BIOCHEMICAL MARKERS OF BONE TURNOVER IN CAMURATI-ENGELMANN-DISEASE - A REPORT ON 4 CASES IN ONE FAMILY

Moderate increases in ''classical'' biochemical markers of bone turnov er have been described only in some patients with Camurati-Engelmann d isease. However, the determination of the following ''new'' markers ha s not been previously performed: serum osteocalcin (BGP), bone alkalin e phosphatase (BAP), carboxyterminal propeptide of type I procollagen (PICP), aminoterminal propeptide of type I procollagen (PINP), tartrat e-resistant acid phosphatase (TRAP), telopeptide carboxyterminal of ty pe I collagen (ICTP), urinary pyridinoline (PYR), crosslinked N-telope ptides of type I collagen (NTX), and Crosslaps (CL). Such a determinat ion may improve the evaluation of the disease activity. To evaluate th e usefulness of biochemical markers of bone turnover reflecting Camura ti-Engelmann disease activity we measured the levels of all these mark ers in four affected patients. The results were compared with bone sci ntigraphic indices of disease activity. Except for PICP and TRAP, bone formation and resorption markers were abnormal in all patients and we re related to bone scan indices of disease activity. Among the markers of bone formation PINP, BAP, and BGP showed the highest values, where as NTX and CL were the most sensitive markers of bone resorption. Thes e results suggest that the determination of NTX or CL, and PINP or eit her BAP and BGP, associated with bone scan evaluation, provides the be st assessment of Camurati-Engelmann disease activity.