sj-docx-1-jet-10.1177_15266028231213611 – Supplemental material for The Additional Value of Activated Clotting Time–Guided Heparinization During Interventions for Peripheral Arterial Disease

Supplemental material, sj-docx-1-jet-10.1177_15266028231213611 for The Additional Value of Activated Clotting Time–Guided Heparinization During Interventions for Peripheral Arterial Disease by Liliane C. Roosendaal, Mila Radović, Max Hoebink, Arno M. Wiersema, Jan D. Blankensteijn and Vincent Jongkind in Journal of Endovascular Therapy</p

sj-docx-1-jet-10.1177_15266028231199714 – Supplemental material for Perprocedural Heparinization in Non-cardiac Arterial Procedures: The Current Practice in the Netherlands

Supplemental material, sj-docx-1-jet-10.1177_15266028231199714 for Perprocedural Heparinization in Non-cardiac Arterial Procedures: The Current Practice in the Netherlands by Liliane C. Roosendaal, Max Hoebink, Arno M. Wiersema, Kak K. Yeung, Jan D. Blankensteijn and Vincent Jongkind in Journal of Endovascular Therapy</p

[18F]NaF PET/CT scan as an early marker of heterotopic ossification in fibrodysplasia ossificans progressiva

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease with a progressive course characterized by episodically local flare-ups, which often but not always leads to heterotopic bone formation (HO). Recently, we showed that [18F]NaF PET/CT may be the first tool to monitor progression of a posttraumatic flare-up leading to new HO, which was demonstrated in a patient with FOP who underwent a maxillofacial surgery. This paper evaluates [18F]NaF PET/CT as a marker of FOP disease activity, comparing its use with other imaging modalities known in literature. In addition, the follow-up of a spontaneous flare-up in a 19-year old patient is presented showing high muscle [18F]NaF uptake in one defined part within the flare-up area after three weeks. During follow-up [18F]NaF PET /CT scan revealed newly formed heterotopic bone but only in this previously active [18F]NaF region. In conclusion, increased muscle [18F]NaF uptake may predict future HO development in FOP patients. At present [18F]NaF PET/CT appears to be a sensitive imaging modality to serve as a noninvasive marker for bone formation and to monitor disease activity during flare-ups in FOP

Deterioration of pulmonary function: An early complication in Fibrodysplasia Ossificans Progressiva

Fibrodysplasia Ossificans Progressiva (FOP) is a genetic disease characterized by the formation of heterotopic ossification (HO) in connective tissues. HO first develops in the thoracic region, before more peripheral sites are affected. Due to HO along the thoracic cage, its movements are restricted and pulmonary function deteriorates. Because development of HO is progressive, it is likely that pulmonary function deteriorates over time, but longitudinal data on pulmonary function in FOP are missing. Longitudinal pulmonary function tests (PFTs) from seven FOP patients were evaluated retrospectively to assess whether there were changes in pulmonary function during aging. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), total lung capacity (TLC), residual volume (RV) and diffusing lung capacity for carbon dioxide divided by alveolar volume (DLCO/VA) were included. In addition, HO volume along the thorax together with its progression as identified by whole body low dose CT scans were correlated to PFT data. Per patient, aged 7–57 years at the time of the first PFT, three to nine PFTs were available over a period of 6–18 years. Restrictive pulmonary function, identified by TLC or suspected by FVC, was found in all, but one, patients. In three patients, TLC, FVC or both decreased further during the follow-up period. All, but one, patients had an increased RV. The DLCO/VA ratio was normal in all FOP patients. Interestingly, FEV1 increased after a surgical intervention to unlock the jaw. In four out of five patients total HO volume in the thoracic region progressed beyond early adulthood, but no further decline in FVC was observed. In conclusion, restrictive pulmonary function was found in the majority of patients already at an early age. Our data suggest that the deterioration in pulmonary function is age dependent