Cases reported "Short Bowel Syndrome"

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1/224. Favorable nutritional outcome after isolated liver transplantation for liver failure in a child with short bowel syndrome.

    A patient with short gut syndrome presented with end-stage liver disease. She underwent an isolated liver transplantation at the age of 3.5 years. parenteral nutrition was discontinued 1.5 years after surgery. At 7 years of age, the patient has maintained normal nutritional indices and growth while on a normal oral diet. This observation suggests that liver transplantation alone can be a valuable alternative to the combined small bowel/liver transplantation in short bowel syndrome when intestinal adaptation is expected and if the ileocecal valve is present and that improvement of gut function can occur after successful liver transplantation. ( info)

2/224. Living-related small bowel transplantation: the first case in japan.

    Intestinal failure has been managed with total parenteral nutrition (TPN), but occasionally complications such as obliteration of venous access or liver dysfunction occur. To overcome such complications, small bowel transplantation (SBT) was introduced. Since the introduction of tacrolimus in 1990, successful SBT cases have been reported. We performed SBT by using a living donor for a child with short bowel syndrome. The recipient (2.5 years old, male) was born with intestinal necrosis secondary to midgut volvulus. The length of the remaining small bowel was 30 cm. While being managed with TPN, his venous access gradually obliterated. Long-term survival could not be expected because of the difficulty in securing TPN access. The donor was his mother, whose distal ileum (100 cm) was used as a graft. The immunosuppression regimen consisted of tacrolimus, steroids and azathioprine. Three episodes of severe rejection and subsequent episodes of viral (EBV, CMV) infection were managed with steroid pulse therapy and antiviral drugs, respectively. The recipient suffered from anastomotic stenosis, and an operation was performed 13 months after transplantation to resect the stenotic segment. However, the patient died of pneumocystis carinii pneumonia 16 months after transplantation. We conclude that organ retrieval from a living donor can be performed safely for SBT, but further study of the management of rejection as well as of viral infection is necessary, as it is for non-living-related SBT. ( info)

3/224. Home parenteral nutrition for the patient with bowel syndrome and ostomy.

    For patients who have lost all function of their gastrointestinal tract, home parenteral nutrition represents not just enormous costsavings over inpatient parenteral nutrition, it also allows patients to resume many of the routines of their daily life, at home. ( info)

4/224. The first case report of the use of a zoom videoendoscope for the evaluation of small bowel graft mucosa in a human after intestinal transplantation.

    BACKGROUND: Control of allograft rejection remains the most difficult dilemma in intestinal transplantation. Standard endoscopic surveillance to date has not been always accurate in the diagnosis of rejection. We describe the first application of a zoom video endoscope in monitoring graft mucosa in humans after intestinal transplantation. METHOD: A zoom video endoscope, which can magnify the image up to 100-fold, was used in this study. The patient was a 31-year-old man who received an isolated intestinal transplant. Surveillance endoscopy with the zoom video endoscope was performed through the ileostomy. Endoscopic biopsies were done at the same time. RESULTS: The zoom video endoscope showed the microscopic architecture of the graft mucosa such as villi and crypts with outstanding quality. We found that an enlargement of the crypt areas appeared to correlate with morphologic changes of early rejection. This finding was reversed with the treatment of rejection. CONCLUSIONS: The zoom video endoscope successfully showed the detailed information of intestinal mucosa. The ability to visualize a more representative view of the graft mucosa could lead to better detection of early rejection. A greater experience with this unique method will provide more accurate assessment of the intestinal allograft. ( info)

5/224. Mucous fistula refeeding in neonates with short bowel syndrome.

    BACKGROUND/PURPOSE: Neonates with enterostomies commonly suffer from a functional short bowel syndrome (SBS) and have a greater risk of electrolyte and fluid loss with poor weight gain. The authors describe their experience with refeeding stoma effluent into the mucous fistula in neonates. methods: A 5-year (1993 to 1997) chart review of neonates with stoma effluent refeeding was undertaken. Demographics, medical history, surgical procedures, timing, and duration of refeedings were reviewed. Enteral and total parenteral nutritional (TPN) requirements, electrolyte, and acid-base disturbances were recorded. RESULTS: Six neonates (gestational ages of 27 to 38 weeks, birth weights of 533 to 3400 g) were identified with nutritional or electrolyte complications before the commencement of refeeding. enterostomy indications included necrotizing enterocolitis (n = 2), intestinal atresia type 3b (n = 1), complications from ruptured omphalocoele (n = 1), congenital adhesive band obstruction (n = 1), and midgut volvulus after congenital diaphragmatic hernia repair (n = 1). weight gain during refeeding ranged from 5 to 25 g/kg/d with duration of refeeding lasting 16 to 169 days (two neonates were refed at home) until reanastomoses were done 6 to 44 weeks after the original surgery. There were no complications, and TPN requirements were diminished or eliminated. CONCLUSION: This technique represents a simple and safe method, which lessens the need for TPN and electrolyte supplementation in neonates with enterostomies and SBS before reanastomosis. ( info)

6/224. Liquid pancreatic enzyme therapy for a patient with short bowel syndrome and chronic pancreatitis in a complicated case of Crohn's disease.

