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Fibrous dysplasia

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https://www.readbyqxmd.com/read/29218289/craniofacial-fibrous-dysplasia-of-zygomaticomaxillary-complex
#1
Kumar Nilesh, Prashant Punde, M I Parkar
Fibrous dysplasia is a benign bone disease first described by Lichtenstein in 1938. It is characterized by progressive replacement of normal bone with fibro-osseous connective tissue. When the disease involves craniofacial skeleton, it results in significant disfigurement and other functional problems. This paper reports a case of large craniofacial fibrous dysplasia involving zygomaticomaxillary complex in a 24-year old male patient. Clinical presentation and imaging characteristics of the pathology is discussed in detail...
September 2017: World Journal of Plastic Surgery
https://www.readbyqxmd.com/read/29192304/association-of-hearing-loss-and-otologic-outcomes-with-fibrous-dysplasia
#2
Alison M Boyce, Carmen Brewer, Timothy R DeKlotz, Christopher K Zalewski, Kelly A King, Michael T Collins, H Jeffrey Kim
Importance: Fibrous dysplasia (FD) and McCune-Albright syndrome (MAS) are rare bone and endocrine disorders in which expansile fibro-osseous lesions result in deformity, pain, and functional impairment. The effect of FD on hearing and otologic function has not been established. Objectives: To characterize audiologic and otologic manifestations in a large cohort of individuals with FD/MAS and to investigate potential mechanisms of hearing loss. Design, Setting, and Participants: In this natural history study, individuals with craniofacial FD seen at a clinical research center underwent clinical, biochemical, computed tomographic, audiologic, and otolaryngologic evaluations...
November 30, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/29188758/utility-of-intraoperative-computed-tomography-for-cochlear-implantation-in-patients-with-difficult-anatomy
#3
Christine S Kim, Alice Z Maxfield, David Foyt, Robert J Rapoport
OBJECTIVE AND IMPORTANCE: To describe cases that illustrate the utility of intraoperative computed tomography (CT) in cochlear implantation of patients with difficult temporal bone anatomy. CLINICAL PRESENTATION: A 2-year-old male with congenital X-linked stapes gusher syndrome and a 2-year-old female with enlarged vestibular aqueduct underwent successful cochlear implantation with the help of intraoperative CT. In the latter case, the initial intraoperative C-arm fluoroscopy suggested malposition of the electrode, however, was not able to provide details for adjustments...
November 30, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/29187205/combined-double-chambered-right-ventricle-tricuspid-valve-dysplasia-ventricular-septal-defect-and-subaortic-stenosis-in-a-dog
#4
Iuliu Scurtu, Flaviu Tabaran, Mircea Mircean, Gavril Giurgiu, Andras Nagy, Cornel Catoi, Dan G Ohad
BACKGROUND: Double chambered right ventricle (DCRV) is a congenital heart anomaly where the right ventricle is divided into two chambers. We describe, for the first time, an unusual combination of DCRV combined with some other congenital heart defects. CASE PRESENTATION: A 1.2-year-old Golden Retriever was presented with lethargy, exercise intolerance and ascites. Physical examination revealed an irregularly irregular pulse and a grade V/VI, systolic, right cranial murmur...
November 29, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/29184807/zoledronic-acid-in-pediatric-metabolic-bone-disorders
#5
REVIEW
Sasigarn A Bowden, John D Mahan
Zoledronic acid (ZA), a highly potent intravenous bisphosphonate (BP), has been increasingly used in children with primary and secondary osteoporosis due to its convenience of shorter infusion time and less frequent dosing compared to pamidronate. Many studies have also demonstrated beneficial effects of ZA in other conditions such as hypercalcemia of malignancy, fibrous dysplasia (FD), chemotherapy-related osteonecrosis (ON) and metastatic bone disease. This review summarizes pharmacologic properties, mechanism of action, dosing regimen, and therapeutic outcomes of ZA in a variety of metabolic bone disorders in children...
October 2017: Translational pediatrics
https://www.readbyqxmd.com/read/29184270/recurrent-psammomatoid-juvenile-ossifying-fibroma-with-aneurysmal-bone-cyst-an-unusual-case-presentation
#6
Swati S Gotmare, Avinash Tamgadge, Sandhya Tamgadge, Kashmira S Kesarkar
Juvenile ossifying fibroma (JOF) is a rare, benign, locally aggressive entity of the extragnathic craniofacial bones with a high tendency towards recurrence. Two distinctive microscopic patterns of juvenile ossifying fibroma have been described: a trabecular juvenile ossifying fibroma (TrJOF) and a psammomatoid juvenile ossifying fibroma (PJOF). Psammomatoid variant is predominantly a craniofacial lesion and occurs rarely in the jaws. The pathognomonic histopathologic feature is the presence of spherical ossicles, which are similar to psammoma bodies...
