Sean E. Kennedy

Survival and clinical outcomes of children starting renal replacement therapy in the neonatal period

End-stage renal disease requiring renal replacement therapy (RRT) during the neonatal period is a very rare condition, and little information is available regarding long-term RRT and outcomes. To gain more information, we performed a collaborative study on patient characteristics and treatment outcomes in children who started RRT as neonates during their first month of life between 2000 and 2011 who were prospectively registered in the ESPN/ERA-EDTA, the IPPN (since 2007), the Japanese registry, or the Australian and New Zealand Dialysis and Transplant (ANZDATA) registry. During the first month of life, 264 patients from 32 countries started RRT and were followed for a median of 29 months (interquartile range 11-60 months). Most neonates (242) started on peritoneal dialysis, 21 started on hemodialysis, and 1 patient with a transplant. The most important causes of renal failure were congenital anomalies of the kidney and urinary tract in 141, cystic kidneys in 35, and cortical necrosis in 30. Within 2 years after the start of RRT, 69 children changed dialysis modality and 53 received a renal transplant. After a median of 7 months, 45 children had died, mainly because of infection, resulting in an estimated 2-year survival of 81%, and 5-year survival of 76%. Growth retardation (63%), anemia (55%), and hypertension (57%) were still major problems after 2 years. Thus, relatively good medium-term patient survival may be achieved with RRT started during the neonatal period, but specific therapeutic challenges continue to exist in this age group.

A systematic review of the psychometric properties of transition readiness assessment tools in adolescents with chronic disease

BackgroundHealth care transition of adolescents with chronic conditions may be unsuccessful when patients have not acquired the necessary skills and developmental milestones. It is therefore critical for health care providers to assess the readiness for transition of their adolescent patients. This is currently hindered by the lack of a recognised, well-established transition-readiness assessment tool.MethodsWe conducted a systematic review of all transition-readiness tools for adolescents with chronic medical conditions published in peer-reviewed journals. Tools were rated by the methodological quality of the validation studies, and the psychometric measurement qualities of each tool.ResultsTen different assessment tools were identified. Seven targeted specific diseases and 3 tools were generic. Most tools were poorly validated with only one tool, the Transition Readiness Assessment Questionnaire (TRAQ) demonstrating adequate content validity, construct validity, and internal consistency.ConclusionThe TRAQ was the best-validated transition-readiness tool, with additional benefits of disease-neutrality. Further research should focus on testing the predictive validity of this tool, and exploring correlation with transition-outcomes, in an international population.

Waiting time and outcome of kidney transplantation in adolescents.

Background. The major cause of late graft failure in adolescent kidney transplant recipients is thought to be nonadherence with medications. Delaying transplantation in adolescents may lead to improved adherence but at the cost of longer time on dialysis. To determine if waiting time on dialysis is a risk factor for graft survival in adolescents, we compared the outcomes of kidney transplants according to age and time on dialysis. Methods. We analyzed data from the Australian and New Zealand Dialysis and Transplant Registry on 2,739 primary kidney transplants performed between 1980 and 2004 in recipients less than 30 years old. Outcomes according to age at transplantation and waiting time were analyzed by Kaplan-Meier curves, log-rank tests, and Cox proportional hazard tests. Results. Overall five- and 10-year graft survival rates were significantly worse in adolescents (65% and 50%, respectively) compared to recipients aged two to 10 years (74% and 58%) and 20 to 29 years (72% and 57%). Waiting time on dialysis was an independent risk factor for failure of living donor grafts in adolescents (hazard ratio 0.53, P=0.03). Five- and 10-year graft survival of preemptive grafts in adolescents were 82% and 70%, respectively, which were similar to survival rates of preemptive grafts in other age groups. Conclusions. Reduced graft survival rates in adolescent recipients are not seen after preemptive transplants. Preemptive grafts are associated with a 50% reduction in the risk of graft failure. Delaying transplantation in adolescents may expose them to increased risk of poorer outcomes.

HBV associated nephrotic syndrome: resolution with oral lamivudine

A 6 year old boy presenting with a five month history of fever, lethargy, and anorexia, was found to have hepatitis B associated membranous glomerulonephropathy and nephrotic syndrome. After two months treatment with oral lamivudine, his proteinuria cleared and serum albumin and aminotransferases normalised, associated with disappearance of hepatitis B e antigen (HBeAg) and appearance of anti-HBeAg antibodies. After 12 months, without side effects, lamivudine was discontinued. He remains well 11 months off treatment.

