Colitis ulcerosa asociada a hepatitis autoinmune: ¿una forma diferencial de enfermedad inflamatoria intestinal? / Ulcerative colitis associated with autoimmune hepatitis: a differential form of inflammatory bowel disease?

La enfermedad inflamatoria intestinal (EII) es un trastorno inflamatorio crónico del tracto intestinal de patogénesis multifactorial, con posible asociación a distintos desórdenes autoinmunes, entre los cuales se encuentra la hepatitis autoinmune (HAI). Se ha postulado una serie de características diferenciales de la EII asociada a HAI en niños. Nuestro objetivo es describir las características diferenciales observadas en nuestros pacientes con EII asociada a HAI respecto a aquellos con formas clásicas de la enfermedad, confirmando dicha singularidad

Inflammatory bowel Disease (IBD) is a group of chronic inflammatory diseases that can be associated with different autoimmune diseases, including autoimmune hepatitis (AIH).Some specific and differential characteristics in children with IBD associated to AIH have beendescribed. Our aim is to describe the clinical pattern of this association observed in our patients,confirming its differential characteristics as compared to classical IBD in children

[Ulcerative colitis associated with autoimmune hepatitis: a differential form of inflammatory bowel disease?]. / Colitis ulcerosa asociada a hepatitis autoinmune: ¿una forma diferencial de enfermedad inflamatoria intestinal?

Inflammatory bowel Disease (IBD) is a group of chronic inflammatory diseases that can be associated with different autoimmune diseases, including autoimmune hepatitis (AIH). Some specific and differential characteristics in children with IBD associated to AIH have been described. Our aim is to describe the clinical pattern of this association observed in our patients, confirming its differential characteristics as compared to classical IBD in children.

Enfermedad de Crohn y eritema nudoso: ¿es útil la nutrición enteral exclusiva? / Crohn's disease and erythema nodusum: Is exclusive enteral nutrition useful?

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Valoración del uso de «Lactobacillus reuteri» en el tratamiento de los cólicos del lactante: estudio piloto / Valuation the use of Lactobacillus reuteri in the treatment of infant colic: a pilot study

Introducción: El cólico del lactante es un cuadro de malestar abdominal, definido por Wessel y recogido en los criterios de Roma III. Aunque se considera benigno, guarda cierta relación con los marcadores inflamatorios, y provoca un trastorno en el lactante y una gran ansiedad en sus familiares. Últimamente el uso de probióticos como Lactobacillus reuteri ha obtenido buenos resultados en otras poblaciones europeas. Objetivo: Como la microbiota modula la respuesta inflamatoria intestinal y depende de factores ambientales y genéticos, propusimos un estudio piloto para tratar de reproducir estos resultados en nuestra población. Método: Estudio observacional y prospectivo de la sintomatología relacionada con los cólicos en 17 lactantes sanos, a los que se prescribió L. reuteri DSM 17938, indicación aprobada por la Agencia Española Reguladora de la Alimentación, en todos los casos con el consentimiento informado y escrito de los familiares. Se realizó un seguimiento mediante un cuestionario de datos clínicos y dos visitas presenciales para el control y la determinación de calprotectina en heces. El estudio estadístico se llevó a cabo mediante el programa Stata v11, con el test de la ji al cuadrado y un análisis de regresión lineal. Resultados: Los parámetros clínicos, como el tiempo del llanto y el malestar del lactante, fueron mejorando según pasaban las semanas. La percepción de mejoría y la disminución de los niveles de calprotectina en heces también mostraron una evolución paralela. Las curvas de peso y talla no se vieron afectadas. Conclusiones: El uso de probióticos mejora claramente la sintomatología del lactante, reduce la ansiedad familiar, disminuye los valores de los marcadores inflamatorios intestinales y no interfiere en el desarrollo ponderoestatural de los pacientes (AU)

