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Low-density lipoprotein (LDL) apheresis has been used increasingly in clinical practice for the treatment of renal diseases with nephrotic syndrome (NS), specifically focal segmental glomerulosclerosis (FSGS). Persistent hyperlipidemia for prolonged periods is nephrotoxic and leads to chronic progressive glomerular and tubulointerstitial injury. Effective management of hyperlipidemia with HMG-CoA reductase inhibitors or LDL apheresis in drug-resistant NS patients may prevent the progression of renal disease and, in some patients, resolution of NS symptoms. Available literature reveals beneficial effects of LDL apheresis for NS refractory to drug therapy. Here we update on the current understanding of lipid nephrotoxicity and application of LDL apheresis to prevent progression of renal diseases., Author(s): Rupesh Raina [sup.1] [sup.2] , Vinod Krishnappa [sup.2] [sup.3] Author Affiliations: (Aff1) grid.413473.6, 0000 0000 9013 1194, Department of Pediatric Nephrology, Akron Children's Hospital, , Akron, OH, USA (Aff2) [...]

Intradialytic hypotension (IDH) is a common adverse event resulting in premature interruption of hemodialysis, and consequently, inadequate fluid and solute removal. IDH occurs in response to the reduction in blood volume during ultrafiltration and subsequent poor compensatory mechanisms due to abnormal cardiac function or autonomic or baroreceptor failure. Pediatric patients are inherently at risk for IDH due to the added difficulty of determining and attaining an accurate dry weight. While frequent blood pressure monitoring, dialysate sodium profiling, ultrafiltration-guided blood volume monitoring, dialysate cooling, hemodiafiltration, and intradialytic mannitol and midodrine have been used to prevent IDH, they have not been extensively studied in pediatric population. Lack of large-scale studies on IDH in children makes it difficult to develop evidence-based management guidelines. Here, we aim to review IDH preventative strategies in the pediatric population and outlay recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup. Without strong evidence in the literature, our recommendations from the expert panel reflect expert opinion and serve as a valuable guide., Author(s): Rupesh Raina [sup.1] [sup.2] , Stephanie Lam [sup.3] , Hershita Raheja [sup.4] , Vinod Krishnappa [sup.2] [sup.5] , Daljit Hothi [sup.6] , Andrew Davenport [sup.7] , Deepa Chand [sup.8] [...]

Context: Unraveling the intricacies of what factors influence advance care planning (ACP) is an ongoing research challenge. Research shows much ACP is crisis-based and takes place at the end of life. Complicating this late-stage approach may be demographic differences based on race, ethnicity and socioeconomic status. Objective: We examined the relationship between demographic factors, chronic health conditions, and healthcare utilization in predicting who was most likely to engage in ACP activities, including designating a durable power of attorney for healthcare (DPOAHC), having a living will, and discussing wishes with family or others. Methods: We conducted a secondary analysis using 2018 Health and Retirement Study (HRS) exit data provided by a proxy for the deceased participant that matched the 2016 survey participant data (N = 884). Generalized linear mixed models were used for the analysis. Results: The number of chronic health conditions and healthcare utilization were not associated with ACP activities, but several of the demographic variables showed strong associations. Participants who were female, white, older, and from a higher socioeconomic status were more likely to have engaged in ACP. Conclusion: People continue to defer ACP discussions and documentation end of life or when facing medical crises. More needs to be done to reach out to younger adults, racial minorities, and those with lower socioeconomic status to encourage them to engage in ACP. [ABSTRACT FROM AUTHOR]

Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi‐organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over‐activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy‐associated aHUS, and aHUS in renal transplant patients. [ABSTRACT FROM AUTHOR]

Purpose: Neonatal renal cystic diseases have a great impact on the morbidity and mortality of the affected neonates and infants. A good insight into the pathophysiology, diagnosis and treatment options of various neonatal renal cystic diseases aid in early diagnosis and intervention, thereby preventing complications.Methods: PubMed search was done for articles on "neonatal renal cystic diseases" and relevant publications including reviews were considered for our article.Results: Both hereditary and nonhereditary causes of cystic kidney diseases can result in severe morbidity and mortality. The main diagnostic modality is ultrasound imaging and most of the neonatal renal cystic diseases are detected during prenatal ultrasound screening. Commonly encountered neonatal renal cystic diseases are autosomal dominant polycystic kidney disease, autosomal recessive polycystic kidney disease and multicystic dysplastic kidney.Conclusions: A thorough knowledge of various renal cystic diseases can be of extreme prognostic value. Physicians should be aware of the impact of early diagnosis and intervention on the lives of those affected. Further research about treatment of these diseases is ongoing and can result in breakthrough therapies for these patients. [ABSTRACT FROM AUTHOR]

