Nutrients
Association of Dysphagia and Oral Involvement with Growth and Nutritional Status in Pediatric Lipoid Proteinosis: A Prospective Cross-Sectional Study
By Teker-Duztas D and An İ.
Abstract
Background/objectives
Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis characterized by oral and lingual involvement, frequently accompanied by dysphagia and recurrent oral ulcers. The relationships between these clinical manifestations and nutritional status or growth have not been clearly established. This study evaluated nutritional, growth, and micronutrient statuses in pediatric patients with LP and investigated their associations with dysphagia, oral ulcer frequency, and a composite oral involvement score.
Methods
This prospective, cross-sectional, single-center study enrolled 30 pediatric patients with LP. Anthropometric measurements, laboratory results, and clinical data regarding oral involvement and dysphagia were collected. A study-specific composite oral involvement score (range: 0-4) was calculated based on restricted mouth opening, restricted tongue movement, presence of a thick or woody tongue, and active oral ulcers.