A And P Kyriakou Childrens Hospital

Athens, Greece

A And P Kyriakou Childrens Hospital

Athens, Greece

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Verrina E.,Dialysis and Transplantation Unit | Groothoff J.W.,Emma Childrens Hospital | Melgar A.A.,Hospital La Paz | Edefonti A.,Fondazione Ca Granda IRCCS Ospedale Maggiore Policlinico | And 12 more authors.
Nephrology Dialysis Transplantation | Year: 2013

Background. The prevalence of childhood overweight is rising worldwide, but in children on renal replacement therapy (RRT) a poor nutritional status is still the primary concern. We aimed to study the prevalence of, and factors associated with, underweight and overweight/obesity in the European paediatric RRT population. Moreover, we assessed the evolution of body mass index (BMI) after the start of RRT. © The Author 2013. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.


Kolomvos N.,National and Kapodistrian University of Athens | Kamperos G.,National and Kapodistrian University of Athens | Theologie-Lygidakis N.,National and Kapodistrian University of Athens | Chrysomali E.,National and Kapodistrian University of Athens | And 2 more authors.
Journal of Craniofacial Surgery | Year: 2012

This article describes the first published case of coexistence in a child of a rare hybrid odontogenic ghost cell tumor and a solitary cutaneous pilomatrixoma. An 11-year-old boy presented with a large well-defined unilocular radiolucent lesion in the right posterior mandible. Marsupialization followed by enucleation of the remaining lesion at a later period was the treatment of choice. Histopathologic analysis revealed a hybrid tumor demonstrating areas identical to calcifying cystic odontogenic tumor, ameloblastoma, ameloblastic fibro-odontoma, ameloblastic fibromyxoma, and adenoid odontogenic tumor. A cutaneous nodule was also removed from the facial area and demonstrated classic features of pilomatrixoma on histopathology. Sixteen cases of hybrid calcifying cystic odontogenic tumor associated with odontogenic tumors other than ameloblastomas and odontomas are referred in the literature to date. Young males are frequently affected, and the mandible is the most common site of involvement. The occurrence in the same patient of 2 distinctive entities, which both demonstrate ghost/shadow cells, may be a coincidental finding or suggest a common origin regarding the histogenesis of these cells. Alternatively, future molecular studies may clarify possible genetic or/and predisposing factors for the development of these lesions. Copyright © 2012 by Mutaz B. Habal, MD.


Kotsakis S.D.,Hellenic Pasteur Institute | Miriagou V.,Hellenic Pasteur Institute | Vetouli E.E.,A And P Kyriakou Childrens Hospital | Bozavoutoglou E.,A And P Kyriakou Childrens Hospital | And 3 more authors.
Antimicrobial Agents and Chemotherapy | Year: 2015

The cephalosporinase CMY-107, a Tyr199Cys mutant form of CMY-2 encoded by an IncI self-transferable plasmid carried by an Escherichia coli clinical strain, was characterized. The enzyme hydrolyzed oximino-cephalosporins and aztreonam more efficiently than CMY-2 did. © 2015, American Society for Microbiology. All Rights Reserved.


Chesnaye N.C.,ESPN ERA EDTA Registry and ERA EDTA Registry | Schaefer F.,University of Heidelberg | Groothoff J.W.,Emma Childrens Hospital | Caskey F.J.,Richard Bright Renal Unit | And 12 more authors.
Nephrology Dialysis Transplantation | Year: 2015

