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Öğe ADCK4-Associated Glomerulopathy Causes Adolescence-Onset FSGS(Amer Soc Nephrology, 2016) Korkmaz, Emine; Lipska-Zietkiewicz, Beata S.; Boyer, Olivia; Gribouval, Olivier; Fourrage, Cecile; Tabatabaei, Mansoureh; Schnaidt, Sven; Gucer, Safak; Kaymaz, Figen; Arici, Mustafa; Dinckan, Ayhan; Mir, Sevgi; Bayazit, Aysun K.; Emre, Sevinc; Balat, Ayse; Rees, Lesley; Shroff, Rukshana; Bergmann, Carsten; Mourani, Chebl; Antignac, Corinne; Ozaltin, Fatih; Schaefer, FranzHereditary defects of coenzyme Q(10) biosynthesis cause steroid-resistant nephrotic syndrome (SRNS) as part of multiorgan involvement but may also contribute to isolated SRNS. Here, we report 26 patients from 12 families with recessive mutations in ADCK4. Mutation detection rate was 1.9% among 534 consecutively screened cases. Patients with ADCK4 mutations showed a largely renal-limited phenotype, with three subjects exhibiting occasional seizures, one subject exhibiting mild mental retardation, and one subject exhibiting retinitis pigmentosa. ADCK4 nephropathy presented during adolescence (median age, 14.1 years) with nephrotic-range proteinuria in 44% of patients and advanced CKD in 46% of patients at time of diagnosis. Renal biopsy specimens uniformly showed FSGS. Whereas 47% and 36% of patients with mutations in WT1 and NPHS2, respectively, progressed to ESRD before 10 years of age, ESRD occurred almost exclusively in the second decade of life in ADCK4 nephropathy. However, CKD progressed much faster during adolescence in ADCK4 than in WT1 and NPHS2 nephropathy, resulting in similar cumulative ESRD rates (>85% for each disorder) in the third decade of life. In conclusion, ADCK4-related glomerulopathy is an important novel differential diagnosis in adolescents with SRNS/FSGS and/or CKD of unknown origin.Öğe Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group(Turkish J Pediatrics, 2010) Besbas, Nesrin; Ozaltin, Fatih; Emre, Sevinc; Anarat, Ali; Alpay, Harika; Bakkaloglu, Aysin; Baskin, Esra; Buyan, Necla; Donmez, Osman; Dusunsel, Ruhan; Ekim, Mesiha; Gok, Faysal; Gur-Guven, Ayfer; Kavukcu, Salih; Mir, Sevgi; Sonmez, FerahThe clinical course of focal segmental glomerulosclerosis (FSGS) is heterogeneous in children. To evaluate the clinical course and the predictors of outcome in Turkish children with primary FSGS, a retrospective study was conducted by the Turkish Pediatric Nephrology Study Group in 14 pediatric nephrology centers. Two hundred twenty-two patients (92 boys, 130 girls, aged 1-16 years) with biopsy-proven primary FSGS were included. One hundred forty-eight patients were followed-up for a median of 51 months (range: 0.26-270). The clinical course was characterized by complete remission in 50 (33.8%), persistent proteinuria in 50 (33.8%) and progression to renal failure in 48 (32.4%) patients. Progression to end-stage renal disease (ESRD) was significantly higher in patients who did not attain remission. Complete remission, partial remission and progress to renal failure were recorded in 37%, 32% and 28%, respectively, of the patients (n=73) treated with prednisone combined cyclophosphamide/cyclosporine A. However, in patients (n=33) treated with pulse methyl prednisolone plus oral prednisone (up to 20 months) combined with cyclophosphamide, complete remission in 51.5% and partial remission in 27.3% of the patients were noted. Progression to renal failure was observed in 9.1% of this group of patients. Multivariate analysis showed that only plasma creatinine at presentation was an independent predictive value for outcome. Patients with serum creatinine level higher than 1.5 mg/dl had 6.6 times increased rate of progression to renal failure. Failure to achieve remission is a predictor of renal failure in children with primary FSGS. The use of immunosuppressive treatment in conjunction with prolonged steroid seems beneficial in primary FSGS in children.