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Fibrous dysplasia

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https://www.readbyqxmd.com/read/28721303/value-of-99m-tc-mdp-spect-ct-and-18-f-fdg-pet-ct-scanning-in-the-evaluation-of-malignantly-transformed-fibrous-dysplasia
#1
Wei-Jun Wei, Zhen-Kui Sun, Chen-Tian Shen, Xin-Yun Zhang, Juan Tang, Hong-Jun Song, Zhong-Ling Qiu, Quan-Yong Luo
Although fibrous dysplasia is not considered a potentially premalignant disorder, malignant transformation occurs. Because of its rarity, radiographic features of malignantly transformed fibrous dysplasia on cross-sectional imaging modalities are less recognized, making diagnosis and differential diagnosis of the disease quite difficult in clinical practice. In this study, we analyzed the clinical characteristics, imaging features, pathology findings and surgery strategies of 19 malignantly transformed fibrous dysplasia...
2017: American Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28708654/the-status-of-affected-infraorbital-nerve-and-inferior-alveolar-nerve-in-patients-with-jaw-fibrous-dysplasia-a-clinical-and-radiographic-evaluation
#2
Zhen Du, Yifeng Jiang, Long Chen
The affected infraorbital nerve (IFBN) and inferior alveolar nerve (IFAN) status in patients with jaw fibrous dysplasia has not been definitely depicted. In this study, the authors try to explore the status of affected IFBN and IFAN in patients with jaw fibrous dysplasia. Ten patients with jaw fibrous dysplasia were included in this study. The complaints of numbness in the IFBA and IFAN innervated area were asked and recorded, and careful clinical examination was performed to evaluate the touch sense, pain sense, pressure sense, and temperature sense in the IFBA and IFAN innervated areas...
July 13, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28699175/long-term-health-outcomes-of-adults-with-mccune-albright-syndrome
#3
S C Wong, M Zacharin
CONTEXT: McCune Albright Syndrome (MAS) is associated with numerous health problems. Comprehensive long term health problems of adults with MAS are less well defined in the literature. OBJECTIVE: Our objective is to report comprehensive health outcomes of adults with MAS (> 18 years). DESIGN: Retrospective case note review of 16 adults with MAS managed by one clinician. Results expressed as median (range) RESULTS: The study included 16 adults (7 males) with MAS...
July 12, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28697788/anatomic-histopathologic-and-echocardiographic-features-in-a-dog-with-an-atypical-pulmonary-valve-stenosis-with-a-fibrous-band-of-tissue-and-a-patent-ductus-arteriosus
#4
Hakyoung Yoon, Jaehwan Kim, Sang-Soep Nahm, Kidong Eom
BACKGROUND: Congenital pulmonary valve stenosis and patent ductus arteriosus are common congenital heart defects in dogs. However, concurrence of atypical pulmonary valve stenosis and patent ductus arteriosus is uncommon. This report describes the anatomic, histopathologic, and echocardiographic features in a dog with concomitant pulmonary valve stenosis and patent ductus arteriosus with atypical pulmonary valve dysplasia that included a fibrous band of tissue. CASE PRESENTATION: A 1...
July 11, 2017: Acta Veterinaria Scandinavica
https://www.readbyqxmd.com/read/28692759/sperm-morphology-assessment-pathophysiology-clinical-relevance-and-state-of-the-art-in-2017
#5
REVIEW
N Gatimel, J Moreau, J Parinaud, R D Léandri
For over 30 years, sperm morphology assessment has been one of the most common tests in evaluation of fertility. This review examines the clinical relevance of sperm morphology assessment in the diagnosis of infertility and in assisted reproductive technology, as well as its analytical reliability. Publications on the pathophysiology, the analytical reliability of the test and its clinical relevance in diagnosis and in Assisted Reproductive Technology (ART) were evaluated. This review compared and discussed study methodologies and results, including patient characteristics, preparation, smear staining methods and classification systems...
