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https://www.readbyqxmd.com/read/28797219/unresolving-trismus-following-third-molar-surgery-report-of-a-case-of-fibrodysplasia-ossificans-progressiva-with-review-of-literature
#1
B R Rajanikanth, Kavitha Prasad, K Vineeth, Sushma M N Sonale, Khuteja Al-Kubra
BACKGROUND: Trismus is a problem commonly encountered by the dental practitioner. It has a number of potential causes, and its treatment will depend on the cause. However, there are very few reports of trismus due to fibrodysplasia ossificans progressiva (FOP) following third molar surgery. CLINICAL PRESENTATION: FOP is a rare human genetic disorder with characteristic clinical features like progressive formation of extraskeletal bone or heterotopic ossification and congenital malformation of the great toes...
August 11, 2017: Cranio: the Journal of Craniomandibular Practice
https://www.readbyqxmd.com/read/28795074/unsupervised-exercise-induced-myositis-ossificans-in-the-brachialis-muscle-of-a-young-healthy-male-a-case-report
#2
Nikiforos Galanis, Chara Stavraka, Evdoxia Valavani, John Kirkos
No abstract text is available yet for this article.
July 2017: Orthopaedic Journal of Sports Medicine
https://www.readbyqxmd.com/read/28782882/the-expansion-of-heterotopic-bone-in-fibrodysplasia-ossificans-progressiva-is-activin-a-dependent
#3
Jaymin Upadhyay, LiQin Xie, Lily Huang, Nanditha Das, Rachel C Stewart, Morgan C Lyon, Keryn Palmer, Saathyaki Rajamani, Chris Graul, Merryl Lobo, Tyler J Wellman, Edward J Soares, Matthew D Silva, Jacob Hesterman, Lili Wang, Xialing Wen, Xiaobing Qian, Kalyan Nannuru, Vincent Idone, Andrew J Murphy, Aris N Economides, Sarah J Hatsell
Fibrodysplasia Ossificans Progressiva (FOP) is a rare autosomal dominant disorder that is characterized by episodic yet cumulative heterotopic ossification (HO) in skeletal muscles, tendons, and ligaments over a patient's lifetime. FOP is caused by missense mutations in the type I Bone Morphogenetic Protein (BMP) receptor ACVR1. We have determined that the formation of heterotopic bone in FOP requires activation of mutant ACVR1 by Activin A, in part by showing that prophylactic inhibition of Activin A blocks HO in a mouse model of FOP...
August 7, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28780628/surgical-excision-of-symptomatic-mature-posttraumatic-myositis-ossificans-characteristics-and-outcomes-in-32-athletes
#4
Sakari Orava, Juha-Jaakko Sinikumpu, Janne Sarimo, Lasse Lempainen, Gideon Mann, Iftach Hetsroni
PURPOSE: The purpose of this study was to report characteristics and outcomes of surgical excision of symptomatic mature posttraumatic myositis ossificans in adult athletes. The hypothesis was that surgical excision of the ossified mass in these circumstances can effectively relief symptoms and result in return to high-level sports with minimal postoperative complications. METHODS: All operations involving excision of posttraumatic heterotopic ossifications performed between 1987 and 2015 were reviewed...
August 5, 2017: Knee Surgery, Sports Traumatology, Arthroscopy: Official Journal of the ESSKA
https://www.readbyqxmd.com/read/28780023/shared-acvr1-mutations-in-fop-and-dipg-opportunities-and-challenges-in-extending-biological-and-clinical-implications-across-rare-diseases
#5
Harry J Han, Payal Jain, Adam C Resnick
Gain-of-function mutations in the Type I Bone Morphogenic Protein (BMP) receptor ACVR1 have been identified in two diseases: Fibrodysplasia Ossificans Progressiva (FOP), a rare autosomal dominant disorder characterized by genetically driven heterotopic ossification, and in 20-25% of Diffuse Intrinsic Pontine Gliomas (DIPGs), a pediatric brain tumor with no effective therapies and dismal median survival. While the ACVR1 mutation is causal for FOP, its role in DIPG tumor biology remains under active investigation...
