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2023

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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PEDIATRIC INTESTINAL FAILURE – THE EXPERIENCE OF A THIRD LINE HOSPITAL

Sara Nóbrega1; Joana Faustino2; Miguel Correia1; António Pedro Campos1; Raul Silva1

1 – Nutrition and Respiratory Special Care Unit, Hospital Dona Estefânia, Centro Hospitalar e Universitário de Lisboa Central, Lisbon, Portugal
2 – Pediatrics Department, Hospital Dona Estefânia, Centro Hospitalar e Universitário de Lisboa Central, Lisbon, Portugal

11th Conference Excellence in Pediatrics 2019; Apresentação sob a forma de comunicação oral

Introdução: Intestinal failure (IF) is defined as a critical reduction in the functioning intestinal mass below the amount necessary for adequate absorption of fluids, electrolytes or nutrients required for normal growth and survival, if no long-term parenteral nutrition is provided. IF is an uncommon and complex condition, and the impact on the individuals and family is enormous.
Objectivo: This study analyzed patients followed at a specialized Nutritional Unit in a third line pediatric hospital, between 2015 and 2019.
Métodos: Retrospective and descriptive study of 29 patients with IF. Demographic and clinical data, complications, treatments and evolution are presented.
Resultados: This study included 29 children with IF. The main cause was short bowel syndrome (SBS, n=22), the majority of them secondary to midgut volvulus, intestinal atresia, gastroschisis and necrotizing enterocolitis; intestinal pseudoobstruction (n=3), Mitchell Riley syndrome (n=2), Hirschprung's disease (n=1) and inflammatory bowel disease (n=1). The median of surgery was 3/children, 10 having performed serial transverse enteroplasty (STEP) and longitudinal intestinal lengthening and tailoring (LILT). In SBS children, the median of residual short bowel length was 24 cm, three of them removed the colon and 18 the ileocaecal valve. The median of central venous catheter (CVC) sepsis was 2,3/1000 days of CVC, the majority due to Staphylococci coagulase negative and Gram negative bacteria. other complications were: CVC fractures (n=14), CVC-related venous thrombosis (n=3), food allergy (n=3) and pancreatitis (n=2). IF associated liver disease (IFALD) occurred in 48% of cases. One patient underwent intestinal transplantation (IT). Three deaths occurred because of CVC sepsis, IFALD and one after IT.
Conclusão: We report a particular group of patients, dependent on parenteral nutrition who have an increased risk of complications, parenteral nutrition associated and others. Moreover, they may need surgical rescue procedures to improve functioning gut and promote adaptation. IT should be offered as last line strategy. IF centers of excellence and medical-surgical cooperation play a vital role.

Palavras Chave: pediatric intestinal failure, parenteral nutrition, short bowel syndrome