Search engine for discovering works of Art, research articles, and books related to Art and Culture
Javascript must be enabled to continue!
Recovery from a variegate porphyria by a liver transplantation
View through CrossRef
The porphyrias are a group of inherited or acquired enzymatic defects of heme biosynthesis. Each type of porphyria has a characteristic pattern of overproduction and accumulation of heme precursors based on the location of dysfunctional enzyme in the heme synthetic pathway. Variegate porphyria, one of the acute hepatic porphyrias, is characterized by a partial reduction in protoporphyrinogen oxidase, the seventh enzyme of the heme biosynthetic pathway. A case of liver transplantation is described with a recovery from a variegate porphyria. Acute porphyria is commonly worsened by a wide variety of medications. We describe a step-by-step perioperative management protocol. (Liver Transpl 2004;10:935-938.)
Ovid Technologies (Wolters Kluwer Health)
Title: Recovery from a variegate porphyria by a liver transplantation
Description:
The porphyrias are a group of inherited or acquired enzymatic defects of heme biosynthesis.
Each type of porphyria has a characteristic pattern of overproduction and accumulation of heme precursors based on the location of dysfunctional enzyme in the heme synthetic pathway.
Variegate porphyria, one of the acute hepatic porphyrias, is characterized by a partial reduction in protoporphyrinogen oxidase, the seventh enzyme of the heme biosynthetic pathway.
A case of liver transplantation is described with a recovery from a variegate porphyria.
Acute porphyria is commonly worsened by a wide variety of medications.
We describe a step-by-step perioperative management protocol.
(Liver Transpl 2004;10:935-938.
).
Related Results
The incidence of inherited porphyrias in Europe
The incidence of inherited porphyrias in Europe
AbstractRetrospective estimates of the prevalence of porphyrias have been reported but there has been no large scale prospective study of their incidence. The European Porphyria Ne...
The porphyrias
The porphyrias
Abstract
The porphyrias are a remarkable family of metabolic disorders characterized biochemically by overproduction of haem precursors, principally in the liver and...
Current therapeutic strategies for erectile function recovery after radical prostatectomy – literature review and meta-analysis
Current therapeutic strategies for erectile function recovery after radical prostatectomy – literature review and meta-analysis
Radical prostatectomy is the most commonly performed treatment option for localised prostate cancer. In the last decades the surgical technique has been improved and modified in or...
Modern Principles of Liver Fibrosis Diagnosis in Patients With Chronic Diffuse Liver Lesions: Literature Review and Clinical Case Description
Modern Principles of Liver Fibrosis Diagnosis in Patients With Chronic Diffuse Liver Lesions: Literature Review and Clinical Case Description
Introduction. Chronic diffuse liver lesions, which is at a certain stage and phase of the disease characterized by excessive accumulation of components of the extracellular matrix,...
Porphyria: A metabolic disorder
Porphyria: A metabolic disorder
Porphyrias are heterogenous group of metabolic disorders arising from defects in the heme biosynthetic pathway, each being characterized by a specific partial enzyme deficiency. Al...
Etiology and Outcome of Acute Liver Failure: Experience from a Liver Transplantation Centre in Montreal
Etiology and Outcome of Acute Liver Failure: Experience from a Liver Transplantation Centre in Montreal
BACKGROUND: Acute liver failure is a rare condition in which massive liver injury is associated with the rapid development of hepatic encephalopathy. Although viral hepatitis and d...
Key terms and definitions in acute porphyrias: Results of an international Delphi consensus led by the European porphyria network
Key terms and definitions in acute porphyrias: Results of an international Delphi consensus led by the European porphyria network
AbstractAcute porphyrias are a group of rare inherited disorders causing acute neurovisceral attacks. Many terms used frequently in the literature and clinical practice are ambiguo...
Psychiatric Disorder in Porphyria: A Case Series
Psychiatric Disorder in Porphyria: A Case Series
ABSTRACT
Porphyria is a group of rare genetic disorders that affect the heme production pathway due to specific enzyme abnormalities. These condi...