| Section 1: Fetal Hematology And Antenatal Diagnosis Of Hematological Disorders |
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Section Editors: Anupam Sachdeva, Shubha R. Phadke |
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1 Physiology of Hematopoiesis |
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3 | (5) |
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Steps in Fetal Hematopoiesis |
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3 | (1) |
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Normal Blood Counts in Fetus |
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4 | (1) |
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Regulation of Hematopoiesis |
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4 | (1) |
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5 | (1) |
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Erythroid Lineage Differentiation |
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5 | (1) |
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Differentiation of White Blood Cells |
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6 | (1) |
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Clinical Significance of Fetal Hematopoiesis |
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6 | (2) |
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2 Developmental Hemostasis and Physiology of Hemostasis in the Fetus and the Newborn |
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8 | (5) |
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9 | (1) |
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9 | (1) |
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10 | (1) |
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Role of Micro-RNA in Developmental Hemostasis |
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11 | (1) |
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Significance of Understanding Fetal and Neonatal Hemostasis |
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11 | (2) |
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3 Granulocytes: Development and Physiology |
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13 | (6) |
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13 | (1) |
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13 | (1) |
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14 | (1) |
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Infection in Neonates: Granulocyte Response |
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15 | (2) |
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17 | (2) |
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4 Developmental Aspects of Platelets |
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19 | (6) |
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Developmental Aspects of Platelets |
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19 | (1) |
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Megakaryocytes during Fetal Development |
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19 | (2) |
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Megakaryocyte Maturation and Development |
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21 | (1) |
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22 | (1) |
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22 | (1) |
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22 | (2) |
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Practical Aspects of Megakaryocyte Cell Biology and Clinical Use of Thrombopoietic Agents |
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24 | (1) |
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25 | (8) |
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25 | (1) |
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25 | (1) |
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Noninvasive Predictors of Fetal Anemia |
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26 | (1) |
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Ultrasonography-based Predictors of Fetal Anemia |
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26 | (1) |
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Plan for Conservative Management or Invasive Sampling |
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27 | (1) |
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Intrauterine Fetal Transfusion:The Technique |
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28 | (5) |
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6 Thalassemia in the Fetus: Prenatal Diagnosis |
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33 | (5) |
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Preventive Strategies for Beta-thalassemia |
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33 | (5) |
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7 Genetic Counseling for Hematological Disorders |
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38 | (5) |
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Burden of the Inherited Hematological Disorders |
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38 | (1) |
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Genetic Testing versus Genetic Screening |
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38 | (1) |
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38 | (1) |
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39 | (4) |
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8 Fetal Hematological Disorders: Future Ahead |
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43 | (6) |
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43 | (1) |
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44 | (5) |
| Section 2: RBC Disorders And Anemia In Newborn |
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Section Editors: M.R. Lokeshwar, Ratna Sharma |
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9 Developmental Erythropoiesis |
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49 | (9) |
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49 | (1) |
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49 | (1) |
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Embryonic Development of Erythropoiesis |
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49 | (3) |
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Erythroid Cell Maturation |
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52 | (2) |
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54 | (1) |
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Sequence of Activation of Globin Genes |
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55 | (1) |
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55 | (3) |
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10 Normal Hematological Values and Variation in Newborn Period with Special Reference to Hb and RBC Parameters |
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58 | (5) |
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58 | (1) |
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Normal Hematological Values and Variation in Newborn Period |
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59 | (4) |
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11 Effect of Nutritional Deficiency Anemia during Pregnancy on Fetus and Newborn |
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63 | (7) |
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63 | (1) |
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63 | (1) |
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63 | (1) |
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Maternal Effects of Anemia |
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64 | (1) |
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64 | (2) |
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Treatment of Iron Deficiency Anemia |
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66 | (1) |
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Folic Acid Deficiency Anemia |
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67 | (1) |
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67 | (1) |
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68 | (2) |
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70 | (4) |
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70 | (1) |
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70 | (1) |
