Hepatic ultrastructural features distinguish paediatric Wilson disease from <scp>NAFLD</scp> and autoimmune hepatitis

Alqahtani, Saleh; Chami, Rose; Abuquteish, Dua; Vandriel, Shannon M.; Yap, Charesse; Kukkadi, Liyana; Parmar, Aishwarya; Mundh, Amrita; Roberts, Eve A.; Kamath, Binita M.; Siddiqui, Iram

doi:10.1111/liv.15319

Cited by 9 publications

(5 citation statements)

References 33 publications

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“…When this balance is disrupted, excessive copper accumulation in the liver can lead to liver damage, known as WD. Early changes of WD include hepatic steatosis, and over time, fibrosis around the portal vein progresses to cirrhosis [ 17 – 18 ]. Early diagnosis and timely treatment of cirrhosis are crucial for managing chronic liver diseases.…”

Section: Discussionmentioning

confidence: 99%

Construction of a nomogram for predicting compensated cirrhosis with Wilson’s disease based on non-invasive indicators

Li,

Wang,

Zhu

2024

BMC Med Imaging

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Background Wilson’s disease (WD) often leads to liver fibrosis and cirrhosis, and early diagnosis of WD cirrhosis is essential. Currently, there are few non-invasive prediction models for WD cirrhosis. The purpose of this study is to non-invasively predict the occurrence risk of compensated WD cirrhosis based on ultrasound imaging features and clinical characteristics. Methods A retrospective analysis of the clinical characteristics and ultrasound examination data of 102 WD patients from November 2018 to November 2020 was conducted. According to the staging system for WD liver involvement, the patients were divided into a cirrhosis group (n = 43) and a non-cirrhosis group (n = 59). Multivariable logistic regression analysis was used to identify independent influencing factors for WD cirrhosis. A nomogram for predicting WD cirrhosis was constructed using R analysis software, and validation of the model’s discrimination, calibration, and clinical applicability was completed. Due to the low incidence of WD and the small sample size, bootstrap internal sampling with 500 iterations was adopted for validation to prevent overfitting of the model. Results Acoustic Radiation Force Impulse (ARFI), portal vein diameter (PVD), and serum albumin (ALB) are independent factors affecting WD cirrhosis. A nomogram for WD cirrhosis was constructed based on these factors. The area under the ROC curve (AUC) of the model’s predictive ability is 0.927 (95% CI: 0.88–0.978). As demonstrated by 500 Bootstrap internal sampling validations, the model has high discrimination and calibration. Clinical decision curve analysis shows that the model has high clinical practical value. ROC curve analysis of the model’s rationality indicates that the model’s AUC is greater than the AUC of using ALB, ARFI, and PVD alone. Conclusion The nomogram model constructed based on ARFI, PVD, and ALB can serve as a non-invasive tool to effectively predict the risk of developing WD cirrhosis.

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Section: Discussionmentioning

confidence: 99%

Construction of a nomogram for predicting compensated cirrhosis with Wilson’s disease based on non-invasive indicators

Li,

Wang,

Zhu

2024

BMC Med Imaging

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“…We found that portal lymphoplasmacytic infiltration, lobular inflammation combined with more than moderate interface hepatitis and lymphoplasmacytic infiltration are the main features of AIH. Other chronic liver diseases such as DILI, viral hepatitis, and Wilson's disease may also be comorbid with interface hepatitis ( 39 ), which is usually mild or moderate. The lobular hepatitis with lymphoplasmacytic infiltration is considered the characteristic histological feature of acute-onset AIH, which is not included in the revised and simplified IAIHG systems ( 2 , 40 ).…”

Section: Discussionmentioning

confidence: 99%

“…AIH, autoimmune hepatitis; SLE, systemic lupus erythematosus; IgG, immunoglobulin G; GLB, seroglobulin; ULN, upper limit of normal; ANA, antinuclear antibodies; anti-LKM1, antibodies to liver-kidney microsomal1; SMA, smooth muscle antibodies; anti-LC1, antibodies to liver cytosol type1; Ro-52, Ro-52 antibodies. viral hepatitis, and Wilson's disease may also be comorbid with interface hepatitis (39), which is usually mild or moderate. The lobular hepatitis with lymphoplasmacytic infiltration is considered the characteristic histological feature of acute-onset AIH, which is not included in the revised and simplified IAIHG systems (2,40).…”

Section: Discussionmentioning

confidence: 99%

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

Ma,

Liu,

et al. 2024

Front. Pediatr.

