European chronic kidney disease registries for children not on kidney replacement therapy: tools for improving health systems and patient-centred outcomes

Chronic kidney disease (CKD) in children, from birth to late adolescence, is a unique and highly challenging condition that requires epidemiological research and large-scale, prospective cohort studies. Since its first launch in 2007, the European Society for Paediatric Nephrology/European Renal Association (ESPN/ERA) Registry has collected data on patients on kidney replacement therapy (KRT). However, slowing the progression of CKD is of particular importance and thus the possibility to extend the current registry dataset to include patients in CKD stages 4–5 should be a priority. A survey was sent to the national representatives within the ESPN/ERA Registry to collect information on whether they are running CKD registries. All the representatives from the 38 European countries involved in the ESPN/ERA Registry participated in the survey. Eight existing CKD registries have been identified. General characteristics of the national registry and detailed data on anthropometry, laboratory tests and medications at baseline and at follow-up were collected. Results provided by this survey are highly promising regarding the establishment of an ESPN CKD registry linked to the ESPN/ERA KRT registry and subsequently linking it to the ERA Registry with the same patient identifier, which would allow us to monitor disease progression in childhood and beyond. It is our belief that through such linkages, gaps in patient follow-up will be eliminated and patient-centred outcomes may be improved

Psychosocial environment for the integrated education opportunities of the disabled in Lithuania

BACKGROUND: The policy of the diminution of the social isolation of the disabled is the main objective of the strategy of the EU new policy concerning the disabled. Lithuanian society faces this objective as well. For this reason, this study aiming at providing the theoretical basis for and predicting the possible psycho-social environment in an integrated education system, as well as at the evaluation of the reasons for the formation of a positive approach to the disabled, is especially relevant, since it creates the prerequisites for the optimisation of the process of the integration of disabled schoolchildren into the general system of education. METHOD: The sample of the study consisted of 2471 children from the same schools: not integrated (1958), integrated (126) and special schools (382). Empirical methods: questionnaire poll, comparative analysis. The statistical analysis was carried out using SAS. RESULTS: Our study showed that the majority of schoolchildren without disabilities and disabled schoolchildren have positive intentions for interpersonal interactions (>82%) and positive emotions (>69%) independently of the discrepant character of interpersonal contacts, different conditions of education and family life, and despite of low level of knowledge. CONCLUSION: The results of the study confirmed positive intentions for interpersonal interaction between disabled schoolchildren and schoolchildren without disabilities, as well as a positive character of emotions, and disprove the unsound myth of the opponents of the social integration of the disabled stating that disabled children in comprehensive schools would undoubtedly experience offence from their peers without disabilities

The European Rare Kidney Disease Registry (ERKReg): objectives, design and initial results

BACKGROUND: The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient's outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. The diagnosis (Orphacode) and diagnostic information (clinical, imaging, histopathological, biochemical, immunological and genetic) are recorded. Anthropometric, kidney function, and disease-specific management and outcome items informing a set of 61 key performance indicators (KPIs) are obtained annually. Data quality is ensured by automated plausibility checks upon data entry and regular offline database checks prompting queries. Centre KPI statistics and benchmarking are calculated automatically. RESULTS: Within the first 24 months since its launch, 7607 patients were enrolled to the registry at 45 pediatric and 12 specialized adult nephrology units from 21 countries. A kidney disease diagnosis had been established in 97.1% of these patients at time of enrolment. While 199 individual disease entities were reported by Orphacode, 50% of the cohort could be classified with 11, 80% with 43 and 95% with 92 codes. Two kidney diagnoses were assigned in 6.5% of patients; 5.9% suffered from syndromic disease. Whereas glomerulopathies (54.8%) and ciliopathies including autosomal dominant polycystic kidney disease (ADPKD) (31.5%) were the predominant disease groups among adults, the pediatric disease spectrum encompassed congenital anomalies of the kidney and urinary tract (CAKUT) (33.7%), glomerulopathies (30.7%), ciliopathies (14.0%), tubulopathies (9.2%), thrombotic microangiopathies (5.6%), and metabolic nephropathies (4.1%). Genetically confirmed diagnoses were reported in 24% of all pediatric and 12% adult patients, whereas glomerulopathies had been confirmed by kidney biopsy in 80.4% adult versus 38.5% pediatric glomerulopathy cases. CONCLUSIONS: ERKReg is a rapidly growing source of epidemiological information and patient cohorts for clinical research, and an innovative tool to monitor management quality and patient outcomes

