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White matter disorders with autosomal dominant heredity: a review with personal clinical case studies and their MRI findingsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion [ndash] The diagnosis within the group of leukoencephalopathies thus characterized by MRI relies mainly upon clinical and genetic analysis. The differential diagnosis against treatable leukoencephalopathies is increasingly relevant. (Source: Acta Neurologica Scandinavica)
Source: Acta Neurologica Scandinavica - November 19, 2009 Category: Neurology Authors: C. Sundal, S. Ekholm, O. Andersen Source Type: journals

Amyloidosis and Neurodegenerative Diseases: Current Treatments and New Pharmacological OptionsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Pharmacology 2010;85:1-17 (DOI:10.1159/000259044) (Source: Karger Publishers)
Source: Karger Publishers - November 17, 2009 Category: Cancer & Oncology Source Type: journals

Microalbuminuria as an early marker of renal involvement in Behcet's disease: it is associated with neurological involvement and duration of the diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Background Despite its nature as a systemic vasculitis, renal involvement is known to occur infrequently in Behçet's Disease (BD).Objectives Our aim was to investigate proteinuria, microhematuria and microalbuminuria in 24-h urine and evaluate subclinical or symptomatic renal involvement in BD patients.Methods Two hundred and eleven patients who fulfilled the International Behçet's Disease criteria were included in the study. After urine analysis, five of 12 patients who were found to have proteinuria underwent renal biopsy, while 199 patients without proteinuria were investigated for microalbuminuria (MA).Results A tota...
Source: Journal of the European Academy of Dermatology and Venereology - November 17, 2009 Category: Dermatology Authors: M Kavala, F Menteş, E Kocaturk, H Ergin, B Can, Z Turkoglu, S Südogan Source Type: journals

Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNPEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this report, we describe the clinical, histopathological and pathological prion protein (PrPSc) characteristics of two Dutch patients carrying novel adjacent stop codon mutations in the C-terminal part of PRNP, resulting in either case in hereditary prion protein amyloidoses, but with strikingly different clinicopathological phenotypes. The patient with the shortest disease duration (27 months) carried a Y226X mutation and showed PrP-CAA without any neurofibrillary lesions, whereas the patient with the longest disease duration (72 months) had a Q227X mutation and showed an unusual Gerstmann-Sträussler-Schei...
Source: Acta Neuropathologica - November 12, 2009 Category: Neurology Tags: Acta Neuropathologica Source Type: journals

Molecular basis for insulin fibril assembly [Biophysics_And_Computational_Biology]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In the rare medical condition termed injection amyloidosis, extracellular fibrils of insulin are observed. We found that the segment of... (Source: Proceedings of the National Academy of Sciences)
Source: Proceedings of the National Academy of Sciences - November 10, 2009 Category: Science Authors: Ivanova, M. I., Sievers, S. A., Sawaya, M. R., Wall, J. S., Eisenberg, D. Tags: Biophysics_And_Computational_Biology Source Type: journals

Amyloidosis relapsing after autologous stem cell transplantation treated with Bortezomib: normalization of detectable serum-free light chains and reversal of tissue damage with improved suitability for transplant.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19903681 [PubMed - as supplied by publisher] (Source: Haematologica)
Source: Haematologica - November 10, 2009 Category: Hematology Authors: Brunvand MW, Bitter M Tags: Haematologica Source Type: journals

Cerebral amyloidosis: amyloid subunits, mutants and phenotypes.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cerebral amyloid diseases are part of a complex group of chronic and progressive entities bracketed together under the common denomination of protein folding disorders and characterized by the intra- and extracellular accumulation of fibrillar aggregates. Of the more than 25 unrelated proteins known to produce amyloidosis in humans only about a third of them are associated with cerebral deposits translating in cognitive deficits, dementia, stroke, cerebellar and extrapyramidal signs, or a combination thereof. The familial forms reviewed herein, although infrequent, provide unique paradigms to examine the role of amyloi...
Source: Cellular and Molecular Life Sciences : CMLS - November 7, 2009 Category: Cytology Authors: Rostagno A, Holton JL, Lashley T, Revesz T, Ghiso J Tags: Cell Mol Life Sci Source Type: journals

The “other” vasculitis syndromes and kidney involvementEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and/or are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of this group as it involves vessels of any size on both the arterial and venous side. In addition to renal vascular involvement, Behçet disease may involve the kidney through glomerulonephritis, secondary amyloidosis and, rarely, tubulointerstital involvement. Vasculitis secondary to infections, malignancy, and drugs are not common among children. Howe...
Source: Pediatric Nephrology - November 6, 2009 Category: Urology & Nephrology Tags: Pediatric Nephrology Source Type: journals

