Novel Mutation in the SLC5A1 Gene Causing Glucose-Galactose Malabsorption: First Confirmed Case From Central America.

Congenital glucose-galactose malabsorption is a rare cause of life-threatening diet-induced diarrhea in infants. Mutations in the SLC5A1 gene, which encodes for the sodium-dependent glucose transporter, result in large-volume diarrhea due to aberrant glucose and galactose transport across the intestinal brush border. The diagnosis can be made clinically based on the presence of diarrhea soon after birth, evidence of carbohydrate malabsorption in the stool, and resolution of diarrhea with dietary elimination of glucose and galactose. Genetic testing can confirm the diagnosis. Here we report the first confirmed case of glucose-galactose malabsorption in an infant from Central America due to a novel mutation in the SLC5A1 gene. The patient began growing and thriving after being diagnosed and with the correct dietary interventions.

Gastrointestinal Symptoms Profile in Gastroparesis Compared to Other Functional and Organic Gastrointestinal Diseases.

OBJECTIVES: The primary objective was to compare the patient-reported gastrointestinal symptoms profiles of pediatric patients with gastroparesis to pediatric patients with 1 of 7 other functional gastrointestinal disorders and organic gastrointestinal diseases using the Pediatric Quality of Life Inventory (PedsQL) Gastrointestinal Symptoms Scales. METHODS: The gastrointestinal symptoms profiles of 64 pediatric patients with gastroparesis who manifested abnormal gastric retention based on gastric emptying scintigraphy testing were compared to 582 pediatric patients with 1 of 7 physician-diagnosed gastrointestinal disorders (functional abdominal pain, irritable bowel syndrome, functional dyspepsia, gastroesophageal reflux disease, functional constipation, Crohn disease, ulcerative colitis). The PedsQL Gastrointestinal Symptoms Scales encompass 10 individual multi-item scales which measure stomach pain, stomach discomfort when eating, food and drink limits, trouble swallowing, heartburn and reflux, nausea and vomiting, gas and bloating, constipation, blood in poop, and diarrhea/fecal incontinence, with an overall total gastrointestinal symptoms score. RESULTS: The gastrointestinal symptoms profile analysis identified significantly worse overall total gastrointestinal symptoms scores between pediatric patients with gastroparesis compared to all other gastrointestinal groups except for irritable bowel syndrome (most P s < 0.001), with significant differences for stomach discomfort when eating compared to all 7 other gastrointestinal groups (most P s < 0.001). Nausea and vomiting were significantly worse for gastroparesis compared to all other gastrointestinal groups except for functional dyspepsia (all P s < 0.001). CONCLUSIONS: Pediatric patients with gastroparesis self-reported significantly worse overall total gastrointestinal symptoms compared to all other gastrointestinal diagnostic groups except for irritable bowel syndrome, with stomach discomfort when eating and nausea and vomiting symptoms exhibiting the greatest differences compared to most gastrointestinal diagnostic groups.

Gastrointestinal Symptoms Profile in Pediatric Patients With Gastroparesis Compared to Healthy Controls.

OBJECTIVES: The primary objective was to compare the patient-reported gastrointestinal symptoms profiles of pediatric patients with gastroparesis to matched healthy controls using the Pediatric Quality of Life Inventory™ (PedsQL™) Gastrointestinal Symptoms Scales. The secondary objectives were to compare pediatric patients with gastroparesis to pediatric patients with gastroparesis-like symptoms and normal gastric emptying and to compare pediatric patients with gastroparesis-like symptoms and normal gastric emptying to matched healthy controls. METHODS: The PedsQL™ Gastrointestinal Symptoms Scales were completed by 64 pediatric patients with gastroparesis, 59 pediatric patients with gastroparesis-like symptoms and normal gastric emptying, and 200 age, gender, and race/ethnicity matched healthy controls. The PedsQL™ Gastrointestinal Symptoms Scales encompass 10 individual multi-item scales which measure stomach pain, stomach discomfort when eating, food and drink limits, trouble swallowing, heartburn and reflux, nausea and vomiting, gas and bloating, constipation, blood in poop, and diarrhea/fecal incontinence. Based on gastric emptying scintigraphy testing, those with abnormal gastric retention were classified as having gastroparesis. RESULTS: The gastrointestinal symptoms profile analysis identified large differences between those with gastroparesis compared to healthy controls (most P <0.001), with the largest effect sizes for upper gastrointestinal symptoms including stomach pain, stomach discomfort when eating, food and drink limits, nausea, and vomiting. Those with gastroparesis self-reported similar gastrointestinal symptoms to those with normal gastric emptying, except for increased constipation. CONCLUSIONS: Pediatric patients with gastroparesis self-reported broad multidimensional gastrointestinal symptoms profiles in comparison to healthy controls with large differences, indicating the critical need for more highly efficacious interventions to bring patient functioning within the normal range of healthy functioning.

