Cholestatic jaundice early in life is infrequent but always abnormal and must be investigated because early diagnosis is crucial to identify treatable diseases, improving the outcome and quality of life of patients. Some diagnostic tools are efficient but expensive and frequently they are not available everywhere, making difficult to reach universally applicable algorithms. In fact, criteria available in the literature often depend on the facilities of the centres where studies are performed. This explains why until today clinical assessment and frequent clinical check-ups early in life remain being main diagnostic tools for a timely diagnosis and management. This is a narrative review updates the state of the art on neonatal cholestasis and based on the evidence gathered a diagnostic approach is proposed., which considers alternatives depending on the local capacities, emphasizing the need of early recognition of altered stools and conjugated bilirubin, and opportune referral to specialized centres.
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