Real-world efficacy of single-agent belantamab mafodotin in relapsed systemic AL amyloidosis.

Systemic light chain (AL) amyloidosis is a relapsing plasma cell disorder. Therapy is limited, particularly for triple-class refractory disease. We report the use of belantamab mafodotin, a BCMA-directed drug-antibody conjugate, for relapsed AL amyloidosis, including patients traditionally excluded from clinical trials. Thirty-one patients were reviewed, with a median of three prior lines of therapy. The median follow-up was 12 months (95% CI 4-19), and a median of five doses were delivered. The best haematological overall response rate was 71%, and the complete/very good partial response was 58%. Sixty-eight percent had keratopathy and improved in all. Belantamab mafodotin has high efficacy and good tolerability in patients with relapsed AL amyloidosis.

The persistence of SARS-CoV-2 neutralizing antibodies after COVID-19: A one-year observation. Is a SARS-CoV-2 vaccination booster dose necessary?

Introduction: The aim of this study was to investigate the persistence of SARS-CoV-2 neutralizing antibodies (NAbs) one year after contracting COVID-19. Material and methods: The study included 38 patients - 34 men and 4 women - suffering from COVID-19 between March 15 and May 26, 2020. The median age in the group was 31 years, ranging from 22 to 67 years. The levels of neutralizing antibodies were measured at three time-points - baseline, 6 months, and 12 months. The primary endpoint was a post-infection positive result for NAbs (> 15 AU/ml; Liaison SARS-CoV-2 S1/S2 IgG quantitative test) 12 months after infection. Results: The median level of NAbs after 12 months was 26.5 AU/ml. At the end of observation (12 months), 21 of the 38 patients had a NAb level of >15 AU/ml (positive). The median antibody half-life was 5.8 months. Conclusions: A high percentage of the patients maintained positive levels of antibodies 6 and 12 months after COVID-19 infection. The dynamics of the antibody level decline suggests the need for booster vaccination at least once a year.

Recovery of dialysis patients with COVID-19: health outcomes 3 months after diagnosis in ERACODA.

BACKGROUND: Coronavirus disease 2019 (COVID-19)-related short-term mortality is high in dialysis patients, but longer-term outcomes are largely unknown. We therefore assessed patient recovery in a large cohort of dialysis patients 3 months after their COVID-19 diagnosis. METHODS: We analyzed data on dialysis patients diagnosed with COVID-19 from 1 February 2020 to 31 March 2021 from the European Renal Association COVID-19 Database (ERACODA). The outcomes studied were patient survival, residence and functional and mental health status (estimated by their treating physician) 3 months after COVID-19 diagnosis. Complete follow-up data were available for 854 surviving patients. Patient characteristics associated with recovery were analyzed using logistic regression. RESULTS: In 2449 hemodialysis patients (mean ± SD age 67.5 ± 14.4 years, 62% male), survival probabilities at 3 months after COVID-19 diagnosis were 90% for nonhospitalized patients (n = 1087), 73% for patients admitted to the hospital but not to an intensive care unit (ICU) (n = 1165) and 40% for those admitted to an ICU (n = 197). Patient survival hardly decreased between 28 days and 3 months after COVID-19 diagnosis. At 3 months, 87% functioned at their pre-existent functional and 94% at their pre-existent mental level. Only few of the surviving patients were still admitted to the hospital (0.8-6.3%) or a nursing home (∼5%). A higher age and frailty score at presentation and ICU admission were associated with worse functional outcome. CONCLUSIONS: Mortality between 28 days and 3 months after COVID-19 diagnosis was low and the majority of patients who survived COVID-19 recovered to their pre-existent functional and mental health level at 3 months after diagnosis.

Underdiagnosis of Major Depressive Episodes in Hemodialysis Patients: The Need for Screening and Patient Education.

