Fibrodysplasia ossificans progressiva (FOP), sometimes known as myositis ossificans progressiva, is an ultra-rare disease in which bone is formed in muscular tissue, tendons and ligaments. This is known as heterotopic ossification (HO). FOP is caused by a heterozygous mutation in the highly conserved ACVR1/ALK2 gene which affects about 1 in 1.
View Article and Find Full Text PDFFibrodysplasia ossificans progressiva (FOP) is a rare disease in which heterotopic ossification (HO) is formed in muscles, tendons and ligaments. Traumatic events, including surgery, are discouraged as this is known to trigger a flare-up with risk of subsequent HO. Anesthetic management for patients with FOP is challenging.
View Article and Find Full Text PDFIn the field of rare bone diseases in particular, a broad care team of specialists embedded in multidisciplinary clinical and research environment is essential to generate new therapeutic solutions and approaches to care. Collaboration among clinical and research departments within a University Medical Center is often difficult to establish, and may be hindered by competition and non-equivalent cooperation inherent in a hierarchical structure. Here we describe the "collaborative organizational model" of the Amsterdam Bone Center (ABC), which emerged from and benefited the rare bone disease team.
View Article and Find Full Text PDFIntroduction: In The Netherlands there is no consensus about criteria for cancelling helicopter emergency medical services (HEMS) dispatches. This study assessed the ability of the primary HEMS dispatch criteria to identify major trauma patients. The predictive power of other early prehospital parameters was evaluated to design a safe triage model for HEMS dispatch cancellations.
View Article and Find Full Text PDFIntroduction: The Revised Trauma Score is used worldwide in the prehospital setting and provides a snapshot of patient's physiological state. Several studies have shown that the reliability of the RTS is high in trauma outcomes. In the Netherlands, Helicopter Emergency Medical Services (HEMS) are mostly used for delivery of specialized trauma teams on-scene and occasionally for patient transportation.
View Article and Find Full Text PDFBackground: The trauma centre of the Trauma Center Region North-West Netherlands (TRNWN) has consensus criteria for Mobile Medical Team (MMT) scene dispatch. The MMT can be dispatched by the EMS-dispatch centre or by the on-scene ambulance crew and is transported by helicopter or ground transport. Although much attention has been paid to improve the dispatch criteria, the MMT is often cancelled after being dispatched.
View Article and Find Full Text PDF