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Sample records for laryngeal amyloidosis presenting

  1. Laryngeal Amyloidosis Mimicking Glottic Cancer: A Case Report

    International Nuclear Information System (INIS)

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

    2010-01-01

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

  2. 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.

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

    NARCIS (Netherlands)

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

    2016-01-01

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

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

    NARCIS (Netherlands)

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

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

  5. Laryngeal amyloidosis with laryngocele: MRI and CT

    International Nuclear Information System (INIS)

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

    1998-01-01

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

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

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    Terry Golombick

    2015-01-01

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

  7. 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.

  8. 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

  9. 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.

  10. 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.

  11. 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.

  12. 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.

  13. 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

  14. Dysphonia – the single symptom of rifampicin resistant laryngeal tuberculosis

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    Paulauskienė Iveta

    2016-01-01

    Full Text Available Tuberculosis is still the most frequent granulomatous laryngeal disease. Absence of pathognomonic symptoms and change in clinical pattern frequently leads to misdiagnosis and delayed treatment. Hoarseness is the commonest symptom of laryngeal tuberculosis and constitutional symptoms are usually rare. However dysphonia can be caused by many other more common conditions. Hoarseness can be a symptom of organic (nodules and polyps of vocal folds, tumors, vocal fold paresis or functional (functional dysphonia, laryngeal conversion disorder, paradoxical vocal folds motion conditions. Rarely systemic diseases as amyloidosis, sarcoidosis, Wegener’s granulomatosis or tuberculosis can cause vocal dysfunction too. That is why laryngeal tuberculosis is often forgotten in case of persistent hoarseness. In this article, we present a case of a young previously healthy woman, complaining of persistent hoarseness with no other leading symptoms. Though endoscopic image suggested a malignancy, histology showed granulomatous lesion. Detailed examination revealed laryngeal and pulmonary tuberculosis resistant to rifampicin. Conclusion: Dysphonia can be the only one symptom of laryngeal tuberculosis. The disease should be taken into consideration when a patient complains of persistent hoarseness in order to avoid delays in treatment and spread of infection.

  15. Laryngeal sarcoidosis: a case report presenting transglottic involvement

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    Broek, E.M.J.M. van den; Heijnen, B.J.; Verbist, B.M.; Sjögren, E.V.

    2013-01-01

    Isolated laryngeal sarcoidosis is a very rare disease. In most cases, it will present as a supraglottic pale edematous swelling. In our case, the patient presented with hoarseness and dyspnea during exertion. Laryngeal examination did show not only supraglottic edema but also prominent subglottic

  16. Laryngeal sarcoidosis: a case report presenting transglottic involvement.

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    van den Broek, Emke M J M; Heijnen, Bas J; Verbist, Berit M; Sjögren, Elisabeth V

    2013-09-01

    Isolated laryngeal sarcoidosis is a very rare disease. In most cases, it will present as a supraglottic pale edematous swelling. In our case, the patient presented with hoarseness and dyspnea during exertion. Laryngeal examination did show not only supraglottic edema but also prominent subglottic swelling and edematous true vocal folds. Histology showed noncaseating granulomas. After excluding other causes and localizations, the patient was diagnosed with laryngeal sarcoidosis and treated with systemic corticosteroid with good result. We describe our case of isolated transglottic sarcoidosis and discuss the disease, its presentation, diagnosis, and therapeutic options. Copyright © 2013 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  17. 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.

  18. 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.

  19. Laryngeal carcinoma presenting as polymyositis: A paraneoplastic syndrome

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    Ritesh Sahu

    2016-01-01

    Full Text Available Laryngeal carcinoma is rarely associated with paraneoplastic syndrome. Inflammatory myopathy presenting as paraneoplastic event is commonly associated with carcinomas of ovary, lung, pancreas, stomach, colorectal, and non-Hodgkin′s lymphoma. We report a case of elderly male, who presented with proximal muscle weakness and found to be associated with laryngeal carcinoma. Diagnosis of polymyositis (PM was confirmed based on clinical features, laboratory test, and muscle biopsy. Exclusion of other commonly associated malignancies was done. This patient improved gradually after 6 months of immunosuppressive therapy and management of underlying cancer.

  20. 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

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

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    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.

  2. Laryngeal sarcoidosis: presentation and management in the pediatric population.

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    Strychowsky, Julie E; Vargas, Sara O; Cohen, Ezra; Vielman, Rene; Son, Mary Beth; Rahbar, Reza

    2015-09-01

    Sarcoidosis is a disease characterized by systemic non-necrotizing granulomas of unknown etiology. Laryngeal sarcoidosis is extremely uncommon, especially among pediatric patients. The clinical presentation and management of this entity in the pediatric population are poorly understood. A comprehensive search in PubMed was conducted to identify all cases in the published literature. We also present a case of isolated pediatric laryngeal sarcoidosis and outline the multidisciplinary approach to evaluation and management. A previously healthy 13-year-old female presented with a five-month history of mild dysphonia, dyspnea on exertion, and diffuse supraglottic edema. Biopsy showed non-necrotizing granulomas. Treatment with methotrexate led to marked improvement. The literature search identified seven previously published cases of pediatric laryngeal sarcoidosis, four in which disease was isolated to the larynx. All patients presented with a symptomatic and diffusely edematous supraglottis. Diagnoses were based on supraglottic biopsies showing non-necrotizing granulomas; all other possible etiopathologies were excluded. Three patients responded to corticosteroid therapy alone, one patient to tumor necrosis factor (TNF) inhibitor and methotrexate, and the remainder to a combination of corticosteroid therapy and surgical debulking. Laryngeal sarcoidosis in the pediatric population is challenging to diagnose and manage. When epithelioid granulomas are encountered histologically, other causes of granulomatous inflammation must be ruled out before a diagnosis of sarcoidosis can be made. Corticosteroid therapy alone may be ineffective. Medical therapy with methotrexate alone or in combination with TNF inhibitors versus surgical debulking alone or as part of multimodality treatment should be considered. A multidisciplinary approach with involvement of an otolaryngologist, pathologist, and rheumatologist is suggested. Copyright © 2015 Elsevier Ireland Ltd. All rights

  3. Rheumatic polymyalgia like presentation of multiple myeloma and amyloidosis

    International Nuclear Information System (INIS)

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

    2005-01-01

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

  4. 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

  5. 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.

  6. Laryngeal Schwannoma: A Case Presentation and Review of the Mayo Clinic Experience.

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    Romak, Jonathan J; Neel, H Bryan; Ekbom, Dale C

    2017-01-01

    The aim of this study was to clarify the nature of laryngeal schwannomas through review of the experience of a single institution during a 104-year period. This is a retrospective case series. The Mayo Clinic, Rochester, Minnesota clinical and surgical pathology database was reviewed for the years 1985-2011. Four cases of laryngeal schwannoma were identified. These cases were pooled with a previously published series of laryngeal schwannomas treated at our institution between 1907 and 1986. The characteristics of all 11 cases were studied, and relevant literature was reviewed. A total of 11 cases of schwannoma of the larynx were identified. The mean age at presentation was 48 years (range 12-73 years). The most common presenting symptoms were dysphonia and dysphagia. The most frequently involved primary site was the false vocal fold (six patients), followed by the aryepiglottic fold (three), epiglottis (two), subglottis (two), ventricle (one), true vocal fold (one) and postcricoid region (one). The mean maximal tumor diameter was 2.5 cm. In all but one case, surgical excision was curative with no recurrence during recorded follow up ranging from 1 to 17 years. Laryngeal schwannomas, although rare, should be considered in the differential diagnosis of laryngeal tumors. They occur most frequently in the false vocal fold and present most commonly with dysphonia and/or dysphagia. Surgical excision is the treatment of choice. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  7. 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.

  8. Amyloidosis

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

  9. 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 neuro...

  10. 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.

  11. Mediastinal and bronchovascular amyloidosis

    International Nuclear Information System (INIS)

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

    1990-01-01

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

  12. [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.

  13. 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.

  14. 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.

  15. Laryngeal fracture due to blunt trauma presenting with pneumothorax and pneumomediastinum.

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    Narcı, Adnan; Embleton, Didem Baskın; Ayçiçek, Abdullah; Yücedağ, Fatih; Cetinkurşun, Salih

    2011-01-01

    Injuries due to traffic accidents are frequent in childhood, and they have high mortality and morbidity. Laryngeal injury due to a traffic accident is a rare pathology and might be missed if not suspected. Here we present a laryngeal fracture in a child after a blunt chest trauma during a traffic accident that presented with pneumomediastinum and pneumothorax. A 14-year-old girl was referred for pneumomediastinum. Her physical examination was normal except subcutaneous emphysema, edema and tenderness in the cervical area, hoarseness, facial and extremity abrasions and ecchymoses. Chest tomography revealed pneumothorax and pneumomediastinum, and cranial tomography revealed maxillofacial fractures. Upper airway damage was suspected, flexible endoscopy revealed right vocal cord paralysis and cervical tomography revealed thyroid cartilage fracture. The fracture was repaired and tracheotomy was performed. She was discharged on postoperative day 6. Facial fractures were repaired in another center. Tracheotomy was removed on postoperative day 20. Her hoarseness, although decreased, still persists. Pneumomediastinum is a rare result of a laryngeal fracture and if not suspected, the fracture can easily be missed. It should be kept in mind after blunt cervical trauma with pneumomediastinum and/or pneumothorax. Direct endoscopy and cervical tomography may be necessary for the differential diagnosis. Copyright © 2011 S. Karger AG, Basel.

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

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    Hanks Douglas K

    2011-01-01

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

  17. 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.

  18. 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

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

    Science.gov (United States)

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

    2008-02-01

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

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

    Science.gov (United States)

    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.

  1. 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.

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

    Science.gov (United States)

    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.

  3. AA amyloidosis complicating the hereditary periodic fever syndromes.

    Science.gov (United States)

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

    2013-04-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Mari Kantanen

    2014-07-01

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

  6. Amyloidosis and Kidney Disease

    Science.gov (United States)

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

  7. Laryngeal debridement: an alternative treatment for a laryngopyocele presenting with severe airway obstruction.

    Science.gov (United States)

    Fraser, L; Pittore, B; Frampton, S; Brennan, P; Puxeddu, R

    2011-04-01

    The laryngocele is an abnormal saccular dilatation of the ventricle of Morgagni, which maintains its communication with the laryngeal vestibule. Three types of laryngoceles have been described: internal, external, and combined or mixed in relation to the position of the sac with respect to the thyrohyoid membrane. If the laryngocele becomes obstructed and infected it leads to the so-called laryngopyocele which, although a rare disease (8% of laryngoceles), can become an emergency causing severe airway obstruction needing urgent management, even tracheostomy. An alternative method is presented of emergency management of an internal laryngopyocele causing severe airway obstruction using a laryngeal microdebrider and avoiding tracheostomy.

  8. 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.

  9. Diagnostic studies in amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Bouke Pier Cornelis

    2007-01-01

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

  10. Treatment of amyloidosis.

    Science.gov (United States)

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

    1995-08-01

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

  11. Primary laryngeal tuberculosis mimicking laryngeal carcinoma: CT scan features

    International Nuclear Information System (INIS)

    El Kettani, N Ech-Cherif; El Hassani, MR; Chakir, N; Jiddane, M

    2010-01-01

    Laryngeal tuberculosis is a rare disease. It is almost always associated with pulmonary tuberculosis. It occurs generally in adults without BCG vaccination or in cases of the acquired immune deficiency syndrome. On laryngoscopy and imaging, it often simulates laryngeal carcinoma, and confirmation is always histological. We report the case of a 36-year-old man who presented to our hospital with dysphonia and dysphagia. Laryngoscopy revealed a lesion of the left vocal cord and the ventricular strip. CT scan found focal, regular thickening of the left vocal cord, associated with irregular thickening of the posterior laryngeal wall. A biopsy confirmed the diagnosis of tuberculosis

  12. Laryngeal Leishmaniasis

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    Moraes, Bruno Teixeira de

    2012-01-01

    Full Text Available Introduction: Leishmaniasis is classified into three clinical presentations: visceral, coetaneous and mucocutaneous. The latter is usually secondary to hematogenous spread after months or years of skin infection and can manifest as infiltrative lesions, ulcerated or vegetating in nose, pharynx, larynx and mouth, associated or not with ganglionics infarction. Laryngeal involvement is part of the differential diagnosis of lesions in this topography as nonspecific chronic laryngitis, granulomatosis and even tumors of the upper aerodigestive tract presenting atypical evolution. Sometimes it is difficult for the correct diagnosis of Leishmaniasis, with description of cases in the literature were conducted improperly. Objective: The objective of this study is to report a case of laryngeal Leishmaniasis addressing the difficulty of diagnosis, complications and treatment applied. Case Report: A patient with pain throat, dysphagia, odynophagia, dysphonia and weight loss, with no improvement with symptomatic medication. At telelaringoscopy, infiltrative lesion showed nodular supraglottis. He underwent a tracheotomy for airway obstruction and biopsy with immunohistochemical study for a definitive diagnosis of laryngeal Leishmaniasis. The patient was referred to the infectious diseases that initiated treatment with N-methylglucamine antimoniate with satisfactory response to therapy. Final Comments: Faced with a clinical suspicion of granulomatous diseases, it is essential to follow protocol laboratory evaluation associated with histological injury, to get a precise definition etiological without prolonging the time of diagnosis. Medical treatment for mucosal Leishmaniasis, recommended by the World Health Organization, was adequate in the case of laryngeal disorders, with complete resolution of symptoms.

  13. A laryngeal presentation of Churg-Strauss syndrome in childhood

    International Nuclear Information System (INIS)

    AlAmmar, Ahmed Y; Yasin, Subhan S; AlMuhsen, Saleh Zaid; AlSaadi Muslim M; AlSohaibanic, Mohammad O

    2009-01-01

    A 10- year-old female, known to have bronchial asthma, presented with an unusual laryngeal lesion, eventually diagnosed as Churg-Strauss syndrome (CSS). She was referred to our hospital with history of recurrent stridor. On endoscopyhe, the larynx showed signs similar to recurrent respiratory papillomatosis (RRP). CSS is a systemic disorder and is now defined as one of the ANCA (antineutrophil cytoplasmic antibodies) - associated vasculitides. CSS is a systemic disease that may involve unusual sites like the laryynx. Such an unusual presenatation of CSS should be kept in mind, especially in patients with history of asthma. (author)

  14. A laryngeal presentation of Churg-Strauss syndrome in childhood

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    AlAmmar, Ahmed Y; Yasin, Subhan S; AlMuhsen, Saleh Zaid [Dept. of Otolaryngology, Head and Neck Surgery, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia); M, AlSaadi Muslim [Dept. of Pediatrics, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia); AlSohaibanic, Mohammad O [Dept. of Pathology, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia)

    2009-07-01

    A 10- year-old female, known to have bronchial asthma, presented with an unusual laryngeal lesion, eventually diagnosed as Churg-Strauss syndrome (CSS). She was referred to our hospital with history of recurrent stridor. On endoscopyhe, the larynx showed signs similar to recurrent respiratory papillomatosis (RRP). CSS is a systemic disorder and is now defined as one of the ANCA (antineutrophil cytoplasmic antibodies) - associated vasculitides. CSS is a systemic disease that may involve unusual sites like the laryynx. Such an unusual presenatation of CSS should be kept in mind, especially in patients with history of asthma. (author)

  15. 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.

  16. 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.

  17. 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.

  18. Macular Amyloidosis and Epstein-Barr Virus

    Directory of Open Access Journals (Sweden)

    Yalda Nahidi

    2016-01-01

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

  19. 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.

  20. 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.

  1. 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.

  2. 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.

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

    Science.gov (United States)

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

    2015-10-21

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

  4. Cardiac amyloidosis detection with pyrophosphate-99mTc scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

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

    2008-07-01

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

  5. 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

  6. CT findings of laryngeal tuberculosis : comparison with laryngeal carcinoma

    International Nuclear Information System (INIS)

    Kim, Man Deuk; Kim, Dong Ik; Lee, Byung Hee; Sung, Ki Joon; Jung, Tae Sub; Cho, Jae Min; Yune, Heun Yune; Kim, Sun Yong

    1996-01-01

    To determine the value of CT(Computerized Tomography) in the diagnosis of laryngeal tuberculosis and to assess to what extent its characteristic findings different from those of laryngeal carcinoma. CT scans of twelve patients with laryngeal tuberculosis were reviewed and compared with those of fifteen patients with laryngeal cancer, retrospectively. Clinical symptoms, laryngoscopic examinations and the presence of pulmonary tuberculosis chest radiographs were also reviewed. In laryngeal tuberculosis, bilateral symmetric or asymmetric involvement was noted in nine(75%) patients, while unilateral involvement was seen in three(25%). This was significantly different from laryngeal cancer in which unilateral involvement was noted in twelve patients(80%). Diffuse thickening of the free margin of the epiglottis was a characteristic and frequent finding in tuberculosis(n=6, 50%). No deep submucosal infiltration of preepiglottic and paralaryngeal fat spaces is seen in tuberculosis in spite of large areas of involvement of laryngeal mucosa, while twelve patients(80%) with laryngeal cancer showed thickened deep infiltration which resulted in a submucosal mass. CT was useful in the diagnosis of laryngeal tuberculosis and its CT findings were characterized by bilateral involvement, thickening of the free margin of the epiglottis and good preservation of preepiglottic and paralaryngeal fat spaces in spite of large areas of involvement

  7. 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

  8. Laryngeal chondrosarcoma of the arytenoid cartilage presenting as bilateral vocal fold immobility: a case report and literature review.

    Science.gov (United States)

    Hu, Rong; Xu, Wen; Liu, Honggang; Chen, Xuejun

    2014-01-01

    To describe an atypical case of laryngeal chondrosarcoma of arytenoid cartilage presenting as bilateral vocal fold immobility and to avoid potential missed diagnosis. Our case study included a detail history, physical and radiological examination, laryngeal electromyography (LEMG), and surgical treatment and pathology analysis. We compared it with the previously discussed cases of chondrosarcoma of arytenoid cartilage in the literature. Chondrosarcomas of the arytenoid cartilage is rare, and to date only approximately 10 cases have been reported. We reported a case of a 51-year-old man with 1 month of persistent dyspnea presenting with bilateral vocal fold immobility without neoplasms in larynx. The LEMG showed no obvious abnormality. The cervical-enhanced computed tomography (CT) found no significant signs of a mass except for localized high-density areas in arytenoid cartilage. Right arytenoidectomy and biopsy were performed under general anesthesia with CO2 laser with the pathological diagnosis of chondroma. A total laryngectomy was performed 2 years later, and low-grade chondrosarcoma was the final diagnosis. Laryngeal chondrosarcomas of the arytenoid cartilage are rare. It is easily neglected, especially in those cases presenting with idiopathic vocal fold immobility without any obvious signs of neoplasms. The LEMG and laryngeal CT are necessary. Sometimes, a biopsy of the arytenoid cartilage is essential. Copyright © 2014 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    Mihajlo Gjeorgjievski

    2015-01-01

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

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

    Science.gov (United States)

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

    2018-03-01

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

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

    Directory of Open Access Journals (Sweden)

    Jinze Qian

    2010-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Jingjing Li

    2017-07-01

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

  13. 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

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

    Science.gov (United States)

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

    2009-01-01

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

  15. Primary (AL) amyloidosis with gastrointestinal involvement

    DEFF Research Database (Denmark)

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

    2009-01-01

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

  16. Imaging of laryngeal trauma

    Energy Technology Data Exchange (ETDEWEB)

    Becker, Minerva, E-mail: Minerva.Becker@hcuge.ch [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14 (Switzerland); Leuchter, Igor, E-mail: Igor.Leuchter@hcuge.ch [Department of Otorhinolaryngology and Cervico-facial Surgery, University Hospital of Geneva, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14 (Switzerland); Platon, Alexandra, E-mail: Alexandra.Platon@hcuge.ch [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14 (Switzerland); Becker, Christoph D., E-mail: Christoph.Becker@hcuge.ch [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14 (Switzerland); Dulguerov, Pavel, E-mail: Pavel.Dulguerov@hcuge.ch [Department of Otorhinolaryngology and Cervico-facial Surgery, University Hospital of Geneva, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14 (Switzerland); Varoquaux, Arthur, E-mail: Arthur.Varoquaux@hcuge.ch [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14 (Switzerland)

    2014-01-15

    External laryngeal trauma is a rare but potentially life-threatening situation in the acutely injured patient. Trauma mechanism and magnitude, maximum focus of the applied force, and patient related factors, such as age and ossification of the laryngeal cartilages influence the spectrum of observed injuries. Their correct diagnosis and prompt management are paramount in order to avoid patient death or long-term impairment of breathing, swallowing and speaking. The current review provides a comprehensive approach to the radiologic interpretation of imaging studies performed in patients with suspected laryngeal injury. It describes the key anatomic structures that are relevant in laryngeal trauma and discusses the clinical role of multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the acute emergency situation. The added value of two-dimensional multiplanar reconstructions (2D MPR), three-dimensional volume rendering (3D VR) and virtual endoscopy (VE) for the non-invasive evaluation of laryngeal injuries and for treatment planning is discussed. The clinical presentation, biomechanics of injury, diagnostic pitfalls and pearls, common and uncommon findings are reviewed with emphasis of fracture patterns, involvement of laryngeal joints, intra- and extralaryngeal soft tissue injuries, and complications seen in the acute emergency situation. The radiologic appearance of common and less common long-term sequelae, as well as treatment options are equally addressed.

  17. Imaging of laryngeal trauma

    International Nuclear Information System (INIS)

    Becker, Minerva; Leuchter, Igor; Platon, Alexandra; Becker, Christoph D.; Dulguerov, Pavel; Varoquaux, Arthur

    2014-01-01

    External laryngeal trauma is a rare but potentially life-threatening situation in the acutely injured patient. Trauma mechanism and magnitude, maximum focus of the applied force, and patient related factors, such as age and ossification of the laryngeal cartilages influence the spectrum of observed injuries. Their correct diagnosis and prompt management are paramount in order to avoid patient death or long-term impairment of breathing, swallowing and speaking. The current review provides a comprehensive approach to the radiologic interpretation of imaging studies performed in patients with suspected laryngeal injury. It describes the key anatomic structures that are relevant in laryngeal trauma and discusses the clinical role of multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the acute emergency situation. The added value of two-dimensional multiplanar reconstructions (2D MPR), three-dimensional volume rendering (3D VR) and virtual endoscopy (VE) for the non-invasive evaluation of laryngeal injuries and for treatment planning is discussed. The clinical presentation, biomechanics of injury, diagnostic pitfalls and pearls, common and uncommon findings are reviewed with emphasis of fracture patterns, involvement of laryngeal joints, intra- and extralaryngeal soft tissue injuries, and complications seen in the acute emergency situation. The radiologic appearance of common and less common long-term sequelae, as well as treatment options are equally addressed

  18. 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.

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

    Science.gov (United States)

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

    2003-01-01

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

  20. AL Amyloidosis Complicated by Persistent Oral Bleeding

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2017-09-01

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

  4. Laryngeal granuloma: a complication of prolonged endotracheal intubation.

    OpenAIRE

    Keiser, G. J.; Bozentka, N. E.; Gold, B. D.

    1991-01-01

    Laryngeal granuloma is an uncommon complication arising from irritation of the laryngeal structures. We present a case where bilateral laryngeal granulomas became clinically evident 3 mo after orthognathic surgery. The patient, a 19-yr-old female, developed acute dyspnea after experiencing gradual voice loss. Excision of the lesions under endotracheal general anesthesia led to an uneventful outcome. The causes, predisposing factors, diagnostic features, and treatment of laryngeal granuloma ar...

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

    Science.gov (United States)

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

    2018-01-01

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

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

    Science.gov (United States)

    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.

  7. Amyloidosis of the thyroid gland: ultrasonographic aspect

    International Nuclear Information System (INIS)

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

    1998-01-01

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

  8. Lichen amyloidosis in an unusual location.

    Science.gov (United States)

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

    2007-06-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

  10. Management of Advanced Laryngeal Cancer

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    Patrick Sheahan

    2014-04-01

    Full Text Available Squamous cell carcinoma of the larynx continues to be the commonest head and neck cancer in many Western countries. The larynx plays a key role for many essential functions, including breathing, voice production, airway protection, and swallowing. The goals of laryngeal cancer treatment are thus to provide best possible oncologic control, while optimizing functional outcomes. In recent decades, the treatment paradigm for advanced laryngeal cancer has shifted from one of primary surgery (total laryngectomy as gold standard, toward non-surgical organ-preserving treatment using radiotherapy or chemoradiotherapy. However, concerns have emerged regarding functional outcomes after chemoradiotherapy, as well as possible decreased overall survival in patients with laryngeal cancer. The purpose of the present review is to review surgical and non-surgical options for treatment of advanced laryngeal cancer, as well as the evidence supporting each of these.

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

    Science.gov (United States)

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

    2017-11-01

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

  12. Amyloidosis in alkaptonuria.

    Science.gov (United States)

    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.

  13. 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

  14. Primary localised cutaneous amyloidosis - a systematic review

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  15. Characteristics of AA amyloidosis patients in San Francisco.

    Science.gov (United States)

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

    2016-04-01

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

  16. Diagnostic and therapeutic approach of systemic amyloidosis

    NARCIS (Netherlands)

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

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

  17. Clinical approach of patients with systemic amyloidosis

    NARCIS (Netherlands)

    Hazenberg, Bouke

    2011-01-01

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

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

    Science.gov (United States)

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

    2006-06-01

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

  19. 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

  20. 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

  1. Congenital laryngeal anomalies,

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    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  2. Hereditary Transthyretin Amyloidosis in Eight Chinese Families

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    Ling-Chao Meng

    2015-01-01

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

  3. Isolated laryngeal myasthenia gravis for 26 years.

    Science.gov (United States)

    Renard, Dimitri; Hedayat, Amir; Gagnard, Corinne

    2015-02-01

    Laryngeal myasthenia gravis is a relatively rare variant of myasthenia gravis. A vast portion of patients with initial laryngeal myasthenia gravis develop involvement of ocular and/or extra-ocular muscles during the years after symptom onset although a minority of laryngeal myasthenia gravis patients continues to have isolated laryngeal muscle involvement for several years. We present a 58-year-old woman with recurrent episodic isolated dysphonia (associated with diffuse bilateral vocal cord paresis on laryngoscopy) since the age of 32. Dysphonia became permanent since 6 months. A diagnosis of laryngeal myasthenia gravis was made based on abnormal single-fiber electromyography and spectacular response to pyridostigmine treatment. Repetitive nerve stimulation was normal and anti-acetylcholine receptor and anti-muscle specific tyrosine kinase antibodies were absent. This case shows that laryngeal myasthenia gravis can be isolated during 26 years of follow-up. We propose that even when myasthenia gravis seems unlikely as underlying mechanism of isolated dysphonia (because of lack of antibodies, normal repetitive nerve stimulation, and absence of extra-laryngeal involvement after years of follow-up), single-fiber electromyography should be performed and myasthenia gravis treatment should be tried. Copyright © 2014 Elsevier B.V. All rights reserved.

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

    Science.gov (United States)

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

    2018-05-18

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

  5. 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

  6. Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis.

    Science.gov (United States)

    Pajor, Anna Maria; Kwiatkowska, Sylwia; Kroczyńska-Bednarek, Jadwiga; Piotrowski, Wojciech J

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

  7. Clinical study of T1 and T2 laryngeal cancers. Key points for laryngeal preservation

    International Nuclear Information System (INIS)

    Nasu, Takashi; Koike, Shuji; Inamura, Hiroo; Aoyagi, Masaru; Namura, Tadashi

    2004-01-01

    Between 1989 and 2003, we treated 129 patients with T1 and T2 laryngeal cancers. The purpose of this study was to estimate the management of T1 and T2 laryngeal cancers, referring to the relationship with the T classification, subtype, treatment, prognosis and laryngeal preservation. The treatment plan for T1 and T2 laryngeal cancers is fundamentally radiotherapy. To raise the laryngeal preservation rate, concurrent chemoradiotherapy by FAR therapy, carboplatin (CBDCA), docetaxel (DOC) and laser treatment was performed for the T2 cases. The 5-year survival rates of the T1 and T2 cases were 94.7% and 94.8%, respectively. The 5-year laryngeal preservation rates of the T1 and T2 cases were 97.1% and 72.3%, respectively. The 5-year survival rates of the glottic cancer and supraglottic cancer cases were 96.7% and 87.0% and the 5-year laryngeal preservation rates of these cases were 97.1% and 57.2%, respectively. Particularly in T2 supraglottic laryngeal cancer, the laryngeal preservation rate is not improved even with concurrent chemoradiotherapy by CBDCA and FAR therapy. To improve the laryngeal preservation rate in T2 supraglottic laryngeal cancer, it is necessary to consider concurrent chemoradiotherapy by DOC or hyperfractionation. (author)

  8. 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

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

    Science.gov (United States)

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

    2014-12-01

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

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

    Science.gov (United States)

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

    2013-04-01

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

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

    Science.gov (United States)

    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

  12. 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.)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Irina Mikhailovna Marusenko

    2009-01-01

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

  17. 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.

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

    Directory of Open Access Journals (Sweden)

    Giuseppe Caparrotti

    2016-03-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  20. 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.

  1. Laryngeal Chondrosarcoma: A rare cause of critical upper airway obstruction.

    LENUS (Irish Health Repository)

    Tuite, K

    2018-01-01

    Laryngeal cancers are rare, encompassing around one percent of all cancers. Suspicion should be raised if a patient presents with classical signs and symptoms; i.e. dysphonia, inspiratory stridor, dysphagia, odynophagia, neck mass, or persistent cough. Laryngeal chondrosarcoma is a rare form of laryngeal cancer, the diagnosis of which can be difficult. The case in question describes an unusual presentation of one such case, and its subsequent investigation, management and outcome.

  2. Laryngeal neurinoma. Differential diagnosis of submucosal laryngeal tumors

    International Nuclear Information System (INIS)

    Higuera, A.; Palomo, V.; Munoz, R.; Sanchez, F.

    2002-01-01

    Laryngeal neurinoma is a rare benign tumor that appears as a submucosal mass, generally in the supraglottic region. We report the case of a patient with dysphonia of long evolution caused by a neurinoma. We discuss the radiological findings of the tumor and the value of computed tomography (CT) in the diagnosis of this and other submucosal laryngeal lesions. (Author) 16 refs

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

    Science.gov (United States)

    Manur, Rashmi; Lamzabi, Ihab

    2018-06-01

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

  4. Septal graft in laryngeal reconstruction

    International Nuclear Information System (INIS)

    Bahannan, Abdulrahman; Slavicek, A.; Taudy, M.; Chovanec, M.

    2006-01-01

    A 62-year-old woman presented with symptoms of dyspnea. Ultrasonography and computed tomography examinations revealed mass extending from the cricoid cartilage to the left lobe of thyroid gland and thyroid cartilage. Cytology revealed possibility of cartilaginous origin, which was proven to be chondrosarcoma (Grade 1) from the biopsy specimen obtained during panendosopy. She underwent one stage radical resection and immediate reconstruction of laryngeal skeleton defect by mucocartilaginous graft from the nasal septum. Her postoperative course was optimal with preservation of the laryngeal functions. Twenty-eight months postoperatively, she had to undergo total laryngectomy as a salvage procedure for the advanced local recurrence. We report on the relatively easy technique for functional reconstruction of the large laryngeal defect with the employment cartilage graft from the nasal septum. (author)

  5. [Laryngeal adduction reflex].

    Science.gov (United States)

    Ptok, M; Bonenberger, S; Miller, S; Kühn, D; Jungheim, M

    2014-07-01

    Laryngeal Adductor Reflex Background: A rapid closure of the vocal folds is necessary, whenever foreign materials or food particles penetrate into the larynx. Otherwise a passage of these particles into the trachea or the lower respiratory tract would be imminent. An aspiration could mechanically block the respiratory tract and cause severe dyspnoea or cause aspiration pneumonia. For this systematic review a selective literature research in PubMed and Scopus using the keywords "laryngeal adductor reflex" and "vocal fold closure" has been carried out. Apart from the oesophago-glottal and pharyngo-glottal closure reflexes, the laryngeal adductor reflex (LAR) has been investigated in particular. The LAR qualifies as a reflectory laryngeal adductor mechanism and involves early, presumably di- or oligosynaptic ipsilateral LAR1 as well as late polysynaptic ipsi- and contralateral LAR2 components. In clinical routine diagnostic settings of dysphagia, LAR is only assessed qualitatively and usually triggered by air pulses or tactile stimulation. Dysphagiologists often find that not only the laryngeal sensibility in general is impaired, but especially the protective laryngeal adduction mechanism, which results in a higher risk of aspiration. Thus, it appears mandatory to test the LAR not only qualitatively but also quantitatively. Unfortunately a valid and reliable method that can be employed in clinical practice has not yet been put forward. © Georg Thieme Verlag KG Stuttgart · New York.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-10-15

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

  7. EXPERIENCE OF USING ALLOGENIC BIOIMPLANTS IN LARYNGEAL RESECTION

    Directory of Open Access Journals (Sweden)

    E. N. Novozhilova

    2017-01-01

    Full Text Available Introduction. Currently, a great importance is being attached to improvement of the surgical component of combination treatment of locally advanced laryngeal cancer. New technological capabilities (transoral microsurgery of the larynx and robotic surgery offer great opportunities for early cancer stages. However, in some cases capabilities of endoscopic laser intervention are limited. Therefore, open laryngeal resection is still relevant as it serves as the only type of radical organ preservation treatment for stages Т2–Т3. But major laryngeal resection is associated with a problem of tissue defect closure.The article describes data on the use of biocompatible materials, their advantages and disadvantages. The study objective is to present experience of using a Russian allogenic bioimplant for plastic reconstruction of the opening of the larynx after laryngeal resection.Materials and methods. The authors present their experience of using a Russian bioimplant produced in collaboration with the Samara Tissue Bank of the Research Institute of Experimental Medicine and Biotechnology of the Samara State Medical University. The material was tested in anterolateral laryngeal resection with simultaneous reconstruction in 5 patients with stages Т2–Т3 laryngeal cancer and in a patient with chondrosarcoma.Conclusion. The Russian biocompatible implant served as a reliable, simple, cheap, and effective variant of plastic material for reconstruction of the larynx.

  8. Amyloidosis cutis dyschromica: A rare reticulate pigmentary dermatosis

    Directory of Open Access Journals (Sweden)

    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.

  9. Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease.

    Science.gov (United States)

    Priyamvada, P S; Morkhandikar, S; Srinivas, B H; Parameswaran, S

    2015-01-01

    Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig) light chains. In heavy chain amyloidosis (AH), deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL), the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda) AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  11. Autologous Stem Cell Transplant for AL Amyloidosis

    Directory of Open Access Journals (Sweden)

    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.

  12. Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease

    Directory of Open Access Journals (Sweden)

    P S Priyamvada

    2015-01-01

    Full Text Available Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig light chains. In heavy chain amyloidosis (AH, deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL, the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

  13. Recent Insights into the Pathogenesis of Type AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    J. C. H. van der Hilst

    2011-01-01

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

  14. Laryngeal Sensation Before and After Clearing Behaviors

    Science.gov (United States)

    Bonilha, Heather Shaw; Gerlach, Terri Treman; Sutton, Lori Ellen; Dawson, Amy Elizabeth; Nietert, Paul J

    2013-01-01

    Purpose People frequently present to voice clinics with complaints of irritating laryngeal sensations. Clinicians attempt to reduce the irritating sensations and their common sequela, coughing and throat clearing, by advocating for techniques that remove the irritation with less harm to the vocal fold tissue. Despite the prevalence of patients with these complaints, it is not known if the less harmful techniques recommended by clinicians are effective at clearing irritating laryngeal sensations or that irritating laryngeal sensations are, in fact, more frequent in people with voice disorders than people without voice disorders. Method Assessments of participant reported laryngeal sensation, pre- and post- clearing task, were obtained from 22 people with and 24 people without a voice disorder. Six clearing tasks were used to preliminarily evaluate the differing effects of tasks believed to be deleterious and ameliorative. Results People with and without voice disorders reported pre-clear laryngeal sensation at a similar rate. Post-clear sensation was less likely to be completely or partially removed in people with voice disorders than in the non-voice disordered group. Hard throat clear and swallow with water were the most effective techniques at removing laryngeal sensation. Conclusions The findings provide initial evidence for some of the clinical practices common to treating patients with voice disorders and chronic clearing such as advocating for swallowing a sip of water as a replacement behavior instead of coughing or throat clearing. However, the findings raise questions about other practices such as associating irritating laryngeal sensation with a voice disorder. PMID:22717491

  15. [A case of laryngeal papilloma with sudden dyspnea].

    Science.gov (United States)

    Pabiszczak, Maciej; Wierzbicka, Małgorzata; Borucki, Lukasz; Iwanik, Katarzyna

    2005-01-01

    Laryngeal papilloma is one of the most common non-malignant tumors of the larynx. In adult they are included to pre-cancerous diseases. In morphological examination, it is a solid tumor. Often it is possible to cure them during one surgical procedure. The disease is mostly localized on the anterior commissurae region, vocal fold, ventricules, and on the laryngeal surface of the epiglottis. If the malignant transformation is suspected, a large excision with margin has to be performed, completed by a histological evaluation. A case of a huge laryngeal papilloma with dyspnea is presented.

  16. Childhood Laryngeal Tumors Treatment (PDQ®)—Health Professional Version

    Science.gov (United States)

    Laryngeal tumors in children are rare and can be benign (papillomatosis) or malignant. Rhabdomyosarcoma is the most common cancer of the larynx in children. Get comprehensive information about childhood laryngeal tumors, including histology, presentation, and treatment in this summary for clinicians.

  17. Epigenetic Dysregulation in Laryngeal Squamous Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Thian-Sze Wong

    2012-01-01

    Full Text Available Laryngeal carcinoma is a common head and neck cancer with poor prognosis. Patients with laryngeal carcinoma usually present late leading to the reduced treatment efficacy and high rate of recurrence. Despite the advance in the use of molecular markers for monitoring human cancers in the past decades, there are still no reliable markers for use to screen laryngeal carcinoma and follow the patients after treatment. Epigenetics emerged as an important field in understanding the biology of the human malignancies. Epigenetic alterations refer to the dysregulation of gene, which do not involve the alterations of the DNA sequence. Major epigenetic changes including methylation imbalance, histone modification, and small RNA dysregulation could play a role in the development of human malignancies. Global epigenetic change is now regarded as a molecular signature of cancer. The characteristics and behavior of a cancer could be predicted based on the specific epigenetic pattern. We here provide a review on the understanding of epigenetic dysregulation in laryngeal carcinoma. Further knowledge on the initiation and progression of laryngeal carcinoma at epigenetic level could promote the translation of the knowledge to clinical use.

  18. Leukocyte derived chemotaxin 2 (ALECT2 amyloidosis

    Directory of Open Access Journals (Sweden)

    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.

  19. [Acute laryngitis and epiglottitis in children].

    Science.gov (United States)

    Mimouni, Olivia; Nicollas, Richard; Roman, Stéphane; Triglia, Jean-Michel

    2007-10-31

    The anatomical characteristic of the pediatric larynx allows physicians to better understand the incidence of symptomatic and severe presentations of acute laryngitis, which are frequent pediatric emergencies. Subglottis laryngitis and epiglottitis must be distinguished from each other. These two diseases are absolutely different: the first one is essentially viral and usually moderate, even though acute respiratory distress can occur. The other (epiglottitis) is bacterial, essentially caused by Haemophilus influenza B (Hi-B), and can be life threatening. The anti Hi-B vaccine leads to a decrease of frequency but does not make them disappear. Moreover, even if a child has a history of the Hi-B vaccine, diagnosis of epiglottitis can not to be ruled out. Lastly, in case of acute laryngeal dyspnea in a child, one must think about a foreign body.

