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Assessment and management of obesity and metabolic syndrome in children with CKD stages 2–5 on dialysis and after kidney transplantation—clinical practice recommendations from the Pediatric Renal Nutrition Taskforce
Stella Stabouli, Nonnie Polderman, Christina L. Nelms, Fabio Paglialonga, Michiel J. S. Oosterveld, Larry A. Greenbaum, Bradley A. Warady, Caroline Anderson, Dieter Haffner, An Desloovere, Leila Qizalbash, José Renken-Terhaerdt, Jetta Tuokkola, Johan Vande Walle, Vanessa Shaw, Mark Mitsnefes & Rukshana Shroff
doi : 10.1007/s00467-021-05148-y
Pediatric Nephrology volume 37, pages1–20 (2022)
Obesity and metabolic syndrome (O&MS) due to the worldwide obesity epidemic affects children at all stages of chronic kidney disease (CKD) including dialysis and after kidney transplantation. The presence of O&MS in the pediatric CKD population may augment the already increased cardiovascular risk and contribute to the loss of kidney function. The Pediatric Renal Nutrition Taskforce (PRNT) is an international team of pediatric renal dietitians and pediatric nephrologists who develop clinical practice recommendations (CPRs) for the nutritional management of children with kidney diseases. We present CPRs for the assessment and management of O&MS in children with CKD stages 2–5, on dialysis and after kidney transplantation. We address the risk factors and diagnostic criteria for O&MS and discuss their management focusing on non-pharmacological treatment management, including diet, physical activity, and behavior modification in the context of age and CKD stage. The statements have been graded using the American Academy of Pediatrics grading matrix. Statements with a low grade or those that are opinion-based must be carefully considered and adapted to individual patient needs based on the clinical judgment of the treating physician and dietitian. Research recommendations are provided. The CPRs will be periodically audited and updated by the PRNT.
A practical approach to the genomics of kidney disorders
Eleanor Hay, Thomas Cullup & Angela Barnicoat
doi : 10.1007/s00467-021-04995-z
Pediatric Nephrology volume 37, pages21–35 (2022)
Rapid technological advances in genomic testing continue to increase our understanding of the genetic basis of a wide range of kidney disorders. Establishing a molecular diagnosis benefits the individual by bringing an end to what is often a protracted diagnostic odyssey, facilitates accurate reproductive counselling for families and, in the future, is likely to lead to the delivery of more targeted management and surveillance regimens. The selection of the most appropriate testing modality requires an understanding both of the technologies available and of the genetic architecture and heterogeneity of kidney disease. Whilst we are witnessing a far greater diagnostic yield with broader genetic testing, such approaches invariably generate variants of uncertain significance and secondary incidental findings, which are not only difficult to interpret but present ethical challenges with reporting and feeding back to patients and their families. Here, we review the spectrum of nephrogenetic disorders, consider the optimal approach to genetic testing, explore the clinical utility of obtaining a molecular diagnosis, reflect on the challenges of variant interpretation and look to the future of this dynamic field.
Optimizing the corticosteroid dose in steroid-sensitive nephrotic syndrome
Martin T. Christian & Andrew P. Maxted
doi : 10.1007/s00467-021-04985-1
Pediatric Nephrology volume 37, pages37–47 (2022)
The use of corticosteroids in the treatment of steroid-sensitive nephrotic (SSNS) syndrome in children has evolved surprisingly slowly since the ISKDC consensus over 50 years ago. From a move towards longer courses of corticosteroid to treat the first episode in the 1990s and 2000s, more recent large, well-designed randomized controlled trials (RCTs) have unequivocally shown no benefit from an extended course, although doubt remains whether this applies across all age groups. With regard to prevention of relapses, daily ultra-low-dose prednisolone has recently been shown to be more effective than low-dose alternate-day prednisolone. Daily low-dose prednisolone for a week at the time of acute viral infection seems to be effective in the prevention of relapses but the results of a larger RCT are awaited. Recently, corticosteroid dosing to treat relapses has been questioned, with data suggesting lower doses may be as effective. The need for large RCTs to address the question of whether corticosteroid doses can be reduced was the conclusion of the authors of the recent corticosteroid therapy for nephrotic syndrome in children Cochrane update. This review summarizes development in thinking on corticosteroid use in SSNS and makes suggestions for areas that merit further scrutiny.
