Webcam Use in the Neonatal Intensive Care Unit during the First Year of the SARS-CoV-2 Pandemic.

OBJECTIVE: The aim of this study was to evaluate patterns of webcam use in families of patients admitted to a neonatal intensive care unit (NICU) during the first year of the pandemic and characterize the families who used the technology. STUDY DESIGN: Retrospective chart review of the medical records and logins of our live webcam system was conducted for 2020. RESULTS: From January 1, 2020 to December 31, 2020, 843 infants were admitted to the NICU, with lengths of stay range of 1 to 169 days. More than half (n = 496, 58.8%) of all families of infants admitted to the NICU used the webcam system during the period of study. The number of webcam users did not change between the pre-coronavirus disease 2019 (COVID-19) and during COVID-19 time periods, or during versus after NICU visitor restrictions. Among webcam users, the median (interquartile range) number of logins per day was 2.9 (2.9) and ranged from 0 to 44. There were significant differences among races, site of admission, and diagnosis groups. Families of White infants had more frequent use compared with families of Black infants. Families of infants admitted for surgical reasons had more frequent use compared with those admitted for medical reasons. Among 284 of the 496 (57.3%) webcam users, we documented logins from 37 states in the United States and from 10 different countries. CONCLUSION: Webcam use is common in the NICU. There was no increase in webcam use within the first year of the COVID-19 pandemic in the NICU. More studies are needed to evaluate how this technology is used in the NICU and its impact on patients and families. KEY POINTS: · Webcam use in the NICU is common.. · Visitor restrictions did not increase webcam use.. · We observed racial differences in webcam use..

Utility of the 2018 revised ISN/RPS thresholds for glomerular crescents in childhood-onset lupus nephritis: a Pediatric Nephrology Research Consortium study.

BACKGROUND: The revised 2018 ISN/RPS Classification System for lupus nephritis (LN) includes calculations for both activity index (A.I.) and chronicity index (C.I.). Unchanged were the thresholds of < 25%, 25-50%, and > 50% crescents to distinguish between mild, moderate, and severe activity/chronicity. We aimed to evaluate these thresholds for percent crescents in childhood-onset LN. METHODS: Eighty-six subjects < 21 years of age were enrolled from the Pediatric Glomerulonephritis with Crescents Registry, a retrospective multi-center cohort sponsored by the Pediatric Nephrology Research Consortium. Thresholds of 10%, 25%, and 50% for both cellular/fibrocellular and fibrous crescents were interrogated for primary outcomes of kidney failure, eGFR, and eGFR slope. RESULTS: Median age at time of initial biopsy was 14 years (range 1-21). Median follow-up time was 3 years (range 1-11). Cumulative incidence of kidney failure was 6% at 1 year and 10% at latest follow-up. Median eGFR slope was - 18 mL/1.73 m2/min (IQR - 51 to + 8) at 1 year and - 3 mL/min/1.73 m2/year (IQR - 19 to + 6) at latest follow-up. We found no difference in kidney failure at the proposed < 25% and 25-50% cellular crescents thresholds, and thus added a new provisional threshold of 10% that better predicted outcomes in children. Moreover, use of 10% and 25% thresholds for fibrous crescents showed a fourfold and sevenfold increase in risk of kidney failure. CONCLUSIONS: In children with crescentic LN, use of 10% and 25% thresholds for cellular crescents better reflects disease activity, while these thresholds for fibrous crescents better discriminates kidney disease outcomes. A higher resolution version of the Graphical abstract is available as Supplementary information.

Substance use among adolescents and young adults with chronic kidney disease or kidney failure.

Substance use, a significant public health issue, is well described in the adult chronic kidney disease (CKD) population. Knowledge about substance use in the adolescent and young adult (AYA) CKD population such as prevalence, impact on kidney function, medication adherence, and psychosocial well-being remain largely unknown. Awareness of and inquiring about substance use is paramount to providing evidence-based care and preparation to transition to adult-focused health services. The authors in this review identify commonly used substances (alcohol, tobacco, marijuana, etc.) and how they impact kidney function and care of the AYA with CKD or kidney failure. Recommendations for screening and intervention strategies are provided.

