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Background: The prevalence of complex chronic conditions (CCC), which cause serious limitations and require specialized care, is increasing. The diagnosis of a CCC is a health-illness transition for children and their parents, representing a long-term change leading to greater vulnerability. Knowing the characteristics of these transitional processes is important for promoting safe transitions in this population. This scoping review aimed to map the available evidence on health-illness transition processes in children with complex chronic conditions and their parents in the context of healthcare., Methods: Six databases were searched for studies focusing on children aged 0-21 years with CCC and their parents experiencing health-illness transition processes, particularly concerning adaptation to illness and continuity of care, in the context of healthcare. Studies within this scope carried out between 2013 and 2023 and written in Portuguese or English were identified. The articles were selected using the PRISMA methodology. The data were extracted to an instrument and then presented with a synthesizing approach supporting the interpretation of the results., Results: Ninety-eight methodologically broad but predominantly qualitative articles were included in this review. Children with CCC have specific needs associated with complex and dynamic health-illness transitions with a multiple influence in their daily lives. Several facilitating factors (p.e. positive communication and a supportive therapeutic relationship with parents and professionals, as well as involvement in a collaborative approach to care), inhibiting factors (p.e. the complexity of the disease and therapeutic regime, as well as the inefficient organization and coordination of teams) and both positive (p.e. well-being and better quality of life) and negative response patterns (p.e. negative feelings about the chronic illness) were identified. Some interventions to support the transitional process also emerged from the literature. Pediatric palliative care is seen as a good practice and an integrative approach for these children and families., Conclusion: Health professionals play a fundamental role in supporting the transitional process and promoting positive response patterns. More significant investment is needed at the clinical and academic levels regarding production and dissemination of knowledge in this area to ensure the awareness of children with CCC and that their needs are fully enhanced., Review Registration: https://doi.org/10.17605/OSF.IO/QRZC8 ., (© 2024. The Author(s).)

Purpose: To evaluate the progression of chorioretinal atrophic areas associated with myopic choroidal neovascularization (CNV) in high myopic patients followed by a minimum period of 10 years., Patients and Methods: Patients with myopic CNV lesions that achieved clinical and structural remissions over 10 years of follow-up were included. Medical records were reviewed for CNV characterization and treatment, best-corrected visual acuity at baseline (BCVA0), immediately after the last treatment (BCVA1) and at the latest visit (BCVA2). Fundus autofluorescence (FAF) was used to quantify the amount of atrophic area increase per year associated with the treated myopic CNV lesion. The first FAF performed after treatment suspension (FAF1) was compared with the most recent exam (FAF2)., Results: Thirty-six eyes from 36 patients were included. Mean total follow-up was 12.38 ± 2.68 years. Mean number of intravitreal injections (IVI) was 12.50 ± 12.40 and 25% of the eyes had previous treatment with photodynamic therapy (PDT). Mean improvement between BCVA0 and BCVA1 was 5.58 ± 15.98 letters (p < 0.001). However, a drop of 8.03 ± 12.25 letters was noticed between BCVA1 and BCVA2. FAF1 was 6.34 ± 4.92mm 2 and increased to 9.88 ± 7.56mm 2 (3.54 ± 3.79mm 2 variation p < 0.001). The mean growth rate of the atrophic area was 0.89 ± 0.84mm 2 per year. BCVA2 negatively correlated with FAF2 (k = -0.498, p = 0.002) being worse in patients with higher atrophic area growth rate (k = -0.341, p = 0.042). Eyes treated with PDT needed less IVI (5.89 ± 5.21 vs 14.70 ± 13.36, p = 0.008) but had larger FAF1 (9.80 ± 5.33 vs 5.19 ± 4.27, p = 0.013) and FAF2 (16.05 ± 7.10 vs 7.83 ± 6.63, p = 0.003). Hypothyroidism was associated with higher atrophy growth rate (1.55 ± 1.15 vs 0.73 ± 0.67, p = 0.016)., Conclusion: This research demonstrates the importance of chorioretinal atrophy progression after myopic CNV lesions regression and its impact on visual prognosis, reporting a mean yearly growth of 0.89 mm 2 in atrophic areas. Previous treatment with PDT and hypothyroidism were identified as risk factors associated with larger atrophic areas and worse visual outcomes., Competing Interests: The authors have no conflicts of interest to declare in this work., (© 2024 Ferreira et al.)

