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Bruno Law-Ye, Chloé Hangard, Adrien Felter, Dominique Safa, Philippe Denormandie, François Genet, Robert-Yves Carlier
BACKGROUND: Neurogenic Myositis Ossificans (NMO) is a rare disabling pathology characterized by peri-articular heterotopic ossifications following severe peripheral or central nervous system injuries. It results in ankylosis and vessels or nerves compressions. Our study aimed to describe the pre-operative findings of patients with NMO of the hip using biphasic computerized tomography (CT). METHODS: Between 2006 and 2012, we retrospectively analyzed 101 consecutive patients with hip NMO...
October 18, 2016: BMC Musculoskeletal Disorders
Laura Hildebrand, Katja Stange, Alexandra Deichsel, Manfred Gossen, Petra Seemann
Patients with Fibrodysplasia Ossificans Progressiva (FOP) suffer from ectopic bone formation, which progresses during life and results in dramatic movement restrictions. Cause of the disease are point mutations in the Activin A receptor type 1 (ACVR1), with p.R206H being most common. In this study we compared the signalling responses of ACVR1(WT) and ACVR1(R206H) to different ligands. ACVR1(WT), but not ACVR1(R206H) inhibited BMP signalling of BMP2 or BMP4 in a ligand binding domain independent manner. Likewise, the basal BMP signalling activity of the receptor BMPR1A or BMPR1B was inhibited by ACVR1(WT), but enhanced by ACVR1(R206H)...
October 4, 2016: Cellular Signalling
Jennifer C Hausmann, Jorden Manasse, Sarah Churgin, Howard Steinberg, Victoria L Clyde, Roberta Wallace
A 15-yr-old sexually intact female vampire bat ( Desmodus rotundus ) was diagnosed with myositis ossificans traumatica of the abdominal wall. The bat presented with a large ulcerated firm mass along the abdomen. Radiographs and cytology were performed, followed by surgical exploration. The mass was determined to be nonresectable and the bat was euthanized. Histopathology showed severe necrotizing, degenerative, and pyogranulomatous myositis with osseous and cartilaginous metaplasia, fibrosis, and ulceration, which were consistent with myositis ossificans traumatica...
September 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Quoc P Tran, Kannan Thanikachalam
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
A Nagaraja, N Govindraj Kumar, B Jambukeshwar Kumar, Raghavendra M Naik, Y Jyoti Sangineedi
Pathological conditions can give rise to calcifications within oral mucosa representing either a local or systemic disturbance. Inflammation, trauma, debris acting as nidus and vascular lesions have been attributed as principal causes for occurrence of calcifications within the oral mucosa. Occurrence of multiple calcified thrombi (phleboliths) is considered pathognomonic for hemangiomas and vascular malformations in the oral and maxillofacial region. Isolated occurrence of phlebolith in oral mucosa though very rare, especially without any underlying vascular lesions, can be diagnostically challenging...
2016: Journal of Contemporary Dental Practice
M Li, Y P Cai, K Y Lu, Y Chen, X Zhu, Y Yin, J Tang
Objective: To investigate the role of SATB2 in the pathological diagnosis and differential diagnosis of osteosarcoma. Methods: Immunostaining of SATB2 was performed in 47 cases of osteosarcomas, 5 osteoblastomas, 4 fibrous dysplasias, 5 myositis ossificans, 10 chondroblastomas, 8 chondrosarcomas, 5 Ewing sarcomas, 5 undifferentiated pleomorphic sarcomas, 6 fibrosarcomas and 2 leiomyosarcomas. Results: All osteoblastomas (5/5) and myositis ossificans (5/5), 83.0%(39/47) of osteosarcomas and 2/10 of chondroblastomas showed nuclear immunoreactivity for SATB2...
