Therapy For Short Bowel Syndrome Research Articles

Disaccharidase activities and fat assimilation in pediatric patients after intestinal transplantation.

Intestinal transplantation has become an accepted therapy for short bowel syndrome and other types of intestinal failure. In order to assess digestive capabilities and feeding practices in a group of 22 pediatric patients after intestinal transplantation, we assessed mucosal disaccharidase activities and assimilation of total dietary lipid and vitamin E. Twelve of the patients had undergone contemporaneous liver transplantation. Mucosal biopsies were assayed for disaccharidase activities between 15 and 412 days after transplantation in 7 of the 22 when all were receiving some enteral nutrition and were free of rejection. Coefficients of lipid absorption were determined in those patients receiving total enteral feeding (two-thirds polymeric/one-third elemental) between 43 and 1032 days after transplantation; oral vitamin E tolerance tests were done at about the same time. Activities of lactase, sucrase, maltase, and palatinase consistently exceeded reference ranges (P<0.05). Mean coefficient of lipid absorption equaled 86+/-12% and was not influenced by duration of time after transplantation. No patient required dietary lipid restriction. No significant absorption of vitamin E was demonstrated until 160 days after transplantation. Vitamin E absorption did correlate with length of time elapsed after surgery (r=0.64, P<0.0011). The results of this investigation show that, in the absence of histologic or clinical indications of allograft rejection, pediatric intestinal transplant recipients do not have primary disaccharidase deficiencies. Similarly, absorption of usual dietary lipid content is adequate once weaning from parenteral nutrition is complete. In contrast, early assimilation of vitamin E is poor. Vitamin E absorption subsequently improves, but the mechanism is obscure.

LIPID AND VITAMIN E ASSIMILATION IN PEDIATRIC PATIENTS FOLLOWING INTESTINAL TRANSPLANTATION

73 Background. Intestinal transplantation has become an accepted therapy for short bowel syndrome and other types of intestinal failure. In order to clarify the tempo of intestinal allograft function following transplantation, we assessed absorption of total dietary lipid and vitamin E. Methods. Eighteen patients were studied, 10 of whom underwent simultaneous liver transplantation. Coefficients of lipid absorption were determined 25 times between 43 and 1032 days (mean=217±210 days) post-operatively. All were receiving total enteral feeding, polymeric in 68% of patients and amino acid-based (mainly Neocate®) in the remainder. Oral vitamin E tolerance tests were done at about the same time. Vitamin E absorption was expressed as the maximum increase in the ratio of vitamin E to total serum lipid over the baseline (ΔE/lipid) following a test dose of 100 U/kg. Mucosal biopsies that were always obtained contemporaneously were uniformly normal. Results. Mean coefficient of lipid absorption equaled 86±12% and was not influenced by duration of time following transplantation or by type of transplant performed (isolated bowel vs. liver/bowel). No patient required dietary lipid restriction. No significant absorption of vitamin E was demonstrated until 160 days following transplantation. Vitamin E absorption did correlate with length of time elapsed after surgery (r=0.64, p<0.0011) but not type of transplant. ΔE/lipid rarely exceeded 4 mg/g. Conclusions. The results of this investigation show that in the absence of allograft rejection, pediatric intestinal transplant recipients have adequate absorption of dietary lipid once weaning from parenteral nutrition is complete. In contrast, early assimilation of vitamin E is poor. Vitamin E absorption subsequently improves, but the mechanism is obscure.

Conjugated bile acid replacement therapy for short-bowel syndrome

Conjugated bile acid replacement therapy for short-bowel syndrome

A modification of the Bianchi intestinal lengthening procedure with a single anastomosis

Background/Purpose: Intestinal lengthening, as described by Bianchi, is the most commonly used method of gastrointestinal reconstruction for the therapy of short bowel syndrome. It divides the bowel in two longitudinal halves based on the bifurcated mesenteric blood supply, then reconnects the two halves in series with the rest of the small intestine. This requires three end-to-end narrow anastomoses in addition to the two longitudinal sutures lines. The authors describe a modification of the Bianchi procedure that lengthens the small intestine with a single wide end-to-end anastomosis thus avoiding two suture lines and their potential complications. Methods: The anatomic principle used in this modification is the same as that described by Bianchi. The mesentery is separated based on the bifurcated vessels. With sequential firings of the stapler, the division of the intestine begins obliquely, proceeds longitudinally as in the Bianchi procedure, then ends obliquely at the other end of the intestine. This results in two divided segments that remain attached to the proximal and distal intestine and end in tapered staple lines. The two ends are sewn into a wide tapered anastomosis, reestablishing intestinal continuity with a single anastomosis instead of three. Conclusions: This simple modification, which we have used on two patients so far, decreases the number of anastomoses in an intestinal lengthening procedure from three narrow ones to one that is wider and tapered, thus reducing the risk of leaks and strictures. Because the end result is a lengthened intestinal segment similar to the Bianchi procedure, it should be equally effective in the therapy of short bowel syndrome.

