David T. Selewski, David J. Askenazi, Kianoush Kashani, Rajit K. Basu, Katja M. Gist, Matthew W. Harer, Jennifer G. Jetton, Scott M. Sutherland, Michael Zappitelli, Claudio Ronco, Stuart L. Goldstein & Theresa Ann Mottes
doi : 10.1007/s00467-020-04828-5
Pediatric Nephrology volume 36, pages733–746(2021)
Jiwon M. Lee, Andreas Kronbichler, Jae Il Shin & Jun Oh
doi : 10.1007/s00467-020-04476-9
Pediatric Nephrology volume 36, pages747–761(2021)
Steroid-resistant nephrotic syndrome (SRNS) remains a challenge for paediatric nephrologists. SRNS is viewed as a heterogeneous disease entity including immune-based and monogenic aetiologies. Because SRNS is rare, treatment strategies are individualized and vary among centres of expertise. Calcineurin inhibitors (CNI) have been effectively used to induce remission in patients with immune-based SRNS; however, there is still no consensus on treating children who become either CNI-dependent or CNI-resistant. Rituximab is a steroid-sparing agent for patients with steroid-sensitive nephrotic syndrome, but its efficacy in SRNS is controversial. Recently, several novel monoclonal antibodies are emerging as treatment option, but their efficacy remains to be seen. Non-immune therapies, such as angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers, have been proven efficacious in children with SRNS and are recommended as adjuvant agents. This review summarizes and discusses our current understandings in treating children with idiopathic SRNS.
Ariela Benigni, Simona Buelli & Donald E Kohan
doi : 10.1007/s00467-020-04518-2
Pediatric Nephrology volume 36, pages763–775(2021)
Chronic kidney disease (CKD) is the main cause of end-stage renal disease worldwide arising as a frequent complication of diabetes, obesity, and hypertension. Current therapeutic options, mainly based of inhibition of the renin-angiotensin system (RAS), provide imperfect renoprotection if started at an advanced phase of the disease, and treatments that show or even reverse the progression of CKD are needed. The endothelin (ET) system contributes to the normal renal physiology; however, robust evidence suggests a key role of ET-1 and its cognate receptors, in the progression of CKD. The effectiveness of ET receptor antagonists in ameliorating renal hemodynamics and fibrosis has been largely demonstrated in different experimental models. A significant antiproteinuric effect of ET receptor antagonists has been found in diabetic and non-diabetic CKD patients even on top of RAS blockade, and emerging evidence from ongoing clinical trials highlights their beneficial effects on a wide range of kidney disorders.
Pepe M. Ekulu, Agathe B. Nkoy, Oyindamola C. Adebayo, Orly K. Kazadi, Michel N. Aloni, Fanny O. Arcolino, Rene M. Ngiyulu, Jean-Lambert E. Gini, François B. Lepira, Lamberthus P. Van den Heuvel & Elena N. Levtchenko
doi : 10.1007/s00467-020-04553-z
Pediatric Nephrology volume 36, pages777–788(2021)
Individuals of African origin have an increased risk of developing various progressive chronic kidney diseases (CKD). This risk has been attributed to genetic variants (G1, G2) in apolipoprotein-L1 (APOL1) gene. In the pediatric population, especially in children affected by sickle cell disease (SCD), by human immunodeficiency virus (HIV), or with various glomerular diseases, APOL1 risk variants have been associated with the development of hypertension, albuminuria, and more rapid decline of kidney function. The present review focuses on existing APOL1-related epidemiological data in children with CKD. It also includes data from studies addressing racial disparities in CKD, the APOL1-related innate immunity, and the relationship between APOL1 and CKD and pathogenic pathways mediating APOL1-related kidney injury.
