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1.
J Med Case Rep ; 18(1): 122, 2024 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-38509559

RESUMO

BACKGROUND: Short bowel syndrome (SBS) in adults is defined as having less than 180 to 200 cm of remaining small bowel. Many literature sources do not provide precise epidemiological data, and challenges in estimating the prevalence of SBS include its multifactorial etiology and varying definitions. The most common pathologies leading to SBS include Crohn disease, mesenteric ischemia, radiation enteritis, post-surgical adhesions, and post-operative complications. CASE PRESENTATION: This article presents a clinical case of a 76-year-old Lithuanian patient who underwent parenteral nutrition for four months due to SBS. Before the following diagnosis, the patient had undergone two surgeries. During the hospitalization, life-threatening conditions such as stercoral peritonitis, septic shock, and acute respiratory failure, were observed and treated. As a result of SBS, hypoproteinemia and hypoalbuminemia developed, leading to the prescription of full parenteral nutrition. After correcting the malnutrition, a third surgery was performed, resulting in the discontinuation of parenteral nutrition and the resumption of a regular diet. CONCLUSIONS: Parenteral nutrition is the sole effective method for preserving the lives of patients with a short segment of the intestine. While on parenteral nutrition, patients can be prepared for reconstructive surgery.


Assuntos
Doença de Crohn , Síndrome do Intestino Curto , Adulto , Humanos , Idoso , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/etiologia , Nutrição Parenteral/efeitos adversos , Intestino Delgado , Intestinos/cirurgia , Doença de Crohn/complicações
2.
Clin Nutr ESPEN ; 60: 281-284, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38479922

RESUMO

BACKGROUND AND AIMS: Short bowel syndrome is a malabsorption disorder typically caused by the physical loss of a portion of the intestine, whereafter the body is unable to adequately absorb nutrients, fluids, and electrolytes. Many patients with short bowel syndrome are reliant on home parenteral nutrition through a tunneled or peripherally inserted central catheter to ensure sufficient hydration and nutrition. Central venous catheters are a nidus for bacteria, and patients are at risk for infections associated with high levels of morbidity and mortality. Lactobacillus is a ubiquitous microorganism that most frequently colonizes mucosal surfaces such as the gastrointestinal tract. Lactobacillus bacteremia is rare, with limited occurrence in current medical literature. METHODS: Our patient is a 60-year-old female with a past medical history significant for multiple abdominal surgeries resulting in short bowel syndrome, with subsequent dependence on home parenteral nutrition via peripherally inserted central catheter. She had type III chronic intestinal failure, category D2, and stage 1 moderate malnutrition. She was originally admitted to the hospital for a presumed pulmonary embolism and was found to have a deep vein thrombosis in the setting of her peripherally inserted central catheter. On admission her abdominal exam was unremarkable, she denied abdominal pain, and her only gastrointestinal complaint was chronic stable diarrhea. During the hospitalization she developed severe left lower quadrant abdominal pain and noted decreased frequency of her bowel movements. A computed tomography scan of her abdomen revealed chronic stable intestinal distension and was concerning for obstruction. Clinically she remained without symptoms of acute obstruction or ileus. During the admission she became febrile, with blood cultures from her peripherally inserted central catheter and peripheral IV growing out gram negative rods determined to be lactobacillus bacteremia. The infectious disease team recommended removal of her peripherally inserted central catheter given their concern for a line infection. RESULTS: The patient was treated with broad-spectrum antibiotics, did well clinically, and was ultimately discharged following reinsertion of her peripherally inserted central catheter after negative repeat blood cultures. Though she initially did well in the outpatient setting, she ultimately passed away ten months later after re-presenting to the hospital in septic shock, secondary to bowel ischemia and suspected fungemia of her peripherally inserted central catheter. CONCLUSIONS: In this case report, we describe an unusual case of a patient with short bowel syndrome on chronic parenteral nutrition who developed catheter-associated lactobacillus bacteremia - the first reported case in an adult patient on parenteral nutrition.


