A REVIEW OF PERITONEAL DIALYSIS CATHETER INSERTION AND ITS COMPLICATIONS IN CHILDREN AND INFANTS

R KANAGARATNAM1, C CUI1, J FARQUHAR2, J MCCLUAND2, D HAHN2, A DURKAN2
1University Of Sydney, Camperdown, Australia, 2Department of Nephrology, The Children’s Hospital at Westmead, , Australia

Aims: To assess rates of PD catheter revision required within 1 month of insertion and to assess complication rates in the first year.
Background: Successful peritoneal dialysis (PD) is dependent upon effective catheter placement. There is limited data on early complication rates following catheter insertion in children.
Methods: A retrospective medical record review was conducted of double-cuff Tenckhoff PD catheters placed for proposed long-term renal replacement at The Children’s Hospital Westmead between 2007 and 2017. Patients undergoing catheter insertion for acute kidney injury were excluded. Multivariate regression analysis was used to look for risk factors for catheter failure.
Results : Forty three catheters were placed in 38 children. Median age at insertion was 7.1 years (range 0.1-16 years). Six catheters (14%) required revision within the first month due to impaired drainage from an ineffective catheter tip position. The median primary functional catheter lifetime was 322 days (3-893). In the first year, there were 20 episodes of peritonitis and 33 episodes of exit site infections (ESI) in 18 children. Six catheters were removed due to infection. There were 2 episodes of catheter migration requiring surgical intervention. There was no significant association between any complication and age, weight, sex, concurrent omentectomy or catheter insertion technique.
Conclusions: Approximately 1 in 7 children will require catheter revision within a month of insertion. Peritonitis or ESI are common but no significant associations were found between the method of catheter insertion and the likelihood of catheter revision, blockage or infection.


Biography:
Roshana Kanagaratnam is a 3rd year medical student at UNSW. Her research interests within nephrology include paediatrics, best practice and shared decision making

QUANTIFICATION OF MENDELIAN GENETIC DISEASE IN THE PAEDIATRIC RENAL CLINIC

Y UPENDRAN1, D MOWAT1,2, S  ALEXANDER3,4, S KENNEDY1,5, H McCARTHY1,3,4,5
1School of Women’s and Children’s Health, University of New South Wales, Sydney, Australia, 2Department of Clinical Genetics, Sydney Children’s Hospital, Randwick, Australia, 3Department of Nephrology, Children’s Hospital at Westmead, Westmead, Australia, 4Discipline of Paediatrics, University of Sydney, Sydney, Australia, 5Department of Nephrology, Sydney Children’s Hospital, Randwick, Australia

Aim: To determine the incidence of renal disease with a likely genetic aetiology in new referrals to tertiary paediatric nephrology within New South Wales (NSW).
Background: The introduction of massively parallel sequencing technology into the clinical sphere now provides additional, readily available and cost-effective testing for most genetic renal diseases. Yet, the size of the at-risk population who might benefit from this service is unknown which prevents accurate planning of services.
Methods: An algorithm was configured to determine children who had renal disease with a possible Mendelian genetic aetiology. A retrospective review of medical records was then undertaken for all new referrals to tertiary paediatric nephrology units within NSW from 2011-2015.  
Data collected included diagnosis, demographics and outcomes for those considered at-risk.
Results: Of 2285 new referrals seen during this period, 477 (20.9%) patients had a renal condition with a possible genetic basis. Syndromic CAKUT, glomerulopathies and ciliopathies accounted for >70% of the at-risk cohort. 211 (44%) of those at-risk had a positive family history although recorded consanguinity was low. Co-morbidities were common, particularly neurocognitive (21%). 185 (39%) had genetic testing of some form and in 105 of these (57%) a disease causing variant was found.
Conclusion: Approximately 100 children present to NSW tertiary nephrology per year with a disease of possible genetic aetiology. This has significant implications for themselves and their family. Service planning will ensure that this under recognised need is met with appropriate resources particularly counselling and sequencing technology. Future studies will look to further understand and address the needs of these children and their families.


Biography:
Mr Upendran is a fourth year medical student who completed this project as an independent learning project at the University of New South Wales.