The clinical and radiological cerebrovascular abnormalities associated with renovascular hypertension in children: a systematic review
Nadeesha L Mudalige, Chavini Ranasinghe & Jelena Stojanovic
doi : 10.1007/s00467-021-05165-x
Pediatric Nephrology volume 37, pages49–59 (2022)
Renovascular disease is an important secondary cause of hypertension in childhood. In this cohort, many may have undiagnosed cerebrovascular disease, and some children present acutely with cerebrovascular complications. However, these associations are yet to be defined in the literature.
Biomarkers of acute kidney injury in pediatric cardiac surgery
Candice Torres de Melo Bezerra Cavalcante, Marcelo Borges Cavalcante, Klebia Magalhães Pereira Castello Branco, Titus Chan, Isabel Cristina Leite Maia, Ronald Guedes Pompeu, Andrea Consuelo de Oliveira Telles, Anna Karina Martins Brito & Alexandre Braga Libório
doi : 10.1007/s00467-021-05094-9
Pediatric Nephrology volume 37, pages61–78 (2022)
Acute kidney injury (AKI) is characterized by a sudden decrease in kidney function. Children with congenital heart disease are a special group at risk of developing AKI. We performed a systematic review of the literature to search for studies reporting the usefulness of novel urine, serum, and plasma biomarkers in the diagnosis and progression of AKI and their association with clinical outcomes in children undergoing pediatric cardiac surgery. In thirty studies, we analyzed the capacity to predict AKI and poor outcomes of five biomarkers: Cystatin C, Neutrophil gelatinase-associated lipocalin, Interleukin-18, Kidney injury molecule-1, and Liver fatty acid–binding protein. In conclusion, we suggest the need for further meta-analyses with the availability of additional studies.
Early microvascular complications in type 1 and type 2 diabetes: recent developments and updates
Kalie L. Tommerdahl, Allison L. B. Shapiro, Edward J. Nehus & Petter Bjornstad
doi : 10.1007/s00467-021-05050-7
Pediatric Nephrology volume 37, pages79–93 (2022)
The prevalence of youth-onset diabetes is progressing rapidly worldwide, and poor glycemic control, in combination with prolonged diabetes duration and comorbidities including hypertension, has led to the early development of microvascular complications including diabetic kidney disease, retinopathy, and neuropathy. Pediatric populations with type 1 (T1D) and type 2 (T2D) diabetes are classically underdiagnosed with microvascular complications, and this leads to both undertreatment and insufficient attention to the mitigation of risk factors that could help attenuate further progression of complications and decrease the likelihood for long-term morbidity and mortality. This narrative review aims to present a comprehensive summary of the epidemiology, risk factors, symptoms, screening practices, and treatment options, including future opportunities for treatment advancement, for microvascular complications in youth with T1D and T2D. We seek to uniquely focus on the inherent challenges of managing pediatric populations with diabetes and discuss the similarities and differences between microvascular complications in T1D and T2D, while presenting a strong emphasis on the importance of early identification of at-risk youth. Further investigation of possible treatment mechanisms for microvascular complications in youth with T1D and T2D through dedicated pediatric outcome trials is necessary to target the brief window where early pathological vascular changes may be significantly attenuated.
Fabry disease and kidney involvement: starting from childhood to understand the future
Roberto Chimenz, Valeria Chirico, Caterina Cuppari, Giorgia Ceravolo, Daniela Concolino, Paolo Monardo & Antonio Lacquaniti
doi : 10.1007/s00467-021-05076-x
Pediatric Nephrology volume 37, pages95–103 (2022)
The accumulation of globotriaosylceramide (Gb-3) in multiple organs, such as the heart, kidney, and nervous system, due to mutations in the galactosidase alpha (GLA) gene, represents the key point of Fabry disease (FD). The common symptoms appear in childhood or adolescence, including neuropathic pain, angiokeratoma, acroparesthesia, and corneal opacities. A multi-organ involvement induces a significant deterioration in the quality of life with high mortality in adulthood. The accumulation of Gb-3 involves all types of kidney cells beginning at fetal development, many years before clinical manifestations. A decline in the glomerular filtration rate is rare in children, but it can occur during adolescence. Pediatric patients rarely undergo kidney biopsy that could assess the efficacy of enzyme replacement therapy (ERT) behind its diagnostic role. To date, diagnosis is achieved by detecting reduced ?-Gal-A activity in leukocytes and plasma, allowing for the early start of ERT. This review focuses on pediatric kidney involvement in FD, analyzing in depth its diagnostic processes and treatment options.
