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Pediatric solid organ transplantation (SOT) is a preferred treatment for medically suitable children with end-stage organ failure. Still, many of them have no access to transplantation owing to socioeconomic constraints or lack of transplant facilities in low- and middle-income countries (LMIC). Establishing pediatric SOT programs in LMIC offers children the opportunities to receive transplant care in more familiar home environments as well as help curtail transplant tourism and improve transplant outcomes as pediatric transplantation would be performed ethically and legally. The International Pediatric Transplant Association (IPTA) is a professional organization aiming to promote safe, ethical, and high-quality pediatric transplantation worldwide. This society paper describes major obstacles to pediatric SOT in LMIC and provides guidance on developing and/or expanding pediatric SOT programs in such countries. We also summarize available resources from the IPTA Outreach Program to help establish and support pediatric SOT programs in LMIC.

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Supplemental Material, sj-docx-1-ptd-10.1177_08968608221091023 for Risk factors and outcomes of neonates with acute kidney injury needing peritoneal dialysis: Results from the prospective TINKER (The Indian PCRRT-ICONIC Neonatal Kidney Educational Registry) study by Sidharth Kumar Sethi, Sanjay Wazir, Jagdish Sahoo, Gopal Agrawal, Naveen Bajaj, Naveen Parkash Gupta, Shishir Mirgunde, Binesh Balachandran, Kamran Afzal, Anubha Shrivastava, Jyoti Bagla, Sushma Krishnegowda, Ananth Konapur, Azmeri Sultana, Kritika Soni, Nikhil Nair, Divya Sharma, Prajit Khooblall, Avisha Pandey, Khalid Alhasan, Mignon McCulloch, Timothy Bunchman, Abhishek Tibrewal and Rupesh Raina in Peritoneal Dialysis International

Supplemental Material, sj-docx-2-ptd-10.1177_08968608221091023 for Risk factors and outcomes of neonates with acute kidney injury needing peritoneal dialysis: Results from the prospective TINKER (The Indian PCRRT-ICONIC Neonatal Kidney Educational Registry) study by Sidharth Kumar Sethi, Sanjay Wazir, Jagdish Sahoo, Gopal Agrawal, Naveen Bajaj, Naveen Parkash Gupta, Shishir Mirgunde, Binesh Balachandran, Kamran Afzal, Anubha Shrivastava, Jyoti Bagla, Sushma Krishnegowda, Ananth Konapur, Azmeri Sultana, Kritika Soni, Nikhil Nair, Divya Sharma, Prajit Khooblall, Avisha Pandey, Khalid Alhasan, Mignon McCulloch, Timothy Bunchman, Abhishek Tibrewal and Rupesh Raina in Peritoneal Dialysis International

Contains fulltext : 290812.pdf (Publisher’s version ) (Open Access) Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85-90% of patients attain complete remission of proteinuria within 4-6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70-80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given.

Supplemental Material, sj-pptx-1-ptd-10.1177_0896860820982120 for ISPD guidelines for peritoneal dialysis in acute kidney injury: 2020 Update (paediatrics) by Peter Nourse, Brett Cullis, Fredrick Finkelstein, Alp Numanoglu, Bradley Warady, Sampson Antwi and Mignon McCulloch in Peritoneal Dialysis International

The global nephrology community recognizes the need for a cohesive strategy to address the growing problem of end-stage kidney disease (ESKD). In March 2018, the International Society of Nephrology hosted a summit on integrated ESKD care, including 92 individuals from around the globe with diverse expertise and professional backgrounds. The attendees were from 41 countries, including 16 participants from 11 low- and lower-middle-income countries. The purpose was to develop a strategic plan to improve worldwide access to integrated ESKD care, by identifying and prioritizing key activities across 8 themes: (i) estimates of ESKD burden and treatment coverage, (ii) advocacy, (iii) education and training/workforce, (iv) financing/funding models, (v) ethics, (vi) dialysis, (vii) transplantation, and (viii) conservative care. Action plans with prioritized lists of goals, activities, and key deliverables, and an overarching performance framework were developed for each theme. Examples of these key deliverables include improved data availability, integration of core registry measures and analysis to inform development of health care policy; a framework for advocacy; improved and continued stakeholder engagement; improved workforce training; equitable, efficient, and cost-effective funding models; greater understanding and greater application of ethical principles in practice and policy; definition and application of standards for safe and sustainable dialysis treatment and a set of measurable quality parameters; and integration of dialysis, transplantation, and comprehensive conservative care as ESKD treatment options within the context of overall health priorities. Intended users of the action plans include clinicians, patients and their families, scientists, industry partners, government decision makers, and advocacy organizations. Implementation of this integrated and comprehensive plan is intended to improve quality and access to care and thereby reduce serious health-related suffering of adults and children affected by ESKD worldwide.

