The association between socioeconomic disadvantage and parent-rated health in children and adolescents with chronic kidney disease-the Kids with CKD (KCAD) study.

OBJECTIVE: To determine the association of socioeconomic disadvantage and parent-rated health in children with chronic kidney disease (CKD). METHODS: A total of 377 children (aged 6-18 years) with CKD stages I-V (n = 199), on dialysis (n = 43), or with a kidney transplant (n = 135) were recruited from 2012 to 2016 in Australia and New Zealand. Associations of five socioeconomic status (SES) components and the global SES index with parent-rated health of the child were examined using adjusted logistic regression. RESULTS: The median age of participants was 12.6 years (interquartile range (IQR) 8.9-15.5). In the entire cohort, the adjusted odds ratios (ORs) and 95% confidence intervals (CIs) for poor parent-rated health were 1.85 (1.13-3.03) for lower household income, 1.78 (1.08-2.96) for families that did not own their own home, 2.50 (1.50-4.16) for caregivers who rated their financial status as poor, 0.84 (0.51-1.38) for lower educational attainment, and 1.68 (1.04-2.72) for children whose primary caregivers were unemployed. With reference to the highest global SES index quartile, adjusted ORs for poor parent-rated health in descending order were 1.49 (0.69-3.21), 2.11 (1.06-4.20), and 2.20 (1.09-4.46), respectively. The association between low SES and poor parent-rated health was modified by CKD stage, where lower global SES index was independently associated with poor parent-rated health in children with CKD stages I-V, but not children on dialysis or with kidney transplants (p = 0.04). CONCLUSIONS: Low SES is associated with poor parent-rated health in children with CKD stages I-V, but not children on dialysis and with kidney transplants.

Children's experiences and expectations of kidney transplantation: A qualitative interview study.

AIM: Kidney transplantation offers improved quality of life and life expectancy compared with dialysis for children. This study aims to understand the experiences and expectations of children during the kidney transplantation process to inform clinical care. METHODS: Face-to-face, semi-structured interviews were conducted with 13 children and adolescents aged between 7 and 17 years in New Zealand who had received a kidney transplantation. Findings were conceptualized using thematic analysis with inductive coding. RESULTS: Three major themes were identified: transplant as the goal (the only real treatment and escaping dialysis); dealing with negative emotions (coping with anxiety and fear, guilt for siblings and burden of parent as donor); and enhancing understanding and knowledge (individualised education and reassurance from peer support). CONCLUSION: Children and adolescents view transplantation as freedom from dialysis and return to a more normal life. Children focus on the positive aspects of transplantation to reduce anxiety and be reassured in the face of uncertainty. Complex emotions arise when thinking about their donor. Children recognize transplantation is not a return to full health and actively seek out ways to self-manage their care, while remaining anxious about their future.

Treatment of recurrent focal segmental glomerulosclerosis post-kidney transplantation in Australian and New Zealand children: A retrospective cohort study.

Disease recurrence affects around a third of renal transplants for children with FSGS and is associated with poor graft outcomes. Unfortunately, there are no large trials guiding treatment for recurrent FSGS. We aimed to describe current therapies and treatment response for recurrent FSGS in 4 centres in Australia and New Zealand. Data were collected on children (age <18 years) with recurrent FSGS (1990-2015). We reviewed patient charts to obtain clinical information. Ethics approval was obtained from the relevant boards. Complete records were available on 24 patients (62% female, 54% Caucasian). Median time to first recurrence was 4 days (IQR 2-5 days). There were 14 separate treatment regimens, involving an average of 2 agents. The most common therapies were plasma exchange (20/24 patients, 83%), cyclosporin (15/24, 63%), and methylprednisolone (9/24, 38%). Full remission was achieved in 15 (63%), partial remission in 2 (8%), and no remission in 7 (29%) patients. Of the patients with no remission, 5 lost their graft to recurrent disease and 1 to concurrent acute vascular rejection. The plethora of different treatment regimens reflects the poor evidence guiding management for recurrent FSGS. More research is needed to improve outcomes.

