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A new team for the new year
Giovanni Montini & Joseph T. FlynnÂ
doi : 10.1007/s00467-022-05804-x
Volume 38, issue 1, January 2023
Glomerular B7-1 staining: toward precision medicine for treatment of recurrent focal segmental glomerulosclerosis
Chia-shi Wang, William E. Smoyer & Gabriel Cara-FuentesÂ
Therapeutic trials in difficult to treat steroid sensitive nephrotic syndrome: challenges and future directions
Ashlene M. McKay, Rulan S. Parekh & Damien NooneÂ
doi : 10.1007/s00467-022-05520-6
Steroid sensitive nephrotic syndrome is a common condition in pediatric nephrology, and most children have excellent outcomes. Yet, 50% of children will require steroid-sparing agents due to frequently relapsing disease and may suffer consequences from steroid dependence or use of steroid-sparing agents.
The learning health system for pediatric nephrology: building better systems to improve health
Charles D. Varnell Jr, Peter Margolis, Jens Goebel & David K. HooperÂ
doi : 10.1007/s00467-022-05526-0
Learning health systems (LHS) align science, informatics, incentives, and culture for continuous improvement and innovation.
Neonatal fluid overload—ignorance is no longer bliss
Lucinda J. Weaver, Colm P. Travers, Namasivayam Ambalavanan & David AskenaziÂ
doi : 10.1007/s00467-022-05514-4
Excessive accumulation of fluid may result in interstitial edema and multiorgan dysfunction. Over the past few decades, the detrimental impact of fluid overload has been further defined in adult and pediatric populations.
The treatment of atypical hemolytic uremic syndrome with eculizumab in pediatric patients: a systematic review
Raquel Medeiros de Souza, Bernardo Henrique Mendes Correa, Paulo Henrique Moreira Melo, Pedro Antunes Pousa, Tamires Sara Campos de Mendonça, Lucas Gustavo Castelar Rodrigues & Ana Cristina Simões e SilvaÂ
doi : 10.1007/s00467-022-05683-2
The atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy associated with high morbidity and high mortality. Eculizumab, a humanized anti-C5 monoclonal antibody, was the first medication approved for treating aHUS in 2011.
Should we screen for intracranial aneurysms in children with autosomal dominant polycystic kidney disease?
Emma Y. X. Walker & Matko MarlaisÂ
doi : 10.1007/s00467-022-05432-5
This is an overview of the challenges associated with screening for asymptomatic intracranial aneurysms (ICA) in children with autosomal dominant polycystic kidney disease (ADPKD).
Nutritional management of the infant with chronic kidney disease stages 2–5 and on dialysis
Vanessa Shaw, Caroline Anderson, An Desloovere, Larry A. Greenbaum, Dieter Haffner, Christina L. Nelms, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, José Renken-Terhaerdt, Stella Stabouli, Jetta Tuokkola, Johan Vande Walle, Bradley A. Warady & Rukshana ShroffÂ
doi : 10.1007/s00467-022-05529-x
The nutritional management of children with chronic kidney disease (CKD) is of prime importance in meeting the challenge of maintaining normal growth and development in this population.
A 16-month-old boy with nephrotic syndrome of apparent idiopathic origin: Question
Laura Beaudoin, Iris Puyol, Natalia Luján Robledo & Alejandro BalestracciÂ
A 16-month-old boy with nephrotic syndrome of apparent idiopathic origin: Answer
Laura Beaudoin, Iris Puyol, Natalia Luján Robledo & Alejandro BalestracciÂ
Unexpected finding in kidney biopsy of a child with nephrotic proteinuria: Questions
Meral Torun Bayram, Gizem Yildiz, Ahmet Okay CaÄŸlayan, Ayfer Ulgenalp, Sadiye Mehtat Unlu, Alper Soylu & Salih KavukcuÂ
Unexpected finding in kidney biopsy of a child with nephrotic proteinuria: Answers
Meral Torun Bayram, Gizem Yildiz, Ahmet Okay CaÄŸlayan, Ayfer Ulgenalp, Sadiye Mehtat Unlu, Alper Soylu & Salih KavukcuÂ
Clinical and genetic characteristics of Tunisian children with infantile nephropathic cystinosis
Mariem El Younsi, Médiha Trabelsi, Sandra Ben Youssef, Inès Ouertani, Yousra Hammi, Ahlem Achour, Faouzi Maazoul, Maher Kharrat, Tahar Gargah & Ridha M’radÂ
doi : 10.1007/s00467-022-05525-1
Nephropathic cystinosis is an autosomal recessive disease caused by a mutation in the CTNS gene which encodes cystinosin, a lysosomal cystine transporter. The spectrum of mutations in the CTNS gene is not well defined in the North African population.
