disorders of hemoglobin pdf
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 cells first ... thalassemia syndromes and certain unstable hemoglobin disorders. Polymorphic variation in the amounts and/or functional capacity of AHSP might explain some of the clinical variability seen in patients...
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Chapter 099. Disorders of Hemoglobin (Part 8) pdf
... 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. The most significant ... pathogenesis of sickle cell crisis, and their suppression may be an important benefit of hydroxyurea therapy. Hydroxyurea should be considered in patients experiencing repeated episodes of acute...
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COLOR ATLAS OF DISEASES AND DISORDERS OF CATTLE_2 pdf
... to acidosis. All of these factors can precipitate laminitis/coriosis, the consequences of which are abnormal horn growth and hoof wear, softening of the sole horn, dropping of the distal phalanx ... once and preferably several times daily. Loss of appetite, progressive signs of dullness, inability to sit up COLOR ATLAS OF DISEASES AND DISORDERS OF CATTLE1027dermatitis in the interdigital ... required.Management: white line disorders are primarily a defect of the corium leading to the production of defec-tive cement. Coriosis may be the result of a range of factors including trauma...
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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 critical ... 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 pair of α-like ... alter clinical behavior. Function of Hemoglobin To support oxygen transport, hemoglobin must bind O2 efficiently at the partial pressure of oxygen (PO2) of the alveolus, retain it, and release...
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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 exposures B. Sulfhemoglobin due to ... life. Acquired hemoglobinopathies include modifications of the hemoglobin molecule by toxins (e.g., acquired methemoglobinemia) and abnormal hemoglobin synthesis (e.g., high levels of HbF production...
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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 profile ... The number of severe cases of thalassemia in the United States is about 1000. Sickle cell disease is the most common structural hemoglobinopathy occurring in heterozygous form in ~8% of American...
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Chapter 099. Disorders of Hemoglobin (Part 5) pps
... 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 ... Chapter 099. Disorders of Hemoglobin (Part 5) Sickle Cell Syndromes The sickle cell syndromes are caused by a ... reticulocytes) that are abnormally adherent to the endothelium of small venules. These abnormalities provoke unpredictable episodes of microvascular vasoocclusion and premature RBC destruction...
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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 reticulocytes in vasoocclusion might ... invariably produces wide analysis will yield individualized profiles that predict a patient's clinical course. Nevertheless, a number of interesting patterns have emerged from these modifying...
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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 periods of ... syndromes are suspected on the basis of hemolytic anemia, RBC morphology (Fig. 99-4), and intermittent episodes of ischemic pain. Diagnosis is confirmed by hemoglobin electrophoresis and the...
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Chapter 099. Disorders of Hemoglobin (Part 9) docx
... aid in control. Chapter 099. Disorders of Hemoglobin (Part 9) Unstable hemoglobins occur sporadically, often by spontaneous new mutations. Heterozygotes are often symptomatic because a significant ... complications of chronic hemolysis, even if anemia is absent. Precipitation of unstable hemoglobins is aggravated by oxidative stress, e.g., infection, antimalarial drugs. High-O2 affinity hemoglobin ... variant accounts for a portion of the total hemoglobin. Symptomatic unstable hemoglobins tend to be β-globin variants, because sporadic mutations affecting only one of the four α-globins would...
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Chapter 030. Disorders of Smell, Taste, and Hearing (Part 3) pdf
... amount of salt in their diet. Taste Compared with disorders of smell, gustatory disorders are uncommon. Loss of olfactory sensitivity is often accompanied by complaints of loss of the sense of ... that expected on the basis of chance because of the inclusion of some odorants that act by trigeminal stimulation. Olfactory threshold testing is another method of assessing olfactory function. ... side of the nose. CT or MRI of the head is required to rule out paranasal sinusitis; neoplasms of the anterior cranial fossa, nasal cavity, or paranasal sinuses; or unsuspected fractures of...
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Chapter 030. Disorders of Smell, Taste, and Hearing (Part 4) pdf
... margin and dorsum of the tongue; at the junction of the dorsum and the base of the tongue; and in the palate, epiglottis, larynx, and esophagus. Figure 30-2 Chapter 030. Disorders of Smell, Taste, ... taste buds of the anterior two-thirds of the tongue are innervated by the gustatory fibers that travel in a branch of the facial nerve (VII) called the chorda tympani. The taste buds of the posterior ... Definitions Disturbances of the sense of taste may be categorized as total ageusia, total absence of gustatory function or inability to detect the qualities of sweet, salt, bitter, or sour;...
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Chapter 030. Disorders of Smell, Taste, and Hearing (Part 9) pdf
... the cause of Chapter 030. Disorders of Smell, Taste, and Hearing (Part 9) Disorders of the Sense of Hearing Hearing loss can result from disorders of the auricle, external auditory canal, ... canal; atresia or neoplasms of the canal; perforations of the tympanic membrane; disruption of the ossicular chain, as occurs with necrosis of the long process of the incus in trauma or ... scan of temporal bone; t , MRI scan. Conductive Hearing Loss This results from obstruction of the external auditory canal by cerumen, debris, and foreign bodies; swelling of the lining of...
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