Eculizumab exposure in children and young adults: indications, practice patterns, and outcomes-a Pediatric Nephrology Research Consortium study.

BACKGROUND: Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab. METHODS: A retrospective, cohort analysis was performed on the clinical and biomarker characteristics of eculizumab-exposed patients < 25 years of age seen across 21 centers of the Pediatric Nephrology Research Consortium. Patients were included if they received at least one dose of eculizumab between 2008 and 2015. Traditional summary statistics were applied to demographic and clinical data. RESULTS: A total of 152 patients were identified, mean age 9.1 (+/-6.8) years. Eculizumab was used "off-label" in 44% of cases. The most common diagnoses were aHUS (47.4%), Shiga toxin-producing Escherichia coli HUS (12%), unspecified thrombotic microangiopathies (9%), and glomerulonephritis (9%). Genetic testing was available for 60% of patients; 20% had gene variants. Dosing regimens were variable. Kidney outcomes tended to vary according to diagnosis. Infectious adverse events were the most common adverse event (33.5%). No cases of meningitis were reported. Nine patients died of noninfectious causes while on therapy. CONCLUSIONS: This multi-center retrospective cohort analysis indicates that a significant number of children and young adults are being exposed to C5 blockade for off-label indications. Dosing schedules were highly variable, limiting outcome conclusions. Attributable adverse events appeared to be low. Cohort mortality (6.6%) was not insignificant. Prospective studies in homogenous disease cohorts are needed to support the role of C5 blockade in kidney outcomes.

HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome.

Steroid-sensitive nephrotic syndrome (SSNS) accounts for >80% of cases of nephrotic syndrome in childhood. However, the etiology and pathogenesis of SSNS remain obscure. Hypothesizing that coding variation may underlie SSNS risk, we conducted an exome array association study of SSNS. We enrolled a discovery set of 363 persons (214 South Asian children with SSNS and 149 controls) and genotyped them using the Illumina HumanExome Beadchip. Four common single nucleotide polymorphisms (SNPs) in HLA-DQA1 and HLA-DQB1 (rs1129740, rs9273349, rs1071630, and rs1140343) were significantly associated with SSNS at or near the Bonferroni-adjusted P value for the number of single variants that were tested (odds ratio, 2.11; 95% confidence interval, 1.56 to 2.86; P=1.68×10(-6) (Fisher exact test). Two of these SNPs-the missense variants C34Y (rs1129740) and F41S (rs1071630) in HLA-DQA1-were replicated in an independent cohort of children of white European ancestry with SSNS (100 cases and ≤589 controls; P=1.42×10(-17)). In the rare variant gene set-based analysis, the best signal was found in PLCG2 (P=7.825×10(-5)). In conclusion, this exome array study identified HLA-DQA1 and PLCG2 missense coding variants as candidate loci for SSNS. The finding of a MHC class II locus underlying SSNS risk suggests a major role for immune response in the pathogenesis of SSNS.

Tobacco and the pediatric chronic kidney disease population.

Tobacco use and exposure are preventable causes of morbidity and mortality. Whereas the impact of this public health issue is well described in adults with kidney disease, its role in the pediatric chronic kidney disease (CKD) population is largely unknown. This review discusses the prevalence of tobacco use and exposure in children with CKD, updates the reader on how tobacco affects the kidney, and presents intervention strategies relevant to this patient population.

Tobacco exposure in children and adolescents with chronic kidney disease: parental behavior and knowledge. A study from the Midwest Pediatric Nephrology Consortium.

AIM: The incidence of cardiovascular disease (CVD) in children with chronic kidney disease (CKD) is high. Exposure to second hand smoke (SHS) is a known risk factor for CVD. Due to a recent report of high incidence of SHS in children with CKD, we sought to investigate via questionnaire the smoking behaviors of caregivers of children with CKD. MATERIAL AND METHODS: A cross sectional study was conducted in which caregivers of children and adolescents with CKD were asked to complete a single anonymous self-administered survey. RESULTS: Almost 40% of children and adolescents lived with one or more smokers. Over half of smokers smoked in the presence of their children and in the car. Smokers were significantly less aware of the detrimental effect of SHS exposure on the renal health of their children. Among smokers, almost 70% reported they had not been advised by their child's nephrologist to quit tobacco use. CONCLUSION: There is a high prevalence of SHS exposure among children and adolescents with CKD, which may contribute to CVD. Caregivers are not fully aware of the detrimental effects of SHS exposure on the renal health of their children.

