The spectrum of thrombotic microangiopathy related to monoclonal gammopathy.

Background: Recent studies showed a high prevalence of monoclonal gammopathy (MG) in patients with thrombotic microangiopathy (TMA) aged over 50 years and suggested that complement dysregulation is pivotal for the disease to develop. Here, we studied this premise in seven patients with TMA and coexisting MG. Methods: Patients with TMA on kidney biopsy and/or peripheral blood were recruited from the prospective COMPETE cohort (NCT04745195) and Limburg Renal Registry. Patients were screened for complement dysregulation, including genetics/factor H autoantibodies (FHAA) and functional ex vivo testing on microvascular endothelial cells. Results: Seven (8%) out of 84 patients with TMA presented with a coexisting MG. MG clustered in patients aged over 50 years (n/N = 6/32, 19%). C4 and/or C3 levels were low in three patients, while four patients presented with normal complement levels. None of the patients carried rare variants in complement genes. Massive ex vivo C5b9 formation on the endothelium was noted in one patient; purified IgG from this patient caused massive ex vivo C5b9 formation via the alternative pathway of complement activation, pointing to complement dysregulation in the fluid phase. Kidney biopsies from other nephropathies linked to MG rarely exhibited concurrent TMA (n/N = 1/27, 4%). Conclusions: MG clustered in patients with TMA aged over 50 years. TMA and coexisting MG represents a heterogeneous disease spectrum, including a small subset of patients who may present with complement dysregulation.

Insight into advance care planning for patients on dialysis.

CONTEXT: Advance care planning is not included in regular clinical care for patients on dialysis. Insight into life-sustaining treatment preferences and communication about end-of-life care is necessary to develop interventions to improve advance care planning for patients on dialysis. OBJECTIVES: This cross-sectional observational study aimed to understand the preferences for life-sustaining treatments of outpatients on dialysis and to study the quality of patient-physician communication about end-of-life care and barriers and facilitators to this communication. METHODS: The following outcomes were assessed in 80 clinically stable dialysis patients: demographics, clinical characteristics, life-sustaining treatment preferences (cardiopulmonary resuscitation and mechanical ventilation, and Willingness to Accept Life-Sustaining Treatment instrument), preference for site of death, quality of communication (Quality of Communication Questionnaire), and barriers and facilitators to communication about end-of-life care (Barriers and Facilitators Questionnaire). RESULTS: Patients were able to indicate their preferences for life-sustaining treatments and site of death. Preferences for life-sustaining treatments depend on the specific treatment, the expected outcome of treatment, and likelihood of an adverse outcome. Life-sustaining preferences were discussed with the nephrologist by 30.3% of the patients. Quality of the patient-physician communication about end-of-life care was rated poor. This study identified several barriers and facilitators to end-of-life care communication. CONCLUSION: Patients should receive information about treatment burden, expected outcome, and the likelihood of an adverse outcome when discussing life-sustaining treatments. Quality of patient-physician communication about end-of-life care needs to improve. Barriers and facilitators to communication about end-of-life care provide direction for future interventions to facilitate advance care planning for patients on dialysis.

An evaluation of blood volume changes during ultrafiltration pulses and natriuretic peptides in the assessment of dry weight in hemodialysis patients.

Changes in blood volume (BV) during dialysis as well as plasma levels of brain natriuretic peptide (BNP) and N-terminal (NT) pro-BNP levels are possible tools to assess dry weight in hemodialysis (HD) patients. The aim of the study was to compare these parameters with other non-invasive techniques used to assess dry weight in HD patients, and to study their relation with intradialytic hypotension (IDH) and the presence of cardiovascular disease BV changes during HD, both during regular dialysis and during an ultrafiltration pulse, plasma levels of NT pro-BNP and BNP, and vena cava diameter index (VCDI) were assessed in a cohort of 66 HD patients, which was subdivided according to tertiles of total body water (TBW) corrected for body weight, assessed by bioimpedance analysis. Parameters were also related to the presence of IDH and history of cardiovascular disease. The decline in BV during regular dialysis and during an ultrafiltration pulse, as well as VCDI and BNP were significantly different between the tertiles of normalized TBW, but refill after the ultrafiltration pulse and NT pro-BNP were not. Only VCDI and the decline in BV during regular dialysis were significantly different between patients with or without IDH. Vena cava diameter index, BNP, and NT pro-BNP were significantly higher in patients with cardiovascular disease. Using bioimpedance as the reference method, changes in BV, either during regular dialysis or during an ultrafiltration pulse, as well as VCDI and BNP are all indicative of hydration state in dialysis patients, but refill after an ultrafiltration pulse is not. Only VCDI and BV changes were related to IDH. The presence of cardiovascular disease appears to influence both VCDI as well as BNP.