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1.
Osteoporos Int ; 27(1): 81-92, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26138583

RESUMO

UNLABELLED: Treatment with zoledronic acid (ZA) over 2 years, among 33 children with osteogenesis imperfecta (OI) and five Bruck syndrome cases, showed reduction in fracture rates, pain, and improvement in bone mineral density (BMD) and motor milestones of development. This is the first study reporting the use of bisphosphonates in patients with Bruck syndrome (BS). INTRODUCTION: OI and BS are genetic disorders that result in bone fragility and reduced BMD. There is little literature describing the efficacy and safety of ZA in this population. In this study, we assess the response to treatment with ZA at six monthly intervals in Egyptian children with OI and BS for a period of 2 years. METHODS: Thirty-three patients with OI and five patients with BS were treated with 0.1 mg/kg ZA intravenously every 6 months for 2 years during which they were followed up using different parameters. A clinical severity score (CSS) was applied to the patients before and 2 years after the start of therapy. Comparison of disease severity and response to ZA treatment between autosomal-dominant (AD) and autosomal-recessive (AR) OI patients was also done. RESULTS: After 6 months of treatment, OI and BS patients showed a significant increase in BMD Z-scores (P < 0.003 in the spine and P < 0.004 in the hip), together with a significant drop in fracture rate (P < 0.001), relief of pain (P < 0.001), and improvement in ambulation (P < 0.001). CSS was significantly reduced after 2 years of treatment in both OI and BS patients. AR-OI patients were more severely affected than AD-OI patients and showed more significant improvement. CONCLUSION: Zoledronic acid proved to be safe and effective in the treatment of OI and BS. The biannual infusion protocol was convenient to patients. There was a positive correlation between disease severity and benefits of the treatment. The use of the CSS proved to be of value in the assessment of the degree of severity in OI, and with some modifications, it was a valuable tool for the assessment of response to treatment.


Assuntos
Conservadores da Densidade Óssea/uso terapêutico , Difosfonatos/uso terapêutico , Imidazóis/uso terapêutico , Osteogênese Imperfeita/tratamento farmacológico , Adolescente , Densidade Óssea/efeitos dos fármacos , Criança , Pré-Escolar , Difosfonatos/administração & dosagem , Esquema de Medicação , Feminino , Fraturas Ósseas/etiologia , Fraturas Ósseas/fisiopatologia , Fraturas Ósseas/prevenção & controle , Humanos , Imidazóis/administração & dosagem , Lactente , Infusões Intravenosas , Masculino , Osteogênese Imperfeita/complicações , Osteogênese Imperfeita/fisiopatologia , Medição da Dor/métodos , Estudos Prospectivos , Índice de Gravidade de Doença , Ácido Zoledrônico
2.
Biotechnol Lett ; 27(10): 737-9, 2005 May.
Artigo em Inglês | MEDLINE | ID: mdl-16049744

RESUMO

A method for the preparation of uniform gel-disks for enzyme and cell immobilisation, as well as for characterisation of gel mechanical stability, is described. The apparatus comprises a stainless steel base unit and glass parallel plates, designed to permit easy and fast production of multiple homogeneous gel sheets of variable thickness.


Assuntos
Técnicas de Química Analítica/instrumentação , Géis/química , Carragenina/química , Técnicas de Química Analítica/métodos , Géis/normas , Vidro , Polietilenoimina/química , Aço Inoxidável
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