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.

Post-COVID-19 outcomes of non-dialysis dependent chronic kidney disease patients: a national, multicenter, controlled study.

PURPOSE: Coronavirus disease 2019 (COVID-19) has a higher mortality in the presence of chronic kidney disease (CKD). However, there has not been much research in the literature concerning the outcomes of CKD patients in the post-COVID-19 period. We aimed to investigate the outcomes of CKD patients not receiving renal replacement therapy. METHODS: In this multicenter observational study, we included CKD patients with a GFR < 60 ml/min/1.73 m2 who survived after confirmed COVID-19. Patients with CKD whose kidney disease was due to diabetic nephropathy, polycystic kidney disease and glomerulonephritis were not included in this study. CKD patients with similar characteristics, who did not have COVID-19 were included as the control group. RESULTS: There were 173 patients in the COVID-19 group and 207 patients in the control group. Most patients (72.8%) were treated as inpatient in the COVID-19 group (intensive care unit hospitalization: 16.7%, acute kidney injury: 54.8%, needing dialysis: 7.9%). While there was no significant difference between the baseline creatinine values of the COVID-19 group and the control group (1.86 and 1.9, p = 0.978, respectively), on the 1st month, creatinine values were significantly higher in the COVID-19 group (2.09 and 1.8, respectively, p = 0.028). Respiratory system symptoms were more common in COVID-19 patients compared to the control group in the 1st month and 3rd month follow-ups (p < 0.001). Mortality at 3 months after the diagnosis of COVID-19 was significantly higher in the COVID-19 group than in the control group (respectively; 5.2% and 1.4%, p:0.037). Similarly, the rate of patients requiring dialysis for COVID-19 was significantly higher than the control group (respectively; 8.1% and 3.4%, p: 0.045). CONCLUSIONS: In CKD patients, COVID-19 was associated with increased mortality, as well as more deterioration in kidney function and higher need for dialysis in the post-COVID-19 period. These patients also had higher rate of ongoing respiratory symptoms after COVID-19.

Favipiravir therapy for Covid-19 infection and tacrolimus toxicity in a kidney transplant patient on chronic eculizumab therapy.

Although the latest data show that complement activation has an essential role in the pathogenesis and severity of Covid-19, the data on the prognosis of patients using complement inhibitors during Covid-19 infection are scarce. There is no specific treatment for Covid-19 yet. The introduction of novel agents such as favipiravir may affect metabolism of immunosuppressive drugs. We report the clinical course of Covid-19 in a kidney transplant patient with atypical haemolytic uraemic syndrome on chronic eculizumab therapy. The patient had mild Covid-19 but had severe tacrolimus toxicity, which may be associated with favipiravir and eculizumab. The mild course of Covid-19 in our patient is encouraging for eculizumab use; on the other hand, unusually high levels of tacrolimus that we observed underlines the importance of frequent drug level monitoring in transplanted patients who are receiving new drugs.

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.

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.

Middle-term outcomes in renal transplant recipients with COVID-19: a national, multicenter, controlled study.

Background: In this study, we evaluated 3-month clinical outcomes of kidney transplant recipients (KTR) recovering from COVID-19 and compared them with a control group. Method: The primary endpoint was death in the third month. Secondary endpoints were ongoing respiratory symptoms, need for home oxygen therapy, rehospitalization for any reason, lower respiratory tract infection, urinary tract infection, biopsy-proven acute rejection, venous/arterial thromboembolic event, cytomegalovirus (CMV) infection/disease and BK viruria/viremia at 3 months. Results: A total of 944 KTR from 29 different centers were included in this study (523 patients in the COVID-19 group; 421 patients in the control group). The mean age was 46 ± 12 years (interquartile range 37-55) and 532 (56.4%) of them were male. Total number of deaths was 8 [7 (1.3%) in COVID-19 group, 1 (0.2%) in control group; P = 0.082]. The proportion of patients with ongoing respiratory symptoms [43 (8.2%) versus 4 (1.0%); P < 0.001] was statistically significantly higher in the COVID-19 group compared with the control group. There was no significant difference between the two groups in terms of other secondary endpoints. Conclusion: The prevalence of ongoing respiratory symptoms increased in the first 3 months post-COVID in KTRs who have recovered from COVID-19, but mortality was not significantly different.

