Andrés Nascimento, Carlos Ortez, Jessica Expósito, Laura Carrera, Silvia Cerezo, Stephanie Lotz, Irene Zschaeck, Agustín Lujan, Chiara Gatnau, Berta Estévez, Eduardo Tizzano, Daniel Natera De Benito
{"title":"[小儿神经肌肉疾病的特殊治疗]。","authors":"Andrés Nascimento, Carlos Ortez, Jessica Expósito, Laura Carrera, Silvia Cerezo, Stephanie Lotz, Irene Zschaeck, Agustín Lujan, Chiara Gatnau, Berta Estévez, Eduardo Tizzano, Daniel Natera De Benito","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>This article provides an overview of neuromuscular diseases in childhood for pediatric neurologists, highlighting conditions with available specific treatments. It focuses on spinal muscular atrophy (SMA), where disease-modifying therapies have changed the natural history of the disease. Congenital myasthenic syndromes are addressed next, emphasizing the importance of genetic diagnosis for tailored therapies. In the field of muscular dystrophies, we will highlight advances in Duchenne. Mitochondrial myopathies are also reviewed, with mention of treatments such nucleoside for timidine kinase deficiency. Pompe disease is highlighted due to the availability of enzyme replacement therapy and finally, the article discusses treatable metabolic myopathies, such as riboflavin transporter deficiencies. This review aims to promote early diagnosis and personalized management in neuromuscular disorders.</p>","PeriodicalId":18419,"journal":{"name":"Medicina-buenos Aires","volume":"85 Suppl 4 ","pages":"34-40"},"PeriodicalIF":0.6000,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Neuromuscular diseases in pediatrics with specific treatments].\",\"authors\":\"Andrés Nascimento, Carlos Ortez, Jessica Expósito, Laura Carrera, Silvia Cerezo, Stephanie Lotz, Irene Zschaeck, Agustín Lujan, Chiara Gatnau, Berta Estévez, Eduardo Tizzano, Daniel Natera De Benito\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>This article provides an overview of neuromuscular diseases in childhood for pediatric neurologists, highlighting conditions with available specific treatments. It focuses on spinal muscular atrophy (SMA), where disease-modifying therapies have changed the natural history of the disease. Congenital myasthenic syndromes are addressed next, emphasizing the importance of genetic diagnosis for tailored therapies. In the field of muscular dystrophies, we will highlight advances in Duchenne. Mitochondrial myopathies are also reviewed, with mention of treatments such nucleoside for timidine kinase deficiency. Pompe disease is highlighted due to the availability of enzyme replacement therapy and finally, the article discusses treatable metabolic myopathies, such as riboflavin transporter deficiencies. This review aims to promote early diagnosis and personalized management in neuromuscular disorders.</p>\",\"PeriodicalId\":18419,\"journal\":{\"name\":\"Medicina-buenos Aires\",\"volume\":\"85 Suppl 4 \",\"pages\":\"34-40\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2025-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Medicina-buenos Aires\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Medicina-buenos Aires","FirstCategoryId":"3","ListUrlMain":"","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
[Neuromuscular diseases in pediatrics with specific treatments].
This article provides an overview of neuromuscular diseases in childhood for pediatric neurologists, highlighting conditions with available specific treatments. It focuses on spinal muscular atrophy (SMA), where disease-modifying therapies have changed the natural history of the disease. Congenital myasthenic syndromes are addressed next, emphasizing the importance of genetic diagnosis for tailored therapies. In the field of muscular dystrophies, we will highlight advances in Duchenne. Mitochondrial myopathies are also reviewed, with mention of treatments such nucleoside for timidine kinase deficiency. Pompe disease is highlighted due to the availability of enzyme replacement therapy and finally, the article discusses treatable metabolic myopathies, such as riboflavin transporter deficiencies. This review aims to promote early diagnosis and personalized management in neuromuscular disorders.