000			02684nam a22003735i 4500
001			82487
003			ES-MaUEC
005			20230207040413.0
007			cr nn 008mamaa
008			151012s2015 gw | s |||| 0|eng d
020			_a9783319227146
040			_aES-MaUEC
050		4	_aRC606 _b2015 EB
082	0	4	_a616.079
245	0	0	_aAgammaglobulinemia _cedited by Alessandro Plebani, Vassilios Lougaris
250			_a1st ed.
260			_aCham, Switzerland _bSpringer _c2015
300			_a1 recurso en línea (IX, 119 p.) 22 il., 18 il. col.
336			_aTexto (visual) _btxt _2rdacontent
337			_aelectrónico _bc _2rdamedia
338			_arecurso electrónico _bcr _2rdacarrier
338			_aonline resource _bcr _2rdacarrier
490	1		_aRare Diseases of the Immune System _x2282-6505 _v4
520			_aThis book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discussing the highly orchestrated early B cell development in the bone marrow and the genes involved based on both human and animal models. The pathogenesis and clinical presentation of X-linked agammaglobulinemia, caused by mutations in the BTK (Brutonâ€{u3834}yrosine kinase) gene, are then presented in detail, followed by descriptions of the clinical manifestations and molecular basis of the less frequent autosomal recessive and autosomal dominant forms of agammaglobulinemia.ÂСtientsâ€{u0B61}nagement in terms of respiratory complications, gammaglobulin replacement therapy and the potential value of novel experimental therapeutic strategies areÂ{u0929}scussed. The bookâ€{u3823}losing chapters offer a comprehensive and updated description of mutations in the BTK gene, and the expression and function of BTK in cells other than B cells.
942			_2lcc _cLE
650		7	_aAgammaglobulinemia _9152793 _0comprobar BNE19973146947 _2embne
700	1		_aPlebani, Alessandro _eeditor literario _995462 _0Local
700	1		_aLougaris, Vassilios _eeditor literario _995463 _0Local
856	4	0	_uhttps://go.openathens.net/redirector/universidadeuropea.es?url=https://link.springer.com/book/10.1007/978-3-319-22714-6 _zAcceso a este recurso digital (usuarios Universidad Europea de Madrid)
907			_a.b12911276 _b10-10-17 _c10-02-16
988			_aSpringer_Medicine_2015
998			_am _a_alco _a_vill _b14-12-16 _cm _dz _ea _feng _ggw _h0
945			_aRC606 .A336 2015 EB _g1 _ieBOOK _j0 _lmae _o- _pEUR0.00 _q- _r- _sb _t15 _u0 _v0 _w0 _x0 _y.i11578956 _z06-04-17
999			_c82487 _d82487 _x1