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We investigated the risks of post-acute and chronic adverse kidney outcomes of SARS-CoV-2 infection in the pediatric population via a retrospective cohort study using data from the RECOVER program. We included 1,864,637 children and adolescents under 21 from 19 children's hospitals and health institutions in the US with at least six months of follow-up time between March 2020 and May 2023. We divided the patients into three strata: patients with pre-existing chronic kidney disease (CKD), patients with acute kidney injury (AKI) during the acute phase (within 28 days) of SARS-CoV-2 infection, and patients without pre-existing CKD or AKI. We defined a set of adverse kidney outcomes for each stratum and examined the outcomes within the post-acute and chronic phases after SARS-CoV-2 infection. In each stratum, compared with the non-infected group, patients with COVID-19 had a higher risk of adverse kidney outcomes. For patients without pre-existing CKD, there were increased risks of CKD stage 2+ (HR 1.20; 95% CI: 1.13-1.28) and CKD stage 3+ (HR 1.35; 95% CI: 1.15-1.59) during the post-acute phase (28 days to 365 days) after SARS-CoV-2 infection. Within the post-acute phase of SARS-CoV-2 infection, children and adolescents with pre-existing CKD and those who experienced AKI were at increased risk of progression to a composite outcome defined by at least 50% decline in estimated glomerular filtration rate (eGFR), eGFR <15 mL/min/1.73m2, End Stage Kidney Disease diagnosis, dialysis, or transplant.
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IMPORTANCE: Pediatric patients with complex medical problems benefit from pediatric sub-specialty care; however, a significant proportion of children live greater than 80 mi. away from pediatric sub-specialty care. OBJECTIVE: To identify current knowledge gaps and outline concrete next steps to make progress on issues that have persistently challenged the pediatric nephrology workforce. EVIDENCE REVIEW: Workforce Summit 2.0 employed the round table format and methodology for consensus building using adapted Delphi principles. Content domains were identified via input from the ASPN Workforce Committee, the ASPN's 2023 Strategic Plan survey, the ASPN's Pediatric Nephrology Division Directors survey, and ongoing feedback from ASPN members. Working groups met prior to the Summit to conduct an organized literature review and establish key questions to be addressed. The Summit was held in-person in November 2023. During the Summit, work groups presented their preliminary findings, and the at-large group developed the key action statements and future directions. FINDINGS: A holistic appraisal of the effort required to cover inpatient and outpatient sub-specialty care will help define faculty effort and time distribution. Most pediatric nephrologists practice in academic settings, so work beyond clinical care including education, research, advocacy, and administrative/service tasks may form a substantial amount of a faculty member's time and effort. An academic relative value unit (RVU) may assist in creating a more inclusive assessment of their contributions to their academic practice. Pediatric sub-specialties, such as nephrology, contribute to the clinical mission and care of their institutions beyond their direct billable RVUs. Advocacy throughout the field of pediatrics is necessary in order for reimbursement of pediatric sub-specialist care to accurately reflect the time and effort required to address complex care needs. Flexible, individualized training pathways may improve recruitment into sub-specialty fields such as nephrology. CONCLUSIONS AND RELEVANCE: The workforce crisis facing the pediatric nephrology field is echoed throughout many pediatric sub-specialties. Efforts to improve recruitment, retention, and reimbursement are necessary to improve the care delivered to pediatric patients.
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BACKGROUND: Sugammadex is a pharmacologic agent that provides rapid reversal of neuromuscular blockade via encapsulation of the neuromuscular blocking agent (NMBA). The sugammadex-NMBA complex is primarily cleared through glomerular filtration from the kidney, raising the possibility that alterations in renal function could affect its elimination. In pediatric patients, the benefits of sugammadex have led to widespread utilization; however, there is limited information on its application in pediatric renal impairment. This study examined sugammadex use and postoperative outcomes in pediatric patients with severe chronic renal impairment at our quaternary pediatric referral hospital. METHODS: After IRB approval, we performed a retrospective analysis in pediatric patients with stage IV and V chronic kidney disease who received sugammadex from January 2017 to March 2022. Postoperative outcomes studied included new or increased respiratory requirement, unplanned intensive care unit (ICU) admission, postoperative pneumonia, anaphylaxis, and death within 48 h postoperatively, unplanned deferral of intraoperative extubation, and repeat administrations of NMBA reversal after leaving the operating room. RESULTS: The final cohort included 17 patients ranging from 8 months to 16 years old. One patient required new postoperative noninvasive ventilation on postoperative day 2, which was credited to hypervolemia. Another patient had bronchospasm intraoperatively resolving with medication, which could not definitively be associated sugammadex administration. There were no instances of deferred extubation, unplanned ICU or need for supplemental oxygen after tracheal extubation identified. CONCLUSION: No adverse effects directly attributable to sugammadex in pediatric patients with severe renal impairment were detected. There may be a role for utilization of sugammadex for neuromuscular reversal in this population.
