Calcific uraemic arteriolopathy.
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ABSTRACT:
SUBMITTER: Disbrow MB
PROVIDER: S-EPMC4693125 | biostudies-other | 2015 Aug
REPOSITORIES: biostudies-other
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Project description:The uraemic syndrome is a complex clinical picture developing in the advanced stages of chronic kidney disease, resulting in a myriad of complications and a high early mortality. This picture is to a significant extent defined by retention of metabolites and peptides that with a preserved kidney function are excreted or degraded by the kidneys. In as far as those solutes have a negative biological/biochemical impact, they are called uraemic toxins. Here, we describe the historical evolution of the scientific knowledge about uraemic toxins and the role played in this process by the European Uraemic Toxin Work Group (EUTox) during the last two decades. The earliest knowledge about a uraemic toxin goes back to the early 17th century when the existence of what would later be named as urea was recognized. It took about two further centuries to better define the role of urea and its link to kidney failure, and one more century to identify the relevance of post-translational modifications caused by urea such as carbamoylation. The knowledge progressively extended, especially from 1980 on, by the identification of more and more toxins and their adverse biological/biochemical impact. Progress of knowledge was paralleled and impacted by evolution of dialysis strategies. The last two decades, when insights grew exponentially, coincide with the foundation and activity of EUTox. In the final section, we summarize the role and accomplishments of EUTox and the part it is likely to play in future action, which should be organized around focus points like biomarker and potential target identification, intestinal generation, toxicity mechanisms and their correction, kidney and extracorporeal removal, patient-oriented outcomes and toxin characteristics in acute kidney injury and transplantation.
| S-EPMC8371716 | biostudies-literature
Project description:Calcific aortic stenosis (AS) is the most prevalent heart valve disorder in developed countries. It is characterized by progressive fibro-calcific remodelling and thickening of the aortic valve leaflets that, over years, evolve to cause severe obstruction to cardiac outflow. In developed countries, AS is the third-most frequent cardiovascular disease after coronary artery disease and systemic arterial hypertension, with a prevalence of 0.4% in the general population and 1.7% in the population >65 years old. Congenital abnormality (bicuspid valve) and older age are powerful risk factors for calcific AS. Metabolic syndrome and an elevated plasma level of lipoprotein(a) have also been associated with increased risk of calcific AS. The pathobiology of calcific AS is complex and involves genetic factors, lipoprotein deposition and oxidation, chronic inflammation, osteoblastic transition of cardiac valve interstitial cells and active leaflet calcification. Although no pharmacotherapy has proved to be effective in reducing the progression of AS, promising therapeutic targets include lipoprotein(a), the renin-angiotensin system, receptor activator of NF-?B ligand (RANKL; also known as TNFSF11) and ectonucleotidases. Currently, aortic valve replacement (AVR) remains the only effective treatment for severe AS. The diagnosis and staging of AS are based on the assessment of stenosis severity and left ventricular systolic function by Doppler echocardiography, and the presence of symptoms. The introduction of transcatheter AVR in the past decade has been a transformative therapeutic innovation for patients at high or prohibitive risk for surgical valve replacement, and this new technology might extend to lower-risk patients in the near future.
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Project description:BackgroundComplement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab.MethodsWe identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 10 9 /L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration.ResultsTwenty-nine patients with secondary aHUS (15 drug-induced, 8 associated with systemic diseases, 2 with postpartum, 2 with cancer-related, 1 associated with acute humoral rejection and 1 with intestinal lymphangiectasia) were included in this study. The reason to initiate eculizumab treatment was worsening of renal function and persistence of TMA despite treatment of the TMA cause and plasmapheresis. All patients showed severe MAHA and renal function impairment (14 requiring dialysis) prior to eculizumab treatment and 11 presented severe extrarenal manifestations. A rapid resolution of the TMA was observed in 20 patients (68%), 15 of them showing a ≥50% serum creatinine reduction at the last follow-up. Comprehensive genetic and molecular studies in 22 patients identified complement pathogenic variants in only 2 patients. With these two exceptions, eculizumab was discontinued, after a median of 8 weeks of treatment, without the occurrence of aHUS relapses.ConclusionShort treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition.
| S-EPMC5410989 | biostudies-literature
Project description:Ravulizumab (Ultomiris®), a humanized monoclonal antibody that inhibits complement protein C5, is indicated for the treatment of atypical haemolytic uraemic syndrome (aHUS) in several countries, including the USA and those of the EU. Ravulizumab has been re-engineered from eculizumab to extend its terminal elimination half-life, resulting in a more convenient maintenance dosage regimen of once every 4-8 weeks compared with once every 2-3 weeks for eculizumab. In single-arm phase 3 trials, ravulizumab resolved thrombotic microangiopathy in 54% and 78% of treatment-naïve adult and paediatric patients with aHUS, respectively, within 26 weeks. Ravulizumab was also effective in patients with postpartum aHUS and paediatric patients who responded to eculizumab and later switched to ravulizumab. Ravulizumab was generally well tolerated, with no unexpected safety events. The most common treatment-related adverse events with ravulizumab in treatment-naïve patients include headache, diarrhoea and vomiting. With its convenient once every 4-8 weeks maintenance regimen, ravulizumab is an important treatment option for aHUS in adult and paediatric patients.
