Detlef Bockenhauer, Nine V. A. M. Knoers & Daniel G. Bichet
doi : 10.1007/s00467-022-05815-8
Volume 38, issue 4, April 2023
Lucy Plumb & Stephen D. Marks
Sushmita Banerjee, Jayati Sengupta & Surupa Basu
doi : 10.1007/s00467-022-05698-9
Hypovitaminosis D has been reported to be common in chronic kidney disease (CKD) as well as in proteinuric disorders. We reviewed available evidence to assess clinically relevant effects of low vitamin D status and native vitamin D (NVD) therapy, in pediatric renal diseases.
Amelia K. Le Page, Naganandini Nagasundaram, Ari E. Horton & Lilian M. Johnstone
doi : 10.1007/s00467-022-05721-z
Transthoracic echocardiography is commonly used to identify structural and functional cardiac abnormalities that can be prevalent in childhood chronic kidney failure (KF). Left ventricular mass (LVM) increase is most frequently reported and may persist post-kidney transplant especially with hypertension and obesity.
Sian E. Piret & Sandeep K. Mallipattu
doi : 10.1007/s00467-022-05748-2
The kidney, and in particular the proximal tubule (PT), has a high demand for ATP, due to its function in bulk reabsorption of solutes.
Priyanka Khandelwal, Srinivasavaradan Govindarajan & Arvind Bagga
doi : 10.1007/s00467-022-05769-x
Lupus nephritis (LN) has variable prevalence, severity, and outcomes across the world.
Machi Kaneko & Shaun W. Jackson
doi : 10.1007/s00467-022-05670-7
Childhood-onset systemic lupus erythematosus (SLE) is characterized by increased rates of kidney involvement, termed lupus nephritis.
Bahar Büyükkaragöz & Sevcan A. Bakkaloğlu
doi : 10.1007/s00467-022-05668-1
Serum osmolality is the sum of the osmolalities of every single dissolved particle in the blood such as sodium and associated anions, potassium, glucose, and urea. Under normal conditions, serum sodium concentration is the major determinant of serum osmolality.
Emre Leventoğlu, Bahriye Uzun Kenan, Eylül Pınar Çakır, Zeynep İlkşen Hocoğlu, Tuğba Şişmanlar Eyüboğlu, Bahar Büyükkaragöz, Ayşe Tana Aslan & Oğuz Söylemezoğlu
Emre Leventoğlu, Bahriye Uzun Kenan, Eylül Pınar Çakır, Zeynep İlkşen Hocoğlu, Tuğba Şişmanlar Eyüboğlu, Bahar Büyükkaragöz, Ayşe Tana Aslan & Oğuz Söylemezoğlu
Irina Gavryutina, Renee Bargman, Asma Shaoba, Hussam Alharash & Anil Mongia
Irina Gavryutina, Renee Bargman, Asma Shaoba, Hussam Alharash & Anil Mongia
Sevgin Taner, Elif Afat Turgut, Elif Akkaya, Metin Cil & Umit Celik
Sevgin Taner, Elif Afat Turgut, Elif Akkaya, Metin Cil & Umit Celik
Darshan B. Patel, Amanda C. Farris, Christian Hanna & Faris Hashim
doi : 10.1007/s00467-022-05657-4
A 12-year-old female with a past medical history of serine protease 1 (PRSS1)-related hereditary pancreatitis and multiple admissions for recurrent acute pancreatitis (AP) was admitted to the hospital with abdominal pain.
Darshan B. Patel, Amanda C. Farris, Christian Hanna & Faris Hashim
Zainab Arslan, Hazel Webb, Emma Ashton, Becky Foxler, Kjell Tullus, Aoife Waters & Detlef Bockenhauer
doi : 10.1007/s00467-022-05569-3
Primary steroid resistant nephrotic syndrome (SRNS) is thought to have either genetic or immune-mediated aetiology. Knowing which children to screen for genetic causes can be difficult.
Kentaro Nishi, Osamu Uemura, Ryoko Harada, Masaki Yamamoto, Yusuke Okuda, Kenichiro Miura, Yoshimitsu Gotoh, Tomoo Kise, Daishi Hirano, Yuko Hamasaki, Naoya Fujita, Toru Uchimura, Takeshi Ninchoji, Tetsuya Isayama, Riku Hamada, Koichi Kamei, Tetsuji Kaneko & Kenji Ishikura on behalf of the Pediatric CKD Study Group in Japan in conjunction with the Committee of Measures for Pediatric CKD of the Japanese Society of Pediatric Nephrology
doi : 10.1007/s00467-022-05703-1
Severe congenital anomalies of the kidney and urinary tract (CAKUT) progress to infantile kidney failure with replacement therapy (KFRT). Although prompt and precise prediction of kidney outcomes is important, early predictive factors for its progression remain incompletely defined.