Probiotics for treatment of chronic constipation in children.

BACKGROUND: Functional constipation is defined as chronic constipation with no identifiable underlying cause. It is a significant cause of morbidity in children, accounting for up to 25% of visits to paediatric gastroenterologists. Probiotic preparations may sufficiently alter the gut microbiome and promote normal gut physiology in a way that helps relieve functional constipation. Several studies have sought to address this hypothesis, as well as the role of probiotics in other functional gut disorders. Therefore, it is important to have a focused review to assess the evidence to date. OBJECTIVES: To evaluate the efficacy and safety of probiotics for the management of chronic constipation without a physical explanation in children. SEARCH METHODS: On 28 June 2021, we searched CENTRAL, MEDLINE, Embase, CINAHL, AMED, WHO ICTR, and ClinicalTrials.gov, with no language, date, publication status, or document type limitations. SELECTION CRITERIA: We included randomised controlled trials (RCTs) that assessed probiotic preparations (including synbiotics) compared to placebo, no treatment or any other interventional preparation in people aged between 0 and 18 years old with a diagnosis of functional constipation according to consensus criteria (such as Rome IV). DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: We included 14 studies (1127 randomised participants): 12 studies assessed probiotics in the treatment of functional constipation, whilst two studies investigated synbiotic preparations. Three studies compared probiotics to placebo in relation to the frequency of defecation at study end, but we did not pool them as there was very significant unexplained heterogeneity. Four studies compared probiotics to placebo in relation to treatment success. There may be no difference in global improvement/treatment success (RR 1.29, 95% CI 0.73 to 2.26; 313 participants; low-certainty evidence). Five studies compared probiotics to placebo in relation to withdrawals due to adverse events, with the pooled effect suggesting there may be no difference (RR 0.64, 95% CI 0.21 to 1.95; 357 participants; low-certainty evidence). The pooled estimate from three studies that compared probiotics plus an osmotic laxative to osmotic laxative alone found there may be no difference in frequency of defecation (MD -0.01, 95% CI -0.57 to 0.56; 268 participants; low-certainty evidence). Two studies compared probiotics plus an osmotic laxative to osmotic laxative alone in relation to global improvement/treatment success, and found there may be no difference between the treatments (RR 0.95, 95% CI 0.79 to 1.15; 139 participants; low-certainty evidence). Three studies compared probiotics plus osmotic laxative to osmotic laxative alone in relation to withdrawals due to adverse events, but it is unclear if there is a difference between them (RR 2.86, 95% CI 0.12 to 68.35; 268 participants; very low-certainty evidence). Two studies compared probiotics versus magnesium oxide. It is unclear if there is a difference in frequency of defecation (MD 0.28, 95% CI -0.58 to 1.14; 36 participants), treatment success (RR 1.08, 95% CI 0.74 to 1.57; 36 participants) or withdrawals due to adverse events (RR 0.50, 95% CI 0.05 to 5.04; 77 participants). The certainty of the evidence is very low for these outcomes. One study assessed the role of a synbiotic preparation in comparison to placebo. There may be higher treatment success in favour of synbiotics compared to placebo (RR 2.32, 95% CI 1.54 to 3.47; 155 participants; low-certainty evidence). The study reported that there were no withdrawals due to adverse effects in either group. One study assessed a synbiotic plus paraffin compared to paraffin alone. It is uncertain if there is a difference in frequency of defecation (MD 0.74, 95% CI -0.96, 2.44; 66 participants; very low-certainty evidence), or treatment success (RR 0.91, 95% CI 0.71 to 1.17; 66 participants; very low-certainty evidence). The study reported that there were no withdrawals due to adverse effects in either group. One study compared a synbiotic preparation to paraffin. It is uncertain if there is a difference in frequency of defecation (MD -1.53, 95% CI -3.00, -0.06; 60 participants; very low-certainty evidence) or in treatment success (RR 0.86, 95% CI 0.65, 1.13; 60 participants; very low-certainty evidence). The study reported that there were no withdrawals due to adverse effects in either group. All secondary outcomes were either not reported or reported in a way that did not allow for analysis. AUTHORS' CONCLUSIONS: There is insufficient evidence to conclude whether probiotics are efficacious in successfully treating chronic constipation without a physical explanation in children or changing the frequency of defecation, or whether there is a difference in withdrawals due to adverse events when compared with placebo. There is limited evidence from one study to suggest a synbiotic preparation may be more likely than placebo to lead to treatment success, with no difference in withdrawals due to adverse events. There is insufficient evidence to draw efficacy or safety conclusions about the use of probiotics in combination with or in comparison to any of the other interventions reported. The majority of the studies that presented data on serious adverse events reported that no events occurred. Two studies did not report this outcome. Future studies are needed to confirm efficacy, but the research community requires guidance on the best context for probiotics in such studies, considering where they should be best considered in a potential treatment hierarchy and should align with core outcome sets to support future interpretation of findings.

