Short bowel syndrome (SBS) is a severely disabling and potentially life-threatening condition. Survival data for patients with SBS are limited. This study aimed to investigate prognostic factors in patients with SBS undergoing surgery. We reviewed the medical records of 27 consecutive patients with SBS who were treated at our hospital between January 2018 and December 2022. SBS was defined as a remaining small bowel length <200 cm, excluding patients with Crohn disease. Of the 27 patients identified, 17 were males and 10 were females, with a median age of 77 (46-90) years and a total observation time of 137 (2-1628) days. All patients underwent surgery and received parenteral nutrition (PN) and follow-up in our hospital. Superior mesenteric artery stenosis (44.4%) and nonocclusive mesenteric ischemia (25.9%) most commonly caused SBS. The median residual small bowel length and postoperative hospital stay were 50 (5-150) cm and 48 (2-104) days, respectively. Jejunostomy was performed in 17 (62.9%) patients, and 4 (14.8%) patients were weaned off their PN. Death occurred in 14 (51.8%), and the median survival time was 209 days. The survival outcome was compared between the survival (n = 13) and the death groups (n = 14). Jejunostomy and PN rates were significantly higher in the death group (P < .01, P = .03, respectively). SBS is associated with significantly higher mortality rates. Jejunostomy and long PN duration are significantly associated with death in patients with SBS.

BACKGROUND: Short bowel syndrome (SBS) is the predominant cause of paediatric intestinal failure. Although life-saving, parenteral nutrition (PN) is linked to complications and may impact quality of life (QoL). Most children will experience intestinal rehabilitation (IR), but the mechanisms underpinning this remain to be understood. SBS is characterised by abnormal microbiome patterns, which might serve as predictive indicators for IR. We aim to characterise the microbiome profiles of children with SBS during IR, concurrently exploring how parental perspectives of QoL relate to IR.
METHODS: This study will enrol a minimum of 20 paediatric patients with SBS (0-18 years). Clinical data and biological samples will be collected over a 2-year study period. We will apply 16S rRNA gene sequencing to analyse the microbiome from faecal and gut tissue samples, with additional shotgun metagenomic sequencing specifically on samples obtained around the time of IR. Gas chromatography with flame ionisation detection will profile faecal short-chain fatty acids. Plasma citrulline and urinary intestinal fatty acid binding proteins will be measured annually. We will explore microbiome-clinical covariate interactions. Furthermore, we plan to assess parental perspectives on QoL during PN and post-IR by inviting parents to complete the Paediatric Quality of Life questionnaire at recruitment and after the completion of IR.
BACKGROUND: Ethical approval was obtained from the East Midlands-Nottingham 2 Research Ethics Committee (22/EM/0233; 28 November 2022). Recruitment began in February 2023. Outcomes of the study will be published in peer-reviewed scientific journals and presented at scientific meetings. A lay summary of the results will be made available to participants and the public.
BACKGROUND: ISRCTN90620576.

BACKGROUND: Pediatric patients with intestinal failure require long-term parenteral nutrition owing to impaired enteral nutrition absorption. A potential complication is essential fatty acid deficiency (EFAD), resulting from decreased linoleic and α-linolenic acid concentrations and defined by an increased triene:tetraene ratio (TTR; Mead acid:arachidonic acid). Historically, soybean oil lipid emulsion (SOLE) was the only commercially available parenteral lipid in the United States. Recently, a composite lipid emulsion (CLE) and fish oil lipid emulsion (FOLE) received US Food and Drug Administration approval. This study investigated whether lipid emulsion regimen impacts EFAD incidence in pediatric patients with intestinal failure.
METHODS: This study was a 10-year retrospective cohort study of pediatric patients with intestinal failure who received parenteral SOLE, CLE, or FOLE. The primary outcome was EFAD incidence, defined as a TTR ≥ 0.2. Secondary outcomes included TTR ≥ 0.05, cholestasis incidence, lipid dose effect on EFAD incidence, and fatty acid parameter differences.
RESULTS: A total of 144 fatty acid profiles from 47 patients were reviewed. EFAD did not occur in any lipid emulsion group. There were no differences in the incidence of TTR ≥ 0.05 or cholestasis. The effect of dose could not be evaluated because of no EFAD incidence. Lastly, although each group had varied fatty acid parameters, none saw decreased essential fatty acid levels.
CONCLUSIONS: This study found that, with close monitoring, the lipid emulsion regimen did not impact EFAD incidence. This suggests that FOLE and CLE do not increase EFAD risk compared with SOLE in pediatric patients with intestinal failure.

