Primary eosinophilic gastrointestinal disorders in children who have received food oral immunotherapy

BACKGROUND: Food oral immunotherapy (OIT) involves the administration of the food allergen causing the symptoms, in order to induce tolerance. Primary eosinophilic gastrointestinal disorders (PEGDs) are characterised by an eosinophil-rich inflammation affecting different locations of the digestive tract. We present a series of patients with PEGDs in a group of children following OIT with milk and/or egg. MATERIAL AND METHODS: A prospective study during the period 2006-2014 was performed in paediatric patients subjected to OIT with milk and/or egg. When these children present persistent gastrointestinal symptoms, they are referred to the Paediatric Gastroenterology Unit for evaluation. RESULTS: Primary eosinophilic gastrointestinal disorders were diagnosed in eight of the 128 cases of OIT (6.25%). The time to PEGDs development was variable: two cases showed symptoms during OIT, and the rest with a median time of 29 months (15-48 months). Food treatment discontinuation was not required in four of the five cases of eosinophilic oesophagitis, although food removal was necessary in patients with eosinophilic gastroenteritis. CONCLUSIONS: We report the highest prevalence of PEGDs in children subjected to OIT, and the first cases of eosinophilic gastroenteritis following food OIT. The monitoring of new digestive signs and symptoms after OIT is crucial for the diagnosis of these disorders, and prolonged follow-up is required. The management of such patients and the need or not to eliminate the food should be assessed on an individualised basis, according to the severity of the condition, its evolution and response to different treatment alternatives

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Primary eosinophilic gastrointestinal disorders in children who have received food oral immunotherapy.

BACKGROUND: Food oral immunotherapy (OIT) involves the administration of the food allergen causing the symptoms, in order to induce tolerance. Primary eosinophilic gastrointestinal disorders (PEGDs) are characterised by an eosinophil-rich inflammation affecting different locations of the digestive tract. We present a series of patients with PEGDs in a group of children following OIT with milk and/or egg. MATERIAL AND METHODS: A prospective study during the period 2006-2014 was performed in paediatric patients subjected to OIT with milk and/or egg. When these children present persistent gastrointestinal symptoms, they are referred to the Paediatric Gastroenterology Unit for evaluation. RESULTS: Primary eosinophilic gastrointestinal disorders were diagnosed in eight of the 128 cases of OIT (6.25%). The time to PEGDs development was variable: two cases showed symptoms during OIT, and the rest with a median time of 29 months (15-48 months). Food treatment discontinuation was not required in four of the five cases of eosinophilic oesophagitis, although food removal was necessary in patients with eosinophilic gastroenteritis. CONCLUSIONS: We report the highest prevalence of PEGDs in children subjected to OIT, and the first cases of eosinophilic gastroenteritis following food OIT. The monitoring of new digestive signs and symptoms after OIT is crucial for the diagnosis of these disorders, and prolonged follow-up is required. The management of such patients and the need or not to eliminate the food should be assessed on an individualised basis, according to the severity of the condition, its evolution and response to different treatment alternatives.

Recurrent arrival of oil to Galician coast: the final step of the Prestige deep oil spill.

Yearly monitoring in one of the most affected coastal zones by the Prestige oil spill, namely Nemiña and O Rostro beaches (NW Spain), has been carried out since 2004. Topographic data of beaches revealed seasonal altimetric changes up to 4m that would prevent the on shore persistence of oil. However, surficial and subsurficial oil was detected in the intertidal area of both beaches in all campaigns. The hydrocarbon analysis confirmed that this oil corresponded to the Prestige oil, even nine years after the accident. Tar balls were highly biodegraded suggesting that the oil was accumulated on the subtidal sediments for a long time and transported to the coast by the action of waves. The present work provides new evidence of the long term persistence of deep oil spills from wrecks in marine areas where the hydrodynamic conditions play a twofold key role, in determining the exposed coastal area to recurrent contamination and in burying and resurfacing the oil in the intertidal zone.

Linking glycolysis with oxidative stress in neural cells: a regulatory role for nitric oxide.

