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    1318 results match your criteria Amyloidosis Primary Systemic

    1 OF 27

    Regional differences in prognostic value of cardiac valve plane displacement in systemic light-chain amyloidosis.
    J Cardiovasc Magn Reson 2017 Nov 9;19(1):87. Epub 2017 Nov 9.
    Department of Cardiology, University of Heidelberg, INF 410, 69120, Heidelberg, Germany.
    Background: To compare the prognostic value of cardiac valve plane displacement (CVPD) on various locations in cardiac light chain (AL) amyloidosis.

    Methods: Consecutive patients with biopsy-proven cardiac involvement in AL amyloidosis who had undergone cardiovascular magnetic resonance (CMR) between 2005 and 2014 in our institution, were retrospectively identified and data analyzed. The primary combined endpoint was all-cause mortality or heart transplantation. Read More

    Elevation of Plasmin-α2-plasmin Inhibitor Complex Predicts the Diagnosis of Systemic AL Amyloidosis in Patients with Monoclonal Protein.
    Intern Med 2017 Oct 11. Epub 2017 Oct 11.
    Department of Gastroenterology, Rheumatology, and Clinical Immunology, Sapporo Medical University School of Medicine, Japan.
    Objective The complication of systemic immunoglobulin light chain (AL) amyloidosis in patients with monoclonal immunoglobulin affects the prognosis, but amyloid deposition in tissues is sometimes difficult to detect due to bleeding tendencies and preferential distributions. However, fibrinolysis is known to be exacerbated in patients with systemic AL amyloidosis specifically. We therefore explored new biomarkers for predicting a diagnosis of systemic AL amyloidosis focusing on coagulation and fibrinolysis markers. Read More

    Infectious and non-infectious complications in primary immunodeficiency disorders: an autopsy study from North India.
    J Clin Pathol 2017 Sep 28. Epub 2017 Sep 28.
    Department of Paediatrics (Allergy and Immunology Unit), Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
    Background: Primary immunodeficiency disorders (PID) include a wide spectrum of inherited disorders characterised by functional abnormalities of one or more components of the immune system. Recent updates from the genomic data have contributed significantly to its better understanding with identification of new entities. Diagnosis is always challenging due to their variable clinical presentation. Read More

    Current insight in the localized insulin-derived amyloidosis (LIDA): clinico-pathological characteristics and differential diagnosis.
    Pathol Res Pract 2017 Oct 1;213(10):1237-1241. Epub 2017 Sep 1.
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:
    Background: In diabetic patients, subcutaneous insulin injection may cause several types of injection site-related lesions, such as lipoatrophy, insulin-induced cutaneous lipohypertrophy (IICL), allergic reaction, and iatrogenic localized insulin-derived amyloidosis (LIDA). Among these complications, both IICL and LIDA present as tumor-like and slow growing lesions; and they may be confused with one another. The clinical implication and management of IICL and LIDA are different. Read More

    The Lung in Dysregulated States of Humoral Immunity.
    Respiration 2017 15;94(5):389-404. Epub 2017 Sep 15.
    Université Paris 13, Sorbonne-Paris-Cité, EA2363, Paris, France.
    In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Read More

    Hepatic Amyloidosis: Something That Can camouflage and Deceive our Perception!
    Indian J Med Paediatr Oncol 2017 Apr-Jun;38(2):236-239
    Department of Gastroenterology, Kerala Institute of Medical Science and Research Centre, Trivandrum, Kerala, India.
    Amyloidosis is a multi-systemic diffusely infiltrating disease due to extracellular deposition of protein-mucopolysaccharide complexes. The type of protein deposited determines the subgroup of amyloid. Hepatic amyloidosis is a rare infiltrating disease affecting the hepatic parenchyma. Read More

    A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient.
    Cureus 2017 Jun 29;9(6):e1409. Epub 2017 Jun 29.
    Cardiology, Staten Island University Hospital, Northwell Health.
    Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Read More

