A single‐center experience with liver transplantation for Wilson’s disease
Identifieur interne : 000E76 ( Main/Exploration ); précédent : 000E75; suivant : 000E77A single‐center experience with liver transplantation for Wilson’s disease
Auteurs : Adrian P. Martin [Allemagne] ; Michael Bartels [Allemagne] ; Jens Redlich [Allemagne] ; Johann Hauss [Allemagne] ; Josef Fangmann [Allemagne]Source :
- Clinical Transplantation [ 0902-0063 ] ; 2008-03.
English descriptors
Abstract
Abstract: Wilson’s disease is an inherited disorder of copper metabolism, presenting with prominent hepatic and neurologic manifestations. There is an established place for liver transplantation in the presence of liver disease, while the indication for neurologic manifestations is debated. Between 1993 and 2005, 11 patients were liver transplanted for Wilson’s disease at our institution. We retrospectively reviewed the medical records of the patients. The pathology of the explanted livers was analyzed. The patients were divided into three groups based on the evolution of the disease. Postoperative data gathered included patient and graft outcome, complications, neurologic status, and copper metabolism. Six males and five females were transplanted at a mean age of 29.7 yr (range 15–48 yr). Three patients had a fulminant presentation, two patients had decompensation of established disease, and six patients had chronic disease. Neurologic features were prominent in five patients. The pathologic analysis of the explanted graft showed cirrhosis in all patients. The five patients with fulminant and acute on chronic presentations also showed necrosis in the explant. The mean postoperative follow‐up was 56.8 months (range 10–129 months). Two patients were re‐transplanted. One patient died because of severe sepsis. Two patients with severe neurologic dysfunction showed significant remission of symptoms. Liver transplantation is a safe and effective treatment for both acute and chronic presentations of Wilson’s disease. Acute presentation correlates with the presence of necrosis in the explanted liver. In our series, there was a relevant improvement of the neurologic features after transplantation.
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DOI: 10.1111/j.1399-0012.2007.00777.x
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There is an established place for liver transplantation in the presence of liver disease, while the indication for neurologic manifestations is debated. Between 1993 and 2005, 11 patients were liver transplanted for Wilson’s disease at our institution. We retrospectively reviewed the medical records of the patients. The pathology of the explanted livers was analyzed. The patients were divided into three groups based on the evolution of the disease. Postoperative data gathered included patient and graft outcome, complications, neurologic status, and copper metabolism. Six males and five females were transplanted at a mean age of 29.7 yr (range 15–48 yr). Three patients had a fulminant presentation, two patients had decompensation of established disease, and six patients had chronic disease. Neurologic features were prominent in five patients. The pathologic analysis of the explanted graft showed cirrhosis in all patients. The five patients with fulminant and acute on chronic presentations also showed necrosis in the explant. The mean postoperative follow‐up was 56.8 months (range 10–129 months). Two patients were re‐transplanted. One patient died because of severe sepsis. Two patients with severe neurologic dysfunction showed significant remission of symptoms. Liver transplantation is a safe and effective treatment for both acute and chronic presentations of Wilson’s disease. Acute presentation correlates with the presence of necrosis in the explanted liver. In our series, there was a relevant improvement of the neurologic features after transplantation.</div></front></TEI><affiliations><list><country><li>Allemagne</li></country><region><li>District de Leipzig</li><li>Saxe (Land)</li></region><settlement><li>Leipzig</li></settlement></list><tree><country name="Allemagne"><region name="Saxe (Land)"><name sortKey="Martin, Adrian P" sort="Martin, Adrian P" uniqKey="Martin A" first="Adrian P." last="Martin">Adrian P. Martin</name></region><name sortKey="Bartels, Michael" sort="Bartels, Michael" uniqKey="Bartels M" first="Michael" last="Bartels">Michael Bartels</name><name sortKey="Fangmann, Josef" sort="Fangmann, Josef" uniqKey="Fangmann J" first="Josef" last="Fangmann">Josef Fangmann</name><name sortKey="Hauss, Johann" sort="Hauss, Johann" uniqKey="Hauss J" first="Johann" last="Hauss">Johann Hauss</name><name sortKey="Redlich, Jens" sort="Redlich, Jens" uniqKey="Redlich J" first="Jens" last="Redlich">Jens Redlich</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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