Publications by authors named "Marcus Weitz"

25 Publications

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Prophylactic antithrombotic management in adult and pediatric kidney transplantation: A systematic review and meta-analysis.

Pediatr Transplant 2021 Apr 7:e14021. Epub 2021 Apr 7.

Department of General Pediatrics and Haematology/Oncology, University Children's Hospital Tuebingen, Tuebingen, Germany.

Background: RGT is a major cause for early graft loss after KTx. Although evidence-based recommendations are lacking, aP is often used to prevent RGT. This systematic review aimed to determine the effectiveness and safety of aP in adult and pediatric KTx recipients.

Methods: MEDLINE, EMBASE, Cochrane Controlled Trials Register, conference proceedings, and electronic databases for trial registries were searched for eligible studies using search terms relevant to this review (April 21, 2020). The systematic review was carried out following the recommendations of the Cochrane Collaboration and the Prefered Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Statement.

Results: Twelve studies comprising 2370 patients (adult = 1415, pediatric = 955) were included, of which three were RCTs. The overall risk for developing RGT was lower in the group with aP compared with the control group (RR 0.24, 95% confidence interval 0.12-0.49). The antithrombotic drugs used were heparin (7/12), acetylsalicylic acid (2/12), a combination of both (2/12), and dipyridamole (1/12) with a high variability in timing, dosing, and mode of application. Adverse effects were reported rarely, with minor bleeding as the main complication. The non-randomized studies had significant risks of bias in the domains of patient selection, confounder, and measurement of outcomes.

Conclusion: Based on pooled analysis, aP seems to reduce the risk of RGT in KTx. However, the reliability of these results is limited, as the quality of the available studies is poor and information on adverse effects associated with aP is scarce. Additional high-quality research is urgently needed to provide sufficient data supporting the use of aP in KTx.
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http://dx.doi.org/10.1111/petr.14021DOI Listing
April 2021

Febrile Urinary Tract Infections in Children with Primary Non-Refluxing Megaureter: A Systematic Review and Meta-Analysis.

Klin Padiatr 2020 Dec 17. Epub 2020 Dec 17.

Pediatric Nephrology, University Children's Hospital Tübingen, Tuebingen, Germany.

Background: Knowledge of the baseline risk of febrile urinary tract infections in patients with primary non-refluxing megaureter can help clinicians to make informed decisions for offering continuous antibiotic prophylaxis.

Objective: The primary objective of this systematic review was to determine the pooled prevalence of febrile urinary tract infections in patients with primary non-refluxing megaureter selected for primary non-surgical management independent of associated attributed risk factors at initial presentation in order to assess the value of continuous antibiotic prophylaxis.

Methods: MEDLINE, EMBASE, and Cochrane Controlled Trials Register electronic databases were searched for eligible studies without language and time restriction. The systematic review was carried out following the recommendations of the Cochrane Collaboration and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses. (PROSPERO registration number CRD42018104752).

Results: Of 25 871 records, 16 studies (n=749 patients) were eligible for inclusion. The overall pooled prevalence of febrile urinary tract infections in patients with primary non-refluxing megaureter was 14.35% (95% confidence interval: 8.8-22.6). The calculated number needed to treat for patients on continuous antibiotic prophylaxis to prevent one single febrile urinary tract infection over the course of 1-2 years would be 4.3.

Conclusion: Based on the current available evidence the use of continuous antibiotic prophylaxis for children with PM selected for primary non-surgical treatment should be taken into consideration, at least in patients with urinary outflow impairment, higher grade of ureteral dilatation, and for children in the first months of life.
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http://dx.doi.org/10.1055/a-1303-4695DOI Listing
December 2020

Current practice of antithrombotic prophylaxis in pediatric kidney transplantation-Results of an international survey on behalf of the European Society for Paediatric Nephrology.

Pediatr Transplant 2020 11 18;24(7):e13799. Epub 2020 Aug 18.

Department of General Pediatrics and Hematology/Oncology, University Hospital Tübingen, University Children`s Hospital, Tübingen, Germany.

Background: Renal graft thrombosis (RGT) is one of the main causes for early graft loss in pediatric kidney transplantation (KTx). Despite the lack of evidence-based recommendations, antithrombotic prophylaxis (aP) is used to prevent RGT.

Methods: An online survey supported by the European Society for Pediatric Nephrology was developed to investigate the current practice of aP in pediatric KTx recipients <18 years.

Results: A total of 80 pediatric KTx centers from 37 countries participated in the survey. Antithrombotic prophylaxis was performed in 96% of the pediatric renal transplant centers (all/selected patients: 54%/42%). The main overall used drugs were as follows: low-molecular-weight heparin (89%), unfractionated heparin (UFH) (69%), and acetylsalicylic acid (ASS) (55%). Ten different aP management strategies were identified as follows: 51% used a single drug and 48% combined two drugs sequentially. The corresponding centers started aP predominantly within 24 hours after pediatric KTx; 51% preferred UFH for starting aP. In centers switching to a second drug (51%), this change was performed after 10 ± 6 days; of these 57% preferred ASS for maintenance aP. Reported median aP duration was 51 days (range 1-360).

Conclusions: Despite the use of aP in almost all responding pediatric KTx centers, there is no uniform management strategy. Notwithstanding, UFH seems to be the preferred drug for the early post-operative period of pediatric KTx, and ASS for maintenance prophylaxis following pediatric KTx. Prospective studies are needed to further evaluate the benefits and risks of aP, preferably resulting in guidelines for the management in pediatric KTx.
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http://dx.doi.org/10.1111/petr.13799DOI Listing
November 2020

Paediatric end-stage renal disease and renal replacement therapy in Switzerland: survival and treatment trends over four decades.

Swiss Med Wkly 2020 Jul 27;150:w20300. Epub 2020 Jul 27.

Swiss Paediatric Renal Registry, Institute of Social and Preventive Medicine, Research Group on Child and Adolescent Health, University of Bern, Switzerland / Nephrology Unit, University Children's Hospital, University of Zurich, Switzerland.

Background: Renal replacement therapy for paediatric end-stage renal disease (ESRD) has developed steadily since its introduction five decades ago. Continuous and long-term analysis of patient outcomes is essential for quality control.

Methods: The Swiss Paediatric Renal Registry, founded in 1970, includes patients diagnosed with ESRD, defined as dialysis for more than three months or renal transplantation, at age <20 years. Here we describe the incidence, primary renal disease, treatment modalities and long-term outcomes over 45 years.