Amyloid in bone marrow smears of patients affected by multiple myelomaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract Systemic AL amyloidosis is associated with nearly 15% of cases of multiple myeloma, but data on the frequency and significance of amyloid deposits in the bone marrow of patients affected by multiple myeloma without clinical signs of systemic amyloidosis are scanty. Bone marrow smears of 166 unselected patients affected by multiple myeloma (126 at diagnosis and 40 after treatment) were stained with Congo red and studied by transmission and birefringence microscopy. Both focal and diffuse storages were considered positive. Overall, 67 patients were positive and 99 were negative to Congo red and apple-...
Source: Annals of Hematology - November 5, 2009 Category: Hematology Tags: Annals of Hematology Source Type: journals

Restrictive cardiomyopathiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
constitute a heterogenous group of heart muscle conditions that all have, in common, the symptoms of heart failure. Diastolic dysfunction with preserved systolic function is often the only echocardiographic abnormality that may be noted, although systolic dysfunction may also be an integral part of some specific pathologies, particularly in the most advanced cases such as amyloid infiltration of the heart. By far, the majority of restrictive cardiomyopathies are secondary to a systemic disorder such as amyloidosis, sarcoidosis, scleroderma, haemochromatosis, eosinophilic heart disease, or as a result of radiation treatmen...
Source: European Journal of Echocardiography - November 4, 2009 Category: Cardiology Authors: Nihoyannopoulos, P., Dawson, D. Tags: BRITISH SOCIETY OF ECHOCARDIOGRAPHY AUTUMN SUPPLEMENT 2009 Source Type: journals

Tissue Doppler imaging and contrast-enhanced cardiac magnetic resonance in primary cardiac amyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A 64-year-old female presented with biventricular heart failure. Echocardiography revealed features suggestive of amyloidosis, including segmental impairment of longitudinal strain (rate) in the septal and anterior segments, which matched delayed gadolinium-enhanced cardiac magnetic resonance. Guided endomyocardial biopsy confirmed the presence of perivascular amyloid deposits. Tissue Doppler and gadolinium-enhanced magnetic resonance may be helpful in delineating cardiac amyloidosis. (Source: European Journal of Echocardiography)
Source: European Journal of Echocardiography - November 4, 2009 Category: Cardiology Authors: Smedema, J.-P., James, R. Tags: CASE REPORTS Source Type: journals

Chapter 6: The chaperone action of Clusterin and its putative role in quality control of extracellular protein folding.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The function(s) of clusterin may depend upon its topological location. A variety of intracellular "isoforms" of clusterin have been reported but further work is required to better define their identity. The secreted form of clusterin has a potent ability to inhibit both amorphous and amyloid protein aggregation. In the case of amorphous protein aggregation, clusterin forms stable, soluble high-molecular-weight complexes with misfolded client proteins. Clusterin expression is increased during many types of physiological and pathological stresses and is thought to function as an extracellular chaperone (EC). The patholog...
Source: Advances in Cancer Research - November 4, 2009 Category: Cancer & Oncology Authors: Wyatt A, Yerbury J, Poon S, Dabbs R, Wilson M Tags: Adv Cancer Res Source Type: journals

Cardiac transplantation should be considered in selected patients with either AL or hereditary forms of amyloidosis: the UK National Amyloidosis Centre experienceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Internal Medicine Journal)
Source: Internal Medicine Journal - November 4, 2009 Category: Internal Medicine Authors: S. D. J. Gibbs, P. T. Sattianayagam, P. N. Hawkins, J. D. Gillmore Tags: Letters to the Editor Source Type: journals

Heart rate variability in familial Mediterranean feverEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, patients with FMF who are continuously treated with low-dose colchicine have not developed amyloidosis and have normal HRV parameters in the supine and upright position. Further investigation of occult dysautonomia in FMF is needed. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00296-009-1214-yAuthors Naomi Nussinovitch, Chaim Sheba Medical Center Hypertension Unit, Department of Internal Medicine D Tel Hashomer IsraelAvi Livneh, Sheba Medical Center, The Heller Institute of Medical Research Department of Medicine F Tel Hashomer IsraelKeren Katz, Tel Aviv University Sackler Faculty...
Source: Rheumatology International - November 2, 2009 Category: Rheumatology Tags: Rheumatology International Source Type: journals

