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.

Case report: Short-term eculizumab use in atypical HUS associated with Lemierre's syndrome and post-infectious glomerulonephritis.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by genetic abnormalities, infections, autoimmune diseases, drugs, and malignancies. Anti-C5 monoclonal antibody eculizumab is the mainstay of treatment of aHUS caused by the genetic defects of the alternative complement pathway. However, the utility of eculizumab in non-genetic forms of aHUS and the timing of treatment discontinuation remain controversial. Here, we report successful short-term eculizumab use in two young adult patients with aHUS due to rare infectious and autoimmune etiologies: Lemierre's syndrome and post-infectious glomerulonephritis, respectively. Eculizumab was rapidly discontinued in both patients with no aHUS recurrence during long-term follow-up. Considering its favorable safety profile with appropriate meningococcal prophylaxis, eculizumab can be considered as a treatment option for non-genetic aHUS.

Prevention and management of CMV infection in pediatric solid organ transplant recipients.

Human cytomegalovirus (CMV) remains one of the most common opportunistic infections following solid organ transplantation in children. CMV causes morbidity and mortality through direct tissue-invasive disease and indirect immunomodulatory effects. In recent years, several new agents have emerged for the prevention and treatment of CMV disease in solid organ transplant recipients. However, pediatric data remain scarce, and many of the treatments are extrapolated from the adult literature. Controversies exist about the type and duration of prophylactic therapies and the optimal dosing of antiviral agents. This review provides an up-to-date overview of treatment modalities used to prevent and treat CMV disease in solid organ transplant (SOT) recipients.

Cystinuria: An Overview of Diagnosis and Medical Management.

Cystinuria is a genetic disorder that causes recurrent nephrolithiasis. It is the most common type of monogenic stone disease accounting for 6%-8% of pediatric nephrolithiasis. Due to recurrent episodes of nephrolithiasis, it is associated with a very high prevalence of chronic kidney disease. Life-long medical treatment to reduce stone formation is critical in preventing chronic kidney disease and renal failure in cystinuria. In this article, we provide an overview of cystinuria with a special emphasis on medical treatment options including new agents such as alpha-lipoic acid.