Pediatric Nephrology




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What is the true burden of chronic kidney disease in children worldwide?

Jérôme Harambat & Iona Madden

doi : 10.1007/s00467-022-05816-7

Volume 38, issue 5, May 2023

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What to do with kidney length and volumes in large individuals?

Guido Filler, Laura Torres-Canchala, Ajay P. Sharma, Maria E. Díaz González de Ferris & Jaime M. Restrepo

doi : 10.1007/s00467-022-05830-9

In a recent article in Pediatric Nephrology, Pierluigi Marzuillo et al. from Universita degli Studi della Campania Luigi, Vanvitelli, Italy, describe their work on how to assess kidney length in children and adolescents who are overweight or obese, based on height versus body surface area [1]. Marzuillo et al.

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Tertiary lymphoid tissues in kidney diseases: a perspective for the pediatric nephrologist

Takahisa Yoshikawa, Yu Ho Lee, Yuki Sato & Motoko Yanagita

doi : 10.1007/s00467-022-05770-4

Chronic kidney disease (CKD) is a major public health problem worldwide. In the pediatric population, CKD is also an important health issue because it causes several comorbid conditions that can have long-term consequences beyond the pediatric age.

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The complement system in pediatric acute kidney injury

Erin K. Stenson, Jessica Kendrick, Bradley Dixon & Joshua M. Thurman

doi : 10.1007/s00467-022-05755-3

The complement cascade is an important part of the innate immune system. In addition to helping the body to eliminate pathogens, however, complement activation also contributes to the pathogenesis of a wide range of kidney diseases.

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Relationship between nocturnal enuresis and sleep in children and adolescents

Ana Elisa Ribeiro Fernandes, José Reinaldo Corrêa Roveda, Carolina Rodrigues Fernandes, Diego Ferreira Silva, Isabela Cristina de Oliveira Guimarães, Eleonora Moreira Lima, Flávia Cristina de Carvalho Mrad & Mônica Maria de Almeida Vasconcelos

doi : 10.1007/s00467-022-05818-5

Nocturnal enuresis (NE) is a multifactorial and complex condition. One less understood factor in its pathophysiology is the enuretic inability to wake up when the bladder is full (impaired arousal).

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How to take advantage of easily available biomarkers in patients with IgA nephropathy: IgA and C3 in serum and kidney biopsies

Małgorzata Mizerska-Wasiak

doi : 10.1007/s00467-022-05644-9

IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. It is diagnosed based on clinical and histological features including predominant IgA deposits in kidney biopsy.

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Acute and chronic kidney complications in children with type 1 diabetes mellitus

Giulio Rivetti, Brenden E. Hursh, Emanuele Miraglia del Giudice & Pierluigi Marzuillo

doi : 10.1007/s00467-022-05689-w

Children with type 1 diabetes mellitus (T1DM) have an increased risk of developing kidney involvement. Part of the risk establishes at the beginning of T1DM. In fact, up to 65% of children during T1DM onset may experience an acute kidney injury (AKI) which predisposes to the development of a later chronic kidney disease (CKD).

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Nephrotic syndrome in autosomal dominant polycystic kidney disease in children versus adults: Questions

Mohammad Ilyas, Rudolph Well, Aleena Blake & Asad Tolaymat

doi : 10.1007/s00467-022-05597-z

A 7-year-old male, a known case of autosomal dominant polycystic kidney disease (ADPKD), presented to the emergency department with periorbital swelling, abdominal distension with pain, and decreased urine output. During hospitalization, he was found to have abdominal swelling with anasarca.