Assuntos
Bacteriemia , Cateteres Venosos Centrais , Enteropatias , Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Humanos , Adulto , Feminino , Pessoa de Meia-Idade , Lactobacillus , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/terapia , Cateteres Venosos Centrais/efeitos adversos , Cateteres Venosos Centrais/microbiologia , Bacteriemia/epidemiologia , Nutrição Parenteral no Domicílio/efeitos adversos , Dor Abdominal/etiologia
3.
Clin Nutr ESPEN ; 60: 298-302, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38479925

RESUMO

BACKGROUND & AIMS: Disruptions in the home parenteral nutrition (HPN) process may lead to failure to achieve the intended treatment purposes. This study aimed to evaluate the mid-term clinical outcome in a group of patients with short bowel syndrome (SBS) after a sudden change in the type of home parenteral nutrition programs from customized to commercially premixed admixtures. METHODS: The study conducted in 2020 identified 51 patients with SBS: 27 (53%) women and 24 (47%) men. The SBS were classified as SBS with end jejunostomy 23 (45%) patients, with jejuno-colon anastomosis 23 (45%) patients, with jejuno-ileo anastomosis and total colon in continuity 5 (10%) patients. The following therapeutic program-related data were analysed: changes in nutritional status, body mass index (BMI), Controlling Nutritional Status (CONUT) score, and biochemical assessment. For statistical analysis, the Wilcoxon rank-sum and signed-rank paired tests with continuity corrections were used to compare the results. A p-value of <0.001 was considered statistically significant. RESULTS: There was no statistically significant difference between the analysed groups in total energy, amino acid concentrations, and intravenous volume supplementation. BMI and CONUT assessments of nutritional status and selected biochemical parameters were stable during the study period. CONCLUSIONS: The study demonstrated that a sudden change in the HPN therapy program from parenteral admixtures, tailored to meet individual patients' needs, to commercially premixed admixtures had no significant impact on the mid-term clinical condition of patients with SBS.


Assuntos
Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Masculino , Humanos , Feminino , Síndrome do Intestino Curto/terapia , Estado Nutricional , Índice de Massa Corporal
4.
Nutr Clin Pract ; 39 Suppl 1: S17-S28, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38429962

RESUMO

Fat malabsorption is central to the pathophysiology of short bowel syndrome (SBS). It occurs in patients with insufficient intestinal surface area and/or function to maintain metabolic and growth demands. Rapid intestinal transit and impaired bile acid recycling further contribute to fat malabsorption. A significant portion of patients require parenteral nutrition (PN) for their survival but may develop sepsis and liver dysfunction as a result. Despite advancements in the treatment of SBS, fat malabsorption remains a chronic issue for this vulnerable patient population. Peer-reviewed literature was assessed on the topic of fat malabsorption in SBS. Current management of patients with SBS involves dietary considerations, PN management, antidiarrheals, glucagon-like peptide 2 agonists, and multidisciplinary teams. Clinical trials have focused on improving intestinal fat absorption by facilitating fat digestion with pancreatic enzymes. Targeting fat malabsorption in SBS is a potential pathway to improving lifestyle and reducing morbidity and mortality in this rare disease.


Assuntos
Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/terapia , Intestinos , Nutrição Parenteral , Absorção Intestinal , Dieta
5.
J Pediatr Gastroenterol Nutr ; 78(2): 217-222, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38374557

RESUMO

BACKGROUND: D-lactic acidosis (DLA) is a serious complication of short bowel syndrome (SBS) in children with intestinal failure (IF). Malabsorbed carbohydrates are metabolized by bacteria in the intestine to D-lactate which can lead to metabolic acidosis and neurologic symptoms. METHODS: A retrospective chart review was performed in children ≤18 years old with SBS who had one of the following criteria: unexplained metabolic acidosis, neurologic signs or symptoms, history of antibiotic therapy for small bowel bacterial overgrowth, or high clinical suspicion of DLA. Cases had serum D-lactate concentration >0.25 mmol/L; controls with concentrations ≤0.25 mmol/L. RESULTS: Of forty-six children, median age was 3.16 (interquartile range (IQR): 1.98, 5.82) years, and median residual bowel length was 40 (IQR: 25, 59) cm. There were 23 cases and 23 controls. Univariate analysis showed that cases had significantly lower median bicarbonate (19 vs. 24 mEq/L, p = 0.001), higher anion gap (17 vs. 14 mEq/L, p < 0.001) and were less likely to be receiving parenteral nutrition, compared with children without DLA. Multivariable analysis identified midgut volvulus, history of intestinal lengthening procedure, and anion gap as significant independent risk factors. Midgut volvulus was the strongest independent factor associated with DLA (adjusted odds ratio = 17.1, 95% CI: 2.21, 133, p = 0.007). CONCLUSION: DLA is an important complication of pediatric IF due to SBS. Patients with IF, particularly those with history of midgut volvulus, having undergone intestinal lengthening, or with anion gap acidosis, should be closely monitored for DLA.


