Identifying barriers and utility of obtaining ambulatory blood pressure monitoring in a pediatric chronic kidney disease population.

BACKGROUND: Hypertension is a prevalent complication of Chronic Kidney Disease (CKD) and Ambulatory Blood Pressure Monitoring (ABPM) is the gold standard for diagnosis. The aim of our study was to assess the usefulness of obtaining ABPM and to identify barriers to ABPM in this pediatric patient population. METHOD: In this retrospective analysis of patients with CKD stage 3-5 who were seen in one academic medical center's outpatient Pediatric Nephrology clinics between 2018 and 2021, we performed logistic regression to evaluate for associations between demographic factors and odds of having an ABPM. RESULT: Among 96 patients included in the study, 48 patients carried a diagnosis of hypertension. 31 patients had ABPM performed with usable data. In those who had ABPM done, 21 had normotension and 10 had undertreated hypertension. Our study also showed 1 had masked hypertension and 5 had white coat hypertension or effect. We did not find a statistically significant difference in those who did or did not undergo ABPM evaluation based on gender, previous diagnosis of hypertension, distance from clinic, language preference, or racial or ethnic identity. CONCLUSION: ABPM is a useful tool in our CKD population for the diagnosis and management of hypertension. We did not identify specific barriers to ABPM in our CKD population, and there were no differences in patients who obtained ABPM when looking at specific demographic and disease characteristics. Given these findings, we recommend focusing on areas of future improvement in spheres of patient and provider education as well as better quantification using surveys to further illuminate barriers.

Pediatric renal transplant biopsy with ultrasound guidance: the 'core' essentials.

This review provides a comprehensive and practical approach to pediatric percutaneous renal transplant biopsies, highlighting techniques and strategies to optimize adequate sample yield and ensure patient safety. In children with end-stage renal disease, transplantation is the preferred choice of therapy, providing for overall lower long-term morbidity and mortality compared with dialysis. In the ongoing management of renal transplant patients, core tissue sampling via a percutaneous renal biopsy remains the gold standard when transplant dysfunction is suspected. Indications for renal transplant biopsy and techniques/tools for adequate sample yield are discussed. Strategies for common challenges such as poor visualization and renal transplant mobility are addressed. We discuss the clinical signs, techniques and imaging findings for common complications including hematomas, arteriovenous fistulas and pseudoaneurysms. Although the percutaneous renal transplant biopsy procedure is generally safe with rare complications, care must be taken to ensure major complications are promptly recognized and treated. Adequate tissue samples obtained via renal biopsy are imperative to promptly identify transplant rejection to provide valuable information for patient diagnosis, treatment and outcomes. Radiologist and nephrologist attention to proper ultrasound techniques and optimal biopsy tools are critical to ensure tissue adequacy and minimize complications.

Challenges managing end-stage renal disease and kidney transplantation in a child with MTFMT mutation and moyamoya disease.

Moyamoya disease is a chronic cerebrovascular disorder with progressive stenosis. We describe a four-yr-old female with features of moyamoya disease referred to our center for kidney transplant evaluation with ESRD secondary to presumed renal dysplasia along with concern for cerebral vascular anomalies. With her constellation of organ involvement, a genetic workup revealed a homozygous, frameshift mutation in the mitochondrial methionyl-tRNA formyltransferase gene. Given her vascular anomalies and evidence of prior infarcts seen on cerebral imaging, it was felt that her risk of future stroke events was high and that hypotension or intravascular volume depletion would further exacerbate this risk. In hopes of improving her tenuous cerebral perfusion, she underwent a bilateral temporal craniotomy for superficial temporal artery to middle cerebral artery bypass. We highlight the challenges faced in a child with ESRD and kidney transplantation when cerebral vasculature is compromised. A multidisciplinary approach is critical in determining the need for a revascularization procedure prior to transplant and to help reduce the risk of ischemic or hemorrhagic events in this patient population.

Second malignant neoplasms among children, adolescents and young adults with Wilms tumor.

BACKGROUND: The goal of this study was to describe the incidence, characteristics, and outcomes of secondary malignant neoplasms (SMN) in survivors of Wilms tumor. PROCEDURES: Patients who were 0-20 years of age at time of primary diagnosis with Wilms tumor and reported to the Surveillance, Epidemiology, and End Results [SEER] program between 1973 and 2011 were eligible for inclusion in the cohort. We used competing risks methods to estimate the cumulative incidence of SMNs and assess contributing factors for developing SMN. We estimated standardized incidence ratios (SIR), absolute excess risk and overall survival after SMN using standard methods. RESULTS: Within the SEER database, 2,851 patients were diagnosed with Wilms tumor as their first malignancy. Of these, 34 patients were reported to have a SMN. Cumulative incidence of for a secondary malignancy was 0.6% (95% confidence interval [95% CI] 0.3-1.0%) at 10 years, 1.6% (95% CI 1.0-2.3%) at 20 years, and 3.8% (95% CI 2.4-5.9%) at 30 years. Median time from primary diagnosis to SMN diagnosis was 12.5 years. SIR for SMN for survivors of Wilms tumor was 3.4 (95% CI 2.2-4.9) with an absolute excess risk of 7.6 per 10,000 persons per year. Exposure to radiation did not significantly increase risk for development of second malignancy. Overall survival for patients with SMN was 64.5% at 5 years. CONCLUSION: Survivors of Wilms tumor are at an increased risk of SMN compared to the general population, but the added risk is relatively small compared to other pediatric cancers.