LONG-TERM GLOMERULAR CAPILLARY GROWTH IN LAMBS FOLLOWING PRETERM BIRTH AND POSTNATAL VENTILATION

F JIANG1, MJ  DAHL2, K ALBERTINE2, MJ BLACK1, MR SUTHERLAND1
1Biomedicine Discovery Institute, Department of Anatomy and Developmental Biology, Monash University, Clayton, Australia, 2Department of Pediatrics, University of Utah, Salt Lake City, USA

Aim: To determine the long-term impact of preterm birth with postnatal ventilation on glomerular capillary morphology in lambs.
Background: Preterm birth (delivery <37 weeks gestation) can affect ongoing kidney development in neonates, resulting in the formation of glomeruli with shrunken capillary tufts. We have also shown, in lambs, that preterm birth and postnatal ventilation lead to substantial reductions in glomerular capillary length and filtration surface area at 3 days after birth. If this persists, it may result in renal dysfunction and nephron loss; indeed, preterm birth is an important risk factor for hypertension and chronic kidney disease.
Methods: Lambs were delivered preterm at 128 days (~0.85) of gestation, ventilated for 3-6 days, and then euthanised at 5 months term-equivalent age. Preterm lambs were compared to unventilated age-matched lambs born at term (n=8/group). Stereological methods were used to determine glomerular volume and glomerular capillary length and surface area in the outer, mid, and inner regions of the renal cortex. Glomerulosclerosis was assessed histologically.
Results: Glomeruli located in the inner cortex were significantly larger than those in the mid and outer cortex, but there was no significant difference in glomerular volume between the Preterm and Term lambs. Similarly, there was no significant effect of preterm birth on mean capillary length per glomerulus (Term: 8.1 ± 0.4 mm, Preterm: 7.6 ± 0.2 mm; p=0.32) or glomerular capillary surface area. Levels of glomerulosclerosis were low in all lambs, with no difference between groups.
Conclusions: Findings suggest that early-life impairments in glomerular capillary growth following preterm birth and ventilation may not persist long-term; the underlying causes of hypertension and renal disease in individuals born preterm remain to be fully elucidated.


Biography:
Charles (Fan) Jiang is an Honours student at the Biomedicine Discovery Institute, Department of Anatomy and Developmental Biology, Monash University.

THE URINARY PROTEOME OF PRETERM NEONATES: ASSOCIATIONS WITH GESTATIONAL AGE AND RENAL FUNCTION

M SUTHERLAND1, A SHAH2,3, I HANCHAPOLA3, C HUANG3, R SCHITTENHELM3, M BLACK1
1Biomedicine Discovery Institute, Department of Anatomy and Developmental Biology, Monash University, Clayton, Australia, 2Monash Bioinformatics Platform, Monash University, Clayton, Australia, 3Monash Biomedical Proteomics Facility, Monash University, Clayton, Australia

Aim: To determine the impact of gestational age on the urinary proteome of preterm infants, and identify candidate biomarkers of renal dysfunction/injury.
Background: Preterm infants (born <37 weeks gestation) are highly susceptible to acute kidney injury (AKI). Serum creatinine and urine output, currently used in the diagnosis of AKI in infants, are both late-onset and non-specific markers that can also be influenced by renal functional maturity. As yet, no AKI diagnostic criteria have been developed specifically for preterm infants. Early diagnosis, enabling immediate intervention, is key to mitigating the adverse impact of neonatal AKI on long-term renal health. Therefore, there is a critical need to identify specific AKI biomarkers (independent of renal maturity) that can be assessed non-invasively in this vulnerable population.
Methods: Spot urine samples were collected from 30 preterm infants (born 24-35 weeks gestation) at postnatal day 7. Global protein quantification was performed using nano-LC-ESI DIA mass spectrometry. Linear regression models were used to determine the associations between individual protein levels, gestational age, and measures of renal function (eg. serum creatinine, fractional excretion of sodium (FENa), and pathological proteinuria (≥ 500mg/L)).
Results: A total of 1,255 urinary proteins were quantified across the samples. Of these, 283 (23%) were significantly correlated with gestational age at birth, including some known indicators of glomerular (neprilysin, podocalyxin) and tubular (cystatin C, cubilin) injury. After correcting for gestational age, proteins most strongly correlated with renal function parameters (p<0.0001) included PPGB (serum creatinine: r²=0.598), CSTN1 (FENa: r²=0.744), and AATC (proteinuria: r²=0.677).
Conclusions: Gestational age at birth significantly impacts the early postnatal urinary proteome of preterm infants. Longitudinal studies are now required to assess the utility of candidate biomarkers.


Biography:
Dr. Megan Sutherland is a NHMRC CJ Martin Early Career Research Fellow at the Biomedicine Discovery Institute, Department of Developmental Biology, Monash University. Her research is focused on understanding the impact of preterm birth on renal development and function.

