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Commentary on “Pediatric Idiopathic Steroid-sensitive Nephrotic Syndrome Diagnosis and Therapy - Short version of the updated German Best Practice Guideline (S2e)”
Rasmus Ehren, Marcus R. Benz, Paul T. Brinkkötter, Jörg Dötsch, Wolfgang R. Eberl, Jutta Gellermann, Peter F. Hoyer, Isabelle Jordans, Clemens Kamrath, Markus J. Kemper, Kay Latta, Dominik Müller, Jun Oh, Burkhard Tönshoff, Stefanie Weber & Lutz T. Weber on behalf of the German Society for Pediatric Nephrology
doi : 10.1007/s00467-021-05136-2
Pediatric Nephrology volume 36, pages2961–2966 (2021)
Editorial with respect to “Corticosteroids to prevent kidney scarring in children with a febrile urinary tract infection—a randomized trial”
Vasikar Murugapoopathy & Indra R. Gupta
doi : 10.1007/s00467-021-05051-6
Pediatric Nephrology volume 36, pages2967–2969 (2021)
Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy —short version of the updated German best practice guideline (S2e) — AWMF register no. 166-001, 6/2020
Rasmus Ehren, Marcus R. Benz, Paul T. Brinkkötter, Jörg Dötsch, Wolfgang R. Eberl, Jutta Gellermann, Peter F. Hoyer, Isabelle Jordans, Clemens Kamrath, Markus J. Kemper, Kay Latta, Dominik Müller, Jun Oh, Burkhard Tönshoff, Stefanie Weber & Lutz T. Weber on behalf of the German Society for Pediatric Nephrology
doi : 10.1007/s00467-021-05135-3
Pediatric Nephrology volume 36, pages2971–2985 (2021)
Idiopathic nephrotic syndrome is the most frequent glomerular disease in children in most parts of the world. Children with steroid-sensitive nephrotic syndrome (SSNS) generally have a good prognosis regarding the maintenance of normal kidney function even in the case of frequent relapses. The course of SSNS is often complicated by a high rate of relapses and the associated side effects of repeated glucocorticoid (steroid) therapy. The following recommendations for the treatment of SSNS are based on the comprehensive consideration of published evidence by a working group of the German Society for Pediatric Nephrology (GPN) based on the systematic Cochrane reviews on SSNS and the guidelines of the KDIGO working group (Kidney Disease - Improving Global Outcomes).
Autism spectrum disorder and kidney disease
Joanna Clothier & Michael Absoud
doi : 10.1007/s00467-020-04875-y
Pediatric Nephrology volume 36, pages2987–2995 (2021)
Neurodevelopmental impairments have been recognised as a major association of paediatric kidney disease and bladder dysfunction, presenting challenges to clinicians and families to provide reasonable adjustments in order to allow access to investigations and treatments. Autism spectrum disorder (ASD) is a common neurodevelopmental disorder characterised by impairments in social interaction/communication and repetitive sensory-motor behaviours. Mental health, learning and physical co-morbidities are common. There is emerging evidence that ASD and kidney disease have some overlaps with genetic copy number variants and environmental factors contributing to shared pathogenesis. Prevalence rates of ASD in kidney disease are currently not known. A high index of suspicion of underlying ASD is required when a young person presents with communication difficulties, anxiety or behaviour that challenges, which should then trigger referral for a neurodevelopmental and behavioural assessment. We discuss practical approaches for providing care, which include understanding methods of communication and sensory, behavioural and environmental adaptations.
Acute kidney injury and aging
Monica Chang-Panesso
doi : 10.1007/s00467-020-04849-0
Pediatric Nephrology volume 36, pages2997–3006 (2021)
Our aging population is growing and developing treatments for age-related diseases such as Alzheimer’s and Parkinson’s disease has taken on an increasing urgency and is accompanied by high public awareness. The already high and rising incidence of acute kidney injury (AKI) in the elderly, however, has received relatively little attention despite the potentially fatal outcomes associated with an episode of AKI in this age group. When discussing AKI and aging, one should consider two aspects: first, elderly patients have an increased susceptibility to an AKI episode, and second, they have decreased kidney repair after AKI given the high incidence of progression to chronic kidney disease (CKD). It is unclear if the same factors that drive the increased susceptibility to AKI could be playing a role in the decreased repair capacity or if they are totally different and unrelated. This review will examine current knowledge on the risk factors for the increased susceptibility to AKI in the elderly and will also explore potential aspects that might contribute to a decreased kidney repair response in this age group.
