Literature DB >> 2040350

Percentile curves for red cell indices of beta zero-thalassaemia heterozygotes in infancy and childhood.

R Galanello1, F Lilliu, F Bertolino, A Cao.   

Abstract

This paper describes the percentile curves for red blood cell (RBC) count, Hb, mean corpuscular volume (MCV) and mean corpuscular haemoglobin (MCH) values of beta zero-thalassaemia heterozygotes during infancy, childhood and adolescence. Hb values were about 2 g/dl below those of normal controls with a progressive increase with age paralleling the normal developmental trend. The Hb increase with age was due to a progressive rise in the Hb content per cell, the number of RBC remaining nearly constant. MCV and MCH values also increased with age with a pattern parallel to normal control. Because of the high prevalence of alpha-thalassaemia in the Sardinian population, to which all the subjects investigated belong, the 3rd MCH-MCV percentile curves of normal overlap the 97th curve of beta-thalassaemia heterozygotes. The HbA2 levels, however, were always increased as compared to normal. These results confirm in children than screening for heterozygous beta-thalassaemia in populations with a high incidence of alpha and beta-thalassaemia by MCV-MCH determination may overlook a sizeable proportion of beta-thalassaemia carriers. The knowledge of the extent of variation of RBC indices of beta-thalassaemia heterozygotes during infancy, childhood and adolescence, is very useful for the evaluation of a child presenting with a mild microcytic anaemia.

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Year:  1991        PMID: 2040350     DOI: 10.1007/bf02093721

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  13 in total

1.  Microchromatography of hemoglobins. II. A simplified procedure for the determination of hemoglobin A2.

Authors:  T H Huisman; W A Schroeder; A N Brodie; S M Mayson; J Jakway
Journal:  J Lab Clin Med       Date:  1975-10

2.  Heterozygous beta-thalassemia: relationship between the hematological phenotype and the type of beta-thalassemia mutation.

Authors:  C Rosatelli; G B Leoni; T Tuveri; M T Scalas; A Mosca; R Galanello; D Gasperini; A Cao
Journal:  Am J Hematol       Date:  1992-01       Impact factor: 10.047

3.  Serum iron concentration and transferrin saturation in the diagnosis of iron deficiency in children: normal developmental changes.

Authors:  M A Koerper; P R Dallman
Journal:  J Pediatr       Date:  1977-12       Impact factor: 4.406

4.  Thalassemia intermedia resulting from a mild beta-thalassemia mutation.

Authors:  M C Rosatelli; L Oggiano; G Battista Leoni; T Tuveri; A Di Tucci; M T Scalas; F Dore; P Pistidda; A Massa; M Longinotti
Journal:  Blood       Date:  1989-02       Impact factor: 22.113

5.  Hematocrit: maturity relationship in adolescence.

Authors:  W A Daniel
Journal:  Pediatrics       Date:  1973-09       Impact factor: 7.124

6.  [Determination of serum iron and iron-binding capacity without deproteinization].

Authors:  K Lauber
Journal:  Z Klin Chem Klin Biochem       Date:  1965-06

7.  Prospective study of red blood cell indices, hemoglobin A2, and hemoglobin F in infants heterozygous for Beta-thalassemia.

Authors:  R Galanello; M A Melis; R Ruggeri; A Cao
Journal:  J Pediatr       Date:  1981-07       Impact factor: 4.406

8.  Hematology of beta-thalassemia trait--age-related developmental aspects and intrafamilial correlations.

Authors:  B W Berman; A K Ritchey; J F Jekel; A D Schwartz; D K Guiliotis; H A Pearson
Journal:  J Pediatr       Date:  1980-12       Impact factor: 4.406

9.  Phenotypic effect of heterozygous alpha and beta 0-thalassemia interaction.

Authors:  M A Melis; M Pirastu; R Galanello; M Furbetta; T Tuveri; A Cao
Journal:  Blood       Date:  1983-07       Impact factor: 22.113

10.  beta zero thalassemia in Sardinia is caused by a nonsense mutation.

Authors:  R F Trecartin; S A Liebhaber; J C Chang; K Y Lee; Y W Kan; M Furbetta; A Angius; A Cao
Journal:  J Clin Invest       Date:  1981-10       Impact factor: 14.808

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Authors:  Shahina Daar; David Gravell
Journal:  Sultan Qaboos Univ Med J       Date:  2006-06

Review 2.  Krüppeling erythropoiesis: an unexpected broad spectrum of human red blood cell disorders due to KLF1 variants.

Authors:  Andrew Perkins; Xiangmin Xu; Douglas R Higgs; George P Patrinos; Lionel Arnaud; James J Bieker; Sjaak Philipsen
Journal:  Blood       Date:  2016-02-22       Impact factor: 22.113

  2 in total

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