Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloid deposition in secondary amyloidosis frequently involves thyroid gland, but rarely is responsible of a goiter. Amyloid goiter in secondary amyloidosis is characterized by deposition of amyloid A protein (AA) in the gland, associated to atrophic follicles. We identified cases of amyloid goiter in the files of our department in the period from 1985 to 1998. Five cases of amyloid goiter with ingravescent symptomatology, characterized by dyspnea, dysphagia and hoarseness were selected. In four cases of five we observed predisposing conditions as, for example, tuberculosis, Crohn's disease, or rheumatoid arthritis. In all cases the symptoms relative to thyroid enlargement preceded or, anyway, predominated over other clinical evidence of systemic amyloidosis. In one case a symptomatology of systemic amyloidosis was not evident. We would like to underline that in all cases the immunoreactivity for amyloid A in the amorphous material present in the gland permitted the diagnosis of secondary amyloidosis even in the absence of systemic symptoms.
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PMID:Amyloid goiter: the first evidence in secondary amyloidosis. Report of five cases and review of literature. 1108 24

Oesophageal motility disorders comprise various abnormal manometric patterns which usually present with dysphagia or chest pain. Some, such as achalasia, are diseases with a well defined pathology, characteristic manometric features, and good response to treatments directed at the pathophysiological abnormalities. Other disorders, such as diffuse oesophageal spasm and hypercontracting oesophagus, have no well defined pathology and could represent a range of motility changes associated with subtle neuropathic changes, gastro-oesophageal reflux, and anxiety states. Although manometric patterns have been defined for these disorders, the relation with symptoms is poorly defined and the response to medical or surgical therapy unpredictable. Hypocontracting oesophagus is generally caused by weak musculature commonly associated with gastro-oesophageal reflux disease. Secondary oesophageal motility disorders can be caused by collagen vascular diseases, diabetes, Chagas' disease, amyloidosis, alcoholism, myxo-oedema, multiple sclerosis, idiopathic pseudo-obstruction, or the ageing process.
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PMID:Oesophageal motility disorders. 1180 95

Amyloidoses are a group of disorders in which deposition of abnormal amounts of protein complexes (amyloid) occurs in a variety of tissues. The upper aerodigestive tract may be affected, particularly the larynx, but hypopharyngeal involvement is rarely reported. We present a unique case of amyloidosis of the post-cricoid region causing dysphagia. This case report highlights the need for otolaryngologists to consider the possibility of submucosal amyloid deposition, in the absence of mucosal lesions, in patients who present with dysphagia secondary to an obstructive lesion of the post cricoid region.
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PMID:A rare case of dysphagia: hypopharyngeal amyloidosis masquerading as a post-cricoid tumour. 1186 Jun 56

Laryngeal amyloidosis is an uncommon disease accounting for 0.68% of benign tumors of the larynx. It can present as a nodular tumor or diffuse subepithelial deposition forming a bulging mass and causing hoarseness, dysphagia or even airway obstruction. We present our experience in the management of laryngeal amyloidosis, and discuss the clinical features and histopathological characteristics of the disease. Reports on benign laryngeal tumor from June 1988 to June 1998 at Taipei VGH were searched to identify and collect amyloid cases for chart review. Five cases of laryngeal amyloidosis were found in the records. The sites of involvement were false cord, ventricle, post-cricoid, and arytenoid-epiglottic fold. No evidence of systemic amyloidosis involvement was observed for any of the five cases. All cases were treated with CO2 laser excision. Recurrence occurred in two patients within one year. More extensive CO2 laser excision was subsequently performed, with no more recurrences noted as of the last follow-ups. Laryngeal amyloidosis is a benign disease; however, surgical management is required when it is symptomatic. Recurrences or large-sized tumors may be a problem; however, advances in laser technology and the cumulative experience of treating benign lesions of the larynx have made CO2 laser endoscopic excision an effective treatment option.
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PMID:Management of laryngeal amyloidosis--the experience of Taipei Veterans General Hospital. 1193 73

Rheumatic diseases cover a wide spectrum of clinical syndromes and frequently present with gastrointestinal alterations. Systemic amyloidosis is associated with infectious diseases or chronic inflammatory processes such as rheumatoid arthritis and it can also affect the gastrointestinal tract. Although esophageal involvement is difficult to quantify because its course is frequently asymptomatic, systemic amyloidosis is recognized as a cause of motor disorders of the esophagus. Typical manometric patterns, including achalasia, are usually absent. Esophageal involvement due to amyloid deposits usually corresponds to primary amyloidosis as only a few cases of secondary esophageal deposits (type AA) have been described. We describe a new case of this exceptional association that first presented as dysphagia in a patient with rheumatoid arthritis. The initial suspicion of pseudoachalasia led to the definitive diagnosis of secondary amyloidosis.
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PMID:[Pseudoachalasia and secondary amyloidosis in a patient with rheumatoid arthritis]. 1206 3