August 2, 2017: Bone
https://www.readbyqxmd.com/read/28758906/activin-a-enhances-mtor-signaling-to-promote-aberrant-chondrogenesis-in-fibrodysplasia-ossificans-progressiva
#6
Kyosuke Hino, Kazuhiko Horigome, Megumi Nishio, Shingo Komura, Sanae Nagata, Chengzhu Zhao, Yonghui Jin, Koichi Kawakami, Yasuhiro Yamada, Akira Ohta, Junya Toguchida, Makoto Ikeya
Fibrodysplasia ossificans progressiva (FOP) is a rare and intractable disease characterized by extraskeletal bone formation through endochondral ossification. Patients with FOP harbor point mutations in ACVR1, a type I receptor for BMPs. Although mutated ACVR1 (FOP-ACVR1) has been shown to render hyperactivity in BMP signaling, we and others have uncovered a mechanism by which FOP-ACVR1 mistransduces BMP signaling in response to Activin-A, a molecule that normally transduces TGF-β signaling. Although Activin-A evokes enhanced chondrogenesis in vitro and heterotopic ossification (HO) in vivo, the underlying mechanisms have yet to be revealed...
July 31, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28756986/extraskeletal-aneurysmal-bone-cyst-report-of-a-case-and-review-of-the-literature
#7
Liurka V Lopez, Michael G Rodriguez, Gene P Siegal, Shi Wei
Aneurysmal bone cyst (ABC) is an expansile cystic lesion that may affect any bone of the skeleton. Although exceedingly rare, lesions with histomorphologic characteristics of an ABC have reportedly originated within soft tissue. Extraskeletal ABC may mimic a variety of benign and malignant lesions and can be confused with other more common or rare giant cell-rich tumors of soft tissue, especially myositis ossificans. Clinical, radiological and histologic correlation is crucial in reaching the correct diagnosis...
July 6, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28754575/heterotopic-bone-induction-via-bmp-signaling-potential-therapeutic-targets-for-fibrodysplasia-ossificans-progressiva
#8
Takenobu Katagiri, Sho Tsukamoto, Mai Kuratani
More than 50years ago, Marshal M. Urist detected "heterotopic bone-inducing activity" in demineralized bone matrix. This unique activity was referred to as "bone morphogenetic protein (BMP)" because it was sensitive to trypsin digestion. Purification of the bone-inducing activity from demineralized bone matrix using a bone-inducing assay in vivo indicated that the original "BMP" consisted of a mixture of new members of the transforming growth factor-β (TGF-β) family. The establishment of new in vitro assay systems that reflect the bone-inducing activity of BMPs in vivo have revealed the functional receptors and downstream effectors of BMPs...
July 25, 2017: Bone
https://www.readbyqxmd.com/read/28751434/atypical-presentation-and-management-of-fibrodysplasia-ossificans-progressiva
#9
André Grenho, Joana Arcângelo, Augusto Martins
We report a case of an 18-year-old woman, with bilateral acute inflammatory pain on the hip area, during the premenstrual period, and progressive increase in volume and rigidity of both hips. Bilateral exuberant soft tissue calcifications were present on the radiographic exams, and the patient also presented with bilateral short-length hallux valgus. A heterozygous mutation in the protein kinase domain of ACVR1 gene was found, allowing the diagnosis of fibrodysplasia ossificans progressive. Due to the relation between the disease flares and the premenstrual period, the patient was put into a chemically induced amenorrhea, with no new inflammatory crises since...
July 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28745249/-a-hardening-in-the-forearm-after-an-olecranon-fracture
#10
C M M Peeters, J C Sluimer, T Gosens
A 77-year-old male who had had an olecranon fracture 15 years ago presented himself with mild pain of the right elbow. Physical examination revealed painless hardening of the finger and wrist flexors in an area of 10 cm from the origin. Conventional radiographs showed a mature myositis ossificans.
