Pressão inspiratória nasal para acompanhamento longitudinal de crianças jovens com distrofia muscular de Duchenne

França – As medidas tradicionais de função respiratória em crianças com distrofia muscular de Duchenne ( DMD) são baseadas em pressão inspiratória máxima ( PImax ) e capacidade vital (CV ) . Nesta pesquisa a medida da pressão inspiratória nasal, mais fácil de obter em crianças com distrofia muscular de Duchenne, foi utilizada no acompanhamento de longo prazo das crianças em comparação a prova de função pulmonar. tradicional. A pressão inspiratória nasal conseguiu detectar alterações mais precocemente, permitindo o acompanhamento dos pacientes mesmo quando a capacidade vital estava em valores normais.

O resumo em inglês pode ser lido abaixo:

(Eur. Respir. J., Sep 2013; 42: 671 – 680) Sniff nasal inspiratory pressure in the longitudinal assessment of young Duchenne muscular dystrophy children

Véronique Nève, Jean-Marie Cuisset, Jean-Louis Edmé, Alain Carpentier, Mike Howsam, Olivier Leclerc, and Régis Matran

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