WordNet

imposing in scale or scope or degree or power; "massive retaliatory power"; "a massive increase in oil prices"; "massive changes"
imposing in size or bulk or solidity; "massive oak doors"; "Moores massive sculptures"; "the monolithic proportions of Stalinist architecture"; "a monumental scale" (同)monolithic, monumental
being the same substance throughout; "massive silver"
consisting of great mass; containing a great quantity of matter; "Earth is the most massive of the terrestrial planets"
lysis of bone caused by disease or infection or inadequate blood supply

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『大きくて重い』,どっしりした;大規模な / (容ぼう,特に頭が)がっちりした / (精神などが)しっかりした,堂々とした / (投薬量が)定量以上の

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1. ランゲルハンス細胞組織球症の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of langerhans cell histiocytosis
2. 小児における末梢のリンパ節腫脹 peripheral lymphadenopathy in children etiology
3. 軟組織および骨に影響を与えるさまざまな良性疾患に対する抗腫瘍療法 antineoplastic therapy for miscellaneous benign diseases affecting soft tissue and bone
4. 原発性骨髄線維症の臨床症状および診断 clinical manifestations and diagnosis of primary myelofibrosis
5. 非ホジキンリンパ腫の評価、病期分類、治療反応性の評価 evaluation staging and response assessment of non hodgkin lymphoma

English Journal

Viewpoints on vessels and vanishing bones in Gorham-Stout disease.

Dellinger MT1, Garg N2, Olsen BR3.Author information 1Division of Surgical Oncology, Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA; The Lymphatic Malformation Institute, Bethesda, MD, USA. Electronic address: mdellinger@lmiresearch.org.2The Lymphatic Malformation Institute, Bethesda, MD, USA.3Department of Developmental Biology, Harvard School of Dental Medicine, Boston, MA, USA. Electronic address: bjorn_olsen@hms.harvard.edu.AbstractGorham-Stout disease (GSD) is a rare disorder characterized by the proliferation of endothelial-lined vessels in bone and the progressive destruction of bone. Although Jackson described the first case of GSD in 1838, the clinical and histological features of GSD were not defined until Gorham and Stout published their report on massive osteolysis in 1955. In the years since Gorham and Stout's groundbreaking publication, more than 300 cases of GSD have been described in the literature. These reports have revealed that the progressive resorption of bone in GSD causes severe physical deformities, disabilities, and life-threatening complications. Unfortunately, the underlying cause of GSD remains unknown and, as a result, the therapeutic options for individuals with GSD are limited. Here we review the latest advances in GSD research and present strategies to address basic and clinical research questions related to GSD.
Bone.Bone.2014 Jun;63C:47-52. doi: 10.1016/j.bone.2014.02.011. Epub 2014 Feb 26.
Gorham-Stout disease (GSD) is a rare disorder characterized by the proliferation of endothelial-lined vessels in bone and the progressive destruction of bone. Although Jackson described the first case of GSD in 1838, the clinical and histological features of GSD were not defined until Gorham and Sto
PMID 24583233

RANKL inhibitors induce osteonecrosis of the jaw in mice with periapical disease.

