caa a22 4500 467934908 CHVBK 20180406152954.0 cr unu---uuuuu 170328e20060801xx s 000 0 eng 10.1007/s00408-005-2584-x doi (NATIONALLICENCE)springer-10.1007/s00408-005-2584-x Finsterer Josef Krankenstalt Rudolfstiftung, Postfach 20, 1180, Vienna, Austria aut Cardiopulmonary Support in Duchenne Muscular Dystrophy [Elektronische Daten] [Josef Finsterer] Duchenne muscular dystrophy (DMD) is an X-linked, rapidly progressive myopathy affecting the limb muscles, the respiratory muscles, the heart, the intestines, and the brain. Since about 90% of DMD patients die from muscular respiratory failure or cardiomyopathy, early and adequate therapy is essential. Ventilatory failure from muscle weakness requires mechanical support for ventilation and coughing as soon as there is symptomatic nocturnal hypoventilation. Today noninvasive positive-pressure ventilation (NIPPV) is the method of choice for supportive long-term mechanical ventilation in DMD. For assisted coughing, various methods are available, among which the mechanical in-exsufflator is the most widely used device. There is large nonrandomized clinical trial evidence that NIPPV improves quality of life and prolongs the lives of DMD patients if medical, social, economic, and ethical issues, raised by the availability of long-term NIPPV, are adequately addressed. Cardiac involvement in DMD manifests as impulse generation or impulse conduction abnormalities or cardiomyopathy. Cardiac abnormalities in DMD respond well to adequate therapy. Though DMD is ultimately a fatal disease, quality of life and life expectancy can be markedly improved if cardiopulmonary manifestations are adequately treated. Springer Science+Business Media, Inc., 2006 Respiratory failure nationallicence Mechanical ventilation nationallicence Neuromuscular nationallicence Myopathy nationallicence Heart failure nationallicence Lung Springer-Verlag; www.springer-ny.com 184/4(2006-08-01), 205-215 0341-2040 184:4<205 2006 184 408 https://doi.org/10.1007/s00408-005-2584-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00408-005-2584-x text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Finsterer Josef Krankenstalt Rudolfstiftung, Postfach 20, 1180, Vienna, Austria aut NATIONALLICENCE 773 0- Lung Springer-Verlag; www.springer-ny.com 184/4(2006-08-01), 205-215 0341-2040 184:4<205 2006 184 408 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer