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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anesthesia may be administered to patients with Duchenne's muscular dystrophy, but cases are reported in which apparently healthy children suffer hyperkalemic cardiac arrest. We present the case of a 5-year-old boy whose muscular dystrophy was discovered following a fatal, perioperative cardiac arrest in the postanesthesia care unit.
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PMID:The postoperative cardiovascular arrest of a 5-year-old male: an initial presentation of Duchenne's muscular dystrophy. 1718 46

Patients with Becker's and Duchenne's muscular dystrophy occasionally have myocardial involvement leading to end-stage heart failure. Heart transplantation is established as an effective therapy. Achieving successful outcomes in this challenging group requires special consideration during the perioperative period to limit preoperative deconditioning, minimize anesthesia complications, and rapidly institute rehabilitation with appropriate precautions. We reviewed our recent experience with Becker's muscular dystrophy patients and discuss the management of perioperative issues specific to this patient group.
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PMID:Becker's muscular dystrophy and orthotopic heart transplantation: perioperative considerations. 1654 61

Severe muscular dystrophy (MD) has historically led to death in early adulthood, due to mainly cardiopulmonary complications. However, with newer methods of cardiac and respiratory management, survival has improved, and patients with MD are more frequently undergoing procedures requiring deep sedation or anesthesia. Respiratory management of these patients during procedures is challenging; safe and effective options for respiratory support are needed. In this report, we describe our experience using the laryngeal mask airway (LMA) to provide respiratory support during deep sedation or anesthesia for eight patients with severe MD during the following medical procedures: eight percutaneous endoscopic gastrostomy (PEG) placements, three lithotripsies, and placement of an implantable cardioverter-defibrillator. We also review the benefits and risks of the LMA in the context of other respiratory support options for people with MD, and the integral role of non-invasive positive pressure ventilation (NPPV) during induction of and recovery from deep sedation or general anesthesia.
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PMID:Use of the laryngeal mask airway in patients with severe muscular dystrophy who require sedation or anesthesia. 1699 27

Clinically significant liver damage in patients taking therapeutic doses of paracetamol is very rare. We report two cases of fulminant hepatic failure caused by therapeutic (4 g x day(-1)) paracetamol administration on our Intensive Care Unit. Both patients had a muscular dystrophy and presented with a chest infection on a background of endstage neuromuscular respiratory failure. We also noted one further similar case in the literature and suggest a relationship between muscular dystrophy and paracetamol-induced hepatotoxicity. In this report we discuss in detail possible mechanisms that may account for this apparent association, which include altered pharmacokinetics, reduced glutathione stores, malnutrition and hypoxic injury.
Anaesthesia 2008 Jan
PMID:Acute liver failure following therapeutic paracetamol administration in patients with muscular dystrophies. 1808 77

Adverse reactions to genral anesthesia, which partly resembled malignant hyperthermia (MH), were more frequent in muscular dystrophy than in controls. In the present study, 35 cases so far reported in Duchenne or Becker muscular dystrophy (DMD or BMD) were analyzed and their pathogenesis was discussed. Cardiac involvements were sole manifestations in 7 cases. In other 28 cases, the acute rhabdomyolysis was the most prevailing manifestation. About 60% of myolysis cases were associated with muscle contracture (rigidity) or other hypermetabolic signs such as hypercapnia, hyperthermia and metabolic acidosis. Cases with BMD were more hyperthermic than with DMD. These results suggest Ca ion-induced hypermetabolic reactions are also present in dystrophinopathy, which have been assumed as core syndromes of the classical (gene-defined) MH. However, question whether the abnormal Ca ion is from the extracellular or intracellular stores is still unclear. Circumstancial evidences suggest that the Ca-induced Ca release (CICR) mechanism might also be involved. Endogenous redox modulators such as nitric oxide or reactive oxygen species in the dystrophic muscle might contribute to the perturbed Ca ion homeostasis.
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PMID:[Malignant hyperthermia-like reactions in Duchenne or Becker muscular dystrophy: review and hypothesis]. 1832 2

