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Anesthesia for Muscular dystrophy

Juan Tong, Fu-Chu Chen, Gui-Yuan Li, Xiang-Ao Kong, Ying He, Hong Yao
OBJECTIVE: To observe the immunoregulatory effect of electroacupuncture (EA) intervention for muscular dystrophy chronic obstructive pulmonary disease (COPD) rats, so as to investigate its underlying mechanism in improving respiratory function. METHODS: Forty male SD rats were randomly divided into 5 groups: normal, model, EA, exercise, and EA+ exercise ( n =8 in each). The muscular dystrophy COPD model was established by placing the rats in a closed box to be exposed to cigarette smoke (3-10 cigarettes/time) for 60 min, twice daily, 6 days a week for 90 days...
April 25, 2018: Zhen Ci Yan Jiu, Acupuncture Research
Alexander B Froyshteter, Tarun Bhalla, Joseph D Tobias, Gregory S Cambier, Christopher T Mckee
Patients with Duchenne muscular dystrophy (DMD) often have systemic manifestations with comorbid involvement of the cardiac and respiratory systems that increase the risk of anesthetic and perioperative morbidity. These patients frequently develop progressive myocardial involvement with cardiomyopathy, depressed cardiac function, and arrhythmias. The latter may necessitate the placement of an automatic implantable cardioverter defibrillator (AICD) insertion. As a means of avoiding the need for general anesthesia and its inherent potential of morbidity, regional anesthesia may be used in specific cases...
April 2018: Saudi Journal of Anaesthesia
Masoud Parish, Haleh Farzin
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.
2018: International Medical Case Reports Journal
Ariane Boivin, Richard Antonelli, Navil F Sethna
BACKGROUND: In past decades, Duchenne muscular dystrophy patients have been living longer and as the disease advances, patients experience multisystemic deterioration. Older patients often require gastrostomy tube placement for nutritional support. For optimizing the perioperative care, a practice of multidisciplinary team can better anticipate, prevent, and manage possible complications and reduce the overall perioperative morbidity and mortality. AIMS: The aim of this study was to review our experience with perioperative care of adolescent and young adults with Duchenne muscular dystrophy undergoing gastrostomy by various surgical approaches in order to identify challenges and improve future perioperative care coordination to reduce morbidity...
February 2018: Paediatric Anaesthesia
Helga Cristina Almeida da Silva, Marcia Hiray, Mariz Vainzof, Beny Schmidt, Acary Souza Bulle Oliveira, José Luiz Gomes do Amaral
BACKGROUND AND OBJECTIVES: Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. CASE REPORT: Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040IU...
May 30, 2017: Revista Brasileira de Anestesiologia
Ji Eun Kim, Hea Rim Chun
INTRODUCTION: Anesthetic management of patients with Duchenne muscular dystrophy (DMD) is complicated because these patients are more sensitive to nondepolarizing neuromuscular blocking agents (NMBAs) and are vulnerable to postoperative complications, such as postoperative residual curarization and respiratory failure. Sugammadex is a new reversal agent for aminosteroidal NMBAs, but its safety in children is controversial. CLINICAL FEATURES: An 11-year-old boy with DMD underwent general anesthesia for a percutaneous nephrolithotomy...
March 2017: Medicine (Baltimore)
Adele D'Amico, Michela Catteruccia, Giovanni Baranello, Luisa Politano, Alessandra Govoni, Stefano Carlo Previtali, Marika Pane, Maria Grazia D'Angelo, Claudio Bruno, Sonia Messina, Federica Ricci, Elena Pegoraro, Antonella Pini, Angela Berardinelli, Ksenjia Gorni, Roberta Battini, Gianluca Vita, Federica Trucco, Marianna Scutifero, Roberta Petillo, Paola D'Ambrosio, Anna Ardissone, Barbara Pasanisi, Giuseppe Vita, Tiziana Mongini, Maurizio Moggio, Giacomo Pietro Comi, Eugenio Mercuri, Enrico Bertini
Despite all the advances in diagnosis and management of Duchenne muscular dystrophy over the past 50 years, the average age at diagnosis in most countries in the world around is still around 4-5 years. This retrospective study investigates the age at diagnosis in Italy in the past 10 years. We report findings from 384 boys who were diagnosed with DMD from 2005 to 2014. The mean age at first medical contact, which raised the suspicion of DMD, was 31 months. The mean age at diagnosis was 41 months. The finding that more frequently brought to suspect a DMD was the incidental finding of consistent elevated creatine kinase serum level detected during routine assessments in children undergoing general anesthesia or with intercurrent illness...
