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Craniofacial fibrous dysplasia

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https://www.readbyqxmd.com/read/28468170/might-trauma-be-a-triggering-factor-for-craniofacial-fibrous-dysplasia
#1
Mehmet Onur Yüksel, Mehmet Sabri Gürbüz, Hilmi Onder Okay, Mehmet Esref Kabalar
Fibrous dysplasia (FD) is a rare, benign disease of unclear etiology where normal bone is replaced with abnormal fibrous and weak osseous tissue. Any bone of the skeleton might be involved but skull is one of the most commonly affected sites. Fibrous dysplasia is known to be caused by a genetic mutation leading to inappropriate proliferation and differentiation of osteoblastic cells. However; it is not known whether any triggering factor exists which might contribute to this genetic mutation. The authors postulated that trauma might be a triggering factor for this disease...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28302454/craniofacial-fibrous-dysplasia-a-10-case-series
#2
A Couturier, O Aumaître, L Gilain, B Jean, T Mom, M André
OBJECTIVES: Fibrous dysplasia of bone is a rare sporadic benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. Sarcomatous transformation is exceptional. Lesions may involve one bone (monostotic) or several (polyostotic). Fibrous dysplasia may be associated with café-au-lait skin macules and endocrinopathy in McCune-Albright syndrome, or with myxoma in Mazabraud's syndrome. METHODS: We report ten cases of patients followed up for craniofacial fibrous dysplasia in our center between 2010 and 2015...
March 14, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28228794/prompt-clinical-and-biochemical-response-to-denosumab-in-a-young-adult-patient-with-craniofacial-fibrous-dysplasia
#3
Cristina Eller-Vainicher, Diego Sergio Rossi, Giuseppe Guglielmi, Giada Anna Beltramini, Elisa Cairoli, Antonio Russillo, Giovanna Mantovani, Anna Spada, Iacopo Chiodini
BACKGROUND: We report on the clinical and biochemical outcomes in a 20-year-old male suffering from active craniofacial monostotic fibrous dysplasia (MFD) of the left mandible treated with the RANK-L inhibitor, denosumab, following unsatisfactory responses to prior long-term bisphosphonates therapy. RESULTS: The patient had been treated over 9 years with pamidronate (cumulative dose of 810 mg) with incomplete control of pain. Following initiation of denosumab 60 mg subcutaneously, bone pain and bone turnover markers (osteocalcin, total and bone alkaline phosphatase and carboxy-terminal cross-linking telopeptide of type I collagen) were monitored over a 27 months period...
September 2016: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/28228793/craniofacial-fibrous-dysplasia-report-of-a-case-with-diverse-radiological-spectrum
#4
Silky Rajesh Punyani, Saurabh Srivastava, Vishal Ramesh Jasuja
A young male of Asian-Indian ethnicity reported with a complaint of a painless, slow growing swelling over the left side of lower jaw. A thorough clinical history was taken and detailed radiological exam performed. The conventional radiographic examination revealed a mixed radiolucent-radiopaque lesion with unique appearances on different radiographs. Additional computed tomographic examination discovered the involvement of several bones in the skull base. Subsequent to histopathological confirmation a final diagnosis of craniofacial fibrous dysplasia was made...
September 2016: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/28118187/might-trauma-be-a-triggering-factor-for-craniofacial-fibrous-dysplasia
#5
Mehmet Onur Yüksel, Mehmet Sabri Gürbüz, Hilmi Onder Okay, Mehmet Esref Kabalar
Fibrous dysplasia (FD) is a rare, benign disease of unclear etiology where normal bone is replaced with abnormal fibrous and weak osseous tissue. Any bone of the skeleton might be involved but skull is one of the most commonly affected sites. Fibrous dysplasia is known to be caused by a genetic mutation leading to inappropriate proliferation and differentiation of osteoblastic cells. However; it is not known whether any triggering factor exists which might contribute to this genetic mutation. The authors postulated that trauma might be a triggering factor for this disease...