BACKGROUND: Low neighborhood income is linked with increased hospitalizations for central line-associated bloodstream infections (CLABSIs) in pediatric short bowel syndrome (SBS). We assessed whether this relationship varies by hospital center.
METHODS: We performed a retrospective cohort study using the Pediatric Health Information System (2018-2023) database for patients <18 years old with SBS (N = 1210) at 24 hospitals in the United States. Using 2015 US Census data, we determined the estimated median household income of each patient\'s zip code. Hospital-level neighborhood income was defined as the median of the estimated median household income among patients at each hospital. We applied an extension of Cox regression to assess risk for CLABSI hospitalization.
RESULTS: Among 1210 children with 5255 hospitalizations, most were <1 year on initial admission (53%), male (58%), and publicly insured (69%). Hospitals serving low-income neighborhoods served more female (46% vs 39%), Black (29% vs 22%), and Hispanic (22% vs 16%) patients with public insurance (72% vs 65%) residing in the southern United States (47% vs 21%). In univariate analysis, low hospital-level neighborhood income was associated with increased risk of CLABSI hospitalization (rate ratio [RR], 1.48; 95% CI, 1.21-1.83; P < 0.001). These findings persisted in multivariate analysis (RR, 1.43; 95% CI, 1.10-1.84; P < 0.01) after adjusting for race, ethnicity, insurance, region, and patient-level neighborhood income.
CONCLUSIONS: Hospitals serving predominantly low-income neighborhoods bear a heavier burden of CLABSI hospitalizations for all their patients across the socioeconomic spectrum. Hospital initiatives focused on CLABSI prevention may be pivotal in addressing this disparity.

BACKGROUND: Home parenteral nutrition (HPN) is often cycled nocturnally and is expected to result in glucose intolerance and sleep disruption partly due to circadian misalignment. This study aimed to define the metabolic response when HPN is cycled during the daytime compared to overnight.
METHODS: This secondary analysis leveraged samples from a clinical trial in adults with short bowel syndrome consuming HPN (ClinicalTrials.gov: NCT04743960). Enrolled patients received 1 week of HPN overnight followed by 1 week of HPN during the daytime. Fasting blood samples were collected following each study period and global metabolic profiles were examined from plasma samples. Differential metabolite abundance was determined from normalized and scaled data using adjusted Linear Models for MicroArray Data models followed by pathway enrichment analysis.
RESULTS: Nine patients (mean age, 52.6 years; 78% female; mean BMI 20.7 kg/m2) provided samples. Among 622 identified metabolites, changes were observed in 36 metabolites at Punadj < 0.05 with higher abundance of fatty acids, long-chain and polyunsaturated fatty acids (Dihomo-gamma-linolenic acid, arachidonate (20:4n6), docosahexaenoate (DHA; 22:6n3)) and glycerolipids with daytime infusions. Enrichment analysis identified changes in pathways related to the biosynthesis of unsaturated fatty acids, d-arginine, and d-ornithine metabolism, and linoleic acid metabolism (Punadj<0.05).
CONCLUSIONS: Daytime infusions of HPN may result in changes in circulating lipids and amino acid composing metabolic pathways previously implicated in circadian rhythms. As this is the first untargeted metabolomics study of HPN, larger studies are needed.

