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Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7e
Chapter 135. Sickle Cell Disease
Sections: Sickle Cell Disease: Introduction, Extremity Pain, Abdominal Pain, Respiratory Distress and Chest Pain, Fever, Anemia, Neurologic Complaints, GU Complaints, Treatment, Acknowledgments, References.
Topics Discussed: sickle cell anemia with crisis; sickle cell disease.
Excerpt:"Sickle cell anemia (SCA) is found primarily in children of African,
Arab, or Mediterranean descent. It is caused by a single amino acid
substitution in the sixth position of the  -globulin
chain of hemoglobin A (HbA). Children homozygous for this genetic
abnormality have classic hemoglobin SS (HbSS) disease, whereas heterozygotes
for HbS with a second -globulin chain that is
normal are considered to have sickle cell trait. Other patients
may be heterozygous for HbS coupled with a heterozygous abnormality
causing HbC or -thalassemia. In general, patients
with HbSC and the 90% of S/-thalassemia
patients with some normal chains have fewer infectious
and ischemic complications than patients with HbSS...."
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