Crohn's disease is one of the leading causes of intestinal failure. The term ‘type 2’ intestinal failure is used to describe the relatively rare type of intestinal failure that occurs in association with septic, metabolic and complex nutritional complications, typically following surgical resection and/or laparostomy for intra-abdominal sepsis. A multidisciplinary approach to the management of patients with type 2 intestinal failure is crucial, and it is helpful to approach patient care in a structured manner using the ‘sepsis-nutrition-anatomy-plan’ algorithm: resolution of sepsis is required before adequate nutritional repletion can be achieved, and it is crucial to optimise nutritional status, and define intestinal anatomy before delineating a definitive medical or surgical plan. A structured approach to the management of patients with inflammatory bowel disease, who have developed type 2 intestinal failure, should reduce the likelihood of these patients developing ‘type 3’ intestinal failure, which is characterised by the need for long-term parenteral nutrition. However, Crohn's disease is still the commonest indication for home parenteral nutrition in the UK.

Pharmacological, surgical and technological advances have resulted in children now surviving through adolescence into adulthood with conditions that were previously unseen by adult services. Arranging transition for young people on home parenteral nutrition (HPN) to the adult sector is one of greatest challenges for health services that care for young people. Transition is not only a key quality issue for health services, but is a multidimensional process covering psychosocial, educational and vocational aspects. Poorly-planned transition may result in difficulties when young people access adult specialist services. As a consequence, there may be increased risk of non-adherence or lack of follow-up, which carries dangers of morbidity and mortality as well as poor social and educational outcomes. Transition does not end at the exit from the paediatric clinic, but continues into the adult sector, which needs to provide developmentally-appropriate clinical care. Recent Department of Health initiatives are aimed at ensuring that young people do not miss out on healthcare during the transfer between paediatric and adult services. Transfer can be a major, often daunting, event for young people. Parents may also fear transfer and need to learn to ‘let go’ of some control, which may be particularly difficult with a young person on HPN.

Patients with irreversible intestinal failure and complications of parenteral nutrition should now be routinely considered for small intestine transplantation. Despite attempts for >40 years immunological graft intolerance presented an impenetrable barrier to successful engraftment until the development in the late 1970s of the powerful calcineurin-inhibitor immunosuppressive agents. Their use over the last 17 years has led to small intestinal transplantation being generally considered as a routine option for patients with irreversible intestinal failure and failing parenteral nutrition. The 1-year patient survival rates (%) are now excellent for renal (95), liver (78), heart (82) and lung (75) transplantation. In contrast, survival rates for small intestinal transplantation have been slow to improve, although they are now approaching those for lung and liver transplantation (intestine 78%, intestine and liver 60%, multivisceral 66%), and well-performing centres report recent 1-year graft survival rates as high as 92%. Patient 5-year survival (%) has also improved (intestine alone 50, intestine and liver 50 and multivisceral 62) and compares increasingly favourably with renal (85), liver (67), heart (67) and lung (46). Currently, small intestinal transplantation is reserved for patients with irreversible small intestinal failure who have a poor prognosis on parenteral nutrition. However, as 5-year patient survival following intestinal transplantation approaches that for parenteral nutrition there will be increasing pressure to offer this modality of treatment as an alternative to parenteral nutrition, especially for those patients who have a poor quality of life as a result of parenteral nutrition.

Managing infants, children and adolescents, ranging from premature infants to 18-year-old adolescents, on parenteral nutrition (PN) is a challenge. The ability of children to withstand starvation is limited and, unlike adults, children require nutrition for growth. PN in children is often required secondary to a congenital bowel problem rather than because of an acquired condition. Conditions requiring PN include motility disorders, congenital disorders of the intestinal epithelium and short-bowel syndrome (SBS). Intestinal failure may be temporary and children with SBS may be weaned from PN. However, other children require permanent PN. There are no comprehensive guidelines for the nutritional requirements of children and adolescents requiring PN. Practice in individual centres is based on clinical experience rather than clinical trials. Requirements are assessed on an individual basis according to age, nutritional status and clinical condition. These requirements need regular review to ensure that they remain appropriate for the changing age and weight of the child. Assessments of intakes use different methods, e.g. reference tables and predictive equations. Complications of PN include infection, accidental damage to, or removal of, the line and cholestatic liver disease. Home parenteral nutrition (HPN) is associated with fewer line infections and allows continuation of nutritional support in a more normal environment, encouraging normal development and participation in family activities. However, having a child at home on HPN is associated with physical and psychological stresses. A feeling of depression, loneliness and social isolation is common amongst children and their families. Home-care services are essential to supporting children at home and should be tailored to, and sensitive to, the individual needs of each family.

