Nutritional status at Wilson’s disease and its effect on oxidative stress

Aim. To study characteristic features of nutritional status of patients with Wilson ’s disease depending on stage of liver damage and determine the impact of malnutrition on oxidative stress level.

Methods. The study included 73 subjects, 33 (15 men and 18 women) had a confi rmed diagnosis of Wilson’s disease. All patients with Wilson’s disease (the study group) had liver damage: non-cirrhotic stages — 12 (36.3%), cirrhosis — 21 (63.6%). The control group consisted of 40 healthy subjects (20 men and 20 women) with neither body weight defi ciency, nor obesity, nor liver disease. All patients were conducted standard blood tests (clinical and biochemical blood tests), measuring  malonodialdehyde (MDA) in blood serum by high-performance liquid chromatography, as well as assessment of height, weight and BMI (Body Mass Index) and analysis the body mass composition with bioelectricalimpedanceanalyzer “Diamant AIST”.

Results. 1. Malnutrition was more common among patients with liver cirrhosis (28.6%) than in patients with non-cirrhotic stages (8.3%).
2. The nutritional status of patients with liver cirrhosis was signifi cantly diff erent from the control group: BMI, total protein and albumin levels, lymphocytes and arm muscular circumference were signifi cantly lower in patients with liver cirrhosis compared to the control group. The decrease in fat and muscular mass was more pronounced among women than men with liver cirrhosis.
3. The group of patients with chronic hepatitis did not significantly statistically diff er from the control group in most nutritional status parameters, except for albumin level, which was signifi cantly lower in persons of both sexes with noncirrhotic stages; total protein level and body fat percent were signifi cantly higher in men with non-cirrhotic stages than in the control group.
4. The marker of oxidative stress level was signifi cantly higher in patients with liver cirrhosis, and was higher in patients with malnutrition.

1. Russian clinical guidelines of diagnosis and treatment of Wilson disease. Moscow, 2015. Available at: https://mosgorzdrav.ru/ru-RU/science/default/download/79.html

2. Kathawala M., Hirschfield G. M. Insights into the management of Wilson’s disease // Therapeutic Advances in Gastroenterology. 2017, vol. 10 (11), pp. 889–905.

3. Koroj P. V. Bolezn' Vil'sona-Konovalova. Chast' I: Jetiologija, patogenez, klinicheskie projavlenija i skrining [Wilson disease. Part I: Etiology, pathogenesis, clinical manifestations and screening].Vestnik molodogo uchenogo – Bulletin of young scientist, 2014, vol. 7, no. 3–4, pp. 56–63.

4. Ferenci P., Caca K., Loudianos G. et al. Diagnosis and phenotypic classification of Wilson disease. Liver International. 2003, no. 23, pp. 139–142.

5. Clinical guidelines of Union of Pediatricians of Russia “Disorders of copper metabolism (Wilson disease) in children, 2016”. Available at: http://astgmu.ru/wp-content/uploads/2018/10/Narusheniya-obmena-medi-boleznVilsona-u-detej-2016.pdf

6. Saunders J., Brian A., Wright M. et al. Malnutrition and nutrition support in patients with liver disease. Frontline Gastroenterology. 2010, vol. 1, pp.105–111.

7. W. Hermann. Classification and differential diagnosis of Wilson’s disease. Ann Transl Med. 2019, vol. 7, no. 2, p. 63. doi: 10.21037/atm.2019.02.07

8. Sini M., Sorbello O, Civolani A. et al. Non-invasive assessment of hepatic fibrosis in a series of patients with Wilson's Disease. Dig Liver Dis. 2012, vol. 44, no. 6, pp.487–491. doi: 10.1016/j.dld.2011.12.010

9. Joseph T., Reddy V., Girish P. V. et al. Transient Elastography (Fibroscan) Assessment in Patients with Hepatic and Neurological Wilson's Disease. Clinical and experimintal hepatology. 2016, vol. 6, no. 1, pp. 93–94 doi: 10.1016/j.jceh.2016.06.157

10. Jashin Ja.I., Jashin Ja. I. Vysokojeffektivnaja zhidkostnaja hromatografija markerov okislitel'nogo stressa [High performance liquid chromatography of oxidative stress markers]. Analitika – Analytics, 2011, no. 1. pp. 34–43.

