Search

Introduction: We present the development and validation of an ELISA test based on human recombinant tissue transglutaminase (h-tTG) for the detection of IgG anti-transglutaminase (IgG anti-h-tTG).Objective: To demonstrate that Eu-tTG® IgG umana kit is of help in diagnosing coeliac patients with IgA deficiency.Subjects: 154 sera were from healthy anti-endomysium (AEA, Antiendomysium Eurospital) and IgA anti-transglutaminase (IgA anti-h-tTG, Eu-tTG IgA umana®, Eurospital) negative subjects, 105 were from celiac patients all AEA and IgA anti-h-tTG positive and 11 sera were from IgA deficient celiac patients negative for both AEA and IgA anti-h-tTG.Results: 3 out of 154 healthy subjects were above the cut-off values for IgG anti-h-tTG (specificity of 98%). 50 out of 105 celiac patients were negative for IgG anti-h-tTG (sensitivity of 52%). All 11 IgA deficient celiac patients were positive for IgG anti-h-tTG (sensitivity of 100%).Conclusions: These results show that Eu-tTG®IgG umana kit has good specificity for screening purposes. The sensitivity is very low with celiac patients that are able to produce anti-transglutaminase IgA but is very high (>99%) with those patients that are unable to produce IgA antibodies. The combined use of Eu-tTG®IgG umana and the Eu-tTG®IgA umana seems to provide the most powerful diagnostic tool for the screening of celiac disease to date. [Copyright &y& Elsevier]

Objective: To describe the findings of children with Robin Sequence (RS) who received sensory-motor-oral stimulation combined with early sucking during mandibular distraction osteogenesis (MDO), compared with children who did not receive the intervention., Design: A quasi-experimental study. Setting: A tertiary public hospital. Patients: Children with RS referred to MDO. A historical group from the same population but managed according to the institution's standard protocol (no sucking) served as a control group. Interventions: Sensory-motor-oral stimulation, including sucking, starting 24 h after MDO (intervention group). Main Outcome Measure: Our hypothesis is that sensory-motor-oral stimulation, including sucking during the DOM process, do not negatively affect surgical outcomes., Results: Twenty-nine children were included. Eight (72.7%) of the 11 patients in the intervention group and 13 (72.2%) of the 18 controls had MDO complications, with no significant difference between the groups ( p  = 1.000). The most common surgical outcome was antibiotic therapy for surgical site infection (76.2%). Six months after MDO, 22 (75.9%) children attained full oral feeding or associated with alternative feeding methods., Conclusion: The intervention group did not have higher complication rates, from a surgical point of view, than control group. The protocol adopted by some centers that contraindicates sucking during MDO should be revised to consider the benefits of such stimulation. Keywords: Pierre Robin Syndrome, deglutition, therapeutics, child development., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To evaluate the combined impact of videofluoroscopic swallow study (VFSS) and therapeutic feeding and swallowing interventions on clinical outcomes in children with oropharyngeal dysphagia (OPD)., Methods: This was an uncontrolled longitudinal analytical study in which OPD patients were evaluated before and after VFSS. Children ≤ 24 months of age diagnosed with OPD in a clinical setting and undergoing VFSS for investigation and management of OPD were included in the study. The study participants received therapeutic feeding and swallowing interventions after having undergone VFSS, being followed at an outpatient clinic for pediatric dysphagia in order to monitor feeding and swallowing difficulties. Respiratory and feeding outcomes were compared before and after VFSS., Results: Penetration/aspiration events were observed in 61% of the VFSSs (n = 72), and therapeutic feeding and swallowing interventions were recommended for 97% of the study participants. After the VFSS, there was a reduction in the odds of receiving antibiotic therapy (OR = 0.007) and in the duration of antibiotic therapy (p = 0.014), as well as in the odds of being admitted to hospital (p = 0.024) and in the length of hospital stay (p = 0.025). A combination of oral and enteral feeding became more common than oral or enteral feeding alone (p = 0.002)., Conclusions: A high proportion of participants exhibited penetration/aspiration on VFSS. Therapeutic feeding and swallowing interventions following a VFSS appear to be associated with reduced respiratory morbidity in this population.

