[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.

[DETERMINING THE DEGREE OF DISRUPTION OF THE STRUCTURE OF THE LIVER AND THE SEVERITY OF PORTAL HYPERTENSION IN CHILDREN].

AIM: To develop a system to define the degree of liver disruption and severity of portal hypertension in children based on the International Classification of Functioning, Disability and Health (ICF). PATIENTS AND METHODS: Studied the results of laboratory and instrumental methods 382 children: 267 patients with various liver diseases, including 49 patients who underwent liver transplantation, and 115 children without liver disease. RESULTS: Based on analysis of statistical data obtained were identified 10 indicators, a set of changes which can be used to assess the degree of disruption of the structure of the liver and the severity of portal hypertension: indicators that reflect the severity of fibrosis and cirrhosis of the liver (METAVIR score on a scale at fibroelastometrii, scores are Desmet at morphological study of the liver) and indicators that reflect the severity of portal hypertension (the diameter of the portal vein, splenic vein diameter, the length of the spleen, recanalization of the umbilical vein, esophageal varices, ascites, hydropericardium, hydrothorax). Each of the indicators was assessed on a 5-point system. Number of points reflects the sum of the changes of these parameters. Decrease the number of points on 0-4% (38-40 points) is regarded as a lack of structural failure of the liver and the severity of portal hypertension by 5-24% (30-37 points)--minor violations on 25-49% (20-29 points) -moderation disorders, 50-95% (3-12 points)--severe handicaps, 96-100% (0-2 points)--absolute violation. Studied the dynamics of children with autoimmune hepatitis, Wilson's disease and chronic hepatitis C. CONCLUSION: The proposed scoring system for assessing the degree of disruption of the structure of the liver and the severity of portal hypertension can be used as an objective criterion of the severity of the pathological process, to estimate the dynamics of defeat against the background of the therapy, determining the prognosis of the disease and as a criterion of the indications for liver transplantation.

[The Relationship of the Degree of Impairment of the Structure and Function of the Liver with its Chronic Illnesses in Children].

OBJECTIVE: Our aim was to on the basis of determining the degree of violation of the structure and function of the liver establish their relationships and to assess the dynamics of liver disease in its chronic illnesses in children. METHODS: With the help of the developed scoring systems were used to assess the degree of liver dysfunction and the degree of disruption of the structure of the liver and the severity of portal hypertension. RESULTS: The results of the diagnostic methods 252 children aged 1 to 17 years (mean age of 11.8±3,5) with Wilson disease (WD), autoimmune hepatitis (AIH), chronic hepatitis C (CHC) were analyzed; 48 patients underwent liver transplantation. In children with WD, AIHand CHC liverfunction reduced by 41.3±12.9% to 28.8±12.5% and 19.1±7.8% respectively. Structure of the liver in children with WD, AIH and CHC was disturbed by 25.0±8.1% to 20.4±9.2% and 6.8±4.4% respectively. Thefunction and structure violations of the liver more pronounced in liver cirrhosis. The use of the developed scoring systems to monitor the severity of liver damage in the dynamics and evaluation of the effectiveness of the therapy is demonstrated. The degree of liver dysfunction is directly dependent on the degree of its structure. Abnormal liverfunction ≥40% and ≥40% of its structure with treatment failure can be used as a criterion of indicationsfor elective liver transplantation with its chronic diseases in children. CONCLUSION: Developed a point system to determine liver function and a point system to determine disruption of the structure of the liver and the severity of portal hypertension in children can serve as an objective criterion for assessing the severity of liver disease, monitoring their changes in the dynamics with the assessment of the effectiveness of the therapy and making decisions about the need for routine liver transplantation in its chronic illnesses in children.

[The experience of applying system of continuous monitoring of content of glucose in children with glycogen disease].

The sampling included 23 children with glycogen disease. All patients were examined using system of continuous monitoring of content of glucose applied during 72 hours. It was established that hypoglycemia was detected in 19 (82.6%) children. At that, in 7 (30.4%) children the level of glucose was below detected range (< 2.2 mmol/l). In patients ignoring proposed recommendations (lack of compliance) expression of hypoglycemia was reliably higher than in children being on a diet and following recommendations of physician. In primary patients as compared with secondary patients rate and duration of hypoglycemia in blood serum activity of aspartate aminotransferase also was reliably higher. Independently of all that, the more frequently hypoglycemia developed the more expressed hypoglycemia was. Therefore, continuous monitoring of content of glucose in intercellular fluid is an effective instrument for detecting degree of compensation of carbohydrate metabolism in patients with glycogen disease. The day continuous monitoring of level of glucose permits to provide the most complete picture of fluctuations of glycaemia during a day. The obtained data can be used as a basis for composing an optimal algorithm of diet therapy.

