000			nab a22 7a 4500
999			_c17045 _d17045
003			PC17045
005			20221102114603.0
008			221102b xxu||||| |||| 00| 0 eng d
040			_cH12O
041			_aspa, eng
100			_91074 _aGutiérrez Rivas, Eduardo _eNeurología
245	0	0	_aGuía para el seguimiento de la enfermedad de Pompe de inicio tardío. _h[guía clínica]
260			_bRevista de neurología, _c2015
300			_a60(7):321-8.
500			_aFormato Vancouver: Gutiérrez Rivas E, Illa I, Pascual Pascual SI, Pérez López J, Vílchez Padilla JJ, Bautista Lorite J et al; Sociedad Española de Medicina Interna; Sociedad Española de Neurología; Sociedad Española de Neumología y CirugíaTorácica. Guía para el seguimiento de la enfermedad de Pompe de inicio tardío. Rev Neurol. 2015 Apr 1;60(7):321-8.
501			_aPMID: 25806482
504			_aContiene 47 referencias
520			_aAlthough treatment with alglucosidase alfa has helped improve the prognosis of patients with late-onset Pompe disease, both the development of the disease and the effectiveness of the treatment need to be monitored on a regular basis. This is the reason that has led a committee of Spanish experts to draw up a series of guidelines on how to follow up these patients. The committee proposes a model of follow-up tests for late-onset Pompe disease. First of all, the nutritional status and swallowing function must be evaluated. Second, and due to the variability of the clinical features, the committee recommends the simultaneous use of several scales to measure different functions and parameters. Thus, muscular force is assessed with the Medical Research Council scale; motor functioning, with the six-minute walk test and timed tests; disability, with the Rasch-built Pompe-specific Activity scale; respiratory functioning, with measurement of the forced vital capacity and oxygen saturation; and fatigue, with the fatigue intensity scale. Lastly, the safety and tolerability of enzyme replacement therapy are controlled by registering and treating the potential side effects and measurement of the anti-alglucosidase alfa antibodies. A number of different general recommendations are also included.
710			_9267 _aServicio de Neurología-Neurofisiología
856			_uhttp://pc-h12o-es.m-hdoct.a17.csinet.es/pdf/pc/1/pc17045.pdf _ySolicitar documento
942			_2ddc _cGUI _n0