Jaundice - investigation of prolonged
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Prolonged jaundice = Jaundice persisting for more than 14 days.
Children with clinically apparent jaundice present after 14 days of life require:
- Clinical review including examination of stool colour
- Conjugated and total bilirubin measured
Any delay in follow-up for children with neonatal jaundice can lead to poor long term outcomes.
Primary investigation of prolonged jaundice will include: feeding history, examination of the baby, examination of stool colour (acholic stools are highly characteristic of cholestasis in infancy). Initial laboratory tests should include:
- Bilirubin (total & direct (conjugated)). Bilirubin fractionation is the most important test in any infant who has more than two weeks of jaundice.
- Urine (for infection and reducing substances)
- and possibly a G6PD screen.
- Total conjugated bilirubin < 20 umol/L and
- Conjugated bilirubin <20% total bilirubin
Children with unconjugated hyperbilirubinaemia require assessment for underlying diagnosis and therapy.
- Clinical assessment - sepsis
- Does the child require phototherapy?
- Urine for Dipstick +- culture?
- Consider FBC and film/ G6PD/ Coombs for haemolysis
- Review maternal blood group for ABO/ rhesus incompatibility
- Total conjugated bilirubin > 20 umol/L or
- Conjugated bilirubin >20% total bilirubin
Any patient with conjugated hyperbilirubinaemia will be urgently reviewed by the Paediatric Gastroenterology/Hepatology service.
Important clinic issues
- Most children with conjugated hyperbilirubinaemia look well but may have important disease.
- Stool colour - pale stool and dark urine suggests Biliary Atresia
- All children with conjugated hyperbilirubinamia require additional Vitamin A,D,E,K
Causes of neonatal cholestasis
Disorders in bold are more common or have specific therapy available
|Bile duct abnormalities||Endocrine disorders||Inherited and metabolic disorders|
Spontaneous perforation of bile ducts
Neonatal sclerosing cholangitis
Bile acid synthesis disorders
Progressive familial intrahepatic cholestasis
Neimann-Pick type C
Congenital disorders of glycosylation
Fatty acid oxidation disorders
Hereditary Fructose Intolerance
Urinary tract infection
(toxoplasmosis, rubella, CMV, herpes viruses)
Human Herpes virus-6, Varicella-zoster
HIV, Hepatitis B
Echo, Adeno, Coxsackie-virus
Foetal alcohol syndrome
|Vascular Disorders||Chromosomal Disorders||Miscellaneous|
Congestive heart failure
|Trisomy 21, 13, 18
ARC syndrome (Arthrogryposis, renal tubular dysfunction and cholestasis)
Adapted from McKiernan 2002
Flow Chart for Investigation of Neonatal Cholestasis
- Acholic stools are highly characteristic of cholestasis in infancy.
- Bilirubin fractionation is the most important test in any infant who has more than two weeks of jaundice.
- Liver enzymes are of limited help to differentiate between hepatocellular and cholestatic liver injury.
- Synthetic liver function is best assessed by albumin level and clotting function. Untreated hypothrombinaemia may lead to spontaneous bleeding and intracranial haemorrhage.
First line investigations conjugated Hyperbilirubinaemia
|FBC and blood film|
|Total and conjugated bilirubin|
|AST, ALT, GGT, ALP|
|Blood group and coombs|
|T4 and TSH|
|α1 Antitrypsin phenotype (not level)|
|Cholesterol / triglycerides|
|INR/ APTT/ Fibrinogen|
|Blood sugar q4 hours first 24hours|
|Guthrie card result review|
|Maternal toxoplasma serology|
|Maternal Syphilis status|
|Maternal Rubella status|
|Maternal Hepatitis B status|
Second line investigations conjugated Hyperbilirubinaemia
|Urine organic acids|
|Urine amino acids|
|Serum amino acids|
|Herpes simplex PCR
(if clinically suspected)
|Hepatitis A Virus IgM|
|Epstein Barr Virus serology|
|Urine calcium/Phosphate creatinine ratio|
Download printable pdf of Second Line Investigations
Third line investigations conjugated Hyperbilirubinaemia
(repeat if normal & suspect diagnosis)
|Urine bile acids|
|Very long chain fatty acids|
|White Blood Cell enzymes if indicated|
|Muscle/ skin biopsy|
|Transferrin Isoelectric Focusing|
|Bone Marrow aspirate|
- 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.
|Available preparation: Vitadol C® = 2000 micrograms vitamin A per gram = 1 ml =7500 IU|
|Starting dose is 1ml once daily|
|NB: Vitadol C® is only partially subsidised in the community; families should be informed they will be required to pay a part charge for this medicine.|
|Starting dose = 30-50 nanograms/kg once a day rounded to nearest 100 nanograms|
|Available preparation = Alfacalcidol (One-Alpha drops®) = 100 nanograms of 1-alpha-OH vitamin D3 per drop.|
|NB: This preparation should be prescribed in drops.|
|Starting dose = 50 mg (68 IU) once a day.|
|Available preparation = d-alpha-tocopheryl acetate (Micelle E®) = 156 IU/ml|
|Suggested dose is therefore 0.5 ml once daily.|
|NB: SPECIAL AUTHORITY is required for vitamin E administration and will be completed by the child's paediatrician prior to the child leaving hospital.|
|Suggested dose = 2.5 mg once a day|
|This is ¼ of an adult 10 mg Konakion tablet which can be crushed and mixed with water|
Mckiernan P. Neonatal cholestasis. Seminars in Neonatology. 2002 7 (2): 153 - 165
Did you find this information helpful?
- Date last published: 01 April 2010
- Document type: Clinical Guideline
- Services responsible: Paediatric Gastroenterology/Hepatology
- Author(s): Simon Chin, Stephen Mouat
- Editor: Greg Williams
- Review frequency: 2 years
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