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Airway and alveolar nitric oxide production, lung function, and pulmonary blood flow in sickle cell disease

Alan Lunt1,2, Na'eem Ahmed1, Gerrard F Rafferty1

  • 1Division of Asthma, Allergy and Lung Biology, MRC Centre for Allergic Mechanisms in Asthma, King's College London, London, UK.

Pediatric Research
|October 23, 2015
PubMed

Insights

Children with sickle cell disease (SCD) show airway obstruction not linked to asthma. Increased alveolar nitric oxide (NO) production and pulmonary blood flow, not airway NO, were observed in SCD patients.

Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Biomedical Engineering

Background:

  • Children with sickle cell disease (SCD) frequently exhibit obstructive lung function abnormalities.
  • These abnormalities may stem from asthma or increased pulmonary blood volume, necessitating accurate diagnosis for effective treatment.
  • Exhaled nitric oxide (FeNO) is elevated in asthma but can also increase due to alveolar production, complicating its interpretation in SCD.

Purpose of the Study:

  • To investigate differences in airway and alveolar nitric oxide (NO) production between children with SCD and healthy controls.
  • To determine the underlying mechanisms of lung function abnormalities in pediatric SCD patients.
  • To differentiate between asthma-related and other causes of airway obstruction in SCD.

Main Methods:

  • Assessed lung function, including respiratory system resistance.
  • Measured airway NO flux and alveolar NO production.
  • Quantified effective pulmonary blood flow in 18 SCD children and 18 matched controls.

Main Results:

  • SCD children demonstrated significantly higher respiratory system resistance, alveolar NO production, and pulmonary blood flow compared to controls.
  • No significant difference in airway NO flux was found between SCD children and controls.
  • In SCD children, alveolar NO production and concentration correlated with pulmonary blood flow.

Conclusions:

  • Elevated airway NO flux was not observed in SCD children, and it did not correlate with airway obstruction.
  • These findings suggest that airway obstruction in some SCD children may not be primarily due to asthma.
  • Increased alveolar NO production and pulmonary blood flow are key features of lung dysfunction in pediatric SCD.
Abstract

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