RESUMEN
A 2-year-old male with VACTERL association and asthma presented to the emergency room due to asthma exacerbation. Chest radiography revealed lingular pneumonia and thickening of the left paraspinal line of the gastroesophageal junction. Chest computed tomography confirmed a heterogeneous fluid- and gas-filled structure at the left posterior lateral posterolateral aspect of the esophagus, which was suspected to be an esophageal diverticulum on an upper gastrointestinal series. The esophageal diverticulum was excised via left thoracoscopy, and pathological examination revealed pancreatic tissue. Heterotopic pancreas lacks anatomical, vascular, or ductal continuity with the native pancreas. It is usually asymptomatic, but when discovered, it usually occurs later in life. It has been described in the foregut, but is not as common in the esophagus, especially in the pediatric population. This case report highlights the rare occurrence, and importance of considering, esophageal heterotopic pancreas within an esophageal diverticulum in an asymptomatic patient with VACTERL association.
RESUMEN
Surgical repair of type C esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is complicated by an anastomotic leak in 10%-30% of cases with associated morbidity. A novel procedure in the pediatric population, endoscopic vacuum-assisted closure (EVAC), accelerates the healing of esophageal leaks by using the effects of VAC therapy, including fluid removal and stimulation of granulation tissue formation. We report 2 additional cases of chronic esophageal leak treated with EVAC in EA patients. The first is a patient with a previously repaired type C EA/TEF and left congenital diaphragmatic hernia complicated by an infected diaphragmatic hernia patch erosion into the esophagus and colon. Additionally, we discuss a second case using EVAC for early anastomotic leak following type C EA/TEF repair in a patient who was later found to have a distal congenital esophageal stricture.
RESUMEN
Pancreatic lithiasis, the formation of calcifications in the pancreatic duct, occurs uncommonly in pediatric patients but can occur more frequently with chronic pancreatitis (CP). Cystic fibrosis (CF) is one of the major causes of pancreatic lithiasis in pediatric patients, with mutations in the CF transmembrane conductance regulator (CFTR) gene reported in up to 23% of pediatric CP patients. Mutations in the CFTR gene can lead to mild cases of CF, which may delay diagnosis and treatment. In such cases, pancreatitis can be the presenting symptom in children with CF. We report a unique case of a 10-year-old female with previously undiagnosed and untreated CF presenting with abdominal pain, vomiting, and obstructive jaundice. Her pancreatic lithiasis and biliary obstruction were successfully treated with endoscopic retrograde cholangiopancreatography (ERCP).
RESUMEN
Repetitive, low-dose (metronomic; METRO) drug administration of some anticancer agents can overcome drug resistance and increase drug efficacy in many cancers, but the mechanisms are not understood fully. Previously, we showed that METRO dosing of topotecan (TOPO) is more effective than conventional (CONV) dosing in aggressive human prostate cancer (PCa) cell lines and in mouse tumor xenograft models. To gain mechanistic insights into METRO-TOPO activity, in this study we determined the effect of METRO- and CONV-TOPO treatment in a panel of human PCa cell lines representing castration-sensitive/resistant, androgen receptor (+/-), and those of different ethnicity on cell growth and gene expression. Differentially expressed genes (DEGs) were identified for METRO-TOPO therapy and compared to a PCa patient cohort and The Cancer Genome Atlas (TCGA) database. The top five DEGs were SERPINB5, CDKN1A, TNF, FOS, and ANGPT1. Ingenuity Pathway Analysis predicted several upstream regulators and identified top molecular networks associated with METRO dosing, including tumor suppression, anti-proliferation, angiogenesis, invasion, metastasis, and inflammation. Further, the top DEGs were associated with increase survival of PCa patients (TCGA database), as well as ethnic differences in gene expression patterns in patients and cell lines representing African Americans (AA) and European Americans (EA). Thus, we have identified candidate pharmacogenomic biomarkers and novel pathways associated with METRO-TOPO therapy that will serve as a foundation for further investigation and validation of METRO-TOPO as a novel treatment option for prostate cancers.