  20. Inducible Laryngeal Obstruction: Excessive Dynamic Airway Collapse vs. Inducible Laryngeal Obstruction

    Science.gov (United States)

    2017-10-20

    REPORT TYPE 10/20/2017 Poster 4. TITLE AND SUBTITLE Inducible Laryngeal Obstrnction: Excessive Dynamic Airway Collapse vs. Inducible Laryngeal...REPORT b.ABSTRACT c. THIS PAGE ABSTRACT OF PAGES 3. DATES COVERED (From - To) 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER

  1. Diagnosis and management with botulinum toxin in 11 cases of laryngeal synkinesis.

    Science.gov (United States)

    Lekue, Asier; García-López, Isabel; Santiago, Susana; Del Palacio, Antonio; Gavilán, Javier

    2015-09-01

    Laryngeal synkinesis is a vocal fold movement disorder produced by a misdirected reinnervation after a recurrent laryngeal nerve injury. Its symptoms differ greatly between patients, requiring diverse therapeutical approaches. We aim to describe our experience in the diagnosis and treatment of different laryngeal synkinesis presentations. 11 patients diagnosed between 2011 and 2014 in a tertiary referral center with laryngeal synkinesis confirmed by laryngeal electromyography were included in our study. All medical records and laryngoscopic and electromyographic data were reviewed retrospectively. Four patients had previous unilateral vocal fold palsy and seven had a bilateral palsy with different degrees of clinical involvement. All of them showed paradoxical movements during inhalation in videofibrolaryngoscopic examination. Laryngeal electromyography confirmed the diagnosis of laryngeal synkinesis. Dyspnea was the main presentation symptom. Three patients with mild symptoms were not treated. Patients with unilateral vocal fold immobility were successfully treated with periodic botulinum toxin injections. Patients with bilateral immobility had a good initial response to botulinum toxin, although in some of them, a posterior cordectomy had to be finally performed. In conclusion, laryngeal synkinesis is a heterogeneous clinic entity that appears in patients with unilateral or bilateral vocal fold paralysis. Videofibrolaryngoscopy and laryngeal electromyography are essential to a correct diagnosis. Botulinum toxin injections are the main treatment for symptomatic cases, even if in bilateral palsy cases more aggressive treatments are often required.

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

    Directory of Open Access Journals (Sweden)

    Mysore Venkataram

    2002-01-01

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

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

    Science.gov (United States)

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

    2018-04-01

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

  4. 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

  5. Laryngeal Dysfunction: Assessment and Management for the Clinician.

    Science.gov (United States)

    Hull, James H; Backer, Vibeke; Gibson, Peter G; Fowler, Stephen J

    2016-11-01

    The larynx is one of the most highly innervated organs in humans and serves a number of vitally important, complex, and highly evolved biological functions. On a day-to-day basis, the larynx functions autonomously, addressing several roles including airway protection, swallowing, and phonation. In some situations the larynx appears to adopt a functional state that could be considered maladaptive or "dysfunctional." This laryngeal dysfunction can underpin and account for a number of respiratory symptoms that otherwise appear incongruous with a clinical disease state and/or contribute to the development of symptoms that appear "refractory" to treatment. These include conditions associated with a heightened tendency for inappropriate laryngeal closure (e.g., inducible laryngeal obstruction), voice disturbance, and chronic cough. Recognition of laryngeal dysfunction is important to deliver targeted treatment and failure to recognize the condition can lead to repeated use of inappropriate treatment. Diagnosis is not straightforward, however, and many patients appear to present with symptoms attributable to laryngeal dysfunction, but in whom the diagnosis has been overlooked in clinical work-up for some time. This review provides an overview of the current state of knowledge in the field of laryngeal dysfunction, with a focus on pragmatic clinical assessment and management.

  6. Treatment of laryngeal radionecrosis with hyperbaric oxygen therapy. A case report

    International Nuclear Information System (INIS)

    Nishida, Akiko T.; Honda, Nobumitsu; Tsujimura, Mika; Adachi, Tsunemichi; Fujiki, Nobuya; Miyata, Kouji; Fukushima, Hideyuki; Kitani, Yoshiharu

    2007-01-01

    Laryngeal necrosis is a rare complication of radiotherapy for early-staged laryngeal or hypopharyngeal cancer. However, when it occurs, there is no definitive treatment and laryngectomy is frequently required. Here we present a case of advanced laryngeal necrosis (grade 4, Chandler classification) treated with hyperbaric oxygen (HBO) therapy. A 60-year-old woman received radiotherapy with 64.8 Gy for early-staged hypopharyngeal cancer in combination with three courses of cisplatin (40 mg/body/week). She developed pharyngeal pain and progressive dyspnea because of laryngeal edema 3 months after the completion of radiotherapy, and received an emergent tracheostomy. In order to differentiate radiation injury from cancer recurrence, biopsies were obtained under direct laryngoscopy and showed intense necrosis without malignancy. Since laryngeal radionecrosis was presumed, the patient received HBO therapy at 2 atmospheres absolute (ATA) with 100% O 2 for 2 hours per session. After 20 HBO treatments, laryngeal edema improved and her clinical symptoms including pharyngeal pain, swallowing pain and dysphagia, were ameliorated significantly. However, decannulation has not been achieved because of bilateral vocal cord fixation. We considered that HBO therapy was effective in the present case and that HBO therapy should be recommended as a therapeutic option whenever laryngeal necrosis occurs and there is a chance to save the larynx. (author)

  7. 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,

  8. 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...

  9. Inferior alveolar nerve injury with laryngeal mask airway: a case report

    Directory of Open Access Journals (Sweden)

    Masud Sarmad

    2011-03-01

    Full Text Available Abstract Introduction The incidence of damage to the individual cranial nerves and their branches associated with laryngeal mask airway use is low; there have been case reports of damage to the lingual nerve, hypoglossal nerve and recurrent laryngeal nerve. To the best of our knowledge we present the first reported case of inferior alveolar nerve injury associated with laryngeal mask airway use. Case presentation A 35-year-old Caucasian man presented to our facility for elective anterior cruciate ligament repair. He had no background history of any significant medical problems. He opted for general anesthesia over a regional technique. He was induced with fentanyl and propofol and a size 4 laryngeal mask airway was inserted without any problems. His head was in a neutral position during the surgery. After surgery in the recovery room, he complained of numbness in his lower lip. He also developed extensive scabbing of the lower lip on the second day after surgery. The numbness and scabbing started improving after a week, with complete recovery after two weeks. Conclusion We report the first case of vascular occlusion and injury to the inferior alveolar nerve, causing scabbing and numbness of the lower lip, resulting from laryngeal mask airway use. This is an original case report mostly of interest for anesthetists who use the laryngeal mask airway in day-to-day practice. Excessive inflation of the laryngeal mask airway cuff could have led to this complication. Despite the low incidence of cranial nerve injury associated with the use of the laryngeal mask airway, vigilant adherence to evidence-based medicine techniques and recommendations from the manufacturer's instructions can prevent such complications.

  10. Evaluation of laryngeal cartilage calcification in computed tomography

    International Nuclear Information System (INIS)

    Laskowska, K.; Serafin, Z.; Lasek, W.; Maciejewski, M.; Wieczor, W.; Wisniewski, S.

    2008-01-01

    Computed tomography (CT) is one of the basic methods used for laryngeal carcinoma diagnostics. Osteosclerotic and osteolytic changes of the cartilages are considered as a common radiologic symptom of laryngeal neoplasms. The aim of this paper was to evaluate the prevalence of both osteosclerotic changes and focal calcification defects, which may be suggestive of osteolysis. Calcification was assessed in the thyroid, the cricoid and the arytenoids cartilages on CT images of the neck. We have retrospectively analyzed neck CT examinations of 50 patients without any laryngeal pathology in anamnesis. The grade and symmetry of calcifications was assessed in the thyroid, the cricoid and the arytenoids cartilages. Calcification of the laryngeal cartilages was present in 83% of the patients. Osteosclerotic lesions of the thyroid cartilage were seen in 70% of the patients (asymmetric in 60% of them), of the cricoid catrilage in 50% (asymmetric in 60%), and of the arytenoid cartilages in 24% (asymmetric in 67%). Focal calcification defects were present in the thyroid cartilage in 56% of the patients (asymmetric in 67% of them), in the cricoid catrilage in 8% (asymmetric in all cases), and in the arytenoid cartilages in 20% (asymmetric in 90%). Osteosclerotic changes and focal calcification defects, which may suggest osteolysis, were found in most of the patients. Therefore, they cannot be used as crucial radiological criteria of neoplastic invasion of laryngeal cartilages. (authors)

  11. Inferior alveolar nerve injury with laryngeal mask airway: a case report.

    LENUS (Irish Health Repository)

    Hanumanthaiah, Deepak

    2011-01-01

    The incidence of damage to the individual cranial nerves and their branches associated with laryngeal mask airway use is low; there have been case reports of damage to the lingual nerve, hypoglossal nerve and recurrent laryngeal nerve. To the best of our knowledge we present the first reported case of inferior alveolar nerve injury associated with laryngeal mask airway use.

  12. 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...

  13. Cuff leak test and laryngeal survey for predicting post-extubation stridor.

    Science.gov (United States)

    Patel, Anit B; Ani, Chizobam; Feeney, Colin

    2015-02-01

    Evidence for the predictive value of the cuff leak test (CLT) for post-extubation stridor (PES) is conflicting. We evaluated the association and accuracy of CLT alone or combined with other laryngeal parameters with PES. Fifty-one mechanically ventilated adult patients in a medical-surgical intensive care unit were tested prior to extubation using; CLT, laryngeal ultrasound and indirect laryngoscopy. Biometric, laryngeal and endotracheal tube (ETT) parameters were recorded. PES incidence was 4%. CLT demonstrated 'no leak' in 20% of patients. Laryngeal oedema was present in 10% of the patients on indirect laryngoscopy, and 71% of the patients had a Grades 1-3 indirect laryngoscopic view. Mean air column width on laryngeal ultrasound was 0.66 ± 0.15 cm (cuff deflated), mean ratio of ETT to laryngeal diameter was 0.48 ± 0.07, and the calculated CLT and laryngeal survey composite was 0.86 ± 1.25 (range 0-5). CLT and the CLT and Laryngeal survey composite measure were not associated with or predict PES. Age, sex, peri-extubation steroid use, intubation duration and body mass index were not associated with PES. Even including ultrasonographic and indirect laryngoscopic examination of the airway, no single aspect of the CLT or combination with laryngeal parameters accurately predicts PES.

  14. Laryngeal Chondrosarcoma as a Rare Cause of Subglottic Stenosis

    Directory of Open Access Journals (Sweden)

    Kerem Kökoğlu

    2014-01-01

    Full Text Available Laryngeal chondrosarcoma (CS is a very rare entity. It is usually seen in 50–80-year olds. It is developed from cricoid cartilage largely. Patients have laryngeal CS complaint of respiratuvar distress, dysphonia, and dysphagia generally. A submucous mass is usually seen in physical examination with an intact mucosa. Distant metastasis is rare in CSs. Main treatment is surgical excision. An 82-year-old patient who has respiratuvar distress is presented in this paper and laryngeal CS is reviewed in the light of the literature.

  15. Laryngeal dysfunction after thyroid surgery: diagnostic and treatments

    OpenAIRE

    FINCK, Camille

    2006-01-01

    Vocal fold hypomobility after thyroidectomy is a frequent complication of thyroidectomy. Laryngeal nerve paresis or paralysis may present with various symptoms like dysphagia, aspiration, voice alteration or dyspnea. Are described: the normal anatomophysiology of the larynx, the symptoms of nerve trauma following thyroidectomy, techniques of thoroughfull laryngeal and voice examination, some clinical entities( unilateral recurrent nerve paralysis, bilateral recurrent nerve paralysis, superior...

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

    Science.gov (United States)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Satoshi Yamasaki

    2013-09-01

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

  18. Comparison of Medical and Voice Therapy for reflux Laryngitis Based on Acoustic and Laryngeal Characteristics

    Directory of Open Access Journals (Sweden)

    Abbas Dehestani Ardakani

    2011-12-01

    Full Text Available Background and Aim: Reflux laryngitis is extremely common among patients with voice disorder. Medical therapy approaches are not efficient enough. The main goal of this study is to assess the acoustic and laryngeal characteristics of patients with dysphonia before and after medical or voice therapy, and to evaluate the effectiveness of each.Methods: In this retrospective study, 16 reflux laryngitis patients were assessed. Five received complete voice therapy, tow ceased voice therapy and nine received medication. Perceptual voice evaluation was performed by a speech-language pathologist, the severity of voice problem was calculated, based on the affected acoustic and laryngeal characteristics pre- and post-treatment.Results: Post-treatment evaluation in patients who received complete voice therapy indicates 80 percent improvement in the severity of disorder and 100 percent improvement in the perceptual voice evaluation. After medical therapy, we observed that voice disorder and perceptual voice evaluation are improved 44 and 66 percent respectively. The improvement was statistically significant in both treatment approaches: complete voice therapy (P=0.039 and medical therapy (p=0.017.Conclusion: In patients with reflux laryngitis, most acoustic and laryngeal characteristics were normal and satisfying after the treatment. It can be concluded that the proficiency of voice therapy in improving the acoustic and laryngeal characteristics is comparable to medical therapy

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

    Science.gov (United States)

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

    2017-11-01

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

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

    Science.gov (United States)

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

    2007-01-01

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

  1. 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

  2. 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%.

  3. Laryngeal plexiform neurofibroma in a child.

    Science.gov (United States)

    Kasapoglu, Fikret; Ozdemircan, Talip; Erisen, Levent

    2013-06-01

    Neurofibromatosis (NF) is a genetically inherited, autosomal dominant disease, characterized by multiple cafe au lait spots, cutaneous neurofibromas and "Lisch nodules." Neurofibromatosis can develop from a neural source at any age. However, neurofibroma of the larynx is extremely rare and is usually manifested by obstructive airway symptoms. We encountered a 5-year-old child presenting with stridor and dyspnea, who had a diagnosis of laryngeal plexiform neurofibroma. The purpose of our report is the consideration of laryngeal NF in the differential diagnosis of dyspnea in infants and children.

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

    Science.gov (United States)

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

    2012-07-01

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

  5. Amiloidosis bucal Oral amyloidosis

    Directory of Open Access Journals (Sweden)

    Isabella Lima Arrais Ribeiro

    2012-03-01

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

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

    Science.gov (United States)

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

    2018-04-26

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

  7. An Unusual Laryngeal Foreign Body in Adult.

    Science.gov (United States)

    Ndiaye, Cire; Regonne, Eric Joel; Ahmed, Houra; Diom, Evelyne Siga; Deguenonvo, Richard Edouard Alain; Mbaye, Aminata; Zemene, Yilkal; Ndiaye, Issa Cheikh

    2016-01-01

    The accidental aspiration of a foreign body is a frequent domestic accident among children but a rare occurrence in adults. The laryngeal impaction of a coin is an unusual accident; only a few cases have been reported in the literature. Diagnosis is mostly achieved by clinicoradiological examinations. The authors report an uncommon case of laryngeal impaction of a coin in a 21-year-old patient, presenting with dysphonia without dyspnea or stridor. The extraction was performed by endoscopy.

  8. An Unusual Laryngeal Foreign Body in Adult

    OpenAIRE

    Ndiaye, Cire; Regonne, Eric Joel; Ahmed, Houra; Diom, Evelyne Siga; Deguenonvo, Richard Edouard Alain; Mbaye, Aminata; Zemene, Yilkal; Ndiaye, Issa Cheikh

    2016-01-01

    The accidental aspiration of a foreign body is a frequent domestic accident among children but a rare occurrence in adults. The laryngeal impaction of a coin is an unusual accident; only a few cases have been reported in the literature. Diagnosis is mostly achieved by clinicoradiological examinations. The authors report an uncommon case of laryngeal impaction of a coin in a 21-year-old patient, presenting with dysphonia without dyspnea or stridor. The extraction was performed by endoscopy.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. A case of adult congenital laryngeal cleft asymptomatic until hypopharynx cancer treatment.

    Science.gov (United States)

    Shimizu, Kotaro; Uno, Atsuhiko; Takemura, Kazuya; Ashida, Naoki; Oya, Ryohei; Kitamura, Takahiro; Takenaka, Yukinori; Yamamoto, Yoshifumi

    2018-06-01

    Laryngeal cleft is an anomaly of failed posterior closure of the larynx. Most cases are diagnosed and need treatment early in life due to respiratory and swallowing problems. We report an unusual case of a 66-year-old man with an asymptomatic laryngeal cleft until treatment for hypopharyngeal cancer. During concurrent chemoradiotherapy (CCRT), despite reduced tumor volume, he presented severe dysphagia and dyspnea, followed by severe pneumonia twice. Because CCRT had to be discontinued, a pharyngolaryngectomy was performed for the cancer treatment. The resected specimen showed total removal of the tumor and a total longitudinal cleft of the cricoid cartilage, classified as a type III laryngeal cleft by the Benjamin and Inglis' classification. A review of computed tomography images indicated that the redundant mucosa from bilateral edges closed the separation of the posterior cricoid cartilage and narrowed the laryngeal airway during CCRT. Adult presentations of laryngeal cleft are quite rare with only ten reported cases in English literature; the present case is of the oldest patient. Undiagnosed cases with laryngeal cleft may exist asymptomatically or without severe symptoms. The awareness of this condition may increase its diagnosis as a cause of diseases such as aspiration and recurrent pneumonia even in adult patients. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Acromegaly presented as a cause of laryngeal dyspnea.

    Science.gov (United States)

    Saussez, S; Mahillon, V; Chantrain, G; Thill, M P; Lequeux, T

    2007-12-01

    Acromegalic patients can develop obstructive sleep apnea syndrome or upper airflow obstruction. The development of dyspnea is unusual and the fixation of both vocal cords is exceptional. We report the case of a patient with bilateral vocal cord paralysis. Fiberoptic laryngoscopy and computed tomography (CT) of the neck showed a supra-glottic stenosis due to a swelling of the soft tissue. A tracheostomy was first performed. Thereafter, micro-laryngoscopy using laser vaporisation of the supra-glottic soft tissue was attempted but failed to remove the tracheostomy canula. Finally, blood tests and cerebral MRI revealed an acromegaly. The patient underwent a trans-sphenoidal resection of the pituitary adenoma. Fifteen months later, fiberoptic laryngoscopy showed bilateral restoration of vocal cord mobility and the tracheostomy canula was successfully removed after 18 months. Vocal cord fixation is probably due to hypopharyngeal and laryngeal soft tissue swelling and can be reversible after successful treatment of the adenoma.

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

    Science.gov (United States)

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

    2015-09-01

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

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

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

    2006-07-01

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

  14. Prevalence of laryngeal alterations in patients with erosive esophagitis

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    Coelho, Marina Serrato

    2010-06-01

    Full Text Available Introduction: The association between gastroesophageal reflux disease (GERD and laryngeal disorders has been much debated in recent years. Recent studies suggest an association between laryngeal symptoms and pharyngeal symptoms extra-esophageal reflux, as atypical presentation of Gastroesophageal Reflux Disease. Objectives: To correlate the presence of laryngeal to the grades of erosive esophagitis. Methods: A prospective study. Patients with findings of esophagitis on endoscopy were categorized according to LosAngeles and submitted a questionnaire followed by laryngoscopy. The chi-square test was used for statistical analysis (p<0.05. Results: Patients with typical symptoms of gastroesophageal reflux disease accounted for 96.6%. Eighteen had changes consistent with class A (60%, class B with seven (7% and 5 with classes C + D (16.6%. The presence of laryngeal changes were more prevalent in more severe esophagitis (grades C and D Los Angeles when compared to milder forms (classes A and B, a statistically significant difference (p<0.05. Conclusion: The laryngeal disorders are frequent findings in patients with esophagitis, more frequent the greater the degree of esophageal injury.

  15. MRI and echocardiography in the diagnosis of cardiac amyloidosis

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    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)

  16. 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.

  17. Laryngeal cancer at the Korle Bu Teaching Hospital Accra Ghana

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    Kitcher, E.D.; Cheyuo, C.; Yarney, J.; Gyasi, R. K.

    2006-01-01

    Laryngeal cancer is the commonest head and neck cancer seen at the Ear Nose and Throat (ENT) Unit Korle Bu Teaching Hospital. The aim of this study was to determine the number of cases of laryngeal cancer seen at the Korle Bu Teaching Hospital, establish epidemiological parameters of the disease and to outline preventive measures. One hundred and fifteen (115) patients who were managed for laryngeal cancer from 1st January 1998 to 31st December 2003 were studied retrospectively with respect to age, sex, duration of symptoms at presentation, risk factors, symptoms complex, histopathology, stage of tumor, details of treatment offered and follow up. The age range was 17-85 years with a mean of 55.5 years (SD10.7). Majority of the patients (90.4%) were above 40 years. The commonest symptom at presentation was dysphonia. A significant proportion of cases (37.3%) presented with locally advanced disease. The commonest histological type of laryngeal tumour seen was squamous cell carcinoma. The treatment offered consisted of radiotherapy for 83 (79.8%) patients and total laryngectomy with neck dissection when necessary for 17 (16.3%) patients who also had postoperative radiotherapy. Only 58 (69.9%) patients completed radiotherapy treatment and in all 32 (24.3 %) patients did not report for any treatment. Majority of patients failed to report for post treatment follow-up. We conclude that significant number of patients with laryngeal cancer presented with locally advanced disease and dysphonia was the commonest symptom. (au)

  18. Dysphonia as a sign of HPV laryngeal infection: a case report.

    Science.gov (United States)

    Longubuco, Carlos Eduardo Gama; dos Reis, Helena Lucia Barroso; Cavalcante, Fernanda Sampaio; de Pinho, Carla Renata Petillo; Oliveira, Nathalia Silva; Nicol, Alcina Frederica; Zamolyi, Renata Quintella; Ferreira, Dennis de Carvalho

    2014-12-11

    Voice dysfunction or dysphonia may be associated with several clinical conditions. Among these, laryngeal human papillomavirus (HPV)-induced lesions should be considered as a possible causative factor. We report a case of dysphonia in a patient presenting with an HPV laryngeal lesion. We also discuss the clinical features of the disease, its histopathological findings, and treatment and rigorous follow-up. We report a case of laryngeal papilloma in a 29-year-old, Afro-descendant, male patient with dysphonia. He was a non-smoker and was not a drug user. Videolaryngostroboscopy revealed signs suggestive of pharyngolaryngeal reflux. The right vocal fold presented with a papillomatous aspect in the posterior third, which underwent excision. Histopathological examination showed a nodular lesion of the right vocal fold, conclusive of squamous papilloma with absence of malignancy. Patients presenting with persistent voice dysfunction or dysphonia should be investigated for possible laryngeal HPV infection. Diagnostic confirmation by HPV genotyping is important for follow-up of potential recurrence.

  19. Anesthesia management for a case of laryngeal keel placement

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    Kundan Gosavi

    2017-01-01

    Full Text Available Congenital laryngeal web is a rare anomaly with incidence of 1 in 10,000 births. Its clinical presentation may range from an asymptomatic patient or mild hoarseness of voice to severe respiratory stridor. The primary goals of surgical intervention for congenital laryngeal web are to establish a patent airway and to achieve a good voice quality. As recurrence rate after plain excision of laryngeal web is very high, its removal may be coupled by placement of a silastic keel in between vocal cords. Endolaryngeal placement of a keel is definitely less invasive than laryngofissure, but little is known about its anesthesia management. Frequent ventilatory adjustment and endotracheal tube (ETT manipulations are needed along with vigilant monitoring. Risk of perforation or accidental dislodgment of the ETT and laryngeal edema are other concerns in management. We report a case.

  20. An Unusual Laryngeal Foreign Body in Adult

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    Cire Ndiaye

    2016-01-01

    Full Text Available The accidental aspiration of a foreign body is a frequent domestic accident among children but a rare occurrence in adults. The laryngeal impaction of a coin is an unusual accident; only a few cases have been reported in the literature. Diagnosis is mostly achieved by clinicoradiological examinations. The authors report an uncommon case of laryngeal impaction of a coin in a 21-year-old patient, presenting with dysphonia without dyspnea or stridor. The extraction was performed by endoscopy.

  1. 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.

  2. 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

  3. Diagnosis of secondary amyloidosis in alkaptonuria.

    Science.gov (United States)

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

    2014-09-26

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

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

    Science.gov (United States)

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

    2014-07-01

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

  5. 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.

  6. 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

  7. Botulinum toxin injection in laryngeal dyspnea.

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    Woisard, Virginie; Liu, Xuelai; Bes, Marie Christine Arné; Simonetta-Moreau, Marion

    2017-02-01

    Data, regarding the use of botulinum toxin (BT-A) in laryngeal dyspnea, are scarce, coming from some cases reports in the literature, including Vocal fold paralysis, laryngeal dystonia, vocal cord dysfunction also called paradoxical motion of the vocal fold (PMVF), and post-neuroleptic laryngeal dyskinesia. There is no consensus regarding the muscles and the doses to inject. The aim of this study is to present a retrospective review of patients treated in our ENT Department by BT-A injection in this indication. This study is a retrospective study describing patients who underwent an injection of botulinum toxin for laryngeal dyspnea in the ENT Department from 2005 to 2015 years. The inclusion criteria were a dyspnea associated with a laryngeal dysfunction, confirmed by flexible fiberoptic nasopharyngolaryngoscopy. Information concerning the causes of the dyspnea, the botulinum toxin BT-A injections procedure, post-injection follow-up, and respiratory outcome were collected for all patients included. In the group of 13 patients included, the main cause identified as principal factor linked with the short breath was: a bilateral VF paralysis (Patel et al., Otolaryngol Head Neck Surg 130:686-689, 7), laryngeal dystonia (Balkissoon and Kenn, Semin Respir Crit Care Med 33:595-605, 2), Anxiety syndrome associated with unilateral vocal fold paralysis or asthma (Marcinow et al., Laryngoscope 124:1425-1430, 3), and an isolated asthma (Zwirner et al., Eur Arch Otorhinolaryngol 254:242-245, 1). Nine out of the thirteen patients were improved by the injections. A BT-A-induced stable benefit for four patients led them to stop the injections in the follow-up. Good outcome was observed in five other patients (main cause: bilateral VP paralysis), allowing a progressive lengthening of the delay between BT-A injections. Four patients did not report a positive risk/benefit ratio after BT-A injections; two of them (with bilateral VF paralysis), because of respiratory side effects and

  8. 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.

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

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

    2010-05-01

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

  10. [Laryngeal foreign bodies: management in children in Senegal].

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    Diop, E M; Tall, A; Diouf, R; Ndiaye, I C

    2000-01-01

    Inhalation of foreign bodies is a frequent accident in children. It remains severe in the case of laryngeal foreign bodies. Retrospectively, for a 16-year period, 65 laryngeal foreign bodies have been treated (44.8%), among 145 cases of airway foreign bodies, in the ENT department of Dakar University hospital. Etiological, clinical and therapeutic aspects were reviewed. Average age was 36 months, with a sex-ratio of 2.42 in favour of males. The time lag (time between the accident and admission to the department) was particularly long; 73.33% of the children were admitted more than 24 hours after the event. Eighty-three percent of the patients presented greater or lesser laryngeal dyspnea. Tracheostomy was performed in 55.4% of the patients. Average duration for abiation of the canula was ten days. Three cases of death were recorded (4.16%). The frequency of 44.8% for laryngeal localization of foreign bodies appears to be the highest in the literature. If the appropriate treatment for foreign bodies in the respiratory tract is endoscopic removal, the tracheostomy nevertheless occupies a central place in the management of the disease. This procedure may be recommended to all ENT specialists working in similar conditions. In spite of its inherent complications, tracheostomy allows reduction of mortality in relation to laryngeal foreign bodies. Improvement of prognosis requires prevention based on widespread public information and improving technical infrastructures.

  11. Relationship Between Laryngeal Electromyography and Video Laryngostroboscopy in Vocal Fold Paralysis.

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    Maamary, Joel A; Cole, Ian; Darveniza, Paul; Pemberton, Cecilia; Brake, Helen Mary; Tisch, Stephen

    2017-09-01

    The objective of this study was to better define the relationship of laryngeal electromyography and video laryngostroboscopy in the diagnosis of vocal fold paralysis. Retrospective diagnostic cohort study with cross-sectional data analysis METHODS: Data were obtained from 57 patients with unilateral vocal fold paralysis who attended a large tertiary voice referral center. Electromyographic findings were classified according to recurrent laryngeal nerve, superior laryngeal nerve, and high vagal/combined lesions. Video laryngostroboscopy recordings were classified according to the position of the immobile fold into median, paramedian, lateral, and a foreshortened/hooded vocal fold. The position of the paralyzed vocal fold was then analyzed according to the lesion as determined by electromyography. The recurrent laryngeal nerve was affected in the majority of cases with left-sided lesions more common than right. Vocal fold position differed between recurrent laryngeal and combined vagal lesions. Recurrent laryngeal nerve lesions were more commonly associated with a laterally displaced immobile fold. No fold position was suggestive of a combined vagal lesion. The inter-rater reliability for determining fold position was high. Laryngeal electromyography is useful in diagnosing neuromuscular dysfunction of the larynx and best practice recommends its continued implementation along with laryngostroboscopy. While recurrent laryngeal nerve lesions are more likely to present with a lateral vocal fold, this does not occur in all cases. Such findings indicate that further unknown mechanisms contribute to fold position in unilateral paralysis. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  12. 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.

  13. Laryngeal manifestations of relapsing polychondritis and a novel treatment option.

    Science.gov (United States)

    Childs, Lesley F; Rickert, Scott; Wengerman, Oscar C; Lebovics, Robert; Blitzer, Andrew

    2012-09-01

    Laryngotracheal involvement in relapsing polychondritis (RP) is rare. However, it is one of the most common causes of death in this patient population. We present three patients who primarily presented with laryngeal manifestations of RP and a novel treatment option for bamboo nodules. Retrospective chart review and comprehensive review of the literature. Two patients first presented to an otolaryngologist because of hoarseness and chronic cough that eventually progressed to dyspnea upon exertion. Laryngeal examination revealed subglottic stenoses. Upon rheumatologic workup both were diagnosed with RP. After treatment with steroids and immunosuppressive drugs, one of the patient's laryngeal symptoms improved, whereas the other required dilation procedures. Neither patient had classic auricular or nasal symptoms upon initial presentation. The third patient was being treated for spasmodic dysphonia and was noted to have bamboo nodules with accompanying dysphonia. Rheumatologic workup revealed RP and systemic treatment ensued. Unfortunately, her symptoms of hoarseness persisted despite systemic treatment. A pulsed-potassium-titanyl-phosphate (KTP) laser was applied to the bilateral bamboo nodules, which eventually caused resolution of her vocal fold lesions and dysphonia. We present three patients with RP, all of whom sought health care by an otolaryngologist primarily. Awareness of this disease entity and the possibility for early laryngeal involvement is crucial for proper care of those with this life-threatening disease. Copyright © 2012 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  14. Laryngeal adenoid cystic carcinoma: case report

    Directory of Open Access Journals (Sweden)

    André Del Negro

    Full Text Available CONTEXT: Adenoid cystic carcinomas are malignant tumors that occur in both the major and the minor salivary glands. A laryngeal location is rare because of the paucity of accessory salivary glands in this area. Adenoid cystic carcinomas account for less than 1% of all malignant tumors in the larynx, and only about 120 cases have been reported in the literature. These tumors have a slight female predisposition, and their peak incidence is in the fifth and sixth decades of life. In this article, we describe a case of laryngeal adenoid cystic carcinoma and discuss its clinical characteristics and treatment. CASE REPORT: We report on a case of laryngeal adenoid cystic carcinoma in a 55 year-old female patient who presented with dyspnea and hoarseness. Features of the diagnostic and therapeutic evaluation are described and the clinical management of such cases is outlined. The clinical course, definitive treatment strategy and surgical procedure, and also adjuvant treatment with irradiation are discussed. Although the tumor is radiosensitive, it is not radiocurable.

  15. Primary conjunctival amyloidosis

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Andrew Kelsey, MD

    2016-03-01

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

  17. Laryngeal Aerodynamics in Healthy Older Adults and Adults with Parkinson's Disease

    Science.gov (United States)

    Matheron, Deborah; Stathopoulos, Elaine T.; Huber, Jessica E.; Sussman, Joan E.

    2017-01-01

    Purpose: The present study compared laryngeal aerodynamic function of healthy older adults (HOA) to adults with Parkinson's disease (PD) while speaking at a comfortable and increased vocal intensity. Method: Laryngeal aerodynamic measures (subglottal pressure, peak-to-peak flow, minimum flow, and open quotient [OQ]) were compared between HOAs and…

  18. Clinical Features and Differential Diagnoses in Laryngeal Mucoepidermoid Carcinoma

    OpenAIRE

    Mokhtari, Sepideh; Mokhtari, Saeedeh

    2011-01-01

    Mucoepidermoid carcinoma is the most common malignant tumor of salivary glands. However, it is a rare entity in larynx. Laryngeal cases are frequently misdiagnosed with other malignancies and they are under-reported. So, recognizing the clinical and histological features of this tumor is essential. Laryngeal mucoepidermoid carcinoma can arise in supraglottis, glottis and subglottis. Generally, it presents as a submucosal mass; therefore, progressive symptoms without any identifiable lesion in...

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

    Science.gov (United States)

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

    2006-08-01

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

  20. Cyclooxygenase-2 expression and clinical parameters in laryngeal squamous cell carcinoma, vocal fold nodule, and laryngeal atypical hyperplasia.

    Science.gov (United States)

    Sayar, Cağdaş; Sayar, Hamide; Özdemir, Süleyman; Selçuk, Tahsin; Görgülü, Orhan; Akbaş, Yücel; Kemal Olgun, Mustafa

    2013-01-01

    The diagnostic role of cyclooxygenase-2 (COX-2) expression in laryngeal atypical hyperplasia, vocal fold nodule, and laryngeal squamous cell carcinoma was examined. Specimens obtained from patients diagnosed with vocal fold nodule (n = 35), atypical hyperplasia (n = 35), laryngeal squamous cell carcinoma (n = 35), and clinical parameters were evaluated retrospectively. Although no staining was observed in patients with vocal fold nodules, staining was noted in laryngeal atypical hyperplasia and squamous cell carcinoma. The percentage of COX-2 staining was the highest in the carcinoma group. It was determined that COX-2 staining was significantly associated with laryngeal squamous cell carcinoma. It should be noted that overexpression of COX-2, a potentially important factor in the evolution of carcinogenesis in precancerous lesions, might be an indicator of the development of carcinoma. Copyright © 2012 Wiley Periodicals, Inc.

  1. Allergic laryngitis: unraveling the myths.

    Science.gov (United States)

    Stachler, Robert J; Dworkin-Valenti, James P

    2017-06-01

    This article provides a thorough review of the literature highlighting the articles that have advanced our knowledge about the sensitivity of the larynx to allergens in the air or ones consumed. This area of inquiry requires continued interest and investigation. As the field of clinical laryngology changes, and more information is discovered about the possible causal association between allergy and vocal pathologies, practicing otolaryngologists, allergists, and other medical professionals may discover more comprehensive methods to evaluate and treat their allergic patients, particularly those who present with complaints of dysphonia, dysphagia, laryngopharyngeal reflux (LPR), and/or dyspnea. There continues to be epidemiological studies designed to describe the relationship of allergy to vocal symptoms and signs. Both population and smaller studies have recently attempted to link these two conditions. Unfortunately, the patient with chronic laryngeal complaints is often tagged by default with the diagnosis of LPR and treated with proton pump inhibitors, which are not always beneficial. The endoscopic assessment may not be as reliable to make the diagnosis of LPR as the examination is subjective and the inter-rater reliability is low. It has been demonstrated by direct laryngeal provocation studies that sticky-viscous endo-laryngeal mucous is the only reliable finding consistently associated with allergy potential allergic tissue reactivity. The interrelationship of allergic sensitivity and chronic laryngitis in certain individuals is becoming clearer because our knowledge of inquiry has increased and the available routine technology to diagnose these conditions has remarkably improved. Notwithstanding these advancements, much more research is needed on this subject to reduce the frequency of mis-diagnoses and mis-management of allergic patients.

  2. A case of laryngeal neurofibroma associated with neurofibromatosis type 2

    International Nuclear Information System (INIS)

    Cihangiroglu, M.; Yilmaz, S.; Yildirim, H.; Ozdemir, H.; Altinsoy, B.; Ogur, E.

    2002-01-01

    Purpose: Laryngeal neurofibromas have been reported in 16 patients with NF1, and schwannomas, in two patient with NF1 and 2 patients with NF2. To the best our knowledge our case is the first to document a laryngeal neurofibroma in a patient with NF2. Another unique feature of our case is the coexistence of multiple intramedullary tumors, which has not previously been reported in a patient with a laryngeal neurofibroma. Material and methods: A 32-year-old woman presented with a history of cataract, hoarseness and dysphonia since childhood, which had recently become worse. The patient also had hearing disability for low frequencies. Results: Laryngoscopy revealed a 2x2x3.5 cm smooth-surfaced submucosal supraglottic mass. On CT of the neck, the lesion was seen as a round and well-defined hypopharyngeal mass extended through and obliterating the left supraglottic space. It was hypodense on unenhanced CT images and slightly enhanced with IV contrast administration. On MR imaging, the mass was heterogeneously hypointense on T1-weighted images and hyperintense on T2-weighted images, with moderate homogenous enhancement after gadolinium administration. Bilateral vestibular schwannomas and multiple intramedullary masses (presumed to be ependymoma or astrocytoma) were delineated on these MR images The patient was diagnosed as having NF-2 and the laryngeal mass was totally resected. On histopathological examination, the mass were consistent with neurofibroma. Conclusion: Dysphonia and hoarseness may be the only presenting symptoms suggesting the possibility of a laryngeal nerve sheath tumor, and neurofibroma should be included in differential diagnosis of laryngeal masses in patients with NF2. (authors)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  4. The effect of azelastine hydrochloride on radiation dermatitis and pharyngo-laryngeal mucositis in radiotherapy for laryngeal cancer

    International Nuclear Information System (INIS)

    Sako, Tsukasa; Ishiguro, Ruichiro; Morimoto, Noriko; Sakamoto, Yutaka; Fukuda, Hiroyuki

    1998-01-01

    It has recently been suggested that reactive oxides produced by inflammation may result in cell injury, leading to mucositis and dermatitis. Azelastine hydrochloride suppresses the production of cytokines and reactive oxygen species, and some reports have documented its effectiveness in treating radiation mucositis and dermatitis. Therefore, we investigated the effectiveness of azelastine hydrochloride in preventing these diseases during radiation therapy for laryngeal cancer. Subjects were patients with laryngeal carcinomas who received curative radiation therapy. A close of 1 mg of azelastine hydrochloride was administered orally twice a day, from the start of the radiation therapy until one-four weeks after the completion of therapy. Chronological changes in the pharyngo-laryngeal cavity and the neck skin of the patients who received azelastine hydrochloride were compared with those of patients who did not. In the patients who received the azelastine hydrochloride, the onset of pharyngo-laryngeal mucositis and dermatitis was suppressed; symptoms were relieved earlier and were not exacerbated. No severe side effects were observed, and the effectiveness of the radiation therapy was not affected. The administration of azelastine hydrochloride concurrently with radiation therapy for laryngeal cancer suppressed the onset of pharyngo-laryngeal mucositis and dermatitis and alleviated the severity of these diseases. (K.H.)

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

    Science.gov (United States)

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

    2013-12-17

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

  6. Radiation therapy for life-threatening huge laryngeal hemangioma involving pharynx and parapharyngeal space.

    Science.gov (United States)

    Huang, Chun-Ming; Lee, Ka-Wo; Huang, Chih-Jen

    2013-04-01

    Adult hemangiomas are rare, slowly progressing vascular tumors. Potential complications include laryngeal involvement or massive tumor burden. A case of recurrent, bulky laryngeal hemangioma involving the parapharyngeal space is presented herein. The clinical course and treatment are described, and a series of MRI studies are compared to demonstrate the treatment response. A 35-year-old woman presented with progressive hoarseness, neck fullness, and intermittent dyspnea caused by a bulky laryngeal hemangioma. Steroid therapy had a limited response. Radiation therapy with a total dose of 40 Gray (Gy) in 20 fractions successfully relieved her symptoms. Image studies after therapy revealed moderate tumor regression. The patient showed no serious complications during the next 2 years of follow-up. Radiation therapy may be effective in intractable and function-threatening laryngeal hemangiomas. Copyright © 2012 Wiley Periodicals, Inc.

  7. Laryngeal Chondroma: An Unusual Complication Endotracheal Entubation.

    Science.gov (United States)

    Gökdoğan, Ozan; Koybasioglu, Ahmet; Ileri, Fikret

    2016-06-01

    Laryngeal cartilaginous framework tumors are very rare. Chondroma and chondrosarcoma are the most common types of these tumors. A 27-year-old man with a history of intubation presented with exercise-induced dyspnea. A computed tomography scan of larynx showed a rounded and circumscribed mass without infiltration of the adjacent structures which obstructs 75% of airway. Histopathological investigation of the mass revealed the chondroma of the larynx. The patients' history of intubation trauma with the subsequent progressive onset of clinical symptoms demonstrates the relationship between these 2 entities. Clinicians should consider laryngeal chondroma in the differential diagnosis of dyspnea after endotracheal intubation.

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

    Science.gov (United States)

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

    2018-02-23

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

  9. 18F-fluorodeoxyglucose positron emission tomography might be useful for diagnosis of hepatic amyloidosis

    Directory of Open Access Journals (Sweden)

    Tawada A

    2014-06-01

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

  10. Pediatric mumps with laryngeal edema.

    Science.gov (United States)

    Hattori, Yujiro; Oi, Yasufumi; Matsuoka, Ryo; Daimon, Yumi; Ito, Asami; Kubota, Wataru; Konishi, Kyoko; Onguchi, Toshimi; Sato, Akihiro; Yamashita, Yukio; Ishihara, Jun

    2013-10-01

    Mumps virus infection primarily affects the salivary glands and may incur various complications. Laryngeal edema is such a rare complication that few adult cases have been reported. We report the first known pediatric patient with mumps with laryngeal edema. An 8-year-old boy developed dyspnea after a rapidly progressive swelling of his face and neck. Laryngoscopy revealed edematous changes in the supraglottic and subglottic regions, and computed tomography confirmed significant laryngeal edema in addition to swelling of the cervical soft tissue and the salivary glands. Laboratory findings revealed a high serum amylase level and confirmed the diagnosis of mumps. Intravenous steroid administration alleviated the dyspnea, although the patient required temporary tracheal intubation to maintain airway patency. He did not need tracheotomy and did not experience any other complications. Laryngeal edema must be regarded as a rare, potentially life-threatening complication of mumps. When mumps is diagnosed with significant swelling of the neck, an emergency airway should be established to prevent airway obstruction.

  11. Section four: laryngitis and dysphonia.

    Science.gov (United States)

    Hueston, William J; Kaur, Dipinpreet

    2013-12-01

    Acute laryngitis is most often caused by viral illnesses through direct inflammation of the vocal cords or from irritation due to postnasal drainage. Bacterial infections, such as acute epiglottitis, also can cause dysphonia but typically have other systemic symptoms as well as respiratory distress. Chronic laryngitis is characterized by symptoms lasting more than 3 weeks. Chronic vocal cord issues can be related to overuse or stress on the vocal cords resulting in nodules or polyps. Individuals in certain occupations, such as singers, school teachers, and chemical workers, are at greater risk of chronic laryngitis. The diagnostic approach to chronic laryngitis should include visualization of the vocal cords to rule out potential malignant lesions. For acute and chronic overuse symptoms, the best treatment is vocal rest. The use of antibiotics or decongestants should be discouraged. Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.

  12. Primary laryngeal localization of multiple myeloma: A case report

    OpenAIRE

    Allegra, Eugenia; Marino, Nicol?; Modica, Domenico; Emmanuele, Carmela; Saita, Vincenzo

    2017-01-01

    Multiple myeloma is a lymphoproliferative disease that may involve the bone marrow as well as extramedullary soft tissues. However, laryngeal localization of multiple myeloma is extremely rare. We herein present the case of a 68-year-old male patient with a history of dyspnea, dysphonia and dysphagia. Laryngoscopic examination revealed a lesion involving the right glottis and right vestibular (false) vocal fold, with absence of ipsilateral laryngeal motility and constriction of the airway. Co...

  13. 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

  14. 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...

  15. 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...

  16. hemangiomes larynges de l'adulte a propos de 9 cas adult laryngeal ...

    African Journals Online (AJOL)

    16 juin 2006 ... pique, avec hémostase locale. Deux récidives ont été notées, pour lesquels une seconde exérèse instrumentale par voie endoscopique a été réalisée avec une évolution favorable. Le recul moyen était de 9 ans. SUMMARY. The adult laryngeal hemangioma is extremely rare and presents histological and ...

  17. [The systemic approach to the rehabilitation of the patients presenting with laryngeal cancer after the resection of the organ and laryngectomy with tracheoesophageal by-pass and endoprosthetics].

    Science.gov (United States)

    Kryukov, A I; Reshetov, I V; Kozhanov, L G; Sdvizhkov, A M; Kozhanov, A L

    The objective of the present study was to enhance the effectiveness of rehabilitation of the patients presenting with laryngeal cancer after the resection of the organ and laryngotomy with tracheoesophageal by-pass and endoprosthetics. Our experience in this field is based on the treatment of 102 patients. They were distributed by the nosological forms as follows: primary laryngeal cancer in 97 patients including T1NoMo - 8 (8.2%), T2NoMo - 63 (65%), T3NoMo - 18 (17.6%), T2N1Mo - 1 (0.9%), T4NoMo - 3 (2.9%), and T4N1M0 - 4 (3.9%), root of the tongue cancer spreading over the vestibular part of the larynx in one patient, laryngeal sarcoma in one patient, relapse of cancer following the full-dose radiotherapy and organ-sparing surgery in 3 patients. Laryngeal resection was performed in 83 patients; 19 patients underwent laryngectomy with tracheoesophageal by-pass and endoprosthetics using a domestically manufactured voice prosthesis. The systemic approach to the rehabilitation of the patients and the use of the proposed treatment algorithm made it possible to restore the function of the larynx by means of organ-sparing surgery in 79 (95.1%) of the 83 patients. The vocal function in the patients following laryngectomy with tracheoesophageal by-pass and endoprosthetics was restored in 18 (94.7%) of the 19 patients.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-02-15

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

  19. A case of laryngeal angioleiomyoma and review of literature

    OpenAIRE

    Zhao, Xue; Yu, Dan; Zhao, Yin; Liu, Yan; Qi, Xinmeng; Jin, Chunshun

    2015-01-01

    Angioleiomyoma is a rare benign vascular smooth muscle tumor that arise from the tunica media of veins and arteries. Here a case of laryngeal angioleiomyoma in a 57-year-old Chinese man is reported. The patient presented with dysphagia for one and half-month and dyspnea during the previous one week, was hospitalized for treatment with a tracheotomy and laryngofissure with the unblock mass excision. Final pathological evaluation of the neoplasm confirmed a diagnosis of laryngeal angioleiomyoma...

  20. l'amylose laryngée à propos d'un cas laryngeal location of amylosis

    African Journals Online (AJOL)

    maxillofac. 1992 ; 93 : 54-57. 5-Vicente Villagomez, Felicitos Santos, Ramiro Santos and al. Amyloidosis: Uncommon cause of dysphonia. Head and Neck Surgery 2004, 13: 1275-276. 6- Quinquenel ML, Le Coza, Desrues B et ...

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

    Science.gov (United States)

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

    2013-10-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Dysphagia Caused by Chronic Laryngeal Edema.

    Science.gov (United States)

    Delides, Alexander; Sakagiannis, George; Maragoudakis, Pavlos; Gouloumi, Αlina-Roxani; Katsimbri, Pelagia; Giotakis, Ioannis; Panayiotides, John G

    2015-10-01

    A rare case of a young female with chronic diffuse laryngeal edema causing severe swallowing difficulty is presented. The patient was previously treated with antibiotics and steroids with no improvement. Diagnosis was made with biopsy of the epiglottis under local anesthesia in the office.

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

    Directory of Open Access Journals (Sweden)

    Raffaele Scala

    2015-01-01

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

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

    International Nuclear Information System (INIS)

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

    1987-01-01

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

  6. MRI of pathology-proven peripheral nerve amyloidosis

    International Nuclear Information System (INIS)

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

    2017-01-01

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

  7. MRI of pathology-proven peripheral nerve amyloidosis

    Energy Technology Data Exchange (ETDEWEB)

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

    2017-01-15

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

  8. Laryngeal sensitivity evaluation and dysphagia: Hospital Sírio-Libanês experience

    Directory of Open Access Journals (Sweden)

    Orlando Parise Junior

    Full Text Available CONTEXT: Laryngeal sensitivity is important in the coordination of swallowing coordination and avoidance of aspiration. OBJECTIVE: To briefly review the physiology of swallowing and report on our experience with laryngeal sensitivity evaluation among patients presenting dysphagia. TYPE OF STUDY: Prospective. SETTING: Endoscopy Department, Hospital Sírio-Libanês. METHODS: Clinical data, endoscopic findings from the larynx and the laryngeal sensitivity, as assessed via the Flexible Endoscopic Evaluation of Swallowing with Sensory Testing (FEESST protocol (using the Pentax AP4000 system, were prospectively studied. The chi-squared and Student t tests were used to compare differences, which were considered significant if p < or = 0.05. RESULTS: The study included 111 patients. A direct association was observed for hyperplasia and hyperemia of the posterior commissure region in relation to globus (p = 0.01 and regurgitation (p = 0.04. Hyperemia of the posterior commissure region had a direct association with sialorrhea (p = 0.03 and an inverse association with xerostomia (p = 0.03. There was a direct association between severe laryngeal sensitivity deficit and previous radiotherapy of the head and neck (p = 0.001. DISCUSSION: These data emphasize the association between proximal gastroesophageal reflux and chronic posterior laryngitis, and suggest that decreased laryngeal sensitivity could be a side effect of radiotherapy. CONCLUSIONS: Even considering that these results are preliminary, the endoscopic findings from laryngoscopy seem to be important in the diagnosis of proximal gastroesophageal reflux. Study of laryngeal sensitivity may have the potential for improving the knowledge and clinical management of dysphagia.

  9. ‘ SILENT’ LARYNGEAL FOREIGN BODY

    OpenAIRE

    Chandrasekhar; Sreenivas

    2015-01-01

    Laryngeal foreign bodies in adults are rare. The foreign bodies accidentally entering the larynx are symptomatic in the form of choking , stridor or even death. We are presenting a rare case of foreign body in the larynx in a 42 year old male who was symptom free except for dysphonia. The foreign body was removed successfully under local anesthesia.

  10. Laryngeal morbidity after tracheal intubation

    DEFF Research Database (Denmark)

    Sørensen, M K; Rasmussen, N; Kristensen, M S

    2013-01-01

    BACKGROUND: Tracheal intubation may cause vocal fold damage. The trial was designed to assess laryngeal morbidity comparing the Endoflex(®) tube with a conventional endotracheal tube with stylet. We hypothesised that laryngeal morbidity within the first 24 h after extubation would be lower...... with the Endoflex tube than with the conventional endotracheal tube with stylet because of less rigidity. METHODS: This randomised trial included 130 elective surgical patients scheduled for general anaesthesia with endotracheal intubation. Pre- and post-operative assessment of hoarseness, vocal fold pathology......% with the Endoflex tube and 55% with the endotracheal tube with stylet at 24 h after extubation (P = 0.44). Post-operative vocal fold injury was present in 23% in the Endoflex tube group and in 36% in the endotracheal tube with stylet group (P = 0.13). The increase in shimmer, the voice analysis variable reflecting...

  11. 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.

    Science.gov (United States)

    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

  12. Changes in survival in locally advanced laryngeal carcinoma over past three decades

    International Nuclear Information System (INIS)

    EL-Zawahry, I.M.Sh.

    2013-01-01

    Laryngeal cancer is the commonest carcinoma of the head and neck region with Squamous-cell carcinomas (SCC) in 90 % of cases (Birchall Met al 2008) The incidence of laryngeal cancer was relatively about 160,000 new cases per year. The disease predominantly affects men; about 2.4% of all cancer cases and 2.1% of all cancer deaths worldwide ,also in United States in 2012 the Es)mated new cases is 12,360 and deaths from laryngeal cancer is 3,650. The majority of patients with SCC of larynx present with locally advanced (LA) disease.(American Cancer Society, 2012) The incidence of laryngeal cancer in the Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK), Faculty of Medicine, Cairo University from year 2005 - 2008 is 3.1%per year. Advanced laryngeal cancer is generally considered as the disease in stages III and IV based on the primary tumor extension and/or the presence of metastatic lymph node(s) in the neck and it accounts for roughly 40% to 50% of patients with laryngeal cancer (Chen AY, et al 2006). From the second half of the 20th century total laryngectomy combined with a neck dissection was considered a treatment of choice for advanced laryngeal cancer (Genden EM et al, 2007). In most institutions, postoperative radiotherapy as adjuvant treatment following ablative surgery with radiation doses up to 60-66 Gy has also become the standard approach for patients with stage III-IV laryngeal cancer (Corvo R , 2007). However, the treatment of advanced laryngeal cancer seams to be a permanent challenge, but the management of patients with advanced laryngeal cancer has become more complex as other modalities including induction chemotherapy followed by radiotherapy or concurrent chemo-radiotherapy have evolved with the goal of preserving the larynx and reserved total laryngectomy as a salvage procedure for cases with less than 50% response to induction chemotherapy or in those who have persistent disease following concurrent chemo

  13. Guideline of transthyretin-related hereditary amyloidosis for clinicians

    Directory of Open Access Journals (Sweden)

    Ando Yukio

    2013-02-01

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

  14. Use of laryngeal mask airway for prolonged ventilatory support in a preterm newborn.

    Science.gov (United States)

    Fernández-Jurado, Ma Isabel; Fernández-Baena, Mariano

    2002-05-01

    We present the case report of a preterm, low weight newborn with dysmorphic features and micrognathia in whom a laryngeal mask airway was inserted and maintained for 44 h for ventilatory support after several failed intubations. No complications associated with laryngeal mask airway use were apparent.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-01-15

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

  16. Cardiac amyloidosis

    International Nuclear Information System (INIS)

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

    2005-01-01

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

  17. Laryngeal paralysis associated with a muscle pseudotumour in a young dog

    Directory of Open Access Journals (Sweden)

    Francesca Rizzo

    2017-08-01

    Full Text Available An 18-month-old male entire Bloodhound dog was presented with a six-week history of progressive inspiratory dyspnoea, stridor, dysphonia and exercise intolerance. CT scan performed elsewhere had revealed the presence of an unencapsulated nodular mass (3x1x5 cm dorsal to the larynx and first tracheal rings. Laryngoscopy demonstrated the presence of bilateral laryngeal paralysis and distorted laryngeal architecture suggestive of extraluminal compression. Histopathology results of incisional biopsies from the mass were suggestive of a benign non-neoplastic muscular lesion. Surgery was performed to manage laryngeal paralysis and attempt mass excision. A second histopathology examination confirmed an inflammatory and dysplastic lesion suggestive of a pseudotumour. All clinical signs resolved after surgery and at the 13 months follow-up the dog remains asymptomatic. To the authors’ knowledge, this is the first report of a case of laryngeal paralysis caused by a muscle pseudotumour in a young dog.

  18. A case of laryngeal palsy and persistent aspiration pneumonia following radiotherapy for laryngeal carcinoma

    International Nuclear Information System (INIS)

    Nakao, Kazunari; Tayama, Niro; Mizuno, Masahiro; Niimi, Seiji.

    1997-01-01

    A 80-year-old man developed impairment in his laryngeal movement, vocal fold fixation and severe misdeglutition after radiotherapy for laryngeal carcinoma. Despite of several surgical treatments for aspiration pneumonia, the misdeglutition did not cease because of the stiffness in his larynx until a laryngectomy was finally performed. The resected larynx showed marked fibrosis, and it was considered as a late complication of radiotherapy. The treatment course in this difficult case is discussed. (author)

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

    Directory of Open Access Journals (Sweden)

    Cemal Selçuk İşoğlu

    2015-06-01

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

  20. SELECTIVE LARYNGEAL ABDUCTOR REINNERVATION IN CATS USING A PHRENIC-NERVE TRANSFER AND ORG-2766

    NARCIS (Netherlands)

    MAHIEU, HF; VANLITHBIJL, JT; GROENHOUT, C; TONNAER, JADM; DEWILDE, P

    Reinnervation of the recurrent laryngeal nerve following nerve injury often leads to laryngeal synkinesis. Selective reinnervation of adductor and abductor muscles might be able to avoid synkinesis. This study presents the results of selective abductor reinnervation in cats, using a phrenic nerve

  1. Contemporary management of advanced laryngeal cancer.

    Science.gov (United States)

    Britt, Christopher J; Gourin, Christine G

    2017-10-01

    The treatment of advanced laryngeal cancer has undergone a paradigm shift in recent years, with an increase in chemoradiation for organ preservation and a decrease in primary surgery. This review will summarize the contemporary management of advanced laryngeal cancer and discuss treatment-related toxicity and strategies to improve outcomes. NA.

  2. [A web-based integrated clinical database for laryngeal cancer].

    Science.gov (United States)

    E, Qimin; Liu, Jialin; Li, Yong; Liang, Chuanyu

    2014-08-01

    To establish an integrated database for laryngeal cancer, and to provide an information platform for laryngeal cancer in clinical and fundamental researches. This database also meet the needs of clinical and scientific use. Under the guidance of clinical expert, we have constructed a web-based integrated clinical database for laryngeal carcinoma on the basis of clinical data standards, Apache+PHP+MySQL technology, laryngeal cancer specialist characteristics and tumor genetic information. A Web-based integrated clinical database for laryngeal carcinoma had been developed. This database had a user-friendly interface and the data could be entered and queried conveniently. In addition, this system utilized the clinical data standards and exchanged information with existing electronic medical records system to avoid the Information Silo. Furthermore, the forms of database was integrated with laryngeal cancer specialist characteristics and tumor genetic information. The Web-based integrated clinical database for laryngeal carcinoma has comprehensive specialist information, strong expandability, high feasibility of technique and conforms to the clinical characteristics of laryngeal cancer specialties. Using the clinical data standards and structured handling clinical data, the database can be able to meet the needs of scientific research better and facilitate information exchange, and the information collected and input about the tumor sufferers are very informative. In addition, the user can utilize the Internet to realize the convenient, swift visit and manipulation on the database.

  3. Factors Associated with Clinical and Topographical Features of Laryngeal Tuberculosis.

    Directory of Open Access Journals (Sweden)

    João Gustavo Corrêa Reis

    Full Text Available Laryngeal tuberculosis (LTB is the most frequent granulomatous disease of the larynx and represents less than 2% of extrapulmonary TB cases. There are no pathognomonic clinical and endoscopic features of this disease and studies on LTB that can assist in its diagnostic characterization are lacking.To identify factors associated with clinical and topographical features of LTB.a retrospective cross-sectional study was conducted from the medical records of 36 patients with confirmed LTB diagnosis.Dysphonia and cough were the main symptoms presented by patients and the true vocal folds the most frequently affected site. The average of the duration of the disease evolution was significantly higher in patients with dysphonia than in patients without this symptom. We observed association between dysphonia and true vocal fold lesions and between odynophagia and lesions in the epiglottis, arytenoids and aryepiglottic folds. Odynophagia was more frequent in individuals with lesions in four or more laryngeal sites. Weight loss equal or above 10% of the body weight was more frequent in patients with odynophagia as first symptom and in patients with ulcerated lesion. Dyspnea on exertion was more frequent in individuals with more extensive laryngeal lesions. The percentage of smokers with lesions in four or more laryngeal sites was greater than that found in non-smokers. Laryngeal tissue fragment bacilloscopy and culture examinations were less positive than sputum ones.Smoking appears to be associated with the development of more extensive LTB lesions, and LTB with dyspnea on exertion and odynophagia with consequent impairment of nutritional status. We emphasize the need for histopathologic confirmation, once positive sputum bacteriological examinations seem not to necessarily reflect laryngeal involvement.

  4. Factors Associated with Clinical and Topographical Features of Laryngeal Tuberculosis.

    Science.gov (United States)

    Reis, João Gustavo Corrêa; Reis, Clarissa Souza Mota; da Costa, Daniel César Silva; Lucena, Márcia Mendonça; Schubach, Armando de Oliveira; Oliveira, Raquel de Vasconcellos Carvalhaes; Rolla, Valéria Cavalcanti; Conceição-Silva, Fátima; Valete-Rosalino, Cláudia Maria

    2016-01-01

    Laryngeal tuberculosis (LTB) is the most frequent granulomatous disease of the larynx and represents less than 2% of extrapulmonary TB cases. There are no pathognomonic clinical and endoscopic features of this disease and studies on LTB that can assist in its diagnostic characterization are lacking. To identify factors associated with clinical and topographical features of LTB. a retrospective cross-sectional study was conducted from the medical records of 36 patients with confirmed LTB diagnosis. Dysphonia and cough were the main symptoms presented by patients and the true vocal folds the most frequently affected site. The average of the duration of the disease evolution was significantly higher in patients with dysphonia than in patients without this symptom. We observed association between dysphonia and true vocal fold lesions and between odynophagia and lesions in the epiglottis, arytenoids and aryepiglottic folds. Odynophagia was more frequent in individuals with lesions in four or more laryngeal sites. Weight loss equal or above 10% of the body weight was more frequent in patients with odynophagia as first symptom and in patients with ulcerated lesion. Dyspnea on exertion was more frequent in individuals with more extensive laryngeal lesions. The percentage of smokers with lesions in four or more laryngeal sites was greater than that found in non-smokers. Laryngeal tissue fragment bacilloscopy and culture examinations were less positive than sputum ones. Smoking appears to be associated with the development of more extensive LTB lesions, and LTB with dyspnea on exertion and odynophagia with consequent impairment of nutritional status. We emphasize the need for histopathologic confirmation, once positive sputum bacteriological examinations seem not to necessarily reflect laryngeal involvement.

  5. SWALLOWING IN PATIENTS WITH LARYNGITIS

    Directory of Open Access Journals (Sweden)

    Isabela MODA

    Full Text Available ABSTRACT BACKGROUND: Dysphagia is described as a complaint in 32% of patients with laryngitis. OBJECTIVE: The objective of this investigation was to evaluate oral and pharyngeal transit of patients with laryngitis, with the hypothesis that alteration in oral-pharyngeal bolus transit may be involved with dysphagia. METHODS: Videofluoroscopic evaluation of the swallowing of liquid, paste and solid boluses was performed in 21 patients with laryngitis, 10 of them with dysphagia, and 21 normal volunteers of the same age and sex. Two swallows of 5 mL liquid bolus, two swallows of 5 mL paste bolus and two swallows of a solid bolus were evaluated in a random sequence. The liquid bolus was 100% liquid barium sulfate and the paste bolus was prepared with 50 mL of liquid barium and 4 g of food thickener (starch and maltodextrin. The solid bolus was a soft 2.2 g cookie coated with liquid barium. Durations of oral preparation, oral transit, pharyngeal transit, pharyngeal clearance, upper esophageal sphincter opening, hyoid movement and oral-pharyngeal transit were measured. All patients performed 24-hour distal esophageal pH evaluation previous to videofluoroscopy. RESULTS: The evaluation of 24-hour distal esophageal pH showed abnormal gastroesophageal acid reflux in 10 patients. Patients showed longer oral preparation for paste bolus and a faster oral transit time for solid bolus than normal volunteers. Patients with laryngitis and dysphagia had longer preparation for paste and solid boluses, and a faster oral transit time with liquid, paste and solid boluses. CONCLUSION: A longer oral preparation for paste and solid boluses and a faster transit through the mouth are associated with dysphagia in patients with laryngitis.

  6. SWALLOWING IN PATIENTS WITH LARYNGITIS.

    Science.gov (United States)

    Moda, Isabela; Ricz, Hilton Marcos Alves; Aguiar-Ricz, Lilian Neto; Dantas, Roberto Oliveira

    2018-01-01

    Dysphagia is described as a complaint in 32% of patients with laryngitis. The objective of this investigation was to evaluate oral and pharyngeal transit of patients with laryngitis, with the hypothesis that alteration in oral-pharyngeal bolus transit may be involved with dysphagia. Videofluoroscopic evaluation of the swallowing of liquid, paste and solid boluses was performed in 21 patients with laryngitis, 10 of them with dysphagia, and 21 normal volunteers of the same age and sex. Two swallows of 5 mL liquid bolus, two swallows of 5 mL paste bolus and two swallows of a solid bolus were evaluated in a random sequence. The liquid bolus was 100% liquid barium sulfate and the paste bolus was prepared with 50 mL of liquid barium and 4 g of food thickener (starch and maltodextrin). The solid bolus was a soft 2.2 g cookie coated with liquid barium. Durations of oral preparation, oral transit, pharyngeal transit, pharyngeal clearance, upper esophageal sphincter opening, hyoid movement and oral-pharyngeal transit were measured. All patients performed 24-hour distal esophageal pH evaluation previous to videofluoroscopy. The evaluation of 24-hour distal esophageal pH showed abnormal gastroesophageal acid reflux in 10 patients. Patients showed longer oral preparation for paste bolus and a faster oral transit time for solid bolus than normal volunteers. Patients with laryngitis and dysphagia had longer preparation for paste and solid boluses, and a faster oral transit time with liquid, paste and solid boluses. A longer oral preparation for paste and solid boluses and a faster transit through the mouth are associated with dysphagia in patients with laryngitis.

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

    Science.gov (United States)

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

    2018-05-08

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

  8. T2 laryngeal cancer study in our department

    International Nuclear Information System (INIS)

    Ikenoya, Yoichi; Shimane, Toshikazu; Kobayashi, Sei

    2011-01-01

    Laryngeal cancer is the most common malignant tumor in the head and neck region. Because early detection and treatment are possible, outcomes are relatively good. Many studies have reported on the treatment of laryngeal cancer. Different hospitals have used generally similar treatment regimens. However, factors such as laryngeal preservation and the treatment of choice for patients with T2 laryngeal cancer still differ among hospitals. Survival rates can be increased depending on treatment, sometimes at the cost of losing voice functions that could have been preserved. In our department, we have emphasized curative treatment and the preservation of organs and functions. We have mainly used chemoradiotherapy concurrently with S-1 and nedaplatin for the treatment of T2 laryngeal cancer. We studied 27 patients (23 men and 4 women) with T2 laryngeal cancer, who received first-line therapy in our department from April 2005 through March 2010. Their mean age was 64.1 years (range, 42 to 80). The mean follow-up period was 30.6 months (range, 2 to 60 months). The tumor-node-metastasis classification was T2N0M0 in 24 patients, T2N1M0 in 1, and T2N2bM0 in 2.In our department, the disease-specific survival rate was 96.3%. The complete response rate was 88.9%, and the laryngeal preservation rate was 92.6%. (author)

  9. Laryngeal Cysts in Adults: Simplifying Classification and Management.

    Science.gov (United States)

    Heyes, Richard; Lott, David G

    2017-12-01

    Objective Laryngeal cysts may occur at any mucosa-lined location within the larynx and account for 5% to 10% of nonmalignant laryngeal lesions. A number of proposed classifications for laryngeal cysts exist; however, no previously published classification aims to guide management. This review analyzes contemporary laryngeal cyst management and proposes a framework for the terminology and management of cystic lesions in the larynx. Data Sources PubMed/Medline. Review Methods A primary literature search of the entire Medline database was performed for all titles of publications pertaining to laryngeal cysts and reviewed for relevance. Full manuscripts were reviewed per the relevance of their titles and abstracts, and selection into this review was according to their clinical and scientific relevance. Conclusion Laryngeal cysts have been associated with rapid-onset epiglottitis, dyspnea, stridor, and death; therefore, they should not be considered of little significance. Symptoms are varied and nonspecific. Laryngoscopy is the primary initial diagnostic tool. Cross-sectional imaging may be required, and future use of endolaryngeal ultrasound and optical coherence tomography may revolutionize practice. Where possible, cysts should be completely excised, and there is growing evidence that a transoral approach is superior to transcervical excision for nearly all cysts. Histology provides definitive diagnosis, and oncocytic cysts require close follow-up. Implications for Practice A new classification system is proposed that increases clarity in terminology, with the aim of better preparing surgeons and authors for future advances in the understanding and management of laryngeal cysts.

  10. Laryngeal neurinoma. Differential diagnosis of submucosal laryngeal tumors; Neurinoma laringeo. Diagnostico diferencial de tumoraciones submucosas laringeas

    Energy Technology Data Exchange (ETDEWEB)

    Higuera, A.; Palomo, V.; Munoz, R.; Sanchez, F.

    2002-07-01

    Laryngeal neurinoma is a rare benign tumor that appears as a submucosal mass, generally in the supraglottic region. We report the case of a patient with dysphonia of long evolution caused by a neurinoma. We discuss the radiological findings of the tumor and the value of computed tomography (CT) in the diagnosis of this and other submucosal laryngeal lesions. (Author) 16 refs.

  11. Paediatric laryngeal granular cell tumour

    Directory of Open Access Journals (Sweden)

    Dauda Ayuba

    2009-01-01

    Full Text Available Granular cell tumour (GCT affecting the larynx is not common, especially in children. Most cases are apt to be confused with respiratory papilloma and may even be mistaken for a malignant neoplasia. We present a case of laryngeal GCT in a 12-year-old child to emphasize that the tumour should be regarded in the differential of growths affecting the larynx in children.

  12. Risk factors for laryngeal trauma and granuloma formation in pediatric intubations.

    Science.gov (United States)

    Jang, Minyoung; Basa, Krystyne; Levi, Jessica

    2018-04-01

    Intubation has been associated with laryngeal injury that often resolves spontaneously without complication. We present a case of a child intubated for less than 48 hours, who presented with dysphonia and intermittent dyspnea two months after intubation due to epiglottic and vocal process granulomas. This is unusual in that multiple granulomas were found in the posterior glottis and supraglottis after short-term intubation. Our objective was to determine if there are risk factors for developing persistent post-intubation sequelae, including the delayed presentation and unusual location of post-intubation granulomas in our case. Case report and systematic literature review. Pubmed database, which is inclusive of MEDLINE, was used to perform a literature review with the search terms ((pediatric OR children OR neonatal OR infant) AND (laryngeal OR supraglottic) AND intubation AND (granuloma OR injury OR complication)). Only English language results were reviewed. Titles and abstracts from 379 results were reviewed. Full text was reviewed from all original studies which included human pediatric subjects and endoscopic examinations after endotracheal intubation. In our case, laryngeal granuloma size reduced significantly after starting anti-reflux medications. The remainder was removed with laryngeal microdebrider with no recurrence at 3 weeks and 2.5 years post-operatively. Overall, 28 of the 379 studies reviewed identified evidence of laryngeal trauma due to intubation, however only 6 studies documented any type of supraglottic injury. Risk factors identified for developing post-intubation sequelae included intubation duration greater than 24 h; trauma to the larynx via various mechanisms including traumatic intubation, need for reintubation and tube changes, and increased movement while intubated; and presence of respiratory tract infection during intubation. Trauma to the larynx during intubation should be avoided to minimize post-intubation injury in pediatric

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

    Science.gov (United States)

    Tamura, Hiroaki; Hasegawa, Kiminori

    2009-02-01

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

  14. Case study: lessons from a laryngeal abscess

    African Journals Online (AJOL)

    pathology is explored, as well as the diagnostic error that could have resulted in serious complications. Keywords: asthma, error, laryngeal abscess, squamous carcinoma. Introduction .... tified or cultured and syphilis serology was normal. The laryngeal biopsies confirmed a well-differentiated keratinising squamous.

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

    Science.gov (United States)

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

    1988-01-01

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

  16. 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.)

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

    Science.gov (United States)

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

    2014-06-01

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

  18. 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

  19. Ionising rays and laryngeal carcinomas

    International Nuclear Information System (INIS)

    Martin, G.; Glanz, H.; Kleinsasser, O.

    1979-01-01

    Review of the literature and report of a new case of laryngeal cancer after irradiation of a benign lesion of the neck. These cases obviously become rare since benign lesions are no longer irradiated. Today the risk of inducing a second carcinoma by a successful irradiation of the first tumor becomes more important. A study of 109 patients, irradiated for laryngeal carcinoma and surviving with no evidence of disease for a period of at least 5 years has been performed. 8 of these patients developed a second primary in the previously irradiated area after 7-15 years. These second carcinomas are not rare if one considers that most patients with laryngeal carcinoma are 60-70 years old and therefore the life expectance on an average is low. These facts should be taken into consideration when deciding between surgical or radiation therapy in younger patients with high life expectance. (orig.) [de

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

    Science.gov (United States)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2012-07-01

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

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

    Science.gov (United States)

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

    2016-09-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

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

    2014-01-01

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

  7. Clinical manifestation of Laryngeal Tuberculosis | Abdalla | Sudan ...

    African Journals Online (AJOL)

    All tuberculous patients with laryngeal symptoms and those diagnosed histologically to have laryngeal tuberculosis were included. Results: Eight patients were studied; they were five males and three females, with age range between 12-70 years (mean 41years). Strider, dysphonia and dysphagia were the main complaints.

  8. Multidisciplinary Management of Laryngeal Carcinoma

    International Nuclear Information System (INIS)

    Mendenhall, William M.; Mancuso, Anthony A.; Hinerman, Russell W.; Malyapa, Robert S.; Werning, John W.; Amdur, Robert J.; Villaret, Douglas B.

    2007-01-01

    The management of head and neck cancer has evolved into a multidisciplinary approach in which patients are evaluated before treatment and decisions depend on prospective multi-institutional trials, as well as retrospective outcome studies. The choice of one or more modalities to use in a given case varies with the tumor site and extent, as exemplified in the treatment of laryngeal squamous cell carcinomas. The goals of treatment include cure, laryngeal voice preservation, voice quality, optimal swallowing, and minimal xerostomia. Treatment options include transoral laser excision, radiotherapy (both definitive and postoperative), open partial laryngectomy, total laryngectomy, and neck dissection. The likelihood of local control and preservation of laryngeal function is related to tumor volume. Patients who have a relatively high risk of local recurrence undergo follow-up computed tomography scans every 3-4 months for the first 2 years after radiotherapy. Patients with suspicious findings on computed tomography might benefit from fluorodeoxyglucose positron emission tomography to differentiate post-radiotherapy changes from tumor

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

    Science.gov (United States)

    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.

  10. Surgical management of macroglossia secondary to amyloidosis.

    Science.gov (United States)

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

    2016-04-11

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

  11. Expression of Podoplanin in Laryngeal Squamous Cell Carcinoma and Dysplasia.

    Science.gov (United States)

    Ibrahim, Badawia Bayoumy; Salem, Mostafa Mohamed; Khairy, Rasha Ahmed; Al Gunaid, Reema Abdul Rahman

    2017-05-01

    In human cancers, podoplanin expression and its correlation with tumour invasive potential raise its possible role as a diagnostic and prognostic marker for cancer. To investigate the immunohistochemical expression of podoplanin in laryngeal Squamous Cell Carcinoma (SCC) and dysplasia. This study included a total of 60 archived, formalin fixed, paraffin embedded tissue blocks of 40 cases of laryngeal SCC and 20 cases of dysplastic lesions. The samples were immunohistochemically analysed for podoplanin expression. Podoplanin expression was significantly higher in laryngeal SCC (90%) than laryngeal dysplastic lesions (55%) (p-value=0.002). The expression of podoplanin was significantly increased with the higher grades of dysplasia (p-value=0.016). A significant positive correlation was detected between podoplanin expression in laryngeal SCC and depth of tumour invasion (p-value=0.035), and stage (p-value=0.026). The high expression of podoplanin in laryngeal SCC and its significant correlation with poor prognostic parameters recommends podoplanin as a prognostic marker in laryngeal SCC. In addition, increased podoplanin expression with higher grades of dysplasia, supports its role in malignant transformation and allows us to recommend its evaluation in premalignant lesions.

  12. [Recurrent laryngeal papillomatosis complicated by decompensated respiratory failure in two children].

    Science.gov (United States)

    Aurégan, C; Thierry, B; Blanchard, M; Chéron, G

    2015-11-01

    Laryngeal papillomatosis is a rare, potentially severe cause of recurrent laryngeal dyspnea. It should be known as a cause of laryngeal dyspnea resistant to the usual treatments. We report on two pediatric cases of severe laryngeal papillomatosis with respiratory distress and failure. These observations illustrate the importance of early adequate treatment. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  13. Variable Origin of the Superior Laryngeal Artery and Its Clinical Significance

    OpenAIRE

    Soubhagya R. Nayak1*, Ashwin Krishnamurthy2, Latha V. Prabhu2, Bhagath Kumar Potu3, Ishwar B. Bagoji4, Jiji PJ2 and Ganesh Kumar Chettiar2

    2011-01-01

    The superior laryngeal artery (SLA) is the dominant arterial supply of the laryngeal muscles, mucosa and glands. The purpose of the present study was to document the variable origin of the SLA in the carotid triangle. Although the variation in the SLA origin and morphology is important during the partial laryngectomy and reconstruction surgery of the larynx, the description of the SLA in modern literature is vague. The anatomy of SLA was studied in 37 adult South Indian preserved cadavers age...

  14. Laryngeal ultrasound and pediatric vocal fold nodules.

    Science.gov (United States)

    Ongkasuwan, Julina; Devore, Danielle; Hollas, Sarah; Jones, Jeremy; Tran, Brandon

    2017-03-01

    The term vocal fold nodules refers to bilateral thickening of the membranous folds with minimal impairment of the vibratory properties of the mucosa. Nodules are thought to be related to repetitive mechanical stress, associated with voice use patterns. Diagnosis is typically made in the office via either rigid or flexible laryngeal stroboscopy. Depending on the individual child, obtaining an optimal view of the larynx can be difficult if not impossible. Recent advances in high-frequency ultrasonography allows for transcervical examination of laryngeal structures. The goal of this project was to determine if laryngeal ultrasound (LUS) can be used to identify vocal fold nodules in dysphonic children. Prospective case-control study in which the patient acted as his or her own control. Forty-six pediatric patients were recruited for participation in this study; the mean age was 4.8 years. Twenty-three did not have any vocal fold lesions and 23 had a diagnosis of vocal fold nodules on laryngeal stroboscopy. Recorded LUSs were reviewed by two pediatric radiologists who were blinded to the nodule status. There was substantial inter-rater agreement (κ = 0.70, 95% confidence interval [CI]: 0.50-0.89) between the two radiologists regarding the presence of nodules. There was also substantial agreement (κ = 0.87, 95% CI: 0.72-1) between LUS and laryngeal stroboscopy. Sensitivity of LUS was 100% (95% CI: 85%-100%) and specificity was 87% (95% CI: 66%-97%). LUS can be used to identify vocal fold nodules in children with substantial agreement with laryngeal stroboscopy. 3b Laryngoscope, 127:676-678, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  15. [Current status and prospect of photodynamic therapy in laryngeal diseases].

    Science.gov (United States)

    Zhang, C; Jiang, J Q

    2018-04-07

    Laryngeal diseases are closely related to the swallowing and speech function of the patients.Protecting and restoring laryngeal function, while curing lesions, is vital to patients' quality of life.Photodynamic therapy (PDT) is a minimally invasive method which is widely used in the treatment of tumor, precancerous lesions, and inflammatory diseases.In recent years, it has been shown to have a protective effect on normal structures. This article reviews the clinical outcomes of laryngeal diseases treated with PDT since 1990 in order to evaluate its efficacy and significance. The complete remission rate of early-stage laryngeal tumors and precancerous lesions after PDT is 77.6%(249/321), and a promising effect on recurrent laryngeal papillomatosis has been observed thus far. The prolonged adverse effects of the first-generation photosensitizers have limited the application of PDT. With the improvement of photosensitizers and treatment strategies, PDT promises to be a safe, effective, and minimally invasive treatment method for laryngeal diseases.

  16. Swallowing assessment in early laryngeal cancer patients treated either with surgery or radiotherapy

    International Nuclear Information System (INIS)

    Celedon L, Carlos; Gambi A, Galo; Royer F, Michel; Esquivel C, Patricia; Arteaga J, Patricia; Valdes P, Constanza

    2008-01-01

    Swallowing is a complex neuromuscular process that requires anatomical indemnity and an adequate coordination of several organs. Laryngeal cancer treatment may cause swallowing disorders. Traditionally, a high frequency of this type of disorder after surgery has been reported, but no actual data concerning its incidence in patients undergoing radiotherapy for early laryngeal cancer has been published. Aim. To compare swallowing disorders frequency posterior to treatment in early laryngeal cancer patients. Material and Method. Two groups of early laryngeal cancer patients were transversally studied, one treated with vertical partial surgery (CP), and the other treated exclusively with radiotherapy. Each patient had otorhinolaryngological, nasofibroscopic and video fluoroscopic evaluations after treatment. Differences between groups were compared using the - square test. Results. Twenty patients per group were entered in this study, predominantly males of similar age. Both groups presented a high incidence of aspiration symptoms (55% in RT and 35% in CP). There were no significant differences between both groups. Discussion and Conclusion. A high incidence of swallowing disorders in patients treated for early laryngeal cancer was found. It should then be considered as a frequent alteration in this group of patients, either treated with RT or CP

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

    Directory of Open Access Journals (Sweden)

    Erkan Dervisoglu

    2015-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    International Nuclear Information System (INIS)

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

    1990-01-01

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

  20. Correction: Inferior alveolar nerve injury with laryngeal mask airway: a case report.

    LENUS (Irish Health Repository)

    Hanumanthaiah, Deepak

    2011-11-30

    ABSTRACT: Following the publication of our article [Inferior alveolar nerve injury with laryngeal mask airway: a case report. Journal of Medical Case Reports 2011, 5:122] it was brought to our attention that we inadvertently used the registered trademark of the Laryngeal Mask Company Limited (LMA) as the abbreviation for laryngeal mask airway. A Portex(R) Soft Seal(R) Laryngeal Mask was used and not a device manufactured by the Laryngeal Mask Company.