Results: This paper reports on 367 children and adolescents treated with chronic renal replacement therapy in Switzerland. Incidence was 5.4 per million children per year, with a tendency to increase over time. The primary renal disease was congenital anomalies of the kidney and the urinary tract in 133 (36%), monogenetic hereditary diseases in 122 (33%) and acquired diseases in 112 (31%) patients. The first renal replacement therapy was haemodialysis in 194 (53%), peritoneal dialysis in 116 (32%) and pre-emptive renal transplantation in 57 (15%) patients. Over the years, pre-emptive renal transplantation became more frequent (34% of all first renal replacement therapies in 2006–2015), reducing the duration of dialysis. Median time on dialysis until transplantation decreased from 1.60 years in 1981–90 to 0.34 years in 2010–15. Over the four decades 1970–80, 1981–90,1991–2000 and 2001–10, the one-year graft survival rate improved from 0.76 to 0.80, 0.89 and then 0.96; and the five-year graft survival rate improved from 0.44 to 0.64, 0.84 and 0.89, respectively. The five-year patient survival rates for the four decades were 0.83, 0.99, 0.93 and 0.94; and the 10-year patient survival rates were 0.75, 0.96, 0.88 and 0.94, respectively. In the four cohorts starting renal replacement therapy in the 70s, 80s, 90s and 00s, the number of children alive after five years of renal replacement therapy increased from 15 to 24, 47 and then 45 respectively. In total, 29 patients (8%) died during chronic renal replacement therapy with ESRD before the age of 20 years.

Conclusion: Over time, a higher number of children on renal replacement therapy survived, graft survival improved, and the duration of dialysis before renal transplantation decreased.
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http://dx.doi.org/10.4414/smw.2020.20300DOI Listing
July 2020

CASPR2 autoimmunity in children expanding to mild encephalopathy with hypertension.

Neurology 2020 06 18;94(22):e2290-e2301. Epub 2020 May 18.

From the Division of Pediatric Epileptology (S. Syrbe), Centre for Paediatrics and Adolescent Medicine, University Hospital Heidelberg, Germany; Division of Pediatric Neurology (G.M.S., R.S.), University Children's Hospital Zurich; Department of Neurology (J.B., R.I.F.), University & University Hospitals of Geneva, Switzerland; Division of Pediatric Neurology (I.B.), Developmental Neurology and Social Pediatrics, Department of Pediatrics and Epilepsy Center for Children, Adolescents and Adults, University Hospital LMU Munich; Laboratory Krone (C.I.B., C.G.B.), Bad Salzuflen; Department of Pediatrics and Pediatric Neurology (P.H.), Faculty of Medicine, Georg August University, Goettingen; Department of Child Neurology (J.K., A.W.), University Children's Hospital, Tuebingen; Epilepsy Center Bethel (T.P., C.G.B.), Krankenhaus Mara, Bielefeld, Germany; Clinic of Immunology (E.P.-M.), University Hospital Zurich; Kantonsspital Graubünden (S. Schmid, S. Strozzi), Chur; Pediatric Nephrology Unit (M.W.), University Children's Hospital Zurich, Switzerland; Division of Child Neurology and Metabolic Medicine (A.Z.), Centre for Paediatrics and Adolescent Medicine, University Hospital Heidelberg; Institute of Clinical Chemistry (K.-P.W., F.L.), Neuroimmunology Section, University Hospital Schleswig-Holstein Kiel/Lübeck; Department of Neurology (K.-P.W.), University of Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-University Kiel, Germany.

Objective: To delineate autoimmune disease in association with contactin-associated protein 2 (CASPR2) antibodies in childhood, we reviewed the clinical phenotype of children with CASPR2 antibodies.

Methods: Retrospective assessment of patients recruited through laboratories specialized in autoimmune CNS disease.

Results: Ten children with serum CASPR2 antibodies were identified (age at manifestation 18 months to 17 years). Eight children with CASPR2 antibody titers from ≥1:160 to 1:5,120 had complex autoimmune diseases with an age-dependent clinical phenotype. Two children with structural epilepsy due to CNS malformations harbored nonspecific low-titer CASPR2 antibodies (serum titers 1:80). The clinical symptoms of the 8 children with high-titer CASPR2 antibodies were general weakness (8/8), sleep dysregulation (8/8), dysautonomia (8/8) encephalopathy (7/8), neuropathic pain (7/8), neuromyotonia (3/8), and flaccid paresis (3/8). Adolescents (3/8) showed pain, neuromyotonia, and encephalopathy, whereas younger children (5/8) displayed severe hypertension, encephalopathy, and hormonal dysfunction mimicking a systemic disease. No tumors were identified. Motor symptoms remitted with immunotherapy. Mild behavioral changes persisted in 1 child, and autism spectrum disorder was diagnosed during follow-up in a young boy.

Conclusion: High-titer CASPR2 antibodies are associated with Morvan syndrome in children as young as 2 years. However, CASPR2 autoimmunity mimics systemic disease and hypertensive encephalopathy in children younger than 7 years. The outcome following immunotherapy was mostly favorable; long-term behavioral impairment may occur in younger children.
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http://dx.doi.org/10.1212/WNL.0000000000009523DOI Listing
June 2020

The use of cinacalcet after pediatric renal transplantation: an international CERTAIN Registry analysis.

Pediatr Nephrol 2020 09 4;35(9):1707-1718. Epub 2020 May 4.

Reference Center for Rare Renal Disorders, Reference Center for Rare Disorders of Calcium and Phosphate Metabolism, Department of Pediatric Nephrology, Rheumatology and Dermatology, Femme Mère Enfant Hospital, Bron Cedex, France.

Background: Secondary hyperparathyroidism (SHPT) may persist after renal transplantation (RTx), inducing hypophosphatemia and hypercalcemia that precludes the use of vitamin D analogs. The calcimimetic cinacalcet improved plasma calcium and parathyroid hormone (PTH) levels in randomized controlled trials in adults after RTx, but pediatric data are scarce.

Methods: In this retrospective study, we analyzed 20 pediatric patients from the Cooperative European Paediatric Renal TransplAnt Initiative (CERTAIN) Registry who received cinacalcet after RTx. The results are presented as median and interquartile range (25th-75th percentile).

Results: At 13.7 (11.0-16.5) years of age, 20 pediatric patients received a renal allograft. Cinacalcet was introduced at 0.4 (0.3-2.7) years post-transplant at an estimated glomerular filtration rate (eGFR) of 50 (34-66) mL/min/1.73 m, plasma calcium of 2.58 (2.39-2.71) mmol/L, age-standardized (z score) phosphate of - 1.7 (- 2.7-- 0.4), and PTH of 136 (95-236) ng/L. The starting dose of cinacalcet was 0.5 (0.3-0.8) mg/kg per day, with a maximum dose of 1.1 (0.5-1.3) mg/kg per day. With a follow-up of 3.0 (1.5-3.6) years on cinacalcet therapy, eGFR remained stable; PTH levels decreased to 66 (56-124) ng/L at the last follow-up (p = 0.015). One patient displayed hypocalcemia (1.8 mmol/L). Cinacalcet was withdrawn in three patients (hypocalcemia, parathyroidectomy, incompliance). Nephrocalcinosis of the graft was not reported.

