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1.
Pediatr Surg Int ; 40(1): 226, 2024 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-39145817

RESUMO

PURPOSE: Patients with intestinal failure (IF) require long-term parenteral nutrition using central venous catheters (CVCs), which often require replacement. We adopted a less fallible guidewire replacement (GWR) method and verified its effectiveness and validity. METHODS: We enrolled 108 cases that underwent a CVC replacement with "GWR" method with IF at our department between 2013 and 2023. We retrospectively reviewed patients' clinical details with tunneled CVC (Hickman/Broviac catheter). For the analysis, we compared for the same time period the catheter exchange method "Primary placement"; newly inserted catheter by venipuncture. RESULTS: The success rate of catheter replacement using GWR was 94.4%. There were six unsuccessful cases. A log-rank test showed no significant difference in catheter survival between primary placement and the GWR, and the time to first infection was significantly longer in the GWR (p = 0.001). Furthermore, no significant differences were observed between the two methods until the first infection, when the exchange indication was limited to infections. In the same way, when the indication was restricted to catheter-related bloodstream infection, there was no significant difference in catheter survival between the two approaches. CONCLUSION: Our GWR procedure was easy to perform and stable, with a high success rate and almost no complications. Moreover, using a guidewire did not increase the frequency of catheter replacement and the infection rate.


Assuntos
Cateterismo Venoso Central , Cateteres Venosos Centrais , Insuficiência Intestinal , Humanos , Estudos Retrospectivos , Masculino , Feminino , Cateterismo Venoso Central/métodos , Insuficiência Intestinal/terapia , Lactente , Pré-Escolar , Criança , Infecções Relacionadas a Cateter , Nutrição Parenteral/métodos , Resultado do Tratamento , Remoção de Dispositivo/métodos , Adolescente
2.
FASEB J ; 38(15): e23847, 2024 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-39096137

RESUMO

Intestinal failure-associated liver disease (IFALD) is a serious complication of long-term parenteral nutrition in patients with short bowel syndrome (SBS), and is the main cause of death in SBS patients. Prevention of IFALD is one of the major challenges in the treatment of SBS. Impairment of intestinal barrier function is a key factor in triggering IFALD, therefore promoting intestinal repair is particularly important. Intestinal repair mainly relies on the function of intestinal stem cells (ISC), which require robust mitochondrial fatty acid oxidation (FAO) for self-renewal. Herein, we report that aberrant LGR5+ ISC function in IFALD may be attributed to impaired farnesoid X receptor (FXR) signaling, a transcriptional factor activated by steroids and bile acids. In both surgical biopsies and patient-derived organoids (PDOs), SBS patients with IFALD represented lower population of LGR5+ cells and decreased FXR expression. Moreover, treatment with T-ßMCA in PDOs (an antagonist for FXR) dose-dependently reduced the population of LGR5+ cells and the proliferation rate of enterocytes, concomitant with decreased key genes involved in FAO including CPT1a. Interestingly, however, treatment with Tropifexor in PDOs (an agonist for FXR) only enhanced FAO capacity, without improvement in ISC function and enterocyte proliferation. In conclusion, these findings suggested that impaired FXR may accelerate the depletion of LGR5 + ISC population through disrupted FAO processes, which may serve as a new potential target of preventive interventions against IFALD for SBS patients.


Assuntos
Hepatopatias , Receptores Citoplasmáticos e Nucleares , Síndrome do Intestino Curto , Transdução de Sinais , Células-Tronco , Humanos , Síndrome do Intestino Curto/metabolismo , Síndrome do Intestino Curto/patologia , Receptores Citoplasmáticos e Nucleares/metabolismo , Células-Tronco/metabolismo , Masculino , Hepatopatias/metabolismo , Hepatopatias/patologia , Hepatopatias/etiologia , Feminino , Criança , Insuficiência Intestinal/metabolismo , Pré-Escolar , Lactente , Mucosa Intestinal/metabolismo , Mucosa Intestinal/patologia , Receptores Acoplados a Proteínas G/metabolismo , Proliferação de Células , Intestinos/patologia , Enterócitos/metabolismo
3.
Nutrients ; 16(15)2024 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-39125394

