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

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

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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

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.

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

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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.

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

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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.

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

Amyloidosis and Kidney Disease

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... 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 ...

[Hepatic amyloidosis as cause of severe intrahepatic cholestasis].

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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.

Frictional amyloidosis in Oman - A study of ten cases

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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.

Diagnostic studies in amyloidosis

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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

Localized Lymph Node Light Chain Amyloidosis

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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.

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.

Dysphagia as initial presentation of primary amyloidosis

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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.

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

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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.

Macular Amyloidosis and Epstein-Barr Virus

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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.

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

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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.

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

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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

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.

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

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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.

[Amyloidosis complicating spondyloarthropathies: Study of 15 cases].

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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.

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

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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.

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

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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)

Mediastinal and bronchovascular amyloidosis

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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

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

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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.

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

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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.

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

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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

Laryngeal Amyloidosis Mimicking Glottic Cancer: A Case Report

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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

Laryngeal Amyloidosis Mimicking Glottic Cancer: A Case Report

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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.

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.

An interesting case of renal amyloidosis

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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.

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

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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.

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

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

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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.

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

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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.

Amyloidosis of the thyroid gland: ultrasonographic aspect

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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

Lichen amyloidosis in an unusual location.

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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.

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.

Amyloidosis of the renal pelvis presenting as flank pain

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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.

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

Primary localised cutaneous amyloidosis - a systematic review

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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...

Diagnostic and therapeutic approach of systemic amyloidosis

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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

Clinical approach of patients with systemic amyloidosis

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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

Cardiac amyloidosis detection with pyrophosphate-99mTc scintigraphy

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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

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

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.

[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.

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

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

Dysautonomias in Amyloidosis: : Need for an interdisciplinary approach

NARCIS (Netherlands)

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

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

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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.

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

Dialysis-related amyloidosis: challenges and solutions

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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

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.)

AA amyloidosis complicating the hereditary periodic fever syndromes.

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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

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.

SECONDARY AMYLOIDOSIS WITH LUNG INVOLVEMENT INA FEMALE PATIENT WITH RHEUMATOID ARTHRITIS

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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.

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

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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

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.

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

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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.

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

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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.

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.

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

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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.

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

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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.

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.

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

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.

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.

Recent Insights into the Pathogenesis of Type AA Amyloidosis

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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.

Ursodeoxycholic acid for treatment of cholestasis in patients with hepatic amyloidosis

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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.

Inhibitory effect of propolis on the development of AA amyloidosis.

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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.

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.

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

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,

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

DEFF Research Database (Denmark)

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...

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.

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

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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.

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.

Clinical outcomes and survival in AA amyloidosis patients

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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

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

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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.

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

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%.

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.

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

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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.

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.

Hereditary Transthyretin Amyloidosis in Eight Chinese Families

Directory of Open Access Journals (Sweden)

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.

Diagnosis of secondary amyloidosis in alkaptonuria.

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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.

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

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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.

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.

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

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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.

Thermal Stability Threshold for Amyloid Formation in Light Chain Amyloidosis

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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.

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.

Activated microglia mediate synapse loss and short-term memory deficits in a mouse model of transthyretin-related oculoleptomeningeal amyloidosis.

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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.

Cancer-testis antigen expression and immunogenicity in AL amyloidosis

International Nuclear Information System (INIS)

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

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

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.

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.

Renal amyloidosis in a child with sickle cell anemia.

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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.

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

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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

{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.

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

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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

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

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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.

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

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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.

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

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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

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

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...

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...

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.

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.

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

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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.

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

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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.

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.

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

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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.

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

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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.

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

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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.

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.

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.)

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.)

Guideline of transthyretin-related hereditary amyloidosis for clinicians

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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.

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

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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

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.

Cardiac amyloidosis

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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

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

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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

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

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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.

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

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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.

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

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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.

Laryngeal amyloidosis with laryngocele: MRI and CT

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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.)

The biodistribution and imaging of a novel amyloid probe 131I-serum amyloid P-component in mice model of amyloidosis

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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)

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

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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.

Amyloidosis in association with spontaneous feline immunodeficiency virus infection.

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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.

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

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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.

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.

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

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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

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.)

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

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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.

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

International Nuclear Information System (INIS)

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

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

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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.

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

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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.

Toxicity of injected 137CsCl in beagle dogs. X

International Nuclear Information System (INIS)

Rebar, A.H.; Hanika-Rebar, C.; Benjamin, S.A.; Boecker, B.B.; Jones, R.K.; McClellan, R.O.; Pickrell, J.A.

1977-01-01

Studies on the metabolism, dosimetry and effects of intravenously administered 137 CsCl in the Beagle dog are being conducted to aid in assessing the biologic consequences of exposure to 137 Cs such as occurs in the event of certain nuclear accidents. Effects of the chronic, relatively uniform whole-body exposure produced by 137 Cs are being compared with other diverse radiation dose patterns resulting from inhalation of radioactive aerosols. Sixty-six dogs were entered into the study: six with a mean initial 137 Cs body burden of 3780 μCi/kg and five groups of 12 dogs each with mean initial 137 Cs body burdens of 2820, 1940, 1420, 970 and 0 μCi/kg. Eighteen of the dogs injected with 137 CsCl have died: 11 from 19 to 81 days after injection and cumulative whole-body doses of 860 to 1400 rads died with severe bone marrow damage and resultant pancytopenia; two at 2471 days and at 2707 days after injection and cumulative doses of 690 and 1800 rads with a neurofibrosarcoma of the liver and a mast cell tumor of the skin; five from 693 to 3162 days after injection and cumulative doses of 840 to 2000 rads with shock, aspiration pneumonia, severe arthritis, renal amyloidosis and congestive heart failure. Three control dogs died; one at 647 days with auto-immune hemolytic anemia, one at 2442 days with renal amyloidosis and one at 3088 days with disseminated complex mammary adenocarcinoma. Serial observations are continuing on the 36 137 CsCl dogs and nine control dogs. The 137 CsCl dogs now alive have cumulative whole-body doses of 550 to 2200 rads from 2777 to 3031 days after injection

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.

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

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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.

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.

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.

Surgical management of macroglossia secondary to amyloidosis.

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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.

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

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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.

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.

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

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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.

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

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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....

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

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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

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

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

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.

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.

Cardioverter-defibrillator implantation in myeloma-associated cardiac amyloidosis.

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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.

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

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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.

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

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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.

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

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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.

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

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)

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.)

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

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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.)

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

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

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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.

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.

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 ...

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

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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

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 ...

Long-term follow-up of high-pressure injection injuries to the hand

NARCIS (Netherlands)

Wieder, Anat; Lapid, Oren; Plakht, Ygal; Sagi, Amiram

2006-01-01

High-pressure injection injury is an injury caused by accidental injection of substances by industrial equipment. This injury may have devastating sequelae. The goal of this study was to assess the long-term outcome of high-pressure injection injury to the hand. In this historical prospective study,

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.

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

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...

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.

Amiloidosis bucal Oral amyloidosis

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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.

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

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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.

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

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.

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

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...

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

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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.

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.

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.

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...

The value of short-term pain relief in predicting the long-term outcome of 'indirect' cervical epidural steroid injections.

Science.gov (United States)

Joswig, Holger; Neff, Armin; Ruppert, Christina; Hildebrandt, Gerhard; Stienen, Martin Nikolaus

2018-05-01

The predictive value of short-term arm pain relief after 'indirect' cervical epidural steroid injection (ESI) for the 1-month treatment response has been previously demonstrated. It remained to be answered whether the long-term response could be estimated by the early post-interventional pain course as well. Prospective observational study, following a cohort of n = 45 patients for a period of 24 months after 'indirect' ESI for radiculopathy secondary to a single-level cervical disk herniation (CDH). Arm and neck pain on the visual analog scale (VAS), health-related quality of life with the Short Form-12 (SF-12), and functional outcome with the Neck Pain and Disability (NPAD) Scale were assessed. Any additional invasive treatment after a single injection (second injection or surgery) defined treatment outcome as 'non-response'. At 24 months, n = 30 (66.7%) patients were responders and n = 15 (33.3%) were non-responders. Non-responders exited the follow-up at 1 month (n = 10), at 3 months (n = 4), and at 6 months (n = 1). No patients were injected again or operated on between the 6- and 24-month follow-up. Patients with favorable treatment response at 24 months had significantly lower VAS arm pain (p  50% short term pain reduction was not a reliable predictor of the 24-month responder status. SF-12 and NPAD scores were better among treatment responders in the long term. Patients who require a second injection or surgery after 'indirect' cervical ESI for a symptomatic CDH do so within the first 6 months. Short-term pain relief cannot reliably predict the long-term outcome.

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.

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.

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

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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.

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.

Riboflavin laurate nanosuspensions as an intramuscular injection for long-term riboflavin supplementation.

Science.gov (United States)

Du, Lina; Li, Guanglong; Jin, Yiguang; Wang, Lin; Xu, Qishou; Dong, Junxing

2013-06-25

The aim of this study was to prepare riboflavin laurate (RFL) nanosuspensions as an intramuscular injection for long-term riboflavin supplementation. Stable RFL nanosuspensions were obtained by injecting RFL/poloxamer solution in N,N-dimethyl formamide into a trehalose solution. Long soft nanostructures initially appeared and then tube-like rigid nanostructures were obtained after removal of solvents according to the transmission electron microscopic images. The nanosuspensions had narrow size distribution and the mean size was about 300 nm. Molecular self-assembly of RFL may drive the formation of nanostructures. RFL formed a monolayer at the air/water interface and poloxamer 188 could insert into the monolayer. The nanosuspensions were intramuscularly injected into rats to provide long-term riboflavin supplementation for more than 30 days in light of body weight, food intake, and urinary riboflavin. The nanosuspensions were also used to resist the riboflavin deficiency induced by methotrexate chemotherapy. RFL nanosuspensions are a promising nanomedicine for long-term riboflavin supplementation. Copyright © 2013 Elsevier B.V. All rights reserved.

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

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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.

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

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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.

Skin deposits in hereditary cystatin C amyloidosis

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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...

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

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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

Short-term outcome of fluoroscopic-guided steroid injection therapy of lumber facet cyst-induced radicular pain

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Kwon, Mi Ri; Kwon, Jong Won; Lee, Jong Seo; Kim, Eu Sang [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2015-04-15

To determine the short-term effect of fluoroscopic-guided steroid injection therapy of lumbar facet cyst-induced radicular pain. Seventeen patients with radiculopathy due to lumbar synovial cysts, who were treated with fluoroscopically guided injection, were retrospectively evaluated. All plain radiographic images and MR images before the therapy were reviewed. Five patients underwent only the facet joint injection, whereas twelve patients underwent the facet joint injection with perineural injection therapy. The clinical course of pain was evaluated on the first follow-up after therapy. Effective pain relief was achieved in 11 (64.7%) of the 17 patients. Among 12 patients who underwent facet joint injection with perineural injection, 9 patients (75%) had an effective pain relief. Of 5 patients, 2 (40%) patients only took the facet joint injection and had an effective pain relief. Fluoroscopic-guided steroid injection therapy shows a good short-term effect in patients with symptomatic lumbar facet joint synovial cysts.

Localized amyloidosis of the stomach mimicking a superficial gastric cancer.

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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.

Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis.

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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.

Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis

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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

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

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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

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

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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

Rheumatic polymyalgia like presentation of multiple myeloma and amyloidosis

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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

PLASMA CYTOKINES LEVELS IN PATIENTS UNDERGOING LONG-TERM HAEMODIALYSIS

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D. S. Polyakov

2011-01-01

Full Text Available Аbstract.  Patients  with  end-stage  renal  disease  need  their  kidney  functions  to  be  replaced.  Chronic haemodialysis represents a most common method of such substitution treatment. This procedure results in successful survival of such patients for years. Chronic haemodialysis is accompanied by a complication which is known as β2-microglobulin amyloidosis. In this case, amyloid substance consisting of β2-microglobulin (β2-MG accumulates in bones, ligaments and joints. Biological causes of β2-MG amyloidosis are still not established. To elucidate the role of inflammation in the pathogenesis of β2-MG amyloidosis, the levels of  IL-2,  IL-4,  IL-6,  IL-8,  IL-10,  GM-CSF,  IFNγ, TNFα were quantified in plasma of patients undergoing  long-term haemodialysis. Mean amounts of all the mentioned cytokines in haemodialysis patients proved to be significantly higher than in control group consisting of healthy subjects. When comparing a group receiving standard  dialysis  procedure  versus  a  subgroup  receiving  haemodiafiltration,  a  single  reliable  difference  was revealed for GM-CSF levels (p < 0.04, without any differences shown for other cytokines. With increasing terms of chronic haemodialysis, the levels of IL-2, IL-4, IL-6, IL-8, GM-CSF, IFNγ, TNFα were increased, or, at least, they did not decrease. After three years of dialysis, IL-10 concentrations were statistically indistinguishable from normal levels. In patients undergoing haemodiafiltration, plasma levels of IL-2, IL-4, IL-8, GM-CSF, IFNγ, TNFα did not drop with increasing terms of dialysis. The levels of IL-6 and IL-10 decreased after three years of dialysis, to near-normal levels.In general, these results suggest that IL-10 and IL-6 may be regarded as candidates for further studies as potential markers of β2-microglobulin amyloidosis. (Med. Immunol., 2011, vol. 13, N 2-3, pp 211-218

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.

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

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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.)

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.

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

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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.

Fluoroscopically Guided Sacroiliac Joint Injections: Comparison of the Effects of Intraarticular and Periarticular Injections on Immediate and Short-Term Pain Relief.

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Nacey, Nicholas C; Patrie, James T; Fox, Michael G

2016-11-01

The purpose of this study was to determine whether intraarticular sacroiliac joint injections provide greater immediate and short-term pain relief than periarticular sacroiliac joint injections do. The records of all fluoroscopically guided sacroiliac joint injections performed over a 4-year period were identified. Patients who received an injection of 0.5 mL of bupivacaine and 0.5 mL (20 mg) of triamcinolone and who had preinjection, immediate, and 1-week postinjection pain scores (0-10 numeric scale) were included. Images from the procedures were retrospectively reviewed by two musculoskeletal radiologists to determine intraarticular or periarticular administration of the injection with discrepancies resolved by consensus. One hundred thirteen injections in 99 patients (65 women, 34 men; mean age, 59.4 years) met the inclusion criteria. There were 55 intraarticular and 58 periarticular injections. The mean preinjection, immediate, and 1-week postinjection pain scores for the intraarticular injections were 6.0, 1.6, and 4.1 and for the periarticular injections were 6.1, 2.0, and 4.2. The mean immediate and 1-week postinjection pain reduction were statistically significant in both groups (p sacroiliac joint injections provide statistically significant immediate and 1-week postinjection pain relief, no significant difference in the degree of pain relief achieved with intraarticular and periarticular injections was noted.

Anisomycin Injection in Area CA3 of the Hippocampus Impairs Both Short-Term and Long-Term Memories of Contextual Fear

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Remaud, Jessica; Ceccom, Johnatan; Carponcy, Julien; Dugué, Laura; Menchon, Gregory; Pech, Stéphane; Halley, Helene; Francés, Bernard; Dahan, Lionel

2014-01-01

Protein synthesis is involved in the consolidation of short-term memory into long-term memory. Previous electrophysiological data concerning LTP in CA3 suggest that protein synthesis in that region might also be necessary for short-term memory. We tested this hypothesis by locally injecting the protein synthesis inhibitor anisomycin in hippocampal…

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

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

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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.

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

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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.

Short-term cessation of sex work and injection drug use: evidence from a recurrent event survival analysis.

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Gaines, Tommi L; Urada, Lianne A; Martinez, Gustavo; Goldenberg, Shira M; Rangel, Gudelia; Reed, Elizabeth; Patterson, Thomas L; Strathdee, Steffanie A

2015-06-01

This study quantitatively examined the prevalence and correlates of short-term sex work cessation among female sex workers who inject drugs (FSW-IDUs) and determined whether injection drug use was independently associated with cessation. We used data from FSW-IDUs (n=467) enrolled into an intervention designed to increase condom use and decrease sharing of injection equipment but was not designed to promote sex work cessation. We applied a survival analysis that accounted for quit-re-entry patterns of sex work over 1-year stratified by city, Tijuana and Ciudad Juarez, Mexico. Overall, 55% of participants stopped sex work at least once during follow-up. Controlling for other characteristics and intervention assignment, injection drug use was inversely associated with short-term sex work cessation in both cities. In Ciudad Juarez, women receiving drug treatment during follow-up had a 2-fold increase in the hazard of stopping sex work. In both cities, income from sources other than sex work, police interactions and healthcare access were independently and significantly associated with shorter-term cessation. Short-term sex work cessation was significantly affected by injection drug use. Expanded drug treatment and counseling coupled with supportive services such as relapse prevention, job training, and provision of alternate employment opportunities may promote longer-term cessation among women motivated to leave the sex industry. Copyright © 2015 Elsevier Ltd. All rights reserved.

Neuron and neuroblast numbers and cytogenesis in the dentate gyrus of aged APPswe/PS1dE9 transgenic mice: Effect of long-term treatment with paroxetine.

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Olesen, Louise Ørum; Sivasaravanaparan, Mithula; Severino, Maurizio; Babcock, Alicia A; Bouzinova, Elena V; West, Mark J; Wiborg, Ove; Finsen, Bente

2017-08-01

Altered neurogenesis may influence hippocampal functions such as learning and memory in Alzheimer's disease. Selective serotonin reuptake inhibitors enhance neurogenesis and have been reported to reduce cerebral amyloidosis in both humans and transgenic mice. We have used stereology to assess the longitudinal changes in the number of doublecortin-expressing neuroblasts and number of granular neurons in the dentate gyrus of APP swe /PS1 dE9 transgenic mice. Furthermore, we investigated the effect of long-term paroxetine treatment on the number of neuroblasts and granular neurons, hippocampal amyloidosis, and spontaneous alternation behaviour, a measure of spatial working memory, in transgenic mice. We observed no difference in granular neurons between transgenic and wild type mice up till 18months of age, and no differences with age in wild type mice. The number of neuroblasts and the performance in the spontaneous alternation task was reduced in aged transgenic mice. Paroxetine treatment from 9 to 18months of age reduced hippocampal amyloidosis without affecting the number of neuroblasts or granular neurons. These findings suggest that the amyloidosis affects the differentiation of neuroblasts and spatial working memory, independent of changes in total granular neurons. Furthermore, while long-term paroxetine treatment may be able to reduce hippocampal amyloidosis, it appears to have no effect on total number of granular neurons or spatial working memory. Copyright © 2017 Elsevier Inc. All rights reserved.

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

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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.

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.

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

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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

Pathology of AA amyloidosis in domestic sheep and goats.

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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.

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

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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.

Comparison of long-term voice outcomes after vocal fold augmentation using autologous fat injection by direct microlaryngoscopy versus office-based calcium hydroxylapatite injection.

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Zeleník, Karol; Walderová, Radana; Kučová, Hana; Jančatová, Debora; Komínek, Pavel

2017-08-01

The objective is to compare the long-term voice outcomes of vocal fold augmentation (VFA) using autologous fat injection via direct microlaryngoscopy versus office-based calcium hydroxylapatite (CaHA) injection. Patients with glottal insufficiency and a gap no greater than 3 mm caused by unilateral vocal fold paralysis or vocal fold atrophy were prospectively recruited to the study from September 2012 to September 2015. From September 2012 to May 2014, VFA was only performed using autologous fat via direct microlaryngoscopy under general anesthesia (N = 14). From May 2014 to September 2015, VFA was performed as an office-based procedure using a transoral approach to inject CaHA (N = 17). Videolaryngostroboscopic evaluation, subjective satisfaction with voice, voice handicap index (VHI), and maximal phonation time (MPT) were analyzed pre-injection and 12 months after VFA. A total of 31 patients were analyzed. One year after VFA, 67.8% of the patients were satisfied with their voice, with no significant difference between groups (P = 0.247). The mean improvement in VHI in the autologous fat group was 31.6 ± 16.82 versus 35 ± 27.24 in the CaHA group (P = 0.664). MPT improvement was also similar in the two groups: 5.5 ± 2.52 for the autologous fat group versus 6.0 ± 3.98 for the CaHA group (P = 0.823). Both autologous fat injection via direct microlaryngoscopy and office-based CaHA injection have good long-term results. There were no differences in the treatment results of the two procedures 1 year after injection.

Long-term CO2 injection and its impact on near-surface soil microbiology.

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Gwosdz, Simone; West, Julia M; Jones, David; Rakoczy, Jana; Green, Kay; Barlow, Tom; Blöthe, Marco; Smith, Karon; Steven, Michael; Krüger, Martin

2016-12-01

Impacts of long-term CO 2 exposure on environmental processes and microbial populations of near-surface soils are poorly understood. This near-surface long-term CO 2 injection study demonstrated that soil microbiology and geochemistry is influenced more by seasonal parameters than elevated CO 2 Soil samples were taken during a 3-year field experiment including sampling campaigns before, during and after 24 months of continuous CO 2 injection. CO 2 concentrations within CO 2 -injected plots increased up to 23% during the injection period. No CO 2 impacts on geochemistry were detected over time. In addition, CO 2 -exposed samples did not show significant changes in microbial CO 2 and CH 4 turnover rates compared to reference samples. Likewise, no significant CO 2 -induced variations were detected for the abundance of Bacteria, Archaea (16S rDNA) and gene copy numbers of the mcrA gene, Crenarchaeota and amoA gene. The majority (75%-95%) of the bacterial sequences were assigned to five phyla: Firmicutes, Proteobacteria, Actinobacteria, Acidobacteria and Bacteroidetes The majority of the archaeal sequences (85%-100%) were assigned to the thaumarchaeotal cluster I.1b (soil group). Univariate and multivariate statistical as well as principal component analyses showed no significant CO 2 -induced variation. Instead, seasonal impacts especially temperature and precipitation were detected. © FEMS 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

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

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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.

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

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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

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.

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.

Short-term outcome after intravitreal ranibizumab injections for the treatment of retinopathy of prematurity.

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Castellanos, María Ana Martínez; Schwartz, Shulamit; García-Aguirre, Gerardo; Quiroz-Mercado, Hugo

2013-07-01

To evaluate ocular outcome in premature infants treated with intravitreal ranibizumab injections for retinopathy of prematurity (ROP) over a period of 3 years. An interventional case series. Premature infants with high-risk prethreshold or threshold ROP with plus disease received an off label monotherapy with intravitreal injections of ranibizumab. The primary outcome was treatment success defined as regression of neovascularisation (NV) and absence of recurrence. The secondary outcomes were ocular and systemic adverse events and visual acuity. Six eyes were included in the study and treated with intravitreal injections of ranibizumab. All showed complete resolution of NV after a single injection. The anti-angiogenic intravitreal injections allowed for continued normal vessel growth into the peripheral retina, without any signs of disease recurrence or progression during the follow up period. No ocular or systemic adverse effects were observed. Three years of follow up in a small series suggest that intravitreal ranibizumab injections for ROP result in apparently preserved ocular outcome. Further large scale studies are needed to address the long-term safety and efficacy.

Long-term continuous energy injection in the afterglow of GRB 060729

International Nuclear Information System (INIS)

Xu Ming; Huang Yongfeng; Lu Tan

2009-01-01

A long plateau phase and an amazing level of brightness have been observed in the X-ray afterglow of GRB 060729. This peculiar light curve is likely due to long-term energy injection in external shock. Here, we present a detailed numerical study of the energy injection process of magnetic dipole radiation from a strongly magnetized millisecond pulsar and model the multi-band afterglow observations. It is found that this model can successfully explain the long plateaus in the observed X-ray and optical afterglow light curves. The sharp break following the plateaus could be due to the rapid decline of the emission power of the central pulsar. At an even later time (∼ 5 x 10 6 s), an obvious jet break appears, which implies a relatively large half opening angle of θ ∼ 0.3 for the GRB ejecta. Due to the energy injection, the Lorentz factor of the outflow is still larger than two even at 10 7 s after the GRB trigger, making the X-ray afterglow of this burst detectable by Chandra even 642 d after the burst.

Intrathecal injection of naked plasmid DNA provides long-term expression of secreted proteins.

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Hughes, Travis S; Langer, Stephen J; Johnson, Kirk W; Chavez, Raymond A; Watkins, Linda R; Milligan, Erin D; Leinwand, Leslie A

2009-01-01

Therapeutic benefit has been reported to result from intrathecal (i.t.) injection of transgene vectors, including naked DNA. However, most studies using naked DNA have measured only the transgene expression of intracellular proteins. Here we demonstrate that i.t. injection of naked DNA can result in long-term expression of secreted proteins. Plasmids expressing either secreted alkaline phosphatase (SEAP) or human interleukin-10 (hIL-10) were injected into the i.t. space in rats, and transgene products were repeatedly measured in the cerebrospinal fluid (CSF). Both SEAP and hIL-10 were maximal at 1 and 2 days after the injection and still detectable at 4 months. The utilization of a plasmid having two features that are hypothesized to increase gene expression (matrix attachment regions (MARs) and lack of CpG dinucleotides) resulted in a significant increase in gene expression. Reinjection of SEAP or hIL-10 plasmids after 4 months significantly increased protein levels at 1 and 14 days after the reinjection. SEAP was uniformly distributed between the DNA delivery site (approximately vertebral level T13) and the lumbar puncture site (L5/L6 inter-vertebral space), was reduced at the cisterna magna, and was detectable, though at much lower levels, in serum. These data suggest that naked DNA has the potential to be used as a therapeutic tool for applications that require long-term release of transgenes into the CSF.

Radiological features of osteoarticular changes in long-term (15 years) hemodialysis patients

International Nuclear Information System (INIS)

Orzincolo, C.; Ghedini, M.; Cardona, P.; Bedani, P.L.; Storari, A.; Scutellari, P.N.; Cavallari, L.

1991-01-01

Osteoarticular complications, which are characterized by osseous pain, pathologic fractures, and decreased articular mobility, represent one of the mayor problems affecting long-term (over 15 years) hemodialysis patients. These changes seem to have a multifactorial etiology; they include osteomalacia, secondary hyperparathyroidism, and dialysis-related amyloidosis. Ten patients (5 males and 5 females, mean age 55± 7 years) on long-term (over 15 years) hemodialysis were submitted to X-ray examinations of the skull, spine, shoulders, wrists, pelvis, and knees. Serum calcium, phosphorous, parathyroid hormone, alkaline phosphatase, and basal aluminium levels were also calculated. Osteopenia was demonstrated in all patients. Seven of them had alterations due to hyperparathyroidism. Six patients exhibited signs related to dialysis spondyloarthropathy; in 9 cases amyloid lesions, geodes, and erosions were present in wrists, humeral heads, or hips. One patients exhibited osteomalacic changes. Most long-terms dialysis patients presented multifactorial osteoarticular changes due to hyperparathyroidism, osteomalacia and dialysis-related amyloidosis. Clinical sympoms and decreased articular mobility appeared to be due mainly to amyloid osteoarthropathy

Laryngeal electromyography-guided hyaluronic acid vocal fold injection for unilateral vocal fold paralysis: a prospective long-term follow-up outcome report.

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Wang, Chen-Chi; Chang, Ming-Hong; Jiang, Rong-San; Lai, Hsiu-Chin; De Virgilio, Armando; Wang, Ching-Ping; Wu, Shang-Heng; Liu, Shi-An; Liang, Kai-Li

2015-03-01

Unilateral vocal fold paralysis (UVFP) is a common voice disorder that may cause glottal closure insufficiency with hoarseness of voice. Laryngeal electromyography (LEMG)-guided hyaluronic acid vocal fold (VF) injection has been proposed as a treatment option to improve glottal closure with a satisfactory short-term effect. To our knowledge, this study reports the first long-term follow-up result of this treatment modality. To present the long-term treatment results of LEMG-guided hyaluronic acid VF injection for UVFP. Prospective study of the treatment results of 74 patients who received LEMG-guided hyaluronic acid VF injection for UVFP at a tertiary referral medical center from March 2010 to February 2013. In the office-based procedure, 1.0 mL of hyaluronic acid was injected via a 26-gauge monopolar injectable needle electrode into paralyzed thyroarytenoid muscles by LEMG guidance. Various glottal closure evaluations such as normalized glottal gap area, maximal phonation time, phonation quotient, mean airflow rate, perceptual GRBAS (grade, roughness, breathiness, asthenia, strain) scale, and Voice Handicap Index were compared before and after injection using the nonparametric Wilcoxon signed rank test within 1 month, at 6 months, and at the last follow-up examination. Sixty patients had been followed up for at least 6 months. Forty-four patients received only 1 injection, and 16 patients received repeated injections (2 injections for 13 patients and 3 for 3 patients). All the glottal closure parameters improved significantly (P injection and 16 (22%) who received repeated injections did not require another treatment after long-term follow-up. Laryngeal electromyography-guided hyaluronic acid VF injection is an option for treating UVFP with satisfactory results.

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

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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.

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

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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.

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

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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

Polydimethylsiloxane Injection Laryngoplasty for Unilateral Vocal Fold Paralysis: Long-Term Results.

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Mattioli, Francesco; Bettini, Margherita; Botti, Cecilia; Busi, Giulia; Tassi, Sauro; Malagoli, Andrea; Molteni, Gabriele; Trebbi, Marco; Luppi, Maria Pia; Bergamini, Giuseppe; Presutti, Livio

2017-07-01

To analyze the long-term objective, perceptive, and subjective outcomes after endoscopic polydimethylsiloxane (PDMS) injection laryngoplasty in unilateral vocal fold paralysis. A retrospective study carried out between January 2008 and January 2012. Head and Neck Department, University Hospital of Modena, Modena, Italy. This was a retrospective analysis of 26 patients with unilateral vocal fold paralysis who underwent endoscopic injection of PDMS under general anesthesia. A voice evaluation protocol was performed for all patients, which included videolaryngostroboscopy, maximum phonation time, fundamental frequency, analysis of the harmonic structure of the vowel /a/ and the word /aiuole/, Grade of Dysphonia, Instability, Roughness, Breathiness, Asthenia, and Strain scale, and Voice Handicap Index. The protocol was performed before surgery, in the immediate postoperative period, and at least 3 years after surgery. The mean follow-up period was 73 months (range 39-119 months). The statistical analysis showed a significant improvement (P injection laryngoplasty with PDMS guarantees long-lasting effects over time. No complications were reported in our series. Injection laryngoplasty with PDMS can be considered to be a minimally invasive and safe technique for the treatment of unilateral vocal fold paralysis. Moreover, it allows very good and stable results to be obtained over time, avoiding repeated treatments and improving the quality of life of the patients. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

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

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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

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.

