Database : MEDLINE
Search on : Fibrous and Dysplasia and Of and Bone [Words]
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[PMID]: 29471062
[Au] Autor:Karaca A; Malladi VR; Zhu Y; Tafaj O; Paltrinieri E; Wu JY; He Q; Bastepe M
[Ad] Address:Endocrine Unit, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA.
[Ti] Title:Constitutive stimulatory G protein activity in limb mesenchyme impairs bone growth.
[So] Source:Bone;110:230-237, 2018 Feb 20.
[Is] ISSN:1873-2763
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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. Here we generated mice expressing a constitutively active Gsα mutant (Gsα-R201H) conditionally upon Cre recombinase (cGsα mice). Gsα-R201H was expressed in cultured bone marrow stromal cells from cGsα mice upon adenoviral-Cre transduction. When crossed with mice in which Cre is expressed in a tamoxifen-regulatable fashion (CAGGCre-ER™), tamoxifen injection resulted in mosaic expression of the transgene in double mutant offspring. We then crossed the cGsα mice with Prx1-Cre mice, in which Cre is expressed in early limb-bud mesenchyme. The double mutant offspring displayed short limbs at birth, with narrow hypertrophic chondrocyte zones in growth plates and delayed formation of secondary ossification center. Consistent with enhanced Gsα signaling, bone marrow stromal cells from these mice demonstrated increased levels of c-fos mRNA. Our findings indicate that constitutive Gsα activity during limb development disrupts endochondral ossification and bone growth. Given that Gsα haploinsufficiency also leads to short bones, as in patients with Albright's hereditary osteodystrophy, these results suggest that a tight control of Gsα activity is essential for normal growth plate physiology.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:Publisher

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[PMID]: 29517538
[Au] Autor:Fournel L; Rapicetta C; Fraternali A; Bellafiore S; Paci M; Lococo F
[Ti] Title:Fibrous Dysplasia of the Rib Mimicking a Malignant Bone Tumor at SPECT/CT with 99mTc-MDP.
[So] Source:Clin Nucl Med;, 2018 Mar 07.
[Is] ISSN:1536-0229
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1097/RLU.0000000000002015

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[PMID]: 29504223
[Au] Autor:Simm PJ; Biggin A; Zacharin MR; Rodda CP; Tham E; Siafarikas A; Jefferies C; Hofman PL; Jensen DE; Woodhead H; Brown J; Wheeler BJ; Brookes D; Lafferty A; Munns CF; APEG Bone Mineral Working Group
[Ad] Address:Department of Endocrinology and Diabetes, Royal Children's Hospital, Melbourne, Victoria, Australia.
[Ti] Title:Consensus guidelines on the use of bisphosphonate therapy in children and adolescents.
[So] Source:J Paediatr Child Health;54(3):223-233, 2018 Mar.
[Is] ISSN:1440-1754
[Cp] Country of publication:Australia
[La] Language:eng
[Ab] Abstract: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). The use of bisphosphonates in non-fragility conditions, such as fibrous dysplasia, avascular necrosis, bone cysts and hypercalcaemia, is also discussed. While these guidelines provide an evidence-based approach where possible, further research is required in all clinical applications in order to strengthen the recommendations made.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180305
[Lr] Last revision date:180305
[St] Status:In-Data-Review
[do] DOI:10.1111/jpc.13768

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[PMID]: 29434178
[Au] Autor:Akashi M; Matsuo K; Shigeoka M; Kakei Y; Hasegawa T; Tachibana A; Furudoi S; Komori T
[Ad] Address:Department of Oral and Maxillofacial Surgery, Kobe University Graduate School of Medicine, Kobe, Japan.
[Ti] Title:A Case Series of Fibro-Osseous Lesions of the Jaws.
[So] Source:Kobe J Med Sci;63(3):E73-E79, 2017 Dec 18.
[Is] ISSN:1883-0498
[Cp] Country of publication:Japan
[La] Language:eng
[Ab] Abstract: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. Eleven patients (three men, eight women; median age 24.5 years, range 15-57 years) were enrolled. Although radiological diagnoses of fibrous dysplasia (FD) corresponded with histopathological diagnoses in seven patients, mismatches between radiological findings and histopathological results were found in three patients. In one patient, suspected diagnosis with radiological examinations was malignant lymphoma or FD. In two patients, the histopathological differentiation between FD and ossifying fibroma (OF) was difficult. One patient had lesion recurrence which was suspected to be OF with surgical findings and postoperative course after the initial surgery. In three patients, infections of FD were found. Preoperative diagnosis of OF with radiographic feature of unilocular radiolucency is difficult. In cases in which histopathological differentiation between FD and OF is difficult, operative findings should be used because OF is often found to be well-encapsulated and easily enucleated. Bone in FD showing mixed radiolucent-radiopaque may be vulnerable to infection.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180301
[Lr] Last revision date:180301
[St] Status:In-Process

