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  • Correction to: C3 levels and acute outcomes in Shiga toxin-related hemolytic uremic syndrome.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-11-02
    Alejandro Balestracci,Luciana Meni Battaglia,Ismael Toledo,Laura Beaudoin,Caupolican Alvarado

    Due to an unfortunate error during the processing of the article, the spelling of the second author name was incorrect.

    更新日期:2020-01-04
  • An uncommon case of arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome and review of the renal involvement: Questions.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Minh Dien Duong,Chelsi M Rose,Kimberly J Reidy,Marcela Del Rio

    Arthrogryposis, renal dysfunction, and cholestasis syndrome is a rare autosomal recessive disorder caused by mutations in the VPS33B and VIPAR genes. Most cases are fatal within the first year of life. Here we describe one of the two oldest patients with arthrogryposis, renal dysfunction, and cholestasis syndrome. This is a 12-year-old Hispanic female, from a non-consanguineous parents, diagnosed with an incomplete phenotype of arthrogryposis, renal dysfunction, and cholestasis syndrome with arthrogryposis and renal tubular dysfunction but without cholestasis. At 11 years of age, she was found to have impaired renal function, nephrotic-range proteinuria, Fanconi syndrome, and distal renal tubular acidosis. She also had hypercalciuria, nephrogenic diabetes insipidus, and small kidneys by renal ultrasound. Genetic analysis using whole exome sequencing showed a mutation and a partial deletion in the VPS33B gene. Further studies showed that the mother has a partial deletion in the VPS33B gene. Her medication regimen includes potassium citrate and enalapril.

    更新日期:2020-01-04
  • Renal aspects of metabolic acid-base disorders in neonates.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-11-21
    Silvia Iacobelli,Jean-Pierre Guignard

    Acid-base homeostasis is one of the most tightly regulated systems in the body. Maintaining the acid-base balance is particularly challenging for preterm infants and growing neonates. The kidney, which represents the crucial ultimate line of defense against disturbances of acid-base balance, undergoes a complex maturation process during the transition from a fetal to an extra-uterine environment. This review article summarizes the physiology of acid-base regulation by the immature human kidney and discusses disorders of acid-base balance, such as metabolic acidosis, respiratory acidosis, metabolic alkalosis, and respiratory alkalosis. In conditions of metabolic acidosis, the serum anion gap and the urinary anion gap can be useful tools to define the nature of the acidosis. Metabolic acidosis can reflect a decrease in glomerular filtration rate, or be the consequence of selective disorders of proximal or distal tubular function. Most tubulopathies associated with metabolic acidosis observed in neonates are primary, hereditary, isolated tubulopathies. Proximal renal tubular acidosis is characterized by bicarbonate wasting, while the distal types of renal tubular acidosis are secondary to distal acidification defects. All tubulopathies are associated with hypokalemia, with the exception of type 4 hyperkalemic distal renal tubular acidosis. The transporter defects in the various acid-base tubulopathies are now well defined. Treatment of the acidosis varies according to the site and mechanism of the defect. Chronic renal tubular acidosis or alkalosis severely impair growth and calcium metabolism. Early rational therapeutic intervention can prevent some of the consequences of the disorders and improves the prognosis.

    更新日期:2020-01-04
  • Histological prognostic factors in children with Henoch-Schönlein purpura nephritis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-11-07
    Jean-Daniel Delbet,Guillaume Geslain,Martin Auger,Julien Hogan,Rémi Salomon,Michel Peuchmaur,Georges Deschênes,David Buob,Cyrielle Parmentier,Tim Ulinski

    BACKGROUND The management of IgA vasculitis with nephritis (IgAVN) remains controversial because of the difficulty to identify prognostic factors. This study reports the prognosis of children with IgAVN in relation to histological parameters. METHODS All children with IgAVN diagnosed between 2000 and 2015 in three pediatric nephrology centers were included. The following histological parameters were analyzed: mesangial proliferation (MP), endocapillary proliferation (EP), crescents, active, or chronic tubular and interstitial lesions (TIa lesions/TIc lesions), and segmental glomerulosclerosis (GS). Clinical and biological data were collected at the time of renal biopsy. The primary endpoint was IgAVN remission defined as a proteinuria < 200 mg/l without renal failure. RESULTS One hundred fifty-nine children were included with a median age of 7.6 years. Acute glomerular or TI lesions including MP, EP, crescents, and TIa lesions were observed, respectively, in 81%, 86%, 49%, and 21% of patients. Chronic glomerular lesions including GS and TIc lesions were observed in 6 and 7% of patients. Median initial proteinuria was 330 mg/mmol, albuminemia 32 g/l, and eGFR 110 ml/min/1.73 m2. One hundred twelve (70%) patients were in remission at the end of a median follow-up of 37.4 months. Chronic lesions were significantly associated with the absence of remission in multivariate analysis, whereas EP, crescents and TIa were not associated with a poor prognosis. CONCLUSIONS Of children with IgAVN, 30% present a persistent renal disease at the end of a 3-year follow-up. Chronic histological lesions, but not EP or crescents, are associated with a bad prognosis and must be evaluated in IgAVN histological classification.

    更新日期:2020-01-04
  • Biomarkers that differentiate false positive urinalyses from true urinary tract infection.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Nader Shaikh,Judith M Martin,Alejandro Hoberman,Megan Skae,Linette Milkovich,Christi McElheny,Robert W Hickey,Lucine V Gabriel,Diana H Kearney,Massoud Majd,Eglal Shalaby-Rana,George Tseng,Jay Kolls,William Horne,Zhiguang Huo,Timothy R Shope

    BACKGROUND The specificity of the leukocyte esterase test (87%) is suboptimal. The objective of this study was to identify more specific screening tests that could reduce the number of children who unnecessarily receive antimicrobials to treat a presumed urinary tract infection (UTI). METHODS Prospective cross-sectional study to compare inflammatory proteins in blood and urine samples collected at the time of a presumptive diagnosis of UTI. We also evaluated serum RNA expression in a subset. RESULTS We enrolled 200 children; of these, 89 were later demonstrated not to have a UTI based on the results of the urine culture obtained. Urinary proteins that best discriminated between children with UTI and no UTI were involved in T cell response proliferation (IL-9, IL-2), chemoattractants (CXCL12, CXCL1, CXCL8), the cytokine/interferon pathway (IL-13, IL-2, INFγ), or involved in innate immunity (NGAL). The predictive power (as measured by the area under the curve) of a combination of four urinary markers (IL-2, IL-9, IL-8, and NGAL) was 0.94. Genes in the pathways related to inflammation were also upregulated in serum of children with UTI. CONCLUSIONS Urinary proteins involved in the inflammatory response may be useful in identifying children with false positive results with current screening tests for UTI; this may reduce unnecessary treatment.

    更新日期:2020-01-04
  • Functional roles of Grainyhead-like transcription factors in renal development and disease.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-17
    Felix J Boivin,Kai M Schmidt-Ott

    Proper renal function relies on the tightly regulated development of nephrons and collecting ducts. This process, known as tubulogenesis, involves dynamic cellular and molecular changes that instruct cells to form highly organized tubes of epithelial cells which compartmentalize the renal interstitium and tubular lumen via assembly of a selective barrier. The integrity and diversity of the various renal epithelia is achieved via formation of intercellular protein complexes along the apical-basal axis of the epithelial cells. In recent years, the evolutionarily conserved family of Grainyhead-like (GRHL) transcription factors which encompasses three mammalian family members (Grainyhead-like 1, 2, 3) has emerged as a group of critical regulators for organ development, epithelial differentiation, and barrier formation. Evidence from transgenic animal models supports the presence of Grainyhead-like-dependent transcriptional mechanisms that promote formation and maintenance of epithelial barriers in the kidney. In this review, we highlight different Grhl-dependent mechanisms that modulate epithelial differentiation in the kidney. Additionally, we discuss how disruptions in these mechanisms result in impaired renal function later in life.

