An environmental trigger has been proposed as an inciting factor in the development of anti-GBM disease. This multicenter, observational study sought to define the national incidence of anti-GBM ...disease during an 11-year period (2003-2014) in Ireland, investigate clustering of cases in time and space, and assess the effect of spatial variability in incidence on outcome.
We ascertained cases by screening immunology laboratories for instances of positivity for anti-GBM antibody and the national renal histopathology registry for biopsy-proven cases. The population at risk was defined from national census data. We used a variable-window scan statistic to detect temporal clustering. A Bayesian spatial model was used to calculate standardized incidence ratios (SIRs) for each of the 26 counties.
Seventy-nine cases were included. National incidence was 1.64 (95% confidence interval 95% CI, 0.82 to 3.35) per million population per year. A temporal cluster (n=10) was identified during a 3-month period; six cases were resident in four rural counties in the southeast. Spatial analysis revealed wide regional variation in SIRs and a cluster (n=7) in the northwest (SIR, 1.71; 95% CI, 1.02 to 3.06). There were 29 deaths and 57 cases of ESRD during a mean follow-up of 2.9 years. Greater distance from diagnosis site to treating center, stratified by median distance traveled, did not significantly affect patient (hazard ratio, 1.80; 95% CI, 0.87 to 3.77) or renal (hazard ratio, 0.76; 95% CI, 0.40 to 1.13) survival.
To our knowledge, this is the first study to report national incidence rates of anti-GBM disease and formally investigate patterns of incidence. Clustering of cases in time and space supports the hypothesis of an environmental trigger for disease onset. The substantial variability in regional incidence highlights the need for comprehensive country-wide studies to improve our understanding of the etiology of anti-GBM disease.
Objective
Obesity and many of its comorbidities can be improved by nutritional therapy, lifestyle modification, pharmacotherapy, and surgical intervention. Relatively little is known about patients' ...preferences for the range of obesity treatments. The present study was undertaken to identify factors that may influence these preferences. By evaluating patient‐preferred treatment options and factors influencing patients, treatment adherence and efficacy may be improved. Our objective was to identify factors that influence patient preferences and subsequent choice of obesity treatment among those seeking treatment for obesity‐related complications.
Methods
Participatory action research, using purposeful sampling, was used to recruit 33 patients with obesity complications. Recruitment took place in specialist clinics for non‐alcoholic fatty liver disease, diabetes, hypertension, and chronic kidney disease. Sixteen males and 17 females aged 18–70 years with a BMI>35 kg/m2 were recruited. Prior to the interview, participants watched a 60‐min video explaining nutritional therapies, pharmacotherapies, and surgical therapies in equipoise. Data were collected in one‐to‐one semi‐structured interviews using zoom or the telephone; reflective thematic analysis was used.
Results
Four themes emerged: 1) structural factors, 2) autonomy, 3) interaction with formal care, and 4) the emotional and physical consequences of obesity. 39% of participants preferred nutritional therapy with support from medical professionals. 27% chose bariatric surgery. 24% chose pharmacotherapy alone, while 6% chose pharmacotherapy combined with nutritional therapy, 3% of participants wanted no intervention.
Conclusion
The challenges can be addressed by increasing support for healthcare professionals toward enhancing both their knowledge and the health literacy of patients. Future research should focus on improving access to treatment pathways for patients as well as developing health literacy programs and educational programs for healthcare professionals.
By evaluating patient‐preferred treatment options and factors influencing them, treatment adherence and efficacy can be improved. This study found that the factors which influence decisions around treatment options for obesity complications include structural factors of healthcare setting, autonomy, interaction with formal care, and emotional and physical consequences of obesity. These challenges can be addressed by increasing support for healthcare professionals toward enhancing both their knowledge and the health literacy of patients. Future research on health policy should focus on improving access to treatment pathways for patients as well as developing health literacy programs and educational programs for healthcare professionals.
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
People with severe mental health difficulties (SMHDs) and concurrent kidney disease have less access to quality kidney care and worse clinical outcomes. Our research investigates the barriers and ...facilitators to effective kidney care for people with SMHDs, and how care might be improved for this underserved population.
We conducted semi-structured interviews with twenty-two physical (
= 14) and mental (
= 8) healthcare professionals with experience working with people with SMHDs and concurrent kidney disease. Interview data were analysed and interpreted using reflexive thematic analysis.
Four themes were generated from the data: 1.
describes how some people with SMHDs need additional support when accessing kidney care due to challenges with their mental state, motivation, cognitive difficulties, or mistrust of the healthcare system. 2. "
" describes how the separation of physical and mental healthcare, combined with under-resourcing and understaffing, results in poorer outcomes for people with SMHDs. 3. "
" describes how many renal healthcare providers have limited confidence in their understanding of mental health and their ability to provide care for people with SMHDs. 4. "
" describes how stigma towards people with SMHDs can negatively impact quality of care.
Healthcare professionals accounts' describe how people with SMHDs and kidney disease can have favourable outcomes if they have appropriate hospital, community and social supports. Findings indicate that effective management of kidney disease for people with SMHDs requires integrated physical and mental health care, which takes an individualised "whole person" approach to addressing the interaction between kidney disease and mental health.
Krüppel-like factor 6 (KLF6) is a DNA-binding protein containing a triple zinc-fingered motif and plays a key role in the regulation of cell proliferation, differentiation, and development. More ...recently it has been implicated in hepatic fibrosis via its binding to the transforming growth factor (TGF)-β control element. In the kidney, epithelial-mesenchymal transition (EMT) is a major contributor to the pathogenesis of renal fibrosis, with TGF-β1 being a key mediator of EMT. The present study aimed to determine the role of KLF6 and TGF-β1 in EMT in proximal tubule cells. To determine the relevance in clinical disease, KLF6 was measured in kidneys of streptozotocin-induced diabetic Ren-2 rats and in cells exposed to high (30 mM) glucose. TGF-β1 was confirmed to induce EMT by morphological change, loss of E-cadherin, and gain in vimentin expression. KLF6 mRNA expression was concomitantly measured. To determine the role of KLF6 in EMT, the above markers of EMT were determined in KLF6-silenced (small interfering RNA) and KLF6-overexpressing proximal tubule cells. KLF6 overexpression significantly promoted a phenotype consistent with EMT. High glucose induced KLF6 in proximal tubule cells ( P < 0.05). This increase in KLF6 in response to high glucose was TGF-β1 mediated. In an in vivo model of diabetic nephropathy KLF6 increased at week 8 ( P < 0.05). KLF6 plays a permissive role in TGF-β1-induced EMT in proximal tubule cells. Its upregulation in in vivo models of diabetic nephropathy suggests it as a potential therapeutic target.
We report a case of a 60 year-old female with persistent refractory hypomagnaesemia caused by HNF1B gene deletion. Her hypomagnaesemia had been attributed to the use of adjuvant platinum chemotherapy ...administered 2 years previously following a haemicolectomy for colorectal cancer. However it emerged that she had HNF1B gene deletion and had been diagnosed with MODY thirteen years previously. Her hypomagnaesemia was in fact longstanding and predated chemotherapy use. Previous abdominal imaging showed bilateral renal cysts and uterine didelphys which are consistent with anatomical features of HNF1B dysfunction. Her hypomagnaesemia is most likely related to HNF1-MODY. She continues to be managed with regular magnesium infusions and will likely require this as a lifelong measure. This case served as a reminder to look beyond medications as a potential cause for hypomagnesaemia and consider rare genetic conditions.
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NUK, OILJ, SAZU, UKNU, UL, UM, UPUK
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare multisystem autoimmune disease. There is a need for interoperable national registries to enable reporting of ...real-world long-term outcomes and their predictors in AAV.
The Irish National Rare Kidney Disease (RKD) registry was founded in 2012. To date, 842 patients with various forms of vasculitis have been recruited across eight nephrology, rheumatology and immunology centres. We focus here on patient- and disease- characteristics, treatment and outcomes of the 397 prospectively recruited patients with AAV.
Median age was 64 years (IQR 55-73), 57.9% were male, 58.9% had microscopic polyangiitis and 85.9% had renal impairment. Cumulative one- and five-year patient survival was 94% and 77% respectively. Median follow-up was 33.5 months (IQR 10.7-52.7). After controlling for age, baseline renal dysfunction (p = 0.04) and the burden of adverse events (p <0.001) were independent predictors of death overall. End-stage-kidney-disease (ESKD) occurred in 73 (18.4%) patients; one- and five-year renal survival was 85% and 79% respectively. Baseline severity of renal insufficiency (p = 0.02), urine soluble CD163 (usCD163) (p = 0.002) and "sclerotic" Berden histological class (p = 0.001) were key determinants of ESKD risk.
Long-term outcomes of Irish AAV patients are comparable to other reported series. Our results emphasise the need for personalisation of immunosuppression, to limit treatment toxicity, particularly in those with advanced age and renal insufficiency. Baseline usCD163 is a potential biomarker for ESKD prediction and should be validated in a large independent cohort.
