BBSome-deficient cells activate intraciliary CDC42 to trigger actin-dependent ciliary ectocytosis.

Bardet-Biedl syndrome (BBS) is a pleiotropic ciliopathy caused by dysfunction of the BBSome, a cargo adaptor essential for export of transmembrane receptors from cilia. Although actin-dependent ectocytosis has been proposed to compensate defective cargo retrieval, its molecular basis remains unclear, especially in relation to BBS pathology. In this study, we investigated how actin polymerization and ectocytosis are regulated within the cilium.

Fine-Tuning of the Optical and Electrochemical Properties of Ruthenium(II) Complexes with 2-Arylbenzimidazoles and 4,4'-Dimethoxycarbonyl-2,2'-bipyridine.

A series of cyclometalated complexes of ruthenium (II) with four different substituents in the aryl fragment of benzimidazole was synthesized in order to study the effect of substituent donation on the electronic structure of the substances. The resulting complexes were studied using X-ray diffraction, NMR spectroscopy, MALDI mass spectrometry, electron absorption spectroscopy, luminescence spectroscopy, and cyclic voltammetry as well as DFT/TDDFT was also used to interpret the results. All the complexes have intense absorption in the range of up to 700 nm, the triplet nature of the excited state was confirmed by measurement of luminescence decay.

[The effect of the FODMAP and rebamipid diet on the activity of disaccharidases in patients with enteropathy with impaired membrane digestion].

Aim: To compare the effect of a diet low in fermentable oligo-, di-, monosaccharides and polyols (fermentable oligosaccharides, disaccharides, monosaccharides and polyols - FODMAP) and rebamipide on carbohydrate tolerance and disaccharidases activity in patients with maldigestive enteropathy (ENMP).

Materials And Methods: The study included 61 patients with ENMP with reduced small intestine carbohydrases. Their glucoamylase activity was 100 ng glucose/mg tissue × min (quartile 53, 72), maltase - 504 (quartile 258, 708), sucrase - 43 (quartile 25, 58), lactase - 8 (quartile 4, 20).

[Zonulin and I-FABP are markers of enterocyte damage in celiac disease].

Aim: To evaluate the level of serum I-FABP (Fatty-Acid-Binding Protein a protein that binds fatty acids) and fecal zonulin as markers of the permeability of the mucous membrane of the small intestine in celiac patients.

Materials And Methods: A total of 151 celiac patients (25 men and 126 women) were examined. The median age was 42 years.

A Unique Observation of a Patient with Vulto-van Silfhout-de Vries Syndrome.

Introduction: Vulto-van Silfhout-de Vries Syndrome (VSVS; OMIM#615828) is a rare hereditary disease associated with impaired intellectual development and speech, delayed psychomotor development, and behavioral anomalies, including autistic behavioral traits and poor eye contact. To date, 27 patients with VSVS have been reported in the literature. Materials and Methods: We describe a 23-year-old male patient with autism spectrum disorder (ASD) who was admitted to the gastroenterological hospital with signs of pseudomembranous colitis.

[Celiac crisis in patients with celiac disease. Case report].

Celiac crisis (CC) is a rare life-threatening course of celiac disease, observed mainly in children. In adults, CK can be the first manifestation of the disease and, very rarely, a relapse that occurs in patients who do not follow the gluten-free diet (AGD). Triggers can be stress, surgery, childbirth, etc.

[Chronic intestinal pseudoobstruction: difficulties in diagnosis and treatment. Case report].

The article presents a clinical case of a 23-year-old patient with an extremely severe congenital form of chronic intestinal pseudoobstruction coupled with a neuromyopathy,colon malrotation, malabsorption, bacterial overgrowth syndrome, cholelithiasis and gastrostasis, which excluded bowel transplantation. Long-term treatment in the intensive care unit with combined, mainly parenteral nutrition for 6 months, using antibiotics, prokinetics, intestinal decompression allowed to achieve partial stabilization of the patients condition and transfer to home treatment with the continuation of adequate complex therapy.

