Assessment and factors affecting quality of life among patients with Wilson’s disease
Abstract Wilson’s disease is caused by abnormal copper metabolism resulting in deposition in various organs, including the brain, liver, and cornea, thus disrupting organ function. It is characterized by encephalopathy, extrapyramidal symptoms, progressive liver failure, and copper ring deposition i...
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| Format: | Article |
| Language: | English |
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Nature Portfolio
2024-04-01
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| Series: | Scientific Reports |
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| Online Access: | https://doi.org/10.1038/s41598-024-59377-w |
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| author | Tingting Zhan Yuxiang Guan Caijie Sun Lili Wang Yan Wang Xiang Li |
| author_facet | Tingting Zhan Yuxiang Guan Caijie Sun Lili Wang Yan Wang Xiang Li |
| author_sort | Tingting Zhan |
| collection | DOAJ |
| description | Abstract Wilson’s disease is caused by abnormal copper metabolism resulting in deposition in various organs, including the brain, liver, and cornea, thus disrupting organ function. It is characterized by encephalopathy, extrapyramidal symptoms, progressive liver failure, and copper ring deposition in the cornea. Management of this disease should include quality of life maintenance; however, relevant studies on this topic are lacking. This study aimed to assess the factors affecting the quality of life (QoL) of patients with Wilson’s disease. A cross-sectional survey using convenience sampling was conducted between July 2020 and March 2021 at the hospital. Data on patient characteristics, 36-item Short-Form General Health Survey, Uniform Wilson Disease Rating Scale, and Hamilton Depression Rating Scale scores were collected. Associations among quality of life depression, anxiety, and Wilson’s disease progression were examined using Pearson correlation analysis. Factors affecting the quality of life of patients, including depression, anxiety, liver function, clinical symptoms, diet, liver function, brain magnetic resonance imaging (MRI) findings, disease duration, Barthel Index, and Morse scores were examined using multivariate linear regression analysis. This study included 134 patients with Wilson’s disease whose mean age was 29.12 ± 8.59 years. The mean QoL score in the patient group was 71.38 ± 9.55 points and was negatively correlated with anxiety (r = − 0.883, P = 0.000), depression (r = − 0.852 P = 0.000), and clinical symptoms (r = − 0.542, P = 0.000) scores. Anxiety, depression, and clinical symptoms severity are vital factors for the QoL of patients with Wilson’s disease. The study provides foundational evidence to design novel interventions, including symptom management, diet, and self-care ability, which can help in improving the quality of life in patients with Wilson’s disease and decreasing the burden associated with this disease. |
| first_indexed | 2024-04-24T07:14:52Z |
| format | Article |
| id | doaj.art-27290f6750a446a582328f360b110ecd |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-04-24T07:14:52Z |
| publishDate | 2024-04-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-27290f6750a446a582328f360b110ecd2024-04-21T11:18:45ZengNature PortfolioScientific Reports2045-23222024-04-011411510.1038/s41598-024-59377-wAssessment and factors affecting quality of life among patients with Wilson’s diseaseTingting Zhan0Yuxiang Guan1Caijie Sun2Lili Wang3Yan Wang4Xiang Li5Department of Brain Center, The First Affiliated Hospital of Anhui University of Chinese MedicineDepartment of Brain Center, The First Affiliated Hospital of Anhui University of Chinese MedicineSchool of Nursing, Anhui University of Traditional Chinese MedicineSchool of Nursing, Anhui University of Traditional Chinese MedicineSchool of Nursing, Anhui University of Traditional Chinese MedicineDepartment of Brain Center, The First Affiliated Hospital of Anhui University of Chinese MedicineAbstract Wilson’s disease is caused by abnormal copper metabolism resulting in deposition in various organs, including the brain, liver, and cornea, thus disrupting organ function. It is characterized by encephalopathy, extrapyramidal symptoms, progressive liver failure, and copper ring deposition in the cornea. Management of this disease should include quality of life maintenance; however, relevant studies on this topic are lacking. This study aimed to assess the factors affecting the quality of life (QoL) of patients with Wilson’s disease. A cross-sectional survey using convenience sampling was conducted between July 2020 and March 2021 at the hospital. Data on patient characteristics, 36-item Short-Form General Health Survey, Uniform Wilson Disease Rating Scale, and Hamilton Depression Rating Scale scores were collected. Associations among quality of life depression, anxiety, and Wilson’s disease progression were examined using Pearson correlation analysis. Factors affecting the quality of life of patients, including depression, anxiety, liver function, clinical symptoms, diet, liver function, brain magnetic resonance imaging (MRI) findings, disease duration, Barthel Index, and Morse scores were examined using multivariate linear regression analysis. This study included 134 patients with Wilson’s disease whose mean age was 29.12 ± 8.59 years. The mean QoL score in the patient group was 71.38 ± 9.55 points and was negatively correlated with anxiety (r = − 0.883, P = 0.000), depression (r = − 0.852 P = 0.000), and clinical symptoms (r = − 0.542, P = 0.000) scores. Anxiety, depression, and clinical symptoms severity are vital factors for the QoL of patients with Wilson’s disease. The study provides foundational evidence to design novel interventions, including symptom management, diet, and self-care ability, which can help in improving the quality of life in patients with Wilson’s disease and decreasing the burden associated with this disease.https://doi.org/10.1038/s41598-024-59377-wWilson’s diseaseQuality of lifeDepressionAnxietyCopper deposits |
| spellingShingle | Tingting Zhan Yuxiang Guan Caijie Sun Lili Wang Yan Wang Xiang Li Assessment and factors affecting quality of life among patients with Wilson’s disease Scientific Reports Wilson’s disease Quality of life Depression Anxiety Copper deposits |
| title | Assessment and factors affecting quality of life among patients with Wilson’s disease |
| title_full | Assessment and factors affecting quality of life among patients with Wilson’s disease |
| title_fullStr | Assessment and factors affecting quality of life among patients with Wilson’s disease |
| title_full_unstemmed | Assessment and factors affecting quality of life among patients with Wilson’s disease |
| title_short | Assessment and factors affecting quality of life among patients with Wilson’s disease |
| title_sort | assessment and factors affecting quality of life among patients with wilson s disease |
| topic | Wilson’s disease Quality of life Depression Anxiety Copper deposits |
| url | https://doi.org/10.1038/s41598-024-59377-w |
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