The purpose of this clinical report is to describe a 10-year clinical outcome of advanced surface ablation with photorefractive keratectomy (PRK) in a patient who had been previously incorrectly diagnosed with keratoconus (KC). Corneal ectasia is a rare but extremely relevant complication of laser vision correction, and KC represents a major contraindication for these procedures. Nonetheless, some surface ablation procedures, such as PRK, might be a valid option for particular patients with atypical corneal topography or subclinical or mild forms of KC. Patient education and complete preoperative refractive multimodal imaging are essential for a more conscious therapeutic decision, minimizing iatrogenic ectasia, as well as decreasing the number of patients who are incorrectly denied refractive surgery, as was the patient presented in this study., Competing Interests: The authors have declared financial relationships, which are detailed in the next section., (Copyright © 2024, Vilares Morgado et al.)

Purpose: To report clinical findings and prognostic factors for visual and morphological outcomes in patients with Acanthamoeba keratitis (AK)., Methods: Single-center, retrospective, longitudinal study of 51 cases of AK diagnosed by real-time polymerase chain reaction (RT-PCR) between March 2010 and October 2022. The primary outcome was the final best corrected visual acuity (BCVA). Poor visual outcome was defined as a final BCVA ≥ 1 logMAR unit, while good visual outcome was defined as a final BCVA < 1 logMAR unit. Eyes from these two groups were compared, regarding demographic and initial clinical variables, anti-Acanthamoeba treatment used, and complications of the disease. Early diagnosis was defined as ≤ 14 days from symptom onset to diagnostic confirmation and initiation of Acanthamoeba medical treatment. Multivariable logistic regression was used to determine predictors of poor visual outcome., Results: A total of 51 eyes from 46 patients diagnosed with AK, all contact lens (CL) wearers, were included in this study. Average follow-up was 39.0 ± 30.2 [total range 14-120] months. Thirty-one eyes (60.8 %) presented good visual outcome, with a lower baseline age (30.5 ± 9.0 vs. 42.3 ± 15.8; p = 0.020), better initial BCVA (0.8 ± 0.7 logMAR units vs. 1.3 ± 0.9 logMAR units; p = 0.047), higher rate of early diagnosis (45.2 % vs. 5.6 %; p = 0.004), and higher rate of therapeutic epithelial debridement (64.5 % vs. 10 %; p < 0.001). 20 eyes (39.2 %) presented poor visual outcome, with 12 eyes undergoing evisceration/enucleation (23.5 %). These 20 eyes presented a higher rate of complications (90 % vs. 61.3 %; p = 0.031). In multivariable analysis, early diagnosis of AK (OR 19.78; 95 % CI 2.07-189.11; p = 0.010) and therapeutic epithelial debridement (OR 19.02; 95 % CI 3.27-110.57; p = 0.001) were associated with a good visual outcome., Conclusions: In the present study, poor visual outcome was present in 39 % of affected eyes. Early AK diagnosis (≤14 days from symptom onset) and therapeutic epithelial debridement were associated with good final visual outcome., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: To report the 4-year outcomes of transepithelial accelerated corneal collagen crosslinking (TE-ACXL) in the treatment of eyes with progressive keratoconus (KC)., Methods: Eyes of patients who underwent TE-ACXL (6mW/cm 2 for 15 minutes) for progressive KC and presented 48 months of follow-up were included. Corrected distance visual acuity (CDVA), keratometry measurements (Kmax, maximum keratometry, Kmean, mean keratometry and Astg, corneal astigmatism), thinnest corneal thickness (PachyMin), and topographic, and tomographic indices (specifically the posterior radius of curvature from the 3.0 mm centered on the thinnest point of the cornea (PRC), and the D-index) were analysed preoperatively and every 12 months after TE-ACXL, up to 48 months. Progression after TE-ACXL was considered when eyes presented ≥1 criteria: (1) increase of ≥1D in Kmax or increase of ≥0.75D in Kmean or increase of ≥1D in Astg; (2) reduction of ≥0.085 mm in PRC; (3) decrease ≥5% in PachyMin., Results: 41 eyes from 30 patients were included, with a mean age at crosslinking of 20.90±4.69 years. There was a significant increase in Kmean (+0.64±1.04 D, p <0.001; +0.98 ± 1.49 D, p <0.001; +1.27±2.01 D, p <0.001; +1.13±2.00 D, p =0.006) and a significant decrease in PRC throughout follow-up (-0.12±0.22, p=0.002; -0.15±0.24, p <0.001; -0.17±0.43, p =0.021; -0.16±0.43, p =0.027). PachyMin decreased significantly at 36 and 48 months (-8.50±15.93 μm, p =0.004; -7.82±18.37, p =0.033). According to our progression criteria, there was a major progression rate throughout follow-up (57.1%, 61.1%, 58.8%, and 67.9%, respectively). Surgery and follow-up were uneventful in all subjects. Eleven eyes (26.8%) required further procedures, ≥36 months after the initial TE-ACXL, due to persistent progressive disease., Conclusion: TE-ACXL proved to be a safe therapeutic option for progressive KC. However, its efficacy is deemed unsatisfactory, as a notable proportion of affected eyes may continue to advance within a 4-year timeframe, necessitating additional procedures to halt the disease's course., Competing Interests: The authors have no conflicts of interest to declare for this work., (© 2024 Vilares-Morgado et al.)

