Effect of the dialysis fluid buffer on peritoneal membrane function in children.

Background And Objectives: Double-chamber peritoneal dialysis fluids exert less toxicity by their neutral pH and reduced glucose degradation product content. The role of the buffer compound (lactate and bicarbonate) has not been defined in humans.

Design, Setting, Participants, & Measurements: A multicenter randomized controlled trial in 37 children on automated peritoneal dialysis was performed.

Regional citrate anticoagulation is safe in intermittent high-flux haemodialysis treatment of children and adolescents with an increased risk of bleeding.

Background: Regional citrate anticoagulation (RCA) is strongly recommended for adults with an increased risk of bleeding complications. The objective of this retrospective analysis was to evaluate an RCA protocol concerning feasibility and safety in intermittent high-flux haemodialysis (iHD) treatment in children and adolescents.

Methods: Eighteen children and adolescents aged 5-17 years (median 15 years) underwent 74 iHD treatment sessions with RCA.

Relapsing peritonitis in children who undergo chronic peritoneal dialysis: a prospective study of the international pediatric peritonitis registry.

Background And Objectives: The International Pediatric Peritonitis Registry (IPPR) was established to collect prospective data regarding peritoneal dialysis (PD)-associated peritonitis in children. In this report, we present the IPPR results that pertain to relapsing peritonitis (RP).

Design, Setting, Participants, & Measurements: This was an online, prospective entry into the IPPR of data that pertain to peritonitis cases by participating centers.

Continuous venovenous haemodialysis (CVVHD) and continuous peritoneal dialysis (CPD) in the acute management of 21 children with inborn errors of metabolism.

Background: Newborns with inborn errors of metabolism often present with hyperammonaemic coma, requiring prompt diagnosis and specific medical therapy, nutritional support and efficient toxin removal. Little information regarding the efficacy and safety of continuous venovenous haemodialysis (CVVHD) as an option for extracorporal ammonia detoxification in children is available.

Methods: Twenty-one patients with hyperammonaemia [19 neonates (mean age 4.

Reduced systemic advanced glycation end products in children receiving peritoneal dialysis with low glucose degradation product content.

Background: Glucose degradation products (GDP) in peritoneal dialysis (PD) solutions are toxic to the peritoneal membrane and promote the formation of advanced glycation end products (AGE), which contribute to accelerated atherosclerosis and amyloidosis. Double chamber PD solutions have a markedly reduced GDP content.

Methods: We analysed GDP and AGE kinetics in 21 children (7 months to 18 years) on automated PD in a prospective multicentre trial with randomized administration of single chamber, high-GDP and double-chamber, low-GDP dialysis solution for 12 weeks each.

Clearance and removal of oxalate in children on intensified dialysis for primary hyperoxaluria type 1.

Patients with end-stage renal failure owing to primary hyperoxaluria type 1 (PH1) receive dialysis while waiting for transplantation. So far, dialysis has not been shown to overcome the problem of ongoing oxalate production and deposition at extrarenal sites. We report on six children with PH1 who had to be dialyzed for a median period of 2.

Antiviral treatment of chronic hepatitis B with lamivudine in pediatric renal transplantation.

Renal transplantation in patients with chronic hepatitis B virus (HBV) infection is known to be associated with an increased risk for exacerbation of liver dysfunction. Lamivudine has been proven to be a potent inhibitor of hepatitis B virus replication in adults after kidney transplantation. Little is known about its efficacy and safety in pediatric renal transplant recipients.

A randomized crossover trial comparing sevelamer with calcium acetate in children with CKD.

Background: A multicenter, randomized, open-label, crossover study was performed to compare the efficacy and safety of sevelamer, a calcium-free phosphate binder, with calcium acetate in pediatric patients with chronic kidney disease (CKD).

Methods: Children (age, 0.9 to 18 years) with CKD undergoing hemodialysis or peritoneal dialysis or with a glomerular filtration rate of 20 or greater and less than 60 mL/min/1.

