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1.
JPEN J Parenter Enteral Nutr ; 48(4): 495-501, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38400572

RESUMEN

BACKGROUND: Central line-associated bloodstream infections are a major concern for children with intestinal failure and in animal research using parenteral nutrition (PN). In neonatal piglets receiving PN, we compared sepsis, line occlusions, line replacements, mortality, and costs with and without the use of a 4%-tetrasodium ethylenediaminetetraacetic acid (T-EDTA) locking solution. METHODS: We performed a retrospective review of piglets with a central venous jugular catheter enrolled in 14-day exclusive PN (TPN) trials or in 7-day short bowel syndrome (SBS) trials, before and after initiation of T-EDTA. Lines were locked with a 1-ml solution for 2 h daily (T-EDTATPN, n = 17; T-EDTASBS, n = 48) and compared with our prior standard of care using 1.5-ml heparin flushes twice daily (CONTPN, n = 34; CONSBS, n = 48). Line patency and signs of sepsis were checked twice daily. Jugular catheters were replaced for occlusions whenever possible. Humane end points were used for sepsis not responding to antibiotic treatment or unresolved catheter occlusions. RESULTS: Compared with CON, sepsis was reduced using T-EDTA, significantly for TPN (P = 0.006) and with a trend for SBS piglets (P = 0.059). Line occlusions necessitating line changes were reduced 15% in TPN studies (P = 0.16), and no line occlusions occurred for T-EDTA SBS piglets. CONCLUSION: In our neonatal piglet research, use of T-EDTA locking solution decreased sepsis and, although not statistically significant, reduced occlusions requiring line replacements. Given the expense of animal research, adding a locking solution must be cost-effective, and we were able to show that T-EDTA significantly reduced total research costs and improved animal welfare.


Asunto(s)
Animales Recién Nacidos , Cateterismo Venoso Central , Catéteres Venosos Centrales , Ácido Edético , Sepsis , Animales , Estudios Retrospectivos , Porcinos , Ácido Edético/administración & dosificación , Ácido Edético/farmacología , Cateterismo Venoso Central/efectos adversos , Infecciones Relacionadas con Catéteres , Nutrición Parenteral/métodos , Síndrome del Intestino Corto/terapia , Modelos Animales de Enfermedad
2.
JPEN J Parenter Enteral Nutr ; 48(2): 231-238, 2024 02.
Artículo en Inglés | MEDLINE | ID: mdl-38062898

RESUMEN

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%.


Asunto(s)
Insuficiencia Intestinal , Síndrome del Intestino Corto , Lactante , Humanos , Recién Nacido , Niño , Síndrome del Intestino Corto/terapia , Estudios Retrospectivos , Nutrición Parenteral , Intestino Delgado
3.
JPEN J Parenter Enteral Nutr ; 48(1): 47-56, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-37465871

RESUMEN

BACKGROUND: Central line-associated bloodstream infections (CLABSI) are a serious complication in children with intestinal failure. This study assessed the incremental costs of 4% tetrasodium ethylenediaminetetraacetic acid (EDTA) compared with taurolidine lock and heparin lock per quality-adjusted life-year (QALY) gained in children with intestinal failure from the healthcare payer and societal perspective. METHODS: A Markov cohort model of a 1-year-old child with intestinal failure was simulated until the age of 17 years (time horizon), with a cycle length of 1 month. The health outcome measure was QALYs, with results expressed in terms of incremental costs and QALYs. Model parameters were obtained from published literature and institutional data. Deterministic, probabilistic, and scenario sensitivity analyses were performed. RESULTS: 4% Tetrasodium EDTA was dominant (more effective and less expensive) compared with taurolidine and heparin, yielding an additional 0.17 QALYs with savings of CAD$88,277 compared with heparin, and an additional 0.06 QALYs with savings of CAD$52,120 compared with taurolidine lock from the healthcare payer perspective. From the societal perspective, 4% tetrasodium EDTA resulted in savings of CAD$90,696 compared with heparin and savings of CAD$36,973 compared with taurolidine lock. CONCLUSIONS: This model-based analysis indicates that 4% tetrasodium EDTA can be considered the optimal strategy compared with taurolidine and heparin in terms of cost-effectiveness. The decision uncertainty can be reduced by conducting further research on the model input parameters. An expected value of perfect information analysis can identify what model input parameters would be most valuable to focus on.


Asunto(s)
Insuficiencia Intestinal , Sepsis , Niño , Humanos , Adolescente , Lactante , Ácido Edético/uso terapéutico , Análisis Costo-Beneficio , Heparina/uso terapéutico
4.
Clin Nutr ; 42(12): 2363-2371, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37862822

RESUMEN

BACKGROUND & AIMS: A growing proportion of children with short bowel syndrome (SBS) remain dependent on long-term parenteral nutrition (PN). Teduglutide offers the potential for more children to decrease PN support and achieve enteral autonomy (EA), but at a significant expense. This study aims to assess the incremental costs of teduglutide plus standard of care compared to standard of care alone in weaning PN support per quality-adjusted life year (QALY) gained in children with SBS. METHODS: This is a cost-utility analysis comparing teduglutide with standard of care alone in children with SBS. A microsimulation model of children with SBS on PN aged 1-17 years was constructed over a time horizon of six years, with a cycle length of one month. The study adopted the healthcare system and societal payer perspectives in Ontario, Canada. The health outcome measure was QALYs, with results expressed in terms of incremental costs and QALYs. Scenario analyses were performed to examine the effects of different time horizons, timing of teduglutide initiation, and modeling cost of teduglutide based on pediatric weight-dosing. RESULTS: Incremental healthcare system costs for teduglutide compared to standard of care were CAD$441,314 (95% CI, 414,006 to 441,314) and incremental QALYs were 1.80 (95% CI, 1.70 to 1.89) resulting in an incremental cost-effectiveness ratio (ICER) of CAD$285,334 (95% CI, 178,209 to 392,459) per QALY gained. Incremental societal costs were CAD$418,504 (95% CI, 409,487 to 427,522) and incremental societal QALYs were 1.91 (95% CI, 1.85 to 1.98) resulting in an ICER of CAD$261,880 (95% CI, 136,887 to 386,874) per QALY gained. Scenario analysis showed that teduglutide was cost-effective when it was started two years after intestinal resection (ICER CAD$48,741, 95% CI, 17,317 to 80,165) and when its monthly cost was adjusted using weight-based dosing, avoiding wastage of the remaining 5 mg dose vial (Teduglutide dominated over SOC as the less costly and most effective strategy). CONCLUSIONS: Although teduglutide was not cost-effective in weaning PN support in children with SBS, starting teduglutide once natural intestinal adaptation is reduced and adjusting its monthly cost to reflect cost by volume as dictated by weight-based dosing rendered the intervention cost-effective relative to standard of care. These results indicate the potential for clinicians to re-assess optimal time for initiation of teduglutide after intestinal resection, drug manufacturers to consider the use of multi-dose or paediatric-dose vials, and the opportunity for decision-makers to re-evaluate teduglutide funding.


Asunto(s)
Síndrome del Intestino Corto , Humanos , Niño , Síndrome del Intestino Corto/tratamiento farmacológico , Análisis Costo-Beneficio , Destete , Fármacos Gastrointestinales/uso terapéutico , Nutrición Parenteral , Ontario
5.
JPEN J Parenter Enteral Nutr ; 47(7): 930-937, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37392380

RESUMEN

BACKGROUND: Reports of essential fatty acid deficiency (EFAD) in patients receiving parenteral nutrition (PN) and a composite lipid (mixed oil intravenous lipid emulsion [MO ILE]) are predominantly when managed by lipid restriction. The objective of this study was to determine the prevalence of EFAD in patients with intestinal failure (IF) who are PN dependent without lipid restriction. METHODS: We retrospectively evaluated patients, ages 0-17 years, followed by our intestinal rehabilitation program between November 2020 and June 2021 with PN dependency index (PNDI) of >80% on a MO ILE. Demographic data, PN composition, PN days, growth, and plasma fatty acid profile were collected. A plasma triene-tetraene (T:T) ratio >0.2 indicated EFAD. Summary statistics and Wilcoxon rank sum test evaluated to compare between PNDI category and ILE administration (grams/kilograms/day). P < 0.05 was considered significant. RESULTS: Twenty-six patients (median age, 4.1 years [interquartile range (IQR) = 2.4-9.6]) were included. The median duration of PN was 1367 days (IQR = 824-3195). Sixteen patients had a PNDI of 80%-120% (61.5%). Fat intake for the group was 1.7 g/kg/day (IQR = 1.3-2.0). The median T:T ratio was 0.1 (IQR = 0.1-0.2) with no values >0.2. Linoleic and arachidonic acid were low in 85% and 19% of patients, respectively; however, Mead acid was normal in all patients. CONCLUSION: This report is the largest to date on the EFA status of patients with IF on PN. These results suggest that, in the absence of lipid restriction, EFAD is not a concern when using MO ILEs in children receiving PN for IF.


Asunto(s)
Insuficiencia Intestinal , Humanos , Niño , Preescolar , Estudios Retrospectivos , Ácidos Grasos Esenciales , Aceites de Pescado , Nutrición Parenteral/métodos , Emulsiones Grasas Intravenosas , Aceite de Soja
6.
J Pediatr Gastroenterol Nutr ; 77(4): 558-564, 2023 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-37434279

RESUMEN

OBJECTIVES: Data on the relationship between body composition (BC) and physical activity (PA) in children with intestinal failure (IF) are lacking. The objectives were to collect data on PA and BC in children with IF, both parenterally and enterally fed, and to assess the relationship between PA and BC. METHODS: Cross-sectional study in children 5-18 years with IF including those receiving parenteral nutrition (PN) and those fully enterally fed. PA levels were measured using accelerometry. BC was measured by dual-energy X-ray absorptiometry. Data were compared to age- and sex-matched population norms using t tests. Regression analysis assessed the relationship between BC and PA. RESULTS: Fifty-eight children with IF (38 males), mean (SD) age of 10.0 (3.5) years, 20 dependent on PN were included. Patients with IF had significantly fewer steps per day ( P ≤ 0.001) compared with literature controls, with a mean (SD) of 7,972 (3,008) and 11,749 (1,106), respectively. There were no significant differences between patients receiving PN and those enterally fed, but both groups were significantly less active than literature controls ( P < 0.001). Patients with IF had higher fat mass and lower fat-free mass compared to literature controls ( P = 0.008). PA had a significant effect on BC ( r2 = 0.32, P < 0.001). CONCLUSIONS: Children with IF, those receiving PN and those fully enterally fed, are at risk of decreased PA and altered BC. PA should be part of ongoing rehabilitation and management to optimize outcomes.


Asunto(s)
Insuficiencia Intestinal , Masculino , Niño , Humanos , Estudios Transversales , Nutrición Parenteral , Composición Corporal , Ejercicio Físico
7.
JPEN J Parenter Enteral Nutr ; 47(8): 1038-1046, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37416983

RESUMEN

BACKGROUND: Children with intestinal failure without liver disease may be given soy-based lipid emulsion (SLE) or mixed lipid emulsion (MLE; containing soy, medium-chain triglyceride, olive, and/or fish oils). Both differ in essential fatty acid content: MLE has added arachidonic acid (AA) and docosahexaenoic acid (DHA). The aim of this study, in neonatal piglets, was to compare serum and tissue fatty acid composition when the emulsions were given at unrestricted doses. METHODS: We compared SLE (n = 15) and MLE (n = 15) at doses of 10-15 g/kg/day in parenteral nutrition (PN). On day 14 we collected serum and tissues. Using gas-liquid chromatography, percentage fatty acids were measured in serum, brain, and liver phospholipid. Comparisons were made to reference values from litter-matched controls (n = 8). RESULTS: Comparing median values, linoleic acid (LA) was lower for MLE vs SLE in serum (-27%), liver (-45%), and brain (-33%) (P < 0.001). AA was lower for MLE in serum (-25%), liver (-40%), and brain (-10%). DHA was higher for MLE in serum (+50%), liver (+200%), and brain (+10%). AA levels were lower for MLE vs control piglets in serum (-81%), liver (-63%), and brain (-9%). DHA levels were higher in serum (+41%), liver (+38%), and brain (+19%). CONCLUSION: This study in piglets has shown that, at unrestricted doses, MLE treatment is associated with low serum and tissue AA compared with SLE and healthy litter-matched controls. Although not yet proven, low tissue AA levels may have functional consequences, and these data support current practice avoiding MLE dose restriction.


Asunto(s)
Emulsiones Grasas Intravenosas , Ácidos Grasos , Niño , Animales , Humanos , Porcinos , Emulsiones Grasas Intravenosas/química , Nutrición Parenteral/métodos , Aceites de Pescado/química , Fosfolípidos , Ácidos Docosahexaenoicos , Ácido Araquidónico , Ácidos Grasos Esenciales , Aceite de Soja
8.
Am J Emerg Med ; 71: 217-224, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37453161

