RESUMO
UNLABELLED: Management of type III osteogenesis imperfecta (O.I.) (brittle bone disease) is primarily supportive; early introduction of cyclic intravenous pamidronate administration in children younger than 2 years of age is an innovative and promising therapeutic approach. CASE REPORT: We present the case of a 6-month-old infant, whose preliminary data have already been partly published, with severe type III O.I. referred because of aching and crumbling from multiple fractures. Cyclic intravenous disodic pamidronate administration improved the clinical status (fracture incidence, pain, growth curve) and biological status (bone density, osseous alkaline phosphatases, urinary desoxypiridoline excretion), allowing a remarkable recovery. CONCLUSION: Biphosphonates are a new and innovative therapeutic agent in O.I. Clinical safety, easy administration, and overall efficacy are likely to extend their use in severe type III O.I. from the very first months of life, the time of best efficacy.
Assuntos
Difosfonatos/uso terapêutico , Osteogênese Imperfeita/tratamento farmacológico , Fatores Etários , Fosfatase Alcalina/sangue , Densidade Óssea , Esquema de Medicação , Transtornos do Crescimento/etiologia , Humanos , Lactente , Infusões Intravenosas , Masculino , Osteogênese Imperfeita/classificação , Osteogênese Imperfeita/complicações , Osteogênese Imperfeita/diagnóstico por imagem , Osteogênese Imperfeita/metabolismo , Dor/etiologia , Pamidronato , Radiografia , Resultado do TratamentoRESUMO
Several unique effects of subinhibitory concentrations of C14 and C15 macrolides on Pseudomonas aeruginosa infection are described in vitro: P. aeruginosa virulence factors inhibition, bacterial biofilm disruption, P. glycoprotein expression upregulation, anti-inflammatory and immunoregulatory effects. Clinical trials in vivo are warranted to assess the potential usefulness of macrolides for treatment of chronic infections caused by P. aeruginosa.