International Pediatric Nephrology Association Research Articles

IPNA clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis nephritis.

IgA nephropathy and IgA vasculitis with nephritis, albeit rare, represent two relatively frequent glomerular conditions in childhood. Compared to adults, pediatric IgA nephropathy has a more acute presentation, most frequently with synpharyngitic macrohematuria and histologically with more intense inflammation and less intense chronic damage. Management of these conditions is controversial and supported by little high-quality evidence. The paucity of evidence is due to the disease heterogeneity, its inter-ethnic variability, and the difficulty of extrapolating data from adult studies due to the peculiarities of the condition in children. IgA vasculitis with nephritis is a kidney manifestation of a systemic disorder, typical of the pediatric age, in which both the diagnosis of kidney involvement and its management are poorly defined, and an interdisciplinary approach is crucial. Both conditions can have a profound and long-lasting impact on kidney function and the global health of affected children. The International Pediatric Nephrology Association has therefore convened a diverse international group of experts from different disciplines to provide guidance on the recommended management of these conditions in children and to establish common definitions and define priorities for future high-quality, evidence-based collaborative studies for the benefit of children.

Structures, Organization, and Delivery of Kidney Care to Children Living in Low-Resource Settings

IntroductionThere is a disparity in the availability of healthcare for children in resource-constrained countries. The International Pediatric Nephrology Association (IPNA) commissioned an initiative exploring the challenges in the care of children with kidney disease in low/middle-income countries(L/MICs) with a focus on human, diagnostic, and therapeutic resources. MethodsA survey was sent by e-mail to all members of IPNA and its affiliated regional/national societies residing in L/MICs. Data was extracted from individual responses after merging duplicate data. Descriptive analysis was done using Microsoft Excel. ResultsResponses were obtained from 245 centers across 62 countries representing 88% of the L/MIC pediatric population. Regional disparity in the availability of basic diagnostic and therapeutic resources was noted. Even when resources were available, they were not accessible or affordable in 15-20% of centers. Acute and chronic dialysis were available in 85% and 75% of centers respectively. Lack of trained nurses, pediatric-specific supplies, and high costs were barriers to providing dialysis in these regions. Kidney transplantation was available in 32 % of centers, with the cost of transplantation and lack of surgical expertise reported as barriers. About 65 % of centers reported that families with chronic disease opted to discontinue care with financial burden as the most common reason cited. ConclusionThe survey highlights the existing gaps in workforce, diagnostic, and therapeutic resources for pediatric kidney care in resource-constrained regions. We need to strengthen the healthcare workforce, address disparities in healthcare resources and funding, and advocate for equitable access to medications, and kidney replacement therapy.

Comparison of three spot proteinuria measurements for pediatric nephrotic syndrome: based on the International pediatric Nephrology Association 2022 Guidelines

Background Pediatric nephrotic syndrome (NS) requires routine proteinuria monitoring, which is costly and affects patients’ quality of life. The gold-standard 24-h urine protein (UP) measurement is challenging in children, and first-morning urine collection requires specific conditions, making it difficult in outpatient settings. Studies have reported comparability of second or random morning urine sample to the first-morning specimen. This study aimed to compare outcomes of random morning proteinuria measurements to 24-h UP and the roles of the urinary protein creatinine ratio (UPCR) and dipstick tests in pediatric NS, based on International Pediatric Nephrology Association (IPNA) 2022 Guidelines. Method Twenty-four-hour and morning urine samples were collected from 92 pediatric NS patients. These were subjected to automated analyses for 24-h UP, UPCR, and semi-automated dipstick analysis. A blinded doctor performed manual dipstick analysis. Results UPCR had a stronger correlation with 24-h UP than with automated and manual urine dipstick tests. UPCR had the highest sensitivity and specificity for predicting no remission/relapse and high sensitivity but low specificity for complete remission. The optimal UPCR cutoff for remission was 0.44 mg/mg and for no remission/relapse was 2.08 mg/mg. Automated and manual dipstick tests demonstrated limited sensitivity but high specificity and similar AUC values for remission/relapse. Conclusion UPCR was sensitive and specific for diagnosing no remission/relapse and sensitive but not specific for detecting remission. Manual and automated urine dipstick tests were comparable for remission and no remission/relapse detection. This study supports the IPNA 2022 Guidelines, as 2 mg/mg was the optimal UPCR cutoff for no remission/relapse, while for remission the optimal cutoff was 0.4 mg/mg.

#3670 THE ROLE OF CALCINEURIN INHIBITORS IN MONOGENIC PEDIATRIC STEROID RESISTANT NEPHROTIC SYNDROME: A RETROSPECTIVE, MULTICENTER STUDY

Abstract Background and Aims Response to calcineurin inhibitors (CNI) is associated with a significant improvement in long-term kidney survival of children with non-genetic steroid resistant nephrotic syndrome (SRNS). On the contrary, these agents are considered non-efficacious in monogenic SRNS and are contraindicated according to the latest International Pediatric Nephrology Association (IPNA) Clinical Practice Recommendations for SRNS. However, there is evidence suggesting that remission with CNI therapy in this subgroup of children with SRNS is possible, but no studies to date have assessed this question in a systematic way. We aimed to study the incidence of response to CNI in children with monogenic SRNS, factors predictive of remission and the effect of treatment on kidney survival. Method Retrospective cohort study of children 0–18 years with genetically confirmed SRNS treated with a CNI for at least 3 months. Demographic, clinical, genetic, biochemical, histopathologic and treatment data were collected at various time points; at clinical diagnosis, 6, 12, 24 months from CNI onset, and last available follow-up. Pathogenicity of all reported variants was assessed by a dedicated geneticist according to the American College of Medical Genetics guidelines and only patients with a pathogenic genotype were included in the analysis[1]. Patients were classified according to their response to CNI as complete, partial or non-responders based on the IPNA Clinical Practice Recommendations for SRNS[2]. Results 141 patients from 37 international paediatric nephrology centers were included in the study. At 6 months from CNI initiation and at last visit, 27.6% and 22.5%, respectively, demonstrated either complete or partial response (“at least partial response”). Median observation time between CNI initiation and last visit was 42.1 months (IQR 20.1–65.6) and was comparable between patients with no response and at least partial response (42.3 vs 41.6 months; P>0.05). No serious adverse effects mandating treatment discontinuation were reported. Children demonstrating at least partial response at 6 months had a lower risk of progression to kidney failure at last visit versus non-responders (hazard ratio [95%CI] 0.25, [0.10–0.62]; P = 0.003). Subgroup analysis of patients with a follow-up of at least 2 years revealed similar results with a hazard ratio of 0.35 (95%CI 0.14–0.91; P = 0.03). Of the various clinical, biochemical, genetic, histopathologic and treatment parameters tested in a multivariable logistic regression model only higher serum albumin at CNI onset was associated with higher likelihood of response at 6 months (odds ratio [95% CI] 1.16, [1.08–1.24]; P < 0.001). Conclusion Our findings suggest that CNI use could be considered in monogenic SRNS and that achievement of response in this setting can alter an otherwise dismal long-term kidney outcome.

Lessons learned from regional training of paediatric nephrology fellows in Africa.

Access to care for children with kidney disease is limited in less well-resourced regions of the world and paediatric nephrology (PN) workforce development with good practical skills is critical. Retrospective review of a PN training program and trainee feedback from 1999 to 2021, based at Red Cross War Memorial Children's Hospital (RCWMCH), University of Cape Town. A regionally appropriate 1-2-year training program enrolled 38 fellows with an initial 100% return rate to their country of origin. Program funding included fellowships from the International Pediatric Nephrology Association (IPNA), International Society of Nephrology (ISN), International Society of Peritoneal Dialysis (ISPD), and the African Paediatric Fellowship Program (APFP). Fellows were trained on both in- and out-patient management of infants and children with kidney disorders. "Hands-on skills" training included examination, diagnosis and management skills, practical insertion of peritoneal dialysis catheters for management of acute kidney injury and kidney biopsies. Of 16 trainees who completed > 1year of training, 14 (88%) successfully completed subspecialty exams and 9 (56%) completed a master's degree with a research component. PN fellows reported that their training was appropriate and enabled them to make a difference in their respective communities. This training program has successfully equipped African physicians with the requisite knowledge and skills to provide PN services in resource-constrained areas for children with kidney disease. The provision of funding from multiple organizations committed to paediatric kidney disease has contributed to the success of the program, along with the fellows' commitment to build PN healthcare capacity in Africa. A higher resolution version of the Graphical abstract is available as Supplementary information.

