Consensus on COVID-19 vaccination in pediatric oncohematological patients, on behalf of infectious working group of Italian Association of Pediatric Hematology Oncology

Vaccines represent the best tool to prevent the severity course and fatal consequences of pandemic by new Coronavirus 2019 infection (SARS CoV 2). Considering the limited data on vaccination of pediatric oncohematological patients, we develop a Consensus document to support the Italian pediatric hematological oncological (AIEOP) centers in a scientifically correct communication with families and patients and to promote vaccination. The topics of the Consensus were: SARS CoV 2 infection and disease (COVID 19) in the pediatric subject s; COVID 19 vaccines (type, schedule); which and when to vaccinate; contraindications and risk of serious adverse events; rare adverse events; third dose and vaccination after COVID 19; and other general prevention measures. Using the Delphi methodology fo r Consensus, 21 statements and their corresponding rationale were elaborated and discussed with the representatives of 31 centers, followed by voting. AIEOP Centers showed an overall agreement on all the statements that were therefore approved. This consensus document highlights that children and adolescents affected by hematological and oncological diseases are a fragile category. Vaccination plays an important role to prevent COVID19, to permit the regular administration of chemotherapy or other treatmen ts, to perform control visits and hospital admissions, and to prevent treatment delays.

Supernumerary Remnant Vas Deferens Like Wolffian-Derivatives in a Young Child With Abdominal Cocoon Syndrome—A Clinicopathological Novelty

The abdominal cocoon syndrome is a rare cause of recurring intestinal obstruction in children. It refers to encasement of the small bowel by a fibrocollagenous membrane forming a cocoon. We report a nine year old male presenting with abdominal pain, distension, bilious vomiting and inability to pass stool and flatus for two days. In view of a persistently increasing bilious nasogastric output, an urgent exploratory laparotomy was performed. The small bowel loops were matted together forming a cocoon densely adherent to the parietal peritoneum with supra-colic fibrous bands. The bands histologically displayed multiple ductal remnants with epithelium resembling that of ductus deferens. These structures showed immunopositivity for pan-cytokeratin and basal CD10.Workup for tuberculosis and other etiological causes was unremarkable. This is the first documented case of abdominal cocoon in a pediatric subject associated with supernumerary wolffian remnants.

ECMO usefulness in refractory shock secondary to Phenylephrine poisoning: About a case in a Pediatric subject

Introduction: Phenylephrine is an alpha-agonist with vasoconstrictor effect. Systemic absorption or adverse reaction can cause complications that can be lethal. In those cases of adverse drug reactions with failure to conventional management, the use of ECMO has been reported as a treatment modality. Clinical case: We report the case of a 13 years old teenager, who presented acute pulmonary edema and acute heart failure subsequent to the application of topical phenylephrine during the a rhino-septoplasty procedure, and that warranted placement of ECMO due to failure of conventional treatment. Discussion: Venoarterial ECMO was placed by central cannulation, with ventilatory and hemodynamic improvement, performing the explant successful 4th postoperative day. Conclusions: ECMO as a "bridge to recovery" may be an alternative in those cases of pulmonary or cardiac failure and refractory with a poor response to conventional treatment, as well as an option to save the life of these patients.

Perioperative Management of Hemostasis with Recombinant FIX Fc Fusion Protein in Subjects Undergoing Surgery in the B-YOND Study

