NCT
INICIO RECIENTES ARCHIVO COVID-19
INICIO RECIENTES ARCHIVO PUBLICAR INSTRUCCIONES
PARA AUTORES COVID-19 ENTRAR / REGISTRARSE HISTORIA NOSOTROS DIRECTORIO CONTACTO
Guía para el diagnóstico y tratamiento de las alteraciones respiratorias en las enfermedades neuromusculares
I. Antecedentes de la rehabilitación pulmonar en enfermedades neuromusculares
II. Clasificación general de las enfermedades neuromusculares que cursen con alteraciones respiratorias
III. Fisiopatología de las enfermedades neuromusculares
IV. Diagnóstico de las alteraciones respiratorias
V. Tratamiento
VI. Calidad de vida en padecimientos neuromusculares
VII. Cuidados paliativos
VIII. Situación en México de los pacientes con enfermedades neuromusculares y costos





2011, Número 1

2011; 70 (1)






RESUMEN

La medicina mexicana tiene una gran deuda con un grupo de pacientes que han sido y, por desgracia, en muchos casos siguen siendo invisibles para el gremio médico. Podemos mencionar la más frecuente y mortal Distrofia Muscular Duchenne (DMD). Desde que su autor, Guillaume Benjamin Armand Duchenne, describió en 1860 la enfermedad, si bien es cierto que ha habido avances importantes relacionados con el conocimiento de su etiología y su fisiopatología, no ha sido así con su tratamiento, menos aún con su prevención, y por lo que se refiere al tratamiento de sus complicaciones, el desinterés ha sido patente.
El consenso para elaborar la Guía para el Diagnóstico y Tratamiento de las Alteraciones Respiratorias en las Enfermedades Neuromusculares se llevó a cabo precisamente debido a la evidente falta de conocimiento sobre las complicaciones respiratorias de estas enfermedades y su tratamiento, lo mismo que a la consecuente falta de registros y estadísticas, todo lo cual ha generado que en los centros especializados se vea con indiferencia a este tipo de pacientes y se les cierren las puertas de la atención médica.

PALABRAS CLAVE

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REFERENCIAS

  1. Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J 2006;28:549-555.

  2. Uña R, Ureta P, Uña S, Maseda E, Criado A. Ventilación mecánica no invasiva. Rev Esp Anestesiol Reanim 2005;2:88-100.

  3. Rudas F. Ventilación mecánica en urgencias, ASPAME. Accesado en: agosto, 2008. Accesible en: www.aspame.net

  4. Lisboa C, Díaz O, Fadic R. Ventilación mecánica no invasiva en pacientes con enfermedades neuromuscualres y en pacientes con alteraciones de la caja torácica. Arch Bronconeumol 2003;39:314-320.

  5. Jeppesen J, Green A, Steffensen B.F., Rahbek J. The Duchenne muscular dystrophy population in Denmark, 1977-2001: prevalence, incidence and survival in relation to the introduction of ventilator use. Neuromuscul Disord 2003;13:804-812.

  6. Eagle M, Baudouin S, Chandler C, Giddings D, Bullock R, Bushby K. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 2002;12: 926-929.

  7. Alexander MA, Johnson EW, Petty J, Stauch D. Mechanical ventilation of patients with late stage Duchenne muscular dystrophy: management in the home. Arch Phys Med Rehabil 1979;60:289-292.

  8. Torres Y, Vergara P, Hurtados O, Socorro D. Fisioterapia respiratoria en el lesionado medular. Rev Cubana Ortop Traumatol 2001;15:43-45.

  12. López-Pisón, Rebage, Baldellou-Vázquez, et ál. Enfermedades neuromusculares hereditarias en pediatría. Nuestra experiencia de 14 años. Rev Neurol 2005;41:145-150.

  13. Bertorini T. Evaluación de los pacientes enfermedades neuromusculares. Médico Inter Americano 2003;21:2.

  14. López Vega JM, Calleja J, Combarros O, et ál. Motor neuron disease incantabria. Acta Neurol Sacand 1988;77:1-5.

  15. Grupo de Estudio de Enfermedades Neuromusculares. Sociedad Española de Neurología, 2009.

  16. Bach J. Management of patients with neuromuscular disease. In: Hanley and Belfus, editor. New York: Elsevier; 2004.

  17. Guía de las enfermedades neuromusculares. Información y apoyo a las familias, ASEM. Madrid: Formación Alcalá; 2008.

  18. Hill NS. Neuromuscular disease in respiratory and critical care medicine, Respir Care 2006;51:1065-1071.

  19. Ambrosino N, Carpene N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J 2009;34:444-451.

  20. Masdeu MJ, Ferrer A. Función de los músculos respiratorios en las enfermedades neuromusculares. Arch Bronconeumol 2003;39:176-183.

  21. Benditt JO. Initiating noninvasive management of respiratory insufficiency in neuromuscular diseases. Pediatrics 2009;123 Suppl 4:S236-S238.

  22. Howart RS, Davinson C. Long term ventilation in neurogenis respiratory failure. J Neurol Neurosurg Psychiatry 2003;74 Suppl 3:iii24-iii30.

  23. Gozal D. Pulmonary manifestation of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy. Padiatr Pulmono. 2000;29:141-150.

  24. Vega-Briceño L, Contreras I, Prado F. Evaluación respiratoria de la enfermedad neuromuscular en niños. Santiago: Pontificia Universidad Católica de Chile, Hospital Josefina Martínez, Escuela de Medicina.

  25. Bach J, Niranjan V, Weaver B. Spinal Muscular Atrophy Type 1. Chest 2000;117:1100-1105.

  26. Chiarini Testaa MB, Pagliettia MG, Pavonea M, Schiavinoa A, Pedacea C, Cutrera R. Respiratory problems in spinal muscular atrophy in the paediatric age group. Pediatr and Child Health 2009;(19 Supl):S123-S126.

  27. Special considerations in the respiratory Management of Spinal Muscular Atrophy. Pediatrics 2009;123:S245-S249.

  28. Wang CH, Finkel RS, Bertini ES, et ál. Consensus Statement for Standard of Care in Spinal Muscular Atrophy. J Child Neurol 2007;22:1027.

  29. Giannini A, Pinto AM, Rossetti G, Prandi E, Tiziano D, Brahe C y Nardocci N. Respiratory failure in infants due to spinal muscular atrophy with respiratory distress type 1. Intensive Care Med 2006;32:1851-1855.

  30. Goto K, Song MD, Lee JH, Arahata K. Genetic analysis of fascioscapulohumeral muscular dystrophy (FSHD). Rinsho Shinkeigaku 1995;35:12,1416-8.

  31. Hewitt JE., Lyle R, Clark LN, et ál. Analysis of the tandem repeat locus D4Z4 associated with fascioscapulohumeral muscular dystrophy. Hum Mol Genet 1994;3(8):1287-1295.

