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Sample records for systemic al amyloidosis

  1. AL Amyloidosis

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    Desport Estelle

    2012-08-01

    Full Text Available Abstract Definition of the disease AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig light chains (LC (most commonly of lambda isotype usually secreted by a small plasma cell clone. Most patients have evidence of isolated monoclonal gammopathy or smoldering myeloma, and the occurrence of AL amyloidosis in patients with symptomatic multiple myeloma or other B-cell lymphoproliferative disorders is unusual. The key event in the development of AL amyloidosis is the change in the secondary or tertiary structure of an abnormal monoclonal LC, which results in instable conformation. This conformational change is responsible for abnormal folding of the LC, rich in β leaves, which assemble into monomers that stack together to form amyloid fibrils. Epidemiology AL amyloidosis is the most common type of systemic amyloidois in developed countries with an estimated incidence of 9 cases/million inhabitant/year. The average age of diagnosed patients is 65 years and less than 10% of patients are under 50. Clinical description The clinical presentation is protean, because of the wide number of tissues or organs that may be affected. The most common presenting symptoms are asthenia and dyspnoea, which are poorly specific and may account for delayed diagnosis. Renal manifestations are the most frequent, affecting two thirds of patients at presentation. They are characterized by heavy proteinuria, with nephrotic syndrome and impaired renal function in half of the patients. Heart involvement, which is present at diagnosis in more than 50% of patients, leading to restrictive cardiopathy, is the most serious complication and engages prognosis. Diagnostic methods The diagnosis relies on pathological examination of an involved site showing Congo red-positive amyloid deposits, with typical apple-green birefringence under polarized light, that stain positive with an anti-LC antibody by immunohistochemistry and

  2. Primary (AL) amyloidosis with gastrointestinal involvement

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    Madsen, Lone Galmstrup; Gimsing, Peter; Schiødt, Frank V

    2009-01-01

    OBJECTIVE: Immunoglobulin light-chain (AL) amyloidosis is a rare disease that can affect several organs. The aim of this study was to characterize patients with gastrointestinal manifestations of AL amyloidosis, in terms of symptoms, biochemistry, and outcome. MATERIAL AND METHODS: Retrospectivel...

  3. Imaging findings and literature review of 18F-FDG PET/CT in primary systemic AL amyloidosis

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    Lee, Joo Hee; Lee, Ga Yeon; Kim, Seok Jin; Kim, Ki Hyun; Jeon, Eun Seok; Lee, Kyung Han; Kim, Byung Tae; Choi, Joon Young

    2015-01-01

    Although several case reports and case series have described 18 F-FDG PET/CT in amyloidosis, the value of 18 F-FDG PET/CT for diagnosing amyloidosis has not been clarified. We investigated the imaging findings of 18 F-FDG PET/CT in patients with primary systemic AL amyloidosis. Subjects were 15 patients (M:F = 12:3; age, 61.5 ± 7.4 years) with histologically confirmed primary systemic AL amyloidosis who underwent pretreatment 18 F-FDG PET/CT to rule out the possibility of malignancy or for initial workup of alleged cancer. For involved organs, visual and semiquantitative analyses were performed on 18 F-FDG PET/CT images. In total, 22 organs (10 hearts, 5 kidneys, 2 stomachs, 2 colons, 1 ileum, 1 pancreas, and 1 liver) were histologically confirmed to have primary systemic AL amyloidosis. F-FDG uptake was significantly increased in 15 of the 22 organs (68.2 %; 10 hearts, 2 kidneys, 1 colon, 1 ileum, and 1 liver; SUV max  = 7.0 ± 3.2, range 2.1–14.1). However, in 11 of 15 PET-positive organs (78.6 %; 10 hearts and the ileum), it was difficult to differentiate pathological uptake from physiological uptake. Definitely abnormal 18 F-FDG uptake was found in only 4 of the 22 organs (18.2 %; 2 kidneys, 1 colon, and the liver). 18 F-FDG uptake was negative for pancreas and gastric lesions. Although 18 F-FDG PET/CT showed high uptake in two-thirds of the organs involving primary systemic AL amyloidosis, its sensitivity appeared to be low to make differentiation of pathological uptake from physiological uptake. However, due to the small number of cases, further study for the role of 18 F-FDG PET/CT in amyloidosis will be warranted

  4. What is the role of giant cells in AL-amyloidosis?

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    Olsen, K E; Sletten, K; Sandgren, O

    1999-01-01

    of some cases of systemic AL-amyloidosis. Based on these findings and electron microscopic studies, it is discussed whether the giant cells actively participate in amyloid fibril formation by uptake and modification of the precursor protein or the giant cells are part of a foreign body reaction. Included....... In this work it is shown that that there is a difference between localized and systemic amyloidosis in respect to accompanying giant cells which constantly are found associated with amyloid deposits in localized AL-amyloidosis. In addition, giant cells were found together with amyloid deposits in lymph nodes...

  5. Imaging findings and literature review of {sup 18}F-FDG PET/CT in primary systemic AL amyloidosis

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    Lee, Joo Hee; Lee, Ga Yeon; Kim, Seok Jin; Kim, Ki Hyun; Jeon, Eun Seok; Lee, Kyung Han; Kim, Byung Tae; Choi, Joon Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2015-09-15

    Although several case reports and case series have described {sup 18}F-FDG PET/CT in amyloidosis, the value of {sup 18}F-FDG PET/CT for diagnosing amyloidosis has not been clarified. We investigated the imaging findings of {sup 18}F-FDG PET/CT in patients with primary systemic AL amyloidosis. Subjects were 15 patients (M:F = 12:3; age, 61.5 ± 7.4 years) with histologically confirmed primary systemic AL amyloidosis who underwent pretreatment {sup 18}F-FDG PET/CT to rule out the possibility of malignancy or for initial workup of alleged cancer. For involved organs, visual and semiquantitative analyses were performed on {sup 18}F-FDG PET/CT images. In total, 22 organs (10 hearts, 5 kidneys, 2 stomachs, 2 colons, 1 ileum, 1 pancreas, and 1 liver) were histologically confirmed to have primary systemic AL amyloidosis. F-FDG uptake was significantly increased in 15 of the 22 organs (68.2 %; 10 hearts, 2 kidneys, 1 colon, 1 ileum, and 1 liver; SUV{sub max} = 7.0 ± 3.2, range 2.1–14.1). However, in 11 of 15 PET-positive organs (78.6 %; 10 hearts and the ileum), it was difficult to differentiate pathological uptake from physiological uptake. Definitely abnormal {sup 18}F-FDG uptake was found in only 4 of the 22 organs (18.2 %; 2 kidneys, 1 colon, and the liver). {sup 18}F-FDG uptake was negative for pancreas and gastric lesions. Although {sup 18}F-FDG PET/CT showed high uptake in two-thirds of the organs involving primary systemic AL amyloidosis, its sensitivity appeared to be low to make differentiation of pathological uptake from physiological uptake. However, due to the small number of cases, further study for the role of {sup 18}F-FDG PET/CT in amyloidosis will be warranted.

  6. Autologous Stem Cell Transplant for AL Amyloidosis

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    Vivek Roy

    2012-01-01

    Full Text Available AL amyloidosis is caused by clonal plasma cells that produce immunoglobulin light chains which misfold and get deposited as amyloid fibrils. Therapy directed against the plasma cell clone leads to clinical benefit. Melphalan and corticosteroids have been the mainstay of treatment for a number of years and the recent availability of other effective agents (IMiDs and proteasome inhibitors has increased treatment options. Autologous stem cell transplant (ASCT has been used in the treatment of AL amyloidosis for many years. It is associated with high rates of hematologic response and improvement in organ function. However, transplant carries considerable risks. Careful patient selection is important to minimize transplant related morbidity and mortality and ensure optimal patient outcomes. As newer more affective therapies become available the role and timing of ASCT in the overall treatment strategy of AL amyloidosis will need to be continually reassessed.

  7. The Clinical Presentation and Management of Systemic Light-Chain Amyloidosis in China.

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    Huang, Xiang-Hua; Liu, Zhi-Hong

    2016-04-01

    Amyloidosis includes a group of diseases characterized by the extracellular deposition of various fibrillary proteins that can autoaggregate in a highly abnormal fibrillary conformation. The amyloid precursor protein of systemic light-chain (AL) amyloidosis is comprised of monoclonal light chains that are due to plasma cell dyscrasia. The clinical presentation of patients with AL amyloidosis varies from patient to patient. Current treatment strategies target the clone in order to decrease the production of the pathologic light chains. Recent advances in therapy have helped many patients with AL amyloidosis achieve hematologic and organ responses. AL amyloidosis is the most common type of systemic amyloidosis in China with increasing morbidity and a high mortality rate. The clinical presentation of AL amyloidosis is variable, and the median overall survival was found to be 36.3 months. The disease prognosis and risk stratification are linked to serialized measurement of cardiac biomarkers and free light chains. The treatment of AL amyloidosis is mainly based on chemotherapy and autologous hematopoietic stem cell transplantation (ASCT). The use of novel agents (thalidomide, lenalidomide, and bortezomib) alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored. AL amyloidosis is the most common type of systemic amyloidosis in China with increasing morbidity and a high mortality rate. The lack of prospective clinical trials using the current therapies is a challenge for evidence-based decision making concerning the treatment of AL amyloidosis. (1) AL amyloidosis is the most prevalent type of amyloidosis accounting for 65% of the amyloidosis-diagnosed patients in the UK and for 93% of the amyloidosis-diagnosed patients in China. The predisposition of men over women to develop AL amyloidosis might be higher in China than in Western countries (2:1 vs. 1.3:1). Both in the East and West, incidence increases with age. At

  8. Overview of systemic and localized amyloidosis

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    Saulius Girnius

    2013-10-01

    Full Text Available Amyloidosis is a family of protein misfolding disorders, in which insoluble fibrillar proteins deposit extracellularly and cause end organ damage. Depending on the precursor protein, clinical manifestations in amyloidosis vary significantly. In systemic amyloidosis, the heart, kidneys, and nerves are most commonly affected, resulting in congestive heart failure, arrhythmia, nephrotic syndrome, renal failure, and peripheral and autonomic neuropathies. In localized amyloidosis, amyloid deposits at the site of production, so only one organ is disrupted. Once amyloidosis is confirmed histologically, the precursor subtype must be identified using immunohistochemistry, immunofixation, electron microscopy, or laser microdissection and mass spectrometry. Treatment should not be initiated prior to the identification of the type of amyloidosis. Currently, treatment focuses on the suppression of the precursor protein: in AL amyloidosis, chemotherapy or autologous stem cell transplants suppress production of immunoglobulin light chains; in AA amyloidosis, anti-microbial and anti-inflammatory agents suppress amyloid A production; and in AF amyloidosis, a liver transplantation removes the source of mutant transthyretin protein production. Newer drugs are being developed to target amyloidosis at an epigenetic level or stabilize folding intermediates, but there are currently in development.http://dx.doi.org/10.7175/rhc.v4i4.662

  9. Purpuric halo around hemangioma as a clue for primary systemic amyloidosis: Case report and review of literature

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    Yin-Shuo Chang

    2018-03-01

    Full Text Available Mucocutaneous lesion appears in up to 40% of patients with primary systemic amyloidosis (AL amyloidosis. The cutaneous signs may be co-expressed with other organ involvement or even solely presented as the first clue. We reported a case of systemic AL amyloidosis who was initially presented as a solitary hemangioma with a purpuric halo. Excisional biopsy revealed a hemangioma with amyloid deposited in thickened vascular walls. Systemic AL amyloidosis was diagnosed after thorough survey. The stage of disease at the time of initial treatment is the greatest prognostic factor. To our knowledge, this is the first case of systemic AL amyloidosis initially presented as a purpuric halo around hemangioma in Taiwan. This target-like lesion should be linked to systemic AL amyloidosis and early diagnosis is extraordinary important.

  10. Cancer-testis antigen expression and immunogenicity in AL amyloidosis

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    Rosenzweig, M A; Landau, H; Seldin, D; O'Hara, C; Girnius, S; Hanson, N; Frosina, D; Sedrak, C; Arcila, M; Comenzo, R L; Giralt, S; Gnjatic, S; Jungbluth, A A; Koehne, G

    2012-01-01

    Light-chain amyloidosis (AL) is a plasma cell dyscrasia closely related to multiple myeloma. In multiple myeloma, the cancer-testis antigens (CTAs) CT7 (MAGE-C1), CT10 (MAGE-C2) and MAGE-A CTAs are expressed in up to 80% of cases. In this study, we investigated the expression and immunogenicity of several CTAs in patients with AL amyloidosis in a total of 38 bone marrow specimens by employing standard immunohistochemistry techniques on paraffin-embedded archival tissues. Plasma samples from 35 patients (27 with matched bone marrow samples) were also analyzed by ELISA for sero reactivity to a group of full-length CTA proteins. CT7 was present in 25/38 (66%) while CT10 was demonstrated in 3/38 and GAGE in 1/38 AL amyloid cases. The expression pattern was mostly focal. There were no significant differences with regard to organ involvement, response to treatment, or prognosis in CTA positive compared to negative cases. None of the specimens showed spontaneous humoral immunity to CT7, but sero reactivity was observed in individual patients to other CTAs. This study identifies CT7 as the prevalent CTA in plasma cells of patients with AL amyloidosis. Further analyses determining the biology of CTAs in AL amyloidosis and their value as potential targets for immunotherapy are warranted

  11. Oral purpura as the first manifestation of primary systemic amyloidosis.

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    McCormick, Robert Stuart; Sloan, Philip; Farr, David; Carrozzo, Marco

    2016-07-01

    Oral blood blisters and purpura are rare features of primary systemic amyloidosis (amyloid light-chain (AL) amyloidosis). We report a case in which these unusual presentations led to a diagnosis of amyloidosis, which enabled effective treatment before organ failure. Copyright © 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  12. Bendamustine-Induced Nephrogenic Diabetes Insipidus in a Patient With AL Amyloidosis.

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    Uwumugambi, Nsabimana A; Sanchorawala, Vaishali; Shelton, Anthony C; Stern, Lauren; Gordon, Craig E

    2017-02-01

    Nephrogenic diabetes insipidus is a condition characterized by polyuria with dilute urine due to the inability of the principal cells of the renal collecting ducts to respond to antidiuretic hormone and concentrate urine. Nephrogenic diabetes insipidus can be drug induced, and several chemotherapeutic agents have been reported to cause it. Bendamustine is a traditional chemotherapeutic agent being studied for treatment for relapsed systemic AL amyloidosis. We report a case of a 59-year-old man with AL amyloidosis who developed partial nephrogenic diabetes insipidus after receiving bendamustine for treatment of AL amyloidosis. The nephrogenic diabetes insipidus responded well to sodium restriction, hydrochlorothiazide, and desmopressin treatment, allowing the patient to receive subsequent bendamustine cycles without polyuria. Nephrogenic diabetes insipidus resolved shortly after completion of bendamustine therapy. Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  13. AL Amyloidosis Complicated by Persistent Oral Bleeding

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    Luiz Antonio Liarte Marconcini

    2015-01-01

    Full Text Available A case of amyloid light chain (AL amyloidosis is presented here with uncontrolled bleeding after a nonsurgical dental procedure, most likely multifactorial in nature, and consequently treated with a multidisciplinary approach.

  14. The Prevalence and Management of Systemic Amyloidosis in Western Countries.

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    Nienhuis, Hans L A; Bijzet, Johan; Hazenberg, Bouke P C

    2016-04-01

    Amyloidosis has been a mystery for centuries, but research of the last decennia has clarified many of the secrets of this group of diseases. A protein-based classification of amyloidosis helps to understand problems that were part of the obsolete clinical classification in primary, secondary, and familial amyloidosis. All types of amyloid are secondary to some underlying precursor-producing process: each type is caused by a misfolded soluble precursor protein that becomes deposited as insoluble amyloid fibrils. The incidence of amyloidosis is not well documented, but probably falls between 5 and 13 per million per year. Prevalence data are scarce, but one UK study indicates about 20 per million inhabitants. Amyloidosis can be localized (amyloid deposited in the organ or tissue of precursor production) or systemic (amyloid at one or more sites distant from the site of precursor production). The major systemic types of amyloidosis are AL (associated with a light chain-producing plasma cell dyscrasia), AA (associated with longstanding inflammation), wild-type ATTR (associated with normal transthyretin and old age), and hereditary ATTR (associated with a transthyretin mutation) amyloidosis. Imaging techniques, such as cardiac ultrasound, magnetic resonance imaging, bone scintigraphy, and serum amyloid P component scintigraphy, are useful both for diagnosing amyloidosis and for assessing disease severity. Serologic markers are useful for detecting organ disease and disease monitoring during follow-up. Current treatment modalities are directed against the ongoing supply of precursor proteins and thereby aim to stop further accumulation of amyloid. Novel treatment modalities, such as interference with amyloid formation and even removal of amyloid, are being studied. A well-thought and planned monitoring during follow-up helps to assess the effect of treatment and to early detect possible progression of amyloidosis. Clinical management comprises histologic proof of amyloid

  15. Unique type of isolated cardiac valvular amyloidosis

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    Reehana Salma

    2006-10-01

    Full Text Available Abstract Background Amyloid deposition in heart is a common occurrence in systemic amyloidosis. But localised valvular amyloid deposits are very uncommon. It was only in 1922 that the cases of valvular amyloidosis were reported. Then in 1980, Goffin et al reported another type of valvular amyloidosis, which he called the dystrophic valvular amyloidosis. We report a case of aortic valve amyloidosis which is different from the yet described valvular amyloidosis. Case presentation A 72 years old gentleman underwent urgent aortic valve replacement. Intraoperatively, a lesion was found attached to the inferior surface of his bicuspid aortic valve. Histopathology examination of the valve revealed that the lesion contained amyloid deposits, identified as AL amyloidosis. The serum amyloid A protein (SAP scan was normal and showed no evidence of systemic amyloidosis. The ECG and echocardiogram were not consistent with cardiac amyloidosis. Conclusion Two major types of cardiac amyloidosis have been described in literature: primary-myelomatous type (occurs with systemic amyolidosis, and senile type(s. Recently, a localised cardiac dystrophic valvular amyloidosis has been described. In all previously reported cases, there was a strong association of localised valvular amyloidosis with calcific deposits. Ours is a unique case which differs from the previously reported cases of localised valvular amyloidosis. In this case, the lesion was not associated with any scar tissue. Also there was no calcific deposit found. This may well be a yet unknown type of isolated valvular amyloidosis.

  16. Stabilisation of Laryngeal AL Amyloidosis with Long Term Curcumin Therapy

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    Terry Golombick

    2015-01-01

    Full Text Available Multiple myeloma (MM, smoldering myeloma (SMM, and monoclonal gammopathy of undetermined significance (MGUS represent a spectrum of plasma cell dyscrasias (PCDs. Immunoglobulin light chain amyloidosis (AL falls within the spectrum of these diseases and has a mortality rate of more than 80% within 2 years of diagnosis. Curcumin, derived from turmeric, has been shown to have a clinical benefit in some patients with PCDs. In addition to a clinical benefit in these patients, curcumin has been found to have a strong affinity for fibrillar amyloid proteins. We thus administered curcumin to a patient with laryngeal amyloidosis and smoldering myeloma and found that the patient has shown a lack of progression of his disease for a period of five years. This is in keeping with our previous findings of clinical benefits of curcumin in patients with plasma cell dyscrasias. We recommend further evaluation of curcumin in patients with primary AL amyloidosis.

  17. Significant association between renal function and area of amyloid deposition in kidney biopsy specimens in both AA amyloidosis associated with rheumatoid arthritis and AL amyloidosis.

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    Kuroda, Takeshi; Tanabe, Naohito; Hasegawa, Eriko; Wakamatsu, Ayako; Nozawa, Yukiko; Sato, Hiroe; Nakatsue, Takeshi; Wada, Yoko; Ito, Yumi; Imai, Naofumi; Ueno, Mitsuhiro; Nakano, Masaaki; Narita, Ichiei

    2017-06-01

    The kidney is a major target organ for systemic amyloidosis, which results in proteinuria and an elevated serum creatinine level. The clinical manifestations and precursor proteins of amyloid A (AA) and light-chain (AL) amyloidosis are different, and the renal damage due to amyloid deposition also seems to differ. The purpose of this study was to clarify haw the difference in clinical features between AA and AL amyloidosis are explained by the difference in the amount and distribution of amyloid deposition in the renal tissues. A total of 119 patients participated: 58 patients with an established diagnosis of AA amyloidosis (AA group) and 61 with AL amyloidosis (AL group). We retrospectively investigated the correlation between clinical data, pathological manifestations, and the area occupied by amyloid in renal biopsy specimens. In most of the renal specimens the percentage area occupied by amyloid was less than 10%. For statistical analyses, the percentage area of amyloid deposition was transformed to a common logarithmic value (Log 10 %amyloid). The results of sex-, age-, and Log 10 %amyloid-adjusted analyses showed that systolic blood pressure (SBP) was higher in the AA group. In terms of renal function parameters, serum creatinine, creatinine clearance (Ccr) and estimated glomerular filtration rate (eGFR) indicated significant renal impairment in the AA group, whereas urinary protein indicated significant renal impairment in the AL group. Pathological examinations revealed amyloid was predominantly deposited at glomerular basement membrane (GBM) and easily transferred to the mesangial area in the AA group, and it was predominantly deposited at in the AL group. The degree of amyloid deposition in the glomerular capillary was significantly more severe in AL group. The frequency of amyloid deposits in extraglomerular mesangium was not significantly different between the two groups, but in AA group, the degree amyloid deposition was significantly more severe, and

  18. Light chain (AL amyloidosis: update on diagnosis and management

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    Rosenzweig Michael

    2011-11-01

    Full Text Available Abstract Light chain (AL amyloidosis is a plasma cell dyscrasia characterized by the pathologic production of fibrillar proteins comprised of monoclonal light chains which deposit in tissues and cause organ dysfunction. The diagnosis can be challenging, requiring a biopsy and often specialized testing to confirm the subtype of systemic disease. The goal of treatment is eradication of the monoclonal plasma cell population and suppression of the pathologic light chains which can result in organ improvement and extend patient survival. Standard treatment approaches include high dose melphalan (HDM followed by autologous hematopoietic stem cell transplantation (SCT or oral melphalan with dexamethasone (MDex. The use of novel agents (thalidomide, lenalidomide and bortezomib alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored. A risk adapted approach to SCT followed by novel agents as consolidation reduces treatment related mortality with promising outcomes. Immunotherapeutic approaches targeting pathologic plasma cells and amyloid precursor proteins or fibrils are being developed. Referral of patients to specialized centers focusing on AL amyloidosis and conducting clinical trials is essential to improving patient outcomes.

  19. Incidence and survival in non-hereditary amyloidosis in Sweden

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    Hemminki Kari

    2012-11-01

    Full Text Available Abstract Background Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics. Methods Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008. Results The incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years. However, as rheumatoid arthritis deaths also occurred in other diagnostic subtypes, the incidence of secondary systemic amyloidosis was likely to be about 2.0 per million. The median survival of patients with organ-limited amyloidosis was 6 years. Most myeloma deaths occurred in patients diagnosed with unspecified or ‘other’ amyloidosis. These subtypes probably accounted for most of immunoglobulin light chain (AL amyloidosis cases; the median survival time was 3 years. Conclusions The present diagnostic categorization cannot single out AL amyloidosis in the Swedish discharge data but, by extrapolation from myeloma cases, an incidence of 3.2 per million could be ascribed to AL amyloidosis. Similarly, based on rheumatoid arthritis death rates, an incidence of 2.0 could be ascribed to secondary systemic amyloidosis.

  20. Symptomatic Primary (AL Amyloidosis of the Stomach and Duodenum

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    Reidar Fossmark

    2013-01-01

    Full Text Available Primary (AL amyloidosis of the gastrointestinal tract is relatively rare, and symptomatic amyloidosis of the stomach is even more seldom. We present the case of a patient who was referred to upper endoscopy because of weight loss, nausea, and vomiting. Large areas of intramucosal hemorrhages were seen, and biopsies resulted in profuse bleeding stopped with endoscopic clips. The biopsies showed amyloid depositions and further workup revealed that the patient also had cardiac and neuropathic involvements. The patient started treatment with dexamethasone, melphalan and bortezomib. After treatment was started the nausea and epigastric discomfort improved, and a reduction in the biochemical markers troponin T, NT-proBNP, and M-component was observed. Gastric amyloidosis is rarely seen at upper endoscopy in patients without a previously established diagnosis, but the unusual endoscopic findings and bleeding tendency after biopsy should be kept in mind by gastroenterologists.

  1. Ventricular fibrillation after bortezomib therapy in a patient with systemic amyloidosis

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    Satoshi Yamasaki

    2013-09-01

    Full Text Available A 64-year-old female was diagnosed with systemic amyloidosis associated with multiple myeloma. Bortezomib and dexamethasone-therapy was initiated; however, she developed lethal ventricular fibrillation (VF and cardiac arrest after 84 hours of therapy. Cardiopulmonary resuscitation using direct current shocks with epinephrine and amiodarone was initiated but failed to receive cardiac function. Although her arterial pulsations recovered immediately after the injection of vasopressin, she died of heart failure 8 hours after the onset of VF. Cardiac amyloidosis was verified by autopsy. Although the direct association of bortezomib with lethal VF remained to be clarified in our patient, the current report emphasizes on bortezomib as a substantial risk factor for cardiomyocyte damage. The potential risk of lethal events associated with cardiac amyloidosis should be carefully considered during bortezomib treatment for patients with AL amyloidosis.

  2. Abdominal fat pad excisional biopsy for the diagnosis and typing of systemic amyloidosis.

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    Garcia, Yessica; Collins, A Bernard; Stone, James R

    2018-02-01

    In the past, the diagnosis and typing of amyloidosis often required an invasive biopsy of an internal organ, such as the heart or kidneys. Abdominal fat pad excisional biopsy (FPEB) offers a less invasive approach, but the sensitivity of this technique has been unclear. To determine the sensitivity of FPEB for immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, we performed a retrospective clinicopathologic analysis of 97 patients who had undergone FPEB, of which 16 were positive for amyloid. The most significant pretest feature predicting a positive FPEB was a serum free light chain κ/λ ratio less than .5, and in this group of patients the probability of a positive biopsy was dependent on the size of the biopsy (P=.004). In FPEBs, the amyloid was present in multiple distinct patterns: pericellular, septal, medium-sized vessel, small vessel, and nodular. For patients with AL amyloidosis for which direct typing was attempted using the FPEB tissue, the amyloid was successfully typed in the FPEB in 90% of cases. The overall sensitivity of FPEB was 79% for AL amyloidosis and 12% for ATTR amyloidosis (P=.0003). In patients with AL amyloidosis, the sensitivity of FPEB was dependent on biopsy size, with small biopsies (≤700 mm 3 ) having a sensitivity of ~50%, and large biopsies (>700 mm 3 ) having a sensitivity of ~100%. This study demonstrates that FPEB has high sensitivity for AL amyloidosis, and can be routinely used to type the amyloid. However, FPEB has low sensitivity for ATTR amyloidosis in our patient population. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Characteristics and Long-Term Outcome of Patients with Systemic Immunoglobulin Light-Chain Amyloidosis

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    Nelson, Lærke Marie; Gustafsson, Finn; Gimsing, Peter

    2015-01-01

    Background/Aims: Immunoglobulin light-chain (AL) amyloidosis is a systemic disorder that causes progressive organ dysfunction. The optimal treatment strategy requires accurate patient stratification with an emphasis on the extent of cardiac involvement. Reports on its prognosis are sparse...... and predominantly originate from highly selected centers. We aimed to evaluate patient characteristics and outcomes in a cohort treated at a single center. Methods: This is a single-center retrospective study of 63 consecutive patients diagnosed with AL amyloidosis between January 2000 and December 2012. Patients...... were evaluated by treatment strategy and cardiac involvement. Results: The mean age at diagnosis was 61.4 years (±8.9), and 39 patients (62%) were male. Thirty-two (51%) patients presented with cardiac amyloid involvement (CA) and the remaining 31 (49%) had noncardiac amyloidosis (NCA). The median...

  4. {sup 18}F-FDG PET/CT in Primary AL Hepatic Amyloidosis Associated with Multiple Myeloma

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    Son, Youn Mi; Bak, Cheol Hee [Seoul Medical Center, Seoul (Korea, Republic of); Choi, Joon Young; Cheon, Mi Ju; Kim, Young Eun; Lee, Kyung Han; Kim, Byung Tae [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2011-10-15

    We report here on a rare case of primary AL hepatic amyloidosis associated with multiple myeloma in a 64-year-old woman. The patient was referred for evaluating her progressive jaundice and right upper quadrant pain. {sup 18}F-fluorodeoxyglucose ({sup 18}F-FDG) positron emission tomography (PET)/computed tomography (CT) showed diffusely and markedly increased {sup 18}F-FDG uptake in the liver. Although there have been several case studies showing positive {sup 18}F-FDG uptake in pulmonary amyloidosis, to the best of our knowledge, the {sup 18}F-FDG PET/CT findings of hepatic amyloidosis or primary hepatic amyloidosis associated with multiple myeloma have not been reported previously.

  5. IgG,kappa monoclonal gammopathy of unknown significance with AL amyloidosis simulating giant cell arteritis

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    Pompilian Valer Mihai

    2017-09-01

    Full Text Available Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA. We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension. Muscular strength was normal. She had high ESR and CRP; in this clinical context, GCA was suspected. A gamma spike on serum protein electrophoresis raised the suspicion of monoclonal gammopathy (MG. Immunoelectrophoresis revealed monoclonal bands for IgG and kappa chains. Massive deposits of amyloid and no inflammation were found on temporal artery biopsy. Multiple myeloma and lymphoma were ruled out. A diagnosis of AL amyloidosis complicating MGUS was formulated. She did well on therapy with bortezomib, cyclophosphamide and dexamethasone. Cases published in medical literature reveal amyloidosis mimicking GCA in the setting of established MGUS. As far as we know, this is the first case of MGUS with IgG and kappa chains in which a GCA-like picture induced by amyloidosis was present from the very onset.

  6. Dysautonomias in Amyloidosis: : Need for an interdisciplinary approach

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    Hazenberg, B. P. C.

    2013-01-01

    Systemic amyloidosis is a life-threatening and frequently unrecognized cause of dysautonomia. Autonomic neuropathy is a common manifestation of AL amyloidosis (caused by deposition of an immunoglobulin free light chain produced by an underlying plasma cell clone) and of autosomal dominant hereditary

  7. Comparison of Different Stem Cell Mobilization Regimens in AL Amyloidosis Patients.

    Science.gov (United States)

    Lisenko, Katharina; Wuchter, Patrick; Hansberg, Marion; Mangatter, Anja; Benner, Axel; Ho, Anthony D; Goldschmidt, Hartmut; Hegenbart, Ute; Schönland, Stefan

    2017-11-01

    High-dose melphalan (HDM) and autologous blood stem cell transplantation (ABSCT) is an effective treatment for transplantation-eligible patients with systemic light chain (AL) amyloidosis. Whereas most centers use granulocyte colony-stimulating factor (G-CSF) alone for mobilization of peripheral blood stem cells (PBSC), the application of mobilization chemotherapy might offer specific advantages. We retrospectively analyzed 110 patients with AL amyloidosis who underwent PBSC collection. Major eligibility criteria included age CSF (n = 78, 71%); ifosfamide/G-CSF (n = 14, 13%); or other regimens (n = 8, 7%). AL amyloidosis patients with predominant heart involvement and/or status post heart transplantation were mobilized with G-CSF only (n = 10, 9%). PBSC collection was successful in 101 patients (92%) at first attempt. The median number of CD34 + cells was 8.7 (range, 2.1 to 45.5) × 10 6 CD34 + /kg collected in a median of 1 leukapheresis (LP) session. Compared with G-CSF-only mobilization, a chemo-mobilization with CAD/G-CSF or ifosfamide/G-CSF had a positive impact on the number of collected CD34 + cell number/kg per LP (P CSF mobilization (median CTC: grade 3; range, 1 to 4). Toxicity in patients undergoing ifosfamide/G-CSF mobilization was higher than in with those who received G-CSF-only mobilization. HDM and ABSCT were performed in 100 patients. Compared with >6.5 × 10 6 transplanted CD34 + cells/kg, an ABSCT with 1 × 10 9 /L and a reduced platelet count CSF mobilization alone is also safe and effective. Considering the hematopoietic reconstitution and long-term stem cell function, our results provide a rationale to collect and transplant as many as >6.5 × 10 6 CD34 + cells/kg, if feasible with reasonable effort. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

  8. Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component

    International Nuclear Information System (INIS)

    Hawkins, P.N.; Lavender, J.P.; Pepys, M.B.

    1990-01-01

    In systemic amyloidosis the distribution and progression of disease have been difficult to monitor, because they can be demonstrated only by biopsy. Serum amyloid P component (SAP) is a normal circulating plasma protein that is deposited on amyloid fibrils because of its specific binding affinity for them. We investigated whether labeled SAP could be used to locate amyloid deposits. Purified human SAP labeled with iodine-123 was given intravenously to 50 patients with biopsy-proved systemic amyloidosis--25 with the AL (primary) type and 25 with the AA (secondary) type--and to 26 control patients with disease and 10 healthy subjects. Whole-body images and regional views were obtained after 24 hours and read in a blinded fashion. In the patients with amyloidosis the 123I-SAP was localized rapidly and specifically in amyloid deposits. The scintigraphic images obtained were characteristic and appeared to identify the extent of amyloid deposition in all 50 patients. There was no uptake of the 123I-SAP by the control patients and the healthy subjects. In all patients with AA amyloidosis the spleen was affected, whereas the scans showed uptake in the heart, skin, carpal region, and bone marrow only in patients with the AL type. Positive images were seen in six patients in whom biopsies had been negative or unsuccessful; in all six, amyloid was subsequently found on biopsy or at autopsy. Progressive amyloid deposition was observed in 9 of 11 patients studied serially. Scintigraphy after the injection of 123I-SAP can be used for diagnosing, locating, and monitoring the extent of systemic amyloidosis

  9. Diagnostic and therapeutic approach of systemic amyloidosis

    NARCIS (Netherlands)

    Hazenberg, BPC; van Gameren, [No Value; Bijzet, J; Jager, PL; van Rijswijk, MH

    Amyloidosis is a group of diseases, all characterised by deposition of protein fibrils with a beta-sheet structure. This structure generates affinity of amyloid for Congo red dye and is resistant to proteolysis. Three types of systemic amyloidosis are important for the clinician: AA (related to

  10. Clinical approach of patients with systemic amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Bouke

    2011-01-01

    Amyloidosis is the name of diseases characterised by deposition of protein fibrils with a beta-sheet structure. This beta-sheet structure generates affinity of amyloid for Congo red dye and is resistant to proteolysis. The main three types of systemic amyloidosis are AA (related to underlying

  11. Extracardiac 18F-florbetapir imaging in patients with systemic amyloidosis: more than hearts and minds.

    Science.gov (United States)

    Wagner, T; Page, J; Burniston, M; Skillen, A; Ross, J C; Manwani, R; McCool, D; Hawkins, P N; Wechalekar, Ashutosh D

    2018-07-01

    18 F-Florbetapir has been reported to show cardiac uptake in patients with systemic light-chain amyloidosis (AL). This study systematically assessed uptake of 18 F-florbetapir in patients with proven systemic amyloidosis at sites outside the heart. Seventeen patients with proven cardiac amyloidosis underwent 18 F-florbetapir PET/CT imaging, 15 with AL and 2 with transthyretin amyloidosis (ATTR). Three patients had repeat scans. All patients had protocolized assessment at the UK National Amyloidosis Centre including imaging with 123 I-serum amyloid P component (SAP). 18 F-Florbetapir images were assessed for areas of increased tracer accumulation and time-uptake curves in terms of standardized uptake values (SUV mean ) were produced. All 17 patients showed 18 F-florbetapir uptake at one or more extracardiac sites. Uptake was seen in the spleen in 6 patients (35%; 6 of 9, 67%, with splenic involvement on 123 I-SAP scintigraphy), in the fat in 11 (65%), in the tongue in 8 (47%), in the parotids in 8 (47%), in the masticatory muscles in 7 (41%), in the lungs in 3 (18%), and in the kidney in 2 (12%) on the late half-body images. The 18 F-florbetapir spleen retention index (SRI) was calculated. SRI >0.045 had 100% sensitivity/sensitivity (in relation to 123 I-SAP splenic uptake, the current standard) in detecting splenic amyloid on dynamic imaging and a sensitivity of 66.7% and a specificity of 100% on the late half-body images. Intense lung uptake was seen in three patients, one of whom had lung interstitial infiltration suggestive of amyloid deposition on previous high-resolution CT. Repeat imaging showed a stable appearance in all three patients suggesting no early impact of treatment response. 18 F-Florbetapir PET/CT is a promising tool for the detection of extracardiac sites of amyloid deposition. The combination of uptake in the heart and uptake in the spleen on 18 F-florbetapir PET/CT, a hallmark of AL, suggests that this tracer holds promise as a screening tool

  12. New insights into systemic amyloidosis: primary amyloidosis associated with tubercular lymphadenitis

    Directory of Open Access Journals (Sweden)

    Shivraj Meena, Nirmal Ghati, Rita Sood, Naval Kishore Vikram

    2014-11-01

    Full Text Available Tuberculosis is generally followed by secondary amyloidosis. The association of primary systemic amyloidosis with tuberculosis is very rare. There is only one case thus far reported in literature. We report such a rare case of primary amyloidosis with tuberculous lymphadenopathy. A 45 year old woman presented at the medicine department of all India institute of medical sciences , New Delhi with on & off erythematous rashes over both eyes for 1 year; low grade fever, fatigue and significant weight loss for 4 months, dysphagia for solid food since 1 month. Main finding on examination were pallor, macroglossia, bilateral periorbital erythematous rashes (racoon eyes, hepatomegaly & cardiomegaly. She had raised serum alkaline phosphatase level. Chest x-ray revealed cardiomegaly. USG abdomen revealed multiple retroperitoneal mesenteric lymph nodes and hepatomegaly. USG guided FNAC from mesenteric lymph node showed acid fast bacillus. Histological examination of liver biopsy showed amyloid deposition on congo red stain. Patient was treated with DOTS category I ATT with Bortezomib and Dexamethasone based weekly chemotherapy.

  13. Primary Systemic Amyloidosis Presenting as Swollen Dense Breast: A Case Report

    International Nuclear Information System (INIS)

    Lee, Byung Hoon; Kim, Mi Young; Kim, Su Young; Hwang, Yoon Joon; Han, Yoon Hee; Seo, Jung Wook; Kim, Yong Hoon; Cha, Soon Joo; Hur, Gham; Joo, Mee

    2006-01-01

    Breast involvement of primary systemic amyloidosis is rare. This is a rare case of breast amyloidosis presenting as a diffuse infiltrative lesion. We present the mammographic, ultrasound, and MR findings of a systemic primary amyloidosis involving the breast with diffuse infiltrative pattern

  14. Optimization of Serum Immunoglobulin Free Light Chain Analysis for Subclassification of Cardiac Amyloidosis.

    Science.gov (United States)

    Halushka, Marc K; Eng, George; Collins, A Bernard; Judge, Daniel P; Semigran, Marc J; Stone, James R

    2015-06-01

    Accurate and rapid classification of cardiac amyloidosis is important for patient management. We have optimized the use of serum free light chain kappa and lambda values to differentiate immunoglobulin light chain amyloid (AL) amyloidosis from transthyretin amyloid and amyloid A using 85 cases of tissue-proven cardiac amyloidosis, in which there was direct classification of amyloidosis by mass spectrometry or immunofluorescence. The serum free light chain kappa/lambda ratios were non-overlapping for the three major groups: AL-lambda (0.01-0.41, n = 30), non-AL (0.52-2.7, n = 43), and AL-kappa (6.7-967, n = 12). A kappa/lambda ratio value between 0.5 and 5.0 had 100 % sensitivity and 100 % specificity for distinguishing AL amyloidosis from non-AL amyloidosis. This optimized range for serum light chain kappa/lambda ratio provides extremely robust classification of cardiac amyloidosis. Cases of cardiac amyloidosis in which the serum kappa/lambda free light chain ratio falls close to these new cutoff values may benefit most from direct amyloid subtyping.

  15. Anxiety and depression among amyloid light-chain cardiac amyloidosis patients: The role of life satisfaction.

    Science.gov (United States)

    Smorti, Martina; Guarnieri, Silvia; Bergesio, Franco; Perfetto, Federico; Cappelli, Francesco

    2016-06-01

    The present study aimed to provide a contribution to the study of a rare disease, amyloid light-chain (AL) cardiac amyloidosis, which is the most common type of systemic amyloidosis. In AL amyloidosis prognosis is determined by cardiac involvement. Although the association between psychological distress (e.g. anxiety and depression) and AL cardiac amyloidosis is documented, very little is known about the psychosocial variables that may mediate the association. The aim of the study is therefore to examine the potential mediating role of life satisfaction in the relationship between cardiac symptom severity (independent variable) and anxious and depressive symptoms (dependent variables) in AL patients. Forty-three AL amyloidosis patients (57.1% males) with cardiac amyloidosis were administered the Satisfaction with Life Scale, the State-Trait Anxiety Inventory and the Centre for Epidemiological Study-Depression Scale. Clinical variables such as months since cardiac symptom onset and cardiac symptom severity were collected. Findings showed significant relationships between symptom severity and psychological disorders (e.g. anxiety and depression) and these were mediated by life satisfaction. Overall, findings highlight the importance of subjective well-being (e.g. life satisfaction) to reduce anxious and depressive symptoms and to improve general health in AL patients. © The European Society of Cardiology 2015.

  16. Bilateral Non-arteritic Anterior Ischaemic Optic Neuropathy as the Presentation of Systemic Amyloidosis.

    Science.gov (United States)

    Kanaan, M Z; Lorenzi, A R; Thampy, N; Pandit, R; Dayan, Margaret

    2017-12-01

    A 75-year-old hypertensive female with stable idiopathic intermediate uveitis presented with bilateral sequential optic neuropathy with optic disc swelling. The optic neuropathy in the first affected eye (right) was thought to be due to non-arteritic anterior ischaemic optic neuropathy (NAION). Asymptomatic left optic disc swelling was found at routine review 2 months later, and a diagnosis of giant cell arteritis (GCA) was sought. Temporal artery duplex ultrasound showed the "halo sign," but a subsequent temporal artery biopsy showed light-chain (AL) amyloidosis with no signs of giant cell arteritis. In this case, bilateral sequential ischaemic optic neuropathy mimicking non-arteritic anterior ischaemic optic neuropathy was the presenting sign of systemic amyloidosis involving the temporal arteries.

  17. Renal amyloidosis: a synopsis of its clinical presentation, diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Elena V. Zakharova

    2015-04-01

    Full Text Available Amyloidosis is a heterogeneous group of hereditary and acquired diseases in which normally soluble plasma proteins are deposited in the extracellular and/or intracellular space in abnormal, insoluble, fibrillar form. Renal damage is one of the most common features of systemic amyloidosis, and the presentation is most commonly due to the consequences of renal involvement, with proteinuria and progressive renal decline. Progression to end-stage renal failure is common. Early diagnosis of systemic amyloidosis is difficult. Renal amyloidosis typically presents with nephrotic syndrome and/or renal failure. Treatment of AL amyloidosis aims to reduce production of the monoclonal immunoglobulin precursor via chemotherapy. Current options for treatment include melphalan+dexamethasone or cyclophosphamide-bortezomib-dexamethasone regimens, or in selected patients, high-dose melphalan with autologous stem cell transplantation. The focus of current research is on pharmacological therapy to solubilize amyloid fibrils and increase tissue catabolism of amyloid deposits.

  18. 2A4 binds soluble and insoluble light chain aggregates from AL amyloidosis patients and promotes clearance of amyloid deposits by phagocytosis †.

    Science.gov (United States)

    Renz, Mark; Torres, Ronald; Dolan, Philip J; Tam, Stephen J; Tapia, Jose R; Li, Lauri; Salmans, Joshua R; Barbour, Robin M; Shughrue, Paul J; Nijjar, Tarlochan; Schenk, Dale; Kinney, Gene G; Zago, Wagner

    2016-09-01

    Amyloid light chain (AL) amyloidosis is characterized by misfolded light chain (LC) (amyloid) deposition in various peripheral organs, leading to progressive dysfunction and death. There are no regulatory agency-approved treatments for AL amyloidosis, and none of the available standard of care approaches directly targets the LC protein that constitutes the amyloid. NEOD001, currently in late-stage clinical trials, is a conformation-specific, anti-LC antibody designed to specifically target misfolded LC aggregates and promote phagocytic clearance of AL amyloid deposits. The present study demonstrated that the monoclonal antibody 2A4, the murine form of NEOD001, binds to patient-derived soluble and insoluble LC aggregates and induces phagocytic clearance of AL amyloid in vitro. 2A4 specifically labeled all 21 fresh-frozen organ samples studied, which were derived from 10 patients representing both κ and λ LC amyloidosis subtypes. 2A4 immunoreactivity largely overlapped with thioflavin T-positive labeling, and 2A4 bound both soluble and insoluble LC aggregates extracted from patient tissue. Finally, 2A4 induced macrophage engagement and phagocytic clearance of AL amyloid deposits in vitro. These findings provide further evidence that 2A4/NEOD001 can effectively clear and remove human AL-amyloid from tissue and further support the rationale for the evaluation of NEOD001 in patients with AL amyloidosis.

  19. 2A4 binds soluble and insoluble light chain aggregates from AL amyloidosis patients and promotes clearance of amyloid deposits by phagocytosis †

    Science.gov (United States)

    Renz, Mark; Torres, Ronald; Dolan, Philip J.; Tam, Stephen J.; Tapia, Jose R.; Li, Lauri; Salmans, Joshua R.; Barbour, Robin M.; Shughrue, Paul J.; Nijjar, Tarlochan; Schenk, Dale; Kinney, Gene G.; Zago, Wagner

    2016-01-01

    Abstract Amyloid light chain (AL) amyloidosis is characterized by misfolded light chain (LC) (amyloid) deposition in various peripheral organs, leading to progressive dysfunction and death. There are no regulatory agency–approved treatments for AL amyloidosis, and none of the available standard of care approaches directly targets the LC protein that constitutes the amyloid. NEOD001, currently in late-stage clinical trials, is a conformation-specific, anti-LC antibody designed to specifically target misfolded LC aggregates and promote phagocytic clearance of AL amyloid deposits. The present study demonstrated that the monoclonal antibody 2A4, the murine form of NEOD001, binds to patient-derived soluble and insoluble LC aggregates and induces phagocytic clearance of AL amyloid in vitro. 2A4 specifically labeled all 21 fresh-frozen organ samples studied, which were derived from 10 patients representing both κ and λ LC amyloidosis subtypes. 2A4 immunoreactivity largely overlapped with thioflavin T–positive labeling, and 2A4 bound both soluble and insoluble LC aggregates extracted from patient tissue. Finally, 2A4 induced macrophage engagement and phagocytic clearance of AL amyloid deposits in vitro. These findings provide further evidence that 2A4/NEOD001 can effectively clear and remove human AL-amyloid from tissue and further support the rationale for the evaluation of NEOD001 in patients with AL amyloidosis. PMID:27494229

  20. Safety and efficacy of high-dose melphalan and auto-SCT in patients with AL amyloidosis and cardiac involvement.

    Science.gov (United States)

    Girnius, S; Seldin, D C; Meier-Ewert, H K; Sloan, J M; Quillen, K; Ruberg, F L; Berk, J L; Doros, G; Sanchorawala, V

    2014-03-01

    In Ig light chain (AL) amyloidosis, cardiac involvement is associated with worse prognosis and increased treatment-related complications. In this retrospective cohort study, we assessed survival, hematologic and cardiac responses to high-dose melphalan and auto-SCT (HDM/SCT) in patients with AL amyloidosis and cardiac involvement, stratified by cardiac biomarkers brain natriuretic peptide and Troponin I, analogous to the Mayo cardiac staging. Forty-seven patients underwent HDM/SCT based upon functional measures; six patients had modified cardiac stage I disease, seventeen had modified cardiac stage II disease and twenty-four had modified cardiac stage III disease. Treatment-related mortality was 4% for all patients and 8% for patients with stage III disease. Three-year survival was 88% and EFS was 47%; these did not differ by stage. By intention-to-treat analysis, 27% of patients achieved a hematologic complete response and 32% a very good partial response, of whom 70 and 45%, respectively, have not required additional therapy at 36 months. Cardiac response was achieved in 53% of patients. We conclude that with appropriate patient selection and a risk-adapted treatment approach, HDM/SCT is safe and effective in patients with AL amyloidosis and cardiac involvement.

  1. Auto-SCT improves survival in systemic light chain amyloidosis: a retrospective analysis with 14-year follow-up.

    Science.gov (United States)

    Parmar, S; Kongtim, P; Champlin, R; Dinh, Y; Elgharably, Y; Wang, M; Bashir, Q; Shah, J J; Shah, N; Popat, U; Giralt, S A; Orlowski, R Z; Qazilbash, M H

    2014-08-01

    Optimal treatment approach continues to remain a challenge for systemic light chain amyloidosis (AL). So far, Auto-SCT is the only modality associated with long-term survival. However, failure to show survival benefit in randomized study raises questions regarding its efficacy. We present a comparative outcome analysis of Auto-SCT to conventional therapies (CTR) in AL patients treated over a 14-year period at our institution. Out of the 145 AL amyloidosis patients, Auto-SCT was performed in 80 patients with 1-year non-relapse mortality rate of 12.5%. Novel agents were used as part of induction therapy in 56% of transplant recipients vs 46% of CTR patients. Hematological and organ responses were seen in 74.6% and 39% in the Auto-SCT arm vs 53% and 12% in the CTR arm, respectively. The projected 5-year survival for Auto-SCT vs CTR was 63% vs 38%, respectively. Landmark analysis of patients alive at 1-year after diagnosis showed improved 5-year OS of 72% with Auto-SCT vs 65% in the CTR arm. In the multivariate analysis, age SCT were associated with improved survival. In conclusion, Auto-SCT is associated with long-term survival for patients with AL amyloidosis.

  2. Subtle neuropsychiatric and neurocognitive changes in hereditary gelsolin amyloidosis (AGel amyloidosis)

    OpenAIRE

    Kantanen, Mari; Kiuru-Enari, Sari; Salonen, Oili; Kaipainen, Markku; Hokkanen, Laura

    2014-01-01

    Hereditary gelsolin amyloidosis (AGel amyloidosis) is an autosomal dominant form of systemic amyloidosis caused by a c.640G>A or c.640G>T mutation in the gene coding for gelsolin. Principal clinical manifestations include corneal lattice dystrophy, cranial neuropathy and cutis laxa with vascular fragility. Signs of minor CNS involvement have also been observed, possibly related to cerebral amyloid angiopathy (CAA). To investigate further if AGel amyloidosis carries a risk for a specific neuro...

  3. Visualization of multiple organ amyloid involvement in systemic amyloidosis using {sup 11}C-PiB PET imaging

    Energy Technology Data Exchange (ETDEWEB)

    Ezawa, Naoki; Katoh, Nagaaki; Yoshinaga, Tsuneaki [Shinshu University School of Medicine, Department of Medicine (Neurology and Rheumatology), Nagano (Japan); Oguchi, Kazuhiro [Jisenkai Brain Imaging Research Center, Matsumoto (Japan); Yazaki, Masahide [Shinshu University School of Health Sciences, Department of Biomedical Laboratory Sciences, Matsumoto (Japan); Shinshu University, Institute for Biomedical Sciences, Matsumoto (Japan); Sekijima, Yoshiki [Shinshu University School of Medicine, Department of Medicine (Neurology and Rheumatology), Nagano (Japan); Jisenkai Brain Imaging Research Center, Matsumoto (Japan); Shinshu University, Institute for Biomedical Sciences, Matsumoto (Japan)

    2018-03-15

    To investigate the utility of Pittsburgh compound B (PiB) positron emission tomography (PET) imaging for evaluating whole-body amyloid involvement in patients with systemic amyloidosis. Whole-body {sup 11}C-PiB PET was performed in seven patients with systemic immunoglobulin light-chain (AL) amyloidosis, seven patients with hereditary transthyretin (ATTRm) amyloidosis, one asymptomatic TTR mutation carrier and three healthy controls. The correlations between clinical organ involvement, radiological {sup 11}C-PiB uptake and histopathological findings were analysed for each organ. Organ involvement on {sup 11}C-PiB PET imaging showed good correlations with the clinical findings for the heart and stomach. Abnormal tracer uptake was also observed in the spleen, lachrymal gland, submandibular gland, sublingual gland, lymph node, brain, scalp, extraocular muscles, nasal mucosa, pharynx, tongue and nuchal muscles, most of which were asymptomatic. Physiological tracer uptake was universally observed in the urinary tract (kidney, renal pelvis, ureter and bladder) and enterohepatic circulatory system (liver, gallbladder, bile duct and small intestine) in all participants. Most of the patients and one healthy control subject showed asymptomatic tracer uptake in the lung and parotid gland. The peripheral nervous system did not show any tracer uptake even in patients with apparent peripheral neuropathy. Histological amyloid deposition was confirmed in biopsied myocardium and gastric mucosa where abnormal {sup 11}C-PiB retention was observed. {sup 11}C-PiB PET imaging can be used clinically in the systemic evaluation of amyloid distribution in patients with AL and ATTRm amyloidosis. Quantitative analysis of {sup 11}C-PiB PET images may be useful in therapy evaluation and will reveal whether amyloid clearance is correlated with clinical response. (orig.)

  4. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL) : A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis

    NARCIS (Netherlands)

    Gertz, MA; Comenzo, R; Falk, RH; Fermand, JP; Hazenberg, BP; Hawkins, PN; Merlini, G; Moreau, P; Ronco, P; Sanchorawala, [No Value; Sezer, O; Solomon, A; Grateau, G

    We undertook this study to develop uniformly accepted criteria for the definition of organ involvement and response for patients on treatment protocols for immunoglobulin light-chain amyloidosis (AL). A consensus panel was convened comprising 13 specialists actively involved in the treatment of

  5. Treatment of amyloidosis.

    Science.gov (United States)

    Tan, S Y; Pepys, M B; Hawkins, P N

    1995-08-01

    Amyloidosis is the extracellular deposition of normally soluble autologous protein in a characteristic abnormal fibrillar form. Systemic amyloidosis and some local forms are progressive, cause major morbidity, and are often fatal. No treatment specifically causes the resolution of amyloid deposits, but therapy that reduces the supply of amyloid fibril precursor proteins can improve survival and preserve organ function. Major regression of amyloid occurs in at least a proportion of such cases, suggesting that the clinical improvement reflects mobilization of amyloid. The clearest evidence for regression of amyloid has been obtained in juvenile rheumatoid arthritis patients with AA amyloidosis treated with chlorambucil. This drug suppresses the acute phase production of serum amyloid A protein, the precursor of AA amyloid fibrils, and is associated with remission of proteinuria and greatly improved survival. In many such patients, scintigraphy with serum amyloid P component shows major regression of amyloid over 12 to 36 months and frequently reveals a discrepancy between the local amyloid load and organ dysfunction. Measurement of target organ function is therefore not an adequate method for monitoring treatment aimed at promoting the resolution of amyloid. In monoclonal immunoglobulin light chain (AL) amyloidosis the aim of treatment is to suppress the underlying B-cell clone and, therefore, production of the amyloid fibril precursor protein. This can be difficult to achieve or sustain and, since the prognosis is so poor, many patients die before benefits of therapy are realized. A recent development has been the introduction of liver transplantation as treatment for familial amyloid polyneuropathy caused by transthyretin gene mutations. This leads to the disappearance of variant transthyretin from the plasma and halts progression of the neurologic disease. Features of autonomic neuropathy frequently ameliorate, and improvement in peripheral motor nerve function

  6. Nutritional status independently affects quality of life of patients with systemic immunoglobulin light-chain (AL) amyloidosis.

    Science.gov (United States)

    Caccialanza, Riccardo; Palladini, Giovanni; Klersy, Catherine; Cereda, Emanuele; Bonardi, Chiara; Cameletti, Barbara; Montagna, Elisabetta; Russo, Paola; Foli, Andrea; Milani, Paolo; Lavatelli, Francesca; Merlini, Giampaolo

    2012-03-01

    Nutritional status is an independent prognostic factor in immunoglobulin light-chain amyloidosis (AL), but its influence on quality of life (QoL) is unknown. The aim of this cross-sectional study was to investigate the association between nutritional status and QoL in AL patients at diagnosis. One hundred and fifty consecutive patients with biopsy-proven AL were assessed for nutritional status by anthropometry [body mass index, unintentional weight loss (WL) in the previous 6 months and mid-arm muscle circumference (MAMC)], biochemistry (serum prealbumin), and semiquantitative food intake at referral. QoL was assessed by the Medical Outcomes Study 36-item Short Form General Health Survey. The composite physical component summary (PCS) and the mental component summary (MCS) for AL outpatients were 36.2 ± 10.1 and 44.9 ± 11.3, respectively (p performance status, the number of organs involved, the severity of cardiac damage, C-reactive protein, energy intake, and WL, PCS was significantly lower for serum prealbumin Nutritional status independently affects QoL in AL patients since diagnosis. Nutritional evaluation should be integral part of the clinical assessment of AL patients. Nutritional support intervention trials are warranted in such patients' population.

  7. Utility of abdominal skin plus subcutaneous fat and rectal mucosal biopsy in the diagnosis of AL amyloidosis with renal involvement.

    Directory of Open Access Journals (Sweden)

    Ting Li

    Full Text Available Skin fat biopsy of the abdominal wall is a simple and safe method for detecting amyloidosis, and rectal mucosal biopsy is also frequently used for screening for the disease; however, the sensitivity of these approaches has not been fully studied. The aim of this study was to evaluate the efficacy of skin fat biopsy combined with rectal mucosal biopsy as a screening procedure for the diagnosis of systemic immunoglobulin light-chain (AL amyloidosis.We retrospectively analyzed 224 AL amyloidosis patients confirmed by renal biopsy, including a test group of 165 patients and validation group of 59 patients. Surgical skin fat biopsy from the abdominal wall and rectal mucosal biopsy under endoscopy was performed to obtain specimens. Congo red staining and immunofluorescence staining with antibodies against light chains were performed to type the disease. Pathology reports were reviewed to assess the diagnostic sensitivity of skin fat biopsy and rectal mucosal biopsy. Diagnostic specificity was not examined in the present study, because no healthy volunteers and only few patients with other diseases had performed immunofluorescence staining on skin fat and rectal specimens.Of the 165 patients in the test group, Congo red staining of skin fat and rectal mucosal specimens was associated with a sensitivity of 89.3% and 94.8%, respectively. The sensitivity increased to 98.9% by combining both biopsy methods. Immunofluorescence stains were positive in 81.1% of patients undergoing skin fat biopsy and 84.7% of patients undergoing rectal mucosal biopsy. Immunofluorescence stains yielded positive results in 86.7% of cases combining skin fat biopsy with rectal mucosal biopsy. The diagnostic results also performed well in the validation group.Surgical skin biopsy including the subcutaneous fat pad can be performed safely at the bedside and is useful for diagnosing AL amyloidosis. Combining skin fat biopsy with rectal mucosal biopsy may identify amyloid deposits in

  8. Generalised pustular psoriasis, psoriatic arthritis and nephrotic syndrome associated with systemic amyloidosis.

    Science.gov (United States)

    David, M; Abraham, D; Weinberger, A; Feuerman, E J

    1982-09-01

    The case report is presented of a psoriatic patient with arthropathy, generalised pustular psoriasis and nephrotic syndrome, in whom systemic amyloidosis developed. The literature reports 13 cases of psoriasis associated with amyloidosis, 3 of whom suffered from pustular psoriasis as does our case. With the addition of our case, 12 of these 14 had concomitant arthropathy. This seems to suggest that arthritis is an important factor in the appearance of amyloidosis. Rectal biopsy and/or renal biopsy may be helpful in establishing the diagnosis of amyloidosis relatively early in patients with psoriatic arthritis.

  9. An interesting case of renal amyloidosis

    Directory of Open Access Journals (Sweden)

    A Hajra

    2016-01-01

    Full Text Available In amyloidosis, there is an extracellular deposition of beta-sheet fibrils. Over 25 proteins have been demonstrated to form amyloid. One of them is Ig amyloid light (AL chains. We are presenting a 40-year-old female who presented with progressive kyphoscoliosis for last 2 years following a minor trauma and bilateral pedal edema for last 3 months. On further investigation, we found that she had a biclonal variety of MM with amyloidosis of kidney leading to massive proteinuria. Very few case reports are available where patient with biclonal variety of MM develop renal amyloidosis.

  10. Bone marrow amyloid spherulites in a case of AL amyloidosis.

    Science.gov (United States)

    Bommannan B K, Karthik; Sonai, Mukinkumar; Sachdeva, Man Updesh Singh

    2016-05-01

    Parallel arrangement of β-pleated sheets by amyloidogenic proteins is a well known phenomenon. Rarely, amyloid fibrils undergo radial orientation to form globular structures called spherulites. These amyloid spherulites show Maltese cross pattern under polarized microscopy. The clinical significance of amyloid spherulites is undetermined. Amyloidogenic proteins like insulin and β-lactoglobulin form spherulites in vitro. The senile plaques of Alzheimer's disease rarely form in vivo spherulites. Amyloid spherulites have been described in the liver and small intestine. For the first time, we document amyloid spherulite formation in the bone marrow biopsy of an AL amyloidosis patient. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Histological regression of amyloid in AL amyloidosis is exclusively seen after normalization of serum free light chain

    NARCIS (Netherlands)

    van Gameren, Ingrid I.; van Rijswijk, Martin H.; Bijzet, Johan; Vellenga, Edo; Hazenberg, Bouke P.

    Background Histological regression of amyloid has not been studied systematically but is assessed by clinical parameters. We analyzed the change of amyloid deposition in fat tissue in patients with AL amyloidosis following chemotherapy and studied the relation with type of hematologic response.

  12. Upper Gastrointestinal Bleeding from Gastric Amyloidosis in a Patient with Smoldering Multiple Myeloma

    Directory of Open Access Journals (Sweden)

    Mihajlo Gjeorgjievski

    2015-01-01

    Full Text Available Amyloidosis is a common complication of patients with monoclonal gammopathy of undetermined significance (MGUS, smoldering multiple myeloma (SMM, and multiple myeloma (MM. This proteinaceous material can be deposited intercellularly in any organ system, including the gastrointestinal (GI tract. In the GI tract, amyloidosis affects the duodenum most commonly, followed by the stomach and colorectum. Gastric amyloidosis causes symptoms of nausea, vomiting, early satiety, abdominal pain, and GI bleeding. A case of upper GI bleeding from gastric amyloidosis is presented in a patient with SMM. Esophagogastroduodenoscopy (EGD revealed a gastric mass. Endoscopic biopsies revealed amyloid deposition in the lamina propria, consistent with gastric amyloidosis. Liquid chromatography tandem mass spectrometry performed on peptides extracted from Congo red-positive microdissected areas of paraffin-embedded stomach specimens revealed a peptide profile consistent with AL- (lambda- type amyloidosis. Based on this and multiple other case reports, we recommend that patients with GI bleeding and MGUS, SMM, or MM undergo EGD and pathologic examination of endoscopic biopsies of identified lesions using Congo red stains for amyloidosis for early diagnosis and treatment.

  13. Thermal Stability Threshold for Amyloid Formation in Light Chain Amyloidosis

    Directory of Open Access Journals (Sweden)

    Tanya L. Poshusta

    2013-11-01

    Full Text Available Light chain (AL amyloidosis is a devastating disease characterized by amyloid deposits formed by immunoglobulin light chains. Current available treatments involve conventional chemotherapy and autologous stem cell transplant. We have recently concluded a phase III trial comparing these two treatments. AL amyloidosis patients who achieve hematological complete response (CR do not necessarily achieve organ response regardless of the treatment they received. In order to investigate the possible correlation between amyloid formation kinetics and organ response, we selected AL amyloidosis patients from the trial with kidney involvement and CR after treatment. Six patients were selected and their monoclonal immunoglobulin light chains were characterized. The proteins showed differences in their stability and their kinetics of amyloid formation. A correlation was detected at pH 7.4, showing that less stable proteins are more likely to form amyloid fibrils. AL-T03 is too unstable to form amyloid fibrils at pH 7.4. This protein was found in the only patient in the study that had organ response, suggesting that partially folded species are required for amyloid formation to occur in AL amyloidosis.

  14. Independent Prognostic Value of Stroke Volume Index in Patients With Immunoglobulin Light Chain Amyloidosis.

    Science.gov (United States)

    2018-05-01

    Heart involvement is the most important prognostic determinant in AL amyloidosis patients. Echocardiography is a cornerstone for the diagnosis and provides important prognostic information. We studied 754 patients with AL amyloidosis who underwent echocardiographic assessment at the Mayo Clinic, including a Doppler-derived measurement of stroke volume (SV) within 30 days of their diagnosis to explore the prognostic role of echocardiographic variables in the context of a well-established soluble cardiac biomarker staging system. Reproducibility of SV, myocardial contraction fraction, and left ventricular strain was assessed in a separate, yet comparable, study cohort of 150 patients from the Pavia Amyloidosis Center. The echocardiographic measures most predictive for overall survival were SV index <33 mL/min, myocardial contraction fraction <34%, and cardiac index <2.4 L/min/m 2 with respective hazard ratios (95% confidence intervals) of 2.95 (2.37-3.66), 2.36 (1.96-2.85), and 2.32 (1.91-2.80). For the subset that had left ventricular strain performed, the prognostic cut point was -14% (hazard ratios, 2.70; 95% confidence intervals, 1.84-3.96). Each parameter was independent of systolic blood pressure, Mayo staging system (NT-proBNP [N-terminal pro-B-type natriuretic peptide] and troponin), and ejection fraction on multivariable analysis. Simple predictive models for survival, including biomarker staging along with SV index or left ventricular strain, were generated. SV index prognostic performance was similar to left ventricular strain in predicting survival in AL amyloidosis, independently of biomarker staging. Because SV index is routinely calculated and widely available, it could serve as the preferred echocardiographic measure to predict outcomes in AL amyloidosis patients. © 2018 American Heart Association, Inc.

  15. Systemic AL amyloidosis with unusual cutaneous presentation unmasked by carotenoderma

    Czech Academy of Sciences Publication Activity Database

    Hůlková, H.; Vlášková, H.; Elleder, M.; Svojanovský, J.; Ševela, K.; Krusová, D.; Hanuš, J.; Souček, M.; Vězda, P.; Márová, I.; Feit, J.; Zambo, I.; Kovačevicova, M.; Kostrouchová, V.; Kostrouch, Z.; Novák, Petr

    2014-01-01

    Roč. 21, č. 5 (2014), s. 57-61 ISSN 1350-6129 R&D Projects: GA MŠk ED1.1.00/02.0109; GA MŠk(CZ) EE2.3.20.0055; GA MŠk LO1211; GA MŠk EE2.3.30.0003 Grant - others:Masaryk University, Brno(CZ) MUNI/A/1012/2009; GA MŠk(CZ) Prvouk-P27/LF1/1; Karlova Universita(CZ) UNCE 20422; Universita Karlova(CZ) UNCE 204011; Universita Karlova(CZ) PRVOUK-P24/LF1/3 Institutional support: RVO:61388971 Keywords : alzheimer * gene * amyloidosis Subject RIV: EC - Immunology Impact factor: 2.010, year: 2014

  16. Clinical diagnosis and typing of systemic amyloidosis in subcutaneous fat aspirates by mass spectrometry-based proteomics.

    Science.gov (United States)

    Vrana, Julie A; Theis, Jason D; Dasari, Surendra; Mereuta, Oana M; Dispenzieri, Angela; Zeldenrust, Steven R; Gertz, Morie A; Kurtin, Paul J; Grogg, Karen L; Dogan, Ahmet

    2014-07-01

    Examination of abdominal subcutaneous fat aspirates is a practical, sensitive and specific method for the diagnosis of systemic amyloidosis. Here we describe the development and implementation of a clinical assay using mass spectrometry-based proteomics to type amyloidosis in subcutaneous fat aspirates. First, we validated the assay comparing amyloid-positive (n=43) and -negative (n=26) subcutaneous fat aspirates. The assay classified amyloidosis with 88% sensitivity and 96% specificity. We then implemented the assay as a clinical test, and analyzed 366 amyloid-positive subcutaneous fat aspirates in a 4-year period as part of routine clinical care. The assay had a sensitivity of 90%, and diverse amyloid types, including immunoglobulin light chain (74%), transthyretin (13%), serum amyloid A (%1), gelsolin (1%), and lysozyme (1%), were identified. Using bioinformatics, we identified a universal amyloid proteome signature, which has high sensitivity and specificity for amyloidosis similar to that of Congo red staining. We curated proteome databases which included variant proteins associated with systemic amyloidosis, and identified clonotypic immunoglobulin variable gene usage in immunoglobulin light chain amyloidosis, and the variant peptides in hereditary transthyretin amyloidosis. In conclusion, mass spectrometry-based proteomic analysis of subcutaneous fat aspirates offers a powerful tool for the diagnosis and typing of systemic amyloidosis. The assay reveals the underlying pathogenesis by identifying variable gene usage in immunoglobulin light chains and the variant peptides in hereditary amyloidosis. Copyright© Ferrata Storti Foundation.

  17. 18F-fluorodeoxyglucose positron emission tomography might be useful for diagnosis of hepatic amyloidosis

    Directory of Open Access Journals (Sweden)

    Tawada A

    2014-06-01

    Full Text Available Akinobu Tawada,1 Tatsuo Kanda,1 Takashi Oide,2 Toshio Tsuyuguchi,1 Fumio Imazeki,1,3 Yukio Nakatani,2 Osamu Yokosuka11Department of Gastroenterology, 2Department of Diagnostic Pathology, Chiba University Hospital, Chuo-ku, Chiba, Japan; 3Safety and Health Organization, Chiba University, Inage-ku, Chiba, JapanAbstract: We report on a woman with hepatic involvement of primary systemic (immunoglobulin light chain, AL amyloidosis. Her diagnosis was confirmed by liver biopsy. Clinical symptoms of hepatic amyloidosis are generally mild at its first stage, with most frequent findings being hepatomegaly and alkaline phosphatase elevation. Recent advances in the understanding of the pathophysiology of systemic amyloidosis have made several treatments available. However, its prognosis is occasionally poor. Because liver biopsy is not always safe, other modalities for the diagnosis are needed. Of interest was that fluorodeoxyglucose (FDG uptake into the liver was observed, compared with that into the spleen, in this patient, indicating that FDG positron emission tomography and computed tomography might be useful for the diagnosis of hepatic amyloidosis with mild liver dysfunction.Keywords: amyloidosis, diagnosis, hepatic involvement, FDG PET

  18. Diagnostic studies in amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Bouke Pier Cornelis

    2007-01-01

    In this thesis two diagnostic techniques are studied in amyloidosis. Systemic amyloidosis is characterized by deposition of amyloid fibrils (tiny fibres) throughout the body resulting in damage of vital organs. Amyloid can be detected in a tissue specimen stained with Congo red: red-stained amyloid

  19. Increased Prognostic Value of Query Amyloid Late Enhancement Score in Light-Chain Cardiac Amyloidosis.

    Science.gov (United States)

    Wan, Ke; Sun, Jiayu; Han, Yuchi; Liu, Hong; Yang, Dan; Li, Weihao; Wang, Jie; Cheng, Wei; Zhang, Qing; Zeng, Zhi; Chen, Yucheng

    2018-02-23

    Late gadolinium enhancement (LGE) pattern is a powerful imaging biomarker for prognosis of cardiac amyloidosis. It is unknown if the query amyloid late enhancement (QALE) score in light-chain (AL) amyloidosis could provide increased prognostic value compared with LGE pattern.Methods and Results:Seventy-eight consecutive patients with AL amyloidosis underwent contrast-enhanced cardiovascular magnetic resonance imaging. Patients with cardiac involvement were grouped by LGE pattern and analyzed using QALE score. Receiver operating characteristic curve was used to identify the optimal cut-off for QALE score in predicting all-cause mortality. Survival of these patients was analyzed with the Kaplan-Meier method and multivariate Cox regression. During a median follow-up of 34 months, 53 of 78 patients died. The optimal cut-off for QALE score to predict mortality at 12-month follow-up was 9.0. On multivariate Cox analysis, QALE score ≥9 (HR, 5.997; 95% CI: 2.665-13.497; Pvalue in AL cardiac amyloidosis. QALE score ≥9 has added value to differentiate prognosis in AL amyloidosis patients with a subendocardial LGE pattern.

  20. Localized Lymph Node Light Chain Amyloidosis

    Directory of Open Access Journals (Sweden)

    Binod Dhakal

    2015-01-01

    Full Text Available Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and review the literature of lymph node amyloidosis discussing work-up and risk factors for systemic progression.

  1. Longitudinal left ventricular function for prediction of survival in systemic light-chain amyloidosis: incremental value compared with clinical and biochemical markers.

    Science.gov (United States)

    Buss, Sebastian J; Emami, Mostafa; Mereles, Derliz; Korosoglou, Grigorios; Kristen, Arnt V; Voss, Andreas; Schellberg, Dieter; Zugck, Christian; Galuschky, Christian; Giannitsis, Evangelos; Hegenbart, Ute; Ho, Anthony D; Katus, Hugo A; Schonland, Stefan O; Hardt, Stefan E

    2012-09-18

    The aim of the study was to determine whether longitudinal left ventricular (LV) function provides prognostic information in a large cohort of patients with systemic light-chain (AL) amyloidosis. AL amyloidosis is associated with a high incidence of cardiovascular events. Reduced myocardial longitudinal function is one of the hallmarks of myocardial involvement in this rare disease. Two hundred six consecutive patients with biopsy-proven AL amyloidosis were investigated in this prospective observational study. Echocardiographic imaging parameters, mean tissue Doppler-derived longitudinal strain (LS), and two-dimensional global longitudinal strain (2D-GLS) of the LV, cardiac serological biomarkers, and comprehensive clinical disease characteristics were assessed. The primary endpoint was all-cause mortality or heart transplantation. After a median follow-up of 1207 days, LS and 2D-GLS were significant predictors of survival in AL amyloidosis. The cutoff values discriminating survivors from nonsurvivors were -10.65% for LS and -11.78% for 2D-GLS. In a multivariable echocardiographic Cox model, only diastolic dysfunction and 2D-GLS remained as independent predictors of survival. In comprehensive clinical models, 2D-GLS (p < 0.0001), diastolic dysfunction (p < 0.01), the pathologic free light chains (p < 0.05), cardiac troponin-T (cTnT) (p < 0.01), and the Karnofsky index (p < 0.001) remained as independent predictors. 2D-GLS delineated a superior prognostic value compared with that derived from pathologic free light chains or cTnT in patients evaluated before firstline chemotherapy (n = 113; p < 0.0001), and remained the only independent predictor besides the Karnofsky index in subjects with preserved LV ejection fraction (≥50%; n = 127; p < 0.01). LS and 2D-GLS both offered significant incremental information (p < 0.001) for the assessment of outcome compared with clinical variables (age, Karnofsky index, and New York Heart Association functional class) and

  2. [AA amyloidosis: a little-known complication of chronic leg ulcer].

    Science.gov (United States)

    Waton, J; Fays-Michel, S; Chandeclerc, M L; Corby, S; Cuny, J F; Barbaud, A; Schmutz, J-L

    2008-02-01

    AA amyloidosis, secondary to inflammatory chronic diseases like rheumatoid arthritis, is often complicated by renal failure. Chronic inflammatory dermatoses constitute rare causes of AA amyloidosis. We describe two cases of AA amyloidosis discovered after renal failure in patients presenting leg ulcers for several years. AL amyloidosis was suspected in both cases because of a history of monoclonal gammopathy in one patient and of plasmocytoma in the other. The diagnosis of AA amyloidosis was confirmed on renal histology through the detection of AA antibodies in amyloid deposits. No extrarenal amyloidosis was seen in either patient and there were no inflammatory diseases other than chronic leg ulcers. AA amyloidosis is caused by serum amyloid protein A (SAA), a reactive inflammatory protein. AA amyloidosis is thus caused by chronic inflammatory diseases, but only rarely by cutaneous inflammatory diseases. To our knowledge, the literature contains only seven other published cases of AA amyloidosis secondary to chronic leg ulcers. A review of the literature does not indicate whether cure of ulcers has any effect on the accompanying renal failure. We imagine that AA amyloidosis secondary to leg ulcer is in fact under-diagnosed. However, since the first specific treatment for AA amyloidosis is currently being evaluated by the Food and Drug Administration, it is essential that this serious complication of chronic leg ulcers be widely recognised.