Reducing Discharge Medication Reconciliation Errors at a Pediatric Neurology Inpatient Unit.

Background and Objectives: Medication reconciliation errors are a common problem in health care, particularly during transitions of care. Discharge medication reconciliation (DMR) errors in a pediatric setting can range from 26% to 42.2%. We conducted a quality improvement project to decrease DMR error rate at Dayton Children's Hospital in Dayton, Ohio. Methods: We conducted 2 interventions, each with 3 Plan-Do-Study-Act cycles from September 2021 through February 2023. The first intervention focused on using current specialty neurology nurses as scribes and creating a template note to include the plan of care and review of DMR before discharge. Our second intervention consisted of standardizing the seizure rescue medication order by creating an order panel within our electronic medical record system for all the rescue medications presently available. Medication errors were documented by the specialty neurology nurse during a phone conversation on the next business day post discharge. DMR error rates were calculated for each week using a control chart. Medication errors and patient harm were classified according to the National Coordinating Council for Medication Error Reporting and Prevention Index. Results: One hundred six errors were noted. Of these, 98 (92%) occurred in patients with seizure and 64 (60%) were related to prescription of seizure rescue medication specifically. The baseline error rate was calculated at 15.7% or 7 errors per month (January 2021 through June 2021). The average error rate dropped from 15.7% to 5.3% (2 errors per month) after initiation of our first intervention (September 2021). Twelve weeks after initiation of the second intervention, a 2.9% (1 error per month) was noted. Afterward, there was a ten-week period of 0% errors. Discussion: Sustainable reduction of DMR errors in pediatric patients with epilepsy was achieved by using specialty neurology nurses to scribe the care plan and creating order panels to facilitate accuracy of discharge medication orders without additional cost to the hospital.

Secondhand smoke exposure is associated with proteinuria in children with chronic kidney disease.

BACKGROUND: In adults with chronic kidney disease (CKD), cigarette smoking is associated with an increased risk for CKD progression and transplant failure. In children, secondhand smoke (SHS) exposure has been associated with elevated blood pressure. There are no studies on the prevalence and effect of SHS exposure in CKD. METHODS: Subjects were enrolled in the Chronic Kidney Disease in Children (CKiD) Study, an observational cohort of 366 children aged 1 to 16 years with CKD. Secondhand smoke exposure was obtained via questionnaire. SHS exposure was also determined based on urine cotinine (Ucot) measurements (1 ng/mL ≤ Ucot < 75 ng/mL). The cross-sectional association of SHS exposure with proteinuria was assessed. RESULTS: Using Ucot, 22 % of subjects were exposed to SHS. SHS exposure was significantly associated with lower maternal education and African American race, and a greater prevalence of nephrotic range proteinuria and left ventricular hypertrophy. In a multivariate model (including sex, age, race, maternal education, income level, private insurance status, abnormal birth history and CKD diagnosis), the prevalence odds of nephrotic range proteinuria was 2.64, (95 % confidence interval 1.08, 6.42) higher in children exposed to SHS compared to those unexposed. CONCLUSIONS: In our cohort of children with CKD, SHS exposure was common (22 %) and independently associated with nephrotic range proteinuria. Exposure to SHS may be an important factor to consider in CKD progression.

Chronic kidney disease, pediatric nephrologists, and tobacco counseling: perceptions and practice patterns. A study from the Midwest Pediatric Nephrology Consortium.

We sought to identify practice patterns of pediatric nephrologists for tobacco counseling, because of a high incidence of secondhand smoke exposure and tobacco use in adolescents with chronic kidney disease. Counseling was minimal for several reasons, thus increasing the risk for heart disease inherent in children with chronic kidney disease.

Cigarette smoking and second-hand smoking exposure in adolescents with chronic kidney disease: a study from the Midwest Pediatric Nephrology Consortium.

