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The field of pediatrics in Europe is characterized by the diversities, variations, and heterogeneities of child health care services provided in 53 European countries with more than 200 million children below 18 years of age. Managing the health care of infants, children, and adolescents in Europe requires balancing clinical aims, research findings, and socioeconomic goals within a typical environment characterized by cultural and economic complexity and large disparity in availability, affordability, and accessibility of pediatric care. Since its foundation in 1976, the European Paediatric Association-Union of National European Paediatric Societies and Associations has worked to improve both medical care of all children and cooperation of their caretakers in Europe. Such a report has been conceived in the strong belief that broadening of the intellectual basis of the European Paediatric Association-Union of National European Paediatric Societies and Associations and creating a multidisciplinary society will be necessary to reduce fragmentation of pediatrics and tackle the legal, economic, and organizational challenges of child health care in Europe.

Background There is limited data on the use and functionality level of electronic health records (EHRs) supporting primary child health care in Europe. Our objective was to determine European primary child healthcare providers' use of EHRs, and functionality level of the systems used. Methods European primary care paediatricians, paediatric subspecialists and family doctors were invited by European Academy of Paediatrics Research in Ambulatory Setting Network (EAPRASnet) country coordinators to complete a web-based survey on the use of EHRs and the systems' functionalities. Binomial logistic analysis has been used to evaluate the effect of specialty and type of practice on the use of EHRs. Results The survey was completed by 679 child primary healthcare providers (response rate 53%). Five hundred and fifty four responses coming from 10 predominant countries were taken for further analysis. EHR use by respondents varied widely between countries, all electronic type use ranging between 7% and 97%. There was no significant difference in EHR use between group practice and solo practitioners, or between family doctors and primary care paediatricians. History and physical examination can be properly recorded by respondents in most countries. However, growth chart plotting capacity in some countries ranges between 22% and 50%. Vaccination recording capacity varies between 50% and 100%, and data exchange capacity with immunization databases is mostly limited, ranging between 0% and 54%. Conclusions There is marked heterogeneity in the use and functionalities of EHRs used among child primary child healthcare providers in Europe. More importantly, lack of critical paediatric supportive functionalities like growth tracking and vaccination status has been documented in some countries. There is a need to explore the reasons for these findings, and to develop a cross European paediatric EHR standards.

Previous articles published in the European Paediatric Association (EPA) pages have discussed the importance of children's well-being and its role in different areas of children's health, such as well-child care and child-friendly health care. The goal of this commentary is to raise pediatricians' awareness on the risks of children's overexposure to digital media. It is important to protect the psychological and emotional health of children from uncontrolled external influences to preserve their well-being. We also emphasize the importance of alerting parents and tutors to the risks related to the unrefrained use of the Internet.

Hoch spezialisierte Kindermedizin dient der Verbesserung von kindgerechter Gesundheitsversorgung bei Kindern mit komplizierten und seltenen Erkrankungen. Die Erstellung von Modellen zur Strukturierung und Verteilung von padiatrischen Kompetenzzentren ist in Europa weder durch nationale noch durch EU-Gesetzesregelungen zufriedenstellend koordiniert. Kompetenzzentren haben sich in allen europaischen Landern vorwiegend spontan entwickelt und berucksichtigen in der Regel mehr individuelle Bedurfnisse als allgemeingesellschaftliche Erfordernisse. Dabei hat sich nicht die Konzeption von Infrastruktur und Behandlungsprozessen individueller Kompetenzzentren als Problem erwiesen, sondern deren Koordinierung. Folglich scheint das Hauptproblem darin zu liegen, dass die Etablierung von kinderfreundlichen, uberregional ausgewogenen und okonomisch tragbaren Modellen aufgrund einseitiger Sichtweisen behindert wurde. Diese allzu menschlichen Hindernisse konnen wahrscheinlich nur durch eine Kombination von „bottom up“ und „top down“ Entscheidungen beseitigt werden, d. h. subjektive, objektive, interaktionelle und systemische Sichtweisen mussen nicht nur starker berucksichtigt werden, sondern die Organisation von Kompetenzzentren setzt eine dem Ganzen gegenuber verantwortliche soziookonomische Haltung aller Meinungsmacher voraus, deren Kooperation und entscheidenden Willen zum zugigen Konsens.

Objective The Wuerzburger Psychologische Kurz-Diagnostik (WUEP-KD) is a short screening battery for cognitive deficits in children with brain tumour. We report on its psychometric quality and testing efficiency. Material and methods WUEP-KD was founded on Cattell-Horn-Carroll (CHC) framework of cognitive abilities. We assessed the construct validity of the short battery by conducting factor analysis and the concurrent validity by multiple linear regressions with Kaufman Assessment Battery for Children (K-ABC). The concurrent validity was explored by multiple linear regressions with Wechsler Intelligence Scale for Children (WISC). The discriminant validity was examined by a reanalysis of harmful effects of brain tumour treatments in a medulloblastoma cohort. Results The construct validity assessment revealed three neuropsychological domains: cognitive operations, executive abilities, and psychomotor abilities. The retest reliabilities for individual testing and the convergent coefficients of the WUEP-KD with K-ABC and WISC yielded satisfactory results. The cognitive effects of different treatment modalities in the medulloblastoma cohort matched exactly previously reported data on the decline of general intelligence scores and delivered the details for the harmful effects. An in-depth analysis based on Hedges' g effect sizes confirmed specific harmful late effects on all abilities of cognitive operations, on the executive ability of perceptual speed and on psychomotor ability of movement steadiness. Conclusion WUEP-KD is a valid and efficient short test instrument, which may be especially useful in larger cohorts, multicenter settings or if patients do not tolerate longer tests. Due to its foundation on the CHC framework, our findings provide a rationale to create a common data set along with scores from other factor-based tests in international studies.

