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New Zealand Child and Youth Clinical Networks (NZCYCN) NZCYCN national guidelines Clinical Guideline

Biliary Atresia (BA) - guidelines


and management of
Date last published: 14 March 2019

Biliary Atresia (BA) is the commonest neonatal liver disease in New Zealand affecting 1 in 8000 live births,
with increased frequency in Maori and Paci c children (approximately 1 in 5000). It is the most common
indication for liver transplantation in childhood.

This document is only valid for the day on which it is accessed. Please read our disclaimer.

The National Paediatric Gastroenterology Clinical Network has agreed that ALL infants with conjugated hyperbilirubinaemia should be
referred to a Paediatric Gastroenterologist or Hepatologist for initial investigation and subsequent management. For North Island
patients, the Paediatric Gastroenterology and Hepatology service is based at Starship Child Health, Auckland and for South Island
patients, the Paediatric Gastroenterology and Hepatology service is based at Christchurch Hospital. The Gastroenterology or
Hepatology team will ensure that all investigations have been completed and other liver diseases considered before the child is
considered for laparotomy and possible Kasai portoenterostomy

Background
Biliary Atresia (BA) is the commonest neonatal liver disease in New Zealand affecting 1 in 8000 live births, with increased frequency in Maori and
Paci c children (approximately 1 in 5000). It is the most common indication for liver transplantation in childhood.

Features of BA include conjugated jaundice, pale stools and dark urine. The treatment is surgical (Kasai portoenterostomy) which is most
successful if performed early, preferably before 6 weeks of age. Approximately 60% of infants with BA will have a successful Kasai (bilirubin
<25mmol/L by 6 months of age). 

Infants with BA who have unsuccessful Kasai will require transplantation. Those with successful Kasai may still require transplantation due to
portal hypertension, recurrent cholangitis and complications of cirrhosis. 

The increased frequency in NZ and the tight timeframe in which to perform surgery means the emphasis is on early referral to a paediatric
gastroenterology service and prompt diagnosis.

Please note:

The need for isotope excretion scan has been removed in this version of the guideline given it lacks speci city to distinguish biliary
atresia from any other form of cholestatic liver disease and has the potential to delay diagnosis
Drug Dosage 
Exclusion of alpha-1-antitrypsin de ciency by blood testing has also been removed due to low yield and potential to delay diagnosis of
biliary atresia while awaiting results.
Information for Families 

Diagnosis
Share 
History and examination

Cardinal features include: Print 


jaundice

pale stools, becoming paler once meconium clears



dark urine
stool colour should be directly observed by a healthcare professional ( https://www.childliverdisease.org/Information/Baby-
jaundice/Yellow-Alert-App)

There should be an absence of features which may indicate other neonatal liver disease, such as:

Congenital heart disease

Facial features of Alagille syndrome

Family history of genetic liver disease

Alpha-1-antitrypsin phenotype should be requested but the result is not critical prior to operative cholangiogram and Kasai in babies
whose ultrasound and liver biopsy are highly suggestive of BA

Alpha-1-antitrypsin level is not recommended as it is an acute phase marker and cannot accurately differentiate between carriers and
affected individuals

Syndromic biliary atresia (biliary atresia-splenic malformation)

10% cases have extra-hepatic features which may include:

Abdominal situs inversus

Intestinal malrotation

Polysplenia (often poorly functional)

Pre-duodenal portal vein

Portal vein and IVC anomalies

Congenital heart disease

These children have unique considerations if liver transplantation is required

Investigations

Any infant with jaundice beyond two weeks of age or pale stools should undergo clinical assessment and have blood sent for a split bilirubin. If
conjugated hyperbilirubinaemia (>20 mmol/L or >20% total bilirubin) is con rmed, perform rst line investigations as per Starship Clinical
Guidelines.