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

    DEFF Research Database (Denmark)

    Müller, Hanna; Renner, Marcus; Bergmann, Frank

    2013-01-01

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

  2. External laryngeal manipulation done by the laryngoscopist makes the best laryngeal view for intubation

    Directory of Open Access Journals (Sweden)

    Mohamed Shaaban Ali

    2014-01-01

    Full Text Available Purpose: External laryngeal manipulation (ELM is used to get better laryngeal view during direct laryngoscopy. This study was designed to test the hypothesis that ELM done by the intubating anesthetist (laryngoscopist offers the best laryngeal view for tracheal intubation. Materials and method: A total of 160 patients underwent different surgical procedures were included in this study. Percentage of glottic opening (POGO score and Cormack and Lehane scale were used as outcome measures for comparison between different laryngoscopic views. Four views were described; basic laryngoscopic view and then views after ELM done by the assistant, by the laryngoscopist and finally by the assistant after the guidance from the laryngoscopist respectively. The last three views compared with the basic laryngoscopic view. Results: ELM done by the laryngoscopist or by the assistant after guidance from the laryngoscopist showed significant improvement of Cormack grades and POGO scores compared with basic laryngoscopic view. Number of patients with Cormack grade1 increased from 39 after direct laryngoscopy to 97 and 96 patients (P < 0.001 by Fisher′s exact test, after ELM done by the laryngoscopist and that done by the assistant after guidance from the anesthetist respectively. Furthermore, the number of patients with POGO scores of 100% increased from 39 after direct laryngoscopy to 78 and 61 (P < 0.01 patients after ELM done by the laryngoscopist and that done by the assistant after guidance from the anesthetist respectively. Conclusion: It appeared from this study that ELM done by the anesthetist makes the best laryngeal view for tracheal intubation.

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

    International Nuclear Information System (INIS)

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

    1979-01-01

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

  4. A case of vogt-koyanagi-harada syndrome with persistent dyspnea secondary to laryngeal edema.

    Science.gov (United States)

    Mantopoulos, Dimosthenis; deSilva, Brad W; Cebulla, Colleen M

    2014-01-01

    We report a case of laryngeal edema associated with the Vogt-Koyanagi-Harada (VKH) syndrome. A 32-year-old African-American female presented with a 12-day prodrome, including headache, tinnitus and shortness of breath, which preceded sudden photophobia and bilateral visual loss. Examination and clinical testing were most consistent with VKH, and the patient improved with intravenous methylprednisolone therapy. The patient had persistent dyspnea, which was out of proportion to chest CT findings and which was exacerbated during a recurrence of VKH. Flexible fiberoptic laryngoscopy with stroboscopy revealed diffuse laryngeal edema. Symptoms were alleviated with breathing exercises. Several autoimmune diseases may cause diffuse laryngeal edema. In this case, VKH was associated with the patient's glottic edema and dyspnea. We recommend that laryngeal edema be considered in the differential diagnosis for patients with dyspnea and VKH.

  5. Failed tracheal intubation using a laryngoscope and intubating laryngeal mask.

    Science.gov (United States)

    Asai, T; Hirose, T; Shingu, K

    2000-04-01

    To report unexpected failed tracheal intubation using a laryngoscope and an intubating laryngeal mask, and difficult ventilation via a facemask, laryngeal mask and intubating laryngeal mask, in a patient with an unrecognized lingual tonsillar hypertrophy. A 63-yr-old woman, who had undergone clipping of an aneurysm seven weeks previously, was scheduled for ventriculo-peritoneal shunt. At the previous surgery, there had been no difficulty in ventilation or in tracheal intubation. Her trachea remained intubated nasally for 11 days after surgery. Preoperatively, her consciousness was impaired. There were no restrictions in head and neck movements or mouth opening. The thyromental distance was 7 cm. After induction of anesthesia, manual ventilation via a facemask with a Guedel airway was suboptimal and the chest expanded insufficiently. At laryngoscopy using a Macintosh or McCoy device, only the tip of the epiglottis, but not the glottis, could be seen, and tracheal intubation failed. There was a partial obstruction during manual ventilation through either the intubating laryngeal mask or conventional laryngeal mask; intubation through each device failed. Digital examination of the pharynx, after removal of the laryngeal mask, indicated a mass occupying the vallecula. Lingual tonsillar hypertrophy (1 x 1 x 2 cm) was found to be the cause of the failure. Awake fibrescope-aided tracheal intubation was accomplished. Unexpected lingual tonsillar hypertrophy can cause both ventilation and tracheal intubation difficult, and neither the laryngeal mask nor intubating laryngeal mask may be helpful in the circumstances.

  6. Laryngeal paralysis in dogs: An update on recent knowledge

    Directory of Open Access Journals (Sweden)

    Adriaan M. Kitshoff

    2013-04-01

    Full Text Available Laryngeal paralysis is the effect of an inability to abduct the arytenoid cartilages during inspiration, resulting in respiratory signs consistent with partial airway obstruction. The aetiology of the disease can be congenital (hereditary laryngeal paralysis or congenital polyneuropathy, or acquired (trauma, neoplasia, polyneuropathy, endocrinopathy. The most common form of acquired laryngeal paralysis (LP is typically seen in old, large breed dogs and is a clinical manifestation of a generalised peripheral polyneuropathy recently referred to as geriatric onset laryngeal paralysis polyneuropathy. Diagnosing LP based on clinical signs, breed and history has a very high sensitivity (90% and can be confirmed by laryngeal inspection. Prognosis after surgical correction depends on the aetiology: traumatic cases have a good prognosis, whereas tumour-induced or polyneuropathy-induced LP has a guarded prognosis. Acquired idiopathic LP is a slow progressive disease, with dogs reaching median survival times of 3–5 years after surgical correction.

  7. [Hypothyroidism incidence after multimodal treatment for laryngeal cancer].

    Science.gov (United States)

    Ortega-Gutiérrez, César; Luna-Ortiz, Kuauhyama; Villavicencio-Valencia, Verónica; Herrera Gómez, Angel; Téllez-Palacios, Daniela; Contreras-Buendía, Marlen

    2012-01-01

    Hypothyroidism following total laryngectomy or radiotherapy treatment for laryngeal cancer is not a rare event, especially in advanced stages. There are no reports on the incidence of hypothyroidism in patients who received chemotherapy and radiotherapy. The objective of this study is to determine the incidence of thyroid dysfunction in a group of patients with laryngeal cancer who underwent surgery as sole treatment, total laryngectomy or radiotherapy alone, and patients with combined treatment: surgery plus radiotherapy, concomitant chemoradiation therapy and chemoradiation therapy plus salvage surgery. A prospective study of patients diagnosed with laryngeal cancer whose serum TSH and T4 levels were evaluated in a serial fashion. 70 patients with laryngeal cancer were studied; the average age at diagnosis was 70.2 years. Male patients were more affected, with a men-women ratio of 3.6:1. Glottic localization was the most frequent (44%). 64% of tumors were locally advanced carcinomas and 51% received multimodal treatment. 45 patients (63%) were diagnosed with hypothyroidism; 49% of the patients with subclinical hypothyroidism, and 51% with clinical hypothyroidism. Hypothyroidism is a complication following treatment for laryngeal cancer. It is recommended to evaluate the thyroid function periodically for timely detection.

  8. Metastasis of Laryngeal Squamous Cell Carcinoma to Bilateral Thigh Muscles

    Directory of Open Access Journals (Sweden)

    Zarah Lucas

    2014-01-01

    Full Text Available Importance. Laryngeal cancer infrequently results in distant metastases, but metastasis to skeletal muscle is extremely uncommon. Observations. A 55-year-old male presenting with progressive dyspnea and hoarseness was found to have Stage IVA T4aN2cM0 laryngeal cancer and eventually underwent total laryngectomy. Before the patient could be started on adjuvant chemoradiation, the patient developed masses on both thighs. Biopsy revealed metastatic squamous cell carcinoma consistent with the primary laryngeal cancer. He was offered palliative chemotherapy; however, he developed new soft tissue masses to the left of his stoma and in the prevertebral area one week later. He also had new cervical and supraclavicular nodes and a pathological compression fracture of L3. Patient died within 4 months of diagnosis. Conclusions. Distant metastasis such as skeletal metastasis portends a poor prognosis. Further studies are required to determine the best course of treatment in these patients.

  9. Computer tomography in complex diagnosis of laryngeal cancer

    International Nuclear Information System (INIS)

    Savin, A.A.

    1999-01-01

    To specify the role of computer tomography in the diagnosis of malignant of the larynx. Forty-two patients with suspected laryngeal tumors were examined: 38 men and 4 women aged 41-68 years. X-ray examinations included traditional immediate tomography of the larynx. Main X-ray and computer tomographic symptoms of laryngeal tumors of different localizations are described. It is shown that the use of computer tomography in complex diagnosis of laryngeal cancer permits an objective assessment of the tumor, its structure and dissemination, and of the regional lymph nodes [ru

  10. Dysphagia and laryngeal pathology in post-surgical cardiothoracic patients.

    Science.gov (United States)

    Miles, Anna; McLellan, Naomi; Machan, Rochelle; Vokes, David; Hunting, Alexandra; McFarlane, Mary; Holmes, Jennifer; Lynn, Kelly

    2018-02-09

    Cardiothoracic surgery is known to result in dysphagia and laryngeal injury. While prevalence has been explored, extent, trajectory and longevity of symptoms are poorly understood. This retrospective, observational study explored dysphagia and laryngeal injury in patients following cardiothoracic surgery referred for instrumental swallowing assessment. Clinical notes and endoscopic recordings of 106 patients (age range 18-87yrs; mean 63yrs; SD 15yrs) (including 190 endoscopes) at one large tertiary centre were reviewed by two speech-language pathologists and a laryngologist. Standardized measures of laryngeal anatomy and physiology, New Zealand Secretion Scale, Penetration-Aspiration scale and Yale Residue Scale were rated. Prevalence of abnormality included 39% silent aspiration, 65% laryngeal edema and 61% vocal paralysis. The incidence of pneumonia was 36% with a post-operative stroke rate of 14%. Forty percent of patients were receiving a standard diet by discharge from acute care; while, 24% continued to require enteral feeding and 8% received laryngeal surgery within twelve months of discharge. Vocal fold motion impairment was significantly associated with ventilation time and tracheostomy tube duration (pdysphagia and laryngeal injury in patients following cardiothoracic surgery may allow early management and prevention of secondary complications. Copyright © 2018 Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

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

    2003-01-01

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

  14. Immunological responses against human papilloma virus and human papilloma virus induced laryngeal cancer.

    Science.gov (United States)

    Chitose, Shun-ichi; Sakazaki, T; Ono, T; Kurita, T; Mihashi, H; Nakashima, T

    2010-06-01

    This study aimed to clarify the local immune status in the larynx in the presence of infection or carcinogenesis associated with human papilloma virus. Cytological samples (for human papilloma virus detection) and laryngeal secretions (for immunoglobulin assessment) were obtained from 31 patients with laryngeal disease, during microscopic laryngeal surgery. On histological examination, 12 patients had squamous cell carcinoma, four had laryngeal papilloma and 15 had other benign laryngeal disease. Cytological samples were tested for human papilloma virus DNA using the Hybrid Capture 2 assay. High risk human papilloma virus DNA was detected in 25 per cent of patients (three of 12) with laryngeal cancer. Low risk human papilloma virus DNA was detected only in three laryngeal papilloma patients. The mean laryngeal secretion concentrations of immunoglobulins M, G and A and secretory immunoglobulin A in human papilloma virus DNA positive patients were more than twice those in human papilloma virus DNA negative patients. A statistically significant difference was observed between the secretory immunoglobulin A concentrations in the two groups. Patients with laryngeal cancer had higher laryngeal secretion concentrations of each immunoglobulin type, compared with patients with benign laryngeal disease. The study assessed the mean laryngeal secretion concentrations of each immunoglobulin type in the 12 laryngeal cancer patients, comparing human papilloma virus DNA positive patients (n = 3) and human papilloma virus DNA negative patients (n = 9); the mean concentrations of immunoglobulins M, G and A and secretory immunoglobulin A tended to be greater in human papilloma virus DNA positive cancer patients, compared with human papilloma virus DNA negative cancer patients. These results suggest that the local laryngeal immune response is activated by infection or carcinogenesis due to human papilloma virus. The findings strongly suggest that secretory IgA has inhibitory activity

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

    Science.gov (United States)

    2018-05-01

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

  16. Leishmaniasis isoleret til larynx som årsag til kronisk laryngitis

    DEFF Research Database (Denmark)

    Kaltoft, Mikkel; Munch-Petersen, Helga Richert; Møller, Henrik

    2010-01-01

    Mucosal leishmaniasis is uncommon outside Central and South America, where it is commonly caused by Leishmania (L.) braziliensis. We present a case of isolated laryngeal leishmaniasis detected in a 78-year-old male, who presented with chronic hoarseness. Histologic examination of biopsies taken...

  17. A Case of Vogt-Koyanagi-Harada Syndrome with Persistent Dyspnea Secondary to Laryngeal Edema

    Directory of Open Access Journals (Sweden)

    Dimosthenis Mantopoulos

    2014-11-01

    Full Text Available Purpose: We report a case of laryngeal edema associated with the Vogt-Koyanagi-Harada (VKH syndrome. Patient and Methods: A 32-year-old African-American female presented with a 12-day prodrome, including headache, tinnitus and shortness of breath, which preceded sudden photophobia and bilateral visual loss. Examination and clinical testing were most consistent with VKH, and the patient improved with intravenous methylprednisolone therapy. Results: The patient had persistent dyspnea, which was out of proportion to chest CT findings and which was exacerbated during a recurrence of VKH. Flexible fiberoptic laryngoscopy with stroboscopy revealed diffuse laryngeal edema. Symptoms were alleviated with breathing exercises. Conclusions: Several autoimmune diseases may cause diffuse laryngeal edema. In this case, VKH was associated with the patient's glottic edema and dyspnea. We recommend that laryngeal edema be considered in the differential diagnosis for patients with dyspnea and VKH.

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

    Directory of Open Access Journals (Sweden)

    Masaru Matsui

    2014-01-01

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

  19. Inducible laryngeal obstruction

    DEFF Research Database (Denmark)

    Halvorsen, Thomas; Walsted, Emil Schwarz; Bucca, Caterina

    2017-01-01

    Inducible laryngeal obstruction (ILO) describes an inappropriate, transient, reversible narrowing of the larynx in response to external triggers. ILO is an important cause of a variety of respiratory symptoms and can mimic asthma. Current understanding of ILO has been hampered by imprecise nomenc...

  20. A new classification system for congenital laryngeal cysts.

    Science.gov (United States)

    Forte, Vito; Fuoco, Gabriel; James, Adrian

    2004-06-01

    A new classification system for congenital laryngeal cysts based on the extent of the cyst and on the embryologic tissue of origin is proposed. Retrospective chart review. The charts of 20 patients with either congenital or acquired laryngeal cysts that were treated surgically between 1987 and 2002 at the Hospital for Sick Children, Toronto were retrospectively reviewed. Clinical presentation, radiologic findings, surgical management, histopathology, and outcome were recorded. A new classification system is proposed to better appreciate the origin of these cysts and to guide in their successful surgical management. Fourteen of the supraglottic and subglottic simple mucous retention cysts posed no diagnostic or therapeutic challenge and were treated successfully by a single endoscopic excision or marsupialization. The remaining six patients with congenital cysts in the study were deemed more complex, and all required open surgical procedures for cure. On the basis of the analysis of the data of these patients, a new classification of congenital laryngeal cysts is proposed. Type I cysts are confined to the larynx, the cyst wall composed of endodermal elements only, and can be managed endoscopically. Type II cysts extend beyond the confines of the larynx and require an external approach. The Type II cysts are further subclassified histologically on the basis of the embryologic tissue of origin: IIa, composed of endoderm only and IIb, containing endodermal and mesodermal elements (epithelium and cartilage) in the wall of the cyst. A new classification system for congenital laryngeal cysts is proposed on the basis of the extent of the cyst and the embryologic tissue of origin. This classification can help guide the surgeon with initial management and help us better understand the origin of these cysts.

  1. Laryngeal Electromyography for Prognosis of Vocal Fold Paralysis.

    Science.gov (United States)

    Pardo-Maza, Adriana; García-Lopez, Isabel; Santiago-Pérez, Susana; Gavilán, Javier

    2017-01-01

    This study aimed to determine the value of laryngeal electromyography in the prognosis of vocal fold paralysis. This is a retrospective descriptive study. This study included 80 patients diagnosed with unilateral or bilateral vocal fold paralysis on flexible laryngoscopy between 2002 and 2014 in a tertiary medical center. Laryngeal electromyography using a standardized protocol was performed; the outcome measures were classified and analyzed into two groups according to the degree of injury. Group 1 included patients with mild to moderate injury, and group 2 included patients with severe to complete injury. Prognosis was correlated with vocal fold motion recovery status with a minimum of 6 months of follow-up since the symptoms onset using positive and negative predictive values. Sixty patients showed acute or chronic recurrent laryngeal neuropathy in laryngeal electromyography. Twelve of 41 patients included in group 1 recovered motion, and 30 of 35 patients included in group 2 did not recover, resulting in 88.2% of positive predictive value and 35.7% of negative predictive value. Our data confirm that laryngeal electromyography is a useful clinical tool in predicting poor recovery in patients with vocal fold paralysis. It allows identification of candidates for early intervention. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  2. Prenatal development supports a single origin of laryngeal echolocation in bats.

    Science.gov (United States)

    Wang, Zhe; Zhu, Tengteng; Xue, Huiling; Fang, Na; Zhang, Junpeng; Zhang, Libiao; Pang, Jian; Teeling, Emma C; Zhang, Shuyi

    2017-01-09

    Bat laryngeal echolocation is considered as one of the most complex and diverse modes of auditory sensory perception in animals and its evolutionary history has been the cause of many scientific controversies in the past two decades. To date, the majority of scientific evidence supports that bats (Chiroptera) are divided into two subordinal groups: Yinpterochiroptera, containing the laryngeal echolocating superfamily Rhinolophidae as sister taxa to the non-laryngeal echolocating family Pteropodidae; and Yangochiroptera, containing all other laryngeal echolocating lineages. This topology has led to an unanswered question in mammalian biology: was laryngeal echolocation lost in the ancestral pteropodids or gained convergently in the echolocating bat lineages? To date, there is insufficient and conflicting evidence from fossil, genomic, morphological and phylogenomic data to resolve this question. We detail an ontogenetic study of fetal cochlear development from seven species of bats and five outgroup mammals and show that in early fetal development, all bats including the non-laryngeal echolocating pteropodids have a similarly large cochlea typically associated with laryngeal echolocation abilities. The subsequent cochlear growth rate in the pteropodids is the slowest of all mammals and leads to the pteropodids and the non-echolocating lineages eventually sharing a similar cochlear morphospace as adults. The results suggest that pteropodids maintain a vestigial developmental stage indicative of past echolocation capabilities and thus support a single origin of laryngeal echolocation in bats.

  3. Laryngeal electromyography in movement disorders: preliminary data

    Directory of Open Access Journals (Sweden)

    Kimaid Paulo A.T.

    2004-01-01

    Full Text Available This study describes preliminary laryngeal electromyography (LEMG data and botulinum toxin treatment in patients with dysphonia due to movement disorders. Twenty-five patients who had been clinically selected for botulinum toxin administration were examined, 19 with suspected laryngeal dystonia or spasmodic dysphonia (SD, 5 with vocal tremor, and 1 with Gilles de la Tourette syndrome (GTS. LEMG evaluations were performed before botulinum toxin administration using monopolar electrodes. Electromyography was consistent with dystonia in 14 patients and normal in 5, and differences in frequency suggesting essential tremor in 3 and Parkinson tremors in 2. The different LEMG patterns and significant improvement in our patients from botulinum toxin therapy has led us to perform laryngeal electromyography as a routine in UNICAMP movement disorders ambulatory.

  4. Laryngeal sensation and pharyngeal delay time after (chemo)radiotherapy.

    Science.gov (United States)

    Maruo, Takashi; Fujimoto, Yasushi; Ozawa, Kikuko; Hiramatsu, Mariko; Suzuki, Atsushi; Nishio, Naoki; Nakashima, Tsutomu

    2014-08-01

    The objective of the study was to evaluate the association between changes in laryngeal sensation and initiation of swallowing reflex or swallowing function before and after (chemo)radiotherapy. A prospective study was conducted in a tertiary referral university hospital. Thirteen patients who received (chemo)radiotherapy for treatment of laryngeal or hypopharyngeal cancer were included. Laryngeal sensation was evaluated at the tip of the epiglottis before and 1, 3 months, and 1 year after (chemo)radiotherapy. Videofluoroscopy was performed at the same time. Quantitative determinations included changes in laryngeal sensation, computed analysis of pharyngeal delay time, the distance and velocity of hyoid bone movement during the phase of hyoid excursion, and pharyngeal residue rate (the proportion of the bolus that was left as residue in the pharynx at the first swallow). Laryngeal sensation significantly deteriorated 1 month after (chemo)radiotherapy, but there was a tendency to return to pretreatment levels 1 year after treatment. Neither pharyngeal delay time nor displacement of the hyoid bone changed significantly before and after (chemo)radiotherapy. In addition, there was no significant difference in the mean velocity of hyoid bone movement and the amount of stasis in the pharynx at the first swallow before and after (chemo)radiotherapy. After (chemo)radiotherapy, laryngeal sensation deteriorated. But, in this study, videofluoroscopy showed that swallowing reflex and function were maintained.

  5. The CT features of recurrent laryngeal nerve paralysis

    International Nuclear Information System (INIS)

    Wu Bin; Peng Weijun; Gu Yajia; Yang Tianxi; Wang Hongshi

    2006-01-01

    Objective: To describe the CT appearance of recurrent laryngeal nerve paralysis, discuss the anatomic and pathologic basis of this paralysis, and evaluate CT diagnosis. Methods: 32 cases of recurrent laryngeal nerve paralysis clinical confirmed were analyzed retrospectively. All of these patients had the CT scans from the level of hyoid bone to the upper thorax, the slice and interval are 5 mm. Results: CT findings of recurrent laryngeal nerve paralysis included: oblique of aryepiglottic fold, dislocation of arytenoid cartilage and cricoarytenoid joint, dilation and relaxation of piriform sinus for 27 cases (84.4%); wide and asymmetrical ventricle of larynx for 16 cases (50.0%); asymmetrical and fix of vocal fold for 11 cases (34.4%) et al. Conclusion: The recurrent laryngeal nerve innervate all the intrinsic muscles of the larynx except cricothyroid muscle, paralysis of the nerve leads to atrophy of related muscles. CT scan demonstrate the larynx morphologic changes of recurrent nerve paralysis and is helpful to identify the etiology. (authors)

  6. 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

  7. Case report: human papilloma virus type 120-related papillomatosis mimicking laryngeal carcinoma.

    Science.gov (United States)

    Belvisi, Valeria; Martellucci, Salvatore; Garbuglia, Anna Rosa; Del Borgo, Cosmo; Martellucci, Stanislao; Baiocchini, Andrea; Manicone, Anna Maria; Bagni, Oreste; Mastroianni, Claudio Maria; Gallo, Andrea

    2017-10-01

    The relationship between human papilloma virus (HPV) and upper respiratory tract pathology was better understood in recent years and represents now an issue of particular interest in carcinogenesis and in immunocompromised host. We describe a case in which a rare genotype HPV-related papillomatosis mimics laryngeal carcinoma in an immunocompromised host. A 54-year-old woman with a history of HIV-HCV coinfection and anal and laryngeal cancer successfully treated some years before was hospitalized for severe dyspnea, cough and dysphagia. Fiberoptic endoscopic evaluation raised the suspicion of tumor relapse showing the presence of a large glottic-supraglottic ulcerated mass. Several laryngeal biopsies demonstrated koilocytosis and p16 expression, according to a possible HPV infection, and focal figures of mild dysplasia of epithelium. 18 F-FDG PET/CT did not show high glycolytic activity at laryngeal level. An invasive upper respiratory tract papillomatosis in an immunocompromised host was suspected because of the patient's clinical improvement after antiretroviral therapy. Pharyngeal swab and oral rinse harboured the same HPV120 genotype sequence, a betapapillomavirus of recent description and not yet related to any similar clinical presentations.

  8. Dialysis-related amyloidosis: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Scarpioni R

    2016-12-01

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

  9. Solitary plasmacytoma of spine with amyloidosis

    Directory of Open Access Journals (Sweden)

    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

  10. Laryngeal cancer at the Korle Bu Teaching Hospital, Accra, Ghana ...

    African Journals Online (AJOL)

    Majority of the patients (90.4%) were above 40 years. The commonest symptom at presentation was dysphonia. A significant proportion of cases (37.3%) presented with locally advanced disease. The commonest histological type of laryngeal tumour seen was squamous cell carcinoma. The treatment offered consisted of ...

  11. Childhood Laryngeal Tumors Treatment (PDQ®)—Patient Version

    Science.gov (United States)

    Childhood laryngeal (throat) tumors are tumors of the larynx (voice box). They can be benign (papillomatosis) or cancer. Treatment depends on the type of cell the cancer grew from. Get information about the symptoms, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood laryngeal tumors in this expert-reviewed summary.

  12. Quantitative PCR Analysis of Laryngeal Muscle Fiber Types

    Science.gov (United States)

    Van Daele, Douglas J.

    2010-01-01

    Voice and swallowing dysfunction as a result of recurrent laryngeal nerve paralysis can be improved with vocal fold injections or laryngeal framework surgery. However, denervation atrophy can cause late-term clinical failure. A major determinant of skeletal muscle physiology is myosin heavy chain (MyHC) expression, and previous protein analyses…

  13. [Study on the correlation between EGFR-STAT3 signal pathway and laryngeal papilloma].

    Science.gov (United States)

    Wang, Xinhua; Sun, Jingwu

    2009-09-01

    To explore the relationship between the expression of EGFR and STAT3 in human laryngeal papilloma and its biological behavior. Reverse transcription polymerase chain reaction(RT-PCR), immunohistochemical staining and Western blot were used to evaluate the mRNA and protein expression of EGFR and STAT3 (p-STAT3) in 42 laryngeal papilloma tissues and 15 samples of normal laryngeal tissue, and the relationship between the protein expression of them and clinic pathological parameters was also analyzed. The mRNA expression levels of EGFR and STAT3 in laryngeal papilloma tissue were significantly higher than that in normal laryngeal tissue (P papilloma than normal laryngeal tissue by immunohistochemistry and western blot (P papilloma (P papilloma (P papilloma,, and the persistent activation of STAT3 gene plays an important role in the recurrence and canceration of laryngeal papilloma.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2010-01-15

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

  15. 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

  16. Laryngeal paralysis in dogs: An update on recent knowledge

    Directory of Open Access Journals (Sweden)

    Adriaan M. Kitshoff

    2013-04-01

    Full Text Available Laryngeal paralysis is the effect of an inability to abduct the arytenoid cartilages duringinspiration, resulting in respiratory signs consistent with partial airway obstruction. Theaetiology of the disease can be congenital (hereditary laryngeal paralysis or congenitalpolyneuropathy, or acquired (trauma, neoplasia, polyneuropathy, endocrinopathy. Themost common form of acquired laryngeal paralysis (LP is typically seen in old, large breeddogs and is a clinical manifestation of a generalised peripheral polyneuropathy recentlyreferred to as geriatric onset laryngeal paralysis polyneuropathy. Diagnosing LP based onclinical signs, breed and history has a very high sensitivity (90% and can be confirmed bylaryngeal inspection. Prognosis after surgical correction depends on the aetiology: traumaticcases have a good prognosis, whereas tumour-induced or polyneuropathy-induced LP has aguarded prognosis. Acquired idiopathic LP is a slow progressive disease, with dogs reachingmedian survival times of 3–5 years after surgical correction.

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

    Directory of Open Access Journals (Sweden)

    S. Seijo Ríos

    2008-12-01

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

  18. 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.

  19. 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.

  20. 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 ...

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

    Directory of Open Access Journals (Sweden)

    Mizejewski GJ

    2017-04-01

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

  2. Childhood Laryngeal Dystonia Following Bilateral Globus Pallidus Abnormality: A Case Study and Review of Literature

    Directory of Open Access Journals (Sweden)

    Mohammad Javad Saeedi Borujeni

    2017-01-01

    Full Text Available Introduction:Dystonia is a disorder of movement caused by various etiologies. Laryngeal dystonia is caused by the spasm of laryngeal muscles. It is a disorder caused by vocal fold movement in which excessive adduction or abduction of the vocal folds occurs during speech. The pathophysiology of this type of dystonia is not fully known. Some researchers have suggested that basal ganglia structures and their connections with cortical areas have been involved in the pathogenesis of dystonia. Case Report:In this paper a 7.5-year-old boy suffering from laryngeal dystonia with bilateral lesions in Globus Pallidus is presented. The patient also suffered from swallowing problems, monotone voice, vocal tremor, hypersensitivity of gag reflex, and stuttering. Drug treatment failed to cure him; therefore, he was referred to rehabilitation therapy.  Conclusion:In conclusion, special attention should be brought upon laryngeal dystonia, especially in patients showing Extra-pyramidal symptoms and/or abnormalities of the basal ganglia. In children, laryngeal dystonia may be potentially fatal. Lack of consideration for this condition during rehabilitation therapy can lead to serious consequences for a child.

  3. 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 ...

  4. Podoplanin expression in the development and progression of laryngeal squamous cell carcinomas

    Science.gov (United States)

    2010-01-01

    Background Podoplanin expression is attracting interest as a marker for cancer diagnosis and prognosis. We therefore investigated the expression pattern and clinical significance of podoplanin during the development and progression of laryngeal carcinomas. Results Podoplanin expression was determined by immunohistochemistry in paraffin-embedded tissue specimens from 84 patients with laryngeal premalignancies and 53 patients with laryngeal squamous cell carcinomas. We found podoplanin expression extending from the basal to the suprabasal layer of the epithelium in 37 (44%) of 84 dysplastic lesions, whereas normal epithelium showed negligible expression. Patients carrying podoplanin-positive lesions had a higher laryngeal cancer incidence than those with negative expression reaching borderline statistical significance (51% versus 30%, P = 0.071). Podoplanin expression in laryngeal carcinomas exhibited two distinct patterns. 20 (38%) cases showed diffuse expression in most tumour cells and 33 (62%) focal expression at the proliferating periphery of tumour nests. High podoplanin expression was inversely correlated with T classification (P = 0.033), disease stage (P = 0.006), and pathological grade (P = 0.04). There was a trend, although not significant, towards reduced disease-specific survival for patients with low podoplanin levels (P = 0.31) and diffuse expression pattern (P = 0.08). Conclusions Podoplanin expression increases in the early stages of laryngeal tumourigenesis and it seems to be associated with a higher laryngeal cancer risk. Podoplanin expression in laryngeal squamous cell carcinomas, however, diminishes during tumour progression. Taken together, these data support a role for podoplanin expression in the initiation but not in the progression of laryngeal cancers. PMID:20196862

  5. Podoplanin expression in the development and progression of laryngeal squamous cell carcinomas

    Directory of Open Access Journals (Sweden)

    Fresno Manuel F

    2010-03-01

    Full Text Available Abstract Background Podoplanin expression is attracting interest as a marker for cancer diagnosis and prognosis. We therefore investigated the expression pattern and clinical significance of podoplanin during the development and progression of laryngeal carcinomas. Results Podoplanin expression was determined by immunohistochemistry in paraffin-embedded tissue specimens from 84 patients with laryngeal premalignancies and 53 patients with laryngeal squamous cell carcinomas. We found podoplanin expression extending from the basal to the suprabasal layer of the epithelium in 37 (44% of 84 dysplastic lesions, whereas normal epithelium showed negligible expression. Patients carrying podoplanin-positive lesions had a higher laryngeal cancer incidence than those with negative expression reaching borderline statistical significance (51% versus 30%, P = 0.071. Podoplanin expression in laryngeal carcinomas exhibited two distinct patterns. 20 (38% cases showed diffuse expression in most tumour cells and 33 (62% focal expression at the proliferating periphery of tumour nests. High podoplanin expression was inversely correlated with T classification (P = 0.033, disease stage (P = 0.006, and pathological grade (P = 0.04. There was a trend, although not significant, towards reduced disease-specific survival for patients with low podoplanin levels (P = 0.31 and diffuse expression pattern (P = 0.08. Conclusions Podoplanin expression increases in the early stages of laryngeal tumourigenesis and it seems to be associated with a higher laryngeal cancer risk. Podoplanin expression in laryngeal squamous cell carcinomas, however, diminishes during tumour progression. Taken together, these data support a role for podoplanin expression in the initiation but not in the progression of laryngeal cancers.

  6. [Expression and clinical significance of CD45RO in laryngeal carcinoma tissue].

    Science.gov (United States)

    Li, Manyi; Liu, Jishengi; Zhou, Hui; Wu, Wenying; Xiao, Gensheng; Yu, Yafeng; Guo, Lingchuan

    2014-03-01

    To investigate the role and significance of CD45RO in occurance and development in laryngeal squamous carcinoma, and to provide some valuable clues for searching new approaches to assess prognosis and theoretical basis for tumor biotherapy. The expression of CD45RO protein in 50 cases of laryngeal squamous carcinoma and 10 cases normal mucos was detected by immunohistochemical S-P method. The positive rate of CD45RO was 30% and 86% respectively in normal tissue and laryngeal squamous cell carcinoma tissue. The expresion of CD45RO was significantly and negatively associated with local metastatic of lymph nodes 0.713, P < 0.05) and tumor sites (r = -0.750, P < 0.05), but it have no notable difference with pathology differentiation, age, infiltrating depth and clinical stages in 50 cases of laryngeal squamous cell cancer. (1) The expresion of CD45RO in laryngeal squamous cell cancer is more than that in normal tissue. (2) It is possible that overexpresion of CD45RO in laryngeal squamous cell carcinoma cut local metastatic lymph nodes. (3) It is probable that overexpresion of CD45RO in laryngeal squamous cell cancer made for prognosis of patients. (4) Other than UICC-TNM stage, pathology differentiation, it provide valuable clues for searching new approaches to assess prognosis of laryngeal squamous cell carcinoma.

  7. Effects of Voice Therapy on Laryngeal Motor Units During Phonation in Chronic Superior Laryngeal Nerve Paresis Dysphonia.

    Science.gov (United States)

    Kaneko, Mami; Hitomi, Takefumi; Takekawa, Takashi; Tsuji, Takuya; Kishimoto, Yo; Hirano, Shigeru

    2017-09-26

    Injury to the superior laryngeal nerve can result in dysphonia, and in particular, loss of vocal range. It can be an especially difficult problem to address with either voice therapy or surgical intervention. Some clinicians and scientists suggest that combining vocal exercises with adjunctive neuromuscular electrical stimulation may enhance the positive effects of voice therapy for superior laryngeal nerve paresis (SLNP). However, the effects of voice therapy without neuromuscular electrical stimulation are unknown. The purpose of this retrospective study was to demonstrate the clinical effectiveness of voice therapy for rehabilitating chronic SLNP dysphonia in two subjects, using interspike interval (ISI) variability of laryngeal motor units by laryngeal electromyography (LEMG). Both patients underwent LEMG and were diagnosed with having 70% recruitment of the cricothyroid muscle, and 70% recruitment of the cricothyroid and thyroarytenoid muscles, respectively. Both patients received voice therapy for 3 months. Grade, roughness, breathiness, asthenia, and strain (GRBAS) scale, stroboscopic examination, aerodynamic assessment, acoustic analysis, and Voice Handicap Index-10 were performed before and after voice therapy. Mean ISI variability during steady phonation was also assessed. After voice therapy, both patients showed improvement in vocal assessments by acoustic, aerodynamic, GRBAS, and Voice Handicap Index-10 analysis. LEMG indicated shortened ISIs in both cases. This study suggests that voice therapy for chronic SLNP dysphonia can be useful for improving SLNP and voice quality. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    Francesca Caso

    2013-01-01

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

  9. Schwannoma of the Recurrent Laryngeal Nerve : A Rare Entity

    NARCIS (Netherlands)

    de Heer, Linda M; Teding van Berkhout, F; Priesterbach, Loudy; Buijsrogge, Marc P

    Neurogenic tumors are the most common posterior mediastinal tumors in adults. Schwannomas originating from the recurrent laryngeal nerve are rare. The present study describes a 46-year-old man with a tumor in the left superior mediastinum. Because of the narrow relationship with the aorta and the

  10. Laryngeal paralysis in dogs: an update on recent knowledge.

    Science.gov (United States)

    Kitshoff, Adriaan M; Van Goethem, Bart; Stegen, Ludo; Vandekerckhov, Peter; de Rooster, Hilde

    2013-04-05

    Laryngeal paralysis is the effect of an inability to abduct the arytenoid cartilages during inspiration, resulting in respiratory signs consistent with partial airway obstruction. The aetiology of the disease can be congenital (hereditary laryngeal paralysis or congenital polyneuropathy), or acquired (trauma, neoplasia, polyneuropathy, endocrinopathy). The most common form of acquired laryngeal paralysis (LP) is typically seen in old, large breed dogs and is a clinical manifestation of a generalised peripheral polyneuropathy recently referred to as geriatric onset laryngeal paralysis polyneuropathy. Diagnosing LP based on clinical signs, breed and history has a very high sensitivity (90%) and can be confirmed bylaryngeal inspection. Prognosis after surgical correction depends on the aetiology: traumatic cases have a good prognosis, whereas tumour-induced or polyneuropathy-induced LP has a guarded prognosis. Acquired idiopathic LP is a slow progressive disease, with dogs reaching median survival times of 3-5 years after surgical correction.

  11. Angled telescopic surgery, an approach for laryngeal diagnosis and surgery without suspension

    Directory of Open Access Journals (Sweden)

    Plínio Ferreira Morgado

    1999-09-01

    Full Text Available CONTEXT: Many methods have been used successfully for the diagnosis and treatment of laryngeal diseases. Microscopic and, recently, telescopic surgery represent the state of the art in endoscopic laryngeal surgery but drawbacks are possible during their application. To keep the suspension apparatus adequately positioned, excessive force is sometimes placed on the upper teeth and tongue with the laryngoscope tube causing damage. Complications in relation to the pharynx, larynx and cardiovascular system have also been reported. OBJECTIVE: In order to reduce complications resulting from the manipulation or stimulation of the upper aerodigestive tract and from torque forces on the upper teeth. We present a method of larynx surgery in which laryngeal suspension is not required. DESIGN: Technical report. TECHNIQUES: We have devised a fiber-optic telescope with its 40mm distal portion deviated 60 degrees from the direction of the proximal portion. This angle was taken by measuring patients immediately before standard microlaryngeal surgery was performed. The surgical instruments have the same angle as the telescope, in order to work on the larynx. This technique provides an image that is not limited by the distal aperture of the laryngoscope and has an advantage in that magnification and illumination may be provided by changing the distance of the lesion from the tip of the instrument. we have operated on four patients with laryngeal diseases and have had no complications as a result of this approach. We feel that this technique gives us the freedom to view the lesions better and helps to minimize the drawbacks caused by laryngeal suspension.

  12. Laryngoscopic and spectral analysis of laryngeal and pharyngeal configuration in non-classical singing styles.

    Science.gov (United States)

    Guzman, Marco; Lanas, Andres; Olavarria, Christian; Azocar, Maria Josefina; Muñoz, Daniel; Madrid, Sofia; Monsalve, Sebastian; Martinez, Francisca; Vargas, Sindy; Cortez, Pedro; Mayerhoff, Ross M

    2015-01-01

    The present study aimed to assess three different singing styles (pop, rock, and jazz) with laryngoscopic, acoustic, and perceptual analysis in healthy singers at different loudness levels. Special emphasis was given to the degree of anterior-posterior (A-P) laryngeal compression, medial laryngeal compression, vertical laryngeal position (VLP), and pharyngeal compression. Prospective study. Twelve female trained singers with at least 5 years of voice training and absence of any voice pathology were included. Flexible and rigid laryngeal endoscopic examinations were performed. Voice recording was also carried out. Four blinded judges were asked to assess laryngoscopic and auditory perceptual variables using a visual analog scale. All laryngoscopic parameters showed significant differences for all singing styles. Rock showed the greatest degree for all of them. Overall A-P laryngeal compression scores demonstrated significantly higher values than overall medial compression and VLP. High loudness level produced the highest degree of A-P compression, medial compression, pharyngeal compression, and the lowest VLP for all singing styles. Additionally, rock demonstrated the highest values for alpha ratio (less steep spectral slope), L1-L0 ratio (more glottal adduction), and Leq (more vocal intensity). Statistically significant differences between the three loudness levels were also found for these acoustic parameters. Rock singing seems to be the style with the highest degree of both laryngeal and pharyngeal activity in healthy singers. Although, supraglottic activity during singing could be labeled as hyperfunctional vocal behavior, it may not necessarily be harmful, but a strategy to avoid vocal fold damage. Copyright © 2015 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

  14. The Laryngeal Mask Airway Supreme™: safety and efficacy during ...

    African Journals Online (AJOL)

    Background: Laryngeal Mask Airway Supreme™ (LMA Supreme™) is a new single-use polyvinyl chloride supraglottic device that offers gastric access. To date, studies that have tested the LMA Supreme™) for use in laparoscopic surgery have been reported. We present the largest evaluative study that describes the use of ...