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

Short-term intraocular pressure changes after intravitreal injection of bevacizumab in diabetic retinopathy patients

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Farhood QK

2014-03-01

Full Text Available Qasim Kadhim Farhood,1 Sinan Mohammad Twfeeq21College of Medicine, Babylon University, Babylon; 2Al-Jumhori teaching hospital, Mosul, IraqBackground: This study examined the changes in short-term intraocular pressure (IOP in a prospective series of patients undergoing intravitreal bevacizumab injection. The aim was to evaluate the frequency and predictive factors related to intraocular pressure (IOP elevation in patients receiving intravitreal bevacizumab.Patients and methods: This study included 52 patients with diabetic retinopathy between 28 to 75 years of age with a mean age of 51 years; 30 (58% were females, and 22 (42% were males. All patients received bevacizumab (1.25 mg/0.05 mL injected intravitreally in a standard fashion between May 2012 to February 2013 in the AL-Jumhoury teaching hospital. IOP was measured at baseline, 5, 10, and 30 minutes after injection using Goldman applanation tonometry.Statistics: Data were analyzed using the SPSS v.12.0 for windows. Basic, demographic, and clinical data were analyzed using means, proportions, and appropriate 95% confidence intervals (CIs.Results: Most patients (85% were diagnosed with proliferative diabetic retinopathy, while 15% presented with diabetic macular edema. The mean IOP values at baseline, 5, 10, and 30 minutes after injection were 14.0 mmHg (95% CI 13.4–14.7, 36.1 mmHg (95% CI 33.5–38.6, 25.7 mmHg (95% CI 23.8–27.5, and 15.5 mmHg (95% CI 12.4–16.51, respectively. Regression analysis showed a trend toward phakic patients having higher IOP at 30 minutes.Conclusion: Intravitreal injection of bevacizumab is safe with respect to short-term IOP changes, as almost all IOP returned to a safe range (<25 mmHg within 30 minutes. Elevated IOP 30 minutes after injection only occurs rarely, so routine prophylactic use of anti-glaucoma medication is not indicated.Keywords: intravitreal injection, bevacizumab, intraocular pressure, Goldmann applanation tonometry

Jet flow analysis of liquid poison injection in a CANDU reactor using source term

International Nuclear Information System (INIS)

Chae, Kyung Myung; Choi, Hang Bok; Rhee, Bo Wook

2001-01-01

For the performance analysis of Canadian deuterium uranium (CANDU) reactor shutdown system number 2 (SDS2), a computational fluid dynamics model of poison jet flow has been developed to estimate the flow field and poison concentration formed inside the CANDU reactor calandria. As the ratio of calandria shell radius over injection nozzle hole diameter is so large (1055), it is impractical to develop a full-size model encompassing the whole calandria shell. In order to reduce the model to a manageable size, a quarter of one-pitch length segment of the shell was modeled using symmetric nature of the jet; and the injected jet was treated as a source term to avoid the modeling difficulty caused by the big difference of the hole sizes. For the analysis of an actual CANDU-6 SDS2 poison injection, the grid structure was determined based on the results of two-dimensional real- and source-jet simulations. The maximum injection velocity of the liquid poison is 27.8 m/s and the mass fraction of the poison is 8000 ppm (mg/kg). The simulation results have shown well-established jet flow field. In general, the jet develops narrowly at first but stretches rapidly. Then, the flow recirculates a little in r-x plane, while it recirculates largely in r-θ plane. As the time goes on, the adjacent jets contact each other and form a wavy front such that the whole jet develops in a plate form. his study has shown that the source term model can be effectively used for the analysis of the poison injection and the simulation result of the CANDU reactor is consistent with the model currently being used for the safety analysis. In the future, it is strongly recommended to analyze the transient (from helium tank to injection nozzle hole) of the poison injection by applying Bernoulli equation with real boundary conditions

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.

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.

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

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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.

Evaluation of the long-term efficacy of CT-guided epidural steroid injection for the treatment of sciatica

International Nuclear Information System (INIS)

Tong Guohai; Wang Wei; Chen Wei; Chen Kemin

2005-01-01

Objective: To evaluate the long-term efficacy of CT-guided epidural steroid injection for the treatment of sciatica. Methods: CT-guided epidural steroid injection was performed in 180 patients with sciatica from May 1998 to March 2004, and all patients had failure to previous conservative treatment. Visual analogue scale was used to evaluate the pain of the patient before and after the treatment. Results: Follow-up was taken for 112 cases during 1-6 years after the treatment, 89 patients (79.5%) had successful long-term outcome and 80 patients (71.4%) were satisfied. Conclusions: CT-guided epidural steroid injection can reduce low back pain and radical pain. It should be preferentially considered as the first choice when conservative treatments are failed. (authors)

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.

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

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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.

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.

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.

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.

Long-term Dose Stability of OnabotulinumtoxinA Injection for Adductor Spasmodic Dysphonia: A 19-Year Single Institution Experience

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Paul Paddle

2017-11-01

Full Text Available ObjectivesAdductor spasmodic dysphonia (AdSD is a focal dystonia predominantly involving the laryngeal adductor muscles. AdSD is reported to be a largely non-progressive neurological disorder, though fluctuations in symptom severity do occur. Repeated laryngeal onabotulinumtoxinA (BTX-A injections are the primary management for AdSD. A number of studies have demonstrated long-term dose stability as evidence of this long-term disease stability.MethodsA retrospective review was performed on all patients undergoing BTX-A injections for AdSD from April 1994 to September 2013 by a single laryngologist at a tertiary referral laryngology center. Patient demographics, injection doses, use of diazepam and/or lidocaine, and self-reported vocal function were recorded. Multiple linear regression analyses were performed.Results83 patients underwent a total of 1,168 injections over 19 years. The mean starting dose was 2.35 MU (0.79 SD. The mean long-term dose was 2.36 MU (0.79 SD. After adjusting for confounders, the change in the relative dose of BTX-A, with every year elapsed since initial dose was 0.13% (95% confidence interval −0.31 to 0.57%, p = 0.568.ConclusionBTX-A dose is stable over time in our large cohort of patients treated with bilateral thyroarytenoid injections for AdSD.

Long-term persistence with injectable therapy in relapsing-remitting multiple sclerosis: an 18-year observational cohort study.

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Simon Zhornitsky

Full Text Available Disease modifying therapies (DMTs reduce the frequency of relapses and accumulation of disability in multiple sclerosis (MS. Long-term persistence with treatment is important to optimize treatment benefit. This long-term, cohort study was conducted at the Calgary MS Clinic. All consenting adults with relapsing-remitting MS who started either glatiramer acetate (GA or interferon-β 1a/1b (IFN-β between January 1st, 1996 and July 1st, 2011 were included. Follow-up continued to February 1st, 2014. Time-to-discontinuation of the initial and subsequently-prescribed DMTs (switches was analysed using Kaplan-Meier survival analyses. Group differences were compared using log-rank tests and multivariable Cox regression models. Analysis included 1471 participants; 906 were initially prescribed GA and 565 were initially prescribed IFN-β. Follow-up information was available for 87%; 29 (2% were lost to follow-up and 160 (11% moved from Southern Alberta while still using DMT. Median time-to-discontinuation of all injectable DMTs was 11.1 years. Participants with greater disability at treatment initiation, those who started treatment before age 30, and those who started between 2006 and 2011 were more likely to discontinue use of all injectable DMTs. Median time-to-discontinuation of the initial DMT was 8.6 years. Those initially prescribed GA remained on treatment longer. Of 610 participants who discontinued injectable DMT, 331 (54% started an oral DMT, or a second-line DMT, or resumed injectable DMT after 90 days. Persistence with injectable DMTs was high in this long-term population-based study. Most participants who discontinued injectable DMT did not remain untreated. Further research is required to understand treatment outcomes and outcomes after stopping DMT.

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.

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

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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

Long-term follow-up after urethral injection with polyacrylamide hydrogel for female stress incontinence

DEFF Research Database (Denmark)

Mouritsen, Lone; Lose, Gunnar; Møller-Bek, Karl

2014-01-01

Urethral injection therapy for treatment of stress urinary incontinence has been in use for years, but only a few long-term follow-up studies have been published. Twenty-five women, injected with polyacrylamide hydrogel 8 years earlier, were invited for follow-up. Twenty-four could be contacted; 15...... had had no further treatment, seven had been re-operated with placement of mid-urethral slings, and two had been re-injected with polyacrylamide hydrogel. Eleven women attended for objective examination; all non-attenders were interviewed by telephone. Subjectively, in 44% the stress incontinence...... was cured or much improved, with a positive outcome according to the King's Health Questionnaire. Objectively, all patients had visible polyacrylamide hydrogel deposits on vaginal ultrasonography. No local adverse reactions were seen in the vaginal mucosa. The results of a later mid-urethral sling were...

Injection Laryngoplasty Materials

OpenAIRE

Haldun Oðuz

2013-01-01

Injection laryngoplasty is one of the treatment options for voice problems. In the recent years, more safe and more biocompatible injection materials are available on the market. Long and short term injection materials are discussed in this review.

Irreversible muscle damage in bodybuilding due to long-term intramuscular oil injection.

Science.gov (United States)

Banke, I J; Prodinger, P M; Waldt, S; Weirich, G; Holzapfel, B M; Gradinger, R; Rechl, H

2012-10-01

Intramuscular oil injections generating slowly degrading oil-based depots represent a controversial subject in bodybuilding and fitness. However they seem to be commonly reported in a large number of non-medical reports, movies and application protocols for 'site-injections'. Surprisingly the impact of long-term (ab)use on the musculature as well as potential side-effects compromising health and sports ability are lacking in the medical literature. We present the case of a 40 year old male semi-professional bodybuilder with systemic infection and painful reddened swellings of the right upper arm forcing him to discontinue weightlifting. Over the last 8 years he daily self-injected sterilized sesame seed oil at numerous intramuscular locations for the purpose of massive muscle building. Whole body MRI showed more than 100 intramuscular rather than subcutaneous oil cysts and loss of normal muscle anatomy. 2-step septic surgery of the right upper arm revealed pus-filled cystic scar tissue with the near-complete absence of normal muscle. MRI 1 year later revealed the absence of relevant muscle regeneration. Persistent pain and inability to perform normal weight training were evident for at least 3 years post-surgery. This alarming finding indicating irreversible muscle mutilation may hopefully discourage people interested in bodybuilding and fitness from oil-injections. The impact of such chronic tissue stress on other diseases like malignancy remains to be determined. © Georg Thieme Verlag KG Stuttgart · New York.

Safety of radiofrequency treatment over human skin previously injected with medium-term injectable soft-tissue augmentation materials: a controlled pilot trial.

Science.gov (United States)

Alam, Murad; Levy, Ross; Pajvani, Urvi; Pavjani, Urvi; Ramierez, James A; Guitart, Joan; Veen, Heather; Gladstone, Hayes B

2006-03-01

Several soft-tissue augmentation materials are now available for reduction of nasolabial fold creases and perioral rhytides. Nasolabial folds and perioral rhytides can also be improved by skin tightening delivered by non-ablative radiofrequency (RF) treatment. The purpose of this study was to assess the safety of RF treatment over skin areas recently injected with medium-term injectable soft-tissue augmentation materials. Five subjects were assigned to the experimental arm (augmentation materials plus RF) and one to the control arm (augmentation materials alone). Each subject received injections of 0.3 mL of hyaluronic acid derivative (Restylane) and calcium hydroxylapatite (Radiesse) 3 cm apart on the upper inner arm. Two weeks later, two non-overlapping passes of RF (Thermage ThermaCool TC) were delivered at 63.5 setting with medium-fast 1.5 cm2 tip over injected sites in all of the experimental subjects. Punch skin biopsies were obtained 3 days later from each of the two injection sites on each subject. Light microscopy and digital photomicrographs obtained at low, medium, and high power showed no difference between filler materials in experimental and control subjects. In both cases filler was evident at the deep dermal-subcutaneous junction. Nodule formation, foreign body extravasation, or hemorrhage/clot was not observed grossly or histologically. Subjects and physicians did not report any difference in signs and symptoms between the experimental and control arms. Slightly increased transitory pain was noted when RF was delivered over filler versus over normal skin. Applying RF treatment over the same area 2 weeks after deep dermal injection with hyaluronic acid derivatives or calcium hydroxylapatite does not appear to cause gross morphological changes in the filler material or surrounding skin. Further studies with different parameters are necessary to confirm these findings. 2006 Wiley-Liss, Inc.

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.

Short- and long-term efficacy of intra-articular injections with betamethasone as part of a treat-to-target strategy in early rheumatoid arthritis

DEFF Research Database (Denmark)

Hetland, Merete Lund; Østergaard, Mikkel; Ejbjerg, Bo

2012-01-01

To investigate the short-term and long-term efficacy of intra-articular betamethasone injections, and the impact of joint area, repeated injections, MRI pathology, anticyclic citrullinated peptide (CCP) and immunoglobulin M rheumatoid factor (IgM-RF) status in patients with early rheumatoid arthr...

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.

Vocal Fold Augmentation with Injectable Polycaprolactone Microspheres/Pluronic F127 Hydrogel: Long-Term In Vivo Study for the Treatment of Glottal Insufficiency

Science.gov (United States)

Kwon, Seong Keun; Kim, Hee-Bok; Song, Jae-Jun; Cho, Chang Gun; Park, Seok-Won; Choi, Jong-Sun; Ryu, Junsun; Oh, Se Heang; Lee, Jin Ho

2014-01-01

There is increasing demand for reconstruction of glottal insufficiency. Several injection materials have been examined for this purpose, but all had limitations, such as poor long-term durability, migration from the injection site, inflammation, granuloma formation, and interference with vocal fold vibration due to viscoelastic mismatch. Here, we developed a novel injection material, consisting of polycaprolactone (PCL) microspheres, which exhibits better viscoelasticity than conventional materials, and Pluronic F127 carrier, which decreases the migration of the injection materials. The material was injected into rabbits with glottal insufficiency and compared with the FDA-approved injection material, calcium hydroxylapatite (CaHA). Endoscopic and histological examinations indicated that PCL/Pluronic F127 remained at the injection site with no inflammatory response or granuloma formation, whereas CaHA leaked out and migrated from the injection site. Therefore, vocal fold augmentation was almost completely retained during the 12-month follow-up period in this study. Moreover, induced phonation and high-speed recording of vocal fold vibration showed decreased vocal fold gap area in the PCL/Pluronic F127 group. Our newly developed injection material, PCL/Pluronic F127, permits efficient augmentation of paralyzed vocal fold without complications, a concept that can be applied clinically, as demonstrated by the successful long-term follow-up. PMID:24465582

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

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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.

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

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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.

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

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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.

Shortlasting Unilateral Neuralgiform Conjunctival Injection and Tearing Syndrome: The Term and New View Points

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Antonaci, Fabio; Fredriksen, Torbjørn; Pareja, Juan A.; Sjaastad, Ottar

2018-01-01

A solitary patient with symptoms similar to those of shortlasting unilateral neuralgiform conjunctival injection and tearing (SUNCT) was first mentioned in 1978. The term SUNCT was first used in 1991. SUNCT is an acronym; the “S” signifies “Shortlasting”; the “U” symbolizes “Unilateral”; “N” stands for “Neuralgiform”; the “C” for “Conjunctival injection”; and “T” for “Tearing.” The term short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms were marketed in 2004. The terminology and new view points are discussed and nosography proposal for SUNCT is presented. PMID:29740387

Additional short-term plutonium urinary excretion data from the 1945-1947 plutonium injection studies

International Nuclear Information System (INIS)

Moss, W.D.; Gautier, M.A.

1985-01-01

A review of original injection experimental records at LASL suggest that the power function fit is a good choice to describe early plutonium excretion; however, the later period (300-, 500-, and 1600-day) results along with 10,000 day results show a significant departure from the Langham power function model used to describe long-term Pu excretion. The authors suggest that since the 523- and 1600-day data in question influenced the mathematical development of the Langham power function equation, its use in predicting Pu body burdens from long-term excretion data should be discouraged. 9 references, 2 figures, 2 tables

Long-term efficacy and safety of self-intracavernous injection of prostaglandin E1 for treatment of erectile dysfunction in China.

Science.gov (United States)

He, L; Wen, J; Jiang, X; Chen, H; Tang, Y

2011-06-01

The study evaluated the long-term efficacy and safety profiles of self-intracavernous injection of prostaglandin E1 (PGE1) for erectile dysfunction (ED). Four hundred and sixteen ED patients were treated with self-intracavernous injection of PGE1 from January 1998 to December 2007 in our outpatient service. Follow-up was made to investigate the efficacy and side effects of this treatment. It was found that 261 patients (62.7%) felt satisfied and kept using this treatment due to its advantages of satisfactory efficacy and reasonable expense. Twenty-seven of them (6.5%) got rid of PGE1 treatment after five times injections and did not need any other drugs to maintain satisfactory sexual lives. Two hundred and fourteen (51.4%) patients kept using this treatment for over 1 year, 26 (6.2%) over 5 years, 12 (2.9%) over 8 years and 7 (1.7%) over 10 years. The major complications of self-intracavernous injection of PGE1 include fibrosis of corpus cavernosum (three cases), ecchymosis associated with vascular injury due to injection (23 cases) and pain associated with injection (295 cases). There were no patients displaying priapism. It is concluded that self-intracavernous injection of PGE1 is a safe and effective treatment for ED with various aetiologies and a broad range of severity, and no serious complications were observed after long-term application. © 2011 Blackwell Verlag GmbH.

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)

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.

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

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

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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

, ECV ≥ 44.0% remained prognostic (log rank P = 0.029). Median native T1 (1456 ms) was not prognostic for mortality (Tarone-Ware, P = 0.069). During a short-term follow-up, both ECV and LGE are independently prognostic for mortality in AL amyloidosis. In patients with a similar LGE pattern, ECV remained prognostic. Native T1 was not found to be a prognostic factor.

Short-term disposition of TCDD in rats after iv injection

International Nuclear Information System (INIS)

Ernst, S.W.; Weber, L.W.D.; Rozman, K.; Stahl, B.U.

1990-01-01

Male Sprague-Dawley rats (235-290gr) were injected iv via tail vein with 14 C-TCDD (10 μg/kg, 1 μCi/kg) in a 10% oil/water emulsion (4 ml/kg). 4 rats per group were sacrificed 5, 15 or 45 minutes and 3, 8 or 24 hours after dosing. TCDD attained highest radioactivity levels immediately after injection in blood and in tissues with high blood flow such as lung, spleen, heart, olfactory epithelium (maximum at 5 min post injection), brown adipose tissue and muscle (15 min). At 5 min these tissues accounted for about 30% of total radioactivity. The disposition profile of 14 C-radioactivity was 1st order biphasic with the first phase lasting less than 3 hrs. After this time radioactivity levels in blood were lowest of all tissues. Low-perfused tissues such as skin and white adipose tissue (WAT) displayed distinct uptake phases with peak levels of radioactivity occurring after 8 and 24 hours, respectively. Liver, a major target and storage organ of TCDD toxicity, reached 80% of maximum within 45 min and maximum by 8 hours; about 45% of dose were associated with liver tissue at these time points. This is consistent with a two-phase short-term tissue distribution of TCDD. During the first phase most of the TCDD dissolved in oil spheres distributed into highly perfused organs; during the following phase it was redistributed to poorly perfused, but rather lipophilic organs. Two tissues did not fit this pattern. Liver, highly perfused but lower in fat content than WAT, accumulated about one-half of total TCDD which is known to be bound to microsomal proteins. Brain, despite its high blood supply and lipophilicity, attained concentrations of TCDD comparable only to or even lower than in blood. This is surprising because it is in contrast to the disposition of other compounds with similar physicochemical properties

Short Term Analgesic Effects of 5% Dextrose Epidural Injections for Chronic Low Back Pain: A Randomized Controlled Trial.

Science.gov (United States)

Maniquis-Smigel, Liza; Dean Reeves, Kenneth; Jeffrey Rosen, Howard; Lyftogt, John; Graham-Coleman, Cassie; Cheng, An-Lin; Rabago, David

2017-02-01

Hypertonic dextrose injection (prolotherapy) is reported to reduce pain including non-surgical chronic low back pain (CLBP), and subcutaneous injection of 5% dextrose is reported to reduce neurogenic pain, hyperalgesia and allodynia. The mechanism in both cases is unclear, though a direct effect of dextrose on neurogenic pain has been proposed. This study assessed the short-term analgesic effects of epidural 5% dextrose injection compared with saline for non-surgical CLBP. Randomized double-blind (injector, participant) controlled trial. Adults with moderate-to-severe non-surgical low back pain with radiation to gluteal or leg areas for at least 6 months received a single epidurogram-confirmed epidural injection of 10 mL of 5% dextrose or 0.9% saline using a published vertical caudal injection technique. The primary outcome was change in a numerical rating scale (NRS, 0 - 10 points) pain score between baseline and 15 minutes; and 2, 4, and 48 hours and 2 weeks post-injection. The secondary outcome was percentage of participants achieving 50% or more pain improvement at 4 hours. No baseline differences existed between groups; 35 participants (54 ± 10.7 years old; 11 female) with moderate-to-severe CLBP (6.7 ± 1.3 points) for 10.6 ± 10.5 years. Dextrose participants reported greater NRS pain score change at 15 minutes (4.4 ± 1.7 vs 2.4 ± 2.8 points; P = 0.015), 2 hours (4.6 ± 1.9 vs 1.8 ± 2.8 points; P = 0.001), 4 hours (4.6 ± 2.0 vs 1.4 ± 2.3 points; P dextrose recipients and 19% (3/16) of saline recipients reported ≥ 50% pain reduction at 4 hours (P dextrose on pain at the dorsal root level; waning pain control at 2 weeks suggests the need to assess the effect of serial dextrose epidural injections in a long-term study with robust outcome assessment.

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.

Automatic estimation of aquifer parameters using long-term water supply pumping and injection records

Science.gov (United States)

Luo, Ning; Illman, Walter A.

2016-09-01

Analyses are presented of long-term hydrographs perturbed by variable pumping/injection events in a confined aquifer at a municipal water-supply well field in the Region of Waterloo, Ontario (Canada). Such records are typically not considered for aquifer test analysis. Here, the water-level variations are fingerprinted to pumping/injection rate changes using the Theis model implemented in the WELLS code coupled with PEST. Analyses of these records yield a set of transmissivity ( T) and storativity ( S) estimates between each monitoring and production borehole. These individual estimates are found to poorly predict water-level variations at nearby monitoring boreholes not used in the calibration effort. On the other hand, the geometric means of the individual T and S estimates are similar to those obtained from previous pumping tests conducted at the same site and adequately predict water-level variations in other boreholes. The analyses reveal that long-term municipal water-level records are amenable to analyses using a simple analytical solution to estimate aquifer parameters. However, uniform parameters estimated with analytical solutions should be considered as first rough estimates. More accurate hydraulic parameters should be obtained by calibrating a three-dimensional numerical model that rigorously captures the complexities of the site with these data.

Fluoroscopically guided caudal epidural steroid injection for management of degenerative lumbar spinal stenosis: short-term and long-term results

Energy Technology Data Exchange (ETDEWEB)

Lee, Joon Woo; Myung, Jae Sung; Kang, Heung Sik [Seoul National University Bundang Hospital, Department of Radiology, Seong Nam, Gyeongi-do (Korea); Park, Kun Woo; Yeom, Jin S. [Seoul National University Bundang Hospital, Department of Orthopaedic Surgery, Seong Nam, Gyeongi-do (Korea); Kim, Ki-Jeong; Kim, Hyun-Jib [Seoul National University Bundang Hospital, Department of Neurosurgery, Seong Nam, Gyeongi-do (Korea)

2010-07-15

To evaluate the short-term and long-term effects of fluoroscopically guided caudal epidural steroid injection (ESI) for the management of degenerative lumbar spinal stenosis (DLSS) and to analyze outcome predictors. All patients who underwent caudal ESI in 2006 for DLSS were included in the study. Response was based on chart documentation (aggravated, no change, slightly improved, much improved, no pain). In June 2009 telephone interviews were conducted, using formatted questions including the North American Spine Society (NASS) patient satisfaction scale. For short-term and long-term effects, age difference was evaluated by the Mann-Whitney U test, and gender, duration of symptoms, level of DLSS, spondylolisthesis, and previous operations were evaluated by Fisher's exact test. Two hundred and sixteen patients (male: female = 75:141; mean age 69.2 years; range 48{proportional_to}91 years) were included in the study. Improvements (slightly improved, much improved, no pain) were seen in 185 patients (85.6%) after an initial caudal ESI and in 189 patients (87.5%) after a series of caudal ESIs. Half of the patients (89/179, 49.8%) replied positively to the NASS patient satisfaction scale (1 or 2). There were no significant outcome predictors for either the short-term or the long-term responses. Fluoroscopically guided caudal ESI was effective for the management of DLSS (especially central canal stenosis) with excellent short-term and good long-term results, without significant outcome predictors. (orig.)

A New Sacroiliac Joint Injection Technique and Its Short-Term Effect on Chronic Sacroiliac Region Pain.

Science.gov (United States)

Do, Kyung Hee; Ahn, Sang Ho; Jones, Rodney; Jang, Sung Ho; Son, Su Min; Lee, Dong Gyu; Cho, Hee Kyung; Choi, Gyu Sik; Cho, Yun-Woo

2016-10-01

Sacroiliac joint (SIJ) injections have been used to provide short-term relief of SIJ pain. In this study, the authors investigated a new technique using a superior approach. Twenty four patients with chronic SI joint paint were recruited. Each patient was treated with a single SIJ intra-articular injection plus a periarticular injection of local anesthetic and corticosteroid in one procedure. Technical accuracy of the intra-articular procedure was determined by having 2 independent observers review and rate the quality of arthrograms obtained. Treatment effects were evaluated using a numerical rating scale, the Oswestry disability index (ODI) and global perceived effect (GPE). Both independent observers agreed that satisfactory arthrograms were obtained in all patients. Pain scores and disability were significantly reduced at 2 weeks and 4 weeks after treatment. Nineteen patients (79%) reported satisfaction with treatment. No serious adverse effects were encountered. The superior approach consistently achieves good access to the SI joint, and achieves outcomes that are compatible with those of other techniques. The superior approach constitutes an alternative to other techniques for injections into the SI joint. © 2016 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Labeling of hepatic glycogen after short- and long-term stimulation of glycogen synthesis in rats injected with 3H-galactose

International Nuclear Information System (INIS)

Michaels, J.E.; Garfield, S.A.; Hung, J.T.; Cardell, R.R. Jr.

1990-01-01

The effects of short- and long-term stimulation of glycogen synthesis elicited by dexamethasone were studied by light (LM) and electron (EM) microscopic radioautography (RAG) and biochemical analysis. Adrenalectomized rats were fasted overnight and pretreated for short- (3 hr) or long-term (14 hr) periods with dexamethasone prior to intravenous injection of tracer doses of 3H-galactose. Analysis of LM-RAGs from short-term rats revealed that about equal percentages (44%) of hepatocytes became heavily or lightly labeled 1 hr after labeling. The percentage of heavily labeled cells increased slightly 6 hr after labeling, and unlabeled glycogen became apparent in some hepatocytes. The percentage of heavily labeled cells had decreased somewhat 12 hr after labeling, and more unlabeled glycogen was evident. In the long-term rats 1 hr after labeling, a higher percentage of heavily labeled cells (76%) was observed compared to short-term rats, and most glycogen was labeled. In spite of the high amount of labeling seen initially, the percentage of heavily labeled hepatocytes had decreased considerably to 55% by 12 hr after injection; and sparsely labeled and unlabeled glycogen was prevalent. The EM-RAGs of both short- and long-term rats were similar. Silver grains were associated with glycogen patches 1 hr after labeling; 12 hr after labeling, the glycogen patches had enlarged; and label, where present, was dispersed over the enlarged glycogen clumps. Analysis of DPM/mg tissue corroborated the observed decrease in label 12 hr after administration in the long-term animals. The loss of label observed 12 hr after injection in the long-term pretreated rats suggests that turnover of glycogen occurred during this interval despite the net accumulation of glycogen that was visible morphologically and evident from biochemical measurement

Sex work involvement among women with long-term opioid injection drug dependence who enter opioid agonist treatment.

Science.gov (United States)

Marchand, Kirsten; Oviedo-Joekes, Eugenia; Guh, Daphne; Marsh, David C; Brissette, Suzanne; Schechter, Martin T

2012-01-25

Substitution with opioid-agonists (e.g., methadone) has shown to be an effective treatment for chronic long-term opioid dependency. Survival sex work, very common among injection drug users, has been associated with poor Opioid Agonist Treatment (OAT) engagement, retention and response. Therefore, this study was undertaken to determine factors associated with engaging in sex work among long-term opioid dependent women receiving OAT. Data from a randomized controlled trial, the North American Opiate Medication Initiative (NAOMI), conducted in Vancouver and Montreal (Canada) between 2005-2008, was analyzed. The NAOMI study compared the effectiveness of oral methadone to injectable diacetylmorphine or injectable hydromorphone, the last two on a double blind basis, over 12 months. A research team, independent of the clinic services, obtained outcome evaluations at baseline and follow-up (3, 6, 9, 12, 18 and 24 months). A total 53.6% of women reported engaging in sex work in at least one of the research visits. At treatment initiation, women who were younger and had fewer years of education were more likely to be engaged in sex work. The multivariate logistic generalized estimating equation regression analysis determined that psychological symptoms, and high illicit heroin and cocaine use correlated with women's involvement in sex work during the study period. After entering OAT, women using injection drugs and engaging in sex work represent a particularly vulnerable group showing poorer psychological health and a higher use of heroin and cocaine compared to women not engaging in sex work. These factors must be taken into consideration in the planning and provision of OAT in order to improve treatment outcomes. NCT00175357.

