Recurrent Plunging Ranula Due to a Sublingual Ectopic Gland: A Rare Clinical Entity.

Ranula is a benign cystic lesion caused by the escape and collection of salivary mucus. Classically, it is divided into simple ranulas, a cystic mass in the floor of the mouth, and diving/plunging/cervical ranulas, a submandibular mass without apparent intraoral involvement. Although plunging ranula is a well-documented cause of neck swelling, its association with the presence of ectopic sublingual glands is extremely rare, with less than five cases reported. Other cervical cystic lesions may have the same clinical aspect; therefore, advanced diagnostic techniques like a CT scan or MRI play a critical role in early diagnosis. Different approaches have been used to treat ranulas, including non-invasive, minimally invasive, and surgical techniques. The purpose of this paper is to highlight a case report of a giant plunging ranula due to an anatomical aberration of the right sublingual gland, along with a significant literature review.

Volanesorsén, esperanza terapéutica para el síndrome de quilomicronemia familiar / Volanesorsén, therapeutic hope for family chylimicronemia syndrome

Objective: To reduce severe hypertriglyceridaemia and episodes of pancreatitis in patients with familial chylomicronemia syndrome (FCHS), in whom the response to diet and triglyceride (TG) lowering treatment has not been sufficient. Method: A 46-year-old woman diagnosed with genetically confirmed FFCS, with heterozygous presence of two variants and very severe elevation of triglycerides (≥2000 mg/dL), multiple admissions for acute pancreatitis since the age of 19, with associated side effects such as pancreatoprive Diabetes Mellitus with need for insulin and severe hepatic steatosis with grade I fibrosis diagnosed by liver biopsy. Given the intolerance to fibrates and insufficient response to diet and high doses of ω-3 fatty acids, we started treatment with Volanesorsen. Result: After 6 admissions for acute pancreatitis from January to April 2020, treatment with Volanesorsen was started on 7 August. Platelets at the start of treatment were 283x103/mm3 and triglycerides 1878 mg/dL. Platelet monitoring was performed every 2 weeks and at all times the figure remained >140x103/mm3. The treatment was well tolerated and after three months, the targets for continuing Volanesorsen were reached, reducing TG by more than 25% and reaching 624 mg/dL with platelets in the normal range. Conclusion: Volanesorsen is indicated as an adjunct to diet in adult patients with genetically confirmed FQS at high risk of pancreatitis, in whom the response to diet and triglyceride-lowering treatment has not been sufficient. (AU)

Objetivo: Reducción de la hipertrigliceridemia severa y episodios de pancreatitis en pacientes con síndrome de quilomicronemia familiar (SQF), en quienes la respuesta a la dieta y al tratamiento de reducción de triglicéridos (TG) no ha sido suficiente. Material y método: Mujer de 46 años diagnosticada de SQF confirmado genéticamente, con presencia en heterocigosis de dos variantes y con elevación muy grave de triglicéridos (≥2000 mg/dL), múltiples ingresos por pancreatitis agudas desde los 19 años, con efectos colaterales asociados como Diabetes Mellitus pancreatopriva con necesidad de insulina y esteatosis hepática severa con fibrosis grado I diagnosticada por biopsia hepática. Ante la intolerancia a fibratos e insuficiente respuesta a la dieta y altas dosis de ácidos grasos ω-3, iniciamos tratamiento con Volanesorsén. Resultado: Tras 6 ingresos por pancreatitis aguda desde enero hasta abril de 2020, el 7 de agosto inicia tratamiento con Volanesorsén. Plaquetas al inicio del tratamiento de 283x103/mm3 y triglicéridos 1878 mg/dL. Se realizó una monitorización plaquetaria cada 2 semanas y en todo momento la cifra se mantuvo >140x103/mm3 . El tratamiento fue bien tolerado y tras tres meses, se alcanzan los objetivos para poder continuar con Volanesorsén, reduciendo los TG más del 25% y alcanzando 624 mg/dL con plaquetas en rango de la normalidad. Conclusión: Volanesorsén está indicado como complemento a la dieta en pacientes adultos con SQF confirmado genéticamente y con riesgo alto de pancreatitis, en quienes la respuesta a la dieta y al tratamiento de reducción de triglicéridos no ha sido suficiente. (AU)

Hurler Syndrome: Orofacial Clinical Findings.

