An Incidental Finding of a Massive Loculated Pericardial Effusion in a Patient Presenting With Inferior ST-Elevation Myocardial Infarction.

Pericardial effusion, commonly associated with malignancies such as lung, breast, and esophageal cancers through local extension, or leukemia, lymphoma, and melanoma via metastatic dissemination, is rarely observed in renal cell carcinoma (RCC). This report presents a rare case of a large loculated pericardial effusion in a 68-year-old male, potentially linked to RCC, who concurrently presented with an inferior wall ST-elevation myocardial infarction (STEMI). The patient, with a history of hypertension, hyperlipidemia, end-stage renal disease, coronary artery disease, and former smoking, exhibited symptoms including chest pain, diaphoresis, and shortness of breath, but no fever, chills, or night sweats. Diagnostic imaging revealed a significant pericardial effusion and a renal mass consistent with RCC, along with potential pulmonary metastases. Despite the complexity and high-risk nature of his condition, exacerbated by recent STEMI and dual antiplatelet therapy, a multidisciplinary approach was employed. This case emphasizes the need for careful management and tailored treatment strategies in patients with multiple coexisting conditions, highlighting the critical role of comprehensive diagnostic evaluation and collaborative care in improving patient outcomes.

Effective Non-invasive Treatment of Lingual Hematoma in an End-Stage Renal Disease (ESRD) Patient on Rivaroxaban: A Case Report.

Lingual hematoma is a rare, life-threatening condition that can obstruct the airway. We report a 73-year-old male with end-stage renal disease (ESRD) who developed lingual hematoma while on rivaroxaban. He presented with odynophagia and significant tongue swelling. Treatment with vitamin K, dexamethasone, tranexamic acid, and prothrombin complex concentrate led to rapid improvement without the need for intubation. This case highlights the importance of prompt medical management to prevent airway obstruction in similar patients.

Mind the Anion Gap: 5-Oxoproline-Induced High Anion Gap Metabolic Acidosis in End-Stage Renal Disease.

A rare complication, 5-oxoproline-induced high anion gap metabolic acidosis (HAGMA) is associated with chronic acetaminophen use, predominantly reported in outpatient settings. However, its occurrence in hospitalized patients, particularly those with end-stage renal disease (ESRD), remains underreported. We present a case of a 74-year-old female with ESRD on hemodialysis who developed HAGMA highly suspicious for 5-oxoproline toxicity from acetaminophen usage following cardiac surgery. Despite a standard analgesic dose, the patient's renal impairment likely predisposed her to 5-oxoproline accumulation, resulting in severe metabolic acidosis. Discontinuation of acetaminophen led to the resolution of HAGMA, highlighting the importance of recognizing this rare but potentially life-threatening complication in the inpatient and critical care setting. This case suggests a potential interaction between acetaminophen metabolism and renal dysfunction in the pathogenesis of 5-oxoproline-induced HAGMA.

Pachydermoperiostosis Presenting With End-Stage Kidney Disease.

Pachydermoperiostosis, also known as Touraine-Solente-Golé syndrome, is an uncommon hereditary condition. This condition includes skin thickening (pachydermia), abnormalities of the bones (periostosis), and digital clubbing (acropachy). We present a case of complete pachydermoperiostosis who presented with end-stage kidney disease. Chronic tubulointerstitial disease secondary to long-term analgesics and complementary and alternative medications was considered the likely etiology for renal dysfunction. The patient underwent serial hemodialysis followed by arteriovenous fistula surgery. In view of significant synovial inflammation, he was also given a selective COX-2 inhibitor. Pachydermoperiostosis is a rare condition, and although there is no therapy for the condition itself, medicinal or surgical interventions can effectively control its secondary effects.

Minimisation of dialysis risk in hospital patients with chronic kidney disease (MinDial): study protocol for a multicentre, stepped-wedge, cluster-randomised controlled trial.