RESUMO

The Short Bowel Syndrome (SBS) Registry (NCT01990040) is a multinational real-world study evaluating the long-term safety of teduglutide in patients with SBS and intestinal failure (SBS-IF) in routine clinical practice. This paper describes the study methodology and baseline characteristics of adult patients who have (ever-treated) or have never (never-treated) received teduglutide. A total of 1411 adult patients (679 ever-treated; 732 never-treated) were enrolled at 124 sites across 17 countries. The mean (standard deviation [SD]) age at enrollment was 55.4 (15.46) years, and 60.2% of patients were women. Crohn's disease was the most common cause of major intestinal resection in both ever-treated (34.1%) and never-treated patients (20.4%). A similar proportion of ever-treated and never-treated patients had a prior history of colorectal polyps (2.7% vs. 3.6%), whereas proportionally fewer ever-treated patients reported a history of colorectal cancer (1.8% vs. 6.2%) or any malignancy (17.7% vs. 30.0%) than never-treated patients. Never-treated patients received a numerically greater mean (SD) volume of parenteral nutrition and/or intravenous fluids than ever-treated patients (12.4 [8.02] vs. 10.1 [6.64] L/week). Ever-treated patients received a mean teduglutide dosage of 0.05 mg/kg/day. This is the first report of patient baseline characteristics from the SBS Registry, and the largest cohort of patients with SBS-IF to date. Overall, ever-treated and never-treated patients had similar baseline characteristics. Differences between treatment groups may reflect variations in patient selection and degree of monitoring.


Assuntos
Fármacos Gastrointestinais , Peptídeos , Sistema de Registros , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/tratamento farmacológico , Feminino , Masculino , Pessoa de Meia-Idade , Peptídeos/uso terapêutico , Adulto , Idoso , Fármacos Gastrointestinais/uso terapêutico , Insuficiência Intestinal/tratamento farmacológico , Resultado do Tratamento , Doença de Crohn/tratamento farmacológico
4.
Neurogastroenterol Motil ; 36(9): e14853, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38973248

RESUMO

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with - the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.


Assuntos
Nutrição Parenteral , Humanos , Nutrição Parenteral/métodos , Eixo Encéfalo-Intestino/fisiologia , Insuficiência Intestinal/terapia , Nutrição Parenteral no Domicílio
5.
Gastroenterol Clin North Am ; 53(3): 461-472, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39068007

RESUMO

Intestinal failure manifests as an impaired capacity of the intestine to sufficiently absorb vital nutrients and electrolytes essential for growth and well-being in pediatric and adult populations. Although parenteral nutrition remains the mainstay therapeutic approach, the pursuit of a definitive and curative strategy, such as regenerative medicine, is imperative. Substantial advancements in the field of engineered intestinal tissues present a promising avenue for addressing intestinal failure; nevertheless, extensive research is still necessary for effective translation from experimental benchwork to clinical bedside applications.


Assuntos
Intestinos , Engenharia Tecidual , Humanos , Engenharia Tecidual/métodos , Intestinos/transplante , Insuficiência Intestinal/terapia , Bioengenharia/métodos , Medicina Regenerativa/métodos , Alicerces Teciduais
6.
Clin Nutr ; 43(8): 1844-1851, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38959661

RESUMO

BACKGROUND: Life expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary. Therefore, we investigated the perceptions of health care professionals from various disciplines working in this specific field, concerning effective interventions regarding transition to adult health care. AIM: To create a standardized protocol which provides practical guidance for health care professionals in order to bridge the gap between pediatric and adult health care and to facilitate successful transition of children with chronic intestinal failure. METHODS: A survey consisting of 20 interventions for transition was sent out to members of the Intestinal Failure working group of European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies (ERNICA) and the Network of Intestinal Failure and Intestinal Transplant in Europe (NITE) group - European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) healthcare professionals in 48 medical centers in various countries. Next to 20 interventions, an open-ended question to fill in any other suggestion with respect to most effective intervention was included. Interventions scoring higher than 80% by the participants were included in the protocol. Interventions scoring between 50% and 80% and other own suggestions were discussed during a consensus meeting and included when consensus, defined as unanimous agreement, was reached. Interventions scoring as effective by < 50% of participants were excluded directly. RESULTS: A total of 80 healthcare professionals from 33 medical centers (participation rate 69%) participated. The protocol consisted of modifiable components expected to be targets of interventions. The most important key outcomes of the survey were: 1) assessment of patient's transition readiness and provision of knowledge to the patient by the pediatric team, 2) involvement of parents in the transition process, and 3) collaboration between the pediatric and adult chronic intestinal failure team. In addition it is advised that the transition process should start 1-2 years before transfer. A nurse specialist working in both services should form a bridge. All interventions must be tailor-made and based on the maturity of the patient. CONCLUSION: This study provides a protocol describing transition of children with chronic intestinal failure from pediatric to adult care. This international protocol will serve as practical guidance for pediatric chronic intestinal failure which will provide a more structured, optimal transition process. It is advised to use this protocol as a formal checklist that can be placed in the patient's chart to review and track the transition process by CIF team members. Future research investigating transition readiness of CIF patients is needed.


