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IgA vasculitis or Henoch-Sch?nlein purpura: genetics and beyond
Marija Jelusic & Mario Sestan
doi : 10.1007/s00467-021-04987-z
Pediatric Nephrology volume 36, pages2149–2153 (2021)
Metabolic programming of nephron progenitor cell fate
Giovane G Tortelote, Mariel Col?n-Leyva & Zubaida Saifudeen
doi : 10.1007/s00467-020-04752-8
Pediatric Nephrology volume 36, pages2155–2164 (2021)
Metabolic pathways are one of the first responses at the cellular level to maternal/fetal interface stressors. Studies have revealed the previously unrecognized contributions of intermediary metabolism to developmental programs. Here, we provide an overview of cellular metabolic pathways and the cues that modulate metabolic states. We discuss the developmental and physiological implications of metabolic reprogramming and the key role of metabolites in epigenetic and epiproteomic modifications during embryonic development and with respect to kidney development and nephrogenesis.
Novel insights in the genetics of steroid-sensitive nephrotic syndrome in childhood
Stephanie Dufek-Kamperis, Robert Kleta, Detlef Bockenhauer, Daniel Gale & Mallory L. Downie
doi : 10.1007/s00467-020-04780-4
Pediatric Nephrology volume 36, pages2165–2175 (2021)
Steroid-sensitive nephrotic syndrome (SSNS) is the most common form of nephrotic syndrome in childhood and there is growing evidence that genetics play a role in the susceptibility for the disease. Familial clustering has been observed and has led to several studies on familial SSNS trying to identify a monogenic cause of the disease. Until now, however, none of these have provided convincing evidence for Mendelian inheritance. This and the phenotypic variability within SSNS suggest a complex inheritance pattern, where multiple variants and interactions between those and the environment play roles in disease development. Genome-wide association studies (GWASs) have been used to investigate this complex disease. We herein highlight new insights in the genetics of the disease provided by GWAS and identify how these insights fit into our understanding of the pathogenesis of SSNS.
Policy in pediatric nephrology: successes, failures, and the impact on disparities
Jill R. Krissberg, Scott M. Sutherland, Lisa J. Chamberlain & Paul H. Wise
doi : 10.1007/s00467-020-04755-5
Pediatric Nephrology volume 36, pages2177–2188 (2021)
Pediatric nephrology has a history rooted in pediatric advocacy and has made numerous contributions to child health policy affecting pediatric kidney diseases. Despite this progress, profound social disparities remain for marginalized and socially vulnerable children with kidney disease. Different risk factors, such as genetic predisposition, environmental factors, social risk factors, or health care access influence the emergence and progression of pediatric kidney disease, as well as access to life-saving interventions, leading to disparate outcomes. This review will summarize the breadth of literature on social determinants of health in children with kidney disease worldwide and highlight policy-based initiatives that mitigate the adverse social factors to generate greater equity in pediatric kidney disease.
Childhood HIV-associated nephropathy: 36 years later
Patricio E. Ray, Jinliang Li, Jharna R. Das & Pingtao Tang
doi : 10.1007/s00467-020-04756-4
Pediatric Nephrology volume 36, pages2189–2201 (2021)
HIV-associated nephropathy (HIVAN) predominantly affects people of African ancestry living with HIV who do not receive appropriate antiretroviral therapy (ART). Childhood HIVAN is characterized by heavy proteinuria and decreased kidney function. Kidney histology shows mesangial expansion, classic or collapsing glomerulosclerosis, and microcystic renal tubular dilatation leading to kidney enlargement. The pathogenesis of HIVAN involves the kidney recruitment of inflammatory cells and the infection of kidney epithelial cells. In addition, both viral and genetic factors play key roles in this disease. Modern ART has improved the outcome and decreased the prevalence of childhood HIVAN. However, physicians have had modest success providing chronic ART to children and adolescents, and we continue to see children with HIVAN all over the world. This article discusses the progress made during the last decade in our understanding of the pathogenesis and treatment of childhood HIVAN, placing particular emphasis on the mechanisms that mediate the infection of kidney epithelial cells, and the roles of cytokines, the HIV-Tat gene, and the Apolipoprotein-1 (APOL1) gene risk variants in this disease. In view of the large number of children living with HIV at risk of developing HIVAN, better prevention and treatment programs are needed to eradicate this disease.