  15. A survey of practice patterns in the use of laryngeal mask by pediatric anesthesiologists.

    Science.gov (United States)

    Patel, Anuradha; Clark, Scott R; Schiffmiller, Moshe; Schoenberg, Catherine; Tewfik, George

    2015-11-01

    Laryngeal mask is frequently the airway device of choice in routine general anesthesia for many procedures in children. Several studies have described the use of laryngeal masks in unconventional situations. This survey was undertaken to assess how laryngeal masks are being used by pediatric anesthesiologists. The 40-question electronic survey using SurveyMonkey™ was sent to 2740 members of the Society for Pediatric Anesthesia (SPA). This survey assessed the age, work environment, types of practice, and training levels, as well as clinical situations in which the practitioners use laryngeal masks across different pediatric age groups. Seven hundred and forty-three (27.1%) responses were obtained. The use of laryngeal mask increased as the patient age increased in nearly every queried situation. The practitioners routinely utilize laryngeal masks in a variety of challenging scenarios, such as in patients with a recent upper respiratory infection, in the difficult airway, remote locations, and long-duration surgeries. A small percentage of pediatric anesthesiologists use laryngeal masks in laparoscopic surgery and prone position procedures. Pediatric anesthesiologists are using laryngeal masks in both routine and challenging/unconventional situations. Although many of the uses for laryngeal masks are not explicitly stated in the manufacturer guidelines, literature and current practice support the use of laryngeal masks in several of these scenarios. © 2015 John Wiley & Sons Ltd.

  16. Muscle Bioenergetic Considerations for Intrinsic Laryngeal Skeletal Muscle Physiology

    Science.gov (United States)

    Sandage, Mary J.; Smith, Audrey G.

    2017-01-01

    Purpose: Intrinsic laryngeal skeletal muscle bioenergetics, the means by which muscles produce fuel for muscle metabolism, is an understudied aspect of laryngeal physiology with direct implications for voice habilitation and rehabilitation. The purpose of this review is to describe bioenergetic pathways identified in limb skeletal muscle and…

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Assessment of laryngeal functions in patients with squamous cell carcinoma of the larynx or hypopharynx treated with concurrent chemoradiotherapy

    International Nuclear Information System (INIS)

    Taguchi, Takahide; Tsukuda, Mamoru; Nagao, Jun-ichi

    2010-01-01

    The laryngeal functions in patients with laryngeal or hypopharyngeal cancer treated with concurrent chemoradiotherapy (CCRT) were evaluated. We reviewed the records of 32 patients with resectable stage II to IV squamous cell carcinoma of the larynx or hypopharynx treated with CCRT as a initial treatment between October 1998 and September 2003. A questionnaire survey and measurements of maximum phonation time, GRBAS and range of voice/speaking fundamental frequency were performed, and the method of food intake in the swallowing ability scale and the presence of tracheostoma were assessed for evaluating laryngeal functions after CCRT. After CCRT for laryngeal or hypopharyngeal cancer, the phonatory functions appeared to be preserved in 75% of the patients in terms of sustainability; however, there were some cases presenting hoarseness and narrowed range of voice. Oral intake was possible in most cases (97%) without a history of aspiration pneumonia. In 2 cases, permanent tracheostoma had to be retained. It was concluded that laryngeal functions could be preserved in most cases after CCRT, though the tracheostoma might not be closed in some patients with laryngeal cancer. (author)

  19. 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

  20. Complications of laryngeal framework surgery (phonosurgery).

    Science.gov (United States)

    Tucker, H M; Wanamaker, J; Trott, M; Hicks, D

    1993-05-01

    The rising popularity of surgery involving the laryngeal framework (surgical medialization of immobile vocal folds, vocal fold tightening, pitch variation, etc.) has resulted in increasing case experience. Little has appeared in the literature regarding complications or long-term results of this type of surgery. Several years' experience in a major referral center with various types of laryngeal framework surgery has led to a small number of complications. These have included late extrusion of the prosthesis and delayed hemorrhage. A review of these complications and recommendations for modification of technique to minimize them in the future are discussed.

  1. Assessment of quality of life in patients with laryngeal cancer: A review of articles.

    Science.gov (United States)

    Kolator, Mateusz; Kolator, Patrycja; Zatoński, Tomasz

    2018-04-19

    This article presents a review of the medical literature published between 1994 and 2014 with the use of the PubMed database concerning quality-of-life instruments for head and neck cancer patients used to assess general well-being of patients with laryngeal cancer. The PubMed database was searched for articles containing the keywords "quality of life", "laryngeal neoplasm" and "questionnaires". The resulting articles were reviewed and analyzed. After the identification of questionnaires, an additional search was performed. The articles and questionnaires were described and analyzed. In 43 articles, the authors used questionnaires specific to the head and neck regions in order to assess the quality of life in patients with laryngeal cancer. Four different questionnaires were identified. The European Organization for Research and Treatment of Cancer (EORTC) questionnaire is most commonly used to assess the quality of life in patients with laryngeal cancer. Questionnaires are generally used in order to select from a range of different treatment methods. There are a few head and neck cancer-related quality-of-life instruments which are widely used to assess the quality of life in patients with laryngeal cancer, but they are not dedicated to that region of the body. Today, there is much more attention paid to the quality of life; therefore, there is a real need to develop specific scales for different types of cancer.

  2. Laryngeal Adenoid Cystic Carcinoma: A Systematic Review.

    Science.gov (United States)

    Marchiano, Emily; Chin, Oliver Y; Fang, Christina H; Park, Richard Chan; Baredes, Soly; Eloy, Jean Anderson

    2016-03-01

    Adenoid cystic carcinoma is a malignant minor salivary gland tumor that represents laryngeal tumors. The submucosal location of laryngeal adenoid cystic carcinoma (LACC) results in delayed presentation. Here, we present the first systematic review of reported cases of LACC to determine trends in presentation, diagnostic and treatment modalities, and patient outcome. PubMed, Web of Science, MEDLINE, and EMBASE databases. A search of the above databases was done to identify articles reporting cases of LACC. The variables included in the analysis were patient demographics, presenting symptoms, tumor location, imaging, treatment, follow-up time, recurrence, and outcome. A total of 50 articles and 120 cases were included in the review. The most common presenting symptom was dyspnea (48.8%), followed by hoarseness (43.9%). LACC arose most frequently from the subglottis (56.7%). At presentation, 14.6% (13 of 89) of patients had regional disease. The average follow-up time was 54.0 months. At follow-up, distant metastasis was reported in 30 cases (33.3%). Surgery alone (43.3%) and surgery with radiotherapy (43.3%) were used most frequently and resulted in 57.1% and 55.3% of patients alive with no evidence disease at follow-up, respectively. LACC was most often located in the subglottis. Patients commonly presented with dyspnea and hoarseness. In this systematic review, surgery with radiotherapy and surgery alone were the most commonly employed treatment modalities, and both resulted in slightly more than 50% of patients alive with no evidence of disease at follow-up. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  3. Laryngitis (For Kids)

    Science.gov (United States)

    ... to change your diet and give up some foods that make the problem worse. Can I Prevent It? To prevent laryngitis, try not to talk or yell in a way that hurts your voice. A humidifier that puts more water into the air may also help keep your throat from drying out. Also, never smoke and try not to ...

  4. Self evaluation of communication experiences after laryngeal cancer – A longitudinal questionnaire study in patients with laryngeal cancer

    Directory of Open Access Journals (Sweden)

    Finizia Caterina

    2008-03-01

    Full Text Available Abstract Background Aim of this longitudinal study was to investigate the sensitivity to change of the Swedish Self Evaluation of Communication Experiences after Laryngeal Cancer questionnaire (the S-SECEL, addressing communication dysfunction in patients treated for laryngeal cancer. Previous studies have highlighted the need for more specific questionnaires for this purpose. Methods 100 patients with Tis-T4 laryngeal cancer were included prior to treatment onset. Patients answered four questionnaires at six occasions during one year; the S-SECEL, the European Organisation for Research and Treatment of Cancer (EORTC Core Quality of Life Core Questionnaire (QLQ-C30 supplemented by the Head and Neck cancer module (QLQ-H&N35 and the Hospital Anxiety and Depression (HAD scale. In addition, performance status was assessed. Differences within groups were tested with the Wilcoxon paired signed ranks test and between-group analyses were carried out using the Mann-Whitney U test. Magnitude of group differences was analyzed by means of effect sizes. Results The S-SECEL was well accepted with a response rate of 76%. Communication dysfunction increased at 1 month, followed by a continuous decrease throughout the year. Changes were statistically significant at most measurement, demonstrating the sensitivity of the S-SECEL to changes in communication over time. The S-SECEL and the EORTC QLQ-C30 with the QLQ-H&N35 demonstrated similar results; however the S-SECEL was more sensitive regarding communication dysfunction. The largest changes were found in the most diagnose specific items concerning voice and speech. Conclusion The S-SECEL was investigated in the largest Scandinavian longitudinal study concerning health-related quality of life (HRQL in laryngeal cancer patients. The questionnaire was responsive to change and showed convergent results when compared to established HRQL questionnaires. Our findings also indicate that the S-SECEL could be a more

  5. Neurophysiology and Clinical Implications of the Laryngeal Adductor Reflex.

    Science.gov (United States)

    Domer, Amanda S; Kuhn, Maggie A; Belafsky, Peter C

    2013-09-01

    The laryngeal adductor reflex (LAR) is an involuntary protective response to stimuli in the larynx. The superior laryngeal nerve (SLN) acts as the afferent limb and the recurrent laryngeal nerve (RLN) as the efferent limb of this reflex, which is modulated by the central nervous system. Perhaps the most clinically significant application of the LAR is its use in laryngopharyngeal (LP) sensory discrimination testing. Importantly, aberrations in the LAR may predict dysphagia or portend clinical phenotypes of chronic cough, vocal cord dysfunction or pediatric apneas. LP sensation is a potential target for interventions addressing the aforementioned conditions though currently remains an area of active investigation.

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

    Directory of Open Access Journals (Sweden)

    Balwani Manish R

    2015-04-01

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

  7. Basaloid Squamous Cell Carcinoma: An Unusual Ball-Valve Laryngeal Obstruction

    Directory of Open Access Journals (Sweden)

    Sien Hui Tan

    2015-05-01

    Full Text Available Introduction: A rare case of basaloid squamous cell carcinoma (BSCC of the larynx, which has not been previously reported, is described. Case Report: A 60-year-old man was presented to the Otolaryngology Department with progressive dyspnoea and dysphagia to solids for over a period of 1 week. Direct laryngoscopy revealed a tumour at the laryngeal aspect of the epiglottis, which prolapsed into the laryngeal inlet each time the patient inspired. This resulted in an inspiratory stridor despite adequate glottic opening and normal mobility of the vocal cords.   Conclusion:  Therefore, in cases where a ball-valve lesion causes intermittent life-threatening airway obstruction, BSCC of the larynx, though rare, must be considered as a differential diagnosis.

  8. 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

  9. Advantages, Disadvantages, Indications, Contraindications and Surgical Technique of Laryngeal Airway Mask.

    Science.gov (United States)

    Jannu, Anubhav; Shekar, Ashim; Balakrishna, Ramdas; Sudarshan, H; Veena, G C; Bhuvaneshwari, S

    2017-12-01

    The beauty of the laryngeal mask is that it forms an air tight seal enclosing the larynx rather than plugging the pharynx, and avoid airway obstruction in the oropharynx. The goal of its development was to create an intermediate form of airway management face mask and endotracheal tube. Indication for its use includes any procedure that would normally involve the use of a face mask. The laryngeal mask airway was designed as a new concept in airway management and has been gaining a firm position in anesthetic practice. Despite wide spread use the definitive role of the laryngeal mask airway is yet to be established. In some situations, such as after failed tracheal intubation or in oral surgery its use is controversial. There are several unresolved issues, for example the effect of the laryngeal mask on regurgitation and whether or not cricoids pressure prevents placement of mask. We review the techniques of insertion, details of misplacement, and complications associated with use of the laryngeal mask. We then attempt to clarify the role of laryngeal mask in air way management during anesthesia, discussing the advantages and disadvantages as well as indications and contraindications of its use in oral and maxillofacial surgery.

  10. Human papilloma virus prevalence in laryngeal squamous cell carcinoma.

    Science.gov (United States)

    Gungor, A; Cincik, H; Baloglu, H; Cekin, E; Dogru, S; Dursun, E

    2007-08-01

    To determine the prevalence and type of human papilloma virus deoxyribonucleic acid (DNA) in cases of laryngeal squamous cell carcinoma. We analysed the prevalence of human papilloma virus infection in archived paraffin block specimens taken from 99 cases of laryngeal squamous cell carcinoma between 1990 and 2005, using polymerase chain reaction techniques. Biopsy specimens from five proven verrucous skin lesions were used as positive controls, and peripheral blood samples from five healthy volunteers were used as negative controls. Four test samples were found to have inadequate deoxyribonucleic acid purity and were therefore excluded from the study. Human papilloma virus deoxyribonucleic acid was detected in seven of 95 cases of laryngeal squamous cell carcinoma (7.36 per cent). Human papilloma virus genotyping revealed double human papilloma virus infection in three cases and single human papilloma virus infection in the remaining four cases. The human papilloma virus genotypes detected were 6, 11 and 16 (the latter detected in only one case). In our series, a very low human papilloma virus prevalence was found among laryngeal squamous cell carcinoma cases. The human papilloma virus genotypes detected were mostly 6 and/or 11, and 16 in only one case. To the best of our knowledge, this is the first report of human papilloma virus prevalence in laryngeal squamous cell carcinoma, based on polymerase chain reaction genotyping in a Turkish population.

  11. Computed tomography of laryngeal carcinoma correlated with histopathological findings

    Energy Technology Data Exchange (ETDEWEB)

    Cai, Yi-Long

    1988-09-01

    Since the development of conservation laryngeal surgery and the advent of computed tomography (CT) scanners, a precise preoperative evaluation of the extent of laryngeal cancer has been of prime importance. Eight patients with known carcinoma of the larynx were examined with CT of the larynx prior to surgery, and whole-mount serial sections of the extirpated larynx were compared with the corresponding level of CT sections to evaluate the reliability of CT during my study abroad in Japan from Dec. 1985 through Dec. 1986. 1. The results indicated that CT scanning accurately delineates the anatomic location and pathologic extent of the tumor three-dimensionally in all cases examined. There is also good demonstration of the anterior commissure and preepiglottic, paraglottic and subglottic spaces which are sometimes poorly seen by laryngoscopy or by any other means. 2. Determination of invasion of the laryngeal cartilage by tumor proved to be very difficult to diagnose with CT. 3. The CT images obtained while the patient is breathing quietly, coupled with additional sections at the level of the vocal cord during slight valsalva maneuver afford good visualization of laryngeal tumors. 4. It should be emphasized that a thorough pathologic examination of extirpated specimens with serial sections is essential for laryngeal surgeons, because it is impossible to determine the patient's prognosis without microscopic demonstration of the degree of invasion.

  12. Intraoperative Recurrent Laryngeal Nerve Monitoring in a Patient with Contralateral Vocal Fold Palsy

    Directory of Open Access Journals (Sweden)

    Bub-Se Na

    2017-10-01

    Full Text Available Recurrent laryngeal nerve injury can develop following cervical or thoracic surgery; however, few reports have described intraoperative recurrent laryngeal nerve monitoring. Consensus regarding the use of this technique during thoracic surgery is lacking. We used intraoperative recurrent laryngeal nerve monitoring in a patient with contralateral vocal cord paralysis who was scheduled for completion pneumonectomy. This case serves as an example of intraoperative recurrent laryngeal nerve monitoring during thoracic surgery and supports this indication for its use.

  13. 2A4 binds soluble and insoluble light chain aggregates from AL amyloidosis patients and promotes clearance of amyloid deposits by phagocytosis †.

    Science.gov (United States)

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

    2016-09-01

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

  14. 2A4 binds soluble and insoluble light chain aggregates from AL amyloidosis patients and promotes clearance of amyloid deposits by phagocytosis †

    Science.gov (United States)

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

    2016-01-01

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

  15. Laryngeal chondrosarcoma - Ten years of experience

    Directory of Open Access Journals (Sweden)

    José Fernando dos Santos Oliveira

    2014-07-01

    Full Text Available INTRODUCTION: Laryngeal involvement by cartilaginous tumors is rare. However, although accounting for only 1% of laryngeal tumor pathology, they are the most frequently occurring non-epithelial neoplasms. The most probable location is the endolaryngeal surface of the cricoid cartilage. Their symptoms are variable, depending on the size and location, and may include hoarseness, stridor, and dyspnea. Treatment is based on surgical excision. Some centers take into account the degree of differentiation and whether it is a case of relapse when deciding to perform a radical resection. AIM: To evaluate this disease in a sample of the Portuguese population. METHODS: A review of the medical records from 2002 to 2012 by assessment of clinical processes was performed. Data on demographics, clinical treatments, and outcomes were collected. RESULTS: Six patients were included in the study. Five of them underwent total laryngectomy, and in one case, partial excision of the thyroid cartilage was performed. None of the patients had either metastases or tumor-related death. CONCLUSION: Laryngeal chondrosarcomas remain a rare disease of unknown etiology, with slow and insidious symptoms. The treatment is surgical, with favorable prognosis, and metastases rarely occur. The main concern regards their propensity to relapse.

  16. Assessment of human papilloma virus infection in adult laryngeal papilloma using a screening test.

    Science.gov (United States)

    Makiyama, Kiyoshi; Hirai, Ryoji; Matsuzaki, Hiroumi; Ikeda, Minoru

    2013-03-01

    Human papilloma virus (HPV) infection is involved in both juvenile and adult laryngeal papilloma. We wished to determine which types of adult laryngeal papilloma were clinically related to HPV infection. We hypothesized that multiple-site and recurrent papillomas would have a strong relationship to HPV and conducted the present study to test this hypothesis. Thirteen male patients with adult laryngeal papilloma who underwent resection of papilloma between August 2006 and September 2009 were studied. We examined the relationships between whether the tumor was solitary or multiple, presence or absence of recurrence after surgery, and HPV infection. High-risk HPV types (HPV-DNA types 16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, 59, and 68) and low-risk HPV types (6, 11, 42, 43, and 44) were tested by a liquid-phase hybridization method. In addition, HPV typing was performed for patients positive for low-risk HPV types. Twenty patients with laryngeal carcinoma or laryngeal leukoplakia were enrolled as the control group. In the laryngeal papilloma group, all patients tested were negative for high-risk HPV and 69.2% were positive for low-risk HPV. Typing performed for seven of the patients who tested positive for low-risk HPV showed that one patient was positive for HPV-11, whereas the remaining six patients were positive for HPV-6. All patients with recurrent laryngeal papillomatosis (RLP) were positive for low-risk HPV. All patients who were positive for low-risk HPV had RLP. Tumor samples from repeat operations were positive for low-risk HPV in all patients tested. HPV was not detected in the control group. The relationship between RLP and low-risk HPV was strong, with all cases that were positive for low-risk HPV showing recurrence. Tumor tissue resected at the time of repeat surgery was positive for low-risk HPV in all cases tested. Copyright © 2013 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  17. Asbestos, dental x-rays, tobacco, and alcohol in the epidemiology of laryngeal cancer

    International Nuclear Information System (INIS)

    Hinds, M.W.; Thomas, D.B.; O'Reilly, H.P.

    1979-01-01

    A case-control study of 47 laryngeal cancers in males of three counties of Washington State was conducted. Personal interview was used to obtain information on smoking, alcohol use, exposure to asbestos, and other substances, and x-rays of the head and neck area. Smoking and alcohol consumption were found to increase risk of laryngeal cancer independently, with a clear dose-response relationship. Neither asbestos exposure nor exposure to other substances was found to significantly increase the risk of laryngeal cancer, although the relative risk with asbestos exposure was 1.75. Lifetime history of exposure to dental x-rays on five or more occasions was associated with significantly increased risk of laryngeal cancer among heavy smokers but not among light smokers. The importance of tobacco and alcohol in the epidemiology of laryngeal cancer was re-affirmed, the importance of asbestos exposure was brought into question, and a possible relationship of laryngeal cancer with exposure to dental x-rays among heavy smokers was demonstrated

  18. Nuclear medicine imaging of locally advanced laryngeal and hypopharyngeal cancer

    Science.gov (United States)

    Medvedeva, A.; Chernov, V.; Zeltchan, R.; Sinilkin, I.; Bragina, O.; Chijevskaya, S.; Choynzonov, E.; Goldberg, A.

    2017-09-01

    The diagnostic capabilities of nuclear medicine imaging in the detection and assessment of the spread of laryngeal/hypopharyngeal cancer were studied. A total of 40 patients with histologically verified laryngeal and hypopharyngeal cancer and 20 patients with benign laryngeal lesions were included into the study. Submucosal injections of 99mTc-MIBI and 99mTc-Alotech were made around the tumor. Single photon emission computed tomography (SPECT) was performed 20 minutes after the injection of 99mTc-MIBI. Sentinel lymph nodes (SLNs) were detected in 26 patients. In 18 hours after the injection of 99mTc-Alotech, SPECT was performed. In 24 hours after the injection of 99mTc-Alotech, intraoperative SLN detection was performed using Gamma Finder II. SPECT with 99mTc-MIBI revealed laryngeal and hypopharyngeal tumors in 38 of the 40 patients. The 99mTc-MIBI uptake in metastatic lymph nodes was visualized in 2 (17%) of the 12 patients. Twenty eight SLNs were detected by SPECT and 31 SLNs were identified using the intraoperative gamma probe. The percentage of 99mTc-Alotech in the SLN was 5-10% of the radioactivity in the injection site by SPECT and 18-33% by intraoperative gamma probe detection. Thus, SPECT with 99mTc-MIBI is an effective tool for the diagnosis of laryngeal/hypopharyngeal cancer. The sensitivity, specificity and accuracy of this technique were 95%, 80% and 92%, respectively. The use of 99mTc-Alotech for the detection of SLNs in patients with laryngeal/hypopharyngeal cancer is characterized by 92.8% sensitivity.

  19. Transoral robotic-assisted laryngeal cleft repair in the pediatric patient.

    Science.gov (United States)

    Leonardis, Rachel L; Duvvuri, Umamaheswar; Mehta, Deepak

    2014-09-01

    To assess the feasibility of performing robotic-assisted laryngeal cleft repair in the pediatric population. Retrospective chart review at a tertiary academic children's hospital. All patients underwent transoral robotic-assisted laryngeal cleft repair from March 2011 to June 2013. Demographics, robotic docking time, operative time, and postoperative course and swallowing function were collected and analyzed. Five children, three male and two female, underwent successful transoral robotic-assisted laryngeal cleft repair for closure of a type I laryngeal cleft. Mean age at time of surgery was 21.6 months (standard deviation 6.1 months; range, 15-29 months). From case 1 to case 5, robotic docking time (18-10 minutes), robotic operative time (102-36 minutes), and total operating room time (173-105 minutes) decreased. There were no complications with time until extubation (range, 2-3 days), length of intensive care unit stay (range, 3-4 days), and total hospital stay (range, 3-5 days) within acceptable range following laryngeal cleft repair. Modified barium swallow (two patients) or fiberoptic endoscopic evaluation of swallowing (three patients) was performed postoperatively, with all patients showing complete resolution of penetration and aspiration. In addition, all patients experienced subjective resolution of dysphagia and/or choking with feeds postoperatively. Transoral robotic-assisted laryngeal cleft repair may offer specific advantages over a traditional endoscopic approach. In our experience, the procedure was well tolerated and associated with definitive surgical cure in all patients. The scope of robotic technology continually expands and should be considered a feasible tool at an institution-based level. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

  20. Type I-II laryngeal cleft: clinical course and outcome.

    Science.gov (United States)

    Slonimsky, Guy; Carmel, Eldar; Drendel, Michael; Lipschitz, Noga; Wolf, Michael

    2015-04-01

    Laryngeal cleft (LC) is a rare congenital anomaly manifesting in a variety of symptoms, including swallowing disorders and aspirations, dyspnea, stridor and hoarseness. The mild forms (types I-II) may be underdiagnosed, leading to protracted symptomatology and morbidity. To evaluate the diagnostic process, clinical course, management and outcome in children with type I-II laryngeal clefts. We conducted a retrospective case analysis for the years 2005-2012 in a tertiary referral center. Seven children were reviewed: five boys and two girls ranging in age from birth to 5 years. The most common presenting symptoms were cough, aspirations and pneumonia. Evaluation procedures included fiber-optic laryngoscopy (FOL), direct laryngoscopy (DL) and videofluoroscopy. Other pathologies were seen in three children. Six children underwent successful endoscopic surgery and one child was treated conservatively. The postoperative clinical course was uneventful in most of the cases. Types I-II LC should be considered in the differential diagnosis of children presenting with protracted cough and aspirations. DL is crucial for establishing the diagnosis. Endoscopic surgery is safe and should be applied promptly when conservative measures fail.

  1. Reversing Age Related Changes of the Laryngeal Muscles by Chronic Electrostimulation of the Recurrent Laryngeal Nerve.

    Directory of Open Access Journals (Sweden)

    Michael Karbiener

    Full Text Available Age related atrophy of the laryngeal muscles -mainly the thyroarytenoid muscle (TAM- leads to a glottal gap and consequently to a hoarse and dysphonic voice that significantly affects quality of life. The aim of our study was to reverse this atrophy by inducing muscular hypertrophy by unilateral functional electrical stimulation (FES of the recurrent laryngeal nerve (RLN in a large animal model using aged sheep (n = 5. Suitable stimulation parameters were determined by fatiguing experiments of the thyroarytenoid muscle in an acute trial. For the chronic trial an electrode was placed around the right RLN and stimulation was delivered once daily for 29 days. We chose a very conservative stimulation pattern, total stimulation time was two minutes per day, or 0.14% of total time. Overall, the mean muscle fiber diameter of the stimulated right TAM was significantly larger than the non-stimulated left TAM (30μm±1.1μm vs. 28μm±1.1 μm, p<0.001. There was no significant shift in fiber type distribution as judged by immunohistochemistry. The changes of fiber diameter could not be observed in the posterior cricoarytenoid muscle (PCAM. FES is a possible new treatment option for reversing the effects of age related laryngeal muscle atrophy.

  2. 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

  3. Aerodynamic and Nonlinear Dynamic Acoustic Analysis of Tension Asymmetry in Excised Canine Larynges

    Science.gov (United States)

    Devine, Erin E.; Bulleit, Erin E.; Hoffman, Matthew R.; McCulloch, Timothy M.; Jiang, Jack J.

    2012-01-01

    Purpose: To model tension asymmetry caused by superior laryngeal nerve paralysis (SLNP) in excised larynges and apply perturbation, nonlinear dynamic, and aerodynamic analyses. Method: SLNP was modeled in 8 excised larynges using sutures and weights to mimic cricothyroid (CT) muscle function. Weights were removed from one side to create tension…

  4. Laryngeal neuropathy of Charcot-Marie-Tooth disease: further observations and novel mutations associated with vocal fold paresis.

    Science.gov (United States)

    Benson, Brian; Sulica, Lucian; Guss, Joel; Blitzer, Andrew

    2010-02-01

    To describe and define laryngeal neuropathy in Charcot-Marie-Tooth (CMT) disease. Retrospective record review from a university laryngology practice. Four adult CMT patients presented with laryngeal symptoms. Three patients exhibited bilateral vocal fold palsy, in each case with more severe hypomobility on the left. One case exhibited an isolated left vocal fold palsy. All patients complained of hoarseness and stridor, three had dyspnea, two patients had dysphagia, and one had obstructive sleep apnea (OSA). One patient has required airway surgery to date. Genetic testing revealed known sequence alterations in one case and sequence alterations previously not associated with laryngeal dysfunction in two cases. One case was familial and two were sporadic; information is not available in a fourth. The clinical course of the cases suggests slowly progressive neuropathy that appears to be nerve length dependent. The lack of severe respiratory distress despite dense bilateral paresis is consistent with existing reports and with the reported low rate of tracheostomy in adults with laryngeal manifestations of CMT. Genetic testing does not currently inform expectations or management of laryngeal disease. Dyspnea, dysphagia, and OSA symptoms in patients with CMT require careful laryngologic evaluation.

  5. Changes in the frequency of swallowing during electrical stimulation of superior laryngeal nerve in rats.

    Science.gov (United States)

    Tsuji, Kojun; Tsujimura, Takanori; Magara, Jin; Sakai, Shogo; Nakamura, Yuki; Inoue, Makoto

    2015-02-01

    The aim of the present study was to investigate the adaptation of the swallowing reflex in terms of reduced swallowing reflex initiation following continuous superior laryngeal nerve stimulation. Forty-four male Sprague Dawley rats were anesthetized with urethane. To identify swallowing, electromyographic activity of the left mylohyoid and thyrohyoid muscles was recorded. To evoke the swallowing response, the superior laryngeal nerve (SLN), recurrent laryngeal nerve, or cortical swallowing area was electrically stimulated. Repetitive swallowing evoked by continuous SLN stimulation was gradually reduced, and this reduction was dependent on the resting time duration between stimulations. Prior SLN stimulation also suppressed subsequent swallowing initiation. The reduction in evoked swallows induced by recurrent laryngeal nerve or cortical swallowing area stimulation was less than that following superior laryngeal nerve stimulation. Decerebration had no effect on the reduction in evoked swallows. Prior subthreshold stimulation reduced subsequent initiation of swallowing, suggesting that there was no relationship between swallowing movement evoked by prior stimulation and the subsequent reduction in swallowing initiation. Overall, these data suggest that reduced sensory afferent nerve firing and/or trans-synaptic responses, as well as part of the brainstem central pattern generator, are involved in adaptation of the swallowing reflex following continuous stimulation of swallow-inducing peripheral nerves and cortical areas. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. [Establishment of a comprehensive database for laryngeal cancer related genes and the miRNAs].

    Science.gov (United States)

    Li, Mengjiao; E, Qimin; Liu, Jialin; Huang, Tingting; Liang, Chuanyu

    2015-09-01

    By collecting and analyzing the laryngeal cancer related genes and the miRNAs, to build a comprehensive laryngeal cancer-related gene database, which differs from the current biological information database with complex and clumsy structure and focuses on the theme of gene and miRNA, and it could make the research and teaching more convenient and efficient. Based on the B/S architecture, using Apache as a Web server, MySQL as coding language of database design and PHP as coding language of web design, a comprehensive database for laryngeal cancer-related genes was established, providing with the gene tables, protein tables, miRNA tables and clinical information tables of the patients with laryngeal cancer. The established database containsed 207 laryngeal cancer related genes, 243 proteins, 26 miRNAs, and their particular information such as mutations, methylations, diversified expressions, and the empirical references of laryngeal cancer relevant molecules. The database could be accessed and operated via the Internet, by which browsing and retrieval of the information were performed. The database were maintained and updated regularly. The database for laryngeal cancer related genes is resource-integrated and user-friendly, providing a genetic information query tool for the study of laryngeal cancer.

  7. 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...

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

    Directory of Open Access Journals (Sweden)

    Barai Sukanta

    2005-01-01

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

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

    Science.gov (United States)

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

    2018-07-01

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

  10. Development of a system to monitor laryngeal movement during swallowing using a bend sensor.

    Directory of Open Access Journals (Sweden)

    Qiang Li

    Full Text Available BACKGROUND: Swallowing dysfunction (also known as dysphagia, which results in a deterioration of nutritional intake, slows rehabilitation and causes aspiration pneumonia, is very common following neurological impairments. Although videofluorographic (VF examination is widely used for detecting aspiration, an objective and non-invasive method for assessing swallowing function has yet to be established because of a lack of adequate devices and protocols. In this paper, a bend sensor whose resistance is altered by bending was introduced to monitor swallowing-related laryngeal movement. METHODS: Six healthy male volunteers were recruited in the present study. Specific time points on the signal waveform produced by the bend sensor were defined to describe laryngeal movement by differential analysis. Additionally, the physiological significance of the obtained waveform was confirmed by analyzing the sequential correlations between the signal waveform from the bend sensor and hyoid bone kinetics simultaneously recorded by VF. RESULTS: Seven time points were successfully defined on the signal waveform to reference laryngeal movement. Each time point was well correlated with certain VF events, with evidence of no significant time lags, and there were positive correlations between waveform time points and matched VF events. Furthermore, obvious similarities were noticed between the duration of each phase on the signal waveform and the duration of the matched hyoid bone activity. CONCLUSIONS: The present monitoring system using a bend sensor might be useful for observing the temporal aspects of laryngeal movement during swallowing, and it was well coordinated with hyoid bone movement.

  11. Can flow-volume loops be used to diagnose exerciseinduced laryngeal obstructions?

    DEFF Research Database (Denmark)

    Christensen, Pernille Melia; Maltbæk, Niels; Jørgensen, Inger M

    2013-01-01

    BACKGROUND: Pre- and post-exercise flow-volume loops are often recommended as an easy non-invasive method for diagnosing or excluding exercise-induced laryngeal obstructions in patients with exercise-related respiratory symptoms. However, at present there is no evidence for this recommendation...

  12. The role of computed tomography in the laryngeal injury

    International Nuclear Information System (INIS)

    Bae, Hoon Sik

    1984-01-01

    Computed tomography of the larynx represents a major advance in laryngology. Even in severe injury the larynx can be examined easily and conveniently by CT at the same time as the brain and facial structures without moving the patient, who need only lie down and breathe quietly during the study. Computed tomography permitted a much more detailed appraisal of laryngeal dysfunction in patients with blunt laryngeal trauma (3 cases) and strangulation injury (2 cases). Computed tomography of the larynx undoubtedly played a determinant role in patient management. Computed tomography was helpful in evaluating the laryngeal cartilages and deep spaces of the larynx which was difficult to examine by the laryngoscope. Follow-up computed tomography made it possible to evaluate the postoperative results

  13. Tracheobronchial Amyloidosis Mimicking Tracheal Tumor

    Directory of Open Access Journals (Sweden)

    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.

  14. 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.

  15. ['Laryngeal neuropathy' and 'irritable larynx syndrome': synonyms or distinct entities?].

    Science.gov (United States)

    Meyer, S; Ptok, M

    2012-10-01

    The term 'laryngeal neuropathy' (LN) has first been used in veterinary medicine to describe an idiopathic and typically exercise induced inspiratory noise in horses.Nowadays, the term is often used in relation with intermittent vocal cord pareses in humans. Some authors use the term 'irritable larynx syndrome' (ILS) in a similar context. This article reviews the state of knowledge regarding LN and ILS and discusses the somewhat confusing terminology.For this systematic review a selective literature research in PubMed has been carried out.35 articles were found, which report on LN in animals and 17 articles reported on humans. 4 of these articles used the term 'irritable larynx syndrome'.Laryngeal neuropathy in horses usually affects the left recurrent laryngeal nerve and results in decreased vocal cord abduction and an inspiratory roaring or whistling noise, particularly during exercise. In dogs LN has been reported to also occur bilaterally. In association with humans LN has not been defined clearly in the literature. The term ILS on the other hand has only been used in relation to humans. The term describes a hypersensitivity of the laryngeal structures towards external stimuli, which causes symptoms such as dyspnea or cough among others. Sufficient knowledge does not exist for either of the 2 diseases, ILS or LN. As of yet, the term LN should not be used in human medicine to describe according symptoms of unknown aetiology. The term 'laryngeal movement disorder' seems a lot more appropriate. The symptom oriented term irritable larynx syndrome also seems suitable to describe laryngeal hypersensitivity appropriately. © Georg Thieme Verlag KG Stuttgart · New York.

  16. 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...

  17. Effect of human papilloma virus expression on clinical course of laryngeal papilloma.

    Science.gov (United States)

    Kim, Kwang Moon; Cho, Nam Hoon; Choi, Hong Shik; Kim, Young Ho; Byeon, Hyung Kwon; Min, Hyun Jin; Kim, Se-Heon

    2008-10-01

    Our observations suggest that human papilloma virus (HPV) 6/11 is the main causative agent of laryngeal papilloma and that detection of active HPV DNA expression may be helpful in identifying patients with aggressive recurrent laryngeal papilloma. HPV is assumed to be the main causative agent of this disease. We investigated the expression of the entire genotype of HPV in cases of laryngeal papilloma and correlated their expression with the clinical course of the disease. Seventy cases of laryngeal papilloma were evaluated for the presence of the HPV genome by in situ hybridization (ISH) using wide-spectrum HPV DNA probe. Specific types of HPV infection were determined by DNA ISH using type-specific HPV DNA probes (HPV 6, 11, 16, 18, 31, 33). Separate analyses were conducted comparing viral types, frequency of recurrences and duration of disease-free periods. We detected HPV DNA in 40 of the 70 laryngeal papilloma cases (57%). In particular, HPV DNA was detected in 75% of the juvenile types. There were significant associations between HPV and laryngeal papilloma (p<0.01). Among the HPV-positive cases, major specific types were HPV 6/11 (97%). Significant associations were also noted between viral expression and clinical course.

  18. Endoscopic approach for a laryngeal neoplasm in a dog

    Directory of Open Access Journals (Sweden)

    Pedro Paulo Maia Teixeira

    2015-01-01

    Full Text Available Laryngeal and tracheal tumors are rare in pets; some piece of information on their disease behavior, therapy and evolution are limited. Neoplasms in this area are a diagnostic challenge. In many cases, they can be biopsied and excised using endoscopic instruments, but there is no report of this in canines. The goal of this study is to report a successful case of a laryngeal neoplasm removal through endoscopy. A head and neck radiogram revealed a mass in the laryngeal lumen protruding into the trachea. The patient then underwent an endoscopy to confirm the radiographic diagnosis and to surgically remove the tumor. The histopathological diagnosis was poorly differentiated carcinoma. The most appropriate treatment for laryngeal tumors is the resection of the submucosa or a partial laryngectomy however, partial and total laryngectomies are associated with many postoperative complications. In contrast, the endoscopic approach allows for highly magnified visualization of the lesion in situ, which facilitates the surgical removal of the mass through videosurgery. With little manipulation of the affected area, the chances of postoperative complications are reduced, leading to a more rapid recovery.

  19. Spasmodic dysphonia: a laryngeal control disorder specific to speech.

    Science.gov (United States)

    Ludlow, Christy L

    2011-01-19

    Spasmodic dysphonia (SD) is a rare neurological disorder that emerges in middle age, is usually sporadic, and affects intrinsic laryngeal muscle control only during speech. Spasmodic bursts in particular laryngeal muscles disrupt voluntary control during vowel sounds in adductor SD and interfere with voice onset after voiceless consonants in abductor SD. Little is known about its origins; it is classified as a focal dystonia secondary to an unknown neurobiological mechanism that produces a chronic abnormality of laryngeal motor neuron regulation during speech. It develops primarily in females and does not interfere with breathing, crying, laughter, and shouting. Recent postmortem studies have implicated the accumulation of clusters in the parenchyma and perivascular regions with inflammatory changes in the brainstem in one to two cases. A few cases with single mutations in THAP1, a gene involved in transcription regulation, suggest that a weak genetic predisposition may contribute to mechanisms causing a nonprogressive abnormality in laryngeal motor neuron control for speech but not for vocal emotional expression. Research is needed to address the basic cellular and proteomic mechanisms that produce this disorder to provide intervention that could target the pathogenesis of the disorder rather than only providing temporary symptom relief.

  20. The Recurrent Laryngeal Nerve and Thyroid Surgery; Who to Scope ...

    African Journals Online (AJOL)

    Review with Meta Analysis of Studies Comparing. Intra-Operative Neuromonitoring of Recurrent. Laryngeal Nerves Versus Visualization Alone. During Thyroid Surgery. J Surg Res. 2014; 181(1):. 152-61. 6. Hermann M, Alk G, Roka R, et al. Laryngeal. Recurrent Nerve Injury in Surgery for Benign. Thyroid Diseases: Effect of ...

  1. Application of mitomycin C after endoscopic lysis of congenital laryngeal web combined with epiglottic hypoplasia in a middle-aged man.

    Science.gov (United States)

    Roh, Jong-Lyel

    2006-04-01

    Laryngeal webs and epiglottic hypoplasias are uncommon congenital anomalies. Anterior glottic web combined with epiglottic hypoplasia was found in a middle-aged man presenting with hoarseness and dyspnea on exertion. This can be considered as a unique isolated defect of the larynx during early fetal development. The laryngeal web can be successfully treated in a single stage with endoscopic lysis and topical application of mitomycin C for prevention of anterior glottic restenosis. This case and prior reports suggest that the novel approach may be effective in the treatment of laryngeal webs.

  2. Bronchial or Laryngeal Obstruction Induced by Exercise?

    Directory of Open Access Journals (Sweden)

    Ayoub Bey

    2017-06-01

    Full Text Available A child suspected of exercise-induced laryngeal obstruction and asthma is examined by laryngoscopy and respiratory resistance (Rrs after exercise challenge. Immediately at exercise cessation, the visualized adduction of the larynx in inspiration is reflected in a paroxystic increase in Rrs. While normal breathing has apparently resumed later on during recovery from exercise, the pattern of Rrs in inspiration is observed to reoccur following a deep breath or swallowing. The procedure may thus help diagnosing the site of exercise-induced obstruction when laryngoscopy is not available and identify re-inducers of laryngeal dysfunction.