Vaccination Practices in Pediatric Dialysis Patients Across Europe. A European Pediatric Dialysis Working Group and European Society for Pediatric Nephrology Dialysis Working Group Study

Background: Data on the immunization practices in pediatric chronic kidney disease (CKD) patients are scarce. The purpose of this study was to evaluate current vaccination practices for children on dialysis across European pediatric nephrology centers. Methods: A total of 18 tertiary pediatric nephrology centers from 12 European countries were included in the study. The data on universal national immunization programs and immunization practices for children with chronic disease or risk were recorded from European Center for Disease Prevention and Control and the World Health Organization. The immunization practices and center protocols for monitoring antibody titers after vaccination in dialysis patients were obtained through a questionnaire. Results: All centers included in the study recommended immunization against hepatitis B virus (HBV), diphtheria, tetanus, pertussis, Hemophilus influenzae type b (Hib), poliomyelitis, measles, mumps, rubella (MMR), and streptococcus pneumonia in dialysis patients. In 16 centers, dialysis patients were vaccinated against influenza virus annually. HBV protective antibody titers were measured in 17 centers (during dialysis period in 14 centers, during pre-renal transplantation preparations in 14 centers or in both times in 11 centers). Hepatitis A virus (HAV) was reported to be followed in 13 centers, in 8 centers during dialysis period, and in 11 centers during pre-RTx preparations. MMR and varicella-zoster virus (VZV) protective antibody titers were measured during the dialysis period or before renal transplantation (RTx) in 12 and 15 centers, respectively, and in 6 centers both titers were checked both times. Conclusion: There are variations in vaccination practice across Europe. Children with CKD, those undergoing dialysis, and transplant candidates should receive age-appropriate vaccinations before RTx as well as before the transition to adult nephrology clinics and antibody levels should be monitored to evaluate the immunization status before and after RTx. (C) 2017 S. Karger AG, Basel.Peer reviewe

Management of children with congenital nephrotic syndrome: challenging treatment paradigms

Background: Management of children with congenital nephrotic syndrome (CNS) is challenging. Bilateral nephrectomies followed by dialysis and transplantation are practiced in most centres, but conservative treatment may also be effective. / Methods: We conducted a 6-year review across members of the European Society for Paediatric Nephrology Dialysis Working Group to compare management strategies and their outcomes in children with CNS. / Results: Eighty children (50% male) across 17 tertiary nephrology units in Europe were included (mutations in NPHS1, n = 55; NPHS2, n = 1; WT1, n = 9; others, n = 15). Excluding patients with mutations in WT1, antiproteinuric treatment was given in 42 (59%) with an increase in S-albumin in 70% by median 6 (interquartile range: 3–8) g/L (P < 0.001). Following unilateral nephrectomy, S-albumin increased by 4 (1–8) g/L (P = 0.03) with a reduction in albumin infusion dose by 5 (2–9) g/kg/week (P = 0.02). Median age at bilateral nephrectomies (n = 29) was 9 (7–16) months. Outcomes were compared between two groups of NPHS1 patients: those who underwent bilateral nephrectomies (n = 25) versus those on conservative management (n = 17). The number of septic or thrombotic episodes and growth were comparable between the groups. The response to antiproteinuric treatment, as well as renal and patient survival, was independent of NPHS1 mutation type. At final follow-up (median age 34 months) 20 (80%) children in the nephrectomy group were transplanted and 1 died. In the conservative group, 9 (53%) remained without dialysis, 4 (24%; P < 0.001) were transplanted and 2 died. / Conclusion: An individualized, stepwise approach with prolonged conservative management may be a reasonable alternative to early bilateral nephrectomies and dialysis in children with CNS and NPHS1 mutations. Further prospective studies are needed to define indications for unilateral nephrectomy

