Abstract
Summary
To determine the prevalence, distribution, age-related changes and treatment of pain in fibrous dysplasia, we studied 78 children and adults. Pain was common, more prevalent and intense in adults, sometimes requiring narcotic analgesia. It was often untreated, especially in children, and surprisingly severity did not correlate with skeletal disease burden.
Introduction
Pain is common in fibrous dysplasia (FD), but relatively unstudied. We studied a well-characterized population of patients with a spectrum of disease.
Methods
Thirty-five children (16 male, 19 female, mean age 11.4 (range 5–18)) and 43 adults (15 male, 28 female, 23–62 yrs, mean age 40.3 (range 23–62)) were studied. Bone scans were used to identify the location and extent of disease. The Brief Pain Inventory was used to determine severity.
Results
Pain at sites of FD was common, reported by 67% of the population, but more prevalent and severe in the adult group than the children (81% and 49%, respectively p < 0.005, severity 4.1/10, and 2.8/10, respectively, p < 0.01). Surprisingly, there was no correlation between pain severity and skeletal disease burden. Children were more likely than adults to be untreated for pain (44% vs. 26%).
Conclusions
Pain, which was sometimes severe, was common in subjects with FD. It was often un- or under-treated, especially in children. The prevalence and severity of pain was greater in the adult group, but unrelated to the burden of FD.
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References
Collins MT, Bianco P (2003) Fibrous dysplasia. In: Favus MJ (ed) Primer on the metabolic bone diseases and disorders of mineral metabolism. American Society for Bone and Mineral Research, Washington, DC, pp 466–470
Bianco P, Robey PG, Wientroub S (2003) Fibrous dysplasia. In: Glorieux FH, Pettifor J, Juppner H (eds) Pediatric bone: biology and disease. Academic Press, Elsevier, New York, NY, pp 509–539
Bianco P, Riminucci M, Majolagbe A, Kuznetsov SA, Collins MT, Mankani MH, Corsi A, Bone HG, Wientroub S, Spiegel AM, Fisher LW, Robey PG (2000) Mutations of the GNAS1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune-Albright fibrous dysplasia of bone. J Bone Miner Res 15:120–128
Weinstein LS, Shenker A, Gejman PV, Merino MJ, Friedman E, Spiegel AM (1991) Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med 325:1688–1695
Shenker A, Chanson P, Weinstein LS, Chi P, Spiegel AM, Lomri A, Marie PJ (1995) Osteoblastic cells derived from isolated lesions of fibrous dysplasia contain activating somatic mutations of the Gs alpha gene. Hum Mol Genet 4:1675–1676
Schwindinger WF, Francomano CA, Levine MA (1992) Identification of a mutation in the gene encoding the alpha subunit of the stimulatory G protein of adenylyl cyclase in McCune-Albright syndrome. Proc Natl Acad Sci USA 89:5152–5156
Lichtenstein L (1938) Polyostotic fibrous dysplasia. Arch Surg 36:874–898
Lichtenstein L, Jaffe HL (1942) Fibrous dysplasia of bone: a condition affecting one, several or many bones, graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Path 777–816
Feuillan PP (1997) McCune-Albright syndrome. Curr Ther Endocrinol Metab 6:235–239
Mastorakos G, Mitsiades NS, Doufas AG, Koutras DA (1997) Hyperthyroidism in McCune-Albright syndrome with a review of thyroid abnormalities sixty years after the first report. Thyroid 7:433–439
Akintoye SO, Chebli C, Booher S, Feuillan P, Kushner H, Leroith D, Cherman N, Bianco P, Wientroub S, Robey PG, Collins MT (2002) Characterization of gsp-mediated growth hormone excess in the context of McCune-Albright syndrome. J Clin Endocrinol Metab 87:5104–5112
Collins MT, Chebli C, Jones J, Kushner H, Consugar M, Rinaldo P, Wientroub S, Bianco P, Robey PG (2001) Renal phosphate wasting in fibrous dysplasia of bone is part of a generalized renal tubular dysfunction similar to that seen in tumor-induced osteomalacia. J Bone Miner Res 16:806–813
McCune DJ (1936) Osteitis fibrosa cystica: the case of a nine-year-old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism. Am J Dis Child 743–744
Albright F, Butler AM, Hampton AO, Smith P (1937) Syndrome characterized by osteitis fibrosa disseminata, areas, of pigmentation, and endocrine dysfunction, with precocious puberty in females: report of 5 cases. N Engl J Med 216:727–746
Leet AI, Chebli C, Kushner H, Chen CC, Kelly MH, Brillante BA, Robey PG, Bianco P, Wientroub S, Collins MT (2004) Fracture incidence in polyostotic fibrous dysplasia and the McCune-Albright syndrome. J Bone Miner Res 19:571–577
