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https://www.readbyqxmd.com/read/28641932/myositis-ossificans-in-elbow-mimicking-soft-tissue-sarcoma-similar-clinical-and-radiological-findings
#1
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
https://www.readbyqxmd.com/read/28629737/the-obligatory-role-of-activin-a-in-the-formation-of-heterotopic-bone-in-fibrodysplasia-ossificans-progressiva
#2
Dana M Alessi Wolken, Vincent Idone, Sarah J Hatsell, Paul B Yu, Aris N Economides
Fibrodysplasia Ossificans Progressiva (FOP) is a rare genetic disorder that presents at birth with only minor patterning defects, but manifests its debilitating pathology early in life with episodic, yet progressive and cumulative, heterotopic ossification (HO) of ligaments, tendons, and a subset of major skeletal muscles. The resulting HO lesions are endochondral in nature, and appear to be linked to inflammatory stimuli arising in association with known injuries, or from inflammation linked to normal tissue repair...
June 16, 2017: Bone
https://www.readbyqxmd.com/read/28627475/joint-specific-risk-of-impaired-function-in-fibrodysplasia-ossificans-progressiva-fop
#3
Robert J Pignolo, Blythe P Durbin-Johnson, David M Rocke, Frederick S Kaplan
BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) causes progressive disability due to heterotopic ossification from episodic flare-ups. Using data from 500 FOP patients (representing 63% of all known patients world-wide), age- and joint-specific risks of new joint involvement were estimated using parametric and nonparametric statistical methods. RESULTS: Compared to data from a 1994 survey of 44 individuals with FOP, our current estimates of age- and joint-specific risks of new joint involvement are more accurate (narrower confidence limits), based on a wider range of ages, and have less bias due to its greater comprehensiveness (captures over three-fifths of the known FOP patients worldwide)...
June 13, 2017: Bone
https://www.readbyqxmd.com/read/28623757/heterotopic-mesenteric-and-abdominal-wall-ossification-two-case-reports-in-one-institution
#4
Cátia Ferreira, Carina Gomes, Ana Melo, Nádia Tenreiro, Bruno Pinto, Herculano Moreira, Artur Ribeiro, Paulo Avelar
INTRODUCTION: Heterotopic ossification occurs when bone develops in tissues which usually don't undergo ossification. Heterotopic mesenteric ossification, also known as intra-abdominal myositis ossificans, is a rare and benign form of ossification, usually related with previous abdominal surgery or trauma. PRESENTATION OF CASES: We report two cases of heterotopic ossification both after multiple abdominal surgeries, with intraoperative findings of mesenteric and abdominal wall ossification...
June 8, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28606101/international-physician-survey-on-management-of-fop-a-modified-delphi-study
#5
Maja Di Rocco, Genevieve Baujat, Marta Bertamino, Matthew Brown, Carmen L De Cunto, Patricia L R Delai, Elisabeth M W Eekhoff, Nobuhiko Haga, Edward Hsiao, Richard Keen, Rolf Morhart, Robert J Pignolo, Frederick S Kaplan
Fibrodysplasia ossificans progressiva (FOP), a disabling disorder of progressive heterotopic ossification (HEO), is caused by heterozygous gain-of- function mutations in Activin receptor A, type I (ACVR1, also known as ALK2), a bone morphogenetic protein (BMP) type I receptor. Presently, symptomatic management is possible, but no definitive treatments are available. Although extensive guidelines for symptomatic management are widely used, regional preferences exist. In order to understand if there was worldwide consensus among clinicians treating FOP patients, an expert panel of physicians directly involved in FOP patient care was convened...
June 12, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28600150/longitudinal-patient-reported-mobility-assessment-in-fibrodysplasia-ossificans-progressiva-fop
#6
Frederick S Kaplan, Mona Al Mukaddam, Robert J Pignolo
BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a rare, disabling genetic disorder characterized by episodic soft tissue swelling (flare-ups) that leads to progressive heterotopic ossification and restricted joint mobility. METHODS: Here we present the first longitudinal patient-reported mobility assessment (PRMA) in FOP based on a simple evaluation tool. At initial presentation and follow-up (1-11year span; median: 6 year span), 64 patients (36 females; 28 males) with classic FOP completed a questionnaire designed to rapidly assess mobility at 15 sites (three axial; six upper limb, and six lower limb)...
June 6, 2017: Bone
https://www.readbyqxmd.com/read/28579631/fibrodysplasia-ossificans-progressiva-a-case-report
#7
Richard O Baidoo, Makafui S Dayie
Fibrodysplasia Ossificans Progressiva is a rare debilitating disorder of the musculoskeletal system affecting one in two million individuals. It is characterized by progressive extraskeletal ossification of soft tissues resulting in the original skeleton being encased in unyielding new bone leading to disability and ultimately death from cardiorespiratory failure. The present case brings to light the delays and potential pitfalls in diagnosis as a result of the rarity of the condition.
