Endocrinology Research and Practice
Case Report

Osteogenesis Imperfecta in Adult Twins Responded To Treatment With Pamidronate

1.

Selçuk University, School of Medicine, Endocrinology and Metabolism, Konya, Turkey

2.

Selçuk Üniversitesi Meram Tıp Fakültesi, Endokrinoloji ve Metabolizma Bilim Dalı, Konya, Türkiye

Endocrinol Res Pract 2011; 15: 39-43
Read: 2083 Downloads: 565 Published: 01 June 2011

ABSTRACT

Bisphosphonates are strong inhibitors of bone resorption and are used in the treatment of osteoporosis. Bisphosphonates are known to be effective in prevention of fractures, improvement of bone mineral density as well as in relieving bone pain in osteogenesis imperfecta (OI) patients. Recent studies have shown that especially intravenous pamidronate may be more effective when given in childhood and adolescence. This effect was also shown in adult OI patients in some clinical trials.
22-year-old twin brothers known to have OI were admitted to our endocrinology and metabolism outpatient clinic. On medical history, OI was diagnosed at the age of three and for the last eight years, they were not able to walk and were using wheelchairs. On physical examination, blue sclerae and dentinogenesis imperfecta were detected in both patients. According to the expanded Sillence classification of OI, the clinical findings were consistent with type IV OI.  Intravenous pamidronate treatment was given three times at four-month intervals, according to Montreal protocol. During this period, the patients were also doing isometric exercises and were on physical therapy, diet, and bioresonance therapy.
At the end of one year, bone pain regressed significantly in both patients and they were able to walk independently. These outcomes demonstrate that in selected adult OI patients, intravenous pamidronate treatment may be beneficial in preventing bone fractures and relieving pain. 

 

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EISSN 2822-6135