Results 91 to 100 of about 141,957 (168)

Systemic amyloidosis in England: an epidemiological study. [PDF]

, 2013
Epidemiological studies of systemic amyloidosis are scarce and the burden of disease in England has not previously been estimated. In 1999, the National Health Service commissioned the National Amyloidosis Centre (NAC) to provide a national clinical ...
Smith, CJ, Banypersad, SM, Whelan, CJ, Venner, CP, Dungu, J, Pinney, JH, Lachmann, HJ, Taube, JB, Gillmore, JD, Gibbs, SD, Hawkins, PN, Wechalekar, AD   +11 more
core  

Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis

JACC: Advances
Background: Wild-type transthyretin amyloidosis (ATTRwt amyloidosis) is primarily diagnosed in elderly men but diagnoses in younger patients and women have recently increased.
Nerea Mora-Ayestaran, MD, Angela Dispenzieri, MD, Arnt V. Kristen, MD, Mathew S. Maurer, MD, Igor Diemberger, MD, Brian M. Drachman, MD, Martha Grogan, MD, Pritam Gupta, PhD, Oliver Glass, PhD, Leslie Amass, PhD, Pablo Garcia-Pavia, MD, PhD, Michele Emdin, Mazen Hanna, Olga Azevedo, Calogero Lino Cirami, Jose Gonzalez Costello, David Slosky, Henning Moelgaard, Jose Nativi Nicolau, Scott Hummel, Eun-Seok Jeon, Nowell Fine, Srinivas Murali, Edward Miller, Sanjiv Shah, Ronald Witteles, Marcia Waddington-Cruz, Daniel Lenihan, Yoshiki Sekijima, Johan Van Cleemput, Edileide de Barros Correia, Eve Cariou, Dianna Quan, Miriam Freimer, David Steidley, Anna Hüsing-Kabar, Violaine Plante-Bordeneuve, Hans Nienhuis, Jonas Wixner, Jeffrey Ralph, Hector Ventura, Sasa Zivkovic, Diego Delgado, Roberto Fernandéz Torrón, Stephen Gottlieb, William Cotts, Jose Tallaj, Robert Brunkhorst, Michael Polydefkis, Christopher Mueller, Carsten Tschoepe, Juan Gonzalez Moreno, Nitasha Sarswat, Jin Luo, James Tauras, Alberta Warner   +55 more
doaj   +1 more source

Diagnosis and treatment of gastrointestinal dysfunction in hereditary TTR amyloidosis

, 2019
Purpose: To review the management of gastrointestinal symptoms in patients with hereditary transthyretin amyloidosis, discussing diagnostic evaluations, assessment of disease progression and therapeutic strategies that could be implemented in routine ...
Suhr, Ole B., Obici, Laura, Suhr, Ole B.,   +2 more
core   +1 more source

Cardiac Amyloidosis: A Narrative Review of Diagnostic Advances and Emerging Therapies

Biomedicines
Background/Objectives: Cardiac amyloidosis (CA) is an underdiagnosed and potentially life-threatening infiltrative cardiomyopathy characterized by the extracellular deposition of misfolded amyloid fibrils in cardiac tissue. It is most commonly associated
Dana Emilia Movila, Alexandru Catalin Motofelea, Dragos Cozma, Oana Albai, Alexandra Christa Sima, Minodora Andor, Tudor Ciocarlie, Simona Ruxanda Dragan   +7 more
doaj   +1 more source

Hereditary transthyretin amyloidosis (ATTRv)

Hereditary transthyretin (TTR) amyloidosis (ATTRv amyloidosis) is a devastating disease characterized by broad range of clinical manifestations, including predominantly neurological, predominantly cardiac, and mixed phenotypes.
Kourek, Christos, Iliodromitis, Efstathios K., Paraskevaidis, Ioannis, Briasoulis, Alexandros, Magouliotis, Dimitrios E., Triposkiadis, Filippos, Starling, Randall C., Xanthopoulos, Andrew, Zakynthinos, George E.   +8 more
core  

Improving genetic testing pathways for transthyretin amyloidosis in France: challenges and strategies

Orphanet Journal of Rare Diseases
Transthyretin amyloidosis (ATTR) is a severe and rare disease characterized by the progressive deposition of misfolded transthyretin proteins, causing irreversible organ damage.
Bérénice Hebrard, Marie-Lise Babonneau, Philippe Charron, Emilie Consolino, Benjamin Dauriat, Delphine Dupin-Deguine, Dominique Fargeaud, Agnès Farrugia, Anna-Gaëlle Giguet-Valard, Damien Guijarro, Jocelyn Inamo, Julien Jeanneteau, Jean-Michaël Mazzella, Claire-Cécile Michon, Gilles Millat, Frédéric Mouquet, Silvia Oghina, Yann Pereon, Vianney Poinsignon, Julie Pompougnac, Julie Proukhnitzky, Elise Schaefer, Franck Sturtz, Mathilde Trosdorf, Anne Auguste, Giorgia Canali, Alexandre Combes, Benoît Funalot, Thibaud Damy   +28 more
doaj   +1 more source

Tafamidis Reduces Death and Hospitalization for Acute Heart Failure in Octogenarian Patients With Transthyretin Cardiac Amyloidosis: A Propensity Score–Weighted Cohort Study

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Background Transthyretin cardiac amyloidosis results from the deposition of misfolded amyloid fibrils in cardiac tissue, leading to progressive heart failure.
Louise Zmuda, Amira Zaroui, Mounira Kharoubi, Emmanuelle Boutin, Frédéric Roca, Silvia Oghina, Emmanuel Teiger, Marie Laurent, Florence Canoui‐Poitrine, Thibaud Damy, Amaury Broussier   +10 more
doaj   +1 more source

Specific therapeutic options for transthyretin amyloidosis

The epidemiology of systemic amyloidosis has recently undergone a spectacular transformation. While light-chain amyloidosis remains a rare disease with a stable incidence, chronic inflammatory disease associated amyloidosis (AA) is diagnosed less ...
Zoltán Pozsonyi
core   +1 more source

Protein mis-folding and human disease

, 2010
Serum Amyloid P Component (SAP), a putative molecular chaperone, is a homopentamericplasma protein of 25kDa subunits. It binds to the amyloid fibrils ofmisfolded proteins, which cause amyloidosis in humans. SAP not only stabilizesamyloid fibrils but also
Pal, Mohinder
core   +1 more source

Real-world tafamidis experience in hereditary transthyretin amyloidosis with peripheral neuropathy in Brazil

Background Tafamidis is a kinetic stabilizer that binds to the transthyretin (TTR) gene, inhibiting its dissociation. It is the only disease-modifying treatment for hereditary TTR amyloidosis with peripheral neuropathy (ATTRv-PN) available in the ...
Mariana Guedes, Luiz Felipe Pinto, Carlos P. Gomez, Marcus V. Pinto, Renata Gervais de Santa Rosa, Marcia Waddington-Cruz, Paula Accioli, Moises Dias, Gabriela Amorim, Roberto C. Pedrosa   +9 more
core   +1 more source