Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by debilitating heterotopic ossification (HO). The retinoic acid receptor gamma agonist, palovarotene, and antibody-mediated activin A blockade have entered human clinical trials, but how these therapeutic modalities affect the behavior of pathogenic fibro/adipogenic progenitors (FAPs) is unclear. Using live-animal luminescence imaging, we show that transplanted pathogenic FAPs undergo rapid initial expansion, with peak number strongly correlating with HO severity. Palovarotene significantly reduced expansion of pathogenic FAPs, but was less effective than activin A inhibition, which restored wild-type population growth dynamics to FAPs. Palovarotene pretreatment did not reduce FAPs' skeletogenic potential, indicating that efficacy requires chronic administration. Although palovarotene inhibited chondrogenic differentiation in vitro and reduced HO in juvenile FOP mice, daily dosing resulted in aggressive synovial joint overgrowth and long bone growth plate ablation. These results highlight the challenge of inhibiting pathological bone formation prior to skeletal maturation.
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http://dx.doi.org/10.7554/eLife.40814 | DOI Listing |
BMC Surg
December 2024
Department of Spine Surgery, Tianjin Union Medical Center, Tianjin, 300121, China.
Background: The best open side for unilateral open-door laminoplasty (UODL) to treat inconsistent cervical ossification of the posterior longitudinal ligament (OPLL) needs to be identified.
Methods: Thirty-one individuals with inconsistent OPLL who underwent UODL between January 2016 and December 2018 were retrospectively divided into two groups: when the side of the open door was consistent with the side of the larger ossification occupancy area, patients were placed in the Consistent group; when the side of the open door was contralateral to the side of the larger ossification occupancy area, patients were placed in the Contralateral group. The following parameters were evaluated: neck disability index (NDI) score, Japanese Orthopaedic Association (JOA) score, visual analog scale (VAS) score, postoperative laminoplasty opening width and angle, and spinal cord diameter ratio.
BMC Musculoskelet Disord
December 2024
Department of Orthopedic Surgery, Nara Medical University, 840 Shijo-cho Kashihara City, Nara, 6348522, Japan.
Background: Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic non-inflammatory disorder characterized by enthesopathy and osteophyte formation. DISH can also cause several other symptoms. Limited range of motion (ROM) is the most common symptom; however, dysphagia and respiratory distress are clinically important symptoms.
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December 2024
Division of Spine Surgery, Department of Orthopaedics, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China. Electronic address:
BMC Musculoskelet Disord
December 2024
Department of Orthopedics Surgery, Shandong Provincial Hospital affiliated to Shandong First Medical University, 324 Jing Wu Road, Jinan, 250021, China.
Background: The present study aimed to compare the functional outcomes of hinged external fixators and non-external fixation in open elbow arthrolysis (OEA) for post-traumatic elbow stiffness (PTES) and to evaluate their applicability and limitations in patients with posttraumatic elbow stiffness.
Methods: The clinical data of patients with PTES treated with OEA at our hospital between March 2015 and June 2022 were retrospectively analyzed. The assessed variables were the operation time, intraoperative blood loss volume, duration of hospitalization, and treatment costs.
Arkh Patol
December 2024
St. Petersburg Research Institute of Phthisiopulmonology, St. Petersburg, Russia.
Microscopic changes in the lung biopsies of 16 adult patients of both sexes without clear clinical symptoms are described. According to X-ray examination data, disseminated process in the lungs was diagnosed in 4 patients, interstitial pneumonia in 4, osteoplastic pneumopathy in 3, as well as tuberculosis, bronchiolitis and a number of others. Microscopic examination showed that the patients had various forms of petrification and ossification of the lungs, diagnosed clinically and radiologically in only 3 cases.
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