    更新日期:2020-01-04
  • Effects of changes in adult erythropoietin dosing guidelines on erythropoietin dosing practices, anemia, and blood transfusion in children on hemodialysis: findings from North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS).
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Sarah A Twichell,Elizabeth A K Hunt,Karen Martz,Michael J G Somers,

    BACKGROUND While adult hemodialysis (HD) patients have increased morbidity with higher target hemoglobin levels, similar findings have not been demonstrated in pediatric patients. We evaluated changes in transfusions, anemia frequency, and erythropoietin (epo) dosing among pediatric HD patients before, during, and after implementation of federal dialysis payment policies regarding epo dosing for adult HD patients. METHODS This is a retrospective cohort study of pediatric HD patients enrolled in NAPRTCS. We evaluated need for transfusion, anemia, median hemoglobin, and median epo dose 6 months after starting HD in 3 eras: baseline (2003-2007), implementation (2008-2011), and post implementation (2012-2016). We used multivariate logistic regression models to evaluate potential differences in transfusion across the eras. RESULTS Six months after dialysis initiation, 12.6% of patients required transfusion pre-implementation, 17.9% during implementation, and 15.5% post implementation. Anemia occurred in 17.4% of patients pre, 23.5% during, and 23.8% post implementation, with median hemoglobin levels of 11.9 g/dL pre, 11 g/dL during, and 11 g/dL post implementation. Epo use was high across all 3 eras, but epo dosing decreased during and post implementation, despite more anemia during these periods. Odds of transfusion in implementation era compared with pre-implementation was 1.75 (95% CI 1.11-2.77) and odds of transfusion in post implementation era compared with pre was 1.19 (95% CI 0.71-1.98), controlling for age, race, gender, and prior transplant status. CONCLUSIONS During and following implementation of adult epo dosing guidelines, transfusion and anemia frequency increased in pediatric HD patients. Ideal target hemoglobin levels for pediatric dialysis patients warrant further study.

    更新日期:2020-01-04
  • Plasma pseudouridine levels reflect body size in children on hemodialysis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Frank J O'Brien,Tammy L Sirich,Abigail Taussig,Enrica Fung,Lakshmi L Ganesan,Natalie S Plummer,Paul Brakeman,Scott M Sutherland,Timothy W Meyer

    BACKGROUND Dialysis in children as well as adults is prescribed to achieve a target spKt/Vurea, where Vurea is the volume of distribution of urea. Waste solute production may however be more closely correlated with body surface area (BSA) than Vurea which rises in proportion with body weight. Plasma levels of waste solutes may thus be higher in smaller patients when targeting spKt/Vurea since they have higher BSA relative to body weight. This study measured levels of pseudouridine (PU), a novel marker solute whose production is closely proportional to BSA, to test whether prescription of dialysis to a target spKt/Vurea results in higher plasma levels of PU in smaller children. METHODS PU and urea nitrogen (ureaN) were measured in plasma and dialysate at the midweek hemodialysis session in 20 pediatric patients, with BSA ranging from 0.65-1.87m2. Mathematical modeling was employed to estimate solute production rates and average plasma solute levels. RESULTS The dialytic clearance (Kd) of PU was proportional to that of ureaN (average KdPU/KdUreaN 0.69 ± 0.13, r2 0.84, p < 0.001). Production of PU rose in proportion with BSA (r2 0.57, p < 0.001). The pretreatment plasma level of PU was significantly higher in smaller children (r2 0.20, p = 0.051) while the pretreatment level of ureaN did not vary with size. CONCLUSIONS Prescribing dialysis based on urea kinetics may leave uremic solutes at higher levels in small children. Measurement of a solute produced proportional to BSA may provide a better index of dialysis adequacy than measurement of urea.

    更新日期:2020-01-04
  • Kidney and organoid single-cell transcriptomics: the end of the beginning.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-01-05
    Parker C Wilson,Benjamin D Humphreys

    Single-cell RNA sequencing (scRNA-seq) technologies are increasingly being applied to reveal cellular heterogeneity in kidney development and disease. In just the last year, multiple scRNA-seq datasets have been generated from kidney organoids, developing mouse and human kidney, adult kidney, and kidney cancer. The data generated enables a much deeper understanding of biological processes within and between cells. It has also elucidated unforeseen cell lineage relationships, defined the presence of off-target cell types in kidney organoids, and revealed a diverse inflammatory response in a human kidney allograft undergoing rejection. This review summarizes the recent rapid progress in scRNA-seq of the kidney and outlines future directions for single-cell technologies as applied to the kidney.

    更新日期:2020-01-04
  • C3 levels and acute outcomes in Shiga toxin-related hemolytic uremic syndrome.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-09-03
    Alejandro Balestracci,Luciana Meni Bataglia,Ismael Toledo,Laura Beaudoin,Caupolican Alvarado

    BACKGROUND The correlation between complement activation and severity of hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli (STEC-HUS) has been examined in few studies, with conflicting results. We investigated whether C3 levels on admission are associated with worse acute outcomes. METHODS Demographic, clinical, and laboratory variables were compared between dialyzed and non-dialyzed patients and between those with or without extrarenal complications. Univariate and multivariate analyses were performed; odds ratio (OR) and 95% confidence interval (95%CI) were calculated. C3 concentrations were correlated with dialysis length (Spearman test) and ROC curves with area under the curves (AUC) were calculated to identify C3 concentrations able to discriminate patients with dialysis requirements and complicated course. RESULTS Among 49 children, 33 had normal and 16 had decreased C3 concentrations. Higher hemoglobin, lactic dehydrogenase, urea and creatinine and lower albumin, sodium, and C3 and C4 concentrations at admission were associated with dialysis requirement; only creatinine remained significant (p = 0.03, OR 2.1, 95%CI 1.34-2.7) by multivariate analysis. Patients with a complicated course presented higher leukocyte count, hemoglobin and lactic dehydrogenase and lower albumin, sodium, and C3 and C4. In the multivariate analysis, leukocyte count (p = 0.02, OR 2.6, 95%CI 1.4-4.3) and C3 concentration (p = 0.039, OR 1.7, 95%CI 1.1-2.73) were independently associated with a complicated disease. C3 levels correlated with dialysis length (r = - 0.42, p = 0.002); nevertheless, they were unable to discriminate dialysis requirement (AUC = 0.25, 95%CI 0.11-0.38) and extrarenal complications (AUC = 0.24, 95%CI 0.11-0.4). CONCLUSIONS Our study suggests that decreased C3 levels at admission are associated with a more complicated STEC-HUS episode.

    更新日期:2020-01-04
  • Risk factors for complications of percutaneous ultrasound-guided renal biopsy in children.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Jhao-Jhuang Ding,Shih-Hua Lin,Jing-Long Huang,Tai-Wei Wu,Shao-Hsuan Hsia,Jainn-Jim Lin,Yu-Ching Chou,Min-Hua Tseng

    BACKGROUND Percutaneous ultrasound-guided renal biopsy (PURB) is an invasive but essential procedure in establishing the histologic diagnosis of pediatric renal diseases. Large studies which describe PURB complications and its contributory risk factors are scarce in the pediatric literature. METHODS Patients who underwent real-time PURB from September 2011 to August 2017 were retrospectively reviewed. Data pertaining to clinical characteristics, histologic diagnosis and biopsy-related complications were collected. In addition, the risk factors for complications were also analyzed. RESULTS Overall, 183 patients (109 females) were enrolled and 201 biopsies were obtained. The mean age was 14.4 ± 13.7 years. Over 98% of the biopsies were considered adequate in quality. The major complications were perirenal hematoma requiring blood transfusion (4 cases, 2.0%), followed by perirenal abscess (1 case, 0.5%) and arteriovenous fistula (1 case, 0.5%). All patients recovered without sequelae after treatment. Hypertension, low estimated glomerular filtration rate (eGFR) and anemia were more common in patients with complication than in those without. Further logistic regression model analysis demonstrated that eGFR <30 ml/1.73m2/min was an independent risk factor for major complications. CONCLUSIONS Perirenal hematoma needing blood transfusion is the most common major complication for children undergoing renal biopsy. Low eGFR is an independent risk factor for major complications. Early recognition and timely treatment should be delivered to children with renal function impairment accordingly.

    更新日期:2020-01-04
  • Quality improvement in pediatric nephrology-a practical guide.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-01-07
    Marie-Michele Gaudreault-Tremblay,Rory F McQuillan,Rulan S Parekh,Damien Noone

    Improving quality of care delivery is an important focus for all practicing physicians. Frontline clinicians are in a great position to identify clinical problems and find innovative solutions. The current review describes the method used for quality improvement based on the Model for Improvement, a structural framework to guide improvement work. At its basis are three fundamental questions: What are we trying to accomplish? How will I know that a change will lead to improvement? And what changes could we make that will result in improvement? This preparation phase aims to identify and understand the problem, choose an intervention, and determine reliable measures to gauge improvement. The intervention is then tested using PLAN-DO-STUDY-ACT (PDSA) cycles, an iterative approach to systematically improve processes and outcomes. PLAN focuses on defining the goal of the cycle and describing in details what will be done. DO concentrates on the concrete application of the plan. STUDY focuses on data analyses as ACT identifies lessons learned from the cycle and orientate the goals of the following PDSA cycle. Learning from each cycle, developing an interdisciplinary team and repeated interventions are core principles involved in implementing a sustainable quality improvement program. The Model for Improvement will be illustrated by a common quality problem in pediatric nephrology.

    更新日期:2020-01-04
  • Ofatumumab rescue treatment in post-transplant recurrence of focal segmental glomerulosclerosis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Manuela Colucci,Raffaella Labbadia,Marina Vivarelli,Francesca Diomedi Camassei,Francesco Emma,Luca Dello Strologo

    BACKGROUND Treatment of post-transplant focal segmental glomerulosclerosis (FSGS) recurrence is still debated. The use of the fully human anti-CD20 monoclonal antibody ofatumumab has been suggested. CASE-DIAGNOSIS/TREATMENT Two boys with FSGS received a kidney transplantation at the age of 15 years from a deceased and a living donor. Maintenance therapy consisted of calcineurin inhibitors, antiproliferative agents, and prednisone. Early post-transplant FSGS recurrence was observed after 2 and 3 days. Rituximab infusion and plasmapheresis sessions were performed with transient clinical improvement in the first patient, and no apparent response in the second patient. Both patients were treated with two ofatumumab infusions, which induced in patient #1 a complete and stable remission for more than 12 months and in patient #2 a partial remission with a progressive reduction of proteinuria and normalization of serum protein levels. CONCLUSIONS Ofatumumab may be a therapeutic option for post-transplant FSGS recurrence in patients who respond poorly to rituximab.

    更新日期:2020-01-04
  • Recalibration of cystatin C using standardized material in Siemens nephelometers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    George J Schwartz,Christopher Cox,Jesse C Seegmiller,Paula S Maier,Donna DiManno,Sue L Furth,Bradley A Warady,Alvaro Munoz

    BACKGROUND Cystatin C is a key GFR biomarker. Recently, Siemens recalibrated the assay based on certified reference material ERM-DA471/IFCC. The NIH-funded longitudinal chronic kidney disease in children (CKiD) study has > 3000 cystatin C measurements based on a pre-IFCC calibrator provided by Siemens. Since cystatin C values for CKiD are now standardized to IFCC certified reference material, it is important to relate the IFCC-calibrated results to the previous values so that there are no discontinuous results. METHODS We diluted cystatin C ERM-DA471/IFCC (5.48 mg/L) into buffer and compared results with predicted ones. We then updated the cystatin C application on our BN II nephelometer to provide results based on pre-IFCC and IFCC calibrations of CKiD specimens simultaneously. We assayed 51 previously analyzed sera and 62 fresh additional specimens. RESULTS The predicted concentrations from the IFCC standard were consistently 17% higher than the measured values using the pre-IFCC calibration (y = 1.1686x). Similarly, the re-run and fresh sample concentrations were 17% higher via the IFCC calibration than by the pre-IFCC calibration (y = 1.168x). There was very high reliability in the measurements using the previous calibration for re-run specimens (0.99) and for 33 pristine specimens using IFCC calibration (0.99). CONCLUSIONS We confirm the recalibration proposed by Siemens. To convert pre-IFCC results to IFCC-calibrated concentrations, the value is multiplied by 1.17. Conversely, one divides IFCC-calibrated results by 1.17 to estimate GFR via previously published pre-IFCC CKiD eGFR equations. For older adolescents, cystatin C has already been standardized and can be directly applied to the CKD-EPI equations.

    更新日期:2020-01-04
  • An infant with hyponatremia, hyperkalemia, and metabolic acidosis associated with urinary tract infection: Answers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-05-06
    Bahriye Atmis,İhsan Turan,Engin Melek,Aysun Karabay Bayazit

    更新日期:2019-11-01
  • Structural renal abnormalities in the DICER1 syndrome: a family-based cohort study.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-09-05
    Nicholas E Khan,Alexander Ling,Molly E Raske,Laura A Harney,Ann G Carr,Amanda Field,Anne K Harris,Gretchen M Williams,Louis P Dehner,Yoav H Messinger,D Ashley Hill,Kris Ann P Schultz,Douglas R Stewart

    BACKGROUND The DICER1 syndrome is a tumor-predisposition disorder caused by germline pathogenic variation in DICER1 and is associated with cystic nephroma and other renal neoplasms. Dicer1 mouse and rare human DICER1 syndrome case reports describe structural kidney and collecting system anomalies. We investigated renal function and the frequency of structural abnormalities of the kidney and collecting system in individuals with germline loss-of-function variants in DICER1. METHODS In this family-based cohort study, prospectively ascertained germline DICER1-mutation carriers (DICER1-carriers) and unaffected family controls were evaluated at the National Institutes of Health Clinical Center with renal ultrasound and comprehensive laboratory testing. Two radiologists reviewed the imaging studies from all participants for structural abnormalities, cysts, and tumors. RESULTS Eighty-nine DICER1-carriers and 61 family controls were studied. Renal cysts were detected in 1/33 DICER1-carrier children without history of cystic nephroma. Similar proportions of adult DICER1-carriers (8/48; 17%) and controls (11/50; 22%) had ultrasound-detected renal cysts (P = 0.504). 8/89 (9%) DICER1-carriers harbored ultrasound-detected structural abnormalities of varying severity within the collecting system or kidney, nephrolithiasis, or nephrocalcinosis. None of the family controls (0/61) had similar findings on ultrasound (P = 0.02). No meaningful differences in renal laboratory values between DICER1-carriers and unaffected family controls were observed. CONCLUSIONS Our report is the first to systematically characterize renal function and anatomy in a large prospective cohort of DICER1-carriers and DICER1-negative family controls. DICER1-carriers may be at increased risk of structural anomalies of the kidney or collecting system. The role for DICER1 in renal morphogenesis merits additional investigation.

    更新日期:2019-11-01
  • Is too much salt harmful? Yes.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Róbert Agócs,Dániel Sugár,Attila J Szabó

    The contribution of high sodium intake to hypertension and to the severity of immune-mediated diseases is still being heatedly debated in medical literature and in the lay media. This review aims to demonstrate two conflicting views on the topic, with the first part citing the detrimental effects of excessive salt consumption. Sodium plays a central role in volume and blood pressure homeostasis, and the positive correlation between sodium intake and blood pressure has been extensively researched. Despite the fact that the average of global daily salt consumption exceeds recommendations of international associations, health damage from excessive salt intake is still controversial. Individual differences in salt sensitivity are in great part attributed to this contradiction. Patients suffering from certain diseases as well as other vulnerable groups-either minors or individuals of full age-exhibit more pronounced blood pressure reduction when consuming a low-sodium diet. Furthermore, findings from the last two decades give insight into the concept of extrarenal sodium storage; however, the long-term consequences of this phenomenon are lesser known. Evidence of the relationship between sodium and autoimmune diseases are cited in the review, too. Nevertheless, further clinical trials are needed to clarify their interplay. In conclusion, for salt-sensitive risk groups in the population, even stricter limits of sodium consumption should be set than for young, healthy individuals. Therefore, the question raised in the title should be rephrased as follows: "how much salt is harmful" and "for whom is elevated salt intake harmful?"

    更新日期:2019-11-01
  • IgA nephropathy: is a new approach beyond proteinuria necessary?
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-02-20
    Eduardo Gutiérrez

    更新日期:2019-11-01
  • 更新日期:2019-11-01
  • A rare cause of proteinuria after kidney transplantation: Answers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-05-02
    Nilüfer Göknar,Seha Saygılı,Nur Canpolat,Yasemin Özlük,Işın Kılıçaslan,Lale Sever,Salim Çalışkan

    更新日期:2019-11-01
  • The implications of focal segmental glomerulosclerosis in children with IgA nephropathy.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Hernán Trimarchi,Rosanna Coppo

    Focal segmental glomerular sclerotic lesions in IgA nephropathy (IgAN), considered for years a chronic histologic feature related to proteinuria in remnant nephrons without any active role in the pathogenesis and progression of glomerular damage of IgAN, have been recently reconsidered. The Oxford classification of IgAN reported it as the "S" score and found it to be an independent risk factor for progression of IgAN. Its prognostic value was confirmed also in children. The identification of some histologic subvariants of the S lesion has produced interesting insights into different pathogenetic mechanisms of glomerular damage in IgAN. Tip lesion and podocyte hypertrophy are considered secondary to active podocytopathy and are correlated with higher levels of proteinuria and a faster decline in glomerular filtration rate. Moreover, endocapillary and mesangial hypercellularity might contribute in children with IgAN to formation and progression of S lesions. Considering the pathophysiology of these processes, children with some S features may benefit not only from nephroprotective measures but also from immunosuppression.

    更新日期:2019-11-01
  • A boy with IgA vasculitis and anuria: Answers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Vivek Sharma,Sidharth Kumar Sethi,Rupesh Raina,Shyam Bansal,S S Baijal,Vijay Kher

    更新日期:2019-11-01
  • A boy with IgA vasculitis and anuria: Questions.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Vivek Sharma,Sidharth Kumar Sethi,Rupesh Raina,Shyam Bansal,S S Baijal,Vijay Kher

    更新日期:2019-11-01
  • Correction to: Neonatal hypertension: cases, causes, and clinical approach.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-06-30
    Michelle C Starr,Joseph T Flynn

    The original version of this article unfortunately contained a mistake. Due to a production error, the wrong "Key summary points" were included. The correct key summary points are listed below.

    更新日期:2019-11-01
  • 更新日期:2019-11-01
  • Pediatric intradialytic hypotension: recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-02-09
    Rupesh Raina,Stephanie Lam,Hershita Raheja,Vinod Krishnappa,Daljit Hothi,Andrew Davenport,Deepa Chand,Gaurav Kapur,Franz Schaefer,Sidharth Kumar Sethi,Mignon McCulloch,Arvind Bagga,Timothy Bunchman,Bradley A Warady

    Intradialytic hypotension (IDH) is a common adverse event resulting in premature interruption of hemodialysis, and consequently, inadequate fluid and solute removal. IDH occurs in response to the reduction in blood volume during ultrafiltration and subsequent poor compensatory mechanisms due to abnormal cardiac function or autonomic or baroreceptor failure. Pediatric patients are inherently at risk for IDH due to the added difficulty of determining and attaining an accurate dry weight. While frequent blood pressure monitoring, dialysate sodium profiling, ultrafiltration-guided blood volume monitoring, dialysate cooling, hemodiafiltration, and intradialytic mannitol and midodrine have been used to prevent IDH, they have not been extensively studied in pediatric population. Lack of large-scale studies on IDH in children makes it difficult to develop evidence-based management guidelines. Here, we aim to review IDH preventative strategies in the pediatric population and outlay recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup. Without strong evidence in the literature, our recommendations from the expert panel reflect expert opinion and serve as a valuable guide.

    更新日期:2019-11-01
  • 更新日期:2019-11-01
  • Short stature in advanced pediatric CKD is associated with faster time to reduced kidney function after transplant.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-01-11
    Yijun Li,Larry A Greenbaum,Bradley A Warady,Susan L Furth,Derek K Ng

    BACKGROUND Among children who receive a kidney transplant, short stature is associated with a more complicated post-transplant course and increased mortality. Short stature prior to transplant may reflect the accumulated risk of multiple factors during chronic kidney disease (CKD); however, its relationship with post-transplant kidney function has not been well characterized. METHODS In the Chronic Kidney Disease in Children (CKiD) cohort restricted to children who received a kidney transplant, short stature (i.e., growth failure) was defined as age-sex-specific height < 3rd percentile. The outcome was time to estimated glomerular filtration rate (eGFR) < 45 ml/min/1.73 m2 after transplant. Parametric survival models, including adjustment for disease severity, socioeconomic status (SES), and parental height by inverse probability weighting, described the relative times to eGFR< 45 ml/min/1.73 m2. RESULTS Of 138 children (median CKD duration at transplant: 13 years), 20% (28) had short stature before the transplant. The median time to eGFR < 45 ml/min/1.73 m2 after kidney transplantation was 6.6 years and those with short stature had a significantly faster time to the poor outcome (log-rank p value 0.004). Children with short stature tended to have lower SES, nephrotic proteinuria, higher blood pressure, and lower mid-parental height before transplant. After adjusting for these variables, children with growth failure had 40% shorter time to eGFR < 45 ml/min/1.73 m2 than those with normal stature (relative time 0.60, 95%CI 0.32, 1.03). CONCLUSIONS Short stature was associated with a faster time to low kidney function after transplant. SES, disease severity, and parental height partially explained the association. Clinicians should be aware of the implications of growth failure on the outcome of this unique population, while continued attempts are made to define modifiable factors that contribute to this association.

    更新日期:2019-11-01
  • Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-01-05
    Gül Özçelik,Hafize Emine Sönmez,Sezgin Şahin,Ayşim Özağarı,Meral Torun Bayram,Rümeysa Yasemin Çiçek,Evrim Kargın Çakıcı,Elif Çomak,Kenan Barut,Nihal Şahin,Sevcan Bakkaloğlu,İbrahim Gökçe,Ali Düzova,Yelda Bilginer,Ceyhun Açarı,Engin Melek,Beltinge Demircioğlu Kılıç,Semanur Özdel,Amra Adroviç,Özgür Kasapçopur,Erbil Ünsal,Harika Alpay,Diclehan Orhan,Rezan Topaloğlu,Ruhan Düşünsel,Seza Özen

    OBJECTIVE Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS). METHODS Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically. RESULTS Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007). CONCLUSIONS Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis.

    更新日期:2019-11-01
  • Predictors of grade 3-5 vesicoureteral reflux in infants ≤ 2 months of age with pyelonephritis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-28
    Hilla Bahat,Mai Ben-Ari,Tomer Ziv-Baran,Amos Neheman,Ilan Youngster,Michael Goldman

    BACKGROUND This study aimed to assess predictors for grade 3-5 vesicoureteral reflux (VUR) in infants ≤ 2 months of age admitted for first urinary tract infection (UTI). METHODS Retrospective cohort study of 195 infants ≤ 2 months admitted to a pediatric ward for first UTI between 2006 and 2017. Clinical, laboratory, and imaging data were collected from electronic medical charts. We examined associations between grade 3-5 VUR and different patient characteristics. RESULTS Twenty infants (10%) were diagnosed with grade 3-5 VUR; all had fever. Infants with grade 3-5 VUR had higher blood neutrophil percentage (BNP) (65% vs. 46%, P < 0.001), higher neutrophil-to-lymphocyte ratio (NLR) (2.6 vs. 1.3, P < 0.001), more renal ultrasound abnormalities (prenatal 26% vs. 5%, P = 0.007; postnatal 84% vs. 55%, P = 0.015), and Pseudomonas UTI (15% vs. 1%, respectively, P < 0.001). NLR > 1.65 showed sensitivity 100% and specificity 61% for detecting grade 3-5 VUR. BNP > 53% showed sensitivity 100% and specificity 60% for detecting grade 3-5 VUR. BNP was the best single marker for grade 3-5 VUR with area under the curve (AUC) of 0.82 (95% CI 0.75-0.89). In a multivariate model, AUC for combination of BNP and hydronephrosis was 0.86 (95% CI 0.79-0.93, P = 0.007). CONCLUSIONS Infants ≤ 2 months of age admitted for a first UTI are at risk for grade 3-5 VUR and thus should undergo a voiding cystourethrography (VCUG) if their renal ultrasound is abnormal or if they have Pseudomonas UTI. Avoiding VCUG can be considered in afebrile infants and in infants with BNP < 53% or NLR < 1.65.

    更新日期:2019-11-01
  • Persistent fever in a pediatric renal transplant patient: Answers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-21
    Neziha Celebi,Jesus G Vallejo,Olive S Eckstein,Jessica Geer,Jyotinder N Punia,Ewa Elenberg

    更新日期:2019-11-01
  • Persistent fever in a pediatric renal transplant patient: Questions.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-21
    Neziha Celebi,Jesus G Vallejo,Olive S Eckstein,Jessica Geer,Jyotinder N Punia,Ewa Elenberg

    更新日期:2019-11-01
  • Haptoglobin degradation product as a novel serum biomarker for hematopoietic stem cell transplant-associated thrombotic microangiopathy.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-21
    Meredith P Schuh,Michael R Bennett,Adam Lane,Sonata Jodele,Benjamin L Laskin,Prasad Devarajan

    BACKGROUND Hematopoietic stem cell transplant (HSCT)-associated thrombotic microangiopathy (TA-TMA) is a well-known complication of HSCT and carries high risk of morbidity and mortality. A lack of consistent non-invasive diagnostic criteria can delay diagnosis and lead to irreversible organ damage. METHODS Serum samples of 100 patients that underwent HSCT at Cincinnati Children's Hospital were serially collected. Unbiased proteomic profiling by SELDI-TOF-MS was performed on serum from TA-TMA patients at baseline (pre-HSCT), 2 weeks before TMA diagnosis (pre-TMA), and at clinical TMA diagnosis. Two proteins with mass to charge ratios of 12-13 kDa were consistently elevated at the 2 week pre-TMA time point by SELDI-TOF, compared to control samples. Potential peptides were isolated and analyzed by liquid chromatography-tandem mass spectrometry (LC-MS/MS) on the Linear Trap Quadropole (LTQ). A MASCOT search identified haptoglobin fragments in the 12-17-kDa range. Western blot was performed to validate haptoglobin fragments as a potential biomarker. RESULTS Western blot of TA-TMA patients showed haptoglobin fragments at 12, 14, and 17 kDa that varied between baseline, pre-TMA, and TMA time points for each patient. By densitometric analysis, the 17-kDa fragment in the pre-TMA samples differed significantly from TMA diagnosis (p < 0.0001). There was no significant difference in the concentrations of the 12-kDa and 14-kDa fragments. CONCLUSION The 17-kDa haptoglobin degradation product may represent a novel early serum biomarker for TA-TMA that could potentially allow for earlier diagnosis and intervention.

    更新日期:2019-11-01
  • Low-dose rituximab is no less effective for nephrotic syndrome measured by 12-month outcome.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-20
    Andrew P Maxted,Rebecca A Dalrymple,Denise Chisholm,John McColl,Yincent Tse,Martin T Christian,Ben C Reynolds

    OBJECTIVE Rituximab is an effective treatment for children with steroid dependent or frequently relapsing nephrotic syndrome. The optimum dosing schedule for rituximab has not been established. We hypothesized that a single low dose of 375 mg/m2 would have comparable outcomes to higher doses in reducing the frequency of relapse and time to B cell reconstitution. METHODS We conducted a multicenter retrospective observational cohort study of children with steroid-sensitive frequently relapsing nephrotic syndrome. Data were extracted from clinical records including the dates of diagnosis, treatment, relapses, the use of concomitant immunosuppression, and lymphocyte subset profiling. Patients treated earlier received variable doses of rituximab, although typically two doses of 750 mg/m2. Later, patients received the current regimen of a single dose of 375 mg/m2. The primary outcome was an absence of clinically confirmed relapse 12 months following rituximab administration. Secondary outcomes were median time to relapse, probability of being relapse-free at 6 and 24 months and time to reconstitution of CD19+ B cells. RESULTS Sixty patients received 143 courses of rituximab. Seven different dosing regimen strategies were used, ranging between 375 and 750 mg/m2 per dose, with administration of 1-4 doses. There was no significant difference in event-free survival at 12 months between dosing strategies. The median time to reconstitution of B cells was not significantly different between groups. CONCLUSIONS Use of a single low-dose regimen of rituximab in the management of frequently relapsing nephrotic syndrome does not affect the probability of relapse at 12 months or time to B cell reconstitution compared to a conventional higher dose.

    更新日期:2019-11-01
  • Chronic kidney disease following twin-to-twin transfusion syndrome-long-term outcomes.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-19
    Nabil Ziad Melhem,Sarah Ledermann,Lesley Rees

    BACKGROUND Amongst other sequelae, acute kidney injury (AKI) is a well-recognised post-natal complication of twin-to-twin transfusion syndrome (TTTS). Despite this, there has been a lack of data reporting long-term renal outcomes. Our aim was to report the long-term renal outcomes of infants born with TTTS. METHODS We performed a retrospective case note review of all infants referred to our centre between 1998 and 2018 with a primary diagnosis of TTTS. Subjects with confirmed TTTS were divided into a chronic kidney disease (CKD) group and a non-CKD group for comparison. RESULTS Twenty-six infants with TTTS were included for analysis. Eight (31%) subjects developed CKD. Within the CKD group, 50% went on to require long-term renal replacement therapy (RRT) of whom all underwent renal transplantation. For subjects who had neonatal AKI, cumulative survival rate before RRT at 5 and 10 years was 79% and 70%, respectively. Subjects with CKD had a significantly higher incidence of AKI in the neonatal period and were more likely to be the donor twin. Gestational age at birth, gender, antenatal interventions and comorbidities did not affect long-term renal outcome between the two groups. CONCLUSION This is the first long-term follow-up study demonstrating that CKD progressing to the need for RRT can develop after TTTS. Donor-twin status and neonatal AKI associated with adverse long-term outcomes warranting long-term surveillance in this group.

    更新日期:2019-11-01
  • Neurocognitive and functional outcomes at 5 years of age after renal transplant in early childhood.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-17
    Jillian Popel,Rachel Joffe,Bryan V Acton,Gwen Y Bond,Ari R Joffe,Julian Midgley,Charlene M T Robertson,Reg S Sauve,Catherine J Morgan

    BACKGROUND Clinicians often use information about developmental outcomes in decision-making around offering complex, life-saving interventions in children such as dialysis and renal transplant. This information in children with end-stage renal disease (ESRD) is limited, particularly when ESRD onset is in infancy or early childhood. METHODS Using data from an ongoing prospective, longitudinal, inception cohort study of children with renal transplant before 5 years of age, we evaluated (1) the risk of adverse neurocognitive and functional outcomes at 5 years of age and (2) predictors of developmental outcomes. RESULTS We found evidence of neurocognitive sequelae of ESRD in very young children; however, developmental outcomes appear remarkably better when compared with findings of two or three decades ago. Less time on dialysis predicted higher developmental scores, and hemodialysis was associated with poorer developmental outcomes. CONCLUSIONS Our data suggest that renal replacement therapies in young children are associated with acceptable developmental outcome. Programs to identify those with developmental delays and provide early intervention may allow achievement of the child's full potential.

    更新日期:2019-11-01
  • The aminoglycoside geneticin permits translational readthrough of the CTNS W138X nonsense mutation in fibroblasts from patients with nephropathic cystinosis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-11-11
    Emma J Brasell,LeeLee Chu,Reyhan El Kares,Jung Hwa Seo,Robin Loesch,Diana M Iglesias,Paul Goodyer

    BACKGROUND Cystinosis is an ultrarare disorder caused by mutations of the cystinosin (CTNS) gene, encoding a cystine-selective efflux channel in the lysosomes of all cells of the body. Oral therapy with cysteamine reduces intralysosomal cystine accumulation and slows organ deterioration but cannot reverse renal Fanconi syndrome nor prevent the eventual need for renal transplantation. A definitive therapeutic remains elusive. About 15% of cystinosis patients worldwide carry one or more nonsense mutations that halt translation of the CTNS protein. Aminoglycosides such as geneticin (G418) can bind to the mammalian ribosome, relax translational fidelity, and permit readthrough of premature termination codons to produce full-length protein. METHODS To ascertain whether aminoglycosides permit readthrough of the most common CTNS nonsense mutation, W138X, we studied the effect of G418 on patient fibroblasts. RESULTS G418 treatment induced translational readthrough of CTNSW138X constructs transfected into HEK293 cells and expression of full-length endogenous CTNS protein in homozygous W138X fibroblasts. CONCLUSIONS Reduction in intracellular cystine indicates that the CTNS protein produced is functional as a cystine transporter. Interestingly, similar effects were seen even in W138X compound heterozygotes. These studies establish proof-of-principle for the potential of aminoglycosides to treat cystinosis and possibly other monogenic diseases caused by nonsense mutations.