Sex work involvement among women with long-term opioid injection drug dependence who enter opioid agonist treatment

Directory of Open Access Journals (Sweden)

Marchand Kirsten

2012-01-01

Full Text Available Abstract Background Substitution with opioid-agonists (e.g., methadone has shown to be an effective treatment for chronic long-term opioid dependency. Survival sex work, very common among injection drug users, has been associated with poor Opioid Agonist Treatment (OAT engagement, retention and response. Therefore, this study was undertaken to determine factors associated with engaging in sex work among long-term opioid dependent women receiving OAT. Methods Data from a randomized controlled trial, the North American Opiate Medication Initiative (NAOMI, conducted in Vancouver and Montreal (Canada between 2005-2008, was analyzed. The NAOMI study compared the effectiveness of oral methadone to injectable diacetylmorphine or injectable hydromorphone, the last two on a double blind basis, over 12 months. A research team, independent of the clinic services, obtained outcome evaluations at baseline and follow-up (3, 6, 9, 12, 18 and 24 months. Results A total 53.6% of women reported engaging in sex work in at least one of the research visits. At treatment initiation, women who were younger and had fewer years of education were more likely to be engaged in sex work. The multivariate logistic generalized estimating equation regression analysis determined that psychological symptoms, and high illicit heroin and cocaine use correlated with women's involvement in sex work during the study period. Conclusions After entering OAT, women using injection drugs and engaging in sex work represent a particularly vulnerable group showing poorer psychological health and a higher use of heroin and cocaine compared to women not engaging in sex work. These factors must be taken into consideration in the planning and provision of OAT in order to improve treatment outcomes. Trial Registration NCT00175357.

Efficacy and safety of corticosteroid injections and other injections for management of tendinopathy: a systematic review of randomised controlled trials.

Science.gov (United States)

Coombes, Brooke K; Bisset, Leanne; Vicenzino, Bill

2010-11-20

Few evidence-based treatment guidelines for tendinopathy exist. We undertook a systematic review of randomised trials to establish clinical efficacy and risk of adverse events for treatment by injection. We searched eight databases without language, publication, or date restrictions. We included randomised trials assessing efficacy of one or more peritendinous injections with placebo or non-surgical interventions for tendinopathy, scoring more than 50% on the modified physiotherapy evidence database scale. We undertook meta-analyses with a random-effects model, and estimated relative risk and standardised mean differences (SMDs). The primary outcome of clinical efficacy was protocol-defined pain score in the short term (4 weeks, range 0-12), intermediate term (26 weeks, 13-26), or long term (52 weeks, ≥52). Adverse events were also reported. 3824 trials were identified and 41 met inclusion criteria, providing data for 2672 participants. We showed consistent findings between many high-quality randomised controlled trials that corticosteroid injections reduced pain in the short term compared with other interventions, but this effect was reversed at intermediate and long terms. For example, in pooled analysis of treatment for lateral epicondylalgia, corticosteroid injection had a large effect (defined as SMD>0·8) on reduction of pain compared with no intervention in the short term (SMD 1·44, 95% CI 1·17-1·71, ptendon rupture). By comparison with placebo, reductions in pain were reported after injections of sodium hyaluronate (short [3·91, 3·54-4·28, peffective than was eccentric exercise. Despite the effectiveness of corticosteroid injections in the short term, non-corticosteroid injections might be of benefit for long-term treatment of lateral epicondylalgia. However, response to injection should not be generalised because of variation in effect between sites of tendinopathy. None. Copyright © 2010 Elsevier Ltd. All rights reserved.

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

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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

Sequential injection lab-on-valve: the third generation of flow injection analysis

DEFF Research Database (Denmark)

Wang, Jianhua; Hansen, Elo Harald

2003-01-01

Termed the third generation of flow injection analysis, sequential injection (SI)-lab-on-valve (LOV) has specific advantages and allows novel, unique applications - not least as a versatile front end to a variety of detection techniques. This review presents snd discusses progress to date of the ...

Percutaneous ethanol injection therapy (PEIT) for small hepatocellular carcinomas (HCCs) : prognostic factors affecting short-term follow-up results

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Kim, Ah Young; Han, Joon Koo; Kim, Tae Kyoung; Yun, Eun Joo; Lee, Ki Yeol; Choi, Byung Ihn [Seoul National Univ. (Korea, Republic of). Coll. of Medicine

1998-06-01

The purpose of this study is to analyze the effect of various parameters used in PEIT on the short-term results of this procedure for the treatment of small HCC. Among 76 lesions in 63 patients with HCCs, 66 were diagnosed by tissue biopsy (n=30) or according to clinical features, tumor marker, and the results of angiography and other diagnostic imagings (n=36). These patients underwent PEIT between November 1993 and October 1996; the indications for PEIT were nodular lesions less than 3 cm in size, less than three in number, and unsuitable for surgical resection or transcatheter arterial chemoembolization therapy. The effect of PEIT was evaluated by two phase spiral CT one month later (64 lesions) or by serial ultrasound for 6 months. We analyzed and graded the short-term effect of PEIT on HCCs as complete remission (CR), partial remission (PR) or no change/aggravation (NC/AG). We also evaluated the correlation between the short-term results of PEIT and variable parameters such as size of the lesion, frequency of PEI per session, child classification of the patient, the ratio of amount of injected ethanol per volume of the lesion, and {alpha}-fetoprotein level before the procedure. The therapeutic effect of PEIT was CR in 45 lesions (59.2%), PR in 18 (23.7%) and NC/AG in 13 (17.1%). The size of the tumor, child-pugh class, number of injections per session and ratio of injected volume of ethanol to tumor volume were factors affecting the therapeutic result (p < 0.05). Sufficient knowledge of prognostic factors affecting the short-term results of PEIT might help improve the effects of therapy in patients its small HCCs. (author). 19 refs., 6 figs.

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.

Long-term follow-up of ultrasound-guided botulinum toxin-A injections for sialorrhea in neurological dysphagia.

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Barbero, Pierangelo; Busso, Marco; Tinivella, Marco; Artusi, Carlo Alberto; De Mercanti, Stefania; Cucci, Angele; Veltri, Andrea; Avagnina, Paolo; Calvo, Andrea; Chio', Adriano; Durelli, Luca; Clerico, Marinella

2015-12-01

Literature provides reports only of a limited follow-up single injection of botulinum toxin-A (BoNT-A) in patients with sialorrhea. The aim of our study is to evaluate the long-lasting efficacy and safety of ultrasound-guided BoNT-A injections for severe sialorrhea secondary to neurological dysphagia. We enrolled 38 severe adult sialorrhea patients referred consecutively to the neurology unit and performed bilateral parotid and submandibular gland BoNT-A injections under ultrasound guidance. The outcomes of the study were reduction of sialorrhea, duration of therapeutic effect, and subjective patient- and caregiver-reported satisfaction. A total of 113 BoNT-A administrations were given during the study period with a mean duration of follow-up of 20.2 ± 4.4 months. We observed a significant decrease from baseline in mean number of daily aspirations and a significant improvement in patient- and caregiver-reported outcomes following ultrasound-guided BoNT-A injections (p < 0.001 vs baseline for all comparisons) and the mean duration of the efficacy was 5.6 ± 1 months. No major treatment-related adverse events occurred and a low incidence of minor adverse events was reported. This study confirms the long-lasting efficacy and safety of ultrasound-guided BoNT-A injections for sialorrhea, regardless of the causative neurological disorder. These results should encourage the use of BoNT-A in the treatment of severe sialorrhea and highlight the role of ultrasound guidance to obtain optimal results in terms of safety and reproducible outcomes.

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

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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.

Long-term Results of Intravitreal Anti-VEGF Injections in Wet AMD: A Meta-Analysis.

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Gerding, H

2016-04-01

Although intravitreal anti-VEGF medications are widely used in age-related macular degeneration, no systematic data analysis is available on the long-term prognosis of this relatively new therapeutic approach. A meta-analysis was performed on available Medline literature. 13 relevant clinical studies (14 case series) could be identified, covering 10 247 treated eyes. The majority of available reports originate from single centre retrospective real-life environments. The mean improvement in average visual gain was 0.9 ± 0.5 (mean ± 1 standard deviation, median; 0.8 lines) at year 1, 1.2 ± 1.1 (median: 1.1) letters at year 2, 0.7 ± 1.0 (median: 0.7) letters at year 3, and 0.2 ± 0.8 (median: 0.5), 0.4 ± 0.4 (median: 0.5) at years 4 and 5. The drop-out rates in these studies was relatively high. At the end of year 3, the average percentage of observed eyes was 44.3 ± 18.4 % (mean ± 1 standard deviation), at the end of year 4 23.5 ± 23.9 % and after years 6 and 7 below 10 % (8.2 and 7.9 %). The mean treatment frequency of injections in all available studies was highest in year 1 (6.4 ± 1.2, 6.1 - mean ± SD; median), followed by relatively consistent mean values of 4.1 and 5.1 (year (Y)2: 4.4, Y3: 4.3, Y4: 4.7, Y5: 4.1, Y6: 5.1, Y7: 4.7) injections per year. The results of this meta-analysis clearly indicate that intravitreal anti-VEGF injection therapy is capable of maintaining visual acuity on a long-term basis of at least 4-5 years. Georg Thieme Verlag KG Stuttgart · New York.

Anesthetic efficacy of a repeated intraosseous injection following a primary intraosseous injection.

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Jensen, Joanne; Nusstein, John; Drum, Melissa; Reader, Al; Beck, Mike

2008-02-01

The purpose of this prospective, randomized, single-blinded study was to determine the anesthetic efficacy of a repeated intraosseous injection given 30 minutes after a primary intraosseous injection. Using a crossover design, 55 subjects randomly received a primary X-tip intraosseous injection (Dentsply Inc, York, PA) of 1.4 mL of 2% lidocaine with epinephrine (using the Wand; Milestone Scientific, Deerfield, IL) and a repeated intraosseous or mock injection at 30 minutes in two appointments. The first molar and adjacent teeth were pulp tested every 2 minutes for a total of 120 minutes. Success was defined as obtaining two consecutive 80 readings with the electric pulp tester. Success of the initial intraosseous injection was 100% for the first molar. The repeated intraosseous injection mimicked the initial intraosseous injection in terms of pulpal anesthesia and statistically provided another 15 minutes of pulpal anesthesia. In conclusion, using the methodology presented, repeating the intraosseous injection 30 minutes after an initial intraosseous injection will provide an additional 15 minutes of pulpal anesthesia.

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...

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

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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.

Do cervical epidural injections provide long-term relief in neck and upper extremity pain? A systematic review.

Science.gov (United States)

Manchikanti, Laxmaiah; Nampiaparampil, Devi E; Candido, Kenneth D; Bakshi, Sanjay; Grider, Jay S; Falco, Frank J E; Sehgal, Nalini; Hirsch, Joshua A

2015-01-01

The high prevalence of chronic persistent neck pain not only leads to disability but also has a significant economic, societal, and health impact. Among multiple modalities of treatments prescribed in the management of neck and upper extremity pain, surgical, interventional and conservative modalities have been described. Cervical epidural injections are also common modalities of treatments provided in managing neck and upper extremity pain. They are administered by either an interlaminar approach or transforaminal approach. To determine the long-term efficacy of cervical interlaminar and transforaminal epidural injections in the treatment of cervical disc herniation, spinal stenosis, discogenic pain without facet joint pain, and post surgery syndrome. The literature search was performed from 1966 to October 2014 utilizing data from PubMed, Cochrane Library, US National Guideline Clearinghouse, previous systematic reviews, and cross-references. The evidence was assessed based on best evidence synthesis with Level I to Level V. There were 7 manuscripts meeting inclusion criteria. Of these, 4 assessed the role of interlaminar epidural injections for managing disc herniation or radiculitis, and 3 assessed these injections for managing central spinal stenosis, discogenic pain without facet joint pain, and post surgery syndrome. There were 4 high quality manuscripts. A qualitative synthesis of evidence showed there is Level II evidence for each etiology category. The evidence is based on one relevant, high quality trial supporting the efficacy of cervical interlaminar epidural injections for each particular etiology. There were no randomized trials available assessing the efficacy of cervical transforaminal epidural injections. Paucity of available literature, specifically conditions other than disc herniation. This systematic review with qualitative best evidence synthesis shows Level II evidence for the efficacy of cervical interlaminar epidural injections with local

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

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Hund E

2012-06-01

removing the main source of mutant TTR. Recently, orally administered tafamidis meglumine has been approved by European authorities for treatment of FAP. The substance has been shown to stabilize the TTR tetramer, thereby improving the outcome of patients with TTR-FAP. Various other strategies have been studied in vitro to prevent TTR amyloidosis, including gene therapy, immunization, dissolution of TTR aggregates, and free radical scavengers, but none of them is ready for clinical use so far.Keywords: FAP, transthyretin, amyloidosis, treatment, therapy

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.

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

Client satisfaction among participants in a randomized trial comparing oral methadone and injectable diacetylmorphine for long-term opioid-dependency

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Brissette Suzanne

2011-07-01

Full Text Available Abstract Background Substitution with opioid-agonists (e.g., methadone has shown to be an effective treatment for chronic long-term opioid dependency. Patient satisfaction with treatment has been associated with improved addiction treatment outcomes. However, there is a paucity of studies evaluating patients' satisfaction with Opioid Substitution Treatment (OST. In the present study, participants' satisfaction with OST was evaluated at 3 and 12 months. We sought to test the relationship between satisfaction and patients' characteristics, the treatment modality received and treatment outcomes. Methods Data from a randomized controlled trial, the North American Opiate Medication Initiative (NAOMI, conducted in Vancouver and Montreal (Canada between 2005-2008, was analyzed. The NAOMI study compared the effectiveness of oral methadone vs. injectable diacetylmorphine over 12 months. A small sub-group of patients received injectable hydromorphone on a double blind basis with diacetylmorphine. The Client Satisfaction Questionnaire (CSQ-8 was used to measure satisfaction with treatment. CSQ-8 scores, as well as retention and response to treatment, did not differ between those receiving hydromorphone and diacetylmorphine at 3 or 12 months assessments; therefore, these two groups were analyzed together as the 'injectable' treatment group. Results A total of 232 (92% and 237 (94% participants completed the CSQ-8 at 3 and 12 months, respectively. Participants in both groups were highly satisfied with treatment. Independent of treatment group, participants satisfied with treatment at 3 months were more likely to be retained at 12 months. Multivariate analysis indicated that satisfaction was greater among those randomized to the injection group after controlling for treatment effectiveness. Participants who were retained, responded to treatment, and had fewer psychological symptoms were more satisfied with treatment. Finally, open-ended comments were made by

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

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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.

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

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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.

CT-guided cervical nerve root injections: comparing the immediate post-injection anesthetic-related effects of the transforaminal injection with a new indirect technique

International Nuclear Information System (INIS)

Sutter, Reto; Pfirrmann, Christian W.A.; Peterson, Cynthia K.; Zanetti, Marco; Hodler, Juerg

2011-01-01

To describe an ''indirect'' cervical nerve root injection technique with a dorsal approach that should carry less inherent risk than the ''direct'' cervical transforaminal injection approach, and to compare the immediate post-injection results of the two procedures. The indirect and direct cervical nerve root injection procedures are described in detail. Fifty-three consecutive patients receiving the indirect nerve root injections during 2009-2010 were age- and gender-matched to 53 patients who underwent direct transforaminal nerve root injections performed in 2006. Pain level data were collected immediately before and 20-30 min after each procedure. The percentages of pain change in the two groups were compared using the unpaired Student's t test. Fifty-two men (mean age 49) and 54 women (mean age 55) were included. The mean percentage of pain reduction for patients receiving indirect nerve root injections was 38.4% and for those undergoing the direct nerve root injections approach it was 43.2%. This was not significantly different (P = 0.455). No immediate or late adverse effects were reported after either injection procedure. The indirect cervical nerve root injection procedure is a potentially safer alternative to direct cervical transforaminal nerve root injections. The short-term pain reduction is similar using the two injection methods. (orig.)

Treatment of achalasia: the short-term response to botulinum toxin injection seems to be independent of any kind of pretreatment

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Meining A

2002-08-01

Full Text Available Abstract Background It has been suggested that intrasphincteric injection of botulinum toxin (BTX may represent an alternative therapy to balloon dilatation in achalasia. The aim of the present study was to test the effectiveness of botulinum toxin injections in achalasia patients, as assessed using lower oesophageal sphincter pressure (LOSP and symptom scores, and to compare the response in patients with different types of pretreatment (no previous treatment, balloon dilatation, myotomy, BTX injection. Methods Forty patients who presented with symptomatic achalasia were treated with BTX injection (48 injections in 40 patients. Some of the patients had received prior treatment (seven with myotomy, seven with dilatation and eight with BTX. The symptoms were assessed using a global symptom score (0–10, which was evaluated before treatment, 1 week afterwards, and 1 month afterwards. Manometry was also carried out before and after treatment. Three different selections of patients were studied: all patients; untreated patients; and patients with prior BTX, dilatation, or myotomy. Results After BTX injection, there was a significant reduction in LOSP (before, 38.2 ± 11.3 mmHg; 1 week after, 20.5 ± 6.9 mmHg; 1 month after, 17.8 ± 6.8 mmHg; P Conclusions BTX injection offers an alternative treatment for achalasia which is safe and can be performed in an outpatient setting. The initial response to BTX, in terms of symptom scores and LOSP, appears to be independent of any prior treatment. A number of patients do not adequately respond to balloon dilatation or myotomy, which are the first-line treatment modalities in achalasia patients. BTX injection can be performed in these patients, and symptomatic benefit can be expected in the same percentages as with BTX injection in untreated patients.

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.

Injection and lessons for 2012

International Nuclear Information System (INIS)

Bracco, C.; Barnes, M.J.; Bartmann, W.; Cornelis, K.; Drosdal, L.N.; Goddard, B.; Kain, V.; Meddahi, M.; Mertens, V.; Uythoven, J.

2012-01-01

Injection of 144 bunches into the LHC became fully operational during the 2011 run and one nominal injection of 288 bunches was accomplished. Several mitigation solutions were put in place to minimise losses from the Transfer Line (TL) collimators and losses from kicking de-bunched beam during injection. Nevertheless, shot-by- shot and bunch-by-bunch trajectory variations, as well as long terms drifts, were observed and required a regular re-steering of the TL implying a non negligible amount of time spent for injection setup. Likely sources of instability have been identified (i.e. MKE and MSE ripples) and possible cures to optimise 2012 operation are presented. Well defined references for TL steering will be defined in a more rigorous way in order to allow a more straightforward and faster injection setup. Encountered and potential issues of the injection system, in particular the injection kickers MKI, are discussed also in view of injections with a higher number of bunches. (authors)

Injection and lessons for 2012

CERN Document Server

Bracco, C; Bartmann, W; Cornelis, K; Drosdal, L N; Goddard, B; Kain, V; Meddahi, M; Mertens, V; Uythoven, J

2012-01-01

Injection of 144 bunches into the LHC became fully operational during the 2011 run and a nominal injection of 288 bunches was accomplished during MD time. Several mitigation solutions were put in place to minimise losses from the transfer line (TL) collimators and losses from kicking debunched beam during injection. Nevertheless, shot-by-shot and bunch-by-bunch trajectory variations, as well as long terms drifts, were observed and required a regular resteering of the TL implying a non negligible amount of time spent for injection setup. Likely sources of instability have been identified (i.e. MKE and MSE ripples) and possible cures to optimise 2012 operation are presented. Well defined references for TL steering will be defined in a more rigorous way in order to allow a more straightforward and faster injection setup. Encountered and potential issues of the injection system, in particular the injection kickers MKI, are discussed also in view of injections with a higher number of bunches.

Steroid injections in the treatment of humeral unicameral bone cysts: long-term follow-up and review of the literature.

Science.gov (United States)

Pavone, V; Caff, G; Di Silvestri, C; Avondo, S; Sessa, G

2014-05-01

Retrospective evaluation of long-term effectiveness of the steroid injections treatment in patients with unicameral bone cysts (UBC). From January 1993 to April 2005, 23 children affected by proximal humeral UBC were evaluated according to the Neer-Cole classification system and treated with serial methylprednisolone acetate's injections. The patients were followed up at 1, 3, 6 and 12 months and then every year until the adolescence. After treatment, in 15 out of 23 patients (65.2%), the humeral cysts were referred, respectively, as Grade 1 and in four as Grade 2. In 4 patients, a refracture occurred. Statistical analysis showed an overall good response in 82.6% of patients at the end of the follow-up. Minor complication including skin discoloration accounted for 13.04%. The steroid injections showed to be an alternative excellent treatment for UBC, with complete healing of the lesions in the majority of cases. This procedure is not expensive, mini-invasive, with low surgical risk and short hospitalization.

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.

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

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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

Long-term complications of polyethylene glycol injection to the face.

Science.gov (United States)

Altintas, Hande; Odemis, Mustafa; Bilgi, Selcuk; Cakmak, Ozcan

2012-04-01

Currently, filling, smoothing, or recontouring the face through the use of injectable fillers is one of the most popular forms of cosmetic surgery. Because these materials promise a more youthful appearance without anesthesia in a noninvasive way, various fillers have been used widely in different parts of the world. However, most of these fillers have not been approved by the Food and Drug Administration, and their applications might cause unpleasant disfiguring complications. This report describes a case of foreign body granuloma in the cheeks secondary to polyethylene glycol injection and shows the possible complications associated with the use of facial fillers.

Proportion of long-term injection drug users as an indicator to characterize the state and prognosis of HIV-epidemic within a certain territory

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Andreeva, Tatiana

2012-07-01

Full Text Available BACKGROUND: Epidemics of drug use and thus the spread of HIV have different duration in different regions, and, therefore, the prognosis of these epidemics may differ. We aimed to assess indicators measuring the peculiarities of injection drug use epidemics by region, informative for prevention activities among vulnerable to HIV groups. METHODS: Data from cross-sectional survey of 4026 injection drug users (IDUs conducted in 2007 in Ukraine were analyzed. The outcome measure was a binary variable depicting whether a respondent injects drugs for 20 years and over. Binary Logistic Regression in SPSS software was used to test associations with socio-demographic characteristics. RESULTS: More respondents from Odessa, Mykolayiv, Dnipropetrovsk, Cherkassy, Poltava, and Crimea regions inject over 20 years. Older respondents were more likely to belong to the group of long-term users. Men were more likely to inject over 20 years than women. Those respondents who were married, but did not live with their spouse or other sexual partner were more likely to inject longer than 20 years compared to those single or in a stable marriage. Those respondents who use opiates or combine them with stimulants were more likely to inject over 20 years and those who use only stimulants were more likely to inject less than 20 years. CONCLUSION: Injection drug use in Ukraine started earlier among men, on certain territories and was associated with opiate use. Percentage of IDUs who inject for more than 20 years was found to be a good indicator to distinguish territories with long-lasting epidemics.

Trapping of gun-injected plasma by a tokamak

International Nuclear Information System (INIS)

Leonard, A.W.; Dexter, R.N.; Sprott, J.C.

1986-01-01

It is shown that a plasma produced by a Marshall gun can be injected into and trapped by a tokamak plasma. Gun injection raises the line-averaged density and peaks the density profile. Trapping of the gun-injected plasma is explainable in terms of a depolarization current mechanism

Effects of single injection of local anesthetic agents on intervertebral disc degeneration: ex vivo and long-term in vivo experimental study.

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Koji Iwasaki

Full Text Available Analgesic discography (discoblock can be used to diagnose or treat discogenic low back pain by injecting a small amount of local anesthetics. However, recent in vitro studies have revealed cytotoxic effects of local anesthetics on intervertebral disc (IVD cells. Here we aimed to investigate the deteriorative effects of lidocaine and bupivacaine on rabbit IVDs using an organotypic culture model and an in vivo long-term follow-up model.For the organotypic culture model, rabbit IVDs were harvested and cultured for 3 or 7 days after intradiscal injection of local anesthetics (1% lidocaine or 0.5% bupivacaine. Nucleus pulposus (NP cell death was measured using confocal microscopy. Histological and TUNEL assays were performed. For in vivo study, each local anesthetic was injected into rabbit lumbar IVDs under a fluoroscope. Six or 12 months after the injection, each IVD was prepared for magnetic resonance imaging (MRI and histological analysis.In the organotypic culture model, both anesthetic agents induced time-dependent NP cell death; when compared with injected saline solution, significant effects were detected within 7 days. Compared with the saline group, TUNEL-positive NP cells were significantly increased in the bupivacaine group. In the in vivo study, MRI analysis did not show any significant difference. Histological analysis revealed that IVD degeneration occurred to a significantly level in the saline- and local anesthetics-injected groups compared with the untreated control or puncture-only groups. However, there was no significant difference between the saline and anesthetic agents groups.In the in vivo model using healthy IVDs, there was no strong evidence to suggest that discoblock with local anesthetics has the potential of inducing IVD degeneration other than the initial mechanical damage of the pressurized injection. Further studies should be performed to investigate the deteriorative effects of the local injection of analgesic agents

Medium-term and long-term outcomes of interventions for primary psoas tendinopathy.

Science.gov (United States)

Garala, Kanai; Prasad, Vishnu; Jeyapalan, Kanagaratnam; Power, Richard A

2014-05-01

To assess medium- and long-term outcomes of psoas tendinopathy to psoas tenotomy and image-guided steroid injections. This is a 14-year retrospective case-control study to identify the efficacy of psoas tenotomy and image-guided steroid injections. This study was undertaken in a secondary care setting. Patients with confirmed psoas tendinopathy were followed up by postal questionnaire, which included a nonarthritic hip score (NAHS) and a study patient satisfaction questionnaire. Patients underwent image-guided steroid injections. Depending on the analgesic or symptomatic relief, some patients proceeded to psoas tenotomy. Response to steroid injection. Pain relief and symptomatic relief after the surgery. Twenty-three patients were reviewed with a 70% follow-up over a time of 49 months for surgery (range, 13-144 months) and 77 months for injection (range, 14-160 months). Eight patients had a lasting response to injection and required no further intervention, and 15 patients proceeded to psoas tenotomy using a medial Ludloff approach. The average NAHS scores after the surgery and injection were 66.15 and 76.08, respectively. Ten patients reported pain relief after their tenotomy, and 5 patients reported no change in pain. All 8 patients, who only underwent injection, reported lasting pain relief. Local steroid injections can provide long-term relief for patients presenting with psoas tendinopathy. For those patients with only temporary relief from injection, psoas tenotomy can provide good long-term pain relief.

Short-term muscle atrophy caused by botulinum toxin-A local injection impairs fracture healing in the rat femur.

Science.gov (United States)

Hao, Yongqiang; Ma, Yongcheng; Wang, Xuepeng; Jin, Fangchun; Ge, Shengfang

2012-04-01

Damaged bone is sensitive to mechanical stimulation throughout the remodeling phase of bone healing. Muscle damage and muscular atrophy associated with open fractures and subsequent fixation are not beneficial to maintaining optimum conditions for mechanical stability. The aim of this study was to investigate whether local muscle atrophy and dysfunction affect fracture healing in a rat femur fracture model. We combined the rat model of a short period atrophy of the quadriceps with femur fracture. Forty-four-month-old male Wistar rats were adopted for this study. Two units of botulinum toxin-A (BXTA) were administered locally into the right side of the quadriceps of each rat, while the same dose of saline was injected into the contralateral quadriceps. After BXTA had been fully absorbed by the quadriceps, osteotomy was performed in both femurs with intramedullary fixation. Gross observation and weighing of muscle tissue, X-ray analysis, callus histology, and bone biomechanical testing were performed at different time points up to 8 weeks post-surgery. Local injection of BXTA led to a significant decrease in the volume and weight of the quadriceps compared to the control side. At the eighth week, the left side femurs of the saline-injected quadriceps almost reached bony union, and fibrous calluses were completely calcified into woven bone. However, a gap was still visible in the BXTA-treated side on X-ray images. As showed by bone histology, there were no mature osseous calluses or woven bone on the BXTA-treated side, but a resorption pattern was evident. Biomechanical testing indicated that the femurs of the BXTA-treated side exhibited inferior mechanical properties compared with the control side. The inferior outcome following BXTA injection, compared with saline injection, in terms of callus resistance may be the consequence of unexpected load and mechanical unsteadiness caused by muscle atrophy and dysfunction. Copyright © 2011 Orthopaedic Research Society.

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

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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.

Long-Term Effects of Botulinum Toxin Complex Type A Injection on Mechano- and Metabo-Sensitive Afferent Fibers Originating from Gastrocnemius Muscle.

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Guillaume Caron

Full Text Available The aim of the present study was to investigate long term effects of motor denervation by botulinum toxin complex type A (BoNT/A from Clostridium Botulinum, on the afferent fibers originating from the gastrocnemius muscle of rats. Animals were divided in 2 experimental groups: 1 untreated animals acting as control and 2 treated animals in which the toxin was injected in the left muscle, the latter being itself divided into 3 subgroups according to their locomotor recovery with the help of a test based on footprint measurements of walking rats: i no recovery (B0, ii 50% recovery (B50 and iii full recovery (B100. Then, muscle properties, metabosensitive afferent fiber responses to potassium chloride (KCl and lactic acid injections and Electrically-Induced Fatigue (EIF, and mechanosensitive responses to tendon vibrations were measured. At the end of the experiment, rats were killed and the toxin injected muscles were weighted. After toxin injection, we observed a complete paralysis associated to a loss of force to muscle stimulation and a significant muscle atrophy, and a return to baseline when the animals recover. The response to fatigue was only decreased in the B0 group. The responses to KCl injections were only altered in the B100 groups while responses to lactic acid were altered in the 3 injected groups. Finally, our results indicated that neurotoxin altered the biphasic pattern of response of the mechanosensitive fiber to tendon vibrations in the B0 and B50 groups. These results indicated that neurotoxin injection induces muscle afferent activity alterations that persist and even worsen when the muscle has recovered his motor activity.

Long-Term Effects of Botulinum Toxin Complex Type A Injection on Mechano- and Metabo-Sensitive Afferent Fibers Originating from Gastrocnemius Muscle.

