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Paediatrics: Jaundice
Jaundice occurs when serum bilirubin >25–30mmol/L. It is rare outside the neonatal period.
Jaundice
Jaundice occurs when serum bilirubin >25–30mmol/L. It is rare outside the neonatal period. First, determine the SBR and conjugated (direct) fraction. Unconjugated jaundice is rarely due to liver disease. Conjugated jaundice (>20mmol/L) is due to liver disease and requires investigation.
Unconjugated jaundice
Due to excess bilirubin production, impaired liver uptake, or conjugation.
Causes
- Haemolysis (spherocytosis, G6PD deficiency, sickle cell anaemia, thalassaemia, HUS).
- Defective bilirubin conjugation (Gilbert syndrome, Crigler–Najjar syndrome).
Intrahepatic cholestasis
Here, jaundice is due to hepatocyte damage +/– cholestasis. There is un-conjugated +/– conjugated hyperbilirubinaemia.
Causes
Infectious
- Viral hepatitis, including chronic hepatitis.
- Bacterial hepatitis (leptospirosis [Weil’s disease], septicaemia, Mycoplasma, liver abscess).
- Toxoplasma gondii.
Toxic
- Drugs or poisons, e.g. paracetamol overdose, sodium valproate, anti-TB drugs, cytotoxic drugs.
- Fungi (Amanita phalloides).
Metabolic
- Galactosaemia, hereditary fructose intolerance.
- Tyrosinaemia type 1.
- Wilson’s disease.
- A1-antitrypsin deficiency.
- Hypothyroidism.
- Peroxisomal disorders, e.g. Zellweger syndrome.
- Dubin–Johnson syndrome, Rotor syndrome.
Biliary hypoplasia
- Non-syndromic.
- Syndromic, e.g. Alagille syndrome.
Cardiovascular
- Budd–Chiari syndrome.
- Right heart failure.
Autoimmune hepatitis
Autoimmune hepatitis.
Cholestatic (obstructive) jaundice
Conjugated hyperbilirubinaemia is due to bile tract obstruction.
Causes
- Biliary atresia.
- Choledochal cyst.
- Caroli’s disease.
- Primary sclerosing cholangitis (commonly associated with IBD).
- Cholelithiasis (may be secondary to chronic haemolysis).
- Cholecystitis.
- Cystic fibrosis.
- Obstructive tumours or cysts.
Management of jaundice
Full history
e.g. medications, family history, overseas travel, past blood transfusions, jaundice contacts, pale stools, or dark urine (cholestasis).
Examination
Vital signs; conscious level (hepatic coma); hepatic stigmata (= chronic liver disease); pallor (haemolysis); hepatomegaly; splenomegaly; ascites; peripheral oedema.
Investigations
Depending on which of the above pattern presents these may include:
- FBC, blood film, reticulocyte count.
- Coagulation studies.
- U&E, SBR (total and conjugated), LFT, albumin, total protein, TFT.
- Viral serology (hepatitis A, B, C, EBV, CMV), blood culture, leptospira and toxoplasma antibody titres.
- IEM screen, ammonia, copper studies (serum copper i, d, or n, serum caeruloplasmin ‘fall’ in Wilson’s disease), blood glucose, A1-antitrypsin level, galactose-1-uridyl-phosphatase level.
- Immunoglobulins, anti-nuclear antibody, smooth muscle and liver/ kidney antibodies (autoimmune hepatitis);
- The abdominal US, abdominal CT/MRI, biliary scintigraphy, e.g. hepatoiminodiacetic acid [HIDA] scan).
- Liver biopsy.
Treatment
- Remove or treat the underlying cause.
- Correct blood glucose if it is low.
- Correct any clotting abnormalities.
- Phototherapy may be helpful only if jaundice has a significant unconjugated component, e.g. Crigler–Najjar syndrome
- Treat any associated anaemia if due to haemolysis.
- Treat liver failure as appropriate.