Actividad Antioxidante, Antibacteriana y Citostática de Extractos de Cúrcuma (Curcuma Longa)

Resumen Parte de la Industria Farmacéutica se desarrolla sobre la base de nuevas plantas descubiertas por los pueblos indígenas, la Cúrcuma, lo utilizaban de manera tradicional para el tratamiento de afecciones cutáneas, hepáticas, digestivas, cancerígenas, infecciones parasitarias, virales, bacterianas, entre otras. Objetivos: evaluar la actividad antioxidante, antibacteriana, tóxica, citostática y determinar la composición fitoquímica de extractos de Curcuma longa cultivada en el sector de Cristal Mayu del Chapare. Métodos: se prepararon dos extractos etanólicos 70% y 96%, se cuantificó los polifenoles por el método de Folin-ciocalteu, se evaluó la actividad antioxidante con el reactivo DPPH, se evaluó la actividad antibacteriana con dos bacterias gram negativas (E. coli y K. pneumoniae) y una bacteria gram positiva (S. aureus), se determinó la toxicidad sobre la Artemia salina y la actividad citostática mediante el ensayo de inhibición del crecimiento de la raíz de lechuga. Se cuantificó el total de curcuminoides. Resultados: el extracto al 96% presenta mayor cantidad de fenoles totales y posee actividad antioxidante. Solo el extracto al 96% presenta actividad antibacteriana frente a S. aureus, E. coli, K. pneumopniae. Por otro lado, la Cúrcuma presenta toxicidad frente a la Artemia salina, inhibe el crecimiento de la raíz de lechuga y posee un 5% de curcuminoides totales. Conclusiones: la caracterización de los compuestos confirma su composición como polifenoles, lo cual se relaciona a la actividad biológica de éstos, encontrándose que la Cúrcuma posee actividades antioxidantes, antibacterianas, citotóxicas y su importancia en el área de la salud como una alternativa para el tratamiento de muchas enfermedades.

Abstract Part of the pharmaceutical industry is developed on the basis of new plants discovered by indigenous peoples, turmeric, used in a traditional way for the treatment of skin, liver, digestive, carcinogenic, parasitic, viral, bacterial infections, among others. Objective: to evaluate the antioxidant, antibacterial, toxic and cytostatic activity and to determine the phytochemistry of the turmeric (Curcuma longa) extract, grown in the Cristal Mayu area of Chapare. Methods: two ethanolic extracts 70% and 96% were prepared, polyphenols were quantified by Folin-ciocalteu method, antioxidant activity was evaluated with DPPH reagent, antibacterial activity was evaluated with two gram negative bacteria (E. coli and K. pneumoniae) and one gram positive bacteria (S. aureus), toxicity on Artemia salina and cytostatic activity was determined by lettuce root growth inhibition assay. Total curcuminoids were quantified. Results: the 96% extract has a higher amount of total phenols and antioxidant activity. Only the 96% extract showed antibacterial activity against S. aureus, E. coli, K. pneumopniae. On the other hand, turmeric has toxicity against Artemia salina, inhibits the growth of lettuce root and has 5% of total curcuminoids. Conclusions: characterization of the compounds confirms their composition as polyphenols, which relates to their biological activity, showing that Turmeric has antioxidant, antibacterial and cytotoxic activity, as well as its importance in the healthcare field as an alternative treatment for various diseases.

