Naslov
Our experience in bisphosphonate therapy for osteogenesis imperfecta

Autori
Huzjak, Nevenka ; Barišić, Ingeborg ; Rešić, Arnes ; Vrdoljak, Javor ; Kušec, Vesna ; Antičević, Darko ; Dodig, Damir ; Primorac, Dragan

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
8th International Conference on osteogenesis imperfecta : abstracts / - Annecy, 2002, P28-P28

Skup
International Conference on osteogenesis imperfecta (8 ; 2002)

Mjesto i datum
Annecy, Francuska, 01.09.2002. - 03.09.2002

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
osteogenesis imperfecta ; bisphosphonates

Sažetak
Severe osteogenesis imperfecta (OI) is a hereditary disorder characterised by increased bone fragility and progressive bone deformity. Secondary osteoporosis is an important feature of OI. So far, no effective medical treatment is available. Antiresorptive acitivity of the aminobisphosphonates may improve clinical outcome in children. Aim was to assess the clinical impact of the administration of bisphosphonates in Croatian OI patients. We introduced therapy in 1998 encouraged by parents from Croatian Society of OI (HUOI). Here we report results of 1-4 years treatment with intravenous pamidronate (APD) in ten children (four girls) of age 3 months - 11 years at entry, with severe OI. Pamidronate was administered in cycles as monthly infusions at a daily dose of 1-1, 5 mg/kg during 6 months following pause for three months, or the same dose for three days every four months. All took 500-1000 mg calcium from food or supplements and 1000 IU of vitamin D daily. During treatment DEXA measurements showed a gradual increase in bone denisity in all paitents. Number of confirmed fractures decreased in all. The reduction in pain and imporvement in well-being and ability were impressive in two boys who had been confined to a wheelchair and now they walk using crutches. Well-known acute phase reactors were noted during first infusion cycle in two children and asymptomatic hypocalcaemia in three children. During the treatment three children gained weight compared to their height. Although the bisphosphonates do not correct basic abnormality in OI, they significantly alter the natural course of the disease and improve patients quality of life. For the time being they seem not only effective but also devoid of any adverse effects on bone growth and remodelling.

Izvorni jezik
Engleski

Znanstvena područja
Javno zdravstvo i zdravstvena zaštita

POVEZANOST RADA