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Umfang: 1 online resource (vii, 289 pages) : , digital, PDF file(s).

ISBN: 1-316-84152-9 , 1-316-84243-6 , 1-139-94243-3

Serie: Cambridge medicine

Originaltitel: Pediatric hematology (Wynn)

Inhalt: Hematology is often considered a challenging subject by students and junior medical staff alike. Using key scientific and clinical principles, this succinct guide provides a summary of modern day-to-day clinical practice in paediatric hematology. Focusing on the facts that underpin patient management, each chapter offers an initial summary of a particular paediatric hematologic condition. Several key clinical scenarios set out how common difficulties should be managed, from the neonate with line thrombosis, to the newly presenting patient with leukaemia, and the child who has suffered a stroke. Covering all hematology topics on the syllabus of the American Board of Paediatrics subspecialty examinations, this authoritative guide is ideal for both postgraduates and junior doctors, whose understanding of hematological conditions will increase greatly upon reading. This comprehensive and practical book specifically aims to equip clinicians to diagnose and manage children with hematological illness, and to support them and their families.

Anmerkung: Title from publisher's bibliographic system (viewed on 28 Feb 2017). , Cover -- Half-title -- Title page -- Copyright information -- Table of contents -- Preface -- Chapter 1 Normal Hematopoiesis and the Physiology of Blood -- Introduction -- The Site of Blood Production -- Clinical Implications -- Amount and Rate of Blood Production -- Clinical Implications -- The Bone Marrow Environment -- Clinical Implications -- Understanding the Bone Marrow Compartment -- Clinical Implications -- Hematopoietic Growth Factors -- Clinical Implications -- Specific Hematinics -- Hemoglobin -- Clinical Implications -- RBCs and Oxygen Delivery -- Clinical Implications -- Vascular Endothelium -- Clinical Implications -- Conclusions -- Chapter 2 The Coagulation System -- Introduction -- Virchow's Triad -- The Hemostatic System -- The Coagulation System -- Developmental Hemostasis -- Chapter 3 Common Laboratory Tests Used in Hematology and How to Interpret Them -- Introduction -- Full Blood Examination -- Hemostatic Testing -- Coagulation Testing -- Prothrombin Time -- Activated Partial Thromboplastin Time -- Thrombin Time -- Fibrinogen -- Further Coagulation Assays -- Chapter 4 Reference Ranges in Children -- Introduction -- Clinical Significance of Reference Ranges in Children -- Determining Reference Ranges -- Full Blood Examination -- Coagulation Assays -- Chapter 5 Neonatal Anemia -- Introduction -- Physiologic Anemia of Infancy in Term and Preterm Infants -- Causes of Anemia in the Neonatal Period -- Blood Loss -- Decreased Production -- Immune-Mediated RBC Destruction -- RBC Enzyme Deficiency -- RCB Membrane Defects -- Hemoglobin Disorders -- Clinical Evaluation of Neonatal Anemia -- Laboratory Evaluation in Neonatal Anemia -- Role of RBC Transfusions -- Recombinant Erythropoietin (r-HuEPO) in Anemia of Prematurity -- Supplemental Iron in Preterm Infants. , Chapter 6 Neonatal Abnormal Myelopoiesis and the Hematology of Down Syndrome -- Transient Abnormal Myelopoiesis (TAM) -- Epidemiology and Diagnosis of TAM -- Management of TAM -- Acute Myeloid Leukemia of DS (ML-DS) -- Acute Lymphoid Leukemia of DS -- Hematologic Abnormalities That Are Not Associated with Leukemia in Children with DS -- Chapter 7 Neonatal Thrombocytopenia -- Introduction -- Developmental