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1.
J Prosthodont ; 29(8): 725-729, 2020 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-32794594

RESUMEN

Amyloidosis of the tongue can result in significant and irreversible alterations of tooth position and function due to prolonged application of imbalanced force on the teeth by the enlarged tongue. Due to the rarity of this oral form of systemic disease, little has been elucidated on management of the resulting impaired oral function. While surgery can address the size of the tongue, it carries significant morbidities, enlargement can recur, and does not address adverse tooth positioning. Prosthetic rehabilitation can more aptly restore oral function but it also needs to be tailored based on the patient's expectations and goals as well as biologic and mechanical parameters of treatment. This report discusses an effective and noninvasive application of a tooth-supported, removable prosthesis with an onlay occlusal design to restore occlusion, speech, and esthetics in a patient with tongue-based amyloidosis.


Asunto(s)
Amiloidosis , Estética Dental , Enfermedades de la Lengua , Amiloidosis/complicaciones , Oclusión Dental , Humanos , Lengua/cirugía
3.
Am J Cardiol ; 128: 140-146, 2020 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-32650908

RESUMEN

Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized infiltrative cardiomyopathy in which conduction system disease is common. The aim of our study was to define the incidence and prevalence of high-grade atrioventricular (AV) block requiring pacemaker implantation in our quaternary referral center. This was a single-center retrospective cohort study of 369 consecutive patients with ATTR-CA who underwent 12-lead electrocardiogram at the time of ATTR-CA diagnosis. During a mean follow-up of 28 months, serial ECGs and the electronic medical record were examined for the development of high-grade AV block and pacemaker implantation. Wild-type ATTR-CA (wtATTR-CA) was diagnosed in 261 patients and 108 had hereditary ATTR-CA (hATTR-CA). A total of 35 (9.5%) had high-grade AV block requiring pacemaker implantation at the time of diagnosis of ATTR-CA. The most common conduction abnormalities evident on the baseline ECG were a wide QRS complex, present in 51% with wtATTR-CA and 48% with hATTR-CA (p = 0.62), followed by first-degree AV block, which was present in 49% with wtATTR-CA and 43% with hATTR-CA (p = 0.31). During follow-up, high-grade AV block developed in 10% of those with hATTR-CA and 12% of patients with wtATTR-CA (p = 0.64). On multivariable models, high-grade AV block was not significantly associated with increased mortality. More advanced ATTR-CA stage and a history of obstructive coronary artery disease were associated with increased mortality on multivariable models. In conclusion, the incidence and prevalence of high-grade AV block is high in patients with ATTR-CA. Patients with ATTR-CA require close monitoring during follow-up for the development of conduction system disease.


Asunto(s)
Neuropatías Amiloides Familiares/fisiopatología , Bloqueo Atrioventricular/epidemiología , Cardiomiopatías/fisiopatología , Mortalidad , Anciano , Anciano de 80 o más Años , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/genética , Amiloidosis/complicaciones , Amiloidosis/fisiopatología , Bloqueo Atrioventricular/etiología , Bloqueo Atrioventricular/terapia , Bloqueo de Rama/epidemiología , Bloqueo de Rama/etiología , Estimulación Cardíaca Artificial , Cardiomiopatías/complicaciones , Cardiomiopatías/genética , Estudios de Cohortes , Electrocardiografía , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Mutación , Marcapaso Artificial , Prealbúmina/genética , Prevalencia , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Síndrome del Seno Enfermo/epidemiología , Síndrome del Seno Enfermo/etiología
4.
Rev Cardiovasc Med ; 21(2): 181-190, 2020 Jun 30.
Artículo en Inglés | MEDLINE | ID: mdl-32706207

RESUMEN

Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. No specific therapies have been shown to improve survival for the vast majority of HFpEF cases. Restrictive cardiomyopathies account for a significant portion of HFpEF patients and are characterized by diastolic dysfunction due to infiltration of the myocardium or ventricular hypertrophy. Many of these restrictive diseases occur in the context of myocardial infiltration by other substances such as amyloid, iron or glycogen or endomyocardial fibrosis. These infiltrative diseases usually have important clues in the clinical picture and on cardiac imaging that may allow differentiation from the usual HFpEF phenotype (that is commonly seen in the older, hypertensive patient). Noninvasive diagnosis has replaced endomyocardial biopsy for most instances in the workup of these conditions. Early recognition is important to institute specific therapies and to improve prognosis. In this review, we describe 4 major infiltrative cardiomyopathies (Cardiac Amyloidosis, Sarcoidosis, Hemochromatosis and Fabry disease), and their key imaging features.