Epidemiology and outcomes of pneumococcal sepsis in children with nephrotic syndrome in a developing country
Georgie Mathew, Anish Sam George, R. V. Deepthi, Winsley Rose, Valsan Philip Verghese, Rosemol Varghese, Balaji Veeraraghavan & Indira AgarwalÂ
doi : 10.1007/s00467-022-05550-0
Pneumococcal infections are common in children with nephrotic syndrome. Knowledge of the commonly available serotypes and antibiotic susceptibility will help in prevention and appropriate management of pneumococcal sepsis, especially in resource-limited countries.
Dipping at home: is it better, easier, and more convenient? A feasibility and acceptability study of a novel home urinalysis using a smartphone application
Daniella Levy Erez, Hannah Derwick, Susan Furth, Lance Ballester, Stephanie Omuemu, Yonathan Adiri, Jonah Mink & Michelle DenburgÂ
doi : 10.1007/s00467-022-05556-8
Monitoring proteinuria in patients with kidney disease is of crucial importance given its implications for long-term disease progression and clinical management.
Benefit of B7-1 staining and abatacept for treatment-resistant post-transplant focal segmental glomerulosclerosis in a predominantly pediatric cohort: time for a reappraisal
George W. Burke III, Jayanthi Chandar, Junichiro Sageshima, Mariella Ortigosa-Goggins, Pooja Amarapurkar, Alla Mitrofanova, Marissa J. Defreitas, Chryso P. Katsoufis, Wacharee Seeherunvong, Alexandra Centeno, Javier Pagan, Lumen A. Mendez-Castaner, Adela D. Mattiazzi, Warren L. Kupin, Giselle Guerra, Linda J. Chen, Mahmoud Morsi, Jose M. G. Figueiro, Rodrigo Vianna, Carolyn L. Abitbol, David Roth, Alessia Fornoni, Phillip Ruiz, Gaetano Ciancio & Eduardo H. GarinÂ
doi : 10.1007/s00467-022-05549-7
Primary FSGS manifests with nephrotic syndrome and may recur following KT. Failure to respond to conventional therapy after recurrence results in poor outcomes. Evaluation of podocyte B7-1 expression and treatment with abatacept (a B7-1 antagonist) has shown promise but remains controversial.
Congenital anomalies of the kidney and urinary tract (CAKUT) in critically ill infants: a multicenter cohort study
Esther Huimin Leow, Jan Hau Lee, Christoph P. Hornik, Yong Hong Ng, Thomas Hays, Reese H. Clark, Veeral N. Tolia & Rachel G. GreenbergÂ
doi : 10.1007/s00467-022-05542-0
The aim of the study was to determine the prevalence of congenital anomalies of the kidney and urinary tract (CAKUT) in the neonatal intensive care unit (NICU) and to evaluate risk factors associated with worse outcomes. We hypothesized that infants with CAKUT with extra-renal manifestations have higher mortality.
Kidney length standardized to body length predicts outcome in infants with a solitary functioning kidney
Douglas G. Matsell, Carol Bao, Teagan Po White, Ella Chan, Eli Matsell, Dan Cojocaru, Lydia Bajno & Marisa CatapangÂ
doi : 10.1007/s00467-022-05544-y
Infants with a solitary functioning kidney (SFK) are at risk for chronic kidney injury (CKI). Lack of compensatory kidney growth (CKG) is associated with CKI, but measuring CKG is challenging since it is typically reported relative to normal kidneys.
