Fibrodysplasia Ossificans Progressiva Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility
CONCLUSION: Most fractures treated nonoperatively in individuals with FOP healed with few flareups, little or no HO, and preservation of mobility, suggesting an uncoupling of fracture repair and HO, which are two inflammation-induced processes of endochondral ossification. These findings underscore the importance of considering nonoperative treatment for fractures in individuals with FOP. Physicians who treat fractures in patients with FOP should consult with a member of the International Clinical Council listed in the FOP Treatment Guidelines (https://www.iccfop.org).LEVEL OF EVIDENCE: Level IV, therapeutic study.PMID:371...
Source: Pain Physician - May 8, 2023 Category: Anesthesiology Authors: Carter M Lindborg Mona Al Mukaddam Genevieve Baujat Tae-Joon Cho Carmen L De Cunto Patricia L R Delai Elisabeth M W Eekhoff Nobuhiko Haga Edward C Hsiao Rolf Morhart Ruben de Ruiter Christiaan Scott Petra Seemann Ma łgorzata Szczepanek Jacek Tabarkiewicz Source Type: research

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility
CONCLUSION: Most fractures treated nonoperatively in individuals with FOP healed with few flareups, little or no HO, and preservation of mobility, suggesting an uncoupling of fracture repair and HO, which are two inflammation-induced processes of endochondral ossification. These findings underscore the importance of considering nonoperative treatment for fractures in individuals with FOP. Physicians who treat fractures in patients with FOP should consult with a member of the International Clinical Council listed in the FOP Treatment Guidelines ( https://www.iccfop.org ).LEVEL OF EVIDENCE: Level IV, therapeutic study.PMID:3...
Source: Clinical Orthopaedics and Related Research - May 8, 2023 Category: Orthopaedics Authors: Carter M Lindborg Mona Al Mukaddam Genevieve Baujat Tae-Joon Cho Carmen L De Cunto Patricia L R Delai Elisabeth M W Eekhoff Nobuhiko Haga Edward C Hsiao Rolf Morhart Ruben de Ruiter Christiaan Scott Petra Seemann Ma łgorzata Szczepanek Jacek Tabarkiewicz Source Type: research

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility
CONCLUSION: Most fractures treated nonoperatively in individuals with FOP healed with few flareups, little or no HO, and preservation of mobility, suggesting an uncoupling of fracture repair and HO, which are two inflammation-induced processes of endochondral ossification. These findings underscore the importance of considering nonoperative treatment for fractures in individuals with FOP. Physicians who treat fractures in patients with FOP should consult with a member of the International Clinical Council listed in the FOP Treatment Guidelines ( https://www.iccfop.org ).LEVEL OF EVIDENCE: Level IV, therapeutic study.PMID:3...
Source: Pain Physician - May 8, 2023 Category: Anesthesiology Authors: Carter M Lindborg Mona Al Mukaddam Genevieve Baujat Tae-Joon Cho Carmen L De Cunto Patricia L R Delai Elisabeth M W Eekhoff Nobuhiko Haga Edward C Hsiao Rolf Morhart Ruben de Ruiter Christiaan Scott Petra Seemann Ma łgorzata Szczepanek Jacek Tabarkiewicz Source Type: research

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility
CONCLUSION: Most fractures treated nonoperatively in individuals with FOP healed with few flareups, little or no HO, and preservation of mobility, suggesting an uncoupling of fracture repair and HO, which are two inflammation-induced processes of endochondral ossification. These findings underscore the importance of considering nonoperative treatment for fractures in individuals with FOP. Physicians who treat fractures in patients with FOP should consult with a member of the International Clinical Council listed in the FOP Treatment Guidelines ( https://www.iccfop.org ).LEVEL OF EVIDENCE: Level IV, therapeutic study.PMID:3...
Source: Clinical Orthopaedics and Related Research - May 8, 2023 Category: Orthopaedics Authors: Carter M Lindborg Mona Al Mukaddam Genevieve Baujat Tae-Joon Cho Carmen L De Cunto Patricia L R Delai Elisabeth M W Eekhoff Nobuhiko Haga Edward C Hsiao Rolf Morhart Ruben de Ruiter Christiaan Scott Petra Seemann Ma łgorzata Szczepanek Jacek Tabarkiewicz Source Type: research

Direct BMP signaling to chordoblasts is required for the initiation of segmented notochord sheath mineralization in zebrafish vertebral column development
The vertebral column, with the centra as its iteratively arranged building blocks, represents the anatomical key feature of the vertebrate phylum. In contrast to amniotes, where vertebrae are formed from chondrocytes and osteoblasts deriving from the segmentally organized neural crest or paraxial sclerotome, teleost vertebral column development is initiated by chordoblasts of the primarily unsegmented axial notochord, while sclerotomal cells only contribute to later steps of vertebrae formation. Yet, for both mammalian and teleostean model systems, unrestricted signaling by Bone Morphogenetic Proteins (BMPs) or retinoic ac...
Source: Frontiers in Endocrinology - May 8, 2023 Category: Endocrinology Source Type: research