NO (nitric oxide) participates in a considerable number of physiological functions. At the biochemical level, most of its actions can be ascribed to its ability to bind, and activate, soluble guanylate cyclase. However, mounting evidence now strongly suggests that the NO-mediated inhibition of cytochrome c oxidase, the terminal complex of the mitochondrial respiratory chain, may be a further step of a cell signalling process involved in the regulation of important cellular functions. In most cells, including neurons and astrocytes, NO reversibly, and irreversibly, modulates O(2) consumption, a phenomenon through which NO signals certain pathways relevant for neuronal survival. Here, we propose that besides the control of mitochondrial bioenergetics, NO finely modulates the balance between glucose consumption through the glycolytic pathway and the pentose phosphate pathway in neurons. This may have implications for our understanding of the mechanisms of neurodegeneration due to oxidative and nitrosative stress.

Manifestación atípica de infección por Salmonella enteritidis / Atypical presentation of infection by Salmonella enteritidis

Es ampliamente conocido que la manifestación clínica más común de la infección por Salmonella enteritidis es la diarrea enteroinvasiva. Sin embargo, deberíamos tener presentes otras formas de presentación gastrointestinal menos frecuentes, en las que el dolor abdominal es selectivo y no siempre la diarrea es el síntoma principal. Éste es el caso de la ileítis terminal de causa infecciosa. La ileítis infecciosa es la infección del íleon terminal y ciego causada fundamentalmente por S. enteritidis, Yersinia enterocolítica y Campylobacter jejuni. Clínicamente, se presenta como dolor selectivo en el cuadrante inferior derecho, habitualmente con diarrea escasa o ausente, síntomas que pueden simular una apendicitis aguda. La ecografía abdominal es una técnica inocua y que suele ser válida para la discriminación entre ambas entidades a fin de evitar cirugías innecesarias. Presentamos un caso de ileítis infecciosa por S. enteritidis, destacando los hallazgos ecográficos sobre los que se estableció dicho diagnóstico

It is widely knownt hat the commonest clinical manifestation of infection by Salmonella enteritidis is diarrhea produced by this enteroinvasive pathogen. Nevertheless, we should not forget other less frequent forms of gastrointestinal presentation, such as terminal ileitis due to infection, in which abdominal pain rather than diarrhea can be the major symptom. One of these is bacterial ileocecitis. S. enteritidis, Yersinia enterocolitica and Campylobacter jejuni may specifically infect the ileocecal area. In these patients, right lower abdominal pain is the predominant symptom and diarrhea is frequently absent or only mild, symptoms that can mimic appendicitis. Ultrasound is a non invasive technique that usually helps to distinguish between these two entities and, thus, to prevent an unnecessary laparotomy. We report the case of a child with bacterial ileitis caused by S. enteritidis, with special attention to the ultrasound findings that led to the definitive diagnosis

Colestasis como forma atípica de presentación de infección por virus de Epstein-Barr / Cholestasis as an atypical presentation of Epstein-Barr virus infection

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Fórmulas especiales / Special formulas

Las fórmulas especiales se utilizan como tratamiento nutricional en situaciones de intolerancia y/o alergia a los componentes de las leches infantiles. Dentro de las fórmulas modificadas en hidratos de carbono, el tipo más frecuentemente utilizado es la leche sin lactosa, sobre todo en casos de intolerancia a esta azúcar secundaria a lesión de la mucosa intestinal. Dado que las fórmulas de soja contienen una proteína no láctea y carecen de lactosa, son las indicadas en la galactosemia. Las fórmulas modificadas en proteínas se utilizan principalmente en cuadros de alergia e intolerancia a proteínas de leche de vaca. Dentro de este grupo están los hidrolizados de alto grado de proteínas de la leche de vaca, las fórmulas elementales y las fórmulas de soja. Estas últimas están indicadas en casos de alergia a las proteínas de la leche de vaca (APLV) de manifestación no digestiva en lactantes mayores de 6 meses de edad. Las fórmulas elementales se utilizan en casos graves de alergia a las PLV que no responden a los hidrolizados de alto grado, frecuentemente en el contexto de múltiples alergias alimentarias, o en casos de daño intestinal grave. Los hidrolizados de proteínas de bajo grado están contraindicados en los cuadros de alergia o intolerancia a las PLV. Las fórmulas antirreflujo deben ser utilizadas sólo en niños seleccionados cuya ganancia ponderal se vea comprometida por las pérdidas de nutrientes asociadas a las regurgitaciones (AU)