November 2017: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29165438/our-experience-in-the-surgical-management-of-craniofacial-fibrous-dysplasia-what-has-changed-in-the-last-10-years
#7
V Valentini, A Cassoni, V Terenzi, M Della Monaca, M T Fadda, O Rajabtork Zadeh, I Raponi, A Anelli, G Iannetti
The mainstay of treatment of craniofacial dysplasia (CFD) remains surgery once clinical observation has been excluded. Nevertheless, disagreement remains about the type of surgical intervention (remodelling versus radical resection). The aim of this paper is to present our experience until 2013 comparing CFD management between 1980 and 2002 and between 2003 and 2013 and to propose our surgical algorithm. From January 2003 to December 2013, 41 new patients (18 males and 23 females) with histologically demonstrated CFD presented to our Department...
October 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/29162285/-proptosis-as-presenting-sign-of-craniofacial-fibrous-dysplasia
#8
S Azennoud, R Arouagh, S Harchali, S El Merrouni, M Zarrouki, M El Yadari, F El Ibrahimi, O Bahia, A El Hassan, A Berraho
No abstract text is available yet for this article.
November 18, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29158412/induced-gnas-r201h-expression-from-the-endogenous-gnas-locus-causes-fibrous-dysplasia-by-up-regulating-wnt-%C3%AE-catenin-signaling
#9
Sanjoy Kumar Khan, Prem Swaroop Yadav, Gene Elliott, Dorothy Zhang Hu, Ruoshi Xu, Yingzi Yang
Fibrous dysplasia (FD; Online Mendelian Inheritance in Man no. 174800) is a crippling skeletal disease caused by activating mutations of the GNAS gene, which encodes the stimulatory G protein Gαs FD can lead to severe adverse conditions such as bone deformity, fracture, and severe pain, leading to functional impairment and wheelchair confinement. So far there is no cure, as the underlying molecular and cellular mechanisms remain largely unknown and the lack of appropriate animal models has severely hampered FD research...
November 20, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29157551/fibro-osseous-and-other-lesions-of-bone-in-the-jaws
#10
REVIEW
Mansur Ahmad, Laurence Gaalaas
Fibroosseous lesions in the jaws have similar histologic and radiographic features. Despite their similarity, management varies significantly. In this article, common fibroosseous lesions and key radiographic features are described. Many of the fibroosseous lesions are diagnosed radiographically, without performing histologic examinations. For some of the fibroosseous lesions, for example, periapical osseous dysplasia, histologic examination is contraindicated. Cherubism and fibrous dysplasia have specific radiographic findings; these conditions can be diagnosed radiographically...
January 2018: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/29125822/liposclerosing-myxofibrous-tumor-a-rare-tumor-of-proximal-femur
#11
Ozan Beytemür, Ümit Seza Tetikkurt, Cem Albay, Gonca Kavşut, Akif Güleç
Liposclerosing myxofibrous tumor is a very rarely observed benign fibro-osseous lesion, which contains complex histological structures. It was first described in the year 1986. Histopathological examination of the lesion may show myxoid areas, osteoclastic activity, bone trabeculae similar to fibrous dysplasia, fat necrosis, ischemic ossification and rarely cartilage components. This lesion, which is particularly localized to the proximal femur, is thought to be associated with fibrous dysplasia. In this case report, we aim to present a liposclerosing myxofibrous tumor case, which was treated surgically, in light of the literature review...
December 2017: Eklem Hastalıkları Ve Cerrahisi, Joint Diseases & related Surgery
https://www.readbyqxmd.com/read/29123414/low-grade-central-osteosarcoma-in-proximal-humerus-a-rare-entity
#12
Fan Tang, Li Min, Yong Zhou, Yi Luo, Chongqi Tu
Low-grade central osteosarcoma is a rare subtype of tumor with low-grade malignancy. Currently, wide resection with negative resection margin is the standard treatment for this disease. The role of neoadjuvant chemotherapy in low-grade central osteosarcoma was controversial and was mostly considered for tumors containing high-grade focal areas. Local tumor recurrences often exhibited a tumor with higher histologic grade or differentiation with the potential for metastases. In low-grade central osteosarcoma, timely wide resection after definite diagnosis can result in 5-year survival for almost 90%...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29109141/teaching-neuroimages-craniofacial-fibrous-dysplasia-loss-of-vision-after-head-trauma
#13
Fikret Sarihan, Kristel M Kasius
No abstract text is available yet for this article.
November 7, 2017: Neurology
https://www.readbyqxmd.com/read/29104223/detection-of-rare-somatic-gnas-mutation-in-mccune-albright-syndrome-using-a-novel-peptide-nucleic-acid-probe-in-a-single-tube
#14
Fu-Sung Lo, Tai-Long Chen, Chiuan-Chian Chiou
McCune-Albright syndrome (MAS) is characterized by the triad of precocious puberty, café au lait pigmentation, and polyostotic fibrous dysplasia (FD) of bone, and is caused by post-zygotic somatic mutations-R201H or R201C-in the guanine nucleotide binding protein, alpha stimulating (GNAS) gene. In the present study, a novel peptide nucleic acid (PNA) probe with fluorescent labeling was designed to detect trace amounts of somatic mutant GNAS in a single tube reaction. The method was applied to screen GNAS mutations in six patients with MAS/FD...
