tailieunhanh - Chapter 099. Disorders of Hemoglobin (Part 10)

Low-affinity hemoglobins should be considered in patients with cyanosis or a low hematocrit with no other reason apparent after thorough evaluation. The P 50 test confirms the diagnosis. Counseling and reassurance are the interventions of choice. Methemoglobin should be suspected in patients with hypoxic symptoms who appear cyanotic but have a Pa O2 sufficiently high that hemoglobin should be fully saturated with oxygen. A history of nitrite or other oxidant ingestions may not always be available; some exposures may be unapparent to the patient, and others may result from suicide attempts. The characteristic muddy appearance of freshly drawn blood can be. | Chapter 099. Disorders of Hemoglobin Part 10 Low-affinity hemoglobins should be considered in patients with cyanosis or a low hematocrit with no other reason apparent after thorough evaluation. The P50 test confirms the diagnosis. Counseling and reassurance are the interventions of choice. Methemoglobin should be suspected in patients with hypoxic symptoms who appear cyanotic but have a PaO2 sufficiently high that hemoglobin should be fully saturated with oxygen. A history of nitrite or other oxidant ingestions may not always be available some exposures may be unapparent to the patient and others may result from suicide attempts. The characteristic muddy appearance of freshly drawn blood can be a 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 levels 15 levels 60 are usually lethal. Intravenous injection of 1 mg kg of methylene blue is effective emergency therapy. Milder cases and follow-up of severe cases can be treated orally with methylene blue 60 mg three to four times each day or ascorbic acid 300-600 mg d . Thalassemia Syndromes Introduction The thalassemia syndromes are inherited disorders of a- or 0-globin biosynthesis. The reduced supply of globin diminishes production of hemoglobin tetramers causing hypochromia and microcytosis. Unbalanced accumulation of a and 0 subunits occurs because the synthesis of the unaffected globins proceeds at a normal rate. Unbalanced chain accumulation dominates the clinical phenotype. Clinical severity varies widely depending on the degree to which the synthesis of the affected globin is impaired altered synthesis of other globin chains and coinheritance of other abnormal globin alleles. Clinical Manifestations of 0-Thalassemia Syndromes Mutations causing thalassemia can affect any step in the pathway of globin gene expression transcription processing of the mRNA precursor translation and .

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• Disorders of Hemoglobin
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