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)

Edemas como presentación de enteropatía pierde-proteínas / Edema as the form of presentation of protein-losing enteropathy

No disponible

[Increasing diagnosis of eosinophilic esophagitis in Spain]. / Aumento del diagnóstico de esofagitis eosinofílica en nuestro medio.

INTRODUCTION: Eosinophilic esophagitis is a chronic inflammation of the esophagus characterized by marked eosinophilic infiltration. It is frequently associated with other allergic diseases. In the last few years, the incidence of eosinophilic infiltration has shown a striking increase in Spain, partly due to better knowledge of the disease and to the correct diagnosis of patients previously thought to be suffering from gastroesophageal reflux. MATERIAL AND METHODS: To report our experience in the diagnosis of eosinophilic esophagitis in the last few years and compare our data with previously published findings and current knowledge of the subject. RESULTS: From January 1997 to November 2003, 11 patients (nine boys and two girls) were diagnosed with eosinophilic esophagitis. The mean age at diagnosis was 9 years and 7 months. The most frequent symptoms were dysphagia with solids and food impaction. Seven patients had a familial history of allergy. Allergic tests were positive in eight patients; five had food allergy, five were positive for aeroallergens and two patients had mixed allergy. Endoscopy showed esophageal trachealization in two patients and papular whitish exudate in a further two; both these findings are characteristic of eosinophilic esophagitis. Endoscopic appearance was normal in seven patients. Esophageal biopsies showed > 20 eosinophils/hpf. Five patients had eosinophilic infiltration in other parts of the digestive tract. All the patients with food allergy were put on exclusion diets. Three patients received systemic steroids and cromolyn sodium and three received montelukast, with good response in all patients. CONCLUSIONS: The incidence of eosinophilic esophagitis is increasing. This disease should be considered in patients with longstanding symptoms presumed to be caused by gastroesophageal reflux or motility disorders with poor response to standard therapy. Eosinophilic esophagitis is frequently associated with allergy. A normal appearance of esophageal mucosa on endoscopy should not prevent the clinician from taking multiple biopsies. Patients with eosinophilic esophagitis show good response to anti-allergic treatment.

Aumento del diagnóstico de esofagitis eosinofílica en nuestro medio / Increasing diagnosis of eosinophilic esophagitis in Spain

Introducción: La esofagitis eosinofílica es una inflamación crónica del esófago con alto grado de infiltración eosinófila. Relacionada con otros procesos alérgicos, su prevalencia está aumentando llamativamente en los últimos años en nuestro medio, lo cual, con toda probabilidad se ha visto beneficiado por un mejor diagnóstico de cuadros clínicos antes clasificados como reflujo gastroesofágico (RGE). Material y métodos: Recoger nuestra experiencia en el diagnóstico de esofagitis eosinofílica en los últimos años comparando nuestros hallazgos con los datos publicados y los conocimientos actuales sobre el tema. Resultados: Desde enero de 1997 hasta noviembre de 2003, 11 pacientes (9 varones, 2 mujeres) fueron diagnosticados de esofagitis eosinofílica. La edad media al diagnóstico fue de 9 años y 7 meses. Los síntomas más frecuentes eran disfagia para sólidos e impactación de alimentos. Siete pacientes tenían antecedentes alérgicos. Las pruebas alérgicas presentaban positividad en 8 pacientes; cinco alergia alimentaria, cinco frente a neumoalérgenos y dos mixta. Endoscópicamente se observó traquealización esofágica en 2 pacientes y exudado papular blanquecino en otros dos, siendo ambos hallazgos bastante característicos. El aspecto endoscópico fue normal en 7 pacientes. El estudio anatomopatológico mostraba un recuento de eosinófilos superior a 20 por campo de gran aumento. Cinco pacientes presentaban infiltración eosinofílica en otras partes del tubo digestivo. Se instauró dieta de exclusión en los pacientes con alergia alimentaria. Tres pacientes recibieron esteroides sistémicos y cromoglicato, y tres fueron tratados con montelukast, con buena respuesta. Conclusiones: La esofagitis eosinofílica es una entidad en aumento que debe entrar en el diagnóstico diferencial de los pacientes con síntomas indicativos de RGE o trastornos motores esofágicos con mala respuesta a tratamientos convencionales. Con frecuencia aparece en pacientes con antecedentes alérgicos. La normalidad endoscópica no debe evitar la toma de múltiples biopsias esofágicas ante la sospecha clínica. Los pacientes muestran buena respuesta a tratamientos antialérgicos (AU)

