title: Feeding problems and malnutrition in spinal muscular atrophy type II.
abstract: The aim of the study was to conduct a survey using a dedicated questionnaire to assess feeding difficulties and weight gain in a population of 122 Spinal Muscular Atrophy (SMA) type II patients, aged between 1 and 47 years. All the answers were entered in a database and were analysed subdividing the cohort into age groups (1-5, 6-10, 11-14, 15-19, 20-29, and 30-50 years). Six out of our 122 patients (5%), all younger than 11 years, had weights more than 2SD above the median for age matched controls, whilst 45 (37%) had weights less than 2SD below the median. Chewing difficulties were reported in 34 of the 122 patients (28%) and limitation in the ability to open the mouth in 36 (30%) and both were increasingly more frequent with age. Swallowing difficulties were reported in 30 patients (25%). The results of our survey suggest that a number of patients with SMA type II have limited jaw opening, and chewing and swallowing difficulties. Our findings raise a few issues concerning standards of care that should be implemented in the monitoring and management of feeding difficulties and weight gain.
authors.affiliation: Department of Child Neurology, Policlinico Gemelli, Largo Gemelli, Rome, Italy.
authors.forename: Sonia
authors.initials: S
authors.lastname: Messina
authors.forename: Marika
authors.initials: M
authors.lastname: Pane
authors.forename: Paola
authors.initials: P
authors.lastname: De Rose
authors.forename: Isabella
authors.initials: I
authors.lastname: Vasta
authors.forename: Domenica
authors.initials: D
authors.lastname: Sorleti
authors.forename: Annie
authors.initials: A
authors.lastname: Aloysius
authors.forename: Federico
authors.initials: F
authors.lastname: Sciarra
authors.forename: Fortunato
authors.initials: F
authors.lastname: Mangiola
authors.forename: Maria
authors.initials: M
authors.lastname: Kinali
authors.forename: Enrico
authors.initials: E
authors.lastname: Bertini
authors.forename: Eugenio
authors.initials: E
authors.lastname: Mercuri
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003680:Deglutition Disorders / Q000209:etiology*; D001068:Feeding and Eating Disorders / Q000145:classification / Q000209:etiology*; D006801:Humans; D007223:Infant; D044342:Malnutrition / Q000209:etiology* / Q000628:therapy; D008875:Middle Aged; D009752:Nutritional Status; D018529:Nutritional Support; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications / Q000503:physiopathology* / Q000628:therapy; D011795:Surveys and Questionnaires; D015430:Weight Gain