Avaliação da resposta à teriparida em pacientes portadores de osteoporose pseudoglioma. / Evaluation of response to teriparida in patients with osteoporosis pseudogliom.

AUTOR(ES)
FONTE

IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia

DATA DE PUBLICAÇÃO

24/11/2011

RESUMO

INTRODUCTION: Osteoporosis Pseudoglioma (OPPG) is characterized by severe juvenile-onset osteoporosis and ocular abnormalities. It is caused by one of several inactivating mutations in LRP5, a gene importantly involved in bone formation. OBJECTIVE: The objective of this study was to evaluate the efficacy of teriparatide in a young man with OPPG. PATIENT AND METHOD: The subject of this case report is a 19-year-old man with congenital blindness and low trauma fractures due to OPPG. A 2-year course of teriparatide, 20 mcg/day, was initiated after a 6-year course of intravenous pamidronate infusions, the latter 3 years of which had minimal effects on bone mineral density (BMD). Measurements in serum were made of C-terminal telopeptide of type I collagen (CTX), N-terminal propeptide of type I collagen (P1NP), total and ionized calcium, phosphate, uric acid, complete blood count, renal and liver function tests. Urinary calcium/creatinine ratio was determined. BMD was measured by DXA yearly. RESULTS: BMD increased by 9.7% in lumbar BMD and 10.2% in right femur hip. CTX rose early, peaking in month 3, followed by an increase in P1NP, peaking in month 9. Both indices returned to baseline by month 24. The increase in CTX followed by P1NP is an unusual time course when teriparatide is used to treat osteoporosis but may be typical of low bone turnover states. There were no adverse events. CONCLUSION: In a patient with OPPG, teriparatide markedly increased BMD in the lumbar spine and femur hip.

ASSUNTO(S)

teriparatida, osteoporose/terapia, resultado de tratamento, serotonina endocrinologia pth (1-34), bone turnover markers, bone mineral density, lrp5 mutation.

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