Pediatric Nephrology




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Promising biomarkers in pediatric chronic kidney disease through the kaleidoscope of CAKUT background complexity

Aleksandra Stankovic 

doi : 10.1007/s00467-020-04877-w

Pediatric Nephrology volume 36, pages1321–1325(2021)

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The USPSTF call to inaction on blood pressure screening in children and adolescents

Bonita Falkner & Empar Lurbe

doi : 10.1007/s00467-021-04926-y

Pediatric Nephrology volume 36, pages1327–1329(2021)

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The dietary management of potassium in children with CKD stages 2–5 and on dialysis—clinical practice recommendations from the Pediatric Renal Nutrition Taskforce

An Desloovere, José Renken-Terhaerdt, Jetta Tuokkola, Vanessa Shaw, Larry A. Greenbaum, Dieter Haffner, Caroline Anderson, Christina L. Nelms, Michiel J. S. Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, Bradley A. Warady, Rukshana Shroff & Johan Vande Walle

doi : 10.1007/s00467-021-04923-1

Pediatric Nephrology volume 36, pages1331–1346(2021)

Dyskalemias are often seen in children with chronic kidney disease (CKD). While hyperkalemia is common, with an increasing prevalence as glomerular filtration rate declines, hypokalemia may also occur, particularly in children with renal tubular disorders and those on intensive dialysis regimens. Dietary assessment and adjustment of potassium intake is critically important in children with CKD as hyperkalemia can be life-threatening. Manipulation of dietary potassium can be challenging as it may affect the intake of other nutrients and reduce palatability. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPRs) for the dietary management of potassium in children with CKD stages 2–5 and on dialysis (CKD2–5D). We describe the assessment of dietary potassium intake, requirements for potassium in healthy children, and the dietary management of hypo- and hyperkalemia in children with CKD2–5D. Common potassium containing foods are described and approaches to adjusting potassium intake that can be incorporated into everyday practice discussed. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. 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. These CPRs will be regularly audited and updated by the PRNT.

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Nephropathic cystinosis: an update on genetic conditioning

Rezan Topaloglu 

doi : 10.1007/s00467-020-04638-9

Pediatric Nephrology volume 36, pages1347–1352(2021)

Cystinosis is an autosomal recessive lysosomal storage disorder caused by CTNS gene mutations. The CTNS gene encodes the protein cystinosin, which transports free cystine from lysosomes to cytoplasm. In cases of cystinosin deficiency, free cystine accumulates in lysosomes and forms toxic crystals that lead to tissue and organ damage. Since CTNS gene mutations were first described, many variations have been identified that vary according to geographic region, although the phenotype remains the same. Cystinosis is a hereditary disease that can be treated with the cystine-depleting agent cysteamine. Cysteamine slows organ deterioration, but cannot treat renal Fanconi syndrome or prevent eventual kidney failure; therefore, novel treatment modalities for cystinosis are of great interest to researchers. The present review aims to highlight the geographic differences in cystinosis—specifically in terms of its genetic aspects, clinical features, management, and long-term complications.

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Calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene mutations: a systematic review

Georgia Malakasioti, Daniela Iancu & Kjell Tullus

doi : 10.1007/s00467-020-04695-0

Pediatric Nephrology volume 36, pages1353–1364(2021)

Calcineurin inhibitor (CNI) use in genetic steroid-resistant nephrotic syndrome (SRNS) is controversial as response rate is reported to be lower than non-genetic disease and no plausible mechanism of action is known.

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Deficiency of CFHR plasma proteins and autoantibody positive hemolytic uremic syndrome: treatment rationale, outcomes, and monitoring

Franca Iorember & Anjali Nayak

doi : 10.1007/s00467-020-04652-x

Pediatric Nephrology volume 36, pages1365–1375(2021)

Deficiency of Complement Factor H Related (CFHR) plasma proteins and Autoantibody Positive Hemolytic Uremic Syndrome (DEAP-HUS) is a subtype of atypical hemolytic uremic syndrome, known to be associated with significant morbidity. Its pathogenesis is linked to the production of IgG autoantibodies against complement factor H, a regulator of the alternative complement pathway. The binding of the autoantibodies to the C terminal of complement factor H interferes with its regulatory function, leading to increased activation of the alternative complement pathway and consequent endothelial cellular damage. Early diagnosis and initiation of appropriate therapy is reported to lead to favorable outcomes. Institution of plasma exchange therapy within 24 h of diagnosis has been shown to rapidly lower antibody levels, leading to clinical improvement. Adjunctive immunosuppression therapy suppresses antibody production and helps in maintaining long-term clinical remission of the disease. Available data advocates a treatment regimen that combines plasma therapy (preferably plasma exchange) and immunosuppression to halt disease process and sustain long-term disease remission.

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Kidney outcomes for children with lupus nephritis

Louise Oni, Rachael D Wright, Stephen Marks, Michael W Beresford & Kjell Tullus

doi : 10.1007/s00467-020-04686-1

Pediatric Nephrology volume 36, pages1377–1385(2021)

Systemic lupus erythematosus is a rare lifelong multi-systemic autoimmune condition. Juvenile-onset SLE (JSLE) is recognized to have a more active disease course when compared with adult-onset disease and patients have a worse long-term survival. Kidney involvement occurs in over 50% of children and treatment decisions are guided by the histological classification. Several international groups have produced treatment protocols that rely on an intense period of immunosuppression to halt the acute kidney inflammatory process, followed by maintenance therapy with close observation for disease improvement and prompt evaluation of disease flares. A reduced glomerular filtration rate at presentation is predictive of later stage chronic kidney disease (CKD) in multivariate analysis. Kidney remission remains suboptimal with only 40–60% of patients achieving complete remission. Kidney flares are seen in over a third of patients. The rate of CKD 5 is reported to be up to 15% and the presence of lupus nephritis (LN) has an established link with an associated increase in mortality. In established kidney failure, transplantation seems to be the optimal kidney replacement modality for this group of patients, ideally after a period of disease quiescence. Modified outcome measures in clinical trials have demonstrated that biologic agents can be effective in this disease. Current biologic agents under investigation include obinutuzimab, belimumab, atacicept, anifrolumab, tocilizumab, eculizumab, dapirolizumab, and abatacept. Future research should focus on discovering early disease biomarkers, including surrogates for later cardiovascular disease, and evaluating biological agents as adjuncts to improve the rates of complete remission and subsequently influence the kidney outcome. The aim of this review article is to summarize the current kidney outcomes for this disease with a view to identifying key areas that may help to reduce the risk of long-term CKD.

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Childhood risk factors for adulthood chronic kidney disease

Michal Stern-Zimmer, Ronit Calderon-Margalit, Karl Skorecki & Asaf Vivante

doi : 10.1007/s00467-020-04611-6

Pediatric Nephrology volume 36, pages1386–1396(2021)

Chronic kidney disease (CKD) is a major public health challenge, affecting as much as 8 to 18% of the world population. Identifying childhood risk factors for future CKD may help clinicians make early diagnoses and initiation of preventive interventions for CKD and its attendant comorbidities as well as monitoring for complications. The purpose of this review is to describe childhood risk factors that may predict development of overt kidney disease later in life. Currently, there are multiple childhood risk factors associated with future onset and progression of CKD. These risk factors can be grouped into five categories: genetic factors (e.g., monogenic or risk alleles), perinatal factors (e.g., low birth weight and prematurity), childhood kidney diseases (e.g., congenital anomalies, glomerular diseases, and renal cystic ciliopathies), childhood onset of chronic conditions (e.g., cancer, diabetes, hypertension, dyslipidemia, and obesity), and different lifestyle factors (e.g., physical activity, diet, and factors related to socioeconomic status). The available published information suggests that the lifelong risk for CKD can be attributed to multiple factors that appear already during childhood. However, results are conflicting on the effects of childhood physical activity, diet, and dyslipidemia on future renal function. On the other hand, there is consistent evidence to support follow-up of high-risk groups.

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Rituximab in children with steroid sensitive nephrotic syndrome: in quest of the optimal regimen

Eugene Yu-hin Chan & Kjell Tullus

doi : 10.1007/s00467-020-04609-0

Pediatric Nephrology volume 36, pages1397–1405(2021)

Rituximab has emerged as an effective and important therapy in children with complicated frequently relapsing and steroid-dependent nephrotic syndrome to induce long-term disease remission and avoid steroid toxicities. The optimal rituximab regimen is not totally well defined, and there are many varying practices worldwide. We will in this review describe how patient factors, rituximab dose, and use of maintenance immunosuppression affect treatment outcomes. Specifically, low-dose rituximab without concomitant immunosuppression is associated with shorter relapse-free duration while other regimens have comparable outcomes. Patients with more severe disease generally have worse response to rituximab. Although rituximab appears to be generally safe, there are growing concerns of chronic hypogammaglobulinemia and impaired immunity especially in young children. Reliable prognostications and biomarkers for guiding subsequent treatments to avoid excessive treatments are yet to be identified. In this review, we will outline the, as we see it, best approach of rituximab in childhood steroid sensitive nephrotic syndrome at the present state of knowledge.

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2020 update on the renin–angiotensin–aldosterone system in pediatric kidney disease and its interactions with coronavirus

Ana Cristina Sim?es e Silva, Katharina Lanza, Vit?ria Andrade Palmeira, Larissa Braga Costa & Joseph T. Flynn

doi : 10.1007/s00467-020-04759-1

Pediatric Nephrology volume 36, pages1407–1426(2021)

The last decade was crucial for our understanding of the renin–angiotensin–aldosterone system (RAAS) as a two-axis, counter-regulatory system, divided into the classical axis, formed by angiotensin-converting enzyme (ACE), angiotensin II (Ang II), and the angiotensin type 1 receptor (AT1R), and the alternative axis comprising angiotensin-converting enzyme 2 (ACE2), angiotensin-(1-7) (Ang-(1-7)), and the Mas receptor. Breakthrough discoveries also took place, with other RAAS endopeptides being described, including alamandine and angiotensin A. In this review, we characterize the two RAAS axes and the role of their components in pediatric kidney diseases, including childhood hypertension (HTN), pediatric glomerular diseases, congenital abnormalities of the kidney and urinary tract (CAKUT), and chronic kidney disease (CKD). We also present recent findings on potential interactions between the novel coronavirus, SARS-CoV-2, and components of the RAAS, as well as potential implications of coronavirus disease 2019 (COVID-19) for pediatric kidney diseases.

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Renal tumors in tuberous sclerosis complex

Peter Trnka & Sean E. Kennedy

doi : 10.1007/s00467-020-04775-1

Pediatric Nephrology volume 36, pages1427–1438(2021)

Tuberous sclerosis complex (TSC) is a multisystem hereditary disorder characterized by the growth of benign tumors (hamartomas) in multiple organs, including the kidneys. Renal angiomyolipomas (AML) are a major diagnostic feature of TSC and are present in the majority of patients by adulthood. However, AML are usually asymptomatic during childhood when neurological and developmental manifestations are the main source of morbidity. Kidney manifestations of TSC have historically been the main cause of morbidity and mortality of adults with TSC. The recognition that the complications of TSC are caused by dysregulation of the mammalian target of rapamycin (mTOR) pathway has led to an enormous progress in the management of patients with TSC in the last two decades, the establishment of diagnostic guidelines, and trials which have shown the therapeutic benefit of mTOR inhibitors. Kidney surveillance of children with TSC now provides the opportunity for timely interventions to reduce the impact of TSC in adulthood. In this review, we discuss the current management of kidney tumors associated with TSC, including the diagnosis, surveillance, and treatment options for these lesions. We also present outcome data from international registries demonstrating the effectiveness of the current management strategies. With clear management guidelines and efficient treatment of kidney tumors, we envisage that the long-term outcomes of patients with TSC will further improve in the future.

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Maturation of glomerular filtration rate in neonates and infants: an overview

Silvia Iacobelli & Jean-Pierre Guignard

doi : 10.1007/s00467-020-04632-1

Pediatric Nephrology volume 36, pages1439–1446(2021)

Glomerular filtration rate (GFR) increases progressively throughout fetal life, matures rapidly after birth according to gestational and post-menstrual age, and reaches adult values by 1-year post-natal age. GFR is considered the best marker of kidney function, and in clinical practice, estimated GFR is useful to anticipate complications, establish prognosis, and facilitate treatment decisions. This review article summarizes the maturation of glomerular filtration and the factors and conditions that modulate and impair developing glomerular filtration, and discusses the techniques available to assess GFR in neonates and infants. We focused on simple, reliable, easily available, and cheap techniques to estimate GFR, which may provide valuable information on the renal aspects of the clinical care of this group of patients.

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Metabolic acidosis with increased anion gap, oxaluria, and acute kidney injury: Questions

Bagdagul Aksu, Erkin Rahimov, Alev Yilmaz, Zeynep Yuruk Yildirim, Ilmay Bilge, Sevinc Emre & Aydan Sirin

doi : 10.1007/s00467-020-04757-3

Pediatric Nephrology volume 36, pages1447–1448(2021)

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Metabolic acidosis with increased anion gap, oxaluria, and acute kidney injury: Answers

Bagdagul Aksu, Erkin Rahimov, Alev Yilmaz, Zeynep Yuruk Yildirim, Ilmay Bilge, Sevinc Emre & Aydan Sirin

doi : 10.1007/s00467-020-04758-2

Pediatric Nephrology volume 36, pages1449–1451(2021)

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Macroscopic hematuria in a patient with leukemia: Questions

Mihriban ?n?zü, ?nci Yaman Bajin, Zehra Ayd?n, ?zlem Yüksel Aksoy, Altan Güne?, Hüsniye Ne?e Yaral? & Umut Selda Bayrakç?

doi : 10.1007/s00467-020-04767-1

Pediatric Nephrology volume 36, pages1453–1454(2021)

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Macroscopic hematuria in a patient with leukemia: Answers

Mihriban ?n?zü, ?nci Yaman Bajin, Zehra Ayd?n, ?zlem Yüksel Aksoy, Altan Güne?, Hüsniye Ne?e Yaral? & Umut Selda Bayrakç?

doi : 10.1007/s00467-020-04778-y

Pediatric Nephrology volume 36, pages1455–1456(2021)

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Rocky road—an uncommon reason for kidney allograft dysfunction: Questions

Saritha Ranabothu, Richard Blaszak, Chris Larsen & Brendan Crawford

doi : 10.1007/s00467-020-04683-4

Pediatric Nephrology volume 36, pages1457–1459(2021)

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Rocky road—an uncommon reason for kidney allograft dysfunction: Answers

Saritha Ranabothu, Richard Blaszak, Chris Larsen & Brendan Crawford

doi : 10.1007/s00467-020-04688-z

Pediatric Nephrology volume 36, pages1461–1463(2021)

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Urinary biomarkers as point-of-care tests for predicting progressive deterioration of kidney function in congenital anomalies of kidney and urinary tract: trefoil family factors (TFFs) as the emerging biomarkers

Sachit Anand, Minu Bajpai, Tripti Khanna & Alok Kumar

doi : 10.1007/s00467-020-04841-8

Pediatric Nephrology volume 36, pages1465–1472(2021)

Children with congenital anomalies of kidney and urinary tract (CAKUT) are at high risk of progressive deterioration of kidney function and further developing stage 5 chronic kidney disease (CKD 5), even after a successful surgery. This prospective study was designed to determine whether urinary biomarkers can predict progressive deterioration of kidney function in children with CAKUT.

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Reduced urinary excretion of neutrophil gelatinase-associated lipocalin as a risk factor for recurrence of febrile urinary tract infection in children

Sohsaku Yamanouchi, Takahisa Kimata, Yuko Akagawa, Shohei Akagawa, Jiro Kino, Shoji Tsuji & Kazunari Kaneko

doi : 10.1007/s00467-020-04863-2

Pediatric Nephrology volume 36, pages1473–1479(2021)

This study aimed to test the hypothesis that reduced urinary excretions of neutrophil gelatinase-associated lipocalin (NGAL) predispose children to recurrence of febrile urinary tract infection (fUTI).

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Neutrophil gelatinase-associated lipocalin for urinary tract infection and pyelonephritis: a systematic review

Kai Shaikh, Vinod Rajakumar, Victor A. Osio & Nader Shaikh

doi : 10.1007/s00467-020-04854-3

Pediatric Nephrology volume 36, pages1481–1487(2021)

The sensitivity and specificity of the leukocyte esterase test are relatively low for a screening test for urinary tract infection (UTI). More accurate tests could reduce both overtreatment and missed cases. This study aimed to determine whether neutrophil gelatinase-associated lipocalin (NGAL) can replace leukocyte esterase in the diagnosis of UTI and/or whether NGAL accurately identifies children with acute pyelonephritis.

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Bladder/bowel dysfunction in pre-school children following febrile urinary tract infection in infancy

Sofia Sj?str?m, Ulla Sillén, Marc Bachelard, Ewa Johansson, Per Brandstr?m, Anna-Lena Hellstr?m & Kate Abrahamsson

doi : 10.1007/s00467-020-04853-4

Pediatric Nephrology volume 36, pages1489–1497(2021)

An association between bladder-bowel dysfunction (BBD) and urinary tract infection (UTI) is well-known. However, a question less explored is whether children with UTI early in life also have increased prevalence of BBD after they are toilet-trained. In this study, consecutively selected children with pyelonephritis during their first year of life were assessed for BBD at pre-school age.

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Severely ill pediatric patients with Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS) who suffered from multiple organ involvement in the early stage

Mariana Luna, Mariana Kamariski, Iliana Principi, Victoria Bocanegra & Patricia G. Vallés

doi : 10.1007/s00467-020-04829-4

Pediatric Nephrology volume 36, pages1499–1509(2021)

Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is the main cause of pediatric acute kidney injury (AKI) in Argentina. Endothelial injury is the trigger event in the microangiopathic process. The host inflammatory response to toxin and E. coli lipopolysaccharide (LPS) is involved in disease pathophysiology.

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First-week urine beta-2 microglobulin levels in term healthy neonates

Shimrit Tzvi-Behr, Heftziba Ivgi, Yaacov Frishberg & Efrat Ben Shalom

doi : 10.1007/s00467-020-04839-2

Pediatric Nephrology volume 36, pages1511–1514(2021)

Beta-2 microglobulin (?2mG) is a low-molecular-weight protein that is almost exclusively eliminated through the kidneys. It is freely filtered in the glomeruli and almost completely reabsorbed and degraded in the proximal tubules. Normal urinary ?2mG levels are very low (between 0.04 and 0.22 mg/L). No reference values are known in infants and young children.

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Distribution of proteinuria- and albuminuria-to-creatinine ratios in preterm newborns

Laure Ponthier, Marine Trigolet, Thierry Chianea, Fabienne Mons, Catherine Yardin, Vincent Guigonis & Chahrazed El Hamel

doi : 10.1007/s00467-020-04838-3

Pediatric Nephrology volume 36, pages1515–1524(2021)

Urine protein assessment is important when glomerular disease or injury is suspected. Normal values of proteinuria already published for preterm newborns suffer from limitation, with small cohorts of patients. This prospective study was conducted to update the urine total protein- and albumin-to-creatinine ratio values.

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Low agreement between kidney volume and kidney length z-scores

Laura Torres-Canchala, Martin Rengifo, Guido Filler, Juan C. Arias, Oscar Ramirez & Jaime M. Restrepo

doi : 10.1007/s00467-020-04836-5

Pediatric Nephrology volume 36, pages1525–1532(2021)

Pediatric nephrologists use kidney length and kidney volume z-scores to longitudinally assess normal nephron endowment. However, most radiologists only report kidney length. Agreement between kidney length and kidney volume z-scores in children has been understudied. This study aims to assess agreement between kidney length and kidney volume z-scores in children.