Alnylam Presents New Pre-Clinical Data on ALN-TTR, an RNAi Therapeutic for the Treatment of Transthyretin-Mediated AmyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
– New Data Demonstrate Durable In Vivo Efficacy for ALN-TTR – CAMBRIDGE, Mass.--(BUSINESS WIRE)--Nov 2, 2009 - Alnylam Pharmaceuticals, Inc. (Nasdaq: ALNY), a leading RNAi therapeutics company, announced today that it presented new... (Source: Drugs.com - Clinical Trials)
Source: Drugs.com - Clinical Trials - November 2, 2009 Category: Pharmaceuticals Source Type: clinical trials

Unusual combination of tracheobronchopathia osteochondroplastica and AA amyloidosis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this report, a case initially suspected to be asthma bronchiole that could not be treated, was radiologically diagnosed as TO, and also secondary amyloidosis is presented. A 53 years, man patient reported a 3 years history of dyspnea. Pulmonary function tests (PFTs) showed an obstructive pattern. Chest X-rays revealed right middle lobe atelectasis. FOB and CT detected nodular lesions in the trachea and in the anterior and lateral walls of the main bronchi. AA amyloidosis was confirmed by endobronchial biopsy. In the abdominal fat pad biopsy, amyloidosis was not detected. Asthma bronchiole was excluded by PFTs. This case...
Source: Yonsei Medical Journal - October 31, 2009 Category: Universities & Medical Training Authors: Kirbaş G, Dağli CE, Tanrikulu AC, Yildiz F, Bükte Y, Senyiğit A, Kiyan E Tags: Yonsei Med J Source Type: journals

Binding of epigallocatechin-3-gallate to transthyretin modulates its amyloidogenicityEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study we tested (−)-epigallocatechin-3-gallate (EGCG), the most abundant catechin of green tea, as an inhibitor of TTR amyloid formation. We demonstrate that EGCG binds to TTR “in vitro” and “ex vivo” and that EGCG inhibits TTR aggregation “in vitro” and in a cell culture system. These findings together with the low toxicity of the compound raise the possibility of using EGCG in a therapeutic approach for familial amyloidotic polyneuropathy, the most frequent form of hereditary TTR amyloidosis.Structured summary: MINT-7294529: TTR (uniprotkb:P02766) and TTR (uniprotkb:P02766) bind (MI:0407) by comigra...
Source: FEBS Letters - October 26, 2009 Category: Biochemistry Authors: Nelson Ferreira, Isabel Cardoso, Maria Rosário Domingues, Rui Vitorino, Margarida Bastos, Guangyue Bai, Maria João Saraiva, Maria Rosário Almeida Tags: Research Letters with SDA Source Type: journals

Pseudomembranous Aspergillus tracheobronchitis in a heart transplant recipientEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report the case of a patient who received a heart transplant (HT) because of cardiac amyloidosis and who developed pseudomembranous AT. Possible risk factors concurrent in this case were splenectomy, lymphocytopenia, and previous cytomegalovirus infection. Chest computed tomography scan showed thickening of the left bronchi and a 'tree-in-bud' pattern with multiple small nodules. Bronchoscopic examination revealed raised yellowish pseudomembranous plaques on the tracheobronchial tree. Bronchoalveolar lavage and aspirate cultures yielded Aspergillus fumigatus. The patient recovered with voriconazole. Clinicians should be...
Source: Transplant Infectious Disease - October 21, 2009 Category: Transplant Surgery Authors: A. Ramos, J. Segovia, M. Gómez-Bueno, C. Salas, M.T. Lázaro, I. Sanchez, L. Pulpón Source Type: journals

Amyloidosis of lacrimal glandEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This report describes a female patient with isolated amyloidosis of the lacrimal gland. A 45-year-old Indian woman presented with a swelling over the left lacrimal gland region. Computed tomography showed uniform enlargement of the lacrimal gland. A lacrimal gland biopsy revealed amyloidosis. No systemic involvement was detected on further investigation. To our knowledge, this is the first report of lacrimal gland amyloidosis from India and our report also highlights the importance of lacrimal gland biopsy in diagnosing lacrimal gland masses. (Source: Indian Journal of Ophthalmology)
Source: Indian Journal of Ophthalmology - October 21, 2009 Category: Opthalmology Authors: Prabhakaran Venkatesh C, Babu Kalpana, Mahadevan Anitha, Murthy Sowmya Raveendra Source Type: journals

