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BACKGROUND: Viral upper respiratory tract infections trigger nephrotic syndrome relapses. Few data exist on the impact of the SARS-CoV-2 pandemic on the risk of relapse in children with idiopathic nephrotic syndrome (INS). METHODS: In a Belgian and Italian cohort of children with INS, we performed a retrospective analysis on the number and duration of relapses observed in 3 different periods in 2020: first COVID period, February 15-May 31; second COVID period, June 1-September 14; third COVID period, September 15-December 31. Relapse rates were compared to those of the previous 5 years (PRECOVID period). For the years 2019 and 2020, all causes and INS relapse-related hospitalizations were recorded. Hospitalizations and deaths due to SARS-CoV-2 infection were also recorded. In the Belgian cohort, SARS-CoV-2 serologies were performed. RESULTS: A total of 218 patients were enrolled, and 29 (13.3%) were diagnosed with new-onset INS during the COVID period. Relapse rates per 1000 person-days were as follows: 3.2in the PRECOVID period, 2.7 in the first COVID period, 3.3 in the second COVID period, and 3.0 in the third COVID period. The incidence rate ratio for the total COVID period was 0.9 (95%CI 0.76 to 1.06; P = 0.21) as compared to the PRECOVID period. During 2020, both the proportion of patients hospitalized for recurrence (14.2% vs. 7.6% in 2019; P = 0.03) and the rate of hospitalization for recurrence (IRR 1.97 (95%CI 1.35 to 2.88); P = 0.013) were higher compared to 2019. In December 2020, anti-SARS-CoV-2 antibodies were detected in 31% of the Belgian cohort. Patients with positive and negative SARS-CoV-2 serology did not differ significantly in relapse rate (2.4 versus 4.2 per 1000 person-days). The number of new INS cases remained similar between 2020, 2019, and 2018. CONCLUSION: The first year of the SARS-CoV-2 pandemic did not significantly affect the relapse rate in children with INS. No serious infections were reported in this population of immunosuppressed patients. A higher resolution version of theGraphical abstract is available as Supplementary information.

The principles and use of plasmapheresis are often little understood by intensivists. We propose to review the principles, the main indications, and the methods of using this technique.

Potassium abnormalities are frequent in intensive care but their incidence in the emergency department is unknown.We describe the spectrum of potassium abnormalities in our tertiary-level pediatric emergency department.Retrospective case-control study of all the patients admitted to a single-center tertiary emergency department over a 2.5-year period. We compared patients with hypokalemia (3.0mEq/L) and patients with hyperkalemia (6.0mEq/L) against a normal randomized population recruited on a 3:1 ratio with potassium levels between 3.5 and 5mEq/L.Between January 1, 2013 and August 31, 2016 we admitted 108,209 patients to our emergency department. A total of 9342 blood samples were tested and the following potassium measurements were found: 60 cases of hypokalemia (2.8±0.2mEq/L) and 55 cases of hyperkalemia (6.4±0.6mEq/L). In total, 200 patients with normokalemia were recruited (4.1±0.3mEq/L). The main causes of the disorders were non-specific: lower respiratory tract infection (23%) and fracture (15%) for hypokalemia, lower respiratory tract (21.8%) and ear-nose-throat infections (20.0%) for hyperkalemia. Patients with hyperkalemia had an elevated creatinine level (0.72±1.6 vs. 0.40±0.16mg/dL, P0.0001) with lower bicarbonate (19.4±3.8 vs. 21.8±2.8mmol/L, P=0.0001) and higher phosphorus levels (1.95±0.6 vs. 1.42±0.27mg/dL, P=0.0001). Patients with hypokalemia had an elevated creatinine level (0.66±0.71 vs. 0.40±0.16mg/dL, P0.0001) and a lower phosphorus level (1.12±0.31 vs. 1.42±0.27mg/dL, P=0.0001). We did not observe significant differences in pH, PCODyskalemia is rare in emergency department patients: 0.64% for hypokalemia and 0.58% for hyperkalemia. This condition could be explained by a degree of renal failure due to transient volume disturbance. The main mechanism is dehydration due to digestive losses, polypnea in young patients, and poor intake. In the case of hypokalemia, poor intake and digestive losses could be the main explanation. These disorders resolve easily with feeding or perfusion and do not impair development.Dyskalemia is rare in emergency department patients and is easily resolved with feeding or perfusion. A plausible etiological mechanism is a transient volume disturbance. Dyskalemia is not predictive of poor development in the emergency pediatric population.

Rare autopsy studies have described smaller kidneys as well as urinary tract anomalies in Down syndrome. This observation has never been investigated in vivo and little is known about the possible consequences upon kidney function. Here we wish to confirm whether children with Down syndrome have smaller kidneys and to evaluate their kidney function in vivo.This retrospective cohort study enrolled 49 children with Down syndrome, as well as 49 age- and sex-matched controls at the Queen Fabiola Children's University Hospital in Brussels, Belgium. Doppler and kidney ultrasonography, spot urine albumin to creatinine ratio, estimated glomerular filtration rate (eGFR), and anthropometric data were recorded.Kidney size in children with Down syndrome was smaller than age- and sex-matched controls in terms of length (p 0.001) and volume (p 0.001). Kidney function based on eGFR was also decreased in Down syndrome compared to historical normal. Twenty-one of the children with Down syndrome (42%) had eGFR 90 mL/min/1.73 mChildren with Down syndrome have significantly smaller kidneys than age-matched controls as well as evidence of decreased kidney function. These findings, in addition to well-noted increased kidney and urologic anomalies, highlight the need for universal anatomical and functional assessment of all individuals with Down syndrome. A higher resolution version of the Graphical abstract is available as Supplementary information.

