[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.

[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.

[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.

[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.

Concordance between breath test and histologic damage in Helicobacter pylori associated infections in infancy.

OBJECTIVE: the purpose of this study was to investigate the concordance between urea breath test values and the histological severity of gastric mucosa lesions in children. METHOD: forty children ranging in age from 3 to 17 years were examined endoscopically because of abdominal pain and positive breath test results. Histological status was determined by the intensity of the inflammatory infiltrate and by the depth of the damage. Histologically, four groups were considered: normal-appearing gastric mucosa (group 0), mild antrum gastritis (group 1), moderate antrum gastritis (group 2), and severe antrum gastritis (group 3). RESULTS: normal-appearing gastric mucosa was observed in 8 children with a mean breath test value of 44.07; mild chronic gastritis was observed in 17 children with a mean breath test value of 36.15; moderate gastritis was demonstrated in 10 children with a mean breath test value of 48.50, and severe gastritis was observed in 5 children with a mean breath test value of 52.31. CONCLUSION: we conclude that there is no concordance between urea breath test values and histological severity of gastric mucosa lesions in children.

Role of manometry, defecography and anal endosonography in the evaluation of colorectal disorders.

OBJECTIVE: altered motility or anatomy of the rectum, anus and perineal floor may lead to symptoms which are unresponsive to routine therapeutic approaches. These disturbances usually lead to constipation, fecal incontinence, or both. Different tests and techniques for evaluating anorectal and perineal disorders, developed in the last two decades, make a better understanding of these disorders possible. This study was designed to evaluate the diagnostic benefits of combining manometry, defecography and anal endosonography in the assessment of patients with anorectal disorders. METHODS: twenty-five children with constipation (with or without soiling), incontinence and/or prolapse underwent anal manometry, defecography and anal endosonography. Group A consisted of 9 children with fecal incontinence, group B consisted of 10 children with constipation with soiling, and group C comprised 6 children with constipation without soiling. RESULTS: in group A resting incontinence was associated with a hypotonic external sphincter in 4 out of 9 patients, 2 of whom had internal anal sphincter thinning. In group B resting incontinence was associated with a hypotonic external sphincter in 8 out of 10 patients, 6 of whom had internal anal sphincter thinning. In group C these associations were not seen in any of the patients. CONCLUSIONS: barium enema is not sufficient for an accurate diagnosis of anorectal disorders. No single test is capable of revealing the type of disease. Anal manometry, defecography and endosonography are complementary procedures in the assessment of this group of disorders. This new approach will improve our knowledge of the pathogenesis of these disorders in children. However, further studies are needed to obtain conclusive evidence.

[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.

[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.

[Dental plaque in Helicobacter pylori infection]. / Estudio de la placa dental en la infección por Helicobacter pylori.

OBJECTIVE: The aim of this study was to determine the role of Helicobacter pylori infection in dental plaque from 53 children, between 3 and 17 years of age, with recurrent abdominal pain and/or upper gastrointestinal bleeding. PATIENTS AND METHODS: Dental plaque was analyzed by using polymerase chain reaction (PCR) for a specific internal urease gene (as described by Clayton). Esophago-gastro-duodenoscopy was performed after the dental plaque was obtained and biopsies were taken from the gastric antrium and fundus and analyzed by PCR. An individual interview was performed to know the customs and attitudes about bucodental hygiene. RESULTS: None of the children were positive with the PCR test in dental plaque, while 35 children (66%) had a positive gastric PCR result. CONCLUSIONS: This study does not confirm the hypothesis that dental plaque might act as a reservoir for this microorganism.

[Solitary ulcer of the rectum. Apropos of a case in childhood and review of bibliography]. / Ulcera solitaria de recto. A propósito de un caso en edad pediátrica y revisión de la bibliografía.

A case of a chronically constipated 10 year-old-girl, who had severe rectal bleeding associated with her monthly bowel movement is presented. Barium enema revealed an irregular and rigid area in rectal and sigmoid wall. Endoscopy showed an irregular erosion of 4 by 2 cm, from which biopsies were taken. These were reported as compatible with solitary ulcer of rectum. Patient was treated with dietetic management and clinically and psychiatrically controlled. Two years after initial admission she had no clinical or endoscopic evidence of disease.

[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.