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Arteriohepatic Dysplasia: A Benign Syndrome of Intrahepatic Cholestasis with Multiple Organ Involvement

CAROLINE A. RIELY, M.D.; EDWARD COTLIER, M.D.; PAMELA S. JENSEN, M.D.; and GERALD KLATSKIN, M.D.
[+] Article and Author Information

Grant support: Clinical studies were done in the Yale Children's and Adult Clinical Research Centers, supported by Grant RR-125, GCRC Branch, Division of Research Resources, National Institutes of Health. Dr. Riely was a Teaching and Research Scholar of the American College of Physicians when these studies were conducted.

▸Requests for reprints should be addressed to Caroline A. Riely, M.D.; Liver Study Unit, Yale University School of Medicine; 333 Cedar Street; New Haven, CT 06510.


New Haven, Connecticut


© 1979 American College of PhysiciansAmerican College of Physicians


Ann Intern Med. 1979;91(4):520-527. doi:10.7326/0003-4819-91-4-520
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Arteriohepatic dysplasia (Alagille's syndrome) is presumed to be one of the familial intrahepatic cholestatic syndromes, all of which present with neonatal jaundice or failure to thrive, or both. We report the findings in five patients with this syndrome, four of whom have been followed into adulthood. In addition to hepatic dysfunction, patients had abnormalities of the cardiovascular system, eyes, bones, central nervous system, kidney, endocrine system, and habitus. Analysis of these cases allows a more complete characterization of this syndrome and shows that the cholestasis improves, although the abnormalities of the hands and face become more pronounced, with age. Patients with arteriohepatic dysplasia display the variability in expression seen in many autosomal-dominant conditions. New findings in the eye and spine provide markers specific for this syndrome and serve to differentiate it from other forms of cholestatic liver disease.

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