Biomaterial Database

Recurrence of the Budd-Chiari syndrome after orthotopic liver transplantation. 1999

J C Rückert, and R I Rückert, and B Rudolph, and J M Müller

Clinic of Surgery, Humboldt University Medical School (Charite) Berlin, Germany.

Obstruction of the hepatic venous outflow with or without involvement of the vena cava results in the Budd-Chiari syndrome (BCS). BCS may be limited to the liver but there is a variety of systemic disorders forming the etiology of BCS in the majority of cases. Surgery has a major impact on treatment of the BCS within a wide range of therapeutic strategies. The ultimate option of surgical management of the BCS is orthotopic liver transplantation (OLTx). The case of a patient with recurrent disease more than 5 years after OLTx for BCS due to paroxysmal nocturnal hemoglobinuria is analyzed with complete documentation. The literature is reviewed and the probable underlying causes for recurrent disease after OLTx for BCS are discussed including therapeutic consequences.

UI	MeSH Term	Description	Entries
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D011183	Postoperative Complications	Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery.	Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D012008	Recurrence	The return of a sign, symptom, or disease after a remission.	Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D005260	Female		Females
D005500	Follow-Up Studies	Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.	Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006457	Hemoglobinuria, Paroxysmal	A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.	Paroxysmal Cold Hemoglobinuria,Paroxysmal Nocturnal Hemoglobinuria,Marchiafava-Micheli Syndrome,Paroxysmal Hemoglobinuria,Paroxysmal Hemoglobinuria, Cold,Paroxysmal Hemoglobinuria, Nocturnal,Cold Paroxysmal Hemoglobinuria,Hemoglobinuria, Cold Paroxysmal,Hemoglobinuria, Nocturnal Paroxysmal,Hemoglobinuria, Paroxysmal Cold,Hemoglobinuria, Paroxysmal Nocturnal,Marchiafava Micheli Syndrome,Nocturnal Paroxysmal Hemoglobinuria,Syndrome, Marchiafava-Micheli
D006502	Budd-Chiari Syndrome	A condition in which the hepatic venous outflow is obstructed anywhere from the small HEPATIC VEINS to the junction of the INFERIOR VENA CAVA and the RIGHT ATRIUM. Usually the blockage is extrahepatic and caused by blood clots (THROMBUS) or fibrous webs. Parenchymal FIBROSIS is uncommon.	Chiari's Syndrome,Hepatic Venous Outflow Obstruction,Hepatic Vein Thrombosis,Budd Chiari Syndrome,Chiari Syndrome,Chiaris Syndrome,Hepatic Vein Thromboses,Thromboses, Hepatic Vein,Thrombosis, Hepatic Vein,Vein Thromboses, Hepatic,Vein Thrombosis, Hepatic
D006503	Hepatic Veins	Veins which drain the liver.	Hepatic Vein,Vein, Hepatic,Veins, Hepatic
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man