Short, frequent, 5-days-per-week, in-center hemodialysis versus 3-days-per week treatment: a randomized crossover pilot trial through the Midwest Pediatric Nephrology Consortium.

Background: No controlled trials in children with end-stage kidney disease have assessed the benefits of more frequently administered hemodialysis (HD).

Methods: We conducted a multicenter, crossover pilot trial to determine if short, more frequent (5 days per week) in-center HD was feasible and associated with improvements in blood pressure compared with three conventional HD treatments per week. Because adult studies have not controlled for the weekly duration of dialysis, we fixed the total treatment time at 12 h a week of dialysis during two 3-month study periods; only frequency varied from 5 to 3 days per week between study periods.

Comparison of isotonic and hypotonic intravenous maintenance fluids: a randomized clinical trial.

Importance: Use of hypotonic intravenous fluids for maintenance requirements is associated with increased risk of hyponatremia that results in morbidity and mortality in children. Clinical trial data comparing isotonic and hypotonic maintenance fluids in nonsurgical hospitalized pediatric patients outside intensive care units are lacking.

Objective: To compare isotonic (sodium chloride, 0.

Successful treatment of DEAP-HUS with eculizumab.

Background: Deficiency of complement factor H-related (CFHR) proteins and CFH autoantibody-positive hemolytic uremic syndrome (DEAP-HUS) represents a unique subgroup of complement-mediated atypical HUS (aHUS). Autoantibodies to the C-terminus of CFH block CFH surface recognition and mimic mutations found in the genetic form of (CFH-mediated) aHUS. CFH autoantibodies are found in 10-15 % of aHUS patients and occur--so far unexplained--almost exclusively in the background of CFHR1 or CFHR3/CFHR1 deletions.

Bolus fluid therapy and sodium homeostasis in paediatric gastroenteritis.

Aim: The study aims to assess the risk of developing hyponatraemia when large-volume bolus fluid rehydration therapy is administered.

Methods: We conducted a prospective randomised study in a tertiary-care centre emergency department. Participants included children with gastroenteritis and dehydration requiring intravenous rehydration.

Intensive hemodialysis associates with improved survival compared with conventional hemodialysis.

Patients undergoing conventional maintenance hemodialysis typically receive three sessions per week, each lasting 2.5-5.5 hours.

Attitudes of caregivers to management of end-stage renal disease in infants.

Objectives: To characterize the attitudes of pediatric nephrologists caring for infants with end-stage renal disease (ESRD) compared with attitudes from a survey published in 1998. Nephrology nurses and social workers were included.

Methods: An e-mail survey was distributed to pediatric nephrology teams in Canada, Germany, Japan, the United Kingdom, and the United States.

Body size in children with chronic kidney disease after gastrostomy tube feeding.

Outcome body size of gastrostomy tube (g-tube)-fed children with chronic kidney disease (CKD) was investigated. CKD patients, stages 2-5, who had a g-tube inserted and removed between 1985 and 2007 were retrospectively reviewed (n=20) for anthropometrics, lab values, and steroid use from insertion to latest date. CKD patients never having had a g-tube placed (n=82) acted as the comparison population with similar data collection at start and end of the latest 5-year period.

Interprovincial differences in the achievement of K/DOQI targets of mineral metabolism in Canada.

Background: Abnormalities in mineral metabolism in chronic kidney disease are associated with increased morbidity and mortality. The Kidney Disease Outcomes Quality Initiative (K/DOQI) clinical practice guidelines were established in 2003 to address issues in the management of mineral and bone metabolism. The goal of this study was to compare (i) mineral metabolism control among Canadian haemodialysis (HD) patients with K/DOQI-defined targets and Dialysis Outcomes and Practice Patterns Study II (DOPPS II) data and (ii) the effect of different treatment strategies.

Interventions for bone disease in children with chronic kidney disease.

Background: Bone disease is common in children with chronic kidney disease (CKD) and when untreated may result in bone deformities, bone pain, fractures and reduced growth rates.

Objectives: To investigate the benefits and harms of interventions for preventing and treating bone disease in children with CKD.

Search Strategy: The Cochrane Renal Group's specialised register, the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, reference lists and abstracts were searched without language restriction.

Platelet-associated complement factor H in healthy persons and patients with atypical HUS.

Atypical hemolytic uremic syndrome (aHUS) is associated with complement system dysregulation, and more than 25% of pediatric aHUS cases are linked to mutations in complement factor H (CFH) or CFH autoantibodies. The observation of thrombocytopenia and platelet-rich thrombi in the glomerular microvasculature indicates that platelets are intimately involved in aHUS pathogenesis. It has been reported that a releasable pool of platelet CFH originates from alpha-granules.

A novel disease-causing mutation in AVPR2: Q96H.

A 4-month-old male infant was diagnosed with nephrogenic diabetes insipidus (NDI). Genetic testing of the arginine vasopressin receptor-2 (AVPR2) yielded a novel X-linked mutation, termed Q96H, in both the propositus and his mother; there was no family history. Protein sequence comparison between AVPR subtypes shows that Q96 is part of a highly conserved motif.

Evaluating methods for improving ultrafiltration in pediatric hemodialysis.

