Calciphylaxis epidemiology, risk factors, treatment and survival among French chronic kidney disease patients: a case-control study.
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ABSTRACT:
SUBMITTER: Gaisne R
PROVIDER: S-EPMC7045437 | biostudies-literature | 2020 Feb
REPOSITORIES: biostudies-literature
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Calciphylaxis epidemiology, risk factors, treatment and survival among French chronic kidney disease patients: a case-control study.
Gaisne Raphaël R Péré Morgane M Menoyo Victorio V Hourmant Maryvonne M Larmet-Burgeot David D
BMC nephrology 20200226 1
<h4>Background</h4>Calcific Uremic Arteriolopathy (CUA) is a rare disease, causing painful skin ulcers in patients with end stage renal disease. Recommendations for CUA management and treatment are lacking.<h4>Methods</h4>We conducted a retrospective cohort study on CUA cases identified in western France, in order to describe its management and outcome in average clinical practices. Selection was based on the Hayashi diagnosis criteria (2013) extended to patients with eGFR < 30 mL/min/1.73m<sup> ...[more]
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Project description:Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly seen in patients with chronic kidney failure treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal kidney function. In this review, we discuss the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and nonuremic calciphylaxis. High-quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence and poorly understood pathogenesis and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by a multidisciplinary team for patients with calciphylaxis.
| S-EPMC4696752 | biostudies-literature
Project description:BackgroundHealth care professionals are caught between the wish of patients to speed up health-related communication via emails and the need for protecting health information.ObjectiveWe aimed to analyze the demographic characteristics of patients providing an email, and study the distribution of emails' domain names.MethodsWe used the information system of the European Hospital Georges Pompidou (HEGP) to identify patients who provided an email address. We used a 1:1 matching strategy to study the demographic characteristics of the patients associated with the presence of an email, and described the characteristics of the emails used (in terms of types of emails-free, business, and personal).ResultsOverall, 4.22% (41,004/971,822) of the total population of patients provided an email address. The year of last contact with the patient is the strongest driver of the presence of an email address (odds ratio [OR] 20.8, 95% CI 18.9-22.9). Patients more likely to provide an email address were treated for chronic conditions and were more likely born between 1950 and 1969 (taking patients born before 1950 as reference [OR 1.60, 95% CI 1.54-1.67], and compared to those born after 1990 [OR 0.56, 95% CI 0.53-0.59]). Of the 41,004 email addresses collected, 37,779 were associated with known email providers, 31,005 email addresses were associated with Google, Microsoft, Orange, and Yahoo!, 2878 with business emails addresses, and 347 email addresses with personalized domain names.ConclusionsEmails have been collected only recently in our institution. The importance of the year of last contact probably reflects this recent change in contact information collection policy. The demographic characteristics and especially the age distribution are likely the result of a population bias in the hospital: patients providing email are more likely to be treated for chronic diseases. A risk analysis of the use of email revealed several situations that could constitute a breach of privacy that is both likely and with major consequences. Patients treated for chronic diseases are more likely to provide an email address, and are also more at risk in case of privacy breach. Several common situations could expose their private information. We recommend a very restrictive use of the emails for health communication.
| S-EPMC7946586 | biostudies-literature
Project description:Background and aimsCalciphylaxis is a rare condition associated with very high mortality in patients with end-stage kidney disease. Data from country-based registries have been an invaluable resource for a better understanding of the natural history and management for this condition. This study aimed to investigate the current management strategies and outcomes of patients enrolled in the United Kingdom Calciphylaxis study (UKCS).MethodsThe study was conducted on 89 patients registered in the UKCS since 2012. The initial analysis included a description of the baseline characteristics, management strategies and outcomes on follow-up until May 2020. Further analysis included a comparison of the mortality outcome of the UKCS patients who were receiving haemodialysis with a propensity score matched cohort of haemodialysis patients from the Chronic Renal Insufficiency Standards Implementation Study- Haemodialysis (CRISIS-HD).ResultsMedian age of the cohort was 59 years, with a predominance of females (61%) and Caucasian (95%) ethnicity. About 54% of the patients were diabetic and 70% were receiving haemodialysis at study entry. The skin lesions were mostly distributed in the lower extremities (48%). Sodium thiosulphate and calcimimetic were the most widely used management strategies. The mortality rate was 72 deaths per hundred patient-years (50 deaths observed in 69.5 patient years). Complete wound healing was noted in 17% and bacteraemia was reported in 26% of patients. In a comparative analysis of the matched haemodialysis patients, the presence of calciphylaxis in 62 patients showed a strong association with all-cause mortality (HR 6.96; p < 0.001), with annual mortality 67% versus 10.2% in haemodialysis patients without calciphylaxis.ConclusionsThis UK wide study strengthens the evidence that calciphylaxis is a strong and independent risk factor associated with all-cause mortality; no significant benefit was shown with any individual treatment modality. Until further evidence becomes available, a multifaceted approach would be the appropriate treatment strategy in the management of this extremely serious condition.