Conclusions: This pilot study suggests that cinacalcet as off-label therapy for SHPT after pediatric RTx is efficacious in controlling post-transplant SHPT with acceptable tolerability. Continuing cinacalcet even with normal PTH can lead to dangerous life-threatening hypocalcemia. Therefore, at each subsequent visit, the need to continue cinacalcet must be assessed.
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http://dx.doi.org/10.1007/s00467-020-04558-8DOI Listing
September 2020

Timing of voiding cystourethrography after febrile urinary tract infection in children: a systematic review.

Arch Dis Child 2020 03 29;105(3):264-269. Epub 2019 Aug 29.

Nephrology, University Children's Hospital Zurich, Zurich, Switzerland

Background: Despite a trend towards early voiding cystourethrography (VCUG) after febrile urinary tract infection (fUTI) in children, clinical guidelines do not comment on the optimal timing and current practice varies considerably.

Objective: To assess whether the detection rate of vesicoureteric reflux (VUR) in children depends on the time period of VCUG procedure after onset of antibiotic therapy.

Methods: MEDLINE, EMBASE and Cochrane Controlled Trials Register electronic databases were searched for eligible studies without language or time restriction (19 November 2018). Inclusion criteria were (1) patients <18 years of age; (2) VCUG performed in patients with fUTI after onset of antibiotic therapy either in the same patient population or in two or more different patient populations within one study at different time periods; and (3) with reported detection rate of VUR. The systematic review was carried out following the recommendations of the Cochrane Collaboration and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses.

Results: Of 4175 records, nine studies were included (number of patients, n=1745) for the main outcome prevalence of VUR by VCUG <8 days compared with VCUG ≥8 days after onset of antibiotic therapy. Pooled overall prevalence of VUR was not significantly different between the early and the late VCUG groups (risk ratio 0.98, 95% CI 0.81 to 1.19). Prevalence of VUR stratified by grade was not significantly different between the two groups.

Conclusion: Early VCUG within 8 days after onset of antibiotic therapy does not affect the prevalence of VUR.

Trial Registration Number: CRD42018117545.
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http://dx.doi.org/10.1136/archdischild-2019-316958DOI Listing
March 2020

Association between timing of dialysis initiation and clinical outcomes in the paediatric population: an ESPN/ERA-EDTA registry study.

Nephrol Dial Transplant 2019 11;34(11):1932-1940

Department of Pediatric Nephrology, Gazi University, Ankara, Turkey.

Background: There is no consensus regarding the timing of dialysis therapy initiation for end-stage kidney disease (ESKD) in children. As studies investigating the association between timing of dialysis initiation and clinical outcomes are lacking, we aimed to study this relationship in a cohort of European children who started maintenance dialysis treatment.

Methods: We used data on 2963 children from 21 different countries included in the European Society of Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association Registry who started renal replacement therapy before 18 years of age between 2000 and 2014. We compared two groups according to the estimated glomerular filtration rate (eGFR) at start: eGFR ≥8 mL/min/1.73 m2 (early starters) and eGFR <8 mL/min/1.73 m2 (late starters). The primary outcomes were patient survival and access to transplantation. Secondary outcomes were growth and cardiovascular risk factors. Sensitivity analyses were performed to account for selection- and lead time-bias.

Results: The median eGFR at the start of dialysis was 6.1 for late versus 10.5 mL/min/1.73 m2 for early starters. Early starters were older [median: 11.0, interquartile range (IQR): 5.7-14.5 versus 9.4, IQR: 2.6-14.1 years]. There were no differences observed between the two groups in mortality and access to transplantation at 1, 2 and 5 years of follow-up. One-year evolution of height standard deviation scores was similar among the groups, whereas hypertension was more prevalent among late initiators. Sensitivity analyses resulted in similar findings.

Conclusions: We found no evidence for a clinically relevant benefit of early start of dialysis in children with ESKD. Presence of cardiovascular risk factors, such as high blood pressure, should be taken into account when deciding to initiate or postpone dialysis in children with ESKD, as this affects the survival.
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http://dx.doi.org/10.1093/ndt/gfz069DOI Listing
November 2019

Membranoproliferative glomerulonephritis and C3 glomerulopathy in children: change in treatment modality? A report of a case series.

Clin Kidney J 2018 Aug 28;11(4):479-490. Epub 2018 Feb 28.

Pediatric Nephrology Unit, University Children's Hospital Zurich, Zurich, Switzerland.

Background: Membranoproliferative glomerulonephritis (MPGN) with immune complexes and C3 glomerulopathy (C3G) in children are rare and have a variable outcome, with some patients progressing to end-stage renal disease (ESRD). Mutations in genes encoding regulatory proteins of the alternative complement pathway and of complement C3 (C3) have been identified as concausative factors.

Methods: Three children with MPGN type I, four with C3G, i.e. three with C3 glomerulonephritis (C3GN) and one with dense deposit disease (DDD), were followed. Clinical, autoimmune data, histological characteristics, estimated glomerular filtration rate (eGFR), proteinuria, serum C3, genetic and biochemical analysis were assessed.

Results: The median age at onset was 7.3 years and the median eGFR was 72 mL/min/1.73 m. Six children had marked proteinuria. All were treated with renin-angiotensin-aldosterone system (RAAS) blockers. Three were given one or more immunosuppressive drugs and two eculizumab. At the last median follow-up of 9 years after diagnosis, three children had normal eGFR and no or mild proteinuria on RAAS blockers only. Among four patients without remission of proteinuria, genetic analysis revealed mutations in complement regulator proteins of the alternative pathway. None of the three patients with immunosuppressive treatment achieved partial or complete remission of proteinuria and two progressed to ESRD and renal transplantation. Two patients treated with eculizumab revealed relevant decreases in proteinuria.

Conclusions: In children with MPGN type I and C3G, the outcomes of renal function and response to treatment modality show great variability independent from histological diagnosis at disease onset. In case of severe clinical presentation at disease onset, early genetic and biochemical analysis of the alternative pathway dysregulation is recommended. Treatment with eculizumab appears to be an option to slow disease progression in single cases.
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http://dx.doi.org/10.1093/ckj/sfy006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6070093PMC
August 2018

Outcome of renal transplantation in small infants: a match-controlled analysis.