Adequate ultrafiltration (UF) is necessary for good health, but it can be hindered by the development of intradialytic symptoms and hypotension. To determine whether sodium ramping, UF profiles and mannitol could improve UF in children, we instituted a standardized prescription for chronic hemodialysis in our unit. We prospectively analyzed 506 treatments from ten patients.

Central venous lines for chronic hemodialysis: survey of the Midwest Pediatric Nephrology Consortium.

Central venous lines (CVL) continue to be the most commonly used vascular access device for children on hemodialysis (HD). Despite their frequent use, little is known regarding the frequency of CVL-related intradialytic complications that could interfere with delivery of effective dialysis. To better assess this, we conducted a cross-sectional study of ten HD centers within the Midwest Pediatric Nephrology Consortium.

Anemia in children with chronic kidney disease.

Anemia is a common feature of chronic kidney disease, but the management of anemia in children is complex. Erythropoietin and supplemental iron are used to maintain hemoglobin levels. The National Kidney Foundation-Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) clinical practice guidelines for the management of anemia specifically in children were recently published.

Short-term effects of nocturnal haemodialysis on carnitine metabolism.

Background: Functional carnitine deficiency [as indicated by an abnormal acyl-carnitine/free-carnitine (AC:FC) ratio] is commonly seen in patients with end-stage renal disease (ESRD), resulting in significant clinical detriments including anaemia, cardiomyopathy and muscle weakness. Nocturnal haemodialysis (NHD) (5-6 sessions per week, 8 h per treatment) has been reported to reverse several surrogate markers of uraemia. Conversely, as a consequence of increased dialysis dose, NHD may have the potential to aggravate plasma nutrient deficiencies.

Quality of life in children with chronic kidney disease-patient and caregiver assessments.

Background: Children with chronic kidney disease (CKD) require strict dietary and lifestyle modifications, however, there is little information on their quality of life. Our objective was to compare health-related quality of life (HRQOL) in children with different stages of CKD to each other and to a control population.

Methods: A cross-sectional assessment of HRQOL for physical, emotional, social and school domains was performed using the PedsQL Generic Core Scale.

Calcium and phosphate balance in adolescents on home nocturnal haemodialysis.

Studies in adults show superior serum phosphate and parathyroid hormone (PTH) control on slow nocturnal haemodialysis (NHD) compared with conventional haemodialysis. We studied the progress of four children aged 12, 13, 14 and 16 years after they had been initiated on NHD. The follow-up period ranged from 6 months to 20 months.

Outpatient pediatric renal transplant biopsy--is it safe?

The safe observation time following pediatric renal transplant biopsy is unknown. To predict the safety of day-care pediatric renal transplant biopsy, we retrospectively evaluated the timing, incidence and severity of post-biopsy complications in children observed overnight. Biopsies were performed under real time ultrasound guidance using an 18-gauge Bard Biopty needle.

The use of darbepoetin in infants with chronic renal impairment.

Darbepoetin is a newer analogue of epoetin, with a longer half-life, that allows less frequent administration. There are currently no published data available for its use in infants. We report our experience with this drug in infants with chronic renal impairment, weighing less than 8 kg.

Home nocturnal hemodialysis in children.

Objective: To describe the effect of home nocturnal hemodialysis (NHD) in North American children.

Study Design: Four teenagers underwent NHD for 8 hours, 6 to 7 nights/week, using either central venous lines or fistulae for periods of 6 to 12 months. Blood flow approximated 200 mL/min, and dialysate flow was 300 mL/min; the dialysate contained potassium and phosphate.

Darbepoetin alfa (Aranesp) in children with chronic renal failure.

Background: Darbepoetin alfa use has been reported in 7 children with chronic renal failure (CRF). Our objective was to evaluate the efficacy and safety of darbepoetin and determine a therapeutic dose in a larger sample of children with CRF.

Methods: Twenty-six children with chronic renal insufficiency (CRI) GFR <30 mL/min/1.

Development of a nocturnal home hemodialysis (NHHD) program for children.

Whereas the success of nocturnal home hemodialysis (NHHD) as a treatment modality for adults has been clearly demonstrated, there is very little experience with NHHD in children. There are genuine concerns related to patient safety, as well as a potentially overwhelming burden on the parents of children, while supervising NHHD. Each of these issues has been considered in the development of the NHHD program for children in Toronto.

Outcome of isolated antenatal hydronephrosis.

Objective: To define the clinical outcome in isolated antenatal hydronephrosis (ANH), defined as pelviectasis without vesicoureteral reflux or urinary tract obstruction.

Study Design And Patients: We analyzed prospectively gathered data from patients with isolated ANH. Pelviectasis, graded using the anterior-posterior diameter reference criteria, was defined by the status of the more severely affected kidney.

Vesicoureteral reflux in infants with isolated antenatal hydronephrosis.

Standardized evaluation of all newborns with antenatally recognized hydronephrosis (ANH) at The Hospital for Sick Children (HSC) has included voiding cystourethrography (VCUG). This paper reviews this protocol to determine: (1) the prevalence of vesicoureteral reflux (VUR) in isolated ANH and (2) the value of performing VCUG in cases of mild hydronephrosis, defined as renal pelvis dilatation <10 mm on postnatal ultrasonography (US). A retrospective chart review was performed on infants referred with ANH.