Special formulas are used for nutritional treatment in situations of intolerance and/or allergy to components of infant formula. Lactose-free infant formula is mainly used for intolerance to this carbohydrate associated to intestinal mucosa injury. Because soy formulas are lactose-free, they are appropiate for use in infants with galactosemia. Formulas with modified proteins are the most commonly used for infants with cow’s milk allergy or cow’s milk intolerance. The main types of this group are: extensively hydrolyzed formula, soy-based formula and free aminoacid-based formula. Soy formulas are indicated in infants older than 6 month with extra-intestinal symptoms. Free aminoacid-based formula are indicated in more severe cases of milk protein, multiple food allergies resistant to extensively hydrolyzed formula therapy and in cases of severe intestinal damage Partially hydrolyzed formulas are not recommended in infants with cow ́s milk protein allergy. Antiregurgitation formula should be used only in selected infants with failure to thrive caused by excessive nutrient losses associated with regurgitation (AU)

Sangrado por el pezón en un lactante / Bleeding nipple in a infant

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Manifestaciones gastrointestinales en al púrpura de Schönlein-Henoch / Gastrointestinal manifestations in Henoch-Schönlein purpura

Objetivo: Describir la clínica, el diagnóstico y el tratamiento de la afectación digestiva secundaria a púrpura de Schdnlein-Henoch (PSH). Material y métodos: Se revisaron las historias de los pacientes diagnosticados de púrpura de Schdnlein-Henoch en dos hospitales pediátricos, en los últimos nueve años. Resultados: El número de pacientes con afectación digestiva fue de 40 (56% de un total de 71). La media de edad fue de 6 años y 1 mes (2-13,5). Los síntomas digestivos precedieron a la afectación cutánea en 27 pacientes (67%). Treinta y ocho pacientes padecieron dolor abdominal cólico, 12 tuvieron rectorragia, uno presentó shock hipovolémico secundario a hematemesis, y en un caso hubo enteropatía pierde-proteínas. Se efectuó ecografía abdominal a 5 pacientes, colonoscopia a 2, y esofagogastroscopia a uno. Se administraron corticoides a 7 pacientes (17%), con una dosis media de 1.5 mg/kg/día (intervalo: 1-2 mg/kg/día) y una mediana de duración del tratamiento de 7 días (3-30). La indicación en todos los casos fue la presencia de dolor abdominal refractario. La evolución tras el tratamiento con esteroides fue satisfactoria en 6 de los 7 pacientes. Conclusiones: 1. La afectación digestiva precedió en un 67% de los casos a la afectación cutánea, por lo que estimamos que la PSH debe tenerse en cuenta ante una abdominalgia de etiología no aclarada. 2. Aunque la hemorragia digestiva fue una de sus manifestaciones, ésta fue, generalmente, de escasa cuantía, y sólo se efectuó endoscopia en un 25% de los casos. 3. Los corticoides se mostraron eficaces a la hora de resolver el dolor abdominal intenso

Objective: To describe the signs and symptoms, diagnosis and treatment of gastrointestinal involvement secondary to Henoch-Schönlein purpura. Material and methods: The medical histories of patients diagnosed with Henoch-Schönlein purpura over the last nine years in two pediatric hospitals were reviewed. Results: Forty patients (56% of a total of 71) presented gastrointestinal symptoms. The mean age was 6 years and 1 month (range: 2 to 13.5 years). The gastrointestinal symptoms preceded the cutaneous involvement in 27 patients (67%). Thirty-eight patients complained of colicky abdominal pain, 12 patients presented rectal bleeding, 1 patient experienced hypovolemic shock secondary to hematemesis and there was one case of protein-losing enteropathy. Abdominal ultrasound was performed in five patients, colonoscopy in two and esophagogastroscopy in one. Corticosteroids were administered to seven patients (17%), at a mean dose of 1.5 mg/kg body weight/day (range: 1 to 2 mg/kg/day) for a mean duration of 7 days (range: 3 to 30 days). The indication was the presence of refractory abdominal pain in every case. The outcome after steroid therapy was satisfactory in 6 of the 7 patients. Conclusions: In 67% of the cases, gastrointestinal involvement preceded cutaneous involvement; thus, we consider that Henoch-Schdnlein purpura should be taken into account when dealing with abdominal pain of uncertain etiology. Although gastrointestinal hemorrhage was one of its manifestations, the amount was generally small. and endoscopy was performed in only 25% of the cases. Corticosteroids proved effective in correcting severe abdominal pain

[An association between moya-moya disease and morning glory anomaly]. / Asociación entre enfermedad de moya moya y anomalía de morning glory.

