As a modality for early enteral nutrition, breast milk is protective against the development of PNALD in infants receiving PN for >4 weeks.

Objectives Gastric infection with Helicobacter pylori (H. pylori), a strong risk factor for gastric cancer, is highly prevalent in children residing in the Colombian Andes. We aimed to validate the use of the Entero-test to culture and genotype H. pylori strains from asymptomatic Colombian children. Methods Children (age 10–15y, n=110, 80 of which were H. pylori-positive by the urea breath test, or UBT) were subjected to the Entero-test, and strings were cultured and/or used for DNA extraction for PCR. These children had been treated for H. pylori in 2007. A second population of children (age 10–15y, n= 95), who had not been previously treated were also subjected to the Entero-test. Results Of UBT+ children in the treated group, 29/80 (36%) Entero-test samples were H. pylori culture positive; 29 additional string extracts were tested by PCR for the H. pylori virulence factors cagA and vacA. PCR from cultures and extracts yielded a Sensitivity of 74% and Specificity of 87%. In the untreated group, 16 of 94 UBT+ children (17%) produced Entero-tests that were culture positive. Fifty-eight of 94 (62%) string extracts were PCR positive for cagA and/or vacA. In previously treated children, H. pylori strains were more often the less virulent vacA s2 (P=0.001), m2 (P=0.006), and i2 genotypes (P=0.039). Conclusions The Entero-test may be used as a non-invasive test to detect H. pylori in asymptomatic children residing in high risk areas for gastric cancer. Treatment of H. pylori in children was associated with less virulent genotypes.

Background: Wilson disease (WD) and glucose transporter type 1 (GLUT1) deficiency syndrome are two syndromes with different modes of inheritance but share certain similarities on neurological presentation. To date we have not found previous reports of an association between these two disorders.Case Presentation: Here we describe a 9-year-old male with global developmental delay that presented with intermittent and sudden onset weakness that first occurred at age 3. He was diagnosed with a mutation in the SLC2A1 (Solute Carrier Family 2 Member 1) gene, which results in GLUT1 deficiency. A ketogenic diet could not be started because of unexplained elevated liver enzymes. Due to his liver enzymes' persistent elevation, further investigations demonstrated mildly decreased ceruloplasmin levels, high basal 24-h urinary copper excretion, and an elevated hepatic parenchymal copper concentration on liver biopsy, consistent with WD. Genetic testing revealed two separate mutations in the ATP7B (ATPase Copper Transporting Beta) gene, consistent with WD. The patient was treated with a low copper diet, zinc acetate, and trientine hydrochloride. When liver enzymes normalized, he was subsequently started on a ketogenic diet with improvement in neurological symptoms. His neurological symptoms were most likely secondary to GLUT1 deficiency syndrome, as WD's neurological symptoms are primarily observed in the second decade of life.Conclusion: Recent studies have demonstrated the importance of genetic testing upon unexplained persistent elevation of liver enzymes. This case highlights the importance of carefully evaluating a patient with an unexplained liver disorder, even in the presence of primary neurological disease, as it can have significant therapeutic implications.

the common standards for abnormality (less than or equal to 2.8 mm2/mmHg and less than or equal to 2.0 mm2/mmHg). Fisher's exact analysis was completed to determine whether abnormal IRP and abnormal DI are related. Results: In patients with a normal IRP (, 15) as measured by HRIM, the mean DI was 4.02 with a standard deviation of 2.89. In patients with abnormal IRP ( $ 15), the mean DI was 3.03 with a standard deviation of 2.58. The difference in the mean DIs was statistically significant (p-value 5 0.0234, t-test) . Table illustrates the number of patients in each IRP and DI subgroup using both cutoff standards. There was no statistically significant difference in the observed patient frequencies of any classification than would be expected by chance. Conclusion: We found that a normal IRP (, 15mmHg) is associated with higher DIs and that an abnormal IRP ($15 mmHg) was associated with a DI # 3.1. However, abnormal DI by the standards of either cutoff was not related to having abnormal IRP. Our data supports that both a high IRP and reduced DI suggest impaired LES relaxation, but a DI of less than 2.0 and 2.8 does not predict an IRP of less than 15 mmHg, suggesting some variability between the two metrics. While the chi-squared analysis approached but did not achieve statistical significance in this study, repetition with larger sample sizes in the future may yield a clearer relationship between abnormal IRP and abnormal DI.

rare and include worsening of demyelinating diseases, and neuropathies, often commencing months following treatment.to date. METHODS: We report the case of a 7 year old boy who was diagnosed with ulcerative colitis and primary sclerosing cholangitis at 6 and half years of age, who continued to have mild disease, manifested primarily by ongoing hematochezia and anemia despite Balsalazide and 6-Mercaptopurine therapy. His course to this point was unremarkable and his disease severity was mild with a Pediatric Ulcerative Colitis Score of 30. He was admitted for blood transfusion and underwent endoscopy and colonoscopy. He then received his first infusion of Infliximab. Other than mild sinus arrhythmia, he tolerated this well. There were no other notable events surrounding the infusion, he was interacting appropriately with normal vital signs and was discharged home. RESULTS: Within 6 hours of his infusion, he awoke from sleep from a severe headache and developed acute nausea and emesis. Half an hour later, he was slumped to one side and parents brought to a local emergency room. Upon arrival, he was completely unresponsive and emergently intubated. Given slight hypotension, he was suspected to have an anaphylactic reaction and received intravenous fluids, packed red blood cells and Epinephrine. Once transferred to our pediatric center, CT image of his brain revealed multifocal bilateral hypodense lesions with surrounding edema involving multiple lobes, the pons, and brainstem. Left sided lesions were largest and consistent with parenchymal hemorrhage. He was started on hypertonic saline and mannitol and external ventricular drains were promptly inserted, but his initial intracranial pressures were low. MRI imaging confirmed bilateral multi-territory infarcts that appeared to be arterial in nature without evidence of arterial clots, or venous infarcts. A workup for infectious encephalitis was negative, as was investigation for underlying anatomic anomalies of his vasculature. His presentation was not in keeping with an underlying vasculitis. An echocardiogram showed a dilated left ventricle, but did not show evidence of an arterial-septal defect, intracardiac thrombi or other abnormality. CONCLUSIONS: Our patient met criteria for brain death within 5 days following his single dose of Infliximab. Family refused an autopsy. We report a case of acute hemorrhagic stroke and ultimately fatal outcome following an initial infusion of Infliximab. Although the etiology of this event remains unclear, the close temporal relation to the Infliximab infusion cannot be ignored. To our knowledge, such a life threatening reaction within hours of Infliximab infusion has not been reported.