Dietary composition modulates brain mass and amyloid beta levels in a mouse model of aggressive Alzheimer's amyloid pathologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
ObjectiveAlzheimer's disease (AD) is a progressive neurodegenerative disease of the central nervous system (CNS). Recently, an increased interest in the role diet plays in the pathology of AD has resulted in a focus on the detrimental effects of diets high in cholesterol and fat and the beneficial effects of caloric restriction. The current study examines how dietary composition modulates cerebral amyloidosis and neuronal integrity in the TgCRND8 mouse model of AD. Methods: From 4 wks until 18 wks of age, male and female TgCRND8 mice were maintained on one of four diets: (1) reference (regular) commercial chow; (2) high fa...
Source: Molecular Neurodegeneration - October 20, 2009 Category: Neurology Authors: Steve PedriniCarlos ThomasHannah BrautigamJames SchmeidlerLap HoPaul FraserDavid WestawayPeter St George HyslopRalph MartinsJoseph BuxbaumGiulio PasinettiDara DicksteinPatrick HofMichelle EhrlichSam Gandy Source Type: journals

IgA nephropathy associated with ankylosing spondylitis is not controlled by infliximab therapyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report here a case of IgA nephropathy associated with AS that became symptomatic, whereas infliximab therapy efficiently controlled the rheumatological disease. This suggests that even though infliximab therapy effectively controls rheumatological manifestations, it may not be able to prevent IgA nephropathy associated with AS. Thus, this case report illustrates the complexity of the physiopathology of both diseases. (Source: Nephrology Dialysis Transplantation)
Source: Nephrology Dialysis Transplantation - October 15, 2009 Category: Urology & Nephrology Authors: Jacquet, A., Francois, H., Frangie, C., Yahiaoui, Y., Ferlicot, S., Micelli, C., Mariette, X., Durrbach, A. Tags: Exceptional Case Source Type: journals

Ultrasonography of Bovine Urinary Tract DisordersEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This article describes the anatomy, scanning technique, indications, limitations, normal and pathologic sonographic appearance of the bovine urinary tract. References from horses and humans are included, especially when the sonographic findings in these species may complement the understanding of similar diseases reported in cattle. (Source: Veterinary Clinics of North America: Food Animal Practice)
Source: Veterinary Clinics of North America: Food Animal Practice - October 12, 2009 Category: Veterinary Research Authors: Martina Floeck Source Type: journals

Glucocorticoids/prednisolone: Masking of dialysis-related amyloidosis: case reportEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Reactions)
Source: Reactions - October 12, 2009 Category: Drugs & Pharmacology Tags: Short communication Source Type: journals

A case with rheumatoid arthritis and systemic reactive AA amyloidosis showing rapid regression of amyloid deposition on gastroduodenal mucosa after a combined therapy of corticosteroid and etanerceptEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract Systemic reactive amyloid A (AA) amyloidosis is one of the critical complications associated with rheumatoid arthritis (RA). Recently, there are several useful reports of anti-tumor necrosis factor therapy for RA-related systemic reactive AA amyloidosis patients. However, the time-kinetic transition between effective anti-inflammatory therapies and regression of AA amyloid deposits remains uncertain. Here, we report a RA patient with systemic reactive AA amyloidosis who was successfully treated with prednisolone and etanercept, showing marked regression of gastroduodenal mucosal amyloid deposits wit...
Source: Rheumatology International - October 10, 2009 Category: Rheumatology Tags: Rheumatology International Source Type: journals

How I treat amyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Amyloidosis is an uncommon disorder in which proteins change conformation, aggregate, and form fibrils that infiltrate tissues, leading to organ failure and death. The most frequent types are light-chain (AL) derived from monoclonal B-cell disorders producing amyloidogenic immunoglobulin light chains, and the hereditary and "senile systemic" (ATTR) variants from mutant and wild-type transthyretin (TTR). Diagnosis requires tissue biopsy. AL is more frequent and causes more organ disease than ATTR. Although both can cause cardiomyopathy and heart failure, AL progresses more quickly, so survival depends on timely diagnosis. T...
Source: Blood - October 8, 2009 Category: Hematology Authors: Comenzo, R. L. Tags: How I Treat, Free Research Articles, Lymphoid Neoplasia, Clinical Trials and Observations Source Type: journals