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Nephrotic syndrome in autosomal dominant polycystic kidney disease in children versus adults: Answers

Mohammad Ilyas, Rudolph Well, Aleena Blake & Asad Tolaymat

doi : 10.1007/s00467-022-05617-y

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An adolescent with abdominal pain and tremors: Questions

Angelina Dixon & Vivian Shih

doi : 10.1007/s00467-022-05724-w

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An adolescent with abdominal pain and tremors: Answers

Angelina Dixon & Vivian Shih

doi : 10.1007/s00467-022-05727-7

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Hyponatremia in a patient with diabetic ketosis: Questions

Mihriban İnözü, Ayşe Derya Buluş & Yüksel Yaşartekin

doi : 10.1007/s00467-022-05699-8

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Hyponatremia in a patient with diabetic ketosis: Answers

Mihriban İnözü, Ayşe Derya Buluş & Yüksel Yaşartekin

doi : 10.1007/s00467-022-05704-0

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Nephrotic syndrome, skin involvement, and chronic lung disease: Questions

Bahriye Atmis, Derya Cevizli, Cagla Cagli, Emel Saribas, Veysel Karakulak, Dilek Ozcan, Beyza Irem Gok, Kivilcim Eren Erdogan, Gulfiliz Gonlusen & Aysun K. Bayazit

doi : 10.1007/s00467-022-05709-9

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Nephrotic syndrome, skin involvement, and chronic lung disease: Answers

Bahriye Atmis, Derya Cevizli, Cagla Cagli, Emel Saribas, Veysel Karakulak, Dilek Ozcan, Beyza Irem Gok, Kivilcim Eren Erdogan, Gulfiliz Gonlusen & Aysun K. Bayazit

doi : 10.1007/s00467-022-05711-1

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The retinal phenotype in primary hyperoxaluria type 2 and 3

Johannes Birtel, Roselie M. Diederen, Philipp Herrmann, Sophie Kaspar, Bodo B. Beck, Sander F. Garrelfs, Bernd Hoppe & Peter Charbel Issa

doi : 10.1007/s00467-022-05765-1

The primary hyperoxalurias (PH1-3) are rare inherited disorders of the glyoxylate metabolism characterized by endogenous overproduction of oxalate.

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Urinary complement proteins are increased in children with IgA vasculitis (Henoch-Schönlein purpura) nephritis

Rachael D. Wright, Julien Marro, Sarah J. Northey, Rachel Corkhill, Michael W. Beresford & Louise Oni

doi : 10.1007/s00467-022-05747-3

Children with immunoglobulin A vasculitis (IgAV Henoch-Schönlein purpura) frequently encounter nephritis (IgAV-N) with 1–2% risk of kidney failure. The pathophysiology of IgAV-N is not fully understood with speculation that complement may contribute. The aim of this study was to identify whether urinary complement proteins are increased in children with IgAV-N.

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Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression

Agnes Trautmann, Svenja Seide, Beata S. Lipska-Ziętkiewicz, Fatih Ozaltin, Maria Szczepanska, Marta Azocar, Augustina Jankauskiene, Alexandra Zurowska, Salim Caliskan, Bassam Saeed, William Morello, Francesco Emma, Mieczyslaw Litwin, Alexey Tsygin, Svitlana Fomina, Anna Wasilewska, Anette Melk, Elisa Benetti, Jutta Gellermann, Natasa Stajic, Marcin Tkaczyk, Sergey Baiko, Larisa Prikhodina, Dagmar Csaicsich, Anna Medynska, Regina Krisam, Heike Breitschwerdt & Franz Schaefer for the PodoNet Consortium

doi : 10.1007/s00467-022-05762-4

The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction.

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Phenotypic characterization of a pediatric cohort with cystinuria and usefulness of newborn screening

Juan Alberto Piñero-Fernández, Carmen Vicente-Calderón, María José Lorente-Sánchez, María Jesús Juan-Fita, José María Egea-Mellado & Inmaculada C. González-Gallego

doi : 10.1007/s00467-022-05732-w

Cystinuria is an inherited metabolic disease involving the defective transport of cystine and the dibasic amino acids in the renal proximal tubules that causes the formation of stones in the urinary system. In our regional child health program, cystinuria is included in newborn metabolic screening. Our objectives are the phenotypic characterization of our cystinuric pediatric cohort and to present our experience in neonatal cystinuria screening.

