The pattern of lung function tests in children with sickle cell disease: A case-control study

Area of Science:

• Pediatric Pulmonology
• Hematology
• Inherited Disorders

Background:

• Sickle cell disease (SCD) is an inherited disorder associated with a pro-inflammatory state affecting multiple organ systems, including the lungs.
• Pulmonary complications are a significant concern in children with SCD, impacting their overall health and quality of life.

Purpose of the Study:

• To analyze pulmonary function tests (PFTs) and fractional exhaled nitric oxide (FeNO) levels in children with SCD.
• To compare PFTs and FeNO levels between children with SCD and healthy controls.
• To investigate factors associated with pulmonary function abnormalities in pediatric SCD.

Main Methods:

• A study involving 100 children with stable SCD (aged 6-20 years) and 100 age- and height-matched healthy controls.
• Exclusion criteria included spinal deformities, cardiorespiratory symptoms, and parental smoking.
• Measurements included forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and fractional exhaled nitric oxide (FeNO).

Main Results:

• Children with SCD demonstrated significantly lower FEV1, FVC, FEV1/FVC ratio, and PEFR compared to controls (p < 0.0001 for all).
• FeNO levels were also significantly lower in children with SCD (p < 0.0001).
• Pulmonary function abnormalities were associated with younger age, while increased weight and longer hydroxyurea duration correlated with improved PFTs.

Conclusions:

• Children with SCD frequently present with restrictive, obstructive, or mixed pulmonary function patterns.
• Fractional exhaled nitric oxide (FeNO) levels do not correlate with the severity of pulmonary function test abnormalities in pediatric SCD.