| S-EPMC8494680 | biostudies-literature
Project description:Objective The renal excretion function of patients with chronic kidney disease (CKD) is reduced, and the nervous system toxic reactions of antibiotics are prone to occur. The purpose of this study is to screen out some risk factors for patients with CKD to suffer from antibiotic-associated encephalopathy (AAE). Design A case–control study. Setting A tertiary hospital in China. Participants The medical records of patients who were hospitalised for CKD and infectious diseases in our hospital from January 2010 to December 2019. All patients used antibiotics to treat infectious diseases during hospitalisation. All patients were divided into two groups according to whether they developed AAE during hospitalisation. The patients with CKD without AAE were selected as the control group (n=120), and the patients with CKD with AAE were regarded as the AAE group (n=102). Interventions This study systematically analysed its clinical manifestations, laboratory examinations, prognosis, etc, and summarised the risk factors related to AAE in patients with CKD. Primary outcome Screening risk factors of AAE in patients with CKD. Results Logistic regression analysis showed that coronary heart disease, as well as abnormal indicators of haemoglobin, albumin, uric acid and blood phosphorus were independent risk factors for patients with CKD with AAE (OR values were 4.137, 0.963, 0.849, 0.996 0.161, respectively, all p<0.05). The case fatality rate (Pearson χ2=7.524, p=0.006), rehospitalisation rate (Pearson χ2=6.187, p=0.013) and treatment costs (t=−8.44, p<0.001) in encephalopathy group are significantly higher than the control group. Conclusions Patients with CKD with AAE will increase the case fatality rate and cause poor prognosis. Coronary heart disease, as well as decreased levels of haemoglobin, albumin, uric acid, and blood phosphorus are independent risk factors for patients with CKD with AAE. Timely intervention of these risk factors may reduce the incidence of AAE and improve the prognosis.
| S-EPMC9764618 | biostudies-literature
Project description:ImportanceCalciphylaxis is a rare disease with high mortality mainly involving patients with chronic kidney disease (CKD). Sodium thiosulphate (STS) has been used as an off-label therapeutic in calciphylaxis, but there is a lack of clinical trials and studies that demonstrate its effect compared with those without STS treatment.ObjectiveTo perform a meta-analysis of the cohort studies that provided data comparing outcomes among patients with calciphylaxis treated with and without intravenous STS.Data sourcesPubMed, Embase, Cochrane Library, Web of Science, and ClinicalTrials.gov were searched using relevant terms and synonyms including sodium thiosulphate and calci* without language restriction.Study selectionThe initial search was for cohort studies published before August 31, 2021, that included adult patients diagnosed with CKD experiencing calciphylaxis and could provide a comparison between patients treated with and without intravenous STS. Studies were excluded if they reported outcomes only from nonintravenous administration of STS or if the outcomes for CKD patients were not provided.Data extraction and synthesisRandom-effects models were performed. The Egger test was used to measure publication bias. Heterogeneity was assessed using the I2 test.Main outcomes and measuresSkin lesion improvement and survival, synthesized as ratio data by a random-effects empirical Bayes model.ResultsAmong the 5601 publications retrieved from the targeted databases, 19 retrospective cohort studies including 422 patients (mean age, 57 years; 37.3% male) met the eligibility criteria. No difference was observed in skin lesion improvement (12 studies with 110 patients; risk ratio, 1.23; 95% CI, 0.85-1.78) between the STS and the comparator groups. No difference was noted for the risk of death (15 studies with 158 patients; risk ratio, 0.88; 95% CI, 0.70-1.10) and overall survival using time-to-event data (3 studies with 269 participants; hazard ratio, 0.82; 95% CI, 0.57-1.18). In meta-regression, lesion improvement associated with STS negatively correlated with publication year, implying that recent studies are more likely to report a null association compared with past studies (coefficient = -0.14; P = .008).Conclusions and relevanceIntravenous STS was not associated with skin lesion improvement or survival benefit in patients with CKD experiencing calciphylaxis. Future investigations are warranted to examine the efficacy and safety of therapies for patients with calciphylaxis.