Subject(s)
Neuromuscular Blockade , Renal Insufficiency, Chronic , Sugammadex , Humans , Sugammadex/administration & dosage , Retrospective Studies , Child , Male , Female , Adolescent , Child, Preschool , Infant , Neuromuscular Blockade/methods , Postoperative Complications , Neuromuscular Nondepolarizing Agents/administration & dosageABSTRACT
Primary glomerular diseases are rare entities. This has hampered efforts to better understand the underlying pathobiology and to develop novel safe and effective therapies. NEPTUNE is a rare disease network that is focused on patients of all ages with minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. It is a longitudinal cohort study that collects detailed demographic, clinical, histopathologic, genomic, transcriptomic, and metabolomic data. The goal is to develop a molecular classification for these disorders that supersedes the traditional pathological features-based schema. Pediatric patients are important contributors to this ongoing project. In this review, we provide a snapshot of the children and adolescents enrolled in NEPTUNE and summarize some key observations that have been made based on the data accumulated during the study. In addition, we describe the development of NEPTUNE Match, a program that aims to leverage the multi-scalar information gathered for each individual patient to provide guidance about potential clinical trial participation based on the molecular characterization and non-invasive biomarker profile. This represents the first organized effort to apply principles of precision medicine to the treatment of patients with primary glomerular disease. NEPTUNE has proven to be an invaluable asset in the study of glomerular diseases in patients of all ages including children and adolescents.
Subject(s)
Glomerulosclerosis, Focal Segmental , Humans , Child , Adolescent , Glomerulosclerosis, Focal Segmental/genetics , Male , Female , Glomerulonephritis, Membranous/pathology , Glomerulonephritis, Membranous/genetics , Longitudinal Studies , Nephrosis, Lipoid/diagnosis , Rare Diseases/genetics , Rare Diseases/therapy , Rare Diseases/diagnosis , Child, Preschool , Cohort Studies , Precision Medicine/methodsSubject(s)
Kidney Diseases , Child , Humans , Kidney Diseases/diagnosis , Kidney Diseases/epidemiology , Kidney Diseases/therapyABSTRACT
Rationale & Objective: PRESERVE seeks to provide new knowledge to inform shared decision-making regarding blood pressure (BP) management for pediatric chronic kidney disease (CKD). PRESERVE will compare the effectiveness of alternative strategies for monitoring and treating hypertension on preserving kidney function; expand the National Patient-Centered Clinical Research Network (PCORnet) common data model by adding pediatric- and kidney-specific variables and linking electronic health record data to other kidney disease databases; and assess the lived experiences of patients related to BP management. Study Design: Multicenter retrospective cohort study (clinical outcomes) and cross-sectional study (patient-reported outcomes [PROs]). Setting & Participants: PRESERVE will include approximately 20,000 children between January 2009-December 2022 with mild-moderate CKD from 15 health care institutions that participate in 6 PCORnet Clinical Research Networks (PEDSnet, STAR, GPC, PaTH, CAPRiCORN, and OneFlorida+). The inclusion criteria were ≥1 nephrologist visit and ≥2 estimated glomerular filtration rate (eGFR) values in the range of 30 to <90 mL/min/1.73 m2 separated by ≥90 days without an intervening value ≥90 mL/min/1.73 m2 and no prior dialysis or kidney transplant. Exposures: BP measurements (clinic-based and 24-hour ambulatory BP); urine protein; and antihypertensive treatment by therapeutic class. Outcomes: The primary outcome is a composite event of a 50% reduction in eGFR, eGFR of <15 mL/min/1.73 m2, long-term dialysis or kidney transplant. Secondary outcomes include change in eGFR, adverse events, and PROs. Analytical Approach: Longitudinal models for dichotomous (proportional hazards or accelerated failure time) and continuous (generalized linear mixed models) clinical outcomes; multivariable linear regression for PROs. We will evaluate heterogeneity of treatment effect by CKD etiology and degree of proteinuria and will examine variation in hypertension management and outcomes based on socio-demographics. Limitations: Causal inference limited by observational analyses. Conclusions: PRESERVE will leverage the PCORnet infrastructure to conduct large-scale observational studies that address BP management knowledge gaps for pediatric CKD, focusing on outcomes that are meaningful to patients. Plain-Language Summary: Hypertension is a major modifiable contributor to loss of kidney function in chronic kidney disease (CKD). The purpose of PRESERVE is to provide evidence to inform shared decision-making regarding blood pressure management for children with CKD. PRESERVE is a consortium of 16 health care institutions in PCORnet, the National Patient-Centered Clinical Research Network, and includes electronic health record data for >19,000 children with CKD. PRESERVE will (1) expand the PCORnet infrastructure for research in pediatric CKD by adding kidney-specific variables and linking electronic health record data to other kidney disease databases; (2) compare the effectiveness of alternative strategies for monitoring and treating hypertension on preserving kidney function; and (3) assess the lived experiences of patients and caregivers related to blood pressure management.
