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Fibrous dysplasia

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https://www.readbyqxmd.com/read/29053652/a-report-on-the-clinical-pathological-correlations-of-788-gingival-lesion
#1
A Gambino, M Carbone, R Broccoletti, P Carcieri, D Conrotto, M Carrozzo, P-G Arduino
BACKGROUND: The diagnosis and treatment of a variety of non-plaque related gingival diseases have become an integrated aspect of everyday dentistry. The aim of this study was to analyse the relationship between clinical appearance and histopathological features of gingival lesions in a large Northern Italian population. MATERIAL AND METHODS: A retrospective study of 788 cases of gingival and alveolar mucosal biopsies was set up. Statistical analysis was performed by calculating the odds ratio and 95% confidence interval (C...
October 20, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
https://www.readbyqxmd.com/read/29038079/optical-coherence-tomography-in-the-management-of-skull-base-fibrous-dysplasia-with-optic-nerve-involvement
#2
Joshua Loewenstern, Christopher M Hernandez, Carolyn Chadwick, Amish Doshi, Rudrani Banik, Christopher A Sarkiss, Joshua Bederson, Raj K Shrivastava
BACKGROUND: Fibrous dysplasia (FD) of the skull base can present with optic nerve compression. As most patients initially do not experience vision loss, controversy exists whether to proceed with prophylactic surgical decompression or elect for conservative observation. Optical coherence tomography (OCT), a physiologic imaging modality widely used to assess the condition of the retinal nerve fiber layer (RNFL), has been useful in monitoring compressive tumors on the optic nerve. The present study evaluated the potential use of OCT in the management of FD patients with optic nerve involvement...
October 13, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29022055/illness-perceptions-are-associated-with-quality-of-life-in-patients-with-fibrous-dysplasia
#3
B C J Majoor, C D Andela, C R Quispel, M Rotman, P D S Dijkstra, N A T Hamdy, A A Kaptein, N M Appelman-Dijkstra
Fibrous dysplasia (FD) is a rare bone disorder in which normal bone is replaced by fibrous tissue resulting in pain, deformities, pathological fractures or asymptomatic disease. Illness perceptions are patients' cognitions and emotions about their illness and its treatment, which may impact on Quality of Life (QoL). Here, we explore illness perceptions in patients with FD compared to other disorders, identify factors associated with illness perceptions and evaluate their relationship with QoL. Ninety-seven out of 138 eligible patients from the LUMC FD cohort completed the Illness Perception Questionnaire-Revised (IPQ-R) and the Short Form-36 (SF-36)...
October 11, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/29021887/a-modified-technique-of-fixation-for-proximal-femoral-valgus-osteotomy-in-abnormal-bone-a-report-of-two-cases
#4
S Logheswaren, A R Sulaiman, I Munajat
The ideal size of intramedullary device to fix corrective osteotomy of proximal femur in abnormal bone in children and small patients may not be easily available. We report the successful use of Rush rod in combination with multiple Kirschner wires to fix the corrective osteotomy of coxa vara and shepherd crook deformity in two patients with osteogenesis imperfecta and fibrous dysplasia. The union was achieved on time, neck shaft angle and rotation were maintained.
July 2017: Malaysian Orthopaedic Journal
https://www.readbyqxmd.com/read/28966816/natural-history-of-cranial-fibrous-dysplasia-revealed-during-long-term-follow-up-case-report-and-literature-review
#5
David L Penn, Richard J Tartarini, Carolyn H Glass, Umberto De Girolami, Amir A Zamani, Ian F Dunn
BACKGROUND: Fibrous dysplasia (FD) is a rare developmental disease characterized by the replacement of bone marrow with proliferating fibro-osseous tissue. There exist three forms of FD-monostotic, polyostotic, and that associated with McCune-Albright syndrome. The disease can present in different locations and with a variety of symptoms. One of the more common locations of FD occurrence is the craniofacial region. Treatment of asymptomatic FD often involves conservative management with serial imaging...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28965204/dental-manifestations-of-pediatric-bone-disorders
#6
REVIEW
Juan F Yepes
PURPOSE OF REVIEW: Several bone disorders affecting the skeleton often are manifest in the maxillofacial region. This review presents the most common bone disorders in children and their dental-oral manifestations: fibrous dysplasia, Paget's disease, osteogenesis imperfecta, renal osteodystrophy, hypophosphatasia, and osteoporosis. The specific intraoral characteristics will reviewed in detail. RECENT FINDINGS: Recent studies confirmed the close relationship between the mandible and the maxilla with the most prevalent systemic bone disorders in children...