Competing Interests: The authors have no conflicts of interest to declare for this communication.

Purpose: To evaluate whether repeated intravitreal injections (IVI) with an anti-vascular endothelial growth factor (anti-VEGF) agent are associated with glaucomatous progression in eyes with glaucoma spectrum diseases (GSD)., Methods: Single-center, retrospective, longitudinal study of patients with bilateral and similar GSD who: (1) received ≥8 IVI in only one eye during the study period; (2) had ≥2 retinal nerve fiber layer thickness (RNFL) measurements obtained by spectral-domain optical coherence tomography (SD-OCT) at least 12 months apart. The primary outcome was the absolute RNFL thickness change, comparing injected and fellow uninjected eyes. Linear mixed effects models were constructed, including a multivariable model., Results: Sixty-eight eyes from 34 patients were included, 34 injected and 34 fellow uninjected eyes. Average baseline age was 67.68±21.77 years with a follow-up of 3.66±1.89 years and 25.12±14.49 IVI. RNFL thickness decreased significantly from 80.92±15.78 to 77.20±17.35 μm ( p <0.001; -1.18±1.93 μm/year) in injected eyes and from 79.95±17.91 to 76.61±17.97 μm ( p <0.001; -1.07±0.98 μm/year) in uninjected eyes. In a multivariable linear mixed model of injected eyes, only higher baseline RNFL thickness (p < 0.001) significantly predicted higher absolute RNFL thickness loss. Neither absolute RNFL thickness variation ( p =0.716) nor RNFL rate ( p =0.779) was significantly different between paired injected and uninjected eyes. Absolute IOP variation was not significantly different between groups (16.62±4.77 to 15.09±4.34 mmHg in injected eyes and 17.68±5.01 to 14.50±3.39 mmHg in fellow uninjected eyes; p =0.248). The proportion of eyes receiving glaucoma medical treatment increased significantly in both groups (55.9% to 76.5% in injected eyes; p =0.039; 58.8% to 76.5% in uninjected eyes; p = 0.031). The number of glaucoma medications also increased significantly in both groups (1.03±1.11 to 1.59±1.18 glaucoma medications in injected eyes; p =0.003; 1.09±1.11 to 1.56±1.19 glaucoma medications in uninjected eyes; p =0.003)., Conclusion: Repeated IVI do not seem to accelerate glaucomatous progression. Future studies with a longer follow-up are needed., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 Vilares-Morgado et al.)