Hurler syndrome (HS) belongs to the category of mucopolysaccharidosis (MPS), a spectrum of rare genetic disorders of the mucopolysaccharides metabolism. This syndrome is due to a defect in α-iduronidase, an enzyme responsible for the degradation of the glycosaminoglycans (GAGs) heparin and dermatan sulfate. Intra and extracellular accumulation of these non-metabolized substances may lead to multisystemic dysfunction, with severe stomatognathic involvement that may often need treatment. The aim of this article is to present the heterogeneity of orofacial and radiographic findings observed in two patients with HS with long-term follow-up, who were referred to our Stomatology department.

Major anthocyanins in elderberry effectively trap methylglyoxal and reduce cytotoxicity of methylglyoxal in HepG2 cell line.

The accumulation of advanced glycation end-products (AGEs) in the body is implicated in numerous diseases, being methylglyoxal (MGO) one of the main precursors. One of the strategies to reduce AGEs accumulation might be acting in an early stage of glycation by trapping MGO. Thus, this work aimed to evaluate, for the first time, the potential of elderberries polyphenols to trap MGO, access the formation of MGO adducts, and evaluate the cytoprotection effect in HepG2 and Caco-2 cells. The results demonstrated that monoglycosylated anthocyanins (cyanidin-3-glucoside and cyanidin-3-sambubioside) are very efficient in trapping MGO, forming mono- and di-adducts. Quercetin-3-glucoside and quercetin-3-rutinoside reacted slowly, while diglycosylated anthocyanins did not react. The trapping of MGO by elderberry monoglycosylated anthocyanins significantly decreased the MGO cytotoxicity in HepG2 cells (∼70 % of cell viability), while the effect in Caco-2 cells was lower (∼50 %). Thus, elderberry phenolics present antiglycation potential by trapping MGO.

Oral Self-Mutilation in Lesch-Nyhan Syndrome: A Case Report.

Lesch-Nyhan syndrome (LNS) is an inherited recessive X-related disorder caused by a deficiency of the purine salvage enzyme hypoxanthine-guanine phosphoribosyltransferase. It is characterized by dystonia and compulsive self-mutilation, in particular, biting behavior on the oral mucosa, tongue, lips, fingers, and shoulders, typically before one year of age. The majority of these patients require several procedures, including dental extractions, to prevent significant secondary lesions. This article aims to report a clinical case of a 12-year-old boy with an LNS diagnosis who was referred to the Paediatric Stomatology Department of Central Lisbon University Hospital. Since the age of eight, the patient had displayed self-harm behavior, with arm and oral injuries. On evaluation, he presented with deep ulcerated lesions on the lips and tongue, with substance loss associated with a significant decrease in food intake and consequent weight loss. The management included conservative therapy with gabapentin, lorazepam, and botulinum toxin injections. A successful reduction of self-mutilation with no signs of new lesions in the oral cavity and an improvement in nutritional status were reported. The therapeutic approach is essential to provide the best quality of life for patients and their caregivers. To delay radical treatments, multiple therapeutic options can be used. The oral pathology team considered that the most appropriate therapy was botulinum toxin A injections along with therapeutic adjustment, which was effective in wound healing and self-mutilation behavior ceasing at the two-month follow-up.

Ingestão cáustica pediátrica em Minas Gerais ­ Brasil: um estudo observacional do Hospital Universitário de Uberlândia / Pediatric caustic ingestion in Minas Gerais ­ Brazil: an observational study at the University Hospital of Uberlândia

