Ultra-short bowel syndrome during infancy: improving outcomes and novel therapies

Abstract

Purpose of review Ultra-short bowel syndrome is relatively rare and has not yet been extensively reported. In ultra-short bowel syndrome, poor absorption of nutrients and dysmotility, interfere with fluid, energy, electrolyte and micronutrient balance. Patients with this disorder are managed through prolonged parenteral nutrition with the ultimate goal of achieving enteral autonomy. Overall outcomes of these patients are dependent on postsurgical bowel anatomy (residual length, intact colon, ostomy closure timing), incidence of sepsis, and care by a multidisciplinary specialized team. Recent findings Over the years, standardization of management has improved outcomes. This includes central line care, lipid alternatives, enteral therapy, medications (antidiarrheal agents, acid suppression medications, bile acid binding salts, and enteral antibiotics) including Teduglutide. Bowel lengthening procedures have also proven beneficial, and finally bowel transplant does remain an option for a patient in whom rehabilitation has failed. Summary Although there are many factors that influence outcomes of ultra-short bowel patients, novel therapies such as Teduglutide have been introduced with the aim of improving intestinal adaptation. Surgical lengthening and transplant are viable options in the setting of failed rehabilitation.

Successful experience in the use of teduglutide in children with short bowel syndrome

“…Большинство побочных явлений, связанных с терапией ТГ, обычно лёгкой или средней тяжести, проявлялись преимущественно абдоминальными болями, которые были вполне ожидаемы при СКК у больных и купировались через 2-3 нед без последующего повторения. Реабилитационные мероприятия у детей с СКК должны включать коррекцию нарушенного всасывания и восстановление нормального трофического статуса; купирование диареи, профилактику и лечение нарушений микробиоты кишечника; уменьшение общих дигестивных изменений [35]. Клинические примеры показали эффективность и безопасность ТГ в лечении детей с СКК.…”

Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes

“…(3) Treatments for chronic DIF and NDIF able to reduce PN dependency over time (e.g., analog of glucagon like peptide 2 and surgical procedures to increase mucosal surface area in SBS or prokinetic drugs in PIPO [15,21,22,26,27,29,49,62,[81][82][83][84][85][86]90,[122][123][124] The advances in EN tolerance over time and the progressive decreasing in calories and fluids provided by PN will be the clinical markers of successful application of specific and non-specific therapies.…”