Evgenia Gurevich, Yael Borovitz, Shelli Levi, Sharon Perlman & Daniel Landau
doi : 10.1007/s00467-022-05740-w
Idiopathic infantile hypercalcemia (IIH) etiologies include pathogenic variants in CYP24A1, leading to increased 1,25(OH)2 D, hypercalciuria and suppressed parathyroid hormone (PTH), and in SLC34A1 and SLC34A3, leading to the same metabolic profile via increased phosphaturia. IIH has not been previously described in CKD due to kidney hypodysplasia (KHD).
Wesley Hayes, David J. Sas, Daniella Magen, Hadas Shasha-Lavsky, Mini Michael, Anne-Laure Sellier-Leclerc, Julien Hogan, Taylor Ngo, Marianne T. Sweetser, John M. Gansner, Tracy L. McGregor & Yaacov Frishberg
doi : 10.1007/s00467-022-05684-1
Primary hyperoxaluria type 1 (PH1) is a rare genetic disease that causes progressive kidney damage and systemic oxalosis due to hepatic overproduction of oxalate.
Nori J. L. Smeets, Esther M. M. Teunissen, Kim van der Velden, Maurice J. P. van der Burgh, Demi E. Linders, Elodie Teesselink, Dirk-Jan A. R. Moes, Camilla Tøndel, Rob ter Heine, Arno van Heijst, Michiel F. Schreuder & Saskia N. de Wildt
doi : 10.1007/s00467-022-05651-w
Acute kidney injury (AKI) and augmented renal clearance (ARC), both alterations of the glomerular filtration rate (GFR), are prevalent in critically ill children and neonates. AKI and ARC prevalence estimates are based on estimation of GFR (eGFR) using serum creatinine (SCr), which is known to be inaccurate.
Koichi Kamei, Masao Ogura, Mai Sato, Kentaro Nishi, Kensuke Shoji, Takanori Funaki, Chikara Ogimi & Shuichi Ito
doi : 10.1007/s00467-022-05633-y
As there are no large-scale reports of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) mRNA vaccination in patients with nephrotic syndrome using immunosuppressive agents, we conducted the prospective study.
Joyita Bharati, Karalanglin Tiewsoh, Lesa Dawman, Tarvinder Singh, Ujjwal Gorsi, Arun Prabhahar Rajarajen, Aakanksha Sharma, Rahul Chanchlani, Raja Ramachandran & Harbir Singh Kohli
doi : 10.1007/s00467-022-05693-0
Reports on long-term complications of childhood-onset nephrotic syndrome (NS), such as obesity, osteoporosis, growth failure, and hypertension, are mostly from developed countries not representing South Asian ethnicities
Shiying Liu, William S. Bush, Kristy Miskimen, Agustin Gonzalez-Vicente, Jessica N. Cooke Bailey, Ioanna Konidari, Jacob L. McCauley, John R. Sedor, John F. O’Toole & Dana C. Crawford
doi : 10.1007/s00467-022-05696-x
Minimal change disease (MCD) is the major cause of childhood idiopathic nephrotic syndrome, which is characterized by massive proteinuria and debilitating edema. Proteinuria in MCD is typically rapidly reversible with corticosteroid therapy, but relapses are common, and children often have many adverse events from the repeated courses of immunosuppressive therapy.
Tracy E. Hunley, Guillermo Hidalgo, Kar Hui Ng, Yoko Shirai, Kenichiro Miura, Hostensia M. Beng, Qiang Wu, Motoshi Hattori & William E. Smoyer
doi : 10.1007/s00467-022-05637-8
Nephrotic syndrome (NS) is a common pediatric kidney disease, yet current treatments for complicated NS are only partially effective and have significant toxicity. There is no Food and Drug Administration (FDA)- or European Medicines Agency (EMA)-approved safe and effective treatment for NS.
