To the content
4 . 2019

The new aspects of clinical nutrition at Wilson disease: actuality and perspectives

Abstract

Wilson disease is hereditary disorder of copper metabolism, based on defect of cooper excretion, which leads to accumulation of cooper in the liver and brain. This disease is one of the most difficult to diagnose. Without treatment disease brings to early disability and lethal outcome. In the article, domestic and foreign approaches to dietary management of Wilson disease have been compared. Diet is not recommended as sole therapy. The degree of restriction of the products containing copper now is discussed. According to the Russian clinical guidelines of diagnosis and treatment of Wilson disease exception of products, copper content in which exceeds 0.5 mg/100 g (liver, shellfish, nuts, cocoa products, mushrooms, bean and some grains) is recommended, while in EASL clinical guidelines there are no any information about restriction of the products containing copper. It is necessary to pay attention not only to cooper restriction, but also to qualitative components of diet. Protein is important part of nutrition under liver disease. According to ESPEN guidelines, the recommended protein intake at chronic hepatitis and cirrhosis is 1.2-1.5 g/kg/day. Dairy products and whey protein are good sources of protein, they almost do not contain cooper, therefore they can be used without restrictions at Wilson disease (in case of normal lactose and milk protein tolerance). The reduce of consumption of sugar, refined carbohydrates and trans fats is also recommended. Dietary recommendations must take into consideration the nutrition status of the patient (protein energy malnutrition, normal body weight, obesity) and degree of liver damage (chronic hepatitis, cirrhosis). It is necessary to develop individualization of diet, increasing efficiency of medicinal treatment of Wilson disease.

Keywords:Wilson disease, cooper, protein energy malnutrition

For citation: Baranovsky A.Yu., Belodedova AS., Fedorova T.F., Palgova L.K., Grigoreva E.Yu. The new aspects of clinical nutrition at Wilson disease: actuality and perspectives. Voprosy pitaniia [Problems of Nutrition]. 2019; 88 (4): 12-7. doi: 10.24411/0042-8833-2019-10036 (in Russian)

References

1. Hramova E.B., Horosheva E.Yu., Gorohova N.E. Difficult diagnosis: patient with Wilson disease. Meditsinskaya nauka i obrazovaniye Urala [Medical Science and Education of the Urals]. 2017; (2): 128-31. (in Russian)

2. Russian clinical guidelines of diagnosis and treatment of Wilson disease. Moscow, 2015. URL: https://mosgorzdrav.ru/ru-RU/science/ 15. default/download/79.html (in Russian)

3. Balashova M.S., Solov'eva O.V., Fastovets S.V., Tuluzanovskaya I.G., Filimonov M.I., Bayazutdinova G.M., et al. The clinical value 16. of ATP7B sequencing in the diagnosis of Wilson's disease. Meditsin-skaya genetika [Medical Genetics]. 2016; 15 (7): 14-6. (in Russian)

4. Gupta A., Lutsenko S. Human copper transporters: mechanism, role in human diseases and therapeutic potential. Future Med Chem. 17. 2009; 1 (6): 1125-42.

5. Czfonkowska A., Schilsky M.L. Wilson disease. Handbook of clinical neurology. Elsevier, 2017: 248 p.

6. Koroy P.V. Wilson disease. Part I: Etiology, pathogenesis, clinical manifestations and screening. Vestnik molodogo uchenogo [Bulletin of Young Scientist]. 2014; 7 (3-4): 56-63. (in Russian)

7. Ferenci P., Caca K., Loudianos G., Mieli-Vergani G., Tanner S., Sternlieb I., et al. Diagnosis and phenotypic classification of Wilson disease. Liver Int. 2003; 23: 139-42.

8. Kathawala M., Hirschfield G.M. Insights into the management of Wilson's disease. Ther Adv Gastroenterol. 2017; 10 (11): 889-905.