Science.gov (United States)

Caron, Guillaume; Marqueste, Tanguy; Decherchi, Patrick

2015-01-01

The aim of the present study was to investigate long term effects of motor denervation by botulinum toxin complex type A (BoNT/A) from Clostridium Botulinum, on the afferent fibers originating from the gastrocnemius muscle of rats. Animals were divided in 2 experimental groups: 1) untreated animals acting as control and 2) treated animals in which the toxin was injected in the left muscle, the latter being itself divided into 3 subgroups according to their locomotor recovery with the help of a test based on footprint measurements of walking rats: i) no recovery (B0), ii) 50% recovery (B50) and iii) full recovery (B100). Then, muscle properties, metabosensitive afferent fiber responses to potassium chloride (KCl) and lactic acid injections and Electrically-Induced Fatigue (EIF), and mechanosensitive responses to tendon vibrations were measured. At the end of the experiment, rats were killed and the toxin injected muscles were weighted. After toxin injection, we observed a complete paralysis associated to a loss of force to muscle stimulation and a significant muscle atrophy, and a return to baseline when the animals recover. The response to fatigue was only decreased in the B0 group. The responses to KCl injections were only altered in the B100 groups while responses to lactic acid were altered in the 3 injected groups. Finally, our results indicated that neurotoxin altered the biphasic pattern of response of the mechanosensitive fiber to tendon vibrations in the B0 and B50 groups. These results indicated that neurotoxin injection induces muscle afferent activity alterations that persist and even worsen when the muscle has recovered his motor activity.

Comparison of MELCOR modeling techniques and effects of vessel water injection on a low-pressure, short-term, station blackout at the Grand Gulf Nuclear Station

International Nuclear Information System (INIS)

Carbajo, J.J.

1995-06-01

A fully qualified, best-estimate MELCOR deck has been prepared for the Grand Gulf Nuclear Station and has been run using MELCOR 1.8.3 (1.8 PN) for a low-pressure, short-term, station blackout severe accident. The same severe accident sequence has been run with the same MELCOR version for the same plant using the deck prepared during the NUREG-1150 study. A third run was also completed with the best-estimate deck but without the Lower Plenum Debris Bed (BH) Package to model the lower plenum. The results from the three runs have been compared, and substantial differences have been found. The timing of important events is shorter, and the calculated source terms are in most cases larger for the NUREG-1150 deck results. However, some of the source terms calculated by the NUREG-1150 deck are not conservative when compared to the best-estimate deck results. These results identified some deficiencies in the NUREG-1150 model of the Grand Gulf Nuclear Station. Injection recovery sequences have also been simulated by injecting water into the vessel after core relocation started. This marks the first use of the new BH Package of MELCOR to investigate the effects of water addition to a lower plenum debris bed. The calculated results indicate that vessel failure can be prevented by injecting water at a sufficiently early stage. No pressure spikes in the vessel were predicted during the water injection. The MELCOR code has proven to be a useful tool for severe accident management strategies

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.

Neuron and neuroblast numbers and cytogenesis in the dentate gyrus of aged APP(swe)/PS1(dE9) transgenic mice

DEFF Research Database (Denmark)

Olesen, Louise Orum; Sivasaravanaparan, Mithula; Severino, Maurizio

2017-01-01

Altered neurogenesis may influence hippocampal functions such as learning and memory in Alzheimer's disease. Selective serotonin reuptake inhibitors enhance neurogenesis and have been reported to reduce cerebral amyloidosis in both humans and transgenic mice. We have used stereology to assess the...... working memory, independent of changes in total granular neurons. Furthermore, while long-term paroxetine treatment may be able to reduce hippocampal amyloidosis, it appears to have no effect on total number of granular neurons or spatial working memory....... the longitudinal changes in the number of doublecortin-expressing neuroblasts and number of granular neurons in the dentate gyrus of APPswe/PS1dE9 transgenic mice. Furthermore, we investigated the effect of long-term paroxetine treatment on the number of neuroblasts and granular neurons, hippocampal amyloidosis......Altered neurogenesis may influence hippocampal functions such as learning and memory in Alzheimer's disease. Selective serotonin reuptake inhibitors enhance neurogenesis and have been reported to reduce cerebral amyloidosis in both humans and transgenic mice. We have used stereology to assess...

UV-visible digital imaging of split injection in a Gasoline Direct Injection engine

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Merola Simona Silvia

2015-01-01

Full Text Available Ever tighter limits on pollutant emissions and the need to improve energy conversion efficiency have made the application of gasoline direct injection (GDI feasible for a much wider scale of spark ignition engines. Changing the way fuel is delivered to the engine has thus provided increased flexibility but also challenges, such as higher particulate emissions. Therefore, alternative injection control strategies need to be investigated in order to obtain optimum performance and reduced environmental impact. In this study, experiments were carried out on a single-cylinder GDI optical engine fuelled with commercial gasoline in lean-burn conditions. The single-cylinder was equipped with the head of a commercial turbocharged engine with similar geometrical specifications (bore, stroke, compression ratio and wall guided fuel injection. Optical accessibility was ensured through a conventional elongated hollow Bowditch piston and an optical crown, accommodating a fused-silica window. Experimental tests were performed at fixed engine speed and injection pressure, whereas the injection timing and the number of injections were adjusted to investigate their influence on combustion and emissions. UV-visible digital imaging was applied in order to follow the combustion process, from ignition to the late combustion phase. All the optical data were correlated with thermodynamic analysis and measurements of exhaust emissions. Split injection strategies (i.e. two injections per cycle with respect to single injection increased combustion efficiency and stability thanks to an improvement of fuel air mixing. As a consequence, significant reduction in soot formation and exhaust emission with acceptable penalty in terms of HC and NOx were measured.

Endoscopic Injection of Dextranomer/Hyaluronic Acid as First-Line Treatment in 851 Consecutive Children with High-Grade Vesicoureteral Reflux: Efficacy and Long-Term Results.

Science.gov (United States)

Friedmacher, Florian; Colhoun, Eric; Puri, Prem

2018-03-15

Endoscopic injection of dextranomer/hyaluronic is widely acknowledged as first-line treatment of lower grade vesicoureteral reflux. Our objective was to demonstrate its long-term efficacy and safety in eradicating high-grade reflux. Eight-hundred-fifty-one children (518 girls, 333 boys), median age 2.3 years (2 months-13.7 years), underwent endoscopic correction of high-grade vesicoureteral reflux using dextranomer/hyaluronic acid. Reflux was unilateral in 415 cases and bilateral in 436, comprising 1,287 refluxing units: grade IV in 1,153 (89.6%) and grade V in 134 (10.4%). 99m technetium-dimercaptosuccinic acid imaging identified renal scarring in 317 (37.3%) patients. Follow-up ultrasound and voiding cystourethrogram were performed 3 months post intervention and renal ultrasound annually thereafter. Median follow-up was 8.5 years (6 months-16 years). Overall resolution rate after the first endoscopic injection was 895/1,287 (69.5%): 70.4% in grade IV and 61.9% in grade V, respectively. Reflux resolved after a second injection in 259 (20.1%) and after a third in 133 (10.4%). Persistent reflux after initial treatment was significantly more common in infants reflux resolution, 43 (5.1%) children developed febrile urinary tract infections: 24 (55.8%) in the first, 15 (34.9%) in the second and 4 (9.3%) after ≥3 years. Of these, 6 had reflux recurrence and 8 demonstrated neocontralateral grade III reflux, which was successfully treated with single endoscopic injection of dextranomer/hyaluronic acid. Endoscopic injection of dextranomer/hyaluronic acid is an efficient and safe long-term treatment for grade IV and V vesicoureteral reflux, which can be easily repeated in cases of failure with a high subsequent resolution rate. Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

Long-term correction of obesity and diabetes in genetically obese mice by a single intramuscular injection of recombinant adeno-associated virus encoding mouse leptin

Science.gov (United States)

Murphy, John E.; Zhou, Shangzhen; Giese, Klaus; Williams, Lewis T.; Escobedo, Jaime A.; Dwarki, Varavani J.

1997-01-01

The ob/ob mouse is genetically deficient in leptin and exhibits a phenotype that includes obesity and non-insulin-dependent diabetes melitus. This phenotype closely resembles the morbid obesity seen in humans. In this study, we demonstrate that a single intramuscular injection of a recombinant adeno-associated virus (AAV) vector encoding mouse leptin (rAAV-leptin) in ob/ob mice leads to prevention of obesity and diabetes. The treated animals show normalization of metabolic abnormalities including hyperglycemia, insulin resistance, impaired glucose tolerance, and lethargy. The effects of a single injection have lasted through the 6-month course of the study. At all time points measured the circulating levels of leptin in the serum were similar to age-matched control C57 mice. These results demonstrate that maintenance of normal levels of leptin (2–5 ng/ml) in the circulation can prevent both the onset of obesity and associated non-insulin-dependent diabetes. Thus a single injection of a rAAV vector expressing a therapeutic gene can lead to complete and long-term correction of a genetic disorder. Our study demonstrates the long-term correction of a disease caused by a genetic defect and proves the feasibility of using rAAV-based vectors for the treatment of chronic disorders like obesity. PMID:9391128

Amiloidose e insuficiência renal crônica terminal associada à hanseníase Amyloidosis and end-stage renal disease associated with leprosy

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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.

Two injection digital block versus single subcutaneous palmar injection block for finger lacerations.

Science.gov (United States)

Okur, O M; Şener, A; Kavakli, H Ş; Çelik, G K; Doğan, N Ö; Içme, F; Günaydin, G P

2017-12-01

We aimed to compare two digital nerve block techniques in patients due to traumatic digital lacerations. This was a randomized-controlled study designed prospectively in the emergency department of a university-based training and research hospital. Randomization was achieved by sealed envelopes. Half of the patients were randomised to traditional (two-injection) digital nerve block technique while single-injection digital nerve block technique was applied to the other half. Score of pain due to anesthetic infiltration and suturing, onset time of total anesthesia, need for an additional rescue injection were the parameters evaluated with both groups. Epinephrin added lidocaine hydrochloride preparation was used for the anesthetic application. Visual analog scale was used for the evaluation of pain scores. Outcomes were compared by using Mann-Whitney U test and Student t-test. Fifty emergency department patients ≥18 years requiring digital nerve block were enrolled in the study. Mean age of the patients was 33 (min-max: 19-86) and 39 (78 %) were male. No statistically significant difference was found between the two groups in terms of our main parameters; anesthesia pain score, suturing pain score, onset time of total anesthesia and rescue injection need. Single injection volar digital nerve block technique is a suitable alternative for digital anesthesias in emergency departments.

Effect of injection timing on combustion and performance of a direct injection diesel engine running on Jatropha methyl ester

Energy Technology Data Exchange (ETDEWEB)

Jindal, S. [Mechanical Engineering Department, College of Technology & Engineering, Maharana Pratap University of Agriculture and Technology, Udaipur 313001 (India)

2011-07-01

The present study aims at evaluation of effect of injection timing on the combustion, performance and emissions of a small power diesel engine, commonly used for agriculture purpose, running on pure biodiesel, prepared from Jatropha (Jatropha curcas) vegetable oil. The effect of varying injection timing was evaluated in terms of thermal efficiency, specific fuel consumption, power and mean effective pressure, exhaust temperature, cylinder pressure, rate of pressure rise and the heat release rate. It was found that retarding the injection timing by 3 degrees enhances the thermal efficiency by about 8 percent.

Long-term Results of Percutaneous Ethanol Injection for the Treatment of Hepatocellular Carcinoma in Korea

International Nuclear Information System (INIS)

Sung, Yon Mi; Choi, Don Gil; Lim, Hyo K.

2006-01-01

To evaluate the long-term follow-up results of percutaneous ethanol injection (PEI) for the treatment of hepatocellular carcinoma (HCC) in Korea. Sixty-eight nodular HCCs initially detected in 64 patients, were subjected to US-guided PEI as a first-line treatment. Long-term survival rates, local tumor progression rates, and complications were evaluated, as were the influences of tumor size and Child-Pugh class on these variables. No major complications occurred. The overall survival rates of the 64 patients at three and five years were 71% and 39%, and their cancer-free survival rates were 22% and 15%, respectively. The overall survival rate of patients with a small HCC (≤ 2 cm) was significantly higher (p = 0.014) than that of patients with a medium-sized HCC (≤ 2 cm). The overall survival rate of patients with Child- Pugh class A was significantly higher (p = 0.049) than that of patients with Child- Pugh class B. Of 59 cases with no residual tumor, local tumor progression was observed in ablation zones in 18, and this was not found to be significantly influenced by tumor size or Child-Pugh class. The results of our investigation of the long-term survival rates of PEI in HCC patients in Korea (a hepatitis B virus-endemic area) were consistent with those reported previously in hepatitis C endemic areas. Patients with a smaller tumor or a better liver function exhibited superior survival rates

Groundwater monitoring for deep-well injection

International Nuclear Information System (INIS)

Chia, Y.; Chiu, J.

1994-01-01

A groundwater monitoring system for detecting waste migration would not only enhance confidence in the long-term containment of injected waste, but would also provide early warnings of contamination for prompt responses to protect underground sources of drinking water (USDWs). Field experiences in Florida have demonstrated monitoring water quality and fluid pressure changes in overlying formations is useful in detecting the upward migration of injected waste. Analytical and numerical solutions indicate changes in these two monitoring parameters can vary on the basis of hydrogeologic characteristics, operation conditions, and the distances from the injection well to the monitoring wells and to the preferential hydrologic conduits. To detect waste migration through defects around the wellbore or the leaky containment interval, groundwater monitoring wells should be placed as close as possible to an injection well. In the vertical direction, a monitoring well completed in a permeable interbed within the containment interval is expected to have the highest potential for detecting upward migration. Another acceptable horizon for groundwater monitoring is the lower portion of the buffer brine aquifer immediately above the containment interval. Monitoring wells in USDWs may be needed when waste has been detected in deeper formations or when leakage out of well casings poses a concern. A monitoring well open to the injection interval is of little value in alleviating the concerns of long-term upward migration. Moreover, the installation of the well could create additional preferential pathways. Complications in groundwater monitoring may arise at existing injection sites, especially with prior releases. It is also important to recognize that monitoring in the vicinity of the wellbore may not be effective for detecting waste migration through unidentified unplugged wells or undetected transmissive fractures

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.

Bladder base/trigone injection is safe and as effective as bladder body injection of onabotulinumtoxinA for idiopathic detrusor overactivity refractory to antimuscarinics.

Science.gov (United States)

Kuo, Hann-Chorng

2011-09-01

The purpose of this study was to evaluate the efficacy and safety of onabotulinumtoxinA injections at bladder base/trigone and compare with injections at bladder body or bladder body/trigone for the treatment of idiopathic detrusor overactivity (IDO) refractory to antimuscarinics. A single blind, randomized, paralleled, actively controlled trial was performed in patients with urodynamically proven IDO who failed antimuscarinic therapy. Patients were randomly assigned to receive intravesical injections of 100 U of onabotulinumtoxinA into three different bladder sites. All treatments were evaluated by voiding diary variables, urgency severity score, urodynamic studies, and patient perception of bladder condition. Long-term success rates over 12 months were also determined. Among the patients, 37 were randomized to injections in the bladder body, 35 into the bladder body/trigone, and 33 into the bladder base/trigone. Successful results were reported in 76 (72%) patients at 3 months: 26 (70%) in the bladder body group, 26 (74%) in the bladder body/trigone group, and 24 (73%) in the bladder base/trigone group. There were no significant differences in success rates, changes in urgency and urgency incontinence episodes, urodynamic variables, or long-term success rates among the three subgroups. The incidence of adverse events was similar among three groups. No vesicoureteral reflux was noted in all patients with or without involving trigone injection. Intravesical onabotulinumtoxinA injection is an effective treatment for IDO regardless of the bladder injection site. Bladder base/trigone injection is as safe and effective as bladder body injections with or without trigone involvement. Copyright © 2011 Wiley-Liss, Inc.

Incidence of plantar fascia ruptures following corticosteroid injection.

Science.gov (United States)

Kim, Chul; Cashdollar, Michael R; Mendicino, Robert W; Catanzariti, Alan R; Fuge, LaDonna

2010-12-01

Plantar fasciitis is commonly treated with corticosteroid injections to decrease pain and inflammation. Therapeutic benefits often vary in terms of efficacy and duration. Rupture of the plantar fascia has been reported as a possible complication following corticosteroid injection. A retrospective chart review of 120 patients who received corticosteroid injection for plantar fasciitis was performed at the authors' institution to determine the incidence of plantar fascia rupture. The plantar fascia rupture was diagnosed clinically and confirmed with magnetic resonance imaging. Various factors were analyzed, including the number of injections, interval between injections, body mass index (BMI), and activity level. Four patients (2.4%) consequently experienced plantar fascia rupture following an average of 2.67 injections. The average BMI of these patients was 38.6 kg/m². The authors conclude that corticosteroid injection therapy appears to be a safe and effective form of nonoperative treatment with minimal complications and a relatively low incident of plantar fascia rupture.

Autologous Blood Injection Works for Recalcitrant Lateral Epicondylitis.

Science.gov (United States)

Bostan, Bora; Balta, Orhan; Aşçı, Murat; Aytekin, Kürşad; Eser, Enes

2016-03-01

Recalcitrant lateral epicondylitis may be a disabling condition. Treatment of this condition is still controversial. In the present prospective study, we evaluated the long-term results of autologous blood injection for the treatment of recalcitrant lateral epicondylitis. Prospective clinical study. A total of 42 elbows of 40 consecutive patients (28 female, 12 male) were enrolled in this prospective study. Seven patients left the study (3 patients moved to another city, 1 patient died in the second week due to a heart condition, 1 patient quit the study because of the resolution of pain in the fourth week and 2 patients did not agree to the second injection). Thirteen patients were lost to third year follow-up. Therefore, a total of 21 elbows of 20 patients with 3 years of follow-up were included in this study. The mean age of the patients was 47.25 years (range, 20-68 years). Visual analogue scale (VAS), Nirschl score and grip strength were significantly improved after injections when compared to before treatment. The best improvement in terms of grip strength, Nirschl score and VAS score was detected at the one year follow-up. The improvement in Nirschl and VAS score sustained until the third year. We suggest that autologous blood injection for the treatment of recalcitrant lateral epicondylitis is an effective, safe and successful procedure in the long-term.

Safe and successful endoscopic initial treatment and long-term eradication of gastric varices by endoscopic ultrasound-guided Histoacryl (N-butyl-2-cyanoacrylate) injection.

Science.gov (United States)

Gubler, Christoph; Bauerfeind, Peter

2014-09-01

Optimal endoscopic treatment of gastric varices is still not standardized nowadays. Actively bleeding varices may prohibit a successful endoscopic injection therapy of Histoacryl® (N-butyl-2-cyanoacrylate). Since 2006, we have treated gastric varices by standardized endoscopic ultrasound (EUS) guided Histoacryl injection therapy without severe adverse events. We present a large single-center cohort over 7 years with a standardized EUS-guided sclerotherapy of all patients with gastric varices. Application was controlled by fluoroscopy to immediately detect any glue embolization. Only perforating veins located within the gastric wall were treated. In the follow up, we repeated this treatment until varices were eradicated. Utmost patients (36 of 40) were treated during or within 24 h of active bleeding. About 32.5% of patients were treated while visible bleeding. Histoacryl injection was always technically successful and only two patients suffered a minor complication. Acute bleeding was stopped in all patients. About 15% (6 of 40) of patients needed an alternative rescue treatment in the longer course. Three patients got a transjugular portosystemic shunt and another three underwent an orthotopic liver transplantation. Mean long-term survival of 60 months was excellent. Active bleeding of gastric varices can be treated successfully without the necessity of gastric rinsing with EUS-guided injection of Histoacryl.

Advocacy for Gender Affirming Care: Learning from the Injectable Estrogen Shortage.

Science.gov (United States)

Geffen, Sophia; Horn, Tim; Smith, Kimberleigh Joy; Cahill, Sean

2018-01-01

Hormone therapy is medically necessary for many transgender individuals. The U.S. Food and Drug Administration (FDA) and pharmaceutical companies' failure to guarantee a supply of injectable estrogen in 2016 and 2017 for transgender individuals is a violation of their right to comprehensive medical treatment, free of discrimination. A series of advocacy actions eventually led to all formulations of injectable estrogen being restored to market; however, long-term solutions to supply interruptions of injectable estrogen are needed. Long-term solutions should address the lack of federally funded research and, consequently, evidence-based practice on hormone therapy for gender affirmation.

Percutaneous ethanol injection of hyperfunctioning thyroid nodules: long-term follow-up in 125 patients.

Science.gov (United States)

Tarantino, Luciano; Francica, Giampiero; Sordelli, Ignazio; Sperlongano, Pasquale; Parmeggiani, Domenico; Ripa, Carmine; Parmeggiani, Umberto

2008-03-01

The purpose of this study was to assess the long-term efficacy of percutaneous ethanol injection (PEI) for the treatment of hyperfunctioning thyroid nodules. One hundred twenty-five patients (88 women, 37 men; age range, 17-76 years; mean age, 53 years) with 127 hyperfunctioning thyroid nodules (volume, 1.2-90 mL; mean, 10.3 mL) were treated with PEI. There were 1-11 PEI sessions per patient (average, 3.9) performed, with injection of 1-14 mL of ethanol per session (total injected ethanol per patient, 3-108 mL; mean, 14.0 mL). Efficacy of the treatment was assessed with color Doppler sonography; scintigraphy; and free triiodothyronine (FT3), free thyroxine (FT4), and thyroid-stimulating hormone (TSH) assays. Follow-up (9-144 months; median, 60 months) was performed with TSH and color Doppler sonography every 2 months for 6 months and every 6 months thereafter. Three (2.4%) of 125 patients refused completion of PEI therapy because of pain. Results are reported in 122 patients with 124 nodules. All 122 patients showed posttreatment normal levels of FT3, FT4, and TSH. A complete cure (absent uptake in the nodule and recovery of normal uptake in the thyroid parenchyma) was obtained in 113 (93%) of 122 patients-115 (92.7%) of 124 treated nodules. Residual hyperfunctioning nodular tissue along with decreased thyroid parenchyma uptake (partial cure) was present in nine patients accounting for nine (7.3%) of 124 nodules. Rates of complete cure after PEI were: overall nodules, 115 (92.7%) of 124; nodules nodules > 10 to nodules > 30 to nodules > 60 mL, three (100%) of three. The overall rate of major complications (transient laryngeal nerve damage, two patients; abscess and hematoma, one patient each) was four (3.2%) of 125 patients. Follow-up examinations showed marked shrinkage of 112 treated nodules ranging from 50% to 90% of the pretreatment volume (mean, 66%) and new growth of hyperfunctioning tissue in four patients at color Doppler sonography and scintigraphy at 12

Amyloid arthropathy of the hip joint: MR demonstration of presumed amyloid lesions in 152 patients with long-term hemodialysis

International Nuclear Information System (INIS)

Otake, S.; Yamana, D.; Tsuruta, Y.; Mizutani, H.; Ohba, S.

1998-01-01

The aim of this study was to determine the spectrum of MR findings of presumed amyloid arthropathy of the hip joints in patients on long-term hemodialysis. We prospectively performed T1- and T2-weighted spin-echo imaging on 152 consecutive patients on hemodialysis. The duration of hemodialysis ranged from 5 months to 24 years, 2 months (mean: 8 years, 8 months). The frequency, location, and signal intensity of bone lesions were assessed. In 12 cases with contrast-enhanced MR examination, enhancement pattern of bone lesions, synovial lesions, and intra-articular lesions were characterized. Bone lesions presumed to be amyloid deposits were identified in 60 patients (39 %). Magnetic resonance imaging revealed that amyloid lesions were more extensive than anticipated by plain radiographs. All bone lesions showed decreased signal intensity on T1-weighted images. On T2-weighted images, bone lesions showed increased signal intensity in 32 patients (54 %), decreased signal intensity in 11 patients (18 %), and both increased and decreased signal intensity in 17 patients (28 %). Following intravenous injection of gadolinium-based contrast, all bone lesions showed moderate enhancement. Synovial thickening could not be identified on T1- and T2-weighted images. However, contrast-enhanced images showed thickened synovial membrane, which could be differentiated from joint fluid. Intra-articular nodules showed decreased or intermediate signal intensity on T1-weighted images and decreased signal intensity on T2-weighted images; the intra-articular nodules were contiguous with subchondral bone lesions. Magnetic resonance imaging is useful for evaluating the distribution and extent of amyloidosis of the hip joints in patients undergoing long-term hemodialysis. (orig.) (orig.)

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.

Single-use autoinjector for once-weekly intramuscular injection of IFNβ-1a.

Science.gov (United States)

Limmroth, Volker; Gerbershagen, Kathrin

2014-12-01

IFNβ products and glatiramer acetate are established treatment first-line options in long-term disease-modifying therapy of multiple sclerosis (MS). These self-injectable medications are used once weekly to once daily. Injection-related issues are common patient-cited reasons for nonadherence. Autoinjectors have been shown to support long-term adherence to injectable medications. The ability to self-inject in MS patients has been associated with a reduced risk of missed injections and drug discontinuation, and a beneficial effect on patient independence. The recently introduced easy-to-use prefilled once-weekly pen is a safe and effective device for intramuscular (IM) IFNβ-1a application and provides a convenient method for self-injection. We reviewed the available published evidence on the characteristics of this device. The once-weekly pen facilitates self-injection and was preferred over prefilled syringes by patients in a prospective open-label, multicenter Phase IIIb trial in MS patients who had been using IM IFNβ-1a in prefilled syringes. The simple and safe handling, shielded short needle, single-use disposable design and virtually painless injection by the device may contribute to adherence, quality of life and independence in patients using IM IFNβ-1a.

Effect of feeding greater amounts of dietary energy for a short-term with or without eCG injection on reproductive performance, serum metabolites and hormones in ewes.

Science.gov (United States)

Habibizad, Javad; Riasi, Ahmad; Kohram, Hamid; Rahmani, Hamid Reza

2015-09-01

This study was conducted to compare the effect of transient high-energy diet in a short-term period with or without eCG injection on ovarian follicle development, twining rate, serum metabolites and hormones in ewes. A total of 45 estrous cyclic Naeini ewes were randomly assigned to three experimental groups: 1-Control (control), 2-High energy short-term feeding (HE), and 3-high energy short-term feeding + eCG injection (HEe). Ewes were housed in individual pens with free access to feed and water. The stage of the estrous cycle of all ewes was synchronized by insertion of intravaginal progesterone sponges. Focus feeding started from 4 days before until 1 day after sponge removal. Follicle development was monitored from 4 days before until 1 day after sponge removal and blood samples were taken during this time. Results showed that ewes fed high energy diets (HE and HEe) had greater (P ewes fed high energy diets had less (P ewes. Copyright © 2015 Elsevier B.V. All rights reserved.

Autologous Blood Injection Works for Recalcitrant Lateral Epicondylitis

Directory of Open Access Journals (Sweden)

Bora Bostan

2016-04-01

Full Text Available Background: Recalcitrant lateral epicondylitis may be a disabling condition. Treatment of this condition is still controversial. Aims: In the present prospective study, we evaluated the long-term results of autologous blood injection for the treatment of recalcitrant lateral epicondylitis. Study Design: Prospective clinical study. Methods: A total of 42 elbows of 40 consecutive patients (28 female, 12 male were enrolled in this prospective study. Seven patients left the study (3 patients moved to another city, 1 patient died in the second week due to a heart condition, 1 patient quit the study because of the resolution of pain in the fourth week and 2 patients did not agree to the second injection. Thirteen patients were lost to third year follow-up. Therefore, a total of 21 elbows of 20 patients with 3 years of follow-up were included in this study. The mean age of the patients was 47.25 years (range, 20-68 years. Results: Visual analogue scale (VAS, Nirschl score and grip strength were significantly improved after injections when compared to before treatment. The best improvement in terms of grip strength, Nirschl score and VAS score was detected at the one year follow-up. The improvement in Nirschl and VAS score sustained until the third year. Conclusion: We suggest that autologous blood injection for the treatment of recalcitrant lateral epicondylitis is an effective, safe and successful procedure in the long-term.

Epidural steroid injection in patients with lumbosacral radiculopathy in Abuja, Nigeria

Directory of Open Access Journals (Sweden)

Ahidjo A Kawu

2012-01-01

Full Text Available Objective : This prospective-controlled observational study looked at well-matched patients with spinal pain and radicular symptoms, caused by lumbar intervertebral disc herniation to compare the short-term clinical outcome of transforaminal and interlaminar epidural steroid injection (ESI in a resource challenged tertiary institution in Nigeria. Materials and Methods : 49 patients with radicular symptoms who were matched for age, symptom duration, magnetic resonance imaging findings, and pre-injection revised Oswentry Disability Index (ODI score and Visual Analogue Scale (VAS were assigned into ESI technique. The ODI and VAS score were analyzed immediately after an injection and upon follow-up (average 178.5 days, also with the need for repeated injections and surgical interventions over a 1-year follow-up interval. Result: In the transforaminal group (25 patients, there was a statistically significant improvement in the ODI scores from before the injection (ODI mean 62.4 to immediately after the injection (ODI mean 24.4, P < 0.01, and upon follow-up (ODI mean 20.8, P < 0.01. 9 patients (18.4% required 1 or 2 repeated injections, 3 (6.1% patients underwent surgery and 2 (4% patients lost to follow-up. In the interlaminar group (24 patients, there was a statistically significant improvement in the ODI scores from before the injection (ODI mean 60.7 to immediately after the injection (ODI mean 30.1, P < 0.01, but not upon follow-up (ODI mean 43.2, P = 0.09. 11 (22.4% patients required 1 or 2 repeated injection, 4 (8% patients underwent surgery and 3 (6.1% patients were lost to follow-up. There is an average of 2 fold improvement of transforaminal ESI over interlaminar ESI in a 40 point scale of ODI score on follow-up, which was statistically significant (P < 0.01. The VAS showed similar pattern with the ODI scores in the study. Conclusion : Transforaminal ESI to treat symptomatic lumbar disc herniation resulted in better short-term pain

Specific localization and imaging of amyloid deposits in vivo using 123I-labeled serum amyloid P component

International Nuclear Information System (INIS)

Hawkins, P.N.; Myers, M.J.; Epenetos, A.A.; Caspi, D.; Pepys, M.B.

