Sickle hemoglobinopathies

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P A Lane1

  • 1Colorado Sickle Cell Treatment and Research Center, University of Colorado Health Sciences Center, Denver 80262.

Current Opinion in Pediatrics
|February 1, 1993
PubMed

Insights

Neonatal screening and comprehensive care significantly reduce sickle hemoglobinopathy mortality. However, new research offers hope for better management of complications and potential curative therapies.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Genetics

Background:

  • Neonatal screening and comprehensive care, including penicillin prophylaxis and immunizations, have greatly decreased infant mortality from sickle hemoglobinopathies.
  • Despite advances in understanding pathophysiology, current treatments inadequately prevent acute noninfectious complications (e.g., pain crises, strokes) and chronic organ damage.

Purpose of the Study:

  • To review recent advancements in understanding sickle hemoglobinopathies.
  • To highlight improvements in managing acute complications and the emergence of potentially curative therapies.

Main Methods:

  • Review of recent publications on sickle hemoglobinopathies.
  • Analysis of current therapeutic approaches and ongoing clinical trials.

Main Results:

  • Increased knowledge of factors contributing to clinical heterogeneity in sickle hemoglobinopathies.
  • Modest improvements in identifying and treating acute complications.
  • Emerging hope for curative treatments, with ongoing trials for hydroxyurea and bone marrow transplantation.

Conclusions:

  • While neonatal screening and supportive care have improved survival, significant challenges remain in managing disease complications.
  • Recent research offers promising avenues for more effective treatments and potential cures for sickle hemoglobinopathies.

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