Hepatorenal syndrome in children: a review
Priscila Menezes Ferri Liu, Sarah Tayn? de Carvalho, Pollyanna Faria Fradico, Maria Luiza Barreto Cazumb?, Ramon Gustavo Bernardino Campos & Ana Cristina Sim?es e Silva
doi : 10.1007/s00467-020-04762-6
Pediatric Nephrology volume 36, pages2203–2215 (2021)
Hepatorenal syndrome (HRS) occurs in patients with cirrhosis or fulminant hepatic failure and is a kind of pre-renal failure due to intense reduction of kidney perfusion induced by severe hepatic injury. While other causes of pre-renal acute kidney injury (AKI) respond to fluid infusion, HRS does not. HRS incidence is 5% in children with chronic liver conditions before liver transplantation. Type 1 HRS is an acute and rapidly progressive form that often develops after a precipitating factor, including gastrointestinal bleeding or spontaneous bacterial peritonitis, while type 2 is considered a slowly progressive form of kidney failure that often occurs spontaneously in chronic ascites settings. HRS pathogenesis is multifactorial. Cirrhosis causes portal hypertension; therefore, stasis and release of vasodilator substances occur in the hepatic vascular bed, leading to vasodilatation of splanchnic arteries and systemic hypotension. Many mechanisms seem to work together to cause this imbalance: splanchnic vasodilatation; vasoactive mediators; hyperdynamic circulation states and subsequent cardiac dysfunction; neuro-hormonal mechanisms; changes in sympathetic nervous system, renin-angiotensin system, and vasopressin. In patients with AKI and cirrhosis, fluid expansion therapy needs to be initiated as soon as possible and nephrotoxic drugs discontinued. Once HRS is diagnosed, pharmacological treatment with vasoconstrictors, mainly terlipressin plus albumin, should be initiated. If there is no response, other options can include surgical venous shunts and kidney replacement therapy. In this regard, extracorporeal liver support can be a bridge for liver transplantation, which remains as the ideal treatment. Further studies are necessary to investigate early biomarkers and alternative treatments for HRS.
Transplantation outcomes in patients with primary hyperoxaluria: a systematic review
Elisabeth L. Metry, Liza M. M. van Dijk, Hessel Peters-Sengers, Michiel J.S. Oosterveld, Jaap W. Groothoff, Rutger J. Ploeg, Vianda S. Stel & Sander F. Garrelfs
doi : 10.1007/s00467-021-05043-6
Pediatric Nephrology volume 36, pages2217–2226 (2021)
Primary hyperoxaluria type 1 (PH1) is characterized by hepatic overproduction of oxalate and often results in kidney failure. Liver-kidney transplantation is recommended, either combined (CLKT) or sequentially performed (SLKT). The merits of SLKT and the place of an isolated kidney transplant (KT) in selected patients are unsettled. We systematically reviewed the literature focusing on patient and graft survival rates in relation to the chosen transplant strategy.
Pediatric onco-nephrology: time to spread the word
Arwa Nada & Jennifer G Jetton
doi : 10.1007/s00467-020-04800-3
Pediatric Nephrology volume 36, pages2227–2255 (2021)
Onco-nephrology has been a growing field within the adult nephrology scope of practice. Even though pediatric nephrologists have been increasingly involved in the care of children with different forms of malignancy, there has not been an emphasis on developing special expertise in this area. The fast pace of discovery in this field, including the development of new therapy protocols with their own kidney side effects and the introduction of the CD19-targeted chimeric antigen receptor T cell (CAR-T) therapy, has introduced new challenges for general pediatric nephrologists because of the unique effects of these treatments on the kidney. Moreover, with the improved outcomes in children receiving cancer therapy come an increased number of survivors at risk for chronic kidney disease related to both their cancer diagnosis and therapy. Therefore, it is time for pediatric onco-nephrology to take its spot on the expanding subspecialties map in pediatric nephrology.
Seasonal variation of blood pressure in children
Niels Ziegelasch, Mandy Vogel, Werner Siekmeyer, Heiko Billing, Ingo D?hnert & Wieland Kiess
doi : 10.1007/s00467-020-04823-w
Pediatric Nephrology volume 36, pages2257–2263 (2021)
Seasonal blood pressure (BP) variation is mostly found between the summer and winter months. Guidelines for diagnosis and treatment of hypertension in children have not considered this variation until recently. This review aims to present an overview of seasonal BP variation in childhood along with potential underlying pathophysiological mechanisms and long-term implications as well as conclusions for future studies. In pediatric cohorts, seven studies investigated seasonal changes in BP. These changes amount to 3.4–5.9 mmHg (or 0.5–1.5 mmHg per ??1 °C difference in environmental temperature) in systolic BP with a peak in fall or winter. Potential mechanisms and mediators of seasonal BP variation include sympathetic activation of the nervous system with an increase of urinary and plasma norepinephrine levels in the winter season. Additionally, the physical activity among children and adolescents was inversely correlated with BP levels. Temperature sensitivity of BP and pediatric BP levels predict future systolic BP and target-organ damage. Therefore, cardiovascular events may even be long-term complications of seasonal BP variation in pediatric hypertensive patients. Overall, these data strongly suggest an important effect of ambient temperature on BP in children. Additional studies in pediatric cohorts are needed to define how best to incorporate such variation into clinical practice.