[DETERMINING THE DEGREE OF LIVER DYSFUNCTION IN CHILDREN WITH POSITIONS OF THE INTERNATIONAL CLASSIFICATION OF FUNCTIONING, DISABILITY AND HEALTH (ICF)].

METHODS: Based on a retrospective analysis of biochemical blood parameters which characterize the role of liver function in the metabolism of proteins, fats and carbohydrates (considered indicators of ALT, AST, De Ritis coefficient, bilirubin, albumin, fibrinogen, prothrombin, transferrin, ceruloplasmin, cholesterol, urea, ammonia, glucose, lactate) in 95 children without liver pathology, 15 children who died of liver failure, 295 patients with various liver diseases who were treated in the SCCH, a scale system was developed as a support tool to assess liver dysfunction. RESULTS: Each biochemical indicator was assessed on a five-point scale. The level of a biochemical indicator, which corresponded to the absence of disorders, was estimated as 4 points, corresponding to "insignificant disorders"--as 3 points, "moderate disorders"--as 2 points, "severe disorders"--as 1 point, "absolute disorders"--as 0 points. The total score is the estimate of the degree of liver dysfunction. According to the recommendations of the International Classification of Functioning, Limitations of vital activities and Health, the decrease of the number of points on 0-4% (54-56 points) corresponds to the absence of the liver dysfunction, on 5-24% (43-53 points)--insignificant disorders of liver function, on 25-49% (29-42 points)--moderate hepatic impairment, on 50-95% (3-28 points)--severe disturbances of liver function, on 96-100% (0-2 points)--absolute dysfunction of the liver. CONCLUSIONS: A scoring system of assessing liver dysfunction can be applied at any stage of the examination and treatment of children of any age, as used in biochemical parameters do not depend on the age of the patient. It is an objective criterion for assessing the degree of liver dysfunction and can be used to assess the severity of the pathological process in the dynamics determining the prognosis of the disease and can be the criterion of the indications for liver transplantation, and also used during the of medico-social expert examination.

[The Study of Psychological Characteristics of Children and Adolescents with Digestive Diseases].

OBJECTIVE: The present study was aimed at the psychological characteristics of children with different chronic diseases of the digestive system and the identification of key factors influencing the formation of their personality. METHODS: The continuous prospective study of psychological peculiarities of 125 patients (7-17 y.o.) with diseases of the digestive system who have been monitored at Scientific Centre of Children's Health (Moscow) was performed. As research methods were used: analysis of medical and pedagogical documentation, participant observation, educational experiment, interview, questionnaires and projective methods. RESULTS: The study involved 125 patients aged 7-17 years (12.4 averagely) with diseases of the digestive system including teens--68 (54%), primary school pupil--57 (46%). The number of boys significantly prevailed over the number of girls (2:1). The study confirmed the existence of a close relationship and mutual influence of three factors: the physical condition, social situation of development, and individual psychological characteristics of children. According to the psychological characteristics the children can be combined in three groups: Group I (47 of 125 people; 37.6%) - children with bad psychological status. This is most typicalfor children with active form of chronic diseases requiring intensive medical assistance (37 of 47people; 78.7%). Group II (59 of 125 people; 47.2%)--children with instable psychological condition, with risk of neurotization. This group mainly comprises patients with chronic diseases at the stage of unstable clinical remission with preserved or compensated functions of organism bodies and systems or with incomplete compensation of functions requiring long term supportive treatment (45 of 59 people; 76.3%). Group III--patients with rather stable psychological condition (19 of 125 people; 15.2%). It comprises patients with rather stable psychological condition, anyway, with expressed psychological vulnerability in stress situation. Most children suffer from chronic diseases of different etiology at the stage of clinical remission with rare aggravations, with preserved or compensated functions, provided there are no aggravations of the basic disease (17of 19 people; 89.5%). CONCLUSION: Most children with diseases of the digestive system have the psychological difficulties, while their identity is often formed in the distorted social conditions. Therefore, these children need psychological and pedagogical support, as well the special psychological and pedagogical conditions for the realization of their mental and cognitive capacities. To put together a program of psychological and educational assistance we should take into account the child's age at the moment of occurrence of the disease, etiology, duration and severity of the illness, the nature and degree of non-compliance of social environmental.