Fibroblast growth factor 23—Klotho and hypertension: experimental and clinical mechanisms
Michael Freundlich, Gerardo Gamba & Bernardo Rodriguez-Iturbe
doi : 10.1007/s00467-020-04843-6
Pediatric Nephrology volume 36, pages3007–3022 (2021)
Hypertension (HTN) and chronic kidney disease (CKD) are increasingly recognized in pediatric patients and represent risk factors for cardiovascular morbidity and mortality later in life. In CKD, enhanced tubular sodium reabsorption is a leading cause of HTN due to augmented extracellular fluid volume expansion. The renin-angiotensin-aldosterone system (RAAS) upregulates various tubular sodium cotransporters that are also targets of the hormone fibroblast growth factor 23 (FGF23) and its co-receptor Klotho. FGF23 inhibits the activation of 1,25-dihydroxyvitamin D that is a potent suppressor of renin biosynthesis. Here we review the complex interactions and disturbances of the FGF23–Klotho axis, vitamin D, and the RAAS relevant to blood pressure regulation and discuss the therapeutic strategies aimed at mitigating their pathophysiologic contributions to HTN.
Preventing tuberculosis in paediatric kidney transplant recipients: is there a role for BCG immunisation pre-transplantation in low tuberculosis incidence countries?
Alasdair Bamford, Garth Dixon, Nigel Klein, Stephen D. Marks, Nicole Ritz, Steven B. Welch & Marc Tebruegge
doi : 10.1007/s00467-020-04844-5
Pediatric Nephrology volume 36, pages3023–3031 (2021)
The risk of tuberculosis (TB) disease is increased in children with chronic kidney disease (CKD), even higher in stage 5 CKD/kidney failure and especially high after kidney transplantation due to immunosuppression. TB disease may follow recent primary infection, or result from reactivation of latent infection. Reactivation is more common in adults, while progression following primary infection makes up a greater proportion of disease in children. Recommendations for preventing TB disease in some low TB incidence countries have previously included offering Bacillus Calmette-Guérin (BCG) vaccine to all children listed for kidney transplant if they had not received this as part of previous national immunisation programmes. Based on the available evidence, we recommend modifying this practice, focusing instead on awareness of risk factors for TB exposure, infection and disease and the use of appropriate testing strategies to identify and treat TB infection and disease.
A systematic review of urine biomarkers in children with IgA vasculitis nephritis
Chloe E. C. Williams, Aileen Toner, Rachael D. Wright & Louise Oni
doi : 10.1007/s00467-021-05107-7
Pediatric Nephrology volume 36, pages3033–3044 (2021)
Nephritis is a recognised complication of IgA vasculitis (IgAV, Henoch-Schönlein purpura) contributing to 1–2% of all chronic kidney disease (CKD) stage 5. Improved understanding may reduce irreversible damage in IgAV nephritis (IgAV-N).
Familial hypomagnesemia with hypercalciuria and nephrocalcinosis
Mònica Vall-Palomar, Leire Madariaga & Gema Ariceta
doi : 10.1007/s00467-021-04968-2
Pediatric Nephrology volume 36, pages3045–3055 (2021)
Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC; OMIM 248250) is a rare autosomal recessive kidney disease caused by mutations in the CLDN16 or CLDN19 genes encoding the proteins claudin-16 and claudin-19, respectively. These are involved in paracellular magnesium and calcium transport in the thick ascending limb of Henle’s loop and account for most of the magnesium reabsorption in the tubules. FHHNC is characterized by hypomagnesaemia, hypercalciuria, and nephrocalcinosis, and progresses to kidney failure, requiring dialysis and kidney transplantation mainly during the second to third decades of life. Patients carrying CLDN19 mutations frequently exhibit associated congenital ocular defects leading to variable visual impairment. Despite this severe clinical course, phenotype variability even among siblings has been described in this disease, suggesting unidentified epigenetic mechanisms or other genetic or environmental modifiers. Currently, there is no specific therapy for FHHNC. Supportive treatment with high fluid intake and dietary restrictions, as well as magnesium salts, thiazides, and citrate, are commonly used in an attempt to retard the progression of kidney failure. A kidney transplant remains the only curative option for kidney failure in these patients. In this review, we summarize the current knowledge about FHHNC and discuss the remaining open questions about this disorder.
Non-immunosuppressive therapies for childhood IgA nephropathy
Yuko Shima, Koichi Nakanishi & Norishige Yoshikawa
doi : 10.1007/s00467-021-04954-8
Pediatric Nephrology volume 36, pages3057–3065 (2021)
IgA nephropathy (IgAN) is the most common chronic primary glomerulonephritis in both children and adults, and 20–30% of patients with persistent hematuria/proteinuria progress to kidney failure within 20 years. In Japan, most cases of childhood IgAN are detected by school screening programs during the early onset of the disease when hematuria/proteinuria are asymptomatic and kidney function is normal. Therefore, it is possible to follow the detailed clinical course and appropriate therapeutic interventions from early onset of the disease. Data on non-immunosuppressive therapies for children with IgAN are highly limited. The Japanese Pediatric IgA Nephropathy Treatment Study Group was organized in 1989 to conduct clinical trials and accumulate data on treatments for childhood IgAN. In this review, we focus on non-immunosuppressive therapies, notably with renin-angiotensin-aldosterone system (RAAS) inhibitors for childhood IgAN and related clinical trials conducted primarily in Japan. We also describe the anti-inflammatory and antiproteinuric effects of RAAS inhibitors in IgAN, differences in treatment regimens because of the acute and active pathological features of childhood IgAN, adverse events of RAAS inhibitors, other non-immunosuppressive treatment options, and future directions.
Oral health in children with chronic kidney disease
Elizabeth Velan & Barbara Sheller
doi : 10.1007/s00467-020-04913-9
Pediatric Nephrology volume 36, pages3067–3075 (2021)
The review summarizes the current understanding of dental health in children with chronic kidney disease (CKD). Oral conditions associated with CKD and its medical and surgical management have been described in cohort studies. Children with CKD may present with severe developmental defects of enamel (DDE) including discoloration, pitting, and reduced hardness leading to extensive tooth wear with normal function. The alkaline oral pH resulting from the uremia of CKD inhibits cariogenic bacteria, reduces dental caries risk, and increases accumulation of dental calculus. The malnutrition, acidosis, growth hormone resistance, anemia, and renal osteodystrophy in CKD provide multiple mechanisms for abnormal craniofacial growth and delayed tooth eruption. Following successful kidney transplant, caries risk increases due to normalization of oral pH in the presence of DDE; optimized diet and oral hygiene become critical in caries control. Post-transplant medications including cyclosporine A and calcium channel blockers may cause gingival overgrowth which in severe cases requires gingival surgery to allow tooth eruption, improve appearance, or permit orthodontic treatment. Immune suppression with sirolimus or everolimus may cause severe debilitating oral ulcerations. Long-term immune suppression increases the risk for development of oral candidiasis and oral cancers. Dental examinations and treatment are recommended for children with all stages of CKD to mitigate adverse oral outcomes of the disease and its management.
