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Pulmonary complications of sickle cell disease in children

Jason B Caboot1, Julian L Allen

  • 1Division of Pulmonary Medicine and Cystic Fibrosis Center, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399, USA.

Current Opinion in Pediatrics
|May 14, 2008
PubMed

Insights

Sickle cell disease (SCD) significantly impacts lung health in children, leading to conditions like asthma, acute chest syndrome, and pulmonary hypertension. Understanding these pulmonary complications is crucial for improving treatment strategies and patient outcomes.

Area of Science:

  • Pulmonary Medicine
  • Pediatric Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is a prevalent genetic disorder affecting 1 in 600 African-Americans.
  • The lungs are a primary target organ in SCD, contributing to significant childhood morbidity and mortality.
  • Pulmonary complications are a major concern for children with SCD.

Purpose of the Study:

  • To review recent literature on pulmonary complications in pediatric sickle cell disease.
  • To highlight the associations between SCD and various respiratory conditions.
  • To emphasize the need for further research into the interrelationships of these pulmonary issues.

Main Methods:

  • Literature review of recent studies on sickle cell disease and pulmonary manifestations.
  • Synthesis of evidence linking asthma, airway hyperreactivity, acute chest syndrome, and pulmonary hypertension with SCD.
  • Analysis of findings related to sleep-disordered breathing in children with SCD.

Main Results:

  • Strong associations found between asthma, airway hyperreactivity, and SCD.
  • Acute chest syndrome (ACS) in SCD is linked to worse pulmonary function and vaso-occlusive crisis.
  • Pulmonary hypertension and sleep-disordered breathing are increasingly recognized in children with SCD.

Conclusions:

  • Key pulmonary complications in SCD include airway hyperreactivity, ACS, chronic sickle lung disease, pulmonary hypertension, and sleep-disordered breathing.
  • Understanding the interplay between these conditions is vital for developing effective therapies.
  • Further research is needed to elucidate the mechanisms and improve management of pulmonary issues in pediatric SCD.
Abstract

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