Blood Pressure Outcomes in NICU-Admitted Infants with Neonatal Hypertension: A Pediatric Nephrology Research Consortium Study.

OBJECTIVE: To describe the blood pressure outcomes of infants admitted to the neonatal intensive care unit (NICU) with idiopathic (nonsecondary) hypertension (HTN) who were discharged on antihypertensive therapy. STUDY DESIGN: Retrospective, multicenter study of 14 centers within the Pediatric Nephrology Research Consortium. We included all infants with a diagnosis of idiopathic HTN discharged from the NICU on antihypertensive treatment. The primary outcome was time to discontinuation of antihypertensive therapy, grouped into (≤6 months, >6 months to 1 year, and >1 year). Comparisons between groups were made with χ2 tests, Fisher's exact tests, and ANOVA. RESULTS: Data from 118 infants (66% male) were included. Calcium channel blockers were the most prescribed class of antihypertensives (56%) in the cohort. The percentages remaining on antihypertensives after NICU discharge were 60% at 6 months, 26% at 1 year, and 7% at 2 years. Antenatal steroid treatment was associated with decreased likelihood of antihypertensive therapy >1 year after discharge. CONCLUSIONS: This multicenter study reports that most infants admitted to the NICU diagnosed with idiopathic HTN will discontinue antihypertensive treatment by 2 years after NICU discharge. These data provide important insights into the outcome of neonatal HTN, but should be confirmed prospectively.

Mycophenolate Mofetil for Severe IgA Vasculitis Nephropathy in Children: An Observational Study.

Rationale & Objective: Individuals with IgA vasculitis nephritis (IGAVN) may develop rapidly progressive glomerulonephritis and/or nephrotic-range proteinuria, which are associated with worse prognosis. We report our experience of treatment of children with IGAVN with nephrotic-range proteinuria. Study Design: Case series. Setting & Participants: We retrospectively analyzed all children who presented with IGAVN, cutaneous purpura, and nephrotic-range proteinuria from January 1, 2000 until December 31, 2018. Outcome: We evaluated time required to achieve remission of proteinuria, resolution of hematuria, and glomerular filtration rate (GFR) at 12 months and last follow-up. Results: Twelve patients, 8 boys and 4 girls, mean age 7.5 years (range 4-15) were included in the study. Mean urinary protein to creatinine ratio (UPC) was 12.5 ± 8.7 mg/mg and GFR 90.7 ± 19.1 mL/min/1.73 m2 before initiation of immunosuppression. All patients were treated with steroids and mycophenolate mofetil. Mean UPC declined progressively from 12.5 mg/mg to 4.6, 2.7, 0.3, and 0.2 mg/mg after 1, 3, 6, and 12 months, respectively. All patients achieved remission of proteinuria (UPC <0.3 mg/mg) and normalization of kidney function (GFR 102.2 ± 8.0 mL/min/1.73 m2) at 12 months. Immunosuppression was successfully withdrawn in all patients, and at last follow-up (mean 33.5 months), all patients except one remained in remission. All patients except one that relapsed maintained normal GFR at the last follow-up. Limitations: Retrospective study, single-center experience, no standard immunosuppressive protocol, lack of control group. Conclusions: Remission can be achieved in patients with IGAVN and nephrotic-range proteinuria using mycophenolate mofetil-based immunosuppression. Magnitude of proteinuria is a key laboratory finding that correlates with time to achieve remission. Prolonged follow-up of patients with severe IGAVN is warranted.

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.

Can transition to adult care for transplant recipients be improved by intensified services while patients are still in pediatrics?

Context-Transferring out of pediatrics is a vulnerable time for transplant recipients. Use of a transition coordinator before and after transfer improves outcomes, although it is unclear whether placing a transition coordinator in pediatrics alone is beneficial. Objective-To determine if incorporating a transition coordinator in pediatrics only is associated with stable outcomes for kidney transplant recipients. Design-A retrospective chart review was conducted on outcomes for kidney transplant recipients who shifted service location between 2008 and 2012. Setting-A pediatric and adult transplant unit. Patients-Twenty-two patients transferred during the study period. Intervention-Twelve patients received more intensified preparation from the team's social worker, whose role was aligned with a transition coordinator position; 10 patients received standard care. Main Outcome Measures-The primary outcome was medication adherence, using a validated measure, standard deviations of tacrolimus blood levels. A standard deviation greater than 2.5 has been established as a threshold associated with poor outcomes such as rejection. Standard deviation of tacrolimus levels was compared for 1 year before and 1 year after transfer. Results-Medication adherence worsened from 1 year before (2.03 [SD, 0.75]) to 1 year after transfer (2.95 [SD, 1.38]; t = -;3.07, P = .007). A repeated-measures analysis of variance indicated that this pattern was the same for patients who did and patients who did not receive intensified services in pediatrics (F1,16 = 1.07, P = .32).

