The Improving Renal Outcomes Collaborative: Blood Pressure Measurement in Transplant Recipients.

Background And Objectives: Hypertension is highly prevalent in pediatric kidney transplant recipients and contributes to cardiovascular death and graft loss. Improper blood pressure (BP) measurement limits the ability to control hypertension in this population. Here, we report multicenter efforts from the Improving Renal Outcomes Collaborative (IROC) to standardize and improve appropriate BP measurement in transplant patients.

Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities.

Hypertension affects one billion people and is a principal reversible risk factor for cardiovascular disease. Pseudohypoaldosteronism type II (PHAII), a rare Mendelian syndrome featuring hypertension, hyperkalaemia and metabolic acidosis, has revealed previously unrecognized physiology orchestrating the balance between renal salt reabsorption and K(+) and H(+) excretion. Here we used exome sequencing to identify mutations in kelch-like 3 (KLHL3) or cullin 3 (CUL3) in PHAII patients from 41 unrelated families.

A novel deletion in the RCA gene cluster causes atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a complex complement-mediated disease that progresses to end-stage renal failure (ESRF) in 50% of cases. Dysregulation of the alternative pathway (AP) of the complement cascade manifests as microangiopathic anaemia and thrombocytopenia. Multiple genes in the AP have been implicated in disease pathogenesis.

Renal pathology in the pediatric transplant patient.

Renal transplantation is a therapeutic goal for children with advanced chronic kidney disease. There are many causes of renal dysfunction in children with allografts--the transplanted kidney can develop a variety of morphologic alterations leading to dysfunction. Evaluation of the kidney biopsy is one of the best methods of determining the cause of graft dysfunction.

Early renal benefit of rapamycin combined with reduced calcineurin inhibitor dose in pediatric heart transplantation patients.

Background: Calcineurin inhibitors such as cyclosporine are effective in preventing rejection in recipients of solid organ transplants. Unfortunately, the prolonged use of calcineurin inhibitors may result in progressive renal injury.

Methods: We studied the renal function of 15 pediatric heart transplant recipients who were taking calcineurin inhibitors.

Systemic Hypertension: Management in Children and Adolescents.

Recognition of systemic hypertension in children and adolescents requires careful blood pressure measurement using proper technique to compare with appropriate normative data. Selected use of ambulatory blood pressure monitoring can identify children with "white coat" hypertension, thus avoiding unnecessary diagnostic testing and treatment in these children. Nonpharmacologic therapies including dietary sodium restriction, weight loss, and exercise may benefit children and adolescents with borderline hypertension and mild essential hypertension.