May 2017: Neuromuscular Disorders: NMD
Karel Heytens, Jan De Bleecker, Walter Verbrugghe, Jonathan Baets, Luc Heytens
In view of the enormous popularity of mass sporting events such as half-marathons, the number of patients with exertional rhabdomyolysis or exercise-induced heat stroke admitted to intensive care units (ICUs) has increased over the last decade. Because these patients have been reported to be at risk for malignant hyperthermia during general anesthesia, the intensive care community should bear in mind that the same risk of life-threatening rhabdomyolysis is present when these patients are admitted to an ICU, and volatile anesthetic sedation is chosen as the sedative technique...
February 4, 2017: World Journal of Critical Care Medicine
F Tatulli, A Caraglia, A Delcuratolo, S Cassano, G S Chetta
INTRODUCTION: Inguinal hernia repairs are routinely performed as outpatient procedures in most patients, whereas a few require admission due to clinical or social peculiarities. Muscular dystrophies are inherited disorders characterized by progressive muscle wasting and weakness. In case of surgery there is no definite recommendation for either general or regional anesthesia. CASE REPORT: This contribution regards a 48 y. o. male patient diagnosed with Becker Muscular Dystrophy by muscle biopsy 10 years earlier...
September 2017: Il Giornale di Chirurgia
Ashwin Subramaniam, Robert Grauer, David Beilby, Ravindranath Tiruvoipati
Myotonic dystrophy (DM), though rare, can significantly complicate anesthesia due to muscular and extra-muscular involvement. When this condition is compounded by a pheochromocytoma, anesthetizing such patients becomes extra challenging. We present a case report of a 61-year-old lady with congenital DM, with the whole gamut of associated features, was diagnosed with a noradrenaline secreting paraganglionoma following investigation of refractory hypertension. We anesthetized her for an open resection of the lesion...
November 2016: Journal of Clinical Anesthesia
Özlem Özmete, Mesut Şener, Esra Çalışkan, Anış Arıboğan
Duchenne's muscular dystrophy (DMD) is a neuromuscular disease with a progressive course. It is the most common and most severe muscular dystrophic disorder for which the application of anesthesia is critical, due to muscle weakness, and cardiac and pulmonary involvement. Successful application of spinal anesthesia in a 2-year-old boy with DMD undergoing bilateral inguinal hernia repair is described in the present report. It is proposed that spinal anesthesia is an effective alternative to general anesthesia in certain pediatric patients, including those with DMD, for whom general anesthesia poses increased risk...
April 2016: Aǧrı: Ağrı (Algoloji) Derneği'nin Yayın Organıdır, the Journal of the Turkish Society of Algology
Frederic Shapiro, Umeshkumar Athiraman, David J Clendenin, Monica Hoagland, Navil F Sethna
BACKGROUND: The objectives are to review the anesthetic management and anesthetic-related adverse events in patients undergoing muscle biopsy for a broad spectrum of neuromuscular disorders (NMD). AIM: The study aims to assess the hypothesis that perceived awareness of potential anesthesia-induced hyperkalemia and MH in patients with NMD reduces the frequency of such events. METHODS: A 20-year retrospective review of 877 consecutive patients undergoing muscle biopsy to establish diagnoses of NMD has been performed...
July 2016: Paediatric Anaesthesia
Shuhei Matsui, Satoshi Tanaka, Kenkichi Kiyosawa, Toshiyuki Tanaka, Mikito Kawamata
A 39-year-old female with facioscapulohumeral muscular dystrophy (FSHD) was scheduled for thoracoscopic resection of an anterior mediastinal tumor. She had slowly progressive weakness and atrophy in the fascial and shoulder girdle muscles. General anesthesia was induced and maintained with propofol, remifentanil, and fentanyl combined with thoracic paravertebral block. Rocuronium-induced neuromuscular blockade was evaluated with acceleromyography at the corrugator supercilii, masseter, and adductor pollicis muscles...
December 2015: Masui. the Japanese Journal of Anesthesiology
Seung Uk Bang, Yee Suk Kim, Woo Jin Kwon, Sang Mook Lee, Soo Hyang Kim
General anesthesia and central neuraxial blockades in patients with severe Duchenne muscular dystrophy are associated with high risks of complications, including rhabdomyolysis, malignant hyperthermia, hemodynamic instability, and postoperative mechanical ventilation. Here, we describe peripheral nerve blocks as a safe approach to anesthesia in a patient with severe Duchenne muscular dystrophy who was scheduled to undergo surgery. A 22-year-old male patient was scheduled to undergo reduction and internal fixation of a left distal femur fracture...
