Fibrous Dysplasia Of Bone
Mostrando 1-12 de 17 artigos, teses e dissertações.
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1. Effects of zoledronic acid therapy in fibrous dysplasia of bone: a single-center experience
ABSTRACT Objective: Fibrous dysplasia (FD) is a rare bone disorder that can involve any part of the skeleton, leading to bone pain, deformities, and fractures. Treatment with intravenous bisphosphonates has been used with variable results. Therefore, we aimed to evaluate the effects of zoledronic acid (ZA) therapy in patients with monostotic or polyostotic
Archives of Endocrinology and Metabolism. Publicado em: 2022
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2. McCune-Albright syndrome - A case report with transmission electron microscopy
Abstract McCune - Albright syndrome is a genetic disease with cutaneous mosaicism caused by post-zygotic activating mutations in GNAS locus, it has a triad of fibrous bone dysplasia, café-au-lait macules and precocious puberty. We examined a 22-year-old female patient with café au lait spot in right side of the abdomen, with a chessboard - like distributio
Anais Brasileiros de Dermatologia. Publicado em: 2022
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3. Pharmacological management of osteogenesis
Osteogenesis and bone remodeling are complex biological processes that are essential for the formation of new bone tissue and its correct functioning. When the balance between bone resorption and formation is disrupted, bone diseases and disorders such as Paget's disease, fibrous dysplasia, osteoporosis and fragility fractures may result. Recent advances in
Clinics. Publicado em: 2014-06
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4. Padronização das técnicas de PNA e PCR em tempo real para detecção das mutações ativadoras no GNAS na síndrome de McCune-Albright / Standardization of the PNA and real time techniques for the detection of activating mutations in the GNAS in McCune-Albright syndrome
The McCune-Albright Syndrome (MAS) is a genetic disease, with incidence estimated at 1/100.000 and 1/1000000 cases per year. MAS is clinically characterized by the triad: bone fibrous dysplasia (FD) café-au-lait skin spots and endocrine hyperfunction, such as: precocious puberty (PP), Cushing s syndrome, hyperthyroidism and acromegaly. The diagnosis of MAS
IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia. Publicado em: 05/10/2012
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5. Análise molecular dos genes HRPT2 e CICLINA D1 na displasia fibrosa, no fibroma ossificante e no osteossarcoma dos maxilares
Fibrous dysplasia (FD), ossifying fibroma (OF), and osteosarcoma (OS) are examples of bone-related lesions. Recent studies have already shown genetic alterations of the HRPT2 tumor suppressor gene in sporadic and syndromic OF. The present study first sought to raise the hypothesis of whether or not similar alterations would in fact represent one of the under
IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia. Publicado em: 21/05/2012
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6. Caracterização da displasia fibrosa em imagens de tomografia computadorizada helicoidal empregando a análise da lacunaridade / Characterization of fibrous dysplasia in helical computed tomography images employing the analysis of lacunarity
Fibrous dysplasia is an alteration of development characterized by replacing normal bone for dense connective tissue and immature trabecular bones, typically found in teenagers and young adults. Genetic modification which involves alpha-Gs protein appears to be the basis of the process. The exact incidence and prevalence are difficult to be established, but
IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia. Publicado em: 04/05/2012
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7. Análise das características clinicopatológicas de displasias fibrosas e fibromas ossificantes centrais envolvendo mandíbula e maxila : estudo colaborativo internacional / Clinicopathological analysis of fibrous dysplasia and central ossifying fibroma of the jaws : an international collaborative study
A displasia fibrosa (DF) e o fibroma ossificante central (FOC) fazem parte de um grupo de lesões conhecido como fibro-ósseas benignas (LFOB) e afetam principalmente a maxila, a mandíbula e ossos da região craniofacial. Caracterizam-se pela substituição do tecido ósseo normal por uma matriz de tecido conjuntivo fibroso com níveis variados de material
IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia. Publicado em: 16/09/2011
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8. Expressão de reguladores da reabsorção óssea (RANK/RANKL/OPG) e formação óssea (osteocalcina) em lesões realcionadas ao osso e osteossarcoma / Markers of bone remodeling in neoplastic and bone-related lesions
The RANK (receptor activator of nuclear factor Kappa-Beta)-RANKL (receptor activator of nuclear factor-kappa beta ligand)-OPG (osteoprotegerin) system is the principal means of differentiating and activating osteoclasts. Changes along this path have been associated with various bone related lesions (BRL), whether benign or malignant, such as osteosarcoma (OS
IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia. Publicado em: 19/03/2010
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9. The surgical treatment of fibrous dysplasia. With emphasis on recent contributions from cranio-maxillo-facial surgery.
Fibrous dysplasia is a congenital, metabolic, nonfamilial disturbance that occurs in one or more bones, at times in association with skin pigmentations or endocrine abnormalities. The authors report on a large personal series of 23 patients with fibrous dysplasia involving the craniofacial skeleton. The etiology, clinical findings, pathology, and differentia
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10. Fibrous dysplasia of the orbit.
Twelve patients with fibrous dysplasia of the orbit are reviewed and the ophthalmic findings described. Three case histories are presented in detail. Six patients were managed conservatively; four have shown radiological progression of the disease. Six patients underwent surgery. A conservative procedure, comprising debulking dysplastic bone, was carried out
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11. Increased IL-6-production by cells isolated from the fibrous bone dysplasia tissues in patients with McCune-Albright syndrome.
McCune-Albright syndrome (MAS) is characterized by café-au-lait spot, multiple endocrine hyperfunction, and polyostotic fibrous dysplasia. A somatic point mutation of Gsalpha protein was reported to decrease GTPase activity, leading to increase in the GSalpha-associated hormone actions via cAMP. IL-6 is known to stimulate osteoclast formation and in the IL-
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12. Reproduction of human fibrous dysplasia of bone in immunocompromised mice by transplanted mosaics of normal and Gsalpha-mutated skeletal progenitor cells.
We have isolated progenitor cells from the stromal system of the fibrous dysplastic marrow of patients with McCune-Albright Syndrome. Analysis of the Gsalpha gene from individual colonies provided direct evidence for the presence of two different genotypes within single fibrous dysplastic lesions: marrow stromal cells containing two normal Gsalpha alleles, a