Assuntos
Acidose Láctica , Acidose , Anormalidades do Sistema Digestório , Insuficiência Intestinal , Volvo Intestinal , Síndrome do Intestino Curto , Humanos , Criança , Pré-Escolar , Adolescente , Acidose Láctica/etiologia , Acidose Láctica/terapia , Volvo Intestinal/complicações , Estudos de Casos e Controles , Estudos Retrospectivos , Acidose/complicações , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/terapia , Ácido Láctico
6.
BMJ Case Rep ; 17(1)2024 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-38272521

RESUMO

Crohn's disease patients often need regular home parenteral nutrition (HPN) for intestinal failure due to multiple intestinal resections. Trace elements are necessary for long-term HPN but the requirement volume of iron is undetermined. We describe three patients with Crohn's disease with short bowel syndrome (SBS) who had iron overload as a result of long-term HPN including iron. Serum ferritin level was significantly decreased through depleting intravenous iron administration in all cases. One patient needed regular insulin injection and phlebotomy for diabetes mellitus due to hemochromatosis, and intravenous iron administration had a significant impact on the patient's health. Long-term routine intravenous iron administration should be cautious in SBS patients to avoid the overload.


Assuntos
Doença de Crohn , Sobrecarga de Ferro , Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Oligoelementos , Humanos , Doença de Crohn/complicações , Doença de Crohn/cirurgia , Oligoelementos/uso terapêutico , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/terapia , Ferro , Sobrecarga de Ferro/etiologia
7.
Scand J Gastroenterol ; 59(4): 401-410, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38217349

RESUMO

OBJECTIVES: Comprehensive follow-up data from the largest hospital district in Finland was used to assess hospital-based healthcare resource utilization (HCRU) and expenses, incidence and prevalence, survival, and effect of comorbidities/complications on survival of adult patients with intestinal failure due to short bowel syndrome (SBS-IF). METHODS: This study utilized electronic healthcare data covering all ≥18-year-old patients with SBS-IF at the Hospital District of Helsinki and Uusimaa in Finland between 2010 and 2019. Patients were followed from SBS-IF onset until the end of 2020 or death and compared to birth year and sex-matched control patients without SBS-IF. RESULTS: The study included 77 patients with SBS-IF (cases) and 363 controls. Cases had high HCRU; the cumulative expenses were about tenfold compared to the controls, at the end of the study (€123,000 vs. €14,000 per patient). The expenses were highest during the first year after SBS-IF onset (€53,000 per patient). Of the cases with a median age 62.5 years, 51.9% died during study time. The median survival was 4.4 years from SBS-IF onset and cases died 13.5 times more likely during the follow-up compared to controls. Mortality risk was lower in female cases (hazard ratio (HR) 0.46; 95% confidence intervals (CI) 0.24, 0.9) and higher with presence of comorbidities (Charlson comorbidity index HR 1.55; 95% CI 1.2, 2.0) and mesenteric infarction (HR 4.5; 95% CI 1.95, 10.36). The incidence of adult SBS-IF was 0.6 per 100,000 adults. CONCLUSION: Our study demonstrates a high demand for healthcare support and elevated mortality in adult SBS-IF-patients. Our results suggest that the presence of comorbidities is a key driver for mortality.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Adulto , Humanos , Feminino , Pessoa de Meia-Idade , Adolescente , Síndrome do Intestino Curto/epidemiologia , Síndrome do Intestino Curto/terapia , Gastos em Saúde , Finlândia/epidemiologia , Atenção à Saúde , Estudos Retrospectivos
8.
Nutr Clin Pract ; 39(1): 154-167, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37245122

RESUMO

BACKGROUND: Children with short bowel syndrome (SBS) have complex care needs, most of which are met in the home by family caregivers who may experience a range of stressors unique to this experience. Prior research suggests that parents of children with SBS have poorer health-related quality of life than peers parenting children without health needs, but the mechanisms shaping parent outcomes are understudied. METHODS: A pilot survey was developed using a community-driven research design to measure the impact of disease-specific items on parent-perceived well-being. The cross-sectional survey, which included both closed-ended and open-ended items, was distributed to a convenience sample of parents of children with SBS. Quantitative and qualitative data were integrated for a mixed-methods analysis of how individual items impacted parent well-being. RESULTS: Twenty parents completed the survey. Sleep interruptions, lack of support and resources, and psychological stressors and their mental health implications were more frequently reported as stressors than logistics related to caregiving (e.g., managing therapies and preparing specialized meals). CONCLUSION: The impact of a child's SBS on parent well-being may stem mainly from three interconnected domains: poor sleep and its consequences, lack of access to support and resources, and a range of psychological stressors that affect parent mental health. Understanding the mechanisms through which SBS shapes parent well-being is a necessary first step for developing targeted interventions to support parents and provide family-centered care.