Introduction: Infantile colic is a picture of abdominal discomfort, defined by Wessel and included in the Rome III criteria. Although considered benign somewhat related inflammatory markers, causing a disorder in infants and a major anxiety in their family. Lately the use of probiotics such as Lactobacillus reuteri have been successful in other European populations. Objective: Microbiota modulates intestinal inflammatory response and depends on genetic and environmental factors. We proposed a pilot study to try to reproduce the European results in our population. Method: Observational prospective study of symptoms associated with colic in 17 healthy infants who were prescribed L. reuteri DSM 17938, indication approved by the Spanish Regulatory Food Agency. In all the written and informed consent of relatives was obteined. Follow-up clinical data using questionnaires and two physical visits to control and determination of calprotectin in feces. Statistical analysis with Stata v11 program chi 2 test and linear regression analysis. Results: Evolution of clinical parameters such as time crying and infant discomfort were lower as the weeks passed. The perception of improvement and the reduction of faecal calprotectin levels also showed a parallel evolution. The height and weight were not affected. Conclusions: The use of L. reuteri clearly improves symptoms and reduces infant family anxiety, and the values of intestinal inflammatory markers. Its use does not interfere with the development of weight-height patients. Studies are necessary with larger populations to confirm these initial data (AU)

Problemas nutricionales percibidos por los pediatras en niños españoles menores de 3 años / Nutritional problems perceived by pediatricians in Spanish children younger than 3 years

Introducción: Los padres son los principales responsables de la educación nutricional de sus hijos y es labor del pediatra orientar y resolver problemas al respecto. En este estudio se pretende conocer la relevancia de las cuestiones nutricionales en la consulta del pediatra y los principales problemas nutricionales percibidos en los menores de 3 años. Métodos: Estudio descriptivo, transversal en dos fases. La primera consistió en entrevistas y grupos de discusión con 30 pediatras seleccionados aleatoriamente en Madrid y Barcelona. Los resultados se utilizaron para diseñar el cuestionario online (76 preguntas relacionadas con la nutrición en niños de 0-3 años) de la siguiente fase, en la que participaron pediatras seleccionados de forma aleatoria y representativa del territorio nacional. Resultados: De 258 pediatras seleccionados, completaron el cuestionario 151, que atendían a una media de 588 pacientes/mes. Los principales problemas nutricionales percibidos hasta los 12 meses fueron las deficiencias de hierro y vitaminas y la escasa ganancia de peso, y posteriormente la ingesta excesiva de carbohidratos y lípidos y el sobrepeso. Los padres fueron considerados los actores principales en la salud nutricional de sus hijos, pero su preocupación por esta cuestión se reduce significativamente (p < 0,0001) con la edad del niño. Los aspectos considerados más importantes para obtener unos buenos hábitos alimentarios fueron la alimentación adaptada a las necesidades del niño y respetar el tiempo de sueño. El porcentaje de pacientes que recibe recomendaciones de hábitos nutricionales varió del 88% (0-6 meses) al 61% (24-36 meses). Conclusiones: A pesar de considerar la salud nutricional importante, la intervención educativa del pediatra al respecto no es óptima. Los problemas nutricionales percibidos fueron distintos según la edad (AU)

Introduction: Parents are most responsible for nutritional education of children, and pediatritians must advise and help them with their doubts. The purpose of this study was to know the relevance of nutritional topics in daily practice and the main nutritional problems perceived by pediatritians in children under 3 years of age. Methods: Descriptive, cross-sectional study performed in 2 stages. First stage consisted on discussion meetings with 30 random-selected pediatritians from Madrid and Barcelona. Results were used to design the on-line questionnaire of the second stage (76 questions related to nutrition in children under 3 years). A random and representative sample of Spanish pediatritians was selected for this stage. Results: One hundred and fifty one pediatritians, among 258 sampled, completed the questionnaire. They referred to see a mean of 588 patients/month. The main perceived nutritional problems in the first year of life were iron and vitamin deficiencies and poor weight gain. In the next months, excess in carbohydrates and lipid intake and overweight were the main problems perceived. Parents were considered the main actors regarding their children's nutritional health, but their concern in these questions significantly reduced with children's age (p < 0,0001). Factors considered to have the greatest relevance in the acquisition of good nutritional habits were to provide a diet adjusted for children's requirements and to observe the appropriate sleeping hours. The proportion of children who receive nutritional counseling varied from 88% (0-6 months) to 61% (24-36 months). Conclusions: Despite of the relevance given by pediatritians, educational intervention regarding nutritional health is not ideal. Nutritional problems perceived by pediatritians varied with children's age (AU)

[Nutritional problems perceived by pediatricians in Spanish children younger than 3 years]. / Problemas nutricionales percibidos por los pediatras en niños Españoles menores de 3 años.

