Cases reported "Amyloidosis"

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1/182. Tc-99m HDP uptake in cardiac amyloidosis.

    amyloidosis is characterized by the soft-tissue deposition of amyloid protein. It may occur as a primary disorder but more often is seen as a manifestation of chronic illness. Scattered reports of the affinity of amyloid for bone scanning agents have appeared over many years. Isolated cardiac uptake of Tc-99m HDP is described in a patient with biopsy-proved cardiac amyloidosis on a background of tuberculosis, prostate cancer, and coronary artery disease.
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2/182. Generalized AA-amyloidosis in a 58-year-old Caucasian woman with an 18-month history of gastrointestinal tuberculosis.

    We report on a 58-year-old Caucasian woman who went to a general practitioner about recurrent abdominal pain, night sweats and weight loss of a few weeks' duration. Once gynaecological disease had been ruled out, the patient was admitted to hospital with severe abdominal pain and intestinal obstruction and a right-sided hemicolectomy was performed. Following the investigation of osteolytic lumbar vertebrae, 18 months after visiting the general practitioner the patient was finally found to be suffering from generalized AA-amyloidosis secondary to gastrointestinal tuberculosis. This had been misinterpreted as Crohn's disease. Re-examination of the specimens from the right-sided hemicolectomy demonstrated that scanty deposits of AA-amyloid were present 9 months after the first presentation. AA-amyloid can thus be present in serious inflammatory disease even during the first 9 months after the initial clinical presentation.
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keywords = abdominal pain
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3/182. Systemic amyloidosis and sacroiliitis in a patient with systemic lupus erythematosus.

    We report a case of a 25-year-old female with juvenile onset systemic lupus erythematosus who developed systemic secondary amyloidosis with renal and gastrointestinal involvement. She has also had radiological signs of bilateral asymptomatic sacroiliitis without lower back pain or hla-b27 antigen.
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4/182. Primary amyloidosis presenting as a nasopharyngeal mass.

    Amyloid is defined as a pathologic proteinaceous substance which, when deposited between the cells of tissues and organs, leads to various clinical conditions. immunohistochemistry has allowed for better classification and understanding of the pathophysiology of amyloidosis. In the upper aerodigestive tract, amyloidosis is a rare condition occurring most frequently in the larynx. We present the case of a 42-year-old woman with complete nasal obstruction due to primary nasopharyngeal amyloidosis. This represents the first reported case of primary nasopharyngeal amyloidosis containing both the lambda and kappa immunoglobulin light chains. The clinical and radiologic findings, as well as the management of primary amyloidosis of the upper aerodigestive tract, will be discussed. A review of the literature pertaining to nasal and nasopharyngeal amyloidosis will be presented.
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5/182. Monoclonality of infiltrating plasma cells in primary pulmonary nodular amyloidosis: detection with polymerase chain reaction.

    AIMS: To investigate the relation between localised amyloidosis and immunocytic dyscrasia. methods: Open lung biopsy specimens from a 72 year old man with multiple nodules in the right middle and lower lung were stained with haematoxylin-eosin, congo red, and antibodies against IgG, IgA, IgM, and kappa and lambda light chains. Semi-nested PCR amplification for the immunoglobulin heavy chain (IgH) gene was performed using consensus primers for the VDJ region of the IgH gene, FR3A, LJH, and VLJH. RESULTS: The biopsy specimens contained eosinophilic amorphous material stained with Congro red and anti-kappa light chain, and surrounded by inflammatory cells intermingled with plasma cells. plasma cells in the adjacent amorphous material showed cytoplasmic staining with anti-kappa. polymerase chain reaction revealed a discrete amplified band of apparently uniform size with background smear. CONCLUSIONS: Primary AL type localised amyloidosis involves local accumulation of monoclonal plasma cells and their secreted products, as in nodular cutaneous amyloidosis. Localised AL type nodular amyloidosis is a separate entity in amyloidosis.
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6/182. Pulmonary alveolar-septal amyloidosis presenting as heart failure with unilateral pleural effusion: a case report.