Persistent Troponin Elevation in a Patient with Cardiac AmyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A 79-year-old patient repeatedly presented with chest discomfort and dyspnea on exertion. With echocardiography a prominent left ventricular and septal hypertrophy was detected with reduced left ventricular function. Despite successful revascularization and excellent results after stenting, the patient showed persistently elevated troponin levels. To investigate the abnormal findings of persistent troponin elevation, septal hypertrophy, and heart failure we performed endomyocardial biopsies which showed widespread myocardial amyloidosis. Amyloid subtyping revealed transthyretin amyloidosis. This is the first case showing p...
Source: Clinical Cardiology - October 7, 2009 Category: Cardiology Authors: Bjoern F. Kraemer, Peter Seizer, Tobias Geisler, Karin Klingel, Reinhard Kandolf, Stephan Lindemann, Meinrad Gawaz Source Type: journals

Essential role of tau phosphorylation in adult hippocampal neurogenesisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
An increased hippocampal neurogenesis has been observed in Alzheimer disease (AD), the most common neurodegenerative disorder characterized with accumulation of [beta]-amyloid (A[beta]) and hyperphosphorylated tau (p-tau). Studies in transgenic mouse models suggest that the amyloidosis suppresses adult neurogenesis. Although emerging evidence links tau to neurodevelopment, the direct data regarding tau phosphorylation in adult neurogenesis is missing. Here, we found that the immature neurons, identified by doublecortin (DCX) and neurogenic differentiation factor (neuroD), were only immunoreactive to p-tau but not to the no...
Source: Hippocampus - October 7, 2009 Category: Neurology Authors: Xiao-Ping Hong, Cai-Xia Peng, Wei Wei, Qing Tian, Ying-Hua Liu, Xiu-Qing Yao, Yao Zhang, Fu-Yuan Cao, Qun Wang, Jian-Zhi Wang Source Type: journals

Localized amyloidosis at the site of enfuvirtide injection.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19805777 [PubMed - in process] (Source: Annals of Internal Medicine)
Source: Annals of Internal Medicine - October 5, 2009 Category: Internal Medicine Authors: Morilla ME, Kocher J, Harmaty M Tags: Ann Intern Med Source Type: journals

A novel Y331X nonsense mutation in TNFRSF1A gene in two unrelated Turkish families with periodic fever syndromeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, in Turkish patients, with dominantly inherited recurrent fever, TRAPS is a diagnosis worthy of attention and novel mutations have to be reported with phenotype associations. (Source: International Journal of Immunogenetics)
Source: International Journal of Immunogenetics - October 4, 2009 Category: Genetics & Stem Cells Authors: N. Kutukculer, N. Gulez, N. Karaca, G. Aksu, A. Berdeli Source Type: journals

Conformational diseases: looking into the eyes.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conformational diseases, a general term comprising more than 40 disorders are caused by the accumulation of unfolded or misfolded proteins. Improper protein folding (misfolding) as well as accrual of unfolded proteins can lead to the formation of disordered (amorphous) or ordered (amyloid fibril) aggregates. The gradual accumulation of protein aggregates and the acceleration of their formation by stress explain the characteristic late or episodic onset of the diseases. The best studied in this group are neurodegenerative diseases and amyloidosis accompanied by the deposition of a specific aggregation-prone proteins or ...
Source: Brain Research Bulletin - October 2, 2009 Category: Neurology Authors: Surguchev A, Surguchov A Tags: Brain Res Bull Source Type: journals

Vitreous Amyloidosis in Two Sisters as the Indication of Transthyretin-Related Familial Form of Systemic Amyloidosis Among Liver Transplantation CandidatesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Vitreous amyloidosis, a rare condition characteristic of the familial form of systemic amyloidosis with polyneuropathy, is caused by a mutation in the transthyretin (TTR) gene. Herein we have presented 2 sisters with involvement of the vitreous body, which prompted a subsequent diagnosis of the TTR-related, familial form of systemic amyloidosis. Due to the progressive character of the disease and poor prognosis, the patients have been considered for liver transplantation, which at present is the only treatment option for this disease. (Source: Transplantation Proceedings)
Source: Transplantation Proceedings - October 1, 2009 Category: Transplant Surgery Authors: R. Niemczyk, J. Brydak-Godowska, D. Kęcik, T. Wagner, P. Lewandowski, M. Kęcik, D. Zygier, U. Ołdakowska-Jedynak Tags: Liver Transplantation Source Type: journals

