Distrofia muscular de Duchenne |
Manfred Baumgartner, Daniel Argüello Ruiz.
REVISTA MEDICA DE COSTA RICA Y CENTROAMERICA LXV (586) 315-318;2008
La distrofia muscular de Duchenne, es la más frecuente de la niñez. Es un desorden de carácter hereditario recesivo ligado al cromosoma X, caracterizada por debilidad muscular rápidamente progresiva, la cual empieza por los músculos de la pelvis y proximales de las piernas y luego afecta todo el cuerpo, con un pronóstico de vida no mayor de tres décadas.
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Consenso del American College of Chest Physicians sobre el manejo respitarorio relacionado a pacientes con distrofina muscular de Duchenne sometidos a anestesia o sedación |
The American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation.
Birnkrant DJ.
MetroHealth Medical Center, Case Western Reserve University, Department of Pediatrics, Department of Pediatrics, 2500 MetroHealth Dr, Cleveland, OH 44109, USA.
Pediatrics. 2009 May;123 Suppl 4:S242-4.
Abstract
This is a summary of the presentation on the American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing sedation or anesthesia, presented as part of the program on pulmonary management of pediatric patients with neuromuscular disorders at the 30th Annual Carrell-Krusen Neuromuscular Symposium on February 20, 2008.
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| Influencia de la progresión de la enfermedad sobre el efecto de bloqueo neuromuscular del mivacurio en niños y adolescentes con distrofina muscular de Duchenne |
Influence of disease progression on the neuromuscular blocking effect of mivacurium in children and adolescents with Duchenne muscular dystrophy.
Ihmsen H, Schmidt J, Schwilden H, Schmitt HJ, Muenster T.
Department of Anesthesiology, Friedrich-Alexander University Erlangen-Nuremberg, Erlangen, Germany.
Anesthesiology. 2009 May;110(5):1016-9. Abstract
BACKGROUND: Studies with nondepolarizing neuromuscular blocking agents showed a delayed onset and prolonged recovery in patients with Duchenne muscular dystrophy. The objective of this study was to investigate if these alterations depend on disease progression. METHODS: The authors studied 11 children (6-9 yr) with moderate Duchenne muscular dystrophy, 11 adolescents (12-16 yr) with advanced Duchenne muscular dystrophy, and 2 age-matched control groups of 8 patients each (5-9 and 10-17 yr). Anesthesia was performed with propofol and remifentanil. Patients received a single intravenous dose of 0.2 mg/kg mivacurium. Neuromuscular transmission was monitored by acceleromyography. The time course of neuromuscular blockade was characterized by the onset time and the times to different levels of recovery. RESULTS: Onset and duration of neuromuscular blockade were significantly prolonged in adolescent Duchenne muscular dystrophy patients (onset time, 4.0 min; recovery index, 12.3 min; median), as compared with Duchenne muscular dystrophy children (onset time, 2.3 min; recovery index, 6.8 min), and also as compared with young controls (onset time, 2.0 min; recovery index, 4.4 min) and adolescent controls (onset time, 2.5 min; recovery index, 4.8 min). Within the Duchenne muscular dystrophy patients, onset time and recovery index increased significantly with age. In the control group, age had no effect. CONCLUSIONS: The neuromuscular blocking effects of mivacurium showed a significant age dependency in Duchenne muscular dystrophy patients, which was most probably caused by the progression of the disease.
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| Inicio y duración del bloqueo neuromuscular inducido por mivacurio en pacientes con distrofina muscular de Duchenne |
Onset and duration of mivacurium-induced neuromuscular block in patients with Duchenne muscular dystrophy.
Schmidt J, Muenster T, Wick S, Forst J, Schmitt HJ.
Department of Anaesthesiology, Friedrich-Alexander University, Erlangen-Nuremberg, Krankenhausstrasse 12, D-91054 Erlangen, Germany.
