Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study
| Autor(a) principal: | |
|---|---|
| Data de Publicação: | 2021 |
| Outros Autores: | , , , , , , , , , , , , , , , , , , , |
| Tipo de documento: | Artigo |
| Idioma: | eng |
| Título da fonte: | Repositório Institucional da FIOCRUZ (ARCA) |
| Texto Completo: | https://www.arca.fiocruz.br/handle/icict/46480 |
Resumo: | Declaration of Competing Interest AG, AMM, ASSP, AXA, BFFR, CML, FCS, JFSF, LCLL, LRG, TBT, JICFN: declared no conflict of interest. ACMS, ALB, CAK, DDGH, EKEAL, EMR, MCSR, MK, RCFB: received funds or reimbursement from BioMarin Brasil Farmaceutica LTDA for attending related symposia, or speaker honoraria. DDGH: received consulting fees from BioMarin Brasil Farmaceutica LTDA. |
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Horovitz, Dafne Dain GandelmanLeão, Emília Katiane Embiruçu de AraújoRibeiro, Erlane M.Martins, Ana M.Barth, Anneliese LopesNeri, João I. C. F.Kerstenetzky, MarceloSiqueira, Ana C. M.Ribeiro, Bethania F. R.Kim, Chong A.Santos, Francisca C.Franco, José F. S.Lichtvan, Leniza C. L.Giuliani, Liane R.Rodrigues, Maria do C. S.Bonatti, Renata C. F.Teixeira, Thaís B.Gonçalves, AlexandraLourenço, Charles M.Pereira, Ane S. S.Acosta, Angelina X.2021-03-31T19:51:48Z2021-03-31T19:51:48Z2021HOROVITZ, Dafne Dain Gandelman et al. Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study. Molecular Genetics and Metabolism, p. 1-6, 2021.1096-7192https://www.arca.fiocruz.br/handle/icict/4648010.1016/j.ymgme.2021.02.006Declaration of Competing Interest AG, AMM, ASSP, AXA, BFFR, CML, FCS, JFSF, LCLL, LRG, TBT, JICFN: declared no conflict of interest. ACMS, ALB, CAK, DDGH, EKEAL, EMR, MCSR, MK, RCFB: received funds or reimbursement from BioMarin Brasil Farmaceutica LTDA for attending related symposia, or speaker honoraria. DDGH: received consulting fees from BioMarin Brasil Farmaceutica LTDA.Fundação Oswaldo Cruz. Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Centro de Genética Médica. Rio de Janeiro, RJ, Brasil.Universidade Federal da Bahia. Serviço de Genética Médica. Salvador, BA, Brasil. / Universidade do Estado da Bahia. Salvador, BA, Brasil.Hospital Albert Sabin. Fortaleza, CE, Brasil.Universidade Federal de São Paulo. Centro de Referência em Erros Inatos do Metabolismo. São Paulo, SP, Brasil.Fundação Oswaldo Cruz. Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Centro de Genética Médica. Rio de Janeiro, RJ, Brasil.Centro de Reabilitação Infantil de Natal. Natal, RN, Brasil.Hospital Maria Lucinda. Centro de Referência em Doenças Raras. Recife, PE, Brasil.Instituto de Medicina Integral Professor Fernando Figueira. Centro de Tratamento de Erros Inatos do Metabolismo. Recife, PE, Brasil.Fundação Hospital do Acre. Rio Branco, AC, BrasilUniversidade de São Paulo. Faculdade de Medicina. Instituto da Criança. São Paulo, SP, Brasil.Hospital Universitário do Maranhão. São Luís, MA, Brasil.PUC-Campinas. Enzyme Replacement Therapy Service at Hospital e Maternidade Celso Pierro. Campinas, SP, Brasil / Universidade de São Paulo. Centro de Biotecnologia. Instituto de Pesquisa de energia Nuclear. São Paulo, SP, Brasil.Universidade Federal do Paraná. Curitiba, PR, Brasil.Universidade Federal do Mato Grosso do Sul. Faculdade de Medicina. Campo Grande, MS, Brasil.Hospital Universitário Cassiano Antonio de Moraes. / Universidade Federal do Espírito Santo. Empresa Brasileira de Serviços Hospitalares. Vitória, ES, Brasil.Universidade Federal do Triângulo Mineiro. Uberaba, MG, Brasil.Associação de Pais e Amigos dos Excepcionais de Anápolis. Anápolis, GO, Brasil.Centro Universitário Estácio de Ribeirão Preto. Faculdade de Medicina. Ribeirão Preto, SP, Brasil.Universidade do Estado da