Perioperative management of children with urea cycle disorders.

BACKGROUND: Urea cycle disorders are congenital metabolism errors that affect ammonia elimination. Clinical signs and prognosis are strongly influenced by peak ammonia levels. Numerous triggers associated with metabolic decompensation have been described with many of them, including fasting or stress, being related to the perioperative period. AIMS: We aimed to assess perioperative complications in pediatric patients with urea cycle disorders requiring general anesthesia in our center. METHODS: We reviewed the clinical history of all the pediatric patients with a confirmed urea cycle disorders diagnosis requiring surgery or a diagnostic procedure with anesthesia between January 2002 and June 2018. RESULTS: We included 33 operations (major surgery, minor surgery, and diagnostic procedures) carried out on 10 patients via different anesthetic techniques. We observed the following complications: intraoperative hyperglycemia in one case, postoperative vomiting in eight cases, and slightly increased postoperative ammonia levels (54, 59, and 69 µmol/L) with normal preoperative levels in three cases without associated metabolic decompensation. There were two cases of perioperative hyperammonemia (72 and 69 µmol/L) secondary to preoperative metabolic decompensation (137 and 92 µmol/L) with the levels progressively dropping and normalizing in the first 24-48 hours, respectively. CONCLUSIONS: Procedures under anesthesia on pediatric patients with urea cycle diseases should be performed by experienced multidisciplinary teams at specialized centers. Perioperative management focused on avoiding catabolism (especially during fasting) and monitoring signs associated with metabolic decompensation to allow for its early treatment should be included in routine anesthetic techniques for children with urea cycle disorders.

Endoscopic ultrasonography-fine needle aspiration versus PET-CT in undiagnosed mediastinal and upper abdominal lymphadenopathy: a comparative clinical study.

BACKGROUND AND STUDY AIMS: The wide use of PET-CT for the staging of neoplasms has extended to enlarged lymph nodes of unknown origin. Nevertheless, upper abdominal and mediastinal nodes are accessible to endoscopic ultrasonography-fine needle aspiration (EUS-FNA), providing a cytological diagnosis, with a high diagnostic yield in previous reports. In this paper, we have compared the accuracy of both procedures in this particular setting. PATIENTS AND METHODS: After the finding of a lymphadenopathy in a conventional CT, both PET-CT and EUS-FNA were performed. The endoscopist had no information about PET-CT results. FNA was performed after a systematic EUS exam, with a 25 G needle and no suction. We considered the pathologic results as the gold standard or, if not available, the patients' outcome as a surrogate marker. RESULTS: A total of 54 patients were included. Common locations of nodes included subcarinal space (33.3%), porta hepatis (31.5%), upper mediastinum (15%), peripancreatic (7.4%), and other locations (12.8%). Adequate specimens were obtained in 48/54 patients (89%). The most common diagnoses based on cytology were benign/reactive (42%), epidermoid carcinoma (8.4%), lymphoma (8.4%), and ductal adenocarcinoma of pancreatic origin (6.3%). PET was positive in 67% of patients. The sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy of EUS-FNA were 91.3, 100, 100, 92.5, and 95.8%, respectively. The same values for PET-CT were 75, 25, 50, 50, and 50%, respectively. CONCLUSION: In our series, EUS-FNA has proven to be the best diagnostic procedure to accurately establish the etiology of isolated adenopathies, showing a much better diagnostic yield than PET-CT, the role of which should be re-evaluated in this setting.

Wireless capsule endoscopy: perspectives beyond gastrointestinal bleeding.

Wireless capsule endoscopy (CE) is a technology developed for the endoscopic exploration of the small bowel. The first capsule model was approved by the Food and Drug Administration in 2001, and its first and essential indication was occult gastrointestinal (GI) bleeding. Over subsequent years, this technology has been refined to provide superior resolution, increased battery life, and capabilities to view different parts of the GI tract. Indeed, cases for which CE proved useful have increased significantly over the last few years, with new indications for the small bowel and technical improvements that have expanded its use to other parts of the GI tract, including the esophagus and colon. The main challenges in the development of CE are new devices with the ability to provide therapy, air inflation for a better vision of the small bowel, biopsy sampling systems attached to the capsule and the possibility to guide and move the capsule with an external motion control. In this article we review the current and new indications of CE, and the evolving technological changes shaping this technology, which has a promising potential in the coming future of gastroenterology.

New insights to occult gastrointestinal bleeding: From pathophysiology to therapeutics.

Obscure gastrointestinal bleeding is still a clinical challenge for gastroenterologists. The recent development of novel technologies for the diagnosis and treatment of different bleeding causes has allowed a better management of patients, but it also determines the need of a deeper comprehension of pathophysiology and the analysis of local expertise in order to develop a rational management algorithm. Obscure gastrointestinal bleeding can be divided in occult, when a positive occult blood fecal test is the main manifestation, and overt, when external sings of bleeding are visible. In this paper we are going to focus on overt gastrointestinal bleeding, describing the physiopathology of the most usual causes, analyzing the diagnostic procedures available, from the most classical to the novel ones, and establishing a standard algorithm which can be adapted depending on the local expertise or availability. Finally, we will review the main therapeutic options for this complex and not so uncommon clinical problem.