Kooperativer Bibliotheksverbund

Berlin Brandenburg

and
and

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
Filter
  • Abridged
Type of Medium
Language
Year
  • 1
    Article
    Article
    BMJ Publishing Group Ltd and British Cardiovascular Society
    Language: English
    In: Heart, 11 November 2012, Vol.98(21), p.1546
    Description: Cardiac amyloidosis of transthyretin fibril protein (ATTR) type is an infiltrative cardiomyopathy characterised by ventricular wall thickening and diastolic heart failure. Increased access to cardiovascular magnetic resonance imaging has led to a marked increase in referrals to our centre of Caucasian patients with wild-type ATTR (senile systemic) amyloidosis and Afro-Caribbean patients with the hereditary ATTR V122I type. Both subtypes present predominantly as isolated cardiomyopathy. The differential diagnosis includes cardiac amyloid light-chain (AL) amyloidosis, which has a poorer prognosis and can be amenable to chemotherapy. We review here the clinical features of cardiac ATTR amyloidosis and describe the diagnostic tests to determine ATTR type. Correct diagnosis is ever more crucial given that several novel therapies for ATTR amyloidosis are on the near horizon.
    Keywords: Cardiac Amyloidosis ; Cardiovascular Mri ; Cardiomyopathy ; Valvular Disease ; Cardiac Function ; Cardiac Remodelling ; Interventional Cardiology ; Non-Coronary Intervention ; Percutaneous Valve Therapy ; Heart Failure ; Systolic Heart Failure ; Diastolic Dysfunction ; Myocardial Disease ; Cardiomyopathy Restrictive
    ISSN: 1355-6037
    ISSN: 13556037
    E-ISSN: 1468-201X
    E-ISSN: 1468201X
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 2
    Language: English
    In: The Lancet, 25 June 2016, Vol.387(10038), pp.2641-2654
    Description: Tissue deposition of protein fibrils causes a group of rare diseases called systemic amyloidoses. This Seminar focuses on changes in their epidemiology, the current approach to diagnosis, and advances in treatment. Systemic light chain (AL) amyloidosis is the most common of these conditions, but wild-type transthyretin cardiac amyloidosis (ATTRwt) is increasingly being diagnosed. Typing of amyloid fibrils, a critical determinant of therapy, has improved with the wide availability of laser capture and mass spectrometry from fixed histological tissue sections. Specific and accurate evaluation of cardiac amyloidosis is now possible using cardiac magnetic resonance imaging and cardiac repurposing of bone scintigraphy tracers. Survival in AL amyloidosis has improved markedly as novel chemotherapy agents have become available, but challenges remain in advanced disease. Early diagnosis, a key to better outcomes, still remains elusive. Broadening the amyloid-specific therapeutic landscape to include RNA inhibitors, fibril formation stabilisers and inhibitors, and immunotherapeutic targeting of amyloid deposits holds promise to transform outcomes in systemic amyloidoses.
    Keywords: Medicine
    ISSN: 0140-6736
    E-ISSN: 1474-547X
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 3
    In: Circulation, 2015, Vol.132(16), pp.1570-1579
    Description: BACKGROUND—: The prognosis and treatment of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardiac involvement. Cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is a reference standard for the diagnosis of cardiac amyloidosis, but its potential for stratifying risk is unknown. METHODS AND RESULTS—: Two hundred fifty prospectively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic resonance. Subjects were followed up for a mean of 24±13 months. LGE was performed with phase-sensitive inversion recovery (PSIR) and without (magnitude only). These were compared with extracellular volume measured with T1 mapping. PSIR was superior to magnitude-only inversion recovery LGE because PSIR always nulled the tissue (blood or myocardium) with the longest T1 (least gadolinium). LGE was classified into 3 patterns: none, subendocardial, and transmural, which were associated with increasing amyloid burden as defined by extracellular volume (P〈0.0001), with transitions from none to subendocardial LGE at an extracellular volume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR). Sixty-seven patients (27%) died. Transmural LGE predicted death (hazard ratio, 5.4; 95% confidence interval, 2.1–13.7; P〈0.0001) and remained independent after adjustment for N-terminal pro-brain natriuretic peptide, ejection fraction, stroke volume index, E/E′, and left ventricular mass index (hazard ratio, 4.1; 95% confidence interval, 1.3–13.1; P〈0.05). CONCLUSIONS—: There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after adjustment for known prognostic factors.
