Anthropometric measurement standardization for a multicenter nutrition survey in children with spinal muscular atrophy

Simona Bertoli,Alessandro Leone,Giovanni Baranello,Andrea Foppiani,Ester Giaquinto,Ramona De Amicis,Chiara Mastella,Michela Bassano,Alberto Battezzati

doi:10.1038/s41430-019-0392-2

Abstract

Spinal muscular atrophy (SMA) is a neuromuscular disease associated with nutritional status derangement and altered body composition. New drugs are changing the natural history of the disease, so now more than ever is important to focus on the correct assessment of nutritional status in SMA. We implemented a standardization process for the anthropometric measurement as part of our ongoing longitudinal study of growth patterns in SMA patients. It features a procedural manual, included in this communication, observers training and reliability assessment, of which we publish the values obtained in our pilot study. The standardization process was able to produce inter-observer reliability values in agreement with the literature and a procedure manual is now available for multicentre studies of nutritional status and body composition in SMA and possibly other pediatric neuromuscular disorders.

Highlights

Spinal muscular atrophy (SMA) is a rare and severe neuromuscular disease associated with feeding disorders [1], changes in energy metabolism and impairment of nutritional status [2]
The recent advent of new drugs and clinical trials for SMA are changing the natural history of the disease [1], so more than ever is important to focus on the correct assessment of nutritional status in SMA patients
There were no significant differences in %technical error of measurement (TEM) of the measurement performed, indicating that there was no difference in measurement reliability between healthy and SMA children

Summary

Introduction

Spinal muscular atrophy (SMA) is a rare and severe neuromuscular disease associated with feeding disorders [1], changes in energy metabolism and impairment of nutritional status [2]. The recent advent of new drugs and clinical trials for SMA are changing the natural history of the disease [1], so more than ever is important to focus on the correct assessment of nutritional status in SMA patients. These authors contributed : Simona Bertoli, Andrea Foppiani

Methods

Results

Conclusion