Study objective: Low bone mineral density is a common complication of cysti
c fibrosis (CF), and recent studies have implicated vitamin D insufficiency
as a significant etiologic factor. The aim of this study was to establish
whether there was bone biopsy evidence of vitamin D deficiency osteomalacia
in patients with CF and to document the general histomorphometric characte
ristics of CF bone.
Patients and methods: A retrospective descriptive and histomorphometric stu
dy of postmortem L2/L3 vertebral bone biopsy specimens was undertaken on ti
ssue from 11 posttransplant CF patients and 4 nontransplanted CF patients.
Control data were derived from postmortem bone specimens from 15 young adul
ts.
Results: Bone from all CF patients was characterized by severe osteopenia i
n both trabecular and cortical bone. At the cellular level, there was decre
ased osteoblastic and increased osteoclastic activity, The reduction in ost
eoblastic activity was due to both a decrease in osteoblast number and a de
crease in the biosynthetic potential of osteoblasts, The osteoclastic chang
es were due to an increase in the number of osteoclasts, The increase in os
teoclasis and the uncoupling of osteoblastic and osteoclastic activity resu
lted in an increase in resorptive surfaces. Although there were few signifi
cant differences between the transplanted and nontransplanted CF groups, bo
th cortical and trabecular bone mass tended to be lower after transplantati
on. None of the CF undecalcified biopsy specimens showed osteoid parameters
characteristic of vitamin D deficiency osteomalacia,
Conclusions: CF patients have an unusual and complex pattern of cellular ch
anges within bone that are not typical of vitamin D deficiency osteomalacia
.