  3. Revisiting renal amyloidosis with clinicopathological characteristics, grading, and scoring: A single-institutional experience.

    Science.gov (United States)

    Kalle, Abhiram; Gudipati, Archana; Raju, Sree Bhushan; Kalidindi, Karthik; Guditi, Swarnalatha; Taduri, Gangadhar; Uppin, Megha S

    2018-01-01

    Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Renal biopsy helps in diagnosis and also predicting the clinical course by applying scoring and grading to the biopsy findings. The study includes all cases of biopsy-proven renal amyloidosis from January 2008 to May 2017. Light microscopic analysis; Congo red with polarization; IF; IHC for Amyloid A, kappa, and lambda; and bone marrow evaluation were done. Classification of glomerular amyloid deposition and scoring and grading are done as per the guidelines of Sen S et al . There are 40 cases of biopsy-proven renal amyloidosis with 12 primary and 23 secondary cases. Mean age at presentation was 42.5 years. Edema was the most common presenting feature. Secondary amyloidosis cases were predominant. Tuberculosis was the most common secondary cause. Multiple myeloma was detected in four primary cases. Grading of renal biopsy features showed a good correlation with the class of glomerular involvement. Clinical history, IF, and IHC are essential in amyloid typing. Grading helps provide a subtle guide regarding the severity of disease in the background of a wide range of morphological features and biochemical values. Typing of amyloid is also essential for choosing the appropriate treatment.

  4. Atypical rapid progression of osteoarticular amyloidosis involving the hip in a patient on hemodialysis using polyacrylonitrile membranes

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kenneth S. [University of Wisconsin School of Medicine and Public Health, Department of Radiology, University of Wisconsin Hospital and Clinics, Madison, WI (United States); Holsbeeck, Marnix T. van [Wayne State School of Medicine, Department of Radiology, Henry Ford Hospital, Detroit, MI (United States); Abbud, Alexander [Wayne State School of Medicine, Department of Pathology, Henry Ford Hospital, Detroit, MI (United States)

    2010-01-15

    Amyloidosis related to dialysis is a well-known complication affecting many organ systems, in particular the musculoskeletal system. In 1985 Shirahama et al. (Biochem Biophys Res Commun 53:705-709, 1985) identified beta-2 microglobulin (MG) as the offending constituent by using protein purification techniques. Amyloidosis has been increasing in prevalence because of longer life spans and increased chronic medical conditions such as end-stage renal disease. When dialysis-related amyloidosis involves the musculoskeletal system, it affects the shoulder girdle, the so called shoulder pad sign, the wrist, hip, knee, and spine (Resnick, Diagnosis of bone and joint disorders, 4th edn., pp. 2054-2058 and 2176-2183, 2002). Other osteoarticular manifestations of amyloidosis include osteoporosis, lytic lesions, and pathologic fractures. It has been well documented that the prevalence of amyloid is dependent on duration of dialysis - over 90% in patients on dialysis for over 7 years (Jadoul, Nephrol Dial Transplant 13:61-64, 1998). However, a recent changeover to high-flux membranes used in hemofiltration has been reported to delay its onset (Campistol et al., Contrib Nephrol 125:76-85, 1999). We report on the radiographic, nuclear medicine, and computed tomography (CT) findings of osteoarticular amyloidosis involving the hip, and sequence its atypical rapid onset. The imaging, histopathological findings, and differential diagnosis are discussed. (orig.)

  5. Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death.

    Science.gov (United States)

    Muchtar, Eli; Gertz, Morie A; Kumar, Shaji K; Lacy, Martha Q; Dingli, David; Buadi, Francis K; Grogan, Martha; Hayman, Suzanne R; Kapoor, Prashant; Leung, Nelson; Fonder, Amie; Hobbs, Miriam; Hwa, Yi Lisa; Gonsalves, Wilson; Warsame, Rahma; Kourelis, Taxiarchis V; Russell, Stephen; Lust, John A; Lin, Yi; Go, Ronald S; Zeldenrust, Steven; Kyle, Robert A; Rajkumar, S Vincent; Dispenzieri, Angela

    2017-04-13

    In light of major advances in immunoglobulin light chain (AL) amyloidosis, we evaluated the trends in presentation, management, and outcome among 1551 newly diagnosed AL amyloidosis patients seen in our institution from 2000 to 2014. As compared with the 2 intervals 2000-2004 and 2005-2009, patients diagnosed in 2010-2014 were less likely to have >2 involved organs. Utilization of autologous stem cell transplant (ASCT) was similar across all periods, about one-third of patients, but there was an increase in the use of pre-ASCT bortezomib induction and of unattenuated melphalan conditioning in 2010-2014 compared with earlier periods. Non-ASCT first-line regimen changed with 65% of patients in 2010-2014 received bortezomib-based therapy, 79% of patients in 2005-2009 received melphalan-dexamethasone, and 64% of patients in 2000-2004 received melphalan-prednisone. The rate of better than very good partial response (VGPR) was higher in more recent periods (66% vs 58% vs 51%; P = .001), a change largely driven by improved VGPR rates in the non-ASCT population. Overall survival (OS) has improved, with inflection points for improvement differing for the ASCT and non-ASCT groups. In the ASCT population, the greatest gains were after 2010 (4-year OS, 91% compared with 73% and 65%). In the non-ASCT group, greatest gains were after 2005 (4-year OS, 38%, 32%, and 16%). Fewer patients died within 6 months of diagnosis in the 2 later periods (24% vs 25% vs 37%; P < .001). Overall, outcomes among patients with AL amyloidosis have improved with earlier diagnosis, higher rates of VGPR, lower early mortality, and improved OS. © 2017 by The American Society of Hematology.

  6. Modified high-dose melphalan and autologous SCT for AL amyloidosis or high-risk myeloma: analysis of SWOG trial S0115.

    Science.gov (United States)

    Sanchorawala, V; Hoering, A; Seldin, D C; Finn, K T; Fennessey, S A; Sexton, R; Mattar, B; Safah, H F; Holmberg, L A; Dean, R M; Orlowski, R Z; Barlogie, B

    2013-11-01

    We designed a trial using two sequential cycles of modified high-dose melphalan at 100 mg/m(2) and autologous SCT (mHDM/SCT) in AL amyloidosis (light-chain amyloidosis, AL), AL with myeloma (ALM) and host-based high-risk myeloma (hM) patients through SWOG-0115. The primary objective was to evaluate OS. From 2004 to 2010, 93 eligible patients were enrolled at 17 centers in the United States (59 with AL, 9 with ALM and 25 with hM). The median OS for patients with AL and ALM was 68 months and 47 months, respectively, and has not been reached for patients with hM. The median PFS for patients with AL and ALM was 38 months and 16 months, respectively, and has not been reached for patients with hM. The treatment-related mortality (TRM) was 12% (11/93) and was observed only in patients with AL after SCT. Grade 3 and higher non-hematologic adverse events were experienced by 81%, 67% and 57% of patients with AL, ALM and hM, respectively, during the first and second HDM/SCT. This experience demonstrates that with careful selection of patients and use of mHDM for SCT in patients with AL, ALM and hM, even in the setting of a multicenter study, OS can be improved with acceptable TRM and morbidity.

  7. Amyloidosis involving the respiratory system: 5-year′s experience of a multi-disciplinary group′s activity

    Directory of Open Access Journals (Sweden)

    Raffaele Scala

    2015-01-01

    Full Text Available Amyloidosis may involve the respiratory system with different clinical-radiological-functional patterns which are not always easy to be recognized. A good level of knowledge of the disease, an active integration of the pulmonologist within a multidisciplinary setting and a high level of clinical suspicion are necessary for an early diagnosis of respiratory amyloidosis. The aim of this retrospective study was to evaluate the number and the patterns of amyloidosis involving the respiratory system. We searched the cases of amyloidosis among patients attending the multidisciplinary rare and diffuse lung disease outpatients′ clinic of Pulmonology Unit of the Hospital of Arezzo from 2007 to 2012. Among the 298 patients evaluated during the study period, we identified three cases of amyloidosis with involvement of the respiratory system, associated or not with other extra-thoracic localizations, whose diagnosis was histo-pathologically confirmed after the pulmonologist, the radiologist, and the pathologist evaluation. Our experience of a multidisciplinary team confirms that intra-thoracic amyloidosis is an uncommon disorder, representing 1.0% of the cases of rare and diffuse lung diseases referred to our center. The diagnosis of the disease is not always easy and quick as the amyloidosis may involve different parts of the respiratory system (airways, pleura, parenchyma. It is therefore recommended to remind this orphan disease in the differential diagnosis of the wide clinical scenarios the pulmonologist may intercept in clinical practice.

  8. Subtle neuropsychiatric and neurocognitive changes in hereditary gelsolin amyloidosis (AGel amyloidosis).

    Science.gov (United States)

    Kantanen, Mari; Kiuru-Enari, Sari; Salonen, Oili; Kaipainen, Markku; Hokkanen, Laura

    2014-01-01

    Hereditary gelsolin amyloidosis (AGel amyloidosis) is an autosomal dominant form of systemic amyloidosis caused by a c.640G>A or c.640G>T mutation in the gene coding for gelsolin. Principal clinical manifestations include corneal lattice dystrophy, cranial neuropathy and cutis laxa with vascular fragility. Signs of minor CNS involvement have also been observed, possibly related to cerebral amyloid angiopathy (CAA). To investigate further if AGel amyloidosis carries a risk for a specific neuropsychological or psychiatric symptomatology we studied 35 AGel patients and 29 control subjects. Neuropsychological tests showed abnormalities in visuocontructional and -spatial performance in AGel patients, also some indication of problems in processing efficacy was found. At psychiatric evaluation the patient group showed more psychiatric symptomatology, mainly depression. In brain MRI, available in 16 patients and 14 controls, we found microhemorrhages or microcalcifications only in the patient group, although the number of findings was small. Our study shows that AGel amyloidosis can be associated with visuoconstructional problems and depression, but severe neuropsychiatric involvement is not characteristic. The gelsolin mutation may even induce cerebrovascular fragility, but further epidemiological and histopathological as well as longitudinal follow-up studies are needed to clarify gelsolin-related vascular pathology and its clinical consequences.

  9. Subtle neuropsychiatric and neurocognitive changes in hereditary gelsolin amyloidosis (AGel amyloidosis

    Directory of Open Access Journals (Sweden)

    Mari Kantanen

    2014-07-01

    Full Text Available Hereditary gelsolin amyloidosis (AGel amyloidosis is an autosomal dominant form of systemic amyloidosis caused by a c.640G>A or c.640G>T mutation in the gene coding for gelsolin. Principal clinical manifestations include corneal lattice dystrophy, cranial neuropathy and cutis laxa with vascular fragility. Signs of minor CNS involvement have also been observed, possibly related to cerebral amyloid angiopathy (CAA. To investigate further if AGel amyloidosis carries a risk for a specific neuropsychological or psychiatric symptomatology we studied 35 AGel patients and 29 control subjects. Neuropsychological tests showed abnormalities in visuocontructional and -spatial performance in AGel patients, also some indication of problems in processing efficacy was found. At psychiatric evaluation the patient group showed more psychiatric symptomatology, mainly depression. In brain MRI, available in 16 patients and 14 controls, we found microhemorrhages or microcalcifications only in the patient group, although the number of findings was small. Our study shows that AGel amyloidosis can be associated with visuoconstructional problems and depression, but severe neuropsychiatric involvement is not characteristic. The gelsolin mutation may even induce cerebrovascular fragility, but further epidemiological and histopathological as well as longitudinal follow-up studies are needed to clarify gelsolin-related vascular pathology and its clinical consequences.

  10. An Incidental Finding of AL-associated Amyloidosis Presenting as Gastric Ulcers

    Directory of Open Access Journals (Sweden)

    Nadia Huq

    2018-01-01

    Full Text Available Gastrointestinal tract amyloidosis has been reported in rare instances and related symptoms are usually nonspecific to the disease process. We present a patient who initially had melena on anticoagulation and endoscopy revealed a bleeding gastric ulcer. Hemostasis was achieved. The patient had a recurrence of symptoms despite being off anticoagulation months later and at that time repeat endoscopy showed multiple gastric ulcers with surrounding friable mucosa. Biopsy results were significant for light chain associated-amyloidosis. This case represents a rare cause of gastric ulcer.

  11. Amyloidosis

    Science.gov (United States)

    ... is less common with modern dialysis techniques. Race. People of African descent appear to be at higher risk of carrying a genetic mutation associated with the type of amyloidosis that can harm the heart. Complications The potential complications of amyloidosis ...

  12. Laryngeal Amyloidosis Mimicking Glottic Cancer: A Case Report

    International Nuclear Information System (INIS)

    Lee, Sun Jin; Kim, Jee Young; Ahn, Kook Jin; Kim, Bum Soo; Park, Young Hak

    2010-01-01

    Amyloidosis is a slowly progressive, benign disease that is characterized by the extracellular deposition of fibrillar proteins in many different tissues and organs throughout the body. Primary amyloidosis can be subdivided into the systemic and localized forms. The localized form is less common than the systemic form and the larynx is the most frequently affected site. The importance of laryngeal amyloidosis lies in its possible confusion with glottic cancer because of the clinical feature. We report here on a case of laryngeal amyloidosis in a 47-year-old man who suffered from progressive dyspnea

  13. Laryngeal Amyloidosis Mimicking Glottic Cancer: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sun Jin; Kim, Jee Young; Ahn, Kook Jin; Kim, Bum Soo [The Catholic University of Korea, Seoul (Korea, Republic of); Park, Young Hak [St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2010-08-15

    Amyloidosis is a slowly progressive, benign disease that is characterized by the extracellular deposition of fibrillar proteins in many different tissues and organs throughout the body. Primary amyloidosis can be subdivided into the systemic and localized forms. The localized form is less common than the systemic form and the larynx is the most frequently affected site. The importance of laryngeal amyloidosis lies in its possible confusion with glottic cancer because of the clinical feature. We report here on a case of laryngeal amyloidosis in a 47-year-old man who suffered from progressive dyspnea.

  14. Cardiac amyloidosis detection with pyrophosphate-99mTc scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Souza, D.S.F.; Ichiki, W.A.; Coura Filho, G.B.; Izaki, M.; Giorgi, M.C.P.; Soares Junior, J; Meneghetti, J.C. [Universidade de Sao Paulo (FM/USP), SP (Brazil). Fac. de Medicina. Instituto do Coracao. Servico de Medicina Nuclear e Imagem Molecular

    2008-07-01

    Full text: Introduction: Amyloidosis is a rare disease, characterized by extracellular deposition of insoluble amyloid fibrils in organs and tissues. It may affect virtually any system, preferably heart, kidneys and liver. The cardiac involvement produces a spectrum of clinical features, usually with progressive dysfunction. Early diagnosis is important for institution of appropriate therapy. Case report: Male patient, 75 years old, with diagnosed congestive heart failure functional class III and Mobitz II second-degree atrial-ventricular block, was hospitalized for implantation of definitive cardiac pacemaker. Patient mentioned history of worsening effort dyspnoea over a one-month period, progressing to minimum effort, orthopnea, paroxysmal nocturnal dyspnoea and paroxysms of dry cough, and swelling of lower limbs. Echocardiography showed diffuse hypertrophy of left ventricle (LV), with systolic dysfunction due to diffuse hypokinesia and hyperrefringent aspect in the septum. It was questioned a cardiac infiltrating process. Cardiac amyloidosis was considered as a diagnostic hypothesis. The patient underwent a pyrophosphate-{sup 99m}Tc scintigraphy, which showed abnormal tracer uptake in the heart projection, with diffuse pattern on the left ventricle walls, compatible with the clinical suspicion cardiac amyloidosis, which was later confirmed by endomyocardial biopsy. Discussion: In this case report, the patient had clinical and other auxiliary examinations, such as electrocardiography and Doppler echocardiography, compatible with cardiac amyloidosis, which led to implementation with pyrophosphate-{sup 99m}Tc scintigraphy and later endomyocardial biopsy. Cardiac amyloidosis occurs in about half the cases of primary amyloidosis (AL) and is rare in secondary amyloidosis (AA). Its clinical presentation is polymorphic and it can be classified into four distinctive types: restrictive cardiomyopathy, systolic dysfunction, postural hypotension and conduction disorders

  15. Cardiac amyloidosis detection with pyrophosphate-99mTc scintigraphy

    International Nuclear Information System (INIS)

    Souza, D.S.F.; Ichiki, W.A.; Coura Filho, G.B.; Izaki, M.; Giorgi, M.C.P.; Soares Junior, J; Meneghetti, J.C.

    2008-01-01

    Full text: Introduction: Amyloidosis is a rare disease, characterized by extracellular deposition of insoluble amyloid fibrils in organs and tissues. It may affect virtually any system, preferably heart, kidneys and liver. The cardiac involvement produces a spectrum of clinical features, usually with progressive dysfunction. Early diagnosis is important for institution of appropriate therapy. Case report: Male patient, 75 years old, with diagnosed congestive heart failure functional class III and Mobitz II second-degree atrial-ventricular block, was hospitalized for implantation of definitive cardiac pacemaker. Patient mentioned history of worsening effort dyspnoea over a one-month period, progressing to minimum effort, orthopnea, paroxysmal nocturnal dyspnoea and paroxysms of dry cough, and swelling of lower limbs. Echocardiography showed diffuse hypertrophy of left ventricle (LV), with systolic dysfunction due to diffuse hypokinesia and hyperrefringent aspect in the septum. It was questioned a cardiac infiltrating process. Cardiac amyloidosis was considered as a diagnostic hypothesis. The patient underwent a pyrophosphate- 99m Tc scintigraphy, which showed abnormal tracer uptake in the heart projection, with diffuse pattern on the left ventricle walls, compatible with the clinical suspicion cardiac amyloidosis, which was later confirmed by endomyocardial biopsy. Discussion: In this case report, the patient had clinical and other auxiliary examinations, such as electrocardiography and Doppler echocardiography, compatible with cardiac amyloidosis, which led to implementation with pyrophosphate- 99m Tc scintigraphy and later endomyocardial biopsy. Cardiac amyloidosis occurs in about half the cases of primary amyloidosis (AL) and is rare in secondary amyloidosis (AA). Its clinical presentation is polymorphic and it can be classified into four distinctive types: restrictive cardiomyopathy, systolic dysfunction, postural hypotension and conduction disorders. Cardiac

  16. Prognosis of Light Chain Amyloidosis With Preserved LVEF: Added Value of 2D Speckle-Tracking Echocardiography to the Current Prognostic Staging System.

    Science.gov (United States)

    Barros-Gomes, Sergio; Williams, Brittney; Nhola, Lara F; Grogan, Martha; Maalouf, Joseph F; Dispenzieri, Angela; Pellikka, Patricia A; Villarraga, Hector R

    2017-04-01

    This study evaluated whether 2-dimensional speckle-tracking echocardiography (2D-STE) has incremental value for prognosis over traditional clinical, echocardiographic, and serological markers-with main focus on the current prognostic staging system-in light-chain (AL) amyloidosis patients with preserved left ventricular ejection fraction. Cardiac amyloidosis (CA) is the major determinant of outcome in AL amyloidosis. The current prognostic staging system is based primarily on serum levels of cardiac troponin T (cTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain differential (FLC-diff). Consecutive patients with biopsy-proven AL amyloidosis and left ventricular ejection fraction ≥55% were divided into group 1 with CA (n = 63) and group 2 without CA (n = 87). Global longitudinal strain (GLS) by 2D-STE was performed with Vivid E9 (GE Healthcare Co., Milwaukee, Wisconsin) and syngo Velocity Vector Imaging (VVI) software (Siemens Medical Solutions USA, Inc., Malvern, Pennsylvania) (GLS GE and GLS VVI , respectively). Thirty-two deaths (51%) occurred in group 1 and 13 (15%) in group 2 (p ≤ 0.001). Group 1 had thicker walls, lower early diastolic tissue Doppler velocity at septal mitral annulus, and greater left ventricular mass, left atrial volume, glomerular filtration rate, FLC-diff, cTnT, and NT-proBNP (p value over cTnT, NT-proBNP, and FLC-diff. For survival analysis limited to group 2 (non-CA), GLS GE and GLS VVI both predicted all-cause mortality (GLS GE HR: 1.23; 95% CI: 1.03 to 1.47 [p = 0.02]; GLS VVI HR: 1.22; 95% CI: 1.01 to 1.49 [p = 0.04], respectively). 2D-STE predicted outcome and provided incremental prognostic information over the current prognostic staging system, especially in the group without CA. Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  17. Amyloidosis of the renal pelvis presenting as flank pain

    Directory of Open Access Journals (Sweden)

    Rachel Shikhman, D.O.

    2018-02-01

    Full Text Available Amyloidosis is a rare disease defined by accumulation of extracellular amyloid systemically or within a specific organ. Localized amyloidosis of the genitourinary system is extremely rare, with the predominate location being the bladder. The imaging findings are often nonspecific and mimic urothelial carcinoma. We present a 49-year-old woman with a chief complaint of flank pain. A filling defect was discovered on radiological imaging. The defect was subsequently biopsied and proven to be a primary amyloidosis of the renal pelvis. We then review the radiological findings of amyloidosis of the genitourinary system.

  18. Kinetic studies with iodine-123-labeled serum amyloid P component in patients with systemic AA and AL amyloidosis and assessment of clinical value

    NARCIS (Netherlands)

    Jager, PL; Hazenberg, BPC; Franssen, EJF; Limburg, PC; van Rijswijk, MH; Piers, DA

    In systemic amyloidosis, widespread amyloid deposition interferes with organ function, frequently with fatal consequences. Diagnosis rests on demonstrating amyloid deposits in the tissues, traditionally with histology although scintigraphic imaging with radiolabeled serum amyloid P component (SAP)

  19. Light-chain amyloidosis presenting with rapidly progressive submucosal hemorrhage of the stomach

    Directory of Open Access Journals (Sweden)

    Song-Yi Kim

    2016-04-01

    Full Text Available The gastrointestinal tract is frequently in involved light-chain (AL amyloidosis, but significant hemorrhagic complications are rare. A 71-year-old man presented to our hospital with dyspepsia and heartburn for 1 month. Gastroscopy revealed a large submucosal hematoma at the gastric fundus. Two days later, a follow-up gastroscopy indicated extensive expansion of the hematoma throughout the upper half of the stomach. The hematoma displayed ongoing expansion during the endoscopic examination, suggesting that rupture was imminent. Emergency total gastrectomy was performed, and amyloidosis was confirmed after examining the surgical specimen. Bone marrow examination revealed multiple myeloma, and serum immunoglobulin assay confirmed the diagnosis of myeloma-associated AL amyloidosis. At manuscript submission, the patient was doing well and was undergoing chemotherapy.

  20. Iodine-123-labelled serum amyloid P component scintigraphy in amyloidosis

    International Nuclear Information System (INIS)

    Saile, R.; Deveaux, M.; Marchandise, X.; Duquesnoy, B.

    1993-01-01

    This study describes the results of scintigraphy with iodine-123-labelled serum amyloid P component (SAP) as a means of establishing the distribution of organ involvement in amyloidosis. The significance of 123 I-SAP scans obtained in 15 patients with biopsy-proven AA or AL amyloidosis is discussed. Biopsy-proven amyloidosis was typically confirmed by scintigraphy, though such confirmation was not obtained in the kidneys in six patients with histological proof of extensive renal amyloid deposition. This lack of uptake may have been due to the accumulation of a major part of the 123 I-SAP in the spleen and/or liver. Twenty-four hour whole-body retention of 123 I-SAP was higher in patients with amyloidosis than in controls. Twenty-four hour tracer accumulation of the radioactivity in the extravascular compartment was notably greater in patients than in controls and appeared to be a good diagnostic criterion. We conclude that 123 I-SAP scintigraphy may be helpful for the evaluation of organ involvement not only in patients with biopsy-proven amyloidosis but also when a biopsy cannot be performed or when a strong suspicion of amyloidosis exists in spite of repeated negative biopsises. (orig.)

  1. LGE Provides Incremental Prognostic Information Over Serum Biomarkers in AL Cardiac Amyloidosis.

    Science.gov (United States)

    Boynton, Samuel J; Geske, Jeffrey B; Dispenzieri, Angela; Syed, Imran S; Hanson, Theodore J; Grogan, Martha; Araoz, Philip A

    2016-06-01

    This study sought to determine the prognostic value of cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) in amyloid light chain (AL) cardiac amyloidosis. Cardiac involvement is the major determinant of mortality in AL amyloidosis. CMR LGE is a marker of amyloid infiltration of the myocardium. The purpose of this study was to evaluate retrospectively the prognostic value of CMR LGE for determining all-cause mortality in AL amyloidosis and to compare the prognostic power with the biomarker stage. Seventy-six patients with histologically proven AL amyloidosis underwent CMR LGE imaging. LGE was categorized as global, focal patchy, or none. Global LGE was considered present if it was visualized on LGE images or if the myocardium nulled before the blood pool on a cine multiple inversion time (TI) sequence. CMR morphologic and functional evaluation, echocardiographic diastolic evaluation, and cardiac biomarker staging were also performed. Subjects' charts were reviewed for all-cause mortality. Cox proportional hazards analysis was used to evaluate survival in univariate and multivariate analysis. There were 40 deaths, and the median study follow-up period was 34.4 months. Global LGE was associated with all-cause mortality in univariate analysis (hazard ratio = 2.93; p < 0.001). In multivariate modeling with biomarker stage, global LGE remained prognostic (hazard ratio = 2.43; p = 0.01). Diffuse LGE provides incremental prognosis over cardiac biomarker stage in patients with AL cardiac amyloidosis. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  2. {sup 123}I-Labelled metaiodobenzylguanidine for the evaluation of cardiac sympathetic denervation in early stage amyloidosis

    Energy Technology Data Exchange (ETDEWEB)

    Noordzij, Walter; Glaudemans, Andor W.J.M.; Rheenen, Ronald W.J. van; Dierckx, Rudi A.J.O.; Slart, Riemer H.J.A. [University of Groningen, Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, PO Box 30.001, Groningen (Netherlands); Hazenberg, Bouke P.C. [University of Groningen, Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, Groningen (Netherlands); Tio, Rene A. [University of Groningen, Department of Cardiology, University Medical Center Groningen, Groningen (Netherlands)

    2012-10-15

    Cardiac amyloidosis is a rare disorder, but it may lead to potentially life-threatening restrictive cardiomyopathy. Cardiac manifestations frequently occur in primary amyloidosis (AL) and familial amyloidosis (ATTR), but are uncommon in secondary amyloidosis (AA). Echocardiography is the method of choice for assessing cardiac amyloidosis. Amyloid deposits impair the function of sympathetic nerve endings. Disturbance of myocardial sympathetic innervations may play an important role in the remodelling process. {sup 123}I-MIBG can detect these innervation changes. Patients with biopsy-proven amyloidosis underwent general work-up, echocardiography and {sup 123}I-MIBG scintigraphy. Left ventricular internal dimensions and wall thickness were measured, and highly refractile cardiac echoes (sparkling) were analysed. Early (15 min) and late (4 h) heart-to-mediastinum ratio (HMR) and wash-out rate were determined after administration of MIBG. Included in the study were 61 patients (30 women and 31 men; mean age 62 years; 39 AL, 11 AA, 11 ATTR). Echocardiographic parameters were not significantly different between the groups. Sparkling was present in 72 % of ATTR patients, in 54 % of AL patients and in 45 % of AA patients. Mean late HMR in all patients was 2.3 {+-} 0.75, and the mean wash-out rate was 8.6 {+-} 14 % (the latter not significantly different between the patient groups). Late HMR was significantly lower in patients with echocardiographic signs of amyloidosis than in patients without (2.0 {+-} 0.70 versus 2.8 {+-} 0.58, p < 0.001). Wash-out rates were significantly higher in these patients (-3.3 {+-} 9.9 % vs. 17 {+-} 10 %, p < 0.001). In ATTR patients without echocardiographic signs of amyloidosis, HMR was lower than in patients with the other types (2.0 {+-} 0.59 vs. 2.9 {+-} 0.50, p = 0.007). MIBG HMR is lower and wash-out rate is higher in patients with echocardiographic signs of amyloidosis. Also, {sup 123}I-MIBG scintigraphy can detect cardiac denervation in

  3. Systemic amyloidosis due to unknown multiple myeloma in small bowel pseudo-obstruction: case report

    Directory of Open Access Journals (Sweden)

    Giuseppe Caparrotti

    2016-03-01

    Full Text Available Amyloidosis is a pathologic diagnosis characterized by extracellular deposition of insoluble protein fibrils in various organs and tissues. There are two main forms of amyloidosis, primary amyloidosis, and secondary amyloidosis. Gastrointestinal involvement is common in both amyloidosis forms. We describe the case of a 78-year-old woman taken to the operating room for small bowel obstruction, found to have pseudo-obstruction and enteritis. Exploratory laparotomy revealed gastric mass and histological examen showed extensive amyloid deposition consistent with amyloidosis. Hematological evaluation revealed unknown multiple myeloma. This case report and literature data suggest to perform a hematological examination in patients with amyloidosis diagnosis to exclude a multiple myeloma or other plasma cell disorders

  4. The Hidden Cost of Untreated Paragangliomas of the Head and Neck: Systemic Reactive (AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    Erkan Dervisoglu

    2015-01-01

    Full Text Available We report a case of a 51-year-old man who was diagnosed with systemic reactive (AA amyloidosis in association with untreated glomus jugulare and glomus caroticum tumors. He refused radiotherapy and renal replacement therapy. Paragangliomas, although rare, should be considered one of the tumors that can result in AA amyloidosis.

  5. Diagnostic accuracy of subcutaneous abdominal fat tissue aspiration for detecting systemic amyloidosis and its utility in clinical practice

    NARCIS (Netherlands)

    van Gameren, Ingrid I.; Hazenberg, BPC; Bijzet, J.; van Rijswijk, M.H.

    Objective. Aspiration of subcutaneous abdominal fat is a simple and fast method for detecting systemic amyloidosis; however, the sensitivity of this approach remains undetermined. The aim of this study was to assess the accuracy of fat tissue aspiration for detecting systemic amyloidosis and the

  6. Lichen amyloidosis in an unusual location.

    Science.gov (United States)

    Jhingan, A; Lee, J S S; Kumarasinghe, S P W

    2007-06-01

    We report lichen amyloidosis occurring on the upper lip and nasolabial folds of a 61-year-old woman from Singapore. She had a past history of systemic lupus erythematosus, which was in remission for three years. There had been no lesions of lupus erythematosus in this area. Clinically, the lesions were skin-coloured, firm papules and our differential diagnoses included trichoepithelioma, papular sarcoid or lupus miliaris disseminatus faciei. Skin biopsy from one of the lesions showed amyloid deposits in the dermis which were Congo red stain positive. These deposits also showed apple green birefringence. Immunohistochemical staining of the amyloid deposits stained positive for cytokeratins (CK) 5 and 6, and negative for CK 14. The kappa and lambda stains were equivocal. Further investigations, including multiple myeloma screen and rectal biopsy, ruled out systemic amyloidosis. There was no other evidence of cutaneous amyloidosis on her limbs or trunk. She refused treatment for her lesions. This case highlights the commonly-seen form of primary localised cutaneous amyloidosis in an unusual location.

  7. Primary localised cutaneous amyloidosis - a systematic review

    DEFF Research Database (Denmark)

    Kaltoft, Britta; Schmidt, Grethe; Lauritzen, Anne Falensteen

    2013-01-01

    Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the literature and to evaluate the risk of developing systemic amyloidosis (SA) and the risk of local recurrence of primary localised...

  8. Thoracic and abdominal SPECT imaging in systemic amyloidosis in identifying multiorgan involvement

    International Nuclear Information System (INIS)

    Wellman, H.N.; Benson, M.D.; Park, H.M.; Siddiqui, A.R.; Krepshaw, J.D.

    1988-01-01

    Thirty-three patients with systemic amyloidosis have been studied. Thoracic single photon emission computed tomography (SPECT) for myocardial involvement and skeletal imaging were performed with Tc-99m PYP, and abdominal SPECT with TcS colloid. Myocardial wall involvement was easily discernible with SPECT in 17 cases, and in many with normal ultrasonography. PYP uptake was also observed in liver (five patients), kidneys (four patients), and soft tissues (two patients). Most patients had widespread degenerative joint disease. With TcS colloid, intrinsic liver abnormalities were found in four patients, hepatomegaly in seven, and splenic infiltration in two. Nuclear SPECT and planar imaging characterize the distribution of systemic amyloidosis in organs, distribution not readily identified with other diagnostic modalities

  9. The Prevalence and Management of Systemic Amyloidosis in Western Countries

    NARCIS (Netherlands)

    Nienhuis, Hans L A; Bijzet, Johan; Hazenberg, Bouke P C

    BACKGROUND: Amyloidosis has been a mystery for centuries, but research of the last decennia has clarified many of the secrets of this group of diseases. A protein-based classification of amyloidosis helps to understand problems that were part of the obsolete clinical classification in primary,

  10. Systemic AA amyloidosis in the red fox (Vulpes vulpes).

    Science.gov (United States)

    Rising, Anna; Cederlund, Ella; Palmberg, Carina; Uhlhorn, Henrik; Gaunitz, Stefan; Nordling, Kerstin; Ågren, Erik; Ihse, Elisabet; Westermark, Gunilla T; Tjernberg, Lars; Jörnvall, Hans; Johansson, Jan; Westermark, Per

    2017-11-01

    Amyloid A (AA) amyloidosis occurs spontaneously in many mammals and birds, but the prevalence varies considerably among different species, and even among subgroups of the same species. The Blue fox and the Gray fox seem to be resistant to the development of AA amyloidosis, while Island foxes have a high prevalence of the disease. Herein, we report on the identification of AA amyloidosis in the Red fox (Vulpes vulpes). Edman degradation and tandem MS analysis of proteolyzed amyloid protein revealed that the amyloid partly was composed of full-length SAA. Its amino acid sequence was determined and found to consist of 111 amino acid residues. Based on inter-species sequence comparisons we found four residue exchanges (Ser31, Lys63, Leu71, Lys72) between the Red and Blue fox SAAs. Lys63 seems unique to the Red fox SAA. We found no obvious explanation to how these exchanges might correlate with the reported differences in SAA amyloidogenicity. Furthermore, in contrast to fibrils from many other mammalian species, the isolated amyloid fibrils from Red fox did not seed AA amyloidosis in a mouse model. © 2017 The Protein Society.

  11. Nodular immunocyte-derived (AL) amyloidosis in the trachea of a dog.

    Science.gov (United States)

    Besancon, M Faulkner; Stacy, Brian A; Kyles, Andrew E; Moore, Peter F; Vernau, William; Smarick, Sean D; Rasor, Liberty A

    2004-04-15

    A 7-year-old castrated male Miniature Schnauzer was examined because of labored breathing and episodes of respiratory distress that progressed to collapse. On cervical radiographs, a focal soft tissue mass in the caudal cervical portion of the trachea was observed, and during tracheoscopy, a 1 x 1 cm, pedunculated, multinodular, pink, intraluminal mass extending from the dorsal tracheal membrane and obstructing approximately 80% of the tracheal lumen was seen. Tracheal resection and anastomosis was performed to remove the mass, and the dog recovered without complications. On histologic examination, the mass consisted of a large accumulation of homogeneous, faintly fibrillar eosinophilic material admixed with a predominantly plasma cell infiltrate; examination of sections stained with thioflavin T and Congo red stain confirmed that the eosinophilic material was amyloid. A diagnosis of nodular, immunocyte-derived (AL) amyloidosis was made. Seventeen months after surgery, the dog had a relapse of respiratory distress because of an extramedullary plasmacytoma involving the trachea.

  12. High-dose melphalan and autologous stem cell transplantation for AL amyloidosis: recent trends in treatment-related mortality and 1-year survival at a single institution

    Science.gov (United States)

    Seldin, D. C.; Andrea, N.; Berenbaum, I.; Berk, J. L.; Connors, L.; Dember, L. M.; Doros, G.; Fennessey, S.; Finn, K.; Girnius, S.; Lerner, A.; Libbey, C.; Meier-Ewert, H. K.; O’Connell, R.; O’Hara, C.; Quillen, K.; Ruberg, F. L.; Sam, F.; Segal, A.; Shelton, A.; Skinner, M.; Sloan, J. M.; Wiesman, J. F.; Sanchorawala, V.

    2017-01-01

    Treatment with high-dose melphalan chemotherapy supported by hematopoietic rescue with autologous stem cells produces high rates of hematologic responses and improvement in survival and organ function for patients with AL amyloidosis. Ongoing clinical trials explore pre-transplant induction regimens, post-transplant consolidation or maintenance approaches, and compare transplant to non-transplant regimens. To put these studies into context, we reviewed our recent experience with transplant for AL amyloidosis in the Amyloid Treatment and Research Program at Boston Medical Center and Boston University School of Medicine. Over the past 10 years, there was a steady reduction in rates of treatment-related mortality and improvement in 1-year survival, now approximately 5% and 90%, respectively, based upon an intention-to-treat analysis. Median overall survival of patients treated with this approach at our center exceeds 7.5 years. PMID:21838459

  13. MRI in cardiac sarcoidosis and amyloidosis; MRT bei kardialer Sarkoidose und Amyloidose

    Energy Technology Data Exchange (ETDEWEB)

    Bauner, K.U. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Wintersperger, B. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); University of Toronto, Department of Medical Imaging, Toronto General Hospital, Toronto, ON (Canada)

    2013-01-15

    Sarcoidosis and amyloidosis are both multisystem disorders, which may involve the heart; however, isolated cardiac disease is rare. Diagnosis of cardiac sarcoidosis and amyloidosis is crucial because the patient prognosis is dependent on cardiac involvement and early treatment. Echocardiography is the first line imaging modality in the diagnostic work-up of both diseases, possibly giving hints towards the correct diagnosis. Besides myocardial biopsy and radionuclide studies cardiac magnetic resonance imaging (MRI) is routinely performed in patients suspect of having infiltrative cardiomyopathy. The T1 mapping procedure is currently being evaluated as a new technique for detection and quantification of global myocardial enhancement, as seen in cardiac amyloidosis. Sensitivities and specificities for detection of cardiac sarcoidosis and amyloidosis can be significantly improved by MRI, especially with late gadolinium enhancement (LGE) imaging. In cardiac sarcoidosis the use of LGE is outcome-related while in amyloidosis analysis of T1-mapping may be of prognostic value. If cardiac involvement in sarcoidosis or amyloidosis is suspected cardiac MRI including LGE should be performed for establishing the diagnosis. (orig.) [German] Die Sarkoidose und Amyloidose sind Multisystemerkrankungen, in deren Verlauf es zu einer kardialen Beteiligung kommen kann. Bildgebend wird als primaeres Verfahren die Echokardiographie eingesetzt. Zur weiteren Diagnostik wird neben der Biopsie und nuklearmedizinischen Verfahren v. a. die MRT herangezogen. Als neuere Technik zur Darstellung globaler diffuser Kontrastmittelanreicherungen, wie sie im Rahmen der Amyloidose vorkommen, wird z. Z. das T1-Mapping evaluiert. Durch den Einsatz der MRT, insbesondere des Late-Gadolinium-Enhancements (LGE), koennen die Sensitivitaet und Spezifitaet in der Diagnostik der kardialen Sarkoidose und Amyloidose entscheidend verbessert werden. Bei der Sarkoidose stellt das Vorhandensein eines LGE einen

  14. Magnetic resonance imaging with liver-specific contrast agent in primary amyloidosis and intrahepatic cholestasis

    Energy Technology Data Exchange (ETDEWEB)

    Moeller, J.M.; Santoni-Rugiu, E.; Chabanova, E.; Logager, V.; Hansen, A.B.; Thomsen, H.S. [Depts. of Radiology and Pathology, Copenhagen Univ. Hospital, Herlev (Denmark)

    2007-02-15

    Magnetic resonance imaging (MRI) findings in hepatic amyloidosis are not well defined. Here, we report on a patient with renal failure caused by primary amyloidosis (AL type) who developed jaundice. Ultrasound and computed tomography were normal except for some ascites. MRI with oral manganese-containing contrast agent revealed several focal areas without contrast uptake in the hepatocytes and no bile secretion after 8 hours. No extrahepatic bile obstructions were found. Liver biopsy showed severe intraportal, vascular, and parenchymal amyloidosis causing severe cholestasis and atrophy of hepatocytes.

  15. Amyloidosis diagnosed in cytology specimen of pleural effusion: A case report.

    Science.gov (United States)

    Manur, Rashmi; Lamzabi, Ihab

    2018-06-01

    Amyloidosis is a rare condition resulting from extracellular deposition of amyloid, a fibrillary material derived from various precursor proteins. Involvement of the pleura by amyloidosis is a rare but serious complication. Pleural amyloidosis is primarily diagnosed by identifying amyloid deposition by histology on pleural biopsy specimens. Hereby, we report a case of systemic amyloidosis where we were able to identify amyloid in a pleural effusion specimen sent for cytopathology evaluation. A 59-year-old male with newly diagnosed multiple myeloma and systemic amyloidosis underwent therapeutic thoracentesis. The H&E stained cell block sections revealed a single, less than one millimeter focus of waxy material surrounded by a rim of reactive mesothelial cells suspicious for amyloid deposit in a background of fibrin, lymphocytes, and reactive mesothelial cells. The focus stained salmon pink with Congo-red special stain and showed apple-green birefringence under polarized light. Our finding suggests that pleural involvement in patients with systemic amyloidosis can be identified on effusion specimens and avert the need for more invasive procedures like pleural or pulmonary parenchymal biopsies. © 2017 Wiley Periodicals, Inc.

  16. Diagnostic performance and prognostic value of extravascular retention of I-123-labeled serum amyloid P component in systemic amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Bouke P. C.; van Rijswijk, Martin H.; Lub-de Hooge, Marjolijn N.; Vellenga, Edo; Haagsma, Elizabeth B.; Posthumus, Marcel D.; Jager, Pieter L.

    Serum amyloid P component (SAP) binds to amyloid.I-123-SAP scintigraphy is used to evaluate the extent and distribution of amyloid in systemic amyloidosis and has great clinical value in the detection of systemic amyloidosis. The aim of the study was to assess during scintigraphy the diagnostic

  17. Significant association between renal function and amyloid-positive area in renal biopsy specimens in AL amyloidosis

    Directory of Open Access Journals (Sweden)

    Kuroda Takeshi

    2012-09-01

    Full Text Available Abstract Background The kidney is a major target organ for systemic amyloidosis that often affects the kidney including proteinura, and elevated serum creatinine (Cr. The correlation between amount of amyloid deposits and clinical parameters is not known. The aim of this study was to clarify correlation the amyloid area in all renal biopsy specimen and clinical parameters. Methods Fifty-eight patients with an established diagnosis of AL amyloidosis participated in the study. All patients showed amyloid deposits in renal biopsies. We retrospectively investigated the correlation between clinical data and amyloid occupied area in whole renal biopsy specimens. Results The area occupied by amyloid was less than 10% in 57 of the 58 patients, and was under 2% in 40. For statistical analyses, %amyloid-positive areas were transformed to common logarithmic values (Log10%amyloid. Cr showed significant correlation with Log10%amyloid and estimated glomerular filtration rate (eGFR showed the significant negative correlation. Patient age, cleatinine clearance (Ccr, blood urea nitorogen, and urinary protein was not significantly correlated with Log10%amyloid. The correlation with other clinical factors such as sex, and serum concentrations of total protein, albumin, immunoglobulins, compliments was evaluated. None of these factors significantly correlated with Log10%amyloid. According to sex- and age- adjusted multiple linear regression analysis, Log10%amyloid had significant positive association with Cr and significant negative association with eGFR. Conclusion There is significant association between amyloid-positive area in renal tissue and renal function, especially Cr and eGFR. The level of Cr and eGFR may be a marker of amount of amyloid in renal tissue.

  18. Renal failure due to primary amyloidosis: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Ramon Andrade Bezerra de Mello

    Full Text Available CONTEXT: Primary amyloidosis, also known as AL amyloidosis, is commonly caused by clonal expansion of plasma cells in the bone marrow, thereby segregating light chains of clonal immunoglobulin that settle in tissues in the form of insoluble amyloid fibrils. The aim of this study was to report a case of primary amyloidosis with renal failure, diagnosed in Hospital São João, Porto, Portugal, focusing on the diagnostic difficulties and presenting a literature review. CASE REPORT: A 68-year-old Caucasian man was admitted to the Internal Medicine Department of the hospital with a condition of anasarca and nephrotic syndrome. After performing a renal biopsy that tested positive using Congo red and immunohistochemistry, lambda light chain amyloidosis was diagnosed. This evolved into terminal renal disease, which led to hemodialysis and several episodes of urinary and catheter infections. He was started on chemotherapy, consisting of bortezomib 0.7 mg/m² and dexamethasone 40 mg in six cycles. This led to clinical improvement, stabilization of the illness and good tolerance of the treatment. CONCLUSION: Amyloidosis is a rare entity that is difficult to diagnose. This is because of the unspecific early clinical manifestations of the disease. The hypothesis of amyloidosis is only considered when specific organ failure occurs. This case consisted of primary amyloidosis with involvement of the kidneys as an initial presentation of the disease and its difficulties were shown, going from the clinical approach to the final diagnosis.

  19. Intensity-modulated radiotherapy for localized nasopharyngeal amyloidosis. Case report and literature review

    International Nuclear Information System (INIS)

    Luo, Ming; Peng, Gang; Shi, Liangliang; Li, Zhenyu; Fei, Shijiang; Ding, Qian; Cheng, Jing; Ming, Xing

    2016-01-01

    Primary localized amyloidosis is characterized by the deposition of amyloid proteins restricted to one organ, without systemic involvement. Primary nasopharyngeal amyloidosis is an exceedingly rare condition, for which the standard treatment remains unknown. Because of its challenging anatomical position, surgery alone hardly results in complete resection of the localized amyloidosis. Therefore, an interdisciplinary planning board to design optimal treatment is of particular importance. A 39-year-old man presented with a several-week history of nasal obstruction and epistaxis. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed the presence of a retro-odontoid nonenhancing soft tissue mass. The endoscopic biopsy demonstrated that the mass was amyloid in nature. An extensive systemic workup revealed an absence of inflammatory process, systemic amyloidosis, or plasma cell dyscrasia. The patient was treated with a combination of surgery and radiotherapy, showing no evidence of recurrence or progression at his 1-year follow-up. Primary solitary amyloidosis is a rare form of amyloidosis. To the best of our knowledge, this is the first report of a nasopharyngeal amyloidosis case treated with excision and radiation leading to complete remission. Because of the difficulty for surgeons to achieve radical resection with such lesions, radiotherapy proved to be an excellent adjuvant treatment in this case. (orig.) [de

  20. Localized gastrointestinal amyloidosis presenting with protein-losing enteropathy and massive hemorrhage

    Directory of Open Access Journals (Sweden)

    Bárbara Corrêa

    Full Text Available Amyloidosis of the gastrointestinal tract is usually a systemic disease. Localized gastrointestinal amyloidosis without evidence of extraintestinal involvement or an associated plasma cell dyscrasia is uncommon and does not usually cause death. We report a case of a patient with localized gastrointestinal amyloidosis who presented with protein-losing enteropathy and a fatal upper gastrointestinal bleed.

  1. Intensity-modulated radiotherapy for localized nasopharyngeal amyloidosis. Case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Luo, Ming [Hubei University of Medicine, Department of Clinical Oncology, Taihe Hospital, Shiyan, Hubei (China); Peng, Gang; Shi, Liangliang; Li, Zhenyu; Fei, Shijiang; Ding, Qian; Cheng, Jing [HuaZhong University of Science and Technology, Cancer Center, Union Hospital, Tongji Medical College, Wuhan (China); Ming, Xing [Hubei University of Medicine, Department of infection control and prevention, Taihe Hospital, Shiyan, Hubei (China)

    2016-12-15

    Primary localized amyloidosis is characterized by the deposition of amyloid proteins restricted to one organ, without systemic involvement. Primary nasopharyngeal amyloidosis is an exceedingly rare condition, for which the standard treatment remains unknown. Because of its challenging anatomical position, surgery alone hardly results in complete resection of the localized amyloidosis. Therefore, an interdisciplinary planning board to design optimal treatment is of particular importance. A 39-year-old man presented with a several-week history of nasal obstruction and epistaxis. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed the presence of a retro-odontoid nonenhancing soft tissue mass. The endoscopic biopsy demonstrated that the mass was amyloid in nature. An extensive systemic workup revealed an absence of inflammatory process, systemic amyloidosis, or plasma cell dyscrasia. The patient was treated with a combination of surgery and radiotherapy, showing no evidence of recurrence or progression at his 1-year follow-up. Primary solitary amyloidosis is a rare form of amyloidosis. To the best of our knowledge, this is the first report of a nasopharyngeal amyloidosis case treated with excision and radiation leading to complete remission. Because of the difficulty for surgeons to achieve radical resection with such lesions, radiotherapy proved to be an excellent adjuvant treatment in this case. (orig.) [German] Die primaere lokalisierte Amyloidose ist durch die Ablagerung von Amyloidproteinen gekennzeichnet, die sich auf ein Organ beschraenkt, also nicht systemisch ist. Eine primaere Amyloidose im Nasen-Rachen-Raum ist ausserordentlich selten, bisher gibt es keine Standardtherapie. Ihre anatomische Position bedeutet eine Herausforderung, nur selten resultiert eine chirurgische Intervention in einer vollstaendigen Resektion der lokalisierten Amyloidose. Daher ist die Beteiligung mehrerer Disziplinen fuer eine optimale Behandlung von besonderer

  2. Amyloidosis and Kidney Disease

    Science.gov (United States)

    ... a fist. They are located just below the rib cage, one on each side of the spine. ... by dialysis-related amyloidosis can lead to bone fractures. Dialysis-related amyloidosis can also cause tears in ...

  3. SECONDARY AMYLOIDOSIS WITH LUNG INVOLVEMENT INA FEMALE PATIENT WITH RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Irina Mikhailovna Marusenko

    2009-01-01

    Full Text Available The paper considers the problem of secondary amyloidosis that more frequently occurs in patients with various arthritides, both seropositive and seronegative. According to the data available in the literature, the most common manifestations of secondary amyloidosis are involvements of the kidney, liver, nervous system, and, less frequently, the lung. The authors describe their own observation of secondary amyloidosis in rheumatoid arthritis, which is accompanied by the involvement of the lung, kidney, and intestine, resulting in fatal outcome. The lifetime diagnosis of amyloidosis was histologically verified.

  4. Localized gastric amyloidosis differentiated histologically from scirrhous gastric cancer using endoscopic mucosal resection: a case report

    Directory of Open Access Journals (Sweden)

    Kamata Tsugumasa

    2012-08-01

    Full Text Available Abstract Introduction Amyloidosis most often manifests as a systemic involvement of multiple tissues and organs, and an amyloidal deposit confined to the stomach is extremely rare. It is sometimes difficult to provide a definitive diagnosis of localized gastric amyloidosis by biopsy specimen and diagnosis of amyloidosis in some cases has been finalized only after surgical resection of the stomach. Case presentation A 76-year-old Japanese woman with epigastric discomfort underwent an esophagogastroduodenoscopy procedure. The esophagogastroduodenoscopy revealed gastric wall thickening, suggesting scirrhous gastric carcinoma, at the greater curvature from the upper to the lower part of the gastric corpus. A biopsy specimen revealed amyloid deposits in the submucosal layer with no malignant findings. We resected a representative portion of the lesion by endoscopic mucosal resection using the strip biopsy method to obtain sufficient tissue specimens, and then conducted a detailed histological evaluation of the samples. The resected specimens revealed deposition of amyloidal materials in the gastric mucosa and submucosa without any malignant findings. Congo red staining results were positive for amyloidal protein and exhibited green birefringence under polarized light. Congo red staining with prior potassium permanganate incubation confirmed the light chain (AL amyloid protein type. Based on these results, gastric malignancy, systemic amyloidosis and amyloid deposits induced by inflammatory disease were excluded and this lesion was consequently diagnosed as localized gastric amyloidosis. Our patient was an older woman and there were no findings relative to an increase in gastrointestinal symptoms or anemia, so no further treatment was performed. She continued to be in good condition without any finding of disease progression six years after verification of our diagnosis. Conclusions We report an unusual case of primary amyloidosis of the stomach

  5. Three cases of systemic amyloidosis successfully diagnosed by subcutaneous fat tissue biopsy of the hip

    Directory of Open Access Journals (Sweden)

    Arahata M

    2016-08-01

    Full Text Available Masahisa Arahata,1 Shigeru Shimadoi,1 Satosi Yamatani,1 Shin-ichi Hayashi,2 Shigeharu Miwa,2 Hidesaku Asakura,3 Shinji Nakao4 1Department of Internal Medicine, Nanto Municipal Hospital, Nanto, 2Department of Diagnostic Pathology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, 3Department of Internal Medicine (III, 4Department of Cellular Transplantation Biology, Division of Cancer Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan Abstract: Fine-needle aspiration biopsy of the abdominal fat pad is considered to be a minimally invasive procedure for diagnosing systemic amyloidosis. However, this procedure is sometimes difficult and can be dangerous for elderly patients whose abdominal fat layer is thin because of malnutrition. In such cases, alternative diagnostic methods are required. We report three elderly patients with heart failure complicated by malnutrition. In all cases, electrocardiogram showed low voltage in the limb leads and a pseudoinfarct pattern in the chest leads, and echocardiography showed left ventricular wall thickening with granular sparkling appearance. These patients were suspected of having amyloid cardiomyopathy but could not undergo myocardial biopsies because of their poor conditions. After failed attempts at biopsy of the abdominal fat pad or the other organs, subcutaneous fat tissue biopsy over the hip led to the diagnosis of systemic amyloidosis with cardiomyopathy. The resultant diagnosis guided us to choose the appropriate treatment for the patients. This article illustrates that subcutaneous fat tissue biopsy of the hip could be a useful procedure for diagnosing systemic amyloidosis in elderly patients, particularly when a fat tissue biopsy of the abdomen is associated with a high risk of complications because of malnutrition. Keywords: systemic amyloidosis, amyloid cardiomyopathy, fine-needle aspiration biopsy, subcutaneous fat tissue, hip

  6. Isolated Light Chain Amyloidosis Involving the Parotid Gland: A Case Report.

    Science.gov (United States)

    Gareb, Barzi; Perry, Michael; Tadrous, Paul Joseph

    2018-03-08

    Amyloidosis in the parotid gland is rare and is usually associated with systemic amyloidosis. Localized amyloidosis in the parotid gland is extremely rare. We present a case of localized light chain amyloidosis of the parotid gland without systemic involvement. A 70-year-old woman presented with an asymptomatic swelling of the right parotid region. The findings of a physical examination, hematologic and biochemical investigations, imaging, and cytology were inconclusive. The patient underwent an extracapsular dissection of the right parotid gland. Histologic analysis showed that the tissue of the right parotid gland mostly consisted of amyloid deposition. The amyloid stained with antibodies to lambda light chains. Additional investigations showed no systemic involvement. The patient is asymptomatic 5 months after surgery. Clinicians should be aware of the possibility of localized amyloid light chain amyloidosis in the parotid gland, especially if magnetic resonance imaging, computed tomography imaging, and ultrasound findings are inconclusive, and they should recognize, evaluate, and treat it accordingly. Copyright © 2018 American Association of Oral and Maxillofacial Surgeons. All rights reserved.

  7. Dysphagia as initial presentation of primary amyloidosis

    Directory of Open Access Journals (Sweden)

    Piyush Ranjan

    2013-01-01

    Full Text Available Amyloidosis involves all parts of the gastrointestinal tract including the esophagus. The esophageal involvement in amyloidosis has been reported to vary from 13% in a radiology study to 22% in an autopsy series; however, such patients have symptoms of gastroesophageal reflux. Dysphagia is an uncommon presentation of amyloidosis. We report a 64-year-old patient who presented with progressive dysphagia of 4 months duration which was confirmed to be due to primary amyloidosis with multiple myeloma. The esophageal involvement by amyloidosis was confirmed by esophageal mucosal biopsies, and 22-channel high-resolution manometry.

  8. Regional differences in prognostic value of cardiac valve plane displacement in systemic light-chain amyloidosis.

    Science.gov (United States)

    Ochs, Marco M; Fritz, Thomas; Arenja, Nisha; Riffel, Johannes; Andre, Florian; Mereles, Derliz; Siepen, Fabian Aus dem; Hegenbart, Ute; Schönland, Stefan; Katus, Hugo A; Friedrich, Matthias G W; Buss, Sebastian J

    2017-11-09

    To compare the prognostic value of cardiac valve plane displacement (CVPD) on various locations in cardiac light chain (AL) amyloidosis. Consecutive patients with biopsy-proven cardiac involvement in AL amyloidosis who had undergone cardiovascular magnetic resonance (CMR) between 2005 and 2014 in our institution, were retrospectively identified and data analyzed. The primary combined endpoint was all-cause mortality or heart transplantation. Systolic CVPD were obtained from standard cine bSSFP in 2-, 3- and 4-chamber views at anterior aortic plane systolic excursion (AAPSE); anterior, anterolateral, inferolateral, inferior, inferoseptal mitral (MAPSE); and lateral tricuspid (TAPSE) annular segments. We identified 68 patients (58 ± 10 years; 59% male). Median follow-up period was 1.2 years (IQR, 0.3-4.1). Significant differences in CVPD between patients who reached a primary endpoint (n = 44) and transplant-free survivors were found only for AAPSE (6.1 mm (IQR, 4.6-9.4) vs. 8.8 mm (IQR, 6.9-10.4); p = 0.02) and MAPSE anterolateral (7.3 mm (IQR, 5.4-11.7) vs. 10.5 mm (IQR, 8.1-13.4); p = 0.03). AAPSE (χ 2  = 15.6; p = 0.0002) provided the best predictive value for transplant-free survival compared to all other valvular plane locations. A high-risk cutoff (AAPSE ≤ 7.6 mm) was calculated by ROC analysis to predict all-cause death or heart transplantation within 6 months from index examination (AUC = 0.80; CI: 0.68 to 0.89; p model of late gadolinium enhancement and global longitudinal strain (GLS) (∆χ 2  = 5.8, p = 0.02) as well as to a clinical model including Karnofsky index and NT-proBNP (∆χ 2  = 6.2, p = 0.01). In patients with cardiac involvement in AL amyloidosis, systolic CVPD obtained from standard long axis cine views appear to indicate outcome better, when obtained in the anterior aortic plane (AAPSE) and provide incremental prognostic value to LGE and strain measurements.

  9. Macular Amyloidosis and Epstein-Barr Virus

    Directory of Open Access Journals (Sweden)

    Yalda Nahidi

    2016-01-01

    Full Text Available Background. Amyloidosis is extracellular precipitation of eosinophilic hyaline material of self-origin with special staining features and fibrillar ultrastructure. Macular amyloidosis is limited to the skin, and several factors have been proposed for its pathogenesis. Detection of Epstein-Barr virus (EBV DNA in this lesion suggests that this virus can play a role in pathogenesis of this disease. Objective. EBV DNA detection was done on 30 skin samples with a diagnosis of macular amyloidosis and 31 healthy skin samples in the margin of removed melanocytic nevi by using PCR. Results. In patients positive for beta-globin gene in PCR, BLLF1 gene of EBV virus was positive in 23 patients (8 patients in case and 15 patients in the control group. There was no significant difference in presence of EBV DNA between macular amyloidosis (3.8% and control (23.8% groups (P=0.08. Conclusion. The findings of this study showed that EBV is not involved in pathogenesis of macular amyloidosis.

  10. Amyloid Load in Fat Tissue Reflects Disease Severity and Predicts Survival in Amyloidosis

    NARCIS (Netherlands)

    Van Gameren, Ingrid I.; Hazenberg, Bouke P. C.; Bijzet, Johan; Haagsma, Elizabeth B.; Vellenga, Edo; Posthumus, Marcel D.; Jager, Pieter L.; Van Rijswijk, Martin H.

    Objective. The severity of systemic amyloidosis is thought to be related to the extent of amyloid deposition. We studied whether amyloid load in fat tissue reflects disease severity and predicts survival. Methods. We studied all consecutive patients with systemic amyloidosis seen between January

  11. Localized amyloidosis of the stomach mimicking a superficial gastric cancer.

    Science.gov (United States)

    Kagawa, Miwako; Fujino, Yasuteru; Muguruma, Naoki; Murayama, Noriaki; Okamoto, Koichi; Kitamura, Shinji; Kimura, Tetsuo; Kishi, Kazuhiro; Miyamoto, Hiroshi; Uehara, Hisanori; Takayama, Tetsuji

    2016-06-01

    A 73-year-old man was referred to our hospital for further examination of a depressed lesion in the stomach found by cancer screening gastroscopy. A barium upper gastrointestinal series showed an area of irregular mucosa measuring 15 mm on the anterior wall of the gastric body. Esophagogastroduodenoscopy revealed a 15 mm depressed lesion on the anterior wall of the lower gastric body. We suspected an undifferentiated adenocarcinoma from the appearance and took some biopsies. However, histology of the specimens revealed amyloidal deposits in the submucosal layer without malignant findings. Congo red staining was positive for amyloidal protein and green birefringence was observed under polarized light microscopy. Congo red staining with prior potassium permanganate incubation confirmed the light chain (AL) amyloid type. There were no amyloid deposits in the colon or duodenum. Computed tomography of the chest, abdomen, and pelvis showed no remarkable findings. Thus, this case was diagnosed as a localized gastric amyloidosis characterized by AL type amyloid deposition in the mucosal or submucosal layer. As the clinical outcome of gastric AL amyloidosis seems favorable, this case is scheduled for periodic examination to recognize potential disease progression and has been stable for 2 years.

  12. Mouse senile amyloid fibrils deposited in skeletal muscle exhibit amyloidosis-enhancing activity.

    Directory of Open Access Journals (Sweden)

    Jinze Qian

    2010-05-01

    Full Text Available Amyloidosis describes a group of protein folding diseases in which amyloid proteins are abnormally deposited in organs and/or tissues as fine fibrils. Mouse senile amyloidosis is a disorder in which apolipoprotein A-II (apoA-II deposits as amyloid fibrils (AApoAII and can be transmitted from one animal to another both by the feces and milk excreted by mice with amyloidosis. Thus, mouse AApoAII amyloidosis has been demonstrated to be a "transmissible disease". In this study, to further characterize the transmissibility of amyloidosis, AApoAII amyloid fibrils were injected into transgenic Apoa2(cTg(+/- and normal R1.P1-Apoa2(c mice to induce AApoAII systemic amyloidosis. Two months later, AApoAII amyloid deposits were found in the skeletal muscles of amyloid-affected mice, primarily in the blood vessels and in the interstitial tissues surrounding muscle fibers. When amyloid fibrils extracted from the skeletal muscles were subjected to Western blot analysis, apoA-II was detected. Amyloid fibril fractions isolated from the muscles not only demonstrated the structure of amyloid fibrils but could also induce amyloidosis in young mice depending on its fibril conformation. These findings present a possible pathogenesis of amyloidosis: transmission of amyloid fibril conformation through muscle, and shed new light on the etiology involved in amyloid disorders.

  13. [Amyloidosis complicating spondyloarthropathies: Study of 15 cases].

    Science.gov (United States)

    Rodríguez-Muguruza, Samantha; Martínez-Morillo, Melania; Holgado, Susana; Saenz-Sarda, Xavier; Mateo, Lourdes; Tena, Xavier; Olivé, Alejandro

    2015-10-21

    Secondary amyloidosis (AA) is a rare complication of rheumatic diseases. The aim of this study was to determine the frequency of symptomatic amyloidosis AA in patients with spondyloarthropathy. Retrospective study (1984-2013). We reviewed the medical records of patients with spondyloarthropathy who had a histological diagnosis of amyloidosis AA (15 patients). We identified 1.125 patients with spondyloarthropathies. Fifteen (1.3%) patients with amyloidosis AA were recruited. It was suspected in 14 patients (93.3%) because of nephrotic syndrome in most of them: 14 were symptomatic (93.3%): 5 (33.3%) ankylosing spondylitis (AS), 5 (33.3%) spondylitis associated with inflammatory bowel diseases (IBD), 4 (26.7%) psoriatic arthritis, and one (6.7%) reactive arthritis. The mean disease duration was 23.9 years. Mortality after one and 5 years of follow-up was 30 and 50% respectively. The frequency of clinical amyloidosis AA in our patients was 1.3%. There was a marked male predominance, with AS or IBD. Clinical amyloidosis was diagnosed at a relatively late stage in spondyloarthropathy. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  14. Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

    Science.gov (United States)

    Blank, Norbert; Hegenbart, Ute; Dietrich, Sascha; Brune, Maik; Beimler, Jörg; Röcken, Christoph; Müller-Tidow, Carsten; Lorenz, Hanns-Martin; Schönland, Stefan O

    2018-03-01

    To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis. Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. Patients with AA amyloidosis had either familial Mediterranean fever (FMF) or long-standing rheumatic diseases as underlying inflammatory disease (n = 111, median age 46 years). However, in a significant proportion of patients with AA amyloidosis no primary disease was identified (idiopathic AA; n = 37, median age 60 years). Patients with idiopathic AA amyloidosis were more obese and older than patients with AA amyloidosis secondary to FMF or rheumatic diseases. Serum leptin levels correlated with the body mass index (BMI) in all types of AA amyloidosis. Elevated leptin levels of more than 30 µg/l were detected in 18% of FMF/rheumatic + AA amyloidosis and in 40% of patients with idiopathic AA amyloidosis (p = .018). Finally, the SAA1 polymorphism was confirmed as a susceptibility factor for AA amyloidosis irrespective of the type of the disease. Obesity, age and the SAA1 polymorphism are susceptibility factors for idiopathic AA amyloidosis. Recent advances in treatment of FMF and rheumatic disorders will decrease the incidence of AA amyloidosis due to these diseases. Idiopathic AA, however, might be an emerging problem in the ageing and increasingly obese population.

  15. Mediastinal and bronchovascular amyloidosis

    International Nuclear Information System (INIS)

    Biggemann, M.; Hilweg, D.; Kappes, R.

    1990-01-01

    The clinical and radiographic aspects of an unusual case of mediastinal and bronchovascular amyloidosis are presented. Besides hilar and mediastinal lymphadenopathy, extensive amyloid deposition in the peribronchial and perivascular connective tissue sheats can be observed. Plain film radiographs and CT demonstrate an uncommon pattern of increased bronchovascular markings, which is discussed. Because of peribronchial amyloid deposits, bronchoscopy is of no help in demonstrating this special type of tracheobronchial amyloidosis. (orig.) [de

  16. Myocardial 99mTc-MDP uptake on the bone scintigraphy in the hemodialysis-associated amyloidosis: a case report

    International Nuclear Information System (INIS)

    Fard Esfahani, A.; Assadi, M.; Eftekhari, M.; Fallahi Sichani, B.; Beiki, D.; Akbarpour, S.; Mohammadian, M.

    2005-01-01

    Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different organs, where it usually causes some type of dysfunction. Its cause is unknown. Five different types of amyloidosis have been described according to the underlying disease; immunoglobulin amyloidosis, ,familial amyloidosis, senile systemic amyloidosis, secondary amyloidosis and hemodialysis-associated amyloidosis. We report a case of hemodialysis-associated amyloidosis in a 56-year-old man that radionuclide imaging demonstrated intense uptake of Tc-99m MDP within the myocardium. The diagnosis of amyloidosis was established by analysis of aspirated abdominal fat, although other non- invasive modalities didn't reveal any positive findings. The,first clue to the possible presence of amyloidosis in this case was provided by the radionuclide bone scan performed, which revealed intense tracer uptake in the heart suggesting amyloid deposit. We conclude that in cases of extraosseous accumulation of' Tc-99 MDP especially as a diffuse pattern of myocardial uptake, a diagnosis of amyloidosis should be considered, in an appropriate clinical setting

  17. Scintigraphic imaging and turnover studies with iodine-131 labelled serum amyloid P component in systemic amyloidosis

    International Nuclear Information System (INIS)

    Hawkins, P.N.; Pepys, M.B.; Aprile, C.; Capri, G.; Vigano, L.; Munzone, E.; Gianni, L.; Merlini, G.

    1998-01-01

    Radiolabelled serum amyloid P component (SAP) is a specific tracer for amyloid. Iodine-123 has ideal physical characteristics for scintigraphy but is expensive and not widely available. Here we report serial imaging and turnover studies in which we labelled SAP with iodine-131, a cheap alternative isotope which would be expected to yield poorer images but permit more prolonged turnover measurements. Imaging and plasma clearance and whole body retention (WBR) of tracer were studied for up to 7 days in ten patients with proven systemic AL amyloidosis and two patients in whom the diagnosis was suspected, after i.v. administration of about 37 MBq of 131 I-SAP. Normal blood pool images were obtained in the latter two subjects and amyloidosis was subsequently refuted histologically. WBR at 48 h was 65% of the injected dose (i.d.). Among the other ten patients, amyloid deposits were identified in the spleen in eight cases, liver in five and kidneys in four; other sites that gave positive results included bone, joints and soft tissues, and the myocardium in one case. Up to 95% of the tracer localised into amyloid within 6-h, and the values for WBR became progressively more discriminating during the study period, exceeding the normal reference value ( 131 I-SAP produced diagnostic scans in every patient in this series and, coupled with the detailed turnover information, is adequate for monitoring disease progress. (orig.)

  18. Amyloidosis in association with spontaneous feline immunodeficiency virus infection.

    Science.gov (United States)

    Asproni, Pietro; Abramo, Francesca; Millanta, Francesca; Lorenzi, Davide; Poli, Alessandro

    2013-04-01

    Tissues from 34 naturally feline immunodeficiency virus (FIV)-infected cats, 13 asymptomatic cats and 21 cats with signs of feline acquired immunodeficiency syndrome (F-AIDS), and 35 FIV-seronegative subjects were examined to determine the presence of amyloid deposits. Twenty experimentally FIV-infected cats and five specific pathogen-free (SPF) control cats were also included in the study. Paraffin-embedded sections from kidney and other organs were submitted to histological and histochemical analysis. Amyloid deposits were identified by a modified Congo red stain and confirmed by electron microscopy to demonstrate the presence of amyloid fibrils in amyloid positive glomeruli. In all positive cases, secondary amyloidosis was identified with potassium permanganate pretreatment and amyloid type was further characterised by immunohistochemistry using primary antibodies against human AA and feline AL amyloids. Amyloid deposits were present in different tissues of 12/34 (35%) naturally FIV-infected cats (seven presenting F-AIDS and five in asymptomatic phase) and in 1/30 FIV-seronegative cats. All the experimentally FIV-infected and SPF subjects showed no amyloid deposits. Amyloidosis has been reported in human lentiviral infections, and the data reported here demonstrate the need, in naturally FIV-infected cats, to consider the presence of amyloidosis in differential diagnosis of hepatic and renal disorders to better assess the prognosis of the disease.

  19. Amiloidosis bucal Oral amyloidosis

    Directory of Open Access Journals (Sweden)

    Isabella Lima Arrais Ribeiro

    2012-03-01

    , confirmado con la coloración del espécimen con el reactivo rojo congo. Los depósitos de amiloide fueron encontrados en el tejido conjuntivo, que con la luz polarizada presentó birrefringencia. Tal hallazgo fue preocupante, ya que la amiloidosis afecta diversos tejidos, lo que puede provocar complicaciones sistémicas. Por esa razón la paciente fue orientada a buscar atención médica. Sin embargo, abandonó el tratamiento y falleció 6 meses después del diagnóstico de la enfermedad. Lesiones bucales aparentemente simples pueden revelar enfermedades raras y de difícil tratamiento. El diagnóstico preciso y la supervisión médica son fundamentales para la sobrevida del paciente.Amyloidosis is an uncommon complicated disease of a difficult diagnosis occurring due to the amyloid substance depot in the extracellular medium. Being diagnosed in the oral cavity, the patient must to be supervised to assess the potential systemic complications of disease. The aim of present paper was to present a case of oral amyloidosis in a female patient ages 72 presenting with traumatic fibroma. After performance of a biopsy and the histopathological examination, the diagnosis was the presence of amyloidosis, confirmed with the help of the sample using Congo red reactant. Amyloid depots were found in the conjunctive tissue which under the polarized light showed birefringence. This finding was worrying since the amyloidosis involves different tissues leading to systemic complications. Thus, the patient was oriented to search medical care; however she abandons treatment dying 6 months after diagnosis of the disease. The apparently single oral injuries may to reveal uncommon diseases and of difficult treatment. The precise treatment and the medical supervision are essential in the patient's survival.

  20. [Value of aspiration biopsy of subcutaneous fat in amyloidosis].

    Science.gov (United States)

    Ponce, P; Carvalho, F; Coelho, A

    1986-01-01

    Fine-needle aspiration of subcutaneous fat (FNAF) was performed in 24 patients, 12 with previously diagnosed amyloidosis presenting with proteinuria or nephrotic syndrome, and 12 presenting a nephrotic syndrome without amyloidosis on renal biopsy. FNAF was positive in 10 of 12 patients with amyloidosis (sensitivity: 83%) and negative in 12 of 12 patients with non-amyloid nephrotic syndrome (specificity: 100%). Considering a 2.5 to 10% prevalence of amyloidosis in adult patients with proteinuria or nephrotic syndrome, a positive FNAF is diagnostic of amyloidosis, and a negative FNAF rules out the diagnosis with a probability of 98 to 99%. FNAF is a simple and safe method which can be useful in patients who cannot undergo a renal biopsy.

  1. Combined pulmonary involvement in hereditary lysozyme amyloidosis with associated pulmonary sarcoidosis: a case report.

    Science.gov (United States)

    McCarthy, Cormac; Deegan, Alexander P; Garvey, John F; McDonnell, Timothy J

    2013-12-17

    Sarcoidosis is a multisystem inflammatory disorder of unknown cause which can affect any organ system. Autosomal dominant lysozyme amyloidosis is a very rare form of hereditary amyloidosis. The Arg64 variant is extraordinarily rare with each family showing a particular pattern of organ involvement, however while Sicca syndrome, gastrointestinal involvement and renal failure are common, lymph node involvement is very rare. In this case report we describe the first reported case of sarcoidosis in association with hereditary lysozyme amyloidosis.

  2. Long-term event-free and overall survival after risk-adapted melphalan and SCT for systemic light chain amyloidosis.

    Science.gov (United States)

    Landau, H; Smith, M; Landry, C; Chou, J F; Devlin, S M; Hassoun, H; Bello, C; Giralt, S; Comenzo, R L

    2017-01-01

    Stem cell transplantation (SCT), an effective therapy for amyloid light chain (AL) amyloidosis patients, is associated with low treatment-related mortality (TRM) with appropriate patient selection and risk-adapted dosing of melphalan (RA-SCT). Consolidation after SCT increases hematologic complete response (CR) rates and may improve overall survival (OS) for patients with SCT with or without consolidation. Melphalan was administered at 100 (14%), 140 (52%) and 200 (34%) mg/m 2 . The TRM rate at 100 days was 5%. RA-SCT resulted in CR in 24% (3 months) and 48% (12 months) of patients. The CR rate was particularly high (62%) in patients offered bortezomib consolidation. With a median follow-up among survivors of 7.7 years, median event-free survival (EFS) with RA-SCT was 4.04 years (95% confidence interval (CI): 3.41-5.01 years); median OS was 10.4 years (95% CI: 7.3-not achieved). Patients with CR at 12 months after SCT had significantly longer EFS (P=0.01) and OS (P=0.04). In a multivariate analysis, melphalan dose had no impact on EFS (P=0.26) or OS (P=0.11). For selected patients, RA-SCT was safe and was associated with extended long-term survival. With the availability of novel agents for consolidation, RA-SCT remains a very effective and important backbone treatment for AL amyloidosis.