Abstract: Pain management in end stage renal disease (ESRD) patients is a complex and challenging task to accomplish, and effective pain and symptom control improves quality of life. Pain is prevalent in more than 50% of hemodialysis patients and up to 75% of these patients are treated ineffectively due to its poor recognition by providers. A good history for PQRST factors and intensity assessment using visual analog scale are the initial steps in the management of pain followed by involvement of palliative care, patient and family counseling, discussion of treatment options, and correction of reversible causes. First line should be conservative management such as exercise, massage, heat/cold therapy, acupuncture, meditation, distraction, music therapy, and cognitive behavioral therapy. Analgesics are introduced according to WHO guidelines (by the mouth, by the clock, by the ladder, for the individual, and attention to detail) using three‐step analgesic ladder model. Neuropathic pain can be controlled by gabapentin and pregabalin. Substitution/addition of opioid analgesics are indicated if pain control is not optimal. Commonly used opioids in ESRD patients are tramadol, oxycodone, hydromorphone, fentanyl, methadone, and buprenorphine. Methadone, fentanyl, and buprenorphine are the ideal analgesics in ESRD. However, complex pain syndrome requires multidrug analgesic regimen comprising opioids, non‐opioids, and adjuvant medication, which should be individualized to the patient to achieve adequate pain control. [ABSTRACT FROM AUTHOR]

Hemodialysis (HD) in neonates and infants poses unique challenges due to high risks of mortality attributable to obligatory small blood flow volumes. Although HD is often necessary in neonates, its effectiveness and feasibility are poorly understood. The aim of this review is to describe in detail the few studies reporting on HD in neonates and infants (<12 months old) and then dissertate more broadly on the subject with an emphasis on recent innovations with potential to overcome traditional barriers for effective HD in this population. We detail the clinical characteristics, outcomes, technical considerations, maintenance and complications associated with HD, and provide guidance for addressing challenges associated with HD in this population. [ABSTRACT FROM AUTHOR]

Abstract: Atypical hemolytic uremic syndrome (aHUS) is a rare life‐threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta‐analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations. A PubMed/Medline search was conducted for “atypical hemolytic uremic syndrome” case reports published between November 2005 and November 2015. R Version 3.2.2 was used to calculate descriptive statistics and perform univariate analyses. Wilcoxon rank‐sum test was used to compare time to symptoms resolution, creatinine and platelet count normalization across the treatment and mutation carrier groups. A total of 259 aHUS patients were reported in 176 articles between 2005 and 2015. In the last 5‐year period compared to the precedent, there was an increase in the number of aHUS cases reported (180 vs. 79 cases) and the use of eculizumab also increased (6.3% to 46.1%, P < 0.000), although plasma exchange usage did not change (P = 0.281). CFH antibodies were present in a significantly higher number of patients treated with plasma exchange therapy (19.1%, P = 0.000) while none of the non‐plasma exchange therapy group had CFH antibodies. Most common mutation was CFH (50%, 69/139) followed by CFHR1 (35%, 30/85), MCP (22.8%, 23/101) and CFI (16.6%, 17/102). Time to symptoms resolution and serum creatinine or platelet count normalization were not significantly different between eculizumab and non‐eculizumab group (P = 0.166, P = 0.361, P = 0.834), and between plasma exchange and non‐plasma exchange group (P = 0.150, P = 0.135, P = 0.784). However, both eculizumab and plasma exchange groups had early platelet recovery (22 vs. 30 days and 25.5 vs. 32.5 days), faster creatinine normalization (27 vs. 30.5 days and 27 vs. 37 days) and interestingly, a longer period for symptoms resolution (45.5 vs. 21 days and 30 vs. 18.5 days) compared to non‐eculizumab and non‐plasma exchange groups. Mortality rate decreased with the use of eculizumab significantly (P = 0.045) compared to non‐eculizumab group and there was no change in mortality rate with the use of plasma exchange therapy (P = 0.760) compared to non‐plasma exchange group. Plasma exchange continues to be the initial treatment of choice for aHUS. Although significant reduction in the mortality rate was noted with the use of eculizumab, there were no differences in time to resolution of symptoms or serum creatinine or platelet normalization with the use of either eculizumab or plasma therapy. Atypical HUS is acute and life‐threatening, so plasma exchange may be initiated before the confirmed diagnosis and in patients positive for CFH antibodies. Eculizumab therapy should be considered once aHUS is confirmed by genetic testing. [ABSTRACT FROM AUTHOR]

