HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Fibrous dysplasia & McCune-Albright syndrome: an experience from a tertiary care centre in north India.

AbstractBACKGROUND & OBJECTIVES:
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.
INTERPRETATION & 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.
AuthorsSanjay Kumar Bhadada, Anil Bhansali, Sambit Das, Ramanbir Singh, R Sen, A Agarwal, B R Mittal, Uma Nahar, Pinaki Dutta, Niranjan Khandelwal
JournalThe Indian journal of medical research (Indian J Med Res) Vol. 133 Pg. 504-9 (May 2011) ISSN: 0971-5916 [Print] India
PMID21623035 (Publication Type: Journal Article)
Chemical References
  • Diphosphonates
Topics
  • Adolescent
  • Adult
  • Child
  • Diphosphonates (therapeutic use)
  • Female
  • Fibrous Dysplasia, Polyostotic (drug therapy, epidemiology, pathology, surgery)
  • Humans
  • India (epidemiology)
  • Male
  • Middle Aged
  • Retrospective Studies
  • Treatment Outcome

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: