Trends in Pediatric Intestinal Failure: A Multicenter, Multinational Study

Gattini, Daniela; Roberts, A; Wales, Paul W.; Beath, S. V.; Evans, Helen; Hind, Jonathan; Mercer, David F.; Wong, Theodoric; Yap, Jason; Belza, Christina; Huysentruyt, Koen; Avitzur, Yaron

doi:10.1016/j.jpeds.2021.06.025

Cited by 39 publications

(69 citation statements)

References 49 publications

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“…Many IRP’s across North America and Europe are reporting >90% long-term survival in children with IF (3,27), however at least 20% continue to depend on long-term PN support at 72 months after diagnosis (4). While children without end-stage disease may appear to have typical physical function during routine clinic examination, a thorough and focused examination of muscle function and strength is important, and in many cases will reveal deficiencies.…”

Section: Discussionmentioning

confidence: 99%

Muscle Strength, Agility, and Body Composition in Children With Intestinal Failure on Parenteral Nutrition

Patterson

Betts

et al. 2022

Journal of Pediatric Gastroenterology &Amp; Nutrition

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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 dualenergy 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.

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Section: Discussionmentioning

confidence: 99%

Muscle Strength, Agility, and Body Composition in Children With Intestinal Failure on Parenteral Nutrition

Patterson

Betts

et al. 2022

Journal of Pediatric Gastroenterology &Amp; Nutrition

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“…Survival of these patients also depends on comorbidities and complications due to their condition as they are exposed to Catheter Induced Bloodstream Infections, IF Associated Liver Disease, thrombosis, electrolyte dysfunction, and malnutrition. In the last years, survival has improved after the introduction of multidisciplinary intestinal rehabilitation programs, autologous bowel-lengthening procedures, and the use of new and improved formulas in PN ( 34 ). To improve their chances of survival and intestinal function, we should promptly refer these patients to highly specialized centers to get a multidisciplinary treatment that involves different professional medical and surgical treatment.…”

Section: Discussionmentioning

confidence: 99%

Use of Teduglutide in Children With Intestinal Failure: A Systematic Review

et al. 2022

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Background and ObjectivesShort-bowel syndrome (SBS) results from the loss of a significant portion of the small intestine leading to a state of malabsorption. After an intestinal loss, there is a process of adaptation involving the Glucagon-Like Peptide-2 (GLP-2), an enteroendocrine peptide also involved in nutrient absorption. Teduglutide is a recombinant analog of GLP-2 approved in 2016 to treat selected SBS pediatric patients who are dependent on parenteral support. The present systematic review aims to evaluate the efficacy of Teduglutide in pediatric patients with SBS in reducing the need for parenteral nutrition (PN).Materials and MethodsWe performed a literature search on MEDLINE and Embase to include articles up to November 2021. We included articles that involved using Teduglutide in the SBS pediatric population to define its efficacy in reducing the need for PN. The key words used were GLP-2, teduglutide, child.ResultsFourteen studies completely fulfilled the inclusion criteria. Two hundred 23 patients were treated with Teduglutide, and the median duration of treatment was 45 weeks (IQR: 36–52.5 weeks). One-hundred and fifty-two patients were treated with 0.05 mg/Kg/d of subcutaneous Teduglutide, 38 received 0.025 mg/Kg/d and 8 received either 0.125 mg/Kg/d or 0.20 mg/Kg/d. A total of 36 patients achieved enteral autonomy (EA) after a median of 24 weeks of treatment (IQR: 24–48 weeks) and 149 patients showed a reduction in PN needs in terms of volume, calories, or hours per day. Eleven studies reported complications: gastrointestinal were the most common, with 89 cases reported in treated patients and 11 in non-treated patients.ConclusionTeduglutide appears safe and effective in reducing PN requirements and improving EA in the pediatric population. However, more studies are needed to understand its efficacy in the long term and after discontinuation and possible complications.Systematic Review Registration[https://www.crd.york.ac.uk/prospero/], identifier [CRD42022301593].

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“…La eficacia del manejo entregado en UCE, se ve reflejada en 2 aspectos: El primero, y más importante, es el porcentaje de autonomía enteral al alta y, en segundo lugar, la disminución de la desnutrición al egreso. Respecto a la autonomía enteral, ésta se logró en un 66,7%, siendo comparable con el 56,9% de 15,20,22 . El grupo de pacientes que no lograron autonomía presentaban factores pronósticos desfavorables 20 : clasificación anatómica tipo I y II, ausencia de VIC, remanente intestinal < 40 cm y NM.…”

Section: Discussionunclassified

Insuficiencia Intestinal Pediátrica, 10 años de experiencia en una Unidad de Cuidados Especializados

et al. 2022

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La definición de Insuficiencia Intestinal (II) incluye la reducción de masa intestinal funcional y requerimiento de nutrición parenteral (NP) prolongada para lograr el desarrollo y crecimiento en la niñez. Objetivo: Evaluar la experiencia de una Unidad especializada en pacientes pediátricos con II, describiendo las características clínicas de aquellos admitidos desde noviembre 2009 a diciembre 2019. Pacientes y Método: Revisión del registro clínico de 24 casos que cumplieron los criterios de inclusión. Se recolectaron las siguientes variables: sexo, antecedentes neonatales, edad de ingreso, unidad de procedencia, diagnóstico antropométrico de ingreso, causa de insuficiencia intestinal, tiempo de hospitalización, edad de egreso de la unidad, datos antropométricos al egreso y dependencia de nutrición parenteral al alta. En aquellos pacientes con diagnóstico de Síndrome Intestino Corto (SIC) se identificó la causa de la resección intestinal y las características del remanente intestinal: clasificación anatómica, longitud de remanente (definiendo ultracorto como < 25 cm), presencia de válvula ileocecal y características del colon. Resultados: la mediana de edad al ingreso fue de 7,8 meses. Diecisiete casos eran prematuros. Etiología de la II: 10 pacientes con SIC, 6 casos de Neuromiopatía intestinal (NM), 7 pacientes con SIC asociado a NM y 1 caso Linfangiectasia intestinal. En la etiología de SIC encontrada en este grupo, la atresia intestinal con 8 casos y la enterocolitis necrotizante(ECN) 9 casos, fueron las causas principales en similar proporción. Ocho pacientes no tenían Válvula ileocecal (VIC). Según clasificación anatómica: 1 era tipo I, 8 tipo II y 8 tipo III. Por longitud intestinal 3 eran ultracortos, además de tipo II; en aquellos con remanente > 40 cm, 7 eran tipo III. El promedio global de estadía hospitalaria fue 456 días. Se logró autonomía enteral en 16 pacientes y 8 casos requirieron NP Domiciliaria. Conclusiones: La II requiere de soporte vital: NP y hospitalización prolongada. Su principal causa es el SIC secundario a atresia intestinal congénita y enterocolitis necrotizante. No obstante, la alta frecuencia de NM encontrada puede atribuirse al protocolo local de análisis. La mayoría de nuestros pacientes presentaban factores pronósticos desfavorables, sin embargo, el manejo por el equipo especializado permitió lograr autonomía enteral en 66,7% de los casos.

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Trends in Pediatric Intestinal Failure: A Multicenter, Multinational Study

Cited by 39 publications

References 49 publications

Muscle Strength, Agility, and Body Composition in Children With Intestinal Failure on Parenteral Nutrition

Muscle Strength, Agility, and Body Composition in Children With Intestinal Failure on Parenteral Nutrition

Use of Teduglutide in Children With Intestinal Failure: A Systematic Review

Insuficiencia Intestinal Pediátrica, 10 años de experiencia en una Unidad de Cuidados Especializados

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