title: Fibrous dysplasia & McCune-Albright syndrome: an experience from a tertiary care centre in north India.
abstract: OBJECTIVE
  Fibrous dysplasia (FD) is a rare metabolic bone disease and information available from India is limited to only anecdotal case reports. We describe the clinical profile and therapeutic outcome of 25 patients with FD observed over a period of 14 yr in a tertiary care centre from north India.
  
  
  METHODS
  In this retrospective study patients (n = 25) with diagnosis of fibrous dysplasia based on either classical radiological features and/or histological evidence on bone biopsy, were analyzed. Associated endocrinopathies if any, were evaluated. The diagnosis of McCune Albright syndrome (MAS) was considered when fibrous dysplasia was accompanied by either café-au-lait macules and/or endocrinopathies. The clinical presentation, biochemical parameters and imaging were analysed. Seven patients received bisphosphonate therapy. The final outcome and side effects were noted.
  
  
  RESULTS
  Age of the patients ranged from 7 to 48 yr (mean ± SD, 24.2 ± 11.4 yr) with a lag time between onset of symptoms and presentation ranging from 1 to 20 yr (mean ± SD, 6.6 ± 6.2 yr). The mean duration of follow up was 3.5 ± 2.1 yr. Eighteen (72%) patients had polyostotic disease while the remaining had monostotic FD. Eight patients had endocrinopathies: five had acromegaly, one each had gonadotropin independent precocious puberty (GIPP), hyperthyroidism and hypophosphatemic rickets. One child with GIPP later developed hyperthyroidism. McCune Albright syndrome was observed in 10 (40%) patients. A majority of the patients underwent various minor or major surgical procedures and seven patients received bisphosphonates for recurrent pathological fractures. Bone pain was reduced in all bisphosphonate treated patients with a decrease in subsequent fractures.
  
  
  CONCLUSIONS
  This series of FD patients from north India shows the varying presentations of this rare disease. Medical treatment with bisphosphonates appears to be potentially rewarding.
authors.affiliation: Department of Endocrinology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
authors.forename: Sanjay Kumar
authors.initials: SK
authors.lastname: Bhadada
authors.forename: Anil
authors.initials: A
authors.lastname: Bhansali
authors.forename: Sambit
authors.initials: S
authors.lastname: Das
authors.forename: Ramanbir
authors.initials: R
authors.lastname: Singh
authors.forename: R
authors.initials: R
authors.lastname: Sen
authors.forename: A
authors.initials: A
authors.lastname: Agarwal
authors.forename: B R
authors.initials: BR
authors.lastname: Mittal
authors.forename: Uma
authors.initials: U
authors.lastname: Nahar
authors.forename: Pinaki
authors.initials: P
authors.lastname: Dutta
authors.forename: Niranjan
authors.initials: N
authors.lastname: Khandelwal
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004164:Diphosphonates / Q000627:therapeutic use*; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007194:India / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D004164:Diphosphonates