Outcomes and risk factors for death among hospitalized children and adolescents with kidney diseases and COVID-19: an analysis of a nationwide database
Mariana A. Vasconcelos, Ana Carmen Q. Mendonça, Enrico A. Colosimo, Noureddin Nourbakhsh, HercÃlio Martelli-Júnior, Ludmila R. Silva, Maria Christina L. Oliveira, Clara C. Pinhati, Robert H. Mak, Ana Cristina Simões e Silva & Eduardo A. OliveiraÂ
doi : 10.1007/s00467-022-05588-0
Patients with kidney diseases (KD) appear to be at particularly high risk for severe COVID-19. This study aimed to characterize the clinical outcomes and risk factors for COVID-19-related death in a large cohort of hospitalized pediatric patients with KD.
Plasma levels of carboxylic acids are markers of early kidney dysfunction in young people with type 1 diabetes
Timothy Vigers, Carissa Vinovskis, Lu-Ping Li, Pottumarthi Prasad, Hiddo Heerspink, Angelo D’Alessandro, Julie A. Reisz, Federica Piani, David Z. Cherney, Daniel H. van Raalte, Kristen J. Nadeau, Meda E. Pavkov, Robert G. Nelson, Laura Pyle & Petter BjornstadÂ
doi : 10.1007/s00467-022-05531-3
We compared plasma metabolites of amino acid oxidation and the tricarboxylic acid (TCA) cycle in youth with and without type 1 diabetes mellitus (T1DM) and related the metabolites to glomerular filtration rate (GFR), renal plasma flow (RPF), and albuminuria.
Telehealth survey of providers and caregivers of children on peritoneal dialysis during the COVID-19 pandemic
Stephanie L. Clark, Brandy Begin, Heidi G. De Souza, Kathleen Mallett, Melisha G. Hanna, Troy Richardson, Megan Esporas, Ariana Bowie, Karri Taylor, Laura Castellanos Reyes, Monica Hughey, Alicia Neu & Bradley A. WaradyÂ
doi : 10.1007/s00467-022-05543-z
There has been growing support for the adoption of telehealth (TH) services in pediatric populations.
An initiative to improve effluent culture detection among pediatric patients undergoing peritoneal dialysis through process improvement
Scott F. Pangonis, Joshua K. Schaffzin, Donna Claes, Joel E. Mortenson & Edward NehusÂ
doi : 10.1007/s00467-022-05533-1
Peritonitis is a significant cause of morbidity and healthcare cost among pediatric patients undergoing peritoneal dialysis. Culture-negative peritonitis has been associated with an increased risk of technique failure.
Immature granulocytes as biomarkers of inflammation in children with predialysis chronic kidney disease
Nuran Cetın, Evin Kocaturk, Aslı Kavaz Tufan, Zeynep Kusku Kıraz & Ozkan AlatasÂ
doi : 10.1007/s00467-022-05530-4
Chronic inflammation in patients with predialysis chronic kidney disease (CKD) is quite common. We aimed to investigate the relationship of the percentage of immature granulocytes (IG%) and immature granulocyte count (IGC) with inflammation in children with predialysis CKD.
Missing trial results: analysis of the current publication rate of studies in pediatric dialysis from 2003 to 2020
Christian Patry, Alexander Fichtner, Britta Höcker, Markus Ries, Claus Peter Schmitt & Burkhard TönshoffÂ
doi : 10.1007/s00467-022-05553-x
Decision-making in the field of pediatric dialysis requires evidence from clinical trials, but, similar to other fields of pediatric medicine, might be affected by a low trial publication rate.
Improved equations to estimate GFR in Chinese children with chronic kidney disease
Ying Tang, Ling Hou, Tingting Sun, Shanping Li, Junli Cheng, Dan Xue, Xiuli Wang & Yue DuÂ
doi : 10.1007/s00467-022-05552-y
There is currently no specific equation for estimating glomerular filtration rate (GFR) in Chinese children with chronic kidney disease (CKD). The commonly used equations are less robust than expected; we therefore sought to derive more appropriate equations for GFR estimation.
