title: Biochemical markers of bone turnover in Camurati-Engelmann disease: a report on four cases in one family.
abstract: Moderate increases in "classical" biochemical markers of bone turnover have been described only in some patients with Camurati-Engelmann disease. However, the determination of the following "new" markers has not been previously performed: serum osteocalcin (BGP), bone alkaline phosphatase (BAP), carboxyterminal propeptide of type I procollagen (PICP), aminoterminal propeptide of type I procollagen (PINP), tartrate-resistant acid phosphatase (TRAP), telopeptide carboxyterminal of type I collagen (ICTP), urinary pyridinoline (PYR), crosslinked N-telopeptides of type I collagen (NTX), and Crosslaps (CL). Such a determination may improve the evaluation of the disease activity. To evaluate the usefulness of biochemical markers of bone turnover reflecting Camurati-Engelmann disease activity we measured the levels of all these markers in four affected patients. The results were compared with bone scintigraphic indices of disease activity. Except for PICP and TRAP, bone formation and resorption markers were abnormal in all patients and were related to bone scan indices of disease activity. Among the markers of bone formation PINP, BAP, and BGP showed the highest values, whereas NTX and CL were the most sensitive markers of bone resorption. These results suggest that the determination of NTX or CL, and PINP or either BAP and BGP, associated with bone scan evaluation, provides the best assessment of Camurati-Engelmann disease activity.
authors.affiliation: Department of Rheumatology, Metabolic Bone Disease Unit, Hospital Clínic i Provincial, Villarroel 170, 08036 Barcelona, Spain.
authors.forename: M V
authors.initials: MV
authors.lastname: Hernández
authors.forename: P
authors.initials: P
authors.lastname: Peris
authors.forename: N
authors.initials: N
authors.lastname: Guañabens
authors.forename: L
authors.initials: L
authors.lastname: Alvarez
authors.forename: A
authors.initials: A
authors.lastname: Monegal
authors.forename: F
authors.initials: F
authors.lastname: Pons
authors.forename: A
authors.initials: A
authors.lastname: Ponce
authors.forename: J
authors.initials: J
authors.lastname: Muñoz-Gómez
mesh_terms: D000135:Acid Phosphatase / Q000378:metabolism; D000328:Adult; D000469:Alkaline Phosphatase / Q000378:metabolism; D000596:Amino Acids / Q000652:urine; D015415:Biomarkers / Q000032:analysis* / Q000097:blood / Q000652:urine; D001846:Bone Development / Q000502:physiology; D001862:Bone Resorption / Q000503:physiopathology; D003966:Camurati-Engelmann Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology*; D002851:Chromatography, High Pressure Liquid; D003094:Collagen / Q000378:metabolism; D024042:Collagen Type I; D005260:Female; D006801:Humans; D007527:Isoenzymes / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D015675:Osteocalcin / Q000097:blood; D010446:Peptide Fragments / Q000097:blood / Q000378:metabolism; D010455:Peptides / Q000378:metabolism; D011347:Procollagen / Q000097:blood / Q000378:metabolism; D000071681:Tartrate-Resistant Acid Phosphatase
chemical_list: D000596:Amino Acids; D015415:Biomarkers; D024042:Collagen Type I; D007527:Isoenzymes; D010446:Peptide Fragments; D010455:Peptides; D011347:Procollagen; C052929:collagen type I trimeric cross-linked peptide; C041876:procollagen Type III-N-terminal peptide; C042813:procollagen type I carboxy terminal peptide; D015675:Osteocalcin; C015484:pyridinoline; D003094:Collagen; D000469:Alkaline Phosphatase; D000135:Acid Phosphatase; D000071681:Tartrate-Resistant Acid Phosphatase