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RATIONALE & OBJECTIVE: The lived experience of children with chronic kidney disease (CKD) is poorly characterized. We examined the associations between patient-reported outcome (PRO) scores measuring their fatigue, sleep health, psychological distress, family relationships, and global health with clinical outcomes over time in children, adolescents, and younger adults with CKD and investigated how the PRO scores of this group compare with those of other children, adolescents, and younger adults. STUDY DESIGN: Prospective cohort study. SETTING & PARTICIPANTS: 212 children, adolescentss, and adults aged 8 to 21 years with CKD and their parents recruited from 16 nephrology programs across North America. PREDICTORS: CKD stage, disease etiology, and sociodemographic and clinical variables. OUTCOME: PRO scores over 2 years. ANALYTICAL APPROACH: We compared PRO scores in the CKD sample with a nationally representative general pediatric population (ages 8 to 17 years). Change of PROs over time and association of sociodemographic and clinical variables with PROs were assessed using multivariable regression models. RESULTS: For all time points, 84% of the parents and 77% of the children, adolescents, and younger adults completed PRO surveys . The baseline PRO scores for the participants with CKD revealed a higher burden of fatigue, sleep-related impairment, psychological distress, impaired global health, and poorer family relationships compared with the general pediatric population, with median score differences≥1 SD for fatigue and global health. The baseline PRO scores did not differ by CKD stage or glomerular versus nonglomerular etiology. Over 2 years, PROs were stable with a<1-point annual change on average on each measure and intraclass correlation coefficients ranging from 0.53 to 0.79, indicating high stability. Hospitalization and parent-reported sleep problems were associated with worse fatigue, psychological health, and global health scores (all P<0.04). LIMITATIONS: We were unable to assess responsiveness to change with dialysis or transplant. CONCLUSIONS: Children with CKD experience a high yet stable burden of impairment across numerous PRO measures, especially fatigue and global health, independent of disease severity. These findings underscore the importance of assessing PROs, including fatigue and sleep measures, in this vulnerable population. PLAIN-LANGUAGE SUMMARY: Children with chronic kidney disease (CKD) have many treatment demands and experience many systemic effects. How CKD impacts the daily life of a child is poorly understood. We surveyed 212 children, adolescents, and younger adults with CKD and their parents over 24 months to assess the participants' well-being over time. Among children, adolescents, and younger adults with CKD we found a very high and persistent burden of psychological distress that did not differ by degree of CKD or type of kidney disease. The participants with CKD endorsed greater impairment in fatigue and global health compared with healthy children, adolescents, and younger adults, and parent-reported sleep problems were associated with poorer patient-reported outcome (PRO) scores across all domains. These findings emphasize the importance of including PRO measures, including fatigue and sleep measures, into routine clinical care to optimize the lived experience of children with CKD.