This article aims to identify the reasons why patients with major depressive episode (MDE) do not seek treatment for their mental disorder. 89 out of 208 persons screened were diagnosed with major depressive episode using the Mini-International Neuropsychiatric Interview. 85 individuals with untreated depression filled out the following questionnaires: Beck Depression Inventory, List of Explanations of Well-Being (LEWB), Brief Measure to Assess Perception of Self-Influence on the Course of the Disease, Coping Inventory for Stressful Situations, Brief Method of Evaluating Coping with Disease, and Metacognitions Questionnaire. There were 43 women (50.6%) and 42 men (49.4%), aged 24 to 93 years (Mean (M) = 68.26 years; Standard Deviation (SD) = 14.19 years), with dialysis vintage ranging from 1 month to 33 years (M = 70.63 months; SD = 75.26 months). Among study patients, 70.6% declared that depression was the cause of their poor well-being, 75.3% attributed their depressive symptoms to kidney failure, and 49.4%, more specifically, to hemodialysis. A total of 64.7% of patients had a low perception of self-influence on the course of their kidney disease, and 58.5% presented a coping style focused on emotions. The most frequent dysfunctional metacognitive beliefs were negative beliefs about not controlling one's own thoughts. This attitude was related to the low perception of self-influence on the course of the disease, maladaptive coping styles, and dysfunctional metacognitive beliefs.

Comparison of Granulomatosis with Polyangiitis Clinical Features in Patients Depending on Involvement or Absence of Kidney Involvement.

BACKGROUND: GPA is necrotizing inflammation of the small vessels with granulomas. Kidney involvement deteriorated its prognosis. OBJECTIVE: Comparison of GPA patients with Kidney (KI) and without Kidney Involvement (nKI). MATERIALS AND METHODS: We conducted a cross-sectional study of 50 consecutive adult GPA patients, 25 KI from Nephrology and 25 nKI from the Rheumatology Department of Central Clinical Hospital Ministry of Interior in Warsaw. We analyzed clinical features, organ involvement, laboratory, serological, imaging, histopathological data, BVAS and treatment. RESULTS: The mean age of KI patients was statistically older than nKI (67.3±9.5 vs. 55.1±15.9, p=0.002). Generalized, severe, disease resistance was observed respectively in 92% vs. 44%, p<0.001. The number of red blood cells (3.47 vs. 4.41T/l, p<0.001), hemoglobin (10.0 vs. 12.9 g/dl, p<0.001) was lower in KI, higher mean serum creatinine (3.95 vs. 0.89 mg/dl, p<0.001), lower GFR (20.1 vs. 79.3, p<0.001), higher CRP (median: 43.4 vs. 2.0 mg/l, p<0.001), BVAS (16.6±4.4 vs. 10.1±6.2, p<0.001), c-ANCA (median: 119.0 vs. 15.2CU, p=0.017). Nodules in 28% KI, in 4% nKI (p=0.048) in chest X-ray, infiltration in 43.5% KI, in 15% nKI (p=0.042) in HRCT were observed. Skin granulomas were found in 61.5% nKI vs. 18.2% KI, (p=0.047). Renal biopsy revealed in KI patients focal segmental glomerulonephritis in 11.8%, crescentic glomerulonephritis in 17.6%, and pauci-immune crescentic glomerulonephritis in 70.6%. CONCLUSION: In patients with KI we found generalized, severe, resistant GPA, higher BVAS more frequently than inpatients without KI. The results of laboratory parameters were worse in patients with KI. Aggressive immunosuppressive treatment is often used in the KI group.

Convalescent plasma treatment is associated with lower mortality and better outcomes in high-risk COVID-19 patients - propensity-score matched case-control study.

OBJECTIVE: This study aimed to investigate the efficacy and safety of convalescent plasma (CP) transfusion in a group of high-risk COVID-19 patients. METHODS: This prospective study included 204 patients from a single tertiary-care hospital, hospitalized with COVID-19, of whom 102 were treated with CP administration and standard care (PG) and 102 others who received standard care only (CG). The CG was selected from 336 hospitalized patients using the propensity-score matching (PSM) technique using age, MEWS score, and comorbidities. The primary outcome was mortality rate; secondary outcomes were the requirement of a ventilator, length of ventilator need, length of intensive care unit (ICU) stay, and length of overall hospital confinement. Additionally, parameters predicting death in COVID-19 patients were identified. RESULTS: Findings confirmed a significantly lower mortality rate in the PG versus the CG (13.7% vs. 34.3 %, p = 0.001) and a significant difference in the cumulative incidence of death between the two groups (p < 0.001). CP treatment was associated with lower risk of death (OR = 0.25 CI95 [0.06; 0.91], p = 0.041). There were no significant differences in ICU stay, ventilator time, and hospitalization time between the two groups. CONCLUSIONS: A significantly lower mortality rate was observed in the group of patients treated with CP. Age, presence of cardiac insufficiency, active cancer, a ventilator requirement, and length of hospitalization significantly increased the risk of death in both groups. Our study shows that CP affords better outcomes when administrated in the earlier stage of high-risk COVID-19 disease.