Editorial introduction

Editorial introduction

Transition of Kidney Care at 18: Challenges and Practical Solutions for India.

Health-care transition (HCT) from pediatric-centered to adult-oriented health-care setting is more than a simple transfer of care. It is a carefully planned movement specially tailored for the needs of adolescents and young adults (AYAs). Similar to other chronic diseases, the need for HCT for AYAs with kidney disease has been well established by the International Society of Nephrology (ISN) and the International Pediatric Nephrology Association (IPNA) consensus statements since 2011. However, successful HCT in India and other low- and middle-income countries (LMICs) has been limited. Undertaking the HCT program in India requires involvement of many stakeholders, that is, AYAs, parents/caregivers, health-care providers, and the health-care system. In this article, we discuss the need for HCT, the challenges faced during the transition, and the recommended models for HCT in kidney care. We focus on the unique challenges faced in India and conclude with practical suggestions to implement HCT in our setting.

Steroid-sensitive nephrotic syndrome in children.

A group of experts from the International Pediatric Nephrology Association (IPNA) has recently updated recommendations for steroid-resistant and steroid-sensitive nephrotic syndrome (SRNS and SSNS) in children [1, 2]. SSNS is now defined as idiopathic nephrotic syndrome [spot urinary protein:creatinine ratio (UPCR) ≥200 mg/mmol and albuminemia <30 g/l] responsive to 4 weeks of corticosteroid therapy with or without a confirmation period of 2 additional weeks (late SSNS). We present a summary of the new definitions and recommendations for diagnosis and management of SSNS in children (Fig. 1). ... ... ... ... ... There is no randomized controlled trial comparing a 4 + 4 to a 6 + 6-week course of treatment for the initial episode of SSNS. Therefore, both are recommended in line with Kidney Disease: Improving Global Outcomes guidelines [2, 4, 5].

Рекомендации Международной ассоциации детской нефрологии по диагностике и ведению детей со стероидорезистентным нефротическим синдромом (СРНС)

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Overcoming barriers and building a strong peritoneal dialysis programme - Experience from three South Asian countries.

The development of peritoneal dialysis (PD) programmes in lower-resource countries is challenging. This article describes the learning points of establishing PD programmes in three countries in South Asia (Nepal, Sri Lanka and Pakistan). The key barriers identified were government support (financial), maintaining stable supply of PD fluids, lack of nephrologist and nurse expertise, nephrology community bias against PD, lack of nephrology trainee awareness and exposure to this modality. To overcome these barriers, a well-trained PD lead nephrologist (PD champion) is needed, who can advocate for this modality at government, professional and community levels. Ongoing educational programmes for doctors, nurses and patients are needed to sustain the PD programmes. Support from well-established PD centres and international organisations (International Society of Peritoneal Dialysis (ISPD), International Society of Nephrology (ISN), International Pediatric Nephrology Association (IPNA) are essential.

Sixth Congresses of the Macedonian Society for Nephrology, Dialysis, Transplantation and Artificial Organs (MSNDTAO) - Stimulus for the Development of Nephrology in N. Macedonia.