Pediatr Nephrol 2018 06 13;33(6):1057-1068. Epub 2018 Mar 13.

Department of Paediatrics I, University Children's Hospital, Heidelberg, Germany.

Background: Infants with a body weight of less than 10 kg are often not considered to be suitable candidates for renal transplantation (RTx). The objective of this study was to evaluate this arbitrary weight threshold for pediatric RTx.

Methods: We conducted a multicenter, retrospective, match-controlled cohort study on infants weighing less than 10 kg at time of engrafting (low-weight group [LWG], n = 38) compared to a matched control group (n = 76) with a body weight of 10-15 kg, using data from the first 2 years post-transplant derived from the CERTAIN Registry.

Results: Patient survival was 97 and 100% in the LWG and control groups, respectively (P = 0.33), and death-censored graft survival was 100 and 95% in the LWG and control groups, respectively (P = 0.30). Estimated glomerular filtration rate at 2 years post-transplant was excellent and comparable between the groups (LWG 77.6 ± 34.9 mL/min/1.73 m; control 74.8 ± 29.1 mL/min/1.73 m; P = 0.68). The overall incidences of surgery-related complications (LWG 11%, control 23%; P = 0.12) and medical outcome measures (LWG 23%, control 36%, P = 0.17) were not significantly different between the groups. The medical outcome measures included transplant-related viral diseases (LWG 10%, control 21%; P = 0.20), acute rejection episodes (LWG 14%, control 29%; P = 0.092), malignancies (LWG 3%, control 0%; P = 0.33) and arterial hypertension (LWG 73%, control 67%; P = 0.57).

Conclusions: These data suggest that RTx in low-weight children is a feasible option, at least in selected centers with appropriate surgical and medical expertise.
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http://dx.doi.org/10.1007/s00467-018-3895-5DOI Listing
June 2018

Imaging in children with unilateral ureteropelvic junction obstruction: time to reduce investigations?

Eur J Pediatr 2017 Sep 15;176(9):1173-1179. Epub 2017 Jul 15.

University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

The objective of the study was the development of an abridged risk-stratified imaging algorithm for the management of children with unilateral ureteropelvic junction obstruction (UPJO). Data on timing, frequency and duration of diagnostic imaging in children with unilateral UPJO was extracted retrospectively. Based on these findings, an abridged imaging algorithm was developed without changing the intended management by the clinicians and the outcome of the individual patient. The potential reduction of imaging studies was analysed and stratified by risk and management groups. The reduction in imaging studies, seen for ultrasound (US) and functional imaging (FI), was 45% each. On average, this is equivalent to 3 US and 1 FI studies less for every patient within the study period. The change was more pronounced in the low-risk groups. Progression of UPJO never occurred after 2 years of age and all secondary surgeries were carried out until the age of 3.

Conclusions: Although our findings need to be validated by further prospective research, the developed imaging algorithm represents a risk-stratified approach towards less imaging studies in children with unilateral UPJO, and a follow-up beyond 3 years of age should be considered only in selected cases at the discretion of the clinician. What is Known: • ultrasound and functional imaging represent an integral part of therapeutic decision-making in children with unilateral ureteropelvic junction obstruction • imaging studies cannot accurately assess which patients are in need of surgical intervention, therefore close, serial imaging is preferred What is New: • a new, risk-stratified imaging algorithm was developed for the first 3 years of life • applying this algorithm could lead to a considerable reduction of imaging studies, and also the associated risks and health-care costs.
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http://dx.doi.org/10.1007/s00431-017-2966-0DOI Listing
September 2017

Primary non-surgical management of unilateral ureteropelvic junction obstruction in children: a systematic review.

Pediatr Nephrol 2017 Dec 23;32(12):2203-2213. Epub 2016 Dec 23.

Department of Nephrology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

Ureteropelvic junction obstruction (UPJO) is the most common obstructive uropathy and its optimal management remains controversial. However, there is a current trend towards non-surgical management. We aimed to determine the effects of the non-surgical management in children with unilateral UPJO. For a systematic review, we searched MEDLINE, EMBASE, CENTRAL, clinical trials registries, and selected conference proceedings for eligible studies. Any type of study reporting the outcomes renal function, secondary surgical intervention, drainage pattern or hydronephrosis of non-surgical management in children with unilateral UPJO was included. Data from 20 studies were extracted and evaluated by two independent authors. The pooled prevalence was 21% for split renal function deterioration, 27.9% for secondary surgical intervention, 3.2% for progressive hydronephrosis, and 82.2% for improved drainage pattern. Not all patients with surgical intervention regained split renal function from enrolment. Renal imaging methods did not strongly correlate with each other. Many studies had to be excluded because of a lack of detection of an obstruction or mixed populations with bilateral UPJO or other uropathies. The variable definitions of UPJO, different criteria for surgical intervention, incongruity of management protocols, and the imprecise reporting of outcomes were limiting factors in the comparability of the results, leading to heterogeneity in meta-analyses. Although the available evidence cannot recommend or refute the current non-surgical management, the systematic review clarifies aspects of the ongoing controversy by providing realistic estimates for non-surgical management in children with unilateral UPJO. Additionally, it reveals unclear potential risks, particularly for long-term outcomes, which were rarely reported.
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http://dx.doi.org/10.1007/s00467-016-3566-3DOI Listing
December 2017

To screen or not to screen for vesicoureteral reflux in children with ureteropelvic junction obstruction: a systematic review.

Eur J Pediatr 2017 Jan 25;176(1):1-9. Epub 2016 Nov 25.

Department of Nephrology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

Ureteropelvic junction obstruction (UPJO) and vesicoureteral reflux (VUR) are the most common uropathies. The co-occurrence of both anomalies has led to the practice of screening for VUR in children with UPJO to prevent deterioration of kidney function due to renal scarring following urinary tract infections (UTIs). We determined the prevalence of VUR in children with UPJO for a critical assessment of VUR screening by voiding cystourethrography (VCUG). A systematic search strategy in MEDLINE, EMBASE, and CENTRAL was carried out for all articles that included VCUG, and renal scintigraphy or any other appropriate imaging technique for the diagnosis of UPJO. Twenty studies were eligible for inclusion. We found a pooled prevalence for VUR of 8.2 % (95 % CI = 3.6-12.7), about a threefold increase compared to the general pediatric population. VUR occurred bilateral or contralateral to the kidney with UPJO in 5.7 % (95 % CI = 3.0-8.5), equivalent to 75 % of all children with VUR. Considering the effect size of VUR treatment with antibiotics, about 207 and 278 children would need to undergo VCUG to prevent one febrile UTI and one case of renal scarring by 1-2 years, respectively.