INTRODUCTION: Parents are most responsible for nutritional education of children, and pediatricians must advise and help them with their doubts. The purpose of this study was to know the relevance of nutritional topics in daily practice and the main nutritional problems perceived by pediatricians in children under 3 years of age. METHODS: Descriptive, cross-sectional study performed in 2 stages. First stage consisted on discussion meetings with 30 random-selected pediatricians from Madrid and Barcelona. Results were used to design the on-line questionnaire of the second stage (76 questions related to nutrition in children under 3 years). A random and representative sample of Spanish pediatricians was selected for this stage. RESULTS: One hundred and fifty one pediatricians, among 258 sampled, completed the questionnaire. They referred to see a mean of 588 patients/month. The main perceived nutritional problems in the first year of life were iron and vitamin deficiencies and poor weight gain. In the next months, excess in carbohydrates and lipid intake and overweight were the main problems perceived. Parents were considered the main actors regarding their children's nutritional health, but their concern in these questions significantly reduced with children's age (p < 0,0001). Factors considered to have the greatest relevance in the acquisition of good nutritional habits were to provide a diet adjusted for children's requirements and to observe the appropriate sleeping hours. The proportion of children who receive nutritional counseling varied from 88% (0-6 months) to 61% (24-36 months). CONCLUSIONS: Despite of the relevance given by pediatricians, educational intervention regarding nutritional health is not ideal. Nutritional problems perceived by pediatricians varied with children's age.

Recomendaciones para el tratamiento del estreñimiento funcional / Guidelines for the treatment of functional constipation

El estreñimiento es un problema clínico frecuente. Afecta del 2 al 30% de los niños, según el criterio diagnóstico utilizado. El tratamiento recomendado se basa en: a) explicación-desmitificación; b) desimpactación fecal y c) mantenimiento con cambios en la dieta, modificación de los hábitos higiénicos y el uso de laxantes. En la última década ha variado el sistema de vaciado intestinal; de la clásica vía rectal hemos pasado al uso generalizado de la vía oral a base de polietilenglicol 3350.Se debe tener en cuenta las necesidades individuales y las preferencias del paciente. El tratamiento de mantenimiento se iniciará inmediatamente después de lograr el vaciado intestinal. Está basado en la modificación de los hábitos dietéticos, higiénicos y en el uso de laxantes. Una adecuada ingesta de fibra y agua, el entrenamiento del hábito defecatorio y el uso de laxantes orales logra una defecación diaria y no dolorosa, evitando la reacumulación de heces. El uso de enemas puede ser efectivo para la desimpactación, pero tiene el riesgo de lesión traumática y no es adecuado para el tratamiento de mantenimiento. Los laxantes recomendados son los osmóticos. El tratamiento de primera elección en niños de cualquier edad es el polietilenglicol 3350 por su seguridad, efectividad y tolerancia. Su dosis varia entre 0,25 a 1,5g/kg. El mejor conocimiento de la fisiología de la defecación ha permitido el desarrollo de nuevos fármacos: bloqueantes de los receptores de la serotonina o activadores de los canales del cloro (AU)

Constipation is common in childhood. It can affect around 5–30% of the child population, depending on the criteria used for diagnosis. The currently recommended treatment is based on three main points: a) explanation, b) disimpaction and c) maintenance therapy consisting of diet changes, behavioural modification, and the use of laxatives. In the last decades treatment on disimpaction have changed radically from the rectal route to the oral route with polyethylene glycol 3350 (PEG), the most used and accepted regimen nowadays. Treatment and care should take into account the individual needs and preferences of the patient. Good communication is essential, to allow patients to reach informed decisions about their care. Maintenance therapy consists of dietary interventions, toilet training, and laxatives to obtain daily painless defaecation in order to prevent re-accumulation of stools. Maintenance therapy should be start as soon as the child's bowel is disimpacted. Early intervention with oral laxatives may improve complete resolution of functional constipation. Enemas using phosphate, mineral oil, or normal saline are effective in relieving rectal impaction, but carry the risk of mechanical trauma and are not recommended for maintenance therapy in the paediatric population. Among osmotic agents, polyethylene glycol 3350 plus electrolyte solutions appear to be the first-line drug treatment to use in children of any age, as it is safe, effective, and well-tolerated. Recommended doses ranges from 0.25 to 1.5g/kg. Advances in the understanding of the gastrointestinal enteric nervous system and epithelial function have led to the development of new substances that bind to serotonin receptors or are chloride channel activators (AU)

[Guidelines for the treatment of functional constipation]. / Recomendaciones para el tratamiento del estreñimiento funcional.