Background: Hemolytic uremic syndrome (HUS) is rare in neonates. It is probably an under-recognized condition in the early postnatal period as it presents similarly to the most common perinatal asphyxia and to differentiate the two conditions is challenging. We describe the clinical presentation of a potential new subtype of neonatal HUS triggered by hypoxic-ischemic event. Our patient was successfully treated by a single dose of Eculizumab as early as at 9 days of life. Case Report: A 35-weeks infant was born with low hemoglobin and subsequently developed respiratory distress, hypotension, and acidosis. Blood transfusion was administered, acidosis corrected, neurological examination remained reassuring. Few hours later he developed renal failure, macroscopic hematuria, hemobilia, thrombocytopenia and coagulopathy refractory to platelet and fresh frozen plasma transfusions. No infection was found. Haptoglobin was non-measurable, and schistocytes present, complement factors C3, C4 and B were low, FBb increased. HUS was suspected. A single dose of Eculizumab™ was administered on day 9 of life. No genetic mutation of atypical HUS was found. He was discharged with improving renal function and developing cholestasis. Conclusion: In neonates with hemolytic anemia, thrombocytopenia, hematuria and renal failure, HUS should be suspected. In neonatal HUS Eculizumab should be considered as first-line therapy and discontinuation can be considered if no genetic mutation is found and clinical condition improves. In very young patients, cholestasis could appear as potential side effect of Eculizumab™., SCOPUS: ar.j, info:eu-repo/semantics/published

Severe acute respiratory syndrome coronavirus 2, the virus responsible of the current COVID-19 pandemic, has limited impact in the pediatric population. Children are often asymptomatic or present mild flu-like symptoms. We report the case of a COVID-19-infected adolescent presenting severe rhabdomyolysis and acute kidney injury without any fever or respiratory symptoms.

Kidney transplantation (KT) is often delayed in small children because of fear of postoperative complications. We report early- and long-term outcomes in children transplanted at ≤15 kg in the two largest Belgian pediatric transplant centers. Outcomes before (period 1) and since the introduction of basiliximab and mycophenolate-mofetil in 2000 (period 2) were compared. Seventy-two KTs were realized between 1978 and 2016: 38 in period 1 and 34 in period 2. Organs came from deceased donors in 48 (67%) cases. Surgical complications occurred in 25 KTs (35%) with no significant difference between the two periods. At least one acute rejection (AR) occurred in 24 (33%) KTs with significantly less patients experiencing AR during period 2: 53% and 12% in period 1 and, period 2 respectively (P < 0.001). Graft survival free of AR improved significantly in period 2 compared with period 1: 97% vs. 50% at 1 year; 87% vs. 50% at 10 years post-KT (P = 0.003). Graft survival tended to increase over time (period 1: 74% and 63% at 1 and 5 years; period 2: 94% and 86% at 1 and 5 years; P = 0.07), as well as patient survival. Kidney transplantation in children ≤15 kg remains a challenging procedure with 35% of surgical complications. However, outcomes improved and are nowadays excellent in terms of prevention of AR, patient and graft survival. ispartof: TRANSPLANT INTERNATIONAL vol:31 issue:7 pages:720-728 ispartof: location:Switzerland status: published

Resume Le syndrome nephrogenique d’antidiurese inappropriee (SNAI) est une affection rare caracterisee par une incapacite renale a diluer les urines, entrainant une hyponatremie severe et persistante. En raison de son expression clinique variable, son diagnostic reste un defi pour les cliniciens. Nous rapportons un cas dont le diagnostic precoce a permis d’eviter les complications liees a une hyponatremie severe. Il s’agissait d’un garcon de 1 mois adresse pour une hyponatremie persistante et des vomissements. Il etait ne a terme apres une grossesse sans particularite avec un poids de naissance normal et evolution ponderale normale. L’examen clinique a l’admission etait normal. Sur le plan biologique, il existait une hyponatremie (125 mmol/L) et une hemodilution. La natriurese etait a 59 mmol/L. Il a ete initialement conclu a un syndrome de secretion inappropriee en hormone antidiuretique (SIADH) et un traitement oral par uree a ete instaure. Les analyses sanguines approfondies ont cependant revele l’absence d’arginine-vasopressine (AVP) dans le sang, suggerant un SNAI. Ceci ete confirme par la mise en evidence de la mutation R137C du gene codant le recepteur de type V2 de l’AVP (AVPR2). Dans ce cas de SNAI, le diagnostic et la prise en charge precoce ont permis d’eviter les complications neurologiques associees a une hyponatremie severe. Ce diagnostic doit etre evoque devant toute hyponatremie persistante associee a une hemodilution en l’absence d’AVP. Il est confirme par le sequencage du gene AVPR2.