    Nodular cutaneous amyloidoma of the extremity secondary to chronic granulomatous inflammation in setting of sarcoidosis.
    J Cutan Pathol 2017 Sep 19;44(9):801-804. Epub 2017 Jul 19.
    Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine, Miami, Florida.
    Soft tissue amyloidoma is a rare condition that presents primarily in the abdomen and/or mediastinum and more uncommonly on the extremities. Soft tissue amyloidomas on the extremities have been associated with chronic inflammation, particularly when accompanied by AA-type amyloid deposition as seen in local trauma, surgery, hypertension and diabetes. To our knowledge, this is the first reported case of nodular cutaneous amyloidoma in the setting of systemic and cutaneous sarcoidosis. Read More

    Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report.
    BMC Gastroenterol 2017 Jun 5;17(1):72. Epub 2017 Jun 5.
    The Third Xiangya Hospital of Central South University, 138 Tongzipo Road, Yuelu District Changsha, Hunan, 410013, China.
    Background: AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it's rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing immunofixation and endoscopy with multiple biopsies. We would like to emphasize the value of precise diagnostic process of AL amyloidosis. Read More

    Amyloidosis Cutis Dyschromica, a Rare Cause of Hyperpigmentation: A New Case and Literature Review.
    Pediatrics 2017 May;139(5)
    Department of General Pediatrics and Division of Pediatric Endocrinology and Diabetes, University Children's Hospital Heidelberg, Heidelberg, Germany; and.
    Amyloidosis cutis dyschromica is a rare form of primary cutaneous amyloidosis without systemic involvement and characterized by asymptomatic, progressive hyper- and hypopigmentation. We present the first case of a patient with amyloidosis cutis dyschromica diagnosed previously elsewhere as having Addison disease with generalized hyperpigmentation of the skin. This case suggests that in patients presenting with asymptomatic cutaneous dyschromia a skin biopsy for histopathological examination should be considered. Read More

    Common Fibril Structures Imply Systemically Conserved Protein Misfolding Pathways In Vivo.
    Angew Chem Int Ed Engl 2017 Jun 23;56(26):7510-7514. Epub 2017 May 23.
    Institute of Protein Biochemistry, Ulm University, Helmholtzstrasse 8/1, 89081, Ulm, Germany.
    Systemic amyloidosis is caused by the misfolding of a circulating amyloid precursor protein and the deposition of amyloid fibrils in multiple organs. Chemical and biophysical analysis of amyloid fibrils from human AL and murine AA amyloidosis reveal the same fibril morphologies in different tissues or organs of one patient or diseased animal. The observed structural similarities concerned the fibril morphology, the fibril protein primary and secondary structures, the presence of post-translational modifications and, in case of the AL fibrils, the partially folded characteristics of the polypeptide chain within the fibril. Read More

    Pattern of acute glomerulonephritis in adult population in Dubai: A single-center experience.
    Saudi J Kidney Dis Transpl 2017 May-Jun;28(3):571-578
    Department of Nephrology, Dubai Hospital, Dubai Health Authority, Dubai, United Arab Emirates.
    Epidemiological data of renal diseases have great geographic variability throughout the world. Due to the lack of a national renal data registry system, there is no information on the prevalence rate, clinical and pathological features of various glomerulonephritis (GN) in the United Arab Emirates (UAE). In a retrospective cross-sectional study, we analyzed 158 renal biopsies done in Dubai Hospital, UAE, between the years of 2005 and September 2014, with an aim to determine the prevalence rate and frequency of different pathological patterns of GN in adult patients who presented with proteinuria ± hematuria. Read More

    Complications of hidradenitis suppurativa.
    Semin Cutan Med Surg 2017 Jun;36(2):79-85
    Department of Dermatology, University of California, San Francisco, USA.
    Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by recurrent painful nodules and abscesses involving intertriginous areas. Repeated episodes of profound inflammation in HS can lead to a number of complications, causing significant morbidity and decreasing quality of life. Complications of HS may affect the skin alone or may have systemic impact. Read More