Nationwide conversion to generic tacrolimus in pediatric kidney transplant recipients.

BACKGROUND: Bioequivalence between Tacrolimus Prograf® and generic tacrolimus formulations has been demonstrated in adult populations, however clinical experience and safety data regarding generic tacrolimus in pediatric transplant recipients is limited. This study aimed to evaluate conversion from Tacrolimus Prograf® to Sandoz® in pediatric renal transplant recipients nationwide. The primary outcome was a change in mean trough tacrolimus concentration. Additionally, changes in tacrolimus intra-patient coefficient of variation (CoV), allograft function, requirement for dose adjustments, and episodes of biopsy-proven rejection were evaluated. METHODS: Retrospective cohort study in 37 pediatric renal transplant recipients who switched to Tacrolimus Sandoz®. Each patient had three pre-conversion tacrolimus trough and creatinine concentrations within the 4 months prior and three post-conversion concentrations on day 3, 10, and the next subsequent level. Mean pre- and post-conversion tacrolimus trough concentrations and glomerular filtration rate (eGFR) were calculated. Tacrolimus concentration, CoV, and creatinine differences were compared by paired t test. RESULTS: Thirty-seven patients (41% females, age 3-18 years) were included. Average intra-patient difference in trough tacrolimus concentration was 0.05µg/l (95% CI -0.37 to 0.47). Average intra-patient difference in eGFR was -1.20 ml/min/1.732 (95% CI -3.53 to 1.13). Three patients had acute rejection during 12 months post-conversion compared to none during 12 months pre-conversion. CONCLUSIONS: Pediatric renal transplant recipients can be converted from Tacrolimus Prograf® to Sandoz® with negligible change in trough concentration, dose adjustments, or immediate allograft function. Of concern was the number of acute rejection episodes, however non-adherence contributed to at least one episode and this difference was determined clinically and statistically not significant.

Phenotypic variability of Dent disease in a large New Zealand kindred.

BACKGROUND: Dent disease 1 is a rare cause of chronic kidney disease (CKD) in childhood secondary to mutations in the gene encoding the chloride-proton exchanger, CLC-5, which is found mainly in the proximal tubule. Clinical manifestations are variable and there are no known genotype-phenotype correlations. CASE DIAGNOSIS/TREATMENT: The proband was identified as having a mutation in CLCN5. The extended family of the proband was invited to participate in a study of Dent disease after several males were noted to have a history of CKD. Urine retinol binding protein, urine calcium, serum creatinine, and DNA samples were collected for analysis. Ten hemizygous males and 6 heterozygous females were identified. Advanced CKD was detected in 3 males (1 child). Renal biopsies in 4 children showed both glomerular and tubulo-interstitial changes. There was no correlation between age and disease severity. CONCLUSIONS: This is the first reported family from the southern hemisphere with this condition. A novel CLCN5 mutation is described, c.1618G>C (p.Ala540Pro). The severity of renal disease varies greatly among individuals.

Laparoscopic versus open peritoneal dialysis catheter insertion for the management of pediatric acute kidney injury.

BACKGROUND: Acute pediatric dialysis is provided by a single center in New Zealand. Most acute dialysis in our center is performed in the under 5 age group. The advantage of using peritoneal dialysis (PD) in these children is the ability to perform continuous renal replacement therapy without always requiring an ICU setting, avoiding central venous access and promoting greater cardiovascular stability. The disadvantage of PD in the acute setting includes the requirement for immediate use and the potential for early leaks due to peritoneal disruption with resulting delayed use and restricted volumes. There is a growing trend toward minimally invasive surgery and the laparoscopic method allows this. Surgeons at this center have been using a laparoscopic technique since 2005. METHODS: We performed a 10-year review of acute PD at the Starship Hospital from 2003 to 2013. Data on 102 children who met the criteria were collected. RESULTS: These 102 children had 113 acute PD catheters. The two groups were comparable in terms of age and reason for presentation. The median age of the laparoscopic group was 2 years (interquartile range [IQR] 6) and the open group was 3 years (IQR 3.2). The predominant diagnosis for both groups was hemolytic uremic syndrome (HUS) accounting for 71% of laparoscopic cases, and 72% of open cases. The incidence of infection was 0% versus 7% in the laparoscopic versus open approach. Ten percent of patients required further manipulation of the catheter after initial insertion in the laparoscopic group, compared with 11% in the open approach. Conversion to hemodialysis (HD) due to catheter-related complications was seen in 10% of laparoscopic cases and 9% of the open cases. Dialysate fluid leak was noted in 26% in the laparoscopic group compared with 11% in the open group (p = 0.08). Anesthesia time is longer in the laparoscopic group (p = 0.008). CONCLUSION: We found no significant differences in complication rates between laparoscopic and open surgical approaches regarding acute PD catheter insertion. We saw a trend in increased leakage with laparoscopic procedures and a significantly longer operative time. We concluded that the laparoscopic approach in the acute situation for emergency dialysis is safe and effective.