Resume Objectifs Decrire les realites liees au diagnostic et au traitement de l’infection urinaire (IU) chez les enfants febriles vietnamiens, ainsi que les caracteristiques cliniques et la frequence des germes uropathogenes et leur taux de resistance. Methodes Etude prospective de 28 mois realisee chez les enfants febriles traites pour IU documentee dans le departement de nephrologie de l’hopital pediatrique Nhi-Dong 2 a Ho-Chi-Minh-Ville (Vietnam). Apres l’obtention de prelevements sanguins et urinaires, les enfants ont ete traites par la ceftriaxone intraveineuse (75 mg/kg/j). Apres 48 heures d’apyrexie, le relais a ete pris par cefixime (8 mg/kg/j) par voie orale pour une duree de traitement totale de 14 jours. L’adaptation du traitement n’etait envisagee que chez les enfants ne repondant pas cliniquement, sans tenir compte de l’antibiogramme. Resultats Cent-quarante-trois enfants ont ete traites (51 % de filles) dont 80 % avaient eu leur premier episode d’IU avant l’âge de 2 ans. Escherichia coli etait responsable de 80 % des IU, avec un taux de resistance de 91 % a l’ampicilline et de 74 % au cotrimoxazole. Des enterobacteries productrices de β-lactamases a spectre etendu (BLSE) ont ete isolees dans 52 % des cultures. Selon l’antibiogramme, le traitement initial par ceftriaxone etait inapproprie dans 63 % des cas. Conclusions E. coli est responsable de la majorite des IU chez l’enfant vietnamien avec un taux eleve de resistance aux antibiotiques de premiere ligne. La production de BLSE est extremement elevee. Nous proposons une prise en charge plus rationnelle des enfants febriles avec IU ainsi qu’un traitement antibiotique empirique tenant compte de l’emergence des germes multiresistants.

Background: We present a rare early presentation of a ADCK4-related glomerulopathy. This case is of interest as potentially treatable if genetic results are timely obtained. Case presentation: We report the case of a 5-year-old boy who was identified with significant proteinuria by a urinary routine screening program for school children. Physical examination revealed dysplastic ears and abnormal folded pinna. Albumin level was 41 g/L (39-53 g/L), and urine proteins/creatinine ratio was 2.6 g/g. Renal ultrasound showed enlarged kidneys and perimedullary hyperechogenicity. Treatment by angiotensin-converting-enzyme inhibitor was not beneficial. Renal biopsy showed signs of focal segmental glomerulosclerosis. After 4 years of follow-up, he developed a clinical nephrotic syndrome and no response to prednisone and other immunosuppressive agents was obtained. Within 6 months, he was in end-stage-renal-failure (ESRF) and hemodialysis was started. He was transplanted at 10 years with his mother's kidney. Genes known to be responsible in steroid-resistant nephrotic syndromes were tested. Our patient is compound heterozygous for two mutations in the aarF domain-containing-kinase 4 (ADCK4) gene. ADCK4 gene is one of the genes involved in coenzyme Q10 (CoQ10) biosynthesis, is located in chromosome 19q13.2 and expressed in podocytes. ADCK4 mutations show a largely renal-limited phenotype. The nephropathy usually presents during adolescence, fast evolves towards ESRF, and may be treatable by CoQ10 supplementation if started early in the disease. Our patient presented nephrotic range proteinuria at 5 years, and he reached ESRF at 10 years. Conclusion: ADCK4-related glomerulopathy is an important novel and potentially treatable cause of isolated nephropathy not only in adolescents, but also in children in their first decade of life. Discovery of important proteinuria in an asymptomatic child should prompt early genetic investigations., SCOPUS: ar.j, info:eu-repo/semantics/published

The main criteria used for deciding on surgery in children with presumed antenatally detected pelviureteric junction obstruction (PPUJO) are the level of hydronephrosis (ultrasonography), the level of differential renal function (DRF) and the quality of renal drainage after a furosemide challenge (renography), the importance of each factor being far from generally agreed. Can we predict, on the basis of ultrasound parameters, the patient in whom radionuclide renography can be avoided?We retrospectively analysed the medical charts of 81 consecutive children with presumed unilateral PPUJO detected antenatally. Ultrasound and renographic studies performed at the same time were compared. Anteroposterior pelvic diameter (APD) and calyceal size were both divided into three levels of dilatation. Parenchymal thickness was considered either normal or significantly decreased. Acquisition of renograms under furosemide stimulation provided quantification of DRF, quality of renal drainage and cortical transit.The percentages of patients with low DRF and poor drainage were significantly higher among those with major hydronephrosis, severe calyceal dilatation or parenchymal thinning. Moreover, impaired cortical transit, which is a major risk factor for functional decline, was seen more frequently among those with very severe calyceal dilatation. However, none of the structural parameters obtained by ultrasound examination was able to predict whether the level of renal function or the quality of drainage was normal or abnormal. Alternatively, an APD30 mm, a calyceal dilatation of10 mm and a normal parenchymal thickness were associated with a low probability of decreased renal function or poor renal drainage.In the management strategy of patients with prenatally detected PPUJO, nuclear medicine examinations may be postponed in those with an APD30 mm, a calyceal dilatation of10 mm and a normal parenchymal thickness. On the contrary, precise estimation of DRF and renal cortical transit should be performed in patients with APD30 mm, major calyceal dilatation and/or parenchymal thinning.