    Multifocal primary cutaneous nodular amyloidosis.
    Dermatol Online J 2017 May 15;23(5). Epub 2017 May 15.
    Department of Dermatology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
    Nodular cutaneous amyloidosis (NCA), the least common form of primary cutaneous amyloidosis, is characterized clinically by waxy, purpuric plaques and nodules and histologically by amyloid deposits in the dermis and subcutaneous tissue. We present a patient who developed multiple, non-contiguous NCA lesions over a three year period without evidence of systemic disease. We reviewed the literature and found few other cases of this unusual presentation. Read More

    AL Amyloidoma of the Skin/Subcutis: Cutaneous Amyloidosis, Plasma Cell Dyscrasia or a Manifestation of Primary Cutaneous Marginal Zone Lymphoma?
    Am J Surg Pathol 2017 Aug;41(8):1069-1076
    Departments of *Pathology †Medicine, Nova Scotia Health Authority (Central Zone) ‡Department of Pathology, Dalhousie University, Halifax, NS, Canada §Department of Dermatology, Fundación Jiménez Díaz, University Hospital La Princesa, Madrid, Spain ∥Dermatopathology Friedrichshafen, Friedrichshafen, Germany ¶Institute of Pathology and Microbiology, Wilhelminenspital, Vienna, Austria #Department of Dermatology, Research Unit Dermatopathology, Medical University of Graz, Graz, Austria.
    It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Read More

    Protein Misfolding, Amyloid Formation, and Human Disease: A Summary of Progress Over the Last Decade.
    Annu Rev Biochem 2017 Jun 12;86:27-68. Epub 2017 May 12.
    Department of Chemistry, Centre for Misfolding Diseases, University of Cambridge, Cambridge CB2 1EW, United Kingdom; email:
    Peptides and proteins have been found to possess an inherent tendency to convert from their native functional states into intractable amyloid aggregates. This phenomenon is associated with a range of increasingly common human disorders, including Alzheimer and Parkinson diseases, type II diabetes, and a number of systemic amyloidoses. In this review, we describe this field of science with particular reference to the advances that have been made over the last decade in our understanding of its fundamental nature and consequences. Read More

    Flow cytometry-based immunophenotypic analysis of primary systemic light chain amyloidosis.
    Oncol Lett 2017 Apr 22;13(4):2691-2697. Epub 2017 Feb 22.
    Department of Hematology, First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China.
    Using flow cytometry, the present study aimed to investigate the immunophenotypic characteristics of malignant plasma cells (PCs) in the bone marrow of patients with primary systemic light chain amyloidosis (AL) compared with the characteristics of patients with multiple myeloma (MM). Flow cytometric results of 51 patients with AL and 150 patients with MM were reviewed. The proportion of total bone marrow PCs in the patients with AL was significantly lower than that in the patients with MM, 1. Read More

    Amyloid in bone marrow smears in systemic light-chain amyloidosis.
    Amyloid 2017 Mar 23;24(1):52-59. Epub 2017 Apr 23.
    a Amyloidosis Center, Department of Medicine V , University Hospital Heidelberg , Heidelberg , Germany.
    We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Read More

    A case of amyloid myopathy diagnosed during the treatment of myopathy associated with anti-signal recognition particle antibodies.
    Rinsho Shinkeigaku 2017 04 30;57(4):168-173. Epub 2017 Mar 30.
    Department of Neurology, Shizuoka General Hospital.
    A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. Read More

    Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review.
    Am J Clin Dermatol 2017 Mar 24. Epub 2017 Mar 24.
    Department of Dermatology, University Hospital Jena, Erfurter Straße 35, Jena, 07743, Germany.
    Background: Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. Lichen amyloidosis, macular amyloidosis, and (primary localized cutaneous) nodular amyloidosis are different subtypes of PLCA.