9. EASL Clinical Practice Guidelines: Wilson's disease. J Hepatol. 2012; 56 (3): 671-85. URL: https://doi.org/10.1016/jjhep.2011.11.007

10. Roberts E.A., Schilsky M.L. American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008; 47: 2089-111.

11. Skurihin I.M., Volgarev M.N. The chemical composition of food. Book 2: Reference tables of the content of amino acids, fatty acids, vitamins, macro-and microelements, organic acids and carbohydrates. Moscow: Agropromizdat, 1987: 360 p. (in Russian)

12. Brewer G.J., Yuzbasiyan-Gurkan V., Dick R., Wang Y., Johnson V. Does a vegetarian diet control Wilson's disease? J Am Coll Nutr. 1993; 12: 527-30.

13. Brewer G.J. Wilson's disease: a clinician's guide to recognition, diagnosis, and management. Springer, 2001: 190 p.

14. Einer C., Leitzinger C., Lichtmannegger J., Eberhagen C., Rieder T., Borchard S., et al. A high-calorie diet aggravates mitochondrial dysfunction and triggers severe liver damage in Wilson disease rats. Cell Mol Gastroenterol Hepatol. 2019; 7 (7): 571-96. URL: https:// doi.org/10.1016/j.jcmgh.2018.12.005

15. Kieffer D.A., Medici V. Wilson disease: at the crossroads between genetics and epigenetics - a review of the evidence. Liver Res. 2017; 1: 121-30.

16. Narayanankutty A., Palliyil D.M., Kuruvilla K., Raghavamenon A.C. Virgin coconut oil reverses hepatic steatosis by restoring redox homeostasis and lipid metabolism in male Wistar rats. J Sci Food Agric. 2018; 98: 1757-64.

17. Otuechere C.A., Madarikan G., Simisola T., Bankole O., Osho A. Virgin coconut oil protects against liver damage in albino rats challenged with the anti-folate combination, trimethoprim-sulfamethoxazole. J Basic Clin Physiol Pharmacol. 2014; 25: 249-53.

18. Soto-Alarcon S.A., Valenzuela R., Valenzuela A., Videla L.A. Liver protective effects of extra virgin olive oil: interaction between its chemical composition and the cell-signaling pathways involved in protection. Endocr Metab Immune Disord Drug Targets. 2018; 18: 75-84.

19. Valenzuela R., Espinosa A., Llanos P., Hernandez-Rodas M.C., Barrera C., Vergara D., et al. Anti-steatotic effects of an n-3 LCPUFA and extra virgin olive oil mixture in the liver of mice subjected to high-fat diet. Food Funct. 2016; 7: 140.

20. Plauth M., Cabre E., Riggio O., et al. ESPEN guidelines on enteral nutrition: liver disease. Clin Nutr. 2006; 25 (2): 285-94.

21. Ljashhenko Yu.N. Enteral nutrition formulations in Russia. Eksperimental'naya i klinicheskaya gastoenterologiya [Experimental and Clinical Gastroenterology]. 2009; (2): 134-47. (in Russian)

22. Yonezawa K., Nakagama H., Tajima R. Effects of soy protein isolate on LEC rats, a model of Wilson disease: mechanisms underlying enhancement of liver cell damage. Biochem Biophys Res Commun. 2003; 302 (2): 271-4.

23. Zhigal'cova O.A., Lihachev S.A., Pleshko I.V. Nutritional status of patients with Wilson disease. Gastroenterologiya Sankt-Peterburga [Gastroenterology of Saint Petersburg]. 2011; (4): 10. (in Russian)

All articles in our journal are distributed under the Creative Commons Attribution 4.0 International License (CC BY 4.0 license)

SCImago Journal & Country Rank
Scopus CiteScore
CHIEF EDITOR
CHIEF EDITOR
Viktor A. Tutelyan
Full Member of the Russian Academy of Sciences, Doctor of Medical Sciences, Professor, Scientific Director of the Federal Research Centre of Nutrition, Biotechnology and Food Safety (Moscow, Russia)

Journals of «GEOTAR-Media»