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70 | (1) |
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70 | (1) |
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71 | (1) |
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Optimizing Nutritional Support |
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72 | (2) |
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13 Clinical Aspect of Immune Hemolytic Disease of Newborn |
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74 | (7) |
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Rh Blood Group System and Rh Isoimmunization |
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74 | (1) |
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75 | (1) |
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76 | (1) |
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Diagnosis of Immune Hemolytic Disease in the Newborn |
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76 | (1) |
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Management of Rh Isoimmunization |
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77 | (1) |
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ABO Blood Group System and ABO Hemolytic Disease |
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78 | (3) |
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14 Laboratory Management of ABO and Rh Hemolytic Disease of the Fetus and Newborn |
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81 | (6) |
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Pathophysiology of Hemolytic Disease of the Newborn |
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81 | (1) |
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ABO Hemolytic Disease of the Newborn |
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81 | (1) |
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81 | (1) |
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81 | (1) |
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Rh Hemolytic Disease of the Fetus and Newborn |
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82 | (1) |
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Maternal Laboratory Investigations |
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83 | (1) |
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Spectrophotometric Analysis of Amniotic Fluid |
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84 | (1) |
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84 | (3) |
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15 Nonimmune Hemolytic Anemia |
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87 | (15) |
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Confounding Factors in the Evaluation of Neonatal Hemolysis |
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88 | (1) |
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Extrinsic Causes of Hemolysis In Neonates |
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88 | (3) |
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Disorders of the RBC Membrane |
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91 | (3) |
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94 | (2) |
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Hemoglobinopathies in the Neonate |
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96 | (6) |
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16 Inherited Bone Marrow Failure Syndromes |
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102 | (9) |
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102 | (4) |
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106 | (3) |
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Severe Congenital Neutropenia |
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109 | (1) |
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Thrombocytopenia Absent Radii |
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109 | (2) |
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17 Polycythemia and Hyperviscosity Syndrome in Newborn |
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111 | (8) |
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111 | (1) |
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111 | (1) |
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112 | (1) |
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112 | (1) |
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113 | (1) |
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Medical Management of Polycythemia |
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113 | (6) |
| Section 3: Coagulation Disorders In Newborn |
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Section Editors: Mamta Manglani, Jagdish Chandra |
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18 Congenital Coagulation Disorders in the Neonate |
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119 | (8) |
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119 | (1) |
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119 | (1) |
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Laboratory Investigations and Interpretation |
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120 | (2) |
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Specific Coagulation Factor Deficiencies |
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122 | (1) |
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Von Willebrand Factor Deficiency |
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122 | (1) |
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122 | (2) |
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Hereditary Deficiencies of Multiple Coagulation Factors |
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124 | (1) |
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Defects in the Fibrinolytic Pathway |
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125 | (2) |
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19 Vitamin K Deficiency Bleeding |
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127 | (8) |
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127 | (1) |
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128 | (1) |
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129 | (1) |
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129 | (1) |
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130 | (1) |
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131 | (1) |
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Risks and Disadvantages of Different Forms of Vitamin K Prophylaxis |
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132 | (3) |
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20 Disseminated Intravascular Coagulation in the Neonatal Period |
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135 | (6) |
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135 | (1) |
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Pathogenesis/Pathophysiology |
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136 | (1) |
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136 | (1) |
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136 | (2) |
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138 | (1) |
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Outcomes/Prognostic Factors |
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139 | (2) |
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21 Thrombophilia in Newborns |
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141 | (12) |
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141 | (1) |
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141 | (3) |
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144 | (1) |
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144 | (1) |
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145 | (1) |
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146 | (7) |
| Section 4: Platelets, Platelet Disorders And Approach To Bleeding In Newborn |
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Section Editors: Nitin K. Shah, Amita Mahajan |
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22 Approach to Neonatal Thrombocytopenia |
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153 | (6) |
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Neonatal Megakaryopoiesis and Thrombopoiesis |