Benedetta Chiodini, Anita Sofia Bellotti, William Morello, Chiara Bulgaro, Ilaria Farella, Mario Giordano, Giovanni Montini, Khalid Ismaili & Karl Martin Wissing
doi : 10.1007/s00467-022-05702-2
Viral upper respiratory tract infections trigger nephrotic syndrome relapses. Few data exist on the impact of the SARS-CoV-2 pandemic on the risk of relapse in children with idiopathic nephrotic syndrome (INS).
Weiran Zhou, Hui Wang, Shuzhen Sun, Ying Shen, Xuemei Liu, Junhui Zhen, Hongxia Zhang, Fan Duan, Yanyan Pan & Linlin Dong
doi : 10.1007/s00467-022-05725-9
C4d may be used as a marker to evaluate the condition and prognosis of adults with IgA nephropathy, but there have been few studies of children with IgA nephropathy.
William Morello, Federica Alessandra Vianello, Chiara Bulgaro & Giovanni Montini
doi : 10.1007/s00467-022-05736-6
Children with underlying kidney diseases display a mild course of SARS-CoV-2 infection, but they only accounted for a minority of cases until the spread of the Omicron variant. Nonetheless, idiopathic nephrotic syndrome (INS) has been advocated as a predictor of worse outcome.
Hakan Kisaoglu, Ozge Baba & Mukaddes Kalyoncu
doi : 10.1007/s00467-022-05742-8
Lupus low disease activity state (LLDAS) is a treatment target for patients with SLE and is associated with decreased risk for severe flare and new damage.
Jenelle Cocorpus, Julie Jun, Abby Basalely, Laura Castellanos, Pamela Singer, Rachel Frank, Olivia Bullaro, Shari Gurusinghe & Christine B. Sethna
doi : 10.1007/s00467-022-05619-w
Pediatric chronic disease impacts the affected child and their family structure. There is limited literature investigating the psychosocial impact of nephrotic syndrome on families.
�ukasz Obrycki, Jędrzej Sarnecki, Marianna Lichosik, Małgorzata Sopińska, Małgorzata Placzyńska, Małgorzata Stańczyk, Julia Mirecka, Agnieszka Wasilewska, Maciej Michalski, Weronika Lewandowska, Tadeusz Dereziński, Michał Pac, Natalia Szwarc, Karol Annusewicz, Viktoriia Rekuta, Karolis Ažukaitis, Andrius Čekuolis, Aldona Wierzbicka, Augustina Jankauskiene, Bolesław Kalicki, Katarzyna Jobs, Marcin Tkaczyk, Janusz Feber & Mieczysław Litwin
doi : 10.1007/s00467-022-05667-2
Kidney size evaluation is an essential examination in pediatric nephrology. While body length/height is the best predictor of kidney length, age-based and body surface area (BSA)–based normative values may be useful in clinical practice or research. This study aimed to establish ultrasound-based kidney length lambda-mu-sigma (LMS) percentiles by age and BSA in healthy children.
Carla Galvez, Paola Krall, Alejandro Rojas, Jun Oh & Francisco Cano
doi : 10.1007/s00467-022-05694-z
Hemolytic uremic syndrome secondary to Shiga-toxin-producing Escherichia coli infection (STEC-HUS) generally shows a favorable outcome. Few cases develop extra-renal complications, since neurological involvement is an important cause of morbidity and mortality.
Katarina Robertsson Grossmann, Liya Vishnevskaya, Sandra Diaz Ruiz, Karolina Kublickiene, Peter Bárány, Mats Blennow & Milan Chromek
doi : 10.1007/s00467-022-05705-z
Acute kidney injury (AKI) remains common among infants with hypothermia-treated hypoxic-ischaemic encephalopathy (HIE). Little is known about long-term kidney outcomes following hypothermia treatment. We recently reported that 21% of survivors of hypothermia-treated HIE had decreased estimated glomerular filtration rate (eGFR) based on plasma creatinine in early adolescence.
Deniz Karakaya, Evrim Kargın Çakıcı, Fatma Yazılıtaş, Tülin Güngör, Evra Çelikkaya, Esra Bağlan & Mehmet Bülbül
doi : 10.1007/s00467-022-05754-4
The most well-known and common long-term complication in children with renal parenchymal scarring (RPS) is hypertension (HT). The present study aimed to evaluate the presence of HT in children with RPS based on ambulatory blood pressure monitoring (ABPM) and to compare the patients’ blood pressure (BP) to that in healthy controls matched for age, gender, and BMI.
