RT Journal Article
SR Electronic
T1 Hip fracture and bone histomorphometry in a young adult with cystic fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 478
OP 479
DO 10.1183/09031936.99.14247899
VO 14
IS 2
A1 CS Haworth
A1 AJ Freemont
A1 AK Webb
A1 ME Dodd
A1 PL Selby
A1 EB Mawer
A1 JE Adams
YR 1999
UL http://erj.ersjournals.com/content/14/2/478.abstract
AB A 25-yr-old male with cystic fibrosis sustained a fragility fracture of the left femoral neck, which required surgical correction. He had several risk factors for the development of low bone density and despite treatment with an oral bisphosphonate, his bone mineral density reduced further. The patient died 2 yrs after sustaining the fracture. Bone specimens obtained at post mortem demonstrated severe cortical and trabecular osteopenia, but the histological features were not typical of osteoporosis or osteomalacia. Osteoporosis is thought to be a common complication of cystic fibrosis. The novel histomorphometric appearances reported here suggest that the bone disease of cystic fibrosis may be more complex and possibly unique. Labelled bone biopsies are required to clarify the bone defect leading to low bone density in cystic fibrosis patients so that appropriate therapeutic strategies can be developed.