Assuntos
Insuficiência Intestinal , Nutrição Parenteral no Domicílio , Transição para Assistência do Adulto , Humanos , Nutrição Parenteral no Domicílio/métodos , Insuficiência Intestinal/terapia , Doença Crônica/terapia , Transição para Assistência do Adulto/normas , Adulto , Criança , Inquéritos e Questionários , Europa (Continente)
7.
Gastroenterol Clin North Am ; 53(3): 441-452, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39068005

RESUMO

This study explores intestinal transplantation (ITx) as a viable treatment option for intestinal failure (IF). Historical development, donor and recipient considerations, surgical techniques, immunosuppression, and outcomes, are reviewed with particular emphasis to the value of living donor ITx. The review highlights the evolution of ITx and emphasizes the ongoing need for patient-specific selection processes. In the realm of pediatric ITx, the article underlines the significance of early intervention to mitigate IF-related liver disease. Overall, it provides a comprehensive overview of this life-saving procedure.


Assuntos
Intestinos , Doadores Vivos , Humanos , Intestinos/transplante , Insuficiência Intestinal/cirurgia , Transplante de Órgãos/métodos
8.
J Pediatr Gastroenterol Nutr ; 79(2): 290-300, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38873891

RESUMO

OBJECTIVES: Patients with short bowel syndrome-associated intestinal failure (SBS-IF) require long-term parenteral nutrition and/or intravenous fluids (PN/IV) to maintain fluid or nutritional balance. We report the long-term safety, efficacy, and predictors of response in pediatric patients with SBS-IF receiving teduglutide over 96 weeks. METHODS: This was a pooled, post hoc analysis of two open-label, long-term extension (LTE) studies (NCT02949362 and NCT02954458) in children with SBS-IF. Endpoints included treatment-emergent adverse events (TEAEs) and clinical response (≥20% reduction in PN/IV volume from baseline). A multivariable linear regression identified predictors of teduglutide response; the dependent variable was mean change in PN/IV volume at each visit over 96 weeks. RESULTS: Overall, 85 patients were analyzed; 78 patients received teduglutide in the parent and/or LTE studies (any teduglutide [TED] group), while seven patients did not receive teduglutide in either the parent or LTE studies. Most TEAEs were moderate or severe in intensity in both groups. By week 96, 82.1% of patients from the any TED group achieved a clinical response, with a mean fluid decrease of 30.1 mL/kg/day and an energy decrease of 21.6 kcal/kg/day. Colon-in-continuity, non-White race, older age at baseline, longer duration of teduglutide exposure, and increasing length of remaining small intestine were significantly associated with a reduction in mean PN/IV volume requirements. CONCLUSIONS: In pediatric patients with SBS-IF, teduglutide treatment resulted in long-term reductions in PN/IV requirements. The degree of PN/IV volume reduction depended on the duration of teduglutide exposure, underlying bowel anatomy, and demographics.


