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Lian X, Jing Y, Luo T, Guo Y, Lin Y. Pharmacological interventions for the management of anesthesia and sedation in patients with Duchenne muscular dystrophy: a systematic review and meta-analysis. Front Med (Lausanne) 2025; 12:1497538. [PMID: 39931560 PMCID: PMC11808030 DOI: 10.3389/fmed.2025.1497538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2024] [Accepted: 01/06/2025] [Indexed: 02/13/2025] Open
Abstract
Background Patients with duchenne muscular dystrophy (DMD) have an increased risk of complications when they undergo sedation or general anesthesia. However, due to improvements in cardiopulmonary therapies during anesthetic care, patients with DMD are experiencing an unprecedented duration of survival. We performed a systematic analysis to assess the benefits and risks of pharmacological interventions for the management of anesthesia and sedation in DMD patients. Methods We included any type of study reporting any drug intervention to manage anesthesia and sedation in participants previously diagnosed with DMD. Our primary outcomes were the onset time, recovery time, and neurodevelopmental disabilities. Seven electronic databases and three clinical trial registry platforms were searched. Data from the eligible studies were combined to calculate pooled risk ratios or standardized mean differences, and some included studies are presented in a narrative synthesis. Results Forty studies with 196 DMD participants were included in the analysis. Compared with those of the control group, the sensitivity of patients with DMD to neuromuscular blocking agents (NMBAs) may have resulted in a prolonged onset time [MD = -0.96, 95% CI (0.71, 2.60), I 2 = 33%, P < 0.0001] and recovery time [MD = 2.22, 95% CI (1.14, 3.30), I 2 = 76%, P < 0.0001] from anesthesia. The neuromuscular blocking effects showed a significant age dependence in DMD patients, and the safe use of 2 mg/kg sugammadex to antagonize deep neuromuscular blockade and rapid recovery has been reported. Furthermore, DMD patients are at risk of developing malignant hyperpyrexia with general/inhaled anesthesia, and dantrolene is often used for effective rescue. In addition, general anesthesia and central neuraxial blockade in patients with severe DMD are unsafe because respiratory depression and myocardial complications may occur after the administration of volatile anesthetics and depolarizing muscle relaxants (succinylcholine) during the induction of anesthesia. Conclusions Patients with DMD are more sensitive to NMBAs with delayed onset times and prolonged recovery times. Precautions for DMD patients should include quantitative neuromuscular monitoring, electrocardiographic monitoring and rapid airway protection throughout anesthesia. Compared with general anesthesia, regional anesthesia may be a relatively safe option.
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Affiliation(s)
- Xianghong Lian
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Yang Jing
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Ting Luo
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Yixin Guo
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Yunzhu Lin
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
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Kotha R, Jones A, Girgenti GT, Nahrwold DA. Anesthetic Management of a Patient With McArdle Disease: A Case Report and Review of the Literature. Cureus 2023; 15:e40092. [PMID: 37425602 PMCID: PMC10328145 DOI: 10.7759/cureus.40092] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/07/2023] [Indexed: 07/11/2023] Open
Abstract
McArdle disease (glycogen storage disorder type V) is a rare inherited condition resulting in impaired energy metabolism. Challenges in anesthetized patients with McArdle disease include hypoglycemia, rhabdomyolysis, myoglobinuria, acute renal failure, and postoperative fatigue. We review the literature and discuss a successful anesthetic that had no perioperative complications for a patient with McArdle disease undergoing robotic-assisted lung wedge resection. Preoperatively, we obtained a complete blood count, chemistry panel, and creatine kinase level. Intraoperatively, we proceeded with general endotracheal anesthesia and monitored point-of-care electrolytes, hemoglobin, and blood glucose. The patient had an uneventful postoperative recovery and was discharged home on postoperative Day 3. Patients with McArdle disease can undergo safe surgery with appropriate perioperative planning and a well-managed anesthetic. Efforts should focus on mitigating the risks of hypoglycemia, rhabdomyolysis, myoglobinuria, acute renal failure, and postoperative fatigue.