OBJECTIVE: To report the results and successes of intestinal transplantation (ITx) in the most active European centres, to emphasize that, although it is a difficult procedure, it should remain a therapeutic option for children with total, definitive and complicated intestinal failure when intestinal rehabilitation fails.
METHODS: We retrospectively collected data about all patients less than 18 receiving an ITx from 2010 to 2022 in 8 centres, and outcomes in July 2022.
RESULTS: ITx was performed in 155 patients, median age 6.9 years, in 45% for short bowel syndromes, 22% congenital enteropathies, 25% motility disorders, and 15% re-transplantations. Indications were multiple in most patients, intestinal failure-associated liver disease in half. The graft was in 70% liver-containing. At last follow up 64% were alive, weaned from parenteral nutrition, for 7.9 years; 27% had died and the graft was removed in 8%, mostly early after ITx.
CONCLUSIONS: ITx, despite its difficulties, can give a future to children with complicated intestinal failure. It should be considered among the therapeutic options offered to patients with a predicted survival rate lower than that after ITx. Patients should be early discussed within multidisciplinary teams in ITx centres, to avoid severe complications impacting the results of ITx, or even to avoid ITx.

BACKGROUND: Malrotation and volvulus classically present with bilious vomiting. It is more common earlier in life, but there are other causes of bile-stained vomiting. This leads some clinicians to \'watch and wait\'. In the presence of a volvulus, this is potentially a fatal decision. It is not clear from the literature if there is a safe time window in which children can be observed in the hope of avoiding transfers or radiological investigations.
OBJECTIVE: To determine whether time to identification and management of midgut volvulus correlated with morbidity and mortality; and whether there were patterns to transition of care.
METHODS: Multicentre, retrospective review of all children with malrotation ± volvulus at two tertiary children\'s hospitals in Brisbane from 2000 to 2012. Data collected included age at presentation, timing between symptom onset and presentation, radiological findings, and definitive surgical management. Outcomes included patient length of stay (LOS), total parenteral nutrition (TPN) duration, re-operations and death.
RESULTS: There were 96 cases of malrotation identified, with 23 excluded (elective operation, insufficient data). Neonates made up 66% of included cases. Only 14% of cases were over 12 months old. Bilious vomiting or bile-stained aspirates were the presenting symptoms in 71% (52). Overall mortality was 5.56%. Time from symptom onset to presentation or management was not significantly associated with morbidity or mortality. More than half (53%, 39/73) of patients received total parenteral nutrition; 20/39 for more than 10 days. Neonates and infants had a significantly higher rate of TPN compared with older children (P < 0.001). Those requiring TPN post-operatively had a significantly higher mortality compared with those who did not (P = 0.02). Time from symptom onset to presentation or definitive management was not significantly associated with LOS, TPN duration, or need for re-operation.
CONCLUSIONS: Malrotation remains a time-critical diagnosis to secure and treat. Even a short duration of symptoms can be associated with high morbidity or mortality. There is no place for \'watch and wait\' for such patients, and malrotation/volvulus should be emergently actively excluded with contrast studies.