The Macedonian Society for Nephrology, Dialysis, Transplantation and Artificial Organs (MSNDTAO) was established on September 1, 1992 in order to stimulate the development of nephrology and to unite experts from all professions interested in the research and treatment of kidney patients. Momir H. Polenakovic was elected President of MSNDTAO.The First congress of MSNTAO was held in Ohrid from October, 7 to 10, 1993 in hotel "Desaret" under the auspices of the first President of R. Macedonia, Kiro Gligorov. During the Congress, the Balkan Cities Association of Nephrology, Dialysis, Transplantation and Artificial Organs (BANTAO) was formed on October 9, 1993. The idea of cooperation with the Department of Nephrology from the Ege University, Izmir, R. Turkey was promoted. Some of the presented papers were published in the Proceedings "Nephrology '93" as a supplement to the Macedonian medical review. The "Doctrine for performing nephrological activity in the Republic of Macedonia" was also promoted.The Second congress of MSNDTAO was held in Struga, R. Macedonia from September, 6 to 10, 1997 together with the Second BANTAO Congress. Selected papers were printed in the Macedonian Medical Review.The Third congress of MSNDTAO was held from May, 30 to June 2, 2001 in Skopje, at the Macedonian Academy of Sciences and Arts (MASA). Proceedings of papers was also published.In 2011 M. H. Polenakovic was elected Honorary President of MSNDTAO for life, and Goce Spasovski was elected the current President of MSNDTAO.The Fourth congress of MSNDTAO was held in Ohrid from June, 21 to 24, 2012 together with the Fourth Meeting of the South-Eastern European Pediatric Nephrology Working Group (SEEPNWG).The Fifth congress of MSNDTAO was held from June, 9 to 12, 2016 in Skopje, at MASA together with the 6th Southeastern European Pediatric Nephrology Working Group (SEPNWG) Meeting and IPNA (International Pediatric Nephrology Association) Teaching Course. The VIII Macedonian-Croatian Nephrology Days were also held during the Congress.The Sixth congress of MSNDTAO was held from September, 26 to 29, 2019 along with the 15th BANTAO Congress, in Skopje, at MASA. The Congress also marked the 80th anniversary of the birth of M. H. Polenakovic.Apart from the participants from R. Macedonia, the congress was attended by renowned nephrologists from Europe and the world.A novelty of the last congresses was the organization of 9 CME (Continuing Medical Education) courses with famous lecturers. CME courses supported by the ERA-EDTA and / or the ISN were as follows: 2005 - Ohrid (BANTAO), 2006 - Skopje (MSNDTAO), 2009 - Skopje (MSNDTAO), 2010 - Skopje (ERA-EDTA - ESAO), 2011 Skopje (ERA-EDTA - EUTox group), 2012 - Ohrid (MSNDTAO), 2014 -Skopje (MSNDTAO), 2016 - Skopje (MSNDTAO), 2018 - Skopje (MSNDTAO - ERA-EDTA & ISN), 2019 - Skopje (BANTAO - ISN & ERA-EDTA).In addition to the congresses, MSNDTAO has held meetings with nephrological associations from the neighboring countries as Croatia, Serbia, Albania and Kosovo.The congresses of MSNDTAO contributed to the introduction and education of doctors in the diagnosis and treatment of kidney patients. They were a strong stimulus for the development of nephrology in R. Macedonia and beyond.

Pediatric kidney transplantation in China: an analysis from the IPNA Global Kidney Replacement Therapy Registry.

The International Pediatric Nephrology Association (IPNA) Global Kidney Replacement Therapy (KRT) Registry was established to evaluate the incidence and outcomes of kidney replacement therapy (dialysis and transplantation) provided to children worldwide. Analysis of registry data for separate regions is feasible. Three centers located in Shanghai, Guangzhou, and Zhengzhou, which have the greatest number of pediatric kidney transplantation cases in China, participated in this analysis of transplant data. Data were registered by each center for patients under the age of 19years who received a single-organ kidney transplant for the first time between 2011 and 2018. In total, 415 patients (59.8% male) aged 1.4-18.7 (median 12.1) years were followed for 0.3-97.1 (median 27.7) months. The number of kidney transplants increased from a total of 129 during 2011-2014 to 286 cases during 2015-2018. 85.8% of patients received the transplanted kidney from a pediatric (age < 19years) donor, and deceased donors accounted for 94% of all donors. 8.0% of grafts were lost. One and 5-year patient survival rates were 97.6% and 95.5%, respectively. The major cause of death was infection (7/14). Similar graft and patient survival rates were observed for organs from pediatric and adult donors in 6-11 and 12-18year recipient age groups, whereas recipients < 6years showed inferior patient and graft survival. Pediatric kidney transplantation shows favorable short-term and medium-term outcomes in China. Our experience supports use of pediatric donors in pediatric kidney transplantation, but attention directed to the outcome of recipients aged under 6 is necessary. Graphical abstract.

