PDF(Pubmed)
Abstract：
Hajdu-Cheney syndrome is a rare genetic disease. Its main features include phenotypic variability, age-dependent progression and the presence of acroosteolysis of the distal phalanges and generalized osteoporosis, which have significant disabling potential. Currently, there is no effective curative treatment, so nursing care is essential to ensure the maintenance of the quality of life of these patients. The main objective of this study was to establish a specific standardized nursing care plan using the NANDA-NIC-NOC taxonomy. The application of a care plan as such would improve the quality of life of patients affected by this rare disease, will contribute to increasing healthcare professionals\' knowledge on this matter and will support future studies on this disease.
摘要：
Hajdu-Cheney综合征是一种罕见的遗传性疾病。其主要特征包括表型变异性，年龄依赖性进展以及远端指骨和全身性骨质疏松的存在，具有显著的致残潜力。目前,没有有效的治疗方法，因此，护理对于确保这些患者的生活质量至关重要。这项研究的主要目的是使用NANDA-NIC-NOC分类法建立特定的标准化护理计划。这样的护理计划的应用将提高受这种罕见疾病影响的患者的生活质量，将有助于增加医疗保健专业人员对这一问题的知识，并将支持未来对这一疾病的研究。