Bahia. Salvador, BA, Brasil.Universidade Federal da Bahia. Serviço de Genética Médica. Salvador, BA, Brasil.Patients with mucopolysaccharidosis type VI (MPS VI) present with a wide range of disease severity and clinical manifestations, with significant functional impairment and shortened lifespan. Enzyme replacement therapy (ERT) with galsulfase has been shown to improve clinical and biochemical parameters including patient survival, quality of life and growth. The present study is a resurvey of 34 Brazilian MPS VI patients with rapidly progressive disease (classical phenotype) who initiated ERT with galsulfase under five years of age and had been on ERT until data collection in 2019, with few exceptions (n = 4 patients who died before 2019). Anthropometric measures, urinary glycosaminoglycans, and data regarding cardiac, orthopedic, neurologic, sleep apnea, hearing and ophthalmologic outcomes were filled in by specialists. Pubertal development, clinical complications, hospitalizations, and surgeries were also assessed. In this resurvey study, treatment with galsulfase has shown to be safe and well tolerated in MPS VI patients who initiated ERT under the age of 5 years and who have been undergoing ERT for approximately 10 years. Mortality rate suggests that early initiation of ERT may have a positive impact on patients' survival, improving but not preventing disease progression and death. MPS VI patients on ERT also showed improved growth velocity and the pubertal development was normal in all surviving patients. Follow-up data on pneumonia and hospitalization suggest that early ERT may have a protective effect against major respiratory complications. Cardiac valve disease progressed since their prior evaluation and spinal cord compression was observed in a large number of patients, suggesting that these disease complications were not modified by ERT.engElsevier [Commercial Publisher], Academic Press [Associate Organisation], Society for Inherited Metabolic Disorders [Associate Organisation]Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey studyinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleEnzyme replacement therapyGalsulfaseLong-term follow-upLysosomal storage disorderMPS VIMucopolysaccharidosis VIMaroteaux-Lamy Syndromeinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da FIOCRUZ (ARCA)instname:Fundação Oswaldo Cruz (FIOCRUZ)instacron:FIOCRUZLICENSElicense.txtlicense.txttext/plain; charset=utf-83134https://www.arca.fiocruz.br/bitstream/icict/46480/1/license.txt0ab46789d568c4ba27c7b5d29a9fe9c4MD51ORIGINALLong-term impact of early initiation 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| dc.title.pt_BR.fl_str_mv |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| title |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| spellingShingle |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study Horovitz, Dafne Dain Gandelman Enzyme replacement therapy Galsulfase Long-term follow-up Lysosomal storage disorder MPS VI Mucopolysaccharidosis VI Maroteaux-Lamy Syndrome |
| title_short |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| title_full |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| title_fullStr |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| title_full_unstemmed |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| title_sort |
Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study |
| author |
Horovitz, Dafne Dain Gandelman |
| author_facet |
Horovitz, Dafne Dain Gandelman Leão, Emília Katiane Embiruçu de Araújo Ribeiro, Erlane M. Martins, Ana M. Barth, Anneliese Lopes Neri, João I. C. F. Kerstenetzky, Marcelo Siqueira, Ana C. M. Ribeiro, Bethania F. R. Kim, Chong A. Santos, Francisca C. Franco, José F. S. Lichtvan, Leniza C. L. Giuliani, Liane R. Rodrigues, Maria do C. S. Bonatti, Renata C. F. Teixeira, Thaís B. Gonçalves, Alexandra Lourenço, Charles M. Pereira, Ane S. S. Acosta, Angelina X. |
