泰-萨克斯病（一种罕见的儿科疾病）患者的护理计划
Nursing Care Plan for Patients with Tay-Sachs-A Rare Paediatric Disease.
作者信息
Cortés-Martín Jonathan, Piqueras-Sola Beatriz, Sánchez-García Juan Carlos, Reinoso-Cobo Andrés, Ramos-Petersen Laura, Díaz-Rodríguez Lourdes, Rodríguez-Blanque Raquel
机构信息
Research Group CTS1068, Andalusia Research Plan, Junta de Andalucía, Nursing Department, Faculty of Health Sciences, University of Granada, 18071 Granada, Spain.
Hospital University Virgen de las Nieves, 18014 Granada, Spain.
出版信息
J Pers Med. 2023 Aug 1;13(8):1222. doi: 10.3390/jpm13081222.
Tay-Sachs disease is classified as a rare paediatric disease of metabolic origin. It is an autosomal recessive inherited disease. The gene responsible for the disease is known as HEXA, and it is located on chromosome 15(15q23). There is currently no effective treatment for Tay-Sachs disease; hence, it is an incurable disease in which patients do not live for more than five years, meaning that nursing care takes on greater importance to maintain quality of life. The main objective of this work is to develop a specific standard nursing care plan by applying an inductive research method supported by nursing methodology using the NANDA-NIC-NOC taxonomy and validated by the Delphi method. This care plan will improve the knowledge of health professionals on this topic and support future studies on the disease. Following its implementation, the care plan proposed in this study aims to increase the quality of life of patients diagnosed with this disease.
泰-萨克斯病被归类为一种罕见的代谢性儿科疾病。它是一种常染色体隐性遗传病。导致该疾病的基因名为HEXA，位于15号染色体（15q23）上。目前尚无治疗泰-萨克斯病的有效方法；因此，它是一种不治之症，患者存活时间不超过五年，这意味着护理对于维持生活质量更为重要。本研究的主要目的是通过采用以护理方法学为支撑的归纳研究方法，运用NANDA-NIC-NOC分类法并经德尔菲法验证，制定一份特定的标准护理计划。该护理计划将提高医护人员对这一主题的认识，并为该疾病的未来研究提供支持。本研究提出的护理计划在实施后旨在提高被诊断患有这种疾病的患者的生活质量。
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