How Dialysis Patients Cope with a Curfew? A Comparison of Psychological Status between Hemodialysis and Peritoneal Dialysis Patients During the COVID-19 Pandemic.

INTRODUCTION: There are many differences between hemodialysis (HD) and peritoneal dialysis (PD) treatments, including their impact on the psychological status of the patients. In this study, our aim was to compare the psychological statuses of HD and PD patients during the social isolation period due to the COVID-19 pandemic. METHODS: We conducted this cross-sectional study on adult HD and PD patients when the curfew measures were in effect. We used an electronic form composed of 3 sections to collect data. In the first section, we collected data on the demographics and clinical and laboratory parameters of the patients. The second and third sections consisted of the Hospital Anxiety and Depression Scale (HADS) and the Impact of Event Scale-Revised (IES-R) questionnaires, respectively. RESULTS: The HD (n = 116) and PD (n = 130) groups were similar regarding age and sex, and they had similar HADS anxiety scores. HADS depression scores were higher in PD patients (p = 0.052). IES-R scores were significantly higher in PD patients in comparison to HD patients (p = 0.001). Frequencies of abnormal HADS-anxiety (p = 0.035) and severe psychological impact (p = 0.001) were significantly higher in PD patients. DISCUSSION/CONCLUSION: During the social isolation period due to the COVID-19 pandemic, HD patients had better mood profiles than PD patients. A more stable daily routine, an uninterrupted face-to-face contact with health-care workers, and social support among patients in the in-center dialysis environment might be the cause of the favorable mood status. PD patients might need additional psychological support during those periods.

Characteristics and outcomes of acute kidney injury in hospitalized COVID-19 patients: A multicenter study by the Turkish society of nephrology.

BACKGROUND: Acute kidney injury (AKI) is common in coronavirus disease-2019 (COVID-19) and the severity of AKI is linked to adverse outcomes. In this study, we investigated the factors associated with in-hospital outcomes among hospitalized patients with COVID-19 and AKI. METHODS: In this multicenter retrospective observational study, we evaluated the characteristics and in-hospital renal and patient outcomes of 578 patients with confirmed COVID-19 and AKI. Data were collected from 34 hospitals in Turkey from March 11 to June 30, 2020. AKI definition and staging were based on the Kidney Disease Improving Global Outcomes criteria. Patients with end-stage kidney disease or with a kidney transplant were excluded. Renal outcomes were identified only in discharged patients. RESULTS: The median age of the patients was 69 years, and 60.9% were males. The most frequent comorbid conditions were hypertension (70.5%), diabetes mellitus (43.8%), and chronic kidney disease (CKD) (37.6%). The proportions of AKI stages 1, 2, and 3 were 54.0%, 24.7%, and 21.3%, respectively. 291 patients (50.3%) were admitted to the intensive care unit. Renal improvement was complete in 81.7% and partial in 17.2% of the patients who were discharged. Renal outcomes were worse in patients with AKI stage 3 or baseline CKD. The overall in-hospital mortality in patients with AKI was 38.9%. In-hospital mortality rate was not different in patients with preexisting non-dialysis CKD compared to patients without CKD (34.4 versus 34.0%, p = 0.924). By multivariate Cox regression analysis, age (hazard ratio [HR] [95% confidence interval (95%CI)]: 1.01 [1.0-1.03], p = 0.035], male gender (HR [95%CI]: 1.47 [1.04-2.09], p = 0.029), diabetes mellitus (HR [95%CI]: 1.51 [1.06-2.17], p = 0.022) and cerebrovascular disease (HR [95%CI]: 1.82 [1.08-3.07], p = 0.023), serum lactate dehydrogenase (greater than two-fold increase) (HR [95%CI]: 1.55 [1.05-2.30], p = 0.027) and AKI stage 2 (HR [95%CI]: 1.98 [1.25-3.14], p = 0.003) and stage 3 (HR [95%CI]: 2.25 [1.44-3.51], p = 0.0001) were independent predictors of in-hospital mortality. CONCLUSIONS: Advanced-stage AKI is associated with extremely high mortality among hospitalized COVID-19 patients. Age, male gender, comorbidities, which are risk factors for mortality in patients with COVID-19 in the general population, are also related to in-hospital mortality in patients with AKI. However, preexisting non-dialysis CKD did not increase in-hospital mortality rate among AKI patients. Renal problems continue in a significant portion of the patients who were discharged.

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.

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.