Conclusion: Against this background, screening for VUR needs to be scrutinized and restricted to selected risk groups. What is known: • Screening of patients with ureteropelvic junction obstruction (UPJO) for vesicoureteral reflux (VUR) is recommended based on a small number of repeatedly cited studies. • The lack of conclusive evidence results in different treatment strategies and leads to difficulties when communicating diagnoses and treatment options to parents. What is new: • A robust prevalence for VUR in children with UPJO based on all published evidence and the resulting number needed to screen are given for decision-making in daily clinical practice. • The results may be a precursor for implementation into guidelines.
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http://dx.doi.org/10.1007/s00431-016-2818-3DOI Listing
January 2017

Balancing competing needs in kidney transplantation: does an allocation system prioritizing children affect the renal transplant function?

Transpl Int 2017 Jan 2;30(1):68-75. Epub 2016 Nov 2.

University Children's Hospital Zurich, Zurich, Switzerland.

Children often merit priority in access to deceased donor kidneys by organ-sharing organizations. We report the impact of the new Swiss Organ Allocation System (SOAS) introduced in 2007, offering all kidney allografts from deceased donors <60 years preferentially to children. The retrospective cohort study included all paediatric transplant patients (<20 years of age) before (n = 19) and after (n = 32) the new SOAS (from 2001 to 2014). Estimated glomerular filtration rate (eGFR), urine protein-to-creatinine ratio (UPC), need for antihypertensive medication, waiting times to kidney transplantation (KTX), number of pre-emptive transplantations and rejections, and the proportion of living donor transplants were considered as outcome parameters. Patients after the new SOAS had significantly better eGFRs 2 years after KTX (Mean Difference, MD = 25.7 ml/min/1.73 m , P = 0.025), lower UPC ratios (Median Difference, MeD = -14.5 g/mol, P = 0.004), decreased waiting times to KTX (MeD = -97 days, P = 0.021) and a higher proportion of pre-emptive transplantations (Odds Ratio = 9.4, 95% CI = 1.1-80.3, P = 0.018), while the need for antihypertensive medication, number of rejections and living donor transplantations remained stable. The new SOAS is associated with improved short-term clinical outcomes and more rapid access to KTX. Despite lacking long-term research, the study results should encourage other policy makers to adopt the SOAS approach.
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http://dx.doi.org/10.1111/tri.12874DOI Listing
January 2017

Surgery versus non-surgical management for unilateral ureteric-pelvic junction obstruction in newborns and infants less than two years of age.

Cochrane Database Syst Rev 2016 Jul 14;7:CD010716. Epub 2016 Jul 14.

Pediatric Nephrology, Children's Hospital Tuebingen, Hoppe-Seyler-Strasse 1, Tuebingen, Germany, 72076.

Background: Unilateral ureteric-pelvic junction obstruction (UPJO) is the most common cause of obstructive uropathy and may lead to renal impairment and loss of renal function. The current diagnostic approach with renal imaging cannot reliably determine which newborns and infants less than two years of age have a significant obstruction and are at risk for permanent kidney damage. There is therefore no consensus on optimal therapeutic management of unilateral UPJO.

Objectives: To assess the effects of surgical versus non-surgical treatment options for newborns and infants less than two years of age with unilateral UPJO.

Search Methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (Issue 6, 2016), MEDLINE/Ovid, and EMBASE/Ovid databases from their inception to 13 June 2016. We searched the reference lists of potentially relevant studies without using any language restriction. We also searched the following trial registers for relevant registered studies: www.clinicaltrials.gov/; ISRCTN registry (controlled-trials.com/); www.trialscentral.org/; apps.who.int/trialsearch/; www.drks.de/; and www.anzctr.org.au/trialSearch.aspx.

Selection Criteria: We selected randomised and quasi-randomised controlled trials comparing surgical with non-surgical interventions for the treatment of unilateral UPJO.

Data Collection And Analysis: Two review authors independently assessed study eligibility and risk of bias of included studies and extracted data. In case of disagreements we consulted a third review author. The data reported in the two included studies did not allow us to perform a meta-analysis.

Main Results: We found only two studies at high risk of bias that were eligible for inclusion in this review. The total sample size, including both trials, was small (n = 107 participants less than six months of age from the UK and USA), and not all prespecified outcome measures were assessed. Reported measures only accounted for the short-term follow-ups. The mean split renal function was not statistically different between the surgical and non-surgical group at the six-month or one-year time point (very low-quality evidence). The surgical group showed a significantly less obstructed drainage pattern and a lower urinary tract dilatation than the non-surgical group (very low-quality evidence). Transfer from the non-surgical group to the surgical group was reported for about one out of five participants. Split renal function after secondary surgical intervention was reported with variable results, but most of the participants reverted to pre-deteriorated values. The studies either provided no or insufficient data on the following outcome measures: postoperative complications, UPJO-associated clinical symptoms, costs of interventions, radiation exposure, quality of life, and adverse effects.

Authors' Conclusions: We found limited evidence assessing the benefits and harms of surgical compared to non-surgical treatment options for newborns and infants less than two years of age with unilateral UPJO. The majority of participants in the non-surgical treatment group did not experience any significant deterioration of split renal function, and only about 20% of them underwent secondary surgical intervention, with minor risk of permanent deteriorated split renal function. The study follow-up period was too short to assess the long-term effects on split renal function in both treatment groups. We need further randomised controlled trials with sufficient statistical power and an adequate follow-up period to determine the optimal therapy for newborns and infants less than two years of age with unilateral UPJO.
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http://dx.doi.org/10.1002/14651858.CD010716.pub2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6457949PMC
July 2016

Outcome after prenatal diagnosis of congenital anomalies of the kidney and urinary tract.

Eur J Pediatr 2016 May 25;175(5):667-76. Epub 2016 Jan 25.

University Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032, Zurich, Switzerland.

Unlabelled: Congenital anomalies of the kidney and urinary tract are common findings on fetal ultrasound. The aim of this prospective observational study was to describe outcome and risk factors in 115 patients born 1995-2001. All prenatally diagnosed children were stratified into low- and high-risk group and followed postnatally clinically and by imaging at defined endpoints. Risk factors were evaluated using odds ratios. Neonatal diagnosis included pelvi-ureteric junction obstruction (n = 33), vesicoureteral reflux (n = 27), solitary mild pelvic dilatation (postnatal anteroposterior diameter 5-10 mm; n = 25), and further diagnosis as primary obstructive megaureter, unilateral multicystic dysplastic kidney, renal dysplasia and posterior urethral valves. In 38 children with prenatal isolated hydronephrosis, ultrasound normalized at median age of 1.2 years (range 0.1-9). Surgery was performed in 34 children at median age of 0.4 years (0.1-10.8). Persistent renal anomalies without surgery were present in 43 children and followed in 36 for median time of 16 years (12.2-18). Oligohydramnios and postnatal bilateral anomalies were significantly associated with surgery and impaired renal function.

