Liver Transplant Long Flashcards
Causes of Chronic Liver Disease?
“WATCH ID”
o Wilson’s
o Alpha-1 antitrypsin
o Tyrosinaemia
o CF
o Hereditary Fructose Intolerance
o Infection - TORCH, echovirus, adenovirus, parvovirus B19, E. coli UTI, EBV, hepatitis A, B, C
o Drugs: Isoniazid, methyldopa, anti-metabolites
Young child (<5yo)
o Infection - TORCH, echovirus, adenovirus, parvovirus B19, E. coli UTI
o Structural – EHBA, choledochal cyst, Alagille, biliary hypoplasia
o Metabolic - CF, AAT deficiency, HFI; Galactosaemia, tyrosinaemia, Niemann-Pick, Glycogen storage diseases - type IA & IV (Von Gierke & Anderson)
o Endocrine – hypothyroidism, hypopituitarism
o TPN
o Idiopathic neonatal hepatitis (25%)
Older child (>5yo)
o Infectious - EBV, CMV, toxoplasmosis, hepatitis A, B, C
o Metabolic – Wilson’s disease, HFI, CF, A1AT deficiency
o Autoimmune chronic active hepatitis –> A/w thyroiditis, glomerulonephritis, erythema nodosum
o PSC from IBD (>10yo) –> A/w arthritis, erythema nodosum, uveitis
Causes of CLD in younger child <5?
o Infection - TORCH, echovirus, adenovirus, parvovirus B19, E. coli UTI
o Structural – EHBA, choledochal cyst, Alagille, biliary hypoplasia
o Metabolic: CF, AAT deficiency, HFI, Galactosaemia, tyrosinaemia, Niemann-Pick, Glycogen storage diseases - type IA & IV (Von Gierke & Anderson)
o Endocrine – hypothyroidism, hypopituitarism
o TPN
o Idiopathic neonatal hepatitis (25%)
Causes of CLD in older child >5?
o Infectious - EBV, CMV, toxoplasmosis, hepatitis A, B, C
o Metabolic – Wilson’s disease, HFI, CF, A1AT deficiency
o Autoimmune chronic active hepatitis –> A/w thyroiditis, glomerulonephritis, erythema nodosum
o PSC from IBD (>10yo) –> A/w arthritis, erythema nodosum, uveitis
Complications of CLD?
o PHT –> haematemesis, PR bleeding
o Ascites and bacterial infections
o Growth, nutrition, puberty
o Micro and macronutrient deficiencies
o Blood - ↑ PT, ↓ platelets (hypersplenism)
Signs of CLD?
1) General – jaundice, encephalopathy [liver failure]
2) Hands – leukonychia, clubbing, palmar erythema, xanthomata (b/n fingers & on extensor surfaces), asterixis [liver failure], Muehrcke’s lines (transverse white lines on finger nails - hypoalbuminemia), Blue lunulae – Wilson’s disease.
3) Arms – spider naevi, bruising, scratch marks, xanthomata, asterixis (separate fingers & extend wrist 15 seconds)
4) Face – eyelid xanthomata, scleral icterus/pallor, corneal xerosis/opacification/clouding, fetor hepaticus (sweet smell, secondary to inability of diseased liver to demethylate methionine)- [liver failure]
5) Chest – gynaecomastia, spider naevi, pectoral muscle wasting, loss of body hair
6) Abdomen – dilated veins, hepatosplenomegaly, ascites, testicular atrophy, epigastric murmur
7) Legs – oedema, muscle wasting, bruising
Signs of CLD complications?
“HEPATIC”
o Hepatorenal/hepatopulmonary/hypersplenism –> splenomegaly correlates poorly w degree of HTN,
o Encephalopathy/oesophageal varices
o Portal HTN/ Poor synthetic function
a) Portal HTN: Dilated collateral veins - haematemesis (oesophageal varices), abdominal wall paraumbilical veins (caput medusa) & haemorrhoids, Cruveilhier-Baumgarten murmur (auscultate epigastrium with Valsalva to increase intra-abdominal pressure, secondary to falciform ligament varices)
b) Synthetic function: Jaundice, ↑ PT, ↓ albumin (ascites), Proximal myopathy, ↑ toxic metabolites (hepatic encephalopathy)
o Ascites/Asterixis
o Thrombosis – portal vein
o Infection (SBP)
o Coagulopathy/carcinoma (inc risk HCC)
+ Malnutrition: Macronutrient – fat & protein, Micronutrient – ADEK deficiency
Signs of CLD treatment complications?
