Ch 20 - Liver disorders

Question 1

Prolonged neonatal jaundice: time frame (1) definition & features (3)

Answer 1

> 2 weeks (is usually a self resolving unconjugated bilirubinaemia) Liver disease - conjugated Br >20 umol/l

F - pale stools, dark urine, bleeding tendency, FTT

Question 2

Biliary atresia: definition (1) Features (5) Ix (3) Management (2)

Answer 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

Question 3

Choledochal cysts: definition, features (4), Ix (2), maangement (2), complications (2)

Answer 3

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

Question 4

Neonatal heptatis syndrome: causes ( 5), contrast to biliary atresia - in terms of presentation(2)

Answer 4

Causes: congenital infection CF Galactosaemia inborn errors of metabolism alpha 1 anti trypsin def

Contrast - these babies present at birth with IUGR + hepatosplenomegaly

Question 5

Alpha-1-antitrypsin def: Features (4), Ix (1), management (1)

Answer 5

F - prolonged neonatal jaundice, hepatosplenomegaly, bleeding (vit K def), pulmonary disease later in life

Ix - plasma A1AT levels

M - Transplant

Question 6

Galactosaemia: features (4), Ix (2), Management (1)

Answer 6

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

Question 7

Alagille syndrome - intrahepatic biliary hypoplasia: features (5)

Answer 7

intrahepatic biliary hypoplasia intense pruritus FTT, traingular facies eye defects congenital heart disease (pulmonary stenosis)

Question 8

Hep A: definition, features, Ix, management

Answer 8

D - RNA virus spread via faecal-oral transmission F - acute liver disease which self resolves Ix - HAV IgM M - no treatment

Question 9

Hep B: transmission: transmission (3) features (3), Ix (antibodies/antigens)

Answer 9

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)

Question 10

Hep B: management for acute, chronic (3), prevention (2)

Answer 10

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

Question 11

Hep C: defintion, features (2), management (2)

Answer 11

RNA virus spread by blood products and perinatally

F - chronic infection > Cirrhosis/ HCC

M - peg IFN alpha 2b + Ribavarin after 4 yrs of age

Question 12

Hep D: definition, features (1)

Answer 12

D - defective RNA virus dependent on HBV for replication > CO-INFECTION only F - acute exacerbation of chronic HBV, high risk of cirrhosis

Question 13

Acute liver failure defintion

Answer 13

massive hepatic necrosis > loss of liver function +/- hepatic encephalopathy. Rare but high mortality

Question 14

Acute liver failure causes (4)

Answer 14

In children most commonly paracetamol OD Non A-G hep viruses metabolic abnormalities - Wilson’s, tyrosinaemia autoimmune hep Reye syndrome

Question 15

Acute liver failure: features (5)

Answer 15

Jaundice encephalopathy coagulopathy hypoglycaemia irritability/confusion/drowsiness electrolyte disturbances

Question 16

Acute liver failure: Ix (4)

Answer 16

ALT/ AST - very elvated (10-100 times)

ALP elevated

Very abnormal coagulation

Hyperammonaemia ABG EEG for encephalopathy, CT may show cerebral oedema

Question 17

Management of Acute liver failure (4)

Answer 17

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

Question 18

Reye syndrome/ reye like syndrome: defintion, causes

Answer 18

D - acute non-inflammatory encephalitis + microvesicular fatty liver infiltration C - unknown, but associated with Aspirin (since stopping aspirin for

Question 19

Causes of chronic liver disease (5)

Answer 19

Hep B/ C Wilson’s Drugs - nitrofurantoin/ NSAIDs IBD A1AT

Question 20

Autoimmune hepatitis: Features (3)

Answer 20

Mainly 7-10yr F

Can present as acute/chronic liver disease or fulminant hepatic failure

Autoimmune features - rash, SLE, IBD, haemolytic anaemia

Question 21

Autoimmune hepatitis: Ix (abs - 3), management (2)

Answer 21

Ix - Anti ANA, Anti SM, anti LKM (liver kidney microsomal), elevated IgG (will see a hypergammaglobulinaemia) M - prednisolone + azathioprine

Question 22

CF: features (3), Ix (1), management (3)

Answer 22

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

Question 23

Wilson’s disease: features (4), Ix (3), Management (3)

Answer 23

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

Question 24

Non alcoholic fatty liver disease (NAFLD): features (2), Ix (3), management (1)

Answer 24

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

Question 25

Cirrhosis: definition, features (5)

Answer 25

D - marked fibrosis + regenerating nodules

F - palmar erythema, malnutrition, spider naevi, splenomegaly, dilated abdo veins. If decompensated - abnormal LFTs, prolonged PT, hypoalbuminaemia

Question 26

Oesophageal varices: consequence of what? diagnosis (1), management (3)

Answer 26

Consequence of portal HTN Diagnosis - upper GI endoscopy M - treat acute bleeding with transfusion + H2 blockers, if persistent - terlipressin, sclerotherapy

Question 27

Ascites: management (3)

Answer 27

contibutory factors - low albumin, renal impairment M - Fluid + sodium restriction, diuretics, albumin infusion

Question 28

Spontaneous bacterial peritonitis: when to consider (3), Diagnosis (1), treatment (1)

Answer 28

If unexplained fever, abdo pain, deterioration in Hepatic function D - diagnositic paracentesis - send fluid for culture M - broad spectrum ABs

Question 29

Management of children with liver disease: nutrition (2), vitamins (4) , pruritis (3)

Answer 29

Nutrition - high carb high protein diet - increase cal by 50% Vitamins - fat soluble vits - ADEK

Pruritis - loose clothing, emmolients, ursodeoxycholic acid

Question 30

Indications for liver transplant (3)

Answer 30

Poor QOL severe malnutrition - despite intense nutritional therapy recurrent complications FTT

Question 31

Complications of liver transplant (3)

Answer 31

Rejection

Sepsis

primary non-function