Successful treatment with daratumumab of a patient with monoclonal lambda light chain disease presenting as nephrotic syndrome and crescentic glomerulonephritis.

Monoclonal immunoglobulin deposition disease (MIDD) are a group of systemic diseases, characterized by deposition of monoclonal immunoglobulin predominantly in the kidney. In the absence of overt hematologic disease, MIDD are classified as a part of monoclonal gammopathy of renal significance. Patients with MIDD may present with a nephrotic syndrome and kidney function impairment. Treatment usually include anti-plasma cell therapy. Here we report a case of a 54-year-old female who presented with nephrotic syndrome related to light chain deposition disease of lambda type. Due to a complicated clinical course (including cardiac injury and thromboembolic stroke), plasma-cell targeted therapy was stopped. A few months later, the patient presented with severe acute kidney injury. Kidney biopsy revealed crescentic glomerulonephritis, and immunofluorescence staining was positive for lambda chain. Treatment with daratumumab was initiated resulting in stabilization of kidney function and partial nephrotic syndrome remission.

Clinical presentation and outcome of hemodialysis tunneled catheter-related bloodstream infection in older persons.

BACKGROUND: The use of a tunneled catheter as the primary vascular access among old hemodialysis patients is frequent. Catheter-related bloodstream infection (CRBSI) is a common complication, associated with increased mortality. Data regarding the clinical presentation and outcomes of CRBSI among old hemodialysis patients is limited. METHODS: All chronic hemodialysis patients hospitalized between 2010 and 2022 with CRBSI were included. Patients were classified into two groups: old adults (≥ 75) and younger patients. Clinical, microbiological, and outcome data were collected and analyzed. RESULTS: One hundred and fifty-four patients with CRBSI were identified. Fifty-seven were aged ≥ 75 years. Mean age in the older and younger groups was 81.2 ± 5 and 59.7 ± 12.7, respectively. Male gender was predominant (64%). Charlson comorbidity score and Pitt bacteremia score were comparable among both groups. Norton score < 14 was more common among old persons (n = 24, 67% versus n = 21, 31%, p < 0.001), as well as nursing-home residence. Gram-negative pathogens and Staphylococcus aureus were common in both groups. The frequency of inappropriate empirical antimicrobial treatment was higher among older persons. Overall, in-hospital and 90-day mortality was high (age ≥ 75, 36.8%, age < 75, 24.7%, p = 0.14). Age was not significantly associated with mortality after adjustment for low Norton score, residence, and inappropriate antimicrobial therapy as well as resistance patterns of bloodstream isolates [OR = 1.2 (95% CI 0.4-3.3), p = 0.76]. CONCLUSIONS: Clinical characteristics and outcomes of CRBSI were comparable among old and young hemodialysis patients. However, the high mortality rate in this cohort suggests that the use of tunneled catheters as a permanent vascular access should be discouraged in both patient groups.

Pseudohyperkalemia in chronic lymphocytic leukemia: Prevalence, impact, and management challenges.

The term pseudohyperkalemia refers to a false elevation in serum potassium levels due to potassium release from cells in vitro. Falsely elevated potassium levels have been reported in patients with thrombocytosis, leukocytosis, and hematologic malignancies. This phenomenon has been particularly described in chronic lymphocytic leukemia (CLL). Leukocyte fragility, extremely high leukocyte counts, mechanical stress, higher cell membrane permeability related to an interaction with lithium heparin in plasma blood samples, and metabolite depletion due to a high leukocyte burden have been reported to contribute to pseudohyperkalemia in CLL. The prevalence of pseudohyperkalemia is up to 40%, particularly in the presence of a high leukocyte count (>50 × 109/L). The diagnosis of pseudohyperkalemia is often overlooked, which may result in unnecessary and potentially harmful treatment. The use of whole blood testing and point-of-care blood gas analysis, along with thorough clinical evaluation, may help differentiate between true and pseudohyperkalemic episodes.

