title: Hb Nottingham or alpha 2 beta 2 98 (FG5) Val-->Gly in a Czech child.
abstract: We report a fourth case of Hb Nottingham [alpha 2 beta 2 98 (FG5) Val-->Gly] observed in an 8-year-old girl in the Czech Republic with clinical and laboratory symptoms of severe hemolytic anemia. The unstable hemoglobin probably represents a de novo mutation, since the parents of the patient and the two siblings do not exhibit any hematological abnormalities. Splenectomy had a beneficial effect on the degree of hemolysis, as well as on the Hb level.
authors.affiliation: Institute of Hematology and Blood Transfusion, Prague, Czech Republic.
authors.forename: V
authors.initials: V
authors.lastname: Brabec
authors.forename: K
authors.initials: K
authors.lastname: Indrák
authors.forename: H
authors.initials: H
authors.lastname: Fortová
authors.forename: J
authors.initials: J
authors.lastname: Suttnar
authors.forename: B
authors.initials: B
authors.lastname: Blazek
authors.forename: J
authors.initials: J
authors.lastname: Cermák
authors.forename: V
authors.initials: V
authors.lastname: Divoký
authors.forename: L
authors.initials: L
authors.lastname: Vepreková
authors.forename: J
authors.initials: J
authors.lastname: Zeman
mesh_terms: D000595:Amino Acid Sequence; D002851:Chromatography, High Pressure Liquid; D018153:Czech Republic; D004247:DNA / Q000032:analysis; D005260:Female; D006455:Hemoglobins, Abnormal / Q000032:analysis* / Q000235:genetics; D006801:Humans; D007223:Infant; D008969:Molecular Sequence Data; D010446:Peptide Fragments / Q000235:genetics
chemical_list: D006455:Hemoglobins, Abnormal; D010446:Peptide Fragments; C075791:hemoglobin Nottingham; D004247:DNA