000			01751na a2200253 4500
003			PC3635
005			20180417114629.0
008			130622s2011 xxx||||| |||| 00| 0 eng d
040			_cH12O
041			_aeng
100			_aAllende Martínez, Luis Miguel _91508 _eInmunología
100			_aRamos Amador, José Tomás _91027 _ePediatría
245	0	0	_aPFAPA syndrome in siblings. Is there a genetic background? _h[artículo]
260			_bEuropean Journal of Pediatrics, _c2011
300			_a170(12):1563-1568.
500			_aFormato Vancouver: Antón-Martín P, Ortiz Movilla R, Guillén Martín S, Allende LM, Cuesta Rubio MT, López González MF, et al. PFAPA syndrome in siblings. Is there a genetic background? Eur J Pediatr. 2011;170(12):1563-8.
501			_aPMID: 21537926
504			_aContiene 22 referencias.
520			_a"PFAPA syndrome" is an autoinflammatory entity composed of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. There have been many reports of children with the disease, but only occasionally have been described in siblings, and no specific genetic mutation has been determined yet. Corticosteroids are the mainstay in the treatment of the acute attacks. The role of surgery in long-term follow-up (tonsillectomy with or without adenoidectomy) is controversial. We report two brothers affected with the syndrome, in whom corticosteroids as the only treatment led to an improvement. A genetic work-up was performed, making very unlikely other possible syndromes of recurrent fever.
710			_9395 _aServicio de Inmunología
710			_9446 _aServicio de Pediatría-Neonatología
856			_uhttp://pc-h12o-es.m-hdoct.a17.csinet.es/pdf/pc3635.pdf _ySolicitar documento
942			_2ddc _cART _n0
999			_c3635 _d3635