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1.
Pediatr Surg Int ; 40(1): 120, 2024 May 04.
Article En | MEDLINE | ID: mdl-38702423

PURPOSE: To assess the neurodevelopment outcomes of children younger than 42 months of age with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by a Pediatric Multidisciplinary Intestinal Rehabilitation Program from a public tertiary hospital in Brazil. METHODS: Bayley III scale was administered in children aged 2 to 42 months with IF and receiving PN for more than 60 days. Composite scores in cognitive, motor, and language domains were analyzed. Developmental delay was defined as a performance 2 standard deviations (SD) below the average at the 3 domains. Association between Bayley III composite scores and clinical variables related to IF were tested. RESULTS: Twenty-four children with median (IQR) age of 17.5 months (9-28.5) were studied, 58.3% were male. Developmental delay was found in 34%, 33% and 27% of the patients in cognitive, motor, and language domains, respectively. There was no significant association between the Bayley-III composite scores and length of hospitalization, prematurity, and number of surgical procedures with anesthesia. CONCLUSION: The study demonstrated impairments in the cognitive, motor and language domains in approximately one-third of young patients with IF on prolonged PN.


Intestinal Failure , Parenteral Nutrition , Humans , Male , Female , Brazil/epidemiology , Infant , Parenteral Nutrition/methods , Parenteral Nutrition/statistics & numerical data , Child, Preschool , Developmental Disabilities/etiology , Neurodevelopmental Disorders/epidemiology , Neurodevelopmental Disorders/etiology
2.
Clin Nutr ESPEN ; 61: 140-144, 2024 Jun.
Article En | MEDLINE | ID: mdl-38777425

INTRODUCTION: Patients with intestinal failure (IF) are often dependent on PN for provision of calories and nutrients for survival. Similar to chronic intestinal failure (CIF) patients, those who have AIF are also at risk of IFALD, which is a poorly understood but potentially fatal condition. The local incidence of IFALD amongst AIF patients is not known. OBJECTIVES: The primary objective of this study was to determine the incidence of IFALD in AIF patients on short-term PN. Secondary objectives were to analyse patient and PN risk factors of IFALD, and clinical outcomes of length of stay (LOS) and inpatient mortality. DESIGN: This was a retrospective cross-sectional cohort study of hospitalised adult patients with AIF prescribed with short-term PN. All adult patients aged 21 years and above who received PN for at least 5 consecutive days and had normal liver function tests (LFTs) at the time of PN initiation were included in this study. RESULTS: A total of 171 patients were enrolled in this study, with 77 (45%) having deranged LFTs at the end of PN therapy and categorised under the IFLAD group. The patient cohort was predominantly male (92 [54%]) and had a median age of 68 years (IQR 59-76). Patients with IFALD at the end of PN therapy had higher diabetes prevalence (36% vs 26%, p = 0.2) and were on PN for a longer duration (median [IQR]: 12 [8-17] vs 8 [6-15] days, p = 0.003) than those without IFALD. There were no significant differences in patient and PN characteristics between the IFLAD and non-IFALD group. The multivariable models showed that the IFALD cohort had longer hospital stays (HR 0.90, 95% CI 0.65-1.23) and lower odds of inpatient death (OR 0.75, 95% CI 0.12-4.60), though both findings are not statistically significant (p = 0.5, 0.7). CONCLUSION: In this study, IFALD is a common phenomenon in AIF and the incidence was found to be an estimated 50% amongst patients on short-term PN with similar clinical outcomes between the two groups.


Intestinal Failure , Length of Stay , Liver Diseases , Parenteral Nutrition , Humans , Male , Female , Retrospective Studies , Cross-Sectional Studies , Middle Aged , Aged , Liver Diseases/mortality , Liver Diseases/epidemiology , Risk Factors , Intestinal Failure/therapy , Incidence , Hospital Mortality , Adult , Liver Function Tests
3.
Gastroenterol Clin North Am ; 53(2): 233-244, 2024 06.
Article En | MEDLINE | ID: mdl-38719375

Outcomes for patients with chronic intestinal failure have improved with organization of experts into multidisciplinary teams delivering care in intestinal rehabilitation programs. There have been improvements in understanding of intestinal failure complications as well as development of newer therapies that have amplified the improvements in survival. In spite of this encouraging trend, patients who fail PN are often referred too late for intestinal transplantation. The author proposes a more rational framework that might allow earlier identification of intestinal failure patients at risk for PN-failure, who could appropriately be considered earlier for intestinal transplantation with improvements in overall outcomes.


