Role of home-blended tube feedings in pediatric patients with gastrostomy tubes: A retrospective study.

Background: Both blended tube feed (BTF) and commercial tube feed (CTF) can be administered through a gastrostomy tube (GT). There is very little evidence about using home BTF (HBTF). Nevertheless, families increasingly request this type of nutrition because they attribute some benefits to it.

[Taurolidine lock in pediatric patients with intestinal failure. A practical guideline from the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP)].

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.

Early use of teduglutide in paediatric patients with intestinal failure is associated with a greater response rate: a multicenter study.

Teduglutide is a glucagon-like-peptide-2 analogue that reduces the need for parenteral support in patients with short bowel syndrome (SBS). Nevertheless, data about long-term therapy with teduglutide in children are still scarce. Our objective was to describe the real-life experience with teduglutide in children with SBS over the last 5 years in Spain.

[The registers of pediatric patients with home artificial nutrition. The Spanish experience].

Background: the number of infants and children who receive artificial nutrition at home has been steadily increasing over the last decades, as better outcomes for children with chronic conditions have been achieved. In order to evaluate the need of resources to implement the technique it is necessary to know how many patients benefit from home artificial nutrition. This information can be estimated from the register of patients, when available.

Which is the best route to achieve nutritional goals in pediatric ECMO patients?

Objectives: Estimating caloric intake and choosing route of administration are fundamental in the nutritional support of patients being supported by extracorporeal membrane oxygenation (ECMO). The aim of this study was to review the nutritional intervention carried out in a pediatric cohort in a third-level hospital.

Methods: This was a prospective descriptive study.

[Home and Ambulatory Artificial Nutrition (NADYA) Group report: home parenteral nutrition in Spain, 2019].

Aim: to communicate the home parenteral nutrition (HPN) data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2019.

Experience With Teduglutide in Pediatric Short Bowel Syndrome: First Real-life Data.

Objectives: The aim of the study was to describe the experience with teduglutide of several Spanish hospitals in pediatric patients with SBS (SBS).

Methods: Seventeen pediatric patients with intestinal failure associated with SBS were treated with teduglutide. Patients received 0.

[Home and Ambulatory Artificial Nutrition (NADYA) Group Report. Home parenteral nutrition in Spain, 2018].

Aim: to communicate home parenteral nutrition (HPN) data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2018 Material and methods: descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1st, 2018 to December 31st, 2018.

Acquired and Innate Immunity Impairment and Severe Disseminated Infection in a Patient With a NF-κB1 Deficiency.

NF-κB1 is a master regulator of both acquired and innate responses. loss-of-function mutations elicit a wide clinical phenotype with asymptomatic individuals at one end of the spectrum and patients with common variable immunodeficiency, combined immunodeficiency or autoinflammation at the other. Impairment of acquired and innate immunity and disseminated infection expands the clinical and immunological phenotype of NF-κB1 deficiency.

Acute Liver Failure: Outcome and Value of Pediatric End-Stage Liver Disease Score in Pediatric Cases.

Purpose: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission.

Methods: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival.

Diagnostic Yield of Next-generation Sequencing in Very Early-onset Inflammatory Bowel Diseases: A Multicentre Study.

Background And Aims: An expanding number of monogenic defects have been identified as causative of severe forms of very early-onset inflammatory bowel diseases [VEO-IBD]. The present study aimed at defining how next-generation sequencing [NGS] methods can be used to improve identification of known molecular diagnosis and to adapt treatment.

Methods: A total of 207 children were recruited in 45 paediatric centres through an international collaborative network [ESPGHAN GENIUS working group] with a clinical presentation of severe VEO-IBD [n = 185] or an anamnesis suggestive of a monogenic disorder [n = 22].

A New Case of Congenital Malabsorptive Diarrhea and Diabetes Secondary to Mutant .

Congenital diarrheal disorders are a group of rare enteropathies that often present with life-threatening diarrhea in the first weeks of life. Enteric anendocrinosis, characterized by a lack of intestinal enteroendocrine cells due to recessively inherited mutations in the () gene, has been described as a cause of congenital malabsorptive diarrhea. Diabetes mellitus also is typically associated with mutations, be it early onset or a later presentation.

Lack of Accuracy of Biomarkers and Physical Examination to Detect Bacterial Infection in Febrile Infants.

Objectives: The aim of this study was to analyze the usefulness of physical examination, C-reactive protein (CRP), procalcitonin (PCT), white blood cell (WBC) count, and absolute neutrophils counts (ANCs) for the diagnosis of invasive bacterial infections (IBIs) and potentially serious bacterial infections in infants younger than the age of 3 months presenting with fever without source (FWS) to the emergency department (ED).

Methods: A descriptive retrospective study that includes all infants aged younger than 3 months who presented with FWS to the ED between July 2008 and January 2012. We evaluated diagnostic performance for each test by receiver operating characteristic curve analysis.

[Not Available].

Introducción: la nutrición parenteral domiciliaria se ha convertido en un punto clave en el tratamiento de pacientes con fracaso intestinal crónico. A pesar de los importantes avances que se han producido en las últimas décadas, tanto en los accesos vasculares, como en las soluciones empleadas, las infecciones asociadas a catéter venoso central siguen constituyendo una de las complicaciones más importantes. Dentro de las estrategias para la prevención o el tratamiento de estas infecciones se encuentra el empleo de sellados con antisépticos, como el etanol o la taurolidina, o de antibióticos.

Adolescent with neuropsychiatric symptoms associated with novel influenza A (H1N1) virus infection.

Neurologic complications have been described previously in association with seasonal influenza A or B viruses, but the frequency with which these occur with novel influenza A (H1N1) virus infection is unknown. We describe a case of an adolescent with a bizarre neuropsychiatric picture associated with novel influenza A (H1N1) virus infection.