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BackgroundChildren with autoimmune hepatitis (AIH) often present with symptoms similar to those of other liver diseases. This study consists of a comparison between the clinical and histological characteristics of AIH and those of other four AIH-like liver diseases [i.e., drug-induced liver injury (DILI), gene deficiency, infectious liver disease and other etiology of liver disease], as well as an evaluation of the AIH scoring system's diagnostic performance.MethodsAll children with AIH-like liver disease at our center from January 2013 to December 2022 were included. The clinical and histological characteristics of the AIH group were retrospectively analyzed and compared with those of the other four groups.ResultsA total of 208 children were included and divided into AIH group (18 patients), DILI group (38 patients), gene deficiency group (44 patients), infectious liver disease group (74 patients), and other etiology group (34 patients). The antinuclear antibodies (ANA) ≥ 1:320 rate was significantly higher in the AIH compared to the other four groups after multiple testing correction (p < 0.0125), while patients with positive antibodies to liver-kidney microsomal-1 (anti-LKM1, n = 3) and smooth muscle antibodies (SMA, n = 2) were only observed in the AIH group. The positive rates of antibodies to liver cytosol type1 (anti-LC1) and Ro52 were higher than those in the other four groups. The serum immunoglobulin G (IgG) and globulin levels, as well as the proportions of portal lymphoplasmacytic infiltration, lobular hepatitis with more than moderate interface hepatitis, and lobular hepatitis with lymphoplasmacytic infiltration, were significantly higher in the AIH group than in the other four groups after multiple testing correction (p < 0.0125). The cirrhosis rate in the AIH group was higher than that in the DILI and infectious liver disease groups (p < 0.0125). Both the simplified (AUC > 0.73) and the revised systems (AUC > 0.93) for AIH have good diagnostic performance, with the latter being superior (p < 0.05).ConclusionPositive autoantibodies (ANA ≥ 1:320 or anti-LKM1 positive, or accompanied by SMA, anti-LC1 or Ro-52 positive) and elevated serum IgG or globulin levels contribute to early recognition of AIH. The presence of lobular hepatitis with more than moderate interface hepatitis and lymphoplasmacytic infiltration contribute to the diagnosis of AIH.

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“…Ultrastructural findings of mitochondrial abnormalities are important to distinguish Wilson’s disease from nonalcoholic FLD and autoimmune hepatitis. Mitochondrial polymorphism, crista tip expansion, membrane replication, and matrix density in Wilson’s disease group were significantly higher than those in the other two groups [ 55 ].…”

Section: Discussionmentioning

confidence: 99%

Analysis of risk factors for fatty liver disease in children with Wilson’s disease

Jia,

Wang,

Tao

et al. 2024

European Journal of Gastroenterology &Amp; Hepatology

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Background and aims Many children with Wilson’s disease are complicated with dyslipidemia. The aim of this study was to investigate the risk factors for the development of fatty liver disease (FLD) in children with Wilson’s disease. Methods We evaluated sex, age, weight, the disease course, treatment course, clinical classification, alanine transaminase (ALT), aspartate transaminase, γ-glutamyl transpeptidase, total biliary acid, triglyceride, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, homocysteine, uric acid, fibrinogen (FBG), creatinine, procollagen III N-terminal propeptide, laminin, hyaluronic acid, type IV collagen, and performed receiver operating characteristic curve analysis to investigate the forecast value of individual biochemical predictors and combined predictive indicators to evaluate FLD in Wilson’s disease. Results The multivariate logistic regression analysis revealed that ALT [odds ratio (OR), 1.011; 95% confidence interval (CI), 1.004–1.02; P = 0.006], uric acid (OR, 1.01; 95% CI, 1.002–1.018; P = 0.017), FBG (OR, 3.668; 95% CI, 1.145–13.71; P = 0.037), creatinine (OR, 0.872; 95% CI, 0.81–0.925; P < 0.001), and laminin (OR, 1.01; 95% CI, 1.002–1.018; P = 0.017) acted as independent risk factors in Wilson’s disease complicated with FLD. The receiver operating characteristic curves for combined predictive indicators demonstrated an area under the curve values of 0.872, which was found to be a significant predictors for FLD in Wilson’s disease. Conclusions We screened out the most important risk factors, namely ALT, uric acid, creatinine, FBG, and laminin for Wilson’s disease complicated with FLD. The joint prediction achieved is crucial for identifying children with Wilson’s disease complicated with FLD.

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Hepatic ultrastructural features distinguish paediatric Wilson disease from NAFLD and autoimmune hepatitis

Cited by 9 publications

References 33 publications

Construction of a nomogram for predicting compensated cirrhosis with Wilson’s disease based on non-invasive indicators

Construction of a nomogram for predicting compensated cirrhosis with Wilson’s disease based on non-invasive indicators

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

Analysis of risk factors for fatty liver disease in children with Wilson’s disease

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