High-Volume Hemodiafiltration with Step-Down Approach versus Standard-of-Care Continuous Renal Replacement Therapy Approach in Critically Ill Burn Patients.

INTRODUCTION: Acute kidney injury (AKI) is a common complication of severe burn injuries and contributes to morbidity and mortality. It is exacerbated in burn patients by elevated serum creatinine and pro-inflammatory cytokines, leading to immune dysregulation. Chronic renal replacement therapy is standard of care and removes cytokines to return the body to homeostasis. Continuous veno-venous hemodiafiltration (CVVHDF) is a high-filtration method to enhance cytokine clearance; we analyze a step-down approach for improved outcomes in burn patients. METHODS: This study reviewed 15 burn patients at Akron Children's Hospital stratified into 2 groups: high-flow CVVHDF with step-down approach versus standard CVVHDF. A normocarbia bicarbonate-based dialysate solution and citrate anticoagulation was applied, and blood flow rate was maintained greater than 200 mL/min. RESULTS: Fifteen burn patients at Akron Children's Hospital were separated into groups managed with high-flow CVVHDF (n = 9) and standard-flow CVVHDF (n = 6). All 15 developed AKI symptoms and diuretic-resistant fluid overload, with 4/15 displaying fluid overload greater than 40%. The most common indication for hemofiltration was acute tubular necrosis (11/15). Average time on CVVHDF was 20.2 days and length of admission was 58.6 days. Vasodepressor dependency index was significantly reduced in the high-flow group at 48 h, but no significant difference in mortality was identified. No significant difference was identified in adverse reactions, notably electrolyte imbalances. CONCLUSION: The literature on the efficacy of high-flow CVVHDF is limited. This study suggests improved mortality rates and length of stay with high flow compared to the literature. Further studies with multicenter involvement are necessary.

Impact of SARS-CoV-2 infection on pain crisis and acute chest syndrome in patients with sickle cell anemia: A retrospective multi-cohort study based on US national data from 2020 to 2022.

COVID-19 infection has been a significant contributor to global morbidity and mortality, especially among those patients with chronic diseases. The Centers for Disease Control and Prevention have classified sickle cell disease (SCD) as a condition that increases the risk of severe illness from COVID-19 infection. A retrospective study was conducted using the TRiNetX health research network database to identify SCA patients ( HbSS, Sbeta-thalassemia zero) who had SARS-CoV-2 infection over 2 years; these were compared with similar patients who did not have the infection in terms of demographics, pain control, and laboratory parameters COVID-19 illness impacts [ain crises and ACS, and prior vaccination against influenza and COVID-19 may represent a protective factor for developing pain crises.

Hemochromatosis in an Adult Female With Previous Iron Deficiency Anemia on Iron Supplementation.

A 56-year-old female presented with a diagnosis of iron deficiency anemia over three years prior (on oral iron supplementation) and presented with altered mental status. She was admitted to the Coronary Care Unit for troponinemia and T-wave inversions. Two-dimensional echocardiography revealed hypokinesia of the left ventricle (LV) anterior wall with reduced ejection fraction. The patient was stabilized on metoprolol and heparin infusions, but heparin was discontinued after iron studies revealed overload (iron: 159 ug/dL, 100% saturation, ferritin: 1480 ng/mL). Cardiac MRI revealed mixed concentric-eccentric LV hypertrophy, raising concern for severe iron overload. Mutation analysis of the HFE  (homeostatic iron regulator) gene responsible for hereditary hemochromatosis was negative for a homozygous mutation. Hematology was consulted to establish outpatient follow-up and consider treatments for the acquired hemochromatosis. Acquired hemochromatosis typically occurs in the setting of multiple blood product transfusions. Oral supplementation is typically mitigated by limited bioavailability. Follow-up was needed to track the patient's iron deficiency anemia and identify resolution and potential toxicity. Further research into predisposing factors for hemochromatosis in patients on oral supplementation without HFE mutations is indicated.

Pediatric-type follicular lymphoma in a Crohn's disease patient receiving anti-α4ß7-integrin therapy: A case report.

BACKGROUND: Patients with autoimmune conditions receiving immunosuppressants are at risk of non-Hodgkin lymphomas (NHL). Vedolizumab (anti-α4ß7-integrin antibody), a treatment-of-choice for Crohn's disease (CD), reduces inflammatory lymphocyte trafficking into the intestinal mucosa. This effect is believed to be confined to the colon. CASE SUMMARY: We report the case of a CD patient on vedolizumab for five years who developed pediatric-type follicular lymphoma. Work-up prior to therapy revealed a reduction in circulating T-lymphocytes and their suppressed response to mitogens. Rituximab, cyclophosphamide, vincristine, and prednisone chemo-immunotherapy resulted in durable lymphoma remission, and vedolizumab treatment was continued. While the patient's T-lymphocyte population and immunoglobulin production recovered, the T-lymphocyte mitogen response remained suppressed. CONCLUSION: This patient's NHL may be linked to receiving anti-α4ß7 therapy. Further research could be beneficial to determine if proactive surveillance for NHL and other systemic diseases is indicated in patients on vedolizumab.