  3. Cardiac amyloidosis

    International Nuclear Information System (INIS)

    Marin, Jorge E; Duque, Mauricio; Medina, Luis E; Uribe, William; Velasquez, Jorge E

    2005-01-01

    Amyloidosis is an infiltrative systemic disease that may involve the heart. it has a genetic etiology and is an important cause of restrictive cardiomyopathy. It may involve all heart structures but has a great affinity for myocardial tissue. Diastolic dysfunction is the most early and frequent manifestation, although due to myocardial infiltration, it may progress to systolic dysfunction, resulting in a rigid heart syndrome. There is also an involvement of the conducting system. The condition may be suspected in any patient with cardiomegalia of unexplained cause. Among the diagnostic tools, the voltage/mass relation may be kept in mind. endomyocardial biopsy is useful although it is not always positive through histological verification. The treatment consists of supportive measures and selected cases may benefit with hepatic transplantation

  4. Prognostic and Added Value of Two-Dimensional Global Longitudinal Strain for Prediction of Survival in Patients with Light Chain Amyloidosis Undergoing Autologous Hematopoietic Cell Transplantation.

    Science.gov (United States)

    Pun, Shawn C; Landau, Heather J; Riedel, Elyn R; Jordan, Jonathan; Yu, Anthony F; Hassoun, Hani; Chen, Carol L; Steingart, Richard M; Liu, Jennifer E

    2018-01-01

    Autologous hematopoietic cell transplantation (HCT) is a first-line therapy for prolonging survival in patients with light-chain (AL) amyloidosis. Cardiac involvement is the most important determinant of survival. However, patients with advanced cardiac involvement have often been excluded from HCT because of high risk for transplantation-related mortality and poor overall survival. Whether baseline left ventricular global longitudinal strain (GLS) can provide additional risk stratification and predict survival after HCT in this high-risk population remains unclear. The aim of this study was to evaluate the prognostic implication of baseline GLS and the added value of GLS beyond circulating cardiac biomarkers for risk stratification in patients with AL amyloidosis undergoing HCT. Eighty-two patients with newly diagnosed AL amyloidosis who underwent upfront HCT between January 2007 and April 2014 were included in the study. Clinical, echocardiographic, and serum cardiac biomarker data were collected at baseline and 12 months following HCT. GLS measurements were performed using a vendor-independent offline system. The median follow-up time for survivors was 58 months. Sixty-four percent of patients were in biomarker-based Mayo stage II or III. GLS, brain natriuretic peptide, troponin, and mitral E/A ratio were identified as the strongest predictors of survival (P value that best discriminated survivors from nonsurvivors, and the application of this cutoff value provided further mortality risk stratification within each Mayo stage. GLS is a strong predictor of survival in patients with AL amyloidosis undergoing HCT, potentially providing incremental value over serum cardiac biomarkers for risk stratification. GLS should be considered as a standard parameter along with serum cardiac biomarkers when evaluating eligibility for HCT or other investigational therapies. Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  5. Amyloidosis of the thyroid gland: ultrasonographic aspect

    International Nuclear Information System (INIS)

    Moya, M.I.; Vilas, I.; Menargues, M.A.; Hernandez, M.

    1998-01-01

    Subclinical amyloid infiltration of the thyroid gland is very common. However, amyloidosis rarely provokes thyroid symptoms. We describe a case of goiter due to secondary amyloidosis and review the characteristic ultrasound findings associated with this condition. (Author) 6 refs

  6. Programmed initiation of hemodialysis for systemic amyloidosis patients associated with rheumatoid arthritis.

    Science.gov (United States)

    Kuroda, Takeshi; Tanabe, Naohito; Kobayashi, Daisuke; Sato, Hiroe; Wada, Yoko; Murakami, Shuichi; Sakatsume, Minoru; Nakano, Masaaki; Narita, Ichiei

    2011-09-01

    Reactive amyloidosis is a serious systemic disease in rheumatoid arthritis (RA). Amyloid protein can be deposited in kidneys, heart or gastrointestinal tract leading to organ failure. Renal involvement is a well-known complication in amyloidosis as this may culminate in end-stage renal disease (ESRD). Hemodialysis (HD) is always considered the treatment of choice for such patients; however, the prognosis is usually poor due to a large number of sudden deaths immediately following HD therapy. To circumvent the problem of HD initiation while instituting HD safety, we devised a plan to start HD and compare patient's survival with our previous data. Sixty-three patients were treated with HD. They were categorized according to the initiation of first dialysis. All patients were divided into planned, unplanned and programmed initiation groups. First dialysis that had been initiated as not urgent was considered 'planned' (20 patients). First dialysis that had been performed urgently for life-threatening renal insufficiency was considered 'unplanned' (31 patients). First dialysis that had been initiated as not urgent and according to our dialysis program was considered 'programmed' (12 patients). Survival of these 63 patients from the initiation of HD at 38 days was 75%, at 321 days was 50% and at 1,784 days was 25%. Patients with unplanned initiation of HD showed a significant poor survival compared with those of both planned and programmed initiation. Additionally, patients with planned and programmed initiation of HD showed no significant difference for the patients' survival. Our study demonstrates that patients with amyloidosis have a higher mortality rate. Nevertheless, programmed initiation of HD will improve the prognosis of patients with ESRD. Such possibility needs to be considered in more detail in the future.

  7. Spontaneous, Experimentally Induced, and Transmissible AA Amyloidosis in Japanese Quail ( Coturnix japonica).

    Science.gov (United States)

    Nakayama, Yumi; Kamiie, Junichi; Watanabe, Gen; Suzuki, Kazuhiko; Murakami, Tomoaki

    2017-11-01

    The authors describe a spontaneous case of amyloid A (AA) amyloidosis in an adult female Japanese quail ( Coturnix japonica). The bird developed AA amyloidosis secondary to chronic peritonitis caused by a Gram-negative bacillus infection. Mild amyloid deposition was also identified in the intestinal tract of apparently healthy adult individuals, suggesting that quail may develop intestinal amyloidosis with age. Based on these observations, it was hypothesized that quail can develop AA amyloidosis following inflammatory stimulation with lipopolysaccharide (LPS). Therefore, adult quail were repeatedly injected with LPS and the development of AA amyloidosis was confirmed. The amyloid deposition in this model increased when quail amyloid was intravenously injected as an amyloid-enhancing factor. The experiments were repeated with young quail, but amyloid deposits were not observed following LPS injections. However, AA amyloidosis did develop when quail amyloid was injected in addition to LPS. These results indicated that adult quail develop AA amyloidosis after inflammatory stimulation with LPS. Furthermore, quail AA amyloidosis was shown to have transmissibility regardless of age. Interestingly, the authors found that administration of chicken amyloid fibrils also induced AA amyloidosis in young quail. This is the first report of cross-species transmission of avian AA amyloidosis.

  8. Amyloidosis in alkaptonuria.

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    Millucci, Lia; Braconi, Daniela; Bernardini, Giulia; Lupetti, Pietro; Rovensky, Josef; Ranganath, Lakshminaryan; Santucci, Annalisa

    2015-09-01

    Alkaptonuria (AKU) is an ultra-rare inborn error of metabolism developed from the lack of homogentisic acid oxidase activity, causing homogentisic acid (HGA) accumulation that produces an HGA-melanin ochronotic pigment, of hitherto unknown composition. Besides the accumulation of HGA, the potential role and presence of unidentified proteins has been hypothesized as additional causal factors involved in ochronotic pigment deposition. Evidence has been provided on the presence of serum amyloid A (SAA) in several AKU tissues, which allowed classifying AKU as a novel secondary amyloidosis. In this paper, we will briefly review all direct and indirect lines of evidence related to the presence of amyloidosis in AKU. We also report the first data on abnormal SAA serum levels in a cohort of AKU patients.

  9. NUCLEAR IMAGING IN THE DIAGNOSIS OF CARDIAC AMYLOIDOSIS

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    V. B. Sergienko

    2018-01-01

    Full Text Available Histological analysis of endomyocardial tissue is still the gold standard for the diagnosis of cardiac amyloidosis but has its limitations. Accordingly, there is a need for noninvasive techniques to cardiac amyloidosis diagnostics. Echocardiography and magnetic resonance imaging can show characteristics which may not be very specific for cardiac amyloid. Recently, new opportunities of nuclear imaging in risk stratification and assessment of prognosis for patients with cardiac amyloidosis have appeared. During the last two decades different classes of radiopharmaceuticals have been developed based on compounds tropic to the components of amyloid infiltrates. In this paper we describe the current possibilities and perspectives of nuclear medicine techniques in patients with cardiac amyloidosis, including osteotropic and neurotropic scintigraphy, single-photon and positron emission tomography

  10. Systemic Amyloidosis and Testicular Interstitial Tumor in a Zebra Finch (Taeniopygia guttata: a Case Report in Iran

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    Mehrnoush Moeini Jazani

    2011-09-01

    Full Text Available Abstract Systemic amyloidosis and testicular interstitial tumor are rare conditions in birds and this is the first report in Iran. A male zebra finch was presented because of white diarrhea, anorexia, loss of weight and lethargy. At necropsy, the small intestine was edematous and congested. The spleen appeared pale. The liver was large, firm and brown. One testis was cystic and neoplastic and the remaining testis was atrophic. Histologically, amyloid materials were seen predominantly in the liver and spleen. Hyaline substances were deposited in the Disse space and in the media of blood vessels of the liver. In spleen, marked deposits thickened the basement membranes of blood vessels and extended into the surrounding parenchyma. In addition, there were lesser degrees of amyloidosis in other organs such as small intestine. Amyloid stained positively with Congo red. In testis, there was encapsulated unilateral interstitial cell tumor, with multiple foci of necrosis and hemorrhage. The neoplastic cells were round to polyhedral, with small round hyperchromatic nuclei and finely vacuolated cytoplasm. Signs of feminization were observed. The cause of amyloidosis in this study was not conclusively identified.

  11. Characteristics of AA amyloidosis patients in San Francisco.

    Science.gov (United States)

    Lejmi, Hiba; Jen, Kuang-Yu; Olson, Jean L; James, Sam H; Sam, Ramin

    2016-04-01

    AA amyloidosis due to subcutaneous injection of drugs of abuse has been described in the USA, but all the existing literature is from more than 20 years ago. There is more recent literature from Europe. We have observed a high incidence of AA amyloidosis in the county hospital in San Francisco. Here, we describe 24 patients who had kidney biopsy-proven AA amyloidosis from our hospital from 1998 to 2013. All the patients were thought to have AA amyloidosis from skin popping of illicit drugs after having exhausted the intravenous route. These patients with biopsy-proven AA amyloidosis were analysed further. All patients were found to have hepatitis C infection, hypertension was not common, most had advanced kidney failure, and acidosis was common as was tubulointerstitial involvement on the kidney biopsy. Other organ involvement included hepatomegaly and splenomegaly in a number of patients; direct myocardial involvement was not seen, but pulmonary hypertension, history of deep vein thrombosis and pulmonary embolism were common. The prognosis of these patients was poor. The mortality rate approached 50% 1 year after biopsy, and most of the patient needed dialysis shortly after diagnosis. Cessation of drug use seemed beneficial but rarely achievable. AA amyloidosis from skin popping is common in San Francisco. Most patients with renal involvement end up on dialysis, and mortality rates are exceedingly high. © 2015 Asian Pacific Society of Nephrology.

  12. Prognostic value of left atrial function in systemic light-chain amyloidosis: a cardiac magnetic resonance study.

    Science.gov (United States)

    Mohty, Dania; Boulogne, Cyrille; Magne, Julien; Varroud-Vial, Nicolas; Martin, Sylvain; Ettaif, Hind; Fadel, Bahaa M; Bridoux, Frank; Aboyans, Victor; Damy, Thibaud; Jaccard, Arnaud

    2016-09-01

    Cardiac involvement in systemic light-chain amyloidosis (AL) imparts an adverse impact on outcome. The left atrium (LA), by virtue of its anatomical location and muscular wall, is commonly affected by the amyloid process. Although LA infiltration by amyloid fibrils leads to a reduction in its pump function, the infiltration of the left ventricular (LV) myocardium results in diastolic dysfunction with subsequent increase in filling pressures and LA enlargement. Even though left atrial volume (LAV) is an independent prognostic marker in many cardiomyopathies, its value in amyloid heart disease remains to be determined. In addition, few data are available as to the prognostic value of LA function in systemic AL. Using cardiac magnetic resonance (CMR), the current study aims to assess the prognostic significance of the maximal LAV and total LA emptying fraction (LAEF) in patients with AL. Fifty-four consecutive patients (age 66 ± 10 years, 59% males) with confirmed systemic AL and mean LV ejection fraction of 60 ± 12% underwent CMR. As compared with patients with no or minimal cardiac involvement (Mayo Clinic [MC] stage I), those at moderate and high risk (MC stages II and III) had significantly larger indexed maximal LAV (36 ± 15 vs. 46 ± 13 vs. 52 ± 19 mL/m(2), P = 0.03) and indexed minimal LAV (20 ± 6 vs. 34 ± 11 vs. 44 ± 17 mL/m(2), P 16% (37 ± 11 vs. 94 ± 4%, P = 0.001). In multivariate analysis, lower LAEF remained independently associated with a higher risk of 2-year mortality (HR = 1.08 per 1% decrease, 95% CI: 1.02-1.15, P = 0.003). In patients with systemic AL, LAEF as assessed by CMR is associated with NYHA functional class, MC stage, myocardial LGE and 2-year mortality. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.

  13. Hereditary Transthyretin Amyloidosis in Eight Chinese Families

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    Ling-Chao Meng

    2015-01-01

    Full Text Available Background: Mutations of transthyretin (TTR cause the most common type of autosomal-dominant hereditary systemic amyloidosis, which occurs worldwide. To date, more and more mutations in the TTR gene have been reported. Some variations in the clinical presentation are often observed in patients with the same mutation or the patients in the same family. The purpose of this study was to find out the clinicopathologic and genetic features of Chinese patients with hereditary TTR amyloidosis. Methods: Clinical and necessary examination materials were collected from nine patients of eight families with hereditary TTR amyloidosis at Peking University First Hospital from January 2007 to November 2014. Sural nerve biopsies were taken for eight patients and skin biopsies were taken in the calf/upper arm for two patients, for light and electron microscopy examination. The TTR genes from the nine patients were analyzed. Results: The onset age varied from 23 to 68 years. The main manifestations were paresthesia, proximal and/or distal weakness, autonomic dysfunction, cardiomyopathy, vitreous opacity, hearing loss, and glossohypertrophia. Nerve biopsy demonstrated severe loss of myelinated fibers in seven cases and amyloid deposits in three. One patient had skin amyloid deposits which were revealed from electron microscopic examination. Genetic analysis showed six kinds of mutations of TTR gene, including Val30Met, Phe33Leu, Ala36Pro, Val30Ala, Phe33Val, and Glu42Gly in exon 2. Conclusions: Since the pathological examinations of sural nerve were negative for amyloid deposition in most patients, the screening for TTR mutations should be performed in all the adult patients, who are clinically suspected with hereditary TTR amyloidosis.

  14. Evaluation of 61 Secondary Amyloidosis Patients: A Single-Center Experience from Turkey

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    Can Huzmeli

    2016-09-01

    Full Text Available Aim: To evaluate demographic,clinical and laboratory characteristics, causes, MEFV gene mutations, and mortality rates of patients with secondary amyloidosis. Material and Method: 61 patients who had been diagnosed with secondary amyloidosis by renal and rectal biopsy between 2007 and 2013 in the nephrology clinic of Cumhuriyet University, Faculty of Medicine, were included in the study. Demographic characteristics, causes of secondary amyloidosis, MEFV gene mutations, end-stage renal failure (ESRF, renal transplantation, and mortality rates were examined retrospectively. Results: In etiological terms, Familial Mediterranean Fever (FMF occurrence was 62.2% (38, bronchiectasis and emphysema 9.8% (6, tuberculosis 4.9% (3, coexistence of FMF and ankylosing spondylitis 3.2% (2, coexistence of FMF and rheumatoid arthritis 1.6% (1, coexistence of FMF and systemic lupus erythematosus (SLE 1.6% (1, osteomyelitis 1.6% (1, septic arthritis 1.6% (1, Crohn%u2019s disease 1.6% (1, colon cancer 1.6% (1, coexistence of bronchiectasis and tuberculosis 1.6% (1, rheumatoid arthritis 1.6% (1, and idiopathic cases 6.5% (4. Proteinuria was determined at nephrotic level among 68% (32 of 47 patients who had secondary amyloidosis. MEFV gene mutation of 45 patients with secondary amyloidosis was assessed. Most patients had M694V gene mutation. Surprisingly, we detected heterozygous E148Q mutation in 3 cases. 12 cases died; of these, 9 had ESRF. Five cases with ESRF underwent renal transplantation. Discussion: We found FMF as the most common cause for secondary AA amyloidosis in this study. Further studies should be done with larger or multicenter cohorts.

  15. Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre.

    Science.gov (United States)

    Lane, Thirusha; Pinney, Jennifer H; Gilbertson, Janet A; Hutt, David F; Rowczenio, Dorota M; Mahmood, Shameem; Sachchithanantham, Sajitha; Fontana, Marianna; Youngstein, Taryn; Quarta, Candida C; Wechalekar, Ashutosh D; Gillmore, Julian D; Hawkins, Philip N; Lachmann, Helen J

    2017-09-01

    Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology of AA amyloidosis over a 25-year period at a single national referral centre. We conducted a retrospective study of all patients diagnosed with AA amyloidosis who had attended the centre between 1990 and 2014 inclusive. Six hundred and twenty-five patients were studied in three cohorts: C1: 1990-1997; C2: 1998-2006; C3: 2007-2014. Mean age at presentation increased from 46 in C1 to 56 in C3 (p AA amyloidosis over a quarter of a century, reflecting advances in therapeutics and overall management of complex chronic disease in an ageing population. AA amyloidosis of uncertain aetiology presents an emerging major problem. Newer techniques such as next-generation sequencing may aid diagnosis and effective treatment, thereby improving overall survival.

  16. [Amyloidosis maculosa: diagnosis in primary care].

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    Toribio da Pena, S R; Olmos, O; Borbujo, J; Bastos Amigo, J A; Jiménez-Sánchez, F; Alonso, A

    1990-01-01

    Amyloidosis maculosa is a clinical entity with low incidence factor in our medium, which basically affects middle-aged women. The lesion is characterised by the presence of poorly defined, hyperpigmented, brownish or greyish maculae that converge and focus basically on the upper back and shoulders, usually accompanied by pruritus. Three patients were erroneously catalogued for years as having pityriasis versicolor. Two of these patients presented a typical clinical amyloidosis maculosa, and the third presented a less common manifestation of the disease: a single, well-defined lesion in the subscapular region. We believe that the approach to the diagnosis of pityriasis versicolor with hyperpigmented lesions that do not respond to specific treatment should be revised. Although amyloidosis maculosa has a low incidence in our medium, it is an entity which should not be discarded in these cases.

  17. Renal AA amyloidosis in a patient with hereditary complete complement C4 deficiency

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    Imed Helal

    2011-01-01

    Full Text Available Hereditary complete C4 deficiency has until now been reported in 30 cases only. A disturbed clearance of immune- complexes probably predisposes these individuals to systemic lupus erythematosus, other immune- complex diseases and recurrent microbial infections. We present here a 20- year- old female with hereditary complete C4 deficiency. Renal biopsy demonstrated renal AA amyloidosis. This unique case further substantiates that deficiency of classical pathway components predisposes to the development of recurrent microbial infections and that the patients may develop AA amyloidosis. Furthermore, in clinical practice, the nephrotic syndrome occurring in a patient with hereditary complete complement C4 deficiency should lead to the suspicion of renal AA amyloidosis.

  18. [The prognostic value of baseline serum free light chain in cardiac amyloidosis].

    Science.gov (United States)

    Zhao, Lei; Tian, Zhuang; Fang, Quan

    2016-03-01

    To analyze the prognostic value of baseline serum free light chain (sFLC) in light-chain (AL) cardiac amyloidosis. Twenty-seven patients with AL cardiac amyloidosis were retrospectively reviewed from January 2014 to January 2015. sFLC was measured by immuoturbidimetric assay. Baseline characteristics, echocardiographic parameters and electrocardiogram data were analyzed. According to the median baseline dFLC (involved sFLC minus uninvolved sFLC), patients were categorized into either the low dFLC(≤ 307 mg/L) or the high dFLC group (>307 mg/L). More subjects in the high dFLC group with early/late diastolic mitral velocity ratio (E/A ratio) over 2 (71.4% vs 30.8%, P=0.035), and subjects in this group had a shorter median survival time than those in the low dFLC group (3 months vs 17 months, P=0.004). A similar phenomenon for median survival time was observed when the subjects were redivided either by a new cut-off value of 180 mg/L for dFLC (low dFLC group: 17 months; high dFLC group: 4 months, P=0.014) or a κ/λ ratio, in which subjects with κ type sFLC-ratio ≤ 19.6 and λ type sFLC-ratio>0.065 were in the low sFLC-ratio group (17 months) and those with κ type sFLC-ratio > 19.6 and λ type sFLC-ratio ≤ 0.065 were in the high sFLC-ratio group (4 months, P=0.023). In multivariate analysis, dFLC and New York Heart Association (NYHA)classification of cardiac function were two risk factors associated with all-cause mortality in patients, among which the hazard ratio for higher dFLC was 4.28 (95%CI 1.55-11.8, P=0.005). The level of sFLC could be a marker for the prognosis of AL cardiac amyloidosis.

  19. AA amyloidosis complicating the hereditary periodic fever syndromes.

    Science.gov (United States)

    Lane, Thirusha; Loeffler, Jutta M; Rowczenio, Dorota M; Gilbertson, Janet A; Bybee, Alison; Russell, Tonia L; Gillmore, Julian D; Wechalekar, Ashutosh D; Hawkins, Philip N; Lachmann, Helen J

    2013-04-01

    AA amyloidosis is a life-threatening complication of the hereditary periodic fever syndromes (HPFS), which are otherwise often compatible with normal life expectancy. This study was undertaken to determine the characteristics, presentation, natural history, and response to treatment in 46 patients who had been referred for evaluation at the UK National Amyloidosis Centre. Disease activity was monitored by serial measurement of serum amyloid A. Renal function was assessed by measurement of serum creatinine and albumin levels, the estimated glomerular filtration rate, and proteinuria from 24-hour urine collections. The amyloid load was measured by serum amyloid P scintigraphy. Twenty-four patients had familial Mediterranean fever, 12 patients had tumor necrosis factor receptor-associated periodic syndrome, 6 patients had cryopyrin-associated periodic syndromes, and 4 patients had mevalonate kinase deficiency. The median age at onset of HPFS was 5 years; median age at presentation with AA amyloidosis was 38 years. Diagnosis of an HPFS had not been considered prior to presentation with AA amyloidosis in 23 patients (50%). Eleven patients (24%) had end-stage renal failure (ESRF) at presentation; of these, 3 had received transplants prior to referral. A further 13 patients developed ESRF over the followup period, with 10 undergoing renal transplantation. The median time to progression to ESRF from onset of AA amyloidosis was 3.3 years (interquartile range [IQR] 2-8), with a median time to transplant of 4 years (IQR 3-6). Eleven patients (24%) died. The median survival in the entire cohort was 19 years from diagnosis of AA amyloidosis. Of the 37 patients who were treated successfully, or in whom at least partial suppression of the underlying HPFS was achieved, 17 (46%) showed amyloid regression, 14 (38%) showed a stable amyloid load, and 2 (5%) showed increased amyloid deposition over the followup period. AA amyloidosis remains a challenging and serious late complication

  20. Primary Hepatic Amyloidosis: Report of an Unusual Case Presenting as a Mass

    International Nuclear Information System (INIS)

    Son, Rak Chae; Chang, Jae Chun; Choi, Joon Hyuk

    2011-01-01

    Hepatic involvement of amyloidosis is common. Diffuse infiltration with hepatomegaly is a usual radiologic finding of hepatic amyloidosis. To our knowledge, this is the first case of amyloidosis involving the liver that presented as a mass.

  1. Cardiac amyloidosis induces up-regulation of Deleted in Malignant Brain Tumors 1 (DMBT1)

    DEFF Research Database (Denmark)

    Müller, Hanna; Renner, Marcus; Bergmann, Frank

    2013-01-01

    Amyloidosis is a life-threatening protein misfolding disease and affects cardiac tissue, leading to heart failure, myocardial ischemia and arrhythmia. Amyloid deposits result in oxidative stress, inflammation and apoptosis. The purpose of this study was to examine the role of innate defense compo...... components, i.e., Deleted in Malignant Brain Tumors 1 (DMBT1) and the complement system, in different types of cardiac amyloidosis....

  2. Churg-Strauss syndrome associated with AA amyloidosis: a case report.

    Science.gov (United States)

    Maamar, Mouna; Tazi-Mezalek, Zoubida; Harmouche, Hicham; El Hamany, Zitouna; Adnaoui, Mohammed; Aouni, Mohammed

    2012-01-01

    Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day. A diagnosis of Churg-Strauss syndrome was established based on five criteria of the American College of Rheumatology (ACR). Renal biopsy showed an important type AA amyloid deposit. The patient was treated with steroids with a good response of the vasculitis and amyloidosis with disappearance of the proteinuria.

  3. Serum amyloid A protein in amyloidosis, rheumatic, and neoplastic diseases

    International Nuclear Information System (INIS)

    Benson, M.D.; Cohen, A.S.

    1979-01-01

    Serum levels of amyloid protein A (SAA) have been shown to be elevated in different types of amyloidosis and in rheumatic diseases by radioimmunoassay using 125 iodine labeled AA and anti-AA. SAA levels were elevated in both primary and secondary amyloidosis, but there were highly significant differences between these levels. In heredofamilial amyloid, SAA levels were within normal limits. While the mean SAA level was elevated in persons over 70 years, the fact that some persons in this age group had normal levels suggested that marked elevation after age 70 may be due to occult inflammatory or neoplastic disease. High SAA levels in patients with rheumatoid arthritis correlated, in most cases, with physician evaluation of disease activity and Westergren ESR. SAA levels in patients with systemic lupus erythematosus were lower than those in patients with rheumatoid arthritis, and most patients with degenerative joint disease had normal levels. Very high levels of SAA were found in patients with neoplastic diseases. Patients with carcinoma of the lung and bowel had much higher levels than patients with carcinoma of the breast. Determination of SAA levels may be of value in evaluating different forms of systemic amyloidosis, assessing the activity of rheumatic disease, and screening for occult inflammatory or neoplastic disease

  4. Oral involvement in a case of AA amyloidosis: a case report

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    Yıldız Levent

    2010-06-01

    Full Text Available Abstract Introduction Deposition of amyloid fibrils derived from circulating acute-phase reactant serum amyloid A protein causes systemic amyloidosis, a serious inflammatory disorder. We document a male patient who developed reactive amyloidosis (AA type, most likely secondary to his long standing periodontitis. Case presentation A 67-year-old Turkish man complained of pain in his oral cavity (burning mouth especially on the tongue, and had difficulty chewing and swallowing foods. A careful dental/periodontal examination was performed, including assessment of plaque, gingival condition and periodontal probing depths on all his remaining teeth. Prosthetic rehabilitation was provided three months after the completion of his periodontal and surgical therapy. The concentration of serum inflammatory markers including erythrocyte sedimentation rate, white blood cell count, fibrinogen and high sensitive C-reactive protein were measured at baseline, at the second and sixth weeks, and at three and six months after the periodontal and surgical therapy. Conclusions Oral examination revealed a few papules on the dorsum of the tongue with two slightly painful, small ulcers, localized on the vestibule of the mouth. The mean probing depth was 9.10 ± 0.84 mm. Biopsies of the tongue, buccal mucosa and retromolar trigone were performed and amyloid deposits were found. The serum inflammatory markers improved more dramatically at the second week of periodontal therapy than any other time intervals. Amyloidosis may manifest as periodontal destruction that leads to severe chronic periodontitis. Proper periodontal treatment may alleviate systemic inflammatory mediators caused by the amyloidosis.

  5. Amyloidosis cutis dyschromica: A rare reticulate pigmentary dermatosis

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    Shyam Verma

    2015-01-01

    Full Text Available We are reporting a rare case of amyloidosis cutis dyschromica in a 41-year-old man. This is a rare form of primary cutaneous amyloidosis characterized by reticulate pigmentation with hypopigmented and hyperpigmented macules, onset in childhood, familial tendency in some, occasional mild itching and deposition of amyloid in the papillary dermis. Our case also had multiple bilaterally symmetrical hyperpigmented keratotic papules abutting the axillary vault resembling those seen in Dowling-Deogs disease. The other unusual feature in this patient was the strong family history of vitiligo, which we are unable to explain. We have also tried to explain the mechanism leading to the hyperpigmentation and hypopigmentation in amyloidosis cutis dyschromica.

  6. MRI of pathology-proven peripheral nerve amyloidosis

    International Nuclear Information System (INIS)

    McKenzie, Gavin A.; Broski, Stephen M.; Howe, Benjamin M.; Spinner, Robert J.; Amrami, Kimberly K.; Dispenzieri, Angela; Ringler, Michael D.

    2017-01-01

    To highlight the MRI characteristics of pathologically proven amyloidosis involving the peripheral nervous system (PNS) and determine the utility of MRI in directing targeted biopsy for aiding diagnosis. A retrospective study was performed for patients with pathologically proven PNS amyloidosis who also underwent MRI of the biopsied or excised nerve. MRI signal characteristics, nerve morphology, associated muscular denervation changes, and the presence of multifocal involvement were detailed. Pathology reports were reviewed to determine subtypes of amyloid. Charts were reviewed to gather patient demographics, neurological symptoms and radiologist interpretation. Four men and three women with a mean age of 62 ± 11 years (range 46-76) were identified. All patients had abnormal findings on EMG with mixed sensorimotor neuropathy. All lesions demonstrated diffuse multifocal neural involvement with T1 hypointensity, T2 hyperintensity, and variable enhancement on MRI. One lesion exhibited superimposed T2 hypointensity. Six of seven patients demonstrated associated muscular denervation changes. Peripheral nerve amyloidosis is rare, and the diagnosis is difficult because of insidious symptom onset, mixed sensorimotor neurologic deficits, and the potential for a wide variety of nerves affected. On MRI, peripheral nerve involvement is most commonly characterized by T1 hypointensity, T2 hyperintensity, variable enhancement, maintenance of the fascicular architecture with fusiform enlargement, multifocal involvement and muscular denervation changes. While this appearance mimics other inflammatory neuropathies, MRI can readily detect neural changes and direct-targeted biopsy, thus facilitating early diagnosis and appropriate management. (orig.)

  7. MRI of pathology-proven peripheral nerve amyloidosis

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    McKenzie, Gavin A.; Broski, Stephen M.; Howe, Benjamin M.; Spinner, Robert J.; Amrami, Kimberly K.; Dispenzieri, Angela; Ringler, Michael D. [Mayo Clinic, Department of Musculoskeletal Radiology, Rochester, MN (United States)

    2017-01-15

    To highlight the MRI characteristics of pathologically proven amyloidosis involving the peripheral nervous system (PNS) and determine the utility of MRI in directing targeted biopsy for aiding diagnosis. A retrospective study was performed for patients with pathologically proven PNS amyloidosis who also underwent MRI of the biopsied or excised nerve. MRI signal characteristics, nerve morphology, associated muscular denervation changes, and the presence of multifocal involvement were detailed. Pathology reports were reviewed to determine subtypes of amyloid. Charts were reviewed to gather patient demographics, neurological symptoms and radiologist interpretation. Four men and three women with a mean age of 62 ± 11 years (range 46-76) were identified. All patients had abnormal findings on EMG with mixed sensorimotor neuropathy. All lesions demonstrated diffuse multifocal neural involvement with T1 hypointensity, T2 hyperintensity, and variable enhancement on MRI. One lesion exhibited superimposed T2 hypointensity. Six of seven patients demonstrated associated muscular denervation changes. Peripheral nerve amyloidosis is rare, and the diagnosis is difficult because of insidious symptom onset, mixed sensorimotor neurologic deficits, and the potential for a wide variety of nerves affected. On MRI, peripheral nerve involvement is most commonly characterized by T1 hypointensity, T2 hyperintensity, variable enhancement, maintenance of the fascicular architecture with fusiform enlargement, multifocal involvement and muscular denervation changes. While this appearance mimics other inflammatory neuropathies, MRI can readily detect neural changes and direct-targeted biopsy, thus facilitating early diagnosis and appropriate management. (orig.)

  8. Proteomic analysis of highly prevalent amyloid A amyloidosis endemic to endangered island foxes.

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    Patricia M Gaffney

    Full Text Available Amyloid A (AA amyloidosis is a debilitating, often fatal, systemic amyloid disease associated with chronic inflammation and persistently elevated serum amyloid A (SAA. Elevated SAA is necessary but not sufficient to cause disease and the risk factors for AA amyloidosis remain poorly understood. Here we identify an extraordinarily high prevalence of AA amyloidosis (34% in a genetically isolated population of island foxes (Urocyon littoralis with concurrent chronic inflammatory diseases. Amyloid deposits were most common in kidney (76%, spleen (58%, oral cavity (45%, and vasculature (44% and were composed of unbranching, 10 nm in diameter fibrils. Peptide sequencing by mass spectrometry revealed that SAA peptides were dominant in amyloid-laden kidney, together with high levels of apolipoprotein E, apolipoprotein A-IV, fibrinogen-α chain, and complement C3 and C4 (false discovery rate ≤ 0.05. Reassembled peptide sequences showed island fox SAA as an 111 amino acid protein, most similar to dog and artic fox, with 5 unique amino acid variants among carnivores. SAA peptides extended to the last two C-terminal amino acids in 5 of 9 samples, indicating that near full length SAA was often present in amyloid aggregates. These studies define a remarkably prevalent AA amyloidosis in island foxes with widespread systemic amyloid deposition, a unique SAA sequence, and the co-occurrence of AA with apolipoproteins.

  9. Mutations in specific structural regions of immunoglobulin light chains are associated with free light chain levels in patients with AL amyloidosis.

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    Tanya L Poshusta

    Full Text Available BACKGROUND: The amyloidoses are protein misfolding diseases characterized by the deposition of amyloid that leads to cell death and tissue degeneration. In immunoglobulin light chain amyloidosis (AL, each patient has a unique monoclonal immunoglobulin light chain (LC that forms amyloid deposits. Somatic mutations in AL LCs make these proteins less thermodynamically stable than their non-amyloidogenic counterparts, leading to misfolding and ultimately the formation of amyloid fibrils. We hypothesize that location rather than number of non-conservative mutations determines the amyloidogenicity of light chains. METHODOLOGY/PRINCIPAL FINDINGS: We performed sequence alignments on the variable domain of 50 kappa and 91 lambda AL light chains and calculated the number of non-conservative mutations over total number of patients for each secondary structure element in order to identify regions that accumulate non-conservative mutations. Among patients with AL, the levels of circulating immunoglobulin free light chain varies greatly, but even patients with very low levels can have very advanced amyloid deposition. CONCLUSIONS: Our results show that in specific secondary structure elements, there are significant differences in the number of non-conservative mutations between normal and AL sequences. AL sequences from patients with different levels of secreted light chain have distinct differences in the location of non-conservative mutations, suggesting that for patients with very low levels of light chains and advanced amyloid deposition, the location of non-conservative mutations rather than the amount of free light chain in circulation may determine the amyloidogenic propensity of light chains.

  10. Surgical management of advanced ocular adnexal amyloidosis.

    Science.gov (United States)

    Patrinely, J R; Koch, D D

    1992-06-01

    Ocular adnexal amyloidosis is characterized by amyloid deposition within the deep connective tissue layers of the eyelids, conjunctiva, and anterior orbit. Management of advanced cases has traditionally been unsatisfactory, with either no surgery offered because of fear of hemorrhage or an en bloc resection performed of the entire involved area. We present two cases of advanced periorbital amyloidosis successfully managed by preserving the anatomic planes of the eyelids and meticulously debulking the deposits with a spooned curette. Lax eyelid tendons and aponeuroses were simultaneously repaired, and no sacrifice of eyelid tissues was necessary. One patient remained asymptomatic for 2 years after surgery before developing early reaccumulation in the lower eyelids. The other patient required additional eyelid debulking and ptosis revision 8 months after surgery, but was in stable condition at follow-up 2 years after surgery. This technique offers safe, easily repeatable, nondestructive treatment for advanced periocular amyloidosis.

  11. Recent Insights into the Pathogenesis of Type AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    J. C. H. van der Hilst

    2011-01-01

    Full Text Available The amyloidoses are a group of life-threatening diseases in which fibrils made of misfolded proteins are deposited in organs and tissues. The fibrils are stable, insoluble aggregates of precursor proteins that have adopted an antiparallel β-sheet structure. In type AA, or reactive, amyloidosis, the precursor protein of the fibrils is serum amyloid A (SAA. SAA is a 104-amino-acid protein that is produced in the liver in response to proinflammatory cytokines. Although the protein that is produced by the liver contains 104 amino acids, only the N-terminal 66–76 amino acids are found in amyloid fibrils. Furthermore, SAA has been shown to have an α-helical structure primarily. Thus, for SAA to be incorporated into an amyloid fibril, two processes have to occur: C-terminal cleavage and conversion into a β-sheet. Only a minority of patients with elevated SAA levels develop amyloidosis. Factors that contribute to the risk of amyloidosis include the duration and degree of SAA elevation, polymorphisms in SAA, and the type of autoinflammatory syndrome. In the Hyper-IgD syndrome, amyloidosis is less prevalent than in the other autoinflammatory diseases. In vitro work has shown that the isoprenoid pathway influences amyloidogenesis by farnesylated proteins. Although many proteins contain domains that have a potential for self-aggregation, amyloidosis is only a very rare event. Heat shock proteins (HSPs are chaperones that assist other proteins to attain, maintain, and regain a functional conformation. In this review, recent insights into the pathogenesis of amyloidosis are discussed, in addition to a new hypothesis for a role of HSPs in the pathogenesis of type AA.

  12. Churg-Strauss syndrome associated with AA amyloidosis: a case ...

    African Journals Online (AJOL)

    Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/μL), positive ...

  13. Ursodeoxycholic acid for treatment of cholestasis in patients with hepatic amyloidosis

    Directory of Open Access Journals (Sweden)

    Faust Dominik

    2009-01-01

    Full Text Available Background. Amyloidosis represents a group of different diseases characterized by extracellular accumulation of pathologic fibrillar proteins in various tissues and organs. Severe amyloid deposition in the liver parenchyma has extrahepatic involvement predominantly in the kidney or heart. We evaluated the effect of ursodeoxycholic acid, in four patients with severe hepatic amyloidosis of different etiologies, who presented with increased alkaline phosphatase and γ-glutamyl transferase. Case report. The study included four patients who presented with amyloidosis-associated intrahepatic cholestasis. Three of them had renal amyloidosis which developed 1-3 years before cholestasis occurred, the remaining one having intrahepatic cholestasis as the primary sign of the disease. Amyloidosis was identified from liver biopsies in all patients by its specific binding to Congo red and green birefringence in polarized light. The biochemical nature and the class of amyloid deposits were identified immunohistochemically. In addition to their regular treatment, the patients received 750 mg ursodeoxycholic acid per day. After 2-4 weeks all patients had a significant decrease of serum alkaline phosphatase and γ-glutamyl transferase, and their general status significantly improved. Conclusion. Treatment with ursodeoxycholic acid may be beneficial in patients with hepatic amyloidosis, and do extend indications for the use of ursodeoxycholic acid in amyloidotic cholestatic liver disease.

  14. Long-term follow-up after surgery in localized laryngeal amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Aldert J. C.; Hazenberg, Bouke P. C.; Dikkers, Frederik G.

    2016-01-01

    To study effectiveness of surgery and watchful waiting in localized laryngeal amyloidosis, retrospective case series. This retrospective study comprises all consecutive patients with localized laryngeal amyloidosis surgically treated in a tertiary hospital between 1994 and February 2016. Recurrence

  15. Long-term follow-up after surgery in localized laryngeal amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Aldert J. C.; Hazenberg, Bouke P. C.; Dikkers, Frederik G.

    To study effectiveness of surgery and watchful waiting in localized laryngeal amyloidosis, retrospective case series. This retrospective study comprises all consecutive patients with localized laryngeal amyloidosis surgically treated in a tertiary hospital between 1994 and February 2016. Recurrence

  16. Intraventricular dyssynchrony in light chain amyloidosis: a new mechanism of systolic dysfunction assessed by 3-dimensional echocardiography

    Directory of Open Access Journals (Sweden)

    Truran Seth

    2008-08-01

    Full Text Available Abstract Background Light chain amyloidosis (AL is a rare but often fatal disease due to intractable heart failure. Amyloid deposition leads to diastolic dysfunction and often preserved ejection fraction. We hypothesize that AL is associated with regional systolic dyssynchrony. The aim is to compare left ventricular (LV regional synchrony in AL subjects versus healthy controls using 16-segment dyssynchrony index measured from 3-dimension-al (3D echocardiography. Methods Cardiac 3D echocardiography full volumes were acquired in 10 biopsy-proven AL subjects (60 ± 3 years, 5 females and 10 healthy controls (52 ± 1 years, 5 females. The LV was subdivided into 16 segments and the time from end-diastole to the minimal systolic volume for each of the 16 segments was expressed as a percent of the cycle length. The standard deviations of these times provided a 16-segment dyssynchrony index (16-SD%. 16-SD% was compared between healthy and AL subjects. Results Left ventricular ejection fraction was comparable (control vs. AL: 62.4 ± 0.6 vs. 58.6 ± 2.8%, p = NS. 16-SD% was significantly higher in AL versus healthy subjects (5.93 ± 4.4 vs. 1.67 ± 0.87%, p = 0.003. 16-SD% correlated with left ventricular mass index (R 0.45, p = 0.04 but not to left ventricular ejection fraction. Conclusion Light chain amyloidosis is associated with left ventricular regional systolic dyssynchrony. Regional dyssynchrony may be an unrecognized mechanism of heart failure in AL subjects.

  17. Frictional amyloidosis in Oman - A study of ten cases

    Directory of Open Access Journals (Sweden)

    Mysore Venkataram

    2002-01-01

    Full Text Available Macular amyloidosis is an important cause for cutaneous pigmentation, the aetiology of which is poorly understood. Friction has recently been implicated the causation of early lesions, referred to as frictional amyloidosis. Confirmation of diagnosis by the detect on of amyloid using histochemical stains is inconsistent. Ten patients with pigmentation suggestive of macular amyloidosis were studied with detailed history, clinical examination, biopsy for histochemistry and electron microscopy. Nine out of ten patients had a history of prolonged friction with various objects such as bath sponges, brushes, towels, plant sticks and leaves. Amyloid was demonstrated by histochemical staining in only six out of ten cases. In the remaining four cases, amyloid was detected by electron microscopy. These consisted of aggregates of non-branching, extracellular, intertwining fibres measuring between 200-500 nm in length and between 20-25 nm in diameter. The study confirms the role of friction in the causation of this condition. Histochemical stains are not always successful in the detection of amyloid and electron microscopy is helpful for confirming its presence. The term frictional amyloidosis aptly describes the condition.

  18. Inhibitory effect of propolis on the development of AA amyloidosis.

    Science.gov (United States)

    Harata, Daichi; Tsuchiya, Yuya; Miyoshi, Tomoyuki; Yanai, Tokuma; Suzuki, Kazuhiko; Murakami, Tomoaki

    2018-04-01

    In the several types of amyloidoses, participation of oxidative stresses in the pathogenesis and the effect of antioxidants on amyloidosis have been reported. Meanwhile, the relationship between oxidative stresses and pathogenesis of amyloid A (AA) amyloidosis is still unclear. In this study, we used an antioxidant, Brazilian propolis, to investigate the inhibitory effects on AA amyloidosis. The results showed that AA deposition was inhibited by administration of propolis. Increased expression of antioxidant markers was detected in molecular biological examinations of mice treated with propolis. Although serum amyloid A (SAA) levels were strongly correlated with the immunoreactive area of AA deposits in the control group, the correlation was weaker in the propolis-treated groups. In addition, there were no changes in SAA levels between the control group and the propolis-treated groups. The results indicate that propolis, an antioxidant, may induce inhibitory effects against AA amyloidosis.

  19. Lichen amyloidosis induced on the upper back by long-term friction with a nylon towel.

    Science.gov (United States)

    Yoshida, Aki; Takahashi, Kazuhiro; Tagami, Hachiro; Akasaka, Toshihide

    2009-01-01

    Primary localized cutaneous amyloidosis can take several clinical forms. In Asia, macular amyloidosis caused by prolonged friction from a rough nylon towel or brush is common, and macular amyloidosis and lichen amyloidosis occasionally occur together, as so-called biphasic amyloidosis. We report herein the case of an 83-year-old Japanese man with lichen amyloidosis caused by prolonged nylon towel friction. This patient presented with unique symmetrical papular lesions on the upper back and shoulders. Lesions comprised slightly shiny, brownish, fine uniform papules approximately 0.5 mm in diameter, showing a partially linear, annular or rippled arrangement. Although this case was caused by prolonged nylon towel friction, no coexisting macular lesions could be found. To the best of our knowledge, this represents the first case of lichen amyloidosis induced by nylon towel friction in the absence of the macular amyloidosis that is usually observed in such cases. We instructed the patient to stop the habit of nylon towel rubbing and prescribed a topical steroid ointment and cepharanthine. After 6 months of treatment, papular lesions became clearly flatter.

  20. Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis.

    Science.gov (United States)

    Perlini, Stefano; Salinaro, Francesco; Musca, Francesco; Mussinelli, Roberta; Boldrini, Michele; Raimondi, Ambra; Milani, Paolo; Foli, Andrea; Cappelli, Francesco; Perfetto, Federico; Palladini, Giovanni; Rapezzi, Claudio; Merlini, Giampaolo

    2014-05-01

    Cardiac amyloidosis represents an archetypal form of restrictive heart disease, characterized by profound diastolic dysfunction. As ejection fraction is preserved until the late stage of the disease, the majority of patients do fulfill the definition of diastolic heart failure, that is, heart failure with preserved ejection fraction (HFpEF). In another clinical model of HFpEF, that is, pressure-overload hypertrophy, depressed midwall fractional shortening (mFS) has been shown to be a powerful prognostic factor. To assess the potential prognostic role of mFS in cardiac light-chain amyloidosis with preserved ejection fraction, we enrolled 221 consecutive untreated patients, in whom a first diagnosis of cardiac light-chain amyloidosis was concluded between 2008 and 2010. HFpEF was present in 181 patients. Patients in whom cardiac involvement was excluded served as controls (n = 121). Prognosis was assessed after a median follow-up of 561 days. When compared with light-chain amyloidosis patients without myocardial involvement, cardiac light-chain amyloidosis was characterized by increased wall thickness (P model. In cardiac light-chain amyloidosis with normal ejection fraction, depressed circumferential mFS, a marker of myocardial contractile dysfunction, is a powerful predictor of survival.

  1. Longitudinal study of experimental induction of AA amyloidosis in mice seeded with homologous and heterologous AA fibrils.

    Science.gov (United States)

    Muhammad, Naeem; Murakami, Tomoaki; Inoshima, Yasuo; Ishiguro, Naotaka

    2016-09-01

    To investigate pathogenesis and kinetics of experimentally induced murine AA amyloidosis seeded with homologous (murine) and heterologous (bovine) AA fibrils. Experimental AA amyloidosis was induced by administration of inflammatory stimulus and preformed AA fibrils to a total of 111 female C57/Black mice. In this longitudinal study, heterologous (bovine) as well as homologous (murine) AA fibrils were injected intraperitoneally to mice in various combinations. Re-stimulation was done at 120 or 300 days post first inoculation. To analyze the intensity of amyloid depositions in mice organs, immunohistochemical techniques and image J software were used. Assessment of cytokines level in sera was done using a Mouse Th1/Th2/Th17 Cytokine CBA Kit. Incidence and severity of AA amyloidosis were quite low in mice inoculated with heterologous bovine AA fibrils than homologous murine one. Homologous AA fibrils administration at first and second inoculation caused maximum amount of amyloid depositions and severe systemic form of amyloidosis. Increase in the level of pro-inflammatory cytokine IL-6 was observed after first inoculation, while second inoculation caused a further increase in the level of anti-inflammatory cytokine IL-10. AA amyloidosis can be induced by heterologous as well as homologous AA fibrils. Severity of AA amyloidosis induced with homologous AA fibrils is higher compared to heterologous AA fibrils.

  2. Breast amyloidosis in a female patient with multiple myeloma: Ultrasonographic and mammographic findings

    Energy Technology Data Exchange (ETDEWEB)

    Woo, Ah Rhm; Kim, Joon Mee; Nam, Se Jin [Inha University Hospital, Incheon (Korea, Republic of)

    2017-05-15

    Amyloidosis is a rare disease characterized by pathological protein deposits in organs or tissues. Breast involvement by amyloidosis is rare. We report a female patient with amyloidosis in the breast, with underlying multiple myeloma, which presents as a focal asymmetry on a screening mammogram and a low suspicious mass lesion by ultrasonography.

  3. Treatment with biologic agents improves the prognosis of patients with rheumatoid arthritis and amyloidosis.

    Science.gov (United States)

    Kuroda, Takeshi; Tanabe, Naohito; Kobayashi, Daisuke; Sato, Hiroe; Wada, Yoko; Murakami, Shuichi; Saeki, Takako; Nakano, Masaaki; Narita, Ichiei

    2012-07-01

    Reactive amyloid A (AA) amyloidosis is a serious and life-threatening systemic complication of rheumatoid arthritis (RA). We evaluated the safety of therapy with anti-tumor necrosis factor and anti-interleukin 6 biologic agents in RA patients with reactive AA amyloidosis, together with prognosis and hemodialysis (HD)-free survival, in comparison with patients with AA amyloidosis without such therapy. One hundred thirty-three patients with an established diagnosis of reactive AA amyloidosis participated in the study. Clinical data were assessed from patient records at the time of amyloid detection and administration of biologics. Survival was calculated from the date when amyloid was first demonstrated histologically or the date when biologic therapy was started until the time of death or to the end of 2010 for surviving patients. Patients who had started HD were selected for inclusion only after the presence of amyloid was demonstrated. Fifty-three patients were treated with biologic agents (biologic group) and 80 were not (nonbiologic group). Survival rate was significantly higher in the biologic group than in the nonbiologic group. Nine patients in the biologics group and 33 in the nonbiologic group started HD. Biologic therapy had a tendency for reduced risk of initiation of HD without any statistical significance. Patients with amyloidosis have a higher mortality rate, but the use of biologic agents can reduce risk of death. The use of biologics may not significantly influence the HD-free survival rate.

  4. Clinical outcomes and survival in AA amyloidosis patients

    Directory of Open Access Journals (Sweden)

    Yavuz Ayar

    Full Text Available Abstract Aim Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Methods: A total of 81 patients (51 males, 30 females with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. Results: Most of the patients (55.6% had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2% and rheumatoid arthritis (10.6% in the good outcome group and malignancy (20% in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively. Median duration of renal survival was 65 ± 3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p = 0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p = 0.006 and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p = 0.027 were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3 ± 16 months. Conclusion Our study indicated that familial Mediterranean fever constituted

  5. A large-sized bubbling appearance of the glomerular basement membrane in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis.

    Science.gov (United States)

    Suga, Norihiro; Miura, Naoto; Uemura, Yuko; Nakamura, Toshinobu; Morita, Hiroyuki; Banno, Shogo; Imai, Hirokazu

    2011-12-01

    We report an unusual pathological finding, a large-sized bubbling appearance of the glomerular basement membrane (GBM), in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis. The first renal biopsy specimen from 10 years ago, when systemic lupus erythematosus was diagnosed, demonstrated mild mesangial proliferation and subepithelial deposits (WHO classification: III + V). Light microscopy of the current biopsy using periodic acid methenamine silver (PAMS) stain demonstrated a large-sized bubbling appearance of the GBM; however, very weak immunoglobulin and complement deposition was observed in immunofluorescence studies. Routine electron microscopy demonstrated partial subendothelial expansion with electron-lucent materials, but no electron-dense deposits or amyloid fibrils. Electron microscopy with PAMS stain revealed electron-lucent endothelial scalloping, including some cellular components and microspheres in the GBM; however, it is not clear if these materials are derived from endothelial cells. One possibility is that these unique findings represent a recovery phase of lupus membranous nephritis; another is that these findings correspond to a new disease entity.

  6. Efficacy of etanercept in patients with AA amyloidosis secondary to rheumatoid arthritis.

    Science.gov (United States)

    Nakamura, T; Higashi, S; Tomoda, K; Tsukano, M; Baba, S

    2007-01-01

    The efficacy of biological therapies in rheumatoid arthritis (RA) is well known, but their hypothetical benefit in amyloid A (AA) amyloidosis secondary to RA still remains to be considered. We evaluated the efficacy and safety of etanercept in serum amyloid A (SAA) 1.3 allele Japanese patients with AA amyloidosis secondary to RA. Seven RA patients with histologically confirmed AA amyloidosis and renal involvement who were treated with etanercept were enrolled. They all had the SAA1.3 allele, which has been shown to be a risk factor not only for the association of AA amyloidosis but also for a poor prognosis in Japanese RA patients. Efficacy was assessed as a sustained decrease in RA inflammation and an amelioration of renal function. RA inflammation and AA amyloidosis were improved and stabilized after 43.4 +/- 16.5 weeks. At week 20 the number of tender (p = 0.017) and swollen (p = 0.017) joints, and levels of serum C-reactive protein (p = 0.018) and albumin (p = 0.045) had improved. The values for SAA, serum creatinine, calculated creatinine clearance, and proteinuria also ameliorated. No severe adverse events were observed. One patient eventually had to go on hemodialysis but her tolerance of etanercept remained stable. Etanercept can be used safely and effectively in AA amyloidosis secondary to RA with renal involvement, and is of clinical benefit in the short-term, even in patients on hemodialysis. It appears that SAA1.3 allele may be used as a clinical parameter for the introduction of etanercept in Japanese RA with AA amyloidosis.

  7. Fecal transmission of AA amyloidosis in the cheetah contributes to high incidence of disease

    Science.gov (United States)

    Zhang, Beiru; Une, Yumi; Fu, Xiaoying; Yan, Jingmin; Ge, FengXia; Yao, Junjie; Sawashita, Jinko; Mori, Masayuki; Tomozawa, Hiroshi; Kametani, Fuyuki; Higuchi, Keiichi

    2008-01-01

    AA amyloidosis is one of the principal causes of morbidity and mortality in captive cheetahs (Acinonyx jubatus), which are in danger of extinction, but little is known about the underlying mechanisms. Given the transmissible characteristics of AA amyloidosis, transmission between captive cheetahs may be a possible mechanism involved in the high incidence of AA amyloidosis. In this study of animals with AA amyloidosis, we found that cheetah feces contained AA amyloid fibrils that were different from those of the liver with regard to molecular weight and shape and had greater transmissibility. The infectious activity of fecal AA amyloid fibrils was reduced or abolished by the protein denaturants 6 M guanidine·HCl and formic acid or by AA immunodepletion. Thus, we propose that feces are a vehicle of transmission that may accelerate AA amyloidosis in captive cheetah populations. These results provide a pathogenesis for AA amyloidosis and suggest possible measures for rescuing cheetahs from extinction. PMID:18474855

  8. Amyloidosis Associated with Neoplastic Diseases

    African Journals Online (AJOL)

    1974-09-21

    Sep 21, 1974 ... adrenals, pancreas, a salivary gland, in a small scar in the myocardium, and in .... Biopsy specimens obtained through colonoscopy de- monstrated the ... cancer, found amyloidosis in 16, establishing an in- cidence of 0,4%.

  9. Imaging cardiac amyloidosis: a pilot study using 18F-florbetapir positron emission tomography

    International Nuclear Information System (INIS)

    Dorbala, Sharmila; Vangala, Divya; Semer, James; Strader, Christopher; Bruyere, John R.; Moore, Stephen C.; Di Carli, Marcelo F.; Falk, Rodney H.

    2014-01-01

    Cardiac amyloidosis, a restrictive heart disease with high mortality and morbidity, is underdiagnosed due to limited targeted diagnostic imaging. The primary aim of this study was to evaluate the utility of 18 F-florbetapir for imaging cardiac amyloidosis. We performed a pilot study of cardiac 18 F-florbetapir PET in 14 subjects: 5 control subjects without amyloidosis and 9 subjects with documented cardiac amyloidosis. Standardized uptake values (SUV) of 18 F-florbetapir in the left ventricular (LV) myocardium, blood pool, liver, and vertebral bone were determined. A 18 F-florbetapir retention index (RI) was computed. Mean LV myocardial SUVs, target-to-background ratio (TBR, myocardial/blood pool SUV ratio) and myocardial-to-liver SUV ratio between 0 and 30 min were calculated. Left and right ventricular myocardial uptake of 18 F-florbetapir were noted in all the amyloid subjects and in none of the control subjects. The RI, TBR, LV myocardial SUV and LV myocardial to liver SUV ratio were all significantly higher in the amyloidosis subjects than in the control subjects (RI median 0.043 min -1 , IQR 0.034 - 0.051 min -1 , vs. 0.023 min -1 , IQR 0.015 - 0.025 min -1 , P = 0.002; TBR 1.84, 1.64 - 2.50, vs. 1.26, IQR 0.91 - 1.36, P = 0.001; LV myocardial SUV 3.84, IQR 1.87 - 5.65, vs. 1.35, IQR 1.17 - 2.28, P = 0.029; ratio of LV myocardial to liver SUV 0.67, IQR 0.44 - 1.64, vs. 0.18, IQR 0.15 - 0.35, P = 0.004). The myocardial RI, TBR and myocardial to liver SUV ratio also distinguished the control subjects from subjects with transthyretin and those with light chain amyloid. 18 F-Florbetapir PET may be a promising technique to image light chain and transthyretin cardiac amyloidosis. Its role in diagnosing amyloid in other organ systems and in assessing response to therapy needs to be further studied. (orig.)

  10. A prospective study of nutritional status in immunoglobulin light chain amyloidosis

    DEFF Research Database (Denmark)

    Sattianayagam, PT; Lane, T; Fox, Z

    2013-01-01

    Weight loss is common in systemic immunoglobulin light chain amyloidosis but there are limited data on the impact of nutritional status on outcome. Using the Patient-Generated Subjective Global Assessment (PG-SGA) score, we prospectively examined nutritional status in 110 consecutive newly-diagno...

  11. Globular hepatic amyloid is highly sensitive and specific for LECT2 amyloidosis.

    Science.gov (United States)

    Chandan, Vishal S; Shah, Sejal S; Lam-Himlin, Dora M; Petris, Giovanni De; Mereuta, Oana M; Dogan, Ahmet; Torbenson, Michael S; Wu, Tsung-Teh

    2015-04-01

    Globular hepatic amyloid (GHA) is rare, and its clinical significance remains unclear. Recently, leukocyte chemotactic factor-associated amyloidosis (ALECT2) has been reported to involve the liver, showing a globular pattern. We reviewed 70 consecutive cases of hepatic amyloidosis to determine the prevalence and morphology of hepatic amyloid subtypes, especially ALECT2 and its association with GHA. Each case was reviewed for amyloid subtype (immunohistochemistry and/or mass spectrometry), its pattern (linear or globular), and distribution (vascular, perisinusoidal, or stromal). In addition, 24 cases of confirmed hepatic ALECT2 on mass spectrometry from our consultation files were also reviewed. LECT2 immunostaining was performed in 49 cases. Of the 70 cases, immunoglobulin light chain (AL) type was most common with 41 cases (59%), followed by transthyretin (ATTR) 15 cases (22%), 3 cases each of fibrinogen A (AFib) (4%), serum amyloid A (AA) (4%), and ALECT2 (4%), 2 cases of apolipoproteins (AApoA1) (3%), and 3 cases (4%) were unclassified. Three of our 70 cases (4%), with ALECT2, and all 24 cases (100%) of mass spectrometry-confirmed hepatic ALECT2 showed only GHA deposits in the hepatic sinusoids and portal tracts. Three (4%) other cases of AL type showed a focal globular pattern admixed with prominent linear amyloid. None of the other amyloid subtypes showed GHA. LECT2 immunostain was positive in all 27 cases (100%) of ALECT2 and negative in the other 22 non-ALECT2 cases (100%) (14 AL, 5 ATTR, 1 AA, 1 AFib, 1 AApoA1). Pure GHA is uncommon (4%) but is highly specific for ALECT2, and LECT2 immunostain is helpful in confirming this amyloid type.

  12. Cerebral amyloidosis associated with cognitive decline in autosomal dominant Alzheimer disease.

    Science.gov (United States)

    Wang, Fen; Gordon, Brian A; Ryman, Davis C; Ma, Shengmei; Xiong, Chengjie; Hassenstab, Jason; Goate, Alison; Fagan, Anne M; Cairns, Nigel J; Marcus, Daniel S; McDade, Eric; Ringman, John M; Graff-Radford, Neill R; Ghetti, Bernardino; Farlow, Martin R; Sperling, Reisa; Salloway, Steve; Schofield, Peter R; Masters, Colin L; Martins, Ralph N; Rossor, Martin N; Jucker, Mathias; Danek, Adrian; Förster, Stefan; Lane, Christopher A S; Morris, John C; Benzinger, Tammie L S; Bateman, Randall J

    2015-09-01

    To investigate the associations of cerebral amyloidosis with concurrent cognitive performance and with longitudinal cognitive decline in asymptomatic and symptomatic stages of autosomal dominant Alzheimer disease (ADAD). Two hundred sixty-three participants enrolled in the Dominantly Inherited Alzheimer Network observational study underwent neuropsychological evaluation as well as PET scans with Pittsburgh compound B. One hundred twenty-one participants completed at least 1 follow-up neuropsychological evaluation. Four composite cognitive measures representing global cognition, episodic memory, language, and working memory were generated using z scores from a battery of 13 standard neuropsychological tests. General linear mixed-effects models were used to investigate the relationship between baseline cerebral amyloidosis and baseline cognitive performance and whether baseline cerebral amyloidosis predicts cognitive change over time (mean follow-up 2.32 years ± 0.92, range 0.89-4.19) after controlling for estimated years from expected symptom onset, APOE ε4 allelic status, and education. In asymptomatic mutation carriers, amyloid burden was not associated with baseline cognitive functioning but was significantly predictive of longitudinal decline in episodic memory. In symptomatic mutation carriers, cerebral amyloidosis was correlated with worse baseline performance in multiple cognitive composites and predicted greater decline over time in global cognition, working memory, and Mini-Mental State Examination. Cerebral amyloidosis predicts longitudinal episodic memory decline in presymptomatic ADAD and multidomain cognitive decline in symptomatic ADAD. These findings imply that amyloidosis in the brain is an indicator of early cognitive decline and provides a useful outcome measure for early assessment and prevention treatment trials. © 2015 American Academy of Neurology.

  13. Pulmonary uptake of 198Au during liver scanning in two patients with amyloidosis

    International Nuclear Information System (INIS)

    Goebel, R.

    1976-01-01

    Lung uptake of 198 Au-colloid, injected for a liver scan, is demonstrated in two patients with systemic amyloidosis. The incidence, importance and mechanism of this phenomenon is discussed. (orig.) [de

  14. Therapeutic Benefits of Tocilizumab Vary in Different Organs of a Patient with AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    Masaru Matsui

    2014-01-01

    Full Text Available Systemic reactive AA amyloidosis is a life-threatening complication of chronic inflammatory diseases. Anti-interleukin-6 receptor, tocilizumab (TCZ, has been shown to improve clinical symptoms of patients with AA amyloidosis, accompanied with regression of the amyloid deposition. We report a case of AA amyloidosis evaluated by histology of multiple organs before and after TCZ treatment. A woman in her 60s with rheumatoid arthritis was referred to our hospital because of cardiac and renal dysfunction. A gastric and renal biopsy revealed the deposition of AA amyloid, and echocardiography revealed concentric left ventricular hypertrophy. Her estimated glomerular filtration rate was decreased to 8.6 mL/min/1.73 m2, and B-type natriuretic peptide, C-reactive protein, and serum amyloid A protein were significantly elevated. TCZ treatments markedly decreased her serum amyloid A protein and C-reactive protein levels, but hemodialysis was required 1 year later. Endoscopic gastric rebiopsy 3 years after initiation of TCZ treatments revealed the regression of amyloid deposition and echocardiography revealed improvement of her left ventricular hypertrophy. However, a renal rebiopsy revealed that the amyloid deposition had not regressed. In conclusion, these observations suggest that the therapeutic effects of TCZ can vary among organs in patients with AA amyloidosis.

  15. Long-term mortality outcome in patients with reactive amyloidosis associated with rheumatoid arthritis.

    Science.gov (United States)

    Kuroda, Takeshi; Tanabe, Naohito; Harada, Takashi; Murakami, Syuichi; Hasegawa, Hisashi; Sakatsume, Minoru; Nakano, Masaaki; Gejyo, Fumitake

    2006-07-01

    It is well established that amyloidosis is a serious clinical complication that can influence the prognosis of patients with rheumatoid arthritis (RA). The purpose of the study was to obtain information on the survival and the hemodialysis (HD) of patients with amyloidosis. Eighty patients (9 men and 71 women) who were diagnosed with amyloidosis by biopsy and definite or classical RA were studied retrospectively. The average duration of RA prior to the diagnosis of amyloidosis was 15.4+/-9.4 years. The average period from the diagnosis of amyloidosis to death was 67.4 months. Forty-nine patients died of the disease (32 cases with HD and 17 cases without HD). Thirty-one patients lived (7 cases with HD and 24 cases without HD). Regarding the survival of these patients, 49 (61.3%) of the 80 patients have died. Survival rate at 28 months was 75%; at 67 months, it was 50%; and at 111 months, it was down to 25%. Mortality rate was 11.9% per year. Survival rate in dialysis at 9.8 months was 75%; at 60.6 months, it dropped to 50%; and at 100.0 months, to 25%. As for patients' survival, high onset age of amyloidosis was the major determining factor for poor survival in these patients (ppatients also had poor survival (p=0.07). The long-term results were very encouraging to initiate HD in patients with end-stage renal disease due to reactive amyloidosis associated with RA.

  16. Early Impairment of Cardiac Function and Asynchronization of Systemic Amyloidosis with Preserved Ejection Fraction Using Two-Dimensional Speckle Tracking Echocardiography.

    Science.gov (United States)

    Huang, He; Jing, Xian-chao; Hu, Zhang-xue; Chen, Xi; Liu, Xiao-qin

    2015-12-01

    To observe the ventricular global and regional function of the patients with systemic amyloidosis using two-dimensional speckle tracking echocardiography. The study enrolled 31 consecutive biopsy-proved patients with systemic amyloidosis who underwent echocardiographic examination and EF ≥ 55% and 37 age- and gender-matched healthy controls. We compared systolic strain and strain rate, diastolic strain rate, time to peak strain, peak delay time in longitudinal, radial, circumferential directions in 16 left ventricular segments. The global peak systolic longitudinal and radial strain of left ventricle, peak systolic longitudinal strain and strain rate, diastolic strain rate of right ventricular free wall were also compared. (1) Global peak systolic longitudinal strain (GPSLS), peak systolic longitudinal strain (PSLS) and strain rate (PSLSR), peak early diastolic longitudinal strain rate (PELSR) in 16 segments were decreased in case (P < 0.05). (2) Peak systolic radial strain and strain rate of inferoseptum and inferolateral at the level of papillary muscle were lower (P < 0.05), and peak early diastolic radial strain rate (PERSR) was reduced (P < 0.05). (3) Peak early diastolic circumferential strain rate was lower (P < 0.05). (4) Time to peak systolic longitudinal, radial, circumferential strain was longer, and peak delay time at the same level retarded (P < 0.05). (5) Into right ventricular wall, PSLS and PSLSR at mid-segment, and PSLSR, PELSR, peak atrial systolic longitudinal strain rate (PALSR) at basal were reduced (P < 0.05). (6) Inverse correlation between interventricular septum (IVS) thickness and GPSLS and GPSRS was found (P < 0.05). Systolic and diastolic dysfunction existed in systemic amyloidosis with preserved EF. Mechanical contraction disorder may be one reason for systolic dysfunction. GPLSR and GPRSR were negatively related to IVS thickness. © 2015, Wiley Periodicals, Inc.

  17. Etanercept In the treatment of AA-amyloidosis in juvenile rheumatoid arthritis

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    H Mihels

    2004-01-01

    Full Text Available Objective. To study effect of etanercept, an antagonist of serum A amyloid production in the AA amyloidosis treatment in juvenile idiopathic arthritis (JIA. Material and methods. Etanercept was administered to all pts with AA amyloidosis admitted to Garmisch-Paterkirchen pediatric rheumatological clinic beginning with 2000. C-reactive protein (CRP, degree of proteinuria and serum creatinin were used as preliminary outcome measures. Results. 11 pts with seronegative JIA (6 boys and 5 girls were included in the study. Mean follow up duration was l,9±l.01 years. 8 children had systemic, 2 - olygoarticular and one - polyarticular disease onset. Before the study all pts had CRP level elevation (1,03-8,29 mg/dl, mean 4,53 mg/dl. 8 from 11 had marked proteinuria (364-7400 mg/24 hours, mean 1186 mg/24 hours. 2 from 11 had serum creatinin elevation. During etanercept treatment CRP level normalized in 2 and significantly decreased in 4 pts. Proteinuria decreased in 4 from 8 pts. Significant change of creatinin level was not achieved. One girl who did not have improvement during etanercept treatment showed CRP normalization and decrease of proteinuria when the drug was changed to infliximab. Conclusion. Treatment with etanercept provided improvement in almost 2/3 from 11 pts with JIA and AA amyloidosis. Etanercept may be the drug of choice in pts with normal creatinine level in the absence of proteinuria. When it fails another tumor necrosis factor antagonist such as infliximab should be used. It is necessary to extend volume and duration of the study to get more reliable data on etanercept efficacy in JIA pts with AA amyloidosis.

  18. [Hepatic amyloidosis as cause of severe intrahepatic cholestasis].

    Science.gov (United States)

    Gavilán, J C; Bermúdez, F J; Márquez, A; Sánchez-Carrillo, J J; González-Santos, P

    2003-01-01

    The liver is frequently involved by amyloidosis, but hyperbilirubinemia and liver failure are uncommon features. A mild elevation of the serum alkaline phosphatase value and, less frequently, hepatomegaly are the most common findings. Usually the patients have no symptoms related with the liver involvement; the clinical manifestation and the long term prognosis depends on the renal and cardiac disease. We report an unusual clinical presentation of primary amyloidosis in a previously asymptomatic 65 years old woman who was admitted to the hospital because of ictericia and ascitis mimicking a drug induced acute hepatic failure.

  19. Diagnosis of secondary amyloidosis in alkaptonuria.

    Science.gov (United States)

    Millucci, Lia; Ghezzi, Lorenzo; Bernardini, Giulia; Braconi, Daniela; Lupetti, Pietro; Perfetto, Federico; Orlandini, Maurizio; Santucci, Annalisa

    2014-09-26

    Alkaptonuria (AKU) is an inborn error of catabolism due to a deficient activity of homogentisate 1,2-dioxygenase. Patients suffer from a severe arthropathy, cardiovascular and kidney disease but other organs are affected, too. We found secondary amyloidosis as a life-threatening complication in AKU, thus opening new perspectives for its treatment. We proved that methotrexate and anti-oxidants have an excellent efficacy to inhibit the production of amyloid in AKU model chondrocytes. Owing to the progressive and intractable condition, it seems important to detect amyloid deposits at an early phase in AKU and the choice of specimens for a correct diagnosis is crucial. Ten AKU subjects were examined for amyloidosis; abdominal fat pad aspirates, labial salivary gland, cartilage and synovia specimens were analysed by CR, Th-T, IF, TEM. Amyloid was detected in only one abdominal fat pad specimen. However, all subjects demonstrated amyloid deposition in salivary glands and in other organ biopsies, indicating salivary gland as the ideal specimen for early amyloid detection in AKU. This is, at the best of our knowledge, the first report providing correct indications on the diagnosis of amyloidosis in AKU, thus offering the possibility of treatment of such co-morbidity to AKU patients. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_185.

  20. Outcomes after Bronchoscopic Procedures for Primary Tracheobronchial Amyloidosis: Retrospective Study of 6 Cases

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    Ihsan Alloubi

    2012-01-01

    Full Text Available Respiratory amyloidosis is a rare disease which refers to localized aberrant extracellular protein deposits within the airways. Tracheobronchial amyloidosis (TBA refers to the deposition of localized amyloid deposits within the upper airways. Treatments have historically focused on bronchoscopic techniques including debridement, laser ablation, balloon dilation, and stent placement. We present the outcomes after rigid bronchoscopy to remove the amyloid protein causing the airway obstruction in 6 cases of tracheobronchial amyloidosis. This is the first report of primary diffuse tracheobronchial amyloidosis in our department; clinical features, in addition to therapy in the treatment of TBA, are reviewed. This paper shows that, in patients with TBA causing airway obstruction, excellent results can be obtained with rigid bronchoscopy and stenting of the obstructing lesion.

  1. Outcomes after bronchoscopic procedures for primary tracheobronchial amyloidosis: retrospective study of 6 cases.

    Science.gov (United States)

    Alloubi, Ihsan; Thumerel, Matthieu; Bégueret, Hugues; Baste, Jean-Marc; Velly, Jean-François; Jougon, Jacques

    2012-01-01

    Respiratory amyloidosis is a rare disease which refers to localized aberrant extracellular protein deposits within the airways. Tracheobronchial amyloidosis (TBA) refers to the deposition of localized amyloid deposits within the upper airways. Treatments have historically focused on bronchoscopic techniques including debridement, laser ablation, balloon dilation, and stent placement. We present the outcomes after rigid bronchoscopy to remove the amyloid protein causing the airway obstruction in 6 cases of tracheobronchial amyloidosis. This is the first report of primary diffuse tracheobronchial amyloidosis in our department; clinical features, in addition to therapy in the treatment of TBA, are reviewed. This paper shows that, in patients with TBA causing airway obstruction, excellent results can be obtained with rigid bronchoscopy and stenting of the obstructing lesion.

  2. Acquired A amyloidosis from injection drug use presenting with atraumatic splenic rupture in a hospitalized patient: a case report

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    Hanks Douglas K

    2011-01-01

    Full Text Available Abstract Introduction Little is known about splenic rupture in patients who develop systemic acquired A amyloidosis. This is the first report of a case of atraumatic splenic rupture in a patient with acquired A amyloidosis from chronic injection drug use. Case presentation A 58-year-old Caucasian man with a long history of injection drug use, hospitalized for infective endocarditis, experienced atraumatic splenic rupture and underwent splenectomy. Histopathological and microbiological analyses of the splenic tissue were consistent with systemic acquired A amyloidosis, most likely from injection drug use, that led to splenic rupture without any recognized trauma or evidence of bacterial embolization to the spleen. Conclusion In patients with chronic inflammatory conditions, including the use of injection drugs, who experience acute onset of left upper quadrant pain, the diagnosis of atraumatic splenic rupture must be considered.

  3. Detection of amyloid in abdominal fat pad aspirates in early amyloidosis: Role of electron microscopy and Congo red stained cell block sections

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    Sumana Devata

    2011-01-01

    Full Text Available Background: Fine-needle aspiration biopsy (FNA of the abdominal fat pad is a minimally invasive procedure to demonstrate tissue deposits of amyloid. However, protocols to evaluate amyloid in fat pad aspirates are not standardized, especially for detecting scant amyloid in early disease. Materials and Methods: We studied abdominal fat pad aspirates from 33 randomly selected patients in whom subsequent tissue biopsy, autopsy, and/or medical history for confirmation of amyloidosis (AL were also available. All these cases were suspected to have early AL, but had negative results on abdominal fat pad aspirates evaluated by polarizing microscopy of Congo Red stained sections (CRPM. The results with CRPM between four reviewers were compared in 12 cases for studying inter observer reproducibility. 24 cases were also evaluated by ultrastructural study with electron microscopy (EM. Results: Nine of thirty-three (27% cases reported negative by polarizing microscopy had amyloidosis. Reanalysis of 12 mixed positive-negative cases, showed considerable inter-observer variability with frequent lack of agreement between four observers by CRPM alone (Cohen′s Kappa index of 0.1, 95% CI -0.1 to 0.36. EM showed amyloid in the walls of small blood vessels in fibroadipose tissue in four out of nine cases (44% with amyloidosis. Conclusion: In addition to poor inter-observer reproducibility, CRPM alone in cases with scant amyloid led to frequent false negative results (9 out of 9, 100%. For improved detection of AL, routine ultrastructural evaluation with EM of fat pad aspirates by evaluating at least 15 small blood vessels in the aspirated fibroadipose tissue is recommended. Given the high false negative rate for CRPM alone in early disease, routine reflex evaluation with EM is highly recommended to avert the invasive option of biopsying various organs in cases with high clinical suspicion for AL.