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153 | (1) |
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153 | (1) |
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Causes of Neonatal Thrombocytopenia |
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154 | (5) |
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23 Neonatal Thrombocytopenia: Syndromic Causes |
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159 | (4) |
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Causes of Syndromic Thrombocytopenia in Newborn |
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159 | (4) |
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24 Qualitative Platelet Disorders |
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163 | (11) |
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163 | (1) |
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163 | (1) |
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Clinical Features of Platelet Functional Defects |
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163 | (1) |
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Hereditary Platelet Dysfunction Disorders |
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163 | (3) |
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Acquired Platelet Function Defects |
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166 | (1) |
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167 | (1) |
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Recommendations for Specimen Collection and Processing |
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167 | (5) |
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Challenges in Diagnosing Platelet Function Disorders in Infants |
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172 | (1) |
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Treatment of Qualitative Platelet Defects |
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172 | (2) |
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25 Approach to a Bleeding Neonate |
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174 | (9) |
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174 | (1) |
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175 | (1) |
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175 | (1) |
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176 | (1) |
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Diagnosis of Bleeding in a Neonate |
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176 | (1) |
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Recent Development in the Diagnostics |
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177 | (1) |
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177 | (2) |
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179 | (1) |
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180 | (3) |
| Section 5: Neutrophil Disorders |
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Section Editors: Bharat Agarwal, Tulika Seth |
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183 | (5) |
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Neutrophil Counts in Newborn |
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183 | (1) |
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Mechanism of Neutrophilia in Newborns |
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183 | (1) |
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Factors Affecting Neutrophil Counts |
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184 | (1) |
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Disorders with Neutrophilia |
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185 | (3) |
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27 Important Causes of Neutropenia including Inherited Conditions: Diagnosis and Work-up |
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188 | (10) |
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188 | (1) |
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189 | (3) |
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Neutropenia Associated with Metabolic Diseases |
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192 | (1) |
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192 | (1) |
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Neutropenia Caused by Extrinsic Factors |
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192 | (1) |
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193 | (2) |
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Therapy for Neonatal Neutropenia |
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195 | (3) |
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28 Neutrophil Function Defects |
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198 | (8) |
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198 | (1) |
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199 | (1) |
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Disorders of Degranulation |
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199 | (2) |
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Disorders of Microbicidal Metabolism |
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201 | (3) |
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Approach to the Diagnosis of Neutrophil Function Defects |
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204 | (2) |
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29 Management of Neutropenia and Infections |
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206 | (11) |
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Management of Neutropenia and Infections |
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206 | (1) |
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207 | (1) |
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208 | (1) |
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Clinical Evaluation of Neutropenia in Neonates |
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209 | (1) |
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Laboratory Evaluation of Neutropenia in Neonates |
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209 | (1) |
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Clinical Management of a Neonate with Neutropenia |
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210 | (1) |
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210 | (2) |
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How to Use Recombinant G-CSF? |
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212 | (1) |
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212 | (5) |
| Section 6: Fetal And Neonatal Malignancy |
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Section Editors: Girish Chinnaswamy, Arpita Bhattacharya |
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217 | (8) |
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217 | (1) |
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217 | (1) |
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217 | (1) |
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Challenges in Management of Infantile Cancers |
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218 | (1) |
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Pain Assessment and Management |
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218 | (1) |
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Common Hematolymphoid and Solid Tumors in Infants |
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218 | (1) |
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219 | (1) |
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219 | (1) |
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220 | (1) |
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220 | (1) |
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220 | (2) |
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222 | (1) |
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222 | (1) |
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Atypical Teratoid or Rhabdoid Tumor |
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222 | (1) |
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222 | (1) |
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Screening for Cancers in Infants: is there any Role? |
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222 | (1) |
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Balancing Cure and Quality of Life |