RESUMEN

OBJECTIVE: to determine diagnostic accuracy of an US-MRI clinical diagnostic pathway to detect appendicitis in the emergency department (ED). STUDY DESIGN: prospective cohort study of 624 previously healthy children 4-17 years old undergoing US for suspected appendicitis and clinical re-assessment. Children with non-diagnostic USs and persistent appendicitis concern/conclusive US-reassessment discrepancies underwent ultra-rapid MRI (US-MRI pathway), interpreted as positive, negative or non-diagnostic. Cases with missed appendicitis, negative appendectomies, and CT utilization were considered clinically diagnostically inaccurate. Primary outcome was the proportion of accurate diagnoses of appendicitis/lack thereof by the pathway. RESULTS: 150/624 (24%) children had appendicitis;255 USs (40.9%) were non-diagnostic. Of 139 US-MRI pathway children (after 117 non-diagnostic and 22 conclusive USs), 137 [98.6%; 95% CI 0.96-1.00] had clinically accurate outcomes (1 CT, 1 negative appendectomy): sensitivity 18/18 [100%], specificity 119/121 [98.3%], positive predictive value 18/20 [90.5%], negative predictive value 119/119 [100%]. MRI imaging accuracy was 134/139 (96.4%); 3 MRIs were non-diagnostic (no appendicitis). In the overall algorithm, 616/624 [98.7% (0.97-0.99)] patients had accurate outcomes: 147/150 (98.0%) appendicitis cases had confirmatory surgeries (3 CTs) and 469/474 (98.9%) appendicitis-negative children had no surgery/CT. CONCLUSION: this study demonstrated high clinical accuracy of the US-rapid-MRI pathway in suspected pediatric appendicitis after non-diagnostic US.


Asunto(s)
Apendicitis , Niño , Humanos , Preescolar , Adolescente , Estudios Prospectivos , Apendicitis/diagnóstico por imagen , Apendicitis/cirugía , Imagen por Resonancia Magnética/métodos , Apendicectomía , Valor Predictivo de las Pruebas , Ultrasonografía , Sensibilidad y Especificidad , Estudios Retrospectivos
9.
JPEN J Parenter Enteral Nutr ; 47(7): 920-929, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37355855

RESUMEN

BACKGROUND: The objective of the study was to compare bioelectrical impedance analysis (BIA) and skinfolds with dual energy x-ray absorptiometry (DXA) in the assessment of body composition of children with intestinal failure. DXA is the reference method for body composition assessment in clinical settings. METHODS: Children aged 1-18 years with intestinal failure whohave DXA as part of routine clinical monitoring were eligible. BIA measured total body water on the same day as DXA. Skinfold measurements were taken at four sites: triceps, biceps, subscapular, and suprailiac. Percentage of fat mass (%FM) and fat-free mass (%FFM) were derived from resistance and reactance measured by BIA by using age-specific equations. Percentage of FM was calculated from skinfold measures by using age-specific equations. Data on patient characteristics, intestinal failure-related factors, and feeding method were collected. Paired t test examined differences in %FM and %FFM and Bland-Altman analysis determined the agreement between BIA, skinfolds, and DXA. Marginal linear model assessed the effect of age, sex, and feeding method on the difference in body composition obtained between DXA and BIA and between DXA and skinfolds. RESULTS: Sixty-eight children with intestinal failure, mean age 8.9 ± 4.2 years, were studied. There was no difference between %FFM and %FM obtained by DXA and BIA (P = 0.26), with a mean bias (95% CI) of -0.69 (-1.9 to 0.5) for %FFM. Sex and age were individually and jointly associated with the bias observed between DXA and BIA (P < 0.05). Skinfold and DXA measurements were significantly different (P < 0.05). CONCLUSIONS: BIA is an acceptable clinical tool for assessing body composition in pediatric intestinal failure.


Asunto(s)
Insuficiencia Intestinal , Humanos , Niño , Preescolar , Adolescente , Impedancia Eléctrica , Composición Corporal , Absorciometría de Fotón/métodos , Sesgo , Índice de Masa Corporal , Reproducibilidad de los Resultados
10.
Nutr Clin Pract ; 38 Suppl 1: S98-S113, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37115028

RESUMEN

Intestinal failure (IF) is a complex medical condition that is caused by a constellation of disorders, resulting in the gut's inability to adequately absorb fluids and nutrients to sustain hydration, growth, and survival, thereby requiring the use of parenteral fluid and/or nutrition. Significant advancements in intestinal rehabilitation have resulted in improved survival rates for individuals with IF. There are distinct differences, however, related to etiology, adaptive potential and complications, and medical and surgical management when comparing children with adults. The purpose of this review is to contrast the similarities and differences between these two distinct groups and provide insight for future directions, as a growing population of pediatric patients will cross into the adult world for IF management.