Removal of metabolites, cytokines and hepatic growth factors by extracorporeal liver support in children.

Background: Molecular Adsorbents Recirculating System (MARS)-mini has recently been approved and applied in children with hepatic failure. However, its indication, efficacy and capability to induce liver regeneration remain unclear. The aim of our pilot study in children was to analyse the impact of MARS on markers of detoxification and regeneration.

BIOKID: randomized controlled trial comparing bicarbonate and lactate buffer in biocompatible peritoneal dialysis solutions in children [ISRCTN81137991].

Background: Peritoneal dialysis (PD) is the preferred dialysis modality in children. Its major drawback is the limited technique survival due to infections and progressive ultrafiltration failure. Conventional PD solutions exert marked acute and chronic toxicity to local tissues.

Improved acidosis correction and recovery of mesothelial cell mass with neutral-pH bicarbonate dialysis solution among children undergoing automated peritoneal dialysis.

Acid-base balance and peritoneal membrane longevity are of utmost relevance for pediatric patients undergoing peritoneal dialysis (PD). PD fluids with neutral pH and reduced glucose degradation product contents are considered more biocompatible, because they preserve peritoneal cell functions in vitro. To investigate the clinical effects of a novel PD fluid buffered with 34 mM pure bicarbonate at neutral pH, a randomized, prospective, crossover comparison with conventional, acidic, 35 mM lactate PD fluid was performed for two consecutive 12-wk periods with 28 children (age, 6 mo to 15 yr) undergoing automated PD (APD).

Novel paracellin-1 mutations in 25 families with familial hypomagnesemia with hypercalciuria and nephrocalcinosis.

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is an autosomal recessive tubular disorder that is frequently associated with progressive renal failure. The primary defect is related to impaired tubular reabsorption of magnesium and calcium in the thick ascending limb of Henle's loop. Mutations in PCLN-1, which encodes the renal tight junction protein paracellin-1 (claudin-16), were identified as the underlying genetic defects.

[Electron beam computed tomography of the heart before kidney transplantation. Severe coronary disease in a 21-year old woman with nephrocalcinosis due to infantile hypercalcemia syndrome].

Background: Cardiovascular diseases are very common in patients with end-stage renal disease and are the underlying cause of approximately half the deaths in dialysis patients. In those patients vascular calcifications are typically seen in the tunica media and therefore represent histopathological changes different to those in atherosclerosis. For the evaluation of cardiovascular risk in chronic dialysis patients, a method is needed to reliably identify patients who have to undergo invasive diagnostics.

Platelet adenylyl cyclase signaling remains unaltered in children undergoing hemodialysis treatment.

Patients with chronic renal failure exhibit multiple endocrine, gastrointestinal and cardiovascular abnormalities, many of which may be explained by alterations of adenylyl cyclase (AC) responsiveness and/or G-protein expression. Since such alterations were previously reported, e.g.

[Home blood pressure self-measurement in children and adolescents with renal replacement therapy].

Background: Most patients with terminal renal failure show arterial hypertension. In addition to casual blood pressure measurements in the clinic, home blood pressure measurement is recommended for these patients to control arterial blood pressure.

Patients: The study was performed in children with hemodialysis (HD; n = 11), peritoneal dialysis (PD; n = 14) or after renal transplantation (NTX; n = 21) from one department of Pediatric Nephrology.

Mutations in the chloride channel gene, CLCNKB, leading to a mixed Bartter-Gitelman phenotype.

Gitelman syndrome is an inherited renal disorder characterized by impaired NaCl reabsorption in the distal convoluted tubule and secondary hypokalemic alkalosis. In clinical practice, it is distinguished from other hypokalemic tubulopathies by the presence of both hypomagnesemia and normocalcemic hypocalciuria. To date, only mutations in a single gene encoding the thiazide-sensitive NaCl cotransporter have been found as the molecular basis of GS.

Familial hypomagnesaemia with hypercalciuria and nephrocalcinosis maps to chromosome 3q27 and is associated with mutations in the PCLN-1 gene.