Massive macrophage lipid accumulation presenting as hepatosplenomegaly and lymphadenopathy associated with long-term total parenteral nutrition therapy for short bowel syndrome

We present a unique case of massive splenomegaly, hepatomegaly, and lymphadenopathy caused by lipid-laden macrophages in a 50 year old white female with short-bowel syndrome treated with long-term total parenteral nutrition. Using transmission electron microscopy and special stains we were able to show that the total parenteral nutrition lipid component was composed of lipid droplets and micelles morphologically identical to those found in lipid-laden macrophages which had accumulated in the patient's reticuloendothelial system leading to massive splenomegaly, hepatomegaly (without evidence of steatosis) and lymphadenopathy. While this phenomenon has been reported in animal models, no human cases have been previously reported.

Surgical aspects of the short-bowel syndrome

Background: Surgeons are frequently confronted with patients with the short-bowel syndrome. Important surgical issues are maintaining intestinal continuity, treating complications, and performing procedures to improve intestinal function. Methods: A comprehensive review of the English language literature and the author's own experience were employed to make recommendations about surgical management of the short-bowel syndrome. Conclusion: At the time of initial resection, ostomy formation is often prudent. The decision to restore continuity at a later time should balance anticipated functional outcome against potential complications. Several surgical strategies can be employed at reoperation in these patients to minimize further loss of intestine. Prophylactic cholecystectomy should be considered because of the increased risk of cholelithiasis. Gastric hypersecretion rarely requires operative therapy. Surgical therapy for the short-bowel syndrome includes procedures to slow intestinal transit, optimize intestinal function, and increase intestinal surface area. The choice of operation is influenced by intestinal remnant length and caliber and its function. Only a small proportion of patients are candidates for nontransplant procedures, of which intestinal lengthening is most efficacious. Intestinal transplantation, either alone or combined with the liver, is emerging as the most promising therapy in short-bowel syndrome.

Segmental intestinal transplantation can be an adequate therapy for short bowel syndrome in growing dogs

This study was undertaken to investigate whether twostage segmental small bowel allotransplantation can maintain growth and development of young dogs (16 weeks, 5 to 6 kg) with surgically created short bowel syndrome (SBS). After near-total small bowel resection (group 1; n=3), irreversible weight loss was noted. After a sham operation (group 2; n=3), no growth disturbances were found. Major histocompatibility matched small bowel transplantation (SBT) with cyclosporine A as immunosuppressant, was performed in two stages (group 3; n=7). During the first stage, one meter of jejunoileum from an adult donor was placed as a Roux loop. Four weeks later, the native small bowel was removed and replaced by the graft. Only one dog survived long-term; the dogs died from infectious complications. The addition of selective decontamination of the digestive tract and early gastrostomy feeding (group 4; n=10) resulted in long-term survival in 60%. Follow-up at 4 months showed that their growth was about 20% compromised compared with that of the sham-operated animals. Functional analysis showed that electrolytes, urea, and D-xylose were normal, but there was an increase in the lactulose:mannitol ratio, fecal fat excretion, and postheparin diamine oxidase release. These results show that under the conditions described, segmental SBT functions sufficiently to treat SBS but does not maintain normal growth.

Enteral nutrition as primary therapy in short bowel syndrome.

The spectacular success of parenteral nutrition in supporting patients during small intestinal adaptation after massive resection, tends to obscure the prolonged periods often needed for such adaptation to take place. After neonatal small intestinal resection for example, it may take more than five years before adaptation is complete. There is therefore a strong argument for examining ways in which adaptation can be facilitated, in particular, by the addition of novel substrates to enteral feeds. Pectin is completely fermented by colonic bacteria to short chain fatty acids. In the rat, addition of pectin to enteral feeds led to a more rapid adaptive response in both the small and large intestine after massive small intestinal resection, although faecal nitrogen losses were increased. In a similar rat model, the provision of 40% of non-protein energy as short chain triglycerides facilitated the adaptive response in the jejunum, colon, and pancreas. The importance of glutamine as a metabolic substrate for the small intestine makes it another potential candidate and some, but not all animal studies, have suggested a therapeutic effect: increasing the glutamine content of feeds to 25% of total amino acids produced enhanced jejunal and ileal hyperplasia, even on a hypocaloric feed, and an improved overall weight gain. Studies in humans are very limited, but such promising results in the experimental animal suggest that this is probably a fruitful area for further study.