38 research outputs found
Prognosis of Transthyretin Cardiac Amyloidosis Without Heart Failure Symptoms.
BACKGROUND
Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly recognized as a treatable cause of heart failure (HF). Advances in diagnosis and therapy have increased the number of patients diagnosed at early stages, but prognostic data on patients without HF symptoms are lacking. Moreover, it is unknown whether asymptomatic patients benefit from early initiation of transthyretin (TTR) stabilizers.
OBJECTIVES
The aim of this study was to describe the natural history and prognosis of ATTR-CM in patients without HF symptoms.
METHODS
Clinical characteristics and outcomes of patients with ATTR-CM without HF symptoms were retrospectively collected at 6 international amyloidosis centers.
RESULTS
A total of 118 patients (78.8% men, median age 66 years [IQR: 53.8-75 years], 68 [57.6%] with variant transthyretin amyloidosis, mean left ventricular ejection fraction 60.5% ± 9.9%, mean left ventricular wall thickness 15.4 ± 3.1 mm, and 53 [45%] treated with TTR stabilizers at baseline or during follow-up) were included. During a median follow-up period of 3.7 years (IQR: 1-6 years), 38 patients developed HF symptoms (23 New York Heart Association functional class II and 14 functional class III or IV), 32 died, and 2 required cardiac transplantation. Additionally, 20 patients received pacemakers, 13 developed AF, and 1 had a stroke. Overall survival was 96.5% (95% CI: 91%-99%), 90.4% (95% CI: 82%-95%), and 82% (95% CI: 71%-89%) at 1, 3, and 5 years, respectively. Treatment with TTR stabilizers was associated with improved survival (HR: 0.31; 95% CI: 0.12-0.82; P = 0.019) and remained significant after adjusting for sex, age, ATTR-CM type, and estimated glomerular filtration rate (HR: 0.18; 95% CI: 0.06-0.55; P = 0.002).
CONCLUSIONS
After a median follow-up period of 3.7 years, 1 in 3 patients with asymptomatic ATTR-CM developed HFÂ symptoms, and nearly as many died or required cardiac transplantation. Treatment with TTR stabilizers was associated with improved prognosis.This work was supported by grants from Instituto de Salud Carlos III
(PI18/0765 and PI20/01379). Dr Gonzalez-Lopez has received speaker
fees from Pfizer and Alnylam; has received consulting fees from Pfizer
and Proclara; and has received research and educational support to
her institution from Pfizer, BridgeBio, and Alnylam. Dr Obici has
received speaker and consulting fees from Pfizer, Alnylam, and
Akcea. Dr AbouEzzeddine has received research grant support from
Pfizer. Dr Mussinelli has received speaker fees from Pfizer and Akcea.
Dr Dispenzieri has received consulting fees from Janssen and Akcea;
and has received research support from Pfizer, Alnylam, Celgene, and
Takeda. Dr Perlini has received speaker and consulting fees from
Pfizer, Alnylam, and Akcea. Dr Palladini has received speaker fees
from Janssen-Cilag, Pfizer, and Siemens; and has participated on an
advisory board for Janssen Cilag. Dr Damy has received research
grants or consulting fees from Alnylam, Akcea, Pfizer, and Prothena.
Dr Grogan has received research grant support and consulting fees to
her institution from Alnylam, Eidos, Pfizer, and Prothena. Dr Maurer
has received grant support from National Institutes of Health
(R01HL139671-01, R21AG058348, and K24AG036778); has received
consulting income from Pfizer, GlaxoSmithKline, Eidos, Prothena,
Akcea, and Alnylam; and has received clinical trial funding to his
institution from Pfizer, Prothena, Eidos, and Alnylam. Dr Garcia-Pavia
has received speaker fees from Pfizer, BridgeBio, Alnylam, and Ionis;
has received consulting fees from Pfizer, BridgeBio, AstraZeneca,
NovoNordisk, Neuroimmune, Alnylam, Alexion, and Attralus; and has
received research and educational support to his institution from
Pfizer, BridgeBio, and Alnylam. All other authors have reported that
they have no relationships relevant to the contents of this paper to
disclose.S
The production of a physiological puzzle: how Cytisus adami confused and inspired a century’s botanists, gardeners, and evolutionists
‘Adam’s laburnum’ (or Cytisus adami), produced by accident in 1825 by Jean-Louis Adam, a nurseryman in Vitry, became a commercial success within the plant trade for its striking mix of yellow and purple flowers. After it came to the attention of members of La Société d’Horticulture de Paris, the tree gained enormous fame as a potential instance of the much sought-after ‘graft hybrid’, a hypothetical idea that by grafting one plant onto another, a mixture of the two could be produced. As I show in this paper, many eminent botanists and gardeners, including Charles Darwin, both experimented with Adam’s laburnum and argued over how it might have been produced and what light, if any, it shed on the laws of heredity. Despite Jean-Louis Adam’s position and status as a nurseryman active within the Parisian plant trade, a surprising degree of doubt and scepticism was attached to his testimony on how the tree had been produced in his nursery. This doubt, I argue, helps us to trace the complex negotiations of authority that constituted debates over plant heredity in the early 19th century and that were introduced with a new generation of gardening and horticultural periodicals
Structure-oriented prediction in complex networks
Complex systems are extremely hard to predict due to its highly nonlinear interactions and rich emergent properties. Thanks to the rapid development of network science, our understanding of the structure of real complex systems and the dynamics on them has been remarkably deepened, which meanwhile largely stimulates the growth of effective prediction approaches on these systems. In this article, we aim to review different network-related prediction problems, summarize and classify relevant prediction methods, analyze their advantages and disadvantages, and point out the forefront as well as critical challenges of the field
ENTEROBACTERIES (NOUVEAU TEST DE DETECTION DE LA RESISTANCE PAR CEPHALOSPORINASE PLASMIDIQUE)
PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF
Sarah Dewis, The Loudons and the Gardening Press: A Victorian Cultural Industry. Farnham: Ashgate, 2014. Pp. xvi + 278. ISBN 978-1-4094-6922-3. £65.00 (hardback).
Le modèle du préfet français en Roumanie au xixe siècle
Dans son Message au trône de janvier 1865, l’Assemblée Roumaine faisait l’éloge des réformes institutionnelles accomplies pendant le règne du prince Alexandru Ioan Cuza : « Ces institutions, basées sur les principes éternels de 1789, sont très chères à notre nation et l’Assemblée […] assume devant Votre Majesté le devoir sacré de les défendre et de les conserver comme le bien le plus précieux, comme le patrimoine le plus saint de notre société ». Une des créations de l’année 1864 fut le préfe..