Intestines , Humans , Intestines/transplantation , Intestinal Failure/therapy , Parenteral Nutrition , Patient Selection
4.
Gastroenterol Clin North Am ; 53(2): 329-341, 2024 06.
Article En | MEDLINE | ID: mdl-38719382

Infants and children with intestinal failure are at risk for pediatric feeding disorders, which challenge their oral feeding development. This article explores these challenges and offers several practical strategies that can be used by multidisciplinary care teams and at-home caregivers to help support the development of oral feeding in these children and eventually lead to their attaining enteral autonomy.


Enteral Nutrition , Short Bowel Syndrome , Humans , Short Bowel Syndrome/therapy , Short Bowel Syndrome/complications , Infant , Enteral Nutrition/methods , Child , Child, Preschool , Intestinal Failure/therapy , Intestinal Failure/etiology
5.
Hum Immunol ; 85(3): 110809, 2024 May.
Article En | MEDLINE | ID: mdl-38724327

Intestinal transplantation (Itx) can be a life-saving treatment for certain patient populations, including those patients with intestinal failure (IF) who develop life-threatening complications due to the use of parenteral nutrition (PN). Most patients who have undergone Itx are eventually able to tolerate a full oral diet. However, little guidance or consensus exists regarding optimizing the specific components of an oral diet for Itx patients, including macronutrients, micronutrients and dietary patterns. While oral dietary prescriptions have moved to the forefront of primary and preventive care, this movement has yet to occur across the field of organ transplantation. Evidence to date points to the role of systemic chronic inflammation (SCI) in a wide variety of chronic diseases as well as post-transplant graft dysfunction. This review will discuss current trends in oral nutrition for Itx patients and also offer novel insights into nutritional management techniques that may help to decrease SCI and chronic disease risk as well as optimize graft function.


Inflammation , Intestines , Humans , Inflammation/etiology , Inflammation/immunology , Intestines/transplantation , Intestines/immunology , Organ Transplantation/adverse effects , Intestinal Failure/therapy , Intestinal Failure/etiology , Postoperative Complications/etiology , Postoperative Complications/immunology , Nutritional Status
6.
Clin Nutr ; 43(6): 1627-1634, 2024 Jun.
Article En | MEDLINE | ID: mdl-38772069

BACKGROUND & AIMS: Loss of venous access is threatening for patients with intestinal failure (IF) under long-term parenteral nutrition (PN). We aimed to identify the incidence of central venous catheter (CVC) complications, compare different devices, and analyze interventional recanalizing procedures to restore the patency of occluded CVCs. METHODS: For this retrospective cohort study, patient data from a prospective IF database spanning 16 years was analyzed at a tertiary referral center. Catheter dwell times (CDTs) were distinguished by Kaplan-Meier survival analysis and subgroup analyses were performed for different CVC types (tunneled/port catheters). Specific complications (occlusion, catheter-related infection (CRI), displacement, and material defect) were analyzed. Explantation rates and CDTs were compared. RESULTS: Overall, 193 CVCs in 77 patients with IF under PN could be enrolled (62.524 "CVC-days"). Broviac type "B" was found to be significantly superior to type "A" regarding occlusion, CRI, and material defects (log-rank test: p = 0.05; p = 0.026; p = 0.005 respectively). Port catheters were displaying the highest incidence of CRI (2.13 events/1000 catheter days). Interventional catheter recanalization was performed 91 times and significantly increased the CDT from a median of 131 days (IQR: 62; 258) to 389 days (IQR: 262; 731) (Mann-Whitney-U-test: p= <0.001) without increasing complications. CONCLUSIONS: Different complication rates and CDT were seen depending on CVC type. Tunneled catheters were significantly superior concerning CRI. Interventional catheter recanalization is a viable alternative to fibrinolytics to restore CVC patency, but long-term patency data is scarce.


