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Background There is increasing focus on the problems involved in the transition and transfer of young adult patients from paediatric to adult renal units. This situation was addressed by the 2011 International Pediatric Nephrology Association/International Society of Nephrology (IPNA/ISN) Consensus Statement on transition. Methods We performed a survey of transition practices of 15 paediatric nephrology units across Europe 2 years after publication of the consensus statement. Results Two thirds of units were aware of the guidelines, and one third had integrated them into their transition practice. Forty-seven per cent of units transfer five or fewer patients with chronic kidney disease (CKD) stage 5 per year to a median of five adult centres, with higher numbers of CKD stages 2-4 patients. Seventy-three per cent of units were required by the hospital or government to transfer patients by a certain age. Eighty per cent of units commenced transition planning after the patient turned 15 years of age and usually within 1-2 years of the compulsory transfer age. Forty-seven per cent of units used a transition or transfer clinic. Prominent barriers to effective transition were patient and parent attachment to the paediatric unit and difficulty in allowing the young person to perform self-care. Conclusions Whereas awareness of the consensus statement is suboptimal, it has had some impact on practice. Adult nephrologists receive transferred patients infrequently, and the process of transition is introduced too late by paediatricians. Government- and hospital-driven age-based transfer policies distract focus from the achievement of competencies in self care. Variable use of transition clinics, written patient information and support groups is probably due to economic and human-resource limitations. The consensus statement provides a standard for evolving and evaluating transitionpolicies jointly agreed upon by paediatric and adult units. Keywords Adolescence * Chronic kidney disease * Guidelines * Survey * Transition * Young adult, Introduction Increasing numbers of children with chronic illness, including chronic kidney disease (CKD), are surviving into young adulthood and requiring transition to adult services [1]. Numbers transferring within localities may [...]

Chronic kidney disease (CKD) and the need for renal replacement therapy (RRT) can place a great strain on the child and family. As well as the medical and nutritional prescription, each child and family requires an individual psychosocial prescription that requires input from multiprofessional team members. The information needs of each child and family need to be constantly evaluated as well as the choice of therapy in relation to social, psychological and economic factors. Many tertiary units lack adequate 'time' to deliver such assessments and coordinate the support and respite care for those on long-term dialysis, especially when significant numbers of children are now accepted onto RRT programmes with co-morbidities. National and international standards are needed for the staffing of comprehensive tertiary paediatric renal units as well as studies evaluating supportive care to families. Keywords Psychosocial * Renal replacement therapy * Chronic kidney disease (CKD) * Multiprofessional team * Respite care, Introduction The diagnosis of chronic illness not only has implications for the child, but also for the whole family. A child with chronic kidney disease (CKD) can be offered treatment, [...]

Background Many factors may impact upon choice of renal replacement therapy (RRT) for children and adolescents, including patient and family choice, patient size and distance from the renal centre as well as logistic issues such as facilities and staffing at the unit. We report a survey of factors influencing treatment choice in 14 European paediatric nephrology units. Methods A questionnaire was developed by consensus and completed by 14 members of the European Paediatric Dialysis Working Group on facilities, staffing and family assessments impacting on choice of therapy as well as choice of therapy for 97 patients commencing initial RRT in 2011. Results All units offered all modalities of RRT, but there were limitations for pre-emptive transplantation (PET) and largely adult surgical dependence for creation of arteriovenous fistulae and transplantation. The average waiting time for a deceased donor kidney was 18.5 (range 3-36) months. Full time dietetic support was available in six of the 14 units. There was no social worker, psychology, play therapy or teaching support in three, two, seven and four units, respectively. Assessment by other members of the multidisciplinary team and home visits before choice of therapy was carried out in 50 % of units, and although all patients were discussed at team meetings, the medical opinion predominated. In terms of types of RRT, 50 % of patients were commenced on chronic peritoneal dialysis (PD), 34 % on haemodialysis (HD) and 16 % underwent pre-emptive transplantation (PET). Chronic PD predominated in patients aged 10 years. Patient and family choice and age or size of patient were predominant factors in choice of therapy with a predictable decline in renal function favouring PET and social factors HD. Conclusions Chronic peritoneal dialysis predominated as primary choice of RRT, especially in younger children. The PET rates remain low. The influence of surgeons predominanted, and national transplant rules may be significant. Most units had insufficient multiprofessional support, which may impact upon initial choice of therapy as well as sustaining families through RRT. Keywords Choice of therapy * Renal replacement therapy * Dialysis * Pre-emptive transplantation * Psychosocial * Pediatric, Introduction The choice of chronic renal replacement therapy (RRT) for each individual child and family can be a complex issue. It involves consideration of the facilities and expertise available at [...]