    更新日期:2019-11-01
  • An unusual cause of nephrotic syndrome: Answers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-11-09
    Zeynep Yuruk Yildirim,Melis Ozkan,Alev Yilmaz,Hülya Kayserili,Cemile Pehlivanoglu,Sevinc Emre,Ahmet Nayir

    更新日期:2019-11-01
  • An unusual cause of nephrotic syndrome: Questions.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-11-09
    Zeynep Yuruk Yildirim,Melis Ozkan,Alev Yilmaz,Hülya Kayserili,Cemile Pehlivanoglu,Sevinc Emre,Ahmet Nayir

    更新日期:2019-11-01
  • Dangerous hyperkalemia in a newborn: Answers.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-11-02
    Aliza Mittal,Daisy Khera,Varuna Vyas,Bharat Choudhary,Kuldeep Singh

    更新日期:2019-11-01
  • Dangerous hyperkalemia in a newborn: Questions.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-11-02
    Aliza Mittal,Daisy Khera,Varuna Vyas,Bharat Choudhary,Kuldeep Singh

    更新日期:2019-11-01
  • Lisinopril versus lisinopril and losartan for mild childhood IgA nephropathy: a randomized controlled trial (JSKDC01 study).
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-10-05
    Yuko Shima,Koichi Nakanishi,Mayumi Sako,Mari Saito-Oba,Yuko Hamasaki,Hiroshi Hataya,Masataka Honda,Koichi Kamei,Kenji Ishikura,Shuichi Ito,Hiroshi Kaito,Ryojiro Tanaka,Kandai Nozu,Hidefumi Nakamura,Yasuo Ohashi,Kazumoto Iijima,Norishige Yoshikawa,

    BACKGROUND Persistent proteinuria seems to be a risk factor for progression of renal disease. Its reduction by angiotensin-converting inhibitors (ACEIs) or angiotensin II receptor blockers (ARBs) is renoprotective. Our previous pilot study showed that 2-year lisinopril therapy is effective and safe for children with mild IgA nephropathy. When combined with ACEI and ARB, reported results are of greater decrease in proteinuria than monotherapy in chronic glomerulonephritis, including IgA nephropathy. To date, however, there have been no randomized controlled trials in children. METHODS This is an open-label, multicenter, prospective, and randomized phase II controlled trial of 63 children with biopsy-proven proteinuric mild IgA nephropathy. We compared efficacy and safety between patients undergoing lisinopril monotherapy and patients undergoing combination therapy of lisinopril and losartan to determine better treatment for childhood proteinuric mild IgA nephropathy. RESULTS There was no difference in proteinuria disappearance rate (primary endpoint) between the two groups (cumulative disappearance rate of proteinuria at 24 months: 89.3% vs 89% [combination vs monotherapy]). Moreover, there were no significant differences in side effects between the two groups. CONCLUSIONS We propose lisinopril monotherapy as treatment for childhood proteinuric mild IgA nephropathy as there are no advantages of combination therapy. CLINICAL TRIAL REGISTRATION Clinical trial registry, UMIN ID C000000006, https://www.umin.ac.jp .

    更新日期:2019-11-01
  • Efficacy of low-dose daily versus alternate-day prednisolone in frequently relapsing nephrotic syndrome: an open-label randomized controlled trial.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-09-09
    Menka Yadav,Aditi Sinha,Priyanka Khandelwal,Pankaj Hari,Arvind Bagga

    BACKGROUND While patients with frequently relapsing nephrotic syndrome (FRNS) are initially treated with long-term alternate-day prednisolone, relapses and adverse effects are common. In an open-label randomized controlled trial, we compared the efficacy of therapy with low-dose daily to standard alternate-day prednisolone in reducing relapse rates over 12-month follow-up. METHODS Consecutive patients, aged 2-18 years, with FRNS were included. Following therapy of relapse, prednisolone was tapered to 0.75 mg/kg on alternate days. Stratifying for steroid dependence, patients were randomly assigned to prednisolone at 0.2-0.3 mg/kg daily or 0.5-0.7 mg/kg alternate day for 12 months. Relapses were treated with daily prednisolone, followed by return to intervention. Primary outcome was the incidence of relapses. Proportion with therapy failure (≥ 2 relapses in any 6 months or significant steroid toxicity) and sustained remission, cumulative prednisolone intake and adverse events were evaluated. RESULTS Patients receiving daily prednisolone (n = 30) showed significantly fewer relapses than those on alternate-day therapy (n = 31) (0.55 relapses/person-year versus 1.94 relapses/person-year; incidence rate ratio 0.28; 95% CI 0.15, 0.52). Daily therapy was associated with higher rates of sustained remission at 6 months (73.3 versus 48.4%) and 1 year (60 versus 31.6%; log rank p = 0.013), lower rates of treatment failure at 6 months (3.3 versus 32.8%) and 1 year (6.7 versus 57.4%; p < 0.0001), and lower prednisolone use (0.27 ± 0.07 versus 0.39 ± 0.19 mg/kg/day; p = 0.003). Three and two patients need to receive the study intervention to enable sustained remission and prevent treatment failure, respectively. CONCLUSIONS In patients with FRNS, daily administration of low-dose prednisolone is more effective than standard-dose alternate day therapy in lowering relapse rates, sustaining remission, and enabling steroid sparing.

    更新日期:2019-11-01
  • Uric acid and progression of chronic kidney disease.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-06-23
    Donald J Weaver

    The association between serum uric acid levels and human disease has garnered intense interest over the last decade including chronic kidney disease. Animal studies have provided evidence for a potential mechanistic role of uric acid in promoting progression of chronic kidney disease. Epidemiologic studies have also suggested an association between elevated serum uric acid levels and worsening renal function in the general population as well as in patients with chronic kidney disease. However, there is currently insufficient evidence to recommend the use of uric acid-lowering therapy to delay progression of chronic kidney disease in this patient population. Adequately powered, randomized, placebo-controlled trials are required to more precisely evaluate the risk and benefits of uric acid-lowering therapy in pediatric patients.

    更新日期:2019-11-01
  • Treating the idiopathic nephrotic syndrome: are steroids the answer?
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-06-06
    Georges Deschênes,Claire Dossier,Julien Hogan

    The use of steroids in idiopathic nephrotic syndrome is the major discovery of the twentieth century in the field of pediatric nephrology. At onset of the twenty-first century, steroids remain the first line of treatment at first flare. All the protocols to treat the first flare are similar by a common sequence including a first phase of daily prednisolone/prednisone at a dose of 60 mg/m2/day for at least 4 weeks followed by an alternate-day regimen for several weeks. It appears that a cumulated dose of 2240 mg/m2 given in 8 weeks at the first flare without tapering sequence is not inferior to increased dose and duration in terms of prevalence of frequent relapsers and the subsequent cumulated dose of steroids at 24 months of follow-up. A higher cumulated dose might only be interesting in patients aged below 4 years although a formal demonstration is still missing. Several retrospective studies are concordant to suggest that intravenous methylprednisolone pulses are useful to reach a full urinary remission in case of oral resistance to 4 weeks of oral prednisone/prednisolone. A majority of patients have multiple relapses after the treatment of the first flare and half meet the definition of steroid dependency. In those patients, long-lasting alternate-day prednisone/prednisolone therapy does not lead to long-lasting remission, opening the question of the best strategy of immunosuppression.

    更新日期:2019-11-01
  • Neonatal hypertension: cases, causes, and clinical approach.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-05-29
    Michelle C Starr,Joseph T Flynn

    Neonatal hypertension is increasingly recognized as dramatic improvements in neonatal intensive care, advancements in our understanding of neonatal physiology, and implementation of new therapies have led to improved survival of premature infants. A variety of factors appear to be important in determining blood pressure in neonates, including gestational age, birth weight, and postmenstrual age. Normative data on neonatal blood pressure values remain limited. The cause of hypertension in an affected neonate is often identified with careful diagnostic evaluation, with the most common causes being umbilical catheter-associated thrombosis, renal parenchymal disease, and chronic lung disease. Clinical expertise may need to be relied upon to decide the best approach to treatment in such patients, as data on the use of antihypertensive medications in this age group are extremely limited. Available data suggest that long-term outcomes are usually good, with resolution of hypertension in most infants. In this review, we will take a case-based approach to illustrate these concepts and to point out important evidence gaps that need to be addressed so that management of neonatal hypertension may be improved.

    更新日期:2019-11-01
  • Focus on neonatal and infantile onset of nephrogenic syndrome of inappropriate antidiuresis: 12 years later.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-03-17
    Flaminia Bardanzellu,Maria Cristina Pintus,Valentina Masile,Vassilios Fanos,Maria Antonietta Marcialis

    Nephrogenic syndrome of inappropriate antidiuresis (NSIAD), first described in 2005, is a rare genetic X-linked disease, presenting with hyponatremia, hyposmolarity, euvolemia, inappropriately concentrated urine, increased natriuresis, and undetectable or very low arginine-vasopressine (AVP) circulating levels. It can occur in neonates, infants, or later in life. NSIAD must be early recognized and treated to prevent severe hyponatremia, which can show a dangerous impact on neonatal outcome. In fact, it potentially leads to death or, in case of survival, neurologic sequelae. This review is an update of NSIAD 12 years after the first description, focusing on reported cases of neonatal and infantile onset. The different molecular patterns affecting the AVP receptor 2 (V2R) and determining its gain of function are reported in detail; moreover, we also provide a comparison between the different triggers involved in the development of hyponatremia, the evolution of the symptoms, and modality and efficacy of the different treatments available.

    更新日期:2019-11-01
  • Zebrafish as a model for kidney function and disease.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-03-05
    Priya Outtandy,Claire Russell,Robert Kleta,Detlef Bockenhauer

    Kidney disease is a global problem with around three million people diagnosed in the UK alone and the incidence is rising. Research is critical to develop better treatments. Animal models can help to better understand the pathophysiology behind the various kidney diseases and to screen for therapeutic compounds, but the use especially of mammalian models should be minimised in the interest of animal welfare. Zebrafish are increasingly used, as they are genetically tractable and have a basic renal anatomy comparable to mammalian kidneys with glomerular filtration and tubular filtration processing. Here, we discuss how zebrafish have advanced the study of nephrology and the mechanisms underlying kidney disease.

    更新日期:2019-11-01
  • Voiding dysfunction in children. Pelvic-floor exercises or biofeedback therapy: a randomized study.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-09-13
    Mônica Vasconcelos,Eleonora Lima,Letícia Caiafa,Alessandra Noronha,Renata Cangussu,Suzely Gomes,Raquel Freire,Maria Teresa Filgueiras,Junia Araújo,Gisele Magnus,Cláudia Cunha,Enrico Colozimo

    Fifty-six patients 5.9-15.2 years old with dysfunctional elimination syndrome (DES) unimproved by previous therapies were randomly distributed into two voiding training programs: group 1 contained 26 patients submitted to 24 training sessions over a 3-month period; group 2 contained 30 patients submitted to 16 sessions over a 2-month period. Both groups adhered to a voiding and drinking schedule, received instruction on adequate toilet posture, were reinforced through the maintenance of voiding diaries, and went through proprioceptive and pelvic floor muscle training (Kegel exercises). Group 2 patients also received biofeedback therapy. Clinical evaluation was carried out before each program's initiation and 1, 6, and 12 months after each program's termination. All patients were submitted to renal ultrasonography and dynamic ultrasonography before and 6 months after each program's conclusion. Millivoltage recordings of pelvic floor muscles were compared before and after training. Urinary continence was improved after completion of either training program. Only those patients who received biofeedback training showed a significant decrease in postvoiding residual (PVR) urine as detected by dynamic ultrasonography. Our results show that either training regime can reduce episodic urinary incontinence and urinary tract infection but that further study is required to identify the optimal training duration.

    更新日期:2019-11-01
  • Intensified and daily hemodialysis in children might improve statural growth.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-08-31
    Michel Fischbach,Joëlle Terzic,Soraya Menouer,Céline Dheu,Sylvie Soskin,Agnès Helmstetter,Marie-Claire Burger

    In children conventional hemodialysis does not often improve growth. We determined linear growth in five children on in-center intensified and daily hemodialysis (IDd) regimen, with a mean age of 8 years 7 months at enrollment. Four of five were on growth hormone started for a median of 28.5 months before IDd. IDd was delivered 5 to 6 times weekly, for three hours each session. Mean follow up of IDd was 18.6 months. Dropout from IDd was kidney transplantation (n=4) or transfer to another center (n=1). IDd and free diet improved appetite, thereby protein intake, was above 2 g/kg/BW. Median weekly Kt/V(urea) was 9.1 (8.7 to 10.4). Predialysis phosphorus blood levels were higher at the start (2.04+/-0.34 mmol/L) than at end of IDd (1.39+/-0.41 mmol/L) without need for carbonate of calcium in four of five cases. During conventional dialysis ht SDS decreased from -0.8 to -1.44, which occurred predominantly before rhGH start. Conversion to IDd significantly increased growth velocity to a mean of 13 cm/year (10.3-18) with a mean change of +1.84 ht SDS/year (0.4 to 2.7). This preliminary report suggests the potential efficacy of IDd regimen in promising growth velocity, either directly from a higher dialysis dose or indirectly through an improved nutritional status.

    更新日期:2019-11-01
  • Low hair selenium and plasma glutathione peroxidase in children with chronic renal failure.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-08-29
    Erol Ortaç,Ozan Ozkaya,Recep Saraymen,Nurdan Yildiz,Abdülkerim Bedir,Necla Buyan,Kenan Bek,Ali Okuyucu,Kemal Baysal

    Selenium (Se) is a trace element that incorporates into the selenoenzyme glutathione peroxidase (GSH-Px). There are conflicting results regarding the Se levels and activity of GSH-Px in adult uremic patients. The aim of this study was to determine (1) the hair Se status, (2) the possible relation between the hair Se status and the antioxidant enzyme, GSH-Px, and (3) the influence of different treatment procedures on hair Se status and GSH-Px activity in children with CRI, those treated conservatively and those on HD and on CAPD. Ninety-three patients, including 32 patients with CRI, treated conservatively, 42 PD patients, 19 HD patients and 34 healthy children were enrolled in the study. The hair Se level was measured by the atomic absorption spectrophotometer method. Plasma GSH-Px activity was determined using a Randox test combination (RANSEL). Hair Se levels were significantly lower in the CRI, CAPD, and HD groups when compared to the control group (P=0.001, P=0.001, and P=0.001, respectively). Plasma GSH-Px activity was significantly lower in the CRI, CAPD, and HD groups when compared to the control group (P=0.001, P=0.001, and P=0.001, respectively). Plasma GSH-Px activity correlated with the GFR in patients with CRI and the control group (P=0.000; r(2)=0.60). There was no correlation between plasma GSH-Px and hair Se levels in the patient and control groups. These results revealed a decreased hair Se level and impaired antioxidative capacity in children with CRI on CAPD and HD. The lack of any relation between plasma GSH-Px and hair Se suggests that plasma GSH-Px is not a good marker of Se stores.

    更新日期:2019-11-01
  • The novel bone alkaline phosphatase B1x isoform in children with kidney disease.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-08-26
    Diana Swolin-Eide,Sverker Hansson,Lasse Larsson,Per Magnusson

    The bone alkaline phosphatase (BALP) B1x isoform has previously only been identified in some adults with chronic kidney disease on dialysis and in human bone tissue. Twenty-nine patients, 3-20 years of age, with reduced renal function due to a variety of kidney diseases were examined. We measured parathyroid hormone (PTH), biointact (whole 1-84) PTH, osteoprotegerin (OPG), CrossLaps (CTX), tartrate-resistant acid phosphatase isoform 5b (TRACP 5b) type I procollagen intact amino-terminal propeptide (PINP), osteocalcin, total alkaline phosphatase (ALP), and BALP isoforms B/I, B1x, B1, and B2. Fifty percent higher levels were detected of PTH vs. biointact PTH, demonstrating non-(1-84) PTH fragments detected by the PTH assay. Increased activities were found in five, four, and three patients for total ALP, B1, and B2, respectively. Sixteen (55%) patients had increased B/I levels. B1x was identified in two (7%) patients, who had OPG levels in the higher range independently of age, glomerular filtration rate (GFR), and biointact PTH. B1x was identified prior to and after 9 days of growth hormone (GH) therapy in one patient but not after 1, 3, 6, and 12 months, however. In conclusion, our study demonstrates that the novel BALP B1x isoform is occasionally found to be present in children with kidney disease but to a lesser degree in comparison with adults with chronic kidney disease on dialysis. It is essential to perform bone histomorphometry for future investigations in order to elucidate the exact nature of circulating B1x in patients with kidney disease for accurate classification of type of renal bone disease.