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Affiliation(s)
- Rohini Kotha
- Anesthesiology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, USA
| | - Anastasia Jones
- Anesthesiology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, USA
- Anesthesiology, University of Florida College of Medicine, Gainesville, USA
| | - Gregory T Girgenti
- Anesthesiology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, USA
| | - Daniel A Nahrwold
- Anesthesiology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, USA
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Management of patients susceptible to malignant hyperthermia: A surgeon's perspective. Int J Pediatr Otorhinolaryngol 2022; 159:111187. [PMID: 35660936 DOI: 10.1016/j.ijporl.2022.111187] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/01/2022] [Revised: 03/28/2022] [Accepted: 05/21/2022] [Indexed: 11/22/2022]
Abstract
OBJECTIVES Malignant hyperthermia (MH) susceptibility caries broad implications for the care of pediatric surgical patients. While precautions must often be taken for only a vague family history, two options exist to assess MH-susceptibility. We evaluate the use of MH precautions and susceptibility testing at a freestanding children's hospital. METHODS This single institution retrospective cohort study identified patients of any age who received general anesthetics utilizing MH precautions over a five-year period. The electronic medical record was further queried for patients diagnosed with MH. The indication for MH precautions and uses of susceptibility testing are assessed. Secondary outcomes included a diagnosis of bona fide MH. RESULTS A total of 125 patients received 174 anesthetics with MH precautions at a mean age of 114 months (0-363 months). Otolaryngology was the procedural service most frequently involved in the care of the cohort (n = 45; 26%). A reported personal or family history of MH (n = 102; 59%) was the most common indication for precautions, followed by muscular dystrophy (n = 29; 17%). No MH events occurred in the cohort and further review of ICD-9 and -10 diagnosis codes found no MH diagnoses. No study subjects received muscle biopsy and contracture testing and only 5 (4%) underwent genetic testing for genomic variants known to cause MH susceptibility. A case example is given to highlight the implications of a reported MH history. CONCLUSION Otolaryngologists should maintain a familiarity with the precautions necessary to manage patients at risk for MH and MH-like reactions. Without an accessible test to rule out susceptibility, surgeons must rely on a careful history to appropriately utilize precautions. An inappropriate label of "MH-susceptible" may result in decreased access to care and treatment delays.
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Becker muscular dystrophy: case report, review of the literature, and analysis of differentially expressed hub genes. Neurol Sci 2021; 43:243-253. [PMID: 34731335 DOI: 10.1007/s10072-021-05499-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2021] [Accepted: 07/21/2021] [Indexed: 10/19/2022]
Abstract
INTRODUCTION Becker muscular dystrophy (BMD) is a genetic and progressive neuromuscular disease caused by mutations in the dystrophin gene with no available cure. A case report and comprehensive review of BMD cases aim to provide important clues for early diagnosis and implications for clinical practice. Genes and pathways identified from microarray data of muscle samples from patients with BMD help uncover the potential mechanism and provide novel therapeutic targets for dystrophin-deficient muscular dystrophies. METHODS We describe a BMD family with a 10-year-old boy as the proband and reviewed BMD cases from PubMed. Datasets from the Gene Expression Omnibus database were downloaded and integrated with the online software. RESULTS The systematic review revealed the clinical manifestations and mutation points of the dystrophin gene. Gene ontology analysis showed that extracellular matrix organization and extracellular structure organization with enrichment of upregulated genes coexist in three datasets. We present the first report of TUBA1A involvement in the development of BMD/Duchenne muscular dystrophy (DMD). DISCUSSION This study provides important implications for clinical practice, uncovering the potential mechanism of the progress of BMD/DMD, and provided new therapeutic targets.
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Peng L, Wei W. Anesthesia management in a pediatric patient with Becker muscular dystrophy undergoing laparoscopic surgery: A case report. World J Clin Cases 2021; 9:8852-8857. [PMID: 34734066 PMCID: PMC8546828 DOI: 10.12998/wjcc.v9.i29.8852] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/18/2021] [Revised: 06/22/2021] [Accepted: 08/09/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Patients with Becker muscular dystrophy (BMD) have a high risk of developing hyperkalemia, rhabdomyolysis, and malignant hyperthermia when exposed to volatile anesthetics and depolarizing muscle relaxants. Patients with BMD are also prone to respiratory depression after general anesthesia. Thus, it is extremely challenging for anesthesiologists to manage anesthesia in BMD patients, particularly in pediatric BMD patients. Here, we present successful anesthesia management using transversus abdominis plane block (TAPB) combined with total intravenous anesthesia (TIVA) in a pediatric BMD patient undergoing laparoscopic inguinal hernia repair.
