Avaliação evolutiva dos parâmetros ventilatórios e da pressão inspiratória máxima em pacientes com distrofia muscular de Duchenne

França - a função respiratória é comprometida com a evolução da distrofia muscular de Duchenne, especialmente a partir da adolescência; nesta pesquisa; durante 7 anos dez meninos com Duchenne foram acompanhados dos 9,1+ 1 ano até os 16 + 1,4 anos. Os resultados demonstraram que a pressão inspiratória máxima apresentou redução desde o início do acompanhamento sendo que os parâmetros ventilatórios foram os melhores para acompanhamento nas fases mais avançadas da doença.

O resumo em inglês pode ser lido abaixo:

(Pediatr Pulmonol. 2010; 45:552-559) Ventilatory parameters and maximal respiratory pressure changes with age in Duchenne muscular dystrophy patients

Jerome Gayraud, Michele Ramonatxo, François Rivier, MD, Véronique Humberclaude, Basil Petrof, Stefan Matecki

The aim of this longitudinal study was to precise, in children with Duchenne muscular dystrophy, the respective functional interest of ventilatory parameters (Vital capacity, total lung capacity and forced expiratory volume in one second [FEV1]) in comparison to maximal inspiratory pressure (Pimax) during growth. In ten boys the mean age of 9.1 ± 1 years) to mean age of 16 ± 1.4 years followed over a period of 7 years, we found that: (1) ventilatory parameters expressed in percentage of predicted value, after a normal ascending phase, start to decrease between 11 and 12 years, (2) Pimax presented only a decreasing phase since the beginning of the study and thus was already at 67% of predicted value at 12 years while ventilatory parameters was still normal, (3) after 12 years the mean slopes of decrease per year of vital capacity and FEV1 were higher (10.7 and 10.4%) than that of Pimax (6.9%), (4) at 15 years mean values of vital capacity and FEV1 (53.3 and 49.5% of predicted values) was simlar to that of Pimax (48.3%). In conclusion, if at early stages of the disease, Pimax is a more reliable index of respiratory impaiment than ventilatory parameters, the follow-up of ventilatory parameters, when they start to decrease, is a better indicator of disease progression and, at advanced stages they provided same information about the functional impact of disease.

Fonte: http://distrofiamuscular.net/noticias.htm