Abstract:
:Treatment of moderate and severe forms of osteogenesis imperfecta (OI) with cyclic pamidronate at the Reference Center for OI Treatment in Southern Brazil was studied. A retrospective cohort study was conducted from 2002 to 2012. Data were obtained during inpatient (drug infusion) and outpatient care. Clinical data, including the presence of blue sclerae, dentinogenesis imperfecta, history and site of the fractures, biochemical data, including calcium, phosphorus, and alkaline phosphatase levels, were systematically collected. Bone mineral density (BMD) was measured using dual energy X-ray absorptiometry (DXA). Forty-five patients (26 females) were included in the study, and the age of the patients at the time of diagnosis ranged from 1 to 144 months, with a median age (p25-p75) of 38 (5-96) months. Most cases presented OI-4 (51.1%), and the median age of the patients at the start of treatment was 3.3 years (25-75 percentiles: 0.5 - 8.7 years). Twenty-four patients (54.5%) had some adverse events or intercurrences during treatment, and the treatment compliance mean was 92.3% (± 10.7). The treatment with intravenous pamidronate has shown to be safe, well-tolerated, and effective in regard to the improvement of BMD and the reduction of the number of fractures in children and adolescents with OI.
journal_name
Genet Mol Bioljournal_title
Genetics and molecular biologyauthors
Pinheiro B,Zambrano MB,Vanz AP,Brizola E,Souza LT,Félix TMdoi
10.1590/1678-4685-GMB-2018-0097subject
Has Abstractpub_date
2019-01-01 00:00:00pages
252-260issue
1 suppl 1eissn
1415-4757issn
1678-4685pii
S1415-47572019005019401journal_volume
42pub_type
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journal_title:Genetics and molecular biology
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