1988-01-01

Highly specific, high-resolution scintigraphic images of amyloid-laden organs in mice with experimentally induced amyloid A protein (AA) amyloidosis were obtained after intravenous injection of 123 I-labeled serum amyloid P component (SAP). Interestingly, a much higher proportion (up to 40%) of the injected dose of heterologous human SAP localized to amyloid and was retained there than was the case with isologous mouse SAP, indicating that human SAP binds more avidly to mouse AA fibrils than does mouse SAP. Specificity of SAP localization was established by the failure of the related proteins, human C-reactive protein and Limulus C-reactive protein, to deposit significantly in amyloid and by the absence of human SAP deposition in nonamyloidotic organs. However, only partial correlations were observed between the quantity of SAP localized and two independent estimates, histology and RIA for AA of the amount of amyloid in particular organs. It is not clear which of the three methods used reflects better the extent or clinical significance of the amyloid deposits but in vivo localization of radiolabeled SAP, detectable and quantifiable by gamma camera imaging, is apparently extremely sensitive. These findings establish the use of labeled SAP as a noninvasive in vivo diagnostic probe in experimental amyloidosis, potentially capable of revealing the natural history of the condition, and suggest that it may also be applicable generally as a specific targeting agent for diagnostic and even therapeutic purposes in clinical amyloidosis

Accidental intra-arterial injection of fluorescein dye.

Science.gov (United States)

Bovino, J A; Marcus, D F

1984-12-01

During fluorescein angiography, sodium fluorescein dye intended for intravenous use was inadvertently injected into an artery in the antecubital fossa. An immediate and dramatic orange discoloration of the skin distal to the injection combined with intense burning pain of the right forearm and hand were noted. The patient was treated with ice packs and analgesics. The fluorescein angiogram showed a delayed arm to eye circulation time, but was of normal quality. There were no long-term complications.

Controlled CO2 injection into a shallow aquifer and leakage detection monitoring practices at the K-COSEM site, Korea

Science.gov (United States)

Lee, S. S.; Joun, W.; Ju, Y. J.; Ha, S. W.; Jun, S. C.; Lee, K. K.

2017-12-01

Artificial carbon dioxide injection into a shallow aquifer system was performed with two injection types imitating short- and long-term CO2 leakage events into a shallow aquifer. One is pulse type leakage of CO2 (6 hours) under a natural hydraulic gradient (0.02) and the other is long-term continuous injection (30 days) under a forced hydraulic gradient (0.2). Injection and monitoring tests were performed at the K-COSEM site in Eumseong, Korea where a specially designed well field had been installed for artificial CO2 release tests. CO2-infused and tracer gases dissolved groundwater was injected through a well below groundwater table and monitoring were conducted in both saturated and unsaturated zones. Real-time monitoring data on CO2 concentration and hydrochemical parameters, and periodical measurements of several gas tracers (He, Ar, Kr, SF6) were obtained. The pulse type short-term injection test was carried out prior to the long-term injection test. Results of the short-term injection test, under natural hydraulic gradient, showed that CO2 plume migrated along the preferential pathway identified through hydraulic interference tests. On the other hand, results of the long-term injection test indicated the CO2 plume migration path was aligned to the forced hydraulic gradient. Compared to the short-term test, the long-term injection formed detectable CO2 concentration change in unsaturated wellbores. Recovery data of tracer gases made breakthrough curves compatible to numerical simulation results. The monitoring results indicated that detection of CO2 leakage into groundwater was more effectively performed by using a pumping and monitoring method in order to capture by-passing plume. With this concept, an effective real-time monitoring method was proposed. Acknowledgement: Financial support was provided by the "R&D Project on Environmental Management of Geologic CO2storage" from the KEITI (Project number : 2014001810003)

Particle balance studies in TEXTOR during experiments of pellet injection, helium injection, and ICR-heating

International Nuclear Information System (INIS)

Banno, T.; Finken, K.H.; Gray, D.S.; Winter, J.

1995-01-01

Analysis based on the particle conservation law has been carried out to observe the global fuelling process in tokamak discharges. The response of the net recycling flux from the first wall is investigated in the tokamak TEXTOR, using calibrated signals of the gas feed rate, the neutral gas pressure in the vessel, the total amount of electrons, and the particle removal rates by the ALT-II belt-pump limiter and by a main pump unit. Net absorption (pumping) of hydrogen by the wall is observed for almost all tokamak discharges since a new wall conditioning technique called siliconisation is employed. The net absorption or fuelling depending on the discharge condition influenced by injection of pellets, by helium gas injection combined with neutral beam injection, and by rf heating can be interpreted in terms of the particle-induced desorption effect with depth profile taken into consideration. ((orig.))

Long-term follow-up of a case of intravenous elementary mercury injection

International Nuclear Information System (INIS)

Walter, E.

1986-01-01

Elementary mercury is usually intravenously injected with suicidal intent. It is floated to the heart and lungs but is also deposited in the abdominal organs. Case histories presented in the literature so far have been followed up clinically and roentgenologically for up to three years. We report one patient attempting suicidal mercury injection, whom we were able to follow up for 10 years. It could be demonstrated that quite in contrast to former suggestions elementary mercury is dissolved and oxidised in the body. Chronic poisoning with mercury compounds causes continuing damage, particularly to the kidneys. Apart from that question, the element's pattern of spread within the body, toxicological issues, particular pathologic anatomic changes, their demonstrability on X-ray films and their clinical relevance are all discussed in this paper. (orig.) [de

Safe injection procedures, injection practices, and needlestick ...

African Journals Online (AJOL)

Results: Safe injection procedures regarding final waste disposal were sufficiently adopted, while measures regarding disposable injection equipment, waste containers, hand hygiene, as well as injection practices were inadequately carried out. Lack of job aid posters that promote safe injection and safe disposal of ...

Collagenase injections for the treatment of single cords in cases of Dupuytren’s contracture – a prospective intervention study of long-term experience with Xiapex

Directory of Open Access Journals (Sweden)

Fischer, Lisa Maria

2017-04-01

Full Text Available Introduction: The gold standard in the treatment of Dupuytren’s contracture is surgical therapy. Alternatives are percutaneous needle fasciotomy and radiation in exceptional cases. Injection treatments with Xiapex (Pfizer are a new therapy option. This collagenase, extracted from clostridium histolyticum, is used to break down the affected tissue cords. The objective of this study is to examine the effect and long-term success of treatment with Xiapex.Methods: In this study, Xiapex treatment was conducted on a sample group of 19 patients with Dupuytren’s contracture. The injection was placed either on the cord at the level of the metacarpophalangeal (MCP joint (n=17 or of the proximal interphalangeal (PIP joint (n=7. Break-up of the cord occurred 24 hours after the injection. The neutral zero method was used to assess the extent of movement. The Michigan Hand Outcomes Questionnaire (MHQ was selected for evaluation of the general hand function in 16 patients. The WHO-5 and the EQ-5D VAS Score were used as a measure of the patients’ satisfaction and their state of health. All values were collected both pre-injection as well as 1 year post-injection.Results: Out of 19 patients in our sample group, 16 patients (≈84% benefitted in terms of improvement in mobility. Overall, the range of movement increased by Ø 26° in the affected finger. A separate assessment demonstrated that:The range of movement increased by 77% in the MCP joint. The extent of movement pre-injection was Ø (0-28-78 and post-injection it was Ø (0-9-81 with an improvement of Ø 22°. In the PIP joint, only slight improvement was observed (Ø pre (0-27-93; post (0-24-95.The MHQ increased from Ø 76% (R: 32–97% to 81% (R: 39–100%.The painfulness decreased from Ø 19% (R: 0–55% to Ø11% (R: 0–55%, corresponding to Ø 43%. Satisfaction increased in 72% of patients by Ø 21%.According to WHO-5, patient satisfaction pre-injection was Ø 20 (R: 11–25, and 1 year after

The short- and medium-term effectiveness of CT-guided selective cervical nerve root injection for pain and disability

Energy Technology Data Exchange (ETDEWEB)

Desai, Amidevi; Saha, Shouvik; Sharma, Naveen; Huckerby, Lauren; Houghton, Russell [Guy' s and St. Thomas' Hospitals, Department of Radiology, London (United Kingdom)

2014-07-15

CT-guided cervical nerve root injection with corticosteroid and/or local anesthetic is a recognized technique in the evaluation and treatment of cervical radiculopathy. There are few prospective studies on the efficacy of the various techniques employed in cervical nerve root injection. We present our results from a 1-year prospective series using a CT-guided anterolateral transforaminal approach for cervical nerve root injection of bupivacaine and dexamethasone. Pain using a numeric rating scale was assessed at pre-injection, 15 min post-injection, 1 month, and 3 months. Disability was assessed using the Oswestry Neck Disability Index (NDI) questionnaire at pre-injection, 1 month post-injection, and 3 months. In total, 50 patients were followed for 3 months. The mean reductions in pain were: 15 min (77 %), 1 month (39 %), and 3 months (33 %). The mean reductions in NDI were: 1 month (26 %) and 3 months (also 26 %). Results were statistically significant. CT-guided selective cervical nerve root injection in the treatment of cervical radicular pain and related disability produces statistically significant reductions in pain and disability to at least 3 months post-procedure. (orig.)

The short- and medium-term effectiveness of CT-guided selective cervical nerve root injection for pain and disability

International Nuclear Information System (INIS)

Desai, Amidevi; Saha, Shouvik; Sharma, Naveen; Huckerby, Lauren; Houghton, Russell

2014-01-01

CT-guided cervical nerve root injection with corticosteroid and/or local anesthetic is a recognized technique in the evaluation and treatment of cervical radiculopathy. There are few prospective studies on the efficacy of the various techniques employed in cervical nerve root injection. We present our results from a 1-year prospective series using a CT-guided anterolateral transforaminal approach for cervical nerve root injection of bupivacaine and dexamethasone. Pain using a numeric rating scale was assessed at pre-injection, 15 min post-injection, 1 month, and 3 months. Disability was assessed using the Oswestry Neck Disability Index (NDI) questionnaire at pre-injection, 1 month post-injection, and 3 months. In total, 50 patients were followed for 3 months. The mean reductions in pain were: 15 min (77 %), 1 month (39 %), and 3 months (33 %). The mean reductions in NDI were: 1 month (26 %) and 3 months (also 26 %). Results were statistically significant. CT-guided selective cervical nerve root injection in the treatment of cervical radicular pain and related disability produces statistically significant reductions in pain and disability to at least 3 months post-procedure. (orig.)

Model complexity and choice of model approaches for practical simulations of CO₂ injection, migration, leakage and long-term fate

Energy Technology Data Exchange (ETDEWEB)

Celia, Michael A. [Princeton Univ., NJ (United States)

2016-12-30

This report documents the accomplishments achieved during the project titled “Model complexity and choice of model approaches for practical simulations of CO₂ injection,migration, leakage and long-term fate” funded by the US Department of Energy, Office of Fossil Energy. The objective of the project was to investigate modeling approaches of various levels of complexity relevant to geologic carbon storage (GCS) modeling with the goal to establish guidelines on choice of modeling approach.

Fluid injection and induced seismicity

Science.gov (United States)

Kendall, Michael; Verdon, James

2016-04-01

The link between fluid injection, or extraction, and induced seismicity has been observed in reservoirs for many decades. In fact spatial mapping of low magnitude events is routinely used to estimate a stimulated reservoir volume. However, the link between subsurface fluid injection and larger felt seismicity is less clear and has attracted recent interest with a dramatic increase in earthquakes associated with the disposal of oilfield waste fluids. In a few cases, hydraulic fracturing has also been linked to induced seismicity. Much can be learned from past case-studies of induced seismicity so that we can better understand the risks posed. Here we examine 12 case examples and consider in particular controls on maximum event size, lateral event distributions, and event depths. Our results suggest that injection volume is a better control on maximum magnitude than past, natural seismicity in a region. This might, however, simply reflect the lack of baseline monitoring and/or long-term seismic records in certain regions. To address this in the UK, the British Geological Survey is leading the deployment of monitoring arrays in prospective shale gas areas in Lancashire and Yorkshire. In most cases, seismicity is generally located in close vicinity to the injection site. However, in some cases, the nearest events are up to 5km from the injection point. This gives an indication of the minimum radius of influence of such fluid injection projects. The most distant events are never more than 20km from the injection point, perhaps implying a maximum radius of influence. Some events are located in the target reservoir, but most occur below the injection depth. In fact, most events lie in the crystalline basement underlying the sedimentary rocks. This suggests that induced seismicity may not pose a leakage risk for fluid migration back to the surface, as it does not impact caprock integrity. A useful application for microseismic data is to try and forecast induced seismicity

LHC Injection Beam Quality During LHC Run I

CERN Document Server

AUTHOR|(CDS)2079186; Kain, Verena; Stapnes, Steinar

The LHC at CERN was designed to accelerate proton beams from 450 GeV to 7 TeV and collide them in four large experiments. The 450 GeV beam is extracted from the last pre-accelerator, the SPS, and injected into the LHC via two 3 km long transfer lines, TI 2 and TI 8. The injection process is critical in terms of preservation of beam quality and machine protection. During LHC Run I (2009-2013) the LHC was filled with twelve high intensity injections per ring, in batches of up to 144 bunches of 1.7*10^11 protons per bunch. The stored beam energy of such a batch is already an order of magnitude above the damage level of accelerator equipment. Strict quality and machine protection requirements at injection have a significant impact on operational efficiency. During the first years of LHC operation, the injection phase was identified as one of the limiting factors for fast LHC turnaround time. The LHC Injection Quality Check (IQC) software framework was developed as a part of this thesis to monitor the beam quality...

Outcome analysis of two different injection solutions for epidural injection in radicular lumbar backache syndromes

International Nuclear Information System (INIS)

Saqib, M.; Mukhtar, M.; Bhatti, S.N; Afridi, E.A.K.; Khan, R.

2017-01-01

Background: Backache is a significant source of disability and suffering in our society. The treatment modalities need continued enhancement in order to achieve the desired goals of lowering morbidity and financial losses while improving the response of the patient. Methods: This prospective comparative study was conducted at the department of Orthopaedics and Spine Surgery, Khyber Teaching Hospital Peshawar from July 2013 to June 2015. Two interventional groups were designated; Group 1 was comprised of 54 patients who were injected with epidural bupivacaine plus methylprednisolone while Group 2 included 55 patients who were injected with bupivacaine only. Outcome was assessed using the visual analogue scale and Oswestry disability index (ODI). Results: Fifty-five female and 54 male patients with mean age 49.37 years+-10.46 SD, Mean symptoms duration was 15.01 months+-9.32 SD. Common presenting symptoms were backache (77.1 percent), lower limbs pain (66.1 percent), dermatomal paresthesias (54.1 percent) and neurogenic claudication in 57.8 percent patients. The mean visual analogue score (VAS) after injection was 3.18+-1.29 while mean ODI after injection was 23.615. There was a statistically significant reduction in VAS scores (2-sided p=0.003, OR =4.03, 95 percent CI: 1.535-10.60) following the injection. Conclusion: An epidural spinal injection is a viable option for achieving relief of pain and improves functioning in individuals with radicular backache. However, further research is advised in order to clarify the role of ESI for long-term relief. (author)

Long-Term Outcomes in Patients with Neovascular Age-Related Macular Degeneration Who Maintain Dry Macula after Three Monthly Ranibizumab Injections.

Science.gov (United States)

Kim, Kyung Min; Kim, Jae Hui; Chang, Young Suk; Kim, Jong Woo; Kim, Chul Gu

2018-01-01

To evaluate long-term changes in visual acuity and retinal microstructure in patients with neovascular age-related macular degeneration (AMD) who had maintained dry macula after initial treatment. This retrospective observational study included 55 eyes that were diagnosed with neovascular AMD, were treated with three monthly ranibizumab injections, and maintained dry macula during a two-year follow-up. Best-corrected visual acuity (BCVA) at three months and at the final follow-up were compared, and the degree of visual improvement was compared between eyes with and without improvement of the ellipsoid zone. In addition, the incidence of improvement of the ellipsoid zone was compared between eyes with different extents of disruption. The mean follow-up period was 30.3 ± 4.1 months. BCVA at three months and at the final follow-up was 0.51 ± 0.46 and 0.45 ± 0.49 (P200 μm of disruption of the ellipsoid zone, 15 (42.9%) showed improvement of the ellipsoid zone, and the improvement in BCVA was greater in these eyes than that in the remaining 20 eyes (P=0.021). A higher incidence of improvement of the ellipsoid zone was noted in eyes with 200 to 800 μm of disruption than in eyes with >800 μm of disruption (P<0.001). Long-term improvement in visual acuity was noted in eyes that had maintained dry macula after three ranibizumab injections. The status of the ellipsoid zone at three months was closely associated with visual improvement.

Fibrous Myopathy as a Complication of Repeated Intramuscular Injections for Chronic Headache

Directory of Open Access Journals (Sweden)

R Burnham

2006-01-01

Full Text Available Two cases of fibrous myopathy associated with repeated, long-term intramuscular injections for treatment of chronic temporomandibular joint pain and chronic headache, respectively, are described. Both patients developed severe, function-limiting contractures in upper and lower extremity muscles used as injection sites. In one of the cases, the contractures were painful. Electrophysiological testing, magnetic resonance imaging and muscle biopsy results were all consistent with myopathy and replacement of skeletal muscle with noncontractile fibrous tissue. These cases are presented to increase awareness of fibrous myopathy and to promote surveillance for this serious potential complication of long-term intramuscular injections in chronic headache and other pain patients.

Temporal pore pressure induced stress changes during injection and depletion

Science.gov (United States)

Müller, Birgit; Heidbach, Oliver; Schilling, Frank; Fuchs, Karl; Röckel, Thomas

2016-04-01

Induced seismicity is observed during injection of fluids in oil, gas or geothermal wells as a rather immediate response close to the injection wells due to the often high-rate pressurization. It was recognized even earlier in connection with more moderate rate injection of fluid waste on a longer time frame but higher induced event magnitudes. Today, injection-related induced seismicity significantly increased the number of events with M>3 in the Mid U.S. However, induced seismicity is also observed during production of fluids and gas, even years after the onset of production. E.g. in the Groningen gas field production was required to be reduced due to the increase in felt and damaging seismicity after more than 50 years of exploitation of that field. Thus, injection and production induced seismicity can cause severe impact in terms of hazard but also on economic measures. In order to understand the different onset times of induced seismicity we built a generic model to quantify the role of poro-elasticity processes with special emphasis on the factors time, regional crustal stress conditions and fault parameters for three case studies (injection into a low permeable crystalline rock, hydrothermal circulation and production of fluids). With this approach we consider the spatial and temporal variation of reservoir stress paths, the "early" injection-related induced events during stimulation and the "late" production induced ones. Furthermore, in dependence of the undisturbed in situ stress field conditions the stress tensor can change significantly due to injection and long-term production with changes of the tectonic stress regime in which previously not critically stressed faults could turn to be optimally oriented for fault reactivation.

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

Long-Term Carbon Injection Field Test for 90% Mercury Removal for a PRB Unit a Spray Dryer and Fabric Filter

Energy Technology Data Exchange (ETDEWEB)

Sjostrom, Sharon; Amrhein, Jerry

2009-04-30

The power industry in the U.S. is faced with meeting regulations to reduce the emissions of mercury compounds from coal-fired plants. Injecting a sorbent such as powdered activated carbon (PAC) into the flue gas represents one of the simplest and most mature approaches to controlling mercury emissions from coal-fired boilers. The purpose of this test program was to evaluate the long-term mercury removal capability, long-term mercury emissions variability, and operating and maintenance (O&M) costs associated with sorbent injection on a configuration being considered for many new plants. Testing was conducted by ADA Environmental Solutions (ADA) at Rocky Mountain Power’s (RMP) Hardin Station through funding provided by DOE/NETL, RMP, and other industry partners. The Hardin Station is a new plant rated at 121 MW gross that was first brought online in April of 2006. Hardin fires a Powder River Basin (PRB) coal and is configured with selective catalytic reduction (SCR) for NOx control, a spray dryer absorber (SDA) for SO2 control, and a fabric filter (FF) for particulate control. Based upon previous testing at PRB sites with SCRs, very little additional mercury oxidation from the SCR was expected at Hardin. In addition, based upon results from DOE/NETL Phase II Round I testing at Holcomb Station and results from similarly configured sites, low native mercury removal was expected across the SDA and FF. The main goal of this project was met—sorbent injection was used to economically and effectively achieve 90% mercury control as measured from the air heater (AH) outlet to the stack for a period of ten months. This goal was achieved with DARCO® Hg-LH, Calgon FLUEPAC®-MC PLUS and ADA Power PAC PREMIUM brominated activated carbons at nominal loadings of 1.5–2.5 lb/MMacf. An economic analysis determined the twenty-year levelized cost to be 0.87 mills/kW-hr, or $15,000/lb Hg removed. No detrimental effects on other equipment or plant operations were observed. The

The association between neighborhood socioeconomic disadvantage and high-risk injection behavior among people who inject drugs.

Science.gov (United States)

DeCuir, Jennifer; Lovasi, Gina S; El-Sayed, Abdulrahman; Lewis, Crystal Fuller

2018-02-01

Although much research has been conducted on the determinants of HIV risk behavior among people who inject drugs (PWID), the influence of the neighborhood context on high-risk injection behavior remains understudied. To address this gap in the literature, we measured associations between neighborhood socioeconomic disadvantage and high-risk injection behavior, and determined whether these associations were modified by drug-related police activity and syringe exchange program (SEP) accessibility. Our sample was comprised of 484 pharmacy-recruited PWID in New York City. Measures of neighborhood socioeconomic disadvantage were created using data from the 2006-2010 American Community Survey. Associations with high-risk injection behavior were estimated using multivariable Poisson regression. Effect modification by drug-related police activity and SEP accessibility was assessed by entering cross-product terms into adjusted models of high-risk injection behavior. Neighborhood socioeconomic disadvantage was associated with decreased receptive syringe sharing and unsterile syringe use. In neighborhoods with high drug-related police activity, associations between neighborhood disadvantage and unsterile syringe use were attenuated to the null. In neighborhoods with high SEP accessibility, neighborhood disadvantage was associated with decreased acquisition of syringes from an unsafe source. PWID in disadvantaged neighborhoods reported safer injection behaviors than their counterparts in neighborhoods that were relatively better off. The contrasting patterns of effect modification by SEP accessibility and drug-related police activity support the use of harm reduction approaches over law enforcement-based strategies for the control of blood borne virus transmission among PWID in disadvantaged urban areas. Copyright © 2017 Elsevier B.V. All rights reserved.

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.

Risperidone long-acting injection: a review of its long term safety and efficacy

Directory of Open Access Journals (Sweden)

Michael K Rainer

2008-08-01

Full Text Available Michael K RainerMemory-Clinic and Psychiatric Department, Donauspital, Vienna, AustriaAbstract: A long-acting form of the second-generation antipsychotic drug risperidone is now broadly available for the treatment of schizophrenia and closely related psychiatric conditions. It combines the advantage of previously available depot formulations for first-generation drugs with the favorable characteristics of the modern “atypical” antipsychotics, namely higher efficacy in the treatment of the negative symptoms of schizophrenia and reduced motor disturbances. Published clinical studies show an objective clinical efficacy (as per psychiatric symptom scores and relapse data that exceeds that of oral atypical antipsychotics when patients are switched to the long-acting injectable form, a low incidence of treatment-emergent extrapyramidal side effects, and very good acceptance by patients. Available data for maintenance treatment of bipolar disorder show equivalence with the oral form instead of superiority, but are still limited. As it seems likely that efficacy benefits are mostly due to the fact that the injectable form reduces the demand for patient compliance to one physician visit every 2 weeks instead of self-administration on a daily or twice-daily basis, additional potential could exist in other psychiatric disorders where atypical antipsychotic drugs are of benefit but where patient adherence to treatment schedules is typically low.Keywords: risperidone, schizophrenia, psychotic disorders, patient compliance; delayed-action preparations, injections, intramuscular

Intracoronary Injection of CD34-Cells in Chronic Ischemic Heart Failure

DEFF Research Database (Denmark)

Hansen, Morten; Nyby, Sebastian; Eifer Møller, Jacob

2014-01-01

Objectives: Seven years ago, the DanCell study was carried out to test the hypothesis of improvement in left ventricular ejection fraction (LVEF) following repeated intracoronary injections of autologous bone marrow-derived stem cells (BMSCs) in patients suffering from chronic ischemic heart...... was significantly associated with survival (hazard ratio: 0.90, 95% CI: 0.82-1.00, p = 0.04). Conclusions: Intracoronary injections of a high number of CD34(+) cells may have a beneficial effect on chronic ischemic heart failure in terms of long-term survival. © 2014 S. Karger AG, Basel....

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.

Carbon dioxide fluid-flow modeling and injectivity calculations

Science.gov (United States)

Burke, Lauri

2011-01-01

At present, the literature lacks a geologic-based assessment methodology for numerically estimating injectivity, lateral migration, and subsequent long-term containment of supercritical carbon dioxide that has undergone geologic sequestration into subsurface formations. This study provides a method for and quantification of first-order approximations for the time scale of supercritical carbon dioxide lateral migration over a one-kilometer distance through a representative volume of rock. These calculations provide a quantified foundation for estimating injectivity and geologic storage of carbon dioxide.

Randomised controlled trial of local corticosteroid injections for de Quervain's tenosynovitis in general practice

Directory of Open Access Journals (Sweden)

Groenier Klaas H

2009-10-01

Full Text Available Abstract Background De Quervain's tenosynovitis is a stenosing tenosynovitis of the first dorsal compartment of the wrist and leads to wrist pain and to impaired function of the wrist and hand. It can be treated by splinting, local corticosteroid injection and operation. In this study effectiveness of local corticosteroid injections for de Quervain's tenosynovitis provided by general practitioners was assessed. Methods Participants with de Quervain's tenosynovitis were recruited by general practitioners. Short-term outcomes (one week after injections were assessed in a randomised, placebo-controlled trial. Long-term effectiveness was evaluated in an open prospective cohort-study of steroid responders during a follow-up period of 12 months. Participants were randomised to one or two local injections of 1 ml of triamcinolonacetonide (TCA or 1 ml of NaCl 0.9% (placebo. Non-responders to NaCl were treated with additional TCA injections. Main outcomes were immediate treatment response, severity of pain, improvement as perceived by participant and functional disability using sub items hand and finger function of the Dutch Arthritis Impact Measurement Scale (Dutch AIMS-2-HFF. Results 11 general practitioners included 21 wrists in 21 patients. The TCA-group had better results for short-term outcomes treatment response (78% vs. 25%; p = 0.015, perceived improvement (78% vs. 33%; p = 0.047 and severity of pain (4.27 vs. 1.33; p = 0.031 but not for the Dutch-AIMS-HFF (2.71 vs. 1.92; p = 0.112. Absolute risk reduction for the main outcome short-term treatment response was 0.55 (95% CI: 0.34, 0.76 with a number needed to treat of 2 (95% CI: 1, 3. In the cohort of steroid responders (n = 12 the beneficial effects of steroid injections were sustained during the follow-up of 12 months regarding severity of pain (p = 0.67 and scores of Dutch AIMS-2-HFF (p = 0.36, but not for patient perceived improvement (p = 0.02. No adverse events were observed during the 12

The research of new type stratified water injection process intelligent measurement technology

Science.gov (United States)

Zhao, Xin

2017-10-01

To meet the needs of injection and development of Daqing Oilfield, the injection of oil from the early stage of general water injection to the subdivision of water is the purpose of improving the utilization degree and the qualified rate of water injection, improving the performance of water injection column and the matching process. Sets of suitable for high water content of the effective water injection technology supporting technology. New layered water injection technology intelligent measurement technology will be more information testing and flow control combined into a unified whole, long-term automatic monitoring of the work of the various sections, in the custom The process has the characteristics of "multi-layer synchronous measurement, continuous monitoring of process parameters, centralized admission data", which can meet the requirement of subdivision water injection, but also realize the automatic synchronization measurement of each interval, greatly improve the efficiency of tiered injection wells to provide a new means for the remaining oil potential.

Double Tunneling Injection Quantum Dot Lasers for High Speed Operation

Science.gov (United States)

2017-10-23

Double Tunneling-Injection Quantum Dot Lasers for High -Speed Operation The views, opinions and/or findings contained in this report are those of...SECURITY CLASSIFICATION OF: 1. REPORT DATE (DD-MM-YYYY) 4. TITLE AND SUBTITLE 13. SUPPLEMENTARY NOTES 12. DISTRIBUTION AVAILIBILITY STATEMENT 6...State University Title: Double Tunneling-Injection Quantum Dot Lasers for High -Speed Operation Report Term: 0-Other Email: asryan@vt.edu Distribution

Numerical investigation of the effect of injection strategy on mixture formation and combustion process in a port injection natural gas rotary engine

International Nuclear Information System (INIS)

Fan, Baowei; Pan, Jianfeng; Yang, Wenming; Liu, Yangxian; Bani, Stephen; Chen, Wei

2017-01-01

Highlights: • For injection timing, the fuel movement is controlled by the intensity of the vortex I. • For injection duration, the fuel movement is controlled by the value of jet flux. • The ideal fuel distribution at ignition timing for high combustion rate is studied. • The optimal injection strategy had an increase in the peak pressure and NO emissions. - Abstract: This work aimed to numerically study the influence of injection strategy on mixture formation and combustion process in a port injection natural gas rotary engine. On the base of a 3D dynamic simulation model which was established in our previous work, some critical information was obtained, which was difficult to obtain through experiment, in terms of the flow field, the fuel distribution, the temperature field and the concentration fields of some intermediates. Simulation results showed that for mixture formation, the movements of fuel in injection stage were mainly controlled by the intensity of the vortex I for injection timing, and the value of jet flux for injection duration respectively. With retarded injection timing, the decreasing intensity of the vortex I resulted in less fuel moving toward the back of the combustion chamber. With the extension in injection duration, the decreasing value of jet flux resulted in more fuel staying at the back of the combustion chamber. For combustion process, the overall combustion rate for injection strategy which had an injection timing of 390 °CA (BTDC) and injection duration of 51.5 °CA (case ID4) was the fastest. This was mainly due to the fact that the accumulation area of fuel was at the middle and front of the combustion chamber. Meanwhile, fuel concentration near the leading and trailing spark plugs was conducive for the flame kernel formation. Compared with the injection strategy which had an injection timing of 450 °CA (BTDC) and an injection duration of 55 °CA (case IT1), the improved combustion rate of case ID4 had a 23% increase in

Long-term results of percutaneous ethanol injection therapy for hepatocellular carcinoma in cirrhosis: a European experience

International Nuclear Information System (INIS)

Lencioni, R.; Pinto, F.; Armillotta, N.; Bassi, A.M.; Moretti, M.; Di Giulio, M.; Marchi, S.; Uliana, M.; Della Capanna, S.; Lencioni, M.; Bartolozzi, C.

