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Crohn's disease is a panenteric, transmural inflammatory disease of unknown origin. Although primarily managed medically, 70% to 90% of patients will require surgical intervention. Surgery for small bowel Crohn's is usually necessary for unrelenting stenotic complications of the disease. Fistula, abscess, and perforation can also necessitate surgical intervention. Most patients benefit from resection or strictureplasty with an improved quality of life and remission of disease, but recurrence is common and 33% to 82% of patients will need a second operation, and 22% to 33% will require more than two resections. Short-bowel syndrome is unavoidable in a small percentage of Crohn's patients because of recurrent resection of affected small bowel and inflammatory destruction of the remaining mucosa. Although previously a lethal and unrelenting disease with death caused by malnutrition, patients with short-bowel syndrome today can lead productive lives with maintenance on total parenteral nutrition (TPN). This lifestyle, however, does not come without a price. Severe TPN-related complications, such as sepsis of indwelling central venous catheters and liver failure, do occur. Future developments will focus on more powerful and effective anti-inflammatory medication specifically targeting the immune mechanisms responsible for Crohn's disease. Successful medical management of the disease will alleviate the need for surgical resection and reduce the frequency of short-bowel syndrome. Improving the efficacy of immunosuppression and the understanding of tolerance induction should increase the safety and applicability of small-bowel transplant for those with short gut. Tissue engineering offers the potential to avoid immunosuppression altogether and supplement intestinal length using the patient's own tissues.
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PMID:The long-term results of resection and multiple resections in Crohn's disease. 1070 28

Infants with very low birth weight (VLBW) are at increased risk of cholestasis when compared with older infants and children. Factors associated with this increased risk of cholestasis include immaturity of the biliary excretory system, a diminished immune response to sepsis, an increased incidence of necrotizing enterocolitis and short bowel syndrome, as well as an increased exposure to parenteral nutrition (PN). The current literature on cholestasis in VLBW infants and the factors that mediate the initiation and progression of cholestatic liver damage is reviewed. A protocol for managing infants with cholestatic jaundice is presented, and a case report is included that shows use of the protocol to normalize the bilirubin in a VLBW infant with severe cholestatic jaundice.
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PMID:Management of cholestasis in infants with very low birth weight. 1080 32

Small bowel transplantation is gradually changing from an experimental procedure to a very desirable and viable treatment option in children with irreversible intestinal failure due to either short bowel syndrome or functional impairment. Long term total parenteral nutrition and home parenteral nutrition would be necessary to manage these children in the absence of a small bowel transplant programme. Parenteral nutrition is also associated with complications which can result in chronic liver disease. In India, there is no infrastructure for this treatment option and even if it was there the cost of this method of treatment is likely to be more than the cost of post-operative immunosuppression. Small bowel can be transplanted as an isolated graft, in combination with the liver or as part of a multivisceral transplant. The operative techniques have been standardised. Major post-operative complications result from sepsis and lymphoproliferative diseases. The best results have been obtained with a combined liver and small bowel transplant.
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PMID:Current status of small bowel transplantation in children. 1083 57

A living-related small bowel transplantation (SBT) was performed in two pediatric patients with short bowel syndrome. In both cases, the donor was the patient's mother. The distal ileum (100 cm, 120 cm) was harvested and the ileocolic vessels, ileocecal valve, and terminal ileum were left intact. The two donors were discharged from the hospital on postoperative days 15 and 6, respectively. Recipient 1 was a 2 year 6 month-old boy with short bowel syndrome who underwent SBT due to loss of venous access. The graft vein was anastomosed to the recipient's infrarenal inferior vena cava. Despite triple immunosuppression (tacrolimus, steroid, and azathioprine), there were four episodes of rejection. The patient had been on total parenteral nutrition for almost his entire posttransplant course. He died from Pneumocystis carinii pneumonia 16 months after the transplantation. Recipient 2 was a 4 year 5 month-old girl with short bowel syndrome who underwent an isolated small bowel transplantation because of recurrent line sepsis. Her pretransplant bilirubin was 8.0 mg/dl and a biopsy showed severe fibrosis. The graft vein was anastomosed to the recipient's inferior mesenteric vein. After transplantation, her bilirubin level became normal within 10 days. Triple immunosuppression (tacrolimus, steroid, and cyclophosphamide) together with a 3-day course of OKT-3 made her post-transplant course feasible. After overcoming a single episode of rejection she left the hospital 4 months after SBT. The patient is currently (10 months after transplantation) hospitalized due to rejection, which is being successfully controlled, and she is off total parenteral nutrition. From our experience, harvesting of the distal ileum for use as a bowel graft can be safely performed. The advantages of living-related grafts, optimal graft length, and choice of vascular reconstruction in SBT are yet to be explored.
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PMID:Small bowel transplantation using grafts from living-related donors. Two case reports. 1111 92

Patients with short bowel syndrome (SBS) receiving total parenteral nutrition (TPN) have a high incidence of catheter-related sepsis, one of its major complications. The aim of this study was to correlate the length of remaining small bowel (RSB) with septic episodes related to the central venous catheter in a group of patients with severe SBS with home TPN. The length of the RSB (<50 cm or > or = 50 cm) was related to the frequency of catheter sepsis, time until the first episode, and the agents responsible in eight SBS patients receiving home TPN. There were 13 episodes of catheter infection (0.88 per patient-year). The group with a shorter RSB length (five patients) presented 1.3 to 2.76 infections/year and 2 to 9 months until the first episode, compared to 0 to 0.75 infections/ year (p = 0.0357) and 11 to 65 months until the first episode (p = 0.0332) in the group with the longer RSB. In the first group, the agents isolated were Enterobacteriae (Enterobacter sp., Klebsiella sp., Pseudomonas sp., and Proteus sp.) in eight episodes and Candida sp. in one. In the latter sepsis was caused by Staphylococcus sp. in three episodes and Pseudomonas sp. in one. Therefore patients with remaining small bowel shorter than 50 cm have a higher frequency of catheter-related sepsis, particularly by enteric microorganisms. This might be an evidence of the occurrence of bacterial translocation and its role in the pathogenesis of catheter-related sepsis in patients with an extremely short RSB receiving home TPN.
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PMID:Remaining small bowel length: association with catheter sepsis in patients receiving home total parenteral nutrition: evidence of bacterial translocation. 1119 20

