Eosinophilic esophagitis that develops during therapy with proton pump inhibitors : case series and possible mechanisms.

Therapy with proton-pump inhibitors (PPIs) results in remission in at least one third of patients with esophageal eosinophilia, presumably because of both their acid-related and anti-inflammatory mechanisms of action. However, eosinophilic esophagitis (EoE) may also develop during therapy with PPIs. We present a case series of four children who were initially diagnosed with infectious esophagitis, gastroesophageal reflux disease or gastric ulcer, who had no eosinophilic infiltration of the esophagus, but subsequently developed symptoms, endoscopic features and histological picture of typical EoE. We discuss mechanisms of action of PPIs of likely relevance to an increased risk of development of EoE in some patients, such as their influence on mucosal barrier function, interference with pH-related protein digestion by pepsin, and antigen processing by immune cells.

Consensus guidelines of ECCO/ESPGHAN on the medical management of pediatric Crohn's disease.

Children and adolescents with Crohn's disease (CD) present often with a more complicated disease course compared to adult patients. In addition, the potential impact of CD on growth, pubertal and emotional development of patients underlines the need for a specific management strategy of pediatric-onset CD. To develop the first evidenced based and consensus driven guidelines for pediatric-onset CD an expert panel of 33 IBD specialists was formed after an open call within the European Crohn's and Colitis Organisation and the European Society of Pediatric Gastroenterolog, Hepatology and Nutrition. The aim was to base on a thorough review of existing evidence a state of the art guidance on the medical treatment and long term management of children and adolescents with CD, with individualized treatment algorithms based on a benefit-risk analysis according to different clinical scenarios. In children and adolescents who did not have finished their growth, exclusive enteral nutrition (EEN) is the induction therapy of first choice due to its excellent safety profile, preferable over corticosteroids, which are equipotential to induce remission. The majority of patients with pediatric-onset CD require immunomodulator based maintenance therapy. The experts discuss several factors potentially predictive for poor disease outcome (such as severe perianal fistulizing disease, severe stricturing/penetrating disease, severe growth retardation, panenteric disease, persistent severe disease despite adequate induction therapy), which may incite to an anti-TNF-based top down approach. These guidelines are intended to give practical (whenever possible evidence-based) answers to (pediatric) gastroenterologists who take care of children and adolescents with CD; they are not meant to be a rule or legal standard, since many different clinical scenario exist requiring treatment strategies not covered by or different from these guidelines.

Miotomía de Heller laparoscópica: resultados en niños / Outcome of laparoscopic heller myotomy in children

Objetivos. El presente estudio tuvo como objetivo evaluar los resultados a corto y medio plazo de la miotomía de Heller laparoscópica. Métodos. Fueron incluidos niños sometidos a cirugía desde 2002 hasta 2010. La cirugía fue propuesta tras el fracaso de las dilataciones endoscópicas. Todos los pacientes fueron sometidos a esofagocardiomiotomía (Heller) con funduplicatura anterior (Dor) por laparoscopia. La demografía, síntomas de presentación y detalles perioperatorios fueron analizados. Los resultados fueron evaluados por los registros médicos y la aplicación de un cuestionario de auto-evaluación (GIQLI modificado), cubriendo síntomas gastrointestinales superiores) con 14 preguntas cada una puntuada de 0 (peor) hasta 4 (mejor o normal).Resultados. Seis pacientes (5 varones) con una edad media de 12,2 años (rango: 0,8-14,2) fueron sometidos a cirugía. Los síntomas de presentación fueron: disfagia (83,3%), pérdida de peso (50%), vómitos (33,3%), tos (33,3%) y dolor torácico (16,7%). Todos los pacientes fueron operados por laparoscopia sin conversiones y no hubo complicaciones intra, ni postoperatorias. Con una mediana de seguimiento de 5 años (rango: 2-10), ninguno de los pacientes fue re-operado. Cinco pacientes tienen hábitos alimentarios normales; el caso restante presentó episodios recurrentes de disfagia, requiriendo dilataciones endoscópicas periódicas (cada 6 meses). El GIQLI total presentó una media de 49,3 puntos (rango, 45-52), lo que representa 88,1% del máximo posible. La puntuación para la frecuencia de episodios de disfagia fue 1,6 ± 1,4; la puntuación para el grado de disfagia fue 3 en todos los pacientes. Conclusiones. La miotomía de Heller laparoscópica es efectiva y segura en niños, ofreciendo una calidad de vida buena y duradera; a pesar de frecuentes, los episodios de disfagia son ligeros (AU)

