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Aim of the Study: To describe perianal Crohn's disease behavior and the role of biological therapy in a sample of pediatric patients., Methods: A retrospective study of pediatric patients with Crohn's disease (CD) treated in our institution from 2017 to 2021, with a minimum follow up period of 6 months, was conducted. Patients were divided whether they had perianal disease (PD) or not. Baseline characteristics, extension of disease, growth failure rate, aggressive pattern rate, use of biological therapy and need for surgery, among other variables, were compared between both groups. Clinical and/or radiological improvement in the last 6 months of follow up was considered good control of PD., Results: Seventy eight pediatric patients with CD were included. Median age at diagnosis was 10.5 years, and median follow up time was 3.8 years. 64.1% patients were male. Of all, 15 (19.2%) had perianal disease, of which 10 had fistulizing findings and 5 had non fistulizing findings. PD was presented at diagnosis in 8 patients, and the rest developed it in a median time of 1 year from diagnosis. PD was associated with growth failure (p = 0.003), use of biological therapies (p = 0.005), and need for second line of biologics (p = 0.005). Most patients (12/15, 80%) had good control of PD with the treatment received., Conclusions: CD patients with PD seem to need a more aggressive treatment, with biological therapies playing a key role for its handling nowadays. These patients require close nutritional evaluation that ensures proper development and growth.

Objective: To study gastroesophageal reflux (GER) in children undergoing gastrostomy in a single pediatric institution., Material and Methods: A retrospective study of patients undergoing gastrostomy from 2000 to 2017 was carried out. Demographic data, clinical data, progression, and complications were recorded. GER was considered positive in patients with clinical signs requiring antisecretory treatment, prokinetic treatment, or anti-reflux surgery to control symptoms., Results: 207 patients with a median age of 2 years [R: 0.25-18] were included. Neurological impairment was the most frequent underlying condition (74%). Swallowing difficulty and undernourishment were the main surgical indications for gastrostomy. Prior to gastrostomy, 96 out of 207 patients (46%) showed GER symptoms. Combined fundoplication and gastrostomy was performed in 41 (43%) patients with preexisting GER, 6 of whom showed GER worsening (4 required redo fundoplication). 5 complications following fundoplication were noted - gastric perforation, sustained Dumping syndrome, and gastroesophageal stenosis. 55 out of 96 (57%) patients with preexisting GER underwent gastrostomy alone. Clinical signs disappeared in 16 of them (29%) and improved or stabilized in 19 (35%). GER worsening occurred in 20 patients (36%), with subsequent fundoplication being required in 10 cases. In patients with no previous clinical signs (111 out of 207), GER symptoms occurred following gastrostomy in just 18 cases (16%), and only 2 patients required fundoplication., Conclusions: In our experience, routine anti-reflux surgery combined with gastrostomy is not justified. Individualized fundoplication should be considered in case of medical treatment failure. Further studies with an adequate design are required to establish which patients could really benefit from this procedure.

Objective: To describe our experience in the diagnostic and therapeutic management of patients with acute abdomen as the main manifestation of SARS-CoV-2 infection., Material and Methods: A descriptive study of patients with clinical signs of acute abdomen diagnosed with COVID-19 and admitted at out healthcare facility from April 1 to May 10, 2020 was carried out. Clinical records were reviewed for data collection purposes., Results: A series of 14 patients (9 male and 5 female) with a median age of 9.5 years was analyzed. All patients had abdominal pain. There were 11 patients with fever, 9 patients with vomit or diarrhea, and 9 patients with clinically suspected surgical pathology (acute appendicitis or peritonitis). Increased acute phase reactants and coagulation disorders were a common characteristic at blood tests. An abdominal ultrasonography was carried out in all patients, and a CT-scan was performed in 4 patients, which demonstrated inflammatory signs in the terminal ileum, the ileocecal valve and the ascending colon, as well as gallbladder edema. Conservative management was decided upon in all patients except one, and eight patients required intensive care admission for support treatment., Conclusions: Gastrointestinal symptoms can be the primary manifestation of the new coronavirus infection, which simulates an acute abdomen with a potentially unfavorable evolution. For an accurate diagnosis to be achieved, a good clinical record and a comprehensive physical exploration, as well as complementary tests in search of characteristic findings of COVID-19, should be carried out.

