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8 Ways Amyloidosis Affects the Body

Healthline

Amyloidosis is a condition that affects various body tissues and organs. But it’s much more complicated and symptomatic than it sounds. The symptoms and severity of amyloidosis can vary between individuals, although some symptoms are more common than others. Read on to learn more about the ways amyloidosis can affect the body, and what you can do to manage these symptoms. Kidney issues Amyloid protein...

Abdominal Wall Fat Pad Biopsy

A.D.A.M. Multimedia Encyclopedia, Penn State Hershey Medical Center

An abdominal wall fat pad biopsy is the removal of a small part of the abdominal wall fat pad for laboratory study of the tissue.   Alternative Names Amyloidosis – abdominal wall fat pad biopsy; Abdominal wall biopsy; Biopsy – abdominal wall fat pad   How the Test is Performed? Needle aspiration is the most common method of taking an abdom...

AL Amyloidosis and Fatigue

Myeloma UK

Fatigue is a feeling of near constant exhaustion that differs from the everyday tiredness that comes with the demands of a busy daily life. Unlike ordinary tiredness, it is not caused by activity or exertion and is not relieved by rest or sleep.   Over time, fatigue can be profoundly debilitating as it can have an impact on your physical, emotional and psychological wellbeing. It is a common complication of AL amyloidosi...

AL Amyloidosis and the Kidney

Myeloma UK

In AL amyloidosis, an abnormal protein called amyloid is produced and accumulates in tissues and organs. The build-up of amyloid  protein is called an amyloid deposit. Deposits can occur in various organs or tissues, including the kidneys, and affect their function. The kidneys and the heart are the organs most commonly affected by amyloid deposits.   What do the Kidneys do? Most healthy peo...

AL Amyloidosis FAQs

Myeloma UK

What is AL amyloidosis? The term ‘amyloidosis’ is a general term used for a group of conditions where an abnormal protein, called amyloid, accumulates in the tissues. The build-up of amyloid protein is called an ‘amyloid deposit’. Deposits can occur in various organs or tissues and cause problems. Facts about AL amyloidosis • Amyloid can build up in the kidneys, heart, liver,...

Amyloid Neuropathy

Johns Hopkins Medicine

Disorders of peripheral nerves are the most common neurological complications of systemic amyloidosis; an illness where a protein called amyloid is deposited in tissues and organs. Amyloidosis can affect peripheral sensory, motor or autonomic nerves and deposition of amyloid lead to degeneration and dysfunction in these nerves. Symptoms The typical symptoms of amyloid neuropathy are due to sensory and autonomic...

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A Study of the Neuropathy Associated With Transthyretin Amyloidosis (ATTR) in the UK

Journal of Neurology, Neurosurgery, and Psychiatry

Background: Hereditary transthyretin amyloidosis (ATTR) is usually characterised by a progressive peripheral and autonomic neuropathy often with associated cardiac failure and is due to dominantly inherited transthyretin mutations causing accelerated amyloid deposition. The UK population is unique in that the majority of patients have the T60A missense mutation in ATTR where tyrosine is replaced by adenine at position...

AA Amyloidosis Treated With Tocilizumab: Case Series and Updated Literature Review

Amyloid: The International Journal of Experimental and Clinical Investigation

Background: In published case reports, tocilizumab (TCZ) has shown good efficacy for AA amyloidosis in almost all patients. We investigated the efficacy and safety of TCZ in AA amyloidosis in a multicentre study of unselected cases. Methods: We e-mailed rheumatology and internal medicine departments in France, Switzerland and North Africa by using the Club Rhumatismes Inflammation (CRI)...

AA Amyloidosis: Pathogenesis and Targeted Therapy

Annual Review of Pathology

The understanding of why and how proteins misfold and aggregate into amyloid fibrils has increased considerably during recent years. Central to amyloid formation is an increase in the frequency of the β-sheet structure, leading to hydrogen bonding between misfolded monomers and creating a fibril that is comparably resistant to degradation. Generation of amyloid fibrils is nucleation dependent, and once formed, fibrils recruit and catalyze the...

AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

Journal of the American College of Cardiology

The amyloidoses are a group of protein-folding disorders in which >=1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid.  Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chai...

AL Amyloidosis Presenting With Limb Girdle Myopathy

Practical Neurology

An elderly Caucasian man presented with a 10-month history of proximal myopathy and dysphagia. His serum creatine kinase (CK) was elevated at 877 U/L (normal 40-320) and electromyography confirmed a myopathic process. Blood and urine tests suggested myeloma; bone marrow examination showed 30% plasma cells and stained positive for amyloid. The muscle biopsy was initially reported as normal but in the light of the bone marrow report, the biopsy...

AL Amyloidosis: From Molecular Mechanisms to Targeted Therapies

Hematology / The Education Program of the American Society of Hematology

Systemic amyloidosis is caused by misfolding and extracellular deposition of circulating proteins as amyloid fibrils, resulting in the dysfunction of vital organs. The most common systemic amyloidosis, light-chain (AL) amyloidosis, is caused by misfolded light chains produced by a small, dangerous B-cell clone. The process of amyloid formation, organ targeting, and damage is multifaceted and, after disease initiation, the complexity of the dow...

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Guidance for Industry AL Amyloidosis — Developing Drugs for Treatment

Amyloidosis Research Consortium

The purpose of this guidance is to assist sponsors in the clinical development of medical products (ie, drugs and therapeutic biological products for humans) for the treatment of patients with immunoglobulin light chain (AL) amyloidosis. Transthyretin (ATTR) amyloidosis will be addressed in a separate guidance document.   This guidance is a result of collaboration between the US Food and Drug Administration (F...