  3. 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.

  4. Laryngeal obstruction caused by lymphoma in an adult dairy cow

    OpenAIRE

    Lardé, Hélène; Nichols, Sylvain; Babkine, Marie; Chénier, Sonia

    2014-01-01

    A Holstein cow was presented for inspiratory dyspnea. Endoscopic evaluation revealed swollen arytenoids and a presumptive diagnosis of bilateral arytenoidal chondritis was made. A partial arytenoidectomy was performed, the right arytenoid was submitted for histopathology, and a diagnosis of laryngeal lymphoma was made. Due to the poor prognosis, the cow was euthanized.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-06-15

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

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. The Use of Cryotherapy for Papilloma and Early Laryngeal Cancers: Long-term Results.

    Science.gov (United States)

    Benninger, Michael S; Derakhshan, Adeeb; Milstein, Claudio F

    2015-07-01

    Retrospective chart review. To determine the efficacy of adjuvant cryotherapy in the treatment of early glottic cancer and laryngeal papillomatosis. The use of cryotherapy in conjunction with traditional modalities has recently been proposed to improve voice outcomes in patients with early laryngeal cancer as compared to pretreatment conditions. This study investigates its utility in improving oncological outcomes and decreasing recurrences of laryngeal papillomatosis. Patients with either early glottic cancer or laryngeal papillomatosis that received cryotherapy as part of their surgical regimen were investigated. All patients were seen at a large tertiary care center within a 10-year window. Demographic data were collected and all postoperative notes were reviewed. Recurrences of the laryngeal cancer were noted, as was the duration of time between successive papillomatosis operations. The charts of 54 glottic cancer and 29 papillomatosis patients that received cryotherapy were reviewed. One patient from the papillomatosis cohort was excluded from statistical analysis due to lack of follow-up. Overall, 16 (30%) of the laryngeal cancer patient experienced a malignant recurrence. The overall 5-year survival of these patients was 98% and the 5-year disease-free survival was 74%. The use of adjuvant cryotherapy in the treatment of laryngeal papillomatosis extended the duration of time between surgeries by an average of 79 days (P=.23). The use of adjuvant cryotherapy in the treatment of early glottic cancer does not improve the rate of carcinoma recurrences. Additionally, cryotherapy does not result in a statistically significant increase in the duration of disease-free period for laryngeal papillomatosis patients, although the observed increase may be clinically important. © The Author(s) 2015.

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

    International Nuclear Information System (INIS)

    Goebel, R.

    1976-01-01

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

  10. Neuronal Activation in the Medulla Oblongata during Selective Elicitation of the Laryngeal Adductor Response

    Science.gov (United States)

    Ambalavanar, Ranjinidevi; Tanaka, Yasumasa; Selbie, W. Scott; Ludlow, Christy L.

    2008-01-01

    Swallow and cough are complex motor patterns elicited by rapid and intense electrical stimulation of the internal branch of the superior laryngeal nerve (ISLN). The laryngeal adductor response (LAR) includes only a laryngeal response, is elicited by single stimuli to the ISLN, and is thought to represent the brain stem pathway involved in laryngospasm. To identify which regions in the medulla are activated during elicitation of the LAR alone, single electrical stimuli were presented once every 2 s to the ISLN. Two groups of 5 cats each were studied; an experimental group with unilateral ISLN stimulation at 0.5 Hz and a surgical control group. Three additional cats were studied to evaluate whether other oral, pharyngeal or respiratory muscles were activated during ISLN stimulation eliciting LAR. We quantified up to 22 sections for each of 14 structures in the medulla to determine if regions had increased Fos-like immunoreactive neurons in the experimental group. Significant increases (p medulla. PMID:15212423

  11. Evaluating Post-Radiotherapy Laryngeal Function with Laryngeal Videostroboscopy in Early Stage Glottic Cancer

    Directory of Open Access Journals (Sweden)

    Ariel E. Marciscano

    2017-06-01

    Full Text Available ObjectiveDysphonia is common among patients with early stage glottic cancer. Laryngeal videostroboscopy (LVS has not been routinely used to assess post-radiotherapy (RT voice changes. We hypothesized that LVS would demonstrate improvement in laryngeal function after definitive RT for early-stage glottic cancer.Study designBlinded retrospective review of perceptual voice and stroboscopic parameters for patients with early glottic cancer and controls.SettingHigh-volume, single-institution academic medical center.Subjects and methodsFifteen patients underwent RT for Tis-T2N0M0 glottic cancer and were evaluated with serial LVS exams pre- and post-RT. Stroboscopic assessment included six parameters: vocal fold (VF vibration, VF mobility, erythema/edema, supraglottic compression, glottic closure, and secretions. Grade, roughness, breathiness, asthenia, strain (GRBAS voice perceptual scale was graded in tandem with LVS score. Assessments were grouped by time interval from RT: pre-RT, 0–4, 4–12, and >12 months post-RT.Results60 LVS exams and corresponding GRBAS assessments were reviewed. There were significant improvements in ipsilateral VF motion (P = 0.03 and vibration (P = 0.001 and significant worsening in contralateral VF motion (P < 0.001 and vibration (P = 0.008 at >12 months post-RT. Glottic closure significantly worsened, most prominent >12 months post-RT (P = 0.01. Composite GRBAS scores were significantly improved across all post-RT intervals.ConclusionLVS proved to be a robust tool for assessing pre- and post-RT laryngeal function. We observed post-RT improvement in ipsilateral VF function, a decline in contralateral VF function, and decreased glottic closure. These results demonstrate that LVS can detect meaningful changes in VF and glottic function and support its use for post-RT evaluation of glottic cancer patients.

  12. Impacted Laryngeal Foreign Body in a Child: A Diagnostic and ...

    African Journals Online (AJOL)

    Impacted laryngeal foreign body could lead to catastrophic consequences if appropriate diagnostic and therapeutic procedures are not promptly instituted. A case of 4‑year‑old child who presented with a 4‑day history of probable ingestion or aspiration of a pen part and history of occasional noisy breathing on exertion and ...

  13. Some Comparative Anatomical and Histological Studies on the Laryngeal Cartilages of Buffaloes, Camels and Donkeys

    Directory of Open Access Journals (Sweden)

    Eman A. Eshra

    2016-01-01

    Full Text Available Comparative studies concerned the upper air ways of domestic animals are few. So this study was carried out to compare between the larynx of buffaloes, camels and donkeys. The present investigation was carried out on 39 larynxes, 13 larynxes (7 males, 6 females of each species. Ten heads from each species were used for gross anatomical study; the remained three heads were used for the histological study. Results revealed that, the laryngeal cartilages of the three species were consisted of three single cartilages; the thyroid, the cricoid and the epiglottis, and two paired cartilages; the arytenoid and the corniculate. The cuneiform cartilages were paired cartilages present only in the larynx of the donkey. Thyroid, arytenoid and cricoid cartilages were of hyaline type, while the epiglottis, cuniform and corniculate cartilages and the vocal process of the arytenoid cartilage were of elastic type. The laryngeal epithelium of aditus laryngis, greater part of epiglottis and vocal folds was lined by non-keratinized stratified squamous epithelium. The remained parts of laryngeal epithelium from base of epiglottis and entire parts caudal to vocal folds were lined by pseudostratified columnar ciliated epithelium with goblet cells. The laryngeal glands of lamina propria were of mixed types in buffaloes and donkeys but in camels it was pure mucous glands. This study will fill a gap in the field of comparative anatomy and help other clinical investigation applied on these animals.

  14. [Evaluation and treatment of children's laryngeal clefts].

    Science.gov (United States)

    Chen, C; Tan, L T; Xu, Z M

    2018-01-07

    Objectives: To provide the experience about the diagnostic process and following management, and to discuss the outcome and predictors in children with laryngeal cleft (LC). Methods: A retrospective case study was conducted at an academic children's hospital. Thirty children were diagnosed as laryngeal cleft between January 2016 and April 2017.Airway evaluations were performed using both flexible and rigid endoscopy, and swallowing evaluations were performed using fiberoptic endoscopic examination of swallowing or modified barium swallow. Results: Of 30 cases, 18 were male and 12 were female, ranging in age from birth to 8 years. Two cases were diagnosed as type 0 LC, and they were offered thickened liquid without medication. Throughout follow-up, they remained asymptomatic and showed no respiratory complications. Nineteen children were diagnosed as type Ⅰ LC. Six of them were significantly improved by anti-reflux therapy and feeding instructions. Four children were concomitant with swallowing dysfunction and/or neuromuscular disorders, and they were given a tracheotomy and routine management. Another 4 children were submitted surgical repair when routine treatment failed, and their symptoms were relieved. Five children were concomitant with larygomalacia, and their symptoms were totally ameliorated by supraglottoplasty. Three children were diagnosed as type Ⅱ LC. Two of them received surgical repair and clinically improved, and the rest one was treated by anti-reflux therapy and still under follow-up. Three children were diagnosed as type Ⅲ LC. One of them was underwent surgical repair and clinically improved. Two children were tracheotomized and treated by anti-reflux therapy. Three cases were diagnosed as type Ⅳ LC at birth and no one survived. Conclusions: Laryngeal cleft is a rare congenital anomaly manifesting with a variety of symptoms, including swallowing disorder, aspirations, dyspnea, stridor and hoarseness. Diagnosis and treatment of laryngeal

  15. Reducing the negative vocal effects of superficial laryngeal dehydration with humidification.

    Science.gov (United States)

    Levendoski, Elizabeth Erickson; Sundarrajan, Anusha; Sivasankar, M Preeti

    2014-07-01

    Environmental humidification is a simple, cost-effective method believed to reduce superficial laryngeal drying. This study sought to validate this belief by investigating whether humidification treatment would reduce the negative effects of superficial laryngeal dehydration on phonation threshold pressure (PTP). Phonation threshold pressure data analysis may be vulnerable to bias because of lack of investigator blinding. Consequently, this study investigated the extent of PTP analysis reliability between unblinded and blinded investigators. Healthy male and female adults were assigned to a vocal fatigue (n = 20) or control group (n = 20) based on their responses to a questionnaire. PTP was assessed after 2 hours of mouth breathing in low humidity (dehydration challenge), following a 5-minute break in ambient humidity, and after 2 hours of mouth breathing in high humidity (humidification). PTP significantly increased following the laryngeal dehydration challenge. After humidification, PTP returned toward baseline. These effects were observed in both subject groups. PTP measurements were highly correlated between the unblinded and blinded investigator. Humidification may be an effective approach to decrease the detrimental voice effects of superficial laryngeal dehydration. These data lay the foundation for future investigations aimed at preventing and treating the negative voice changes associated with chronic, surface laryngeal drying.

  16. Spasmodic Dysphonia: a Laryngeal Control Disorder Specific to Speech

    Science.gov (United States)

    Ludlow, Christy L.

    2016-01-01

    Spasmodic dysphonia (SD) is a rare neurological disorder that emerges in middle age, is usually sporadic, and affects intrinsic laryngeal muscle control only during speech. Spasmodic bursts in particular laryngeal muscles disrupt voluntary control during vowel sounds in adductor SD and interfere with voice onset after voiceless consonants in abductor SD. Little is known about its origins; it is classified as a focal dystonia secondary to an unknown neurobiological mechanism that produces a chronic abnormality of laryngeal motor neuron regulation during speech. It develops primarily in females and does not interfere with breathing, crying, laughter, and shouting. Recent postmortem studies have implicated the accumulation of clusters in the parenchyma and perivascular regions with inflammatory changes in the brainstem in one to two cases. A few cases with single mutations in THAP1, a gene involved in transcription regulation, suggest that a weak genetic predisposition may contribute to mechanisms causing a nonprogressive abnormality in laryngeal motor neuron control for speech but not for vocal emotional expression. Research is needed to address the basic cellular and proteomic mechanisms that produce this disorder to provide intervention that could target the pathogenesis of the disorder rather than only providing temporary symptom relief. PMID:21248101

  17. Radiographic assessment of laryngeal reflexes in ketamine-anesthetized cats

    International Nuclear Information System (INIS)

    Robinson, E.P.; Johnston, G.R.

    1986-01-01

    The competence of the laryngeal closure reflexes of cats anesthetized with ketamine was assessed. Radiographic evaluations of the respiratory and digestive tracts were made after colloidal barium suspension was instilled into the pharynges of conscious and ketamine-anesthetized cats. There was a significant ketamine dose-related response of spread of contrast medium into the supraglottic laryngeal area and into the stomach 2 minutes after contrast medium was instilled into the pharynx (P less than 0.05). Cats did not aspirate contrast medium into the lower respiratory tract. Three ketamine-anesthetized cats aspirated contrast medium into the subglottic area of the larynx, and 2 of these cats also aspirated the material into the cranial part of the trachea. This material was coughed up and swallowed within 5 minutes. Transit time of contrast medium into the stomach seemed to be increased in 11 of the 15 cats given the larger dosages of ketamine (24, 36, 48 mg/kg of body weight), compared with that in conscious cats and those given ketamine at 12 mg/kg. Competent laryngeal protective reflexes in cats can be maintained with ketamine anesthesia. Contrast radiography could be used as a diagnostic aid in ketamine-anesthetized cats suspected of laryngeal reflex abnormalities

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

    Directory of Open Access Journals (Sweden)

    Per Westermark

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

  19. [The forensic medical assessment of the results of a study of laryngeal injuries in blunt trauma to the neck].

    Science.gov (United States)

    Svetlakov, A V; Korenev, S A; Akishin, A N

    1997-01-01

    Presents the methodological principles and succession of examination of the basic formations of the larynx in cases with blunt injuries of the neck. Describes variants of anatomic structure of the sublingual bone and laryngeal cartilages influencing the morphology of injuries thereof. Offers differential diagnostic criteria of various mechanisms of fractures of the basic formations and recommendations on medical criminological assessment of laryngeal injuries.

  20. Diode Laser for Laryngeal Surgery: a Systematic Review.

    Science.gov (United States)

    Arroyo, Helena Hotz; Neri, Larissa; Fussuma, Carina Yuri; Imamura, Rui

    2016-04-01

    Introduction The diode laser has been frequently used in the management of laryngeal disorders. The portability and functional diversity of this tool make it a reasonable alternative to conventional lasers. However, whether diode laser has been applied in transoral laser microsurgery, the ideal parameters, outcomes, and adverse effects remain unclear. Objective The main objective of this systematic review is to provide a reliable evaluation of the use of diode laser in laryngeal diseases, trying to clarify its ideal parameters in the larynx, as well as its outcomes and complications. Data Synthesis We included eleven studies in the final analysis. From the included articles, we collected data on patient and lesion characteristics, treatment (diode laser's parameters used in surgery), and outcomes related to the laser surgery performed. Only two studies were prospective and there were no randomized controlled trials. Most of the evidence suggests that the diode laser can be a useful tool for treatment of different pathologies in the larynx. In this sense, the parameters must be set depending on the goal (vaporization, section, or coagulation) and the clinical problem. The literature lacks studies on the ideal parameters of the diode laser in laryngeal surgery. The available data indicate that diode laser is a useful tool that should be considered in laryngeal surgeries. Thus, large, well-designed studies correlated with diode compared with other lasers are needed to better estimate its effects.

  1. Treatment of Laryngeal Radionecrosis with Hyperbaric Oxygen Therapy: A Case Report

    Directory of Open Access Journals (Sweden)

    Ying-Che Hsu

    2005-02-01

    Full Text Available An 81-year-old male with early-stage laryngeal carcinoma had been treated with 60 Gy curative radiotherapy. He complained of a sore throat, foul odor in the mouth, progressive dyspnea, and fever 2 months after the completion of radiotherapy. Direct laryngoscopy revealed narrowing of the glottis with diffuse ulcerative necrotic tissue. Biopsies at multiple sites and pathology revealed intense coagulation necrosis with complete denudation of covering epithelium without any malignancy. Since laryngeal radionecrosis was suspected, the patient received hyperbaric oxygen (HBO therapy 40 times for 1 hour of 100% O2 at 2 atm absolute pressure. His clinical symptoms gradually improved and repeated endolaryngeal biopsies were undertaken near the end of HBO therapy and again 6 months later. The patient's larynx healed completely with diffuse fibrosis and no malignant cells were found on pathology. Radionecrosis must be differentiated from cancer recurrence following curative radiotherapy for early laryngeal cancer. HBO therapy could be a useful treatment adjunct for laryngeal radionecrosis.

  2. Increased vocal intensity due to the Lombard effect in speakers with Parkinson's disease: simultaneous laryngeal and respiratory strategies.

    Science.gov (United States)

    Stathopoulos, Elaine T; Huber, Jessica E; Richardson, Kelly; Kamphaus, Jennifer; DeCicco, Devan; Darling, Meghan; Fulcher, Katrina; Sussman, Joan E

    2014-01-01

    The objective of the present study was to investigate whether speakers with hypophonia, secondary to Parkinson's disease (PD), would increases their vocal intensity when speaking in a noisy environment (Lombard effect). The other objective was to examine the underlying laryngeal and respiratory strategies used to increase vocal intensity. Thirty-three participants with PD were included for study. Each participant was fitted with the SpeechVive™ device that played multi-talker babble noise into one ear during speech. Using acoustic, aerodynamic and respiratory kinematic techniques, the simultaneous laryngeal and respiratory mechanisms used to regulate vocal intensity were examined. Significant group results showed that most speakers with PD (26/33) were successful at increasing their vocal intensity when speaking in the condition of multi-talker babble noise. They were able to support their increased vocal intensity and subglottal pressure with combined strategies from both the laryngeal and respiratory mechanisms. Individual speaker analysis indicated that the particular laryngeal and respiratory interactions differed among speakers. The SpeechVive™ device elicited higher vocal intensities from patients with PD. Speakers used different combinations of laryngeal and respiratory physiologic mechanisms to increase vocal intensity, thus suggesting that disease process does not uniformly affect the speech subsystems. Readers will be able to: (1) identify speech characteristics of people with Parkinson's disease (PD), (2) identify typical respiratory strategies for increasing sound pressure level (SPL), (3) identify typical laryngeal strategies for increasing SPL, (4) define the Lombard effect. Copyright © 2014 Elsevier Inc. All rights reserved.

  3. 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

  4. Recurrent respiratory papillomatosis: HPV genotypes and risk of high-grade laryngeal neoplasia.

    Directory of Open Access Journals (Sweden)

    Turid Omland

    Full Text Available Patients with recurrent respiratory papillomatosis (RRP in Norway treated between 1987 and 2009 were recruited to this cohort study. They were followed from disease onset and data recorded until January 2012. Here, we describe the distribution of human papillomavirus (HPV genotypes, the prevalence of multiple HPV infections, and the risk of high-grade laryngeal neoplasia and respiratory tract invasive carcinoma in a large cohort of patients with RRP. We also examined whether HPV genotype, gender, age or clinical course are risk factors for this development. Clinical records and histological specimens were reviewed. Using formalin-fixed paraffin-embedded biopsies, HPV genotyping were performed by quantitative polymerase chain reaction assays identifying 15 HPV types. HPV-negative specimens were analyzed by metagenomic sequencing. Paraffin blocks were available in 224/238 patients. The DNA quality was approved in 221/224 cases. HPV DNA was detected in 207/221 patients and all were HPV 6 or HPV 11 positive, comprising HPV 6 in 133/207, HPV 11 in 40/207 cases and HPV 6/11 in 15/207 cases. Co-infection with one or two high-risk HPV types together with HPV 6 or HPV 11 was present in 19/207 patients. Metagenomic sequencing of 14 HPV-negative specimens revealed HPV 8 in one case. In total, 39/221 patients developed high-grade laryngeal neoplasia. 8/221 patients developed carcinoma of the respiratory tract (six patients with laryngeal carcinoma and two patients with lung carcinoma. High-grade laryngeal neoplasias were found more frequently in HPV-negative versus HPV-positive patients, (RR = 2.35, 95% CI 1.1, 4.99, as well as respiratory tract carcinomas (RR = 48, 95% CI 10.72, 214.91. In summary, the majority of RRP were associated with HPV 6 and/or 11. HPV-negative RRP biopsies occurred more frequently in adult-onset patients, and were associated with an increased risk of laryngeal neoplasia and carcinoma in the respiratory tract.

  5. Study on the correlation between extracellular matrix protein-1 and the growth, metastasis and angiogenesis of laryngeal carcinoma.

    Science.gov (United States)

    Meng, Xin-Yu; Liu, Juan; Lv, Feng; Liu, Ming-Qiu; Wan, Jing-Ming

    2015-01-01

    To investigate the correlation between extracellular matrix protein-1 (ECM1) and the growth, metastasis and angiogenesis of laryngeal carcinoma. Forty-five samples with laryngeal benign and malignant tumors confirmed by pathology in Laiwu City People's Hospital from March 2006 to March 2011 were collected, in which there were 29 cases with laryngeal carcinoma and 16 with benign tumors. The expression of ECM1 and factor VIII-related antigens in patients with laryngeal carcinoma and those with benign tumors was respectively detected using immunohistochemical method, and the correlation between ECM1 staining grade and microvessel density (MVD) was analyzed. In laryngeal carcinoma tissue, ECM1 was mainly expressed in cytoplasm, less in cytomembrane or intercellular substance. With abundant expression in the tissue of laryngeal benign tumors (benign mesenchymoma and hemangioma), ECM1 was primarily expressed in the connective tissue, which was different from the expression in laryngeal carcinoma tissue. The proportion of positive ECM1 staining (++) in patients with laryngeal carcinoma was dramatically higher than those with benign tumors (pcorrelation analysis revealed that ECM1 staining grade in laryngeal carcinoma tissue had a significantly-positive correlation with MVD (r=0.866, p=0.000). ECM1 expression in laryngeal carcinoma is closely associated with tumor cell growth, metastasis and angiogenesis, which can be considered as an effective predictor in the occurrence and postoperative recurrence of laryngeal carcinoma.

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

    Science.gov (United States)

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

    2001-03-01

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

  7. [Role of gastroesophageal reflux in acute recurrent laryngeal dyspnea in children].

    Science.gov (United States)

    Andrieu-Guitrancourt, J; Dehesdin, D; Le Luyer, B; Fouin, M; Peron, J M

    1984-01-01

    Recurrent laryngeal croup (RLC) in children is rarely discussed unless permanent laryngeal obstruction is involved or there is an allergic basis. An atypical case is described where gastro-esophageal reflux (GER) was not only associated with but was also directly related to the provocation of each period. A series of 15 patients with RLC were investigated for the presence of GER, and this was confirmed in 7 cases. These findings suggest that GER may be responsible for the onset of RLC, as is now admitted for numerous respiratory manifestations in children. Moreover, GER may be implicated in the course of various laryngeal obstructive lesions, particularly subglottic angioma.

  8. Voice-related of quality of life after treatment for laryngeal cancer

    International Nuclear Information System (INIS)

    Oridate, Nobuhiko; Furuta, Yasushi; Homma, Akihiro; Suzuki, Seigo; Suzuki, Fumiyuki; Hatakeyama, Hiromitsu; Taki, Shigenari; Sakashita, Tomohiro; Fukuda, Satoshi

    2007-01-01

    There are many methods of treating laryngeal cancers: definitive irradiation with or without concurrent chemotherapy, endoscopic laser surgery, partial surgery techniques, and total laryngectomy. Few studies have been reported on vocal function and quality of life of patients after the definitive treatment for laryngeal cancer, using single voice related QOL measures. The aim of this study was to examine voice related QOL in patients treated with various modalities using a Voice-Related Quality of Life (V-RQOL) questionnaire as well as Voice Handicap Index-10 (VHI-10) questionnaire. Between August 2006 and May 2007, survey data on voice utilizing the V-RQOL and VHI-10 were obtained from a total of 130 patients who had received definitive treatment for laryngeal cancers with follow-up at the Hokkaido University Hospital, and who were alive with no evidence of malignancy at the time of survey. Mean V-RQOL scores of the patients who received radiation therapy (n=63), chemoradiotherapy (n=29), laser resection (n=14) and total laryngectomy (n=27) as the final treatment for laryngeal cancer were 92.6, 92.9, 85.5, and 68.4, respectively. Mean VHI-10 values were 2.87, 2.34, 5.43, and 11.26. Such measures of quality of life are important endpoints to help judge the overall effectiveness of treatment options for laryngeal cancers. (author)

  9. Recurrent Respiratory Papillomatosis or Laryngeal Papillomatosis

    Science.gov (United States)

    ... Home » Health Info » Voice, Speech, and Language Recurrent Respiratory Papillomatosis or Laryngeal Papillomatosis On this page: What ... find additional information about RRP? What is recurrent respiratory papillomatosis? Recurrent respiratory papillomatosis (RRP) is a disease ...

  10. Skin deposits in hereditary cystatin C amyloidosis

    DEFF Research Database (Denmark)

    Benedikz, Eirikur; Blöndal, H; Gudmundsson, G

    1990-01-01

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

  11. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy: A Retrospective Study of 5 Cases

    Directory of Open Access Journals (Sweden)

    Yanyan Niu

    2017-01-01

    Full Text Available This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8–384 months. One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient’s general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.

  12. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases.

    Science.gov (United States)

    Niu, Yanyan; Li, Yongjin; Wang, Jian; Jin, Xiaofeng; Yang, Dahai; Huo, Hong; Li, Wuyi

    2017-01-01

    This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8-384 months). One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient's general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.

  13. Application of primary cell cultures of laryngeal carcinoma and laser scanning cytometry in the evaluation of tumor reactivity to cisplatinum.

    Directory of Open Access Journals (Sweden)

    Krzysztof Kupisz

    2008-06-01

    Full Text Available Unsatisfactory effects of treatment of laryngeal carcinoma patients stimulate the clinicians as well as researchers to develop new more effective treatment models and to find new reliable prognostic factors. The aim of the present study was the evaluation of the use of primary cell cultures of the laryngeal carcinoma and laser scanning cytometry (LSC in the assessment of tumor reactivity to cisplatinum. Nineteen primary cultures of laryngeal carcinoma cells established from fragments of laryngeal carcinoma infiltrations were cultured with or without cisplatin, stained with monoclonal antibodies against P53 and BCL-2 proteins and analyzed by LSC. Cisplatin added to the culture medium leads to the significant increase of P53 expression and decrease of BCL-2 expression. Moreover, changes of P53 and BCL-2 expressions were significantly correlated. Our findings of apoptosis regulatory mechanisms could be useful in patient qualification for the chemotherapeutic follow-up treatment.

  14. Application of primary cell cultures of laryngeal carcinoma and laser scanning cytometry in the evaluation of tumor reactivity to cisplatinum

    International Nuclear Information System (INIS)

    Klatka, J.; Trojanowski, P.; Paduch, R.; Pozarowski, P.; Rolinski, J.; Pietruszewska, W.; Kupisz, K.

    2008-01-01

    Unsatisfactory effects of treatment of laryngeal carcinoma patients stimulate the clinicians as well as researchers to develop new more effective treatment models and to find new reliable prognostic factors. The aim of the present study was the evaluation of the use of primary cell cultures of the laryngeal carcinoma and laser scanning cytometry (LSC) in the assessment of tumor reactivity to cis platinum. Nineteen primary cultures of laryngeal carcinoma cells established from fragments of laryngeal carcinoma infiltrations were cultured with or without cisplatin, stained with monoclonal antibodies against P53 and BCL-2 proteins and analyzed by LSC. Cisplatin added to the culture medium leads to the significant increase of P53 expression and decrease of BCL-2 expression. Moreover, changes of P53 and BCL-2 expressions were significantly correlated. Our findings of apoptosis regulatory mechanisms could be useful in patient qualification for the chemotherapeutic follow-up treatment. (author)

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

    Directory of Open Access Journals (Sweden)

    Arahata M

    2016-08-01

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

  16. Neuromagnetic detection of the laryngeal area: Sensory-evoked fields to air-puff stimulation.

    Science.gov (United States)

    Miyaji, Hideaki; Hironaga, Naruhito; Umezaki, Toshiro; Hagiwara, Koichi; Shigeto, Hiroshi; Sawatsubashi, Motohiro; Tobimatsu, Shozo; Komune, Shizuo

    2014-03-01

    The sensory projections from the oral cavity, pharynx, and larynx are crucial in assuring safe deglutition, coughing, breathing, and voice production/speaking. Although several studies using neuroimaging techniques have demonstrated cortical activation related to pharyngeal and laryngeal functions, little is known regarding sensory projections from the laryngeal area to the somatosensory cortex. The purpose of this study was to establish the cortical activity evoked by somatic air-puff stimulation at the laryngeal mucosa using magnetoencephalography. Twelve healthy volunteers were trained to inhibit swallowing in response to air stimuli delivered to the larynx. Minimum norm estimates was performed on the laryngeal somatosensory evoked fields (LSEFs) to best differentiate the target activations from non-task-related activations. Evoked magnetic fields were recorded with acceptable reproducibility in the left hemisphere, with a peak latency of approximately 100ms in 10 subjects. Peak activation was estimated at the caudolateral region of the primary somatosensory area (S1). These results establish the ability to detect LSEFs with an acceptable reproducibility within a single subject and among subjects. These results also suggest the existence of laryngeal somatic afferent input to the caudolateral region of S1 in human. Our findings indicate that further investigation in this area is needed, and should focus on laryngeal lateralization, swallowing, and speech processing. Copyright © 2013 Elsevier Inc. All rights reserved.

  17. [First confirmed case of laryngeal diphtheria in Djibouti].

    Science.gov (United States)

    Koeck, J L; Merle, C; Bimet, F; Kiredjian, M; Goullin, B; Teyssou, R

    2000-01-01

    The first bacteriologically confirmed case of laryngeal diphtheria in Djibouti was reported in 1998. It involved a three-year-old native-born infant who had been vaccinated during the first year of life with three doses of a combined vaccine against diphtheria, tetanus, poliomyelitis, and pertussis. A rapid clinical improvement was observed under erythromycin treatment. Other cases of laryngeal diphtheria have been observed. It is important to reverse decreasing vaccinal coverage in Djibouti and to warn incoming travelers of the need to be adequate immunized against diphtheria. Enhanced epidemiologic surveillance of this disease is also needed.

  18. MicroRNA-196a is a putative diagnostic biomarker and therapeutic target for laryngeal cancer.

    Directory of Open Access Journals (Sweden)

    Koichiro Saito

    Full Text Available BACKGROUND: MicroRNA (miRNA is an emerging subclass of small non-coding RNAs that regulates gene expression and has a pivotal role for many physiological processes including cancer development. Recent reports revealed the role of miRNAs as ideal biomarkers and therapeutic targets due to their tissue- or disease-specific nature. Head and neck cancer (HNC is a major cause of cancer-related mortality and morbidity, and laryngeal cancer has the highest incidence in it. However, the molecular mechanisms involved in laryngeal cancer development remain to be known and highly sensitive biomarkers and novel promising therapy is necessary. METHODOLOGY/PRINCIPAL FINDINGS: To explore laryngeal cancer-specific miRNAs, RNA from 5 laryngeal surgical specimens including cancer and non-cancer tissues were hybridized to microarray carrying 723 human miRNAs. The resultant differentially expressed miRNAs were further tested by using quantitative real time PCR (qRT-PCR on 43 laryngeal tissue samples including cancers, noncancerous counterparts, benign diseases and precancerous dysplasias. Significant expressional differences between matched pairs were reproduced in miR-133b, miR-455-5p, and miR-196a, among which miR-196a being the most promising cancer biomarker as validated by qRT-PCR analyses on additional 84 tissue samples. Deep sequencing analysis revealed both quantitative and qualitative deviation of miR-196a isomiR expression in laryngeal cancer. In situ hybridization confirmed laryngeal cancer-specific expression of miR-196a in both cancer and cancer stroma cells. Finally, inhibition of miR-196a counteracted cancer cell proliferation in both laryngeal cancer-derived cells and mouse xenograft model. CONCLUSIONS/SIGNIFICANCE: Our study provided the possibilities that miR-196a might be very useful in diagnosing and treating laryngeal cancer.

  19. Tracheal and laryngeal tumors in the dog and cat: literature review and 13 additional patients

    International Nuclear Information System (INIS)

    Carlisle, C.H.; Biery, D.N.; Thrall, D.E.

    1991-01-01

    Primary tumors of the larynx or trachea are uncommon in the dog and cat. In a review of the English language literature, description of 65 such patients were found. In a search of the Veterinary Teaching Hospitals of the University of Pennsylvania and North Carolina State University, an additional 13 previously unreported patients were identified, bringing the total to at least 78. Of these 78, there have been 16 canine tracheal, 7 feline tracheal, 34 canine laryngeal and 21 feline laryngeal tumors. In the canine and feline trachea, osteochondroma and epithelial malignancies, respectively, appear to be the most common. Epithelial malignancies appear to be the most common tumor of the canine larynx whereas lymphosarcoma appears to be the most common feline laryngeal tumor. In patients described herein, tumors produced clinical signs consistent with airway obstruction. Voice alteration was common in patients with laryngeal tumors. Patients were middle-aged to older, except for dogs with osteochondroma. This compares favorably to historical data. All tumors in this study were readily seen radiographically, with most laryngeal and tracheal tumors appearing as masses within the lumen of the airway. Mineralization was uncommon except for canine osteochondromas. Feline laryngeal tumors in this study appeared as generalized laryngeal thickening rather than as a distinct mass. Response of canine and feline tracheal and laryngeal thickening rather than as a distinct mass. Response of canine and feline tracheal and laryngeal tumors to treatment can not be adequately assessed from available data. Benign tumors of the larynx or trachea may be amenable to complete excision. Neoplastic lesions must be differentiated from polyps or abscesses within the upper airway as these may appear radiographically identical to primary tumors. This can be achieved by endoscopic evaluation and biopsy of airway masses before formulating a prognosis

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

    Science.gov (United States)

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

    2016-06-01

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

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

    Science.gov (United States)

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

    2015-05-01

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

  2. Should patients with laryngeal small cell neuroendocrine carcinoma receive prophylactic cranial irradiation?

    NARCIS (Netherlands)

    Coca-Pelaz, Andres; Devaney, Kenneth O.; Rodrigo, Juan P.; Halmos, Gyorgy B.; Strojan, Primoz; Mendenhall, William M.; Eisbruch, Avraham; Smee, Robert; Kusafuka, Kimihide; Rinaldo, Alessandra; Ferlito, Alfio

    2016-01-01

    While small cell neuroendocrine carcinomas (SCNCs) most often arise in the lung, extrapulmonary SCNCs arise in a variety of locations-including the head and neck region. In particular, laryngeal SCNCs-while rare tumors-are nevertheless recognized as distinct lesions. The rarity of laryngeal SCNC

  3. Apropos of a case of cutaneous metastasis from laryngeal cancer with review of literature

    Directory of Open Access Journals (Sweden)

    Romeeta Trehan

    2015-01-01

    Full Text Available Cutaneous metastasis from laryngeal carcinoma is a rare occurrence. A 55-year-old male patient with supraglottic cancer was treated with concurrent chemoradiation. Eighteen months later, he presented with ulceroproliferative growth on dorsum of the right hand. Biopsy revealed metastatic squamous cell carcinoma. Further investigations revealed underlying bone destruction with lung metastasis. In view of poor general condition and widespread dissemination of disease, palliative radiotherapy was delivered to the hand of the patient. He achieved satisfactory palliation in form of pain relief, control of bleeding, and discharge. The present report serves to emphasize the importance of properly diagnosing metastatic spread to unusual sites. Such metastasis is rare and is associated with a poor prognosis. Treatment is usually aimed at providing pain relief in these patients with limited life expectancy. Hence, we present a case of extensive cutaneous metastasis from laryngeal carcinoma with review of the literature.

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

    Science.gov (United States)

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

    2015-08-01

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

  5. Disseminated cutaneous histoplasmosis with laryngeal involvement ...

    African Journals Online (AJOL)

    Disseminated cutaneous histoplasmosis with laryngeal involvement in a setting of immune reconstitution inflammatory syndrome. ... Grocott-Gomori methenamine silver and Periodic acid–Schiff (PAS) stains revealed a relative paucity of intracellular, narrow-neck budding fungal organisms. Culture findings confirmed the ...

  6. Laryngeal granuloma in a thorougbread horse: a multidisciplinary study

    OpenAIRE

    Morales B, Abelardo; Campos A, Gerardo; Zerpa, Héctor; Fernández, David; García, Francisco; Bermúdez G, Víctor; Morales B, María

    2011-01-01

    A two year-old racing thoroughbred mare presented with loud respiratory stertor, that was auscultated in the tracheal and laryngeal regions. Upon palpitation of the larynx, no changes were noted. The upper respiratory tract was examined via a fiberoptic endoscopy where the nasal passages were observed to be unchanged and the scope passed with normal resistance. The guttural pouches and their openings were normal. The right arytenoid cartilage appeared thickened throughout its length and faile...

  7. The risk factors of laryngeal pathology in Korean adults using a decision tree model.

    Science.gov (United States)

    Byeon, Haewon

    2015-01-01

    The purpose of this study was to identify risk factors affecting laryngeal pathology in the Korean population and to evaluate the derived prediction model. Cross-sectional study. Data were drawn from the 2008 Korea National Health and Nutritional Examination Survey. The subjects were 3135 persons (1508 male and 2114 female) aged 19 years and older living in the community. The independent variables were age, sex, occupation, smoking, alcohol drinking, and self-reported voice problems. A decision tree analysis was done to identify risk factors for predicting a model of laryngeal pathology. The significant risk factors of laryngeal pathology were age, gender, occupation, smoking, and self-reported voice problem in decision tree model. Four significant paths were identified in the decision tree model for the prediction of laryngeal pathology. Those identified as high risk groups for laryngeal pathology included those who self-reported a voice problem, those who were males in their 50s who did not recognize a voice problem, those who were not economically active males in their 40s, and male workers aged 19 and over and under 50 or 60 and over who currently smoked. The results of this study suggest that individual risk factors, such as age, sex, occupation, health behavior, and self-reported voice problem, affect the onset of laryngeal pathology in a complex manner. Based on the results of this study, early management of the high-risk groups is needed for the prevention of laryngeal pathology. Copyright © 2015 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  8. Association of history of allergies and influenza-like infections with laryngeal cancer in a case-control study.

    Science.gov (United States)

    Filippidis, Filippos T; Schwartz, Stephen M; Becker, Nikolaus; Dyckhoff, Gerhard; Kirschfink, Michael; Dietz, Andreas; Becher, Heiko; Ramroth, Heribert

    2015-08-01

    Prior studies suggest that history of allergy and infections early in life might be inversely associated with cancer. We explored the association between allergies, recent influenza infections and laryngeal cancer risk. We used data from a case-control study which included 229 cases of laryngeal cancer and 769 population controls matched for age and sex. History of a physician-diagnosed allergy, influenza-like infections in the past 5 years, smoking, alcohol consumption and occupational exposure to carcinogens were self-reported. Allergies were classified into two groups (Type I and Type IV), according to the underlying immunologic mechanism. Conditional logistic regression models were fitted using laryngeal cancer as the outcome, adjusting for smoking, alcohol consumption and occupational exposure and stratified for age and sex. Having any allergy was not associated significantly with laryngeal cancer. Although Type I and Type IV allergies were non-significantly associated with laryngeal cancer, Type IV allergies showed a strong inverse association after adjusting for smoking and alcohol (OR 0.50, 95 % CI 0.22-1.2). Participants who reported at least one influenza-like infection during the past 5 years were significantly less likely to have laryngeal cancer (OR 0.57, 95 % CI 0.39-0.81). After considering fever (≥38.5 °C) as a criterion for influenza infection, the association between influenza infection and laryngeal cancer was even stronger (OR 0.29, 95 % CI 0.13-0.63). We found no significant association between any allergy and laryngeal cancer, some indication of an inverse association between Type IV allergy and laryngeal cancer, whereas recent influenza infections were inversely associated with laryngeal cancer risk.

  9. Laryngeal sarcoidosis: a case-control study.

    Science.gov (United States)

    Duchemann, Boris; Lavolé, Armelle; Naccache, Jean-Marc; Nunes, Hilario; Benzakin, Sylvain; Lefevre, Marine; Kambouchner, Marianne; Périé, Sophie; Valeyre, Dominique; Cadranel, Jacques

    2014-10-20

    We undertook a study on a series of laryngeal sarcoidosis (LS), a very rare and often threatening localization to better specify laryngeal manifestations, sarcoidosis clinical expression and long-term follow-up. This was a retrospective case-control study. All LS patients from two French centers were included and compared to sarcoidosis patients without laryngeal localization with two controls for one patient. Twelve consecutive LS patients were recruited between 1993 and 2011. LS revealed sarcoidosis in eight cases (67%). The most common symptoms were hoarseness (77%), inspiratory dyspnea (38%) and dysphagia (38%). Epidemiological characterisics were not different. Extrapulmonary localizations were significantly more common in LS patients than in controls (92% vs. 54%, p=0.02), particularly lupus pernio (25% vs. 0%, p=0.03) and nasosinusal involvement (83% vs. 4%, p<0.01) while thoracic involvement was less frequent (58% vs 100%, p < 0.01). Treatment rates were higher in the LS group (92% vs. 58%, p=0.04), and treatment duration was longer (median: 81 vs. 13 months, p=0.04), with frequent long-term treatment (67%, N=8/12). Two patients underwent surgery. One patient needed temporary tracheostomy during the course of the disease; Remission rates were lower in LS patients (9% vs. 58% at 2 years p<0.01). Eventually, there was no death nor definitive tracheotomy. LS is a rare condition that is often associated to other loco-regional localizations. LS are often difficult to manage. Survival is good but may require a medico-surgical approach.