Comparison of reticulocyte hemoglobin equivalent with traditional markers of iron and erythropoiesis in pediatric dialysis.

BACKGROUND: Anemia is a major complication for patients on chronic dialysis. Erythropoietin is effective if iron is available, however unnecessary iron supplementation results in iron overload. Reticulocyte hemoglobin equivalent (Ret-He) may be useful for assessing iron status. METHODS: A national retrospective cohort study including all children on chronic dialysis in New Zealand between 2007 and 2013, pairing Ret-He with demographic information, anemia indices, and markers of iron status. RESULTS: In 606 observations, we found a modest relationship between Ret-He and transferrin saturation (TSAT) (r = 0.34, p < 0.001) and a poor correlation between Ret-He and ferritin (r = 0.09, p = 0.04). There was a negative correlation between ferritin and hemoglobin (r = -0.14, p = 0.002), a weak positive correlation between TSAT and hemoglobin (r = 0.12, p = 0.007), and a modest positive correlation between Ret-He and hemoglobin (r = 0.22, p < 0.001). The diagnostic performance of Ret-He to detect absolute iron deficiency (cut-off value 28.9 pg, sensitivity 90 %, specificity 75 %, AUC 0.87) was good. CONCLUSIONS: Ret-He is a more relevant marker of iron status than ferritin and TSAT. This supports prospectively testing Ret-He to distinguish between iron deficiency and suboptimal erythropoietin dosing as competing causes for anemia. Ferritin is an unhelpful biomarker of iron deficiency in this setting.

Socio-economic status and quality of life in children with chronic disease: A systematic review.

Reduced quality of life (QoL) is a known consequence of chronic disease in children, and this association may be more evident in those who are socio-economically disadvantaged. The aims of this systematic review were to assess the association between socio-economic disadvantage and QoL among children with chronic disease, and to identify the specific socio-economic factors that are most influential. MEDLINE, Embase and PsycINFO were searched to March 2015. Observational studies that reported the association between at least one measure of social disadvantage in caregivers and at least one QoL measure in children and young people (age 2-21 years) with a debilitating non-communicable childhood disease (asthma, chronic kidney disease, type 1 diabetes mellitus and epilepsy) were eligible. A total of 30 studies involving 6957 patients were included (asthma (six studies, n = 576), chronic kidney disease (four studies, n = 796), epilepsy (14 studies, n = 2121), type 1 diabetes mellitus (six studies, n = 3464)). A total of 22 (73%) studies reported a statistically significant association between at least one socio-economic determinant and QoL. Parental education, occupation, marital status, income and health insurance coverage were associated with reduced QoL in children with chronic disease. The quality of the included studies varied widely and there was a high risk of reporting bias. Children with chronic disease from lower socio-economic backgrounds experience reduced QoL compared with their wealthier counterparts. Initiatives to improve access to and usage of medical and psychological services by children and their families who are socio-economically disadvantaged may help to mitigate the disparities and improve outcomes in children with chronic illnesses.

Infectious outcomes following gastrostomy in children receiving peritoneal dialysis.