Constipation is common in childhood. It can affect around 5-30% of the child population, depending on the criteria used for diagnosis. The currently recommended treatment is based on three main points: a) explanation, b) disimpaction and c) maintenance therapy consisting of diet changes, behavioural modification, and the use of laxatives. In the last decades treatment on disimpaction have changed radically from the rectal route to the oral route with polyethylene glycol 3350 (PEG), the most used and accepted regimen nowadays. Treatment and care should take into account the individual needs and preferences of the patient. Good communication is essential, to allow patients to reach informed decisions about their care. Maintenance therapy consists of dietary interventions, toilet training, and laxatives to obtain daily painless defaecation in order to prevent re-accumulation of stools. Maintenance therapy should be start as soon as the child's bowel is disimpacted. Early intervention with oral laxatives may improve complete resolution of functional constipation. Enemas using phosphate, mineral oil, or normal saline are effective in relieving rectal impaction, but carry the risk of mechanical trauma and are not recommended for maintenance therapy in the paediatric population. Among osmotic agents, polyethylene glycol 3350 plus electrolyte solutions appear to be the first-line drug treatment to use in children of any age, as it is safe, effective, and well-tolerated. Recommended doses ranges from 0.25 to 1.5g/kg. Advances in the understanding of the gastrointestinal enteric nervous system and epithelial function have led to the development of new substances that bind to serotonin receptors or are chloride channel activators.

Manifestaciones cutáneas de la enfermedad inflamatoria intestinal / Skin manifestations of pediatric inflammatory bowel disease

La enfermedad inflamatoria intestinal (enfermedad de Crohn [EC], colitis ulcerosa, colitis indeterminada o enfermedad inflamatoria intestinal no clasificada) se acompaña de manifestaciones cutáneas hasta entre el 10 y el 15% de los casos. Hay pocos datos de prevalencia de éstas en poblaciones pediátricas, si bien algunos estudios recientes calculan que pueden estar presentes hasta en el 8% de los casos en el diagnóstico. El objetivo de este estudio es comunicar diferentes formas de manifestaciones cutáneas observadas en los pacientes pediátricos con enfermedad inflamatoria intestinal. El caso 1 se trata de un eritema nudoso de aparición al comienzo de la EC. Tiene una presentación típica en forma de nódulos eritematosos dolorosos de localización pretibial; su respuesta es buena y desaparece tras el inicio del tratamiento con infliximab. El caso 2 se trata de un caso con coexistencia de pioderma gangrenoso y síndrome de Sweet de afectación mucocutánea en un paciente con EC. La evolución de las lesiones es rápidamente progresiva y se controla con esteroides sistémicos. Se observa la presencia de importantes lesiones cicatriciales residuales. Los casos 3 y 4 se tratan de EC metastásica en glúteo y vulva, respectivamente, con buena respuesta al tratamiento con infliximab (AU)

Skin manifestations have been described in 10–15% of patients with inflammatory bowel disease (Crohn's disease, ulcerative colitis, indeterminate colitis/inflammatory bowel disease type unclassified). There are limited data on the prevalence of these manifestations in paediatric patients, but recent studies have reported its presence in 8% of them at diagnosis. Our aim is to report the different skin manifestations observed in our paediatric patients with inflammatory bowel disease. Case 1: Erythema nodosum at Crohn's disease diagnosis. Typical presentation with painful erythematous nodules in the pretibial region and with good response to infliximab. Case 2: Coexistence of pyoderma gangrenosum and mucocutaneous Sweet's syndrome in a Crohn's disease patient. A rapidly progressive disease that was controlled with systemic steroids but with significant residual lesions. Case 3 and 4: Metastatic Crohn's disease with good response to infliximab (AU)

[Skin manifestations of pediatric inflammatory bowel disease]. / Manifestaciones cutáneas de la enfermedad inflamatoria intestinal.

Skin manifestations have been described in 10-15% of patients with inflammatory bowel disease (Crohn's disease, ulcerative colitis, indeterminate colitis/inflammatory bowel disease type unclassified). There are limited data on the prevalence of these manifestations in paediatric patients, but recent studies have reported its presence in 8% of them at diagnosis. Our aim is to report the different skin manifestations observed in our paediatric patients with inflammatory bowel disease. Case 1: Erythema nodosum at Crohn's disease diagnosis. Typical presentation with painful erythematous nodules in the pretibial region and with good response to infliximab. Case 2: Coexistence of pyoderma gangrenosum and mucocutaneous Sweet's syndrome in a Crohn's disease patient. A rapidly progressive disease that was controlled with systemic steroids but with significant residual lesions. Case 3 and 4: Metastatic Crohn's disease with good response to infliximab.