Multiple bony amyloidomas as an initial presentation of myeloma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This report may represent the first description of macrofocal myeloma associated with amyloid deposition in an older individual. PMID: 19858048 [PubMed - in process] (Source: Clinical Lymphoma and Myeloma)
Source: Clinical Lymphoma and Myeloma - October 1, 2009 Category: Cancer & Oncology Authors: Kirkel DM, Paal E, Ascensao J, Schechter GP Tags: Clin Lymphoma Myeloma Source Type: journals

Effective Anti-TNF-{alpha} Therapy Can Induce Rapid Resolution and Sustained Decrease of Gastroduodenal Mucosal Amyloid Deposits in Reactive Amyloidosis Associated with Rheumatoid Arthritis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
CONCLUSION: Our results indicate a striking effect of anti-TNF therapy for rapid removal and sustained disappearance of amyloid deposits in gastric mucosal tissue with amelioration of renal functions in patients with reactive amyloidosis due to RA. PMID: 19797512 [PubMed - as supplied by publisher] (Source: J Rheumatol)
Source: J Rheumatol - September 30, 2009 Category: Rheumatology Authors: Kuroda T, Wada Y, Kobayashi D, Murakami S, Sakai T, Hirose S, Tanabe N, Saeki T, Nakano M, Narita I Tags: J Rheumatol Source Type: journals

Renal AA amyloidosis secondary to morbid obesity?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a case of a young patient with morbid obesity and hypertension who was admitted to our hospital for acute renal insufficiency associated with nephrotic range proteinuria which developed while on antibiotic treatment for a respiratory infection. AA Amyloidosis was diagnosed by renal biopsy. Based on recent evidence we hypothesize that morbid obesity could be the underlying cause of the deposit disease. PMID: 19825338 [PubMed - in process] (Source: Clinical Nephrology)
Source: Clinical Nephrology - September 30, 2009 Category: Urology & Nephrology Authors: Alsina E, Martin M, Panadés M, Fernández E, Tags: Clin Nephrol Source Type: journals

Prevention of Amyloidosis in Familial Mediterranean Fever with Colchicine: A Case-Control Study in ArmeniaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Med Princ Pract 2009;18:441-446 (DOI:10.1159/000235892) (Source: Medical Principles and Practice : Last 20 articles)
Source: Medical Principles and Practice : Last 20 articles - September 29, 2009 Category: Internal Medicine Source Type: journals

Aggregates of denatured proteins stimulate nitric oxide and superoxide production in macrophagesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion Our results point at similarities in macrophage responses to denatured nonamyloidogenic proteins and to amyloid fibrils. Thus, the tissue injury observed in amyloidosis may result from overstimulation of mechanisms that, under physiological conditions, enable macrophages to recognize and remove denatured proteins. Content Type Journal ArticleCategory Original Research PaperDOI 10.1007/s00011-009-0096-5Authors Szczepan Jozefowski, Jagiellonian University School of Medicine Department of Immunology Czysta 18 St. 31-121 Cracow PolandJanusz Marcinkiewicz, Jagiellonian University School of Medic...
Source: Inflammation Research - September 29, 2009 Category: Research Tags: Inflammation Research Source Type: journals

Susceptibility to AA amyloidosis in rheumatic diseases: A critical overviewEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Arthritis Care and Research)
Source: Arthritis Care and Research - September 28, 2009 Category: Rheumatology Authors: Laura Obici, Sara Raimondi, Francesca Lavatelli, Vittorio Bellotti, Giampaolo Merlini Tags: Review Articles Source Type: journals

Staged heart transplantation and chemotherapy as a treatment option in patients with severe cardiac light-chain amyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion Heart transplantation in advanced cardiac amyloidosis is a promising approach to interrupting the vicious circle of ineligibility for potential curative chemotherapeutic treatment and extremely poor prognosis of cardiac amyloidosis without chemotherapy. Highly urgent heart transplantation combined with subsequent HDM–ASCT appears to offer a successful treatment option to improve the poor outcome of cardiac amyloidosis. However, it should be restricted to highly selected patients in specialized centres. (Source: European Journal of Heart Failure)
Source: European Journal of Heart Failure - September 28, 2009 Category: Cardiology Authors: Kristen, A. V., Sack, F.-U., Schonland, S. O., Hegenbart, U., Helmke, B. M., Koch, A., Schnabel, P. A., Rocken, C., Hardt, S., Remppis, A., Goldschmidt, H., Karck, M., Ho, A. D., Katus, H. A., Dengler, T. J. Tags: HEART TRANSPLANTATION Source Type: journals

