaly
Alpha-1-antitrypsin def: Features (4), Ix (1), management (1)
F - prolonged neonatal jaundice, hepatosplenomegaly, bleeding (vit K def), pulmonary disease later in life
Ix - plasma A1AT levels
M - Transplant
Galactosaemia: features (4), Ix (2), Management (1)
Very rare disorder
F - poor feeding, vomiting, jaundice, hepatomegaly, liver failure & cataract if left untreated
Ix - urinary galactose, RBC Gal-1-PUT
M - galactose free diet prevents progression but ovarian failure and LDs may occur later anyway
Alagille syndrome - intrahepatic biliary hypoplasia: features (5)
intrahepatic biliary hypoplasia intense pruritus FTT, traingular facies eye defects congenital heart disease (pulmonary stenosis)
Hep A: definition, features, Ix, management
D - RNA virus spread via faecal-oral transmission F - acute liver disease which self resolves Ix - HAV IgM M - no treatment
Hep B: transmission: transmission (3) features (3), Ix (antibodies/antigens)
DNA virus - blood products, renal dialysis, perinatal from mothers F - asymp; chronic liver disease development > cirrhosis/ HCC Ix - HBsAg (ongoing infectivity), HBeAg (high infectivity), HBcAg, HBcAb (acute/past infection)
Hep B: management for acute, chronic (3), prevention (2)
Acute - none
Chronic - interferon, lamivudine, adefovir
Prevention - screen all pregnant women for HBsAg, all positive should have their babies vaccinated for Hep B, also give infants Hep B Ig if mother is HBeAg +ve
Hep C: defintion, features (2), management (2)
RNA virus spread by blood products and perinatally
F - chronic infection > Cirrhosis/ HCC
M - peg IFN alpha 2b + Ribavarin after 4 yrs of age
Hep D: definition, features (1)
D - defective RNA virus dependent on HBV for replication > CO-INFECTION only F - acute exacerbation of chronic HBV, high risk of cirrhosis
Acute liver failure defintion
massive hepatic necrosis > loss of liver function +/- hepatic encephalopathy. Rare but high mortality
Acute liver failure causes (4)
In children most commonly paracetamol OD Non A-G hep viruses metabolic abnormalities - Wilson’s, tyrosinaemia autoimmune hep Reye syndrome
Acute liver failure: features (5)
Jaundice encephalopathy coagulopathy hypoglycaemia irritability/confusion/drowsiness electrolyte disturbances
Acute liver failure: Ix (4)
ALT/ AST - very elvated (10-100 times)
ALP elevated
Very abnormal coagulation
Hyperammonaemia ABG EEG for encephalopathy, CT may show cerebral oedema
Management of Acute liver failure (4)
Maintain blood glucose > 4 mmol/L with dextrose
Prevent sepsis - broad spectrum ABs and antifungals
Prevent haemorrhage - vit K, FFP T
reat cerebral oedema - fluid restriction, mannitol diuresis Urgent transfer to specialist unit
Reye syndrome/ reye like syndrome: defintion, causes
D - acute non-inflammatory encephalitis + microvesicular fatty liver infiltration C - unknown, but associated with Aspirin (since stopping aspirin for
Causes of chronic liver disease (5)
Hep B/ C Wilson’s Drugs - nitrofurantoin/ NSAIDs IBD A1AT
Autoimmune hepatitis: Features (3)
Mainly 7-10yr F
Can present as acute/chronic liver disease or fulminant hepatic failure
Autoimmune features - rash, SLE, IBD, haemolytic anaemia
Autoimmune hepatitis: Ix (abs - 3), management (2)
Ix - Anti ANA, Anti SM, anti LKM (liver kidney microsomal), elevated IgG (will see a hypergammaglobulinaemia) M - prednisolone + azathioprine
CF: features (3), Ix (1), management (3)
Liver disease 2nd most common killer in CF pts after resp disease F - Hepatic steatosis, Thick tenacious bile + abnormal bile acid conc > biliary fibrosis > cirrhosis Ix - histology - nodular cirrhosis, fatty liver, biliary fibrosis
M - Ursodeoxycholic acid, nutritional support, transplant
Wilson’s disease: features (4), Ix (3), Management (3)
F - >3 yrs of age, poor school performance, mood change, tremor, vit D resistant rickets, haemolytic anaemia, Kaysier-Fleischer rings in cornea
Ix - reduced caeruloplasmin, reduced serum Cu, increased urinary Cu
M - Penicillamine (increased Cu urinary excretion), + Zn (reduces Cu absorption), liver transplant, pyridoxine - prevents peripheral neuropathy
Non alcoholic fatty liver disease (NAFLD): features (2), Ix (3), management (1)
F - obese children, usually asymp, may have some RUQ pain
Ix - incidental echogenic liver on USS, slightly elevated transaminases, biopsy - steatosis +/- inflammation/fibrosis
M - weight loss
Cirrhosis: definition, features (5)
D - marked fibrosis + regenerating nodules
F - palmar erythema, malnutrition, spider naevi, splenomegaly, dilated abdo veins. If decompensated - abnormal LFTs, prolonged PT, hypoalbuminaemia
Oesophageal varices: consequence of what? diagnosis (1), management (3)
Consequence of portal HTN Diagnosis - upper GI endoscopy M - treat acute bleeding with transfusion + H2 blockers, if persistent - terlipressin, sclerotherapy
Ascites: management (3)
contibutory factors - low albumin, renal impairment M - Fluid + sodium restriction, diuretics, albumin infusion
Spontaneous bacterial peritonitis: when to consider (3), Diagnosis (1), treatment (1)
If unexplained fever, abdo pain, deterioration in Hepatic function D - diagnositic paracentesis - send fluid for culture M - broad spectrum ABs
Management of children with liver disease: nutrition (2), vitamins (4) , pruritis (3)
Nutrition - high carb high protein diet - increase cal by 50% Vitamins - fat soluble vits - ADEK
Pruritis - loose clothing, emmolients, ursodeoxycholic acid
Indications for liver transplant (3)
Poor QOL severe malnutrition - despite intense nutritional therapy recurrent complications FTT
Complications of liver transplant (3)
Rejection
Sepsis
primary non-function