  32. Chung CS, Morton NE. Discrimination of genetic entities in muscular dystrophy. Am J Human Genetics 1959;11:339-359.

  33. Lunt PW, Harper PS. Genetic counselling in fascioscapulohumeral muscular dystrophy. J Med Genet 1991;28:655-664.

  34. Lunt PW, Jardine PE, Koch MC, et ál. Correlation between fragment size at D4F104S1 and age of onset or at wheelchair use, with a possible generational effect, accounts for much phenotypic variation in 4q35-fascioscapulohumeral muscular dystrophy (FSHD). Molecular Biology and Genetics 1995;4:1243-1244.

  35. Padberg GW. Fascioscapulohumeral disease. Thèse deoctorat. Leiden: Intercontinental Graphics, Université de Leiden, 1982,243.

  36. Stevenson WG, Perloff K, Weiss JN, et ál. Fascioscapulohumeral Muscular Dystrophy: Evidence for selective, genetic electrophysiologic cardiac involvement. J Am Coll Cardiol 1990;15:292-299.

  37. Brouwer OF, Padberg GW, Van der Ploeg RJO, et ál. The influence of handedness on the distribution of muscular weakness of the arm in Fascioscapulohumeral Muscular Dystrophy. Brain 1992;115:1587-1598.

  38. Díaz, Rivière, Barois y cols. Distrophie musculaire facio-scapulo-humérale. AFM, 1999.

  39. Brooke MH. Fascioscapulohumeral dystrophy. En: Brooke MH. A clinician’s view of neuromuscular diseases. 2ª ed. Baltimore: Williams & Wilkins, 1986:158-170.

  40. Siegel IM. Early signs of Landouzy-Dejerine disease: wrist and finger weakness. JAMA 1972;221:302.

  41. Awerbuch GI, Nigro MA, Wishnow R. Beevor’s sign and Fascioscapulohumeral dystrophy. Arch Neurol 1990;47:1208-1209.

  42. Principales enfermedades neuromusculares, Informe Myoline, Evry: Asociación Francesa contra las Miopatías, y Barcelona: Asociación Española contra las Enfermedades Neuromusculares, 2006.

  43. Limb-Girdle, Muscular Dystrophies, MDA, www.mda.org (actualizado a diciembre 2009).

  44. www.nueromusculares.com.ar

  45. ASEM. www.asem-esp.org

  46. Erazo-Torricelli R. Actualización en distrofias musculares. Rev Neurol 2004;39:860-71.

  47. Canavese F, Sussman M. Manifestaciones ortopédicas de Distrofia Miotónica Congénita durante la infancia y la adolescencia. J Pediatrics Orthop 2009;29:208-213.

  48. Rittler M, Feld V, Limontagno M. Distrofia miotónica congénita. Rev Hosp. Mat Inf Ramon Sarda 1997;XVI:34-40.

  49. Wesström G, Bensch J, Schollin J. Congenital Myotonic Dystrophy Incidence, clinical aspects and early prognosis. Acta Paediatr Scand 1986;75:849-54.

  50. www.mda.org/esp-fa-pococomunesMD.html

  51. Volpe JJ. Neurología del recién nacido, 4ª ed. México: McGraw-Hill, 2002:731-745.

  52. Krajewski KM, Shy ME. The floppy infant. Neurology Clinic 2004;22:481-508.

  53. Behrens MI, Jalil P, Serani A, et ál. Possible role of apamin-sensitive K Channels in Myotonic Dystrophy. Muscle and Nerver 1994;17:1264-1270-1294.

  54. Richer L, Shevell M y Miller S. Diagnostic Profile of Neonatal Hypotony: An 11-year study. Pediat Neurol 2001;25:32-37.

  55. Dubowitz V. The floppy Infant. 2ª. ed. Philadelphia: J.B. Lippincott, 1980:33-71.

  56. Prats Viñas JM. Enfoque diagnóstico del niño hipotónico. Revista Pediátrica en línea 2005;2:117-124.

  57. Martínez-Salcedo E, Lloret T, Tarasona JL, Cerdá-Nicolás M. Miopatía Nemalínica Letal y artrogriposis congénita. Rev Neurol 2001;33:552-554.

  58. Jungbluth H. Central Core Disease. Orphanet Journal of Rare Disease 2007;2:25.

  59. Alfonso I, Papazian O, Valencia P. Hipotonía neonatal generalizada. Rev Neurol 2003;37:238-239.

  60. Núñez F. A, Aranguis J, Cattan J, Escobar HR. Síndrome hipotónico del recién nacido. Revista Chilena Pediátrica 2008;79:156-161.

  63. Panitch HB. The pathophysiology of respiratory impairment in pediatric neuromuscular diseases. Pediatrics 2009;Suppl 123:S215-S218.

  64. Benditt J. The neuromuscular respiratory system: physiology, pathophysiology, and a respiratory care approach to patients. Respir Care 2006;51:829-837.

  65. Gibson GJ. Diaphragmatic paresis: pathophysiology, clinical features, and investigation. Thorax 1989;44:960-970.

  66. Galasko CSB, Delaney CM, Williamson JB. Pre-operative spinal stabilization for patients with Duchenne Muscular Dystrophy. Should the minimum pre-operative forced vital capacity be 35%? J Bone Joint Surge Br 1997;79-B Suppl I:111.

  67. Kishnani PS, Steiner RD, Bali D, et ál. Pompe disease diagnosis and management guideline. Genet Med 2006;8:267-288.

  68. Keunen RW, Lambregts PC, Op de Coul AA, et ál. Respiratory failure as initial symptom of acid maltase deficiency. J Neurol Neurosurg Psychiatry 1984; 47:549-552.

  69. Sivak ED, Salanga VD, Wilbourn AJ, et ál. Adult-onset acid maltase deficiency presenting as diaphragmatic paralysis. Ann Neurol 1981;9:613-615.

  70. Sangeetha M. Neuromuscular disease causing acute respiratory failure. Respir Care 2006;51:1016-1023.

  71. West J. Fisiología respiratoria. 7ª ed. Madrid: Médica Panamericana; 2005.p.95-124.

  72. Williams JV, Tierney DF, Parker HR. Surface forces in the lung, atelectasis, and transpulmonary pressure. J Appl Physiol 1966;21:819-827.

  73. Schmidt-Nowara WW, Altman AR. Atelectasis and neuromuscular respiratory failure. Chest 1991,85:792-795.

  74. Estenne M, Gevenois PA, Kinnear W, Soudon P, Heilporn A, De Troyer A. Lung volume restriction in patients with chronic respiratory muscle weakness: the role of microatelectasis. Thorax 1993;48:698-701.