Administration of Intravenous Furosemide in Patients with Acute Infection: Patient Characteristics and Impact on In-Hospital Outcome.

Intravenous (IV) fluid is frequently used to treat patients who have been admitted with an acute infection; among these patients, some will experience pulmonary congestion and will need diuretic treatment. Consecutive admissions to the Internal Medicine Department of patients with an acute infection were included. Patients were divided based on IV furosemide treatment within 48 h after admission. A total of 3556 admissions were included: In 1096 (30.8%), furosemide was administered after ≥48 h, and in 2639 (74.2%), IV fluid was administered within <48 h. Mean age was 77.2 ± 15.8 years, and 1802 (50.7%) admissions were females. In a multivariable analysis, older age (OR 1.01 [95% CI, 1.00-1.01]), male gender (OR 0.74 [95% CI, 0.63-0.86]), any cardiovascular disease (OR 1.51 [95% CI, 1.23-1.85]), congestive heart failure (CHF) (OR 2.81 [95% CI, 2.33-3.39), hypertension (OR 1.42 [95% CI, 1.22-1.67]), respiratory infection (OR 1.38 [95% CI, 1.17-1.63]), and any IV fluid administration (OR 3.37 [95% CI, 2.80-4.06]) were independently associated with furosemide treatment >48 h after hospital admission. In-hospital mortality was higher in patients with furosemide treatment (15.9% vs. 6.8%, p < 0.001). Treatment with furosemide in patients admitted with an infection was found to be associated with prolonged hospital stay and increased in-hospital mortality.

Dialysis modality, humoral response to vaccine, and SARS-CoV-2 infection risk: Comparative prospective evaluation.

BACKGROUND: COVID-19 vaccinations have a central role in decreasing severe SARS-CoV-2 disease complications. This study investigated the long-term humoral immune response to BNT162b2 vaccine among hemodialysis (HD) versus peritoneal dialysis (PD) patients, and their relative risk for COVID-19 infection. METHODS: This prospective, observational study included maintenance HD and PD patients who had received at least two BNT162b2 vaccine doses. Levels of antibodies targeting SARS-CoV-2 spike protein were measured 6 and 12 months after the first vaccine dose, and 2-3 weeks after the third and fourth vaccine doses. Patients were divided according to dialysis modality (HD or PD). Humoral response was evaluated at different time points among different vaccine regimens (two vs. three vs. four doses of vaccine). An adjusted multivariate model was used to assess cumulative risk for SARS-CoV-2 infection. RESULTS: Eighty-seven HD and 36 PD patients were included. Among them, 106 (86%) received at least three vaccine doses. Both HD and PD patients demonstrated marked increases in humoral response 2-3 weeks after the third dose (mean anti-S antibody increased from 452 ± 501 AU/mL to 19,556 ± 14,949 AU/mL, p < 0.001). By 6 months after the third dose, antibody titers had declined significantly (mean anti-S antibody 9841 ± 10,493 AU/mL, p < 0.001). HD patients had higher risk for SARS-CoV-2 infection than PD patients (OR 4.4 [95% CI 1.4-13.6], p = 0.006). In multivariate analysis, the most important predictor for SARS-CoV-2 infection was dialysis modality. CONCLUSION: This study found a high antibody response rate after the third and fourth doses of BNT162b2 vaccine among dialysis patients. Hemodialysis as dialysis modality is an important predictor of COVID-19 infection, despite similar humoral responses to vaccine in peritoneal dialysis.

Acute Kidney Injury and Hair-Straightening Products: A Case Series.