Palliative care for children and young people with stage 5 chronic kidney disease
Finella Craig, Ellen M. Henderson, Bhumik Patel, Fliss E. M. Murtagh & Myra Bluebond-Langner
doi : 10.1007/s00467-021-05056-1
Pediatric Nephrology volume 37, pages105–112 (2022)
Death from stage 5 chronic kidney disease (CKD 5) in childhood or adolescence is rare, but something that all paediatric renal physicians and most paediatricians will encounter. In this paper, we present the literature on three key areas of palliative care practice essential to good clinical management: shared decision-making, advance care planning, and symptom management, with particular reference to CKD 5 where kidney transplant is not an option and where a decision has been made to withdraw or withhold dialysis. Some areas of care, particularly with regard to symptom management, have not been well-studied in children and young people (CYP) with CKD 5 and recommendations with regard to drug choice and dose modification are based on adult literature, known pharmacokinetics, and clinical experience.
Systemic vasculopathy and hypertension in a child: Questions
Emre Levento?lu, Kibriya Fidan, Bahar Büyükkaragöz, Esra Serdaro?lu, Tuba Atalay, Merve Yazol & O?uz Söylemezo?lu
doi : 10.1007/s00467-021-05309-z
Pediatric Nephrology volume 37, pages113–115 (2022)
Systemic vasculopathy and hypertension in a child: Answers
Emre Levento?lu, Kibriya Fidan, Bahar Büyükkaragöz, Esra Serdaro?lu, Tuba Atalay, Merve Yazol & O?uz Söylemezo?lu
doi : 10.1007/s00467-021-05310-6
Pediatric Nephrology volume 37, pages117–119 (2022)
New-onset cough and fever in a toddler with stage 5 chronic kidney disease: Questions
Cassandra A. Petri, Yalile Perez, Leah S. Heidenreich, Carl H. Cramer, Timothy M. Olson, Nathan C. Hull & Christian Hanna
doi : 10.1007/s00467-021-05294-3
Pediatric Nephrology volume 37, pages121–122 (2022)
New-onset cough and fever in a toddler with stage 5 chronic kidney disease: Answers
Cassandra A. Petri, Yalile Perez, Leah S. Heidenreich, Carl H. Cramer, Timothy M. Olson, Nathan C. Hull & Christian Hanna
doi : 10.1007/s00467-021-05301-7
Pediatric Nephrology volume 37, pages123–125 (2022)
Fanconi syndrome, nephrotic-range proteinuria, and hypoalbuminemia in a newborn—Occam’s razor or Hickam’s dictum? Questions
James T. Nugent, Juliann Reardon, Christine Crana, Jason H. Greenberg, Jillian K. Warejko & Julie E. Goodwin
doi : 10.1007/s00467-021-05247-w
Pediatric Nephrology volume 37, pages127–128 (2022)
Fanconi syndrome, nephrotic-range proteinuria, and hypoalbuminemia in a newborn—Occam’s razor or Hickam’s dictum? Answers
James T. Nugent, Juliann Reardon, Christine Crana, Jason H. Greenberg, Jillian K. Warejko & Julie E. Goodwin
doi : 10.1007/s00467-021-05255-w
Pediatric Nephrology volume 37, pages129–132 (2022)
A 7-year-old girl with renal medullary hyperechogenicity and hypertension: Questions
?lknur Giri?gen, Selcuk Yüksel, Furkan Ufuk, Taner Durak & Tülay Becerir
doi : 10.1007/s00467-021-05311-5
Pediatric Nephrology volume 37, pages133–134 (2022)
A 7-year-old girl with renal medullary hyperechogenicity and hypertension: Answers
?lknur Giri?gen, Selcuk Yüksel, Furkan Ufuk, Taner Durak & Tülay Becerir
doi : 10.1007/s00467-021-05314-2
Pediatric Nephrology volume 37, pages135–138 (2022)
Influence of genetic polymorphism in renin-angiotensin system-candidate genes on urinary trefoil family factor 3 levels in children with congenital anomalies of kidney and urinary tract
Sachit Anand, Minu Bajpai, Tripti Khanna & Alok Kumar
doi : 10.1007/s00467-021-05160-2
Pediatric Nephrology volume 37, pages139–145 (2022)
This cross-sectional study aimed to determine the influence of genetic polymorphism in two renin-angiotensin system (RAS)-candidate genes on urinary trefoil family factor 3 (TFF3) levels in children with congenital anomalies of kidney and urinary tract (CAKUT).