[Infliximab as rescue therapy in severe steroid-resistant ulcerative colitis]. / Infliximab como terapia de rescate en colitis ulcerosa grave resistente al tratamiento corticoideo.

Paediatric ulcerative colitis tends to be more severe and more extensive than in adults. Steroid-resistance is also more frequent, producing a high colectomy rate in these patients. Cyclosporine A has showed to be an effective rescue therapy in acute attacks, avoiding colectomy. However, the long-term benefits are less promising and there is also very serious toxicity associated with its use. Therefore, novel effective therapies in paediatric ulcerative colitis are mandatory. We present a patient with a severe attack of steroid-resistant ulcerative-colitis in whom infliximab has proved effective in inducing a maintained remission and in avoiding the need for colectomy.

Infliximab como terapia de rescate en colitis ulcerosa grave resistente al tratamiento corticoideo / Infliximab as rescue therapy in severe steroid-resistant ulcerative colitis

La colitis ulcerosa que se inicia en la edad pediátrica adopta formas más extensas y graves que en la edad adulta. Paralelamente, la resistencia al tratamiento esteroide observada en estos casos condiciona la necesidad de realizar una colectomía a corto y medio plazo en un número importante de estos pacientes. La ciclosporina A ha sido utilizada con éxito en situaciones graves de resistencia a los corticoides. Su efectividad a medio y largo plazo es más reducida y presenta, además, una importante toxicidad. Por tanto, el ensayo de nuevas terapias de rescate constituye una necesidad en la colitis ulcerosa pediátrica. Presentamos el caso de una paciente afectada de colitis ulcerosa resistente a corticoides en quien el tratamiento con infliximab ha sido efectivo para obtener una remisión clínica prolongada, lo que ha evitado la necesidad de realizar una colectomía (AU)

Paediatric ulcerative colitis tends to be more severe and more extensive than in adults. Steroid-resistance is also more frequent, producing a high colectomy rate in these patients. Cyclosporine A has showed to be an effective rescue therapy in acute attacks, avoiding colectomy. However, the long-term benefits are less promising and there is also very serious toxicity associated with its use. Therefore, novel effective therapies in paediatric ulcerative colitis are mandatory. We present a patient with a severe attack of steroid-resistant ulcerative-colitis in whom infliximab has proved effective in inducing a maintained remission and in avoiding the need for colectomy (AU)

Vasculitis cerebral asociada a colitis ulcerosa / Cerebral vasculitis associated with ulcerative colitis

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[Autoimmune hepatitis in pediatric patients]. / Hepatitis autoinmune en la edad pediátrica.

BACKGROUND: Autoimmune hepatitis (AIH) is an inflammatory disease of unknown origin that is responsible for progressive liver necrosis and ultimately cirrhosis. OBJECTIVE: Our aim was to evaluate the characteristics of autoimmune hepatitis presenting in the pediatric age. MATERIAL AND METHODS: We conducted a retrospective study of all patients diagnosed with AIH in our hospital department during the last 10 years. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology, treatment, need for transplant, and clinical evolution. According to the positive level of auto-antibodies, AIH patients were classified as type I AIH (ANA and/or smooth-muscle antibodies) and type II (anti-LKM-1). RESULTS: Seven patients were diagnosed in this period -5 girls (71.5%) and 2 boys (28.5%). Five patients presented with type-I serological markers, and two with type-II markers. Age range at diagnosis was from 21 months to 12 years. In the type-I group, 3 patients presented with acute hepatitis while 2 other patients were diagnosed from laboratory findings while asymptomatic. Elevated aminotransferase (10 times the normal level) was observed in 71.5%, and 85% had elevated immunoglobulins. Treatment with azathioprine and prednisone was started after diagnosis with an average time to remission of 14 months. Two patients relapsed following steroid withdrawal. CONCLUSION: AIH can have different forms of clinical presentation, and is sometimes indistinguishable from viral hepatitis. AIH must be ruled out in patients presenting with concomitant elevation of aminotransferases and immunoglobulins. The commonly accepted treatment is a combination of azathioprine and corticosteroids. A high percentage of patients experience a relapse of disease after steroids are withdrawn. Therefore, some patients will need to stay on combined therapy with minimal doses of steroids.