Assuntos
Qualidade de Vida , Síndrome do Intestino Curto , Criança , Humanos , Síndrome do Intestino Curto/terapia , Estudos Transversais , Pais , Poder Familiar/psicologia
9.
J Pediatr ; 265: 113819, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37940084

RESUMO

OBJECTIVE: To evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS). STUDY DESIGN: We used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospital readmissions, readmissions for central line-associated bloodstream infections (CLABSI), and hospital length of stay (LOS) for patients <18 years with SBS hospitalized between January 1, 2006, and October 1, 2015. We analyzed readmissions with recurrent event analysis and analyzed LOS with linear mixed effects modeling. We used a conceptual model to guide our multivariable analyses, adjusting for race, ethnicity, and insurance status. RESULTS: We included 4289 children with 16 347 hospitalizations from 43 institutions. Fifty-seven percent of the children were male, 21% were Black, 19% were Hispanic, and 67% had public insurance. In univariable analysis, children from low-income neighborhoods had a 38% increased risk for all-cause hospitalizations (rate ratio [RR] 1.38, 95% CI 1.10-1.72, P = .01), an 83% increased risk for CLABSI hospitalizations (RR 1.83, 95% CI 1.37-2.44, P < .001), and increased hospital LOS (ß 0.15, 95% CI 0.01-0.29, P = .04). In multivariable analysis, the association between low-income neighborhoods and elevated risk for CLABSI hospitalizations persisted (RR 1.70, 95% CI 1.23-2.35, P < .01, respectively). CONCLUSIONS: Children with SBS from low-income neighborhoods are at increased risk for hospitalizations due to CLABSI. Examination of specific household- and neighborhood-level factors contributing to this disparity may inform equity-based interventions.


Assuntos
Síndrome do Intestino Curto , Criança , Humanos , Masculino , Feminino , Síndrome do Intestino Curto/epidemiologia , Síndrome do Intestino Curto/terapia , Renda , Hospitalização , Tempo de Internação , Atenção à Saúde
10.
Nutr Clin Pract ; 39(1): 227-234, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37898826

RESUMO

Patients with short bowel syndrome (SBS) are often managed by expert multidisciplinary teams. One of the main goals in the management of SBS is the weaning of parenteral support (PS). Weaning of PS removes the risks associated with long-term central line placement and eliminates the need for intestinal transplant. Whereas several papers detail the ongoing care and management of patients with SBS who are dependent on PS, there are few reports discussing the successful weaning of PS in this patient population. We present five case studies examining weaning of PS in adult patients with <60-cm small bowel and partial or full colon who were enrolled in a multidisciplinary adult intestinal rehabilitation program. The case studies demonstrate weaning can occur >2 years after initial assessment and enrollment in an expert program. The ability of the program to focus on individualized care and frequent patient communication is key to PS weaning in patients with SBS.


Assuntos
Intestino Delgado , Síndrome do Intestino Curto , Adulto , Humanos , Estudos Retrospectivos , Nutrição Parenteral , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/complicações
11.
Am J Clin Nutr ; 119(2): 569-577, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38043867

