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  • 1
    Online Resource
    Online Resource
    Estimated costs for Duchenne muscular dystrophy care in Brazil
    Springer Science and Business Media LLC ; 2023
    In:  Orphanet Journal of Rare Diseases Vol. 18, No. 1 ( 2023-06-22)
    Details
    In: Orphanet Journal of Rare Diseases, Springer Science and Business Media LLC, Vol. 18, No. 1 ( 2023-06-22)
    Abstract: The economic burden of rare diseases on health systems is still not widely measured, with the generation of accurate information about the costs with medical care for subjects with rare diseases being crucial when defining health policies. Duchenne Muscular Dystrophy (DMD) is the most common form of muscular dystrophy, with new technologies recently being studied for its management. Information about the costs related to the disease in Latin America is scarce, and the objective of this study is to evaluate the annual hospital, home care and transportation costs per patient with DMD treatment in Brazil. Results Data from 27 patients were included, the median annual cost per patient was R$ 17,121 (IQR R$ 6,786; 25,621). Home care expenditures accounted for 92% of the total costs, followed by hospital costs (6%) and transportation costs (2%). Medications and loss of family, and patient’s productivity are among the most representative consumption items. When disease worsening due to loss of the ability to walk was incorporated to the analysis, it was shown that wheelchair users account for an incremental cost of 23% compared with non-wheelchair users. Conclusions This is an original study in Latin America to measure DMD costs using the micro-costing technique. Generating accurate information about costs is crucial to provide health managers with information that could help establish more sustainable policies when deciding upon rare diseases in emerging countries.
    Type of Medium: Online Resource
    ISSN: 1750-1172
    URL: Article
    DOI: 10.1186/s13023-023-02767-6
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2225857-7
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  • 2
    Online Resource
    Online Resource
    Serum myostatin as a candidate disease severity and progression biomarker of spinal muscular atrophy
    Oxford University Press (OUP) ; 2024
    In:  Brain Communications
    Details
    In: Brain Communications, Oxford University Press (OUP)
    Abstract: The identification of biomarkers for spinal muscular atrophy (SMA) is crucial for predicting disease progression, severity, and response to new disease-modifying therapies. This study aimed to investigate the role of serum levels of myostatin and follistatin as biomarkers for SMA, considering muscle atrophy secondary to denervation as the main clinical manifestation of the disease. The study evaluated the differential gene expression of myostatin and follistatin in a lesional model of gastrocnemius denervation in mice, as well as in a meta-analysis of three datasets in transgenic mice models of SMA, and in two studies involving humans with SMA. Subsequently, a case-control study involving 27 SMA patients and 27 controls was conducted, followed by a 12-month cohort study with 25 SMA cases. Serum levels of myostatin and follistatin were analyzed using enzyme-linked immunosorbent assay at a single center in southern Brazil. Skeletal muscle gene expression of myostatin decreased and of follistatin increased following lesional muscle denervation in mice, consistent with findings in the SMA transgenic mice meta-analysis and in the iliopsoas muscle of 5 patients with SMA type 1. Median serum myostatin levels were significantly lower in SMA patients (98 pg/mL; 5-157) compared to controls (412 pg/mL; 299-730) (p & lt; 0.001). Lower myostatin levels were associated with greater disease severity based on clinician-rated outcomes (Rho = 0.493-0.812; p & lt; 0.05). After 12 months, there was a further reduction in myostatin levels among SMA cases (p = 0.021). Follistatin levels did not differ between cases and controls, and no significant changes were observed over time. The follistatin:myostatin ratio was significantly increased in SMA subjects and inversely correlated with motor severity. Serum myostatin levels show promise as a novel biomarker for evaluating the severity and progression of SMA. The decrease in myostatin levels and the subsequent favorable environment for muscle growth may be attributed to denervation caused by motor neuron dysfunction.
    Type of Medium: Online Resource
    ISSN: 2632-1297
    URL: Article
    DOI: 10.1093/braincomms/fcae062
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2024
    detail.hit.zdb_id: 3020013-1
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  • 3
    Online Resource
    Online Resource
    Muscle Strength, Joint Range of Motion, and Gait in Children and Adolescents With Osteogenesis Imperfecta
    Ovid Technologies (Wolters Kluwer Health) ; 2014
    In:  Pediatric Physical Therapy Vol. 26, No. 2 ( 2014), p. 245-252
    Details
    In: Pediatric Physical Therapy, Ovid Technologies (Wolters Kluwer Health), Vol. 26, No. 2 ( 2014), p. 245-252
    Type of Medium: Online Resource
    ISSN: 0898-5669
    URL: Article
    DOI: 10.1097/PEP.0000000000000042
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2014
    detail.hit.zdb_id: 2071155-4
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  • 4
    Online Resource
    Online Resource
    Efeitos da estimulação elétrica neuromuscular em pacientes com paralisia cerebral do tipo diplegia espástica
    Convergences Editorial ; 2018
    In:  Fisioterapia Brasil Vol. 6, No. 1 ( 2018-03-18), p. 6-9
    Details
    In: Fisioterapia Brasil, Convergences Editorial, Vol. 6, No. 1 ( 2018-03-18), p. 6-9
    Abstract: O objetivo deste trabalho foi avaliar os efeitos da utilização da estimulação elétrica neuromuscular (EENM) na funcionalidade, tônus muscular e marcha de pacientes com paralisia cerebral (PC) do tipo diplegia espástica. Quatro crianças entre 3,7 e 8 anos realizaram, durante dezesseis semanas, tratamento com EENM numa freqüência de três vezes por semana, seguido de treino de marcha. Todos foram avaliados no iní­cio e no final do tratamento. Os parâmetros utilizados foram largura de pulso de 0,3 ms, freqüência de 40 Hz, tempo de subida e descida de 2 s; tempo de sustentação e repouso de 6 s, e intensidade de corrente conforme tolerância da criança. Nossos resultados demonstraram uma melhora na funcionalidade e na marcha nas crianças. Palavras-chave: paralisia cerebral, diplegia, estimulação elétrica.
