Congenital laryngo-tracheo-esophageal clefts: updates from a quaternary care pediatric airway unit.

PURPOSE: To review the operative techniques, outcomes, and complications following surgery in pediatric patients with laryngo-tracheo-esophageal clefts (LTEC). We describe a new combined approach to treat long LTECs. METHODS: Twenty-five patients underwent surgical repair for LTEC from March 2012 to July 2022 at our hospital. Every patient underwent a diagnostic endoscopy under general anesthesia and spontaneous ventilation to assess the LTEC and synchronous aero-digestive comorbidities/malformations. All patients underwent at least one surveillance endoscopy after the repair at our institution. RESULTS: The patients had multiple other malformations, specifically gastro-intestinal, synchronous airway, and cardiac. The cleft distribution according to the modified Benjamin and Inglis classification was type I (n = 5, 20%), type II (n = 6, 24%), type IIIa (n = 8, 32%), type IIIb (n = 4, 16%), and type IVa (n = 2, 8%). The median follow-up was 44.6 months. Five patients (20%) had undergone previous cleft corrective surgery(s). Seven patients (28%) had partial to complete breakdown of the repair, needing additional intervention(s), and two required a combined-open plus endoscopic repair. Preoperatively, most patients (n = 18, 72%) needed a feeding assistance. At latest follow-up, feeding assistance was weaned off in 13 out of 18 patients, which was a 72% improvement. Ten patients (40%) needed ventilation assistance before the surgery. Post-operatively, ventilatory assistance was weaned off in 6 patients, meaning a 60% improvement. CONCLUSION: LTEC are rare malformations, and their management needs precise diagnosis, appropriate surgical planning, and execution, and dedicated post-operative care. Primary and revision repair of long clefts with tracheal extension may require a combined approach.

Hereditary alpha tryptasemia is not associated with specific clinical phenotypes.

BACKGROUND: Hereditary alpha tryptasemia (HαT) is found in approximately 7% of the population. Associations with a variety of clinical symptoms including gastric reflux, joint hypermobility, dysautonomia, flushing and pruritus, and hymenoptera allergy have variably been described in prior reports. However, our understanding of this genetic trait is limited by a paucity of published studies, referral bias, and conflicting findings at clinical presentation. OBJECTIVE: The purpose of this study was to assess the clinical phenotype of HαT in a random biorepository population and in patients with and without mastocytosis referred to the allergy clinic. METHODS: Tryptase copy number allele was assessed using digital droplet PCR. Participants with or without HαT were interviewed and examined by a clinician and surveyed regarding their medical history and symptomology. RESULTS: HαT was identified in 7.5% of the random biorepository samples and in 18% of patients with mastocytosis. There was no difference in the clinical symptomology or medical history of individuals with HαT compared to controls. Average baseline serum tryptase was higher in individuals with HαT compared to controls, but there was no difference in urinary mast cell activation products. CONCLUSIONS: Elevated baseline serum tryptase was the only consistent phenotypic marker for HαT in this study. There was a higher frequency of HαT in patients with mastocytosis than in the general population.

Corrigendum: Analysis of a Large Standardized Food Challenge Data Set to Determine Predictors of Positive Outcome Across Multiple Allergens.

[This corrects the article DOI: 10.3389/fimmu.2018.02689.].

Analysis of a Large Standardized Food Challenge Data Set to Determine Predictors of Positive Outcome Across Multiple Allergens.

Background: Double-blind placebo-controlled food challenges (DBPCFCs) remain the gold standard for the diagnosis of food allergy; however, challenges require significant time and resources and place the patient at an increased risk for severe allergic adverse events. There have been continued efforts to identify alternative diagnostic methods to replace or minimize the need for oral food challenges (OFCs) in the diagnosis of food allergy. Methods: Data was extracted for all IRB-approved, Stanford-initiated clinical protocols involving standardized screening OFCs to a cumulative dose of 500 mg protein to any of 11 food allergens in participants with elevated skin prick test (SPT) and/or specific IgE (sIgE) values to the challenged food across 7 sites. Baseline population characteristics, biomarkers, and challenge outcomes were analyzed to develop diagnostic criteria predictive of positive OFCs across multiple allergens in our multi-allergic cohorts. Results: A total of 1247 OFCs completed by 427 participants were analyzed in this cohort. Eighty-five percent of all OFCs had positive challenges. A history of atopic dermatitis and multiple food allergies were significantly associated with a higher risk of positive OFCs. The majority of food-specific SPT, sIgE, and sIgE/total IgE (tIgE) thresholds calculated from cumulative tolerated dose (CTD)-dependent receiver operator curves (ROC) had high discrimination of OFC outcome (area under the curves > 0.75). Participants with values above the thresholds were more likely to have positive challenges. Conclusions: This is the first study, to our knowledge, to not only adjust for tolerated allergen dose in predicting OFC outcome, but to also use this method to establish biomarker thresholds. The presented findings suggest that readily obtainable biomarker values and patient demographics may be of use in the prediction of OFC outcome and food allergy. In the subset of patients with SPT or sIgE values above the thresholds, values appear highly predictive of a positive OFC and true food allergy. While these values are relatively high, they may serve as an appropriate substitute for food challenges in clinical and research settings.

[Ambulatory surgery in urology]. / Chirurgie ambulatoire en urologie : il est temps d'en parler.

The volume of ambulatory surgery in urology increases every year. Successful ambulatory care is defined by low hospital (re)admission rates, insuring optimal oncologic and functional outcomes. New medical techniques such as minimally invasive surgical approaches and less side effects of anesthesia have helped the development of ambulatory surgery. Additionally, it is essential to lower the burden of healthcare systems financially, without compromising quality of care. Ambulatory surgery needs a multidisciplinary team involvement, where the general practitioner has a key role.