July 10, 2017: Andrology
https://www.readbyqxmd.com/read/28676838/effect-of-intranasal-calcitonin-in-a-patient-with-mccune-albright-syndrome-fibrous-dysplasia-and-refractory-bone-pain
#6
Tayane Muniz Fighera, Poli Mara Spritzer
McCune-Albright syndrome (MAS) is a rare disease defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin spots, and precocious puberty. No available treatment is effective in changing the course of fibrous dysplasia of bone, but symptomatic patients require therapeutic support to reduce bone pain and prevent fractures and deformities. We report the case of a 27-year-old woman with MAS and severe fibrous dysplasia. She was diagnosed with MAS at 4 years of age and, during follow-up, she had multiple pathological fractures and bone pain refractory to treatment with bisphosphonates, tricyclic antidepressants, and opioids...
2017: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/28660402/bisphosphonate-induced-zebra-lines-in-fibrous-dysplasia-of-bone-histo-radiographic-correlation-in-a-case-of-mccune-albright-syndrome
#7
Alessandro Corsi, Ernesto Ippolito, Pamela G Robey, Mara Riminucci, Alan Boyde
Bisphosphonates (BPs) are currently used in the treatment of diverse bone diseases including fibrous dysplasia of bone (FD). In pediatric patients, a radiographic consequence of cyclical administration of BPs is the development of apo-, epi-, and meta-physeal sclerotic bands, otherwise known as zebra lines, which result from the temporary inhibition of osteoclastic activity at the time of drug treatment. We report here on a child with McCune-Albright syndrome (FD in addition to hyperfunctioning endocrinopathies and skin hyperpigmentation) treated with cyclical intravenous infusions of pamidronate in which conventional radiography, contact microradiography, histology, and backscattered electron image analysis demonstrated that zebra lines formed only where bone was normal, were arrested at the boundary between FD-unaffected and FD-affected bone where bone is sclerotic, and were absent within the undermineralized FD bone...
June 28, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28642192/applications-of-3d-orbital-computer-assisted-surgery-cas
#8
P Scolozzi
INTRODUCTION: The purpose of the present report is to describe the indications for use of 3D orbital computer-assisted surgery (CAS). PATIENTS AND METHODS: We analyzed the clinical and radiological data of all patients with orbital deformities treated using intra-operative navigation and CAD/CAM techniques at the Hôpitaux Universitaires de Genève, Switzerland, between 2009 and 2016. We recorded age and gender, orbital deformity, technical and surgical procedure and postoperative complications...
June 19, 2017: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28612379/mccune-albright-syndrome-associated-bone-marrow-failure-and-extramedullary-haematopoeisis-secondary-to-fibrous-dysplasia
#9
Ali Jassem Mahdi, Phillip Connor, Indu Thakur
No abstract text is available yet for this article.
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28608487/intramuscular-myxoma-and-fibrous-dysplasia-of-bone-presents-as-mazabraud-s-syndrome
#10
Benny Yau, Hock W Kua, Michael F Lo
No abstract text is available yet for this article.
June 13, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28588314/frequency-of-gnas-r201h-substitution-mutation-in-polyostotic-fibrous-dysplasia-pyrosequencing-analysis-in-tissue-samples-with-or-without-decalcification
#11
Su-Jin Shin, Seok Joo Lee, Sang Kyum Kim
Guanine nucleotide-binding protein/α-subunit (GNAS) mutations are involved in fibrous dysplasia (FD) pathogenesis. Here, we analyzed GNAS mutations in FD which were performed by pyrosequencing DNA isolated from formalin-fixed paraffin-embedded (FFPE) tissue. The mutation detection rate was determined in FD specimens with and without decalcification. GNAS mutation was identified in 28 cases out of 87 FDs (32.18%) [p.R201C (N = 14) and p.R201H (N = 14)]. GNAS mutation was more likely to occur in polyostotic FD (7/28, 25...