Thurid Ahlenstiel-Grunow & Lars Pape
doi : 10.1007/s00467-020-04522-6
Pediatric Nephrology volume 36, pages789–796(2021)
After pediatric kidney transplantation, immunosuppressive therapy causes an increased risk of severe viral complications, especially from cytomegalovirus (CMV), BK polyomavirus (BKPyV) or Epstein-Barr virus (EBV), and less frequent from adenovirus (ADV). However, suitable predictive markers for the individual outcome of viral infections are missing and the therapeutic management remains a challenge to the success of pediatric kidney transplantation. Virus-specific T cells are known for controlling viral replication and there is growing evidence that virus-specific T cells may serve as a prognostic marker to identify patients at risk for viral complications. This review provides an overview of the usability of virus-specific T cells for improving diagnostic and therapeutic management of viral infections with reference to the necessity of antiviral prophylaxis, timing of pre-emptive therapy, and dosing of immunosuppressive medication after pediatric kidney transplantation. Several studies demonstrated that high levels of virus-specific T cells are associated with decrease of virus load and favorable outcome, whereas lack of virus-specific T cells coincided with virus-induced complications. Accordingly, the additional monitoring of virus-specific T cells aims to personalize the management of antiviral therapy, identify overimmunosuppression, and avoid unnecessary therapeutic interventions. Prospective randomized trials in pediatric kidney recipients comparing standard antiviral and immunosuppressive regimens with T cell-guided therapeutic interventions are needed, before monitoring of virus-specific T cells is implemented in the routine care of pediatric kidney graft recipients.
Jennifer R. Charlton, Edwin J. Baldelomar, Dylan M. Hyatt & Kevin M. Bennett
doi : 10.1007/s00467-020-04534-2
Pediatric Nephrology volume 36, pages797–807(2021)
Studies of human nephron number have been conducted for well over a century and have uncovered a large variability in nephron number. However, the mechanisms influencing nephron endowment and loss, along with the etiology for the wide range among individuals are largely unknown. Advances in imaging technology have allowed investigators to revisit the principles of renal structure and physiology and their roles in the progression of kidney disease. Here, we will review the latest data on the influences impacting nephron number, innovations made over the last 6 years to understand and integrate renal structure and function, and new developments in the tools used to count nephrons in vivo.
Juan C. Kupferman, Marc B. Lande, Stella Stabouli, Dimitrios I. Zafeiriou & Steven G. Pavlakis
doi : 10.1007/s00467-020-04550-2
Pediatric Nephrology volume 36, pages809–823(2021)
Cerebrovascular disease (stroke) is one of the ten leading causes of death in children and adolescents. Multiple etiologies, from arteriopathies to prothrombic states, can cause stroke in youth. In adult stroke, hypertension has been shown to be the single most important modifiable risk factor. Although hypertension has not been strongly identified as a risk factor in childhood stroke to date, there is preliminary evidence that suggests that hypertension may also be associated with stroke in children. In this review, we summarize the literature that may link hypertension to stroke in the young. We have identified a series of barriers and limitations in the fields of pediatric hypertension and pediatric neurology that might explain why hypertension has been overlooked in childhood stroke. We suggest that hypertension may be a relevant risk factor that, alone or in combination with other multiple factors, contributes to the development of stroke in children. Currently, there are no consensus guidelines for the management of post-stroke hypertension in children. Thus, we recommend that blood pressure be assessed carefully in every child presenting with acute stroke in order to better understand the effects of hypertension in the development and the outcome of childhood stroke. We suggest a treatment algorithm to help practitioners manage hypertension after a stroke.