BACKGROUND: Smoking and second-hand smoking [SHS] cause significant cardiovascular mortality and morbidity. In healthy individuals and adults with chronic kidney disease [CKD], cigarette smoking is associated with albuminuria, increased risk for CKD, increased graft loss and progression of renal insufficiency. In children, SHS has been associated with higher blood pressure variability, blood pressure load, elevated C-reactive protein and decreased cognitive function. Using a survey document and urine cotinine, we sought to investigate prevalence of cigarette use and SHS in adolescents with CKD. METHODS: A cross-sectional study was conducted in which adolescents aged 13 to 18 years with CKD were asked to complete a single anonymous self-administered survey. In addition, a single freshly voided urine sample for cotinine measurement was obtained from eligible subjects. RESULTS: Of 182 subjects, 60 (34%), 25 (14%) and 93 (52%) were transplant recipients, were dialysis dependent and had a glomerulopathy, respectively. Renal status was lacking in four. Twenty-four per cent (24%) had smoked at some point in their lives, and 13% had smoked within the last 30 days of taking the survey. Fifty-two per cent (52%) of all respondents reported living with an adult who smoked, and 54% reported having friends that smoked. Forty-seven per cent (47%) and 44% of those who had never smoked lived with an adult and had friends that smoked, respectively. There was a discrepancy rate of 7% between self-reported non-smokers and urine cotinine, suggesting smoking rates were higher. The highest cotinine/creatinine levels among the non-smokers were observed in those who lived with a smoker and had friends that smoked. CONCLUSION: Among adolescents with CKD, cigarette smoking and SHS exposure are prevalent and may be important variables to consider when evaluating renal and cardiovascular risk factors and outcomes in children with CKD.

Patient generated health data: Benefits and challenges.

Patient Generated Health Data (PGHD) is defined as data generated by and from patients.1 The use of PGHD has rapidly increased with the widespread availability of smart phone mobile health applications (mHealth apps) and wearable devices. Currently, the vast majority of PGHD is generated via the use of mHealth apps and wearables like "Fitbit" or medical devices such as a continuous glucose monitoring device. There are many benefits of PGHD including increased monitoring of children's chronic health conditions outside clinical care to supplement ambulatory clinic visits, improved health outcomes, increased patient awareness and engagement and improved patient-provider communication. When leveraged properly, PGHD can be a powerful tool in delivering safe, effective, patient centered, efficient and equitable care as outlined by the Institute of Medicine (IOM).2 The challenges that limit collection, use and acceptance of PGHD include limited access to the internet, inability to incorporate PGHD into clinical workflows, data privacy and security concerns and apprehension about accuracy and safety of mHealth apps. These issues can lead to a lack of use or compliance with devices or apps associated with PGHD. To ensure optimal health benefits, agreement to leverage PGHD should be a joint decision between the clinician and the patient/caregiver. Future steps to ensure safety and clinical relevance of PGHD include involving regulatory authorities, device manufacturers and professional bodies to develop standards for mHealth apps and wearables to promote uncomplicated PGHD integration into workflows, easy and secure sharing of PGHD. Wearable technology, medical devices and smart phone apps become more advanced and widespread among the population, there will be an increasing potential for PGHD to facilitate personalized, efficient, and collaborative care resulting in improved health outcomes for children and adolescent and young adult. More research and innovation is needed to facilitate this transition.

Patient Portal, Patient-Generated Images, and Medical Decision-Making in a Pediatric Ambulatory Setting.

BACKGROUND: Electronic health record (EHR) patient portals are a secure electronic method of communicating with health care providers. In addition to sending secure messages, images, and videos generated by families can be sent to providers securely. With the widespread use of smart phones, there has been an increase in patient-generated images (PGI) sent to providers via patient portals. There are few studies that have evaluated the role of PGI in medical decision-making. OBJECTIVES: The study aimed to characterize PGI sent to providers via a patient portal, determine how often PGI-affected medical decision-making, and determine the rate of social PGI sent via patient portal. METHODS: A retrospective chart review of PGI uploaded to a children's hospital's ambulatory patient portal from January 2011 to December 2017 was conducted. Data collected included patient demographics, number and type of images sent, person sending images (patient or parent/guardian), and whether an image-affected medical decision-making. Images were classified as medical related (e.g., blood glucose readings and skin rashes), nonmedical or administrative related (e.g., medical clearance or insurance forms), and social (e.g., self-portraits and camp pictures). RESULTS: One hundred forty-three individuals used the portal a total of 358 times, sending 507 images over the study period. Mean (standard deviation) patient age was 9.5 (5.9) years, 50% were females, 89% were White, and 64% had private insurance. About 9% of images were sent directly by patients and the rest by parents/guardians. A total of 387 (76%) images were sent for medical related reasons, 20% for nonmedical, and 4% were deemed social images. Of the 387 medical related images, 314 (81%) affected medical decision-making. CONCLUSION: PGI-affected medical decision-making in most cases. Additional studies are needed to characterize use of PGI in the pediatric population.