The prevalence of obesity in the general population is high and it is inevitable that artificial feeding will be needed from time to time in the obese patient, particularly in the critical care setting. Against a background of generous endogenous stores of energy as adipose tissue and the ability of obese individuals to survive starvation longer than non-obese individuals, emphasis is placed on preserving lean body mass and optimizing physiological function. Insulin resistance is typical of the obese individual and is exacerbated by stress; overfeeding is dangerous, particularly if it results in hyperglycaemia. Refeeding syndrome also has to be avoided. Weight may be difficult to measure and lean body mass difficult to assess. Calculation of energy requirements is therefore problematic in practice in the obese individual and there is substantial evidence from controlled clinical trials of the safety of feeding at or below resting energy expenditure. If this approach is taken it is wise to provide a more generous than normal protein intake and to beware of patients with a very high baseline urinary N excretion.

The British Artificial Nutrition Survey 2001 recorded 507 home parenteral nutrition (HPN) patients (Crohn's disease 31.5%, vascular disease 19.7%, cancer 6.9%). Parenteral nutrition was administered via tunnelled central line (92%) and supplied by a commercial homecare company in 89% of cases. The majority of HPN patients live at home (95.5%) with an independent life (74%), normal activity (59.2%) and 92% survive 1 year. However, there is good evidence that the geographical distribution of HPN patients is uneven (prevalence no patients to thirty-six patients per million of the population) suggesting inequity of access. Patients are increasingly concerned about the distances travelled to main centres and variable standards of more local support. Funding issues continue to cause difficulties as commissioning of health care transfers from Health Authorities to Primary Care Trusts. The two nationally-funded intestinal failure units provide HPN services to 220 HPN patients. HPN-related readmissions have displaced those awaiting admission for intestinal failure treatment, for which the waiting list mortality in one unit has risen to 14%. The government has now recognised HPN as a specialised service distinct from intestinal failure and that existing medium-sized HPN units should be encouraged to take on HPN patients from intestinal failure units and smaller units. In Scotland a Managed Clinical HPN Network supported by the Scottish administration now cares for seventy-two patients under common protocols. The challenge for the future is how to provide high-quality care to all who need it in the rest of the UK.

Surgery plays a key role in the management of both acute and, less frequently, chronic intestinal failure. Acute intestinal failure frequently requires surgical treatment when it arises as a consequence of intestinal fistulation or obstruction. In specialised clinical practice approximately 50% of acute intestinal failure is associated with intestinal fistulas and in approximately 50% of patients, this condition arises as part of the natural history or complicating treatment for Crohn's disease. A considerable proportion of such patients have abdominal infection and present complex nutritional and metabolic problems. The most important aspect of the surgical management of patients with acute intestinal failure associated with intra-abdominal infection is management of sepsis, since recovery is unlikely in the presence of active infection. Moreover, effective nutritional support and restoration of body composition is not possible if sepsis remains unresolved. Surgical strategies to deal with intra-abdominal infection may involve percutaneous drainage, laparotomy and resection of fistulating segments of intestine and, when infection is persistent and contamination extensive, laparostomy (a technique in which the abdomen is left open and allowed to heal by secondary intention). Surgical treatment should not only be timely and effective, but also aimed at preventing secondary damage to the small intestine, in order to minimise the risk of short bowel syndrome. In some cases a proximal defunctioning stoma may be required, with prolonged nutritional support, using either home total parenteral nutrition or feeding via the defunctioned distal gut (fistuloclysis), pending restoration of intestinal continuity. The role of surgical treatment for patients with short bowel syndrome is less clear. While surgery is frequently required for the management of complications of short bowel syndrome (including gallstones and possibly peptic ulcer disease), the role of intestinal lengthening and tapering procedures (to increase functional intestinal length), and artificial valves, reversed segments and colonic interposition (to reduce intestinal transit) remains controversial. For some patients with short bowel syndrome and, in particular, those with combined intestinal and hepatic failure, intestinal transplantation may become the treatment of choice as long-term results continue to improve.

