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Article
June 1980

Infantile Cholestatic Liver Disease

Author Affiliations

Genetic Counseling Center
Department of Pathology The Children's Mercy Hospital 24th at Gilham Rd Kansas City, MO 64108

Am J Dis Child. 1980;134(6):623. doi:10.1001/archpedi.1980.02130180079024
Abstract

Correspondence pertaining to material published in the Journal will be published, if found suitable, as space permits. Submit double-spaced copy clearly marked "For publication" and signed by all authors; references should conform to Journal format; maximum length: 500 words. Copyright assignment signed by all authors is required. The Editor reserves the right to edit such letters, which should be received within six weeks of publication of the Journal article in question.

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

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