CASE SUMMARY A 2-year-old boy, weighing 15 kg, with BMD, was scheduled for laparoscopic inguinal hernia repair. TIVA was used for induction, and continuous infusions of short-acting intravenous anesthetics combined with TAPB were performed for anesthesia maintenance. Moreover, TAPB provided good postoperative analgesia. The patient underwent uneventful surgery and anesthesia, and over the 17 mo follow-up period showed no anesthesia-induced complications.
CONCLUSION TAPB combined with TIVA, using short-acting intravenous anesthetic agents, can provide safe and effective anesthesia management in pediatric BMD patients undergoing short-term abdominal surgery.
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Affiliation(s)
- Ling Peng
- Department of Anaesthesiology, West China Hospital of Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Wei Wei
- Department of Anaesthesiology, West China Hospital of Sichuan University, Chengdu 610041, Sichuan Province, China
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Zhou SY, Wang D, Liu C, Zhang S, Shan BL, Ma HC. Laparoscopic gynecological surgery in an adult woman with Becker muscular dystrophy performed with sevoflurane with cisatracurium anesthesia: A case report. Medicine (Baltimore) 2020; 99:e19733. [PMID: 32311965 PMCID: PMC7220258 DOI: 10.1097/md.0000000000019733] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
RATIONALE Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) are progressive neuromuscular disorders caused by mutations in the dystrophin gene. The management of anesthesia in patients with BMD is complicated because they are highly sensitive to the conventional anesthetics such as volatile anesthetics and muscle relaxants. It is reported that anesthesia in patients with DMD is associated with several complications. However, a few case reports have been published on adult patients with BMD undergoing surgery with general anesthesia. Reports indicate that children with BMD may experience some serious complications with flurane-inhaled anesthesia. However, no study has yet shown that the use of flurane-induced anesthesia in adults with DMD carries high risks. PATIENT CONCERNS We describe a 56-year-old woman with BMD who was scheduled for laparoscopic hysterectomy and bilateral adnexectomy under general anesthesia due to a mass in the uterus. The patient was diagnosed with BMD 20 years back and reported that during this period, she was able to walk slowly with help during her daily life. Additionally, she also had a history of hypertension since 4 years and type 2 diabetes mellitus since 2 years. DIAGNOSIS The patient was postmenopausal and presented with abnormal uterine bleeding and elevated CA125. Abdominal ultrasonography revealed diffuse enlargement of the uterus and hypoechoic internal echoes. These findings were suggestive of diffuse adenomyosis with multiple uterine leiomyomas, which would have adverse effects later in her life. Therefore, the patient required surgery to address the symptoms and further confirm the diagnosis. The final diagnosis was confirmed by histopathological analysis. INTERVENTIONS The patient was scheduled for laparoscopic hysterectomy and bilateral adnexectomy. Anesthesia was induced and maintained by a combination of intravenous and inhalation anesthetic agents, particularly cisatracurium besilate and inhaled. sevoflurane. OUTCOMES The duration of anesthesia and postoperative period were uneventful. At the end of the operation, the patient had normal vital signs and was fully conscious. The patient was followed up for 8 months and no complications were noted during this period. LESSONS The combination of sevoflurane and cisatracurium besilate is a safe and effective method for the anesthetic management of adult patients with BMD scheduled for laparoscopic gynecological surgery. On the other hand, it is important to be aware of even rare complications of procedures, so that necessary precautions can be undertaken. Further investigations are necessary to determine the safe dosage of volatile anesthetics specifically for this clinical scenario so that anesthesiologists can use this combination method more accurately and precisely.
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Affiliation(s)
| | | | | | - Shi Zhang
- Department of Orthopaedic Surgery, The First Hospital of Jilin University, Changchun, Jilin, China
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Parish M, Farzin H. Adult patient with Becker dystrophy undergoing orthopedic surgery: an anesthesia challenge. Int Med Case Rep J 2018; 11:33-36. [PMID: 29497338 PMCID: PMC5818840 DOI: 10.2147/imcrj.s150037] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023] Open
Abstract
Muscular dystrophies are considered to be a series of neuromuscular diseases with genetic causes and are characterized by progressive muscle weakness and degeneration of the skeletal muscle. The case of an adult man with Becker dystrophy referred for repair of the patella tendon tearing and patella fracture is described. He underwent successful surgery using total intravenous anesthesia without any complications.