RATIONALE & OBJECTIVE: Keratin-based hair-straightening treatment is a popular hair-styling method. The majority of keratin-based hair-straightening products in Israel contain glycolic acid derivatives, which are considered safe when used topically. Systemic absorption of these products is possible, and anecdotal reports have described kidney toxicity associated with their use. We report a series of cases of severe acute kidney injury (AKI) following use of hair-straightening treatment in Israel during the past several years. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: We retrospectively identified 26 patients from 14 medical centers in Israel who experienced severe AKI and reported prior treatment with hair-straightening products in 2019-2022. FINDINGS: The 26 patients described had a median age of 28.5 (range, 14-58) years and experienced severe AKI following a hair-straightening procedure. The most common symptoms at presentation were nausea, vomiting, and abdominal pain. Scalp rash was noted in 10 (38%) patients. Two patients experienced a recurrent episode of AKI following a repeat hair-straightening treatment. Seven patients underwent kidney biopsies, which demonstrated intratubular calcium oxalate deposition in 6 and microcalcification in tubular cells in 1. In all biopsies, signs of acute tubular injury were present, and an interstitial infiltrate was noted in 4 cases. Three patients required temporary dialysis. LIMITATIONS: Retrospective uncontrolled study, small number of kidney biopsies. CONCLUSIONS: This series describes cases of AKI with prior exposure to hair-straightening treatments. Acute oxalate nephropathy was the dominant finding on kidney biopsies, which may be related to absorption of glycolic acid derivatives and their metabolism to oxalate. This case series suggests a potential underrecognized cause of AKI in the young healthy population. Further studies are needed to confirm this association and to assess the extent of this phenomenon as well as its pathogenesis.

An unexpected cause of colitis.

Gastrointestinal manifestations are common among patients with advanced kidney disease. Common symptoms include poor appetite, nausea, and vomiting. Prevalent lesions include esophagitis, gastritis, and duodenitis. Uremia-associated colitis is extremely rare. In this case report we present a young patient who present with end-stage kidney disease of unknown origin accompanied by abdominal pain and vomiting. Computed tomography showed severe bowel wall thickening of the colon. Due to extreme uremic state uremia-associated colitis was suspected and hemodialysis was initiated immediately, resulted in clinical and radiology improvement.

Humoral Response and SARS-CoV-2 Infection Risk following the Third and Fourth Doses of the BNT162b2 Vaccine in Dialysis Patients.

The optimal SARS-CoV-2 vaccination schedule in dialysis patients and the potential need for a fourth vaccine dose are debatable. We prospectively assessed the humoral responses to three and four doses of BNT162b2 among dialysis patients. The study included 106 dialysis patients; 60 (56.6%) and 46 (43.4%) received 3 and 4 vaccine doses, respectively. Anti-spike (anti-S) antibody titers significantly increased after the third vaccine dose, followed by a decline, yet still remained higher than all previous measurements. The fourth vaccine dose led to another profound rise in anti-S titers. The absolute increase following the fourth dose correlated with response to the third dose. Infection risk however was similar between patients vaccinated with three or four doses.

Metabolic alkalosis in infants treated with peritoneal dialysis.

BACKGROUND: Acid-base balance is maintained by kidney excretion of titratable acids and bicarbonate reabsorption. Metabolic alkalosis is uncommon in dialysis-treated patients. The aim of this retrospective study was to assess the rate of metabolic alkalosis in pediatric patients treated with peritoneal dialysis. METHODS: Medical records of children treated with peritoneal dialysis in Shaare Zedek Medical Center from January 2000 to June 2021 were reviewed and compared with young adults currently treated with peritoneal dialysis. Demographic, clinical, and peritoneal dialysis characteristics were extracted from the medical records. RESULTS: Thirty chronic peritoneal dialysis patients were included in our study, seven under 2 years, 13 between 2 and 18 years, and 10 adults. 90.3% of the measurements in infants showed metabolic alkalosis compared to 32.3% in the 2-18-year group and none in the adult group. Higher size-adjusted daily exchange volume, lack of urine output, and high lactate-containing dialysate were associated with metabolic alkalosis. Alkalosis was not explained by vomiting, diuretic therapy, or carbonate-containing medications. High transport membrane, low dietary protein, and malnutrition, all previously reported explanations for metabolic alkalosis, were not found in our study. CONCLUSIONS: Metabolic alkalosis is common in infants treated with peritoneal dialysis as opposed to older children and adults. High lactate-containing dialysate is a possible explanation. Higher size-adjusted daily dialysate exchange volume, which may reflect higher bicarbonate absorption, is another independent predictor of alkalosis. Acid-base status should be closely followed in infants, and using a dialysis solution with lower bicarbonate or lactate level should be considered. A higher resolution version of the graphical abstract is available as Supplementary Information.

