Endocrine Abstracts

Prevalence of Paget’s disease (PD) of bone as well as clinical and demographical presentation may differ between the populations.

Aim: In this study we evaluate clinical and demographical parameters of Paget’s disease patients followed from endocrinology clinics in Turkey.

Method: An invitation was sent to all tertiary endocrinology clinics to complete a survey on demographic clinical, laboratory parameters, treatment modalities of patients with PD. Clinically or histologically proven 185 PD cases reported from 16 centers.

Results: A cohort of PD has female preponderance (FM/M:105/80) with a mean age of 57±10 years at onset. Its clinical features are bone pain, back pain and headache, Fracture and typical skeletal involvement reported in five and 18 patients respectively. Only two patients have family history for PD. 67.5% (n:125) patients have polyostotic disease. Skull (41.6%), pelvis (53.5%), spine (41%) and femur (25.4%) are being the commonly affected bone sites. Seventeen patients with skull involvement reported to have hearing loss. The biochemical profile at diagnosis had a mean alkaline phosphatase (ALP) 5520±652 IU/l (range 280–5762 IU/l), serum calcium: 9.2±1 mg/dl, iPTH:73.9±65 pg/ml, 25OHvitD:32.6±27 ng/ml. Five patients wasn’t need treatment. Intravenous bisphosphonates was the most commonly used drugs (42% and 23% of patines treated with zoledronic acid 5 mg and pamidronate 60–90 mg respectively) remaining given high dose oral bisphosphonate (alendronate 40 mg/day or risedronate). Most cases respond well with decreased Serum ALP level (117±114 IU/l) at the 6th month of the therapy. Duration of follow up was 7.5±6.5 years.

Conclusion: In this group of PD from Turkey female predominance and polyostotic disease, no significant family history. Classical clinical and biochemical features respond well to intravenous or high dose bisphosphonate therapy and need a lifetime follow-up.