Anesthetic Management of a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency
Patients with inborn errors of metabolism require special considerations in perioperative care. In the following case report, we describe the successful management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency, a deficit that causes a secondary carnitine deficiency and impaired β oxi...
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| Veröffentlicht in: | Anesthesia and analgesia 2008-08, Vol.107 (2), p.648-650 |
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| container_title | Anesthesia and analgesia |
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| creator | Robbins, Karen A. León-ruiz, Elias N. |
| description | Patients with inborn errors of metabolism require special considerations in perioperative care. In the following case report, we describe the successful management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency, a deficit that causes a secondary carnitine deficiency and impaired β oxidation. Patients may have significant underlying cardiomyopathy, and are at risk for metabolic decompensation, acidosis, and hypoglycemia during periods of stress. |
| doi_str_mv | 10.1213/ane.0b013e3181770fcc |
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In the following case report, we describe the successful management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency, a deficit that causes a secondary carnitine deficiency and impaired β oxidation. Patients may have significant underlying cardiomyopathy, and are at risk for metabolic decompensation, acidosis, and hypoglycemia during periods of stress.</description><subject>Adult</subject><subject>Amino Acid Metabolism, Inborn Errors - complications</subject><subject>Amino Acid Metabolism, Inborn Errors - diagnosis</subject><subject>Anesthesia</subject><subject>Anesthesia, General</subject><subject>Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy</subject><subject>Biological and medical sciences</subject><subject>Carbon-Carbon Ligases - deficiency</subject><subject>Cardiomyopathies - complications</subject><subject>Carnitine - deficiency</subject><subject>Female</subject><subject>Humans</subject><subject>Leucine - metabolism</subject><subject>Medical sciences</subject><issn>0003-2999</issn><issn>1526-7598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkNuKFDEQhoMo7rj6BiJ9o3dZK4dO0pfDrCfYRUG9Dkm6YrdmutdOhrHf3gw7uGCKUFTx_ZXUT8hLBleMM_HWTXgFHphAwQzTGmIIj8iGtVxR3XbmMdkAgKC867oL8iznn7VkYNRTcsGMEgKk3pCv2wlzGbCMobl1k_uBe5xKM8fGNV9cGU_FcSxDI-gtlmFNYZnLPK2J7uZts3OLn_-syWVsrjGOofJhfU6eRJcyvjjnS_L9_btvu4_05vOHT7vtDQ2SK0FRa99KaFsXg_ZGYWccM2h6QBmZkcpAJWLwfc_61rNQe5Fr7nyvJSgvLsmb-7l3y_z7UNew-zEHTKlaMx-yVZ1grRSqgvIerJ_PecFo75Zx75bVMrAnM21V2P_NrLJX5_kHv8f-QXR2rwKvz4DLwaW4uCmM-R_Hoa7HNTy8f5xTwSX_SocjLnZAl8pg4XRa0VEOYGoA0HqlEH8B0K-OWg</recordid><startdate>20080801</startdate><enddate>20080801</enddate><creator>Robbins, Karen A.</creator><creator>León-ruiz, Elias N.</creator><general>International Anesthesia Research Society</general><general>Lippincott</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20080801</creationdate><title>Anesthetic Management of a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency</title><author>Robbins, Karen A. ; León-ruiz, Elias N.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4263-e77b54055afc7b86e98a18e8d0e4f18468077bfcbdd1d5b1c184f272abd7406b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Adult</topic><topic>Amino Acid Metabolism, Inborn Errors - complications</topic><topic>Amino Acid Metabolism, Inborn Errors - diagnosis</topic><topic>Anesthesia</topic><topic>Anesthesia, General</topic><topic>Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy</topic><topic>Biological and medical sciences</topic><topic>Carbon-Carbon Ligases - deficiency</topic><topic>Cardiomyopathies - complications</topic><topic>Carnitine - deficiency</topic><topic>Female</topic><topic>Humans</topic><topic>Leucine - metabolism</topic><topic>Medical sciences</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Robbins, Karen A.</creatorcontrib><creatorcontrib>León-ruiz, Elias N.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Anesthesia and analgesia</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Robbins, Karen A.</au><au>León-ruiz, Elias N.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Anesthetic Management of a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency</atitle><jtitle>Anesthesia and analgesia</jtitle><addtitle>Anesth Analg</addtitle><date>2008-08-01</date><risdate>2008</risdate><volume>107</volume><issue>2</issue><spage>648</spage><epage>650</epage><pages>648-650</pages><issn>0003-2999</issn><eissn>1526-7598</eissn><coden>AACRAT</coden><abstract>Patients with inborn errors of metabolism require special considerations in perioperative care. In the following case report, we describe the successful management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency, a deficit that causes a secondary carnitine deficiency and impaired β oxidation. Patients may have significant underlying cardiomyopathy, and are at risk for metabolic decompensation, acidosis, and hypoglycemia during periods of stress.</abstract><cop>Hagerstown, MD</cop><pub>International Anesthesia Research Society</pub><pmid>18633047</pmid><doi>10.1213/ane.0b013e3181770fcc</doi><tpages>3</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Anesthesia and analgesia, 2008-08, Vol.107 (2), p.648-650 |
| issn | 0003-2999 1526-7598 |
| language | eng |
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| source | MEDLINE; Journals@Ovid LWW Legacy Archive; EZB-FREE-00999 freely available EZB journals |
| subjects | Adult Amino Acid Metabolism, Inborn Errors - complications Amino Acid Metabolism, Inborn Errors - diagnosis Anesthesia Anesthesia, General Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy Biological and medical sciences Carbon-Carbon Ligases - deficiency Cardiomyopathies - complications Carnitine - deficiency Female Humans Leucine - metabolism Medical sciences |
| title | Anesthetic Management of a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency |
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