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Affiliation(s)
- Masoud Parish
- Anesthesiology Department, Tabriz University of Medical Sciences, Shohada Teaching Hospital, Tabriz, Iran
| | - Haleh Farzin
- Anesthesiology Department, Tabriz University of Medical Sciences, Shohada Teaching Hospital, Tabriz, Iran
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Abstract
This review identifies disease states associated with malignant hyperthermia susceptibility based on genotypic and phenotypic findings, and a framework is established for clinicians to identify a potentially malignant hyperthermia–susceptible patient.
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Iwata M, Kuzumoto N, Akasaki Y, Morioka M, Nakayama K, Matsuzawa N, Kimoto K, Shimomura T. The ultrasound-guided nerve blocks of abdominal wall contributed to anesthetic management of cholecystectomy in a patient with Becker muscular dystrophy without using muscle relaxants. JA Clin Rep 2017; 3:64. [PMID: 29457107 PMCID: PMC5804660 DOI: 10.1186/s40981-017-0134-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/28/2017] [Accepted: 11/28/2017] [Indexed: 12/12/2022] Open
Abstract
Becker muscular dystrophy (BMD) is a progressive neuromuscular disorder caused by mutations in the dystrophin gene. The sensitivity to non-depolarizing muscle relaxant in a patient with muscle dystrophy is reportedly higher than that in normal individuals, and the duration of the effect is known to be prolonged. In this report, we present the case of a 58-year-old man with BMD who underwent laparoscopic cholecystectomy for symptomatic cholelithiasis under total intravenous anesthesia without the use of muscle-relaxant drugs and supplemented with regional anesthesia. Anesthesia was induced and maintained with propofol, remifentanil, and fentanyl; ultrasound-guided bilateral rectus sheath block (RSB) and right-sided subcostal transversus abdominis plane block (TAP) were performed. The procedure required conversion to open surgery because of hard conglutination; intraoperative and postoperative periods were uneventful. Adequate analgesia was maintained after extubation because of the effect of RSB and TAP.
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Affiliation(s)
- Masato Iwata
- Department of Anesthesiology, Nara Prefecture general Medical Center, 30-1, Hiramatsu 1-chome, Nara city, Nara 631-0846 Japan
| | - Naoya Kuzumoto
- Department of Anesthesiology, Nara Prefecture general Medical Center, 30-1, Hiramatsu 1-chome, Nara city, Nara 631-0846 Japan
| | - Yuka Akasaki
- Department of Anesthesiology, Nara Prefecture general Medical Center, 30-1, Hiramatsu 1-chome, Nara city, Nara 631-0846 Japan
| | - Masayo Morioka
- Department of Anesthesiology, Nara Prefecture general Medical Center, 30-1, Hiramatsu 1-chome, Nara city, Nara 631-0846 Japan
| | - Kana Nakayama
- Department of Anesthesiology, Nara Prefecture general Medical Center, 30-1, Hiramatsu 1-chome, Nara city, Nara 631-0846 Japan
| | - Nobuyoshi Matsuzawa
- Department of Anesthesiology, Nara Prefecture general Medical Center, 30-1, Hiramatsu 1-chome, Nara city, Nara 631-0846 Japan
| | - Katsuhiro Kimoto
- 2Department of Anesthesiology, Nara Medical University, Kashihara, Japan
| | - Toshiyuki Shimomura
- Perioperative Care Center, Nara Prefecture General Medical Center, Nara, Japan
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11
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Gray RM. Anesthesia-induced rhabdomyolysis or malignant hyperthermia: is defining the crisis important? Paediatr Anaesth 2017; 27:490-493. [PMID: 28306187 DOI: 10.1111/pan.13130] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/29/2017] [Indexed: 12/11/2022]
Abstract
Anesthesia-induced rhabdomyolysis and malignant hyperthermia occur in response to the same agents and present with similar clinical features. Distinguishing between these diagnoses can be challenging in a clinical crisis yet making the distinction is critical as prompt appropriate management may be life-saving, whereas delayed recognition and/or inappropriate management will almost certainly result in death. This focused review examines the differences between these conditions and focuses on the emergency management of anesthesia-induced rhabdomyolysis.