Monitoring dialysis adequacy: history and current practice
Linda Ding, James Johnston & Maury N. Pinsk
doi : 10.1007/s00467-020-04816-9
Pediatric Nephrology volume 36, pages2265–2277 (2021)
Dialysis adequacy for pediatric patients has largely followed the trends in adult dialysis by judging the success or adequacy of peritoneal or hemodialysis with urea kinetic modeling. While this provides a starting point to establish a dose of dialysis, it is clear that urea is only part of the picture. Many clinical parameters and interventions now have been identified that are just as impactful on mortality and morbidly as urea clearance. As such, our concept of adequacy is evolving to include non-urea parameters and assessing the impact that following an “adequate therapy” has on patient lives. As we move to a new era, we consider the impact these therapies have on patients and how it affects the quality of their lives; we must take these factors into consideration to achieve a therapy that is not just adequate, but livable.
Malignancies after pediatric solid organ transplantation
Cal Robinson, Rahul Chanchlani & Abhijat Kitchlu
doi : 10.1007/s00467-020-04790-2
Pediatric Nephrology volume 36, pages2279–2291 (2021)
As life expectancy among pediatric solid organ transplant recipients (SOTRs) improves, the risk of comorbid conditions such as malignancy post-transplantation has also increased. SOTRs are at elevated risks of post-transplantation lymphoproliferative disorders (PTLDs), and skin and solid cancers. PTLDs typically occur early following transplantation, while skin and solid cancers frequently arise in young adulthood (25–40 years). By 30 years following transplantation, 26–41% of pediatric SOTRs have developed cancer. Different risk factors exist for PTLD, and skin and solid cancers, which are modified by cumulative immunosuppression, infections, transplanted organ, and the underlying disease process associated with initial organ failure (e.g., kidney failure). Optimal cancer treatment strategies depend on the specific cancer type, stage, and patient comorbidities. Immunosuppression reduction may be beneficial for certain cancers but must be considered against the risks of acute and chronic rejection and allograft loss. Lifestyle counseling regarding smoking avoidance and sun protection, as well as human papillomavirus vaccination, is an important aspect of cancer prevention. Currently, no cancer screening guidelines exist specifically for pediatric SOTRs. Adult population screening guidelines have not been validated in transplant populations. Therefore, an individualized approach should be taken to cancer screening for pediatric SOTRs, accounting for other cancer risk factors.
Urinary stone and infection does not always mean a ‘chicken and egg dilemma’: Questions
Adem Yasin K?ksoy & Orhan G?rükmez
doi : 10.1007/s00467-021-04975-3
Pediatric Nephrology volume 36, pages2293–2294 (2021)
Urinary stone and infection does not always mean a ‘chicken and egg dilemma’: Answers
Adem Yasin K?ksoy & Orhan G?rükmez
doi : 10.1007/s00467-021-04979-z
Pediatric Nephrology volume 36, pages2295–2297 (2021)
Rickets, elevated fibroblast growth factor-23 and mild anemia: Questions
Judith Sebestyen VanSickle, Tarak Srivastava, Penny Monachino & Uri S. Alon
doi : 10.1007/s00467-021-05000-3
Pediatric Nephrology volume 36, page2299 (2021)
Rickets, elevated fibroblast growth factor-23 and mild anemia: Answers
Judith Sebestyen VanSickle, Tarak Srivastava, Penny Monachino & Uri S. Alon
doi : 10.1007/s00467-021-05012-z
Pediatric Nephrology volume 36, pages2301–2304 (2021)
An unusual cause of immune complex-mediated membranoproliferative glomerulonephritis in a child: Questions
Cemile Pehlivanoglu, Fat?ma Zerenler Gursoy & Betul Sozeri
doi : 10.1007/s00467-021-05003-0
Pediatric Nephrology volume 36, pages2305–2306 (2021)
An unusual cause of immune complex-mediated membranoproliferative glomerulonephritis in a child: Answers
Cemile Pehlivanoglu, Fat?ma Zerenler Gursoy & Betul Sozeri
doi : 10.1007/s00467-021-05015-w
Pediatric Nephrology volume 36, pages2307–2310 (2021)
HLA-DQ and HLA-DRB1 alleles associated with Henoch-Sch?nlein purpura nephritis in Finnish pediatric population: a genome-wide association study
Mikael Koskela, Julia Nihtil?, Elisa Ylinen, Kaija-Leena Kolho, Matti Nuutinen, Jarmo Ritari & Timo Jahnukainen
doi : 10.1007/s00467-021-04955-7
Pediatric Nephrology volume 36, pages2311–2318 (2021)
The pathophysiology of Henoch-Sch?nlein purpura (HSP) is still unclear, but several findings suggest that genetic factors may influence disease susceptibility. We aimed to perform a genome-wide association study (GWAS) in pediatric HSP patients with an emphasis on severe HSP nephritis.