Asunto(s)
Amiloidosis/diagnóstico por imagen , Cardiomiopatías/diagnóstico por imagen , Enfermedad de Fabry/diagnóstico por imagen , Insuficiencia Cardíaca/etiología , Hemocromatosis/diagnóstico por imagen , Volumen Sistólico , Función Ventricular Izquierda , Amiloidosis/complicaciones , Amiloidosis/fisiopatología , Amiloidosis/terapia , Cardiomiopatías/complicaciones , Cardiomiopatías/fisiopatología , Cardiomiopatías/terapia , Diagnóstico Diferencial , Diagnóstico Precoz , Enfermedad de Fabry/complicaciones , Enfermedad de Fabry/fisiopatología , Enfermedad de Fabry/terapia , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/terapia , Hemocromatosis/complicaciones , Hemocromatosis/fisiopatología , Hemocromatosis/terapia , Humanos , Valor Predictivo de las Pruebas , Pronóstico
6.
Sci Rep ; 10(1): 8183, 2020 05 18.
Artículo en Inglés | MEDLINE | ID: mdl-32424118

RESUMEN

In preceding efforts, we demonstrated that antibiotic (ABX) cocktail-mediated perturbations of the gut microbiome in two independent transgenic lines, termed APPSWE/PS1ΔE9 and APPPS1-21, leads to a reduction in Aß deposition in male mice. To determine whether these observed reductions of cerebral Aß amyloidosis are specific to any individual antibiotic or require the synergistic effects of several antibiotics, we treated male APPPS1-21 transgenic mice with either individual ABX or an ABX cocktail and assessed amyloid deposition. Specifically, mice were subject to oral gavage with high dose kanamycin, gentamicin, colistin, metronidazole, vancomycin, individually or in a combination (ABX cocktail) from postnatal days (PND) 14 to 21, followed by ad libitum, low-dose individual ABX or ABX cocktail in the drinking water until the time of sacrifice. A control group was subject to gavage with water from PND 14 to 21 and received drinking water till the time of sacrifice. At the time of sacrifice, all groups showed distinct cecal microbiota profiles with the highest differences between control and ABX cocktail-treated animals. Surprisingly, only the ABX cocktail significantly reduced brain Aß amyloidosis compared to vehicle-treated animals. In parallel studies, and to assess the potential exposure of ABX to the brain, we quantified the levels of each ABX in the brain by liquid chromatography-mass spectrometry (LC-MS) at PND 22 or at 7 weeks of age. With the exception of metronidazole (which was observed at less than 3% relative to the spiked control brains), we were unable to detect the other individual ABX in brain homogenates. Our findings suggest that synergistic alterations of gut microbial consortia, rather than individual antimicrobial agents, underlie the observed reductions in brain amyloidosis.


Asunto(s)
Enfermedad de Alzheimer/complicaciones , Amiloidosis/complicaciones , Amiloidosis/microbiología , Antibacterianos/farmacología , Microbioma Gastrointestinal/efectos de los fármacos , Animales , Ciego/efectos de los fármacos , Ciego/microbiología , Masculino , Ratones , Ratones Transgénicos
7.
Acta Haematol ; 143(4): 343-351, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32408301

RESUMEN

Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the importance of multidisciplinary care.


Asunto(s)
Amiloidosis/complicaciones , Cardiomiopatías/diagnóstico , Cardiomiopatías/etiología , Cardiomiopatías/terapia , Amiloidosis/diagnóstico , Amiloidosis/terapia , Terapia Combinada , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Pruebas de Función Cardíaca , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , Fenotipo
8.
Am J Kidney Dis ; 76(2): 295-297, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32362418