Emerging monitoring technologies in kidney transplantation
Abdulla Ehlayel, K’joy J. A. Simms & Isa F. Ashoor
doi : 10.1007/s00467-021-04929-9
Pediatric Nephrology volume 36, pages3077–3087 (2021)
Non-invasive technologies to monitor kidney allograft health utilizing high-throughput assays of blood and urine specimens are emerging out of the research realm and slowly becoming part of everyday clinical practice. HLA epitope analysis and eplet mismatch score determination promise a more refined approach to the pre-transplant recipient–donor HLA matching that may lead to reduced rejection risk. High-resolution HLA typing and multiplex single antigen bead assays are identifying potential new offending HLA antibody subtypes. There is increasing recognition of the deleterious role non-HLA antibodies play in post-transplant outcomes. Donor-derived cell-free DNA detected by next-generation sequencing is a promising biomarker for kidney transplant rejection. Multi-omics techniques are shedding light on discrete genomic, transcriptomic, proteomic, and metabolomic signatures that correlate with and predict allograft outcomes. Over the next decade, a comprehensive approach to optimize kidney matching and monitor transplant recipients for acute and chronic graft dysfunction will likely involve a combination of those emerging technologies summarized in this review.
Timing of procedural interventions in childhood renovascular hypertension
Zubin J. Modi & David B. Kershaw
doi : 10.1007/s00467-021-05152-2
Pediatric Nephrology volume 36, pages3089–3096 (2021)
Renovascular hypertension (RVHTN) is a rare, often complex condition due to multiple etiologies including congenital stenoses, vasculitides, and fibromuscular dysplasia. Among children with RVHTN who require multiple and escalating medications to control blood pressure, the optimal timing of a procedural intervention involves a balance of numerous factors.
An unusual disease mimicking congenital nephrotic syndrome: Questions
Nurdan Y?ld?z, Mehtap Sak, Sabahat Inan?r, Bilge Sahin Akkelle & Harika Alpay
doi : 10.1007/s00467-021-05010-1
Pediatric Nephrology volume 36, pages3097–3098 (2021)
An unusual disease mimicking congenital nephrotic syndrome: Answers
Nurdan Y?ld?z, Mehtap Sak, Sabahat Inan?r, Bilge Sahin Akkelle & Harika Alpay
doi : 10.1007/s00467-021-05013-y
Pediatric Nephrology volume 36, pages3099–3102 (2021)
Hypocalcemia not related to chronic kidney disease: Questions
Cengiz Zeybek, Onur Akin & Ahmet Bolat
doi : 10.1007/s00467-021-05029-4
Pediatric Nephrology volume 36, pages3103–3104 (2021)
Hypocalcemia not related to chronic kidney disease: Answers
Cengiz Zeybek, Onur Akin & Ahmet Bolat
doi : 10.1007/s00467-021-05036-5
Pediatric Nephrology volume 36, pages3105–3107 (2021)
A 17-year-old boy with progressive abdominal pain: Questions
Olivia Gilbert, Mingshan Lai, Jessica Zagory, Randall Craver, Amanda Messer & Isa F. Ashoor
doi : 10.1007/s00467-021-05030-x
Pediatric Nephrology volume 36, pages3109–3110 (2021)
A 17-year-old boy with progressive abdominal pain: Answers
Olivia Gilbert, Mingshan Lai, Jessica Zagory, Randall Craver, Amanda Messer & Isa F. Ashoor
doi : 10.1007/s00467-021-05037-4
Pediatric Nephrology volume 36, pages3111–3113 (2021)
A 5-year-old girl with bony deformities and disproportionate short stature: Questions
Rangaswamy Darshan, Sriram Krishnamurthy, Bobbity Deepthi, Pediredla Karunakar, Aakash Chandran Chidambaram & Arumugom Archana
doi : 10.1007/s00467-021-05031-w
Pediatric Nephrology volume 36, pages3115–3116 (2021)
A 5-year-old girl with bony deformities and disproportionate short stature: Answers
Rangaswamy Darshan, Sriram Krishnamurthy, Bobbity Deepthi, Pediredla Karunakar, Aakash Chandran Chidambaram & Arumugom Archana
doi : 10.1007/s00467-021-05038-3
Pediatric Nephrology volume 36, pages3117–3121 (2021)
Long-term complications of systemic oxalosis in children—a retrospective single-center cohort study
Efrat Ben-Shalom, Ruth Cytter-Kuint, Choni Rinat, Rachel Becker-Cohen, Shimrit Tzvi-Behr, Jenny Goichberg, Vardit Peles & Yaacov Frishberg
doi : 10.1007/s00467-021-05002-1
Pediatric Nephrology volume 36, pages3123–3132 (2021)
Systemic oxalosis is a severe complication seen in primary hyperoxaluria type I patients with kidney failure. Deposition of insoluble calcium oxalate crystals in multiple organs leads to significant morbidity and mortality.
