Hemoglobin Bart's Disease in an Italian Boy

HEMOGLOBIN is a tetramer consisting of two pairs of globin chains, each of which is associated with a heme group. In adults, the predominant hemoglobin — hemoglobin A — is composed of two α-globin and two β-globin chains (α2β2); the principal fetal hemoglobin — hemoglobin F — is made up of two α-globin and two γ-globin chains (α2γ2). α-Globin chains are coded for by two adjacent α-globin genes on the short arm of each chromosome 16. The embryonic ζ-globin chains, which resemble α-globin chains, are coded for by a ζ-globin gene on . . .