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05). TNC patients (17/23) more often than PFIC patients (20/42, P=.041) harboured biallelic non-null variants (predicted not to terminate translation prematurely). TNC patient livers (7/7) more often than PFIC patient livers (5/16, P=.005) expressed immunohistochemically detectable BSEP. Kaplan-Meier analysis showed better prognosis for patients with BSEP expression (P=.009). Too few BRIC patients were available for statistical study. Neonatal cholestasis associated with biallelic PPV in ABCB11 can resolve temporarily or persistently in