Assuntos
Fármacos Gastrointestinais , Peptídeos , Síndrome do Intestino Curto , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Fármacos Gastrointestinais/uso terapêutico , Insuficiência Intestinal/complicações , Nutrição Parenteral , Peptídeos/uso terapêutico , Síndrome do Intestino Curto/tratamento farmacológico , Síndrome do Intestino Curto/complicações , Resultado do Tratamento
9.
J Pediatr Gastroenterol Nutr ; 79(2): 269-277, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38828718

RESUMO

BACKGROUND: Long-term outcomes of congenital diarrheas and enteropathies (CODE) are poorly described. We evaluated the morbidity and mortality of children with CODE followed by an intestinal rehabilitation program (IRP) compared to children with short bowel syndrome (SBS). METHODS: Matched case-control study of children with intestinal failure (IF) due to CODE (diagnosed between 2006 and 2020; N = 15) and SBS (N = 42), matched 1:3, based on age at diagnosis and duration of parenteral nutrition (PN). Nutritional status, growth, and IF-related complications were compared. Survival and enteral autonomy were compared to a nonmatched SBS cohort (N = 177). RESULTS: Fifteen CODE patients (five males, median age 3.2 years) were followed for a median of 2.9 years. Eleven children were alive at the end of the follow-up, and two achieved enteral autonomy. The CODE group had higher median PN fluid and calorie requirements than their matched SBS controls at the end of the follow-up (83 vs. 45 mL/kg/day, p = 0.01; 54 vs. 30.5 kcal/kg/day, p < 0.01), but had similar rates of growth parameters, intestinal failure associated liver disease, central venous catheter complications and nephrocalcinosis. Kaplan-Meier analyses of 10-year survival and enteral autonomy were significantly lower in CODE patients compared to the nonmatched SBS population (60% vs. 89% and 30% vs. 87%, respectively; log-rank p < 0.008). CONCLUSIONS: Despite higher PN needs in CODE, rates of IF complications were similar to matched children with SBS. Enteral autonomy and survival rates were lower in CODE patients. Treatment by IRP can mitigate IF-related complications and improve CODE patient's outcome.


Assuntos
Diarreia , Nutrição Parenteral , Síndrome do Intestino Curto , Humanos , Masculino , Feminino , Estudos de Casos e Controles , Pré-Escolar , Síndrome do Intestino Curto/reabilitação , Síndrome do Intestino Curto/terapia , Diarreia/etiologia , Criança , Lactente , Resultado do Tratamento , Insuficiência Intestinal , Estudos Retrospectivos , Seguimentos , Estado Nutricional
10.
J Pediatr Gastroenterol Nutr ; 79(2): 278-289, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38828781

RESUMO

OBJECTIVES: To review recent evaluations of pediatric patients with intestinal failure (IF) for intestinal transplantation (ITx), waiting list decisions, and outcomes of patients listed and not listed for ITx at our center. METHODS: Retrospective chart review of 97 patients evaluated for ITx from January 2014 to December 2021 including data from referring institutions and protocol laboratory testing, body imaging, endoscopy, and liver biopsy in selected cases. Survival analysis used Kaplan-Meier estimates and Cox proportional hazards regression. RESULTS: Patients were referred almost entirely from outside institutions, one-third because of intestinal failure-associated liver disease (IFALD), two-thirds because of repeated infective and non-IFALD complications under minimally successful intestinal rehabilitation, and a single patient because of lost central vein access. The majority had short bowel syndrome (SBS). Waiting list placement was offered to 67 (69%) patients, 40 of whom for IFALD. The IFALD group was generally younger and more likely to have SBS, have received more parenteral nutrition, have demonstrated more evidence of chronic inflammation and have inferior kidney function compared to those offered ITx for non-IFALD complications and those not listed. ITx was performed in 53 patients. Superior postevaluation survival was independently associated with higher serum creatinine (hazard ratio [HR] 15.410, p = 014), whereas inferior postevaluation survival was associated with ITx (HR 0.515, p = 0.035) and higher serum fibrinogen (HR 0.994, p = 0.005). CONCLUSIONS: Despite recent improvements in IF management, IFALD remains a prominent reason for ITx referral. Complications of IF inherent to ITx candidacy influence postevaluation and post-ITx survival.


