Risk Factors for Developing Inflammatory Bowel Disease Within and Across Families with a Family History of IBD.

INTRODUCTION: A family history of inflammatory bowel disease [IBD] is the strongest risk factor for disease. However, some first-degree relatives (FDRs) will develop disease, while others will not. METHODS: Using the nationwide Danish National Patient Register, we examined risk factors in families with two or more affected FDRs. First, we compared exposures between siblings with and without IBD within the same family [within-family analysis]. Second, we compared exposures between individuals with and without IBD across all families [across-family analysis]. Exposures included sex, birth order, mode of delivery, antibiotics, personal and family history of immune-mediated diseases, gastrointestinal infections, and surgical history preceding diagnosis. Uni- and multivariable conditional logistic regression analyses were conducted. RESULTS: In the 'within-family analysis', 1669 families were included [1732 cases, 2447 controls]. Female sex (adjusted odds ratio [aOR]: 1.40, 95% confidence interval [CI] 1.23, 1.59), history of ankylosing spondylitis [aOR: 2.88, 95% CI 1.05, 7.91] and exposure to antibiotics [aOR: 1.28, 95% CI 1.02, 1.61] increased the risk for IBD. In the 'across-family analysis', 1254 cases and 37 584 controls were included, confirming an association with prior ankylosing spondylitis [aOR: 3.92, 95% CI 1.38, 11.12] and exposure to antibiotics [aOR: 1.29, 95% CI 1.04, 1.60]. Having two or more relatives [aOR: 6.26, 95% CI 1.34, 29.29] or a sibling with IBD [aOR: 1.36, 95% CI 1.18, 1.57] increased the risk of IBD. Appendectomy reduced the risk of ulcerative colitis [aOR: 0.32, 95% CI 0.14, 0.72]. CONCLUSION: In families with IBD, we identified risk factors for the unaffected FDR to develop disease. These findings provide an opportunity for counselling IBD relatives.

Use of home parenteral nutrition in severely neurologically impaired children.

OBJECTIVE: To review the outcome of children with severe neurological impairment (NI) and intestinal failure (IF) referred to our specialist multidisciplinary IF rehabilitation service and to discuss implications. DESIGN: Case report series, descriptive analysis. SETTING: IF rehabilitation programme at a tertiary children's hospital in the UK. PATIENTS: Children with severe NI referred to our IF rehabilitation programme from 2009 to 2019. MAIN OUTCOME MEASURES: Demographic and social data, diagnosis, clinical condition, use of home parenteral nutrition (HPN), complications, ethics review outcome and advance care plans. RESULTS: Six patients with severe NI were referred to our IF rehabilitation service. Consent for publication was obtained from five families. After thorough medical review and clinical ethics committee assessment, three children started HPN, one had intravenous fluids in addition to enteral feed as tolerated and one intravenous fluids only. The HPN children survived 3-7.08 years (median 4.42 years) on treatment. Objective gastrointestinal signs, for example, bleeding improved without excessive HPN-related complications. Symptomatic improvement was less clear. Analgesia was reduced in three of the five children. All cases had detailed symptom management and advance care plans regularly updated. CONCLUSIONS: HPN can play a role in relieving gastrointestinal signs/symptoms in children with severe NI and IF. HPN can be conceptualised as part of good palliative care if judged to be in the child's best interests. However, given its risks and that HPN has the potential to become inappropriately life-sustaining, a thorough ethics review and evaluation should be performed before it is initiated, withheld or withdrawn in children with severe NI.

Early life exposures and the risk of inflammatory bowel disease: Systematic review and meta-analyses.

BACKGROUND: Early life exposures impact immune system development and therefore the risk of immune-mediated diseases, including inflammatory bowel disease (IBD). We systematically reviewed the impact of pre-, peri­, and postnatal exposures up to the age of five years on subsequent IBD diagnosis. METHODS: We identified case-control and cohort studies reporting on the association between early life environmental factors and Crohn's disease (CD), ulcerative colitis (UC), or IBD overall. Databases were search from their inception until May 24th, 2019 until July 14th, 2020. We conducted meta-analyses for quantitative review of relevant risk factors that were comparable across studies and qualitative synthesis of the literature for a wide range of early life exposures, including maternal health and exposures during pregnancy, perinatal factors, birth month and related-factors, breastfeeding, hygiene-related factors and social factors, immigration, antibiotics, offspring health, including infections, and passive smoking. PROSPERO registration: CRD42019134980. FINDINGS: Prenatal exposure to antibiotics (OR 1.8; 95% CI 1.2-2.5) and tobacco smoke (OR 1.5; 95% CI 1.2-1.9), and early life otitis media (OR 2.1; 95% CI 1.2-3.6) were associated with IBD. There was a trend towards an association between exposure to antibiotics in infancy and IBD (OR: 1.7, 95% CI 0.97, 2.9), supported by positive data on population-based data. Breastfeeding was protective against IBD. Other early life risk factors had no association with IBD, but data were limited and heterogenous. INTERPRETATION: Early life is an important period of susceptibility for IBD development later in life. Tobacco smoke, infections and antibiotics were associated positively, and breastfeeding was associated negatively with IBD. Our findings offer an opportunity to develop primary prevention strategies. FUNDING: This study did not receive any funding.