ANCA-associated vasculitis patients treated in Polish intensive care units - retrospective characteristics based on the POLVAS registry.

INTRODUCTION: ANCA-associated vasculitides (AAV) is a group of rare disorders where inflammation and damage of the small blood vessels lead to dysfunction of the supplied organs. In severe flares of the disease patients may require intensive care unit (ICU) admission and treatment. The study aims to characterize Polish patients with AAV who were admitted to the ICU and compare them to the others. MATERIAL AND METHODS: An observational, retrospective study based on the POLVAS - registry of Polish adult patients with AAV was carried out. Patients admitted to the ICU (ICU group) were identified and compared with the patients who did not require ICU admission (non-ICU group). Characteristics and comparison between groups were made using standard statistic descriptive methods. RESULTS: 30 patients admitted to the ICU were identified among 573 cases included in the registry. All patients in the ICU group with available data were ANCA positive. The clinical manifestations related to the ICU admission were respiratory, renal and central nervous system involvement. The treatment regimen for remission induction was similar in both groups. Almost half of the patients in the ICU-group (48.3%) required dialysis, whereas in the non-ICU group it was 21.8% (P = 0.01). Infections were also more frequent in the ICU group (72.4% vs. 36.9% P < 0.001). The mortality rate among patients who needed ICU treatment was significantly higher when compared to the rest of the patients (53.6% vs. 7.8%; P < 0.001). CONCLUSIONS: In the Polish AAV cohort one in twenty patients required ICU admission. This group was characterized by multiple organ involvement and high mortality.

FRAX prognostic and intervention thresholds in the management of major bone fractures in hemodialysis patients: A two-year prospective multicenter cohort study.

PURPOSE: The usefulness of FRAX in predicting major bone fractures in patients with end-stage kidney disease on maintenance hemodialysis treatment has been confirmed in previous studies. For meaningful clinical use, the prognostic and intervention FRAX thresholds need to be established. METHODS: The primary aim of our study was to calculate the optimal cut-off point of FRAX for the best prediction of an increased bone fracture risk in dialysis patients and additionally, to propose its intervention threshold, indicating the need for antifracture pharmacological treatment. The study included 718 hemodialysis patients, who were followed up for two years. Thirty low-energy major bone fractures were diagnosed during the study period. We used the Polish version of FRAX (without the DXA examination) and some particular variables of the FRAX calculator. The optimal cut-off point for prediction of an increased major bone fracture risk was based on the analysis of the sensitivity and specificity curves of FRAX. RESULTS: The analysis revealed FRAX >5% (sensitivity of 70.0%, specificity of 69.8%) as the prognostic threshold for major bone fractures. Its sensitivity for bone fracture prediction was significantly higher, but specificity lower than those of FRAX ≥10%, used in general Polish population. The reason for this can be an underestimation of bone fracture risk with FRAX in dialysis patients. CONCLUSIONS: We conclude that the FRAX prognostic threshold for identification of an increased risk of major bone fractures in hemodialysis patients is >5%. We propose to use this specific value of FRAX as an intervention threshold for pharmacological antifracture treatment in hemodialysis patients.

Clinicopathologic correlations of renal pathology in the adult population of Poland.