Assuntos
Intestinos , Listas de Espera , Humanos , Estudos Retrospectivos , Masculino , Feminino , Criança , Pré-Escolar , Lactente , Intestinos/transplante , Adolescente , Insuficiência Intestinal , Síndrome do Intestino Curto/cirurgia , Hepatopatias/cirurgia
11.
JPEN J Parenter Enteral Nutr ; 48(6): 718-725, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38850079

RESUMO

BACKGROUND: Chronic intestinal failure (CIF) refers to the long-lasting reduction of gut function below the minimum necessary to absorb macronutrients, water, and/or electrolytes. Patients with CIF likely develop various forms of malnutrition and dehydration, yet studies that focus primarily on body composition are lacking. Therefore, this study aimed to evaluate the body composition of adult patients with CIF. METHODS: This retrospective descriptive cohort study was performed at the Radboud University Medical Center, a tertiary referral center for CIF treatment in the form of home parenteral nutrition. We collected available bioelectrical impedance analysis (BIA) data from routine care between 2019 and 2023. The primary outcome was body composition, which was evaluated by assessing body mass index (BMI), fat-free mass index (FFMI), and fat percentage (fat%). RESULTS: Overall, 147 adult patients with CIF were included with a median (interquartile range) age of 58 (25-68) years; 69% were female. The mean (SD) BMI was 22.1 (4.3) kg/m2, FFMI was 14.2 (1.9) kg/m2 in females and 17.0 (2.0) kg/m2 in males, and fat% was 33.7% (6.8%) in females and 24.6% (6.4%) in males. 63% had an FFMI below references, and 48% had a high fat%. CONCLUSION: This study found that most adult patients with CIF have an unfavorable body composition characterized by a high fat% and low FFMI despite having a normal mean BMI. These results highlight the necessity for in-depth nutrition assessment, including BIA measurement. Moreover, future studies should focus on exercise interventions to increase FFMI and improve body composition and function.


Assuntos
Composição Corporal , Índice de Massa Corporal , Insuficiência Intestinal , Nutrição Parenteral no Domicílio , Humanos , Nutrição Parenteral no Domicílio/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto , Idoso , Doença Crônica , Insuficiência Intestinal/terapia , Impedância Elétrica , Estudos de Coortes , Desnutrição/etiologia
12.
JPEN J Parenter Enteral Nutr ; 48(6): 693-699, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38850512

RESUMO

BACKGROUND: Home parenteral nutrition (HPN) is a life-saving therapy required for the management of type III intestinal failure, one of the rarest organ failures. It requires a multidisciplinary approach to manage the complexity of the underlying medical, surgical, and nutrition issues, but the current levels of healthcare funding in Australia are unknown. This study aimed to quantify the caseload, staffing, and capacity of existing HPN centers nationally. METHODS: This was a cross-sectional survey inviting centers known to provide HPN care. The survey was designed to capture metrics related to the national framework for the delivery of HPN. These centered on staffing levels, patient load, capacity to audit key outcomes, and service challenges. RESULTS: A total of 24 (89%) of 27 invited centers responded to the survey. There were 17 (71%) adult centers and 7 (29%) pediatric centers. Adult centers managed a median of 12 (interquartile range [IQR]: 6-25) patients vs 16 (IQR: 9-17) in pediatric centers. Several centers did not have dedicated funding for core team members. The total funded clinician time each week per patient was 7 min (IQR: 0-12 min) in adult centers and 14 min (IQR: 10-21 min) in pediatric centers. Fewer than half of centers reported having sufficient resources to regularly audit key metrics. CONCLUSION: The availability of dedicated expertise to manage the highly complex needs of people living with type III intestinal failure is lacking in Australia. Current funding of HPN services falls well short of being sufficient to meet the requirements outlined in the national quality framework.


Assuntos
Nutrição Parenteral no Domicílio , Humanos , Estudos Transversais , Austrália , Nutrição Parenteral no Domicílio/economia , Nutrição Parenteral no Domicílio/estatística & dados numéricos , Inquéritos e Questionários , Insuficiência Intestinal/terapia , Recursos em Saúde/estatística & dados numéricos
13.
JPEN J Parenter Enteral Nutr ; 48(6): 700-707, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38923449

