Agha Shabbir Ali, Sadia Saeed, Yasser Masood, Nayla Asghar, Aamir Rashid, Mahwish Faizan, Fatima Sajjad, Abid Ali Qureshi, Javed Asghar, Farooq Rasool.
Bisphosphonate therapy improves biochemical, radiological and clinical parameters in children with osteogenesis imperfecta.
Pak Paed J Jan ;34(3):148-53.

Background: Osteogenesis imperfacta is the most common congenital osteoporotic disease of connective tissue characterized by fractures and deformities. There is increasing use of pamidronate for symptomatic relief in these children. We intend to demonstrate the clinical, biochemical and radiological effects of pamidronate in moderate to severe Osteogenesis imperfacta. Subjects & Methods: In this observational uncontrolled study 30 children from 1 month to 17 years age were enrolled and Inj. Pamidronate as infusion was given to children with moderate to severe disease. Dose used was 0.5 mg / kg/ day for children ? 3 years and 1mg/kg/day for older ? 3 years age. It was given on three consecutive days per month for three years. Clinical status, biochemical changes, Bone Mineral Density (BMD) and radiological changes were assessed during treatment. Results: Sustained reduction in serum alkaline phosphatas was observed in all children. BMD was increased in all children but the increase was more marked in younger children (increased from 0.1865 ± 0.05 to 0.3773) than older children (increased from 0.3383 ± 0.02 to 0.5121 ± 0.03). Mean coronal area increased from 14.85 to 19.19 suggesting new bone formation. T score improved from mean of - 5.56 to – 4.42 both results were again more pronounced in younger children than older patient. Healing rate of bones did not change. But incidence of fracture was markedly reduced from average of 3.25 ± 1.3 to 1.05 ± 0.4 after pamidronate therapy. Relief of fatigue and bone pains was a consistent finding in all children and resulted in improvement in ambulation grade in fourteen children treated by bisphosphonate.

PakMediNet -Pakistan's largest Database of Pakistani Medical Journals - http://www.pakmedinet.com