The status of the pediatric workforce in child healthcare has been discussed for several years, debating oversupply and inefficient services. These debates are influenced by the widely accepted assumption that the future child population will require more pediatric and more general healthcare. The ratio of 4000 children per pediatrician that was regarded to be appropriate in the 1980s has decreased to 1400:1 in recent years.This ongoing trend is supported by our findings from 2013 to 2018 in 16 European countries, irrespective of the underlying child healthcare system.

Die Digitalisierung des Gesundheitswesens schreitet voran und ermoglicht neue Denkweisen in der Kinderheilkunde. In der Vergangenheit haben Kinderarzte in den primaren und den sekundaren Versorgungsstufen einen geringen aktiven Anteil an padiatrischer Forschung gehabt. In Deutschland sind Lucken der padiatrischen Versorgungsforschung unubersehbar. Onlineforschung hat die traditionellen Forschungsmethoden erweitert. Neben neuen methodischen Moglichkeiten zu Datenspeicherung, -sicherheit, -sammlung und -design werden auch neue ethische und rechtliche Fragen aufgeworfen, die sich mit Datenanonymitat, -vertraulichkeit und -uberflutung befassen. Die digitale Dokumentation des Messbaren fordert v. a. die Klarung der Frage, welche Daten fur die Verbesserung der Gesundheitsversorgung sinnvoll sind. Die Digitalisierung erleichtert die Zusammenarbeit aller Padiater auf dem Gebiet der Forschung. Sie kann dazu beitragen, die Fragmentierung padiatrischer Forschung zu verringern und komplexe Probleme der Kindergesundheit durch Systemdenken zu losen.

Renal replacement therapy has become available for the majority of patients suffering from severe congenital chronic kidney disease (CKD). Data on the long-term neurocognitive outcome and the impact of early kidney transplantation (KTx) in this setting is unclear. Neurocognitive outcomes in 15 patients (11 male) with isolated congenital CKD (stage 3-5) requiring KTx at a mean age of 2.8 ± 1.3 were assessed at a mean age of 8.3 ± 1.4 years. Patients underwent neurological examination and testing for neuromotor and neurocognitive function using three independent tests. Pre-emptive KTx was performed in six patients, and nine patients were dialyzed prior to KTx for a mean period of 11.1 ± 8.6 months. Neuromotor function was abnormal in 8/15 patients. HAWIK-III showed a global intelligence quotient (IQ) of 93.5 ± 11.4 (P = 0.05) due to a significantly reduced performance IQ of 89.1 ± 11.3 (P 0.01). In three patients, the global IQ was clinically significantly reduced by1 SD to85. In patients with neuromotor dysfunction, performance IQ was lower than in patients with normal neuromotor function (83.8 ± 10.2 vs. 96.2 ± 9.0, P = 0.04). Time on dialysis was inversely correlated to verbal IQ (r = 0.78, P = 0.02). Pre-emptive KTx and duration of dialysis treatment3 months was associated with superior neurocognitive outcome. Early (pre-emptive) KTx results in superior long-term neurocognitive outcome in children with severe congenital CKD.

This study reports the geographical incidence of successful pregnancies in women on renal replacement therapy (RRT) and related information on gestation and clinical status of newborns. The impact of successful pregnancy on graft function was assessed by means of a retrospective case-control study. Since 1977 special questionnaires have been sent to each dialysis and transplant centre which reported babies born to mothers on RRT on the yearly centre questionnaire. After 10 years of data collection, a total of 490 pregnancies and 500 babies were available for analysis. A percentage of 88.4 of the babies were born to mothers with a functioning graft, 11.2% to mothers on chronic haemodialysis, and the remaining 0.4% to mothers on CAPD. Almost 50% of all successful pregnancies werereported from the UK. The number of successful pregnancies increased steadily and in parallel with the increasing number of females of childbearing age with a functioning renal transplant. The majority of mothers delivered at age 24-32. For transplanted mothers delivery occurred most commonly during the 3rd and 4th year after successful transplantation. In approximately 85% of cases the duration of pregnancy was shorter than the lower 10th percentile of normal. Birthweight was reduced in accordance with gestational age. Newborn mortality was 1.8%. Fifty-three mothers with a successful pregnancy in 1984-1987 were computer matched with controls according to a number of criteria. The serum creatinine concentration recorded in coded form at the end of each year on the individual EDTA patient questionnaire was used to assess changes in graft function. In 94% of these cases the serum creatinine, recorded 0-11 months before delivery, did not exceed 160 umol/1. Graft function deteriorated in 18% ofmothers as compared to 24% of controls. Twentyfour to 36 months postpartum, changes of serum creatinine were similar in test cases and controls, suggesting that a successful pregnancy does not adversely affect graft function if this was stable and well preserved at the time of conception

