Characterising nutrition status in a regional cohort of children with primary ciliary dyskinesia

Luise V Marino, Amanda Harris, Carolyn Johnstone, Amanda Friend, Victoria Keenan, Hannah Wilkins, Jane Lucas, Philip Calder, Woolf Walker

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Abstract

Introduction: Primary ciliary dyskinesia (PCD) is a rare, heterogeneous genetic disorder where impaired mucociliary clearance is caused by dysfunctional motile cilia. There is limited characterisation of the nutritional status of children with PCD but one recent paper found that lower body mass index (BMI) was associated with worse lung function (FEV1). Bioimpedance spectroscopy (BIS) has been used to identify patients at risk of needing nutritional intervention but this has not been assessed in PCD patients. Aim To assess, in depth, the nutritional status of children with PCD. Methods: All children (n=40) with PCD, -2 z scores (mean (SD) -0.49 z scores (±1.1) vs. 0.2 a score (±0.7), p=0.05. Significant differences were seen in BIS measurements (bioelectrical phase angle 500) in children with a fat free mass index -2 z scores (p=0.002), these patients would not have been identified as at nutritional risk by BMI alone. PCD patients had a higher incidence of Vitamin D insufficiency (
Original languageEnglish
JournalEuropean Respiratory Journal, Supplement
Volume52
Issue numberS62
DOIs
Publication statusPublished - 19 Nov 2018

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