A device for measuring human breath ammonia was developed based on a single use, disposable, inkjet printed ammonia sensor fabricated using polyaniline nanoparticles. The device was optimized for ...sampling ammonia in human breath samples by addressing issues such as variations in breath sample volume, flow rate, sources of oral ammonia, temperature and humidity. The resulting system was capable of measuring ammonia in breath from 40 to 2993 ppbv (r 2 = 0.99, n = 3) as correlated with photoacoustic laser spectroscopy and correlation in normal human breath samples yielded a slope of 0.93 and a Pearson correlation coefficient of 0.9705 (p < 0.05, n = 11). Measurement of ammonia in the breath of patients with end-stage kidney disease demonstrated its significant reduction following dialysis, while also correlating well with blood urea nitrogen (BUN) (r = 0.61, p < 0.01, n = 96). Excellent intraindividual correlations were demonstrated between breath ammonia and BUN (0.86 to 0.96), which demonstrates the possibility of using low cost point of care breath ammonia systems as a noninvasive means of monitoring kidney dysfunction and treatment.
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IJS, KILJ, NUK, PNG, UL, UM
Abstract
Background and Aims
Patients with ESRD have a 4-fold higher risk of fracture compared to the general population. (1) Transplant bone disease is a combination of mineral bone disease ...sustained pre transplantation and damage incurred post transplantation, risk factors for which include immunosuppression and immobility in the early post transplantation period. Given the high morbidity, mortality and financial burden associated with fractures, the management and prevent of osteoporosis is fundamental. (2) We aimed to compare the management of MBD in our patient cohort to international nephrology and rheumatology guidelines. (1, 3)
Method
A retrospective review of all patients post kidney transplantation attending our department was carried out. Information pertaining to patient demographics, renal history, osteoporosis investigation and management was collected. The results were analysed with Microsoft Excel.
Results
154 patient records (92 male, 62 female) were reviewed. Mean age was 52 years (range 19-80). Mean eGFR was 54ml/min (range 9-90). 82% of patients were taking corticosteroids, the mean dose was 5mg. 9% were taking corticosteroids pre-transplantation. 11 patients (7%) had a history of parathyroidectomy. 19% of patients had a history of a fracture. 26% of patients had a DEXA scan performed which demonstrated 50% had osteopenia and 29% had osteoporosis. The mean fracture risk assessment tool (FRAX) score (risk of having an osteoporosis-related fracture in the next 10 years) for a major osteoporotic fracture was 10.2% (range 2.1-34%) and for a hip fracture was 3.1% (range 0.2-18). Based on these scores 19% and 26% of patients respectively met the criteria for treatment. Overall, 58% of patients were on treatment for either osteoporosis prevention or management or for mineral bone disease. 74% of patients with osteopenia were on treatment (32% calcium supplementation, 5% alfacalcidol, and 63% cholecalciferol). 82% of patients with osteoporosis were on treatment (45% cinacalcet, 36% cholecalciferol, 18% bisphosphonate and 9% denosumab). 27% of patients with osteoporosis were managed in keeping the guidelines.
Conclusion
Our cohort have significant risk factors for osteoporosis including maintenance corticosteroid treatment and previous fractures. There is a high prevalence of osteoporosis in our cohort compared to that reported internationally (4) and they had a high predicted rate of future fractures. Increased screening with targeted treatment is required to reduce the risk of fractures in our post renal transplant patients.
ABSTRACT
Background
People with chronic kidney disease (CKD) experience high levels of psychological distress, which is associated with higher mortality and adverse health outcomes. Little is known ...about the rates of a range of mental health difficulties or rates of suicide attempts in people with CKD.
Methods
Individuals with CKD (n = 268; age range 18–94 years, mean = 49.96 years) on haemodialysis (n = 79), peritoneal dialysis (n = 46), transplant recipients (n = 84) and who were not on renal replacement therapy (RRT; n = 59) were recruited through the Irish Kidney Association social media pages and three Irish hospitals. Participants completed surveys to gather demographics and mental health histories, the Hospital Anxiety and Depression Scale (HADS) and the 12-item Short Form Health Survey (SF-12) to measure health-related quality of life (HRQoL).
Results
A total of 23.5% of participants self-reported they had received a mental health diagnosis, with depression (14.5%) and anxiety (14.2%) being the most common, while 26.4% of participants had experienced suicidal ideation and 9.3% had attempted suicide. Using a clinical cut-off ≥8 on the HADS subscales, current levels of clinically significant anxiety and depression were 50.7% and 35.4%, respectively. Depression levels were slightly higher for those on haemodialysis compared with those with a transplant and those not on RRT. Depression, anxiety and having a mental health diagnosis were all associated with lower HRQoL.
Conclusions
People with CKD in Ireland experience high levels of psychological distress, mental health difficulties, suicidal ideation and suicide attempts. The identification of and intervention for mental health difficulties in CKD should be prioritised in clinical care.
Graphical Abstract
Graphical Abstract
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