Catheter-Related Infections , Central Venous Catheters , Intestinal Failure , Humans , Male , Female , Retrospective Studies , Central Venous Catheters/adverse effects , Middle Aged , Incidence , Catheter-Related Infections/epidemiology , Intestinal Failure/therapy , Parenteral Nutrition , Catheterization, Central Venous/adverse effects , Catheterization, Central Venous/methods , Adult , Aged , Catheter Obstruction/statistics & numerical data
7.
Gastroenterol Clin North Am ; 53(2): 309-327, 2024 06.
Article En | MEDLINE | ID: mdl-38719381

Achieving feeding skills and food acceptance is a multi-layered process. In pediatric intestinal failure (PIF), oral feeding is important for feeding skills development, physiologic adaptation, quality of life and the prevention of eating disorders. In PIF, risk factors for feeding difficulties are common and early data suggests that feeding difficulties are prevalent. There is a unique paradigm for the feeding challenges in PIF. Conventional definitions of eating disorders have limited application in this context. A pediatric intestinal failure associated eating disorder (IFAED) definition that includes feeding/eating skills dysfunction, psychosocial dysfunction, and the influence on weaning nutrition support is proposed.


Feeding and Eating Disorders , Humans , Child , Feeding and Eating Disorders/complications , Risk Factors , Intestinal Failure/therapy , Feeding Behavior
8.
Pediatr Surg Int ; 40(1): 97, 2024 Apr 06.
Article En | MEDLINE | ID: mdl-38581576

PURPOSE: The effect of different types of lipid emulsion may guide therapy of patients with intestinal failure (IF) to limit morbidity such as intestinal failure-associated liver disease (IFALD). METHODS: A retrospective chart review of pediatric patients with IF who received soybean oil lipid emulsion (SL) or mixed oil lipid emulsion (ML) was performed. Data over 1 year were collected. RESULTS: Forty-five patients received SL and 34 received ML. There were no differences in the incidence (82 versus 74%, P = 0.35) or resolution (86 versus 92%, P = 0.5) of IFALD between the cohorts. The median dose of ML was higher compared to SL (2 versus 1 g/kg/day, P < 0.001). If resolved, IFALD resolved rapidly in the ML cohort compared to the SL cohort (67 versus 37 days, P = 0.01). Weight gain was higher in the ML compared to the SL cohort at resolution of IFALD or 1 year from diagnosis of IF (P = 0.009). CONCLUSION: The administration of ML did not alter the incidence or resolution of IFALD compared to SL in pediatric IF. There was rapid resolution of IFALD and enhanced weight gain in the ML cohort compared to SL in pediatric IF.


Intestinal Diseases , Intestinal Failure , Liver Diseases , Liver Failure , Humans , Child , Fat Emulsions, Intravenous/therapeutic use , Parenteral Nutrition , Retrospective Studies , Intestinal Diseases/drug therapy , Liver Diseases/complications , Liver Failure/complications , Soybean Oil/therapeutic use , Weight Gain , Fish Oils
9.
Nutrients ; 16(8)2024 Apr 14.
Article En | MEDLINE | ID: mdl-38674859

Background and aims: Home parenteral nutrition (HPN) is a life-saving treatment for patients affected by chronic intestinal failure (CIF). Both this clinical condition and its therapy require radical lifestyle modifications, affecting life quality and psychological balance in patients as well as family members. Patient psychological burden has rarely been taken into consideration, not to mention that of caregivers. This study aims to evaluate the levels of anxiety in CIF patients on HPN, and their caregivers, consequently determining their impact on the psychological and physical aspects. Methods: After a brief introductory interview, adult patients on HPN for CIF and their caregivers were asked to fill in the HAMA-A questionnaire. Results: Fifty patients and their respective caregivers were enrolled. Mean HAMA-A scores were similar in patients and caregivers and testified the presence of a mild to severe impact of CIF and HPN in both groups, with a significantly higher impact on female patients and caregivers. After adjusting age, education level, duration of CIF and HPN dependence, and degree of kinship, no differences were revealed in the scores. Conclusions: The study confirms that CIF patients on HPN and their caregivers have a significant anxiety burden independently from the duration of the disease, therefore needing appropriate support.


