scholarly journals Short bowel syndrome in infancy: recent advances and practical management

2020 ◽  
pp. flgastro-2020-101457
Author(s):  
Elena Cernat ◽  
Chloe Corlett ◽  
Natalia Iglesias ◽  
Nkem Onyeador ◽  
Julie Steele ◽  
...  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Intestinal Failure ◽  
Rare Condition ◽  
Oral Feeding ◽  
Short Bowel ◽  
Intestinal Continuity ◽  
Gastrointestinal Reconstruction ◽  
Lipid Reduction

Short bowel syndrome (SBS) is a rare condition characterised by extensive loss of intestinal mass secondary to congenital or acquired disease. The outcomes are determined by dependency on parenteral nutrition (PN), its possible complications and factors that influence intestinal adaptation. In order to achieve the best results, patients should be managed by a specialised multidisciplinary team with the aims of promoting growth and development, stimulating intestinal adaptation and preventing possible complications. This involves timely surgical management aimed at rescuing maximum bowel length and eventually re-establishing intestinal continuity where appropriate. A combination of enteral and parenteral nutrition needs to be targeted towards maintaining a balance between fulfilling the nutritional and metabolic needs of the child while preventing or at least minimising potential complications. Enteral nutrition and establishment of oral feeding play a fundamental role in stimulating bowel adaptation and promoting enteral autonomy. Other measures to promote enteral autonomy include the chyme recycling in patients where bowel is not in continuity, autologous gastrointestinal reconstruction and pharmacological treatments, including promising new therapies like teduglutide. Strategies such as lipid reduction, changing the type of lipid emulsion and cycling PN are associated with a reduction in the rates of intestinal failure–associated liver disease. Even though vast improvements have been made in the surgical and medical management of SBS, there is still lack of consensus in many aspects and collaboration is essential.

scholarly journals Congenital Short Bowel Syndrome: A Case Report and Review of the Literature

2008 ◽  
Vol 22 (1) ◽  
pp. 71-74 ◽  
Author(s):  
Mohammed Hasosah ◽  
Daniel A Lemberg ◽  
Eric Skarsgard ◽  
Richard Schreiber
Keyword(s):  
Small Bowel ◽  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Rare Condition ◽  
Normal Growth ◽  
Upper Gastrointestinal Endoscopy ◽  
Term Survival ◽  
Short Bowel ◽  
Upper Gastrointestinal

Congenital short bowel syndrome (SBS) is a rare condition of the newborn, with several reports demonstrating high mortality. A six-week-old boy presented with chronic diarrhea and failure to thrive. An upper gastrointestinal endoscopy showed a straight duodenum, and multiple small bowel biopsies were histologically normal. An upper gastrointestinal series showed malrotation. At laparotomy, the small bowel was 50 cm in length, confirming the diagnosis of congenital SBS. Parenteral nutrition was initiated and enteral feeding with an amino acid-based formula containing long-chain fatty acids was introduced early and gradually advanced. At the last follow-up examination at 24 months, he was thriving on a regular diet, with normal growth and development. Long-term survival of children with congenital SBS is now possible if enteral feeds are introduced early to promote intestinal adaptation, with subsequent weaning off parenteral nutrition.

scholarly journals P582 Insights on the sphingolipid profile in patients with short bowel syndrome and consecutive intestinal failure

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S536-S536
Author(s):  
A Kubesch ◽  
F Knauer ◽  
D Thomas ◽  
K Jakobi ◽  
N Filmann ◽  
...  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Failure ◽  
Rare Condition ◽  
University Hospital ◽  
Short Bowel ◽  
Protein Levels ◽  
Sphingosine 1 Phosphate ◽  
Male Patients ◽  
The University

Abstract Background Short bowel syndrome (SBS) is a rare condition, and providing care for these patients can be challenging. Investigating the serologic sphingolipid profile has enhanced the understanding of various pathophysiologic conditions. However, data on the serologic sphingolipid profile in patients with SBS are –to date- not available. Methods Serum probes from patients with SBS treated in the outpatient clinic of the Department of Gastroenterology and Hepatology of the University Hospital Frankfurt were included and analyzed retrospectively. Serologic sphingolipid (SL) and especially ceramide (Cer) levels were assessed by LC-MS/MS and correlated with clinical and laboratory chemical parameters. Results A total of 37 (16 male) patients with SBS were included, 11 with Crohn’s disease (CD) (29.73%). Patients’ median age was 62 (24–88), with a median weight of 59 kilograms (42–75). Patients with less than 100 centimeters of small intestine had significantly higher creatinine and lower total protein levels, whereas the serologic SL-profile was not significantly affected. Furthermore, Sphingosine-1-Phosphate (S1P) and C18-Cer were increased in patients with SBS with CD as the underlying etiology. No significant differences in the sphingolipidomic profile were observed between patients on total parenteral nutrition than patients with oral and parenteral nutrition and patients with associated elevation of the liver enzymes. Conclusion Conclusion: Our study provides first insights into the serologic SL profile of patients with SBS. We showed that patients with CD present with an altered SL profile compared to other aetiologies, while the occurrence of liver disease in this single-center cohort does not appear to affect the serologic SL profile in respective patients.

