Differentials
Dubin-Johnson syndrome
SIGNS / SYMPTOMS
Clinically similar, no differentiating symptoms or signs.
INVESTIGATIONS
Liver pigmentation present. In RS, the liver has a normal appearance.
Failure to visualise the gall bladder on oral cholecystography.[4]
Total urinary coproporphyrin excretion is either normal or moderately increased, but >80% is excreted as coproporphyrin I.[4][8] In RS, the proportion is much lower at approximately 65%.
Gilbert's syndrome
Crigler-Najjar syndrome (type I and II)
SIGNS / SYMPTOMS
Diagnosed usually during the neonatal period.
More intense jaundice.
INVESTIGATIONS
Presence of unconjugated hyperbilirubinaemia in serum and urine.[17]
Extra-hepatic biliary obstruction
SIGNS / SYMPTOMS
Pruritus.
Acholic stools.
INVESTIGATIONS
Elevated alkaline phosphatase.
Elevated gamma-GT.
Familial intra-hepatic cholestasis
SIGNS / SYMPTOMS
Pruritus.
Age usually <1 year.
INVESTIGATIONS
Elevated bile acids.
Abnormal LFTs.
Benign recurrent intra-hepatic cholestasis
SIGNS / SYMPTOMS
Recurrent attacks of cholestasis lasting several days to months.
Each episode preceded by intense pruritus and malaise, lassitude, and sometimes diarrhoea.
Pale stools during the episode.
Weight loss due to steatorrhoea.
INVESTIGATIONS
LFTs during an episode reflect cholestasis with elevated serum bile acids and alkaline phosphatase.
Biopsy specimens during an attack show bile plugs within the ducts.
Between attacks liver histology is normal apart from mild portal zone fibrosis.[23]
Drug-induced hepatotoxicity
SIGNS / SYMPTOMS
History of exposure to a known hepatotoxic agent or drug causing cholestasis (e.g., isoniazid, halothane, alpha-methyl dopa, erythromycin, chlorpromazine, oestrogens/oral contraceptives, ciclosporin, haloperidol).
INVESTIGATIONS
Abnormal LFTs.
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