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.

A comparison of antibody response in kidney transplant recipients and healthcare workers who had PCR-confirmed COVID-19 infection.

BACKGROUND: Data on antibody response following COVID-19 in kidney transplant recipients is scarce. This crosssectional study aims to investigate the antibody response to COVID-19 among kidney transplant recipients. METHODS: We recruited 46 kidney transplant recipients with RT-PCR-confirmed COVID-19 and 45 recipients without COVID-19 history. We also constructed two control groups (COVID-19 positive and negative) from a historical cohort of healthcare workers. We used age and sex-based propensity score matching to select the eligible subjects to the control groups. We measured the SARS-CoV-2 IgG levels quantitatively using the Abbott ARCHITECT system. An antibody level above 1.4 S/C was defined as positivity. RESULTS: Transplant recipients with COVID-19 had a higher BMI, and COVID-19 history in a household member was more common than that of the transplant recipient without COVID-19. IgG seropositivity rate (69.6% vs. 78.3%, p = 0.238) and the median IgG level (3.28 [IQR: 0.80-5.85] vs. 4.59 [IQR: 1.61-6.06], p = 0.499) were similar in COVID-19-positive transplant recipients and controls. Kidney transplant recipients who had a longer duration between RT-PCR and antibody testing had lower antibody levels (r = -0.532, p < 0.001). DISCUSSION: At the early post-COVID-19 period, kidney transplant recipients have a similar antibody response to controls. However, these patients' antibody levels and immunity should be closely monitored in the long term.

ANCA-associated vasculitis flare might be provoked by COVID-19 infection: a case report and a review of the literature.

Mesangial immunoglobulin A (IgA) deposition is the hallmark of IgA nephropathy (IgAN). In some cases, crescentic involvement that might be associated with systemic leucocytoclastic vasculitis is documented. In such cases, the disease is called Henoch-Schönlein purpura (IgA vasculitis). Even more rarely, the coexistence of IgAN and anti-neutrophil cytoplasmic antibody (ANCA) seropositivity has been reported. IgAN might be complicated by acute kidney injury (AKI) due to different causes. Herein we present a patient with mesangial IgA deposition and ANCA seropositivity who developed AKI, haematuria and haemoptysis during the course of coronavirus disease 2019 (COVID-19) disease and was diagnosed with ANCA-associated vasculitis based on clinical, laboratory and radiological findings. The patient was treated successfully with immunosuppressive therapy. We also made a systematic review of the literature to reveal and present the cases with COVID-19 and ANCA-associated vasculitis.

ANCA-associated vasculitis flare might be provoked by COVID-19 infection: a case report and a review of the literature

Mesangial IgA deposition is the hallmark of IgA nephropathy. In some cases, crescentic involvement which might be associated with systemic leukocytoclastic vasculitis is documented;in such cases, the disease is called Henoch-Schonlein purpura (IgA vasculitis). Even more rarely, the coexistence of IgA nephropathy and ANCA seropositivity was reported. IgA nephropathy might be complicated by acute kidney injury due to different causes. Herein, we present a patient with mesangial IgA deposition and ANCA seropositivity, who developed acute kidney injury, hematuria, and hemoptysis during the course of COVID-19 disease and was diagnosed with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on clinical, laboratory, and radiological findings. The patient was treated successfully with immunosuppressive therapy. We also made a systematic review of the literature to reveal and present the cases with COVID-19 and ANCA-associated vasculitis. Graphical Graphical

The impact of the COVID-19 pandemic on Fabry Disease Patients: an examination of Mood Status, Therapy Adherence, and COVID-19 infection.

BACKGROUND: Fabry disease (FD) is a rare metabolic disorder, in which a lifelong enzyme replacement therapy (ERT) constitutes the cornerstone of disease-specific therapy. In this study, we examined the effects of the COVID-19 pandemic and lockdown measures on the management of FD patients. METHODS: We collected data in three main domains; mood status, adherence to ERT, and COVID-19 infection. We used the Hospital Anxiety and Depression Scale (HADS) to evaluate the mood statuses of FD patients and the Morisky Medication Adherence Scale (MMAS) and the Medication Adherence Report Scale (MARS) to assess patients' adherence to non-disease specific therapy. We also examined a control group to compare the mood status data. RESULTS: A total of 67 FD patients (males: 47.8%, mean age: 37.0 years) were recruited to the study, of which 58 were receiving ERT. Both the HADS depression and anxiety scores were higher in the control group compared to FD patients. During the first wave of the pandemic, 25 patients reported to have missed an infusion for a mean of 2.3 ± 1.7 doses and half of the patients had adopted a home-based infusion treatment regimen. COVID-19 infection developed in 25 patients, of which one died. The majority of our patients (71.6%) have had at least one shot of the vaccine. CONCLUSION: We found that FD patients were more resilient to the negative psychological effects of lockdown. Traumatic growth may be an important factor in explaining this finding. Government-supported home therapy programs might be beneficial for FD patients to increase the therapy adherence.