11. Baranovsky A. Ju. Dietologija 5-e izd [Dietology 5th ed.] / pod red. A. Ju. Baranovskogo [under A. Y. Baranovsky ed.]. St. Petersburg, Piter Publ., 2017, 1104 p.

12. Horoshilov I. E. Sarkopenija u bol'nyh: vozmozhnosti diagnostiki i perspektivy lechenija [Sarcopenia in patients: diagnosis possibilities and therapy perspective]. Lechashhij vrach – Attending physician, 2017, no. 8, pp. 36–40.

13. Zajcev V. M., Lifljandskij V. G., Marinkin V. I. Prikladnaja medicinskaja statistika [Applied medical statistics]. St. Petersburg, Foliant Publ., 2003, 432 p.

14. Rivera-Irigoin R, Abilés J. Nutritional support in patients with liver cirrhosis. Gastroenterol Hepatol. 2012; 35: 594–601.

15. Koofy, N.E., Moawad, E.M.I., Fahmy, M. et al. Anthro po metric, biochemical and clinical assessment of malnutrition among Egyptian children with chronic liver diseases: a single institutional cross-sectional study. BMC Gastroenterol. 2019; 19; 223.

16. Pashayee-Khamene F, Saber-Firoozi M, Hatami B, et al. Food groups intake of cirrhotic patients, comparison with the nutritional status and disease stage. Gastroenterol Hepatol Bed Bench. 2019; 12(3): 226–232.

17. Zhigal'cova O. A., Lihachev S. A., Pleshko I. V. Status pitanija u pacientov s bolezn'ju Vil'sona-Konovalova [Nutritional status of patients with Wilson disease]. Gastrojenterologija Sankt-Peterburga [Gastroenterology of Saint-Petersburg]. 2011, no.4, 10 P.

18. Paradies G, Paradies V, Ruggiero FM, Petrosillo G. Oxidative stress, cardiolipin and mitochondrial dysfunction in nonalcoholic fatty liver disease. World J Gastroenterol. 2014; 20(39): 14205–14218

19. Masarone M., Rosato V., Dallio M. Role of Oxidative Stress in Pathophysiology of Nonalcoholic Fatty Liver Disease. Oxidative medicine and cellular longevity. 2018, no.3, pp.1–14

20. T. E. Promenasheva, L. S. Kolesnichenko, N. M. Kozlova. Rol' oksidativnogo stressa i sistemy glutationa v patogeneze nealkogol'noj zhirovoj bolezni pecheni [Role of oxidative stress and glutathione system in pathogenesis of non-alcoholic fatty liver disease]. Bjulleten' VSNC SO RAMN – Bulletin of Eastern-Siberian scientific centre of the Sibirian section of the Russian Academy of Medical Sciences, 2014, no. 5(99). pp.80–83.

21. Nagasaka, H., Inoue, I., Inui, A. et al. Relationship between oxidative stress and antioxidant systems in the liver of patients with Wilson disease: Hepatic manifestation in Wilson disease as a consequence of augmented oxidative stress. Pediatric Research. 2006; 60: 472–477.

22. Kalita, J., Kumar, V., Misra, U. K. et al. A study of oxidative stress, cytokines and glutamate in Wilson disease and their asymptomatic siblings. Journal of Neuroimmunology. 2014; 274 (1–2): 141–148.

23. Sarode G.V., Kim K., Kieff er D. A. et al. Metabolomics profi les of patients with Wilson disease reveal a distinct metabolic signature. Metabolomics. 2019; 15: 43.

24. Bañuls, C., de Marañón, A.M., Castro-Vega, I. et al. Role of Endoplasmic Reticulum and Oxidative Stress Parameters in the Pathophysiology of Disease-Related Malnutrition in Leukocytes of an Outpatient Population. Nutrients. 2019; 11(8): 1838.