BACKGROUND: Fluid resuscitation is the recommended management of shock, but increased mortality in febrile African children in the FEAST trial. We hypothesised that fluid bolus-induced deaths in FEAST would be associated with detectable changes in cardiovascular, neurological, or respiratory function, oxygen carrying capacity, and blood biochemistry. METHODS: We developed composite scores for respiratory, cardiovascular, and neurological function using vital sign data from the FEAST trial, and used them to compare participants from FEAST with those from four other cohorts and to identify differences between the bolus (n=2097) and no bolus (n=1044) groups of FEAST. We calculated the odds of adverse outcome for each ten-unit increase in baseline score using logistic regression for each cohort. Within FEAST participants, we also compared haemoglobin and plasma biochemistry between bolus and non-bolus patients, assessed the effects of these factors along with the vital sign scores on the contribution of bolus to mortality using Cox proportional hazard models, and used Bayesian clustering to identify subgroups that differed in response to bolus. The FEAST trial is registered with ISRCTN, number ISRCTN69856593. FINDINGS: Increasing respiratory (odds ratio 1·09, 95% CI 1·07-1·11), neurological (1·26, 1·21-1·31), and cardiovascular scores (1·09, 1·05-1·14) were associated with death in FEAST (all p

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No Abstract. South African Medical Journal Vol. 96(5) 2006: 419

Spontaneous pathologic arterial calcifications in childhood can occur in generalized arterial calcification of infancy (GACI) or in pseudoxanthoma elasticum (PXE). GACI is associated with biallelic mutations in ENPP1 in the majority of cases, whereas mutations in ABCC6 are known to cause PXE. However, the genetic basis in subsets of both disease phenotypes remains elusive. We hypothesized that GACI and PXE are in a closely related spectrum of disease. We used a standardized questionnaire to retrospectively evaluate the phenotype of 92 probands with a clinical history of GACI. We obtained the ENPP1 genotype by conventional sequencing. In those patients with less than two disease-causing ENPP1 mutations, we sequenced ABCC6. We observed that three GACI patients who carried biallelic ENPP1 mutations developed typical signs of PXE between 5 and 8 years of age; these signs included angioid streaks and pseudoxanthomatous skin lesions. In 28 patients, no disease-causing ENPP1 mutation was found. In 14 of these patients, we detected pathogenic ABCC6 mutations (biallelic mutations in eight patients, monoallelic mutations in six patients). Thus, ABCC6 mutations account for a significant subset of GACI patients, and ENPP1 mutations can also be associated with PXE lesions in school-aged children. Based on the considerable overlap of genotype and phenotype of GACI and PXE, both entities appear to reflect two ends of a clinical spectrum of ectopic calcification and other organ pathologies, rather than two distinct disorders. ABCC6 and ENPP1 mutations might lead to alterations of the same physiological pathways in tissues beyond the artery.

Background: Despite the undeniable diagnostic benefits of urodynamic studies (UDS), their adoption into clinical practice in Africa has been slow. This study aimed to review the use of invasive UDS in children at a tertiary paediatric hospital in South Africa. Methods: A retrospective analysis of 1108 UDS was conducted. Patient demographic characteristics, primary diagnosis, indication and urodynamic outcomes were reviewed. Presence of urodynamic high-risk features were documented, and a comparison was made between the first study and follow-up study. Results: This study revealed increasing trends in the use of UDS from 2015. Referrals were from Urology (37.7%), Spinal defects clinic (34.4%), Nephrology (20.8%) and other departments (7.0%). The most common reason for referral was review of medical treatment (36.5%). Spinal dysraphism (58.3%) accounted for the majority of conditions seen. Majority (59.1%) of the patients were receiving more than one type of bladder treatment at the time of their first study, with clean intermittent catheterisation (46.5%) being the most common form of bladder management. 97.5% of studies were performed using transurethral bladder catheterization. Urodynamic diagnosis was neurogenic in 74.0%, anatomical (12.2%), functional (8.8%) and normal (5.0%). There was statistically significant improvement in bladder compliance, detrusor leak point pressure and detrusor sphincter dyssynergia between the first study and a subsequent study following therapeutic intervention. Conclusion: The unique ability of UDS to demonstrate changes in detrusor pressures, which is a common reason for therapy failure, makes UDS an invaluable tool in the diagnosis and management of children with lower urinary tract dysfunction.