Gastric accommodation: Physiology, diagnostic modalities, clinical relevance, and therapies.

BACKGROUND: Gastric accommodation is an essential gastric motor function which occurs following ingestion of a meal. Impaired gastric fundic accommodation (IFA) is associated with dyspeptic symptoms. Gastric accommodation is mediated by the vagal pathway with several important physiologic factors such as duodenal nutrient feedback playing a significant role. IFA has been described as a pathophysiologic factor in several gastrointestinal disorders including functional dyspepsia, diabetic gastropathy, post-Nissen fundoplication, postsurgical gastrectomy, and rumination syndrome. Modalities for gastric accommodation assessment include gastric barostat, intragastric meal distribution via scintigraphy, drinking tests (eg, water load), SPECT, MRI, 2D and 3D ultrasound, and intragastric high-resolution manometry. Several treatment options including sumatriptan, buspirone, tandospirone, ondansetron, and acotiamide may improve symptoms by increasing post-meal gastric volume. PURPOSE: Our aim is to provide an overview of the physiology, diagnostic modalities, and therapies for IFA. A literature search was conducted on PubMed, Google Scholar, and other sources to identify relevant studies available until December 2020. Gastric accommodation is an important gastric motor function which if impaired, is associated with several upper gastrointestinal disorders. There are an increasing number of gastric accommodation testing modalities; however, each has facets which warrant consideration. Evidence regarding potentially effective therapies for IFA is growing.

PedsQL™ Gastroparesis Symptoms Module Domain and Item Development: Qualitative Methods.

OBJECTIVES: Like adults, children suffer from gastroparesis; however, there are currently no validated instruments to determine the impact of gastroparesis in pediatric patients. The objective of this study was to develop the items and domains to support the content validity of the new Pediatric Quality of Life Inventory (PedsQL™) Gastroparesis Symptoms Module. METHODS: Patients were recruited from the National Institute of Diabetes and Digestive and Kidney Diseases Pediatric Gastroparesis Registry. The qualitative methods involved an iterative process comprising a literature review of existing questionnaires and gastroparesis clinical research, an expert review panel of pediatric gastroenterologists who provided feedback on the conceptual framework in developing the semi-structured interview, and in-depth focus interviews with six pediatric patients with gastroparesis and five of their parents (one did not participate) in developing relevant domains and item content. In the subsequent cognitive interviews phase, five additional patients with gastroparesis and their parents provided detailed feedback on item content, relevance, importance, and understandability of the domains and items. RESULTS: Ten domains/scales were derived from the qualitative methods, with item content saturation achieved at 67 items, with no further themes or content identified during the final cognitive interviews. The Module is comprised of 10 individual scales measuring nausea, stomach fullness when eating, vomiting, dry heaves, heartburn and reflux, stomach pain and hurt, food and drink limits, bloating, appetite, and worry. CONCLUSIONS: Our study supports the content validity of the new PedsQL Gastroparesis Symptoms Module. The Module field test study will be conducted in a multisite national study.

Meal-Induced Symptoms in Children with Dyspepsia-Relationships to Sex and the Presence of Gastroparesis.

