August 1980

Infantile Cholestatic Liver Disease-Reply

Author Affiliations

Division of Gastroenterology Department of Pediatrics University of California School of Medicine San Francisco, CA 94143

Am J Dis Child. 1980;134(8):802. doi:10.1001/archpedi.1980.02130200069027

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In Reply.—We are gratified that Drs Harris and Beatty have seen a case that resembles the one reported by us. It was our intent to point out that PAS-positive, diastase-resistant granules may be observed during the first two months in periportal hepatocytes and in Kupffer cells of patients heterozygous for alpha-1-antitrypsin deficiency. As indicated in our article, examination with the electron microscope demonstrated that these deposits were located in the rough endoplasmic reticulum of hepatocytes. In contrast, in patients with cholestasis due to other causes, PAS-positive, diastase-resistant granules may be seen in damaged hepatocytes not usually confined to peripheral regions of lobules, and concentrated in lysosomes rather than in expanded regions of the rough endoplasmic reticulum. The deposits of lipofuscin-melanin pigment observed in Dubin-Johnson syndrome, to which Drs Harris and Beatty refer, are panlobular in distribution and have a dark brown color that may be readily distinguished from the