Chapurlat R, Meunier PJ (1998) Bisphosphonates and bone remodeling: effectiveness in Paget’s disease, fibrous dysplasia and osteoporosis. Rev Chir Orthop Reparatrice Appar Mot 84:743–751
Plotkin H, Rauch F, Zeitlin L, Munns C, Travers R, Glorieux FH (2003) Effect of pamidronate treatment in children with polyostotic fibrous dysplasia of bone. J Clin Endocrinol Metab 88:4569–4575
DiCaprio MR, Enneking WF (2005) Fibrous dysplasia. Pathrophysiology, evaluation, and treatment. J Bone Joint Surg Am 87:1848–1864
Ippolito E, Bray EW, Corsi A, De Maio F, Exner UG, Robey PG, Grill F, Lala R, Massobrio M, Pinggera O, Riminucci M, Snela S, Zambakidis C, Bianco P (2003) Natural history and treatment of fibrous dysplasia of bone: a multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society. J Pediatr Orthop B 12:155–177
Kelly MH, Brillante B, Kushner H, Gehron Robey P, Collins MT (2005) Physical function is impaired but quality of life preserved in patients with fibrous dysplasia of bone. Bone 37:388–394
McCaffery M, Pasero C (1999) Pain clinical manual. Mosby, Inc, St Louis
Daut RL, Cleeland CS, Flanery RC (1983) Development of the Wisconsin brief pain questionnaire to assess pain in cancer and other diseases. Pain 17:197–210
Collins MT, Kushner H, Reynolds JC, Chebli C, Kelly MH, Gupta A, Brillante B, Leet AI, Riminucci M, Robey PG, Bianco P, Wientroub S, Chen CC (2005) An instrument to measure skeletal burden and predict functional outcome in fibrous dysplasia of bone. J Bone Miner Res 20:219–226
Keller S, Bann CM, Dodd SL, Schein J, Mendoza TR, Cleeland CS (2004) Validity of the brief pain inventory for use in documenting the outcomes of patients with noncancer pain. Clin J Pain 20:309–318
Tan G, Jensen MP, Thornby JI, Shanti BF (2004) Validation of the brief pain inventory for chronic nonmalignant pain. J Pain 5:133–137
Mendoza T, Mayne T, Rublee D, Cleeland C (2005) Reliability and validity of a modified brief pain inventory short form in patients with osteoarthritis. Eur J Pain
Walco G, Ilowite, NT (1991) Vertical versus horizontal visual analogue scales of pain intensity in children. Journal of Pain Symptom Management 6
McGrath P (1990) Pain in children. The Guilford Press, New York
Schechter NL (2003) Pain in infants, children and adolescents. Lippincott Williams & Wilkins, Philadelphia
Dorfman HD, Czerniak, B (1998) Fibroosseous lesions. In: Dorfman HD, Czerniak B (eds) Bone tumors. Mosby, St. Louis, pp 441–491
Brunner HI, Klein-Gitelman MS, Miller MJ, Trombley M, Baldwin N, Kress A, Johnson AL, Barron AC, Griffin TA, Passo MH, Lovell DJ (2004) Health of children with chronic arthritis: relationship of different measures and the quality of parent proxy reporting. Arthritis Rheum 51:763–773
Kim H, Neubert JK, Rowan JS, Brahim JS, Iadarola MJ, Dionne RA (2004) Comparison of experimental and acute clinical pain responses in humans as pain phenotypes. J Pain 5:377–384
Berkley KJ (1997) Sex differences in pain. Behav Brain Sci 20:371–380; discussion 435–513
Raj PP (2000) Practical management of pain. Mosby, Inc, St Louis
Howard RF (2003) Current status of pain management in children. Jama 290:2464–2469
Chapurlat R, Delmas PD, Liens D, Meunier PJ (2002) Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. J Bone Miner Res 10:1746–1752
Parisi MS, Oliveri MB, Gomez Acotto C, Mautalen C (2001) Intravenous pamidronate increases bone mineral density and reduces bone remodeling markers in fibrous dysplasia. Bone 29:300–301
Zacharin M, O’Sullivan M (2000) Intravenous pamidronate treatment of polyostotic fibrous dysplasia associated with the McCune Albright syndrome. J Pediatr 137:403–409
Lala R, Matarazzo P, Bertelloni S, Buzi F, Rigon F, de Sanctis C (2000) Pamidronate treatment of bone fibrous dysplasia in nine children with McCune-Albright syndrome. Acta Paediatr 89:188–193
Liens D, Delmas PD, Meunier PJ (1994) Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet 343:953–954
Acknowledgement
This study was supported by the Division of Intramural Research of the National Institute of Dental and Craniofacial Research, Intramural Research Program, National Institutes of Health, Department of Health and Human Services.
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Kelly, M.H., Brillante, B. & Collins, M.T. Pain in fibrous dysplasia of bone: age-related changes and the anatomical distribution of skeletal lesions. Osteoporos Int 19, 57–63 (2008). https://doi.org/10.1007/s00198-007-0425-x
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DOI: https://doi.org/10.1007/s00198-007-0425-x