Anesthesia Central is an all-in-one web and mobile solution for treating patients before, during, and after surgery. This collection of drug, procedures and test information is derived from Davis’s Drug, MGH Clinical Anesthesia Procedures, Pocket Guide to Diagnostic Tests, and MEDLINE Journals. Explore these free sample topics:
-- The first section of this topic is shown below --
First Things First
- Inherited hemoglobinopathies caused by disordered Hb synthesis
- Name is based on which chain subunit is underproduced; however, this underproduction leads to overproduction of other subunits.
- So a pt with beta-thalassemia may have excess alpha, delta, gamma subunits
- Anemia is a consequence of ineffective erythropoiesis and hemolysis combined.
- Often the complications of iron overload syndromes prompt the complication leading to ICU admission.
- The overall contribution of these two processes differs in various forms of thalassemia.
- Cooley’s anemia is a severe form of beta-thalassemia that may be seen in ICU care.
- Globin reduction contributes to diminished hemoglobin molecules (hypochromasia) and small structure (microcytosis).
- Development of unchecked accumulation of subunits causes unstable molecules, leading to apoptosis and hemolysis and poor iron acclimation.
- Anemia causes excess erythropoietin, but this is counteracted by ineffective production.
- Accelerated apoptosis is major factor in dysfunctional erythropoiesis, caused by excess chain deposition in erythroid progeny.
- Beta-thalassemia is common in Mediterranean countries, North Africa, Middle East, India, and Europe.
- Alpha-thalassemia: usually of Asian origin but also seen in India, the Middle East, and Africa; pts with severe types rarely survive (hydrops fetalis)
- Both types can co-exist, usually lessening the severity of disease.