Patients with functional bowel disorder have disaccharidase deficiency: A single-center study from Russia.

Background: Functional bowel disorder (FBD) may be caused by a decrease in disaccharidase activity. Thus, the timely diagnosis of disaccharidase deficiency could lead to a better prognosis in patients with this condition.

Aim: To determine the potential value of intestinal disaccharidases glucoamylase, maltase, sucrase, and lactase in understanding the etiology and pathogenesis of FBD.

[Chronic intestinal pseudo-obstruction].

Chronic intestinal pseudo-obstruction a rare violation of the motor skills of the gastrointestinal complex, similar to mechanical obstruction, but without a mechanical obstacle. The development of chronic intestinal pseudo-obstruction is caused by a disturbance on the part of the smooth muscles and the nervous system of the gastrointestinal system. Common symptoms include constipation, abdominal pain, nausea, vomiting, bloating.

[COVID-19 and the small intestine].

The SARS-CoV-2 virus enters the body through the angiotensin-converting enzyme 2 (ACE-2), which is the entry point of the virus into the cell. The most dense fabric of ACE-2 is the lungs. The small intestine also contains large amounts of ACE-2 in the enterocyte membrane and is often involved in this process.

[The role of therapeutic exercises in blood pressure correction, vascular wall stiffness and disorders of bone and mineral metabolism in postmenopausal hypertensive patients].

Objective: To study the role of therapeutic exercises (TE) in the correction of blood pressure, stiffness of the vascular wall and disorders of bone mineral metabolism in postmenopausal hypertensive patients.

Material And Methods: The study included 78 patients (mean age was 53.32±7.

[Screening for celiac disease among patients of the gastroenterological profile].

Aim: To determine the frequency of celiac disease (CD) among gastroenterological patients and criteria for its active detection.

Materials And Methods: 1.358 patients referred for gastroenterologist consultation from 2016 to 2019 was conducted, of which 140 had CD (339 males 24.

[Enteropathy with impaired membrane digestion and the prospects for cytoprotective therapy].

The article describes enteropathy with impaired membrane digestion (EIMD) as a new nosological form. The main clinical manifestation of EIMD is the poor tolerance of food products, in particular carbohydrates and a decrease in the activity of membrane enzymes, in particular, carbohydrates, in the mucous membrane of the small intestine. The cause of the disease can be acute intestinal infections, viruses, drugs and other agents that damage the small intestine.

[Nonspecific parameters of the immune inflammatory response as a link in the pathogenesis of remodeling of the vascular wall and destruction of bone tissue in women with arterial hypertension in postmenopausal women.].

Recently, they increasingly began to pay attention to the role of a nonspecific immune-inflammatory vascular response as a link in general pathogenetic mechanisms with a change in the elastic properties of arteries and phenomena of destructive bone changes, which at the subclinical level is of great importance for the prevention of the development of socially significant diseases. A total of 104 patients were examined (mean age 57.45 years), which were divided into three groups.

Celiac disease associated with ulcerative colitis.

The article provides clinical observation of a patient who was diagnosed with celiac disease when he was 52 years (Marsh stage IIIB). Following gluten-free diet (GFD) clinical remission and restoration of small intestinal mucosa (SIM) structure occurred, however in 6 years ulcerative colitis developed and an impairment of SIM morphological structure was identified (Marsh stage IIIA). Ulcerative colitis and celiac disease remission is supported by GFD, anti-cytokine therapy (adalimumab) in combination with mesalazine.

Rebamipide increases the disaccharidases activity in patients with enteropathy with impaired membrane digestion. Pilot study.

Aim: To evaluate the effectiveness of enteroprotector Rebamipide in the treatment of enteropathy with impaired membrane digestion (EIMD).

