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Clinical profile of adult and pediatric patients with hepatic Wilson’s disease

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Abstract

Background

The clinical profile varies in patients with Wilson’s disease (WD). There is paucity of data regarding adult and pediatric patients with hepatic WD.

Methods

As many as 140 consecutive patients diagnosed with hepatic WD between December 2006 and January 2021 were included in the study. Data was collected regarding the demographic parameters, clinical presentation, extrahepatic organ involvement, liver histology and laboratory investigations. Adult and children (0–14 years) with hepatic WD were compared regarding these features.

Result

Eighty-eight adults and 52 children were included in the study. The median age of presentation was 17 years (range: 1.1–42 years). Male preponderance was seen (adult 68/88, 69%; children 40/52, 77%). Adults as compared to children presented more commonly as cirrhosis (52/88 vs. 15/52, p = 0.0005) and with hepatic decompensation (35/88 vs. 9/52, p = 0.005). Presentation with acute-on-chronic liver failure (ACLF) was more common in children (10/52 vs. 2/88, p = 0.0005). Twenty-eight-day mortality was 50% (5/10) in children and none in adults presenting with ACLF. Nazer’s Prognostic Index (≥ 7) and New Wilson Index were more accurate in predicting mortality among children with ACLF with AUROC 1, while AARC (APASL ACLF Research Consortium) was less accurate with AUROC 0.45. Liver histology findings were similar in adults and children. Extrahepatic involvement was also similar. (8/88 in adults vs. 3/52 children, p value 0.48).

Conclusion

Most patients with WD present as cirrhosis in adulthood. ACLF is more common in children. Nazer’s prognostic index and new Wilson Index score are accurate in predicting mortality in children with ACLF.

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Authors and Affiliations

  1. Department of Gastroenterology, Seth G S Medical College and K E M Hospital, Gastroenterology Office, 9th Floor, New Building, Parel, Mumbai, 400 012, India

    Sanjay Kumar, Mohd Irtaza, Biswa Ranjan Patra, Praveen Kumar Rao, Amrit Gopan, Aditya P. Kale & Akash Shukla

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  1. Sanjay Kumar
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  2. Mohd Irtaza
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  3. Biswa Ranjan Patra
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  4. Praveen Kumar Rao
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  6. Aditya P. Kale
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  7. Akash Shukla
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Contributions

Conceptualization- AS, SK. Data curation- SK, MI, BRP, PKR Formal analysis- SK, MI, AG Funding acquisition- Not applicable. Investigation- SK, MI, BRP, PKR, AG, APK Methodology- SK, MI, BRP, PKR, AG, APK Project administration -AS, SK, MI, APK Resources - AS, SK, MI. Software- Not applicable Supervision - AS, SK. Validation- AS, SK. Visualization- SK, MI, BRP, PKR. Roles/Writing - original draft- SK, MI, AS. Writing - review & editing- SK, BRP, MI, AS.

Corresponding author

Correspondence to Akash Shukla.

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Competing interests

SK, MI, BRP, PKR, AG, APK and AS declare that they have no conflict of interest. 

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The authors are solely responsible for the data and the contents of the paper. In no way, the Honorary Editor-in-Chief, Editorial Board Members, the Indian Society of Gastroenterology or the printer/publishers are responsible for the results/findings and content of this article.

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Kumar, S., Irtaza, M., Patra, B.R. et al. Clinical profile of adult and pediatric patients with hepatic Wilson’s disease. Indian J Gastroenterol (2024). https://doi.org/10.1007/s12664-024-01586-2

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