Chapter 099 Disorders of Hemoglobin (Part 13) ppsx
Chapter 099. Disorders of Hemoglobin (Part 13) ppsx
... hypochromia, and hemoglobin levels
rarely <1 g/L (<10 g/dL).
Hereditary Persistence of Fetal Hemoglobin
HPFH is characterized by continued synthesis of high levels of HbF in
adult life. ...
metering pump. The constant presence of the drug improves the efficiency of
chelation and protects tissues from occasional releases of the most toxic fraction of
iron—low-mol...
Chapter 099. Disorders of Hemoglobin (Part 10) ppsx
... inherited disorders of α- or β-globin
biosynthesis. The reduced supply of globin diminishes production of hemoglobin
tetramers, causing hypochromia and microcytosis. Unbalanced accumulation of α ... critical clue. The best diagnostic test is
methemoglobin assay, which is usually available on an emergency basis.
Methemoglobinemia often causes symptoms of cerebral ischemia at...
Chapter 099. Disorders of Hemoglobin (Part 12) ppsx
... that affect the LCR of the α-globin gene cluster.
Diagnosis and Management of Thalassemias
The diagnosis of β-thalassemia major is readily made during childhood on
the basis of severe anemia ... the characteristic signs of massive
ineffective erythropoiesis: hepatosplenomegaly, profound microcytosis, a
characteristic blood smear (Fig. 99-5), and elevated levels of HbF, HbA
2...
Chapter 099. Disorders of Hemoglobin (Part 1) pptx
... 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...
Chapter 099. Disorders of Hemoglobin (Part 2) pdf
... 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...
Chapter 099. Disorders of Hemoglobin (Part 3) pptx
... 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...
Chapter 099. Disorders of Hemoglobin (Part 4) pptx
... 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...
Chapter 099. Disorders of Hemoglobin (Part 5) pps
... 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...
Chapter 099. Disorders of Hemoglobin (Part 6) ppt
... 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...
Chapter 099. Disorders of Hemoglobin (Part 7) ppt
... 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...
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