Diagnosing polycystic ovary syndrome (PCOS) during adolescence is challenging since normal pubertal development overlap typical features of this syndrome. The authors aim to summarize the existing evidence concerning PCOS in adolescence, particularly its diagnostic criteria and therapeutic options. A search throughout medical databases such as PubMed and MedScape was performed. Diagnostic criteria include irregular menstrual cycles according to time postmenarche and evidence of clinical hyperandrogenism and/or biochemical hyperandrogenism, provided other causes have been excluded. Polycystic ovarian morphology ought not to be used as a diagnostic criterion. Treatment should target manifestations and/or comorbidities, even in the absence of a definite diagnosis. Lifestyle interventions are the first-line treatment. Combined oral contraceptives, metformin or antiandrogens may also be considered as adjuvants. Screening for PCOS in adolescence is crucial as it allows an early intervention on the symptoms and comorbidities presented leading to better long-term reproductive and metabolic outcomes., Competing Interests: The authors have no conflict of interests to declare., (Federação Brasileira de Ginecologia e Obstetrícia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Introduction: Endoscopy remains the exam of choice in the evaluation of activity in Crohn's disease (CD) after surgery (ACD-AS). However, intestinal ultrasound (IUS) may represent a noninvasive alternative. The objective of this study is to determine the diagnostic accuracy of this modality compared to endoscopy., Material and Methods: This is a cross-sectional study, comprising a period of 14 months, carried out in patients with established CD and ileocecal resection due to the disease. IUS (HI-VISION Avius®, Tokyo, Japan) was performed with linear probe B-mode/Doppler prior to ileocolonoscopy. IUS and ileocolonoscopy were performed on the same day by 2 specialists in Gastroenterology dedicated to ultrasound and inflammatory bowel disease, in a double-blind mode. Collected demographic and clinical data (Harvey-Bradshaw Index [HBI]; remission ≤4), serological/fecal inflammatory parameters (leukocytes [4-10 × 10 9 cells/L], C-reactive protein [≤0.5 mg/dL], and fecal calprotectin [<50 mg/kg]), endoscopy (Rutgeerts score: remission 3 mm and/or Limberg score >1) was abnormal in 61.5% ( n = 24) of the cases. Endoscopic remission (Rutgeerts score

Background: High-quality cardiopulmonary resuscitation (CPR) remains essential to improve the outcome of patients in sudden cardiorespiratory arrest. Feedback on performance is a crucial component of the learning processes associated with simulation and has been shown to improve CPR quality during simulated cardiac arrest on mannequins. This study aims to evaluate skills acquisition using a new low-cost feedback device for CPR self-training when compared to standard training methods., Methods: Thirty-nine pregraduated medical and biomedical engineering students were recruited for a longitudinal double-blinded randomized control study. For training Basic Life Support skills, the control group used a standard task-trainer and received feedback from an instructor. The intervention group used the same standard task-trainer, instrumented with the CPR Personal Trainer that provided automated performance feedback (with no instructor) on compression-related parameters. Students' knowledge and skills were assessed before and after training, through a theoretical knowledge test and 2 minutes of CPR practical performance., Results: The theoretical tests showed an improvement both in the intervention and in the control group. For each compression-related parameters (hands position, recoil, rate, and depth), significant increase in scores is observed, between the pre- and the post-test, in both groups. The intervention and control groups presented identical mean differences for the total score (0.72 vs 0.72), with no statistical difference ( P  = 0.754)., Conclusions: The proposed tool proved to be effective in the acquisition of compression-related skills, with similar outcomes as the traditional instructor-based method, corroborating the hypothesis that a low-cost tool with feedback for CPR self-training can provide an alternative or a complementary extension to traditional training methods. The system can also be considered cost-efficient as it reduces the permanent presence of an instructor for the chest compressions training, promoting regular training outside formal training courses., Competing Interests: Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article.The authors declare no conflicts of interest., (Copyright © 2018 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of PBJ-Associação Porto Biomedical/Porto Biomedical Society. All rights reserved.)