Cerebral Amyloidosis: Postmortem Detection with Human 7.0-T MR Imaging System [Neuroradiology]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: The finding of postmortem susceptibility-weighted changes in the cerebral cortex of patients with cerebral amyloidosis with a human 7.0-T MR imaging system opens up the possibility of obtaining in vivo radiologic evidence of cerebral amyloid β deposition. Supplemental material: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.2533090490/-/DC1 © RSNA, 2009 (Source: Continuous Publishing articles)
Source: Continuous Publishing articles - September 28, 2009 Category: Radiology Authors: van Rooden, S., Maat-Schieman, M. L. C., Nabuurs, R. J. A., van der Weerd, L., van Duijn, S., van Duinen, S. G., Natte, R., van Buchem, M. A., van der Grond, J. Tags: Neuroradiology Source Type: journals

Cerebral Amyloidosis: Postmortem Detection with Human 7.0-T MR Imaging System.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: The finding of postmortem susceptibility-weighted changes in the cerebral cortex of patients with cerebral amyloidosis with a human 7.0-T MR imaging system opens up the possibility of obtaining in vivo radiologic evidence of cerebral amyloid beta deposition. Supplemental material: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.2533090490/-/DC1 (c) RSNA, 2009. PMID: 19789230 [PubMed - as supplied by publisher] (Source: Radiology)
Source: Radiology - September 28, 2009 Category: Radiology Authors: van Rooden S, Maat-Schieman ML, Nabuurs RJ, van der Weerd L, van Duijn S, van Duinen SG, Natté R, van Buchem MA, van der Grond J Tags: Radiology Source Type: journals

Spontaneous gallbladder pathology in baboonsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion Many epidemiologic similarities exist between GBP in baboons and humans suggesting that the baboon may serve as a reliable animal model system for investigating GBP in humans. (Source: Journal of Medical Primatology)
Source: Journal of Medical Primatology - September 27, 2009 Category: Research Authors: J.L. Slingluff, J.T. Williams, L. Blau, A. Blau, E.J Dick Jr, G.B. Hubbard Source Type: journals

Spontaneous pathology of the baboon endocrine systemEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions Endocrine disease in baboons is common and shares clinical and biochemical characteristics with endocrine disease in humans. (Source: Journal of Medical Primatology)
Source: Journal of Medical Primatology - September 27, 2009 Category: Research Authors: R. Guardado-Mendoza, E.J. Dick Jr, L.M. Jimenez-Ceja, A. Davalli, A.O. Chavez, F. Folli, G.B. Hubbard Source Type: journals

Quantitative assessment of myocardial T2 relaxation times in cardiac amyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
To evaluate cardiac MRI (CMR) in the diagnosis of cardiac amyloidosis by comparing the T2 relaxation times of left ventricular myocardium in a pilot patient group to a normal range established in healthy controls.Forty-nine patients with suspected amyloidosis-related cardiomyopathy underwent comprehensive CMR examination, which included assessment of myocardial T2 relaxation times, ventricular function, resting myocardial perfusion, and late gadolinium enhancement (LGE) imaging. T2-weighted basal, mid, and apical left ventricular slices were acquired in each patient using a multislice T2 magnetization preparation spiral se...
Source: Journal of Magnetic Resonance Imaging - September 25, 2009 Category: Radiology Authors: Patrick Sparrow, Afsaneh Amirabadi, Marshall S. Sussman, Narinder Paul, Naeem Merchant Source Type: journals

K3 Fragment of Amyloidogenic β2-Microglobulin Forms Ion Channels: Implication for Dialysis Related AmyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Journal of the American Chemical Society, Volume 0, Issue 0, Articles ASAP (As Soon As Publishable). (Source: Journal of the American Chemical Society)
Source: Journal of the American Chemical Society - September 25, 2009 Category: Chemistry Tags: article Source Type: journals

Cardiac amyloidosis in African Americans: Comparison of clinical and laboratory features of transthyretin V122I amyloidosis and immunoglobulin light chain amyloidosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions: ATTR V122I and AL are equally prevalent as the cause of cardiomyopathy in African Americans referred for a diagnosis of amyloidosis. Available therapy for AL underscores the need for early and accurate determination of amyloid type. (Source: American Heart Journal)
Source: American Heart Journal - September 24, 2009 Category: Cardiology Authors: Lawreen H. Connors, Tatiana Prokaeva, Amareth Lim, Roger Théberge, Rodney H. Falk, Gheorghe Doros, Alan Berg, Catherine E. Costello, Carl O'Hara, David C. Seldin, Martha Skinner Tags: Genetics Source Type: journals

