Abstract
The role of Kayser-Fleischer rings is described in 67 patients with Wilson's disease, both in the asymptomatic and symptomatic stage of the disease during life-long therapy, which lasted up to 22 years. The rings were missing in 60% of patients in the presymptomatic stage and in 2% of those in the symptomatic stage at the time of diagnosis. The Kayser-Fleischer rings disappeared in 81% of the patients (completely in 41% and incompletely in 59%). In 2 of the 6 asymptomatic patients the rings did not reabsorb even after therapy of more than 10 years. The fading of Kayser-Fleischer rings seems to be independent not only of the stage of the disease but also of the effectiveness of the decopperizing treatment.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Bachmann H (1982) Die Wilsonsche Erkrankung in der DDR — Ergebnisse genealogischer, epidemiologischer, formal- und populations-genetischer Untersuchungen und ihre Anwendung in diagnostischer Erfassung und genetischer Beratung. Dtsch Gesundheitswes 37:1228–1229
Bachmann H, Lößner J, Biesold D (1979) Untersuchungen zur Wilsonschen Erkrankung in der DDR, Teil I. Genetik und Epidemiologie. Z Gesamte Inn Med 34:744–748
Belkin M, Chajek T, Zeimer R, Friedman G, Melemed E (1976) Non-invasive quantitation of corneal copper in hepatolenticular degeneration (Wilson's disease). Lancet I:391–392
Biesold D, Günther K (1972) An improved method for investigation of copper metabolism in patients with Wilson's disease using64Cu. Clin Chim Acta 42:353–359
Biesold D, Lößner J, Bachmann H, Günther K (1980) Asymptomatic cases with Wilson's disease. In: Di Benedetta C (eds) Multidisciplinary approach to brain development. Elsevier/North Holland Biomedical Press, Amsterdam, pp 537–546
Brand I, Takats I (1951) Histochemische Untersuchungen des Kayser-Fleischerschen Hornhautringes. Graefe's Arch Clin Exp Ophthalmol 151:391–398
Deiss A, Lynch RE, Lee GR, Cartwright GE (1971) Long-term therapy of Wilson's disease. Ann Intern Med 57:57–65
Dobyns WB, Goldstein NP, Gordon H (1979) Clinical spectrum of Wilson's disease (hepatolenticular degeneration). Mayo Clin Proc 54:35–42
Harry J, Tripathi R (1970) Kayser-Fleischer ring. A pathological study. Br J Ophthalmol 54:794–800
Hiti H, Harpf H, Wutz W, Schmut O (1980) Die Rückbildung eines Kayser-Fleischer-Ringes bei Morbus Wilson unter Penicillamintherapie. Klin Monatsbl Augenheilkd 176: 235–238
Johnson BL (1973) Ultrastructure of the Kayser-Fleischer ring. Am J Ophthalmol 76:455–461
Johnson RE, Campbell RJ (1982) Wilson's disease. Electron microscopic, X-ray energy spectroscopic, and atomic absorption spectroscopic studies of corneal copper deposition and distribution. Lab Invest 46:564–569
Levi AJ, Sherlock Sh, Scheuer PJ, Cumings JN (1967) Presymptomatic Wilson's disease. Lancet II: 575–579
Lößner J, Storch W, Bachmann H, Biesold D, Kühn HJ (1980) Untersuchungen zur Wilsonschen Erkrankung in der DDR, Teil III. Diagnose und Therapie. Z Gesamte Inn Med 35:161–166
Mitchell AM, Heller GL (1968) Changes in Kayser-Fleischer ring during treatment of hepatolenticular degeneration. Arch Ophthalmol 80:622–624
Sternlieb I (1966) The Kayser-Fleischer ring. Med Radiogr Photogr 42:13–15
Sternlieb I, Scheinberg IH (1964) Penicillamine therapy for hepatolenticular degeneration. JAMA 189:748–754
Sussman W, Scheinberg IH (1969) Disappearance of Kayser-Fleischer rings. Arch Ophthalmol 82:738–741
Walshe JM (1976) Wilson's disease (hepatolenticular degeneration). In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 27, part I. North-Holland, Amsterdam, pp 379–414
Walshe JM (1983) Hudson memorial lecture: Wilson's disease. Genetics and biochemistry — their relevance to therapy. J Inherited Metab Dis 6 [Suppl 1]: 51–58
Werlin StL, Grand RJ, Perman JA, Watkins JB (1978) Diagnostic dilemmas of Wilson's disease: diagnosis and treatment. Pediatrics 62:47–51
Author information
Authors and Affiliations
Additional information
This study was supported by the Ministerium für Gesundheitswesen der Deutschen Demokratischen Republik
Rights and permissions
About this article
Cite this article
Lößner, A., Lößner, J., Bachmann, H. et al. The Kayser-Fleischer ring during long-term treatment in Wilson's disease (hepatolenticular degeneration). Graefe's Arch Clin Exp Ophthalmol 224, 152–155 (1986). https://doi.org/10.1007/BF02141489
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02141489