We describe the results of eculizumab treatment of a patient with pachymeningitis, inflammatory infiltration of the left frontal lobe, and cerebral hematoma, who presented with progressive vision loss, epileptic seizures, and abnormal pattern of the complement system parameters. A 30-year-old female patient, initially diagnosed with hypereosinophilia and a tumour of the left orbit, developed a significant visual impairment in the left eye, progressive vision loss in the right eye, and neurological symptoms in the form of epileptic seizures and behavioural changes. Magnetic resonance imaging (MRI) revealed thickening of the dura mater in the left frontal area, slight oedema of the cortex, and subcortical white matter. Orbit biopsy showed non-specific inflammatory infiltrates. Despite the initial good response, symptoms progressed during treatment with glucocorticoids and immunosuppressants. Increased activity of the alternative complement pathway accompanied by a low level of its main inhibitor, factor H (FH), and the presence of anti-FH autoantibodies, was found. Genetic analysis revealed several missense variants of complement proteins, including two disease-linked mutations in FH (p.H402Y) and FI (T300A). An attempt to apply a complement C5 blocker, eculizumab, has been made. Neurological symptoms subsided, vision loss was inhibited, laboratory parameters improved, and discontinuation of steroid therapy was possible. The case underlines the role of complement system dysregulation in neurological distress.
Background: NeoDura (Medprin Biotech Gmbh) is an absorbable dural repair patch consisting of degradable poly-L-lactic acid and porcine gelatin that provides a hermetic closure of the dura mater (Medprin Biotech. Neodura. Dural Repair Patch [Brochure].
Objective: Retinopathy of prematurity (ROP) is a leading cause of preventable childhood blindness in preterm infants with low birth weight. The efficacy and safety of prophylactic agents, including vitamin A, propranolol and lipids, in reducing ROP incidence remain unclear. This systematic review and meta-analysis evaluated the effectiveness and safety of these agents in preventing ROP.
CYP1B1 is the most common gene implicated in primary congenital glaucoma (PCG) - the most common form of childhood glaucoma. How CYP1B1 mutations cause PCG is not known. Understanding the mechanism of PCG caused by CYP1B1 mutations is crucial for disease management, therapeutics development, and potential prevention.
Deep learning models are used to minimize the number of polyps that goes unnoticed by the experts and to accurately segment the detected polyps during interventions. Although state-of-the-art models are proposed, it remains a challenge to define representations that are able to generalize well and that mediate between capturing low-level features and higher-level semantic details without being redundant. Another challenge with these models is that they are computation and memory intensive, which can pose a problem with real-time applications.
Background: Little is known about the impact of PM on people with disabilities. We aimed to explore the association between PM and hospitalization via the emergency department (ED admission) among people with disabilities, together with the attributable ED admission cases and costs.
Methods: We applied a time-stratified case-crossover design adjusting ozone, holiday, and temperature using seven years (2015-2021) of claim-based data on ED admissions from the Korean National Health Insurance Database.
