Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder associated with deficiency of thymidine phosphorylase (TP). Associated manifestations include visual and hearing impairments, peripheral neuropathies, leukoencephalopathy, and malnutrition from concomita...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Anesthesiology |
| Online Access: | http://dx.doi.org/10.1155/2015/453714 |
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| author | Vianey Q. Casarez Acsa M. Zavala Pascal Owusu-Agyemang Katherine Hagan |
| author_facet | Vianey Q. Casarez Acsa M. Zavala Pascal Owusu-Agyemang Katherine Hagan |
| author_sort | Vianey Q. Casarez |
| collection | DOAJ |
| description | Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder associated with deficiency of thymidine phosphorylase (TP). Associated manifestations include visual and hearing impairments, peripheral neuropathies, leukoencephalopathy, and malnutrition from concomitant gastrointestinal dysmotility and pseudoobstruction. Given the altered metabolic state in these patients, specific consideration of medication selection is advised. This case report will describe the anesthetic management used in a 10-year-old girl with MNGIE. She had multiple anesthetics while undergoing allogeneic hematopoietic stem cell transplantation. This case report will discuss the successful repeated use of the same anesthetic in this pediatric patient, with the avoidance of volatile anesthetic agents, propofol, and muscle relaxant. |
| format | Article |
| id | doaj-art-e27a562256fb4dbdad9de7c563fc1a92 |
| institution | Kabale University |
| issn | 2090-6382 2090-6390 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Anesthesiology |
| spelling | doaj-art-e27a562256fb4dbdad9de7c563fc1a922025-02-03T05:52:32ZengWileyCase Reports in Anesthesiology2090-63822090-63902015-01-01201510.1155/2015/453714453714Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal EncephalopathyVianey Q. Casarez0Acsa M. Zavala1Pascal Owusu-Agyemang2Katherine Hagan3Department of Anesthesiology & Perioperative Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 409, Houston, TX 77030, USADepartment of Anesthesiology & Perioperative Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 409, Houston, TX 77030, USADepartment of Anesthesiology & Perioperative Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 409, Houston, TX 77030, USADepartment of Anesthesiology & Perioperative Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 409, Houston, TX 77030, USAMitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder associated with deficiency of thymidine phosphorylase (TP). Associated manifestations include visual and hearing impairments, peripheral neuropathies, leukoencephalopathy, and malnutrition from concomitant gastrointestinal dysmotility and pseudoobstruction. Given the altered metabolic state in these patients, specific consideration of medication selection is advised. This case report will describe the anesthetic management used in a 10-year-old girl with MNGIE. She had multiple anesthetics while undergoing allogeneic hematopoietic stem cell transplantation. This case report will discuss the successful repeated use of the same anesthetic in this pediatric patient, with the avoidance of volatile anesthetic agents, propofol, and muscle relaxant.http://dx.doi.org/10.1155/2015/453714 |
| spellingShingle | Vianey Q. Casarez Acsa M. Zavala Pascal Owusu-Agyemang Katherine Hagan Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy Case Reports in Anesthesiology |
| title | Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy |
| title_full | Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy |
| title_fullStr | Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy |
| title_full_unstemmed | Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy |
| title_short | Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy |
| title_sort | anesthetic management of a child with mitochondrial neurogastrointestinal encephalopathy |
| url | http://dx.doi.org/10.1155/2015/453714 |
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