  4. Senile amyloidosis and neuron binding antibody in the aging Syrian hamster

    International Nuclear Information System (INIS)

    Blumenthal, H.T.; Musacchia, X.J.

    1985-01-01

    The effects of age, sex, and irradiation on the genesis of amyloidosis, neuron-binding antibody (NBA), and the concomitant appearance of these two phenomena were studied in a colony of Syrian hamsters. In nonirradiated controls amyloidosis increased in prevalence with age after 12 months, and prevalence was higher in females than in males. Irradiation had the effect of advancing the appearance of amyloidosis to the 7-12 months group but did not intensify the amyloidotic process. IgG binding to the nucleus or cytoplasm of neurons was rare, and, despite the fact that IgM and IgA binding to these structures was present in about one-third of the animals, there was neither an aging nor an irradiation effect. The only statistically significant findings with respect to the concomitant occurrence of amyloid and NBA were negative correlations between nuclear IgM and IgA binding and amyloidosis. Of the various species thus far studied, the hamster is the first in which there has been no aging effect in respect to NBA

  5. Long-term TNF-alpha blockade in patients with amyloid A amyloidosis complicating rheumatic diseases.

    Science.gov (United States)

    Fernández-Nebro, Antonio; Olivé, Alejandro; Castro, María Carmen; Varela, Angela Herranz; Riera, Elena; Irigoyen, Maria V; García de Yébenes, María Jesús; García-Vicuña, Rosario

    2010-05-01

    To evaluate the effectiveness and safety of anti-tumor necrosis factor therapy in patients with amyloid A amyloidosis. Multicenter, controlled, dynamic prospective cohort study of 36 patients with amyloid A amyloidosis (94% kidney involvement) treated with anti-tumor necrosis factor agents (drug exposure of 102.97 patient-years). As an external control group, 35 propensity score-matched non-amyloid patients were chosen from the Base de Datos de Productos Biológicos de la Sociedad Española de Reumatología registry. The end points were kidney response and progression, anti-tumor necrosis factor continuation rate, patient survival, and adverse events. At the end of follow-up, a kidney response was observed in 12 of 22 patients (54.5%) and a kidney progression was observed in 6 of 36 patients (17%). The kidney amyloidosis remained stable in 16 of 36 patients (44%). The level of acute phase reactants diminished but did not reach the normal level. The continuation rates of anti-tumor necrosis factor drugs among patients with amyloid A amyloidosis after 1, 2, 3, and 4 or more years were 80%, 80%, 61%, and 52%, respectively, comparable to controls. The 5-year cumulative survival of amyloid A amyloidosis cases was 90.6%, and the 10-year survival was 78.5%. In a multivariate Cox regression analysis, the duration of amyloidosis and the level of proteinuria at the onset of anti-tumor necrosis factor treatment were independent predictors of treatment failure, whereas the level of proteinuria was the only factor that predicts mortality. Most adverse events were similar in both groups, although the number of infections was 3 times higher in amyloid A amyloidosis cases. Anti-tumor necrosis factor drugs are effective in treating amyloid A amyloidosis, although they might increase the risk of infection. Copyright 2010 Elsevier Inc. All rights reserved.

  6. A Concise Review of Amyloidosis in Animals

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    Moges Woldemeskel

    2012-01-01

    Full Text Available Amyloidosis refers to a group of protein misfolding diseases characterized by deposition of a particular amyloid protein in various organs and tissues of animals and humans. Various types and clinical forms of amyloidosis, in which the pathology and pathogenesis is diverse depending upon the underlying causes and species affected, are reported in domestic and wild animals. The clinical findings are also quite variable consequent to the variation of the tissues and organs involved and the extent of functional disruption of the affected organs in various animal species. The affected organs may be enlarged and exhibit variable pallor grossly, or the amyloid deposit may be discernible only after microscopic examination of the affected tissues. Amyloid appears as a pale eosinophilic homogenous extracellular deposit in tissues. However, microscopic examination and Congo red staining with green birefringence under polarized light are needed to confirm amyloid and differentiate it from other apparently similar extracellular deposits such as collagen and fibrin. Identifying the type of amyloid deposit needs immunohistochemical staining, ultrastructural characterization of the amyloid fibril, and if feasible also genetic studies of the involved species for clinical and prognostic purposes. This paper provides a concise review of the occurrence of amyloidosis in domestic and wild animals.

  7. Renal amyloidosis in a child with sickle cell anemia.

    Science.gov (United States)

    Simşek, Behçet; Bayazit, Aysun K; Ergin, Melek; Soran, Mustafa; Dursun, Hasan; Kilinc, Yurdanur

    2006-06-01

    The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.

  8. Two distinct clinical courses of renal involvement in rheumatoid patients with AA amyloidosis.

    Science.gov (United States)

    Uda, Hiroshi; Yokota, Akira; Kobayashi, Kumiko; Miyake, Tadao; Fushimi, Hiroaki; Maeda, Akira; Saiki, Osamu

    2006-08-01

    We conducted a prospective study to investigate whether a correlation exists between the clinical course of renal involvement and the pathological findings of renal amyloidosis in patients with rheumatoid arthritis (RA). Patients with RA of more than 5 years' duration and who did not show renal manifestations were selected and received a duodenal biopsy for the diagnosis of amyloidosis. After the diagnosis of AA amyloidosis, patients received a renal biopsy, and patterns of amyloid deposition were examined. We followed the renal functions (serum levels of blood urea nitrogen and creatinine) of patients diagnosed with AA amyloidosis for 5 years. We diagnosed 53 patients with AA amyloidosis and monitored the renal function of 38 of them for > 5 years. The histological patterns were examined; in the 38 patients there were appreciable variations in the patterns of amyloid deposition. In 27 patients, amyloid deposits were found exclusively in the glomerulus (type 1). In the other 11 patients, however, amyloid deposits were found selectively around blood vessels and were totally absent in the glomerulus (type 2). In type 1 patients with glomerular involvement, renal function deteriorated rapidly regardless of disease state; most patients received hemodialysis. In type 2 patients with purely vascular involvement, however, renal function did not deteriorate significantly. In patients with RA and AA amyloidosis, 2 distinct clinical courses in terms of renal involvement were identified. It is suggested that renal function does not deteriorate when amyloid deposition is totally lacking in the glomerulus.

  9. Peptide p5 binds both heparinase-sensitive glycosaminoglycans and fibrils in patient-derived AL amyloid extracts

    Energy Technology Data Exchange (ETDEWEB)

    Martin, Emily B.; Williams, Angela [Department of Medicine, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States); Heidel, Eric [Department of Surgery, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States); Macy, Sallie [Department of Medicine, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States); Kennel, Stephen J. [Department of Medicine, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States); Department of Radiology, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States); Wall, Jonathan S., E-mail: jwall@utmck.edu [Department of Medicine, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States); Department of Radiology, University of Tennessee Graduate School of Medicine, 1924 Alcoa Highway, Knoxville, TN 37922 (United States)

    2013-06-21

    Highlights: •Polybasic peptide p5 binds human light chain amyloid extracts. •The binding of p5 with amyloid involves both glycosaminoglycans and fibrils. •Heparinase treatment led to a correlation between p5 binding and fibril content. •p5 binding to AL amyloid requires electrostatic interactions. -- Abstract: In previously published work, we have described heparin-binding synthetic peptides that preferentially recognize amyloid deposits in a mouse model of reactive systemic (AA) amyloidosis and can be imaged by using positron and single photon emission tomographic imaging. We wanted to extend these findings to the most common form of visceral amyloidosis, namely light chain (AL); however, there are no robust experimental animal models of AL amyloidosis. To further define the binding of the lead peptide, p5, to AL amyloid, we characterized the reactivity in vitro of p5 with in situ and patient-derived AL amyloid extracts which contain both hypersulfated heparan sulfate proteoglycans as well as amyloid fibrils. Histochemical staining demonstrated that the peptide specifically localized with tissue-associated AL amyloid deposits. Although we anticipated that p5 would undergo electrostatic interactions with the amyloid-associated glycosaminoglycans expressing heparin-like side chains, no significant correlation between peptide binding and glycosaminoglycan content within amyloid extracts was observed. In contrast, following heparinase I treatment, although overall binding was reduced, a positive correlation between peptide binding and amyloid fibril content became evident. This interaction was further confirmed using synthetic light chain fibrils that contain no carbohydrates. These data suggest that p5 can bind to both the sulfated glycosaminoglycans and protein fibril components of AL amyloid. Understanding these complex electrostatic interactions will aid in the optimization of synthetic peptides for use as amyloid imaging agents and potentially as

  10. Silicone Stent Placement for Primary Tracheal Amyloidosis Accompanied by Cartilage Destruction

    OpenAIRE

    Ryu, Duck Hyun; Eom, Jung Seop; Jeong, Ho Jung; Kim, Jung Hoon; Lee, Ji Eun; Jun, Ji Eun; Song, Dae Hyun; Han, Joungho; Kim, Hojoong

    2014-01-01

    Primary tracheal amyloidosis (PTA) can lead to airway obstructions, and patients with severe PTA should undergo bronchoscopic interventions in order to maintain airway patency. Focal airway involvements with amyloidosis can only be treated with mechanical dilatation. However, the PTA with diffused airway involvements and concomitant cartilage destructions requires stent placement. Limited information regarding the usefulness of silicone stents in patients with PTA has been released. Therefore...

  11. Pretargeting immunotherapy: a novel treatment approach for systemic amyloidosis.

    Science.gov (United States)

    Wall, Jonathan S; Foster, James S; Martin, Emily B; Kennel, Stephen J

    2017-09-01

    The amyloidoses are a complex group of disorders characterized by the deposition of proteinaceous amyloid fibrils in vital organs. The deposits are nonimmunogenic and may be composed of one of more than 35 proteins. We have developed a two-stage immunotherapeutic approach using peptides that recognize most, if not all, amyloid deposits to facilitate amyloid clearance. In the first embodiment, we have developed a bifunctional peptope to enhance and expand the utility of currently available antibodies. In the second, we have generated peptide-reactive antibodies that can be targeted to the amyloid deposits by peptides thereby providing alternative reagents for immunotherapy of amyloidosis. These technologies provide tools for treating the many forms of amyloid disease, restoring organ function and enhancing patient survival.

  12. Primary localized amyloidosis of the eyelid

    DEFF Research Database (Denmark)

    Olsen, K E; Sandgren, O; Sletten, K

    1996-01-01

    Primary localized amyloidosis has been described in many different organs in the body. Studies by immunohistochemical techniques have suggested an immunoglobulin light chain origin of the amyloid material. Only in a limited number of cases has the amyloid protein been characterized by amino acid...

  13. Axoval neuropathy as initial manifestation of primary amyloidosis: report of a case submitted to bone marrow transplantation Neuropatia axonal como manifestação inicial de amiloidose primária: relato de caso submetido a transplante de medula óssea

    Directory of Open Access Journals (Sweden)

    Orlando G. Povoas Barsottini

    2004-09-01

    Full Text Available Amyloidosis is a syndrome characterized by deposition of a highly insoluble protein material in the extracellular space that may affect several organs. It may be generalized and idiopathic (primary amyloidosis. We describe the case of a 48 years-old woman with axonal neuropathy associated with proteinuria, whose final investigation resulted in diagnosis of primary amyloidosis (AL. She was submitted to autologous bone marrow transplantation. We discuss some aspects related to diagnosis of neuropathy and current treatment of AL.A amiloidose é uma síndrome caracterizada pela deposição no meio extracelular de material protéico altamente insolúvel e que pode afetar vários órgãos. Pode ocorrer como doença generalizada e pode ser idiopática (amiloidose primária. Descrevemos o caso de mulher de 48 anos com neuropatia axonal associada a proteinúria na qual a investigação final resultou no diagnóstico de amiloidose primária (AL. Foi submetida a transplante autólogo de medula óssea sem complicações. Discutiremos aspectos relacionados ao diagnóstico da neuropatia e do tratamento atual da AL.

  14. Renal Amyloidosis Associated With 5 Novel Variants in the Fibrinogen A Alpha Chain Protein

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    Dorota Rowczenio

    2017-05-01

    Discussion: We report 6 novel mutations in the FGA gene: 5 were associated with renal fibrinogen A alpha chain amyloidosis and 1 was found to be incidental to light-chain amyloid deposits discovered in a patient with a plasma cell dyscrasia. Clinical awareness and suspicion of hereditary amyloidosis corroborated by genetic analysis and adequate typing using combined immunohistochemistry and laser microdissection and mass spectrometry is valuable to avoid misdiagnosis, especially when a family history of amyloidosis is absent.

  15. Serum immunoglobulin free light-chain measurement in primary amyloidosis: prognostic value and correlations with clinical features.

    Science.gov (United States)

    Kumar, Shaji; Dispenzieri, Angela; Katzmann, Jerry A; Larson, Dirk R; Colby, Colin L; Lacy, Martha Q; Hayman, Suzanne R; Buadi, Francis K; Leung, Nelson; Zeldenrust, Steve R; Ramirez-Alvarado, Marina; Clark, Raynell J; Kyle, Robert A; Rajkumar, S Vincent; Gertz, Morie A

    2010-12-09

    Immunoglobulin free light chains (FLCs) are the precursors of amyloid fibrils in primary amyloidosis (AL). We studied the relationship between FLC levels and clinical features in 730 patients with newly diagnosed AL. The plasma cell clone was λ in 72% patients, and κ in 28% patients. κ-AL had more GI tract and liver involvement, where as renal involvement was more with λ-AL. While the overall survival (OS) was similar for κ and λ-AL, the median OS for those without an identifiable serum heavy chain was significantly shorter (12.6 vs 29.9 months; P = .02). The OS was shorter among those with a higher dFLC (involved FLC-uninvolved FLC; κ > 29.4 mg/dL or λ > 18.2 mg/dL using median for cutoff); 10.9 vs 37.1 months; P analysis, dFLC was independent of other prognostic factors. The type of light chain impacts the spectrum of organ involvement and the FLC burden correlates with survival in AL.

  16. Amyloidosis: A story of how inframammary erosions eclipsed inconspicuous periorbital ecchymoses

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    Andrew Kelsey, MD

    2016-03-01

    Full Text Available Systemic amyloidosis is a rare disease that can be rapidly progressive due to widespread organ involvement. There are well-described renal, cardiac, pulmonary, neurological, and dermatologic findings. Here, we outline one patient’s experience with the condition from presentation to making the diagnosis. She presented with pathognomonic dermatologic findings including pinch purpura and ecchymoses found in the skin folds.

  17. Fibrils from designed non-amyloid-related synthetic peptides induce AA-amyloidosis during inflammation in an animal model.

    Directory of Open Access Journals (Sweden)

    Per Westermark

    Full Text Available BACKGROUND: Mouse AA-amyloidosis is a transmissible disease by a prion-like mechanism where amyloid fibrils act by seeding. Synthetic peptides with no amyloid relationship can assemble into amyloid-like fibrils and these may have seeding capacity for amyloid proteins. PRINCIPAL FINDINGS: Several synthetic peptides, designed for nanotechnology, have been examined for their ability to produce fibrils with Congo red affinity and concomitant green birefringence, affinity for thioflavin S and to accelerate AA-amyloidosis in mice. It is shown that some amphiphilic fibril-forming peptides not only produced Congo red birefringence and showed affinity for thioflavin S, but they also shortened the lag phase for systemic AA-amyloidosis in mice when they were given intravenously at the time of inflammatory induction with silver nitride. Peptides, not forming amyloid-like fibrils, did not have such properties. CONCLUSIONS: These observations should caution researchers and those who work with synthetic peptides and their derivatives to be aware of the potential health concerns.

  18. Amyloidoses as seen by the Rheumatologist

    Directory of Open Access Journals (Sweden)

    J.Ch Gerster

    2011-09-01

    Full Text Available Amyloidosis is due to extracellular deposition in various organs and tissues of amorphous materials made of protein fibrils, whose thickness is 10 nm. Seventeen different amyloid fibrils are known (1. Amyloidosis can be localised or systemic. There are 4 systemic amyloidoses (2: Familial amyloidosis with mutated transthyretin. Primary, paraprotein associated, amyloidosis AL. Secondary AA amyloidosis in long- standing inflammation. β2-microglobulin...

  19. Age-dependent cognitive dysfunction in untreated hereditary transthyretin amyloidosis.

    Science.gov (United States)

    Martins da Silva, Ana; Cavaco, Sara; Fernandes, Joana; Samões, Raquel; Alves, Cristina; Cardoso, Márcio; Kelly, Jeffery W; Monteiro, Cecília; Coelho, Teresa

    2018-02-01

    Central nervous system (CNS) involvement in hereditary transthyretin (TTR) amyloidosis has been described in patients whose disease course was modified by liver transplant. However, cognitive dysfunction has yet to be investigated in those patients. Moreover, CNS involvement in untreated patients or asymptomatic mutation carriers remains to be studied. A series of 340 carriers of the TTRVal30Met mutation (180 symptomatic and 160 asymptomatic) underwent a neuropsychological assessment, which included the Dementia Rating Scale-2 (DRS-2), auditory verbal learning test, semantic fluency, phonemic fluency, and trail making test. Cognitive deficits were identified at the individual level, after adjusting the neuropsychological test scores for demographic characteristics (sex, age, and education), based on large national normative data. The presence of cognitive dysfunction was determined by deficit in DRS-2 and/or multiple cognitive domains. Participants were also screened for depression based on a self-report questionnaire. The frequency of cognitive dysfunction was higher (p = 0.003) in symptomatic (9%) than in asymptomatic (2%) carriers. Among older carriers (≥ 50 years), the frequency of cognitive dysfunction was higher (p hereditary TTR amyloidosis patients with peripheral polyneuropathy, even in the early stages of the disease.

  20. Guideline of transthyretin-related hereditary amyloidosis for clinicians

    Directory of Open Access Journals (Sweden)

    Ando Yukio

    2013-02-01

    Full Text Available Abstract Transthyretin amyloidosis is a progressive and eventually fatal disease primarily characterized by sensory, motor, and autonomic neuropathy and/or cardiomyopathy. Given its phenotypic unpredictability and variability, transthyretin amyloidosis can be difficult to recognize and manage. Misdiagnosis is common, and patients may wait several years before accurate diagnosis, risking additional significant irreversible deterioration. This article aims to help physicians better understand transthyretin amyloidosis—and, specifically, familial amyloidotic polyneuropathy—so they can recognize and manage the disease more easily and discuss it with their patients. We provide guidance on making a definitive diagnosis, explain methods for disease staging and evaluation of disease progression, and discuss symptom mitigation and treatment strategies, including liver transplant and several pharmacotherapies that have shown promise in clinical trials.

  1. Profile of renal AA amyloidosis in older and younger individuals: a single-centre experience.

    Science.gov (United States)

    Erdogmus, Siyar; Kendi Celebi, Zeynep; Akturk, Serkan; Kumru, Gizem; Duman, Neval; Ates, Kenan; Erturk, Sehsuvar; Nergizoglu, Gokhan; Kutlay, Sim; Sengul, Sule; Keven, Kenan

    2018-05-18

    In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis. This is a retrospective study involving 53 patients who were diagnosed with AA amyloidosis by kidney biopsy from 2006 to 2016. In all patients, kidney biopsies were performed due to asymptomatic proteinuria, nephrotic syndrome and/or renal insufficiency. The patients were separated into two groups on the basis of age (group I: ≥60 years and group II: AA amyloidosis were as follows: FMF 16 (50%), bronchiectasis 7 (23%), chronic osteomyelitis 2 (6%), inflammatory bowel disease 2 (6%), rheumatoid arthritis 2 (6%), ankylosing spondylitis 1 (3%) and unknown aetiology 2 (6%). The underlying disorders of AA amyloidosis in group II patients were as follows: FMF 17 (81%), Behcet's disease 1 (5%) and unknown aetiology 3 (14%). No statistically significant differences were detected between two groups with regard to systolic and diastolic blood pressures, albumin, proteinuria and lipids. The combination of chronic kidney disease and nephrotic syndrome was the most common clinical presentation in group I (73%) and group II (43%) (p = .05). Compared to the group II, estimated glomerular filtration rate was significantly lower in group I at the time of kidney biopsy (p = .003). At 12-month follow-up, 61% of the group I and 33% of the group II developed end-stage kidney disease requiring dialysis, while 11% of the group I died. Our results indicated that renal AA amyloidosis is a rare disease in advanced age patients. At baseline and follow-up period, advanced age

  2. Anakinra induces complete remission of nephrotic syndrome in a patient with familial mediterranean fever and amyloidosis.

    Science.gov (United States)

    Sevillano, Ángel M; Hernandez, Eduardo; Gonzalez, Esther; Mateo, Isabel; Gutierrez, Eduardo; Morales, Enrique; Praga, Manuel

    2016-01-01

    Renal amyloidosis is one of the most severe complications of familial Mediterranean fever (FMF). Colchicine has reduced the incidence of this complication, which now only appears in untreated, under-treated and resistant patients, but it is usually ineffective in patients with advanced amyloidosis. Here we report a patient with FMF and biopsy-proven amyloidosis who presented with nephrotic syndrome despite colchicine treatment. Anakinra (an interleukin-1β inhibitor) was started and a dramatic complete remission of nephrotic syndrome was observed in the following months. Anakinra can be an effective treatment for FMF patients with severe secondary amyloidosis. Copyright © 2015 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  3. Cases of a Borderline Pathology That Can Mimic Bladder Cancer: Primary Amyloidosis of Urinary Bladder

    Directory of Open Access Journals (Sweden)

    Cemal Selçuk İşoğlu

    2015-06-01

    Full Text Available Amyloidosis is a disease characterised by accumulation of a fibrillar protein called amyloid in the extracellular space. The kidneys, ureters and the bladder can be affected in the urinary tract. However, primary amyloidosis of bladder is a rare entity. Macroscopic hematuria could be the first and only symptom of primary amyloidosis of the bladder; therefore, it has similar findings with urinary tract malignancies. Histopathological evaluation is mandatory for the diagnosis. Follow-up should always include cystoscopic evaluation as recurrence is expected in the natural course.

  4. Effects of Biologic Agents in Patients with Rheumatoid Arthritis and Amyloidosis Treated with Hemodialysis.

    Science.gov (United States)

    Kuroda, Takeshi; Tanabe, Naohito; Nozawa, Yukiko; Sato, Hiroe; Nakatsue, Takeshi; Kobayashi, Daisuke; Wada, Yoko; Saeki, Takako; Nakano, Masaaki; Narita, Ichiei

    Objective Our objective was to examine the safety and effects of therapy with biologics on the prognosis of rheumatoid arthritis (RA) patients with reactive amyloid A (AA) amyloidosis on hemodialysis (HD). Methods Twenty-eight patients with an established diagnosis of reactive AA amyloidosis participated in the study. The survival was calculated from the date of HD initiation until the time of death, or up to end of June 2015 for the patients who were still alive. HD initiation was according to the program of HD initiation for systemic amyloidosis patients associated with RA. Results Ten patients had been treated with biologics before HD initiation for a mean of 28.2 months (biologic group), while 18 had not (non-biologic group). HD was initiated in patients with similar characteristics except for the tender joint count, swollen joint count, and disease activity score (DAS)28-C-reactive protein (CRP). History of biologics showed that etanercept was frequently used for 8 patients as the first biologic. There was no significant difference in the mortality rate according to a Kaplan-Meier analysis (p=0.939) and or associated risk of death in an age-adjusted Cox proportional hazards model (p=0.758) between both groups. Infections were significantly more frequent causes of death in the biologic group than in the non-biologic group (p=0.021). However, treatment with biologics improved the DAS28-CRP score (p=0.004). Conclusion Under the limited conditions of AA amyloidosis treated with HD, the use of biologics might affect infection and thus may not improve the prognosis. Strict infection control is necessary for the use of biologics with HD to improve the prognosis.

  5. Clinical characteristics and SAP scintigraphic findings in 10 patients with AGel amyloidosis.

    Science.gov (United States)

    Rowczenio, Dorota; Tennent, Glenys A; Gilbertson, Janet; Lachmann, Helen J; Hutt, David F; Bybee, Alison; Hawkins, Philip N; Gillmore, Julian D

    2014-12-01

    The clinical features of hereditary gelsolin (AGel) amyloidosis include corneal lattice dystrophy, distal sensorimotor, cranial neuropathy and cutis laxa. To date, four mutations of the gelsolin (GSN) gene encoding the following variants have been identified as the cause of this malady; p.D214N, p.D214Y, p.G194R and p.N211K (this nomenclature includes the 27-residue signal peptide). Interestingly, the latter two variants are associated exclusively with a renal amyloidosis phenotype. Here we report the clinical features in 10 patients with AGel amyloidosis associated with the p.D214N mutation, all of whom underwent whole body (123)I-SAP scintigraphy and were followed up in a single UK Centre for a prolonged period. Two patients, from the same kindred presented with proteinuria; eight subjects had a characteristic AGel amyloidosis phenotype including cranial neuropathy and/or corneal lattice dystrophy. (123)I-SAP scintigraphy revealed substantial renal amyloid deposits in all 10 patients, including those with preserved renal function, and usually without tracer uptake into other visceral organs. (123)I-SAP scintigraphy is a non-invasive technique that aids early diagnosis of patients with this rare disease, especially those who lack a family history and/or present with an unusual clinical phenotype.

  6. Laryngeal amyloidosis with laryngocele: MRI and CT

    International Nuclear Information System (INIS)

    Arslan, A.; Ceylan, N.; Cetin, A.; Demirci, A.

    1998-01-01

    A case of laryngeal amyloidosis associated with a laryngocele is reported. Preoperative CT showed diffuse thickening of the epiglottis, aryepiglottic folds and false vocal cords with well-defined calcific foci. MRI revealed contrast enhancement and increased signal intensity on T2-weighted images. (orig.)

  7. Diagnostic performance of I-123-labeled serum amyloid P component scintigraphy in patients with amyloidosis

    NARCIS (Netherlands)

    Hazenberg, BPC; van Rijswijk, MH; Piers, DA; Lub-de Hooge, MN; Vellenga, E; Haagsma, EB; Hawkins, PN; Jager, PL

    Purpose: To assess the diagnostic accuracy and additional information provided by I-123-labeled serum amyloid P component ( SAP) scintigraphy in patients with systemic and localized amyloidosis. Subjects and Methods: I-123-labeled human SAP was injected intravenously into 20 controls and 189

  8. Drawing attention to a neglected injecting-related harm: a systematic review of AA amyloidosis among people who inject drugs.

    Science.gov (United States)

    Harris, Magdalena; Brathwaite, Rachel; Scott, Jenny; Gilchrist, Gail; Ciccarone, Dan; Hope, Vivian; McGowan, Catherine R

    2018-04-26

    Chronic skin and soft tissue infections (SSTI) among people who inject drugs (PWID) can lead to AA amyloidosis: a serious, yet neglected, multi-organ disease. We aim to synthesize findings on the epidemiology, risk factors, clinical outcomes, screening recommendations and challenges to treatment for AA amyloidosis among PWID. A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). We searched the following bibliographic databases in July 2017: CINAHL Plus, Embase, Global Health, MEDLINE, PsycEXTRA, PsycINFO and SCOPUS. Studies were included if they investigated AA amyloidosis in PWID. Studies were not restricted to location, study type, year or language of publication. Study heterogeneity precluded meta-analysis; we present a narrative review of the literature. Thirty-seven papers from eight countries met inclusion criteria. A total of 781 PWID are reported on, of whom 177 had AA amyloidosis. Where disease causality is established, it is attributed to chronic inflammation caused by injecting-related SSTIs. Most (88.7%) PWID with AA amyloidosis had SSTIs. The proportion of PWID with AA amyloidosis at post-mortem ranged from 1.6% (Germany) to 22.5% (Serbia). Biopsy studies reported from 5.26% (Portugal) to 50% (Germany) of AA amyloidosis in PWID with suspected or known kidney disease. Following diagnosis, the typical trajectory for PWID with AA amyloidosis was rapid deterioration of renal function requiring haemodialysis. Treatment difficulties, end-stage renal failure and premature death from sepsis were observed. Good outcomes, including reversibility of AA amyloidosis, are attributed to rapid treatment of the underlining inflammation and injecting cessation. Notably, given the population in question, no studies were published in addiction or harm reduction journals; most (92%) appeared in specialist nephrology and medical journals. There is strong evidence of an association between skin

  9. 'Care and Prevent': rationale for investigating skin and soft tissue infections and AA amyloidosis among people who inject drugs in London.

    Science.gov (United States)

    Harris, M; Brathwaite, R; McGowan, Catherine R; Ciccarone, D; Gilchrist, G; McCusker, M; O'Brien, K; Dunn, J; Scott, J; Hope, V

    2018-05-08

    Skin and soft tissue infections (SSTIs) are a leading cause of morbidity and mortality among people who inject drugs (PWID). International data indicate up to one third of PWID have experienced an SSTI within the past month. Complications include sepsis, endocarditis and amyloid A (AA) amyloidosis. AA amyloidosis is a serious sequela of chronic SSTI among PWID. Though there is a paucity of literature reporting on AA amyloidosis among PWID, what has been published suggests there is likely a causal relationship between AA amyloidosis and injecting-related SSTI. If left untreated, AA amyloidosis can lead to renal failure; premature mortality among diagnosed PWID is high. Early intervention may reverse disease. Despite the high societal and individual burden of SSTI among PWID, empirical evidence on the barriers and facilitators to injecting-related SSTI prevention and care or the feasibility and acceptability of AA amyloidosis screening and treatment referral are limited. This study aims to fill these gaps and assess the prevalence of AA amyloidosis among PWID. Care and Prevent is a UK National Institute for Health Research-funded mixed-methods study. In five phases (P1-P5), we aim to assess the evidence for AA amyloidosis among PWID (P1); assess the feasibility of AA amyloidosis screening, diagnostic and treatment referral among PWID in London (P2); investigate the barriers and facilitators to AA amyloidosis care (P3); explore SSTI protection and risk (P4); and co-create harm reduction resources with the affected community (P5). This paper describes the conceptual framework, methodological design and proposed analysis for the mixed-methods multi-phase study. We are implementing the Care and Prevent protocol in London. The systematic review component of the study has been completed and published. Care and Prevent will generate an estimate of AA amyloidosis prevalence among community recruited PWID in London, with implications for the development of screening

  10. [A review for recent advances in AA amyloid research and therapeutic approach to AA amyloidosis complicating rheumatoid arthritis].

    Science.gov (United States)

    Tamura, Hiroaki; Hasegawa, Kiminori

    2009-02-01

    AA amyloidosis is a life threatening clinical complication of chronic inflammatory diseases such as rheumatoid arthritis. It has been demonstrated biochemically that amyloidosis resulted from abnormal folding of proteins, which are deposited as insoluble fibrils in extracellular tissue, leading to the disruption of their normal function. In this regard, amyloidosis has been recognized as a conformation disorder. Interestingly, genetic polymorphisms of amyloid precursor protein (SAA) have been reported to associate with increased risk for AA amyloidosis. Also recent biochemical research revealed that SAA is synthesized under the influence of the proinflammatory cytokines, such as IL-6, TNF-alpha, IL-1. Additionally, it was suggested that amyloid deposits in extracellular tissue could reflect to the serum level of SAA in the reversible fashion, leading to the hypothesis that the control of the SAA synthesis could be beneficial to the treatment of amyloidosis. In this context, anti-cytokine therapies may be most effective. Especially the inhibition of IL-6 is critical to suppression of SAA production, so treatment with a humanized monoclonal antibody against human IL-6 receptor may not only ameliorate RA disease activity but also pave the way for the treatment of AA amyloidosis.

  11. Silicone stent placement for primary tracheal amyloidosis accompanied by cartilage destruction.

    Science.gov (United States)

    Ryu, Duck Hyun; Eom, Jung Seop; Jeong, Ho Jung; Kim, Jung Hoon; Lee, Ji Eun; Jun, Ji Eun; Song, Dae Hyun; Han, Joungho; Kim, Hojoong

    2014-06-01

    Primary tracheal amyloidosis (PTA) can lead to airway obstructions, and patients with severe PTA should undergo bronchoscopic interventions in order to maintain airway patency. Focal airway involvements with amyloidosis can only be treated with mechanical dilatation. However, the PTA with diffused airway involvements and concomitant cartilage destructions requires stent placement. Limited information regarding the usefulness of silicone stents in patients with PTA has been released. Therefore, we report a case of diffused PTA with tracheomalacia causing severe cartilage destruction, which is being successfully managed with bronchoscopic interventions and silicone stent placements.

  12. Bilateral optic neuropathy and intraretinal deposits after pars plana vitrectomy in amyloidosis

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    Rossetti Alberto

    2015-01-01

    Full Text Available Pathological examination of material from a nonextensive pars plana vitrectomy (PPV in the right eye provided a diagnosis of nonfamilial amyloidosis in a 68-year-old woman, who presented with bilateral glass wool-like vitreous opacities. Genetic testing revealed a Tyr114Cys mutation in the transthyretin gene. Six months after PPV, perimetry showed intense constriction with a temporal island and central scotoma in the right eye. An extensive PPV was performed in the left eye. Spectral domain optical coherence tomography evidenced bilateral epimacular amyloid deposits and unreported reflective spots within the inner retina. One year later, visual acuity had decreased to 20/400 in the left eye, with mild vitreous opacity, pale cupped optic disc and inferior altitudinal field defect. Bilateral diurnal intraocular pressure, transiently increased after PPV, never exceeded 16 mmHg with medication. Our patient presented optic nerve blood supply impairment, due to amyloidosis, which caused optic atrophy. Epiretinal and intraretinal deposit detection could aid in diagnosing patients with suspected amyloidosis.

  13. Surgical management of macroglossia secondary to amyloidosis.

    Science.gov (United States)

    Gadiwalla, Yusuf; Burnham, Richard; Warfield, Adrian; Praveen, Prav

    2016-04-11

    The authors report a case of amyloidosis-induced macroglossia treated with surgical reduction of the tongue using a keyhole to inverted T method with particular emphasis on the postoperative sequelae. Significant tongue swelling persisted for longer than anticipated requiring tracheostomy to remain in situ for 14 days. 2016 BMJ Publishing Group Ltd.

  14. Focal Amyloidosis of the Orbit Presenting as a Mass: MRI and CT Features

    International Nuclear Information System (INIS)

    Yerli, Hasan; Aydin, Erdinc; Avci, Suat; Haberal, Nihan; Oto, Sibel

    2011-01-01

    Focal orbital amyloidosis is a rare entity and little is known about its magnetic resonance imaging (MRI) features. In this case report, imaging features of a case of focal orbital amyloidosis presenting as a mass have been documented together with its histopathological findings. On MRI, a well-defined mass was seen as isointense with rectus muscle on T1-weighted images and heterogeneously hypointense on T2-weighted images. Punctuate calcifications were observed on the computerized tomography (CT) examination

  15. Cardioverter-defibrillator implantation in myeloma-associated cardiac amyloidosis.

    Science.gov (United States)

    Campanile, Alfonso; Sozzi, Fabiola B; Canetta, Ciro; Danzi, Gian Battista

    2013-01-01

    A 62-year-old woman with multiple myeloma and light-chain amyloidosis with significant heart involvement developed an in-hospital cardiac arrest. After cardiopulmonary resuscitation, a stable sinus rhythm without any cerebral damage was restored, and the patient was admitted to the coronary care unit. A cardioverter-defibrillator was implanted, and it successfully intervened in two sustained ventricular tachycardia episodes and one ventricular fibrillation episode, which were recorded during hospitalization. After achieving discrete cardiac compensation, the patient was transferred to the emergency medicine department where she underwent chemotherapy for multiple myeloma. The patient died 40 days after admission from refractory heart failure. In the literature, there are studies that describe the use of cardioverter-defibrillator implantation in cardiac amyloidosis; however, at present, there is no evidence of a beneficial effect on survival with the use of this intervention. A high index of suspicion for amyloid heart disease and early diagnosis are critical to improving outcomes.

  16. Novel recurrent chromosomal aberrations detected in clonal plasma cells of light chain amyloidosis patients show potential adverse prognostic effect: first results from a genome-wide copy number array analysis.

    Science.gov (United States)

    Granzow, Martin; Hegenbart, Ute; Hinderhofer, Katrin; Hose, Dirk; Seckinger, Anja; Bochtler, Tilmann; Hemminki, Kari; Goldschmidt, Hartmut; Schönland, Stefan O; Jauch, Anna

    2017-07-01

    Immunoglobulin light chain (AL) amyloidosis is a rare plasma cell dyscrasia characterized by the deposition of abnormal amyloid fibrils in multiple organs, thus impairing their function. In the largest cohort studied up to now of 118 CD138-purified plasma cell samples from previously untreated immunoglobulin light chain amyloidosis patients, we assessed in parallel copy number alterations using high-density copy number arrays and interphase fluorescence in situ hybridization (iFISH). We used fluorescence in situ hybridization probes for the IgH translocations t(11;14), t(4;14), and t(14;16) or any other IgH rearrangement as well as numerical aberrations of the chromosome loci 1q21, 8p21, 5p15/5q35, 11q22.3 or 11q23, 13q14, 15q22, 17p13, and 19q13. Recurrent gains included chromosomes 1q (36%), 9 (24%), 11q (24%), as well as 19 (15%). Recurrent losses affected chromosome 13 (29% monosomy) and partial losses of 14q (19%), 16q (14%) and 13q (12%), respectively. In 88% of patients with translocation t(11;14), the hallmark chromosomal aberration in AL amyloidosis, a concomitant gain of 11q22.3/11q23 detected by iFISH was part of the unbalanced translocation der(14)t(11;14)(q13;q32) with the breakpoint in the CCND1/MYEOV gene region. Partial loss of chromosome regions 14q and 16q were significantly associated to gain 1q. Gain 1q21 detected by iFISH almost always resulted from a gain of the long arm of chromosome 1 and not from trisomy 1, whereas deletions on chromosome 1p were rarely found. Overall and event-free survival analysis found a potential adverse prognostic effect of concomitant gain 1q and deletion 14q as well as of deletion 1p. In conclusion, in the first whole genome report of clonal plasma cells in AL amyloidosis, novel aberrations and hitherto unknown potential adverse prognostic effects were uncovered. Copyright© 2017 Ferrata Storti Foundation.

  17. Unsuitable value of abdominal fat tissue aspirate examination for the diagnosis of amyloidosis in long-term hemodialysis patients.

    Science.gov (United States)

    Orfila, C; Goffinet, F; Goudable, C; Eche, J P; Ton That, H; Manuel, Y; Suc, J M

    1988-01-01

    Abdominal fat tissue aspiration was used in 22 long-term hemodialysis patients (5-17 years). Fourteen of these patients had carpal tunnel syndrome and amyloid deposits of beta 2-microglobulin in the synovium. One patient had a spontaneous rupture of the spleen with amyloid deposits in spleen vessels. Seven other patients presented carpal tunnel syndrome and/or articular pains, and radiological lytic lesions in bone, strongly suggesting an amyloid origin. As a control group, in 22 patients with biopsy-proven amyloidosis, abdominal fat tissue aspirates were performed and were studied under the same conditions: by light microscopy these tissues were stained with Congo red and examined with a polarizing microscope; these specimens were also studied by electron microscopy. In all hemodialyzed patients, no amyloid deposit was present in fat tissue with Congo red staining and by electron microscopy. On the contrary, amyloid was observed in 17 of 22 cases in other types of amyloidosis. It seems that this method which has been proved to be simple and sensitive for the diagnosis of systemic amyloidosis is not a good marker for the presence of amyloid in long-term hemodialysis patients.

  18. Laser microdissection and mass spectrometry-based proteomics aids the diagnosis and typing of renal amyloidosis.

    Science.gov (United States)

    Sethi, Sanjeev; Vrana, Julie A; Theis, Jason D; Leung, Nelson; Sethi, Anjali; Nasr, Samih H; Fervenza, Fernando C; Cornell, Lynn D; Fidler, Mary E; Dogan, Ahmet

    2012-07-01

    Accurate diagnosis and typing of renal amyloidosis is critical for prognosis, genetic counseling, and treatment. Laser microdissection and mass spectrometry are emerging techniques for the analysis and diagnosis of many renal diseases. Here we present the results of laser microdissection and mass spectrometry performed on 127 cases of renal amyloidosis during 2008-2010. We found the following proteins in the amyloid deposits: immunoglobulin light and heavy chains, secondary reactive serum amyloid A protein, leukocyte cell-derived chemotaxin-2, fibrinogen-α chain, transthyretin, apolipoprotein A-I and A-IV, gelsolin, and β-2 microglobulin. Thus, laser microdissection of affected areas within the kidney followed by mass spectrometry provides a direct test of the composition of the deposit and forms a useful ancillary technique for the accurate diagnosis and typing of renal amyloidosis in a single procedure.

  19. Dialysis-related amyloidosis: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Scarpioni R

    2016-12-01

    Full Text Available R Scarpioni, M Ricardi, V Albertazzi, S De Amicis, F Rastelli, L Zerbini Department of Nephrology and Dialysis, Azienda Unità Sanitaria Local (AUSL Hospital “Guglielmo da Saliceto”, Piacenza, Italy Abstract: Amyloidosis refers to the extracellular tissue deposition of fibrils composed of low-molecular-weight subunits of a variety of proteins. These deposits may result in a wide range of clinical manifestations depending upon their type, location, and the amount of deposition. Dialysis-related amyloidosis is a serious complication of long-term dialysis therapy and is characterized by the deposition of amyloid fibrils, principally composed of β2 microglobulins (β2M, in the osteoarticular structures and viscera. Most of the β2M is eliminated through glomerular filtration and subsequent reabsorption and catabolism by the proximal tubules. As a consequence, the serum levels of β2M are inversely related to the glomerular filtration rate; therefore, in end-stage renal disease patients, β2M levels increase up to 60-fold. Serum levels of β2M are also elevated in several pathological conditions such as chronic inflammation, liver disease, and above all, in renal dysfunction. Retention of amyloidogenic protein has been attributed to several factors including type of dialysis membrane, prolonged uremic state and/or decreased diuresis, advanced glycation end products, elevated levels of cytokines and dialysate. Dialysis treatment per se has been considered to be an inflammatory stimulus, inducing cytokine production (such as interleukin-1, tumor necrosis factor-α, interleukin-6 and complement activation. The released cytokines are thought to stimulate the synthesis and release of β2M by the macrophages and/or augment the expression of human leukocyte antigens (class I, increasing β2M expression. Residual renal function is probably the best determinant of β2M levels. Therefore, it has to be maintained as long as possible. In this article

  20. Solitary plasmacytoma of spine with amyloidosis

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    Cui-yun SUN

    2017-01-01

    Full Text Available Objective To report the diagnosis and treatment of one case of solitary plasmacytoma of spine with amyloidosis and investigate the clinicopathological features combined with literatures. Methods and Results The patient was a 46-year-old woman. She suffered from weakness of both lower limbs, unsteady gait and numbness of toes for 20 d. MRI examination revealed an irregular mass behind the spinal cord at T5-7 level and T6-7 vertebral body accessory. The enhanced MRI showed obvious heterogeneous enhancement. The border was clear and spinal dura mater was compressed to shift forward. During operation, T5-7 processus spinosus and vertebral laminae were eroded, and the cortex of bone showed "moth-eaten" erosion. The intraspinal and extradural lesion had rich blood supply, loose bone structure and intact spinal dura mater. Histologically, tumor cells were composed of intensive small cells, and focal plasmacytoid cells were seen. Flake pink staining substance was among them. Artificial cracks were common and multinuclear giant tumor cells were scatteredly distributed. Immunohistochemical analysis showed the cytoplasm of tumor cells were diffusely positive for CD138, CD38 and vimentin (Vim,scatteredly positive for leukocyte common antigen (LCA, and negative for immune globulin κ light chain(IgGκ and λ light chain (IgGλ, CD99, S-100 protein (S-100, pan cytokeratin (PCK, epithelial membrane antigen (EMA, HMB45 and CD34. The Ki-67 labeling index was 1.25%. Congo red staining showed the pink staining substance was brownish red. Hybridization in situ examination showed the DNA content of IgGκ was more than that of IgGλ. The final pathological diagnosis was solitary plasmacytoma of spine with amyloidosis. The patient was treated with postoperative chemotherapy, and there was no recurrence or metastasis during 18-month follow-up period. Conclusions Solitary plasmacytoma of spine with amyloidosis is a rare tumor. The imaging features can offer a few

  1. MRI and echocardiography in the diagnosis of cardiac amyloidosis

    International Nuclear Information System (INIS)

    Wang Jing; Kong Xiangquan; Zhou Guofeng; Xu Haibo; Chang Dandan; Feng Yiming; Liu Dingxi; Zhang Li; Xie Mingxing

    2010-01-01

    Objective: To assess the values of MRI and echocardiography for the diagnosis of cardiac amyloidosis (CA). Methods: Eleven cases with CA proved pathologically performed MRI and echocardiography, the findings were analyzed retrospectively. Results: The characteristic features of cardiac amyloidosis on MRI and echocardiography were: diffuse slight myocardial thickening of the left ventricular wall and interventricular septum (11 cases), slight myocardial thickening of the interatrial septum (5 cases), increased left ventricular mass (7 cases), enlarged left atrium (7 cases), impaired ventricular systolic and diastolic function (10 cases), pleural and pericardial effusions (11 and 9 cases). Echocardiography showed that myocardium was hyperechoic and presented as ground glass with some spotty hyperechoes in 6 cases. MRI revealed a distinct diffuse delayed enhancement of subendocardial and entire myocardium in 8 cases. Conclusion: Doppler echocardiography is the first-choice imaging technique and cardiac magnetic resonance imaging can provide more information for the diagnosis of CA. (authors)

  2. Amiloidosis secundaria en la enfermedad inflamatoria intestinal Secondary amyloidosis in Chrohn's disease

    Directory of Open Access Journals (Sweden)

    S. Seijo Ríos

    2008-12-01

    Full Text Available La amiloidosis es una entidad clínica que se produce a consecuencia del depósito a nivel extracelular de un material proteico amorfo, causando una desorganización de la arquitectura normal de múltiples órganos y tejidos y, por tanto, una alteración funcional de los mismos. La amiloidosis secundaria es una complicación infrecuente pero muy grave que aparece en el contexto de neoplasias, enfermedades infecciosas e inflamatorias de curso crónico, como es el caso de la enfermedad inflamatoria intestinal, principalmente enfermedad de Crohn, ensombreciendo el pronóstico de estos pacientes. A continuación presentamos dos casos clínicos correspondientes a dos pacientes con enfermedad de Crohn que desarrollaron amiloidosis secundaria.Amyloidosis is a clinical entity that results from the deposition of an extracellular protein material that causes disruption in the normal architecture of multiple organs and tissues, and impairs their function. Secondary amyloidosis is a rare but serious complication that may worsen the prognosis of patients with cancer, infection or chronic inflammatory disease, including inflammatory bowel disease, particularly Crohn's disease. We report two cases of Crohn's disease associated with secondary amyloidosis.

  3. Estimation of Daily Proteinuria in Patients with Amyloidosis by Using the Protein-To-Creatinine ratio in Random Urine Samples.

    Science.gov (United States)

    Talamo, Giampaolo; Mir Muhammad, A; Pandey, Manoj K; Zhu, Junjia; Creer, Michael H; Malysz, Jozef

    2015-02-11

    Measurement of daily proteinuria in patients with amyloidosis is recommended at the time of diagnosis for assessing renal involvement, and for monitoring disease activity. Renal involvement is usually defined by proteinuria >500 mg/day. We evaluated the accuracy of the random urine protein-to-creatinine ratio (Pr/Cr) in predicting 24 hour proteinuria in patient with amyloidosis. We compared results of random urine Pr/Cr ratio and concomitant 24-hour urine collections in 44 patients with amyloidosis. We found a strong correlation (Spearman's ρ=0.874) between the Pr/Cr ratio and the 24 hour urine protein excretion. For predicting renal involvement, the optimal cut-off point of the Pr/Cr ratio was 715 mg/g. The sensitivity and specificity for this point were 91.8% and 95.5%, respectively, and the area under the curve value was 97.4%. We conclude that the random urine Pr/Cr ratio could be useful in the screening of renal involvement in patients with amyloidosis. If validated in a prospective study, the random urine Pr/Cr ratio could replace the 24 hour urine collection for the assessment of daily proteinuria and presence of nephrotic syndrome in patients with amyloidosis.

  4. Effects of Biologic Agents in Patients with Rheumatoid Arthritis and Amyloidosis Treated with Hemodialysis

    OpenAIRE

    Kuroda, Takeshi; Tanabe, Naohito; Nozawa, Yukiko; Sato, Hiroe; Nakatsue, Takeshi; Kobayashi, Daisuke; Wada, Yoko; Saeki, Takako; Nakano, Masaaki; Narita, Ichiei

    2016-01-01

    Objective Our objective was to examine the safety and effects of therapy with biologics on the prognosis of rheumatoid arthritis (RA) patients with reactive amyloid A (AA) amyloidosis on hemodialysis (HD). Methods Twenty-eight patients with an established diagnosis of reactive AA amyloidosis participated in the study. The survival was calculated from the date of HD initiation until the time of death, or up to end of June 2015 for the patients who were still alive. HD initiation was according ...

  5. Breast cancer and amyloid bodies: is there a role for amyloidosis in cancer-cell dormancy?

    Directory of Open Access Journals (Sweden)

    Mizejewski GJ

    2017-04-01

    Full Text Available Gerald J Mizejewski Wadsworth Center, New York State Department of Health, Albany, NY, USA Abstract: Breast cancer and Alzheimer’s disease (AD are major causes of death in older women. Interestingly, breast cancer occurs less frequently in AD patients than in the general population. Amyloidosis, the aggregation of amyloid proteins to form amyloid bodies, plays a central role in the pathogenesis of AD and other human neuropathies by forming intracellular fibrillary proteins. Contrary to popular belief, amyloidosis is a common occurrence in mammalian cells, and has recently been reported to be a natural physiological process in response to environmental stress stimulations (such as pH and temperature extremes, hypoxia, and oxidative stress. Many proteins contain an intrinsic “amyloid-converting motif”, which acts in conjunction with a specific noncoding RNA to induce formation of proteinaceous amyloid bodies that are stored in intracellular bundles. In cancer cells such as breast and prostate, the process of amyloidosis induces cells to enter a dormant or resting stage devoid of cell division and proliferation. Therefore, cancer cells undergo growth cessation and enter a dormant stage following amyloidosis in the cell; this is akin to giving the cell AD to cease growth. Keywords: α-fetoprotein, noncoding RNA, amyloid bodies, dormancy, breast cancer, Alzheimer’s disease

  6. The specific case: cardiac amyloidosis as differential diagnosis in case of restricted cardiac pump function; Der besondere Fall. Amyloidose des Herzens als Differenzialdiagnose bei eingeschraenkter kardialer Pumpfunktion

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    D' Errico, L. [Universitaetsspital Basel (Switzerland). Klinik fuer Radiologie und Nuklearmedizin; Zellweger, M.; Niemann, T.

    2014-03-15

    The NMR imaging data in combination with clinical characterization and echocardiography are consistent with the diagnosis of a cardiac amyloidosis. The article describes disease pattern and diagnosis based on contrast agent accumulation and diastolic functional disturbances. CT was performed to exclude pulmonary embolism.

  7. Atypical Presentation of Gelsolin Amyloidosis in a Man of African Descent with a Novel Mutation in the Gelsolin Gene.

    Science.gov (United States)

    Oregel, Karlos Z; Shouse, Geoffrey P; Oster, Cyrus; Martinez, Freddy; Wang, Jun; Rosenzweig, Michael; Deisch, Jeremy K; Chen, Chien-Shing; Nagaraj, Gayathri

    2018-03-30

    BACKGROUND Gelsolin amyloidosis is a very rare systemic disease presenting with a pathognomonic triad of corneal lattice dystrophy, cutis laxa, and polyneuropathy. The disease is mostly restricted to a Finnish population with known mutations (G654A, G654T) in exon 4 of the gelsolin gene. The mutations lead to errors in protein processing and folding, and ultimately leads to deposition of an amyloidogenic fragment in the extracellular space, causing the symptoms of disease. CASE REPORT We present a case of gelsolin amyloidosis in a male of African descent with an atypical clinical presentation including fevers, skin rash, polyneuropathy, and anemia. Gelsolin amyloidosis was diagnosed based on mass spectrometry of tissue samples. Importantly, a novel mutation in the gelsolin gene (C1375G) in exon 10 was found in this patient. His atypical presentation can possibly be attributed to the presence of a novel mutation in the gelsolin gene as the likely underlying cause of the syndrome. PCR primers were used to amplify the gelsolin gene from genomic DNA. Purified PCR products were then shipped to Eton Biosciences (San Diego, CA) for sequencing. CONCLUSIONS This study carries several important lessons relevant to the practice of medicine. First, the differential diagnosis for multisystem disease presentations should always include amyloidosis. Second, despite what has been uncovered about the molecular biology of disease, there is always more that can be discovered. Finally, further work to verify the link between this mutation and the clinical syndrome is still needed, as are effective treatments for this disease.

  8. Fragments of the constant region of immunoglobulin light chains are constituents of AL-amyloid proteins

    DEFF Research Database (Denmark)

    Olsen, K E; Sletten, K; Westermark, Per

    1998-01-01

    Immunoglobulin light chains are the precursor proteins of AL-amyloidosis. In the fibril formation process properties of the variable part of the immunoglobulin light chains are believed to be of major importance. In this work it is shown that fragments of the constant part of the immunoglobulin l...... light chain are a constituent of the AL-amyloid proteins of kappa type. A specific antiserum has identified these fragments in gel filtration fractions where the absorbance approached the base line after the main retarded peak. The fragments are small and have been overlooked previously......Immunoglobulin light chains are the precursor proteins of AL-amyloidosis. In the fibril formation process properties of the variable part of the immunoglobulin light chains are believed to be of major importance. In this work it is shown that fragments of the constant part of the immunoglobulin...... in the purification process. The significance of the constant part in AL-proteins is unclear, but adds new aspects to the discussion of pre- or post-fibrillogenic cleavage of the immunoglobulin light chains....

  9. Estimation of daily proteinuria in patients with amyloidosis by using the protein-to-creatinine ratio in random urine sample

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    Giampaolo Talamo

    2015-02-01

    Full Text Available Measurement of daily proteinuria in patients with amyloidosis is recommended at the time of diagnosis for assessing renal involvement, and for monitoring disease activity. Renal involvement is usually defined by proteinuria >500 mg/day. We evaluated the accuracy of the random urine protein-to-creatinine ratio (Pr/Cr in predicting 24 hour proteinuria in patient with amyloidosis. We com- pared results of random urine Pr/Cr ratio and concomitant 24-hour urine collections in 44 patients with amyloidosis. We found a strong correlation (Spearman’s ρ=0.874 between the Pr/Cr ratio and the 24 hour urine protein excretion. For predicting renal involvement, the optimal cut-off point of the Pr/Cr ratio was 715 mg/g. The sensitivity and specificity for this point were 91.8% and 95.5%, respectively, and the area under the curve value was 97.4%. We conclude that the random urine Pr/Cr ratio could be useful in the screening of renal involvement in patients with amyloidosis. If validated in a prospective study, the random urine Pr/Cr ratio could replace the 24 hour urine collection for the assessment of daily proteinuria and presence of nephrotic syndrome in patients with amyloidosis.

  10. Pathology of AA amyloidosis in domestic sheep and goats.

    Science.gov (United States)

    Ménsua, C; Carrasco, L; Bautista, M J; Biescas, E; Fernández, A; Murphy, C L; Weiss, D T; Solomon, A; Luján, L

    2003-01-01

    We describe the main pathologic changes in small ruminants affected by AA amyloidosis, together with the partial sequence of the protein involved. Twenty-one sheep and one goat were selected for presenting macroscopic kidney lesions compatible with systemic amyloidosis. Available tissue samples were studied by histologic, immunopathologic, and ultrastructural means. Renal lesions were characterized grossly by pale cortical surfaces with scattered, miliary, whitish-yellow foci and on cut cortical surfaces by straight, whitish-yellow striations. Gangrenous pneumonia was observed in 16 out of 21 affected sheep (76.2%), although other chronic inflammations were also observed. Amyloid was detected in all grossly affected kidneys using Congo red staining, lesions being most remarkable in glomeruli, affecting 95.5% of animals studied. Congophilic deposits were also observed in intertubular interstitium (68.2%) and medulla (57.1%). All amyloid-affected animals presented proximal convoluted tubule lesions, mostly characterized by an increase in diameter and by hyaline granular degeneration that were responsible for the macroscopic appearance of the kidney. Histologically, amyloid was also seen in blood vessels, spleen, liver, lymph nodes, gastrointestinal tract, and adrenal glands. All amyloid deposits demonstrated greenish-yellow birefringence with polarized light, and the antisera prepared against goat amyloid extracts specifically reacted with birefringent congophilic deposits of both sheep and goats. Ultrastructurally, these deposits were formed by masses of straight, nonbranching fibrils located predominantly in the basement membranes of glomerular capillaries and in the mesangium. Partial sequence of the protein in sheep and goats indicated a high degree of homology with the previously reported sequence of sheep Serum Amyloid A.

  11. Renal amyloid A amyloidosis as a complication of hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Schandorff, Kristine D; Miller, Iben M; Krustrup, Dorrit

    2016-01-01

    Rheumatic disease is the dominant cause of amyloid A (AA) amyloidosis, but other chronic inflammatory diseases may have similar consequences. Hidradenitis suppurativa (HS) is a relatively common, but little known skin disease characterized by chronic inflammation. Here we present a case of chronic...

  12. Primary conjunctival amyloidosis

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    Chandana Chakraborti

    2014-01-01

    Full Text Available A 19-year-old previously healthy male presented with a 4 year history of painless drooping of right upper eyelid.On eversion of the right upper eyelid, a yellowish pink mass was seen in the tarsal region. Rest of the ocular examination was normal in both the eyes. Initial biopsy showed chronic inflammation. Subsequently, the entire mass was excised and histopathological examination showed the presence of amyloid in the subconjunctival stroma. At 3 months follow-up, similar lesion was detected in the right lower, left upper, and lower lid, which were treated with cryotherapy, with partial resolution. Patient has been followed up for more than 2 years without any complaints. To our knowledge, this is the first case report of an isolated primary conjunctival amyloidosis with involvement of both the upper and lower palpebral conjunctiva of either eye. It was treated successfully by excision and cryotherapy.

  13. Secondary renal amyloidosis in a patient of pulmonary tuberculosis and common variable immunodeficiency

    Directory of Open Access Journals (Sweden)

    Balwani Manish R

    2015-04-01

    Full Text Available Common variable immunodeficiency (CVID usually manifests in the second or third decade of life with recurrent bacterial infections and hypoglobulinemia. Secondary renal amyloidosis with history of pulmonary tuberculosis is rare in CVID, although T cell dysfunction has been reported in few CVID patients. A 40-year-old male was admitted to our hospital with a 3-month history of recurrent respiratory infections and persistent pitting pedal edema. His past history revealed 3 to 5 episodes of recurrent respiratory tract infections and diarrhoea each year since last 20 years. He had been successfully treated for sputum positive pulmonary tuberculosis 8 years back. Laboratory studies disclosed high erythrocyte sedimentation rate (ESR, hypoalbuminemia and nephrotic range proteinuria. Serum immunoglobulin levels were low. CD4/CD8 ratio and CD3 level was normal. C3 and C4 complement levels were normal. Biopsy revealed amyloid A (AA positive secondary renal amyloidosis. Glomeruli showed variable widening of mesangial regions with deposition of periodic schiff stain (PAS pale positive of pink matrix showing apple green birefringence on Congo-red staining. Immunohistochemistry was AA stain positive. Immunofluorescence microscopy revealed no staining with anti-human IgG, IgM, IgA, C3, C1q, kappa and lambda light chains antisera. Patient was treated symptomatically for respiratory tract infection and was discharged with low dose angiotensin receptor blocker. An old treated tuberculosis and chronic inflammation due to recurrent respiratory tract infections were thought to be responsible for AA amyloidosis. Thus pulmonary tuberculosis should be considered in differential diagnosis of secondary causes of AA renal amyloidosis in patients of CVID especially in endemic settings.

  14. Fatal acute pancreatitis associated with reactive AA amyloidosis in rheumatoid arthritis with end-stage renal disease: a report of three cases.

    Science.gov (United States)

    Kuroda, Takeshi; Sato, Hiroe; Hasegawa, Hisashi; Wada, Yoko; Murakami, Shuichi; Saeki, Takako; Nakano, Masaaki; Narita, Ichiei

    2011-01-01

    We report three cases of fatal pancreatitis associated with systemic AA amyloidosis in rheumatoid arthritis (RA). All of the patients showed end-stage renal failure, and hemodialysis was introduced during the course of treatment. Autopsy was performed on two of the three patients, and this revealed amyloid deposition on the vascular walls in the pancreas. It was strongly suggested that the acute pancreatitis in all three patients was attributable to deposition of amyloid in vascular and pancreatic tissues. Acute pancreatitis is considered to be a rare complication of end-stage amyloidosis associated with RA, and is frequently fatal. It is important to treat RA patients intensively to avoid such deposition of amyloid.

  15. Leukocyte derived chemotaxin 2 (ALECT2 amyloidosis

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    Uday P Kulkarni

    2015-07-01

    Full Text Available We describe the first case from India of ALECT2 amyloidosis. An adult Punjabi male presented with progressive renal dysfunction and non-nephrotic range proteinuria. Serum protein electrophoresis and Immunofixation were normal, with mildly elevated serum free light chain ratio. Renal biopsy confirmed the presence of amyloid. Immunohistochemistry was negative for monoclonal light chains. Proteomic analysis confirmed the presence of ALECT2 amyloid. The present case highlights the need for confirmatory testing for typing of amyloid.

  16. A new amyloidosis caused by fibrillar aggregates of mutated corneodesmosin

    DEFF Research Database (Denmark)

    Caubet, Cécile; Bousset, Luc; Clemmensen, Ole

    2010-01-01

    with circular dichroism measurements. This identifies HSS as a human amyloidosis related to the aggregation of natively unfolded (mut)CDSN polypeptides into amyloid fibrils.-Caubet, C., Bousset, L., Clemmensen, O., Sourigues, Y., Bygum, A., Chavanas, S., Coudane, F., Hsu, C.-Y., Betz, R. C., Melki, R., Simon, M...

  17. Accumulation of Tc99m-DMSA-3 in the spleen in a case of multiple myeloma with associated amyloidosis

    Directory of Open Access Journals (Sweden)

    Barai Sukanta

    2005-01-01

    Full Text Available We describe a case of a 58-year-old male with longstanding hypertension and Type 2 diabetes mellitus who developed sudden onset renal impairment. The first clue to the possible presence of amyloidosis in this case was provided by the radionuclide renal cortical scan performed with trivalent dimercapto succinic acid (Tc99m-DMSA-3, which revealed intense tracer uptake in the spleen suggesting amyloid deposit. Further workup to ascertain the cause of amyloidosis led to the diagnosis of multiple myeloma. We conclude that in cases of extra-renal or splenic accumulation of Tc99m-DMSA-3, a diagnosis of amyloidosis should be considered, in an appropriate clinical setting.

  18. Rapid intestinal transit as a primary cause of severe chronic diarrhea in patients with amyloidosis.

    Science.gov (United States)

    Guirl, Michael J; Högenauer, Christoph; Santa Ana, Carol A; Porter, Jack L; Little, Katherine H; Stone, Marvin J; Fordtran, John S

    2003-10-01

    The cause of severe diarrhea in patients with systemic amyloidosis is obscure. We therefore performed pathophysiological studies in three such patients in an effort to determine the mechanism of amyloid diarrhea. Epithelial cell absorption rate of electrolytes was measured during steady state GI perfusion of a saline-mannitol solution. GI transit time of PEG and absorption of radiolabeled bile acid were measured simultaneously while subjects ingested three meals per day. To obtain a diarrhea control group for transit time and bile acid absorption, normal subjects were studied when they had diarrhea caused by ingestion of Milk of Magnesia (MOM). Diarrhea could not be explained by malabsorption of ingested nutrients, bacterial overgrowth, bile acid malabsorption, or epithelial cell malabsorption of electrolytes. However, 25% of polyethylene glycol (PEG) ingested with a standard meal was recovered in stool in 45 min, which is 10 times faster than in normal subjects with equally severe diarrhea caused by ingestion of MOM. All of the patients had autonomic neuropathy that remained unrecognized for 15-36 months after onset of chronic diarrhea; it seems likely that this was the cause of rapid transit. Severe chronic diarrhea in three patients with systemic amyloidosis was mediated by extremely rapid transit of chyme and digestive secretions through the intestine.

  19. Constant region of a kappa III immunoglobulin light chain as a major AL-amyloid protein

    DEFF Research Database (Denmark)

    Engvig, J P; Olsen, K E; Gislefoss, R E

    1998-01-01

    AL-amyloidoses are generally described as a group of disorders in which N-terminal fragments of monoclonal immunoglobulin light chains are transferred into amyloid fibrils. We have, by amino acid sequence analyses and immunological methods, characterized the Bence-Jones protein and the correspond......AL-amyloidoses are generally described as a group of disorders in which N-terminal fragments of monoclonal immunoglobulin light chains are transferred into amyloid fibrils. We have, by amino acid sequence analyses and immunological methods, characterized the Bence-Jones protein...... and the corresponding AL protein as a kappa III immunoglobulin light chain from material of a patient with systemic AL-amyloidosis presenting as a local inguinal tumour. The two proteins showed some unique features. The major part of the AL amyloid fibril protein consisted of C-terminal fragments of the Bence......-Jones protein. Furthermore, both the Bence-Jones protein and the AL protein were glycosylated, with possibly a glycosylation in the constant part of the light chain....

  20. Whole body amyloid deposition imaging by 123I-SAP scintigraphy

    NARCIS (Netherlands)

    van Rheenen, Ronald; Glaudemans, Andor; Hazenberg, Bouke

    2011-01-01

    Amyloidosis is the name of a group of diseases characterized by extracellular deposition of amyloid fibrils. Deposition of amyloid can be localized or systemic. The 123I-SAP-scan can be used to image extent and distribution of amyloid deposition in patients with systemic AA, AL and ATTR amyloidosis.

  1. RE: Imaging Features of Hepato-Splenic Amyloidosis at PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Mainenti, Pier Paolo [IBB CNR, Napoli (Italy)

    2012-06-15

    Regarding the imaging of hepatic amyloidosis with PET/CT, we read with interest the case report by Son and colleagues (1) in the Sep-Oct 2011 issue of the Korean Journal of Radiology. A few points need to be discussed with more detail. First, although the enhanced CT scan of the spleen exhibited a decreased and diffuse parenchymal attenuation, this observation was not sufficiently emphasized within the manuscript. The radiological finding of splenic hypoperfusion has been well described in the literature as a marker of systemic amyloidosis, which presents a useful clue when clinical findings fail to determine the proper diagnosis (2). In the case reported by Son and colleagues, if the radiologist had immediately suggested the correct diagnostic hypothesis, the patient would have been directed to have the the appropriate laboratory examinations conducted. Specifically, the abdominal fat aspirate test which would have eliminated the need for the PET/CT scan and a risky invasive procedure such as a liver biopsy. The PET/CT images revealed a marked and diffuse increase in the 18F-FDG uptake in the enlarged liver. How do the Authors explain this finding considering the diagnosis of the disease which is characterized by the deposition and storage of an amorphous substance including amyloid? Did the compressed hepatic cells and the encased bile duct epithelium increase the glucose metabolism or was the amyloid deposition accompanied by the inflammation due to the infiltration of cells? The authors might offer us an hypothesis about the physio-pathological mechanism causing the 18F-FDG uptake in the amyloidotic liver.

  2. Outcome of patients with reactive amyloidosis associated with rheumatoid arthritis in dialysis treatment.

    Science.gov (United States)

    Kuroda, Takeshi; Tanabe, Naohito; Sato, Hiroe; Ajiro, Jyunya; Wada, Yoko; Murakami, Syuichi; Hasegawa, Hisashi; Sakatsume, Minoru; Nakano, Masaaki; Gejyo, Fumitake

    2006-10-01

    The aim was to analyze the clinical outcome of a group of 51 patients diagnosed with systemic amyloidosis associated with rheumatoid arthritis who received hemodialysis (HD) as renal replacement therapy. We monitored the clinical course of the disease and factors that could influence survival. Determination of the onset of the underlying disorder was made retrospectively by reviewing the patient's chart when a diagnosis of amyloid was confirmed. During a 96.9 person-year follow-up, 42 patients died. Survival of these 51 patients from the initiation of HD at 251 days was 50%. Poor prognosis in amyloid patients was mainly due to a large number of sudden deaths immediately following HD therapy. Out of 51 patients 21 needed unplanned initiation of HD. The unplanned initiation was significantly associated with poor survival. Seventy-five percentile of creatinine clearance (Ccr) was 9.7 ml/min, and 75% of these patients who initiated HD had highly impaired renal functional states. These data indicated that amyloidotic patients with HD showed a high mortality rate; therefore, planned initiation of HD was highly recommended to improve the patient's survival. Particular attention was given to the Ccr levels, because the levels of serum creatinine may not be a useful marker for some patients with amyloidosis.

  3. Long-Term Results of Conformal Radiotherapy for Progressive Airway Amyloidosis

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    Truong, Minh Tam, E-mail: mitruong@bu.edu [Department of Radiation Oncology, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA (United States); Kachnic, Lisa A. [Department of Radiation Oncology, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA (United States); Grillone, Gregory A. [Department of Otolaryngology, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA (United States); Bohrs, Harry K.; Lee, Richard [Department of Radiation Oncology, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA (United States); Sakai, Osamu [Department of Radiology, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA (United States); Berk, John L. [Department of Medicine, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA (United States)

    2012-06-01

    Purpose: To evaluate the efficacy of conformal external beam radiotherapy (RT) for local control of progressive airway amyloidosis. Methods and Materials: We conducted a retrospective review of patients with biopsy-proven progressive airway amyloidosis treated with conformal RT between 2000 and 2006 at Boston Medical Center. The patients were evaluated for performance status and pulmonary function, with computed tomography and endoscopy after RT compared with the pretreatment studies. Local control was defined as the lack of progression of airway wall thickening on computed tomography imaging and stable endobronchial deposits by endoscopy. Results: A total of 10 symptomatic airway amyloidosis patients (3 laryngeal and 7 tracheobronchial) received RT to a median total dose of 20 Gy in 10 fractions within 2 weeks. At a median follow-up of 6.7 years (range, 1.5-10.3), 8 of the 10 patients had local control. The remaining 2 patients underwent repeat RT 6 and 8.4 months after initial RT, 1 for persistent bronchial obstruction and 1 for progression of subglottic amyloid disease with subsequent disease control. The Eastern Cooperative Oncology Group performance status improved at a median of 18 months after RT compared with the baseline values, from a median score of 2 to a median of 1 (p = .035). Airflow (forced expiratory volume in 1 second) measurements increased compared with the baseline values at each follow-up evaluation, reaching a 10.7% increase (p = .087) at the last testing (median duration, 64.8 months). Acute toxicity was limited to Grade 1-2 esophagitis, occurring in 40% of patients. No late toxicity was observed. Conclusions: RT prevented progressive amyloid deposition in 8 of 10 patients, resulting in a marginally increased forced expiratory volume in 1 second, and improved functional capacity, without late morbidity.

  4. The prognostic value of T1 mapping and late gadolinium enhancement cardiovascular magnetic resonance imaging in patients with light chain amyloidosis.

    Science.gov (United States)

    Lin, Lu; Li, Xiao; Feng, Jun; Shen, Kai-Ni; Tian, Zhuang; Sun, Jian; Mao, Yue-Ying; Cao, Jian; Jin, Zheng-Yu; Li, Jian; Selvanayagam, Joseph B; Wang, Yi-Ning

    2018-01-03

    Cardiac impairment is associated with high morbidity and mortality in immunoglobulin light chain (AL) type amyloidosis, for which early identification and risk stratification is vital. For myocardial tissue characterization, late gadolinium enhancement (LGE) is a classic and most commonly performed cardiovascular magnetic resonance (CMR) parameter. T1 mapping with native T1 and extracellular volume (ECV) are recently developed quantitative parameters. We aimed to investigate the prognostic value of native T1, ECV and LGE in patients with AL amyloidosis. Eighty-two patients (55.5 ± 8.5 years; 52 M) and 20 healthy subjects (53.2 ± 11.7 years; 10 M) were prospectively recruited. All subjects underwent CMR with LGE imaging and T1 mapping using a Modified Look-Locker Inversion-recovery (MOLLI) sequence on a 3 T scanner. Native T1 and ECV were measured semi-automatically using a dedicated CMR software. The left ventricular (LV) LGE pattern was classified as none, patchy, and global groups. Global LGE was considered when there was diffuse, transmural LGE in more than half of the short axis images. Follow-up was performed for all-cause mortality using Cox proportional hazards regression analysis and Kaplan-Meier survival curves. The patients demonstrated an increase in native T1 (1438 ± 120 ms vs. 1283 ± 46 ms, P = 0.001) and ECV (43.9 ± 10.9% vs. 27.0 ± 1.7%, P = 0.001) compared to healthy controls. Native T1, ECV and LGE showed significant correlation with Mayo Stage, and ECV and LGE showed significant correlation with echocardiographic E/E' and LV ejection fraction. During the follow-up for a median time of 8 months, 21 deaths occurred. ECV ≥ 44.0% (hazard ratio [HR] 7.249, 95% confidence interval (CI) 1.751-13.179, P = 0.002) and global LGE (HR 4.804, 95% CI 1.971-12.926, P = 0.001) were independently prognostic for mortality over other clinical and imaging parameters. In subgroups with the same LGE pattern

  5. Peripherally applied synthetic peptide isoAsp7-Aβ(1-42) triggers cerebral β-amyloidosis.

    Science.gov (United States)

    Kozin, S A; Cheglakov, I B; Ovsepyan, A A; Telegin, G B; Tsvetkov, P O; Lisitsa, A V; Makarov, A A

    2013-10-01

    Intracerebral and intraperitoneal inoculation with β-amyloid-rich brain extracts originating from patients with Alzheimer's disease as well as intracerebral injection of aggregates composed of synthetic Aβ can induce cerebral β-amyloidosis, and associated cognitive dysfunctions in susceptible animal hosts. We have found that repetitive intravenous administration of 100 μg of synthetic peptide corresponding to isoAsp7-containing Aβ(1-42), an abundant age-dependent Aβ isoform present both in the pathological brain and in synthetic Aβ preparations, robustly accelerates formation of classic dense-core congophilic amyloid plaques in the brain of β-amyloid precursor protein transgenic mice. Our findings indicate this peptide as an inductive agent of cerebral β-amyloidosis in vivo.

  6. The hormonal and radiological evaluation of adrenal glands, and the determination of the usefulness of low dose ACTH test in patients with renal amyloidosis.