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 4
    Language: English
    In: Journal of the American College of Cardiology, 30 January 2018, Vol.71(4), pp.463-464
    Description: Wild-type transthyretin amyloid (wtATTR) affects the heart, causing a restrictive cardiomyopathy-deposits can be found in up to 25% of individuals 〉85 years of age at autopsy (1,2). [...]recently, diagnosis required endomyocardial biopsy. [...]the amyloid burden was skewed: rather than a pyramidal distribution...
    Keywords: Medicine
    ISSN: 0735-1097
    E-ISSN: 1558-3597
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 5
    Language: English
    In: Heart, 1 October 2012, Vol.98(19), p.1436
    Description: To measure and assess the significance of myocardial extracellular volume (ECV), determined non-invasively by equilibrium contrast cardiovascular magnetic resonance, as a clinical biomarker in health and a number of cardiac diseases of varying pathophysiology.
    Keywords: Cmr ; Gadolinium ; Extracellular Volume ; Arrhythmias ; Defibrillation ; Cardiac Function ; Imaging and Diagnostics ; Mri ; Myocardial Disease ; Myocardial Fibrosis ; Myocardial Ischaemia and Infarction (Ihd) ; Sudden Adult Death Syndrome ; Cardiomyopathy Hypertrophic ; Arrhythmic Right Ventricular Dysplasia ; Congenital Heart Disease ; Thoracic Imaging ; Cardiac Anatomy ; Paediatric Cardiac Function ; Myocardial Infarction ; Myocytes ; Apoptosis ; Ischaemia Reperfusion ; Myocardial Protection ; Metabolic Medicine ; Diabetic Heart Disease ; Cardiomyopathy ; Cardiomyopathy Apical ; Cardiomyopathy Restrictive ; Hypertrophic Cardiomyopathy ; Cardiac Imaging ; Mri ; Editor'S Choice
    ISSN: 1355-6037
    ISSN: 13556037
    E-ISSN: 1468-201X
    E-ISSN: 1468201X
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 6
    Language: English
    In: Radiology, September 2013, Vol.268(3), pp.858-64
    Description: To investigate equilibrium contrast material-enhanced magnetic resonance (MR) imaging measurement of extracellular volume (ECV) fraction within healthy abdominal tissues and to test the hypotheses that tissue ECV in systemic amyloid light-chain (AL) amyloidosis is greater than in healthy patients and show that this increase correlates with organ amyloid burden. A local ethics committee approved the study and all patients gave written informed consent. Forty healthy volunteers (18 men, 22 women; median age, 43 years; age range, 24-88 years) and 67 patients with AL amyloidosis (43 men, 24 women; median age, 65 years; age range, 38-81 years) underwent equilibrium MR imaging of the upper abdomen. ECV was measured in the liver, spleen, and paravertebral muscle. Patients with amyloidosis also underwent serum amyloid P (SAP) component scintigraphy so that specific organ involvement by amyloid could be scored. Variation in ECV between tissues was assessed by using a Friedman Test. Tissue ECV in healthy and amyloidosis groups were compared by using a Mann-Whitney U test. Spearman correlation was used to test for an association between the organ SAP score and ECV. ECV measured at equilibrium MR imaging varied significantly between organs in healthy volunteers (χ(2) = 31.0; P 〈 .001). ECV was highest in the spleen (0.34), followed by liver (0.29) and muscle (0.09). ECVs measured within the spleen (0.39; P〈 .001), liver (0.31; P = .005), and muscle (0.16; P〈 .001) were significantly higher in patients with amyloidosis than in healthy control subjects. ECV measured in the liver and spleen showed increasing organ amyloid burden assessed at SAP scintigraphy (liver, rs = 0.54; spleen, rs = 0.57). Equilibrium MR imaging can be used to define ECV within healthy tissues. ECV is increased in amyloidosis compared with healthy tissues, and this increase correlates with rising tissue amyloid burden.