OBJECTIVES: To test the hypothesis that in children with dyspepsia, prospective symptom severity following ingestion of a meal would correlate with percent gastric retention, and those ultimately diagnosed with gastroparesis would report worse symptoms. STUDY DESIGN: Prospective, single-center study with 104 children with dyspepsia completing a prospective dyspepsia symptom questionnaire before and after eating a standardized Tougas meal during gastric emptying scintigraphy. Main outcomes included correlation between gastric retention and symptoms and comparison of symptom severity between those with and without gastroparesis. RESULTS: Fifty-two children (50%) had gastroparesis (gastroparesis: 12.5 ± 2.9 years, 65% female; nongastroparesis: 13.0 ± 2.9 years, 60% female; all P > .05). Bloating was the only symptom significantly worse in youth with gastroparesis. For the entire cohort, bloating and fullness correlated with percent retention. However, in those with gastroparesis, only nausea correlated with retention (4 hours.; rs = 0.275, P < .05). Girls with gastroparesis had significantly worse symptoms (except satiety) when compared with boys with gastroparesis (P < .05). CONCLUSIONS: Overall in children, there is little difference in symptom severity between children with gastroparesis vs normal emptying based on current standards. However, girls with gastroparesis have worse symptoms vs boys with gastroparesis, underscoring a need for further studies into the role of sex in gastroparesis symptoms. In all children, both bloating and fullness correlated modestly with gastric retention, and nausea correlated in those with gastroparesis.

Endoscopic ultrasound-guided diagnosis of Helicobacter pylori-associated gastric Burkitt's lymphoma in an adolescent patient: a rare case.

Primary gastric Burkitt's lymphoma (BL) is rare in the pediatric population. Furthermore, the association of Burkitt's lymphoma with Helicobacter pylori is not well defined. We report a case of primary gastric Burkitt's lymphoma associated with Helicobacter pylori diagnosed in a pediatric patient. This diagnosis was made with the aid of endoscopic ultrasound (EUS)-guided fine-needle biopsy (FNB). This is one of the first pediatric cases of EUS-guided FNB for the diagnosis of H. pylori-associated gastric BL.

Caracterización del síndrome coronario agudo en mujeres / Characterization of acute coronary syndrome in women

RESUMEN Introducción: Las mujeres afectadas por síndromes coronarios agudos (SCA) tienen peor pronóstico y son más propensas a presentar complicaciones. Objetivo: Caracterizar las pacientes con SCA ingresadas en el Hospital Dr. Carlos J. Finlay (La Habana, Cuba) entre junio de 2012 y junio de 2018. Método: Estudio descriptivo transversal con 1252 mujeres. Fueron definidos 2 grupos: SCA con (SCACEST) y sin elevación del segmento ST (SCASEST). Las variables de estudio fueron: grupos de edad, factores de riesgo, características angiográficas y complicaciones intrahospitalarias. Resultados: La media de edad fue 66,2 ± 11,9 años, predominó el SCASEST (73,4% vs. 26,6%). La hipertensión arterial (95,2%), el tabaquismo (37,9%) y la diabetes mellitus (36,3%) fueron factores de riesgo más prevalentes, con diferencias significativas a favor del SCASEST (p<0,004). Los valores de glucemia (69,1% vs. 51,5%; p<0,0001) y colesterol total (46,2% vs. 16,6%; p<0,0001) fueron significativamente mayores en el SCASEST. A un 29,3% de los casos se les realizó coronariografía, donde se encontró una alta prevalencia de lesiones significativas (73,3%) y una incidencia de enfermedad de tronco de 4,9%. Un 23% presentó complicaciones, las más prevalentes fueron la insuficiencia cardíaca (35,1%) y el shock cardiogénico (18,1%), esta última predominó como causa de muerte en el SCACEST (45,1 vs. 11,8; p<0,0001). Ambas complicaciones se relacionaron directamente con la mortalidad (3,8%). Conclusiones: En las mujeres con SCA predominó el SCASEST en edades posmenopáusicas. La hipertensión arterial, la diabetes mellitus y el tabaquismo constituyeron las principales características clínicas. La insuficiencia cardíaca y el shock cardiogénico se asociaron frecuentemente a la mortalidad.