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28741585/a-fatal-case-of-metastatic-squamous-cell-carcinoma-in-a-patient-with-myositis-ossificans-traumatica
#11
I N Vlasveld, H Scheper, J Stalenhoef, J M Baas, J van Dissel
Myositis ossificans traumatica is a rare disease associated with chronic wounds and fistulae. Chronic ulcers, fistulae and wounds can transform into squamous cell carcinoma, the so-called Marjolin's ulcer. We describe a rapid, progressive and fulminant course of a metastatic squamous cell carcinoma arising from a chronic wound in a patient with myositis ossificans traumatica.
July 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28736245/early-clinical-observations-on-the-use-of-imatinib-mesylate-in-fop-a-report-of-seven-cases
#12
Frederick S Kaplan, Jeffrey R Andolina, Peter C Adamson, David T Teachey, Jerry Z Finklestein, David H Ebb, Benjamin Whitehead, Benjamin Jacobs, David M Siegel, Richard Keen, Edward Hsiao, Robert J Pignolo
BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is an ultrarare genetic disorder of progressive, disabling heterotopic ossification (HO) for which there is presently no definitive treatment. Research studies have identified multiple potential targets for therapy in FOP, and novel drug candidates are being developed for testing in clinical trials. A complementary approach seeks to identify approved drugs that could be re-purposed for off-label use against defined targets in FOP...
July 20, 2017: Bone
https://www.readbyqxmd.com/read/28728261/-characteristics-of-the-joint-involvement-in-fibrodysplasia-ossificans-progressiva-and-follow-up
#13
J Zhu, Y Liu, Y Yang, J M Lai, G X Su, M Kang, S N Li, Z X Zhou, F Q Wu
Objective: To summarize the clinical data of 15 patients with fibrodysplasia ossificans progressiva (FOP), follow up and analyze the characteristics of the joint involvement in FOP. Method: From May 2005 to December 2016, fifteen FOP cases had been diagnosed in the Children's Hospital Capital Institute of Pediatrics. All medical records and follow-up data were collected and a retrospective analysis was made on the joint involvement in FOP. Pearson correlation analysis was used for data, P<0.05 for the difference was statistically significant...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28716575/strategic-targeting-of-multiple-bmp-receptors-prevents-trauma-induced-heterotopic-ossification
#14
Shailesh Agarwal, Shawn J Loder, Christopher Breuler, John Li, David Cholok, Cameron Brownley, Jonathan Peterson, Hsiao H Hsieh, James Drake, Kavitha Ranganathan, Yashar S Niknafs, Wenzhong Xiao, Shuli Li, Ravindra Kumar, Ronald Tompkins, Michael T Longaker, Thomas A Davis, Paul B Yu, Yuji Mishina, Benjamin Levi
Trauma-induced heterotopic ossification (tHO) is a condition of pathologic wound healing, defined by the progressive formation of ectopic bone in soft tissue following severe burns or trauma. Because previous studies have shown that genetic variants of HO, such as fibrodysplasia ossificans progressiva (FOP), are caused by hyperactivating mutations of the type I bone morphogenetic protein receptor (T1-BMPR) ACVR1/ALK2, studies evaluating therapies for HO have been directed primarily toward drugs for this specific receptor...
August 2, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28716551/application-of-human-induced-pluripotent-stem-cells-to-model-fibrodysplasia-ossificans-progressiva
#15
Emilie Barruet, Edward C Hsiao
Fibrodysplasia ossificans progressiva (FOP) is a genetic condition characterized by massive heterotopic ossification. FOP patients with mutations in the Activin A type I receptor (ACVR1), a bone morphogenetic protein (BMP) receptor. FOP is a progressive and debilitating disease characterized by bone formation flares that often occur after trauma. Since it is often difficult or impossible to obtain large amounts of tissue from human donors due to the risks of inciting more heterotopic bone formation, human induced pluripotent stem cells (hiPSCs) provide an attractive source for establishing in vitro disease models and for applications in drug screening...