Atypical Hemolytic-Uremic Syndrome: Genetic Basis, Clinical Manifestations, and a Multidisciplinary Approach to Management.

Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) defined by the triad of hemolytic anemia, thrombocytopenia, and acute kidney injury. Microthrombi develop in the glomerular capillaries secondary to endothelial damage and exert shear stress on red blood cells, consume platelets, and contribute to renal dysfunction and failure. Per current understanding of pathophysiology, HUS is classified into infectious, secondary, and atypical disease. The most common etiology is infectious sequelae of Shiga toxin-producing Escherichia coli (STEC); other causative organisms include shigella and salmonella. Secondary HUS arises from cancer, chemotherapy, solid organ and hematopoietic stem cell transplant, pregnancy, or autoimmune disorders. Primary atypical hemolytic-uremic syndrome (aHUS) is associated with genetic mutations in complement and complement regulatory proteins. Under physiologic conditions, complement regulators keep the alternative complement system continuously active at low levels. In times of inflammation, mutations in complement-related proteins lead to uncontrolled complement activity. The hyperactive inflammatory state leads to glomerular endothelial damage, activation of the coagulation cascade, and TMA findings. Atypical hemolytic-uremic syndrome is a rare disorder with a prevalence of 2.21 to 9.4 per million people aged 20 years or younger; children between the ages of 0 and 4 are most affected. Multidisciplinary health care is necessary for timely management of its extra-renal manifestations. These include vascular disease of the heart, brain, and skin, pulmonary hypertension and hemorrhage, and pregnancy complications. Adequate screening is required to monitor for sequelae. First-line treatment is the monoclonal antibody eculizumab, but several organ systems may require specialized interventions and coordination of care with sub-specialists.

Cardiorenal syndrome in the pediatric population: A systematic review.

The concept of cardiorenal syndrome (CRS) is derived from the crosstalk between the heart and kidneys in pathological conditions. Despite the rising importance of CRS, there is a paucity of information on the understanding of its pathophysiology and management, increasing both morbidity and mortality for patients. This review summarizes the existing conceptual pathophysiology of different types of CRS and delves into the associated therapeutic modalities with a focus on pediatric cases. Prospective or retrospective observational studies, comparative studies, case reports, case-control, and cross-sectional studies that include pediatric patients with CRS were included in this review. Literature was searched using PubMed, EMBASE, and Google Scholar with keywords including "cardio-renal syndrome, type," "reno-cardio syndrome," "children," "acute kidney injury," and "acute decompensated heart failure" from January 2000 to January 2021. A total of 14 pediatric studies were ultimately included and analyzed, comprising a combined population of 3608 children of which 32% had CRS. Of the 14 studies, 57% were based on type 1 CRS, 14% on types 2 and 3 CRS, and 7% were on types 4 and 5 CRS. The majority of included studies were prospective cohort, although a wide spectrum was observed in terms of patient age, comorbidities, etiologies, and treatment strategies. Commonly observed comorbidities in CRS type 1 were hematologic, oncologic, cardiology-related side effects, muscular dystrophy, and pneumonia/bronchiolitis. CRS, particularly type 1, is prevalent in children and has a significant risk of mortality. The current treatment regimen primarily involves diuretics, extracorporeal fluid removal, and treatment of underlying etiologies and comorbidities.

Non-anticoagulation pediatric continuous renal replacement therapy methods to increase circuit life.

INTRODUCTION: Acute kidney injury (AKI) is a clinical condition characterized by an abrupt increase in serum creatinine levels due to functional changes in the kidneys from a newfound insult or injury. For supportive treatment, continuous renal replacement therapy (CRRT) is one of the most widely used modalities due to its precise control of fluid balance over extended periods of time. However, its complications include circuit clotting, the most frequent cause for CRRT interruption. Vascular access and circuit management were found to be major determinants of performance efficiency. Anticoagulation required to prevent clotting has the downside of increasing the risk of bleeding, especially in the setting of overdosage. Hence, a delicate balance needs to be maintained consistently. METHODS: This study explores the adequacy of non-anticoagulation measures in the prevention of circuit clotting. A comprehensive literature search was conducted using PubMed/Medline and Embase databases to include all relevant studies. FINDINGS: The most-effective CRRT catheter would be made of nonthrombogenic material, noncuffed and nontunneled with separate lumens for arterial and venous blood. Further, studies show that blood flow during the process is optimized at 200 ml/min, which can be lowered in the pediatric population due to more narrow catheters. Platelet count and hematocrit need to be closely monitored as levels above 450,000 × 106 /L and 0.40, respectively, increase risk of clotting. Predilution is a non-anticoagulation technique to reduce the risk of clotting by returning replacement solution to the blood before it reaches the filter. Also, biocompatible membranes such as polyacrylonitrile or polysulfone activate the coagulation cascade significantly less than the conventional cellulose-based membranes, thereby reducing clotting chances. DISCUSSIONS: With the advent of such techniques and maneuvers, anticoagulation can be efficiently maintained in patients undergoing CRRT without increasing the risk of bleeding.