A need-adapted transition program after pediatric kidney transplantation

AbstractA successful transition of renal transplant recipients from pediatric to adult care requires a structured, need-adapted and multidisciplinary approach to preserve renal graft function during this critical period of life. In this article we present our clinical protocol for transition from pediatric to adult care, which we developed on the basis of the International Society of Nephrology (ISN)/International Pediatric Nephrology Association (IPNA) consensus guidelines influenced by our own experience. This transition program was established in our center in July 2017. The entire transition process is structured and accompanied by a transition key worker (social worker). From 12 years of age we train pediatric renal transplant recipients in medical knowledge, self-management skills and networking with self-help groups. The training is adapted to the individual patient‘s intellectual ability, lasts about 10 years and takes place with increasing intensity. Repeatedly we perform standardized informational interviews and check patient’s knowledge of transplant-related topics. Psychosocial and educational issues are evaluated concomitantly. The actual transfer takes place in a pediatric-adult-transition clinic. Relevant medical and psychosocial aspects are discussed and the future treatment regimen is established. The date of transfer is adapted to the individual patient’s need; it varies between 18 and 24 years of age. In periods of increased risk for non-adherence the transfer is postponed to intensify the efforts for training and assistance. After transfer a standardized evaluation of each individual patient takes place focusing on medical and psychosocial issues and on satisfaction with the transition process. Collection of these data is still in progress and will be analyzed systematically at a later stage in order to evaluate the impact of this new transition program on the stability of transplant function. That analysis might serve as a basis for negotiations about refunding with health insurance companies.

Survey on Management of Transition and Transfer From Pediatric- to Adult-based Care in Pediatric Kidney Transplant Recipients in Europe.

Transition from pediatric- to adult-based healthcare is a challenging period and bears a high risk of medication nonadherence and transplant loss in adolescents and young adults after kidney transplantation. Yet, it remains unclear how the 2011 International Society of Nephrology (ISN)/International Pediatric Nephrology Association (IPNA) guidelines on transition are implemented in practice and which healthcare transition modalities are currently used in Europe. We performed an online survey inviting all members of the European Society of Paediatric Nephrology mailing list to participate. Adherence to ISN/IPNA guidelines was scored with a maximum of 15 points. Thirty-nine centers from 24 countries accounting for approximately 2500 children after kidney transplantation participated in the survey. At 3 centers, patients remained under pediatric care for their whole life, and 5 centers did not use any transition procedure. From the remaining 31 centers, 82% confirmed the existence of at least 1 unwritten transition procedure. None of these centers used IT or social media for patient training in transition. Specialized transition clinics are held at 15 of 31 centers for the patients. Most patients were transferred at 16 to 21 years of age. Transfer age was subject to regulation at 20 of 36 centers. Median score of guidelines adherence was 10 (range, 0-14). The adherence score was significantly correlated with gross national income (r2 = 0.631, P < 0.0001). The 2011 ISN/IPNA guidelines on transition are implemented insufficiently in European pediatric nephrology centers. Therefore, further development is needed, and the use of information technology and social media should be promoted.