December 2016: Ghana Medical Journal
https://www.readbyqxmd.com/read/28549688/fibrodysplasia-ossificans-progressiva-the-patient-voice
#8
Ian L Cali, Laura Rossano
The following essays are the personal statements of two remarkable young individuals, Ian Cali and Laura Rossano, who candidly share their perspectives on living life with fibrodysplasia ossificans progressiva (FOP). These essays are excerpts from the opening comments that Ian and Laura delivered at The First and The Second International FOP Association Drug Development Forums in 2014 and 2016, respectively. We present these unedited essays in this special issue of BONE so that physicians, scientists, and researchers everywhere can glimpse the valiant challenges that envelop the lives of all individuals with FOP and can appreciate that diseases are not just biological processes but indelible human experiences...
May 23, 2017: Bone
https://www.readbyqxmd.com/read/28536749/extraskeletal-osteosarcoma-arising-in-myositis-ossificans-a-case-report-and-review-of-the-literature
#9
Deepika Savant, Shachar Kenan, Samuel Kenan, Leonard Kahn
We report a case of a 23-year-old female with pain and fullness in the right popliteal fossa. An MRI scan demonstrated an ossified enhancing soft tissue mass. Evaluation of the resected specimen showed a high-grade extraskeletal osteosarcoma juxtaposed to the three zones of myositis ossificans. At 1-year follow-up the patient is alive and disease free. A review of the literature purporting to document such an association failed to demonstrate a single case in which an osteosarcoma and MO with its zonal architecture co-existed...
August 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28527295/periostitis-ossificans-arising-in-the-mandibular-bone-of-a-young-patient-report-of-an-unusual-case-and-review-of-the-literature
#10
Masakatsu Fukuda, Katsuyuki Inoue, Hideaki Sakashita
Periostitis ossificans, also known as Garré osteomyelitis, is a specific type of chronic osteomyelitis that forms new bone under the periosteum resulting from a periosteal reaction to chronic inflammation or infections. It commonly affects the mandible secondary to odontogenic infection. The therapeutic approach involves eliminating the infectious cause and antibiotic administration. This report describes an unusual case of periostitis ossificans arising from the mandible of an 11-year-old boy. The cause of infection was correlated with a lower right unerupted third molar, which had no obvious connection with the oral cavity...
April 26, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28484423/evaluation-of-salivary-cytokines-for-diagnosis-of-both-trauma-induced-and-genetic-heterotopic-ossification
#11
Hsiao Hsin Sung Hsieh, Michael T Chung, Ronald M Allen, Kavitha Ranganathan, Joe Habbouche, David Cholok, Jonathan Butts, Arminder Kaura, Ramkumar Tiruvannamalai-Annamalai, Chris Breuler, Caitlin Priest, Shawn J Loder, John Li, Shuli Li, Jan Stegemann, Steven L Kunkel, Benjamin Levi
PURPOSE: Heterotopic ossification (HO) occurs in the setting of persistent systemic inflammation. The identification of reliable biomarkers can serve as an early diagnostic tool for HO, especially given the current lack of effective treatment strategies. Although serum biomarkers have great utility, they can be inappropriate or ineffective in traumatic acute injuries and in patients with fibrodysplasia ossificans progressiva (FOP). Therefore, the goal of this study is to profile the cytokines associated with HO using a different non-invasive source of biomarkers...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28473268/the-congenital-great-toe-malformation-of-fibrodysplasia-ossificans-progressiva-a-close-call
#12
O Will Towler, Eileen M Shore, Meiqi Xu, Abbey Bamford, Ilse Anderson, Robert J Pignolo, Frederick S Kaplan
BACKGROUND: Congenital bilateral hallux valgus with associated absence or fusion of the interphalangeal joint is a classic diagnostic feature of fibrodysplasia ossificans progressiva (FOP), a human genetic disease of extra-skeletal bone formation caused in nearly all cases by a gain-of-function mutation in Activin A Receptor I/Activin-like Kinase 2 (ACVR1/ALK2), which encodes a bone morphogenetic protein (BMP) Type 1 receptor. This toe malformation prompts the suspicion of FOP even before the appearance of extra-skeletal bone...
May 1, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28465250/a-cumulative-analogue-joint-involvement-scale-cajis-for-fibrodysplasia-ossificans-progressiva-fop
#13
Frederick S Kaplan, Mona Al Mukaddam, Robert J Pignolo
BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a catastrophic genetic disorder of progressive heterotopic ossification (HO). Assessment of functional mobility in FOP will be essential to support clinical trials of investigational agents. RESULTS: Of necessity, we developed a simple, rapidly-administered, cumulative analogue joint involvement scale (CAJIS) for FOP based on assessments in 144 individuals worldwide with classic FOP. CONCLUSIONS: CAJIS scores correlated with patient age, activities of daily living, and ambulatory function with excellent inter-rater variability...