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Body surface area-based kidney length percentiles misdiagnose small kidneys in children with overweight/obesity

Pierluigi Marzuillo, Gemma Carreras-Badosa, José-María Martínez-Calcerrada, Stefano Guarino, Pier Luigi Palma, Delfina Petrone, Emanuele Miraglia del Giudice, Judit Bassols & Abel López-Bermejo

doi : 10.1007/s00467-022-05718-8

We evaluated the diagnostic performance of height-, age- and body surface area (BSA)-based kidney length (KL) percentiles in the identification of at least one small kidney (KL < 3rd) and in the prediction of reduced estimated glomerular filtration rate (eGFR) and/or elevated blood pressure (BP) in children with and without overweight (OW)/obesity(OB).

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Epidemiology and clinicopathological characteristics of native kidney disease in children in Flanders, Belgium

Dries Deleersnijder, Noël Knops, Dominique Trouet, Koen Van Hoeck, Sevasti Karamaria, Johan Vande Walle, Reiner Mauel, Louise Cools, Gert Meeus, Amélie Dendooven, Johan De Meester, Wim Laurens & Ben Sprangers on behalf of the FCGG collaborative group

doi : 10.1007/s00467-022-05719-7

The Flemish Collaborative Glomerulonephritis Group (FCGG) registry is a population-based kidney biopsy registry that has been including all native kidney biopsies performed in children in Flanders (Belgium), since 2017.

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Hyperuricemia: an unrecognized risk factor for kidney-related sequelae in children with hemolytic uremic syndrome

Alejandro Balestracci, Luciana Meni Battaglia, Ismael Toledo, Laura Beaudoin, Sandra Mariel Martin, Nicolás Ariel Grisolía & Ronald J. Hogg

doi : 10.1007/s00467-022-05753-5

Chronic kidney-related sequelae after STEC-HUS occur in 20–40% of patients. Hyperuricemia (HU) may cause acute and chronic toxicity involving the kidneys. We retrospectively assessed if there was an association between the presence of HU during the acute illness and that of kidney-related sequelae in children with STEC-HUS.

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Health-related quality of life in adults after pediatric kidney failure in Switzerland

Marc-Andrea Heinzelmann, Claudia E. Kuehni, Katharina Roser, Luzius Mader & Guido F. Laube on behalf of the Swiss Pediatric Renal Registry (SPRR) group

doi : 10.1007/s00467-022-05760-6

Little is known about health-related quality of life (HRQoL) in adults after kidney failure during childhood. In this study, we analyzed HRQoL of adults after pediatric kidney failure in Switzerland and investigated socio-demographic and clinical factors associated with HRQoL.

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Risk factors for childhood chronic kidney disease: a population-based study

Michael Geylis, Tara Coreanu, Victor Novack & Daniel Landau

doi : 10.1007/s00467-022-05714-y

The population-based prevalence and risk factors of childhood chronic kidney disease (CKD) are not well-defined. We ascertained childhood CKD epidemiology and perinatal risk factors, based on a large computerized medical record database that covers most of southern Israel’s population.

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Baseline characteristics of participants in the NAVKIDS2 trial: a patient navigator program in children with chronic kidney disease

Chandana Guha, Rabia Khalid, Anita van Zwieten, Anna Francis, Carmel M. Hawley, Allison Jauré, Armando Teixeira-Pinto, Alistair R. Mallard, Amelie Bernier-Jean, David W. Johnson, Deirdre Hahn, Donna Reidlinger, Elaine M. Pascoe, Elizabeth G. Ryan, Fiona Mackie, Hugh J. McCarthy, Jonathan C. Craig, Julie Varghese, Charani Kiriwandeniya, Kirsten Howard, Nicholas G. Larkins, Luke Macauley, Amanda Walker, Martin Howell, Michelle Irving, Patrina H. Y. Caldwell, Reginald Woodleigh, Shilpanjali Jesudason, Simon A. Carter, Sean E. Kennedy, Stephen I. Alexander, Steven McTaggart & Germaine Wong

doi : 10.1007/s00467-022-05772-2

Children with chronic kidney disease (CKD) require multidisciplinary care to meet their complex healthcare needs. Patient navigators are trained non-medical personnel who assist patients and caregivers to overcome barriers to accessing health services through care coordination. This trial aims to determine the effectiveness of a patient navigator program in children with CKD.