This review includes an analysis of new developments in the field of renography, the predictive factors suggesting the need for pyeloplasty in cases of pelvi-utereric stenosis detected antenatally and integration of the pelvi-ureteric junction stenosis within the framework of antenatally detected hydronephrosis.

Automated flow cytometry of urine remains an incompletely validated method to rule out urinary tract infection (UTI) in children. This cross-sectional analytical study was performed to compare the predictive values of flow cytometry and a dipstick test as initial diagnostic tests for UTI in febrile children and prospectively included 1,106 children (1,247 episodes). Urine culture was used as the gold standard test for diagnosing UTI. The performance of screening tests to diagnose UTI were established using receiver operating characteristic (ROC) analysis. Among these 1,247 febrile episodes, 221 UTIs were diagnosed (17.7% [95% confidence interval {CI}, 15.6 to 19.8%]). The area under the ROC curve for flow cytometry white blood cell (WBC) counts (0.99 [95% CI, 0.98 to 0.99]) was significantly superior to that for red blood cell (0.74 [95% CI, 0.70 to 0.78]) and bacterial counts (0.89 [95% CI, 0.87 to 0.92]) ( P < 0.001). Urinary WBC counts also had a significantly higher area under the ROC curve than that of the leukocyte esterase (LE) dipstick (0.92 [95% CI, 0.90 to 0.94]), nitrite dipstick (0.83 [95% CI, 0.80 to 0.87]), or the combination of positive LE and/or nitrite dipstick (0.91 [95% CI, 0.89 to 0.93]) test ( P < 0.001). The presence of ≥35 WBC/μl of urine was the best cutoff point, yielding both a high sensitivity (99.5% [95% CI, 99 to 100%]) and an acceptable specificity (80.6% [95% CI, 78 to 83%]). Using this cutoff point would have reduced the number of samples sent to the laboratory for culture by 67%. In conclusion, the determination of urinary WBC counts by flow cytometry provides optimal performance as an initial diagnostic test for UTI in febrile children.

The issue of antenatal ultrasound examination has become a routine component for the care of a pregnant woman. Congenital anomalies of the kidney and urinary tract (CAKUT) are among the most frequent organ malformations and constitute the most common cause of chronic renal failure in children. In this chapter we introduce the tools of assessment, describe the spectrum of fetal phenotypes and discuss the various approaches taken in the clinical management of prenatally diagnosed disorders of the kidneys and urinary tract. Based on this body of information, we outline rational antenatal and postnatal investigation strategies.

To track the clinical evolution of febrile urinary tract infection (UTI) diagnosed in 0- to 3-month-old infants and characterize uropathogen frequencies, antimicrobial resistance rates, renal abnormalities, and differences in the sexes in this age group.We observed prospectively 46 infants identified in a cohort of 209 children with first UTI diagnosed between July 2006 and July 2008 at the age of 0 to 3 months. Renal ultrasound scanning and voiding cystourethrography examinations were performed in all infants.Infants3 months old represented 21% of all children with first UTI. Of these children, 26% were female and 74% were male. Escherichia coli was isolated in 88% of cases and had a high rate of resistance to ampicillin (71%) and to trimethoprim/sulfamethoxazole (47%); 21% of children had vesicoureteral reflux, which was of low-grade in 67% of cases, with spontaneous resolution before 2 years in all cases. In infants with normal ultrasound scanning results, a low-grade vesicoureteral reflux was subsequently found in 10% of cases.Infants aged 0 to 3 months represent 21% of children treated for febrile UTI. Boys represent 74% of these cases. E coli is responsible for 88% of UTIs, with a high rate of resistance to antibiotics. When ultrasound scanning examination results are normal, the risk of missing a significant renal abnormality is expected to be extremely low.

The clinical classification of nephrotic syndrome (NS) is based on age at presentation. However, this classification is arbitrary because the majority of early onset NS has a genetic origin and has a widespread age of onset (from fetal life to several years). The aims of this review are to illustrate the knowledge accumulated on congenital nephrotic syndrome (CNS) in terms of genetics, classification, findings at histology and US-based on a review of the literature.

Vesicoureteric reflux (VUR) is a common finding in pediatric practice that occurs in about 1% of children and is often familial, with several genetical loci probably involved. The majority of low-grade cases have a tendency to resolve spontaneously during childhood. However, VUR has been identified as a risk factor for the development of urinary tract infections (UTI) and is present in a third of young children presenting with this problem. In addition, some children with high-grade VUR have already renal lesions before the advent of any UTI. In a subset of affected individuals, these renal parenchymal lesions are associated with systemic hypertension and chronic renal failure but this risk is considerably smaller than previously assumed. In randomized trials of primary VUR diagnosed after urinary infection, prophylactic antibiotics are equivalent to anti-reflux surgery when subsequent renal lesions and UTIs relapses are used as endpoints. Controlled trials comparing the outcome of renal function in children offered either protocol or immediate treatment at recurrences are desperately lacking. This review article aims to summarize the extensive data regarding this controversial condition, to present rational investigation strategy and to analyze management attitudes in the light of the various lines of practice.