1997-01-01

The objective of our work was to evaluate the long-term results of percutaneous ethanol injection (PEI) for the treatment of hepatocellular carcinoma (HCC) in patients with liver cirrhosis. A total of 184 cirrhotic patients with HCC underwent PEI as the only anticancer treatment over an 8-year period. Patients were followed after therapy by means of clinical examinations, laboratory tests, and US and CT studies performed at regular time intervals. Survival rates were determined according to the Kaplan-Meier method. The overall survival was 67% at 3 years, 41% at 5 years, and 19% at 7 years. The 3-, 5-, and 7-year survival rates of patients with single HCC≤3 cm (78, 54, and 28%, respectively) were significantly higher (p<0.01) than those of patients with single HCC of 3.1-5 cm (61, 32, and 16, respectively) or multiple HCCs (51, 21, and 0%, respectively). Survival of Child-Pugh A patients (79% at 3 years, 53% at 5 years, and 32% at 7 years) was significantly longer (p<0.01) than that of Child-Pugh B patients (50% at 3 years, 28% at 5 years, and 8% at 7 years). A selected group of 70 patients with Child-Pugh A cirrhosis and single HCC≤3 cm had a 7-year survival of 42%. Long-term survival of cirrhotic patients with HCC treated with PEI is comparable to that reported in published series of matched patients submitted to surgical resection. (orig.)

Low pressure powder injection moulding of stainless steel powders

Energy Technology Data Exchange (ETDEWEB)

Zampieron, J.V.; Soares, J.P.; Mathias, F.; Rossi, J.L. [Powder Processing Center CCP, Inst. de Pesquisas Energeticas e Nucleares, Sao Paulo, SP (Brazil); Filho, F.A. [IPEN, Inst. de Pesquisas Energeticas e Nucleares, Cidade Univ., Sao Paulo, SP (Brazil)

2001-07-01

Low-pressure powder injection moulding was used to obtain AISI 316L stainless steel parts. A rheological study was undertaken using gas-atomised powders and binders. The binders used were based on carnauba wax, paraffin, low density polyethylene and microcrystalline wax. The metal powders were characterised in terms of morphology, particle size distribution and specific surface area. These results were correlated to the rheological behaviour. The mixture was injected in the shape of square bar specimens to evaluate the performance of the injection process in the green state, and after sintering. The parameters such as injection pressure, viscosity and temperature were analysed for process optimisation. The binders were thermally removed in low vacuum with the assistance of alumina powders. Debinding and sintering were performed in a single step. This procedure shortened considerably the debinding and sintering time. (orig.)

Non-injection Drug Use and Injection Initiation Assistance among People Who Inject Drugs in Tijuana, Mexico.

Science.gov (United States)

Ben Hamida, Amen; Rafful, Claudia; Jain, Sonia; Sun, Shelly; Gonzalez-Zuniga, Patricia; Rangel, Gudelia; Strathdee, Steffanie A; Werb, Dan

2018-02-01

Although most people who inject drugs (PWID) report receiving assistance during injection initiation events, little research has focused on risk factors among PWID for providing injection initiation assistance. We therefore sought to determine the influence of non-injection drug use among PWID on their risk to initiate others. We used generalized estimating equation (GEE) models on longitudinal data among a prospective cohort of PWID in Tijuana, Mexico (Proyecto El Cuete IV), while controlling for potential confounders. At baseline, 534 participants provided data on injection initiation assistance. Overall, 14% reported ever initiating others, with 4% reporting this behavior recently (i.e., in the past 6 months). In a multivariable GEE model, recent non-injection drug use was independently associated with providing injection initiation assistance (adjusted odds ratio [AOR] = 2.42, 95% confidence interval [CI] = 1.39-4.20). Further, in subanalyses examining specific drug types, recent non-injection use of cocaine (AOR = 9.31, 95% CI = 3.98-21.78), heroin (AOR = 4.00, 95% CI = 1.88-8.54), and methamphetamine (AOR = 2.03, 95% CI = 1.16-3.55) were all significantly associated with reporting providing injection initiation assistance. Our findings may have important implications for the development of interventional approaches to reduce injection initiation and related harms. Further research is needed to validate findings and inform future approaches to preventing entry into drug injecting.

Injection, injectivity and injectability in geothermal operations: problems and possible solutions. Phase I. Definition of the problems

Energy Technology Data Exchange (ETDEWEB)

Vetter, O.J.; Crichlow, H.B.

1979-02-14

The following topics are covered: thermodynamic instability of brine, injectivity loss during regular production and injection operations, injectivity loss caused by measures other than regular operations, heat mining and associated reservoir problems in reinjection, pressure maintenance through imported make-up water, suggested solutions to injection problems, and suggested solutions to injection problems: remedial and stimulation measures. (MHR)

Long term safety, efficacy, and patient acceptability of hyaluronic acid injection in patients with painful osteoarthritis of the knee

Science.gov (United States)

McArthur, Benjamin A; Dy, Christopher J; Fabricant, Peter D; Valle, Alejandro Gonzalez Della

2012-01-01

The increasing prevalence of painful knee osteoarthritis has created an additional demand for pharmacologic management to prevent or delay surgical management. Viscosupplementation, via intraarticular injection of hyaluronic acid (HA), aims to restore the favorable milieu present in the nonarthritic joint. The safety profile of intraarticular HA injections for painful knee osteoarthritis is well established, with the most common adverse effect being a self-limited reaction at the injection site. Although acceptance of the early literature has been limited by publication bias and poor study quality, more recent and rigorous meta-analysis suggests that intraarticular HA injection is superior to placebo injection for pain relief and matches, if not surpasses, the effect size of other nonoperative treatments, such as nonsteroidal anti-inflammatory medication. Intraarticular HA injection is effective in providing temporary pain relief in patients with painful knee osteoarthritis. Future investigations should focus on optimizing the composition and administration of HA agents to provide prolonged relief of painful osteoarthritis in the knee and other joints. PMID:23271899

Autologous blood versus corticosteroid local injection for treatment of Lateral Epicondylosis: A Randomized Clinical Trial

Directory of Open Access Journals (Sweden)

Ajit Singh,

2013-08-01

Full Text Available Objective: The objective of the present single blinded prospective randomized control trial was assessment of efficacy of autologous blood injection versus local steroid injection in treatment of lateral epicondylosis of elbow. Methodology: Using a pre-post experimental design, a total of sixty patients of previously untreated lateral epicondylosis were selected; Group 1 (n=30 was administered single injection of autologous blood and Group 2 (n=30 single local corticosteroid injection. Assessment was done at baseline, 2 weeks, 6 weeks and 12 weeks using PRTEE (Patient Rated Tennis Elbow Evaluation score. Results: Pre injection parameters showed no difference between groups (chi square test, p > 0.005. Analysis between groups showed significant decrease in steroid group at very short term - 2 weeks (unpaired t test, p < 0.005.There was no difference between groups at 6 weeks. There was a significant improvement in blood group at medium term -12 weeks (unpaired t test, p < 0.05. Conclusion: Both the interventions were effective in reducing pain and improving functional status of patients in short term, but autologous blood was more effective in longer run.

Water injection profiling

International Nuclear Information System (INIS)

Arnold, D.M.

1982-01-01

A method of neutron-gamma logging is described, in which water, injected in a cased well borehole with peforations, is irradiated with neutrons of 10 MeV or greater, and subsequent gamma radiation is detected by a pair of detectors along the borehole. Counting rates of detectors are analyzed in terms of two gamma ray energy windows. Linear flow velocity of fluid moving downward within the casing is used in conjunction with count rate data to determine volume flow rates of water moving in other directions. Apparatus includes a sonde with a neutron source and appropriate gamma sensors

Large-Signal Injection-Level Spectroscopy of Impurities in Silicon

International Nuclear Information System (INIS)

Ahrenkiel, R.K.; Johnston, S.W.

1998-01-01

Deep level defects in silicon are identified by measuring the recombination lifetime as a function of the injection level. The basic models for recombination at deep and shallow centers is developed. The defect used for the theoretical model is the well-known interstitial Fe ion in silicon. Data are presented on silicon samples ranging in defect content from intentionally Fe-doped samples to an ultra-pure float-zone grown sample. These data are analyzed in terms of the injection-level spectroscopy model

Testosterone Injection

Science.gov (United States)

... typical male characteristics. Testosterone injection works by supplying synthetic testosterone to replace the testosterone that is normally ... as a pellet to be injected under the skin.Testosterone injection may control your symptoms but will ...

Fat Injection: A Systematic Review of Injection Volumes by Facial Subunit.

Science.gov (United States)

Shue, Shirley; Kurlander, David E; Guyuron, Bahman

2017-08-08

Fat grafting to the aging face has become an integral component of esthetic surgery. However, the amount of fat to inject to each area of the face is not standardized and has been based mainly on the surgeon's experience. The purpose of this study was to perform a systematic review of injected fat volume to different facial zones. A systematic review of the literature was performed through a MEDLINE search using keywords "facial," "fat grafting," "lipofilling," "Coleman technique," "autologous fat transfer," and "structural fat grafting." Articles were then sorted by facial subunit and analyzed for: author(s), year of publication, study design, sample size, donor site, fat preparation technique, average and range of volume injected, time to follow-up, percentage of volume retention, and complications. Descriptive statistics were performed. Nineteen articles involving a total of 510 patients were included. Rhytidectomy was the most common procedure performed concurrently with fat injection. The mean volume of fat injected to the forehead is 6.5 mL (range 4.0-10.0 mL); to the glabellar region 1.4 mL (range 1.0-4.0 mL); to the temple 5.9 mL per side (range 2.0-10.0 mL); to the eyebrow 5.5 mL per side; to the upper eyelid 1.7 mL per side (range 1.5-2.5 mL); to the tear trough 0.65 mL per side (range 0.3-1.0 mL); to the infraorbital area (infraorbital rim to lower lid/cheek junction) 1.4 mL per side (range 0.9-3.0 mL); to the midface 1.4 mL per side (range 1.0-4.0 mL); to the nasolabial fold 2.8 mL per side (range 1.0-7.5 mL); to the mandibular area 11.5 mL per side (range 4.0-27.0 mL); and to the chin 6.7 mL (range 1.0-20.0 mL). Data on exactly how much fat to inject to each area of the face in facial fat grafting are currently limited and vary widely based on different methods and anatomical terms used. This review offers the ranges and the averages for the injected volume in each zone. This journal requires that authors assign a level of evidence

Water injection profiling by nuclear logging

International Nuclear Information System (INIS)

Arnold, D.M.

1980-01-01

This invention relates to nuclear logging techniques for determining the volume flow rates and flow directions of injected water moving behind a wellbore casing. The apparatus includes a sonde equipped with a neutron source and dual radiation detectors. Oxygen in the neutron irradiated water is transmitted to 16 N and the resultant primary and Compton scattered gamma rays are detected in two energy windows by both detectors. Count rate data is analysed in terms of the windows to obtain linear flow velocities for water flow within and behind the casing. Volume flow rates are determined for upward and downward flow, and horizontal volume flow into the surrounding formation is calculated. A complete injection profile may thus be obtained. (U.K.)

A randomized clinical trial on comparison of corticosteroid injection with or without splinting versus saline injection with or without splinting in patients with lateral epicondylitis

Directory of Open Access Journals (Sweden)

Mohammad Ali Tahririan

2014-01-01

Full Text Available Background: Lateral epicondylitis is a common problem affecting 1-3% of the population. There has been much debate about the best treatment modality for this condition. There is, however, no conclusive evidence in support of any of the proposed treatment modalities. In this trial, we have studied the effect of corticosteroid injection (with or without splinting with normal saline injection (with or without splinting. Materials and Methods: In this double-blind, randomized clinical trial, individuals were randomly assigned to either of four treatment groups and received either 40 mg depomedrol injection alone, 40 mg depomedrol injection with splinting, normal saline injection alone, or normal saline injection with splinting. They were evaluated using the visual analog scale (VAS at weeks 2, 4 and 24 and with the Oxford elbow scale (OES at 24 weeks. Results: A total of 79 patients were participated in the study. The corticosteroid injection groups had better pain relief as measured by VAS at 2 and 4 weeks compared with the two saline injection groups. Mean VAS difference at week 0 versus week 2 was 4.5 ± 0.9 and 2.8 ± 0.6 in corticosteroid injection groups and saline injection groups respectively (P < 0.01 but at 24 weeks, there was only moderate benefit reported for the group which received steroid injection and splinting (P < 0.01 compared to the saline injection groups. The saline injection groups reported better improvement in OES scores (20.1 ± 3.7 at the end of the trial compared corticosteroid injection groups (16.1 ± 2.9 (P < 0.05. Conclusion: Our results indicate that despite the clear pain reduction benefit associated with steroid injection in short term, this benefit in comparison with normal saline injection fades by the 24 th week of follow-up.

Spray-controlled combustionprocess with piezo injection; Strahlgefuehrtes Brennverfahren mit Piezo-Benzineinspritzung

Energy Technology Data Exchange (ETDEWEB)

Schaupp, U.; Altenschmidt, F.; Bertsch, D.; Laudenbach, N. [DaimlerChrysler AG, Stuttgart (Germany)

2007-07-01

The novel 2nd generation injection system of Mercedes-Benz have resulted in reduced fuel consumption and lower emissions while improving the engine performance. The piezo technology is a great technical advance. The characteristic field range in which stratified charge operation is possible has been extended since the first generation of injection systems. Lower fuel consumption is achieved not only in normal traffic but also when cruising on motorways at constant speed. The advantages are noticeable not only in the test cycle but also in real operation. The piezo injection valve was not available on the market and had to be constructed, including the 200 bar high-pressure fuel injection system. The stability of the injection system and the good mixing characteristics resulted in an optimally combustible mixture at the spark plug. Apart from stability in stratified charge operation, the possibility of multiple injection also has further advantages and potentials in terms of consumption and emissions. (orig.)

Once-monthly paliperidone injection for the treatment of schizophrenia

Directory of Open Access Journals (Sweden)

Delia Bishara

2010-09-01

Full Text Available Delia BisharaPharmacy Department, South London and Maudsley NHS Foundation Trust, London, United KingdomAbstract: Paliperidone palmitate is a new long-acting antipsychotic injection for the treatment of acute and maintenance therapy in schizophrenia. Paliperidone (9-hydroxyrisperidone is the major active metabolite of risperidone and acts at dopamine D2 and serotonin 5HT2A receptors. As with other atypical antipsychotics, it exhibits a high 5HT2A:D2 affinity ratio. It also has binding activity as an antagonist at α1- and α2 adrenergic receptors and H1 histaminergic receptors, but has virtually no affinity for cholinergic receptors. Paliperidone palmitate has been shown to be effective in reducing Positive and Negative Syndrome Scale total scores in four short-term trials in acute schizophrenia. It was also effective as maintenance therapy in a long-term trial in which time to recurrence of symptoms was significantly longer in paliperidone-treated patients compared with placebo. In addition, paliperidone was shown to be noninferior to risperidone long-acting injection in one study, but this noninferiority was not established in another longer study comparing the two drugs. Treatment should be initiated with 234 mg on day 1 and 156 mg on day 8, followed by a recommended monthly maintenance dose of 39–234 mg based on efficacy and tolerability. Paliperidone palmitate is generally well tolerated, although it can cause weight gain and a rise in prolactin levels, which is generally greater in women than in men. Overall, paliperidone palmitate may have advantages over other currently available long-acting injections, and therefore may be a useful alternative for the treatment of schizophrenia, although further long-term trials comparing it with active treatments are warranted.Keywords: paliperidone palmitate, injection, schizophrenia, long-acting

Evaluation of upward migration around a deep injection well

International Nuclear Information System (INIS)

Chia, Yeeping; Chiu, J.

1994-01-01

The long-term containment of injected wastes in the deep subsurface is expected to be achieved under suitable geologic and hydrologic conditions and by the use of competent engineering practices. Field experiences, however, indicate that waste containment may be affected by hydrologic conduits around the injection well. To assess the potential effects of these conduits, upward migration of injected waste is examined through the use of numerical models under various conditions. Test results indicate that without any preferential hydrologic conduits, most of the injected waste moves laterally in the injection interval, whereas only a small amount of waste migrates upward into the containment interval. When vertical fractures in the disturbed zone or defects in the cement seal around the wellbore exist, the contaminant can move rapidly upward along these conduits to an overlying aquifer, from which it migrates in the lateral direction. The contamination of the overlying aquifer that results from the upward migration of injected waste through these conduits cannot be impeded by a thick, low-permeability containment interval. However, when permeable interbeds exist within the containment interval, a significant portion of the waste migrating upward can be diverted laterally before reaching the overlying aquifer. The front of built-up pressure can reach the aquifer or permeable interbed immediately overlying the injection interval through the preferential hydrologic conduits shortly after the injection starts, but it cannot move farther upward because of pressure dissipation in the permeable formation. This study suggests that the injected waste has the potential to migrate upward into overlying formations through preferential migration conduits around the wellbore

Long-term functional improvements in the 2-year treatment of schizophrenia outpatients with olanzapine long-acting injection

Directory of Open Access Journals (Sweden)

Ascher-Svanum H

2014-06-01

Full Text Available Haya Ascher-Svanum,1 Diego Novick,2,3 Josep Maria Haro,4 Jordan Bertsch,4 David McDonnell,1 Holland Detke11Eli Lilly and Company, Indianapolis, IN, USA; 2Eli Lilly and Company, Windlesham, Surrey, UK; 3Departament de Psiquiatria, Universitat Autonoma de Barcelona, Spain; 4Parc Sanitari Sant Joan de Déu, Centro de Investigación Biomédica en Red en el Área de Salud Mental, Universitat de Barcelona, Barcelona, SpainBackground: Little is known about the long-term changes in the functioning of schizophrenia patients receiving maintenance therapy with olanzapine long-acting injection (LAI, and whether observed changes differ from those seen with oral olanzapine.Methods: This study describes changes in the levels of functioning among outpatients with schizophrenia treated with olanzapine-LAI compared with oral olanzapine over 2 years. This was a secondary analysis of data from a multicenter, randomized, open-label, 2-year study comparing the long-term treatment effectiveness of monthly olanzapine-LAI (405 mg/4 weeks; n=264 with daily oral olanzapine (10 mg/day; n=260. Levels of functioning were assessed with the Heinrichs–Carpenter Quality of Life Scale. Functional status was also classified as “good”, “moderate”, or “poor”, using a previous data-driven approach. Changes in functional levels were assessed with McNemar’s test and comparisons between olanzapine-LAI and oral olanzapine employed the Student’s t-test. Results: Over the 2-year study, the patients treated with olanzapine-LAI improved their level of functioning (per Quality of Life total score from 64.0–70.8 (P<0.001. Patients on oral ­olanzapine also increased their level of functioning from 62.1–70.1 (P<0.001. At baseline, 19.2% of the olanzapine-LAI-treated patients had a “good” level of functioning, which increased to 27.5% (P<0.05. The figures for oral olanzapine were 14.2% and 24.5%, respectively (P<0.001. Results did not significantly differ between

Safe injection procedures, injection practices, and needlestick ...

African Journals Online (AJOL)

Nermine Mohamed Tawfik Foda

2017-01-10

Jan 10, 2017 ... sures regarding disposable injection equipment, waste containers, hand hygiene ... injection practices lead to high prevalence of NSSIs in operating rooms. .... guidelines, the availability of training courses to HCWs, and provi-.

Long-Term Prognosis of Plantar Fasciitis

DEFF Research Database (Denmark)

Hansen, Liselotte; Krogh, Thøger Persson; Ellingsen, Torkell

2018-01-01

, exercise-induced symptoms, bilateral heel pain, fascia thickness, and presence of a heel spur) could predict long-term outcomes, (3) to assess the long-term ultrasound (US) development in the fascia, and (4) to assess whether US-guided corticosteroid injections induce atrophy of the heel fat pad. Study....... The risk was significantly greater for women (P heel...... regardless of symptoms and had no impact on prognosis, and neither did the presence of a heel spur. Only 24% of asymptomatic patients had a normal fascia on US at long-term follow-up. A US-guided corticosteroid injection did not cause atrophy of the heel fat pad. Our observational study did not allow us...

Long-term follow-up of corticosteroid injection for traumatic olecranon bursitis.

Science.gov (United States)

Weinstein, P S; Canoso, J J; Wohlgethan, J R

1984-01-01

Forty-seven patients with traumatic olecranon bursitis were evaluated after a mean follow-up of 31 months (range 6 to 62 months). Twenty-two patients treated with bursal aspiration had delayed recovery and no complications of therapy. Twenty-five patients treated with intrabursal injection of 20 mg of triamcinolone hexacetonide had rapid recovery, usually within one week, but suffered complications such as infection (3 cases), skin atrophy (5 cases), and chronic local pain (7 cases). Since spontaneous resolution can be expected, a conservative approach is suggested in the treatment of traumatic olecranon bursitis. Images PMID:6696516

Effect of caudal epidural steroid or saline injection in chronic lumbar radiculopathy: multicentre, blinded, randomised controlled trial

DEFF Research Database (Denmark)

Iversen, Trond; Solberg, Tore K; Romner, Bertil

2011-01-01

To assess the efficacy of caudal epidural steroid or saline injection in chronic lumbar radiculopathy in the short (6 weeks), intermediate (12 weeks), and long term (52 weeks).......To assess the efficacy of caudal epidural steroid or saline injection in chronic lumbar radiculopathy in the short (6 weeks), intermediate (12 weeks), and long term (52 weeks)....

Subcutaneous Injection of Oxyfluorfen Herbicide to the Forearm: Case Report

OpenAIRE

Couceiro, José; Garcia-Portal, Gonzalo; Garcia, Olga

2017-01-01

Background  Oxyfluorfen, a commercially available pesticide, commonly used for weed control in crop production, has been studied in terms of its toxicity, its carcinogenic properties, and its teratogenicity. We have found no reports, however, of the effects produced by an oxyfluorfen injection to the upper limb. Methods  We present the case of a 40 years old psychiatric patient, who reportedly injected her forearm accidentally while fumigating her garden. She was treated with irrigation and o...

Emission Constrained Multiple-Pulse Fuel Injection Optimisation and Control for Fuel-Efficient Diesel Engines

NARCIS (Netherlands)

Luo, X.; Jager, B. de; Willems, F.P.T.

2015-01-01

With the application of multiple-pulse fuel injection profiles, the performance of diesel engines is enhanced in terms of low fuel consumption and low engine-out emission levels. However, the calibration effort increases due to a larger number of injection timing parameters. The difficulty of

Long term management of patients with cryopyrin-associated periodic syndromes (CAPS): focus on rilonacept (IL-1 Trap).

Science.gov (United States)

Church, Leigh D; Savic, Sinisa; McDermott, Michael F

2008-12-01

Cryopyrin-associated periodic syndromes (CAPS) are a group of inherited inflammatory disorders consisting of familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID; also known as chronic infantile neurologic, cutaneous, articular [CINCA] syndrome). These rare disorders are associated with heterozygous mutations in the NLRP3 (CIAS1) gene, which encodes the protein NALP3 or cryopyrin, and inflammation driven by excessive production of the cytokine interleukin-1beta (IL-1beta). Amyloidosis is a serious complication with 25% of MWS patients developing amyloidosis, with occasional fatal consequences, whilst up to 20% of CINCA/NOMID patients die from various complications, before reaching the early adulthood. In some CINCA/NOMID adult survivors amyloidosis can also occur. Prior to the discovery of the CIAS1 gene mutations and the advent of IL-1 targeted therapy, treatment was aimed at suppressing inflammation, with limited success. The selective blockade of IL-1beta, with anakinra (IL-1 receptor antagonist), not only provided supportive evidence for the role of IL-1beta in CAPS, but also demonstrated the efficacy of targeting IL-1beta for treatment of these conditions. In February, 2008, 'Orphan Drug' approval from the Food and Drug Administration (FDA) for rilonacept (IL-1 Trap/Arcalyst(), Regeneron Pharmaceuticals, Inc) was given for the treatment of two CAPS disorders, FCAS and MWS in adults and children 12 years and older, making rilonacept the first therapy approved for the treatment of CAPS.

Short-term effect of ultrasound-guided low-molecular-weight hyaluronic acid injection on clinical outcomes and imaging changes in patients with rheumatoid arthritis of the ankle and foot joints. A randomized controlled pilot trial.

Science.gov (United States)

Wang, Chien-Chih; Lee, Si-Huei; Lin, Hsiao-Yi; Liu, Fu-Wei; Chiou, Hong-Jen; Chan, Rai-Chi; Chou, Chen-Liang

2017-11-01

To determine whether hyaluronic acid (HA) injection into rheumatoid arthritis ankles and feet can achieve improvement in foot function and reduce synovial hyper-vascularization. Forty-four patients with RA having unilateral or bilateral painful ankle and foot involvement (N = 75) were studied. All the patients were randomized to receive HA (N = 40) or lidocaine (LI) (N = 35) injection at 2-week intervals; Clinical assessments were performed using a visual analog scale (VAS) and foot function index (FFI total ) including subscales of pain (FFI pain) before injection at baseline, 4 weeks (first evaluation) and 12 weeks (secondary evaluation). Imaging evaluation based on color Doppler ultrasound (CDUS) and synovitis scores was performed simultaneously. HA injection improved the VAS score (p = .009), FFI pain (p = .041), and FFI total (p = .032) considerably more than LI injections did at the first evaluation. The CDUS values at first evaluation (p = .005) and secondary evaluation (p injections reduced the CDUS values of more than half of the joints (54%, p = .042) while the control group exhibited no change (20%, p = .56). However, HA injection did not reduce the CDUS values more than LI injection did. Regarding the evaluation of synovial hypertrophy, no significant difference was observed between or within the groups in the synovitis scores. HA injection improved short-term foot function and pain reduction. HA injection may have a modest effect in reducing synovial hyper-vascularization. Further large-scale study is warranted to confirm this result.

Study of status of safe injection practice and knowledge regarding injection safety among primary health care workers in Baglung district, western Nepal.

Science.gov (United States)

Gyawali, Sudesh; Rathore, Devendra S; Kc, Bhuvan; Shankar, P Ravi

2013-01-03

Unsafe injection practices and injection overuse are widespread in developing countries harming the patient and inviting risks to the health care workers. In Nepal, there is a dearth of documented information about injection practices so the present study was carried out: a) to determine whether the selected government health facilities satisfy the conditions for safe injections in terms of staff training, availability of sterile injectable equipment and their proper disposal after use and b) to assess knowledge and attitudes of healthcare workers in these health care facilities with regard to injection safety. A descriptive cross-sectional mixed type (qualitative and quantitative) survey was carried out from 18th May to 16th June 2012. In-depth interviews with the in-charges were conducted using a semi-structured questionnaire. Observation of the health facilities using a structured observation tool was done. The data were analysed manually by summarizing, tabulating and presenting in various formats. The in-charges (eight males, two females) who participated in the study ranged in age from 30 to 50 years with a mean age of 37.8 years. Severe infection followed by pain was the most important cause for injection use with injection Gentamicin being most commonly prescribed. New single use (disposable) injections and auto-disable syringes were used to inject curative drugs and vaccines respectively. Sufficient safety boxes were also supplied to dispose the used syringe. All health care workers had received full course of Hepatitis B vaccine and were knowledgeable about at least one pathogen transmitted through unsafe injection practices. Injection safety management policy and waste disposal guideline was not available for viewing in any of the facilities. The office staff who disposed the bio-medical wastes did so without taking any safety measures. Moreover, none of these staff had received any formal training in waste management. Certain safe injection

Study of status of safe injection practice and knowledge regarding injection safety among primary health care workers in Baglung district, western Nepal

Directory of Open Access Journals (Sweden)

Gyawali Sudesh

2013-01-01

Full Text Available Abstract Background Unsafe injection practices and injection overuse are widespread in developing countries harming the patient and inviting risks to the health care workers. In Nepal, there is a dearth of documented information about injection practices so the present study was carried out: a to determine whether the selected government health facilities satisfy the conditions for safe injections in terms of staff training, availability of sterile injectable equipment and their proper disposal after use and b to assess knowledge and attitudes of healthcare workers in these health care facilities with regard to injection safety. Methodology A descriptive cross-sectional mixed type (qualitative and quantitative survey was carried out from 18th May to 16th June 2012. In-depth interviews with the in-charges were conducted using a semi-structured questionnaire. Observation of the health facilities using a structured observation tool was done. The data were analysed manually by summarizing, tabulating and presenting in various formats. Results The in-charges (eight males, two females who participated in the study ranged in age from 30 to 50 years with a mean age of 37.8 years. Severe infection followed by pain was the most important cause for injection use with injection Gentamicin being most commonly prescribed. New single use (disposable injections and auto-disable syringes were used to inject curative drugs and vaccines respectively. Sufficient safety boxes were also supplied to dispose the used syringe. All health care workers had received full course of Hepatitis B vaccine and were knowledgeable about at least one pathogen transmitted through unsafe injection practices. Injection safety management policy and waste disposal guideline was not available for viewing in any of the facilities. The office staff who disposed the bio-medical wastes did so without taking any safety measures. Moreover, none of these staff had received any formal

Permeability of fissured rock - an experimental study with special regard to the water injection test

International Nuclear Information System (INIS)

Schneider, H.J.