Materials And Methods: We examined 102 patients aged 18 to 50 years (41 men and 61 women) with clinical signs of irritable bowel syndrome (n=65), functional diarrhea (n=33), and functional constipation (n=4) according to Rome IV criteria (2016). The activities of glucoamylase (GA), maltase, sucrase and lactase were determined by Dahlquist-Trinder method in duodenal biopsies obtained during esophagogastroduodenoscopy.

Reproductive disorders, osteoporosis and secondary hyperparathyroidism with celiac disease.

A clinical observation of a patient with celiac disease, aged 23, with severe form of osteoporosis, accompanied by atraumatic fractures bones, lameness, late onset of menstruation and pathology of pregnancy is described. It is emphasized that only a timely diagnosis celiac disease and timely prescribed treatment make it possible to avoid severe complications.

The role of small intestine in pathogenesis of common variable immune deficiency.

AbstractThe article presents the results of examination of 32 patients with common variable immune deficiency (barn) with involvement in the patho- logical process of the digestive system. The features of the clinical picture, the content of immunoglobulins in the blood serum, morphological structure of the mucosa and small intestine as well as treatment. Special attention is paid to the small intestine in the pathogenesis of the barn.

[Disaccharidase deficiency and functional bowel diseases].

Aim: To elucidate the role of intestinal carbohydrases (glucoamylase, maltase, sucrose, and lactase) in the etiology and pathogenesis of functional bowel diseases (FBD).

Subjects And Methods: 74 patients (36 men and 38 women) aged 18 to 50 years with FBD were examined. According to Rome IV criteria (2016), there was diarrhea-predominant irritable bowel syndrome (IBS) in 21 patients, functional diarrhea (FD) in 33, constipation-predominant IBS in 6, functional constipation (FC) in 4, and mixed IBS in 10.

[All-Russian Consensus on Diagnosis and Treatment of Celiac Disease in Children and Adults].

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.

[Seronegative celiac disease: A case report].

The paper describes a rare case of celiac disease in the absence of serum anti-tissue transglutaminase (anti-tTG) antibodies. A 51-year-old patient has been suffering from diarrheas for 20 years. He has lost 15 kg gradually; weakness progressed; muscle cramps, leg edemas, and signs of dynamic pseudoobstruction appeared.

[Celiac disease detection rate in gastroenterological patients].

Aim: To determine celiac disease detection rate in patients with digestive disease.

Subjects And Methods: A total of 318 gastroenterological patients admitted to be treated at the Central Research Institute of Gastroenterology in September to October 2012 were examined. The patients' age was 18 to 74 years (mean 51.

[Safety of mesenchymal stromal cell therapy for inflammatory bowel diseases: results of a 5-year follow-up].

Aim: To compare the safety of therapy in patients with ulcerative colitis (UC) and Crohn's disease (CD) who have received combination anti-inflammatory therapy using bone marrow mesenchymal stromal cells (MSC) and standard therapy with 5-aminosalicylic acid, glucocorticosteroids, and immunosuppressive agents.

Subjects And Methods: Unfavorable consequences were analyzed in 103 patients (56 with UC and 47 with CD) with inflammatory bowel disease (IBD) after MSC administration. The findings were compared with data obtained in 208 patients with UC and CD on standard anti-inflammatory therapy.

[Significance of a method for determination of deamidated gliadin peptide in the diagnosis of celiac disease].

Aim: To define the value of a new enzyme immunoassay in determining the level of anti-deamidated gliadin peptide (DGP) antibodies (Abs) in the diagnosis of celiac disease.

Subjects And Methods: One hundred and twenty-four patients treated at the Department of Intestinal Pathology, Central Research Institute of Gastroenterology, were examined. Enzyme-linked immunosorbent assay (ELISA) was employed to determine Abs to tissue transglutaminase (tTG) and DGP of the IgA and IgG classes in the sera of all the patients.

[Reproductive disorders in women with celiac disease: effect of etiotropic therapy].

The study included 132 women (average age 38.5 +/- 1.17 years) with HC observed in the CSRIG from 2000 to 2010.