[Evaluating the efficacy of diet therapy with protein component modification at Wilson disease].

Wilson disease (WD) is a rare hereditary disorder of copper metabolism, based on of the ATP7B gene mutation, resulting in defect of cooper excretion, which leads to accumulation of cooper in tissues and internal organs (especially in the liver and brain). The basic principle of diet therapy for patients with WD is a diet with reduced copper content, adherence to which is accompanied by significant dietary restrictions, so patients with WD, compared to other liver diseases, represent the most difficult contingent for adjustment of diet. The aim: to assess of the effect of diet therapy with modification of the protein component on nutritional status of patients with WD. Material and methods. The study included 33 patients (15 men and 18 women, 31.4±10.2 years old) with WD. All patients had liver damage: non-cirrhotic stages (NCC) - in 12 (36.3%) patients, liver cirrhosis (LC) - in 21 (63.7%) patients. Out of the last, 14 (66.7%) patients had compensated LC, 7 (33.3%) patients had decompensated LC. The average age of the patients. All patients were divided into two groups, comparable by body mass index. For 2 months outpatients of the 1st group (n=17) received a specialized diet with a modification of the protein component, made by incorporating 20 g of dry composite protein mix (containing 50% protein in the form of milk protein concentrate, 4% dietary fiber) into the daily diet. Outpatients of the 2nd group (n=16) received the same diet without modification. All patients were provided anthropometry, including shoulder circumference and triceps skin-fold measurement, and analysis of the body mass composition with bioimpedance analyzer, the index of lean mass was additionally calculated. Clinical and biochemical blood tests were also conducted for all patients. Results and discussion. As a result of the diet therapy, statistically significant (p<0.05) changes were observed in patients of the 1st group who received a diet with a modification of the protein component: an increase in the index of lean mass (by 3.0%) and circumference of the shoulder muscles (by 2.3%), serum total protein and albumin (by 7.9 and 6.1%), an increase in the absolute number of lymphocytes (by 18.8%) and decrease in serum total bilirubin (by 20.2%). A statistically significant decrease in the level of free copper was observed in both groups (by 2.1 and 1.8 fold). Conclusion. The use of a specialized diet with a modification of the protein component, based on the inclusion of a protein composite dry mix in the diet, improves the nutritional status indicators in patients with Wilson disease.

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

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.

[The Russian consensus on the diagnosis and treatment of chronic pancreatitis: Enzyme replacement therapy]. / Rossiiskii konsensus po diagnostike i lecheniiu khronicheskogo pankreatita: zamestitel'naia fermentnaia terapiia.

Pancreatology Club Professional Medical Community, 1A.S. Loginov Moscow Clinical Research and Practical Center, Moscow Healthcare Department, Moscow; 2A.I. Evdokimov Moscow State University of Medicine and Dentistry, Ministry of Health of Russia, Moscow; 3Kazan State Medical University, Ministry of Health of Russia, Kazan; 4Kazan (Volga) Federal University, Kazan; 5Far Eastern State Medical University, Ministry of Health of Russia, Khabarovsk; 6Morozov City Children's Clinical Hospital, Moscow Healthcare Department, Moscow; 7I.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint Petersburg; 8Siberian State Medical University, Ministry of Health of Russia, Tomsk; 9M.F. Vladimirsky Moscow Regional Research Clinical Institute, Moscow; 10Maimonides State Classical Academy, Moscow; 11V.I. Razumovsky State Medical University, Ministry of Health of Russia, Saratov; 12I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow; 13S.M. Kirov Military Medical Academy, Ministry of Defense of Russia, Saint Petersburg; 14Surgut State Medical University, Ministry of Health of Russia, Surgut; 15City Clinical Hospital Five, Moscow Healthcare Department, Moscow; 16Nizhny Novgorod Medical Academy, Ministry of Health of Russia, Nizhny Novgorod; 17Territorial Clinical Hospital Two, Ministry of Health of the Krasnodar Territory, Krasnodar; 18Saint Petersburg State Pediatric Medical University, Ministry of Health of Russia, Saint Petersburg; 19Rostov State Medical University, Ministry of Health of Russia, Rostov-on-Don; 20Omsk Medical University, Ministry of Health of Russia, Omsk; 21Russian Medical Academy of Postgraduate Education, Ministry of Health of Russia, Moscow; 22Novosibirsk State Medical University, Ministry of Health of Russia, Novosibirsk; 23Stavropol State Medical University, Ministry of Health of Russia, Stavropol; 24Kemerovo State Medical University, Ministry of Health of Russia, Kemerovo; 25N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, Moscow; 26A.M. Nikiforov All-Russian Center of Emergency and Radiation Medicine, Russian Ministry for Civil Defense, Emergencies and Elimination of Consequences of Natural Disasters, Saint Petersburg; 27Research Institute for Medical Problems of the North, Siberian Branch, Russian Academy of Sciences, Krasnoyarsk; 28S.P. Botkin City Clinical Hospital, Moscow Healthcare Department, Moscow; 29Tver State Medical University, Ministry of Health of Russia, Tver The Russian consensus on the diagnosis and treatment of chronic pancreatitis has been prepared on the initiative of the Russian Pancreatology Club to clarify and consolidate the opinions of Russian specialists (gastroenterologists, surgeons, and pediatricians) on the most significant problems of diagnosis and treatment of chronic pancreatitis. This article continues a series of publications explaining the most significant interdisciplinary consensus statements and deals with enzyme replacement therapy.

