Chapter 099 Disorders of Hemoglobin (Part 9) docx

... deficiency may aid in control. Chapter 099. Disorders of Hemoglobin (Part 9) Unstable hemoglobins occur sporadically, often by spontaneous new mutations. Heterozygotes are often symptomatic because ... complications of chronic hemolysis, even if anemia is absent. Precipitation of unstable hemoglobins is aggravated by oxidative stress, e.g., infection, antimalarial dru...

Ngày tải lên: 07/07/2014, 04:20

... Predictors of fetal hemoglobin response in children with sickle cell anemia receiving hydroxyurea therapy. Blood 99:10, 2002 [PMID: 11756146] Chapter 099. Disorders of Hemoglobin (Part 14) ... 485–510 Steinberg MH, Benz EJ Jr: Pathobiology of the human erythrocyte and its hemoglobins, in Hematology: Basic Principles and Practice , 3d ed, R Hoffman et al (eds). New York...

Ngày tải lên: 07/07/2014, 04:20

... Chapter 099. Disorders of Hemoglobin (Part 1) Harrison's Internal Medicine > Chapter 99. Disorders of Hemoglobin Disorders of Hemoglobin: Introduction Hemoglobin is ... stigmata. Hemoglobin Structure Different hemoglobins are produced during embryonic, fetal, and adult life (Fig. 99-1). Each consists of a tetramer of globin polypeptide chains: a...

Ngày tải lên: 07/07/2014, 04:20

... the ability of hemoglobin to deliver more oxygen to tissues at low pH. It arises from the stabilizing action of protons on deoxyhemoglobin, which binds protons more readily than oxyhemoglobin ... the tetrameric structure of the proteins, the proper arrangement of the charged amino acids, and interaction with protons or 2,3-BPG. Developmental Biology of Human Hemoglobins Red...

Ngày tải lên: 07/07/2014, 04:20

... biosynthesis) and of structural hemoglobinopathies (e.g., an abnormal amino acid sequence). Hereditary persistence of fetal hemoglobin (HPFH) is characterized by synthesis of high levels of fetal hemoglobin ... Hereditary persistence of fetal hemoglobin persistence of high levels of HbF into adult life V. Acquired hemoglobinopathies A. Methemoglobin due to toxic...

Ngày tải lên: 07/07/2014, 04:20

... of the complete blood cell count (e.g., profound microcytosis with minimal anemia in thalassemia trait). Detection and Characterization of Hemoglobinopathies—General Methods Of the ... mutant hemoglobins can usually be characterized by more specialized techniques such as isoelectric focusing and/or high-pressure liquid chromatography (HPLC). Quantitation of the hemoglobin pr...

Ngày tải lên: 07/07/2014, 04:20

... Chapter 099. Disorders of Hemoglobin (Part 5) Sickle Cell Syndromes The sickle cell syndromes are caused by a ... C:50% Pathophysiology of sickle cell crisis. Several sickle syndromes occur as the result of inheritance of HbS from one parent and another hemoglobinopathy, such as β thalassemia ... reticulocytes) that are abnormally adherent to the endoth...

Ngày tải lên: 07/07/2014, 04:20

... important for prognosticating the clinical severity of disease. Chapter 099. Disorders of Hemoglobin (Part 6) Clinical Manifestations of Sickle Cell Anemia Most patients with sickling ... increase in the hematocrit and feedback inhibition of reticulocytosis might be beneficial, even at the expense of increased blood viscosity. The role of adhesive reticulocyte...

Ngày tải lên: 07/07/2014, 04:20

... tests already discussed. Thorough characterization of the exact hemoglobin profile of the patient is important, because sickle thalassemia and hemoglobin SC disease have distinct prognoses or ... of retinopathy; antibiotic prophylaxis appropriate for splenectomized patients during dental or other invasive procedures; and vigorous oral hydration during or in anticipation of pe...

Ngày tải lên: 07/07/2014, 04:20

... therapy of sickle cell anemia has been the introduction of hydroxyurea as a mainstay of therapy for patients with severe symptoms. Hydroxyurea (10–30 mg/kg per day) increases fetal hemoglobin ... received kidney transplants, but they often experience an increase in the frequency and severity of crises, possibly due to increased infection as a consequence of immunosuppression....

Ngày tải lên: 07/07/2014, 04:20