Teduglutide is a glucagon-like-peptide-2 analogue that reduces the need for parenteral support in patients with short bowel syndrome (SBS). Nevertheless, data about long-term therapy with teduglutide in children are still scarce. Our objective was to describe the real-life experience with teduglutide in children with SBS over the last 5 years in Spain. This was a national multicentre and prospective study of paediatric patients with intestinal failure (IF) treated with teduglutide for at least 3 months. The data included demographic characteristics, medical background, anthropometric data, laboratory assessments, adverse events, and parenteral nutrition (PN) requirements. Treatment response was defined as a > 20% reduction in the PN requirement. The data were collected from the Research Electronic Data Capture (REDCap) database. Thirty-one patients from seven centres were included; the median age at the beginning of the treatment was 2.3 (interquartile range (IQR) 1.4-4.4) years; and 65% of the patients were males. The most frequent cause of IF was SBS (94%). The most common cause of SBS was necrotizing enterocolitis (35%). The median residual bowel length was 29 (IQR 12-40) cm. The median duration of teduglutide therapy was 19 (IQR 12-36) months, with 23 patients (74%) treated for > 1 year and 9 treated for > 3 years. The response to treatment was analysed in 30 patients. Twenty-four patients (80%) had a reduction in their weekly PN energy > 20% and 23 patients (77%) had a reduction in their weekly PN volume > 20%. Among the responders, 9 patients (29%) were weaned off PN, with a median treatment duration of 6 (IQR 4.5-22) months. The only statistically significant finding demonstrated an association between a > 20% reduction in the weekly PN volume and a younger age at the start of treatment (p = 0.028).   Conclusions: Teduglutide seems to be an effective and safe treatment for paediatric patients with IF. Some patients require a prolonged duration of treatment to achieve enteral autonomy. Starting treatment with teduglutide at a young age is associated with a higher response rate. What is Known: •  Glucagon-like peptide-2 (GLP-2) plays a crucial role in the regulation of intestinal adaptation in short bowel syndrome (SBS). Teduglutide is a GLP-2 analog that reduces the need for parenteral support in patients with SBS. • Data about long-term therapy with teduglutide in children in real life are still scarce. What is New: • Most pediatric patients with SBS respond in a satisfactory manner to teduglutide treatment. The occurrence of long-term adverse effects is exceptional. • Starting treatment with the drug at a young age is associated with a greater response rate.

OBJECTIVE: By selectively perfusing the first three jejunal arteries (JA), we aim to assess the individual perfusion length of small bowel (SB) and its impact on nodal resection in stage III-up small-intestinal neuroendocrine tumors (SI-NET).
METHODS: Our anatomical research protocol implies a midline laparotomy and three measures of the SB length. We then perform a classical anterior approach of the superior mesenteric vessels. We carry on with the complete dissection and checking of the superior mesenteric artery (SMA) in order to identify the first three JA. Then we selectively perfuse each artery with colored latex solutions and measure the length of small bowel perfused respectively.
RESULTS: We conducted our protocol on six cadaveric subjects. Mean(SD) SB length was 413(5.7), 535(13.2), 485(15), 353(25.1), 730(17.3) and 525(16° cm respectively from subject one to six. Most JA originated from the left side of the SMA. The first JA originated from its posterior wall in two subjects. Mean(SD) distance of origin of the first three JA was 4.6(1.3)cm, 6(1.1)cm and 7.1(0.9)cm respectively. Mean(SD) diameter of SMA was 10.8(3.3)mm. Mean diameter of the three first JA was 4(1.4)mm, 4(1.5)mm and 5(1.2)mm respectively. Mean(SD) SB length perfused by first and second JA was 224(14.9)cm, 175(8.6)cm, 238.3(7.6)cm, 84.3(5.1)cm, 233.3(5.8)cm and 218.3(10.4)cm respectively from subject one to six.
CONCLUSIONS: We observed a trend suggesting that the first and second JA may sustain a SB length beyond the viable 1.5 m limit, implying the feasibility of stage III-up SI-NET resection with just two JA.

(1) Background: We present the first real-world-data study on teduglutide-treated SBS patients in the Slovak Republic and the first study to enable the comparison of the effects of teduglutide treatment between the adult and pediatric populations. (2) Methods: This was a non-interventional retrospective cohort study of adult and pediatric SBS patients treated with teduglutide. Primary and secondary endpoints were the results of teduglutide use at 12 weeks and 6 months after the initiation of treatment, compared to baseline. (3) Results: Teduglutide treatment led to a statistically significant reduction in the volume of intravenous hydration, HPN caloric intake, HPN and intravenous hydration applications per week and to increased urine output in adult patients. The results in the pediatric population were similar, but not statistically significant. A complete weaning off HPN was achieved in 57.14% of all patients (50.00% of children; 62.50% of adults) after a median of 0.99 years of teduglutide treatment (1.07 and 0.98 years for children and adults, respectively). (4) Conclusions: Teduglutide treatment in SBS patients leads to considerable reduction in or even weaning off PN in both pediatric and adult patients.