Anxiety , Caregivers , Intestinal Failure , Parenteral Nutrition, Home , Humans , Female , Male , Caregivers/psychology , Anxiety/psychology , Middle Aged , Chronic Disease , Parenteral Nutrition, Home/psychology , Adult , Intestinal Failure/therapy , Intestinal Failure/psychology , Surveys and Questionnaires , Aged , Quality of Life , Cost of Illness
10.
JPEN J Parenter Enteral Nutr ; 48(4): 486-494, 2024 May.
Article En | MEDLINE | ID: mdl-38605559

BACKGROUND: Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking. The aim of this study is to evaluate the effectiveness and safety of the current management in an HPN expertise center in the Netherlands. METHODS: We performed a retrospective descriptive cohort study between 2013 and 2022 on children 0-18 years of age with chronic IF requiring long-term HPN. Our primary outcomes were the incidence of S aureus CRBSI per 1000 catheter days, catheter salvage attempt rate, and successful catheter salvage rate. Our secondary outcomes included complications and mortality. RESULTS: A total of 74 patients (39 male; 53%) were included, covering 327.8 catheter years. Twenty-eight patients (38%) had a total of 52 S aureus CRBSIs, with an incidence rate of 0.4 per 1000 catheter days. The catheter salvage attempt rate was 44% (23/52). The successful catheter salvage rate was 100%. No relapse occurred, and no removal was needed after catheter salvage. All complications that occurred were already present at admission before the decision to remove the catheter or not. No patients died because of an S aureus CRBSI. CONCLUSION: Catheter salvage in S aureus CRBSIs in children receiving HPN can be attempted after careful consideration by a multidisciplinary team in an HPN expertise center.


Catheter-Related Infections , Intestinal Failure , Parenteral Nutrition, Home , Staphylococcal Infections , Staphylococcus aureus , Taurine/analogs & derivatives , Thiadiazines , Humans , Parenteral Nutrition, Home/methods , Parenteral Nutrition, Home/adverse effects , Male , Catheter-Related Infections/prevention & control , Catheter-Related Infections/microbiology , Catheter-Related Infections/epidemiology , Retrospective Studies , Female , Child , Child, Preschool , Infant , Staphylococcal Infections/prevention & control , Adolescent , Netherlands , Intestinal Failure/therapy , Infant, Newborn , Central Venous Catheters/adverse effects , Central Venous Catheters/microbiology , Chronic Disease , Incidence , Device Removal , Cohort Studies , Catheterization, Central Venous/adverse effects , Catheters, Indwelling/adverse effects , Catheters, Indwelling/microbiology , Bacteremia/prevention & control , Bacteremia/epidemiology , Bacteremia/etiology
11.
J Paediatr Child Health ; 60(4-5): 132-138, 2024.
Article En | MEDLINE | ID: mdl-38655775

AIMS: The New Zealand National Intestinal Failure and Rehabilitation Service (NZ-NIFRS) was established in October 2015 to gather longitudinal data on the aetiology, clinical course and outcomes of children with intestinal failure (IF). One main objective is to achieve health equity for patients with IF in NZ. METHODS: Clinical outcomes (enteral autonomy, parenteral nutrition (PN) dependence, death or intestinal transplantation) for IF patients diagnosed from October 2015 to 2018 were analysed; comparisons were made by ethnicity and socio-economic status (SES) using published 'prioritised-ethnicity' health data and the NZ index of deprivation, respectively. The Cox proportional-hazards model was used to assess time to enteral autonomy. RESULTS: Of the 208 patients (55.77% male, 43.75% preterm), 170 (81.73%) achieved enteral autonomy and 14 (6.73%) remained PN dependent. Pacific and Maori children accounted for 12.98% and 27.88% of the patient cohort, respectively, compared to 9.46% and 25.65% of the NZ paediatric population. More significantly, IF patients with a high NZ socio-economic deprivation score were overrepresented, with 35.92% in the highest deprivation quintile and 10.19% in the least deprived quintile, compared to 23.53% and 20.31%, respectively, of the NZ paediatric population. There were no significant differences in primary clinical outcomes for any patients based on ethnicity or SES. CONCLUSION: While disparities in ethnicity and social deprivation do exist in the incidence of IF in NZ children, clinical outcomes are similar for children regardless of ethnicity or SES. NZ-NIFRS has achieved one of its core objectives: to achieve health equity for all patients with IF nationwide.