BACKGROUND: Early institution of enteral feeding in paediatric end-stage kidney disease (ESKD) is recommended. For patients on peritoneal dialysis (PD) there is concern that gastrostomy tube (GT) insertion may be complicated by increased peritonitis, in particular fungal. Our unit favours early planned GT insertion, and for those with late presentation, there is prompt consideration of GT insertion following dialysis initiation. This study evaluates our rates of peritonitis with GT insertion following or concurrent with PD initiation. METHODS: This was a retrospective, single-centre, cross-sectional study of of 17 New Zealand children with ESKD who received PD in the period 2000-2011. Inclusion criteria were GT placement while on PD or initiation of PD within 72 h of GT insertion. RESULTS: There were no cases of fungal peritonitis among the 17 children; however, two cases of early peritonitis with organisms derived from the gastrointestinal tract were identified. No statistically significant difference was found between incident rates of bacterial peritonitis before GT placement (0.6 episodes per patient-year; 95% confidence interval (CI) 0.26-1.18) and post-GT placement (1.21 episodes per patient-year; 95% CI 0.69-1.97). CONCLUSION: Fungal peritonitis has never been encountered by out unit during its many years of experience in GT placement in patients without advanced malnutrition. When children on PD have insufficient dietary intake to maintain appropriate growth velocity, enteral feeding should be initiated promptly. A GT is considered to be safe for long-term use in selected patients.

Abdominal examination.

The four short cases in the Clinical Examination give the candidate the opportunity to demonstrate the skills developed during basic training, including tailoring the clinical examination to difficult settings, such as a child of a difficult age, a bored child or a child who needs winning over quickly. The abdominal short case gives the candidate a chance to show their general approach to children and their families as much as their skill in detecting ascites.

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 Maori 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 Maori (61%) and Europeans (88%). No Pacific patients who lost their grafts were re-transplanted within 72 patient-years of follow-up, whereas 14% of Maori patients and 36% of European and Asian patients were retransplanted within five yr. Current programs to improve live and deceased donation within Maori and Pacific people and management of recurrent kidney disease are likely to reduce these disparities.

Congenital nephrotic syndrome with prolonged renal survival without renal replacement therapy.

BACKGROUND: Infants with congenital nephrotic syndrome (CNS) develop severe nephrotic syndrome that is resistant to medical therapy, and bilateral nephrectomy is recommended toward the end of the first year of life followed by renal replacement therapy. CNS infants in New Zealand have been observed to exhibit a different course to those with the typical Finnish mutation. METHODS: A database of CNS children at our center was retrospectively examined. All cases diagnosed between 1975 and 2011 were reviewed. Demographic data, clinical features, genetic mutations, treatment, and outcome were extracted from clinical records. RESULTS: Thirty-five patients with CNS, 23 children of Maori descent, and 12 Caucasians . Fourteen had died of either bacterial sepsis or intracranial thrombosis. Maori children had displayed a highly variable and protracted timeline to end-stage renal disease (ESRD) with median renal survival of 30 years versus 0.7 years in Caucasian patients. Mutation analysis of NPHS1 showed a founder mutation in the Maori population. CONCLUSIONS: Congenital nephrotic syndrome in New Zealand Maori children exhibit a different clinical course to Caucasian children and have a mutation that was first described in this ethnic group.

Resolution of sleep-disordered breathing in a dialysis-dependent child post-renal transplantation.

This case alerts paediatricians and renal physicians to the potential for significant sleep-disordered breathing in children with renal disease, particularly those with end stage kidney disease requiring dialysis. In some cases, management of the underlying renal disease may result in amelioration of the sleep-disordered breathing. Proactive sleep history taking and formal sleep monitoring in experienced centres may be indicated for these children to limit morbidity, especially if respiratory support is indicated.

Rapid national survey of renal stones in New Zealand infants.

AIM: The aim of this study was to determine if there have been recent serious renal problems because of melamine among infants in New Zealand. METHODS: New Zealand paediatricians were surveyed in October 2008 using the New Zealand Paediatric Surveillance Unit network. RESULTS: Two cases of renal stones and none of unexplained renal failure in the previous 12 months were reported. Both cases of renal stones had an identifiable cause, and neither had features of melamine-related stones. CONCLUSION: This survey confirmed the expectation that this was not a discernible problem of recent serious melamine-associated renal damage in New Zealand. The method did however prove to be an effective way of undertaking a rapid determination of a possible recent serious health problem among children.

parents' perspectives and experiences of kidney transplantation in children: A qualitative interview study.

