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Fibrous dysplasia

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https://www.readbyqxmd.com/read/29599748/polyostotic-fibrous-dysplasia-with-and-without-mccune-albright-syndrome-clinical-features-in-a-nordic-pediatric-cohort
#1
Pauliina Utriainen, Helena Valta, Sigridur Björnsdottir, Outi Mäkitie, Eva Horemuzova
Objective: Fibrous dysplasia (FD) presents as skeletal lesions in which normal bone is replaced by abnormal fibrous tissue due to mosaic GNAS mutation. McCune-Albright syndrome (MAS) refers to FD combined with skin (café-au-lait) and endocrine manifestations. This study describes the clinical childhood manifestations of polyostotic FD and MAS in a Nordic cohort. Patients and design: We retrospectively reviewed a cohort of pediatric patients ( n  = 16) with polyostotic FD with or without MAS diagnosed and followed in two Nordic Pediatric tertiary clinics between 1996 and 2017...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29574980/blockade-of-the-renin-angiotensin-aldosterone-system-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-a-double-blind-multicenter-prospective-randomized-genotype-driven-study-brave-study
#2
Elodie Morel, Ab Waheed Manati, Patrice Nony, Delphine Maucort-Boulch, Francis Bessière, Xu Cai, Timothee Besseyre des Horts, Alexandre Janin, Adrien Moreau, Phillippe Chevalier
Arrhythmogenic right ventricular dysplasia (ARVD) is a rare cardiomyopathy characterized by the progressive replacement of cardiomyocytes by fatty and fibrous tissue in the right ventricle (RV). These infiltrations lead to cardiac electrical instability and ventricular arrhythmia. Current treatment for ARVD is empirical and essentially based on treatment of arrhythmia. Thus, there is no validated treatment that will prevent the deterioration of RV function in patients with ARVD. The aim of the BRAVE study is to evaluate the effect of ramipril, an angiotensin-converting enzyme inhibitor, on ventricular myocardial remodeling and arrhythmia burden in patients with ARVD...
March 25, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29561919/reconstruction-of-frontal-defects-with-calvarial-grafts
#3
S Dova, P D Karkos, J Constantinidis
BACKGROUND: There are many options for the reconstruction of frontal sinus wall defect resulting from injury, neoplastic lesions and aesthetic deformities. These mainly include autogenous grafts, allogenous grafts, and alloplastic materials. The aim of the present study is to report the advantages of using autogenous calvarial bone grafts over other reconstruction techniques, in the reconstruction of frontal defects. METHODS: We describe 16 consecutive cases of anterior frontal sinus defects between 2004 and 2015, in which calvarial bone grafts were used...
March 21, 2018: Rhinology
https://www.readbyqxmd.com/read/29561485/surgical-simulation-and-custom-made-implant-for-cranial-fibrous-dysplasia
#4
Yoshiaki Sakamoto, Tomoru Miwa, Kazunari Yoshida, Kazuo Kishi
BACKGROUND: The management of fronto-orbital fibrous dysplasia involves total excision of the dysplastic bone and bone reconstruction using implants. Custom-made implants are used to achieve good contouring. However, the remnant defect after excising the lesion often does not match the implant. METHODS: To design the craniotomy line, a template was prepared by preoperative computed surgical simulation. The template was overlaid on the preoperative cranium, such that its inner surface fits exactly with the cranial surface...
March 20, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29547687/bilateral-tibial-fibrous-dysplasia-in-a-pediatric-patient-treated-with-intramedullary-nailing
#5
Emanuel Rivera-Rosado, David Beaton-Comulada, Eric Hernandez-Ortiz, Pablo V Marrero-Ortiz
Fibrous dysplasia is a benign developmental disorder of bone in which fibrous connective tissue containing abnormal bone with irregular trabeculae replaces normal cancellous bone. It may affect 1 (monostotic) or multiple bones (polyostotic). Polyostotic disease is the less common of the 2, occurring in only 20 to 25% of fibrous dysplasia patients and tending to affect those who are younger than 10 years of age; patients having this form tend to experience bone enlargement beyond normal skeletal maturation, which can cause pain, progressive damage, and increased risk of pathological fracture...
March 2018: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/29543677/fluorescent-in-situ-hybridization-for-tp53-in-the-diagnosis-of-pediatric-osteogenic-sarcoma
#6
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29534205/bifrontal-craniotomy-for-resection-of-recurrent-fibrous-dysplasia-2-dimensional-operative-video
#7
Hussam Abou-Al-Shaar, William T Couldwell
No abstract text is available yet for this article.
March 8, 2018: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29517538/fibrous-dysplasia-of-the-rib-mimicking-a-malignant-bone-tumor-at-spect-ct-with-99mtc-mdp
#8
Ludovic Fournel, Cristian Rapicetta, Alessandro Fraternali, Salvatore Bellafiore, Massimiliano Paci, Filippo Lococo
We herein report a case of a 43-year-old man with a right 8th-rib bone tumor exhibiting features of malignancy at CT-scan. Considering that a SPECT/CT with Tc-MDP showed solitary intense radio-tracer uptake and the inconclusive results from a fine-needle biopsy, surgical en bloc tumor resection was performed. Pathologic analysis revealed a well-limited benign bone lesion ("fibrous rib dysplasia") composed by a cellular fibrous proliferation. Since benign osseous diseases may present an intense scintigraphic tracer uptake (as in this case), we suggest a certain caution when interpreting the results of SPECT/CT with Tc-MDP in order to avoid misdiagnosis and wrong treatments...
