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Development and characterization of 3CaO·P_(2)O_(5)-SiO_(2)-MgO glass-ceramics with different crystallization degree

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Journal of Advanced Ceramics 2013年第4期2卷 378-388页

作者： Juliana Kelmy M.F.DAGUANO Paulo A.SUZUKI Kurt STRECKER JoséMartinho Marques de OLIVEIRA maria helena figueira vaz fernandes Claudinei SANTOS Universidade de São Paulo-Escola de Engenharia de Lorena USP-EEL-Pólo Urbo-Industrials/nGleba AI-6Lorena-SPCEP 12600-000Brazil Universidade Federal de São João del-Rei -UFSJ-CENENCampus S^(to)Antônio-Praça Frei Orlando 170-CentroS.J.del-Rei-MG.CEP 36307-352Brazil Escola Superior Aveiro Norte Edifício Rainha3720-232 O.AzeméisPortugal Universidade de Aveiro Campus Universitário de Santiago 3810-193 AveiroPortugal Universidade do Estado do Rio de Janeiro-Faculdade de Tecnologia de Resende-UERJ-FAT-Rod.Presidente Dutra km298Resende-RJCEP 27537-000Brazil

The CaO-P_(2)O_(5)-SiO_(2)-MgO system presents several compounds used as biomaterials such as hydroxyapatite(HA),tricalcium phosphate(TCP)and TCP with magnesium substituting partial calcium(TCMP).Theβ-TCMP phase with whitlockite structure has interesting biological features and mechanical properties,meeting the requirements of a bioactive material for bone *** this work,the production of Mg-doped TCP,β-TCMP,has been investigated by crystallization from a glass composed of 52.75 wt%3CaO·P_(2)O_(5),30 wt%SiO_(2)and 17.25 wt%MgO(i.e.,31.7 mol%CaO,10.6 mol%P_(2)O_(5),26.6 mol%MgO and 31.1 mol%SiO_(2))using heat treatments between 775℃and 1100℃for up to 8 *** devitrification process of the glass has been accompanied by differential scanning calorimetry(DSC),high-resolution X-ray diffraction(HRXRD),relative density and bending strength *** characterization by HRXRD and DSC revealed the occurrence of whitlockite soon after the bulk glass preparation,a transient non-cataloged silicate between 800℃and 1100℃,and the formation of diopside in samples treated at 1100℃as crystalline *** overall crystalline fraction varied from 26%to 70%depending on the heat ***,contraction of the a-axis lattice parameter and expansion of the c-axis lattice parameter of the whitlockite structure have been observed during the heat treatments,which were attributed to theβ-TCMP formation with the partial substitution of Ca^(2+)by Mg^(2+).Relative densities near 99%and 97%for the glass and glass-ceramics respectively indicated a discrete reduction as a function of the devitrification *** strengths of 70 MPa and 120 MPa were determined for the glass and glass-ceramic material crystallized at 975℃for 4 h,respectively.

关键词： glass-ceramics heat treatment high-resolution X-ray diffraction(HRXRD) bending strength

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Clinical and Technological Dependence Characteristics on a Series of Brazilian Cases with Infantile Onset Pompe Disease in Enzyme Replacement Therapy

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Open Journal of Clinical Diagnostics 2019年第1期9卷 16-32页

作者： Paula De Almeida Thomazinho Eliana Pelissari Regina Celia Beltrao Duarte Carolina Fishinger Moura De Souza Heloise helena Siqueira Borges maria Da Gloria Cruvinel Horta Liane De Rosso Giuliani Ana maria Martins Lilian Stewart Dafne Dain Gandelman Horovitz Juan Clinton Llerena Motor Physical Therapy Unit Fernandes Figueira National Institute/FIOCRUZRio de JaneiroBrazil Clinical Medical Unit Dr.Jeser Amarante Faria Children’s HospitalJoinvilleBrazil Pediatric Neurology Unit Santa Casa de Misericordia do Para FoundationBelemBrazil Sciences Medical Genetics Unit Service of Information on Inborn Errors of MetabolismHospital de Clínicas de Porto AlegrePorto AlegreBrazil Clinical Medical Unit University of Cuiaba UNICCuiabaBrazil Santa Casa de Belo Horizonte Belo HorizonteBrazil Genetics Unit Federal University of Mato Grosso do SulCampo GrandeBrazil Inborn Errors of Metabolism and Genetics Unit Federal University of São PauloUNIFESPSão PauloBrazil Pediatric Intensive Care Unit State Institute of Cardiology of Rio de JaneiroIECACRio de JaneiroBrazil 1 Medical Genetics Unit Fernandes Figueira National Institute of WomenChildren and Adolescent Health/FIOCRUZRio de JaneiroBrasil

Pompe disease (PD) is a rare inborn error of metabolism due to an abnormal acid alpha-glucosidase (GAA) activity that comprises glycogen breakdown mainly in the lysosomes. Since the introduction of enzyme replacement therapy (ERT), with recombinant human GAA for the early onset PD patient, a relevant field of clinical research due to the benefits regarding survival rate has been widely documented worldwide. Objective: To describe the clinical characteristics and the ERT effects in a series of Brazilian patients with infantile onset PD (IOPD) under ERT. Methods: Brazilian patients diagnosed with IOPD under ERT were recruited through their physicians participating in the International Pompe Disease Registry from 2009 to 2017. Data were collected by an online survey. Results: 10 IOPD patients were identified through the survey with a death rate of 30% and technology dependency rate reported as 80% (motor, respiratory or nutritional fields) of the patients. After the third year of ERT, motor disabilities were lost in 50% of ambulated patients. The overall characteristics were similar to international studies. Conclusion: Despite ERT benefits in cardiac involvement, motor disabilities seem to be much more compromised in IOPD patients, with high technology dependence, especially after three years of age.

关键词： Motor Development Child Disability Glycogen Storage Disease Type II Enzyme Replacement Therapy

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