    The case of a 45 year old female with multiple complications of Crohn's disease is reported. After multiple resections in the gastrointestinal tract she had been suffering from short bowel syndrome and severe malnutrition. With a special continuous gastric tube feeding system, she was able to maintain her weight for years. In the beginning of 1997 the enteral nutrition was not longer tolerated for an exacerbation of chronic pancreatitis. There was a weight loss, permanent pain and total parenteral nutrition had to be performed. In this situation a new liquid preparation of pancreatic enzymes which had been tested in the laboratory before, was used for continuous enzyme replacement via gastric tube. In combination with this enzyme preparation, enteral nutrition could successfully be started again. ( info)

7/224. D-lactic acidosis in short bowel syndrome.

    short bowel syndrome can present many complex management issues and may be complicated by various metabolic problems. D-lactic acidosis in the setting of short bowel syndrome has been described only rarely in children. A further case is presented with a review of typical clinical manifestations of D-lactic acidosis and reported management options. Early recognition and appropriate management is essential to avoid morbidity secondary to this complication of short bowel syndrome. Probiotic therapies may have an increasing role in prevention and management of this complication. ( info)

8/224. Clinical use of growth hormone and glutamine in short bowel syndrome.

    growth hormone (GH) and glutamine (GLN) are considered bowel trophic factors and are used experimentally after bowel resection. Their clinical uses in short bowel syndrome (SBS) are still not standardized. It is of interest to verify metabolic, nutritional and side effects of the association of GH and GLN in SBS. Three patients, 39 (A), 33 (B), and 01 years old (C) underwent bowel resection with jejunum anastomosis 15 cm (A) and 60 cm (B) distant from the Treitz angle, and 40 cm (C) preserving the ileo cecal valve. GH Saizen (Serono-A), Genotropin (Pharmacia-B), and Norditropin (Novonordisk C) were administered in doses of 0.14 mg/kg/day. GLN (0.4 g/kg/day) was given orally for 10 days (A), 30 days (B) and 60 days to patient C (0.28 g/kg/day). Central TPN and adequate oral diet was administered according to the bowel adaptation phase. On the first day after beginning treatment patient A exhibited symptoms of hypoglycemia. There were no other side effects. After treatment, body weight was higher and analysis by bioelectrical impedance showed more lean mass and less fat mass compared to pre-treatment measurements. nitrogen retention was progressively higher with treatment. Simultaneous treatment with GH and GLN does not cause significant side effects, and is associated with a favorable distribution of the body compartments and nitrogen retention in patients with the short bowel syndrome. ( info)

9/224. Intestinal lengthening and growth hormone in extreme short bowel syndrome: a case report.

    Recombinant growth hormone (GH) has been used successfully to promote mucosal intestinal adaptation in adults with short bowel syndrome. Here the authors report the case of an infant with extreme short bowel syndrome successfully treated with intestinal lengthening and GH. The patient was born with intestinal atresia leaving only 12 cm of jejunum and 2 cm of ileum with ileocecal valve. A Bianchi procedure extended the length to 25 cm, but 6 months later she still received 50% of her nutritional requirements by parenteral route. A 4-week course of 0.5 U/kg of GH dramatically changed the situation allowing weaning of total parenteral nutrition (TPN) 2 weeks after the end of the treatment with striking nutritional improvement, decrease in the number of stools from 7 to 2 per day, and no secondary effects. The authors believe that GH deserves clinical trial in cases of extreme short bowel. ( info)

10/224. Progressive perinatal bowel obstruction--a rare cause of short-bowel syndrome.

    A girl was born after an uneventful pregnancy of 36 weeks. Prenatally, distended bowel loops had been seen on ultrasound. Multiple small-bowel atresia was diagnosed and treated surgically. In the course of the next eleven weeks, previously patent segments of small bowel became obstructed. In 4 separate operative sessions, several segments of jejunum and ileum were resected, leaving 23 cm of ileum with the ileocecal valve in place. On microscopic examination of all resected material, necrosis of the mucosa was found consistent with ischemia. The child survived and tolerated full enteral feeding at the age of 8.5 months. The origin of the progressive obliterating process remains unknown. ( info)
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