TY - JOUR
T1 - Long-term follow-up of bone mineral density in childhood hypophosphatasia
AU - Girschick, Hermann Josef
AU - Haubitz, Imme
AU - Hiort, Olaf
AU - Schneider, Peter
N1 - Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2007/5
Y1 - 2007/5
N2 - Objective: Hypophosphatasia (HP; MIM 241510) is an inborn error of bone metabolism, characterized by a genetic defect in the gene of the tissue-non-specific alkaline phosphatase TNSALP. Long-term data on bone mineral density measurements are not available. Methods: We have analyzed changes of bone mineral density (pQCT and DXA) prospectively during 4 years of follow-up in a cohort of 6 patients with childhood HP. Results: At diagnosis hypermineralization of the trabecular bone in the metaphyseal area of long bones in affected children was noted. During 4 years of follow-up a gradual, significant decrease of mineralization was noted in the radial metaphyses. In contrast, BMC by DXA and total body DXA values were stable in comparison to healthy controls. During follow-up a systemic hyperprostaglandinism was documented in the majority of the patients. Non-steroidal anti-inflammatory drug treatment was evaluated by measuring prostaglandin excretion in the urine. Conclusions: Metaphyseal hypermineralization in childhood HP, which might be a compensation for a mechanically incompetent bony structure, decreased over time. There might be a pathophysiological link to continually elevated systemic prostaglandins.
AB - Objective: Hypophosphatasia (HP; MIM 241510) is an inborn error of bone metabolism, characterized by a genetic defect in the gene of the tissue-non-specific alkaline phosphatase TNSALP. Long-term data on bone mineral density measurements are not available. Methods: We have analyzed changes of bone mineral density (pQCT and DXA) prospectively during 4 years of follow-up in a cohort of 6 patients with childhood HP. Results: At diagnosis hypermineralization of the trabecular bone in the metaphyseal area of long bones in affected children was noted. During 4 years of follow-up a gradual, significant decrease of mineralization was noted in the radial metaphyses. In contrast, BMC by DXA and total body DXA values were stable in comparison to healthy controls. During follow-up a systemic hyperprostaglandinism was documented in the majority of the patients. Non-steroidal anti-inflammatory drug treatment was evaluated by measuring prostaglandin excretion in the urine. Conclusions: Metaphyseal hypermineralization in childhood HP, which might be a compensation for a mechanically incompetent bony structure, decreased over time. There might be a pathophysiological link to continually elevated systemic prostaglandins.
UR - http://www.scopus.com/inward/record.url?scp=34248191819&partnerID=8YFLogxK
U2 - 10.1016/j.jbspin.2006.06.017
DO - 10.1016/j.jbspin.2006.06.017
M3 - Journal articles
C2 - 17420150
AN - SCOPUS:34248191819
SN - 1297-319X
VL - 74
SP - 263
EP - 269
JO - Joint Bone Spine
JF - Joint Bone Spine
IS - 3
ER -