Aim. The appropriate management of achalasia in children remains debatable. The present study aimed to evaluate the outcome of laparoscopic Heller myotomy by assessing short- and mid-term issues. Methods. Children submitted to surgery from 2002 to 2010 were included. Surgery was proposed after failure of endoscopic dilatations. All patients underwent esophagocardiomyotomy (Heller) plus anterior fundoplication (Dor) by laparoscopy. Demographics, presentation symptoms and perioperative details were analyzed. The outcomes were assessed both by medical records and the application of a 14 items (score 0-worst to 4-best/normal) self-evaluation questionnaire (modified GIQLI - covering only upper gastrointestinal symptoms).Results. Six patients (5 males) with a median age of 12.2 years (range: 0.8-14.2) were submitted to surgery. The presenting symptoms were: dysphagia (83.3%), weight loss (50%), vomiting (33.3%), chronic cough (33.3%), and chest pain (16.7%). All patients were operated on by laparoscopy with no conversions; there were no intra or postoperative complications. At a median follow-up of 5 years (range: 2-10) none of the patients were re-operated. Five patients have normal eating habits; the remaining case presented recurrent episodes of dysphagia requiring regular endoscopic dilatations (every 6 months). The total GIQLI presented a mean score of 49.3 (range, 45-52) representing 88.1% of the theoretical maximum. The score for frequency of dysphagia episodes was 1.6 ± 1.4; all patients scored 3 for the grade of dysphagia. Conclusions. Laparoscopic Heller myotomy is effective and safe for achalasia in children, offering a good and durable quality of life; although frequent, the dysphagia episodes were mild (AU)

Hemangioma reticular do membro inferior associado a anomalias estruturais congénitas: abordagem terapêutica / Reticular hemangioma of the lower extremity associated with congenital structural anomalies: therapeutic approach

O hemangioma reticular é uma variante do hemangioma infantil descrita recentemente, que apresenta uma predilecção pela extremidade inferior epelo períneo. Associa-se, frequentemente, a ulceração recalcitrante, anomalias ano-génito-urinárias e sacrais e, raramente, a sobrecarga cardíaca.Uma recém-nascida prematura apresentava uma mancha com aspecto reticular ao nível do membro inferior esquerdo, nádegas e períneo, dilatações venosas proeminentes na superfície lateral do membro envolvido, genitália externa dismórfica e ânus imperfurado. A ressonância magnética mostrouimperfuração anal, fístula rectovestibular, hipoplasia coccígea, cavidade siringomiélica medular e envolvimento hemangiomatoso retroperitoneal. As características clínicas e imagiológicas eram consistentes com o diagnóstico de hemangioma reticular com anomalias ventro-caudais. Os autores descrevema abordagem terapêutica adoptada nesta doente e a eficácia do encerramento assistido por vácuo de uma úlcera extensa e refractária aos cuidados de penso convencionais (AU)

Reticular hemangioma is a recently described variant of infantile hemangioma that has a predilection for the lower extremity and perineum. It is often associated with recalcitrant ulceration, ano-genito-urinary-sacral anomalies, and rarely with cardiac overload. A premature newborn female presented with patchy, network-like cutaneous staining of the left lower limb, buttocks and perineum, prominent lateral venous dilations of the involved limb,dysmorphic external genitalia and imperforate anus. MRI revealed imperforate anus, rectovestibular fistula, coccygeal hypoplasia, medullary syringomyelic cavity and retroperitoneal hemangiomatous involvement. The clinical and imaging features were those of reticular hemangioma and ventral-caudal anomalies. The authors describe the therapeutic approach of this patient and the efficacy of vacuum-assisted closure of a large intractable ulceration (AU)

[Outcome of laparoscopic Heller myotomy in children]. / Miotomía de Heller laparoscópica: resultados en niños.