La pratique ambulatoire en urologie augmente chaque année. Les avancées médicochirurgicales ont permis de diminuer les gestes invasifs et les effets secondaires des anesthésiques. Sur le plan financier, il est essentiel de contrôler les dépenses de santé publique, tout en maintenant comme premier objectif la sécurité du patient. Les critères de succès d'une prise en charge ambulatoire se mesurent au faible taux d'hospitalisation et de réadmission, sans compromission quant aux résultats de la chirurgie. La prise en charge ambulatoire exige une équipe pluridisciplinaire et le médecin traitant joue un rôle essentiel dans l'itinéraire clinique de son patient.

Landmarking the brain for geometric morphometric analysis: an error study.

Neuroanatomic phenotypes are often assessed using volumetric analysis. Although powerful and versatile, this approach is limited in that it is unable to quantify changes in shape, to describe how regions are interrelated, or to determine whether changes in size are global or local. Statistical shape analysis using coordinate data from biologically relevant landmarks is the preferred method for testing these aspects of phenotype. To date, approximately fifty landmarks have been used to study brain shape. Of the studies that have used landmark-based statistical shape analysis of the brain, most have not published protocols for landmark identification or the results of reliability studies on these landmarks. The primary aims of this study were two-fold: (1) to collaboratively develop detailed data collection protocols for a set of brain landmarks, and (2) to complete an intra- and inter-observer validation study of the set of landmarks. Detailed protocols were developed for 29 cortical and subcortical landmarks using a sample of 10 boys aged 12 years old. Average intra-observer error for the final set of landmarks was 1.9 mm with a range of 0.72 mm-5.6 mm. Average inter-observer error was 1.1 mm with a range of 0.40 mm-3.4 mm. This study successfully establishes landmark protocols with a minimal level of error that can be used by other researchers in the assessment of neuroanatomic phenotypes.

Morphometric analysis of brain shape in children with nonsyndromic cleft lip and/or palate.

The purpose of this study was to test for differences in brain shape among children with cleft palate only (n = 22), children with cleft lip and palate (n = 35), and controls (n = 39) using Euclidean distance matrix analysis. Sixteen percent of interlandmark distances differed between children with cleft palate only and controls, 10% differed between children with cleft lip and palate and controls, and 10% differed between children with cleft palate only and children with cleft lip and palate. Major differences in brain shape associated with cleft lip and/or palate included posterior expansion of the occipital lobe, reorientation of the cerebellum, heightened callosal midbody, and posterior displacement of the caudate nucleus and thalamus. Differences in brain shape unique to cleft palate only and to cleft lip and palate were also identified. These results expand upon previous volumetric studies on brain morphology in individuals with cleft lip and/or palate and provide additional evidence that the primary defect in cleft lip and/or palate results in both facial and brain dysmorphology.

Ecological stress and linear enamel hypoplasia in Cebus.

Although it is assumed that monkeys in some environments experience more nutritional or physiological stress than others, little research has been conducted on this topic. This study examines the relationship between linear enamel hypoplasia (LEH) frequency, a physiological indicator of stress, and environmental stressors. To test this relationship, LEH frequencies were calculated for 144 Cebus from 54 locations in Brazil. Habitat, temperature range, and annual rainfall were compared between individuals with and without LEH. The LEH frequency for Cebus from semideciduous forests was significantly higher than that for monkeys from coastal areas, the rainforest, and the savanna (chi(2) = 9.97, df = 1; P = 0.0016). A significantly higher LEH frequency was also found for monkeys living in environments with the mean annual temperature between 15 and 18 degrees C than for those in environments greater than 18 degrees C (chi(2) = 7.74, df = 1, P = 0.0054). However, no significant difference was found between LEH frequency and annual rainfall (t = 1.22, P = 0.23) or the average difference in rainfall between the driest and wettest months (t = 0.77, P = 0.44). These results indicate that levels of physiological stress can differ among environments and that habitat and temperature, but not precipitation, may be driving the difference in stress levels among environments.

Student laboratory presentations as a learning tool in anatomy education.

Previous studies have shown that anatomy students who complete oral laboratory presentations believe they understand the material better and retain it longer than they otherwise would if they only took examinations on the material; however, we have found no studies that empirically test such outcomes. The purpose of this study was to assess the effectiveness of oral presentations through comparisons with other methods of assessment, most notably, examination performance. Specifically, we tested whether students (n = 256) performed better on examination questions on topics covered by their oral presentations than on other topics. Each student completed two graded, 12-minute laboratory presentations on two different assigned topics during the course and took three examinations, each of which covered a third of the course material. Examination questions were characterized by type (memorization, pathway, analytical, spatial). A two-way repeated measures analysis of variance revealed that students performed better on topics covered by their presentations than on topics not covered by their presentations (P < 0.005), regardless of presentation grade (P > 0.05) and question type (P > 0.05). These results demonstrate empirically that oral presentations are an effective learning tool.

[Lung transplantation results: does a learning curve exist?]. / Résultats après transplantation pulmonaire: existe-t-il une courbe d'apprentissage?

Lung transplantation is an established procedure for end-stage non malignant respiratory disorders. Lung transplantation involves multidisciplinary skills and expertise and one can question the ability of small units to achieve successfully such a demanding therapy. We report here the results from the Lausanne unit of the Geneva-Lausanne lung transplantation center. We have compared the 1993 to 1996 and the 1997-2000 period. For the 29 lung transplantions performed the actuarial survival was 75% at 3 years and 72% at 5 years. There was no significant difference in survival or in functional status between the two periods. We conclude that despite the small number of patients treated, these results compare favourably with published international data. In particular, no learning curve effect was observed.