Mieczys?aw Litwin & Zbigniew Ku?aga
doi : 10.1007/s00467-020-04579-3
Pediatric Nephrology volume 36, pages825–837(2021)
Primary hypertension is the dominant form of arterial hypertension in adolescents. Disturbed body composition with, among other things, increased visceral fat deposition, accelerated biological maturation, metabolic abnormalities typical for metabolic syndrome, and increased adrenergic drive constitutes the intermediary phenotype of primary hypertension. Metabolic syndrome is observed in 15–20% of adolescents with primary hypertension. These features are also typical of obesity-related hypertension. Metabolic abnormalities and metabolic syndrome are closely associated with both the severity of hypertension and the risk of target organ damage. However, even though increased body mass index is the main determinant of blood pressure in the general population, not every hypertensive adolescent is obese and not every obese patient suffers from hypertension or metabolic abnormalities typical for metabolic syndrome. Thus, the concepts of metabolically healthy obesity, normal weight metabolically unhealthy, and metabolically unhealthy obese phenotypes have been developed. The risk of hypertension and hypertensive target organ damage increases with exposure to metabolic risk factors which are determined by disturbed body composition and visceral obesity. Due to the fact that both primary hypertension and obesity-related hypertension present similar pathogenesis, the principles of treatment are the same and are focused not only on lowering blood pressure, but also on normalizing body composition and metabolic abnormalities.
Palanisamy Shanmugasundaram Bharathy, Sriram Krishnamurthy, Arumugom Archana, Pediredla Karunakar, Bobbity Deepthi & Bheemanathi Hanuman Srinivas
doi : 10.1007/s00467-020-04692-3
Pediatric Nephrology volume 36, pages839–841(2021)
Palanisamy Shanmugasundaram Bharathy, Sriram Krishnamurthy, Arumugom Archana, Pediredla Karunakar, Bobbity Deepthi & Bheemanathi Hanuman Srinivas
doi : 10.1007/s00467-020-04700-6
Pediatric Nephrology volume 36, pages843–847(2021)
Guillaume Dorval, Alice Hadchouel, Nathalie Biebuyck-Gougé, Henri Giniès, Marion Rabant, Laureline Berteloot, Romain Berthaud, Marina Avramescu, Brigitte Bader-Meunier & Olivia Boyer
doi : 10.1007/s00467-020-04691-4
Pediatric Nephrology volume 36, pages849–851(2021)
Guillaume Dorval, Alice Hadchouel, Nathalie Biebuyck-Gougé, Henri Giniès, Marion Rabant, Laureline Berteloot, Romain Berthaud, Marina Avramescu, Brigitte Bader-Meunier & Olivia Boyer
doi : 10.1007/s00467-020-04698-x
Pediatric Nephrology volume 36, pages853–856(2021)
Joseph Wilbanks, Jessica Hillyer, Faris Hashim, David Sas & Christian Hanna
doi : 10.1007/s00467-020-04710-4
Pediatric Nephrology volume 36, pages857–858(2021)
Joseph Wilbanks, Jessica Hillyer, Faris Hashim, David Sas & Christian Hanna
doi : 10.1007/s00467-020-04711-3
Pediatric Nephrology volume 36, pages859–861(2021)
Andrzej Bade?ski, Omar Bjanid, Marta Bade?ska, Bartosz Chmiela, Piotr Adamczyk, Grzegorz Kudela, Grzegorz Moskal & Maria Szczepa?ska
doi : 10.1007/s00467-020-04707-z
Pediatric Nephrology volume 36, pages863–864(2021)
Andrzej Bade?ski, Omar Bjanid, Marta Bade?ska, Bartosz Chmiela, Piotr Adamczyk, Grzegorz Kudela, Grzegorz Moskal & Maria Szczepa?ska
doi : 10.1007/s00467-020-04709-x
Pediatric Nephrology volume 36, pages865–868(2021)
Marta Cognigni, Marco Pennesi, Giulia Pennesi & Egidio Barbi
doi : 10.1007/s00467-020-04706-0
Pediatric Nephrology volume 36, pages869–870(2021
Marta Cognigni, Marco Pennesi, Giulia Pennesi & Egidio Barbi
doi : 10.1007/s00467-020-04708-y
Pediatric Nephrology volume 36, pages871–872(2021)
?zgür ?zdemir-?im?ek, Belde Kasap-Demir, G?kçen Erfidan, Seçil Arslansoyu-?amlar, Eren Soyalt?n, Demet Alaygut & Fatma Mutluba?