Introdução: Crianças menores de cinco anos são as principais vítimas de acidentes por ingestão de cáusticos, assim considerando a epidemiologia brasileira e dos riscos à exposição desses agentes ressalta-se a importância deste estudo que objetiva realizar uma análise descritiva dos casos de acidentes cáusticos em pacientes pediátricos atendidos em um hospital universitário de Minas Gerais. Método: Este é um estudo transversal descritivo, de caráter quantitativo, a partir dos dados obtidos de prontuários de pacientes pediátricos (0-13 anos) atendidos por ingestão de substâncias cáusticas, no Hospital de Clínicas da Universidade Federal de Uberlândia (Minas Gerais, Brasil). Resultados: Foram levantados 132 prontuários de crianças atendidas por ingestão de substâncias cáusticas de janeiro de 2011 a abril de 2018. Os acidentes acometeram crianças na faixa etária de 08 meses a 12 anos, sendo que 82,60% dos casos ocorreram em ambiente domiciliar. Entre as principais substâncias ingeridas estão os produtos de limpeza, quanto à sua composição química predominaram soda cáustica, hipoclorito de sódio e amoníaco. A endoscopia digestiva alta (EDA) foi realizada em 104 pacientes. Quase 13% das crianças apresentaram estenose esofágica e necessitaram de dilatação esofágica. No período estudado, foram realizados 296 procedimentos de dilatações, com média de 17,4 procedimentos por paciente. Discussão: Considerando que os acidentes cáusticos são prevalentes em crianças menores de cinco anos e em ambiente domiciliar, as principais substâncias ingeridas são aquelas de caráter alcalino, que causam lesão no trato respiratório e gastrointestinal, sendo a principal consequência a estenose esofágica. Ademais, não há protocolos bem definidos para o manejo e a condução de pacientes que fizeram a ingestão dessas substâncias. As principais limitações do estudo foram o preenchimento incompleto dos prontuários analisados e os trâmites burocráticos para o acesso aos mesmos. Conclusão: Predominaram os acidentes cáusticos domiciliares e em crianças menores de 2 anos, o que implica a necessidade de ações educativas e preventivas. [au]

Introduction: Children under five years old are the principal victims of accidents caused by the ingestion of caustics, so considering Brazilian epidemiology and the risks of exposure to these agents the importance of this study is emphasized. This study aims to carry out a descriptive analysis of cases of caustic accidents in pediatric patients treated in a university hospital in Minas Gerais. Method: This is a descriptive cross-sectional study, with a quantitative character, based on data obtained from medical records of pediatric patients (0-13 years old) treated for ingestion of caustic agents at the Hospital of the Federal University of Uberlândia (Minas Gerais, Brazil). Results: We collected 132 medical records of children treated for ingestion of caustic substances from January 2011 to April 2018. The accidents affected children aged between 8 months and 12 years, with 82.60% of the cases occurring in the home environment. Among the main substances ingested are cleaning products, as for their chemical composition, caustic soda, sodium hypochlorite, and ammonia predominated. Upper digestive endoscopy (UGE) was performed in 104 patients. Almost 13% of the children had esophageal strictures and required esophageal dilation. During the study period, 296 dilatation procedures were performed, with an average of 17.4 procedures per patient. Discussion: Considering that caustic accidents are prevalent in children under five years of age and the home environment, the main substances ingested are those of an alkaline nature, which cause injury to the respiratory and gastrointestinal tract, the main consequence being esophageal stenosis. Furthermore, there are no well-defined protocols for the management of patients who have ingested these substances. The main limitations of the study were the incomplete filling of the medical records analyzed and the bureaucratic procedures for accessing them. Conclusion: Caustic accidents predominated in children under two years old in the home environment, which implies the need for educational and preventive actions. [au]

Eosinophilic esophagitis auxiliary diagnosis based on a peptide ligand to eosinophil cationic protein in esophageal mucus of pediatric patients.

Eosinophilic esophagitis (EoE) is a chronic inflammatory condition of the esophagus characterized by increased number of eosinophils. Currently, EoE diagnosis is based on endoscopic procedures for histopathological examination, eosinophils' counting and, often, in clinical practice, the challenge is the differentiation between EoE and gastroesophageal reflux disease (GERD). Our aim was to develop novel peptide ligand to Eosinophil cationic protein (ECP) present in EoE biopsies of patients with potential to be used for detection. We performed a comparative proteomic analysis using liquid chromatography-tandem mass spectrometry (LC-MS/MS) of esophageal biopsies from pediatric patients with eosinophilic esophagitis, gastroesophageal reflux disease and control individuals. Then, phage display technology was used to select peptides against specific up-regulated protein from EoE patients. Twelve phage clones were selected after three biopanning rounds, and the best phage clone reactivity was evaluated by phage-ELISA assay using esophageal mucus samples from 94 pediatric patients. Mass spectrometry showed that eosinophil cationic protein (ECP) was one of the most up-regulated proteins in EoE patients, which is an eosinophil granule protein usually deposited on tissues to mediate remodeling, but in excess may cause fibrosis and hypertrophy, especially in allergic responses. A highly reactive ECP-ligand peptide (E5) was able to distinguish reactive mucus of EoE patients from GERD and the control individuals by Phage-ELISA, achieving a sensitivity of 84.62%, and a specificity of 82.72%. This is the first study that successfully demonstrated an antibody-like peptide targeting ECP at the esophagus mucus as a useful auxilliary tool for EoE diagnosis with a significant association with atopic disorders and dysphagia.ClinicalTrials.gov no.: NCT03069573.