Intestinal Failure , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Ethnicity , Health Status Disparities , Healthcare Disparities/ethnology , Incidence , New Zealand/epidemiology , Social Class , Socioeconomic Factors , Pacific Island People , Maori People
12.
Pediatr Transplant ; 28(3): e14756, 2024 May.
Article En | MEDLINE | ID: mdl-38623905

INTRODUCTION: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. OBJECTIVES: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. METHODS: The review is based on the authors' experience supported by an in-depth review of the published literature. RESULTS: The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. DISCUSSION: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.


Intestinal Diseases , Intestinal Failure , Short Bowel Syndrome , Transplants , Child , Infant , Humans , Intestines , Intestine, Small , Parenteral Nutrition , Intestinal Diseases/surgery , Short Bowel Syndrome/surgery
13.
Clin Nutr ; 43(6): 1331-1342, 2024 Jun.
Article En | MEDLINE | ID: mdl-38677044

OBJECTIVE: Fecal microbiota was investigated in adult patients with chronic intestinal failure (CIF) due to short bowel syndrome (SBS) with jejunocolonic anastomosis (SBS-2). Few or no data are available on SBS with jejunostomy (SBS-1) and CIF due to intestinal dysmotility (DYS) or mucosal disease (MD). We profiled the fecal microbiota of various pathophysiological mechanisms of CIF. METHODS: Cross-sectional study on 61 adults with CIF (SBS-1 30, SBS-2 17, DYS 8, MD 6). Fecal samples were collected and profiled by 16S rRNA amplicon sequencing. Healthy controls (HC) were selected from pre-existing cohorts, matched with patients by sex and age. RESULTS: Compared to HC, SBS-1, SBS-2 and MD patients showed lower alpha diversity; no difference was found for DYS. In beta diversity analysis, SBS-1, SBS-2 and DYS groups segregated from HC and from each other. Taxonomically, the CIF groups differed from HC even at the phylum level. In particular, CIF patients' microbiota was dominated by Lactobacillaceae and Enterobacteriaceae, while depleted in typical health-associated taxa belonging to Lachnospiraceae and Ruminococcaceae. Notably, compositional peculiarities of the CIF groups emerged. Furthermore, in the SBS groups, the microbiota profile differed according to the amount of parenteral nutrition required and the duration of CIF. CONCLUSIONS: CIF patients showed marked intestinal dysbiosis with microbial signatures specific to the pathophysiological mechanism of CIF as well as to the severity and duration of SBS.


Feces , Gastrointestinal Microbiome , Short Bowel Syndrome , Humans , Male , Female , Cross-Sectional Studies , Middle Aged , Feces/microbiology , Adult , Short Bowel Syndrome/microbiology , Short Bowel Syndrome/physiopathology , Chronic Disease , Aged , Intestinal Failure/microbiology , RNA, Ribosomal, 16S/genetics
14.
Pediatr Surg Int ; 40(1): 79, 2024 Mar 16.
Article En | MEDLINE | ID: mdl-38492075