Functional compensation occurs in a kidney when the function of the contralateral kidney is decreased or absent. What is, however, not documented is the level of unilateral renal impairment at which functional compensation occurs. Split function, as obtained from a radionuclide renogram, can only show the asymmetry between both kidneys. The aim of the present work was to evaluate at what level of unilateral impairment a functional compensation can be observed in the contralateral normal kidney. From a large database, 180 children over 2 years of age with unilateral pathology were retrospectively selected. All of them underwent a radionuclide study, combining a technetium-99m mercaptoacetyltriglycine (Tc-99m MAG3) renogram associated with a chromium-51 ethylenediaminetetraacetic acid (Cr-51 EDTA) overall clearance, allowing a precise estimation of single-kidney glomerular filtration rate (SKGFR). Below 30-35 ml/min/1.73 m2, there was a significant inverse correlation between SKGFR on the normal and the abnormal side. Above this level, no such correlation was observed, the mean SKGFR on the normal side remaining around 58 ml/min/1.73 m2, whatever the value of SKGFR on the abnormal side. In pediatric patients, hyperfunction occurs when SKGFR of the diseased kidney is below 30-35 ml/min/1.73 m2.

Fetal renal pelvis dilatation is a frequent abnormality that has been observed in 4.5% of pregnancies. The majority of these cases have a tendency to resolve during infancy. Nevertheless, fetal renal pelvis dilatation may be due to significant structural abnormalities such as pelvi-ureteric junction stenosis or vesico-ureteral reflux that may adversely affect renal function or cause urinary infection or sepsis. This review article aims to summarize the data regarding fetal renal pelvis dilatation and to analyze controversial attitudes in the light of the various lines of practice, and to present rational antenatal and postnatal investigation strategy.

Because loss of functional renal mass is compensated by hyperfiltration of remaining tissue, one could hypothesize that a damaged kidney might not have the same rate of maturation as the contralateral one. To verify this, maturation was evaluated in children with asymmetrical renal function during early life. Twenty-five children were selected having had 2 (99m)Tc-MAG3 renograms combined with (51)Cr-EDTA clearance measurement, enabling estimation of glomerular filtration rate (GFR), split renal function (SRF), and single kidney GFR (SKGFR). The first test had to be performed before the age of 18 months and SRF on the affected side had to beor =40%. Moreover, GFR had to increase between the 2 tests byor =10 mL(-1) min/1.73 m(2), reflecting maturation due to age. For 18 children SRF changed by between -5% and +5%. For 4 children an increase ofor =5% was observed whereas for the remaining 3 a decrease ofor =5% occurred. For the first 22 kidneys, mean increase of SKGFR was +6.3 mL(-1) min/1.73 m(2)(SD: 6). For the 3 kidneys withor =5% SRF decrease, SKGFR remained unchanged in 2 (+0.3 and -3 mL min(-1)/1.73 m(2)) and increased in the third patient (+15 mL min(-1)/1.73 m(2)). In conclusion, renal maturation is comparable in both the malfunctioning kidney and the contralateral normal functioning side.

Objectives To determine if two successive ultrasound examinations could rule out the presence of clinically significant contralateral anomalies in neonates with multicystic dysplastic kidney (MCDK), thereby avoiding unnecessary voiding cystourethrography (VCUG). Study design We followed 76 newborn infants with antenatally discovered MCDK. Two successive neonatal renal ultrasound examinations were performed, one within the first week and one at around 1 month of life. VCUG and isotopic studies were performed in all infants. Results Urologic anomalies of the contralateral kidney were present in 19 of 76 children (25%): vesicoureteral reflux (VUR) in 16 (21%), ureteropelvic junction obstruction in 2 (3%), and renal duplex kidney in 1 (1%). Sixty-one infants (80% of total) had normal contralateral urinary tract on the 2 successive neonatal renal ultrasound scans. Among them, 4 of 61 (7%) infants presented with low-grade VUR on VCUG that had resolved spontaneously before 2 years of age. The sensitivity, specificity, positive predictive value, and negative predictive value of two successive ultrasound scans in the neonatal period to predict contralateral urological anomalies on VCUG were 75%, 95%, 80%, and 93%, respectively. Conclusions In infants with antenatally diagnosed MCDK, two successive normal neonatal renal ultrasound scans will rule out clinically significant contralateral anomalies, thereby rendering the need for a neonatal VCUG unnecessary.

OBJECTIVE: The purpose of this study was to determine the incidence of minor degrees of renal pelvis dilatation that-is detected by antenatal ultrasound scanning in an unselected population and its value in the prediction of significant uropathies. STUDY DESIGN: This prospective study was conducted over a 24-month period. Infants with an anteroposterior pelvic diameter of greater than or equal to4 mm in the second trimester and/or greater than or equal to7 mm but

Atypical hemolytic uremic syndrome (aHUS) is a life-threatening multisystemic condition often leading to end-stage renal failure. It results from an increased activation of the alternative pathway of the complement system due to mutations of genes coding for inhibitors of this pathway or from autoantibodies directed against them. Eculizumab is a monoclonal antibody directed against complement component C5 and inhibiting the activation of the effector limb of the complement system. Its efficacy has already been demonstrated in aHUS. The present article reports for the first time the use of eculizumab in a patient presenting with aHUS associated with circulating anti-complement Factor H autoantibodies and complicated by cardiac and neurologic symptoms. Our observation highlights the efficacy of eculizumab in this form of aHUS not only on renal symptoms but also on the extrarenal symptoms. It also suggests that eculizumab should be used very promptly after aHUS presentation to prevent life-threatening complications and to reduce the risk of chronic disabilities. To obtain a complete inhibition of the effector limb activation, the advised dosage must be respected. After this initial therapy in the autoimmune aHUS form, a long-term immunosuppressive treatment should be considered, to prevent relapses by reducing anti-complement Factor H autoantibody plasma levels. Copyright © 2014 by the American Academy of Pediatrics.