Subject(s)
Renal Insufficiency, Chronic , Sleep Wake Disorders , Adolescent , Child , Humans , Cohort Studies , Fatigue/epidemiology , Fatigue/etiology , Patient Reported Outcome Measures , Prospective Studies , Renal Insufficiency, Chronic/therapy , Sleep Wake Disorders/epidemiology , Sleep Wake Disorders/etiology , Young AdultABSTRACT
OBJECTIVE: Racial and ethnic disparities are commonplace in health care. Research often relies on sociodemographic information recorded in the electronic health record (EHR). Little evidence is available about the accuracy of EHR-recorded sociodemographic information, and none in pediatrics. Our objective was to determine the accuracy of EHR-recorded race and ethnicity compared to self-report. METHODS: Patients/guardians enrolled in two prospective observational studies (10/2014-1/2019) provided self-reported sociodemographic information. Corresponding EHR information was abstracted. EHR information was compared to self-report, considered "gold standard." Agreement was evaluated with Cohen's kappa. RESULTS: A total of 503 patients (42% female, median age 12.8 years) were identified. Self-reported race (N = 484) was 73% White, 16% Black or African American (AA), 4% Asian, 5% multiracial, and 2% other. Self-reported ethnicity (N = 410) was 9% Hispanic/Latino, and 88% non-Hispanic/Latino. Agreement between self-reported and EHR-recorded race was substantial (kappa = 0.77, 95% CI 0.72-0.83). Race was discordant among 10% (47/476). Hispanic/Latino ethnicity also had strong agreement (kappa = 0.77, 95% CI 0.65-0.89). Among those who self-reported Hispanic/Latino and reported race (N = 21), race was less accurately recorded in the EHR (kappa = 0.26, 95% CI 0-0.54). Race did not match among 43% with recorded race (9/21). Among self-reported racial and/or ethnic minorities, 13% (12/164) were misclassified in the EHR as non-Hispanic White. CONCLUSIONS: We found race and ethnicity are often inaccurately recorded in the EHR for patients who self-identify as minorities, leading to under-representation of minorities in the EHR. Inaccurately recorded race and ethnicity has important implications for disparity research, and for informing health policy. Reliable processes are needed to incorporate self-reported race and ethnicity in the EHR at institutional and national levels.
Subject(s)
Electronic Health Records , Ethnicity , Racial Groups , Self Report , Child , Female , Humans , Male , Hispanic or Latino , Minority Groups , Black or African American , WhiteABSTRACT
BACKGROUND: Renovascular hypertension (RVHTN) is a rare, often complex condition due to multiple etiologies including congenital stenoses, vasculitides, and fibromuscular dysplasia. Among children with RVHTN who require multiple and escalating medications to control blood pressure, the optimal timing of a procedural intervention involves a balance of numerous factors. CASE-DIAGNOSIS/TREATMENT: In this presentation of a 1-month-old girl with RVHTN, the treating medical team had to consider multiple factors in the initial management and timing of interventions to treat her underlying cause of RVHTN, including concerns for kidney health, degree of hypertension, age and size of the patient, and potential methods of procedural intervention. Initially, she was treated conservatively until concern for poor renal growth arose and a durable surgical intervention was thought feasible and safe. CONCLUSION: The evidence regarding the timing of non-medical interventions in pediatric RVHTN is limited. Considerations should include patient age, size, disease severity, comorbid conditions, and degree of medical management required to maintain safe blood pressures that allow for growth and reverse cardiac damage. The optimal interventions have not been evaluated by controlled trials and should be decided on a case-by-case basis with consideration of center expertise and family preferences.