Daratumumab therapy in a pediatric case of C3 nephritic factor-positive proliferative glomerulonephritis with monoclonal IgG deposits.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is an exceedingly rare cause of glomerulonephritis among children for which prognosis is generally poor, with low incidence of remission and high rates of recurrence after transplant. While there are more cases reported in the adult literature, substantial differences in pediatric vs. adult PGNMID render it essential that we further characterize pediatric cases to optimize management. We report the case of a 12-year-old male presenting initially with edema and hypertension who was subsequently diagnosed with IgG3/Kappa-dominant PGNMID. In the absence of any proven therapy and though without a detectable clone, he was empirically treated with daratumumab with positive effect to date. This is the first reported case of daratumumab monotherapy in pediatric PGNMID, as well as the first PGNMID case to detect presence of C3 nephritic factor.

A novel approach to patient portal activation data to power equity improvements.

BACKGROUND: There are significant disparities in access and utilization of patient portals by age, language, race, and ethnicity. MATERIALS AND METHODS: We developed ambulatory and inpatient portal activation equity dashboards to understand disparities in initial portal activation, identify targets for improvement, and enable monitoring of interventions over time. We selected key metrics focused on episodes of care and filters to enable high-level overviews and granular data selection to meet the needs of health system leaders and individual clinical units. RESULTS: In addition to highlighting disparities by age, preferred language, race and ethnicity, and insurance payor, the dashboards enabled development and monitoring of interventions to improve portal activation and equity. DISCUSSION AND CONCLUSIONS: Data visualization tools that provide easily accessible, timely, and customizable data can enable a variety of stakeholders to understand and address healthcare disparities, such as patient portal activation. Further institutional efforts are needed to address the persistent inequities highlighted by these dashboards.

Laser acupuncture reduces pain in pediatric kidney biopsies: a randomized controlled trial.

Evaluate laser acupuncture (LA) as an adjuvant therapy in pain management during percutaneous kidney biopsy procedure in children and adolescents. This prospective, double-blinded, randomized controlled trial enrolled patients aged 7 to 26 years admitted to a children's hospital for percutaneous kidney biopsy. Patients received LA to treatment points (acupuncture group) or sham points (control group) before the procedure. The laser delivered a dose of 42 J/cm over 10 acupoints. Patients and parents rated the pain during and after the biopsy, and change in pain scores were calculated for each patient. Anxiety, vital signs, sedation medication, and patient's biopsy experience were secondary outcomes. Sixty-nine treatments (33 in the acupuncture group and 36 in the control group) were eligible for analysis. Patients in the acupuncture group reported a significantly improved change in the pain score after the biopsy compared with the controls (0.8 vs -0.5, P = 0.044). Patients in the acupuncture group had a statistically significant decrease in procedure vital signs including heart rate (-1.8 vs 5.6, P = 0.043) and respiratory rate (-2.4 vs 0.4, P = 0.045) when compared with controls. Parents also perceived a correspondingly greater improvement in their child's pain for those in the acupuncture group compared with the controls (2.3 vs 0.3, P = 0.04). Adjunctive LA significantly improved pain after pediatric percutaneous kidney biopsies.

Myocardial protection of insulin and potassium in a porcine ischemia-reperfusion model.

BACKGROUND: We previously evaluated cardioprotective effects of glucose-insulin-potassium (GIK) in a porcine ischemia-reperfusion model; our results showed less myocardial pH decrease during ischemia and reperfusion and faster normalization of ATP and glucose during reperfusion. The proposed protective mechanism was facilitation of glucose transport for myocardial metabolism. The objective of this study was to assess the impact of insulin-potassium (IK) alone on myocardial metabolism. METHODS: Male swine received continuous infusion of IK (IK group, n = 10), GIK (GIK group, n = 10), or standard lactated Ringer's (LR) solution (controls, n = 10). Induction of 20 minutes of ischemia in the left anterior descending (LAD) artery distribution was followed by 20 minutes of reperfusion. Real-time biosensors recorded pH and glucose levels in ischemic and nonischemic beds. Myocardial biopsies in the distribution of the LAD assessed ATP levels. Groups were compared using the Kruskal-Wallis and Mann-Whitney tests. RESULTS: Real-time data are presented as percent change from baseline. At less than 10 minutes of ischemia, the average pH change was less for the IK group than the LR group (0.03% +/- 0.21% vs -2.06% +/- 1.23%; P = .001), and the pH change in the IK group was similar to the GIK group. After 10 minutes of ischemia and during the first 10 minutes of reperfusion, the IK group experienced pH changes that were similar to the LR group. Biopsies after 20 minutes of ischemia and 20 minutes of reperfusion showed less of a decline in ATP levels for the IK group compared to the LR group. Glucose at all time points demonstrated no statistically significant differences. CONCLUSION: IK infusion alone demonstrates cardioprotective effects during early ischemia; however, compared to GIK infusion after 20 minutes of ischemia and reperfusion, myocardial pH and glucose levels were not sustained. Although insulin may facilitate glucose transport during ischemia, additional glucose in combination with IK enhances myocardial protection during reperfusion. This finding suggests that GIK enhancement during acute ischemia-reperfusion may improve myocardial protection.