June 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28586604/orbital-involvement-in-sinonasal-diseases
#12
Zia-Us-Salam Qazi, Sarfraz Latif, Sadia Maqsood Awan
BACKGROUND: Orbital involvement in sinonasal diseases can present as proptosis, ophthalmoplegia or even as blindness due to optic nerve damage. There are a number of sinonasal diseases which can involve eyes. The purpose of this study was to enlist diagnoses of all the patients with sinonasal disease, in which orbit was also involved unilaterally or bilaterally and to analyse the management strategy and final outcome in all the cases. METHODS: Hundred consecutive patients having orbital symptoms along with sinonasal complaints that presented in ENT department of Shaikh Zayed federal postgraduate medical institute were included in our prospective study...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28583274/benign-bone-conditions-that-may-be-fdg-avid-and-mimic-malignancy
#13
REVIEW
Thomas C Kwee, John M H de Klerk, Maarten Nix, Ben G F Heggelman, Stefan V Dubois, Hugo J A Adams
Positron emission tomography with the radiotracer (18)F-fluoro-2-deoxy-d-glucose (FDG) plays an important role in the evaluation of bone pathology. However, FDG is not a cancer-specific agent, and knowledge of the differential diagnosis of benign FDG-avid bone alterations that may resemble malignancy is important for correct patient management, including the avoidance of unnecessary additional invasive tests such as bone biopsy. This review summarizes and illustrates the spectrum of benign bone conditions that may be FDG-avid and mimic malignancy, including osteomyelitis, bone lesions due to benign systemic diseases (Brown tumor, Erdheim-Chester disease, Gaucher disease, gout and other types of arthritis, Langerhans cell histiocytosis, and sarcoidosis), benign primary bone lesions (bone cysts, chondroblastoma, chondromyxoid fibroma, desmoplastic fibroma, enchondroma, giant cell tumor and granuloma, hemangioma, nonossifying fibroma, and osteoid osteoma and osteoblastoma), and a group of miscellaneous benign bone conditions (post bone marrow biopsy or harvest status, bone marrow hyperplasia, fibrous dysplasia, fractures, osteonecrosis, Paget disease of bone, particle disease, and Schmorl nodes)...
July 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28580818/xanthomatous-posttraumatic-fibro-osseous-lesion-of-the-rib-a-rare-and-underrecognized-entity-case-report-and-literature-review
#14
Hussein Nassereddine, Frédérique Larousserie, Raphaël Campagna, Yves Castier, Anne Couvelard, Laurence Choudat, Aurélie Sannier
Posttraumatic fibro-osseous lesion (PTFOL) is a rare lesion that typically affects the ribs and is probably a posttraumatic reactive process. Because PTFOL is often misdiagnosed as fibrous dysplasia, osteoid osteoma, benign fibrous histiocytoma or rib metastases, chest wall resection, leading to a significant morbidity, is the main treatment modality. We report the case of a 30-year-old male patient with no history of previous trauma presenting with chest pain. Computed tomography scan showed an eighth left rib well-defined ovoid and hypodense lesion with circumferential sclerotic margin and no cortical breakthrough...
June 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28577616/dnah1-gene-mutations-and-their-potential-association-with-dysplasia-of-the-sperm-fibrous-sheath-and-infertility-in-the-han-chinese-population
#15
Yanwei Sha, Xiaoyu Yang, Libin Mei, Zhiyong Ji, Xu Wang, Lu Ding, Ping Li, Shenmin Yang
OBJECTIVE: To investigate dynein, axonemal, heavy chain 1 (DNAH1) gene mutations that may be associated with dysplasia of the sperm fibrous sheath (DFS) and infertility in the Han Chinese population. DESIGN: Dysfunction of DNAH1 is known to cause multiple morphologic abnormalities of the flagella (MMAF), DFS, and infertility. Whole-exome sequencing was performed in DFS subjects and the healthy control subjects. SETTING: Not applicable. PATIENT(S): Twenty-one patients of Han ethnicity with primary infertility and diagnosed with asthenozoospermia and MMAF, but without primary ciliary dyskinesia...