BACKGROUND: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. METHODS: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. RESULTS: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014. A considerable variability between provinces in the mean number of biopsies performed in the period covered was found, ranging from 5 to 77 PMP/year. The most common renal biopsy diagnoses in adults were immunoglobulin A nephropathy (IgAN) (20%), focal segmental glomerulosclerosis (FSGS) (15%) and membranous glomerulonephritis (MGN) (11%), whereas in children, minimal change disease (22%), IgAN (20%) and FSGS (10%) were dominant. Due to insufficient data on the paediatric population, the clinicopathologic analysis was limited to patients ≥18 years of age. At the time of renal biopsy, the majority of adult patients presented nephrotic-range proteinuria (45.2%), followed by urinary abnormalities (38.3%), nephritic syndrome (13.8%) and isolated haematuria (1.7%). Among nephrotic patients, primary glomerulopathies dominated (67.6% in those 18-64 years of age and 62.4% in elderly patients) with leading diagnoses being MGN (17.1%), FSGS (16.2%) and IgAN (13.0%) in the younger cohort and MGN (23.5%), amyloidosis (18.8%) and FSGS (16.8%) in the elderly cohort. Among nephritic patients 18-64 years of age, the majority (55.9%) suffered from primary glomerulopathies, with a predominance of IgAN (31.3%), FSGS (12.7%) and crescentic GN (CGN) (11.1%). Among elderly nephritic patients, primary and secondary glomerulopathies were equally common (41.9% each) and pauci-immune GN (24.7%), CGN (20.4%) and IgAN (14.0%) were predominant. In both adult cohorts, urinary abnormalities were mostly related to primary glomerulopathies (66.8% in younger and 50% in elderly patients) and the leading diagnoses were IgAN (31.4%), FSGS (15.9%), lupus nephritis (10.7%) and FSGS (19.2%), MGN (15.1%) and pauci-immune GN (12.3%), respectively. There were significant differences in clinical characteristics and renal biopsy findings between male and female adult patients. CONCLUSIONS: The registry data focused new light on the epidemiology of kidney diseases in Poland. These data should be used in future follow-up and prospective studies.

Soluble urokinase-type plasminogen activator receptor (suPAR) in patients with acute pancreatitis (AP) - Progress in prediction of AP severity.

BACKGROUND: Soluble urokinase-type plasminogen activator receptor (suPAR) is a glycoprotein secreted during inflammation and infections. Moreover, increased levels of suPAR are observed after hypoxia and ischaemia. The aim of the study was to assess whether suPAR could represent a useful marker of acute pancreatitis (AP) severity. PATIENTS AND METHODS: We have observed a cohort of 126 prospectively enrolled patients. Based on the presence of persistent organ failure (more than 48 h) and local complications (diagnosis of moderate AP [MSAP]), patients were classified into three groups: mild AP (MAP), moderate and severe AP (SAP). The blood samples were taken on admission for detecting suPAR concentrations. RESULTS: AP was considered severe in 33 patients (26.2%), MSAP was found in 37 patients (29.4%), and MAP was found in 56 patients (44,4%). The AUC for SAP predicted by suPAR was 0.993. The calculated cut-off point for prognosis SAP is 4.75 ng/mL. The BISAP score of ≥3 for detection of SAP had sensitivity and specificity of 94.6% and 63.6%, respectively. The AUC for severity predicted by BISAP amounted to 0.916. Additionally, suPAR turned out to be a good predictor of fatal AP: for the cut-off point 7.05 ng/mL, the AUC was 0.917. The AUC for death prediction in AP patients based on the BISAP score ≥3 was 0.894. CONCLUSIONS: suPAR concentration is a promising new diagnostic and prognostic indicator in SAP obtainable in the early stage of disease. Larger studies are recommended to evaluate this role further.

[Brief measure to assess patient perception of self-influence on the disease course-version for hemodialysis]. / Krótka metoda oceny poczucia wplywu pacjenta na przebieg choroby--wersja dla hemodializowanych.

INTRODUCTION: Self-influence on the disease course has a significant impact on coping with disease and adherence to medical recommendations. AIM: Elaboration of a brief scale for screening of patient perception of self-influence on the course of disease among hemodialysis, which could be used during usual medical practice. METHODS: In the first stage of the study, based on a group focus interview with 6 hemodialysis (mean age 65.2 ± 14.8 year)--5 women (83%) and 1 men (17%), mean time of dialysis 43.8 ± 38 months, a list of 51 characteristics of patients with low (LP) and high perception (HP) of self-influence on the curse of disease, was constructed. In the second stage 99 patients (mean age 66.1 ± 14.7 year)--51 men (52%) and 48 women (48%), mean time of dialysis 48.7 ± 48.1 months, assessed scale reliability. They were selected by their nephrologists to groups with LP and HP. Based on the results 11 items had the strongest power of discrimination of those two groups. 15 (13.2%) hemodialysis refused to participate at this part of study. In a third step 70 (mean age 68 ± 13.3 year) patients--40 men (57.1%) and 30 women (42.9%), mean time of dialysis 48.1 ± 45.6 months, assessed validity of the tool. RESULTS: The reliability alfa-Cronbach = 0.9 and validity tau-Kendall = 0.6. CONCLUSIONS: Scale has a very high reliability and satisfactory validity. It can be used for those of hemodialysis who have problems with adherence to medical recommendations or have difficulty in contact with the medical staff or other patients.

