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Dubin-Johnson Syndrome Presenting During Cardiac Transplantation Evaluation

Dubin-Johnson syndrome is a rare, benign disorder that results in conjugated hyperbilirubinemia. The disease manifests as intermittent jaundice without long-term hepatic or other clinical complications. This article reports a case of Dubin-Johnson syndrome, which was identified during cardiac transp...

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Detalles Bibliográficos
Autores principales: LeVee, Alexis, Cooper, Craig, Russell, Michael B, Sterling, Mark
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7003716/
https://www.ncbi.nlm.nih.gov/pubmed/32064176
http://dx.doi.org/10.7759/cureus.6594
Descripción
Sumario:Dubin-Johnson syndrome is a rare, benign disorder that results in conjugated hyperbilirubinemia. The disease manifests as intermittent jaundice without long-term hepatic or other clinical complications. This article reports a case of Dubin-Johnson syndrome, which was identified during cardiac transplant evaluation for cardiomyopathy secondary to a polyglycogen storage disease. The patient successfully underwent an orthotopic heart transplant. Postoperatively, her conjugated hyperbilirubinemia increased as compared to her baseline but resolved after several weeks. This report briefly reviews the hepatic manifestations in patients with Dubin-Johnson syndrome undergoing major surgery and highlights urinary coproporphyrin as a useful diagnostic test for Dubin-Johnson syndrome.