Literature DB >> 12111189

Progressive neurologic disability in methylmalonic acidemia despite transplantation of the liver.

William L Nyhan1, J Jay Gargus, Karen Boyle, Rick Selby, Richard Koch.   

Abstract

UNLABELLED: Methylmalonic acidemia unresponsive to cobalamin is often fatal in infancy. Patients have been considered candidates for hepatic transplantation and experience has been that the procedure eliminates the life-threatening episodes of ketoacidosis that characterize this disease.
CONCLUSION: experience with a 24-year-old patient treated with hepatic transplantation indicates that this procedure does not prevent progressive renal failure and neurologic dysfunction.

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Year:  2002        PMID: 12111189     DOI: 10.1007/s00431-002-0970-4

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  32 in total

Review 1.  Hepatocyte transplantation for inborn errors of metabolism.

Authors:  A B Burlina
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

2.  Renal transplantation in a boy with methylmalonic acidaemia.

Authors:  Joanna Clare Clothier; Anupam Chakrapani; Mary-Anne Preece; Patrick McKiernan; Rajat Gupta; Anita Macdonald; Sally-Anne Hulton
Journal:  J Inherit Metab Dis       Date:  2011-03-17       Impact factor: 4.982

3.  Correction of methylmalonic aciduria in vivo using a codon-optimized lentiviral vector.

Authors:  Edward S Y Wong; Chantelle McIntyre; Heidi L Peters; Enzo Ranieri; Donald S Anson; Janice M Fletcher
Journal:  Hum Gene Ther       Date:  2014-04-02       Impact factor: 5.695

4.  Liver-directed recombinant adeno-associated viral gene delivery rescues a lethal mouse model of methylmalonic acidemia and provides long-term phenotypic correction.

Authors:  Nuria Carrillo-Carrasco; Randy J Chandler; Suma Chandrasekaran; Charles P Venditti
Journal:  Hum Gene Ther       Date:  2010-09       Impact factor: 5.695

5.  A detailed analysis of methylmalonic acid kinetics during hemodialysis and after combined liver/kidney transplantation in a patient with mut (0) methylmalonic acidemia.

Authors:  Hilary J Vernon; C John Sperati; Joshua D King; Andrea Poretti; Neil R Miller; Jennifer L Sloan; Andrew M Cameron; Donna Myers; Charles P Venditti; David Valle
Journal:  J Inherit Metab Dis       Date:  2014-06-25       Impact factor: 4.982

Review 6.  Genetic and genomic systems to study methylmalonic acidemia.

Authors:  R J Chandler; C P Venditti
Journal:  Mol Genet Metab       Date:  2005-09-22       Impact factor: 4.797

7.  Successful Domino Liver Transplantation from a Patient with Methylmalonic Acidemia.

Authors:  A Khanna; R Gish; S C Winter; W L Nyhan; B A Barshop
Journal:  JIMD Rep       Date:  2015-07-29

Review 8.  Methylmalonic and propionic acidemias: clinical management update.

Authors:  Jamie L Fraser; Charles P Venditti
Journal:  Curr Opin Pediatr       Date:  2016-12       Impact factor: 2.856

Review 9.  Combined liver-kidney transplant for the management of methylmalonic aciduria: a case report and review of the literature.

Authors:  Peter J Mc Guire; Elizabeth Lim-Melia; George A Diaz; Kimiyo Raymond; Alexandra Larkin; Melissa P Wasserstein; Claude Sansaricq
Journal:  Mol Genet Metab       Date:  2007-10-26       Impact factor: 4.797

10.  Long-term rescue of a lethal murine model of methylmalonic acidemia using adeno-associated viral gene therapy.

Authors:  Randy J Chandler; Charles P Venditti
Journal:  Mol Ther       Date:  2009-10-27       Impact factor: 11.454

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