Literature DB >> 16857001

Anaesthesia for liver transplantation in two infants with an organic acidaemia.

Diego Manzoni1, Angelica Spotti, Bruno Carrara, Paolo Gritti, Valter Sonzogni.   

Abstract

Liver transplantation is an accepted option of treatment in patients with inborn errors of metabolism limited to or mainly located into hepatocytes who have not responded well to medical treatment. Recurrent metabolic failure and neurological impairment might be an indication for early transplantation in patients with organic acidaemias. We discuss the anaesthetic management and metabolic implications of acidaemia in the first two cases of successfully treated propionic and methylmalonic acidaemia in Italy. A nine and 12 month follow up did not show any further metabolic failure after the procedure, indicating that early liver transplantation improves the quality of life of these patients.

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Year:  2006        PMID： 16857001     DOI： 10.1111/j.1399-3046.2006.00536.x

Source DB:  PubMed          Journal:  Pediatr Transplant        ISSN： 1397-3142


  5 in total

Review 1.  Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients.

Authors:  Yiouli P Ktena; Trygg Ramstad; Eva H Baker; Jennifer L Sloan; Andrew J Mannes; Irini Manoli; Charles P Venditti
Journal:  J Inherit Metab Dis       Date:  2015-05-19       Impact factor: 4.982

Review 2.  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

3.  Guidelines for the diagnosis and management of methylmalonic acidaemia and propionic acidaemia: First revision.

Authors:  Patrick Forny; Friederike Hörster; Diana Ballhausen; Anupam Chakrapani; Kimberly A Chapman; Carlo Dionisi-Vici; Marjorie Dixon; Sarah C Grünert; Stephanie Grunewald; Goknur Haliloglu; Michel Hochuli; Tomas Honzik; Daniela Karall; Diego Martinelli; Femke Molema; Jörn Oliver Sass; Sabine Scholl-Bürgi; Galit Tal; Monique Williams; Martina Huemer; Matthias R Baumgartner
Journal:  J Inherit Metab Dis       Date:  2021-03-09       Impact factor: 4.750

4.  Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia.

Authors:  Zhi-Gui Zeng; Guang-Peng Zhou; Lin Wei; Wei Qu; Ying Liu; Yu-Le Tan; Jun Wang; Li-Ying Sun; Zhi-Jun Zhu
Journal:  Orphanet J Rare Dis       Date:  2022-02-21       Impact factor: 4.123

Review 5.  Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia.

Authors:  Matthias R Baumgartner; Friederike Hörster; Carlo Dionisi-Vici; Goknur Haliloglu; Daniela Karall; Kimberly A Chapman; Martina Huemer; Michel Hochuli; Murielle Assoun; Diana Ballhausen; Alberto Burlina; Brian Fowler; Sarah C Grünert; Stephanie Grünewald; Tomas Honzik; Begoña Merinero; Celia Pérez-Cerdá; Sabine Scholl-Bürgi; Flemming Skovby; Frits Wijburg; Anita MacDonald; Diego Martinelli; Jörn Oliver Sass; Vassili Valayannopoulos; Anupam Chakrapani
Journal:  Orphanet J Rare Dis       Date:  2014-09-02       Impact factor: 4.123
  5 in total

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