Publication:
Nursing Care Plan for Patients with Hajdu-Cheney Syndrome.

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Date

2022-06-18

Authors

Cortés-Martín, Jonathan
Díaz-Rodríguez, Lourdes
Piqueras-Sola, Beatriz
Sánchez-García, Juan Carlos
Menor-Rodríguez, María José
Rodríguez-Blanque, Raquel

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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.

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Acro-Osteolysis
Hajdu-Cheney Syndrome
Humans
Osteoporosis
Patient Care Planning
Quality of Life
Rare Diseases

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Keywords

Hajdu–Cheney syndrome, NOTCH2, acroosteolysis, clinical practice, healthcare, nursing care plan, rare disease

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