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* From the Manchester Adult Cystic Fibrosis Unit (Drs. Haworth and Egan, Mr. Webb), and the Department of Pathology (Dr. Bishop and Mr. Hasleton), South Manchester University Hospitals NHS Trust, Wythenshawe Hospital, Manchester; the Department of Medicine (Dr. Selby), University of Manchester, Manchester Royal Infirmary, Manchester; and Osteoarticular Pathology (Mr. Freemont), Musculoskeletal Research Group, University of Manchester, Manchester, UK.
Correspondence to: A. Kevin Webb, FRCP, Manchester Adult Cystic Fibrosis Unit, South Manchester University Hospitals NHS Trust, Wythenshawe Hospital, Southmoor Road, Manchester, M23 9LT, UK
Study objective: Low bone mineral density is a common complication of cystic 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 characteristics of CF bone.
Patients and methods: A retrospective descriptive and histomorphometric study of postmortem L2/L3 vertebral bone biopsy specimens was undertaken on tissue from 11 posttransplant CF patients and 4 nontransplanted CF patients. Control data were derived from postmortem bone specimens from 15 young adults.
Results: Bone from all CF patients was characterized by severe osteopenia in both trabecular and cortical bone. At the cellular level, there was decreased osteoblastic and increased osteoclastic activity. The reduction in osteoblastic activity was due to both a decrease in osteoblast number and a decrease in the biosynthetic potential of osteoblasts. The osteoclastic changes were due to an increase in the number of osteoclasts. The increase in osteoclasis and the uncoupling of osteoblastic and osteoclastic activity resulted in an increase in resorptive surfaces. Although there were few significant differences between the transplanted and nontransplanted CF groups, both cortical and trabecular bone mass tended to be lower after transplantation. 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 changes within bone that are not typical of vitamin D deficiency osteomalacia.
Key Words: bone histology • bone histomorphometry • cystic fibrosis • osteomalacia • osteoporosis • vitamin D
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