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PEDIATRICS Vol. 109 No. 3 March 2002, pp. 526-535

AMERICAN ACADEMY OF PEDIATRICS

Health Supervision for Children with Sickle Cell Disease

Sickle cell disease (SCD) is a group of complex genetic disorders with multisystem manifestations. This statement provides pediatricians in primary care and subspecialty practice with an overview of the genetics, diagnosis, clinical manifestations, and treatment of SCD. Specialized comprehensive medical care decreases morbidity and mortality during childhood. The provision of comprehensive care is a time-intensive endeavor that includes ongoing patient and family education, periodic comprehensive evaluations and other disease-specific health maintenance services, psychosocial care, and genetic counseling. Timely and appropriate treatment of acute illness is critical, because life-threatening complications develop rapidly. It is essential that every child with SCD receive comprehensive care that is coordinated through a medical home with appropriate expertise.

Section on Hematology/Oncology and Committee on Genetics

Key Words: SCD, sickle cell disease • HbS, sickle hemoglobin • HbF, fetal hemoglobin • HbSS, sickle cell anemia • HbSC, sickle-hemoglobin C disease • HbF, fetal hemoglobin • HPLC, high-performance liquid chromatography • AAP, American Academy of Pediatrics • CNS, central nervous system • CBC, complete blood cell • TCD, transcranial Doppler • HbA2, hemoglobin A2 • MCV, mean corpuscular volume • Hib, Haemophilus influenzae type b • TIA, transient ischemic attack


Statement of reaffirmation:

AAP Publications Retired and Reaffirmed

Pediatrics 117: 1846-1847. [Full Text]



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