Renal dysplasia (RD) is a common cause of chronic renal failure (CRF) in children. The evolution towards end-stage renal failure is unpredictable due to the paucity of early prognostic factors. In order to identify early prognostic clinical criteria, we have retrospectively analyzed renal function and growth in 11 infants with RD and CRF from birth up to 4 years of age. Children with obstructive RD were not included. Glomerular filtration rate (GFR) was estimated from Schwartz formula. In infants with a GFR below 15 ml/min per 1.73 m2 at 6 months of age (group A, n=5), kidney function did not further improve; 4 reached end-stage renal failure between 8 months and 6 years of age. In contrast, infants with a GFR above 15 ml/min per 1.73 m2 at 6 months of age (group B, n=6) experienced a significant improvement in renal function during follow-up, and none required renal replacement therapy. During the first 3 months of life all infants with RD and CRF developed severe growth retardation. Between 6 months and 4 years of age, children from group B grew significantly better than those from group A. In conclusion, our experience suggests that GFR, estimated from Schwartz formula at 6 months of age, is a useful prognostic factor in infants with RD and CRF. Infants with a GFR below 15 ml/min per 1.73 m2 are at risk of severe growth delay and the need for early renal replacement therapy, whereas those with a GFR above 15 ml/min per 1.73 m2 have a relatively favorable long-term prognosis.

Denys-Drash syndrome and Frasier syndrome are two related conditions caused by mutations of the Wilms tumor gene, WT1. Both syndromes are characterized by male pseudohermaphrodism, a progressive glomerulopathy, and the development of genitourinary tumors. This study examines three girls with steroid-resistant nephrotic syndrome related to mutations in the WT1 gene, but with normal 46, XX karyotype and normal female phenotype. WT1 mutation analysis should be routinely done in females with steroid-resistant nephrotic syndrome.

Objective To determine, in children with antenatally detected pelviureteric junction (PUJ) stenosis, what factors may be predictive for deterioration of differential renal function (DRF) in case of conservative treatment or improvement of DRF in case of pyeloplasty. Methods This study analyzed and compared the initial level of hydronephrosis, DRF, quality of renal drainage, and cortical transit with the late DRF outcome. We reviewed the medical charts of 161 consecutive children with antenatally diagnosed PUJ stenosis during a 10-year period (between 1997 and 2007). From this cohort, we retained 81 children with unilateral PUJ and strictly normal contralateral kidney, with a median follow-up of 67 months. Repeated ultrasounds, voiding cystourethrography, and radionuclide renograms were performed in all children. Results Fifty patients never underwent a surgical intervention (62%), whereas surgical repair (Anderson-Hynes dismembered pyeloplasty) was performed in 31 (38%). During conservative follow-up, DRF deterioration was observed in 11% of patients. After pyeloplasty, DRF improvement was observed in 25% of patients. Abnormal cortical transit was the only predictive factor of DRF deterioration in case of conservative approach, whereas the initial degree of hydronephrosis, or renal drainage, and the initial DRF level were not predictive. In children who were operated on, only impaired cortical transit was predictive of DRF improvement postoperatively. Conclusion Conservative management of children with unilateral PUJ stenosis is a safe procedure. Impaired cortical transit although imperfect, seems the best criterion for identifying children for whom pyeloplasty is warranted.

BACKGROUND: Our objective is to provide the clinical characteristics, uropathogen frequencies, and antimicrobial resistance rates of first urinary tract infection (UTI) diagnosed in febrile Belgian children. The ability of noninvasive ultrasound to detect renal abnormalities and vesicoureteral reflux (VUR) in these patients was also assessed. METHODS: We prospectively followed (median, 20 months) 209 children treated for first febrile UTI. Renal ultrasound (US) and voiding cystourethrography examinations were performed in all patients. RESULTS: Among these children, 63% were females and 37% were males, and 75% of them had their first UTI before the age of 2 years. The most common causative agent was Escherichia coli (91% of cases) with high rate resistance to ampicillin (58%) and trimethoprim/sulfamethoxazole (38%). Of these children, 25% had evidence of VUR (15 boys and 38 girls). VUR was of low grade in 85% of cases. The overall performance of renal US as a diagnostic test to detect significant uropathies excluding low-grade VUR was excellent; the sensitivity attained 97% and the specificity 94%. CONCLUSION: Girls represent 63% of cases with first UTI. For 91% of UTIs, Escherichia coli is held responsible with a high rate of resistance to ampicillin and trimethoprim/sulfamethoxazole. US is an excellent screening tool that allows avoidance of unjustified voiding cystourethrography studies. Copyright © 2011 by Lippincott Williams &Wilkins.

