Jaundice - investigation of prolonged

Definition

Prolonged jaundice = Jaundice persisting for more than 14 days.

Children with clinically apparent jaundice present at 14 days of life or older require:

1. Conjugated and total bilirubin measured

2. Clinical review including examination of stool colour (https://www.childliverdisease.org/wp-content/uploads/2018/01/Yellow-Alert-Stool-Chart-Bookmark.pdf)

Any delay in follow-up for children with neonatal jaundice can lead to poor long term outcomes.

Unconjugated hyperbilirubinaemia

• Total conjugated bilirubin < 20 umol/L and 

• Conjugated bilirubin <20% total bilirubin 

The majority of infants with elevated levels of unconjugated hyperbilirubinaemia will have a non-pathological cause including “breast milk jaundice”.

In a minority, the jaundice may be a marker of important disease including infection, haemolysis and sepsis.

For specific guidance see: https://starship.org.nz/guidelines/jaundice-management-of-neonatal-jaundice/#Atypical-Jaundice-late-onset-and-prolonged

Conjugated hyperbilirubinaemia

• Total conjugated bilirubin > 20 umol/L and

• Conjugated bilirubin >20% total bilirubin

Any patient with conjugated hyperbilirubinaemia should be urgently (same day) discussed with the Paediatric Gastroenterology/Hepatology service.

Important clinic issues

1. Most children with conjugated hyperbilirubinaemia look well but may have important disease.

2. Stool colour - pale stool and dark urine suggests Biliary Atresia (https://www.childliverdisease.org/wp-content/uploads/2018/01/Yellow-Alert-Stool-Chart-Bookmark.pdf)

3. All children with conjugated hyperbilirubinamia require additional Vitamin A,D,E,K

Causes of neonatal cholestasis

There are a number of causes of neonatal cholestasis. Some are serious and some have a time-dependent intervention available.

These include:

• Bile duct abnormalities: biliary atresia, choledochal cyst

• Endocrine: hypopituitarism, hypothyroidism

• Inherited and metabolic: α1- Antitrypsin deficiency, Alagille syndrome, allowimmune liver disease, progressive familial intrahepatic cholestasis

• Infectious: Urinary tract infection, TORCH infections, Varicella CMV and adenovirus.

There are multiple other causes of neonatal cholestasis.

Flow chart for investigation of Neonatal Cholestasis

Note:

• Acholic stools are highly characteristic of cholestasis in infancy. 

• Elevated INR (or PR) requires urgent treatment with vitamin K and repeat INR (or PR) at 4-6 hours. Untreated coagulopathy may lead to spontaneous bleeding and intracranial haemorrhage. 

First line investigations conjugated hyperbilirubinaemia

Second line investigations conjugated hyperbilirubinaemia

Interventions

• All infants with conjugated hyperbilirubinaemia are started on Vit A, D, E, K 

• Early consideration for starting MCT based formula (peptijunior) 

Fat soluble vitamin supplementation

All infants undergoing investigation of conjugated hyperbilirubinaemia should commence fat-soluble vitamin supplementation as soon as possible. 

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: 0.3mL once daily, then titrate dose based on 3 monthly vitamin levels. Note: 0.3mL is equivalent to 10 drops as 1 drop = 0.03mL.

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://pharmac.govt.nz/assets/form-alphatocopherylacetate-VitaminE-and-Retinol-vitaminA.pdf.

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

Vitamin D

Note: Puria® Vitamin D drops have changed their name to Clinicians® Vitamin D drops.

Available preparation: colecalciferol 7500IU/mL or 400 IU/drop oral liquid (Clinicians®)
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://pharmac.govt.nz/assets/form-alphatocopherylacetate-VitaminE-and-Retinol-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.