RESUMO

BACKGROUND: Patients with short bowel syndrome (SBS) dependent on home parenteral nutrition (HPN) commonly cycle infusions overnight, likely contributing to circadian misalignment and sleep disruption. METHODS: The objective of this quasi-experimental, single-arm, controlled, pilot trial was to examine the feasibility, safety, and efficacy of daytime infusions of HPN in adults with SBS without diabetes. Enrolled patients were fitted with a continuous glucose monitor and wrist actigraph and were instructed to cycle their infusions overnight for 1 wk, followed by daytime for another week. The 24-h average blood glucose, the time spent >140 mg/dL or <70 mg/dL, and sleep fragmentation were derived for each week and compared using Wilcoxon signed-rank test. Patient-reported quality-of-life outcomes were also compared between the weeks. RESULTS: Twenty patients (mean age, 51.7 y; 75% female; mean body mass index, 21.5 kg/m2) completed the trial. Overnight infusions started at 21:00 and daytime infusions at 09:00. No serious adverse events were noted. There were no differences in 24-h glycemia (daytime-median: 93.00 mg/dL; 95% CI: 87.7-99.9 mg/dL, compared with overnight-median: 91.1 mg/dL; 95% CI: 89.6-99.0 mg/dL; P = 0.922). During the day hours (09:00-21:00), the mean glucose concentrations were 13.5 (5.7-22.0) mg/dL higher, and the time spent <70 mg/dL was 15.0 (-170.0, 22.5) min lower with daytime than with overnight HPN. Conversely, during the night hours (21:00-09:00), the glucose concentrations were 16.6 (-23.1, -2.2) mg/dL lower with daytime than with overnight HPN. There were no differences in actigraphy-derived measures of sleep and activity rhythms; however, sleep timing was later, and light at night exposure was lower with daytime than with overnight HPN. Patients reported less sleep disruptions due to urination and fewer episodes of uncontrollable diarrhea or ostomy output with daytime HPN. CONCLUSIONS: Daytime HPN was feasible and safe in adults with SBS and, compared with overnight HPN, improved subjective sleep without increasing 24-h glucose concentrations. This trial was registered at clinicaltrials.gov as NCT04743960 (https://classic. CLINICALTRIALS: gov/ct2/show/NCT04743960).


Assuntos
Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Glucose , Nutrição Parenteral no Domicílio/efeitos adversos , Projetos Piloto , Síndrome do Intestino Curto/terapia , Sono
12.
JPEN J Parenter Enteral Nutr ; 48(2): 231-238, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38062898

RESUMO

BACKGROUND: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA. METHODS: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015. The cohort was stratified based on the achievement of EA. Statistical testing was completed using t-test, chi-square, Cox proportional hazards regression model, and Kaplan-Meier analysis. RESULTS: EA was achieved in 229 patients. In the multivariable analysis, the percentage of residual LB (hazard ratio [HR] = 1.02; 95% CI = 1.01-1.02) and SB (HR = 1.01; 95% CI = 1.01-1.02) length, presence of the ileocecal valve (HR = 2.02; 95% CI=1.41-2.88), and not coming from a high-volume transplantation center (HR = 2.42; 95% CI = 1.68-3.49) were positively associated with EA, whereas a negative association was seen with the presence of stoma at the time when shortest remnant was documented (HR = 0.72; 95% CI = 0.52-1.00). EA achievement was significantly different between the anatomical subgroups (log-rank test P < 0.001) with an EA rate of 80.4% in infants with ≥50% SB and LB (median time 209 days); 62.5% with ≥50% SB and <50% LB (397 days); 58.3% with <50% SB and ≥50% LB (1192 days), and 25.9% with <50% SB and LB. Necrotizing enterocolitis (NEC) was not associated with a better achievement of EA (NEC vs other etiologies: log-rank test P = 0.33). CONCLUSIONS: Overall, 62% of infants with IF secondary to SBS achieved EA over a mean time of follow-up of 2.3 years. A colon length of >50% can compensate for the loss of small bowel (<50%) and account for similar EA rates as those in children with residual SB > 50%.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Lactente , Humanos , Recém-Nascido , Criança , Síndrome do Intestino Curto/terapia , Estudos Retrospectivos , Nutrição Parenteral , Intestino Delgado
13.
Nutr Clin Pract ; 39(1): 75-85, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37925666

RESUMO

Enteral autonomy is the primary goal of intestinal failure therapy. Intestinal transplantation (ITx) is an option when enteral autonomy cannot be achieved and management complications become life-threatening. The purpose of this review is to summarize existing medical literature related to nutrition requirements, nutrition status, and nutrition support after pediatric ITx. Achieving or maintaining adequate growth after intestinal transplant in children can be challenging because of episodes of rejection that require the use of corticosteroids, occurrences of infection that require a reduction or discontinuation of enteral or parenteral support, and fat malabsorption caused by impaired lymphatic circulation. Nutrient requirements should be assessed and modified regularly based on nutrition status, growth, ventilatory status, wound healing, and the presence of complications. Parenteral nutrition (PN) should be initiated as a continuous infusion early postoperatively. Enteral support should be initiated after evidence of graft bowel function and in the absence of clinical complications. Foods high in simple carbohydrates should be limited, as consumption may result in osmotic diarrhea. Short-term use of a fat-free diet followed by a low-fat diet may reduce the risk of the development of chylous ascites. Micronutrient deficiencies and food allergies are common occurrences after pediatric ITx. Enteral/oral vitamin and mineral supplementation may be required after PN is weaned. Nutrition management of children after ITx can be challenging for all members of the healthcare team. Anthropometric parameters and micronutrient status should be monitored regularly so that interventions to promote growth and prevent or reverse nutrient deficiencies can be implemented promptly.


