Studies of Chronic Idiopathic Jaundice (Dubin-Johnson Syndrome)

A case of the Dubin-Johnson syndrome complicated by acute hepatitis

“…Several authors have noted marked variation in the amount of pigment between different patients, and indeed between affected members of the same family (Wolf et al, 1960;Arias, 1961;Butt, 1966). Our case demonstrates that Fig.…”

Nonmalformative Cardiovascular Pathology in Infants and Children

“…Studies of families suggest autosomal dominant inheritance with considerable variability of expression [121]. The heart may show extensive deposition of pigment within the myocardial cells.…”

Dublin-Johnson syndrome with some unusual features in a Chinese family.

“…Butt et al (1966) studied 128 of 242 members of a family in which the classical findings of DJS were present. Pigmentation of hepatic cells was found in only 29 of 39 members who submitted to liver biopsy, but there was no correlation between the amounts of liver pigment and the increases in SB or BSP retention.…”

“…The known coexistence of DJS and RS in one family (Arias, 1961) makes it likely that these disorders are variable manifestations of the same mutant gene, and the most generally held view has been that inheritance is in autosomal dominant fashion (Beker and Read, 1958;Mandema et al, 1960;Butt et al, 1966). However, while Butt et al (1966) favoured autosomal dominance they also suggested that the proband in their large family and perhaps one or more of her siblings may have been homozygous for the gene whereas all the other subclinically affected persons were heterozygotes.…”

“…However, while Butt et al (1966) favoured autosomal dominance they also suggested that the proband in their large family and perhaps one or more of her siblings may have been homozygous for the gene whereas all the other subclinically affected persons were heterozygotes. Pereira Lima et al (1966) concluded from a study of six generations of a family in which 3 members had RS, that an autosomal recessive gene was involved.…”