Management of Systemic AL Amyloidosis: Recommendations of the Myeloma Foundation of Australia Medical and Scientific Advisory Group

Internal Medicine Journal

Systemic AL amyloidosis is a plasma cell dyscrasia with a characteristic clinical phenotype caused by multi-organ deposition of an amyloidogenic monoclonal protein. This condition poses a unique management challenge due to the complexity of the clinical presentation and the narrow therapeutic window of available therapies. Improved appreciation of the need for risk stratification, standardised use of sensitive laboratory testing for monitoring...

Standardization of 99m technetium Pyrophosphate Imaging Methodology to Diagnose TTR Cardiac Amyloidosis

Journal of Nuclear Cardiology

Background: Technetium pyrophosphate (99mTc-PYP) imaging to diagnose transthyretin cardiac amyloidosis (ATTR-CA) has been increasingly utilized. The objective of this study is to provide a standardized 99mTc-PYP imaging protocol to diagnose ATTR-CA. Methods: 104 scans from 45 subjects with biopsy-proven ATTR-CA or light-chain cardiac amyloidosis (AL) were assessed. Multiple scans were o...

Daratumumab-Based Therapies in Patients With AL Amyloidosis

American Society of Clinical Oncology

Background: Treatment options for patients (pts) with relapsed/refractory (RR) AL amyloidosis are limited. Daratumumab (dara) has been approved as monotherapy (DMT) or combination therapy (DCT) for multiple myeloma (MM). Data for dara-based therapy (DBT) in AL are sparse. Methods: We studied pts with RR AL without coexisting MM seen at Mayo Clinic from 11/2015 to 02/2018 & treated w...

Implanted Cardiac Defibrillator and Pacemaker Lead Function in Patient With Cardiac Amyloidosis

Journal of the American College of Cardiology

Introduction:  Cardiac amyloidosis (CA) results from myocardial infiltration of amyloid proteins that leads to restrictive cardiomyopathy, heart failure, and conduction abnormalities. Low QRS voltage on surface electrocardiography is characteristic of CA, raising concern for poor R-wave sensing and elevated pacing and defibrillation thresholds in CA patients with Implantable Cardioverter Defibrillator (ICD) or pacemak...

Subcutaneous Daratumumab (DARA SC) Plus Cyclophosphamide, Bortezomib, and Dexamethasone (Cybord) in Patients (Pts) With Newly Diagnosed Amyloid Light Chain (AL) Amyloidosis: Safety Run-in Results of Andromeda

American Society of Clinical Oncology

Background: Systemic AL amyloidosis is characterized by disposition of insoluble amyloid fibrils into tissues and organs via clonal expansion of CD38+ plasma cells. The safety run-in of DARA SC + CyBorD in ANDROMEDA (NCT03201965) is presented. Methods: Eligible pts had >=1 involved organs, ECOG score =<2, absolute neutrophil count >=1.0 × 109/L; hemoglobin >=8.0 g/dL; plate...

A Guide to Transthyretin Amyloidosis

Amyloidosis Foundation

Amyloidosis is a systemic disorder characterized by extra cellular deposition of a protein-derived material, known as amyloid, in multiple organs. Amyloidosis occurs when native or mutant polypeptides misfold and aggregate as fibrils. The amyloid deposits cause local damage to the cells around which they are deposited leading to a variety of clinical symptoms. There are at least 23 different proteins associated with the amyloidoses. <...

A Physician’s Guide to AL (Light Chain) Amyloidosis

Amyloidosis Foundation

Amyloidosis is a disorder in which misfolded native proteins deposit extracellularly and lead to organ damage. Although the various types of amyloidoses share in common the deposition of misfolded proteins that aggregate as fibrils in tissue, they differ in actual protein composition, organ involvement, prognostic implication and most importantly, treatment. It is therefore critically important to determine the type of amyloidosis a patient...

Amyloidosis Diagnosis and Treatment App for Healthcare Professionals

Amyloidosis Research Consortium

Our Amyloidosis Clinical Resources App has been created to provide clinically relevant information that offers healthcare professionals a brief yet practical disease overview and interactive tools which can lead to earlier diagnosis and therapeutic intervention.   Highlights Include: • Disease types and descriptions. • Clinical presentations. • Signs and symp...

Amyloidosis: What Every Physician Needs to Know

Decision Support in Medicine

Amyloidosis is a term for diseases caused by extracellular deposition of protein fibrils. The accepted nomenclature is “AX”, “A” for amyloidosis, and “X” indicating the precursor protein. If the precursor is an immunoglobulin light chain, the disease is termed AL, the most common of the systemic amyloidoses. If it is familial (AF), the precursor protein can be an inherited mutant serum protein such as transthyretin, abbreviated A...

ARC Cardiac Amyloidosis Alert Card

Amyloidosis Research Consortium

Low Voltages For cardiac amyloidosis issues, contraindications exist for calcium channel blockers, Digoxin, ACE inhibitors and beta blockers. Cautious application and careful monitoring are imperative with the presentation of each patient and their specific cardiac condition and only under certain circumstances.   Increased Ventricular Mass Amyloid fibrils mak...

Changing the Perspective on Cardiac Amyloidosis: From Missed and Undiagnosed, to Common and Treatable

Healio Education Lab - Cardiology

Cardiac Amyloidosis is a rare systemic disorder characterized by proteins that possess abnormal conformational features leading them to aggregate and infiltrate tissues in the form of amyloid fibrils resulting in damage to the heart and Heart Failure. Non-specific presenting symptoms and failure to recognize early signs of amyloid heart disease often lead to delay in diagnosis of Cardiac Amyloidosis. Timely diagnosis is critical to managing...

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