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.

Coronavirus Pandemic: Mood Statuses of Renal Transplant Recipients During Social Isolation and Lockdown Periods.

OBJECTIVES: In an attempt to control the new coronavirus pandemic, many countries have taken unprecedented measures, such as extensive social distancing and total lockdowns of cities. Kidney transplant recipients have an increased risk for infectious diseases, including viral infections. In this study, we aimed to investigate the effects of population-wide infection control measures on the mood statuses of kidney transplant recipients. MATERIALS AND METHODS: We used an electronic survey tool to collect demographic and sociocultural data. Additionally, in the same survey, we used 2 questionnaires (the Profile of Mood States and the Hospital Anxiety and Depression Scale) to measure mood statuses of kidney transplant recipients. We also examined a control group and selected eligible participants according to age- and sex-based propensity score matching. RESULTS: We analyzed the data of 308 participants: 154 kidney transplant recipients (mean age of 39.9 ± 10.6 years; 57.1% male) and 154 control participants (mean age of 39.1 ± 10.5; 57.1% male). With regard to the Profile of Mood States questionnaire, total scores and all subscale scores, excluding vigor, were significantly lower in kidney transplant recipients than in the control group, a finding consistent with a better mood status. We found similar findings in anxiety and depression scores for the Hospital Anxiety and Depression Scale questionnaire. CONCLUSIONS: Kidney transplant recipients seem to be resilient to the psychological stress induced by social distancing and lockdown periods. Strict adherence to infection control measures is purposefully suggested in this infection-prone population.

The impact of the COVID-19 pandemic on people with diabetes and diabetes services: A pan-European survey of diabetes specialist nurses undertaken by the Foundation of European Nurses in Diabetes survey consortium.

AIM: To describe diabetes nurses' perspectives on the impact of the COVID-19 pandemic on people with diabetes and diabetes services across Europe. METHODS: An online survey developed using a rapid Delphi method. The survey was translated into 17 different languages and disseminated electronically in 27 countries via national diabetes nurse networks. RESULTS: Survey responses from 1829 diabetes nurses were included in the analysis. The responses indicated that 28% (n = 504) and 48% (n = 873) of diabetes nurses felt the COVID-19 pandemic had impacted 'a lot' on the physical and psychological risks of people with diabetes, respectively. The following clinical problems were identified as having increased 'a lot': anxiety 82% (n = 1486); diabetes distress 65% (n = 1189); depression 49% (n = 893); acute hyperglycaemia 39% (n = 710) and foot complications 18% (n = 323). Forty-seven percent (n = 771) of respondents identified that the level of care provided to people with diabetes had declined either extremely or quite severely. Self-management support, diabetes education and psychological support were rated by diabetes nurse respondents as having declined extremely or quite severely during the COVID-19 pandemic by 31% (n = 499), 63% (n = 1,027) and 34% (n = 551), respectively. CONCLUSION: The findings show that diabetes nurses across Europe have seen significant increases in both physical and psychological problems in their patient populations during COVID-19. The data also show that clinical diabetes services have been significantly disrupted. As the COVID-19 situation continues, we need to adapt care systems with some urgency to minimise the impact of the pandemic on the diabetes population.

Kidney function on admission predicts in-hospital mortality in COVID-19.

BACKGROUND: Recent data have suggested the presence of a reciprocal relationship between COVID-19 and kidney function. To date, most studies have focused on the effect of COVID-19 on kidney function, whereas data regarding kidney function on the COVID-19 prognosis is scarce. Therefore, in this study, we aimed to investigate the association between eGFR on admission and the mortality rate of COVID-19. METHODS: We recruited 336 adult consecutive patients (male: 57.1%, mean age: 55.0±16.0 years) that were hospitalized with the diagnosis of COVID-19 in a tertiary care university hospital. Data were collected from the electronic health records of the hospital. On admission, eGFR was calculated using the CKD-EPI formula. Acute kidney injury was defined according to the KDIGO criteria. Binary logistic regression and Cox regression analyses were used to assess the relationship between eGFR on admission and in-hospital mortality of COVID-19. RESULTS: Baseline eGFR was under 60 mL/min/1.73m2 in 61 patients (18.2%). Acute kidney injury occurred in 29.2% of the patients. In-hospital mortality rate was calculated as 12.8%. Age-adjusted and multivariate logistic regression analysis (p: 0.005, odds ratio: 0.974, CI: 0.956-0.992) showed that baseline eGFR was independently associated with mortality. Additionally, age-adjusted Cox regression analysis revealed a higher mortality rate in patients with an eGFR under 60 mL/min/1.73m2. CONCLUSIONS: On admission eGFR seems to be a prognostic marker for mortality in patients with COVID-19. We recommend that eGFR be measured in all patients on admission and used as an additional tool for risk stratification. Close follow-up should be warranted in patients with a reduced eGFR.