Neonatal Cholestasis
see neonatal jaundice also
Pale stools
Dark urine
Ix (as per SCH Gastro Team)
Urine
Haemolytic disease of the newborn can have cholestasis
Biliary atresia: Fibrotic biliary tree, no/abnormal gall bladder, Kasai best conducted <45-60d
Alagilles: reduced number of interlobular bile ducts on biopsy
Neonatal hepatitis (congenital infection, alpha-1 antitrypsin deficiency; often idiopathic)
Metabolic (galactosaemia, fructose intolerance - ask about sucrose/fructose in food/medication)
CF
TPN related
NB in older children
Cholestasis post haematopoetic cell transplant/chemo/ingestion of alkaloid toxins/radiation Rx or liver transplantation
URSO: increases bile flow
Pale stools
Dark urine
Ix (as per SCH Gastro Team)
Urine
- Urinalysis
- Urine reducing substances
- Microscopy, culture and sensitivity
- PCR for Cytomegalovirus (CMV)
- Metabolic screen: amino acids, organic acids, succinyl acetone, aldolase (Westmead: 98453125)
- Full blood count
- Electrolytes, urea, creatinine
- Liver chemistry tests
- Cholesterol, triglycerides
- Coagulation profile
- Fat soluble vitamins: Vit A, D, E
- Bile salts
- Thyroid function tests
- Iron studies
- Alpha 1 - antitrypsin phenotype (RPA: 95157675)
- Red cell galactose-1-phosphate uridyl transferase (Gal-1-PUT)
- Serology: Toxoplasma, Syphillis, Rubella, EBV, CMV, Herpes simplex, Human herpes virus-6, Hepatitis A/B/C, Enteroviruses, Parvovirus B19
- Newborn screening tests (Westmead: 98453255 / 98453659)
- Transferrin Isoforms
- Abdominal ultrasound
- 99m Technetium labelled HIDA scan (5 days of 5mg/kg/day phenobarbitone premedication)
- Sweat chloride test
- Inform surgical team
- Percutaneous liver biopsy (if no coagulopathy)
- Urine and serum bile acid metabolites
- Alagille syndrome: Chest and thoracolumbar spine XR, cardiology referral for echocardiogram, ophthalmology referral for fundoscopy (posterior embryotoxon)
- Chromosomes (karyotype and banding) if dysmorphic
Haemolytic disease of the newborn can have cholestasis
Biliary atresia: Fibrotic biliary tree, no/abnormal gall bladder, Kasai best conducted <45-60d
Alagilles: reduced number of interlobular bile ducts on biopsy
- Chronic cholestasis (94 percent)
- Cardiac murmur or anomalies, most commonly peripheral pulmonic stenosis (92 percent)
- Dysmorphic facies, consisting of broad nasal bridge, triangular facies, and deep set eyes (91 percent)
- Eye findings, most commonly posterior embryotoxon (prominent Schwalbe line) (80 percent)
- Butterfly vertebrae (67 percent)
- Renal involvement, most commonly renal dysplasia
Neonatal hepatitis (congenital infection, alpha-1 antitrypsin deficiency; often idiopathic)
Metabolic (galactosaemia, fructose intolerance - ask about sucrose/fructose in food/medication)
CF
TPN related
NB in older children
Cholestasis post haematopoetic cell transplant/chemo/ingestion of alkaloid toxins/radiation Rx or liver transplantation
- Hepatic sinusoidal obstruction syndrome (SOS) (previously hepatic veno-occlusive disease (VOD)
- hepatomegaly, right upper quadrant pain, jaundice, and ascites usually within 3w of transplants
- due to occlusion of the terminal hepatic venules and hepatic sinusoids
- Budd-Chiari syndrome clinically identical but hepatic venous outflow obstruction in hepatic veins and IVC.
- GVHD involving liver presentation, differentiated by other system involvement eg diarrhoea/rash
URSO: increases bile flow