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 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 Hypomagnesemia in short bowel syndrome patients

2000 ◽  
Vol 118 (6) ◽  
pp. 169-172 ◽  
Author(s):  
Simone Chaves Miranda ◽  
Michelle Lizzy Bandeira Ribeiro ◽  
Eduardo Ferriolli ◽  
Júlio Sérgio Marchini
Keyword(s):  
Small Bowel ◽  
Short Bowel Syndrome ◽  
Bowel Resection ◽  
Serum Magnesium ◽  
Small Bowel Resection ◽  
University Hospital ◽  
Short Bowel ◽  
Metabolic Unit ◽  
The University

CONTEXT: Magnesium support to small bowel resection patients. OBJECTIVE: Incidence and treatment of hypomagnesemia in patients with extensive small bowel resection. DESIGN: Retrospective study. SETTING: Metabolic Unit of the University Hospital Medical School of Ribeirão Preto, University of São Paulo, Brazil. PATIENTS: Fifteen patients with extensive small bowel resection who developed short bowel syndrome. MAIN MEASUREMENTS: Serum magnesium control of patients with bowel resection. Replacement of magnesium when low values were found. RESULTS: Initial serum magnesium values were obtained 21 to 180 days after surgery. Hypomagnesemia [serum magnesium below 1.5 mEq/l (SD 0.43)] was detected in 40% of the patients [1,19 mEq/l (SD 0.22)]. During the follow-up period, 66% of the patients presented at least two values below reference (1.50 mEq/l). 40% increased their serum values after magnesium therapy. CONCLUSION: Metabolic control of serum magnesium should be followed up after extensive small bowel resection. Hypomagnesemia may be found and should be controlled.

scholarly journals Managing Successful Pregnancies in Patients with Chronic Intestinal Failure on Home Parenteral Nutrition: Experience from a UK National Intestinal Failure Unit

2017 ◽  
Vol 26 (4) ◽  
pp. 375-379
Author(s):  
Ashley Bond ◽  
Dipesh H. Vasant ◽  
Wadiamu Gashau ◽  
Arun Abraham ◽  
Antje Teubner ◽  
...  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Failure ◽  
Home Parenteral Nutrition ◽  
The Other ◽  
Short Bowel ◽  
Child Bearing ◽  
Spontaneous Labour ◽  
Daily Allowance ◽  
Related Line

Background & Aim: There are minimal reports of pregnancy in the chronic intestinal failure (CIF)/ home parenteral nutrition (HPN) population, with some concern relating to the risk associated with such pregnancies. The aim of this retrospective observational study was to evaluate pregnancy experience and outcomes in patients with CIF requiring HPN in a national UK CIF referral centre.Method: All known pregnancies in patients treated with HPN for CIF between 1982 and 2016 were identified retrospectively from a prospectively maintained database. Maternal and foetal outcomes were collated, along with PN requirements before and during pregnancy.Results: There were 5 pregnancies in 5 mothers in our cohort consisting of over 700 patients, males and females. The median age at conception was 28 years (range 24-33 years). The median duration on HPN prior to pregnancy was 26 months (range 4-85 months). One patient had Crohn’s disease, another had pan-enteric dysmotility and the other three patients had ischaemic complications leading to short bowel syndrome (SBS). All mothers were intensively monitored in an obstetric clinic and a dedicated CIF multi-disciplinary clinic throughout pregnancy. There were 2/5 preterm deliveries. One of the preterm deliveries was associated with spontaneous labour and the other had a planned induction as a result of maternal clinical need. Neonatal complications were seen in both prenates. There were no recorded maternal catheter related line infections during the 5 pregnancies.Conclusion: Successful pregnancy whilst receiving HPN is feasible but requires close, intensive monitoring and dedicated optimisation of PN in order to minimise maternal and foetal complications. All women of child-bearing age on HPN should be counselled about pregnancy.Abbreviations: ALP: alkaline phosphatase; ALT: alanine transaminase; CIF: chronic intestinal failure; HPN: home parenteral nutrition; IBD: inflammatory bowel disease; IF: intestinal failure; IFALD: Intestinal Failure associated liver disease; LFT: liver function test; PN: parenteral nutrition; RDA: Recommended daily allowance; SBS: short bowel syndrome; TPN: total parenteral nutrition.

Neonatal and Pediatric Parenteral Nutrition

2012 ◽  
Vol 23 (4) ◽  
pp. 451-464 ◽  
Author(s):  
Anne Gargasz
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Pediatric Patients ◽  
Intestinal Failure ◽  
Nutritional Requirements ◽  
Gastrointestinal Fistula ◽  
Short Bowel ◽  
Therapeutic Modalities ◽  
The 1960S

Parenteral nutrition is one of the most important therapeutic modalities invented in the last several decades. Since its introduction in the 1960s, this modality has saved thousands of lives by providing nutrients parenterally to sustain growth in premature neonates with severe intestinal immaturity and other pediatric patients with intestinal failure, such as a gastrointestinal fistula or short bowel syndrome. Although parenteral nutrition can be a lifesaving treatment, it is not benign. Many complications can result from either short- or long-term usage. This review discusses the nutritional requirements, common complications, medication additives, and special considerations for pediatric patients requiring parenteral nutrition.

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.

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