Asunto(s)
Enfermedades Intestinales , Insuficiencia Intestinal , Síndrome del Intestino Corto , Niño , Adulto , Humanos , Síndrome del Intestino Corto/rehabilitación , Intestinos , Nutrición Parenteral , Tasa de Supervivencia , Enfermedades Intestinales/terapia
11.
Clin Nutr ; 42(2): 136-142, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36563562

RESUMEN

BACKGROUND & AIMS: Non-invasive monitoring of intestinal failure (IF) associated liver disease is an ongoing challenge in children with IF. Our objective was to develop a combined algorithm of clinical, transient elastography (TE) and biochemical parameters to identify liver fibrosis in this population. METHODS: A retrospective cohort study of IF patients followed by our intestinal rehabilitation program between November 2015 to October 2019. Patients with a liver biopsy and TE were included. Demographic and liver function tests were collected. Fibrosis on liver biopsies was graded using the modified Scheuer score. Decision tree based algorithms classified low (F0-F1) versus high (F2-F4) fibrosis scores based on a combination of TE, biochemical and demographic parameters, using 6-fold classification error, sensitivity and specificity cross-validation (CV) scores. RESULTS: 42 patients (74% male, median age 7.6 (4.6; 42.7) months) were evaluated. Median length of PN therapy was 182 (121; 556) days. High fibrosis was present in 40.5% with a median TE of 12.1 (6.7; 12.9) kPa in high fibrosis children. An algorithm, based on cut-off values for TE of 11.3 kPa and AST of 40 U/L, and grouping of the underlying etiology resulted in a correct classification of 88.1% of the pathology scores; with sensitivity 0.82 (95% CI 0.57; 0.96), specificity 0.92 (95% CI 0.74; 0.99), positive predictive value 0.88 (95% CI 0.64; 0.96) and negative predictive value 0.88 (95% CI 0.73; 0.96). The CV classification error was 28.6%, CV sensitivity 72.2% and CV specificity 75.5%. CONCLUSIONS: This algorithm shows promising results that could simplify non-invasive monitoring of liver fibrosis in children with IF. Validation in additional IF cohorts is needed.


Asunto(s)
Diagnóstico por Imagen de Elasticidad , Insuficiencia Intestinal , Humanos , Niño , Diagnóstico por Imagen de Elasticidad/métodos , Estudios Retrospectivos , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/diagnóstico por imagen , Hígado/diagnóstico por imagen , Hígado/patología
12.
J Clin Psychol Med Settings ; 30(3): 589-605, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-36371790

RESUMEN

OBJECTIVES: Despite a focus on neurocognition in pediatric intestinal failure (IF) to date, we examined social-emotional and adaptive functioning. METHODS: Children (N = 63) in our IF rehabilitation program underwent neuropsychological assessments including caregiver- and teacher-reported questionnaires. Results were compared to norms using z-tests. Caregiver and teacher reports were compared using t tests. Medical and demographic factors were examined in an exploratory manner using correlation and targeted regression analyses, adjusting for gestational age and full-scale IQ. RESULTS: Caregiver and teacher reports indicated poorer executive, internalizing, behavioral, and adaptive functioning compared to norms. Teachers reported more executive dysfunctions than caregivers. Necrotizing enterocolitis diagnosis predicted internalizing emotional problems. Immigrant status predicted poorer social and practical adaptive functioning. Living with biological parents predicted fewer externalizing emotional and behavioral problems. CONCLUSIONS: The group displayed social-emotional and adaptive functioning concerns. Identifying medical and demographic risks can allow for screening and intervention.


Asunto(s)
Trastornos de la Conducta Infantil , Insuficiencia Intestinal , Problema de Conducta , Niño , Humanos , Recién Nacido , Emociones , Padres/psicología , Trastornos de la Conducta Infantil/diagnóstico , Trastornos de la Conducta Infantil/psicología
13.
JPEN J Parenter Enteral Nutr ; 47(3): 342-353, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36336350