Familial hypomagnesaemia with hypercalciuria and nephrocalcinosis (FHHNC, MIM 248250) is a complex renal tubular disorder characterised by hypomagnesaemia, hypercalciuria, advanced nephrocalcinosis, hyposthenuria and progressive renal failure. The mode of inheritance is autosomal recessive. A primary defect in the reabsorption of magnesium in the medullary thick ascending limb of the loop of Henle (mTAL) has been proposed to be essential in FHHNC pathophysiology.

Middle molecules in peritoneal equilibration test as a marker of peritoneal stress in children on continuous peritoneal dialysis.

At 1 month, 3 months, 6 months, and more than 6 months after healed peritonitis, we evaluated repeated peritoneal equilibration tests (PETs) for small molecules such as urea, and middle molecules such as cystatin C, beta 2-microglobulin, and alpha 1-microglobulin. We analyzed a total of 104 PETs in 21 children aged 1.7-18.

Spectrum of early onset nephrotic syndrome associated with WT1 missense mutations.

We investigated 17 children with nephrotic syndrome (NS) of early onset (14 aged < 1 year) and rapid progression to end-stage renal disease for the presence of mutations in the Wilms' tumor suppressor gene WT1 on chromosome 11. In eight children (7 genotypic males) an association with Wilms' tumor and/or ambiguous genitalia (Denys-Drash syndrome) was observed. In these eight and two additional female patients with NS only constitutional missense mutations in the WT1 gene were detected; four children presented the so-called hot spot mutation in exon 9 (R394N) and six had different mutations in exons 8 and 9 (4 not previously described).

Value of diuresis renography in the post-natal period of assumed physiological renal immaturity.

The aim of this study was to determine if it is possible to exclude renal obstruction using diuresis renography in the first 6 weeks of life (the period of physiological renal immaturity), thus avoiding unnecessary invasive procedures, such as the Whitaker test or surgery. Diuresis renography with 123I-hippuran was performed in 27 patients aged less than 6 weeks and in 50 older children who acted as a reference group (age 6 weeks to 1 year, n = 28; age 1-10 years, n = 22). All 27 patients had significant dilatation of the pelvicalyceal system on ultrasonography.

Variability of peritoneal equilibration test in children on continuous peritoneal dialysis by repeated testing with solutions of different osmolarity.

To evaluate (1) differences in the peritoneal equilibration test (PET) achieved using continuous peritoneal dialysis (CPD) solutions containing different amounts of glucose and (2) intraindividual reproducibility of PETs performed twice within an interval of 8 months on CPD, we investigated 39 PETs in 13 children aged 2.4-19.0 years (median 10.

Calcium acetate versus calcium carbonate as oral phosphate binder in pediatric and adolescent hemodialysis patients.

Calcium carbonate is widely used as an oral phosphorus binder to control hyperphosphatemia in children on maintenance hemodialysis. Intestinal calcium absorption may induce hypercalcemia, particularly if calcitriol is given simultaneously. In adults, calcium acetate binds phosphorus more effectively than calcium carbonate, while reducing the frequency of hypercalcemic events.

24-hour blood pressure monitoring in children and adolescents after recovery from hemolytic uremic syndrome.

24-hour blood pressure monitoring is a valuable method for the diagnosis of arterial hypertension as well as for assessment of the diurnal rhythm of the arterial blood pressure (BP). The nocturnal decrease of blood pressure ("dipping") may be attenuated or abolished in children with advanced renal failure and glomerular diseases. Arterial hypertension is a longlasting problem in children who had recovered from hemolytic uremic syndrome (HUS).

Blood pressure monitoring at the wrist: is it reliable in children and adolescents?

We studied in children and adolescents the performance of two devices for blood pressure measurements at the wrist: "Blood pressure watch" (BPW) and Omron R1 (OR1). They were tested against auscultatory blood pressure readings at the upper arm. Wrist circumference correlated to the aged was greater than 13.