April 2016: Journal of Anesthesia
Nethra H Nanjundaswamy
Calcinosis cutis is a rare clinical condition wherein calcium is deposited in the layers of the skin. Various types of calcinosis cutis have been described based on histology and location. Airway may be involved only if calcinosis cutis is associated with overlapping syndrome of connective tissue disorders and muscular dystrophy. I report a rare case of dystrophic calcinosis cutis without any connective tissue disorders with the unanticipated difficult airway. A young lady with dystrophic calcinosis cutis of the gluteal region was posted for surgical excision and flap cover procedure under general anesthesia...
September 2015: Anesthesia, Essays and Researches
R V Ranjan, T R Ramachandran, S Manikandan, Roshan John
Limb-girdle muscular dystrophy (LGMD) is an autosomal recessive disorder in which the pelvic or shoulder girdle musculature is predominantly or primarily involved. We report the management of a 27-year-old primigravida with LGMD associated with obesity posted for elective cesarean section. She was successfully managed with epidural anesthesia assisted with noninvasive positive pressure ventilation. She had an uncomplicated intra- and post-operative course.
January 2015: Anesthesia, Essays and Researches
Hirohito Ishii, Kunihide Nakamura, Hiroyuki Nagahama, Masakazu Matsuyama, Jouji Endo, Masanori Nishimura
Duchenne muscular dystrophy (DMD) is an X-linked disease, but female carriers infrequently have some symptoms, who are called manifesting carriers. Here we report a case of a manifesting carrier of DMD with skeletal muscle weakness and cardiac abnormalities such as deterioration of cardiac function and left ventricular dilatation, who successfully underwent cardiac surgery. A 79-year-old female with acute heart failure for severe mitral regurgitation was admitted to our hospital. Surgical replacement of the mitral valve was performed under general anesthesia with intravenous anesthetics and non-depolarizing muscle relaxant...
February 2015: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Satoshi Yamaguchi, Giichiro Ohno, Jiro Kitamura
A 70-year-old man with Becker muscular dystrophy (BMD) underwent laparoscopic rectopexy under general anesthesia. For anesthetic induction, we administered total 0.6 mg · kg-1 of rocuronium with titration. Eight minutes later, train-of-four (TOF) count reached to 0 and the patient was intubated smoothly. One hundred and five minutes later, TOF ratio recovered to 100% and we administered rocuronium 10 mg additionally. Surgery was finished without any problems 95 minutes after thereafter. TOF ratio was 45% and we administered sugammadex 3 mg · kg-1, reversing neuromuscular blockade to TOF ratio 100% within 1...
October 2014: Masui. the Japanese Journal of Anesthesiology
Emmanuelle Salort-Campana, Karine Nguyen, Rafaelle Bernard, Elisabeth Jouve, Guilhem Solé, Aleksandra Nadaj-Pakleza, Julien Niederhauser, Estelle Charles, Elisabeth Ollagnon, Françoise Bouhour, Sabrina Sacconi, Andoni Echaniz-Laguna, Claude Desnuelle, Christine Tranchant, Christophe Vial, Frederique Magdinier, Marc Bartoli, Marie-Christine Arne-Bes, Xavier Ferrer, Thierry Kuntzer, Nicolas Levy, Jean Pouget, Shahram Attarian
BACKGROUND: Facioscapulohumeral muscular dystrophy type 1(FSHD1) is an autosomal dominant disorder associated with the contraction of D4Z4 less than 11 repeat units (RUs) on chromosome 4q35. Penetrance in the range of the largest alleles is poorly known. Our objective was to study the penetrance of FSHD1 in patients carrying alleles ranging between 6 to10 RUs and to evaluate the influence of sex, age, and several environmental factors on clinical expression of the disease. METHODS: A cross-sectional multicenter study was conducted in six French and one Swiss neuromuscular centers...
2015: Orphanet Journal of Rare Diseases
V M Mikhaĭlov, G I Mamaeva
We have studied the influence of NADP+ on routine ECG in 6 months old C57BL/6 and mdx mice. The animals were anestheized by ether before ECG recording. ECG registration was made with the speed of 100 mm per sec. The first ECG recording was made before intraperitoneal NADP+ injection in a dose of 13 or 80 mg/kg. The second ECG recording was made in 10 min after NADP+ injection. Then anesthesia was cut off. The mice were occasionally anestheized 45-60 min later and the third ECG was recorded in 1 h after injection of NADP+...
2013: Tsitologiia
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