Molecular aspects and long-term outcome of patients with primary distal renal tubular acidosis
Sara Gómez-Conde, Alejandro García-Castaño, Mireia Aguirre, María Herrero, Leire Gondra, Nélida García-Pérez, Paula García-Ledesma, Luis Martín-Penagos, Cecilia Dall’Anese, Gema Ariceta, Luis Castaño & Leire Madariaga
doi : 10.1007/s00467-021-05066-z
Pediatric Nephrology volume 36, pages3133–3142 (2021)
Primary distal renal tubular acidosis (dRTA) is a rare genetic disorder caused by impaired distal mechanisms of urinary acidification. Most cases are secondary to pathogenic variants in ATP6V0A4, ATP6V1B1, and SLC4A1 genes, which encode transporters regulating acid-base balance in the collecting duct.
Low-dose versus conventional-dose prednisolone for nephrotic syndrome relapses: a randomized controlled non-inferiority trial
Seenam Sheikh, Kirtisudha Mishra & Manish Kumar
doi : 10.1007/s00467-021-05048-1
Pediatric Nephrology volume 36, pages3143–3150 (2021)
Reduction of steroid exposure in relapses of steroid-sensitive nephrotic syndrome (SSNS) is under-researched.
Congenital anomalies of the kidney and urinary tract in a cohort of 280 consecutive patients with Hirschsprung disease
Alessio Pini Prato, Rossella Arnoldi, Ilaria Falconi, Maria Pia Dusio, Isabella Ceccherini, Augusta Tentori, Enrico Felici & Paolo Nozza
doi : 10.1007/s00467-021-05061-4
Pediatric Nephrology volume 36, pages3151–3158 (2021)
Congenital anomalies of the kidney and urinary tract (CAKUT) have been underestimated in Hirschsprung disease (HSCR). This paper aims at reporting results of patients with HSCR who underwent kidney and urinary tract assessment.
The spectrum of kidney function alterations in adolescents with a solitary functioning kidney
Mathilde Grapin, François Gaillard, Nathalie Biebuyck, Melissa Ould-Rabah, Carole Hennequin, Romain Berthaud, Guillaume Dorval, Thomas Blanc, Maryvonne Hourmant, Nassim Kamar, Lionel Rostaing, Lionel Couzi, Nicolas Garcelon, Dominique Prié, Olivia Boyer & Frank Bienaimé
doi : 10.1007/s00467-021-05074-z
Pediatric Nephrology volume 36, pages3159–3168 (2021)
A precise assessment of glomerular filtration rate is key to delineate the care of children with a solitary functioning kidney (SFK). Data regarding measured GFR (mGFR) in this population is restricted to a single study of 77 individuals, which suggested that a GFR estimation (eGFR) method based on creatinine and cystatin C (eGFR-CKiD2) performed better than Schwartz’s equation (eGFR-Schwartz).
Clinical characteristics and long-term outcomes of endovascular treatment of renal artery fibromuscular dysplasia with branch lesions
Kun Li, Mingzhe Cui, Kewei Zhang, Kai Liang & Shuiting Zhai
doi : 10.1007/s00467-021-05067-y
Pediatric Nephrology volume 36, pages3169–3180 (2021)
Renal artery fibromuscular dysplasia (FMD) can cause arterial stenosis, dissection, and aneurysm of renal arteries. This study aimed to analyze the clinical characteristics and evaluate the long-term outcomes of renal branch artery FMD in children and adults.