  75. Craig MS. Current concepts of respiratory complications of neuromuscular disease in children. Curr Opin Pediatr 2000;12:203-207.

  76. McCool FD, Mayewski RF, Shayne DS, Gibson CJ, Girgs RC, Hyde RW. Intermitent positive pressure breathing in patients with respiratory muscle weakness. Chest 1986;90:546-552.

  77. Lechtzin N, Shade D, Clawson L, Wiener CM. Supramaximal inflation improves lung compliance in subjects with amyotrophic lateral sclerosis. Chest 2006;129:1322-1329.

  78. Lechtzin N, Shade D, Clawson L, Wiener CM. NIPPV slows lung function decline, improves survival in ALS. Neurology 2006;67:761-765, 736-737 .

  79. Birnkrant DJ, Pope JF, Eiben RM. Management of the respiratory complications of neuromuscular diseases in the pediatric intensive care unit. J Child Neurol 1999;14:139-143.

  80. van den Hout HM, Hop W, van Diggelen OP, et ál. The natural course of infantile Pompe’s disease: 20 original cases compared with 133 cases from the literature. Pediatrics 2003;112:332-340.

  81. Kishnani PS, Hwu WL, Mandel H, et ál. A retrospective, multinational, multicenter study on the natural history of infantile-onset Pompe disease. J Pediatr 2006;148:671-676.

  82. Sivak ED, Salanga VD, Wilbourn AJ, et ál. Adult-onset acid maltase deficiency presenting as diaphragmatic paralysis. Ann Neurol 1981;9:613-615.

  83. Mellies U, Ragette R, Schwake C, et ál. Sleep-disordered breathing and respiratory failure in acid maltase deficiency. Neurology 2005;64:1465-1467.

  84. Bach JR, Saporito LR. Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning. Chest 1996;110:1566-1571.

  85. Kishnani PS, Corzo D, Nicolino M, et ál. Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology 2007;68:99-109.

  86. Nicolino M, Byrne B, Wraith JE, et ál. Clinical outcomes after long-term treatment with alglucosidase alfa in infants and children with advanced Pompe disease. Genet Med 2009;11:210-219.

  87. Strothotte S, Strigl-Pill N, Grunert B, et ál. Enzyme replacement therapy with alglucosidase alfa in 44 patients with late-onset glycogen storage disease type 2: 12-month results of an observational clinical trial. J Neurol 2010;257:91-97.

  88. Case L, Koeberl DD, Young SP, et ál. Improvement with ongoing Enzyme Replacement Therapy in advanced late-onset Pompe disease: A case study. Mol Genet Metab 2008;95:233-235.

  89. McNicholas WT. Impact of sleep in respiratory failure. Eur Respir J 1997;10:920-933.

  90. Berthon-Jones M, Sullivan CE. Ventilatory and arousal responses to hypoxia in sleeping humans. Am Rev Respir Dis 1982;125:632-639.

  91. Baydur A. Respiratory muscle strength and control of ventilation in patients with neuromuscular disease. Chest 1991;99:330-338.

  92. Tusiewicz K, Moldofky H, Bryan AC, Bryan MH. Mechanics of the rib cage and diaphragm during sleep. J Appl Physiol 1977;43:600-602.

  93. De Troyer A, Heilporn A. Respiratory mechanics in quadriplegia. The respiratory function of the intercostal muscles. Am Rev Respir Dis 1980;122:591-600.

  94. Lopes JM, Tabachnik E, Muller NL, et ál. Total airway resistance and respiratory muscle activity during sleep. J Appl Physiol 1983;54:773-777.

  95. Hudges DW, Martin RJ, Johnson B, Hill P. Mechanics of the respiratory system and breathing pattern during sleep in normal humans. J Appl Physiol 1984;56:133-137.

  96. Hetzel MR, Clark TJ. Comparison of normal and asthmatic circadian rhythms in peak expiratory flow rate. Thorax 1980;35:732-738.

  97. Bye PT, Ellis ER, Issa FG, Donnelly PM, Sullivan CE. Respiratory failure and sleep in neuromuscular disease. Thorax 1990;45:241-247.

  98. Finnimore AJ, Jackson RV, Morton A, Lynch E. Sleep hypoxia in myotonic dystrophy and its correlation with awake respiratory function. Thorax 1994;49:66-70.

  99. Barbé F, Quera-Salva MA, McCann C, et ál. Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy. Eur Respir J 1994;7:1403-1408.

  100. Suresh S, Wales P, Dakin C, Harris MA, Cooper D. Sleep-related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population. J Paediatr Chil Health 2005;41:500-503.

  101. International classification of sleep disorders. Diagnostic and coding manual. 2ª ed. Westchester, American Academy of Sleep Medicine; 2005.

  102. Kryger M, Steljes DG, Yee WC, Mate E, Smith SA, Mahowald M. Central sleep apnoea in congenital muscular dystrophy. J Neurol Neurosurg Psychiatry 1991;54:710-712.

  103. Khan Y, Heckmatt JZ. Obstructive apnoeas in Duchenne muscular dystrophy. Thorax 1994;49:157-161.

  104. Coccagna G, Mantovani M, Parchi C, Mironi F, Lugaresi E. Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J Neurol Neurosurg Psichiatry 1975;38:977-984.

  105. Khan Y, Heckmatt JZ, Dubowitz V. Sleep studies and supportive ventilatory treatment in patients with congenital muscle disorders. Arch Dis Child 1996;74:195-200.

  106. Brouilette R, Hanson D, David R, et ál. A diagnostic approach to suspected obstructive sleep apnea in children. J Pediatr 1984;105:10-14.

  107. Alves RS, Resende MB, Skomro RP, Souza FJ, Reed UC. Sleep and neuromuscular disorders in children. Sleep Med Rev 2009;13:133-148.

  108. Culebras A, Kelly JJ. Sleep disorders and neuromuscular disease. Rev Neurol Dis 2008;5:153-158.

  109. Katz SL. Assessment of sleep-disordered breathing in pediatric neuromuscular diseases. Pediatrics 2009;Suppl 123:S222-S225.

  110. Ragette R, Mellies U, Schwake C, Voit T, Teschler A. Patterns and predictors of sleep disordered breathing in primary myopathies. Thorax 2002;57:724-728.

  111. Dohna-Schwake C, Ragette R, Mellies U, Straub V, Teschler H, Voit T. Respiratory function in congenital muscular dystrophy and limb girdle muscular dystrophy 21. Neurology 2004;62:513-514.

  112. Vázquez-García JC, Pérez-Padilla R. Valores gasométricos estimados para las principales poblaciones y sitios a mayor altitud. Rev Inst Nac Enf Respir 2000;13:6-13.

  113. Iber C, Ancoli-Israel S, Chesson A y Quan SF. The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications, Westchester. American Academy of Sleep Medicine; 2007.

