Long-term follow-up of bone mineral density in childhood hypophosphatasia - 24/05/07
, Imme Haubitz a, Olaf Hiort b, Peter Schneider c
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Abstract |
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 4years 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 4years 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.
Le texte complet de cet article est disponible en PDF.Keywords : Childhood hypophosphatasia, Bone mineral density, Hypermineralization, Hyperprostaglandinism
Abbreviations : HP, AP, TNSALP, CPPD, NSAID, BMD, BMC, pQCT, DXA, nd
Plan
Vol 74 - N° 3
P. 263-269 - mai 2007 Retour au numéro
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