    Science.gov (United States)

    Gündüz, Z; Keleştimur, F; Durak, A C; Utaş, C; Büyükberber, M; Düşünsel, R; Kurtoğlu, S; Poyrazoglu, M H

    2001-03-01

    Amyloidosis is a multisystem disease which may cause organ loss. Renal involvement is the most common clinical problem in amyloidosis, however involvement of endocrin organs is possible. In this study to assess adrenocortical function and to evaluate the usefulness of low dose ACTH test in patients with renal amyloidosis, we determined cortisol, 17-hydroxyprogesteron (17-OHP) and 11-deoxycortisol (11-DOC) responses to both 1 microg and 250 microg Synacthen. We also determined the size of adrenal glands radiologically by using computerized tomography. Twenty one patients with renal amyloidosis and 16 healthy subjects for hormonal evaluation, and 20 patients with renal amyloidosis and 22 healthy subjects for radiologic evaluation were included in the study. In four patients (19%) peak serum cortisol levels following stimulation with the low dose of Synacthen were less than 20 microg/dL (550 nmol/L). Two of them had also subnormal cortisol response to the 250 microg Synacthen stimulation test. Basal and stimulated levels of 11-DOC were lower than those of control values (p=0.000 and p<0.01 respectively). The mean 11-DOC responses to stimulation with 1 microg Synacthen were also significantly lower than the values obtained after the simulation with 250 microg Synacthen (p<0.01 and p=0.000). Cortisol responses to the stimulation with 250 microg Synacthen were also lower than the control responses (p<0.05). 17-OHP responses were similar to the control values in both tests. In the radiological evaluation the mean maximum width of right adrenal glands and the mean anterior and maximum width of left adrenal glands were significantly greater in the patient group (p<0.01). In conclusion, adrenal involvement and adrenal insufficiency is common in amyloidosis. Low 11-DOC levels in amyloidosis is a new finding and further detailed studies is required to explain its cause.

  7. Autonomic Neuropathy and Albuminocytologic Dissociation in Cerebrospinal Fluid As the Presenting Features of Primary Amyloidosis: A Case Report

    Directory of Open Access Journals (Sweden)

    Jingjing Li

    2017-07-01

    Full Text Available ObjectivePrimary amyloidosis is a disease with a poor prognosis and multi-organ involvement. Here, we report the clinical and pathological features of a patient with primary amyloidosis featuring autonomic neuropathy as the initial symptom and albuminocytologic dissociation in the cerebrospinal fluid (CSF.MethodsThe patient was a 60-year-old Chinese male with numbness, orthostatic hypotension, and gastrointestinal symptoms. For diagnosis, we performed an electromyogram (EMG, lumbar puncture, Bence Jones protein urine test, serum electrophoresis blood test, sural nerve and rectal membrane biopsies, transthyretin (TTR gene sequencing, and bone marrow puncture.ResultsCongo red staining of sural nerve and rectal membrane biopsies showed amyloid deposition and apple-green birefringence was visualized under polarized light microscopy. TTR gene sequencing showed no causative mutation. Following lumbar puncture, normal CSF cell counts and elevated CSF protein concentration (1,680 mg/L were detected. Bone marrow puncture showed that out of the total number of whole blood cells, 0.56% were abnormal plasma cells and that 87.4% of the total number of plasma cells were abnormal. EMG results showed mixed peripheral nerve damage predominately in the sensory nerve fibers.ConclusionObvious symptoms of neuropathy, particularly autonomic neuropathy, albuminocytologic dissociation, and organ function damage suggested a diagnosis of amyloidosis. In such patients, neurologists should use caution to differentiate between chronic inflammatory demyelinating polyneuropathy, primary amyloidosis, and familial amyloid neuropathy.

  8. Skin deposits in hereditary cystatin C amyloidosis

    DEFF Research Database (Denmark)

    Benedikz, Eirikur; Blöndal, H; Gudmundsson, G

    1990-01-01

    Clinically normal skin from 47 individuals aged 9-70 years was investigated. Cystatin C amyloid deposits were found in various locations of the skin by light and/or electron microscopy, in all 12 patients with a clinical history of hereditary cystatin C amyloidosis (HCCA). Six asymptomatic...... individuals, who had the Alu 1 restriction fragment length polymorphism (RFLP) marker reported to cosegregate with the disease, also had cystatin C amyloid deposits in the skin. Three asymptomatic individuals (age 17-46) belonging to the HCCA families were without amyloid in the skin but had Alu 1 RFLP marker...

  9. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis.

    Science.gov (United States)

    Bever, Katherine M; Masha, Luke I; Sun, Fangui; Stern, Lauren; Havasi, Andrea; Berk, John L; Sanchorawala, Vaishali; Seldin, David C; Sloan, J Mark

    2016-01-01

    Patients with immunoglobulin light chain amyloidosis are at risk for both thrombotic and bleeding complications. While the hemostatic defects have been extensively studied, less is known about thrombotic complications in this disease. This retrospective study examined the frequency of venous thromboembolism in 929 patients with immunoglobulin light chain amyloidosis presenting to a single referral center, correlated risk of venous thromboembolism with clinical and laboratory factors, and examined complications of anticoagulation in this population. Sixty-five patients (7%) were documented as having at least one venous thromboembolic event. Eighty percent of these patients had events within one year prior to or following diagnosis. Lower serum albumin was associated with increased risk of VTE, with a hazard ratio of 4.30 (CI 1.60-11.55; P=0.0038) for serum albumin less than 3 g/dL compared to serum albumin greater than 4 g/dL. Severe bleeding complications were observed in 5 out of 57 patients with venous thromboembolism undergoing treatment with anticoagulation. Prospective investigation should be undertaken to better risk stratify these patients and to determine the optimal strategies for prophylaxis against and management of venous thromboembolism. Copyright© Ferrata Storti Foundation.

  10. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis

    Science.gov (United States)

    Bever, Katherine M.; Masha, Luke I.; Sun, Fangui; Stern, Lauren; Havasi, Andrea; Berk, John L.; Sanchorawala, Vaishali; Seldin, David C.; Sloan, J. Mark

    2016-01-01

    Patients with immunoglobulin light chain amyloidosis are at risk for both thrombotic and bleeding complications. While the hemostatic defects have been extensively studied, less is known about thrombotic complications in this disease. This retrospective study examined the frequency of venous thromboembolism in 929 patients with immunoglobulin light chain amyloidosis presenting to a single referral center, correlated risk of venous thromboembolism with clinical and laboratory factors, and examined complications of anticoagulation in this population. Sixty-five patients (7%) were documented as having at least one venous thromboembolic event. Eighty percent of these patients had events within one year prior to or following diagnosis. Lower serum albumin was associated with increased risk of VTE, with a hazard ratio of 4.30 (CI 1.60–11.55; P=0.0038) for serum albumin less than 3 g/dL compared to serum albumin greater than 4 g/dL. Severe bleeding complications were observed in 5 out of 57 patients with venous thromboembolism undergoing treatment with anticoagulation. Prospective investigation should be undertaken to better risk stratify these patients and to determine the optimal strategies for prophylaxis against and management of venous thromboembolism. PMID:26452981

  11. Experimental transmission of AA amyloidosis by injecting the AA amyloid protein into interleukin-1 receptor antagonist knockout (IL-1raKO) mice.

    Science.gov (United States)

    Watanabe, K; Uchida, K; Chambers, J K; Tei, M; Shoji, A; Ushio, N; Nakayama, H

    2015-05-01

    The incidence of AA amyloidosis is high in humans with rheumatoid arthritis and several animal species, including cats and cattle with prolonged inflammation. AA amyloidosis can be experimentally induced in mice using severe inflammatory stimuli and a coinjection of AA amyloid; however, difficulties have been associated with transmitting AA amyloidosis to a different animal species, and this has been attributed to the "species barrier." The interleukin-1 receptor antagonist knockout (IL-1raKO) mouse, a rodent model of human rheumatoid arthritis, has been used in the transmission of AA amyloid. When IL-1raKO and BALB/c mice were intraperitoneally injected with mouse AA amyloid together with a subcutaneous pretreatment of 2% AgNO3, all mice from both strains that were injected with crude or purified murine AA amyloid developed AA amyloidosis. However, the amyloid index, which was determined by the intensity of AA amyloid deposition, was significantly higher in IL-1raKO mice than in BALB/c mice. When IL-1raKO and BALB/c mice were injected with crude or purified bovine AA amyloid together with the pretreatment, 83% (5/6 cases) and 38% (3/8 cases) of IL-1raKO mice and 17% (1/6 cases) and 0% (0/6 cases) of BALB/c mice, respectively, developed AA amyloidosis. Similarly, when IL-1raKO and BALB/c mice were injected with crude or purified feline AA amyloid, 33% (2/6 cases) and 88% (7/8 cases) of IL-1raKO mice and 0% (0/6 cases) and 29% (2/6 cases) of BALB/c mice, respectively, developed AA amyloidosis. These results indicated that IL-1raKO mice are a useful animal model for investigating AA amyloidogenesis. © The Author(s) 2014.

  12. Transbronchial biopsies safely diagnose amyloid lung disease

    Science.gov (United States)

    Govender, Praveen; Keyes, Colleen M.; Hankinson, Elizabeth A.; O’Hara, Carl J.; Sanchorawala, Vaishali; Berk, John L.

    2018-01-01

    Background Autopsy identifies lung involvement in 58–92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort. We reviewed our experience with transbronchial biopsies in patients with amyloidosis to determine the safety and utility of bronchoscopic lung biopsies. Methods We identified patients referred to the Amyloidosis Center at Boston Medical Center with lung amyloidosis diagnosed by transbronchial lung biopsies (TBBX). Amyloid typing was determined by immunohistochemistry or mass spectrometry. Standard end organ assessments, including pulmonary function test (PFT) and chest tomography (CT) imaging, and extra-thoracic biopsies established the extent of disease. Results Twenty-five (21.7%) of 115 patients with lung amyloidosis were diagnosed by TBBX. PFT classified 33.3% with restrictive physiology, 28.6% with obstructive disease, and 9.5% mixed physiology; 9.5% exhibited isolated diffusion defects while 19% had normal pulmonary testing. Two view chest or CT imaging identified focal opacities in 52% of cases and diffuse interstitial disease in 48%. Amyloid type and disease extent included 68% systemic AL disease, 16% localized (lung limited) AL disease, 12% ATTR disease, and 4% AA amyloidosis. Fluoroscopy was not used during biopsy. No procedure complications were reported. Conclusions Our case series of 25 patients supports the use of bronchoscopic transbronchial biopsies for diagnosis of parenchymal lung amyloidosis. Normal PFTs do not rule out the histologic presence of amyloid lung disease. PMID:28393574

  13. Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease

    International Nuclear Information System (INIS)

    van Duinen, S.G.; Castano, E.M.; Prelli, F.; Bots, G.T.A.B.; Luyendijk, W.; Frangione, B.

    1987-01-01

    Hereditary cerebral hemorrhage with amyloidosis in Dutch patients is an autosomal dominant form of vascular amyloidosis restricted to the leptomeninges and cerebral cortex. Clinically the disease is characterized by cerebral hemorrhages leading to an early death. Immunohistochemical studies of five patients revealed that the vascular amyloid deposits reacted intensely with an antiserum raised against a synthetic peptide homologous to the Alzheimer disease-related β-protein. Silver stain-positive, senile plaque-like structures were also labeled by the antiserum, yet these lesions lacked the dense amyloid cores present in typical plaques of Alzheimer disease. No neurofibrillary tangles were present. Amyloid fibrils were purified from the leptomeningeal vessels of one patient who clinically had no signs of dementia. The protein had a molecular weight of ∼ 4000 and its partial amino acid sequence to position 21 showed homology to the β-protein of Alzheimer disease and Down syndrome. These results suggest that hereditary cerebral hemorrhage with amyloidosis of Dutch origin is pathogenetically related to Alzheimer disease and support the concept that the initial amyloid deposition in this disorder occurs in the vessel walls before damaging the brain parenchyma. Thus, deposition of β-protein in brain tissue seems to be related to a spectrum of diseases involving vascular syndromes, progressive dementia, or both

  14. Three-dimensional Speckle Tracking Echocardiography in Light Chain Cardiac Amyloidosis: Examination of Left and Right Ventricular Myocardial Mechanics Parameters.

    Science.gov (United States)

    Urbano-Moral, Jose Angel; Gangadharamurthy, Dakshin; Comenzo, Raymond L; Pandian, Natesa G; Patel, Ayan R

    2015-08-01

    The study of myocardial mechanics has a potential role in the detection of cardiac involvement in patients with amyloidosis. This study aimed to characterize 3-dimensional-speckle tracking echocardiography-derived left and right ventricular myocardial mechanics in light chain amyloidosis and examine their relationship with brain natriuretic peptide. In patients with light chain amyloidosis, left ventricular longitudinal and circumferential strain (n=40), and right ventricular longitudinal strain and radial displacement (n=26) were obtained by 3-dimensional-speckle tracking echocardiography. Brain natriuretic peptide levels were determined. All myocardial mechanics measurements showed differences when compared by brain natriuretic peptide level tertiles. Left and right ventricular longitudinal strain were highly correlated (r=0.95, P<.001). Left ventricular longitudinal and circumferential strain were reduced in patients with cardiac involvement (-9±4 vs -16±2; P<.001, and -24±6 vs -29±4; P=.01, respectively), with the most prominent impairment at the basal segments. Right ventricular longitudinal strain and radial displacement were diminished in patients with cardiac involvement (-9±3 vs -17±3; P<.001, and 2.7±0.8 vs 3.8±0.3; P=.002). On multivariate analysis, left ventricular longitudinal strain was associated with the presence of cardiac involvement (odds ratio = 1.6; 95% confidence interval, 1.04 to 2.37; P=.03) independent of the presence of brain natriuretic peptide and troponin I criteria for cardiac amyloidosis. Three-dimensional-speckle tracking echocardiography-derived left and right ventricular myocardial mechanics are increasingly altered as brain natriuretic peptide increases in light chain amyloidosis. There appears to be a strong association between left ventricular longitudinal strain and cardiac involvement, beyond biomarkers such as brain natriuretic peptide and troponin I. Copyright © 2015 Sociedad Española de Cardiología. Published by

  15. /Cu-Al System

    Science.gov (United States)

    Kish, Orel; Froumin, Natalya; Aizenshtein, Michael; Frage, Nachum

    2014-05-01

    Wettability and interfacial interaction of the Ta2O5/Cu-Al system were studied. Pure Cu does not wet the Ta2O5 substrate, and improved spreading is achieved when relatively a high fraction of the active element (~40 at.% Al) was added. The Al2O3 and AlTaO4 phases were observed at the Ta2O5/Cu-Al interface. A thermodynamic evaluation allowed us to suggest that the lack of wetting bellow 40 at.% Al is due to the presence of a native oxide, which covers the drop. The conditions of the native oxide decomposition and the formation of the volatile Al2O suboxide strongly depend on the vacuum level during sessile drop experiments and the composition of the Cu-Al alloy. In our case, Al contents greater than 40% provides thermodynamic conditions for the formation of Al2O (as a result of Al reaction with Al2O3) and the drop spreading. It was suggested that the final contact angle in the Ta2O5/Cu-Al system (50°) is determined by Ta adsorption on the newly formed alumina interlayer.

  16. Rheumatic polymyalgia like presentation of multiple myeloma and amyloidosis

    International Nuclear Information System (INIS)

    Medina, Yimy F; Martinez, Jose Bernardo; Restrepo, Jose Felix; Rondon, Federico; Iglesias Gamarra, Antonio

    2005-01-01

    Polymyalgia rheumatica is a disorder with defined clinical characteristics and may be the initial expression or to be associated to other diseases. Although it is not associated to neoplastic diseases, may be their initial manifestation. We report a patient who presented the initial complaining of polymyalgia rheumatica as a manifestation of another illness, amyloidosis associated to multiple myeloma. We also discuss the clinical characteristics of these entities and revised the available literature with regard to this association

  17. Human amyloidogenic light chain proteins result in cardiac dysfunction, cell death, and early mortality in zebrafish.

    Science.gov (United States)

    Mishra, Shikha; Guan, Jian; Plovie, Eva; Seldin, David C; Connors, Lawreen H; Merlini, Giampaolo; Falk, Rodney H; MacRae, Calum A; Liao, Ronglih

    2013-07-01

    Systemic amyloid light-chain (AL) amyloidosis is associated with rapidly progressive and fatal cardiomyopathy resulting from the direct cardiotoxic effects of circulating AL light chain (AL-LC) proteins and the indirect effects of AL fibril tissue infiltration. Cardiac amyloidosis is resistant to standard heart failure therapies, and, to date, there are limited treatment options for these patients. The mechanisms underlying the development of cardiac amyloidosis and AL-LC cardiotoxicity are largely unknown, and their study has been limited by the lack of a suitable in vivo model system. Here, we establish an in vivo zebrafish model of human AL-LC-induced cardiotoxicity. AL-LC isolated from AL cardiomyopathy patients or control nonamyloidogenic LC protein isolated from multiple myeloma patients (Con-LC) was directly injected into the circulation of zebrafish at 48 h postfertilization. AL-LC injection resulted in impaired cardiac function, pericardial edema, and increased cell death relative to Con-LC, culminating in compromised survival with 100% mortality within 2 wk, independent of AL fibril deposition. Prior work has implicated noncanonical p38 MAPK activation in the pathogenesis of AL-LC-induced cardiotoxicity, and p38 MAPK inhibition via SB-203580 rescued AL-LC-induced cardiac dysfunction and cell death and attenuated mortality in zebrafish. This in vivo zebrafish model of AL-LC cardiotoxicity demonstrates that antagonism of p38 MAPK within the AL-LC cardiotoxic signaling response may serve to improve cardiac function and mortality in AL cardiomyopathy. Furthermore, this in vivo model system will allow for further study of the molecular underpinnings of AL cardiotoxicity and identification of novel therapeutic strategies.

  18. Microglia in diffuse plaques in hereditary cerebral hemorrhage with amyloidosis (Dutch). An immunohistochemical study

    NARCIS (Netherlands)

    Maat-Schieman, M. L.; Rozemuller, A. J.; van Duinen, S. G.; Haan, J.; Eikelenboom, P.; Roos, R. A.

    1994-01-01

    In hereditary cerebral hemorrhage with amyloidosis (Dutch) (HCHWA-D) beta/A4 amyloid deposition is found in meningocortical blood vessels and in diffuse plaques in the cerebral cortex. Diffuse plaques putatively represent early stages in the formation of senile plaques. Microglia are intimately

  19. Association Between Serum Triglycerides and Cerebral Amyloidosis in Cognitively Normal Elderly.

    Science.gov (United States)

    Choi, Hyo Jung; Byun, Min Soo; Yi, Dahyun; Choe, Young Min; Sohn, Bo Kyung; Baek, Hye Won; Lee, Jun Ho; Kim, Hyun Jung; Han, Ji Young; Yoon, Eun Jin; Kim, Yu Kyeong; Woo, Jong Inn; Lee, Dong Young

    2016-08-01

    Although many preclinical studies have suggested the possible linkage between dyslipidemia and cerebral amyloid deposition, the association between serum lipid measures and cerebral amyloid-beta (Aβ) deposition in human brain is still poorly known. We aimed to investigate the association in cognitively normal (CN) elderly individuals. Cross-sectional study. University hospital dementia clinic. 59 CN elderly. The study measures included comprehensive clinical and neuropsychological assessment based on the CERAD protocol, magnetic resonance imaging and (11)C-labelled Pittsburgh Compound B positron emission tomography scans, and quantification for serum lipid biomarkers. Multiple linear regression analyses showed that a higher serum triglycerides level was associated with heavier global cerebral Aβ deposition even after controlling age, sex, and apolipoprotein E ε4 genotype. Serum apolipoprotein B also showed significant positive association with global cerebral Aβ deposition, but the significance disappeared after controlling serum triglycerides level. No association was found between other lipid measures and global cerebral Aβ deposition. The findings suggest that serum triglycerides are closely associated with cerebral amyloidosis, although population-based prospective studies are needed to provide further evidence of the causative effect of triglycerides on cerebral amyloidosis. Copyright © 2016 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

  20. Amyloidosis, Inflammation, and Oxidative Stress in the Heart of an Alkaptonuric Patient

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    Lia Millucci

    2014-01-01

    Full Text Available Background. Alkaptonuria, a rare autosomal recessive metabolic disorder caused by deficiency in homogentisate 1,2-dioxygenase activity, leads to accumulation of oxidised homogentisic acid in cartilage and collagenous structures present in all organs and tissues, especially joints and heart, causing a pigmentation called ochronosis. A secondary amyloidosis is associated with AKU. Here we report a study of an aortic valve from an AKU patient. Results. Congo Red birefringence, Th-T fluorescence, and biochemical assays demonstrated the presence of SAA-amyloid deposits in AKU stenotic aortic valve. Light and electron microscopy assessed the colocalization of ochronotic pigment and SAA-amyloid, the presence of calcified areas in the valve. Immunofluorescence detected lipid peroxidation of the tissue and lymphocyte/macrophage infiltration causing inflammation. High SAA plasma levels and proinflammatory cytokines levels comparable to those from rheumatoid arthritis patients were found in AKU patient. Conclusions. SAA-amyloidosis was present in the aortic valve from an AKU patient and colocalized with ochronotic pigment as well as with tissue calcification, lipid oxidation, macrophages infiltration, cell death, and tissue degeneration. A local HGD expression in human cardiac tissue has also been ascertained suggesting a consequent local production of ochronotic pigment in AKU heart.

  1. Amyloidosis, Inflammation, and Oxidative Stress in the Heart of an Alkaptonuric Patient

    Science.gov (United States)

    Ghezzi, Lorenzo; Giorgetti, Giovanna; Viti, Cecilia; Geminiani, Michela; Soldani, Patrizia; Lupetti, Pietro; Benvenuti, Chiara; Perfetto, Federico; Spreafico, Adriano; Santucci, Annalisa

    2014-01-01

    Background. Alkaptonuria, a rare autosomal recessive metabolic disorder caused by deficiency in homogentisate 1,2-dioxygenase activity, leads to accumulation of oxidised homogentisic acid in cartilage and collagenous structures present in all organs and tissues, especially joints and heart, causing a pigmentation called ochronosis. A secondary amyloidosis is associated with AKU. Here we report a study of an aortic valve from an AKU patient. Results. Congo Red birefringence, Th-T fluorescence, and biochemical assays demonstrated the presence of SAA-amyloid deposits in AKU stenotic aortic valve. Light and electron microscopy assessed the colocalization of ochronotic pigment and SAA-amyloid, the presence of calcified areas in the valve. Immunofluorescence detected lipid peroxidation of the tissue and lymphocyte/macrophage infiltration causing inflammation. High SAA plasma levels and proinflammatory cytokines levels comparable to those from rheumatoid arthritis patients were found in AKU patient. Conclusions. SAA-amyloidosis was present in the aortic valve from an AKU patient and colocalized with ochronotic pigment as well as with tissue calcification, lipid oxidation, macrophages infiltration, cell death, and tissue degeneration. A local HGD expression in human cardiac tissue has also been ascertained suggesting a consequent local production of ochronotic pigment in AKU heart. PMID:24876668

  2. Cardiac Light Chain Amyloidosis: The Role of Metal Ions in Oxidative Stress and Mitochondrial Damage.

    Science.gov (United States)

    Diomede, Luisa; Romeo, Margherita; Rognoni, Paola; Beeg, Marten; Foray, Claudia; Ghibaudi, Elena; Palladini, Giovanni; Cherny, Robert A; Verga, Laura; Capello, Gian Luca; Perfetti, Vittorio; Fiordaliso, Fabio; Merlini, Giampaolo; Salmona, Mario

    2017-09-20

    The knowledge of the mechanism underlying the cardiac damage in immunoglobulin light chain (LC) amyloidosis (AL) is essential to develop novel therapies and improve patients' outcome. Although an active role of reactive oxygen species (ROS) in LC-induced cardiotoxicity has already been envisaged, the actual mechanisms behind their generation remain elusive. This study was aimed at further dissecting the action of ROS generated by cardiotoxic LC in vivo and investigating whether transition metal ions are involved in this process. In the absence of reliable vertebrate model of AL, we used the nematode Caenorhabditis elegans, whose pharynx is an "ancestral heart." LC purified from patients with severe cardiac involvement intrinsically generated high levels of ROS and when administered to C. elegans induced ROS production, activation of the DAF-16/forkhead transcription factor (FOXO) pathway, and expression of proteins involved in stress resistance and survival. Profound functional and structural ROS-mediated mitochondrial damage, similar to that observed in amyloid-affected hearts from AL patients, was observed. All these effects were entirely dependent on the presence of metal ions since addition of metal chelator or metal-binding 8-hydroxyquinoline compounds (chelex, PBT2, and clioquinol) permanently blocked the ROS production and prevented the cardiotoxic effects of amyloid LC. Innovation and Conclusion: Our findings identify the key role of metal ions in driving the ROS-mediated toxic effects of LC. This is a novel conceptual advance that paves the way for new pharmacological strategies aimed at not only counteracting but also totally inhibiting the vicious cycle of redox damage. Antioxid. Redox Signal. 27, 567-582.

  3. Amiloidose pulmonar: relato de caso de achado radiológico da apresentação nodular em grande fumante Pulmonary amyloidosis: radiographic finding of nodular opacities in a heavy smoker

    Directory of Open Access Journals (Sweden)

    Jorge Montessi

    2007-06-01

    Full Text Available A amiloidose pulmonar é uma doença rara, caracterizada pelo depósito extracelular de proteínas fibrilares no pulmão. Amiloidose é um termo genérico para grupos heterogêneos de doenças, incluindo doença de Alzheimer e diabetes mellitus tipo II. Apresenta-se no aparelho respiratório sob as formas traqueobrônquica, nodular pulmonar e septal alveolar (parenquimatosa difusa. Relata-se o caso de uma mulher, tabagista (20 anos/maço, portadora de amiloidose nodular pulmonar, diagnosticada através de exames pré-operatórios à realização de colecistectomia videolaparoscópica.Pulmonary amyloidosis is a rare disease, characterized by extracellular deposition of fibrillary protein in the lungs. Amyloidosis is a generic term for a heterogeneous group of diseases, including Alzheimer's disease and type 2 diabetes mellitus. In the respiratory system, it appears in various forms: tracheobronchial; nodular pulmonary; and alveolar septal (diffuse parenchymal. We present the case of a woman who was a 20 pack-year smoker and had nodular pulmonary amyloidosis, as diagnosed through tests performed prior to laparoscopic cholecystectomy.

  4. Renal and suprarenal insufficiency secondary to familial Mediterranean fever associated with amyloidosis: a case report

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    Sari Nagehan

    2011-08-01

    Full Text Available Abstract Introduction Familial Mediterranean fever is an autosomal recessive disease that predominantly affects people of the Mediterranean coast. One of the most frequent complications of the disease is amyloidosis. This clinical entity is known as secondary (also called AA amyloidosis. Case presentation In this report, we describe the case of a 33-year-old Turkish man with familial Mediterranean fever and chronic renal insufficiency. He was admitted to our clinic with symptoms of suprarenal insufficiency. The patient died three months later as a result of cardiac arrest. Conclusion Our aim is to make a contribution to the literature by reporting a case of combined insufficiency due to the accumulation of renal and adrenal amyloid in a patient with familial Mediterranean fever, which has very rarely been described in the literature. We hope that adrenal insufficiency, which becomes fatal if not diagnosed and treated rapidly, will come to mind as easily as chronic renal failure in clinical practice.

  5. The pathogenesis of amyloidosis in periodic disease: Some aspects

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    Z. T. Djndoyan

    2014-07-01

    Full Text Available Sufficient information indicating the implication of dysfunction of interleukins (IL-6 and IL-1 in particular in the pathogenesis of amyloidosis in a number of autoinflammatory, rheumatic, and autoimmune diseases, including those in periodic disease (PD, has been recently accumulated. Its genetic defect – pirin mutation – gives rise to an alternative innate immune response (phagocytic cell activation to secrete IL-1 by macrophages and to activate T-helper cells. This causes imbalance in the synthesis of proinflammatory (IL-6, IL-8, and TNF-α and anti-inflammatory (IL-4, IL-10, and IL-1 receptor antagonist cytokines. Moreover, the uncontrolled macrophage (monocyte secretion of a great deal of IL-6 that together with IL-1 is a mediator of the synthesis of the serum amyloid fibril protein precursor SAA by hepatocytes, neutrophils, and fibroblasts plays one of the key roles in the pathogenesis of PD through amyloidosis. With this, IL-6 stimulates the inflammatory process, by enhancing the release of lysosomal enzymes, reactive oxygen species, and eicosanoids (prostaglandins, leukotrienes, thromboxane from the polymorphic nuclear leukocytes, macrophages, endotheliocytes, and fibroblasts and by augmenting the chemotaxis of macrophages and neutrophils, and the degranulation of the latter, i.e. through its action on the effector cells of inflammation, and prepares the tissue basis for amyloid deposits in this fashion. Thus, the analysis of literary and own materials gives grounds to suggest that pirin mutation is a trigger of the synthesis of IL-1 and IL-6 in PD and their hypersecretion is an initial link of the synthesis of SAA.

  6. Experimental induction of chicken amyloid A amyloidosis in white layer chickens by inoculation with inactivated vaccines.

    Science.gov (United States)

    Habibi, Wazir Ahmad; Hirai, Takuya; Niazmand, Mohammad Hakim; Okumura, Naoko; Yamaguchi, Ryoji

    2017-10-01

    We investigated the amyloidogenic potential of inactivated vaccines and the localized production of serum amyloid A (SAA) at the injection site in white layer chickens. Hens in the treated group were injected intramuscularly three times with high doses of inactivated oil-emulsion Salmonella Enteritidis vaccine and multivalent viral and bacterial inactivated oil-emulsion vaccines at two-week intervals. Chickens in the control group did not receive any inoculum. In the treated group, emaciation and granulomas were present, while several chickens died between 4 and 6 weeks after the first injection. Hepatomegaly was seen at necropsy, and the liver parenchyma showed inconsistent discolouration with patchy green to yellowish-brown areas, or sometimes red-brown areas with haemorrhage. Amyloid deposition in the liver, spleen, duodenum, and at injection sites was demonstrated using haematoxylin and eosin staining, Congo red, and immunohistochemistry. The incidence of chicken amyloid A (AA) amyloidosis was 47% (28 of 60) in the treated group. In addition, RT-PCR was used to identify chicken SAA mRNA expression in the liver and at the injection sites. Furthermore, SAA mRNA was detected by in situ hybridization in fibroblasts at the injection sites, and also in hepatocytes. We believe that this is the first report of the experimental induction of systemic AA amyloidosis in white layer chickens following repeated inoculation with inactivated vaccines without the administration of amyloid fibrils or other amyloid-enhancing factors.

  7. Detection of AA76, a Common Form of Amyloid A Protein, as a Way of Diagnosing AA Amyloidosis.

    Science.gov (United States)

    Sato, Junji; Okuda, Yasuaki; Kuroda, Takeshi; Yamada, Toshiyuki

    2016-01-01

    Reactive amyloid deposits consist of amyloid A (AA) proteins, the degradation products of serum amyloid A (SAA). Since the most common species of AA is the amino terminal portion produced by cleavage between residues 76 and 77 of SAA (AA76), the presence of AA76 in tissues could be a consequence of AA amyloid deposition. This study assessed the diagnostic significance of the detection of AA76 for AA amyloidosis using two different approaches. Biopsy specimens (n=130 from 54 subjects) from gastroduodenal mucosa or abdominal fat (n=9 from 9 subjects) of patients who had already been diagnosed with or were suspected of having AA amyloidosis were used. Fixed mucosal sections were subjected to immunohistochemistry using a newly developed antibody recognizing the carboxyl terminal end of AA76 (anti-AA76). The non-fixed materials from gastroduodenal mucosa or abdominal fat were subjected to immunoblotting for detection of the size of AA76. Among the gastroduodenal specimens (n=115) from already diagnosed patients, the positive rates of Congo red staining, immunohistochemistry using anti-AA76, and immunoblotting were 68.4%, 73.0%, and 92.2%, respectively. The anti-AA76 did not stain the supposed SAA in the blood or leakage, which was stained by anti-SAA antibody. AA76 was not detected either by immunohistochemistry or by immunoblot in the materials from patients in whom AA amyloidosis had been ruled out. In the abdominal fat, the immunoblot detected AA76 in 8 materials from 8 already diagnosed patients and did not in 1 patient whose gastroduodenal mucosa was negative. In conclusion, the detection of AA76 may alter the ability to diagnose AA amyloidosis. In immunohistochemistry for fixed specimens, the new anti-AA76 antibody can improve the specificity. Immunoblot for non-fixed materials, which can considerably improve the sensitivity, should be beneficial for small materials like abdominal fat. © 2016 by the Association of Clinical Scientists, Inc.

  8. Cardiac Amyloidosis and its New Clinical Phenotype: Heart Failure with Preserved Ejection Fraction.

    Science.gov (United States)

    Mesquita, Evandro Tinoco; Jorge, Antonio José Lagoeiro; Souza, Celso Vale; Andrade, Thais Ribeiro de

    2017-07-01

    Heart failure with preserved ejection fraction (HFpEF) is now an emerging cardiovascular epidemic, being identified as the main phenotype observed in clinical practice. It is more associated with female gender, advanced age and comorbidities such as hypertension, diabetes, obesity and chronic kidney disease. Amyloidosis is a clinical disorder characterized by the deposition of aggregates of insoluble fibrils originating from proteins that exhibit anomalous folding. Recently, pictures of senile amyloidosis have been described in patients with HFpEF, demonstrating the need for clinical cardiologists to investigate this etiology in suspect cases. The clinical suspicion of amyloidosis should be increased in cases of HFPS where the cardio imaging methods are compatible with infiltrative cardiomyopathy. Advances in cardio imaging methods combined with the possibility of performing genetic tests and identification of the type of amyloid material allow the diagnosis to be made. The management of the diagnosed patients can be done in partnership with centers specialized in the study of amyloidosis, which, together with the new technologies, investigate the possibility of organ or bone marrow transplantation and also the involvement of patients in clinical studies that evaluate the action of the new emerging drugs. Resumo A insuficiência cardíaca com fração de ejeção preservada (ICFEP) é hoje uma epidemia cardiovascular emergente, sendo identificada como o principal fenótipo observado na prática clínica. Está mais associado ao sexo feminino, idade avançada e a comorbidades como hipertensão arterial, diabetes, obesidade e doença renal crônica. A amiloidose é uma desordem clínica caracterizada pelo depósito de agregados de fibrilas insolúveis originadas de proteínas que apresentam dobramento anômalo. Recentemente, têm sido descritos quadros de amiloidose senil em pacientes com ICFEP, demonstrando a necessidade de os cardiologistas clínicos investigarem

  9. Frequency of and Prognostic Significance of Cardiac Involvement at Presentation in Hereditary Transthyretin-Derived Amyloidosis and the Value of N-Terminal Pro-B-Type Natriuretic Peptide.

    Science.gov (United States)

    Klaassen, Sebastiaan H C; Tromp, Jasper; Nienhuis, Hans L A; van der Meer, Peter; van den Berg, Maarten P; Blokzijl, Hans; van Veldhuisen, Dirk J; Hazenberg, Bouke P C

    2018-01-01

    The aim of this study is to assess the prevalence of cardiac involvement in hereditary transthyretin-derived (ATTRm) amyloidosis at the time of diagnosis and to determine the diagnostic and clinical value of N-terminal pro-B-type natriuretic peptide (NT-proBNP). The University Medical Center Groningen is the national center of expertise for amyloidosis. All consecutive patients between 1994 and 2016 with ATTRm amyloidosis were followed prospectively. Baseline was set at the time of the first positive biopsy. All patients underwent a standard cardiac and neurologic work-up. Cardiac involvement was defined by otherwise unexplained left and/or right ventricular wall hypertrophy on cardiac ultrasound and/or advanced conduction disturbances. Seventy-seven patients had ATTRm amyloidosis and were included in the study. The TTR V30M mutation was present in 30 patients (39%). In both the V30M and the non-V30M groups, the neurologic presentation dominated (77% vs 51%), whereas cardiac presentation was infrequent (7% vs 15%). Clinical work-up showed that cardiac involvement was present at baseline in 51% of all patients irrespective of genotype and was associated with increased overall mortality (hazard ratio 5.95, 95% confidence interval 2.12 to 16.7), independent from clinical confounders. At a cutoff level of 125 ng/L, NT-proBNP had a sensitivity of 92% for establishing cardiac involvement. In conclusion, irrespective of the frequent noncardiac presentation of ATTRm amyloidosis, cardiac involvement is already present at diagnosis in half of the patients and is associated with increased mortality. NT-proBNP is a useful marker to determine cardiac involvement in this disease. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Tracheobronchial Amyloidosis Mimicking Tracheal Tumor

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    Elif Tanrıverdi

    2016-01-01

    Full Text Available Tracheobronchial amyloidosis is a rare presentation and accounts for about 1% of benign tumors in this area. The diagnosis of disease is delayed due to nonspecific pulmonary symptoms. Therapeutic approaches are required to control progressive pulmonary symptoms for most of the patients. Herein, we report a case of a 68-year-old man admitted with progressive dyspnea to our institution for further evaluation and management. He was initially diagnosed with and underwent management for bronchial asthma for two years but had persistent symptoms despite optimal medical therapy. Pulmonary computed tomography scan revealed severe endotracheal stenosis. Bronchoscopy was performed and showed endotracheal mass obstructing 70% of the distal trachea and mimicking a neoplastic lesion. The mass was successfully resected by mechanical resection, argon plasma coagulation (APC, and Nd-YAG laser during rigid bronchoscopy. Biopsy materials showed deposits of amorphous material by hematoxylin and eosin staining and these deposits were selectively stained with Congo Red. Although this is a rare clinical condition, this case indicated that carrying out a bronchoscopy in any patient complaining of atypical bronchial symptoms or with uncontrolled asthma is very important.

  11. Predicting glass-forming compositions in the Al-La and Al-La-Ni systems

    International Nuclear Information System (INIS)

    Gargarella, P.; de Oliveira, M.F.; Kiminami, C.S.; Pauly, S.; Kuehn, U.; Bolfarini, C.; Botta, W.J.; Eckert, J.

    2011-01-01

    Research highlights: → The glass-forming ability of the Al-La and Al-La-Ni systems was studied using the λ* and the λ.Δe criteria. → Both criteria predicted with just 1% at. of error the best glass-former verified so far in the Al-La system. → Four new glass-former compositions could be predicted in the Al-La-Ni system using the λ.Δe criterion. → The best glass-former reported so far in the Al-La-Ni system was found. - Abstract: In this work, a criterion considering the topological instability (λ) and the differences in the electronegativity of the constituent elements (Δe) was applied to the Al-La and Al-Ni-La systems in order to predict the best glass-forming compositions. The results were compared with literature data and with our own experimental data for the Al-La-Ni system. The alloy described in the literature as the best glass former in the Al-La system is located near the point with local maximum for the λ.Δe criterion. A good agreement was found between the predictions of the λ.Δe criterion and literature data in the Al-La-Ni system, with the region of the best glass-forming ability (GFA) and largest supercooled liquid region (ΔT x ) coinciding with the best compositional region for amorphization indicated by the λ.Δe criterion. Four new glassy compositions were found in the Al-La-Ni system, with the best predicted composition presenting the best glass-forming ability observed so far for this system. Although the λ.Δe criterion needs further refinements for completely describe the glass-forming ability in the Al-La and Al-La-Ni systems, the results demonstrated that this criterion is a good tool to predict new glass-forming compositions.

  12. Primary tracheobronchial amyloidosis: Two case reports Amiloidose traqueobrônquica primária: A propósito de dois casos clínicos

    Directory of Open Access Journals (Sweden)

    Sandra Saleiro

    2008-07-01

    Full Text Available Primary tracheobronchial amyloidosis is a form of respiratory amyloidosis, characterised by insoluble amyloid fibril proteins deposits along the airways wall. It is an uncommon disease, requiring a tissue sample to establish the definite diagnosis based on specific pathological features. The authors report two cases of tracheobronchial amyloidosis, describing their symptoms and the diagnostic and therapeutic procedures that were performed.A amiloidose traqueobrônquica primária é uma forma de amiloidose respiratória, caracterizada pela presença de depósitos insolúveis de proteína fibrilar amilóide, ao longo da parede das vias aéreas. É uma doença pouco frequente, sendo necessário uma amostra de tecido para estabelecer o diagnóstico definitivo, com base em características histológicas específicas. Os autores relatam dois casos clínicos de amiloidose traqueobrônquica, descrevendo os seus sintomas e os procedimentos diagnósticos e terapêuticos que foram efectuados.

  13. Nonfluent/Agrammatic PPA with In-Vivo Cortical Amyloidosis and Pick’s Disease Pathology

    Directory of Open Access Journals (Sweden)

    Francesca Caso

    2013-01-01

    Full Text Available The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA. She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom, post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer’s disease (AD (CERAD frequent/Braak Stage V was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.

  14. CRYPTOCOCCUS NEOFORMANS VAR. GRUBII-ASSOCIATED RENAL AMYLOIDOSIS CAUSING PROTEIN-LOSING NEPHROPATHY IN A RED KANGAROO (MACROPUS RUFUS).

    Science.gov (United States)

    Thurber, Mary Irene; Gjeltema, Jenessa; Sheley, Matthew; Wack, Ray F

    2017-09-01

    A 10-year-old male castrated red kangaroo (Macropus rufus) presented with mandibular swelling. Examination findings included pitting edema with no dental disease evident on examination or radiographs. The results of blood work were moderate azotemia, hypoalbuminemia, and severely elevated urine protein:creatinine ratio (9.9). Radiographs showed an interstitial pattern of the caudal right lung, and an abdominal ultrasound demonstrated scant effusion. Symptomatic and empirical therapy with antibiotics, anti-inflammatory drugs, and an angiotensin-converting enzyme (ACE) inhibitor did not resolve clinical signs. Due to poor prognosis and declining quality of life, euthanasia was elected. Necropsy revealed chronic granulomatous pneumonia of the caudal right lung lobe with intralesional Cryptococcus, identified as C. neoformans var. grubii by DNA sequencing. Severe bilateral glomerular and tubulointerstitial amyloidosis induced protein-losing nephropathy, leading to tri-cavitary effusion, subcutaneous edema, and cachexia. The authors speculate that renal amyloidosis was associated with chronic cryptococcal pneumonia in this red kangaroo.

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

    Science.gov (United States)

    Halloush, Ruba A; Lavrovskaya, Elena; Mody, Dina R; Lager, Donna; Truong, Luan

    2010-01-15

    Systemic amyloidosis (SA) has a broad nonspecific clinical presentation. Its diagnosis depends on identifying amyloid in tissues. Abdominal fat pad fine needle aspiration (FPFNA) has been suggested as a sensitive and specific test for diagnosing SA. Thirty-nine FPFNA from 38 patients (16 women and 20 men, age range 40-88 years) during a 15-year period were reviewed. Smears and cell blocks were stained with Congo red (CR). A panel of antibodies (serum amyloid protein, serum amyloid A, albumin, transthyretin, kappa light chain and lambda light chain) was used on six cell blocks from five patients. The FNA findings were correlated with clinical and histological follow-up. FPFNAs were positive, confirmed by CR in 5/39 (13%), suspicious in 1/39 (3%), negative in 28/39 (72%), and insufficient for diagnosis in 5/39 (13%) of cases. In all the positive cases, SA was confirmed within 2-16 weeks. Among the 28 negative cases, SA was diagnosed in 21, the rest were lost to follow-up. Among the insufficient cases, SA was diagnosed in four and one was lost to follow-up. Specificity was 100%, whereas sensitivity was 19%. SA typing using cell block sections was successful in three, un-interpretable in one, and negative in two cases. 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.

  16. Phase equilibria and crystalline structure of compounds in the Lu-Al and Lu-Cu-Al systems

    International Nuclear Information System (INIS)

    Kuz'ma, Yu.B.; Stel'makhovich, B.M.; Galamushka, L.I.

    1992-01-01

    Phase equilibria and crystal structure of compounds in Lu-Al and Lu-Cu-Al systems were studied. Existence of Lu 2 Al compound having the structure of the PbCl 2 type is ascertained. Diagram of phase equilibria of Lu-Cu-Al system at 870 K is plotted. Compounds Lu 2 (Cu,Al) 17 (the Th 2 Zn 17 type structure), Lu(Cu,Al) 5 (CaCu 5 type structure), Lu 6 (Cu,Al) 23 (Th 6 Mn 23 type structure) and ∼ LuCuAl 2 have been prepared for the first time. Investigation of component interaction in Lu-Cu-Al system shows that the system is similar to previously studied systems Dy-Cu-Al and Er-Cu-Al. The main difference consists in the absence of LuCuAl 3 compound with rhombic structure of the CeNi 2+x Sb 2-x type in the system investigated

  17. Differential recruitment efficacy of patient-derived amyloidogenic and myeloma light chain proteins by synthetic fibrils-A metric for predicting amyloid propensity.

    Directory of Open Access Journals (Sweden)

    Emily B Martin

    Full Text Available Monoclonal free light chain (LC proteins are present in the circulation of patients with immunoproliferative disorders such as light chain (AL amyloidosis and multiple myeloma (MM. Light chain-associated amyloid is a complex pathology composed of proteinaceous fibrils and extracellular matrix proteins found in all patients with AL and in ~10-30% of patients who presented with MM. Amyloid deposits systemically in multiple organs and tissues leading to dysfunction and ultimately death. The overall survival of patients with amyloidosis is worse than for those with early stage MM.We have developed a sensitive binding assay quantifying the recruitment of full length, patient-derived LC proteins by synthetic amyloid fibrils, as a method for studying their amyloidogenic potential. In a survey of eight urinary LC, both AL and MM-associated proteins were recruited by synthetic amyloid fibrils; however, AL-associated LC bound significantly more efficiently (p < 0.05 than did MM LCs. The LC proteins used in this study were isolated from urine and presumed to represent a surrogate of serum free light chains.The binding of LC to synthetic fibrils in this assay accurately differentiated LC with amyloidogenic propensity from MM LC that were not associated with clinical amyloid disease. Notably, the LC from a MM patient who subsequently developed amyloid behaved as an AL-associated protein in the assay, indicating the possibility for identifying MM patients at risk for developing amyloidosis based on the light chain recruitment efficacy. With this information, at risk patients can be monitored more closely for the development of amyloidosis, allowing timely administration of novel, amyloid-directed immunotherapies-this approach may improve the prognosis for these patients.

  18. Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis

    Directory of Open Access Journals (Sweden)

    Hund E

    2012-06-01

    Full Text Available Ernst HundDepartment of Neurology, University of Heidelberg, Heidelberg, GermanyAbstract: Transthyretin familial amyloid polyneuropathy (TTR-FAP is a fatal clinical disorder characterized by extracellular deposition of abnormal fibrils derived from misfolded, normally soluble transthyretin (TTR molecules. The disease is most commonly caused by a point mutation within the TTR gene inherited in an autosomal dominant fashion. Over 100 of such mutations have been identified, leading to destabilization of the physiological TTR tetramer. As a result, many monomers originate with a tendency for spontaneous conformational changes and self-aggregation. The main clinical feature of TTR-FAP is progressive sensorimotor and autonomic neuropathy. In the beginning, this polyneuropathy predominantly involves small unmyelinated nerve fibers with the result of dissociated sensory loss disproportionately affecting sensation of pain and temperature. Autonomic neuropathy typically accompanies sensory deficits early in the disease course. The symptoms include orthostatic hypotension, constipation alternating with diarrhea, erectile dysfunction, anhydrosis, and urinary retention or incontinence. Later, involvement of motor fibers causes rapidly progressive weakness and gait disturbances. In addition to the peripheral nervous system, the heart and the gut are frequently affected. Onset of symptoms is bimodal, with one peak at age 33 years (early onset and another distinct peak in the sixth decade of life (late onset. The course of TTR-FAP is uniformly progressive and fatal. Death occurs an average of 10.8 years after the onset of symptoms in Portuguese patients, and 7.3 years in late-onset Japanese patients. Common causes include cachexia, cardiac failure, arrhythmia, and secondary infections. Liver transplantation is the standard therapy for patients who are in a clinical condition good enough to tolerate this intervention because it stops progression of neuropathy by

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

    Directory of Open Access Journals (Sweden)

    Halloush Ruba

    2009-01-01

    Full Text Available Introduction: Systemic amyloidosis (SA has a broad nonspecific clinical presentation. Its diagnosis depends on identifying amyloid in tissues. Abdominal fat pad fine needle aspiration (FPFNA has been suggested as a sensitive and specific test for diagnosing SA. Materials and Methods: Thirty-nine FPFNA from 38 patients (16 women and 20 men, age range 40-88 years during a 15-year period were reviewed. Smears and cell blocks were stained with Congo red (CR. A panel of antibodies (serum amyloid protein, serum amyloid A, albumin, transthyretin, kappa light chain and lambda light chain was used on six cell blocks from five patients. The FNA findings were correlated with clinical and histological follow-up. Results: FPFNAs were positive, confirmed by CR in 5/39 (13%, suspicious in 1/39 (3%, negative in 28/39 (72%, and insufficient for diagnosis in 5/39 (13% of cases. In all the positive cases, SA was confirmed within 2-16 weeks. Among the 28 negative cases, SA was diagnosed in 21, the rest were lost to follow-up. Among the insufficient cases, SA was diagnosed in four and one was lost to follow-up. Specificity was 100%, whereas sensitivity was 19%. SA typing using cell block sections was successful in three, un-interpretable in one, and negative in two cases. Conclusion: 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.

  20. Amyloid myopathy: a diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Heli Tuomaala

    2009-08-01

    Full Text Available Amyloid myopathy (AM is a rare manifestation of primary systemic amyloidosis (AL. Like inflammatory myopathies, it presents with proximal muscle weakness and an increased creatine kinase level. We describe a case of AL with severe, rapidly progressive myopathy as the initial symptom. The clinical manifestation and muscle biopsy were suggestive of inclusion body myositis. AM was not suspected until amyloidosis was seen in the gastric mucosal biopsy. The muscle biopsy was then re-examined more specifically, and Congo red staining eventually showed vascular and interstitial amyloid accumulation, which led to a diagnosis of AM. The present case illustrates the fact that the clinical picture of AM can mimic that of inclusion body myositis.

  1. Liquidus projection of the Nb-Cr-Al system near the Al3(Nb,Cr) + Cr(Al,Nb) eutectic region

    International Nuclear Information System (INIS)

    Souza, S.A.; Ferrandini, P.L.; Nunes, C.A.; Coelho, A.A.; Caram, R.

    2006-01-01

    The system Nb-Cr-Al was investigated in the region near the Al 3 (Nb,Cr) + Cr(Al,Nb) eutectic and the liquidus projection of that region was determined based on the microstructural characterization of arc melted alloys. The characterization utilized scanning electron microscopy (SEM), energy dispersive spectroscopy (EDS), differential thermal analysis (DTA) and X-ray diffraction (XRD). The results allowed one to determine three primary solidification liquidus surfaces ((Cr,Al) 2 Nb, Cr(Al,Nb) and Al 3 (Nb,Cr)), that are originated from the binary systems Cr-Nb, Cr-Al and Al-Nb. It is proposed the occurrence of the invariant reaction L + (Cr,Al) 2 Nb ↔ Al 3 (Nb,Cr) + Cr(Al,Nb) and of a point of minimum, which involves a three phase reaction, L ↔ Al 3 (Nb,Cr) + Cr(Al,Nb). All alloys studied showed formation of the Al 3 (Nb,Cr) + Cr(Al,Nb) eutectic as the last solidification step with Al(Nb)Cr 2 precipitating from Cr(Al,Nb)

  2. Thermodynamic modeling of the Ti-Al-Cr ternary system

    International Nuclear Information System (INIS)

    Chen Leyi; Qiu Aitao; Liu Lanjie; Jiang Ming; Lu Xionggang; Li Chonghe

    2011-01-01

    Research highlights: → The full experimental results of the Ti-Al-Cr ternary system and its sub-binary systems are reviewed and analysed in detail. → Based on the latest thermodynamic assessments of the Ti-Al, Ti-Cr and Al-Cr systems and the ternary experimental data in literature, the thermodynamic parameters of the Ti-Al-Cr ternary system are fully assessed by the Calphad method. → The transformation of disorder to order (bcc a 2 to B2) and the new ternary compound L 12T i 25 Cr 8 Al 67 are considered in this work. - Abstract: The Ti-Al-Cr ternary system is one of the most important systems to studying the titanium alloys. Some experimental data of this ternary system are available and a few partial thermodynamic assessments are reported. However, no full thermodynamic descriptions were published. In this study, the previous work on the Ti-Al-Cr system and its related binary systems are reviewed. Based on the thermodynamic descriptions of the Ti-Al, Ti-Cr and Al-Cr systems and the ternary experimental data in literature, the Ti-Al-Cr ternary system is assessed by means of the Calphad method. Several isothermal sections from 1073 K to 1573 K and some invariant reactions are calculated, which are in good agreement with the most of the experimental results.

  3. Tetragonal phase in Al-rich region of U-Fe-Al system

    International Nuclear Information System (INIS)

    Meshi, L.; Zenou, V.; Ezersky, V.; Munitz, A.; Talianker, M.

    2005-01-01

    A new ternary aluminide U 2 FeAl 20 with the approximate composition Al-4.2at% Fe-8.5at% U was observed in the Al-rich corner of the U-Al-Fe system. Transmission electron microscopy and electron microdiffraction technique were used for characterization of the structure of this phase. It has a tetragonal unit cell with the parameters a=12.4A and c=10.3A and can be described by the space group I4-bar 2m

  4. Phase relationships in the Al-rich region of the Al-Cu-Er system

    International Nuclear Information System (INIS)

    Zhang Ligang; Masset, Patrick J.; Cao Fuyong; Meng Fangui; Liu Libin; Jin Zhanpeng

    2011-01-01

    Research highlights: → One ternary phase τ 1 -Al 8 Cu 4 Er in Al-rich region with a composition of 59.4-60.4 at.% Al, 32.2-33.8 at.% Cu, and 6.4-7.7 at.% Er is observed in both as-cast and annealed alloys. At 673 K, the binary Al 3 Er phase dissolves about 3.51 at.% Cu. → The calculated solidification paths (based on the CALPHAD method) of as-cast alloys are in agreement with the experimental results. → It can be found that the resultant thermodynamic database can be applied to case studies of as-cast alloys, showing that the literature thermodynamic description of the Al-Cu-Er system is reliable as a working basis for computer-assisted alloy design. - Abstract: The Al-rich region of the ternary Al-Cu-Er system is investigated using the method of X-ray diffraction, scanning electron microscopy with energy dispersive X-ray spectroscopy. Phase equilibria in the Al-rich region of the Al-Cu-Er system at 673 K have been obtained, and the microstructures of as-cast alloys in the Al-rich region are also investigated. One ternary phase τ 1 -Al 8 Cu 4 Er with a composition of 59.4-60.4 at.% Al, 32.2-33.8 at.% Cu, and 6.4-7.7 at.% Er is observed in both as-cast and annealed alloys. At 673 K, the binary Al 3 Er phase dissolves about 3.51 at.% Cu. The calculated solidification paths (based on the CALPHAD method) of as-cast alloys are in agreement with the experimental results.

  5. The constitution of alloys in the Al-rich corner of the Al-Si-Sm ternary system

    International Nuclear Information System (INIS)

    Markoli, B.; Spaic, S.; Zupanic, F.

    2001-01-01

    The constitution of alloys and the liquidus surface in the Al-rich corner of the Al-Si-Sm ternary system were determined by the examination of controlled heated and cooled specimens, as well as heat-treated specimens by means of optical and scanning electron microscopy, energy-dispersive X-ray spectroscopy, differential thermal analysis and X-ray diffraction. The Al-rich corner of the Al-Si-Sm ternary system comprises five regions of primary crystallisation (α Al , β Si , Al 3 Sm, Al 2 Si 2 Sm and AlSiSm) with following characteristic invariant reaction sequences: ternary eutectic reaction L → α Al + β Si + Al 2 Si 2 Sm, and two liquidus transition reactions, i. e., L + Al 3 Sm → α Al + AlSiSm, and L + AlSiSm → α Al + Al 2 Si 2 Sm. Along with the position of ternary eutectic and both interstitial points in the Al-rich corner of the Al-Si-Sm ternary system, the temperatures for each reaction were determined. (orig.)

  6. Dialysis-related amyloidosis of the hip joints in long-term hemodialysis patients. MRI findings of hip joints in twelve female hemodialysis patients

    International Nuclear Information System (INIS)

    Suzuki, Hitoe; Shibuya, Asuka; Ando, Minoru; Akiba, Takashi; Nitta, Kosaku

    2007-01-01

    We report a female with amyloid arthropathy of the hip joints. She was a 67-year-old woman who had been treated by hemodialysis for 22 years. She had demonstrated a 5-month history of continuous low-grade fever and pain in her left hip and she was finally unable to walk by herself. Findings on X-ray films and MRI of the hip joints suggested avascular necrosis in both femur heads. To palliate symptoms, bipolar surgery on the left hip joint was performed. Pathological examination of bone tissue specimen demonstrated that there was some , β 2 -microglobulin (β 2 -MG)-related amyloid accumulation in the femur head. Based on this clinical experience, we performed MRI screening for amyloid lesions of the hip joints in another 11 asymptomatic female patients undergoing hemodialysis for 20 years or more. Cystic lesions of the hip joints were observed in 8 patients, amyloid arthropathy in 2 patients, and fluid trapped in the joint in 1 patient. Patients with amyloidosis had significantly lower serum β 2 -MG levels than patients without amyloidosis (28.6 mg/L versus 41.4 mg/L; p=0.0339). Our findings show that dialysis-related amyloidosis of the hip joints is one of the potential and significant problems in female patients on long-term hemodialysis therapy. It may be important to screen for this pathological condition in long-term hemodialysis patients. (author)

  7. Case series

    African Journals Online (AJOL)

    abp

    19 oct. 2017 ... Traitement des amyloses AL systémiques: à propos de 25 cas. Treatment of systemic AL amyloidosis: about 25 cases. Hicham Eddou1,&, Ali Zinebi1, Hicham El Maaroufi2, Mohammed Karim Moudden1, Kamal Doghmi2, Mohammed Mikdame2,. Mohammed El Baaj1. 1Service de Médecine Interne, Hôpital ...

  8. Psychopathological dimensions in subjects with hereditary ATTR V30M amyloidosis and their relation with life events due to the disease.

    Science.gov (United States)

    Lopes, Alice; Fonseca, Isabel; Sousa, Alexandra; Rodrigues, Carla; Branco, Margarida; Coelho, Teresa; Sequeiros, Jorge; Freitas, Paula

    2018-03-01

    Chronic physical illness has been associated with emotional distress. Chronic diseases may change usual family patterns with economic, social and family losses. Hereditary ATTR V30M amyloidosis is a rare, fatal inherited systemic amyloidosis, with chronic evolution and beginning in adulthood. To evaluate psychopathological dimensions and how they correlated with disease-related life events, 209 symptomatic and asymptomatic carriers, participated in the study. Sociodemographic and Family and Personal History Disease questionnaires and brief symptom inventory (BSI) were applied. BSI indices, global severity index (GSI), positive symptom index (PSI) and positive symptom total (PST) scored higher than general population. Independent predictors for GSI >0.83 were female sex (OR = 3.46, p = .005) and being symptomatic carriers (OR = 3.03, p = .039). Independent predictors of a PST >26.99 were female sex (OR = 3.74, p = .012) symptomatic carrier (OR = 5.32, p = .025), age between 15 and 24 years at affected parent's death (OR = 5.26, p = .04). Independent predictors of a PSI >1.56 were being asymptomatic carrier (OR = 6.3, p = .036); to have children (OR = 3.19, p = .043) and have ≤14 years at parent's disease onset (OR = 6.39, p = .05). Results point to an important vulnerability of this population for psychological distress and psychiatric disease. Early life events related to disease, being sick and sex are associated with psychopathological distress.

  9. Formation of Al3Ti/Mg composite by powder metallurgy of Mg-Al-Ti system.

    Science.gov (United States)

    Yang, Zi R; Qi Wang, Shu; Cui, Xiang H; Zhao, Yu T; Gao, Ming J; Wei, Min X

    2008-07-01

    An in situ titanium trialuminide (Al 3 Ti)-particle-reinforced magnesium matrix composite has been successfully fabricated by the powder metallurgy of a Mg-Al-Ti system. The reaction processes and formation mechanism for synthesizing the composite were studied by differential scanning calorimetry (DSC), x-ray diffractometry (XRD), scanning electron microscopy (SEM) and energy-dispersive x-ray spectroscopy (EDS). Al 3 Ti particles are found to be synthesized in situ in the Mg alloy matrix. During the reaction sintering of the Mg-Al-Ti system, Al 3 Ti particles are formed through the reaction of liquid Al with as-dissolved Ti around the Ti particles. The formed intermetallic particles accumulate at the original sites of the Ti particles. As sintering time increases, the accumulated intermetallic particles disperse and reach a relatively homogeneous distribution in the matrix. It is found that the reaction process of the Mg-Al-Ti system is almost the same as that of the Al-Ti system. Mg also acts as a catalytic agent and a diluent in the reactions and shifts the reactions of Al and Ti to lower temperatures. An additional amount of Al is required for eliminating residual Ti and solid-solution strengthening of the Mg matrix.

  10. The constitution of alloys in the Al-rich corner of the Al-Si-Sm ternary system

    Energy Technology Data Exchange (ETDEWEB)

    Markoli, B.; Spaic, S. [Ljubljana Univ. (Slovenia). Faculty of Natural Science and Engineering; Zupanic, F. [Maribor Univ. (Slovenia). Faculty of Mechanical Engineering

    2001-09-01

    The constitution of alloys and the liquidus surface in the Al-rich corner of the Al-Si-Sm ternary system were determined by the examination of controlled heated and cooled specimens, as well as heat-treated specimens by means of optical and scanning electron microscopy, energy-dispersive X-ray spectroscopy, differential thermal analysis and X-ray diffraction. The Al-rich corner of the Al-Si-Sm ternary system comprises five regions of primary crystallisation ({alpha}{sub Al}, {beta}{sub Si}, Al{sub 3}Sm, Al{sub 2}Si{sub 2}Sm and AlSiSm) with following characteristic invariant reaction sequences: ternary eutectic reaction L {yields} {alpha}{sub Al} + {beta}{sub Si} + Al{sub 2}Si{sub 2}Sm, and two liquidus transition reactions, i. e., L + Al{sub 3}Sm {yields} {alpha}{sub Al} + AlSiSm, and L + AlSiSm {yields} {alpha}{sub Al} + Al{sub 2}Si{sub 2}Sm. Along with the position of ternary eutectic and both interstitial points in the Al-rich corner of the Al-Si-Sm ternary system, the temperatures for each reaction were determined. (orig.)

  11. Performance of the ALS injection system

    International Nuclear Information System (INIS)

    Kim, C.H.

    1993-05-01

    The authors started commissioning the Advanced Light Source (ALS) storage ring on January 11, 1993. The stored beam reached 60 mA on March 24, 1993 and 407 mA on April 9, 1993. The fast pace of storage ring commissioning can be attributed partially to the robust injection system. In this paper they describe the operating characteristics of the ALS injection system

  12. AL Amyloidosis and Agent Orange

    Science.gov (United States)

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  13. Low serum levels of prohepcidin, but not hepcidin-25, are related to anemia in familial amyloidosis TTR V30M.

    NARCIS (Netherlands)

    Beirao, I.; Almeida, S.; Swinkels, D.W.; Costa, P.M.; Moreira, L.; Fonseca, I.; Freitas, C.; Cabrita, A.; Porto, G.

    2008-01-01

    Familial amyloidosis TTR V30M (FAP-I) usually presents as a sensorimotor and autonomic neuropathy. Anemia was first described in this disease more than 20 years ago and classified as an anemia of chronic disease. However, so far no studies have addressed the role of inflammatory proteins in this

  14. Importance of pharmacokinetic studies in the management of acquired factor X deficiency.

    Science.gov (United States)

    Lim, Ming Y; McCarthy, Timothy; Chen, Sheh-Li; Rollins-Raval, Marian A; Ma, Alice D

    2016-01-01

    Up to 14% of individuals with systemic AL amyloidosis develop acquired factor X deficiency, which occurs due to adsorption of factor X onto amyloid fibrils. Although baseline factor X levels are not predictive of bleeding risk in these patients, serious hemorrhagic complications can occur, particularly during invasive procedures. Optimal management strategies to attenuate bleeding risk in these patients are unknown. We describe our experience in the management of acquired factor X deficiency, secondary to systemic AL amyloidosis, in a case series of three patients who received prothrombin complex concentrates (PCCs) for treatment and prevention of bleeding events. We performed a retrospective review extracting information on baseline demographics, laboratory data, pharmacokinetic (PK) studies, and clinically documented bleeding events. Our case series demonstrates that individuals with acquired factor X deficiency secondary to amyloidosis have variable laboratory and clinical responses to PCCs. This is likely due to distinct amyloid loads and fibril sequences, leading to different binding avidities for factor X. Our data emphasize the importance of performing PK testing prior to any invasive procedures to determine the dose and frequency interval to achieve adequate factor X levels for hemostasis, given the variable response between individuals. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  15. The Y-Cu-Al system

    International Nuclear Information System (INIS)

    Krachan, T.; Stel'makhovych, B.; Kuz'ma, Yu.

    2003-01-01

    The phase diagram of the Y-Cu-Al system at 820 K has been constructed using X-ray powder diffraction. The existence of earlier known ternary aluminides has been confirmed and their homogeneity regions and atomic distributions in the structures have been determined: YCu 4.6-4.0 Al 7.4-8.0 (ThMn 12 -type R I =0.049), Y 2 Cu 12.0-10.5 Al 5.0-6.5 (Th 2 Zn 17 -type R I =0.092), YCu 1.0-1.1 Al 1.0-0.9 (Fe 2 P-type R I =0.068). It has been shown that the structure of Y(Cu,Al) 3 is characterized by an ordered distribution of the Cu and Al atoms and it should be referred as Ca 3 Cu 2 Al 7 structure type (R I =0.060) besides the PuNi 3 structure type with statistical occupancies of the smaller atoms. At the investigated temperature the compound YCu 1.0-0.25 Al 3.0-3.75 (BaAl 4 -type) was not observed. However, we found the ternary aluminide with composition Y 3 Cu 2.7-2.0 Al 8.3-9.0 and related La 3 Al 11 -type (space group Immm, a=0.4192-0.4228, b=1.2423-1.2557, c=0.9812-0.9895 nm, R I =0.069). The compounds YCu 6.8 Al 4.2 (space group Fddd, Tb(Cu 0.58 Al 0.42 ) 11 -type, a=1.42755, b=1.48587, c=0.65654 nm, R I =0.062) and YCu 6.5 Al 4.5 (space group I4 1 /amd, BaCd 11 -type, a=1.02774, c=0.65838 nm, R I =0.071) have been found and structurally refined for the first time

  16. Value of tissue Doppler-derived Tei index and two-dimensional speckle tracking imaging derived longitudinal strain on predicting outcome of patients with light-chain cardiac amyloidosis.

    Science.gov (United States)

    Liu, Dan; Hu, Kai; Herrmann, Sebastian; Cikes, Maja; Ertl, Georg; Weidemann, Frank; Störk, Stefan; Nordbeck, Peter

    2017-06-01

    Prognosis of patients with light-chain cardiac amyloidosis (AL-CA) is poor. Speckle tracking imaging (STI) derived longitudinal deformation parameters and Doppler-derived left ventricular (LV) Tei index are valuable predictors of outcome in patients with AL-CA. We estimated the prognostic utility of Tei index and deformation parameters in 58 comprehensively phenotyped patients with AL-CA after a median follow-up of 365 days (quartiles 121, 365 days). The primary end point was all-cause mortality. 19 (33%) patients died during follow-up. Tei index (0.89 ± 0.29 vs. 0.61 ± 0.16, p < 0.001) and E to global early diastolic strain rate ratio (E/GLSR dias ) were higher while global longitudinal systolic strain (GLS sys ) was lower in non-survivors than in survivors (all p < 0.05). Tei index, NYHA functional class, GLS sys and E/GLSR dias were independent predictors of all-cause mortality risk, and Tei index ≥0.9 (HR 7.01, 95% CI 2.43-20.21, p < 0.001) was the best predictor of poor outcome. Combining Tei index and GLS sys yielded the best results on predicting death within 1 year (100% with Tei index ≥0.9 and GLS sys ≤13%) or survival (95% with Tei index ≤0.9 and GLS sys ≥13%). We conclude that 1-year mortality risk in AL-CA patients can be reliably predicted using Tei index or deformation parameters, with combined analysis offering best performance.

  17. The Successful Diagnosis and Typing of Systemic Amyloidosis Using A Microwave-Assisted Filter-Aided Fast Sample Preparation Method and LC/MS/MS Analysis.

    Directory of Open Access Journals (Sweden)

    Weiyi Sun

    Full Text Available Laser microdissection followed by mass spectrometry has been successfully used for amyloid typing. However, sample contamination can interfere with proteomic analysis, and overnight digestion limits the analytical throughput. Moreover, current quantitative analysis methods are based on the spectrum count, which ignores differences in protein length and may lead to misdiagnoses. Here, we developed a microwave-assisted filter-aided sample preparation (maFASP method that can efficiently remove contaminants with a 10-kDa cutoff ultrafiltration unit and can accelerate the digestion process with the assistance of a microwave. Additionally, two parameters (P- and D-scores based on the exponentially modified protein abundance index were developed to define the existence of amyloid deposits and those causative proteins with the greatest abundance. Using our protocol, twenty cases of systemic amyloidosis that were well-typed according to clinical diagnostic standards (training group and another twenty-four cases without subtype diagnoses (validation group were analyzed. Using this approach, sample preparation could be completed within four hours. We successfully subtyped 100% of the cases in the training group, and the diagnostic success rate in the validation group was 91.7%. This maFASP-aided proteomic protocol represents an efficient approach for amyloid diagnosis and subtyping, particularly for serum-contaminated samples.

  18. The Successful Diagnosis and Typing of Systemic Amyloidosis Using A Microwave-Assisted Filter-Aided Fast Sample Preparation Method and LC/MS/MS Analysis.

    Science.gov (United States)

    Sun, Weiyi; Sun, Jian; Zou, Lili; Shen, Kaini; Zhong, Dingrong; Zhou, Daobin; Sun, Wei; Li, Jian

    2015-01-01

    Laser microdissection followed by mass spectrometry has been successfully used for amyloid typing. However, sample contamination can interfere with proteomic analysis, and overnight digestion limits the analytical throughput. Moreover, current quantitative analysis methods are based on the spectrum count, which ignores differences in protein length and may lead to misdiagnoses. Here, we developed a microwave-assisted filter-aided sample preparation (maFASP) method that can efficiently remove contaminants with a 10-kDa cutoff ultrafiltration unit and can accelerate the digestion process with the assistance of a microwave. Additionally, two parameters (P- and D-scores) based on the exponentially modified protein abundance index were developed to define the existence of amyloid deposits and those causative proteins with the greatest abundance. Using our protocol, twenty cases of systemic amyloidosis that were well-typed according to clinical diagnostic standards (training group) and another twenty-four cases without subtype diagnoses (validation group) were analyzed. Using this approach, sample preparation could be completed within four hours. We successfully subtyped 100% of the cases in the training group, and the diagnostic success rate in the validation group was 91.7%. This maFASP-aided proteomic protocol represents an efficient approach for amyloid diagnosis and subtyping, particularly for serum-contaminated samples.

  19. Study of the ternary alloy systems Al-Ni-Fe and Al-Cu-Ru with special regard to quasicrystalline phases

    International Nuclear Information System (INIS)

    Lemmerz, U.

    1996-07-01

    Two ternary alloy-systems, the Al-Ni-Fe system and the Al-Cu-Ru system were studied with special regard to quasicrystalline phases. Isothermal sections were established in both systems in the stoichiometric area of the quasicrystalline phase. In the Al-Ni-Fe system a new stable decagonal phase was found. Its stoichiometric range is very small around Al 71.6 Ni 23.0 Fe 5.4 . The temperature range in which it is stable lies between 847 and 930 C. The decagonal phase undergoes a eutectoid reaction to the three crystalline phases Al 3 Ni 2 , Al 3 Ni and Al 13 Fe 4 at 847 C. It melts peritectically at 930 C forming Al 13 Fe 4 , Al 3 Ni 2 and a liquid. The investigations in the Al-Cu-Ru system concentrated on the phase equilibria between the icosahedral phase and its neighbouring phases in a temperature range between 600 and 1000 C. The icosahedral phase was observed in the whole temperature range. The investigated stoichiometric area extends down to Al contents of 45%, which allows the fields of existence to be determined for the ternary phases α-AlCuRu, the icosahedral phase and Al 7 Cu 2 Ru. Binary phases were determined down to the upper (high Al content) border of AlRu. No hitherto unknown phase was observed in the investigated area. Rietveld analyses were carried out on α-AlCuRu and Al 7 Cu 2 Ru showing some discrepancies from the α-AlMnSi structure taken as a base for α-AlCuRu and confirming the Al 7 Cu 2 Fe structure for Al 7 Cu 2 Ru. (orig.)

  20. 75 FR 65279 - Schedule for Rating Disabilities; AL Amyloidosis (Primary Amyloidosis)

    Science.gov (United States)

    2010-10-22

    ... not be diagnosed until it has resulted in considerable multi-organ damage (to heart, kidney, liver... surgically. Organ transplants (for example, of a kidney or the heart) have extended the lives of a small... result in the expenditure by State, local, and Tribal governments, in the aggregate, or by the private...

  1. Aluminium-rich corner in Al-Cu-La system

    International Nuclear Information System (INIS)

    Yunusov, I.; Ganiev, I.N.

    1990-01-01

    Aluminium corner of Al-Cu-La system are investigated by means of microstructural and differential thermal analysis. Existence of LaCu 2 Al 10 and LaCu 0.5 Al 3.5 ternary compounds in the system is confirmed and it is shown, as well, both compounds are in two-phase equilibrium with aluminium solid solution and form with it and between each other eutectic type state diagrams. State diagrams for quasibinary sections are plotted

  2. Isothermal cross-sections of Sr-Al-Ge and Ba-Al-Ge systems at 673 K

    International Nuclear Information System (INIS)

    Kutsenok, N.L.; Yanson, T.I.

    1987-01-01

    X-ray and microstructural analyses are used to study phase equilibria in Sr-Al-Ge and Ba-Al-Ge systems. Existence of SrAl 2 Ge 2 , Sr(Al, Ge) 2 Ba(Al, Ge) 2 , Sr 3 Al 2 Ge 2 , Ba 3 Al 2 Ge 2 ternary compounds is confirmed, a new BaGe 4 binary compound and also new ternary compounds of approximate composition Sr 57 Al 30 Ge 13 and Ba 20 Al 40 Ge 40 , which crystal structure is unknown, are detected. Aluminium solubility in SrAl 4 and BaAl 4 binary compounds (0.05 atomic fraction) is determined. Ba(Al, Ge) 2 compound homogeneity region is defined more exactly (aluminium content varies from 0.27 to 0.51 at. fractions)

  3. Phase equilibria of the Mo-Al-Ho ternary system

    Energy Technology Data Exchange (ETDEWEB)

    Li, Yitai; Chen, Xiaoxian; Liu, Hao [Guangxi Univ., Nanning (China). College of Materials Science and Engineering; Guangxi Univ., Nanning (China). Guangxi Key Laboratory of Processing for Non-ferrous Metal and Featured Materials; Zhan, Yongzhong [Guangxi Univ., Nanning (China). College of Materials Science and Engineering; Guangxi Univ., Nanning (China). Guangxi Key Laboratory of Processing for Non-ferrous Metal and Featured Materials; Guangxi Univ., Nanning (China). Center of Ecological Collaborative Innovation for Aluminum Industry

    2017-08-15

    Investigation into the reactions and phase equilibria of transition metal elements (i.e. Mo, Zr, Cr, V and Ti), Al and rare earths is academically and industrially important for the development of both refractory alloys and lightweight high-temperature materials. In this work, the equilibria of the Mo-Al-Ho ternary system at 773 K have been determined by using X-ray powder diffraction and scanning electron microscopy equipped with energy dispersive X-ray analysis. A new ternary phase Al{sub 4}Mo{sub 2}Ho has been found and the other ternary phase Al{sub 43}Mo{sub 4}Ho{sub 6} is observed. Ten binary phases in the Al-Mo and Al-Ho systems, including Al{sub 17}Mo{sub 4} rather than Al{sub 4}Mo, have been determined to exist at 773 K. The homogeneity ranges of AlMo{sub 3} and Al{sub 8}Mo{sub 3} phase are 7.5 at.% and 1 at.%, respectively. According to the phase-disappearing method, the maximum solubility of Al in Mo is about 16 at.%.