Assuntos
Apoio Nutricional , Síndrome do Intestino Curto , Criança , Humanos , Intestinos/transplante , Intestino Delgado , Nutrição Parenteral , Micronutrientes , Síndrome do Intestino Curto/terapia
14.
Clin Nutr ESPEN ; 58: 270-276, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38057017

RESUMO

BACKGROUND & AIMS: Short bowel syndrome (SBS) is the leading cause of chronic intestinal failure. The duration of parenteral support (PS) and the long-term micronutrient needs in children with SBS vary, based on their clinical and anatomical characteristics. Our study aimed to review the clinical course and identify high risk patient groups for prolonged PS and long-term micronutrient supplementation. METHODS: A retrospective review was conducted on electronic medical records of children with SBS and chronic intestinal failure who were enrolled in the multidisciplinary intestinal rehabilitation program at Manchester Children's Hospital, UK. Children were included in the review if they required PN for more than 60 days out of 74 consecutive days and had at least 3 years of follow-up. Statistical analysis was performed using IBM SPSS Statistics 24.0. RESULTS: 40 children with SBS achieved enteral autonomy (EA) and 14 remained dependent on PS after 36 months of follow up. Necrotizing enterocolitis was the most common cause for intestinal resection (38.9%) followed by gastroschisis (22.2%), malrotation with volvulus (20.4%), segmental volvulus (9.3%) and long segment Hirschsprung disease (1.9%). Those who achieved EA had significantly longer intestinal length 27.5% (15.0-39.3) than those who remained on PS 6.0% (1.5-12.5) (p < 0.001). Type I SBS was only found in the PS cohort. Median PN dependence was 10.82 months [IQR 5.73-20.78]. Congenital diagnosis was associated with longer PN dependence (21.0 ± 20.0) than acquired (8.7 ± 7.8 months), (p = 0.02). The need for micronutrient supplementation was assessed after the transition to EA; 87.5% children had at least one micronutrient depletion, most commonly Vitamin D (64.1%), followed by iron (48.7%), Vitamin B12 (34.2%), and vitamin E (28.6%). Iron deficiency and vitamin A depletion were correlated with longer PS after multivariate analysis (OR: 1.103, 1.006-1.210, p = 0.037 and OR: 1.048, 0.998-1.102, p = 0.062 respectively). CONCLUSION: In our cohort, small bowel length was the main predictor for EA. Children on longer PS, had more often a congenital cause of resection and were at risk for micronutrient deficiencies in EA.


Assuntos
Insuficiência Intestinal , Micronutrientes , Nutrição Parenteral , Síndrome do Intestino Curto , Oligoelementos , Criança , Humanos , Recém-Nascido , Enteropatias/etiologia , Enteropatias/terapia , Insuficiência Intestinal/etiologia , Insuficiência Intestinal/terapia , Volvo Intestinal/complicações , Micronutrientes/administração & dosagem , Micronutrientes/deficiência , Micronutrientes/uso terapêutico , Estudos Retrospectivos , Síndrome do Intestino Curto/etiologia , Síndrome do Intestino Curto/terapia , Oligoelementos/administração & dosagem , Oligoelementos/deficiência , Oligoelementos/uso terapêutico , Nutrição Parenteral/métodos
15.
Rev Paul Pediatr ; 42: e2023064, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38126603