Duchenne's muscular dystrophy (DMD) is the most common and severe form of myopathy occurring in pediatric patients. Sensitivity of patients with DMD to sedative, anesthetic and neuromuscular blocking agents may result in intraoperative and early postoperative cardiovascular and respiratory complications, as well as prolonged recovery from anesthesia. Anesthetic management of these patients is challenging and may cause serious problems to the anesthesiologist. We report the use of a total intravenous anesthesia technique (TIVA) with remifentanil and propofol without muscle relaxants, associated with intrathecal morphine in three children with DMD undergoing posterior spinal surgery (PSS). Tracheal intubation was successfully done with good conditions. The intraoperative course of these patients was uneventful. Controlled hypotension, rapid recovery and uneventful postoperative period were achieved with this technique. In conclusion, good conditions for tracheal intubation, controlled hypotension, rapid recovery and uneventful postoperative period can be achieved with this anesthesia technique in patients with DMD.
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PMID:Use of remifentanil and propofol without muscle relaxant combined with intrathecal morphine in children with Duchenne's muscular dystrophy undergoing spinal surgery. 1879 56

A non-randomised retrospective study to compare the results of surgical correction of scoliosis in Duchenne's muscular dystrophy (DMD) patients using three different instrumentation systems-Sublaminar instrumentation system (Group A), a hybrid of sublaminar and pedicle screw systems (Group B) and pedicle screw system alone (Group C). Between 1993 and 2003, 43 patients with DMD underwent posterior spinal fusion and instrumentation. Group A (n = 19) had sublaminar instrumentation system, Group B (n = 13) had a hybrid construct and Group C (n = 11) was treated with pedicle system. The mean blood loss in Group A was 4.1 l, 3.2 l in Group B and 2.5 l in Group C. Average operating times in Group A, B and C were 300, 274 and 234 min, respectively. Mean pre-operative, post-operative and final Cobb angle in Group A was 50.05 +/- 15.46 degrees , 15.68 +/- 11.23 degrees and 21.57 +/- 11.63 degrees , Group B was 17.76 +/- 8.50 degrees , 3.61 +/- 2.53 degrees and 6.69 +/- 4.19 degrees and Group C was 25.81 +/- 9.94 degrees , 5.45 +/- 3.88 degrees , 8.90 +/- 5.82 degrees , respectively. Flexibility index or the potential correction calculated from bending radiographs were 60 +/- 6.33, 70 +/- 4.65 and 67 +/- 6.79% for Group A, Group B and Group C respectively. The percentage correction achieved was 72.5 +/- 14.5% in Group A, 82 +/- 6% in Group B and 82 +/- 8% in Group C. The difference between percentage correction achieved and the flexibility index was 12.45 +/- 8.22, 12.05 +/- 1.3 and 15.00 +/- 1.21% in Group A, B and C, respectively The percentage loss of correction in Cobb angles at final follow-up in Group A, B and C was 12.5 +/- 3.5, 16.5 +/- 1. and 12.5 +/- 2.5%, respectively. Complications seen in Group A were three cases of wound infection and two cases of implant failure; Group B had a single case of implant failure and Group C had one patient with wound infection and one case with a partial screw pull out. Early surgery and smaller curve corrections appears to be the current trend in the management of scoliosis in DMD. This has been possible due to early curve detection and surgery thus having the advantage of less post-operative respiratory complications and stay in paediatric intensive care. Also, early surgery avoids development of pelvic deformity and extension of instrumentation to the pelvis thereby reducing blood loss. This trend reflects the advent of newer and safer instrumentation systems, advanced techniques in anaesthesia and cord monitoring. Sublaminar instrumentation system group had increased operating times and blood loss compared to both the hybrid and pedicle screw instrumentation systems due to increased bleeding from epidural vessels and pelvic instrumentation. Overall, the three instrumentation constructs appear to provide and maintain an optimal degree of correction at medium to long term follow up but the advantages of lesser blood loss and surgical time without the need for pelvic fixation seem to swing the verdict in favour of the pedicle screw system.
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PMID:Scoliosis in Duchenne's muscular dystrophy: a changing trend in surgical management : a historical surgical outcome study comparing sublaminar, hybrid and pedicle screw instrumentation systems. 1976 Feb 71