Myocarditis has in rare cases been associated with COVID-19 infection and has emerged as a possible rare side effect of vaccination with anti-COVID-19 messenger RNA vaccines. However, little is known about possible COVID-19 infection- and/or vaccination-related myocarditis relapse in patients with previous clinically suspected or biopsy-proven myocarditis. Myocarditis may relapse, particularly in females with immune-mediated/autoimmune features and a predisposing immunogenetic background. We aimed to assess the prevalence of myocarditis relapse during the COVID-19 outbreak and following COVID-19 vaccination in a cohort of patients with prior myocarditis. We included in the analysis myocarditis patients on active follow-up, for whom COVID-19 infection and vaccination statuses were known, and collected data on clinical, laboratory and echocardiographic findings, and myocarditis relapse. We enrolled 409 patients, of whom 114 (28%) reported COVID-19 infection and 347 (85%) completed the vaccination scheme. Only one patient, having COVID-19 infection before the vaccination campaign started, was admitted to hospital because of pneumonia; the remaining patients had an uneventful COVID-19 infection course, with only mild symptoms. No myocarditis relapse was recorded following COVID-19 infection or vaccination. Moreover, the frequency of new myocarditis cases following the COVID-19 outbreak was not different compared to the three-year period preceding the COVID-19 era. In conclusion, in our cohort of patients with prior myocarditis, both COVID-19 infection and vaccination were uneventful.

Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies published to date have evaluated only respiratory outcomes, and the available evidence on the improvement of swallowing is questionable. To conduct a systematic review and meta-analysis of studies evaluating swallowing in children with Robin Sequence before and after airway clearance procedures. The research question was developed based on the PICO strategy. The literature search was performed in electronic databases and gray literature. Studies were selected by 3 independent reviewers. The risk of bias and level of evidence of the studies were assessed. A proportion meta-analysis was performed to calculate the prevalence of dysphagia after airway clearance procedures. The search identified 4938 studies, 5 of which were included. All studies had limitations in terms of design and sample size. The prevalence of dysphagia after airway clearance was obtained by analyzing treatment subgroups: mandibular distraction osteogenesis, mandibular distraction osteogenesis + tracheostomy tube, and nasopharyngeal tube. Clinical and/or instrumental assessment was assessed by a swallowing specialist. The meta-analysis was precluded by the limitations of the studies, especially regarding sample size, which affected the accuracy of the findings. Dysphagia remained unresolved in 55% of children (95% CI 1-99%). The methodological quality of the studies indicated a high risk of bias and very low level of evidence. It was not possible to confirm that airway clearance techniques used in Robin Sequence improve dysphagia., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To investigate the accuracy of clinical evaluation of swallowing in a sample of children with laryngomalacia or glossoptosis and describe the prevalence of dysphagia in each of these diseases, as well as characterize the swallow response to speech and language therapy interventions., Study Design: Children aged 1 month to 11 years receiving care at the Department of Otolaryngology, Hospital de Clínicas de Porto Alegre, Brazil, were evaluated in a cross-sectional design. Evaluation of swallowing was performed at two time points by two blinded speech-language pathologists, one responsible for clinical evaluation and the other for videofluoroscopic study. The protocols employed were based on the instruments proposed by DeMatteo et al. (DeMatteo C, Matovich D, Hjartarson A. Comparison of clinical and videofluoroscopic evaluation of children with feeding and swallowing difficulties. Dev Med Child Neurol 2005;47:149-157.)., Results: The study sample consisted of 29 patients: 10 patients with laryngomalacia and 19 patients with glossoptosis. The sensitivity of clinical evaluation did not exceed 50% in any of the evaluations, but specificity reached 100% in some cases, using thickened liquids. The prevalence of dysphagia was 100%, and the use of thickened liquids significantly reduced tracheal aspiration., Conclusions: Dysphagia was highly prevalent in this sample. The sensitivity of clinical evaluation to detect laryngeal penetration and tracheal aspiration was low, as the majority of aspiration events were silent. The videofluoroscopic study is important in order to determine a safest method to feed the patient. Pediatr Pulmonol. 2017;52:41-47. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Objective: To evaluate the performance of two glossoptosis airway obstruction classifications in predicting symptom severity and laryngeal exposure difficulty in Robin Sequence (RS) patients., Setting: Public tertiary hospital otolaryngology section (Hospital de Clínicas de Porto Alegre - HCPA)., Patients: All RS patients diagnosed at HCPA from October 2012 to February 2015 were enrolled, a total of 58 individuals. They were classified in isolated RS, RS-Plus and syndromic RS., Intervention: Patients were submitted to sleep endoscopy and a score was attributed according to Yellon and de Sousa by a blinded researcher. Symptom severity evaluation was performed as defined by Cole classification., Main Outcome Measure: Association between endoscopic findings and clinical symptoms severity and laryngeal exposure difficulty., Results: Twenty four patients were identified as isolated RS (41.4%), 19 patients presented as RS-Plus (32.7%) and 15 patients had well defined diagnosed syndromes (25.9%). Concomitant airway anomalies were found in 18 patients (31%). Specifically 17.4% in isolated RS, 55.6% in RS- Plus and 28.6% in the syndromic group had such anomalies (P = 0,03). Probability of presenting severe clinical symptoms as graded by Cole was higher in grade 3 Yellon classification (68.4%, P = 0.012) and in moderate and severe de Sousa classification (61.5% and 62.5%, respectively, P = 0.015) than in milder grades of obstruction. This findings were considered significant even after controlling for patient age. Laryngeal exposure difficulty was correlated with de Sousa and Yellon (Rho = 0,41 and Rho = 0,43, respectively; P < 0,05)., Conclusion: Patients with higher degrees of obstruction in sleep endoscopy had a higher probability of presenting a more severe clinical manifestation and a more difficult laryngeal exposure. Since the number of patients included in this study was small for subgroup analyses, it is not clear if this association is restricted to a specific group of RS., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Introduction Surgical repair of congenital heart disease in the first years of life compromises the coordination of the suction, breathing, and swallowing functions. Objective To describe the alterations in swallowing found in infants with congenital heart defect during their hospitalization. Methods Prospective, cross-sectional study in a reference hospital for heart disease. The sample consisted of 19 postsurgical patients who underwent an evaluation of swallowing. The infants included were younger than 7 months and had a diagnosis of congenital heart defect and suspected swallowing difficulties. Results Of the 19 infants with congenital heart defect, the median age was 3.2 months. A significant association was found between suction rhythm and dysphagia (p = 0.036) and between oral-motor oral feeding readiness and dysphagia (p = 0.014). Conclusions The data suggest that dysphagia often occurs after surgery in infants with congenital heart defect. Infants with congenital heart defect had very similar behavior to preterm infants in terms of oral feeding readiness.