Öğe Genetic screening in adolescents with steroid-resistant nephrotic syndrome(Elsevier Science Inc, 2013) Lipska, Beata S.; Iatropoulos, Paraskevas; Maranta, Ramona; Caridi, Gianluca; Ozaltin, Fatih; Anarat, Ali; Balat, Ayse; Gellermann, Jutta; Trautmann, Agnes; Erdogan, Ozlem; Saeed, Bassam; Emre, Sevinc; Bogdanovic, Radovan; Azocar, Marta; Balasz-Chmielewska, Irena; Benetti, Elisa; Caliskan, Salim; Mir, Sevgi; Melk, Anette; Ertan, Pelin; Baskin, Esra; Jardim, Helena; Davitaia, Tinatin; Wasilewska, Anna; Drozdz, Dorota; Szczepanska, Maria; Jankauskiene, Augustina; Serna Higuita, Lina Maria; Ardissino, Gianluigi; Ozkaya, Ozan; Kuzma-Mroczkowska, Elzbieta; Soylemezoglu, Oguz; Ranchin, Bruno; Medynska, Anna; Tkaczyk, Marcin; Peco-Antic, Amira; Akil, Ipek; Jarmolinski, Tomasz; Firszt-Adamczyk, Agnieszka; Dusek, Jiri; Simonetti, Giacomo D.; Gok, Faysal; Gheissari, Alaleh; Emma, Francesco; Krmar, Rafael T.; Fischbach, Michel; Printza, Nikoleta; Simkova, Eva; Mele, Caterina; Ghiggeri, Gian Marco; Schaefer, FranzGenetic screening paradigms for congenital and infantile nephrotic syndrome are well established; however, screening in adolescents has received only minor attention. To help rectify this, we analyzed an unselected adolescent cohort of the international PodoNet registry to develop a rational screening approach based on 227 patients with nonsyndromic steroid-resistant nephrotic syndrome aged 10-20 years. Of these, 21% had a positive family history. Autosomal dominant cases were screened for WT1, TRPC6, ACTN4, and INF2 mutations. All other patients had the NPHS2 gene screened, and WT1 was tested in sporadic cases. In addition, 40 sporadic cases had the entire coding region of INF2 tested. Of the autosomal recessive and the sporadic cases, 13 and 6%, respectively, were found to have podocin-associated nephrotic syndrome, and 56% of them were compound heterozygous for the nonneutral p.R229Q polymorphism. Four percent of the sporadic and 10% of the autosomal dominant cases had a mutation in WT1. Pathogenic INF2 mutations were found in 20% of the dominant but none of the sporadic cases. In a large cohort of adolescents including both familial and sporadic disease, NPHS2 mutations explained about 7% and WT1 4% of cases, whereas INF2 proved relevant only in autosomal dominant familial disease. Thus, screening of the entire coding sequence of NPHS2 and exons 8-9 of WT1 appears to be the most rational and cost-effective screening approach in sporadic juvenile steroid-resistant nephrotic syndrome.Öğe Low levels of urinary epidermal growth factor predict chronic kidney disease progression in children(Elsevier Science Inc, 2019) Azukaitis, Karolis; Ju, Wenjun; Kirchner, Marietta; Nair, Viji; Smith, Michelle; Fang, Zhiyin; Thurn-Valsassina, Daniela; Bayazit, Aysun; Niemirska, Anna; Canpolat, Nur; Bulut, Ipek Kaplan; Yalcinkaya, Fatos; Paripovic, Dusan; Harambat, Jerome; Cakar, Nilgun; Alpay, Harika; Lugani, Francesca; Mencarelli, Francesca; Civilibal, Mahmut; Erdogan, Hakan; Gellermann, Jutta; Vidal, Enrico; Tabel, Yilmaz; Gimpel, Charlotte; Ertan, Pelin; Yavascan, Onder; Melk, Anette; Querfeld, Uwe; Wuehl, Elke; Kretzler, Matthias; Schaefer, Franz; Arbeiter, Klaus; Rosales, Alejandra; Dusek, Jiri; Zaloszyc, Ariane; Querfeld, Uwe; Gellermann, Jutta; Liebau, Max; Weber, Lutz; Muschiol, Evelin; Buescher, Rainer; Oh, Jun; Melk, Anette; Thurn-Valassina, Daniela; Haffner, Dieter; Schaefer, Franz; Gimpel, Charlotte; John, Ulrike; Wygoda, Simone; Jeck, Nikola; Wigger, Marianne; Testa, Sara; Murer, Luisa; Matteucci, Chiara; Jankauskiene, Augustina; Azukaitis, Karolis; Drozdz, Dorota; Lugani, Francesca; Zurowska, Aleksandra; Zaniew, Marcin; Litwin, Mieczyslaw; Nimierska, Anna; Teixeira, Ana; Peco-Antic, Amira; Paripovic, Dusan; Laube, Guido; Dali, Cocuk Nefrolojisi Bilim; Anarat, Ali; Bayazit, Aysun; Duzova, Ali; Bilginer, Yelda; Caliskan, Salim; Canpolat, Nur; Civilibal, Mahmut; Mir, Sevgi; Soezeri, Betul; Kranz, Brigitta; Mencarelli, Francesca; Dorn, Brigitte; Yalcinkaya, Fatos; Baskin, Esra; Cakar, Nilgun; Soylemezoglu, Oguz; Emre, Sevinc; Candan, Cengiz; Kiyak, Aysel; Ozcelik, Gul; Alpay, Harika; Shroff, Rukshana; Rachin, Bruno; Harambat, Jerome; Szczepanska, Maria; Erdogan, Hakan; Donmez, Osman; Balat, Ayse; Aksu, Nejat; Tabel, Yilmaz; Ertan, Pelin; Yilmaz, Ebru; Anarat, Ali; Bakkaloglu, Aysin; Ozaltin, Fatih; Peco-Antic, Amira; Querfeld, Uwe; Gellermann, Jutta; Sallay, Peter; Drozdz, Dorota; Bonzel, Klaus-Eugen; Wingen, Anna-Margrete; Urowska, Aleksandra