January 23, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28050513/an-atypical-presentation-of-multiple-central-osteomas-mimicking-craniofacial-fibrous-dysplasia-a-pictorial-essay
#6
Rashmi Kewal Agarwal, Amit A Mhapuskar, Manjula Hebbale, Meenal Tepan, Ayushee
Osteoma is benign neoplasm with slow growth characterized by deposition of compact lamellar cortical or cancellous bone creating a tumour mass. It is still unclear whether osteomas are benign neoplasms or hamartomas. They have typical clinical presentations and are easily diagnosed with the help of radiographs. We present a rare case of non-syndromic multiple osteomas in the craniofacial region which are typically restricted to the midline and presents radiographically as craniofacial fibrous dysplasia causing a diagnostic dilemma...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28007843/clinical-characteristics-and-management-of-growth-hormone-excess-in-patients-with-mccune-albright-syndrome
#7
Yong Yao, Yang Liu, Linjie Wang, Kan Deng, Hongbo Yang, Lin Lu, Feng Feng, Bing Xing, Hui You, Zimeng Jin, Renzhi Wang, Hui Pan, Shi Chen, Huijuan Zhu
OBJECTIVE: McCune-Albright syndrome (MAS) is a sporadic, postzygotic disease presenting with fibrous dysplasia, cafe-au-lait spots and multiple endocrinopathies. Growth hormone (GH) excess is an uncommon but potentially severe complication of MAS. This study aims to describe the clinical manifestations of GH excess in the context of MAS and analyze the responses of these patients to treatments. DESIGN: Retrospective clinical study. METHODS: Clinical data from 52 MAS patients were analyzed...
March 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28005795/orbitocranial-fibrous-dysplasia-outcome-of-radical-resection-and-immediate-reconstruction-with-titanium-mesh-and-pericranial-flap
#8
Khalid Nasser Fadle, Ahmed Gaber Hassanein, Abdin K Kasim
INTRODUCTION: Fibrous dysplasia (FD) is a non-neoplastic developmental fibro-osseous disease. It represents 2.5% of all bone tumors and 5% to 7% of the benign bone tumors. Orbitocranial region is involved in about 20% of the patients. The main presentations are craniofacial deformity and headache. Loss of vision is the most devastating result of this disease. There is no medical treatment to cure or prevent FD. Radiation therapy is contraindicated. Surgery for the orbitocranial FD is often challenging because of the proximity of neurovascular and ocular structures...
November 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27922965/prevalence-of-different-forms-and-involved-bones-of-craniofacial-fibrous-dysplasia
#9
Liya Yang, Huanhuan Wu, Jianjian Lu, Li Teng
BACKGROUND: The purposes of this study were to analyze the prevalence of various craniofacial bones involved in patients with craniofacial fibrous dysplasia (CFD) and to demonstrate the most common form and bone involvement in patients with CFD for surgeons. METHODS: To address the research purpose, the authors designed and performed a systematic review with meta-analysis. A comprehensive electronic search without date was performed in August 2013. Data extracted from the previously published literature were analyzed with STATA 11...
January 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27922893/craniofacial-fibrous-dysplasia-retrospective-study-on-the-relationship-between-the-tumor-volume-changes-and-the-circulating-serum-calcitonin-and-serum-alkaline-phosphatase
#10
Mohammed Ahmed Hussein, In Sik Yun, Bo Ok Kim, Yong Oock Kim
BACKGROUND: The purpose of this study was to determine the usefulness of serum alkaline phosphatase (ALP) and calcitonin in the follow-up of tumor volume changes in patients with craniofacial fibrous dysplasia. METHODS: Twenty patients with isolated craniofacial fibrous dysplasia were included, who met our criteria for long-term follow-up. Three-dimensional computed tomography scans were obtained, and tumor segmentation was performed. The tumor volume was measured preoperatively, immediate postoperative and during long-term follow-up...