Subject(s)
Fibromuscular Dysplasia , Hypertension, Renovascular , Hypertension , Renal Artery Obstruction , Blood Pressure , Child , Female , Humans , Hypertension, Renovascular/etiology , Hypertension, Renovascular/therapy , Infant , Renal Artery Obstruction/surgeryABSTRACT
RATIONALE & OBJECTIVE: The impact of chronic kidney disease (CKD) on inpatient health care use is unknown. This study aimed to describe the prevalence of pediatric CKD among children hospitalized in the United States and examine the association of CKD with hospital outcomes. STUDY DESIGN: Cross-sectional national survey of pediatric discharges. SETTING & PARTICIPANTS: Hospital discharges of children (aged>28 days to 19 years) with a chronic medical diagnosis included in the Healthcare Cost and Utilization Project Kids' Inpatient Database for 2006, 2009, 2012, and 2016. PREDICTOR: Presence of primary or coexisting CKD as identified by diagnosis codes. OUTCOMES: Length of stay (LOS), cost, and mortality. ANALYTICAL APPROACH: Multivariable analysis using Poisson, gamma, and logistic regressions were performed for LOS, cost, and mortality, respectively. RESULTS: A chronic medical condition was present in 6,524,745 estimated discharges during the study period and CKD was present among 3.9% of discharges (96.1% without CKD). Those with CKD had a longer LOS (median of 2.8 [IQR, 1.4-6.0] days compared with 1.8 [IQR, 1.0-4.4] days for those without a CKD diagnosis; P<0.001). Median cost was higher in the CKD group compared with the group without CKD, at $8,755 (IQR, $4,563-18,345) and $5,016 (IQR, $2,860-10,109) per hospitalization, respectively (P<0.001). Presence of CKD was associated with a longer LOS (29.9% [95% CI, 27.2%-32.6%] longer than those without CKD), higher cost (61.3% [95% CI, 57.4%-65.4%] greater than those without CKD), and higher risk for mortality (OR, 1.51 [95% CI, 1.40-1.63]). LIMITATIONS: Lack of access to and adjustment for confounders including patient readmission and laboratory data. CONCLUSIONS: Pediatric CKD was associated with longer LOS, higher costs, and higher risk for mortality compared with hospitalizations with other chronic illnesses. Further studies are needed to better understand the health care needs and delivery of care to hospitalized children with CKD.
Subject(s)
Databases, Factual/trends , Hospitalization/trends , Patient Acceptance of Health Care , Renal Insufficiency, Chronic/mortality , Renal Insufficiency, Chronic/therapy , Child , Cohort Studies , Cross-Sectional Studies , Female , Humans , Male , Renal Insufficiency, Chronic/diagnosis , United States/epidemiologyABSTRACT
INTRODUCTION: Autosomal recessive polycystic kidney disease (ARPKD) is a rare cause of renal failure with a highly variable clinical course. Patients who are symptomatic early in life frequently require early nephrectomy and peritoneal dialysis. In these patients there are little data to guide clinicians on whether to select unilateral nephrectomy or bilateral nephrectomy at the initial operative intervention. We review our experience with this disease process. METHODS: A retrospective review was performed of 11 patients at our institution with ARPKD symptomatic within the first month of life. Charts were reviewed for relevant clinical data, and patients were divided into groups based on undergoing either unilateral or bilateral nephrectomy at their initial intervention. The decision for unilateral versus bilateral nephrectomy was decided by the clinical team without any available guidelines. RESULTS: Of the 11 patients reviewed, two patients died within the first two weeks from other complications. The remaining 9 all required nephrectomy, with 5 undergoing synchronous bilateral nephrectomy, and 4 undergoing initial unilateral nephrectomy. All four patients required removal of their contralateral kidney, a median of 25.5â¯days later. There was no difference in mortality, ventilator free days, or time to full feeds between the two groups, although the group undergoing initial unilateral nephrectomy had more TPN days than their counterparts (28 vs 17â¯days, pâ¯=â¯0.014). CONCLUSIONS: In our cohort, there were few significant differences between the groups based on choice of initial unilateral or bilateral nephrectomy, and all children ultimately required removal of both kidneys. These data suggest that anesthetic exposures and other clinical outcomes might be optimized by initial bilateral nephrectomy. LEVEL OF EVIDENCE: III.