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.
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PMID:Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. 1132 44

Short bowel syndrome is a spectrum of malnutrition resulting from inadequate bowel length. In infant and pediatric patients, the most common causes are necrotizing enterocolitis, abdominal wall defects, jejunal ileal atresia, and mid gut volvulus. There appear to be regional variations in etiology. Since the publication of Wilmore's classic monograph in 1972, there have been significant improvements in monitoring and nutritional support. In the modern era, survival rate ranges from 80% to 94%, and the presence or absence the ileal cecal valve appears to not impact on mortality rate, but does significantly affect the length of time on total parenteral nutrition TPN. The most common morbidities remain sepsis, both central line related and bacterial overgrowth, and TPN cholestasis. Long-term recovery of these children often is remarkably normal, but there is a 10% to 15% incidence of neurologic and developmental defects. The clinical and ethical considerations around the care of infants with 20 to 40 cm of residual bowel remains controversial, as does the place of intestinal transplantation, especially in patients developing gut failure in infancy. Perioperative surgical decision making plays a critical role in the long-term outcome of these patients. This chapter presents an overview of the current status of care and outcome in this difficult population; these topics are further expanded in subsequent chapters.
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PMID:Short bowel syndrome in infants and children: an overview. 1132 5

Intestinal transplantation has emerged as a feasible alternative in the treatment of children with short gut syndrome. The challenges in the management of these patients include maintaining a tight balance between the degree of immunosuppression necessary to prevent graft-versus-host disease and rejection. At the same time, this amount of immunosuppression is associated with a high risk for lymphoproliferative disorders and intestinal-derived sepsis. Current 3-year patient and graft survival rates are 55% and 50%, respectively. The indications, morbidity, and timing for referral are discussed.
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PMID:Intestinal transplantation for children with short bowel syndrome. 1132 11

Sepsis in short bowel syndrome (SBS) is due in part to bacterial translocation (BT). Parenteral nutrition (PN) is often necessary in SBS and promotes BT. The presence of ileocecal valve (ICV) has been considered as a good prognostic factor in the outcome of this children. The aim of this study was to asses the effect of the presence or absence of ICV and cecum in five different models of gut resection in the rat. Fifty-five adult Wistar rats were randomly assigned to one of five groups: Group 1 (N = 14): standard rat chow + 80% small bowel resection. Group 2 (N = 10): standard rat chow + 80% small bowel resection including cecum. Group 3 (N = 10): standard rat chow + 80% small bowel resection including ICV. Group 4 (N = 11): NP + 80% small bowel resection. Group 5 (N = 10): NP + 80% small bowel resection including ICV and cecum. Ten days after surgery they were sacrificed and mesenteric lymph nodes (MLN), spleen and peripheral (PBL) and portal blood (POBL) specimens were recovered and cultured. Groups 3 (without ICV, with cecum) and 5 (without ICV, without cecum) showed 60% BT in MLN and POBL, and groups 1 and 4 (with ICV, without cecum) 93% and 91% respectively (p < 0.05). In PBL, group 3 (without ICV, with cecum) showed also less BT than groups 1 and 4 (10% vs 43% and 55% respectively, p < 0.05) and group 5 (without ICV and cecum) had less BT than groups 1, 2 and 4 (0% vs 43%, 30% and 55%, p < 0.01). In conclusion, these results suggest that the absence of ICV decreases BT and that the cecum does not seems to play a role on his.
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PMID:[Bacterial translocation associated with short bowel: role of ileocecal valve and cecum]. 1148 Jan 92

The treatment of children with intestinal failure should be predicated upon three overriding goals: 1) to keep the patient well nourished by parenteral nutrition (TPN), 2) to minimize the fecal loss of fluid, electrolytes, and nutrients, and 3) to enhance the natural process of intestinal adaptation whenever possible. The first goal is relatively easy to accomplish in the short- or intermediate-term, but difficult to accomplish for more than a few years because of recurrent septicemia, loss of venous access, and cholestatic liver disease. The risks of sepsis and loss of venous access can be minimized through meticulous central line care and the use of appropriate antibiotics when indicated. Cycling TPN and limiting parenteral protein intake sometimes ameliorates cholestasis. The second goal is only partially achievable regardless of the cause of intestinal failure. Fluid and electrolyte secretion often can be reduced but not normalized with antisecretory drugs. Bacterial overgrowth can be treated with the judicious use of antibiotics. The third goal generally can be accomplished only in a subpopulation of patients with surgically created short bowel. In these children, a satisfactory increase in surface area can occur only if nutrients are delivered directly into the bowel lumen. The trophic effects of glutamine, growth hormone, and other hormones remain to be universally accepted. Surgical bowel lengthening or bowel tapering can sometimes enhance intestinal function among patients with short bowel syndrome. If medical or nontransplantation surgical management of intestinal failure is unsuccessful, and the patient develops irreversible TPN-associated complications, transplantation of the intestine should be strongly considered.
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PMID:Small Intestinal Failure in Children. 1156 Jul 89


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