Asunto(s)
Amiloidosis/patología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/patología , Nefropatías Diabéticas/patología , Glomerulonefritis/patología , Riñón/patología , Nefrosis Lipoidea/patología , Lesión Renal Aguda/etiología , Lesión Renal Aguda/patología , Anciano , Anciano de 80 o más Años , Amiloidosis/complicaciones , Amiloidosis/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , China , Estudios Transversales , Nefropatías Diabéticas/complicaciones , Nefropatías Diabéticas/diagnóstico , Femenino , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnóstico , Glomerulonefritis por IGA/complicaciones , Glomerulonefritis por IGA/diagnóstico , Glomerulonefritis por IGA/patología , Glomerulonefritis Membranosa/complicaciones , Glomerulonefritis Membranosa/diagnóstico , Glomerulonefritis Membranosa/patología , Glomeruloesclerosis Focal y Segmentaria/complicaciones , Glomeruloesclerosis Focal y Segmentaria/diagnóstico , Glomeruloesclerosis Focal y Segmentaria/patología , Hematuria/etiología , Hematuria/patología , Humanos , Masculino , Nefrosis Lipoidea/complicaciones , Nefrosis Lipoidea/diagnóstico , Síndrome Nefrótico/etiología , Síndrome Nefrótico/patología , Proteinuria/etiología , Proteinuria/patología , Insuficiencia Renal Crónica/etiología , Insuficiencia Renal Crónica/patología
9.
Cardiovasc Pathol ; 48: 107218, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32388447

RESUMEN

Cardiac amyloid A (AA) amyloidosis is rare. We present the case of a 72-year-old woman with obstructive hypertrophic cardiomyopathy (HCM) and biopsy-proven renal AA amyloidosis whose dyspnea and exercise intolerance had worsened over the previous year. Her AA amyloidosis was suspected to be secondary to chronic diverticulitis for which she had undergone hemicolectomy and sigmoidectomy 3 years prior. Echocardiographic findings were consistent with worsening left ventricular outflow tract obstruction at rest. Cardiac magnetic resonance imaging revealed patchy areas of midwall late gadolinium enhancement. Right ventricular endomyocardial biopsy did not reveal amyloid deposition, and cardiac technetium-99m pyrophosphate scintigraphy did not suggest transthyretin amyloidosis. The patient underwent septal myectomy with resection of an accessory papillary muscle. Pathological examination of the myectomy specimen was consistent with HCM. In addition, there was a thick layer of diffuse endocardial and vascular amyloid deposition that was identified as AA type by laser-microdissection with liquid chromatography-coupled tandem-mass spectrometry. This case report highlights the presence of 2 distinct disease processes occurring simultaneously and the importance of tissue diagnosis of AA amyloidosis, a condition that is not commonly associated with HCM.


Asunto(s)
Amiloidosis/complicaciones , Cardiomiopatía Hipertrófica/complicaciones , Insuficiencia Cardíaca/etiología , Enfermedades Renales/complicaciones , Miocardio/patología , Obstrucción del Flujo Ventricular Externo/etiología , Anciano , Amiloidosis/metabolismo , Amiloidosis/patología , Amiloidosis/fisiopatología , Procedimientos Quirúrgicos Cardíacos , Cardiomiopatía Hipertrófica/metabolismo , Cardiomiopatía Hipertrófica/patología , Cardiomiopatía Hipertrófica/fisiopatología , Femenino , Insuficiencia Cardíaca/metabolismo , Insuficiencia Cardíaca/patología , Insuficiencia Cardíaca/fisiopatología , Humanos , Enfermedades Renales/metabolismo , Enfermedades Renales/patología , Miocardio/metabolismo , Proteína Amiloide A Sérica/metabolismo , Resultado del Tratamiento , Función Ventricular Izquierda , Obstrucción del Flujo Ventricular Externo/metabolismo , Obstrucción del Flujo Ventricular Externo/patología , Obstrucción del Flujo Ventricular Externo/fisiopatología
13.
Medicine (Baltimore) ; 99(13): e19462, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32221068

RESUMEN

INTRODUCTION: Primary mucosa-associated lymphoid tissue (MALT) lymphomas originating in thymus is rare. And, there have been few reports of patients with MALT coexisting with amyloidosis. As far as we know, this was the first case report on MALT lymphoma associated with renal amyloidosis. PATIENT CONCERNS: A 57-year-old man presented with nephrotic syndrome. Further workup revealed IgM-Lambda type monoclonal gammopathy. Bone marrow biopsy showed 8% clonal plasma cells. Renal biopsy confirmed the diagnosis of Lambda light chain AL amyloidosis. positron emission tomography/computed tomography showed thymic lesions which upon biopsy were diagnosed as MALT lymphoma of the thymus. DIAGNOSIS: Primary thymic MALT lymphoma complicated with renal amyloidosis. INTERVENTIONS: The patient underwent surgical resection of the thymus mass and 2 courses of chemotherapy. OUTCOMES: Follow-up data showed that the patient survived 18 months after surgical excision and chemotherapy. CONCLUSION: The case highlights the importance of screening for malignancy in patients with renal amyloidosis.