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[PMID]: 28449700
[Au] Autor:Majoor BCJ; Andela CD; Bruggemann J; van de Sande MAJ; Kaptein AA; Hamdy NAT; Dijkstra PDS; Appelman-Dijkstra NM
[Ad] Address:Department of Orthopaedic Surgery, Center for Bone Quality Leiden University Medical Center, Albinusdreef 2, Postzone J11, PO Box 9600, 2300 RC, Leiden, The Netherlands. b.c.j.majoor@lumc.nl.
[Ti] Title:Determinants of impaired quality of life in patients with fibrous dysplasia.
[So] Source:Orphanet J Rare Dis;12(1):80, 2017 Apr 27.
[Is] ISSN:1750-1172
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life. In this study we evaluate quality of life in patients with fibrous dysplasia using the Short Form-36 and the Brief Pain Inventory questionnaires. Data were compared with those of the general Dutch population. RESULTS: Out of 138 patients from a cohort of 255 patients with fibrous dysplasia that were sent questionnaires assessing quality of life and pain, the response rate was 70.3%, with 97 patients, predominantly female (65%), completing the questionnaires. Monostotic fibrous dysplasia was predominant (n = 62, 64%). Fibrous dysplasia patients had significantly lower quality of life outcome scores than the general Dutch population for all tested domains of the Short Form-36 except for the "Mental health" and the "Role emotional" domains. More severe forms of fibrous dysplasia, had the more severe Short-Form-36 quality of life outcomes, but there was no significant difference in Brief Pain Inventory domains between different subtypes of fibrous dysplasia. Quality of life was lower in patients with higher disease burden, as reflected by high skeletal burden scores (p = 0.003) and high levels of P1NP (p = 0.002). CONCLUSION: We demonstrate impairments in all domains of quality of life, except for 'Mental health' and 'Role emotional' domains, across the wide spectrum of fibrous dysplasia including its milder forms. We identified high skeletal burden scores, reflecting disease severity, as the most consistent predictor of impaired quality of life. Our findings hold significant clinical implications as they draw attention to the clinically unmet need to address quality of life issues in the management of patients with all subtypes of fibrous dysplasia, including its milder forms.
[Mh] MeSH terms primary: Fibrous Dysplasia, Monostotic/physiopathology
Fibrous Dysplasia, Polyostotic/physiopathology
[Mh] MeSH terms secundary: Adolescent
Adult
Aged
Child
Child, Preschool
Female
Fibrous Dysplasia, Monostotic/complications
Fibrous Dysplasia, Polyostotic/complications
Humans
Infant
Male
Middle Aged
Pain/etiology
Pain/physiopathology
Quality of Life
Surveys and Questionnaires
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[Js] Journal subset:IM
[Da] Date of entry for processing:170429
[St] Status:MEDLINE
[do] DOI:10.1186/s13023-017-0629-x

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[PMID]: 29488004
[Au] Autor:Ostertag H; Glombitza S
[Ad] Address:Pathologisches Institut, Klinikum Region Hannover, Haltenhoffstraße 41, 30167, Hannover, Deutschland. helmut.ostertag@krh.eu.
[Ti] Title:Die aktivierende GNAS-Mutation : Eine Bestandsaufnahme bei der Fibrösen Dysplasie, der ihr assoziierten Syndrome sowie weiterer skelettaler und extraskelettaler Läsionen. [The activating GNAS mutation : A survey of fibrous dysplasia, its associated syndromes, and other skeletal and extraskeletal lesions].
[So] Source:Pathologe;, 2018 Feb 27.
[Is] ISSN:1432-1963
[Cp] Country of publication:Germany
[La] Language:ger
[Ab] Abstract: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. Research into the impact of this mutation has increased the understanding of these up to now solely descriptively defined diseases and also allowed easier discrimination of various fibro-osseous skeletal lesions. Current insights suggest that this mutation may also play a pivotal role in other extraskeletal neoplasias.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE; REVIEW
[Em] Entry month:1803
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[St] Status:Publisher
[do] DOI:10.1007/s00292-018-0417-y

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[PMID]: 29294508
[Au] Autor:Guatta R; Scolozzi P
[Ad] Address:Department of Clinical Neurosciences, Neurosurgery, University Hospitals of Geneva, Geneva, Switzerland.
[Ti] Title:Bone Recontouring by Guided Surgical Navigation Integrating "Mirroring" Computational Planning in the Management of Severe Fronto-orbital Asymmetry in Fibrous Dysplasia.
[So] Source:J Neurol Surg A Cent Eur Neurosurg;79(2):181-185, 2018 Mar.
[Is] ISSN:2193-6323
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:We report the successful treatment of severe fronto-orbital asymmetry in a 20-year-old man with fibrous dysplasia by bone recontouring using a specific approach combining "mirroring" virtual computational planning with intraoperative guided surgical navigation.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[St] Status:In-Process
[do] DOI:10.1055/s-0037-1615286