  4. Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

    Directory of Open Access Journals (Sweden)

    Yong Hwan Ahn

    2018-05-01

    Full Text Available Esophagogastroduodenoscopy for cancer screening was performed in a 55-year-old woman as part of a health screening program, and revealed a depressed lesion approximately 20 mm in diameter in the lesser curvature of the mid-gastric body. Several biopsy specimens were collected as the lesion resembled early gastric cancer; however, histopathologic evaluation revealed chronic active gastritis with an ulcer and amorphous eosinophilic material deposition. Congo red staining identified amyloid proteins, and apple-green birefringence was shown using polarized light microscopy. Immunohistochemical staining revealed the presence of kappa and lambda chain-positive plasma cells. There was no evidence of underlying plasma cell dyscrasia or amyloid deposition in other segments of the gastrointestinal tract. Echocardiography and computed tomography of the chest, abdomen, and pelvis did not show any significant findings. Thus, the patient was diagnosed with localized gastric amyloidosis with kappa and lambda light chain coexpression.

  5. Phase equilibria of the Al-Pr-Zr ternary system at 773 K

    International Nuclear Information System (INIS)

    She, Jia; Zhan, Yongzhong; Li, Chunliu; Du, Yong; Xu, Honghui; He, Yuehui

    2010-01-01

    The phase equilibria of the Al-Pr-Zr ternary system at 773 K have been investigated mainly by means of X-ray powder diffraction (XRD), scanning electron microscopy (SEM) and differential thermal analysis (DTA). The 14 binary compounds, i.e. AlZr 3 , AlZr 2 , Al 2 Zr 3 , Al 3 Zr 4 , AlZr, Al 3 Zr 2 , Al 2 Zr, Al 3 Zr, α-Al 11 Pr 3 , Al 3 Pr, Al 2 Pr, β-AlPr, AlPr 2 and β-AlPr 3 were confirmed. No binary compound was found in the Pr-Zr binary system. The result shows that the isothermal section of the Al-Pr-Zr ternary system at 773 K consists of 17 single-phase regions, 31 two-phase regions and 15 three-phase regions. All the intermediate compounds phases in this system have not a remarkable solid solution at 773 K. No ternary compound is found in this work.

  6. Phase diagrams of aluminium alloys of Al-Cu-Mg, Al-Mg-Si-Cu, and Al-Mg-Li system

    International Nuclear Information System (INIS)

    Ber, L.B.; Kaputkin, E.Ya.

    2001-01-01

    Isothermal diagrams of phase transformations (DPT) and temperature-time charts (TTC) of variation of electric conductivity and of mechanical features at tension were plotted following thermal treatment according to the pattern of direct hardening and ageing and according to the pattern of normal aging for D16 commercial alloy, Al-Cu-Mg model alloy of the same system, AD37 commercial alloys of Al-Mg-Si-Cu and 1424 one of Al-Li-Mg system. Phase transformations were studied by means of fluorescence electron microscopy, micro-X-ray spectral analysis, X-ray phase analysis of single crystals and polycrystals and differential scanning calorimetry. For every alloy comparison of TTC and DPT enables to clarity the mechanism of phase composition effect on features and to optimize conditions of hardening cooling and ageing [ru

  7. Experimental and thermodynamic investigation of Al-Cu-Nd ternary system

    Energy Technology Data Exchange (ETDEWEB)

    Bai, W.M. [School of Materials Science and Engineering, Central South University, Changsha, 410083 (China); Jiang, Y. [Hunan Sushi Guangbo Testing Techniques Co. LTD, Changsha (China); Guo, Z.Y.; Zeng, L.J.; Tan, M.Y. [School of Materials Science and Engineering, Central South University, Changsha, 410083 (China); Meggs, C. [School of Metallurgy and Materials, The University of Birmingham, Edgbaston, Birmingham, B15 2TT (United Kingdom); Zhang, L.G., E-mail: ligangzhang@csu.edu.cn [School of Materials Science and Engineering, Central South University, Changsha, 410083 (China); Liu, L.B., E-mail: pdc@csu.edu.cn [School of Materials Science and Engineering, Central South University, Changsha, 410083 (China); Jin, Z.P. [School of Materials Science and Engineering, Central South University, Changsha, 410083 (China)

    2017-07-01

    The phase relationships in the Al–Cu-Nd ternary system at 673 K have been investigated by X-ray powder diffraction (XRD) and scanning electron microscope (SEM) with energy disperse X-ray spectroscopy (EDS) in backscattered electron imaging (BSE) modes. The existence of six ternary Stoichiometric compounds, namely τ{sub 1}-Al{sub 8}Cu{sub 4}Nd, τ{sub 2}-Al{sub 9}Cu{sub 8}Nd{sub 2}, τ{sub 3}-Al{sub 6}Cu{sub 7}Nd, τ{sub 4}-Al{sub 2.4}Cu{sub 8.6}Nd, τ{sub 5}-Al{sub 3}CuNd, τ{sub 6}-AlCuNd, have been confirmed. A complete thermodynamic description of the Al–Cu-Nd ternary system coupled with the CALPHAD method is obtained based on experimental results and first-principles calculations. The calculated phase equilibria were in agreement with the available experimental data. - Highlights: • Phase relationships in the Al-Cu-Nd system has been systematically investigated. • 9 three-phase regions and 4 two-phase regions are confirmed. • A complete thermodynamic description of the Al-Cu-Nd system is obtained. • Results of first-principle calculation consist with thermodynamic calculation.

  8. Cellular processing of the amyloidogenic cystatin C variant of hereditary cerebral hemorrhage with amyloidosis, Icelandic type

    DEFF Research Database (Denmark)

    Benedikz, Eirikur; Merz, G S; Schwenk, V

    1999-01-01

    of an amyloidogenic mutation on the intracellular processing of its protein product. The protein, a mutant of the cysteine protease inhibitor cystatin C, is the amyloid precursor protein in Hereditary Cerebral Hemorrhage with Amyloidosis--Icelandic type (HCHWA-I). The amyloid fibers are composed of mutant cystatin C...... (L68Q) that lacks the first 10 amino acids. We have previously shown that processing of wild-type cystatin C entails formation of a transient intracellular dimer that dissociates prior to secretion, such that extracellular cystatin C is monomeric. We report here that the cystatin C mutation engenders...

  9. Isothermal sections at 500 deg C of the Dy-V-Al and Dy-Cr-Al systems in the aluminium rich regions

    International Nuclear Information System (INIS)

    Rykhal', R.M.; Zarechnyuk, O.S.; Mats'kiv, O.P.

    1979-01-01

    X-ray diffraction and microscopic analyses have been used to investigate the ternary system dysprosium-vanadium-aluminium in the aluminium rich region. In the system Dy-V-Al two ternary compounds have been found: DyV 2 Al 20 (cubic structure, CeCr 2 Al 20 type, a=14.54 A and approximately DyVAl 8 (hexagonal crystal system, structure unknown, a=10.86, c=17.71 A, c/a=1.631). In the system dysprosium-chromium-aluminium three ternary compounds have been found: DyCr 2 Al 20 (cubic structure, CeCr 2 Al 20 type, a=14.39), approximately equal to DyCrAl 8 ) hexagonal crystal system, structure type unkown a=10.75, c=17.60 A, c/a=1.637) and DyCr 4 Al 8 (tetragonal structure, CeMn 4 Al 8 type, a=8.87, c=5.04 A, c/a=0.568). Isothermal sections of the systems Dy-V-Al and Dy-Cr-Al have been plotted at 500 deg C

  10. Total Artificial Heart Implantation as a Bridge to Heart Transplantation in an Active Duty Service Member With Amyloid Cardiomyopathy.

    Science.gov (United States)

    Scully, Michael S; Wessman, Dylan E; McKee, James M; Francisco, Gregory M; Nayak, Keshav R; Kobashigawa, Jon A

    2017-03-01

    Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant. A 47-year-old active duty male initially evaluated for atypical chest pain was found to have severe concentric left ventricular hypertrophy on echocardiogram but normal voltage on electrocardiogram. Cardiac magnetic resonance imaging, laboratory studies, and bone marrow biopsy established the diagnosis of cardiac amyloidosis. At the time of diagnosis, the patient's prognosis was very poor with a median survival of 5 months on the basis of the Mayo Clinic revised prognostic staging system for amyloidosis. The patient developed rapidly progressive left ventricular dysfunction and heart failure leading to cardiac arrest. The patient received a total artificial heart as a bridge to orthotopic heart and kidney transplantation and eventual stem cell transplant. He continues to be in remission and has a fair functional capacity without restriction in activities of daily living or moderate exercise. Amyloid CM is a rare and devastating disease. The natural course of the disease has made heart transplant in these patients controversial. Modern advancements in chemotherapies and advanced heart failure treatments have improved outcomes for select patients with AL amyloid CM undergoing heart transplantation. There is ongoing research seeking improvement in treatment options and outcomes for patients with this deadly disease. Reprint & Copyright © 2017 Association of Military Surgeons of the U.S.

  11. Phase equilibria of the Al-Pr-Zr ternary system at 773 K

    Energy Technology Data Exchange (ETDEWEB)

    She, Jia [Laboratory of Nonferrous Metal Materials and New Processing Technology, Ministry of Education, Guangxi University, Nanning, Guangxi 530004 (China); Zhan, Yongzhong, E-mail: zyzmatres@yahoo.com.c [Laboratory of Nonferrous Metal Materials and New Processing Technology, Ministry of Education, Guangxi University, Nanning, Guangxi 530004 (China); Li, Chunliu [Laboratory of Nonferrous Metal Materials and New Processing Technology, Ministry of Education, Guangxi University, Nanning, Guangxi 530004 (China); Du, Yong; Xu, Honghui; He, Yuehui [State Key Laboratory of Powder Metallurgy, Central South University, Changsha, Hunan 410083 (China)

    2010-07-30

    The phase equilibria of the Al-Pr-Zr ternary system at 773 K have been investigated mainly by means of X-ray powder diffraction (XRD), scanning electron microscopy (SEM) and differential thermal analysis (DTA). The 14 binary compounds, i.e. AlZr{sub 3}, AlZr{sub 2}, Al{sub 2}Zr{sub 3}, Al{sub 3}Zr{sub 4}, AlZr, Al{sub 3}Zr{sub 2}, Al{sub 2}Zr, Al{sub 3}Zr, {alpha}-Al{sub 11}Pr{sub 3}, Al{sub 3}Pr, Al{sub 2}Pr, {beta}-AlPr, AlPr{sub 2} and {beta}-AlPr{sub 3} were confirmed. No binary compound was found in the Pr-Zr binary system. The result shows that the isothermal section of the Al-Pr-Zr ternary system at 773 K consists of 17 single-phase regions, 31 two-phase regions and 15 three-phase regions. All the intermediate compounds phases in this system have not a remarkable solid solution at 773 K. No ternary compound is found in this work.

  12. The Advanced Light Source (ALS) Radiation Safety System

    International Nuclear Information System (INIS)

    Ritchie, A.L.; Oldfather, D.E.; Lindner, A.F.

    1993-08-01

    The Advanced Light Source (ALS) at the Lawrence Berkeley Laboratory (LBL) is a 1.5 Gev synchrotron light source facility consisting of a 120 kev electron gun, 50 Mev linear accelerator, 1.5 Gev booster synchrotron, 200 meter circumference electron storage ring, and many photon beamline transport systems for research. Figure 1. ALS floor plan. Pairs of neutron and gamma radiation monitors are shown as dots numbered from 1 to 12. The Radiation Safety System for the ALS has been designed and built with a primary goal of providing protection against inadvertent personnel exposure to gamma and neutron radiation and, secondarily, to enhance the electrical safety of select magnet power supplies

  13. Interface phenomena in the Y2O3/(Al-Cu) system

    International Nuclear Information System (INIS)

    Barzilai, S.; Aizenshtein, M.; Froumin, N.; Frage, N.

    2006-01-01

    Wetting behavior and the interface reaction in the Y 2 O 3 /(Cu-Al) system were investigated at 1423 K. A contact angle of about 130 o was measured in the Y 2 O 3 /Cu system. Aluminum addition to copper improves wetting and the transition from non-wetting to wetting (θ ≤ 90 o ) was observed for the alloy with 50 at.% Al. The microstructure examination of the interface indicates that Al reacts with yttria, yttrium dissolves in the melt and a crater of AlYO 3 is formed at the substrate. The interface interaction in the Y 2 O 3 /(Cu-Al) system is in a good agreement with the results of a thermodynamic analysis in the Y-Al-Cu-O system. The crater depth and the macroscopic final contact angles are correlated with the Y and Al activities in the melt

  14. Frequency of and Prognostic Significance of Cardiac Involvement at Presentation in Hereditary Transthyretin-Derived Amyloidosis and the Value of N-Terminal Pro-B-Type Natriuretic Peptide

    NARCIS (Netherlands)

    Klaassen, Sebastiaan H C; Tromp, Jasper; Nienhuis, Hans L A; van der Meer, Peter; van den Berg, Maarten P; Blokzijl, Hans; van Veldhuisen, Dirk J; Hazenberg, Bouke P C

    2018-01-01

    The aim of this study is to assess the prevalence of cardiac involvement in hereditary transthyretin-derived (ATTRm) amyloidosis at the time of diagnosis and to determine the diagnostic and clinical value of N-terminal pro-B-type natriuretic peptide (NT-proBNP). The University Medical Center

  15. Al-Si/Al2O3 in situ composite prepared by displacement reaction of CuO/Al system

    Directory of Open Access Journals (Sweden)

    Zhang Jing

    2010-02-01

    Full Text Available Al2O3 particle-reinforced ZL109 composite was prepared by in situ reaction between CuO and Al. The microstructure was observed by means of OM, SEM and TEM. The Al2O3 particles in sub-micron sizes distribute uniformly in the matrix, and the Cu displaced from the in situ reaction forms net-like alloy phases with other alloy elements. The hardness and the tensile strength of the composites at room temperature have a slight increase as compared to that of the matrix. However, the tensile strength at 350 ℃ has reached 90.23 MPa, or 16.92 MPa higher than that of the matrix. The mechanism of the reaction in the CuO/Al system was studied by using of differential scanning calorimetry(DSC and thermodynamic calculation. The reaction between CuO and Al involves two steps. First, CuO reacts with Al to form Cu2O and Al2O3 at the melting temperature of the matrix alloy, and second, Cu2O reacts with Al to form Cu and Al2O3 at a higher temperature. At ZL109 casting temperature of 750–780 ℃, the second step can also take place because of the effect of exothermic reaction of the first step.

  16. Czech Registry of Monoclonal Gammopathies - Technical Solution, Data Collection and Visualisation.

    Science.gov (United States)

    Brozova, L; Schwarz, D; Snabl, I; Kalina, J; Pavlickova, B; Komenda, M; Jarkovský, J; Němec, P; Horinek, D; Stefanikova, Z; Pour, L; Hájek, R; Maisnar, V

    2017-01-01

    The Registry of Monoclonal Gammopathies (RMG) was established by the Czech Myeloma Group in 2007. RMG is a registry designed for the collection of clinical data concerning diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies. Data on patients with monoclonal gammopathy of undetermined significance (MGUS), Waldenström macroglobulinaemia (WM), multiple myeloma (MM) or primary AL ("amyloid light-chain") amyloidosis are collected in the registry. Nineteen Czech centres and four Slovak centres currently contribute to the registry. The registry currently contains records on more than 5,000 patients with MM, almost 3,000 patients with MGUS, 170 patients with WM and 26 patients with primary AL amyloidosis, i.e. more than 8,000 records on patients with monoclonal gammopathies altogether. This paper describes technology employed for the collection, storage and subsequent online visualisation of data. The CLADE-IS platform is introduced as a new system for the collection and storage of data from the registry. The form structure and functions of the new system are described for all diagnoses in general; these functions facilitate data entry to the registry and minimise the error rate in data. Publicly available online visualisations of data on patients with MGUS, WM, MM or primary AL amyloidosis from all Czech or Slovak centres are introduced, together with authenticated visualisations of data on patients with MM from selected centres. The RMG represents a data basis that makes it possible to monitor the disease course in patients with monoclonal gammopathies on the population level.Key words: Registry of Monoclonal Gammopathies - RMG - registries - monoclonal gammopathies - CLADE-IS - data visualisation - database.

  17. Phase equilibria of Al3(Ti,V,Zr) intermetallic system

    International Nuclear Information System (INIS)

    Park, S.I.; Han, S.Z.; Choi, S.K.; Lee, H.M.

    1996-01-01

    Trialuminides such as DO 22 -structured Al 3 Ti are promising candidates as potential materials for elevated temperature applications because of their attractive high temperature strength and excellent oxidation resistance along with their low density. However, in the tetragonal structure, slip systems are restricted due to low symmetry and the primary deformation mode is twinning. And, therefore, monolithic trialuminide compounds have been very impractical to be used as structural materials. When transition elements such as Ti, V and Zr which constitute trialuminides are alloyed in aluminum, they have low solubilities and low diffusion coefficients in the Al matrix. If precipitated as trialuminide intermetallics, they maintain a small lattice mismatch with the Al matrix, which reduces the interfacial energy between matrix and precipitates. As a result, these precipitates would have a large coarsening resistance in the matrix. As most of the previous works have been concentrated on the microstructural stability and mechanical properties, thermochemical properties will be treated in this work. In this study, phase equilibria and diagrams of Al 3 (Ti,V,Zr) systems will be experimentally determined and then thermodynamically analyzed with a hope to extend to the Al-Al 3 (Ti,V,Zr) composite system. This approach will then be used as a guide for alloy design of Al-Al 3 (Ti,V,Zr) composite system

  18. Directional solidification and characterization of the Al Nb2 - Al3 Nb eutectic system

    International Nuclear Information System (INIS)

    Trevisan, Eduardo A.O.; Andreotti, Fabio; Caram, Rubens

    1996-01-01

    The manufacturing of components to operate at high temperatures requires the use of metallic materials which can keep satisfactory mechanical and chemical properties, even at temperatures beyond 1000 deg C. An interesting alternative is the use of directionally solidified eutectic alloys. The eutectic alloy solidification makes possible the production of 'in situ' composite. A potentially useful system for manufacturing structural materials is the Al-Nb eutectic system. The aim of this work is to present the directional solidification of the Al-Nb eutectic alloy. (author)

  19. Phase equilibrium in Al-Li-Ca(Sr, Ba) systems at 423 K

    International Nuclear Information System (INIS)

    Ganieva, N.I.; Nazarov, Kh.M.; Ganiev, I.N.

    1999-01-01

    The isothermic cross section of the Al-Li-Ca(Sr, Ba) systems at 423 K is obtained. It is shown that the Al-Li-Sr ternary system as well as the Li-Sr binary system differ by nature of components in interaction from the Al-Li-Ca(Ba) systems. This is obvious by formation of the SrAl 2 Li 2 ternary intermetallide, which is not observed in the systems with participation of barium and calcium [ru

  20. Hot cracks formation nature in welds Al-Mg-Li and Al-Cu-Li alloy systems

    International Nuclear Information System (INIS)

    Ryazantsev, V.I.; Fedoseev, V.A.

    1997-01-01

    Mechanism of cleavage formation in alloy systems Al-Mg-Li and Al-Cu-Li welds at thermal test is proposed. This mechanism is connected with stitching spacing and stretching in direction of main deformation of intermetallic compounds inclusions and with active gases movement into the liquid phase [ru

  1. Amiloidose manifestando-se com pseudo-hipertrofia muscular Amyloidosis presenting as muscle pseudohypertrophy

    Directory of Open Access Journals (Sweden)

    Rodrigo Bortoli

    2007-12-01

    Full Text Available Amiloidose tipo AL é uma doença rara causada pela deposição extracelular de fragmentos de cadeias leves monoclonais em órgãos e tecidos. Pode apresentar-se com uma ampla variedade de sinais e sintomas, e o acometimento cutâneo-muscular, simulando pseudo-hipertrofia muscular, é um achado muito raro. São descritos dois casos que apresentaram tal manifestação. CASO 1 - Mulher, 61 anos, há quatro meses com história de mialgia e aumento da massa muscular nas cinturas pélvica, escapular e região cervical. Não havia alterações significativas ao exame físico, exceto aparente hipertrofia muscular difusa e discreta macroglossia. CASO 2 - Homem, 51 anos, há dois anos com cansaço e espessamento cutâneo progressivo do dorso, pescoço e braços. Em outros serviços levantou suspeitas diagnósticas de esclerodermia ou de escleredema de Buschke; desde fevereiro de 2007 passou a ser acompanhado nesse serviço e referia, havia cerca de um ano, disfagia para sólidos, disartria e dificuldade para movimentar a língua. Chamava atenção em seu exame o porte físico atlético com musculatura torácica proeminente, porém referia não fazer exercícios físicos. Em ambos os casos, a biópsia cutânea foi realizada com identificação do depósito amilóide por meio da coloração de vermelho congo.AL amyloidosis is a rare disease secondary to extracellular deposition of light chains fragments in organs and tissues. It can cause a wide variety of signs and symptoms, being the muscular pseudohypertrophy form a very rare finding. CASE 1 - a 61-year-old female had a history of myalgia and increase of muscular mass on pelvic and scapular girdle and cervical region. Besides the generalized muscular hypertrophy and discrete macroglossia, the rest of physical examination was normal. CASE 2 - a 51-year-old male complained of tiredness and progressive cutaneous thickening on his thorax, neck and arms for the last two years. Initially, he was misdiagnosed

  2. Experimental study and thermodynamic re-assessment of the Al-B system

    Energy Technology Data Exchange (ETDEWEB)

    Mirkovic, Djordje; Groebner, Joachim; Schmid-Fetzer, Rainer; Fabrichnaya, Olga; Lukas, Hans Leo

    2004-12-14

    Controversial thermodynamic data in previous assessments of the Al-B system were used to identify a key experiment regarding the enthalpy of formation of AlB{sub 2} and its peritectic decomposition temperature. Experiments were performed using AlB{sub 2} powder purified by vacuum sublimation. Differential scanning calorimetry (DSC) in a heat-flux twin cylindrical Calvet-type calorimetric system was utilized to measure the enthalpy of decomposition and the reaction temperatures. Samples were characterized by X-ray powder diffraction (XRD). Based on that, a comprehensive and consistent re-assessment of the Al-B system was performed. The incongruent melting of AlB{sub 2} occurs at 972 deg. C and a eutectoid decomposition of AlB{sub 2} at 213 deg. C is predicted from the thermodynamic description. The present enthalpy data are supported by realistic values of absolute entropies of solid Al-B phases. The formation of AlB{sub 2} was never observed on cooling, it is extremely sluggish. Upon heating AlB{sub 2}, the peritectic reaction does not produce perfectly crystalline AlB{sub 12}. Based on that and constraints in the thermodynamic data, it is derived that the stability range of AlB{sub 2} in equilibrium with perfect crystalline AlB{sub 12} may be even more narrow than 972-213 deg. C.

  3. Amiloidose renal em cão Shar-Pei: Relato de Caso Renal amyloidosis in a Shar-Pei dog: A case report

    Directory of Open Access Journals (Sweden)

    J.L. Reis Jr.

    2001-08-01

    Full Text Available O presente relato descreve os achados clínicos e anatomopatológicos de um caso de amiloidose renal em um cão macho de nove anos da raça Shar-Pei. O animal apresentava quadro clínico de esporotricose e de insuficiência renal e exames positivos para erlichiose e leishmaniose. No dia anterior ao óbito, o cão apresentou apatia, desidratação e anúria. À necropsia foram observados inúmeros pontos milimétricos esbranquiçados localizados no córtex renal e hepatização do lobo diafragmático esquerdo. O achado histológico mais importante foi deposição de material eosinofílico, amorfo e acelular localizado nos tufos glomerulares que se corou positivamente pelo vermelho congo (amilóide. Observaram-se nefrite supurada multifocal, espessamento da cápsula de Bowman e broncopneumonia supurada crônica, com fibrose intensa. A origem da amiloidose, no presente caso, poderia ser hereditária, assemelhando-se à amiloidose familiar descrita em cães da raça Shar-Pei, ou ser devida à inflamação supurada crônica e/ou leishmaniose.The clinical and pathological findings of a case of renal amyloidosis in a nine-year-old male Shar-Pei dog were described. Clinically, there were signs of sporotrichosis and renal insufficiency, besides being positive to leishmaniasis and ehrlichiosis. On the day before death, the animal became apathetic, dehydrated and anuric. On gross examination, there were several whitish millimetric spots seen widespread in both renal cortices and consolidation of the left diaphragmatic pulmonary lobe. The most important microscopic finding was a deposition of amorphous acellular material on the glomerular tufts which stained positively by congo red stain. Other changes were multifocal suppurative nephritis, thickening of the Bowman capsule and chronic suppurative bronchopneumonia. The origin of the amyloidosis in this case could be hereditary, being similar to familiar amyloidosis described in Shar-Pei breed, or due to

  4. The Advanced Light Source (ALS) Radiation Safety System

    International Nuclear Information System (INIS)

    Ritchie, A.; Oldfather, D.; Lindner, A.

    1993-05-01

    The Advanced Light Source (ALS) at the Lawrence Berkeley Laboratory (LBL) is a 1.5 GeV synchrotron light source facility consisting of a 120 keV electron gun, 50 MeV linear accelerator, 1.5 Gev booster synchrotron, 200 meter circumference electron storage ring, and many photon beamline transport systems for research. The Radiation Safety System for the ALS has been designed and built with a primary goal of providing protection against inadvertent personnel exposure to gamma and neutron radiation and, secondarily, to enhance the electrical safety of select magnet power supplies

  5. [A representative case of joint contracture as a main feature of AL amyloid deposits identified in the skeletal muscles].

    Science.gov (United States)

    Matsumura, Erika; Yamaguchi, Tetsuto; Tomidokoro, Yasushi; Ishii, Akiko; Tamaoka, Akira

    2014-01-01

    A 68-year-old man, with a history of type 2 diabetes mellitus and chronic kidney impairment, had been suffering from progressive knee joint contracture and dysesthesia of the lower extremities for 4 years. When he walked, his knees remained bent owing to contracture of the knee joints. There was no evidence of muscle pseudohypertrophy, intramuscular nodules, or muscle weakness. Clinical examination revealed IgA λ M-protein, reticular high-signal intensity lesions demonstrated by magnetic resonance T2-short TI IR(STIR) imaging of the lower extremity muscles, and a mixture of neurogenic and myogenic changes demonstrated by needle electromyography. A biopsy specimen from the vastus lateralis muscle identified Aλ amyloid deposits around the vessels, establishing a diagnosis of amyloid myopathy based on systemic AL amyloidosis. This case demonstrated that joint contracture and reticular lesions shown by magnetic resonance STIR imaging of the muscles can alert the physician to consider muscle biopsy to investigate deposition of amyloid in the skeletal muscles even in the absence of muscle pseudohypertrophy or weakness, both of which are characteristic of amyloid myopathy.

  6. Structure and phase composition of Al-Ce-Cu system alloys in range of quasi-binary Al-Al8CeCu4 section

    International Nuclear Information System (INIS)

    Belov, N.A.; Khvan, A.V.

    2007-01-01

    The phase diagram of the Al-Cu-Ce system in the quasibinary section area of Al-Al 8 CeCu 4 has been investigated by metallographic, thermal, micro-X-ray spectral and X-ray structural analyses. The parameters of the eutectic reaction L→(Al)+CeCu 4 Al 8 : T=610 Deg C were found out; the composition was 14% Cu and 7% Ce. This eutectics is of a disperse structure and the ternary compound contained is capable of fragmentation and spheroidizing in the heating process (starting from 540 Deg C). It was demonstrated that the area of optimal (Al)+CeCu 4 Al 8 eutectics-based alloy compositions was within the narrow limits. That is related to the fact that at a comparatively little variation of the Cu:Ce=2 ratio solidus sharply decreases and, as a result, the crystallization interval considerably extends [ru

  7. The ALS Gun Electronics system

    International Nuclear Information System (INIS)

    Lo, C.C.

    1993-05-01

    The ALS Gun Electronics system has been designed to accommodate gun with a custom made socket and high speed electronics circuit which is capable of producing single and multiple electron bunches with time jitters measured at better than 50 PS. The system generates the gated RF signal at ground level before sending it up to the 120 KV-biased gun deck via a fiber optic cable. The current pulse width as a function of grid bias, using an Eimac 8847A planar triode simulating an electron gun, was measured to show the relationship between the two parameters

  8. The ALS gun electronics system

    International Nuclear Information System (INIS)

    Lo, C.C.

    1993-01-01

    The ALS Gun Electronics system has been designed to accommodate the gun with a custom made socket and a high speed electronics circuit which is capable of producing single and multiple electron bunches with time jitters measured at better than 50 PS. The system generates the gated RF signal at ground level before sending it up to the 120 KV-biased gun deck via a fiber optic cable. The current pulse width as a function of grid bias, using an Eimac 8847A planar triode simulating an electron gun, was measured to show the relationship between the two parameters

  9. The Ag-Al-Cu system

    Energy Technology Data Exchange (ETDEWEB)

    Witusiewicz, V.T. [ACCESS e. V., Intzestr. 5, D-52072 Aachen (Germany)]. E-mail: victor@access.rwth-aachen.de; Hecht, U. [ACCESS e. V., Intzestr. 5, D-52072 Aachen (Germany); Fries, S.G. [ACCESS e. V., Intzestr. 5, D-52072 Aachen (Germany); Rex, S. [ACCESS e. V., Intzestr. 5, D-52072 Aachen (Germany)

    2005-01-25

    The thermodynamic description of the Ag-Al-Cu system is obtained by modelling the Gibbs energy of all individual phases in the system using the CALPHAD approach. The model parameters have been evaluated, by means of a computer optimisation technique, based on the descriptions of the constituent binaries proposed in the first part of the work and relevant experimental information for ternary alloys both from literature and own experimental measurements. Several vertical and isothermal sections, the liquidus surface and some thermodynamic properties are calculated using the evaluated parameters. A good agreement between the calculations and the experimental data is achieved.

  10. Rheumatic masks of plasma cell dyscrasias

    Directory of Open Access Journals (Sweden)

    Vladimir Ivanovich Vasilyev

    2012-01-01

    Full Text Available Objective: to consider clinical practice problems in the differential diagnosis of different types of plasma cell dyscrasias (PCD. Subjects and methods. Fourteen patients (8 men and 6 women aged 52±12 years, in whom rheumatic diseases (RD were ruled out and who were diagnosed as having primary PCD: different types of myeloma in 7 patients, myeloma + AL-amyloidosis in 2, AL-amyloidosis in 3, and Waldenstrom’s macroglobulinemia in 2, were examined. Results and discussion. The most common maldiagnosed RDs in patients with PCD were seronegative rheumatoid arthritis (RA, systemic lupus erythematosus, Sjogren’s disease, and different forms of vasculitis. The most frequent masks of RD were kidney (78% and osteoarticular system (64% lesions, vascular disorders (36%, peripheral polyneuropathies (36%, and enlarged salivary glands with xerostomia (28.5%. Serum and urine immunochemical study should be performed in all patients who have clinical manifestations of seropositive RA, spondyloarthritis, intensive bone pain syndrome, ulceronecrotic vasculitis, enlarged submandibular salivary glands with macroglossia in the absence of markers of autoimmune disease for the timely diagnosis of PCD and the exclusion of RD. The paper estimates the sensitivity and specificity of main methods used to diagnose different types of PCD.

  11. Glass forming ability of the Al-Ce-Ni system; Avaliacao da capacidade de formacao vitrea do sistema Al-Ce-Ni

    Energy Technology Data Exchange (ETDEWEB)

    Triveno Rios, C. [Engenharia Mecanica, Universidade Federal de Mato Grosso, Rondonopolis, MT (Brazil)], e-mail: triveno@ufmt.br; Surinach, S.; Baro, M.D. [Departamento de Engenharia de Materiais - Universidade Federal de Sao Carlos, SP (Brazil); Bolfarini, C.; Botta, W.J.; Kiminami, C.S. [Departamento de Fisica da Universidade Autonoma de Barcelona, Bellaterra (Spain)

    2010-07-01

    In the present work, the glass forming ability (GFA) and its compositional dependence on Al-Ni-Ce system alloys were investigated in function of several thermal parameters. Rapidly quenched Al{sub 85}Ni{sub 15}-{sub X}Ce{sub X} (X=4,5,6,7,10), Al{sub 90}Ni{sub 5}Ce{sub 5}, Al{sub 89}Ni{sub 2}.{sub 4}Ce{sub 8}.{sub 6}, Al{sub 80}Ni{sub 15.6}Ce{sub 4}.{sub 4} and Al{sub 78}Ni{sub 18.5}Ce{sub 3.5} amorphous ribbons were produced by melt-spinning and the structural transformation during heating was studied using a combination of X-ray diffraction (XRD) and differential scanning calorimetry (DSC). The results showed that the GFA and the thermal stability in the Al-rich corner of Al- Ni-Ce system alloys were enhanced by increasing the solute content and specifically the Ce content (author)

  12. Phase equilibria in Dy-Cu-Al system at 500 deg C

    International Nuclear Information System (INIS)

    Kuz'ma, Yu.B.; Milyan, V.V.

    1989-01-01

    Using the methods of X-ray diffraction analysis a diagram of phase equilibria in Dy-Cu-Al system at 500 deg C is plotted. Boundaries of solid solutions on the basis of DyCu 2 , DyCu and DyAl 2 compounds are determined and homogeneity regions of ternary compounds Dy 2 (Cu, Al) 7 and Dy(CuAl) 5 are ascertained. Compounds DyCuAl 3 , Dy 4 Cu 4 Al 11 and Dy 5 Cu 6 Al 9 have been detected for the first time

  13. Interfacial phenomena in the reactions of Al-B, Al-Ti-B, and Al-Zr-B alloys with KF-AlF3 and NaF-AlF3 melts

    International Nuclear Information System (INIS)

    Lee, M.S.; Terry, B.S.; Grieveson, P.

    1993-01-01

    The interfacial phenomena occurring during the contacting of liquid Al-B, Al-Ti-B, and Al-Zr-B melts with KF-AlF 3 liquid fluxes have been investigated by optical examination of quenched metal drops previously immersed in the liquid fluxes. Reactions in the Al-B/KF-AlF 3 system involve the formation of metastable AlB 12 at the metal/flux interface. At high KBF 4 levels in the flux, the AlB 12 is dispersed in the flux and also at low KBF 4 levels in the metal. Reactions in the Al-Ti-B/KF-AlF 3 system involve the formation of TiB 2 , which may be dispersed in either the metal or the flux depending upon the composition of the flux. The results obtained for the Al-Ti-B/NaF-AlF 3 and Al-Zr-B/KF-AlF 3 systems were similar to those observed for the Al-Ti-B/KF-AlF 3 system

  14. Thermodynamic modeling of the Al-U and Co-U systems

    International Nuclear Information System (INIS)

    Wang, J.; Liu, X.J.; Wang, C.P.

    2008-01-01

    The thermodynamic assessments of the Al-U and Co-U systems have been carried out by using the CALPHAD (Calculation of Phase Diagrams) method on the basis of the experimental data including thermodynamic properties and phase equilibria. Gibbs free energies of the solution phases were described by the subregular solution models with the Redlich-Kister equation, and those of the intermetallic compounds described by the sublattice models. A consistent set of thermodynamic parameters has been derived for describing the Gibbs free energies of each solution phase and intermetallic compounds in the Al-U and Co-U binary systems. The calculated phase diagrams and thermodynamic properties in the Al-U and Co-U systems are in good agreement with experimental data

  15. Phase equilibria and stability of the B2 phase in the Ni-Mn-Al and Co-Mn-Al systems

    International Nuclear Information System (INIS)

    Kainuma, R.; Ise, M.; Ishikawa, K.; Ohnuma, I.; Ishida, K.

    1998-01-01

    The phase equilibria and ordering reactions in the composition region up to 50 at.% Al have been investigated in the Ni-Mn-Al and Co-Mn-Al systems at temperatures in the interval 850-1200 C mainly by the diffusion couple method. The compositions of the γ (A1: fcc-Ni, -Co, γ-Mn), γ' (L1 2 : Ni 3 Al), β (B2: NiAl, CoAl, NiMn), β-Mn (A13: β-Mn type), δ-Mn (A2: bcc-Mn) and ε (A3: hcp-(Mn, Al)) phases in equilibrium and the critical boundaries of the A2/B2 continuous ordering transition in the bcc phase region have been determined. It is shown that in the Mn-rich portion of the ternary systems both continuous and discontinuous A2 to B2 ordering transitions exist. The A2+B2 two-phase region in the isothermal sections has a lenticular shape and exists over a wide temperature range. The phase equilibria between the γ, γ', β, β-Mn, δ-Mn and ε phases are presented and the stability of the ordered bcc aluminides is discussed. (orig.)

  16. α-NiPt(Al) and phase equilibria in the Ni-Al-Pt system at 1150 deg C

    International Nuclear Information System (INIS)

    Hayashi, S.; Ford, S.I.; Young, D.J.; Sordelet, D.J.; Besser, M.F.; Gleeson, B.

    2005-01-01

    The α-NiPt(Al) phase and its associated equilibria in the Ni-Al-Pt system at 1150 deg C were investigated by analyzing equilibrated bulk alloys and the interdiffusion zones of diffusion couples. Phase constitutions, tie-lines and microstructures were determined using a combination of techniques, including high-energy synchrotron X-ray diffraction, scanning electron microscopy and electron probe microanalysis. A large Pt solubility limit was found to exist in the β-NiAl, ∼42 at.%, and in γ'-Ni 3 Al, ∼32 at.%. The α-NiPt(Al) phase was found to have wide Pt solubility range of about 33-60 at.% and to skew along an almost constant Pt/Al ratio of 1.5. The α-NiPt(Al) has an ordered face-centered tetragonal L1 0 crystal structure, with the Al and Pt atoms found to be preferentially located in the corners and prismatic faces, respectively. The temperature dependence of the lattice parameters and unit cell volume of the α phase were also determined

  17. Amyloidosis, synucleinopathy, and prion encephalopathy in a neuropathic lysosomal storage disease: the CNS-biomarker potential of peripheral blood.

    Directory of Open Access Journals (Sweden)

    Bartholomew J Naughton

    Full Text Available Mucopolysaccharidosis (MPS IIIB is a devastating neuropathic lysosomal storage disease with complex pathology. This study identifies molecular signatures in peripheral blood that may be relevant to MPS IIIB pathogenesis using a mouse model. Genome-wide gene expression microarrays on pooled RNAs showed dysregulation of 2,802 transcripts in blood from MPS IIIB mice, reflecting pathological complexity of MPS IIIB, encompassing virtually all previously reported and as yet unexplored disease aspects. Importantly, many of the dysregulated genes are reported to be tissue-specific. Further analyses of multiple genes linked to major pathways of neurodegeneration demonstrated a strong brain-blood correlation in amyloidosis and synucleinopathy in MPS IIIB. We also detected prion protein (Prnp deposition in the CNS and Prnp dysregulation in the blood in MPS IIIB mice, suggesting the involvement of Prnp aggregation in neuropathology. Systemic delivery of trans-BBB-neurotropic rAAV9-hNAGLU vector mediated not only efficient restoration of functional α-N-acetylglucosaminidase and clearance of lysosomal storage pathology in the central nervous system (CNS and periphery, but also the correction of impaired neurodegenerative molecular pathways in the brain and blood. Our data suggest that molecular changes in blood may reflect pathological status in the CNS and provide a useful tool for identifying potential CNS-specific biomarkers for MPS IIIB and possibly other neurological diseases.

  18. Advanced launch system (ALS) - Electrical actuation and power systems improve operability and cost picture

    Science.gov (United States)

    Sundberg, Gale R.

    1990-01-01

    To obtain the Advanced Launch System (ALS) primary goals of reduced costs and improved operability, there must be significant reductions in the launch operations and servicing requirements relative to current vehicle designs and practices. One of the primary methods for achieving these goals is by using vehicle electrrical power system and controls for all aviation and avionics requirements. A brief status review of the ALS and its associated Advanced Development Program is presented to demonstrate maturation of those technologies that will help meet the overall operability and cost goals. The electric power and actuation systems are highlighted as a sdpecific technology ready not only to meet the stringent ALS goals (cryogenic field valves and thrust vector controls with peak power demands to 75 hp), but also those of other launch vehicles, military ans civilian aircraft, lunar/Martian vehicles, and a multitude of comercial applications.

  19. Advanced Launch System (ALS): Electrical actuation and power systems improve operability and cost picture

    Science.gov (United States)

    Sundberg, Gale R.

    1990-01-01

    To obtain the Advanced Launch System (ALS) primary goals of reduced costs and improved operability, there must be significant reductions in the launch operations and servicing requirements relative to current vehicle designs and practices. One of the primary methods for achieving these goals is by using vehicle electrical power system and controls for all actuation and avionics requirements. A brief status review of the ALS and its associated Advanced Development Program is presented to demonstrate maturation of those technologies that will help meet the overall operability and cost goals. The electric power and actuation systems are highlighted as a specific technology ready not only to meet the stringent ALS goals (cryogenic field valves and thrust vector controls with peak power demands to 75 hp), but also those of other launch vehicles, military and civilian aircraft, lunar/Martian vehicles, and a multitude of commercial applications.

  20. Hydrogen migration dynamics in hydrated Al clusters: The Al17(−)·H2O system as an example

    International Nuclear Information System (INIS)

    Álvarez-Barcia, S.; Flores, J. R.

    2014-01-01

    The Al m (−) ·(H 2 O) n systems are known to undergo water splitting processes in the gas phase giving H k Al m (OH) k (−) ·(H 2 O) n−k systems, which can generate H 2 . The migration of H atoms from one Al atom to another on the cluster's surface is of critical importance to the mechanism of the complete H 2 production process. We have applied a combination of Molecular Dynamics and Rice-Ramsperger-Kassel-Marcus theory including tunneling effects to study the gas-phase evolution of HAl 17 (OH) (−) , which can be considered a model system. First, we have performed an extensive search for local minima and the connecting saddle points using a density functional theory method. It is found that in the water-splitting process Al 17 (−) ·(H 2 O) → HAl 17 (OH) (−) , the H atom which bonds to the Al cluster losses rather quickly its excess energy, which is easily “absorbed” by the cluster because of its flexibility. This fact ultimately determines that long-range hydrogen migration is not a very fast process and that, probably, tunneling only plays a secondary role in the migration dynamics, at least for moderate energies. Reduction of the total energy results in the process being very much slowed down. The consequences on the possible mechanisms of H 2 generation from the interaction of Al clusters and water molecules are discussed

  1. AL amyloid imaging and therapy with a monoclonal antibody to a cryptic epitope on amyloid fibrils.

    Directory of Open Access Journals (Sweden)

    Jonathan S Wall

    Full Text Available The monoclonal antibody 2A4 binds an epitope derived from a cleavage site of serum amyloid protein A (sAA containing a -Glu-Asp- amino acid pairing. In addition to its reactivity with sAA amyloid deposits, the antibody was also found to bind amyloid fibrils composed of immunoglobulin light chains. The antibody binds to synthetic fibrils and human light chain (AL amyloid extracts with high affinity even in the presence of soluble light chain proteins. Immunohistochemistry with biotinylated 2A4 demonstrated positive reaction with ALκ and ALλ human amyloid deposits in various organs. Surface plasmon resonance analyses using synthetic AL fibrils as a substrate revealed that 2A4 bound with a K(D of ∼10 nM. Binding was inhibited in the presence of the -Glu-Asp- containing immunogen peptide. Radiolabeled 2A4 specifically localized with human AL amyloid extracts implanted in mice (amyloidomas as evidenced by single photon emission (SPECT imaging. Furthermore, co-localization of the radiolabeled mAb with amyloid was shown in biodistribution and micro-autoradiography studies. Treatment with 2A4 expedited regression of ALκ amyloidomas in mice, likely mediated by the action of macrophages and neutrophils, relative to animals that received a control antibody. These data indicate that the 2A4 mAb might be of interest for potential imaging and immunotherapy in patients with AL amyloidosis.

  2. Activated microglia mediate synapse loss and short-term memory deficits in a mouse model of transthyretin-related oculoleptomeningeal amyloidosis.

    Science.gov (United States)

    Azevedo, E P; Ledo, J H; Barbosa, G; Sobrinho, M; Diniz, L; Fonseca, A C C; Gomes, F; Romão, L; Lima, F R S; Palhano, F L; Ferreira, S T; Foguel, D

    2013-09-05

    Oculoleptomeningeal amyloidosis (OA) is a fatal and untreatable hereditary disease characterized by the accumulation of transthyretin (TTR) amyloid within the central nervous system. The mechanisms underlying the pathogenesis of OA, and in particular how amyloid triggers neuronal damage, are still unknown. Here, we show that amyloid fibrils formed by a mutant form of TTR, A25T, activate microglia, leading to the secretion of tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and nitric oxide. Further, we found that A25T amyloid fibrils induce the activation of Akt, culminating in the translocation of NFκB to the nucleus of microglia. While A25T fibrils were not directly toxic to neurons, the exposure of neuronal cultures to media conditioned by fibril-activated microglia caused synapse loss that culminated in extensive neuronal death via apoptosis. Finally, intracerebroventricular (i.c.v.) injection of A25T fibrils caused microgliosis, increased brain TNF-α and IL-6 levels and cognitive deficits in mice, which could be prevented by minocycline treatment. These results indicate that A25T fibrils act as pro-inflammatory agents in OA, activating microglia and causing neuronal damage.

  3. The `principle of invariance' in currency systems: a comment on Caianiello et al.

    Science.gov (United States)

    Bouhdaoui, Y.; Bounie, D.; Van Hove, L.

    2012-04-01

    In an often-cited article in this journal, Caianiello et al. (1982. International Journal of General Systems, 8 (2), 81-92) formulate a 'principle of invariance' for currency systems. They build on this principle to explain the distribution of the coins and banknotes in circulation over the different denominations, and analyse how a currency system adjusts to inflation. This paper shows that Caianiello et al.'s distribution law is flawed because the principle of invariance is false.

  4. CuGaTe2-CuAlTe2 system

    International Nuclear Information System (INIS)

    Bodnar', I.V.

    2003-01-01

    The results of studies on the chemical interaction in the CuGaTe 2 -CuAlTe 2 as well as on the thermal and optical properties of the formed solid solutions are presented. It is shown, that continuous number of solid solutions are formed in the CuGaTe 2 -CuAlTe 2 system, which crystallize in the chalcopyrite structure. The diagram of state of this system is plotted. The thermal expansion of these materials is studied through the dilatometric method. The linear dependence of the thermal expansion coefficient on the composition is established. The concentration dependences of the forbidden zone width diverge from the linearity [ru

  5. The Al-rich region of the Al-Mn-Ni alloy system. Part II. Phase equilibria at 620-1000 oC

    International Nuclear Information System (INIS)

    Balanetskyy, S.; Meisterernst, G.; Grushko, B.; Feuerbacher, M.

    2011-01-01

    Research highlights: → Phase equilibria in the Al-rich region of the Al-Mn-Ni alloy system were studied at 1000, 950, 850, 750, 700, 645 and 620 deg. C by means of SEM, TEM, powder XRD and DTA. → Three ternary thermodynamically stable intermetallics, the φ-phase (Al 5 Co 2 -type, hP26, P63/mmc; a = 0.76632(16), c = 0.78296(15) nm), the κ-phase (κ-Al 14.4 Cr 3.4 Ni l.1 -type, hP227, P63/m; a = 1.7625(10), c = 1.2516(10) nm), and the O-phase (O-Al 77 Cr 14 Pd 9 -type, Pmmn, oP650,: a = 2.3316(16), b = 1.2424(15), c = 3.2648(14) nm), as well as three ternary metastable phases, the decagonal D 3 -phase with periodicity about 1.25 nm, the Al 9 (Mn,Ni) 2 -phase (Al 9 Co 2 -type, P1121/a, mP22; a = 0.8585(16), b = 0.6269(9), c = 0.6205(11) nm, β = 95.34(10) o ) and the O 1 -phase (basecentered orthorhombic, a ∼ 23.8, b ∼ 12.4, c ∼ 32.2 nm) were revealed. → The existence of a thermodynamically stable R-phase of stoichiometry Al 60 Mn 11 Ni 4 , reported earlier in literature, was not confirmed in the present study. - Abstract: Phase equilibria in the Al-rich region of the Al-Mn-Ni alloy system were studied at 1000, 950, 850, 750, 700, 645 and 620 o C. Three ternary thermodynamically stable intermetallics, the φ-phase (Al 5 Co 2 -type, hP26, P6 3 /mmc; a = 0.76632(16), c = 0.78296(15) nm), the κ-phase (κ-Al 14.4 Cr 3.4 Ni l.1 -type, hP227, P6 3 /m; a = 1.7625(10), c = 1.2516(10) nm), and the O-phase (O-Al 77 Cr 14 Pd 9 -type, Pmmn, oP650,: a = 2.3316(16), b = 1.2424(15), c = 3.2648(14) nm), as well as three ternary metastable phases, the decagonal D 3 -phase with periodicity about 1.25 nm, the Al 9 (Mn,Ni) 2 -phase (Al 9 Co 2 -type, P112 1 /a, mP22; a = 0.8585(16), b = 0.6269(9), c = 0.6205(11) nm, β = 95.34(10) o ) and the O 1 -phase (base-centered orthorhombic, a ∼ 23.8, b ∼ 12.4, c ∼ 32.2 nm) were revealed. Their physicochemical behaviour in the Al-Mn-Ni alloy system was studied.

  6. Influence of material and testing parameters on the lifetime of TBC systems with MCrAlY and NiPtAl bondcoats

    Energy Technology Data Exchange (ETDEWEB)

    Song, Peng

    2012-08-31

    The oxidation behavior of the bond coat is an important factor determining the lifetime of thermal barrier coatings (TBC) in the advanced gas turbine components. In the present work, the effect of various testing parameters, such as hot/cold dwell time, heating/cooling rate, atmosphere composition on the bondcoat oxidation and associated TBC lifetime has been investigated. The range of coating systems included Electron Beam - Physical Vapor Deposited (EB-PVD) and Air Plasma Sprayed (APS) TBC's with MCrAlY (M = Ni, Co) and NiPtAl-bondcoats of various compositions. The effect of the testing parameters strongly depended on the type and properties of the studied system. The lifetime of EB-PVD TBC systems with conventional MCrAlY and NiPtAl bondcoats forming uniform, flat alumina scales was found to be limited by critical scale thickness, upon which a rapid crack propagation at the scale/bondcoat interface results in macroscopic failure. The lifetime of such systems was found to be affected by factors, which influence the scale growth rate and adherence (in particular by oxygen partial pressure (pO{sub 2}) and water vapor content in the test gas in the case of MCrAlY), whereas the temperature cyclic frequency showed no significant effect. NiPtAl bondcoats showed a superior behavior than the conventional MCrAlY-bondcoats due to slower scale growth rate and better scale adherence. For EB-PVD TBC systems with Zr-doped MCrAlYbondcoats the lifetime is mainly determined by the crack growth rate in the inhomogeneous inwardly growing oxide scales, whereas the lifetime is not dependent on the pO{sub 2} but rather on the cyclic frequency. For APS TBC systems the bondcoat oxidation is only one of several factors determining the ceramic topcoat lifetime. Therefore the oxide scale adherence is of less importance for lifetime of APS TBCs as compared to EBPVD TBCs. For the former systems, the cracks initiated at the convex asperities of the rough oxide scale / bondcoat interface

  7. Amiloidose e insuficiência renal crônica terminal associada à hanseníase Amyloidosis and end-stage renal disease associated with leprosy

    Directory of Open Access Journals (Sweden)

    Geraldo Bezerra da Silva Júnior

    2010-08-01

    Full Text Available O envolvimento renal na hanseníase é diverso, incluindo glomerulonefrites, amiloidose e nefrite túbulo-intersticial. Um homem de 58 anos foi admitido com edema de membros inferiores e dispnéia. Na admissão, havia retenção de escórias nitrogenadas, anemia, hipercalemia e acidose metabólica, com necessidade de hemodiálise. Referia história de hanseníase virchoviana. Foi realizada biopsia renal, compatível com amiloidose. O paciente evoluiu estável, sem recuperação da função renal, permanecendo em tratamento hemodialítico. A hanseníase deve ser investigada em todo paciente com perda de função renal, sobretudo naqueles que apresentam lesões cutâneas ou outras manifestações sugestivas de hanseníase.Renal involvement in leprosy includes glomerulonephritis, amyloidosis and tubulointerstitial nephritis. A 58-year-old man was admitted with complaints of lower limb edema and dyspnea. At admission, nitrogen retention, anemia, hyperkalemia and metabolic acidosis were observed, requiring hemodialysis. The patient had a history of lepromatous leprosy. A renal biopsy was performed that was compatible with amyloidosis. The patient had a stable outcome, but without renal function recovery and remained on regular hemodialysis. Leprosy should be investigated in every patient with renal function loss, particularly in those with cutaneous lesions or other manifestations suggestive of leprosy.

  8. Experimental investigation and thermodynamic modeling of the Ni-Al-Ru ternary system

    International Nuclear Information System (INIS)

    Zhu, J.; Zhang, C.; Cao, W.; Yang, Y.; Zhang, F.; Chen, S.; Morgan, D.; Chang, Y.A.

    2009-01-01

    In this study, a thermodynamic description of the Ni-Al-Ru system was obtained in terms of experimental phase equilibrium data as well as first-principles-calculated energetics. The calculated isotherms and liquidus projection using this description are in accord with the experimental data obtained in the present study as well as those previously reported in the literature, and the model-calculated enthalpies of formation agree with subsequent first-principles-calculated values. During the thermodynamic modeling of the Ni-Al-Ru system, we encountered great challenges initially to account for one of the experimentally determined isotherms reported in the literature. To reconcile these discrepancies, additional experiments were carried out and the results obtained supported the phase equilibrium data reported in the literature. The model-calculated enthalpies agree with subsequent first-principles-calculated values. The approach adopted in this study using phase equilibrium data of Ni-Al-Ru and the descriptions of Ni-Al and Ni-Ru to identify errors in the enthalpy of AlRu could be a general tool that could be used for other systems

  9. Phase relations in the SiC-Al2O3-Pr2O3 system

    International Nuclear Information System (INIS)

    Pan, W.; Wu, L.; Jiang, Y.; Huang, Z.

    2016-01-01

    Phase relations in the Si-Al-Pr-O-C system, including the SiC-Al 2 O 3 -Pr 2 O 3 , the Al 2 O 3 -Pr 2 O 3 -SiO 2 and the SiC-Al 2 O 3 -Pr 2 O 3 -SiO 2 subsystems, were determined by means of XRD phase analysis of solid-state-reacted samples fabricated by using SiC, Al 2 O 3 , Pr 2 O 3 and SiO 2 powders as the starting materials. Subsolidus phase diagrams of the systems were presented. Two Pr-aluminates, namely PrAlO 3 (PrAP) and PrAl 11 O 18 (β(Pr) β-Al 2 O 3 type) were formed in the SiC-Al 2 O 3 -Pr 2 O 3 system. SiC was compatible with both of them. Pr-silicates of Pr 2 SiO 5 , Pr 2 Si 2 O 7 and Pr 9.33 Si 6 O 26 (H(Pr) apatite type) were formed owing to presence of SiO 2 impurity in the SiC powder. The presence of the SiO 2 extended the ternary system of SiC-Al 2 O 3 -Pr 2 O 3 into a quaternary system of SiC-Al 2 O 3 -SiO 2 -Pr 2 O 3 (Si-Al-Pr-O-C). SiC was compatible with Al 2 O 3 , Pr 2 O 3 and the Pr-silicates. The effect of SiO 2 on the phase relations and liquid phase sintering of SiC ceramics was discussed.

  10. A study of the Al-rich part of the Al-Re alloy system

    International Nuclear Information System (INIS)

    Balanetskyy, S.; Grushko, B.

    2008-01-01

    The Al-rich part of the Al-Re phase diagram was reinvestigated by scanning and transmission electron microscopy, powder X-ray diffraction and differential thermal analysis. The earlier reported Al 12 Re, Al 6 Re, and Al 11 Re 4 phases were confirmed. Close to the Al 4 Re composition a high-temperature h-Al 4 Re and a low-temperature l-Al 4 Re phase were found to be formed at slightly different compositions. Both exhibited compositional ranges. A new phase was revealed close to the Al 3 Re composition. The Al 11 Re 4 phase is formed congruently at 1650 deg. C, Al 3 Re peritectically at 1610 deg. C, h-Al 4 Re peritectically at 1410 deg. C, l-Al 4 Re peritectically at ∼1000 deg. C, Al 6 Re peritectically at 813 deg. C and Al 12 Re peritectically at 746 deg. C. Finally, the Al 12 Re-(Al) eutectic is formed at 658 deg. C. The Al 3 Re and h-Al 4 Re are decomposed by eutectoid reactions at ∼946 and 833 deg., respectively. The structure of the h-Al 4 Re phase has not yet been clarified, but it is distinctly different from the Al 4 W phase suggested earlier. The structure of l-Al 4 Re was confirmed. The Al 3 Re phase was found to have a monoclinic structure with a = 0.5180(4) nm, b = 3.97641(9) nm, c = 0.4955(3) nm and β = 99.55(7) o

  11. Insulin deficiency exacerbates cerebral amyloidosis and behavioral deficits in an Alzheimer transgenic mouse model

    Directory of Open Access Journals (Sweden)

    Teng Wei-Ping

    2010-11-01

    Full Text Available Abstract Background Although increasing evidence has indicated that brain insulin dysfunction is a risk factor for Alzheimer disease (AD, the underlying mechanisms by which insulin deficiency may impact the development of AD are still obscure. Using a streptozotocin (STZ-induced insulin deficient diabetic AD transgenic mouse model, we evaluated the effect of insulin deficiency on AD-like behavior and neuropathology. Results Our data showed that administration of STZ increased the level of blood glucose and reduced the level of serum insulin, and further decreased the phosphorylation levels of insulin receptors, and increased the activities of glycogen synthase kinase-3α/β and c-Jun N-terminal kinase in the APP/PS1 mouse brain. We further showed that STZ treatment promoted the processing of amyloid-β (Aβ precursor protein resulting in increased Aβ generation, neuritic plaque formation, and spatial memory deficits in transgenic mice. Conclusions Our present data indicate that there is a close link between insulin deficient diabetes and cerebral amyloidosis in the pathogenesis of AD.

  12. Calculation of solidification microstructure maps for the system Al-Fe-Si

    International Nuclear Information System (INIS)

    Gilgien, P.

    1996-01-01

    Computer programs have been developed in order to calculate solidification microstructure maps for binary and ternary alloys. These programs are based on recent analytical models for the constrained growth of dendrites and eutectics. Due to the importance of phase diagrams data, programs for the calculation of growth kinetics are coupled with ThermoCalc, a commercial software for phase diagram calculations. These programs have been used to calculate a solidification microstructure map for the Al-Fe system from 0 to 4 at%Fe. Comparison of the calculated results with an experimental solidification microstructure map from the literature shows that all microstructure transitions were predicted. Nevertheless there remain significant discrepancies between some calculated and experimental transition velocities. The programs were also used to calculate solidification microstructure maps in the Al-rich corner of the Al-Fe-Si system (0 to 8 at% Fe and 0 to 8 at% Si). In this case also, calculated results were in satisfactory agreement with experimental solidification microstructure maps, although the comparison was only partial since experimental ternary microstructure maps are less complete than for the binary system, and because the available thermodynamic database does not, as yet, include metastable phases. Laser surface remelting experiments were carried out on an Al-4 at% Fe alloy in order to link results from the literature, obtained at high solidification rates by laser surface remelting and at low solidification rates by Bridman experiments. Finally, Bridman experiments were carried out with an Al-2.63 wt% Fe alloy in order to determine the critical velocity at which a planar Al-Al 13 Fe 4 eutectic front is destabilised in a cellular eutectic by a small amount of Si. The critical solidification velocity thus obtained was in agreement with a criterion of constitutional undercooling. (author) figs., tabs., refs

  13. Investigation of the 600 C isothermal section of the Fe-Al-Ce ternary system

    Energy Technology Data Exchange (ETDEWEB)

    Zheng, Huiyun; Yin, Fucheng [Xiangtan Univ., Hunan (China). School of Materials Science and Engineering; Xiangtan Univ., Hunan (China). Key Laboratory of Materials Design and Preparation Technology of Hunan Province; Li, Zhi [Xiangtan Univ., Hunan (China). School of Materials Science and Engineering; Xiangtan Univ., Hunan (China). Key Laboratory of Materials Design and Preparation Technology of Hunan Province; Xiangtan Univ., Hunan (China). Key Laboratory of Key Film Materials and Application for Equipment (Hunan province); Ji, Li [South China University of Technology, Guangdong (China). School of Materials Science and Engineering

    2017-01-15

    The isothermal section of the Fe-Al-Ce system at 600 C was determined by means of scanning electron microscopy coupled with energy dispersive spectroscopy and X-ray powder diffraction. Twenty three-phase regions were confirmed experimentally, and two three-phase regions could be deduced in this section. Five ternary compounds, i. e., τ{sub 1}, τ{sub 2}, τ{sub 3}, τ{sub 5}, and τ{sub 6}, exist at 600 C. The Fe{sub 2}Ce phase contains 6.6 at.% Al in the Fe-Al-Ce system. The Fe solubility in α-Al, αAl{sub 11}Ce{sub 3}, αAl{sub 3}Ce, Al{sub 2}Ce, AlCe, and AlCe{sub 3} is approximately 1.7 at.%, 1.1 at.%, 1.2 at.%, 1.3 at.%, 5.8 at.%, and 0.1 at.%, respectively, and the solubility of Ce in α-Al, FeAl{sub 3}, Fe{sub 2}Al{sub 5}, FeAl{sub 2}, and FeAl is approximately 0.1 at.%, 1.2 at.%, 1.9 at.%, 0.9 at.%, and 3.7 at.%, respectively.

  14. A thermodynamic assessment of the La-Al system

    International Nuclear Information System (INIS)

    Yin, F.; Su, X.; Li, Z.; Huang, M.; Shi, Y.

    2000-01-01

    The optimized descriptions of the phase diagram and thermodynamic properties of the La-Al system have been obtained from experimental thermodynamic and phase diagram data by means of the computer program thermo-calc based on the least squares method, using models for the Gibbs energy of individual phases. The system contains six intermetallic compounds. A consistent set of thermodynamic parameters was derived. Optimized and experimental data are in good agreement (orig.)

  15. Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease.

    Science.gov (United States)

    Priyamvada, P S; Morkhandikar, S; Srinivas, B H; Parameswaran, S

    2015-01-01

    Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig) light chains. In heavy chain amyloidosis (AH), deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL), the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda) AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

  16. Isothermal section of the Er-Fe-Al ternary system at 800 oC

    International Nuclear Information System (INIS)

    Jemmali, M.; Walha, S.; Pasturel, M.; Tougait, O.; Ben Hassen, R.; Noel, H.

    2010-01-01

    Physico-chemical analysis techniques, including X-ray diffraction and Scanning Electron Microscope-Energy Dispersive X-ray Spectroscopy, were employed to construct the isothermal section of the Er-Fe-Al system at 800 o C. At this temperature, the phase diagram is characterized by the formation of five intermediate phases, ErFe 12-x Al x with 5 ≤ x ≤ 8 (ThMn 12 -type), ErFe 1+x Al 1-x with -0.2 ≤ x ≤ 0.75 (MgZn 2 -type), ErFe 3-x Al x with 0.5 2 Al-type), Er 2 Fe 17-x Al x with 4.74 ≤ x ≤ 5.7 (TbCu 7 -type) and Er 2 Fe 17-x Al x with 5.7 2 Zn 17 -type), seven extensions of binaries into the ternary system; ErFe x Al 3-x with x 3 Cu-type), ErFe x Al 2-x with x ≤ 0.68 (MgCu 2 -type), Er 2 Fe x Al 1-x with x ≤ 0.25 (Co 2 Si-type), ErFe 2-x Al x with x ≤ 0.5 (MgCu 2 -type), ErFe 3-x Al x with x ≤ 0.5 (Be 3 Nb-type), Er 6 Fe 23-x Al x with x ≤ 8 (Th 6 Mn 23 -type), and Er 2 Fe 17-x Al x with x ≤ 4.75 (Th 2 Ni 17 -type) and one intermetallic compound; the ErFe 2 Al 10 (YbFe 2 Al 10 -type).

  17. Primary extranodal marginal zone B-cell lymphoma with AL amyloidosis in cerebral parenchyma in an immunocompetent patient

    Directory of Open Access Journals (Sweden)

    Tadashi Terada, M.D., Ph.D.

    2015-09-01

    Full Text Available Herein reported is an extremely rare case of primary MALT lymphoma of cerebral parenchyma. A 79-year-old man presented with paresis. Imaging modalities identified a tumor measuring 3 cm in diameter in right cerebral parenchyma. An operation completely resected the tumor. Macroscopically, the tumor was well defined, but showed mild infiltrative features. Histologically, the tumor showed proliferation of small atypical lymphocytes separated by fibrous septae with AL amyloid depositions. No apparent plasma cell differentiation was seen. The tumor cells showed monotonous appearances with hyperchromatic nuclei but without nucleoli and nuclear indentations. Immunoblastic cells were scattered. Immunohistochemically, tumor cells were positive for vimentin, CD45, CD20, CD79α, bcl-2, CD3 (focal, CD45RO (focal, CD5 (focal, CD10, CD23, bcl-6, CD138, p53, and Ki-67 (labeling = 27%. The immunoblastic cells were positive for CD30. The lymphoid cells were negative for Epstein–Barr virus (EBV-related molecules of EBV latent membrane protein-1 (LMP-1 and EBV early RNAs (EBER. They were also negative for cytokeratins AE 1/3 and CAM5.2, cyclin D1, CD34, GFAP, α-smooth muscle actin, and S100 protein. Because of the heterogeneity of tumor cells and positive AL amyloid deposition, the author diagnosed it as primary MALT lymphoma. The patient is now free from tumors. Differential diagnosis was discussed.

  18. Predictions of thermomagnetic properties of Laves phase compounds: TbAl2, GdAl2 and SmAl2 performed with ATOMIC MATTERS MFA computation system

    Science.gov (United States)

    Michalski, Rafał; Zygadło, Jakub

    2018-04-01

    Recent calculations of properties of TbAl2 GdAl2 and SmAl2 single crystals, performed with our new computation system called ATOMIC MATTERS MFA are presented. We applied localized electron approach to describe the thermal evolution of Fine Electronic Structure of Tb3+, Gd3+ and Sm3+ ions over a wide temperature range and estimate Magnetocaloric Effect (MCE). Thermomagnetic properties of TbAl2, GdAl2 and SmAl2 were calculated based on the fine electronic structure of the 4f8, 4f7 and 4f5 electronic configuration of the Tb3+ and Gd3+ and Sm3+ ions, respectively. Our calculations yielded: magnetic moment value and direction; single-crystalline magnetization curves in zero field and in external magnetic field applied in various directions m(T,Bext); the 4f-electronic components of specific heat c4f(T,Bext); and temperature dependence of the magnetic entropy and isothermal entropy change with external magnetic field - ΔS(T,Bext). The cubic universal CEF parameters values used for all CEF calculations was taken from literature and recalculated for universal cubic parameters set for the RAl2 series: A4 = +7.164 Ka04 and A6 = -1.038 Ka06. Magnetic properties were found to be anisotropic due to cubic Laves phase C15 crystal structure symmetry. These studies reveal the importance of multipolar charge interactions when describing thermomagnetic properties of real 4f electronic systems and the effectiveness of an applied self-consistent molecular field in calculations for magnetic phase transition simulation.

  19. Thermodynamic modeling of the Mg-Al-Ca system

    Energy Technology Data Exchange (ETDEWEB)

    Janz, A.; Groebner, J. [Clausthal University of Technology, Institute of Metallurgy, Robert-Koch-Str. 42, D-38678 Clausthal-Zellerfeld (Germany); Cao, H.; Zhu, J.; Chang, Y.A. [Department of Materials Science and Engineering, University of Wisconsin, 1509 University Ave., Madison, WI 53706 (United States); Schmid-Fetzer, R. [Clausthal University of Technology, Institute of Metallurgy, Robert-Koch-Str. 42, D-38678 Clausthal-Zellerfeld (Germany)], E-mail: schmid-fetzer@tu-clausthal.de

    2009-02-15

    A thermodynamic model has been developed that provides a quantitative description for a wide area of the Mg-Al-Ca system. All available experimental data plus new key experiments using differential scanning calorimetry/differential thermal analysis have been considered to create a dataset which reproduces the primary crystallizing phases, the extensive ternary solubilities of binary phases and the ternary C36 Laves phase. This enables validated thermodynamic calculations in various areas of this ternary system.

  20. Neutron diffraction determination of atomic mean-square displacements in cubic compounds of Ni-Al and Ni-Al-Cu systems

    International Nuclear Information System (INIS)

    Khidirov, I.; Mukhtarova, N.N.

    2002-01-01

    The atomic mean-square displacements (AMSD) are some of important characteristics of the solid and can be the main information for determination of a number of other characteristics of substances. In the work AMSD is determined for a number of cubic compounds of Ni-Al, Ni-Al-Cu systems immediately from intensities of neutron diffraction maxima. It is shown by the offered method that in all NiAl x and NiAlCu x compounds with the CsCl - type structure AMSD are near each other and they are practically constant. Therefore it is possible to assume that within the homogeneity region of these compounds the interatomic bond forces are changed insignificantly

  1. Composition of Cu/Al system constructed by means of dynamic atomic deposition

    International Nuclear Information System (INIS)

    Tashlykov, I.S.; Tul'ev, V.V.

    2011-01-01

    Rutherford backscattering and RUMP simulation programme have been applied to investigate composition of Cu/Al system prepared using dynamic atomic deposition process when deposition of Cu thin film on Al substrate was assisted with 6 keV Ar + ions irradiation. It is estimated that thin ( ~15 nm) surface layer consists of ~50 at.% Cu, ~10 at.% Ar, ~4 at.% O and the remaining is Al. Dynamic deposition of Cu on Al substrate is accompanied with radiation enhanced diffusion of Cu, O, Ar atoms in substrate and out diffusion of Al atoms in deposited Cu coating. (authors)

  2. The Prognosis of the Phase Equilibrium Diagram of the System Al-Cu-Si

    Directory of Open Access Journals (Sweden)

    Florentina Cziple

    2007-10-01

    Full Text Available The paper presents a model for establishing the mathematical functions of the liquidus and solidus curves, from the binary diagrams Al-Si, Si-Cu, Cu-Al and their use in the prognosis of the phase equilibrium diagram from the ternary system Al-Cu-Si. We have studied the model of the non-ideal liquid solution of the regular type. The calculus and graphic plotting of the equations for the binary systems has been performed on the computer

  3. Calculation of Gibbs energy of Zr-Al-Ni, Zr-Al-Cu, Al-Ni-Cu and Zr-Al-Ni-Cu liquid alloys based on quasiregular solution model

    International Nuclear Information System (INIS)

    Li, H.Q.; Yang, Y.S.; Tong, W.H.; Wang, Z.Y.

    2007-01-01

    With the effects of electronic structure and atomic size being introduced, the mixing enthalpy as well as the Gibbs energy of the ternary Zr-Al-Cu, Ni-Al-Cu, Zr-Ni-Al and quaternary Zr-Al-Ni-Cu systems are calculated based on quasiregular solution model. The computed results agree well with the experimental data. The sequence of Gibbs energies of different systems is: G Zr-Al-Ni-Cu Zr-Al-Ni Zr-Al-Cu Cu-Al-Ni . To Zr-Al-Cu, Ni-Al-Cu and Zr-Ni-Al, the lowest Gibbs energy locates in the composition range of X Zr 0.39-0.61, X Al = 0.38-0.61; X Ni = 0.39-0.61, X Al = 0.38-0.60 and X Zr = 0.32-0.67, X Al = 0.32-0.66, respectively. And to the Zr-Ni-Al-Cu system with 66.67% Zr, the lowest Gibbs energy is obtained in the region of X Al = 0.63-0.80, X Ni = 0.14-0.24

  4. Effect of Al in the H2 storage properties of Li-NH system

    International Nuclear Information System (INIS)

    Fernandez Albanesi, L; Lago, Marcelo N; Arneodo Larochette, P; Gennari, F.C

    2012-01-01

    In this work is studied the effect of the addition of aluminum on the properties of hydrogen absorption-desorption system LiNH 2 : 1,5 LiH. The samples were prepared adding the element Al to the system LiNH 2 :1.5 LiH in different ways (as aluminum metal, as aluminum chloride and as lithium aluminum hydride). In all these mixtures was maintained the concentration of Al in 1 mol%. A good distribution of Al is achieved by mechanical milling. Quantitative hydrogen absorption and desorption were measured by volumetric method with a Sieverts-type apparatus in the temperature range of 250 to 300 o C and 700 kPa of H 2 pressure. The systems with Al showed better absorption kinetics and greater stability after cycles of hydrogen absorption-desorption. The hydrogen capacity achieved by these samples was 5.3 wt% at 300 and 275 o C. The mixture with AlCl 3 displays the same characteristics at 250 o C, showing the better H 2 storage properties

  5. Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease

    Directory of Open Access Journals (Sweden)

    P S Priyamvada

    2015-01-01

    Full Text Available Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig light chains. In heavy chain amyloidosis (AH, deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL, the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

  6. Thermodynamic assessments and inter-relationships between systems involving Al, Am, Ga, Pu, and U

    Energy Technology Data Exchange (ETDEWEB)

    Perron, A., E-mail: perron1@llnl.gov [Lawrence Livermore National Laboratory, Livermore, CA 94550 (United States); Turchi, P.E.A.; Landa, A. [Lawrence Livermore National Laboratory, Livermore, CA 94550 (United States); Oudot, B.; Ravat, B.; Delaunay, F. [CEA-Centre de Valduc, 21120, Is sur Tille (France)

    2016-12-15

    A newly developed self-consistent CALPHAD thermodynamic database involving Al, Am, Ga, Pu, and U is presented. A first optimization of the slightly characterized Am-Al and completely unknown Am-Ga phase diagrams is proposed. To this end, phase diagram features as crystal structures, stoichiometric compounds, solubility limits, and melting temperatures have been studied along the U-Al → Pu-Al → Am-Al, and U-Ga → Pu-Ga → Am-Ga series, and the thermodynamic assessments involving Al and Ga alloying are compared. In addition, two distinct optimizations of the Pu-Al phase diagram are proposed to account for the low temperature and Pu-rich region controversy. The previously assessed thermodynamics of the other binary systems (Am-Pu, Am-U, Pu-U, and Al-Ga) is also included in the database and is briefly described in the present work. Finally, predictions on phase stability of ternary and quaternary systems of interest are reported to check the consistency of the database.

  7. The Al-rich region of the Al–Fe–Mn alloy system

    International Nuclear Information System (INIS)

    Balanetskyy, S.; Pavlyuchkov, D.; Velikanova, T.; Grushko, B.

    2015-01-01

    Highlights: • Constitution of Al–Fe–Mn was studied above 50 at.% Al at 650–1070 °C. • AlMn (A2) and AlFe (B2) phases form a continuous compositional region. • Al 8 Mn 5 and Al 8 Fe 5 γ-brass type phases form a continuous compositional region. • Al 13 Fe 4 , Al 5 Fe 2 , Al 2 Fe, Al 6 Mn, Al 11 Mn 4 , γ 2 exhibit wide ternary extensions. • Four ternary intermetallics were revealed. - Abstract: Phase equilibria in the Al-rich region of the Al–Fe–Mn alloy system were studied at 1070, 1020, 950, 875, 800, 740, 695 and 650 °C. The continuous region of the bcc solid solution was estimated between the Al–Mn and Al–Fe terminals. Also the isostructural high-temperature Al–Mn and Al–Fe γ 1 -phases (γ-brass type structure) form continuous regions. The Al 6 Mn, high-temperature T-Al 11 Mn 4 and low-temperature γ 2 phases dissolve up to 9.0, 14.5 and 31.0 at.% Fe, respectively, while the M-Al 13 Fe 4 , Al 5 Fe 2 and Al 2 Fe phases dissolve up to 15.5, 11.5 and 10.0 at.% Mn, respectively. The thermodynamically stable decagonal D 3 -phase with periodicity of 1.25 nm in the specific direction and two periodic intermetallics designated φ (P6 3 /mmc; a = 0.7554, c = 0.7872 nm) and κ (P6 3 /m; a = 1.7630, c = 1.2506 nm) were identified. An additional ternary phase of unknown structure was also revealed

  8. Lack of evidence for protein AA reactivity in amyloid deposits of lattice corneal dystrophy and amyloid corneal degeneration.

