During the adaptation phase enteral nutrition should be introduced, preferably orally, in order to stimulate oral motor activity and to avoid feeding aversion behavior. reduction of the gut mass or function, below the minimum needed to digest and absorb sufficient nutrients and fluids for adequate growth and development in children [1,2]. Intestinal failure (IF) can be caused by several disorders of the gastrointestinal tract, which may be categorized into three main groups; (1) anatomical disorders (e.g., short bowel syndrome) (2) neuromuscular diseases (e.g., chronic intestinal pseudo-obstruction) and (3) mucosal intestinal diseases (e.g., microvillous inclusion disease) . Short bowel syndrome (SBS) is the main cause of IF, accounting for at least 40% of the cases [3,4,5]. According to the Dutch National Working group on SBS, short bowel syndrome is defined as 70% resection of the small bowel and/or the residual small bowel length distal to ligament of Treitz of 50 cm in premature neonates, 75 cm of term neonates, and 100 cm in children 1 year . SBS may result from an extensive resection due to congenital defects such as intestinal atresia and gastroschisis, or postnatal ischemia of the bowel from necrotizing enterocolitis Pravastatin sodium or volvulus [5,7,8]. The reduction of the absorptive and digestive surface and the resulting decreased availability of digestive enzymes and transport proteins causes malabsorption in SBS patients . Furthermore, some underlying disorders such as gastroschisis and intestinal atresia not only affect the remaining bowel length, but may Pravastatin sodium also have impact on the residual bowels (motor) function and adaptation potential . In clinical practice SBS can be discerned into three phases. The first phase occurs directly after resection and is characterized by diarrhea and massive loss of fluids and electrolytes, causing increased risk of dehydration, and impaired gut motility also occurs . Shortly after resection the remaining part Pravastatin sodium of the bowel attempts to increase its fluid and nutrient absorption . This is the second phase where the process of bowel adaptation begins and can last up to one to four years [1,13]. It includes muscular hypertrophy (increased bowel diameter and wall thickness) and mucosal hyperplasia . The adaptation process is more pronounced in the ileum than in the jejunum. Bowel adaptation processes should lead to full intestinal autonomy. The third phase is the so-called resting state in which a status quo has arisen regarding the tolerance of enteral nutrition and hence the need for parenteral nutrition. It is characterized by permanent degree of malabsorption, there might be an electrolyte imbalance, bone demineralization, and kidney or gallstones might occur. Vitamin and mineral deficiencies are frequently seen in this phase. The clinical manifestation of SBS is determined by the residual functional length of the jejunum and ileum, the presence of an enterostomy, the presence (or absence) of the ileocecal valve, the remaining functional length of the colon, underlying pathology, and possible complications . These factors affect bowel adaptation and therefore the functionality of the gastrointestinal tract, which in turn affects feeding options. Therefore, recommendations for the type and duration of parenteral, enteral, and oral nutrition are variable, with the childs age as an additional key factor. The goals of nutritional support in patients with SBS are twofoldproviding safe, adequate supplemental nutrients to preserve lean body mass and function, and if possible, supporting and accelerating the bodys own adaptive mechanisms . Maintaining simultaneously optimal nutritional status and achieving intestinal adaptation is a clinical challenge in patients with SBS. Both growth and development of the child as well as bowel adaptation should be Rabbit Polyclonal to ZFHX3 considered synergistically as primary outcome parameters . The aim of this review is to provide an overview of nutritional feeding strategies in this heterogeneous population. 2. Nutritional Management According to Phases SBS In order to meet the nutritional needs of the child, it is initially necessary to provide parenteral nutrition (PN). Clinical experience shows that PN is sometimes given in addition to enteral nutrition for a long period of time in order to be able to meet the energy and nutrient requirements. Sometimes PN is provided for several years and some children remain on PN for life. During the acute phase PN is indispensable and often fluid and electrolyte losses need to be additionally compensated. During this phase minimal enteral feeding should be started as soon as possible to promote bowel adaptation . The aim of PN therapy is.