BackgroundConsiderable disparities exist in the provision of paediatric renal replacement therapy (RRT) across Europe. This study aims to determine whether these disparities arise from geographical differences in the occurrence of renal disease, or whether country-level access-to-care factors may be responsible. MethodsIncidence was defined as the number of new patients aged 0-14 years starting RRT per year, between 2007 and 2011, per million children (pmc), and was extracted from the ESPN/ERA-EDTA registry database for 35 European countries. Country-level indicators on macroeconomics, perinatal care and physical access to treatment were collected through an online survey and from the World Bank database. The estimated effect is presented per 1SD increase for each indicator. ResultsThe incidence of paediatric RRT in Europe was 5.4 cases pmc. Incidence decreased from Western to Eastern Europe (-1.91 pmc/1321 km, P < 0.0001), and increased from Southern to Northern Europe (0.93 pmc/838 km, P = 0.002). Regional differences in the occurrence of specific renal diseases were marginal. Higher RRT treatment rates were found in wealthier countries (2.47 pmc/€10 378 GDP per capita, P < 0.0001), among those that tend to spend more on healthcare (1.45 pmc/1.7% public health expenditure, P < 0.0001), and among countries where patients pay less out-of-pocket for healthcare (-1.29 pmc/11.7% out-of-pocket health expenditure, P < 0.0001). Country neonatal mortality was inversely related with incidence in the youngest patients (ages 0-4, -1.1 pmc/2.1 deaths per 1000 births, P = 0.10). Countries with a higher incidence had a lower average age at RRT start, which was fully explained by country GDP per capita. ConclusionsInequalities exist in the provision of paediatric RRT throughout Europe, most of which are explained by differences in country macroeconomics, which limit the provision of treatment particularly in the youngest patients. This poses a challenge for healthcare policy makers in their aim to ensure universal and equal access to high-quality healthcare services across Europe. © 2015 The Author. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.


PubMed | University of Tirana, Institute of Social and Preventive Medicine, Copenhagen University, University of Kiev and 10 more.
Type: Journal Article | Journal: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association | Year: 2015

Considerable disparities exist in the provision of paediatric renal replacement therapy (RRT) across Europe. This study aims to determine whether these disparities arise from geographical differences in the occurrence of renal disease, or whether country-level access-to-care factors may be responsible.Incidence was defined as the number of new patients aged 0-14 years starting RRT per year, between 2007 and 2011, per million children (pmc), and was extracted from the ESPN/ERA-EDTA registry database for 35 European countries. Country-level indicators on macroeconomics, perinatal care and physical access to treatment were collected through an online survey and from the World Bank database. The estimated effect is presented per 1SD increase for each indicator.The incidence of paediatric RRT in Europe was 5.4 cases pmc. Incidence decreased from Western to Eastern Europe (-1.91 pmc/1321 km, P < 0.0001), and increased from Southern to Northern Europe (0.93 pmc/838 km, P = 0.002). Regional differences in the occurrence of specific renal diseases were marginal. Higher RRT treatment rates were found in wealthier countries (2.47 pmc/10 378 GDP per capita, P < 0.0001), among those that tend to spend more on healthcare (1.45 pmc/1.7% public health expenditure, P < 0.0001), and among countries where patients pay less out-of-pocket for healthcare (-1.29 pmc/11.7% out-of-pocket health expenditure, P < 0.0001). Country neonatal mortality was inversely related with incidence in the youngest patients (ages 0-4, -1.1 pmc/2.1 deaths per 1000 births, P = 0.10). Countries with a higher incidence had a lower average age at RRT start, which was fully explained by country GDP per capita.Inequalities exist in the provision of paediatric RRT throughout Europe, most of which are explained by differences in country macroeconomics, which limit the provision of treatment particularly in the youngest patients. This poses a challenge for healthcare policy makers in their aim to ensure universal and equal access to high-quality healthcare services across Europe.


Zurowska A.M.,Medical University of Gdańsk | Fischbach M.,Hopital de Hautepierre | Watson A.R.,University of Nottingham | Edefonti A.,Clinica Pediatrica De Marchi | Stefanidis C.J.,A and P Kyriakou Childrens Hospital
Pediatric Nephrology | Year: 2013

Background: To provide recommendations for the care of infants with stage 5 chronic kidney disease (CKD5). Setting: European Paediatric Dialysis Working Group. Data Sources: Literature on clinical studies involving infants with CKD5 (end stage renal failure) and consensus discussions within the group. Recommendations: There has been an important change in attitudes towards offering RRT (renal replacement therapy) to both newborns and infants as data have accumulated on their improved survival and long-term outcomes. The management of this challenging group of patients differs in a number of ways from that of older children. The authors have summarised the basic recommendations for treating infants with CKD5 in order to support the multidisciplinary teams who endeavour on this difficult task. © 2012 The Author(s).