scholarly journals Serial transverse enteroplasty and nipple valve construction, two life saving techniques for patients with short bowel syndrome, a report of 5 cases

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Mojtaba Shafiekhani ◽  
Nazanin Azadeh ◽  
Kiarash Ashrafzadeh ◽  
Maryam Esmaeili ◽  
Hamed Nikoupour
Keyword(s):  
Small Intestine ◽  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Ileocecal Valve ◽  
Intestinal Transit Time ◽  
Short Bowel ◽  
Nipple Valve ◽  
Gastrointestinal Reconstruction ◽  
Serial Transverse Enteroplasty

Abstract Background Various abdominal pathologies end up with surgical resection of small intestine. When the small intestine remnant is too short for adequate fluid and micronutrients absorption, short bowel syndrome is diagnosed. The disabling condition needs a multidisciplinary approach to design parenteral nutrition, care for thrombotic, hepatic and infectious complications and gradually wean the patient from parenteral nutrition. Various surgical techniques have been introduced to increase absorptive mucosa and enhance the intestinal adaptation process. Serial transverse enteroplasty and nipple valve reconstruction are among the procedures, which will be discussed in the current article. Case presentation Herein, we presented 5 cases of short bowel syndrome as a consequence of abdominal laparotomies, patients were referred to our center to receive parenteral nutrition and to be prepared for the final autologous gastrointestinal reconstruction or intestinal transplantation, if indicated. Conclusion Patient’s age, performance status and bowel remnant length determines the appropriate technique for autologous gastrointestinal reconstruction. Serial transverse enteroplasty is designed to increase bowel’s length by creating zigzag patterns through dilated bowel loops. Presence of ileocecal valve is crucial to delay intestinal transit time and to prevent colonic bacterial transfer to ileum. Patient’s with ileocecal valve loss benefit from creating an artificial valve, namely, nipple valve.

Short Bowel Syndrome: A Paradigm for Intestinal Adaptation to Nutrition?

2020 ◽  
Vol 40 (1) ◽  
pp. 299-321 ◽  
Author(s):  
Johanne Le Beyec ◽  
Lore Billiauws ◽  
André Bado ◽  
Francisca Joly ◽  
Maude Le Gall
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Metabolic Diseases ◽  
Current Knowledge ◽  
Intestinal Adaptation ◽  
Intestinal Failure ◽  
Extensive Resection ◽  
Oral Nutrition ◽  
Short Bowel ◽  
Gut Hormone

Short bowel syndrome (SBS) is a rare disease that results from extensive resection of the intestine. When the remaining absorption surface of the intestine cannot absorb enough macronutrients, micronutrients, and water, SBS results in intestinal failure (IF). Patients with SBS who suffer from IF require parenteral nutrition for survival, but long-term parenteral nutrition may lead to complications such as catheter sepsis and metabolic diseases. Spontaneous intestinal adaptation occurs weeks to months after resection, resulting in hyperplasia of the remnant gut, modification of gut hormone levels, dysbiosis, and hyperphagia. Oral nutrition and presence of the colon are two major positive drivers for this adaptation. This review aims to summarize the current knowledge of the mechanisms underlying spontaneous intestinal adaptation, particularly in response to modifications of luminal content, including nutrients. In the future, dietary manipulations could be used to treat SBS.

scholarly journals Early effectivity of teduglutide for the treatment of short bowel sydrome – our first clinical experience

2021 ◽  
pp. 230
Author(s):  
◽  
◽  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Treatment Effectiveness ◽  
Intestinal Adaptation ◽  
Standard Of Care ◽  
Home Parenteral Nutrition ◽  
Short Bowel ◽  
Post Surgery ◽  
Advanced Therapy ◽  
New Treatment

Background Short bowel syndrome is an uncommon malabsorptive condition most frequently caused by massive surgical resection of the small intestine. Standard of care is home parenteral nutrition. Teduglutide, glucagon like peptide 2 analog, was approved to treat patients with short bowel syndrome, who are stable following a period of post-surgery intestinal adaptation. Case presentation We report two cases of patients with short bowel syndrome on home parenteral nutrition, treated with teduglutide, novel advanced therapy. We monitored the effectiveness and tolerance of the new treatment. Conclusion Teduglutide increases intestinal absorption, causes cryptal hyperplasia, villous hypertrophy, angiogenesis, and allows weaning from parenteral nutrition. We confirmed the early onset of treatment effectiveness in the 4th month. Early effectiveness of treatment allowed a reduction in the volume of home parenteral nutrition with an improvement in quality of life.

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