The spectrum of co-existing disease in children with established kidney failure using registry and linked electronic health record data.

Background: Children with established kidney failure may have additional medical conditions influencing kidney care and outcomes. This cross-sectional study aimed to examine the prevalence of co-existing diseases captured in the electronic hospital record compared to UK Renal Registry (UKRR) data and differences in coding.

Methods: The study population comprised children aged < 18 years receiving kidney replacement therapy (KRT) in England and Wales on 31/12/2016.

Psychosocial Health Among Young Adults With Kidney Failure: A Longitudinal Follow-up of the SPEAK (Surveying Patients Experiencing Young Adult Kidney Failure) Study.

Rationale & Objective: There have been no longitudinal studies examining the evolution of psychosocial health of young adults with kidney failure as they age. We aimed to address this in the Surveying Patients Experiencing Young Adult Kidney Failure-2 (SPEAK-2) study.

Study Design: 5-year follow-up longitudinal survey of the original SPEAK cohort.

Associations with kidney transplant survival and eGFR decline in children and young adults in the United Kingdom: a retrospective cohort study.

Background: Although young adulthood is associated with transplant loss, many studies do not examine eGFR decline. We aimed to establish clinical risk factors to identify where early intervention might prevent subsequent adverse transplant outcomes.

Methods: Retrospective cohort study using UK Renal Registry and UK Transplant Registry data, including patients aged < 30 years transplanted 1998-2014.

Associations with Wellbeing and Medication Adherence in Young Adults Receiving Kidney Replacement Therapy.

Background And Objectives: Young adults receiving kidney replacement therapy (KRT) have impaired quality of life and may exhibit low medication adherence. We tested the hypothesis that wellbeing and medication adherence are associated with psychosocial factors.

Design, Setting, Participants, & Measurements: We conducted a cross-sectional online survey for young adults on KRT.

Psychosocial Health and Lifestyle Behaviors in Young Adults Receiving Renal Replacement Therapy Compared to the General Population: Findings From the SPEAK Study.

Rationale & Objective: Patients in late adolescence and early adulthood receiving renal replacement therapy (RRT) face disruption to normal activities, which affects well-being. We aimed to define psychosocial and lifestyle outcomes for young adults on RRT compared to the general population.

Study Design: We undertook a cross-sectional survey (the SPEAK [Surveying Patients Experiencing Young Adult Kidney Failure] Study) using validated measures and general population comparator data from the Health Survey for England and Avon Longitudinal Study of Parents and Children.

Young adults' perspectives on living with kidney failure: a systematic review and thematic synthesis of qualitative studies.

Introduction: Young adults fare worse than younger adolescents or older adults on a broad range of health indicators. Those with a chronic illness such as renal failure are a particularly vulnerable group, who experience poor outcomes compared with both children and older adults. Understanding how being in receipt of renal replacement therapy (RRT) affects the lives of young adults might help us to better prepare and support these individuals for and on RRT, and improve outcomes.

Sociodemographic, Psychologic Health, and Lifestyle Outcomes in Young Adults on Renal Replacement Therapy.

Background And Objectives: Young adults receiving RRT face additional challenges in life. The effect of established kidney failure on young adulthood is uncertain. We aimed to establish the psychosocial and lifestyle status of young adults receiving RRT.

Continually improving standards of care: The UK Renal Registry as a translational public health tool.

A disease registry uses observational study methods to collect defined data on patients with a particular condition for a predetermined purpose. By providing comprehensive standardised data on patients with kidney disease, renal registries aim to provide a 'real world' representation of practice patterns, treatment and patient outcomes that may not be captured accurately by other methods, including randomised controlled trials. Additionally, using registries to measure variations in outcomes and audit care against standards is crucial to understanding how to improve quality of care for patients in an efficacious and cost-effective manner.

The clinical epidemiology of young adults starting renal replacement therapy in the UK: presentation, management and survival using 15 years of UK Renal Registry data.

Background: Clinical epidemiology data for young adults on renal replacement therapy (RRT) are lacking. While mostly transplanted, they have an increased risk of graft loss during young adulthood.

Methods: We combined the UK Renal Registry paediatric and adult databases to describe patient characteristics, transplantation and survival for young adults.

UK Renal Registry 18th Annual Report: Chapter 10 Clinical, Haematological and Biochemical Parameters in Patients Receiving Renal Replacement Therapy in Paediatric Centres in the UK in 2014: National and Centre-specific Analyses.

The median height z-score for paediatric patients on dialysis was −2.1 and for those with a functioning transplant −1.3.

UK Renal Registry 18th Annual Report: Chapter 4 Demography of Patients Receiving Renal Replacement Therapy in Paediatric Centres in the UK in 2014.

A total of 917 children and young people under 18 years with established renal failure (ERF) were receiving treatment at paediatric nephrology centres in 2014.At the census date (31st December 2014), 79.3% of prevalent paediatric patients aged ,18 years had a functioning kidney transplant, 11.

Autoimmune haemolytic anaemia associated with Epstein Barr virus infection as a severe late complication after kidney transplantation and successful treatment with rituximab: case report.

Background: Autoimmune haemolytic anaemia (AIHA) is a rare complication following kidney transplantation and usually occurs early in its course. It is characterised by autoantibodies or alloantibodies directed against red blood cells (RBCs).

Case Presentation: We describe a 44 year old woman who presented 5 years after kidney transplantation with profound transfusion dependent warm AIHA.

UK Renal Registry 17th Annual Report: Chapter 9 Clinical, Haematological and Biochemical Parameters in Patients Receiving Renal Replacement Therapy in Paediatric Centres in the UK in 2013: National and Centre-specific Analyses.

Background: The Paediatric Registry analyses renal replacement therapy (RRT) data in children. All 13 UK paediatric nephrology centres submit electronic data.

Aims: To provide centre specific data and to determine adherence to relevant audit standards.

UK Renal Registry 17th Annual Report: Chapter 4 Demography of the UK Paediatric Renal Replacement Therapy Population in 2013.

Aims: To describe the demographics of the paediatric renal replacement therapy (RRT) population under the age of 18 years in the UK and to analyse changes in demography over time.

Methods: Data were collected electronically from all 13 paediatric renal centres within the UK. A series of cross-sectional and longitudinal analyses were performed to describe the demographics of paediatric RRT patients.

HNF1B-associated renal and extra-renal disease-an expanding clinical spectrum.

Heterozygous mutations in the gene that encodes the transcription factor hepatocyte nuclear factor 1β (HNF1B) represent the most common known monogenic cause of developmental kidney disease. Renal cysts are the most frequently detected feature of HNF1B-associated kidney disease; however, other structural abnormalities, including single kidneys and renal hypoplasia, and electrolyte abnormalities can also occur. Extra-renal phenotypes might also be observed; consequently, HNF1B-associated disease is considered a multi-system disorder.

The HNF4A R76W mutation causes atypical dominant Fanconi syndrome in addition to a β cell phenotype.

Background: Mutation specific effects in monogenic disorders are rare. We describe atypical Fanconi syndrome caused by a specific heterozygous mutation in HNF4A. Heterozygous HNF4A mutations cause a beta cell phenotype of neonatal hyperinsulinism with macrosomia and young onset diabetes.