April 29, 2017: Bone
https://www.readbyqxmd.com/read/28423433/-myositis-ossificans-traumatica-in-the-craniocervical-junction-a-case-report-and-review-of-literature
#14
Andreas Reinke, Michael Kraus, Alexander Wild
Background Myosits ossificans (MO) is a rare but important differential diagnosis for a heterotrophic bony tumor in the muscles. It is often misdiagnosed as a malignant tumor. With a previous trauma the diagnosis is myositis ossificans traumatic (MOT). In most cases, it is benign and predominantly seen in the big muscles. But there can be malignant etiologies too. Case Description We report a rare case of MO in the muscle of the craniocervical junction. This 37-year-old woman had a riding accident years ago...
April 19, 2017: Zeitschrift Für Orthopädie und Unfallchirurgie
https://www.readbyqxmd.com/read/28420848/myositis-ossificans-of-the-hip-due-to-pyogenic-arthritis-caused-by-campylobacter-fetus-subspecies-fetus
#15
Sho Nishiguchi, Ichiro Sekine, Shun Kuroda, Morihiko Sato, Izumi Kitagawa
A 61-year-old woman was admitted with severe hip pain causing immobility and high serum levels of inflammatory markers. The patient had a medical history of diabetes. She had been scheduled to undergo right hip replacement surgery for the treatment of osteoarthritis associated with gradually progressive pain. On admission, an enhanced abdominal computed tomography scan showed an abnormal increase in synovial fluid surrounding the right ilium, with piriformis muscle calcification. Subsequent blood and specimen cultures identified Campylobacter fetus subspecies fetus...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28416432/-transverse-reductional-anomaly-and-atypical-fibrodysplasia-ossificans-progressiva-a-case-diagnosed-late
#16
J Paysal, C Sarret, E Merlin, R Ravazzolo, R Bocciardi, J-M Garcier, S Monnot, F Laffargue, G Baujat, S Echaubard
Fibrodysplasia ossificans progressiva (FOP) is a rare disease characterized by the association of congenital bone abnormalities and extraskeletal ossification flare-ups occurring in muscles and fasciae. Early diagnosis is important to prevent ossification flare-ups, but some atypical presentations can lead to errors in diagnosis and therefore delay. Here, we report on a case of an atypical presentation of FOP in a girl, in whom prominent transverse reductional abnormalities delayed diagnosis. The patient developed extraskeletal ossifications and progressive fibrosis that led to motor restrictions...
April 14, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28405043/anaesthetic-management-of-a-child-with-stone-man-syndrome-look-before-you-leap
#17
Geeta Kamal, Anju Gupta, Sapna Batla, Nishkarsh Gupta
Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP...
March 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28394244/a-zebrafish-model-of-human-fibrodysplasia-ossificans-progressiva
#18
Melissa LaBonty, Nicholas Pray, Pamela C Yelick
Fibrodysplasia ossificans progressiva (FOP) is a rare, autosomal dominant genetic disorder in humans characterized by explosive inflammatory response to injury leading to gradual ossification within fibrous tissues, including skeletal muscle, tendons, and ligaments. A variety of animal models are needed to study and understand the etiology of human FOP. To address this need, here we present characterizations of the first adult zebrafish model for FOP. In humans, activating mutations in the Type I BMP/TGFβ family member receptor, ACVR1, are associated with FOP...
April 10, 2017: Zebrafish
https://www.readbyqxmd.com/read/28390760/restricted-mandibular-movement-attributed-to-ossification-of-mandibular-depressors-and-medial-pterygoid-muscles-in-patients-with-fibrodysplasia-ossificans-progressiva-a-report-of-3-cases
#19
Tetsuko Okuno, Hitoshi Suzuki, Akio Inoue, Jingo Kusukawa
Fibrodysplasia ossificans progressiva (FOP) is an extremely rare genetic condition characterized by congenital malformation and progressive heterotopic ossification (HO) caused by a recurrent single nucleotide substitution at position 617 in the ACVR1 gene. As the condition progresses, HO leads to joint ankylosis, breathing difficulties, and mouth-opening restriction, and it can shorten the patient's lifespan. This report describes 3 cases of FOP confirmed by genetic testing in patients with restricted mouth opening...
March 16, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28385291/management-of-myositis-ossificans-of-the-hand-a-case-report-and-a-review-of-the-literature
#20
Mohammad M Al-Qattan, Lenah Al-Fahdil, Haifa M Al-Shammari, Amin I Joarder
Myositis ossificans is a rare form of self-limiting heterotopic ossification of muscles. Most cases are seen in the thigh; the standard approach to these cases has been nonsurgical management awaiting spontaneous resolution. We report on a rare case of myositis ossificans of the hand with severe symptoms treated with early marginal excision without a trial of nonsurgical management.
April 3, 2017: Journal of Hand Surgery
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