Background: Acute post streptococcal glomerulonephritis (APSGN), although not a common cause of progressive kidney failure, is an important cause of paediatric hospital admission, parental worry, and acute kidney injury (AKI). In well-resourced settings, there has been a decline over the last three decades in the incidence of APSGN in children with this condition almost disappearing in Central Europe. However, this is not the case in less privileged countries such as in South Africa (SA) where APSGN is still a major public health problem and a frequent cause of paediatric hospital admission and AKI. Nevertheless, APSGN in South Africa has not been studied adequately in recent times and not currently addressed as an important public health issue. Objectives: In this retrospective study we describe the occurrence of APSGN, the aetiology, clinical presentation, and complications among children (

Background Adolescents and young adults [AYA] are important users of the nephrology health care services. Worldwide, there is a paucity of data on AYA kidney disease and outcomes. This study evaluates kidney outcomes, survival and challenges faced by AYA in a South African setting. Methods This 5-year retrospective study included AYA [aged 10-24] with chronic kidney disease, at a tertiary nephrology service in South Africa. Descriptive analysis characterised the aetiology of kidney disease. A comparative analysis of baseline characteristics, outcomes and social challenges were performed between patients attending a dedicated AYA clinic and those attending the standard adult clinics [non-AYA clinics]. Primary composite outcome assessed included doubling of creatinine, reduction of eGFR >40%, end-stage kidney disease and death. Logistic regression evaluated associations between relevant variables, death and lost to follow up [LTFU]. Results The total AYA cohort consisted of 292 patients, 111 (38.0%) attended the AYA clinic and 181 (62.0%) the non-AYA clinics. The main aetiologies of disease were glomerular 212 (72.6%), congenital anomalies of the urinary tract 31 (10.6%), and hereditary conditions 24 (8.2%). There was a significantly lower mortality (p=0.007) and reduction in LTFU (p=0.012) in the cohort attending the AYA clinic. A statistically significant composite outcome (p=0.018), with improved kidney survival was found in the AYA clinic group. High proportions of nonadherence (33.9%) and substance use (25.0%) was demonstrated in both cohorts. Conclusion This study adds to the dearth of literature on AYA kidney disease. A dedicated nephrology AYA clinic is shown to have lower mortality, less LTFU and improved kidney outcomes, which is essential in a resource-limited setting where access to kidney replacement therapy is restricted.

Introduction: Dialysis is a temporary renal replacement therapy (RRT) to keep the child healthy and alive when in end stage kidney failure (ESKF) while being worked up for kidney transplant, the preferred treatment. Chronic peritoneal dialysis (PD) is the preferred first choice of dialysis modality in many centers because of its advantages over hemodialysis (HD). In recent years, there have been advances to improve the performance and survival of PD as a modality for renal replacement. Despite these improvements, complications still arise, sometimes warranting a switch to HD. We sought to investigate the extent to which children at Red Cross War Memorial Children's Hospital (RCWMCH) drop-out from chronic PD and describe some of the reasons for this drop-out. Objectives: To describe the rate of drop-out of children with ESKD from chronic PD, the timing and factors associated with this drop-out at RCWMCH. Methods: This was a retrospective descriptive study, carried out in the renal ward, E2, of RCWMCH in Cape Town. Eligible participants were identified from the renal transplant waiting lists over the study period. Patient folders were retrieved following ethical approval, for extraction of relevant data. Outcome measures: Proportion dropping-out during the study period (permanent switch to HD or death from PD related complications), factors associated with dropout and time from initiation of chronic PD to drop-out. Utility of the study: Findings from this study will help in designing strategies to improve chronic PD patient outcomes, prolongation of PD technique survival and reducing the costs of chronic dialysis at RCWMCH. Results: A total of 111 children were listed for transplantation between January 2009 and December 2018, 67 were treated with PD. Complete data was available for 52 of the 67 children who received PD. Overall, 17/52 (32.7%) dropped-out during the study period. Most (>50%) of them dropped-out within the first 1-2 years of being on PD. The only significant associated factor was one or more episodes of peritonitis. Recommendation: There is a need to step up measures to prevent peritonitis in chronic PD patients so as to prolong stay on PD until a kidney transplant is available, as well as improve kidney transplantation rates. Dissemination of results: Results were presented at the Department of Pediatrics and Child Health Research Day 2019 and at the world congress of nephrology international conference 2021. Results will be submitted for publication in a peer reviewed journal.

