A Fabry Disease Patient Who Developed Hypersensitivity Reaction against Agalsidase Beta following COVID-19 Infection

Fabry disease (FD) is a rare, X-linked inherited lysosomal storage disorder, characterized by the accumulation of globotriaosylceramide (Gb3) due to the deficiency or absence of alpha-galactosidase A. Due to the accumulation of Gb3, cardiac, renal, neurological, and skin manifestations can be observed. Enzyme replacement therapy (ERT) with agalsidase alfa or agalsidase beta is the cornerstone in the management of FD. Both enzymes are clinically effective and widely used. In this study, we present a 19-year-old male patient with FD who had received ERT for almost two and half years without any complications. In January 2021, he was diagnosed with COVID-19 infection. Later, he developed an infusion reaction during his first ERT infusion following the resolution of COVID-19 infection. The patient experienced shortness of breath, shivering, and rash. Despite decreased infusion rate and premedication in repetitive infusion, his symptoms were not resolved. Subsequently, he developed an IgE antibody against agalsidase beta, and his skin prick test was positive. Since IgG positivity against agalsidase beta was also detected, agalsidase beta was replaced with agalsidase alfa. The patient did not experience any allergic reaction with agalsidase alfa. Moderate to severe allergic reactions during ERT infusion should be alarming for IgE development. Furthermore, COVID-19 should be considered a trigger for allergic reaction against ERT in patients with FD

Impact of COVID-19 lockdown on mood status: A comparison of hemodialysis and oncology patients

Introduction Coronavirus related lockdown measures had a severe psychological toll. We investigated the effects of population-wide infection control measures on hemodialysis (HD) and oncology patients' mood statuses. Methods An electronic survey was used to collect data. We used the Profile of Mood States (POMS) and the Hospital Anxiety and Depression Scale (HADS) to measure the mood statuses. Results The study groups composed of 400 HD, 234 oncology patients, and 276 controls. HADS anxiety and total POMS scores were significantly higher in the control group than that of both HD and oncology patients, a finding consistent with a worse mood status. In multivariate models, the male gender and the perception of having received appropriate medical support during the pandemic period were independent predictors of better POMS and HADS-anxiety and depression scores. Conclusion HD and oncology patients appear to be less affected by the psychological stress induced by social distancing and lockdown periods

Relationship between disease awareness and severity of kidney disease in autosomal dominant polycystic kidney disease patients

Introduction Polycystic kidney disease (PKD) is responsible for 5%-10% of end-stage renal disease. We examined the relationship between renal and extrarenal findings, disease severity, and the level of consciousness of PKD patients. Methods Patients were asked to answer the questionnaire about PKD. Disease severity was determined according to estimated glomerular filtration rate, and disease awareness was assessed by adapting the Disease Perception Scale to PKD. Awareness of patients was evaluated comparatively with chronic kidney disease stage, age, region, and symptoms. Results One out of five patients does not know that this disease is inherited. Mean awareness scores of the patients decreased significantly with increasing age. Awareness scores were significantly higher in patients with flank pain, hematuria, and urinary tract stones. Conclusion Although PKD is the most common hereditary kidney disease, the rate of patients' knowledge on this subject is low. Increased awareness might lead to better treatment in those patients