Objective: To detect tumor-infiltrating lymphocytes (TILs) in the peripheral blood (PB) of a preclinical neuroblastoma model., Materials and Methods: Two types of preclinical models - immunodeficient mice and immunocompetent mice - were generated by injecting a cell suspension of neuroblastoma cell line NB36769 with MYCN gene (TH-MYCN+) overexpression. Spleen, tumor, and peripheral blood were studied using flow cytometry to detect PD-1+ T-cells. TCR-β immunosequencing was performed in matched samples (tumor and peripheral blood)., Results: Most PB T-cells of immunodeficient mice were CD4 (control: 83.1%; tumor: 86.1%), with a small proportion of PD-1+ T-cells (control: 0.4%; tumor: 0.3%). However, the percentage of PD-1+ T-cells in the spleen was higher (control: 6.5%; tumor: 6.2%), and it was expressed in the CD4+ subset only. Regarding the TCR repertoire of immunocompetent mice, the proportion of the 10 most frequent sequences was significantly higher in tumors (11.09% ± 2.83%) than in the peripheral blood (1.59% ± 0.59%) (p=0.024). These findings are suggestive of clonotype enrichment within the tumor. 9 out of the 10 most frequent tumor clones were identified in the matched peripheral blood sample in 2 mice, and 6 out of 10 in one mouse. In addition, TILs with shared sequences from different animals were found., Conclusions: Our results in terms of immunophenotype and clonality suggest the presence of PB T-cells which could include TILs in a preclinical neuroblastoma model.

Background To describe the complications and long-term results in patients with multiple endocrine neoplasia type 2A (MEN 2A) syndrome in whom a prophylactic thyroidectomy was performed, in relation to the recommendations of the American Thyroid Association (ATA). Methods A retrospective study of 14 patients with MEN 2A thyroidectomized between 2000 and 2017. We reviewed demographic, clinical, analytical and radiological data. Postoperative complications and long-term follow-up were analyzed. Results We treated eight boys and six girls with a median age of 5 years old (range 2-10). The predominant genetic mutation belonged to codon 634 (8/14, 57.14%). Total thyroidectomy (TT) without cervical lymphadenectomy was performed in all patients. A right upper parathyroidectomy was performed in one patient due to intraoperative suspicion of increased volume. Histological study revealed no alterations. Two patients presented transient hypocalcemia postoperatively and no patient had permanent hypocalcemia or nerve damage. Pathological anatomy confirmed medullary thyroid microcarcinoma in 5/14 patients: all carrying codon 634 mutation and three of them with preoperative basal calcitonin levels <20 pg/mL. No recurrences or metastases have been detected after a mean follow-up of 8 years. A patient with codon 634 mutation developed a unilateral pheochromocytoma at 25 years of age. No patient has presented hyperparathyroidism. Conclusions Prophylactic thyroidectomy without cervical lymphadenectomy is an effective and safe preventive treatment in patients with MEN 2A syndrome when it is performed by experienced surgeons in reference centers.

Objectives: To estimate the recurrence rate of intussusception after successful enema reduction and to analyze the costs of hospital admission with the current management of this pathology., Material and Methods: A retrospective study over 5 years of 97 patients with confirmed diagnosis of intussusception was undertaken. Medical records were evaluated for patient demographic, clinical and radiological data. Patients with enema-reduced intussusception were selected and data respecting to timing and outcome of recurrences, and length of stay were analyzed. Recurrence was defined as a new episode of intussusception within 72 hours of the initial presentation. Costs were calculated using hospital-specific data., Results: During the study period there were 81/97 children with successful enema reduction. Mean length of stay was 35 hours in the conservative treatment group. There were 8 episodes of recurrence, 5 of them within the first 24 hours (6.17%), for an overall recurrence rate of 9.88%. 7 children were treated by repeated enema and only one patient needed surgery for persistent illness. Assuming the overall recurrence rate of 9.88%, it would require hospitalizing 16 patients to identify a single recurrence with a cost of 1,723.75 € per patient., Conclusions: Given the low recurrence rate for enema-reduced intussusception and the possibility of repeated enema for their treatment in most of recurrences, we strongly advocated for the outpatient management as a safe and cost-effective alternative.