May 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29504223/consensus-guidelines-on-the-use-of-bisphosphonate-therapy-in-children-and-adolescents
#9
Peter J Simm, Andrew Biggin, Margaret R Zacharin, Christine P Rodda, Elaine Tham, Aris Siafarikas, Craig Jefferies, Paul L Hofman, Diane E Jensen, Helen Woodhead, Justin Brown, Benjamin J Wheeler, Denise Brookes, Antony Lafferty, Craig F Munns
Bisphosphonate therapy is the mainstay of pharmacological intervention in young people with skeletal fragility. The evidence of its use in a variety of conditions remains limited despite over three decades of clinical experience. On behalf of the Australasian Paediatric Endocrine Group, this evidence-based consensus guideline presents recommendations and discusses the graded evidence (using the GRADE system) for these recommendations. Primary bone fragility disorders such as osteogenesis imperfecta are considered separately from osteoporosis secondary to other clinical conditions (such as cerebral palsy, Duchenne muscular dystrophy)...
March 2018: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29488004/-the-activating-gnas-mutation-a-survey-of-fibrous-dysplasia-its-associated-syndromes-and-other-skeletal-and-extraskeletal-lesions
#10
REVIEW
H Ostertag, S Glombitza
Fibrous dysplasia of bone is a connatal but not hereditary disease with monostotic or polyostotic manifestations and may be associated either with the extraskeletal disease McCune-Albright syndrome or with myxoma of the skeletal muscle, termed Mazabraud syndrome.The confirmation of recurrent chromosomal aberrations may lead to the conclusion that fibrous dysplasia is a neoplasia rather than a dysplastic skeletal disease.The primary cause of all forms of the described diseases is the activating GNAS mutation, which is detectable in almost all lesions...
February 27, 2018: Der Pathologe
https://www.readbyqxmd.com/read/29485574/locally-aggressive-fibrous-dysplasia-mimicking-malign-calvarial-lesion
#11
Hayri Ogul, Emine Keskin
Fibrous dysplasia is an unusual benign bone tumor. It is divided into 3 groups as monostotic, polyostotic, and craniofacial form. The authors reported an unusual patient with fibrous dysplasia with an aggressive radiologic appearance.
February 26, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29471062/constitutive-stimulatory-g-protein-activity-in-limb-mesenchyme-impairs-bone-growth
#12
Anara Karaca, Vijayram Reddy Malladi, Yan Zhu, Olta Tafaj, Elena Paltrinieri, Joy Y Wu, Qing He, Murat Bastepe
GNAS mutations leading to constitutively active stimulatory G protein alpha-subunit (Gsα) cause different tumors, fibrous dysplasia of bone, and McCune-Albright syndrome, which are typically not associated with short stature. Enhanced signaling of the parathyroid hormone/parathyroid hormone-related peptide receptor, which couples to multiple G proteins including Gsα, leads to short bones with delayed endochondral ossification. It has remained unknown whether constitutive Gsα activity also impairs bone growth...
February 20, 2018: Bone
https://www.readbyqxmd.com/read/29434178/a-case-series-of-fibro-osseous-lesions-of-the-jaws
#13
Masaya Akashi, Kenji Matsuo, Manabu Shigeoka, Yasumasa Kakei, Takumi Hasegawa, Akira Tachibana, Shungo Furudoi, Takahide Komori
The aim of this case series was to reveal the difficulties in diagnosing fibro-osseous lesions with radiological and histopathological examinations and quantify the potential risk of infection to fibro-osseous legions. To analyze the concordance between radiological and histopathological diagnoses, this retrospective case series included patients who were clinically diagnosed with fibro-osseous lesions via radiological findings and excluded the patients who did not undergo histopathological examinations. This study also included the patients in whom histopathological results confirmed fibro-osseous legions when preoperative radiological diagnosis did not include fibro-osseous legions...
December 18, 2017: Kobe Journal of Medical Sciences
https://www.readbyqxmd.com/read/29424156/-particle-impaction-bone-graft-and-plate-internal-fixation-for-the-treatment-of-proximal-femoral-bone-tumor-or-tumor-lesion
#14
Hong-Tao Li, Yong-Zhi Yang, De-Bao Zhang, Gui-Shan Gu
OBJECTIVE: To evaluate the clinical feasibility of particle impaction bone graft and plate internal fixation for the treatment of proximal femoral bone tumors or tumor disease. METHODS: From January 2013 to January 2016 a total of 26 cases of the proximal femur bone tumors or tumor lesions, neither pathological fracture, were retrospectively analyzed, including 12 males and 14 females with an average age of 34.2 years old ranging from 8 to 62 years old. The pathologic result involved fibrous dysplasia in 11 cases, bone isolation bone cyst in 7 cases, giant cell tumors of bone in 3 cases, aneurysm sample bone cyst in 3 cases, non ossifying fibroma in 1 case, benign fibrous histiocytoma in 1 case...