MEFV mutations in Egyptian patients suffering from familial Mediterranean fever: analysis of 12 gene mutationsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of the study is to screen 12 MEFV gene mutations in Egyptian patients with familial Mediterranean fever (FMF) and to study the initial hypothesis that the phenotypic expression of the disease may be attributable to the existence of a particular mutation. We enrolled 136 Egyptian patients (74 males, and 62 females) with a clinical diagnosis of FMF. DNA was amplified by PCR and subjected to reverse hybridization for the detection of 12 MEFV gene mutations. The phenotypic expression of the disease was compared in two subgroups according to the presence of homozygote E148Q and M694V gene mutations. The most ...
Source: Rheumatology International - September 24, 2009 Category: Rheumatology Tags: Rheumatology International Source Type: journals

Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behçet’s diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We present a teenager who had severe FMF and Behçet’s disease and developed moderate proteinuria. Renal biopsy showed secondary amyloidosis. Anakinra was started at 1 mg/kg/day subcutaneously along with colchicine treatment. The clinical response was excellent. Acute phase reactants decreased. The level of proteinuria and renal functions remained stable and the hypoalbuminemia returned to normal. Her clinical and laboratory symptoms returned when anakinra had to be stopped at 6 months. Thus, the drug was restarted and she is now clinically in excellent condition a year after the start of therapy. She has ...
Source: Clinical Rheumatology - September 23, 2009 Category: Rheumatology Tags: Clinical Rheumatology Source Type: journals

[Solitary pulmonary nodule.]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Primary pulmonary amyloidosis is rare. Three patterns of involvement have been described: tracheobronchial, nodular and diffuse parenchymal. The nodular parenchymal amyloid deposits are often multiple, much less common focal. We hereby present a case of a 70 year old patient, a former smoker, with coincidentally diagnosed solitary, pulmonary nodule, a rather common finding in CT. The list of differential diagnosis is long, as shown above, the amyloidosis is a quite uncommon cause, but it should be kept in mind. PMID: 19774519 [PubMed - in process] (Source: Praxis)
Source: Praxis - September 22, 2009 Category: Journals (General) Authors: Schaeffler F, Kos S Tags: Praxis (Bern 1994) Source Type: journals

Amyloidosis: an unusual case of persistent oral ulcerationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a case of systemic amyloidosis, with an unusual oral presentation, in a 70-year-old patient suffering from light chain myeloma. The patient presented with extensive ulceration of the tongue and alveolar ridges, and a large swelling in the floor of mouth. Incisional biopsies of the tongue and floor of mouth confirmed amyloid deposition within the tissues with evidence of necrotic ulceration. Amyloid deposition in the oral cavity usually manifests as macroglossia, however it can present elsewhere in the mouth as nodular or plaquelike lesions. Ulceration is a rare finding. This case highlights the variable nature of...
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - September 22, 2009 Category: ENT & OMF Authors: Sarah Francesca Viggor, Cristina Frezzini, Paula M. Farthing, Christine O. Freeman, Christine M. Yeoman, Martin H. Thornhill Tags: Online Only Articles Source Type: journals

Frequencies and Types of Arrhythmias in Patients With Systemic Light-Chain Amyloidosis With Cardiac Involvement Undergoing Stem Cell Transplantation on Telemetry MonitoringEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cardiac patients with systemic light-chain amyloidosis have a high incidence of arrhythmias and arrhythmia-related death. We aimed to describe the arrhythmias, determine patient characteristics associated with the development of ventricular arrhythmias, and the utility of telemetric monitoring in patients with cardiac involvement due to AL amyloidosis undergoing stem cell transplantation (SCT). Arrhythmia events of 24 consecutive cardiac patients with AL who underwent SCT with continuous telemetric monitoring were retrospectively reviewed. The relation between number and severity of ventricular arrhythmias (ventricular tac...
Source: The American Journal of Cardiology - September 22, 2009 Category: Cardiology Authors: Yuliya B. Goldsmith, Jennifer Liu, Joanne Chou, James Hoffman, Raymond L. Comenzo, Richard M. Steingart Tags: Cardiomyopathy Source Type: journals