September 30, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28963390/acromegaly-with-hypophosphataemia-mccune-albright-syndrome
#7
Rimesh Pal, Pinaki Dutta, Kanchan Kumar Mukherjee, Anil Bhansali
A 38-year-old man presented with excessive height gain and progressive enlargement of the extremities since childhood. This was compounded by lower limb deformities over the past 5 years. On examination, his height was 196 cm, he had macroglossia, acral enlargement, seborrhoea, hyperhidrosis-suggesting acrogigantism. He had facial asymmetry, wind-swept deformity of lower limbs and a café-au-lait macule over his trunk. Investigations revealed normal-sized pituitary gland with dysplastic cranial bones. Isotope bone scintigraphy was suggestive of polyostotic fibrous dysplasia...
September 28, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28958926/emergency-optic-canal-decompression-for-vision-salvage-in-fibrous-dysplasia-a-case-report
#8
Reem Elwy, Murat Gokden, Rongsheng Cai
BACKGROUND: The most common neurological manifestations of fibrous dysplasia (FD) are vision and hearing loss. Optic decompression for progressive vision loss has been shown to yield positive results in terms of visual outcomes, however, emergency optic decompression surgery for sudden loss of vision in FD has not to date been reported in the pediatric population. We report the first case of FD presenting with sudden vision loss and successfully managed with emergency optic decompression...
September 26, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28940541/ebstein-anomaly-and-sudden-childhood-death
#9
Amanda Freeman, Roger W Byard
A 13-year-old girl is reported who died suddenly and unexpectedly in her sleep from previously undiagnosed Ebstein anomaly. At autopsy, there was dilatation of the right atrium with marked dilatation of the right auricle and apical displacement of the tricuspid valve into the right ventricular cavity with atrialization of the upper portion of the right ventricle. There were also prominent dysplastic changes in both the septal and posterior leaflets of the tricuspid valve with thickening of the valve and fusion of leaflets to the wall of the ventricle...
September 20, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28918753/neonatal-intestinal-obstruction-associated-with-situs-inversus-totalis-two-case-reports-and-a-review-of-the-literature
#10
Rahul Gupta, Varsha Soni, Prakash Devidas Valse, Ram Babu Goyal, Arun Kumar Gupta, Praveen Mathur
BACKGROUND: The association of neonatal intestinal obstruction with situs inversus totalis is extremely rare with only few cases reported in the literature to date. This association poses dilemmas in management. We present two such cases (of Indian origin), and briefly discuss the pertinent literature and measures to prevent unfavorable outcome. CASE PRESENTATION: Case 1: a 1-month-old preterm (1300 g) male neonate belonging to Hindu (Indian) ethnicity presented with recurrent bile-stained vomiting, non-passage of stools and epigastric fullness...
September 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28894655/intraoperative-evaluation-of-body-surface-improvement-by-an-augmented-reality-system-that-a-clinician-can-modify
#11
Daisuke Mitsuno, Koichi Ueda, Tomoki Itamiya, Takashi Nuri, Yuki Otsuki
BACKGROUND: Augmented reality (AR) technology that can combine computer-generated images with a real scene has been reported in the medical field recently. We devised the AR system for evaluation of improvements of the body surface, which is important for plastic surgery. METHODS: We constructed an AR system that is easy to modify by combining existing devices and free software. We superimposed the 3-dimensional images of the body surface and the bone (obtained from VECTRA H1 and CT) onto the actual surgical field by Moverio BT-200 smart glasses and evaluated improvements of the body surface in 8 cases...
August 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28856726/increased-risk-of-breast-cancer-at-a-young-age-in-women-with-fibrous-dysplasia
#12
Bas C J Majoor, Alison M Boyce, Judith V M G Bovée, Vincent T H B M Smit, Michael T Collins, Anne-Marie Cleton-Jansen, Olaf M Dekkers, Neveen A T Hamdy, Sander P D Dijkstra, Natasha M Appelman-Dijkstra
Background Fibrous dysplasia is a rare bone disorder caused by mutations of the GNAS-gene, which are also identified in malignancies. We explored the potential relationship between breast cancer and fibrous dysplasia in two fibrous dysplasia cohorts from the Netherlands and the USA Patients and Methods Data on fibrous dysplasia and breast cancer diagnosis were retrieved from hospital-records of 134 (Netherlands) and 121 (USA) female patients. Results were validated with breast cancer data of 645 female fibrous dysplasia patients from the Dutch Pathology Registry (PALGA)...
August 30, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28845837/mazabraud-syndrome-associated-with-mccune-albright-syndrome-a-case-report-and-review-of-the-literature
#13
Alessio Biazzo, Andrea Di Bernardo, Antonina Parafioriti, Norberto Confalonieri
Mazabraud syndrome is a very rare benign disorder characterized by the association of monostotic or polyostotic fibrous dysplasia and one or multiple intramuscular myxomas. McCune -Albright syndrome is a rare benign disorder characterized by the association of polyostotic fibrous dysplasia, cafè-au-lait skin pigmentations and endocrine dysfunction, such as precocious puberty, diabetes mellitus, goiter and breast fibroadenomatosis. The association of Mazabraud syndrome and McCune-Albright in the same patient is an anecdotal event...