Renal Survival in Children with Glomerulonephritis with Crescents: A Pediatric Nephrology Research Consortium Cohort Study.

There is no evidence-based definition for diagnosing crescentic glomerulonephritis. The prognostic implications of crescentic lesions on kidney biopsy have not been quantified. Our objective was to determine risk factors for end-stage kidney disease (ESKD) in patients with glomerulonephritis and crescents on kidney biopsy. A query of the Pediatric Nephrology Research Consortium's Pediatric Glomerulonephritis with Crescents registry identified 305 patients from 15 centers. A retrospective cohort study was performed with ESKD as the primary outcome. Median age at biopsy was 11 years (range 1-21). The percentage of crescents was 3-100% (median 20%). Etiologies included IgA nephropathy (23%), lupus (21%), IgA vasculitis (19%) and ANCA-associated GN (13%), post-infectious GN (5%), and anti-glomerular basement membrane disease (3%). The prevalence of ESKD was 12% at one year and 16% at last follow-up (median = 3 years, range 1-11). Median time to ESKD was 100 days. Risk factors for ESKD included %crescents, presence of fibrous crescents, estimated GFR, and hypertension at biopsy. For each 1% increase in %crescents, there was a 3% decrease in log odds of 1-year renal survival (p = 0.003) and a 2% decrease in log odds of renal survival at last follow-up (p < 0.001). These findings provide an evidence base for enrollment criteria for crescentic glomerulonephritis in future clinical trials.

Risk Behaviors in Teens with Chronic Kidney Disease: A Study from the Midwest Pediatric Nephrology Consortium.

INTRODUCTION: There is a paucity of information about risk behaviors in adolescents with chronic kidney disease (CKD). We designed this study to assess the prevalence of risk behaviors among teens with CKD in the United States and to investigate any associations between risk behavior and patient or disease characteristics. METHODS: After informed consent, adolescents with CKD completed an anonymous, confidential, electronic web-based questionnaire to measure risk behaviors within five domains: sex, teen driving, alcohol and tobacco consumption, illicit drug use, and depression-related risk behavior. The reference group was composed of age-, gender-, and race-matched US high school students. RESULTS: When compared with controls, teens with CKD showed significantly lower prevalence of risk behaviors, except for similar use of alcohol or illicit substances during sex (22.5% vs. 20.8%, p=0.71), feeling depressed for ≥2 weeks (24.3% vs. 29.1%, p=0.07), and suicide attempt resulting in injury needing medical attention (36.4% vs. 32.5%, p=0.78). Furthermore, the CKD group had low risk perception of cigarettes (28%), alcohol (34%), marijuana (50%), and illicit prescription drug (28%). Use of two or more substances was significantly associated with depression and suicidal attempts (p < 0.05) among teens with CKD. CONCLUSIONS: Teens with CKD showed significantly lower prevalence of risk behaviors than controls. Certain patient characteristics were associated with increased risk behaviors among the CKD group. These data are somewhat reassuring, but children with CKD still need routine assessment of and counselling about risk behaviors.

Racial differences in pediatric renal transplantation-24-year single center experience.

Racial disparities in quality of health are major issues affecting healthcare delivery. While substantial studies have been done in adults with end-stage renal disease (ESRD), similar studies are lacking in pediatric patients with ESRD. We retrospectively analyzed our transplant database from 1978-2002 to identify racial trends in a pediatric ESRD population. Significant racial differences were noted in number of pre-emptive transplants, type of graft received, height at transplant and causes of ESRD.