    Objective: The aim of this study was to review the current reported treatment options for PLCA. Read More

    Treatment patterns and outcome following initial relapse or refractory disease in patients with systemic light chain amyloidosis.
    Am J Hematol 2017 Jun 26;92(6):549-554. Epub 2017 Apr 26.
    Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
    We analyzed the outcomes following initial relapse or refractory disease in systemic light chain amyloidosis (AL) and the impact of type of therapy employed.A total of 1327 patients with AL seen at Mayo Clinic within 90 days of diagnosis, between 2006 and 2015, were reviewed. The study included 366 patients experiencing a documented hematological or organ relapse or refractory disease requiring start of second line therapy. Read More

    Total Artificial Heart Implantation as a Bridge to Heart Transplantation in an Active Duty Service Member With Amyloid Cardiomyopathy.
    Mil Med 2017 Mar;182(3):e1858-e1860
    Cedars-Sinai Medical Center, Heart Institute, 127 South San Vincente Boulevard, Los Angeles, CA 90048.
    Introduction: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. Read More

    Primary Hepatic Amyloidosis Presenting as Acute-on-Chronic Liver Failure.
    ACG Case Rep J 2017 15;4:e22. Epub 2017 Feb 15.
    Department of Hepatology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi, India.
    Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. Read More

    Relapsing bullous amyloidosis of the oral mucosa and acquired cutis laxa in a patient with multiple myeloma: a rare triple association.
    Clin Exp Dermatol 2017 Mar 1. Epub 2017 Mar 1.
    Department of Dermatology, Hospital Universitario La Paz, Madrid, Spain.
    It is well known that primary systemic amyloidosis [light chain (AL) amyloidosis] is associated with hidden dyscrasia or multiple myeloma. Acquired cutis laxa (cutis laxa acquisita; CLA) has also been described in patients with plasma cell dyscrasias, including multiple myeloma. We report a case in which haemorrhagic oral bullae were the first sign of an undiagnosed primary systemic amyloidosis related to multiple myeloma IgG-λ and previously diagnosed CLA. Read More

    Modulation of amyloid assembly by glycosaminoglycans: from mechanism to biological significance.
    Biochem Cell Biol 2017 Jun 1;95(3):329-337. Epub 2017 Feb 1.
    Department of Chemistry, Pharmaqam, C.P. 8888, Succursale Centre-Ville, Université du Québec à Montréal (UQAM), Montreal, QC H3C 3P8, Canada.
    Glycosaminoglycans (GAGs) are long and unbranched polysaccharides that are abundant in the extracellular matrix and basement membrane of multicellular organisms. These linear polyanionic macromolecules are involved in many physiological functions from cell adhesion to cellular signaling. Interestingly, amyloid fibrils extracted from patients afflicted with protein misfolding diseases are virtually always associated with GAGs. Read More

    Amyloid cardiomyopathy.
    Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2017 Jun 1;161(2):117-127. Epub 2017 Feb 1.
    Department of Hemato-Oncology, University Hospital Olomouc, Czech Republic.
    Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. It is caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, or familial amyloidosis. Read More

    Amyloidosis of the Lung.
    Arch Pathol Lab Med 2017 Feb;141(2):247-254
    Context: -Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy). Read More

    The amyloidogenic light chain is a stressor that sensitizes plasma cells to proteasome inhibitor toxicity.
    Blood 2017 Apr 27;129(15):2132-2142. Epub 2017 Jan 27.
    Unit of Age Related Diseases, Division of Genetics and Cell Biology.
    Systemic light chain (AL) amyloidosis is caused by the clonal production of an unstable immunoglobulin light chain (LC), which affects organ function systemically. Although pathogenic LCs have been characterized biochemically, little is known about the biology of amyloidogenic plasma cells (PCs). Intrigued by the unique response rates of AL amyloidosis patients to the first-in-class proteasome inhibitor (PI) bortezomib, we purified and investigated patient-derived AL PCs, in comparison with primary multiple myeloma (MM) PCs, the prototypical PI-responsive cells. Read More

    Isolated primary amyloidosis of the inferior rectus muscle mimicking Graves' orbitopathy.
    Einstein (Sao Paulo) 2016 Oct-Dec;14(4):553-556
    Hospital Israelita Albert Einstein, São Paulo, SP, Brazil.
    The diagnosis of Graves' orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves' orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction. This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves' orbitopathy. Read More