GSE228522 Transcriptomic differences underlying the Activin-A induced large osteoclast formation in both healthy control and Fibrodysplasia Ossificans Progressiva osteoclasts
Contributors : T Schoenmaker ; R Volckmann ; T J de Vries ; E M EekhoffSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensActivin-A has been identified as the driver for heterotopic ossification in FOP. It's effect on osteoclast formation is not extensively studied in control or FOP samples. (Source: GEO: Gene Expression Omnibus)
Source: GEO: Gene Expression Omnibus - April 7, 2023 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research

A follow-up report on the published paper Social and clinical impact of COVID-19 on patients with fibrodysplasia ossificans progressiva
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder associated with increased immune activity and severe, progressive heterotopic ossification. We previously described a cohort of 32 patient... (Source: Orphanet Journal of Rare Diseases)
Source: Orphanet Journal of Rare Diseases - March 20, 2023 Category: Internal Medicine Authors: Hayley Wallace, Rhonda H. Lee and Edward C. Hsiao Tags: Letter to the Editor Source Type: research

Medullary Tegmental Cap Dysplasia: Fetal and Postnatal Presentations of a Unique Brainstem Malformation [PEDIATRICS]
CONCLUSIONS: This is the first study to describe a series of 13 patients with medullary tegmental cap dysplasia. The cap has different shapes: distinct in Joubert-Boltshauser syndrome and fibrodysplasia ossificans progressive. Due to the variations in the clinical, imaging, and postmortem findings, we conclude that there are multiple etiologies and pathophysiology. We suggest that in some patients, the pathophysiology might be abnormal axonal guidance. (Source: American Journal of Neuroradiology)
Source: American Journal of Neuroradiology - March 9, 2023 Category: Radiology Authors: Gafner, M., Garel, C., Leibovitz, Z., Valence, S., Krajden Haratz, K., Oegema, R., Mancini, G. M. S., Heron, D., Bueltmann, E., Burglen, L., Rodriguez, D., Huisman, T. A. G. M., Lequin, M. H., Arad, A., Kidron, D., Muqary, M., Gindes, L., Lev, D., Boltsha Tags: PEDIATRICS Source Type: research

A Pharmacokinetic, Safety, and Tolerability Trial of Palovarotene in Healthy Japanese and Non-Japanese Participants
ConclusionsJapanese and non-Japanese groups had similar pharmacokinetic profiles, indicating that palovarotene dose adjustments are not necessary for Japanese patients with FOP. (Source: European Journal of Drug Metabolism and Pharmacokinetics)
Source: European Journal of Drug Metabolism and Pharmacokinetics - February 18, 2023 Category: Drugs & Pharmacology Source Type: research

Knowledge and awareness about fibrodysplasia ossificans progressiva among dental students
J Adv Pharm Technol Res. 2022 Dec;13(Suppl 2):S554-S558. doi: 10.4103/japtr.japtr_165_22. Epub 2022 Dec 30.ABSTRACTFibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder which is autosomal dominant distinguished by congenital malformations of large toes and flare ups, etc. It is a disorder of connective tissue, with heterotopic ossifications seen with skeletal muscles, tendons, and cartilages and also called as Stone man disease, myositis ossificans, and Munchmeyer disease. The main objective of the study is to assess and create the awareness about FOP among dental students. An online-based survey was condu...
Source: Adv Data - February 17, 2023 Category: Epidemiology Authors: L Akshayaa S Kavitha V Vishnu Priya R Gayathri Source Type: research

Reduction of New Heterotopic Ossification (HO) in the Open ‐Label, Phase 3 MOVE Trial of Palovarotene for Fibrodysplasia Ossificans Progressiva (FOP)
ABSTRACTFibrodysplasia ossificans progressiva (FOP) is an ultra-rare, severely disabling genetic disorder of progressive heterotopic ossification (HO). The single-arm, open-label, phase 3 MOVE trial (NCT03312634) assessed efficacy and safety of palovarotene, a selective retinoic acid receptor gamma agonist, in patients with FOP. Findings were compared with FOP natural history study (NHS; NCT02322255) participants untreated beyond standard of care. Patients aged ≥4 years received palovarotene once daily (chronic: 5 mg; flare-up: 20 mg for 4 weeks, then 10 mg for ≥8 weeks; weight-adjusted if skeletally immatur...
Source: Journal of Bone and Mineral Research - January 26, 2023 Category: Orthopaedics Authors: Robert J. Pignolo, Edward C. Hsiao, Mona Al Mukaddam, Genevi ève Baujat, Staffan K. Berglund, Matthew A. Brown, Angela M. Cheung, Carmen De Cunto, Patricia Delai, Nobuhiko Haga, Peter Kannu, Richard Keen, Kim‐Hanh Le Quan Sang, Edna E. Man Tags: Clinical Trial Source Type: research