Long-term clinical characteristics and renal prognosis of children with tubulointerstitial nephritis and uveitis syndrome
Asako Hayashi, Toshiyuki Takahashi, Yasuhiro Ueda, Yasuyuki Sato & Takayuki Okamoto
doi : 10.1007/s00467-021-04956-6
Pediatric Nephrology volume 36, pages2319–2325 (2021)
Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, especially in children. Owing to the short-term observational period and the small number of patients analyzed in previous reports, the long-term clinical and laboratory characteristics and renal prognosis of children with TINU syndrome remain unclear.
APOL1 in an ethnically diverse pediatric population with nephrotic syndrome: implications in focal segmental glomerulosclerosis and other diagnoses
Andreia Watanabe, Mara Sanches Guaragna, Vera Maria Santoro Belangero, Fernanda Maria Serafim Casimiro, Jo?o Bosco Pesquero, Luciana de Santis Feltran, Lilian Monteiro Pereira Palma, Patr?cia Varela, Precil Diego Miranda de Menezes Neves, Antonio Marcondes Lerario, Marcela Lopes de Souza, Maricilda Palandi de Mello, Anna Cristina Gerv?sio de Brito Lutaif, Cassio Rodrigues Ferrari, Matthew Gordon Sampson, Luiz Fernando Onuchic & Paulo Cesar Koch Nogueira
doi : 10.1007/s00467-021-04960-w
Pediatric Nephrology volume 36, pages2327–2336 (2021)
APOL1 high-risk genotypes (HRG) are associated with increased risk of kidney disease in individuals of African ancestry. We analyzed the effects of APOL1 risk variants on an ethnically diverse Brazilian pediatric nephrotic syndrome (NS) cohort.
Ten-year trends in epidemiology and outcomes of pediatric kidney replacement therapy in Europe: data from the ESPN/ERA-EDTA Registry
Marjolein Bonthuis, Enrico Vidal, Anna Bjerre, ?zlem Aydo?, Sergey Baiko, Liliana Garneata, Isabella Guzzo, James G. Heaf, Timo Jahnukainen, Marc Lilien, Tamara Mallett, Gabriel Mirescu, Elena A. Mochanova, Eva Nüsken, Katherine Rascher, Dimitar Roussinov, Maria Szczepanska, Michel Tsimaratos, Askiti Varvara, Enrico Verrina, Bojana Veselinovi?, Kitty J. Jager & Jérôme Harambat
doi : 10.1007/s00467-021-04928-w
Pediatric Nephrology volume 36, pages2337–2348 (2021)
For 10 consecutive years, the ESPN/ERA-EDTA Registry has included data on children with stage 5 chronic kidney disease (CKD 5) receiving kidney replacement therapy (KRT) in Europe. We examined trends in incidence and prevalence of KRT and patient survival.
Eculizumab exposure in children and young adults: indications, practice patterns, and outcomes—a Pediatric Nephrology Research Consortium study
Melissa Muff-Luett, Keia R. Sanderson, Rachel M. Engen, Rima S. Zahr, Scott E. Wenderfer, Cheryl L. Tran, Sheena Sharma, Yi Cai, Susan Ingraham, Erica Winnicki, Donald J. Weaver, Tracy E. Hunley, Stefan G. Kiessling, Meredith Seamon, Robert Woroniecki, Yosuke Miyashita, Nianzhou Xiao, Abiodun A. Omoloja, Sarah J. Kizilbash, Asif Mansuri, Mahmoud Kallash, Yichun Yu, Ashley K. Sherman, Tarak Srivastava & Carla M. Nester
doi : 10.1007/s00467-021-04965-5
Pediatric Nephrology volume 36, pages2349–2360 (2021)
Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab.
Cystic kidney diseases associated with mutations in phosphomannomutase 2 promotor: a large spectrum of phenotypes
Guillaume Dorval, Cécile Jeanpierre, Vincent Morinière, Carole Tournant, Bettina Bessières, Tania Attié-Bittach, Jeanne Amiel, Emmanuel Spaggari, Yves Ville, Elodie Merieau, Marie-Claire Gubler, Sophie Saunier & Laurence Heidet
doi : 10.1007/s00467-021-04953-9
Pediatric Nephrology volume 36, pages2361–2369 (2021)
Co-occurrence of polycystic kidney disease and hyperinsulinemic hypoglycemia has been reported in children in a few families associated with a variant in the promotor of the PMM2 gene, at position -167 upstream of the coding sequence. PMM2 encodes phosphomannomutase 2, a key enzyme in N-glycosylation. While biallelic coding PMM2 mutations are involved in congenital disorder of glycosylation CDG1A, that particular variant in the promoter of the gene, either in the homozygous state or associated with a mutation in the coding exons of the gene, is thought to restrict the N-glycosylation defect to the kidney and the pancreas.