BACKGROUND: Early identification of patients with chronic kidney disease (CKD) and advancing kidney insufficiency, followed by specialist care, can decelerate the progression of the disease. However, awareness of the importance and possible consequences of kidney insufficiency is low among doctors and patients. Since kidney insufficiency can be asymptomatic even in higher stages, it is often not even known to those belonging to risk groups. This study aims to clarify whether, for hospitalised patients with advanced chronic kidney disease, a risk-based appointment with a nephrology specialist reduces disease progression. METHODS: The target population of the study is hospitalised CKD patients with an increased risk of end-stage renal disease (ESRD), more specifically with an ESRD risk of at least 9% in the next 5 years. This risk is estimated by the internationally validated Kidney Failure Risk Equation (KFRE). The intervention consists of a specific appointment with a nephrology specialist after the hospital stay, while control patients are discharged from the hospital as usual. Eight medical centres include participants according to a stepped-wedge design, with randomised sequential centre-wise crossover from recruiting patients into the control group to recruitment to the intervention. The estimated glomerular filtration rate (eGFR) is measured for each patient during the hospital stay and after 12 months within the regular care by the general practitioner. The difference in the change of the eGFR over this period is compared between the intervention and control groups and considered the primary endpoint. DISCUSSION: This study is designed to evaluate the effect of risk-based appointments with nephrology specialists for hospitalised CKD patients with an increased risk of end-stage renal disease. If the intervention is proven to be beneficial, it may be implemented in routine care. Limitations will be examined and discussed. The evaluation will include further endpoints such as non-guideline-compliant medication, economic considerations and interviews with contributing physicians to assess the acceptance and feasibility of the intervention. TRIAL REGISTRATION: German Clinical Trials Register DRKS00029691 . Registered on 12 September 2022.

Assessment of essential and toxic trace element levels in erythrocytes of hemodialysis patients with end-stage renal disease.

BACKGROUND: Chronic kidney disease (CKD) is a global public health problem, resulting in end-stage kidney disease, cardiovascular disease, and premature death. AIM: The aim of the study was to determine the profile of essential and toxic trace elements in erythrocytes of patients with end-stage renal disease (ESRD) and their relationship with selected anthropometric and biochemical parameters. METHODS: The present study compared the profiles of trace elements, including toxic sub-stances, in the erythrocytes of 80 hemodialysis patients with CKD with 40 healthy subjects. All patients had stage 5 CKD. The levels of Cd and Pb were determined by graphite furnace atomic absorption spectrometry and levels of Fe, Mn, Zn, Cu Cr, Ni, and Li by inductively coupled plasma atomic emission spectrometry. RESULTS: The ESRD patients demonstrated significantly lower Fe and Zn concentrations and significantly higher Mn and Li and toxic Pb and Cd concentrations in erythrocytes compared to those of the healthy controls. Negative correlations were observed, among others, between the concentrations of Cu, Li, and creatinine; Cu and phosphates; Mn, Pb, and transferrin saturation while positive correlations were noted between Cu, Cr, and transferrin and Pb, Cr, and the normalized protein catabolism rate. CONCLUSIONS: The higher concentrations of toxic elements present in the erythrocytes of CKD patients might have resulted from the reduced ability of the kidneys to excrete them. Moreover, differences in the concentrations of essential elements (Fe, Mn, Zn) between the two groups indicated that their resorption in the kidneys of CKD patients was impaired. Patients with CKD might benefit from interventions intended to reduce high, toxic concentrations of Pb and Cd and Li and Mn as an alternative supportive treatment. Iron and zinc supplementation should be a component for the treatment of anemia in CKD patients.

Evaluation of a proteomic signature coupled with the kidney failure risk equation in predicting end stage kidney disease in a chronic kidney disease cohort.