Introduction: Ulcerative colitis (UC) is a chronic disease but its progressive character, with structural damage, is insufficiently studied., Objectives: To analyze a group of patients without referral bias, regarding its clinical course, the morphological damage, and functional status., Methods: We evaluated UC patients diagnosed between January 1, 2000 and December 31, 2004, living in the direct referral area of the hospital and determined the medication use, colectomy rate, structural damage ("lead pipe," stenosis, pseudopolyps, fibrous bridges), and anorectal function (prospective evaluation with the Cleveland Clinic Incontinence Score [CCIS] and the Fecal Incontinence Quality of Life Scale)., Results: We identified 104 patients, 47% female, with a mean age at diagnosis of 38 ± 17 years, 24% with proctitis, 57% with left colitis, and 19% with pancolitis. In 3 patients, it was not possible to obtain follow-up data. Of the studied patients, 56% needed corticosteroid therapy, 38% immunosuppressants, and 16% anti-tumor necrosis factors (anti-TNFs). After a mean follow-up of 13 ± 2 years, we found structural damage in 25 patients (24%): 5% with proctocolectomy, 15% with "lead pipe," 16% with pseudopolyps, and 3% with stenosis and fibrous bridges. Reference to functional anorectal disorders was identified in 49%, mostly previous and self-limited episodes of incontinence, but including persistent incontinence in 10% (CCIS 8 ± 4.8). There was an increased incidence of structural damage and anorectal dysfunction in patients who needed corticosteroid therapy ( p = 0.001), immunosuppressants ( p < 0.001), and anti-TNFs ( p = 0.002) and an association of structural damage with anorectal dysfunction ( p < 0.001). There was no association between age and anorectal dysfunction, including incontinence episodes., Conclusions: UC is a disease with structural and functional consequences in a significant subset of patients. This should be incorporated when defining the therapeutic strategy.

Introduction: We intend to evaluate clinically, topographically and morphologically all surgical specimens sent by the Department of Ophthalmology of Hospital de Braga to the Department of Pathology of the same hospital., Material and Methods: Two hundred and fifty eight surgically obtained specimens, from the Department of Ophthalmology of Hospital de Braga, analyzed in the Department of Pathology, from January 2002 to June 2015, were characterized. Data was arranged according to year, age, sex, topography and morphological diagnosis according to the SNOMED® coding system., Results: Mean age at time of diagnosis was 54.6 years old; 52.3% were male subjects. The number of specimens was relatively stable until the year 2010, with a significant increase between 2011 and 2013. Most specimens sent corresponded to eyelid (54.7%), followed by conjunctiva (26.7%); the most common pathological diagnosis was malignant epithelial lesions (22.48%), followed by melanocytic tumours (22.09%) and benign epithelial lesions (17.05%)., Discussion: The results are distinct from previous publications presumably because of differences between the populations submitted to analysis., Conclusion: This is the first indexed publication characterizing surgical specimens from a Department of Ophthalmology in Portugal; moreover, it also includes an extensive review of global epidemiological data about ophthalmic surgical specimens.

Introduction: Sinistral, or left-sided, portal hypertension (SPH) is a rare entity, with multiple potential causes. Gastrointestinal variceal bleeding and hypersplenism are its' major clinical manifestations. The main aim of the present study is to summarize the clinical features of patients with SPH., Patients and Methods: This was a retrospective analysis of consecutive patients with present or previous diagnosis of SHP, observed in a Gastroenterology Department, in a period of 2 years. Patients with clinical, radiological or laboratory alterations suggestive of cirrhosis were excluded. Causes of SPH, clinical manifestations and outcomes were registered. Potential factors associated with gastrointestinal bleeding were analyzed., Results: In the study period a total of 22 patients (male - 17; mean age - 59.6 ± 10.6 years) with SHP were included. Clinical manifestations were: asymptomatic/unspecific abdominal pain (n = 14); gastrointestinal bleeding (n = 8). Eleven (50%) patients had increased aminotransferases, GGT and/or alkaline phosphatase although liver function was normal in all of them. Causes of SPH were chronic pancreatitis (n = 7), acute pancreatitis (n = 7), pancreatic cancer (n = 4), pancreatic surgery (n = 3) and arteriovenous malformation (n = 1). All patients had gastric and/or esophageal varices and seven had splenomegaly. Five (22.7%) had thrombocytopenia, associated with hypersplenism. Five patients (22.7%) were submitted to endoscopic treatment and eight were submitted to splenic artery embolization and/or splenectomy. There were no cases of variceal rebleeding and two patients died. Patients without liver enzymes elevation had a higher probability of gastrointestinal bleeding (87.5% vs. 28.6%; p  = 0.024)., Conclusions: Acute and chronic pancreatitis are the major causes of SHP. Gastrointestinal bleeding is the most important clinical manifestation and patients without liver enzyme elevation seem more prone to bleed. Specific treatment is seldom performed or needed.

