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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Iron deficiency in Brazilian infants with sickle cell disease

Priscila C Rodrigues1, Rocksane C Norton, Mitiko Murao

  • 1Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG. Médica, Fundação Hemominas, Belo Horizonte, MG. Brazil. <vianamb@gmail.com>

Jornal De Pediatria
|August 16, 2011
PubMed

Insights

Most infants with sickle cell disease (SCD) do not have iron deficiency, but some do, especially those with SC hemoglobin. Iron supplementation may be considered but should stop after blood transfusions.

Area of Science:

  • Hematology
  • Pediatrics
  • Nutritional Science

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder.
  • Iron metabolism in infants with SCD requires careful monitoring.
  • Prophylactic iron supplementation guidelines for infants with SCD are not well-established.

Purpose of the Study:

  • To evaluate iron deficiency or overload in infants with SCD.
  • To inform decisions regarding prophylactic iron supplementation for this population.

Main Methods:

  • A cross-sectional and retrospective study of 135 infants under 2 years old with SS or SC hemoglobin.
  • Assessed iron status using mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), transferrin saturation (TS), and ferritin.
  • 12.6% of infants received blood transfusions before laboratory tests.

Main Results:

  • SC hemoglobin infants showed significantly lower ferritin and TS (p < 0.001).
  • 17.8% of infants met criteria for iron deficiency, predominantly those with SC hemoglobin (p = 0.003).
  • Iron deficiency was present in 19.5% of infants not transfused; elevated ferritin was observed in 11.3%, mostly transfused infants.

Conclusions:

  • Most infants with SCD do not develop iron deficiency, but a notable deficit exists in some.
  • Infants with SCD, particularly SC hemoglobin type, may benefit from prophylactic iron.
  • Iron supplementation should be discontinued following the first blood transfusion in infants with SCD.
Abstract

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