Graft dilatation and Barrett's esophagus in adults after gastric pull-up and jejunal interposition for long-gap esophageal atresia.

BACKGROUND: Esophageal replacement (ER) with gastric pull-up (GPU) or jejunal interposition (JI) used to be the standard treatment for long-gap esophageal atresia (LGEA). Changes of the ER grafts on a macro- and microscopic level however, are unknown. AIM: To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA. METHODS: A cohort study was conducted including all LGEA patients ≥ 16 years who had undergone GPU or JI between 1985-2003 at two tertiary referral centers in the Netherlands. Patients underwent clinical assessment, contrast study and endoscopy with biopsy. Data was collected prospectively. Group differences between JI and GPU patients, and associations between different outcome measures were assessed using the Fisher's exact test for bivariate variables and the Mann-Whitney U-test for continuous variables. Differences with a P-value < 0.05 were considered statistically significant. RESULTS: Nine GPU patients and eleven JI patients were included. Median age at follow-up was 21.5 years and 24.4 years, respectively. Reflux was reported in six GPU patients (67%) vs four JI patients (36%) (P = 0.37). Dysphagia symptoms were reported in 64% of JI patients, compared to 22% of GPU patients (P = 0.09). Contrast studies showed dilatation of the jejunal graft in six patients (55%) and graft lengthening in four of these six patients. Endoscopy revealed columnar-lined esophagus in three GPU patients (33%) and intestinal metaplasia was histologically confirmed in two patients (22%). No association was found between reflux symptoms and macroscopic anomalies or intestinal metaplasia. Three GPU patients (33%) experienced severe feeding problems vs none in the JI group. The median body mass index of JI patients was 20.9 kg/m2 vs 19.5 kg/m2 in GPU patients (P = 0.08). CONCLUSION: The majority of GPU patients had reflux and intestinal metaplasia in 22%. The majority of JI patients had dysphagia and a dilated graft. Follow-up after ER for LGEA is essential.

Hypermethylation of CTDSPL2 prior to necrotizing enterocolitis onset.

Background: Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency in preterm infants. Epigenetic changes in DNA methylation may be present prior to NEC onset. Methods: 24 preterm infants with NEC and 45 matched controls were included. Human DNA was isolated from stool samples and methylation of CTDSPL2, HERC1, NXPE3 and PTGDR was measured using pyrosequencing. Results: CTDSPL2 displayed a higher DNA methylation of 51% compared with 17% in controls, prior to NEC onset (p = 0.047). Discussion: Noninvasive measurement of methylation in stool allows for comparison with healthy preterm controls. This potentially allows future biomarker or risk predictor use. The effect of CTDSPL2 hypermethylation on gene expression remains unclear.

What is this article about? Necrotizing enterocolitis (NEC) is a common emergency condition affecting the gastrointestinal system of preterm infants. Epigenetic changes in DNA methylation may be present in infants before the onset of NEC. DNA methylation is a natural process that can help turn genes on or off, thereby affecting their function. This study focused on measuring the amount of DNA methylation in certain genes in preterm infants who developed NEC. What were the results? This study included 24 preterm infants with NEC and 45 matched healthy controls. The researchers isolated human DNA from stool samples, and the amount of DNA methylation of four specific genes was measured. They found that one of the genes, CTDSPL2, had significantly higher DNA methylation in infants who later developed NEC than in healthy infants. What do the results of the study mean? In this study, researchers found that CTDSPL2 showed a higher level of DNA methylation in stool samples of infants who later developed NEC. The effect of this change remains unclear, but may affect the way cells grow and respond to injury or infection, which could contribute to the development of NEC. Measuring DNA methylation in stool samples provides a noninvasive method for identifying DNA methylation changes in preterm infants. Comparing the amount of DNA methylation in healthy infants with that in preterm infants at risk of NEC may help predict the risk of developing NEC.

Centralization of pediatric surgical care in the Netherlands: Lessons learned.

Centralization of care is a difficult process, as there are several stakeholders that are involved and should be heard. What can be the best option for a small group of patients may be detrimental to a larger group of patients that cannot be adequately treated close to home. The weighing of these factors is different in every environment. One universal rule however is: if you don't do it yourselves, others will do it for you. In the Netherlands, pediatric oncology, including surgery, is centralized in one center (Utrecht) with the help of several shared care centers scattered throughout the country for things that can be managed close to home.

Jejunoileal atresia and cystic fibrosis: don't miss it.

BACKGROUND: While an increased prevalence of cystic fibrosis (CF) in patients with jejunal atresia and ileal atresia (JIA) has been described previously, it still may not be a practice routine to indicate a sweat test or DNA test for CFTR mutations in newborns presenting with JIA. Leading textbooks do not mention JIA as a possible presenting clinical feature of CF. We describe two cases of JIA with a delayed diagnosis of CF (4 months [post mortem] and 19 months). This led to a retrospective review of all patients with JIA in our hospital. We hypothesised that also in the past although indicated further testing for CF had not always been performed. METHODS: Over an 18-year period from January 1991 until December 2008, all cases of JIA in our centre were reviewed (n=50). We compared patients who have been tested for CF (n=18) with patients who have not been tested for CF (n=32), with respect to their patient characteristics, either by logistic regression analysis or a nonparametric test (p<0.05). RESULTS: Of all 50 patients the proportion of infants actually tested for CF was 18 (36%). A statistical significant difference between the group of patients who were tested for CF versus the group of those who were not tested was found in a higher occurrence of postoperative bilious retention after 7 days (56% versus 25%, respectively), and postoperative complications (78% versus 34%, respectively). CF was confirmed in 4 (8%). CONCLUSION: Testing for CF in newborns presenting with JIA does not appear to be common practice. A timely diagnosis of CF leads to presymptomatic treatment and has beneficial effects on morbidity and mortality. CF should be tested for in all children with JIA. We recommend a sweat test for term children and CFTR DNA testing as a first step for preterm infants. Medical professional awareness may be increased if future editions of leading text books in the relevant fields should include JIA as an indication to follow an appropriate CF-diagnostic algorithm. TRIAL REGISTRATION: Statement on reporting of a clinical trial: This article is not based on a clinical trial.