A-thalassemia trait

Haploinsufficiency, or when 50% of normal gene activity is inadequate a-Thalassemia trait and the a-globin gene P-Thalassemia trait and the P-globin gene... 

Hydrops fetalis 0 a-Thalassemia trait 0.25 Lethal (death Cord blood mostly Hb... 

Hemoglobin H disease —/-a 1 a-Thalassemia trait 0.25 Severe to Cord blood about 25%... 

The heterozygous state is also known as a-thalassemia trait. The a-chain genes are present as two copies per file ///C /...s%20and%20Settings/Bariski/Desktop/26134 9780471022053 9780585329482 0471022055/files/page 77.html[ 12/10/2009 10 12 40]... 

G7. Gerald, P. S., and Diamond, L. K., A new hereditary hemoglobinopathy (the Lepore trait) and its interaction with thalassemia trait. Blood 13, 835-844 (1958). 

M3. Malamos, B., Fessas, P., and Stamatoyannopoulos, G., Types of thalassemia-trait carriers as revealed by a study of their incidence in Greece. Brit. J. Haematol. 8, 5-14 (1962). 

Guidelines for the investigation of the a and P thalassemia traits. The thalassemia working party of the BCSH General Hematology Task Force. J Clin Pathol 1994 47 289-95. 

Ayi, K., Turrini, F., Piga, A., and Arese, P. (2004). Enhanced phagocytosis of ring-parasitized mutant erythrocytes a common mechanism that may explain protection against falciparum malaria in sickle trait and beta-thalassemia trait. Blood 104,3364—3371. 

Both a- and (b-thalassemia trait usually confer some protection from malaria on the heterozygote hence, the mutation survives and can be found in areas of high malaria incidence. 

Observational studies The safety of topical silver sulfadiazine has been reviewed and the adverse effects of the silver and sulfadiazine components discussed [182 ]. Allergic reactions with cross-sensitivity to antibiotic sulfonamides can occur, but there have been no reports of severe allergic reactions. Hemolytic anemia in G6PD-defi-cient patients can occur. Methemoglobinemia occurred in a 3-year-old child with bums and a dominant beta thalassemia trait treated with silver sulfadiazine. 

Krishnaja, A. P. Sharma, N. K. Heterogeneity in chemical mutagen-induced chromosome damage after G2 phase exposure to bleomycin, Ara-C and gentian violet in cultured lymphocytes of P-thalassemia traits. Mutat. Res. 1995, 331, 143-148. 

Figure 7. Microchromatography on DEAE-cellulose. A, The levels of Hb-At in normal adults, in p thalassemia heterozygotes, in Hb S traits, and in patients with sickle cell anemia and HbS-p-thalassemia (Georgia survey). B, The levels of Hb-At in normal adults and in p-thalassemia heterozygotes (Macedonia survey).

Figure 12, Heat denaturation curves of hemoglobin from three members of a family with Hb-Leslie, a newly discovered unstable variant with a deletion of residue 131 of the p-chain, G.P.Sr. has Hb-Leslie p-thalassemia the %Hb-Leslie is 85% (DEAE-Sephadex chromatography) Gr.P. has Hb-LesUe- Hb-C %Hb--Leslie is 28% M.B. fm Hb LesUe trait %Hb Leslie is 28%,...

Silvestroni, E., Bianco, I., and Baglioni, C., Interaction of hemoglobin Lepore with sickle cell trait and microcythemia (thalassemia) in a southern Italian family. Blood 25, 457-469 (1965). 

Sturgeon, P., Itano, H. A., and Valentine, W. N., Chronic hemolytic anemia associated with thalassemia and sickling traits. Blood 7, 350-357 (1952). 

Figure 31-12 HPLC chromatograms obtained on the Bio-Rad Variant p-Tha short program for a, Hb Bart s b, pAthaiassemia major c, B -thalassemia homozygous E d, Hb H e, homozygous S f,S trait g, homozygous C h, C trait /, Hb S-Hb G Philadelphia, (from Qarke GM, Trefor N, Higgins TN. Laboratory Investigation of Hemogiobinopatbies and Thalassemias Review and Update. Clin Chem 2000 46 1284-90.)...

E. Thalassemia is autosomal recessive so Micawley Talltwin can get the disease only if both the parents were to be heterozygotes (or a carrier) of that particular trait. 

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