Obstructive Bilateral Renal Fungal Bezoars in an Extremely Premature Neonate Treated With Antifungals and Urokinase Irrigation: A Case Report and Review of the Literature.

Background: An ex-27-week gestation female infant developed bilateral forearm nodules at 4 weeks of life during treatment for methicillin-sensitive Staphylococcus aureus bacteremia. A pure growth of Candida albicans was isolated on culture of both sterile aspiration of the forearm abscess and urine without evidence of methicillin-sensitive Staphylococcus aureus . The patient went on to develop bilateral obstructive renal fungal bezoars at 11 weeks of life.

Enrolment of the first paediatric cohort into the Australian lupus registry and biobank: A single-centre experience.

Introduction: We aim to report on the feasibility of establishment of the first paediatric cohort as part of the longitudinal database of the Australian Lupus Registry and Biobank (ALRB) and to describe the enrolment data with a focus on clinical characteristics, serological data, treatment strategies and patient/parent-reported outcome measures.

Methods: All patients under the age of 18 years with a diagnosis of systemic lupus erythematosus (SLE) attending the paediatric rheumatology service of a single, tertiary hospital were identified. Patients were enrolled in the ALRB if they met ≥4/11 of the American College of Rheumatology (ACR) 1997 SLE classification criteria or the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria.

Hospital admissions associated with dehydration in childhood kidney transplantation.

Background: Paediatric kidney transplant recipients may be at a particular risk of dehydration due to poor kidney concentrating capacity and illness associated with poor fluid intake or losses. In this population, creatinine rise may be more likely with relatively mild dehydration, which may trigger hospital admission. This study describes hospital admissions in the first 12 months after transplantation with diagnosis of graft dysfunction associated with dehydration due to illness or poor fluid intake.

Echocardiogram screening in pediatric dialysis and transplantation.

Transthoracic echocardiography is commonly used to identify structural and functional cardiac abnormalities that can be prevalent in childhood chronic kidney failure (KF). Left ventricular mass (LVM) increase is most frequently reported and may persist post-kidney transplant especially with hypertension and obesity. While systolic dysfunction is infrequently seen in childhood chronic KF, systolic strain identified by speckle tracking echocardiography has been frequently identified in dialysis and it can also persist post-transplant.

Clinical impact of genomic testing in patients with suspected monogenic kidney disease.

Purpose: To determine the diagnostic yield and clinical impact of exome sequencing (ES) in patients with suspected monogenic kidney disease.

Methods: We performed clinically accredited singleton ES in a prospectively ascertained cohort of 204 patients assessed in multidisciplinary renal genetics clinics at four tertiary hospitals in Melbourne, Australia.

Results: ES identified a molecular diagnosis in 80 (39%) patients, encompassing 35 distinct genetic disorders.

Metabolite diagnosis of primary hyperoxaluria type 3.

Background: Primary hyperoxaluria type 3 (PH3) is a recently described cause of childhood renal calculi. It results from mutations in the HOGA1 gene and most cases have been diagnosed after clinical ascertainment, exclusion of other genetic hyperoxalurias and mutation testing. Metabolite testing has not been widely applied but holds promise for the rapid screening and diagnosis of patients who are not specifically suspected to have PH3.

The Use of Peritoneal Dialysis in Phenobarbitone Toxicity in a Critically Unwell Neonate.

Background: Phenobarbitone (PB) is the first-line anti-convulsant for neonatal seizures. The use of peritoneal dialysis (PD) to enhance drug elimination in cases of neonatal PB overdose has not been reported.

Objective: To report a case of neonatal severe PB toxicity and review the elimination of PB by PD.

Australian deceased donor kidney allocation protocols: Transplant waiting and graft quality for children and adolescents.

DD kidney allocation protocols may influence timing of transplantation and graft quality for pediatric recipients. This study aimed to evaluate the effects of these protocols, including pediatric priority, on waiting time on dialysis, transplant type, donor age, and HLA matching according to state of transplant in Australia. De-identified information on patients <15 yr of age who commenced RRT in NSW, Qld, and Victoria from 2002 to 2011 was retrieved from the ANZDATA.

Copy-number variation associated with congenital anomalies of the kidney and urinary tract.

Background: The most common cause of end-stage renal disease in children can be attributed to congenital anomalies of the kidney and urinary tract (CAKUT). Despite this high incidence of disease, the genetic mutations responsible for the majority of CAKUT cases remain unknown.

Methods: To identify novel genomic regions associated with CAKUT, we screened 178 children presenting with the entire spectrum of structural anomalies associated with CAKUT for submicroscopic chromosomal imbalances (deletions or duplications) using single-nucleotide polymorphism (SNP) microarrays.

Paediatric urological investigations--dose comparison between urology-related and CT irradiation.

Background: Urological investigation in children frequently involves high radiation doses; however, the issue of radiation for these investigations receives little attention compared with CT.

Objective: To compare the radiation dose from paediatric urological investigations with CT, which is commonly regarded as the more major source of radiation exposure.

Materials And Methods: We conducted a retrospective audit in a tertiary paediatric centre of the number and radiation dose of CT scans, micturating cystourethrography exams and urological nuclear medicine scans from 2006 to 2011.

Cystinosis and lupus erythematosus: coincidence or causation.

A 14-year-old boy with known stable cystinosis, treated with cysteamine since infancy, presented with a deterioration of renal function with haematuria in conjunction with a nodular rash, arthralgia, leucopenia, hypocomplementaemia and raised antinuclear antibodies. He was diagnosed with spontaneous onset of systemic lupus erythematosus (SLE), and his renal biopsy was consistent with lupus nephritis. It is unusual for patients with one severe disease to develop another disease process completely unrelated to their original condition, but it can occur.