Clinical Experience of a Long-acting Pegylated Erythropoietin-Stimulating Agent in Pediatric Chronic Kidney Disease.

Objective: Management of anemia of chronic kidney disease (CKD) often includes subcutaneous or intravenous administration of erythropoietin-stimulating agents (ESAs). Mircera, a pegylated continuous erythropoietin receptor agonist, has a longer duration of action and requires less frequent administration than other ESAs. Pediatric experience with Mircera is limited.

Thromboprophylaxis in congenital nephrotic syndrome: 15-year experience from a national cohort.

Introduction: Congenital nephrotic syndrome (CNS) is an ultra-rare disease associated with a pro-thrombotic state and venous thromboembolisms (VTE). There is very limited evidence evaluating thromboprophylaxis in patients with CNS. This study aimed to determine the doses and duration of treatment required to achieve adequate thromboprophylaxis in patients with CNS.

Fludrocortisone-a treatment for tubulopathy post-paediatric renal transplantation: A national paediatric nephrology unit experience.

Calcineurin inhibitors post-renal transplantation are recognized to cause tubulopathies in the form of hyponatremia, hyperkalemia, and acidosis. Sodium supplementation may be required, increasing medication burden and potentially resulting in poor compliance. Fludrocortisone has been beneficial in addressing tubulopathies in adult studies, with limited paediatric data available.

Fifteen-minute consultation: the management of microscopic haematuria.

Haematuria can be a troublesome symptom with various different methods of presentation and aetiologies. Microscopic haematuria is a common coincidental finding often found when the patient has presented for another reason. We will discuss the subject of haematuria but will focus the majority of this article on the discussion of microscopic haematuria, including a definition, the important features to cover in the history and examination, aetiologies to suspect in children and infants, and a suggested approach to assessing these patients in secondary care.

Bilateral ureteric obstruction secondary to renal papillary necrosis.

A 2-year-old male presented to hospital with a 5-day history of vomiting and pyrexia. He was initially treated with non-steroidal medication as an anti-pyretic. Initial investigations demonstrated a raised urea and creatinine and he was treated with intravenous fluids.

Reversible chronic pulmonary fibrosis associated with MMF in a pediatric patient: a case report.

We describe a case of chronic mineralizing pulmonary elastosis in a seven-yr-old boy following DD renal transplantation for Wilms tumour. Fourteen months post-transplantation he developed respiratory symptoms with lung biopsy demonstrating chronic mineralizing pulmonary elastosis thought to be secondary to immunosuppression with MMF. Symptomatic resolution occurred following MMF cessation.

Body composition and nutritional intake in children with chronic kidney disease.

The aim of this study was to assess body composition in children with chronic renal failure (CRF) and post renal transplantation (Tx), and to compare it to body mass index (BMI) and nutritional intake. Dietary assessment using 3-day diaries, total and regional body composition assessment by dual x-ray energy absorptiometry of 50 CRF children (29M, 21F), median age 8.9 yrs and 50 Tx children (32M, 18F), median age 12.

Renal transplant biopsy specimen adequacy in a paediatric population.

Updated guidelines on the diagnosis of acute allograft rejection including criteria for biopsy specimen adequacy were published in 1999. We sought to determine the adequacy of specimens in paediatric transplant patients and identify factors influencing adequacy. All renal transplant biopsies performed between 1998 and 2003 were classified as adequate (n =25), minimal (n =19) or inadequate (n =27) in accordance with the Banff 97 criteria, and the histological diagnoses were documented.

Bone mineral content, corrected for height or bone area, measured by DXA is not reduced in children with chronic renal disease or in hypoparathyroidism.

The combination of poor growth and parathyroid and mineral disorders complicates the diagnosis of renal bone disease in children with chronic renal insufficiency (CRI), and the role of dual X-ray absorptiometry (DXA) is unclear. We aimed to examine the role of DXA in assessing variation in size-adjusted bone mineral content (BMC) in children with CRI and compare it with a cohort with hypoparathyroidism (HPT) and pseudo-hypoparathyroidism (PHPIa). In 29 patients with CRI (21 male) with a median age of 11 years (10th, 90th centiles 4.