    We report a case of pulmonary alveolar-septal amyloidosis associated with chylothorax and paraproteinemia initially presenting as congestive heart failure with unilateral pleural effusion. A 72-year-old man was initially diagnosed with congestive heart failure based on the correlation of clinical manifestation and chest radiography. Concentric left ventricular hypertrophy with mild hypokinesis of the left ventricle was found on cardiac echography. Thoracocentesis was performed for right-sided pleural effusion, which persisted despite medical treatment; chylous exudate was aspirated. Because the etiology of the exudative pleural effusion was undetermined, the patient underwent a thoracotomy that showed pulmonary alveolar-septal amyloidosis. immunoglobulin m paraproteinemia was identified by serum immunoelectrophoresis. We conclude that it is imperative to search for the cause of an undetermined exudative pleural effusion, with particular attention to chylothorax and amyloidosis as the differential diagnoses.
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ranking = 3.040287119071
keywords = chest
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7/182. Familial primary cutaneous amyloidosis in a South African family.

    Four members of a white South African family, spanning four generations were diagnosed as suffering from familial primary lichen amyloidosis. All showed similar clinical features which included scaling papules on the lower legs and arms, and a pebbled, lichenified appearance of the skin on the back. The diagnosis was confirmed by light and electron microscopy. This is the eleventh report of familial primary cutaneous amyloidosis and the first in south africa.
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8/182. Amyloid goiter with parathyroid involvement: a case report and review of the literature.

    Although involvement of the thyroid gland by amyloid is a relatively common phenomenon, diffuse enlargement of the gland secondary to infiltration by amyloid is infrequent. We report the pathologic findings in a case of primary amyloid goiter with involvement of a parathyroid gland in an euthyroid patient who presented to his physician with an enlarged thyroid, upper airway obstructive symptoms, and dysphagia. The clinical features of amyloid goiter, including difficulties in preoperative diagnosis and management, are discussed.
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9/182. Bronchial amyloidosis successfully treated with low-dose long-term erythromycin therapy.

    A 69-year-old man was admitted for evaluation of an abnormal chest X-ray. A diagnosis of primary bronchial amyloidosis was made on the basis of the chest X-ray, CT scans and bronchial biopsy specimens. The patient was treated with low-dose long-term erythromycin therapy (600 mg/day). After four months of therapy, chest CT scans, bronchoscopic findings and bronchial biopsy specimens revealed significant improvement of inflammatory changes. Low-dose erythromycin therapy may be helpful in terms of its anti-inflammatory effects for patients with bronchial amyloidosis.
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ranking = 9.120861357213
keywords = chest
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10/182. A case of extramedullary plasmacytoma arising from the nasal septum.

    A rare case of extramedullary plasmacytoma arising from the nasal septum with localized amyloid deposition is reported. A 75-year-old woman presented with a history of post-nasal discharge and nasal obstruction for several months. Endonasal endoscopic observation revealed the presence of a mass arising from her nasal septum with an extension to the nasopharynx. No cervical lymph nodes were palpable. The biopsy specimen was diagnosed as a plasmacytoma (IgG, lambda-light chain type). Both serum myeloma-protein and urine Bence-Jones protein were negative. bone marrow biopsy, a chest radiograph, total body skeletal survey and 67Ga- and 99mTc-scintigrams showed no other systemic lesions. These findings confirmed the diagnosis of extramedullary plasmacytoma in the nasal septum. The patient received irradiation of 40 Gy without clinically detectable reduction of tumour size. The patient eventually underwent complete resection of the tumour by KTP/532 laser under endonasal endoscopic control. Pathologically, the tumour mass was composed mainly of amyloid deposition with a marked reduction of tumour cells. This indicated the radiosensitiveness of tumour cells, which was clinically masked by the increased amyloid deposition. The clinical presentation, pathological features and surgical procedures are described with a review of the literature.
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keywords = chest
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