    Keywords: Meglumine ; Organometallic Compounds ; Amyloidosis -- Pathology ; Extracellular Fluid -- Cytology ; Image Enhancement -- Methods ; Liver -- Pathology ; Magnetic Resonance Imaging -- Methods ; Spleen -- Pathology
    ISSN: 00338419
    E-ISSN: 1527-1315
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 7
    In: The New England Journal of Medicine, 2005, Vol.352(22), pp.2356-2356
    Description: To the Editor: Familial amyloidotic polyneuropathy is a fatal autosomal dominant disease caused by amyloidogenic genetic variants of transthyretin. The liver is the predominant source of circulating transthyretin, and liver transplantation is the only treatment available for the disease. 1 Livers explanted from patients with familial amyloidotic polyneuropathy contain only microscopic amyloid deposits in hilar vessels and nerves and are otherwise uninvolved. Since 1995, more than 300 such livers removed at transplantation have been used sequentially as donor grafts for recipients with liver cancer or end-stage liver disease, in so-called domino liver transplantation. 2 We report here a case of systemic transthyretin . . .
    Keywords: Adult–Etiology ; Amyloid Neuropathies, Familial–Surgery ; Carcinoma, Hepatocellular–Complications ; Hepatitis C–Chemistry ; Humans–Surgery ; Liver–Adverse Effects ; Liver Neoplasms–Analysis ; Liver Transplantation–Analysis ; Male–Analysis ; Middle Aged–Analysis ; Prealbumin–Analysis ; Prealbumin;
    ISSN: 0028-4793
    E-ISSN: 1533-4406
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 8
    In: The New England Journal of Medicine, 2013, Vol.369(9), pp.819-829
    Description: Background Transthyretin amyloidosis is caused by the deposition of hepatocyte-derived transthyretin amyloid in peripheral nerves and the heart. A therapeutic approach mediated by RNA interference (RNAi) could reduce the production of transthyretin. Methods We identified a potent antitransthyretin small interfering RNA, which was encapsulated in two distinct first- and second-generation formulations of lipid nanoparticles, generating ALN-TTR01 and ALN-TTR02, respectively. Each formulation was studied in a single-dose, placebo-controlled phase 1 trial to assess safety and effect on transthyretin levels. We first evaluated ALN-TTR01 (at doses of 0.01 to 1.0 mg per kilogram of body weight) in 32 patients with transthyretin amyloidosis and then evaluated ALN-TTR02 (at doses of 0.01 to 0.5 mg per kilogram) in 17 healthy volunteers. Results Rapid, dose-dependent, and durable lowering of transthyretin levels was observed in the two trials. At a dose of 1.0 mg per kilogram, ALN-TTR01 suppressed transthyretin, with a mean reduction at day 7 of 38%, as compared with placebo (P=0.01); levels of mutant and nonmutant forms of transthyretin were lowered to a similar extent. For ALN-TTR02, the mean reductions in transthyretin levels at doses of 0.15 to 0.3 mg per kilogram ranged from 82.3 to 86.8%, with reductions of 56.6 to 67.1% at 28 days (P〈0.001 for all comparisons). These reductions were shown to be RNAi-mediated. Mild-to-moderate infusion-related reactions occurred in 20.8% and 7.7% of participants receiving ALN-TTR01 and ALN-TTR02, respectively. Conclusions ALN-TTR01 and ALN-TTR02 suppressed the production of both mutant and nonmutant forms of transthyretin, establishing proof of concept for RNAi therapy targeting messenger RNA transcribed from a disease-causing gene. (Funded by Alnylam Pharmaceuticals; ClinicalTrials.gov numbers, NCT01148953 and NCT01559077 .) Transthyretin amyloidosis is largely caused by synthesis of mutant transthyretin in the liver and deposition of transthyretin in other organs. A therapeutic approach mediated by RNA interference resulted in reduced transthyretin levels in affected patients and in controls. Transthyretin amyloidosis is a life-threatening disorder caused by the deposition of hepatocyte-derived transthyretin amyloid in various tissues and organs.1,2 Circulating transthyretin is derived from the liver3 and can form amyloid deposits in peripheral nerves and in the gastrointestinal tract, heart, and kidneys. Transthyretin is also synthesized by the retina and choroid plexus,4,5 which can lead to vitreal and leptomeningeal deposits. More than 100 genetic variants of the gene encoding transthyretin (TTR) are associated with autosomal dominant forms of the disease, known as familial amyloidotic polyneuropathy6–8 and familial amyloidotic cardiomyopathy.9–11 The most common mutation associated . . .