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223 | (2) |
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225 | (5) |
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225 | (1) |
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Etiology and Pathogenesis |
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225 | (1) |
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226 | (1) |
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226 | (1) |
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226 | (1) |
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Management and Outcomes for Major Subtypes of Neonatal Leukemia |
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226 | (4) |
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230 | (8) |
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230 | (1) |
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Benign Neonatal Liver Tumors |
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230 | (1) |
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Infantile Hepatic Hemangioma |
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230 | (2) |
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Malignant Neonatal Liver Tumors |
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232 | (2) |
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234 | (1) |
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234 | (1) |
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235 | (3) |
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238 | (6) |
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238 | (1) |
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238 | (1) |
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Ploidy (Tumor Cell DNA Content) |
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238 | (1) |
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Familial Neuroblastoma and Genetic Susceptibility |
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239 | (1) |
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239 | (1) |
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239 | (1) |
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240 | (1) |
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241 | (1) |
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241 | (1) |
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242 | (2) |
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34 Renal Tumors in Neonates |
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244 | (6) |
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Overview of the Common Renal Tumors |
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244 | (1) |
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245 | (1) |
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246 | (1) |
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Malignant Rhabdoid Tumor of the Kidney |
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246 | (1) |
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Clear Cell Sarcoma of the Kidney |
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246 | (1) |
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Ossifying Renal Tumor of Infancy |
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247 | (1) |
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247 | (1) |
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Miscellaneous Renal Tumors |
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247 | (3) |
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35 Neonatal Soft Tissue Sarcomas |
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250 | (4) |
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Venkatraman Radhakrishnan |
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Approach to a Neonate with Soft Tissue Swelling |
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250 | (1) |
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Common Soft Tissue Neoplasms |
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251 | (3) |
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36 Fetal and Neonatal Germ Cell Tumors |
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254 | (9) |
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254 | (1) |
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Etiology and Pathogenesis |
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254 | (1) |
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254 | (2) |
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256 | (1) |
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Diagnosis and Work-up in Fetal and Neonatal Germ Cell Tumors |
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257 | (1) |
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Management of Fetal and Neonatal Germ Cell Tumors |
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258 | (2) |
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Outcomes and Prognostic Factors |
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260 | (1) |
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Late Effects of Treatment and Follow-up |
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260 | (1) |
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260 | (3) |
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37 Retinoblastoma and its Prenatal Screening |
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263 | (4) |
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263 | (1) |
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263 | (4) |
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38 Pharmacology of Anticancer Drugs in Neonate |
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267 | (3) |
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267 | (1) |
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267 | (1) |
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268 | (1) |
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268 | (2) |
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39 Challenges in Neonatal Malignancies |
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270 | (7) |
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270 | (1) |
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Challenges in Neonatal Malignancies |
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270 | (1) |
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271 | (1) |
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272 | (1) |
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Problems Specific to Developing Countries |
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273 | (1) |
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Lack of Adequate Expertise, Equipment and Infrastructure |
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273 | (4) |
| Section 7: Blood Transfusion (Component Therapy) And Supportive Therapy |
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Section Editors: Anand Deshpande, Avneet Kaur |
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40 Cellular Blood Components in Neonatal Practice |
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277 | (6) |
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Various Components of Blood |
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277 | (1) |
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277 | (1) |
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Packed Red Blood Transfusions in Neonates |
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277 | (1) |
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Evidence for Threshold of Transfusion |
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278 | (1) |
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279 | (1) |
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Dose and Duration of Therapy |
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279 | (1) |
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279 | (1) |
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280 | (1) |
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Types of Platelets Available |
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281 | (1) |
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281 | (1) |
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Indications of Irradiated Cellular Products |