doi : 10.1007/s00467-020-04670-9
Pediatric Nephrology volume 36, pages873–874(2021)
?zgür ?zdemir-?im?ek, Belde Kasap-Demir, G?kçen Erfidan, Seçil Arslansoyu-?amlar, Eren Soyalt?n, Demet Alaygut & Fatma Mutluba?
doi : 10.1007/s00467-020-04675-4
Pediatric Nephrology volume 36, pages875–879(2021)
Priyanka Khandelwal, Mahesh V, Vijay Prakash Mathur, Sumantra Raut, Thenral S. Geetha, Sandhya Nair, Pankaj Hari, Aditi Sinha & Arvind Bagga
doi : 10.1007/s00467-020-04747-5
Pediatric Nephrology volume 36, pages881–887(2021)
Distal renal tubular acidosis (RTA) is typically caused by defects in ATP6V0A4, ATP6V1B1, and SLC4A1, accounting for 60–80% of patients. Genes recently implicated include FOXI1, ATP6V1C2, and WDR72, of which WDR72 is associated with dental enamel defects.
Kazuki Tanaka, Brigitte Adams, Alvaro Madrid Aris, Naoya Fujita, Masayo Ogawa, Stephan Ortiz, Marc Vallee & Larry A. Greenbaum
doi : 10.1007/s00467-020-04774-2
Pediatric Nephrology volume 36, pages889–898(2021)
Atypical hemolytic uremic syndrome (aHUS) is a rare, complement-mediated disease associated with poor outcomes if untreated. Ravulizumab, a long-acting C5 inhibitor developed through minimal, targeted modifications to eculizumab was recently approved for the treatment of aHUS. Here, we report outcomes from a pediatric patient cohort from the ravulizumab clinical trial (NCT03131219) who were switched from chronic eculizumab to ravulizumab treatment.
Yuko Shima, Koichi Nakanishi, Hironobu Mukaiyama, Yu Tanaka, Takuzo Wada, Ryojiro Tanaka, Hiroshi Kaito, Kandai Nozu, Mayumi Sako, Kazumoto Iijima & Norishige Yoshikawa
doi : 10.1007/s00467-020-04772-4
Pediatric Nephrology volume 36, pages899–908(2021)
IgA nephropathy (IgAN) is characterized by predominant mesangial IgA deposition. Some patients with IgAN demonstrate IgA deposition in glomerular peripheral capillaries (cap-IgA). The clinicopathological significance of cap-IgA remains incompletely investigated in children.
Manuela Colucci, Barbara Ruggiero, Alessandra Gianviti, Maria Manuela Rosado, Rita Carsetti, Claudia Bracaglia, Fabrizio De Benedetti, Francesco Emma & Marina Vivarelli
doi : 10.1007/s00467-020-04761-7
Pediatric Nephrology volume 36, pages909–916(2021)
Children with systemic lupus erythematosus (SLE) frequently have kidney involvement. Lupus nephritis sometimes presents alone, without systemic SLE features, representing the so-called full-house nephropathy (FHN). Distinguishing patients with SLE or FHN has therapeutic and prognostic implications.
Johannes Hofer, Magdalena Riedl Khursigara, Markus Perl, Thomas Giner, Alejandra Rosales, Gerard Cortina, Siegfied Waldegger, Therese Jungraithmayr & Reinhard Würzner
doi : 10.1007/s00467-020-04751-9
Pediatric Nephrology volume 36, pages917–925(2021)
The complement factor H antibody (CFH-Ab)–associated hemolytic uremic syndrome (HUS) forms a distinct subgroup within the complement-mediated HUS disease spectrum. The autoimmune nature of this HUS subgroup implies the potential benefit of a targeted immunosuppressive therapy. Data on long-term outcome are scarce.