Until recently, renogram, performed in children with pelvi-ureteric junction stenosis detected antenatally, has not been able to predict the probability of function improvement after surgery or the risk of function deterioration in case of conservative attitude. Recently, Schlotmann et al. have suggested that cortical transit might have this predictive role. The aim of this study, focused on those kidneys with severely impaired cortical transit, was to verify this statement.All renograms performed in children during a 3-year period (n=729) were retrospectively reviewed and 19 pediatric patients were selected based on an antenatally detected unilateral pelvi-ureteric junction syndrome, the existence of at least two renograms during the follow-up and a severe cortical transit impairment, visually defined. Twenty-six pairs of data could be analyzed and allowed comparing preoperative and postoperative differential renal function (DRF), and the DRF changes during the conservative management.Among the 16 patients who underwent pyeloplasty, 10 showed a significant DRF improvement. Among the 10 patients with conservative follow-up, four showed a significant DRF deterioration.Severely impaired cortical transit seems to be a valuable marker of those patients who could benefit from a pyeloplasty, either because of the high probability of postoperative DRF improvement, or because of DRF deterioration in case of an conservative approach. However, a normal cortical transit, as defined in this study, does not exclude the risk of DRF deterioration. Alternatively, the design of this study does not allow excluding the fact that DRF might improve after pyeloplasty despite a normal cortical transit.

The aim of this study was to report on the clinical characteristics and outcomes of Belgian children with chronic kidney disease (CKD). Between 2001 and 2005, we followed 143 new successive patients younger than 20 years of age with a glomerular filtration rate of

The aim of the study was to present our experience in treating children with genetic forms of nephrotic syndrome and diagnosing these diseases. We retrospectively reviewed the clinical data, mutational analyses, histopathological features, treatment modalities, and outcome of 26 consecutive children (20 families) suffering from congenital and/or steroid-resistant nephrotic syndrome who were assessed by genetic analysis. Ten out of 26 children (38%) had congenital nephrotic syndrome, 4/26 (15%) had infantile nephrotic syndrome, 10/26 (38%) had late-onset nephrotic syndrome, and 2/26 (9%) had asymptomatic proteinuria. We detected a mutation in 21/26 (81%) patients and in 15/20 (75%) families. NPHS1 mutation analyses were positive in 4/20 (20%), NPHS2 mutations in 4/20 (20%), WT1 mutations in 4/20 (20%), and PLCE1 mutations in 3/20 (15%) families. NPHS1 and PLCE1 mutations were solely found in patients with the earliest onset. The majority of patients, especially those with early onset of nephrotic syndrome, had serious adverse events related to the nephrotic status, and 19/26 (73%) reached end-stage renal failure at a median age of 27 months. Genetic forms of nephrotic syndrome comprise a heterogeneous group of genetic mutations. The progression toward end-stage renal failure is the rule but is highly variable between patients.

The aim of this study was to investigate the etiology and treatment modalities and to determine mortality risks in hospitalized children with chronic renal failure (CRF) in Ho Chi Minh City, Vietnam. We reviewed the records of 310 children with CRF hospitalized in Ho Chi Minh City from January 2001 to December 2005. The average annual number cases was 4.8 per million child population native to Ho Chi Minh City. Median age was 14 years; 85% of patients were in end-stage renal failure. Associated illnesses were anemia (96%), hypertension (74%), and cardiopulmonary diseases (39%). Causes of included glomerulonephritis (30%) and congenital/hereditary anomalies (20%), but in 50% of children, the etiology was unavailable. Seventy-three percent of cases with end-stage renal failure did not benefit from renal replacement therapy. During hospitalization, 47 patients (15%) died. Mortality risks were higher in young children (1-4 years), in boys, and in patients with acquired pathologies. Severe metabolic acidosis was the main predictive factor of mortality by multivariate regression analysis. Our data shows a poor outcome due to late referral and limited facilities for renal replacement therapy in children with CRF hospitalized in Ho Chi Minh City.

The authors wish to highlight the evolution that has occurred in fetal ultrasound in recent years. A first significant evolution lies in the increasing contribution of first trimester ultrasound for the detection of fetal anomalies. Malformations of several organs and systems have been diagnosed during the first trimester. Furthermore the systematic measurement of the fetal neck translucency has led to increasing rate of detection of aneuploidies and heart malformations. For several years now, three-dimensional (3D) and 4D ultrasound (US) have been used as a complementary tool to 2D US for the evaluation of fetal morphology. This brings an improved morphologic assessment of the fetus. Applications of the techniques are increasing, especially for the fetal face, heart and extremities. The third field where fetal US is continuously providing important information is the knowledge of the natural history of diseases. This has brought significant improvement in the postnatal management of several diseases, especially urinary tract dilatation and broncho-pulmonary malformation.