Fluid resuscitation in acute pancreatitis: Normal saline or lactated Ringer's solution?

AIM: To investigate whether administration of Ringer's solution (RL) could have an impact on the outcome of acute pancreatitis (AP). METHODS: We conducted a retrospective study on 103 patients [68 men and 35 women, mean age 51.2 years (range, 19-92 years)] hospitalized between 2011 and 2012. All patients admitted to the Department of Gastroenterology of the Central Clinical Hospital of the Ministry of Interior (Poland) with a diagnosis of AP who had disease onset within 48 h of presentation were included in this study. Based on the presence of persistent organ failure (longer than 48 h) as a criterion for the diagnosis of severe AP (SAP) and the presence of local complications [diagnosis of moderately severe AP (MSAP)], patients were classified into 3 groups: mild AP (MAP), MSAP and SAP. Data were compared between the groups in terms of severity (using the revised Atlanta criteria) and outcome. Patients were stratified into 2 groups based on the type of fluid resuscitation: the 1-RL group who underwent standard fluid resuscitation with a RL 1000 mL solution or the 2-NS group who underwent standard fluid resuscitation with 1000 mL normal saline (NS). All patients from both groups received an additional 5% glucose solution (1000-1500 mL) and a multi-electrolyte solution (500-1000 mL). RESULTS: We observed 64 (62.1%) patients with MAP, 26 (25.24%) patients with MSAP and 13 (12.62%) patients with SAP. No significant difference in the distribution of AP severity between the two groups was found. In the 1-RL group, we identified 22 (55.5%) MAP, 10 (25.5%) MSAP and 8 (20.0%) SAP patients, compared with 42 (66.7%) MAP, 16 (24.4%) MSAP and 5 (7.9%) SAP cases in the 2-NS group (P = 0.187). The volumes of fluid administered during the initial 72-h period of hospitalization were similar among the patients from both the 1-RL and 2-NS groups (mean 3400 mL vs 3000 mL, respectively). No significant differences between the 1-RL and 2-NS groups were found in confirmed pancreatic necrosis [10 patients (25%) vs 12 patients (19%), respectively, P = 0.637]. There were no statistically significant differences between the 1-RL and 2-NS groups in the percentage of patients who required enteral nutrition (23 patients vs 17 patients, respectively, P = 0.534). Logistic regression analysis confirmed these findings (OR = 1.344, 95%CI: 0.595-3.035, P = 0.477). There were no significant differences between the 1-RL and 2-NS groups in mortality and the duration of hospital stay (median of 9 d for both groups, P = 0.776). CONCLUSION: Our study failed to find any evidence that the administration of RL in the first days of AP leads to improved clinical outcomes.

Urinary neutrophil gelatinase-associated lipocalin as an early predictor of disease severity and mortality in acute pancreatitis.

OBJECTIVES: In reference to our earlier publication, laboratory tests that reflect severe intravascular volume depletion can be used for predicting the severity of acute pancreatitis (AP). The aim of the study was to assess whether urinary level of neutrophil gelatinase-associated lipocalin (NGAL) could represent a useful marker of AP severity. METHODS: We observed a cohort of 104 prospectively enrolled patients. The patients were classified into 3 groups: mild AP, moderately severe AP, and severe AP. Urine samples were collected on admission (NGAL-as) and during the first 24 hours (NGAL-first day) for examination of urinary level of NGAL concentrations from the first day. RESULTS: Acute pancreatitis was considered severe in 16 (15%) patients, moderately severe in 25 (24%) patients, and mild in 63 (61%) patients.There were statistically significant trends for an increase in severity (P = 0.04, P = 0.003) and mortality (P < 0.031, P = 0.01) with raising NGAL-as and NGAL-first day concentrations, respectively. The areas under the curve for severity predicted by NGAL-as and NGAL-first day were 0.75 and 0.93, respectively. The areas under the curve for mortality prediction by NGAL-as and NGAL-first day were 0.980 and 0.92, respectively. CONCLUSIONS: The urinary level of NGAL is a promising new diagnostic and prognostic factor for severe AP in an early stage of the disease.