Katharina Monika Heuchel, Fabian Ebach, Ebru Aileen Alsat, Heiko Reutter, Andreas Mueller & Alina Christine Hilger
doi : 10.1007/s00467-022-05713-z
Intrauterine growth restriction (IUGR) has been associated with changes in kidney anatomy, nephrogenesis and the vascular system, resulting in secondary arterial hypertension and kidney damage in adulthood. Here, we compare routine clinical and metabolic parameters between IUGR and non-IUGR study participants in the neonatal and early infant period.
Ramnath Balasubramanian, Rachel Folwell, Arran Wheatley, Heidi Ramsey, Carmen Barton, Christopher J. D. Reid & Manish D. Sinha
doi : 10.1007/s00467-022-05673-4
We developed a paediatric haemodialysis trigger tool (pHTT) for application per haemodialysis (HD) session in children receiving intermittent in-centre HD and systematically monitored adverse events.
Rajiv Sinha, Rana Saha, Deblina Dasgupta, Niladri Bose, Shamik Ghosh, Ashok Modi, Bikramjit Das, Mignon McCulloch & Yincent Tse
doi : 10.1007/s00467-022-05700-4
Despite its utility, uncertainty exists on the feasibility of acute peritoneal dialysis (PD) and optimal PD catheter type for very low birth weight (VLBW < 1500 g) and extremely low birth weight (ELBW < 1000 g) infants.
Ann Christin Gjerstad, Rannveig Skrunes, Camilla Tøndel, Anders Åsberg, Sabine Leh, Claus Klingenberg, Henrik Døllner, Clara Hammarstrøm & Anna Kristina Bjerre
doi : 10.1007/s00467-022-05706-y
There is scarce information on biopsy-verified kidney disease in childhood and its progression to chronic kidney disease stage 5 (CKD 5). This study aims to review biopsy findings in children, and to investigate risk of kidney replacement therapy (KRT).
Amy J. Kogon, Jennifer Roem, Michael F. Schneider, Mark M. Mitsnefes, Babette S. Zemel, Bradley A. Warady, Susan L. Furth & Nancy M. Rodig
doi : 10.1007/s00467-022-05655-6
Obesity is prevalent among children with chronic kidney disease (CKD) and is associated with cardiovascular disease and reduced quality of life. Its relationship with pediatric CKD progression has not been described.
Naoaki Mikami, Riku Hamada, Ryoko Harada, Yuko Hamasaki, Kenji Ishikura, Masataka Honda & Hiroshi Hataya
doi : 10.1007/s00467-022-05720-0
Icodextrin has a lower absorption rate, and icodextrin peritoneal dialysate contributes to more water removal than glucose dialysate in patients with high peritoneal permeability. There are limited data on icodextrin dialysate use in children.
Olga Charnaya, Laura Zeiser, Dolev Yisar, Aviva Goldberg, Dorry L. Segev, Allan Massie, Jacqueline Garonzik-Wang & Priya Verghese
doi : 10.1007/s00467-022-05676-1
Disparities in pediatric kidney transplantation (KT) result in reduced access and worse outcomes for minority children. We assessed the impact of recent systems changes on these disparities.
Eytan Kaplan, Gili Kadmon, Elhanan Nahum, Hadas Alfandary, Orly Haskin & Avichai Weissbach
doi : 10.1007/s00467-022-05691-2
Blood pressure (BP) monitoring following pediatric kidney transplantation is essential for optimizing graft perfusion. Differences between invasive BP and noninvasive BP (NIBP) measurements are sometimes considerable. We aimed to assess agreement between invasive BP and NIBP in pediatric patients after kidney transplantation and compare with measurements obtained by systolic Doppler with manual sphygmomanometer as a reference technique.
Natalie Wyatt, Karen Norman, Kate Ryan, Mohan Shenoy, Michal Malina, Lasanthi Weerassoriya, Jack Merritt, Ramnath Balasubramanian & Wesley Hayes
doi : 10.1007/s00467-022-05690-3
Intravenous fluid administration is an essential part of perioperative care for children receiving a kidney transplant. There is a paucity of evidence to guide optimal perioperative fluid management.