| S-EPMC10134003 | biostudies-literature
Project description:POEMS Syndrome is a constellation of findings including Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Calciphylaxis, a microangiopathy involving vascular calcification and thrombotic occlusions, occurs rarely in POEMS. We present a case of prominent calciphylaxis that antedated the diagnosis of POEMS. The patient presented with extensive ecchymoses progressing to necrotic lesions in the setting of acute renal injury. Previously, she had chronic slowly progressive polyneuropathy, splenomegaly, hypothyroidism, amenorrhea, and ascites. Calciphylaxis was diagnosed on skin biopsy, and POEMS was diagnosed based upon clinical findings plus a bone marrow biopsy showing 15% lambda chain restricted plasma cells. Treatment for the calciphylaxis was supportive with fluids, tissue debridement, wound vacuum devices and antibiotics for secondary infection. Myeloma was treated with bortezomib and steroids. All aspects of the patient's manifestations improved. We conclude that calciphylaxis can be a prominent feature of POEMS and can appear prior to recognition of the full-blown syndrome.
| S-EPMC10907981 | biostudies-literature
Project description:Chronic kidney disease (CKD) is a complex condition with a prevalence of 10-15% worldwide. An inverse-graded relationship exists between cardiovascular events and mortality with kidney function which is independent of age, sex, and other risk factors. The proportion of deaths due to heart failure and sudden cardiac death increase with progression of chronic kidney disease with relatively fewer deaths from atheromatous, vasculo-occlusive processes. This phenomenon can largely be explained by the increased prevalence of CKD-associated cardiomyopathy with worsening kidney function. The key features of CKD-associated cardiomyopathy are increased left ventricular mass and left ventricular hypertrophy, diastolic and systolic left ventricular dysfunction, and profound cardiac fibrosis on histology. While these features have predominantly been described in patients with advanced kidney disease on dialysis treatment, patients with only mild to moderate renal impairment already exhibit structural and functional changes consistent with CKD-associated cardiomyopathy. In this review we discuss the key drivers of CKD-associated cardiomyopathy and the key role of hypertension in its pathogenesis. We also evaluate existing, as well as developing therapies in the treatment of CKD-associated cardiomyopathy.
| S-EPMC9831930 | biostudies-literature
Project description:Importance:Calciphylaxis is a rare skin disease with high morbidity and mortality that frequently affects patients with renal disease. Hypercoagulable conditions are frequently observed in both patients with calciphylaxis and those with chronic kidney disease (CKD), complicating our understanding of which hypercoagulable conditions are specific to calciphylaxis. Objective:To identify hypercoagulable conditions that are risk factors for developing calciphylaxis while controlling for CKD. Design, Setting, and Participants:This was a case-control study, comparing the hypercoagulability status of patients with calciphylaxis and with renal disease with that of a matched control population at 2 large urban academic hospitals in Boston, Massachusetts. Retrospective medical record review of laboratory values was performed to identify patients with hypercoagulable conditions. Case and control patients were further stratified based on both severity of CKD and warfarin. Patients with a dermatologic diagnosis of calciphylaxis between 2006 and 2014 and concomitant CKD were included as cases (n?=?38). Three controls (n?=?114) per case patient with CKD were included, and were matched by age, sex, and race. Main Outcomes and Measures:The rate of various hypercoagulable states (ie, antithrombin III [ATIII] deficiency, protein C and S deficiency, factor V Leiden mutation, prothrombin gene mutation [G20210A], elevated factor VIII level, lupus anticoagulant, anti-IgG or IgM cardiolipin antibodies, heparin-induced thrombocytopenia antibodies, and elevation of homocysteine) in patients with calciphylaxis compared with their matched controls. Results:Of the calciphylaxis cohort, 28 (58%) were female and 18 (55%) were non-Hispanic white. Among all patients, lupus anticoagulant (13 [48%] positive in cases vs 1 [5%] in controls; P?=?.001), protein C deficiency (9 [50%] vs 1 [8%]; P?=?.02), and combined thrombophilias (18 [62%] vs 10 [31%]; P?=?.02) were found to be significantly associated with calciphylaxis. In a subanalysis of patients with stage 5 CKD, only lupus anticoagulant (12 [53%] vs 9 [0%]; P?=?.01) and combined thrombophilia (15 [63%] vs 1 [8%]; P?=?.004) remained significantly associated with calciphylaxis. In a separate subanalysis of warfarin-unexposed patients, only lupus anticoagulant (7 [50%] vs 1 [6%]; P?=?.01) and protein C deficiency (5 [46%] vs 10 [0%]; P?=?.04) remained significantly associated with calciphylaxis. Conclusions and Relevance:Presence of lupus anticoagulant and combined thrombophilias are risk factors for the development of calciphylaxis in patients with late-stage renal disease. Clinicians should be aware of these associations in patients with impaired kidney function and may consider increased screening and appropriate anticoagulation treatment to reduce the risk of calciphylaxis development.