Intestinal ganglioneuromatosis is a rare disorder of the enteric nervous system. It is often associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2b but, more rarely, it can present in a sporadic and isolated form. A 66-year-old man presented with a 14-year history of iron deficiency anaemia, with no visible bleeding, requiring occasional blood transfusions. Haematological causes of anaemia were thoroughly excluded, and conventional endoscopic and radiological examinations showed no lesions. Capsule enteroscopy identified an ulcerated stenosis in the small bowel, but the biopsies taken at balloon enteroscopy were inconclusive. The patient underwent a laparotomy, which revealed a stiff and ulcerated stenosis in the ileum. Histological analysis demonstrated the presence of diffuse intestinal ganglioneuromatosis. Three years after surgery, the patient is asymptomatic, with normal haemoglobin levels. This case demonstrates an isolated form of intestinal ganglioneuromatosis, with an atypical presentation, difficult to diagnose despite an exhaustive evaluation., (2015 BMJ Publishing Group Ltd.)

Background: Ulcerative colitis (UC) is associated with an increased risk of colorectal cancer (CRC). Aberrant crypt foci (ACF) are important biomarkers of sporadic CRC risk. Their correlation with the risk of intraepithelial neoplasia (IN) in UC remains unclear., Aims: To assess whether ACF are a risk factor for IN in long-standing UC and to investigate any correlation between the clinico-epidemiological characteristics and prevalence/number of ACF in these patients., Methods: Seventy-six patients with long-standing UC were prospectively screened by colonoscopy with chromoendoscopy-guided endomicroscopy. ACF were sought in the lower rectum., Results: Eight INs were detected in seven (9.2%) patients. The ACF prevalence and mean number were 60.5% and 2.4 ± 2.8, respectively. The number of ACF was independently associated with the risk of having IN (odds ratio = 1.338; 95% confidence interval 1.030-1.738). ACF number revealed a good calibration (area under the receiver operating characteristic curve = 0.829) and discriminative ability (p = 0.205, Hosmer-Lemeshow test) for the prediction of synchronous IN. Patients with ≥3 ACF have a significantly higher prevalence of IN than patients with <3 ACF (22.6% vs. 0%, p = 0.001). Using this cut-off value, the performance of ACF in predicting the presence of IN was as follows: sensitivity = 100%, specificity = 65.2%, positive predictive value = 22.6%, and negative predictive value = 100%. Age >40 years, family history of CRC, and increased body mass index (BMI) were associated with a significantly higher number of ACF., Conclusion: Long-standing UC patients with ≥3 ACF have a significantly higher likelihood of having IN. Age >40 years, family history of CRC, and increased BMI have significant positive associations with the number of ACF.

Ileal intussusception is the invagination of the small intestine within itself and accounts for 1% of cases of acute obstruction. However, physicians do not initially consider intussusception as a possible diagnosis of obstruction due to its rarity in adults. Herein, we report the case of a 22-year-old male who was admitted to the Emergency Department with continuous abdominal pain. Ultrasonography and computed tomography revealed an ileal intussusception. The patient underwent surgical removal of the segment of the small bowel. Unexpectedly, pathology revealed that the invagination occurred due to a parasite egg, with features suggestive of Schistosoma species. Schistosomiasis, although considered a parasitic disease in tropical countries, is not absent from Europe and though it is highly improbable, it may be responsible for cases of intussusception in adults.