BACKGROUND: Intestinal Failure, parenteral nutrition (PN) dependence, and subsequent liver disease are the most challenging and life-threatening complications of short bowel syndrome experienced by patients with total intestinal aganglionosis. Skipped Aganglionic Lengthening Transposition (SALT) showed to be a promising procedure to overcome such problems. We herein report the results of two patients who underwent SALT at the Umberto Bosio Center for Digestive Diseases. PATIENTS AND METHODS: Between November 2019 and July 2022, 2 patients with total intestinal aganglionosis underwent SALT as autologous intestinal lengthening procedure. Perioperative data and long-term outcomes are reported. Patient #1-A 18 month-old male (PN dependant) with 30 cm of ganglionated bowel at birth experienced a 35% increase of intestinal length after SALT (from 43 to 58 cm) thanks to three 5 cm interposed aganglionic loops. Postoperative course was uneventful and he was totally weaned by PN after 28 months postoperatively. He is without PN only receiving enteric feeding 53 months after the procedure. Patient #2-A 11 year-old female (PN dependant) with 100 cm of ganglionated jejunum underwent SALT at 11 years and experienced a 19% increase of bowel length thanks to four 5 to 7 cm interposed aganglionic loops. Postoperatively she required excision of two out of the four loops due to severe strictures and inadequate perfusion with a subsequent overall 10% increase of length after SALT. Of note, she improved significantly with a progressive reduction of PN that has been stopped after 18 months. CONCLUSION: Skipped aganglionic lengthening transposition (SALT) seems to be very effective in improving nutrients absorption in patients with total intestinal aganglionosis by increasing absorptive bowel surface and decelerating intestinal flow for a longer and more effective contact of enteric material with ileal mucosa. Provided these impressive results are confirmed in the very long-term, SALT could become a valid alternative for the treatment of patients with total intestinal aganglionosis carrying at birth at least 20 to 30 cm of ganglionated jejunum.


Hirschsprung Disease , Intestinal Failure , Short Bowel Syndrome , Child , Female , Humans , Infant , Male , Intestine, Small , Intestines/surgery , Short Bowel Syndrome/surgery , Treatment Outcome
15.
Zhonghua Wei Chang Wai Ke Za Zhi ; 27(3): 231-235, 2024 Mar 25.
Article Zh | MEDLINE | ID: mdl-38532584

Intestinal failure is a syndrome characterized by a diminished intestinal function that is inadequate to maintain normal digestion and absorption, leading to systemic metabolic disorder and requiring long-term nutritional supplementation to sustain health and growth. Short bowel syndrome (SBS) is one of the primary causes of intestinal failure. Given the significant differences among SBS patients, nutritional treatment strategies should emphasize individualization. This review focuses on SBS, combining its anatomical and pathological characteristics, to introduce nutritional support treatment plans and experiences for patients with intestinal failure.


Intestinal Failure , Short Bowel Syndrome , Humans , Nutritional Support , Intestines
16.
Chirurgie (Heidelb) ; 95(5): 375-381, 2024 May.
Article De | MEDLINE | ID: mdl-38427034

BACKGROUND: Intestinal transplantation (ITx) is the only causal treatment for complicated chronic intestinal failure after mesenteric ischemia and impending failure of parenteral supplementation. Isolated or combined ITx with the inclusion of the intestine is associated with demanding immunological, perioperative and infection associated challenges. AIM: The characterization of chronic intestinal failure, the indications, transplant survival, transplantation techniques and success rates. MATERIAL AND METHODS: Collection, summary and critical appraisal of international guidelines, the guidelines of the German Medical Chamber, and the international literature. RESULTS: The first successful ITx were performed in 1987 and 1988 at the University of Kiel Germany and the University of Pittsburgh, USA. The number of ITx rose continuously but in phases from the end of the 1990s to over 200 per year but has currently decreased to 100-150 per year due to optimized intestinal rehabilitation. While the 1­year and 3­year transplant survival rates were 30% and 20% before 1991, they increased in phases up to 60% and 50%, respectively, after 1995 and have now achieved almost 80% and 70%, respectively. CONCLUSION: The substantial improvement in the results of ITx can be partly explained by progress in operative techniques, intensive care medicine and a better understanding of mucosal immunity; however, optimized strategies in immunosuppression as well as prevention of infectious diseases and malignancies have also made decisive contributions.


