Body mass index in type 2 spinal muscular atrophy: a longitudinal study

Gloria Ferrantini, Giorgia Coratti, Roberta Onesimo, Simona Lucibello, Sarah Bompard, Ida Turrini, Graziamaria Cicala, Michela Caprarelli, Maria Carmela Pera, Chiara Bravetti, Beatrice Berti, Valentina Giorgio, Claudio Bruno, Noemi Brolatti, Chiara Panicucci, Adele D’Amico, Antonella Longo, Chiara Leoni, Valeria A. Sansone, Emilio AlbamonteSonia Messina, Maria Sframeli, Enrico Bertini, Enrico Silvio Bertini, Marika Pane, Eugenio Maria Mercuri

Risultato della ricerca: Contributo in rivistaArticolo in rivista


The aim of this retrospective study was to review body mass index (BMI) in a large cohort of Italian pediatric type 2 spinal muscular atrophy (SMA) patients, aged between 0 and 20 years and to establish possible differences in relation to a number of variables such as ventilation, motor function, and survival motor neuron 2 gene copies. Cross-sectional data were collected from 102 patients for a total of 344 visits. Standard growth charts for height and weight were used as reference, with age adjusted BMI calculated using the Center for Disease and Prevention Children's BMI Tool. In the 344 visits, weight ranged between 3.90 and 83 kg, and the BMI between 8.4 and 31.6 with a BMI/age z-scores < - 2SD present in 28% and BMI/age z-scores > + 2SD in 9% of the measurements. The BMI/age z-scores were relatively stable < 5 years of age with an increasing number of patients < - 2SD after the age of 5, and a wider range of BMI/age z-scores after the age of 13. A difference on the BMI/age z-scores was found among the different age subgroups (< 5, 5-12, >= 13 years). A multivariate analysis in 58 patients with longitudinal assessments showed that baseline BMI/age z-scores and gender were significantly contributing to the changes while other variables were not.Conclusion: Our results confirm that careful surveillance of weight and BMI/age z-scores is needed in type 2 SMA. Further studies, including assessments of chewing and swallowing and of lean/fat body mass, will help to better understand the possible mechanisms underlying weight issues.
Lingua originaleEnglish
pagine (da-a)1923-1932
Numero di pagine10
RivistaEuropean Journal of Pediatrics
Stato di pubblicazionePubblicato - 2022


  • Adolescent
  • Adult
  • Body Mass Index
  • Body Weight
  • Body mass index
  • Child
  • Child, Preschool
  • Children
  • Cross-Sectional Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Longitudinal Studies
  • Muscular Atrophy, Spinal
  • Neonate
  • Nutritional status
  • Retrospective Studies
  • Spinal muscular atrophy
  • Young Adult


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