OBJECTIVE: To assess the clinical outcome and imaging features of neonatal primary vesicoureteral reflux (VUR). STUDY DESIGN: We prospectively followed 43 infants with primary VUR identified from among a cohort of 497 infants with fetal renal pelvis dilatation. Postnatal renal ultrasound (US) examinations were performed at 5 days and 1, 3, 6, 12, and 24 months of life. Voiding cystourethrography was performed in the neonatal period and repeated at 12 and 24 months when VUR was persistent. Two radioisotopic examinations, including a 99mTc-MAG3 renogram and a plasma clearance of Cr-51 EDTA, were performed in all children with high-grade reflux. RESULTS: The incidence of primary VUR in our study group was 9%. Among the 43 patients followed, 11 (26%) had high-grade (IV-V) VUR and 32 (74%) had low-grade VUR. Resolution of reflux occurred in 2 of 11 (18%) patients with high-grade VUR and in 29 of 32 (90.6%) patients with low-grade VUR at age 2 years (P < .001). At age 2 years, 91% of the low-grade refluxing kidneys were normal on US, compared with only 35% of the high-grade refluxing kidneys. Split renal function was within normal range and single-kidney GFR was significantly increased in 13 of the 17 high-grade refluxing kidneys during follow-up. Furthermore, a strong association between dysplasia on US and poor renal function outcome was found. CONCLUSIONS: In most infants with VUR, the reflux is of low grade and resolves rapidly. In those children with high-grade VUR, spontaneous resolution is rare at age 2 years, but persistent reflux rarely impairs the maturation of renal function.

The existence of supranormal differential renal function in unilateral hydronephrosis remains controversial. While some authors consider it as fact, others believe that it is just a technical artifact. Within our department, chromium-51 ethylene diamine tetra-acetic acid (Cr-EDTA) renal clearance is systematically performed in conjunction with technetium-99m mercaptoacetyltriglycine (MAG3) renograms to derive an absolute single kidney glomerular filtration rate (SKGFR). Our data allows us to ascertain whether supranormal differential renal function in unilateral hydronephrosis might be due to hypofunction of the contralateral kidney. Children with marked unilateral hydronephrosis were selected from a large database of MAG3 diuretic(s) renograms. We excluded patients with posterior urethral valves, duplex anomalies, neurogenic bladder, solitary kidney, and those who underwent any previous urological surgery. We also excluded children who had an early furosemide injection (F0 procedure), selecting only those having received furosemide at the end of the renogram (F+20 test). Seventy-three patients (92 renograms) fulfilled these criteria. Differential renal function was calculated using the integral method. Hydronephrotic kidney with a relative uptake > or =55% was defined as supranormal. Six renograms (four patients) demonstrated supranormal relative function on the hydronephrotic side. However, the SKGFR of these kidneys was in all cases within the range of normal values, while the contralateral side demonstrated borderline low SKGFR. Increased relative function on the side of the hydronephrotic kidney is relatively infrequent. When it occurs, it may be related to a borderline hypofunction of the contralateral kidney.

Summary Objective: A drop of split renal function often constitutes a criterion for pyeloplasty in hydronephrosis since it is considered as representing deterioration of the affected kidney. The aim of this work was to determine, in a selected population of patients with a drop of split function of at least 5%, if the evolution of single kidney glomerular filtration rate (SKGFR) was parallel to the one of split renal function. Methods: From a large data basis, we found retrospectively only 29 children (10 below and 19 above two years of age at first examination) having had at least two Tc-99m mertiatide (Tc-99m MAG3) renographic explorations for various urological diseases, with a decrease of split function of at least 5% between the two examinations. Evolution of split function was compared to evolution of SKGFR obtained by means of the combination of Tc-99m MAG3 split function and overall glomerular filtration rate as given by the chromium Cr 51 ethylenediamine tetraacetic acid (EDTA) clearance. Results: For the group above two years of age, SKGFR increased or remained stable in 63% of the cases, while in the children less than 2 years of age, a decrease of SKGFR was never observed, according to the maturation of overall GFR in this age group. Thus, the decrease of split function was not necessarily associated with a similar decrease of SKGFR. Conclusion: In patients with unilateral or bilateral urological disorders, deterioration of split renal function does not necessary correspond to a loss of function of the affected kidney. SKGFR often modifies the interpretation of split function.

Objectives: To assess the yield of neonatal renal ultrasonography as a predictor of abnormalities on voiding cystourethrography (VCUG). Study design: We prospectively followed 264 infants with antenatal renal pelvis dilation. Two successive neonatal renal ultrasound examinations were performed at day 5 and 1 month after birth. VCUG was performed in all infants. Results: Neonatal ultrasound findings were abnormal in 190 infants (72% of total). Among them, 63 (33%) had an abnormal VCUG. The most common abnormalities were primary vesico-ureteral reflux (VUR) in 30 infants, VUR into the lower pole of a duplex kidney in 14, refluxing megaureter in 9, posterior urethral valves in 5, and ureterocele in 5. When both neonatal ultrasound findings were normal (74 infants), the VCUG showed abnormalities in only 5 (6.7%) patients (4 low-grade primary VUR and 1 posterior urethral valve). The sensitivity, specificity, positive predictive value, and negative predictive value of 2 successive ultrasound scans in the neonatal period to predict an abnormality on VCUG were 93%, 35%, 33%, and 93%, respectively. Conclusions: A normal-appearing urinary tract on 2 successive neonatal ultrasound scans rarely coexists with abnormal findings at VCUG. Therefore, in such patients, VCUG does not seem justified. (J Pediatr 2002;141:21-4)