Impacto de un cribado oportunista de hipercolesterolemia familiar (estudio CRIBACOL) en el area sanitaria de Vigo / Impact of an Opportunist Screening for Family Hypercholesterolemia (CRIBACOL study) in the sanitary area of Vigo

Objective: To assess the effectiveness of the diagnosis of Familial Hypercholesterolemia (FH) through opportunistic screening in the health area of Vigo. Material and Methods: An opportunistic screening was carried out retrospectively on all patients in the Vigo Health Area who had been requested to determine their LDLc during 2018. The inclusion criterion was LDL>250 mg/dL, and the exclusion criteria (TSH >4 mIU/L, A1C>6.5%, fasting glucose>126 mg/dL, Triglycerides>150 mg/dL, GGT>55 IU/L and/or alkaline phosphatase>135 IU/L, proteinuria>3g/ L and serum albumin <30g/L). Opportunistic screening was performed using the Modulab Gold (IZASA) program. Results: The total number of LDL determinations was 236,528 out of 185,095 patients. 233 patients met the inclusion criteria. 162 were discarded due to the exclusion criteria. 71 patients with a possible diagnosis of HF were obtained. These patients underwent a clinical interview applying the criteria of the Dutch Lipid Clinics Network (DLCN). The results were: men (21.12%) and women (78.87%); the mean age was 58 years; had hypertension (22.53%), diabetes (1.4%), smoker (23.49%), received statins (63.38%). The results of applying the DLCN criteria were diagnosis possible (53.52%), probable (32.39%) and certain (14.08%). Conclusion: HF is an underdiagnosed and undertreated disease. The application of an opportunistic screening method with an alarm system for health professionals who request lipid profiles can make an early diagnosis of this disease with high cardiovascular risk. (AU)

Looking in Depth at Oxidized Cholesteryl Esters by LC-MS/MS: Reporting Specific Fragmentation Fingerprints and Isomer Discrimination.

Cholesteryl esters (CE) are prone to oxidation under increased oxidative stress conditions, but little is known about oxidized CE species (oxCE). To date, only a few oxCE have been identified, however, mainly based on the detection of molecular ions by mass spectrometry (MS) or target approaches for specific oxCE. The study of oxCE occurring from radical oxidation is still scarcely addressed. In this work, we made a comprehensive assessment of oxCE derivatives and their specific fragmentation patterns to identify detailed structural features and isomer differentiation using high-resolution C18 HPLC-MS- and MS/MS-based lipidomic approaches. The LC-MS/MS analysis allowed us to pinpoint oxCE structural isomers of long-chain and short-chain species, eluting at different retention times (tR). Data analysis revealed that oxCE can be modified either in the fatty acyl moiety or in the cholesterol ring. The location of the hydroxy/hydroperoxy group originates characteristic fragment ions, namely the unmodified cholestenyl cation (m/z 369) for the isomer with oxidation in the fatty acyl chain or ions at m/z 367 and m/z 385 (369 + 16) when oxygenation occurs in the cholesterol ring. Additionally, we identified CE 18:2 and 20:4 aldehydic and carboxylic short-chain products that showed a clear fragmentation pattern that confirmed the modification in the fatty acyl chain. Specific fragmentation fingerprinting allowed discrimination of the isobaric short-chain species, namely carboxylic short-chain products, from hydroxy aldehyde short-chain products, with a hydroxycholesterol moiety. This new information is important to identify different oxCE in biological samples and will contribute to unraveling their role in biological conditions and diseases such as cardiovascular disease.

Characterization of Non-volatile Oxidation Products Formed from Triolein in a Model Study at Frying Temperature.