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. Keywords Infants * Neonates * Chronic kidney disease (CKD) * Haemodialysis (HD) * Peritoneal dialysis (PD), Introduction The European Paediatric Dialysis Working Group (EPDWG) was established in 1999 and comprises of paediatric nephrologists with a major interest in dialysis from 13 European countries. Recommendations produced by [...]

Down's syndrome (DS) is a genetic disorder caused by full or partial trisomy of human chromosome 21 and presents with many clinical phenotypes including a reduced incidence of solid tumours (1,2). Recent work with the Ts65Dn model of DS, which has orthologues of about 50% of the genes on chromosome 21 (Hsa21), has indicated that three copies of the ETS2 (ref. 3) or DS candidate region 1 (DSCR1) genes (4) (a previously known suppressor of angiogenesis (5,6)) is sufficient to inhibit tumour growth. Here we use the Tc1 transchromosomic mouse model of DS (7) to dissect the contribution of extra copies of genes on Hsa21 to tumour angiogenesis. This mouse expresses roughly 81% of Hsa21 genes but not the human DSCR1 region. We transplanted B16F0 and Lewis lung carcinoma tumour cells into Tc1 mice and showed that growth of these tumours was substantially reduced compared with wild-type littermate controls. Furthermore, tumour angiogenesis was significantly repressed in Tc1 mice. In particular, in vitro and in vivo angiogenic responses to vascular endothelial growth factor (VEGF) were inhibited. Examination of the genes on the segment of Hsa21 in Tc1 mice identified putative anti-angiogenic genes (ADAMTS1 (8,9) and ERG (10)) and novel endothelial cell-specific genes (11), never previously shown to be involved in angiogenesis (JAM-B (12) and PTTG1IP), that, when overexpressed, are responsible for inhibiting angiogenic responses to VEGF. Three copies of these genes within the stromal compartment reduced tumour angiogenesis, explaining the reduced tumour growth in DS. Furthermore, we expect that, in addition to the candidate genes that we show to be involved in the repression of angiogenesis, the Tc1 mouse model of DS will permit the identification of other endothelium-specific anti-angiogenic targets relevant to a broad spectrum of cancer patients., DS, or trisomy 21, is responsible for developmental abnormalities, learning disability, cardiovascular defects and dementia (1). As well as these phenotypes, which have been well documented in DS, three independent [...]

Inhibitors of [[alpha].sub.v][[beta].sub.3] and [[alpha].sub.v][[beta].sub.5] integrin have entered clinical trials as antiangiogenic agents for cancer treatment but generally have been unsuccessful. Here we present in vivo evidence that low (nanomolar) concentrations of RGD-mimetic [[alpha].sub.v][[beta].sub.3] and [[alpha].sub.v][[beta].sub.5] inhibitors can paradoxically stimulate tumor growth and tumor angiogenesis. We show that low concentrations of these inhibitors promote VEGF-mediated angiogenesis by altering [[alpha].sub.v][[beta].sub.3] integrin and vascular endothelial growth factor receptor-2 trafficking, thereby promoting endothelial cell migration to VEGF. The proangiogenic effects of low concentrations of RGD-mimetic integrin inhibitors could compromise their efficacy as anticancer agents and have major implications for the use of RGD-mimetic compounds in humans., Inhibitors of angiogenesis can suppress tumor growth in preclinical models and have entered the clinic as prospective anticancer therapeutics (1,2). Tumor angiogenesis is predominantly driven by vascular endothelial growth factor [...]

Adolescent patients are recognised as having a number of problems related to their stage of development in addition to those associated with chronic illness (McDonagh 2000, Viner and Keane 1998). [...]