    Lung adenocarcinoma expressing receptor for advanced glycation end-products with primary systemic AL amyloidosis: a case report and literature review.
    BMC Cancer 2017 01 5;17(1):22. Epub 2017 Jan 5.
    Department of Respiratory Medicine, Juntendo University School of Medicine & Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.
    Background: Receptor for advanced glycation end-products (RAGE), a receptor for amyloids, is constitutively expressed in lungs and generally observed to be downregulated in lung cancer tissues. However, increasing levels of RAGE or serum amyloids is associated with poor outcome in lung cancer patients. We report a rare case of primary systemic amyloid light-chain (AL) amyloidosis in biopsy-proven multiple organs with early-stage non-small cell lung cancer (NSCLC) that displayed strong staining for RAGE in the tumour tissue. Read More

    Coexistence of amyloidosis and Primary Sjögren's Syndrome: An overview Amyloidosis and Sjögren's syndrome.
    Curr Rheumatol Rev 2017 Jan 3. Epub 2017 Jan 3.
    Immunology and Rheumatology Department. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI. CP 14080.Mexico City, Mexico.
    Background: The association of primary Sjögren´s syndrome (SS) and amyloidosis has been recognized but scarcely assessed.

    Objective: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions.

    Methods: PubMed and CINAHL databases were searched from inception until April 2016 for the key words: amyloidosis, amyloid, Sjögren's syndrome and their combinations. Read More

    Anterior Aortic Plane Systolic Excursion: A Novel Indicator of Transplant-Free Survival in Systemic Light-Chain Amyloidosis.
    J Am Soc Echocardiogr 2016 Dec 27;29(12):1188-1196. Epub 2016 Oct 27.
    Department of Cardiology, University of Heidelberg, Heidelberg, Germany.
    Background: Anterior aortic plane systolic excursion (AAPSE) was evaluated in the present pilot study as a novel echocardiographic indicator of transplant-free survival in patients with systemic light-chain amyloidosis.

    Methods: Eighty-nine patients with light-chain amyloidosis were included in the post-hoc analysis. A subgroup of 54 patients with biopsy-proven cardiac amyloid infiltration were compared with 41 healthy individuals to evaluate the discriminative ability of echocardiographic findings. Read More

    Systemic Light Chain Amyloidosis Mimicking Rheumatic Disorders.
    Case Rep Med 2016 29;2016:7649510. Epub 2016 Nov 29.
    Lupus Center of Excellence, Allegheny Health Network, 4800 Friendship Avenue, Suite 2600, North Tower, Pittsburgh, PA 15224, USA.
    Secondary amyloidosis can complicate chronic inflammatory autoimmune diseases. However, the clinical findings of primary amyloidosis may mimic those of primary rheumatologic disorders. We present the case of a 53-year-old woman who presented with dystrophic nail changes, dry eyes, bilateral carpal tunnel syndrome, Raynaud's phenomenon, and high titer positive nucleolar pattern antinuclear antibody. Read More

    Ectropion and Conjunctival Mass in a Patient with Primary Bilateral Conjunctival Amyloidosis.
    Case Rep Ophthalmol Med 2016 16;2016:5610753. Epub 2016 Nov 16.
    Department of Ophthalmology, American University of Beirut Medical Center, Beirut, Lebanon.
    Background. Amyloidosis is a group of disorders characterized by deposition of an extracellular protein, known as amyloid, in an abnormal fibrillar form with highly characteristic histopathologic staining properties. The clinical presentation can vary from a focal, localized lesion where amyloidosis has minor clinical consequences to extensive systemic disease that can involve any organ system of the body. Read More

