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- Mapping the Landscape: Simulation Centers in Portugal.Publication . Silva, Bruno Miguel; Norte, Gustavo; Lito, Pedro; Garcia, PedroIntroduction: Simulation-based training has emerged as a vital component of healthcare education. This study aims to characterize Portuguese simulation centers concerning their geographic distribution and key features, providing stakeholders with valuable insights to inform strategic decisions. Methods: A cross-sectional survey-based study was conducted over two years (2021-2023) to investigate the geographical dispersion and characteristics of simulation centers in Portugal. Descriptive statistics and thematic analysis were used to analyze data. Results: Twenty-three Portuguese simulation centers were included. Major urban areas and coastal regions bring together 20 simulation centers (86.96%). A large percentage (71.93%) of centers were affiliated with academic institutions, while five centers (21.74%) were clinically affiliated. Emergency care, Anesthesiology and Intensive Medicine, Pediatrics, and Gynecology and Obstetrics were identified as the national key areas of intervention. Discussion: Significant geographical disparity raises concerns about unequal access to professional training opportunities using simulation. Centers should be encouraged to incorporate developing technologies and innovative pedagogical methodologies and to expand their training repertoire into relatively uncharted territories. Conclusion: Several issues have been identified within the national simulation network. Stakeholders and policymakers should prioritize equitable access, bolster the prevalence of clinical affiliated centers, foster innovation, and facilitate strategic coordination.
- Individualized Fortification Based on Measured Macronutrient Content of Human Milk Improves Growth and Body Composition in Infants Born Less than 33 Weeks: A Mixed-Cohort StudyPublication . Cardoso, M; Virella, D; Papoila, AL; Alves, M; Macedo, I; e Silva, D; Pereira-da-Silva, LThe optimal method for human milk (HM) fortification has not yet been determined. This study assessed whether fortification relying on measured HM macronutrient content (Miris AB analyzer, Upsala, Sweden) composition is superior to fortification based on assumed HM macronutrient content, to optimize the nutrition support, growth, and body composition in infants born at <33 weeks' gestation. In a mixed-cohort study, 57 infants fed fortified HM based on its measured content were compared with 58 infants fed fortified HM based on its assumed content, for a median of 28 and 23 exposure days, respectively. The ESPGHAN 2010 guidelines for preterm enteral nutrition were followed. Growth assessment was based on body weight, length, and head circumference Δ z-scores, and the respective growth velocities until discharge. Body composition was assessed using air displacement plethysmography. Fortification based on measured HM content provided significantly higher energy, fat, and carbohydrate intakes, although with a lower protein intake in infants weighing ≥ 1 kg and lower protein-to-energy ratio in infants weighing < 1 kg. Infants fed fortified HM based on its measured content were discharged with significantly better weight gain, length, and head growth. These infants had significantly lower adiposity and greater lean mass near term-equivalent age, despite receiving higher in-hospital energy and fat intakes, with a mean fat intake higher than the maximum recommended and a median protein-to-energy ratio intake (in infants weighing < 1 kg) lower than the minimum recommended.
- Changes in Microbiota Profile in the Proximal Remnant Intestine in Infants Undergoing Surgery Requiring EnterostomyPublication . Barreiros-Mota, I; R. Araújo, J; Marques, C; Sousa, L; Morais, J; Castela, I; Faria, A; Neto, MT; Cordeiro-Ferreira, G; Virella, D; Pita, A; Pereira-da-Silva, L; Calhau, CEarly-life gut dysbiosis has been associated with an increased risk of inflammatory, metabolic, and immune diseases later in life. Data on gut microbiota changes in infants undergoing intestinal surgery requiring enterostomy are scarce. This prospective cohort study examined the enterostomy effluent of 29 infants who underwent intestinal surgery due to congenital malformations of the gastrointestinal tract, necrotizing enterocolitis, or spontaneous intestinal perforation. Initial effluent samples were collected immediately after surgery and final effluent samples were collected three weeks later. Gut microbiota composition was analysed using real-time PCR and 16S rRNA gene sequencing. Three weeks after surgery, an increase in total bacteria number (+21%, p = 0.026), a decrease in Staphylococcus (-21%, p = 0.002) and Candida spp. (-16%, p = 0.045), and an increase in Lactobacillus (+3%, p = 0.045) and in less abundant genera belonging to the Enterobacteriales family were found. An increase in alpha diversity (Shannon's and Simpson's indexes) and significant alterations in beta diversity were observed. A correlation of necrotizing enterocolitis with higher Staphylococcus abundance and higher alpha diversity was also observed. H2-blockers and/or proton pump inhibitor therapy were positively correlated with a higher total bacteria number. In conclusion, these results suggest that positive changes occur in the gut microbiota profile of infants three weeks after intestinal surgery.