RESUMEN

BACKGROUND: Improved survival rates for children with intestinal failure (IF) have resulted in an increased population of children receiving long-term parenteral nutrition (PN). Our objective was to determine burden on caregivers of children with IF receiving long-term PN. METHODS: We performed a cross-sectional study of caregivers of children with IF receiving long-term PN in our intestinal rehabilitation program. A healthy comparison group matched on age of the child was enrolled. All participants completed standardized questionnaires, including the Parental Stress Index - Short Form (PSI-SF), Hospital Anxiety and Depression Scale (HADS), and PedsQL Family Impact Module (PedsQL FIM). Univariate analysis was completed using a Student t test and chi-square, with an alpha value of <0.05 considered significant. RESULTS: Thirty-eight caregivers of children with IF and 29 caregivers of healthy children consented, with response rates of 89% and 96.5%, respectively. Our study demonstrated increased stress for caregivers compared with comparison parents (PSI-SF total score of 83 [SD = 26.8] vs 62.9 [SD = 13.5]; P < 0.01). Caregivers had increased anxiety (HADS anxiety score of 9.3 [SD = 4.8] vs 6.7 [SD = 3.2]; P = 0.02) and higher depression scores (HADS depression score of 6.3 [SD = 4.3] vs 4.1 [SD = 2.6]; P = 0.02) compared with the comparison group. Caregivers of children with IF demonstrated decreased health-related quality of life (HRQoL) (reduced PedsQL FIM total score of 50.6 [SD = 18.2] vs 84.1 [SD = 20.5]; P < 0.01). CONCLUSIONS: Our results demonstrated significant burden of care in caregivers of children with IF receiving long-term PN, with elevated stress, anxiety, and depression and decreased HRQoL.


Asunto(s)
Insuficiencia Intestinal , Calidad de Vida , Humanos , Niño , Estudios Transversales , Cuidadores , Depresión/epidemiología , Ansiedad , Encuestas y Cuestionarios , Nutrición Parenteral
14.
Am J Transplant ; 22(11): 2608-2615, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35833730

RESUMEN

Deciding which patients would benefit from intestinal transplantation (IT) remains an ethical/clinical dilemma. New criteria* were proposed in 2015: ≥2 intensive care unit (ICU) admissions, loss of ≥3 central venous catheter (CVC) sites, and persistently elevated conjugated bilirubin (CB ≥ 75 µmol/L) despite 6 weeks of lipid modification strategies. We performed a retrospective, international, multicenter validation study of 443 children (61% male, median gestational age 34 weeks [IQR 29-37]), diagnosed with IF between 2010 and 2015. Primary outcome measure was death or IT. Sensitivity, specificity, NPV, PPV, and probability of death/transplant (OR, 95% confidence intervals) were calculated for each criterion. Median age at IF diagnosis was 0.1 years (IQR 0.03-0.14) with median follow-up of 3.8 years (IQR 2.3-5.3). Forty of 443 (9%) patients died, 53 of 443 (12%) were transplanted; 11 died posttransplant. The validated criteria had a high predictive value of death/IT; ≥2 ICU admissions (p < .0001, OR 10.2, 95% CI 4.0-25.6), persistent CB ≥ 75 µmol/L (p < .0001, OR 8.2, 95% CI 4.8-13.9). and loss of ≥3 CVC sites (p = .0003, OR 5.7, 95% CI 2.2-14.7). This large, multicenter, international study in a contemporary cohort confirms the validity of the Toronto criteria. These validated criteria should guide listing decisions in pediatric IT.


Asunto(s)
Unidades de Cuidados Intensivos , Intestinos , Niño , Humanos , Masculino , Recién Nacido , Lactante , Femenino , Estudios Retrospectivos , Resultado del Tratamiento , Intestinos/trasplante , Estudios de Cohortes
15.
J Pediatr Gastroenterol Nutr ; 75(4): 438-443, 2022 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-35830733

RESUMEN

OBJECTIVES: With increasing number of children with intestinal failure (IF) on long-term parenteral nutrition (PN), this study assesses the impact of IF on muscle strength, speed, and agility and body composition (BC), identifying clinical factors that may predict impairment. METHODS: Cross-sectional study in children 5-18 years with IF on PN. Assessments included Bruininks-Oseretsky Test of Motor Proficiency-2 strength and agility subtest (BOT-2), and grip strength. BC data from dual-energy x-ray absorptiometry (DXA) measurements and clinical variables were collected by chart abstraction. Data were compared to age and sex matched controls and population norms. RESULTS: Twenty-one children with IF (14 males), median age 8.33 (IQR: 6.96-11.04) years and 33 controls (20 males), 8.25 (6.67-10.79) years were included. Strength and agility ( P < 0.001) and grip strength ( P = 0.001) differed between groups. Nine of 21 (43%) of children with IF scored >1 standard deviation (SD) below mean on BOT-2 and 13 of 21 (62%) had grip strength >1 SD below mean. DXA measurements showed 10 of 18 (56%) of children had lower fat-free mass (FFM)% and higher fat mass (FM)% than reference norms. Decreased FFM% was associated with lower BOT-2 scores ( r = 0.479; P = 0.044) and grip strength scores >1 SD below mean ( P = 0.047). Additional clinical factors significantly impacting strength and agility included prematurity, height, hospitalizations, sepsis, and small bowel length. CONCLUSIONS: Children with IF are at risk of decreased muscle strength and agility, along with altered BC. Ongoing medical, nutritional, and rehabilitation intervention is vital to optimize outcomes.


