Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia
Background Pyruvate dehydrogenase complex (PDHC) deficiency is a rare mitochondrial disorder caused by a genetic mutation affecting the activity of the PDHC enzyme, which plays a major role in the tricarboxylic cycle. Few cases of surgery or anesthesia have been reported. Moreover, there is no recom...
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| Format: | Article |
| Language: | English |
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Korean Society of Anesthesiologists
2023-07-01
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| Series: | Anesthesia and Pain Medicine |
| Subjects: | |
| Online Access: | http://www.anesth-pain-med.org/upload/pdf/apm-23034.pdf |
| _version_ | 1827824953355927552 |
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| author | Won Yong Lim Hyeon-Jeong Lee Eun Ji Park Soeun Jeon Wangseok Do Hyae Jin Kim Dowon Lee Jeong-Min Hong |
| author_facet | Won Yong Lim Hyeon-Jeong Lee Eun Ji Park Soeun Jeon Wangseok Do Hyae Jin Kim Dowon Lee Jeong-Min Hong |
| author_sort | Won Yong Lim |
| collection | DOAJ |
| description | Background Pyruvate dehydrogenase complex (PDHC) deficiency is a rare mitochondrial disorder caused by a genetic mutation affecting the activity of the PDHC enzyme, which plays a major role in the tricarboxylic cycle. Few cases of surgery or anesthesia have been reported. Moreover, there is no recommended anesthetic method. Case A 24-month-old child with a PDHC deficiency presented to the emergency room with respiratory failure, mental decline, systemic cyanosis, and lactic acidosis. During hospitalization period, the patient presented with pneumothorax, pneumoperitoneum, and multiple air pockets in the heart. Two surgeries were performed under general anesthesia using an inhalational anesthetic agent. The patient was discharged with home ventilation. Conclusions Anesthesiologists should be wary of multiple factors when administering anesthesia to patients with PDHC deficiency, including airway abnormalities, acid-base imbalance, intraoperative fluid management, selection of appropriate anesthetics, and monitoring of lactic acid levels. |
| first_indexed | 2024-03-12T02:33:40Z |
| format | Article |
| id | doaj.art-4e256e4273cc43a784b8be79af94583f |
| institution | Directory Open Access Journal |
| issn | 1975-5171 2383-7977 |
| language | English |
| last_indexed | 2024-03-12T02:33:40Z |
| publishDate | 2023-07-01 |
| publisher | Korean Society of Anesthesiologists |
| record_format | Article |
| series | Anesthesia and Pain Medicine |
| spelling | doaj.art-4e256e4273cc43a784b8be79af94583f2023-09-05T01:46:22ZengKorean Society of AnesthesiologistsAnesthesia and Pain Medicine1975-51712383-79772023-07-0118327027410.17085/apm.230341204Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesiaWon Yong Lim0Hyeon-Jeong Lee1Eun Ji Park2Soeun Jeon3Wangseok Do4Hyae Jin Kim5Dowon Lee6Jeong-Min Hong7 Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea Department of Anesthesiology and Pain Medicine, School of Dentistry, Kyungpook National University, Daegu, Korea Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, KoreaBackground Pyruvate dehydrogenase complex (PDHC) deficiency is a rare mitochondrial disorder caused by a genetic mutation affecting the activity of the PDHC enzyme, which plays a major role in the tricarboxylic cycle. Few cases of surgery or anesthesia have been reported. Moreover, there is no recommended anesthetic method. Case A 24-month-old child with a PDHC deficiency presented to the emergency room with respiratory failure, mental decline, systemic cyanosis, and lactic acidosis. During hospitalization period, the patient presented with pneumothorax, pneumoperitoneum, and multiple air pockets in the heart. Two surgeries were performed under general anesthesia using an inhalational anesthetic agent. The patient was discharged with home ventilation. Conclusions Anesthesiologists should be wary of multiple factors when administering anesthesia to patients with PDHC deficiency, including airway abnormalities, acid-base imbalance, intraoperative fluid management, selection of appropriate anesthetics, and monitoring of lactic acid levels.http://www.anesth-pain-med.org/upload/pdf/apm-23034.pdfacid-base imbalanceanesthesia, inhalationlactic acidosismitochondrial diseasepyruvate dehydrogenase complex deficiency disease |
| spellingShingle | Won Yong Lim Hyeon-Jeong Lee Eun Ji Park Soeun Jeon Wangseok Do Hyae Jin Kim Dowon Lee Jeong-Min Hong Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia Anesthesia and Pain Medicine acid-base imbalance anesthesia, inhalation lactic acidosis mitochondrial disease pyruvate dehydrogenase complex deficiency disease |
| title | Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia |
| title_full | Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia |
| title_fullStr | Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia |
| title_full_unstemmed | Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia |
| title_short | Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia |
| title_sort | perioperative considerations of pyruvate dehydrogenase complex deficiency a case report of two consecutive anesthesia |
| topic | acid-base imbalance anesthesia, inhalation lactic acidosis mitochondrial disease pyruvate dehydrogenase complex deficiency disease |
| url | http://www.anesth-pain-med.org/upload/pdf/apm-23034.pdf |
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