Assessment of the renal angina index for the prediction of acute kidney injury in patients admitted to a European pediatric intensive care unit.

BACKGROUND: Acute kidney injury (AKI) is associated with worse outcomes and increased morbidity and mortality in pediatric intensive care unit (PICU) patients. The renal angina index (RAI) has been proposed as an early prediction tool for AKI development. OBJECTIVES: The objective was to evaluate outcomes of RAI-positive patients and to compare RAI performance with traditional AKI markers across different patient groups (medical/post-surgical). This was an observational retrospective study. All children admitted to a tertiary hospital PICU over a 3-year period were included. Electronic medical records were reviewed. Day 1 RAI was calculated, as was the presence and staging of day 3 AKI. RESULTS: A total of 593 patients were included; 56% were male, the mean age was 55 months, and 17% had a positive RAI. This was associated with day 3 AKI development and worse outcomes, such as greater need for kidney replacement therapy, longer duration of mechanical ventilation, vasoactive support and PICU stay, and higher mortality. For all-stage kidney injury, RAI presented a sensitivity of 87.5% and a specificity of 88.1%. Prediction of day 3 all-stage AKI by RAI had an AUC=0.878; its performance increased for severe AKI (AUC = 0.93). RAI was superior to serum creatinine increase and KDIGO AKI staging on day 1 in predicting severe AKI development. The performance remained high irrespective of the type of admission. CONCLUSIONS: The RAI is a simple and inexpensive tool that can be used with medical and post-surgical PICU patients to predict AKI development and anticipate complications, allowing for the adoption of preventive measures.

Paracetamol Allergy: A Case of a 9-Year-Old Female with a History of Atopy.

Background: We describe a rare case involving paracetamol, a commonly used drug worldwide. Increased paracetamol consumption increases the risk of adverse drug reactions. Case Presentation: This is a case of a 9-year-old girl who visited the emergency department due to sudden onset sneezing, nasal itching, urticaria, and angioedema after paracetamol ingestion. The diagnostic and etiologic studies revealed an immunoglobulin E (IgE)-mediated hypersensitivity mechanism to paracetamol. Conclusion: Few cases of this phenomenon have been reported in previous literature. As confirmed in this study, a negative skin prick test did not exclude hypersensitivity, and conducting intradermal tests (IDTs) increased diagnostic accuracy. The patient had a positive IDT, confirming the underlying IgE-mediated reaction. The follow-up of a confirmed paracetamol hypersensitivity implies patient education about avoidance of any paracetamol-containing formulation, including combination products and clarification of available alternative drugs. This is likely the first publication documenting IgE-mediated paracetamol allergy in pediatric patients. We intend to underline the clinical benefits of diagnostic confirmation toward suspected drug hypersensitivity reactions in children, a particularly useful topic for pediatricians and pediatric allergists.

[Satisfação com o Internato Médico em Portugal]. / Satisfação com o Internato Médico em Portugal.

INTRODUCTION: In the last years, the global context of medical education and Medical Residency programs in Portugal suffered substantial changes. The primary objective of this study was to evaluate and characterize medical residents ́ satisfaction with medical residency programs in Portugal and to identify features that could be improved. MATERIAL AND METHODS: We utilized as model the survey Postgraduate Hospital Educational Environment Measure that has been developed in the United Kingdom and is speci cally targeted to medical residents. The survey was translated and adapted to the Portuguese reality. The survey was available online during April and May of 2016. RESULTS: A total of 3456 responses were obtained, corresponding to a response rate of 35%. Endocrinology/Nutrition, Cardiology, Anesthesiology, Family Physician and Gastroenterology were the specialties in which the degree of satisfaction was higher, while Forensic Medicine, Medical Oncology, Internal Medicine, General Surgery and Pneumology showed the lowest level of satisfaction. DISCUSSION: This study presented a high response rate when compared to previous studies. Portuguese medical residents presented high levels of satisfaction. Depending on year of medical residency, region, type of specialty and type of hospital marked asymmetries were noticed. CONCLUSION: The survey ́s results should constitute in the future a support tool for the implementation of local and national measures relating to the medical residency. It is advisable to regularly conduct satisfaction surveys to medical residents.