To biopsy or not to biopsy: Henoch-Schönlein nephritis in children, a 5-year follow-up study
Marina Avramescu, Annie Lahoche, Julien Hogan, Rémi Salomon, Gwenaëlle Roussey, Justine Bacchetta, Stéphane Decramer, Tim Ulinski, Coralie Barbe & Christine Pietrement
doi : 10.1007/s00467-021-05086-9
Pediatric Nephrology volume 37, pages147–152 (2022)
The prognosis of Henoch-Schönlein purpura (HSP), IgA vasculitis, depends on kidney involvement. There is no consensus on the initiation of treatment for HSP nephritis (HSPN). Some centres start treatment before performing a kidney biopsy (KB) while in others, treatment is dictated by the importance of the clinical, biological and histological signs. The aim of this study was to evaluate which of these two approaches is associated with a better kidney outcome at 5-year follow-up.
De novo lupus-like glomerulonephritis after pediatric non-kidney organ transplantation
Cristina M. Farkas-Skiles, Robert B. Ettenger, Jonathan E. Zuckerman, Meghan Pearl, Robert S. Venick & Patricia L. Weng
doi : 10.1007/s00467-021-05194-6
Pediatric Nephrology volume 37, pages153–161 (2022)
We propose a novel clinically significant finding, de novo lupus-like glomerulonephritis (DNLLGN), in patients with autoantibodies and kidney abnormalities in pediatric liver transplant (LT) and intestinal inclusive transplants (ITx).
Maternal antibiotic exposure during pregnancy is a risk factor for community-acquired urinary tract infection caused by extended-spectrum beta-lactamase-producing bacteria in infants
Ji Hyun Kim, Juyoung Lee, Dong Hyun Kim, Ji Young Park, Hyunju Lee, Hee Gyung Kang & Yo Han Ahn
doi : 10.1007/s00467-021-05163-z
Pediatric Nephrology volume 37, pages163–170 (2022)
This study aimed to investigate the risk factors for community-acquired urinary tract infection (UTI) caused by extended-spectrum beta-lactamase (ESBL)-positive bacteria in infants.
Biomarkers for febrile urinary tract infection in children
Nader Shaikh, Hui Liu, Marcia Kurs-Lasky & Catherine S Forster
doi : 10.1007/s00467-021-05173-x
Pediatric Nephrology volume 37, pages171–177 (2022)
The sensitivity and specificity of the leukocyte esterase test for the diagnosis of urinary tract infection (UTI) are suboptimal. Recent studies have identified markers that appear to more accurately differentiate children with and without UTI. The objective of this study was to determine the accuracy of these markers, which included CCL3, IL-8, CXCL1, TNF-alpha, IL-6, IFN-gamma, IL-17, IL-9, IL-2, and NGAL, in the diagnosis of UTI.
Discordances between pediatric and adult thresholds in the diagnosis of hypertension in adolescents with CKD
Elizabeth Black, Jason Lee, Joseph T. Flynn, Charles E. McCulloch, Joshua A. Samuels, Divya Seth, Bradley Warady, Susan Furth, Mark Mitsnefes & Elaine Ku
doi : 10.1007/s00467-021-05166-w
Pediatric Nephrology volume 37, pages179–188 (2022)
Adolescents with chronic kidney disease (CKD) are a unique population with a high prevalence of hypertension. Management of hypertension during the transition from adolescence to adulthood can be challenging given differences in normative blood pressure values in adolescents compared with adults.