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Affiliation(s)
- Rebecca M Gray
- Division of Paediatric Anaesthesia, Department of Anaesthesia and PeriOperative Medicine, University of Cape Town, Cape Town, South Africa
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Caroline P, Marie-Cécile N, Demet Y, Francis V. Case Report: Red Urine After Day Care Strabismus Surgery. ACTA ACUST UNITED AC 2017; 8:75-77. [PMID: 28045721 DOI: 10.1213/xaa.0000000000000430] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
In the absence of surgery on the urinary tract, the emission of red urine after anesthesia should be considered as a diagnostic emergency because it can be a sign of hematuria, hemoglobinuria, blood transfusion reaction, significant myoglobinuria, or porphyria.This case describes the management of a 12-year-old boy who presented red urine at the day care unit after strabismus surgery.
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Affiliation(s)
- Pregardien Caroline
- *Departments of Anesthesiology, Paediatric Neurology and Ophtalmology, Cliniques Universitaires Saint-Luc, Brussels, Belgium; and †Department of Anesthesiology, Hôpital Jeanne de Flandre, Lille, France
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Abstract
BACKGROUND It has been known that skeletal muscles show atrophic changes after prolonged sedation or general anesthesia. Whether these effects are due to anesthesia itself or disuse during anesthesia has not been fully clarified. Autophagy dysregulation has been implicated in muscle-wasting conditions. This study tested the hypothesis that the magnitude of skeletal muscle autophagy is affected by both anesthesia and immobility. METHODS The extent of autophagy was analyzed chronologically during general anesthesia. In vivo microscopy was performed using green fluorescent protein-tagged LC3 for the detection of autophagy using sternomastoid muscles of live mice during pentobarbital anesthesia (n = 6 and 7). Western blotting and histological analyses were also conducted on tibialis anterior muscles (n = 3 to 5). To distinguish the effect of anesthesia from that due to disuse, autophagy was compared between animals anesthetized with pentobarbital and those immobilized by short-term denervation without continuation of anesthesia. Conversely, tibialis anterior and sternomastoid muscles were electrically stimulated during anesthesia. RESULTS Western blots and microscopy showed time-dependent autophagy up-regulation during pentobarbital anesthesia, peaking at 3 h (728.6 ± 93.5% of basal level, mean ± SE). Disuse by denervation without sustaining anesthesia did not lead to equivalent autophagy, suggesting that anesthesia is essential to cause autophagy. In contrast, contractile stimulation of the tibialis anterior and sternomastoid muscles significantly reduced the autophagy up-regulation during anesthesia (85% at 300 min). Ketamine, ketamine plus xylazine, isoflurane, and propofol also up-regulated autophagy. CONCLUSIONS Short-term disuse without anesthesia does not lead to autophagy, but anesthesia with disuse leads to marked up-regulation of autophagy.
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Aristizabal JF, Smit RM. Orthodontic treatment in a patient with unilateral open-bite and Becker muscular dystrophy. A 5-year follow-up. Dental Press J Orthod 2015; 19:37-45. [PMID: 25628078 PMCID: PMC4347409 DOI: 10.1590/2176-9451.19.6.037-045.oar] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2013] [Accepted: 11/01/2013] [Indexed: 11/23/2022] Open
Abstract
INTRODUCTION: Becker muscular dystrophy is an X-chromosomal linked anomaly characterized by
progressive muscle wear and weakness. This case report shows the orthodontic
treatment of a Becker muscular dystrophy patient with unilateral open bite. METHODS: To correct patient's malocclusion, general anesthesia and orthognathic surgery
were not considered as an option. Conventional orthodontic treatment with
intermaxillary elastics and muscular functional therapy were employed instead.
RESULTS: After 36 months, open bite was corrected. The case remains stable after a 5-year
post-treatment retention period.