| S-EPMC8052220 | biostudies-literature
Project description:Calcific tendonitis, or calcifying tendonitis, is a common disorder characterized by the multifocal accumulation of basic calcium phosphate crystals within the rotator cuff tendons. In most cases, the multifocal calcifications are located 1 to 2 cm from the insertion of the supraspinatus tendon on the greater tuberosity. The initial treatment should be nonoperative including oral anti-inflammatory medication and physical therapy. If this is unsuccessful, arthroscopic debridement of the deposit is effective. The technique used is an arthroscopic localization and debridement without associated subacromial decompression. The rotator cuff should be evaluated for partial- and full-thickness tears before and after the debridement of calcifications. If a partial- or full-thickness rotator cuff tendon tear is identified, it should be treated in a fashion consistent with those without associated calcium deposits. In our hands, tears 5 mm or greater in depth are repaired using a tendon-to-tendon or tendon-to-bone technique. Tears with less depth are debrided and then left alone. Arthroscopic debridement of calcific tendonitis can yield excellent functional results and high patient satisfaction.
| S-EPMC4044535 | biostudies-literature
Project description:BackgroundUraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy.Case presentationA 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain.ConclusionsUraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia.
| S-EPMC11010437 | biostudies-literature
Project description:ObjectiveTo identify prognostic factors for the effectiveness of needle aspiration of calcific deposits (NACD) for rotator cuff calcific tendinitis (RCCT) METHODS: One hundred forty-nine patients with symptomatic RCCT were included in a prospective cohort study. Pain (VAS), shoulder function (SST and DASH), and quality of life (EQ-5D) were assessed at baseline and at 3, 6, and 12 months post-NACD. Univariate analyses (independent t tests or Mann-Whitney U tests depending on the distribution of data) were performed to build a multivariable linear regression model. Stepwise regression analysis through backward elimination was performed to evaluate the effect of predefined prognostic factors on the outcome.ResultsPatients who underwent multiple NACD procedures had less reduction of pain (p?<?0.01). Furthermore, a larger reduction in VAS pain scores at 3 months post-NACD was associated with a larger reduction in VAS pain scores at 12 months (p?<?0.01). More improvement of SST and DASH scores at 3 months was associated with better SST, DASH, and EQ-5D scores at 12 months (p?<?0.01). Smaller-size calcific deposits were associated with less improvement of DASH (p?=?0.03) and EQ-5D scores (p?=?0.01). A longer duration of symptoms prior to NACD was associated with less improvement of EQ-5D scores (p?=?0.01).ConclusionsA good initial response after NACD is associated with better outcomes at 12 months. Patients with a longer duration of symptoms prior to NACD and patients who require multiple procedures showed inferior outcomes in terms of pain reduction and improvement of quality of life. Smaller-size calcific deposits are associated with a less favorable outcome of shoulder function and quality of life scores and might therefore be less susceptible for NACD.Key points• A good initial response to NACD is associated with a better outcome in the longer term. • A longer duration of symptoms and the need for multiple NACD procedures are associated with inferior outcomes. • Smaller-size calcific deposits seem less susceptible for NACD.
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Project description:Rotator cuff tendonitis is a very common diagnosis of the shoulder that usually responds to conservative management. However, calcific tendonitis occurs less frequently and often necessitates surgical intervention. The etiology and treatment options for this disorder remain controversial among orthopaedic shoulder specialists. Calcific tendonitis frequently presents within the supraspinatus tendon and rarely appears within the subscapularis tendon. We present a case and accompanying video technique of arthroscopic treatment of subscapularis calcific tendonitis.
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Project description:Calcific aortic valve disease (CAVD) is a highly prevalent cardiovascular disorder accounting for a rising economic and social burden on aging populations. In spite of continuing study on the pathophysiology of disease, there remain no medical therapies to prevent the progression of CAVD. The discovery of biomarkers represents a potentially complementary approach in stratifying risk and timing of intervention in CAVD and has the advantage of providing insight into causal factors for the disease. Biomarkers have been studied extensively in atherosclerotic cardiovascular disease, with success as additive for clinical and scientific purposes. Similar research in CAVD is less robust; however, the available studies of biomarkers in CAVD show promise for enhanced clinical decision making and identification of causal factors for the disease. This comprehensive review summarizes available established and novel biomarkers in CAVD, their contributions toward an understanding of pathophysiology, their potential clinical utility, and provides an outline to direct future research in the field.
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