Saúde óssea em meninos com distrofia muscular de Duchenne em terapia delongo prazo diária de deflazacort
Canadá – esta pesquisa foi realizada pelo grupo que mais experiência tem com o uso de corticóides em distrofia muscular. Trinta e nove meninos com Duchenne foram tratados com deflazacort diariamente por longo prazo. A densitometria óssea foi obtida no início e ao longo do tratamento. Nove fraturas em ossos longos foram observadas em 8 pacientes, duas delas antes do tratamento. Sete fraturas de ossos vertebrais foram observadas em 6 pacientes após 5 anos ou mais do uso dos corticóides. Os valores de densitometria óssea foram corrigidos para a altura e porcentagem de massa gorda e permaneceram estáveis por longo tempo do tratamento. A saúde óssea é influenciada pela doença, pelos corticóides, pela deambulação e pela massa gorda e estes parâmetros devem ser considerados na análise dos resultados.
O resumo em inglês pode ser lido abaixo:
(Neuromuscular Disorders, 2012) Bone health in boys with Duchenne Muscular Dystrophy on long-term daily deflazacort therapy
A.L. Mayo, B.C. Craven, L.C. McAdamc,, W.D. Biggar – Canada
Quality of life in Duchenne Muscular Dystrophy (DMD) has improved significantly with corticosteroid treatment. However, corticosteroids decrease bone mass and increase vertebral fragility fracture risk. We report on bone health in 39 boys with DMD on
long-term deflazacort (0.9 mg/kg/day) therapy. Bone health was defined by lumbar (L1–L4) bone mineral density (BMD), long-bone
and/or symptomatic vertebral fractures. Lumbar BMD was reported as height-adjusted Z-scores at initiation of deflazacort (T0) and
1–2 year intervals thereafter. Subcapital body fat percentage and ambulatory status were recorded. At T0, 39 boys, aged 6.6 ± 1.6 years had height-adjusted BMD Z-score 0.5 ± 0.8, and 23.5 ± 5.0% body fat. Height-adjusted Z-scores remained stable with years of deflazacort until loss of ambulation and accrual of body fat. Nine long-bone fractures occurred in eight ambulating boys, two before T0. Seven vertebral fractures occurred in six non-ambulatory boys after >5 years of deflazacort with height-adjusted Z-score
1.8 ± 0.7, and 47.8 ± 12% body fat. Bone health in DMD is influenced by disease progression, corticosteroids, BMD Z-scores and
fat mass accumulation. Adjustments for short stature must be considered during BMD interpretation. Percent body fat and ambulatory status are useful bone health indicators. Routine use of height adjusted Z-scores is advocated for use in routine clinical practice.