BACKGROUND AND OBJECTIVE: Kidney transplantation offers greater life expectancy, quality of life and participation compared with dialysis, in children with end stage kidney disease. This study explores the perspectives and experiences of parents of children undergoing kidney transplantation, as the experiences of parents in the process of transplantation is not completely understood. METHODS: Face-to-face semi-structured interviews were conducted with parents of transplanted children across New Zealand. Data were analysed using thematic analysis to identify themes of participant experiences and perspectives. RESULTS: We interviewed 13 mothers and four fathers of the transplanted children. Four themes were identified: actively pursuing transplant (the urgency of transplant; needing to drive the transplantation process); lack of on-going support (needing access to specialists; feeling unprepared for demands of transplantation, and vulnerability of unmet emotional concerns), pressure on the family unit (strain of distance; disrupting parent team; added burden of parent as donor; financial stress) and constant concern for the future (living with enduring uncertainty; pressure of responsibility; apprehension of teenage years). CONCLUSIONS: Parents of children need to play an active role in advocating and driving the transplantation process. Transplantation leads to parental role disruption, emotional and financial stress, and insecurity about the future for their child. These findings suggest the need for greater communication and transparency in the transplantation process with parents, improved emotional and financial support for families during and after transplantation, and explicit assistance for parental roles in families when a caregiver is the donor.

Quality of life of children and adolescents with chronic kidney disease: a cross-sectional study.

OBJECTIVE: The aim was to compare quality of life (QoL) among children and adolescents with different stages of chronic kidney disease (CKD) and determine factors associated with changes in QoL. DESIGN: Cross-sectional. SETTING: The Kids with CKD study involved five of eight paediatric nephrology units in Australia and New Zealand. PATIENTS: There were 375 children and adolescents (aged 6-18 years) with CKD, on dialysis or transplanted, recruited between 2013 and 2016. MAIN OUTCOME MEASURES: Overall and domain-specific QoL were measured using the Health Utilities Index 3 score, with a scale from -0.36 (worse than dead) to 1 (perfect health). QoL scores were compared between CKD stages using the Mann-Whitney U test. Factors associated with changes in QoL were assessed using multivariable linear and ordinal logistic regression. RESULTS: QoL for those with CKD stages 1-2 (n=106, median 0.88, IQR 0.63-0.96) was higher than those on dialysis (n=43, median 0.67, IQR 0.39-0.91, p<0.001), and similar to those with kidney transplants (n=135, median 0.83, IQR 0.59-0.97, p=0.4) or CKD stages 3-5 (n=91, 0.85, IQR 0.60-0.98). Reductions were most frequent in the domains of cognition (50%), pain (42%) and emotion (40%). The risk factors associated with decrements in overall QoL were being on dialysis (decrement of 0.13, 95% CI 0.02 to 0.25, p=0.02), lower family income (decrement of 0.10, 95% CI 0.03 to 0.15, p=0.002) and short stature (decrement of 0.09, 95% CI 0.01 to 0.16, p=0.02). CONCLUSIONS: The overall QoL and domains such as pain and emotion are substantially worse in children on dialysis compared with earlier stage CKD and those with kidney transplants.

Epidemiology and outcome of acute kidney injury in New Zealand children.

