Hepatitis with Resulting Mobilization of Hepatic Pigment in a Patient with Dubin-Johnson Syndrome

Hunter, Fred M.; Sparks, Robert D.; Flinner, Robert L.

doi:10.1016/s0016-5085(19)34679-7

Cited by 24 publications

(5 citation statements)

References 7 publications

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“…Ehrlich and asso ciates (161) suggested that the Dubin-Johnson pigment has different staining characteristics and electron microscopic appearance from conventional lip ofuscin, showed its association with abnormal lysosomes and postulated a storage phenomenon. Consistent with this interpretation is the report of a patient with Dubin-Johnson syndrome who developed viral hepatitis at which time hepatic pigment w a s released from damaged parenchymal cells, engulfed by phagocytic cells, and slowly reaccumulated in regenerating par enchymal liver cells (162). The presence of normal lipofuscin in some livers is probably responsible for reports of hybrids of the variolls syndromes rep resented as cases of unconjugated hyperbilirubinemia with hepatic pigmen tation (163,164) and hepatic pigmentation without hyperbilirubinemia (165) Based on staining reactions and physical-chemical studies, Wegmann and associates (166) suggest that the pigment in Dubin-Johnson syndrome is an atypical melanin.…”

Section: Jaundice With Predominantly Conjugated Bilirubin In Serummentioning

confidence: 69%

Hepatic Aspects of Bilirubin Metabolism

Arias¹

1966

Annu. Rev. Med.

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Section: Jaundice With Predominantly Conjugated Bilirubin In Serummentioning

confidence: 69%

Hepatic Aspects of Bilirubin Metabolism

Arias¹

1966

Annu. Rev. Med.

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“…Loss from the liver cells of the typical pigment in patients with the Dubin-Johnson syndrome during acute viral hepatitis has been reported by Hunter (1964) and Masuda (1965). The patient reported by Hunter died four months after the attack of hepatitis, and at necropsy the centrilobular liver cells contained scanty pigment granules.…”

Section: Discussionmentioning

confidence: 70%

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

Varma¹,

Grainger²,

Scheuer³

1970

Gut

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“…Some variability in pigmentation may be due to occurance of coincidental disease such as acute viral hepatitis. These pigments vanish during the course of acute viral hepatitis infections while reappears at covalescence period (20). In chronic hepatitis, pigment granules may be reduced or even disappear gradually and also may develop deep jaundice in patients with DJS (21,22).…”

Section: Discussionmentioning

confidence: 99%

HBsAg-positive patient with Dubin-Johnson Syndrome: a case report

Kurt¹,

Haşimi²,

Öztürk³

et al. 2013

Gulhane Med J

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Dubin-Johnson Syndrome (DJS) is a rare autosomal recessive liver disorder characterized by chronic or intermittent conjugated, mild hyperbilirubinemia, and is caused by deficiency of multidrug resistance protein 2. DJS, is often an occult entity which until various factors accentuate hyperbilirubinemia, resulting in clinically apparent disease. In this study, we present a HBsAg-positive patient with DJS, who has been diagnosed based on elevated urinary coproporphyrin I isomer. The case was a 21-year-old male who complained about dizziness. His laboratory data revealed a chronic HBsAg sero-positivity and a mild conjugated/unconjugated hyperbilirubinemia; remaining analytical results were normal. Any pathological changes of liver and biliary tree were not observed on ultrasonography. Due to darkbrownish pigmentation demonstrated on liver biopsy reminded the DJS. Total porphyrin concentration in urine sample was within the reference range, diagnosis of DJS was confirmed by increased urine coproporphyrin I/total ratio (97%). Currently, DJS is diagnosed by demonstrating characteristic signs of the DJS on multiple diagnostic tests (e.g. histochemical examination of liver biopsy material and/or cholescintigraphic imaging). Although definitive diagnosis of this disorder mostly relies on ABCC2 gene mutation analysis, determination of urinary isomers of coproporphyrin may relatively be an easily accessible and strong biochemical indicator for diagnosis of DJS.

show abstract

Hepatitis with Resulting Mobilization of Hepatic Pigment in a Patient with Dubin-Johnson Syndrome

Cited by 24 publications

References 7 publications

Hepatic Aspects of Bilirubin Metabolism

Hepatic Aspects of Bilirubin Metabolism

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

HBsAg-positive patient with Dubin-Johnson Syndrome: a case report

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