BACKGROUND: The early identification of patients at high-risk for end-stage renal disease (ESRD) is essential for providing optimal care and implementing targeted prevention strategies. While the Kidney Failure Risk Equation (KFRE) offers a more accurate prediction of ESRD risk compared to static eGFR-based thresholds, it does not provide insights into the patient-specific biological mechanisms that drive ESRD. This study focused on evaluating the effectiveness of KFRE in a UK-based advanced chronic kidney disease (CKD) cohort and investigating whether the integration of a proteomic signature could enhance 5-year ESRD prediction. METHODS: Using the Salford Kidney Study biobank, a UK-based prospective cohort of over 3000 non-dialysis CKD patients, 433 patients met our inclusion criteria: a minimum of four eGFR measurements over a two-year period and a linear eGFR trajectory. Plasma samples were obtained and analysed for novel proteomic signals using SWATH-Mass-Spectrometry. The 4-variable UK-calibrated KFRE was calculated for each patient based on their baseline clinical characteristics. Boruta machine learning algorithm was used for the selection of proteins most contributing to differentiation between patient groups. Logistic regression was employed for estimation of ESRD prediction by (1) proteomic features; (2) KFRE; and (3) proteomic features alongside KFRE. RESULTS: SWATH maps with 943 quantified proteins were generated and investigated in tandem with available clinical data to identify potential progression biomarkers. We identified a set of proteins (SPTA1, MYL6 and C6) that, when used alongside the 4-variable UK-KFRE, improved the prediction of 5-year risk of ESRD (AUC = 0.75 vs AUC = 0.70). Functional enrichment analysis revealed Rho GTPases and regulation of the actin cytoskeleton pathways to be statistically significant, inferring their role in kidney function and the pathogenesis of renal disease. CONCLUSIONS: Proteins SPTA1, MYL6 and C6, when used alongside the 4-variable UK-KFRE achieve an improved performance when predicting a 5-year risk of ESRD. Specific pathways implicated in the pathogenesis of podocyte dysfunction were also identified, which could serve as potential therapeutic targets. The findings of our study carry implications for comprehending the involvement of the Rho family GTPases in the pathophysiology of kidney disease, advancing our understanding of the proteomic factors influencing susceptibility to renal damage.

Effect of Cold Versus Hot Snare Polypectomy on Colon Postpolypectomy Bleeding in Patients with End-Stage Renal Disease: A Retrospective Cohort Study.

BACKGROUND: Patients with end-stage renal disease (ESRD) who undergo polypectomy may experience postpolypectomy bleeding. To reduce the risk of delayed postpolypectomy bleeding among the general population, cold snare polypectomy (CSP) is recommended for removing colon polyps smaller than 1 cm. Nevertheless, only few studies have examined the effect of CSP on patients with ESRD. METHODS: We retrospectively analyzed the data of patients with ESRD who underwent colonoscopic polypectomy for polyps larger than 5 mm at a Taiwanese university hospital from January 2014 to January 2023. The main outcome was delayed postpolypectomy bleeding within 30 days. Multivariate analysis was conducted to adjust for major confounders. RESULTS: A total of 557 patients with ESRD underwent colonoscopic polypectomy during the study period: 201 underwent CSP and 356 underwent hot snare polypectomy (HSP). Delayed postpolypectomy bleeding occurred in 27 patients (4.8%). The rate of delayed postpolypectomy bleeding was lower in patients with ESRD who underwent CSP than in those who underwent HSP (1.9% vs. 6.4%, P = 0.022). The percentage of patients who did not experience postpolypectomy bleeding within 30 days after CSP remained lower than that observed after HSP (P = 0.019, log-rank test). Multivariate analysis demonstrated immediate postpolypectomy bleeding and HSP to be independent risk factors for delayed postpolypectomy bleeding. A nomogram prognostic model was used to predict the potential of delayed postpolypectomy bleeding within 30 days in patients with ESRD. CONCLUSIONS: Compared with HSP, CSP is more effective in mitigating the risk of delayed postpolypectomy bleeding in patients with ESRD.