Differences in Neonatal Megakaryopoiesis and Platelets -- Etiologies to Consider Based on Timing of Onset of Thrombocytopenia -- Inherited Thrombocytopenia Presenting in the Neonatal Period -- Immune and Nonimmune Thrombocytopenia -- Immune Thrombocytopenia -- FMAIT/NAIT -- Autoimmune Thrombocytopenia -- Non-Immune-Mediated Thrombocytopenia -- When Are Platelet Transfusions Indicated -- Role of Thrombopoietic Growth Factors in the Management of Neonatal Thrombocytopenia -- Chapter 8 Neonatal Bleeding Disorders -- Developmental Hemostasis -- Bleeding in the Neonatal Period That Suggests a Bleeding Disorder -- Inherited Bleeding Disorders Presenting in the Neonatal Period -- Acquired Bleeding Disorders -- Points to Note in History Taking -- Maternal History -- Family History -- Neonatal History -- Laboratory Investigations -- Pitfalls to Consider While Interpreting Laboratory Evaluations -- Therapeutic Options -- Prenatal Diagnosis -- Chapter 9 Neonatal Thrombotic Disorders -- Introduction -- Vascular-Access-Related Thrombosis -- Umbilical Artery Catheter (UAC)-Associated Thrombosis -- Umbilical Vein Catheter (UVC)-Associated Thrombosis -- Central Venous Line-Associated Thrombosis -- Peripheral Artery Catheter-Associated Thrombosis -- Treatment and Outcome of CVAD-Related Thrombosis -- Renal Vein Thrombosis -- Neonatal Purpura Fulminans -- Arterial Ischemic Stroke (AIS)/Cerebrosinovenous Thrombosis (CSVT). , Thrombophilia Testing in Newborns with Thrombosis -- Chapter 10 Inherited Bone Marrow Failure Syndromes -- Introduction -- Fanconi Anemia -- The Hematologic Features of Fanconi Anemia -- The Nonhematologic Features of FA -- Diagnosis of Fanconi Anemia -- Biologic Basis of Fanconi Anemia -- Treatment of Fanconi Anemia -- Diamond-Blackfan Anemia -- Hematologic Manifestations of Diamond-Blackfan Anemia -- Nonhematologic Manifestations of Diamond-Blackfan Anemia -- Diagnosis of Diamond-Blackfan Anemia -- Biologic Basis of Diamond-Blackfan Anemia -- Treatment of Diamond-Blackfan Anemia -- Dyskeratosis Congenita (DKC) -- Hematologic Manifestations of Dyskeratosis Congenita -- Nonhematologic Manifestations of Dyskeratosis Congenita -- Diagnosis of Dyskeratosis Congenita -- Biology of Dyskeratosis Congenita -- Treatment of Dyskeratosis Congenita -- Shwachman-Diamond Syndrome -- Hematologic Manifestations of Shwachman-Diamond Syndrome -- Nonhematologic Manifestations of Shwachman-Diamond Syndrome -- Diagnosis of Shwachman-Diamond Syndrome -- Biology of Shwachman-Diamond Syndrome -- Management of Shwachman-Diamond Syndrome -- Chapter 11 Acquired Bone Marrow Failure Syndrome: Aplastic Anemia -- Assessment of the Child with Aplastic Anemia -- Presentation of the Child with Aplastic Anemia -- Etiology of SAA: Excluding an Inherited BMF Syndrome -- Etiology of SAA: Investigation of Acquired Aplasia -- Supportive Care -- Specific Therapies of Severe Aplastic Anemia -- Immunosuppressive Therapy (IST) -- Hematopoietic Stem Cell Transplantation (HSCT) -- Chapter 12 Sideroblastic Anemias -- Introduction -- X-Linked Sideroblastic Anemia (XLSA) -- Pearson's Marrow-Pancreas Syndrome -- Sideroblastic Anemia with Immunodeficiency, Recurrent Fever, and Developmental Delay (SIFD) -- Chapter 13 Iron Deficiency -- Regulation of Iron. , Normal Iron Requirements and Iron Metabolism in Children -- Epidemiology of Iron Deficiency in Children -- Clinical Presentations of Iron Deficiency -- Incidental Presentation -- Symptomatic IDA -- Behavioral Abnormalities -- Diagnosis of Iron Deficiency -- Treatment of Iron Deficiency -- Follow-Up of Iron Deficiency -- Iron Toxicity in