Laboratory features

Conjugated hyperbilirubinaemia (>20mmol/L or >20% total bilirubin)

Mild-moderate elevation of AST, ALT, GGT, ALP

INR or prothrombin time should be measured as infants may be vitamin K de cient

Fat soluble vitamin de ciencies (vitamins A, D, E, K) are common

Radiological features

Absent, small or contracted gall bladder on fasting abdominal ultrasound - note the presence of a gall bladder does not exclude BA

Isotope excretion scan is not recommended as it delays diagnosis due to need for phenobarbitone priming. It can however be reserved
Drug Dosage 
for investigation of jaundice in selected infants with ambiguous results, on request of the hepatologist

Histological ndings on percutaneous liver biopsy


Information for Families 
Cholestasis

Fibrosis Share 
Proliferation of bile ductules
Print 
Absence of diagnostic features of other neonatal liver diseases

Liver biopsy should be assessed by a paediatric pathologist experienced in the diagnosis of BA



Features may become more obvious with time so infants with jaundice and pale stool whose initial biopsy is not diagnostic may require a
second biopsy if signs do not improve
Management - pre-operative to 6 months following Kasai

Operative management should be undertaken by a paediatric surgeon experienced in the management of biliary atresia, who performs
cholangiograms and Kasai portoenterostomy on a regular basis.

A cholangiogram that shows a normal intra hepatic biliary tree, normal external hepatic ducts and normal drainage into the duodenum
excludes the diagnosis.

If biliary atresia is con rmed at exploration, the child will proceed to Kasai portoenterostomy at the same operation.

Pre-operative management

INVESTIGATIONS

Initial investigations as per prolonged jaundice guideline determine whether to proceed to operative cholangiogram +/- Kasai
portoenterostomy

Day before Kasai


• FBC                                               • Clotting pro le
• U&E                                                • Group and hold
• LFTs                                              • Chest xray as clinically indicated

Nutritional management

All babies with conjugated jaundice will be seen by a dietitian with experience in managing paediatric liver disease

For formula-fed infants, consideration will be given to switching to a feed with medium chain triglyceride as the predominant lipid
source eg PeptiJunior

For breast-fed infants, consideration will be given to introducing some supplemental formula feeds using a medium chain triglyceride
based feed eg Pepti-Junior while allowing the baby to continue some breast feeding. Another option would be to add MCT to expressed
breast milk

Vitamin supplementation

Children with BA are almost always de cient in fat-soluble vitamins A, D, E and K

Oral supplements should be commenced as soon as conjugated jaundice is detected and should continue throughout - see Starship
Clinical Guideline on Prolonged Jaundice

Baseline levels of vitamins A, D and E should be requested but it is not necessary to await results before commencing supplements

If INR or prothrombin ratio is abnormal, IV vitamin K should be prescribed and continued daily until INR or PR is normal, then switched
to oral
Drug Dosage 
Vitamin doses should be titrated according to growth and vitamin levels 

See Vitamin Supplementation Table for dosing information Information for Families 
Parental education and support
Share
All families whose babies are suspected of having BA will have access to a Clinical Nurse Specialist (CNS) who can provide education
about BA and liver disease
Print 
Families should be offered a referral to a social worker and the Consult Liaison Team for psychological support

Other referrals which may be considered include, to a lactation consultant and maternal mental health on the guidance of the Consult
Liaison Team 

Intra-operative management
Anaesthetic considerations

Percutaneous central line will usually be placed. As far as possible, the right sided neck veins should be avoided as these may be
required during subsequent liver transplantation. Urinary catheterisation will be required

Consideration will be given to epidural analgesia

Surgical approach

Surgery should be carried out in a centre with a multidisciplinary team experienced in the care of paediatric liver disease

A paediatric surgeon with speci c expertise in Kasai portoenterostomy will undertake the surgery

The surgeon will directly note the upper gastrointestinal anatomy along with the appearance of the liver and extra-hepatic biliary tree
and may perform an intra-operative cholangiogram to con rm the ndings

An intraoperative cholangiogram should not be performed as a diagnostic test in isolation, but should be performed with a view to
proceeding to Kasai, should the diagnosis of BA be con rmed.

The type of biliary atresia will be noted for future reference

A standard, open Kasai portoenterostomy is the preferred operation

If evidence of severe cirrhosis with portal hypertension, the surgeon may elect not to proceed with Kasai, in view of the inevitability of
progression of liver disease. These children should receive intensive nutritional support and should be assessed for transplant without
undue delay.

