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. 2022 Feb 11;9(2):243. doi: 10.3390/children9020243
Key Message Box
Suspect CPSS in patients with a constellation of seemingly unrelated symptoms.
Liver Doppler US is the first essential step for diagnosis.
Identification of a CPSS does not obviate the need for a full cholestasis work-up.
Hyperammonemia of unexplained etiology in a neonate or child should prompt screening for CPSS.