[All-Russian Consensus on Diagnosis and Treatment of Celiac Disease in Children and Adults]. / Vserossiiskii konsensus po diagnostike i lecheniyu tseliakii u detei i vzroslykh.

The paper presents the All-Russian consensus on the diagnosis and treatment of celiac disease in children and adults, which has been elaborated by leading experts, such as gastroenterologists and pediatricians of Russia on the basis of the existing Russian and international guidelines. The consensus approved at the 42nd Annual Scientific Session of the Central Research Institute of Gastroenterology on Principles of Evidence-Based Medicine into Clinical Practice (March 2-3, 2016). The consensus is intended for practitioners engaged in the management and treatment of patients with celiac disease. Evidence for the main provisions of the consensus was sought in electronic databases. In making recommendations, the main source was the publications included in the Cochrane Library, EMBASE, MEDLINE, and PubMed. The search depth was 10 years. Recommendations in the preliminary version were reviewed by independent experts. Voting was done by the Delphic polling system.

[THE ROLE OF NUTRITION IN THE GENESIS OF-HEPATIC OSTEODYSTROPHY IN PRIMARY BILIARY CIRRHOSIS].

THE PURPOSE: To evaluate the effect of diet on the development of hepatic osteodystrophy in patients with primary biliary cirrhosis. MATERIALS AND METHODS: In 24 women with PBC, including signs of autoimmune hepatitis (PBC/AIH)--9 persons and 19 women in the control group were analyzed the daily diet, body composition, as well as bone mineral density in the lumbar spine and femur by dual-energy X-ray adsorbtsiometrii (DEXA). THE RESULTS: In PBC incidence of osteoporosis and severe osteoporosis was 22.7% and 9.1%, respectively, and osteopenia--59.1%, with a lesion predominantly lumbar spine. Inadequate intake of calcium in patients with PBC is associated with lower density of the femur. The high protein content in the diet has a protective effect on bone mineral density, associating with the development of osteopenia.

[DIETARY PREVENTION OF PROTEIN-ENERGY MALNUTRITION IN ELDERLY PATIENTS IN THE POSTOPERATIVE PERIOD].

The occurrence of protein-energy malnutrition in the late postoperative period in elderly patients with gastroduodenal ulcers is an indication for the earliest possible appointment of a specialized diet the prevention of this complication. Specific dietary recommendations for the prevention of protein-energy malnutrition in the postoperative period were given.

[THE POSSIBILITY OF DIET THERAPY AND TYPICAL ERRORS IN THE NUTRITION OF PATIENTS WITH BILIARY TRACT DISEASES].

This lecture for the doctors considered the possibility of using power to influence the basic mechanisms of pathogenesis in diseases of the biliary: cholestatic syndrome, impaired physical-chemical properties of bile, inflammation, biliary tract and intestines motor activity disfunction. The clinical case analyzed typical mistakes in nutrition of patients with disorders of the biliary tract.

[Role of transforming growth factor-beta in the development of some liver diseases].

AIM: To investigate the expression of transforming growth factor-beta 1 (TGF-beta1) in the liver tissue of patients with autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and chronic hepatitis C (CHC). MATERIALS AND METHODS: Liver biopsy specimens were histologically and immunohistologically examined in 37 patients with verified liver diseases (12 with AIH, 15 with PBC, and 10 with CHC). RESULTS: The expression of TGF-beta1 in the non-parenchymal liver cells of the patients with AIH and PBC with liver cirrhosis was statistically significantly higher than in those without the latter (p = 0.01 and p = 0.04, respectively). There was a positive relationship between the stage of fibrosis and the absolute content TGF-beta1 and CD68+ cells in the portal tracts (r = 0.51). The higher expression of TGF-beta1 was found in the patients with HCV and AIH than in those with PBC (p = 0.03 and p < 0.05, respectively). There were no differences in the expression of TGF-beta1 in the patients with AIH as compared to those with CHC (p = 0.55). The number of CD68-positive macrophages was higher in CHC than in AIH and PBC (p = 0.004 and p = 0.01, respectively). In autoimmune liver diseases, TGF-beta1 was mainly expressed by the macrophages located in the portal tracts and, in CHC, within the hepatic lobules. CONCLUSION: The enhanced TGF-beta1 expression in the nonparenchymal liver cells in AIH and PBC with liver cirrhosis confirms the role of this cytokine in development of fibrosis in autoimmune liver diseases. That in hepatitis of various etiologies versus PBC suggests that the TGF-beta1 signaling pathway may play an important role in an inappropriate immune response in hepatitides; and the variations found in the location of the macrophages expressing TGF-beta1 reflect a difference in the mechanisms of lesions.