RESUMO

OBJECTIVE: To analyze the bone health of pediatric patients with short bowel syndrome intestinal failure (SBS-IF). DATA SOURCE: An integrative literature review was performed using the data published in the MEDLINE-PubMed and Scientific Electronic Library Online (SciELO) databases between January 2010 and April 2021, and through a manual search of the reference lists of relevant studies. Studies were included if they assessed bone mineral density by the Dual X-Ray Absorptiometry (DXA) technique, incorporated pediatric patients (up to 20 years of age) with SBS under parenteral nutrition (PN) and were written in English. Eleven primary sources met the inclusion criteria for this study. DATA SYNTHESIS: Pediatric patients with SBS-IF under long-term parenteral nutrition experienced frequent changes in bone metabolism, leading to osteoporotic fractures and growth failure. These patients have deficiencies in multiple nutrients, such as calcium, magnesium, phosphorus, and vitamin D. Consequently, there are variations in the secretion and regulation of the parathyroid hormone. In addition, the pharmacotechnical limitations related to calcium and phosphorus in the PN solution, use of glucocorticoids, and difficulty performing physical activity are risk factors for the development of metabolic bone disease in pediatric patients with SBS-IF. CONCLUSIONS: Low bone mineral density was associated with a high risk of developing osteoporosis, fractures, and growth deficiency in pediatric patients with SBS-IF on PN therapy in the long term.


Assuntos
Cálcio , Síndrome do Intestino Curto , Humanos , Criança , Absorciometria de Fóton , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/complicações , Densidade Óssea/fisiologia , Fósforo
16.
Pediatr Surg Int ; 40(1): 8, 2023 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-37999791

RESUMO

PURPOSE: The purpose of this study was to investigate the autophagy associated with apoptosis in hepatic damage in the short bowel syndrome rat model. METHODS: SD rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. Animals were divided into two groups: TPN plus SBS (Control group) or TPN plus SBS plus intravenous administration of HGF (HGF group). On day 7, the rats were harvested, and hepatocellular injury was evaluated. RESULTS: In an SBS rat model, hepatic steatosis and lobular inflammation were histologically suppressed in the HGF group (p < 0.01). The expression of tumor necrosis factor-α in the HGF group tend to be higher than that in the control group (p = 0.13). The gene expression of transforming Growth Factor-ß in the HGF group was suppressed compared to the control group (p < 0.01). HGF treatment may have an antiapoptotic effect via the intrinsic pathway by caspase 9. Protein expressions of Rubicon (p = 0.03) and p62 (p < 0.01) in the HGF group were found to have increased compared to those in the control group. CONCLUSION: The inhibitory effect of HGF on hepatic steatosis remains unclear, and further studies focusing on the mechanisms of fat accumulation are needed.


Assuntos
Hepatopatias , Síndrome do Intestino Curto , Ratos , Animais , Fator de Crescimento de Hepatócito/genética , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/complicações , Ratos Sprague-Dawley , Modelos Animais de Doenças , Hepatopatias/complicações
17.
Nutrients ; 15(19)2023 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-37836505

RESUMO

INTRODUCTION: The human intestinal tract reacts to extensive resection with spontaneous intestinal adaptation. We analyzed whether gene expression analyses or intestinal permeability (IP) testing could provide biomarkers to describe regulation mechanisms in the intestinal barrier in short bowel syndrome (SBS) patients during adaptive response or treatment with the glucagon-like peptide-2 analog teduglutide. METHODS: Relevant regions of the GLP-2 receptor gene were sequenced. Gene expression analyses and immunohistochemistry were performed from mucosal biopsies. IP was assessed using a carbohydrate oral ingestion test. RESULTS: The study includes 59 SBS patients and 19 controls. Increases in gene expression with teduglutide were received for sucrase-isomaltase, sodium/glucose cotransporter 1, and calcium/calmodulin serine protein kinase. Mannitol recovery was decreased in SBS but elevated with teduglutide (Δ 40%), showed a positive correlation with remnant small bowel and an inverse correlation with parenteral support. CONCLUSIONS: Biomarkers predicting clinical and functional features in human SBS are very limited. Altered specific gene expression was shown for genes involved in nutrient transport but not for genes controlling tight junctions. However, mannitol recovery proved useful in describing the absorptive capacity of the gut during adaptation and treatment with teduglutide.


