IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.

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.

Renal replacement therapy for children throughout the world: the need for a global registry.

BACKGROUND: To describe the factors affecting the incidence of renal replacement therapy (RRT) among children, information from RRT registries is required. We aimed to give an overview of existing pediatric RRT registries worldwide, identify regions with a need to commence or increase data collection on pediatric RRT, and provide a rationale for developing a global RRT registry. METHODS: A survey assessing pediatric RRT registry status was sent to International Pediatric Nephrology Associateion (IPNA) members in 127 countries in January 2016. The survey was complemented by a systematic literature search for active pediatric RRT registries. RESULTS: Complete survey responses were retrieved from 94 countries (representing 86.2% of the world childhood population), with 84 (81.2%) having the means to provide RRT to children, given that there are no other limitations such as financial, social, or religious restraints. Fifty-one (35.3%) countries had national registries for both dialysis and transplantation, nine (30.0%) either had a dialysis or a transplant registry, six participated in international registries only (2.7%), and in 18 (13.2%), children on RRT were not followed in any registry. The search identified 92 pediatric RRT registries, primarily national registries located in Europe, North America, and Asia. CONCLUSIONS: Although pediatric RRT can be provided in 84 countries representing 81.2% of the world's childhood population, national pediatric RRT registries are unavailable in many countries. To improve knowledge about the incidence and outcomes of pediatric RRT around the globe, an international population-based pediatric RRT registry has recently been initiated.

Diagnosis and treatment of the hemolytic uremic syndrome disease spectrum in developing regions.

There has been rapid progress in the understanding of the pathophysiology of the hemolytic uremic syndrome (HUS) disease spectrum; thus, complex diagnostic and therapeutic requirements have emerged in parallel. Current recommendations for diagnosis and therapy were rapidly adapted from the prior skilled scientific groundwork. However, such recommendations can be realized only when highly specialized laboratories and sufficient financial resources are available. Thus, many recommendations are not feasible for patients living and working in developing countries. More than one-third of the world's population has no access to essential drugs and more than half of this group lives in the poorest regions of Africa and Asia. From this perspective, distinct initial diagnostic and therapeutic recommendations, as well as international cooperations are needed to complete proper diagnostic work-ups in a stringent and cost-efficient manner and to enable patients to be adequately treated with available resources. However, while costs for complement-targeted drugs remain tremendously high, state-of-the-art treatment options remain unavailable for the vast majority of patients in developing areas.

Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is an important cause of acute kidney injury in children. It is primarily caused by dysregulation of the complement alternative pathway due to genetic mutations, mainly in complement factor H genes, or due to anti-factor H autoantibodies (anti-FH), leading to uncontrolled overactivation of the complement system. Early diagnosis and treatment of autoimmune HUS (AI-HUS) is essential and leads to a favorable outcome. METHODS: Fifty pediatric HUS patients and 50 age- and sex-matched controls were included in the study. Patients were subjected to full history taking, clinical examination, and laboratory testing. All candidates were subjected to an assessment of anti-FH in serum by a homemade enzyme-linked immunosorbent assay technique. RESULTS: A high frequency of serum anti-FH was detected in our aHUS patients. The disease onset of AI-HUS was mainly observed in March and April, with significantly higher rates in school-aged males. All patients who started immunosuppressives early together with plasmapheresis upon detection of their anti-FH had complete renal function recovery. CONCLUSION: The high frequency of AI-HUS revealed in Egyptian HUS children in our study highlights the importance of implementing anti-FH testing in Egypt to provide early recognition for immediate proper management, including early immunosuppressive therapy, and hence improving patient outcomes.

Causes of chronic kidney disease in Egyptian children.

There are very few published reports on the causes of chronic kidney disease (CKD) in Egyptian children. We reviewed the records of 1018 (males 56.7%, age ranged from 1 to 19 years) Egyptian patients suffering from CKD and followed-up at the pediatric nephrology units (outpatient clinics and dialysis units) of 11 universities over a period of two years. The mean of the estimated glomerular filtration rate was 12.5 mL/min/1.73 m 2 . Children with CKD stage I and stage II comprised 4.4% of the studied group, while those with stage III, IV and V comprised 19.7%, 18.3% and 57.6%, respectively. The most common single cause of CKD was obstructive uropathy (21.7%), followed by primary glomerulonephritis (15.3%), reflux/urinary tract infection (14.6%), aplasia/hypoplasia (9.8%) and familial/metabolic diseases (6.8%); unknown causes accounted for 20.6% of the cases. Of the 587 patients who had reached end-stage renal disease, 93.5% was treated with hemodialysis and only 6.5% were treated with peritoneal dialysis.