  114. Redding GJ, Okamoto GA, Guthrie RD, Rollevson D, Milstein JM. Sleep patterns in nonambulatory boys with Duchenne muscular dystrophy. Arch Phys Med Rehabilit 1985;66:818-821.

  115. Hilton-Jones D. Myotonic dystrophic-forgotten aspects of an often neglected condition. Curr Opin Neurol 1997;10:399-401.

  116. Broughton R, Stuss D, Kates M, Roberts J, Dunham W. Neuropsychological deficits and sleep in myotonic dystrophy. Can J Neurol Sci 1990;17:410-415.

  117. Culebras A, Podolsky S, Leopold NA. Absence of sleep-related growth hormone elevations in myotonic dystrophy. Neurology 1977;27:165-167.

  118. Ono S, Takahashi K, Jinnai K, et ál. Loss of serotonin-containing neurons in the raphe of patients with myotonic dystrophy: a quantitative immunohistochemical study and relation to hypersomnia. Neurology 1998;50:535-538.

  119. Orlikowski D, Chevret S, Quera-Salva MA, et ál. Modafinil for the treatment of hypersomnia associated with myotonic muscular dystrophy in adults: a multicenter, prospective, randomized, double-blind, placebo-controlled, 4-week trial. Clin Ther 2009; 31: 1765-1773.

  120. Terzi N, Orlikowski D, Aegerter P, et ál. Breathing-swallowing interaction in neuromuscular patients: A physiological evaluation. Am J Respir Crit Care Med 2007;175:269-276.

  121. Bleeckx D. Disfagia. Evaluación y reeducación de los trastornos de deglución. Madrid: McGraw Hill;2004.p.5-13.

  122. Shinonaga C, Fukuda M, Suzuki Y, et ál. Evaluation of swallowing function in Duchenne muscular dystrophy. Dev Med Child Neurol 2008,50:478-480.

  123. McMillan AS. Internal organization in the human jaw muscles. Crit Rev Oral Biol Med 1994;5:55-89.

  124. Baldwin KM, Haddad F. Effects of different activity and inactivity paradigms on myosin heavy chain gene expression in striated muscle. J Appl Physol 2001;90:345-357.

  125. Morel-Verdebout C, Botteron S, Kiliaridis S. Dentofacial characteristics of growing patients with Duchenne muscular dystrophy: a morphological study. Eur J Orthod 2007;29:500-507.

  126. Ertekin C, Aydogdu I, Yuceyar N. Piecemeal deglutition and dysphagia limit in normal subjects and in patients with swallowing disorders. J Neurol Neurosurg Psychiatry 1996;61:491-496.

  127. Philpot J, Bagnall A, King C, Dubowitz V, Muntoni F. Feeding problems in merosin deficient congenital muscular dystrophy. Arch Dis Child 1999;80:542-547.

  128. Hamada TM, Kobayashi M, Kawazoe Y, Yamada S. Masseteric silent period in patients with Progressive Muscular Dystrophy. J Dent Res 1981;60:67.

  129. Kawazoe Y, Kobayashi M, Tasaka T, Tamamoto M. Effects of therapeutic exercise on masticatory function in patients with progressive muscular dystrophy. J Neurol Neurosurg Psychiatry 1982;45:343-347.

  130. Baijens LW, Speyer R, Roodenburg N y Manni JJ. The effects of neuromuscular electrical stimulation for dysphagia in opercular syndrome: a case study. Eur Arch Otorhinolaryngol 2008;265:825-830.

  131. Bauer W. Electrical treatment of severe head and neck cancer pain. Arch Otolaryngol 1983;109:382-383.

  132. Belafsky P, Speirs J, Hiss S, Postma G. The safety and efficacy of transcutaneous electrical stimulation in treating dysphagia: preliminary experience. NCSHA. Charleston; 2004.

  133. Blumenfeld L, Hahn Y, Lepage A, Leonard R, Belafsky PC. Transcutaneous electrical stimulation versus traditional dysphagia therapy: a nonconcurrent cohort study. Otolaryngol Head Neck Surg 2006;135:754-757.

  134. Bogaardt HCA, van Dam D, Wever NM, Bruggeman C, Koops J y Fokkens WJ. Efficacy of neuromuscular electrostimulation in the treatment of dysphagia in patients with multiple sclerosis. Amsterdam: Academic Medical Center, University of Amsterdam; 2008.

  135. Boswell N. Neuroelectric therapy eliminates xerostomia during radiotherapy. A case history. Med Electron 1989:105-107.

  136. Boswell N, Bauer W. Noninvasive electrical stimulation for the treatment of radiotherapy side effects. Am J Electromedicine 1985;2.

  137. Bulow M, Speyer R, Baijens L, Woisard V, Ekberg O. Neuromuscular Electrical Stimulation (NMES) in stroke patients with oral and pharyngeal dysfunction. Dysphagia 2008;23:302-309.

  138. Carnaby-Mann GD, Crary MA. Adjunctive neuromuscular electrical stimulation for treatment-refractory dysphagia. Ann Otol Rhinol Laryngol 2008;117:279-287.

  139. Carnaby-Mann GD, Crary MA. Examining the evidence on neuromuscular electrical stimulation for swallowing: a meta-analysis. Arch Otolaryngol Head Neck Surg 2007;133:564-571.

  140. Chaudhuri G, Brady S, Caldwell R. Electric stimulation for dysphagia following stroke: Pilot data. Arch Phys Med Rehabil 2006;87:e51.

  141. Crary MA, Carnaby-Mann GD, Faunce A. Electrical stimulation therapy for dysphagia: descriptive results of two surveys. Dysphagia 2007;22:165-173.

  142. Dobbs, MB, Lenke LG. Neuromuscular scoliosis. St. Louis Shriners Hospital, 2008.

  143. Díez U. Patología de la columna: prevención y tratamiento. Bol Pediatr 2007;47 Supl 1:53-58.

  144. Molina A, Ramírez M, García-Casas O, et ál. Nuevas pers­pectivas en el tratamiento quirúrgico de las enfermedades graves de la caja torácica que cursan con afectación respiratoria. Arch Bronconeumol 2003;39:507-513.

  145. González LJ, Soleto M, López VS, Navascués RJ, Cervera BP. Resultados de la instrumentación Galveston-Luque en la escoliosis neuromuscular. Rev Ortop Traumatol 2002;3:227-233.

  146. Finder JD, Birnkrant D, Carl J, et ál. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004;170:456-465.

  147. García Fontecha CG. Escoliosis. Accesible en: www.traumatologiainfantil.com

  150. Jenkins JG, Bohn D, Edmonds JF, et ál. Evaluation of pulmonary function in muscular dystrophy patients requiring spinal surgery. Crit Care Med 1982;10: 645-649.