Stem cell based therapies for the repair and regeneration of various tissues and organs offer a paradigm shift that may provide alternative therapeutic solutions for a number of diseases. This review focuses on skeletal muscle regeneration and repair by adipose-derived stem cells (ASCs) with particular attention to their potential use as a therapy for disorders such as degenerative muscle diseases or skeletal muscle injuries. ASCs can differentiate into skeletal muscle cells in vitro either in co-culture with skeletal myoblasts, or when cultured in medium supplemented with horse serum and/or under reduced serum conditions. In particular, spontaneous fusion of ASCs and subsequent myotube-like formation was observed in early culture passages at high cell density. ASCs have also shown a capacity for myogenic differentiation in vivo. In a murine muscular dystrophy model, ASCs were able to restore muscle function following direct injection into the affected muscle as well as following intravenous systemic administration. Of great importance is the finding that allogeneic ASCs injected into the damaged muscle were not rejected, even without immunosuppressive therapy. Because human adipose tissue is ubiquitous and easily obtainable in large quantities under local anesthesia with little patient discomfort, it presents an appealing source of stem cells for mesenchymal tissue regeneration and engineering.
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PMID:The potential for treatment of skeletal muscle disorders with adipose-derived stem cells. 1994 55

A previously fit 12-yr-old boy, who had no previous history of anaesthesia, underwent general anaesthesia using isoflurane for an elective circumcision. After uneventful surgery and anaesthesia, he suffered a cardiorespiratory arrest in the recovery room. Prompt oxygenation and cardiopulmonary resuscitation (CPR) were instituted. The initial serum potassium was >13 mmol litre(-1) and prolonged CPR was required while potassium levels were reduced. Further investigation demonstrated a creatine kinase (CK) >70 000 U litre(-1) which was consistent with a diagnosis of rhabdomyolysis. Despite requiring CPR for 1 h 45 min and a prolonged intensive care admission for multi-organ failure, the child has made an excellent recovery, including normal cognitive function. Subsequent genetic analysis has shown that the boy has previously undiagnosed Becker's muscular dystrophy. We believe that the patient had acute rhabdomyolysis as a result of a volatile anaesthetic agent in association with an undiagnosed muscular dystrophy. In recent years, largely based on case report literature, there has been a shift in opinion as to the cause of such adverse perioperative events. What was previously thought to be malignant hyperpyrexia (MH) is now considered to be anaesthesia-induced rhabdomyolysis, an alternative and distinct reaction. The distinguishing feature of anaesthesia-induced rhabdomyolysis from MH is an acute rhabdomyolysis, without preceding hypermetabolism.
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PMID:Perioperative cardiac arrest in a patient with previously undiagnosed Becker's muscular dystrophy after isoflurane anaesthesia for elective surgery. 2022 83

Myotonic dystrophy (MD) is a muscle disorder characterized by progressive muscle wasting and weakness, and is the most common form of muscular dystrophy that begins in adulthood, often after pregnancy. MD might be related to occurrence of malignant hyperthermia. Therefore, the cesarean section is often performed for the parturient with MD. We had an experience of combined spinal-epidural anesthesia for cesarean section in a parturient complicated with MD. A 40-year-old woman had rhabdomyolysis caused by ritodrine at 15-week gestation and was diagnosed as MD by electromyography. Her first baby died due to respiratory failure fourth day after birth. She had hatchet face, slight weakness of her lower extremities, and easy fatigability. Her manual muscle test was 5/5 at upper extremities and 4/5 at lower extremities. She underwent emergency cesarean section for premature rupture of the membrane, weak pain during labor, and obstructed labor at 33-week gestation. We placed an epidural catheter from T12/L1 and punctured arachnoid with 25 G spinal needle. We performed spinal anesthesia using 0.5% hyperbaric bupivacaine 1.5 ml and epidural anesthesia using 2% lidocaine 6 ml. Her anesthetic level reached bilaterally to T7 and operation started 18 minutes after combined spinal-epidural anesthesia. Her baby was born 23 minutes after the anesthesia. As her baby was 1/5 at Apgar score, the baby was tracheally intubated and artificially ventilated. The cesarean section was finished in 33 minutes uneventfully. She had no adverse events and was discharged on the 8th postoperative day. Later her baby was diagnosed as congenital MD by gene analysis. Combined spinal-epidural anesthesia with the amide-typed local anesthetic agents could be useful and safe for cesarean section in the parturient with MD.
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PMID:[Combined spinal-epidural anesthesia for cesarean section in a parturient with myotonic dystrophy]. 2071 26


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