Vascular access survival in children and young adults receiving long-term hemodialysis.

Background: The delivery of long-term hemodialysis therapy in children is complicated by smaller vascular caliber and the potential lifelong requirement for hemodialysis access. Various factors have resulted in the increased use of cuffed central venous catheters (CVLs) in preference to autologous arteriovenous fistulae (AVFs) and arteriovenous synthetic grafts (AVGs). The aim of this study is to compare CVL, AVF, and AVG survival and determine factors affecting their survival.

Skeletal disproportion in children with chronic renal disease.

Objectives: To assess stature and skeletal disproportion in children with chronic renal disease.

Methods: Cross-sectional study of height (HT), sitting height (SH), subischial leg length (SILL), sitting height/height ratio (SH:HT) and disproportion score (SH SDS minus SILL SDS) in 56 children (M:35) with median age 11.4 years (range 4.

Glomerular basement membrane thickness in children: a stereologic assessment.

Background: Stereologic methods have emerged as the technique of choice in assessing glomerular basement membrane (GBM) thickness, following conceptual modeling comparing the stereologic technique of harmonic mean of the orthogonal intercept estimation (Th) with the model based method of arithmetic mean estimation (ATH), with no direct comparison undertaken. We undertook to establish the gold standard for GBM estimation and use this technique to establish a range for GBM thickness in children.

Methods: Intra-observer and inter-glomerular variation was estimated in 34 cases with (presumed) normal GBM thickness, using Th, ATH and a rapid direct measurement technique, with intra-observer variation measured in 35 cases with GBM attenuation.

Hypercalcaemia in association with trisomy 21 (Down's syndrome).

The combination of hypercalcaemia, hypercalciuria, and nephrocalcinosis with and without renal impairment is rare in paediatric clinical practice. However, this constellation of findings has been reported in three children with trisomy 21, but the absence of detailed nutritional data has failed to clarify the underlying pathogenesis. This report describes a 4 year old girl with trisomy 21 who was found coincidentally to have hypercalcaemia, hypercalciuria, nephrocalcinosis, and renal impairment in the absence of metabolic alkalosis, following a prolonged period of excessive calcium intake.

Vesicoureteric reflux associated with renal dysplasia in the Wolf-Hirschhorn syndrome.

Wolf-Hirschhorn syndrome (WHS) is caused by a partial deletion of the short arm of chromosome 4 (4p16.3) and is characterized by severe pre- and postnatal growth retardation, developmental delay, and multiple congenital anomalies, including malformations of the urogenital system. We describe the renal and urinary tract phenotype in a series of six children with WHS.

Accuracy of clean-catch urine collection in infancy.

Objective: To compare the accuracy of cultures of urine obtained by clean-catch urine (CCU) collection and suprapubic aspiration (SPA) in infants.

Design: Prospective case series undertaken in a pediatric teaching hospital and associated neonatal unit. Fifty-eight paired urine cultures (CCU collection and SPA) were obtained from 49 infants with suspected urinary tract infection.

Efficacy of gastrostomy feeding in infants and older children receiving chronic peritoneal dialysis.

Objective: To assess the efficacy of supplemental gastrostomy tube (g-tube) feeding in infants and children receiving chronic peritoneal dialysis (CPD).

Design: Retrospective observational study.

Setting: Pediatric nephrology division of tertiary care center.

Complications of gastrostomy feeding in children receiving peritoneal dialysis.

Gastrostomy tube (g-tube) feeding is recognized to improve the nutritional delivery to children with end-stage renal disease. A retrospective study was undertaken assessing the complications of g-tube feeding in children receiving peritoneal dialysis (PD). Twenty-three patients, mean age 3.