Guidelines for the initial management of paediatric liver disease
Investigation and treatment of liver disease with acute onset –
Local hospital protocol
Defined as EITHER sudden onset of jaundice with evidence of liver
aetiology OR incidental discovery of raised transaminases in association with symptoms suggesting acute onset
Age of onset >3 months
Investigate and treat as neonatal jaundice before 3 months Acute Liver Failure (ALF): INR>2.0 due to liver dysfunction of
less than 8 weeks duration without encephalopathy or >1.5 with encephalopathy requires transfer to Transplant Centre.
Ensure given vitamin K parenterally at least once and regularly by mouth
Ensure not hypoglycaemic with BM stix before feeds >4.0 for at least 24
hours – give IV dextrose to ensure blood glucose >4.0 mmol/l.
Ask and record colour of urine and stool.
Take history and perform examination especially for features of chronicity
of liver disease.
Bloods – HAV IgM, FBC, INR, DIC screen, renal, bone liver profiles, total
and conjugated bilirubin levels, HBV SAg.
If INR>2 TREAT AS LIVER FAILURE – If INR raised liver failure may be
Contact SpR at Transplant Centre. Do not wait for results to make contact.
Preliminary investigations – blood and urine cultures, urine drug screen,
plasma paracetomol level if any indication, Immunoglobulins, C3, C4, auto-antibodies, pANCA, copper, zinc, caerulopasmin, hepatitis C antibody, alpha-fetoprotein, liver ultrasound.
Liaise with SpR at Transplant Centre to arrange transfer according to
urgency depending on above information.
Investigations in Acute Liver Failure
Core investigations – prior to all investigation protocols
FBC, retics, film
If prolonged treat with vit K 1mg IV and repeat in 6-8 hr.
Blood Group to be known
-1-antitrypsin phenotype (from
PIZZ – deficiency
parents if transfused) Renal, lipid, bone, and liver profiles,
Profile of liver dysfunction
split bilirubin, creatine kinase Urine C&S
Ethics committee consent obtained for all patients – inform parents as courtesy
Evidence and nature of liver disease
Glucose/BM stick 4 hourly
SAVE INITIAL URINE
Drugs especially paracetomol
Hepatitis A IgM, B IgM anticore, anti Hep C
Hepatitis viruses A, B, C (URGENT to Institute
antibodies, Hep C RNA
of Liver Studies)
Fibrinogen (coagulation form)
Urea cycle disorder
Leptospirosis CMV, EBV, HSV, V-Z adenovirus, Parvovirus (PCR may be necessary) HHV6.
CXR, ECG (ECHO if indicated)
Low serum levels noted at presentation
Blood gases lactate, pyruvate,
PYRUVATE discuss with biochemist first
Management of ALF while waiting for transfer
Nurse child with head elevated at 20o and no neck flexion (to decrease
I.C.P. and minimize cerebral irritability)
DO NOT SEDATE unless already ventilated - this may precipitate
Maintain oxygenation with facial oxygen, unless this increases the
Maintain blood sugar between 4 - 9 mmol/l using minimal fluid volume (40
- 60 ml/kg/day crystalloid) with high dextrose concentrations e.g. 10% - 20% add K as necessary.
Strict monitoring of urinary output and fluid balance. Catheterise if
necessary. Check urinary electrolytes, urea creatinine and osmolarity.
Frequent neuro-obs (hourly to 4 hourly as clinically indicated)
Give one dose of Vit K (1-5 mg IV) to attempt correction of prolonged
clotting time. If frank bleeding (GIT or other) occurs consider prudent use of FFP at 10 ml/kg IV OR cryoprecipitate at 5 ml/kg IV only for bleeding or insertion of monitoring devices. (FFP will mask prognostic value of INR). Factor VIIa may be used for persistent bleeding.
CMV negative irradiated blood required for all children <1 year and agreed
Prophylactic Ranitidine 3 mg/kg/dose tds (max dose 50mg) IV
antacid to prevent gastric/duodenal ulceration.
IV Cefuroxime 20 mg/kg three times daily (Max dose 1.5 gram three times
daily) or Tazocin 90mg/kg/dose tds (mximun single dsoe 4.5g) x 7 days.
Antifungal- Fluconazole - 6mg/kg/day once daily (max 200mg/day once
daily) x7-14 days
Infants <2 should receive acyclovir IV 20mg/Kg 8 hrly for 14 days or until
herpes infection is excluded.
Patients with renal dysfunction on high WBC also require liposomal
Lactulose to produce 2 - 4 soft stools per day if patient conscious - omit if
Stop oral protein initially. Gradually reintroduce 0.5 g-1g/kg/24 hours.
Strict fluid balance is essential in liver failure
Daily weights are required if the child can be moved.
Aim for urine output not less than 0.5 ml/kg/hr
Volume given calculated from formula (If child is not dehydrated and
losses are not abnormal).
Daily Maintenance Fluid = approx 2/3 maintenance
2/3x(100ml/Kg body weight for the first 10 Kg+50ml/Kg for the next 10Kg+10ml/Kg for each
Dextrose >10% or higher if hypoglycaemic (BM<4.0)
1.0 - 3.5 mmol/kg/day
NIL while anuric
Paracetamol Overdose Treatment Protocol
If a Paracetamol overdose is suspected or known, the child must be treated
immediately with N-acetylcysteine, at the local hospital whatever the time
between the alleged overdose and the visit to the hospital. The ‘High Risk
Treatment Line' is used in all cases once a level is known. The N-acetylcysteine
should be continued until the INR is normal (<1.2). Indication for treatment after
known time since ingestion by plasma level corresponding to the High Risk
Treatment Line is shown below.
Liver function tests, paracetamol level, INR, blood sugar, renal function
tests and blood gases including lactate. Blood sugar must be closely monitored - (hourly BM stix).
Urine drug screen should be performed as soon as possible
INR, blood sugar, renal function and blood gases must be repeated at
least twice a day and, if abnormal, three times a day. Start immediately broad spectrum antibiotics if INR abnormal and in the presence of abnormal renal function, liposomal Amphotericin i.v.
Hypoglycaemia has to be avoided and the child should be maintained on
10% dextrose. Higher concentrations of dextrose may be needed.
The most important prognostic parameter is acidosis on day 2. If, despite
N-acetylcysteine treatment and good rehydration, the child becomes acidotic, the prognosis is poor. Acidosis is the best prognostic factor independent from all other factors. Even in the presence of a very prolonged INR, a patient who is not acidotic will have 80% chances of surviving. If the pH is <7.25, there is a 95% mortality, therefore the child should be emergency listed for transplantation.
Other factors predicting a poor outcome are the development of grade III
hepatic encephalopathy with oliguric renal failure (which usually occurs three to four days after ingestion), and/or a prothrombin time of >100 seconds, and raised plasma lactate.
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