| S-EPMC5839276 | biostudies-literature
Project description:In the past 30 years there have been major improvements in the care of children with chronic kidney disease (CKD). However, most of the available epidemiological data stem from end-stage renal disease (ESRD) registries and information on the earlier stages of pediatric CKD is still limited. The median reported incidence of renal replacement therapy (RRT) in children aged 0-19 years across the world in 2008 was 9 (range: 4-18) [corrected] per million of the age-related population). [corrected] The prevalence of RRT in 2008 ranged from 18 to 100 per million of the age-related population. Congenital disorders, including congenital anomalies of the kidney and urinary tract (CAKUT) and hereditary nephropathies, are responsible for about two thirds of all cases of CKD in developed countries, while acquired causes predominate in developing countries. Children with congenital disorders experience a slower progression of CKD than those with glomerulonephritis, resulting in a lower proportion of CAKUT in the ESRD population compared with less advanced stages of CKD. Most children with ESRD start on dialysis and then receive a transplant. While the survival rate of children with ERSD has improved, it remains about 30 times lower than that of healthy peers. Children now mainly die of cardiovascular causes and infection rather than from renal failure.
| S-EPMC3264851 | biostudies-literature
Project description:BACKGROUND: Increasing prevalence of ertapenem-resistant, carbapenem-susceptible Enterobacteriaceae (ERE) in Singapore presents a major therapeutic problem. Our objective was to determine risk factors associated with the acquisition of ERE in hospitalized patients; to assess associated patient outcomes; and to describe the molecular characteristics of ERE. METHODS: A retrospective case-case-control study was conducted in 2009 at a tertiary care hospital. Hospitalized patients with ERE and those with ertapenem-sensitive Enterobacteriaceae (ESE) were compared with a common control group consisting of patients with no prior gram-negative infections. Risk factors analyzed included demographics; co-morbidities; instrumentation and antibiotic exposures. Two parallel multivariate logistic regression models were performed to identify independent variables associated with ERE and ESE acquisition respectively. Clinical outcomes were compared between ERE and ESE patients. RESULTS: Twenty-nine ERE cases, 29 ESE cases and 87 controls were analyzed. Multivariate logistic regression showed that previous hospitalization (Odds ratio [OR], 10.40; 95% confidence interval [CI], 2.19-49.20) and duration of fluoroquinolones exposure (OR, 1.18 per day increase; 95% CI, 1.05-1.34) were unique independent predictors for acquiring ERE. Duration of 4(th)-generation cephalosporin exposure was found to predict for ESE acquisition (OR, 1.63 per day increase; 95% CI, 1.05-2.54). In-hospital mortality rates and clinical response rates were significantly different between ERE and ESE groups, however ERE infection was not a predictor of mortality. ERE isolates were clonally distinct. Ertapenem resistance was likely to be mediated by the presence of extended-spectrum β-lactamases or plasmid-borne AmpC in combination with impermeability due to porin loss and/or efflux pumps. CONCLUSION: Prior hospitalization and duration of fluoroquinolone treatment were predictors of ERE acquisition. ERE infections were associated with higher mortality rates and poorer clinical response rates when compared to ESE infections.
| S-EPMC3312905 | biostudies-other