[Mitochondrial dysfunction in children with hepatic forms of glycogen storage disease].

AIM: The purpose of the study was to assess mitochondrial dysfunction severity in patients with hepatic forms of glycogen storage disease (GSD). PATIENTS AND METHODS: We examined 53 children with GSD in the dynamics. Distribution of children by disease types was: 1st group--children with GSD type I, 2nd group--children with GSD type III, 3rd group--children with GSD type VI and IX; comparison group consisted of 34 healthy children. Intracellular dehydrogenases activity: succinate dehydrogenase (SDH), glycerol-3-phosphate-dehydrogenase (GPDH). nicotinamideadenin-H-dehydrogenase (NADH-D) and lactatdehydrogenase (LDH) was measured using the quantitative cytochemical method in the peripheral lymphocytes. RESULTS: It was revealed decrease of SDH- (p < 0.001) and GPDH-activities (p < 0.001), along with increase of the NADH-D activity (p < 0.05) in all patients with GSD, (SDH/ NADH-D) index was decreased (p < 0.001). LDH activity was increased in groups 1 (p < 0.05) and 3 (p < 0.01), compared with comparison group. The most pronounced intracellular enzymes activity deviations were observed in children with GSD type I, that correspond to more severe clinical form of GSD. It was found strong correlation between intracellular enzymes activity and both hepatomegaly level (R = 0.867) and metabolic acidosis severity (R = 0.987). CONCLUSION: Our investigation revealed features of mitochondrial dysfunction in children with GSD, depending on the GSD type. Activities of lymphocytes enzymes correlates with the main disease severity parameters and can be used as an additional diagnostic criteria in children with hepatic form of GSD.

[Chronic hepatitis C in children: problems and prospects].

The analysis of a problem state of chronic hepatitis C in children was conducted. Data on primary incidence of chronic hepatitis C at the children's population as on the territory of the Russian Federation, and abroad are submitted. Problems of diagnosis of a HCV infection are studied. The survey analysis of risk of contamination is carried out, possible ways of transfer are highlighted, features of a course of a HCV infection in children are shown, synchronising frequency of a process is displayed. The problem is analysed now, and further prospects in treatment of chronic viral hepatitis C in children are estimated.

[History case of multiple hepatic adenomas in adolescent with severe course of glycogen storage disease type lb].

We represented a case history of multiple hepatic adenomas in an adolescent with severe clinical course of glycogen storage disease type lb (compound heterozygous mutations c.1042_1043delCT and c.817G>A in the SLC37A4). The patient was prescribed a raw cornstarch and hepatoprotectors therapy, but he and his parents had low compliance to treatment. At the age of 13,5 years ultrasound investigation and computed tomography revealed multiple adenomas. Due to the severe condition of the patient it was impossible to perform focal hepatic biopsy. At present time the patient receives treatment focused on correction of metabolic disturbances, thereafter an applicability of exploratory puncture will be settled for the further patient surveillance. The modern data on causes and risk factors of hepatic adenomas in such patients, the possibility of their malignization, the algorithm of the follow-up and the methods of treatment are presented in the discussion.

[Low-energy laser therapy of chronic gastritis in children]. / Nizkoénergeticheskaia lazerterapiia khronicheskogo gastrita u detei.

Morphological and morphometric study of gastric mucosa was performed before and after laser therapy in children with chronic gastritis. Features of childhood gastritis are provided and laser therapeutic action in different after treatment periods is characterized.

[Morphological changes in the stomach and duodenum in chronic liver diseases of HB viral etiology]. / Morfologicheskie izmeneniia zheludka i dvenadtsatiperstnoi kishki pri khronicheskikh zabolevaniiakh pecheni HB-virusnoi étiologii.

Morphologic investigation of 72 gastroduodenal biopsy samples from 29 children with chronic persistent and chronic active hepatitis, hepatic cirrhosis, revealed signs of chronic gastroduodenitis. The degree of the gastroduodenal zone lesion was directly related to severity of the primary condition. Comparative data on histopathological changes in the stomach and duodenum in concurrent pathologies suggest that chronic duodenitis may be the primary condition.