March 2017: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/27857528/mccune-albright-syndrome-association-of-fibrous-dysplasia-caf%C3%A3-au-lait-skin-spots-and-hyperthyroidism-case-report
#11
Iulian Raus, Roxana Elena Coroiu
McCune-Albright syndrome is a rare sporadic disease characterized by bone fibrous dysplasia, café-au-lait skin spots and a variable association of hyperfunctional endocrine disorders. Fibrous dysplasia (FD), which can involve the craniofacial, axial, and appendicular skeleton, may range from an isolated, asymptomatic monostotic lesion to a severe disabling polyostotic disease involving the entire skeleton. A twenty-five-year old male patient presented to our clinic with recently developed heart palpitations...
2016: Clujul Medical (1957)
https://www.readbyqxmd.com/read/27747122/use-of-zoledronic-acid-in-paediatric-craniofacial-fibrous-dysplasia
#12
Chiara Di Pede, Sabrina Congedi, Sara Rossin, Antuan Divisic, Alesandra De Gregorio, Caterina Agosto, Igor Catalano, Alessandro Mazza, Leonardo Sartori, Stefano Masiero, Franca Benini
We describe a case of a paediatric patient affected by mandibular fibrous dysplasia (FD) with severe and chronic pain who was successfully treated with zoledronic acid (ZOL): a third-generation bisphosphonate. Further research is needed to assess its safety and efficacy as a treatment option for FD in the paediatric population.
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27702404/-fibrous-dysplasia-a-heterogeneous-disease
#13
B C J Majoor, N M Appelman-Dijkstra, M A J van de Sande, H M Kroon, N A T Hamdy, P D S Dijkstra
Fibrous dysplasia is a rare genetic bone disorder with a wide variation in clinical expression, ranging from asymptomatic patients to severely affected patients with extensive bone disease, pain, repetitive fractures and deformities and serious endocrinological symptoms (McCune-Albright syndrome). Here, we report on three different cases of fibrous dysplasia. First, a 46-year-old woman with a small solitary lesion in the proximal femur. Second, a 25-year-old man with polyostotic disease of the left leg who received both surgical and medical treatment...
2016: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/27649526/outcome-of-long-term-bisphosphonate-therapy-in-mccune-albright-syndrome-and-polyostotic-fibrous-dysplasia
#14
Bas Cj Majoor, Natasha M Appelman-Dijkstra, Martha Fiocco, Michiel Aj van de Sande, Pd Sander Dijkstra, Neveen At Hamdy
McCune-Albright syndrome (MAS) is a rare bone disorder characterized by fibrous dysplasia (FD), endocrinopathies, and café-au-lait patches. FD patients have been shown to respond favorably to treatment with bisphosphonates, but data are scarce in the more severe polyostotic form (PFD), including MAS, and factors determining treatment outcome are not known, particularly in the long-term. We evaluated the biochemical (bone turnover markers [BTMs]) and clinical (pain reduction) outcome of bisphosphonate therapy in 11 patients with MAS and 30 patients with PFD: median duration of treatment 6 years (range, 2 to 25 years)...
February 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/27617807/orbitocranial-fibrous-dysplasia-outcome-of-radical-resection-and-immediate-reconstruction-with-titanium-mesh-and-pericranial-flap
#15
Khalid Nasser Fadle, Ahmed Gaber Hassanein, Abdin K Kasim
INTRODUCTION: Fibrous dysplasia (FD) is a non-neoplastic developmental fibro-osseous disease. It represents 2.5% of all bone tumors and 5% to 7% of the benign bone tumors. Orbitocranial region is involved in about 20% of the patients. The main presentations are craniofacial deformity and headache. Loss of vision is the most devastating result of this disease. There is no medical treatment to cure or prevent FD. Radiation therapy is contraindicated. Surgery for the orbitocranial FD is often challenging because of the proximity of neurovascular and ocular structures...