Providing end-of-life care to patients suffering from chronic kidney disease (CKD) and/or end-stage renal disease often presents ethical challenges to families and health care providers. However, as the conditions these patients present with are multifaceted in nature, so should be the approach when determining prognosis and treatment strategies for this patient population. Having an interdisciplinary palliative team in place to address any concerns that may arise during conversations related to end-of-life care encourages effective communication between the patient, the family and the medical team. Through the use of a case study, the authors demonstrate how an interdisciplinary palliative team can be used to make decisions that satisfy the patient's and the medical team's desires for end-of-life care. [ABSTRACT FROM AUTHOR]

Introduction: Angiotensin-converting enzyme 2 (ACE2) of the renin-angiotensin-aldosterone system (RAAS) serves as a functional receptor to gain entry into the cells for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which causes coronavirus disease 2019 (COVID-19). The interaction between SARS-CoV-2 and ACE2 is a potential virulent factor in infectivity. Our study aims to ascertain the association of RAAS inhibitors with adverse cardiovascular and other outcomes in hospitalized COVID-19 patients., Methods: This is a retrospective study of medical records of ≥ 18-year-old patients hospitalized for COVID-19 from March 2020 to October 2020. Primary outcomes were acute cardiovascular events (ST-elevation myocardial infarction, non-ST elevation myocardial infarction type 1, acute congestive heart failure, acute stroke) and mortality. Secondary outcomes were respiratory failure, need for and duration of mechanical ventilation, acute deep vein thrombosis or pulmonary embolism (DVT/PE), and readmission rate., Results: Among 376 hospitalized COVID-19 patients, 149 were on RAAS inhibitors. No statistically significant differences were found between RAAS inhibitor and non-RAAS inhibitor groups with respect to acute cardiovascular events (6% vs. 6.2%, p=0.94), acute DVT/PE (4.7% vs. 4.8%, p=0.97), hypoxia (62.4% vs. 58.6%, p=0.46), need for mechanical ventilation (18.1% vs. 16.7%, p=0.72), mortality (19.5% vs. 22%, p=0.56), and readmission rate (11.4% vs. 14.1%, p=0.45). Some nuances discovered were a higher rate of hospitalizations among Native Americans receiving RAAS inhibitors (30.2% vs 19.8%) and significantly lower levels of procalcitonin in patients on RAAS inhibitors., Conclusions: Among hospitalized patients with COVID-19, those on RAAS inhibitors showed no significant differences in acute cardiovascular events, acute DVT/PE, hypoxia, need for mechanical ventilation, readmission, or mortality rate compared to those not on them. However, further large-scale studies are needed to validate these findings., (S. Karger AG, Basel.)

Background: Coronavirus disease 2019 (COVID-19) triggers multiple components of the immune system and causes inflammation of endothelial walls across vascular beds, resulting in respiratory failure, arterial and venous thrombosis, myocardial injury, and multi-organ failure leading to death. Early in the COVID-19 pandemic, aspirin was suggested for the treatment of symptomatic individuals, given its analgesic, antipyretic, anti-inflammatory, anti-thrombotic, and antiviral effects. This study aimed to evaluate the association of aspirin use with various clinical outcomes in patients hospitalized for COVID-19., Methods: This was a retrospective study involving patients aged ≥ 18 years and hospitalized for COVID-19 from March 2020 to October 2020. Primary outcomes were acute cardiovascular events (ST elevation myocardial infarction (STEMI), type 1 non-ST elevation myocardial infarction (NSTEMI), acute congestive heart failure (CHF), and acute stroke) and death. Secondary outcomes were respiratory failure, need for mechanical ventilation, and acute deep vein thrombosis (DVT)/pulmonary embolism (PE)., Results: Of 376 patients hospitalized for COVID-19, 128 were taking aspirin. Significant proportions of native Americans were hospitalized for COVID-19 in both aspirin (22.7%) and non-aspirin (24.6%) groups. Between aspirin and non-aspirin groups, no significant differences were found with regard to mechanical ventilator support (21.1% vs. 15.3%, P = 0.16), acute cardiovascular events (7.8% vs. 5.2%, P = 0.32), acute DVT/PE (3.9% vs. 5.2%, P = 0.9), readmission rate (13.3% vs. 12.9%, P = 0.91) and mortality (23.4% vs. 20.2%, P = 0.5); however, the median duration of mechanical ventilation was significantly shorter (7 vs. 9 days, P = 0.04) and median length of hospitalization was significantly longer (5.5 vs. 4 days, P = 0.01) in aspirin group compared to non-aspirin group., Conclusion: No significant differences were found in acute cardiovascular events, acute DVT/PE, mechanical ventilator support, and mortality rate between hospitalized COVID-19 patients who were taking aspirin compared to those not taking aspirin. However, larger studies are required to confirm our findings., Competing Interests: Authors have no conflict of interest to declare., (Copyright 2024, Vinod et al.)