Asunto(s)
Insuficiencia Intestinal , Absorciometría de Fotón , Composición Corporal , Niño , Estudios Transversales , Humanos , Masculino , Fuerza Muscular , Nutrición Parenteral
16.
Semin Pediatr Surg ; 31(3): 151175, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35725052

RESUMEN

Intestinal failure (IF) secondary to short bowel syndrome is a challenging and complex medical condition with significant risk for surgical and medical complications. Significant advancements in the care of this patient population have led to improved survival rates. Due to their intensive medical needs children with IF are at risk for long-term complications that require comprehensive management and close monitoring. The purpose of this paper is to review the available literature emphasizing the surgical aspects of care for children with IF secondary to short bowel syndrome. A key priority in the surgical care of this patient population includes strategies to preserve available bowel and maximize its function. Utilization of novel surgical techniques and autologous bowel reconstruction can have a significant impact on children with IF secondary to short bowel syndrome related to the function of their bowel and ability to achieve enteral autonomy. It is also important to understand the potential long-term complications to ensure strategies are put in place to mitigate risk with early detection to improve long-term outcomes.


Asunto(s)
Insuficiencia Intestinal , Síndrome del Intestino Corto , Niño , Humanos , Intestino Delgado/cirugía , Intestinos , Nutrición Parenteral , Síndrome del Intestino Corto/complicaciones , Síndrome del Intestino Corto/diagnóstico , Síndrome del Intestino Corto/terapia
17.
J Pediatr ; 250: 75-82.e3, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35660493

RESUMEN

OBJECTIVE: To measure the time that caregivers spend on tasks related to providing care to their child with intestinal failure receiving home parenteral nutrition (PN). STUDY DESIGN: We conducted an exploratory cross-sectional study of caregivers of children with intestinal failure receiving long-term PN followed by our intestinal rehabilitation program. Caregivers completed a daily diary of care-related tasks. Data were analyzed using descriptive statistics. Exploratory models were completed to evaluate factors that influenced the amount of time that caregivers spent providing care. SAS University Edition 2018 (SAS Institute, Cary, NC) was used for data analysis with a P value of less than .05 considered significant. RESULTS: Thirty-four caregivers of children with intestinal failure consented with response rates of 85%. The mean age of the primary caregiver was 37 ± 7.9 years of age with 97% being the child's mother. The median PN exposure was 1239 days (IQR, 432-3012). Caregivers reported a median of 29.2 hours per week (IQR, 20.8-45.7 hours per week) of direct medical care. The majority of time was spent on providing PN and care of the central venous catheter (6.1 hours; IQR, 5.2-8.8). CONCLUSIONS: Caregivers of children with intestinal failure receiving long-term PN provide a significant amount of care to ensure their child remains healthy at home. The most significant amounts of time were spent on the administration of the PN and care of the central venous catheter.


Asunto(s)
Enfermedades Intestinales , Insuficiencia Intestinal , Nutrición Parenteral en el Domicilio , Humanos , Niño , Adulto , Cuidadores , Estudios Transversales , Atención al Paciente , Enfermedades Intestinales/terapia
18.
J Pediatr Gastroenterol Nutr ; 74(5): 575-581, 2022 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-35149643

