Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy

Boland, Benoît; Silbert, Peter L.; Groover, Robert V.; Wollan, Peter C.; Silverstein, Marc D.

doi:10.1016/0887-8994(95)00251-0

Cited by 132 publications

(86 citation statements)

References 23 publications

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“…The ages at first walking and loss of ambulation in this study were consistent with past reports (2,3,15,16). The ages at ventilator and tube nutrition introductions and death were similar to those reported in recent studies (3,10,17,18).…”

Section: Discussionsupporting

confidence: 82%

Mental Retardation and Lifetime Events of Duchenne Muscular Dystrophy in Japan

Mochizuki

Miyatake²,

Suzuki³

et al. 2008

Intern. Med.

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Section: Discussionsupporting

confidence: 82%

Mental Retardation and Lifetime Events of Duchenne Muscular Dystrophy in Japan

Mochizuki

Miyatake²,

Suzuki³

et al. 2008

Intern. Med.

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“…In previous studies of anesthesia in patients with DMD [3,5], the average age of the patients was 9 to 14 years. The authors have not collected any cases in patients aged 30 years or older, and case reports of DMD patients aged 30 years or older are especially rare.…”

Section: Discussionmentioning

confidence: 99%

“…According to Boland et al [5], DMD is diagnosed at an average age of 4.6 years. By 10 years of age, patients with DMD rely on wheelchairs.…”

Section: Discussionmentioning

confidence: 99%

Use of remifentanil and propofol without muscle relaxant with Duchenne muscular dystrophy - A case report -

et al. 2018

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Duchenne muscular dystrophy (DMD) is a recessive inherited genetic disorder caused by a mutation in the X chromosome and occurs in approximately 1 in 3,500 males. Generally, degeneration and atrophy of the respiratory muscles and myocardium lead to death before the age of 30 years. During anesthesia, rhabdomyolysis, malignant hyperthermia, and cardiac arrest are known risks caused by an inhalation anesthetic or succinylcholine; therefore, the selection of inhalation anesthetics or muscle relaxants requires caution. According to a study by Muenster et al. [1], 4% of DMD patients had difficult intubations and the frequency was especially high in older patients [2]. Thus, difficulty in securing the airway can be anticipated and sufficient muscle relaxation is required.The risks are known for depolarizing muscle relaxants. For non-depolarizing muscle relaxants, it is generally known that sensitivity increases, but the response and stability have not yet been established [2,3]. Therefore, preoperatively securing the airway and recovering respiratory function postoperatively can be considered the most challenging aspects in anesthesia.Thus, together with a review of the relevant literature, we report a case of general anesthesia management without muscle relaxation in a patient with DMD undergoing surgery for testicular cancer. CASE REPORTA 29-year-old male patient with a height of 160 cm and a weight of 40 kg was diagnosed with testicular cancer and ad- Duchenne muscular dystrophy (DMD) is a relatively rare muscle disease with severe symptoms. Owing to the commonly limited mouth opening, cervical spine immobility, and deformation, DMD patients often present with a difficult airway. Patients with DMD are sensitive to sedation, anesthesia, and neuromuscular blockade. This risk increases as the disease progresses with age. The anesthetic management of these patients can cause various issues, presenting a challenge to anesthesiologists. We administered anesthesia for an orchiectomy in a patient with testicular cancer using total intravenous anesthesia with propofol and remifentanil without muscle relaxants. Although the patient was Mallampati grade IV due to neck stiffness, tracheal intubation was successfully performed with a portable videolaryngoscope. The intraoperative course was uneventful and recovery was rapid without postoperative complications. In conclusion, anesthesia without a muscle relaxant was successful and the patient recovered rapidly, even with a difficult tracheal intubation.

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“…Asociado a la poca ingesta de líquidos se presenta disfunción intestinal crónica con constipación, distensión, hipomotilidad e impactación (23). Actualmente se sospecha una posible predisposición a trombosis venosas pélvicas o de miembros inferiores secundaria a distensión abdominal crónica.…”

Section: Características Clínicasunclassified

Distrofia muscular de Duchenne. Perspectivas desde la rehabilitación

Chaustre

Chona

2011

Rev. Med.

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Duchenne Muscular Dystrophy (DMD) is a type of severe neuromuscular disease that occurs during childhood. It affects approximately 1 in 3500 to 6000 live male births; it is associated with the recessive X chromosome, and it manifest itself in a slightest way as Becker Muscular Dystrophy, due to a mutation in the dystrophin gene located in Xp21. This is a disabling disease that inevitably occurs until a progressive deterioration of the muscles leads to the patient´s death, usually from cardiorespiratory complications. From the point of view of rehabilitation, a series of management strategies have been developed in multidisciplinary groups ranging from gym work with stretch therapists to wheelchair prescriptions, and the development of noninvasive mechanical ventilation which, while they do not modify the genetic basis of the disease, do significantly improve the patient's independence as well as the care given by family and caregivers, and provide a better quality of life. Key words: Duchenne muscular dystrophy, dystrophin, rehabilitation ResumenLa Distrofia Muscular de Duchenne (DMD) es una enfermedad hereditaria de tipo neuromuscular progresiva, que se presenta durante la infancia; afecta aproximadamente 1 de cada 3500 a 6000 niños varones nacidos vivos. El gen DMD que codifica para la distrofina, una proteína estructural del músculo, se localiza en el brazo corto del cromosoma X en la región p21, puede sufrir diferentes mutaciones ocasionando la pérdida parcial o total de la proteína lo que origina la DMD y otras enfermedades relacionadas como la Distrofia Muscular de Becker, que es una distrofia de forma más leve. Esta es una enfermedad discapacitante que se caracteriza por un deterioro progresivo de los músculos, y en las etapas avanzadas de la enfermedad se presentan generalmente complicaciones cardiorespiratorias que son las causas principales que ocasionan la muerte al paciente. Desde el punto de vista de la rehabilitación, cuyo objetivo es retrasar la progresión de la enfermedad, se han venido desarrollado una serie de estrategias de tratamiento y manejo que involucran grupos multidisciplinarios; éstas van desde trabajo en gimnasio con terapeutas para estiramientos hasta prescripción de sillas de ruedas y formulación de ventilación mecánica no invasiva, que si bien se sabe no modifican las bases genéticas de la enfermedad, sí mejoran de forma importante la independencia del paciente, facilitan los cuidados por parte de la familia y los cuidadores y permiten una mejor calidad de vida.Palabras clave: distrofia muscular de Duchenne, distrofina, rehabilitación

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Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy

Cited by 132 publications

References 23 publications

Mental Retardation and Lifetime Events of Duchenne Muscular Dystrophy in Japan

Mental Retardation and Lifetime Events of Duchenne Muscular Dystrophy in Japan

Use of remifentanil and propofol without muscle relaxant with Duchenne muscular dystrophy - A case report -

Distrofia muscular de Duchenne. Perspectivas desde la rehabilitación

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