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Título

CORRELATION BETWEEN AGE, RESPIRATORY AND MOTOR FUNCTION IN CHILDREN WITH LAMA2 CONGENITAL MUSCULAR DYSTROPHY

Resumo

Introduction: LAMA2 Natural history studies have been increasing in the last years due to the new era of emerging gene therapy. In this type of congenital muscular dystrophy, the age is associated with poor respiratory and motor function. A better understanding of how age relates to the course of respiratory and motor aspects can contribute to multidisciplinar management and treatment for this children. This study aimed to investigate the relationship between age, respiratory and motor function in LAMA2 children.
Methods: Eighteen children (11.4±5.8 years; 11 male\7 female; 7 with nocturnal noninvasive ventilation) had the forced vital capacity percentage (FVC) and cough peak flow (CPF) measured by spirometer and peak flow meter, respectively, and the motor function evaluated by three domains and total score of Motor Function Measure (MFM) - 32. The Shapiro‐Wilk test demonstrated that the data were non-normally distributed. The relationships between respiratory and motor functions and also between age and respiratory and motor functions were tested by Spearman correlation test. The correlation coefficients were classified as strong (r≥0.70), moderate (0.40 ≤ r < 0.70), or weak (r ≤0.40).
Results: FVC strongly correlated to MFM-D2 (axial and proximal function) and moderately correlated to MFM-D1 (standing and transfers function) and to MFM-total. No correlation was found between CPF and motor function items, but CPF was moderately correlated to age. Age strongly correlated to MFM-D2 and moderately correlated to MFM-D1 and to MFM-total.
Conclusions: A good pulmonary function (FVC) was positively correlated to high motor function to perform axial\proximal, standing and transfers activities. Interestingly, CFP was not correlated to motor function, but was negatively correlated to age. Addictionaly, age was negatively correlated to axial\proximal and total motor function in LAMA2 children