High Self-Reported Prevalence of Kidney Stones in Trinidad and Tobago: Results of a Cross-Sectional Online Survey.

Introduction No data exist on the prevalence of kidney stone disease in Trinidad and Tobago. Local clinicians have noted that the disease is very common, and this study represents the first attempt to investigate the prevalence of urolithiasis in these islands. Objectives The objective is to estimate the prevalence of kidney stone disease in Trinidad and Tobago and to investigate the epidemiology of the disease. Methods An online survey using the online tool Survey Monkey was distributed among members of the public via instant messaging and social media. The survey captured data relating to the stone status and demographics of respondents. Results 1225 patients completed the survey of whom 46.5% were males and 53.5% were females. Respondents were equally distributed throughout the country. 16.74% of those surveyed indicated that they were currently affected by stones confirmed by imaging. Kidney stones were more common among Trinidadians of East Indian ancestry (20.6% vs 10.6%). Positive correlations were established between kidney stones and the presence of hypertension, diabetes, and gout. Persons with kidney stones were more likely to have a family member with the disease - 45.6% vs 31.4% among those without kidney stones. Conclusion This study demonstrates a high self-reported prevalence of kidney stones in Trinidad and Tobago.

The Accelerated Onset of Calciphylaxis in a 72-Year-Old Female Hemodialysis Patient.

Calciphylaxis is a unique medical condition characterized by calcification of the medial layer of arterioles and soft tissues in a patient's skin at the level of the dermis and subcutaneous adipose tissue. The rate of progression of calciphylaxis is rapid, starting with a reduction of blood flow that leads to ischemic changes in the skin that can manifest as painful cutaneous erythematous nodules or plaques and later as skin ulceration. The majority of patients affected by calciphylaxis have predisposing comorbidities such as end-stage renal disease with a long history of hemodialysis and electrolyte abnormalities in calcium, phosphate, and parathyroid hormone levels. This report presents the case of a 72-year-old female patient on hemodialysis who developed calciphylaxis. The methods for early prognosis (the methods of early diagnosis), including clinical presentation, risk factors, imaging techniques, and laboratory investigations, are discussed. The presented case is particularly noteworthy given the onset of calciphylaxis within a mere three months of initiating hemodialysis, a timeline significantly shorter than the typically observed period in most patients. (The case detailed in this report outlines the rapid onset of calciphylaxis in a patient who was receiving hemodialysis for only three months.) This patient with early-onset calciphylaxis highlights the unpredictable nature of calciphylaxis and the need for increased clinical vigilance even in the initial stages of hemodialysis.

Brown Tumor From Secondary Hyperparathyroidism Mimicking Metastatic Renal Cell Carcinoma in a Patient With End-Stage Renal Disease.

Brown tumors (also known as osteitis fibrosa cystica) are rare complications of end-stage renal disease (ESRD) and secondary hyperparathyroidism (HPT), characterized by focal bone lesions that resemble neoplasms. They are often misdiagnosed as metastatic bone disease, especially in patients with a history of malignancy. We present a case of a 60-year-old man with a history of renal cell carcinoma (RCC), and ESRD on hemodialysis (HD), who developed diffuse bone lesions on imaging with osteolytic/osteoblastic appearance concerning metastases, but on further workup was found to have brown tumors. We discuss the treatment and outcome and briefly review the relevant medical literature.

Hyperkalemic Periodic Paralysis Secondary to End-Stage Renal Disease and Excess Potato Consumption.

Hyperkalemic periodic paralysis is a rare medical condition characterized by periods of extreme muscle weakness or paralysis. While most cases of hyperkalemic periodic paralysis are associated with a genetic channelopathy, cases of secondary hyperkalemic periodic paralysis can pose a challenge for medical personnel in terms of timely recognition. Identification of this medical emergency early in its course is essential to preventing cardiac and neurological sequelae. We report a case of a 58-year-old female who presented with stroke-like symptoms and was found to have secondary hyperkalemic periodic paralysis attributed to the excess consumption of potatoes, a potassium-rich food. This case highlights the importance of considering hyperkalemic periodic paralysis early in the differentiation of patients with end-stage renal disease (ESRD) who present with muscle weakness and stroke-like symptoms.

