High incidence of significant bone loss in patients with severe congenital neutropenia (Kostmann's syndrome).

摘要: Abstract Objective: Clinical observation of bone pain, unusual fractures in two patients, and diffuse osteopenia/osteoporosis led us to assess mineral content density 30 patients with severe congenital neutropenia who were treated recombinant-methionyl-human granulocyte colony-stimulating factor (r-metHuG-CSF). Study design: We reviewed roentgenograms 29 these evaluate loss before during treatment. In addition, 17 the status could be assessed by both quantitative computed tomography (Q-CT; n = 16) dual energy x-ray absorptiometry (DXA; 1). one patient, Q-CT was not possible because vertebral fractures. Results: Of investigated, 15 had evidence observed on spine radiographs ( 5), Q-CT/DXA 1/ 1), or 8). 13 only a lateral radiograph lumbar available, 5 showing either increased kyphosis wedging vertebrae compression bodies, indicating established osteoporosis. eight findings spinal normal. nine taken r-metHuG-CSF Osteoporotic deformation 3) reduced mass seen six patients. The levels serum biochemical markers metabolism all within normal ranges except for mild elevation alkaline phosphatase level. degree did correlate dose duration treatment age sex Conclusions: These data indicate high incidence children neutropenia. underlying pathogenesis demineralization is clear. It more likely that caused pathophysiologic features disease, but it accelerates loss. (J Pediatr 1997;131:592-7)