ABSTARCT Introduction: Women suffering from acute coronary syndromes (ACS) have worse prognosis and are prone to major adverse events. Objective: To characterize female patients with ACS admitted to the Hospital Dr. Carlos J. Finlay (Havana, Cuba) between June 2012 and June 2018. Method: A cross-sectional descriptive study with 1252 women was carried out. Two groups were defined: ST-segment elevation ACS (STE-ACS) and non-ST-segment elevation ACS (NSTE-ACS). Study variables were: age groups, risk factors, angiographic characteristics and in-hospital major adverse events. Results: Mean age was 66.2 ± 11.9 years old, NSTE-ACS predominated (73.4% vs 26,6%). High blood pressure (95.2%), tobacco smoking (37.9%) and diabetes mellitus (36.3%) were the prevailing risk factor, with significant differences in favor of NSTE-ACS (p <0.004). Glycemia values (69.1% vs 51.5%, p <0.00001) and total cholesterol (46.2% vs 16.6%, p <0.00001) were significantly higher in the NSTE-ACS. A 29.3% of cases underwent coronary angiography where a high prevalence of serious lesions of 73.3% and an incidence of left main coronary artery disease of 4.9% were found. A 23% presented major adverse events, heart failure (35.1%) and cardiogenic shock (18.1%) being the most prevalent ones. Cardiogenic shock predominated as cause of death in STE-ACS (45.1 vs 11,8; p<0,0001). Both major adverse events were directly related to mortality (3.8%). Conclusions: There is a predominance of NSTE-ACS in women with ACS in postmenopausal ages. High blood pressure, diabetes mellitus and tobacco smoking were the main clinical characteristics. Cardiogenic shock and heart failure were the major adverse events most frequently associated to mortality.

Análisis de la Sobredemanda del Hospital General de Barrio Obrero. Implicancias organizacionales y funcionales de una microrred urbana. Asunción, Paraguay 2018-2019 / Analysis of the Excess Demand of the Barrio Obrero General Hospital. Organizational and functional implications of an urban micronetwork. Asunción, Paraguay 2018-2019

La ampliación del acceso a servicios sanitarios constituye el objetivo intrínseco del Sistema de Salud, cuyos resultados dependen de dinámicas y preferencias de los usuarios y del equilibrio de variables no controlables para alcanzar mejores resultados. Esta investigación evaluativa, cuali-cuantitativa, de corte transversal identifica algunas de estas variables, en un hospital general, cabecera de una micro red de servicios de salud urbana, en Asunción, Paraguay. La unidad de análisis incluyo actores clave ejerciendo roles directivos, gerenciales y asistenciales y usuarios fidelizados a la unidad efectora estudiada. La muestra incluyo 10 directivos, 20 profesionales de salud y 150 usuarios de consultorio externo, urgencias e internados, en tres turnos de atención. Se aplicaron tres cuestionarios conteniendo preguntas semiestructuradas sobre oferta, demanda, organización, supervisión, subsistema de información, comunicación interna, motivos de consulta, tiempos de espera, capacidad instalada, disponibilidad de insumos y medicamentos, motivos de preferencia y algunas variables sociales, epidemiológicas y culturales. Usuarios desconocen la organización de los servicios que integran la micro red urbana, prefieren el hospital general para acceder efectivamente a medios diagnósticos 82 %, especialistas, medicamentos y capacidad de respuesta integral 57%, 9,9 % posee seguro médico, el 76 % declara empleo formal, 40 % gana más del sueldo mínimo, 95 % de pacientes declaran buena atención en el Hospital General Barrio Obrero, 69 % está satisfecho. El fortalecimiento de los servicios de primer y segundo nivel puede equilibrar la sobredemanda del hospital cabecera, con implementación de innovaciones organizacionales, incremento de la inversión y adecuados planes de comunicación social

The expansion of the access to health services is the intrinsic objective of the health system, whose performance depends on user dynamics and preferences and the equilibrium of independent variables to achieve better results. The objective of this study was to evaluate dynamics and factors related to the excess demand in a general hospital, head of an urban micro network in Asunción, Paraguay. This was a quali-quantitative, cross-sectional evaluative study in which the unit of analysis were health actors in directive, managerial, and care providing roles as well as loyal users. The sample included 10 directors, 20 health professionals and 150 users in the areas of consultations, emergencies and hospitalization of three different shifts. Three questionnaires with semi-structured questions on offer, demand, organization, supervision, information subsystem, internal communication, reasons for consultation, waiting times, installed capacity, availability of supplies and medicines, hospital organization, supervision, reasons for preference and some social, epidemiological and cultural variables. The users did not know the organization of the services that were part of the urban micro network. They preferred the general hospital to effectively access to diagnostic means (82%), to specialists, medicines and capacity of integral response (57%), 9.9% did not have medical insurance, 76% said they had formal jobs, 40% earned more that the minimal wage, 95% said that the Barrio Obrero General Hospital had good attention and 69% of them were satisfied. The strengthening of first and second level services can balance the excess demand of the main hospital, with the implementation of organizational innovations, increased investment and adequate social communication plans

Hematemesis in a Newborn: A Case Report.

