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CLINICAL FEATURES
CLINICAL FEATURES
HEMOGLOBINOPATHIES
Hemoglobin is a conjugated protein which contains a globin fraction bound to 4 heme moieties. There are
4 polypeptide chains within the globin fraction-namely alpha, beta, gamma
and delta. Composition of a normal adult hemoglobin HbA (a, B2)= 96.98%, HbF (a2 Y2) = 0.5-
0.8% and HbA, 1.5-3.7%. In normal fetal hemoglobin, the beta chains replaced by two gamma
chains (a2 y2). The last two vari constitute not more than 5% in normal human adults.
=
Hemoglobinopathies are inherited specific biochemi disorders (quantity or quality) within
the polypeptide cha of globin fraction. Two common varieties are met. Sickle disease is
inherited structural abnormality involvingprim