Antimicrobial cover

IV cefazolin at induction at 30/mg/kg/dose to continue 6 hourly post-operatively for 72 hours

If operating time is > 4 hours or blood loss > 50% total volume re-dosing should occur at 4 hours

Intravenous uids

Intravenous uid management should be prescribed following the Starship Intravenous Fluids Clinical Guideline.

Post-operative management during initial hospital stay

Return to the ward

Most babies can be cared for in the paediatric surgical HDU on ward 24B

Initial management will be undertaken by the Paediatric Surgical team who will transfer the baby to the Paediatric Hepatology
(Gastroenterology) team once the baby is stable

Antimicrobial cover

IV cefazolin 30/mg/kg/dose to continue 6 hourly post-operatively for 72 hours or until the baby is feeding and can tolerate oral
antibiotics

On completion of IV antibiotics commence long term oral cholangitis prophylaxis with co-trimoxazole (2mg/kg/day as trimethoprim
component once daily) for 12 months post Kasai

For children who experience suspected or established cholangitis, co-trimoxazole will be continued until 12 months following the most
recent cholangitis episode Drug Dosage 

Ensure co-trimoxazole dose is regularly adjusted for weight gain


Information for Families 
Steroid therapy

Start steroids on day 1 post op unless there is fever or evidence of sepsis: Share 
Methylprednisolone IV 20mg daily decreasing by 2.5mg daily until 5mg/day
Then prednisolone 5mg daily orally for one further week then stop
Print 
Babies who are otherwise ready for discharge can be transitioned to oral steroids earlier than 7 days

Evidence for the bene t of steroid therapy on the long-term outcome of BA is weak and will be reviewed every 2 years

Fat soluble vitamin supplementation


Oral fat soluble vitamin supplements will be re-commenced as soon as practical following surgery
Vitamin K:
Day 1: Start 2mg IV daily for 3 days
Day 4: Switch to enteral dose as per vitamin supplementation table
If there is a delay to feeding or INR/prothrombin ratio is prolonged, continue IV vitamin K until the baby is feeding and INR/prothrombin
ratio is normal

Analgesia

Analgesia will be provided by oral/enteral (preferred) or IV paracetamol and either an epidural or IV NCA (Nurse Controlled Analgesia)
morphine.

Choice of analgesia on return to the ward is at the discretion of the anaesthetist and dependent on the clinical status of the child

Epidural or NCA will be continued until the child is tolerating feeds and then they will be transitioned to oral medication for 24-48 hours

Paracetamol dosing will be 10 mg/kg/dose up to 4 times daily. In general, non-steroidal anti-in ammatory drugs should be avoided in
children with liver disease

Fluids

Day 1: 70% maintenance uids as per Starship Intravenous Fluids Clinical Guideline - if able, to be administered as 50:50 IVF and
Pedialyte

Day 2: Change Pedialyte to continuous enteral milk feeding via NG tube at 50ml/kg/day in addition to the IV uids. The type of milk feed
will be directed by the paediatric dietitian

Day 3: Commence bolus feeds, the volume and type being directed by the surgical team and paediatric dietitian. IV uids will be weaned
accordingly

Day 4 onwards will depend on progress over the previous days

Nutrition

See uids section above for timing of feed commencement post-operatively

As pre-operatively, the choice of feed depends on whether the baby is predominantly breast or formula fed

For formula-fed infants, a feed with medium chain triglyceride as the predominant lipid source eg PeptiJunior will be used

For breast-fed infants, consideration will be given to using some supplemental formula feeds with a medium chain triglyceride based
feed eg Pepti-Junior while allowing the baby to continue some breast feeding. Another option would be to add MCT to expressed breast
milk

Supplementation with medium chain triglyceride based feeds will be continued at least until it is known whether the Kasai has been
successful

Due to the importance of good nutrition in managing paediatric liver disease, there will be a low threshold to commence nasogastric
tube feeding in the event of sub-optimal weight gain

Monitoring

Stool colour will be recorded daily and in particular whether pigment appears in the stool. One stool sample daily should be collected
into a stool pot for review by the medical team Drug Dosage 
Day 1: FBC, LFTs, coagulation screen, electrolytes

Thereafter, blood tests will be undertaken as clinically indicated but usually at least twice a week in the Information
rst week for Families 

