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- 1. 小児および思春期における良性骨腫瘍の概要 overview of benign bone tumors in children and adolescents
- 2. 骨肉腫：疫学、病因、臨床症状、診断、および組織学的検査 osteosarcoma epidemiology pathogenesis clinical presentation diagnosis and histology
- 3. 思春期早発症の定義、原因、および評価 definition etiology and evaluation of precocious puberty
- 4. 思春期早発症の治療 treatment of precocious puberty
- 5. デスモイド腫瘍：疫学、危険因子、分子学的病因、臨床症状、診断、および局所療法 desmoid tumors epidemiology risk factors molecular pathogenesis clinical presentation diagnosis and local therapy
- Rapid biochemical response to denosumab in fibrous dysplasia of bone: report of two cases.
- Ganda K, Seibel MJ.Author information Department of Endocrinology and Metabolism, Concord Hospital, Level 6 Concord Hospital Medical Centre, Hospital Road, Concord, NSW, 2139, Australia, email@example.com.AbstractWe report on the clinical and biochemical outcomes in two adult patients with active polyostotic fibrous dysplasia (FD) treated with the RANK-L inhibitor, denosumab, following unsatisfactory responses to prior long-term bisphosphonate therapy. A 44-year-old female (case 1) who had received a cumulative dose of 20 mg zoledronic acid over 2.5 years and a 48-year-old male (case 2) who had received a cumulative dose of 45 mg zoledronic acid over 8 years both experienced minimal reductions in pain scores and markers of bone turnover. Following initiation of denosumab 60 mg sc, changes in bone pain, bone turnover [assessed by serum amino-terminal propeptide of type I collagen (PINP) and urinary deoxypyridinoline] were monitored over a period of 20 and 8 months, respectively. Following administration of denosumab, both patients demonstrated a rapid and pronounced biochemical response: Within 4-7 weeks, bone turnover markers fell to levels within the respective reference range, and one patient reported a reduction in pain. Treatment with denosumab was well tolerated. However, transient asymptomatic hypocalcaemia and/or hypophosphatemia associated with a transient two to threefold increase in serum PTH levels was observed in both patients. Dosing intervals for denosumab varied significantly between the two patients, depending on disease activity at baseline. Denosumab appears to be effective in reducing bone turnover in adult patients with active FD. However, caution should be exercised, and patients should be monitored carefully as significant fluctuations in biochemical and hormonal indices can occur.
- Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA.Osteoporos Int.2014 Feb;25(2):777-82. doi: 10.1007/s00198-013-2585-1. Epub 2013 Dec 6.
- We report on the clinical and biochemical outcomes in two adult patients with active polyostotic fibrous dysplasia (FD) treated with the RANK-L inhibitor, denosumab, following unsatisfactory responses to prior long-term bisphosphonate therapy. A 44-year-old female (case 1) who had received a cumulat
- PMID 24311113
- Primary atlantoaxial bone tumors in children: management strategies and long-term follow-up.
- Menezes AH, Ahmed R.Author information Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa.AbstractObject Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up. Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010. Results Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2-18 years). Chordomas received proton or LINAC irradiation, and as of 4-15 years of follow-up, no recurrence has been noted. Conclusions Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.
- Journal of neurosurgery. Pediatrics.J Neurosurg Pediatr.2014 Jan 17. [Epub ahead of print]
- Object Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine
- PMID 24437986
- Clinical and Radiological Observations in a Case Series of 26 Patients with Fibrous Dysplasia.
- Thomsen MD, Rejnmark L.Author information Department of Endocrinology and Internal Medicine (MEA), Aarhus University Hospital, Tage Hansens Gade 2, 8000, Aarhus C, Denmark, firstname.lastname@example.org.AbstractFibrous dysplasia (FD) is a rare disease caused by a sporadic postzygotic missense mutation that leads to abnormal fibroblast proliferation, defective osteoblast differentiation, and increased bone resorption. It may affect one or several bones. Both the mono-ostotic and polyostotic types may be associated with hyperfunctional endocrinopathies and hyperpigmentation of the skin (café-au-lait spots) in the so-called McCune-Albright syndrome (MAS). Due to its rarity, only a few case series are available, making it difficult for most clinicians to judge the severity of the disease. To improve our knowledge of FD, we reviewed all cases of FD treated at our department of endocrinology. Among 26 patients, 17 (65 %) had polyostotic FD, with four being diagnosed with MAS. Patients with polyostotic FD were diagnosed at an earlier age (median 13, range 0.5-64 years) than patients with the mono-ostotic form (median 21, 1-70 years). Craniofacial bones were affected in 80 % of cases, and 66 % complained of bone pain at the affected site, with no difference between patients with mono- and polyostotic FD. Two patients with mono-ostotic and three with polyostotic FD had cranial nerve impairments. Fourteen underwent, at least, one surgery, six (67 %) with mono-ostotic and eight (47 %) with polyostotic FD. Most received treatment with bisphosphonates, but therapy did not result in any clear relief of symptoms or radiological improvements. In conclusion, bone pain is common in patients with FD. The severity of the disease depends on affected bones as mono-ostotic may be as debilitating as polyostotic FD. In our case series, bisphosphonate treatment did not show clear beneficial effects.
- Calcified tissue international.Calcif Tissue Int.2014 Jan 4. [Epub ahead of print]
- Fibrous dysplasia (FD) is a rare disease caused by a sporadic postzygotic missense mutation that leads to abnormal fibroblast proliferation, defective osteoblast differentiation, and increased bone resorption. It may affect one or several bones. Both the mono-ostotic and polyostotic types may be ass
- PMID 24390518
- 症例 肋骨発生の線維性骨異形成症の1例
- 吉田 光輝,日野 弘之,阿部 秀一 [他]
- 胸部外科 65(5), 423-426, 2012-05
- NAID 40019302819
- 久保田 一政,糟谷 周吾,田村 高子,深山 治久,鈴木 康之
- 日本歯科麻酔学会雑誌 40(2), 173-178, 2012-04-15
- NAID 10030554850
- A Case of Sphenoid Fibrous Dysplasia Compressing the Orbital Apex
- 松永 麻美,中川 隆之,荻野 枝里子,伊藤 壽一
- 耳鼻咽喉科臨床 105(10), 951-955, 2012
- … Fibrous dysplasia is a benign proliferative bone disorder and sometimes involves the craniofacial bone including the skull base and orbit. … Image examinations revealed fibrous dysplasia arising from the sphenoid, which compressed the right orbital apex. …
- NAID 130002141422
- dysplasia /dys·pla·sia/ (dis-pla´zhah) 1. abnormality of development. 2. in pathology, alteration in size, shape, and organization of adult cells.dysplas´tic anhidrotic ectodermal dysplasia an inherited disorder characterized by ...
- Secondary aneurysmal bone cyst in fibrous dysplasia of the proximal femur. Orthopedics. 2009 May; 32(5):363. [Orthopedics. 2009] [Abnormal expression of c-myc, p53, p16 protein and GNAS1 gene mutation in fibrous Review ...
|リンク元||「線維性骨異形成」「骨の線維性異形成」「osteitis fibrosa disseminata」|
- fibrous dysplasia、fibrous dysplasia of bone
- 線維性骨形成異常 fibrous bone dysplasia、繊維性骨異形成症、線維性骨異形成症、骨の線維性異形成、播種性線維性骨炎
- fibrous dysplasia of bone
- castanets, clappers, finger cymbals