Abstract
Although the etiology of Paget bone disease (PBD) is unknown, increasing evidence implicates a "slow virus" infection of the skeleton, perhaps in genetically predisposed individuals. PBD is rare in Asia. We describe a Korean family with PBD. The propositus noticed bowed limbs at approximately 25 years of age. Radiologic studies made when he was 55 years old revealed essentially panostotic PBD. Serum alkaline phosphatase (ALP) activity and osteocalcin (OC) levels were markedly elevated. An iliac crest specimen showed classic histopathologic changes of PBD. Additionally, palpable swellings were first observed at age 45 years at his occiput, pubic ramus, ileum, and facial bones. They contained numerous multinucleated cells and were originally diagnosed as giant cell tumors. However, we found that, like osteoclasts, these cells expressed considerable tartrate-resistant acid phosphatase activity. These "extraskeletal osteoclastomas" resolved rapidly with dexamethasone treatment. Two daughters, 20- and 24-years-of-age, were discovered by study of his 5 children to have elevated serum ALP activity and OC levels and widespread PBD. Both women, however, are without palpable masses and are asymptomatic. The propositus' father, who died a...Continue Reading
References
Jul 1, 1977·Transplantation·R J RubinJ T Harrington
Aug 1, 1979·Cancer·T P JacobsR E Canfield
Jun 24, 1978·British Medical Journal·M J GardnerD J Barker
Oct 8, 1976·Science·B G Mills, F R Singer
Jan 1, 1976·Tissue Antigens·P CullenJ Whiteley
May 1, 1992·Tissue Antigens·O Olerup, H Zetterquist
Jul 1, 1991·Radiology·H G PotterJ M Lane
May 1, 1991·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·E S SirisJ L Kelsey
Jul 1, 1990·Endocrinology·I M Dickerson, R E Mains
Oct 1, 1985·The Journal of General Virology·M F BasleR Filmon
Feb 1, 1988·The Journal of Experimental Medicine·A McInnes, D M Rennick
Mar 1, 1988·The Journal of Bone and Joint Surgery. British Volume·P H OsterbergP G Toner
Jan 9, 1986·The New England Journal of Medicine
Feb 1, 1985·The American Journal of Medicine·M P WhyteW A Murphy
Oct 14, 1967·British Medical Journal·J V Jones, M F Reed
Oct 1, 1982·Australian and New Zealand Journal of Medicine·M W TilyardR D Stewart
Jun 18, 1983·British Medical Journal·J C ReasbeckR D Stewart
Dec 1, 1984·Radiology·C J VellengaJ Hermans
Sep 1, 1983·Journal of Epidemiology and Community Health·J A SofaerA E Emery
Jan 1, 1984·Clinical Radiology·F SchubertJ S Harper
May 1, 1982·Calcified Tissue International·C Minkin
Jan 1, 1983·Annals of Internal Medicine·K S UpchurchS M Krane
Feb 1, 1981·Proceedings of the National Academy of Sciences of the United States of America·B G MillsP A Holst
Jan 1, 1981·Metabolic Bone Disease & Related Research·D J Barker
Apr 5, 1980·British Medical Journal·P B Guyer, A T Chamberlain
Oct 1, 1980·Arthritis and Rheumatism·P J MeunierM E Arlot
Jun 1, 1993·Journal of Medical Virology·E J CartwrightP T Sharpe
May 1, 1993·Current Opinion in Rheumatology·S J Gallacher
Jan 1, 1953·Journal of Oral Surgery·A BERGER, H L JAFFE
Jan 1, 1953·Oral Surgery, Oral Medicine, and Oral Pathology·H L JAFFE
Aug 1, 1963·Cancer·R V HUTTERK C FRANCIS
Citations
Apr 20, 2006·Journal of Bone and Mineral Metabolism·Jun HashimotoUNKNOWN Japanese Committee on Clinical Guidelines of Diagnosis and Treatment of Paget's Disease of Bone of the Japan Osteoporosis So
Nov 1, 2001·The Journal of Molecular Diagnostics : JMD·J D McNairnA E Shrimpton
Mar 29, 2002·Contact Dermatitis·S Schliemann-WillersP Elsner
Apr 27, 2011·Nature Reviews. Cardiology·Andrew J Stewart Coats
Feb 20, 2002·European Journal of Clinical Investigation·F TriposkiadisV Thanopoulos
Aug 3, 2002·European Journal of Clinical Investigation·S MandigoutP Obert
May 12, 2001·Journal of Applied Microbiology·L CelliniG Donelli
Oct 31, 2002·Clinical Physiology and Functional Imaging·E KouidiA Deligiannis
Mar 13, 2002·Medicine·Michael P WhyteBarbara G Mills
Jun 27, 2008·International Journal of Health Care Quality Assurance·Chris G Collins, Pauline Joyce
Dec 19, 2002·The Online Journal of Knowledge Synthesis for Nursing·Cynthia D. Adams, Susan Bennett
Dec 25, 2003·Public Health Nursing·Carolyn H Robinson, Sandra P Thomas
Jan 8, 2005·Acta Psychiatrica Scandinavica·H L I NijmanJ J Stolker
Aug 2, 2011·Applied Physiology, Nutrition, and Metabolism = Physiologie Appliquée, Nutrition Et Métabolisme·Darren E R WarburtonRoy J Shephard
Jul 12, 2007·Annals of Nutrition & Metabolism·Guihua ZhangEiji Shimizu
Apr 6, 2005·Medicine and Science in Sports and Exercise·Gregory R AdamsNosratola D Vaziri
Dec 29, 2000·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·M P WhyteW H McAlister
Apr 22, 2008·American Journal of Health Promotion : AJHP·Nicole E I SmithHeather A McKay
Jul 16, 2009·American Journal of Health Promotion : AJHP·Diana RedwoodMartha L Slattery
Mar 18, 2010·Archives of Osteoporosis·Vincenzo NuzzoNicola Brunetti-Pierri
Oct 1, 2011·Seminars in Arthritis and Rheumatism·Pui Yan Jenny Chung, Wim Van Hul
Jul 13, 2006·Annals of the New York Academy of Sciences·Michael P Whyte
Apr 8, 2006·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Adriana Rojas-VillarragaAntonio Iglesias-Gamarra
Jun 13, 2014·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Michael P WhyteNicholas J Shaw