Chunky Mitral Annular Calcification: Caseoma or a Tumor?

Mitral annular calcification (MAC) is relatively common in clinical practice. Females are more often affected than males. Patients with end-stage renal disease have MAC relatively more commonly than the general population. Patients with MAC often develop conduction system disturbances, including advanced atrioventricular blocks. They are also more likely to develop various arrhythmias, including atrial fibrillation. Caseous mitral annulus calcification is a variant of MAC that often looks like a cardiac tumor on an echocardiogram and needs to be differentiated.

New-onset atrial fibrillation following arteriovenous fistula increases adverse clinical events in dialysis patients with end-stage renal disease.

Background: End-stage renal disease (ESRD) patients have a high potential cardiovascular burden, and cardiovascular disease (CVD) is the leading cause of death in maintenance haemodialysis (MHD) patients. Arteriovenous fistula (AVF) is the preferred vascular access for MHD patients, but AVF significantly affects the haemodynamics of the cardiovascular system, leading to or exacerbating CVD, including atrial fibrillation (AF). This study aimed to evaluate the impact of AVF on cardiac function, especially of the left atrium (LA), in patients with ESRD and to further explore the relationship between AVF establishment and the occurrence of AF. Methods: We selected 1,107 ESRD patients on haemodialysis using AVF and 550 patients with tunneled-cuffed catheters (TCC) admitted between January 2016 and December 2022 for follow-up to compare the rate of AF between the two groups. A total of 153 patients in the AVF group with complete information (clinical data, echocardiographic and biochemical indices, and other data) were enrolled and retrospectively analysed for risk factors for the development of AF and were followed up for adverse clinical outcomes (including all-cause death, cardiac death, readmission due to heart failure, and stroke). Results: The incidence of new-onset AF was higher in the AVF group than the TCC group after dialysis access was established (16.30% vs. 5.08%, P < 0.001). Echocardiography showed that the LA anteroposterior diameter increased (P < 0.001) and the incidence of AF increased from 11.76% to 26.14% (P = 0.001) after AVF establishment. Multivariate logistic regression analysis showed that age and LA enlargement were independent risk factors for new-onset AF after AVF establishment (P < 0.05). Adverse clinical outcomes were more common in patients with AF than in patients without AF (P < 0.001). Multivariate Cox risk regression analysis suggested that new-onset AF (HR = 4.08, 95% CI: 2.00-8.34, P < 0.001) and left ventricular systolic dysfunction (HR = 2.42, 95% CI: 1.20-4.88, P = 0.01) after AVF establishment were independent risk factors for adverse clinical outcomes. Conclusion: LA enlargement after AVF establishment is associated with a significant increase in the incidence of AF, in addition, AF which is as an important influential factor in patients with MHD combined other systemic diseases might increase adverse clinical events. Clinical Trial Registration: (NCT06199609).

Progression of Skin Ulcer Secondary to Calciphylaxis in a Patient With End-Stage Renal Disease (ESRD) on Hemodialysis and the Therapeutic Approach.

Calciphylaxis is a rare and severe medical condition characterized by the calcification of small blood vessels and soft tissues, leading to tissue damage, skin ulcers, and intense pain. It most commonly affects individuals with underlying health conditions such as kidney disease, particularly end-stage renal disease (ESRD), and is associated with high mortality rates. Understanding the diagnosis and management of calciphylaxis is crucial for improving patient outcomes. Diagnosing calciphylaxis can be challenging due to its rarity and overlapping symptoms with other skin conditions. Healthcare professionals typically use a combination of clinical evaluation and diagnostic tests to reach a conclusive diagnosis. The management of calciphylaxis is multifaceted and typically involves a collaborative effort between various healthcare specialists, including nephrologists, dermatologists, and wound care experts. The primary goals of treatment are to alleviate pain, promote wound healing, address underlying causes, and prevent further complications.

