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Dubin- johnson syndrome (chronic idiopathic jaundice; rotor syndrome)  389


Dubin- Johnson Syndrome (Chronic Idiopathic Jaundice; Rotor Syndrome)     626i81g ;     626i81g ;     626i81g ;     626i81g ;     626i81g ;     626i81g ;     626i81g ;     626i81g ;     626i81g ;  389

General: Onset in infancy, but may be present at birth; autosomal dominant; liver cells are unable to excrete conjugated bilirubin; manifestations similar to Rotor syndrome, except that in the latter there is no melanin pigment present in the liver cells.

Ocular: Jaundice of sclera and conjunctiva in infancy.

Clinical: Abdominal pain right hypochondrium; nausea; vomiting; diarrhea; anorexia; weakness; hepatomegaly.

Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in liver cells: new clinico-pathologic entity with report of 12 cases. Medicine 1954; 33:155.

Rosenthal P. Neonatal Dubin- Johnson syndrome. J Pediatr Gastroenterol Nutr 1994; 19:255.

Wolkoff AW, et al. Inheritance of the Dubin- Johnson syndrome. N Engl J Med 1973; 288:113.




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