Z.; Balasz, Irena; Trivelli, Antonella; Perfumo, Francesco; Mueller-Wiefel, Dirk-Erhard; Moeller, Kerstin; Offner, Gisela; Enke, Barbara; Wuehl, Elke; Hadtstein, Charlotte; Mehls, Otto; Schaefer, Franz; Emre, Sevinc; Caliskan, Salim; Mir, Sevgi; Wygoda, Simone; Hohbach-Hohenfellner, Katharina; Jeck, Nickola; Klaus, Guenter; Ardissino, Gianluigi; Testa, Sara; Montini, Giovanni; Charbit, Marina; Niaudet, Patrick; Afonso, Alberto Caldas; Fernandes-Teixeira, Ana; Dusek, Jiri; Matteucci, Chiara; Picca, Stefano; Wigger, Marianne; Berg, Ulla B.; Celsi, Giovanni; Fischbach, Michel; Terzic, Joelle; Fydryk, Janusz; Urasinski, Tomasz; Coppo, Rosanna; Peruzzi, Licia; Arbeiter, Klaus; Jankauskiene, Augustina; Grenda, Ryszard; Litwin, Mieczyslaw; Neuhaus, Thomas J.Urinary epidermal growth factor (uEGF) has recently been identified as a promising biomarker of chronic kidney disease (CKD) progression in adults with glomerular disease. Low levels of uEGF predict CKD progression and appear to reflect the extent of tubulointerstitial damage. We investigated the relevance of uEGF in pediatric CKD. We performed a post hoc analysis of the Cardiovascular Comorbidity in Children with CKD (4C) study, which prospectively follows children aged 6-17 years with baseline estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m(2). uEGF levels were measured in archived urine collected within 6 months of enrollment. Congenital abnormalities of the kidney and urinary tract were the most common cause of CKD, with glomerular diseases accounting for <10% of cases. Median eGFR at baseline was 28 ml/min/1.73 m(2), and 288 of 623 participants (46.3%) reached the composite endpoint of CKD progression (50% eGFR loss, eGFR < 10 ml/min/1.73 m(2), or initiation of renal replacement therapy). In a Cox proportional hazards model, higher uEGF/Cr was associated with a decreased risk of CKD progression (HR 0.76; 95% CI 0.69-0.84) independent of age, sex, baseline eGFR, primary kidney disease, proteinuria, and systolic blood pressure. The addition of uEGF/Cr to a model containing these variables resulted in a significant improvement in C-statistics, indicating better prediction of the 1-, 2- and 3-year risk of CKD progression. External validation in a prospective cohort of 222 children with CKD demonstrated comparable results. Thus, uEGF may be a useful biomarker to predict CKD progression in children with CKD.Öğe Pathogens causing urinary tract infections in infants: a European overview by the ESCAPE study group(Springer, 2015) Alberici, Irene; Bayazit, Aysun Karabay; Drozdz, Dorota; Emre, Sevinc; Fischbach, Michel; Harambat, Jerome; Jankauskiene, Augustina; Litwin, Mieczyslaw; Mir, Sevgi; Morello, William; Peco-Antic, Amira; Sallay, Peter; Sever, Lale; Simonetti, Giacomo D.; Szczesniak, Przemyslaw; Teixeira, Ana; Vidal, Enrico; Wuehl, Elke; Mehls, Otto; Weber, Lutz T.; Schaefer, Franz; Montini, GiovanniKnowledge of the distribution spectrum of causative organisms and their resistance patterns has become a core requirement for the rational and effective management of urinary tract infections. In the context of a prospective trial on the use of antibiotic prophylaxis in infants with underling kidney malformations, we conducted an online survey among paediatric nephrologists on positive urine cultures (July 2010-June 2012) from both hospitalized and non-hospitalized infants under 24 months of age. We collected 4745 urine cultures (UCs) at 18 units in 10 European countries. Escherichia coli was the most frequent bacterium isolated from UCs; however, in 10/16 hospitals and in 6/15 community settings, E. coli was isolated in less than 50 % of the total positive UCs. Other bacterial strains were Klebsiella, Enterococcus, Proteus and Pseudomonas not only from hospital settings. E. coli showed a high resistance to amoxicillin and trimethoprim and variable to cephalosporin. Nitrofurantoin had a good rate of efficacy, with 11/16 hospitals and 11/14 community settings reporting a resistance lower than 5 %. Conclusion: E. coli is the most common organism causing UTIs in infants; however, other bacterial strains are frequently isolated. As a result, antibiotic prophylaxis should be more elastic and adaptable over time in order to guarantee maximum efficacy.