Frequency of Pulmonary Hypertension and Its Associated Risk Factors in End-Stage Renal Disease (ESRD) Patients on Maintenance Hemodialysis.

Introduction Pulmonary hypertension (PH) is a recognized complication in patients with end-stage renal disease (ESRD undergoing maintenance hemodialysis (MHD). PH is commonly found in patients with chronic kidney disease (CKD) and ESRD. PH is associated with increased morbidity and mortality in patients with CKD. Methodology This cross-sectional study aimed to assess the prevalence of PH and its associated risk factors in MHD patients. A total of 220 ESRD patients on MHD patients at The Kidney Center, Karachi, Pakistan, aged 18-70 were included. Patients with chronic obstructive lung disease, valvular heart disease, and obstructive sleep apnea were excluded, as these conditions can be responsible for PH. PH was evaluated by echocardiography (ECHO), which was performed by a cardiologist. Results The mean age was 50.65 ± 14.4 years, with 131 (59.5%) males and 89 (40.5%) females. The average duration on hemodialysis was 5.3 ± 2.8 years. Hypertension (89.5%) and ischemic heart disease (24.1%) were prominent comorbidities. Hypertensive nephropathy (42.7%) was the leading cause of ESRD. Left ventricular hypertrophy was mild in most cases (85.5%), whereas regional wall motion abnormality (RWMA) was common (67.3%). The average pulmonary artery pressure was 35.2 ± 15.3 mmHg. Out of 220 patients, 109 patients (49.8%) of them had mild PH, nine patients (4.1%) had severe PH, and 72 patients (32.7%) had moderate PH. Associations between PH and various factors were examined. RWMA, left ventricular hypertrophy, and left ventricular ejection fraction were significantly associated with PH (p < 0.001). Serum calcium and albumin levels were also associated with PH severity (p < 0.05). Other demographic and laboratory parameters did not show a significant association. Conclusion This study highlights the prevalence of PH in MHD patients and identifies associated risk factors. Understanding these associations can aid in better managing PH in ESRD patients.

Improving accuracy of vascular access quality classification in hemodialysis patients using deep learning with K highest score feature selection.

OBJECTIVE: To develop and evaluate a novel feature selection technique, using photoplethysmography (PPG) sensors, for enhancing the performance of deep learning models in classifying vascular access quality in hemodialysis patients. METHODS: This cross-sectional study involved creating a novel feature selection method based on SelectKBest principles, specifically designed to optimize deep learning models for PPG sensor data, in hemodialysis patients. The method effectiveness was assessed by comparing the performance of multiple deep learning models using the feature selection approach versus complete feature set. The model with the highest accuracy was then trained and tested using a 70:30 approach, respectively, with the full dataset and the SelectKBest dataset. Performance results were compared using Student's paired t-test. RESULTS: Data from 398 hemodialysis patients were included. The 1-dimensional convolutional neural network (CNN1D) displayed the highest accuracy among different models. Implementation of the SelectKBest-based feature selection technique resulted in a statistically significant improvement in the CNN1D model's performance, achieving an accuracy of 92.05% (with feature selection) versus 90.79% (with full feature set). CONCLUSION: These findings suggest that the newly developed feature selection approach might aid in accurately predicting vascular access quality in hemodialysis patients. This advancement may contribute to the development of reliable diagnostic tools for identifying vascular complications, such as stenosis, potentially improving patient outcomes and their quality of life.

Venous excess ultrasonography (VExUS) captures dynamic changes in volume status surrounding hemodialysis: A multicenter prospective observational study.

