ICCBH2017 Poster Presentations (1) (209 abstracts)
Long term treatment with intravenous pamidronate in two children with severe form of juvenile Paget's disease
Katja Verfürth 1, , Matthias Hövel 3, , Michael Schündeln 2 , Jens Bauer 1, & Corinna Grasemann 1,
1Pediatric Endocrinology, Universitätsklinikum Essen, Essen, Germany; 2Pediatric Hematology and Oncology, Universitätsklinikum Essen, Essen, Germany; 3Center for Rare Bone Disease, Universitätsklinikum Essen, Essen, Germany; 4Department for Orthopaedics and Emergency Surgery, Universitätsklinikum Essen, Essen, Germany.
Presenting problem: Severe forms of juvenile Paget’s Syndrome (JPD) result in extreme bone turnover, necessitating long-term treatment with anti-resorptive drugs to control for bone pain and modeling of bones.
Objective: To report clinical and biochemical effects of intravenous treatment with Pamidronate in two children with severe forms of JPD over a time period of 3 and 9 years, respectively. Treatment was commenced at 12 months and 3 years of age, respectively.
Clinical management: Over time, doses of Pamidronate and infusion intervals were adjusted according to the presence of bone pain, bone turnover markers and bodyweight. Yearly doses ranged from 5.5 mg/kg per year to 9 mg/kg per year and infusion intervals varied from 3 monthly to 5 weekly. Growth, motoric development (developmental scale), occurrence of fractures, bone turnover markers and bone pain were recorded.
Conclusion: Individualized intravenous treatment with Pamidronate resulted in sufficient control of bone pain and suppression of bone turnover markers, with few side effects. At present, both children are fully ambulatory. Signs of bone pain present about 6 weeks after each treatment in the patients. Motoric development was delayed in both children, but improved significantly with treatment.
Disclosure: The authors declared no competing interests.
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