All cases should be discussed with the liver team at Birmingham Children's Hospital or King's College Hospital at an early stage
Evaluation
- Full history including information on:
- IV injections, needles and needlestick injury
- Infusions of blood products
- Contact with jaundice
- Family history of liver disease
- Sexual contacts
- Parents drug habits and medications
- Patients' medications or other suspect poisons (e.g. mushrooms)
Examination
Pay particular attention to and record:
Degree of encephalopathy:
- Grade I - mild drowsiness, impaired cognition, concentration or psychomotor function
- Grade II - more drowsy, reusable and capable of conversation, but some confusion or disorientation
- Grade III - marked drowsiness, responding to simple commands may be aggressive, agitated or incoherent
- Grade IV - unrouseable
- IVa - responds to pain
- IVb - no response to pain
State of hydration
Evidence of spontaneous bleeding
Evidence of chronic liver disease
Mark upper and lower margins of liver onto abdomen with waterproof marker.
Investigations
Blood
- Full blood count, retics and film
- PT and APPT
- Group and save
- Blood gases
- Glucose, FFA, 3-OH butyrate, lactate
- Ammonia
- Liver Function Tests (AST, ALT, ALP, Alb. SBR, GGT)
- Creatinine, urea and electrolytes
- Calcium, Phosphate and Magnesium
- Paracetamol level (if relevant)
- Plasma for quantitative amino acids
- Alpha-fetoprotein
- Iron, Total iron binding capacity and ferritin
- Immunoglobulins, Anti SMA, ANA, LKM antibodies
- Blood cultures
- Serology for Hepatitis A, B, C, EBV, HIV, CMV, HSV, Leptospira
- save serum*
- Total and free acyl carnitine
Urine
- Culture and Sensitivity
- Osmolarity
- Metabolic screen
- Drug metabolites to Toxicology if indicated
If out of hours please save the above samples
If patient >3 years old
- serum caeruloplasmin and copper
- 24 hour urine collection for copper pre and post penicillamine
Other
- Chest x-ray for left ventricular failure or aspiration
- Abdominal ultrasound for liver size and hepatic vein patency
- Consider cranial CT for evidence of encephalitis, cerebral oedema, raised ICP or intracranial bleed
- EEG for baseline
Management
All patients should have minimal handling and be treated as potentially infectious (gloves and aprons). Nurse head up 10-20 degrees
Monitor
- Blood pressure, heart rate, ECG
- CVP (4-8 mmHG)
- Core-toe temperature
- Urine output by catheter
- Cutaneous oximetry
- Degree of encephalopathy
- Blood glucose 4 hourly
- Gastric pH by nasogastric aspiration 6 hourly
Fluid balance
1. Restrict to reduce intracranial pressure
2. Maintain circulating volume with colloid
Fluids are restricted to 50-75% maintenance (depending on CVP and ICP)
Guidelines:
| Infants - 2 years | 70 ml/kg/day |
| 2 - 5 years | 60 ml/kg/day |
| 5 - 10 years | 50 ml/kg/day |
| >10 years | 40 ml/kg/day |
Electrolytes:
Sodium replacement 50% of maintenance or none depending on electrolytes and hydration.
Potassium 2-4 mmol/kg/d depending on electrolytes
Maintain urine output (0.5-2 mls/kg/hour)
Avoid using FFP in early stages, as coagulation is good guide to prognosis.
FFP may be required for procedures or for active bleeding.
Baseline drugs
Vitamin K
| <1 year | 2.5 mgs/day IV |
| >1 year | 5 mg/day IV IV Vitamin K. |
| >10 years | 10 mgs/day IV |
Give Vit K for 3 consecutive days
Ranitidine - 1-3 mgs/kg/dose TDS
Lactulose - 2-4 rnls/kg/dose TDS
Low protein diet if indicated
N-acetylcysteine
Antibiotics and Antifungal therapy
General PICU management
- Ventilate to normocapnia (PaCO2 4.6 - 5.3 kPa)
- Avoid inducing an alkalosis (shifts ammonia into brain)
- Reverse hypotension / establish target MAP or CPP
- Analgesia / Sedation
- morphine or alfentanyl
- midazolam
- Paralysis if required
- atracurium
- Monitor glucose carefully and treat hypoglycaemia
- Monitor arterial blood pressure / gases
- Monitor CVP
- Treat possible factors resulting in worsening encephalopathy / support liver
- Lactulose
- Consider sepsis
- N-acetylcysteine
- Consider seizures, do EEG if suspicious
- Prior to monitoring ICP correct coagulation profile and platelet count
- This will usually require large fluid volumes and some form of filtration to remove additional fluid:
- Insert vascath
- Filtration: options include plasma filtration or CVVH
- infuse FFP +/- cryoprecipitate
- infuse platelets to keep platelet count >100
- attempt to normalise coagulation / platelets
Treatment principles of raised ICP (in order of importance)
- ensure normocapnia, normoxia
- ensure adequate sedation
- reverse pyrexia
- mannitol +/- hypertonic saline
- mild / moderate hypothermia
- titrated hyperventilation
- thiopentone