Purpose: NOD2 mutations have been linked to an increased risk of Crohn's disease and to some of its phenotypes. The association between NOD2 mutations and susceptibility to ulcerative colitis (UC) remains somewhat controversial and potential correlations between these mutations and UC phenotype have not been studied., Aim: To assess whether NOD2 mutations are a risk factor for UC in Portugal and if there are any genotype-phenotype correlations in these patients., Methods: The three main NOD2 mutations were searched in 200 patients with UC and in 202 healthy controls., Results: NOD2 mutations were present in 28 patients with UC (14.0 %) and in 27 controls (13.4 %) (p = 0.853). Mutation carriers were more likely to receive steroids during the first year of disease than non-carriers (54.2 % vs. 29.6 %, p = 0.018) and among these patients the need for intravenous administration was more frequent in those with the R702W polymorphism (90.0 % vs. 45.5 %, p = 0.014). In patients with severe colitis admitted for intravenous steroids, a greater proportion of mutation carriers was considered intravenous-steroid refractory and required salvage therapy (90.0 % vs. 38.1 %, p = 0.004). Patients with NOD2 mutation were submitted to colectomy more frequently than non-carriers (17.9 % vs. 4.1 %. p = 0.015). No correlation with the need for immunosuppressants/immunomodulators was found., Conclusions: In the Portuguese population, NOD2 mutations do not increase the risk of UC but are associated with a more aggressive course including greater need of steroids in the first year, increased incidence of intravenous-steroid refractoriness and a higher colectomy rate.

Background: Audit Trails (AT) are fundamental to information security in order to guarantee access traceability but can also be used to improve Health information System's (HIS) quality namely to assess how they are used or misused. This paper aims at analysing the existence and quality of AT, describing scenarios in hospitals and making some recommendations to improve the quality of information., Methods: The responsibles of HIS for eight Portuguese hospitals were contacted in order to arrange an interview about the importance of AT and to collect audit trail data from their HIS. Five institutions agreed to participate in this study; four of them accepted to be interviewed, and four sent AT data. The interviews were performed in 2011 and audit trail data sent in 2011 and 2012. Each AT was evaluated and compared in relation to data quality standards, namely for completeness, comprehensibility, traceability among others. Only one of the AT had enough information for us to apply a consistency evaluation by modelling user behaviour., Results: The interviewees in these hospitals only knew a few AT (average of 1 AT per hospital in an estimate of 21 existing HIS), although they all recognize some advantages of analysing AT. Four hospitals sent a total of 7 AT - 2 from Radiology Information System (RIS), 2 from Picture Archiving and Communication System (PACS), 3 from Patient Records. Three of the AT were understandable and three of the AT were complete. The AT from the patient records are better structured and more complete than the RIS/PACS., Conclusions: Existing AT do not have enough quality to guarantee traceability or be used in HIS improvement. Its quality reflects the importance given to them by the CIO of healthcare institutions. Existing standards (e.g. ASTM:E2147, ISO/TS 18308:2004, ISO/IEC 27001:2006) are still not broadly used in Portugal.

Background: Propolis is a resin collected by bees from plant buds and exudates, which is further processed through the activity of bee enzymes. Propolis has been shown to possess many biological and pharmacological properties, such as antimicrobial, antioxidant, immunostimulant and antitumor activities. Due to this bioactivity profile, this resin can become an alternative, economic and safe source of natural bioactive compounds.Antitumor action has been reported in vitro and in vivo for propolis extracts or its isolated compounds; however, Portuguese propolis has been little explored. The aim of this work was to evaluate the in vitro antitumor activity of Portuguese propolis on the human colon carcinoma cell line HCT-15, assessing the effect of different fractions (hexane, chloroform and ethanol residual) of a propolis ethanol extract on cell viability, proliferation, metabolism and death., Methods: Propolis from Angra do Heroísmo (Azores) was extracted with ethanol and sequentially fractionated in solvents with increasing polarity, n-hexane and chloroform. To assess cell viability, cell proliferation and cell death, Sulforhodamine B, BrDU incorporation assay and Anexin V/Propidium iodide were used, respectively. Glycolytic metabolism was estimated using specific kits., Results: All propolis samples exhibited a cytotoxic effect against tumor cells, in a dose- and time-dependent way. Chloroform fraction, the most enriched in phenolic compounds, appears to be the most active, both in terms of inhibition of viability and cell death. Data also show that this cytotoxicity involves disturbance in tumor cell glycolytic metabolism, seen by a decrease in glucose consumption and lactate production., Conclusion: Our results show that Portuguese propolis from Angra do Heroísmo (Azores) can be a potential therapeutic agent against human colorectal cancer.