1987-01-01

The permeability to water of fissured rock is one of the most important design parameters for many underground projects, such as, e.g. the final deposition of radioactive waste. Because the conventional water injection test according to LUGEON for the calculation of permeability to water is associated with a high degree of uncertainty, new test equipment was developed. This equipment works on the principle of the water injection tracer test and multi-level measurements, enabling detailed measurement of the flow process at injection site and in the rock. The tests were carried out in Bunter sandstone and granite. The LUGEON test concept was varied in short-term and long-term tests at identical geological boundary conditions, and with test control at constant pressure on the one hand and at constant injection volume on the other. The test results show that non-steady-state flow occurs with short injection times, whereby the range is limited to the local rock at injection site. An increasing in injection time can lead to an increase in range by a number of factors as well as to steady-state flow conditions. The permeability of the rock types investigated is inhomgeneous and anistropic as a result of the fissured structure. (orig./HP) With 114 figs., 4 tabs [de

Assessment of MRI Contrast Agent Kinetics via Retro-Orbital Injection in Mice: Comparison with Tail Vein Injection.

Science.gov (United States)

Wang, Fang; Nojima, Masanori; Inoue, Yusuke; Ohtomo, Kuni; Kiryu, Shigeru

2015-01-01

It is not known whether administration of contrast agent via retro-orbital injection or the tail vein route affects the efficiency of dynamic contrast-enhanced magnetic resonance imaging (MRI). Therefore, we compared the effects of retro-orbital and tail vein injection on the kinetics of the contrast agent used for MRI in mice. The same group of nine healthy female mice received contrast agent via either route. An extracellular contrast agent was infused via the tail vein and retro-orbital vein, in random order. Dynamic contrast-enhanced MRI was performed before and after administering the contrast agent. The contrast effects in the liver, kidney, lung, and myocardium were assessed. The average total times of venous puncture and mounting of the injection system were about 10 and 4 min for the tail vein and retro-orbital route, respectively. For all organs assessed, the maximum contrast ratio occurred 30 s after administration and the time course of the contrast ratio was similar with either routes. For each organ, the contrast ratios correlated strongly; the contrast ratios were similar. The retro-orbital and tail vein routes afforded similar results in terms of the kinetics of the contrast agent. The retro-orbital route can be used as a simple efficient alternative to tail vein injection for dynamic contrast-enhanced MRI of mice.

Acupuncture Injection Combined with Electrokinetic Injection for Polydimethylsiloxane Microfluidic Devices

Directory of Open Access Journals (Sweden)

Ji Won Ha

2017-01-01

Full Text Available We recently reported acupuncture sample injection that leads to reproducible injection of nL-scale sample segments into a polydimethylsiloxane (PDMS microchannel for microchip capillary electrophoresis. The advantages of the acupuncture injection in microchip capillary electrophoresis include capability of minimizing sample loss and voltage control hardware and capability of introducing sample plugs into any desired position of a microchannel. However, the challenge in the previous study was to achieve reproducible, pL-scale sample injections into PDMS microchannels. In the present study, we introduce an acupuncture injection technique combined with electrokinetic injection (AICEI technique to inject pL-scale sample segments for microchip capillary electrophoresis. We carried out the capillary zone electrophoresis (CZE separation of FITC and fluorescein, and the mixture of 10 μM FITC and 10 μM fluorescein was separated completely by using the AICEI method.

Comparison of Microbial Community Compositions of Injection and Production Well Samples in a Long-Term Water-Flooded Petroleum Reservoir

Science.gov (United States)

Ren, Hong-Yan; Zhang, Xiao-Jun; Song, Zhi-yong; Rupert, Wieger; Gao, Guang-Jun; Guo, Sheng-xue; Zhao, Li-Ping

2011-01-01

Water flooding plays an important role in recovering oil from depleted petroleum reservoirs. Exactly how the microbial communities of production wells are affected by microorganisms introduced with injected water has previously not been adequately studied. Using denaturing gradient gel electrophoresis (DGGE) approach and 16S rRNA gene clone library analysis, the comparison of microbial communities is carried out between one injection water and two production waters collected from a working block of the water-flooded Gudao petroleum reservoir located in the Yellow River Delta. DGGE fingerprints showed that the similarities of the bacterial communities between the injection water and production waters were lower than between the two production waters. It was also observed that the archaeal composition among these three samples showed no significant difference. Analysis of the 16S rRNA gene clone libraries showed that the dominant groups within the injection water were Betaproteobacteria, Gammaproteobacteria and Methanomicrobia, while the dominant groups in the production waters were Gammaproteobacteria and Methanobacteria. Only 2 out of 54 bacterial operational taxonomic units (OTUs) and 5 out of 17 archaeal OTUs in the injection water were detected in the production waters, indicating that most of the microorganisms introduced by the injection water may not survive to be detected in the production waters. Additionally, there were 55.6% and 82.6% unique OTUs in the two production waters respectively, suggesting that each production well has its specific microbial composition, despite both wells being flooded with the same injection water. PMID:21858049

Compressed air injection technique to standardize block injection pressures.

Science.gov (United States)

Tsui, Ban C H; Li, Lisa X Y; Pillay, Jennifer J

2006-11-01

Presently, no standardized technique exists to monitor injection pressures during peripheral nerve blocks. Our objective was to determine if a compressed air injection technique, using an in vitro model based on Boyle's law and typical regional anesthesia equipment, could consistently maintain injection pressures below a 1293 mmHg level associated with clinically significant nerve injury. Injection pressures for 20 and 30 mL syringes with various needle sizes (18G, 20G, 21G, 22G, and 24G) were measured in a closed system. A set volume of air was aspirated into a saline-filled syringe and then compressed and maintained at various percentages while pressure was measured. The needle was inserted into the injection port of a pressure sensor, which had attached extension tubing with an injection plug clamped "off". Using linear regression with all data points, the pressure value and 99% confidence interval (CI) at 50% air compression was estimated. The linearity of Boyle's law was demonstrated with a high correlation, r = 0.99, and a slope of 0.984 (99% CI: 0.967-1.001). The net pressure generated at 50% compression was estimated as 744.8 mmHg, with the 99% CI between 729.6 and 760.0 mmHg. The various syringe/needle combinations had similar results. By creating and maintaining syringe air compression at 50% or less, injection pressures will be substantially below the 1293 mmHg threshold considered to be an associated risk factor for clinically significant nerve injury. This technique may allow simple, real-time and objective monitoring during local anesthetic injections while inherently reducing injection speed.

"Injection first": a unique group of injection drug users in Tijuana, Mexico.

Science.gov (United States)

Morris, Meghan D; Brouwer, Kimberly C; Lozada, Remedios M; Gallardo, Manuel; Vera, Alicia; Strathdee, Steffanie A

2012-01-01

Using baseline data from a study of injection drug users (IDUs) in Tijuana, Mexico (N = 1,052), we identified social and behavioral factors associated with injecting at the same age or earlier than other administration routes of illicit drug use (eg, "injection first") and examined whether this IDU subgroup had riskier drug using and sexual behaviors than other IDUs. Twelve-percent "injected first." Characteristics independently associated with a higher odds of "injection first" included being younger at first injection, injecting heroin as their first drug, being alone at the first injection episode, and having a sexual debut at the same age or earlier as when they initiated drug use; family members' illicit drug use was associated with lower odds of injecting first. When adjusting for age at first injection and number of years injecting, "injection first" IDUs had lower odds of ever overdosing, and ever trading sex. On the other hand, they were less likely to have ever been enrolled in drug treatment, and more commonly obtained their syringes from potentially unsafe sources. In conclusion, a sizable proportion of IDUs in Tijuana injected as their first drug using experience, although evidence that this was a riskier subgroup of IDUs was inconclusive.  Copyright © American Academy of Addiction Psychiatry.

Prediction of the extent of formation damage caused by water injection

Energy Technology Data Exchange (ETDEWEB)

Al-Homadhi, Emad S. [King Saud Univ., Riyadh (Saudi Arabia). Petroleum Engineering Dept.

2013-06-15

As a general practice water is injected along the O/W contact to maintain reservoir pressure during production. Down hole analysis of the injected water shows that, even after surface treatment, it still can contain a considerable amount of solid particles. These particles can bridge formation pores and cause a considerable reduction in the injectivity. To ensure good injectivity over a longer term, the concentration and size of these solids should not exceed certain limits. In this article core flood tests were carried out to simulate high rate injectors. The injected brine contained solid particles in different concentrations and sizes. Particle concentration was between 5 and 20 ppm and the particle mean size was between 2 and 9 {mu}m. The results were presented as damaging ratio versus pore volume injected. Contrarily to previous studies instead of using experimental results in calibrating or evaluating certain theoretical models, the results in this study were directly fitted to produce equations which can predict the extent of damage caused by injected water by knowing the mean size and concentration of the solid particles contained in that water. (orig.)

Trapping of gun-injected plasma by a tokamak

International Nuclear Information System (INIS)

Leonard, A.W.; Dexter, R.N.; Sprott, J.C.

1986-10-01

It is shown that a plasma produced by a Marshall gun can be injected into and trapped by a tokamak plasma. Gun injection raises the line-averaged density and peaks the density profile. Trapping of the gun-injected plasma is explainable in terms of a depolarization current mechanism. A model is developed which describes the slowing of a plasma beam crossing into the magnetic field of a tokamak. The slowing down time is shown to go as tau/sub s/ ∞ n -1 /sub b/T 3 /sub e/(α 0 /L) 2 , where n/sub b/ and T/sub e/ are the density and temperature of the plasma beam and α 0 /L is the pitch of the field lines per unit length in the direction in which the beam is traveling. Experimental tests of this model are consistent with the scaling predictions

Randomised, prospective, non-blinded pilot study comparing the effect of intramuscular steroid injections and intralesional steroid injections in the management of tennis elbow

Science.gov (United States)

Tahir, Hasan; Biro, Izolda; Donnelly, Simon; Greenwood, Mandy

2016-01-01

Background Tennis elbow is an overuse injury affecting people performing repetitive forearm movements. It is a soft tissue disorder that causes significant disability and pain. The aim of the study was to establish that an intramuscular steroid injection is effective in the short-term pain relief and functional improvement of tennis elbow. The severity of pain at the injection site was monitored to determine whether the intramuscular injection is better tolerated than the intralesional injection. Methods and results 19 patients, who had no treatment for tennis elbow in the preceding 3 months, were recruited from Whipps Cross University Hospital, London, and were randomised to receive either 80 mg of intramuscular Depo-Medrone or 40 mg of intralesional Depo-Medrone injection. Blinding proved difficult as the injection sites differed and placebo arms were not included in the study. A Patient-Rated Tennis Elbow Evaluation (PRTEE) Questionnaire and a 10-point Likert scale were used to assess primary outcome. Six weeks after the treatment, there was a reduction in pain, improvement in function and total PRTEE scores in both intramuscular and intralesional groups (p=0.008) using a 95% CI for mean treatment difference of −26 to +16 points. A statistically significant result (p=0.001) in favour of intramuscular causing less pain at the injection site was noted. Conclusion Non-inferiority of intramuscular to intralesional injections was not confirmed; however, the intramuscular injection proved to be effective in reducing tennis elbow-related symptoms and was found less painful at the site of injection at the time of administration. Trial registration number EUDRACT Number: 2010-022131-11. REC Number: 10/H0718/76 (NRES, Central London REC 1). PMID:28879024

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.

Evaluation of performance, safety, subject acceptance, and compliance of a disposable autoinjector for subcutaneous injections in healthy volunteers.

Science.gov (United States)

Berteau, Cecile; Schwarzenbach, Florence; Donazzolo, Yves; Latreille, Mathilde; Berube, Julie; Abry, Herve; Cotten, Joël; Feger, Celine; Laurent, Philippe E

2010-10-05

A disposable autoinjector was developed for subcutaneous (SC) self-injection by patients with chronic diseases. To verify its performance and evaluate its acceptance, a clinical study was conducted in healthy volunteers, comparing SC injections performed by subjects using the autoinjector with SC injections performed by nurses using a syringe. This was a randomized, single-center, crossover study comparing SC self-injection using an autoinjector with SC nurse-administered injection using a syringe. Two volumes (0.2 mL and 1 mL) were injected into healthy volunteers. Study objectives included assessment of the accuracy and consistency of the volume injected by the injection systems, and skin reaction and pain associated with the injection. The fluid depot in the SC tissue layer was evaluated by ultrasound. Subject acceptance was evaluated using questionnaires on attitudes and emotions towards the injection technique, and challenged by seeking the subjects' preferred system for a final study injection or future treatment. A total of 960 injections (480 with autoinjector, 480 with syringe) were performed in 40 subjects. There were no significant differences in mean fluid leakage and injected volumes between the systems. Pain associated with the injection was significantly lower with the auto-injector than with the syringe. Local skin reaction at the injection site was overall satisfactory. Injections were appropriately performed by all subjects. At study end, all 40 subjects preferred the autoinjector for a final study injection and for future treatment. This study indicated that the autoinjector used by the subject was similar to a syringe used by a nurse in terms of performance and safety in administering the injections, and better in terms of pain, overall acceptance, and preference.

Comparative study between botulinum toxin injection and partial division of puborectalis for treating anismus.

Science.gov (United States)

Farid, Mohamed; Youssef, Tamer; Mahdy, Tarek; Omar, Waleed; Moneim, Hesham Abdul; El Nakeeb, Ayman; Youssef, Mohamed

2009-03-01

The objective of this study was to compare the results of partial division of puborectalis (PDPR) versus local botulinum toxin type A (BTX-A) injection in treating patients with anismus. This prospective randomized study included 30 male patients suffering from anismus. Diagnosis was made by clinical examination, barium enema, colonoscopy, colonic transit time, anorectal manometry, balloon expulsion test, defecography, and electromyography. Patients were randomized into: group I which included 15 patients who were injected with BTX-A and group II which included 15 patients who underwent bilateral PDPR. Follow-up was conducted for about 1 year. Improvement was considered when patients returned to their normal habits. BTX-A injection achieved initial success in 13 patients (86.7%). However, long-term success persisted only in six patients (40%). This was in contrast to PDPR which achieved initial success in all patients (100%) with a long-term success in ten patients (66.6%). Recurrence was observed in seven patients (53.8%) and five patients (33.4%) following BTX-A injection and PDPR, respectively. Minor degrees of incontinence were confronted in two patients (13.3%) following PDPR. BTX-A injection seems to be successful for temporary treatment of anismus.

Safe injection procedures, injection practices, and needlestick ...

African Journals Online (AJOL)

Nermine Mohamed Tawfik Foda

2017-01-10

Jan 10, 2017 ... Background: Of the estimated 384,000 needle-stick injuries occurring in hospitals each year, 23% occur in surgical settings. This study was conducted to assess safe injection procedures, injection practices, and circumstances contributing to needlestick and sharps injures (NSSIs) in operating rooms.

The effect of hot water injection on sandstone permeability

DEFF Research Database (Denmark)

Rosenbrand, Esther; Haugwitz, Christian; Jacobsen, Peter Sally Munch

2014-01-01

Seasonal energy storage can be achieved by hot water injection in geothermal sandstone aquifers. We present an analysis of literature data in combination with new short-term flow through permeability experiments in order to address physical and physico-chemical mechanisms that can alter...

FURNACE INJECTION OF ALKALINE SORBENTS FOR SULFURIC ACID REMOVAL

Energy Technology Data Exchange (ETDEWEB)

Gary M. Blythe

2004-01-01

The objective of this project has been to demonstrate the use of alkaline reagents injected into the furnace of coal-fired boilers as a means of controlling sulfuric acid emissions. The project was co-funded by the U.S. DOE National Energy Technology Laboratory under Cooperative Agreement DE-FC26-99FT40718, along with EPRI, the American Electric Power Company (AEP), FirstEnergy Corporation, the Tennessee Valley Authority, and Carmeuse North America. Sulfuric acid controls are becoming of increased interest for coal-fired power generating units for a number of reasons. In particular, sulfuric acid can cause plant operation problems such as air heater plugging and fouling, back-end corrosion, and plume opacity. These issues will likely be exacerbated with the retrofit of selective catalytic reduction (SCR) for NOX control, as SCR catalysts are known to further oxidize a portion of the flue gas SO{sub 2} to SO{sub 3}. The project tested the effectiveness of furnace injection of four different magnesium-based or dolomitic alkaline sorbents on full-scale utility boilers. These reagents were tested during one- to two-week tests conducted on two FirstEnergy Bruce Mansfield Plant (BMP) units. One of the sorbents tested was a magnesium hydroxide slurry byproduct from a modified Thiosorbic{reg_sign} Lime wet flue gas desulfurization process. The other three sorbents are available commercially and include dolomite, pressure-hydrated dolomitic lime, and commercially available magnesium hydroxide. The dolomite reagent was injected as a dry powder through out-of-service burners. The other three reagents were injected as slurries through air-atomizing nozzles inserted through the front wall of the upper furnace. After completing the four one- to two-week tests, the most promising sorbents were selected for longer-term (approximately 25-day) full-scale tests on two different units. The longer-term tests were conducted to confirm sorbent effectiveness over extended operation on two

Evaluation the short term effects on serum creatinine concentration in patients with normal renal function, mild and moderate renal insufficiency after intravenous injection of gadopentetate dimeglumine

International Nuclear Information System (INIS)

Luo Jian; Liu Jing; Wang Xiaoying; Yang Xuedong; Jiang Xuexiang

2010-01-01

Objective: To explore the effects of gadopentetate dimeglumine injection on renal function. Methods: The study included 623 consecutive patients. Their serum creatinine concentrations before and within 3 days after injection of gadopentetate dimeglumine were analyzed. Their eGFR (estimated glomerular filtration rate) were calculated. Patients were divided into two groups according to their injection dose: group received single dose and group received double dose. Patients in each group were subdivided into three subgroups according to their eGFR: subgroup of normal renal function, subgroup of mild renal insufficiency and subgroup of moderate renal insufficiency. Paried sample t test and group design paired sample Rank Sum test were used for statistical analysis. Results: There was no adverse reaction or occurrence of acute renal insufficiency. The mean level of serum creatinine dropped from (74.0± 17.2) μmol/L to (71.5±19.0) μmol/L (t=5.39, P 0.05) in subgroup of mild renal insufficiency under group received double dose and increased from (118.3±15.3) μmol/L to (135.7±8.5) μmol/L (t=2.02, P<0.05) in subgroup of moderate renal insufficiency under group received double dose, the mean level of serum creatinine dropped in all other subgroups. Conclusions: Single dose gadopentetate dimeglumine is safe for patients with normal renal function and mild, moderate renal insufficiency in short term, but patients with abnormal renal function should be followed up after double dose injection. (authors)

Case study on ground surface deformation induced by CO2 injection into coal seam

International Nuclear Information System (INIS)

Li Hong; Tang Chun'an

2010-01-01

To monitor a geomechanical response of injecting CO 2 into relatively shallow coal seams, tiltmeters were set as an array to cover the ground surface area surrounding the injection well, and to measure the ground deformation during a well fracturing stimulation and a short-term CO 2 injection test. In this paper, an attempt to establish a quantitative relationship between the in-situ coal swelling and the corresponding ground deformation was made by means of numerical simulation study. (authors)

Ring current energy injection rate and solar wind-magnetosphere energy coupling

International Nuclear Information System (INIS)

Lee, L.C.; Kan, J.R.; Akasofu, S.-I.

1982-01-01

The purpose of this paper is to (i) formulate the ring current injection rate Usub(R) in terms of phisub(CT) (cross-tail potential drop) by assuming that the ring current formation is a direct consequence of an enhanced convection, (ii) examine the relationship between the injection rate Usub(R) and the power transferred from the solar wind to the magnetosphere and (iii) demonstrate that an enhanced convection indeed leads to the formation of the ring current. (author)

A contoured gap coaxial plasma gun with injected plasma armature.

Science.gov (United States)

Witherspoon, F Douglas; Case, Andrew; Messer, Sarah J; Bomgardner, Richard; Phillips, Michael W; Brockington, Samuel; Elton, Raymond

2009-08-01

A new coaxial plasma gun is described. The long term objective is to accelerate 100-200 microg of plasma with density above 10(17) cm(-3) to greater than 200 km/s with a Mach number above 10. Such high velocity dense plasma jets have a number of potential fusion applications, including plasma refueling, magnetized target fusion, injection of angular momentum into centrifugally confined mirrors, high energy density plasmas, and others. The approach uses symmetric injection of high density plasma into a coaxial electromagnetic accelerator having an annular gap geometry tailored to prevent formation of the blow-by instability. The injected plasma is generated by numerous (currently 32) radially oriented capillary discharges arranged uniformly around the circumference of the angled annular injection region of the accelerator. Magnetohydrodynamic modeling identified electrode profiles that can achieve the desired plasma jet parameters. The experimental hardware is described along with initial experimental results in which approximately 200 microg has been accelerated to 100 km/s in a half-scale prototype gun. Initial observations of 64 merging injector jets in a planar cylindrical testing array are presented. Density and velocity are presently limited by available peak current and injection sources. Steps to increase both the drive current and the injected plasma mass are described for next generation experiments.

A contoured gap coaxial plasma gun with injected plasma armature

International Nuclear Information System (INIS)

Witherspoon, F. Douglas; Case, Andrew; Messer, Sarah J.; Bomgardner, Richard II; Phillips, Michael W.; Brockington, Samuel; Elton, Raymond

2009-01-01

A new coaxial plasma gun is described. The long term objective is to accelerate 100-200 μg of plasma with density above 10 17 cm -3 to greater than 200 km/s with a Mach number above 10. Such high velocity dense plasma jets have a number of potential fusion applications, including plasma refueling, magnetized target fusion, injection of angular momentum into centrifugally confined mirrors, high energy density plasmas, and others. The approach uses symmetric injection of high density plasma into a coaxial electromagnetic accelerator having an annular gap geometry tailored to prevent formation of the blow-by instability. The injected plasma is generated by numerous (currently 32) radially oriented capillary discharges arranged uniformly around the circumference of the angled annular injection region of the accelerator. Magnetohydrodynamic modeling identified electrode profiles that can achieve the desired plasma jet parameters. The experimental hardware is described along with initial experimental results in which approximately 200 μg has been accelerated to 100 km/s in a half-scale prototype gun. Initial observations of 64 merging injector jets in a planar cylindrical testing array are presented. Density and velocity are presently limited by available peak current and injection sources. Steps to increase both the drive current and the injected plasma mass are described for next generation experiments.

Glans Penis Augmentation Using Hyaluronic Acid Gel as an Injectable Filler

Science.gov (United States)

Kwak, Tae Il; Kim, Je Jong

2015-01-01

Glans penis augmentation (GPA) has received little attention from experts despite the existence of a subset of patients who may be dissatisfied with a small glans or poor tumescence of the glans during erection. Recently, GPA using an injectable filler or implantation of a graft or filler has been developed. Despite a demanding injection technique and inevitable uneven undulation of the glandular surface, GPA using injectable hyaluronic acid (HA) gel is a novel and useful therapy and an effective and safe procedure for soft tissue enhancement. For long-term presence of implants, timed supplementation can be used similar to that for fascial plasty. In complications such as mucosal necrosis of the glans penis, most cases occur from the use of non-HA gel or an unpurified form and misunderstanding of the management protocol for immediate side effects. Currently, GPA using injectable HA gel is not recommended in the International Society for Sexual Medicine guideline due to possible sensory loss. In a 5-year long-term follow-up of GPA by subcutaneous injection of HA gel, the residual volume of implants decreased by 15% of the maximal glandular circumference, but was still effective for alleviating the hypersensitivity of the glans penis in premature ejaculation patients. For efficacy in premature ejaculation, selection of appropriate candidates is the most important factor for success. GPA does not harm erectile function and is less invasive and irreversible compared to dorsal neurectomy. To refine the procedure, more interest and well-designed studies are required for the establishment of the procedure. PMID:26331121

Glans Penis Augmentation Using Hyaluronic Acid Gel as an Injectable Filler.

Science.gov (United States)

Moon, Du Geon; Kwak, Tae Il; Kim, Je Jong

2015-08-01

Glans penis augmentation (GPA) has received little attention from experts despite the existence of a subset of patients who may be dissatisfied with a small glans or poor tumescence of the glans during erection. Recently, GPA using an injectable filler or implantation of a graft or filler has been developed. Despite a demanding injection technique and inevitable uneven undulation of the glandular surface, GPA using injectable hyaluronic acid (HA) gel is a novel and useful therapy and an effective and safe procedure for soft tissue enhancement. For long-term presence of implants, timed supplementation can be used similar to that for fascial plasty. In complications such as mucosal necrosis of the glans penis, most cases occur from the use of non-HA gel or an unpurified form and misunderstanding of the management protocol for immediate side effects. Currently, GPA using injectable HA gel is not recommended in the International Society for Sexual Medicine guideline due to possible sensory loss. In a 5-year long-term follow-up of GPA by subcutaneous injection of HA gel, the residual volume of implants decreased by 15% of the maximal glandular circumference, but was still effective for alleviating the hypersensitivity of the glans penis in premature ejaculation patients. For efficacy in premature ejaculation, selection of appropriate candidates is the most important factor for success. GPA does not harm erectile function and is less invasive and irreversible compared to dorsal neurectomy. To refine the procedure, more interest and well-designed studies are required for the establishment of the procedure.

Prevalence and correlates of neck injection among people who inject drugs in Tijuana, Mexico.

Science.gov (United States)

Rafful, Claudia; Wagner, Karla D; Werb, Dan; González-Zúñiga, Patricia E; Verdugo, Silvia; Rangel, Gudelia; Strathdee, Steffanie A

2015-11-01

Injecting drugs in the neck has been related to adverse health conditions such as jugular vein thrombosis, deep neck infections, aneurysm, haematomas, airway obstruction, vocal cord paralysis and wound botulism, among others. We identified prevalence and correlates of neck injection among people who inject drugs (PWID) in Tijuana, Mexico. Beginning in 2011, PWID aged ≥18 years who injected drugs within the last month were recruited into a prospective cohort. At baseline and semi-annually, PWID completed interviewer-administered surveys soliciting data on drug-injecting practices. Logistic regression was used to identify predictors of injecting in the neck as the most frequent injection site at a single visit. Of 380 PWID, 35.3% injected in the neck at least once in the past 6 months, among whom 71.6% reported it as their most common injection site, the most common injecting site after the arms (47%). Controlling for age, years injecting and injecting frequency, injecting heroin and methamphetamine two or more times per day and having sought injection assistance were associated with injecting in the neck [adjusted odds ratios (AOR): 2.12; 95% confidence intervals (CI): 1.27-3.53 and AOR: 2.65; 95% CI: 1.52-4.53 respectively]. Injecting in the neck was very common among PWID in Tijuana and was associated with polydrug use and seeking injection assistance. Tailoring harm reduction education interventions for individuals who provide injection assistance ('hit doctors') may allow for the dissemination of safe injecting knowledge to reduce injection-related morbidity and mortality. © 2015 Australasian Professional Society on Alcohol and other Drugs.

Small-Volume Injections: Evaluation of Volume Administration Deviation From Intended Injection Volumes.

Science.gov (United States)

Muffly, Matthew K; Chen, Michael I; Claure, Rebecca E; Drover, David R; Efron, Bradley; Fitch, William L; Hammer, Gregory B

2017-10-01

In the perioperative period, anesthesiologists and postanesthesia care unit (PACU) nurses routinely prepare and administer small-volume IV injections, yet the accuracy of delivered medication volumes in this setting has not been described. In this ex vivo study, we sought to characterize the degree to which small-volume injections (≤0.5 mL) deviated from the intended injection volumes among a group of pediatric anesthesiologists and pediatric postanesthesia care unit (PACU) nurses. We hypothesized that as the intended injection volumes decreased, the deviation from those intended injection volumes would increase. Ten attending pediatric anesthesiologists and 10 pediatric PACU nurses each performed a series of 10 injections into a simulated patient IV setup. Practitioners used separate 1-mL tuberculin syringes with removable 18-gauge needles (Becton-Dickinson & Company, Franklin Lakes, NJ) to aspirate 5 different volumes (0.025, 0.05, 0.1, 0.25, and 0.5 mL) of 0.25 mM Lucifer Yellow (LY) fluorescent dye constituted in saline (Sigma Aldrich, St. Louis, MO) from a rubber-stoppered vial. Each participant then injected the specified volume of LY fluorescent dye via a 3-way stopcock into IV tubing with free-flowing 0.9% sodium chloride (10 mL/min). The injected volume of LY fluorescent dye and 0.9% sodium chloride then drained into a collection vial for laboratory analysis. Microplate fluorescence wavelength detection (Infinite M1000; Tecan, Mannedorf, Switzerland) was used to measure the fluorescence of the collected fluid. Administered injection volumes were calculated based on the fluorescence of the collected fluid using a calibration curve of known LY volumes and associated fluorescence.To determine whether deviation of the administered volumes from the intended injection volumes increased at lower injection volumes, we compared the proportional injection volume error (loge [administered volume/intended volume]) for each of the 5 injection volumes using a linear

Erythropoietin receptor in human skeletal muscle and the effects of acute and long-term injections with recombinant human erythropoietin on the skeletal muscle

DEFF Research Database (Denmark)

Lundby, Carsten; Hellsten, Ylva; Jensen, Mie B. F.

2008-01-01

The presence and potential physiological role of the erythropoietin receptor (Epo-R) were examined in human skeletal muscle. In this study we demonstrate that Epo-R is present in the endothelium, smooth muscle cells, and in fractions of the sarcolemma of skeletal muscle fibers. To study...... the potential effects of Epo in human skeletal muscle, two separate studies were conducted: one to study the acute effects of a single Epo injection on skeletal muscle gene expression and plasma hormones and another to study the effects of long-term (14 wk) Epo treatment on skeletal muscle structure. Subjects...... was studied in subjects (n = 8) who received long-term Epo administration, and muscle biopsies were obtained before and after. Epo treatment did not alter mean fiber area (0.84 +/- 0.2 vs. 0.72 +/- 0.3 mm(2)), capillaries per fiber (4.3 +/- 0.5 vs. 4.4 +/- 1.3), or number of proliferating endothelial cells...

Tennis elbow: role of local steroid injection.

