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Long-term follow-up of bone mineral density in childhood hypophosphatasia

Hermann Josef Girschick1, Imme Haubitz, Olaf Hiort

  • 1Children's Hospital, Section of pediatric rheumatology and osteology, Universitäts-Kinderklinik, University of Würzburg, Josef-Schneider-Strasse 2, Würzburg 97080, Germany. hermann.girschick@mail.uni-wuerzburg.de

Joint Bone Spine
|April 11, 2007
PubMed

Insights

Childhood hypophosphatasia (HP) shows decreased metaphyseal hypermineralization over four years. This bone metabolism disorder may link to elevated systemic prostaglandins.

Area of Science:

  • Biochemistry
  • Genetics
  • Pediatric Endocrinology

Background:

  • Hypophosphatasia (HP) is an inherited disorder of bone metabolism caused by genetic defects in the tissue-non-specific alkaline phosphatase (TNSALP) gene.
  • Limited long-term data exists on bone mineral density (BMD) in children with HP.

Purpose of the Study:

  • To prospectively analyze changes in BMD over four years in children with HP.
  • To investigate potential links between BMD changes, hyperprostaglandinism, and NSAID treatment in HP.

Main Methods:

  • Prospective 4-year follow-up of 6 pediatric HP patients.
  • Bone mineral density assessment using peripheral quantitative computed tomography (pQCT) and dual-energy X-ray absorptiometry (DXA).
  • Monitoring of urinary prostaglandin excretion to assess systemic hyperprostaglandinism.

Main Results:

  • Initial hypermineralization of trabecular bone in metaphyseal regions was observed at diagnosis.
  • A significant, gradual decrease in radial metaphyseal mineralization occurred over the 4-year follow-up.
  • Bone mineral content (BMC) and total body DXA values remained stable compared to healthy controls.
  • Systemic hyperprostaglandinism was documented in most patients during the follow-up period.

Conclusions:

  • Metaphyseal hypermineralization in childhood HP may represent a compensatory mechanism for structural bone weakness and decreases over time.
  • A potential pathophysiological connection exists between HP and persistently elevated systemic prostaglandin levels.
Abstract

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