Hb G Philadelphia (alpha68 Asn leads to Lys) is widely distributed in black people but is uncommon in North-Africa. Only one case has been previously described in an Arab immigrant. The latter and our propositus originated from North-East morocco. Hb G alpha Philadelphia is stable. The abnormal hemoglobin represented 28% of total hemoglobin in the hemolysate of the propositus, a 7 month old child. At birth, 4 fractions were detected on electrophoresis: Hb A, Hb F, Hb G Philadelphia, which migrated like Hb S, and a mutant alpha2Ggamma2. The oxygen affinity of Hb G Philadelphia was slightly elevated. Cooperativity and Bohr effect were normal. The abnormal hemoglobin was also detected in the father of the propositus in the heterozygote state: its clinical and hematological data were normal. No evidence of thalassemia trait was found in the family. The percentage of abnormal hemoglobin obtained in the propositus and his father is in accordance with the presence of two loci for the alpha chain on the homologous chromosome.

Electrophoresis, Cellulose Acetate↗

PubMedDate not supplied

Dealing with the preschool-age patient.

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Child, Preschool↗

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Iron deficiency and dental caries. A pilot study.

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Child, Preschool↗

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[Prenatal diagnosis of hemoglobinopathies].

Fetal red blood cells are necessary for the antenatal diagnosis of hemoglobinopathies. Fetal blood may be obtained in utero, by amniocentesis or fetoscopy, from the 17th to the 20th week of pregnancy. At this time, biochemical techniques may detect bêta-thalassemia major (fetal synthesis of hemoglobin btaA chain totally absent or less than 2% and sickle cell disease (fetal synthesis of btaS chain, without any production of bêtaA chain). When fetal blood has been severely contaminated by maternal blood, purification techniques allow enrichment of the sample in fetal red blood cells by the elimination of adult reticulocytes which may lead to errors in diagnosis.

Erythrocytes↗