Introduction: Although the prevalence of celiac disease (CD) has been extensively investigated in recent years, an accurate estimate of CD frequency in the European population is still lacking. The aims of this study were: 1) to establish accurately the prevalence of CD in a large sample of the European population (Finland, Germany, Italy, and UK), including both children and adults; and 2) to investigate whether the prevalence of CD significantly varies between different areas of the European continent., Materials and Methods: Samples were drawn from the four populations. All 29,212 participants were tested for CD by tissue transglutaminase (tTG) antibody test. Positive and border-line findings were further tested for serum endomysial antibodies (EMA). All serological determinations were centrally performed. Small-bowel biopsies were recommended to autoantibody-positive individuals. Previously diagnosed cases were identified., Results: The overall CD prevalence (previously diagnosed plus anti-tTG and EMA positives) was 1.0% (95% CI 0.9-1.1). In subjects aged 30-64 years CD prevalence was 2.4% in Finland (2.0-2.8), 0.3% in Germany (0.1-0.4), and 0.7% in Italy (0.4-1.0). Sixty-eight percent of antibody-positive individuals showed small-bowel mucosal changes typical for CD (Marsh II/III lesion)., Conclusions: CD is common in Europe. CD prevalence shows large unexplained differences in adult age across different European countries.

Unlabelled: It is paramount to assess the psychometric properties of self-assessment tools in order to check the tests' reliability and validity, also to enable proper outcome interpretation., Aim: to check the psychometric properties of the IOI-HA (International Outcome Inventory for Hearing Aids) in its Portuguese version, called QI-AASI (International Questionnaire - Individual Sound Amplification Device), in terms of internal uniformity, correlation between the items and reproducibility., Study Design: descriptive, observational and cross-sectional., Materials and Methods: the questionnaire was deployed to 53 hearing aid users, 34 females and 19 males, with ages between 19 and 92 years - from incomplete basic education to complete higher education, encompassing subjects with monoaural and binaural sound amplification., Results: the QI-AASI had a Cronbach Alpha of 0.69. In the correlation among the items, there were numerous significant correlations. The instrument was properly reproducible, except for item # 6, which presented a significant difference in comparing test and the retest., Conclusions: the QI-AASI is suggested in the rehabilitation process of users of hearing aides; nonetheless, the questionnaire can be difficult for subjects with low social and economic status when self-employed.