Frying allows cooking food while promoting their organoleptic properties, imparting crunchiness and flavor. The drawback is the oxidation of triacylglycerides (TAGs), leading to the formation of primary oxidized TAGs. Although they have been associated with chronic and degenerative diseases, they are precursors of pleasant flavors in fried foods. Nevertheless, there is a lack of knowledge about the oxidation species present in foods and their involvement in positive/negative health effects. In this work, high-resolution (HR) C30 reversed-phase (RP)-liquid chromatography (LC)-tandem HR mass spectrometry (MS/MS) was used to identify primary oxidation TAGs resulting from heating triolein (160 °C, 5 min). This allows simulating the initial heating process of frying oils usually used to prepare fried foods, such as chips, crisps, and snacks. Beyond hydroxy, dihydroxy, hydroperoxy, and hydroxy-hydroperoxy derivatives, already reported in phospholipids oxidized by Fenton reaction, new compounds were identified, such as dihydroxy-hydroperoxy-triolein derivatives and positional isomers (9/10- and 9/12-dihydroxy-triolein derivatives). These compounds should be considered when proposing flavor formation pathways and/or mitigating lipid-derived reactive oxygen species occurring during food frying.

Translation and cultural adaptation of the Pediatric Eosinophilic Esophagitis Symptom Score (PEESS v2 0) / Tradução e adaptação cultural da Pediatric Eosinophilic Esophagitis Symptom Score (PEESS v2 0)

Abstract Objectives: To translate and culturally adapt the Pediatric Eosinophilic Esophagitis Symptom Score (version 2.0), a tool used to assess pediatric eosinophilic esophagitis symptoms reported by patients and/or their parents/caregivers. Methods: The Pediatric Eosinophilic Esophagitis Symptom Score was translated through the following stages: initial translation, back-translation, and consensus of independent reviewers through the Delphi technique. The pre-final version of the Pediatric Eosinophilic Esophagitis Symptom Score was applied to five 8-to-18-year-old patients and to ten parents of two-to-18-year-old patients from an outpatient pediatric gastroenterology service (pre-test). Results: During the translation process, no translations presenting with difficult consensus in the review process or grammar inconsistencies were observed. During the pre-test, difficulties in comprehension of some unconventional terms, e.g., "náusea", were observed. Adverbs of frequency, such as "quase nunca" were also identified as being of difficult understanding by patients and parents, and the substitution by the term "raramente" was suggested. Such difficulties may be inherent to the pediatric age group. Age 8 years or above should be considered adequate for the self-reporting of symptoms. Conclusions: The study presents the Brazilian version of the Pediatric Eosinophilic Esophagitis Symptom Score, which is adapted to the Brazilian culture. This version may be introduced as a clinical and research tool for the assessment of patients with esophagic disease symptoms. The Pediatric Eosinophilic Esophagitis Symptom Score is a breakthrough in the evaluation of symptoms of pediatric eosinophilic esophagitis, since it reinforces the importance of self-reporting by patients who experience this disease.

Resumo Objetivo: Traduzir e adaptar culturalmente a Pediatric Eosinophilic Esophagitis Symptom Score (versão 2.0), um instrumento usado para identificar os sintomas relatados pelos pacientes ou seus pais/responsáveis para a avaliação da esofagite eosinofílica pediátrica. Método: Realizamos o processo de tradução a partir da tradução inicial, retrotradução, seguida da etapa de obtenção de consenso por revisores independentes por meio da técnica Delphi. Aplicamos a versão pré-final a cinco pacientes de 8 a 18 anos e dez pais de pacientes de 2 a 18 anos no Serviço de Gastroenterologia Pediátrica (pré-teste). Resultados: No processo de tradução, não encontramos traduções de difícil consenso no processo de revisão ou discordâncias gramaticais. No pré-teste, identificamos dificuldades de entendimento de termos pouco convencionais, como "náusea", com sugestão de substituição para o termo "enjoo". Outra dificuldade encontrada relacionou-se aos advérbios de frequência da escala, como, por exemplo, "quase nunca", foi sugerida a substituição pelo termo "raramente". Essas dificuldades podem ser inerentes à faixa etária pediátrica. A idade dos pacientes a partir de oito anos deve ser considerada adequada para o uso de escores de autorrelato. Conclusões: A tradução do escore de sintomas da esofagite eosinofílica pediátrica produziu uma escala adaptada à cultura brasileira, que poderá ser introduzida como instrumento de investigação clínica e de pesquisa em pacientes com sintomas sugestivos de doenças esofágicas. É um importante avanço na avaliação dos sintomas, já que valoriza o relato dos próprios pacientes que convivem com essa doença.

