To comprehensively assess the disease severity in patients with Wilson’s disease (WD) who had been previously diagnosed and treated with copper removal by Unified Wilson’s Disease Rating Scale (UWDRS) and analyze its influencing factors, in order to provide a reference for patient management and improvement of patients’ health.
Wilson’s disease patients who were admitted to West China Fourth Hospital of Sichuan University from July 2023 to March 2024 were selected as the study subjects, and all patients have been diagnosed according to the diagnostic criteria. The survey was conducted using self-designed structured questionnaires and UWDRS scales. Univariate analysis and multiple linear regression analysis were used to analyze the disease severity and influencing factors of patients with WD who had been previously diagnosed and treated with copper removal.
A total of 103 patients with eligible and complete data were included. The total UWDRS score of patients with WD who underwent copper repulsion therapy was 27.0 (11.5, 60.5), neurological symptom score of 21.0 (4.5, 48.5), hepatic symptom score of 3.0 (1.0, 4.0), and psychiatric symptom score of 5.0 (2.0, 8.0). More than 50% of patients still have symptoms or signs of rapid alternating movements of hand, finger tapping, salivation, gait abnormalities, speech impairment (dysarthria), impaired handwriting, and other bleeding or hematoma. Multiple linear regression analysis showed that education level, classification of first-time diagnosed disease, low-copper diet, social support and medication adherence were the influencing factors of UWDRS total score (P<0.05). The higher the patient’s level of education, medication adherence, and social support are, the lower the UWDRS score is. Patients with hepatic type had lower scores than those with cerebral type, and those who tried to adhere to a low-copper diet had lower scores than those who adhered to a low-copper diet all the time.
More than 50% of the previously diagnosed patients with WD who have been treated with copper removal still have different degrees of neurological symptoms, and the treatment of neurological symptoms needs to be further improved. Education level, classification of first-time diagnosed disease, low-copper diet, social support, and medication adherence were the influencing factors of disease severity in patients with WD.
| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |