MedWorm: Amyloidosis

Drug targets for amyloidosis.

Biochemical Society Transactions — Sun, 21 Mar 2010 03:07:16 +0100

Authors: Kolstoe SE, Wood SP The amyloid hypothesis indicates that protein misfolding is at the root of many neurodegenerative disorders. Small molecules targeting the formation, clearance, aggregation to toxic oligomers or SOD (superoxide dismutase)-like activities of Abeta (amyloid beta-peptide) 1-42 have provided encouraging candidates for AD (Alzheimer's disease) medicines in animal models, although none have yet proved to be effective in human trials. We have been investigating approaches to treat systemic amyloidoses, conditions that show common features with some CNS (central nervous system) disorders. For TTR (transthyretin) amyloidosis, we are seeking small molecule compounds that stabilize the amyloidogenic protein and either prevent its structural transition to the crossed b...

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Stem cell transplantation in patients with autonomic neuropathy due to primary (AL) amyloidosis

Neurology — Mon, 15 Mar 2010 20:00:56 +0100

Conclusions: Patients with autonomic neuropathy secondary to AL amyloidosis can undergo autologous stem cell transplantation with relative safety. Autonomic neuropathy is an independent, adverse determinant of survival in these patients. (Source: Neurology)

Excellent therapeutic effect of tocilizumab on intestinal amyloid a deposition secondary to active rheumatoid arthritis

Clinical Rheumatology — Sun, 14 Mar 2010 12:25:46 +0100

Abstract A 64-year-old woman suffering from progressive amyloid A (AA) amyloidosis of the gastrointestinal (GI) tract, associated with active rheumatoid arthritis, was transferred to our hospital due to hypovolemic shock. Although intensive care, including treatment with prednisolone and methotrexate, improved the hypovolemic shock, paralytic ileus became dominant instead of the marked diarrhea, suggesting the terminal stage of AA amyloidosis of the GI tract. Thus, we administered tocilizumab, a humanized anti-interleukin 6 receptor antibody (8 mg/kg, repeated every 4 weeks). Two weeks after the first injection of tocilizumab, serum AA rapidly returned to their normal ranges in accordance with the amelioration of paralytic ileus and systemic joint pain. Surprisin...

Is type 2 diabetes an amyloidosis and does it really matter (to patients)?

Diabetologia — Sun, 14 Mar 2010 11:24:43 +0100

Content Type Journal ArticleCategory CommentaryDOI 10.1007/s00125-010-1715-yAuthors G. J. S. Cooper, University of Auckland School of Biological Sciences, and Maurice Wilkins Centre of Excellence for Molecular Biodiscovery, Faculty of Science Auckland New ZealandJ. F. Aitken, University of Auckland School of Biological Sciences, and Maurice Wilkins Centre of Excellence for Molecular Biodiscovery, Faculty of Science Auckland New ZealandS. Zhang, University of Auckland School of Biological Sciences, and Maurice Wilkins Centre of Excellence for Molecular Biodiscovery, Faculty of Science Auckland New Zealand Journal DiabetologiaOnline ISSN 1432-0428Print ISSN 0012-186X (Source: Diabetologia)

Hereditary amyloidosis of the Finnish type in a German family: Clinical and electrophysiological presentation

Muscle and Nerve — Fri, 12 Mar 2010 00:00:00 +0100

Hereditary amyloidosis of the Finnish type (HAF, or familial amyloid polyneuropathy type IV) is an autosomal dominant disease that has been described most commonly in the Finnish population but has also been found in some other countries. Herein we report the first German family whose members suffer from this condition. There are no known Finnish ancestors. We performed clinical and electrophysiological examinations in 22 members of this family. All symptomatic family members suffered from facial palsy, and most of them had peripheral neuropathy. One patient had confirmed corneal lattice dystrophy. Additional symptoms were hypoglossal nerve involvement in 5 patients and oculomotor nerve palsy in 1 patient. The lips of all older patients appeared thickened. The causative G654A mutation in t...

Iowa Variant of Familial Alzheimer's Disease: Accumulation of Posttranslationally Modified A{beta}D23N in Parenchymal and Cerebrovascular Amyloid Deposits.

The American Journal of Pathology — Fri, 12 Mar 2010 00:00:00 +0100

Authors: Tomidokoro Y, Rostagno A, Neubert TA, Lu Y, Rebeck GW, Frangione B, Greenberg SM, Ghiso J Mutations within the amyloid-beta (Abeta) sequence, especially those clustered at residues 21-23, which are linked to early onset familial Alzheimer's disease (AD), are primarily associated with cerebral amyloid angiopathy (CAA). The basis for this predominant vascular amyloid burden and the differential clinical phenotypes of cerebral hemorrhage/stroke in some patients and dementia in others remain unknown. The AbetaD23N Iowa mutation is associated with progressive AD-like dementia, often without clinically manifested intracerebral hemorrhage. Neuropathologically, the disease is characterized by predominant preamyloid deposits, severe CAA, and abundant neurofibrillary tangles in the pres...

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Effects of the main green tea polyphenol epigallocatechin-3-gallate on cardiac involvement in patients with AL amyloidosis

Clinical Research in Cardiology — Thu, 11 Mar 2010 02:39:48 +0100

Conclusion Consumption of green tea polyphenol EGCG in patients with cardiac involvement with AL amyloidosis causes a significant decrease in left ventricular wall thickness and mass, as well as an improvement in NYHA functional classification and left ventricular ejection fraction. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00392-010-0142-xAuthors Derliz Mereles, University of Heidelberg Department of Internal Medicine III, Cardiology, Angiology and Pneumology In Neuenheimer Feld 410 69120 Heidelberg GermanySebastian J. Buss, University of Heidelberg Department of Internal Medicine III, Cardiology, Angiology and Pneumology In Neuenheimer Feld 410 69120 Heidelberg GermanyStefan E. Hardt, University of Heidelberg Department of Internal Medicine I...

[A three-year-old girl with abdominal pain and fever.]

Tidsskrift for den Norske Laegeforening — Thu, 11 Mar 2010 00:00:00 +0100

Conclusion. Familial Mediterranean fever is a rare disorder in Norway but frequent in many Mediterranean countries. Common symptoms are recurrent episodes of abdominal pain, chest pain, joint pain and fever. Treatment with colchicine reduces inflammation and the risk of developing amyloidosis. PMID: 20224617 [PubMed - as supplied by publisher] (Source: Tidsskrift for den Norske Laegeforening)

Localized Amyloidosis of the Upper Urinary Tract: A Case Series of Three Patients Managed with Reconstructive Surgery or Surveillance

Journal of Endourology — Wed, 10 Mar 2010 21:04:22 +0100

Journal of Endourology , Vol. 0, No. 0. (Source: Journal of Endourology)

Familial Mediterranean fever gene mutation frequencies and genotype–phenotype correlations in the Aegean region of Turkey

Rheumatology International — Wed, 10 Mar 2010 15:39:04 +0100

This study included 308 patients and 164 healthy controls. Patients were divided into three groups according to Tel-Hashomer criteria; definitive, probable, and suspicious. Among the patients, 146 were women (47.4%) and 162 were men (52.6%). The mean age (±SD) of the patients at the diagnosis was 9.6 ± 3.95 (range 0.5–18). The mean age (±SD) at onset of the symptom was 6.2 ± 3.95 (range 1–18). Symptoms were seen earlier onset in definitive group than the suspicious group in our cohort (4.7 ± 3.9 years, 6.6 ± 3.9 years, respectively; P = 0.001). Clinical features were abdominal pain (83.1%), fever (55%), arthritis (17.1%), myalgia (4.5%), pleuritis (10%), and erysipelas—like erythema (7.7%). Fever, arthralgia, arth...

Metastatic Breast Cancer or Multiple Myeloma? Camouflage by Lytic Lesions

Journal of Oncology — Sun, 07 Mar 2010 13:47:51 +0100

This report addresses the pitfalls of overlapping symptoms and the question of which patients with suspected metastatic disease should undergo a biopsy. (Source: Journal of Oncology)

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Renal biopsy is a relatively safe procedure in cases of suspected amyloidosis and a valuable tool in excluding non-AL forms of the disease

Internal Medicine Journal — Fri, 05 Mar 2010 00:00:00 +0100

(Source: Internal Medicine Journal)

Re-Balancing of Inflammation and Abeta Immunity as a Therapeutic for Alzheimer's Disease-View from the Bedside.

CNS and Neurological Disorders Drug Targets — Fri, 05 Mar 2010 00:00:00 +0100

Authors: Fiala M Morbidities of aging and Alzheimer's disease (AD) have been related to defective functions of both T cells and macrophages leading to brain amyloidosis and inflammation. In AD patients, "inflammaging" may be associated with an increase of incompetent memory T cells and inflammatory cytokines produced by macrophages, whereas defective clearance of amyloid-beta 1-42 (Abeta) may be related to defective transcription of immune genes necessary for Abeta phagocytosis, beta-1,4-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase and Toll-like receptors. However, AD shows considerable heterogeneity of disease manifestations and mechanisms. The approaches to re-balancing Abeta immunity and inflammation are being pursued in transgenic animal models and peripheral blood ...

A case of emphysematous pyelonephritis in a patient with rheumatoid arthritis taking corticosteroid and low-dose methotrexate

APLAR Journal of Rheumatology — Thu, 04 Mar 2010 00:00:00 +0100

In this report, we describe a case of emphysematous pyelonephritis in a patient with RA associated with AA amyloidosis and steroid-induced diabetes mellitus who was taking corticosteroid and low-dose methotrexate. (Source: APLAR Journal of Rheumatology)

Hepatopancreatobiliary manifestations and complications associated with inflammatory bowel disease

Inflammatory Bowel Diseases — Wed, 03 Mar 2010 00:00:00 +0100

Abstract: Diseases involving the hepatopancreatobiliary (HPB) system are frequently encountered in patients with inflammatory bowel disease (IBD). Hepatobiliary manifestations constitute some of the most common extraintestinal manifestations of IBD. They appear to occur with similar frequency in patients with Crohn's disease or ulcerative colitis. HPB manifestations may occur in following settings: 1) disease possibly associated with a shared pathogenetic mechanism with IBD including primary sclerosing cholangitis (PSC), small-duct PSC/pericholangitis and PSC/autoimmune hepatitis overlap, acute and chronic pancreatitis related to IBD; 2) diseases which parallel structural and physiological changes seen with IBD, including cholelithiasis, portal vein thrombosis, and hepatic abscess; and 3) ...

Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38{alpha} MAPK pathway [Cell_Biology]

Proceedings of the National Academy of Sciences — Tue, 02 Mar 2010 17:42:13 +0100

Patients with primary (AL) cardiac amyloidosis suffer from progressive cardiomyopathy with a median survival of less than 8 months and... (Source: Proceedings of the National Academy of Sciences)

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Clinical Features and Treatment Response of Light Chain (AL) Amyloidosis Diagnosed in Patients With Previous Diagnosis of Multiple Myeloma

Mayo Clinic Proceedings — Mon, 01 Mar 2010 18:02:04 +0100

CONCLUSION: Delayed onset of AL amyloidosis is rarely seen in patients with MM and requires a high index of suspicion for prompt diagnosis. Outcome of these patients is poor, especially in the presence of cardiac involvement. (Source: Mayo Clinic Proceedings)

Carotid body tumor and amyloidosis: An uncommon association.

Saudi Journal of Kidney Diseases and Transplantation — Mon, 01 Mar 2010 00:00:00 +0100

Authors: Gupta A, Khaira A, Bhowmik D, Agarwal SK, Tiwari SC PMID: 20228525 [PubMed - in process] (Source: Saudi Journal of Kidney Diseases and Transplantation)

Effects of a lipid environment on the fibrillogenic pathway of the N-terminal polypeptide of human apolipoprotein A-I, responsible for in vivo amyloid fibril formation.

European Biophysics Journal : EBJ — Thu, 25 Feb 2010 00:00:00 +0100

Authors: Monti DM, Guglielmi F, Monti M, Cozzolino F, Torrassa S, Relini A, Pucci P, Arciello A, Piccoli R In amyloidosis associated with apolipoprotein A-I (ApoA-I), heart amyloid deposits are mainly constituted by the 93-residue ApoA-I N-terminal region. A recombinant form of the amyloidogenic polypeptide, named [1-93]ApoA-I, shares conformational properties and aggregation propensity with its natural counterpart. The polypeptide, predominantly in a random coil state at pH 8.0, following acidification to pH 4.0 adopts a helical/molten globule transient state, which leads to formation of aggregates. Here we provide evidence that fibrillogenesis occurs also in physiologic-like conditions. At pH 6.4, [1-93]ApoA-I was found to assume predominantly an alpha-helical state, which undergoes ...

Decrease in the rate of secondary amyloidosis in Turkish children with FMF: are we doing better?

European Journal of Pediatrics — Wed, 24 Feb 2010 06:48:41 +0100

Abstract Familial Mediterranean fever (FMF) is the most common autoinflammatory disease in the world. The most serious complication of FMF is the development of secondary amyloidosis. Besides genetic factors, environment has been implicated in the development of this complication. The main objective of this study is to analyze whether there has been a substantial decrease of secondary amyloidosis in Turkey and possible effective factors. For this purpose, clinical features of the patients diagnosed with secondary amyloidosis between the years 1978 and 1990 were compared with those diagnosed between 2000 and 2009. Severity scores were determined by the use of a scoring system modified for children. Median ages of the group diagnosed between 1978 and 1990 (n = 115; 12.1%...

[Peritoneal AA Amyloidosis Simulating Peritoneal Carcinomatosis: A Case Report.]

Radiologia — Wed, 24 Feb 2010 00:00:00 +0100

We present the case of a patient with a history of Hashimoto's thyroiditis and systemic symptoms who presented with abdominal pain. Physical examination detected a palpable mass in the umbilical region. CT showed thickening of the wall of the colon with infiltration of the adjacent peritoneum and extension to the root of the mesentery with abundant ascites. The histological diagnosis was peritoneal AA amyloidosis. PMID: 20188389 [PubMed - as supplied by publisher] (Source: Radiologia)

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A rare case of primary systemic amyloidosis of the neck with massive cervical lymph node involvement: A case report and review of the literature

Leukemia Research — Tue, 23 Feb 2010 14:33:40 +0100

We present the case of a primary systemic AL amyloidosis with hepatic, cervical, retroperitoneal, axillary and inguinal lymphnode localizations, unresponsive to medical therapy and treated with a surgical approach followed by autologous bone marrow transplantation. We review the pertinent literature with exclusive attention to the otorhinolaryngologic aspect. (Source: Leukemia Research)

Bortezomib With or Without Dexamethasone in Primary Systemic (Light Chain) Amyloidosis [Hematologic Malignancies]

Journal of Clinical Oncology — Thu, 18 Feb 2010 23:00:38 +0100

Conclusion Bortezomib with or without dexamethasone is active in AL amyloidosis and induces rapid responses and high rates of hematologic and organ responses. Serial measurement of cardiac biomarkers is a powerful predictor of outcome. (Source: Journal of Clinical Oncology)

Variable intrafamilial expressivity of the rare tumor necrosis factor-receptor associated periodic syndrome-associated mutation I170N that affects the TNFR1A cleavage site

Modern Rheumatology — Thu, 18 Feb 2010 17:55:24 +0100

We report on a 33-year-old female patient with a relatively mild clinical case of TNF-receptor associated periodic syndrome (TRAPS) and her 58-year-old father in whom end-stage renal disease due to TRAPS-related AA-amyloidosis has already developed. TRAPS was caused by a I170N mutation that has previously not been associated with amyloidosis. It remains unclear if an only mildly affected patient such as ours would benefit from treatment considering her father’s severe course of disease. The relevant literature on this problem is reviewed. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10165-010-0273-yAuthors Petra Lehmann, University Clinic of Regensburg Regensburg GermanyBernd Salzberger, University Clinic of Regensburg Department of Internal Medicine I Regensbu...

Native-unlike Long-lived Intermediates along the Folding Pathway of the Amyloidogenic Protein {beta}2-Microglobulin Revealed by Real-time Two-dimensional NMR [Protein Structure and Folding]

Journal of Biological Chemistry — Fri, 12 Feb 2010 14:36:18 +0100

β2-microglobulin (β2m), the light chain of class I major histocompatibility complex, is responsible for the dialysis-related amyloidosis and, in patients undergoing long term dialysis, the full-length and chemically unmodified β2m converts into amyloid fibrils. The protein, belonging to the immunoglobulin superfamily, in common to other members of this family, experiences during its folding a long-lived intermediate associated to the trans-to-cis isomerization of Pro-32 that has been addressed as the precursor of the amyloid fibril formation. In this respect, previous studies on the W60G β2m mutant, showing that the lack of Trp-60 prevents fibril formation in mild aggregating condition, prompted us to reinvestigate the refolding kinetics of wild type and W60G β2m a...

Clinical and pathological features of renal amyloidosis: An analysis of 32 patients in a single Chinese centre

Nephrology — Thu, 11 Feb 2010 00:00:00 +0100

Conclusion: Renal amyloidosis is frequently neglected by local physicians in China. Middle-aged nephrotic patients with weight loss, organ enlargement and monoclonal light chains in serum or urine should be highly suspected of the disease. Renal biopsies, especially electron microscopy, play a crucial role in the early diagnosis of renal amyloidosis. (Source: Nephrology)

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Recurrent Capillary Leak Syndrome Following Bortezomib Therapy in a Patient with Relapsed Myeloma (March).

The Annals of Pharmacotherapy — Thu, 11 Feb 2010 00:00:00 +0100

CONCLUSIONS: Our report demonstrates CLS as an unusual adverse effect of bortezomib. As bortezomib use may become more common, clinicians should be aware of this novel but potentially life-threatening adverse effect. Based on our experience, timely management with steroids is important in dealing with this complication. PMID: 20150504 [PubMed - as supplied by publisher] (Source: The Annals of Pharmacotherapy)

Amyloid oligomers: spectroscopic characterization of amyloidogenic protein states

FEBS Journal — Wed, 10 Feb 2010 00:00:00 +0100

It is assumed that protein fibrils manifested in amyloidosis result from an aggregation reaction involving small misfolded protein sequences being in an 'oligomeric' or 'prefibrillar' state. This review covers recent optical spectroscopic studies of amyloid protein misfolding, oligomerization and amyloid fibril growth. Although amyloid fibrils have been studied using established protein-characterization techniques throughout the years, their oligomeric precursor states require sensitive detection in real-time. Here, fluorescent staining is commonly performed using thioflavin T and other small fluorescent molecules such as 4-(dicyanovinyl)- julolidine and 1-amino-8-naphtalene sulphonate that have high affinity to hydrophobic patches. Thus, populated oligomeric intermediates and related 'pre...

Renal amyloidosis.

British Journal of Hospital Medicine — Wed, 10 Feb 2010 00:00:00 +0100

Authors: Lachmann HJ, Gillmore JD Renal amyloid deposition is common in systemic amyloidosis. Presentation is usually with proteinuria renal impairment. With effective treatment of the underlying amyloidotic condition and good supportive care renal function can stabilize or improve but many patients still progress to end-stage renal failure. PMID: 20220695 [PubMed - as supplied by publisher] (Source: British Journal of Hospital Medicine)

Biochemical basis of the amyloid diseases.

British Journal of Hospital Medicine — Wed, 10 Feb 2010 00:00:00 +0100

This article discusses the pathogenesis, classification system and means of diagnosis of the amyloid diseases. PMID: 20220693 [PubMed - as supplied by publisher] (Source: British Journal of Hospital Medicine)

Role of Cardiac Magnetic Resonance Imaging in the Detection of Cardiac Amyloidosis

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 08 Feb 2010 22:01:04 +0100

Conclusions LGE is common in CA and detects interstitial expansion from amyloid deposition. Global transmural or subendocardial LGE is most common, but suboptimal myocardial nulling and focal patchy LGE are also observed. LGE-CMR may detect early cardiac abnormalities in patients with amyloidosis with normal left ventricular thickness. The presence and pattern of LGE is strongly associated with clinical, morphologic, functional, and biochemical markers of prognosis. (Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

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MR Imaging and Cardiac Amyloidosis: Where to Go From Here?

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 08 Feb 2010 22:01:04 +0100

(Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

Impact of Liver Transplantation on Transthyretin-Related Ocular Amyloidosis in Japanese Patients [Clinical Sciences]

Archives of Opthalmology — Mon, 08 Feb 2010 20:51:02 +0100

Conclusions Patients with FAP who undergo liver transplantation continue to have a long-term risk of severe ocular manifestations, especially vitreous opacities and glaucoma, which can restrict their daily lives, even after liver transplantation. (Source: Archives of Opthalmology)

Using simple artificial intelligence methods for predicting amyloidogenesis in antibodies

BMC Bioinformatics - Latest articles — Mon, 08 Feb 2010 00:00:00 +0100

Conclusions: This exploratory study indicates that both classication methods may be promising in providing straightforward predictions on the amyloidogenicity of a sequence. Nevertheless, the number of available sequences that satisfy the premises of this study are limited, and are consequently smaller than the ideal training set size. Increasing the size of the training set clearly increases the accuracy, and the expansion of the training set to include not only more derivatives, but more alignments, would make the method more sound. The accuracy of the classiers may also be improved when additional factors, such as structural andphysico-chemical data, are considered. The development of this type of classifier has signicant applications in evaluating engineered antibodies, and may be adap...

Cystatin C is a sensitive marker for detecting a reduced glomerular filtration rate when assessing chronic kidney disease in patients with rheumatoid arthritis and secondary amyloidosis.

Scandinavian Journal of Rheumatology — Sun, 07 Feb 2010 13:08:03 +0100

Conclusions: Serum cysC can identify a reduced GFR more accurately than sCr-eGFRs. Serum cysC >1.09 mg/L (i.e. eGFR(Rule)<60 mL/min/1.73 m(2)) could be a marker of a reduced GFR, and serum cysC >1.365 mg/L would strongly suggest a reduced GFR in patients who have RA with secondary amyloidosis. PMID: 20132068 [PubMed - in process] (Source: Scandinavian Journal of Rheumatology)

Considering amyloidosis in patients with clinically unresponsive vitreous inflammation (Oculoleptomeningeal amyloidosis in 3 individuals with transthyretin variant Tyr69His. Vol. 44[3]).

Canadian Journal of Ophthalmology — Mon, 01 Feb 2010 00:00:00 +0100

Authors: Roe RH, Cunningham ET PMID: 20130721 [PubMed - in process] (Source: Canadian Journal of Ophthalmology)

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Genetic predisposition (NLRP3 V198M mutation) for IL-1–mediated inflammation in a patient with Schnitzler syndrome

Journal of Allergy and Clinical Immunology — Mon, 01 Feb 2010 00:00:00 +0100

To the Editor: Schnitzler syndrome is a rare, enigmatic disorder characterized by chronic urticarial rashes and a monoclonal IgM gammopathy, variably combined with intermittent fever, arthralgia or arthritis, lymphadenopathy, hepatomegaly and/or splenomegaly, leukocytosis, and an elevated erythrocyte sedimentation rate. Patients have an excess risk for Waldenström macroglobulinemia and amyloidosis. Both increased secretion of the proinflammatory cytokine IL-1 from PBMCs and efficacy of anakinra (soluble IL-1 receptor antagonist) treatment suggest an important role of IL-1 in the disease process. However, the cause of increased IL-1 secretion has remained obscure. Here, we report on a gain-of-function mutation (V198M) of a gene (nucleotide-binding domain protein and leucine-rich repeat c...

Nonneoplastic Tracheal and Bronchial Stenoses

Thoracic Surgery Clinics — Mon, 01 Feb 2010 00:00:00 +0100

Nonneoplastic stenosis of proximal airways may result from longstanding intubations or tracheostomy, granulomatous infection, or systemic diseases such as relapsing polychondritis, amyloidosis, Wegener's granulomatosis, sarcoidosis, and inflammatory bowel disease. It also may be caused by saber sheath trachea, tracheobronchopathia osteoplastica, or broncholithiasis. An early diagnosis of the tracheal and bronchial stenosis has become possible with the advent of routine CT imaging. Multiplanar and volume rendering reformations after thin collimation MDCT acquisition help assess the location and extent of the stenosis and characterize the presence, distribution, and type of airway wall thickening. They also help surgeons and endoscopists to select adequate procedures and assess the response ...

Systemic amyloidosis: getting to the heart of the matter.

British Journal of Hospital Medicine — Mon, 01 Feb 2010 00:00:00 +0100

Authors: Hawkins PN Just 10-15 years ago, amyloidosis was poorly understood, difficult to diagnose and type, and was widely thought to be inexorable. Specialist clinical services were not available and many patients did not receive any disease-modifying treatment. To the histopathologist, the inert appearance of amyloid deposits without any accompanying tissue reaction seemed to provide further evidence of their irreversibility. Patients with systemic AL (monoclonal immunoglobulin) amyloidosis, the most common and serious type, usually died within 2 years of diagnosis, and often within 6 months when the heart was involved. The symposium in this issue of the journal provides an update on systemic amyloidosis, with a focus on its pathogenesis and clinical management. Cardiac and renal am...

AA-type amyloidosis in association with non-Hodgkin's lymphoma following CMV viremia: Autopsy case

Pathology International — Tue, 26 Jan 2010 00:00:00 +0100

Amyloidosis in non-Hodgkin's lymphoma (NHL) is known to be of the AL type, and AA-type amyloidosis in NHL is extremely rare. Herein is reported an autopsy case of follicular lymphoma that transformed to diffuse large B-cell lymphoma (DLBCL) in a relapse associated with systemic AA amyloidosis. CMV infection in an immunocompromised state with chemotherapy against DLBCL may have been involved in amyloid accumulation. The serum amyloid A (SAA)1 gene polymorphism, SAA1.2/1.3, might have also been another factor in this case, considering the risk of AA amyloidosis in Japanese patients with rheumatoid arthritis. (Source: Pathology International)

Quiz Page February 2010: A Hemodialysis Patient With Muscle Cramps and Malaise After Virtual Colonoscopy

American Journal of Kidney Diseases — Mon, 25 Jan 2010 16:54:55 +0100

A 58-year-old woman with end-stage renal disease caused by AA amyloidosis on hemodialysis therapy for the past 7 years presents with severe malaise, nausea, vomiting, and diffuse muscle cramps. Symptoms began the previous evening after the patient underwent a virtual colonoscopy required for her maintenance on the kidney transplant waiting list. The procedure had been uneventful. (Source: American Journal of Kidney Diseases)

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Alnylam and Collaborators Present Data from Multiple Pre-Clinical and Clinical Programs at RNAi Keystone Symposium

Drugs.com - Clinical Trials — Thu, 21 Jan 2010 12:50:32 +0100

– New Data Include Progress in Delivery, TTR-Mediated Amyloidosis, and Huntington's Disease Programs – – Additionally, Comprehensive Studies Show Superior Performance of “Canonical” siRNAs Compared... (Source: Drugs.com - Clinical Trials)

An overview on frequency of renal biopsy diagnosis in Brazil: clinical and pathological patterns based on 9617 native kidney biopsies

Nephrology Dialysis Transplantation — Thu, 21 Jan 2010 09:33:20 +0100

Conclusion. FSGS was the most frequent primary glomerular disease, followed closely by MN and IgAN. The predominance of FSGS is in accordance with recent studies all over the world that revealed its frequency is increasing. Lupus nephritis predominated among secondary GN in most regions, a finding observed in other studies. (Source: Nephrology Dialysis Transplantation)

Alnylam And Collaborators Present Data From Multiple Pre-Clinical And Clinical Programs At RNAi Keystone Symposium

Health News from Medical News Today — Thu, 21 Jan 2010 04:00:00 +0100

Alnylam Pharmaceuticals, Inc. (Nasdaq: ALNY), a leading RNAi therapeutics company, today announced that it presented data from multiple pre-clinical and clinical programs at the "RNA Silencing: Mechanism, Biology, and Application" Keystone Symposium held January 14-19, 2010 in Keystone, Colorado. Alnylam and its collaborators presented data from Alnylam's therapeutic programs including transthyretin (TTR)-mediated amyloidosis and Huntington's disease, as well as new data on delivery approaches for the systemic delivery of RNAi therapeutics... (Source: Health News from Medical News Today)

[Systemic amyloidosis: Practical diagnosis.]

Nephrologie & Therapeutique — Thu, 21 Jan 2010 00:00:00 +0100

Authors: Noël LH Amyloid deposits have particular ultrastructural appearance with 7 to 10 nm-diameter fibrils. Amyloid is defined by its tinctorial affinity, which includes Congo red positivity, which must polarize and produce apple-green birefringence. Immunohistochemical characterization allows to know the origin of amyloidosis (characterization, which must be performed on frozen tissue). This restatement is proposed to renal pathologists in indicating the traps of studying amyloidosis, the tissues performing for the diagnosis, the specific staining and the immunohistochemical characterization. PMID: 20097147 [PubMed - as supplied by publisher] (Source: Nephrologie & Therapeutique)

Flexible bronchoscopic management of benign tracheal stenosis: long term follow-up of 115 patients

Journal of Cardiothoracic Surgery — Sun, 17 Jan 2010 00:00:00 +0100

Conclusions: BTS in elderly patients with co-morbidities can be safely and effectively treated by flexible bronchoscopic treatment modalities .The use of HDR brachytherapy to treat granulation tissue formation following successful airway restoration is promising . (Source: Journal of Cardiothoracic Surgery)

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Diagnosis and typing of systemic amyloidosis: The role of abdominal fat pad fine needle aspiration biopsy

CytoJournal — Fri, 15 Jan 2010 14:56:49 +0100

Conclusion:</b> FPFNA for SA is not as good as previously reported. This may be due to different practice setting, level of experience, diagnostic technique, or absence of abdominal soft tissue involvement. A negative result of FPFNA does not exclude SA. Immune phenotyping of amyloid is possible on cell block. (Source: CytoJournal)

Insulins: Localised amyloidosis at injection sites: 4 case reports

Reactions — Mon, 11 Jan 2010 14:23:59 +0100

(Source: Reactions)

Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis

British Journal of Haematology — Fri, 08 Jan 2010 00:00:00 +0100

Serum amyloid P component (SAP) is a universal constituent of amyloid deposits and contributes to their formation and/or persistence. We therefore developed CPHPC ((R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexa-noyl]pyrrolidine-2 carboxylic acid), a novel bis(D-proline) drug, to specifically target SAP and report here a first, exploratory, open label proof of principle study in systemic amyloidosis. CPHPC produced sustained, >95% depletion of circulating SAP in all patients and c. 90% reduction in the SAP content of the two amyloidotic organs that became available. There were no significant adverse effects of either SAP depletion or CPHPC itself. No accumulation of amyloid was demonstrable by SAP scintigraphy in any patient on the drug. In hereditary fibrinogen amyloidosis, which is i...

A novel effect of rivastigmine on pre-synaptic proteins and neuronal viability in a neurodegeneration model of fetal rat primary cortical cultures and its implication in Alzheimer's disease

Journal of Neurochemistry — Thu, 07 Jan 2010 00:00:00 +0100

J. Neurochem. (2010) 10.1111/j.1471-4159.2009.06490.x Alzheimer's disease (AD) is characterized by deposition of amyloid-[beta] peptide plaque, disrupted amyloid-[beta]-precursor protein (APP) metabolism, hyperphosphorylation of Tau leading to neurofibrillary tangles and associated neurotoxicity. Moreover, there is synaptic loss in AD, which occurs early and may precede frank amyloidosis. The central cholinergic system is especially vulnerable to the toxic events associated with AD, and reduced acetylcholine levels in specific brain regions is thought to be central to memory deficits in AD. First-generation cholinesterase inhibitors have provided only symptomatic relief to patients with AD by prolonging the action of remaining acetylcholine with little or no change in the course of the dis...

Alanine to Threonine Substitutions and Amyloid Diseases: Butyrylcholinesterase as a Case Study.

Chemico-Biological Interactions — Thu, 07 Jan 2010 00:00:00 +0100

Authors: Podoly E, Hanin G, Soreq H Alanine-to-threonine (A to T) substitutions caused by Single nucleotide Polymorphisms (SNPs) occur in diverse proteins, and in certain cases these substitutions induce self-aggregation into amyloid fibrils or aggregation in other amyloidogenic proteins. This is compatible with the inverse preferences of alanine to form helices and of threonine to support beta-sheet structures, which are crucial for amyloid fibrils formation. Our interest in these mutations was initiated by studying the potential effects of the A539T substitution in the butyrylcholinesterase BChE-K variant on amyloid fibrils formation in Alzheimer's disease. Other examples are, Parkinson's disease (PD), where A53T alpha-synuclein occurs in Lewy bodies and familial amyloid polyneuropat...

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Systemic AL amyloidosis with acquired factor X deficiency: A study of perioperative bleeding risk and treatment outcomes in 60 patients

American Journal of Hematology — Wed, 06 Jan 2010 00:00:00 +0100

Systemic light-chain (AL) amyloidosis may be associated with acquired factor X (FX) deficiency and optimal management of this coagulopathy is unknown. We reviewed our experience with 60 patients with isolated FX deficiency ([le]50%) due to AL amyloidosis that underwent an invasive procedure between 1975 and 2007. They were classified as having severe ( (Source: American Journal of Hematology)

Analytical performance of the serum free light chain assay

Clinical Chemistry and Laboratory Medicine — Tue, 05 Jan 2010 16:21:34 +0100

Conclusions: These data demonstrate that the results of the FLC assay must be interpreted jointly by the clinician and the biologist, taking into account the individual patient's clinical and biological characteristics. Clin Chem Lab Med 2010;48:73–9. (Source: Clinical Chemistry and Laboratory Medicine)

Diagnostic superiority of a combined assessment of the systolic and early diastolic mitral annular velocities by tissue Doppler imaging for the differentiation of restrictive cardiomyopathy from constrictive pericarditis

Clinical Research in Cardiology — Tue, 05 Jan 2010 06:46:09 +0100

Conclusion TDI provides a diagnostic superiority and an accurate discrimination between RCM and CP by using the combined cutoff value of <8 cm/s for S′ and E′ at both sides of the MA. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00392-009-0106-1Authors Thomas Butz, Ruhr-University Bochum Department of Cardiology, Heart Center Northern-Westphalia Bad Oeynhausen GermanyC. Piper, Ruhr-University Bochum Department of Cardiology, Heart Center Northern-Westphalia Bad Oeynhausen GermanyC. Langer, Ruhr-University Bochum Department of Cardiology, Heart Center Northern-Westphalia Bad Oeynhausen GermanyM. Wiemer, Ruhr-University Bochum Department of Cardiology, Heart Center Northern-Westphalia Bad Oeynhausen GermanyT. Kottmann, Ruhr-University Bochu...

Sonozaki syndrome: case report and review of literature

Rheumatology International — Mon, 04 Jan 2010 18:15:53 +0100

Abstract Sonozaki syndrome—pustulotic arthro-osteitis (PAO) is a relatively rare, chronic illness. This disease belongs to the group of psoriatic arthritis (psoriasis arthropatica, artropatia psoriatica) which in turn belongs to the group of seronegative arthritis. Sonozaki syndrome includes palmoplantar pustulosis, PPP as well as arthro-osteitis. Clinically, symmetrically localised pustulae are observed on feet and hands. Effected joints are painful, swollen with a visible inflammation. Here, we describe a case of a woman aged 55 with a diagnosis of Sonozaki syndrome and hyperthyroidism. At the moment of admission multiple changes in the form of pustulae were observed on hands and soles, filled with pus and blood of the erythemal basis. Oral and genital mucosa were free...

Novel Therapy for Transthyretin–related Ocular Amyloidosis: A Pilot Study of Retinal Laser Photocoagulation

Ophthalmology — Mon, 04 Jan 2010 00:00:00 +0100

Conclusions: Panretinal laser photocoagulation is a safe and well-known procedure that offers a new treatment option to mitigate ocular manifestations in patients with transthyretin-related familial amyloidotic polyneuropathy.Financial Disclosure(s): The authors have no proprietary or commercial interest in any of the materials discussed in this article. (Source: Ophthalmology)

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Anti-aging Studies on the Senescence Accelerated Mouse (SAM) Strains.

Yakugaku Zasshi : Journal of the Pharmaceutical Society of Japan — Fri, 01 Jan 2010 00:00:00 +0100

Authors: Takahashi R Senescence accelerated mouse (SAM), a murine model of accelerated senescence, was established by Toshio Takeda and colleagues. SAM consists of series of SAMP (prone) and SAMR (resistant) lines. All SAMP lines (from SAMP1 to SAMP11) are characterized by accelerated accumulation of senile features, earlier onset and faster progress of age-associated pathological phenotypes, such as amyloidosis, impaired immune response, senile osteoporosis and deficits in learning and memory. These SAMP lines are useful for evaluation of putative anti-aging therapies. For example, SAMP1 line is used to study the anti-aging effect of the antioxidant containing foods and various anti-oxidants, such as coenzyme Q10, vitamin C, lycopene. SAMP8 line exhibiting an early onset of impaired l...

Amyloid Heart Disease

Progress in Cardiovascular Diseases — Fri, 01 Jan 2010 00:00:00 +0100

Abstract: The systemic amyloidoses are an uncommon group of disorders characterized by the extracellular deposition of amyloid in one or more organs. Cardiac deposition, leading to an infiltrative/restrictive cardiomyopathy, is a common feature of amyloidosis. It may be the presenting feature of the disease or may be discovered while investigating a patient presenting with non-cardiac amyloidosis. In this article we review the features of cardiac amyloidosis and its varied manifestations. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is stressed in light of recent advances in therapy which can, when appropriately used, significantly improve prognosis. (Source: Progress in Cardiovascular Diseases)

No regression of renal amyloid mass despite remission of nephrotic syndrome in a patient with TRAPS following etanercept therapy.

Journal of Nephrology — Fri, 01 Jan 2010 00:00:00 +0100

Authors: Simsek I, Kaya A, Erdem H, Pay S, Yenicesu M, Dinc A Reactive systemic (AA) amyloidosis leading to renal failure is the most severe complication of tumor necrosis factor receptor-associated periodic syndrome (TRAPS). There is now growing evidence to suggest that anti-tumor necrosis factor (anti-TNF) agents may be an attractive treatment option for amyloidosis not only in TRAPS but in several forms of secondary amyloidosis complicating inflammatory rheumatic diseases. In most of the reported cases, anti-TNF agents were deemed successful on the basis of regression of proteinuria and either improvement or stabilization of creatinine clearance, while objective proof of renal amyloid regression either by serum amyloid P scintigraphy or biopsy is limited. We herein report a case of ...

Left ventricular hypertrophy: Cardiac magnetic resonance may help differentiate amyloidosis from hypertrophic cardiomyopathy.

Archives of Cardiovascular Diseases — Fri, 01 Jan 2010 00:00:00 +0100

Authors: Mabru M, Dacher JN, Bauer F PMID: 20142122 [PubMed - in process] (Source: Archives of Cardiovascular Diseases)

Secondary amyloidosis of the bladder and massive hematuria.

Actas Urologicas Espanolas — Fri, 01 Jan 2010 00:00:00 +0100

CONCLUSIONS: Despite its rarity, as shown by the few cases reported, secondary amyloidosis of the bladder should be considered in patients already diagnosed with systemic amylodosis and/or the conditions reported who require simple urethral catheterization. PMID: 20223142 [PubMed - as supplied by publisher] (Source: Actas Urologicas Espanolas)

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Systemic-onset juvenile idiopathic arthritis complicated by early onset amyloidosis in a patient carrying a mutation in the MEFV gene

Rheumatology International — Thu, 31 Dec 2009 16:44:51 +0100

Abstract Systemic juvenile idiopathic arthritis (SJIA) is a disorder characterized by arthritis in children starting before 16 years of age associated with daily high fever, persisting for more than 2 weeks, and at least one of the following clinical features: evanescent cutaneous rash, lymphadenopathy, serositis or hepatosplenomegaly. SJIA patients carry a significantly higher frequency of MEFV mutations, the gene responsible for familial Mediterranean fever, and may be characterized by a more aggressive disease. In this line, we describe a 9-year-old girl affected with SJIA who carried a heterozygous G196W mutation in MEFV. Our patient was characterized by an aggressive disease course, resistance to conventional immunosuppressive agents and developed renal amylo...

Original paper Secondary (AA) amyloidosis in systemic onset juvenile idiopathic arthritis - a long-term follow-up study

Articles of Rheumatology - TERMEDIA publishing house — Tue, 29 Dec 2009 07:32:56 +0100

The aim of this study was to determine the clinical outcome and survival of children with long-standing systemic onset juvenile idiopathic arthritis (SOJIA) and amyloidosis. 27 patients (11 male, 16 female) with SOJIA and amyloidosis had an average SOJIA duration of 17.3 yrs. The past and current status were assessed from hospital records, through a questionnaire and a personal interview of all living patients. Amyloidosis was diagnosed (histopathological methods) 1.7-20 yrs after the onset of SOJIA (mean 5.3 ±4.6 yrs). The mean follow-up from the diagnosis of amyloidosis was 11.1 ±7.0 yrs. 24 of 27 patients are currently alive (3 of 27 (11.1%) died). Infection was the cause of death in 2 patients with renal failure. One patient had undergone successful renal transplantation, a...

Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome

Arthritis and Rheumatism — Mon, 28 Dec 2009 00:00:00 +0100

This study was undertaken to assess the long-term efficacy and safety of anakinra treatment in patients with NOMID/CINCA syndrome.We retrospectively analyzed the medical records of NOMID/CINCA syndrome patients referred to 2 centers, who had started anakinra treatment before June 2007.There were 10 patients with NOMID/CINCA syndrome who had been treated with anakinra. The patients' ages at the time anakinra treatment was initiated ranged from 3 months to 20 years. They had been followed up for 26-42 months. Sustained efficacy in the treatment of systemic inflammation and, in some cases, neurologic involvement and growth parameters, was achieved. The dosage of anakinra required for efficacy ranged from 1 to 3 mg/kg/day in the 8 oldest patients and from 6 to 10 mg/kg/day in the 2 youngest. R...

Clinical symptomatic de novo systemic transthyretin amyloidosis 9 years after domino liver transplantation

Liver Transplantation — Wed, 23 Dec 2009 00:00:00 +0100

No abstract. (Source: Liver Transplantation)

In vivo labelling of hippocampal β-amyloid in triple-transgenic mice with a fluorescent acetylcholinesterase inhibitor released from nanoparticles

European Journal of Neuroscience — Mon, 21 Dec 2009 00:00:00 +0100

In conclusion, nanoparticles appear as versatile carriers of AChE inhibitors and other promising drugs for the treatment of AD. (Source: European Journal of Neuroscience)

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Apolipoprotein E protects cultured pericytes and astrocytes from D-Abeta(1-40)-mediated cell death.

Brain Research — Mon, 21 Dec 2009 00:00:00 +0100

In this study we compared the apoE production capacity, the effects of apoE on D-Abeta(1-40) internalization, D-Abeta(1-40) cell surface accumulation and the vulnerability for D-Abeta(1-40)-induced toxicity of either cell type in order to quantify the relative contributions of astrocytes and pericytes in the various processes that contribute to CAA formation. Strikingly, cultured astrocytes produced only 3-10% of the apoE amounts produced by pericytes. Furthermore, pericytes with the apoE epsilon4 allele produced three times less apoE and were more vulnerable to D-Abeta(1-40) treatment than pericytes without an epsilon4 allele. Such relations were not observed with astrocytes in vitro. Both pericytes and astrocytes, however, were protected from Abeta-induced cytotoxicity by high levels of ...

An unusual case of transient dermatological reaction to bortezomib in AL amyloidosis.

International Journal of Hematology — Fri, 18 Dec 2009 00:00:00 +0100

We report an unusual dermatological reaction to bortezomib in a 61-year-old man with AL amyloidosis. Systemic AL amyloidosis is a rare complication of monoclonal gammopathy or myeloma in which abnormally unstable free light chains cause fibrillary deposits in organs leading to multisystem disease. The treatment of AL amyloidosis is directed at the underlying plasma cell dyscrasia and most regimes have been adapted from myeloma, but drug toxicity is more common in AL amyloidosis because of the more extensive nature of the disease. We report a patient who developed asymptomatic purple discolouration of the veins of his left arm several days after receiving the infusion in his left hand, although the infusion itself had been uncomplicated with no extravasation. The discolouration resolved com...

Pulmonary amyloidosis mimicking multiple metastatic lesions on F-18 FDG PET/CT

Lung Cancer — Fri, 18 Dec 2009 00:00:00 +0100

We report a case of amyloid pulmonary nodules with positive FDG uptake that mimic multiple lung metastases. A 54-year-old female patient was referred for the evaluation of multiple lung nodules. A PET/CT scan revealed mild FDG uptake in various sized pulmonary nodules. Resected nodules contained amorphous eosinophilic proteineous material with focal calcification, consistent with amyloidosis. Pulmonary amyloidosis should be added to the differential diagnosis for cases of multiple pulmonary nodules that show positive FDG uptake. (Source: Lung Cancer)

In vivo detection of prion amyloid plaques using [11C]BF-227 PET

European Journal of Nuclear Medicine and Molecular Imaging — Thu, 17 Dec 2009 07:02:34 +0100

Conclusion Although [11C]BF-227 is a non-specific imaging marker of cerebral amyloidosis, it is useful for in vivo detection of PrP plaques in the human brain in GSS, based on the regional distribution of the tracer. PET amyloid imaging might provide a means for both early diagnosis and non-invasive disease monitoring of certain forms of TSEs. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00259-009-1314-7Authors Nobuyuki Okamura, Tohoku University School of Medicine Department of Pharmacology Sendai JapanYusei Shiga, Tohoku University School of Medicine Department of Neurology Sendai JapanShozo Furumoto, Tohoku University School of Medicine Department of Pharmacology Sendai JapanManabu Tashiro, Tohoku University Division of Cyclotron Nuclear Medici...

Delayed Hyper-Enhancement Magnetic Resonance Imaging Provides Incremental Diagnostic and Prognostic Utility in Suspected Cardiac Amyloidosis

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 14 Dec 2009 22:01:29 +0100

Conclusions A characteristic DHE-CMR pattern is more accurate for diagnosis and is a stronger predictor of 1-year mortality in patients with suspected CA as compared with other noninvasive parameters. (Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

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Magnetic Resonance Imaging in Cardiac Amyloidosis

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 14 Dec 2009 22:01:29 +0100

(Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

Cognitive Decline and Brain Volume Loss as Signatures of Cerebral Amyloid-{beta} Peptide Deposition Identified With Pittsburgh Compound B: Cognitive Decline Associated With A{beta} Deposition [Original Contribution]

Archives of Neurology — Mon, 14 Dec 2009 20:51:43 +0100

Conclusion The in vivo measure of cerebral amyloidosis known as [11C]PiB is associated with cross-sectional regionally specific brain atrophy and longitudinal cognitive decline in multiple cognitive domains that occur before the clinical diagnosis of Alzheimer disease. These findings contribute to the understanding of the cognitive and structural consequences of Aβ levels in cognitively healthy older adults. (Source: Archives of Neurology)

[Personal View] Hypothetical model of dynamic biomarkers of the Alzheimer's pathological cascade

Lancet Neurology — Mon, 14 Dec 2009 00:00:00 +0100

Currently available evidence strongly supports the position that the initiating event in Alzheimer's disease (AD) is related to abnormal processing of β-amyloid (Aβ) peptide, ultimately leading to formation of Aβ plaques in the brain. This process occurs while individuals are still cognitively normal. Biomarkers of brain β-amyloidosis are reductions in CSF Aβ42 and increased amyloid PET tracer retention. After a lag period, which varies from patient to patient, neuronal dysfunction and neurodegeneration become the dominant pathological processes. Biomarkers of neuronal injury and neurodegeneration are increased CSF tau and structural MRI measures of cerebral atrophy. Neurodegeneration is accompanied by synaptic dysfunction, which is indicated by decreased fluorodeoxyglucose uptake on ...

Fractal analysis of amyloid plaques in Alzheimer's disease patients and mouse models.

Neurobiology of Aging — Mon, 14 Dec 2009 00:00:00 +0100

Authors: Pirici D, Van Cauwenberghe C, Van Broeckhoven C, Kumar-Singh S The varied morphological and biochemical forms in which amyloid deposits in brain of Alzheimer's disease (AD) patients are complex and their mechanisms of formation are not completely understood. Here we investigated the ability of fractal dimension (FD) to differentiate between the textures of commonly observed amyloid plaques in sporadic and familial AD patients and aged-control individuals as well as in transgenic mouse models of amyloidosis. Studying more than 6000 amyloid plaques immunostained for total Abeta (Abetat), Abeta40 or Abeta42, we show here that Abeta40 FD could efficiently differentiate between (i) AD patients and aged-control individuals (P<0.001); (ii) sporadic and familial AD due to presenili...

Heart transplantation in primary amyloidosis

Revista Brasileira de Cirurgia Cardiovascular — Sat, 12 Dec 2009 16:02:07 +0100

A amiloidose cardíaca é doença altamente limitante da sobrevida, por morte súbita na maioria dos pacientes. Pela agressão contra outros órgãos, particularmente rins e sistema nervoso central, o transplante cardíaco tem sido opção questionável, face à escassez de órgãos. O objetivo é relatar a evolução, com sobrevivência de 7 anos, da paciente após transplante cardíaco por amiloidose, em boas condições. Um ano após o transplante cardíaco, houve indicação de transplante renal, também pela agressão da doença. Esta paciente contrasta com outros três pacientes de nosso serviço que foram a óbito, ainda na fase de avaliação. Apesar de sua natureza multisistêmica, a amiloidose cardíaca pode, em pacientes selecionados, justificar o transplante cardíaco, pela gra...

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Short and long-term outcome of treatment with high-dose melphalan and stem cell transplantation for multiple myeloma-associated AL amyloidosis

Annals of Hematology — Thu, 10 Dec 2009 15:15:45 +0100

In conclusion, HDM/SCT can prolong overall survival in patients with MM/AL who are eligible to receive it. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0874-8Authors Saulius Girnius, Boston University School of Medicine Amyloid Treatment and Research Program Boston MA USADavid C. Seldin, Stem Cell Transplantation Program of the Section of Hematology-Oncology FGH 1007, 820 Harrison Avenue Boston MA 02118 USAMartha Skinner, Boston University School of Medicine Amyloid Treatment and Research Program Boston MA USAKathleen T. Finn, Stem Cell Transplantation Program of the Section of Hematology-Oncology FGH 1007, 820 Harrison Avenue Boston MA 02118 USAKaren Quillen, Stem Cell Transplantation Program of the Section of Hematology-Oncology FGH 1007, 820 Harrison...

Acceleration of brain amyloidosis in an Alzheimer's disease mouse model by a folate, vitamin B6 and B12-deficient diet.

Experimental Gerontology — Wed, 09 Dec 2009 00:00:00 +0100

Authors: Zhuo JM, Praticò D Epidemiological and clinical studies indicate that elevated circulating level of homocysteine (Hcy) is a risk factor for developing Alzheimer's disease (AD). Dietary deficiency of folate, vitamin B6 and B12 results in a significant increase of Hcy levels, a condition also known as hyperhomocysteinemia (HHcy). In the present study we tested the hypothesis that a diet deficient for these three important factors when administered to a mouse model of AD, i.e. Tg2576, will result in HHcy and in an acceleration of their amylodotic phenotype. Compared with Tg2576 mice on regular chow, the ones receiving the diet-deficient for folate, B6 and B12 developed HHcy. This condition was associated with a significant increase in Abeta levels in the cortex and hippocamp...

Oto-Rhino-Laryngology (ORL) tumor presentation in a case of systemic AA amyloidosis.

Dermatol Online J — Thu, 03 Dec 2009 20:46:04 +0100

Authors: Khoudri I, Marcil T, Elmeknassi I, Kzadri M, Ismaili N, Afifi Y, Senouci K, Hassam B A 64-year-old male with no underlying disease presented with the development of multiple skin nodules, loss of sensation in the extremities, hoarseness, macroglossia, and pain in the oral cavity. Direct laryngoscopy showed nodules involving the oral cavity, oropharynx, supraglottic region, and vocal cords. Biopsy from skin nodules showed amyloid deposits staining with Congo red. Immunohistochemical staining was used for AA protein and was positive. Biopsy from the oral floor was also positive for amyloid. Oto-Rhino-Laryngology (ORL) involvement has been reported in approximately 40 percent of AL amyloidosis patients, but does not appear to be frequent in AA amyloidosis. Cutaneous manifestation...

Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens

Blood — Thu, 03 Dec 2009 17:02:29 +0100

In this study, we report the development of a highly specific and sensitive novel test for the typing of amyloidosis in routine clinical biopsy specimens. Our approach combines specific sampling by laser microdissection (LMD) and analytical power of tandem mass spectrometry (MS)–based proteomic analysis. We studied 50 cases of amyloidosis that were well-characterized by gold standard clinicopathologic criteria (training set) and an independent validation set comprising 41 cases of cardiac amyloidosis. By use of LMD/MS, we identified the amyloid type with 100% specificity and sensitivity in the training set and with 98% in validation set. Use of the LMD/MS method will enhance our ability to type amyloidosis accurately in clinical biopsy specimens. (Source: Blood)

Molecular Pathogenesis of Protein Misfolding Diseases: Pathological Molecular Environments Versus Quality Control Systems Against Misfolded Proteins

Journal of Biochemistry — Tue, 01 Dec 2009 15:10:06 +0100

Diverse human diseases, including various neurodegenerative disorders and amyloidoses, are thought to result from the misfolding and aggregation of disease-causative proteins, and thus are collectively called protein misfolding diseases. Natively folded disease-causative proteins generally undergo a β-sheet conformational transition through an energetically unfavourable process, and further polymerize into amyloid fibrils. In the case of β2-microglobulin-related amyloidosis, an extracellular protein misfolding disease, many kinds of biological molecules including glycosaminoglycans, proteoglycans and lipids partially unfold β2-microglobulin and catalyse its subsequent nucleus formation. After amyloid fibrils are formed, these biological molecules stabilize the β2-microg...

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Cerebral Amyloidosis: Postmortem Detection with Human 7.0-T MR Imaging System [Neuroradiology]

Radiology — Tue, 01 Dec 2009 10:43:17 +0100

The results of our study show that abnormalities can be detected in postmortem human brain specimens with cerebral amyloidosis by using a human MR imaging (7.0-T) system; these features primarily include the presence of hypointense foci. (Source: Radiology)

Arterial Compliance And Carotid Atherosclerosis In Apolipoprotein A-I Amyloidosis (Leu75pro)

Artery Research — Tue, 01 Dec 2009 00:00:00 +0100

Background: Hereditary amyloidosis are late-onset autosomal dominant disorders characterized by amyloid deposition in various tissues. Among them, Apolipoprotein A-I amyloidosis (Leu75Pro) is a rare autosomal dominant condition in which renal, hepatic, and testicular involvement has been demonstrated.No data are available about vascular alterations in this condition.Aim of the study was to evaluate arterial stiffness,assessed by pulse wave velocity (PWV)and carotid artery intima-media thickness (IMT),evaluated by ultrasound, in patients with Apolipoprotein A-I amyloidosis (APO AI). Patients: In 104 patients with APO AI(mean age 52±16 years,56 F) and in 104 subjects matched for age, sex, body mass index (BMI) and blood pressure (BP), PWV and IMT were measured. Results: By definition no dif...

Value of phase-sensitive inversion recovery sequence to perform and analyse late gadolinium enhancement in cardiac amyloidosis.

Archives of Cardiovascular Diseases — Tue, 01 Dec 2009 00:00:00 +0100

Authors: Gerbaud E, Lederlin M, Laurent F PMID: 19963197 [PubMed - in process] (Source: Archives of Cardiovascular Diseases)

Effective therapy with tranexamic acid in a case of chronic disseminated intravascular coagulation with acquired alpha2-antiplasmin deficiency associated with AL amyloidosis.

Thrombosis and Haemostasis — Tue, 01 Dec 2009 00:00:00 +0100

Authors: Colucci G, Alberio L, Jahns M, Keller P, Steiner S, Rüsges-Wolter I, Lämmle B PMID: 19967165 [PubMed - in process] (Source: Thrombosis and Haemostasis)

Junior Researcher Travel Awards for XIIth International Symposium on Amyloidosis

ScanGrants feed — Mon, 30 Nov 2009 00:00:00 +0100

April 18 – 21, 2010 Rome, Italy Description: This award is intended to assist young investigators with defraying expenses for travel to this symposium. The award can be used to cover costs for symposium registration, travel, lodging and meals. The foundation will issue awards up to $2000. Eligibility: Pre or postdoctoral trainees, fellows or residents that are authors on an abstract submitted for presentation are eligible. Preference will be given to researchers who are the presenting (first) author. Only abstracts accepted by the symposium committee will be reviewed for this award. Application Instructions: Applications are to be made electronically and are due no later than January 10, 2010. Amyloidosis Foundation, Inc. 7151 N. Main St. Suite 2 Clarkston, MI 48346 (Source...

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Pulmonary amyloidosis in Sjögren's syndrome: a rare diagnosis for nodular lung lesions

APLAR Journal of Rheumatology — Mon, 23 Nov 2009 00:00:00 +0100

Lung involvement in Sjögren's syndrome (SS) can affect trachea, bronchus, small airways, pleura and may cause interstitial lung injury. It may also be associated with malignancies, particularly non-Hodgkin's lymphoma, which is a well-recognized complication of this disease. Here we describe the occurrence of localized amyloidosis presenting as pulmonary nodules in a patient with newly diagnosed SS. We highlight this rare occurrence as a diagnostic possibility that should be considered in the evaluation of pulmonary involvement in this disease. (Source: APLAR Journal of Rheumatology)

Enfuvirtide: Amyloidosis: case report

Reactions — Sun, 22 Nov 2009 14:04:51 +0100

(Source: Reactions)

White matter disorders with autosomal dominant heredity: a review with personal clinical case studies and their MRI findings

Acta Neurologica Scandinavica — Thu, 19 Nov 2009 00:00:00 +0100

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)

Amyloidosis and Neurodegenerative Diseases: Current Treatments and New Pharmacological Options

Karger Publishers — Tue, 17 Nov 2009 23:00:00 +0100

Pharmacology 2010;85:1-17 (DOI:10.1159/000259044) (Source: Karger Publishers)

Microalbuminuria as an early marker of renal involvement in Behcet's disease: it is associated with neurological involvement and duration of the disease

Journal of the European Academy of Dermatology and Venereology — Tue, 17 Nov 2009 00:00:00 +0100

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 total of 34 (16.1%) patients were found to have renal involvement including 22 (11.1%) with MA and 12 with proteinuria (5.6%). Renal biopsies resulted as focal glomeruloscler...

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Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP

Acta Neuropathologica — Thu, 12 Nov 2009 18:38:27 +0100

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-Scheinker disease phenotype with numerous cerebral multicentric amyloid plaques and severe neurofibrillary lesions without PrP-CAA. Western blot analysis in the patient with...

Molecular basis for insulin fibril assembly [Biophysics_And_Computational_Biology]

Proceedings of the National Academy of Sciences — Tue, 10 Nov 2009 19:27:51 +0100

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)

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.

Haematologica — Tue, 10 Nov 2009 00:00:00 +0100

Authors: Brunvand MW, Bitter M PMID: 19903681 [PubMed - as supplied by publisher] (Source: Haematologica)

Cerebral amyloidosis: amyloid subunits, mutants and phenotypes.

Cellular and Molecular Life Sciences : CMLS — Sat, 07 Nov 2009 00:00:00 +0100

Authors: Rostagno A, Holton JL, Lashley T, Revesz T, Ghiso J 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 amyloid in the mechanism of disease pathogenesis and to dissect the link between vascular and parenchymal amyloid d...

The “other” vasculitis syndromes and kidney involvement

Pediatric Nephrology — Fri, 06 Nov 2009 18:58:23 +0100

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. However, vasculitis may be associated with a number of rheumatic diseases in childhood and the auto-inflammatory syndromes (periodic fever syndromes). Auto-inflammatory syn...

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Amyloid in bone marrow smears of patients affected by multiple myeloma

Annals of Hematology — Thu, 05 Nov 2009 18:57:20 +0100

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-green birefringence. In particular, 51 of the 126 patients studied at diagnosis and 16 of the 40 patients with advanced disease were positive. Seventeen patients were r...

Restrictive cardiomyopathies

European Journal of Echocardiography — Wed, 04 Nov 2009 15:08:56 +0100

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 treatment. The much more rare diagnosis of idiopathic restrictive cardiomyopathy is supported only by the absence of specific pathology on either endomyocardial biopsies or at po...

Tissue Doppler imaging and contrast-enhanced cardiac magnetic resonance in primary cardiac amyloidosis

European Journal of Echocardiography — Wed, 04 Nov 2009 15:08:55 +0100

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)

Chapter 6: The chaperone action of Clusterin and its putative role in quality control of extracellular protein folding.

Advances in Cancer Research — Wed, 04 Nov 2009 02:42:03 +0100

Authors: Wyatt A, Yerbury J, Poon S, Dabbs R, Wilson M 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 pathology of a variety of serious human diseases is thought to arise as a consequence of the inappropriate aggregation of s...

Cardiac transplantation should be considered in selected patients with either AL or hereditary forms of amyloidosis: the UK National Amyloidosis Centre experience

Internal Medicine Journal — Wed, 04 Nov 2009 00:00:00 +0100

(Source: Internal Medicine Journal)

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Heart rate variability in familial Mediterranean fever

Rheumatology International — Mon, 02 Nov 2009 18:03:54 +0100

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 of Medicine Tel Aviv IsraelPnina Langevitz, Sheba Medical Center, The Heller Institute of Medical Research Department of Medicine F Tel Hashomer IsraelOlga Feld, Sheba M...

Alnylam Presents New Pre-Clinical Data on ALN-TTR, an RNAi Therapeutic for the Treatment of Transthyretin-Mediated Amyloidosis

Drugs.com - Clinical Trials — Mon, 02 Nov 2009 12:33:44 +0100

– 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)

Left Ventricular Ejection Time on Echocardiography Predicts Long-Term Mortality in Light Chain Amyloidosis

Journal of the American Society of Echocardiography — Mon, 02 Nov 2009 00:00:00 +0100

Conclusion: AL amyloidosis was associated with high long-term mortality. Among echocardiographic and clinical parameters, only ET and alkaline phosphatase had incremental value to HF class in predicting mortality. This may be useful to identify high-risk patients. (Source: Journal of the American Society of Echocardiography)

Absence of Heart Failure in Severe Cardiac and Autonomic Amyloidosis: The Essential Role of Sympathetic Activation and Venous Tone in the Development of the Congestive Heart Failure Syndrome.

Congestive Heart Failure — Sun, 01 Nov 2009 00:00:00 +0100

Authors: Abramov D, Weimer LH, Marboe CC, Shimbo D, King DL, Maurer MS PMID: 19925508 [PubMed - as supplied by publisher] (Source: Congestive Heart Failure)

Pattern of pediatric renal disease observed in native renal biopsies in Pakistan.

Journal of Nephrology — Sun, 01 Nov 2009 00:00:00 +0100

Conclusions: Our pattern of biopsied pediatric renal pathology is similar to that reported recently in series with similar biopsy indications from other parts of the world. PMID: 19967653 [PubMed - in process] (Source: Journal of Nephrology)

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[Effectiveness of dietetic treatment in nephrotic syndrome.]

Nutricion Hospitalaria — Sun, 01 Nov 2009 00:00:00 +0100

We present the case of a male patient suffering from a primary amyloidosis and a nephrotic syndrome who came to dietotherapy clinic. In the first visit we made a nutritional record including an anthropometric evaluation, body composition, biochemistry, and food intake. The patient had an excess of body water, proteinuria, low plasma protein, albumin, prealbumin and HDL cholesterol levels, and high concentrations of total cholesterol, LDL and triglycerides. The consumption of protein and sodium was higher than the recommendation. An individualized diet was made. Six months later, his weight and the excess of body water decreased, but the fat free mass remained unchanged. The levels of albumin and prealbumin increased, the proteinuria decreased. Total cholesterol, LDL and triglycerides decre...

Unusual combination of tracheobronchopathia osteochondroplastica and AA amyloidosis.

Yonsei Medical Journal — Sat, 31 Oct 2009 00:00:00 +0100

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 illustrates that it is possible for TO and amyloidosis to masquerade as asthma. TO and amyloidosis should be suspected in patients of older ages with asthma and especial...

Coexistence of primary AL amyloidosis and POEMS syndrome: Efficacy of melphalan-dexamethasone and role of biochemical markers in monitoring the diseases course

American Journal of Hematology — Thu, 29 Oct 2009 00:00:00 +0100

No abstract. (Source: American Journal of Hematology)

Binding of epigallocatechin-3-gallate to transthyretin modulates its amyloidogenicity

FEBS Letters — Mon, 26 Oct 2009 00:00:00 +0100

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 comigration in non-denaturing gel electrophoresis (MI:0404) (Source: FEBS Letters)

Pseudomembranous Aspergillus tracheobronchitis in a heart transplant recipient

Transplant Infectious Disease — Wed, 21 Oct 2009 23:00:00 +0100

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 aware of AT as a potential cause of respiratory symptoms with few radiographic abnormalities in HT recipients, as delay in performing bronchoscopy could result in an unf...

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Amyloidosis of lacrimal gland

Indian Journal of Ophthalmology — Wed, 21 Oct 2009 13:59:29 +0100

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)

Dietary composition modulates brain mass and amyloid beta levels in a mouse model of aggressive Alzheimer's amyloid pathology

Molecular Neurodegeneration — Tue, 20 Oct 2009 23:00:00 +0100

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 fat/low carbohydrate custom chow (60 kcal% fat/ 30 kcal% protein/ 10 kcal% carbohydrate); (3) high protein/low carbohydrate custom chow (60 kcal% protein/ 30 kcal% fat/ 10 ...

Amyloidosis presenting as postcricoid esophageal stricture

Gastrointestinal Endoscopy — Mon, 19 Oct 2009 00:00:00 +0100

A 44-year-old woman presented with 3 months of progressive dysphagia to both solids and liquids with significant loss of weight. A peripheral blood smear showed a dimorphic (normocytic normochromic and microcytic hypochromic) anemia. Laboratory data included a hemoglobin level of 9.0 g/dL, hematocrit of 34%, and normal renal and liver biochemical test results except for a serum albumin level of 1.7 g/dL. EGD showed diffuse nodularity in the hypopharyngeal and postcricoid region with stricture (A). The vocal cord movements were normal. She underwent esophageal bougienage. Postdilation endoscopy showed similar nodular lesions in the upper esophagus, antrum of stomach, and the second part of the duodenum. Biopsy specimens from all these affected areas of the upper GI tract were taken. Histolo...

IgA nephropathy associated with ankylosing spondylitis is not controlled by infliximab therapy

Nephrology Dialysis Transplantation — Thu, 15 Oct 2009 08:26:24 +0100

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)

Ultrasonography of Bovine Urinary Tract Disorders

Veterinary Clinics of North America: Food Animal Practice — Mon, 12 Oct 2009 15:02:09 +0100

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)

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Glucocorticoids/prednisolone: Masking of dialysis-related amyloidosis: case report

Reactions — Mon, 12 Oct 2009 13:02:30 +0100

(Source: Reactions)

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 etanercept

Rheumatology International — Sat, 10 Oct 2009 07:02:33 +0100

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 within only 4 months. This is the first case report of RA-related systemic reactive AA amyloidosis histopathologically demonstrating rapid regression of amyloid depos...

How I treat amyloidosis

Blood — Thu, 08 Oct 2009 16:03:04 +0100

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. Typing is usually based on clinical and laboratory findings with monoclonal gammopathy evaluation and, if indicated, TTR gene testing. Direct tissue typing is required whe...

Persistent Troponin Elevation in a Patient with Cardiac Amyloidosis

Clinical Cardiology — Wed, 07 Oct 2009 23:00:00 +0100

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 persistent troponin elevation in a patient with tranthyretin amyloidosis. Very few other cases have been published on the topic of cardiac amyloidosis and troponin elevati...

Essential role of tau phosphorylation in adult hippocampal neurogenesis

Hippocampus — Wed, 07 Oct 2009 23:00:00 +0100

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 non-p-tau in adult rat brain and human patients with AD, and the p-tau was coexpressed temporally and spatially with DCX and neuroD in the hippocampal dentate gyrus (DG) of...

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Localized amyloidosis at the site of enfuvirtide injection.

Annals of Internal Medicine — Mon, 05 Oct 2009 23:00:00 +0100

Authors: Morilla ME, Kocher J, Harmaty M PMID: 19805777 [PubMed - in process] (Source: Annals of Internal Medicine)

A novel Y331X nonsense mutation in TNFRSF1A gene in two unrelated Turkish families with periodic fever syndrome

International Journal of Immunogenetics — Sun, 04 Oct 2009 23:00:00 +0100

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)

Conformational diseases: looking into the eyes.

Brain Research Bulletin — Fri, 02 Oct 2009 23:00:00 +0100

Authors: Surguchev A, Surguchov A 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 protein fragments and formation of insoluble fibrils. Amyloidogenic protein accumulation often occurs in the brain tissues, e.g. in Alzh...

Vitreous Amyloidosis in Two Sisters as the Indication of Transthyretin-Related Familial Form of Systemic Amyloidosis Among Liver Transplantation Candidates

Transplantation Proceedings — Thu, 01 Oct 2009 00:00:00 +0100

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)

Multiple bony amyloidomas as an initial presentation of myeloma.

Clinical Lymphoma and Myeloma — Thu, 01 Oct 2009 00:00:00 +0100

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)

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High-Dose Chemotherapy with Autotransplantation in AL Amyloidosis: A Flawed Meta-analysis

Biology of Blood and Marrow Transplantation — Thu, 01 Oct 2009 00:00:00 +0100

The conclusions of the meta-analysis by Mhaskar et al. evaluating high-dose chemotherapy with autotransplantation in immunoglobulin light chain amyloidosis are compromised by the omission of a key publication and flawed analytical techniques. The meta-analysis included 1 prospective randomized study of 100 patients and 2 nonrandomized studies containing a total of 49 patients. The pooled results of these 3 studies showed superior overall survival (OS) with conventional chemotherapy (CC) compared to autologous hematopoietic stem cell transplantation (AHSCT) (hazard ratio 1.79; P=.018). (Source: Biology of Blood and Marrow Transplantation)

Effective Anti-TNF-{alpha} Therapy Can Induce Rapid Resolution and Sustained Decrease of Gastroduodenal Mucosal Amyloid Deposits in Reactive Amyloidosis Associated with Rheumatoid Arthritis.

J Rheumatol — Wed, 30 Sep 2009 23:00:00 +0100

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)

Renal AA amyloidosis secondary to morbid obesity?

Clinical Nephrology — Wed, 30 Sep 2009 23:00:00 +0100

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)

Prevention of Amyloidosis in Familial Mediterranean Fever with Colchicine: A Case-Control Study in Armenia

Medical Principles and Practice : Last 20 articles — Tue, 29 Sep 2009 23:00:00 +0100

Med Princ Pract 2009;18:441-446 (DOI:10.1159/000235892) (Source: Medical Principles and Practice : Last 20 articles)

Aggregates of denatured proteins stimulate nitric oxide and superoxide production in macrophages

Inflammation Research — Tue, 29 Sep 2009 01:07:20 +0100

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 Medicine Department of Immunology Czysta 18 St. 31-121 Cracow Poland Journal Inflammation ResearchOnline ISSN 1420-908XPrint ISSN 1023-3830 (Source: Inflammation Resear...

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Susceptibility to AA amyloidosis in rheumatic diseases: A critical overview

Arthritis Care and Research — Mon, 28 Sep 2009 23:00:00 +0100

No abstract. (Source: Arthritis Care and Research)

Staged heart transplantation and chemotherapy as a treatment option in patients with severe cardiac light-chain amyloidosis

European Journal of Heart Failure — Mon, 28 Sep 2009 23:00:00 +0100

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)

Cerebral Amyloidosis: Postmortem Detection with Human 7.0-T MR Imaging System [Neuroradiology]

Continuous Publishing articles — Mon, 28 Sep 2009 23:00:00 +0100

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)

Cerebral Amyloidosis: Postmortem Detection with Human 7.0-T MR Imaging System.

Radiology — Mon, 28 Sep 2009 23:00:00 +0100

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)

Response: Re: High-Dose Chemotherapy with Autotransplantation in AL Amyloidosis: A Flawed Meta-analysis

Biology of Blood and Marrow Transplantation — Mon, 28 Sep 2009 00:00:00 +0100

The primary claim put forward by Dr. Mehta et al. is that there is “reliable” evidence that autologous stem cell transplantation is superior to conventional chemotherapy. Any reasonable observer would conclude with the main findings of our systematic review that there is a paucity of reliable data on the efficacy of autologous stem cell transplantation in AL amyloidosis, and this is precisely the conclusion of our manuscript . (Source: Biology of Blood and Marrow Transplantation)

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Spontaneous gallbladder pathology in baboons

Journal of Medical Primatology — Sun, 27 Sep 2009 23:00:00 +0100

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)

Spontaneous pathology of the baboon endocrine system

Journal of Medical Primatology — Sun, 27 Sep 2009 23:00:00 +0100

Conclusions Endocrine disease in baboons is common and shares clinical and biochemical characteristics with endocrine disease in humans. (Source: Journal of Medical Primatology)

Quantitative assessment of myocardial T2 relaxation times in cardiac amyloidosis

Journal of Magnetic Resonance Imaging — Fri, 25 Sep 2009 23:00:00 +0100

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 sequence. Slice averaged T2 relaxation times were subsequently calculated offline and compared to the previously established normal range.Twelve of the 49 patients were con...

K3 Fragment of Amyloidogenic β2-Microglobulin Forms Ion Channels: Implication for Dialysis Related Amyloidosis

Journal of the American Chemical Society — Fri, 25 Sep 2009 16:34:01 +0100

Journal of the American Chemical Society, Volume 0, Issue 0, Articles ASAP (As Soon As Publishable). (Source: Journal of the American Chemical Society)

Cardiac amyloidosis in African Americans: Comparison of clinical and laboratory features of transthyretin V122I amyloidosis and immunoglobulin light chain amyloidosis

American Heart Journal — Thu, 24 Sep 2009 16:17:13 +0100

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)

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MEFV mutations in Egyptian patients suffering from familial Mediterranean fever: analysis of 12 gene mutations

Rheumatology International — Thu, 24 Sep 2009 14:34:15 +0100

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 frequent gene mutations in the studied group were V726A, M694V, M680I, E148Q and M694I in 41.2, 32.4, 29.4, 25 and 20.6%, respectively. At least one of these main five f...

Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behçet’s disease

Clinical Rheumatology — Wed, 23 Sep 2009 06:03:06 +0100

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 normal renal functions, normal serum proteins, and normal acute-phase reactants. However, recently, after 18 months of anakinra treatment, her proteinuria gradually...

[Solitary pulmonary nodule.]

Praxis — Tue, 22 Sep 2009 23:00:00 +0100

Authors: Schaeffler F, Kos S 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)

Amyloidosis: an unusual case of persistent oral ulceration

Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics — Tue, 22 Sep 2009 23:00:00 +0100

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 this condition, and how it can present a challenge to clinicians in terms of diagnosis and treatment. (Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiolog...

Frequencies and Types of Arrhythmias in Patients With Systemic Light-Chain Amyloidosis With Cardiac Involvement Undergoing Stem Cell Transplantation on Telemetry Monitoring

The American Journal of Cardiology — Tue, 22 Sep 2009 16:18:50 +0100

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 tachycardia/ventricular fibrillation [VT/VF]) and baseline clinical, laboratory, and echocardiographic data were determined. Atrial and ventricular arrhythmias were found in...

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Journal Scan: Systemic Cardiac Amyloidoses: Disease Profiles and Clinical Courses of the 3 Main Types (Circulation 2009;Sep 14:[Epub ahead of print].)

Cardiosource — Tue, 22 Sep 2009 12:17:51 +0100

The investigators conducted a longitudinal study of 233 patients with cardiac amyloidosis (AL, n = 157; ATTRm, n = 61; ATTRwt, n = 15) at two large Italian centers providing coordinated amyloidosis diagnosis/management facilities since 1990. (Source: Cardiosource)

Fibril protein fragmentation pattern in systemic AL-amyloidosis

The Journal of Pathology — Sun, 20 Sep 2009 23:00:00 +0100

Immunoglobulin light chain (AL)-amyloidosis was one of the first types of amyloidosis discovered and still little is known about its pathogenic mechanisms. One major obstacle is the very heterogeneous condition; in fact, every patient could be considered to have their own disease since symptoms and outcome vary enormously. The reason for this is not known but intrinsic factors of the immunoglobulin light chain (LC) and the fact that every LC is unique seem to be important. Post-translational modifications such as glycosylation and proteolysis are most certainly involved. By using western blotting, we studied in detail the proteolytic pattern in six patients with AL-amyloidosis of kappa type with the aid of three peptide antisera against two domains in the constant segment and one conserved...

Renal involvement in AL amyloidosis: the facts, the promise and the hope

Nephrology Dialysis Transplantation — Sun, 20 Sep 2009 23:00:00 +0100

(Source: Nephrology Dialysis Transplantation)

Clinical outcome of immunoglobulin light chain amyloidosis affecting the kidney

Nephrology Dialysis Transplantation — Sun, 20 Sep 2009 23:00:00 +0100

Conclusions. Presenting 24-h urine protein loss and creatinine values predict which patients will require dialysis. Median survival for patients starting dialysis is <1 year. The presence of light chain amyloid predicts the increased likelihood of renal involvement. (Source: Nephrology Dialysis Transplantation)