Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin
Background and Aim. Significantly reduced serum ceruloplasmin (Cp) is the most important clue in the diagnosis of Wilson’s disease (WD) and is well known to clinicians. The false increase in Cp in some WD patients, which overlaps with that in non-WD liver disease patients, decreases the diagnostic a...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Hindawi-Wiley
2023-01-01
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| Series: | International Journal of Clinical Practice |
| Online Access: | http://dx.doi.org/10.1155/2023/9344891 |
| _version_ | 1827000140158730240 |
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| author | Lin Chen Yongguang Shi Nan Wang Zhuoqi Lou Liya Pan Xiaolan Xu Chensi Wu Yongzhu Han Renmin Yang Wenbin Hu Bing Ruan |
| author_facet | Lin Chen Yongguang Shi Nan Wang Zhuoqi Lou Liya Pan Xiaolan Xu Chensi Wu Yongzhu Han Renmin Yang Wenbin Hu Bing Ruan |
| author_sort | Lin Chen |
| collection | DOAJ |
| description | Background and Aim. Significantly reduced serum ceruloplasmin (Cp) is the most important clue in the diagnosis of Wilson’s disease (WD) and is well known to clinicians. The false increase in Cp in some WD patients, which overlaps with that in non-WD liver disease patients, decreases the diagnostic accuracy. The aims of our study were to understand the factors associated with Cp normalisation in WD patients and identify these WD patients using usual predictors. Methods. We retrospectively screened individuals with serum Cp ≥ 140 mg/L from 1032 WD patients who were hospitalised for the first time. Logistic regression analyses were performed in a case-control study between the WD cohort and another liver disease cohort to explore the independent risk factors for WD diagnosis and establish a regression model to identify them. The follow-up medical records of the WD cohort were subjected to mixed-effects model analysis in a longitudinal study to discover factors associated with Cp normalisation. Results. Eighty-six WD patients and their 353 medical records and another 98 non-WD liver disease patients were included in the present study. Cp normalisation was significantly associated with the copper burden and liver function indexes, such as urinary copper, γ-glutamyltransferase, and albumin (p≤0.001). Logistic regression analysis showed that age and serum creatinine (p≤0.001) were independent risk factors associated with WD. The AUC value of the regression model in the total cohort was 0.926 (p≤0.001). At a cutoff value of ≥0.617 and ≥−1, the positive and negative predictive values were both 90.8% for WD. Conclusion. Increased serum Cp in WD patients is related to excessive copper burden and hepatic injury, and common tests can effectively distinguish WD patients from other liver injury patients. |
| first_indexed | 2024-04-10T04:16:19Z |
| format | Article |
| id | doaj.art-a988056a310d4218b7e44ea8de340032 |
| institution | Directory Open Access Journal |
| issn | 1742-1241 |
| language | English |
| last_indexed | 2025-02-18T10:40:07Z |
| publishDate | 2023-01-01 |
| publisher | Hindawi-Wiley |
| record_format | Article |
| series | International Journal of Clinical Practice |
| spelling | doaj.art-a988056a310d4218b7e44ea8de3400322024-11-02T05:28:29ZengHindawi-WileyInternational Journal of Clinical Practice1742-12412023-01-01202310.1155/2023/9344891Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal CeruloplasminLin Chen0Yongguang Shi1Nan Wang2Zhuoqi Lou3Liya Pan4Xiaolan Xu5Chensi Wu6Yongzhu Han7Renmin Yang8Wenbin Hu9Bing Ruan10State Key Laboratory for Diagnosis and Treatment of Infectious DiseasesDepartment of NeurologyDepartment of Geriatric EndocrinologyState Key Laboratory for Diagnosis and Treatment of Infectious DiseasesState Key Laboratory for Diagnosis and Treatment of Infectious DiseasesState Key Laboratory for Diagnosis and Treatment of Infectious DiseasesState Key Laboratory for Diagnosis and Treatment of Infectious DiseasesDepartment of NeurologyDepartment of NeurologyDepartment of NeurologyState Key Laboratory for Diagnosis and Treatment of Infectious DiseasesBackground and Aim. Significantly reduced serum ceruloplasmin (Cp) is the most important clue in the diagnosis of Wilson’s disease (WD) and is well known to clinicians. The false increase in Cp in some WD patients, which overlaps with that in non-WD liver disease patients, decreases the diagnostic accuracy. The aims of our study were to understand the factors associated with Cp normalisation in WD patients and identify these WD patients using usual predictors. Methods. We retrospectively screened individuals with serum Cp ≥ 140 mg/L from 1032 WD patients who were hospitalised for the first time. Logistic regression analyses were performed in a case-control study between the WD cohort and another liver disease cohort to explore the independent risk factors for WD diagnosis and establish a regression model to identify them. The follow-up medical records of the WD cohort were subjected to mixed-effects model analysis in a longitudinal study to discover factors associated with Cp normalisation. Results. Eighty-six WD patients and their 353 medical records and another 98 non-WD liver disease patients were included in the present study. Cp normalisation was significantly associated with the copper burden and liver function indexes, such as urinary copper, γ-glutamyltransferase, and albumin (p≤0.001). Logistic regression analysis showed that age and serum creatinine (p≤0.001) were independent risk factors associated with WD. The AUC value of the regression model in the total cohort was 0.926 (p≤0.001). At a cutoff value of ≥0.617 and ≥−1, the positive and negative predictive values were both 90.8% for WD. Conclusion. Increased serum Cp in WD patients is related to excessive copper burden and hepatic injury, and common tests can effectively distinguish WD patients from other liver injury patients.http://dx.doi.org/10.1155/2023/9344891 |
| spellingShingle | Lin Chen Yongguang Shi Nan Wang Zhuoqi Lou Liya Pan Xiaolan Xu Chensi Wu Yongzhu Han Renmin Yang Wenbin Hu Bing Ruan Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin International Journal of Clinical Practice |
| title | Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin |
| title_full | Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin |
| title_fullStr | Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin |
| title_full_unstemmed | Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin |
| title_short | Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin |
| title_sort | age and serum creatinine can differentiate wilson disease patients with pseudonormal ceruloplasmin |
| url | http://dx.doi.org/10.1155/2023/9344891 |
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