Abstract

Severe short bowel syndrome (SBS) is a major cause of chronic (Type 3) intestinal failure (IF) where structural and functional changes contribute to malabsorption and risk of micronutrient deficiencies. Chronic IF may be reversible, depending on anatomy and intestinal adaptation, but most patients require long-term nutritional support, generally in the form of parenteral nutrition (PN). SBS management begins with dietary changes and pharmacologic therapies taking into account individual anatomy and physiology, but these are rarely sufficient to avoid PN. New hormonal therapies targeting intestinal adaptation hold promise. Surgical options for SBS including intestinal transplant are available, but have significant limitations. Home PN (HPN) is therefore the mainstay of treatment for severe SBS. HPN involves chronic administration of macronutrients, micronutrients, fluid, and electrolytes via central venous access in the patient’s home. HPN requires careful clinical and biochemical monitoring. Main complications of HPN are related to venous access (infection, thrombosis) and metabolic complications including intestinal failure associated liver disease (IFALD). Although HPN significantly impacts quality of life, outcomes are generally good and survival is mostly determined by the underlying disease. As chronic intestinal failure is a rare disease, registries are a promising strategy for studying HPN patients to improve outcomes.

Highlights

  • Intestinal failure (IF) is a state of “inability to maintain protein-energy, fluid, electrolyte or micronutrient balance” resulting from bowel resection or obstruction, dysmotility, congenital gastrointestinal defects, or loss of absorption as a consequence of disease [1]

  • An adaptive response is seen and manifests as gradually improving absorption of micro and macronutrients, and decreased losses of fluid. This phase is mediated by various intestinal hormones and growth factors, including growth hormone (GH), glucagon like peptide 2 (GLP-2), and epidermal growth factor (EGF), which promote structural and functional changes in the remnant small bowel as well as the colon to increase intestinal absorptive surface and absorptive capacity [11,12]

  • In Canada, more than 90% of Home PN (HPN) use is in the context of short bowel syndrome (SBS)

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Summary

Introduction

Intestinal failure (IF) is a state of “inability to maintain protein-energy, fluid, electrolyte or micronutrient balance” resulting from bowel resection or obstruction, dysmotility, congenital gastrointestinal defects, or loss of absorption as a consequence of disease [1]. Type 1 IF is a transient state such as ileus following abdominal surgery, which may require a brief period of nutritional support, but is simple to manage and fully reverses without sequelae [2]. Type 2 IF occurs in the context of severe illness such as major surgery, where complications due to sepsis, metabolic derangement, and malnutrition require complex multidisciplinary care and specialized nutrition support including parenteral nutrition (PN) [2]. These patients may recover fully or progress to Type 3 intestinal failure, which is a chronic state of IF requiring long-term nutritional support, typically in the form of home parenteral nutrition (HPN) [3]. The purpose of this review is to discuss HPN as a treatment for Type 3 IF, with a focus on short bowel syndrome

Short Bowel Syndrome
Mechanisms of Malabsorption
Diet and Micronutrient Supplementation
Pharmacologic Therapy
Surgical Options
Home Parenteral Nutrition
HPN Initiation
Monitoring
Venous Access Related Complications
Other Metabolic Complications
Outcomes of Home Parenteral Nutrition
The Future of HPN Research
Findings
Conclusions
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