Introdução: Nos últimos anos, o contexto global da formação médica, e em particular do Internato Médico em Portugal, sofreu profundas alterações. O presente estudo teve como objetivo avaliar e caracterizar a satisfação dos médicos internos com a realização do Internato Médico em Portugal e identificar aspetos passíveis de melhoria.Material e Métodos: Foi utilizado como modelo de inquérito o questionário Postgraduate Hospital Educational Environment Measuredesenvolvido no Reino Unido e dirigido a médicos internos, o qual foi traduzido e adaptado à realidade portuguesa. O questionário esteve disponível online durante os meses de abril e maio de 2016.Resultados: Foram obtidas 3456 respostas, correspondendo a uma taxa de resposta de 35%. Endocrinologia/Nutrição, Cardiologia, Anestesiologia, Medicina Geral e Familiar e Gastrenterologia foram as especialidades nas quais o grau de satisfação foi mais elevado,enquanto que Medicina Legal, Oncologia Médica, Medicina Interna, Cirurgia Geral e Pneumologia apresentaram o grau de satisfaçãomais baixo.Discussão: O presente estudo apresenta uma elevada taxa de resposta comparativamente com estudos prévios. A nível nacional, no global, os médicos internos apresentaram níveis elevados de satisfação, destacando-se marcadas assimetrias de acordo com o ano de especialidade, região, tipologia de instituição e de especialidade.Conclusão: Os resultados deste inquérito poderão constituir uma ferramenta de apoio à implementação de medidas de âmbito local enacional relacionadas com o Internato Médico, sendo desejável a realização regular de inquéritos de satisfação aos médico internos.

Endoscopic Submucosal Dissection of Gastric Superficial Lesions: Predictors for Time of Procedure in a Portuguese Center.

BACKGROUND: Endoscopic submucosal dissection (ESD), an endoscopic technique used for treatment of gastric superficial lesions, has been gaining importance on western countries. Procedural times have an impact on various outcomes. AIM: To define which factors from patients, lesions and procedure can predict longer procedural times. METHODS: In a cohort of 127 lesions resected by ESD with IT-knife, after using needle-knife for submucosal layer access, by experienced gastroenterologists, characteristics from the patient (age, gender, presence of co-morbidities, usage and suspension of anti-platelet drugs and general physical condition), lesion (size, histopathological diagnosis at biopsy, location, macroscopic type and submucosal invasion) and procedure (adverse events) were retrospectively analyzed for its impact on time of procedure. Univariate and multivariate analysis were performed. RESULTS: Lesions larger than 20 mm (p < 0.001), on the upper third of the stomach (p = 0.035) and with an ASA score of 3 (p = 0.031) were considered influential factors for a longer procedure time and specifically for a time of procedure longer than 90 min. Existence of intra-procedure adverse events was also a predictor for a procedure time >90 min. Lesion's size >20 mm and location in the upper third were independently associated with a procedure time longer than 90 min (OR 4.91 [95%CI 2.29-10.50] and OR 18.26 [95%CI 2.02-164.78], respectively). CONCLUSION: The time of procedure of ESD for gastric superficial lesions is influenced by size of lesion (>20 mm) and location (upper third of stomach), which predict a time longer than 90 min. This can be useful for better management of workflow, operation, training of teams and anesthesic procedures.

INTRODUÇÃO: A Disseção Endoscópica de Submucosa (ESD), uma técnica utilizada para o tratamento de lesões gástricas superficiais, tem ganho importância no Ocidente. O tempo de procedimento tem impacto nos outcomes. OBJETIVO: Definir os fatores do paciente, da lesão e do procedimento que predizem um tempo de procedimento prolongado. MÉTODOS: Numa coorte de 127 lesões removidas por ESD com IT-knife, após utilização de needle-knife para acesso à camada submucosa, por gastrenterologistas experientes foram analisadas, retrospetivamente, características dos pacientes (idade, género, presença de co-morbilidades, toma/suspensão de agentes anti-plaquetários e condição física), das lesões (tamanho, diagnóstico histopatológico na biopsia, localização, características macroscópicas e invasão submucosa) e do procedimento (complicações). Foi realizada análise univariada e multivariada. RESULTADOS: Lesões >20 mm (p<0,001), no terço superior do estômago (p=0,035) e em pacientes com um índice ASA 3 (p=0,031) foram considerados como fatores com influência para tempo de procedimento prolongado e, especificamente, para um tempo de procedimento superior a 90 minutos. A existência de complicações intra-procedimento também foi considerada como um preditor para um tempo >90 minutos. Uma lesão >20 mm e a localização no terço superior estiveram associadas independentemente com um tempo de procedimento superior a 90 minutos (OR 4.91 [IC 95% 2.29-10.50] e OR 18.26 [IC 95% 2.02-164.78], respetivamente). CONCLUSÕES: O tempo de procedimento da ESD para lesões superficiais gástricas é influenciado pelo tamanho e localização das lesões, sendo preditores de um tempo de procedimento superior a 90 minutos. Estes dados são importantes para melhor gestão da dinâmica de trabalho, procedimento, formação das equipas e procedimentos anestésicos.