OBJECTIVES: To determine the aetiology, incidence and short-term outcomes of New Zealand children with acute kidney injury (AKI) requiring renal replacement therapy (RRT) over a 6-year period. METHODS: A retrospective chart review of all children requiring RRT for AKI from January 2001 to December 2006 at Starship Children's Hospital, Auckland, New Zealand was conducted. The primary outcome was survival to discharge. RESULTS: A total of 226 children required RRT for AKI over the 6-year study period. The annual incidence was 4.0 per 100,000 total population under 15 years of age. The commonest causes of AKI were post cardiac surgery (58%), haemolytic uraemic syndrome (17%), sepsis (13%) and glomerulonephritis (4%). The survival rate to hospital discharge was 89%. A total of 40% of all surviving children had one or more abnormalities at the time of discharge suggestive of ongoing renal dysfunction (hypertension, continuing need for antihypertensive medication, reduced estimated glomerular filtration rate or abnormal urinalysis). More Maori and Pacific Island children were treated for AKI than would be expected from population data (P < 0.0001). Sepsis and glomerulonephritis were seen more commonly as causes of AKI in Maori and Pacific Island children compared with New Zealand European children. CONCLUSION: In our study, 40% of surviving children had evidence of short-term renal dysfunction at discharge following AKI. This suggests that all children should undergo a period of follow-up after any episode of AKI to look for resolution or further development of signs of renal injury.

Neurocognitive and Educational Outcomes in Children and Adolescents with CKD: A Systematic Review and Meta-Analysis.

BACKGROUND AND OBJECTIVES: Poor cognition can affect educational attainment, but the extent of neurocognitive impairment in children with CKD is not well understood. This systematic review assessed global and domain-specific cognition and academic skills in children with CKD and whether these outcomes varied with CKD stage. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Electronic databases were searched for observational studies of children with CKD ages 21 years old or younger that assessed neurocognitive or educational outcomes. Risk of bias was assessed using a modified Newcastle-Ottawa scale. We used random effects models and expressed the estimates as mean differences with 95% confidence intervals stratified by CKD stage. RESULTS: Thirty-four studies (25 cross-sectional, n=2095; nine cohort, n=991) were included. The overall risk of bias was high because of selection and measurement biases. The global cognition (full-scale intelligence quotient) of children with CKD was classified as low average. Compared with the general population, the mean differences (95% confidence intervals) in full-scale intelligence quotient were -10.5 (95% confidence interval, -13.2 to -7.72; all CKD stages, n=758), -9.39 (95% confidence interval, -12.6 to -6.18; mild to moderate stage CKD, n=582), -16.2 (95% confidence interval, -33.2 to 0.86; dialysis, n=23), and -11.2 (95% confidence interval, -17.8 to -4.50; transplant, n=153). Direct comparisons showed that children with mild to moderate stage CKD and kidney transplants scored 11.2 (95% confidence interval, 2.98 to 19.4) and 10.1 (95% confidence interval, -1.81 to 22.0) full-scale intelligence quotient points higher than children on dialysis. Children with CKD also had lower scores than the general population in executive function and memory (verbal and visual) domains. Compared with children without CKD, the mean differences in academic skills (n=518) ranged from -15.7 to -1.22 for mathematics, from -9.04 to -0.17 for reading, and from -14.2 to 2.53 for spelling. CONCLUSIONS: Children with CKD may have low-average cognition compared with the general population, with mild deficits observed across academic skills, executive function, and visual and verbal memory. Limited evidence suggests that children on dialysis may be at greatest risk compared with children with mild to moderate stage CKD and transplant recipients.

ACE inhibitor fetopathy: a case series and survey of opinion amongst New Zealand paediatricians, obstetricians, neonatologists, and nephrologists.

The use of ACE (angiotensin converting enzyme) inhibitors is contraindicated throughout pregnancy due to potential adverse effects to the developing fetus (fetopathy). Despite this, women continue to receive ACE inhibitors both in New Zealand and overseas and large scale epidemiological studies have shown cases of associated harm to infants. We present three New Zealand infants with potential renal complications following in utero ACE inhibitor exposure including hypertension, renal failure and death. We also present data from an email-based survey of experience and opinion from relevant New Zealand specialists on how to best counsel women of child-bearing age regarding ACE inhibitors (quantitative and qualitative data). To our knowledge this is the first data published on this subject in New Zealand. ACE inhibitor exposure in pregnancy may result in potential renal, cardiac and limb complications for the developing fetus. How best to counsel women regarding ACE inhibitors and pregnancy remains an area for further discussion in New Zealand.