September 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Ikram Taam, Khouloud Boussouni, Bouchaib Redouane, Touriya Amil, Rachida Saouab
Myositis ossificans circumscripta (MOC) is a rare condition characterized by nontumoral heterotopic ossification of the soft tissues. This condition affects young subjects, occurring mainly after trauma. It is ubiquitous, predominantly located in girdles and limbs. We report the case of a young patient with paravertebral MOC without traumatic context; the aim of this study was to recall diagnostic criteria and imaging aspects.
2016: Pan African Medical Journal
Serena Cappato, Laura Tonachini, Francesca Giacopelli, Mario Tirone, Luis J V Galietta, Martina Sormani, Anna Giovenzana, Antonello E Spinelli, Barbara Canciani, Silvia Brunelli, Roberto Ravazzolo, Renata Bocciardi
No abstract text is available yet for this article.
September 1, 2016: Disease Models & Mechanisms
Mehmet Sabri Balik, Adem Erkut, Yılmaz Guvercin, Recep Bedir
A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis.
September 2016: Knee Surgery & related Research
Alun Yewlett, Jeff Kitson, Andrew Redfern, Chris Smith
Fibrodysplasia progressiva ossificans (FPO) is an extremely rare condition characterized by abnormal heterotopic bone formation. The condition is eponymously known as 'stoneman' disease because patients can become effectively entombed within abnormal heterotopic bone. We present the first known case of a diaphyseal humeral fracture managed conservatively in an adult patient with this condition. This patient already had a pre-existing bony bar from a fusion mass involving the thoracic spine, scapula and ribs to her proximal humerus splinting the arm in a position of adduction with the palm of her hand facing towards her groin...
January 2016: Shoulder & Elbow
Mariasavina Severino, Marta Bertamino, Domenico Tortora, Giovanni Morana, Sara Uccella, Renata Bocciardi, Roberto Ravazzolo, Andrea Rossi, Maja Di Rocco
BACKGROUND: Fibrodysplasia ossificans progressiva is an autosomal dominant disorder due to germline mutations of ACVR1/ALK2 causing progressive heterotopic endochondral ossifications. Evidence of central nervous system involvement has emerged only recently. METHODS: We performed an observational cross-sectional brain MRI study in 13 patients (8 females, mean age 20 years), examining the relationship of clinical and neuroradiological findings. RESULTS: All patients presented small asymptomatic lesions similar to hamartomas at the level of the dorsal medulla and ventral pons, associated with minor brainstem dysmorphisms and abnormal origin of the vestibulocochlear and facial nerves...
August 26, 2016: Journal of Medical Genetics
Emilie Barruet, Blanca M Morales, Wint Lwin, Mark P White, Christina V Theodoris, Hannah Kim, Ashley Urrutia, Sarah Anne Wong, Deepak Srivastava, Edward C Hsiao
BACKGROUND: The Activin A and bone morphogenetic protein (BMP) pathways are critical regulators of the immune system and of bone formation. Inappropriate activation of these pathways, as in conditions of congenital heterotopic ossification, are thought to activate an osteogenic program in endothelial cells. However, if and how this occurs in human endothelial cells remains unclear. METHODS: We used a new directed differentiation protocol to create human induced pluripotent stem cell (hiPSC)-derived endothelial cells (iECs) from patients with fibrodysplasia ossificans progressiva (FOP), a congenital disease of heterotopic ossification caused by an activating R206H mutation in the Activin A type I receptor (ACVR1)...
2016: Stem Cell Research & Therapy
Ali Birjandinejad, Mohammad-Hossein Taraz-Jamshidi, Sayyed Hadi Sayyed Hosseinian
Progressive osseous heteroplasia is a rare inherited disease that begins with skin ossification and proceeds into the deeper connective tissues. The disease should be distinguished from other genetic disorders of heterotopic ossification including fibrodysplasia ossificans progressiva (FOP) and Albright hereditary osteodystrophy (AHO). We report a case of progressive osseous heteroplasia in a twenty four years old male with a complaint of ankylosis of the entire upper left limb and digital cutaneous lesions and sparing of the other limbs and the axial skeleton...