A new definition of intestinal failure is of reduced intestinal absorption so that macronutrient and/or water and electrolyte supplements are needed to maintain health or growth. Severe intestinal failure is when parenteral nutrition and/or fluid are needed and mild intestinal failure is when oral supplements or dietary modification suffice. Treatment aims to reduce the severity of intestinal failure. In the peri-operative period avoiding the administration of excessive amounts of intravenous saline (9 g NaCl/l) may prevent a prolonged ileus. Patients with intermittent bowel obstruction may be managed with a liquid or low-residue diet. Patients with a distal bowel entero-cutaneous fistula may be managed with an enteral feed absorbed by the proximal small bowel while no oral intake may be needed for a proximal bowel enterocutaneous fistula. Patients undergoing high-dose chemotherapy can usually tolerate jejunal feeding. Rotating antibiotic courses may reduce small bowel bacterial overgrowth in patients with chronic intestinal pseudo-obstruction. Restricting oral hypotonic fluids, sipping a glucose-saline solution (Na concentration of 90–120 mmol/l) and taking anti-diarrhoeal or anti-secretory drugs, reduces the high output from a jejunostomy. This treatment allows most patients with a jejunostomy and >1 m functioning jejunum remaining to manage without parenteral support. Patients with a short bowel and a colon should consume a diet high in polysaccharides, as these compounds are fermented in the colon, and low in oxalate, as 25% of the oxalate will develop as calcium oxalate renal stones. Growth factors normally produced by the colon (e.g. glucagon-like peptide-2) to induce structural jejunal adaptation have been given in high doses to patients with a jejunostomy and do marginally increase the daily energy absorption.

Despite the reported high survival with total parenteral nutrition (TPN) therapy for patients with intestinal failure, a considerable number of patients do not escape the potential risks of TPN-associated complications, including hepatic failure, vanishing of central venous access and line sepsis. Thus, intestinal, liver-intestinal and multivisceral transplantation have recently emerged to rescue those who can no longer be maintained on TPN. Before this development, and for nearly three decades, small-bowel transplantation was plagued with uncontrolled rejection, graft v. host disease and fatal infection. These barriers stemmed from the large gut lymphoid mass and heavy microbial load contained in the intestinal lumen. The recent improvement in survival after the clinical introduction of tacrolimus with achievement of full enteric nutritional autonomy qualified the procedure by the US Health Care Financing Administration as the standard of care for patients with intestinal and TPN failure. The decision was supported by a decade of clinical experience with cumulative improvement in patient and graft survival. In addition, the introduction of new effective immunoprophylactic agents and novel therapeutic approaches has contributed to a further increase in the therapeutic advantages of the procedure. The present review article outlines the current clinical practice of intestinal transplantation and defines new management strategies with the aim of raising the level of the procedure to be a better alternative therapy for TPN-dependent patients.

Intravenous lipid emulsions (ILE) have demonstrated advantages including prevention of essential fatty acid (EFA) deficiency; however, too much EFA can down regulate fatty acid elongation leading to an imbalance of nutritional compounds in plasma and cell membranes. An olive oil-based ILE containing long-chain triacylglycerols (LCT) with a low content (20 %) of PUFA was administered for home parenteral nutrition (HPN) and compared with a conventional soyabean oil-based ILE (PUFA content, 60 %). Thirteen patients (26–92 years) with stable intestinal failure were randomised after a 1-month run-in period with a medium-chain triacylglycerols–LCT-based ILE, to receive 3 months of HPN with either olive oil- (n 6) or soyabean oil-based (n 7) ILE. The nutritional impact and safety of HPN, oral intakes and absorption rates, phospholipid fatty acids in plasma and lymphocyte cell membrane were assessed. The only clinical event reported was one case of pneumonia (soya group). In both groups, 20 : 3n-9:20:4n-6 ratios remained within normal ranges (0·03–0·07). There was a significant increase of γ-linolenic acid (γ-LA) in plasma and lymphocyte cell membrane (P=0·02) and of oleic acid in plasma (P<0·01) in the olive compared with the soya group. A significant correlation was found between γ-LA (day 90 – day 0) in plasma and PUFA parenteral intakes (P=0·02), but neither with fat intakes nor with fat absorption rates. In conclusion, plasma and lymphocyte EFA pattern remained in normal ranges without EFA deficiency with both lipid emulsions, despite a lower content of n-3 and n-6 series with the olive oil-based ILE.

Home parenteral nutrition is an established method of supporting patients with intestinal failure, but this treatment may be life long and imposes severe restrictions on daily life. Impact on quality of life is an important outcome when considering the management of home parenteral nutrition patients. This paper reviews studies in which the quality of life of patients receiving home parenteral nutrition has been assessed. A systematic search of electronic databases and relevant publications was undertaken to identify generic or treatment-specific questionnaires used with home parenteral nutrition patients. Many of the thirty-four reports discovered were small studies. Nineteen used non-specific generic instruments, eight used non-validated questionnaires, four used a combination of both, and three did not use any formal tool. Few systematic patterns emerged. There are few available data on the quality of life of home parenteral nutrition patients, and there is a need for standardised, scientifically validated, treatment-specific instruments to measure quality of life in this population.