Spontaneous Hemopericardium Complicated by Tamponade in a Child With Moderate Hemophilia A: Case-Based Review.

Hemophilia A is the most common severe innate bleeding disorder. It is an X-linked recessive inherited bleeding disorder characterized by a qualitative and/or quantitative deficiency of factor VIII. The clinical manifestation of this disease is hemorrhaging that can affect every organ, in particular joints (hemarthrosis) and muscles (hematoma). Some serious but rare hemorrhages can be life-threatening, in particular hemorrhage of the central nervous system and hemopericardium. We report a rare case of spontaneous hemopericardium complicated by tamponade in a child with moderate hemophilia A treated with Factor VIII replacement infusion and pericardial drainage, with a favorable outcome. To our knowledge, this is the second case described in the literature of spontaneous hemopericardium occurring in a child with hemophilia A. Our case suggests that a dose of 50 IU/kg/8 h of factor VIII maintained for up to one day after removal of the pericardial drain seems to be sufficient to ensure correct hemostasis, though further evidence is needed to confirm this impression.

Acute Flaccid Myelitis in a Pediatric Patient With Coronavirus Disease 2019.

The etiology of acute flaccid myelitis (AFM) has yet to be determined. Viral link has been suggested, but severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated AFM has not been reported in children. We describe a three-year-old boy, with AFM associated with coronavirus disease 2019 (COVID-19) infection. In the era of COVID-19 pandemic, patients with AFM should be tested for SARS-CoV-2.

Allergie aux protéines du lait de vache chez un nourrisson sous allaitement maternel exclusif.

INTRODUCTION: L'allergie aux protéines du lait de vache (APLV) est l'allergie alimentaire la plus fréquente au cours des premières années de vie. Elle est souvent associée à l'introduction des préparations à base de lait de vache et constitue une maladie rare chez les nourrissons allaités. OBJECTIF: Rapporter le cas d'une APLV chez un nourrisson sous allaitement maternel exclusif. Observation médicale. Un nourrisson âgé de 3 mois a été reçu avec une histoire de diarrhée chronique. La mère nie toute introduction de lait artificiel et le nourrisson est exclusivement nourri au sein. La concentration d'anticorps IgE spécifiques du lait de vache était en faveur de l'APLV. En interrogeant à nouveau la mère, elle souligne la notion de consommation d'une grande quantité de produits laitiers. Leur éviction était associée à un développement normal du nourrisson sans problèmes intestinaux. CONCLUSION: L'APLV peut se développer chez les nourrissons exclusivement allaités au sein. Exclure le lait de vache de l'alimentation de la mère est le seul remède quand elle veut encore allaiter.

High-fidelity simulation versus video-based learning in the management of pediatric septic shock: a pilot study.

High-fidelity simulation (HFS) and video-based learning (VBL) promote competence in acute care in a realistic and safe environment. These two modalities have not been compared in pediatric emergency situations. Interns rotating in the pediatric department were randomized for the two educational methods. The delivered learning subject was septic shock in children. The level of knowledge was measured before intervention, immediately after intervention (post-test 1) and 1 week later (post-test 2). Knowledge test scores improved significantly following intervention in both VBL study group and HFS study group (71.5 ± 13.2 [39.0-88.0], p < 0.001 and 80.1 ± 10.3 [57.4-94.5], p < 0.001, respectively). The improvement was significantly higher in HFS study group (p = 0.04). There was a non-significant drop in the retention score evaluated by the post-test 2 in the two groups compared to the post-test 1 score (66.9 ± 15.4 [31.5-86.1], p = 0.17 and 78.8 ± 12.4 [56.0-100.0], p = 0.72 in the VBL and HFS study groups, respectively). The retention score was significantly higher in the HFS group (p = 0.04).Conclusion: High-fidelity simulation and video-based training are both effective educational methods in teaching pediatric emergencies for interns. HFS appears to be superior in enhancing short-term retention. What is Known: • High-fidelity simulation is an effective educational tool to improve learners' knowledge and skills. • Video-based learning is an effective teaching tool in terms of short-term knowledge acquisition. What is New: • High-fidelity simulation is more effective in terms of short-term knowledge and generated more satisfaction than educational video learning.

Clinical and genetic heterogeneity in familial steroid-sensitive nephrotic syndrome.

BACKGROUND: Familial steroid-sensitive nephrotic syndrome (SSNS) is a rare condition. The disease pathophysiology remains elusive. However, bi-allelic mutations in the EMP2 gene were identified, and specific variations in HLA-DQA1 were linked to a high risk of developing the disease. METHODS: Clinical data were analyzed in 59 SSNS families. EMP2 gene was sequenced in families with a potential autosomal recessive (AR) inheritance. Exome sequencing was performed in a subset of 13 families with potential AR inheritance. Two variations in HLA-DQA1 were genotyped in the whole cohort. RESULTS: Transmission was compatible with an AR (n = 33) or autosomal dominant (AD, n = 26) inheritance, assuming that familial SSNS is a monogenic trait. Clinical features did not differ between AR and AD groups. All patients, including primary (n = 7) and secondary steroid resistant nephrotic syndrone (SRNS), (n = 13) were sensitive to additional immunosuppressive therapy. Both HLA-DQA1 variations were found to be highly linked to the disease (OR = 4.34 and OR = 4.89; p < 0.001). Exome sequencing did not reveal any pathogenic mutation, neither did EMP2 sequencing. CONCLUSIONS: Taken together, these results highlight the clinical and genetic heterogeneity in familial SSNS. Clinical findings sustain an immune origin in all patients, whatever the initial steroid-sensitivity. The absence of a variant shared by two families and the HLA-DQA1 variation enrichments suggest a complex mode of inheritance.