    Aβ Amyloid Pathology Affects the Hearts of Patients With Alzheimer's Disease: Mind the Heart.
    J Am Coll Cardiol 2016 Dec;68(22):2395-2407
    Cardiovascular Institute, Beth Israel Deaconess Medical Center, Boston, Massachusetts; Division of Cardiology Massachusetts General Hospital, Boston, Massachusetts. Electronic address:
    Background: Individually, heart failure (HF) and Alzheimer's disease (AD) are severe threats to population health, and their potential coexistence is an alarming prospect. In addition to sharing analogous epidemiological and genetic profiles, biochemical characteristics, and common triggers, the authors recently recognized common molecular and pathological features between the 2 conditions. Whereas cognitive impairment has been linked to HF through perfusion defects, angiopathy, and inflammation, whether patients with AD present with myocardial dysfunction, and if the 2 conditions bear a common pathogenesis as neglected siblings are unknown. Read More

    Bortezomib-dexamethasone versus high-dose melphalan for Japanese patients with systemic light-chain (AL) amyloidosis: a retrospective single-center study.
    Int J Hematol 2017 Mar 10;105(3):341-348. Epub 2016 Nov 10.
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.
    Bortezomib-dexamethasone (BD) and high-dose melphalan (HDM) are effective for systemic light-chain (AL) amyloidosis, but have not been compared in detail. We retrospectively investigated patients treated with BD or HDM at our center between September 2001 and June 2016. Among 234 patients, 20 were treated with BD and 30 received HDM. Read More

    Improved Brain Expression of Anti-Amyloid β scFv by Complexation of mRNA Including a Secretion Sequence with PEG-based Block Catiomer.
    Curr Alzheimer Res 2017 ;14(3):295-302
    Department of Bioengineering, Graduate School of Engineering, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8656, Japan.
    Background: The ever-increasing number of people living with Alzheimer's disease urges to develop more effective therapies. Despite considerable success, anti-Alzheimer immunotherapy still faces the challenge of intracerebral and intracellular delivery. This work introduces in situ production of anti-amyloid beta (Aβ) antibody after intracerebral injection of PEG-PAsp(DET)/mRNA polyplexes as a novel immunotherapy approach and a safer alternative compared to high systemic antibodies doses or administration of adenovirus encoding anti- Aβ antibodies. Read More

    Primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions and an excellent response to systemic acitretin.
    An Bras Dermatol 2016 Sep-Oct;91(5):661-663
    Third Affiliated Hospital, Sun Yat-sen University - Guangdong, China.
    Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Read More

    Repositioning nordihydroguaiaretic acid as a potent inhibitor of systemic amyloidosis and associated cellular toxicity.
    Arch Biochem Biophys 2016 Dec 24;612:78-90. Epub 2016 Oct 24.
    Interdisciplinary Biotechnology Unit, Aligarh Muslim University, Aligarh 202002, India. Electronic address:
    Although the cure of amyloid related neurodegenerative diseases, non-neuropathic amyloidogenic diseases and non-neuropathic systemic amyloidosis are appealing energetic research attempts, beneficial medication is still to be discovered. There is a need to explore intensely stable therapeutic compounds, potent enough to restrict, disrupt or wipe out such toxic aggregates. We had performed a comprehensive biophysical, computational and cell based assay, that shows Nordihydroguaiaretic acid (NA) not only significantly inhibits heat induced hen egg white lysozyme (HEWL) fibrillation but also disaggregates preformed HEWL fibrils and reduces the cytoxicity of amyloid fibrils as well as disaggregated fibrillar species. Read More

    Progressive refractory light chain amyloidosis and multiple myeloma patients are responsive to the addition of clarithromycin to IMiD based therapy.
    Am J Hematol 2017 02 21;92(2):131-135. Epub 2016 Nov 21.
    Department of Hematology, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
    Multiple myeloma (MM) and primary systemic light chain amyloidosis (AL) are both chronic plasma cell dyscrasias with different clinical expression but limited treatment options for relapsed refractory disease. We report the effect of the addition of clarithromycin on 31 MM and 17 AL with relapsed or refractory disease who had an insufficient response or disease progression while on an IMiD based therapy. In this high risk population, hematological response was reported in 48% of MM patients and 94% of AL patients. Read More