    Science.gov (United States)

    Gorevic, P D; Rodrigues, M M; Krachmer, J H; Green, C; Fujihara, S; Glenner, G G

    1984-08-15

    Amyloid fibrils occurring in primary and myeloma-associated (AL), secondary (AA), and certain neuropathic hereditary forms of systemic amyloidosis can be distinguished biochemically or immunohistologically as being composed of immunoglobulin light chain, protein AA, or prealbumin respectively. All types of systemic and several localized forms of amyloidosis contain amyloid P component (protein AP). We studied formalin-fixed tissue from eight cases of lattice corneal dystrophy by the immunoperoxidase method using antisera to proteins AA and AP, to normal serum prealbumin and prealbumin isolated from a case of hereditary amyloidosis, and to light-chain determinants; additional cases were examined by indirect immunofluorescence of fresh-frozen material. We found weak (1:10 dilution) staining with anti-AP, but no reactivity with other antisera. Congo red staining was resistant to pretreatment of sections with potassium permanganate, a characteristic of non-AA amyloid. Two-dimensional gels of solubilized proteins from frozen tissue from two cases of lattice corneal dystrophy resembled those obtained from normal human cornea. Western blots of two cases of polymorphous amyloid degeneration and solubilized protein from normal cornea did not react with radioactive iodine-labeled anti-AA or anti-AP with purified protein AP and unfixed protein AA amyloid tissue as controls. We were unable to corroborate the presence of protein AA in the amyloid deposits of lattice corneal dystrophy. Although staining with antiserum to protein AP was demonstrable, the molecular configuration of this protein in stromal deposits remains to be defined.

  9. Thermodynamic description of the Al-Cu-Yb ternary system supported by first-principles calculations

    Directory of Open Access Journals (Sweden)

    Huang G.

    2016-01-01

    Full Text Available Phase relationships of the ternary Al-Cu-Yb system have been assessed using a combination of CALPHAD method and first principles calculations. A self-consistent thermodynamic parameter was established based on the experimental and theoretical information. Most of the binary intermetallic phases, except Al3Yb, Al2Yb, Cu2Yb and Cu5Yb, were assumed to be zero solubility in the ternary system. Based on the experimental data, eight ternary intermetallic compounds were taken into consideration in this system. Among them, three were treated as line compounds with large homogeneity ranges for Al and Cu. The others were treated as stoichiometric compounds. The calculated phase diagrams were in agreement with available experimental and theoretical data.

  10. Low power rf system for the ALS Linac

    International Nuclear Information System (INIS)

    Lo, C.C.; Taylor, B.; Lancaster, H.

    1991-05-01

    The Linear Accelerator (Linac) in the Advanced Light Source (ALS) is designed to provide either single or multiple bunchers of 50 MeV electrons for the booster synchrotron. Three cavities are used in the Linac for electron bunching. The two subharmonic bunching cavities operate at 124.914 MHz and 499.654 MHz respectively. The S Band buncher operates at 2.997924 GHz. The low level RF system includes a master signal source, RF burst generators, signal phase control, timing trigger generators and a water temperature control system. The design and performance of the system will be described. 7 refs., 3 figs

  11. Modified embedded-atom method interatomic potential for the Fe-Al system

    International Nuclear Information System (INIS)

    Lee, Eunkoo; Lee, Byeong-Joo

    2010-01-01

    An interatomic potential for the Fe-Al binary system has been developed based on the modified embedded-atom method (MEAM) potential formalism. The potential can describe various fundamental physical properties of Fe-Al binary alloys-structural, elastic and thermodynamic properties, defect formation behavior and interactions between defects-in reasonable agreement with experimental data or higher-level calculations. The applicability of the potential to atomistic investigations of various defect formation behaviors and their effects on the mechanical properties of high aluminum steels as well as Fe-Al binary alloys is demonstrated.

  12. Prospects for designing structural cast eutectic alloys on Al-Ce-Ni system base

    International Nuclear Information System (INIS)

    Belov, N.A.; Naumova, E.S.

    1996-01-01

    The phase diagram of Al-Ce-Ni system is built for an aluminium corner at component concentration up to 16 mass %Ce and 8 mass%Ni. A ternary eutectic reaction is established at 12%Ce, 5%Ni and 626 deg C. The ternary eutectic alloy is similar in structure to rapidly cooled Al base alloys with transition metals. The possibility to design new cast alloys based on three-phase (Al)+NiAl 3 +CeAl 4 eutectics is under consideration. Al-Zn-Mg-Cu, Al-Sc and Al-Zr base alloys can be used as (Al) constituent of the eutectics. The new alloys may be considered as heat resistant ones due to the fact that no structural changes are observed in castings on heating up to 350 deg C. 18 refs.; 4 figs.; 2 tabs

  13. l'amylose laryngée à propos d'un cas laryngeal location of amylosis

    African Journals Online (AJOL)

    maxillofac. 1992 ; 93 : 54-57. 5-Vicente Villagomez, Felicitos Santos, Ramiro Santos and al. Amyloidosis: Uncommon cause of dysphonia. Head and Neck Surgery 2004, 13: 1275-276. 6- Quinquenel ML, Le Coza, Desrues B et ...

  14. Design of the ALS transverse coupled-bunch feedback system

    International Nuclear Information System (INIS)

    Barry, W.; Byrd, J.M.; Corlett, J.N.; Hinkson, J.; Johnson, J.; Lambertson, G.R.; Fox, J.D.

    1993-05-01

    Calculations of transverse coupled bunch growth rates in the Advanced Light Source (ALS), a 1.5 GeV electron storage ring for producing synchrotron radiation, indicate the need for damping via a transverse feedback (TFB) system. We present the design of such a system. The maximum bunch frequency is 500 MHz, requiring that the FB system have a broadband response of at least 250 MHz. We described, in detail, the choice of broadband components such as kickers, pickups, power amplifiers, and electronics

  15. The relation between wetting and infiltration behaviour in the Al-1010/TiC and Al-2024/TiC Systems

    International Nuclear Information System (INIS)

    Contreras, A.; Lopez, V.H.; Leon, C.A.; Bedolla, E.; Drew, R.A.A.

    2001-01-01

    Wetting and infiltration behavior of TiC by commercial aluminum (Al-1010) and Al-2024 alloy was investigated. Wettability tests were performed on dense TiC substrates (96.8% theoretical density) using a sessile drop technique in the temperature range of 850 to 1000 deg C under vacuum atmosphere. Pressureless melt infiltration of particulate TiC performs (56% theoretical density) was carried out under an inert atmosphere at temperatures ranging from 900 to 1100 deg C. Infiltration profiles were obtained using a thermogravimetric analyzer (TGA), which measured continuously the weight change of the compacts as the liquid alloy infiltrated From the wetting study, it was found that wettability of TiC by liquid Al-1010 was better than Al-2024. A strong temperature dependence was observed. Electron probe microanalysis (EPMA) indicated that aluminum carbide (Al 4 C 3 ) is formed at the interface in both metal/ceramic assemblies. In agreement with the wetting results. Al-1010 exhibited the highest infiltration rate during composite fabrication. The activation energy determined from the slopes of Arrhenius plots for the infiltration rate at the different temperatures was 172 kJ/mol and 179 kJ/mol for the Al-1010/TiC and Al-2024/TiC systems, respectively. Copyright (2000) AD-TECH - International Foundation for the Advancement of Technology Ltd

  16. Bilateral metachronous periosteal tibial amyloid tumors

    International Nuclear Information System (INIS)

    Murata, H.; Kusuzaki, Katsuyuki; Hashiguchi, S.; Ueda, Hidetaka; Hirasawa, Yasusuke

    2000-01-01

    Localized primary periosteal amyloid tumors are extremely rare. A case of bilateral tibial amyloid tumor is presented. A 62-year-old woman initially presented with a painful mass in the anterior aspect of the right leg. There was no evidence of underlying systemic disease, including chronic infection or malignancy. Based on the results of resistance with Congo red staining to treatment with potassium permanganate and positivity for kappa light chain, we classified this particular case as AL-type amyloidosis. The patient noticed a swelling in the opposite leg 2 years later. The second tumor was also an AL-type amyloidoma. Amyloid tumors are generally solitary. This is the first case of bilateral periosteal amyloid tumors of the AL-type occurring in the tibiae. (orig.)

  17. Generation and evolution of nanoscale AlP and Al{sub 13}Fe{sub 4} particles in Al-Fe-P system

    Energy Technology Data Exchange (ETDEWEB)

    Qiao, Huan; Gao, Tong; Zhu, Xiangzhen; Wu, Yuying; Qian, Zhao; Liu, Xiangfa, E-mail: xfliu@sdu.edu.cn

    2015-02-15

    Highlights: • Diffusion and gradual solid reactions between Al and Fe{sub x}P phases in Al-Fe-P alloy were investigated. • Nanoscale AlP clusters are in-situ generated and evolve during the whole process. • This novel Al-Fe-P alloy has an excellent low-temperature refining performance on hypereutectic Al-Si alloy. - Abstract: In this paper, the gradual solid reactions between Al and Fe{sub x}P phases in Al-Fe-P alloy were investigated. The results show that the whole reaction process undergoes four main stages: the diffusion of Al atom, the generation of (Al, Fe, P) intermediate compound, the precipitation of nano AlP and Al{sub 13}Fe{sub 4} clusters and their growth to submicron particles. The microstructure of Fe-P particles evolves from the “egg-type”, the “sponge-type” to the “sesame-cake” structure. AlP and Al{sub 13}Fe{sub 4} nano phases have in-situ generated and evolved during the whole process. The gradual reaction mechanism has been discussed. Furthermore, a novel Al-Fe-P alloy which contains (Al, Fe, P) intermediate compounds and nano AlP particles has been synthesized and its low-temperature refining performance on A390 alloy has also been investigated.

  18. Simulation of the ALS longitudinal multibunch feedback system

    International Nuclear Information System (INIS)

    Byrd, J.

    1993-05-01

    Longitudinal coupled bunch growth rates in the Advanced Light Source (ALS), a 1.5 GeV electron storage ring for producing synchrotron radiation, indicate the need for damping via a feedback (FB) system. The design of the system is based on the proposed PEP-II longitudinal FB system which uses a digital filter to provide the required phase and amplitude response. We report the results of a detailed computer simulation of the FB system including single particle longitudinal beam dynamics, measured RF cavity fundamental and higher order modes, and response of major FB components such as the power amplifier and kicker. The simulation addresses issues such as required FB power and gain, noise, digital filter effects, and varying initial bunch conditions

  19. Thermodynamic assessment of the Al-Ru system

    CSIR Research Space (South Africa)

    Prins, SN

    2003-03-01

    Full Text Available describes the order disorder transformation with one Gibbs energy function. The RuAl6 phase was described as a stoichiometric phase and the remaining intermetallic phases (Ru4Al13, RuAl2 and Ru2Al3) were modelled with the sublattice model. The solubility...

  20. New investigation of phase equilibria in the system Al-Cu-Si.

    Science.gov (United States)

    Ponweiser, Norbert; Richter, Klaus W

    2012-01-25

    The phase equilibria and invariant reactions in the system Al-Cu-Si were investigated by a combination of optical microscopy, powder X-ray diffraction (XRD), differential thermal analysis (DTA) and electron probe micro analysis (EPMA). Isothermal phase equilibria were investigated within two isothermal sections. The isothermal section at 500 °C covers the whole ternary composition range and largely confirms the findings of previous phase diagram investigations. The isothermal section at 700 °C describes phase equilibria only in the complex Cu-rich part of the phase diagram. A new ternary compound τ was found in the region between (Al,Cu)-γ(1) and (Cu,Si)-γ and its solubility range was determined. The solubility of Al in κ-CuSi was found to be extremely high at 700 °C. In contrast, no ternary solubility in the β-phase of Cu-Al was found, although this phase is supposed to form a complete solid solution according to previous phase diagram assessments. Two isopleths, at 10 and 40 at.% Si, were investigated by means of DTA and a partial ternary reaction scheme (Scheil diagram) was constructed, based on the current work and the latest findings in the binary systems Al-Cu and Cu-Si. The current study shows that the high temperature equilibria in the Cu-rich corner are still poorly understood and additional studies in this area would be favorable.

  1. Construction and evaluation of multi-component Zn-Al based bearing alloys (Zn-Al-Si, Zn-Al-Cu)

    International Nuclear Information System (INIS)

    Shahmiri, M.; Shahin, K.

    2001-01-01

    Zn-Al based alloys, with excellent mechanical properties, are finding increasing applications in various industries, especially bearing and bushing fields. Observed dimensional instabilities, in their multicomponent systems, (e. g. Zn-Al-Si and, Zn-Al Si-Cu), is believed to be as the result of some kinds of phase transformation, due to the temperature variations, while in service. Profound understanding of the phase transformations due to the temperature variation, requires detailed evaluations of the isothermal sections of the multi-components phase diagrams of Zn-Al-Si and, Zn-Al-Si-Cu alloy systems. In the present article, the isothermal sections of the aforementioned ternary and quaternary systems in the solid state regions have been investigated and observed phase transitions have been critically evaluated

  2. Variation of amino acid sequences of serum amyloid a (SAA) and immunohistochemical analysis of amyloid a (AA) in Japanese domestic cats.

    Science.gov (United States)

    Tei, Meina; Uchida, Kazuyuki; Chambers, James K; Watanabe, Ken-Ichi; Tamamoto, Takashi; Ohno, Koichi; Nakayama, Hiroyuki

    2018-02-02

    Amyloid A (AA) amyloidosis, a fatal systemic amyloid disease, occurs secondary to chronic inflammatory conditions in humans. Although persistently elevated serum amyloid A (SAA) levels are required for its pathogenesis, not all individuals with chronic inflammation necessarily develop AA amyloidosis. Furthermore, many diseases in cats are associated with the elevated production of SAA, whereas only a small number actually develop AA amyloidosis. We hypothesized that a genetic mutation in the SAA gene may strongly contribute to the pathogenesis of feline AA amyloidosis. In the present study, genomic DNA from four Japanese domestic cats (JDCs) with AA amyloidosis and from five without amyloidosis was analyzed using polymerase chain reaction (PCR) amplification and direct sequencing. We identified the novel variation combination of 45R-51A in the deduced amino acid sequences of four JDCs with amyloidosis and five without. However, there was no relationship between amino acid variations and the distribution of AA amyloid deposits, indicating that differences in SAA sequences do not contribute to the pathogenesis of AA amyloidosis. Immunohistochemical analysis using antisera against the three different parts of the feline SAA protein-i.e., the N-terminal, central, and C-terminal regions-revealed that feline AA contained the C-terminus, unlike human AA. These results indicate that the cleavage and degradation of the C-terminus are not essential for amyloid fibril formation in JDCs.

  3. Formation of Al3Ti/Mg composite by powder metallurgy of Mg–Al–Ti system

    Directory of Open Access Journals (Sweden)

    Zi R Yang et al

    2008-01-01

    Full Text Available An in situ titanium trialuminide (Al3Ti-particle-reinforced magnesium matrix composite has been successfully fabricated by the powder metallurgy of a Mg–Al–Ti system. The reaction processes and formation mechanism for synthesizing the composite were studied by differential scanning calorimetry (DSC, x-ray diffractometry (XRD, scanning electron microscopy (SEM and energy-dispersive x-ray spectroscopy (EDS. Al3Ti particles are found to be synthesized in situ in the Mg alloy matrix. During the reaction sintering of the Mg–Al–Ti system, Al3Ti particles are formed through the reaction of liquid Al with as-dissolved Ti around the Ti particles. The formed intermetallic particles accumulate at the original sites of the Ti particles. As sintering time increases, the accumulated intermetallic particles disperse and reach a relatively homogeneous distribution in the matrix. It is found that the reaction process of the Mg–Al–Ti system is almost the same as that of the Al–Ti system. Mg also acts as a catalytic agent and a diluent in the reactions and shifts the reactions of Al and Ti to lower temperatures. An additional amount of Al is required for eliminating residual Ti and solid-solution strengthening of the Mg matrix.

  4. Time-resolved studies define the nature of toxic IAPP intermediates, providing insight for anti-amyloidosis therapeutics

    Science.gov (United States)

    Abedini, Andisheh; Plesner, Annette; Cao, Ping; Ridgway, Zachary; Zhang, Jinghua; Tu, Ling-Hsien; Middleton, Chris T; Chao, Brian; Sartori, Daniel J; Meng, Fanling; Wang, Hui; Wong, Amy G; Zanni, Martin T; Verchere, C Bruce; Raleigh, Daniel P; Schmidt, Ann Marie

    2016-01-01

    Islet amyloidosis by IAPP contributes to pancreatic β-cell death in diabetes, but the nature of toxic IAPP species remains elusive. Using concurrent time-resolved biophysical and biological measurements, we define the toxic species produced during IAPP amyloid formation and link their properties to induction of rat INS-1 β-cell and murine islet toxicity. These globally flexible, low order oligomers upregulate pro-inflammatory markers and induce reactive oxygen species. They do not bind 1-anilnonaphthalene-8-sulphonic acid and lack extensive β-sheet structure. Aromatic interactions modulate, but are not required for toxicity. Not all IAPP oligomers are toxic; toxicity depends on their partially structured conformational states. Some anti-amyloid agents paradoxically prolong cytotoxicity by prolonging the lifetime of the toxic species. The data highlight the distinguishing properties of toxic IAPP oligomers and the common features that they share with toxic species reported for other amyloidogenic polypeptides, providing information for rational drug design to treat IAPP induced β-cell death. DOI: http://dx.doi.org/10.7554/eLife.12977.001 PMID:27213520

  5. Hydrogen migration dynamics in hydrated Al clusters: The Al{sub 17}{sup (−)}·H{sub 2}O system as an example

    Energy Technology Data Exchange (ETDEWEB)

    Álvarez-Barcia, S.; Flores, J. R., E-mail: flores@uvigo.es [Facultad de Química, Universidad de Vigo, E-36310-Vigo (Pontevedra) (Spain)

    2014-02-28

    The Al{sub m}{sup (−)}·(H{sub 2}O){sub n} systems are known to undergo water splitting processes in the gas phase giving H{sub k}Al{sub m}(OH){sub k}{sup (−)}·(H{sub 2}O){sub n−k} systems, which can generate H{sub 2}. The migration of H atoms from one Al atom to another on the cluster's surface is of critical importance to the mechanism of the complete H{sub 2} production process. We have applied a combination of Molecular Dynamics and Rice-Ramsperger-Kassel-Marcus theory including tunneling effects to study the gas-phase evolution of HAl{sub 17}(OH){sup (−)}, which can be considered a model system. First, we have performed an extensive search for local minima and the connecting saddle points using a density functional theory method. It is found that in the water-splitting process Al{sub 17}{sup (−)}·(H{sub 2}O) → HAl{sub 17}(OH){sup (−)}, the H atom which bonds to the Al cluster losses rather quickly its excess energy, which is easily “absorbed” by the cluster because of its flexibility. This fact ultimately determines that long-range hydrogen migration is not a very fast process and that, probably, tunneling only plays a secondary role in the migration dynamics, at least for moderate energies. Reduction of the total energy results in the process being very much slowed down. The consequences on the possible mechanisms of H{sub 2} generation from the interaction of Al clusters and water molecules are discussed.

  6. Amyloidosis and spontaneous hepatic bleeding, transcatheter therapy for hepatic parenchymal bleeding with massive intraperitoneal hemorrhage: a case report and review of the literature.

    Science.gov (United States)

    Mousa, Albeir Y; Abu-Halimah, Shadi; Alhalbouni, Saadi; Hass, Stephen M; Yang, Calvin; Gill, Gurpreet; AbuRahma, Ali F; Bates, Mark

    2014-10-01

    Hepatic hemorrhage can be devastating, especially in patients with underlying hepatic pathology. This is a case report of a 50-year-old man who presented to the emergency room with Stage 3 shock as evidenced by a systolic blood pressure of 90 mmHg, a heart rate of 125 beats per minute, respiration of 32, with delayed capillary refill and agitation. At this time, he was found to have a massive spontaneous intra-abdominal hemorrhage with an advanced stage of amyloidosis with multiple organ malfunctions. The initial diagnosis was based on an abdominal computed tomography scan and the patient was taken expeditiously to a hybrid angiography suite for a celiac angiogram. An intraoperative diagnosis of extravasation from amyloid related vasculopathy was made based on the angiographic appearance of hepatic circulation. Coil embolization of the feeding branch of the bleeder was achieved using the interlock coil system and a completion angiogram was done showing complete cessation of active bleeding. The postoperative phase was uneventful and the patient was discharged home on postoperative day three. His postoperative visit at five months later was unremarkable. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  7. The influence of thermal treatment on the phase development in HfO2-Al2O3 and ZrO2-Al2O3 systems

    International Nuclear Information System (INIS)

    Stefanic, G.; Music, S.; Trojko, R.

    2005-01-01

    Amorphous precursors of HfO 2 -AlO 1.5 and ZrO 2 -AlO 1.5 systems covering the whole concentration range were co-precipitated from aqueous solutions of the corresponding salts. The thermal behaviour of the amorphous precursors was examined by differential thermal analysis, X-ray powder diffraction (XRD), laser Raman spectroscopy and scanning electron microscopy. The crystallization temperature of both systems increased with increase in the AlO 1.5 content, from 530 to 940 deg. C in the HfO 2 -AlO 1.5 system, and from 405 to 915 deg. C in the ZrO 2 -AlO 1.5 system. The results of phase analysis indicate an extended capability for the incorporation of Al 3+ ions in the metastable HfO 2 - and ZrO 2 -type solid solutions obtained after crystallization of amorphous co-gels. Precise determination of lattice parameters, performed using whole-powder-pattern decomposition method, showed that the axial ratio c f /a f in the ZrO 2 - and HfO 2 -type solid solutions with 10 mol% or more of Al 3+ approach 1. The tetragonal symmetry of these samples, as determined by laser Raman spectroscopy, was attributed to the displacement of the oxygen sublattice from the ideal fluorite positions. It was found that the lattice parameters of the ZrO 2 -type solid solutions decreased with increasing Al 3+ content up to ∼10 mol%, whereas above 10 mol%, further increase of the Al 3+ content has very small influence on the unit-cell volume of both HfO 2 - and ZrO 2 -type solid solutions. The reason for such behaviour was discussed. The solubility of Hf 4+ and Zr 4+ ions in the aluminium oxides lattice appeared to be negligible

  8. Stability, electronic structures, and mechanical properties of Fe–Mn–Al system from first-principles calculations

    International Nuclear Information System (INIS)

    Liu Ya-Hui; Chong Xiao-Yu; Jiang Ye-Hua; Feng Jing

    2017-01-01

    The stability, electronic structures, and mechanical properties of the Fe–Mn–Al system were determined by first-principles calculations. The formation enthalpy and cohesive energy of these Fe–Mn–Al alloys are negative and show that the alloys are thermodynamically stable. Fe 3 Al, with the lowest formation enthalpy, is the most stable compound in the Fe–Mn–Al system. The partial density of states, total density of states, and electron density distribution maps of the Fe– Mn–Al alloys were analyzed. The bonding characteristics of these Fe–Mn–Al alloys are mainly combinations of covalent bonding and metallic bonds. The stress-strain method and Voigt–Reuss–Hill approximation were used to calculate the elastic constants and moduli, respectively. Fe 2.5 Mn 0.5 Al has the highest bulk modulus, 234.5 GPa. Fe 1.5 Mn 1.5 Al has the highest shear modulus and Young’s modulus, with values of 98.8 GPa and 259.2 GPa, respectively. These Fe–Mn–Al alloys display disparate anisotropies due to the calculated different shape of the three-dimensional curved surface of the Young’s modulus and anisotropic index. Moreover, the anisotropic sound velocities and Debye temperatures of these Fe–Mn–Al alloys were explored. (paper)

  9. Microstructural defects modeling in the Al-Mo system

    International Nuclear Information System (INIS)

    Pascuet, Maria I.; Fernandez, Julian R.; Monti, Ana M.

    2006-01-01

    In this work we have utilized computer simulation techniques to study microstructural defects, such as point defects and interfaces, in the Al-Mo alloy. Such alloy is taken as a model to study the Al(fcc)/U-Mo(bcc) interface. The EAM interatomic potential used has been fitted to the formation energy and lattice constant of the AlMo 3 intermetallic. Formation of vacancies for both components Al and Mo and anti-sites, Al Mo and Mo Al , as well as vacancy migration was studied in this structure. We found that the lowest energy defect complex that preserves stoichiometry is the antisite pair Al Mo +Mo Al , in correspondence with other intermetallics of the same structure. Our results also suggest that the structure of the Al(fcc)/Mo(bcc) interface is unstable, while that of the Al(fcc)/Al 5 Mo interface is stable, as observed experimentally. (author) [es

  10. A Novel Missense Mutation in Oncostatin M Receptor Beta Causing Primary Localized Cutaneous Amyloidosis

    Directory of Open Access Journals (Sweden)

    Marjan Saeedi

    2014-01-01

    Full Text Available Primary localized cutaneous amyloidosis (PLCA is a chronic skin disorder, caused by amyloid material deposition in the upper dermis. Autosomal dominant PLCA has been mapped earlier to pathogenic missense mutations in the OSMR gene, which encodes the oncostatin M receptor ß subunit (OSMRß. OSMRß is interleukin-6 family cytokine receptors and possesses two ligands, oncostatin M and interleukin-31, which both have biologic roles in inflammation and keratinocyte cell proliferation, differentiation, and apoptosis. Here, we identified a new OSMR mutation in a Kurdish family for the first time. Blood samples were taken from all the affected individuals in the family. DNA extraction was performed using salting out technique. Primers were designed for intron flanking individual exons of OSMR gene which were subjected to direct sequencing after PCR amplification for each sample. Sequencing showed a C/T substitution at position 613 in the proband. This mutation results in an L613S (leucine 613 to serine amino acid change. The identified mutation was observed in all affected family members but not in 100 ethnically matched healthy controls. Elucidating the molecular basis of familial PLCA provides new insight into mechanisms of itch in human skin and may lead to new therapeutic targets for pruritus.

  11. Calculation of phase equilibria in Ti-Al-Cr-Mn quaternary system for developing lower cost titanium alloys

    International Nuclear Information System (INIS)

    Lu, X.G.; Li, C.H.; Chen, L.Y.; Qiu, A.T.; Ding, W.Z.

    2011-01-01

    Highlights: → This paper is about the concept of designing the lower cost titanium alloy. → The thermodynamic database of Ti-Al-Cr-Mn system is built up by Calphad method. → The pseudobinary sections with Cr: Mn = 3:1 and Al = 3, 4.5 and 6.0 wt% are calculated. → This may provide the theoretical support for designing the lower cost titanium alloy. - Abstract: The Ti-Al-Cr-Mn system is a potentially useful system for lower cost titanium alloy development; however, there are few reports about the experimental phase diagrams and the thermodynamical assessment for this system. In this study, the previous investigations for the thermodynamic descriptions of the sub-systems in the Ti-Al-Cr-Mn system are reviewed, our previous assessment for the related sub-systems in this quaternary system is summarized, the thermodynamical database of this quaternary system is built up by directly extrapolating from all sub-systems assessed by means of the Calphad method, then the pseudobinary sections with Cr:Mn = 3:1 and Al = 0.0, 3.0, 4.5 and 6.0 wt% are calculated, respectively. These pseudobinary phase diagrams may provide the theoretical support for designing the lower cost titanium alloys with different microstructures (α, α + β, and β titanium alloy).

  12. 37 SITUATIONS OF THE AL-MAJIRI SYSTEM OF EDUCATION IN ...

    African Journals Online (AJOL)

    HP

    option but to recruit them as clerks and office attendants in the heyday of ... derivative of the Arabic terminology al-Muhajir (the traveller or migrant).10 The ... system of education is a sin.16 Through this loophole, the future workforce of the.

  13. Phase relationships in the area of the beta aluminate of the system K{sub 2}O-MgO-AL{sub 2}O{sub 3}; Phasenbeziehungen im Bereich der Beta-Aluminate des Systems K{sub 2}O-MgO-Al{sub 2}O{sub 3}

    Energy Technology Data Exchange (ETDEWEB)

    Kroon, P de

    1996-12-01

    The aim of this work was to be able to make statements about the thermodynamic stability of K-{beta}``-Al{sub 2}O{sub 3} in the pseudo-binary system K{sub 2}O-Al{sub 2}O{sub 3} and in the pseudo-ternary system K{sub 2}O-MgO-Al{sub 2}O{sub 3} relative to the adjacent phases of KAlO{sub 2} {alpha}-Al{sub 2}O{sub 3}, MgAl{sub 2}O{sub 4} and K-{beta}-Al{sub 2}O{sub 3}. (orig./MM) [Deutsch] Ziel dieser Arbeit war es, Aussagen ueber die thermodynamische Stabilitaet von K-{beta}``-Al{sub 2}O{sub 3} im pseudobinaeren System K{sub 2}O-Al{sub 2}O{sub 3} und im pseudoternaeren System K{sub 2}O-MgO-Al{sub 2}O{sub 3} relativ zu den benachbarten Phasen KAlO{sub 2}, {alpha}-Al{sub 2}O{sub 3}, MgAl{sub 2}O{sub 4} und K-{beta}-Al{sub 2}O{sub 3} machen zu koennen. (orig./MM)

  14. Properties of vacancies type defects in intermetallic compounds of the Al-Mo system

    International Nuclear Information System (INIS)

    Pascuet, M.I; Fernandez, J.R; Monti, A.M

    2006-01-01

    There are five intermetallic compounds in the Al-Mo system that are stable at low temperatures. Of these, the richest phases in some of the two components are the compounds Al 12 Mo and AlMo 3 , whose Pearson symbols are cI26 and cP8, respectively. In both structures, the atoms of the minority component occupy positions bcc and each one of them is surrounded by 12 atoms first neighbors of the other component. These 13 atoms form icosahedron shaped units or heaps. Unlike what occurs in Al 12 Mo, the AlMo 3 heaps are superposed by sharing atoms from the majority component. The neighboring environment of the majority component is mixed but differs considerably in one or another intermetallic. In each structure, the sites occupied by any given species are crystallographically equivalent, that is, they can self generate from one of the positions and from the crystalline structure's elements of symmetry. This work studies the energy of vacancies and antisites in both compounds and the atomic-jump processes to vacant sites. Computer simulation techniques were used based on minimizing the system's energy. Many-body embedded-atom potentials were used to represent the atomic interactions. The potential mixture used resulted in an adjustment to the crystalline structure of the AlMo 3 phase at low temperatures and to its formation energy (cw)

  15. Interface reactions in the Al-Si-SiC and Mg-Al-Al{sub 2}O{sub 3} composite systems

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, P.K. [Commission of the European Communities, Petten (Netherlands). Inst. for Advanced Materials; Fazal-Ur-Rehman [Imperial Coll. of Science, Technology and Medicine, London (United Kingdom). Dept. of Materials; Fox, S. [Imperial Coll. of Science, Technology and Medicine, London (United Kingdom). Dept. of Materials; Flower, H.M. [Imperial Coll. of Science, Technology and Medicine, London (United Kingdom). Dept. of Materials; West, D.R.F.

    1995-12-31

    Structural and compositional observations are reported on the influence of the interfaces on the mechanisms and kinetics of liquid metal-ceramic reactions in Al-SiC, Al-Si-SiC, Mg-Al{sub 2}O{sub 3} and Mg-Al-Al{sub 2}O{sub 3} composites. The aluminium based materials contained up to 20 vol% SiC in particulate form, and were produced by a spray casting process; subsequently the interface reactions were studied in samples heated to temperatures up to 1100 C. The reaction product was Al{sub 4}C{sub 3} in both Al-SiC and Al-Si-SiC composites. The influence of the crystallography and topology of the SiC particle surfaces on the nucleation of the Al{sub 4}C{sub 3} has been demonstrated; surface asperities play an important role. Growth of nuclei proceeds to form continuous reaction product layers which control the subsequent kinetics. The magnesium based composites contained 5 vol% Al{sub 2}O{sub 3} fibres (3 {mu}m in diameter), and were produced by a liquid infiltration process. SD Safimax fibres with relatively low and high porosity, and also RF Saffil fibres, with a silica binder, were investigated. Fibre porosity plays a major role in accelerating the penetration of Mg into the fibres with reaction to form MgO. Silica binder on the fibre surface transforms to MgO. The reaction rate was reduced by the presence of aluminium in the matrix. The factors controlling the reactions in the aluminium and magnesium based composites are compared. (orig.)

  16. Antifriction coating of Cu-Fe-Al-Pb system for plain bearings

    Science.gov (United States)

    Kotenkov, Pavel; Kontsevoi, Yurii; Mejlakh, Anna; Pastukhov, Eduard; Shubin, Alexey; Goyda, Eduard; Sipatov, Ivan

    2017-09-01

    Aluminium, copper and their compounds are used in common as basis for antifriction coatings of plain bearings. Antifriction testing of plain bearings (based on Al and Cu) made by leading automotive manufacturers from Germany, Japan, USA, United Kingdom and Russia were carried out to make judicious selection of basis for development of new antifriction material. Testing was carried out using friction machine. It was defined that materials based on Cu provide better durability and robustness of plain bearings in comparison with Al based ones. The new antifriction composite coatings based on copper were developed taking into account the requirements specified for plain bearings of internal-combustion engine. Pilot samples of plain bearings with antifriction coatings of Cu-Fe-Al-Pb system were produced. The antifriction composite having Cu-5Fe-5Al5Fe2-10Pb (mass %) composition has demonstrated low friction factor and high wear-resistance. Metallographic analysis of pilot samples was carried out by means of optical and scanning electron microscopy.

  17. Search for new ternary Al, Ga or In containing phases using information forecasting system

    International Nuclear Information System (INIS)

    Kiseleva, N.N.; Burkhanov, G.S.

    1989-01-01

    Automated system of search for regularities in the formation of crystal phases and forecasting of new compounds with required properties, comprising data base on the properties of ternary inorganic compounds and cybernetic forecasting system, has been developed. General principles of operation of the developed information-forecasting system are considered. Efficiency of the system operation is shown, using as an example the search for new ternary compounds with aluminium, gallium and indium, crystallized in ZrNiAl, TiNiSi, ThCr 2 Si 2 , CaAl 2 Si 2 structural types. Results of the above-mentioned phases forecasting are shown

  18. The code of light: al-ghazali and al-suhrawardi

    OpenAIRE

    Gediminaitė, Barbora

    2017-01-01

    The Code of Light: Al-Ghazali and Al-Suhrawardi The Bachelor’s thesis The Code of Light in Islam: al-Ghazali and al-Suhrawardi presents a comparative study of light. The study analyses the philosophical systems of al-Ghazali and al-Suhrawardi through the reconstruction of the image of light and its operation principles. The main subject of the thesis are the attributes of light. The main problem of the research is the semantics and contextuality of light in Islamic theology and philosophy. Th...

  19. Thermodynamic description of the Al-Cu-Mg-Mn-Si quinary system and its application to solidification simulation

    International Nuclear Information System (INIS)

    Chang, Keke; Liu, Shuhong; Zhao, Dongdong; Du, Yong; Zhou, Liangcai; Chen, Li

    2011-01-01

    By means of the first-principles calculations, the enthalpy of formation for the quaternary phase in the Al-Cu-Mg-Si system was computed. A set of self-consistent thermodynamic parameters for the Al-Cu-Mg-Si and Al-Cu-Mn-Si systems was then obtained using CALPHAD approach taking into account the reliable experimental data and the first-principles calculations. The thermodynamic database for the Al-Cu-Mg-Mn-Si system was developed based on the constituent binary, ternary, and quaternary systems. Comprehensive comparisons between the calculated and measured phase diagrams and invariant reactions showed that the experimental information was satisfactorily accounted for by the present thermodynamic description. The obtained database was used to describe the solidification behavior of Al alloys B319.1 (90.2Al-6Si-3.5Cu-0.3Mg, in wt.%) and B319.1 + xMn (x = 0.5-2, in wt.%) under Gulliver-Scheil non-equilibrium condition. The reliability of the present thermodynamic database was also verified by the good agreement between calculation and experiment for Gulliver-Scheil non-equilibrium solidification.

  20. Reinvestigation of the Ce–Co–Al ternary system: Isothermal section at 973 K

    Energy Technology Data Exchange (ETDEWEB)

    Nasri, N. [Unité de Recherche de Chimie des Matériaux et de l’Environnement, ISSBAT, Université de Tunis El Manar, 9 av. Dr. Zoheir Safi, 1006 Tunis (Tunisia); Gastebois, J. [Institut des Sciences Chimiques de Rennes, Chimie du Solide et Matériaux, UMR CNRS 6226, Université Rennes 1, Campus de Beaulieu, 263 av. Général Leclerc, 35042 Rennes Cedex (France); Pasturel, M., E-mail: mathieu.pasturel@univ-rennes1.fr [Institut des Sciences Chimiques de Rennes, Chimie du Solide et Matériaux, UMR CNRS 6226, Université Rennes 1, Campus de Beaulieu, 263 av. Général Leclerc, 35042 Rennes Cedex (France); Belgacem, B. [Unité de Recherche de Chimie des Matériaux et de l’Environnement, ISSBAT, Université de Tunis El Manar, 9 av. Dr. Zoheir Safi, 1006 Tunis (Tunisia); Péron, I.; Gouttefangeas, F. [Centre de Microscopie Electronique à Balayage et microAnalyse, Université Rennes 1, Campus de Beaulieu, 263 av. Général Leclerc, 35042 Rennes Cedex (France); Ben Hassen, R. [Unité de Recherche de Chimie des Matériaux et de l’Environnement, ISSBAT, Université de Tunis El Manar, 9 av. Dr. Zoheir Safi, 1006 Tunis (Tunisia); and others

    2015-04-15

    Highlights: • The experimental isothermal section at 973 K of the Ce–Co–Al system is presented. • This section contains 5 ternary phases and 7 ternary extensions of pseudo-binaries. • DTA experiments are used to estimate the liquid areas. - Abstract: The Ce–Co–Al ternary phase diagram has been reinvestigated at 973 K by means of powder X-ray diffraction, scanning electron microscopy, energy dispersive spectroscopy and differential thermal analyses. It contains 5 ternary phases (CeCo{sub 2}Al{sub 8}, Ce{sub 2}Co{sub 6}Al{sub 19}, CeCoAl{sub 4}, Ce{sub 3}Co{sub 3}Al{sub 4} and CeCoAl) as well as 7 extensions in the ternary field of pseudo-binaries (CeAl{sub 2−x}Co{sub x} with 0 ⩽ x ⩽ 0.18; CeCo{sub 2−x}Al{sub x} with 0 ⩽ x ⩽ 0.21; CeCo{sub 3−y}Al{sub y} with 0 ⩽ y ⩽ 0.20; Ce{sub 2}Co{sub 7−x}Al{sub x} with 0 ⩽ x ⩽ 0.73; Ce{sub 5}Co{sub 19−y}Al{sub y} with 0 ⩽ y ⩽ 0.20; CeCo{sub 5−x}Al{sub x} with 0 ⩽ x ⩽ 0.71 and Ce{sub 2}Co{sub 17−y}Al{sub y} with 0 ⩽ y ⩽ 2.2). Two liquid areas are present at the Al and Ce-rich corners of this isothermal section.

  1. Experimental Investigation of the 1073 K (800 °C) Isothermal Section of the Al-V-Zr Ternary System

    Science.gov (United States)

    Zhu, Yude; Ouyang, Xuemei; Yin, Fucheng; Zhao, Manxiu; Lou, Jia

    2018-03-01

    This work is focused on an experimental investigation of the phase equilibria of the Al-V-Zr system at 1073 K (800 °C). The phase equilibria were analyzed using scanning electron microscopy (SEM) coupled with energy-dispersive spectrometry and X-ray diffraction. The results confirmed the presence of twelve three-phase regions and one ternary compound, Τ, which contains 10.0 to 16.5 at. pct Zr, 52.8 to 55.2 at. pct Al, and 29.3 to 36.3 at. pct V. The T phase can be in equilibrium with Al8V5, Al3Zr, Al2Zr, and α-V. The T phase belongs to the tetragonal crystal system with confirmed lattice parameters of a = 0.658531 nm and c = 0.517334 nm. The Al2Zr phase region is extraordinarily large and can be in equilibrium with all the compounds in the Al-Zr and V-Zr systems, with the exception of the AlZr phase.

  2. Operation and performance of the PEP-II prototype longitudinal damping system at ALS

    International Nuclear Information System (INIS)

    Teytelman, D.; Claus, R.; Fox, J.

    1995-05-01

    A modular programmable longitudinal feedback system has been developed as a component of the PEP-II R+D program. This system is based on a family of VME and VXI packaged signal processing functions which implement a general purpose digital feedback controller for accelerators with bunch spacings of 2 ns. A complete PEP-II prototype system has been configured and installed for use at the LBL Advanced Light Source. The system configuration used for tests at the ALS is described and results are presented showing the action of the feedback system. Open and closed loop results showing the detection and calculation of feedback signals from bunch motion are presented and the system is shown to damp coupled-bunch instabilities in the ALS. Use of the system for accelerator diagnostics is illustrated via measurement of grow-damp transients which quantify growth rates without feedback, damping rates with feedback, and identify unstable modes

  3. [Amyloid goiter].

    Science.gov (United States)

    Hrívó, A; Péter, I; Bánkúti, B; Péley, G; Baska, F; Besznyák, I

    1999-03-21

    Amyloid goitre is at an extremely rare occurrence. Authors review the origin of disease and its symptoms, diagnostic and therapeutic tools. The disease may be due to either primary or secondary systemic or local amyloidosis. Diagnosis may be made even before surgery on anamnestic data, on very rapid growth of thyroid glands, on diffuse appearance, on other symptoms of systemic amyloidosis, on findings of iconographic procedures and on detection of amyloid in aspirates. Final diagnosis is based on histology. Surgical therapy is aiming at avoidance of the existing and the threatening consequences of expanding mass. The outcome is independent from thyroid surgery, it is related to other manifestations of amyloidosis. Concerning with the present case the chronic superior vena cava syndrome and chylous pleural effusion as first described symptoms and asymptomatic hyperthyroxinaemia is emphasised. Neither other organ involvement, nor primary amyloidogenous molecula was found during the 18 months follow up, so patient has secondary and localised amyloidosis.

  4. Thermodynamic evaluation of the Ti-Al-O ternary system

    International Nuclear Information System (INIS)

    Lee, B.-J.

    1997-01-01

    A thermodynamic evaluation of the Ti-Al-O ternary system has been made by using thermodynamic models for the Gibbs energy of individual phases. A consistent model parameter set was determined so that the calculation of isothermal sections and other thermodynamic quantities becomes possible. The agreement between calculation and corresponding experimental data was generally good along large temperature and composition range. (orig.)

  5. Microstructure evolution of Mo–Si–Al system during self-propagation high-temperature synthesis

    International Nuclear Information System (INIS)

    Jia, Lei; Xie, Hui; Lu, Zhen-lin; Zhang, Chao

    2013-01-01

    Highlights: ► Phase transformation subsequence of the reaction system was given by a sketch. ► Transformation of MoSi 2 to Mo(Si, Al) 2 phase was observed by XRD analysis. ► Variation of diffraction peaks was discussed by lattice parameters calculation. -- Abstract: The microstructure and phase constitution of Mo(Si 1−x , Al x ) 2 alloys (x = 0.03, 0.1 and 0.4) prepared by self-propagation high-temperature synthesis is first investigated using SEM, EDS and XRD analysis. Then the lattice parameters and adiabatic temperature are calculated. Based on the above experimental and calculated results, the variation mechanism of diffraction peaks and phase transformation subsequence of the Mo–MoO 3 –Si–Al powders is discussed. Results show that, when the self-propagation reaction is over, there are a homogeneous Mo–Si–Al alloy melt and a fused Al 2 O 3 with lower density at top. Subsequently, MoSi 2 or Mo(Si, Al) 2 phase nucleates and grows as a primary phase in the Mo–Si–Al alloy melts, and then Al, Si substances are generated from the intergranular residual Al–Si liquid according to Al–Si binary phase diagram. The Al increase in the starting powder mixtures leads to the Al concentration increase in the Mo–Si–Al alloy melt. Consequently, MoSi 2 is transformed to Mo(Si, Al) 2 to phase in which Si is replaced by Al atoms and Al substance in the intergranular zones increased accordingly

  6. Does the urinary protein pattern in AA-Amyloid nephropathy differ from that in other nephropathies?

    International Nuclear Information System (INIS)

    Teppo, A.M.; Maury, C.P.J.

    1986-01-01

    The urinary excretion of six plasma proteins was determined in reactive (secondary) amyloidosis, in rheumatoid arthritis, in systemic lupus erythematosus, in diabetic patients, in patients with chronic glomerulonephritis and in healthy controls. The type of proteinuria in patients with amyloidosis was compared with that of other patient groups and of nephropathies due to glomerulonephritis or diabetes. In amyloidosis the excretion of lambda light chains was slightly higher and that of kappa chains slightly lower than in other proteinurias, consequently the ratio lambda/kappa chains in patients with reactive amyloidosis was higher (p ≤ 0.01) than in other patient groups or in healthy controls. In patients with moderate/heavy proteinuria the excretion of IgG compared with that of albumin was in reactive amyloidosis as well as in diabetic nephropathy lower than in glomerulonephritis (p ≤ 0.05) and suggest the higher selectivity of protein excretion in these patients than in glomerulonephritis. The finding that the ratio of excreted lambda/kappa chains in reactive amyloidosis exceeds that of normal plasma indicates in these patients either increased plasma concentration and/or decreased reabsorption of lambda light chains

  7. The use of subcutaneous fat tissue for amyloid typing by enzyme-linked immunosorbent assay

    DEFF Research Database (Denmark)

    Olsen, K E; Sletten, K; Westermark, Per

    1999-01-01

    for typing the most common systemic amyloidoses of AL, AA, and transthyretin types by enzyme-linked immunosorbent assay (ELISA), using abdominal wall subcutaneous fat biopsy specimens. The method was tested on 21 abdominal fat biopsy specimens that were sent to the laboratory. Of these, 15 contained amyloid......The amyloidoses are biochemically heterogeneous diseases with pathophysiologic deposits of various proteins. The clinical course, prognosis, and therapy are different for each type of amyloidosis and, therefore, a type-specific diagnosis is demanded as early as possible. We describe a method...

  8. Microstructures and phase relationships in the Ti3Al + Nb system

    International Nuclear Information System (INIS)

    Kestner-Weykamp, H.T.; Kaufman, M.J.

    1989-01-01

    Alloys based on the α 2 -Ti 3 Al compound (hexagonal) DO 19 are currently experiencing limited use as advanced aerospace materials. To date, the alloys with the most desirable properties contain additions of β stabilizers, such as Nb, Mo and V, which promote the formation of a two-phase mixture of α 2 +β or α 2 +B2 (where B2 refers to the ordered CsCl structure). Unfortunately, the phase relationships in these systems have not been established in sufficient detail to allow their more widespread application. Recently, there has been a series of investigations aimed at alleviating this deficiency in the ternary Ti-Al-Nb system. These studies have clearly indicated the existence of the ordered B2 phase, which, in the higher Nb alloys, can be retained at room temperature by rapid cooling from the liquid or solid state. The authors describe (TiNb) 3 Al alloys (from 0 to 30 at. pct. Nb) were studies after conventional and nonequilibrium (i.e., rapid solidification) processing with an emphasis on providing further insight into the transformation sequences and phase equilibria in these alloys

  9. Investigation of as-cast alloys in the Pt-Al-Cr system

    International Nuclear Information System (INIS)

    Suess, R.; Cornish, L.A.; Witcomb, M.J.

    2010-01-01

    Platinum-based alloys are being developed which have microstructures that are analogous to the γ/γ' microstructure of the nickel-based superalloys. These Pt-based alloys have the potential to be used for high-temperature applications. The ternary Pt-Al-Cr system was investigated as part of the continued development of a thermodynamic database for the Pt-Al-Cr-Ru system. Scanning electron microscopy (SEM) with energy dispersive X-ray spectroscopy (EDX) and X-ray diffraction (XRD) analyses were used to obtain phase equilibria data. The alloys were studied in the as-cast condition. A solidification projection was constructed and a liquidus surface derived. It was concluded that all phase regions were identified correctly since the results were self-consistent. Three ternary phases and 21 ternary invariant reactions were identified.

  10. Lifetime-determining spalling mechanisms of NiCoCrAlRe/EB-PVD zirconia TBC systems

    Energy Technology Data Exchange (ETDEWEB)

    Fritscher, K.; Schulz, U. [DLR - German Aerospace Center, Institute of Materials Research, 51170 Cologne (Germany); Leyens, C. [Technical University of Brandenburg (BTU) Cottbus, Chair of Physical Metallurgy and Materials Technology, 03046 Cottbus (Germany)

    2007-09-15

    The mechanisms that control the lifetime of thermal barrier coating (TBC) systems have been traced by two particular overlay bondcoats serving as model systems: superalloy pins (IN100, CMSX-4) with two alternative NiCoCrAlRE (RE: Hf, Y) bond coat compositions (i) NiCoCrAlY without and (ii) with co-dopants of silicon and hafnium. On top an electron-beam physical-vapor deposited (EB-PVD) yttria partially stabilized zirconia (YPSZ) TBC commonly mixed with 2 wt.% hafnia, or, rarely with 10 wt.%, was applied. The test pins were thermo-cycled at 1100 and 1150 C until failure. Identical lifetimes in cyclic tests on YPSZ TBCs with 2 (relatively high sintering rate) and 10 wt.% hafnia (relatively low sintering rate) preclude an effect of diffusion mechanisms of the YPSZ TBC on lifetime. The fit of lifetimes and test temperatures to Arrhenius-type relationships gives activation energies for failure. These energies agree with the activation energies for anion and cation diffusion in alumina for the respective bondcoat variant: (i) for the NiCoCrAlY/TBC system for O{sup 2-} diffusion in alumina, (ii) for the NiCoCrAlYSiHf/TBC system for Al{sup 3+} diffusion in alumina. SEM and EDS investigations of the thermally grown oxides (TGOs) confirm the mechanisms responsible for TBC failure as indicated by activation energies. Two categories of failure can be distinguished: (i) NiCoCrAlY coatings fail by an ''adhesive mode of failure'' along smooth bond coat/TGO interfaces driven by a critical TGO thickness. (ii) NiCoCrAlYSiHf coatings fail later and more reluctantly by a ''cohesive'' crack mode via de-cohesion at the TGO/TBC interface. In the latter case a quasi-integrity of the crack-affected TGO is lengthily maintained up to failure by a crack-pinning mechanism which runs via Al{sup 3+} supply from the bondcoat. (Abstract Copyright [2007], Wiley Periodicals, Inc.) [German] Die Mechanismen, welche die Lebensdauer von Waermedaemmschichtsystemen

  11. Influence of nano-AlN particles on thermal conductivity, thermal stability and cure behavior of cycloaliphatic epoxy/trimethacrylate system

    Directory of Open Access Journals (Sweden)

    2011-02-01

    Full Text Available We have prepared a series of nano-sized aluminium nitride (nano-AlN/cycloaliphatic epoxy/trimethacrylate (TMPTMA systems and investigated their morphology, thermal conductivity, thermal stability and curing behavior. Experimental results show that the thermal conductivity of composites increases with the nano-AlN filler content, the maximum value is up to 0.47 W/(m.K. Incorporation of a small amount of the nano-AlN filler into the epoxy/TMPTMA system improves the thermal stability. For instance, the thermal degradation temperature at 5% weight loss of nano-AlN/epoxy/TMPTMA system with only 1 wt% nano-AlN was improved by ~8ºC over the neat epoxy/TMPTMA system. The effect of nano-AlN particles on the cure behavior of epoxy/TMPTMA systems was studied by dynamic differential scanning calorimetry. The results showed that the addition of silane treated nano-AlN particles does not change the curing reaction mechanism and silane treated nano-AlN particles could bring positive effect on the processing of composite since it needs shorter pre-cure time and lower pre-temperature, meanwhile the increase of glass transition temperature of the nanocomposite improves the heat resistance.

  12. The biodistribution and imaging of a novel amyloid probe 131I-serum amyloid P-component in mice model of amyloidosis

    International Nuclear Information System (INIS)

    Wei Hailiang; Zhang Chunli; Wang Rongfu; Zhang Jianhua; Yan Ping; Kang Lei; Guo Fengqin

    2011-01-01

    Objective: To validate 131 I-serum amyloid P-component (SAP) as a novel amyloid probe in mice bearing amyloidosis and evaluate its diagnostic value. Methods: Standard SAP was labeled with 131 I using Iodogen method. Amyloidotic mice model was established by subcutaneous injection of 0.5 ml 10% Casein daily for 21 d, and the control group was injected with 0.5 ml saline. Both groups were injected with 7.4 MBq 131 I-SAP through the tail veins and imaging was performed at 1, 3, 6, 24, 48 and 72 h post injection. In biodistribution study, 30 amyloidotic mice model and 30 controls were injected with 555 kBq 131 I-SAP and were killed evenly at 1, 3, 6, 24, 48 and 72 h post injection. T test was used to analysis the data. Results: The labeling efficiency of 131 I-SAP was 70.6%, and the radiochemical purity was ( 95.5 ±3.4)%. There was significant tracer uptake by liver, spleen and kidney at 24 h in the test group and mild uptake by these organs in the control group. The uptake ratios of liver, spleen and kidney over blood in the test group were 2.201 ±0.301, 2.139 ±0.223, 4.797 ±0.615, vs 0.657 ±0.126, 1.014 ±0.063, 0.607 ±0.028 in the control group,respectively (t=10.747, 11.626 and 15.135, all P<0.01). The uptake differences between the two groups were still statistically significant at 48 h (t=15.128, 4.558, 16.960, all P<0.01). The uptake ratios of spleen over blood between the two groups were not significantly different at 72 h (t=3.022, P>0.05), but the other two uptake ratios were both significantly different (t=7.801, 6.442, both P<0.01). Conclusions: SAP can be reliably labeled with 131 I. The labeled product 131 I-SAP can accumulate in amyloid laden tissues, thus rendering it a potential agent in the detection of amyloidosis. (authors)

  13. 26Al and 36Cl AMS system at University of Tsukuba: A progress report

    International Nuclear Information System (INIS)

    Sasa, Kimikazu; Nagashima, Yasuo; Takahashi, Tsutomu; Seki, Riki; Tosaki, Yuki; Sueki, Keisuke; Bessho, Kotaro; Matsumura, Hiroshi; Miura, Taichi; He, Ming

    2007-01-01

    With the current AMS system on the 12UD Pelletron tandem accelerator at University of Tsukuba, we are able to measure the long-lived radioisotopes of 14 C, 26 Al, 36 Cl and 129 I by employing a molecular pilot beam method that stabilizes the terminal voltage with 0.1% accuracy. Several major improvements have been made to increase the sensitivity of the AMS system to 26 Al and 36 Cl radioisotopes. For 26 Al-AMS, the ions extracted from the ion source have been changed from Al - to AlO - by using an Al 2 O 3 target sample. In addition, 26 MgO - molecular ions are used as a pilot beam to control the terminal voltage through slit current feedback. Also, a fully-stripping AMS technique is used to achieve an effective detection limit of better than 5 x 10 -15 for the 26 Al/ 27 Al ratio. For 36 Cl-AMS, the terminal voltage has been increased from 9 MV to 10 MV, thereby increasing the energy of the 36 Cl 9+ beam to 100 MeV. This energy increment helps us to get a clearer separation between 36 Cl and 36 S in a new ΔE-E detector. In addition, the yield of 36 Cl 9+ is increased by 1.5 times as a result of the higher ion energy at the terminal stripper foil. The standard deviation of the fluctuations of the 36 Cl/ 35 Cl ratio is within ±2%, and the effective detection limit is better than 2.0 x 10 -14

  14. Electrochemical formation of AlN in molten LiCl-KCl-Li{sub 3}N systems

    Energy Technology Data Exchange (ETDEWEB)

    Goto, Takuya [Department of Fundamental Energy Science, Graduate School of Energy Science, Kyoto University, Sakyo, Kyoto 606-8501 (Japan)]. E-mail: goto@energy.kyoto-u.ac.jp; Iwaki, Takayuki [Department of Fundamental Energy Science, Graduate School of Energy Science, Kyoto University, Sakyo, Kyoto 606-8501 (Japan); Ito, Yasuhiko [Department of Fundamental Energy Science, Graduate School of Energy Science, Kyoto University, Sakyo, Kyoto 606-8501 (Japan)

    2005-01-30

    Electrochemical formation of aluminum nitride was investigated in molten LiCl-KCl-Li{sub 3}N systems at 723 K. When Al was anodically polarized at 1.0 V (versus Li{sup +}/Li), oxidation of nitride ions proceeded to form adsorbed nitrogen atoms, which reacted with the surface to form AlN film. The obtained nitrided film had a thickness of sub-micron order. The obtained nitrided layer consisted of two regions; the outer layer involving AlN and aluminum oxynitride and the inner layer involving metallic Al and AlN. When Al electrode was anodically polarized at 2.0 V, anodic dissolution of Al electrode occurred to give aluminum ions, which reacted with nitride ions in the melt to produce AlN particles (1-5 {mu}m of diameter) of wurtzite structure.

  15. Tailoring ultrafine grained and dispersion-strengthened Ti 2 AlC/TiAl ...

    Indian Academy of Sciences (India)

    In situ Ti 2 AlC/TiAl composite was fabricated by hot-pressing method via the reaction system of Ti 3 AlC 2 and Ti-Al pre-alloyed powders at low temperature of 1150 ∘ C. The composite mainly consisted of TiAl, Ti 3 Al and Ti 2 AlC phases. Fine Ti 2 AlC particles were homogeneously distributed and dispersed in the matrix.

  16. Bone Marrow and Kidney Transplant for Patients With Chronic Kidney Disease and Blood Disorders

    Science.gov (United States)

    2017-03-21

    Chronic Kidney Disease; Acute Myeloid Leukemia (AML); Acute Lymphoblastic Leukemia (ALL); Chronic Myelogenous Leukemia (CML); Chronic Lymphocytic Leukemia (CLL); Non-Hodgkin's Lymphoma (NHL); Hodgkin Disease; Multiple Myeloma; Myelodysplastic Syndrome (MDS); Aplastic Anemia; AL Amyloidosis; Diamond Blackfan Anemia; Myelofibrosis; Myeloproliferative Disease; Sickle Cell Anemia; Autoimmune Diseases; Thalassemia

  17. Confocal Cornea Microscopy Detects Involvement of Corneal Nerve Fibers in a Patient with Light-Chain Amyloid Neuropathy Caused by Multiple Myeloma: A Case Report

    Directory of Open Access Journals (Sweden)

    Dietrich Sturm

    2016-06-01

    Full Text Available Changes in the subbasal corneal plexus detected by confocal cornea microscopy (CCM have been described for various types of neuropathy. An involvement of these nerves within light-chain (AL amyloid neuropathy (a rare cause of polyneuropathy has never been shown. Here, we report on a case of a patient suffering from neuropathy caused by AL amyloidosis and underlying multiple myeloma. Small-fiber damage was detected by CCM.

  18. High Density Hydrogen Storage System Demonstration Using NaAlH4 Based Complex Compound Hydrides

    Energy Technology Data Exchange (ETDEWEB)

    Daniel A. Mosher; Xia Tang; Ronald J. Brown; Sarah Arsenault; Salvatore Saitta; Bruce L. Laube; Robert H. Dold; Donald L. Anton

    2007-07-27

    This final report describes the motivations, activities and results of the hydrogen storage independent project "High Density Hydrogen Storage System Demonstration Using NaAlH4 Based Complex Compound Hydrides" performed by the United Technologies Research Center under the Department of Energy Hydrogen Program, contract # DE-FC36-02AL67610. The objectives of the project were to identify and address the key systems technologies associated with applying complex hydride materials, particularly ones which differ from those for conventional metal hydride based storage. This involved the design, fabrication and testing of two prototype systems based on the hydrogen storage material NaAlH4. Safety testing, catalysis studies, heat exchanger optimization, reaction kinetics modeling, thermochemical finite element analysis, powder densification development and material neutralization were elements included in the effort.

  19. Ternary nitrides for hydrogen storage: Li-B-N, Li-Al-N and Li-Ga-N systems

    International Nuclear Information System (INIS)

    Langmi, Henrietta W.; McGrady, G. Sean

    2008-01-01

    This paper reports an investigation of hydrogen storage performance of ternary nitrides based on lithium and the Group 13 elements boron, aluminum and gallium. These were prepared by ball milling Li 3 N together with the appropriate Group 13 nitride-BN, AlN or GaN. Powder X-ray diffraction of the products revealed that the ternary nitrides obtained are not the known Li 3 BN 2 , Li 3 AlN 2 and Li 3 GaN 2 phases. At 260 deg. C and 30 bar hydrogen pressure, the Li-Al-N ternary system initially absorbed 3.7 wt.% hydrogen, although this is not fully reversible. We observed, for the first time, hydrogen uptake by a pristine ternary nitride of Li and Al synthesized from the binary nitrides of the metals. While the Li-Ga-N ternary system also stored a significant amount of hydrogen, the storage capacity for the Li-B-N system was near zero. The hydrogenation reaction is believed to be similar to that of Li 3 N, and the enthalpies of hydrogen absorption for Li-Al-N and Li-Ga-N provide evidence that AlN and GaN, as well as the ball milling process, play a significant role in altering the thermodynamics of Li 3 N

  20. Wetting and interface interactions in the B4C/Al-Me (Me=Cu, Sn) systems

    International Nuclear Information System (INIS)

    Aizenshtein, M.; Froumin, N.; Dariel, M.P.; Frage, N.

    2008-01-01

    The wettability of B 4 C in contact with non-carbide and non-boride forming liquid metals (such as Cu or Sn) has been the subject of several studies. These metals do not wet boron carbide unless a reactive element is added to the melt. The present study is concerned with the addition of Al which completes the series of reactive elements added to the non-wetting metals. While Si represents the elements that form stable carbides and Ti represents the elements that form stable borides, Al belongs to the group of elements that form ternary borocarbides. The wetting experiments in the B 4 C/(Me-Al, Me=Cu, Sn) systems have shown that a ternary product, namely Al 8 B 4 C 7 was formed at the interface and that wetting is governed by the thermodynamic properties of the binary liquid system

  1. Liquid formation in the Y-Si-Al-O-N system

    Energy Technology Data Exchange (ETDEWEB)

    Redington Keely, W.; Redington, M.; Hampshire, S. [Limerick Univ. (Ireland). Materials Research Centre

    2000-07-01

    Some silicon nitride ceramics are sintered using a combination of Y{sub 2}O{sub 3} and Al{sub 2}O{sub 3} additives to form a Y-Si-Al-O-N liquid that promotes densification and allows the {alpha}{yields}{beta} transformation to take place by solution/precipitation through the liquid which cools as an intergranular phase. The type and amount of additive determines the nature and quantity of the resulting grain boundary phase as indicated and this can affect high temperature strength, creep resistance and oxidation resistance. Thus it is important to understand the phase equilibria in these complex systems and then apply this knowledge to processing, the development of beneficial microstructures and the relationship between these and properties. One difficulty is that, for any particular composition, the quantity and composition of the liquid at the sintering temperature is not known accurately and a full compositional representation has not been achieved. This paper attempts to represent the liquid region at various temperatures in the Jaenecke prism of the Y-Si-Al-O-N system. Published information on liquid compositions, standardised into eq.% format, was combined with experimental data interpolated from the less well defined regions to build a composite picture of liquid in this system. Representation was achieved using a customised graphical environment, AutoCAD trademark. AutoLISP trademark routines combined with this drafting package allow data from multiple sources to be integrated. This results in a series of overlays which indicate the liquid region from its initial formation at 1335 C and its subsequent development at different ''milestone'' temperatures. At 1700 C the orthographic projections of the liquid region are combined to give a volume bounded by nurbs surfaces, using ALIAS trademark software, to enable visualisation of the proposed liquid region. (orig.)

  2. Eutectic microstructures of Al-ThA13 and Al-UA14 alloys

    International Nuclear Information System (INIS)

    Vieira, R.R.; Ambrozio Filho, F.

    1990-01-01

    This paper deals on the microstructures of systems Al-ThAl3 and Al-UAL4, which are composed by a non faceted phase (aluminium) and an intermetallic phase (ThAl3 or UAL4) which is normally faceted phase. (C.M.)

  3. The Computerised Calculus in the Prognosis of the Phase Equilibrium Diagram of the Ternary System Al-Cu-Si

    Directory of Open Access Journals (Sweden)

    Florentina A. Cziple

    2006-10-01

    Full Text Available The paper presents a model for establishing the mathematical functions of the liquidus and solidus curves, from the binary diagrams Al-Si, Si-Cu, Cu-Al and their use in the prognosis of the phase equilibrium diagram from the ternary system Al-Cu-Si. We have studied the model of the non-ideal liquid solution of the regular type. The calculus and graphic plotting of the equations for the binary systems has been performed on the computer with the software programmes MathCad 2000 Professional, Statistica 5, Curve Expert, and for the ternary system Al-Cu-Si, with the 3D StudioMax software

  4. RASC-AL (Revolutionary Aerospace Systems Concepts-Academic Linkage): 2002 Advanced Concept Design Presentation

    Science.gov (United States)

    2002-01-01

    The Revolutionary Aerospace Systems Concepts-Academic Linkage (RASC-AL) is a program of the Lunar and Planetary Institute (LPI) in collaboration with the Universities Space Research Association's (USRA) ICASE institute through the NASA Langley Research Center. The RASC-AL key objectives are to develop relationships between universities and NASA that lead to opportunities for future NASA research and programs, and to develop aerospace systems concepts and technology requirements to enable future NASA missions. The program seeks to look decades into the future to explore new mission capabilities and discover what's possible. NASA seeks concepts and technologies that can make it possible to go anywhere, at anytime, safely, reliably, and affordably to accomplish strategic goals for science, exploration, and commercialization. University teams were invited to submit research topics from the following themes: Human and Robotic Space Exploration, Orbital Aggregation & Space Infrastructure Systems (OASIS), Zero-Emissions Aircraft, and Remote Sensing. RASC-AL is an outgrowth of the HEDS-UP (University Partners) Program sponsored by the LPI. HEDS-UP was a program of the Lunar and Planetary Institute designed to link universities with NASA's Human Exploration and Development of Space (HEDS) enterprise. The first RASC-AL Forum was held November 5-8, 2002, at the Hilton Cocoa Beach Oceanfront Hotel in Cocoa Beach, Florida. Representatives from 10 university teams presented student research design projects at this year's Forum. Each team contributed a written report and these reports are presented.

  5. Early accretion of protoplanets inferred from a reduced inner solar system 26Al inventory

    DEFF Research Database (Denmark)

    Schiller, Martin; Connelly, James; Gad, Aslaug C.

    2015-01-01

    value of 5.25×10−5. Based on their similar 54Cr/52Cr ratios, most inner solar system materials likely accreted from material containing a similar 26Al/27Al ratio as the APB precursor at the time of CAI formation. To satisfy the abundant evidence for widespread planetesimal differentiation...

  6. Calumenin interacts with serum amyloid P component

    DEFF Research Database (Denmark)

    Vorum, H; Jacobsen, Christian; Honoré, Bent

    2000-01-01

    We recently reported the identification of human calumenin, a novel Ca(2+) binding, transformation-sensitive and secreted protein [Vorum et al. (1998) Biochim. Biophys. Acta 1386, 121-131; Vorum et al. (1999) Exp. Cell Res. 248, 473-481] belonging to the family of multiple EF-hand proteins...... with calumenin in the presence of Ca(2+). Amino acid sequencing identified this protein as serum amyloid P component (SAP). Furthermore, we verified and characterized the calumenin-SAP interaction by the surface plasmon resonance technique. The findings indicate that calumenin may participate...... in the immunological defense system and could be involved in the pathological process of amyloidosis that leads to formation of amyloid deposits seen in different types of tissues. Udgivelsesdato: 2000-Jan-14...

  7. PEMAHAMAN HADITS TARBAWI BURHAN AL ISLAM AL ZARNUJI DALAM KITAB TA’LIM AL MUTA’ALLIM

    Directory of Open Access Journals (Sweden)

    Benny Afwadzi

    2017-01-01

    Full Text Available This paper explores the understanding of tarbawi (education hadith from one of the major influential clerics in the Islamic educational system, especially in pesantren. He is Burhan al Islam al Zarnuji with his landmark book Ta’lim al Muta’allim. The conclusion that can be drawn is that al Zarnuji has shades of sufistical view in understanding tarbawi hadith. This can be seen in his understanding of hadith on obligation to seek knowledge (thalab al ilmi faridhatun ‘ala kulli muslimin wa muslimatin, which is interpreted as ilm al hal, the science concerning the condition of the human religious or simplistically said as the religious sciences; and the hadith of intention of studying (innama al a’malu bi al niyyat with the intention for the sake of the hereafter and religion, not mundane. Such an understanding is not something taken for granted, but it can be reinterpreted and perfected in accordance with the spirit of the times of context.

  8. Thermodynamic calculation of Al-Gd and Al-Gd-Mg phase equilibria checked by key experiments

    International Nuclear Information System (INIS)

    Groebner, J.; Kevorkov, D.; Schmid-Fetzer, R.

    2001-01-01

    The binary Al-Gd and the ternary Al-Gd-Mg systems were calculated using the Calphad method. It is demonstrated that previous interpretation of ternary liquidus temperatures below 700 C must be related to other phase equilibria. The actual ternary liquidus temperatures are much higher, up to some 600 C above the previous interpretation in literature. They are widely governed by the high-melting compounds Al 2 Gd and Al 3 Gd with liquidus surfaces stretching far into the ternary system. A small number of key experiments in this work confirmed the calculated liquidus temperature and the phase relations. The available experimental data in literature fit excellently with the calculation in the binary Al-Gd system. In the ternary Al-Gd-Mg system, which is shown in several sections of the phase diagram, a good agreement can be observed too, considering the necessary reinterpretation of the liquidus temperatures suggested by Rokhlin et al. Ternary solubilities were not found experimentally. The ternary compound Al 4 GdMg (τ) forms in a ternary peritectic reaction at 761 C. (orig.)

  9. The evolution of interface microstructure in a ZrO2/Ag-Cu-Al-Ti system

    International Nuclear Information System (INIS)

    Lee, Youngmin; Yu, Jin

    1993-01-01

    Among ceramic/metal (C/M) joining technologies, the active filler metal method has been studied extensively due to the simple brazing process and excellent joint strength. Active metal elements, typically Ti, are intentionally added to braze alloys to enhance the formation of reaction products between the ceramic and the braze metal at the C/M interface. In the brazing of Al 2 O 3 with the Ag-Cu-Ti filler metal, reaction products such as γ-TiO, Cu 2 (Ti, Al) 4 O, Ti 3 (Cu 0.76 Al 0.18 Sn 0.06 ) 3 O were found, while products such as Ti 5 Si 3 and TiN formed in the brazing of Si 3 N 4 . The presence of reaction layers at the C/M interface influences the interface strength in a complex way. In Cu/Al 2 O 3 , Co/Al 2 O 3 , Ni/Al 2 O 3 , and Cu/diamond systems, maxima of joint strength were observed at some intermediate Ti addition, while the flexural strength decreased substantially with the thickening of the TiO layer in a ZrO 2 /Ag-Cu-Sn-Ti system. Thus, composition of the braze alloy (particularly, the content of the active metal), process conditions such as brazing temperature and time, microstructure and mechanical properties of reaction products at the C/M interfaces, interfacial chemistry, and residual stress are primary factors to be studied in order to understand the strengths of the C/M interfaces systematically. In the present and the following papers, evolutions of interfacial microstructures at various brazing conditions, and corresponding interface strengths are reported, respectively, for a ZrO 2 /Ag-Cu-Al-Ti system

  10. Fluctuations of charge variance and interaction time for dissipative processes in 27 Al + 27 Al collision

    International Nuclear Information System (INIS)

    Berceanu, I.; Andronic, A.; Duma, M.

    1999-01-01

    The systematic studies of dissipative processes in light systems were completed with experiments dedicated to the measurement of the excitation functions in 19 F + 27 Al and 27 Al + 27 Al systems in order to obtain deeper insight on DNS configuration and its time evolution. The excitation function for 19 F + 27 Al system evidenced fluctuations larger than the statistical errors. Large Z and angular cross correlation coefficients supported their non-statistical nature. The energy dependence of second order observables, namely the second moment of the charge distribution and the product ω·τ (ω - the angular velocity of the DNS and τ its mean lifetime) extracted from the angular distributions were studied for 19 F + 27 Al case. In this contribution we are reporting the preliminary results of similar studies performed for 27 Al + 27 Al case. The variance of the charge distribution were obtained fitting the experimental charge distribution with a Gaussian centered on Z = 13 and the product ω·τ was extracted from the angular distributions. The results for 19 F + 27 Al case are confirmed by a preliminary analysis of the data for 27 Al + 27 Al system. The charge variance and ω·τ excitation functions for Z = 11 fragment are represented together with the excitation function of the cross section. One has to mention that the data for 27 Al + 27 Al system were not corrected for particle evaporation processes. The effect of the evaporation corrections on the excitation function was studied using a Monte Carlo simulation. The α particle evaporation was also included and the evaluation of the particle separation energies was made using experimental masses of the fragments. The excitation functions for 27 Al + 27 Al system for primary and secondary fragments were simulated. No structure due to particle evaporation was observed. The correlated fluctuations in σ Z and ω·τ excitation functions support a stochastic exchange of nucleons as the main mechanism for

  11. Fabrication of Ni-Al/diamond composite based on layered and gradient structures of SHS system

    Directory of Open Access Journals (Sweden)

    Lu Jiafeng

    2017-01-01

    Full Text Available In this paper layered and gradient structures of Ni-Al SHS system were adopted to manufacture Ni-Al/diamond composites. The effect of the layered and the diamond mesh gradient structures of Ni-Al/diamond on the SHS process and the microstructure of the composites were investigated. It is found that with the increasing of the number of layers, the combustion wave velocity is decreased. The combustion wave velocity for diamond mesh size gradient structure of Ni-Al SHS is faster than that for the layered structure. A well bonding can be formed between diamond and the matrix in layered and gradient structure Ni-Al/diamond composites due to the melt of Ni-Cr brazing alloy.

  12. Mechanochemical transformations in Li(Na)AlH4-Li(Na)NH2 systems

    International Nuclear Information System (INIS)

    Dolotko, Oleksandr; Zhang Haiqiao; Ugurlu, Ozan; Wiench, Jerzy W.; Pruski, Marek; Scott Chumbley, L.; Pecharsky, Vitalij

    2007-01-01

    Mechanochemical transformations of tetrahydroaluminates and amides of lithium and sodium have been investigated using gas volumetric analysis, X-ray powder diffraction, solid-state nuclear magnetic resonance (NMR) and transmission electron microscopy. In a transformation of LiAlH 4 and LiNH 2 taken in an 1:1 molar ratio, the amount of released hydrogen (6.6 wt.% after 30 min ball milling) was higher than in any known one pot mechanochemical process involving a hydrogen-containing solid. A total of 4.3 wt.% of hydrogen is released by the NaAlH 4 -NaNH 2 system after 60 min ball milling; and 5.2 wt.% H 2 is released when LiAlH 4 and NaNH 2 or NaAlH 4 and LiNH 2 are ball milled for 90 min and 120 min, respectively. All transformations proceed at room temperature. The mechanism of the overall transformation MAlH 4 (s) + MNH 2 (s) → 2MH(s) + AlN(s) + 2H 2 (g) was identified based on detailed spectroscopic analysis of the intermediate (M 3 AlH 6 ) and final products of the ball milling process

  13. Malamih al-Ashwat al-'Arabiyyah wa Makharijaha

    Directory of Open Access Journals (Sweden)

    Amrah Muhammad Kasim

    2015-12-01

    Full Text Available This writing discusses on al-ashwat al-‘arabiyyah that is known with “phonetic” term. It talks and interprets makharij al-huruf or place for producing sounds and also processing production of sounds by parts of mouth in Arabic language. The problems of sounds and its systems in Arabic language have been conducted by the scholars of al-ashwat al-‘arabiyyah attractively, neatly, and detail and gotten ahead the phonetic western scholars. Part of their analysis results formulate the study about al-ashwat al-sakinah (al-Shamit is known with vocal and consonant sounds in research questions shawait – shawamit. Ibnu Jinni has formulated “harakat”(wowel in Arabic language and dimension of letter  mad and liyn on alif  letter - waw - ya’ (ا و ي, from these letters create  harakat fathah, dammah, and kasrah. Moreover, in this writing also exist the description of hija’i letters and its makhraj that are correct from parts of mouth and the unity of coordinative sounds that produce sounds in morphemes arrangement, in one word that produces meaning. The result of phonological study has divided al-ashwat to be kinds of sound like the pronounced sounds, produced sounds from mouth, and heard sound.

  14. Out of pile French research program on the UMo/Al system: First results

    International Nuclear Information System (INIS)

    Dubois, S.; Mazaudier, F.; Piron, J.P.; Martin, P.; Dumas, J.C.; Huet, F.; Noel, H.; Tougait, O.; Jarousse, C.; Lemoine, P.

    2005-01-01

    This paper focuses on a French scientific program built to gain further knowledge on the UMo/Al interaction and to discuss promising solutions to prevent this interaction under neutron flux. Three themes are thus developed. Thermodynamic investigations, through UMo-Al ternary system studies, may lead to determining the phase equilibria at different temperatures. Metallurgical out-of-pile diffusion couple studies are intended to improve the understanding of diffusion mechanisms and should underline the role of different parameters, which could prevent or decrease the UMo/Al reaction. Finally, the irradiation effect has been taken into account, either through a simulation program or an in-pile one. The results already achieved are described and discussed. (author)

  15. First observations of nuclear rainbow scattering in the 16O+27Al system

    International Nuclear Information System (INIS)

    Pereira, D.; Oliveira, J.R.B.; Chamon, L.C.; Cunsolo, A.; Cappuzzello, F.; Cavallaro, M.; Carbone, D.

    2011-01-01

    Nuclear rainbow structures have been unexpectedly predicted in the elastic channel of heavy nuclear systems like 16 O + 27 Al or 58 Ni by a new generation of coupled channel calculations, with inclusion of an imaginary potential for dissipative processes. Rainbow scattering phenomena are very sensitive to the nuclear potential in the surface interior and therefore very important to test different microscopic ion-ion potentials, as mentioned in, as well as the role of reaction couplings in the elastic channel far from the barrier region. The rainbow pattern has been experimentally well established for lighter systems like alpha-nucleus, 12 C + 12 C, 16 O + 12 C, and 16 O + 16 O - the heaviest heavy-ion system so far that has shown a prominent rainbow structure. By using the large acceptance magnetic spectrometer MAGNEX (LNS, Catania, Italy) in a high-sensitivity and high-precision experiment, we have confirmed some of the predictions cited above, for the 16 O + 27 Al system, at 100 MeV beam energy, up to about 80 degrees in the center of mass. An extremely good resolution (around 90 keV) was achieved, allowing for clear separation of the elastic channel from the first excited state of 27 Al (843.8 keV). Cross sections below 10 -4 mb were measured. Additional experiments are scheduled to April, 2011, in order to explore the backward-angle region. In this presentation, the comparison between the rainbow structures detected in this system and those in lighter systems and with weakly bound nuclei will be discussed, in connection with the predicted Airy minima. (author)

  16. Percolative ionic conduction in the LiAlSiO4 glass-ceramic system

    International Nuclear Information System (INIS)

    Biefeld, R.M.; Pike, G.E.; Johnson, R.T. Jr.

    1977-01-01

    The effect f crystallinity on the lithium ion conductivity in LiAlSiO 4 glass and glass-ceramic solid electrolytes has been determined. The ionic conductivity is thermally activated with an activation energy and pre-exponential factor that change in a marked and nonsimple manner as the volume fraction of crystallinity changes. These results are explained by using a continuum percolation model (effective-medium approximation) which assumes that ionic conduction in the glass-ceramic is almost entirely within the glass phase until the crystalline volume fraction rises above approx. 55%. The LiAlSiO 4 system would seem to be nearly ideal for application of percolation theory since the crystalline phase, β eucryptite, has nearly the same composition as the glass phase. Hence, as the crystallite volume fraction increases in the glass ceramic, the residual glass composition and conductivity remain the same. This is the first application of percolation theory to ionic transport in glass-ceramics and excellent agreement is obtained between theory and experiment for the LiAlSiO 4 system

  17. Formation and microstructure of Al{sub 2}O{sub 3}-YAG eutectic ceramics by phase transformation from metastable system to equilibrium system

    Energy Technology Data Exchange (ETDEWEB)

    Nagira, Tomoya; Yasuda, Hideyuki; Yoshiya, Masato [Department of Adaptive Machine Systems, Osaka University, 2-1 Yamadaoka, Suita, Osaka 565-0871 (Japan)], E-mail: nagira@ams.eng.osaka-u.ac.jp

    2009-05-01

    Unidirectionally solidified Al{sub 2}O{sub 3}-YAG(Y{sub 3}Al{sub 5}O{sub 12}: yttrium-aluminum-garnet) eutectic ceramic composites have been recognized as encouraging heat-resistance materials because of the superior mechanical properties at high temperatures. In addition to the excellent mechanical properties at high temperatures, some interesting solidification phenomena have been reported in the Al{sub 2}O{sub 3}-Y{sub 2}O{sub 3} system. The Al{sub 2}O{sub 3}-YAG equilibrium eutectic at 2099 K and the Al{sub 2}O{sub 3}-YAP metastable eutectic at 1975 K exist in the Al{sub 2}O{sub 3}-Y{sub 2}O{sub 3} system. The heating the metastable eutectic up to temperatures above the metastable eutectic temperature produced the undercooled melt. Solidification in the equilibrium path accompanied the melting of the metastable eutectic. The solidification process using undercooled melt resulted in the fine and uniform eutectic structure. In this study, the effect of the initial Al{sub 2}O{sub 3}-YAP particles size on the undercooled melt formation was examined. The Al{sub 2}O{sub 3}-YAP particles with diameters more than several {mu}m resulted in the transformation through the undercooled melt. EBSD analysis showed that the domains of Al{sub 2}O{sub 3} grains with same crystallographic orientation were observed and that their domain size depended on the Al{sub 2}O{sub 3}-YAP particles size. On the other hand, for the Al{sub 2}O{sub 3}-YAP particles with a diameter of 500 nm, the each Al{sub 2}O{sub 3} grain with diameter of about 1 {mu}m had the different crystallographic orientations, which suggested that the transformation from metastable eutectic to equilibrium eutectic occurred in the solid state. The increase in the Al{sub 2}O{sub 3}-YAP free surface area suppressed the undercooled melt formation.

  18. Investigation of the phase relations in the U-Al-Ge ternary system: Influence of the Al/Ge substitution on the properties of the intermediate phases

    Energy Technology Data Exchange (ETDEWEB)

    Moussa, C.; El Sayah, Z. [Institut des Sciences Chimiques de Rennes, Chimie du Solide et Matériaux, UMR CNRS 6226, Université Rennes 1, Campus de Beaulieu, 35042 Rennes Cedex (France); Chajewski, G. [Institute of Low Temperature and Structure Research, Polish Academy of Sciences, ul. Okólna 2, 50-422 Wrocław (Poland); Berche, A.; Dorcet, V. [Institut des Sciences Chimiques de Rennes, Chimie du Solide et Matériaux, UMR CNRS 6226, Université Rennes 1, Campus de Beaulieu, 35042 Rennes Cedex (France); Pikul, A.P. [Institute of Low Temperature and Structure Research, Polish Academy of Sciences, ul. Okólna 2, 50-422 Wrocław (Poland); Pasturel, M. [Institut des Sciences Chimiques de Rennes, Chimie du Solide et Matériaux, UMR CNRS 6226, Université Rennes 1, Campus de Beaulieu, 35042 Rennes Cedex (France); Joanny, L. [ScanMAT – CMEBA, University of Rennes 1, Campus de Beaulieu, 35042 Rennes Cedex (France); Stepnik, B. [AREVA/CERCA, 10 Rue Juliette Récamier, 69006 Lyon (France); Tougait, O., E-mail: tougait@univ-rennes1.fr [Institut des Sciences Chimiques de Rennes, Chimie du Solide et Matériaux, UMR CNRS 6226, Université Rennes 1, Campus de Beaulieu, 35042 Rennes Cedex (France); Unité de Catalyse et de Chimie du Solide, UMR CNRS 8181, Université de Lille, 59695 Villeneuve d' Ascq (France)

    2016-11-15

    The phase relations within the U-Al-Ge ternary system were studied for two isothermal sections, at 673 K for the whole Gibbs triangle and at 1173 K for the concentration range 25–100 at% U. The identification of the phases, their composition ranges and stability were determined by x-ray powder diffraction, scanning electron microscopy coupled to energy dispersive spectroscopy and differential thermal analysis. The tie-lines and the solubility domains were determined for the U-Ge and U-Al binaries, the UAl{sub 3}-UGe{sub 3} solid-solution and for the unique ternary intermediate phase U{sub 3}Al{sub 2−x}Ge{sub 3+x}. The experimental isopleth section of the pseudo-binary UAl{sub 3}-UGe{sub 3} reveals an isomorphous solid solution based on the Cu{sub 3}Au-type below the solidus. The U{sub 3}Al{sub 2−x}Ge{sub 3+x} solid solution extends for −0.1≤x≤1.35 and −0.2≤x≤1.5 at 673 K and 1173 K respectively. It crystallizes in the I-centered tetragonal symmetry. The reciprocal lattice of several compositions of the U{sub 3}Al{sub 2−x}Ge{sub 3+x} solid solution was examined by electron diffraction at room temperature, revealing the presence of a c-glide plane. Their crystal structure was refined by single crystal x-ray diffraction suggesting an isomorphous solid solution best described with the non-centrosymmetric space group I4cm in the paramagnetic domain. The magnetic measurements confirm the ferromagnetic ordering of the solid solution U{sub 3}Al{sub 2−x}Ge{sub 3+x} with an increase of Tc with the Al content. The thermal variation of the specific heat bear out the magnetic transitions with some delocalized character of the uranium 5f electrons. - Graphical abstract: The phase relations within the U-Al-Ge ternary system were experimentally assessed for two isothermal sections, at 673 K for the whole Gibbs triangle and at 1173 K for the concentration range 25–100 at% U. A complete UAl{sub 3}-UGe{sub 3} solid-solution based on the Cu{sub 3}Au-type forms

  19. The formation of quasicrystal phase in Al-Cu-Fe system by mechanical alloying

    OpenAIRE

    Travessa, Dilermando Nagle; Cardoso, Kátia Regina; Wolf, Witor; Jorge Junior, Alberto Moreira; Botta, Walter José

    2012-01-01

    In order to obtain quasicrystalline (QC) phase by mechanical alloying (MA) in the Al-Cu-Fe system, mixtures of elementary Al, Cu and Fe in the proportion of 65-20-15 (at. %) were produced by high energy ball milling (HEBM). A very high energy type mill (spex) and short milling times (up to 5 hours) were employed. The resulting powders were characterized by X-ray diffraction (XRD), differential scanning calorimetry (DSC) and scanning electron microscopy (SEM). QC phase was not directly formed ...

  20. Proteinuria in children with juvenile idiopathic arthritis: Making the case for early urinary screening

    Directory of Open Access Journals (Sweden)

    Anshuman Saha

    2017-01-01

    Full Text Available Systemic onset juvenile idiopathic arthritis (SOJIA can be associated with proteinuria due to various renal pathologies. We report two pediatric cases with SOJIA and nephrotic syndrome secondary to renal amyloidosis, a very rare complication in children. Once present, amyloidosis heralds a poor prognosis for the patient, though early detection may allow some improvement if the inflammatory arthritis is controlled.

  1. High temperature mechanical behaviour of glass-ceramics in the YSiAlON and ErSiAlON systems

    Energy Technology Data Exchange (ETDEWEB)

    Bondanini, A.; Massouras, G.; Besson, J.L. [ENSCI, Limoges (France). SPCTS

    2002-07-01

    The high temperature mechanical behaviour of oxynitride glass-ceramics in the YSiAlON and ErSiAlON systems was studied in the 950-1150 C temperature range under compressive stresses ranging from 20 to 100 MPa. The parent glass had a composition of 35 Y(or Er)-45 Si-20 Al-83 O-17 N in equivalent percent. Starting from these glasses, glass-ceramics were prepared using a two stage heat treatment: nucleation at the optimum nucleation temperature followed by crystal growth at 1050, 1150 or 1250 C. The two parent glasses had similar viscosities, with that of the Er-glass being slightly less than that of the Y-glass. After the devitrification treatment at 1050 C, B-phase (M{sub 2}SiAlO{sub 5}N) was the only crystalline phase formed in both systems. The creep behaviour was similar for the yttrium and the erbium materials. It was characterised by a long transient stage, due to the viscoelastic response of the residual glass, with recovered strain after unloading decreasing as loading time increased. The creep resistance was compared to that of the parent glasses in terms of apparent viscosity. The crystallisation of 75% of the glass resulted in an increase in viscosity such that a temperature some 100 C higher showed the same viscosity value. After heat treatment at 1150 C, the phase assemblage in the yttrium material changed with the formation of wollastonite and partial conversion of B-phase into Iw-phase. The apparent viscosity was 2 orders of magnitude higher than that of the samples heat treated at 1050 C and no strain recovery was observed upon unloading. In contrast, the erbium materials retained the same microstructure as after the heat treatment at 1050{sup b}C and there was no difference in the creep behaviour of the samples heat treated at 1050 or 1150 C. After a crystallisation treatment at 1250 C of the yttrium parent glass, the glass-ceramic consisted of yttrium aluminium garnet, N-apatite and {beta}-Y{sub 2}Si{sub 2}O{sub 7} and showed excellent creep

  2. Particle Based Alloying by Accumulative Roll Bonding in the System Al-Cu

    Directory of Open Access Journals (Sweden)

    Mathias Göken

    2011-11-01

    Full Text Available The formation of alloys by particle reinforcement during accumulative roll bonding (ARB, and subsequent annealing, is introduced on the basis of the binary alloy system Al-Cu, where strength and electrical conductivity are examined in different microstructural states. An ultimate tensile strength (UTS of 430 MPa for Al with 1.4 vol.% Cu was reached after three ARB cycles, which almost equals UTS of the commercially available Al-Cu alloy AA2017A with a similar copper content. Regarding electrical conductivity, the UFG structure had no significant influence. Alloying of aluminum with copper leads to a linear decrease in conductivity of 0.78 µΩ∙cm/at.% following the Nordheim rule. On the copper-rich side, alloying with aluminum leads to a slight strengthening, but drastically reduces conductivity. A linear decrease of electrical conductivity of 1.19 µΩ∙cm/at.% was obtained.

  3. Al-doped SnO2 nanocrystals from hydrothermal systems

    International Nuclear Information System (INIS)

    Jin Haiying; Xu Yaohua; Pang Guangsheng; Dong Wenjun; Wan Qiang; Sun Yan; Feng Shouhua

    2004-01-01

    Nanoparticles of Al-doped SnO 2 have been hydrothermally synthesized. The influences of the hydrothermal reaction time, the molar ratio of Sn/Al as well as the pH value of the solution have been studied. During the hydrothermal synthesis, the particle's core is rich in Sn and the surface is rich in Al. The Al-rich surface prevents the particles from further growing up either in the hydrothermal condition or during the calcination at 600 deg. C for a short period of time. The optimal hydrothermal synthesis condition of the nanoparticles is pH 5, Sn/Al=4:1 and 12 h at 160 deg. C. The products have been studied by XRD, TEM and 27 Al solid-state NMR

  4. The experimental investigation of phase equilibria in the Al-rich corner within the ternary Al–Mn–Be system

    Energy Technology Data Exchange (ETDEWEB)

    Zupanič, Franc, E-mail: franc.zupanic@um.si [University of Maribor, Faculty of Mechanical Engineering, Smetanova ulica 17, SI-2000 Maribor (Slovenia); Markoli, Boštjan; Naglič, Iztok [University of Ljubljana, Faculty of Natural Sciences and Technologies, Askerceva 12, Ljubljana SI-1000 (Slovenia); Bončina, Tonica [University of Maribor, Faculty of Mechanical Engineering, Smetanova ulica 17, SI-2000 Maribor (Slovenia)

    2013-09-05

    Highlights: •We investigated the constitution of the Al-rich corner of the Al–Mn–Be system. •Be{sub 4}AlMn is a thermodynamically stable phase in the Al-corner of the Al–Mn–Be system. •The T-phase (Al{sub 15}Mn{sub 3}Be{sub 2}) is not a stable phase in the Al-corner at 600 °C. •The λ-Al{sub 4}Mn phase is a stable phase in the Al-corner at 600 °C. •T-phase is a stable phase at 750 °C. -- Abstract: This work investigated the constitution of the Al-rich corner within the ternary Al–Mn–Be phase diagram using SEM + EDS, AES, XRD and DSC. With respect to the results, an isothermal cross-section at 600 °C was established, as well as a prediction of the apparent liquidus projection in the Al-corner. Be{sub 4}AlMn is a thermodynamically stable phase in the Al-rich corner of the ternary phase diagram. The other ternary T-phase, usually designated as Al{sub 15}Mn{sub 3}Be{sub 2}, formed during solidification in alloys with Be:Mn atomic ratios of less than 4:1, and having more than 1.5 at.% Mn. This phase is not a stable phase in the Al-rich corner at 600 °C. In contrast, the λ-Al{sub 4}Mn phase is a stable one. The T-phase is stable over a rather large part of the phase diagram at least within a temperature range close to 750 °C, where it is in equilibrium with the Al-rich liquid phase, and Be{sub 4}AlMn.

  5. In situ synthesis of Ti{sub 2}AlC–Al{sub 2}O{sub 3}/TiAl composite by vacuum sintering mechanically alloyed TiAl powder coated with CNTs

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Jian [Department of Materials Science and Engineering of Tianjin University, Tianjin Key Laboratory of Composite and Functional Materials, Tianjin 300072 (China); Zhao, Naiqin, E-mail: nqzhao@tju.edu.cn [State Key Laboratory of Hydraulic Engineering Simulation and Safety, Tianjin (China); Department of Materials Science and Engineering of Tianjin University, Tianjin Key Laboratory of Composite and Functional Materials, Tianjin 300072 (China); Nash, Philip [Thermal Processing Technology Center, Illinois Institute of Technology, IL (United States); Liu, Enzuo; He, Chunnian; Shi, Chunsheng; Li, Jiajun [Department of Materials Science and Engineering of Tianjin University, Tianjin Key Laboratory of Composite and Functional Materials, Tianjin 300072 (China)

    2013-11-25

    Highlights: •Using zwitterionic surfactant to enhance the dispersion of the CNTs on the powder surface. •CNTs as carbon source decreased the formation temperature of Ti{sub 2}AlC. •Al{sub 2}O{sub 3} was generated in situ from the oxygen atoms introduced in the drying procedure. •Nanosized Ti{sub 3}Al was precipitated at 1250 °C and distribute in the TiAl matrix homogeneously. •Ti{sub 2}AlC–Al{sub 2}O{sub 3}/TiAl composite was synthesized in situ by sintering pre-alloy Ti–Al coated with CNTs. -- Abstract: Bulk Ti{sub 2}AlC–Al{sub 2}O{sub 3}/TiAl composites were in situ synthesized by vacuum sintering mechanically alloyed Ti–50 at.% Al powders coated with carbon nanotubes (CNTs). The pre-alloyed Ti–50 at.% Al powder was obtained by ball milling Ti and Al powders. The multi-walled carbon nanotubes as the carbon resource were covered on the surface of the pre-alloyed powders by immersing them into a water solution containing the CNTs. A zwitterionic surfactant was used to enhance the dispersion of the CNTs on the powder surface. The samples were cold pressed and sintered in vacuum at temperatures from 950 to 1250 °C, respectively. The results show that the reaction of forming Ti{sub 2}AlC can be achieved below 950 °C, which is 150 °C lower than in the Ti–Al–TiC system and 250 °C lower than for the Ti–Al–C system due to the addition of CNTs. Additionally, the reinforcement of Al{sub 2}O{sub 3} particles was introduced in situ in Ti{sub 2}AlC/TiAl by the drying process and subsequent sintering of the composite powders. Dense Ti{sub 2}AlC–Al{sub 2}O{sub 3}/TiAl composites were obtained by sintering at 1250 °C and exhibited a homogeneous distribution of Ti{sub 2}AlC, Al{sub 2}O{sub 3} and precipitated Ti{sub 3}Al particles and a resulting high hardness.

  6. A Comparative Study of Carbon Nanotubes Synthesized from Co/Zn/Al and Fe/Ni/Al Catalyst

    Directory of Open Access Journals (Sweden)

    Ezekiel Dixon Dikio

    2011-01-01

    Full Text Available The catalyst systems Fe/Ni/Al and Co/Zn/Al were synthesized and used in the synthesis of carbon nanotubes. The carbon nanotubes produced were characterized by Field Emission Scanning Electron Microscope (FE-SEM, Energy Dispersive x-ray Spectroscopy (EDS, Raman spectroscopy, Thermogravimetric Analysis (TGA and Transmission Electron Microscope (TEM. A comparison of the morphological profile of the carbon nanotubes produced from these catalysts indicates the catalyst system Fe/Ni/Al to have produced higher quality carbon nanotubes than the catalyst system Co/Zn/Al.

  7. Modern aluminium alloys for aeronautical applications. The role of the Al-Li system

    International Nuclear Information System (INIS)

    Godefroid, L.; Bernardes, A.T.; Bastian, F.L.

    1995-01-01

    A great effort has been made for some Aluminum Industries since the ''80 to develop new alloys with weight reduction, to use in aeronautic industry. The system Al-Li is a good example of this effort, because it presents a low density and a greater stiffness than usual alloys. In this paper we compare some Al-Li alloys with respect to mechanical properties: fracture toughness and resistance to crack propagation on fatigue case. The results are discussed, as well as the nowadays status and perspectives for the use of these alloys. (author)

  8. Ultrasound assisted combustion synthesis of TiC in Al-Ti-C system.

    Science.gov (United States)

    Liu, Zhiwei; Rakita, Milan; Xu, Wilson; Wang, Xiaoming; Han, Qingyou

    2015-11-01

    This research investigated the effects of high-intensity ultrasound on the combustion synthesis of TiC particles in Al-Ti-C system. The process involved that high-intensity ultrasound was applied on the surface of a compacted Al-Ti-C pellet directly through a Nb probe during the thermal explosion reaction. By comparing with the sample without ultrasonic treatment, it was found that the thermal explosion reaction for synthesizing TiC phase could take place thoroughly in the ultrasonically treated sample. During the process of synthesizing TiC phase, the dissolution of solid graphite particles into the Al-Ti melt, as well as the nucleation and growth of TiC particles could be promoted effectively due to the effects of ultrasound, leading to an enhancement of the formation of TiC particles. Ultrasound assisted combustion synthesis as a simple and effective approach was proposed for synthesizing materials in this research. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. Tunnel and electrostatic coupling in graphene-LaAlO3/SrTiO3 hybrid systems

    Directory of Open Access Journals (Sweden)

    I. Aliaj

    2016-06-01

    Full Text Available We report on the transport properties of hybrid devices obtained by depositing graphene on a LaAlO3/SrTiO3 oxide junction hosting a 4 nm-deep 2-dimensional electron system. At low graphene-oxide inter-layer bias, the two electron systems are electrically isolated, despite their small spatial separation. A very efficient reciprocal gating of the two neighboring 2-dimensional systems is shown. A pronounced rectifying behavior is observed for larger bias values and ascribed to the interplay between electrostatic field-effects and tunneling across the LaAlO3 barrier. The relevance of these results in the context of strongly coupled bilayer systems is discussed.

  10. Multiregional Age-Associated Reduction of Brain Neuronal Reserve Without Association With Neurofibrillary Degeneration or β-Amyloidosis.

    Science.gov (United States)

    Wegiel, Jerzy; Flory, Michael; Kuchna, Izabela; Nowicki, Krzysztof; Yong Ma, Shuang; Wegiel, Jarek; Badmaev, Eulalia; Silverman, Wayne P; de Leon, Mony; Reisberg, Barry; Wisniewski, Thomas

    2017-06-01

    Increase in human life expectancy has resulted in the rapid growth of the elderly population with minimal or no intellectual deterioration. The aim of this stereological study of 10 structures and 5 subdivisions with and without neurofibrillary degeneration in the brains of 28 individuals 25-102-years-old was to establish the pattern of age-associated neurodegeneration and neuronal loss in the brains of nondemented adults and elderly. The study revealed the absence of significant neuronal loss in 7 regions and topographically selective reduction of neuronal reserve over 77 years in 8 brain structures including the entorhinal cortex (EC) (-33.3%), the second layer of the EC (-54%), cornu Ammonis sector 1 (CA1) (-28.5%), amygdala, (-45.8%), thalamus (-40.5%), caudate nucleus (-35%), Purkinje cells (-48.3%), and neurons in the dentate nucleus (40.1%). A similar rate of neuronal loss in adults and elderly, without signs of accelerating neuronal loss in agers or super-agers, appears to indicate age-associated brain remodeling with significant reduction of neuronal reserve in 8 brain regions. Multivariate analysis demonstrates the absence of a significant association between neuronal loss and the severity of neurofibrillary degeneration and β-amyloidosis, and a similar rate of age-associated neuronal loss in structures with and without neurofibrillary degeneration. © 2017 American Association of Neuropathologists, Inc. All rights reserved.

  11. The Gd-Co-Al system at 870/1070 K as a representative of the rare earth-Co-Al family and new rare-earth cobalt aluminides: Crystal structure and magnetic properties

    Science.gov (United States)

    Morozkin, A. V.; Garshev, A. V.; Knotko, A. V.; Yapaskurt, V. O.; Mozharivskyj, Y.; Yuan, Fang; Yao, Jinlei; Nirmala, R.; Quezado, S.; Malik, S. K.

    2018-05-01

    The Gd-Co-Al system has been investigated at 870/1070 K by X-ray and elemental EDS analyses. The existence of the known compounds Gd2Co3Al9 (Y2Co3Ga9-type), Gd3Co4.5Al11.5 (Gd3Co4.6Al11) (Gd3Ru4Al12-type), Gd3Co6-7.4Al3-1.6 (CeNi3-type), GdCo1.15-0.65Al0.85-1.35 (MgZn2-type), Gd2Co2Al (Mo2NiB2-type) and Gd3Co3.5-3.25Al0.5-0.75 (W3CoB3-type) has been confirmed at 870/1070 K. Structure types have been determined for Gd2Co6Al19 (U2Co6Al19-type), Gd7Co6Al7 (Pr7Co6Al7-type), Gd6Co2-2.21Al1-0.79 (Ho6Co2Ga-type) and Gd14Co3.2Al2.8 (Gd14Co2.58Al3.42 at 970 K) (Lu14Co3In3-type). The structures of Gd6Co2Al, Gd6Co2.21Al0.79 and Gd14Co2.58Al3.42 flux-grown at 970 K have been refined from the single crystal X-ray diffraction data. Additionally, new ternary compounds Gd2Co5.7-5.3Al1.3-1.7 (Er2Co7-type) and Gd58Co20Al22 (unknown type structure) have been identified. Quasi-binary solid solutions were detected for Gd2Co17, GdCo5, Gd2Co7, GdCo3, GdCo2 and GdAl2 at 870/1070 K, while no appreciable solubility was observed for the other binary compounds in the Gd-Co-Al system. Magnetic properties of the Gd2Co3Al9, Gd3Co4.6Al11, Gd7Co6Al7, Gd6Co2.2Al0.8 and Gd14Co2.58Al3.42 compounds have been studied and are presented in this work. Gd6Co2.2Al0.8, Gd3Co4.6Al11, Gd7Co6Al7 and Gd14Co2.58Al3.42 order ferromagnetically, while Gd2Co3Al9 displays antiferromagnetic transition. Additionally, {Y, Sm, Tb - Tm}2Co6Al19 (U2Co6Al19-type), Yb2Co3Al9 (Y2Co3Ga9-type), {Y, Sm, Tm, Yb}3Co4.6Al11 (Gd3Ru4Al12-type) and Tb7Co6Al7 (Pr7Co6Al7-type) compounds have been synthesized and investigated.

  12. Plastic deformation of Al13Fe4 particles in Al-Al13Fe4 by high-speed compression

    International Nuclear Information System (INIS)

    Yoneyama, N.; Mizoguchi, K.; Kumai, S.; Sato, A.; Kiritani, M.

    2003-01-01

    Spray-formed Al-Fe alloys having undergone high-speed deformation were examined under a high-voltage electron microscope. Two types of specimens were examined; one containing fine Al 13 Fe 4 particles, and the other containing large particles. In the former specimen, deformation is found to proceed in three patterns, depending on specimen thickness and strain rate: (1) without deformation of the Al 13 Fe 4 ; (2) breaking of the Al 13 Fe 4 ; or (3) melting of the Al 13 Fe 4 . Local melting is found to alter some of the Al 13 Fe 4 particles, to impart five-fold symmetry in diffraction or an amorphous structure. In the latter specimen, introduction of glide dislocations enabled us to determine a shear system in the mc102 monoclinic c2/m crystal of Al 13 Fe 4 . On the bases of these observations, the mechanism of high-speed deformation is discussed while taking into account the highly stressed and/or heated states of Al 13 Fe 4 embedded in Al matrix

  13. Al2O3 adherence on CoCrAl alloys

    International Nuclear Information System (INIS)

    Kingsley, L.M.

    1980-04-01

    Adherence of protective oxides on NiCrAl and CoCrAl superalloys has been promoted by a dispersion of a highly oxygen reactive element or its oxide being produced within the protection system. Two aspects of this subject are investigated here: the use of Al 2 O 3 as both the dispersion and protective oxide; and the production of an HfO 2 dispersion while simultaneously aluminizing the alloy. It was found that an Al 2 O 3 dispersion will act to promote the adherence of an external scale of Al 2 O 3 to a degree comparable to previously tested dispersions and an HfO 2 dispersion comparable to that produced by a Rhines pack treatment is produced during aluminization

  14. Development of Cu-Hf-Al ternary systems and tungsten wire/particle reinforced Cu48Hf43Al9 bulk metallic glass composites for strengthening

    International Nuclear Information System (INIS)

    Park, Joyoung; An, Jihye; Choi-Yim, Haein

    2010-01-01

    Stable bulk glass forming alloys can be developed over a wide range of compositions in Cu-Hf-Al ternary systems starting from the Cu 49 Hf 42 Al 9 bulk metallic glass. Ternary Cu-Hf-Al alloys can be cast directly from the melt into copper molds to form fully amorphous strips with thicknesses of 1 to 6 mm. The maximum critical diameter of the new Cu-Hf-Al ternary alloy was 6 mm. X-ray diffraction patterns were used to confirm the amorphous nature of the ternary Cu-Hf-Al alloys. To increase the toughness of these metallic glasses, we reinforced the Cu 48 Hf 43 Al 9 bulk metallic glass-forming liquid with a 50% volume fraction of tungsten particles and an 80% volume fraction of tungsten wires with diameters of 242.4 μm. Composites with a critical diameter of 7 mm and length 70 mm were synthesized. The structure of the composites was confirmed by using X-ray diffraction (XRD), and the scanning electron microscopy (SEM). The mechanical properties of the composites were studied in compression tests. The thermal stability and the crystallization processes of the Cu-Hf-Al alloys and composites were investigated by using differential scanning calorimetry (DSC). Values of the glass transition temperature (T g ), the crystallization temperature (T x ), and the supercooled liquid region (ΔT = T x - T g ) are given in this paper.

  15. Investigations on Cu-Ni and Cu-Al systems with secondary ion mass spectrometry (SIMS)

    International Nuclear Information System (INIS)

    Rodriguez-Murcia, H.; Beske, H.E.

    1976-04-01

    The ratio of the ionization coefficients of secondary atomic ions emitted from the two component systems Cu-Ni and Cu-Al was investigated as a function of the concentration of the two components. In the low concentration range the ratio of the ionization coefficients is a constant. An influence of the phase composition on the ratio of the ionization coefficients was found in the Cu-Al system. In addition, the cluster ion emission was investigated as a function of the concentration and the phase composition of the samples. The secondary atomic ion intensity was influenced by the presence of cluster ions. The importance of the cluster ions in quantitative analysis and phase determination by means of secondary ion mass spectrometry are discussed. (orig.) [de

  16. Equilibrium and kinetic studies of systems of hydrogen isotopes, lithium hydrides, aluminum, and LiAlO2

    International Nuclear Information System (INIS)

    Owen, J.H.; Randall, D.

    1976-01-01

    Tritium might be bred by the 6 Li(n,α)T reaction in a solid lithium alloy or compound in the blanket of a controlled thermonuclear reactor to avoid problems associated with molten lithium or lithium compounds. Li--Al and LiAlO 2 systems containing hydrogen, deuterium, or tritium were studied 10 to 15 years ago at the Savannah River Laboratory. This paper descibes measurements of (1) the distribution of tritium and helium throughout both α and β phases of irradiated Li--Al alloy, (2) the migration rate of tritium to the β phase during moderate heating, (3) equilibrium pressures as functions of temperature of H 2 , D 2 , or T 2 in contact with lithium hydrides + aluminum, Li--Al alloy, or irradiated Li--Al alloy, (4) the equilibrium constant for the reaction LiH + Al → LiAl + 1 / 2 H 2 as a function of temperature, and (5) extraction rates of tritium from irradiated LiAlO 2 targets at elevated temperatures

  17. SAP: structure, function, and its roles in immune-related diseases.

    Science.gov (United States)

    Xi, Dan; Luo, TianTian; Xiong, Haowei; Liu, Jichen; Lu, Hao; Li, Menghao; Hou, Yuqing; Guo, Zhigang

    2015-01-01

    Serum amyloid P component (SAP), also known as pentraxin-2, is a member of the pentraxin protein family with an established relationship to the immune response. In the last century, SAP has been used as a diagnostic marker in amyloidosis diagnosis and patient follow-up. SAP has been thought to have potential for treating and curing amyloidosis and fibrosis diseases. More recently, it has been shown that SAP may serve as both a diagnostic marker and a therapeutic target for many immune-related diseases, such as cardiovascular, pulmonary, nephritic, neurological and autoimmune diseases. In the cardiovascular system, SAP has been defined as the culprit in amyloidosis in the heart. SAP may also exert a protective role during the early stage of atherosclerosis and myocardial fibrosis. In noncardiovascular system diseases, SAP is being developed for the treatment of pulmonary fibrosis. In this review, we summarize SAP history, structure, and its roles in immune-related diseases in different systems with emphasis on the cardiovascular system. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  18. Effect of AlF3 on the Density and Elastic Properties of Zinc Tellurite Glass Systems

    Science.gov (United States)

    Sidek, Haji Abdul Aziz; Rosmawati, Shaharuddin; Halimah, Mohamed Kamari; Matori, Khamirul Amin; Talib, Zainal Abidin

    2012-01-01

    This paper presents the results of the physical and elastic properties of the ternary zinc oxyfluoro tellurite glass system. Systematic series of glasses (AlF3)x(ZnO)y(TeO2)z with x = 0–19, y = 0–20 and z = 80, 85, 90 mol% were synthesized by the conventional rapid melt quenching technique. The composition dependence of the physical, mainly density and molar volume, and elastic properties is discussed in term of the AlF3 modifiers addition that are expected to produce quite substantial changes in their physical properties. The absence of any crystalline peaks in the X-ray diffraction (XRD) patterns of the present glass samples indicates the amorphous nature. The addition of AlF3 lowered the values of the densities in ternary oxyfluorotellurite glass systems. The longitudinal and transverse ultrasonic waves propagated in each glass sample were measured using a MBS8020 ultrasonic data acquisition system. All the velocity data were taken at 5 MHz frequency and room temperature. The longitudinal modulus (L), shear modulus (G), Young’s modulus (E), bulk modulus (K) and Poisson’s ratio (σ) are obtained from both velocities data and their respective density. Experimental data shows the density and elastic moduli of each AlF3-ZnO-TeO2 series are found strongly depend upon the glass composition. The addition of AlF3 modifiers into the zinc tellurite causes substantial changes in their density, molar volume as well as their elastic properties.

  19. Computer modeling of the process of self-propagating high-temperature synthesis in thin system Ni-Al

    International Nuclear Information System (INIS)

    Poletayev, G.M.; Starostenkov, M.D.; Denisova, N.F.; Skakov, M.K.

    2004-01-01

    Full text: The process of synthesis of thermal phases of the system Ni-Al is studied through the method of molecular dynamics. As the object of investigation was chosen two-dimensional crystal, that corresponds to atomic packing laying at the plane of volumetric fcc crystal. Clean Ni was taken as a matrix crystal. A particle of clean Al is packed in the center of matrix block. Beyond the bounds of calculated block crystal packing is repeated with the help of periodical border conditions. The interaction between different pairs of atoms is set by pair potential function of Morse, considering interatomic bonding of the point of the sixth coordinate sphere. The allocation of speeds of atomic function in the system is set through the Boltzmann factor, depending the temperature. When the bicrystal is represented by the ideal atom packing and there are no vacancies , the process of structural adjustment is only observed at the temperature, that is higher than melting point. At that, structural adjustment is observed in circular mechanism of atom allocation, also through the border between phases of clean Ni and Al. As a result, Al particle is transformed, at the border between metals, fields of positional disorder and embryos of intermetallide phases NiAl 2 , Ni 2 Al, Ni 3 Al. The introduction of of free volume through the creation of vacancies significantly lowers the temperature of the beginning of the synthesis process of intermetallide phases. The greatest decrease in temperature to the point of 300 K happens, when the vacancies are located in Ni field of bicrystal, the beginning of the thermo-activation is directly connected with the distance from interphase borders. As the process of thermo-activation continues, vacancies located in Ni matrix right up to seventh neighborhood relatively the border bicrystal. During thermo-activation Al particles enter the field and activate the synthesis process

  20. Amyloidotic muscle pseudohypertrophy: case report Pseudo-hipertrofia muscular associada com amiloidose: relato de caso

    OpenAIRE

    Rosana Herminia Scola; Lineu Cesar Werneck; Cássio Slompo Ramos; Ricardo Pasquini; Hans Graf; Walter Olesko Arruda

    2001-01-01

    The authors report one case of amyloidosis associated with muscular pseudohypertrophy in a 46-year-old woman, who developed weakness, macroglossia and muscle hypertrophy associated with primary systemic amyloidosis. Electromyography showed a myopathic pattern and bilateral carpal tunnel syndrome. The muscle biopsy presented with a type I and II fiber hypertrophy and infiltration of amyloid material in the interstitious space and artery walls. She underwent bone marrow transplantation with sta...

  1. Research on numerical control system based on S3C2410 and MCX314AL

    Science.gov (United States)

    Ren, Qiang; Jiang, Tingbiao

    2008-10-01

    With the rapid development of micro-computer technology, embedded system, CNC technology and integrated circuits, numerical control system with powerful functions can be realized by several high-speed CPU chips and RISC (Reduced Instruction Set Computing) chips which have small size and strong stability. In addition, the real-time operating system also makes the attainment of embedded system possible. Developing the NC system based on embedded technology can overcome some shortcomings of common PC-based CNC system, such as the waste of resources, low control precision, low frequency and low integration. This paper discusses a hardware platform of ENC (Embedded Numerical Control) system based on embedded processor chip ARM (Advanced RISC Machines)-S3C2410 and DSP (Digital Signal Processor)-MCX314AL and introduces the process of developing ENC system software. Finally write the MCX314AL's driver under the embedded Linux operating system. The embedded Linux operating system can deal with multitask well moreover satisfy the real-time and reliability of movement control. NC system has the advantages of best using resources and compact system with embedded technology. It provides a wealth of functions and superior performance with a lower cost. It can be sure that ENC is the direction of the future development.

  2. Viscosity of SiO2-"FeO"-Al2O3 System in Equilibrium with Metallic Fe

    Science.gov (United States)

    Chen, Mao; Raghunath, Sreekanth; Zhao, Baojun

    2013-08-01

    The present study delivered the measurements of viscosities in SiO2-"FeO"-Al2O3 system in equilibrium with metallic Fe. The rotational spindle technique was used in the measurements at the temperature range of 1473 K to 1773 K (1200 °C to 1500 °C). Molybdenum crucibles and spindles were employed in all measurements. The Fe saturation condition was maintained by an iron plate placed at the bottom of the crucible. The equilibrium compositions of the slags were measured by EPMA after the viscosity measurements. The effect of up to 20 mol. pct Al2O3 on the viscosity of the SiO2-"FeO" slag was investigated. The "charge compensation effect" of the Al2O3 and FeO association has been discussed. The modified quasi-chemical viscosity model has been optimized in the SiO2-"FeO"-Al2O3 system in equilibrium with metallic Fe to describe the viscosity measurements of the present study.

  3. Microglia in diffuse plaques in hereditary cerebral hemorrhage with amyloidosis (Dutch). An immunohistochemical study.

    Science.gov (United States)

    Maat-Schieman, M L; Rozemuller, A J; van Duinen, S G; Haan, J; Eikelenboom, P; Roos, R A

    1994-09-01

    In hereditary cerebral hemorrhage with amyloidosis (Dutch) (HCHWA-D) beta/A4 amyloid deposition is found in meningocortical blood vessels and in diffuse plaques in the cerebral cortex. Diffuse plaques putatively represent early stages in the formation of senile plaques. Microglia are intimately associated with congophilic plaques in Alzheimer's disease (AD), but microglial involvement in diffuse plaque formation is controversial. Therefore, we studied the relationship between microglia and diffuse plaques in the cerebral cortex of four patients with HCHWA-D using a panel of macrophage/microglia markers (mAbs LCA, LeuM5, LeuM3, LN3, KP1, OKIa, CLB54, Mac1, Ki-M6, AMC30 and the lectin RCA-1). Eight AD patients, one demented Down's syndrome (DS) patient and four non-demented controls were included for comparison. In controls and HCHWA-D patients ramified or "resting" microglia formed a reticular array in cortical gray and subcortical white matter. Microglial cells in or near HCHWA-D diffuse plaques retained their normal regular spacing and ramified morphology. In AD/DS gray matter more microglial cells were stained than in controls and HCHWA-D patients. Intensely immunoreactive microglia with enlarged cell bodies and short, thick processes clustered in congophilic plaques. In contrast to the resting microglia, these "activated microglia" strongly expressed class II major histocompatibility complex antigen, HLA-DR, and were AMC30-immunoreactive. These findings support the view that microglia play a role in the formation of congophilic plaques but do not initiate diffuse plaque formation. Another finding in this study is the presence of strong monocyte/macrophage marker immunoreactivity in the wall of cortical congophilic blood vessels in HCHWA-D.

  4. Klystron 'efficiency loop' for the ALS storage ring RF system

    International Nuclear Information System (INIS)

    Kwiatkowski, Slawomir; Julian, Jim; Baptiste, Kenneth

    2002-01-01

    The recent energy crisis in California has led us to investigate the high power RF systems at the Advanced Light Source (ALS) in order to decrease the energy consumption and power costs. We found the Storage Ring Klystron Power Amplifier system operating as designed but with significant power waste. A simple proportional-integrator (PI) analog loop, which controls the klystron collector beam current, as a function of the output RF power, has been designed and installed. The design considerations, besides efficiency improvement, were to interface to the existing system without major expense. They were to also avoid the klystron cathode power supply filter's resonance in the loop's dynamics, and prevent a conflict with the existing Cavity RF Amplitude Loop dynamics. This efficiency loop will allow us to save up to 700 MW-hours of electrical energy per year and increase the lifetime of the klystron

  5. ALS-Plus Syndrome: Non-Pyramidal Features in a Large ALS Cohort

    Science.gov (United States)

    McCluskey, Leo; Vandriel, Shannon; Elman, Lauren; Van Deerlin, Vivianna M.; Powers, John; Boller, Ashley; Wood, Elisabeth McCarty; Woo, John; McMillan, Corey T.; Rascovsky, Katya; Grossman, Murray

    2014-01-01

    Objective Autopsy studies show widespread pathology in amyotrophic lateral sclerosis (ALS), but clinical surveys of multisystem disease in ALS are rare. We investigated ALS-Plus syndrome, an understudied group of patients with clinical features extending beyond pyramidal and neuromuscular systems with or without cognitive/behavioral deficits. Methods In a large, consecutively-ascertained cohort of 550 patients with ALS, we documented atypical clinical manifestations. Genetic screening for C9orf72 hexanucleotide expansions was performed in 343 patients, and SOD1, TARDBP, and VCP were tested in the subgroup of patients with a family history of ALS. Gray matter and white matter imaging was available in a subgroup of 30 patients. Results Seventy-five (13.6%) patients were identified with ALS-Plus syndrome. We found disorders of ocular motility, cerebellar, extrapyramidal and autonomic functioning. Relative to those without ALS-Plus, cognitive impairment (8.0% vs 2.9%, p=0.029), bulbar-onset (49.3% vs 23.2%, pALS-Plus. Survival was significantly shorter in ALS-Plus (29.66 months vs 42.50 months, p=0.02), regardless of bulbar-onset or mutation status. Imaging revealed significantly greater cerebellar and cerebral disease in ALS-Plus compared to those without ALS-Plus. Conclusions ALS-Plus syndrome is not uncommon, and the presence of these atypical features is consistent with neuropathological observations that ALS is a multisystem disorder. ALS-Plus syndrome is associated with increased risk for poor survival and the presence of a pathogenic mutation. PMID:25086858

  6. Atrophy rates in asymptomatic amyloidosis: implications for Alzheimer prevention trials.

    Directory of Open Access Journals (Sweden)

    K Abigail Andrews

    % absolute slowing of hippocampal atrophy rate in an 18-month treatment trial. We conclude that hippocampal atrophy may be a feasible outcome measure for secondary prevention studies in asymptomatic amyloidosis.

  7. Whole Exome Sequencing Leading to the Diagnosis of Dysferlinopathy with a Novel Missense Mutation (c.959G>C

    Directory of Open Access Journals (Sweden)

    Abhisek Swaika

    2016-01-01

    Full Text Available Dysferlinopathy is an uncommon, progressive muscular dystrophy that has a wide phenotypic variability and primarily supportive management (Nguyen et al., 2007; Narayanaswami et al., 2014. Amyloid myopathy is a distinct, rare disorder that can present similarly to inflammatory myopathies and requires a high clinical suspicion for early intervention to prolong survival. Amyloid myopathy is typically associated with other systemic manifestations of amyloidosis, but rare cases of isolated amyloid myopathy have been described (Mandl et al., 2000; Hull et al., 2001. Positive Congo red stains on tissue biopsy remain the gold standard for diagnosis (Spuler et al., 1998; Karacostas et al., 2005. A high clinical suspicion and meticulous diagnostic workup that includes novel techniques are necessary for identifying these rare disorders. We report a middle-aged man with progressive leg muscle weakness who was initially treated as having amyloid myopathy but was later diagnosed as having dysferlinopathy by Whole Exome Sequencing (WES analysis. We also report a novel missense mutation (c.959G>C to help correlate in any patient with presumed dysferlinopathy and to add to the already known genotype of this disorder.

  8. Al-Shebab: An Al-Qaeda Affiliate Case Study (2Rev)

    Science.gov (United States)

    2017-10-01

    deputies responsible for finance , administration, and security. Coordination among regional groups is common.10 The deputy leader of al-Shebab, Mukhtar...Maaliya (Ministry of Finance ) controlled taxation. A complex court system also emerged. Multiple training camps were built that focused on hand-to-hand...Nathan Chandler, Counterterrorism and Counterinsurgency in Somalia: Assessing the Campaign Against Al Shabaab, Rand Corporation , 2016, accessed 6/5

  9. Quasiparticle band structure for the Hubbard systems: Application to α-CeAl2

    International Nuclear Information System (INIS)

    Costa-Quintana, J.; Lopez-Aguilar, F.; Balle, S.; Salvador, R.

    1990-01-01

    A self-energy formalism for determining the quasiparticle band structure of the Hubbard systems is deduced. The self-energy is obtained from the dynamically screened Coulomb interaction whose bare value is the correlation energy U. A method for integrating the Schroedingerlike equation with the self-energy operator is given. The method is applied to the cubic Laves phase of α-CeAl 2 because it is a clear Hubbard system with a very complex electronic structure and, moreover, this system provides us with sufficient experimental data for testing our method

  10. Assessment of phase constitution on the Al-rich region of rapidly solidified Al-Co-Fe-Cr alloys

    International Nuclear Information System (INIS)

    Wolf, W.; Bolfarini, C.; Kiminami, C.S.; Botta, W.J.

    2016-01-01

    The formation of quasicrystalline approximants in rapidly solidified Al-Co-Fe-Cr alloys was investigated. Alloys of atomic composition Al 71 Co 13 Fe 8 Cr 8 , Al 77 Co 11 Fe 6 Cr 6 and Al 76 Co 19 Fe 4 Cr 1 were produced using melt spinning and arc melting methods and their microstructural characterization was carried out by X-ray diffraction, scanning electron microscopy and transmission electron microscopy. Up to the present there is no consensus in the literature regarding the formation of quasicrystalline phase or quasicrystalline approximants in the Al 71 Co 13 Fe 8 Cr 8 alloy. This work presents, for the first time, a detailed structural characterization of selected alloys in the Al-Co-Fe-Cr system close to the atomic composition Al 71 Co 13 Fe 8 Cr 8 . The results indicated the samples to be composed, mostly, by two intermetallic phases, which are quaternary extensions of Al 5 Co 2 and Al 13 Co 4 and are quasicrystalline approximants. Although the Al 5 Co 2 phase has already been reported in the Al 71 Co 13 Fe 8 Cr 8 alloy, the presence of the monoclinic Al 13 Co 4 is now identified for the first time in the as cast state. In the binary Al-Co system a quasicrystalline phase is known to form in a rapidly solidified alloy with composition close to the monoclinic and orthorhombic Al 13 Co 4 phases. This binary quasicrystalline phase presents an average valence electron per atom (e/a) between 1.7 and 1.9; thus, in addition to the Al 71 Co 13 Fe 8 Cr 8 alloy, the compositions Al 77 Co 11 Fe 6 Cr 6 and Al 76 Co 19 Fe 4 Cr 1 were chosen to be within the region of formation of the quaternary extension of the Al 13 Co 4 phase and also within the (e/a) of 1.7 to 1.9. However, no quasicrystalline phase is present in any of the studied alloys. The Al-Co-Fe-Cr system, around the compositions studied, is composed of quaternary extensions of Al-Co intermetallic phases, which present solubility of Fe and Cr at Co atomic sites. - Highlights: •The Al rich region of the Al

  11. Phase Diagram of Al-Ca-Mg-Si System and Its Application for the Design of Aluminum Alloys with High Magnesium Content

    Directory of Open Access Journals (Sweden)

    Nikolay A. Belov

    2017-10-01

    Full Text Available The phase transformations in the Al-Ca-Mg-Si system have been studied using thermodynamic calculations and experimental methods. We show that at 10% Magnesium (Mg, depending on the concentrations of calcium (Ca and silicon (Si, the following phases crystallize first (apart from the aluminum (Al solid solution: Al4Ca, Mg2Si, and Al2CaSi2. We have found that the major part of the calculated concentration range is covered by the region of the primary crystallization of the Al2CaSi2 phase. Regardless of the Ca and Si content, the solidification of the aluminum-magnesium alloys ends with the following nonvariant eutectic reaction: L → (Al + Al4Ca + Mg2Si + Al3Mg2. With respect to the temperature and composition of the liquid phase, this reaction is close to the eutectic reaction in the Al-Mg binary system. The addition of Ca and Si to the Al-10% Mg base alloy increases its hardness, reduces its density, and has no negative influence on its corrosion resistance. We have also established that the near-eutectic alloy containing about 3% Ca and 1% Si has the optimum structure.

  12. Single- and dual-wavelength laser pulses induced modification in 10×(Al/Ti)/Si multilayer system

    Energy Technology Data Exchange (ETDEWEB)

    Salatić, B. [University of Belgrade, Institute of Physics Belgrade, Pregrevica 118, 11080 Belgrade (Serbia); Petrović, S., E-mail: spetro@vinca.rs [University of Belgrade, Institute of Nuclear Science-Vinča, POB 522, 11001 Belgrade (Serbia); Peruško, D. [University of Belgrade, Institute of Nuclear Science-Vinča, POB 522, 11001 Belgrade (Serbia); Čekada, M.; Panjan, P. [Jožef Stefan Institute, Jamova 39, 1000 Ljubljana (Slovenia); Pantelić, D.; Jelenković, B. [University of Belgrade, Institute of Physics Belgrade, Pregrevica 118, 11080 Belgrade (Serbia)

    2016-01-01

    Graphical abstract: - Highlights: • Experimental and numerical study of laser-induced ablation and micro-sized crater formation. • Dual-wavelength pulses induce creation of wider and deeper craters due to synergies of two processes. • Sunflower-like structure formed by dual-wavelength pulses at low irradiance. • Numerical model of nanosecond pulsed laser ablation for complex (Al/Ti)/Si system has been developed. - Abstract: The surface morphology of the ablation craters created in the multilayer 10×(Al/Ti)/Si system by nanosecond laser pulses at single- and dual wavelength has been studied experimentally and numerically. A complex multilayer thin film including ten (Al/Ti) bilayers deposited by ion sputtering on Si(1 0 0) substrate to a total thickness of 260 nm were illuminated at different laser irradiance in the range 0.25–3.5 × 10{sup 9} W cm{sup −2}. Single pulse laser irradiation was done at normal incidence in air, with the single wavelength, either at 532 nm or 1064 nm or with both laser light simultaneously in the ratio of 1:10 for energy per pulse between second harmonic and 1064 nm. Most of the absorbed laser energy was rapidly transformed into heat, producing intensive modifications of composition and morphology on the sample surface. The results show an increase in surface roughness, formation of specific nanostructures, appearance of hydrodynamic features and ablation of surface material with crater formation. Applying a small fraction (10%) of the second harmonic in dual-wavelength pulses, a modification of the 10×(Al/Ti)/Si system by a single laser pulse was reflected in the formation of wider and/or deeper craters. Numerical calculations show that the main physical mechanism in ablation process is normal evaporation without phase explosion. The calculated and experimental results agree relatively well for the whole irradiance range, what makes the model applicable to complex Al/Ti multilayer systems.

  13. Tracking the 10Be-26Al source-area signal in sediment-routing systems of arid central Australia

    Science.gov (United States)

    Struck, Martin; Jansen, John D.; Fujioka, Toshiyuki; Codilean, Alexandru T.; Fink, David; Fülöp, Réka-Hajnalka; Wilcken, Klaus M.; Price, David M.; Kotevski, Steven; Fifield, L. Keith; Chappell, John

    2018-05-01

    Sediment-routing systems continuously transfer information and mass from eroding source areas to depositional sinks. Understanding how these systems alter environmental signals is critical when it comes to inferring source-area properties from the sedimentary record. We measure cosmogenic 10Be and 26Al along three large sediment-routing systems ( ˜ 100 000 km2) in central Australia with the aim of tracking downstream variations in 10Be-26Al inventories and identifying the factors responsible for these variations. By comparing 56 new cosmogenic 10Be and 26Al measurements in stream sediments with matching data (n = 55) from source areas, we show that 10Be-26Al inventories in hillslope bedrock and soils set the benchmark for relative downstream modifications. Lithology is the primary determinant of erosion-rate variations in source areas and despite sediment mixing over hundreds of kilometres downstream, a distinct lithological signal is retained. Post-orogenic ranges yield catchment erosion rates of ˜ 6-11 m Myr-1 and silcrete-dominant areas erode as slow as ˜ 0.2 m Myr-1. 10Be-26Al inventories in stream sediments indicate that cumulative-burial terms increase downstream to mostly ˜ 400-800 kyr and up to ˜ 1.1 Myr. The magnitude of the burial signal correlates with increasing sediment cover downstream and reflects assimilation from storages with long exposure histories, such as alluvial fans, desert pavements, alluvial plains, and aeolian dunes. We propose that the tendency for large alluvial rivers to mask their 10Be-26Al source-area signal differs according to geomorphic setting. Signal preservation is favoured by (i) high sediment supply rates, (ii) high mean runoff, and (iii) a thick sedimentary basin pile. Conversely, signal masking prevails in landscapes of (i) low sediment supply and (ii) juxtaposition of sediment storages with notably different exposure histories.

  14. Mechanochemical transformations in Li(Na)AlH{sub 4}-Li(Na)NH{sub 2} systems

    Energy Technology Data Exchange (ETDEWEB)

    Dolotko, Oleksandr [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States); Zhang Haiqiao [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States); Department of Materials Science and Engineering, Iowa State University, Ames, IA 50011-2300 (United States); Ugurlu, Ozan [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States); Department of Materials Science and Engineering, Iowa State University, Ames, IA 50011-2300 (United States); Wiench, Jerzy W. [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States); Pruski, Marek [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States); Scott Chumbley, L. [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States); Department of Materials Science and Engineering, Iowa State University, Ames, IA 50011-2300 (United States); Pecharsky, Vitalij [Ames Laboratory of the U.S. DOE, Iowa State University, Ames, IA 50011-3020 (United States) and Department of Materials Science and Engineering, Iowa State University, Ames, IA 50011-2300 (United States)]. E-mail: vitkp@ameslab.gov

    2007-05-15

    Mechanochemical transformations of tetrahydroaluminates and amides of lithium and sodium have been investigated using gas volumetric analysis, X-ray powder diffraction, solid-state nuclear magnetic resonance (NMR) and transmission electron microscopy. In a transformation of LiAlH{sub 4} and LiNH{sub 2} taken in an 1:1 molar ratio, the amount of released hydrogen (6.6 wt.% after 30 min ball milling) was higher than in any known one pot mechanochemical process involving a hydrogen-containing solid. A total of 4.3 wt.% of hydrogen is released by the NaAlH{sub 4}-NaNH{sub 2} system after 60 min ball milling; and 5.2 wt.% H{sub 2} is released when LiAlH{sub 4} and NaNH{sub 2} or NaAlH{sub 4} and LiNH{sub 2} are ball milled for 90 min and 120 min, respectively. All transformations proceed at room temperature. The mechanism of the overall transformation MAlH{sub 4}(s) + MNH{sub 2}(s) {sup {yields}} 2MH(s) + AlN(s) + 2H{sub 2}(g) was identified based on detailed spectroscopic analysis of the intermediate (M{sub 3}AlH{sub 6}) and final products of the ball milling process.

  15. Effects of O in a binary-phase TiAl-Ti3Al alloy: from site occupancy to interfacial energetics

    International Nuclear Information System (INIS)

    Wei Ye; Xu Huibin; Zhou Hongbo; Zhang Ying; Lu Guanghong

    2011-01-01

    We have investigated site occupancy and interfacial energetics of a TiAl-Ti 3 Al binary-phase system with O using a first-principles method. Oxygen is shown to energetically occupy the Ti-rich octahedral interstitial site, because O prefers to bond with Ti rather than Al. The occupancy tendency of O in TiAl alloy from high to low is α 2 -Ti 3 Al to the γ-α 2 interface and γ-TiAl. We demonstrate that O can largely affect the mechanical properties of the TiAl-Ti 3 Al system. Oxygen at the TiAl-Ti 3 Al interface reduces both the cleavage energy and the interface energy, and thus weakens the interface strength but strongly stabilizes the TiAl/Ti 3 Al interface with the O 2 molecule as a reference. Consequently, the mechanical property variation of TiAl alloy due to the presence of O not only depends on the number of TiAl/Ti 3 Al interfaces but also is related to the O concentration in the alloy.

  16. Rheed Investigation of Pd/Al Bimetallic System on KCl(001) Substrate

    Science.gov (United States)

    Masek, K.; Moroz, V.; Matolín, V.

    Pd/Al alloys have very interesting properties from the point of view of their possible application in heterogeneous catalysis. Preparation of small heteroepitaxial Pd/Al alloy particles opens a new way in studies of the influence of Pd/Al crystallographic structure on the alloy catalytic properties. Pd/Al alloy particles were grown by the molecular beam epitaxy method. Their crystallographical structure was controlled by reflection high energy electron diffraction (RHEED). It was found that Pd deposited on epitaxial 3D Al particles grown on KCl is intermixing with Al. This process is accompanied by the variation of lattice parameter from the Al value to the Pd one.

  17. A strategy for synthesis of pathogenic human immunoglobulin free light chains in E. coli.

    Directory of Open Access Journals (Sweden)

    Paola Rognoni

    Full Text Available Monoclonal immunoglobulin light chains are normally synthesized in excess compared to the heavy chain partners and can be detected in serum and urine ("free" LC. Occasionally free LC are per se cause of organ toxicity, as in free LC-related disorders. In AL amyloidosis, the most common of these conditions, free LC with peculiar biophysical properties related to their primary structure damage target organs and organize in amyloid fibrils. Unlimited availability of well-characterized free LC is instrumental to investigate the toxic effect of these proteins and to study their interactions with targets. We present a straightforward strategy to obtain recombinant monoclonal free LC by using a bacterial system. These proteins, expressed as inclusion bodies, were subjected to solubilization and refolding procedures to recover them in native form. To minimize differences from the circulating natural LC, full-length recombinant LC were expressed, i.e. complete of variable and constant regions, with the original amino acid sequence along the entire protein, and with no purification tags. The strategy was exploited to generate free LC from three AL amyloidosis patients. After purification, recombinant proteins were biochemically characterized and compared to the natural Bence Jones protein isolated from one of the patients. Results showed that the recombinant free LC were properly folded and formed homodimers in solution, similar to the natural Bence Jones protein used for comparison. Furthermore, as proof of pathogenicity, recombinant proteins formed amyloid fibrils in vitro. We believe that the present strategy represents a valuable tool to speed research in free LC-related disorders.

  18. The formation of quasicrystal phase in Al-Cu-Fe system by mechanical alloying

    Directory of Open Access Journals (Sweden)

    Dilermando Nagle Travessa

    2012-10-01

    Full Text Available In order to obtain quasicrystalline (QC phase by mechanical alloying (MA in the Al-Cu-Fe system, mixtures of elementary Al, Cu and Fe in the proportion of 65-20-15 (at. % were produced by high energy ball milling (HEBM. A very high energy type mill (spex and short milling times (up to 5 hours were employed. The resulting powders were characterized by X-ray diffraction (XRD, differential scanning calorimetry (DSC and scanning electron microscopy (SEM. QC phase was not directly formed by milling under the conditions employed in this work. However, phase transformations identified by DSC analysis reveals that annealing after HEBM possibly results in the formation of the ψ QC phase.

  19. Structure of new Al-Si based RQ systems with potentially enhanced stiffness

    International Nuclear Information System (INIS)

    Zigo, J.; Svec, P.; Janickovic, D.; Janotova, I.; Matko, I.; Svec, P. Sr.

    2014-01-01

    Amorphous and nanocrystalline metallic systems prepared by rapid quenching of melt are interesting for many unique properties. Mechanical properties are among the most important ones. Rapid quenching of metallic melt can enhance mechanical properties of material, compared to metal alloys prepared by conventional metallurgy. Aluminium as one of most accessible light-weight metals has wide range of applications as construction material. Alloying aluminium with another element can significantly improve mechanical properties. Reasonable choice for alloying element in terms of sustaining light-weight nature of the material is silicon. Dissolving silicon in aluminium can be achieved, even with the aid of rapid quenching, only up to few weight percent. Increasing the content of dissolved silicon is possible by adding other alloying components. Candidates with respect to low specific mass are transition elements of 4"t"h period (from scandium to zinc). In our experimental study, we have chosen alloying of Al-Si with transition elements (T) iron, cobalt and nickel. Composition of the investigated systems was Al_8_0_-_xT_xSi_2_0 where T = Fe, Co, Ni and x = 0, 5 and 10. (authors)

  20. Micromechanisms of fracture and fatigue in Ti3Al based and TiAl based intermetallics

    International Nuclear Information System (INIS)

    James, A.W.; Chave, R.A.; Hippsley, C.A.; Bowen, P.

    1993-01-01

    Micromechanisms of fracture and fatigue crack growth resistance in specific Ti 3 Al based and TiAl based intermetallics are reviewed. Effects of test temperature, environment and microstructure on crack growth resistance are considered in detail for several Ti 3 Al and Ti'Al based intermetallic systems under development. The implications of these studies for the structural reliability of these materials is also addressed briefly. (orig.)

  1. First discovery and structural characterization of a new compound in Al-Si-O-C system

    International Nuclear Information System (INIS)

    Iwata, Tomoyuki; Kaga, Motoaki; Nakano, Hiromi; Fukuda, Koichiro

    2009-01-01

    A quaternary oxycarbide, [Al 16.77(5) Si 1.23(5) ] Σ 18 [O 3.04(9) C 10.96(9) ] Σ 14 , has been for the first time discovered in the Al-Si-O-C system. The crystal structure was characterized by X-ray powder diffraction, transmission electron microscopy and energy dispersive X-ray spectroscopy (EDX). The atom ratios [Al:Si] were determined by EDX, and the initial structural model was derived by the direct methods. The structural parameters as well as the atom ratios [O:C] were determined by the Rietveld method. The crystal is monoclinic (space group C2/m, Z=1) with lattice dimensions a=0.57404(1) nm, b=0.331435(5) nm, c=1.92410(2) nm, β=90.036(1) o and V=0.366076(9) nm 3 . The final structural model showed the positional disordering of Al/Si sites. The validity of the split-atom model was verified by the three-dimensional electron density distribution, the structural bias of which was reduced as much as possible using the maximum-entropy methods-based pattern fitting (MPF). The reliability indices calculated from the MPF were R wp =4.20% (S=1.14), R p =3.09%, R B =0.92% and R F =1.05%. The crystal was an inversion twin with nearly the same twin fraction. - Graphical abstract: A quaternary oxycarbide firstly discovered in the Al-Si-O-C system. The crystal is an inversion twin, and hence the structure is represented by a split-atom model. The three-dimensional electron density distribution is determined by the maximum-entropy methods-based pattern fitting, being consistent with the disordered structural model.

  2. Determination of γ′+γ / γ Phase Boundary in Ni-Al-Cr System Using DTA Thermal Analysis

    Directory of Open Access Journals (Sweden)

    Maciąg T.

    2016-03-01

    Full Text Available Mechanical properties at elevated temperature, in modern alloys based on intermetallic phase Ni3Al are connected with phase composition, especially with proportion of ordered phase γ′ (L12 and disordered phase γ (A1. In this paper, analysis of one key systems for mentioned alloys - Ni-Al-Cr, is presented. A series of alloys with chemical composition originated from Ni-rich part of Ni-Al-Cr system was prepared. DTA thermal analysis was performed on all samples. Based on shape of obtained curves, characteristic for continuous order-disorder transition, places of course of phase boundaries γ′+γ / γ were determined. Moreover, temperature of melting and freezing of alloys were obtained. Results of DTA analysis concerning phase boundary γ′+γ / γ indicated agreement with results obtained by authors using calorimetric solution method.

  3. Assessment of phase constitution on the Al-rich region of rapidly solidified Al-Co-Fe-Cr alloys

    Energy Technology Data Exchange (ETDEWEB)

    Wolf, W., E-mail: witorw@gmail.com [Programa de Pós-Graduação em Ciência e Engenharia de Materiais, Universidade Federal de São Carlos, Rod. Washington Luiz, Km 235, 13565-905 São Carlos, SP (Brazil); Bolfarini, C., E-mail: cbolfa@ufscar.br [Departamento de Engenharia de Materiais, Universidade Federal de São Carlos, Rod. Washington Luiz, Km 235, 13565-905 São Carlos, SP (Brazil); Kiminami, C.S., E-mail: kiminami@ufscar.br [Departamento de Engenharia de Materiais, Universidade Federal de São Carlos, Rod. Washington Luiz, Km 235, 13565-905 São Carlos, SP (Brazil); Botta, W.J., E-mail: wjbotta@ufscar.br [Departamento de Engenharia de Materiais, Universidade Federal de São Carlos, Rod. Washington Luiz, Km 235, 13565-905 São Carlos, SP (Brazil)

    2016-12-15

    The formation of quasicrystalline approximants in rapidly solidified Al-Co-Fe-Cr alloys was investigated. Alloys of atomic composition Al{sub 71}Co{sub 13}Fe{sub 8}Cr{sub 8}, Al{sub 77}Co{sub 11}Fe{sub 6}Cr{sub 6} and Al{sub 76}Co{sub 19}Fe{sub 4}Cr{sub 1} were produced using melt spinning and arc melting methods and their microstructural characterization was carried out by X-ray diffraction, scanning electron microscopy and transmission electron microscopy. Up to the present there is no consensus in the literature regarding the formation of quasicrystalline phase or quasicrystalline approximants in the Al{sub 71}Co{sub 13}Fe{sub 8}Cr{sub 8} alloy. This work presents, for the first time, a detailed structural characterization of selected alloys in the Al-Co-Fe-Cr system close to the atomic composition Al{sub 71}Co{sub 13}Fe{sub 8}Cr{sub 8}. The results indicated the samples to be composed, mostly, by two intermetallic phases, which are quaternary extensions of Al{sub 5}Co{sub 2} and Al{sub 13}Co{sub 4} and are quasicrystalline approximants. Although the Al{sub 5}Co{sub 2} phase has already been reported in the Al{sub 71}Co{sub 13}Fe{sub 8}Cr{sub 8} alloy, the presence of the monoclinic Al{sub 13}Co{sub 4} is now identified for the first time in the as cast state. In the binary Al-Co system a quasicrystalline phase is known to form in a rapidly solidified alloy with composition close to the monoclinic and orthorhombic Al{sub 13}Co{sub 4} phases. This binary quasicrystalline phase presents an average valence electron per atom (e/a) between 1.7 and 1.9; thus, in addition to the Al{sub 71}Co{sub 13}Fe{sub 8}Cr{sub 8} alloy, the compositions Al{sub 77}Co{sub 11}Fe{sub 6}Cr{sub 6} and Al{sub 76}Co{sub 19}Fe{sub 4}Cr{sub 1} were chosen to be within the region of formation of the quaternary extension of the Al{sub 13}Co{sub 4} phase and also within the (e/a) of 1.7 to 1.9. However, no quasicrystalline phase is present in any of the studied alloys. The Al-Co-Fe-Cr system

  4. A practical approach to the diagnosis of systemic amyloidoses.

    Science.gov (United States)

    Fernández de Larrea, Carlos; Verga, Laura; Morbini, Patrizia; Klersy, Catherine; Lavatelli, Francesca; Foli, Andrea; Obici, Laura; Milani, Paolo; Capello, Gian Luca; Paulli, Marco; Palladini, Giovanni; Merlini, Giampaolo

    2015-04-02

    Accurate diagnosis of systemic amyloidosis is necessary both for assessing the prognosis and for delineating the appropriate treatment. It is based on histologic evidence of amyloid deposits and characterization of the amyloidogenic protein. We prospectively evaluated the diagnostic performance of immunoelectron microscopy (IEM) of abdominal fat aspirates from 745 consecutive patients with suspected systemic amyloidoses. All cases were extensively investigated with clinical and laboratory data, with a follow-up of at least 18 months. The 423 (56.8%) cases with confirmed systemic forms were used to estimate the diagnostic performance of IEM. Compared with Congo-red-based light microscopy, IEM was equally sensitive (75% to 80%) but significantly more specific (100% vs 80%; P 99% of the cases. IEM of abdominal fat aspirates is an effective tool in the routine diagnosis of systemic amyloidoses. © 2015 by The American Society of Hematology.

  5. The Ag-Al-Cu system Part I: Reassessment of the constituent binaries on the basis of new experimental data

    International Nuclear Information System (INIS)

    Witusiewicz, V.T.; Hecht, U.; Fries, S.G.; Rex, S.

    2004-01-01

    Aiming to obtain a reliable description of the ternary Ag-Al-Cu system the thermodynamic evaluation of the constituent binaries Ag-Al, Ag-Cu and Al-Cu are revised by modelling of the Gibbs energy of all individual phases using the CALPHAD approach. The model parameters have been evaluated using a computer optimisation technique based on the established descriptions of the systems and taking into account the data on thermodynamic properties and phase equilibria both reported in recent publications and obtained by own measurements. The phase diagrams and the thermodynamic properties calculated with the evaluated parameters are in good agreement with the corresponding experimental data

  6. Particle variations and effect on the microstructure and microhardness of Ti6al4V hybrid metal matrix system

    CSIR Research Space (South Africa)

    Akinlabi, ET

    2017-01-01

    Full Text Available obtained as follow: 3.0 Ti6Al4 V, B4C and BN, 3.2 Ti6Al4 V, B4C and BN, and 3.4 Ti6Al4 V, B4C and BN systems. Laser coating experiment was done at 1400W. The influence of difference in variation of powder on the microstructure and hardness values...

  7. Comparative study of gamma ray shielding and some properties of PbO–SiO2–Al2O3 and Bi2O3–SiO2–Al2O3 glass systems

    International Nuclear Information System (INIS)

    Singh, K.J.; Kaur, Sandeep; Kaundal, R.S.

    2014-01-01

    Gamma-ray shielding properties have been estimated in terms of mass attenuation coefficient, half value layer and mean free path values, whereas, structural studies have been performed in terms of density, optical band gap, glass transition temperature and longitudinal ultrasonic velocity parameters. X-ray diffraction, UV–visible, DSC and ultrasonic techniques have been used to explore the structural properties of PbO–SiO 2 –Al 2 O 3 and Bi 2 O 3 –SiO 2 –Al 2 O 3 glass systems. - Highlights: • Bi 2 O 3 –SiO 2 –Al 2 O 3 and PbO–SiO 2 –Al 2 O 3 glasses can replace conventional concretes as gamma-ray shielding materials. • Gamma-ray shielding properties improve with the addition of heavy metals. • Rigidity deteriorates with the increase in the content of heavy metals. • Bi 2 O 3 –SiO 2 –Al 2 O 3 glass system is better than PbO–SiO 2 –Al 2 O 3 glass system in terms of gamma-ray shielding as well as structural properties

  8. Modelling Eu(III) speciation in a Eu(III)/PAHA/α-Al2O3 ternary system

    International Nuclear Information System (INIS)

    Janot, Noemie; Reiller, Pascal E.; Benedetti, Marc F.

    2013-01-01

    In this work, modelling of Eu(III) speciation in a ternary system, i.e., in presence of purified Aldrich humic acid (PAHA) and α-Al 2 O 3 , is presented. First, the mineral surface charge is measured by potentiometric titrations and then described using the CD-MUSIC model. This model is also used to describe Eu(III) binding to the α-Al 2 O 3 surface at different pH values, ionic strength and mineral concentrations. Time resolved luminescence spectroscopy (TRLS) is then used to study the binding of Eu(III) to PAHA at pH 4 with different humic acid concentrations. The spectra are used to calculate a spectroscopic 'titration curve', used to determine Eu(III)/PAHA binding parameters in the NICA-Donnan model. Following a previous study (Janot et al., Water Res. 46, 731-740), modelling of the ternary system is based upon the definition of two PAHA pools where one fraction remains in solution and the other is adsorbed onto the mineral surface, with each possessing different proton and metal binding parameters. The modification of protonation behaviour for both fractions is examined using spectrophotometric titrations of the non adsorbed PAHA fraction at different organic/mineral ratios. These data are then used to describe Eu(III) interactions in the ternary system: Eu(III) re-partitioning in the ternary system is calculated for different pH, ionic strength and PAHA concentrations, and results are compared to experimental observations. The model is in good agreement with experimental data, except at high PAHA fractionation rates. Results show that organic complexation dominates over a large pH range, with the predominant species existing as the surface-bound fraction. Above pH 8, Eu(III) seems to be mostly complexed to the mineral surface, which is in agreement with previous spectroscopic observations (Janot et