    Type of Medium: Online Resource
    ISSN: 2526-9747 , 1518-9740
    URL: Issue
    URL: Article
    DOI: 10.33233/fb.v6i1
    DOI: 10.33233/fb.v6i1.1949
    Language: Unknown
    Publisher: Convergences Editorial
    Publication Date: 2018
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  • 5
    Online Resource
    Online Resource
    Estimulação elétrica neuromuscular em crianças com paralisia cerebral do tipo diplegia espástica
    Convergences Editorial ; 2018
    In:  Fisioterapia Brasil Vol. 7, No. 5 ( 2018-03-20), p. 357-362
    Details
    In: Fisioterapia Brasil, Convergences Editorial, Vol. 7, No. 5 ( 2018-03-20), p. 357-362
    Abstract: Nos últimos anos, vários estudos propuseram a utilização da estimulação elétrica como forma de tratamento da paralisia cerebral (PC). Objetivo: Utilizando um delineamento do tipo antes e depois (a within-subjects design) buscou-se avaliar os efeitos da estimulação elétrica neuromuscular (EENM) nas habilidades motoras de crianças com PC do tipo diplegia espástica. Material e método: Doze pacientes (8 do sexo masculino, média de idade 6 anos) foram avaliados antes e depois de 12 semanas de terapia utilizando EENM. As habilidades motoras foram avaliadas através da escala de função motora grossa (GMFM), parâmetros lineares da marcha e amplitude de movimento (ADM) de fl exão dorsal dos tornozelos. Resultados: A diferença entre as médias dos escores da GMFM foram estatisticamente signiï¬ cativos (P = 0,032) quando comparados antes (73,1 ± 17,2) e depois (76,5 ± 16,3) da intervenção com EENM. Quando os pacientes foram classiï¬ cados quanto í  independência para marcha, o escore da GMFM permaneceu signiï¬ cativo apenas nas crianças dependentes de auxí­lio para marcha (P = 0,045). O parâmetro de cadência da marcha diferiu antes e depois da EENM no grupo de crianças independentes para marcha (P = 0,030). Conclusões: Este estudo demonstrou que a EENM pode ser uma ferramenta complementar no manejo de crianças PC do tipo diplégica espástica. Os mecanismos pelos quais a EENM melhora a função motora não estão totalmente esclarecidos. Palavras-chave: paralisia cerebral, análise de marcha, diplegia espástica, estimulação elétrica neuromuscular.
    Type of Medium: Online Resource
    ISSN: 2526-9747 , 1518-9740
    URL: Issue
    URL: Article
    DOI: 10.33233/fb.v7i5
    DOI: 10.33233/fb.v7i5.1930
    Language: Unknown
    Publisher: Convergences Editorial
    Publication Date: 2018
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  • 6
    Online Resource
    Online Resource
    Estimulação elétrica neuromuscular em crianças com paralisia cerebral do tipo diplegia espástica
    Convergences Editorial ; 2017
    In:  Fisioterapia Brasil Vol. 9, No. 4 ( 2017-12-30), p. 224-230
    Details
    In: Fisioterapia Brasil, Convergences Editorial, Vol. 9, No. 4 ( 2017-12-30), p. 224-230
    Abstract: Nos últimos anos, vários estudos propuseram a utilização da estimulação elétrica como forma de tratamento da paralisia cerebral (PC). Objetivo: Utilizando um delineamento do tipo antes e depois (within-subjects design) buscou-se avaliar os efeitos da estimulação elétrica neuromuscular (EENM) nas habilidades motoras de crianças com PC do tipo diplegia espástica. Material e métodos: Doze pacientes (8 do sexo masculino, média de idade 6 anos) foram avaliados antes e depois de 12 semanas de terapia utilizando EENM. As habilidades motoras foram avaliadas através da escala de função motora grossa (GMFM), parâmetros lineares da marcha e amplitude de movimento (ADM) de flexão dorsal dos tornozelos. Resultados: A diferença entre as médias dos escores da GMFM foram estatisticamente significativos (P = 0,032) quando comparados antes (73,1 ± 17,2) e depois (76,5 ± 16,3) da intervenção com EENM. Quando os pacientes foram classificados quanto í  independência para marcha, o escore da GMFM permaneceu significativo apenas nas crianças dependentes de auxí­lio para marcha (P = 0,045). O parâmetro de cadência da marcha diferiu antes e depois da EENM no grupo de crianças independentes para marcha (P = 0,030). Conclusões: Este estudo demonstrou que a EENM pode ser uma ferramenta complementar no manejo de crianças PC do tipo diplégica espástica. Os mecanismos pelos quais a EENM melhora a função motora não estão totalmente esclarecidos.Palavras-chave: paralisia cerebral, análise de marcha, diplegia espástica, estimulação elétrica neuromuscular.
    Type of Medium: Online Resource
    ISSN: 2526-9747 , 1518-9740
    URL: Issue
    URL: Article
    DOI: 10.33233/fb.v9i4
    DOI: 10.33233/fb.v9i4.1710
    Language: Unknown
    Publisher: Convergences Editorial
    Publication Date: 2017
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