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281 | (1) |
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281 | (2) |
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41 Plasma and its Derivatives |
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283 | (3) |
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Coagulation System in Neonates |
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283 | (1) |
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Plasma and its Derivatives |
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284 | (1) |
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Plasma Transfusion Guidelines in Neonates |
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284 | (1) |
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Adverse Effects with Plasma Transfusion |
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285 | (1) |
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42 Technical Aspects of Blood and Blood Components Transfusion including Leukodepletion and Irradiation |
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286 | (8) |
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Some Technical Aspects about the Administration of Blood and Blood Components |
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286 | (2) |
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Leukocyte Depletion and Gamma Irradiation of Blood Components |
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288 | (1) |
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Risk Factors Associated with Occurrence of Transfusion Associated Graft-Versus-Host Disease |
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289 | (1) |
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Indications for Irradiation of Blood and Blood Components |
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289 | (1) |
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Blood and Blood Components to be Irradiated |
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290 | (1) |
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Dosimetry of Blood Irradiator |
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290 | (4) |
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43 Risk of Transfusion in Fetus and Neonate |
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294 | (4) |
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294 | (1) |
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Evaluation of Fetal Anemia |
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294 | (1) |
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Management of Fetal Anemia |
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294 | (1) |
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Risk of Intrauterine Transfusion |
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295 | (1) |
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Intrauterine Platelet Transfusion |
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295 | (1) |
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Adverse Effects of Transfusion in Neonates |
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295 | (1) |
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Transfusion-associated Graft-Versus-Host Disease |
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296 | (2) |
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44 Hematological Growth Factors as Supportive Therapy |
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298 | (19) |
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Granulocyte-colony Stimulating Factor or Granulocyte-macrophage Colony-stimulating Factor |
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298 | (2) |
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Role of G-CSF and GM-CSF in Neonatal Sepsis |
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300 | (1) |
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300 | (2) |
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302 | (1) |
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G-CSF or GM-CSF: Which One has Better Effect in Neonate? |
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302 | (1) |
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Role of G-CSF or GM-CSF in Other Neutropenias |
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302 | (1) |
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Future Proposed Uses of G-CSF or GM-CSF in Neonatal Intensive Care Unit |
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303 | (1) |
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Side Effects of G-CSF or GM-CSF |
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303 | (1) |
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304 | (2) |
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306 | (1) |
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307 | (1) |
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Use in Rhesus Sensitization |
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307 | (1) |
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308 | (1) |
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Other Uses: Neuroprotection |
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308 | (9) |
| Section 8: Immunological Disorders |
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Section Editors: Manisha Madkaikar, Nita Radhakrishnan |
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45 Introduction to Immunology |
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317 | (8) |
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317 | (1) |
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Components of the Innate Immune System |
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318 | (3) |
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Nucleotide Oligomerization Domain Receptors/Nucleotide Oligomerization Domain Leucine-Rich Repeat Containing Receptors |
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321 | (1) |
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321 | (4) |
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46 Fetal and Neonatal Immunity |
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325 | (11) |
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325 | (1) |
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Development of the Immune System |
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326 | (1) |
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327 | (3) |
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330 | (1) |
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Organs of the Immune System |
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331 | (1) |
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Neonatal Humoral Responses |
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331 | (1) |
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Neonatal Cell-mediated Responses |
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331 | (1) |
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Passive Immunity in Neonates |
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332 | (1) |
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332 | (1) |
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Colostrum and Breast Milk |
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332 | (1) |
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Deleterious Effects of Maternal Antibodies |
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333 | (3) |
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47 Infections in Neonatal Period: Primary Immunodeficiency Perspective |
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336 | (5) |
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336 | (1) |
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336 | (1) |
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Early Onset Neonatal Sepsis |
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336 | (1) |
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336 | (1) |
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337 | (1) |
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337 | (1) |
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337 | (1) |
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Prevention of Late Onset Sepsis |
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337 | (1) |