Factors associated with the absence of pharmacological treatment for common modifiable complications in children with chronic kidney disease
Yunwen Xu, Derek K Ng, Susan L Furth, Bradley A Warady & Mark M Mitsnefes
doi : 10.1007/s00467-021-05087-8
Pediatric Nephrology volume 36, pages3181–3189 (2021)
Chronic kidney disease (CKD) is associated with many comorbidities requiring complex management. We described treatment patterns for common modifiable CKD complications (high blood pressure, anemia, hyperphosphatemia, and acidosis) according to severity of CKD and examined factors associated with the absence of drug therapy, among participants with a persistent comorbidity, for 1 year in children enrolled in the CKiD study.
Structured re-training to reduce peritonitis in a pediatric peritoneal dialysis program: a quality improvement intervention
Sharon Teo, Tin Wei Yuen, Clarissa Wei-Shuen Cheong, Md Azizur Rahman, Neha Bhandari, Noor-Haziah Hussain, Hamidah Mistam, Jing Geng, Charmaine Yan-Pin Goh, Mya Than, Yiong-Huak Chan, Hui-Kim Yap & Kar-Hui Ng
doi : 10.1007/s00467-021-05039-2
Pediatric Nephrology volume 36, pages3191–3200 (2021)
Decline in skills and knowledge among patients and/or caregivers contributes to peritoneal-dialysis (PD)-related peritonitis. Re-training is important, but no guidelines exist. We describe the implementation of a structured re-training program to decrease peritonitis rates.
Study of the association between generic and disease-specific quality of life and behavior problems in pediatric patients with chronic kidney disease stage 3 or higher and the quality of life and mental health of their primary caregivers
Renata Oliveira Abrão, Marcos Lopes, Guilherme J. S. Silva, Alexandre A. Ferraro & Vera H. Koch
doi : 10.1007/s00467-021-04986-0
Pediatric Nephrology volume 36, pages3201–3210 (2021)
Chronic kidney disease (CKD) can affect quality of life and mental health of patients and their primary caregivers (PCs) in different disease stages.
Effects of losartan and enalapril on serum uric acid and GFR in children with proteinuria
Charlotte E. Bryant, Azita Rajai, Nicholas J. A. Webb & Ronald J. Hogg
doi : 10.1007/s00467-021-05045-4
Pediatric Nephrology volume 36, pages3211–3219 (2021)
Studies have shown that losartan reduces serum uric acid in adults, unlike angiotensin-converting enzyme inhibitors. A previous study demonstrated that losartan and enalapril had comparable effects on proteinuria in children.
Performance of the various serum creatinine-based GFR estimating equations in pediatric kidney transplant recipients, stratified by age and CKD staging
Raja Dandamudi, Neil Vyas, Stanley P. Hmiel & Vikas R. Dharnidharka
doi : 10.1007/s00467-021-05024-9
Pediatric Nephrology volume 36, pages3221–3228 (2021)
Numerous equations are used to estimate glomerular filtration rate (eGFR), based on serum creatinine (SCr), demographic and anthropometric data, none established in pediatric kidney transplant recipients. This study aimed to validate the available SCr-based eGFR equations in comparison with a measured (mGFR), stratified by chronic kidney disease (CKD) stage and age at the time of testing.
CMV, EBV, JCV and BKV infection and outcome following kidney transplantation in children initiated on a corticosteroid-minimisation immunosuppressive regimen
James McCaffrey, Vijesh J. Bhute & Mohan Shenoy
doi : 10.1007/s00467-021-05047-2
Pediatric Nephrology volume 36, pages3229–3240 (2021)
Modern immunosuppressive regimens in paediatric kidney transplant recipients have contributed to improved long-term allograft survival, but at the expense of an increased incidence of viral infections. Here, we describe, for the first time, the incidence, risk factors and clinical outcome of CMV, EBV, BKV and JCV viraemia in a cohort of paediatric allograft recipients treated with a corticosteroid-minimisation immunosuppressive regimen (CMR).