The Kidney in Pediatric Liver Disease.

There is an intricate relationship between the liver and the kidney, with renal physiology and function intimately involved in many primary disorders of pediatric liver disease. The hemodynamic changes of progressive cirrhosis affect and are directly affected by changes in renal blood flow and renal handling of sodium and free water excretion. Resulting complications of worsening ascites, hyponatremia, and acute kidney injury frequently complicate the care of children with advanced liver disease and contribute significant morbidity and mortality. While liver transplantation may restore hemodynamic stability, nearly 40% of pediatric liver transplant recipients develop chronic kidney disease post-transplant and approximately 25% are left with clinical hypertension. This review seeks to provide a basic understanding of this relationship to enable the provision of optimal care to children with liver disease.

Biliary Atresia Is Associated With Hypertension.

OBJECTIVES: The improved survival of pediatric liver transplant recipients is accompanied by an increase in long-term comorbidities. A recently highlighted concern, hypertension, is associated with chronic kidney disease (CKD) in this population and can result in other target-organ damage during childhood. The prevalence of hypertension in pediatric liver transplantation is imprecisely known. In addition, individual etiologies of liver failure may convey different risks of hypertension. We sought to study the effect of liver transplantation on the prevalence of hypertension and CKD in patients with biliary atresia (BA). METHODS: We conducted a retrospective chart review of 160 patients with BA followed at the Mount Sinai Medical Center, New York, from 1987 to 2012. Data were accumulated from the initial and subsequent visits at approximately 6 months, 1, 3, 5, 10, and 15 years of age. Hypertension was defined as systolic blood pressure >95th percentile for age, sex, height, and/or use of antihypertensive medication. Renal function was examined over time. Data were stratified by liver transplantation status at the time of visit. RESULTS: A high prevalence of hypertension was observed from the initial visit through age 10, independent of transplant status (transplanted: 48% initial visit and 13% after 10 years vs nontransplanted: 55% initial visit and 17% after 10 years [P = ns for transplant status]). Mean estimated glomerular filtration rate (eGFR) was lower among liver transplant patients as compared with nontransplant patients and declined posttransplant. The incidence of CKD was higher among transplant patients. CONCLUSIONS: Hypertension is common among children with BA, independent of liver transplant status. Transplant patients had significantly reduced renal function, which continued to decline over time. Hypertension was not associated with reduced eGFR.

The kidney in pediatric liver transplantation: an updated perspective.

CKD continues to detract from the success of improved survival in pediatric liver transplantation, and its presence is likely under recognized. Here we review the literature regarding the prevalence, etiology, and management of renal dysfunction in pediatric liver transplant recipients. Long-term studies suggest the prevalence of CKD to be 25-38% by 5-10 yr post-transplant. While important, sole use of serum creatinine overestimates renal function in this population. Screening for and treatment of persistent proteinuria and hypertension as well as minimization of nephrotoxic insults are the mainstays to delay or prevent CKD progression. Office-based blood pressure measures are less sensitive than ABPM, which is specifically recommended by the American Heart Association for its ability to diagnose masked hypertension in pediatric liver transplant recipients. Long-term risk of CKD is predominantly secondary to CNI toxicity. CNI minimization protocols have shown promise in slowing progression of CKD while maintaining graft function, but large-scale randomized control trials with long-term follow-up are needed.

Developing methods to compare low-education community-based and university-based survey teams.

We compared the quality of low-education community-based survey teams to college educated graduate students. Our approach was to develop methods, conduct a pilot survey, and report lessons. Community and university teams conducted surveys from non-overlapping random samples of addresses at a public housing development in Boston, Massachusetts. The two types of teams make a similar number of attempts (122 and 124, respectively), and there was no statistically significant difference between the teams in terms of response rate or amount of missing data. Similarly, there was no statistically significant difference in refusal rate or in responses to questions in the survey. There was, however, evidence that the community teams used data tracking forms improperly. This study suggests that it is possible to study the relative quality of community and university-based teams in terms of data collection. The findings also suggest that the two types of teams may be roughly comparable.