Modern pharmacotherapy of patients with short intestinal syndrome. Regional experience in management of patients

The article presents an overview of current trends in the approach to the treatment of patients with short bowel syndrome, including examples of children's patients registered in the palliative care service for children of the Tyumen region. Short bowel syndrome is an extremely rare nosological unit in the practice of a clinician and, as a rule, patients with such a diagnosis need to organize a multidisciplinary approach to treatment, since it requires the involvement of specialists of various profiles, such as: pediatrician, surgeon, gastroenterologist, resuscitator, clinical nutrition specialist / nutritionist, psychologist, procedural nurse, as well as parents/legal representatives. The most common manifestations of the disease are symptoms of malabsorption: dumping syndrome, progressive loss of body weight, deficiency of macro- and micronutrients, stunting of growth and development of all organs and systems, other manifestations of dyspepsia and abdominal pain. Attention is drawn to the fact that the number and intensity of symptoms varies significantly among patients, which may depend on a number of factors (the level and volume of resection, residual length, functional safety of the residual segment, the presence of a stoma / stoma, or a restored intestinal tube, concomitant diseases and possible malformations). The traditional tactics of treating such patients include the need for lifelong parenteral nutrition or, as a forced measure, intestinal transplantation. Rehabilitation measures in children with short bowel syndrome should take into account the requirements of correction of impaired absorption and restoration of normal trophic status; relief of diarrhea, prevention and treatment of dysbiosis; control and correction of general digestive disorders, as well as comorbid conditions. The main alertness in the tactics of managing patients with short bowel syndrome is caused by the need for long-term constant parenteral nutrition, which entails a number of life-threatening risks, such as: catheter-associated conditions (sepsis and thrombosis, which, in addition to aggressive treatment, requires periodic replacement of the central venous access and threatens its complete loss), cholestasis with the outcome of cirrhosis, atrophy of the residual intestinal mucosa, impaired metabolism of D-lactate (with the risk of D-lactate acidosis and oxalates (with the formation of oxalate kidney stones), and also, especially in the absence of resection of the bauginia flap in the outcome, the appearance of excessive bacterial growth syndrome, accompanied by the danger of translocation of intestinal flora into its own plastic and subsequent intestinal-associated sepsis). After analyzing publications and evaluating the experience of working with children with short bowel syndrome in the Tyumen region, we found that the analogue of glucagon-like peptide 2 – teduglutide – makes it possible to reduce the volume of parenteral nutrition, infusion time, and also achieve a complete transition to enteral autonomy. The method of treatment with teduglutide is the most promising for patients of this category, as it reduces the risks associated with parenteral nutrition and improves the prognosis of later life.

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