RESUMO

BACKGROUND: Patients with chronic intestinal failure (CIF) are at increased risk of developing renal impairment. The aim of this study was to evaluate the occurrence of chronic kidney disease (CKD) in patients dependent on home parenteral nutrition (HPN) and assess risk factors for renal impairment, including patients with all mechanisms of CIF. METHODS: This was a cohort study of patients initiated on HPN between March 1, 2015, and March 1, 2020, at a national UK IF Reference Centre. Patients were followed from their first discharge with HPN until HPN cessation or the end of follow-up on December 31, 2021. RESULTS: There were 357 patients included in the analysis. Median follow-up time was 4.7 years. At baseline, >40% of patients had renal impairment, with 15.4% fulfilling the criteria for CKD. Mean estimated glomerular filtration rate (eGFR) decreased significantly during the first year after initiation of HPN from 93.32 ml/min/1.73 m2 to 86.30 ml/min/1.73 m2 at the first year of follow-up (P = 0.002), with sequential stabilization of renal function. Increased age at HPN initiation and renal impairment at baseline were associated with decreased eGFR. By the end of follow-up, 6.7% patients developed renal calculi and 26.1% fulfilled the criteria for CKD. CONCLUSION: This is the largest study of renal function in patients receiving long-term HPN. After the first year following HPN initiation, the rate of decline in eGFR was similar to that expected in the general population. These findings should reassure patients and clinicians that close monitoring of renal function can lead to good outcomes.


Assuntos
Taxa de Filtração Glomerular , Insuficiência Intestinal , Nutrição Parenteral no Domicílio , Insuficiência Renal Crônica , Humanos , Nutrição Parenteral no Domicílio/efeitos adversos , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Estudos Longitudinais , Insuficiência Renal Crônica/terapia , Insuficiência Renal Crônica/complicações , Idoso , Adulto , Fatores de Risco , Insuficiência Intestinal/terapia , Doença Crônica , Estudos de Coortes
14.
JPEN J Parenter Enteral Nutr ; 48(5): 624-632, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38837803

RESUMO

BACKGROUND: Selection of central venous catheter (CVC) lock solution impacts catheter mechanical complications and central line-associated bloodstream infections (CLABSIs) in pediatric patients with intestinal failure. Disadvantages of the current clinical standards, heparin and ethanol lock therapy (ELT), led to the discovery of new lock solutions. High-risk pediatric patients with intestinal failure who lost access to ELT during a recent shortage were offered enrollment in a compassionate use trial with 4% tetrasodium EDTA (T-EDTA), a lock solution with antimicrobial, antibiofilm, and antithrombotic properties. METHODS: We performed a descriptive cohort study including 14 high-risk pediatric patients with intestinal failure receiving 4% T-EDTA as a daily catheter lock solution. CVC complications were documented (repairs, occlusions, replacements, and CLABSIs). Complication rates on 4% T-EDTA were compared with baseline rates, during which patients were receiving either heparin or ELT (designated as heparin/ELT). RESULTS: Patients initiated 4% T-EDTA at the time they were enrolled in the compassionate use protocol. Use of 4% T-EDTA resulted in a 50% reduction in CVC complications, compared with baseline rates on heparin/ELT (incidence rate ratio: 0.50; 95% CI, 0.25-1.004; P = 0.051). CONCLUSION: In a compassionate use protocol for high-risk pediatric patients with intestinal failure, the use of 4% T-EDTA reduced composite catheter complications, including those leading to emergency department visits, hospital admissions, additional procedures, and mortality. This outcome suggests 4% T-EDTA has benefits over currently available lock solutions.


Assuntos
Infecções Relacionadas a Cateter , Cateterismo Venoso Central , Cateteres Venosos Centrais , Ácido Edético , Insuficiência Intestinal , Humanos , Estudos Retrospectivos , Ácido Edético/uso terapêutico , Ácido Edético/administração & dosagem , Cateteres Venosos Centrais/efeitos adversos , Feminino , Masculino , Infecções Relacionadas a Cateter/prevenção & controle , Infecções Relacionadas a Cateter/epidemiologia , Pré-Escolar , Lactente , Cateterismo Venoso Central/efeitos adversos , Criança , Heparina/administração & dosagem , Heparina/efeitos adversos , Ensaios de Uso Compassivo , Estudos de Coortes
15.
Clin Nutr ESPEN ; 62: 247-252, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38857151

RESUMO

AIMS: 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. DISCUSSION: 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.


Assuntos
Intestinos , Humanos , Estudos Retrospectivos , Criança , Masculino , Feminino , Intestinos/transplante , Pré-Escolar , Lactente , Resultado do Tratamento , Adolescente , Insuficiência Intestinal , Síndrome do Intestino Curto/cirurgia , Enteropatias/cirurgia , Europa (Continente) , Nutrição Parenteral
16.
Gastroenterol Clin North Am ; 53(2): 233-244, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38719375

RESUMO

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.