PROGNOSTIC INDICATORS OF RENAL OUTCOMES IN BOYS WITH POSTERIOR URETHRAL VALVES

S KEENAN1, N HOMAIRA1, S KENNEDY1,2
1School of Women’s & Children’s Health, University Of New South Wales, Sydney, Australia, 2Sydney Children’s Hospital , Randwick, Australia

Aims: To identify patient-related factors which predict low glomerular filtration rate (GFR) in boys with posterior urethral valves (PUV).
Background: PUV are a congenital anomaly and an important cause of chronic kidney disease (CKD) in childhood. Up to 40% of boys who are born with PUV will develop end-stage kidney disease (ESKD) by early adulthood. Accurate prognostication of renal outcome is important to counsel families and guide treatment.
Methods: A single-centre cohort study of boys born with PUV between 1999 and 2017. Primary outcomes of interest were renal replacement therapy (RRT) or CKD stage 3-5. Perinatal and clinical variables were analysed using univariate and multivariate Cox models. Prognostic indicators were validated using ROC and survival analysis.
Results: A cohort of 60 boys was identified with median follow up of 61 months (range 6-210 months). Seven (12%) and 20 (33%) boys progressed to RRT and CKD3-5, respectively. On univariate analysis, nadir serum creatinine (SCr) in the first year of life, eGFR at 12 months of age and oligohydramnios were predictors of outcome. Only nadir SCr was predictive of RRT/CKD3-5 on multivariate Cox regression (HR 1.020, 95% CI 1.01-1.03; p<0.01). CKD- and RRT-free survival were significantly predicted by stratifying nadir SCr in to 3 categories: low, < 35μmol/L (<1% developed CKD3-5), moderate, 35-75μmol/L (55% developed RRT/CKD3-5) and high, >75μmol/L (100% developed RRT/CKD3-5). Kaplan-Meier plots showed different event-free survival times between each of the three stratifications of nadir SCr (p<0.001).
Conclusions: Our study confirms the predictive value of nadir SCr, and increased sensitivity of a lower cut-off of 35 μmol/L. This knowledge will assist with prognostication and allows identification of boys who may be suitable for intervention trials.


Biography:
Sarah is a 5th year medical student at the university of New South Wales. This research was conducted as part of B Med Sc (Honours) studies in 2017.

PREDICTORS OF CHRONIC KIDNEY DISEASE PROGRESSION DURING LATE ADOLESCENCE

H THAKKAR1, S KENNEDY1,2
1University Of New South Wales, Sydney, Australia, 2Sydney Children’s Hospital, Randwick, Australia

Background: There is a paucity of evidence about the epidemiology and predictors of progression of chronic kidney disease (CKD) in adolescents.
Aim: To describe the clinical characteristics of a cohort of Australian adolescents with CKD and identify risk factors for disease progression.
Methods: This was a single-centre cohort study of adolescents aged 14 to 18 years, seen in nephrology clinics between 2007 and 2017. Demographic and clinical data were retrieved from electronic medical records. Univariate analysis was performed to identify associations between baseline covariates and progression to a composite outcome of 50% decline in glomerular filtration rate (eGFR) or stage 5 CKD. Significant covariates were included in a logistic regression model.
Results: Two hundred and fifty three adolescents with pre-endstage CKD were seen during this period. The main primary diseases were glomerulopathies (35.2%) and congenital anomalies of the kidney and urinary tract (28.9%). The majority were at CKD Stage 1 (53.3%) or 2 (26.5%) at baseline. Thirteen patients (9.3%) reached the composite outcome. Forty-two patients (30.0%) progressed by at least one CKD stage. Twenty-two of these 42 patients (52.4%) were at milder stages of CKD (Stage 1 or 2) at baseline. In univariate analysis, significant predictors of progression to the composite endpoint included CKD Stage 3 or 4 at entry (p=0.02), severe proteinuria (p=0.018), short stature (p=0.047), anaemia (p=0.07), hypoalbuminemia (p<0.005) and hyperphosphatemia (p<0.005). In the multivariate model, CKD Stage 3 or 4 (OR 5.11, 95% CI 1.15-22.60; p=0.032) and severe proteinuria (OR 9.19, 95% CI 1.26-67.16; p=0.029) were independent predictors.
Conclusion: Prior to transition, kidney function deteriorates even in adolescents at milder stages of CKD. Therefore, close follow-up during the transition period is crucial.


Biography:
Heeral Thakkar is a 5th year medical student at University of New South Wales. This research was conducted as part of an Independent Learning Project in 2017.

