Neurinome du VIII à bilan électrophysiologique négatif.

Electrophysiological features in acoustic neurinoma are reviewed. A case of an acoustic neurinoma with a normal BERA is reported. The authors conclude that no isolated electrophysiological sign is able to disclose a retrocochlear hearing loss. They insist on the necessity to interpret an unilateral hearing loss on basis of an integration of a complete clinical, paraclinical and electrophysiological testing.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Indolent renal involvement with BRAFV600E mutation: Erdheim-Chester, a rare disease with a wide spectrum of clinical manifestations.

We present the case of a patient with three-year indolent bilateral ureteral and perirenal masses. Clinical presentation, radiological context, and histopathological findings with detection of BRAF V600E mutation confirmed the diagnosis of Erdheim-Chester disease (ECD). A review of current knowledge regarding diagnosis, clinical assessment, management, and treatment of ECD is also presented.info:eu-repo/semantics/publishe

Adult onset Kawasaki disease diagnosed by the echocardiographic demonstration of coronary aneurysms

A 17-year-old buy presented with fever, bilateral conjunctival infection, angina and extensive cervical adenopathy, Amoxycillin was started. Ten days later he was admitted to hospital because of persistant high fever, cervical adenopathy, erythrema of the pharynx and tongue and lip fissuration. The most important interventions of his first hospitalization were endotracheal intubation because of increasing dyspnoea due to adult respiratory distress syndrome and haemodialysis for renal insufficiency. His admission to our hospital was marked by the echocardiographic discovery of giant coronary aneurysms in the first few centimeters of both right and left coronary arteries. Coronary angiography confirmed giant aneurysm formation of the right and left coronary arteries. Similarly, medium sized arteries (cerebral, hepatic, mesenteric, iliac) presented abnormalities on angiography. Kawasaki syndrome was the final diagnosis, based on clinical presentation, coronary artery abnormalities and laboratory findings. This is the first description of adult-onset Kawasaki disease with giant coronary aneurysm formation and more generalized arterial involvement. The severity of the clinical symptoms and the severity of the coronary disease indicates that Kawasaki disease of the adult does not always have a benign course.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Humoral immunity to SARS-CoV-2 in kidney transplant recipients and dialysis patients: IgA and IgG patterns unraveled after SARS-CoV-2 infection and vaccination

Background: Infection with SARS-CoV-2 in high-risk groups such as kidney transplant and dialysis patients is shown to be associated with a more serious course of the disease. Four years after the start of the COVID-19 pandemic, crucial knowledge on the immune responses in these patient groups is still lacking. Therefore, this study aimed at investigating the humoral immune response after a SARS-CoV-2 infection compared to vaccination as well as the evolution of immunoglobulins over time. Methods: Kidney transplant recipients, patients on haemodialysis or on peritoneal dialysis and healthy controls were included in this longitudinal multicenter study. SARS-CoV-2 anti-RBD, anti-NP and anti-S1S2 immunoglobulin G (IgG) and A (IgA) as well as the neutralizing antibody capacity were measured. Results: Kidney transplant recipients had a significantly better humoral response to SARS-CoV-2 after infection (86.4%) than after a two-dose mRNA vaccination (55.8%) while seroconversion was comparable in patients on haemodialysis after infection (95.8%) versus vaccination (89.4%). In individuals without prior COVID-19, the IgG levels after vaccination were significantly lower in kidney transplant recipients when compared to all other groups. However, the IgA titres remained the highest in this patient group at each time point, both after infection and vaccination. A history COVID-19 was associated with higher antibody levels after double-dose vaccination in all patient categories and, while decreasing, titres remained high six months after double-dose vaccination. Conclusion: Kidney transplant recipients had a more robust humoral response to SARS-CoV-2 following infection compared to a two-dose mRNA vaccination, while patients on haemodialysis exhibited comparable seroconversion rates. Notably, individuals with prior COVID-19 exhibited higher IgG levels in response to vaccination. Hybrid immunity is thus the best possible defence against severe COVID-19 disease and seems also to hold up for these populations. Next, it is not clear whether the higher IgA levels in the kidney transplant recipients is beneficial for neutralizing SARS-CoV-2 or if it is a sign of disease severity.SCOPUS: ar.jinfo:eu-repo/semantics/publishe