Urinary catheter will be removed as soon as urinary output is stable (aim ≥1mlkg/kg/hour) and will be at the direction of the Paediatric
Surgeon (usually day 2-3) Share 

Prior to discharge from hospital


Print 
The Clinical Nurse Specialist (CNS) will provide further education to the family prior to discharge and ensure family are aware of follow up
arrangements in the community. The family will be provided with;

Discharge letter

Blood forms for ongoing monitoring


The paediatric liver disease - Biliary Atresia: a parent guide

 Kids Foundation welcome pack with application for membership

The CNS will ensure any referrals to home care nursing teams = (for dressing changes or monitoring of weight) after discharge are
completed

The CNS will ensure that the family and GP are aware of the plan for the baby's immunisations which may have been delayed due to
medical reasons eg prednisone

The CNS will provide a copy of the accelerated immunisation schedule to the family and the GP service

For babies who live outside Auckland, the CNS will contact the local paediatric team with details of the follow-up plan, including blood
monitoring and clinic arrangements the accelerated immunisation schedule and the link to the shared care nursing resource 

A prescription for discharge medications will be provided

Repeat referrals can be made to social work and the Consult Liaison Team if required

Post-operative management following discharge (up to 6 months following Kasai)

Blood monitoring

At least weekly LFTs for the rst month following Kasai, spacing out to fortnightly and monthly thereafter for the rst 6 months

Monthly coagulation, FBC, U&E, vitamins A, D, E and other investigations as required

For children outside Auckland and Christchurch, results should be copied (electronically if possible) through to the local paediatric
hepatology service

Other monitoring

Weekly weights for the rst month, preferably by a home care nursing service using the same scales spacing out to fortnightly then
monthly for the rst 6 months

Sub-optimal growth according to the WHO infant growth chart should be reported to the Auckland or Christchurch paediatric hepatology
service

Need for abdominal ultrasound will be determined by progress and will be advised by the Auckland or Christchurch paediatric
hepatology service

Clinic appointments

Frequency will depend on where the baby lives and their progress

Auckland and Christchurch patients will generally remain under the sole care of the paediatric hepatology service unless there are other
indications for referral to general paediatrics

Other patients will predominantly be managed in a shared care model between the Auckland or Christchurch paediatric hepatology
service and a local general paediatrician

As far as possible, infants living outside Auckland and Christchurch will be seen in Paediatric Gastroenterology visiting outreach clinics if
these exist locally

Initially, babies will be reviewed locally a week following discharge, spacing out to fortnightly then a minimum of monthly appointments
Drug Dosage 
At a minimum, the paediatric hepatology service will review the babies at 1, 3 and 6 months following Kasai to determine the success or
otherwise of the operation and to plan future care
Information for Families 
Reasons for more frequent review include, but are not limited to:
- Poor growth
Share 
- Synthetic liver dysfunction
- Ascites
- Portal hypertension and/or gastrointestinal bleeding Print 
- Recurrent cholangitis
- Clear need to proceed to liver transplant assessment

Possible complications

Cholangitis
Portal hypertension

Poor nutritional management

Management after 6 months post Kasai


Management will depend on whether the Kasai operation has been successful or not

Successful Kasai is de ned as a normal bilirubin (< 20 umol/L) at 6 months following Kasai

Children with a successful Kasai can lead normal lives but there is still a high risk of requiring liver transplantation in later infancy,
childhood or adulthood

It is sometimes apparent that the Kasai has failed before 6 months. In these cases, liver transplant assessment should not be delayed

In infants with unsuccessful Kasai, liver transplant assessment is required and management will proceed according to the Starship
Hospital liver transplant protocol

Management of infants with successful Kasai (normal bilirubin at 6 months following Kasai)

Children with a successful Kasai can lead normal lives but there is still a high risk of requiring liver transplantation in later infancy,
childhood or adulthood

Most patients with biliary atresia will develop cirrhosis during childhood

In general, around 20% of patients with BA survive through to adulthood with their native liver

The commonest complications leading to a need for liver transplantation are recurrent cholangitis and the development of portal
hypertension.

Hepatocellular carcinoma can develop in any patient with cirrhosis, even during childhood. 