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24-hour ambulatory blood pressure monitoring 9 years after pediatric cardiac surgery: a pilot and feasibility study

Daniel Fredric, Jason H. Greenberg, Chirag R. Parikh, Prasad Devarajan, Hayton Chui, Vedran Cockovski, Michael Pizzi, Ana Palijan, Erin Hessey, Yaqi Jia, Heather R. Thiessen-Philbrook & Michael Zappitelli

doi : 10.1007/s00467-020-04847-2

Pediatric Nephrology volume 36, pages1533–1541(2021)

Children undergoing cardiac surgery are at risk of high blood pressure (BP), a risk factor for cardiovascular and kidney disease. Twenty-four-hour ambulatory BP monitoring (ABPM) is a reference standard hypertension (HTN) test. Little data exist on ABPM abnormalities in children several years post cardiac surgery. This study aimed to (a) determine ABPM feasibility; (b) describe and compare ABPM measures and abnormalities (percent load, masked HTN [MH]; non-dipping, mean systolic/diastolic BP >?95th percentile; pre-HTN (ABPM); white-coat HTN [WCH]) to casual BP; and (c) compare BP in patients with and without acute kidney injury (AKI).

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Isolated nocturnal hypertension is associated with increased left ventricular mass index in children

Tom?? Seeman, Ond?ej Hradsk? & Ji?? Gil?k

doi : 10.1007/s00467-020-04861-4

Pediatric Nephrology volume 36, pages1543–1550(2021)

Isolated nocturnal hypertension (INH) is associated with increased prevalence of left ventricular hypertrophy (LVH) and cardiovascular morbidity and mortality in adult patients. Unlike in adults, data illustrating the possible association between INH and cardiac target organ damage is lacking in children. This study aimed to investigate whether INH is associated with increased left ventricular mass index (LVMI) and LVH in children.

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Achieved clinic blood pressure level and chronic kidney disease progression in children: a report from the Chronic Kidney Disease in Children cohort

Joseph T. Flynn, Megan K. Carroll, Derek K. Ng, Susan L. Furth & Bradley A. Warady

doi : 10.1007/s00467-020-04833-8

Pediatric Nephrology volume 36, pages1551–1559(2021)

Control of hypertension delays progression of pediatric chronic kidney disease (CKD), yet few data are available regarding what clinic blood pressure (BP) levels may slow progression.

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Efficacy of intradialytic amino acids on nutritional status in children with stage 5 chronic kidney disease

Hagar El-Saied Saad El-Serw, Dina Abdel Rasoul Helal Bassiouni, Angy Adel Al-Wakeil, Ragaa Shawky Aly Ibrahim El-Masry & Ashraf Mohamed Abd EI Basset Bakr

doi : 10.1007/s00467-020-04806-x

Pediatric Nephrology volume 36, pages1561–1569(2021)

Protein energy wasting (PEW) is a common cause of morbidity and mortality in patients with stage 5 chronic kidney disease (CKD 5). Intradialytic parenteral nutrition (IDPN) has been used as a therapy for preventing and treating PEW in children with CKD 5 when other conventional modalities fail. However, not enough data is available to define its effectiveness in treating malnutrition in children. This study aims to investigate potential benefits of IDPN in Egyptian children with CKD 5.

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Eosinophilic peritonitis in children on chronic peritoneal dialysis

Ryutaro Suzuki, Mai Sato, Miki Murakoshi, Chikako Kamae, Toru Kanamori, Kentaro Nishi, Masao Ogura & Koichi Kamei

doi : 10.1007/s00467-020-04832-9

Pediatric Nephrology volume 36, pages1571–1577(2021)

Eosinophilic peritonitis (EP) is sometimes difficult to distinguish from bacterial peritonitis (BP) at onset, as they are often overlapping. Previous reports show EP occurs more frequently in infants, although the reason is unknown.

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Tumor necrosis factor-alpha is associated with mineral bone disorder and growth impairment in children with chronic kidney disease

Kelly Meza, Sharmi Biswas, Yuan-Shan Zhu, Anuradha Gajjar, Eduardo Perelstein, Juhi Kumar & Oleh Akchurin

doi : 10.1007/s00467-020-04846-3

Pediatric Nephrology volume 36, pages1579–1587(2021)

Mineral and bone disorder (MBD) and growth impairment are common complications of pediatric chronic kidney disease (CKD). Chronic inflammation detrimentally affects bone health and statural growth in non-CKD settings, but the impact of inflammation on CKD-MBD and growth in pediatric CKD remains poorly understood. This study assessed associations between inflammatory cytokines with biomarkers of CKD-MBD and statural growth in pediatric CKD.