Prospective determination of the incidence and severity of hyponatraemia in older hospitalised patients with acute urinary tract obstruction.

INTRODUCTION: acute urinary tract obstruction (aUTO) is a common finding in older hospitalised patients. Anecdotal reports described hyponatraemia in patients with aUTO, which subsides rapidly with relief of the obstruction.The aim of this study was to determine the incidence, severity and subsequent correction of hyponatraemia in patients with aUTO. METHODS: this is a prospective, single-centre, observational study including inpatients in the internal medicine and geriatric wards. A total of 204 patients were investigated, 104 with aUTO and 100 controls. The prevalence, severity and associations of hyponatraemia between aUTO and control patients were compared. RESULTS: the incidence of hyponatraemia was similar in aUTO and control groups 28% versus 22%, respectively (P = 0.42). However, the incidence of severe hyponatraemia was significantly higher in the study group 7% versus 1% (P = 0.04). Mean sodium level was lower in hyponatremic patients with aUTO 127.7 ± 5.9 mEq/l versus 130.8 ± 3 mEq/l (P = 0.013). Serum sodium remained largely unchanged in the control group (∆Na 1.5 ± 2.3 mEq/l) but increased significantly within 48 hours following catheter insertion in patients with urinary retention (∆Na 5.3 ± 4.2 mEq/l overall in the aUTO group and 9.6 ± 3 mEq/l in those with urinary retention and severe hyponatraemia (P = 0.002)).

A randomized multicenter trial on a lung ultrasound-guided treatment strategy in patients on chronic hemodialysis with high cardiovascular risk.

Lung congestion is a risk factor for all-cause and cardiovascular mortality in patients on chronic hemodialysis, and its estimation by ultrasound may be useful to guide ultrafiltration and drug therapy in this population. In an international, multi-center randomized controlled trial (NCT02310061) we investigated whether a lung ultrasound-guided treatment strategy improved a composite end point (all-cause death, non-fatal myocardial infarction, decompensated heart failure) vs usual care in patients receiving chronic hemodialysis with high cardiovascular risk. Patient-Reported Outcomes (Depression and the Standard Form 36 Quality of Life Questionnaire, SF36) were assessed as secondary outcomes. A total of 367 patients were enrolled: 183 in the active arm and 180 in the control arm. In the active arm, the pre-dialysis lung scan was used to titrate ultrafiltration during dialysis and drug treatment. Three hundred and seven patients completed the study: 152 in the active arm and 155 in the control arm. During a mean follow-up of 1.49 years, lung congestion was significantly more frequently relieved in the active (78%) than in the control (56%) arm and the intervention was safe. The primary composite end point did not significantly differ between the two study arms (Hazard Ratio 0.88; 95% Confidence Interval: 0.63-1.24). The risk for all-cause and cardiovascular hospitalization and the changes of left ventricular mass and function did not differ among the two groups. A post hoc analysis for recurrent episodes of decompensated heart failure (0.37; 0.15-0.93) and cardiovascular events (0.63; 0.41-0.97) showed a risk reduction for these outcomes in the active arm. There were no differences in patient-reported outcomes between groups. Thus, in patients on chronic hemodialysis with high cardiovascular risk, a treatment strategy guided by lung ultrasound effectively relieved lung congestion but was not more effective than usual care in improving the primary or secondary end points of the trial.