Öğe Pathogens causing urinary tract infections in infants: a European overview by the ESCAPE study group(Springer, 2015) Alberici, Irene; Bayazit, Aysun Karabay; Drozdz, Dorota; Emre, Sevinc; Fischbach, Michel; Harambat, Jerome; Jankauskiene, Augustina; Litwin, Mieczyslaw; Mir, Sevgi; Morello, William; Peco-Antic, Amira; Sallay, Peter; Sever, Lale; Simonetti, Giacomo D.; Szczesniak, Przemyslaw; Teixeira, Ana; Vidal, Enrico; Wuehl, Elke; Mehls, Otto; Weber, Lutz T.; Schaefer, Franz; Montini, GiovanniKnowledge of the distribution spectrum of causative organisms and their resistance patterns has become a core requirement for the rational and effective management of urinary tract infections. In the context of a prospective trial on the use of antibiotic prophylaxis in infants with underling kidney malformations, we conducted an online survey among paediatric nephrologists on positive urine cultures (July 2010-June 2012) from both hospitalized and non-hospitalized infants under 24 months of age. We collected 4745 urine cultures (UCs) at 18 units in 10 European countries. Escherichia coli was the most frequent bacterium isolated from UCs; however, in 10/16 hospitals and in 6/15 community settings, E. coli was isolated in less than 50 % of the total positive UCs. Other bacterial strains were Klebsiella, Enterococcus, Proteus and Pseudomonas not only from hospital settings. E. coli showed a high resistance to amoxicillin and trimethoprim and variable to cephalosporin. Nitrofurantoin had a good rate of efficacy, with 11/16 hospitals and 11/14 community settings reporting a resistance lower than 5 %. Conclusion: E. coli is the most common organism causing UTIs in infants; however, other bacterial strains are frequently isolated. As a result, antibiotic prophylaxis should be more elastic and adaptable over time in order to guarantee maximum efficacy.Öğe Pathogens causing urinary tract infections in infants: a European overview by the ESCAPE study group(Springer, 2015) Alberici, Irene; Bayazit, Aysun Karabay; Drozdz, Dorota; Emre, Sevinc; Fischbach, Michel; Harambat, Jerome; Jankauskiene, Augustina; Litwin, Mieczyslaw; Mir, Sevgi; Morello, William; Peco-Antic, Amira; Sallay, Peter; Sever, Lale; Simonetti, Giacomo D.; Szczesniak, Przemyslaw; Teixeira, Ana; Vidal, Enrico; Wuehl, Elke; Mehls, Otto; Weber, Lutz T.; Schaefer, Franz; Montini, GiovanniKnowledge of the distribution spectrum of causative organisms and their resistance patterns has become a core requirement for the rational and effective management of urinary tract infections. In the context of a prospective trial on the use of antibiotic prophylaxis in infants with underling kidney malformations, we conducted an online survey among paediatric nephrologists on positive urine cultures (July 2010-June 2012) from both hospitalized and non-hospitalized infants under 24 months of age. We collected 4745 urine cultures (UCs) at 18 units in 10 European countries. Escherichia coli was the most frequent bacterium isolated from UCs; however, in 10/16 hospitals and in 6/15 community settings, E. coli was isolated in less than 50 % of the total positive UCs. Other bacterial strains were Klebsiella, Enterococcus, Proteus and Pseudomonas not only from hospital settings. E. coli showed a high resistance to amoxicillin and trimethoprim and variable to cephalosporin. Nitrofurantoin had a good rate of efficacy, with 11/16 hospitals and 11/14 community settings reporting a resistance lower than 5 %. Conclusion: E. coli is the most common organism causing UTIs in infants; however, other bacterial strains are frequently isolated. As a result, antibiotic prophylaxis should be more elastic and adaptable over time in order to guarantee maximum efficacy.