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Dietary fibre intake is low in paediatric chronic kidney disease patients but its impact on levels of gut-derived uraemic toxins remains uncertain

Amina El Amouri, Evelien Snauwaert, Aurélie Foulon, Charlotte Vande Moortel, Maria Van Dyck, Koen Van Hoeck, Nathalie Godefroid, Griet Glorieux, Wim Van Biesen, Johan Vande Walle, Ann Raes & Sunny Eloot

doi : 10.1007/s00467-020-04840-9

Pediatric Nephrology volume 36, pages1589–1595(2021)

Chronic kidney disease (CKD) in children is a pro-inflammatory condition leading to a high morbidity and mortality. Accumulation of organic metabolic waste products, coined as uraemic toxins, parallels kidney function decline. Several of these uraemic toxins are protein-bound (PBUT) and gut-derived. Gut dysbiosis is a hallmark of CKD, resulting in a state of increased proteolytic fermentation that might be counteracted by dietary fibre. Data on fibre intake in children with CKD are lacking. We aimed to assess dietary fibre intake in a paediatric CKD cohort and define its relationship with PBUT concentrations.

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Acute peritoneal dialysis, complications and outcomes in 389 children with STEC-HUS: a multicenter experience

Paula A. Coccia, Flavia B. Ram?rez, Angela D. C. Su?rez, Laura F. Alconcher, Alejandro Balestracci, Laura A. Garc?a Chervo, Iliana Principi, A?da V?zquez, Viviana M. Ratto, Mar?a Celia Planells, Jorge Montero, Mariana Saurit, Maria Graciela Pérez Y Gutiérrez, Mar?a Celeste Puga, Elsa M. Isern, Mar?a Carolina Bettendorff, Marcela V. Boscardin, Marta Baz?n, Mario A. Polischuk, Alejo De Sarrasqueta, Adriana Aralde, Diego B. Ripeau, Daniela C. Leroy, Nahir E. Quijada, Romina S. Escalante, Marta I. Giordano, Cristian S?nchez, Ver?nica S. Selva, Alejandra Caminiti, José Mar?a Ojeda, Pablo Bonany, Sandra E. Morales, Daniel Allende, Mar?a Andrea Arias, Andrea M. Exeni, Jésica D. Geuna & Larisa Arr?a -Show fewer authors

doi : 10.1007/s00467-020-04876-x

Pediatric Nephrology volume 36, pages1597–1606(2021)

Management of acute kidney injury (AKI) in children with hemolytic uremic syndrome induced by a Shiga toxin-producing Escherichia coli infection (STEC-HUS) is supportive; however, 40 to 60% of cases need kidney replacement therapy (KRT). The aim of this study was to analyze procedure complications, especially peritonitis, and clinical outcomes in children with AKI secondary to STEC-HUS treated with acute PD.

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Effect of levocarnitine supplementation on myocardial strain in children with acute kidney injury receiving continuous kidney replacement therapy: a pilot study

Kristen Sgambat, Sarah Clauss & Asha Moudgil

doi : 10.1007/s00467-020-04862-3

Pediatric Nephrology volume 36, pages1607–1616(2021)

Carnitine plays a key role in energy production in the myocardium and is efficiently removed by continuous kidney replacement therapy (CKRT). Effects of levocarnitine supplementation on myocardial function in children receiving CKRT have not been investigated.

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Non-invasive continuous renal tissue oxygenation monitoring to identify preterm neonates at risk for acute kidney injury

Matthew W. Harer, Claudette O. Adegboro, Luke J. Richard & Ryan M. McAdams

doi : 10.1007/s00467-020-04855-2

Pediatric Nephrology volume 36, pages1617–1625(2021)

Near-infrared spectroscopy (NIRS) is an emerging tool to identify signs of inadequate tissue oxygenation in preterm neonates with acute kidney injury (AKI). Previous studies have shown a correlation between low renal tissue oxygenation (RrSO2) in the first 24 hours of age and the later development of AKI. In this prospective clinical trial, NIRS monitoring was used to identify changes in RrSO2 in comparison to traditional AKI markers, serum creatinine (SCr), and urine output (UOP).

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Acute kidney injury in children hospitalized for acute gastroenteritis: prevalence and risk factors

Pierluigi Marzuillo, Maria Baldascino, Stefano Guarino, Silverio Perrotta, Emanuele Miraglia del Giudice & Felice Nunziata

doi : 10.1007/s00467-020-04834-7

Pediatric Nephrology volume 36, pages1627–1635(2021)

We aimed to evaluate prevalence of acute kidney injury (AKI) and its risk factors in children hospitalized for acute gastroenteritis (AGE) to identify early predictors of AKI.

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Consensus acute kidney injury criteria integration identifies children at risk for long-term kidney dysfunction after multiple organ dysfunction syndrome

Stephen M. Gorga, Erin F. Carlton, Joseph G. Kohne, Ryan P. Barbaro & Rajit K. Basu

doi : 10.1007/s00467-020-04865-0

Pediatric Nephrology volume 36, pages1637–1646(2021)

The consensus definition of acute kidney injury (AKI) has evolved since developing the original multiple organ dysfunction syndrome (MODS) definitions. Whether or not risk for adverse short- and long-term outcomes can be identified using the refined AKI criteria in the setting of MODS has not been studied. We hypothesize that incorporation of Kidney Disease: Improving Global Outcome (KDIGO) AKI criteria into existing MODS definitions will have a higher association with major adverse kidney events at 30 days (MAKE30) and will increase the number of patients with MODS.

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Pediatric atypical hemolytic–uremic syndrome due to auto-antibodies against factor H: is there an interest to combine eculizumab and mycophenolate mofetil?

Lucie Matrat, Justine Bacchetta, Bruno Ranchin, Corentin Tanné & Anne-Laure Sellier-Leclerc

doi : 10.1007/s00467-021-05025-8

Pediatric Nephrology volume 36, pages1647–1650(2021)

Atypical hemolytic and uremic syndrome (aHUS), a thrombotic micro-angiopathy (TMA) caused by deregulation in the complement pathway, is sometimes due to the presence of anti-complement factor H (CFH) auto-antibodies. The “standard” treatment for such aHUS combines plasma exchange therapy and immunosuppressive drugs. Eculizumab, a monoclonal antibody that blocks the terminal pathway of the complement cascade, could be an interesting alternative in association with an immunosuppressive treatment for maintenance regimen.

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Regarding: A global anti-B cell strategy combining obinutuzumab and daratumumab in severe pediatric nephrotic syndrome

Alan Majeranowski & Marcin Okr?j

doi : 10.1007/s00467-021-04981-5

Pediatric Nephrology volume 36, pages1651–1652(2021)

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Response to Majeranowski

Claire Dossier & Julien Hogan

doi : 10.1007/s00467-021-04982-4

Pediatric Nephrology volume 36, page1653(2021)

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Treatment strategy for Streptococcus pneumoniae-associated hemolytic uremic syndrome

Iona Madden & Jerome Harambat

doi : 10.1007/s00467-021-05053-4

Pediatric Nephrology volume 36, pages1655–1656(2021)

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H. William Schnaper - Life course journey of a true Mensch

Laurence Greenbaum, Victoria Norwood, Eileen Brewer, William Smoyer, Marva Moxey-Mims, Joseph Flynn, Barbara Fivush, Patrick Brophy, Brad Warady, Sandra Watkins, Isidro Salusky & Rick Kaskel

doi : 10.1007/s00467-021-04959-3

Pediatric Nephrology volume 36, pages1657–1659(2021)

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Correction to: Childhood risk factors for adulthood chronic kidney disease

Michal Stern-Zimmer, Ronit Calderon-Margalit, Karl Skorecki & Asaf Vivante

doi : 10.1007/s00467-020-04663-8

Pediatric Nephrology volume 36, page1661(2021)

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