Science.gov (United States)

Ahmed, Gulzar Saeed; Ali, Muhammad; Trago, Imtiaz Ahmed

2012-01-01

Tennis elbow is a condition, characterised by pain and tenderness over the lateral epicondyle of the humerus, and pain on resisted dorsiflexion of the wrist, middle finger, or both. The aim of this randomised controlled trial was to investigate the short term efficacy of local steroid injection compared with oral and topical NSAIDs. Sixty patients (45 male and 15 female) were included in the study. The mean age was 42 years for men and 40 years for women. They were placed in group A and B (30 cases each). Group A received local steroid injection (triamcinolone 20 mg mixed with lignocaine 2% 1 cc) and topical NSAID cream application (diclofenac diethylammonium) twice a day, tab. diclofenac sodium 50 mg twice a day for 3 weeks. Group B received tab diclofenac 50 mg twice a day and, topical NSAID cream application twice a day for 3 weeks. Assessment of patients was made 3 times; first at the start of the study, 2nd time after 6 weeks, and 3rd time after 12 weeks. A blinded assessor rated the elbow complaints of the patients at resisted dorsiflexion of wrist using VAS (0 = no severity, 1-3 mild, 4-6 moderate, 7-9 sever, 10 = maximum severity). At six weeks, 22 (73.33%) patient in group A had no pain as compared to 7 (23.33%) patients in group B who were pain free (p < 0.0001, chi2 = 38.75). At 12 weeks 27 (90%) patients in group A were pain free compared to group B in which 7 (23.33%) patients were pain free (p < 0.0001, chi2 = 27.56). In patients with tennis elbow, the use of local steroid injection in combination with topical and oral NSAIDs is superior to the use of combination of topical and oral NSAIDs. Better results with combination therapy using local steroid injection may be limited to the short term.

Local corticosteroid versus autologous blood injections in lateral epicondylitis: meta-analysis of randomized controlled trials.

Science.gov (United States)

Sirico, Felice; Ricca, Flavia; DI Meglio, Franca; Nurzynska, Daria; Castaldo, Clotilde; Spera, Rocco; Montagnani, Stefania

2017-06-01

Lateral epicondylitis is a common painful elbow disorder. Several approaches to treatment have been proposed, with a local injection of corticosteroids being the most frequently used. Recent insights into the pathophysiology encouraged the introduction of autologous blood injections as an alternative treatment method. The aim of this meta-analysis is to summarize quantitatively the evidence regarding the efficacy of corticosteroids and autologous blood injections for treatment of pain in lateral epicondylitis. Studies were considered eligible based on the following inclusion criteria: adult human, diagnosis of lateral epicondylitis, randomized controlled trials comparing corticosteroids versus autologous blood injections, pain assessment. Exclusion criteria were previous surgery for lateral epicondylitis or for other elbow disorders, concurrent treatment with drugs or physiotherapy, diagnosis of musculoskeletal systemic disorder. A systematic search of literature was performed according to the PRISMA statement. Effect size of each included study was calculated and analyzed in a random-effects model. Four studies, enrolling total of 218 patients (139 females and 79 males), were included in quantitative analysis. At 2 weeks, there was a trend towards a reduction of VAS score in the corticosteroid group (WMD=2.12 [95% CI: 4.38 to 0.14], P=0.07). No significant differences were recorded in the medium-term (4-12 weeks; WMD=0.85 [95% CI: -0.44 to 2.15], P=0.19) and long-term (24 weeks; WMD=0.63 [95% CI: -2.40 to 3.66], P=0.68) follow-up. Few high-quality trials compare the efficacy of corticosteroid and autologous blood injections in the control of pain related to lateral epicondylitis. Available data indicate that corticosteroids tend to reduce VAS score in short-term follow-up, although these data are not statistically significant. No differences were recorded in the medium and long term. Contrary to popular opinion among medical professionals, and despite

Colistimethate Injection

Science.gov (United States)

... injection is in a class of medications called antibiotics. It works by killing bacteria.Antibiotics such as colistimethate injection will not work for colds, flu, or other viral infections. Using ...

INTRAARTICULAR INJECTION OF HYALURONIC ACID AFTER ARTHROSCOPIC LAVAGE OF THE KNEE: LONG-TERM RESULTS

Directory of Open Access Journals (Sweden)

Lidia Vladimirovna Luchikhina

2013-01-01

Full Text Available Objective: to evaluate the efficiency of arthroscopic lavage in combination with subsequent injection of hyaluronic acid into the joint cavity at shortand long-term follow-ups. Subjects and methods. Eighty-two patients with knee osteoarthrosis (OA were examined in accordance with the American College of Rheumatology criteria. Group 1 consisted of 40 patients only after arthroscopic lavage; Group 2 comprised 42 patients who were administered hyaluronic acid after arthroscopic lavage. Clinical evaluation encompassed pain while walking, resting, and moving (by a visual analogue scale, limited ability in covering 100 m (by a 5-point scale, general clinical evaluation (by a 5-point ordinal scale, the presence or absence of pain after 100-m walking, as well as resting pain (its presence or absence. Results. The treatment effect evaluated using different indicators was comparably positive in both groups within 3 months. Following 3 months of therapy, its effect remained stable and even better in Group 2. The latter showed a particularly noticeable superiority a year later. Thus, there were excellent and good results in 88 and 47.5% in Groups 2 and 1, respectively. The clinical symptoms of the disease were absent in 58% in Group 2 and in only 15% in Group 1. Moreover, Group 1 showed worsening and 20% of the patients had no effect. This trend was also seen while evaluating the therapeutic effectiveness in different periods. Thus, after therapy, no substantial difference was found in both groups, but 3 months later this difference was as many as 0.8 scores and a year later Group 2 had many points in its favor (1.2 scores. Conclusion. Arthroscopic lavage followed by the administration of hyaluronic acid makes it possible to prevent the negative effect of a washing liquid on the metabolism and structure of the articular cartilage and to achieve a long-term effect against the major clinical symptoms (joint pain and function affecting the quality of life. The

Glenohumeral Joint Injections

Science.gov (United States)

Gross, Christopher; Dhawan, Aman; Harwood, Daniel; Gochanour, Eric; Romeo, Anthony

2013-01-01

Context: Intra-articular injections into the glenohumeral joint are commonly performed by musculoskeletal providers, including orthopaedic surgeons, family medicine physicians, rheumatologists, and physician assistants. Despite their frequent use, there is little guidance for injectable treatments to the glenohumeral joint for conditions such as osteoarthritis, adhesive capsulitis, and rheumatoid arthritis. Evidence Acquisition: We performed a comprehensive review of the available literature on glenohumeral injections to help clarify the current evidence-based practice and identify deficits in our understanding. We searched MEDLINE (1948 to December 2011 [week 1]) and EMBASE (1980 to 2011 [week 49]) using various permutations of intra-articular injections AND (corticosteroid OR hyaluronic acid) and (adhesive capsulitis OR arthritis). Results: We identified 1 and 7 studies that investigated intra-articular corticosteroid injections for the treatment of osteoarthritis and adhesive capsulitis, respectively. Two and 3 studies investigated the use of hyaluronic acid in osteoarthritis and adhesive capsulitis, respectively. One study compared corticosteroids and hyaluronic acid injections in the treatment of osteoarthritis, and another discussed adhesive capsulitis. Conclusion: Based on existing studies and their level of evidence, there is only expert opinion to guide corticosteroid injection for osteoarthritis as well as hyaluronic acid injection for osteoarthritis and adhesive capsulitis. PMID:24427384

DEPLETED HYDROCARBON RESERVOIRS AND CO2 INJECTION WELLS –CO2 LEAKAGE ASSESSMENT

Directory of Open Access Journals (Sweden)

Nediljka Gaurina-Međimurec

2017-03-01

Full Text Available Migration risk assessment of the injected CO2 is one of the fi rst and indispensable steps in determining locations for the implementation of projects for carbon dioxide permanent disposal in depleted hydrocarbon reservoirs. Within the phase of potential storage characterization and assessment, it is necessary to conduct a quantitative risk assessment, based on dynamic reservoir models that predict the behaviour of the injected CO2, which requires good knowledge of the reservoir conditions. A preliminary risk assessment proposed in this paper can be used to identify risks of CO2 leakage from the injection zone and through wells by quantifying hazard probability (likelihood and severity, in order to establish a risk-mitigation plan and to engage prevention programs. Here, the proposed risk assessment for the injection well is based on a quantitative risk matrix. The proposed assessment for the injection zone is based on methodology used to determine a reservoir probability in exploration and development of oil and gas (Probability of Success, abbr. POS, and modifi ed by taking into account hazards that may lead to CO2 leakage through the cap rock in the atmosphere or groundwater. Such an assessment can eliminate locations that do not meet the basic criteria in regard to short-term and long-term safety and the integrity of the site

Injection Tests

CERN Document Server

Kain, V

2009-01-01

The success of the start-up of the LHC on 10th of September was in part due to the preparation without beam and injection tests in 2008. The injection tests allowed debugging and improvement in appropriate portions to allow safe, efficient and state-of-the-art commissioning later on. The usefulness of such an approach for a successful start-up becomes obvious when looking at the problems we encountered before and during the injection tests and could solve during this period. The outline of the preparation and highlights of the different injection tests will be presented and the excellent performance of many tools discussed. A list of shortcomings will follow, leading to some planning for the preparation of the run in 2009.

Microwave coagulation therapy and drug injection to treat splenic injury.

Science.gov (United States)

Zhang, Guoming; Sun, Yuanyuan; Yu, Jie; Dong, Lei; Mu, Nannan; Liu, Xiaohong; Liu, Lanfen; Zhang, Yan; Wang, Xiaofei; Liang, Ping

2014-01-01

The present study compares the efficacy of 915- and 2450-MHz contrast-enhanced ultrasound (CEUS)-guided percutaneous microwave coagulation with that of CEUS-guided thrombin injection for the treatment of trauma-induced spleen hemorrhage. In a canine splenic artery hemorrhage model with two levels of arterial diameter (A, microwaves and drug injection. Therapy efficacy was measured by comparing bleeding rate, hemostatic time, bleeding index, bleeding volume, and pathology. The most efficient technique was CEUS-guided 915-MHz percutaneous microwave coagulation therapy in terms of action time and total blood loss. The success rate of the 915-MHz microwave group was higher than that of the 2450-MHz microwave and the drug injection groups (except A level, P microwave group than those in the 2450-MHz microwave and drug injection groups (P microwave group, but pathologic changes of light injury could be seen in the other groups. The present study provides evidence that microwave coagulation therapy is more efficient than thrombin injection for the treatment of splenic hemorrhage. Furthermore, treatment with 915-MHz microwaves stops bleeding more rapidly and generates a wider cauterization zone than does treatment with 2450-MHz microwaves. Copyright © 2014 Elsevier Inc. All rights reserved.

300 Area Uranium Stabilization Through Polyphosphate Injection: Final Report

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Vermeul, Vincent R.; Bjornstad, Bruce N.; Fritz, Brad G.; Fruchter, Jonathan S.; Mackley, Rob D.; Newcomer, Darrell R.; Mendoza, Donaldo P.; Rockhold, Mark L.; Wellman, Dawn M.; Williams, Mark D.

2009-06-30

The objective of the treatability test was to evaluate the efficacy of using polyphosphate injections to treat uranium-contaminated groundwater in situ. A test site consisting of an injection well and 15 monitoring wells was installed in the 300 Area near the process trenches that had previously received uranium-bearing effluents. This report summarizes the work on the polyphosphate injection project, including bench-scale laboratory studies, a field injection test, and the subsequent analysis and interpretation of the results. Previous laboratory tests have demonstrated that when a soluble form of polyphosphate is injected into uranium-bearing saturated porous media, immobilization of uranium occurs due to formation of an insoluble uranyl phosphate, autunite [Ca(UO2)2(PO4)2•nH2O]. These tests were conducted at conditions expected for the aquifer and used Hanford soils and groundwater containing very low concentrations of uranium (10-6 M). Because autunite sequesters uranium in the oxidized form U(VI) rather than forcing reduction to U(IV), the possibility of re-oxidation and subsequent re-mobilization is negated. Extensive testing demonstrated the very low solubility and slow dissolution kinetics of autunite. In addition to autunite, excess phosphorous may result in apatite mineral formation, which provides a long-term source of treatment capacity. Phosphate arrival response data indicate that, under site conditions, the polyphosphate amendment could be effectively distributed over a relatively large lateral extent, with wells located at a radial distance of 23 m (75 ft) reaching from between 40% and 60% of the injection concentration. Given these phosphate transport characteristics, direct treatment of uranium through the formation of uranyl-phosphate mineral phases (i.e., autunite) could likely be effectively implemented at full field scale. However, formation of calcium-phosphate mineral phases using the selected three-phase approach was problematic. Although

Can repeat injection provide clinical benefit in patients with cervical disc herniation and stenosis when the first epidural injection results only in partial response?

Science.gov (United States)

Lee, Jung Hwan; Lee, Sang-Ho

2016-07-01

Epidural steroid injection (ESI) is known to be an effective treatment for neck or radicular pain due to herniated intervertebral disc (HIVD) and spinal stenosis (SS). Although repeat ESI has generally been indicated to provide more pain relief in partial responders after single ESI, there has been little evidence supporting the usefulness of this procedure. The purpose of this study, therefore, was to determine whether repeat ESI at a prescribed interval of 2 to 3 weeks after the first injection would provide greater clinical benefit in patients with partial pain reduction than intermittent ESI performed only when pain was aggravated. One hundred eighty-four patients who underwent transforaminal ESI (TFESI) for treatment of axial neck and radicular arm pain due to HIVD or SS and could be followed up for 1 year were enrolled. We divided the patients into 2 groups. Group A (N = 108) comprised partial responders (numeric rating scale (NRS) ≥ 3 after the first injection) who underwent repeat injection at a prescribed interval of 2 to 3 weeks after the first injection. Group B (N = 76) comprised partial responders who did not receive repeat injection at the prescribed interval, but received intermittent injections only for aggravation of pain. Various clinical data were assessed, including total number of injections during 1 year, NRS duration of Group A, or after first injection in Group B (time to reinjection). Groups A and B were compared in terms of total population, HIVD, and SS. In the whole population, HIVD subgroup, and SS subgroup, patients in Group A required significantly fewer injections to obtain satisfactory pain relief during the 1-year follow-up period. Group A showed a significantly longer time to reinjection and longer NRS Group B did. Repeat TFESI conducted at 2- to 3-week intervals after the first injection in partial responders contributed to greater clinical benefit compared with intermittent TFESI performed only upon pain

Nonlinear dynamic behaviors of an optically injected vertical-cavity surface-emitting laser

International Nuclear Information System (INIS)

Li Xiaofeng; Pan Wei; Luo Bin; Ma Dong; Wang Yong; Li Nuohan

2006-01-01

Nonlinear dynamics of a vertical-cavity surface-emitting laser (VCSEL) with external optical injection are studied numerically. We consider a master-slave configuration where the dynamic characteristics of the slave are affected by the optical injection from the master, and we also establish the corresponding Simulink model. The period-doubling route as well as the period-halving route is observed, where the regular, double-periodic, and chaotic pulsings are found. By adjusting the injection strength properly, the laser can be controlled to work at a given state. The effects of frequency detuning on the nonlinear behaviors are also investigated in terms of the bifurcation diagrams of photon density with the frequency detuning. For weak injection case, the nonlinear dynamics shown by the laser are quite different when the value of frequency detuning varies contrarily (positive and negative direction). If the optical injection is strong enough, the slave can be locked by the master even though the frequency detuning is relatively large

Meropenem Injection

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... injection is in a class of medications called antibiotics. It works by killing bacteria that cause infection.Antibiotics such as meropenem injection will not work for colds, flu, or other viral infections. Taking ...

Chloramphenicol Injection

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... injection is in a class of medications called antibiotics. It works by stopping the growth of bacteria..Antibiotics such as chloramphenicol injection will not work for colds, flu, or other viral infections. Taking ...

The effectiveness of injections of hyaluronic acid or corticosteroid in patients with subacromial impingement: a three-arm randomised controlled trial.

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Penning, L I F; de Bie, R A; Walenkamp, G H I M

2012-09-01

A total of 159 patients (84 women and 75 men, mean age of 53 (20 to 87)) with subacromial impingement were randomised to treatment with subacromial injections using lidocaine with one of hyaluronic acid (51 patients), corticosteroid (53 patients) or placebo (55 patients). Patients were followed up for 26 weeks. The primary outcome was pain on a visual analogue score (VAS), and secondary outcomes included the Constant Murley score, shoulder pain score, functional mobility score, shoulder disability questionnaire and pain-specific disability score. The different outcome measures showed similar results. After three, six and 12 weeks corticosteroid injections were superior to hyaluronic acid injections and only at six weeks significantly better than placebo injections. The mean short-term reduction in pain on the VAS score at 12 weeks was 7% (SD 2.7; 97.5% confidence interval (CI) 0.207 to 1.55; p = 0.084) in the hyaluronic acid group, 28% (SD 2.8; 97.5% CI 1.86 to 3.65; p hyaluronic acid group, 72% (38 of 53) of those in the corticosteroid group and 69% (38 of 55) of those in the placebo group. We were not able to show a convincing benefit from hyaluronic acid injections compared with corticosteroid or placebo injections. Corticosteroid injections produced a significant reduction in pain in the short term (three to 12 weeks), but in the long term the placebo injection produced the best results.

Worldwide Injection Technique Questionnaire Study: Population Parameters and Injection Practices.

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Frid, Anders H; Hirsch, Laurence J; Menchior, Astrid R; Morel, Didier R; Strauss, Kenneth W

2016-09-01

From February 1, 2014, through June 30, 2015, 13,289 insulin-injecting patients from 423 centers in 42 countries took part in one of the largest surveys ever performed in diabetes. The goal was to assess patient characteristics, as well as historical and practical aspects of their injection technique. Results show that 4- and 8-mm needle lengths are each used by nearly 30% of patients and 5- and 6-mm needles each by approximately 20%. Higher consumption of insulin (as measured by total daily dose) is associated with having lipohypertrophy (LH), injecting into LH, leakage from the injection site, and failing to reconstitute cloudy insulin. Glycated hemoglobin values are, on average, 0.5% higher in patients with LH and are significantly higher with incorrect rotation of sites and with needle reuse. Glycated hemoglobin values are lower in patients who distribute their injections over larger injection areas and whose sites are inspected routinely. The frequencies of unexpected hypoglycemia and glucose variability are significantly higher in those with LH, those injecting into LH, those who incorrectly rotate sites, and those who reuse needles. Needles associated with diabetes treatment are the most commonly used medical sharps in the world. However, correct disposal of sharps after use is critically suboptimal. Many used sharps end up in public trash and constitute a major accidental needlestick risk. Use of these data should stimulate renewed interest in and commitment to optimizing injection practices in patients with diabetes. Copyright © 2016 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

Granisetron Injection

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Granisetron immediate-release injection is used to prevent nausea and vomiting caused by cancer chemotherapy and to ... nausea and vomiting that may occur after surgery. Granisetron extended-release (long-acting) injection is used with ...

Edaravone Injection

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Edaravone injection is used to treat amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease; a condition in which ... die, causing the muscles to shrink and weaken). Edaravone injection is in a class of medications called ...

Defibrotide Injection

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Defibrotide injection is used to treat adults and children with hepatic veno-occlusive disease (VOD; blocked blood ... the body and then returned to the body). Defibrotide injection is in a class of medications called ...

Nalbuphine Injection

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... injection is in a class of medications called opioid agonist-antagonists. It works by changing the way ... suddenly stop using nalbuphine injection, you may experience withdrawal symptoms including restlessness; teary eyes; runny nose; yawning; ...

Amyloidoses as seen by the Rheumatologist

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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...

Unilateral versus bilateral thyroarytenoid Botulinum toxin injections in adductor spasmodic dysphonia: a prospective study

Science.gov (United States)

Upile, Tahwinder; Elmiyeh, Behrad; Jerjes, Waseem; Prasad, Vyas; Kafas, Panagiotis; Abiola, Jesuloba; Youl, Bryan; Epstein, Ruth; Hopper, Colin; Sudhoff, Holger; Rubin, John

2009-01-01

Objectives In this preliminary prospective study, we compared unilateral and bilateral thyroarytenoid muscle injections of Botulinum toxin (Dysport) in 31 patients with adductor spasmodic dysphonia, who had undergone more than 5 consecutive Dysport injections (either unilateral or bilateral) and had completed 5 concomitant self-rated efficacy and complication scores questionnaires related to the previous injections. We also developed a Neurophysiological Scoring (NPS) system which has utility in the treatment administration. Method and materials Data were gathered prospectively on voice improvement (self-rated 6 point scale), length of response and duration of complications (breathiness, cough, dysphagia and total voice loss). Injections were performed under electromyography (EMG) guidance. NPS scale was used to describe the EMG response. Dose and unilateral/bilateral injections were determined by clinical judgment based on previous response. Time intervals between injections were patient driven. Results Low dose unilateral Dysport injection was associated with no significant difference in the patient's outcome in terms of duration of action, voice score (VS) and complication rate when compared to bilateral injections. Unilateral injections were not associated with any post treatment total voice loss unlike the bilateral injections. Conclusion Unilateral low dose Dysport injections are recommended in the treatment of adductor spasmodic dysphonia. PMID:19852852

Unilateral versus bilateral thyroarytenoid Botulinum toxin injections in adductor spasmodic dysphonia: a prospective study

Directory of Open Access Journals (Sweden)

Abiola Jesuloba

2009-10-01

Full Text Available Abstract Objectives In this preliminary prospective study, we compared unilateral and bilateral thyroarytenoid muscle injections of Botulinum toxin (Dysport in 31 patients with adductor spasmodic dysphonia, who had undergone more than 5 consecutive Dysport injections (either unilateral or bilateral and had completed 5 concomitant self-rated efficacy and complication scores questionnaires related to the previous injections. We also developed a Neurophysiological Scoring (NPS system which has utility in the treatment administration. Method and materials Data were gathered prospectively on voice improvement (self-rated 6 point scale, length of response and duration of complications (breathiness, cough, dysphagia and total voice loss. Injections were performed under electromyography (EMG guidance. NPS scale was used to describe the EMG response. Dose and unilateral/bilateral injections were determined by clinical judgment based on previous response. Time intervals between injections were patient driven. Results Low dose unilateral Dysport injection was associated with no significant difference in the patient's outcome in terms of duration of action, voice score (VS and complication rate when compared to bilateral injections. Unilateral injections were not associated with any post treatment total voice loss unlike the bilateral injections. Conclusion Unilateral low dose Dysport injections are recommended in the treatment of adductor spasmodic dysphonia.

Piezoelectric Injection Systems

Science.gov (United States)

Mock, R.; Lubitz, K.

The origin of direct injection can be doubtlessly attributed to Rudolf Diesel who used air assisted injection for fuel atomisation in his first self-ignition engine. Although it became apparent already at that time that direct injection leads to reduced specific fuel consumption compared to other methods of fuel injection, it was not used in passenger cars for the moment because of its disadvantageous noise generation as the requirements with regard to comfort were seen as more important than a reduced specific consumption.

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.

Usefulness of intra-articular botulinum toxin injections. A systematic review.

Science.gov (United States)

Khenioui, Hichem; Houvenagel, Eric; Catanzariti, Jean François; Guyot, Marc Alexandre; Agnani, Olivier; Donze, Cécile

2016-03-01

Botulinum toxin is a proven and widely used treatment for numerous conditions characterized by excessive muscular contractions. Recent studies have assessed the analgesic effect of botulinum toxin in joint pain and started to unravel its mechanisms. We searched the international literature via the Medline database using the term "intraarticular botulinum toxin injection" combined with any of the following terms: "knee", "ankle", "shoulder", "osteoarthritis", "adhesive capsulitis of the shoulder". Of 16 selected articles about intraarticular botulinum toxin injections, 7 were randomized controlled trials done in patients with osteoarthritis, adhesive capsulitis of the shoulder, or chronic pain after joint replacement surgery. Proof of anti-nociceptive effects was obtained in some of these indications and the safety and tolerance profile was satisfactory. The studies are heterogeneous. The comparator was usually a glucocorticoid or a placebo; a single study used hyaluronic acid. Pain intensity was the primary outcome measure. The number of randomized trials and sample sizes are too small to provide a satisfactory level of scientific evidence or statistical power. Unanswered issues include the effective dosage and the optimal dilution and injection modalities of botulinum toxin. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Memory enhancement by Tamoxifen on amyloidosis mouse model.

Science.gov (United States)

Pandey, Deepika; Banerjee, Sugato; Basu, Mahua; Mishra, Nibha

2016-03-01

Tamoxifen (TMX) is a selective estrogen receptor modulator (SERM) used in the treatment of breast cancer. Earlier studies show its neuroprotection via regulating apoptosis, microglial functions, and synaptic plasticity. TMX also showed memory enhancement in ovariectomized mice, and protection from amyloid induced damage in hippocampal cell line. These reports encouraged us to explore the role of TMX in relevance to Alzheimer's disease (AD). We report here, the effect of TMX treatment a) on memory, and b) levels of neurotransmitters (acetylcholine (ACh) and dopamine (DA)) in breeding-retired-female mice injected with beta amyloid1-42 (Aβ1-42). Mice were treated with TMX (10mg/kg, i.p.) for 15 days. In Morris water maze test, the TMX treated mice escape latency decreased during training trials. They also spent longer time in the platform quadrant on probe trial, compared to controls. In Passive avoidance test, TMX treated mice avoided stepping on the shock chamber. This suggests that TMX protects memory from Aβ induced toxicity. In frontal cortex, ACh was moderately increased, with TMX treatment. In striatum, dopamine was significantly increased, 3,4-dihydroxyphenylacetic acid (DOPAC) level and DOPAC/DA ratio was decreased post TMX treatment. Therefore, TMX enhances spatial and contextual memory by reducing dopamine metabolism and increasing ACh level in Aβ1-42 injected-breeding-retired-female mice. Copyright © 2015. Published by Elsevier Inc.

Thermorespiratory Response of West African Dwarf Does to Progestagen Injection

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Ogundana, FA.

2005-01-01

Full Text Available The physiological responses in terms of respiration rate (RR and rectal temperature (RT of 30 West African Dwarf (WAD does were monitored after a single im injection of different doses of progestagens meant for synchronising oestrus. The treatments involved im injection of 1 2 ml of sterile water (Control; 2 25 mg Medroxy Progesterone Acetate, MPA; 3 50 mg MPA; 4 50 mg Progesterone and 5 100 mg progesterone. The RR and RT of each animal were measured at 9:00, 13:00 and 17:00 h for 21 days after each injection. In all the treatment groups the RT did not change significantly (P> 0.05 during the weeks following treatment. On the contrary the RR was significantly higher (P< 0.05 in the week after injection than in the pre-injection period. Both the RR and the RT were higher at 13:00 h and 17:00 h than at 9:00 h. The RR was more influenced by the animal body size than the RT, while the ambient temperature and humidity affected both the animal's RT and RR. These results indicate that the thermorespiratory function of WAD does under their native humid environment is not negatively affected by injecting ≤ 100 mg progesterone or ≤ 50 mg MPA to synchronize oestrus.

A contoured gap coaxial plasma gun with injected plasma armature

Energy Technology Data Exchange (ETDEWEB)

Witherspoon, F. Douglas; Case, Andrew; Messer, Sarah J.; Bomgardner, Richard II; Phillips, Michael W.; Brockington, Samuel; Elton, Raymond [HyperV Technologies Corp., Chantilly, Virginia 20151 (United States)

2009-08-15

A new coaxial plasma gun is described. The long term objective is to accelerate 100-200 {mu}g of plasma with density above 10{sup 17} cm{sup -3} to greater than 200 km/s with a Mach number above 10. Such high velocity dense plasma jets have a number of potential fusion applications, including plasma refueling, magnetized target fusion, injection of angular momentum into centrifugally confined mirrors, high energy density plasmas, and others. The approach uses symmetric injection of high density plasma into a coaxial electromagnetic accelerator having an annular gap geometry tailored to prevent formation of the blow-by instability. The injected plasma is generated by numerous (currently 32) radially oriented capillary discharges arranged uniformly around the circumference of the angled annular injection region of the accelerator. Magnetohydrodynamic modeling identified electrode profiles that can achieve the desired plasma jet parameters. The experimental hardware is described along with initial experimental results in which approximately 200 {mu}g has been accelerated to 100 km/s in a half-scale prototype gun. Initial observations of 64 merging injector jets in a planar cylindrical testing array are presented. Density and velocity are presently limited by available peak current and injection sources. Steps to increase both the drive current and the injected plasma mass are described for next generation experiments.

Potential benefits of saturation cycle with two-phase refrigerant injection

International Nuclear Information System (INIS)

Lee, Hoseong; Hwang, Yunho; Radermacher, Reinhard; Chun, Ho-Hwan

2013-01-01

In this paper, a saturation cycle is proposed to enhance a vapor compression cycle performance by reducing thermodynamic losses associated with single phase gas compression and isenthalpic expansion. In order to approach the saturation cycle, a two-phase refrigerant injection technique is applied to the multi-stage cycle. This multi-stage cycle with different options is modeled, and its performance is evaluated under ASHRAE standard operating conditions for air conditioning systems. Moreover, the two-phase refrigerant injection cycle is compared with the typical vapor injection cycle which is utilizing the internal heat exchanger or the flash tank. Low GWP refrigerants are applied to this two-phase refrigerant injection cycle. In terms of the COP and its improvement, R123 has a higher potential than any other refrigerants in the multi-stage cycle. Lastly, practical ideas realizing the saturation cycle are discussed such as multi-stage phase separator, phase separator with helical structure inside, and injection location of the compressor. -- Highlights: • A saturation cycle is proposed to enhance the vapor compression cycle performance. • Two-phase refrigerant injection technique is applied to the multi-stage cycle. • Modeling results of the proposed cycle show the significant performance improvement. • Low GWP refrigerants are applied and R123 shows the highest performance. • New parameters, α and ε, are used to show the potential of the saturation cycle

Increased Use of Injectable Contraception in Sub-Saharan Africa ...

African Journals Online (AJOL)