Screening for Wilson’s disease in acute liver failure: A new scoring system in children
BackgroundWilson’s disease (WD) is a rare cause of acute liver failure (ALF) and has a high fatality rate. Rapid and accurate diagnosis is important for ALF because of WD (ALF-WD). Our objective was to establish a simple, rapid, and accurate diagnostic test to distinguish ALF-WD from non-WD ALF (NWD...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2022-09-01
|
| Series: | Frontiers in Pediatrics |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2022.1003887/full |
| _version_ | 1811271767407198208 |
|---|---|
| author | Cai-Xia Feng Xiu-Qi Chen Xiao-Li He Lian-Cheng Lan Qing Tang Li Huang Qing-Wen Shan |
| author_facet | Cai-Xia Feng Xiu-Qi Chen Xiao-Li He Lian-Cheng Lan Qing Tang Li Huang Qing-Wen Shan |
| author_sort | Cai-Xia Feng |
| collection | DOAJ |
| description | BackgroundWilson’s disease (WD) is a rare cause of acute liver failure (ALF) and has a high fatality rate. Rapid and accurate diagnosis is important for ALF because of WD (ALF-WD). Our objective was to establish a simple, rapid, and accurate diagnostic test to distinguish ALF-WD from non-WD ALF (NWDALF) in children.Materials and methodsThe data from all cases with pediatric ALF were retrospectively collected and analyzed. We performed receiver operator characteristics curve (ROC) analysis and confirmed the optimum cut-off points.ResultsFifty-eight patients with pediatric ALF (12 with WD, 46 with other etiologies) were included. Older age was observed in ALF-WD compared to NWDALF (11.16 ± 2.51 years vs. 3.34 ± 3.81 years, p < 0.001). An analysis based on routine biochemical testings revealed that total bilirubin (TBil), direct bilirubin, indirect bilirubin, alanine aminotransferase (ALT), aspartate aminotransferase (AST), AST:ALT ratio, alkaline phosphatase (ALP), ALP:TBil ratio, serum albumin, gamma-glutamyl transferase, cholinesterase, hemoglobin, and platelet were statistically significant between the ALF-WD and NWDALF groups. The optimum cut-off points were obtained through ROC analysis. A scoring system was formed by assigning a score of 1 or 0 to patients who met the 13 cut-off points. Using ROC analysis, we determined a cut-off point of ≥ 6.5 for ALF-WD with 91.7% sensitivity and 97.8% specificity (p < 0.0001). In addition, a best cut-off point of ≥ 1.5 based on only five variables (ALT, AST, AST:ALT ratio, ALP, and ALP:TBil ratio), had 100% sensitivity and 91.3% specificity for ALF-WD (p < 0.0001). Based on this, when age was calculated as the sixth indicator, the best cut-off value of ≥ 2.5 had 100% sensitivity and 97.8% specificity (p < 00.0001).ConclusionOur study developed a new scoring system that consists of simple laboratory tests with good sensitivity and specificity and can be used by clinicians to quickly distinguish ALF-WD from NWDALF in children. |
| first_indexed | 2024-04-12T22:26:57Z |
| format | Article |
| id | doaj.art-2503bf80b150405abbb2b8f1bbbad694 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-12T22:26:57Z |
| publishDate | 2022-09-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-2503bf80b150405abbb2b8f1bbbad6942022-12-22T03:14:04ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602022-09-011010.3389/fped.2022.10038871003887Screening for Wilson’s disease in acute liver failure: A new scoring system in childrenCai-Xia FengXiu-Qi ChenXiao-Li HeLian-Cheng LanQing TangLi HuangQing-Wen ShanBackgroundWilson’s disease (WD) is a rare cause of acute liver failure (ALF) and has a high fatality rate. Rapid and accurate diagnosis is important for ALF because of WD (ALF-WD). Our objective was to establish a simple, rapid, and accurate diagnostic test to distinguish ALF-WD from non-WD ALF (NWDALF) in children.Materials and methodsThe data from all cases with pediatric ALF were retrospectively collected and analyzed. We performed receiver operator characteristics curve (ROC) analysis and confirmed the optimum cut-off points.ResultsFifty-eight patients with pediatric ALF (12 with WD, 46 with other etiologies) were included. Older age was observed in ALF-WD compared to NWDALF (11.16 ± 2.51 years vs. 3.34 ± 3.81 years, p < 0.001). An analysis based on routine biochemical testings revealed that total bilirubin (TBil), direct bilirubin, indirect bilirubin, alanine aminotransferase (ALT), aspartate aminotransferase (AST), AST:ALT ratio, alkaline phosphatase (ALP), ALP:TBil ratio, serum albumin, gamma-glutamyl transferase, cholinesterase, hemoglobin, and platelet were statistically significant between the ALF-WD and NWDALF groups. The optimum cut-off points were obtained through ROC analysis. A scoring system was formed by assigning a score of 1 or 0 to patients who met the 13 cut-off points. Using ROC analysis, we determined a cut-off point of ≥ 6.5 for ALF-WD with 91.7% sensitivity and 97.8% specificity (p < 0.0001). In addition, a best cut-off point of ≥ 1.5 based on only five variables (ALT, AST, AST:ALT ratio, ALP, and ALP:TBil ratio), had 100% sensitivity and 91.3% specificity for ALF-WD (p < 0.0001). Based on this, when age was calculated as the sixth indicator, the best cut-off value of ≥ 2.5 had 100% sensitivity and 97.8% specificity (p < 00.0001).ConclusionOur study developed a new scoring system that consists of simple laboratory tests with good sensitivity and specificity and can be used by clinicians to quickly distinguish ALF-WD from NWDALF in children.https://www.frontiersin.org/articles/10.3389/fped.2022.1003887/fullacute liver failureWilson’s diseasediagnosischildrenscoring systemroutine biochemical test |
| spellingShingle | Cai-Xia Feng Xiu-Qi Chen Xiao-Li He Lian-Cheng Lan Qing Tang Li Huang Qing-Wen Shan Screening for Wilson’s disease in acute liver failure: A new scoring system in children Frontiers in Pediatrics acute liver failure Wilson’s disease diagnosis children scoring system routine biochemical test |
| title | Screening for Wilson’s disease in acute liver failure: A new scoring system in children |
| title_full | Screening for Wilson’s disease in acute liver failure: A new scoring system in children |
| title_fullStr | Screening for Wilson’s disease in acute liver failure: A new scoring system in children |
| title_full_unstemmed | Screening for Wilson’s disease in acute liver failure: A new scoring system in children |
| title_short | Screening for Wilson’s disease in acute liver failure: A new scoring system in children |
| title_sort | screening for wilson s disease in acute liver failure a new scoring system in children |
| topic | acute liver failure Wilson’s disease diagnosis children scoring system routine biochemical test |
| url | https://www.frontiersin.org/articles/10.3389/fped.2022.1003887/full |
| work_keys_str_mv | AT caixiafeng screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren AT xiuqichen screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren AT xiaolihe screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren AT lianchenglan screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren AT qingtang screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren AT lihuang screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren AT qingwenshan screeningforwilsonsdiseaseinacuteliverfailureanewscoringsysteminchildren |