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Immune Mediated Diseases From Theory to Therapy

:	Michael R. Shurin, Yuri S. Smolkin
:	Michael R Shurin, Yuri S. Smolkin
:	Immune Mediated Diseases From Theory to Therapy
:	Springer-Verlag
:	9780387720050
:	1
:	CHF 189.30
:

:	Nichtklinische Fächer
:	English

:	458
:	Wasserzeichen
:	PC/MAC/eReader/Tablet
:	PDF

This volume includes contributions from the speakers of the Second IMD Congress (September 10-15, 2007; Moscow, Russia) who were eager to share some of the academic and clinical enthusiasm that defines the IMD meetings. The goal of the International Immune-Mediated Diseases: From Theory to Therapy (IMD) Congress is to bring the world's best immunologists and clinicians to Moscow.

	Contents	5
	PREFACE AND ACKNOWLEDGMENTS	10
	List of Contributing Speakers	12
	Immune-Mediated Diseases	21
	1 Immune-Mediated Diseases: Where Do We Stand?	22
	1. Immune-Mediated Diseases: Diversity	22
	2. Immune-Mediated Diseases: Statistics	24
	3. Immune-Mediated Diseases: Correlations	26
	4. Concluding Remarks	29
	References	29
	Immunodeficiencies: News and Updates	32
	2 The Four Most Common Pediatric Immunodeficiencies	33
	1. Transient Hypogammaglobulinemia of Infancy	33
	1.1. Definition and History	33
	1.2. Etiology	34
	1.3. Clinical Features	34
	1.4. Laboratory Features	35
	1.5. Management and Prognosis	35
	2. IgG Subclass Immunodeficiency	35
	2.1. Definition and History	35
	2.2. Etiology	36
	2.3. Clinical Features	36
	2.4. Laboratory Features	37
	2.5. Management and Prognosis	37
	3. Impaired Polysaccharide Responsiveness	38
	3.1. Definition and History	38
	3.2. Etiology	38
	3.3. Clinical Features	39
	3.4. Laboratory Features	39
	3.5. Treatment and Prognosis	39
	4. Selective IgAD Deficiency	40
	4.1. Definition and History	40
	4.2. Etiology	40
	4.3. Clinical Features	41
	4.4. Laboratory Features	41
	4.5. Treatment and Prognosis	41
	5. Conclusions	42
	References	42
	3 Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Inheritance: Model for Autoaggression	45
	1. Introduction	45
	2. Clinical and Pathologic Manifestations of IPEX	46
	2.1. Gastrointestinal Symptoms and Failure to Thrive	46
	2.2. Autoimmune Endocrinopathy	47
	2.3. Autoimmune Hematologic Disorders	47
	2.4. Dermatologic Abnormalities	47
	2.5. Infections	47
	2.6. Other Clinical Manifestations	48
	2.7. Laboratory Findings and Histopathology	48
	3. Molecular Basis of IPEX	48
	4. Function of FOXP3	49
	5. Foxp3 and Treg Cells	50
	6. Fox Mutation Analysis in Patients with the IPEX Phenotype	50
	7. Diagnoses and Treatment of IPEX	51
	8. Animal Models	51
	9. Conclusions	52
	References	52
	4 DiGeorge Syndrome/Velocardiofacial Syndrome: The Chromosome 22q11.2 Deletion Syndrome	55
	1. Nomenclature	55
	2. Genetics	56
	3. Management	58
	4. Conclusions	62
	References	62
	5 Leukocyte Adhesion Deficiencies: Molecular Basis, Clinical Findings, and Therapeutic Options	68
	1. Introduction	68
	2. Leukocyte Adhesion Deficiency I	69
	3. Leukocyte Adhesion Deficiency II	72
	4. Leukocyte Adhesion Deficiency III	74
	5. Conclusions	75
	References	75
	6 Nijmegen Breakage Syndrome	78
	1. Introduction	78
	2. Genetic Basis for NBS	79
	3. Clinical Presentation and Therapy	80
	4. Data from Russian Clinical Children s Hospital	81
	References	82
	7 Neutrophil Activity in Chronic Granulomatous Disease	85
	1. Introduction	85
	2. Experimental Procedures	86
	3. Results	87
	3.1. Analysis o