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A preliminary study of volatile agents or total intravenous anesthesia for neurophysiological monitoring during posterior spinal fusion in adolescents with idiopathic scoliosis. Spine (Phila Pa 1976) 2014; 39:E1318-24. [PMID: 25099322 DOI: 10.1097/brs.0000000000000550] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
STUDY DESIGN A prospective randomized controlled trial. OBJECTIVE The purpose of this study was to prospectively compare the efficacy of neurophysiological monitoring during general anesthesia with either a total intravenous technique or with the volatile anesthetic agent, desflurane. SUMMARY OF BACKGROUND DATA A total intravenous anesthetic technique is generally chosen when neurophysiological monitoring is used as it has been shown to facilitate such monitoring. Despite this, with prolonged infusions of propofol, prolonged awakening times may be seen, which may impact the time required for postoperative neurological assessment or more importantly result in significant delays, should a wake-up test become necessary. To date, there are no prospective trials comparing intravenous techniques with a volatile agent-based anesthetic technique and its effects on neurophysiological monitoring. METHODS This prospective study compares somatosensory evoked potential and motor evoked potential monitoring during posterior spinal fusion in 30 adolescents. The patients were randomized to receive a total intravenous technique with propofol-remifentanil or a volatile agent-based technique with desflurane-remifentanil. RESULTS The groups were similar with regard to age, weight, height, body mass index, Cobb angle, and distribution of Lenke classifications. No differences were noted in anesthesia time, surgery time, intraoperative fluids, or estimated blood loss between the 2 groups. Time to eye opening, time to following commands, and time to tracheal extubation were shorter in the volatile anesthesia group than the total intravenous anesthesia group. No clinically significant difference was noted in the amplitude or latency of somatosensory evoked potential monitoring. Although statistically significantly greater voltage amplitude was required to generate a motor evoked potential, the voltage amount was within a clinically acceptable range. CONCLUSION Our data demonstrate that a volatile agent-based anesthetic regimen is feasible even during neurophysiological monitoring. Advantages include a more rapid awakening and the feasibility of a rapid wake-up test (<5 min) in the event that irreversible changes in neurophysiological monitoring are noted. LEVEL OF EVIDENCE 2.
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Shimauchi T, Yamaura K, Sugibe S, Hoka S. Usefulness of sugammadex in a patient with Becker muscular dystrophy and dilated cardiomyopathy. ACTA ACUST UNITED AC 2014; 52:146-8. [PMID: 25199695 DOI: 10.1016/j.aat.2014.02.005] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2013] [Revised: 02/14/2014] [Accepted: 02/19/2014] [Indexed: 01/16/2023]
Abstract
A 54-year-old patient with Becker muscular dystrophy and dilated cardiomyopathy underwent laparoscopic cholecystectomy under total intravenous anesthesia. Muscle relaxation was induced by rocuronium (0.4 mg/kg body weight) under train-of-four (TOF) ratio monitoring. The TOF ratio was 0 at intubation, and 0.2 at the end of surgery. Residual muscle relaxant activity was successfully reversed by sugammadex (2 mg/kg body weight) without any hemodynamic adverse effects (TOF ratio 1.0 at extubation). The clinical and hemodynamic findings suggest that sugammadex can be safely used in patients with Becker muscular dystrophy and dilated cardiomyopathy.
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Affiliation(s)
- Tsukasa Shimauchi
- Department of Anesthesiology and Critical Care, Kyushu University Hospital, Fukuoka, Japan
| | - Ken Yamaura
- Operating Rooms, Kyushu University Hospital, Fukuoka, Japan.
| | - Sayaka Sugibe
- Department of Anesthesiology and Critical Care, Kyushu University Hospital, Fukuoka, Japan
| | - Sumio Hoka
- Department of Anesthesiology and Critical Care, Kyushu University Hospital, Fukuoka, Japan
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Cripe LH, Tobias JD. Cardiac considerations in the operative management of the patient with Duchenne or Becker muscular dystrophy. Paediatr Anaesth 2013; 23:777-84. [PMID: 23869433 DOI: 10.1111/pan.12229] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/23/2013] [Indexed: 12/22/2022]
Abstract
Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) is a progressive multisystem neuromuscular disorder. In addition to the skeletal muscle, the myocardium in the DMD/BMD patient is dystrophin deficient which results in a progressive cardiomyopathy. The myopathic myocardium poses significant risk of increased morbidity and mortality at the time of major surgical procedures. Careful attention must be given to the DMD/BMD patient during the intraoperative and postoperative period. Anesthesia selection is critical and anesthetics should be avoided which have been shown to be harmful in this patient population. Preanesthesia assessment should include cardiac consultation and detailed preoperative evaluation. Intraoperative management needs to insure that the weakened myocardium is not compromised by physiologic changes such as hypotension or major fluid shifts. Finally, attention to the cardiac status of the patient must continue into the postoperative period. The surgical care of the DMD/BMD patient requires a multispecialty approach to insure operative success.
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Affiliation(s)
- Linda H Cripe
- The Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH 43205-2664, USA.