Child and caregiver perspectives on access to psychosocial and educational support in pediatric chronic kidney disease: a focus group study
Yifan Zhang, Talia Gutman, Allison Tong, Jonathan C. Craig, Aditi Sinha, Allison Dart, Allison A. Eddy, Debbie S. Gipson, Detlef Bockenhauer, Hui-Kim Yap, Jaap Groothoff, Michael Zappitelli, Nicholas J.A.Webb, Stephen I. Alexander, Susan Furth, Susan Samuel, Tom D. Blydt-Hansen, Janis Dionne, Mini Michael, Scott E. Wenderfer, Wolfgang C. Winkelmayer, Steven McTaggart, Amanda Walker, Cortney T. Zimmerman, …Camilla S. HansonÂ
doi : 10.1007/s00467-022-05551-z
Children with chronic kidney disease (CKD) generally have worse educational and psychosocial outcomes compared with their healthy peers.
Survival analysis among pediatric patients receiving kidney replacement therapy: a Japanese nationwide cohort study
Daishi Hirano, Eisuke Inoue, Mayumi Sako, Akira Ashida, Masataka Honda, Shori Takahashi, Kazumoto Iijima & Motoshi Hattori on behalf of the Japanese Society of Pediatric Nephrology
doi : 10.1007/s00467-022-05568-4
Limited data are available on the survival and causes of death in pediatric patients with chronic kidney disease (CKD) stage 5 receiving kidney replacement therapy (KRT) in Asia.
Assessment of the oral health status of children with chronic kidney disease
Berkant Sezer, Remziye Kaya, Nur Kodaman Dokumacıgil, Duygu SıddıkoÄŸlu, Serçin Güven, Nurdan Yıldız, Harika Alpay & Betül KargülÂ
doi : 10.1007/s00467-022-05590-6
There are various oral symptoms related to the disease and its management in individuals with chronic kidney disease (CKD). The aim of the study was to investigate the oral health status of children with different stages of CKD, kidney transplant recipients (KTR), and healthy children.
Early corticosteroid withdrawal is associated with improved adult height in pediatric kidney transplant recipients
Gilles Tourlamain, Mandy Keijzer-Veen, Antonia Bouts, Karlien Cransberg, Huib de Jong, Rani De Pauw, Karen Vanderstraeten, Amina El Amouri, Ann Raes, Noël Knops & Agnieszka PrytuÅ‚aÂ
doi : 10.1007/s00467-022-05581-7
Catch-up growth after pediatric kidney transplantation (kTx) is usually insufficient to reach normal adult height.
Changes in graft function after living donor kidney transplantation in children
Junya Hashimoto, Yuko Hamasaki, Yujiro Aoki, Mai Kubota, Masaki Muramatsu, Takeshi Kawamura, Seiichiro Shishido & Ken SakaiÂ
doi : 10.1007/s00467-022-05540-2
This study aimed to evaluate the change in graft function in two groups stratified by the estimated glomerular filtration rate (eGFR) at 1Â month after transplantation (eGFR-1Â M) in pediatric living donor kidney transplant recipients.
A child with crescentic glomerulonephritis following SARS-CoV-2 mRNA (Pfizer-BioNTech) vaccination
Sujeong Kim, Jiwon Jung, Haeyon Cho, Jina Lee, Heounjeong Go & Joo Hoon LeeÂ
doi : 10.1007/s00467-022-05681-4
There are few reports on kidney complications after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) messenger RNA (mRNA) vaccination, especially in the pediatric population. We report a pediatric case diagnosed with crescentic glomerulonephritis (CrGN) after the second dose of the SARS-CoV-2 mRNA vaccine.
Consider multisystem inflammatory syndrome in children with kidney failure after SARS-CoV-2 vaccination
Josef FinstererÂ
Response to: Before blaming SARS-CoV-2 vaccinations for rhabdomyolysis, other potential triggers should be considered
Jiwon Jung & Joo Hoon LeeÂ
A rare cause of nephrotic syndrome – sphingosine-1-phosphate lyase (SGPL1) deficiency: 2 cases
Ana Paula Spizzirri, Carlos José Cobeñas & Angela del Carmen SuarezÂ
Response to Dr. Spizzirri et al
Tugba Tastemel Ozturk, Fatih Ozaltin & Rezan TopalogluÂ
