MODERN ASPECTS OF PATHOGENETIC TREATMENT WITH ZINC SALTS OF PATIENTS WITH WILSON'S DISEASE IN UKRAINE

  • Ivan Voloshyn-Gaponov State Institution Institute of Neurology, Psychiatry and Narcology of the National Academy of Medical Sciences of Ukraine, Ukraine
Keywords: hepatocerebral degeneration, clinical and neurological symptoms, treatment, zinc salts, penicillamine, trientine, tetrathiomolybdate, Wilson’s disease

Abstract

The aim – to study the effectiveness of zinc salts in the treatment of patients with neurological forms of Wilson's disease (WD).

Materials and methods. The analysis of the treatment results of 128 patients with hepatocerebral degeneration (71 men and 57 women) in the State Institution “Institute of Neurology, Psychiatry and Narcology of the National Academy of Medical Sciences of Ukraine” was carried out. At the time of hospitalization, the age of patients ranged from 5 to 55 years, an average of 27.3 years, and at the time of debut of the disease - from 1 year to 40 years, an average of 21.3 years. 33 patients underwent monotherapy with zinc salts, 63 – combined therapy with small doses of penicillamine and zinc salts, 32 – monotherapy with penicillamine.

Results. Because of the treatment, 67.1 % of patients showed an improvement in neuropsychiatric status: speech improved significantly, tremor of the extremities and the amplitude of hyperkinesis decreased, muscle tone decreased, and cognitive functions improved. According to the international two-level rating scale (UWDRS), the total pathology index decreased by 21 points. Zinc salts are effective and low-toxic and can be the drug of choice in the treatment of patients with hepatocerebral degeneration in the pre-symptomatic stage of the disease, as well as at the stage of maintenance treatment, both as monotherapy and in combination with penicillamine. However, zinc salts and penicillamine are not enough for the treatment and rehabilitation of patients with hepatocerebral degeneration. Therefore, taking into account the clinical picture and the data of additional research methods, it is necessary to conduct courses of symptomatic treatment at least 1-2 times a year.

Conclusions. Thus, it can be noted that zinc salts are very effective and low toxic, and, therefore, can be the drug of choice in the treatment of patients with HCD in the pre-symptomatic stage of the disease, as well as at the stage of maintenance therapy as a single drug, and in combination with penicillamine or other chelate drugs.

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Author Biography

Ivan Voloshyn-Gaponov, State Institution Institute of Neurology, Psychiatry and Narcology of the National Academy of Medical Sciences of Ukraine

MD, Associate Professor, Leading Researcher

Department of Sleep medicine Department of Autoimmune and degenerative diseases of nervous system.  Center of multiple sclerosis

Professor

Department of Clinical Neurology, Psychiatry and Narcology

References

Roberts, E. A., Schilsky, M. L. (2008). Diagnosis and treatment of Wilson disease: An update. Hepatology, 47 (6), 2089–2111. doi: http://doi.org/10.1002/hep.22261

Tsivkovskii, R., Eisses, J. F., Kaplan, J. H. et. al. (2002). Functional properties of the copper-transporting ATPase ATP7B (the Wilson’s disease protein) expressed in insect cells. Journal of Biological Chemistry, 277 (2), 976–983. doi: http://doi.org/10.1074/jbc.m109368200

Brewer, G. J., Askari, F., Lorincz, M. T., Carlson, M., Schilsky, M., Kluin, K. J. et. al. (2006). Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease. Archives of Neurology, 63 (4), 521–527. doi: http://doi.org/10.1001/archneur.63.4.521

Walshe, J. M. (1956). Penicillamine, a new oral therapy for Wilson’s disease. The American Journal of Medicine, 21, 487—495. doi: http://doi.org/10.1016/0002-9343(56)90066-3

Lowette, K. F., Desmet, K., Witters, P., Laleman, W., Verslype, C., Nevens, F. et. al. (2010). Wilsonʼs disease: long-term follow-up of a cohort of 24 patients treated with D-penicillamine. European Journal of Gastroenterology & Hepatology, 22 (5), 564–571. doi: http://doi.org/10.1097/meg.0b013e3283353df8

Merle, U., Schaefer, M., Ferenci, P., Stremmel, W. (2007). Clinical presentation, diagnosis and long-term outcome of Wilson’s disease: a cohort study. Gut, 56, 115–120. doi: http://doi.org/10.1136/gut.2005.087262

Czlonkowska, A., Gajda, J., Rodo, M. (2006). Effectsof long-term treatment in Wilson’s disease with Dpenicillamine and zinc sulphate. Journal of Neurology, 243, 269–273. doi: http://doi.org/10.1007/bf00868525

Schouwink, G. (1961). Dehepatocerebrale degeneratie, met een onderzoek naar de koperstofwisseling. University of Amsterdam.