Halbritter J.,University of Michigan | Diaz K.,University of Michigan | Chaki M.,University of Michigan | Porath J.D.,University of Michigan | And 10 more authors.
Journal of Medical Genetics | Year: 2012

Objective: To identify disease-causing mutations within coding regions of 11 known NPHP genes (NPHP1-NPHP11) in a cohort of 192 patients diagnosed with a nephronophthisis-associated ciliopathy, at low cost. Methods: Mutation analysis was carried out using PCR-based 48.48 Access Array microfluidic technology (Fluidigm) with consecutive next-generation sequencing. We applied a 10-fold primer multiplexing approach allowing PCR-based amplification of 475 amplicons (251 exons) for 48 DNA samples simultaneously. After four rounds of amplification followed by indexing all of 192 patient-derived products with different barcodes in a subsequent PCR, 2×100 paired-end sequencing was performed on one lane of a HiSeq2000 instrument (Illumina). Bioinformatics analysis was performed using 'CLC Genomics Workbench' software. Potential mutations were confirmed by Sanger sequencing and shown to segregate. Results: Bioinformatics analysis revealed sufficient coverage of 30×for 168/192 (87.5%) DNA samples (median 449×) and of 234 out of 251 targeted coding exons (sensitivity: 93.2%). For proof-of-principle, we analysed 20 known mutations and identified 18 of them in the correct zygosity state (90%). Likewise, we identified pathogenic mutations in 34/192 patients (18%) and discovered 23 novel mutations in the genes NPHP3 (7), NPHP4 (3), IQCB1 (4), CEP290 (7), RPGRIP1L (1), and TMEM67 (1). Additionally, we found 40 different single heterozygous missense variants of unknown significance. Conclusions: We conclude that the combined approach of array-based multiplexed PCR-amplification on a Fluidigm Access Array platform followed by next-generation sequencing is highly cost-efficient and strongly facilitates diagnostic mutation analysis in broadly heterogeneous Mendelian disorders.


Hayes W.N.,Nottingham Childrens Hospital | Watson A.R.,Nottingham Childrens Hospital | Callaghan N.,Nottingham Childrens Hospital | Wright E.,Great Ormond Street Hospital | Stefanidis C.J.,A and P Kyriakou Childrens Hospital
Pediatric Nephrology | Year: 2012

Background European and U.S. guidelines emphasise that permanent vascular access in the form of arteriovenous fistulae (AVF) or grafts (AVG) are preferable to central venous catheters (CVC) in paediatric patients on long-term haemodialysis. We report vascular access choice and complication rates in 13 European paediatric nephrology units. Methods A survey of units participating in the European Pediatric Dialysis Working Group requesting data on type of vascular access, routine care and complications in patients on chronic haemodialysis between March 2010 and February 2011. Results Information was complied on 111 patients in 13 participating centres with a median age of 14 (range 0.25-20.2) years. Central venous catheters were used in 67 of 111 (60%) patients, with 42 patients (38%) having an AVF and two patients (2%) having an AVG. Choice of vascular access was significantly related to patient age, with patients with AVF/AVG having a median age of 16 years compared to 12 years for patients with CVCs (p<0.001). Routine CVC exit site care and catheter lock solution use differed between centres. CVC infections requiring intravenous antibiotics were reported at a rate of 1.9 and exit site infections at a rate of 1.8 episodes/1000 catheter days. Overall infective complications necessitating CVC change occurred at a rate of 0.9 episodes/1000 catheter days. No infective complications were reported in patients with AVF/AVG access. The rate of CVC infections requiring intravenous antibiotics was significantly lower in patients in whom CVC exit sites were cleaned weekly as opposed to every dialysis session (relative risk with every session cleaning vs. weekly cleaning 2.58, 95% confidence interval 1.17-5.69). Catheter malfunction (inadequate blood flow) was a more prevalent complication necessitating 22.4 thrombolytic interventions/1000 catheter days and 2.1 CVC changes/1000 catheter days. Conclusions Central venous catheters remain the predominant choice of vascular access in Europe despite problems of malfunction and infection. AVF/AVG were predominantly used in adolescents without reported complications. More regular exit site cleaning may predispose to CVC infection, but this observation requires prospective evaluation. © IPNA 2011.

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