PEDIATRICS Vol. 8 No. 5 November 1951, pp. 628-647
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ATRESIA OF THE INTRAHEPATIC BILE DUCTS

EDWARD H. AHRENS JR. M.D.1, RUTH C. HARRIS M.D.2, and H. EDWARD MACMAHON M.D.2

1 Senior Pediatric Fellow of The National Foundation for Infantile Paralysis., The Departments of the Hospital and Laboratories of the Rockefeller Institute for Medical Research, Babies Hospital of New York, and Tufts College Medical School, Boston.
2 The Departments of the Hospital and Laboratories of the Rockefeller Institute for Medical Research, Babies Hospital of New York, and Tufts College Medical School, Boston.

Case histories of four patients with atresia of the intrahepatic interlobular bile ducts are reviewed. Three of the four also had anomalies of the extrahepatic bile ducts. All had secondary biliary cirrhosis. The course of disease was distinguished by a relatively long life span, all patients surviving more than three years and one remaining alive at five years. Generalized skin xanthomatosis occurred in all patients, together with a marked and characteristic elevation of the serum lipids.

There was no indication on postmortem examination that intrahepatic bile ducts had developed and then become obliterated. The evidence presented in this study suggests that absence of interlobular bile ducts is a developmental anomaly, and that the embryologic development of biliary epithelium is normally independent of the growth of hepatic trabeculae.

Submitted on October 22, 1950




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