Editorial introductions

Editorial introductions

Worldwide view of nephropathic cystinosis: results from a survey from 30 countries

BackgroundNephropathic cystinosis is a rare inherited metabolic disorder leading to progressive renal failure and extra-renal comorbidity. The prognosis relies on early adherence to cysteamine treatment and symptomatic therapies. Developing nations [DiN] experience many challenges for management of cystinosis. The aim of this study was to assess the management characteristics in DiN compared with developed nations [DeN].MethodsA questionnaire was sent between April 2010 and May 2011 to 87 members of the International Pediatric Nephrology Association, in 50 countries.ResultsA total of 213 patients were included from 41 centres in 30 nations (109 from 17 DiN and 104 from 13 DeN). 7% of DiN patients died at a median age of 5 years whereas no death was observed in DeN. DiN patients were older at the time of diagnosis. In DiN, leukocyte cystine measurement was only available in selected cases for diagnosis but never for continuous monitoring. More patients had reached end-stage renal disease in DiN (53.2 vs. 37.9%, p = 0.03), within a shorter time of evolution (8 vs. 10 yrs., p = 0.0008). The earlier the cysteamine treatment, the better the renal outcome, since the median renal survival increased up to 16.1 [12.5−/] yrs. in patients from DeN treated before the age of 2.5 years of age (p = 0.0001). However, the renal survival was not statistically different between DeN and DiN when patients initiated cysteamine after 2.5 years of age. The number of transplantations and the time from onset of ESRD to transplantation were not different in DeN and DiN. More patients were kept under maintenance dialysis in DiN (26% vs.19%, p = 0.02); 79% of patients from DiN vs. 45% in DeN underwent peritoneal dialysis.ConclusionsMajor discrepancies between DiN and DeN in the management of nephropathic cystinosis remain a current concern for many patients living in countries with limited financial resources.

Enuresis: practical guidelines for primary care.

Although the evidence is relatively weak, expert opinion recommends accurate diagnosis of enuresis and the use of enuresis alarms and desmopressin, in addition to behavioural approaches Worldwide, around 10% of 6–7-year-olds suffer from enuresis. Enuresis is therefore highly prevalent, but its impact is often underestimated. Training for family doctors rarely includes specific guidance on enuresis, and the default approach is often to wait for spontaneous resolution. Despite comprehensive enuresis guidelines in specialist journals for secondary and tertiary care, versions for use in primary care are scarce (for example, https://pathways.nice.org.uk/pathways/bedwetting-in-children-and-young-people), and often confusing because of outdated terminology. This article provides practical guidelines and tools to manage enuresis in primary care in children of ≥5 years based on the guidelines of the International Children’s Continence Society (ICCS), following the new ICCS standardisation and subtyping of patients.1–3 The document was developed by the steering committee of World Bedwetting Day 2017 representing the American Academy of Pediatrics (AAP), the North American Paediatric Urology Societies, the European Society for Paediatric Nephrology (ESPN), the European Society for Paediatric Urology (ESPU), the Asia Pacific Association of Paediatric Urology (APAPU), the Sociedad Iberoamericana de Urologia Paediatrica (SIUP), the International Pediatric Nephrology Association (IPNA), and the ICCS. It should be highlighted that this guideline differs to some extent from previous guidelines, based on historical studies, in which there was no differentiation between NMNE and MNE, contrary to current understanding. …

Renal Support for Acute Kidney Injury in the Developing World

There is wide variation in the management of acute kidney injury (AKI) and the practice of renal replacement therapy (RRT) around the world. Clinicians in developing countries face additional challenges due to limited resources, reduced availability of trained staff and equipment, cultural and socioeconomic aspects, and administrative and governmental barriers. In this article, we report the consensus recommendations from the 18th Acute Dialysis Quality Initiative conference in Hyderabad, India. We provide the minimal requirements for provision of acute RRT in developing countries, including patient selection, choice of RRT modality and monitoring, transition, and termination of acute RRT. We also discuss areas of uncertainty and propose themes for future research. These recommendations can serve as a foundation for clinicians to implement renal support for AKI in low resource settings.

Editorial introductions

Editorial introductions

17th Congress of the International Pediatric Nephrology Association September 20-24, 2016

17th Congress of the International Pediatric Nephrology Association September 20-24, 2016