Urinary apolipoprotein AI in children with kidney disease.

Background: Although high-density lipoprotein (HDL) modulates many cell types in the cardiovascular system, little is known about HDL in the kidney. We assessed urinary excretion of apolipoprotein AI (apoAI), the main protein in HDL.

Methods: We enrolled 228 children with various kidney disorders and 40 controls.

Mycophenolate mofetil-related leukopenia in children and young adults following kidney transplantation: Influence of genes and drugs.

MMF is commonly prescribed following kidney transplantation, yet its use is complicated by leukopenia. Understanding the genetics mediating this risk will help clinicians administer MMF safely. We evaluated 284 patients under 21 years of age for incidence and time course of MMF-related leukopenia and performed a candidate gene association study comparing the frequency of 26 SNPs between cases with MMF-related leukopenia and controls.

Immunogenicity of Augmented Compared With Standard Dose Hepatitis B Vaccine in Pediatric Patients on Dialysis: a Midwest Pediatric Nephrology Consortium Study.

Background And Objectives: Patients on maintenance dialysis have a higher risk of unresponsiveness to hepatitis B vaccination and loss of hepatitis B immunity. Adult guidelines recommend augmented dosing (40 mcg/dose), resulting in improved response in adults. We sought to determine whether children on dialysis mount a similar antibody response when given standard or augmented dosing of hepatitis B vaccine.

Hypertension in chronic kidney disease: role of ambulatory blood pressure monitoring.

Children with chronic kidney disease have a markedly increased risk of cardiovascular morbidity and children with end stage renal disease have an estimated 30 times greater risk of cardiovascular mortality than the general pediatric population. In adults, the link between hypertension and cardiovascular disease is well-documented but that association has not been so readily apparent in children with chronic kidney disease. This may be in part because the early changes in blood pressure that occur in these patients do not necessarily manifest with changes in casual blood pressure measurements.

Acute poststreptococcal glomerulonephritis: the most common acute glomerulonephritis.

On the basis of strong research evidence, the prevalence of poststreptococcal glomerulonephritis (PSGN) is decreasing worldwide, although it still remains the leading cause of glomerulonephritis in children. The overall decrease in prevalence of PSGN has been mainly driven by a significant decrease in pyoderma seen in the last half-century, such that postpharyngitic PSGN is most commonly seen in developed nations. On the basis of primarily consensus because of a lack of relevant clinical studies, the latency period between streptococcal infection and the development of nephritis is a hallmark of PSGN, with this period lasting 1 to 2 weeks with pharyngeal infections or 2 to 6 weeks with skin infections.

Hypertension and CKD.

Hypertension is found in more than 50% of pediatric patients with CKD. However, its prevalence varies according to the cause of CKD. It is relatively infrequent in children with structural disorders.

Forced expression of laminin beta1 in podocytes prevents nephrotic syndrome in mice lacking laminin beta2, a model for Pierson syndrome.

Pierson syndrome is a congenital nephrotic syndrome with ocular and neurological defects caused by mutations in LAMB2, the gene encoding the basement membrane protein laminin β2 (Lamβ2). It is the kidney glomerular basement membrane (GBM) that is defective in Pierson syndrome, as Lamβ2 is a component of laminin-521 (LM-521; α5β2γ1), the major laminin in the mature GBM. In both Pierson syndrome and the Lamb2(-/-) mouse model for this disease, laminin β1 (Lamβ1), a structurally similar homolog of Lamβ2, is marginally increased in the GBM, but it fails to fully compensate for the loss of Lamβ2, leading to the filtration barrier defects and nephrotic syndrome.

Serum cystatin C is an early predictive biomarker of acute kidney injury after pediatric cardiopulmonary bypass.

Background And Objectives: Acute kidney injury (AKI) is a frequent complication of cardiopulmonary bypass (CPB). Serum creatinine (SCr), the current standard, is an inadequate marker for AKI since a delay occurs before SCr rises. Biomarkers that are sensitive and rapidly measurable could allow early intervention and improve patient outcomes.

Beneficial effect of cinacalcet in a child with familial hypocalciuric hypercalcemia.

We describe a child with familial hypocalciuric hypercalcemia (FHH) in whom the hypercalcemia seemed to interfere with tissue healing after tympanoplasty. Consequently, he was placed on cinacalcet (30 mg/day), changed after 2 weeks to 60 mg/day. The treatment resulted in a decrease in serum parathyroid hormone (PTH) from 148 to 32 pg/mL (normal 7-75) and ionized calcium from 1.

Ophthalmological aspects of Pierson syndrome.

Purpose: To study the ocular phenotype of Pierson syndrome and to increase awareness among ophthalmologists of the diagnostic features of this condition.

Design: Retrospective, observational case series.

Methods: A multicenter study of 17 patients with molecularly confirmed Pierson syndrome.

Neurodevelopmental deficits in Pierson (microcoria-congenital nephrosis) syndrome.

Pierson syndrome is an autosomal recessive disorder comprising congenital nephrotic syndrome with diffuse mesangial sclerosis and distinct eye abnormalities with microcoria reported as the most prominent clinical feature. LAMB2 mutations leading to lack of laminin beta2 were identified as the molecular cause underlying Pierson syndrome. Although LAMB2 is known to be expressed in the neuromuscular system, and defects of the neuromuscular junctions had been found in laminin beta2-deficient mice, no consistent neurological phenotype has been described clinically in murine or human laminin beta2-deficiency before.

Blood pressure control in pediatric hemodialysis: the Midwest Pediatric Nephrology Consortium Study.

Hypertension is frequent in pediatric patients receiving dialysis, with an especially high rate reported in children on hemodialysis (HD). We performed the present study to assess blood pressure (BP) status and identify risk factors for poor BP control in children on maintenance HD. One month's dialysis records were collected from 71 subjects receiving HD in ten dialysis units participating in the Midwest Pediatric Nephrology Consortium (MWPNC).