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008			170116s2017 ja | s |||| 0|eng d
020			_a9784431560036 _9978-4-431-56003-6
024	7		_a10.1007/978-4-431-56003-6 _2doi
035			_a(DE-He213)978-4-431-56003-6
050		4	_aRB45
072		7	_aMJF _2bicssc
072		7	_aMED038000 _2bisacsh
082	0	4	_a616.15 _223
245	1	0	_aParoxysmal Nocturnal Hemoglobinuria _h[electronic resource] : _bFrom Bench to Bedside / _cedited by Yuzuru Kanakura, Taroh Kinoshita, Jun-ichi Nishimura.
264		1	_aTokyo : _bSpringer Japan : _bImprint: Springer, _c2017.
300			_aVIII, 358 p. 71 illus., 46 illus. in color. _bonline resource.
336			_atext _btxt _2rdacontent
337			_acomputer _bc _2rdamedia
338			_aonline resource _bcr _2rdacarrier
347			_atext file _bPDF _2rda
505	0		_a1 A History of Research of PNH: Defining a Disease -- Part I Basic Sciences in PNH -- 2 Synthesis, genetics and congenital diseases of GPI-anchored proteins -- 3 Animal models of paroxysmal nocturnal hemoglobinuria -- 4 Complement and PNH -- Part II Clinical Sciences in PNH -- 5 Epidemiology in PNH: The PNH Global Registry -- 6 Epidemiology in PNH; Clinical Epidemiological Studies in Korea -- 7 Hemolysis in PNH; Depletion of nitric oxide -- 8 Bone Marrow Failure in PNH -- 9 Thrombophilia in PNH -- 10 Diagnosis and Classification of PNH -- 11 Clinical significance of a small population of glycosyl phosphatidylinositol-anchored membrane proteins (GPI-APs)-deficient cells in the management of bone marrow failure -- 12 CLONAL ORIGIN AND CLONAL SELECTION IN PNH -- 13 Pathogenesis of clonal dominance in PNH: Selection mechanisms in PNH -- 14 Pathogenesis of Clonal Dominance in PNH; Growth Advantage in PNH -- 15 Clinical Management in PNH -- 16 CLINICAL EFFECTS OF ECULIZUMAB IN PNH -- 17 Clinical Effects of Eculizumab in PNH; Extravascular Hemolysis after Eculizumab Treatment -- 18 Clinical Effects of Eculizumab in PNH; Poor-Responders to Eculizumab -- 19 Hematopoietic Stem Cell Transplantation in PNH -- 20 Future strategies of complement inhibition in paroxysmal nocturnal hemoglobinuria -- 21 Pregnancy in paroxysmal nocturnal hemoglobinuria.
520			_aThis volume reviews the fundamental understanding of this potentially life-threatening disease and the advances in treatment that have been achieved with the use of the monoclonal antibody eculizumab. Although the PIGA gene has been known for many years, the mechanism of clonal dominance in paroxysmal nocturnal hemoglobinuria is still largely unknown. This book, Paroxysmal Nocturnal Hemoglobinuria, discusses the direction of continuing research in this area, as well as the potential for the development of management guidelines. It serves as a valuable source of information for both basic scientists and physicians, especially immunologists targeting GPI-anchored proteins and complements, and hematologists specializing in bone marrow failure.
650		0	_aMedicine.
650		0	_aImmunology.
650		0	_aHematology.
650	1	4	_aMedicine & Public Health.
650	2	4	_aHematology.
650	2	4	_aImmunology.
700	1		_aKanakura, Yuzuru. _eeditor.
700	1		_aKinoshita, Taroh. _eeditor.
700	1		_aNishimura, Jun-ichi. _eeditor.
710	2		_aSpringerLink (Online service)
773	0		_tSpringer eBooks
776	0	8	_iPrinted edition: _z9784431560012
856	4	0	_uhttp://dx.doi.org/10.1007/978-4-431-56003-6
912			_aZDB-2-SME
999			_c6304 _d6304