AIM: The appropriate management of achalasia in children remains debatable. The present study aimed to evaluate the outcome of laparoscopic Heller myotomy by assessing short- and mid-term issues. METHODS: Children submitted to surgery from 2002 to 2010 were included. Surgery was proposed after failure of endoscopic dilatations. All patients underwent esophagocardiomyotomy (Heller) plus anterior fundoplication (Dor) by laparoscopy. Demographics, presentation symptoms and perioperative details were analyzed. The outcomes were assessed both by medical records and the application of a 14 items (score 0-worst to 4-best/normal) self-evaluation questionnaire (modified GIQLI - covering only upper gastrointestinal symptoms). RESULTS: Six patients (5 males) with a median age of 12.2 years (range: 0.8-14.2) were submitted to surgery. The presenting symptoms were: dysphagia (83.3%), weight loss (50%), vomiting (33.3%), chronic cough (33.3%), and chest pain (16.7%). All patients were operated on by laparoscopy with no conversions; there were no intra or postoperative complications. At a median follow-up of 5 years (range: 2-10) none of the patients were re-operated. Five patients have normal eating habits; the remaining case presented recurrent episodes of dysphagia requiring regular endoscopic dilatations (every 6 months). The total GIQLI presented a mean score of 49.3 (range, 45-52) representing 88.1% of the theoretical maximum. The score for frequency of dysphagia episodes was 1.6 +/- 1.4; all patients scored 3 for the grade of dysphagia. CONCLUSIONS: Laparoscopic Heller myotomy is effective and safe for achalasia in children, offering a good and durable quality of life; although frequent, the dysphagia episodes were mild.

Rapid test for fecal calprotectin levels in children with Crohn disease.

Assessment of fecal calprotectin, a surrogate marker of mucosal inflammation, is a promising means to monitor therapeutic response in pediatric inflammatory bowel disease, especially if the result is readily available. We tested the performance of a novel calprotectin rapid test, Quantum Blue, versus the conventional enzyme-linked immunosorbent assay in 134 stool samples from 56 pediatric patients with Crohn disease. The intraclass correlation coefficient analysis reflected good agreement (intraclass correlation coefficient 0.97 [95% confidence interval 0.95-0.98]) but agreement was better in lower values, where dilutions were not required. Using a cutoff of 100 µg/g for normal values, the percentage agreement between the 2 tests was 87%. The optimal cutoff values to guide clinical decisions in the therapy of inflammatory bowel disease have yet to be determined.

Does umbilical vein catheterization to exchange transfusion lead to portal vein thrombosis?

UNLABELLED: The aetiopathology of extrahepatic portal vein obstruction is unknown. In retrospective studies, umbilical vein cannulation and sepsis have been alleged to cause portal thrombosis. This prospective study was undertaken to detect whether thrombosis and consequent obstruction of the splenoportal venous system develops after umbilical vein catheterization for exchange transfusion in newborns using Doppler ultrasound. Forty children (M = 24; F = 16) who had undergone exchange transfusion for hyperbilirubinaemia were studied at school age. Maximal duration of the venous umbilical cannulation was 120 min and sepsis did not occur. Clinical, biological and sonographic examinations were normal, except in 3 children. In 2 the left branch of portal vein could not be identified (normal variant). CONCLUSION: Our results show that, in these children, umbilical vein catheterization did not lead to development of portal vein thrombosis. However, when other risk factors such as umbilical infection, traumatic catheterization are associated, children should be screened for obstruction of the portal vein.

Analysis of the allelic diversity of a (CA)n repeat polymorphism among alpha 1-antitrypsin gene products from northern Portugal.

The level of molecular heterogeneity associated with alpha 1-antitrypsin gene products was assessed in the population of northern Portugal using three restriction fragment length polymorphisms (RFLPs) corresponding to specific amino acid substitutions and a highly variable (CA)n repeat polymorphism located at the 5' end of the PI gene. The allelic affinities inferred from the analysis of the DNA polymorphisms essentially agree with the evolutionary pattern proposed for the PI gene products on the basis of their amino acid sequences. PI*Z can be considered the most recent common PI allele and was found to be associated with the same predominant haplotype previously reported in northern European populations, thus confirming the hypothesis that most European Z alleles are derived from a single mutation. However, a rare deficient variant that is the likely result of a recurrent Z mutation on an M2 or M4 background was additionally observed. PIS was also found to be associated with a strongly predominant haplotype and seems to be the second most recent PI common allele, while M2 and M3 show weaker associations, suggesting more ancient origins of their corresponding mutations. M1Ala213 and M1Vat213 display more homogeneous (CA)n allele frequency distributions, M1Ala213 representing the most ancient PI allele as inferred from its highest variance in (CA)n allele length.