Histomorphometric study of bone remodeling in hypophosphatemic vitamin D-resistant rickets.
Static and dynamic histomorphometric parameters were evaluated on undecalcified iliac crest bone biopsies obtained from eight children with untreated hypophosphatemic vitamin D resistant rickets (VDRR) in an attempt to evaluate whether a primary metabolic bone defect contributes to the skeletal disorder observed in that disease. When compared to normal age-matched controls the trabecular calcified bone volume was not decreased and there was no evidence of excessive osteoclastic resorption. Both trabecular and cortical bone envelopes had an excess of osteoid tissue and a decreased extent of the mineralization front. Dual tetracycline labeling revealed a decrease in the osteoblastic calcification rate and a marked prolongation of the mineralization lag time and of the formation period. In the intracortical Haversian system the birthrate of new Basic Multicellular remodeling Units (BMU) was markedly reduced, leading to a marked depression of the bone formation rate at the whole tissue level. The combination of the decreased birthrate of new BMU and the prolonged formation period appears to be characteristic of the disease. These results indicate that abnormal differentiation and function of the osteoblast contribute to the osteomalacic lesion present in VDRR. Defective mineralization and impaired osteoblastic function might be the consequence of the chronic hypophosphatemic state. However, the existence of a primary disorder of the bone cell line cannot be excluded as an explanation of the defective recruitment and function of the bone forming cells.
D000293:Adolescent; D001842:Bone and Bones / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007015:Hypophosphatemia, Familial / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D010006:Osteoblasts / Q000166:cytology; D010010:Osteoclasts / Q000166:cytology; D010012:Osteogenesis