caa a22 4500 46580456X CHVBK 20180323112100.0 cr unu---uuuuu 170327e19900301xx s 000 0 eng 10.1007/BF03349553 doi (NATIONALLICENCE)springer-10.1007/BF03349553 High turnover osteoporosis in acro-osteolysis (Hajdu-Cheney syndrome) [Elektronische Daten] [V. Nunziata, G. di Giovanni, P. Ballanti, E. Bonucci] Acro-osteolysis with diffuse osteoporosis in the absence of other associated diseases is named Hajdu-Cheney syndrome. Reduced bone formation rather than enhanced bone resorption has been indicated as the mechanism of osteoporosis. On the assumption that in this syndrome the active bone resorption which produces distal osteolysis must also predominate in generalized osteoporosis, we investigated boae histology, calcium kinetics, calciotropic hormones and bone markers in a patient suffering from sporadic Hajdu-Cheney syndrome. A radius bone biopsy taken far from the osteolytic lesions showed severe osteoporosis with a marked increase in osteoclastic bone resorption and reduced bone formation. Total body calcium clearance, performed through an analysis of the kinetics of calcium infusion, was 2.8 times higher than in normal controls, indicating the presence of active osteoclastic bone resorption. Serum parathormone, 1, 25-dihydroxycholecalciferol, alkaline phosphatase and urinary hydroxiproline were in the normal range. These data indicate that in Hajdu-Cheney syndrome trabecular osteoporosis is produced by the same mechanism that induces distal osteolysis, which suggests that it may be sustained by local acting factors stimulating osteoclastic resorption. Italian Society of Endocrinology (SIE), 1990 Osteoporosis nationallicence acro-osteolysis nationallicence bone turnover nationallicence bone resorption nationallicence bone formation nationallicence Nunziata V. Istituto di Medicina Interna e Malattie Metaboliche, Università di Napoli, tÏa S. Pansini 5, 80131, Napoli, Italy aut di Giovanni G. Istituto di Medicina Interna e Malattie Metaboliche, Università di Napoli, tÏa S. Pansini 5, 80131, Napoli, Italy aut Ballanti P. Dipartimento di Biopatologia Umana, Università "La Sapienza”, Roma, Italy aut Bonucci E. Dipartimento di Biopatologia Umana, Università "La Sapienza”, Roma, Italy aut Journal of Endocrinological Investigation Springer International Publishing 13/3(1990-03-01), 251-255 0391-4097 13:3<251 1990 13 40618 https://doi.org/10.1007/BF03349553 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF03349553 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Nunziata V. Istituto di Medicina Interna e Malattie Metaboliche, Università di Napoli, tÏa S. Pansini 5, 80131, Napoli, Italy aut NATIONALLICENCE 700 1- di Giovanni G. Istituto di Medicina Interna e Malattie Metaboliche, Università di Napoli, tÏa S. Pansini 5, 80131, Napoli, Italy aut NATIONALLICENCE 700 1- Ballanti P. Dipartimento di Biopatologia Umana, Università "La Sapienza”, Roma, Italy aut NATIONALLICENCE 700 1- Bonucci E. Dipartimento di Biopatologia Umana, Università "La Sapienza”, Roma, Italy aut NATIONALLICENCE 773 0- Journal of Endocrinological Investigation Springer International Publishing 13/3(1990-03-01), 251-255 0391-4097 13:3<251 1990 13 40618 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer