Details

Autor(en) / Beteiligte

• Hepp, Nicola
• Folkestad, Lars
• Møllebæk, Simone
• Frederiksen, Anja Lisbeth
• Duno, Morten
• Jørgensen, Niklas Rye
• Hermann, Anne Pernille
• Jensen, Jens-Erik Beck

Titel

Bone-microarchitecture and bone-strength in a sample of adults with hypophosphatasia and a matched reference population assessed by HR-pQCT and impact microindentation

Ist Teil von

• Bone (New York, N.Y.), 2022-07, Vol.160, p.116420-116420, Article 116420

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2022

Link zum Volltext

Quelle

Elsevier ScienceDirect Journals Complete

Beschreibungen/Notizen

• Hypophosphatasia (HPP) is an autosomal recessive or dominate disease affecting bone mineralization, and adults with HPP are in risk to develop metatarsal stress fractures and femoral pseudofractures. Given to the scarce data on the bone quality and its association to the fracture risk in adults with HPP, this study aimed to evaluate bone turnover, bone strength and structure in adults with HPP. In this cross-sectional study, we included 14 adults with genetically verified HPP and 14 sex-, age-, BMI-, and menopausal status-matched reference individuals. We analyzed bone turnover markers, and measured bone material strength index (BMSi) by impact microindentation. Bone geometry, volumetric density and bone microarchitecture as well as failure load at the distal radius and tibia were evaluated using a second-generation high-resolution peripheral quantitative computed tomography system. Bone turnover markers did not differ between patients with HPP and reference individuals. BMSi did not differ between the groups (67.90 [63.75–76.00] vs 65.45 [58.43–69.55], p = 0.149). Parameters of bone geometry and volumetric density did not differ between adults with HPP and the reference group. Patients with HPP had a tendency toward higher trabecular separation (0.664 [0.613–0.724] mm vs 0.620 [0.578–0.659] mm, p = 0.054) and inhomogeneity of trabecular network (0.253 [0.235–0.283] mm vs 0.229 [0.208–0.252] mm, p = 0.056) as well as lower trabecular bone volume fraction (18.8 [16.4–22.7] % vs 22.8 [20.6–24.7] %, p = 0.054) at the distal radius. In addition, compound heterozygous adults with HPP had a significantly higher cortical porosity at the distal radius than reference individuals (1.5 [0.9–2.2] % vs 0.7 [0.6–0.7] %, p = 0.041). BMSi is not reduced in adults with HPP. Increased cortical porosity may contribute to the occurrence of femoral pseudofractures in compound heterozygous adults with HPP. However, further studies investigating larger cohorts of adults with HPP using methods of bone histomorphometry are recommended to adequately assess the bone quality in adults with HPP. •BMSi is not systematically reduced in adults with HPP.•Low BMSi does not associate with a higher number of fractures in adults with HPP.•Increased cortical porosity may contribute to fracture development in severe HPP.