Circulating calcification inhibitors are associated with arterial damage in pediatric patients with primary hypertension
Piotr Skrzypczyk, Anna Stelmaszczyk-Emmel, Micha? Szyszka, Anna Ofiara & Ma?gorzata Pa?czyk-Tomaszewska
doi : 10.1007/s00467-021-04957-5
Pediatric Nephrology volume 36, pages2371–2382 (2021)
Circulating calcification inhibitors: fetuin A (FA) and osteoprotegerin (OPG) together with soluble ligand of receptor activator of nuclear factor kappa-B (sRANKL) have been linked to vascular calcifications and arterial damage. This study aimed to evaluate relationships between FA, OPG, sRANKL, and arterial damage in children with primary hypertension (PH).
Continued reduction in peritonitis rates in pediatric dialysis centers: results of the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative
Alicia M. Neu, Troy Richardson, Heidi Gruhler De Souza, Allison Redpath Mahon, Mahima Keswani, Joshua Zaritsky, Raj Munshi, Sarah Swartz, Christine B. Sethna, Michael J. G. Somers & Bradley A. Warady for the SCOPE Collaborative Participants
doi : 10.1007/s00467-021-04924-0
Pediatric Nephrology volume 36, pages2383–2391 (2021)
In its first 3 years, the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative demonstrated a statistically significant increase in the likelihood of compliance with a standardized follow-up care bundle and a significant reduction in peritonitis. We sought to determine if compliance with care bundles and low peritonitis rates could be sustained in centers continuously participating for 84 months.
Hemodiafiltration maintains a sustained improvement in blood pressure compared to conventional hemodialysis in children—the HDF, heart and height (3H) study
Francesca De Zan, Colette Smith, Ali Duzova, Aysun Bayazit, Constantinos J Stefanidis, Varvara Askiti, Karolis Azukaitis, Nur Canpolat, Ayse Agbas, Ali Anarat, Bilal Aoun, Sevcan A. Bakkaloglu, Dagmara Borzych-Du?a?ka, Ipek Kaplan Bulut, Sandra Habbig, Saoussen Krid, Christoph Licht, Mieczyslaw Litwin, Lukasz Obrycki, Fabio Paglialonga, Bruno Ranchin, Charlotte Samaille, Mohan Shenoy, Manish D Sinha, Brankica Spasojevic, Alev Yilmaz, Michel Fischbach, Claus Peter Schmitt, Franz Schaefer, Enrico Vidal & Rukshana Shroff -Show fewer authors
doi : 10.1007/s00467-021-04930-2
Pediatric Nephrology volume 36, pages2393–2403 (2021)
Hypertension is prevalent in children on dialysis and associated with cardiovascular disease. We studied the blood pressure (BP) trends and the evolution of BP over 1 year in children on conventional hemodialysis (HD) vs. hemodiafiltration (HDF).
Procalcitonin serum levels in stage 5 chronic kidney disease children on hemodialysis
Antoine Mouche, Cyrielle Parmentier, Claire Herbez Rea, Thérèsa Kwon, Olivia Boyer, Jean Daniel Delbet & Tim Ulinski
doi : 10.1007/s00467-021-04966-4
Pediatric Nephrology volume 36, pages2405–2409 (2021)
Infections are responsible for morbidity and mortality in children on hemodialysis (HD). Procalcitonin (PCT) is rarely used in this population, even though it is an efficient biomarker of infection and sepsis. Our aim was to study PCT baseline level in uninfected children with stage 5 chronic kidney disease (CKD 5) on HD, and determine how to use it in this population.
L-type calcium channel blocker use and proteinuria among children with chronic kidney diseases
Kelsey L. Richardson, Donald J. Weaver Jr, Derek K. Ng, Megan K. Carroll, Susan L. Furth, Bradley A. Warady & Joseph T. Flynn
doi : 10.1007/s00467-021-04967-3
Pediatric Nephrology volume 36, pages2411–2419 (2021)
Hypertension is common among children with chronic kidney disease (CKD), and dihydropyridine calcium channel blockers (dhCCBs) are frequently used as treatment. The impact of dhCCBs on proteinuria in children with CKD is unclear.
Hemodialysis (HD) dose and ultrafiltration rate are associated with survival in pediatric and adolescent patients on chronic HD—a large observational study with follow-up to young adult age
Verena Gotta, Olivera Marsenic, Andrew Atkinson & Marc Pfister
doi : 10.1007/s00467-021-04972-6
Pediatric Nephrology volume 36, pages2421–2432 (2021)
Hemodialysis (HD) dose targets and ultrafiltration rate (UFR) limits for pediatric patients on chronic HD are not known and are derived from adults (spKt/V>1.4 and <13 ml/kg/h). We aimed to characterize how delivered HD dose and UFR are associated with survival in a large cohort of patients who started HD in childhood.
