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Sir.—We read with interest the report by Rosenthal et al (Journal 133:1195-1196, 1979) concerning infantile cholestatic liver disease associated with alpha1-antitrypsin deficiency and the Pi-SZ phenotype. We have seen a similar patient who at 1 month of age had failure to thrive and conjugated hyperbilirubinemia. The only other clinical abnormality was a cholesterol level of 415 mg/dL. Rose bengal sodium I 131 did not enter the intestine.
An operative cholangiogram and open biopsy were performed before the