Conclusion: The majority of children had a favourable postnatal outcome, in particular children with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis. Oligohydramnios and postnatal bilateral anomalies were risk factors for non-favourable outcome.

What Is Known: • In congenital anomalies of the kidney and urinary tract significantly poorer outcome is known in patients with bilateral renal hypoplasia or solitary kidney associated with posterior urethral valves. • Other factors as proteinuria and vesicoureteral reflux were associated with a higher risk of progression to chronic renal failure in these patients. What is New: • Unlike other studies giving us above-mentioned information, we included all patients with any kind of prenatally diagnosed congenital anomalies of the kidney and urinary tract. Our study shows long-term follow up (median 16 years, range 12.2-18 years), especially in patients not needing surgery, but with persistent anomalies. • During postnatal long-term follow up (median 2.2 years, range 0.1-18 years) one third each showed normalization, need of surgery or persistence of anomalies without need of surgery. Our study revealed a good prognosis in the majority of these children, in particular with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis, and revealed oligohydramnios and postnatal bilateral anomalies as risk factors for a non-favourable outcome, defined as need of surgery, persistent anomalies with impaired renal function, end stage renal failure or death.
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http://dx.doi.org/10.1007/s00431-015-2687-1DOI Listing
May 2016

Extracorporeal photopheresis versus alternative treatment for chronic graft-versus-host disease after haematopoietic stem cell transplantation in paediatric patients.

Cochrane Database Syst Rev 2015 Dec 15(12):CD009898. Epub 2015 Dec 15.

Pediatric Nephrology, University Children's Hospital, Steinwiesstrasse 75, Zurich, Switzerland, 8032.

Background: Chronic graft-versus-host disease (GvHD) is a major cause of morbidity and mortality after haematopoietic stem cell transplantation occurring in 6% to 65% of the recipients. Currently, the therapeutic mainstay for chronic GvHD are corticosteroids that are frequently combined with other immunosuppressive agents in people with steroid-refractory manifestations. There is no established standard treatment for steroid-refractory chronic GvHD. The therapeutic options for these patients include extracorporeal photopheresis (ECP), an immunomodulatory treatment that involves ex vivo collection of mononuclear cells from peripheral blood, exposure to the photoactive agent 8-methoxypsoralen, ultraviolet radiation and re-infusion of the processed cell product. The mechanisms of action of ECP are not completely understood. This is an updated version of a Cochrane review first published in 2014.

Objectives: To evaluate the effectiveness and safety of ECP for the management of chronic GvHD in children and adolescents after haematopoietic stem cell transplantation.

Search Methods: We searched the Cochrane Register of Controlled Trials (CENTRAL) (Issue 9, 2015), MEDLINE and EMBASE databases from their inception to 23 September 2015. We searched the reference lists of potentially relevant studies without any language restriction. We searched eight trial registers and five conference proceedings on 29 September 2015.

Selection Criteria: Randomised controlled trials (RCTs) comparing ECP with or without alternative treatment versus alternative treatment alone in paediatric patients with chronic GvHD after haematopoietic stem cell transplantation.

Data Collection And Analysis: Two review authors independently performed the study selection. We resolved disagreements in the selection of trials by consultation with a third review author.

Main Results: No additional studies were identified in this 2015 review update, in total leading to no studies meeting the criteria for inclusion in this review.

Authors' Conclusions: The efficacy of ECP in the treatment of chronic GvHD in paediatric patients after haematopoietic stem cell transplantation based on RCTs cannot be evaluated since the original version of this review and the first review update found no RCTs. Current recommendations are based on retrospective or observational studies only. Thus, ideally, ECP should be applied in the context of controlled trials only. However, performing RCTs in this patient population will be challenging due to the limited number of patients, the variable disease presentation and the lack of well-defined response criteria. International collaboration, multicentre trials and appropriate funding for such trials will be needed. If treatment decisions based on clinical data are made in favour of ECP, patients should be carefully monitored for beneficial and harmful effects. In addition, efforts should be made to share this information with other clinicians, for example by setting up registries for paediatric patients that are treated with ECP.
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http://dx.doi.org/10.1002/14651858.CD009898.pub3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7093760PMC
December 2015

Extracorporeal photopheresis versus standard treatment for acute graft-versus-host disease after haematopoietic stem cell transplantation in paediatric patients.

Cochrane Database Syst Rev 2015 Dec 15(12):CD009759. Epub 2015 Dec 15.

Pediatric Nephrology, University Children's Hospital, Steinwiesstrasse 75, Zurich, Switzerland, 8032.

Background: Acute graft-versus-host disease (aGvHD) is a major cause of morbidity and mortality after haematopoietic stem cell transplantation (HSCT) occurring in 8% to 59% of the recipients. Currently, the therapeutic mainstay for aGvHD is corticosteroids. However, there is no established standard treatment for steroid-refractory aGvHD. Extracorporeal photopheresis (ECP) is a type of immunomodulatory method amongst different therapeutic options that involves ex vivo collection of peripheral mononuclear cells, exposure to the photoactive agent 8-methoxypsoralen and ultraviolet-A radiation, and re-infusion of these treated blood cells to the patient. The mechanisms of action of ECP are not completely understood. This is an updated version of a Cochrane review first published in 2014.

Objectives: To evaluate the effectiveness and safety of ECP for the management of aGvHD in children and adolescents after HSCT.

Search Methods: We searched the Cochrane Register of Controlled Trials (CENTRAL) (Issue 9, 2015), MEDLINE (PubMed) and EMBASE (Ovid) databases from their inception to 23 September 2015. We searched the reference lists of potentially relevant studies without any language restrictions. We searched eight trial registers and four conference proceedings on 29 September 2015.

Selection Criteria: Randomised controlled trials (RCTs) comparing ECP with or without standard treatment versus standard treatment alone in paediatric patients with aGvHD after HSCT.

Data Collection And Analysis: Two review authors independently performed the study selection. We resolved disagreement in the selection of trials by consultation with a third review author.

Main Results: We identified no additional studies in the 2015 review update, in total leading to no studies meeting the criteria for inclusion in this review.

