PEDIATRICS

Inherited Abnormalities of Coagulation

Oct 2, 2017 by in PEDIATRICS Comments Off on Inherited Abnormalities of Coagulation

Bleeding disorders are broadly classified into primary and secondary hemostatic defects. Primary hemostatic disorders (disorders of platelets and von Willebrand factor) mainly result in mucocutaneous bleeding symptoms such as epistaxis,…

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Developmental Hemostasis

Oct 2, 2017 by in PEDIATRICS Comments Off on Developmental Hemostasis

The coagulation system involves a dynamic group of procoagulation and anticoagulation proteins that appear early in fetal life and whose levels change throughout childhood and into the teenage years. This…

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Thalassemias

Oct 2, 2017 by in PEDIATRICS Comments Off on Thalassemias

The thalassemia syndromes are hemoglobin disorders that result from significantly reduced or absent synthesis of either the α- or β-globin chains. The result is a chronic hemolytic anemia with ineffective…

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Sickle Cell Disease in Childhood

Oct 2, 2017 by in PEDIATRICS Comments Off on Sickle Cell Disease in Childhood

Sickle cell disease (SCD) is the name for a group of related blood disorders caused by an abnormal hemoglobin molecule that polymerizes on deoxygenation. SCD affects the entire body, and…

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Abnormalities of the Erythrocyte Membrane

Oct 2, 2017 by in PEDIATRICS Comments Off on Abnormalities of the Erythrocyte Membrane

Primary abnormalities of the erythrocyte membrane are characterized by clinical, laboratory, and genetic heterogeneity. Among this group, hereditary spherocytosis patients are more likely to experience symptomatic anemia. Treatment of hereditary…

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Acquired Aplastic Anemia in Children

Oct 2, 2017 by in PEDIATRICS Comments Off on Acquired Aplastic Anemia in Children

This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed…

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The Inherited Bone Marrow Failure Syndromes

Oct 2, 2017 by in PEDIATRICS Comments Off on The Inherited Bone Marrow Failure Syndromes

Molecular pathogenesis may be elucidated for inherited bone marrow failure syndromes (IBMFS). The study and presentation of the details of their molecular biology and biochemistry is warranted for appropriate diagnosis…

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Development of the Hematopoietic System and Disorders of Hematopoiesis that Present During Infancy and Early Childhood

Oct 2, 2017 by in PEDIATRICS Comments Off on Development of the Hematopoietic System and Disorders of Hematopoiesis that Present During Infancy and Early Childhood

This article reviews the ontogeny of hematopoiesis (embryonic/fetal/newborn phases) and its regulation and provides examples of the disorders of hematopoiesis that present in the newborn or infant and their pathophysiology….

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