Blood monitoring

3 monthly coagulation, FBC, U&E spacing out to 6 monthly in older children

Vitamins A, D, E until levels are normal then annually thereafter

Annual: vitamins A, D, E, AFP, hepatitis A and B serology

For children outside Auckland and Christchurch, results should be copied through to the Auckland or Christchurch paediatric hepatology
service

Other monitoring

Monthly weights for the rst year, preferably by a home care nursing service using the same scales after which growth can be assessed in
clinic appointments

Sub-optimal growth according to the WHO infant growth chart should be reported to the Auckland or Christchurch paediatric hepatology
service

Abdominal ultrasound should be performed annually with speci c attention to:


- Spleen size
- Evidence of cirrhosis and portal hypertension Drug Dosage 
- Dilated bile ducts or bile lakes
- Focal liver lesions
Information for Families 
The paediatric hepatology service will advise whether the USS needs to include Doppler assessment of the hepatic vasculature

For children who are old enough to breath hold on request, elastography (or FibroScan) if available, can be a useful tool toShare
look for

development of brosis

Radiology investigations can usually be performed locally and referred to the paediatric hepatology service via the PACS radiology
Print 
system

Nutrition

Infants whose Kasai has been successful can usually switch to a conventional infant formula once their bilirubin is normal

Solids foods should be introduced at the usual time


Older children with a successful Kasai can eat a normal diet

Children with recurrence of jaundice will likely need liver transplant assessment and nutritional intervention as per the liver transplant
protocol

Fat soluble vitamin supplementation

Fat soluble vitamin supplements can usually be discontinued once the bilirubin has been normal for one month and serum vitamin
levels are normal

Vitamin levels should be monitored at least annually and supplements re-introduced if needed

Clinic appointments

Auckland and Christchurch patients will generally remain under the sole care of the paediatric hepatology service unless there are other
indications for referral to general paediatrics

Other patients will predominantly be managed in a shared care model between the Auckland or Christchurch paediatric hepatology
service and a local general paediatrician

As far as possible, children living outside Auckland and Christchurch will be seen in Paediatric Gastroenterology visiting outreach clinics
if these exist locally

At a minimum, the paediatric hepatology service will review patients at 6 monthly intervals and can alternate visits with the local
paediatrician

Reasons for more frequent review include, but are not limited to:
- Poor growth
- Synthetic liver dysfunction
- Ascites
- Portal hypertension and/or gastrointestinal bleeding
- Recurrent cholangitis
- Clear need to proceed to liver transplant assessment

Transition to adult services

Young people will be transitioned to adult services via the Young Persons Liver Clinic held jointly with the adult hepatology service at
Auckland City Hospital as they may still require liver transplantation at a later stage. Those living outside Auckland will also require a
local transition to adult gastroenterology services

General lifestyle advice will be offered at this stage

Young people with BA will require re-education about their disease and the long-term implications

Complications of Biliary Atresia (BA)

Cholangitis

Background

Common complication in BA and an important determinant of long-term outcome

Repeated episodes may result in progressive liver disease, cirrhosis & portal hypertension Drug Dosage 
Low index of suspicion and low threshold to promptly start antibiotics in event of otherwise unexplained fever and/or rising liver
function tests Information for Families 
Symptoms include fever, worsening jaundice or acholic stool
Share 
Cholangitis can be dif cult to distinguish from a non-speci c viral illness which may also result in fever and deterioration in LFTs

Cholangitis is usually caused by gram negative organism but usually blood cultures are negative. Nonetheless blood cultures should be
Print 
taken before starting IV antibiotics

Investigations

FBC, CRP, LFTs, INR or PR, U&E, blood and urine cultures
Abdominal ultrasound may be necessary to look for dilated bile ducts or bile lakes, evidence of portal hypertension,
hepatosplenomegaly and presence of ascites. The paediatric hepatology service will advise on whether ultrasound is necessary

Treatment

Suspected cholangitis should be discussed with the paediatric hepatology service and length of treatment determined

For events highly likely to be cholangitis, treatment will usually comprise of 2 weeks IV antibiotics and infants should be referred for
central venous line placement if it is dif cult to maintain peripheral IV access

Milder illnesses, especially if mainly of biochemical disturbance, may be able to be treated initially with IV antibiotics but transitioning to
oral therapy

