Bone mineral density and fracture risk in adult patients with hypophosphatasia.

ABSTRACT: In adult hypophosphatasia (HPP) patients, elevated lumbar spine dual X-ray absorptiometry (DXA) values are associated with markers of disease severity and disease-specific fracture risk while femoral bone mineral density (BMD), being largely unaffected by the disease severity, may still be useful to monitor other causes of increased fracture risk due to low BMD.

Introduction

Hypophosphatasia (HPP) is a rare inherited metabolic disorder due to deficient activity of the tissue-nonspecific alkaline phosphatase (TNAP). Clinical manifestation in adult HPP patients is manifold including an increased risk for fractures, but data regarding clinical significance of DXA measurement and associations with fracture risk and disease severity is scarce.

Methods

Retrospective single-center a

SUBMITTER: Genest F 

PROVIDER: S-EPMC7838076 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature