Severe Short Bowel Syndrome Research Articles

Severe short-bowel syndrome after total small bowel resection

Severe short-bowel syndrome after total small bowel resection

Cholecystokinin (CCK) secretion in patients with severe short bowel syndrome (SSBS).

This study examined the effects of a liquid meal on cholecystokinin (CCK) secretion in patients with severe short bowel syndrome (SSBS) receiving home total parenteral nutrition (TPN) support for 5-19 years after massive small bowel resection. Five patients with SSBS due to superior mesenteric artery or vein thrombosis were included. Five healthy volunteers served as controls. Blood was drawn before and 1 hr following consumption of 250 ml of a liquid diet containing 232 kcal with 8 g fat and 8 g protein. Plasma CCK activity was evaluated by amylase bioassay. All patients had stable weight with a normal BMI and serum albumin level, although there were mild abnormalities in their liver function tests. CCK secretion after stimulation was significantly decreased in patients. These results suggest that reduction in intestinal length influences CCK secretion in response to meal stimulation in SSBS patients.

Remaining small bowel length: association with catheter sepsis in patients receiving home total parenteral nutrition: evidence of bacterial translocation.

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.

Cognitive deficits in school-age children with severe short bowel syndrome

Background/Purpose: Improved therapies for the management of short bowel syndrome (SBS) have resulted in the prolonged survival of many children. By early childhood, the physiological sequelae of severe SBS include delayed physical development and metabolic imbalances. However, little is known about how SBS affects brain development. Although many parents report school problems, no controlled study has evaluated the integrity of the central nervous system in SBS children. The purpose of this study was to investigate the neuropsychological status of school-aged SBS children to determine if there were characteristic cognitive impairments that might be amenable to early therapeutic intervention. Methods: SBS children (n = 8; mean age, 116.9 ± 21 months) were compared with an age-matched cystic fibrosis (CF) control group (n = 8; mean age, 118.1 ± 14 months). Groups did not differ in age, grade, or absences. Neuropsychological tests with established sensitivity to CNS integrity compared performance over 6 cognitive domains. Emotional status also was measured. Analyses were completed with 2-tailed t tests. Results: Groups did not differ on tests of intellectual ability and emotional function. Language, memory and learning, and problem-solving testing results indicated no significant group differences. However, the SBS group performed more poorly on measures assessing visual-spatial ability, with P values ranging from.002 to.045. In a subset of subjects, we noted significantly slower left-handed, but not right-handed, performance on measures of finger dexterity and psychomotor speed. Conclusions: Although emotional status did not differ from that of children with CF, SBS patients showed visual-spatial deficits in the company of preserved language, attention and memory, and executive skills. The specificity and consistency of these findings suggests that right hemisphere CNS changes may occur in children with SBS. This unexpected finding, coupled with the indication of left-sided psychomotor slowing in right-handed subjects, raises the possibility that actual brain impairment, rather than developmental delay accompanying slowed physical growth, accounts for these findings. Longitudinal studies are needed to further clarify this issue. The educational significance of the results is discussed. J Pediatr Surg 35:860-865. Copyright © 2000 by W.B. Saunders Company.

Differential jejunal and colonic adaptation due to resection and IGF-I in parenterally fed rats.

Patients with severe short-bowel syndrome (SBS) often require long-term total parenteral nutrition (TPN) to maintain their nutritional status because of limited intestinal adaptation. Growth factors, including insulin-like growth factor I (IGF-I), are under investigation to promote intestinal adaptation and tolerance to oral feeding. We investigated structural and functional adaptation of the jejunum and colon in four groups of rats maintained with TPN for 7 days after a 60% jejunoileal resection and cecectomy or sham surgery and treatment with IGF-I or vehicle. Resection alone did not stimulate jejunal growth. IGF-I significantly increased jejunal mucosal mass, enterocyte proliferation, and migration rates. IGF-I decreased jejunal sucrase specific activity and reduced active ion transport and ionic permeability; resection alone had no effect. In contrast, resection significantly increased colonic mass and crypt depth but had no effect on active ion transport or ionic permeability. IGF-I had minimal effects on colonic structure. IGF-I but not resection stimulates jejunal adaptation, whereas resection but not IGF-I stimulates colonic growth in rats subjected to a model for human SBS. IGF-I treatment may improve intestinal adaptation in humans with SBS.

Severe short-bowel syndrome in children: Clinical experience: J.C. de Agustin, J.J. Vazquez, D. Rodriguez Arnao, et al. Eur J Pediatr Surg 9:236–241, (August), 1999

Severe short-bowel syndrome in children: Clinical experience: J.C. de Agustin, J.J. Vazquez, D. Rodriguez Arnao, et al. Eur J Pediatr Surg 9:236–241, (August), 1999

Severe short-bowel syndrome in children. Clinical experience.

Innovative surgical and pharmacological therapeutic measures in short-bowel syndrome (SBS) are constantly changing the prognosis of this devastating condition. The aim of this paper is to present our most recent experience in the treatment of this disease, with particular emphasis on the impact of home parenteral nutrition (HPN) and the use of growth hormone (GH). A group of 8 patients with severe SBS have been studied for the past 4 years. Intestinal length of less than 25% normal at the time of bowel resection was the criterion for inclusion in this study. Mean age at the time of diagnosis was 2 years (ranging from 1 day to 9 years). The etiology of the SBS was Hirschsprung's disease (n = 3), midgut volvulus (n = 2), gastroschisis (n = 1), omphalocele with ileal atresia and necrotizing enterocolitis (n = 1) and Crohn's disease (n = 1). Length of the residual bowel was 8 and 50 cm with ileocecal valve (ICV) preservation and 23, 27, 30, 50, 70, 100 cm without ICV. Sixty percent of the patients survived. Two patients died due to fulminant gram-negative sepsis and one due to cardiac malformation. Two patients are still on parenteral nutrition (PN) providing 30 and 60% of total calories. Human GH (0.3 U/kg/day) was used in two patients over a period of 28 days. In these patients, an increased tolerance to enteral feeding was observed. HPN was provided in 5 cases, allowing regular school attendance in 3 patients. In 3 cases, discontinuation of the PN was achieved at 24, 25 and 35 months respectively. Human GH can improve tolerance of enteral feeding. HPN has a beneficial effect on child behaviour. Intestinal transplantation must be considered when no other surgical or medical measures are available.

Treatment of severe short bowel syndrome with high dose growth hormone and glutamine

Treatment of severe short bowel syndrome with high dose growth hormone and glutamine

Growth hormone and glutamine do not stimulate intestinal adaptation following massive small bowel resection in the rat.

Certain nutrients and other trophic factors are highly sensitive stimulants of intestinal adaptation following short bowel syndrome. Growth hormone and glutamine in a modified diet have been shown to enhance nutrient absorption in patients with severe short bowel syndrome. However, neither growth hormone nor glutamine is capable of enhancing adaptation in an animal model. This study was conducted to determine if the combination of glutamine and growth hormone could enhance gut adaptation following massive small bowel resection in the rat. Thirty-four male rats received 70% jejunoileal resection. The first group received glycine and rat growth hormone, the second glutamine and rat growth hormone, and the third glycine but no growth hormone. There was no evidence that the combination of glutamine and growth hormone could enhance mucosal mass, mucosal protein, or mucosal DNA levels relative to the other two control groups of animals. Likewise, sucrase activities were not enhanced by glutamine and growth hormone. It is unlikely that the combination of glutamine and growth hormone will be of benefit in the treatment of patients with short bowel syndrome. The results in previous human studies can be alternatively explained by the long-term nonspecific effect of enteral nutrition on gut adaptation.

Intestinal adaptation in short bowel syndrome

Short bowel syndrome (SBS) either in adults or in children is considered as an indication of small bowel transplantation (SBT). 1,2 Severity and prognosis depend on various factors such as small bowel resection length, patient's age, and intestinal length at the time of resection. In pediatrics, most patients, affected by SBS undergo resection when neonates or anyway in the first months of life (necrotizing enterocolitis, congenital anomalies of gastrointestinal troct, abdominal wall defects); only in few patients pathology begins later on, usually for tumoral, vascular or Crohn's diseases. Intestinal adaptation process, characterized by epithelial hyperplasia, has an early onset, within the first days after resection, with a progressive increase of intestinal absorptive surface; such adapting capacity is greater for ileum rather than for jejunum. 2–5 This intestinal adaptation process with mucosal hyperplasia requires an enteral nutrition which acts both directly by nutrients contact on intestinal epithelium and by stimulating the secretion of trophic factors such as enteroglucagon, epidermal growth factor and insulin-like growth factor-1. From the clinical point of view, the term “adaptation” means the total nutritional autonomy with a satisfying height and weight growth. In patients who initially need parenteral nutrition (PN), total intestinal adaptation is considered as attained when they can stop PN. The aim of the present study is to evaluate retrospectively our series of severe SBS patients, with residual intestine shorter than 100 cm, who consequently required a prolonged PN, verifying the possibility and the time of intestinal adaptation and therefore the existence or not of an indication to intestinal transplantation.

Treatment with an HLA-peptide and cyclosporine a prolongs rat small bowel allograft surgival

Background: The ultimate treatment for severe short bowel syndrome is small bowel transplantation (SBT). Current immunosuppression for SBT is relatively ineffective and toxic. Peptides derived from residues 75–84 of the HLA-B7 molecule are immunomodulatory in vitro, and in rodents, when combined with subtherapeutic doses of cyclosporine (CsA), prolong cardiac and skin allograft survival without altering the recipient's rejection kinetics to third party allografts. We investigated the effects of HLA-B7 peptide fragment in a rat model of SBT. Methods: Heterotopic allogeneic SBT was performed in Dagouti (RT1 a) to Lewis (RT1 I) high-responder rat strain combination. B7. 75–84 (40 mg/kg/d) and subtherapeutic CsA (10 mg/kg/d) were administered alone, or in combination, by gavage to allograft recipients on days 0 to 4 after SBT. Recipient pretreatment with B7.75–84 on days −14, −12, −10, and −7 followed by subtherapeutic CsA on days 0 to 4 after SBT was also carried out. Graft rejection was determined by the presence of a palpable abdominal mass on daily examination or by loss of more than 10% initial body weight. Results: Without immunosuppression allografts rejected at a median time of 6 days (range, 5 to 7; n = 7). This was not significantly altered with either CsA therapy alone (median 6 days; range, 6 to 7; n = 6) or B7.75–84 alone (median, 5 days; range, 5 to 6; n = 6). Recipient combination therapy with B7.75–84 and CsA after allografting significantly prolonged allograft survival (median, 11 days; range, 9 to 13; n = 9), as did recipient B7.75–84 pretransplant therapy (median, 10 days; range, 9 to 12; n = 6), when administered over a 2-week period before allografting. Conclusion: Post-SBT recipient treatment with B7.75–84 produced statistically significant improvement in allograft survival only after combination with subtherapeutic CsA. Recipient pre-SBT treatment with B7.75–84 alone however, resulted in statistically significant improvement in allograft survival in combination with post-SBT subtherapeutic CsA. These synergistic effects may be valuable in achieving improved SBT survival clinically and warrant further exploration.

Small intestinal transplantation for irreversible intestinal failure in children.

Permanent intestinal failure, whether secondary to severe gastrointestinal tract dysfunction or short bowel syndrome, remains a challenging pediatric health problem that requires innovative therapeutic modalities. Steatorrhea and intractable diarrhea result in failure to thrive, hypovitaminosis, hypoproteinemia, electrolyte disturbance, and metabolic acidosis if parenteral alimentation (TPN) is begun too late. A variety of medical and surgical treatments have been devised to address each of the underlying causes of childhood intestinal failure. None the less, a significant percentage of children with intestinal failure remain permanently bound to parenteral nutrition. While some of these children suffer minimal morbidity, others succumb to the life-threatening complications of TPN. Until recently they had no hope for survival, but within the past decade small intestinal transplantation has offered them an opportunity for prolonged life emancipated from the shackles of TPN. In this review, we shall discuss in detail microvillus inclusion disease, intestinal pseudoobstruction, and short bowel syndrome, which are the most common causes of pediatric intestinal failure coming to intestinal transplantation. The factors facilitating intestinal adaptation or predisposing to irreversible liver disease in children with short bowel syndrome will be described. We shall also enumerate the indications for pediatric intestinal transplantation and outline the surgical techniques, postoperative management, and results of our experience to date at the University of Pittsburgh Transplantation Institute. Finally we shall recount recent scientific advances likely to improve patient survival in the future.

Intensive Nutritional Support and Remedial Surgical Intervention for Extreme Short Bowel Syndrome

Management of extreme short bowel syndrome (SBS) has changed dramatically over the last 20 years with notable improvements in survival and quality of life in patients with this syndrome. A review of our institution's medical records over a 12-year period (1980-1992) revealed 32 patients with < 100 cm (range, 14-94; median, 40) of functional small bowel after intestinal resection. The causes of intestinal loss included necrotizing enterocolitis (11 cases), atresias (8 cases), long-segment Hirschsprung's disease (5 cases), midgut volvulus (5 cases) and gastroschisis (3 cases). The mean follow-up period was 4.2 years, and four deaths were recorded (12.5%). Survival of eight of nine (88.9%) patients without an ileocecal valve (ICV) and with < 40 cm of small bowel was noted. The absence of an ICV, however, was associated with significantly prolonged total parenteral nutrition. Follow-up surgical procedures, including intestinal lengthening, tapering enteroplasty. Martin's procedure, longitudinal myectomy-myotomy, and ostomy take-down, were performed in 20 of the patients (64%). Prolonged survival and normal development can be expected for the patient with severe SBS who is given meticulous nutritional support and treated with carefully planned secondary surgical intervention. These results are also seen in patients with extreme SBS (< 40 cm residual small bowel length) and no ICV.

Intensive Nutritional Support and Remedial Surgical Intervention for Extreme Short Bowel Syndrome

Summary: Management of extreme short bowel syndrome (SBS) has changed dramatically over the last 20 years with notable improvements in survival and quality of life in patients with this syndrome. A review of our institution's medical records over a 12‐year period (1980–1992) revealed 32 patients with &lt;100 cm (range, 14–94; median, 40) of functional small bowel after intestinal resection. The causes of intestinal loss included necrotizing enterocolitis (11 cases), atresias (8 cases), long‐segment Hirschsprung's disease (5 cases), midgut volvulus (5 cases) and gastroschisis (3 cases). The mean follow‐up period was 4.2 years, and four deaths were recorded (12.5%). Survival of eight of nine (88.9%) patients without an ileocecal valve (ICV) and with &lt;40 cm of small bowel was noted. The absence of an ICV, however, was associated with significantly prolonged total parenteral nutrition. Follow‐up surgical procedures, including intestinal lengthening, tapering enteroplasty, Martin's procedure, longitudinal myectomy‐myotomy, and ostomy takedown, were performed in 20 of the patients (64%). Prolonged survival and normal development can be expected for the patient with severe SBS who is given meticulous nutritional support and treated with carefully planned secondary surgical intervention. These results are also seen in patients with extreme SBS (&lt;40 cm residual small bowel length) and no ICV.

Adaptation in short-bowel syndrome: Reassessing old limits

The improving survival of patients with severe short-bowel syndrome along with the advent of successful intestinal transplantation have accentuated the need to answer two questions. (1) Is there an intestinal length below which adaptation to full enteral nutrition can not be expected to occur? (2) How much time is necessary to complete intestinal adaptation? We reviewed the outcome of 21 infants with less than 50 cm of small intestine to answer these questions. Patients were divided into three groups based on intestinal length, regardless of ileocecal valve status: group I, <10 cm (n = 3); group II, 10 to 30 cm (n = 11); and group III, 30 to 50 cm (n = 7). Data were collected to assess survival, incidence of adaptation, time to adaptation, and causes of mortality. Infants in group I did not achieve intestinal adaptation to full enteral nutrition. One survived and 2 died, one from varicella pneumonia and the other after intestinal transplantation. Eight of the 11 (73%) patients in group II survived and 5 of 8 (63%) survivors achieved full intestinal adaptation after a mean interval of 320 days (range, 148 to 506 days) on parenteral nutrition. Six of the seven patients (86%) in group III survived and all survivors (100%) achieved complete enteral adaptation after an average of 376 days (range, 58 to 727 days). The overall survival was 71% ( 15 21 ), but survival in patients with > 10 cm was 78%. The incidence of fatal catheter sepsis ( 2 21 ) and fatal parenteral nutrition-associated liver failure ( 1 21 ) was low and accounted for two of the six deaths—one patient died as a consequence of both complications. The 10-cm small intestinal length appears to be a new lower limit for the length necessary to achieve enteral nutrition. Patients with > 10 cm of small intestine should be supported on parenteral nutrition with early institution of enteral feedings to promote intestinal adaptation for a minimum of 2 years before transplantation is considered.