FIRST LINE THERAPY

  Cefotaxime 50mg/kg q8 hourly and


Amoxicillin 50mg/kg q8 hourly IV

OR Gentamycin 7.5mg/kg/dose <10 years, 5mg/kg>10 years


Amoxicillin 50mg/kg q8 hourly IV

SECOND LINE THERAPY

  Gentamycin 7.5mg/kg/dose <10 years, 5mg/kg>10 years


Amoxicillin 50mg/kg q8 hourly IV

OR Ceftazidime 50mg/kg q8 hourly and


Amoxicillin 50mg/kg q8 hourly IV

OR Tazocin 90mg/kg q8hourly ( max 4.5gms) and


Amoxicillin 50mg/kg q8 hourly IV

Second line antibiotics should be considered if there is convincing evidence of cholangitis and no improvement on rst line treatment or
previous recurrent episodes of cholangitis

Repeated courses of IV antibiotics for biochemical abnormalities alone should be avoided due to the risk of bacterial resistance and
fungal colonisation

Cholangitis prophylaxis

Co-trimoxazole (2mg/kg/day as trimethoprim component once daily) for 12 months post Kasai

For children who experience suspected or established cholangitis, co-trimoxazole will be continued beyond 12 months following Kasai at
least until 12 months following the most recent cholangitis episode Drug Dosage 
Co-trimoxazole dose must be regularly adjusted for weight gain

Consideration can be given to changing prophylaxis to cipro oxacin if cholangitis break-through occurs Information for Families
on co-trimoxazole at an 
appropriate dose for weight. Note however that cipro oxacin in liquid formulation is not funded in the community and currently requires
NPPA approval Share 
Infectious diseases approval for antibiotics

Antibiotics suggested in this guideline have already been approved by the Infectious Disease team at Starship Hospital Print 

Further approval is not required



Central venous line placement may be required. As far as possible the right sided neck veins should be avoided as there may be required
during subsequent liver transplantation
Preservation of the right internal jugular vein is important for future potential liver failure and transplantation management.

Right side CVL placement should therefore be avoided where possible

If required right sided placement must be discussed with hepatology prior to placement to ensure access is preserved for future
transplant.

Portal hypertension

Portal hypertension is one of the commonest long-term complications of BA and an indication for liver transplant even if Kasai has been
successful.  There are no good non-invasive tools to screen for portal hypertension but clues may include:

Splenomegaly or increasing spleen size

Low or decreasing platelet and white cell counts (hypersplenism)

Ultrasound features such as abnormalities in portal vein ow, splenomegaly, visible varices

The paediatric hepatology service will advise on the need for endoscopy to investigate portal hypertension

If varices are present on endoscopy, these may require treatment, the preferred option being band ligation

Features which suggest varices should be treated include:

Large or rapidly progressive varices grade two varices with high risk stigmata

Anaemia

Suspected or con rmed Gastrointestinal (GI) bleed or indication of a previous GI bleed on endoscopy

Patient lives remotely or far from a local hospital

Gastrointestinal (GI) bleeding should be managed urgently via the GI bleeding protocol and likely indicates a need for liver transplant
assessment. Varices per se may not require transplantation if small, not rapidly progressive and without any GI bleeding. However, all varices
and portal hypertension should be managed by the paediatric hepatology service who can decide on the need for transplantation

Nutritional management in Biliary Atresia


As before Kasai, the choice of feed depends on whether the baby is predominantly breast or formula fed

For formula-fed infants, a feed with medium chain triglyceride as the predominant lipid source eg PeptiJunior will be used

For breast-fed infants, consideration will be given to using some supplemental formula feeds with a medium chain triglyceride based
feed eg Pepti-Junior while allowing the baby to continue some breast feeding. Another option would be to add MCT to expressed breast
milk

Supplementation with medium chain triglyceride based feeds will be continued at least until it is known whether the Kasai has been
successful, after which the infant can be switched to a conventional infant formula

Due to the importance of good nutrition in managing paediatric liver disease, there will be a low threshold to commence nasogastric
tube feeding in the event of sub-optimal weight gain

Older infants requiring intense nutritional supplementation can be changed from PeptiJunior to an MCT based feed containing branched
chain amino acids such as Heparon Junior Drug Dosage 
Solid foods should be encouraged and introduced at the usual time
Information for Families 
Fat soluble vitamin supplementation
Share 
All infants undergoing investigation of conjugated hyperbilirubinaemia should commence fat-soluble vitamin supplementation as soon as
possible. 
Print 
Vitamin A

Available preparation: Optimus Vitamin A drops, 666.7mcg per 2 drops.