  151. Phillips MF, Quinlivan RC, Edwards RH, Calverley PM. Changes in spirometry over time as a prognostic marker in patients with Duchenne musculary dystrophy. Am J Respir Crit Care Med 2001;164:2191-2194.

  152. Gibson GJ. Sipometry: then and now. Breathe 2005;2:270.

  153. Wanger J, Clausen JL, Coates A, et ál. Standardization of the measurement of lung volumes. Eur Respir J 2005;26:511-522.

  154. Sharma GD. Pulmonary function testing in neuromuscular disorders. Pediatrics 2009;123 Suppl 14:S219-S221.

  155. DePalo V, McCool FD. Respiratory muscle evaluation of the patient with neuromuscular disease. Semin Respir Crit Care Med 2002;23:201-209.

  156. Antelo L, Martínez C, Barrio GA. Manifestaciones respiratorias en las enfermedades neuromusculares. En: Cobos N, Pérez-Yarza EG, editores. Tratado de neumología infantil. Madrid: Ergon;2003.p.883-891.

  157. Casado F, Martínez A. Métodos de medida de los gases sanguíneos. En: Ventilación mecánica en recién nacidos, lactantes y niños. Madrid: Ergon; 2004.p.145-151.

  158. Bach JR, Ishikawa Y, Kim H, et ál. Prevention of pulmonary morbidity for patients with Duchene muscular dystrophy. Chest 1997;112:1024-1028.

  159. Langer A. Ventilatory management in muscular dystrophies. Pediatr Pneumol 2000;29:141-150.

  160. Maquilón C. Ventilación no invasiva en pacientes con enfermedades neuromusculares. Consenso Chileno de ventilación no invasiva. Rev Chil Enf Respir 2008;24:192-198.

  161. Misuri G, Lanini B, Gigliotti F, et ál. Mechanism of CO(2) retention in patients with neuromuscular disease. Chest 2000;117:447-453.

  162. Miller RG, Jackson CE, Kasarskis EJ, et ál. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009;73:1218-1226.

  163. Phillips MF, Smith PE, Carroll N, et ál. Nocturnal oxygenation and prognosis in Duchenne muscular dystrophy. Am J Respir Crit Care Med 1999;160:198-202.

  164. Szeinberg A, Tabachnik E, Rashed N, et ál. Cough capacity in patients with muscular dystrophy. Chest 1988;94:1232-1235.

  165. Griggs RC, Donohoe KM, Utell MJ, et ál. Evaluation of pulmonary function in neuromuscular disease. Arch Neurol 1981;38:9-12.

  166. Bimkrant DJ, Panitch HB, Benditt JO, et ál. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest 2007;132:1997-1986.

  167. Nicot F, Hart N, Forin V, et ál. Respiratory muscule testing: a valuable tool for children with neuromuscular disorders. Am J Respir Crit Care Med 2006;174:67-74.

  168. Kang SW, Kang YS, Sohn HS, Park JH, Moon JH. Respiratory Muscle strength and cough capacity in patients with Duchenne Muscular Dystrophy. Yonsei Med J 2006;47:184-190.

  169. Kang SW. Pulmonary rehabilitation in patients with neuromuscular disease. Yonsei Med J 2006;47:307-314.

  170. Sanderds WB. Text book of respiration medicine, 3ª ed. Philadelphia; 2000:553-566.

  171. Cesar M, Castillo S, Montiel G. Ventilación no invasiva en pacientes con enfermedades neuromusculares. Rev Chil Enf Resp 2008;24:192-198.

  172. Bach JR, Gonçalves MR, Páez S, et ál. Expiratory flow maneuvers in patients with neuromuscular diseases. Am J Phys Med Rehabil 2006;85:105-111.

  173. García Fontecha CG. Escoliosis. Accesible en: www.traumatologiainfantil.com (marzo de 2008).

  174. Gupta MC, Wijesekera S, Sossan A, et ál. Reliability of radiographic parameters in neuromuscular scoliosis. Spine (Phila Pa 1976) 2007;32:691-965.

  175. Smith AD, Koreska J, Moseley CF. Progression of scoliosis in Duchenne muscular dystrophy. J Bone Joint Surg Am 1989;71:1066-1074.

  176. Theodoros BG, Geoffrey RB, Elias SV, John KW. A segmental radiological study of the spine and rib – cage in children with progressive infantile idiopathic scoliosis. Scoliosis 2006;1:17.

  177. Díaz JJ, Schröter GC, Shultz IR. Actualización de la evaluación radiológica de la escoliosis. Rev Chil Radiol 2009;15:141-151.

  178. Van Herwaarden MA, Smout AJ. Diagnosis of reflux disease. Baillieres Best Pract Res Clin Gastroenterol 2000;14:759-774.

  179. Bodger K, Trudgill N. Guidelines for Oesophageal Manometry and pH Monitoring. British Society of Gastroenterology. Guidelines in Gastroenterology. November; 2006.

  180. Moayyedi P, Talley NJ. Gastro-oesophageal reflux disease. Lancet 2006;367:2086-2100.

  181. Armstrong D, Marshall JK, Chiba N, et ál. Canadian Consensus Conference on the management of gastroesophageal reflux disease in adults - update 2004. Can J Gastroenterol 2005;19:15-35.

  182. DeVault KR, Castell DO; American College of Gastroenterology. Updated guidelines for the diagnosis and treatment of gastroesophageal reflux disease. Am J Gastroenterol 2005;100:190-200.

  183. Tutuian R, Vela MF, Shay SS, Castell DO. Multichannel Intraluminal Impedance in esophageal function testing and gastroesophageal reflux monitoring. J Clin Gastroenterol 2003;37:206-215.

  187. AARC clinical practice guideline. Directed cough. American Association of Respiratory Care. Respir Care 1993;38:495-499.

  188. Thompson BJ. The physioterapist’s role in rehabilitation of the asthmatic. NZJ Physiotherapy 1973;4:11-16.

  189. Suttton PP, Parker RA, Webber BA, et ál. Assessment of the forced expiration technique, postural drainage and directed coughing in chest physiotherapy. Eur J Respir Dis 1983;64:62-68.

  190. Christensen EF, Nedergaard T, Dahl R. Long-term treatment of chronic bronchitis with positive expiratory pressure mask and chest physiotherapy. Chest 1990;97:645-650.

  191. Oldenburg FA, Dolovich MB, Montgomery JM, Newhouse MT. Effects of postural drainage, exercise and cough on mucus clearance in chronic bronchitis. Am Rev Respir Dis 1979;120:739-745.

  192. Hasani A, Pavia D, Agnew JE, Clarke SW. Regional lung clearance during cough and forced expiration technique (FET): effects of flow and viscoelasticity. Thorax 1994;49:557-561.