Background: We measured anti-transglutaminase (anti-tTG) antibody in the culture medium of intestinal biopsy specimens from patients with suspected celiac disease (CD) and evaluated the relationship between antibody production and severity of intestinal mucosal damage., Methods: We performed diagnostic testing for CD on 273 consecutive patients. In addition to routine histologic evaluation of duodenal biopsy specimens, we assayed anti-tTG antibodies in serum and in the culture medium of duodenal biopsy specimens., Results: CD was diagnosed in 191 of the 273 patients. Sensitivity and specificity of the serum anti-endomysium (EmA) and anti-tTG assays were 83% and 85% and 99% and 95%, respectively, and both had 88% diagnostic accuracy. EmA and anti-tTG assayed in the culture medium had 98% sensitivity, 100% specificity, and 98% diagnostic accuracy (vs serum assays; P <0.0001). Twenty-nine CD patient specimens (16%) were negative for serum anti-tTG and EmA; for 24 of these patients, anti-tTG assay of the culture medium was positive. The CD patients whose biopsy specimens were positive for serum antibodies showed the following intestinal histologies: total villous atrophy, 35%; severe villous atrophy, 25%; mild atrophy, 25%; villi with no atrophy but with increased intraepithelial lymphocytes, 15%. None of the CD patients whose specimens were negative for serum antibodies showed total or severe villous atrophy; 77% had mild villous atrophy, and 23% had no villous atrophy but had increased intraepithelial lymphocyte counts. Mild villous atrophy was also seen in specimens from approximately 15% of patients without CD., Conclusion: Anti-tTG assay of the culture medium of biopsy specimens can improve the accuracy of CD diagnosis in patients negative for serum antibodies.

Objective: Diagnosis of coeliac disease is based on the presence of villous atrophy which recovers following a gluten-free diet. The presence of circulating antiendomysial antibodies as well as their disappearance after a gluten-free diet supports the diagnosis. It has also been demonstrated that antiendomysial antibodies are detectable in supernatants of cultured intestinal biopsies from patients with coeliac disease. The objective of this study was to compare the histology and antiendomysial antibodies in culture supernatants of intestinal biopsies to validate the in vitro organ culture system as a future diagnostic tool for coeliac disease., Material and Methods: Seventy-five antiendomysial serum-positive patients on a gluten-containing diet were evaluated. Patients underwent endoscopy with 5 biopsy fragments: 3 for histology, 1 cultured with and the other without gliadin-peptide activator. Antiendomysial antibodies were evaluated in all culture supernatants., Results: Sixty-eight patients had evidence of villous atrophy, while 73 out of 75 were positive to the organ culture system. The agreement rate between organ culture and histology results was 94%., Conclusions: As all the centres participating in the study obtained good agreement between organ culture and histology results, the new system could be considered a reliable tool for the diagnosis of coeliac disease. Nevertheless, it is possible to highlight cases with an organ culture-positive and -negative histology. This feature could be of considerable interest because, as the sensitivity of organ culture seems to be greater than the initial histology, the new system might be useful in uncertain cases where the risk of missing the diagnosis of coeliac disease is high.

Objective: For celiac disease (CD), screening a trend has recently emerged to measure tissue transglutaminase antibodies (tTGA) by immunoassays instead of the more laborious endomysial antibodies (EmA), as they recognize the same target, tissue transglutaminase (tTG). However, a high rate of false-positive results has been reported in some patient series with diseases known to be associated with CD. Moreover, tTG is a ubiquitous, multifunctional enzyme, overexpressed in experimental models of heart failure. Therefore, we assessed the specificity of tTGA assays in a large series of EmA-negative patients with end-stage heart failure., Methods: We studied 288 patients with end-stage heart failure and 60 blood donors. No subject had clinical evidence of CD or IgA deficiency, and all were EmA negative. Serum IgA and IgG tTGA were measured by means of commercial kits using as substrate, either guinea pig or recombinant human tTG. Blocking studies and Western blots were also performed using recombinant human tTG., Results: All blood donor sera were IgA tTGA negative. IgA tTGA positivity was observed in 47.6% and 49.1% of patients with heart failure using, respectively, guinea pig tTG and recombinant human tTG as substrates. Preincubation of positive sera with recombinant human tTG resulted in 81% blocking of IgA tTGA in immunoassay. Western blot analysis confirmed the presence of antibodies against recombinant human tTG. IgA tTGA-positive sera were also IgG tTGA positive., Conclusions: IgA and IgG tTGA occur in a large number of EmA-negative patients with end-stage heart failure, and their presence is unlikely to be caused by concomitant CD.