Lipomatous Hypertrophy of the Cardiac Interatrial Septum: An Unusual Complication in Long‐Term Home Parenteral Nutrition in Adult Patients

The aim of this study was to report unusual echocardiographic findings in patients on long-term home parenteral nutrition suggesting lipomatous hypertrophy of the cardiac interatrial septum. Seven patients (two women and five men, mean age 58 years) were prospectively evaluated by two-dimensional echocardiography before and during home parenteral nutrition for severe short-bowel syndrome. Parenteral caloric intake was 33 +/- 4 kcal/kg per day (mean +/- SD), with a mean supply of lipid emulsion of 1 g/kg per day. The first echocardiography, performed before initiation of home parenteral nutrition, was normal in the seven patients. The second echocardiography, performed 5 to 43 months (mean 20 months) later, revealed a significant increase of the thickness of the atrial septum in all patients (+8.3 +/- 3.6 mm; p less than 0.01), consistent with the diagnosis of lipomatous hypertrophy in four patients, and moderate fat accumulation in two others. The septum thickness measured after the nutritional support period was significantly correlated (p less than 0.05) with the total amount of nutrient and lipid infused. Cardiac nuclear magnetic resonance imaging was performed in two patients; it suggested the adipose nature of interatrial septum deposit. None of the patients developed clinical or electrocardiographic symptoms during the period of nutrition. We suggest that patients undergoing long-term home parenteral nutrition be screened for this new and unusual cardiac abnormality. Whether the latter might have clinical consequences in these patients remains to be determined.

Intestinal absorption of cholecalciferol and 25-hydroxycholecalciferol in patients with both Crohn's disease and intestinal resection

We compared the intestinal absorption of cholecalciferol and 25-hydroxycholecalciferol in patients with Crohn's disease and resections of the small bowel. Patients were subgrouped into those with small (less than 100 cm), intermediate (100-300 cm), and large (greater than 300 cm) resections. [3H]cholecalciferol or [3H]25-hydroxycholecalciferol were given orally and serial blood samples were taken for measurement of plasma radiolabeled vitamin. Absorption of both forms of the vitamin decreased with extent of resection but 25-hydroxycholecalciferol absorption was always greater than that of cholecalciferol. When compared with normal control subjects, 25-hydroxycholecalciferol absorption in these patients was better maintained than that of cholecalciferol. These data indicate that vitamin D malabsorption reflects the extent of distal small-bowel resection in Crohn's disease. Treatment with oral cholecalciferol is sufficient in those with small or moderate resections but oral 25-hydroxycholecalciferol supplementation may be preferred in those with a severe short-bowel syndrome.

Review: Short‐Bowel Syndrome: A Review of the Role of Nutrition Support

Advances in long-term venous access devices and in parenteral nutrition solutions have made it possible for patients with severe short bowel syndrome to survive and to live in our society. The spectrum of this disease is such that some patients may be able to lessen their dependence or even become free from parenteral therapy. This review will discuss the role of nutrition support in the patient with short bowel syndrome.

Nortriptyline Absorption in Short Bowel Syndrome

Severe short bowel syndrome usually requires a period of parenteral nutrition support until gastrointestinal hypertrophy occurs. Because of the shortened length of the gastrointestinal tract, oral drug therapy can be compromised secondary to decreased absorption. In the case presented, a patient with short bowel syndrome who required parenteral nutrition was able to achieve therapeutic nortriptyline serum concentrations while receiving the drug via the oral route.

Severe short bowel syndrome with a surgically reversed small bowel segment

We report a case of short bowel syndrome (60 cm of jejunum anastomosed to the left colon) with reversal of the distal 15 cm of jejunum in a 21-year-old man. The nutritional absorptive capacity and digestive motility was studied for 18 months postoperatively. His absorptive capacity reached subnormal values allowing him oral nutritive autonomy and normal social life. The results of the manometric study suggested that the reversed segment delayed intestinal transit time. The prolonged contact of the chyme with the intestinal absorptive mucosa possibly increased its absorptive capacity. Our data and the literature reports suggest that reversal of a bowel loop could help wean patients from their dependence on parenteral nutrition.