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[S hemoglobinopathies in Argentina].

M S Abreu1, J A Peñalver

  • 1División Hemato-Oncología, Hospital General de Niños R. Gutiérrez, Buenos Aires, Argentina.

Medicina
|January 1, 1992
PubMed
Summary
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Sickle cell hemoglobinopathies are prevalent in Argentina, particularly in northern regions. This study analyzed 116 cases over 30 years, revealing geographic origins and hematological characteristics.

Area of Science:

  • Hematology
  • Genetics
  • Public Health

Background:

  • Sickle cell hemoglobin (Hb S) is a common abnormal hemoglobin, with distribution linked to malaria endemicity.
  • Understanding sickle cell hemoglobinopathies in Argentina is crucial due to its unique demographic landscape.

Purpose of the Study:

  • To characterize sickle cell hemoglobinopathies diagnosed over a 30-year period at R. Gutiérrez Children's Hospital in Argentina.
  • To analyze the prevalence, geographic distribution, and hematological features of Hb S diagnoses.

Main Methods:

  • Retrospective review of 925 diagnosed hemoglobinopathies over 30 years.
  • Hb S diagnoses confirmed by sickling tests, with additional hematological parameters measured.
  • Hemoglobin A2 and S quantified by electrophoresis; Hb F measured by alkali denaturation.

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Main Results:

  • Hb S was found in 116 (12.5%) of diagnosed hemoglobinopathies.
  • Sickle-cell trait (AS) was the most common (75 cases), primarily originating from northern/central Argentina and neighboring countries.
  • AS cases with abnormal red cell morphology exhibited significantly lower Hb, PCV, MCH, and MCV.

Conclusions:

  • Sickle cell hemoglobinopathies, particularly AS trait, are present in Argentina, with distinct geographic and demographic associations.
  • Hematological values in AS trait are generally normal, but red cell morphology alterations correlate with reduced hematological parameters.