RENAL BIOPSY ADEQUACY AND COMPLICATIONS IN CHILDREN

C CUI1, R KANAGARATNAM1, N GRAF2, A DURKAN2
1University of Sydney, Camperdown, Australia, 2Children’s Hospital at Westmead, Westmead, Australia

Background: Renal biopsy is commonly used in the diagnosis of kidney diseases. Increasingly in paediatric practice, biopsies are done under general anaesthetic by interventional radiologists, who perform their own ultrasound compared to nephrologists who use ultrasonographer guided ultrasound in a sedated child.  Both sets of operators use spring-loaded automated biopsy guns with same gauged needles. The sample adequacy and complication rates have not been determined in the two groups.
Aims: To compare biopsy size and adequacy between radiologists and nephrologists and to compare complication rates.
Methods: We examined consecutive kidney biopsies done between Jan 2008 and Dec 2017 and probed the electronic medical records of patients for data regarding complications.In native biopsies, successful processing for light microscopy, immunofluorescence, and electron microscopy was used as proxy for specimen adequacy. In transplant biopsies, adequate specimens contained at least 10 glomeruli and 2 arteries, as per the Banff criteria.Data were analysed using Mann–Whitney U test and Pearson’s chi-squared test in SPSS .
Results: Radiologists performed 112 of the 355 biopsies obtained. Radiologists obtained more renal cores (median number 3 vs 2, p<0.001), more total glomeruli (median number 32 vs 15, p<0.001), and more glomeruli per core (median number 12.0 vs 7.0, p<0.001). There were no differences in specimen adequacy of native renal biopsies between two groups but radiologists obtained more adequate transplant biopsy specimens (95.0% vs 51.6%, p<0.001).There were no significant differences in complications including haematoma, bleeding, de novo haematuria, transfusion requirement, infections or AV fistula formation between two groups.
Conclusion: Radiologists obtained significantly larger specimens than nephrologists. There were no significant differences in post procedural complications.


Biography:
Chen Cui is a final year medical student from University of Sydney who has a special interest in nephrology.

CHILD AND PARENTAL PERSPECTIVES ON COMMUNICATION AND DECISION-MAKING IN PAEDIATRIC CHRONIC KIDNEY DISEASE: A FOCUS GROUP STUDY

T GUTMAN1,2, C HANSON1,2, S BERNAYS1, J CRAIG1,2, A SINHA3, A DART4, A EDDY5, D GIPSON6, D BOCKENHAUER7, H YAP8, J GROOTHOFF9, M ZAPPITELLI10, N WEBB11, S  ALEXANDER2, S GOLDSTEIN12, S FURTH13, S SAMUEL14, T BLYDT-HANSEN5, J DIONNE5, M MICHAEL15, S WENDERFER15, W WINKELMAYER16, H CURRIER15, S MCTAGGART17, A WALKER18, A RALPH1,2, A JU1,2, L JAMES1,2, S CARTER2, A TONG1,2
1School of Public Health, The University of Sydney, Sydney, Australia, 2Centre for Kidney Research, The Children’s Hospital at Westmead, Sydney, Australia, 3Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India, 4Department of Pediatrics and Child Health, The Children’s Hospital Research Institute of Manitoba, University of Manitoba, Manitoba, Canada, 5Department of Pediatrics, BC Children’s Hospital and University of British Columbia, Vancouver, Canada, 6Division of Nephrology, Department of Pediatrics, University of Michigan, Ann Arbor, United States, 7UCL Centre for Nephrology and Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 8Department of Pediatrics, Yong Loo Lin School of Medicine, National University of Singapore, , Singapore, 9Department of Pediatric Nephrology, Emma Children’s Hospital AMC Academic Medical Center, Amsterdam, The Netherlands, 10Department of Pediatrics, Division of Nephrology, Toronto Hospital for Sick Children, University of Toronto, Toronto, Canada, 11Department of Pediatric Nephrology and NIHR Manchester Clinical Research Facility, University of Manchester, Manchester Academic Health Science Centre, Royal Manchester Children’s Hospital, Manchester, Manchester, United Kingdom, 12Division of Nephrology and Hypertension, Cincinnati Children’s Hospital Medical Center, Cincinnati, United States, 13Departments of Pediatrics and Epidemiology, Perelman School of Medicine and Division of Nephrology, The Children’s Hospital of Philadelphia, Philadelphia, United States, 14Department of Pediatrics, Section of Nephrology, University of Calgary, Calgary, Canada, 15Renal Section, Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston, United States, 16Selzman Institute for Kidney Health, Section of Nephrology, Baylor College of Medicine, Houston, United States, 17Child and Adolescent Renal Service, Lady Cilento Children’s Hospital, Brisbane, Australia, 18Department of Nephrology, Royal Children’s Hospital Melbourne, Department of Paediatrics, University of Melbourne, and Murdoch Children’s Research Institute, Melbourne, Australia