- Congenital Glucagon-like Peptide-1 Deficiency in the Pathogenesis of Protracted Diarrhea in Mitchell–Riley SyndromePublication . Nóbrega, S; Monteiro, MP; Pereira-da-Silva, L; Pereira, SS; Hartmann, B; Holst, JJ; Barbosa Silva, R; Cordeiro-Ferreira, GContext: Mitchell-Riley syndrome due to RFX6 gene mutations is characterized by neonatal diabetes and protracted diarrhea. The RFX6 gene encodes a transcription factor involved in enteroendocrine cell differentiation required for beta-cell maturation. In contrast to the pathway by which RFX6 mutations leads to diabetes, the mechanisms underlying protracted diarrhea are unknown. Objective: To assess whether glucagon-like peptide-1 (GLP-1) was involved in the pathogenesis of Mitchell-Riley syndrome protracted diarrhea. Methods: Two case report descriptions. in a tertiary pediatric hospital. "Off-label" treatment with liraglutide. We describe 2 children diagnosed with Mitchell-Riley syndrome, presenting neonatal diabetes and protracted diarrhea. Both patients had nearly undetectable GLP-1 plasma levels and absence of GLP-1 immunostaining in distal intestine and rectum. The main outcome was to evaluate whether GLP-1 analogue therapy could improve Mitchell-Riley syndrome protracted diarrhea. Results: "Off-label" liraglutide treatment, licensed for type 2 diabetes treatment in children, was started as rescue therapy for protracted intractable diarrhea resulting in rapid improvement during the course of 12 months. Conclusion: Congenital GLP-1 deficiency was identified in patients with Mitchell-Riley syndrome. The favorable response to liraglutide further supports GLP-1 involvement in the pathogenesis of protracted diarrhea and its potential therapeutic use.
- Bone Metabolism in Very Preterm Infants Receiving Total Parenteral Nutrition: do Intravenous Fat Emulsions Have an Impact?Publication . Bridges, KM; Pereira-da-Silva, L; Tou, JC; Ziegler, J; Brunetti, Lery preterm infants (<32 weeks' gestation) are at high risk for impaired skeletal development because of factors that limit the provision of extrauterine nutrients. Cumulative net deficiencies of calcium, phosphorus, docosahexaenoic acid (DHA), and arachidonic acid (ARA) are evident in these infants after prolonged administration of total parenteral nutrition (TPN). This is significant because minerals as well as metabolites of DHA and ARA are important modulators of bone cell differentiation, lengthening of bone, and bone matrix deposition. Furthermore, diets containing only precursors of DHA and ARA result in suboptimal skeletal growth. With the emergence of new intravenous lipid emulsions, it is important to understand the impact of fatty acids on bone metabolism in the third trimester in order to optimize the provision of TPN in very preterm infants. The purpose of this review is to evaluate current evidence regarding intravenous lipid emulsions and bone metabolism in very preterm infants receiving prolonged TPN and to identify areas of research needed.
