Bone Abstracts

Fibrodysplasia ossificans progressiva (FOP) is a rare, extremely disabling genetic disorder characterized by progressive heterotopic ossification preceded by episodic inflammatory soft tissue swellings (flare ups) leading to early death. There is no proven effective treatment yet. We aimed to develop an in vitro system to investigate the working mechanism of flare ups induced ossification.Skin biopsies were obtained from four patients with FOP. ...

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disorder characterized by progressive heterotopic ossification. FOP patients only present great toe malformations at birth. However, as they grow older they develop soft tissue lumps as a result of flare-ups causing the irreversible replacement of skeletal muscle tissue with bone tissue leading to cumulative physical immobility. Classical FOP patients possess a mutation (c.617G>A; R206H) in the activin recept...

Fibrodysplasia ossificans progressiva (FOP) is a rare, autosomal dominant disorder characterized by heterotopic ossification (HO) in muscles, ligaments and tendons. Flare-ups often precede formation of HO, resulting in immobilized joints. Recently, it has been shown that [18F]NaF PET/CT could identify early ossifying disease activity during flare-ups. HO may progress without signs of flare-up, but its underlying physiology is not understood. We wondered whether [18F]NaF PET/CT...

Background: Fibrodysplasia Ossificans Progressiva (FOP) is a progressive disease characterized by periods of heterotopic ossification, often in ligaments. The underlying mechanism is far from clear, partially due to limited access to patient-derived cell models. Periodontal ligament fibroblasts (PLF) from extracted teeth can potentially be used to study deviant bone remodelling processes in vitro since these cells are derived from actual ligaments. They further provid...