Asunto(s)
Amiloidosis/complicaciones , Enfermedades Renales/complicaciones , Linfoma de Células B de la Zona Marginal/complicaciones , Neoplasias del Timo/complicaciones , Antineoplásicos , Humanos , Cadenas lambda de Inmunoglobulina , Linfoma de Células B de la Zona Marginal/tratamiento farmacológico , Linfoma de Células B de la Zona Marginal/cirugía , Masculino , Persona de Mediana Edad , Síndrome Nefrótico/etiología , Neoplasias del Timo/tratamiento farmacológico , Neoplasias del Timo/cirugía
14.
Clin Nucl Med ; 45(5): 385-386, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-32149807

RESUMEN

We present a paraspinal amyloidoma found incidentally in a 77-year-old man during lymphoma workup by F-FDG PET/CT. A solitary FDG-avid paraspinal lesion was seen at T11-T12. MRI showed T2 hypointensity and enhancement. Lymphoma was considered the primary differential due to FDG uptake, but biopsy revealed nodules of extracellular acellular homogeneous material with apple-green birefringence on Congo red stain consistent with amyloidoma. Spinal amyloidoma is rare with few cases reported so far in literature.


Asunto(s)
Amiloidosis/diagnóstico por imagen , Amiloidosis/metabolismo , Fluorodesoxiglucosa F18/metabolismo , Tomografía Computarizada por Tomografía de Emisión de Positrones , Columna Vertebral/diagnóstico por imagen , Columna Vertebral/metabolismo , Anciano , Amiloidosis/complicaciones , Transporte Biológico , Humanos , Hallazgos Incidentales , Linfoma/complicaciones , Linfoma/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino
15.
Can J Cardiol ; 36(3): 373-383, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-32145865

RESUMEN

Amyloidosis is a term used to describe a group of rare heterogeneous diseases that ultimately result in the deposition and accumulation of misfolded proteins. These misfolded proteins, known as amyloids, are associated with a variety of precursor proteins that have amyloidogenic potential. Ultimately, the specific type of amyloidosis is dependent on multiple factors including genetic variability of precursor proteins and the tissue or organ in which the amyloid accumulates. Several types of amyloid have a predilection for the heart and thus contribute to cardiac amyloidosis, a major cause of restrictive cardiomyopathy. Individuals with cardiac amyloidosis present clinically with heart failure with preserved ejection fraction. Although improved diagnostics and increased awareness of cardiac amyloidosis have led to a relative increase in diagnosis, cardiac amyloidosis remains an underrecognized and underdiagnosed cause of heart failure with preserved ejection fraction. It is essential to properly identify cases of cardiac amyloidosis and determine the pathology responsible for the formation of amyloid to appropriately provide management. This review aims to encourage physician awareness of cardiac amyloidosis by focusing on clinical presentation and the distinctions between types. Furthermore, epidemiology is central to understanding the affected demographics and sometimes hereditary nature of the disease. Improved understanding of cardiac amyloidosis will ideally lead to earlier diagnosis and interventions to improve patient outcomes.


Asunto(s)
Amiloidosis/epidemiología , Cardiomiopatías/epidemiología , Amiloidosis/clasificación , Amiloidosis/complicaciones , Cardiomiopatías/clasificación , Cardiomiopatías/complicaciones , Insuficiencia Cardíaca/complicaciones , Humanos
16.
Can J Cardiol ; 36(3): 416-423, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-32145868

RESUMEN

Cardiac amyloidosis occurs secondarily to the deposition of insoluble protein fibrils in cardiac tissue leading to progressive myocardial dysfunction, clinical heart failure, and arrhythmia. In recent years, increasing awareness and improved screening have resulted in an increased prevalence of cardiac amyloidosis, with contemporary estimates reporting a prevalence of 18-55 cases per 100,000 person-years, accounting for > 13% of heart failure hospitalizations. The arrhythmic manifestations of cardiac amyloidosis can range from conduction-system disease and bradyarrhythmias to atrial fibrillation and sudden cardiac death. Bradyarrhythmias and conduction system disease may occur secondarily to amyloid infiltration, but the timing of pacemaker implantation remains unclear. When available, biventricular pacing should be considered in symptomatic patients, particularly in those expected to receive a high burden of ventricular pacing (> 40%). The management of atrial fibrillation can be challenging, because contemporary agents for rate and rhythm control may be poorly tolerated in patients with cardiac amyloidosis. Patients with cardiac amyloidosis also have a high rate of intracardiac thrombus and should be anticoagulated in the presence of atrial fibrillation (regardless of CHADS2 score). We generally consider transesophageal echocardiography before cardioversion regardless of anticoagulation status or duration of arrhythmia. Ventricular arrhythmias may also occur in patients with cardiac amyloidosis, and decisions surrounding implantable cardioverter-defibrillator implantation should balance the risks of ventricular arrhythmia and sudden cardiac death with the competing risks of worsening heart failure and noncardiac death. In this review, we cover the primary arrhythmic manifestations of cardiac amyloidosis and discuss their management considerations.


