The DOI matches its Crossref record; 11 of the 11 references it cites were found in a registry or an index.
Wilson's disease in children: 37-Year experience and revised King's score for liver transplantation
Open accessAbstract
Wilson's disease (WD) is a rare liver-based disorder of copper metabolism. Prognostic criteria described by our group in 1986 to predict death without transplantation have not been universally validated. The clinical features of 88 children were reviewed, retrospectively in 74 and prospectively in 14. Data from the retrospectively recruited patients that died or survived on long-term chelation were used to evaluate the validity of our old scoring system and to devise a new prognostic index, then assessed in the 14 prospectively recruited patients. Using the old scoring system, 5 children scoring ≥ 7, the cutoff value for death without transplantation, survived, whereas 4 scoring ≤ 7 died (sensitivity 87% and specificity 90%). A new index based on serum bilirubin, international normalized ratio, aspartate aminotransferase (AST), and white cell count (WCC) at presentation identified a cutoff score of 11 for death and proved to be 93% sensitive and 98% specific, with a positive predictive value of 88%. When the new index was evaluated prospectively in 14 patients, it predicted the need for transplantation in only the 4 who required it, although 1 child with a score of 11 survived on medical treatment. In conclusion, the new Wilson Index is more sensitive and specific in predicting mortality without transplantation than the old scoring system, but needs to be validated in a larger number of patients. (Liver Transpl 2005;11:441–448.)
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Diagnosis of Wilson's disease: an experience over three decades
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Cited by
EASL Clinical Practical Guidelines on the management of acute (fulminant) liver failure
Wendon, J., Cordoba, J., Dhawan, A., Larsen, F. S., Manns, M., Nevens, F., Samuel, D., Simpson, K. J., Yaron, I., & Bernardi, M. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2016.12.003 · Cited by 917
Self-citationEfficacy and Safety of Oral Chelators in Treatment of Patients With Wilson Disease
Weiss, K. H., Thurik, F., Gotthardt, D. N., Schäfer, M., Teufel, U., Wiegand, F., Merle, U., Ferenci–Foerster, D., Maieron, A., Stauber, R., et al. (2013) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2013.03.012 · Cited by 254
Intrahepatic cholestasis in common chronic liver diseases
Jüngst, C., Berg, T., Cheng, J., Green, R. M., Jia, J., Mason, A. L., & Lammert, F. (2013) · European Journal of Clinical Investigation · doi:10.1111/eci.12128 · Cited by 82
Chelation therapy in liver diseases of childhood: Current status and response
Seetharaman, J., & Sarma, M. S. (2021) · World Journal of Hepatology · doi:10.4254/wjh.v13.i11.1552 · Cited by 16
Metadata
- Authors
- Dhawan, A., Taylor, R. M., Cheeseman, P., De Silva, P., Katsiyiannakis, L., & Mieli-Vergani, G.
- Venue
- Liver Transplantation
- Published
- 2005-04
- Volume
- 11
- Issue
- 4
- Pages
- 441-448
- ISSN
- 1527-6465 (ISSN-L)
- OpenAlex
- W2045623736
- Cited by
- 441
- References
- 11
- FWCI
- 11.17
- Type
- journal article
- Language
- en
- Open access
- bronze
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