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Juvenile Osteoporosis

Juvenile osteoporosis
Objectives
  1. Describe the clinical features of (idiopathic) juvenile osteoporosis
  2. Discuss the natural history of juvenile osteoporosis

Discussion point
  1. What is the role of spine bracing for children with juvenile osteoporosis?

Discussion
Idiopathic juvenile osteoporosis is an ill-understood condition that most often has and onset between ages 8 and 14. Presenting symptoms are long bone fractures, back pain, and difficulty walking. Typically, radiographs demonstrate compression of the vertebra and the metaphyses of long bones. In a recent study, the defect appeared to be secondary to osteoblast dysfunction, children with juvenile osteoporosis have a markedly decreased bone volume and low bone turnover. A previous study had concluded to the contrary. The outlook for spontaneous recovery is good, usually in 2-4 years or at skeletal maturity, but protracted disability has been reported in a small number of adults. Bracing of spinal deformity, a reasonable concept, has been recommended, but the experience is small.

References
  1. Bertelloni S, Baroncelli GI, Di Nero G, Saggese G. Idiopathic juvenile osteoporosis: evidence of normal osteoblast function by 1,25-dihydroxyvitamin D3 stimulation test [published erratum appears in Calcif Tissue Int 1992 Nov; 51( 5): 400]. Calcified Tissue International 1992; 51( 1): 20-3.
  2. Jones ET, Hensinger RN. Spinal deformity in idiopathic juvenile osteoporosis. Spine 1981; 6( 1): 1-4.
  3. Rauch F, Travers R, Norman ME, Taylor A, Parfitt AM, Glorieux FH. Deficient bone formation in idiopathic juvenile osteoporosis: a histomorphometric study of cancellous iliac bone. Journal of Bone & Mineral Research 2000; 15( 5): 957-63.
  4. Smith R. Idiopathic juvenile osteoporosis: experience of twenty-one patients. British Journal of Rheumatology 1995; 34( 1): 68-77.
  5. Zaleske DJ. Metabolic and endocrine abnormalities. In: Morrissy RT, Weinstein SL, editors. Pediatric Orthopaedics. Philadelphia: Lippincott-Raven; 1996. p. 137-201.
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