INTRODUCTION: The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. CASE REPORT: A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imaging were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a puff of smoke . This is a typical imaging for Moyamoya disease. CONCLUSIONS: In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease.

Asociación entre la enfermedad de moya-moya y la anomalía de morning glory / An association between moya-moya disease and morning glory anomaly

Introducción. La anomalía de morning glory (AMG) es una alteración congénita de la papila óptica poco frecuente, que se asocia a una disgenesia vascular retiniana. Se han descrito varios pacientes que presentan esta entidad y alteraciones cerebrovasculares compatibles con la enfermedad de moya-moya (EMM). Presentamos el caso de un niño con esta asociación. Caso clínico. Niño de 5 años, que consultó por haber presentado varios episodios de desconexión con el medio, en algunos casos precedidos de cefalea intensa y asociados a lenguaje incoherente, y, en otras ocasiones, a hipotonía y risa inmotivada, de duración variable. La exploración física y la neurológica fueron normales, salvo la papila del ojo derecho, que era muy grande y excavada, compatible con AMG. La tomografía computarizada craneal y el EEG fueron normales. En la resonancia magnética (RM) se evidenciaron múltiples imágenes puntiformes de vacío de señal en los núcleos de la base sugerentes de aumento de la vascularización y la angio-RM mostró estenosis en la porción distal de ambas carótidas internas, así como en los segmentos proximales de la arteria cerebral anterior y la arteria cerebral media izquierda, con un aumento de la circulación colateral en la zona de los núcleos de la base, compatible con la típica imagen en `volutas de humo', que confirmó el diagnóstico de EMM. Conclusión. En todo paciente diagnosticado de AMG, sobre todo si presenta sintomatología neurológica, se deberá valorar la realización de estudios de angio-RM, para descartar la existencia de EMM (AU)

Introduction. The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. Case report. A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imagine were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a ‘puff of smoke’. This is a typical imagine for Moyamoya disease. Conclusions. In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease (AU)

La patología cerebrovascular aguda en las áreas de cuidados críticos y urgencias de Andalucía. Análisis clínicos-epidemiológicos y de práctica médica / Acute cerebrovascular disease in andalousian critical care and emergency areas. An analysis on clinical, epidemiological and medical practice aspects. EVASCAN Project

Fundamento. Estudio en Andalucía de la epidemiología de la enfermedad cerebrovascular aguda (ECVA), su práctica médica y resultados, identificando áreas de mejora. Método. Estudio observacional prospectivo en 27 hospitales, de muestreo consecutivo en pacientes ingresados en Urgencias y Unidades de Cuidados Intensivos (UCI) aplicando tres cortes transversales mensuales de 24 horas de duración (días 5,15 y 25) del 5 de marzo al 25 de agosto de 1998. Criterios de inclusión: perfil clínico agudo compatible con tomografía computarizada (TC) diagnóstica. Resultados. Población de Urgencias: n = 347,81 por ciento origen isquémico, edad 71 por ciento > 65 años, factores de riesgo: 55,8 por ciento con hipertensión arterial y 16,5 por ciento con fibrilación auricular. Medios de acceso al hospital : 48,7 por ciento medios propios. Tiempo de inicio de los síntomas a su llegada en Urgencias (T1) < 3 h, tiempo de ingreso en Urgencias - TC (T2): 56,8 por ciento < 3 h. Población de UCI: n = 133 (16/27 UCI y 5,9 por ciento total pacientes), 83,9 por ciento origen hemorrágico, edad < 65 años en 67,3 por ciento, medios acceso: 43,8 por ciento por el 061. Tiempos: T1 64,4 por ciento < 3h y T2 79,8 por ciento < 3 h. APACHE III 63,2 (29,2), consumos de recursos estancia: 12 (9,5) días, ventilación mecánica: 74,2 por ciento. Mortalidad hospitalaria: 33,3 por ciento y 53 por ciento a los 12 meses. Conclusiones. La ECVA de origen isquémico queda demorada en su manejo respecto a la forma hemorrágica. Los tiempos y circuitos de manejo intra y extrahospitalarios deben reducirse. En las UCI de hospitales de referencia ingresa la ECVA hemorrágica, globalmente es una causa de ingreso muy baja, caracterizada por una alta gravedad y consumos de recursos así como muy elevada mortalidad evolutiva. (AU)