Aghaloo TL1, Cheong S, Bezouglaia O, Kostenuik P, Atti E, Dry SM, Pirih FQ, Tetradis S.Author information 1Division of Diagnostic and Surgical Sciences, School of Dentistry, University of California, Los Angeles (UCLA), Los Angeles, CA, USA.AbstractAntiresorptive medications are essential in treating diseases of pathologic osteoclastic bone resorption, including bone cancer and osteoporosis. Bisphosphonates (BPs) are the most commonly used antiresorptives in clinical practice. Although inhibition of bone resorption is important in regulating unwanted malignant and metabolic osteolysis, BP treatment is associated with potential side effects, including osteonecrosis of the jaws (ONJ). Recently, non-BP antiresorptive medications targeting osteoclastic function and differentiation, such as denosumab, have entered the clinical arena. Denosumab treatment results in a similar rate of ONJ as BPs. Animal models of ONJ, using high-dose BP treatment in combination with tooth extraction or dental disease, provide valuable tools and insight in exploring ONJ pathophysiology. However, the ability of other antiresorptives to induce ONJ-like lesions in animal models has not been explored. Such studies would be beneficial in providing support for the role of osteoclast inhibition in ONJ pathogenesis versus a direct BP effect on oral tissues. Here, we tested the ability of the receptor activator of NF-κB ligand (RANKL) inhibitors RANK-Fc (composed of the extracellular domain of RANK fused to the fragment crystallizable [Fc] portion of immunoglobulin G [IgG]) and OPG-Fc (composed of the RANKL-binding domains of osteoprotegerin [OPG] linked to the Fc portion of IgG) to induce ONJ in mice in the presence of periapical disease, but in the absence of dental extractions. We demonstrate radiographic evidence of ONJ in RANK-Fc-treated and OPG-Fc-treated mice, including inhibition of bone loss, increased bone density, lamina dura thickening, and periosteal bone deposition. These findings closely resembled the radiographic appearance of an ONJ patient on denosumab treatment. Histologic examination revealed that RANK-Fc treatment and OPG-Fc treatment resulted in absence of osteoclasts, periosteal bone formation, empty osteocytic lacunae, osteonecrosis, and bone exposure. In conclusion, we have successfully induced ONJ in mice with periapical disease, using potent osteoclast inhibitors other than BPs. Our findings, coupled with ONJ animal models using high-dose BPs, suggest that osteoclast inhibition is pivotal to the pathogenesis of ONJ.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.J Bone Miner Res.2014 Apr;29(4):843-54. doi: 10.1002/jbmr.2097.
Antiresorptive medications are essential in treating diseases of pathologic osteoclastic bone resorption, including bone cancer and osteoporosis. Bisphosphonates (BPs) are the most commonly used antiresorptives in clinical practice. Although inhibition of bone resorption is important in regulating u
PMID 24115073

Panostotic expansile bone disease with massive jaw tumor formation and a novel mutation in the signal peptide of RANK.

Schafer AL1, Mumm S, El-Sayed I, McAlister WH, Horvai AE, Tom AM, Hsiao EC, Schaefer FV, Collins MT, Anderson MS, Whyte MP, Shoback DM.Author information 1Department of Medicine, University of California, San Francisco, CA, USA; Endocrine Research Unit, Department of Veterans Affairs Medical Center, San Francisco, CA, USA.AbstractPrecise regulation of bone resorption is critical for skeletal homeostasis. We report a 32-year-old man with a panostotic expansile bone disease and a massive hemorrhagic mandibular tumor. Originally from Mexico, he was deaf at birth and became bow-legged during childhood. There was no family history of skeletal disease. Puberty occurred normally, but during adolescence he experienced difficulty straightening his limbs, sustained multiple fractures, and developed a bony tumor on his chin. By age 18 years, all limbs were misshapen. The mandibular mass grew and protruded from the oral cavity, extending to the level of the lower ribs. Other bony defects included a similar maxillary mass and serpentine limbs. Upon referral at age 27 years, biochemical studies showed serum alkaline phosphatase of 1760 U/L (Nl: 29-111) and other elevated bone turnover markers. Radiography of the limbs showed medullary expansion and cortical thinning with severe bowing. Although the jaw tumors were initially deemed inoperable, mandibular mass excision and staged partial maxillectomy were eventually performed. Tumor histopathology showed curvilinear trabeculae of woven bone on a background of hypocellular fibrous tissue. Fibrous dysplasia of bone was suspected, but there was no mutation in codon 201 of GNAS in samples from blood or tumor. His clinical and radiographic findings, elevated serum markers, and disorganized bone morphology suggested amplified receptor activator of NF-κB (RANK) signaling, even though his disorder differed from conditions with known constitutive activation of RANK signaling (eg, familial expansile osteolysis). We found a unique 12-base pair duplication in the signal peptide of TNFRSF11A, the gene that encodes RANK. No exon or splice site mutations were found in the genes encoding RANK ligand or osteoprotegerin. Alendronate followed by pamidronate therapies substantially decreased his serum alkaline phosphatase activity. This unique patient expands the phenotypes and genetic basis of the mendelian disorders of RANK signaling activation.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.J Bone Miner Res.2014 Apr;29(4):911-21. doi: 10.1002/jbmr.2094.
Precise regulation of bone resorption is critical for skeletal homeostasis. We report a 32-year-old man with a panostotic expansile bone disease and a massive hemorrhagic mandibular tumor. Originally from Mexico, he was deaf at birth and became bow-legged during childhood. There was no family histor
PMID 24014458