Note: Vitadol C® has been delisted from the Pharmaceutical Schedule and supply withdrawn from the NZ market. This has been replaced by

Optimus Vitamin A drops.


Dose: 6 drops once daily, then titrate dose based on 3 monthly vitamin levels. Patients may require up to 10 drops daily to prevent de ciency.
Note: 1 drop = 0.03mL.

Information about converting doses between Vitadol C® and Optimus Vitamin A drops as below. Please prescribe in drops where appropriate.

Dose Equivalency Table

Product Drops mcg IU mL

Vitadol C® 10 666.7 2220.1 0.3

Optimus Vitamin A drops 2 666.7 2220.1 0.06

Conversions for most common dosing regimens:


1mL of Vitadol C = 6 drops of Optimus Vitamin A drops (0.18mL)            
0.3mL of Vitadol C = 2 drops of Optimus Vitamin A drops (0.06mL)

Special notes: Clinicians must complete the PHARMAC application form for funding in the community. This can be completed by any SMO. The
form is accessible here: https://www.pharmac.govt.nz/assets/form-alphatocopherylacetate-and-vitaminA.pdf

Community pharmacies must source the product directly from the manufacturer Optimus Healthcare Auckland, Ph 09 5800915

Vitamin D

Available preparation: colecalciferol 7500IU/mL or 400 IU/drop oral liquid (Puria®)


Dose: 0.5mL once daily (3750 IU), then titrate dose based on 3 monthly vitamin levels
Special notes: the rubber bung can be removed from the bottle to allow dose administration via oral syringe.

Vitamin E

Available preparation: alpha-tocopheryl acetate 156IU/mL (Micel- E®)


Dose: 0.5 mL once daily, then titrate dose based on 3 monthly vitamin levels.
Special notes: clinicians must complete the PHARMAC application form for funding in the community. This can be completed by any SMO.
The form is accessible here: https://www.pharmac.govt.nz/assets/form-alphatocopherylacetate-and-vitaminA.pdf

Vitamin K

Available preparation: Phytomenadione 2mg or 10mg ampoules (Konakion®)


Dose: 2mg once daily and increase according to INR. Doses of 2mg to 10mg once daily may be given either IV or orally.
Special notes: Konakion® is only available as an IV preparation. This can safely be used for oral administration. Note: The 2mg ampoules are
dispensed with an oral syringe so please identify this product when prescribing. Please round dose accordingly to achieve easy to draw up
volumes.

Immunisations
Children should be immunised according to the accelerated schedule (see https://www.health.govt.nz/publication/immunisation-
handbook-2017) Drug Dosage 
Annual in uenza vaccine should be offered
Information for Families 
Hepatitis A and B boosters should be offered if annual screening shows loss of antibody as previous immunisation and protective
antibodies cannot be relied upon in the event of exposure in patients with cirrhosis
Share 
Screening for Hepatocellular Carcinoma (HCC)
Print 
All patients with cirrhosis have a risk of developing HCC and this can occur even in childhood

During childhood, alphafoetoprotein (AFP) should be performed at least annually and more frequently if abnormal

Annual ultrasound is also required to monitor for HCC and should be requested more frequently if AFP is abnormal

HCC can be dif cult to differentiate from regenerative cirrhotic nodules on USS, in which case MRI with contrast should be requested
Once children reach adolescence, they should commence on the adult HCC screening protocol of 6 monthly AFP and USS

HCC in biliary atresia is an indication for liver transplantation

Indications for liver transplantation


Portal hypertension, especially if gastrointestinal bleeding has occurred

Recurrent cholangitis

Recurrence of jaundice and/or synthetic liver failure

Suspected hepatocellular carcinoma

Drug Dosage 

Information for Families 

Share 

Print 