The transcription factor Rfx7 limits metabolism of NK cells and promotes their maintenance and immunity.

Regulatory factor X 7 (Rfx7) is an uncharacterized transcription factor belonging to a family involved in ciliogenesis and immunity. Here, we found that deletion of Rfx7 leads to a decrease in natural killer (NK) cell maintenance and immunity in vivo. Genomic approaches showed that Rfx7 coordinated a transcriptional network controlling cell metabolism. Rfx7-/- NK lymphocytes presented increased size, granularity, proliferation, and energetic state, whereas genetic reduction of mTOR activity mitigated those defects. Notably, Rfx7-deficient NK lymphocytes were rescued by interleukin 15 through engagement of the Janus kinase (Jak) pathway, thus revealing the importance of this signaling for maintenance of such spontaneously activated NK cells. Rfx7 therefore emerges as a novel transcriptional regulator of NK cell homeostasis and metabolic quiescence.

Shp-2 Is Dispensable for Establishing T Cell Exhaustion and for PD-1 Signaling In Vivo.

In chronic infection and cancer, T cells acquire a dysfunctional state characterized by the expression of inhibitory receptors. In vitro studies implicated the phosphatase Shp-2 downstream of these receptors, including PD-1. However, whether Shp-2 is responsible in vivo for such dysfunctional responses remains elusive. To address this, we generated T cell-specific Shp-2-deficient mice. These mice did not show differences in controlling chronic viral infections. In this context, Shp-2-deleted CD8+ T lymphocytes expanded moderately better but were less polyfunctional than control cells. Mice with Shp-2-deficient T cells also showed no significant improvement in controlling immunogenic tumors and responded similarly to controls to α-PD-1 treatment. We therefore showed that Shp-2 is dispensable in T cells for globally establishing exhaustion and for PD-1 signaling in vivo. These results reveal the existence of redundant mechanisms downstream of inhibitory receptors and represent the foundation for defining these relevant molecular events.

Gas-phase structural characterization of neuropeptides Y Y1 receptor antagonists using mass spectrometry: Orbitrap vs triple quadrupole.

Collision induced dissociation of triple quadrupole mass spectrometer (CID-QqQ) and high-energy collision dissociation (HCD) of Orbitrap were compared for four neuropeptides Y Y1 (NPY Y1) receptor antagonists and showed similar qualitative fragmentation and structural information. Orbitrap high resolution and high mass accuracy HCD fragmentation spectra allowed unambiguous identification of product ions in the range 0.04-4.25 ppm. Orbitrap mass spectrometry showed abundant analyte-specific product ions also observed on CID-QqQ. These results show the suitability of these product ions for use in quantitative analysis by MRM mode. In addition, it was found that all compounds could be determined at levels >1 µg L-1 using the QqQ instrument and that the detection limits for this analyzer ranged from 0.02 to 0.6 µg L-1. Overall, the results obtained from experiments acquired in QqQ show a good agreement with those acquired from the Orbitrap instrument allowing the use of this relatively inexpensive technique (QqQ) for accurate quantification of these compounds in clinical and academic applications.

Translation and cultural adaptation of the Pediatric Eosinophilic Esophagitis Symptom Score (PEESS v2.0).

OBJECTIVES: To translate and culturally adapt the Pediatric Eosinophilic Esophagitis Symptom Score (version 2.0), a tool used to assess pediatric eosinophilic esophagitis symptoms reported by patients and/or their parents/caregivers. METHODS: The Pediatric Eosinophilic Esophagitis Symptom Score was translated through the following stages: initial translation, back-translation, and consensus of independent reviewers through the Delphi technique. The pre-final version of the Pediatric Eosinophilic Esophagitis Symptom Score was applied to five 8-to-18-year-old patients and to ten parents of two-to-18-year-old patients from an outpatient pediatric gastroenterology service (pre-test). RESULTS: During the translation process, no translations presenting with difficult consensus in the review process or grammar inconsistencies were observed. During the pre-test, difficulties in comprehension of some unconventional terms, e.g., "náusea", were observed. Adverbs of frequency, such as "quase nunca" were also identified as being of difficult understanding by patients and parents, and the substitution by the term "raramente" was suggested. Such difficulties may be inherent to the pediatric age group. Age 8 years or above should be considered adequate for the self-reporting of symptoms. CONCLUSIONS: The study presents the Brazilian version of the Pediatric Eosinophilic Esophagitis Symptom Score, which is adapted to the Brazilian culture. This version may be introduced as a clinical and research tool for the assessment of patients with esophagic disease symptoms. The Pediatric Eosinophilic Esophagitis Symptom Score is a breakthrough in the evaluation of symptoms of pediatric eosinophilic esophagitis, since it reinforces the importance of self-reporting by patients who experience this disease.