Br J Anaesth. 2005 Dec;95(6):769-72. Epub 2005 Sep 23. Abstract
BACKGROUND: To determine the response to mivacurium, we prospectively studied onset time and complete spontaneous recovery from mivacurium-induced neuromuscular block in patients with Duchenne muscular dystrophy (DMD). METHODS: Twelve boys with DMD, age 5-14 yr, seven of them wheelchair-bound, ASA II-III, and 12 age- and sex-matched controls (ASA I) were enrolled in the study. Anaesthesia was induced with fentanyl 2-3 microg kg(-1) and propofol 3-4 mg kg(-1) titrated to effect, and maintained by continuous i.v. infusion of propofol 8-12 mg kg(-1) and remifentanil as required. The lungs were ventilated with oxygen in air. Neuromuscular transmission was assessed by acceleromyography using train-of-four (TOF) stimulation every 15 s. After baseline readings, a single dose of mivacurium 0.2 mg kg(-1) was given. The following variables were recorded: (i) lag time; (ii) onset time; (iii) peak effect; (iv) recovery of first twitch from the TOF response to 10, 25 and 90% (T(10), T(25), T(90)) relative to baseline; (v) recovery index (time between 25 and 75% recovery of first twitch); and (vi) recovery time (time between 25% recovery of first twitch and recovery of TOF ratio to 90%). For comparison between the groups the Mann-Whitney U-test was applied. RESULTS: There were no differences between the groups in lag time, onset time and peak effect. However, all recorded recovery indices were significantly (P<0.05) prolonged in the DMD group. The median (range) for time points T(10), T(25) and T(90) in the DMD and control group was 12.0 (8-16) vs 8.4 (5-15) min, 14.1 (9-20) vs 10.5 (7-17) min and 26.9 (15-40) vs 15.9 (12-23) min, respectively. The recovery index and recovery time were similarly prolonged in the DMD group.
CONCLUSIONS: These results support the assumption that mivacurium-induced neuromuscular block is prolonged in patients with DMD
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| Distribución de T2 en ventrículo izquierdo en la distrofina muscular de Duchenne |
Left ventricular T2 distribution in Duchenne muscular dystrophy.
Wansapura JP, Hor KN, Mazur W, Fleck R, Hagenbuch S, Benson DW, Gottliebson WM.
Division of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. janaka.wansapura@cchmc.org
J Cardiovasc Magn Reson. 2010 Mar 18;12:14.
Abstract
BACKGROUND: Although previous studies have helped define the natural history of Duchenne muscular dystrophy (DMD)-associated cardiomyopathy, the myocardial pathobiology associated with functional impairment in DMD is not yet known. The objective of this study was to assess the distribution of transverse relaxation time (T2) in the left ventricle (LV) of DMD patients, and to determine the association of myocardial T2 heterogeneity to the severity of cardiac dysfunction. DMD patients (n = 26) and normal control subjects (n = 13) were studied by cardiovascular magnetic resonance (CMR). DMD subject data was stratified based on subject age and LV ejection fraction (EF) into the following groups: A (<12 years old, n = 12); B (>or=12 years old, EF <or= 55%, n = 8) and C (>or=12 years old, EF = 55%, n = 6). Controls were also stratified by age into Groups N1 (<12 years, n = 6) and N2 (>12 years, n = 5). LV mid-slice circumferential myocardial strain (epsilon cc) was calculated using tagged CMR imaging. T2 maps of the LV were generated for all subjects using a black blood dual spin echo method at two echo times. The full width at half maximum (FWHM) was calculated from a histogram of LV T2 distribution constructed for each subject. RESULTS: In DMD subject groups, FWHM of the T2 histogram rose progressively with age and decreasing EF (Group A FWHM= 25.3 +/- 3.8 ms; Group B FWHM= 30.9 +/- 5.3 ms; Group C FWHM= 33.0 +/- 6.4 ms). Further, FWHM was significantly higher in those with reduced circumferential strain (|epsilon cc| <or= 12%) (Group B, and C) than those with |epsilon cc| > 12% (Group A). Group A FWHM was not different from the two normal groups (N1 FWHM = 25.3 +/- 3.5 ms; N2 FWHM= 24.0 +/- 7.3 ms). CONCLUSION: Reduced EF and epsilon cc correlates well with increased T2 heterogeneity quantified by FWHM, indicating that subclinical functional impairments could be associated with pre-existing abnormalities in tissue structure in young DMD patients.
Artículo en PDF |
| Fotografía clínica de miopatía de Duchenne |
En este enlace puede obtener cuatro fotos de DMD Caso clínico de DMD en PDF
Enlace interesante sobre las miopatias hereditarias |
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