This report summarises the outcome of 90 combined kidney/pancreatic grafts performed in Europe in 1986. Data for the combined kidney/pancreas grafts were obtained by a special questionnaire. The one-year patient and kidney graft survival is compared to the results of a group of 389 patients with diabetic nephropathy on the EDTA Registry data file who received kidney grafts alone. The recipients of combined kidney-pancreas grafts were younger, whereas a greater proportion of males received kidney graft alone. Patient survival at one year after transplantation was similar: 89% in recipients of combined transplants compared to 90% in recipients of kidney grafts alone. Kidney graft survival was 78% at one year for recipients of combined grafts versus 76%. It is concluded that pancreas transplantation has little effect on the fate of concomitant kidney grafts. The procedure should - in experienced hands and in selected patients - be almost as safe as kidney grafting alone

Partizipation dient der Verbesserung der Protektion und Pravention von Krankheit und der Provision einer kindgerechten Gesundheitsversorgung. Die Umsetzung des salutogenen Partizipationsmodells beruht auf internationalen Gesetzesregelungen, die zusammen mit anderen, nationalen Regelungen Rahmenbedingungen schaffen wollen. Partizipation beruht auf bioethischen Grundlagen, die subjektive, objektive, interaktionelle und systemische Sichtweisen berucksichtigen. Partizipation setzt eine sozioethische Haltung der Behandler und ihre Kompetenz in der Kommunikation mit Kindern sowie die Entwicklung von Gesundheitskompetenz bei Kindern voraus. Partizipation von europaischen Kindern in der Medizin hat sich trotz der gesetzlichen Grundlagen bisher nicht ausreichend durchgesetzt. Es ist offensichtlich kein isoliert nationales Problem, sondern eine internationale Herausforderung. Unterschiedliche Lander, Berufsgruppen und Generationen haben verschiedene Vorstellungen von Partizipation. Die Implementierung von Partizipation in der Kindermedizin erfordert nicht nur adaquate Gesetzesregelungen, sondern eine professionelle und soziokulturelle Kompetenz – also Grundhaltung – aller Behandler sowie „empowerment“ von Kindern, um im Rahmen der medizinischen Kooperation einen Konsensus und bessere Heilungsergebnisse zu erzielen.

The Council of Europe was founded in 1949 to defend human rights, parliamentary democracy, and the rule of the law in order to promote a European identity based on shared values across different cultures. It started as 10 nations, but now covers 47 nations, which collectively have a population of 200 million children. Although the rights of children are well established in the United Nations Convention on the Rights of the Child, Article 24 specifically mentions two elements—the right to the highest attainable standard of health (“the right to health”) and the right of access to health care (“the right to health care”). The challenge has been to translate these principles into practice by developing a comprehensive and consistent model that informs and influences policy making, planning and the delivery, and improvement of services. Child-Friendly Health Care is the third in a series of reports (which also includes Child-Friendly Social Care, Children’s Participation, and Child-Friendly Justice 1-3 ) that form part of the Council of Europe strategy entitled “Building a Europe for and with Children.” 4 The program’s main

Es stellt sich die Frage, ob zukunftige Gesundheitsreformen die Herausforderungen demografischer und epidemiologischer Veranderungen in der Kinderheilkunde bewaltigen konnen, ohne dabei die Qualitat der Versorgung zu verschlechtern. Aus Sicht der Kinderarzte sind erhebliche Anstrengungen zu treffen, um die Morbiditat und Mortalitat von Kindern in Europa weiter zu senken. Die Erstversorgungssysteme fur Kinder in den 53 europaischen Landern unterscheiden sich deutlich. Die effektive Primarversorgung ist eine Saule der Kindergesundheit. Hinsichtlich der Effektivitat unterschiedlicher Versorgungssysteme besteht jedoch kein Konsens. Ein Mehr an Versorgungsforschung vor und nach Etablierung neuer Ablaufe ist fur die Qualitatsbeurteilung verschiedener padiatrischer Versorgungssysteme unerlasslich. Die Entwicklungen der neuen Morbiditaten werden den Kinderarzt nicht uberflussig machen. Stattdessen definieren sie seinen Arbeitsbereich und -kreis neu und erhalten seine Position im Zentrum der medizinischen Versorgung von Kindern.

Background The attainment of normal growth and maturation remains a major challenge in the management of children and adolescents requiring renal replacement therapy (RRT).

The number of people that refrain from eating meat has apparently increased. This is due to a variety of factors other than economic constraints or religious concerns, which have long been reasons for such dietetic conduct. Several reasons for adopting diets excluding meat include improved health, concerns regarding animal welfare, the use of antibiotics and hormones in livestock, and the excessive exploitation of environmental resource. Consequently, the number of children whose parents decide to follow alternative diets, such as vegetarian, vegan, macrobiotic, or fruitarian, is also growing. Clinical research regarding adults adopting such diets has expanded, and their nutritional impact is now well documented. However, data on children following dietetic regimens excluding meat and the impact on their health are limited. We would like to raise the awareness among pediatricians regarding advising parents during pregnancy and for regularly caring for infants and children who grow up in families consuming atypical dietetic regimes, such as vegetarian and vegan diets.