Associations among erythropoietic, iron-related, and FGF23 parameters in pediatric kidney transplant recipients
Blair Limm-Chan, Katherine Wesseling-Perry, Meghan H. Pearl, Grace Jung, Eileen Tsai-Chambers, Patricia L. Weng & Mark R. Hanudel
doi : 10.1007/s00467-021-05081-0
Pediatric Nephrology volume 36, pages3241–3249 (2021)
In pediatric kidney transplant recipients, anemia is common and oftentimes multifactorial. Hemoglobin concentrations may be affected by traditional factors, such as kidney function and iron status, as well as novel parameters, such as fibroblast growth factor 23 (FGF23).
Acute kidney injury in pediatric non-traumatic rhabdomyolysis
Chon In Kuok & Winnie Kwai Yu Chan
doi : 10.1007/s00467-021-05057-0
Pediatric Nephrology volume 36, pages3251–3257 (2021)
Our study aimed to determine the prevalence of acute kidney injury (AKI) in pediatric non-traumatic rhabdomyolysis, and to identify factors associated with its development.
Serum metabolic profile of postoperative acute kidney injury following infant cardiac surgery with cardiopulmonary bypass
Jesse A. Davidson, Benjamin S. Frank, Tracy T. Urban, Mark Twite, James Jaggers, Ludmila Khailova & Jelena Klawitter
doi : 10.1007/s00467-021-05095-8
Pediatric Nephrology volume 36, pages3259–3269 (2021)
We sought to determine differences in the circulating metabolic profile of infants with or without acute kidney injury (AKI) following cardiothoracic surgery with cardiopulmonary bypass (CPB).
Early relapse of atypical hemolytic uremic syndrome following ABO-incompatible living–related pediatric kidney re-transplant successfully treated with eculizumab
Jelena Stojanovic, Anna Adamusiak, Aoife Waters, Neil J. Sebire, Nicos Kessaris, Nizam Mamode & Stephen D. Marks
doi : 10.1007/s00467-021-05193-7
Pediatric Nephrology volume 36, pages3271–3275 (2021)
A 3-year-old girl with clinical features of atypical HUS (complement Factor I mutation inherited from an asymptomatic mother and Factor H autoantibodies) was treated with plasma exchange, progressed to kidney failure (KF) aged 4 years, and received an en bloc kidney DCD transplant aged 8 years with primary graft non-function necessitating transplant nephrectomy at the time of transplantation. She subsequently underwent re-transplantation from her father. This is a retrospective study of electronic patient records and medical notes.
Atypical HUS and Crohn’s disease—interference of intestinal disease activity with complement-blocking treatment
Orsolya Horváth, Kata Kelen, Zoltán Prohászka, Ádám Hosszú, Attila J Szabó & George S Reusz
doi : 10.1007/s00467-021-05167-9
Pediatric Nephrology volume 36, pages3277–3280 (2021)
In atypical hemolytic-uremic syndrome (aHUS), various defects of the complement system have been reported to explain pathophysiology. Therapeutic options for complement inhibition are well-recognized; however, the links between various immune-derived diseases and aHUS are unclear, and their interference with treatment efficacy during long-term complement-blocking therapy is scarcely known.
Real-world data of six patients with atypical hemolytic uremic syndrome switched to ravulizumab
Rasmus Ehren & Sandra Habbig
doi : 10.1007/s00467-021-05203-8
Pediatric Nephrology volume 36, pages3281–3282 (2021)
Correction to: Preventing tuberculosis in paediatric kidney transplant recipients: is there a role for BCG immunisation pre-transplantation in low tuberculosis incidence countries?
Alasdair Bamford, Garth Dixon, Nigel Klein, Stephen Marks, Nicole Ritz, Steven B. Welch & Marc Tebruegge
doi : 10.1007/s00467-020-04893-w
Pediatric Nephrology volume 36, page3283 (2021)
Abstracts of the 53rd ESPN Annual Meeting, Amsterdam, The Netherlands, September 2021
doi : 10.1007/s00467-021-05210-9
Pediatric Nephrology volume 36, pages3285–3491 (2021)