Introduction: Eosinophilic esophagitis is a chronic inflammation of the esophagus characterized by marked eosinophilic infiltration. It is frequently associated with other allergic diseases. In the last few years, the incidence of eosinophilic infiltration has shown a striking increase in Spain, partly due to better knowledge of the disease and to the correct diagnosis of patients previously thought to be suffering from gastroesophageal reflux. Material and methods: To report our experience in the diagnosis of eosinophilic esophagitis in the last few years and compare our data with previously published findings and current knowledge of the subject. Results: From January 1997 to November 2003, 11 patients (nine boys and two girls) were diagnosed with eosinophilic esophagitis. The mean age at diagnosis was 9 years and 7 months. The most frequent symptoms were dysphagia with solids and food impaction. Seven patients had a familial history of allergy. Allergic tests were positive in eight patients; five had food allergy, five were positive for aeroallergens and two patients had mixed allergy. Endoscopy showed esophageal trachealization in two patients and papular whitish exudate in a further two; both these findings are characteristic of eosinophilic esophagitis. Endoscopic appearance was normal in seven patients. Esophageal biopsies showed > 20 eosinophils/hpf. Five patients had eosinophilic infiltration in other parts of the digestive tract. All the patients with food allergy were put on exclusion diets. Three patients received systemic steroids and cromolyn sodium and three received montelukast, with good response in all patients. Conclusions: The incidence of eosinophilic esophagitis is increasing. This disease should be considered in patients with longstanding symptoms presumed to be caused by gastroesophageal reflux or motility disorders with poor response to standard therapy. Eosinophilic esophagitis is frequently associated with allergy. A normal appearance of esophageal mucosa on endoscopy should not prevent the clinician from taking multiple biopsies. Patients with eosinophilic esophagitis show good response to anti-allergic treatment (AU)

[Doxycycline-induced esophagitis: report of two cases]. / Esofagitis por doxiciclina. A propósito de dos casos.

Drug-induced esophagitis has frequently been reported in adults, with more than 100 substances involved. However, only a few cases occurring in the pediatric population have been published. The most frequently involved drugs are antibiotics and the incidence of esophagitis is thought to be greater than estimated due to the increase in antibiotic prescription in the last few decades. A medical history suggestive of retrosternal pain, odynophagia with or without dysphagia, and recent drug intake are suspicious for this entity. Although most cases are self-limiting, complications such as hemorrhage or perforation have been described. We present two cases of doxycycline-induced esophagitis in two teenagers that illustrate the clinical course, endoscopic pattern and treatment of this entity.

Esofagitis por doxiciclina. A propósito de dos casos / Doxycycline-induced esophagitis: report of two cases

La esofagitis por fármacos es una entidad descrita con relativa frecuencia en adultos y en la que se han implicado alrededor de 100 sustancias. Sin embargo, son escasas las publicaciones que hacen referencia a casos en la población pediátrica. Los fármacos más asociados son los antibióticos y se cree que la incidencia es mayor a la estimada dado el aumento de prescripción de éstos en las últimas décadas. La historia clínica indicativa de dolor retroesternal, odinofagia con o sin disfagia y el antecedente de ingesta de fármaco en un paciente debe hacer pensar en esta entidad. La mayoría de los casos son autolimitados, pero no hay que olvidar que pueden asociarse a complicaciones como hemorragias o perforaciones. Los 2 casos que se presenta ilustran la sintomatología, los hallazgos endoscópicos y el tratamiento en esofagitis por doxiciclina en dos adolescentes

Drug-induced esophagitis has frequently been reported in adults, with more than 100 substances involved. However, only a few cases occurring in the pediatric population have been published. The most frequently involved drugs are antibiotics and the incidence of esophagitis is thought to be greater than estimated due to the increase in antibiotic prescription in the last few decades. A medical history suggestive of retrosternal pain, odynophagia with or without dysphagia, and recent drug intake are suspicious for this entity. Although most cases are self-limiting, complications such as hemorrhage or perforation have been described. We present two cases of doxycycline–induced esophagitis in two teenagers that illustrate the clinical course, endoscopic pattern and treatment of this entity

[The role of IgA and IgA antigliadin antibodies in the diagnosis and management of celiac disease]. / Importancia de los anticuerpos antigliadina de tipo IgA e IgG en el diagnóstico y seguimiento de la E. celíaca.

The authors reports the results of ELISA IgG and IgA antigliadin antibodies measurements in a study of 271 serum samples proceeding from celiac patients (with and without gluten containing diet) and control subjects. IgA antigliadin antibody measurement had the most specificity and positive predictive value, IgG antigliadin antibody measurement had the most sensitivity and negative predictive value. Our results point out that antigliadin antibodies are helpful in the diagnosis and management of celiac disease. Un the same manner, antigliadin antibodies are helpful to evaluate the adherence of patients to gluten-free diet.

[Sudden infant death syndrome (SIDS): identification of infants at risk and their home monitoring. Apropos of 25 cases]. / El síndrome de la muerte súbita del lactante (SMSL): identificación de lactantes de riesgo y su monitorización domiciliaria. A propósito de 25 casos.

The SIDS problem is an important medicosocial question therefore it is necessary to identify the high risk infants in order to try to avoid it. We discuss our two years experience in a Home Apnea Monitoring Program. This article includes the first 25 children on home monitoring: 13 infants. 4 preterms and 8 SIDS siblings. We explain the apnea presentation form, the differential diagnosis methods and the diagnosis protocol for the different high risk groups. We speak about the different information obtained through the Pneumocardiogram (PNG), pH metric, the polysomnogram (PSG).... and the indications for Home Apnea Monitoring and the family role in this program. At the end we indicate that it is necessary to have more information and investigation about SIDS in our country.