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Neonatal Immune System and Predisposition to Infections |
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337 | (1) |
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Uncommon Infections in Neonate and Role of Primary Immunodeficiency |
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338 | (3) |
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48 Approach to Newborns with Immunodeficiency |
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341 | (6) |
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Primary Immunodeficiency Disorders |
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341 | (3) |
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Advanced Tests for Specific Disorders |
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344 | (1) |
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345 | (2) |
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49 Neutrophil Functions and Disorders of Neutrophils in Newborns |
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347 | (7) |
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Normal Development and Function of Neutrophils |
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347 | (1) |
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348 | (6) |
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50 Combined T-and B-cell Defects |
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354 | (12) |
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354 | (1) |
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Etiology and Pathophysiology |
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354 | (3) |
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Types of Combined T-and B-cells Disorders (Classification) |
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357 | (3) |
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Other Immunodeficiency Disorders |
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360 | (1) |
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361 | (1) |
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362 | (1) |
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362 | (2) |
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364 | (1) |
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364 | (2) |
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51 Primary Antibody Deficiencies |
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366 | (10) |
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X-linked Agammaglobulinemia |
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367 | (1) |
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368 | (1) |
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369 | (1) |
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370 | (1) |
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Autosomal Recessive Hypogammaglobulinemia |
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371 | (1) |
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Common Variable Immunodeficiency |
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371 | (1) |
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371 | (1) |
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372 | (1) |
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372 | (1) |
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Selective Immunoglobulin A Deficiency |
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372 | (1) |
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Transient Hypogammaglobulinemia of Infancy |
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373 | (1) |
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Specific Antibody Deficiency |
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373 | (3) |
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52 Combined Immunodeficiencies with Associated or Syndromic Features |
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376 | (11) |
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Congenital Thrombocytopenias |
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376 | (5) |
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Hyperimmunoglobulin E Syndromes |
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381 | (2) |
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Defects of Vitamin B12 and Folate Metabolism |
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383 | (4) |
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53 Immune Dysregulation Syndromes |
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387 | (28) |
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Hemophagocytic Lymphohistiocytosis |
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389 | (1) |
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389 | (1) |
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389 | (3) |
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Genetics of Hemophagocytic Lymphohistiocytosis |
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392 | (2) |
|
Clinical Presentation of Hemophagocytic Lymphohistiocytosis |
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394 | (3) |
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397 | (3) |
|
Treatment of Hemophagocytic Lymphohistiocytosis |
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400 | (4) |
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Autoimmune Lymphoproliferative Syndrome |
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404 | (1) |
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404 | (1) |
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404 | (1) |
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Laboratory Investigations |
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405 | (1) |
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405 | (1) |
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406 | (1) |
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406 | (1) |
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407 | (1) |
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408 | (1) |
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409 | (1) |
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409 | (6) |
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415 | (13) |
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417 | (1) |
|
Etiology and Pathogenesis |
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417 | (3) |
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420 | (3) |
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423 | (1) |
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423 | (5) |
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55 Autoinflammatory Syndromes |
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428 | (8) |
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428 | (1) |
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428 | (1) |
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Classic Autoinflammatory Syndromes |
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429 | (1) |
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430 | (4) |
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434 | (2) |
|
56 Management of Specific Immunodeficiencies |
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436 | (5) |
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|
Immune System of Human Body |
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|
436 | (1) |
|
Classification of Primary Immunodeficiency |
|
|
436 | (1) |
|
Importance of Early Diagnosis |
|
|
436 | (1) |
|
Management of Specific Primary Immunodeficiencies |
|
|
437 | (2) |
|
Vaccination in Patients with Primary Immunodeficiencies |
|
|
439 | (2) |
|
57 Neonatal Screening and Prenatal Diagnosis of Primary Immunodeficiency Disorders |
|
|
441 | (8) |
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|
|
Why should we Include Primary Immunodeficiency Disorders in NBS? |
|
|
441 | (1) |
|
Need for Newborn Screening for SCID and Other T-cell Immunodeficiencies |
|
|
441 | (1) |
|
TREC Assay and its Utility as a Screening Test for SCID |
|
|
442 | (1) |
|
|
|
443 | (1) |
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|
443 | (1) |
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|
|
444 | (2) |
|
Advances in Prenatal Diagnosis Techniques |
|
|
446 | (3) |
| Index |
|
449 | |
