Validation of the IBD-Disk in a Portuguese cohort.

OBJECTIVE: The aim of this study was to validate the IBD-Disk in a Portuguese cohort according to the COnsensus-based Standards for the selection of the health Measurement INstruments (COSMIN) recommendations. METHODS: After translation of the original IBD-Disk to Portuguese, a group of IBD patients was invited to complete the IBD-Disk at baseline (T0), after 1-4 weeks (T1) and after more than 3 months (T2), from July 2020 to February 2021. At T0 and T2, the patients also completed the IBD Disability Index. We evaluated reliability (internal consistency, test-retest, interrater reliability and measurement error), construct validity, responsiveness, interpretability of IBD-Disk, sociodemographic and clinical factors associated with IBD-Disk. RESULTS: At T0, 154 patients (107 - Crohn's disease; 46 - ulcerative colitis) completed the IBD-Disk; 1 - IBD-unclassified). At T1 and T2, 64 and 114 patients repeated the questionnaire, respectively. Factor analysis confirmed the unidimensionality of the scale and reduced the final version to 10 items. Internal consistency was excellent with a Cronbach's α of 0.916. The intraclass correlation coefficient was 0.95 for test-retest (T0 and T1). To evaluate construct validity, the IBD-Disk was compared with the IBD Disability Index and Manitoba IBD Index with a significative positive correlation (r = 0.850 and r = 0.459, respectively; P < 0.001 for both). IBD-Disk scores ranged from 0 to 93 with a mean of 38.18 ± 25.39. Female sex, professional inactivity, longer IBD duration and clinical activity were associated with significantly higher IBD-Disk scores. CONCLUSION: The Portuguese version of IBD-Disk is a reliable and valid tool to assess disability in Portuguese IBD patients.

Physiopathology of late-onset fetal growth restriction.

Fetal growth restriction (FGR) is defined as the inability of the fetus to reach its potential for genetic determination. FGR can have several causes, including genetic syndromes, chromosomal diseases, and infections; however, a vast majority of cases are probably attributed to impaired uterine and placental circulation. The relationships between abnormal placental development and FGR are complex, and studies are generally few, presenting confounding factors. Damage to the uteroplacental circulation associated with vasculogenesis and villus angiogenesis dysfunction are the main factors involved in subsequent FGR. The main receptors involved in FGR include hypoxia-inducible factor (HIF 1, 2, and 3), vascular endothelial growth factor (VEGF), placental growth factor (PlGF), vascular endothelial growth factor C (VEGF-C), soluble Flt-1, soluble endoglin (Seng), angiopoietin-1 and -2 (Ang-1 and Ang-2), tyrosine kinase receptor 1 (Flt-1), tyrosine kinase receptor 2 (Flt-2), vascular endothelial growth factor receptor (VEGFR) 1, 2 and 3, kinase domain receptor (KDR), and vascular endothelial growth factor receptor A (VEGFR-A). Furthermore, failure in trophoblastic invasion and remodeling of spiral arteries has been associated with FGR owing to poor placental perfusion. There are several possible causes for poor remodeling of spiral arteries, which probably vary on a case-to-case basis. Changes in the placental form, macroscopic and microscopic vascular lesions, inflammation, and genetic changes are also related to FGR. Based on gestational age at diagnosis, FGR can be classified as early- (˂32 weeks) and late-onset (≥32 weeks). Moreover, there exist several theories regarding possible pathophysiological differences between early- and late-onset FGR, with some postulating that it the same disease but at different stages or severity. Another hypothesis suggests that the change in the trophoblastic invasion of spiral arteries would be milder. In this article, we address the main mechanisms described in the pathophysiology of FGR and, later, the specific findings in late-onset FGR.

What recovery position should patients adopt after percutaneous liver biopsy?

INTRODUCTION: Percutaneous liver biopsy (PLB) is an invasive procedure used for the assessment of liver diseases. The patient's recovery position after the PLB differs among hospitals and departments. This study aims to evaluate adverse events and patient acceptability according to the recovery position adopted after the PLB. PATIENTS AND METHODS: From September 2014 to March 2017, patients submitted to PLB were randomly assigned to a recovery position arm: right-side position (RRP), dorsal position (DRP), or combined position. A validated numerical rating scale was used to evaluate the level of pain and the overall acceptability of the PLB experience. RESULTS: Ninety (27 patients in RRP, 33 in DRP and 30 in combined position arm) patients were included in the study. There were no differences between the three groups regarding demographic and clinical parameters, except for the number of previous biopsies - higher in the combined group (P=0.03). No major adverse events occurred. Minor complications described were pain (36.7% of patients), vasovagal reaction (2.2%) and nauseas/vomit (3.3%). Pain level and pain duration did not differ significantly between groups. Pain occurred more often in women (P=0.04) and younger patients (P=0.02). The number of passages, operator and previous biopsy did not influence the occurrence of pain. The RRP group considered the procedure less acceptable than the DRP group (P=0.001) or the combined group (P=0.002). There were no differences between the last two arms. CONCLUSION: Although RRP is the most frequently used position, it appears to be less acceptable without any protective role in terms of adverse events.

Mutation of the Surface Layer Protein SlpB Has Pleiotropic Effects in the Probiotic Propionibacterium freudenreichii CIRM-BIA 129.