Utilising biological geotextiles: Introduction to the BORASSUS project and global perspectives

Field and laboratory studies indicate that utilisation of biological geotextiles constructed from palm-leaves and other selected organic materials are an effective, sustainable and economically viable soil conservation technique. The three-year plus (1 July 2005–28 February 2009) EU-funded BORASSUS Project (contract no. INCO-CT-2005-510745) evaluated the long-term effectiveness of biological geotextiles in controlling soil erosion and assessing their sustainability and economic viability. These studies progressed in ten countries, both in the ‘industrial north’ (in Europe) and in the ‘developing south’ (Africa, South America and South East Asia). The studied countries in the ‘developing south’ included Brazil, China, The Gambia, South Africa, Thailand and Vietnam. The ‘industrial north’ countries included Belgium, Hungary, Lithuania and the UK. The main findings of these studies are summarised in this paper and thematic information is presented in the other four papers in this Special Issue. Biological geotextiles offer potentially novel bioengineering solutions to environmental problems, including technologies for soil conservation, sustainable plant production and use of indigenous plants, improved ecosystem management by decreasing deforestation, improving agroforestry and cost-effective biogeotextile applications in diverse environments. Biogeotextiles may provide socio-economic platforms for sustainable development and the benefits for developing countries may include poverty alleviation, engagement of local people as stakeholders, employment for disadvantaged groups, small and medium enterprise (SME) development, earning hard currency, environmental education and local community involvement in land reclamation and environmental education programmes. These benefits are achieved through: (i) promotion of sustainable and environmentally friendly palm-agriculture to discourage deforestation, promoting both reforestation and agroforestry; (ii) construction of biogeotextiles enabling development of a rural labour-intensive industry, particularly encouraging employment of socially disadvantaged groups and (iii) export of biogeotextiles to industrialised countries could earn hard currency for developing economies, based on the principles of fair trade. Research and development activities of the BORASSUS Project have improved our knowledge on the effect of biogeotextile mats on the micro- and macro-soil environments and at larger scales through controlled laboratory and field experiments in diverse environments

Experiences of shame and guilt in anorexia and bulimia nervosa: A systematic review

Objectives Emotional states may play an important role in the development and maintenance of anorexia (AN) and bulimia nervosa (BN). This systematic review aimed to examine the evidence regarding the relationship that shame and guilt have with two eating disorders, AN and BN. Methods Four major databases (Pubmed, PsychINFO, Web of Science, Medline) were searched (up until April 2018) for studies measuring guilt or shame in clinically diagnosed AN and BN groups. Included papers were evaluated for risk of bias. Results Twenty‐four papers met the inclusion criteria. Several methodological issues were noted within the reviewed studies, including a lack of longitudinal data and unaccounted confounding variables. Nonetheless, shame was typically more common in those with AN and BN than controls, was positively related to the severity of symptoms, and associated with the onset of eating disorder‐related difficulties (e.g., binging or purging). Effect sizes were typically moderate to large. The role of guilt was less clear, with few studies and mixed results. Discussion There is preliminary evidence that shame is implicated in the aetiology of AN and BN presentations, whilst there is currently insufficient evidence of such a role for guilt. It remains unclear whether shame is a risk factor for the development of AN and BN or a consequence of these difficulties. Practitioner Points Elevated shame appears to be a feature of anorexia (AN) and bulimia nervosa (BN). Shame appears to fluctuate with the occurrence of eating disordered behaviours like binging, purging or restricted eating. Guilt is less consistently linked to AN and BN presentations. Interventions directed at shame may be helpful for these populations. A lack of longitudinal data means the temporal features of these relationships are still unclear

Hemodialysis in children with ventriculoperitoneal shunts: prevalence, management and outcomes

Peer reviewe