Authors' Conclusions: The efficacy of ECP in the treatment of aGvHD in paediatric patients after HSCT is unknown and its use should be restricted within the context of RCTs. Such studies should address a comparison of ECP alone or in combination with standard treatment versus standard treatment alone. The 2015 review update brought about no additions to these conclusions.
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http://dx.doi.org/10.1002/14651858.CD009759.pub3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7093896PMC
December 2015

Standardized multilevel transition program: Does it affect renal transplant outcome?

Pediatr Transplant 2015 Nov 11;19(7):691-7. Epub 2015 Aug 11.

Nephrology Unit, University Children's Hospital Zurich, Zurich, Switzerland.

The transfer of renal transplant patients from pediatric to adult care is a crucial step with a high risk of subsequent graft loss. Therefore, the transition should be a thoroughly planned, well-designed and multidisciplinary process focused on the individual patient. Our pediatric nephrology department introduced a structured step-by-step transition program supported by a multidisciplinary team of health professionals. The purpose of our study was to determine the effects of the transition program on eGFR and number of ARs in comparison to a group without a transition program at one and three yr after transfer. We conducted a single-center retrospective cohort study of renal transplant patients prior to and after the introduction of the transition program. Multiple regression analysis revealed a significantly lower decline of eGFR in the group with transition program (-11.3 ± 44 mL/min/1.73 m(2) ) compared to the group without transition program (-28.4 ± 33 mL/min/1.73 m(2) ) at three yr after transfer. The number of AR episodes significantly decreased from 34.6% in the group without transition program to 9.1% in the group with transition program. The standardized multilevel transition program seems to have significant positive effects on eGFR and number of AR episodes in renal transplant patients.
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http://dx.doi.org/10.1111/petr.12570DOI Listing
November 2015

Cushing syndrome after bilateral lensectomy.

Eur J Pediatr 2015 Mar 24;174(3):399-401. Epub 2014 Dec 24.

General Paediatrics, University Children's Hospital, 8032, Zurich, Switzerland,

Unlabelled: Iatrogenic Cushing syndrome induced by oral and parenteral corticosteroid administration is a well-known complication, and necessary precautions have to be taken. Cushing syndrome, however, following treatment with glucocorticoid-containing eye drops is a very rare complication. To the best of our knowledge, there have been only four reported cases in the literature. Herein, we present an infant boy who developed Cushing syndrome after receiving dexamethasone-containing eye drops after bilateral cataract extraction to prevent postoperative inflammatory complications. At the age of 5 months, after approx. 3 months of dexamethasone therapy, the patient presented with cushingoid facies, nephrocalcinosis and failure to grow. Iatrogenic Cushing syndrome was diagnosed and dexamethasone-containing eye drops were reduced and eventually stopped. Follow-up examinations revealed catch-up growth.

Conclusion: Ocularly administered corticosteroids may have substantial systemic side effects in infants.
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http://dx.doi.org/10.1007/s00431-014-2477-1DOI Listing
March 2015

Extracorporeal photopheresis versus alternative treatment for chronic graft-versus-host disease after haematopoietic stem cell transplantation in paediatric patients.

Cochrane Database Syst Rev 2014 Feb 25(2):CD009898. Epub 2014 Feb 25.

Department of Pediatrics, University of Tuebingen, Hoppe-Seyler-Strasse 1, Tübingen, Germany, 72076.

Background: Chronic graft-versus-host disease (GvHD) is a major cause of morbidity and mortality after haematopoietic stem cell transplantation occurring in 6% to 65% of the recipients. Currently, the therapeutic mainstay for chronic GvHD are corticosteroids that are frequently combined with other immunosuppressive agents in people with steroid-refractory manifestations. There is no established standard treatment for steroid-refractory chronic GvHD. The therapeutic options in these people include extracorporeal photopheresis (ECP), an immunomodulatory treatment that involves ex vivo collection of mononuclear cells from peripheral blood, exposure to the photoactive agent 8-methoxypsoralen, ultraviolet radiation and re-infusion of the processed cell product. The mechanisms of action of ECP are not completely understood.

Objectives: To evaluate the effectiveness and safety of ECP for the management of chronic GvHD in children and adolescents after haematopoietic stem cell transplantation.

Search Methods: We searched the Cochrane Central Register of Controlled Trials (Issue 9, 2012), MEDLINE and EMBASE databases from their inception to 12 September 2012. We searched the reference lists of potentially relevant studies without any language restriction. We searched eight trial registers and five conference proceedings. We also contacted experts in the field.

Selection Criteria: Randomised controlled trials (RCTs) comparing ECP with or without alternative treatment versus alternative treatment alone in paediatric patients with chronic GvHD after haematopoietic stem cell transplantation.

Data Collection And Analysis: Two review authors independently performed the study selection. We resolved disagreements in the selection of trials by consultation with a third review author.

Main Results: We found no studies meeting the criteria for inclusion in this review.

Authors' Conclusions: The efficacy of ECP in the treatment of chronic GvHD in paediatric patients after haematopoietic stem cell transplantation based on RCTs can currently not be evaluated since we have found no such studies. Current recommendations are based on retrospective or observational studies only. Thus, ideally, ECP should be applied in the context of controlled trials only. However, performing RCTs in this patient population will be challenging due to the limited number of patients, the variable disease presentation and the lack of well-defined response criteria. International collaboration, multicentre trials and appropriate funding for such trials will be needed. If treatment decisions based on clinical grounds in favour of ECP are made, people should be carefully monitored for beneficial and harmful effects and efforts should be made to share this information with other clinicians, for example by setting up registries for paediatric patients that are treated with ECP.
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http://dx.doi.org/10.1002/14651858.CD009898.pub2DOI Listing
February 2014

Extracorporeal photopheresis versus standard treatment for acute graft-versus-host disease after haematopoietic stem cell transplantation in paediatric patients.

Cochrane Database Syst Rev 2014 Feb 25(2):CD009759. Epub 2014 Feb 25.

Pediatric Nephrology, University Children's Hospital, Steinwiesstrasse 75, Zurich, Switzerland, 8032.

Background: Acute graft-versus host disease (aGvHD) is a major cause of morbidity and mortality after haematopoietic stem cell transplantation (HSCT) occurring in 8% to 59% of the recipients. Currently, the therapeutic mainstay for aGvHD is corticosteroids. However, there is no established standard treatment for steroid-refractory aGvHD. Extracorporeal photopheresis (ECP) is a type of immunomodulatory method amongst different therapeutic options that involves ex vivo collection of peripheral mononuclear cells, exposure to the photoactive agent 8-methoxypsoralen and ultraviolet-A radiation, and re-infusion of these treated blood cells to the patient. The mechanisms of action of ECP are not completely understood

Objectives: To evaluate the effectiveness and safety of ECP for the management of aGvHD in children and adolescents after HSCT.

