Life-threatening pediatric poisoning due to ingestion of Bufo bufo toad eggs: A case report.

Bufo parotid glands and eggs contain cardiac glycosides also known as bufadienolides. This class of molecules can cause digoxin-like cardiac toxicity, as they can block the sodium potassium-adenosine triphosphatase (Na/K-ATPase) pump. Poisoning with these toxins is rare but carries a high mortality risk. There are only a few cases of toad poisoning that have been reported worldwide, mainly in the southern hemisphere. We will describe the case of a child on the autistic spectrum disorder who developed an acute and severe cardiac bradyarrhythmia soon after being in a mountain creek. The child ingested a large quantity of Bufo bufo toad eggs and developed bradycardia (35/min) associated with junctional rhythm with narrow QRS complexes. The poison control center (PCC) indicated the use of atropine on the way to the nearest hospital and the administration of antidotal therapy, i.e., anti-digoxine fragment antibodies (DigiFab), as soon as possible. The patient was transferred by air ambulance to the Regional Referral Pediatric Hospital (RRPH), tested for digoxin blood level by immuno-essay (0.68 ng/mL) and successfully treated with five vials of DigiFab, since atropine administration produced only a fleeting effect on the cardiac rhythm. Patient was discharged 48 hours after poisoning. The presence of bufadienolides in the toad eggs was also confirmed. To our knowledge, this is the first report of toad egg poisoning in Europe. The administration of Digifab helped to reverse the bufadienolide cardiac toxicity.

Quality Improvement Initiative Using Blended In Situ Simulation Training on Procedural Sedation and Analgesia in a Pediatric Emergency Department: Better Patient Care at Lower Costs.

INTRODUCTION: There is evidence in the literature for high-fidelity in situ simulation training programs being an effective modality for physicians training. This quality initiative focused on implementation of the procedural sedation and analgesia (PSA) in our pediatric emergency department (PED). The primary outcomes of this study were to evaluate the impact of blended in situ simulation training (BST) program on PSA for closed forearm fracture reduction in the PED and to assess its cost-effectiveness. The secondary outcomes were to estimate this change on PSA's clinical efficacy and safety. METHODS: Between 2014 and 2018, a single-center, quasi-experimental, uncontrolled before and after study on forearm fracture reduction management was conducted. To assess the impact of our BST-PSA course, both historical control and prospective analyses were performed. Statistical significance was based on Fisher exact test or Pearson χ 2 test. RESULTS: Eight hundred eighty-five children met inclusion criteria. A significant difference in the number of PSAs performed in the PED, before and after BST, was found (37% vs. 85.3%, P < 0.001). Furthermore, a reduction in the number of hospitalizations for closed fracture reduction was measured (68.2% vs. 31.8%, P < 0.001). The overall cost savings from the BST-enabled increase in PSAs carried out in the PED was €370,714 ($440,838) with a return on investment of 64:1. No significant increase of PSA-related adverse events was found, and no serious adverse events occurred. CONCLUSIONS: Findings provide evidence of the benefits of implementing BST to enable PSA use in the PED, with an improved patient flow and significant cost savings from avoiding unnecessary hospitalizations.

The development of a Consensus Conference on Pediatric Procedural Sedation in the Emergency Department in Italy: from here where to?

BACKGROUND: In Italy, as in many European countries, Pediatric Emergency Medicine is not formally recognized as a pediatric subspecialty, hindering nation-wide adoption of standards of care, especially in the field of procedural sedation and analgesia (PSA) in the Emergency Department (ED). For this reason PSA in Italy is mostly neglected or performed very heterogeneously and by different providers, with no reference standard. We aimed to describe the procedures and results of the first multidisciplinary and multi-professional Consensus Conference in Italy on safe and effective pediatric PSA in Italian EDs. METHODS: The preparation, organization and conduct of the Consensus Conference, held in Florence in 2017, followed the recommended National methodological standards. Professionals from different specialties across the country were invited to participate. RESULTS: Overall 86 recommendations covering 8 themes (pre-sedation evaluation, pharmacologic agents, monitoring, equipment and discharge checklists, training, non-pharmacologic techniques, the adult ED setting, impact on hospitalizations) were developed, taking into account the Italian training system and healthcare organization characteristics. CONCLUSION: The results of the first multidisciplinary and multi-professional Consensus Conference in Italy are meant to provide up-to-date national guidance to improve the standard of care of children undergoing painful and stressful procedures in the ED. The recommendations will be periodically updated as new relevant evidence is published.