    更新日期:2019-11-01
  • Energy homeostasis and cachexia in chronic kidney disease.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-08-10
    Robert H Mak,Wai Cheung

    Loss of protein stores, presenting as clinical wasting, is reported to have a prevalence of 30-60% and is an important risk factor for mortality in chronic kidney disease (CKD) patients. There is debate as to whether the clinical wasting in CKD patients represents malnutrition or cachexia. Malnutrition results from inadequate intake of nutrients, despite a good appetite, and manifests as weight loss associated with adaptive metabolic responses such as decreased basic metabolic rate and preservation of lean body mass at the expense of fat mass. Furthermore, the abnormalities in malnutrition can usually be overcome simply by supplying more food or altering the composition of the diet. In contrast, cachexia is characterized by maladaptive responses such as anorexia, elevated basic metabolic rate, wasting of lean body tissue, and underutilization of fat tissue for energy. Diet supplementation and intradialytic parenteral nutrition have not been successful in reversing cachexia in CKD. The etiology of cachexia in CKD is complex and multifactorial. Two major factors causing muscle wasting in uremia are acidosis and decreased insulin responses. Inflammation secondary to cytokines may also play a significant role. The hypoalbuminemia of CKD patients is principally associated with inflammation and not changes in food intake. There is also recent evidence that hypothalamic neuropeptides may be important in the downstream signaling of cytokines in the pathogenesis of cachexia in CKD. Elevated circulating levels of cytokines, such as leptin, may be an important cause of uremia-associated cachexia via signaling through the central melanocortin system. Further research into the molecular pathways leading to cachexia may lead to novel therapeutic therapy for this devastating and potentially fatal complication of CKD.

    更新日期:2019-11-01
  • Study on hepatitis B virus pre-S/S gene mutations of renal tissues in children with hepatitis B virus-associated membranous nephropathy.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-06-23
    Se Eun Kim,Yeong Hong Park,Woo Yeong Chung

    This study aims to clarify the prevalence and significance of the emergence of hepatitis B virus (HBV) pre-S/S mutations in children with hepatitis B virus-associated membranous nephropathy (HBVMN). Direct sequencing of polymerase chain reaction products of renal tissue samples that were obtained via percutaneous renal biopsy from seven children revealed the presence of HBV DNA. Seven adr subtypes were analyzed. Deletions in the HBV pre-S region were observed once per seven patients. The deletions were noted in both the pre-S1 (27 bp) and pre-S2 (60 bp) regions. Various point mutations in the HBV pre-S region were detected in all seven patients and proved to be more frequent in the pre-S1 region than in the S2 region. Point mutations in the HBV S region were detected in six patients. Among these mutations, the mutation in the "a" determinant region was noted in five patients. No deletion, however, was observed in the HBV S region. These observations suggested that deletions and point mutations in the HBV pre-S1 and pre-S2 regions and point mutations in the HBV S region, especially the "a" determinant region, are common frequent findings. These results also suggested that HBV pre-S/S region mutations may be involved in the pathogenesis in children with HBVMN.

    更新日期:2019-11-01
  • Computational simulation of renal biopsy accuracy in focal segmental glomerulosclerosis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-06-15
    Asher D Schachter

    The goal of this study was to estimate the diagnostic and prognostic accuracy of renal biopsies in focal segmental glomerulosclerosis (FSGS), accounting for the focal nature of affected glomeruli. Computational simulations were performed on a total of 138,600 virtual kidneys, across a range of FSGS involvement. Simulations were designed to address the diagnostic accuracy of renal biopsies, and the biopsy characteristics required to reflect accurately the true degree of involvement of FSGS in the entire kidney or just the juxtamedullary (JM) region. The diagnostic accuracy of renal biopsies for the detection of at least one FSGS glomerulus exceeded 80% when 10-20% of the kidney was affected by FSGS. Hundreds to thousands of biopsy glomeruli were required to characterize reliably the true extent of FSGS when fewer than 75% of the kidney's glomeruli were affected. Renal biopsies with an average of 20 glomeruli did not accurately reflect the extent of FSGS in kidneys until at least 80% of all glomeruli in the kidney were affected. Targeting JM glomeruli did not result in significant improvements in the prognostic performance characteristics of renal biopsies. These findings suggest that conventional renal biopsies might be inadequate for characterizing the extent of FSGS.

    更新日期:2019-11-01
  • A case of unfulfilled expectations. Cytokines in idiopathic minimal lesion nephrotic syndrome.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2006-03-10
    Carlos E Araya,Clive H Wasserfall,Todd M Brusko,Wei Mu,Mark S Segal,Richard J Johnson,Eduardo H Garin

    Idiopathic minimal lesion nephrotic syndrome (IMLNS) was proposed to be a disorder of T-cell dysfunction by Shalhoub in 1974. The mechanisms by which T-cells increase glomerular permeability have remained elusive (and unproven). There is evidence that IMLNS may be due to a circulating factor released from activated T-cells. In recent years, efforts have been made to identify this pathogenetic cytokine as well as to understand the mechanism(s) for the increased release of this factor. This review attempts to critically analyze the available published data. Using different methodologies, investigators have focused on the production of cytokines in patients with IMLNS during relapse and remission. This has resulted in a plethora of data without definitive conclusions. The pathogenetic cytokine has not been identified, and it is questionable whether there is a Th2 dominance in IMLNS. The review of the available data illustrates the difficulties encountered when one is studying the cytokine secretory pattern in patients with IMLNS. Differences in patient population, type of cells studies, sample preservation, and methodology used to measure cytokines are some of the factors that could account for the disparity of observed results.

    更新日期:2019-11-01
  • Referees for 2019.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null

    更新日期:2019-11-01
  • CoenzymeQ10 therapy in two sisters with CoQ6 mutations with long-term follow-up.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-28
    Mustafa Koyun,Elif Çomak,Sema Akman

    更新日期:2019-11-01
  • Acute kidney injury in neonatal encephalopathy: an evaluation of the AWAKEN database.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-08-30
    Megan J Kirkley,Louis Boohaker,Russell Griffin,Danielle E Soranno,Jason Gien,David Askenazi,Katja M Gist,

    BACKGROUND Acute kidney injury (AKI) is common in neonatal encephalopathy (NE) and is associated with worse outcomes. Our objectives were to determine the incidence, risk factors, and outcomes of AKI in infants with NE. METHODS We performed a retrospective analysis of infants ≥ 34 weeks' gestational age with a diagnosis of NE from the Analysis of Worldwide Acute Kidney injury Epidemiology in Neonates (AWAKEN) database. AKI was defined using the modified Kidney Disease Improving Global Outcomes criteria. Perinatal and postnatal factors were evaluated. Multivariate logistic and linear regressions were performed. RESULTS One hundred and thirteen patients with NE were included. 41.6% (47) developed AKI. Being born outside the admitting institution (OR 4.3; 95% CI 1.2-14.8; p = 0.02), intrauterine growth restriction (OR 10.3, 95% CI 1.1-100.5; p = 0.04), and meconium at delivery (OR 2.8, 95% CI 1.04-7.7; p = 0.04) conferred increased odds of AKI. After controlling for confounders, infants with AKI stayed in the hospital an average of 8.5 days longer than infants without AKI (95% CI 0.79-16.2 days; p = 0.03). CONCLUSIONS In this multi-national analysis, several important perinatal factors were associated with AKI and infants with both NE and AKI had longer length of stay than NE alone. Future research aimed at early AKI detection, renoprotective management strategies, and understanding the long-term renal consequences is warranted in this high-risk group of patients.

    更新日期:2019-11-01
  • Correction to: An open-label, single-dose study to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of cinacalcet in pediatric subjects aged 28 days to < 6 years with chronic kidney disease receiving dialysis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2018-12-12
    Winnie Y Sohn,Anthony A Portale,Isidro B Salusky,Hao Zhang,Lucy L Yan,Bella Ertik,Shahnaz Shahinfar,Edward Lee,Bastian Dehmel,Bradley A Warady

    The original version of this article unfortunately contained three mistakes. In Table 1, the last line under "Key Inclusion Criteria" should read "Normal or clinically acceptable ECGs at screening and at day - 1." In addition, the abbreviation "IP" in the legend to Table 1 stands for "investigational product."

    更新日期:2019-11-01
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