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 pati
ent died 2 Srs after sustaining the fracture, Bone specimens obtained at po
st mortem demonstrated severe cortical and trabecular osteopenia, but the h
istological 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 di
sease of cystic fibrosis may be more complex and possibly unique. Labelled
bone biopsies are required to clarify the bone defect leading to low bone d
ensity in cystic fibrosis patients so that appropriate therapeutic strategi
es can be developed.