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

<h4>Background</h4>Sickle cell disease (SCD) is major inherited disease linked to a pro-inflammatory state, with a widespread involvement seen in all organ systems with lungs being no different. This research aims to analyze pulmonary function tests and fractional exhaled nitric oxide (F...

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Main Authors: Abinaya Kannan, Gaurav Sarnaik, Nikita Agarwal, Atul Jindal
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2025-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0329064
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author Abinaya Kannan
Gaurav Sarnaik
Nikita Agarwal
Atul Jindal
author_facet Abinaya Kannan
Gaurav Sarnaik
Nikita Agarwal
Atul Jindal
author_sort Abinaya Kannan
collection DOAJ
description <h4>Background</h4>Sickle cell disease (SCD) is major inherited disease linked to a pro-inflammatory state, with a widespread involvement seen in all organ systems with lungs being no different. This research aims to analyze pulmonary function tests and fractional exhaled nitric oxide (FeNO) levels of children diagnosed with SCD and comparing them with healthy controls.<h4>Methods</h4>The study involved 100 children with SCD in stable state of health, without pain, crises, or acute illnesses for at least 1-month, and aged between 6-20 years and 100 age- and height-matched controls. Those with spinal deformities, acute or chronic cardiorespiratory symptoms, and cigarette smoking (in parents) were excluded. Measurements of forced vital capacity(FVC) and forced expiratory volume in one second(FEV1) were conducted using an automated single-breath vitalograph. FeNO was recorded using a hand-held device(NIOX MINO). Data werecollected and analyzed.<h4>Results</h4>Children with SCD had significantly lower pulmonary function values compared to controls: FEV1 median difference: 33.5% (95% CI: 27.2-38.0; p < 0.0001), FVC: 25.4% (95% CI: 30.0-32.25; p < 0.0001), FEV1/FVC: 0.088 (95% CI: 0.075-0.083; p < 0.0001), Peak Expiratory Flow Rate (PEFR): 24.8% (95% CI: 16.38-33.8; p < 0.0001), FeNO: 8.17 ppb (95% CI: 5.77-12.65; p < 0.0001). Pulmonary function test (PFT) abnormalities were associated with younger age (p = 0.0022). Age (p = 0.0011) was significantly associated with PFT severity, while blood transfusion frequency, and fractional exhaled nitric oxide (FeNO) levels were not. Increased weight (p = 0.001) and longer duration of hydroxyurea (p = 0.011) were associated with improved PFT severity (based on FEV1 z-scores).<h4>Conclusions</h4>Children with SCD often exhibit restrictive, obstructive, or mixed pulmonary function patterns. FeNO levels donot correlate with PFT severity.
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spelling doaj-art-3971c3875ea347e6bc5de33eb50511122025-08-20T03:57:59ZengPublic Library of Science (PLoS)PLoS ONE1932-62032025-01-01207e032906410.1371/journal.pone.0329064The pattern of lung function tests in children with sickle cell disease: A case-control study.Abinaya KannanGaurav SarnaikNikita AgarwalAtul Jindal<h4>Background</h4>Sickle cell disease (SCD) is major inherited disease linked to a pro-inflammatory state, with a widespread involvement seen in all organ systems with lungs being no different. This research aims to analyze pulmonary function tests and fractional exhaled nitric oxide (FeNO) levels of children diagnosed with SCD and comparing them with healthy controls.<h4>Methods</h4>The study involved 100 children with SCD in stable state of health, without pain, crises, or acute illnesses for at least 1-month, and aged between 6-20 years and 100 age- and height-matched controls. Those with spinal deformities, acute or chronic cardiorespiratory symptoms, and cigarette smoking (in parents) were excluded. Measurements of forced vital capacity(FVC) and forced expiratory volume in one second(FEV1) were conducted using an automated single-breath vitalograph. FeNO was recorded using a hand-held device(NIOX MINO). Data werecollected and analyzed.<h4>Results</h4>Children with SCD had significantly lower pulmonary function values compared to controls: FEV1 median difference: 33.5% (95% CI: 27.2-38.0; p < 0.0001), FVC: 25.4% (95% CI: 30.0-32.25; p < 0.0001), FEV1/FVC: 0.088 (95% CI: 0.075-0.083; p < 0.0001), Peak Expiratory Flow Rate (PEFR): 24.8% (95% CI: 16.38-33.8; p < 0.0001), FeNO: 8.17 ppb (95% CI: 5.77-12.65; p < 0.0001). Pulmonary function test (PFT) abnormalities were associated with younger age (p = 0.0022). Age (p = 0.0011) was significantly associated with PFT severity, while blood transfusion frequency, and fractional exhaled nitric oxide (FeNO) levels were not. Increased weight (p = 0.001) and longer duration of hydroxyurea (p = 0.011) were associated with improved PFT severity (based on FEV1 z-scores).<h4>Conclusions</h4>Children with SCD often exhibit restrictive, obstructive, or mixed pulmonary function patterns. FeNO levels donot correlate with PFT severity.https://doi.org/10.1371/journal.pone.0329064
spellingShingle Abinaya Kannan
Gaurav Sarnaik
Nikita Agarwal
Atul Jindal
The pattern of lung function tests in children with sickle cell disease: A case-control study.
PLoS ONE
title The pattern of lung function tests in children with sickle cell disease: A case-control study.
title_full The pattern of lung function tests in children with sickle cell disease: A case-control study.
title_fullStr The pattern of lung function tests in children with sickle cell disease: A case-control study.
title_full_unstemmed The pattern of lung function tests in children with sickle cell disease: A case-control study.
title_short The pattern of lung function tests in children with sickle cell disease: A case-control study.
title_sort pattern of lung function tests in children with sickle cell disease a case control study
url https://doi.org/10.1371/journal.pone.0329064
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