Production of Adjuvant-Loaded Biodegradable Particles for Use in Cancer Vaccines.

Immune adjuvants, such as ligands for pathogen-associated molecular patterns (PAMPs), have been showing promise in boosting immune responses to tumor associated antigens, and delivering these adjuvants as discrete packages is considered advantageous over delivery in soluble form. Here we describe in detail, methods for independently loading a range of adjuvants into polymer-based biodegradable particles. We also describe the means by which to characterize these particles with respect to adjuvant loading and release kinetics as well as in terms of particle size, shape, and zeta-potential. These adjuvant-loaded particles have the potential to be used in dendritic cell-based uptake experiments performed in vitro or to be used in preclinical cancer vaccine research applications where they can be co-delivered with antigen-loaded particles or some other vaccine component comprising antigenic material.

Characterization of 2,3-diarylxanthones by electrospray mass spectrometry: gas-phase chemistry versus known antioxidant activity properties.

RATIONALE: Xanthones (XH) are a class of heterocyclic compounds widely distributed in nature that hold numerous noteworthy biological and antioxidant activities. Therefore, it is of utmost importance to achieve relevant detailed structural information to understand and assist prediction of their biological properties. The potential relationship between radical-mediated xanthone chemistry in the gas phase and their promising antioxidant activities has not been previously explored. METHODS: Protonated xanthones XH1-9 were generated in the gas phase by electrospray ionization (ESI) and the main fragmentation pathways of the protonated XH1-9 formed due to collision-induced dissociation (CID) were investigated. RESULTS: In the CID-MS/MS spectra of [M+H](+) ions of XH1, XH2 and XH4 the product ions formed due to H2 O elimination corresponding to the base peak of the spectra. For the remaining six xanthones (XH3, XH5-9), showing the most promising biological profile, the product ion produced with the highest relative abundance (RA) corresponded to the one formed through concomitant loss of H2 O plus CO. Indicative of an inexistent or lower biological activity is the combined loss of CO plus O unique to the CID-MS/MS spectra of XH1, XH2, XH4, and XH5. The product ion formed by loss of 64 Da (concomitant loss of two molecules of H2 O plus CO) is only observed for xanthones containing a catechol unit (XH3 and XH6-9). This product ion has the highest RA for the most potent scavenger of reactive oxygen and nitrogen species XH9 that contains two of these catechol moieties. CONCLUSIONS: A strong relationship between some of the biological activities of the studied 2,3-diarylxanthones and their ESI-MS/MS fragmentation spectra was found. The multivariate statistical analysis results suggest that the selected MS features are related to the important biological features. Copyright © 2016 John Wiley & Sons, Ltd.

Assistência a crianças com atraso neuromotor: perfil epidemiológico e experiência interdisciplinar / Assistance to children with neuromotor delay: epidemiologic profile and interdisciplinary experience