Aim: To describe the perspectives of children with chronic kidney disease (CKD) and their parents with regard to communication and decision-making.
Background: Effective communication and shared decision-making improves quality of care and patient outcomes, but can be particularly challenging in pediatric chronic disease as children depend on their parents and clinicians to manage complex healthcare and developmental needs.
Methods: Children with CKD (n=34) and parents (n=62) from six centers across six cities in Australia, Canada and the United States participated in 16 focus groups. Transcripts were analysed thematically.
Results: We identified four themes: (1) disempowered by knowledge imbalance (unprepared and ill-informed, suspicion of censorship, inadequacy as technicians); (2) recognizing own expertise (intuition and instinct unique to parental bond, emerging wisdom and confidence, identifying opportunities for control and inclusion, empowering participation in children); (3) striving to assert own priorities (negotiating broader life impacts, choosing to defer decisional burden, overprotected and overruled, struggling to voice own preferences); and (4) managing child’s involvement (respecting child’s expertise, attributing ‘risky’ behaviors to rebellion, protecting children from illness burden).
Conclusions: Parents value partnership with clinicians and consider long-term and quality of life implications of their child’s illness. Children with CKD want more involvement in treatment decision-making but are limited by vulnerability, fear, and uncertainty. There is a need to support the child to better enable them to become partners in decision-making and prepare them for adulthood. Collaborative and informed decision-making that addresses the priorities and concerns of both children and parents is needed.


Biography:
Talia Gutman is a Research Officer and PhD candidate at the Sydney School of Public Health, The University of Sydney, Australia. Her primary research interest is in the area of patient and caregiver involvement in research in chronic kidney disease. She has conducted international qualitative studies on patient involvement in research in chronic kidney disease and has experience in interviews, focus groups, nominal group technique, and survey research to elicit stakeholder perspectives with the goal of informing patient-centred programs and interventions.

THE MULTIDISCIPLINARY PAEDIATRIC RENAL GENETICS CLINIC: A MODEL FOR MAINSTREAMING GENETICS IN MEDICINE

E WILKINS1,2,3, Z STARK1,2,3,, S WHITE1,2,3,4, A MALLETT1,2,5, C QUINLAN1,2,4,6

1KidGen Renal Genetics Flagship, Australian Genomic Health Alliance, Melbourne, VIC; 2Murdoch Childrens Research Institute, Melbourne VIC; 3Victorian Clinical Genetics Service, Melbourne, VIC; 4Department of Paediatrics, University of Melbourne, Melbourne, VIC; 5Kidney Health Service, Royal Brisbane and Women’s Hospital, Brisbane, QLD; 6Department of Nephrology, Royal Children’s Hospital, Melbourne, VIC

Aim: To evaluate processes and outcomes of the joint Royal Children’s Hospital and Victorian Clinical Genetics Services Renal Genetics Clinic in order to inform future practice.

Background: Our understanding of the genetic factors involved in many conditions is continually evolving, and the availability of genomic testing is rapidly expanding, creating a necessity for new services, new ways of working, and modified professional roles in order to maximize benefit to patients and families. The multidisciplinary clinic is one model to achieve this and integrate genetics into mainstream medicine.

Methods: A retrospective clinical audit of the first 14 months of the Clinic was conducted.

Results: The Clinic was established in February 2016, with the aim of improving diagnosis rates and informing management in patients with suspected genetic renal disease. The clinic team includes a nephrologist, clinical geneticist, genetic counsellor, and administrative support, with each member of the team contributing unique expertise and benefitting from opportunities to cross-train and up-skill.

Patients typically attended for one or more of: clinical diagnostic assessment, genetic counselling, genetic/genomic testing, and/or research recruitment; and benefitted from multidisciplinary case review before, during, and after appointments. In addition, the clinic team contributes to KidGen, an Australian collaboration between clinical, diagnostic and research teams that aims to better understand inherited renal disease.

Data for 34 patients is presented, including instances of genetic diagnosis leading to altered management.

Conclusions: Results of the audit provide insight into the first paediatric multidisciplinary renal genetics clinic in Australasia. Outcome data has demonstrated benefits for patient care, as well as opportunities for biological relatives to access genetics services. Anecdotal evidence supports staff satisfaction with the service model and professional development opportunities.

About ANZSN

The ASM is hosted by Australian and New Zealand Society of Nephrology.

The aims of the Society are to promote and support the study of the kidney and urinary tract in health and disease, and to ensure the highest professional standards for the practice of nephrology in Australia and New Zealand.

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