Purpose — to discover peculiarities of Wilson disease course in children dependently on the variant of liver affection. Materials and methods. Anamnesis of the disease and clinical and paraclinical peculiarities of the course of the disease with consideration of liver affection severity have been studied in 50 children aged 5–17 years. Results. It was estimated that in 52% of children the disease had a form of chronic hepatitis with minimal clinical symptoms, 44% of patients had liver cirrhosis with predominant signs of edematous and ascitic syndrome and 4% had fulminant hepatitis in a debut of the disease. Complicated family anamnesis was detected in 4% of patients. Syndrome of cytolysis predominated in patients with liver damage in the form of chronic hepatitis (р<0.05). Typical biochemical changes in cirrhotic patients were hepatocellular insufficiency with hypoalbuminemia and coagulopathy, hyperbilirubinemia and minimal hypertransaminasemia (р<0.05). Children with cirrhosis in contrast to patients with chronic hepatitis had reliably lower serum ceruloplasmin concentration and reliably higher excretion of copper with urine. Specific for Wilson disease Kayser—Fleischer rings were found out in 36% of patients only. Conclusions. Wilson's disease in children is characterized by progressing course in the form of either chronic hepatitis or liver cirrhosis or, rarely, fulminant hepatitis. Clinical and paraclinical symptoms of the disease are estimated by the severity of liver affection and vary from a course with minimal symptoms in patients with chronic hepatitis to edematous and ascitic syndrome and hepatocellular insufficiency in patients with cirrhosis and fulminant hepatitis. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Wilson's disease, children, chronic hepatitis, liver cirrhosis, course of the disease.
Purpose — analyse the state of the epithelial barrier of the colon in children with different clinical forms. Materials and methods. 42 children with acute chronic colitis were examined, including 28 with ulcerative colitis (14 with active total form, 14 with moderately active segmental); and 14 with chronic non-specific opaque colitis formed a comparison group. Laboratory methods were performed on all patients — hemogram, protein-gram, blood biochemistry, fecal calprotectin concentration; endoscopic examination with biopsy of all colon regions and histological examination of biopts. Results. The clinical manifestations of ulcerative colitis (UC) during the acute period were assessed by the Paediatric Activity Index (PUCAI) and depended on the localization and activity of the inflammatory process. The average for active colitis was found to be 50.2±1.8, moderate to 35.3±1.7, minimum to 24.1±1.2, but for children with total active inflammation 19 per cent of patients had the highest rates: 65, which corresponded to clinical signs of ulcerative colitis, accompanied by unidirectional changes of surface (dystrophic changes of epithelium, crypt deformation, reduced number of flax cells) and deep (diffuse inflammatory infiltration of its own plate, presence of crypt abscesses, cryptites, vascular dilation) structures of the mucous membrane of the large intestine, which are more pronounced in the active total forms of ulcerative colitis. The period of UC exacerbation is characterized by the violation of the epithelial barrier mucous membrane colon due to reduced mucus synthesis and changes in its biochemical properties, low secretory (MUC2) and membrane-associated (MUC4) expression Mucins, mainly in the active total forms of UC, loss of the regulatory effect of the club peptide on regeneration and protection of the mucous membrane of the intestine. Conclusions. Studies based on a pathogenetic approach to determining the cause of the exacerbation of the disease have shown evidence of a significant role in the epithelial barrier of the colon membrane, This is a significant addition to the known knowledge of ulcerative colitis pathogenesis in childhood. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. The authors declare no conflicts of interests. Key words: ulcerative colitis, children, epithelial barrier, mucins, clubs.
Objectives: to determine morphological and immunohistochemical changes in the placentas of displaced pregnant women in order to further improve the antenatal monitoring program.Materials and methods. 96 displaced pregnant women (the main group) took part in the prospective study, who depending on the therapy were divided into: group I – 65 pregnant women who were monitored according to generally accepted recommendations and received routine therapy for prolonging pregnancy, group II – 31 pregnant women who received the proposed treatment and prophylactic complex (micronized progesterone, magnesium citrate in combination with pyridoxine hydrochloride, iron fumarate, a multivitamin complex with 200 mg of docosahexaenoic acid, complex of medical and psychological support for pregnant women). The control group consisted of 39 pregnant women living at territories controlled by Ukraine. The duration of observation was 2 years. Placentas were examined by organometric, macroscopic, general histological and immunohistochemical methods.Results. Organometrically, placentas in the group I had a significant decrease in thickness, mass and size. Edema of Wharton jelly was significantly more pronounced in this group, and in the lumen of vessels there was a more pronounced parietal formation of fibrin blood clots. Manifestations of ischemic infarcts and blood clots, which were located mainly in the paracentral and peripheral zones, were found significantly more frequently among women of the group I. Histologically, tissue changes were noted in the placentas in the group I, which are manifestations of placental damage and characterized by a partial decrease in maternal-fetal-placental perfusion. These changes were combined with focal edema in decidual tissue and dystrophy in decidual cells. In the placentas of women of the group I an uneven focal, sometimes pronounced proliferation of syncytial nodules of a more widespread nature was noted, which is a marker of cellular aging associated with adverse factors affecting the mother’s body. Also, foci of maternal infarcts were noted in the group I, which is associated with a decrease in maternal perfusion in the vessels of the placental barrier.Immunohistochemically, vacuolization and uneven chromatin arrangement were observed in the nuclei of villi cells during Ki-67 immersion, which indicated a decrease in regenerative responses in the placental barrier in groups I and II, especially with elevated cortisol levels. Immunohistochemical studies of the pro-apoptotic marker P-53 showed expression in single nuclei of villi stroma cells.Conclusions. The detected changes in the structures of the placental barrier indicate the predominance of placental disorders in the group I compared to the group II and control groups, especially in the syncytium of the villi, which led to a violation of the placenta functioning. Morphological studies confirmed the significant impact of long-term chronic stress on the formation and functioning of the uterine-placental-fetal complex, as well as the effectiveness of the proposed treatment in preventing the identified disorders.
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