Chemical investigation of the aerial part and the roots of Euphorbia hyberna L. subs. hyberna. resulted in the isolation and identification of four triterpenoids (3β-O-octadecanoyllupeol (1), glut-5-en-3β-ol (2), 24-methylenecicloartan-3β-ol (3) and cicloart-23-ene-3β,25-diol (6)) along with the phenolic compounds ellagic acid (4) and 3,3'-di-O-methylellagic acid (7). Although these are all known compounds, this is the first report of their isolation from this plant. Their structures were elucidated on the basis of spectral methods, including 2D NMR experiences, and confirmed by comparing with the literature data.

A new xanthone (2), 3-(2-hydroxyethoxy)xanthone, and three known compounds, 1-hydroxyxanthone, salicylic acid and the sesquiterpene oplopanone, were isolated for the first time from the rhizomes of Hedychium gardnerianum Rosc. The structures of these compounds were elucidated on the basis of detailed spectral analysis, including 2D NMR experiments.

A new diterpene tetraester, from the jatrophane family, and two new diterpene triesters, with a lathyrane skeleton, have been isolated from the chloroform extract of the roots of Euphorbia hyberna L. The structures of these compounds have been established by spectroscopic methods, including 2D NMR experiments.

An isolate, designated strain FS-1T, was recovered from a ripe fig. Phylogenetic analysis of the 16S rRNA genes and DNA-DNA reassociation values showed that the organism represented a novel species of the genus Leuconostoc closely related to Lactobacillus fructosus. The novel isolate could be distinguished from the type strain of Lactobacillus fructosus by the fatty acid composition and several phenotypic and growth characteristics. In strain FS-1T, 18:1 delta9 (18:1omega9c) was present in relatively large amounts whilst, in Lactobacillus fructosus, this fatty acid was a minor component. Strain FS-1T and Lactobacillus fructosus produced acid in API 50CHL microtubes from glucose, fructose and mannitol within 48 h, whereas only strain FS-1T also fermented trehalose, gluconate, turanose and sucrose after 48 h. Other differences in acid production from carbohydrates also distinguished strain FS-1T from Lactobacillus fructosus. Both organisms were heterofermentative with fructose as a substrate and fermented glucose only in the presence of fructose, as determined by nuclear magnetic resonance studies. Strain FS-1T was catalase-positive. On the basis of the phylogenetic analysis, DNA-DNA reassociation values, physiological and biochemical characteristics and fatty acid composition, the name Leuconostoc ficulneum is proposed for the novel species represented by strain FS-1T, and it is proposed that Lactobacillus fructosus be reclassified in the genus Leuconostoc as Leuconostoc fructosum comb. nov.

Thin-layer chromatographic analysis of the polar lipids of Meiothermus strains revealed two glycolipid bands with similar chromatographic mobility to the major glycolipid of Thermus strains. In this study the glycolipids from the type strains of Meiothermus ruber, Meiothermus chliarophilus, Meiothermus silvanus and Meiothermus cerbereus were characterized using GC, GC/MS, fast atom bombardment MS and chemical methods. All strains contained dihexosyl-(N-acyl)hexosaminylglucosyl diacylglycerols, related in structure to the major glycolipid of Thermus strains but varying in their fatty acylation pattern. The detection of two glycolipid bands by TLC in Meiothermus spp. was attributable to the invariable presence of 2-hydroxyacyl groups N-linked to the hexosamine of the polar head group which cause the glycolipids to be more strongly retained on silica TLC plates than 3-hydroxy or non-hydroxylated N-acyl glycolipids of similar structure that are also present. M. silvanus contained, in addition to these glyceroglycolipids, several glycolipids which were linked to acylated branched octadecanediols rather than to glycerol. The presence of glycolipids containing 2-hydroxyacyl groups N-linked to hexosamine appears to be a stable phenotypic marker that distinguishes the genus Meiothermus from the genus Thermus.