Subject(s)
Nephrectomy , Peritoneal Dialysis , Polycystic Kidney, Autosomal Recessive , Renal Insufficiency , Child , Humans , Infant, Newborn , Polycystic Kidney, Autosomal Recessive/complications , Polycystic Kidney, Autosomal Recessive/surgery , Retrospective StudiesABSTRACT
BACKGROUND: Renovascular hypertension (RVH) associated with renal artery and abdominal aortic narrowings is the third most common cause of pediatric hypertension. Untreated children may experience major cardiopulmonary complications, stroke, renal failure, and death. The impetus of this study was to describe the increasingly complex surgical practice for such patients with an emphasis on anatomic phenotype and contemporary outcomes after surgical management as a means of identifying those factors responsible for persistent or recurrent hypertension necessitating reoperation. METHODS: A retrospective analysis was performed of consecutive pediatric patients with RVH undergoing open surgical procedures at the University of Michigan from 1991 to 2017. Anatomic phenotype and patient risk factors were analyzed to predict outcomes of blood pressure control and the need for secondary operations using ordered and binomial logistic multinomial regression models, respectively. RESULTS: There were 169 children (76 girls, 93 boys) who underwent primary index operations at a median age of 8.3 years; 31 children (18%) had neurofibromatosis type 1, 76 (45%) had abdominal aortic coarctations, and 28 (17%) had a single functioning kidney. Before treatment at the University of Michigan, 51 children experienced failed previous open operations (15) or endovascular interventions (36) for RVH at other institutions. Primary surgical interventions (342) included main renal artery (136) and segmental renal artery (10) aortic reimplantation, renal artery bypass (55), segmental renal artery embolization (10), renal artery patch angioplasty (8), resection with reanastomosis (4), and partial or total nephrectomy (25). Non-renal artery procedures included patch aortoplasty (32), aortoaortic bypass (32), and splanchnic arterial revascularization (30). Nine patients required reoperation in the early postoperative period. During a mean follow-up of 49 months, secondary interventions were required in 35 children (21%), including both open surgical (37) and endovascular (14) interventions. Remedial intervention to preserve primary renal artery patency or a nephrectomy if such was impossible was required in 22 children (13%). The remaining secondary procedures were performed to treat previously untreated disease that became clinically evident during follow-up. Age at operation and abdominal aortic coarctation were independent predictors for reoperation. The overall experience revealed hypertension to be cured in 74 children (44%), improved in 78 (46%), and unchanged in 17 (10%). Children undergoing remedial operations were less likely (33%) to be cured of hypertension. There was no perioperative death or renal insufficiency requiring dialysis after either primary or secondary interventions. CONCLUSIONS: Contemporary surgical treatment of pediatric RVH provides a sustainable overall benefit to 90% of children. Interventions in the very young (<3 years) and concurrent abdominal aortic coarctation increase the likelihood of reoperation. Patients undergoing remedial surgery after earlier operative failures are less likely to be cured of hypertension. Judicious postoperative surveillance is imperative in children surgically treated for RVH.
Subject(s)
Aorta, Abdominal/surgery , Aortic Coarctation/surgery , Blood Pressure , Hypertension, Renovascular/surgery , Renal Artery Obstruction/surgery , Vascular Surgical Procedures , Adolescent , Age Factors , Antihypertensive Agents/therapeutic use , Aorta, Abdominal/abnormalities , Aorta, Abdominal/diagnostic imaging , Aorta, Abdominal/physiopathology , Aortic Coarctation/complications , Aortic Coarctation/diagnostic imaging , Aortic Coarctation/physiopathology , Child , Child, Preschool , Female , Humans , Hypertension, Renovascular/diagnosis , Hypertension, Renovascular/etiology , Hypertension, Renovascular/physiopathology , Male , Renal Artery Obstruction/complications , Renal Artery Obstruction/diagnostic imaging , Renal Artery Obstruction/physiopathology , Retrospective Studies , Treatment Outcome , Vascular Surgical Procedures/adverse effectsABSTRACT