August 23, 2017: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/28840064/polyostotic-craniofacial-fibrous-dysplasia-with-bizarre-radiologic-finding-mandible-anterior-skull-base-frontal-temporal-parietal-and-occipital-bones-involvement
#14
Alireza Tabibkhooei, Morteza Taheri, Sadra Rohani, Alireza Azimi
No abstract text is available yet for this article.
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28839487/computed-tomography-in-craniofacial-fibrous-dysplasia-a-case-series-with-review-of-literature-and-classification-update
#15
REVIEW
Deepak Gupta, Preeti Garg, Amit Mittal
INTRODUCTION: Fibrous dysplasia (FD) is a fibroosseous lesion of the osseous structures of the body. It is not a commonly reported lesion yet it is considered as an important lesion which can affect the maxillofacial region as well. As a result, it can cause deformity of the jaw bones which can further lead to severe facial asymmetry. Craniofacial fibrous dysplasia (CFD) is one of the subtypes of FD that can affect the bones of the craniofacial complex, including the mandible and maxilla...
2017: Open Dentistry Journal
https://www.readbyqxmd.com/read/28830313/characteristics-of-sinonasal-fibrous-dysplasia-experience-from-a-single-department
#16
Chen Duan, Qi Dai, Quan Liu, Hongmeng Yu
OBJECTIVES: We summarized the characteristics of fibrous dysplasia patients in our department to obtain clinical data on this disease. METHODS: We retrospectively reviewed 28 patients who were diagnosed with sinonasal fibrous dysplasia based on pathological examination results from March 2012 to February 2017 in our department. RESULTS: The 28 patients included 16 females and 12 males, and the median age was 31.5 (range, 7-69) years. The three most common symptoms were nasal obstruction (25%), impaired vision (21%) and headache (18%)...
August 22, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28806239/18f-fdg-pet-ct-of-secondary-epithelioid-angiosarcoma-of-the-proximal-femur-in-a-patient-with-polyostotic-fibrous-dysplasia
#17
Michiyuki Hakozaki, Hitoshi Yamada, Osamu Hasegawa, Kazuo Watanabe, Shinichi Konno
Fibrous dysplasia rarely transforms into a secondary malignancy. We present the PET/CT findings at pretreatment and posttreatment in an exceedingly rare case of epithelioid angiosarcoma of the bone secondary to polyostotic fibrous dysplasia.
October 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28792000/-lower-extremity-varicose-veins-in-childhood-and-at-a-young-age-mechanism-of-development-and-specific-features
#18
V V Studennikova, L O Severgina, A N Dzyundzya, I A Korovin
In Russia more than 125,000 patients with various venous diseases, lower extremity varicose veins (LEVV) being predominant, were annually operated on. In recent years, there has been a trend toward younger patients with signs of LEVV. Screening studies have revealed the signs of the disease in 10-15% of high-school children. The high prevalence of LEVV as a whole and its younger onset in recent decades cause more attention to an investigation of the relationship between the development of varicose veins, in childhood and adolescence in particular, and genomic changes...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28761541/post-traumatic-fibrous-dysplasia-of-the-parietal-bone-a-rare-entity
#19
Muhammad Sohail Umerani, Saqib Kamran Bakhshi, Asad Abbas, Salman Sharif, Sidra Arshad
Fibrous dysplasia (FD) is a rare fibro-osseous lesion in which normal bone is replaced by abnormal fibrous tissue. Although a congenital disorder, a single case report of traumatic etiology had been described in the literature. We report a case of monostotic FD of the parietal calvarium in a 21-year-old female patient who presented to us with a single swelling in the parietal region been noticed after head injury sustained at the age of 7 years. After imaging investigations, the lesion was excised via craniotomy followed by cranioplasty in the same sitting...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28761532/skull-base-bony-lesions-management-nuances-a-retrospective-analysis-from-a-tertiary-care-centre
#20
Amit Kumar Singh, Arun Kumar Srivastava, Jayesh Sardhara, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Rabi Narayan Sahu, Awadhesh Kumar Jaiswal, Sanjay Behari
BACKGROUND: Skull base lesions are not uncommon, but their management has been challenging for surgeons. There is large no of bony tumors at the skull base which has not been studied in detail as a group. These tumors are difficult not only because of their location but also due to their variability in the involvement of important local structure. Through this retrospective analysis from a Tertiary Care Centre, we are summarizing the details of skull base bony lesions and its management nuances...
July 2017: Asian Journal of Neurosurgery
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