  193. van Hengstum M, Festen J, Beursken SC, et ál. The effect of positive expiratory pressure versus forced expiration technique on traqueobronchial clearance in chronic bronchitics. Scand J Gastroenterol Suppl 1988;143:114-118

  194. Fink JB. Forced expiratory technique, directed cough, and autogenic drainage. Respir Care 2007;52:1210-1223.

  195. McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest 2006;129(1 Suppl):250S-259S.

  196. McCrory DS, Lewis SZ. Methodology and grading of the evidence for the diagnosis and management of cough: ACCP evidence-based clinical practice guidelines. Chest 2006;129(1 Suppl):28S-32S.

  197. Miller RG, Jackson CE, Kasarskis EJ, et ál. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis. Neurology 2009;73:1218-1226.

  198. Global Physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines. Chest 2006;129(1 Suppl):48S- 53S.

  199. Fauroux B, Guillemont N, Aubertin G, et ál. Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. Chest 2008;133:161-168.

  200. Barach AL, Beck GJ. The ventilatory effects of the head-down position in pulmonary emphysema. Am J Med 1954;16:55-60.

  201. Celli BR. Respiratory muscle function. Clin Chest Med 1986;7:567-584.

  202. Dail CW, Affeldt JE, Collier CR. Clinical aspects of glossopharyngeal breathing; report use one hundred postpoliomyetic. J Am Med Assoc 1955;158:445-449.

  203. Ambrosino A, Foglio K. Selection criteria for pulmonary rehabilitation. Respir Med 1996;90:317-322.

  204. Dail CW. “Glossopharyngeal breathing” by paralyzed patients: a preliminary report. Calif Med 1951;75:217-218

  205. Boitano LJ. Management of airway clearance in neuromuscular disease. Respir Care 2006;51:913-922.

  206. Avendaño M, Güell R. Rehabilitación en pacientes con enfermedad neuromuscular y con deformidades en la caja torácica. Arch Bronconeumol 2003;39:559-565.

  207. ATS. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004;170:456-465.

  208. McCool FD, Tzelepis GE. Inspiratory muscle training in the patient with neuromuscular disease. Phys Ther 1995;75:1006,1014.

  209. Bach JR. Lung inflation by glossopharingeal breathin and “air stacking” in Duchenne muscular dystrophy. Am J Phys Med Rehabil. 2007;86:295-300.

  210. Giménez Servera, Diferentes técnicas de drenaje bronquial; descripción, aplicación y estudios funcionales. Prevención y rehabilitación en patología respiratoria crónica. 2ª ed. Madrid: Interamericana; 2004.p.173-184.

  211. Harries JR, Lawes WE. The advantages of glossopharyngeal breathing. Br Med J 1957;2:1204-1205.

  212. Lange DJ, Lechtzin N. High-frequency chest wall oscillation in ALS: an exploratory randomized, controlled trial. Neurology 2006;67:991-997.

  213. Gozal D. Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy. Pediatr Pulmonol 2000;29:141-150.

  214. McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest 2006;129(1 Suppl):250S-259S.

  215. Metcalf VA. Vital capacity and glossopharyngeal breathing in traumatic quadriplegia. Phys Ther 1966;46:835-838.

  216. Kang SW, Bach JR. Maximum insufflation capacity. Chest 2000;118:61-65.

  217. Ambrosino N, Carpenè N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J 2009;34:444-451.

  218. Pryor JA. Physiotherapy for airway clearance in adults. Eur Respir J 1999;14: 1418-1424.

  219. Chatburn RL. High-frequency assisted airway clearance. Respir Care 2007; 52:1224-1235.

  220. Ugalde V. Pursed lips breathing improves ventilation in myotonic muscular dystrophy. Arch Phys Med Rehabil 2000;81:472-478.

  221. Vincken W. Detection of upper airway muscle involvement in neuromuscular disorders using the flow-volume loop. Chest 1986;90:52-57.

  222. Denehy L. The use of manual hyperinflation in airway clearance. Eur Respir J 1999;14:958-965.

  223. Maxwell LJ, Ellis ER. The effect on expiratory flow rate of maintaining bag compression during manual hyperinflation. Aust J Physiother 2004;50:47-49.

  224. Racca F, Del Sorbo L, Mongini T, Vianello A, Ranieri VH. Respiratory management of acute respiratory failure in neuromuscular diseases. Minerva Anestesiol 2010;76:51-62.

  225. Perrin C, Unterborn J, Ambrosio C, Hill NS. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve 2004; 29:5-27.

  226. Panitch HB. Respiratory issues in the management of children with neuromuscular disease. Respir Care 2006;51:885-893.

  227. Kang SW. Pulmonary rehabilitation in patients with neuromuscular disease. Med J 2006;47:307-314.

  228. Savian C, Paratz J, Davies A. Comparison of the effectiveness of manual and ventilator hyperinflation at different levels of positive end-expiratory pressure in artificially ventilated and intubated intensive care patients. Heart Lung 2006;35: 334-341.

  229. Hodgson C, Ntoumenopoulos G, Dawson H, Paratz J. The Mapleson C circuit clears more secretions than the Laerdal circuit during manual hyperinflation in mechanically-ventilated patients: a randomized cross-over trial. Aust J Physiother 2007;53:33-38.

  230. Berney S, Denehy L, Pretto J. Head-down tilt and manual hyperinflation enhance sputum clearance in patients who are intubated and ventilated. Aust J Physiother 2004;50:9-14.

  231. Siu-Ping J, Yee-Men AJ. Effects of manual hyperinflation and suctioning on respiratory mechanics in mechanically ventilated patients with ventilator-associated pneumonia. Aust J Physiother 2005;51:25-30.

  232. Coccagna G, Manturani M, Parchi C, Mironi F, Lugaresi E. Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J Neurol Neurosurg Psichiatry 1975;38:977-984.

  233. Mellies V, Ragethe R, Duhna-Schwake C, Boehm H, Voit T, Teschler H. Long-term non invasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J 2003;27:631-636.

  234. Guilleminault C, Philip P, Robinson A. Sleep and neuromuscular disease: bilevel positive airway pressure by nasal mask as a treatment for sleep disordered breathing in patients with neuromuscular disease. J Neurol Neurosurg Psychiatry 1998;65:225-232.

  235. Ambrogio C, Lowman X, Kuo M, Malo J, Prasad AR, Parthasarathy S. Sleep and non-invasive ventilation in patients with chronic respiratory insufficiency. Intensive Care Med 2009;35:306-313.

  236. Jaye J, Chatwin M, Dayer M, Morell MJ, Simonds AK. Autotitrating versus standard noninvasive ventilation: a randomized crossover trial. Eur Repir J 2009;33:566-573.