1) Liver transplantation:
o Infection – early (up to 1 month) usually bacterial/fungal; later (2-6 months) usually viral e.g. EBV, CMV, HVZV
o Biliary tract obstruction, hepatic vascular compromise
o Rejection, recurrence of disease, EBV-associated lymphoproliferative malignancy (3-12 months post tx)
2) Surgery scars - Kasai procedure, splenectomy, TIPS (transjugular intrahepatic portosystemic shunt), intravascular access
3) Immunosuppression – steroids, tacrolimus, cyclosporine
4) Antihypertensives - captopril cough
Investigations of CLD in <5y child?
o Infectious – TORCH, urine MCS
o EHBA – acholic stools, liver US, HIDA, liver biopsy, operative cholangiogram (“gold standard”), wedge liver biopsy (if inconclusive)
o Alagille – ECHO for PS, CXR for butterfly vertebrae
o Endocrine – TSH, glucose (hypopituitarism)
o Metabolic
AAT - Alpha-1-antitrypsin assay & Pi phenotype
CF - sweat test, IRT
HFI – fructose-1-phosphae aldolase assay on liver or jejunal biopsy
Zellweger - urinary metabolic screen & organic acids
Tyrosinemia – urinary metabolic screen & amino acids
Galactosemia - red blood cell
Ix for CLD in older child >5y?
1) Infectious
- Hep A, B, C, CMV, EBV, toxoplasmosis
- Hep B – ?also do ANA and anti-smooth muscle antibodies
2) Choledochal cyst – liver ultrasound
3) Autoimmune hepatitis – TSH (thyroiditis), urine microscopy (glomerulonephritis)
4) Metabolic
- Wilson - Serum & urine copper, liver biopsy for copper levels
- PSC from IBD - Visualised on PTC/ERCP (percutaneous transhepatic cholangiography or endoscopic retrograde cholangiography)
- HFI, CF & AAT – as above
Ix for monitoring of CLD?
o Liver - LFT, synthetic function (alb & coags)
o Nutrition - Fat-soluble vitamins (retinol level, vitamin E level, calcium, phosphate, SAP)
o Portal HT – Abdominal US with Doppler studies + portal pressures, endoscopy for varices
o Ascites – Ascitic tap (if SBP)
o Encephalopathy – clinical dx; can do ammonia, glucose, electrolytes
Management of nutrition in CLD?
- High energy, increased fat (MCTs) and high protein diet (low if encephalopathic)
- +/- NG/PEG if anorexic
- Need ADEK, Zn, Fe and Ca supplements and monitoring
- Involve Dietetics
Management of Portal HTN and varices?
1) Medical: Beta blockers (dec bleeding, Increase dose until 80% of baseline heart rate), IV vasopressin
2) Endoscopic techniques: Sclerotherapy, Banding, Balloon tamponade (Sengstaken-Blakemore tube; Cx – suffocation, pulmonary aspiration, oesophageal rupture)
3) Shunts: Splenorenal or portocaval shunts (Potentially limits future liver transplant, May precipitate encephalopathy), Transjugular intrahepatic portosystemic shunt (TIPS) (temporising measure, Only used in kids who are awaiting liver transplantation)
4) Splenectomy –> sequestration/pooling results in decreased platelets and lymphocytes (Avoid if possible: Risk of infection + increased bleeding)
Mx of Pruritis?
o Ursodeoxycholic acid (uncertain benefit)
o Cholestyramine (bile acid resin i.e. binds to bile acid in the gut; adherence difficult; SE – unpalatable, constipation)
o Liver transplantation
o Naltrexone (opioid antagonist)
o Rifampicin (competes with bile acid for hepatic uptake)
Mx of Encephalopathy?
1) Correct precipitant: Protein load ↓, Infection (including SBP), Large GIT bleed, Hypovolaemia, Electrolyte imbalance – hypokalaemia, metabolic alkalosis, hypoglycaemia, Meds – sedatives, thiazides, loop diuretics
2) Oral lactulose: broken down by colonic bacteria into short chain fatty acids, which lowers colonic pH –> favours formation of nonabsorbable NH4+ from NH3, trapping NH3 in the colon + effectively reduces plasma ammonia concentrate
3) Consider oral neomycin (uncertain benefit; Cx – ototoxic, nephrotoxic) or Oral flumazenil (if on benzodiazepines)
Mx of Ascites?
- Restrict sodium/salt
- +/- spironolactone
- +/- ascetic tap