Search Methods: We searched the Cochrane Central Register of Controlled Trials (Issue 9, 2012), MEDLINE/PubMed and EMBASE (Ovid) databases from their inception to 12 September 2012. We searched the reference lists of potentially relevant studies without any language restriction. We searched eight trial registers and four conference proceedings. We also contacted an expert in the field to request information on unpublished study that involves ECP in aGvHD after HSCT.

Selection Criteria: Randomised controlled trials (RCTs) comparing ECP with or without standard treatment versus standard treatment alone in paediatric patients with aGvHD after HSCT.

Data Collection And Analysis: Two review authors independently performed the study selection. We resolved disagreement in the selection of trials by consultation with a third review author.

Main Results: We found no studies meeting the criteria for inclusion in this review.

Authors' Conclusions: The efficacy of ECP in the treatment of aGvHD in paediatric patients after HSCT is unknown and its use should be restricted within the context of RCTs. Such studies should address a comparison of ECP alone or in combination with standard treatment versus standard treatment alone. 
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February 2014

Renal ultrasound volume in children with primary vesicoureteral reflux allows functional assessment.

J Pediatr Urol 2013 Dec 29;9(6 Pt B):1077-83. Epub 2013 Apr 29.

University of Tuebingen, Department of Pediatrics, Hoppe-Seyler-Strasse 1, 72076 Tübingen, Germany. Electronic address:

Objective: Renal scintigraphy represents the current diagnostic standard to assess split kidney function. We tested the hypothesis that the relative renal volume assessed by ultrasound provides an equally reliable but less invasive tool for assessment of kidney function as compared to renal scintigraphy in patients with primary vesicoureteral reflux.

Methods: Renal ultrasound and renal scintigraphy were performed in 85 patients (median age 4.5 years, range 0.25-7.7) and repeated in 74 patients after 2-13 months (mean 7) of the primary investigation. Renal size was measured by ultrasound, and relative renal volume was calculated for each kidney by using the formula of a prolate ellipsoid. Renal function was estimated for each side (split renal function) by scintigraphy with (99m)Tc MAG3.

Results: The mean difference between relative renal volume measured by ultrasound and split renal function determined by renal scintigraphy was 2.8% (standard deviation ± 4.1%; 95% confidence interval 10.8/-5.2%). There was a statistically significant correlation between relative renal volume estimated by ultrasound and split renal function estimated by renal scintigraphy at first examination (r = 0.98; p < 0.001) and at follow-up (r = 0.91; p < 0.001).

Conclusion: We conclude that ultrasound measurement of relative renal volume is capable of assessing split renal function in children with primary vesicoureteral reflux and, thus, should be considered instead of the more invasive MAG3 scintigraphy.
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http://dx.doi.org/10.1016/j.jpurol.2013.03.007DOI Listing
December 2013

Prophylactic eculizumab prior to kidney transplantation for atypical hemolytic uremic syndrome.

Pediatr Nephrol 2011 Aug 10;26(8):1325-9. Epub 2011 May 10.

University Children's Hospital Tuebingen, Tuebingen, Germany.

Atypical hemolytic uremic syndrome (aHUS) in childhood is a rare disease associated with high morbidity and mortality. Most cases progress to end-stage renal failure. In approximately 50% of affected patients, mutations in genes encoding complement proteins are causative of the impairment in the regulation of the complement alternative pathway. This leads to deficient host cell protection and inappropriate complement activation on platelets and endothelial cells, particularly in the kidneys. Complement factor H (FH) heterozygosity induces unregulated activation of the membrane attack complex (MAC) C5b-9. Present therapeutic strategies for aHUS include lifelong plasmapheresis and renal dialysis. Unfortunately, kidney transplantation is frequently an unsatisfactory intervention due to the high rate of post-transplantation HUS recurrence, particularly in patients with FH mutation. Combined liver-kidney transplantation is also associated with poor outcome, mostly as a result of premature liver failure secondary to uncontrolled complement activation. Eculizumab is a complement C5 antibody that inhibits complement factor 5a (C5a) and the formation of the MAC. Thus, this antibody may be a promising new agent for patients with an aHUS undergoing kidney transplantation. We present the first case of a young patient with aHUS who received eculizumab as prophylactic treatment prior to a successful kidney transplantation.
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http://dx.doi.org/10.1007/s00467-011-1879-9DOI Listing
August 2011

Exercise capacity of a contemporary cohort of children with hypoplastic left heart syndrome after staged palliation.

Eur J Cardiothorac Surg 2009 Dec 29;36(6):980-5. Epub 2009 Jul 29.

Department of Paediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Arnold-Heller-Strasse 3, Haus 9, 24105 Kiel, Germany.

Objective: Outcome of staged palliation for hypoplastic left heart syndrome has improved over the past decades. However, only little is known about the exercise capacity of children with palliated hypoplastic left heart syndrome where a systemic right ventricle supports the systemic circulation. The aim of the study was to assess exercise capacity in a contemporary cohort of children with hypoplastic left heart syndrome palliated in a single centre according to a uniform surgical strategy.

Methods: Standardised cardiopulmonary exercise testing on a treadmill was performed in 46 consecutive hypoplastic left heart patients (median age: 6.0 (4.1-11.4) years). All but one patient reached the anaerobic threshold. Exercise data were compared to normal values obtained with a similar exercise protocol in a large cohort of paediatric volunteers.

Results: Oxygen uptake at anaerobic threshold (26.9+/-6.0 ml kg(-1)min(-1); 74.5+/-18.2% of predicted) and maximal oxygen uptake (31.0+/-6.8 ml kg(-1)min(-1); 60.8+/-15.0% of predicted) were significantly reduced compared with controls (P<0.0001 for both). The limitation in exercise capacity was due to an impaired rise in heart rate (158+/-23 bpm; 79.7+/-11.5% of predicted; P<0.0001) and oxygen pulse (4.5+/-1.6 ml per beat; 85.5+/-22.0% of predicted; P<0.0001). Furthermore, respiration during exercise was inefficient with an elevated respiratory rate and reduced maximal tidal volume and minute ventilation at maximal exercise.

Conclusions: The exercise capacity of children with hypoplastic left heart syndrome is markedly reduced. Limitations in heart rate increase and stroke volume augmentation are the major contributors to this. An abnormal ventilatory response to exercise also adds to their limitation in exercise tolerance. However, the degree of physical disability does not justify discouraging these patients from school and leisure sports.
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http://dx.doi.org/10.1016/j.ejcts.2009.06.029DOI Listing
December 2009