Medici, V., Trevisan, C. P., D’Incj, R., Barollo, M., Zancan, L., Fagiuoli, S. et. al. (2006). Diagnosis and management of Wilson’s disease: results of a single center experience. Journal of Clinical Gastroenterology, 40 (10), 936–941. doi: http://doi.org/10.1097/01.mcg.0000225670.91722.59

Sinha, S., Taly, A. B. (2008). Withdrawal of penicillamine from zinc sulphatepenicillamine maintenance therapy in Wilson’s disease: promising, safe and cheap. Journal of the Neurological Sciences, 264 (1-2), 129–132. doi: http://doi.org/10.1016/j.jns.2007.08.006

Wiggelinkhuizen, M., Tilanus, M. E. C., Bollen, C. W., Houwen, R. H. J. (2009). Systematic review: clinical efficacy of chelator agents and zinc in the initial treatment of Wilson disease. Alimentary Pharmacology & Therapeutics, 29 (9), 947–958. doi: http://doi.org/10.1111/j.1365-2036.2009.03959.x

Linn, F. H. H., Houwen, R. H. J., van Hattum, J., van der Kleij, S., van Erpecum, K. J. (2009). Long-term exclusive zinc monotherapy in symptomatic Wilson disease: Experience in 17 patients. Hepatology, 50 (5), 1442–1452. doi: http://doi.org/10.1002/hep.23182

Chang, H., Xu, A., Chen, Z., Zhang, Y., Tian, F., Li, T. (2013). Long-term effects of a combination of D-penicillamine and zinc salts in the treatment of Wilson’s disease in children. Experimental and Therapeutic Medicine, 5 (4), 1129–1132. doi: http://doi.org/10.3892/etm.2013.971

Weiss, K. H., Gotthardt, D. N., Klemm, D., Merle, U., Ferenci–Foerster, D., Schaefer, M. et. al. (2011). Zinc Monotherapy Is Not as Effective as Chelating Agents in Treatment of Wilson Disease. Gastroenterology, 140 (4), 1189–1198. doi: http://doi.org/10.1053/j.gastro.2010.12.034

Hoogenraad, T. U. (2006). Paradigm shift in treatment of Wilson’s disease: zinc therapy now treatment of choice. Brain and Development, 28, 141–146. doi: http://doi.org/10.1016/j.braindev.2005.08.008

Aggarwal, A., Aggarwal, N., Nagral, A., Jankharia, G., Bhatt, M. (2009). A Novel Global Assessment Scale for Wilson’s disease (GAS for WD). Movement Disorders, 24, 509–518. doi: http://doi.org/10.1002/mds.22231

Shimizu, N., Fujiwara, J., Ohnishi, S., Sato, M., Kodama, H., Kohsaka, T. et. al. (2010). Effects of long-term zinc treatment in Japanese patients with Wilson disease: efficacy, stability, and copper metabolism. Translational Research, 156 (6), 350–357. doi: http://doi.org/10.1016/j.trsl.2010.08.007

Stremmel, W. (1991). Wilson Disease: Clinical Presentation, Treatment, and Survival. Annals of Internal Medicine, 115 (9), 720–726. doi: http://doi.org/10.7326/0003-4819-115-9-720

Rakhimova, O. Iu. (2005). Varianty porazheniia pochek pri bolezni Vilsona – Konovalova. Moscow.


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Published
2019-12-09
How to Cite
Voloshyn-Gaponov, I. (2019). MODERN ASPECTS OF PATHOGENETIC TREATMENT WITH ZINC SALTS OF PATIENTS WITH WILSON’S DISEASE IN UKRAINE. Technology Transfer: Innovative Solutions in Medicine, 36-40. https://doi.org/10.21303/2585-663.2019.001094
Section
Medicine and Dentistry