Search results for "Dam"
showing 10 items of 3828 documents
P172 Mainzer lupus score (MLS) a new questionnaire for lupus treatment
2020
Background For the diagnosis and treatment of the systemic lupus erythematosus (SLE), numerous scores and indices already exist with different focal points, which are mostly extensive and time consuming. The MLS is a short newly developed questionnaire consisting out of four items to consider whether it is necessary to adjust current therapy: Occurrence of new lupus-specific symptoms, subjective measurement of quality of life (Qol), change of serological abnormalities and the occurrence of organ damage accrual. Methods Patients who had at least 3 visits since 2016 in our clinic were able to participate. Spearman-correlation was used to correlate MLS and several other well established scores…
Efficacy and safety of open-label caplacizumab in patients with exacerbations of acquired thrombotic thrombocytopenic purpura in the HERCULES study.
2020
BACKGROUND Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Caplacizumab, an anti-von Willebrand Factor Nanobody® , is effective for treating aTTP episodes and is well tolerated. OBJECTIVES AND METHODS In the phase 3 HERCULES trial (NCT02553317), patients with aTTP received double-blind caplacizumab or placebo during daily therapeutic plasma exchange (TPE) and for ≥30 days thereafter. Patients who experienced an exacerbation while on blinded study drug treatment switched to receive open-label caplacizumab plus re-initiation of daily TPE. Exacerbations were defined as recurrence of disease occurring within 30 days after ce…
Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management
2021
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTT…
Algoritmo diagnóstico-terapéutico de las microangiopatías trombóticas. A propósito de 2 casos
2022
Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).
No Evidence for Classic Thrombotic Microangiopathy in COVID-19
2021
Background: Coronavirus disease-2019 (COVID-19) triggers systemic infection with involvement of the respiratory tract. There are some patients developing haemostatic abnormalities during their infection with a considerably increased risk of death. Materials and Methods: Patients (n = 85) with SARS-CoV-2 infection attending the University Medical Center, Mainz, from 3 March to 15 May 2020 were retrospectively included in this study. Data regarding demography, clinical features, treatment and laboratory parameters were analyzed. Twenty patients were excluded for assessment of disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) due to lack of laboratory data. Resu…
Predictors of Progression in Hypertensive Renal Disease in Children
2004
In hypertensive renal disease in children, several risk factors influence the development and the rate of progression of renal damage, including blood pressure levels, proteinuria, lipid disorders, and genetic differences. The impact of blood pressure on renal structures, the most important of the factors, depends not only on blood pressure levels, but also on the persistence of the blood pressure levels over time, mainly during the hours when the patient is resting or sleeping. Abnormal circadian variability is frequently observed in patients with renal damage, and nocturnal blood pressure reduction should be a major therapeutic objective to protect against a decline in renal function. Pro…
1177 The additive prognostic value of end-systolic pressure-volume relation by CMR in patients with with known or suspected coronary artery disease
2020
Abstract Background The variation between rest and peak stress end-systolic pressure-volume relation is an afterload-independent index of left ventricular contractility. This index is easily obtained during routine stress echocardiography but can be derived also during a stress cardiovascular magnetic resonance (CMR) exam, that is the gold standard for the quantification of biventricular volumes. Purpose The aim of this study was to assess for the first time the prognostic value ofdelta rest-stress ESPVR (DESPVR) by dipyridamole stress-CMR in patients with known or suspected coronary artery disease (CAD). Methods One hundred and sixty-six consecutive patients (37 females, main age 61.96 ± 1…
Hypertension in Children and Adolescents
2019
During the last few decades, hypertension in children and adolescents has gained ground in cardiovascular medicine, thanks to the progress made in several areas of pathophysiological and clinical research. The prevalence in school-aged children appears to be increasing, perhaps as a result of the increased prevalence of obesity in the last years. Despite evidence of an increasing prevalence of hypertension in the young, the consequences of early onset hypertension are poorly established and often overlooked. In children hypertension can be caused by multiple conditions, and therefore it is warranted to consider a wide range of differential diagnoses and to search for definable causes of hyp…
Patterns of changes of anti‐ADAMTS13 after plasma exchange
2006
An enzyme-linked immunosorbent assay (ELISA) has recently been developed to detect antibodies against the von Willebrand factor-cleaving protease ADAMTS13 in patients with thrombotic thrombocytopenic purpura (TTP). The ELISA is based on incubation of plasma with immobilized recombinant ADAMTS13 followed by visualization of IgG and IgM antibodies by means of secondary enzyme-labeled antibodies [1]. In a recent study, anti-ADAMTS13 IgG antibodies were detected in most patients with TTP (97%) characterized by severe ADAMTS13 deficiency (< 10% of normal) [1]. The ELISA was more sensitive than the standard inhibitor assay based upon ADAMTS13 neutralizing activity, which gave positive results in …
Cardiac arrhythmia induced by hypothermia in a cardiac model in vitro
2013
The neurological damage after cardiac arrest (CA) constitutes a big challenge of hospital discharge since years. The therapeutic hypothermia therapy (34°C-32°C) has shown its benefit to reduce cerebral oxygen demand and improve neurological outcomes after the cardiac arrest. Despite the fact that induced hypothermia after CA has been shown to increase the hospital survival rate, it can have many adverse effects, among which the cardiac arrhythmia generation represents an important part (up to 34%, according different clinical studies). Compared to studies in vivo, cardiac culture in vitro provides a better spatial resolution at cellular level, which could bring some insights of the mechanis…