We present the case of a term-healthy neonate who developed hematemesis while being admitted at the newborn nursery. The infant was found to have gastric ulcers with duodenal eosinophils. The condition was conservatively managed and the symptoms, including ulcers, resolved with time; however, the etiology of the ulcers is still unknown.

Torsión de puntas en una paciente con síndrome de QT largo congénito durante el puerperio: Presentación de un caso / Torsades de pointes in a patient with congenital long QT syndrome during the puerperium: Case report

RESUMEN El síndrome de QT largo congénito es una enfermedad eléctrica primaria del corazón que predispone a la ocurrencia de arritmias ventriculares malignas. Se traduce en una prolongación del intervalo QT en el electrocardiograma y la torsión de puntas es la arritmia que ocasiona síncope y, en ocasiones, muerte súbita. El embarazo y el puerperio aumentan la incidencia de estos eventos. Se presenta el caso de una puérpera afectada que presentó crisis de ansiedad y desmayos interpretados como psicógenos. Se documentó torsión de puntas sin respuesta a los fármacos antiarrítmicos diponibles y se trasladó al centro de referencia (Instituto de Cardiología y Cirugía Cardiovascular), donde se aumentó la frecuencia de estimulación del marcapasos y, posteriormente, se implantó un desfibrilador automático. Se trata de un caso infrecuente que constituyó un verdadero reto en el tratamiento integral y emergente, todo lo cual posibilitó la supervivencia de la paciente.

ABSTRACT Congenital long QT syndrome is a primary electrical disorder of the heart which predisposes to the occurrence of malignant ventricular arrhythmias. It is characterized by a prolongation of the QT interval on the electrocardiogram and the torsade de pointes is the main associated arrhythmia, resulting in syncope and sudden cardiac death. Pregnancy and puerperium increase the incidence of those events. We present the case of a patient who suffered from this disorder, and during the post-delivery period, she had events of faint and anxiety interpreted as psychogenic. Torsades de pointes without response to the available antiarrhythmic drugs was documented and she was transferred to the reference center (Instituto de Cardiología y Cirugía Cardiovascular), where the pacemaker stimulation frequency was increased and, subsequently, an implantable cardioverter defibrillator was implanted. This is an infrequent case that was a real challenge for the comprehensive and emergent treatment, all of which enabled the survival of the patient.

Childhood gastroparesis is a unique entity in need of further investigation.

BACKGROUND: Despite increasing knowledge regarding gastroparesis (GP) in adults, little is known regarding the incidence, prevalence, and natural history of childhood GP. Exacerbating the knowledge gap in pediatric GP is both the lack of normative data for gastric emptying scintigraphy in children and lack of GP-specific pediatric reported outcome measures. PURPOSE: The aim of this article was to review the available literature on pediatric GP and identify similarities and differences with studies in adults. We performed a comprehensive search in MEDLINE and Google Scholar from inception to April 2019 for articles published in English using the following combination of keywords: gastroparesis, pediatric gastroparesis, outcomes, metoclopramide, erythromycin, domperidone, cisapride, and gastric neurostimulator. The limited available pediatric data, often retrospective, suggest marked differences between adult and pediatric GP in several aspects including etiology, concomitant co-morbidities (eg, psychiatric disorders), clinical symptom presentation, diagnostic evaluation, response to therapies, and clinical outcome. Further research in pediatric GP is needed and holds the promise to further elucidate the mechanisms of this disorder in children and lead to pediatric-focused therapies.