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Patient- and caregiver-reported factors associated with school absenteeism in children with chronic kidney disease

Sarah Craven, Barbara H. Brumbach & Kelsey L. Richardson

doi : 10.1007/s00467-022-05780-2

Children with chronic kidney disease (CKD) are at risk for neurocognitive deficits while simultaneously being at risk for chronic school absenteeism (≥ 18 school days per school year). Chronic school absenteeism compounds the negative impacts of CKD on academic achievement.

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Low dialysate sodium in children and young adults on maintenance hemodialysis: a prospective, randomized, crossover study

Olga Caporale, Silvia Consolo, Francesca S. Grassi, Maria R. Grassi, Giuseppe Puccio, Giovanni Montini & Fabio Paglialonga

doi : 10.1007/s00467-022-05792-y

The optimal dialysate sodium concentration (dNa) in children on hemodialysis (HD) is unknown. The aim of this study was to compare the effect on interdialytic weight gain (IDWG) and blood pressure (BP) of a low (135 mmol/l) and standard dNa (138 mmol/l) in children and young adults on maintenance HD.

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Genotype and phenotype analysis and transplantation strategy in children with kidney failure caused by NPHP

Jianyi Li, Xiaojun Su, Huanxi Zhang, Wenrui Wu, Jianming Li, Yanxu Chen, Jun Li, Qian Fu, Chenglin Wu, Xuhui Zhong, Changxi Wang & Longshan Liu

doi : 10.1007/s00467-022-05763-3

Nephronophthisis-related ciliopathies (NPHP-RC) have strong genotype and phenotype heterogeneity, and the transplantation strategy of Boichis syndrome is still controversial. Our purpose was to examine associations of genotype and phenotype in children with NPHP-RC and analyze the transplantation strategies of different phenotypes.

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Emulation of the control cohort of a randomized controlled trial in pediatric kidney transplantation with Real-World Data from the CERTAIN Registry

Christian Patry, Lukas D. Sauer, Anja Sander, Kai Krupka, Alexander Fichtner, Jolanda Brezinski, Yvonne Geissbühler, Elodie Aubrun, Anna Grinienko, Luca Dello Strologo, Dieter Haffner, Jun Oh, Ryszard Grenda, Lars Pape, Rezan Topaloğlu, Lutz T. Weber, Antonia Bouts, Jon Jin Kim, Agnieszka Prytula, Jens König, Mohan Shenoy, Britta Höcker & Burkhard Tönshoff

doi : 10.1007/s00467-022-05777-x

Randomized controlled trials in pediatric kidney transplantation are hampered by low incidence and prevalence of kidney failure in children. Real-World Data from patient registries could facilitate the conduct of clinical trials by substituting a control cohort. However, the emulation of a control cohort by registry data in pediatric kidney transplantation has not been investigated so far.

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Physical performance capacity after pediatric kidney transplant and clinical parameters associated with physical performance capacity

Heidi Mäenpää, Juuso Tainio, Jari Arokoski & Timo Jahnukainen

doi : 10.1007/s00467-022-05758-0

History of chronic kidney disease and kidney transplantation is known to influence physical performance capacity. The aim of this study was to compare the physical performance of pediatric kidney transplant recipients to healthy controls and to find possible correlations between clinical parameters and physical performance capacity.