July 25, 2017: Zhongguo Gu Shang, China Journal of Orthopaedics and Traumatology
https://www.readbyqxmd.com/read/29416170/benign-aggressive-lesions-of-femoral-head-and-neck-is-salvage-possible
#15
Yogesh Panchwagh, Sujit K Joshi, Parag K Sancheti
Background: Benign aggressive bone lesions of the femoral head and neck are mostly seen in young adults and warrant treatment for pain, impending fracture or established fracture, and disease clearance. It becomes challenging to treat them effectively while attempting salvage of the femoral head and yet achieving long term disease control with minimum complications. We describe our technique and experience in dealing with these lesions which can achieve the above-mentioned goals and can be easily replicated...
January 2018: Indian Journal of Orthopaedics
https://www.readbyqxmd.com/read/29406819/successful-implant-placement-in-a-case-of-florid-cemento-osseous-dysplasia-a-case-report-and-literature-review
#16
Nasrin Esfahanizadeh, Hila Yousefi
Florid cemento-osseus dysplasia (FCOD) has been described as a reactive process in which normal bone is replaced by fibrous connective tissues and cementum-like materials. Radiographically it appears as dense, lobulated masses, often occurring bilaterally with symmetric involvement. In this case report a successful implant placement has been reported in a 62-year-old Caucasian woman with a chief complaint of mandibular partial edentulous. Radiographic images showed the bilateral radio-opaque lesions in edentulous regions of mandible, and mandibular anterior teeth alike...
February 6, 2018: Journal of Oral Implantology
https://www.readbyqxmd.com/read/29404238/fibro-osseous-lesions-of-the-skull-base-in-the-pediatric-population
#17
REVIEW
Meghan Wilson, Carl Snyderman
Fibro-osseous lesions of the skull base include a variety of lesions with different biologic behavior. The most common lesions include fibrous dysplasia, osteoma, aneurysmal bone cyst, and juvenile ossifying fibroma. The diagnosis can usually be established with radiographic imaging. In the absence of symptoms, slow-growing lesions can often be observed. When surgery is indicated for relief of symptoms, endoscopic endonasal techniques are well suited for all age groups.
February 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29393050/sphenoid-bone-fibrous-dysplasia-detected-incidentally-on-bone-scintigraphy-by-the-contribution-of-spect-ct-hybrid-imaging
#18
Hüseyin Şan, Kürşat Okuyucu, Ali Ozan Öner, Özdeş Emer, Alper Özgür Karaçalıoğlu
Fibrous dysplasia (FD) is a benign fibroosseous bone disorder. It has poliostotic and monostotic patterns. Monostotic FD is frequently asymptomatic and is usually discovered incidentally by radiologic imaging performed for other reasons. Bone scintigraphy is valuable for identifying disease extent. Craniofacial FD (CFD) is a form of the disease where lesions are limited to contiguous bones of the craniofacial skeleton. We presented a case with monostotic CFD who was detected incidentally on bone scintigraphy single-photon emission computed tomography/computerized tomography while being investigated for inflammatory arthropaties...
February 1, 2018: Molecular Imaging and Radionuclide Therapy
https://www.readbyqxmd.com/read/29387305/craniomaxillofacial-fibrous-dysplasia-conservative-treatment-and-maxillary-osteotomy-using-the-schuchardt-kufner-technique
#19
Manlio Galiè, Giulia Carnevali, Giovanni Elia, Massimo Pedriali, Luigi C Clauser
Fibrous dysplasia (FD) is a disturbance of the mesenchymal tissue that accounts for 2.5% of all bone tumors and more than 7% of nonmalignant bone tumors. In the craniomaxillofacial region, FD affects the calvaria, skull base, zygoma, and jaws, the prevalent site being the maxilla (50% of cases). Therapy for craniomaxillofacial FD is surgical. The goals of surgery are to prevent functional disorders and restore facial symmetry, volume, and contour. In this article, we present a case of a young female patient affected by right orbital-zygomatic-maxillary FD...
March 2018: Craniomaxillofacial Trauma & Reconstruction
https://www.readbyqxmd.com/read/29354163/secondary-aneurysmal-bone-cyst-in-mccune-albright-syndrome
#20
Symeon Tournis, Alexia Balanika, Panayiotis D Megaloikonomos, Andreas F Mavrogenis
Polyostotic fibrous dysplasia in combination with caféau-lait macules and hyperfunctioning endocrinopathies consists of a rare clinical condition termed as McCune-Albright syndrome. Aneurysmal bone cysts are tumor-like cystic lesions, composed of blood-filled compartments. They may occur as primary lesions or secondary to other pathologies; most commonly giant cell tumors of bone. However, secondary aneurysmal bone cysts in McCune-Albright syndrome are exceptional. We present a 28-year-old female with McCune-Albright syndrome...
September 2017: Clinical Cases in Mineral and Bone Metabolism
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