Assuntos
Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/terapia , Intestino Delgado/patologia , Peptídeo 2 Semelhante ao Glucagon , Biomarcadores , Manitol , Fármacos Gastrointestinais/uso terapêutico
19.
Clin Nutr ; 42(11): 2109-2115, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37751660

RESUMO

RATIONALE: Short Bowel Syndrome (SBS) is the major cause of chronic intestinal failure (IF) and requires parenteral nutrition (PN). After bowel resection, some patients develop spontaneous intestinal adaptations and hyperphagia. Since promoting oral energy intake contributes to PN weaning, this study aims to characterize hyperphagia in patients with SBS and identify its determinants. METHODS: This observational retrospective study included adult patients with SBS who were followed at an expert PN center between 2006 and 2019, with at least 2 separate nutritional assessments. Exclusion criteria were: active neoplasia, alternative treatment for IF or appetite-affecting medication. Resting energy expenditure (REE) was calculated for each patient using the Harris-Benedict equation. Food Intake Ratio (FIR) was calculated by dividing the highest caloric oral intake by REE and hyperphagia was defined as FIR >1.5. RESULTS: Among the 59 patients with SBS included in this study, 82.6% had a FIR >1.5, including 15.5% with a FIR >3. Protein supplied approximately 16% of total energy intake while fat and carbohydrates provided 36% and 48%, respectively. The FIR was independent of gender and whether patients received oral nutrition alone (n = 28) or combined with PN (n = 31). The FIR was also not associated with residual small bowel length, nor the proportion of preserved colon. However, it was negatively correlated with the body mass index (BMI) of these patients (r = -0.533, p < 0.001), whether they had PN support or not. Patients with either a jejuno-colonic (n = 31) or a jejuno-ileal anastomosis (n = 9), had a significantly higher FIR compared to those with an end-jejunostomy (n = 18) (p < 0.05). However, no difference was found in the proportion of calories provided by protein, fat and carbohydrate between the 3 patients groups divided according to the SBS anatomical type. CONCLUSION: A large majority of patients with SBS exhibited a hyperphagia regardless of PN dependence or bowel length, which was inversely correlated with BMI. The presence of the colon in continuity, thus in contact with the nutritional flow, seems to favor a higher oral intake which is beneficial for the nutritional autonomy of patients. This raises the question of a role of colonic microbiota and hormones in this behavior. Finally, this study also revealed an unexpected discrepancy between recommended energy intakes from protein, fat and carbohydrate and the actual intake of patients with SBS.


Assuntos
Síndrome do Intestino Curto , Humanos , Adulto , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/complicações , Estudos Retrospectivos , Hiperfagia/complicações , Colo/cirurgia , Colo/metabolismo , Carboidratos
20.
Surgery ; 173(3): 781-787, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-37534709

RESUMO

BACKGROUND: Children with short bowel syndrome requiring parenteral nutrition are at high risk of recurrent central line-associated bloodstream infections requiring inpatient admission. Predicting responses to treatment at admission could help revise our current treatment algorithm and reduce the length of stay. METHODS: We conducted a retrospective study of all intestinal rehabilitation clinic patients admitted for central line-associated bloodstream infections at our academic hospital between January 2018 and June 2021. Demographic, blood culture, and treatment response data were analyzed. RESULTS: There were 180 separate admissions for central line-associated bloodstream infections, involving 24 patients who met the inclusion and exclusion criteria. The average length of stay was 9.4 days, and 77.1% of the admissions exceeded the predicted length of stay. All patients were treated according to the standard protocols. In the initial blood cultures, 64.4%, 10.6%, and 25% grew a single bacterium, a single fungus, and multiple organisms, respectively. After treatment initiation, 73.3% of single bacterial infections did not show additional positive cultures. However, 78.9% and 48.9% of fungal and multiple organism infections, respectively, had multiple positive cultures, even after initiating treatment. All positive repeat cultures were treated with an ethanol lock for 24 hours in addition to continued treatment. The treatment preserved 90.5% of the catheters. CONCLUSION: Patients experiencing short bowel syndrome admitted for central line-associated bloodstream infections with initial cultures growing fungi or multiple organisms frequently had multiple positive cultures, whereas those with a single bacterial organism did not. Discharging patients who grew a single bacterial organism and placing an initial ethanol lock on those with multiple or fungal organisms could reduce the average length of stay, even if some of these patients would require readmission for line removal.


Assuntos
Bacteriemia , Cateterismo Venoso Central , Síndrome do Intestino Curto , Criança , Humanos , Síndrome do Intestino Curto/terapia , Hemocultura , Cateterismo Venoso Central/efeitos adversos , Tempo de Internação , Estudos Retrospectivos , Bacteriemia/diagnóstico , Bacteriemia/etiologia , Bacteriemia/terapia , Etanol , Hospitais
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