RESUMEN

BACKGROUND AND AIMS: The initial description of a heterozygous dominant ACTG2 variant in familial visceral myopathy was followed by the identification of additional variants in other forms of intestinal dysmotility disorders. we aimed to describe the diverse phenotype of this newly reported and rare disease. METHODS: Report of 4 new patients, and a systematic review of ACTG2-related disorders. we analyzed the population frequency and used in silico gene damaging predictions. Genotype-phenotype correlations were explored. RESULTS: One hundred three patients (52% girls), from 14 publications, were included. Twenty-eight unique variants were analyzed, all exceedingly rare, and 27 predicted to be highly damaging. The median Combined Annotation Dependent Depletion (CADD) score was 29.2 (Interquartile range 26.3-29.4). Most patients underwent abdominal surgery (66%), about half required intermittent bladder catheterization (48.5%), and more than half were parenteral nutrition (PN)-dependent (53%). One-quarter of the patients died (25.7%), and 6 required transplant (5.8%). Girls had a higher rate of microcolon (P  = 0.009), PN dependency (P = 0.003), and death/transplant (P = 0.029) compared with boys, and early disease onset (<2 years of age) was associated with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) features. There was no statistical association between disease characteristics and CADD scores. CONCLUSIONS: Damaging ACTG2 variants are rare, often associated with MMIHS phenotype, and overall have a wide phenotypic variation. Symptoms usually present in the perinatal period but can also appear at a later age. The course of the disease is marked by frequent need for surgical interventions, PN support, and mortality. Poor outcomes are more common among girls with ACTG2 variants.


Asunto(s)
Anomalías Múltiples , Seudoobstrucción Intestinal , Anomalías Múltiples/diagnóstico , Actinas/genética , Colon/anomalías , Femenino , Humanos , Seudoobstrucción Intestinal/diagnóstico , Seudoobstrucción Intestinal/genética , Masculino , Fenotipo , Embarazo , Vejiga Urinaria/anomalías
19.
JPEN J Parenter Enteral Nutr ; 46(6): 1393-1403, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35043436

RESUMEN

BACKGROUND: Short-bowel syndrome (SBS) in neonates is associated with microbial dysbiosis due to intestinal surgery, prolonged hospitalization, enteral nutrition, and repeated antibiotic exposure. Sepsis and liver disease, leading causes of morbidity and mortality in SBS, may relate to such intestinal dysbiosis. We investigated the safety and feasibility of fecal microbial transplant (FMT) to alter intestinal microbial composition in SBS piglets. METHODS: Following a 75% distal small-intestinal resection, piglets were fed parenteral nutrition with an elemental diet and randomized to saline (SAL; n = 12) or FMT (n = 12) treatments delivered by gastric tube on day 2 (d2). The FMT donor was a healthy adult pig. Comparisons were also made to healthy sow-fed littermate controls (SOW; n = 6). Stool samples were collected daily, and tissue samples were collected at baseline and termination. Microbial DNA was extracted from stool and analyzed using 16S ribosomal RNA sequencing. RESULTS: All piglets survived to the end point. On d2-d4, FMT piglets had some differences in microbiota composition compared with SAL, SOW, and donor counterparts. Between base and term, there were transitory changes to alpha and beta diversity in FMT and SAL. CONCLUSION: FMT treatment in postsurgical neonatal piglets with SBS appears safe, with no increase in sepsis and no mortality. In SBS piglets, FMT induced transient changes to the intestinal microbiota. However, these changes did not persist long-term.


Asunto(s)
Sepsis , Síndrome del Intestino Corto , Animales , Disbiosis , Trasplante de Microbiota Fecal , Heces , Intestinos , Sepsis/terapia , Síndrome del Intestino Corto/terapia , Porcinos
20.
JPEN J Parenter Enteral Nutr ; 46(7): 1602-1613, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35088428

RESUMEN

BACKGROUND: Children with intestinal failure (IF) receiving long-term parenteral nutrition (PN) require significant medical care, including high-risk procedures such as accessing a central venous catheter, with the majority provided by family caregivers in the home. This study sought to understand the experiences of family caregivers. METHODS: This was a qualitative study of family caregivers of children with IF. Participants were recruited from an intestinal rehabilitation program to participate in virtual focus groups, which were recorded and transcribed. Thematic analysis was used to capture and describe experiences. RESULTS: Thirteen caregivers providing care to 11 children participated in three virtual focus groups held between May and June 2020. Data analysis revealed five primary themes: (1) caregiving as a 24/7 commitment, (2) facing constant risk of death, (3) chronic illness creates difficult feelings and emotions, (4) effects on all aspects of family life, and (5) adapting and functioning as a family. The findings of this study pair well with the biopsychosocial model highlighting the need for multifaceted assessment and supports. CONCLUSION: The impact of caring for a child with IF receiving long-term PN is significant for caregivers and their families and has implications for all aspects of their lives. A better understanding of the impact on caregivers and their coping strategies lays the groundwork for the optimization of quality of life for caregivers and their family.


Asunto(s)
Cuidadores , Insuficiencia Intestinal , Adaptación Psicológica , Cuidadores/psicología , Niño , Humanos , Nutrición Parenteral , Investigación Cualitativa , Calidad de Vida
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