Aim: Noninvasive Continuous Positive Airway Pressure (nCPAP) and High Flow Nasal Cannula oxygen therapy (HFNC) are non-invasive ventilation (NIV) modalities appropriate for children in developing countries. There is minimal literature describing nCPAP and HFNC use in children with respiratory compromise secondary to non-pulmonary disease. This study aimed to describe the characteristics and outcomes of all children without primary lung pathology, who received nCPAP and HFNC during their admission to Red Cross War Memorial Children's Hospital, Cape Town, South Africa. Methods: This was a prospective observational study of routinely collected data, between August 2015 and January 2016. Primary and secondary outcome measures were NIV failure (progression to intubation and invasive ventilation) and Paediatric Intensive Care Unit (PICU) admission respectively. Comparative statistics were conducted using Mann-Whitney U tests. Data significantly associated with the primary and secondary outcomes on univariate analysis were entered into backward stepwise logistic regression models to determine independent predictive factors. Results: There were 31 cases of nCPAP and one case of HFNC use in 31 patients (median (IQR) age 3.5 (1.8 – 7.6) months. The majority (n=23; 71.9%) presented with primary diarrhoeal disease. There were two deaths (6.5%), 17 (53.1%) Paediatric Intensive Care (PICU) admissions, and five (15.6%) cases received invasive ventilation (NIV failure). Median (IQR) duration of hospital stay was 11.50 (6.0 – 17.5) days. Patients who failed NIV had lower admission SaO2 than those without treatment failure (95 (95-99)% vs 100 (100-100)%; p = 0.03). On multiple logistic regression, lower temperature (OR 0.19; 95% CI 0.05 – 0.78; p = 0.02) and receiving inotropes in the emergency setting, (OR 23.05; 95% CI 1.64 – 325.06; p = 0.02) were independently associated with PICU admission. Conclusions: nCPAP is used clinically for the management of children with respiratory compromise secondary to non-pulmonary illnesses, particularly diarrhoeal disease. Larger controlled clinical studies are needed to determine the effectiveness and utility of nCPAP in this population. HFNC was not commonly used, and this modality requires further investigation in this population.

Includes bibliographical references (leaves 49-57)., Pelvi-ureteric junction obstruction is an important cause of congenital renal and urinary tract abnormality. It is the commonest cause of antenatally detected hydronephrosis. The increasing use of antenatal ultrasound as a screening tool for congenital abnormalities in the developing foetus has resulted in a more frequent rate of detection of foetal hydronephrosis with the likely consequence of significant anxiety among parents. This is because most of these infants with antenatally detected hydronephrosis will be subjected to frequent radiological and other investigations and there will also be concern about outcome. Knowing what postnatal investigations are necessary for any child with this condition and when to do it becomes a priority. This is because it is known that a significant percentage of children with antero-posterior (AP) diameter of 12mm or less experienced complete and spontaneous resolution of the hydronephrosis in early life. This study is a retrospective folder review of one hundred children with PUJ obstruction managed at Red Cross Children’s Hospital over a six-year period from Jan 2002 to Dec 2007.

Includes abstract., Includes bibliographical references (leaves 64-72)., Posterior urethral valve (PUV) is a congenital obstructing membrane of the male urethra. It is the commonest cause of bladder outlet obstruction in male children. PUV as a cause of obstructive uropathy is an important cause of end stage renal failure (ESRF) in children. Early detection and surgical intervention can slow down progression to ESRF.