当前期刊: Pediatric Nephrology Go to current issue    加入关注   
显示样式:        排序: 导出
我的关注
我的收藏
您暂时未登录!
登录
  • Educational review: role of the pediatric nephrologists in the work-up and management of kidney stones.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Carmen Inés Rodriguez Cuellar,Peter Zhan Tao Wang,Michael Freundlich,Guido Filler

    BACKGROUND The incidence of nephrolithiasis in children and adolescents is increasing and appears to double every 10 years. The most important role of the pediatric nephrologist is to diagnose and modify various metabolic and non-metabolic risk factors, as well as prevent long-term complications especially in the case of recurrent nephrolithiasis. OBJECTIVE The purpose of this review is to summarize the existing literature on the etiology and management of pediatric nephrolithiasis. RESULTS The incidence of kidney stones is increasing; dietary and environmental factors are probably the main causes for this increased incidence. In most pediatric patients, the etiology for the kidney stones can be identified. Metabolic factors, such as hypercalciuria and hypocitraturia, urinary tract infection, and urinary stasis, constitute leading causes. Herein, we review the etiologies, diagnostic work-up, and treatment options for the most prevalent causes of kidney stones. The detrimental effects of excessive dietary sodium, reduced fluid intake, and the benefits of plant-based over animal-based protein consumption on urinary crystal formation are discussed. We also review the long-term complications. CONCLUSIONS Pediatric nephrologists have an important role in the diagnostic work-up and prevention of recurring nephrolithiasis.

    更新日期:2020-01-17
  • Treatment of hyperphosphatemia: the dangers of aiming for normal PTH levels
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-12-10
    Dieter Haffner, Maren Leifheit-Nestler

    Abstract Secondary hyperparathyroidism is part of the complex of chronic kidney disease-associated mineral and bone disorders (CKD-MBD) and is linked with high bone turnover, ectopic calcification, and increased cardiovascular mortality. Therefore, measures for CKD-MBD aim at lowering PTH levels, but there is no general consensus on optimal PTH target values. This manuscript is part of a pros and cons debate for keeping PTH levels within the normal range in children with CKD, focusing on the cons. We conclude that a modest increase in PTH most likely represents an appropriate adaptive response to declining kidney function in patients with CKD stages 2–5D, due to phosphaturic effects and increasing bone resistance. There is no evidence for strictly keeping PTH levels within the normal range in CKD patients with respect to bone health and cardiovascular outcome. In addition, the potentially adverse effects of PTH-lowering measures, such as active vitamin D and calcimimetics, must be taken into account. We suggest that PTH values of 1–2 times the upper normal limit (ULN) may be acceptable in children with CKD stage 2–3, and that PTH levels of 1.7–5 times UNL may be optimal in patients with CKD stage 4–5D. However, standard care of CKD-MBD in children relies on a combination of different measures in which the observation of PTH levels is only a small part of, and trends in PTH levels rather than absolute target values should determine treatment decisions in patients with CKD as recommended by the 2017 KDIGO guidelines.

    更新日期:2020-01-17
  • The dietary management of calcium and phosphate in children with CKD stages 2-5 and on dialysis-clinical practice recommendation from the Pediatric Renal Nutrition Taskforce.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Louise McAlister,Pearl Pugh,Laurence Greenbaum,Dieter Haffner,Lesley Rees,Caroline Anderson,An Desloovere,Christina Nelms,Michiel Oosterveld,Fabio Paglialonga,Nonnie Polderman,Leila Qizalbash,José Renken-Terhaerdt,Jetta Tuokkola,Bradley Warady,Johan Vande Walle,Vanessa Shaw,Rukshana Shroff

    In children with chronic kidney disease (CKD), optimal control of bone and mineral homeostasis is essential, not only for the prevention of debilitating skeletal complications and achieving adequate growth but also for preventing vascular calcification and cardiovascular disease. Complications of mineral bone disease (MBD) are common and contribute to the high morbidity and mortality seen in children with CKD. Although several studies describe the prevalence of abnormal calcium, phosphate, parathyroid hormone, and vitamin D levels as well as associated clinical and radiological complications and their medical management, little is known about the dietary requirements and management of calcium (Ca) and phosphate (P) in children with CKD. The Pediatric Renal Nutrition Taskforce (PRNT) is an international team of pediatric renal dietitians and pediatric nephrologists, who develop clinical practice recommendations (CPRs) for the nutritional management of various aspects of renal disease management in children. We present CPRs for the dietary intake of Ca and P in children with CKD stages 2-5 and on dialysis (CKD2-5D), describing the common Ca- and P-containing foods, the assessment of dietary Ca and P intake, requirements for Ca and P in healthy children and necessary modifications for children with CKD2-5D, and dietary management of hypo- and hypercalcemia and hyperphosphatemia. The statements have been graded, and 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.

    更新日期:2020-01-17
  • Diagnostics, treatment, and immune response in BK polyomavirus infection after pediatric kidney transplantation.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Thurid Ahlenstiel-Grunow,Lars Pape

    After pediatric kidney transplantation BK polyomavirus (BKPyV) infections are associated with an increased risk of graft loss by BKPyV-associated nephropathy (BkPyVAN). However, suitable prognostic markers for the individual outcome of BKPyV infections are missing and the management of therapeutic interventions remains a challenge to the success of pediatric kidney transplantation. This review gives an overview on current diagnostic and therapeutic strategies in the field of BKPyV infections after pediatric kidney transplantation. Methods determining the individual immune response to BKPyV are described and their usability is discussed. There is growing evidence that BKPyV-specific T cells (BKPyV-Tvis) may serve as prognostic markers in order to steer immunosuppressive therapy in pediatric kidney recipients with BKPyV viremia in future. Prospective randomized trials in viremic kidney recipients comparing Tvis-steered therapeutic intervention with standard reduction of immunosuppression are needed before implementation of BKPyV-Tvis monitoring in routine care of BKPyV infections.

    更新日期:2020-01-17
  • Prognostic role of acute kidney injury on long-term outcome in infants with hypoxic-ischemic encephalopathy
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-12-11
    Francesco Cavallin, Giulia Rubin, Enrico Vidal, Elisa Cainelli, Luca Bonadies, Agnese Suppiej, Daniele Trevisanuto

    Abstract Background The objective of this study was to evaluate the prognostic role of postnatal acute kidney injury (AKI) on neurodevelopmental outcome in infants with hypoxic-ischemic encephalopathy (HIE) receiving therapeutic hypothermia (TH). Methods This is a prospective observational study including all neonates with HIE receiving TH between 2009 and 2016 at a single center. AKI was classified according to the Kidney Disease: Improving Global Outcomes definition modified for neonatal age. Child development was assessed using the Griffiths Mental Development Scales (GMDS). Study outcome was defined as unfavorable outcome (including death or disability according to GMDS) or favorable otherwise, at 12 and 24 months. Results One-hundred and one neonates (median gestational age 39 weeks) were included. AKI was diagnosed in 10 neonates (10%). Seven patients died within the first year, 35 patients had disability at 12 months, and 45 patients at 24 months. AKI was associated with increased likelihood of unfavorable outcome at 24 months (100% vs. 59% in neonates without AKI; p = 0.01). AKI showed good positive predictive value (1.00, 95% CI 0.71–1.00) and specificity (1.00, 95% CI 0.88–1.00), but poor negative predictive value (0.41, 95% CI 0.30–0.52) and sensitivity (0.19, 95% CI 0.11–0.32) at 24 months. Conclusions AKI might be a reliable indicator of death or long-term disability in infants with HIE receiving TH, but the absence of AKI does not guarantee a favorable long-term outcome.

    更新日期:2020-01-17
  • Steroid therapy in children with IgA nephropathy.
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-02-20
    Alexandra Cambier,Olivia Boyer,Georges Deschenes,James Gleeson,Anne Couderc,Julien Hogan,Thomas Robert

    IgA nephropathy (IgAN) is one the most common primary glomerulonephritis in children and adolescents worldwide, with 20% of children developing end-stage kidney disease (ESKD) within 20 years of diagnosis. There is a need for treatment guidelines, especially for steroids in children with primary IgAN, since the STOP-IgA trial casts doubts on the use of steroids in adults with intermediate risk. Pediatricians are prone to prescribe steroids in addition to renin-angiotensin system blockade (RASB) when proteinuria is > 0.5 g/l, eGFR deteriorates < 70 ml/min/1.73 m2, or when a biopsy sample shows glomerular inflammation. Lack of randomized controlled trials (RCTs) in children with IgAN has led to an absence of consensus on the use of immunosuppressive agents in the treatment of progressive IgAN. This literature review evaluates the available evidence on steroid treatment in children with IgAN.

    更新日期:2020-01-17
  • Long-lasting chronic high load carriage of Epstein-Barr virus is more common in young pediatric renal transplant recipients
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-12-04
    Susanne Westphal Ladfors, Jenny K. Lindahl, Sverker Hansson, Per Brandström, Rune Andersson, Marianne Jertborn, Magnus Lindh, Susanne Woxenius, Vanda Friman

    Abstract Background Epstein-Barr virus (EBV) infections can induce post-transplant lymphoproliferative disorder (PTLD). A chronic high load (CHL), as indicated by long-term high EBV DNA levels after transplantation, has been associated with an enhanced risk of PTLD. We aimed to evaluate incidence, time of occurrence, risk factors, and outcome of EBV CHL carrier state after pediatric renal transplantation. Methods A retrospective study of 58 children aged 1–17 years (median 10), who underwent renal transplantation between January 2004 and June 2017 at a single medical center. EBV IgG antibodies in serum were analyzed before and yearly after transplantation. EBV DNA in whole blood were analyzed weekly for the first 3 months post-transplant, monthly up to 1 year and then at least once yearly. CHL was defined as EBV DNA ≥ 4.2 log10 Geq/ml in > 50% of the samples during ≥ 6 months. Results At transplantation, 31 (53%) patients lacked EBV IgG and 25 (81%) of them developed primary EBV infection post-transplant. Of the 27 seropositive patients, 20 (74%) experienced reactivation of EBV. Altogether, 14 (24%) children developed CHL, starting at a median of 69 days post-transplant and lasting for a median time of 2.3 years (range 0.5–6.5), despite reduction of immunosuppression. Patients with CHL were younger and 11/14 were EBV seronegative at transplantation. No child developed PTLD during median clinical follow-up of 7.8 years (range 0.7–13). Conclusions CHL was frequent, long lasting, and occurred mainly in young transplant recipients. The absence of PTLD suggests that monitoring of EBV DNA to guide immunosuppression was effective.

    更新日期:2020-01-17
  • A cross-sectional study on uric acid levels among Chinese adolescents
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-12-06
    Jie Lu, Wenyan Sun, Lingling Cui, Xinde Li, Yuwei He, Zhen Liu, Hailong Li, Lin Han, Aichang Ji, Can Wang, Hui Zhang, Xiaopeng Ji, Wei Ren, Xuefeng Wang, Changgui Li

    Abstract Background The prevalence of hyperuricemia is increasing in adults, while the prevalence among adolescents is seldom reported. Methods A cross-sectional survey by multistage, stratified sampling method was carried out in Shandong Province during 2017–2018. A total of 9371 adolescents aged from 13 to 19 years were randomly sampled and analyzed in this survey. Results The overall mean serum uric acid (sUA) concentration was 6.08 ± 1.57 mg/dL and overall hyperuricemia prevalence was 25.4% and 60.5% (when hyperuricemia was defined as sUA ≥ 7 mg/dL or ≥ 5.5 mg/dL). Prevalence were 42.3% (male) and 8.0% (female) when limit was 7 mg/dL and prevalence were 82.1% (male) and 38.4% (female) when limit was 5.5 mg/dL. Male gender, increased body mass index, increased waist circumstance, increased triglycerides, increased fasting blood glucose, increased systolic blood pressure, decreased estimated glomerular filtration rate, and positive family gout history were associated with the enhanced risk of hyperuricemia according to univariate and/or multivariate logistic regression analysis. Food intake frequency of carbonate beverage, mutton, and other kinds varied between hyperuricemia adolescents and normal sUA ones. Conclusions The studied adolescent population showed sUA level and hyperuricemia prevalence which are even higher than those of adults in China. The epidemic of youth hyperuricemia may pose a future threat of gout attacks and other hyperuricemia-related diseases, which alarms the public, health professionals and health policy makers to prepare the future health challenges.

    更新日期:2020-01-17
  • The pathogenesis and management of renal scarring in children with vesicoureteric reflux and pyelonephritis.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Vasikar Murugapoopathy,Christine McCusker,Indra R Gupta

    Bacterial urinary tract infections (UTIs) are one of the most common reasons for children to be admitted to hospital. Bacteria infect and invade the bladder (the lower urinary tract) and if the infection disseminates to the upper urinary tract, significant inflammation in the kidneys may arise. Inflammation is a double-edged sword: it is needed to clear bacteria, but if excessive, kidney tissue is injured. During injury, nephrons are destroyed and replaced with deposition of extracellular matrix and a renal scar. In this review, we explore the pathogenesis of UTIs and discuss the risk factors that result in dissemination of bladder infection to the kidneys. Three major risk factors predispose to kidney infections: the presence of vesicoureteric reflux, the presence of bladder and bowel dysfunction, and defects in the ability of the host immune response to clear bacteria. In this review, we will discuss these factors, their relationship to renal scarring, and potential treatments that might be beneficial to prevent renal scar formation in children.

    更新日期:2020-01-17
  • Sex and age as determinants for high blood pressure in pediatric renal transplant recipients: a longitudinal analysis of the CERTAIN Registry
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-12-07
    Rizky I. Sugianto, Bernhard M. W. Schmidt, Nima Memaran, Ali Duzova, Rezan Topaloglu, Tomas Seeman, Sabine König, Luca Dello Strologo, Luisa Murer, Zeynep Birsin Özçakar, Martin Bald, Mohan Shenoy, Anja Buescher, Peter F. Hoyer, Michael Pohl, Heiko Billing, Jun Oh, Hagen Staude, Martin Pohl, Gurkan Genc, Günter Klaus, Caner Alparslan, Ryszard Grenda, Jacek Rubik, Kai Krupka, Burkhard Tönshoff, Elke Wühl, Anette Melk

    High prevalence of arterial hypertension is known in pediatric renal transplant patients, but how blood pressure (BP) distribution and control differ between age groups and whether sex and age interact and potentially impact BP after transplantation have not been investigated.

    更新日期:2020-01-17
  • Energy and protein requirements for children with CKD stages 2-5 and on dialysis–clinical practice recommendations from the Pediatric Renal Nutrition Taskforce
    Pediatr. Nephrol. (IF 2.816) Pub Date : 2019-12-16
    Vanessa Shaw, Nonnie Polderman, José Renken-Terhaerdt, Fabio Paglialonga, Michiel Oosterveld, Jetta Tuokkola, Caroline Anderson, An Desloovere, Laurence Greenbaum, Dieter Haffner, Christina Nelms, Leila Qizalbash, Johan Vande Walle, Bradley Warady, Rukshana Shroff, Lesley Rees

    Abstract Dietary management in pediatric chronic kidney disease (CKD) is an area fraught with uncertainties and wide variations in practice. Even in tertiary pediatric nephrology centers, expert dietetic input is often lacking. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, was established to develop clinical practice recommendations (CPRs) to address these challenges and to serve as a resource for nutritional care. We present CPRs for energy and protein requirements for children with CKD stages 2–5 and those on dialysis (CKD2–5D). We address energy requirements in the context of poor growth, obesity, and different levels of physical activity, together with the additional protein needs to compensate for dialysate losses. We describe how to achieve the dietary prescription for energy and protein using breastmilk, formulas, food, and dietary supplements, which can be incorporated into everyday practice. Statements with a low grade of evidence, or based on opinion, must be considered and adapted for the individual patient by the treating physician and dietitian according to their clinical judgment. Research recommendations have been suggested. The CPRs will be regularly audited and updated by the PRNT.

    更新日期:2020-01-17
  • A single-center study to evaluate the efficacy of a fetal urine peptide signature predicting postnatal renal outcome in fetuses with posterior urethral valves.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Bénédicte Buffin-Meyer,Marcin Tkaczyk,Małgorzata Stańczyk,Benjamin Breuil,Justyna Siwy,Krzysztof Szaflik,Tomasz Talar,Justyna Wojtera,Waldemar Krzeszowski,Stéphane Decramer,Julie Klein,Joost P Schanstra

    BACKGROUND Posterior urethral valves (PUVs) account for 17% of pediatric renal failure. The management of pregnancies involving fetuses with PUV is hampered by the fact that current clinical parameters obtained from fetal ultrasound and/or fetal urine biochemistry are insufficient to predict postnatal renal function. We previously have developed a fetal urine peptide signature (12PUV) that predicted with high precision postnatal renal failure at 2 years of age in fetuses with PUV. Here, we evaluated the accuracy of this signature to predict postnatal renal outcome in fetuses with PUV in an independent single-center study. METHODS Thirty-three women carrying fetuses with suspected PUV were included. Twenty-five fetuses received vesicoamniotic shunts during pregnancy. PUV was confirmed postnatally in 23 patients. Of those 23 fetuses, 2 were lost in follow-up. Four and 3 patients died in the pre- and perinatal periods, respectively. Follow-up renal function at 6 months of age was obtained for the remaining 14 patients. The primary outcome was early renal failure, defined by an eGFR < 60 mL/min/1.73 m2 before 6 months of age or pre- or perinatal death. RESULTS The peptide signature predicted postnatal renal outcome in postnatally confirmed PUV fetuses with an AUC of 0.94 (95%CI 0.74-1.0) and an accuracy of 90% (95%CI 78-100). The signature predicted postnatal renal outcome for the suspected PUV cases with an AUC of 0.89 (95%CI 0.72-0.97) and an accuracy of 84% (95%CI 71-97). CONCLUSIONS This single-center study confirms the predictive power of the previously identified 12PUV fetal urinary peptide signature.

    更新日期:2020-01-17
  • Immunoglobulin serum levels in rituximab-treated patients with steroid-dependent nephrotic syndrome.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Cyrielle Parmentier,Jean-Daniel Delbet,Stéphane Decramer,Olivia Boyer,Julien Hogan,Tim Ulinski

    BACKGROUND Rituximab (RTX) is efficient in steroid-dependent nephrotic syndrome (SDNS) in pediatric and adult patients. The aim of this study is to describe hypogammaglobulinemia as a side effect of RTX treatment. METHODS All pediatric patients (< 18 years old) of four French pediatric nephrology centers who received RTX for SDNS between 2010 and 2015 have been included. Clinical and biological data have been analyzed retrospectively before, during, and after RTX treatment. Hypogammaglobulinemia was defined as an IgG level < - 2 standard deviations for patient age. RESULTS A total of 107 pediatric patients have been included, 65.9% were boys, median age at nephrotic syndrome diagnosis was 3.1 interquartile range [IQ 2.24-5.45] years and age at RTX introduction was 11.7 [IQ 8.6-14.2] years. Twenty-one patients had hypogammaglobulinemia before the initiation of RTX. Of the patients, 25/86 had at least one hypogammaglobulinemia during B cell depletion or after B cell recovery while IgG levels at initiation were normal with a persisting hypogammaglobulinemia for 13 patients 1 year after B cell recovery. Patients who developed hypogammaglobulinemia were younger at RTX initiation with a median age of 8.2 years [IQ 6.3-12.4]. Among all the 46 patients with hypogammaglobulinemia during follow-up, 13 had a concomitant infection. CONCLUSIONS Hypogammaglobulinemia is a frequent complication of RTX treatment in younger children treated for SDNS. The use of RTX in children has to be carefully evaluated and their clinical and biological follow-up should be adapted to the age-dependent risk profile for hypogammaglobulinemia.

    更新日期:2020-01-17
  • Growth plate alterations in chronic kidney disease.
    Pediatr. Nephrol. (IF 2.816) Pub Date : null
    Ángela Fernández-Iglesias,José Manuel López,Fernando Santos

    Growth retardation is a major feature of chronic kidney disease (CKD) of onset in infants or children and is associated with increased morbidity and mortality. Several factors have been shown to play a causal role in the growth impairment of CKD. All these factors interfere with growth by disturbing the normal physiology of the growth plate of long bones. To facilitate the understanding of the pathogenesis of growth impairment in CKD, this review discusses cellular and molecular alterations of the growth plate during uremia, including structural and dynamic changes of chondrocytes, alterations in their process of maturation and hypertrophy, and disturbances in the growth hormone signaling pathway.

    更新日期:2020-01-17
  • 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
Contents have been reproduced by permission of the publishers.
导出
全部期刊列表>>
2020新春特辑
限时免费阅读临床医学内容
ACS材料视界
科学报告最新纳米科学与技术研究
清华大学化学系段昊泓
自然科研论文编辑服务
加州大学洛杉矶分校
上海纽约大学William Glover
南开大学化学院周其林
课题组网站
X-MOL
北京大学分子工程苏南研究院
华东师范大学分子机器及功能材料
中山大学化学工程与技术学院
试剂库存
天合科研
down
wechat
bug