July 14, 2017: Bone
https://www.readbyqxmd.com/read/28705683/periodontal-ligament-fibroblasts-as-a-cell-model-to-study-osteogenesis-and-osteoclastogenesis-in-fibrodysplasia-ossificans-progressiva
#16
Teun J de Vries, Ton Schoenmaker, Dimitra Micha, Jolanda Hogervorst, Siham Bouskla, Tim Forouzanfar, Gerard Pals, Coen Netelenbos, E Marelise W Eekhoff, Nathalie Bravenboer
Fibrodysplasia Ossificans Progressiva (FOP) is a progressive disease characterized by periods of heterotopic ossification of soft connective tissues, including ligaments. Though progress has been made in recent years in unraveling the underlying mechanism, patient-derived cell models are necessary to test potential treatment options. Periodontal ligament fibroblasts (PLF) from extracted teeth can be used to study deviant bone modeling processes in vitro since these cells are derived from genuine ligaments. They further provide a tool to study the hitherto unknown role of the bone morphogenesis protein receptor type 1 (BMPR-1) Activin A type 1 receptor ACVR1-R206H mutation in osteoclastogenesis...
July 10, 2017: Bone
https://www.readbyqxmd.com/read/28688556/pediatric-langerhans-cell-histiocytosis-of-the-lateral-skull-base
#17
Ananya Majumder, Cameron C Wick, Rebecca Collins, Timothy N Booth, Brandon Isaacson, J Walter Kutz
OBJECTIVE: Describe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone. METHODS: A retrospective chart review was performed between 2000 and 2014 at a single tertiary care children's hospital. Fourteen patients were identified with a diagnosis of LCH and involvement of the temporal bone. RESULTS: Ten patients were female and ten were Caucasian...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28679511/traumatic-myositis-ossificans-circumscripta-moc
#18
Manel Landolsi, Tej Mrad
We report a case of a 29-year-old man who had been a victim of a public road accident. Four weeks later, the patient developed an isolated right thigh mass located ventrally in the distal one-third of the thigh. The mass was painful and associated with fever and inflammatory syndrome. Plain radiographs showed a bilateral calcified thickening of soft tissues with well-defined bony margins. Ultrasound objectified diffuse calcifications of soft tissues.CT scan-confirmed the diagnosis of myositis ossificans circumscripta, showing a bilateral thickening of the vastus intermedius chief of the quadriceps dotted with calcifications, extending along the femur axis...
July 5, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28666455/prevalence-of-fibrodysplasia-ossificans-progressiva-fop-in-france-an-estimate-based-on-a-record-linkage-of-two-national-databases
#19
Geneviève Baujat, Rémy Choquet, Stéphane Bouée, Viviane Jeanbat, Laurène Courouve, Amélie Ruel, Caroline Michot, Kim-Hanh Le Quan Sang, David Lapidus, Claude Messiaen, Paul Landais, Valérie Cormier-Daire
BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a rare, severely disabling, and life-shortening genetic disorder that causes the formation of heterotopic bone within soft connective tissue. Previous studies found that the FOP prevalence was about one in every two million lives. The aim of this study is to estimate the FOP prevalence in France by probabilistic record-linkage of 2 national databases: 1) the PMSI (Programme de médicalisation des systèmes d'information), an administrative database that records all hospitalization activities in France and 2) CEMARA, a registry database developed by the French Centres of Reference for Rare Diseases...
June 30, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28641932/myositis-ossificans-in-elbow-mimicking-soft-tissue-sarcoma-similar-clinical-and-radiological-findings
#20
Elena Espinosa Muñoz, Diego Ramírez Ocaña, Ana María Martín García, Carmen Puentes Zarzuela
No abstract text is available yet for this article.
June 19, 2017: Reumatología Clinica
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