Paul Miller, Andrew Y. Xiao, Vanderlene L. Kung, Richard K. Sibley, John P. Higgins, Neeraja Kambham, Vivek Charu, Colin Lenihan, Amanda M. Uber, Elizabeth M. Talley, Neiha Arora, Vighnesh Walavalkar, Zoltan G. Laszik, Cynthia C. Nast & Megan L. Troxell
doi : 10.1007/s00467-020-04763-5
Pediatric Nephrology volume 36, pages927–937(2021)
Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a glomerular disease defined by non-organized glomerular deposits of heavy and light chain–restricted immunoglobulin and is rarely reported in children.
Seha Kamil Sayg?l?, Elif Alt?nay K?rl?, Emre Ta?demir, Nur Canpolat, Salim ?al??kan, Lale Sever, Zübeyr Talat & Bülent ?nal
doi : 10.1007/s00467-020-04781-3
Pediatric Nephrology volume 36, pages939–944(2021)
We evaluated the risk factors for the requirement of surgical intervention in infants with nephrolithiasis.
Selasie Goka, Lawrence Copelovitch & Daniella Levy Erez
doi : 10.1007/s00467-020-04748-4
Pediatric Nephrology volume 36, pages945–951(2021)
Alport syndrome (AS) is a multisystem condition which can result in progressive kidney disease, hearing loss, and ocular changes. X-linked inheritance is observed in 85% of affected individuals. As a result, most prior studies have focused on males. Girls with AS can also be symptomatic although historically thought to have few clinical manifestations in childhood. The objective of the study was to describe the clinical presentation and course of females with AS.
Osamu Uemura, Kenji Ishikura, Tetsuji Kaneko, Daishi Hirano, Yuko Hamasaki, Masao Ogura, Naoaki Mikami, Yoshimitsu Gotoh, Takeshi Sahashi, Naoya Fujita, Masaki Yamamoto, Satoshi Hibino, Masaru Nakano, Yasuhiro Wakano & Masataka Honda
doi : 10.1007/s00467-020-04791-1
Pediatric Nephrology volume 36, pages953–960(2021)
Developmental programming of chronic kidney disease (CKD) in young adults is linked to preterm birth and intrauterine growth restriction (IUGR). Which confers a higher risk of progression to chronic kidney damage in children with very low birth weight (VLBW; born weighing < 1500 g): prematurity or IUGR?
Grace Truong, Sarah Kamal, Joshua A Samuels & Cynthia S Bell
doi : 10.1007/s00467-020-04655-8
Pediatric Nephrology volume 36, pages961–967(2021)
Hypertension (HTN) is common in children and often associated with pathologic progression to end organ damage, specifically left ventricular hypertrophy (LVH).
Qian Fu, Zhi Chen, Jianfeng Fan, Chen Ling, Xiaoman Wang, Xiaorong Liu & Ying Shen
doi : 10.1007/s00467-020-04735-9
Pediatric Nephrology volume 36, pages969–976(2021)
The value of lung ultrasound in adult hemodialysis has been confirmed. The determination of dry weight in children remains challenging. This study explores the usefulness of lung ultrasound in assessing fluid volume change and the possibility of pulmonary ultrasound as a method to monitor dry weight in pediatric dialysis patients.
Karen Vanderstraeten, Rani De Pauw, Noël Knops, Antonia Bouts, Karlien Cransberg, Amina El Amouri, Ann Raes & Agnieszka Prytu?a
doi : 10.1007/s00467-020-04796-w
Pediatric Nephrology volume 36, pages977–986(2021)
Hyperparathyroidism persists in up to 50% of pediatric kidney transplant recipients. The aims of this study were to describe the evolution of parathyroid hormone (PTH) in the first year after transplantation and to identify factors associated with hyperparathyroidism.
Or Golan, Roger Dyer, Graham Sinclair & Tom Blydt-Hansen
doi : 10.1007/s00467-020-04797-9
Pediatric Nephrology volume 36, pages987–993(2021)
Oxythiamine is a uremic toxin that acts as an antimetabolite to thiamine and has been associated with cases of Shoshin beriberi syndrome in adults. We sought to identify whether surgical stress and ischemia/reperfusion injury may precipitate functional thiamine deficiency in children peritransplant.