Propionibacterium freudenreichii is a beneficial Gram-positive bacterium, traditionally used as a cheese-ripening starter, and currently considered as an emerging probiotic. As an example, the P. freudenreichii CIRM-BIA 129 strain recently revealed promising immunomodulatory properties. Its consumption accordingly exerts healing effects in different animal models of colitis, suggesting a potent role in the context of inflammatory bowel diseases. This anti-inflammatory effect depends on surface layer proteins (SLPs). SLPs may be involved in key functions in probiotics, such as persistence within the gut, adhesion to host cells and mucus, or immunomodulation. Several SLPs coexist in P. freudenreichii CIRM-BIA 129 and mediate immunomodulation and adhesion. A mutant P. freudenreichii CIRM-BIA 129ΔslpB (CB129ΔslpB) strain was shown to exhibit decreased adhesion to intestinal epithelial cells. In the present study, we thoroughly analyzed the impact of this mutation on cellular properties. Firstly, we investigated alterations of surface properties in CB129ΔslpB. Surface extractable proteins, surface charges (ζ-potential) and surface hydrophobicity were affected by the mutation. Whole-cell proteomics, using high definition mass spectrometry, identified 1,288 quantifiable proteins in the wild-type strain, i.e., 53% of the theoretical proteome predicted according to P. freudenreichii CIRM-BIA 129 genome sequence. In the mutant strain, we detected 1,252 proteins, including 1,227 proteins in common with the wild-type strain. Comparative quantitative analysis revealed 97 proteins with significant differences between wild-type and mutant strains. These proteins are involved in various cellular process like signaling, metabolism, and DNA repair and replication. Finally, in silico analysis predicted that slpB gene is not part of an operon, thus not affecting the downstream genes after gene knockout. This study, in accordance with the various roles attributed in the literature to SLPs, revealed a pleiotropic effect of a single slpB mutation, in the probiotic P. freudenreichii. This suggests that SlpB may be at a central node of cellular processes and confirms that both nature and amount of SLPs, which are highly variable within the P. freudenreichii species, determine the probiotic abilities of strains.

Digital Rectal Examination and Balloon Expulsion Test in the Study of Defecatory Disorders: Are They Suitable as Screening or Excluding Tests?

Background. Rome III criteria add physiological criteria to symptom-based criteria of chronic constipation (CC) for the diagnosis of defecatory disorders (DD). However, a gold-standard test is still lacking and physiological examination is expensive and time-consuming. Aim. Evaluate the usefulness of two low-cost tests-digital rectal examination (DRE) and balloon expulsion test (BET)-as screening or excluding tests of DD. Methods. We performed a systematic search in PUBMED and MEDLINE. We selected studies where constipated patients were evaluated by DRE or BET. Heterogeneity was assessed and random effect models were used to calculate the sensitivity, specificity, and negative predictive value (NPV) of the DRE and the BET. Results. Thirteen studies evaluating BET and four studies evaluating DRE (2329 patients) were selected. High heterogeneity (I2 > 80%) among studies was demonstrated. The studies evaluating the BET showed a sensitivity and specificity of 67% and 80%, respectively. Regarding the DRE, a sensitivity of 80% and specificity of 84% were calculated. NPV of 72% for the BET and NPV of 64% for the DRE were estimated. The sensitivity and specificity were similar when we restrict the analysis to studies using Rome criteria to define CC. The BET seems to perform better when a cut-off time of 2 minutes is used and when it is compared with a combination of physiological tests. Considering the DRE, strict criteria seem to improve the sensitivity but not the specificity of the test. Conclusion. Neither of the low-cost tests seems suitable for screening or excluding DD.

Emergencies after endoscopic procedures.

Endoscopy adverse events (AEs), or complications, are a rising concern on the quality of endoscopic care, given the technical advances and the crescent complexity of therapeutic procedures, over the entire gastrointestinal and bilio-pancreatic tract. In a small percentage, not established, there can be real emergency conditions, as perforation, severe bleeding, embolization or infection. Distinct variables interfere in its occurrence, although, the awareness of the operator for their potential, early recognition, and local organized facilities for immediate handling, makes all the difference in the subsequent outcome. This review outlines general AEs' frequencies, important predisposing factors and putative prophylactic measures for specific procedures (from conventional endoscopy to endoscopic cholangio-pancreatography and ultrasonography), with comprehensive approaches to the management of emergent bleeding and perforation.

Prenatal Diagnosis of EEC Syndrome with "Lobster Claw" Anomaly by 3D Ultrasound.

THE EEC SYNDROME IS A GENETIC ANOMALY CHARACTERIZED BY THE TRIAD: ectodermal dysplasia (development of anomalies of the structures derived from the embryonic ectodermal layer), ectrodactyly (extremities, hands and feet malformations) and cleft lip and/or palate; these malformations can be seen together or in isolation. The prenatal diagnosis can be made by two-dimensional ultrasonography (2DUS) that identifies the facial and/or limb anomalies, most characteristic being the "lobster-claw" hands. The three-dimensional ultrasonography (3DUS) provides a better analysis of the malformations than the 2DUS. A 25-year-old primigravida, had her first transvaginal ultrasonography that showed an unique fetus with crow-rump length of 47 mm with poorly defined hands and feet,. She was suspected of having sporadic form of EEC syndrome. The 2DUS performed at 19 weeks confirmed the EEC syndrome, showing a fetus with lobster-claw hands (absence of the 2(nd) and 3(rd) fingers), left foot with the absence of the 3rd toe and the right foot with syndactyly, and presence of cleft lip/palate. The 3DUS defined the anomalies much better than 2DUS including the lobster-claw hands.