June 2016: Archives of Bone and Joint Surgery
Kyung Ryeol Lee, So Young Park, Wook Jin, Kyu Yeoun Won
Myositis ossificans (MO) is a benign soft tissue lesion with non-neoplastic heterotopic bone formation. MO in the intermediate and mature stages can be easily diagnosed if characteristic imaging findings such as a peripheral zonal pattern of ossification with variable thickness is observed. However, it is difficult to correctly diagnose early MO because it can mimic malignancy clinically, radiologically, and histopathologically. We report a case of early pseudosarcomatous phase of non-traumatic MO with atypical imaging findings...
October 2016: Skeletal Radiology
Aleksandra Bogdan, Jean Quintin, Frédéric Schuind
PURPOSE: Humero-ulnar external fixation has been proposed to treat complex supracondylar humeral fractures in children. It facilitates fracture reduction and reduces the risk of ulnar nerve lesion, which can occur after cross pinning. METHODS: In a ten year period, 28 children have been operated on in our centre by humero-ulnar external fixation, for Lagrange-Rigault stages III and IV supracondylar humeral fractures. The data about fracture management and early follow-up were obtained from our medical database...
August 9, 2016: International Orthopaedics
Jing Pang, Yue Zuo, Yi Chen, Lige Song, Qi Zhu, Jing Yu, Chang Shan, Zeling Cai, Jijun Hao, Frederick S Kaplan, Eileen M Shore, Keqin Zhang
Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disease of heterotopic endochondral ossification (HEO), and currently no effective therapies are available for this disease. A recurrent causative heterozygous mutation (c.617 G>A; R206H) for FOP was identified in activin receptor type IA (ACVR1), a bone morphogenetic protein (BMP) type I receptor. This mutation aberrantly activates the BMP-Smad1/5/8 signaling pathway and leads to HEO in FOP patients. Here we report development of a soluble recombinant ACVR1-Fc fusion protein by combining the extracellular domain of human wild type ACVR1 and the Fc portion of human immunoglobulin gamma 1 (IgG1)...
November 2016: Bone
Sachi Hasegawa, Teresa Victoria, Hülya Kayserili, Elaine Zackai, Gen Nishimura, Nobuhiko Haga, Yasuharu Nakashima, Osamu Miyazaki, Hiroshi Kitoh
BACKGROUND: We have clinically encountered children with fibrodysplasia ossificans progressiva who had abnormal calcaneal ossification. OBJECTIVE: To evaluate whether calcaneal ossification variants are significant radiographic findings in children with fibrodysplasia ossificans progressiva. MATERIALS AND METHODS: Lateral feet radiographs in nine children who fulfilled the diagnostic criteria of fibrodysplasia ossificans progressiva were reviewed...
October 2016: Pediatric Radiology
Corey R Hopkins
INTRODUCTION: The bone morphogenetic protein (BMP) is a critical signaling pathway and plays a diverse role in embryonic pattern formation and is implicated in a variety of disease processes, including anemia, bone formation, atherosclerosis, skin diseases, and cancers, among others. AREAS COVERED: This review covers small molecule inhibitors/antagonists of BMP in patent applications between 2008 - 2015, along with brief synopses of the disclosed inhibitors in the primary literature...
August 1, 2016: Expert Opinion on Therapeutic Patents
Qays Ahmed Hassan Al-Timimy, Mohammed Shehab Al-Edani
BACKGROUND: Myositis ossificans is a benign, tumor-like lesion characterized by heterotopic ossification of soft tissue that usually affects the elbow and thigh. At different stages of maturity, it show similar histologic appearances with sarcomatous lesions or maturing bone. Misdiagnosis can result in unnecessary radical treatment. CASE REPORT: A 50-year-old woman with dorsal forefoot soft tissue mass was diagnosed as myositis ossificans after surgical excision and confirmed by a histopathological examination...
2016: International Journal of Surgery Case Reports
Andrea Váncsa, Katalin Dankó
Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans...
July 2016: Orvosi Hetilap
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