    [Hepatic amyloidosis as a rare differential diagnosis of progressive liver failure].
    Dtsch Med Wochenschr 2016 Sep 19;141(19):1387-9. Epub 2016 Sep 19.
    Primary systemic amyloidosis is a rare disorder resulting in extracellular deposition of insoluble fibrils in different organs. Liver involvement has been reported. Since hepatic amyloidosis often presents clinically asymptomatic without specific laboratory or imaging hallmarks, diagnosis is challenging. Read More

    Molecular basis of a novel renal amyloidosis due to N184K gelsolin variant.
    Sci Rep 2016 Sep 16;6:33463. Epub 2016 Sep 16.
    CNR Istituto di Biofisica, c/o Dipartimento di Bioscienze, Università degli Studi di Milano, 20133 Milano, Italy.
    Mutations in gelsolin are responsible for a systemic amyloidosis first described in 1969. Until recently, the disease was associated with two substitutions of the same residue, leading to the loss of the calcium binding site. Novel interest arose in 2014 when the N184K variant of the protein was identified as the etiological agent of a novel kidney-localized amyloidosis. Read More

    Localized primary amyloidosis of the breast: a case report and review of the literature.
    BMC Surg 2016 Sep 13;16(1):62. Epub 2016 Sep 13.
    Department of Breast Surgery, Shiga Medical Center for Adults, Shiga, Japan.
    Background: Primary amyloidosis of the breast is an unusual benign disease that mostly occurs in postmenopausal elderly women. Amyloidosis is the deposition of amorphous protein within tissues. Breast biopsy is necessary to make a definite diagnosis in order to avoid unnecessary surgical methods. Read More

    Prognostic Value of Late Gadolinium Enhancement CMR in Systemic Amyloidosis.
    JACC Cardiovasc Imaging 2016 Nov 24;9(11):1267-1277. Epub 2016 Aug 24.
    Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
    Objectives: The aim of this study was to access the prognostic implication of late gadolinium enhancement (LGE) in patients with systemic amyloidosis undergoing cardiac magnetic resonance (CMR).

    Background: Cardiac amyloidosis confers significantly worse prognosis in patients with systemic amyloidosis. CMR imaging has emerged as an attractive noninvasive modality to diagnose cardiac involvement in patients with systemic amyloidosis. Read More

    The Prevalence and Management of Systemic Amyloidosis in Western Countries.
    Kidney Dis (Basel) 2016 Apr 25;2(1):10-9. Epub 2016 Feb 25.
    Department of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
    Background: Amyloidosis has been a mystery for centuries, but research of the last decennia has clarified many of the secrets of this group of diseases. A protein-based classification of amyloidosis helps to understand problems that were part of the obsolete clinical classification in primary, secondary, and familial amyloidosis. All types of amyloid are secondary to some underlying precursor-producing process: each type is caused by a misfolded soluble precursor protein that becomes deposited as insoluble amyloid fibrils. Read More

    Nodular Cutaneous Amyloidosis at the Temple.
    Case Rep Dermatol 2016 May-Aug;8(2):193-6. Epub 2016 Jul 12.
    Department of Dermatology, University Medical Center Regensburg, Regensburg, Germany.
    A 52-year-old woman presented with a large partially yellow and erythematous tumor on her right temple. She reported that it had grown over the last 4 years. Regional lymph nodes were impalpable. Read More

    Transthyretin amyloid neuropathy has earlier neural involvement but better prognosis than primary amyloid counterpart: an answer to the paradox?
    Ann Neurol 2016 Sep 25;80(3):401-11. Epub 2016 Jul 25.
    Department of Neurology, Mayo Clinic, Rochester, MN.
    Objective: To systematically compare transthyretin with primary amyloid neuropathy to define their natural history and the underlying mechanisms for differences in phenotype and natural history.

    Methods: All patients with defined amyloid subtype and peripheral neuropathy who completed autonomic testing and electromyography at Mayo Clinic Rochester between 1993 and 2013 were included. Medical records were reviewed for time of onset of defined clinical features. Read More

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