| author_role |
author |
| author2 |
Leão, Emília Katiane Embiruçu de Araújo Ribeiro, Erlane M. Martins, Ana M. Barth, Anneliese Lopes Neri, João I. C. F. Kerstenetzky, Marcelo Siqueira, Ana C. M. Ribeiro, Bethania F. R. Kim, Chong A. Santos, Francisca C. Franco, José F. S. Lichtvan, Leniza C. L. Giuliani, Liane R. Rodrigues, Maria do C. S. Bonatti, Renata C. F. Teixeira, Thaís B. Gonçalves, Alexandra Lourenço, Charles M. Pereira, Ane S. S. Acosta, Angelina X. |
| author2_role |
author author author author author author author author author author author author author author author author author author author author |
| dc.contributor.author.fl_str_mv |
Horovitz, Dafne Dain Gandelman Leão, Emília Katiane Embiruçu de Araújo Ribeiro, Erlane M. Martins, Ana M. Barth, Anneliese Lopes Neri, João I. C. F. Kerstenetzky, Marcelo Siqueira, Ana C. M. Ribeiro, Bethania F. R. Kim, Chong A. Santos, Francisca C. Franco, José F. S. Lichtvan, Leniza C. L. Giuliani, Liane R. Rodrigues, Maria do C. S. Bonatti, Renata C. F. Teixeira, Thaís B. Gonçalves, Alexandra Lourenço, Charles M. Pereira, Ane S. S. Acosta, Angelina X. |
| dc.subject.en.pt_BR.fl_str_mv |
Enzyme replacement therapy Galsulfase Long-term follow-up Lysosomal storage disorder MPS VI Mucopolysaccharidosis VI Maroteaux-Lamy Syndrome |
| topic |
Enzyme replacement therapy Galsulfase Long-term follow-up Lysosomal storage disorder MPS VI Mucopolysaccharidosis VI Maroteaux-Lamy Syndrome |
| description |
Declaration of Competing Interest AG, AMM, ASSP, AXA, BFFR, CML, FCS, JFSF, LCLL, LRG, TBT, JICFN: declared no conflict of interest. ACMS, ALB, CAK, DDGH, EKEAL, EMR, MCSR, MK, RCFB: received funds or reimbursement from BioMarin Brasil Farmaceutica LTDA for attending related symposia, or speaker honoraria. DDGH: received consulting fees from BioMarin Brasil Farmaceutica LTDA. |
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2021 |
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2021-03-31T19:51:48Z |
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2021-03-31T19:51:48Z |
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2021 |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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HOROVITZ, Dafne Dain Gandelman et al. Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study. Molecular Genetics and Metabolism, p. 1-6, 2021. |
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https://www.arca.fiocruz.br/handle/icict/46480 |
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1096-7192 |
| dc.identifier.doi.none.fl_str_mv |
10.1016/j.ymgme.2021.02.006 |
| identifier_str_mv |
HOROVITZ, Dafne Dain Gandelman et al. Long-term impact of early initiation of enzyme replacement therapy in 34 MPS VI patients: A resurvey study. Molecular Genetics and Metabolism, p. 1-6, 2021. 1096-7192 10.1016/j.ymgme.2021.02.006 |
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https://www.arca.fiocruz.br/handle/icict/46480 |
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eng |
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openAccess |
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Elsevier [Commercial Publisher], Academic Press [Associate Organisation], Society for Inherited Metabolic Disorders [Associate Organisation] |
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Elsevier [Commercial Publisher], Academic Press [Associate Organisation], Society for Inherited Metabolic Disorders [Associate Organisation] |
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