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Acute kidney injury and diabetic kidney disease in children with acute complications of diabetes

Jolanta Soltysiak, Izabela Krzysko-Pieczka, Anna Gertig-Kolasa, Ewa Mularz, Bogda Skowrońska, Danuta Ostalska-Nowicka & Jacek Zachwieja

doi : 10.1007/s00467-022-05735-7

Diabetic ketoacidosis (DKA) and hyperglycaemia without ketoacidosis are common acute complications of diabetes. Their association with acute kidney injury (AKI) and diabetic kidney disease (DKD) was studied.

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Acute kidney injury requiring kidney replacement therapy in childhood lupus nephritis: a cohort study of the Pediatric Nephrology Research Consortium and Childhood Arthritis and Rheumatology Research Alliance

Brian R. Stotter, Ellen Cody, Hongjie Gu, Ankana Daga, Larry A. Greenbaum, Minh Dien Duong, Alexandra Mazo, Beatrice Goilav, Alexis Boneparth, Mahmoud Kallash, Ahmed Zeid, Wacharee Seeherunvong, Rebecca R. Scobell, Issa Alhamoud, Caitlin E. Carter, Siddharth Shah, Caroline E. Straatmann, Bradley P. Dixon, Jennifer C. Cooper, Raoul D. Nelson, Deborah M. Levy, Hermine I. Brunner, Priya S. Verghese & Scott E. Wenderfer

doi : 10.1007/s00467-022-05775-z

Acute kidney injury (AKI) is common in lupus nephritis (LN) and a risk factor for development of chronic kidney disease. In adults with LN, AKI severity correlates with the incidence of kidney failure and patient survival. Data on AKI outcomes in children with LN, particularly those requiring kidney replacement therapy (KRT), are limited.

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Acute kidney injury during cisplatin therapy and associations with kidney outcomes 2 to 6 months post-cisplatin in children: a multi-centre, prospective observational study

Kelly R. McMahon, Asaf Lebel, Shahrad Rod Rassekh, Kirk R. Schultz, Tom D. Blydt-Hansen, Geoffrey D. E. Cuvelier, Cherry Mammen, Maury Pinsk, Bruce C. Carleton, Ross T. Tsuyuki, Colin J. D. Ross, Louis Huynh, Mariya Yordanova, Frédérik Crépeau-Hubert, Stella Wang, Ana Palijan, Jasmine Lee, Debbie Boyko & Michael Zappitelli for the Applying Biomarkers to Minimize Long-Term Effects of Childhood/Adolescent Cancer Treatment (ABLE) Research Study Group

doi : 10.1007/s00467-022-05745-5

Few studies describe acute kidney injury (AKI) burden during paediatric cisplatin therapy and post-cisplatin kidney outcomes. We determined risk factors for and rate of (1) AKI during cisplatin therapy, (2) chronic kidney disease (CKD) and hypertension 2–6 months post-cisplatin, and (3) whether AKI is associated with 2–6-month outcomes.

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Relapsing and refractory peritoneal dialysis peritonitis caused by Corynebacterium amycolatum

Shameer M. Habeeb, Haneen Yamin, Eva Simkova, Hazem S. Awad, Entesar A. Alhammadi, Loai A. Eid, Rubina Lone & Martin Bitzan

doi : 10.1007/s00467-022-05801-0

Peritonitis is an important complication and cause of morbidity in patients undergoing peritoneal dialysis (PD). Corynebacterium species, often considered skin and mucosal contaminants, are a rare cause of PD-associated peritonitis and have been acknowledged in published guidelines for the diagnosis and treatment of PD peritonitis only over the last decade.

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High prevalence of pathogenic variants in Japanese children with steroid-resistant nephrotic syndrome without edema detected by urine screening program

Shuichiro Fujinaga & Koji Sakuraya

doi : 10.1007/s00467-022-05863-0

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Ascertaining pathogenicity of genetic variants: caution required

Zainab Arslan, Elizabeth Watson & Detlef Bockenhauer

doi : 10.1007/s00467-023-05909-x

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