We describe the strengths and challenges of the child health care system in Germany and also provide an outlook on future health plans, focusing on making idiosyncrasies of national health care services in Europe understandable to those pediatricians working in other countries. The aim should be to avoid those unnecessary processes in child care which, unless abandoned, may be responsible for a poor outcome of child health. Larger countries, such as Germany, have many distinct regional differences. When it comes to problem-solving strategies, pediatricians must be aware of unavoidable cultural and historic differences that may influence the outcome of care. Even when assuming unlimited financial resources, different regional priorities might result in diverging goals.

Background Nephropathic cystinosis is a rare inherited metabolic disorder leading to progressive renal failure and extra-renal comorbidity. The prognosis relies on early adherence to cysteamine treatment and symptomatic therapies. Developing nations [DiN] experience many challenges for management of cystinosis. The aim of this study was to assess the management characteristics in DiN compared with developed nations [DeN]. Methods A questionnaire was sent between April 2010 and May 2011 to 87 members of the International Pediatric Nephrology Association, in 50 countries. Results A total of 213 patients were included from 41 centres in 30 nations (109 from 17 DiN and 104 from 13 DeN). 7% of DiN patients died at a median age of 5 years whereas no death was observed in DeN. DiN patients were older at the time of diagnosis. In DiN, leukocyte cystine measurement was only available in selected cases for diagnosis but never for continuous monitoring. More patients had reached end-stage renal disease in DiN (53.2 vs. 37.9%, p = 0.03), within a shorter time of evolution (8 vs. 10 yrs., p = 0.0008). The earlier the cysteamine treatment, the better the renal outcome, since the median renal survival increased up to 16.1 [12.5−/] yrs. in patients from DeN treated before the age of 2.5 years of age (p = 0.0001). However, the renal survival was not statistically different between DeN and DiN when patients initiated cysteamine after 2.5 years of age. The number of transplantations and the time from onset of ESRD to transplantation were not different in DeN and DiN. More patients were kept under maintenance dialysis in DiN (26% vs.19%, p = 0.02); 79% of patients from DiN vs. 45% in DeN underwent peritoneal dialysis. Conclusions Major discrepancies between DiN and DeN in the management of nephropathic cystinosis remain a current concern for many patients living in countries with limited financial resources. Electronic supplementary material The online version of this article (doi:10.1186/s12882-017-0633-3) contains supplementary material, which is available to authorized users.

Western European health systems are not keeping pace with changes in child health needs. Non-communicable diseases are increasingly common causes of childhood illness and death. Countries are responding to changing needs by adapting child health services in different ways and useful insights can be gained through comparison, especially because some have better outcomes, or have made more progress, than others. Although overall child health has improved throughout Europe, wide inequities remain. Health services and social and cultural determinants contribute to differences in health outcomes. Improvement of child health and reduction of suffering are achievable goals. Development of systems more responsive to evolving child health needs is likely to necessitate reconfiguring of health services as part of a whole-systems approach to improvement of health. Chronic care services and first-contact care systems are important aspects. The Swedish and Dutch experiences of development of integrated systems emphasise the importance of supportive policies backed by adequate funding. France, the UK, Italy, and Germany offer further insights into chronic care services in different health systems. First-contact care models and the outcomes they deliver are highly variable. Comparisons between systems are challenging. Important issues emerging include the organisation of first-contact models, professional training, arrangements for provision of out-of-hours services, and task-sharing between doctors and nurses. Flexible first-contact models in which child health professionals work closely together could offer a way to balance the need to provide expertise with ready access. Strategies to improve child health and health services in Europe necessitate a whole-systems approach in three interdependent systems - practice (chronic care models, first-contact care, competency standards for child health professionals), plans (child health indicator sets, reliable systems for capture and analysis of data, scale-up of child health research, anticipation of future child health needs), and policy (translation of high-level goals into actionable policies, open and transparent accountability structures, political commitment to delivery of improvements in child health and equity throughout Europe).

The combination of psychosocial, medical, and educational care usually leads to immediate and long-term extra cost, which may pay out only decades later. This social and economic investment may be regarded by economists as being hardly affordable, especially during recession periods. However, we strongly believe that there are alternatives allowing adequate care even in times of economic turmoil. The aim of this report is to raise the awareness of the pediatric community and the public opinion toward the importance of investing in health programs offering adequate protection, prevention, and provision of care for orphan, abandoned, maltreated, and neglected children.