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Segura LG, Lorenz JD, Weingarten TN, Scavonetto F, Bojanić K, Selcen D, Sprung J. Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures. Paediatr Anaesth 2013; 23:855-64. [PMID: 23919455 DOI: 10.1111/pan.12248] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 07/13/2013] [Indexed: 01/16/2023]
Abstract
BACKGROUND Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. OBJECTIVE To review the perioperative outcomes of patients with DMD and BMD. METHODS We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. RESULTS We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. CONCLUSIONS Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children.
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Affiliation(s)
- Leal G Segura
- Department of Anesthesiology, Mayo Clinic, Rochester, MN 55905, USA
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Nonneman DJ, Brown-Brandl T, Jones SA, Wiedmann RT, Rohrer GA. A defect in dystrophin causes a novel porcine stress syndrome. BMC Genomics 2012; 13:233. [PMID: 22691118 PMCID: PMC3463461 DOI: 10.1186/1471-2164-13-233] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2011] [Accepted: 05/25/2012] [Indexed: 11/25/2022] Open
Abstract
Background Losses of slaughter-weight pigs due to transport stress are both welfare and economic concerns to pork producers. Historically, the HAL-1843 mutation in ryanodine receptor 1 was considered responsible for most of the losses; however, DNA testing has effectively eliminated this mutation from commercial herds. We identified two sibling barrows in the USMARC swine herd that died from apparent symptoms of a stress syndrome after transport at 12 weeks of age. The symptoms included open-mouth breathing, skin discoloration, vocalization and loss of mobility. Results We repeated the original mating along with sire-daughter matings to produce additional offspring. At 8 weeks of age, heart rate and electrocardiographs (ECG) were monitored during isoflurane anesthesia challenge (3% for 3 min). Four males from the original sire-dam mating and two males from a sire-daughter mating died after one minute of anesthesia. Animals from additional litters were identified as having a stress response, sometimes resulting in death, during regular processing and weighing. Affected animals had elevated plasma creatine phosphokinase (CPK) levels before and immediately after isoflurane challenge and cardiac arrhythmias. A pedigree containing 250 pigs, including 49 affected animals, was genotyped with the Illumina PorcineSNP60 Beadchip and only one chromosomal region, SSCX at 25.1-27.7 Mb over the dystrophin gene (DMD), was significantly associated with the syndrome. An arginine to tryptophan (R1958W) polymorphism in exon 41 of DMD was the most significant marker associated with stress susceptibility. Immunoblots of affected heart and skeletal muscle showed a dramatic reduction of dystrophin protein and histopathology of affected hearts indicated muscle fiber degeneration. Conclusions A novel stress syndrome was characterized in pigs and the causative genetic factor most likely resides within DMD that results in less dystrophin protein and cardiac abnormalities that can lead to death under stressful conditions. The identification of predictive markers will allow us to determine the prevalence of this disease in commercial swine populations. This defect also provides a unique biomedical model for human cardiomyopathy associated with muscular dystrophy that may be superior to those available because of the similarities in anatomy and physiology and allow advances in gene therapies for human disease.
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Affiliation(s)
- Dan J Nonneman
- USDA, ARS, US Meat Animal Research Center, Clay Center, Nebraska, USA.
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Abstract
Among the neuromuscular diseases (NMDs) over 800 individual entities have been identified. The vast majority of these diseases occur very seldom but all NMDs together add up to a prevalence of 1:1,500. Accordingly the conclusion "seldom diseases are common" seems to be appropriate. The scope of NMDs is very wide varying from the affection of single muscle groups to the complete musculature, from slowly progressive to fulminant progressive forms, from perinatal to the adult manifestation as well as number, course and character of associated comorbidities. Due to the wide heterogeneity concerning NMDs it is of great importance for anesthesiologists to be knowledgeable on relevant comorbidities as well as indications and contraindications for the various anesthetics.
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Affiliation(s)
- M U Gerbershagen
- Universitätsklinikum Witten-Herdecke mit Sitz in Köln, Krankenhaus Köln-Merheim, Ostmerheimer Str. 200, 51109 Köln.
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From the Literature. Mol Diagn Ther 2010. [DOI: 10.1007/bf03256374] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022]
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Hopkins P. Anaesthesia and the sex-linked dystrophies: between a rock and a hard place. Br J Anaesth 2010; 104:397-400. [DOI: 10.1093/bja/aeq036] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
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