Öğe Quality of life in children with chronic kidney disease (with child and parent assessments)(Springer, 2010) Buyan, Necla; Turkmen, Mehmet Atilla; Bilge, Ilmay; Baskin, Esra; Haberal, Mehmet; Bilginer, Yelda; Mir, Sevgi; Emre, Sevinc; Akman, Sema; Ozkaya, Ozan; Fidan, Kibriya; Alpay, Harika; Kavukcu, Salih; Sever, Lale; Ozcakar, Zeynep Birsin; Dogrucan, NahideHerein the results of a multicenter study from the Turkish Pediatric Kidney Transplantation Study Group are reported. The aims of this study were to compare the quality of life (QoL) scores of Turkish children who are dialysis patients (DP), renal transplant recipients (TR), and age-matched healthy controls and to compare child-self and parent-proxy scores. The Turkish versions of the Kinder Lebensqualitat Fragebogen (KINDLA (R)) questionnaires were used as a QoL measure. The study group consisted of 211 children and adolescents with chronic kidney disease (CKD) (139 TR and 72 DP aged between 4-18 years; 13.7 A +/- 3.5 years) from 11 university hospitals, 129 parents of these patients, 232 age-matched healthy children and adolescents (aged between 4-18 years; 13.1 +/- 3.5 years) and 156 of their parents. Patients with CKD had lower scores in all subscales except for physical well-being than those in the control group. TR had higher scores in physical well-being, self-esteem, friends' subscales, and total scores than DP. Child-self scores were lower than parent-proxy scores, especially in CKD, DP, and control groups. Concordance between parent-proxy and child-self reports in the TR, DP, CKD, and control groups was only moderate for the majority of subscales (r = 0.41-0.61). It was concluded that parent-proxy scores on the QoL were not equivalent to child-self scores and that evaluating both children's and parents' perspectives were important. Additionally, psychosocial counseling is crucial not only for patients with CKD but also for their parents.Öğe Strict Blood-Pressure Control and Progression of Renal Failure in Children(Massachusetts Medical Soc, 2009) Wuehl, Elke; Trivelli, Antonella; Picca, Stefano; Litwin, Mieczyslaw; Peco-Antic, Amira; Zurowska, Aleksandra; Testa, Sara; Jankauskiene, Augustina; Emre, Sevinc; Caldas-Afonso, Alberto; Anarat, Ali; Niaudet, Patrick; Mir, Sevgi; Bakkaloglu, Aysin; Enke, Barbara; Montini, Giovanni; Wingen, Ann-Margret; Sallay, Peter; Jeck, Nikola; Berg, Ulla; Caliskan, Salim; Wygoda, Simone; Hohbach-Hohenfellner, Katharina; Dusek, Jiri; Urasinski, Tomasz; Arbeiter, Klaus; Neuhaus, Thomas; Gellermann, Jutta; Drozdz, Dorota; Fischbach, Michel; Moeller, Kristina; Wigger, Marianne; Peruzzi, Licia; Mehls, Otto; Schaefer, FranzBackground Although inhibition of the renin-angiotensin system delays the progression of renal failure in adults with chronic kidney disease, the blood-pressure target for optimal renal protection is controversial. We assessed the long-term renoprotective effect of intensified blood-pressure control among children who were receiving a fixed high dose of an angiotensin-converting-enzyme (ACE) inhibitor. Methods After a 6-month run-in period, 385 children, 3 to 18 years of age, with chronic kidney disease (glomerular filtration rate of 15 to 80 ml per minute per 1.73 m(2) of body-surface area) received ramipril at a dose of 6 mg per square meter of body-surface area per day. Patients were randomly assigned to intensified blood-pressure control (with a target 24-hour mean arterial pressure below the 50th percentile) or conventional blood-pressure control (mean arterial pressure in the 50th to 95th percentile), achieved by the addition of antihypertensive therapy that does not target the renin-angiotensin system; patients were followed for 5 years. The primary end point was the time to a decline of 50% in the glomerular filtration rate or progression to end-stage renal disease. Secondary end points included changes in blood pressure, glomerular filtration rate, and urinary protein excretion. Results A total of 29.9% of the patients in the group that received intensified blood-pressure control reached the primary end point, as assessed by means of a Kaplan-Meier analysis, as compared with 41.7% in the group that received conventional blood-pressure control (hazard ratio, 0.65; confidence interval, 0.44 to 