OBJECTIVES: High-grade vesicoureteral reflux (VUR, grade IV or V) is a risk factor for renal scarring, impaired renal function, and arterial hypertension. Voiding cystourethrography is the gold standard for detecting the severity of VUR. High-grade VUR is present in the minority of children with urinary tract infection (UTI), thus exposing the majority to invasive diagnostics that have no surgical consequence. We therefore aimed at establishing a noninvasive test to identify children with high-grade VUR. METHODS: In a case-control study, a specific urinary proteome pattern was established by capillary electrophoresis coupled to mass spectrometry in 18 patients with primary VUR grade IV or V, distinguishing these from 19 patients without VUR after UTI. This proteome pattern was independently validated in a blinded cohort of 17 patients with VUR grade IV or V and 19 patients without VUR. RESULTS: Sensitivity in detecting VUR grade IV or V in the blinded study was 88%, specificity was 79%. The test’s accuracy was independent of age, gender, and grade of VUR in the contralateral kidney. The odds ratio of suffering from VUR grade IV or V when tested positive was 28 (95% confidence interval: 4.5 to 176.0). CONCLUSIONS: This noninvasive test is ready for prospective validation in large cohorts with the aim of identifying those children with UTI and hydronephrosis in need of further invasive diagnostics, such as voiding cystourethrography, thus sparing most children without pathologic urinary proteome patterns from additional diagnostics. * Abbreviations: CE-MS — : capillary electrophoresis coupled mass spectrometry CI — : confidence interval CKD — : chronic kidney disease DMSA scan — : dimercaptosuccinic acid scintigraphy GFR — : glomerular filtration rate NPV — : negative predictive value PCT — : serum procalcitonin PPV — : positive predictive value PU — : proteinuria ROC — : receiver operating characteristic SVM — : support vector machine UTI — : urinary tract infection VCUG — : voiding cystourethrography VUR — : vesicoureteral reflux

Diffusion-weighted imaging (DWI) in children with diarrhoea associated haemolytic uraemic syndrome (D+HUS) and cerebral involvement was evaluated retrospectively.DWI within 24 h of onset of neurological symptoms. The apparent diffusion coefficient (ADC) was measured in grey/white matter and correlated with clinical and laboratory findings.DWI was abnormal in all. Abnormal ADC was detected in the supratentorial white matter (6/12) and cortex (1/12), the basal ganglia (5/12), the thalami (4/12), and the cerebellum (1/12). ADC was reduced in 5/12, increased in 4/12, and both in 3/12. Mean serum sodium was lower in patients with DWI abnormalities affecting the white matter (6/12), than in those with basal ganglia/thalamic involvement (6/12). Neurological outcome was normal in 4/11 and abnormal in 7/11, and 1 patient died, outcome did not correlate to either localisation or type of DWI abnormality.In D+HUS with neurological symptoms, early DWI may reveal abnormal ADC not only in the basal ganglia/thalami, but also in the white matter/cortex. Besides thrombotic microangiopathy, toxic effects of shiga toxin, azotaemia and hyponatraemia / hypoosmolality may be involved in cerebral involvement in children with D+HUS. Findings on early MRI seem not to predict clinical course or outcome.• DWI MR imaging may detect early CNS involvement in haemolytic uraemic syndrome • Different pathogenetical mechanisms may contribute to the CNS disease in HUS • Early MRI findings do not seem to allow prediction of clinical outcome.

MPG-EPO is a continuous erythropoietin receptor activator with a longer half-life than darbepoetin, hence requires less frequent injections. It has been successfully used in adults, but currently, there are no published data available for its use in children. This pilot study was performed to verify the effect of MPG-EPO on Hb levels in children. Twelve patients (age 6.4-17.2 yr) were treated with MPG-EPO as an individual "Heilversuch" according to German law after RTx. Five patients were switched from DA, and seven were naive to erythropoietin. Over a period of six months, Hb levels were measured monthly. A median MPG-EPO dose of 2.5 μg/kg was administered intravenously in a single dose every four wk. The median Hb value increased in naive patients from 9.9 to 11.2 g/dL (median, p = 0.004) and from 10.3 to 11.6 g/dL (median, p = 0.39) in patients switched from DA to MPG-EPO. No adverse events secondary to MPG-EPO therapy were detected. Our results indicate that a once-monthly injection of MPG-EPO is an effective treatment of anemia in children after renal transplantation. Larger randomized trials will have to confirm our findings.

Due to its accessibility and availability, ease of collection, and correlation with physiology and pathology, urine is an attractive source for clinical proteomics/peptidomics in urology and nephrology. Here, we review the published findings of a reproducible, high-resolution method for peptidome analysis of naturally occurring human urinary peptides and proteins - ranging from 0.8 to 17.0 kDA - using samples from renal patients analyzed by capillary electrophoresis coupled to mass spectrometry (CE-MS). CE-MS identified children with urodynamically relevant ureteric junction obstruction, vesicoureteric reflux of grades IV and V, glomerulopathies, tubulopathies, and chronic kidney disease. Our analysis revealed that the incorporation of urinary proteome analysis in the initial evaluation of children with urinary tract abnormalities will avoid side effects of radiological imaging techniques, reduce costs, and increase the quality-adjusted life years in this patient population. CE-MS can be recommended for clinical prospective studies on the analysis of naturally occurring urinary peptides in children with urinary tract diseases.