Volumen plaquetario medio y posible valor pronóstico en pacientes con infarto agudo de miocardio / Mean platelet volume and possible prognostic value in patients with acute myocardial infarction

Introducción: Numerosos factores están relacionados con las complicaciones intrahospitalarias en pacientes con infarto agudo de miocardio, la utilidad del volumen plaquetario medio como factor pronóstico es polémica actualmente. Objetivo: Describir la asociación entre el volumen plaquetario medio en pacientes con infarto agudo de miocardio, con los factores de riesgo cardiovascular y la evolución clínica. Método: Estudio observacional en 188 pacientes con infarto agudo del miocardio, que ingresaron en el servicio de Cardiología del Hospital Militar Central "Dr. Carlos J. Finlay". Fueron vigiladas las siguientes complicaciones: muerte cardiaca, insuficiencia cardiaca, angina postinfarto, reinfarto y todas combinadas. Se determinó el volumen plaquetario mediante hemograma automatizado al ingreso y se utilizaron como valores de corte < 9 fL y ≥ 9 fL. Resultados: Predominó el sexo masculino (69,7 por ciento) y mayores de 50 años (91,5 por ciento). Los pacientes hipertensos, diabéticos y fumadores presentaron mayor volumen plaquetario (82,9 por ciento, p= 0,00; 90,4 por ciento, p= 0,03; 89,1 por ciento, p= 0,00 respectivamente). El 22,3 por ciento presentó complicaciones combinadas; la insuficiencia cardiaca (45,2 por ciento) y la angina postinfarto (3,3 por ciento) fueron las más frecuentes. La insuficiencia cardiaca constituyó la complicación asociada con volumen plaquetario elevado (p= 0,027). El volumen plaquetario ≥ 9 fL constituyó un predictor independiente de eventos adversos (p= 0,00; IC 95 por ciento: 3,89-112,908). Conclusiones: El volumen plaquetario medio elevado estuvo asociado a peor evolución clínica intrahospitalaria y la insuficiencia cardiaca la complicación más prevalente. Se asoció además a la hipertensión arterial, tabaquismo y diabetes mellitus(AU)

Introduction: Numerous factors are related to in-hospital complications in patients with acute myocardial infarction, the usefulness of the mean platelet volume as a prognostic factor is currently controversial. Objective: To describe the association between mean platelet volume in patients with acute myocardial infarction, with cardiovascular risk factors and clinical evolution. Method: Observational study in 188 patients with acute myocardial infarction, who were admitted to the Cardiology Department of the Central Military Hospital "Dr. Carlos J. Finlay ". The following complications were monitored: cardiac death, heart failure, post-infarction angina, reinfarction and all combined. Platelet volume was determined by automated blood count at admission and <9 fL and ≥ 9 fL were used as cut-off values. Results: The male sex (69.7 percent) and over 50 years (91.5 percent) predominated. Hypertensive, diabetic and smoking patients presented higher platelet volume (82.9 percent, p = 0.00; 90.4 percent, p = 0.03; 89.1 percent, p = 0.00 respectively). 22.3 percent presented combined complications; Heart failure (45.2 percent) and post-infarction angina (3.3 percent) were the most frequent. Heart failure was the complication associated with high platelet volume (p = 0.027). Platelet volume ≥ 9 fL was an independent predictor of adverse events (p = 0.00; 95 percent CI: 3.89-112.908). Conclusions: The high mean platelet volume was associated with worse intrahospital clinical evolution and heart failure the most prevalent complication. It was also associated with high blood pressure, smoking and diabetes mellitus(AU)

Disección aórtica como componente de alteraciones aórticas múltiples: presentación de caso / Aortic dissection as a component of multiple aortic alterations: case report

La disección aórtica aguda es el episodio catastrófico más frecuente que afecta a la aorta, su mortalidad precoz es muy alta. El factor de riesgo más frecuente es la hipertensión arterial, aunque se describen otros como las enfermedades de la aorta, los traumatismos y el tabaquismo. Se describe el caso de un paciente de 63 años con antecedentes de hipertensión arterial que llega a Urgencias con dolor torácico intenso con irradiación a abdomen y dorso. Se realizó angiotomografía computarizada y se diagnosticó una disección aórtica tipo IIIb de DeBakey, con extensión hasta la arteria femoral común derecha. Como hallazgo se diagnosticaron, además, dos malformaciones congénitas: emergencia de la arteria carótida común izquierda y el tronco arterial braquiocefálico de un solo tronco común (arco aórtico bovino), y un remanente de conducto arterioso. Luego de un mes de internamiento, el paciente fue egresado del servicio con estabilidad clínica, en espera de tratamiento quirúrgico electivo(AU)