Children -- Chapter 14 Disorders of Vitamin B12 and Folate -- Introduction -- Physiology of Vitamin B12 and Folate -- Hematologic and Clinical Manifestations of Vitamin B12 and Folate Deficiency -- Etiology of Megaloblastosis in Childhood -- Diagnosis of Vitamin B12 and Folate Deficiency in Childhood -- Treatment of Vitamin B12 or Folate Deficiency -- Outcome of Vitamin B12 or Folate Deficiency -- Chapter 15 Hemolytic Anemia -- Introduction -- Diagnosis of Hemolysis -- Initial Assessment of the Child with Suspected Hemolysis -- Initial Management of the Child with Hemolysis -- Specific Causes of Hemolysis and Their Acute and Chronic Management -- Chapter 16 Infections in Hematology Patients and the Hematologic Features of Infectious Disease -- Infection in Children with Hematologic Illnesses -- Prophylaxis and Therapy of Bacterial Infection -- Prophylaxis, Diagnosis, and Therapy of Fungal Infection -- Virus Infection during Allogeneic Stem Cell Transplantation -- Pneumacystis jiroveci Infection -- Management of Varicella-Zoster and Measles Exposure in Susceptible Children -- Vaccination after Chemotherapy or HSCT -- Infections That Affect the Blood -- Hematology of Pertussis Infection -- Hematology of EBV Infection -- Hematology of Malaria -- Hematology of Parvovirus Infection -- Chapter 17 Sickle Cell Disease -- Embryonic, Fetal, and Adult Hemoglobin -- Hemoglobin Disorders -- Sickle Cell Syndromes -- Pathophysiology -- Genetic Modifiers of Severity of Sickle Cell Disease -- Diagnosis/Newborn Screening. , Complications of Sickle Cell Disease in Children and Adults -- Infants/Children -- Adults -- Components of Age-Based Approach to Care via Regular Comprehensive Clinic Visits -- Newborn -- Infancy -- Toddler -- Childhood -- Adolescence -- Disease-Altering Agents/Treatment -- Hydroxyurea -- Blood Transfusion and Iron Overload/Chelation -- Hematopoietic Stem Cell Transplantation (HSCT) -- Future Therapy -- Chapter 18 Thalassemia -- Introduction -- Classifications of Different Forms of Thalassemia -- Alpha-Thalassemia -- Beta-Thalassemia -- Hemoglobin E -- Delta-Beta-Thalassemia and Hereditary Persistence of Fetal Hemoglobin (HPFH) -- Beta-Thalassemia Major -- Clinical Features -- Complications -- Management of Anemia -- Iron Overload and Management -- Iron Chelators -- Components of an Age-Based Approach to Care via Regular Comprehensive Clinic Visits -- Genetic Modifiers of Homozygous Beta-Thalassemia -- Prenatal Diagnosis/Newborn Screening/Carrier Detection -- Carrier Detection -- Prenatal Diagnosis -- Newborn Screening -- Hematopoietic Stem Cell Transplantation (HSCT) -- Gene Therapy -- Chapter 19 Neutropenia -- Introduction -- Distinguishing Benign from Severe Neutropenia -- Benign Neutropenias of Childhood -- Chronic Benign Neutropenia/Autoimmune Neutropenia -- Neonatal Alloimmune Neutropenia -- Infection-Induced Neutropenia -- Hypersplenism -- Drug-Induced Neutropenia -- Severe Neutropenia -- Severe Congenital Neutropenia (SCN) -- Cyclic Neutropenia -- Drug-Related Agranulocytosis -- Shwachman-Diamond Syndrome (SDS) -- Myelokathexis/WHIM -- Neutropenia with Other Illnesses -- Neutropenia as part of Constitutional Hypoplastic Anemias and Other Marrow Disorders -- Neutropenia Associated with Nutritional Deficiency -- Neutropenia Associated with Immune Defects -- Neutropenia and Barth Syndrome -- Neutropenia Associated with Metabolic Diseases (GSD1B). , Chapter 20 Neutrophil Function Disorders.

Weitere Ausg.: ISBN 1-107-43936-1

Sprache: Englisch

URL: Volltext  (URL des Erstveröffentlichers)

URL: https://doi.org/10.1017/CBO9781139942430