Bone well being measures in glucocorticoid-treated ambulatory boys with Duchenne muscular dystrophy.
Neuromuscul Disord. 2016 Nov;26(11):760-767
Authors: Tian C, Wong BL, Hornung L, Khoury JC, Miller L, Bange J, Rybalsky I, Rutter MM
Osteoporosis is a serious drawback in boys with Duchenne Muscular Dystrophy (DMD), attributable to muscle weak spot and glucocorticoid remedy. Consensus relating to bone well being evaluation and administration is missing. Lumbar backbone areal bone mineral density (outlined as bone mass per space of bone) by dual-energy X-ray absorptiometry (DXA) is continuously the first measure used, however has limitations for boys with DMD. We retrospectively studied 292 ambulant glucocorticoid-treated boys with DMD categorized by useful mobility rating, FMS 1, 2 or three. We assessed DXA complete physique and lumbar backbone areal bone mineral density and content material Z-scores adjusted for age and top, lateral distal femur areal bone mineral density Z-scores, frequency of fractures, and osteoporosis by Worldwide Society for Scientific Densitometry 2013 standards.
Entire physique and femoral DXA indices decreased, whereas backbone fractures elevated, with declining motor operate. Lumbar backbone areal bone mineral density Z-scores appeared to enhance with declining motor operate. Bone mineral content material Z-scores have been persistently decrease than corresponding bone mineral density Z-scores. Our findings spotlight the complexity of assessing bone well being in boys with DMD. Bone well being indices worsened with declining motor operate in ambulant boys, however interpretation was affected by measure and skeletal web site examined. Entire physique bone mineral content material could also be a useful measure in boys with DMD. Lumbar backbone areal bone mineral density Z-score as an remoted measure could possibly be deceptive. Complete administration of osteoporosis in boys with DMD ought to embody vertebral fracture evaluation.