Ashley Montgomery, Cameron Goff, Bolatito Adeyeri, Liam D. Ferreira, Spoorthi Kamepalli, Jake Lynn, Nhu Thao Nguyen Galvan, Poyyapakkam R. Srivaths, Eileen D. Brewer & Abbas Rana
doi : 10.1007/s00467-022-05722-y
The Kidney Donor Risk Index (KDRI) by Rao et al. was developed to measure the quality of kidney allografts.
Rizky Indrameikha Sugianto, Karen Ostendorf, Elena Bauer, Jeannine von der Born, Jun Oh, Markus J. Kemper, Rainer Buescher, Bernhard M. W. Schmidt, Nima Memaran & Anette Melk
doi : 10.1007/s00467-022-05611-4
Pulse wave velocity (PWV) is a measure of arterial stiffness. We investigated PWV and blood pressure (BP) to determine to what extent BP changes contribute to arterial stiffness, and secondly, to identify influencing factors on BP in children after kidney transplantation.
David J. Askenazi, Brian A. Halloran, Patrick J. Heagerty, Robert H. Schmicker, Sandra E. Juul, Sangeeta Hingorani, Stuart L. Goldstein on behalf of the PENUT Trial Consortium
doi : 10.1007/s00467-022-05688-x
Acute kidney injury (AKI) is common and is associated with poor clinical outcomes in premature neonates. Urine biomarkers hold the promise to improve our understanding and care of patients with kidney disease
Kevin A. Pettit, David T. Selewski, David J. Askenazi, Rajit K. Basu, Brian C. Bridges, David S. Cooper, Geoffrey M. Fleming, Jason Gien, Stephen M. Gorga, Jennifer G. Jetton, Eileen C. King, Heidi J. Steflik, Matthew L. Paden, Rashmi D. Sahay, Michael Zappitelli & Katja M. Gist
doi : 10.1007/s00467-022-05708-w
Acute kidney injury (AKI) and fluid overload (FO) are associated with poor outcomes in children receiving extracorporeal membrane oxygenation (ECMO). Our objective is to evaluate the impact of AKI and FO on pediatric patients receiving ECMO for cardiac pathology.
Tara M. Neumayr, Jeffrey A. Alten, David K. Bailly, Priya N. Bhat, Katie L. Brandewie, J. Wesley Diddle, Muhammad Ghbeis, Catherine D. Krawczeski, Kenneth E. Mah, Tia T. Raymond, Garrett Reichle, Huaiyu Zang, David T. Selewski & NEPHRON Investigators
doi : 10.1007/s00467-022-05697-w
Fluid overload associates with poor outcomes after neonatal cardiac surgery, but consensus does not exist for the most clinically relevant method of measuring fluid balance (FB). While weight change-based FB (FB-W) is standard in neonatal intensive care units, weighing infants after cardiac surgery may be challenging.
Abbie Bauer, Kristen Carlin, Stephen M. Schwartz, Meera Srikanthan, Monica Thakar, Lauri M. Burroughs, Jodi Smith, Sangeeta Hingorani & Shina Menon
doi : 10.1007/s00467-022-05731-x
Acute kidney injury (AKI) is common after hematopoietic cell transplantation (HCT) and is associated with poorer outcomes. Risk factors for AKI after pediatric HCT are not fully understood. The study objective was to assess unique risk factors for AKI in the HCT population and evaluate post-HCT AKI patterns.
Noureddin Nourbakhsh & Nadine Benador
doi : 10.1007/s00467-022-05829-2
Assessment of fluid status in neonatal dialysis has largely focused on traditional tools including clinical assessment, serial weights, and blood pressure (BP) measurements.
Neslihan Cicek, Harika Alpay, Sercin Guven, Ceren Alavanda, Özde Nisa Türkkan, Serim Pul, Ece Demirci, Nurdan Yıldız, Pınar Ata & Ibrahim Gokce
doi : 10.1007/s00467-022-05730-y
Cubilin is one of the receptor proteins responsible for reabsorption of albumin in proximal tubules and is encoded by the CUBN gene. We aimed to evaluate clinical and genetic characterization of six patients with proteinuria who had CUBN mutations.
Chelsea Young, Talia Papiro & Jason H. Greenberg
doi : 10.1007/s00467-022-05712-0
Paxlovid (nirmatrelvir/ritonavir) is a novel drug available under emergency use authorization by the Food and Drug Administration for the treatment of COVID-19 infection. Tacrolimus, a calcineurin inhibitor, is commonly used as an immunosuppressant medication in children with kidney transplants.
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