  237. Khan Y, Heckmatt JZ. Obstructive apnoeas in Duchenne muscular dystrophy. Thorax 1994;49:157-161.

  238. McNicholas WT, Carto JL, Rutherford R, Zamel N, Phillipson EA. Beneficial effects of oxygen in primary alveolar hypoventilation with central sleep apnea. Am Rev Respir Dis 1982;125:773-775.

  239. Goldstein RS, Ramcharan V, Bowes G, McNicholas WT, Bradley D, Phillipson EA. Effects of supplemental oxygen on gas exchange during sleep in patients with severe obstructive lung disease. N Engl J Med 1984;310:425-429.

  240. Finder JD, Birnkrant D, Carl J, et ál. Respiratory care of the patient with Duchenne Muscular Dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004;170:456-465.

  241. Miller RG. The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review): Report of the Quality Standards Subcommitte of the American Academy of Neurology. Neurology 2009;73:1218-1226.

  242. Shneerson JM. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J 2002;20:480-487.

  243. Hill NS. Ventilator management for neuromscular disease. Semin Respir Crit Care Med 2002;23:293-305.

  244. Pérez-Padilla JR, Vázquez-García JV. Estimation of gasometric values at different altitudes above sea level in Mexico. Rev Invest Clin 2000;52:148-155.

  245. Wallgren-Pettersson C, Bushby K, Mellies U, Simonds A; ENMC. 117th ENMC workshop: ventilatory support in congenital neuromuscular disorders -- congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II) 4-6 April 2003, Naarden, The Netherlands. Neuromuscul Disord 2004;14:56-69.

  246. Hess DR. Non invasive ventilation in neuromuscular disease: equipment and application. Respir Care 2006;51:896-912.

  247. Hart N, Hunt A, Polkey MI, Fauroux B, Lofaso F, Simonds AK. Comparison of proportional assist ventilation and pressure support ventilation in chronic respiratory failure due to neuromuscular and chest wall deformity. Thorax 2002;57:979-981.

  248. Porta R, Appendini L, Vitacca M, et ál. Mask proportional assist vs. pressure support ventilation in patients in clinically stable condition with chronic ventilator failure. Chest 2002;122:479-488.

  249. Winck JC, Vitacca M, Morais A, et ál. Tolerance and physiologic effects of nocturnal mask pressure support vs. proportional assist ventilation in chronic ventilatory failure. Chest 2004;126:382-388.

  250. Miyoshi E, Fujino Y, Uchiyama A, Mashimo T, Nishimura M. Effects of gas leak on triggering function, humidification, and inspiratory oxygen fraction during noninvasive positive airway pressure ventilation. Chest 2005;128:3691-3698.

  251. Prinianakis G, Delmastro M, Carlucci A, Ceriana P, Nara S. Effect of varying the pressurisation rate during noninvasive pressure support ventilation. Eur Respir J 2004;23:314-320.

  252. Mehta S, Hill NS. State of the art, noninvasive ventilation. Am J Respir Crit Care Med 2001;163:540-577.

  253. Navalesi P, Fanfulla F, Frigeiro P, Gregoretti C, Nava S. Physiologic evaluation of non invasive mechanical ventilation delivered with three types of masks in patients with chronic hypercapnic respiratory failure. Chest 2000;28:1785-1790.

  254. Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest 1993;103:174-182.

  255. Hill NS. Neuromuscular disease in respiratory and critical care medicine. Conference summary. Respir Care 2006;51:1065-1071.

  256. West J. Fisiología respiratoria. 7ª ed. Madrid: Médica Panamericana; 2005.p.95-124.

  257. Nash G, Bowen JA, Langlinais PC. ”Respirator lung”: a misnomer. Arch Pathol 1971;91: 234-40.

  258. Dreyfuss D, Saumon G. Ventilator-induced lung injury: lessons from experimental studies. Am J Respir Crit Care Med 1998;157:294-323.

  259. Folz RJ, Abushamaa AM, Suliman HB. Extracellular superoxide dismutase in the airways of transgenic mice reduces inflammation and attenuates lung toxicity following hyperoxia. J Clin Invest 1991;103:1055-1066.

  260. Lyall RA, Donaldson N, Fleming T, et ál. A prospective study of quality of life in ALS patients treated with noninvasive ventilation. Neurology 2001;57:153-156.

  261. Melo J, Homma A, Iturriaga E, et ál. Pulmonary evaluation and prevalence of non-invasive ventilation in patients with amyotrophic lateral sclerosis: a multicenter survey and proposal of a pulmonary protocol. J Neurol Sci 1999;169:114-117.

  262. Hernández C, Bergeret JC. Traqueostomía: principios y técnica quirúrgica. Cuad Cir 2007;21:92-98.

  263. Make BJ, Hill NS, Goldberg AI, Bach JR, Criner GJ, Dunne PE, et ál. Mechanical ventilation beyond the intensive care unit. Report of a consensus conference of the American College of Chest Physicians. Chest 1998;113(5 Suppl):289S-344S.

  264. Rocke DA, Murray WB, Rout CC, Gouws E. Relative risk analysis of factors associated with difficult intubation in obstetric anesthesia. Anesthesiology 1992;77:67-73.

  265. Orlikowski D, Prigent H. Noninvasive ventilation as an alternative to endotracheal intubation during tracheotomy in advanced neuromuscular disease. Respir Care 2007;52:1728-1733.

  266. Steven M, Whitaker A. Medical and supportive care among people with ALS in the months before death or tracheostomy. J Pain Symptom Manage 2009; 38: 546-553.

  267. Servera E, Sancho J, Zafra MJ, Catalá A, Vergara P, Marín J. Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil 2005;84:851-857.

  268. Sveean ML, Joppesen TD, Hauerslev S, Køber L, Krag TO, Vissing J. Endurence trainin improus fitness and strength in patients whit Beker muscular dystrophy. Brain 2008;131:2824-2831.

  269. Pieterse AJ, Ten Broek-Pastoo JM, Munneke M, et ál. Exercise therapy and other types of physical therapy for patients with neuromuscular diseases: a systematic review. Arch Phys Med Rehab 2007;88:1452-1464.

  270. Conrads VM, Beckers PJ, Vorlat A, Vrints CJ. Importance of physical rehabilitation before and after cardiac transplantation in a patients with myotonic dystrophy: a case report. Arch Phys Med Rehab 2002;83:724-726.

  271. Eagle M. Report on the muscular dystrophy campaing workshop: exercise in neuromuscular diseases Newcastle. Neuromuscul Disord 2002;12:975-983.

  272. Stöllberger C, Finsterer J, Keller H, Mamole B, Stanly J. Progression of cardiac involvement in patients with myotonic dystrophy and mitochondrial myopathy. Cardiology 1998;90:173-179.

  273. Humbert IA, Poletto CJ, Saxon KG, et ál.The effect of surface electrical stimulation on hyolaryngeal movement in normal individuals at rest and during swallowing. J Appl Physiol 2006;101:1657-1663.