Intestinal Diseases , Intestinal Failure , Mesenteric Ischemia , Short Bowel Syndrome , Humans , Short Bowel Syndrome/surgery , Short Bowel Syndrome/complications , Mesenteric Ischemia/surgery , Mesenteric Ischemia/complications , Intestines/surgery , Intestinal Diseases/complications , Intestinal Diseases/surgery , Chronic Disease
17.
J Pediatr Gastroenterol Nutr ; 78(4): 918-926, 2024 Apr.
Article En | MEDLINE | ID: mdl-38451061

OBJECTIVES: Patients with intestinal failure require central venous access which puts them at risk for central line-associated bloodstream infections (CLABSI). Maintaining vascular patency is critical for this population to receive nutrition support. When CLABSIs occur line salvage can help maintain vascular access. The aim of this study is to assess factors associated with safe and successful central venous catheter salvage. METHODS: Retrospective cohort study of patients with intestinal failure at two tertiary care institutions between 2012 and 2020. The study examined the rates of attempted salvage, factors associated with successful salvage, and complications associated with salvage attempts. RESULTS: Over the study period, 76 patients with intestinal failure were include while central venous access was in place. There were a total of 94 CLABSIs. Salvage was more likely to be attempted when patients were under the direct care of an intestinal rehabilitation service (95% vs. 68%, p = 0.04). The overall successful salvage rate was 91.6% (n = 77). Gram-positive, Gram-negative, and polymicrobial infections had successful salvage rates of 97%, 92%, and 94% respectively. The successful salvage rate for fungal infections was 40%. There was no difference in 30-day complication rates for hospital readmission, intensive care unit admission, and death between patients who underwent salvage attempt and those who did not. CONCLUSIONS: Central line salvage can be safely attempted for many infections in patients with intestinal failure, leading to vascular access preservation.


Catheter-Related Infections , Catheterization, Central Venous , Central Venous Catheters , Intestinal Diseases , Intestinal Failure , Sepsis , Humans , Child , Retrospective Studies , Catheter-Related Infections/epidemiology , Catheter-Related Infections/microbiology , Intestinal Diseases/therapy , Intestinal Diseases/complications , Central Venous Catheters/adverse effects , Central Venous Catheters/microbiology , Catheterization, Central Venous/adverse effects
18.
J Pediatr Gastroenterol Nutr ; 78(5): 1171-1179, 2024 May.
Article En | MEDLINE | ID: mdl-38477361

OBJECTIVES: Paediatric patients with intestinal failure (IF) are at risk for both gastrointestinal (GI) and systemic complications, thus depending on a functioning network of multidisciplinary care. Data on the clinical impact of coronavirus disease 2019 (COVID-19) or the pandemic-related restrictions are limited. We aimed to analyse the clinical course of COVID-19 in children with IF, and to evaluate the perceived impact of the COVID-19 pandemic on IF patients and their caregivers by analysing quality of life (QoL), health-related QoL (HRQoL) and health care. METHODS: Children with IF presenting at our intestinal rehabilitation centre were enrolled and interviewed about test-proven COVID-19 infection. A standardised questionnaire was offered to all caregivers of IF patients and to two control groups (children with inflammatory bowel disease and gastrointestinal healthy children). RESULTS: Between December 2020 and November 2022, 25 out of 127 patients with IF contracted COVID-19. Forty-eight per cent had GI symptoms, 32% required additional intravenous fluids and 20% were hospitalized. Only 25% of vaccinated children showed signs of GI dysfunction, compared to 52% of unvaccinated children. Analysis of 93 questionnaires showed a negative impact on QoL and HRQoL (>66.7% and >27.8%, respectively). IF patients frequently experienced restrictions in health care, including appointments, services and supply of parenteral nutrition or medications. Caregiver burden increased significantly more often in caregivers of children with IF (p = 0.007). CONCLUSIONS: Paediatric patients with IF contracting COVID-19 have an increased risk for GI dysfunction which may be alleviated by vaccination. Children and their caregivers were highly burdened by pandemic-related restrictions and reductions in health care provision.