Assuntos
Intestinos , Humanos , Intestinos/transplante , Insuficiência Intestinal/terapia , Nutrição Parenteral , Seleção de Pacientes
17.
Gastroenterol Clin North Am ; 53(2): 329-341, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38719382

RESUMO

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.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Criança , Pré-Escolar , Humanos , Lactente , Nutrição Enteral/métodos , Insuficiência Intestinal/terapia , Insuficiência Intestinal/etiologia , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/complicações
18.
Hum Immunol ; 85(3): 110809, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38724327

RESUMO

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.


Assuntos
Inflamação , Intestinos , Humanos , Inflamação/etiologia , Inflamação/imunologia , Intestinos/transplante , Intestinos/imunologia , Transplante de Órgãos/efeitos adversos , Insuficiência Intestinal/terapia , Insuficiência Intestinal/etiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/imunologia , Estado Nutricional
19.
Nutr Hosp ; 41(3): 702-705, 2024 Jun 27.
Artigo em Espanhol | MEDLINE | ID: mdl-38726617

RESUMO

Introduction: Objectives: the prevention of central line-associated bloodstream infections is a critical aspect of care for patients with intestinal failure who are treated with parenteral nutrition. The use of taurolidine in this context is becoming increasingly popular, however there is a lack of standardization in its pediatric application. The objective of this work is to develop a guide to support its prescription. Methodology: the guide is based on a review of the literature and expert opinions from the Intestinal Failure Group of the SEGHNP. It was developed through a survey distributed to all its members, addressing aspects of usual practice with this lock solution. Results: this manuscript presents general recommendations concerning taurolidine indications, commercial presentations, appropriate forms of administration, use in special situations, adverse reactions, and contraindications in the pediatric population Conclusions: taurolidine is emerging as the primary lock solution used to prevent central line-associated bloodstream infections, proving to be safe and effective. This guide aims to optimize and standardize its use in pediatrics.


Introducción: Objetivo: la prevención de las infecciones asociadas a catéter ocupa un papel fundamental en los cuidados del paciente en situación de fracaso intestinal en tratamiento con nutrición parenteral. El empleo del sellado del catéter con taurolidina con ese fin se ha generalizado sin que exista una estandarización sobre su uso en población pediátrica. El objetivo de este trabajo es elaborar una guía clínica que sirva de apoyo en su utilización. Métodos: la guía se basa en una revisión de la literatura y en la opinión de expertos del Grupo de Trabajo de Fracaso Intestinal de la SEGHNP recogida a través de una encuesta realizada a todos sus integrantes sobre aspectos de la práctica habitual con este sellado. Resultados: este manuscrito expone unas recomendaciones en cuanto a las indicaciones, presentaciones comerciales disponibles, forma adecuada de administración, uso en situaciones especiales, reacciones adversas y contraindicaciones de la taurolidina en población pediátrica. Conclusiones: el sellado con taurolidina para la prevención de la infección asociada a catéter venoso central se ha mostrado como un tratamiento eficaz y seguro. La presente guía pretender optimizar y homogeneizar su uso en pediatría.


Assuntos
Insuficiência Intestinal , Nutrição Parenteral , Taurina , Tiadiazinas , Humanos , Tiadiazinas/uso terapêutico , Tiadiazinas/efeitos adversos , Criança , Taurina/análogos & derivados , Taurina/uso terapêutico , Nutrição Parenteral/normas , Nutrição Parenteral/métodos , Insuficiência Intestinal/terapia , Infecções Relacionadas a Cateter/prevenção & controle , Cateterismo Venoso Central/efeitos adversos , Anti-Infecciosos/uso terapêutico , Lactente , Pré-Escolar
20.
Gastroenterol Clin North Am ; 53(2): 309-327, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38719381

RESUMO

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.


Assuntos
Transtornos da Alimentação e da Ingestão de Alimentos , Humanos , Criança , Transtornos da Alimentação e da Ingestão de Alimentos/complicações , Fatores de Risco , Insuficiência Intestinal/terapia , Comportamento Alimentar
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