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[PMID]: 29485574
[Au] Autor:Ogul H; Keskin E
[Ad] Address:Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey.
[Ti] Title:Locally Aggressive Fibrous Dysplasia Mimicking Malign Calvarial Lesion.
[So] Source:J Craniofac Surg;, 2018 Feb 26.
[Is] ISSN:1536-3732
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[St] Status:Publisher
[do] DOI:10.1097/SCS.0000000000004453

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[PMID]: 29188758
[Au] Autor:Kim CS; Maxfield AZ; Foyt D; Rapoport RJ
[Ad] Address:a Division of Otolaryngology (CSK, DF) , Albany Medical Center , 50 New Scotland Avenue, MC41, Albany , NY , USA.
[Ti] Title:Utility of intraoperative computed tomography for cochlear implantation in patients with difficult anatomy.
[So] Source:Cochlear Implants Int;19(3):170-179, 2018 May.
[Is] ISSN:1754-7628
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract: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. In both cases, intraoperative CT changed the insertion technique of the operating surgeon and allowed for improved electrode positioning. A 47-year-old female with polyostotic fibrous dysplasia and a 55-year-old male with post-meningitis near-total cochlear obliteration underwent successful cochlear implantation with confirmation of electrode position with intraoperative CT. In the former case, the image-guided navigation system was also implemented. Finally, a 72-year-old female underwent cochlear implantation during which intraoperative C-arm fluoroscopy suggested intra-cochlear insertion. However, postoperative CT showed the electrode extending into the internal auditory canal (IAC), illustrating the limitations of C-arm fluoroscopy. INTERVENTION: Intraoperative CT imaging and image-guided navigation system. CONCLUSION: When faced with challenging temporal bone anatomy, intraoperative CT can provide critical details of the patient's microanatomy that allows for improved localization of the electrode and adjustments in operative techniques for successful cochlear implantation.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180219
[Lr] Last revision date:180219
[St] Status:In-Data-Review
[do] DOI:10.1080/14670100.2017.1403146

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[PMID]: 29192304
[Au] Autor:Boyce AM; Brewer C; DeKlotz TR; Zalewski CK; King KA; Collins MT; Kim HJ
[Ad] Address:Section on Skeletal Disorders and Mineral Homeostasis, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland.
[Ti] Title:Association of Hearing Loss and Otologic Outcomes With Fibrous Dysplasia.
[So] Source:JAMA Otolaryngol Head Neck Surg;144(2):102-107, 2018 Feb 01.
[Is] ISSN:2168-619X
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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. Main Outcomes and Measures: Clinical and radiologic features associated with hearing loss and otologic disease were evaluated. Conductive hearing loss was hypothesized to be associated with narrowing of the external auditory canal (EAC), FD involving the epitympanum, and FD crowding the ossicular chain. Sensorineural hearing loss was hypothesized to be associated with FD affecting the internal auditory canal (IAC) and otic capsule. Results: Of the 130 study participants with craniofacial FD who were evaluated, 116 (89.2%) had FD that involved the temporal bone (median age, 19.6 years; range, 4.6-80.3 years; 64 female [55.2%]), whereas 14 (10.8%) had craniofacial FD that did not involve the temporal bone. Of the 183 ears with temporal bone FD, hearing loss was identified in 41 ears (22.4%) and was conductive in 27 (65.9%), sensorineural in 12 (29.3%), and mixed in 2 (4.9%). Hearing loss was mild and nonprogressive in most participants. Whereas EACs were narrower in ears with FD (mean difference [MD], 0.33 mm; 95% CI, 0.11-0.55 mm), this finding was associated with conductive hearing loss in only 4 participants. Fibrous dysplasia crowding of the ossicles was associated with conductive hearing loss (odds ratio [OR], 5.0; 95% CI, 2.1-11.6). The IAC length was not different between ears with and without FD (MD, -0.37; 95% CI, -0.95 to 0.211); however, canals were elongated in ears with sensorineural hearing loss (MD, -1.33; 95% CI, -2.60 to -0.07). Otic capsule involvement was noted in only 4 participants, 2 of whom had sensorineural hearing loss. Both MAS-associated growth hormone excess (OR, 3.1; 95% CI, 1.3-7.5) and neonatal hypercortisolism (OR, 11; 95% CI, 2.5-55) were associated with an increased risk of hearing loss . Conclusions and Relevance: Hearing loss in craniofacial FD is common and mild to moderate in most individuals. It typically arises from FD crowding of the ossicular chain and elongation of the IAC, whereas EAC stenosis and otic capsule invasion are less common causes. Individuals with craniofacial FD should undergo otolaryngologic evaluation and monitoring, including assessment to identify those with high-risk features.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180216
[Lr] Last revision date:180216
[St] Status:In-Data-Review
[do] DOI:10.1001/jamaoto.2017.2407


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