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Screening Newborns for Sickle Cell Anemia

Am J Dis Child. 1980;134(12):1180. doi:10.1001/archpedi.1980.02130240060026.
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Sir.—Rubin and Rowley's article in the Journal (1979;133:1248-1250) and Miller's editorial in the Journal (1979;133:1235-1236) on newborn screening for sickle cell anemia both suggest that in addition to the problems that can arise through difficulty in making a diagnosis of sickle cell disease during the postnatal period, there is the following problem: confusion within the medical profession both as to methods of diagnosis and definition and to the clinical picture of the varying clinical syndromes. β Thalassemia occurs in the following two forms in the black population: β+, in which varying amounts of hemoglobin (Hb) A are produced, and β°, in which no Hb A is produced. Both are characterized by a low mean corpuscular volume (MCV), an elevated Hb A2 level, and unbalanced α to β synthesis.

As Drs Rubin and Rowley indicate, the clinical picture may vary from a totally benign course to one indistinguishable from Hb


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