  10. Effect of intraoperative neuromonitoring on recurrent laryngeal nerve palsy rates after thyroid surgery—A meta-analysis

    Directory of Open Access Journals (Sweden)

    Shixing Zheng

    2013-08-01

    Conclusion: Based on this meta-analysis, statistically significant differences were determined in terms of the incidences of total and transient recurrent laryngeal nerve palsy after using IONM versus recurrent laryngeal nerve identification alone during thyroidectomy. However, no statistically significant differences were identified regarding the incidence of persistent recurrent laryngeal nerve palsy between groups.

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

    Directory of Open Access Journals (Sweden)

    Ting Li

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

  12. Quality of life of patients with laryngeal cancer treated with radiotherapy

    International Nuclear Information System (INIS)

    Ikeda, Hajime; Sawai, Yuka; Inoue, Toshihiko; Matayoshi, Yoshinobu; Murayama, Shigeyuki; Kozuka, Takahiro.

    1995-01-01

    Radiotherapy (RT) is considered as the best treatment for early laryngeal cancer in terms of survival and preservation of voice. Quality of life (QOL) must also be assessed after new treatments, such as laser treatment or laryngomicrosurgery. The QOL after RT was assessed in 61 patients (57 men and 4 women) with histologically proven laryngeal or hypopharyngeal cancer (glottis T1-2, 48 : supraglottis 11 : hypopharynx, 2). Cobalt-60 gamma rays were emitted via lateral opposed portals that included the glottis/larynx area. The delivered dose was 53 to 70 Gy/23 to 35 Fr (median, 60 Gy/30 Fr). Assessment was done by interview of physicians at periodic follow-up examinations of patients. The census period was June to December 1990. Patients' ages ranged from 37 to 82 years (median, 62 years) at treatment and 38 to 88 years (median, 68 years) at QOL assessment. Most patients enjoyed the similar daily activities and job status as before. Thirty-seven of 61 patients felt occasional hoarseness after RT, and 21 refrained from loud speech. All patients could use the telephone easily, except for one patient who had severe hearing loss. Many patients stopped smoking cigarettes, but most drank as before RT. There were no significant differences in lifestyle quality between less than and more than 5 years after RT. This QOL assessment is applicable to patients with laryngeal cancer. One half of patients treated by RT have symptoms compatible with chronic laryngitis. This study confirms that RT yields good QOL and voice quality for patients with laryngeal cancer. (author)

  13. Should we routinely expose recurrent laryngeal nerve(s) during thyroid surgery

    International Nuclear Information System (INIS)

    Ahmed, M.; Aurangzeb, A.; Rashid, A.Z.; Qureshi, M.A.; Iqbal, N.; Boota, M.; Ashfaq, M.

    2013-01-01

    Objective: To compare the frequency of recurrent laryngeal nerve(s) (RLNs) palsy after various thyroid procedures with and without identification of recurrent laryngeal nerve during the operation. Study Design: Randomized controlled trial. Place and Duration of Study: Department of Surgery, Military Hospital, Rawalpindi, from August 2008 to April 2010. Methodology: Patients undergoing indirect laryngoscopy with normal vocal cords and those with carcinoma and re-do surgery having normal vocal cord were included in the study. Patients with hoarseness of voice, abnormal vocal cord movements and with solitary nodule in the isthmus were excluded. These patients were randomly divided into 2 groups of 50 each using random number tables. RLN was identified by exposing the inferior thyroid artery and traced along its entire course in group-A. Whereas, in group-B, nerves were not identified during the operations. Immediate postoperative direct laryngoscopy was performed by a surgeon with the help of an anaesthesiologist for the assessment of vocal cords. Patients with persistent hoarseness of voice were followed-up with indirect laryngoscopy at 3 and 6 months. Results: Temporary unilateral recurrent laryngeal nerve palsies occurred in 2 (4%) patients in group-A where the voice and cord movements returned to normal in 6 months. In group-B, it occurred in 8 (16%) patients, 2 bilateral (4%) injuries requiring tracheostomy and 6 unilateral injuries (12%). Among the 2 bilateral recurrent laryngeal nerve injuries, the tracheostomy was removed in one case after 6 months with persistent hoarseness of voice but no respiratory difficulty during routine activities. Tracheostomy was permanent in the other case. Among the 6 cases of unilateral nerve injuries, the voice improved considerably in 4 cases within 6 months but in 2 cases hoarseness persisted even after 6 months. Frequency of recurrent laryngeal nerve palsies was significantly lower in group-A as compared to group-B (p = 0

  14. Fasudil inhibits proliferation and migration of Hep-2 laryngeal carcinoma cells

    Directory of Open Access Journals (Sweden)

    Zhang X

    2018-02-01

    Full Text Available Xiaowen Zhang,1 Nan Wu2 1Medical Research Center, Shengjing Hospital of China Medical University, Shenyang, China; 2The Core Laboratory for Public Health Science and Practice, The First Affiliated Hospital of China Medical University, Shenyang, China Background: Rho-kinase signal pathway is a new target for cancer therapy. Fasudil, a selective Rho-kinase inhibitor, is found to exert antitumor effects on several types of cancer, but whether fasudil has antitumor effects on laryngeal carcinoma is still unknown. The aim of this study was to determine the effects of fasudil on laryngeal carcinoma and explore the underlying molecular mechanisms in this process. Methods: After treatment with fasudil, changes in biological behaviors, including the growth, proliferation, clone formation, apoptosis, and migration of human laryngeal carcinoma cells (Hep-2 cells were observed. The influences on apoptotic protease activity factor-1 (APAF-1-mediated apoptosis pathway and the activities of matrix metalloproteinases (MMP-2 and MMP-9 were measured by Western blotting and gelatin zymography assay. Results: Half-maximal inhibitory concentration of fasudil to Hep-2 cells was ~3.40×103 µM (95% CI: 2.53–4.66×103 µM. Moreover, fasudil treatment significantly decreased the ability of growth, proliferation, clone formation, and migration of Hep-2 cells, while remarkably increased the apoptosis rate. Furthermore, the expressions of APAF-1, caspase-9, and caspase-3 significantly increased in fasudil treatment group. Meanwhile, fasudil led to a remarkable decrease in the expressions and activities of MMP-2 and MMP-9. Conclusion: Our findings first demonstrate that fasudil not only inhibits the proliferation of laryngeal carcinoma cells through activating APAF-1-mediated apoptosis pathway, but also prevents migration by inhibiting the activities of MMP-2 and MMP-9. Therefore, fasudil is an attractive antitumor drug candidate for the treatment of laryngeal carcinoma

  15. Laryngeal schwannoma: a case report with emphasis on sonographic findings

    Energy Technology Data Exchange (ETDEWEB)

    Souza, Luis Ronan Marquez Ferreira de, E-mail: luisronan@gmail.com [Universidade Federal do Triangulo Mineiro (UFTM), Uberaba, MG (Brazil); De Nicola, Harley; Yamasaki, Rosiane; Pedroso, Jose Eduardo; Brasil, Osiris de Oliveira Campones do; Yamashita, Helio [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Medicina

    2014-05-15

    Schwannomas are benign nerve sheath tumors composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral, cranial and autonomic nerves. Twenty-five to forty-five percent of all schwannomas occur in the head and neck region, but location of such tumors in the larynx is rarely observed. The present report is aimed at describing a clinical case of laryngeal schwannoma, with emphasis on sonographic findings. (author)

  16. Clinical and laboratory evaluation of thyroid function following total laryngectomy in laryngeal cancer

    Directory of Open Access Journals (Sweden)

    Motasaddi Zarandy M

    2007-07-01

    Full Text Available Background: Hypothyroidism is a well-documented complication after treatment of laryngeal cancer and is particularly significant among patients undergoing laryngectomy. We investigated the frequency of hypothyroidism in patients treated with total laryngectomy for laryngeal cancer. We also evaluated the effect of neck radiotherapy on thyroid function after total laryngectomy for laryngeal cancer. Methods: In a cross-sectional study, we evaluated 31 patients with laryngeal squamous cell carcinoma (mean age 53.6 years. Among these patients, 14 were treated with surgery only and 17 were treated with surgery plus radiotherapy. Laboratory evaluation included levels of thyroid stimulating hormone (TSH, free T4, free T3, and antithyroid antibodies both preoperatively and postoperatively at the first day, as well as one and six months after surgery. Results: All patients had normal thyroid function before treatment; however, after 6 months, five patients (16.1% were hypothyroid. Of these, three patients (9.6% had subclinical symptoms, including elevated thyroid-stimulating hormone with normal free T4, and two patients (6.5% showed clinical symptoms of hypothyroidism. Radiotherapy and neck dissection were significantly associated with higher incidences of hypothyroidism. Conclusion: Our data suggest that hypothyroidism occurs in a substantial proportion of patients undergoing surgery for laryngeal cancer. The results indicate that thyroid function studies should be routinely performed in the follow-up care of laryngeal cancer patients, especially if radiotherapy and neck dissection were part of the treatment. We suggest that this approach will improve the patient's quality of life and diminish the co-morbidity associated with this kind of surgery.

  17. Laryngeal Electromyographic findings in patients with vocal fold motion asymmetry.

    Science.gov (United States)

    Woo, Peak; Isseroff, Tova F; Parasher, Arjun; Richards, Amanda; Sivak, Mark

    2016-08-01

    Vocal fold motion asymmetry (VFMA) is often attributed to vocal fold paresis or an anatomical variant. Although laryngeal electromyography (LEMG) may be used to evaluate patients with vocal fold paresis, electrodiagnostic findings in VFMA have not been well defined. Review of a case series Twenty-five symptomatic patients with VFMA were examined by LEMG, and the findings were analyzed. Although all were thought to have unilateral recurrent laryngeal nerve paresis, LEMG showed only nine to have unilateral recurrent nerve paresis. There were nine with both ipsilateral recurrent laryngeal nerve and superior laryngeal nerve paresis, four with bilateral paresis, and three were normal. Reduced total number of units, reduced recruitment, motor units firing fast, and polyphasic units were more common, whereas fibrillation potentials, fasciculation, positive sharp waves, and complex repetitive discharges were uncommon. The LEMG findings are most consistent with old, healed neuropathy. McNemar's test for the acute versus chronic denervation potentials showed significant differences. VFMA has a high incidence of vocal fold paresis that can be better defined by LEMG. The site and side of paresis is often wrong based on laryngoscopy findings alone. The LEMG findings of VFMA appear to be consistent with old, healed neuropathy 4 Laryngoscope, 126:E273-E277, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2018-03-15

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

  19. Comparison of Ambu® AuraGain™ laryngeal mask and air-Q™ intubating laryngeal airway for blind tracheal intubation in adults: A randomized controlled trial

    OpenAIRE

    Sameer Sethi; Souvik Maitra; Vikas Saini; Tanvir Samara

    2017-01-01

    Background: This study has been designed to compare the performance of Ambu® AuraGain™ laryngeal mask with the air-Q™ as a conduit for blind tracheal intubation in adult patients. Methods: In this prospective randomized controlled trial blind endotracheal intubation success rates were compared between Ambu® AuraGain™ and air-Q™ intubating laryngeal airway in 90 adult patients. Patients were randomized in two equal groups: Group Ambu® AuraGain™ (n = 45) and Group air-Q™ (n = 45). Results...

  20. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases

    OpenAIRE

    Yanyan Niu; Yongjin Li; Jian Wang; Xiaofeng Jin; Dahai Yang; Hong Huo; Wuyi Li

    2017-01-01

    This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymme...

  1. 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.

  2. Laryngeal schwannoma excised under a microlaryngoscope without tracheotomy: A case report

    Science.gov (United States)

    WANG, BAOXIN; DONG, PIN; SHEN, BIN; XU, HONGMING; ZHENG, JIN

    2014-01-01

    Schwannomas are benign encapsulated tumors arising from Schwann cells in the peripheral nervous system. Between 25 and 45% of schwannomas occur in the head and neck region. Schwannomas of the larynx are extremely rare with an incidence of 0.1–1.5% in all benign laryngeal tumors. Laryngeal schwannomas usually occur in females aged between their 4th and 5th decades. The most successful curative method is surgical resection. When selecting the surgical method, the size and location of the tumor, as well as the requests of the patient, should be considered. The present case report describes a 29-year-old female patient with symptoms of hoarseness and dyspnea on exertion. Through endoscopic biopsy, histopathology revealed a schwannoma. Considering the symptoms, age and preferences of the patient, the method of trans-oral microlaryngoscopic excision without tracheotomy was used to excise the tumor located in the aryepiglottic fold. Results from a short-term follow-up showed the postoperative result to be satisfactory. PMID:24669270

  3. Cancer stage and pack-years, but not p16 or HPV, are relevant for survival in hypopharyngeal and laryngeal squamous cell carcinomas.

    Science.gov (United States)

    Dahm, Valerie; Haitel, Andrea; Kaider, Alexandra; Stanisz, Isabella; Beer, Andrea; Lill, Claudia

    2018-05-09

    Recently, p16 has been included in the TNM guideline for oropharyngeal carcinomas. The role of HPV and p16 in hypopharyngeal and laryngeal carcinomas has not yet been established sufficiently. Hundred and thirty-four patients with hypopharyngeal and laryngeal carcinomas were included in this retrospective analysis. Only patients with known HPV status were eligible for the investigation. Survival probabilities were estimated for different risk factors. Eighty-five patients presented with laryngeal carcinoma and 49 patients with hypopharyngeal carcinoma. 8% were HPV positive (10.6% laryngeal, 4.1% hypopharyngeal carcinoma). Median follow-up time was 58 months. We observed a significantly better overall survival for patients with an early tumor stage compared to advanced carcinoma. One of the hypopharyngeal HPV positive carcinomas was also p16 positive and one was p16 negative. Of the nine HPV positive laryngeal carcinomas, four were p16 positive and five p16 negative. Neither patients who were HPV positive nor patients positive for p16 showed a significantly better outcome than HPV or p16 negative patients. In contrast, nicotine pack-years showed a highly significant correlation with survival in our patient collective. The data suggest that tumor stage and nicotine exposure seem to have the highest impact on survival in hypopharyngeal and laryngeal squamous cell carcinoma patients. There is no evidence for a better survival for p16 positive or HPV positive patients with hypopharyngeal or laryngeal squamous cell carcinoma. HPV seems to play a minor role in these entities of head and neck carcinoma.

  4. Carotid endarterectomy significantly improves postoperative laryngeal sensitivity.

    Science.gov (United States)

    Hammer, Georg Philipp; Tomazic, Peter Valentin; Vasicek, Sarah; Graupp, Matthias; Gugatschka, Markus; Baumann, Anneliese; Konstantiniuk, Peter; Koter, Stephan Herwig

    2016-11-01

    Iatrogenic injury of the vagus nerve or its branches during carotid endarterectomy (CEA) can result in globus sensation, dysphagia, and even vocal fold immobility. Knowledge of morphologic and functional laryngopharyngeal outcomes after CEA is poor. The present study was performed to determine potential iatrogenic damage to the laryngeal innervation after CEA. An area of particular interest was the supraglottic sensory threshold, which was examined by Fiberoptic Endoscopic Evaluation of Swallowing With Sensory Testing (FEESST; Pentax Medical Company, Montvale, NJ), a validated and safe method for the determination of the motor and sensory components of swallowing. FEESST was used preoperatively in 32 patients scheduled to undergo CEA and twice postoperatively to examine the motor and sensory components of swallowing. In this endolaryngeal examination, laryngopharyngeal sensory thresholds (in mm Hg) were defined as normal at 6.0 mm Hg APP, with a value >10.0 mm Hg APP indicating abolished laryngeal adductor reflex. Acoustic voice parameters were also analyzed for further functional changes of the larynx. The mean ± standard deviation preoperative FEESST measures showed no significant differences (P = .065) between the operated-on side (6.73 ± 1.73 mm Hg) and the opposite side (5.83 ± 1.68 mm Hg). At 2 days postoperatively, the threshold increased (P = .001) to 7.62 ± 1.98 mm Hg on the operated-on side. A laryngopharyngeal mucosal hematoma on the operated side was endoscopically detectable in eight patients (30.8%); in these patients, we found a markedly elevated (P = .021) measure of 9.50 ± 0.93 mm Hg. On the opposite (nonoperated-on) side of the laryngopharynx, the thresholds remained at the same level as preoperatively over all assessments (P >.05), whereas the differences between the operated and nonoperated-on sides and the hematoma and nonhematoma groups were highly significant (P = .004 and P = .001, respectively). Surprisingly, the

  5. Cervical osteophytes presenting as unilateral vocal fold paralysis and dysphagia.

    Science.gov (United States)

    Yoskovitch, A; Kantor, S

    2001-05-01

    Any process involving either the vagus nerve, its recurrent laryngeal branch or the external branch of the superior laryngeal nerve may cause paralysis of the vocal fold. The most common cause is neoplasm. Clinically, the patients often present with a hoarse, breathy voice as well as symptoms of aspiration. The following represents a unique case of unilateral vocal fold paralysis and dysphagia caused by a degenerative disease of the cervical spine, resluting in extrinsic compression of the recurrent laryngeal nerve.

  6. [Rare problem with the insertion of a Supreme™ laryngeal mask airway device. Case of the trimester].

    Science.gov (United States)

    2014-03-01

    A breast tumor was resected under general anesthesia. After induction, the airway was managed with a Supreme™ laryngeal mask airway device. The insertion of the laryngeal mask airway device, the insertion of the orogastric tube through the drain tube, as well as the mechanical ventilation, were very difficult from the beginning. On removing the laryngeal mask airway device to solve the problem, it was observed that the drain tube was broken, and the orogastric tube had passed into the anterior, laryngeal part of the device through the split. It was later found out that the laryngeal mask airway device, as well as the whole manufacturing batch, had suffered a design modification: the cuff was constructed with a softer material without reinforcement in the tip, and the drain tube had a heat-sealing defect that facilitated the break. The incident was reported to the local supplier and the manufacturer, and the defective batch of laryngeal mask airway devices was recalled. The incident was also reported to other hospitals via SENSAR, to warn other users of the potential dangers of the design modification in the Supreme™ laryngeal mask airway. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

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

    Science.gov (United States)

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

    2011-09-01

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

  8. Effect of laryngeal anesthesia on pulmonary function testing in normal subjects.

    Science.gov (United States)

    Kuna, S T; Woodson, G E; Sant'Ambrogio, G

    1988-03-01

    Pulmonary function tests (PFT) were performed on 11 normal subjects before and after topical anesthesia of the larynx. The PFT consisted of flow volume loops and body box determinations of functional residual capacity and airway resistance, each performed in triplicate. After the first set of tests, cotton pledgets soaked in 4% lidocaine were held in the pyriform sinuses for 2 min to block the superior laryngeal nerves. In addition, 1.5 ml of 10% cocaine was dropped on the vocal cords via indirect laryngoscopy. PFT were repeated 5 min after anesthesia. Besides routine analysis of the flow volume loops, areas under the inspiratory (Area I) and expiratory (Area E) portions of the loops were calculated by planimetry. Area I, peak inspiratory flow (PIF), as well as forced inspiratory flow at 25, 50, and 75% forced vital capacity (FVC), decreased after anesthesia. Peak expiratory flow decreased after anesthesia, but Area E and forced expiratory flow at 25, 50, and 75% FVC were unchanged. This protocol also was performed in 12 normal subjects with isotonic saline being substituted for the lidocaine and cocaine. In this group, no significant differences were observed when flow volume loop parameters were compared before and after topical application of saline. In 5 spontaneously breathing anesthetized dogs, posterior cricoarytenoid muscle and afferent superior laryngeal nerve activity were recorded before and after laryngeal anesthesia performed with the same procedure used in the human subjects. Laryngeal anesthesia resulted in a substantial decrease or a complete disappearance of afferent SLN activity recorded during unobstructed and obstructed respiration. The data suggest that laryngeal receptors help modulate upper airway patency in man.

  9. Twice-a-day fractionated radiotherapy with chemotherapy for advanced laryngeal cancer

    International Nuclear Information System (INIS)

    Karasawa, Kumiko; Okawa, Tomohiko

    1998-01-01

    Twenty-five patients with advanced laryngeal cancer were treated with twice-a-day fractionated radiotherapy (TDFR) to a total dose of 65 Gy to 82 Gy combined with chemotherapy of CDDP and 5-FU between 1994 and 1997. Twenty-two cases (88%) became complete response and 9 cases recurred. The relapse-free rate at 2 years was 49.8%. The laryngeal conserving rate at 2 years was 71.0%, the actuarial 2-year survival rate was 89.9%. In induction chemotherapy (12 cases) no severe toxicity has been observed. In TDFR with concurrent chemotherapy (22 cases), grade 3 hematological toxicity was observed in 4 cases and grade 4 mucosal toxicity in 16 cases. Based on this investigation, it is concluded that TDFR with chemotherapy is a promising modality for advanced laryngeal cancer and toxicity is acceptable. (author)

  10. Modified frontolateral partial laryngectomy operation: combined muscle-pedicle hyoid bone and thyrohyoid membrane flap in laryngeal reconstruction

    International Nuclear Information System (INIS)

    Ouyang, Dian; Liu, Tian-Run; Chen, Yan-Feng; Wang, Jian

    2013-01-01

    Laryngeal reconstruction is needed to preserve laryngeal function in patients who have undergone extensive vertical or frontal partial laryngectomy. However, the procedure remains a difficult challenge. Several reconstruction techniques have been described, but these techniques pose risks of complications such as laryngeal stenosis. This study aimed to evaluate the postoperative course and functional outcomes of a new technique that combined a muscle-pedicle hyoid bone and a thyrohyoid flap during laryngeal reconstruction after tumor resection. Four patients underwent extensive vertical partial or frontal partial laryngectomy for cancer. After tumor resection, laryngeal reconstruction was performed using the proposed technique. Postoperative recovery time, complications, and oncologic results were evaluated. The four patients were successfully treated with the proposed technique. No dyspnea, dysphagia, or death occurred during the postoperative course. Decannulation was performed after a median of 3 days. The average postoperative hospital stay was 7 days. Short-term postoperative functional recovery was normal. No laryngeal stenosis or tumor recurrence was observed in any of the four patients after a follow-up period of more than 24 months. The combination of the muscle-pedicle hyoid bone and the thyrohyoid flap is a reliable procedure for laryngeal reconstruction after extensive vertical partial or frontal partial laryngectomy

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

    Science.gov (United States)

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

    2017-11-01

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

  12. Differentiating laryngeal carcinomas from precursor lesions by diffusion-weighted magnetic resonance imaging at 3.0 T: a preliminary study.

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    De-Sheng Shang

    Full Text Available BACKGROUND: Diffusion-weighted magnetic resonance imaging (DWI has been introduced in head and neck cancers. Due to limitations in the performance of laryngeal DWI, including the complex anatomical structure of the larynx leading to susceptibility effects, the value of DWI in differentiating benign from malignant laryngeal lesions has largely been ignored. We assessed whether a threshold for the apparent diffusion coefficient (ADC was useful in differentiating preoperative laryngeal carcinomas from precursor lesions by turbo spin-echo (TSE DWI and 3.0-T magnetic resonance. METHODS: We evaluated DWI and the ADC value in 33 pathologically proven laryngeal carcinomas and 17 precancerous lesions. RESULTS: The sensitivity, specificity, and accuracy were 81.8%, 64.7%, 76.0% by laryngostroboscopy, respectively. The sensitivity, specificity, and accuracy of conventional magnetic resonance imaging were 90.9%, 76.5%, 86.0%, respectively. Qualitative DWI analysis produced sensitivity, specificity, and accuracy values of 100.0, 88.2, and 96.0%, respectively. The ADC values were lower for patients with laryngeal carcinoma (mean 1.195±0.32×10(-3 mm(2/s versus those with laryngeal precancerous lesions (mean 1.780±0.32×10(-3 mm(2/s; P<0.001. ROC analysis showed that the area under the curve was 0.956 and the optimum threshold for the ADC was 1.455×10(-3 mm(2/s, resulting in a sensitivity of 94.1%, a specificity of 90.9%, and an accuracy of 92.9%. CONCLUSIONS: Despite some limitations, including the small number of laryngeal carcinomas included, DWI may detect changes in tumor size and shape before they are visible by laryngostroboscopy. The ADC values were lower for patients with laryngeal carcinoma than for those with laryngeal precancerous lesions. The proposed cutoff for the ADC may help distinguish laryngeal carcinomas from laryngeal precancerous lesions.

  13. 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

  14. Acoustic and Perceptual Effects of Left-Right Laryngeal Asymmetries Based on Computational Modeling

    Science.gov (United States)

    Samlan, Robin A.; Story, Brad H.; Lotto, Andrew J.; Bunton, Kate

    2014-01-01

    Purpose: Computational modeling was used to examine the consequences of 5 different laryngeal asymmetries on acoustic and perceptual measures of vocal function. Method: A kinematic vocal fold model was used to impose 5 laryngeal asymmetries: adduction, edge bulging, nodal point ratio, amplitude of vibration, and starting phase. Thirty /a/ and /?/…

  15. Ultrasound-Guided Laryngeal Air Column Width Difference as a New Predictor for Postextubation Stridor in Children.

    Science.gov (United States)

    El Amrousy, Doaa; Elkashlan, Mohamed; Elshmaa, Nagat; Ragab, Ahmed

    2018-03-01

    To assess the efficacy of ultrasound-guided laryngeal air column width difference in predicting postextubation stridor in children. Prospective observational study. Single, tertiary care pediatric hospital. This study was carried out at PICU and surgical ICU, Tanta University Hospital on 400 ventilated children between January 2015 and May 2017. Patients who received mechanical ventilation and met criteria for a weaning trial were included. Laryngeal ultrasound and cuff leak test. Ultrasound-guided laryngeal air column width and cuff leak test were measured before extubation. Laryngeal air column width is the width of air between the vocal cords seen by laryngeal ultrasonography. Laryngeal air column width difference is the width difference of air column passed through vocal cords with the balloon cuff inflated and deflated. Three-hundred fifty six patients (89%) had no postextubation stridor, whereas 44 patients (11%) developed postextubation stridor. Postextubation stridor was associated with younger age, less weight, female gender, prolonged duration of intubation, and ICU stay (p column width difference and cuff leak test showed significant decrease (p column width difference at cutoff point of less than 0.8 mm gave a sensitivity of 93%, specificity of 86%, and accuracy of 91%, whereas cuff leak test at less than 11% yielded a sensitivity of (61%), specificity of (53%), and accuracy of (59%) for predicting postextubation stridor. Laryngeal air column width difference measurement may serve as a simple reliable noninvasive method for predicting postextubation stridor in children.

  16. [Postextubation laryngeal edema seven years after undergoing neck dissection].

    Science.gov (United States)

    Daijo, Hiroki; Habara, Toshie; Katagawa, Tomoko; Yoshikawa, Yukiko; Shinomura, Tetsutaro

    2008-05-01

    We report a case of upper airway obstruction after extubation in a 69-year-old female patient who underwent transurethral ureterolithotripsy (TUL). She had underwent bilateral modified radical neck dissection 7 years previously. TUL went smoothly in Trenderenburg position, and the extubation was performed after antagonism of neuromuscular block. The patient was closely observed in the operating theater, but about 10 minutes after extubation, she was noted to have dyspnea and tracheal tug. Dexamathasone 2 mg IV was given but was unsuccessful. Although we could support the airway with bag-mask ventilation, continuous stridor required re-intubation. Direct laryngoscopy revealed severe obstruction caused by laryngeal edema. An otolaryngologist was consulted and he performed tracheostomy. We transferred the patient to the intensive care unit for observation. Flexible fiberoptic scope examination performed on postoperative day (POD) 1 showed the decrease of the laryngeal edema. Tacheal tube was removed on POD 7 and she was discharged from the hospital POD 10 without further complications. Patients after a neck dissection may be at elevated risk for postoperative laryngeal edema caused by lymphatic destruction or venous congestion of the neck.

  17. Relationship between the internal laryngeal nerve and the triticeal cartilage: a potentially unrecognized compression site during anterior cervical spine and carotid endarterectomy operations.

    Science.gov (United States)

    Tubbs, R Shane; Dixon, Joshua F; Loukas, Marios; Shoja, Mohammadali M; Cohen-Gadol, Aaron A

    2010-06-01

    The triticeal cartilage has received scant attention in the literature. To date, its relationship to the nearby internal laryngeal nerve has not been studied. Therefore, to elucidate further this anatomic relationship and its potential surgical implications, this study was performed. Eighty-six adult cadaveric sides underwent dissection of the internal laryngeal nerve near its penetration of the thyrohyoid membrane. The relationship of this nerve to the triticeal cartilage was documented. Measurements and histological analysis were performed on all cartilage specimens. We identified triticeal cartilage in 51% of the specimens and found it to be hyaline in nature. The triticeal cartilage was located in the upper, middle, and lower thirds of the thyrohyoid membrane in 14%, 66%, and 20% of sides, respectively. Regardless of the position of the triticeal cartilage within the thyrohyoid membrane, the internal laryngeal nerve crossed directly over the triticeal cartilage on 59% of sides. When present, the internal laryngeal nerve will cross over the triticeal cartilage in the majority of individuals. This relationship should be borne in mind during surgical manipulation in this area and when placing retractors during anterior neck operations including cervical discectomy/fusion and carotid endarterectomy. Compression of the internal laryngeal nerve against the solid triticeal cartilage can cause laryngeal nerve palsy and increase the risk of resultant postoperative aspiration.

  18. Laryngeal adenocystic carcinoma treated by proton therapy

    International Nuclear Information System (INIS)

    Sugiyama, Tomonori; Araki, Mamika; Fukukita, Kouhei; Yamada, Hiroyuki

    2013-01-01

    Adenocystic carcinoma most commonly develops in the major salivary glands, on the other hand it is rare for adenocystic carcinoma to develop in the larynx. We report a case of adenocystic carcinoma in the larynx. A 54-year-old male was hospitalized with symptoms of hoarseness and dyspnea on exertion. He presented a tumor that developed at the base of the right arytenoid, and covered over the glottis. It was confirmed to be adenocystic carcinoma (solid type) by biopsy. Positron emission tomography (PET)-CT also revealed a left cervical lymph node metastasis and multiple pulmonary metastases (T1N2cM1). He was treated with proton therapy to the larynx to prevent airway obstruction by growth of the tumor and to preserve the larynx because he had uncontrollable pulmonary metastasis. Although the tumor vanished after the treatment, one month later he had halitosis, dyspnea and bilateral vocal cord palsy. Despite administration of an antibacterial drug and steroid, there was no improvement to the narrowness of the glottis. A tracheotomy was therefore performed three months after the proton therapy. PET-CT, which was performed after the tracheotomy, suggested growth of the residual tumor or laryngeal radionecrosis. This study confirmed that proton therapy is effective for adenocystic carcinoma in the larynx. However, proton therapy also was found to cause laryngeal radionecrosis. These results indicate the importance of evaluating the side effects of radiation therapy and providing that information to the patient. (author)

  19. Solitary Laryngeal Metastasis from Transitional Cell Carcinoma of the Kidney: Clinical Case and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Tarek Assi

    2015-01-01

    Full Text Available The urogenital tract is a rare origin of laryngeal metastasis; transitional cell carcinoma with laryngeal metastases had never been reported previously. In this paper, we describe the clinical and pathological characteristics, evolution, and treatment of the first reported case of a laryngeal metastasis of a TCC followed by a brief review of the literature.

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

    Science.gov (United States)

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

    2014-03-01

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

  1. Laryngeal Chondrosarcoma: An Exceptional Localisation of a Not Unfrequent Bone Tumor

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    Mieke Moerman

    2009-01-01

    Full Text Available After osteosarcoma, chondrosarcoma is the second most common primary bone tumor accounting for 26% of all malignancies. In the laryngeal region however, chondrosarcomas are rather rare. Only 300 cases are reported in literature. Considering laryngeal chondrosarcoma, about 75% occur in the cricoid cartilage, whereas 20% occur in the thyroid cartilage. In this paper we report a case of thyroidal chondrosarcoma, and based on a thorough literature search we suggest some practical guidelines concerning diagnosis and therapy.

  2. Effects of Voice Rehabilitation After Radiation Therapy for Laryngeal Cancer: A Randomized Controlled Study

    International Nuclear Information System (INIS)

    Tuomi, Lisa; Andréll, Paulin; Finizia, Caterina

    2014-01-01

    Background: Patients treated with radiation therapy for laryngeal cancer often experience voice problems. The aim of this randomized controlled trial was to assess the efficacy of voice rehabilitation for laryngeal cancer patients after having undergone radiation therapy and to investigate whether differences between different tumor localizations with regard to rehabilitation outcomes exist. Methods and Materials: Sixty-nine male patients irradiated for laryngeal cancer participated. Voice recordings and self-assessments of communicative dysfunction were performed 1 and 6 months after radiation therapy. Thirty-three patients were randomized to structured voice rehabilitation with a speech-language pathologist and 36 to a control group. Furthermore, comparisons with 23 healthy control individuals were made. Acoustic analyses were performed for all patients, including the healthy control individuals. The Swedish version of the Self Evaluation of Communication Experiences after Laryngeal Cancer and self-ratings of voice function were used to assess vocal and communicative function. Results: The patients who received vocal rehabilitation experienced improved self-rated vocal function after rehabilitation. Patients with supraglottic tumors who received voice rehabilitation had statistically significant improvements in voice quality and self-rated vocal function, whereas the control group did not. Conclusion: Voice rehabilitation for male patients with laryngeal cancer is efficacious regarding patient-reported outcome measurements. The patients experienced better voice function after rehabilitation. Patients with supraglottic tumors also showed an improvement in terms of acoustic voice outcomes. Rehabilitation with a speech-language pathologist is recommended for laryngeal cancer patients after radiation therapy, particularly for patients with supraglottic tumors

  3. Effects of Voice Rehabilitation After Radiation Therapy for Laryngeal Cancer: A Randomized Controlled Study

    Energy Technology Data Exchange (ETDEWEB)

    Tuomi, Lisa, E-mail: lisa.tuomi@vgregion.se [Department of Otorhinolaryngology, Head and Neck Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg (Sweden); Andréll, Paulin [Department of Molecular and Clinical Medicine/Multidisciplinary Pain Center, Institute of Medicine, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg (Sweden); Finizia, Caterina [Department of Otorhinolaryngology, Head and Neck Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg (Sweden)

    2014-08-01

    Background: Patients treated with radiation therapy for laryngeal cancer often experience voice problems. The aim of this randomized controlled trial was to assess the efficacy of voice rehabilitation for laryngeal cancer patients after having undergone radiation therapy and to investigate whether differences between different tumor localizations with regard to rehabilitation outcomes exist. Methods and Materials: Sixty-nine male patients irradiated for laryngeal cancer participated. Voice recordings and self-assessments of communicative dysfunction were performed 1 and 6 months after radiation therapy. Thirty-three patients were randomized to structured voice rehabilitation with a speech-language pathologist and 36 to a control group. Furthermore, comparisons with 23 healthy control individuals were made. Acoustic analyses were performed for all patients, including the healthy control individuals. The Swedish version of the Self Evaluation of Communication Experiences after Laryngeal Cancer and self-ratings of voice function were used to assess vocal and communicative function. Results: The patients who received vocal rehabilitation experienced improved self-rated vocal function after rehabilitation. Patients with supraglottic tumors who received voice rehabilitation had statistically significant improvements in voice quality and self-rated vocal function, whereas the control group did not. Conclusion: Voice rehabilitation for male patients with laryngeal cancer is efficacious regarding patient-reported outcome measurements. The patients experienced better voice function after rehabilitation. Patients with supraglottic tumors also showed an improvement in terms of acoustic voice outcomes. Rehabilitation with a speech-language pathologist is recommended for laryngeal cancer patients after radiation therapy, particularly for patients with supraglottic tumors.

  4. A non-recurrent inferior laryngeal nerve in a man undergoing thyroidectomy: a case report

    Directory of Open Access Journals (Sweden)

    Sousa Daniel

    2010-11-01

    Full Text Available Abstract Introduction A non-recurrent variant of the inferior laryngeal nerve has been seldom reported. These reports are mostly based on cadaveric dissection studies or large chart review studies in which the emphasis is placed on the determination of the frequency of the variation, and not on the clinical appearance of this variant. We graphically describe the intraoperative identification of a non-recurrent inferior laryngeal nerve. Case Presentation A 44-year old Caucasian man was referred to the Head and Neck Surgery Outpatient Clinic with the diagnosis of a nodular mass in his left thyroid lobe that had been growing for one year. A fine needle aspiration puncture was compatible with thyroid papillary cancer. It was decided that the patient should undergo total thyroidectomy. During surgery, a non-recurrent right inferior laryngeal nerve was noted. This nerve emanated from the right vagus nerve, entering the larynx 3 cm after its origin. The nerve did not show a recurrent course. The nerve on the left side had a normal configuration. The surgery and post-operative period were uneventful, and the patient had no change in his voice. Conclusion This paper allows those interested to become acquainted with the normal intraoperative appearance of a non-recurrent inferior laryngeal nerve. This will undoubtedly be of significance for all of those performing invasive diagnostic and surgical procedures in the neck and upper thoracic regions, in order to minimize the risk of iatrogenic injury to this nerve. This is of extreme importance, since a unilateral lesion of this nerve may result in permanent hoarseness, and a bilateral lesion may lead to aphonia and life-threatening dyspnea.

  5. Concurrent Chemotherapy and Intensity-Modulated Radiotherapy for Locoregionally Advanced Laryngeal and Hypopharyngeal Cancers

    International Nuclear Information System (INIS)

    Lee, Nancy Y.; O'Meara, William; Chan, Kelvin; Della-Bianca, Cesar; Mechalakos, James G.; Zhung, Joanne; Wolden, Suzanne L.; Narayana, Ashwatha; Kraus, Dennis; Shah, Jatin P.; Pfister, David G.