O atendimento ao paciente pediátrico com atraso no desenvolvimento neuromotor (ADNM), em particular aqueles com diagnóstico de paralisia cerebral (PC), é um grande desafio para os serviços públicos de saúde em todo o mundo. A complexidade do manejo clínico torna relevante uma abordagem integrada e interdisciplinar. O objetivo deste estudo foi conhecer o perfil epidemiológico e as principais comorbidades dos pacientes com ADNM, particularmente com PC, atendidos em um serviço interdisciplinar de referência com 10 anos de experiência no manejo desses pacientes. Estudo transversal descritivo e retrospectivo por meio de análise de prontuários dos pacientes do Ambulatório de Pacientes Especiais do Hospital de Clínicas de Uberlândia no período de 2010 a 2012. Foram analisados 136 prontuários, 79 (58,1%) do sexo masculino e 70 (51,5%) procedentes de Uberlândia. O diagnóstico frequente foi PC 113 (83,1%). A obstipação intestinal (OI) e a doença do refluxo gastroesofágico (DRGE) foram as comorbidades mais frequentes, respectivamente, 85 (62,5%) e 80 (58,8%). Alimentação por via alternativa foi observada em 71 (52,2%), sendo 61 (44,8%) por gastrostomia e 10 (7,3%) por sonda nasoenteral. Doenças respiratórias ocorreram em 59 (43,3%) pacientes e foram responsáveis por internação em 22 (16,1%); 98 (72,0%) pacientes realizavam fisioterapia, 62 (45,6%) fonoterapia e 46 (33,8%) terapia ocupacional. Acompanhamento odontológico foi observadoem 49 (36,0%) deles. O reconhecimento do perfil epidemiológico, comorbidades e complicações mais prevalentes auxiliam a equipe interdisciplinar especializada a desenvolver estratégias para melhor assistência e cuidados aos pacientes com ADNM.

Care rendered to pediatric patients with neuromotor delay, particularly those diagnosed with Cerebral Palsy (CP), is a major challenge to public health services worldwide. The complexity of clinical management makes an integrated and multidisciplinary approach relevant. The aim of this study was to get to know the epidemiological profile and the main co- morbidities of patients with neuromotor delay, who are attended in a multidisciplinary referral service which has 10 years experience in the management of these patients. This study was carried out through cross descriptive and retrospective analysis of medical records of patients at the Special Patient Clinic at the University Hospital of Uberlândia, during the period of 2010-2012. One hundred and thirty six (136) records were analyzed, 79 (58.1%) were male and 70 (51.5 %) coming of Uberlândia. The more frequent diagnosis is CP in 113 (83.1%) Intestinal Constipation (IC) and gastro-esophageal reflux disease (GERD) were the most frequent co-morbidities, 85 (62.5%) and 80 (58.8%) respectively. Feeding by way of alternative means was observed in 71 (52.2%), with 61 (44.8%) gastrostomy and 10 (7.3%) through a nasogastric tube. Respiratory disorders occurred in 59 (43.3%) patients and were responsible for hospitalization in 22 (16.1%) cases. 98 (72.0%) patients underwent physical therapy, 62 (45.6%) speech therapy and 46 (33.8%) occupational therapy. Dental care was observed in 49 (36.0%) patients. Recognition of the epidemiological profile, comorbidities and more prevalent complications assist the specialized interdisciplinary team in developing strategies to best support and care for patients with neuromotor delay

Clinical characteristics and sensitivity to food and inhalants among children with eosinophilic esophagitis.

BACKGROUND: To understand the clinical characteristics and the diagnostic procedures in pediatric patients with eosinophilic esophagitis and to evaluate the sensitivity of the patients to food and inhalant allergens. A cross-sectional study was performed in 35 children with eosinophilic esophagitis during the time period from January 2010 to January 2011. The clinical and epidemiological data were obtained using a questionnaire and medical chart analysis. The body mass index for age was used for the nutritional evaluation (via the Z score). The sensitivity to foods and inhalants was evaluated by performing a skin prick test and atopy patch test. RESULTS: Patients (35 in total, median age 10 years) with a diagnosis of eosinophilic esophagitis were evaluated. The most prevalent symptoms in the patients were vomiting (71.4%) and abdominal pain (51.4%). Endoscopic alterations were observed in 97.2% of the patients. A good nutritional state was observed in 82.8% of the children. The tests demonstrated the presence of food sensitivities and/or aeroallergens in 27 (77.1%) patients, whereas 8 (22.9%) patients did not test positive in any of the tests performed. Among the patients with positive tests, 24 (68.5%) exhibited sensitivity to aeroallergens and 16 (45.7%) were sensitive to foods. The comparison between the sensitive and insensitive groups displayed statistically significant results with respect to sex, symptom prevalence, and 24-hour esophageal pH monitoring. CONCLUSIONS: The patients evaluated in this study displayed clinical characteristics of eosinophilic esophagitis similar to those reported in the literature. The sensitivity to foods determined by the tests was less than that observed in prior studies; however, a marked sensitivity to aeroallergens was observed. The different allergen sensitivity profiles observed in this study suggests that, similar to asthma, the eosinophiic esophagitis disease may exhibit several phenotypes.