Regional citrate anticoagulation (RCA) has been considered to be a standard component of pediatric apheresis therapy for more than a decade. However, data on dosing recommendations and evaluations of the effectiveness and safety of anticoagulation are rarely found in published reports. The aim of this retrospective analysis was to present our single-center experience with RCA in pediatric apheresis therapy with the aim of developing an operating procedure. Five children aged 7–14 years underwent a total of 72 (range 3–44) therapeutic apheresis sessions with RCA in the form of immunoadsorption therapy (2 patients), low-density lipoprotein (LDL)-apheresis (1 patient), and plasmapheresis (two patients). A 3% citrate solution was used. Citrate flow was started at 4.0% of the blood flow velocity and was adapted to match post-filter ionized calcium levels ≤0.30 mmol/l. Once the patient’s ionized calcium fell to

The authors of this commentary, many of whom are part of a working group on social pediatrics supported by some of the European national pediatric societies member of EPA-UNEPSA, conclude that more studies are needed to cross the boundaries between disciplines, for example, looking at the inter-relationship of family life and child health and how parental loss is affecting the micro and macro level of child healthcare service systems. Therefore, this article aims to alert pediatricians to the impact of parental loss on child health and wellbeing. It emphasizes the importance of implementing preventive and social support programs for achieving health promotion and health-protective effects.

Kanzelmeyer NK, Ahlenstiel T, Drube J, Froede K, Kreuzer M, Broecker V, Ehrich JHH, Melk A, Pape L. Protocol biopsy-driven interventions after pediatric renal transplantation. Pediatr Transplantation 2010: 14:1012–1018 © 2010 John Wiley & Sons A/S. Abstract: The therapeutic value of protocol biopsies (PBs) in renal transplant recipients remains unclear. We performed protocol biopsies in 57 children six months after transplantation. We increased the CNI dose in patients with borderline findings. In cases of Banff grade Ia, six prednisolone IV-pulses were given and the CNI dose was increased. CNI toxicity and polyomavirus nephropathy led to a reduction in the CNI dose. GFR was compared with a control group of 51 children with no PBs transplanted in the same period. Forty-two percent of PBs had no pathological changes, 24% IF/TA. Borderline findings were detected in 11%, Banff grade Ia in 15% (CNI), toxicity in 8%, and one case showed polyomavirus nephropathy. GFR after 1.5 and 2.5 yr was similar in both groups. GFR 3.5 yr after transplantation was significantly higher in the intervention group (57 ± 17 vs. 46 ± 20). Patients treated with low-dose CNI and everolimus had a significantly lower number of pathological findings in PBs. The performance of protocol biopsies followed by a standardized treatment algorithm led to better graft function 3.5 yr after transplantation. Prospective randomized studies to confirm our findings are needed.

Berding G, Geisler S, Melter M, Marquardt P, Luhr A, Scheller F, Knoop BO, Pfister E-D, Pape L, Bischoff L, Knapp WH, Ehrich JHH. Estimation of glomerular filtration rate in liver-transplanted children: Comparison of simplified procedures using 51Cr-EDTA and endogenous markers with Sapirstein’s method as a reference standard. Pediatr Transplantation 2010: 14:786–795. © 2010 John Wiley & Sons A/S. Abstract: This study evaluated simple procedures for GFR determination in 48 liver-transplanted children. After injection of 51Cr-EDTA, blood samples were obtained up to four h, and activity retention in the body was measured for 60 min with scintillation probes. As a reference, GFR was calculated according to Sapirstein. Simplified calculations were performed according to Brochner-Mortensen, Russel, Devaux and Oberhausen. Additionally, GFR was determined using plasma creatinine and cystatin C according to Schwartz and Filler, respectively. The reference revealed mildly reduced GFR (62 ± 20 mL/min/1.73 m2). Russel’s method provided the highest degree of correlation (r2 = 0.95), the smallest bias in GFR determination (−2%), and only one false exclusion plus one false diagnosis of chronic kidney disease. Oberhausen’s method with blood sampling at one h post-injection performed slightly worse (r2 = 0.67, bias: 3%). All other methods resulted in significantly different GFR estimates compared to the reference. Nevertheless, notably, the second narrowest 95% limits of agreement (−31% to 45%) was observed using cystatin C. In conclusion, this data implies to prefer Russel’s method as a simplified procedure, and if patients cannot be available long enough (four h) for measurements, Oberhausen’s method instead. If radiotracer methods are not available at all or for screening GFR, cystatin C appears to be the procedure of choice.

Beil S, Drube J, Gluer S, Lehner F, Ehrich JHH, Pape L. End-stage renal disease due to ARPKD in the first months of life: Transplantation or dialysis? – Two case reports. Pediatr Transplantation 2010: 14:E75–E78. © 2009 John Wiley & Sons A/S. Abstract: ARPKD with renal insufficiency during the first months of life is a clinical challenge. We report on two children with ARPKD with massively enlarged kidneys requiring renal replacement therapy in early infancy. Patient 1 developed pulmonary insufficiency due to massively enlarged kidneys. At the age of six months the girl was listed for KT as “high urgency” on the Eurotransplant waiting list. A kidney from a deceased donor was pre-emptively transplanted and simultaneous nephrectomy performed. No postoperative complications were observed, and the patient was discharged from in-patient care 42 days after transplantation. Unexpectedly, she died at the age of one yr due to cerebral vascular spasms of unknown origin. Patient 2 was transferred at the age of three months to our clinic with life-threatening pulmonary insufficiency. Pre-emptive KT was not possible; therefore, bilateral nephrectomy was performed and PD begun. The boy is still doing well on PD one yr later. Pre-emptive KT and bilateral nephrectomy followed by PD are two options for infants with ARPKD and excessive kidney enlargement. PD could be complicated and in some cases become impossible by peritoneal damage during nephrectomy. On the other hand, KT covers a high risk of infections caused by immunosuppression. The decision, which method to choose, should be driven by the individual situation of the patient and the expertise of the center.