Amount and selectivity of proteinuria may predict the treatment response in post-transplant recurrence of focal segmental glomerulosclerosis: a single-center retrospective study
Hideki Ban, Kenichiro Miura, Naoto Kaneko, Yoko Shirai, Tomoo Yabuuchi, Kiyonobu Ishizuka, Hiroko Chikamoto, Yuko Akioka, Satoru Shimizu, Hideki Ishida, Kazunari Tanabe & Motoshi Hattori
doi : 10.1007/s00467-021-04951-x
Pediatric Nephrology volume 36, pages2433–2442 (2021)
Primary focal segmental glomerulosclerosis (FSGS) frequently recurs after kidney transplantation and is associated with poor graft survival. To date, few studies have investigated predictive factors for treatment responses in recurrent FSGS.
Survival and transplant outcomes among young children requiring kidney replacement therapy
Nicholas G. Larkins, Germaine Wong, Stephen I. Alexander, Stephen McDonald, Chanel Prestidge, Anna Francis, Amelia K. Le Page & Wai H. Lim
doi : 10.1007/s00467-021-04945-9
Pediatric Nephrology volume 36, pages2443–2452 (2021)
Young children starting kidney replacement therapy (KRT) suffer high disease burden with unique impacts on growth and development, timing of transplantation and long-term survival. Contemporary long-term outcome data and how these relate to patient characteristics are necessary for shared decision-making with families, to identify modifiable risk factors and inform future research.
Predicting acute rejection in children, adolescents, and young adults with a kidney transplant by assessing barriers to taking medication
Charles D. Varnell Jr, Kristin L. Rich, Bin Zhang, Adam C. Carle, Ahna L. H. Pai, Avani C. Modi & David K. Hooper
doi : 10.1007/s00467-021-04946-8
Pediatric Nephrology volume 36, pages2453–2461 (2021)
Nonadherence to immunosuppression is common among pediatric, adolescent, and young adult kidney transplant recipients and a leading cause of graft loss. Assessing barriers to medication adherence in clinical practice may identify patients at risk for rejection and provide therapeutic targets.
Acute rejection and growth outcomes in paediatric kidney allograft recipients treated with a corticosteroid minimisation immunosuppressive protocol
James McCaffrey & Mohan Shenoy
doi : 10.1007/s00467-021-04948-6
Pediatric Nephrology volume 36, pages2463–2472 (2021)
Corticosteroid minimisation immunosuppressive protocols (CMP) for children are an approach to safely reduce unwanted medication side effects associated with long-term exposure following kidney transplantation. Here, we provide data regarding the incidence of acute rejection and growth over an extended follow-up in children receiving the CMP used in our centre.
Association of non-HLA antibodies against endothelial targets and donor-specific HLA antibodies with antibody-mediated rejection and graft function in pediatric kidney transplant recipients
Alexander Fichtner, Caner Süsal, Britta H?cker, Susanne Rieger, Rüdiger Waldherr, Jens H Westhoff, Anja Sander, Duska Dragun & Burkhard T?nshoff
doi : 10.1007/s00467-021-04969-1
Pediatric Nephrology volume 36, pages2473–2484 (2021)
Non-HLA antibodies against endothelial targets have been implicated in the pathogenesis of antibody-mediated rejection (ABMR), but data in pediatric patients are scarce.
Contrast media exposure in the perioperative period confers no additional risk of acute kidney injury in infants and young children undergoing cardiac surgery with cardiopulmonary bypass
Shengwen Guo, Liting Bai, Yuanyuan Tong, Jin Yu, Peiyao Zhang, Xin Duan & Jinping Liu
doi : 10.1007/s00467-021-04964-6
Pediatric Nephrology volume 36, pages2485–2491 (2021)
Recently, there has been an interest in the temporal relationship between contrast exposure (CM) and cardiac surgery suggesting that a “double hit” on the kidney function in close succession increases the risk of acute kidney injury (AKI) after cardiac surgery. However, data from young children is limited. The purpose of this study was to retrospectively evaluate the effects of preoperative CM exposure on postoperative AKI in infant and young children patients and to further analyze the influence of exposure time interval.
The association of alcohol, cigarette, e-cigarette, and marijuana use with disease severity in adolescents and young adults with pediatric chronic kidney disease
Andrea R. Molino, Judith Jerry-Fluker, Meredith A. Atkinson, Susan L. Furth, Bradley A. Warady & Derek K. Ng
doi : 10.1007/s00467-021-05044-5
Pediatric Nephrology volume 36, pages2493–2497 (2021)
We investigated the putative associations of alcohol, cigarette, e-cigarette, and marijuana use with kidney function and proteinuria among adolescents and young adults (AYA) with pediatric-onset chronic kidney disease (CKD) enrolled in the Chronic Kidney Disease in Children (CKiD) study.
Experience with telemedicine in paediatric nephrology during the COVID pandemic
Sanjeev Gulati & Amrita Sengar
doi : 10.1007/s00467-021-05085-w
Pediatric Nephrology volume 36, pages2499–2500 (2021)
Correction to: The association of alcohol, cigarette, e-cigarette, and marijuana use with disease severity in adolescents and young adults with pediatric chronic kidney disease
Andrea R. Molino, Judith Jerry-Fluker, Meredith A. Atkinson, Susan L. Furth, Bradley A. Warady & Derek K. Ng
doi : 10.1007/s00467-021-05044-5
Pediatric Nephrology volume 36, pages2501–2502 (2021)
Topical Collection: Festschrift for Professor Lesley Rees MD FRCPCH
Special Guest Editor: Michel Baum
doi : 10.1007/s00467-021-05171-z
Pediatric Nephrology volume 36, page2503 (2021)
Festschrift for Professor Lesley Rees MD FRCPCH
Michel Baum
doi : 10.1007/s00467-021-05112-w
Pediatric Nephrology volume 36, page2505 (2021)
The red lipstick mentor
Djalila Mekahli
doi : 10.1007/s00467-021-05096-7
Pediatric Nephrology volume 36, pages2507–2509 (2021)
Prof. Lesley Rees belongs to the women who changed science and has inspired so many young researchers, including myself. These inspiring women deserve more credit and recognition than they get because they serve as a role model for us. Indeed, despite advances towards women empowerment, progress has been slow, and discrepancy persists around the world. Unfortunately, science is not immune to such inequalities, and the voices of female leaders are important in cracking this gender filter. Women represent only a third of researchers globally and often face gender-based discrimination and lack equal opportunities. In this letter, I would like to highlight three astonishing researchers in an effort to underscore the importance of existing women mentorship, while transmitting the pride of the red lipstick rather than the victimization of the red cheeks.
Renal effects of growth hormone in health and in kidney disease
Dieter Haffner, Andrea Grund & Maren Leifheit-Nestler
doi : 10.1007/s00467-021-05097-6
Pediatric Nephrology volume 36, pages2511–2530 (2021)
Growth hormone (GH) and its mediator insulin-like growth factor-1 (IGF-1) have manifold effects on the kidneys. GH and IGF receptors are abundantly expressed in the kidney, including the glomerular and tubular cells. GH can act either directly on the kidneys or via circulating or paracrine-synthesized IGF-1. The GH/IGF-1 system regulates glomerular hemodynamics, renal gluconeogenesis, tubular sodium and water, phosphate, and calcium handling, as well as renal synthesis of 1,25 (OH)2 vitamin D3 and the antiaging hormone Klotho. The latter also acts as a coreceptor of the phosphaturic hormone fibroblast-growth factor 23 in the proximal tubule. Recombinant human GH (rhGH) is widely used in the treatment of short stature in children, including those with chronic kidney disease (CKD). Animal studies and observations in acromegalic patients demonstrate that GH-excess can have deleterious effects on kidney health, including glomerular hyperfiltration, renal hypertrophy, and glomerulosclerosis. In addition, elevated GH in patients with poorly controlled type 1 diabetes mellitus was thought to induce podocyte injury and thereby contribute to the development of diabetic nephropathy. This manuscript gives an overview of the physiological actions of GH/IGF-1 on the kidneys and the multiple alterations of the GH/IGF-1 system and its consequences in patients with acromegaly, CKD, nephrotic syndrome, and type 1 diabetes mellitus. Finally, the impact of short- and long-term treatment with rhGH/rhIGF-1 on kidney function in patients with kidney diseases will be discussed.
Managing heart failure in dialysis patients
Daljit K Hothi
doi : 10.1007/s00467-021-05100-0
Pediatric Nephrology volume 36, pages2531–2535 (2021)
The heart and the kidney are intimately connected. They communicate in a bidirectional manner through a variety of pathways, forming an interdependent relationship. Recognition of this co-dependency is crucial in managing patients with cardiorenal syndrome, as we begin to realise the inevitability of disease progression to both organs; and an approach that focuses treatment on one organ may result in worsening outcome on the other organ. When faced with patients with deteriorating cardiac disease, nephrologists tend to focus on stabilisation of cardiac function and accept the heart disease to be unmodifiable. Likewise, cardiac patients with persistent kidney failure are presented with a poor renal prognosis and prepared for kidney transplantation. Adopting a cardio-protective approach in combination with dialysis optimisation raises hope for a more positive outcome with evidence of cardiac and renal recovery in some patients.
