- Management and therapy of sickle cell disease
by Clarice D Reid; Samuel Charache; Bertram H Lubin*
- Managing Anemia in Pediatric Office Practice: Part 1 (PIR)
The first in a series from Pediatrics in Review on how to manage anemia in the office setting, this article instructs readers to:
- Compare and contrast the pathogenesis of iron deficiency anemia in toddlers and older children.
- Describe the genetic defects resulting in beta-thalassemia and hemoglobin E and differentiate between heterozygous and homozygous defects.
- Outline the molecular basis for alpha-thalassemia syndromes, including silent carrier, alpha-thalassemia trait, hemoglobin H, and Bart’s hemoglobinopathy.
- Describe the pathogenesis of spherocytes in herediatry spherocytosis and the reasons for the shortened red cell survival.
- Characterize the risk of "aplastic crisis" in hereditary spherocytosis and the requisite monitoring and management.
- Managing Anemia in Pediatric Office Practice: Part 2 (PIR)
After completing the second article from this series, readers should be able to:
- Recognize the signs and symptoms of sickle cell vaso-occlusive crisis.
- Explain the factors contributing to the pathogenesis of sickle cell vaso-occlusion.
- Delineate the reasons for the heightened susceptibility to infection in sickle cell disease.
- Describe the biochemical basis for hemolysis in glucose-6-phosphate dehydrogenase deficiency.
- Compare and contrast Diamond-Blackfan anemia and transient erythroblastopenia of childhood.
* Denotes hard copies available in residency office or section offices.
** For references available through MDConsult, visit this page first and click on open generic version. Then, in a separate browser window, click on one of the links below. You can also obtain a personal login through the John Crerar library.
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