Naslov
Bisphosphonate therapy in patients with osteogenesis imperfecta

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

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

Izvornik
29th European symposium on calcified tissues : Abstracts ; u: Calcified Tissue International 70 (2002)(4) 242-292 / - , 2002, 242-242

Skup
European symposium on calcified tissues (29 ; 2002)

Mjesto i datum
Zagreb, Hrvatska, 25.05.2002. - 29.05.2002

Vrsta sudjelovanja
Poster

Vrsta recenzije
Nije recenziran

Ključne riječi
osteogenesis imperfecta ; bisphosphonate therapy

Sažetak
Severe osteogenesis imperfecta (OI) is a hereditary disorder characterised by increased bone fragility and progressive bone deformity. Secondary osteoporosis is and important feature of OI. So far, no effective medical treatment is available. Antiresorptive activity of the aminobisphosphonates may improve clinical outcome in children. 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 1-3 years treatment with intravenous pamidronate (APD) in seven children with severe OI, 4 female and 3 male with age distribution ranging from 3 months to 11 years at the entry. Pamidronate was administered either in the form of monthly infusions at a daily dose of 1-1.5 mg/kg during a period of 6 months followed by a pause of three months, or in the form of three daily infusions every four months, the administered dose being the same. All patients received 500-1000 mg of calcium and 1000 IU of vitamin D daily. During treatment, DEXA measurements showed a gradual increase of bone density. Number of confirmed fractures decreased in all patients. The reduction in pain as well as improvement in well-being and ability were impressive in two male patients who had been confined to a wheelchair, but now they are able to walk using crutches. Well-known acute phase reactions were noted in two children during first infusion cycle and asymptomatic hypocalcaemia was noted in three children. during the treatment body mass index of three children was significantly increased. Although the bisphosphonates do not correct basic abnormalities 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
Kliničke medicinske znanosti

POVEZANOST RADA