Severe parainfluenza pneumonia in a case of transient hypogammalobulinemia of infancy.

Human parainfluenza viruses (HPIVs) infection, largely known to cause self-limiting bronchiolitis and pneumonia in immune competent patients, can lead to severe to fatal pulmonary disease in immune disorders, such as primary or acquired-immune deficiencies. We report the case of a 1-year-old child who developed an acute respiratory distress syndrome. Because of a progressive respiratory failure unresponsive to conventional treatment extracorporeal membrane oxygenation (ECMO) was rapidly started. HPIV-3 infection was diagnosed on the rhinopharyngeal fluid and immunological examinations revealed a hypogammaglobulinemia. A combination therapy with ribavirin, intravenous immunoglobulin (IVIG) and steroid under ECMO support was started with considerable improvement. Subsequent analysis and more specific immunological assessment resulted normal confirming the diagnosis of transient hypogammaglobulinemia of infancy (THI). This case highlights the importance of prompt therapy with early ECMO support in combination with ribavirin, IVIG and steroids in patients affected by severe HPIV-3 pneumonia and THI.

New findings concerning cardiovascular manifestations emerging from long-term follow-up of 150 patients with the Williams-Beuren-Beuren syndrome.

AIMS: We investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome. METHODS AND RESULTS: We reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died. CONCLUSION: Cardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome.

Specific types and subtypes of cardiac defects have been described in children with 22q11.2 deletion syndrome as well as in other genetic syndromes. The conotruncal heart defects occurring in patients with 22q11.2 deletion syndrome include tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, interrupted aortic arch, isolated anomalies of the aortic arch, and ventricular septal defect. These conotruncal heart defects are frequently associated in this syndrome with additional cardiovascular anomalies of the aortic arch, pulmonary arteries, infundibular septum, and semilunar valves complicating cardiac anatomy and surgical treatment. In this review we describe the surgical anatomy, the operative treatment, and the prognostic results of the cardiac defects associated with 22q11.2 deletion syndrome. According to the current literature, in patients with tetralogy of Fallot with/without pulmonary atresia and truncus arteriosus, in spite of the complex cardiac anatomy, the presence of 22q11.2 deletion syndrome does not worsen the surgical prognosis. On the contrary in children with pulmonary atresia with ventricular septal defect and probably in those with interrupted aortic arch the association with 22q11.2 deletion syndrome is probably a risk factor for the operative treatment. The complex cardiovascular anatomy in association with depressed immunological status, pulmonary vascular reactivity, neonatal hypocalcemia, bronchomalacia and broncospasm, laryngeal web, and tendency to airway bleeding must be considered at the time of diagnosis and surgical procedure. Specific diagnostic, surgical, and perioperative protocols should be applied in order to provide appropriate treatment and to reduce surgical mortality and morbidity.

Cardiac outflow tract: a review of some embryogenetic aspects of the conotruncal region of the heart.

A review concerning some embryogenetic aspects of the cardiac outflow tract is presented. Two main topics are discussed: the truncal septation and the secondary heart field. In the context of the septation of the truncus arteriosus, the development of the arterial valves is largely discussed, particularly in reference to the sinuses of Valsalva. Emphasis is also given to the fate of the external myocardial wall of the truncus arteriosus, as this primordial myocardial surface disappears later in the development. Molecular genetics data concerning Sox4 and NF-Atc transcription factors are correlated in the present review with rare forms of truncus malformations encountered in human pathology. The roles exerted by the secondary heart field and the neural crest on the development and growth of the conotruncal musculature are largely discussed. Reported experimental ablations of both secondary heart field and neural crest, showed conotruncal defects such as persistent truncus arteriosus, tetralogy of Fallot, and double-outlet right ventricle, which were considered as the result of a short outflow tract causing, ultimately, a lack of conotruncal rotation. In this regard, some morphologic correlations are carried out, in the present review, between these experimental animal models and human malformations, and it is thought that this sort of conotruncal defects cannot be explained always in terms of conotruncal hypoplasia. Finally, influence of Pitx2c, a left-right laterality signaling gene, on the modulation of the conotruncal rotation, as most recently reported, is emphasized in terms of very likely multifactorial contributions in the embryogenesis of the conotruncal region of the heart.