Christina L. Nelms, Vanessa Shaw, Larry A. Greenbaum, Caroline Anderson, An Desloovere, Dieter Haffner, Michiel J. S. Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, Lesley Rees, José Renken-Terhaerdt, Jetta Tuokkola, Johan Vande Walle, Rukshana Shroff & Bradley A. Warady
doi : 10.1007/s00467-020-04852-5
Pediatric Nephrology volume 36, pages995–1010(2021)
In children with kidney diseases, an assessment of the child’s growth and nutritional status is important to guide the dietary prescription. No single metric can comprehensively describe the nutrition status; therefore, a series of indices and tools are required for evaluation. 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. Herein, we present CPRs for nutritional assessment, including measurement of anthropometric and biochemical parameters and evaluation of dietary intake. 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. Audit and research recommendations are provided. The CPRs will be periodically audited and updated by the PRNT.
Ian Hewitt & Giovanni Montini
doi : 10.1007/s00467-020-04573-9
Pediatric Nephrology volume 36, pages1011–1017(2021)
Vesico-ureteral reflux (VUR) has long been recognized as associated with urinary tract infections (UTIs), renal scarring, and chronic kidney disease (CKD). The concept of “reflux nephropathy” was born, whereby the VUR was considered the culprit, predisposing to recurrent UTIs and providing a conduit whereby the infection could ascend to the kidneys resulting in scarring and destruction. The more severe grades of reflux were thought to place the young child at particular risk of CKD. The question being asked in this pro/con debate is whether VUR is indeed the culprit responsible for a significant proportion of children with CKD, a number of whom progress to end-stage kidney failure (ESKF), and is thus important to find and treat, or is it an innocent bystander associated with CKD and ESKF but not the cause. We believe the latter and will present convincing evidence supported by large scale prospective randomized controlled trials that VUR is not the ogre it was thought to be and is not important to find following a UTI (with some exceptions).
Mitra Basiratnia, Dorna Derakhshan, Babak Shirazi Yeganeh & Ali Derakhshan
doi : 10.1007/s00467-021-04944-w
Pediatric Nephrology volume 36, pages1019–1023(2021)
Coronavirus disease 2019 (COVID-19) is thought to cause kidney injury via a variety of mechanisms. The most common reported kidney injury following COVID-19 infection is acute tubular injury (ATI); however, the procoagulant state induced by the virus may also damage the kidneys.
Marina Giralt, Sara Chocron, Roser Ferrer & Gema Ariceta
doi : 10.1007/s00467-020-04906-8
Pediatric Nephrology volume 36, pages1025–1028(2021)
Primary hypophosphatemic syndromes are a heterogeneous group of rare diseases. In recent years, fibroblast growth factor 23 (FGF23) has been postulated as a useful tool for differential diagnosis of hypophosphatemic rickets characterized by impaired renal phosphate reabsorption. This study aimed to investigate the utility of FGF23 to discriminate between X-linked hypophosphatemic rickets (XLH), an FGF23-driven disease, from other causes of renal phosphate wasting such as Fanconi syndrome (FS), a generalized dysfunction of the proximal tubule unrelated to FGF23.
Ester Conversano & Marco Pennesi
doi : 10.1007/s00467-020-04871-2
Pediatric Nephrology volume 36, page1029(2021)
Clifford E. Kashtan & Oliver Gross
doi : 10.1007/s00467-020-04872-1
Pediatric Nephrology volume 36, page1031(2021)
Kazuki Tanaka, Brigitte Adams, Alvaro Madrid Aris, Naoya Fujita, Masayo Ogawa, Stephan Ortiz, Marc Vallee & Larry A. Greenbaum
doi : 10.1007/s00467-020-04874-z
Pediatric Nephrology volume 36, page1033(2021)
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