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Young people with gastrointestinal conditions on home parenteral nutrition and their transition to adult services
Published in Clarissa Martin, Terence Dovey, Angela Southall, Clarissa Martin, Paediatric Gastrointestinal Disorders, 2019
Intestinal failure is diagnosed when intestinal function becomes insufficient to allow adequate absorption of fluid, electrolyte or nutrient requirements (Puntis, 1995). Home PN is instituted when intestinal failure is expected to last for 3 months or more resulting from a variety of different gastrointestinal diseases. Short bowel syndrome is the most common reason for intestinal failure. Short bowel syndrome is acquired as a result of extensive bowel resection, usually in the neonatal period, but it may be due to Crohn’s disease in children. Permanent intestinal failure may result from inherited conditions diagnosed in the neonatal period, which include: severe motility disorders such as chronic intestinal pseudo-obstruction where children have recurrent symptoms suggesting bowel obstructions in the absence of an obstructing lesion or long-segment Hirschsprung’s diseasecongenital enteropathies, such as microvillus inclusion disease and tufting enteropathy.
The Small Intestine
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
Intractable diarrhoea with impaired absorption of nutrients following resection or bypass of the small intestine, ultimately leading to progressive malnutrition, is referred to as ‘short bowel syndrome’. The most common causes of short bowel syndrome are resection resulting from the management of CD and its complications (which accounts for almost half of cases), mesenteric vascular thrombosis, radiation enteritis and tumours. Although features of short bowel syndrome usually appear when there is less than 200 cm of small bowel, the length and nature of the remaining intestine are also important. In general, diseases which result in short bowel syndrome tend to preferentially affect the distal small intestine, and there is some evidence that the ileum, with its tighter intercellular junctions and consequently better fluid absorptive capacity, can assume the functions of a missing jejunum, but not vice versa. While the ileocaecal valve used to be considered important with regard to preservation of absorptive function, it is more likely that this is a reflection of the associated preservation of the distal ileum and right colon than the valve itself.
Diarrhea in Short Bowel Syndrome
Published in John K. DiBaise, Carol Rees Parrish, Jon S. Thompson, Short Bowel Syndrome Practical Approach to Management, 2017
Badr Al-Bawardy, Vandana Nehra
Diarrhea is the predominant symptom in patients with short bowel syndrome (SBS). There are multiple underlying pathophysiologic mechanisms that interact to cause diarrhea in these patients. Diarrhea is an important problem in SBS as it may result in electrolyte disturbances, volume depletion, weight loss, and numerous nutritional deficiencies and represents an important source of frustration negatively affecting these patients’ quality of life. Indeed, when severe, diarrhea may require frequent intravenous (IV) fluid supplementation or parenteral nutrition (PN). Even in the absence of long-term PN, patients with SBS have a significantly lower quality of life in terms of social functioning and mental health in comparison with matched controls [1]. This is mainly due to the diarrhea and the resulting need for hospitalization for dehydration and management of nutritional deficiencies. In addition, SBS patients with large-volume diarrhea often decrease their oral intake to prevent high stool output or ingest increased oral fluids that result in a worsening of the diarrhea, further contributing to the detriment in their quality of life [2]. Therefore, identifying and treating the underlying cause(s) of diarrhea in these patients is essential to implement a successful management strategy. Fortunately, the majority of patients with SBS are able to control the diarrhea, at least to a degree, and maintain social functioning in the form of employment and activities of daily living [3].
Graft assessment for acute rejection after intestinal transplantation: current status and future perspective
Published in Scandinavian Journal of Gastroenterology, 2021
Intestinal transplantation is defined as a process of grafting an intestinal component (jejunoileum), the development of which was essential to address the issue of intestinal failure and its corresponding complications [1]. Intestinal failure is characterized by an inability of the gastrointestinal tract to meet nutritional demands and thus requiring either temporary or permanent parenteral nutrition to maintain health and/or growth [2]. Most often this condition is caused by short bowel syndrome due to surgical resection, although motility diseases, mucosal defects, obstruction and fistula may also progress to intestinal failure [3]. Overall, the long term survival for patients with non-malignant intestinal failure on PN is excellent (5 year survival 64–91% [4,5]). Therefore, intestinal transplantation is only reserved for patients who have failed parenteral nutrition and developed life-threatening complications [6]. Other indications include lifesaving therapy in portomesenteric thrombosis, frozen abdomen and benign or slow-growing tumour involving the hilum of the liver [7–9]. The three main intestinal transplant procedures that are currently being performed are isolated small bowel, multivisceral (graft including the stomach) and combined liver-intestine grafts. Although, many variations of these procedures exist [1,10].
Advances in non-surgical treatment for pediatric patients with short bowel syndrome
Published in Expert Opinion on Orphan Drugs, 2020
Danielle Wendel, Beatrice E. Ho, Tanyaporn Kaenkumchorn, Simon P. Horslen
Intestinal failure has been defined as the inability of the gut provide the necessary digestion and absorption of nutrients and fluids for a patient to survive with the need for intravenous supplementation to maintain health and/or growth [1,2]. Short bowel syndrome (SBS) due to loss of intestinal length is the most common cause of intestinal failure and the focus of this review. SBS etiology differs depending on age (Table 1). In children, SBS results from conditions leading to extensive intestinal resection and can be divided into prenatal, or congenital, and postnatal categories. The most common condition in children leading to extensive surgical resection and SBS is necrotizing enterocolitis [3]. In adults, more common causes of extensive bowel resection include mesenteric infarction, followed by radiation enteritis and surgical complications [4].
The burden of at-home preparation of lyophilized parenteral medications: an analysis of contributing factors and implications for chronic disease patients and caregivers
Published in Expert Opinion on Drug Delivery, 2019
Chris Franzese, Colby Thomas, Amy Rinaldi, Kevin Constable, Koji Nakamura, Adam Kalbermatten, Marty Coyne
Short bowel syndrome (SBS) is a malabsorptive condition that results from surgical resection or congenital disease of the small intestine, leading to intestinal failure and inability to maintain adequate nutrition from a normal diet [53]. As a result, patients with SBS initially require total parenteral nutrition (TPN) to meet their energy requirements. Although SBS-induced intestinal failure and dependence on TPN is reversible in many patients, a portion of patients may require additional intervention to help restore intestinal function and wean from TPN [54]. Teduglutide is approved for SBS patients as a long-term aid in TPN weaning and may be administrated for several months or up to the duration of a patient’s life [55]. Teduglutide is supplied in lyophilized vials that provide a total of 3.8 mg (0.38 mL) of teduglutide after reconstitution with 0.5 mL of sterile water for injection, which is supplied in pre-filled diluent syringes (Table 1). The recommended dosing is weight-based and is administered once daily by SC injection after reconstitution. Depending on their weight, some patients may require reconstitution of more than one vial to obtain the appropriate dose.