Alpha thalassemia screenign in neonates by mean corpuscular volume and mean corpuscular hemoglobin determination

Schmaier, Alvin H.; Maurer, Harold M.; Johnston, Charles G.; Scott, Robert B.

doi:10.1016/s0022-3476(73)80371-3

Cited by 29 publications

(4 citation statements)

References 8 publications

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“…The mean hemoglobin level and hematocrit were also decreased in this group, but the difference was not statistically significant for the number of infants studied. A recent study has demonstrated similar hematologic abnormalities by showing that six of nine Negro neonates with low MCV and MCH values had Hb Barts levels above 2% in the newborn period (24).…”

Section: Discussionmentioning

confidence: 74%

α-Thalassemia in Negro Infants

et al. 1974

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Section: Discussionmentioning

confidence: 74%

α-Thalassemia in Negro Infants

et al. 1974

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“…There is also a strong negative correlation between the quantity of Hb Bart's and the MCH. All these findings point to the presence of a type of a-thalassaemia rather than a tran sient developmental abnormality as suggest ed by Folayan Esan [4] and Schmaier et al [16].…”

Section: Discussionmentioning

confidence: 75%

Hb Bart’s and its Significance in the South African Negro

Piliszek¹

1979

Acta Haematol

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The haematological indices of cord bloods from 430 South African Negro babies were determined by electronic cell counting and their haemoglobin (Hb) patterns examined by alkaline cellulose acetate electrophoresis. A fast-moving, anodal band, identified as Hb Bart’s, was found in 7 (l,6%) of the specimens, this being the lowest incidence of the variant yet found in an indigenous African population. The levels of Hb Bart’s ranged from 1.3 to 5.5% of the haemoglobin. These findings were confirmed by alkaline-starch gel electrophoresis and at the same time absence of the slow-moving haemoglobin, Hb Constant Spring was established. Subsequent follow-up of 4 of the infants at 4 months of postnatal life showed that the abnormal component had disappeared. The babies with Hb Bart’s had a marked microcytosis and low mean corpuscular haemoglobin levels whilst their parents showed no haematological or electrophoretic signs of _α-thalassaemia. The significance of these findings is discussed in the light of previously reported studies on various Negro groups.

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“…Globin chain synthesis study is used mostly for research purposes and is not practical for screening (2). Red cell indices are probably the easiest way to screen but they are unable to confirm the diagnosis or to detect most cases of heterozygous alpha-2 thalassemia (3)(4)(5)(6). In this paper, we compare two additional methods, the red cell osmotic fragility test (7) and modified hemoglobin (Hb) H inclusion staining (8), in screening alpha-thalassemia traits on the basis of endonuclease gene maping (1,9,10).…”

Section: Introductionmentioning

confidence: 99%

Comparison of two screening methods, modified hb h preparation and the osmotic fragility test, for alpha‐thalassemic traits on the basis of gene mapping

Lin

Jiang²,

Chien³

et al. 1991

Clinical Laboratory Analysis

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We evaluated 61 patients with two screening tests for alpha-thalassemia traits on the basis of endonuclease gene mapping. Comparing these two methods--the osmotic fragility test of the red cell and modified hemoglobin H inclusion staining for the sensitivity--we found that the latter was much superior to the former with 100% sensitivity in detecting heterozygous alpha-1 thalassemia and it was also specific as a confirmatory test for thalassemia traits. Red cell indices are still the basic screening tool and can be used together with modified Hb H inclusion staining. The osmotic fragility test was not better than the red cell indices and was not confirmatory. Besides the MCV, RBC, and discrimination functions, we found that RBC distribution width-standard deviation (RDW-SD) was consistently low in heterozygous alpha-1 thalassemia but not in heterozygous alpha-2 thalassemia. None of the above tests was shown to be really helpful in screening in the latter situation. We conclude that the modified Hb H inclusion staining is superior to the osmotic fragility test in screening of alpha-1 thalassemia.

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Alpha thalassemia screenign in neonates by mean corpuscular volume and mean corpuscular hemoglobin determination

Cited by 29 publications

References 8 publications

α-Thalassemia in Negro Infants

α-Thalassemia in Negro Infants

Hb Bart’s and its Significance in the South African Negro

Comparison of two screening methods, modified hb h preparation and the osmotic fragility test, for alpha‐thalassemic traits on the basis of gene mapping

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