While those who develop Hb Bart's
hydrops fetalis die either in utero or after birth due to severe intrauterine anemia.12
Rousseau and Mathew  performed follow-up DNA analyses on neonates with Hb Bart's
detected in cord blood samples.
Fetus of pregnant patients with Hb Bart's
usually die in utero.
Hydrops fetalis resulting from Hb Bart's disease and molecular characterization of Hb H disease in Turkey have been reported (5); however, the prevalence and distribution of deletional alpha-thalassemia, which is responsible for Hb Bart's disease, is not known.
The couples who have an offspring with Hb Bart's disease represent the at-risk couples who are carriers of deletional alpha-thalassemia.
IEF provides excellent separation of many hemoglobin variants and detects fast-migrating or low concentration hemoglobin variants such as Hb H, Hb Bart's, and delta chain variants.
* Hb H and Hb Bart's are more readily detected and measured by CE than by the HPLC method (4)
Still, a 28-year-old woman from Lumpoon Province in Northern Thailand who was diagnosed with homozygous Hb Hope and HbH disease (--[sup.SEA]/-[[alpha].sup.3.7]) did express a severe microcytic/hypochromic anemia (Hb=5.7g/dL, MCV=62 fL, MCH=16.8pg), elevated HbF (4.3%), Hb Hope (78.1%), with elevated Hb Bart's at birth and HbH in adulthood.
Another clinical concern with thalassemia compound heterozygotes involves the potential of Hb Bart's hydrops fetalis occurring in an infant born to unsuspecting parents.
Quantitation of Hb [A.sub.2], Hb E, Hb A, and Hb F was performed by the BTS program; quantitation of Hb Bart's was performed by the ATS program on the Bio-Rad VARIANT, a fully automated HPLC system that uses double wavelength detection (415 and 690 nm).
In the cord-blood study Hb types, including Hb Bart's and Hb Constant Spring (Hb CS), were also confirmed by isoelectric focusing (Isolab).
The clinical manifestations range from mild anemia with microcytosis (thalassemia trait) to fatal severe anemia (Hb Bart's
hydrops fetalis (7) or [beta]-thalassemia major).