Patient-centered outcomes in pediatric continuous kidney replacement therapy: new morbidity and worsened functional status in survivors
Mallory Smith, Cynthia Bell, Molly Wong Vega, Naile Tufan Pekkucuksen, Laura Loftis, Mona McPherson, Jeanine Graf & Ayse Akcan Arikan
doi : 10.1007/s00467-021-05177-7
Pediatric Nephrology volume 37, pages189–197 (2022)
Ongoing measures to improve pediatric continuous kidney replacement therapy (CKRT) have lowered mortality rates, shifting the focus to survivor functional status. While septic acute kidney injury generates new morbidity in pediatric critically ill patients, acquired morbidities and functional status of CKRT population are unknown. We predicted that CKRT survivors are at risk for new morbidity and would have worse functional status at PICU discharge compared to baseline, and aimed to describe associated factors.
UK experience of ofatumumab in recurrence of focal segmental glomerulosclerosis post-kidney transplant
Ben C. Reynolds, Angela Lamb, Caroline A. Jones, Pallavi Yadav, Kay S. Tyerman & Colin C. Geddes
doi : 10.1007/s00467-021-05248-9
Pediatric Nephrology volume 37, pages199–207 (2022)
Steroid-resistant nephrotic syndrome (SRNS), commonly caused by focal segmental glomerulosclerosis (FSGS), is associated with progression to stage 5 chronic kidney disease, requirement for kidney replacement therapy and a risk of disease recurrence post-kidney transplantation. Ofatumumab (OFA) is a fully humanised monoclonal antibody to CD20, with similar mechanisms of action to rituximab (RTX).
The impact of donor and recipient sex on kidney allograft survival in pediatric transplant recipients
Nadeesha L Mudalige, Chloe Brown & Stephen D Marks
doi : 10.1007/s00467-021-05071-2
Pediatric Nephrology volume 37, pages209–216 (2022)
Transplantation is widely considered the gold standard method of kidney replacement therapy. Despite compelling evidence for biological sexual dimorphisms, the role of donor and recipient sex matching in transplantation is undefined.
Peritoneal dialysis-related peritonitis caused by Gordonia bronchialis: first pediatric report
Valentina Bruno, James Tjon, Sandy Lin, Helen Groves, Kescha Kazmi, Michael Zappitelli & Elizabeth Harvey
doi : 10.1007/s00467-021-05313-3
Pediatric Nephrology volume 37, pages217–220 (2022)
Gordonia species, aerobic, weakly acid-fast, Gram-positive bacilli, are a rare cause of peritonitis in patients undergoing peritoneal dialysis (PD). We report the first pediatric case of PD-related peritonitis caused by Gordonia bronchialis.
Prof. Man-Chun Chiu (1949–2021)
Alison Ma on behalf of Hong Kong Pediatric Nephrology Society, Chinese Society of Pediatric Nephrology and the Asian Pediatric Nephrology Association
doi : 10.1007/s00467-021-05327-x
Pediatric Nephrology volume 37, pages221–222 (2022)
New and available biomarker to predict acute kidney injury after pediatric cardiac surgery
Valdano Manuel & Leonardo A. Miana
doi : 10.1007/s00467-021-05174-w
Pediatric Nephrology volume 37, pages223–224 (2022)
Onset of nephrotic syndrome concomitant to SARS-CoV-2 infection in a 3-year-old child
Alessia Morreale & Maria Luisa Casciana
doi : 10.1007/s00467-021-05307-1
Pediatric Nephrology volume 37, page225 (2022)
Response to Morreale
Nelson Orta & Amado Alvarado
doi : 10.1007/s00467-021-05308-0
Pediatric Nephrology volume 37, page227 (2022)
Referees for 2021
doi : 10.1007/s00467-021-05286-3
Pediatric Nephrology volume 37, pages229–235 (2022)
Correction to: Palliative care for children and young people with stage 5 chronic kidney disease
Finella Craig, Ellen M. Henderson, Bhumik Patel, Fliss E. M. Murtagh & Myra Bluebond-Langner
doi : 10.1007/s00467-021-05261-y
Pediatric Nephrology volume 37, page237 (2022)