Because of its availability, ease of collection, and correlation with physiology and pathology, urine is an attractive source for clinical proteomics/peptidomics. However, the lack of comparable data sets from large cohorts has greatly hindered the development of clinical proteomics. Here, we report the establishment of a reproducible, high resolution method for peptidome analysis of naturally occurring human urinary peptides and proteins, ranging from 800 to 17,000 Da, using samples from 3,600 individuals analyzed by capillary electrophoresis coupled to MS. All processed data were deposited in an Structured Query Language (SQL) database. This database currently contains 5,010 relevant unique urinary peptides that serve as a pool of potential classifiers for diagnosis and monitoring of various diseases. As an example, by using this source of information, we were able to define urinary peptide biomarkers for chronic kidney diseases, allowing diagnosis of these diseases with high accuracy. Application of the chronic kidney disease-specific biomarker set to an independent test cohort in the subsequent replication phase resulted in 85.5% sensitivity and 100% specificity. These results indicate the potential usefulness of capillary electrophoresis coupled to MS for clinical applications in the analysis of naturally occurring urinary peptides.

Background. Hyperphosphataemia in patients with chronic kidney disease (CKD) is associated with mineral and bone disorder and increased cardiovascular mortality. Despite phosphate binders (PB), nutrition counselling and dialysis therapy, the prevalence of hyperphosphataemia remains unacceptably high. It was hypothesized that an inadequate relation of PB dose to meal inorganic phosphorus (iP) content may be an important factor for failure of phosphate management. Methods. The innovative ‘Phosphate Education Program’ (PEP) bases on patient empowerment to eye-estimate meal iP content by newly defined ‘Phosphate Units’ (PU; 1 PU per 100 mg phosphorus) and self-adjust PB dosage to dietary iP intake by an individually prescribed PB/PU ratio (PB pills per PU). In a prospective study, 16 children (aged 4–17 years) with CKD and their parents were trained with the PEP concept and followed up for 24 weeks for changes in serum electrolyte levels, dietary behaviour and PB dose. Results. Within 6 weeks after PEP training, the percentage of children with serum phosphate (PO) >1.78 mmol/ l dropped from 63% (10/16) to 31% (5/16). Mean serum PO level decreased from 1.94 ± 0.23 at baseline to 1.68 ± 0.30 (SD)mmol/l in Week 7–12 (P = 0.02) and to 1.78 ± 0.36 (SD)mmol/l in Week 19–24 (P = 0.2), whereas serum calcium [2.66 ± 0.3 vs 2.60 ± 0.23 (SD)mmol/l in Weeks 7–12 (P = 0.45) and 2.66 ± 0.23 (SD)mmol/l in Week 19– 24 (P = 0.21)] and serum potassium [4.69 ± 0.48 vs 4.58 ± 0.68 (SD)mmol/l in Week 7–12 (P = 0.40) and 4.65 ± 0.49 (SD) mmol/l in Week 19–24 (P = 0.73)] remained unchanged. The mean daily PB dose rose from 6.3 ± 2.9 to 8.2 ± 5.4 (SD) pills during observation period with an increased meal-to-meal variability (P = 0.04). Dietary iP intake was not affected by PEP concept. Conclusion. The empowerment of children with CKD and their parents to self-adjust PB dose to eye-estimated meal iP content significantly improved management of hyperphosphataemia without reducing dietary iP intake.

Recent years has seen an increasing use of regional citrate anticoagulation in pediatric dialysis. Several approaches have been described for monitoring anticoagulation in the extracorporeal circuit, such as serum citrate levels, post-filter ionized calcium (iCa), and activated coagulation time (ACT). However, no standard recommendations have yet been established for applying any of these parameters, especially for iCa. The objective of this retrospective analysis was to establish adequate coagulation management using post-filter iCa values. Normal values for ACTester-based ACT were established using a group of 64 children who were divided into two subgroups, with one subgroup comprising children without chronic kidney disease or coagulation disorder (age 1.2-17.5 years, median 9.7 years) and one consisting of 32 uremic patients (age 0.6-17.5 years, median 13.7 years). In a second group of 13 patients (aged 7-17 years), all of whom were undergoing high-flux dialysis (HD) with regional citrate anticoagulation (RCA), we assessed 73 post-filter blood samples for ionized calcium and ACT. A receiver operating characteristic graph was used to identify the iCa threshold needed to achieve adequate anticoagulation. Normal values for ACT were 90 s [2 standard deviations (SD) 72-109] in healthy children and 94 s (2 SD 75-113) in the uremic children. There was no statistically significant difference between the groups. In the children undergoing HD with RCA, the post-filter iCa level correlated with ACT (r = -0.94, p0.001). A post-filter iCa level ofor = 0.30 mmol/l reliably predicted an ACT120 s. Our citrate protocol [citrate 3% rate (ml/h) approximately blood flow rate (ml/min) x 2] meets the established criteria with a high sensitivity. Based on these results, we conclude that the post-filter iCa level can be reliably used for the management of extracorporeal anticoagulation with citrate in pediatric HD. We recommend the application of our citrate prescription protocol in the setting of pediatric intermittent hemodialysis.