0.94; P = 0.02). The two groups did not differ significantly with respect to the type or incidence of adverse events or the cumulative rates of withdrawal from the study (28.0% vs. 26.5%). Proteinuria gradually rebounded during ongoing ACE inhibition after an initial 50% decrease, despite persistently good blood-pressure control. Achievement of blood-pressure targets and a decrease in proteinuria were significant independent predictors of delayed progression of renal disease. Conclusions Intensified blood-pressure control, with target 24-hour blood-pressure levels in the low range of normal, confers a substantial benefit with respect to renal function among children with chronic kidney disease. Reappearance of proteinuria after initial successful pharmacologic blood-pressure control is common among children who are receiving long-term ACE inhibition. (ClinicalTrials.gov number, NCT00221845.)Öğe Strict Blood-Pressure Control and Progression of Renal Failure in Children(Massachusetts Medical Soc, 2009) Wuehl, Elke; Trivelli, Antonella; Picca, Stefano; Litwin, Mieczyslaw; Peco-Antic, Amira; Zurowska, Aleksandra; Testa, Sara; Jankauskiene, Augustina; Emre, Sevinc; Caldas-Afonso, Alberto; Anarat, Ali; Niaudet, Patrick; Mir, Sevgi; Bakkaloglu, Aysin; Enke, Barbara; Montini, Giovanni; Wingen, Ann-Margret; Sallay, Peter; Jeck, Nikola; Berg, Ulla; Caliskan, Salim; Wygoda, Simone; Hohbach-Hohenfellner, Katharina; Dusek, Jiri; Urasinski, Tomasz; Arbeiter, Klaus; Neuhaus, Thomas; Gellermann, Jutta; Drozdz, Dorota; Fischbach, Michel; Moeller, Kristina; Wigger, Marianne; Peruzzi, Licia; Mehls, Otto; Schaefer, FranzBackground Although inhibition of the renin-angiotensin system delays the progression of renal failure in adults with chronic kidney disease, the blood-pressure target for optimal renal protection is controversial. We assessed the long-term renoprotective effect of intensified blood-pressure control among children who were receiving a fixed high dose of an angiotensin-converting-enzyme (ACE) inhibitor. Methods After a 6-month run-in period, 385 children, 3 to 18 years of age, with chronic kidney disease (glomerular filtration rate of 15 to 80 ml per minute per 1.73 m(2) of body-surface area) received ramipril at a dose of 6 mg per square meter of body-surface area per day. Patients were randomly assigned to intensified blood-pressure control (with a target 24-hour mean arterial pressure below the 50th percentile) or conventional blood-pressure control (mean arterial pressure in the 50th to 95th percentile), achieved by the addition of antihypertensive therapy that does not target the renin-angiotensin system; patients were followed for 5 years. The primary end point was the time to a decline of 50% in the glomerular filtration rate or progression to end-stage renal disease. Secondary end points included changes in blood pressure, glomerular filtration rate, and urinary protein excretion. Results A total of 29.9% of the patients in the group that received intensified blood-pressure control reached the primary end point, as assessed by means of a Kaplan-Meier analysis, as compared with 41.7% in the group that received conventional blood-pressure control (hazard ratio, 0.65; confidence interval, 0.44 to 0.94; P = 0.02). The two groups did not differ significantly with respect to the type or incidence of adverse events or the cumulative rates of withdrawal from the study (28.0% vs. 26.5%). Proteinuria gradually rebounded during ongoing ACE inhibition after an initial 50% decrease, despite persistently good blood-pressure control. Achievement of blood-pressure targets and a decrease in proteinuria were significant independent predictors of delayed progression of renal disease. Conclusions Intensified blood-pressure control, with target 24-hour blood-pressure levels in the low range of normal, confers a substantial benefit with respect to renal function among children with chronic kidney disease. Reappearance of proteinuria after initial successful pharmacologic blood-pressure control is common among children who are receiving long-term ACE inhibition. (ClinicalTrials.gov number, NCT00221845.)