Reducing the burden of cardiovascular disease in children with chronic kidney disease: prevention vs. damage limitation
Rukshana Shroff
doi : 10.1007/s00467-021-05102-y
Pediatric Nephrology volume 36, pages2537–2544 (2021)
Cardiovascular disease (CVD) is a life-limiting condition in patients with chronic kidney disease (CKD) and is rapidly progressive, especially in those with stage 5 CKD and on dialysis. Cardiovascular mortality, although reducing, remains at least 30 times higher than in the general pediatric population. The American Heart Association guidelines for cardiovascular risk reduction in high-risk pediatric patients has stratified pediatric CKD patients in the “high risk” category for the development of CVD, with associated pathological and/or clinical evidence for manifest coronary disease before 30 years of age. While improving patient survival is a key priority, other patient-related outcomes, such as psychosocial development, quality of life and growth are of major importance to children and their caregivers. Once vascular damage or calcification has developed, there are no data to suggest that they can be reversed. Treatments such as intensified dialysis and transplantation may attenuate the progression of subclinical cardiovascular disease, but no treatment to date has shown that the inexorable progression of CVD in CKD can be reversed. Thus, our management must focus on early diagnosis and robust preventative strategies to give our patients the best chance of optimal cardiovascular health and survival. In this review, the pathophysiology and importance of preventing the development of CVD in CKD is discussed.
The role of carnitine in maintenance dialysis therapy
Heather A. Morgans, Vimal Chadha & Bradley A. Warady
doi : 10.1007/s00467-021-05101-z
Pediatric Nephrology volume 36, pages2545–2551 (2021)
Carnitine metabolism and homeostasis is significantly altered in patients receiving maintenance dialysis. Current literature in the adult and pediatric dialysis populations suggest a high prevalence of carnitine deficiency, which may lead to erythropoietin-resistant anemia, cardiomyopathy, and muscle weakness. However, the results of pediatric dialysis studies are limited and have not provided the evidence necessary to support strong recommendations or guidelines pertaining to carnitine management. The characteristics and function of carnitine, the definition and consequences of deficiency, a brief overview of recent adult studies, and current studies on carnitine supplementation in pediatric hemodialysis (HD) and peritoneal dialysis (PD) populations are discussed in this review.
Tubulopathy meets Sherlock Holmes: biochemical fingerprinting of disorders of altered kidney tubular salt handling
Detlef Bockenhauer & Robert Kleta
doi : 10.1007/s00467-021-05098-5
Pediatric Nephrology volume 36, pages2553–2561 (2021)
Evolution moves in mysterious ways. Excretion of waste products by glomerular filtration made perfect sense when life evolved in the ocean. Yet, the associated loss of water and solutes became a problem when life moved onto land: a serious design change was needed and this occurred in the form of ever more powerful tubules that attached to the glomerulus. By reabsorbing typically more than 99% of the glomerular filtrate, the tubules not only minimise urinary losses, but, crucially, also maintain homeostasis: tubular reabsorption and secretion are adjusted so as to maintain an overall balance, in which urine volume and composition matches intake and environmental stressors. A whole orchestra of highly specialised tubular transport proteins is involved in this process and dysfunction of one or more of these results in the so-called kidney tubulopathies, characterised by specific patterns of clinical and biochemical abnormalities. In turn, recognition of these patterns helps establish a specific diagnosis and pinpoints the defective transport pathway. In this review, we will discuss these clinical and biochemical “fingerprints” of tubular disorders of salt-handling and how sodium handling affects volume homeostasis but also handling of other solutes.
Growth in children on kidney replacement therapy: a review of data from patient registries
Marjolein Bonthuis, Jérôme Harambat, Kitty J. Jager & Enrico Vidal
doi : 10.1007/s00467-021-05099-4
Pediatric Nephrology volume 36, pages2563–2574 (2021)
Growth retardation is a major complication in children with chronic kidney disease (CKD) and on kidney replacement therapy (KRT). Conversely, better growth in childhood CKD is associated with an improvement in several hard morbidity–mortality endpoints. Data from pediatric international registries has demonstrated that improvements in the overall conservative management of CKD, the search for optimal dialysis, and advances in immunosuppression and kidney transplant techniques have led to a significant improvement of final height over time. Infancy still remains a critical period for adequate linear growth, and the loss of stature during the first years of life influences final height. Preliminary new original data from the European Society for Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry confirm an association between the final height and the height attained at 2 years in children on KRT.
Outcomes of paediatric kidney transplant recipients using the updated 2013/2017 Banff histopathological classification for antibody-mediated rejection
Evgenia Preka, Thivya Sekar, Sergio C Lopez Garcia, Olivia Shaw, Nicos Kessaris, Nizam Mamode, Jelena Stojanovic, Neil J Sebire, Jon Jin Kim & Stephen D Marks
doi : 10.1007/s00467-021-05103-x
Pediatric Nephrology volume 36, pages2575–2585 (2021)
After the major changes with regard to acute and chronic ABMR in the Banff classification initiated in 2013, there has been an improvement in diagnosing antibody-mediated rejection (ABMR) in adult studies but no data have been published in the paediatric population.