    Keywords: Medicine;
    ISSN: 0028-4793
    E-ISSN: 1533-4406
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 9
    In: Rheumatology, 2017, Vol. 56(9), pp.1484-1491
    Description: Objectives: Pneumococcal, tetanus and influenza vaccinations are recommended for patients with cryopyrin-associated periodic syndromes (CAPS) when treated with immunosuppressive medication. The aim of this publication is to report the safety of pneumococcal and other vaccinations in CAPS patients.Methods: All CAPS patients followed in the β-CONFIDENT (Clinical Outcomes and Safety Registry study of Ilaris patients) registry were analysed if they had received a vaccination. The β-CONFIDENT registry is a global, long-term, prospective, observational registry, capturing and monitoring patients treated with canakinumab.Results: Sixty-eight CAPS patients had received a total of 159 vaccine injections, 107 injections against influenza, 19 pneumococcal vaccinations, 12 against tetanus/diphtheria antigens and 21 other vaccinations. Fourteen per cent of injections had elicited at least one vaccine reaction. All five vaccine-related serious adverse events were associated with pneumococcal vaccination. Vaccine reactions were observed in 70% of pneumococcal vaccinations, compared with 7% in influenza and 17% in tetanus/diphtheria vaccinations. The odds ratios to react to the pneumococcal vaccines compared with influenza and tetanus/diphtheria vaccines were 31.0 (95% CI: 8, 119) and 10.8 (95% CI: 2, 74). Vaccine reactions after pneumococcal vaccinations were more severe and lasted significantly longer (up to 3 weeks) compared with other vaccinations. In two patients, pneumococcal vaccination also elicited symptoms consistent with systemic inflammation due to CAPS reactivation.Conclusion: Pneumococcal vaccines, unlike other vaccines, frequently trigger severe local and systemic inflammation in CAPS patients. Clinicians must balance potential benefits of pneumococcal immunization against safety concerns. The 13-valent pneumococcal conjugate vaccine might be favourable over the polysaccharide vaccine in CAPS patients.
    Keywords: Cryopyrin - Associated Periodic Syndromes ; Caps ; Vaccinations ; Safety
    ISSN: 1462-0324
    E-ISSN: 1462-0332
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 10
    In: Circulation, 2016, Vol.133(12), pp.e450-e451
    Description: We thank Aquaro, Cohen, and colleagues for their interest in our article.1 Cardiac involvement is a chief driver of prognosis in systemic amyloidosis, and the stratification of patients is essential for prognosis and choosing management strategies. Cardiovascular magnetic resonance with late gadolinium enhancement has good diagnostic accuracy for cardiac amyloidosis, but its prognostic impact was uncertain.2–6 This study confirms incremental prognostic information after adjusting for known prognosis factors. We note the comments of Aquaro and colleagues regarding cardiac biopsy, but point out that microscopic histological analyses of these tiny samples is not only open to sampling error, but, crucially, the presence of amyloid in heart muscle is not actually proof of cardiac amyloidosis. It is essential to make the distinction between the presence of amyloid deposits and the clinical syndromes of amyloidosis. Amyloid deposits occur widely throughout the tissues in patients with systemic amyloidosis, often without any clinical consequences, providing the basis for rectal, salivary gland, skin, and fat biopsies to support diagnosis. It is likely …
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
Close ⊗
This website uses cookies and the analysis tool Matomo. Further information can be found on the KOBV privacy pages