Asunto(s)
Amiloidosis/complicaciones , Arritmias Cardíacas/etiología , Arritmias Cardíacas/terapia , Cardiomiopatías/complicaciones , Arritmias Cardíacas/epidemiología , Humanos , Marcapaso Artificial , Medición de Riesgo
17.
Rev. otorrinolaringol. cir. cabeza cuello ; 80(1): 48-53, mar. 2020. tab, graf
Artículo en Español | LILACS | ID: biblio-1099201

RESUMEN

El diagnóstico diferencial de la macroglosia es amplio y puede estar en el contexto de una patología localizada o una enfermedad sistémica. Dentro de las enfermedades sistémicas que se manifiestan con macroglosia se describe la amiloidosis, caracterizada por un depósito irreversible de una proteína amorfa y fibrilar El compromiso de esta patología en el territorio de cabeza y cuello es infrecuente y su depósito en la lengua corresponde a menos del 9% de los casos. Se presenta el caso de un paciente que consultó por dolor en hemilengua derecha, disfagia y baja de peso. Se estudió inicialmente con exámenes de laboratorio y nasofibroscopía, sin hallazgos concluyentes. Dado persistencia de síntomas se realizó resonancia nuclear magnética (RM) que mostraba signos sugerentes de enfermedad de depósito, confirmándose mediante estudios histopatológicos una amiloidosis sistémica secundaria. El paciente fue derivado a hematología para completar estudio e iniciar el tratamiento. La amiloidosis sistémica es un diagnóstico infrecuente, que debe ser considerado por su mal pronóstico vital. El diagnóstico en etapas iniciales puede mejorar sustancialmente la sobrevida y calidad de vida de aquellos que padecen la enfermedad. Por lo anterior, es necesario completar un estudio acabado de la patología, apoyándose en métodos no invasivos como la RM.


The differential diagnosis of macroglossia is broad, and it may be present in the context of a localized pathology or a systemic disease. One of the systemic diseases that present macroglossia is amyloidosis, which is characterized by an irreversible deposit of an amorphous and fibrillar protein. The manifestation of this pathology in head and neck territory is infrequent, and its deposit in the tongue represents less than 9% of all types of amyloidosis. We present the case of a patient, who consulted with pain in the tongue, dysphagia, and weight loss. He was initially studied with laboratory tests and a nasofibroscopy with no conclusive findings. Given the persistence of symptoms, magnetic resonance imaging (MRI) was performed, showing signs of an infiltrative disease, which was confirmed as secondary systemic amyloidosis through histopathological studies. Given the above, the patient was referred to hematology to start treatment. Systemic amyloidosis is an uncommon diagnosis that should be suspected since it implies a poor vital prognosis. Moreover, an early diagnosis can substantially improve the survival rate and quality of life of those who suffer this disease. Therefore, a comprehensive study of this condition is needed, complementing with non-invasive methods such as MRI.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Amiloidosis/complicaciones , Macroglosia/etiología , Mieloma Múltiple/complicaciones , Imagen por Resonancia Magnética , Macroglosia/diagnóstico por imagen
18.
Can J Cardiol ; 36(2): 159-169, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-32036861

