Anaesthetic considerations for liver transplantation in propionic acidemia
Propionic acidemia (PA) is an autosomal recessive disorder of metabolism due to deficiency of the enzyme propionyl-CoA carboxylase (PCC) that converts propionyl-CoA to methylmalonyl-CoA with the help of the cofactor biotin inside the mitochondria. The resultant accumulation of propionyl-CoA causes s...
| 發表在: | Indian Journal of Anaesthesia |
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| Main Authors: | , , , |
| 格式: | Article |
| 語言: | 英语 |
| 出版: |
Wolters Kluwer Medknow Publications
2016-01-01
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| 主題: | |
| 在線閱讀: | http://www.ijaweb.org/article.asp?issn=0019-5049;year=2016;volume=60;issue=1;spage=50;epage=54;aulast=Rajakumar |
| _version_ | 1851907835760214016 |
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| author | Akila Rajakumar Ilankumaran Kaliamoorthy Mettu Srinivas Reddy Mohamed Rela |
| author_facet | Akila Rajakumar Ilankumaran Kaliamoorthy Mettu Srinivas Reddy Mohamed Rela |
| author_sort | Akila Rajakumar |
| collection | DOAJ |
| container_title | Indian Journal of Anaesthesia |
| description | Propionic acidemia (PA) is an autosomal recessive disorder of metabolism due to deficiency of the enzyme propionyl-CoA carboxylase (PCC) that converts propionyl-CoA to methylmalonyl-CoA with the help of the cofactor biotin inside the mitochondria. The resultant accumulation of propionyl-CoA causes severe hyperammonaemia and life-threatening metabolic acidosis. Based on the positive outcomes, liver transplantation is now recommended for individuals with recurrent episodes of hyperammonaemia or acidosis that is not adequately controlled with appropriate medical therapies. We report anaesthetic management of two children with PA for liver transplantation at our institution. It is essential for the anaesthesiologist, caring for these individuals to be familiar with the manifestations of the disease, the triggers for decompensation and management of an acute episode. |
| format | Article |
| id | doaj-art-e59b8a612e864d04a1e5a531f0b183c2 |
| institution | Directory of Open Access Journals |
| issn | 0019-5049 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| spelling | doaj-art-e59b8a612e864d04a1e5a531f0b183c22025-08-19T22:03:08ZengWolters Kluwer Medknow PublicationsIndian Journal of Anaesthesia0019-50492016-01-01601505410.4103/0019-5049.174799Anaesthetic considerations for liver transplantation in propionic acidemiaAkila RajakumarIlankumaran KaliamoorthyMettu Srinivas ReddyMohamed RelaPropionic acidemia (PA) is an autosomal recessive disorder of metabolism due to deficiency of the enzyme propionyl-CoA carboxylase (PCC) that converts propionyl-CoA to methylmalonyl-CoA with the help of the cofactor biotin inside the mitochondria. The resultant accumulation of propionyl-CoA causes severe hyperammonaemia and life-threatening metabolic acidosis. Based on the positive outcomes, liver transplantation is now recommended for individuals with recurrent episodes of hyperammonaemia or acidosis that is not adequately controlled with appropriate medical therapies. We report anaesthetic management of two children with PA for liver transplantation at our institution. It is essential for the anaesthesiologist, caring for these individuals to be familiar with the manifestations of the disease, the triggers for decompensation and management of an acute episode.http://www.ijaweb.org/article.asp?issn=0019-5049;year=2016;volume=60;issue=1;spage=50;epage=54;aulast=RajakumarHyperammonaemic crisis, inborn metabolic errors, liver transplantation, organic acidemia, propionic acidemia and anaesthesia |
| spellingShingle | Akila Rajakumar Ilankumaran Kaliamoorthy Mettu Srinivas Reddy Mohamed Rela Anaesthetic considerations for liver transplantation in propionic acidemia Hyperammonaemic crisis, inborn metabolic errors, liver transplantation, organic acidemia, propionic acidemia and anaesthesia |
| title | Anaesthetic considerations for liver transplantation in propionic acidemia |
| title_full | Anaesthetic considerations for liver transplantation in propionic acidemia |
| title_fullStr | Anaesthetic considerations for liver transplantation in propionic acidemia |
| title_full_unstemmed | Anaesthetic considerations for liver transplantation in propionic acidemia |
| title_short | Anaesthetic considerations for liver transplantation in propionic acidemia |
| title_sort | anaesthetic considerations for liver transplantation in propionic acidemia |
| topic | Hyperammonaemic crisis, inborn metabolic errors, liver transplantation, organic acidemia, propionic acidemia and anaesthesia |
| url | http://www.ijaweb.org/article.asp?issn=0019-5049;year=2016;volume=60;issue=1;spage=50;epage=54;aulast=Rajakumar |
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