Background: The evaluation of volume status is essential to clinical decision-making, yet multiple studies have shown that physical exam does not reliably estimate a patient's intravascular volume. Venous excess ultrasound score (VExUS) is an emerging volume assessment tool that utilizes inferior vena cava (IVC) diameter and pulse-wave Doppler waveforms of the portal, hepatic and renal veins to evaluate venous congestion. A point-of-care ultrasound exam initially developed by Beaubein-Souligny et al., VExUS represents a reproducible, non-invasive and accurate means of assessing intravascular congestion. VExUS has recently been validated against RHC-the gold-standard of hemodynamic evaluation for volume assessment. While VExUS scores were shown to correlate with elevated cardiac filling pressures (i.e., right atrial pressure (RAP) and pulmonary capillary wedge pressure (PCWP)) at a static point in time, the ability of VExUS to capture dynamic changes in volume status has yet to be elucidated. We hypothesized that paired VExUS examinations performed before and after hemodialysis (HD) would reflect changes in venous congestion in a diverse patient population. Methods: Inpatients with end-stage renal disease undergoing intermittent HD were evaluated with transabdominal VExUS and lung ultrasonography before and following HD. Paired t-tests were conducted to assess differences between pre-HD and post-HD VExUS scores, B-line scores and dyspnea scores. Results: Fifty-six patients were screened for inclusion in this study. Ten were excluded due to insufficient image quality or incomplete exams, and forty-six patients (ninety-two paired ultrasound exams) were included in the final analysis. Paired t-test analysis of pre-HD and post-HD VExUS scores revealed a mean VExUS grade change of 0.82 (p<0.001) on a VExUS scale ranging from 0 to 4. The mean difference in B-line score following HD was 0.8 (p=0.001). There was no statistically significant difference in subjective dyspnea score (p=0.41). Conclusions: Large-volume fluid removal with HD was represented by changes in VExUS score, highlighting the utility of the VExUS exam to capture dynamic shifts in intravascular volume status. Future studies should evaluate change in VExUS grade with intravenous fluid or diuretic administration, with the ultimate goal of evaluating the capacity of a standardized bedside ultrasound protocol to guide inpatient volume optimization.

Health-related quality of life for pediatric patients with end-stage kidney disease: A systematic review and meta-analysis of the Pediatric Quality of Life Inventory (PedsQL).

INTRODUCTION: Health-related quality of life (HRQoL) studies demonstrate the impact of end-stage renal disease (ESRD) on the physical and psychosocial development of children. While several instruments are used to measure HRQoL, few have standardized domains specific to pediatric ESRD. This review examines current evidence on self and proxy-reported HRQoL among pediatric patients with ESRD, based on the Pediatric Quality of Life Inventory (PedsQL) questionnaires. METHODS: Following PRISMA guidelines, we conducted a systematic review and meta-analysis on HRQoL using the PedsQL 4.0 Generic Core Scale (GCS) and the PedsQL 3.0 ESRD Module among 5- to 18-year-old patients. We queried PubMed, Embase, Web of Science, CINAHL, and Cochrane databases. Retrospective, case-controlled, and cross-sectional studies using PedsQL were included. FINDINGS: Of 435 identified studies, 14 met inclusion criteria administered in several countries. Meta-analysis demonstrated a significantly higher total HRQoL for healthy patients over those with ESRD (SMD:1.44 [95% CI: 0.78-2.09]) across all dimensional scores. In addition, kidney transplant patients reported a significantly higher HRQoL than those on dialysis (PedsQL GCS, SMD: 0.33 [95% CI: 0.14-0.53]) and (PedsQL ESRD, SMD: 0.65 [95% CI: 0.39-0.90]) concordant with parent-proxy reports. DISCUSSION: Patients with ESRD reported lower HRQoL in physical and psychosocial domains compared with healthy controls, while transplant and peritoneal dialysis patients reported better HRQoL than those on hemodialysis. This analysis demonstrates the need to identify dimensions of impaired functioning and produce congruent clinical interventions. Further research on the impact of individual comorbidities in HRQoL is necessary for developing comprehensive, integrated, and holistic treatment programs.