November 1, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29095483/homozygous-mutation-in-elmo2-may-cause-ramon-syndrome
#15
C Mehawej, A Hoischen, R A Farah, I Marey, M David, S Stora, K Lachlan, H G Brunner, A Mégarbané
We report on a girl, born to first cousin Lebanese parents, with intellectual disability, seizures, repeated gingivorrhagia, enlarged lower and upper jaws, overgrowth of the gums, high arched and narrow palate, crowded teeth, hirsutism of the back, large abdomen and a small umbilical hernia. Cysts of the mandible, fibrous dysplasia of bones, and enlarged adenoids causing around 60% narrowing of the nasopharyngeal airways were noted at radiographic examination. Her brother presented with the same features in addition to a short stature, an ostium secundum, and more pronounced intellectual disability...
November 2, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29074445/fibrous-condylar-dysplasia-resection-and-reconstruction-with-a-custom-made-tmj-prosthesis-using-virtual-surgical-planning
#16
M Fernández-Ferro, J Fernández-Sanromán, A Costas-López, A López-Betancourt
The main objective of this article is to describe the indications for custom-made alloplastic reconstruction of the temporomandibular joint (TMJ) using the new techniques of virtual surgical planning and computer-aided design/computer-aided manufacturing (CAD/CAM) technology through the study of a clinical case and an updated review of the literature. The patient was a 45-year-old male with a fast-growing condylar dysplasia that produced a progressive facial deformity with limited mandibular mobility, which required a radical resection...
October 23, 2017: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29071359/bone-marrow-failure-and-extramedullary-hematopoiesis-in-mccune-albright-syndrome
#17
C Robinson, A M Boyce, A Estrada, D E Kleiner, R Mathew, R Stanton, H Frangoul, M T Collins
In fibrous dysplasia/McCune-Albright syndrome (FD/MAS), bone and bone marrow are, to varying degrees, replaced by fibro-osseous tissue typically devoid of hematopoietic marrow. Despite the extensive marrow replacement in severely affected patients, bone marrow failure is not commonly associated with FD/MAS. We present a 14-year-old girl with FD/MAS, who developed pancytopenia and extramedullary hematopoiesis (EMH) with no identified cause, in the setting of iatrogenic thyrotoxicosis and hyperparathyroidism...
October 25, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/29053652/a-report-on-the-clinical-pathological-correlations-of-788-gingival-lesion
#18
A Gambino, M Carbone, R Broccoletti, P Carcieri, D Conrotto, M Carrozzo, P-G Arduino
BACKGROUND: The diagnosis and treatment of a variety of non-plaque related gingival diseases have become an integrated aspect of everyday dentistry. The aim of this study was to analyse the relationship between clinical appearance and histopathological features of gingival lesions in a large Northern Italian population. MATERIAL AND METHODS: A retrospective study of 788 cases of gingival and alveolar mucosal biopsies was set up. Statistical analysis was performed by calculating the odds ratio and 95% confidence interval (C...
November 1, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
https://www.readbyqxmd.com/read/29038079/optical-coherence-tomography-in-the-management-of-skull-base-fibrous-dysplasia-with-optic-nerve-involvement
#19
Joshua Loewenstern, Christopher M Hernandez, Carolyn Chadwick, Amish Doshi, Rudrani Banik, Christopher A Sarkiss, Joshua Bederson, Raj K Shrivastava
BACKGROUND: Fibrous dysplasia (FD) of the skull base can present with optic nerve compression. As most patients initially do not experience vision loss, controversy exists whether to proceed with prophylactic surgical decompression or elect for conservative observation. Optical coherence tomography (OCT), a physiologic imaging modality widely used to assess the condition of the retinal nerve fiber layer (RNFL), has been useful in monitoring compressive tumors on the optic nerve. The present study evaluated the potential use of OCT in the management of FD patients with optic nerve involvement...
October 13, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29022055/illness-perceptions-are-associated-with-quality-of-life-in-patients-with-fibrous-dysplasia
#20
B C J Majoor, C D Andela, C R Quispel, M Rotman, P D S Dijkstra, N A T Hamdy, A A Kaptein, N M Appelman-Dijkstra
Fibrous dysplasia (FD) is a rare bone disorder in which normal bone is replaced by fibrous tissue resulting in pain, deformities, pathological fractures or asymptomatic disease. Illness perceptions are patients' cognitions and emotions about their illness and its treatment, which may impact on Quality of Life (QoL). Here, we explore illness perceptions in patients with FD compared to other disorders, identify factors associated with illness perceptions and evaluate their relationship with QoL. Ninety-seven out of 138 eligible patients from the LUMC FD cohort completed the Illness Perception Questionnaire-Revised (IPQ-R) and the Short Form-36 (SF-36)...
October 11, 2017: Calcified Tissue International
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