RESUMEN

In this update, we focus on selected topics of high clinical relevance for health care providers who treat patients with heart failure (HF), on the basis of clinical trials published after 2017. Our objective was to review the evidence, and provide recommendations and practical tips regarding the management of candidates for the following HF therapies: (1) transcatheter mitral valve repair in HF with reduced ejection fraction; (2) a novel treatment for transthyretin amyloidosis or transthyretin cardiac amyloidosis; (3) angiotensin receptor-neprilysin inhibition in patients with HF and preserved ejection fraction (HFpEF); and (4) sodium glucose cotransport inhibitors for the prevention and treatment of HF in patients with and without type 2 diabetes. We emphasize the roles of optimal guideline-directed medical therapy and of multidisciplinary teams when considering transcatheter mitral valve repair, to ensure excellent evaluation and care of those patients. In the presence of suggestive clinical indices, health care providers should consider the possibility of cardiac amyloidosis and proceed with proper investigation. Tafamidis is the first agent shown in a prospective study to alter outcomes in patients with transthyretin cardiac amyloidosis. Patient subgroups with HFpEF might benefit from use of sacubitril/valsartan, however, further data are needed to clarify the effect of this therapy in patients with HFpEF. Sodium glucose cotransport inhibitors reduce the risk of incident HF, HF-related hospitalizations, and cardiovascular death in patients with type 2 diabetes and cardiovascular disease. A large clinical trial recently showed that dapagliflozin provides significant outcome benefits in well treated patients with HF with reduced ejection fraction (left ventricular ejection fraction ≤ 40%), with or without type 2 diabetes.


Asunto(s)
Amiloidosis/complicaciones , Amiloidosis/tratamiento farmacológico , Antagonistas de Receptores de Angiotensina/uso terapéutico , Benzoxazoles/uso terapéutico , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia de la Válvula Mitral/complicaciones , Insuficiencia de la Válvula Mitral/cirugía , Neprilisina/antagonistas & inhibidores , Inhibidores del Cotransportador de Sodio-Glucosa 2/uso terapéutico , Cardiopatías/complicaciones , Cardiopatías/tratamiento farmacológico , Insuficiencia Cardíaca/fisiopatología , Humanos , Insuficiencia de la Válvula Mitral/fisiopatología , Ensayos Clínicos Controlados Aleatorios como Asunto , Índice de Severidad de la Enfermedad , Volumen Sistólico
19.
Can J Cardiol ; 36(3): 408-415, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-32037105

RESUMEN

Arrhythmias are a major cause of morbidity and mortality in the course of cardiac amyloidosis (CA). Less commonly, they may be the initial manifestation that lead to the diagnosis. With improved therapeutic interventions for amyloidosis, it is no longer considered to be a terminal untreatable condition, and there is increasing recognition of the role of implantable electronic devices in CA. The frequency and nature of arrhythmias are largely determined by the type of amyloidosis. Bradyarrhythmias are more common in the transthyretin form of amyloidosis, and risk for ventricular arrhythmias is higher in the light-chain form. Pacemaker implantation is often required and effective for alleviation of symptoms. The role of implantable cardioverter-defibrillators (ICDs) remains controversial, especially for primary prevention of sudden death. Traditional risk stratification tools for sudden death do not appear to be applicable to CA, because decline of left ventricular (LV) systolic dysfunction to the point of the usual indication for an ICD implant in other cardiomyopathies, ie, LV ejection fraction ≤ 35%, usually marks end-stage disease in CA when pump failure becomes the predominant cause of death. The challenge remains the identification of markers for sudden death in early stages of the disease. Included in this review is a general overview of available data on the nature of bradycardia and ventricular arrhythmias, including the role of implantable electronic devices for the treatment of these conditions. Published series of ICD use in CA are summarized and the role of newer pacing techniques, including biventricular pacing, is discussed.


Asunto(s)
Amiloidosis/complicaciones , Arritmias Cardíacas/complicaciones , Arritmias Cardíacas/terapia , Cardiomiopatías/complicaciones , Desfibriladores Implantables , Algoritmos , Humanos
20.
J Clin Ultrasound ; 48(3): 168-173, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32003472

RESUMEN

We describe the case of a 41-year-old woman with primary Sjögren's syndrome (SS) who presented multiple recurrences of breast amyloidosis. Each recurrence of breast amyloidosis showed different sonographic features, potentially mimicking malignancy. We briefly discuss the possible cause of this variability in imaging features based on the radiologic-histologic correlation.


Asunto(s)
Amiloidosis/complicaciones , Amiloidosis/diagnóstico por imagen , Enfermedades de la Mama/complicaciones , Enfermedades de la Mama/diagnóstico por imagen , Síndrome de Sjögren/complicaciones , Adulto , Amiloidosis/patología , Amiloidosis/cirugía , Enfermedades de la Mama/patología , Enfermedades de la Mama/cirugía , Femenino , Estudios de Seguimiento , Humanos , Persona de Mediana Edad , Recurrencia , Ultrasonografía/métodos
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