title: Sniff nasal inspiratory pressure in the longitudinal assessment of young Duchenne muscular dystrophy children.
abstract: Traditional measures of respiratory function in children with Duchenne muscular dystrophy (DMD) are based on maximal inspiratory pressure (PImax) and vital capacity (VC). Sniff nasal inspiratory pressure (SNIP) measurements are easily performed by young children with neuromuscular disorders. The clinical value of SNIP in the longitudinal assessment of respiratory weakness remains to be assessed. The objective of the present study was to assess longitudinally the changes in SNIP, PImax and VC with age in DMD children. We hypothesised that their longitudinal assessment would show an earlier decline in SNIP than VC. A 3-year, prospective follow-up, at 6-month intervals of, 33 steroid-naïve, 5-20-year-old DMD patients was analysed using a linear mixed model. SNIP measurements were reliable (within-session coefficient of variation 8%). SNIP and VC increased until 10.5 and 12.5 years of age, respectively, and declined thereafter, while PImax did not change with age. SNIP was an earlier marker of decline in respiratory muscle strength (at 10.5 years) than VC (at 12.5 years) in young DMD patients. SNIP longitudinal assessment is useful in the detection of inspiratory strength decline in young DMD patients when VC values remain within normal values and as an outcome measure in clinical trials for emerging therapeutics in young DMD patients from the age of 5 years.
authors.affiliation: CHRU de Lille, Lille.
authors.forename: Véronique
authors.initials: V
authors.lastname: Nève
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Cuisset
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Edmé
authors.forename: Alain
authors.initials: A
authors.lastname: Carpentier
authors.forename: Mike
authors.initials: M
authors.lastname: Howsam
authors.forename: Olivier
authors.initials: O
authors.lastname: Leclerc
authors.forename: Régis
authors.initials: R
authors.lastname: Matran
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D001239:Inhalation; D016014:Linear Models; D018908:Muscle Weakness / Q000175:diagnosis* / Q000209:etiology; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000175:diagnosis*; D011312:Pressure; D011446:Prospective Studies; D012129:Respiratory Function Tests / Q000379:methods; D012132:Respiratory Muscles / Q000503:physiopathology*; D055815:Young Adult