Clinical manifestations and survival pattern of Wilson's disease

V. S. Richard, V. K. Harris, Shankar Viswanathan, G. Loganathan, G. M. Chandy

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Background. Wilson's disease usually presents in childhood. Early recognition and treatment is crucial to retard the progression of this disease, which can be debilitating, if not fatal. We analysed the clinical manifestations and survival pattern of patients admitted at our centre from 1993 to 1996. Methods. Hospital records of patients were reviewed to obtain the clinical manifestations. The survival status of patients was determined by a prospective follow up. The Kaplan-Meier survival curve and univariate Cox proportional hazards model were used to determine the survival pattern and risk for death in Wilson's disease. Results. A total of 60 patients were studied. The median age at onset of symptoms was 12 years (range 5-52 years). The log rank test showed a significant difference in the mean (SD) survival between patients who presented with hepatic [87.36 (15.26) months] and neurological symptoms [114.33 (9.14) months]. Cox proportional hazards analysis showed a hazard ratio of 4.9 for patients with a hepatic presentation compared to those with neurological presentation. Conclusion. The presentation of Wilson's disease is not limited to the paediatric age group. Patients with a hepatic presentation had a five-fold higher risk of mortality when compared to those with a neurological presentation.

Original languageEnglish (US)
Pages (from-to)301-303
Number of pages3
JournalNational Medical Journal of India
Volume13
Issue number6
StatePublished - Nov 2000
Externally publishedYes

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Hepatolenticular Degeneration
Survival
Liver
Hospital Records
Kaplan-Meier Estimate
Age of Onset
Proportional Hazards Models
Disease Progression
Age Groups
Pediatrics
Mortality

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Richard, V. S., Harris, V. K., Viswanathan, S., Loganathan, G., & Chandy, G. M. (2000). Clinical manifestations and survival pattern of Wilson's disease. National Medical Journal of India, 13(6), 301-303.

Clinical manifestations and survival pattern of Wilson's disease. / Richard, V. S.; Harris, V. K.; Viswanathan, Shankar; Loganathan, G.; Chandy, G. M.

In: National Medical Journal of India, Vol. 13, No. 6, 11.2000, p. 301-303.

Research output: Contribution to journalArticle

Richard, VS, Harris, VK, Viswanathan, S, Loganathan, G & Chandy, GM 2000, 'Clinical manifestations and survival pattern of Wilson's disease', National Medical Journal of India, vol. 13, no. 6, pp. 301-303.
Richard VS, Harris VK, Viswanathan S, Loganathan G, Chandy GM. Clinical manifestations and survival pattern of Wilson's disease. National Medical Journal of India. 2000 Nov;13(6):301-303.
Richard, V. S. ; Harris, V. K. ; Viswanathan, Shankar ; Loganathan, G. ; Chandy, G. M. / Clinical manifestations and survival pattern of Wilson's disease. In: National Medical Journal of India. 2000 ; Vol. 13, No. 6. pp. 301-303.
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