September 9, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27513769/three-dimensional-printing-of-reduction-template-in-the-contouring-of-craniofacial-fibrous-dysplasia
#16
Ruichen Wang, Guizhen Li, Chunming Liu, Chiyu Jia, Yan Han
BACKGROUND: Contouring is a minimal invasive procedure to remove excess lesions and restore facial appearance of patients with craniofacial fibrous dysplasia; however, it is difficult to measure the amount of excess lesions. This study is to demonstrate the use of reduction template produced by three-dimensional printer in contouring procedure. METHODS: Computed tomography data were reconstructed into a three-dimensional model by Mimics software. The unaffected side was mirrored and superimposed on the affected side...
October 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27493082/fibrous-dysplasia-of-bone-craniofacial-and-dental-implications
#17
A B Burke, M T Collins, A M Boyce
Fibrous dysplasia (FD) is a rare bone disease caused by postzygotic somatic activating mutations in the GNAS gene, which lead to constitutive activation of adenylyl cyclase and elevated levels of cyclic AMP, which act on downstream signaling pathways and cause normal bone to be replaced with fibrous tissue and abnormal (woven) bone. The bone disease may occur in one bone (monostotic), multiple bones (polyostotic), or in combination with hyperfunctioning endocrinopathies and hyperpigmented skin lesions (in the setting of McCune-Albright Syndrome)...
August 5, 2016: Oral Diseases
https://www.readbyqxmd.com/read/27405032/juvenile-psammomatoid-ossifying-fibroma-findings-on-bone-scan
#18
Rodrigo Cárdenas-Perilla, Consuelo Santamaria, Juan Manuel Muñoz-Acosta
Juvenile psammomatoid ossifying fibroma is a rare bone-forming tumor seen in craniofacial bones, which affects mainly young patients. We report scintigraphic and SPECT/CT findings of 2 patients diagnosed with this disease. One patient presented with suspicion of fibrous dysplasia and the other with suspected malignancy in the setting of a rapidly growing mass. Both cases highlight the importance of recognizing this type of tumor in young patients with abnormal uptake in paranasal bones and sinuses within the range of potential differential diagnoses including sarcomas, fibrous dysplasia, and odontogenic tumors...
September 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27219238/surgical-management-of-polyostotic-craniofacial-fibrous-dysplasia-long-term-outcomes-and-predictors-for-postoperative-regrowth
#19
Alison M Boyce, Andrea Burke, Carolee Cutler Peck, Craig R DuFresne, Janice S Lee, Michael T Collins
BACKGROUND: The mainstay of treatment for craniofacial fibrous dysplasia is surgical; however, optimal indications and techniques are poorly understood, particularly in polyostotic disease and McCune-Albright syndrome. This study investigated surgical indications and risk factors for recurrence in a large cohort. METHODS: One hundred thirty-three craniofacial fibrous dysplasia subjects in a natural history study were evaluated. Radiographic studies, operative reports, and clinical records were reviewed...
June 2016: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/27057395/mccune-albright-syndrome-with-craniofacial-dysplasia-clinical-review-and-surgical-management
#20
Telmo Augusto Barba Belsuzarri, João Flavio Mattos Araujo, Carlos Alberto Morassi Melro, Maick Willen Fernandes Neves, Juliano Nery Navarro, Leandro Gomes Brito, Luis Otavio Carneiro Pontelli, Luis Gustavo de Abreu Mattos, Tiago Fernandes Gonçales, Wolnei Marques Zeviani
BACKGROUND: Fibrous dysplasia (FD) is a benign fibro-osseous lesion related to an abnormal bone development and replacement by fibrous tissue. FD has three clinical patterns namely monostotic, polyostotic, and the McCune-Albright syndrome (MAS). MAS is a rare genetic disorder (about 3% of all FD's) that comprises a triad of polyostotic FD, café-au-lait skin macules, and precocious puberty. MAS can involve the orbit region and cause stenosis in the optic canal, leading the patient to a progressive visual loss...
2016: Surgical Neurology International
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