SIOD is a multisystem disorder caused by a mutant chromatin remodelling protein. The main clinical findings are spondyloepiphyseal dysplasia with disproportionate growth restriction, defective cellular immunity, and steroid-resistant nephrotic syndrome secondary to biopsy proven FSGS leading to ESRF. Concerning ESRF, kidney transplantation is the therapy of choice since FSGS does not recur in the graft. However, with respect to the underlying immune disorder and the increased susceptibility to life threatening infections, the question of the optimal immunosuppressive therapy after renal transplantation remains unresolved. Under conventional immunosuppressive regimens some SIOD patients have developed severe disseminated cutaneous papilloma virus infections or EBV associated lymphoproliferative disease. We present several cases of children with SIOD (four of five had SMARCAL1 mutations) and monotherapy maintenance immunosuppression after renal transplantation and compare them with 13 patients from the SIOD registry. We have found that post-renal transplantation immunosuppressive monotherapy results in a good outcome with a reduced number of severe infections. Due to the underlying immunodeficiency in SIOD, limited immunosuppression may be possible without increasing the risk of acute or chronic rejection.

Steroidresistente nephrotische Syndrome (SRNS) treten bei primaren und sekundaren Glomerulopathien (GN) auf. Im Zentrum der Pathogenese steht der defekte Podozyt, wobei Podozytopathien angeboren oder erworben sein konnen. Die vorliegende Arbeit fasst den Kenntnisstand zur Therapie der SRNS zusammen. Die meisten Publikationen liesen die atiologische und histologische Heterogenitat der SRNS unberucksichtigt und ordneten die Patienten unselektiv einer idiopathischen Form zu. Das damit einhergehende Ausmas der Fehlinterpretationen hinsichtlich der Therapieeffekte ist schwer abschatzbar. Leider besteht ein Mangel an randomisierten, kontrollierten prospektiven Studien zum SRNS. Im eigenen Patientengut von mehr als 200 Kindern mit SRNS wurden 25% den genetischen, 25% den sekundaren und 50% den idiopathischen Formen zugeordnet. Bei den genetischen Formen wurde durch Immunsuppression meist keine SRNS-Remission erzielt. Bei den sekundaren Formen hing der Therapieerfolg vom Effekt der Behandlung auf die Grundkrankheit ab. Bei den idiopathischen Formen wurden zwischen 50 und 88% komplette Remissionen nach Induktionstherapie mit Ciclosporin A und i.v. Methylprednisolon erreicht.

Only 30 years ago, most children with terminal renal failure did not survive. Modern dialysis therapies (perotineal dialysis and hemodialysis) allow an adequate renal-replacement therapy for children today. The better alternative for children is kidney transplantation, which can be carried out even in small infants, leading to better survival and psychosocial development as well as allowing normal school education. Many allocation systems have increased the priorities for children but even more efforts have to be made to decrease waiting time for pediatric kidney recipients. A primary allocation of kidneys from children and young adults for pediatric recipients might be an important part of the solution of this problem. New immunosuppressive protocols have significantly increased long-term graft function and survival. Living donation, most often performed by the parents, is associated with better survival than donation of organs from deceased donors. In cadaveric donation, organs from pediatric or young adult donors should be preferentially allocated to children due to better future graft function. Pediatric kidney transplantation leads to a good psychosocial outcome for children with end-stage renal disease.

Background. The aetiology and outcome of acute (ARF) and chronic renal failure (CRF) in infants were analysed in a retrospective study. Methods. Between January 1997 and April 2004 all children 100 µmol/l at Hannover Medical School were followed up for up to 6 years. One hundred and nineteen children with a serum creatinine >100 µmol/l were identified, 70 infants suffering from ARF and 49 from chronic kidney disease (CKD), stages 3–5. Results. Renal failure was caused in 49/119 (41%) by congenital and in 70/119 (59%) by acquired diseases. The aetiology of ARF (n = 70) included cardiac (27%), prematurity (27%), septic (10%), hepatic (9%), renal (9%) and other (18%) causes. Twelve infants needed transient dialysis treatment. Renal function recovered in all surviving children. The mortality rate was 37%. Causes of death were unrelated to kidney function. Twenty-one of 49 infants with CKD were dialyzed with a median age of 65 days at the start of dialysis, and 23/49 children received a kidney transplant (RTx). The 5-year patient and graft survival for RTx-children of 95.5% was not different from older children. The 5-year patient survival rate of 26 children with CKD without RTx was 63%. The causes of death were parental refusal of therapy in neonates (n = 4) and life-threatening extra-renal comorbidity (n = 3). Conclusion. Renal replacement therapy offers good chances of survival in infants without life-threatening comorbidity. Patient survival of infants treated for CKD in the first year of life was comparable to that of older children.