COVID-19 , Hospitals, Pediatric , Intestinal Failure , Quality of Life , Humans , COVID-19/epidemiology , COVID-19/complications , Child , Female , Male , Child, Preschool , Adolescent , SARS-CoV-2 , Tertiary Care Centers , Chronic Disease , Caregivers/psychology , Surveys and Questionnaires , Infant
19.
Dig Dis Sci ; 69(5): 1770-1777, 2024 May.
Article En | MEDLINE | ID: mdl-38446306

BACKGROUND & AIMS: The intestinal rehabilitation program (IRP) is a specialized approach to managing patients with intestinal failure (IF). The goal of IRP is to reduce the patient's dependence on parenteral nutrition by optimizing nutrition intake while minimizing the risk of complications and providing individualized medical and surgical treatment. We aimed to provide a thorough overview of our extensive history in adult IRP. METHODS: We reviewed the medical records of adults with IF treated at our center's IRP over the past two decades. We collected data on demographic and clinical results, such as the causes of IF, the current status of the remaining bowel, nutritional support, and complications or mortality related to IF or prolonged parenteral nutrition. RESULTS: We analyzed a total of 47 adult patients with a median follow-up of 6.7 years. The most common cause of IF was massive bowel resection due to mesenteric vessel thrombosis (38.3%). Twenty-eight patients underwent rehabilitative surgery, including 12 intestinal transplants. The 5-year survival rate was 81.9% with 13 patients who expired due to sepsis, liver failure, or complication after transplantation. Of the remaining 34 patients, 18 were successfully weaned off from parenteral nutrition. CONCLUSION: Our results of IRP over two decades suggest that the individualized and multidisciplinary program for adult IF is a promising approach for improving patient outcomes and achieving nutritional autonomy.


Intestinal Failure , Parenteral Nutrition , Tertiary Care Centers , Humans , Female , Male , Adult , Middle Aged , Aged , Retrospective Studies , Treatment Outcome , Young Adult
20.
Nutr Clin Pract ; 39(3): 634-640, 2024 Jun.
Article En | MEDLINE | ID: mdl-38491966

BACKGROUND: Chronic hepatic complications are common in patients with short bowel syndrome-associated intestinal failure (SBS-IF). Teduglutide, a glucagon-like peptide-2 analogue, demonstrated efficacy in reducing parenteral nutrition and/or intravenous fluid dependence among patients with SBS-IF in phase 3 clinical studies. METHODS: This was a post hoc analysis of pooled data from two separate randomized, double-blind, placebo-controlled, multinational phase 3 clinical studies. Adult patients with SBS-IF with parenteral nutrition and/or intravenous fluid dependence without liver disease at baseline were randomized to treatment with the glucagon-like peptide-2 analogue teduglutide (0.05 or 0.10 mg/kg/day) or placebo subcutaneously once daily for 24 weeks. Mixed-effects models assessed the baseline predictors of change in liver chemistries. RESULTS: Between baseline and week 24, teduglutide treatment (n = 109) was associated with least squares mean reductions in aspartate aminotransferase (-7.51 IU/L; P = 0.014), alanine aminotransferase (-12.15 IU/L; P = 0.002), and bilirubin (-5.03 µmol/L [-0.057 mg/dl]; P < 0.001) compared with that of the placebo (n = 59). These values were independent of reductions in parenteral nutrition and/or intravenous fluid dependence. CONCLUSION: Teduglutide treatment was associated with reductions in liver chemistries by week 24, which is beneficial for patients with SBS-IF beyond improvements in parenteral nutrition and/or intravenous fluid dependence. Future studies should examine how long-term teduglutide might mitigate the risk of liver disease in patients with SBS-IF.


Gastrointestinal Agents , Liver , Peptides , Short Bowel Syndrome , Humans , Short Bowel Syndrome/complications , Short Bowel Syndrome/drug therapy , Male , Female , Middle Aged , Peptides/therapeutic use , Double-Blind Method , Adult , Liver/drug effects , Liver/metabolism , Gastrointestinal Agents/therapeutic use , Gastrointestinal Agents/pharmacology , Aspartate Aminotransferases/blood , Parenteral Nutrition/methods , Alanine Transaminase/blood , Aged , Bilirubin/blood , Intestinal Failure/drug therapy , Treatment Outcome , Liver Diseases
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