June 2017: Fertility and Sterility
https://www.readbyqxmd.com/read/28533645/image-findings-of-polyostotic-fibrous-dysplasia-mimicking-metastasis-in-f-18-fdg-positron-emission-tomography-computed-tomography
#16
Koramadai Karuppusamy Kamaleshwaran, Jephy Joseph, Radhakrishnan Kalarikal, Ajit Sugunan Shinto
Fibrous dysplasia (FD) of the bone is characterized by the medullary cavity of bones becoming filled with fibrous tissue, and its etiology remains unknown. It is usually asymptomatic and found incidentally on imaging studies that are performed for other purposes. FD may closely mimic the appearance of bony metastatic disease on radiological examinations. We report the case of a 45-year-old female patient, which appeared to have multiple bone lesions on initial workup images. Subsequently, the bone lesions that showed increased FDG uptake on PET/CT in right femur and tibia were identified as FD...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28533644/atypical-parathyroid-adenoma-with-multiple-brown-tumors-as-initial-presentation-a-rare-entity
#17
V S Krishna Mohan, Manishi L Narayan, Arun Mukka, Bharath Bachimanchi, Amit Kumar Chowhan, B Vijayalakshmi Devi, Suresh Vaikkakara, Alok Sachan
Brown tumors seen in hyperparathyroidism are rare, non-neoplastic lesions because of abnormal bone metabolism, and they can mimic benign bone tumors or malignancy. Although biopsy is considered as the gold standard for diagnosis, it can be inconclusive. As the diagnosis of brown tumors is often challenging, a high index of suspicion is essential for diagnosis. We present a case of 21-year-old woman who presented with multiple painful bony lesions, which were initially misdiagnosed as fibrous dysplasia. Due to persistent bone pain and deterioration in her physical mobility, she was referred to tertiary care centre...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28488027/fibrous-dysplasia-mimicking-bone-metastasis-on-68-ga-psma-pet-mri
#18
Liesbeth De Coster, Raf Sciot, Wouter Everaerts, Olivier Gheysens, Raf Verscuren, Christophe M Deroose, Steven Pans, Koen Van Laere, Karolien E Goffin
No abstract text is available yet for this article.
August 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28481787/evaluation-of-practical-interpretation-hurdles-in-68ga-psma-pet-ct-in-55-patients-physiological-tracer-distribution-and-incidental-tracer-uptake
#19
Julian Kirchner, Benedikt Michael Schaarschmidt, Lino Morris Sawicki, Philipp Heusch, Hubertus Hautzel, Johannes Ermert, Robert Rabenalt, Gerald Antoch, Christian Buchbender
PURPOSE: To investigate the physiologic Ga-PSMA distribution and evaluate focal or diffuse radiotracer uptake in nonprostate cancer malignancies and in incidental findings. METHODS: Ga-PSMA PET/CT scans in 55 men performed for prostate cancer (49) or renal cell carcinoma (6) staging were analyzed retrospectively. Two radiologists evaluated the datasets in 2 reading sessions. First, physiological Ga-PSMA uptake was evaluated. Second, scans were analyzed for incidental uptake...
July 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28479714/nonfamilial-cherubism-a-case-report-and-review-of-literature
#20
Revati Deshmukh, Samir Joshi, Priya Nimish Deo
Cherubism is a rare hereditary developmental condition of the jaws and generally inherited as an autosomal dominant trait. It is also known as familial fibrous dysplasia of the jaws, familial multilocular cystic disease and hereditary fibrous dysplasia of the jaws. The gene for cherubism is mapped to chromosome 4p16.3 may lead to pathologic activation of osteoclasts and disruption of jaw morphogenesis. The lesion usually appears between 2 and 5 years shows a predilection for the mandible and causes a bilateral swelling giving rise to a cherubic chubby appearance...
January 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
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