Importance: Cardiovascular disease (CVD) is a leading cause of death among patients with end-stage renal disease (ESRD). Young adult (ages 22-29 years) have risks for ESRD-associated CVD that may vary from other ages. Objective: To test the hypothesis that young adult-onset ESRD is associated with higher cardiovascular (CV) hospitalizations and mortality with different characteristics than childhood-onset disease. Design, Setting, and Participants: This population-based cohort study used the US Renal Data System to categorize patients who initiated ESRD care between 2003 and 2013 by age at ESRD onset (1-11, 12-21, and 22-29 years). Cardiovascular hospitalizations were identified via International Classification of Diseases, Ninth Revision discharge codes and CV mortality from the Centers for Medicare & Medicaid ESRD Death Notification Form. Patients were censored at death from non-CVD events, loss to follow-up, recovery, or survival to December 31, 2014. Adjusted proportional hazard models (95% CI) were fit to determine risk of CV hospitalization and mortality by age group. Data analysis occurred from May 2016 and December 2017. Exposures: Onset of ESRD. Main Outcomes and Measures: Cardiovascular mortality and hospitalization. Results: A total of 33â¯156 patients aged 1 to 29 years were included in the study population. Young adults (aged 22-29 years) had a 1-year CV hospitalization rate of 138 (95% CI, 121-159) per 1000 patient-years. Young adults had a higher risk for CV hospitalization than children (aged 1-11 years; hazard ratio [HR], 0.41 [95% CI, 0.26-0.64]) and adolescents (aged 12-21 years; HR, 0.86 [95% CI, 0.77-0.97]). Of 4038 deaths in young adults, 1577 (39.1%) were owing to CVD. Five-year cumulative incidence of mortality in this group (7.3%) was higher than in younger patients (adolescents, 4.0%; children, 1.7%). Adjusted HRs for CV mortality were higher for young adults with all causes of ESRD than children (cystic, hereditary, and congenital conditions: HR, 0.22 [95% CI, 0.11-0.46]; P < .001; glomerulonephritis: HR, 0.21 [95% CI, 0.10-0.44]; P < .001; other conditions: HR, 0.33 [95% CI, 0.23-0.49]; P < .001). Adolescents had a lower risk for CV mortality than young adults for all causes of ESRD except glomerulonephritis (cystic, hereditary, and congenital conditions: HR, 0.45 [95% CI, 0.27-0.74]; glomerulonephritis: HR, 0.99 [95% CI, 0.76-1.11]; other: HR, 0.47 [95% CI, 0.40-0.57]). Higher risks for CV hospitalization and mortality were associated with lack of preemptive transplant compared with hemodialysis (hospital: HR, 14.24 [95% CI, 5.92-34.28]; mortality: HR, 13.64 [95% CI, 8.79-21.14]) and peritoneal dialysis [hospital: HR, 8.47 [95% CI, 3.50-20.53]; mortality: HR, 7.86 [95% CI, 4.96-12.45]). Nephrology care before ESRD was associated with lower risk for CV mortality (HR, 0.77 [95% CI, 0.70-0.85]). Conclusions and Relevance: Cardiovascular disease accounted for nearly 40% of deaths in young adults with incident ESRD in this cohort. Identified risk factors may inform development of age-appropriate ESRD strategies to improve the CV health of this population.
Subject(s)
Cardiovascular Diseases/complications , Hospitalization/statistics & numerical data , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/mortality , Adolescent , Adult , Cardiovascular Diseases/epidemiology , Child , Child, Preschool , Female , Humans , Incidence , Infant , Kidney Failure, Chronic/epidemiology , Kidney Failure, Chronic/therapy , Kidney Transplantation/statistics & numerical data , Male , Mortality/trends , Outcome Assessment, Health Care , Renal Dialysis/statistics & numerical data , Risk Factors , United States/epidemiology , Young AdultABSTRACT
INTRODUCTION: The goal of this study was to assess the occurrence of steroid-associated adverse events (SAAE) in patients with primary proteinuric kidney disease. METHODS: The Kidney Research Network Registry consists of children and adults with primary proteinuric kidney disease. SAAEs of interest were hypertension, hyperglycemia and diabetes, overweight and obesity, short stature, ophthalmologic complications, bone disorders, infections, and psychosis. Events were identified using International Classification of Diseases, Ninth Revision/Tenth Revision codes, blood pressures, growth parameters, laboratory values, and medications. Poisson generalized estimating equations tested the association between steroid onset and dose on SAAE risk. RESULTS: A total of 884 participants were included in the analysis; 534 (60%) were treated with steroids. Of these, 62% had at least one SAAE. The frequency of any SAAE after initiation of steroids was 293 per 1000 person-years. The most common SAAEs were hypertension (173.7 per 1000 person-years), diabetes (78.7 per 1000 person-years), obesity (66.8 per 1000 person-years), and infections (46.1 per 1000 person-years). After adjustment for demographics, duration of kidney disease, estimated glomerular filtration rate (eGFR), proteinuria, and other therapies, steroid exposure was associated with a 40% increase in risk of any SAAE (Relative risk [RR]: 1.4; 95% confidence interval [CI]: 1.3-1.6). A 1-mg/kg per day increase in steroid dose was associated with a 2.5-fold increase in risk of any SAAE. CONCLUSION: Most patients with primary proteinuric kidney disease treated with steroids experienced at least one SAAE. Steroid therapy increased risk of hypertension, diabetes, weight gain, short stature, fractures, and infections after adjusting for disease-related factors. This study highlights the importance of surveillance and management of SAAE and provides rationale for the development of steroid minimization protocols.