    2007-01-01

    Purpose: To perform a retrospective review of laryngeal/hypopharyngeal carcinomas treated with concurrent chemotherapy and intensity-modulated radiotherapy (IMRT). Methods and Materials: Between January 2002 and June 2005, 20 laryngeal and 11 hypopharyngeal carcinoma patients underwent IMRT with concurrent platinum-based chemotherapy; most patients had Stage IV disease. The prescription of the planning target volume for gross, high-risk, and low-risk subclinical disease was 70, 59.4, and 54 Gy, respectively. Acute/late toxicities were retrospectively scored using the Common Toxicity Criteria scale. The 2-year local progression-free, regional progression-free, laryngectomy-free, distant metastasis-free, and overall survival rates were calculated using the Kaplan-Meier method. Results: The median follow-up of the living patients was 26 months (range, 17-58 months). The 2-year local progression-free, regional progression-free, laryngectomy-free, distant metastasis-free, and overall survival rate was 86%, 94%, 89%, 92%, and 63%, respectively. Grade 2 mucositis or higher occurred in 48% of patients, and all experienced Grade 2 or higher pharyngitis during treatment. Xerostomia continued to decrease over time from the end of RT, with none complaining of Grade 2 toxicity at this analysis. The 2-year post-treatment percutaneous endoscopic gastrostomy-dependency rate for those with hypopharyngeal and laryngeal tumors was 31% and 15%, respectively. The most severe late complications were laryngeal necrosis, necrotizing fascitis, and a carotid rupture resulting in death 3 weeks after salvage laryngectomy. Conclusion: These preliminary results have shown that IMRT achieved encouraging locoregional control of locoregionally advanced laryngeal and hypopharyngeal carcinomas. Xerostomia improved over time. Pharyngoesophageal stricture with percutaneous endoscopic gastrostomy dependency remains a problem, particularly for patients with hypopharyngeal carcinoma and, to a lesser

  6. Increased respiratory neural drive and work of breathing in exercise-induced laryngeal obstruction.

    Science.gov (United States)

    Walsted, Emil S; Faisal, Azmy; Jolley, Caroline J; Swanton, Laura L; Pavitt, Matthew J; Luo, Yuan-Ming; Backer, Vibeke; Polkey, Michael I; Hull, James H

    2018-02-01

    Exercise-induced laryngeal obstruction (EILO), a phenomenon in which the larynx closes inappropriately during physical activity, is a prevalent cause of exertional dyspnea in young individuals. The physiological ventilatory impact of EILO and its relationship to dyspnea are poorly understood. The objective of this study was to evaluate exercise-related changes in laryngeal aperture on ventilation, pulmonary mechanics, and respiratory neural drive. We prospectively evaluated 12 subjects (6 with EILO and 6 healthy age- and gender-matched controls). Subjects underwent baseline spirometry and a symptom-limited incremental exercise test with simultaneous and synchronized recording of endoscopic video and gastric, esophageal, and transdiaphragmatic pressures, diaphragm electromyography, and respiratory airflow. The EILO and control groups had similar peak work rates and minute ventilation (V̇e) (work rate: 227 ± 35 vs. 237 ± 35 W; V̇e: 103 ± 20 vs. 98 ± 23 l/min; P > 0.05). At submaximal work rates (140-240 W), subjects with EILO demonstrated increased work of breathing ( P respiratory neural drive ( P respiratory mechanics and diaphragm electromyography with endoscopic video, we demonstrate, for the first time, increased work of breathing and respiratory neural drive in association with the development of EILO. Future detailed investigations are now needed to understand the role of upper airway closure in causing exertional dyspnea and exercise limitation. NEW & NOTEWORTHY Exercise-induced laryngeal obstruction is a prevalent cause of exertional dyspnea in young individuals; yet, how laryngeal closure affects breathing is unknown. In this study we synchronized endoscopic video with respiratory physiological measurements, thus providing the first detailed commensurate assessment of respiratory mechanics and neural drive in relation to laryngeal closure. Laryngeal closure was associated with increased work of breathing and respiratory neural drive preceded by an

  7. Primary laryngeal leishmaniasis: A rare case report

    Directory of Open Access Journals (Sweden)

    Kumar Bipin

    2009-01-01

    Full Text Available Laryngeal leishmaniasis is extremely rare. We report a case of primary laryngeal leishmaniasis in a 70-year-old male who was admitted with complaints of gradual progressive hoarseness of the voice, dyspnea, cough for the past 3 months and noisy breathing for the past 5 days. An X-ray of the soft tissue of the neck showed a prevertebral soft tissue mass causing narrowing of the airway at the C6-C7 vertebral level. A computerized tomography (CT scan showed a soft tissue mass in the subglottic region causing significant narrowing of the airway. A direct laryngoscopy showed a pinkish-white, friable mass involving the subglottic region and the anterior half of the vocal cords. With the clinical suspicion of malignancy, an endoscopic biopsy was done. A histopathological examination showed diffuse mixed inflammatory cell infiltrate in subepithelium with numerous Leishmania donovani bodies in the cytoplasm of histiocytes.

  8. Transforming growth factor alpha and epidermal growth factor in laryngeal carcinomas demonstrated by immunohistochemistry

    DEFF Research Database (Denmark)

    Christensen, M E; Therkildsen, M H; Poulsen, Steen Seier

    1993-01-01

    the basal cell layer. The present investigation and our previous results confirm the existence of EGF receptors, TGF-alpha and EGF in laryngeal carcinomas. In addition, we conclude that the conditions do exist for growth factors to act through an autocrine system in poorly differentiated tumours and through......Fifteen laryngeal squamous cell carcinomas were investigated for the presence of transforming growth factor alpha (TGF-alpha) and epidermal growth factor (EGF) using immunohistochemical methods. In a recent study the same material was characterized for epidermal growth factor receptors (EGF...... receptors) which were confined predominantly to the undifferentiated cells. The expression of this growth factor system in malignant cells may play a role in carcinogenesis and/or tumour growth. All carcinomas were positive for TGF-alpha and 12 were positive for EGF. In moderately-to-well differentiated...

  9. Functional reinnervation of vocal folds after selective laryngeal adductor denervation-reinnervation surgery for spasmodic dysphonia.

    Science.gov (United States)

    DeConde, Adam S; Long, Jennifer L; Armin, Bob B; Berke, Gerald S

    2012-09-01

    Selective laryngeal adductor denervation-reinnervation surgery (SLAD-R) offers a viable surgical alternative for patients with adductor spasmodic dysphonia refractory to botulinum toxin injections. SLAD-R selectively denervates the symptomatic thyroarytenoid muscle by dividing the distal adductor branch of the recurrent laryngeal nerve (RLN), and preventing reinnervation, by the proximal RLN and maintaining vocal fold bulk and tone by reinnervating the distal RLN with the ansa cervicalis. We present a patient who had previously undergone successful SLAD-R but presented 10 years postoperatively with a new regional dystonia involving his strap muscles translocated to his reinnervated larynx by his previous ansa-RLN neurorraphy. The patient's symptomatic vocal fold adduction resolved completely on division of the ansa-RLN neurorraphy confirming successful selective functional reinnervation of vocal fold adductors by the ansa cervicalis. Copyright © 2012 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  10. Potential of laryngeal muscle regeneration using induced pluripotent stem cell-derived skeletal muscle cells.

    Science.gov (United States)

    Dirja, Bayu Tirta; Yoshie, Susumu; Ikeda, Masakazu; Imaizumi, Mitsuyoshi; Nakamura, Ryosuke; Otsuki, Koshi; Nomoto, Yukio; Wada, Ikuo; Hazama, Akihiro; Omori, Koichi

    2016-01-01

    Conclusion Induced pluripotent stem (iPS) cells may be a new potential cell source for laryngeal muscle regeneration in the treatment of vocal fold atrophy after recurrent laryngeal nerve paralysis. Objectives Unilateral vocal fold paralysis can lead to degeneration, atrophy, and loss of force of the thyroarytenoid muscle. At present, there are some treatments such as thyroplasty, arytenoid adduction, and vocal fold injection. However, such treatments cannot restore reduced mass of the thyroarytenoid muscle. iPS cells have been recognized as supplying a potential resource for cell transplantation. The aim of this study was to assess the effectiveness of the use of iPS cells for the regeneration of laryngeal muscle through the evaluation of both in vitro and in vivo experiments. Methods Skeletal muscle cells were generated from tdTomato-labeled iPS cells using embryoid body formation. Differentiation into skeletal muscle cells was analyzed by gene expression and immunocytochemistry. The tdTomato-labeled iPS cell-derived skeletal muscle cells were transplanted into the left atrophied thyroarytenoid muscle. To evaluate the engraftment of these cells after transplantation, immunohistochemistry was performed. Results The tdTomato-labeled iPS cells were successfully differentiated into skeletal muscle cells through an in vitro experiment. These cells survived in the atrophied thyroarytenoid muscle after transplantation.

  11. Laryngeal chondroma: report of a case and review of the literature

    International Nuclear Information System (INIS)

    Sanchez, A.; Gonzalez, A.; Baro, P.; Panades, M.J.

    1997-01-01

    Cartilaginous tumors of the larynx are very rare lesions that originate in the cricoid cartilage in 70% of cases. Their growth is slow and affects the subglottal region, although they can present intralaryngeal growth. They usually recur easily because complete resection is hard to achieve, and the histological findings can be very difficult to differentiate from those of chondrosarcoma, especially those of low grade. A histologically confirmed case of laryngeal chondroma is presented and the radiological and histological aspects are discussed. (Author) 14 refs

  12. Very Late-Onset Friedreich Ataxia with Laryngeal Dystonia

    Directory of Open Access Journals (Sweden)

    Silvia Rota

    2014-12-01

    Full Text Available Friedreich ataxia (FRDA is an autosomal recessive neurodegenerative disorder characterized by progressive gait and limb ataxia, cerebellar, pyramidal and dorsal column involvement, visual defects, scoliosis, pes cavus and cardiomyopathy. It is caused by a homozygous guanine-adenine-adenine (GAA trinucleotide repeat expansion in intron 1 of the frataxin gene (FXN on chromosome 9q13-q21.1. Onset is usually in the first or second decade of life; however, late-onset cases of Freidreich ataxia (LOFA, after the age of 25 years, and very late-onset cases of Freidreich ataxia (VLOFA, after the age of 40 years, have been reported. VLOFA is quite rare and usually presents a milder progression of the disease. We report the case of a 64-year-old woman affected with VLOFA whose first symptoms (balance and gait disturbances occurred at the age of 44 years. At the age of 62 years, she started complaining of a slowly progressive dysphonia showing the clinical aspects of laryngeal dystonia. Molecular analysis showed a 210- and 230-trinucleotide GAA repeat expansion in the two alleles of the FXN gene. Laryngeal dystonia has been reported only in very few cases of ataxia syndrome and never before in FRDA patients. It may represent a rare clinical manifestation of VLOFA thus confirming the high variability of the clinical spectrum of FRDA.

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

    Science.gov (United States)

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

    2016-08-01

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

  14. CHARACTERISTICS OF TREATMENT OF PATIENTS WITH ACUTE AND CHRONIC LARYNGITIS DEPENDING ON RESULTS OF BACTERIOLOGICAL EXAMINATION OF LARYNX MICROFLORA

    Directory of Open Access Journals (Sweden)

    K. I. Chuikova

    2014-01-01

    Full Text Available This paper describes study of larynx microflora in patients with acute laryngtis and exacerbations of chronic laryngitis. Therapeutic algorithm based on bacteriologic examination data was developed reduce. New algorithm helps reduce sickness terms and to decrease prevalence of desease.123 patients of age from 18 to 60 were under observation: 43 patients with acute laryngitis and 80 patients with exacerbation of chronic laryngitis. 22 patients with acute laryngitis and 58 patients with exacerbation of chronic laryngitis underwent etiotropic treatment with antibiotics. The group of comparison with acute laryngitis (21 patients and chronic laryngitis (22 patients received treatment according to the conventional scheme.Bacteriologic examination of larynx mucous, clinical study and functional voice test (time of maximum vowels phonation before and after treatment were carried out.As a result of the research it was established that the most common causative agent of acute and chronic inflammatory larynx diseases is S. aureus as a mono culture or combined with other bacterial associations (S. аnhemolyticus, Str. viridans etc..After the end of antibacterial therapy we found symptoms as hoarseness, irritation, dry laryngopharynx, hyperemia and swelling of larynx mucous reduced eather in new treatment group than controlled group. Time of maximum vowels phonation (in seconds also increased significantly.Sickness terms after prescription of new treatment was shorter than in comparison groups: (10.9 ± 7.9 days for acute laryngitis and (12.6 ± 7.3 days for chronic laryngitis respectively. Health index was 20.8 and 19.5% respectively.

  15. Innervation status in chronic vocal fold paralysis and implications for laryngeal reinnervation.

    Science.gov (United States)

    Lin, R Jun; Smith, Libby J; Munin, Michael C; Sridharan, Shaum; Rosen, Clark A

    2018-01-22

    Treatment options for symptomatic unilateral vocal fold paralysis (VFP) include vocal fold augmentation, laryngeal framework surgery, and laryngeal reinnervation. Laryngeal reinnervation (LR) has been suggested to provide "tone" to the paralyzed VF. This implies a loss of tone as a result of denervation without reinnervation. We performed laryngeal electromyography (LEMG) in patients with chronic VFP to understand the innervation status associated with a chronically paralyzed vocal fold. Retrospective review of LEMG data in adult patients with chronic VFP from January 2009 to December 2014. LEMG was performed at least 6 months after-onset of VFP. Qualitative LEMG, quantitative LEMG, and adductory synkinesis testing were performed, and the parameters were collected. Twenty-seven vocal folds were studied (23 unilateral VFP and 2 bilateral VFP). Average age was 59 ± 17 years. The median duration from recurrent laryngeal nerve injury to LEMG was 8.5 months (range 6-90 months). The majority of patients, 24 of 27 (89%), had motor unit potentials during phonation tasks on LEMG, and only 3 of 27 (11%) patients were electrically silent. Quantitative LEMG showed 287.8 mean turns per second (normal ≥ 400). Motor unit configuration was normal in 12 of 27 (44%), polyphasic in 12 of 27 (44%), and absent in the electrically silent patients. Adductory synkinesis was found in 6 of 20 (30%) patients. Chronic vocal fold paralysis is infrequently associated with absent motor-unit recruitment, indicating some degree of preserved innervation and/or reinnervation in these patients. LEMG should be part of the routine workup for chronic VFP prior to consideration of LR. 4. Laryngoscope, 2018. © 2018 The American Laryngological, Rhinological and Otological Society, Inc.

  16. Ortner's Syndrome: Secondary Laryngeal Paralysis Caused by a Great Thoracic Aorta Aneurysm

    Directory of Open Access Journals (Sweden)

    Zangirolami, Ana Claudia Alves

    2015-01-01

    Full Text Available Introduction Recurrent laryngeal nerve injury caused by cardiovascular disease is a rare condition, and often it is the only prominent sign of an imminent break of an aortic artery aneurysm. Objective To report left laryngeal paralysis caused by a great aortic arch aneurysm and to highlight the importance of an otorhinolaryngologic evaluation along with a thoracic radiologic study. Resumed Report A 42-year-old man complained of thickness of his voice and dysphagia for 3 months, but no thoracic pain or other relevant complaints. Video laryngoscopy revealed immobility of his left vocal fold in the paramedian position. Imaging was obtained for investigation, including magnetic resonance imaging of his thorax, which showed a fusiform aneurysm in the aortic arch, leading to recurrent compression of the left laryngeal nerve. The patient was successfully treated with endovascular repair of the aneurysm. At 2-month follow-up, there was still no recovery of the laryngeal mobility. Conclusion An aortic artery aneurysm can suddenly break, requiring emergency heart surgery, and the results can be fatal in many cases. We suggest routine exam of the vocal folds in all patients with a heart condition, and we review the literature and suggest the use of imaging to reduce the number of emergency procedures.

  17. Ortner's Syndrome: Secondary Laryngeal Paralysis Caused by a Great Thoracic Aorta Aneurysm.

    Science.gov (United States)

    Zangirolami, Ana Claudia Alves; Oliveira, Frederico Vieira de; Tepedino, Miguel Soares

    2015-04-01

    Introduction Recurrent laryngeal nerve injury caused by cardiovascular disease is a rare condition, and often it is the only prominent sign of an imminent break of an aortic artery aneurysm. Objective To report left laryngeal paralysis caused by a great aortic arch aneurysm and to highlight the importance of an otorhinolaryngologic evaluation along with a thoracic radiologic study. Resumed Report A 42-year-old man complained of thickness of his voice and dysphagia for 3 months, but no thoracic pain or other relevant complaints. Video laryngoscopy revealed immobility of his left vocal fold in the paramedian position. Imaging was obtained for investigation, including magnetic resonance imaging of his thorax, which showed a fusiform aneurysm in the aortic arch, leading to recurrent compression of the left laryngeal nerve. The patient was successfully treated with endovascular repair of the aneurysm. At 2-month follow-up, there was still no recovery of the laryngeal mobility. Conclusion An aortic artery aneurysm can suddenly break, requiring emergency heart surgery, and the results can be fatal in many cases. We suggest routine exam of the vocal folds in all patients with a heart condition, and we review the literature and suggest the use of imaging to reduce the number of emergency procedures.

  18. [The use of fenspiride for the combined treatment of exacerbation of chronic laryngitis].

    Science.gov (United States)

    Ryabova, M A

    The present study was carried out based at the Department of Otorhinolaryngology of I.P. Pavlov First State Medical University of Saint-Petersburg. The objective of this work was to elucidate the efficacy and safety of fenspiride therapy for the treatment of exacerbation of chronic laryngitis associated with an acute respiratory infection. The patients comprising the main group received fenspiride (Eurespal, 'Servier', France) at the standard dose in addition to the conventional therapy with the use of antibiotics, inhalation, and voice rest. The patients in the group of comparison were treated following the conventional protocol without fenspiride. The clinical symptoms evaluated based on the scoring system, the results of videolaryngoscopy, and computer-assisted analysis of the voice were compared before and after treatment in the patients of both groups. The results of the study have confirmed the high effectiveness and safety of fenspiride therapy of exacerbation of chronic laryngitis.

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

    NARCIS (Netherlands)

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

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

  20. External laryngeal injuries in children--comparison of diagnostic methods.

    Science.gov (United States)

    Zawadzka-Glos, L; Jakubowska, A; Frackiewicz, M; Brzewski, M

    2013-09-01

    The injuries of the larynx constitute around 1% of all injuries. The great majority of the injuries of the larynx happens during playing. The effects of these injuries may appear insignificant however, not always the direct clinical symptoms correlate with the degree of respiratory tract failure. The symptoms of laryngeal injuries depend on the extension and strength of the trauma and always relate to impair patency of respiratory tract. The aim of the study is to compare two diagnostic methods: laryngoscopy and ultrasonography in evaluation of laryngeal injuries in children. In the Department of Pediatric Otolaryngology, Medical University of Warsaw, in the period between 2004 and 2010 there were hospitalised 15 children with external injury of the larynx. From among 15 hospitalized children, 7 were qualified as not serious trauma and were treated preservatively and the other 8 as sever trauma. The mechanism of traumas was diverse. Dyspnea was a predominating symptom, the others included hoarsness, change in voice quality, even aphonia, pain while speaking and swallowing, cough and hemoptysis. Direct laryngoscopy is a standard in diagnostics of the injuries of the larynx. Ultrasonography of the larynx is recommended in every case of laryngeal injury as an additional non-invasive complementary diagnostic examination. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  1. Laryngeal assessment by videolaryngostroboscopy in patients with rheumatoid arthritis.

    Science.gov (United States)

    Gómez-Puerta, José A; Cisternas, Ariel; Hernández, M Victoria; Ruiz-Esquide, Virginia; Vilaseca, Isabel; Sanmartí, Raimon

    2014-01-01

    To evaluate the larynx involvement in patients with rheumatoid arthritis (RA) in a clinical setting and correlate with the different clinical features related to more aggressive disease. Cross-sectional study including 36 consecutive patients with RA. Reflux symptoms were evaluated by the Reflux Symptom Index (RSI) and vocal cord impairment by the Voice Handicap Index-10 (VHI-10). Laryngeal involvement was done by videolaryngostroboscopy (VLS). The mean age was 56,3 ± 14 years with a mean disease duration of 2,6 ± 3,1 years (range 0-16 years). Voice use was considered as professional users in 33%. Twenty-four (67%) out of 36 patients had abnormal findings of VLS. One patient had larynx nodules (bamboo nodules). Eleven patients (31%) were diagnosed with muscle tension dysphonia, and there were symptoms and signs of pharyngeal-laryngeal reflux in 23 (64%) patients. No signs of cricoarytenoid joint impairment was found. Organic larynx involvement was uncommon in patients with RA. However symptoms and signs of pharyngeal-laryngeal reflux were seen in around 60% of patients. There was no correlation between the clinical phenotype, severity of disease, immunological profile or treatment with VLS findings. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  2. Radical radiotherapy for T3 laryngeal cancers

    International Nuclear Information System (INIS)

    Uno, T.; Itami, J.; Kotaka, K.; Toriyama, M.

    1996-01-01

    From 1974 through 1992, 37 previously untreated patients with T3 laryngeal cancer (supraglottic 15, glottic 22) were treated with initial radical radiotherapy and surgery for salvage. Two-year local control rate with radiotherapy alone, ultimate voice preservation rate, and ultimate local control rate for T3 supraglottic cancer were 33%, 33%, and 60%, respectively. Corresponding figures for T3 glottic cancer were 32%, 23%, and 77%, respecitvely. Five-year cause-specific survival rate for T3 supraglottic cancer and glottic cancer were 47% and 77%, respectively. In T3 supraglottic cancer, none of the 4 patients with subglottic tumor extension attained local control by radiotherapy alone, and local-regional recurrence-free time were significantly shorter in patients with subglottic tumor extension or tracheostomy before radiotherapy. There were no serious late complications such as chondronecrosis, rupture of carotid artery attributed to radical radiotherapy, while 3 patients had severe laryngeal edema requiring total laryngectomy. (orig.) [de

  3. Immunohistochemical analysis of laryngeal muscles in normal horses and horses with subclinical recurrent laryngeal neuropathy.

    Science.gov (United States)

    Rhee, Hannah S; Steel, Catherine M; Derksen, Frederik J; Robinson, N Edward; Hoh, Joseph F Y

    2009-08-01

    We used immunohistochemistry to examine myosin heavy-chain (MyHC)-based fiber-type profiles of the right and left cricoarytenoideus dorsalis (CAD) and arytenoideus transversus (TrA) muscles of six horses without laryngoscopic evidence of recurrent laryngeal neuropathy (RLN). Results showed that CAD and TrA muscles have the same slow, 2a, and 2x fibers as equine limb muscles, but not the faster contracting fibers expressing extraocular and 2B MyHCs found in laryngeal muscles of small mammals. Muscles from three horses showed fiber-type grouping bilaterally in the TrA muscles, but only in the left CAD. Fiber-type grouping suggests that denervation and reinnervation of fibers had occurred, and that these horses had subclinical RLN. There was a virtual elimination of 2x fibers in these muscles, accompanied by a significant increase in the percentage of 2a and slow fibers, and hypertrophy of these fiber types. The results suggest that multiple pathophysiological mechanisms are at work in early RLN, including selective denervation and reinnervation of 2x muscle fibers, corruption of neural impulse traffic that regulates 2x and slow muscle fiber types, and compensatory hypertrophy of remaining fibers. We conclude that horses afflicted with mild RLN are able to remain subclinical by compensatory hypertrophy of surviving muscle fibers.

  4. Successful hyperbaric oxygen therapy for laryngeal radionecrosis after chemoradiotherapy for mesopharyngeal cancer. Case report and literature review

    International Nuclear Information System (INIS)

    Abe, Madoka; Terashima, Kotaro; Matsuo, Mioko; Uehara, Satoru; Shioyama, Yoshiyuki; Hara, Iwao

    2012-01-01

    Laryngeal radionecrosis is one of the most troublesome late complications of radiotherapy, because it is frequently resistant to treatment and laryngectomy is required in the worst case. Here, we report a case of laryngeal radionecrosis, successfully treated by use of hyperbaric oxygen (HBO) therapy, in which laryngectomy was avoided. A 67-year-old male received radical chemoradiotherapy (CRT) for mesopharyngeal cancer, which included radiotherapy with a total dose of 71.4 Gy/38 Fr and chemotherapy with CDDP + S-1. He developed dyspnea and throat pain 9 months after completion of CRT. Laryngoscopy revealed vocal cord impairment because of severe laryngeal edema. He was diagnosed as having laryngeal radionecrosis and initially received conservative therapy combined with antibiotics, steroids, and prostaglandins. Because his dyspnea was persistent despite this treatment, HBO therapy was administered 20 times, and resulted in complete remission of the dyspnea. HBO therapy, therefore, is regarded as an effective conservative therapeutic option for laryngeal radionecrosis. (author)

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

    Directory of Open Access Journals (Sweden)

    Rodrigo Bortoli

    2007-12-01

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

  6. High Frequency Jet Ventilation during Transoral Laser Microsurgery for Tis-T2 Laryngeal Cancer.

    Science.gov (United States)

    Mora, Francesco; Missale, Francesco; Incandela, Fabiola; Filauro, Marta; Parrinello, Giampiero; Paderno, Alberto; Della Casa, Palmiro; Piazza, Cesare; Peretti, Giorgio

    2017-01-01

    Transoral laser microsurgery (TLM) for early to intermediate laryngeal squamous cell cancer (SCC) can be technically challenging when adequate exposure of the posterior laryngeal compartment is required due to the presence of the orotracheal tube. The goal of our study was to analyze the efficacy of high frequency jet ventilation (HFJV) in achieving appropriate laryngeal exposure and safe oncologic resection of lesions located in such a position. We reviewed the clinical records of 62 patients affected by Tis-T2 SCC of the posterior laryngeal compartment treated by TLM between 02/2012 and 12/2016. The cohort was divided into two groups according to the anesthesiologic technique used: Group A included patients treated using intraoperative infraglottic HFJV, while Group B encompassed patients treated by standard orotracheal intubation. The main outcome was postoperative surgical margin status. Group comparison analysis was performed. Significant difference in deep margin status was observed between the two groups: in Group A, the rate of negative deep margins was 86% compared to 56% in Group B ( p  = 0.04). A trend of better overall and superficial margin control was observed for patients treated using HFJV (Group A), although no statistical significance was achieved. Use of HFJV during TLM allows easier and safer management of patients affected by Tis-T2 SCC of the posterior laryngeal compartment, reducing the rates of positive superficial and deep surgical margins.

  7. Modified frontolateral partial laryngectomy operation: combined muscle-pedicle hyoid bone and thyrohyoid membrane flap in laryngeal reconstruction

    OpenAIRE

    Ouyang, Dian; Liu, Tian-Run; Chen, Yan-Feng; Wang, Jian

    2013-01-01

    Objective Laryngeal reconstruction is needed to preserve laryngeal function in patients who have undergone extensive vertical or frontal partial laryngectomy. However, the procedure remains a difficult challenge. Several reconstruction techniques have been described, but these techniques pose risks of complications such as laryngeal stenosis. This study aimed to evaluate the postoperative course and functional outcomes of a new technique that combined a muscle-pedicle hyoid bone and a thyrohy...

  8. Using endografts from superelastic titanium-nickelid-based alloy singular tissue plural tissues in organ-preserving surgery of laryngeal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Kulbakin, D. E., E-mail: kulbakin-d@mail.ru [Tomsk Cancer Research Institute, 5 Kooperativny Street, Tomsk, 634050 (Russian Federation); Tomsk State University, 36, Lenin Avenue, Tomsk, 634050 (Russian Federation); Mukhamedov, M. R., E-mail: muhamedov@oncology.tomsk.ru [Tomsk Cancer Research Institute, 5 Kooperativny Street, Tomsk, 634050 (Russian Federation); Siberian State Medical University, 2, Moscow Highway, Tomsk, 634050 (Russian Federation); Choynzonov, E. L., E-mail: choynzonov@gmail.com [Tomsk Cancer Research Institute, 5 Kooperativny Street, Tomsk, 634050 (Russian Federation); Siberian State Medical University, 2, Moscow Highway, Tomsk, 634050 (Russian Federation); National Research Tomsk Polytechnic University, 30, Lenin Avenue, Tomsk, 634050 (Russian Federation); Gynter, V. E., E-mail: tc77@rec.tsu.ru [Tomsk State University, 36, Lenin Avenue, Tomsk, 634050 (Russian Federation); Research Institute of Medical Materials, 17, 19 Gv. Divizii, Tomsk, 634034 (Russian Federation)

    2015-11-17

    Our study has demonstrated feasibility of performing larynx preservation surgeries in patients with recurrent laryngeal cancer after failure of radiotherapy. The technique of combined laryngeal reconstruction with endografts from superelastic titanium-nickelid-based alloy Singular tissue Plural tissues results in improvement of life quality by preserving laryngeal functions.

  9. Risk factors for recurrent laryngeal nerve neuropraxia postthyroidectomy.

    LENUS (Irish Health Repository)

    Sheahan, Patrick

    2012-06-01

    Despite preservation of the recurrent laryngeal nerve (RLN), transient vocal cord paralysis (VCP) occurs after 1.2% to 10.9% of thyroidectomies. The objective of this study was to study risk factors for transient VCP after thyroidectomy.

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

    Science.gov (United States)

    Zhao, Lei; Tian, Zhuang; Fang, Quan

    2016-03-01

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

  11. Heredity of supraglottic exercise-induced laryngeal obstruction

    DEFF Research Database (Denmark)

    Walsted, Emil Schwarz; Hvedstrup, Jeppe; Eiberg, Hans

    2017-01-01

    Respiratory symptoms on exertion, such as shortness of breath and wheezing, are commonly associated with asthma, but might also arise from the larynx [1–3]. In recent years, the emergence of exercise laryngoscopy [4] has led to a better understanding of laryngeal movement during exercise, and ins...

  12. Laryngeal squamous cell papilloma is highly associated with human papillomavirus.

    Science.gov (United States)

    Orita, Yorihisa; Gion, Yuka; Tachibana, Tomoyasu; Ikegami, Kana; Marunaka, Hidenori; Makihara, Seiichiro; Yamashita, Yasuhiko; Miki, Kentaro; Makino, Takuma; Akisada, Naoki; Akagi, Yusuke; Kimura, Miyuki; Yoshino, Tadashi; Nishizaki, Kazunori; Sato, Yasuharu

    2018-04-01

    To delineate the association between characteristics of adult-onset laryngeal squamous cell papilloma and human papillomavirus (HPV) infection. Clinical records and paraffin-embedded specimens of 77 papilloma patients who had been treated between 1998 and 2014 were collected. Of the 77 cases, 34 were identified in the larynx, 28 in the oral cavity and 15 in the oropharynx. Specimens were investigated by polymerase chain reaction (PCR) to detect HPV 6, 11, 16, 18, 31, 33, 35, 52b and 58, and immunohistochemical (IHC) staining for anti-p16INK4a antibody. In 21 cases (61.8%) with laryngeal squamous cell papilloma, various types of HPV were detected: 14 cases (41.2%) were positive of high-risk HPV, 18 (52.9%) were positive of low-risk HPV and 11 (32.4%) were positive of both high-risk HPV and low-risk HPV. Younger patients (papilloma, no malignant transformation was observed during the study period. With IHC staining, positive expression of p16 was observed in 20 cases (58.8%). HPV infection and p16-expression were associated with the pathological finding of koilocytosis. Only four cases (14.3%) showed HPV-positivity in the oral cavity, and none of the 15 oropharyngeal cases were positive for HPV, and none of the oral cavity and oropharyngeal cases showed koilocytosis. Results of HPV-PCR and p16-IHC staining were significantly correlated each other. HPV infection is frequently associated with laryngeal squamous cell papilloma, and koilocytosis is a characteristic pathological finding. To the best of our knowledge, this is the first report which have described infections with multiple HPV types in laryngeal papilloma.

  13. [Prehospital airway management of laryngeal tubes. Should the laryngeal tube S with gastric drain tube be preferred in emergency medicine?].

    Science.gov (United States)

    Dengler, V; Wilde, P; Byhahn, C; Mack, M G; Schalk, R

    2011-02-01

    Laryngeal tubes (LT) are increasingly being used for emergency airway management. This article reports on two patients in whom out-of-hospital intubation with a single-lumen LT was associated with massive pulmonary aspiration in one patient and gastric overinflation in the other. In both cases peak inspiratory pressures exceeded the LT leak pressure of approximately 35 mbar. This resulted in gastric inflation and decreased pulmonary compliance and increased inspiratory pressure further, thereby creating a vicious circle. It is therefore recommended that laryngeal tube suction (LTS) should be used in all cases of emergency airway management and a gastric drain tube be inserted through the dedicated second lumen. Apart from gastric overinflation, incorrect LT/LTS placement must be detected and immediately corrected, e.g. in cases of difficult or impossible gastric tube placement, permanent drainage of air from the gastric tube, decreasing minute ventilation or an ascending capnography curve.

  14. Anesthesia management in pediatric patients with laryngeal papillomatosis undergoing suspension laryngoscopic surgery and a review of the literature.

    Science.gov (United States)

    Bo, Lin; Wang, Bing; Shu, Shi-Yu

    2011-11-01

    The excision of laryngeal papillomas poses a great challenge for both the anesthesiologist and the surgeon. The narrowness of the airways and the great variability of the pathological lesions necessitate close collaboration between the surgical and anesthesia teams to provide optimal operating conditions and ensure adequate ventilation and oxygenation. Our aim was to explore perioperative anesthesia management in pediatric patients during the excision of laryngeal papillomas with a suspension laryngoscope. Fifty-eight pediatric patients suffering from laryngeal papillomas were included in this retrospective study. These patients had degrees of laryngeal obstruction from I to III and underwent suspension laryngoscopic surgery to excise laryngeal papillomas between January 2007 and December 2010. The American Society of Anesthesiologists (ASA) physical status of the patients ranged from I to III. Anesthesia was induced by intravenous administration. Once the child was unconscious, a 2% lidocaine aerosol solution was sprayed over the laryngeal area directly under the laryngoscope. For patients to tolerate suspension laryngoscopy, it is necessary to maintain spontaneous breathing and ensure adequate anesthesia depth. The airway was secured, and sufficient ventilation was established throughout a tracheal tube (ID 2.5 or 3.0) which was placed close to glottis and connected to Jackson Rees system. Hemodynamic parameters and pulse oxygen saturation (SpO(2)) were closely monitored, and adverse events were recorded. Most of the patients 89% (52/58) were hemodynamically stable during the perioperative period. Laryngospasm and laryngeal edema occurred in several children during emergence from the anesthesia. Tracheal intubations were performed in six patients (10.3%). Tracheotomies were performed in two patients. One patient had to be sent to the ICU for comprehensive therapy. The most important consideration for anesthesia during suspension laryngoscopy is (1) the

  15. Outcomes and Resource Utilization of Endoscopic Mass-Closure Technique for Laryngeal Clefts.

    Science.gov (United States)

    Balakrishnan, Karthik; Cheng, Esther; de Alarcon, Alessandro; Sidell, Douglas R; Hart, Catherine K; Rutter, Michael J

    2015-07-01

    To compare resource utilization and clinical outcomes between endoscopic mass-closure and open techniques for laryngeal cleft repair. Case series with chart review. Tertiary academic children's hospital. Pediatric patients undergoing repair for Benjamin-Inglis type 1-3 laryngeal clefts over a 15-year period. All 20 patients undergoing endoscopic repair were included. Eight control patients undergoing open repair were selected using matching by age and cleft type. Demographic, clinical, and resource utilization data were collected. Twenty-eight patients were included (20 endoscopic, 8 open). Mean age, rates of tracheostomy and vocal fold immobility, and distribution of cleft types were not different between the 2 groups (all P > .2). Mean operative time (P = .004) and duration of hospital stay (P group. All repairs were intact in both groups at final postoperative endoscopy. Rates of persistent laryngeal penetration or aspiration on swallow study were not different between groups (P = 1.000), although results were available for only 11 patients. Endoscopic laryngeal cleft repair using a mass-closure technique provides a durable result while requiring significantly shorter operative times and hospital stays than open repair and avoiding the potential morbidity of laryngofissure. However, open repair may allow the simultaneous performance of other airway reconstructive procedures and may be a useful salvage technique when endoscopic repair fails. Postoperative swallowing results require further study. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  16. Laryngeal chondrosarcoma: A systematic review of 592 cases.

    Science.gov (United States)

    Chin, Oliver Y; Dubal, Pariket M; Sheikh, Ahmed B; Unsal, Aykut A; Park, Richard Chan Woo; Baredes, Soly; Eloy, Jean Anderson

    2017-02-01

    Laryngeal chondrosarcomas are rare entities that arise from the cartilaginous structures of the larynx, including the cricoid, thyroid cartilage, epiglottis, and arytenoid cartilages. These tumors represent a minority of malignancies involving the larynx and can be mistaken for benign pathologies. The treatment has historically been surgical excision, often by total laryngectomy. This review investigates treatment modalities and patient outcomes. Systematic review using PubMed/MEDLINE and EMBASE database. The databases were used to identify articles reporting cases of chondrosarcomas occurring exclusively in the larynx. Variables analyzed included patient demographics, presenting symptoms, grade, therapeutic approach, patient outcomes, and follow-up. Five hundred and ninety-two cases were identified. The average age reported was 62.5 years. There was a 3:1 male to female ratio. The most common surgical approach was local excision in 178 cases, followed by total laryngectomy in 174 cases. Nonsurgical treatment such as radiotherapy and chemotherapy was only used in 0.8% and 0.2%, respectively. Disease-specific survival rates for 1, 5, 10, and 20 years were 97.7%, 91.4%, 81.8%, and 68.0%, respectively, with no differences when comparing 5-year survival rates for location, grade, and therapy. Laryngeal chondrosarcomas are rare with a good prognosis. Various surgical approaches exist, with no difference noted in 5-year survival outcomes. Nonsurgical approaches were rarely used for these lesions. N/A. Laryngoscope, 2016 127:430-439, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  17. Reinnervation of the diaphragm by the inferior laryngeal nerve to the phrenic nerve in ventilator-dependent tetraplegic patients with C3-5 damage.

    Science.gov (United States)

    Verin, Eric; Morelot-Panzini, Capucine; Gonzalez-Bermejo, Jesus; Veber, Benoit; Perrouin Verbe, Brigitte; Soudrie, Brigitte; Leroi, Anne Marie; Marie, Jean Paul; Similowski, Thomas

    2017-10-01

    The aim of this study was to evaluate the feasibility of unilateral diaphragmatic reinnervation in humans by the inferior laryngeal nerve. This pilot study included chronically ventilated tetraplegic patients with destruction of phrenic nerve motoneurons. Five patients were included. They all had a high level of tetraplegia, with phrenic nerve motor neuron destruction. They were highly dependent on ventilation, without any possibility of weaning. They did not have other chronic pathologies, especially laryngeal disease. They all had diaphragmatic explorations to diagnose the destruction of the motoneurons of the phrenic nerves and nasoendoscopy to be sure that they did not have laryngeal or pharyngeal disease. Then, surgical anastomosis of the right phrenic nerve was performed with the inferior laryngeal nerve, by a cervical approach. A laryngeal reinnervation was performed at the same time, using the ansa hypoglossi. One patient was excluded because of a functional phrenic nerve and one patient died 6 months after the surgery of a cardiac arrest. The remaining three patients were evaluated after the anastomosis every 6 months. They did not present any swallowing or vocal alterations. In these three patients, the diaphragmatic explorations showed that there was a recovery of the diaphragmatic electromyogram of the right and left hemidiaphragms after 1 year. Two patients had surgical diaphragmatic explorations for diaphragmatic pacing 18-24 months after the reinnervation with excellent results. At 36 months, none of the patients could restore their automatic ventilation. In conclusion, this study demonstrated that diaphragmatic reinnervation by the inferior laryngeal nerve is effective, without any vocal or swallowing complications.

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

    Directory of Open Access Journals (Sweden)

    Jorge Montessi

    2007-06-01

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

  19. Real-time in vivo diagnosis of laryngeal carcinoma with rapid fiber-optic Raman spectroscopy

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    Lin, Kan; Zheng, Wei; Lim, Chwee Ming; Huang, Zhiwei

    2016-01-01

    We assess the clinical utility of a unique simultaneous fingerprint (FP) (i.e., 800-1800 cm−1) and high-wavenumber (HW) (i.e., 2800-3600 cm−1) fiber-optic Raman spectroscopy for in vivo diagnosis of laryngeal cancer at endoscopy. A total of 2124 high-quality in vivo FP/HW Raman spectra (normal = 1321; cancer = 581) were acquired from 101 tissue sites (normal = 71; cancer = 30) of 60 patients (normal = 44; cancer = 16) undergoing routine endoscopic examination. FP/HW Raman spectra differ significantly between normal and cancerous laryngeal tissue that could be attributed to changes of proteins, lipids, nucleic acids, and the bound water content in the larynx. Partial least squares-discriminant analysis and leave-one tissue site-out, cross-validation were employed on the in vivo FP/HW tissue Raman spectra acquired, yielding a diagnostic accuracy of 91.1% (sensitivity: 93.3% (28/30); specificity: 90.1% (64/71)) for laryngeal cancer identification, which is superior to using either FP (accuracy: 86.1%; sensitivity: 86.7% (26/30); specificity: 85.9% (61/71)) or HW (accuracy: 84.2%; sensitivity: 76.7% (23/30); specificity: 87.3% (62/71)) Raman technique alone. Further receiver operating characteristic analysis reconfirms the best performance of the simultaneous FP/HW Raman technique for laryngeal cancer diagnosis. We demonstrate for the first time that the simultaneous FP/HW Raman spectroscopy technique can be used for improving real-time in vivo diagnosis of laryngeal carcinoma during endoscopic examination. PMID:27699131

  20. High Frequency Jet Ventilation during Transoral Laser Microsurgery for Tis-T2 Laryngeal Cancer

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    Francesco Mora

    2017-11-01

    Full Text Available BackgroundTransoral laser microsurgery (TLM for early to intermediate laryngeal squamous cell cancer (SCC can be technically challenging when adequate exposure of the posterior laryngeal compartment is required due to the presence of the orotracheal tube. The goal of our study was to analyze the efficacy of high frequency jet ventilation (HFJV in achieving appropriate laryngeal exposure and safe oncologic resection of lesions located in such a position.MethodsWe reviewed the clinical records of 62 patients affected by Tis-T2 SCC of the posterior laryngeal compartment treated by TLM between 02/2012 and 12/2016. The cohort was divided into two groups according to the anesthesiologic technique used: Group A included patients treated using intraoperative infraglottic HFJV, while Group B encompassed patients treated by standard orotracheal intubation. The main outcome was postoperative surgical margin status. Group comparison analysis was performed.ResultsSignificant difference in deep margin status was observed between the two groups: in Group A, the rate of negative deep margins was 86% compared to 56% in Group B (p = 0.04. A trend of better overall and superficial margin control was observed for patients treated using HFJV (Group A, although no statistical significance was achieved.ConclusionUse of HFJV during TLM allows easier and safer management of patients affected by Tis-T2 SCC of the posterior laryngeal compartment, reducing the rates of positive superficial and deep surgical margins.