Background: The phase 3 B-LONG (adults/adolescents ≥12 years of age) and Kids B-LONG (children <12 years of age) studies demonstrated the safety and efficacy of recombinant FIX Fc fusion protein (rFIXFc) for the control and prevention of bleeding episodes in subjects with severe hemophilia B (FIX levels ≤2 IU/dL). Eligible subjects from either study could continue rFIXFc treatment in the ongoing extension study, B-YOND, during which surgical procedures were allowed. Aims: To describe rFIXFc efficacy and safety in B-YOND subjects undergoing surgery. Methods: B-YOND subjects who underwent surgery received an investigator-determined rFIXFc regimen. Major surgery endpoints included rFIXFc dosing, investigator/surgeon assessment of hemostatic response (4-point scale: excellent, good, fair, poor/none), blood loss, number of transfusions, and safety. For minor surgeries, hemostatic response and dosing are summarized. Surgeries performed up to cutoff for the interim analysis (17 October 2014) were included. For safety analyses, the surgery subgroup included subjects who underwent major surgery during B-YOND and those who underwent major surgery in the parent study but had their surgical/rehabilitation period during B-YOND. Results: Fifteen major surgeries were performed in 8 subjects (7 adults/adolescents and 1 child <12 years of age), most commonly orthopedic procedures (9/15 surgeries). In adults/adolescents undergoing major surgery, the median total rFIXFc dose on the day of surgery was 135.92 IU/kg (range, 60.6-317.9 IU/kg); 12 of these 14 surgeries required a single rFIXFc infusion to maintain hemostasis during surgery. The number of adults and adolescents undergoing major surgery who required an infusion decreased from Day 0 to Day 14. A single rFIXFc infusion of 99.43 IU/kg was required on the day of surgery for the single major surgery in a pediatric subject (tonsillectomy). Of 15 major surgeries, 14 surgeries were assessed for hemostatic response to rFIXFc and all were rated as excellent (n = 13, including the single pediatric surgery) or good (n = 1). Among 12 major surgeries with estimates, blood loss ranged from 0-1,000 mL intraoperatively and 0-1,000 mL postoperatively (for the single pediatric subject, blood loss was 50 mL intraoperatively and 0 mL postoperatively). One additional subject had an estimated intraoperative blood loss of 5,000 mL during liver transplantation and was the only subject who required transfusion (6 units of packed cells, 4 units of platelet concentrate, and 4 units of fresh frozen plasma intraoperatively), consistent with previously reported blood loss for this procedure in the general population (Yuasa et al. Transfusion 2005;45:879). Hemostatic response for this surgery was rated as excellent. No subject reported a bleeding episode during the postoperative/rehabilitation period. Among 12 subjects in the surgery subgroup (8 with major surgery during B-YOND; 4 with major surgery in B-LONG and a surgical/rehabilitation period during B-YOND), 3 subjects (25%) reported ≥1 adverse event (AE) during the perioperative management period. One subject who underwent major surgery experienced 2 serious AEs (anal sphincter atony and epididymitis; considered unrelated to treatment) during the perioperative management period. All AEs were assessed by the investigator as unrelated to treatment and resolved. Of 25 minor surgeries performed in 17 adults/adolescents (none in children), approximately half were dental procedures (12/25 surgeries). No rFIXFc was administered on the day of surgery for 2 minor surgeries. For the other 23 surgeries, the median number of rFIXFc infusions was 1.0 (range, 1-3) and the median total dose of rFIXFc was 79.84 IU/kg (range, 38.5-200.0). Hemostatic response was assessed in 10 of 25 minor surgeries; all were rated as excellent (n = 9) or good (n = 1). Summary/Conclusion: Hemostasis with rFIXFc during surgery was rated favorably by investigators/surgeons, with comparable blood loss to what would be expected for subjects without hemophilia. Taken together with results from the B-LONG and Kids B-LONG studies, these data indicate that rFIXFc is well-tolerated and efficacious for the management of perioperative hemostasis in individuals with severe hemophilia B. Disclosures Ozelo: Baxter: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novo Nordisk: Research Funding, Speakers Bureau; Biogen: Research Funding. Mahlangu:Bayer, CSL, Novo Nordisk, and Biogen: Research Funding, Speakers Bureau; Biotest: Speakers Bureau; Roche: Research Funding; Amgen: Speakers Bureau. Perry:Novo Nordisk: Consultancy, Membership on an entity's Board of Directors or advisory committees; Biogen: Consultancy, Honoraria. Yuan:Biogen: Employment, Equity Ownership. Cristiano:Biogen: Employment, Equity Ownership. Mei:Biogen: Employment, Equity Ownership.

Clerkship Curricular Revision Based on the Ambulatory Pediatric Association and the Council on Medical Student Education in Pediatrics Guidelines: Does It Make a Difference?

Objective. To compare general pediatric knowledge acquisition and clinical problem-solving skills by students pre- and postcurricular reform based on the 1994 Ambulatory Pediatric Association and the Council on Medical Student Education in Pediatrics (APA–COMSEP) curricular guidelines. Setting. A large, urban academic medical center. Subjects. Third-year medical students on a required clerkship in Pediatrics. Intervention. Pre- and postcurricular revision, the students were given both the National Board of Medical Examiners (NBME) Pediatric Subject Examination and an objective examination, which was developed in-house, based on the APA–COMSEP guidelines (Pediatric Clerkship Examination [PCE]). Baseline data before curricular revision were obtained on 52 students from May 1995 to May 1996. After curricular redesign in May 1996, data were obtained on 42 students from May 1996 to May 1997. Curricular revision focused on the following: defining educational principles, selecting teaching strategies, defining learning objectives, implementing the curriculum, and evaluating the students with an examination. Results. Before curricular revision, the average NBME score was 521 ± 122. The average PCE score was 53.7% ± 10.1%. After curricular revision, the average NBME score was 520 ± 109, and the average PCE score was 67.7% ± 8.4%. Content areas showing the greatest improvement were fluids and electrolytes, issues pertaining to the newborn, and health supervision. Conclusions. Our baseline data indicate that despite spending two thirds of the clerkship in the ambulatory setting, students did not acquire adequate general pediatric knowledge or clinical problem-solving ability. After broad clerkship revision based on the APA–COMSEP Core Curriculum, students' acquisition of general pediatric knowledge and clinical problem-solving improved significantly, as measured by the PCE. The overall NBME Pediatric Subject Examination scores did not reflect this increased acquisition of general pediatric knowledge.