- Which Type of Maternal Dietary Polyunsaturated Fat Affects Fetal Adiposity?Publication . Pereira-da-Silva, L; Virella, D
- Pulmonary Arteriovenous Malformation Closure in a Preterm NewbornPublication . Loureiro, P; Laranjo, S; Lage, MJ; Trigo, C; Pinto, FF; Martins, JDF
- Congenital Cytomegalovirus Infection in Twin PregnancyPublication . Carvalho, AA; Silva, CB; Martins, ML; Cassiano Santos, GCassianoCytomegalovirus (CMV) infection is one of the preeminent congenital viral infections, and despite its potential morbidity, uncertainty about its physiopathology, prevention and treatment remains until now. We report a case of a dichorionic and diamniotic twin pregnancy in which only one of the fetus had signs of being affected. The first twin had prenatal diagnosis of intrauterine growth restriction and hyperechogenic bowel, attributable to CMV infection, while there was no evidence of infection of the second one. Prenatal treatment was done with maternal administration of valacyclovir and postnatal treatment of the infected newborn with oral valganciclovir with normal neurodevelopment assessment at 12 months corrected age. In this case, maternal CMV infection was not equally transmitted to both fetuses, suggesting that there may be intrinsic fetal and placental factors influencing both transmission and the clinical features of the infection.
- Eating Habits During Pregnancy of Women Giving Birth Very Prematurely: An Exploratory AnalysisPublication . Teixeira, B; Cardoso, M; Dias, CC; Pereira-da-Silva, L; E Silva, DIntroduction: Preterm birth is increasing worldwide, representing a major cause of death and long-term loss of human potential among survivors. Some morbidities during pregnancy are well-known risk factors for preterm labor, but it is not yet known whether deviations from adequate dietary patterns are associated with preterm delivery. Diet may be an important modulator of chronic inflammation, and pro-inflammatory diets during pregnancy were reported to be associated with preterm birth. The aim of this study was to assess the food consumption during pregnancy of Portuguese women giving birth very prematurely and the association between the food consumption and the major maternal morbidities during pregnancy related with preterm delivery. Material and methods: A single-center cross-sectional observational study including consecutive Portuguese women giving birth before 33 weeks of gestation was conducted. Recall of eating habits during pregnancy was obtained within the first week after delivery, using a semi quantitative food frequency questionnaire validated for Portuguese pregnant women. Results: Sixty women with a median age of 36.0 years were included. Of these, 35% were obese or overweight at the beginning of pregnancy, 41.7% and 25.0% gained excessive or insufficient weight during pregnancy, respectively. Pregnancy-induced hypertension was present in 21.7% of cases, gestational diabetes in 18.3%, chronic hypertension in 6.7%, and type 2 diabetes mellitus in 5.0%. Pregnancy-induced hypertension was significantly associated with increased daily consumption of pastry products (31.2 vs 10.0 g, p = 0.022), fast food (39.6 vs 29.7 g, p = 0.028), bread (90.0 vs 50.0 g, p = 0.005), pasta, rice and potatoes (225.7 vs 154.3 g, p = 0.012). In a multivariate analysis, only bread consumption maintained a significant, albeit weak, association (OR = 1.021; 1.003 - 1.038, p = 0.022). Conclusion: Pregnancy-induced hypertension was associated with increased consumption of pastry products, fast food, bread, pasta, rice, and potatoes, although only bread consumption had a weak but statistically significant association with pregnancy-induced hypertension in a multivariate analysis.
- Acquired Cytomegalovirus Infection in an Extremely Low-Birth Weight Infant Presenting with a Severe Sepsis-Like SyndromePublication . Crisóstomo, MR; Póvoas, M; Pita, AM; Leal, E; Nona, JCytomegalovirus infection is one of the most common congenital infections worldwide. Moreover, it seems to be an important cause of postnatally acquired infection. Perinatal transmission can occur intrapartum (from the birth canal), from a blood transfusion, via maternal breast milk, or from close contact with infected people. The risk of breast milk-acquired cytomegalovirus infection is higher in countries with a high prevalence of cytomegalovirus immunoglobulin G-positive women. Usually, acquired cytomegalovirus infection is asymptomatic, especially in term infants. However, preterm infants can present with a sepsis-like syndrome and multiple organ involvement. A high index of suspicion is required to make an early diagnosis. Therapeutic guidelines for symptomatic postnatal cytomegalovirus infection are not yet available. More studies are required to understand the long-term sequelae of postnatally acquired cytomegalovirus infection and know which is the best strategy to avoid cytomegalovirus post-natal transmission.