Murine renal ischaemia‐reperfusion injury (Methods in Renal Research Paper)

SUMMARY:  Ischaemia/reperfusion is a major cause of acute kidney injury in native and transplant kidneys and is associated with significant morbidity and mortality. Murine models of renal ischaemia/reperfusion injury have great potential to improve understanding of the underlying processes and are an important focus of ongoing research into therapeutic and preventative strategies. Like all experimental models, murine models of renal ischaemia/reperfusion are prone to significant variability and results may be influenced by a number of technical and design factors. In this article we review the factors that may influence experimental results and provide a guide to conducting reproducible experiments in murine renal ischaemia/reperfusion.

An Australian tuberous sclerosis cohort: are surveillance guidelines being met?

Aim:  This study aims to describe the phenotypic and genotypic characteristics of 45 Australian patients with tuberous sclerosis complex (TSC), to assess risk factors for intellectual disability, to compare patients with TSC1 and TSC2 mutations and to assess adherence to surveillance recommendations.

KHA‐CARI guideline: Diagnosis and treatment of urinary tract infection in children

Child & Adolescent Renal Service, Royal Children’s and Mater Children’s Hospitals, University of Queensland, Department of Paediatrics and Child Health, The University of Queensland, Brisbane, Queensland, Department of General Medicine, The Royal Children’s Hospital, Vaccine and Immunisation Research Group and Rotavirus Research Group, Murdoch Childrens Research Institute, Department of Paediatrics, The University of Melbourne, Paediatric Imaging, Monash Health, Monash University, Department of Nephrology, Royal Children’s Hospital Melbourne, Murdoch Childrens Research Institute, University of Melbourne, Melbourne, Victoria, Department of Nephrology, Princess Margaret Hospital for Children, Perth, Western Australia, Nephrology, Sydney Children’s Hospital, School of Women’s & Children’s Health, University of New South Wales, and Centre for Kidney Research, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia

Epidemiology and demography of recently diagnosed cases of posterior urethral valves

Background:Posterior urethral valves (PUV) are a common cause of chronic kidney disease in young children, yet there is a paucity of knowledge about etiology and incidence. We sought to determine the incidence in Australia and to explore whether any demographic features were associated with incidence.Methods:PUV cases born between 2004 and 2009 in New South Wales (NSW) were identified by linkage analysis of two registries. The incidence rate was compared to registries from two other states. An audit was then performed at two pediatric centers.Results:Seventy-one boys were born and diagnosed in NSW with PUV, giving a live-birth incidence of 1.28 per 10,000. Fifty-one cases were treated at the two participating centers. Fifty-three percent of these were suspected on antenatal ultrasound. Of the remaining cases, 45% were detected in the neonatal period, with 50% of all postnatal cases presenting with urinary tract infection. No association was observed between maternal factors or socioeconomic status and PUV incidence.Conclusion:PUV was found in 1 in 7,800, a similar rate to international studies. Although almost half of cases are antenatally detected, a significant proportion present outside the neonatal period. Birth incidence was not associated with maternal or demographic factors.

Antibiotic-related renal failure and cystic fibrosis

Abstract:  Recently published reports suggest that the combination of aminoglycosides with ceftazidime may increase the risk of renal disease in cystic fibrosis. We describe a case of unusually severe acute tubular necrosis occurring in an adolescent with cystic fibrosis receiving i.v. gentamicin plus ceftazidime and discuss the possible mechanisms.

Racial disparities in pediatric kidney transplantation in New Zealand

Racial disparities in transplantation rates and outcomes have not been investigated in detail for NZ, a country with unique demographics. We studied a retrospective cohort of 215 patients <18 yr who started renal replacement therapy in NZ 1990–2012, using the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). Primary outcomes were time to first kidney transplant, death‐censored graft survival, and retransplantation after loss of primary graft. Europeans and Asians were most likely to receive a transplant (92% and 91% transplanted within five yr, respectively), and Pacific and Māori patients were less likely to receive a transplant than Europeans (51% and 46%, respectively), reflecting disparities in live donor transplantation. Pacific patients were more likely to have glomerulonephritis and FSGS. Pacific patients had five‐yr death‐censored graft survival of 31%, lower than Māori (61%) and Europeans (88%). No Pacific patients who lost their grafts were re‐transplanted within 72 patient‐years of follow‐up, whereas 14% of Māori patients and 36% of European and Asian patients were retransplanted within five yr. Current programs to improve live and deceased donation within Māori and Pacific people and management of recurrent kidney disease are likely to reduce these disparities.