  274. Lagorio LA, Carnaby-Mann GD, Crary MA. Cross-system effects of dysphagia treatment on dysphonia: a case report. Cases J 2008;1:67.

  275. Leelamanit V, Limsakul C, Geater A. Synchronized electrical stimulation in treating pharyngeal dysphagia. Laryngoscope 2002;112:2204-2210.

  276. Ludlow CL, Humbert I, Saxon K, Poletto C, Sonies B, Crujido L. Effects of surface electrical stimulation both at rest and during swallowing in chronic pharyngeal dysphagia. Dysphagia 2007;22:1-10.

  277. Shaw GY, Sechtem PR, Searl J, Keller K, Rawi TA, Dowdy E. Transcutaneous neuromuscular electrical stimulation (VitalStim) curative therapy for severe dysphagia: myth or reality? Ann Otol Rhinol Laryngol 2007;116:36-44.

  278. Suiter DM, Leder SB, Ruark JL. Effects of neuromuscular electrical stimulation on submental muscle activity. Dysphagia 2006;21:56-60.

  279. Dieter Heller K, Dieter U, Wirtz C, Siebert CH, Forst R. Spinal stabilization in Duchenne Muscular Dystrophy: Principles of treatment and record of 31 operative treated cases. J Pediatric Orthopaedics B 2001;10:18-24.

  280. Galasko, CSB, Delaney CM, Williamson JB. Pre-operative spinal stabilization for patients with Duchenne Muscular Dystrophy. Should the minimum pre-operative forced vital capacity be 35%? J Bone Joint Surge Br 1997;79-B (Supl I):111S.

  281. Kim YJ, Lenke LG, Bridwell KH, et ál. Pulmonary function in adolescent idiopathic scoliosis relative to the surgical procedure. J Bone Joint Surg Am 2005;87:1534-1541.

  282. Yaszay B, Jazayeri R, Lonner B. The effect of surgical approaches on pulmonary functioning adolescent idiopathic scoliosis. J Spinal Disord Tech 2009;22:278-283.

  283. Mubarak SJ, Morin WD, Leach J. Spinal fusion in Duchenne muscular dystrophy -fixation and fusion to the sacropelvis? J Pediatr Orthop 1993;13: 752-757.

  287. Yusen R. Wath outcomes should be meassured in patients with copd? Chest 2001;119:327.

  288. Jones P. Issues concerning health-related quality of life in COPD. Chest 1995;107 (Supl):187S-193S.

  289. AACCP/AACVPR. Pulmonary Rehabilitation Guidelines in COPD. Chest 1997;112:1363-1396.

  290. Boyer F. Calidad de vida relacionada a la salud en personas con enfermedades neuromusculares hereditarias. Desórdenes Neuromusculares 2006;16:99-106.

  291. Wallack Z. Pulm Rehab Guideliness to Succes, 3ª ed. Philadelphia: Lippincott, Williams & Wilkins

  292. Ahlström G. Disability and quality of life in individuals with muscular dystrophy. Scand J Rehab Med 1996;28:147-157.

  293. 7 Ware. Monitoring health outcomes from the patient’s point of view. Wisconsin: University of Wisconsin Medical School; 1995.

  294. Kohler M. Quality of life, physical disability, and respiratory impairment in Duchenne muscular dystrophy. Am J Respir Crit Care Med 2005;172:1032-1036.

  295. Markström A, Sundell K, Lysdahl M, Andersson G, Schedin U, Klang B. Quality of life evaluation of patients with neuromuscular and skeletal diseases treated with noninvasive and invasive home mechanical ventilation. Chest 2002;122:1695-1700.

  296. Kang SW. Pulmonary rehabilitation in patients with neuromuscular disease. Yonsei Med J 2006;47: 307-314.

  297. Jones PW, Quirk FH, Baveystock CM, Littlejohns P. A self-complete measure of health status for chronic airflow limitation. The St. George’s Respiratory Questionnaire. Am Rev Respir Dis 1992;145:1321-1327.

  298. Sivori M. Nuevo Consenso Argentino de Rehabilitación Respiratoria. Actualización 2008. Medicina 2008;68: 325-344.

  299. Velarde-Jurado E. Evaluación de la calidad de vida. Salud Pública Mex 2002; 44:349-361.

  302. Tournay N. An introduction to pediatric palliative care. Clin Family Practice 2004;6:499-516.

  303. Robert Wood Johnson Foundation. Promoting Excellence in End-of-Life Care HIV/AIDS. Fecha de acceso: marzo, 2007. Accesible en: http://www.promotingexcellence.org/files/public/hiv_report.pdf

  304. Robins J, Colbert A, Scott J. Ventilator dependency: Decision-making, daily functioning and quality of life for patient wit Duchenne muscular dystrophy. Dev Med Child Neurol 1990;32:1078-1086.

  305. Brushby K, Finkel R, Birnkrant D, Case L, Clemens P, Cripe L. Diagnosis and management of Duchenne Dystrophy, part 1: Diagnosis, and pharmacological and psychosocial management. Lancet 2010;9.

  306. Quiles MJ, van der Hofstadt CJ, Quiles Y. Instrumentos de evaluación del dolor en pacientes pediátricos: una revisión (2ª parte). Rev Soc Esp Dolor 2004;11:360-369.

  307. 20 Aniversario de la Publicación de la Escalera Analgésica de la OMS. Cancer Pain Release 2006;19.

  308. Lamelo AF. Control de síntomas en cuidados paliativos. Guías clínicas 2004; 4.

  309. Libran A. Síntomas digestivos. En: Porta J, Gómez-Batiste X, Tuca A, editores. Control de síntomas en pacientes con cáncer avanzado y terminal. 2005.p.91-129

  310. Barboza FM. Evaluación y tratamiento del estreñimiento en niños. Colombia Médica 2005;36(1 Supl):10-15.

  311. Estudio comparado España-Argentina “EM: realidad, necesidades sociales y calidad de vida”. Accesible en: www.msif.org/es/publications/principios_para_promover_la_calidad_de_vida/index.htm

  312. Ley General de Salud, Artículo 166 bis, DOF: 05/01/2009. Gaceta Oficial del Distrito Federal 2008, No. 247.

  316. Domínguez ME. Rehabilitación pulmonar. En: Cano VF, editor. Enfermedades respiratorias. Madrid: Elsevier; 2006.p.391-400.

  317. Domínguez ME. Hipoventilación asociada a trastornos extrapulmonares. En: Diagnóstico y tratamiento en neumología. México: Manual Moderno; 2008.p.495-512.

  318. Domínguez FME. Rehabilitación pulmonar en las enfermedades neuromusculares. En: Pérez F, editor. Neumología pediátrica (en prensa).


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