Laryngeal amyloidosis is relatively uncommon, accounting for only 0.2-1.5% of all laryngeal tumours. Hoarseness, dysphagia and stridor are potential clinical symptoms. A systemic amyloidosis should be excluded even though symptoms are only apparent in the head and neck region. For the localised form, surgical excision of the excess tissue is performed. We discuss the development of the disease, together with its diagnostics and therapy, based on the case report of a 42 year old female patient.
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PMID:[Voice-improving laser-surgical therapy in amyloidosis of the larynx]. 1525 42

The laryngeal amyloidosis is an uncommon disease accounting for 1% of all benign lesions of larynx. The commonest symptom is the dysphonia, sometimes accompanied by stridor, laryngeal globus sensation, dysphagia and, in rare occasions, cough, dyspnea and hemoptysis, specially when the tracheobronchial tree is also affected. This paper describes the case of a 30-year-old female patient, whose main symptoms were progressive dysphonia and dyspnea, admitted at allergy service to rule out asthma. The respiratory function tests showed obstruction in the medium and small caliber ways without reversibility with salbutamol. Biopsies of ventricular band, vocal cord and arytenoid stained with positive Congo red for amyloid tissue, established the laryngeal amyloidosis diagnosis. The complementary studies to rule out amyloid tissue in the remaining tracheobronchial tree were negative. Dyspnea had characteristics of laryngeal origin, caused by a pulmonary ventilation disorder provoked by the difficult arrival of air to alveoli, which caused the decreased partial pressure of oxygen and CO2.
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PMID:[Asthma or laryngeal amyloidosis? A report of a case and literature review]. 1663 59

Primary amyloidosis involving the thyroid gland is rare and limited to case reports. We report the case of a previously healthy 47-year-old female presenting with a 1-month history of nausea, vomiting, and diffuse thyroid enlargement. Over the next 3 months, she simultaneously developed renal insufficiency dysphagia and hoarseness of voice. Biopsies from the gastric antrum, duodenum, bone marrow, and kidney were positive for vascular deposition of amyloid. Ultrasound of the thyroid revealed diffuse enlargement of the thyroid gland, which was 32.8 ML in volume, with diffuse hyperechogenicity. Fine needle aspiration (FNA) biopsy was positive for amyloid by Congo red staining, and cytology was negative for malignancy. The patient was treated with dexamethasone 40 mg daily on days 1-4, 9-12, and 17-20 for 3 months. On 3-month follow-up, the patient's nausea and vomiting had resolved and renal insufficiency improved. Ultrasound of the thyroid demonstrated decrease in the size of the goiter to 23.2 ML. Amyloid goiter is seen only in approximately 0.04% of patients with primary systemic amyloidosis. No data is currently available regarding treatment of primary amyloidosis and its effect on the goiter. However, we have evidence demonstrating that successful treatment of amyloidosis decreases thyroid enlargement and improves organ dysfunction.
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PMID:Amyloid goiter as a manifestation of primary systemic amyloidosis. 1727 55

A 31-year-old woman with a history of bilateral carpal tunnel surgery complained of worsening hand pains and swelling. Subsequently, she presented for rheumatologic evaluation with generalized arthralgias, symmetric polyarthritis of the hands and feet, shiny skin with tightness and thickening, tender periungual erythema, malar rash, and photosensitivity. The only laboratory abnormality found then was a positive antinuclear antibody. Her joint symptoms were responsive to low-dose prednisone and hydroxychloroquine. However, the skin tightness progressed proximally and centrally and developed around the mouth. At that point, more specific autoimmune work-up showed negative relevant antibodies, and repeat antinuclear antibody tests turned out negative. Later, she reported dysphagia and hoarseness, and ecchymotic rashes appeared on the face and forearm. Biopsy of the forearm lesion showed leukocytoclastic vasculitis. Staining for amyloid was negative. Subsequently, she was found to have hypogammaglobulinemia and Bence-Jones proteinuria; the progression of her skin symptoms provoked a repeat skin biopsy with special stains that demonstrated amyloidosis. Bone marrow biopsy showed >75% plasma cells, skeletal survey revealed multiple lytic lesions, and a diagnosis of multiple myeloma with associated amyloidosis was made. Despite the initial features of connective tissue disease in this young woman, a steadfast workup revealed the source of her problem.
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PMID:Scleroderma-like illness as a presenting feature of multiple myeloma and amyloidosis. 1852 36

There are some reports of localized amyloidosis in the larynx, an entity that corresponds to one percent of all benign tumors of this region. However, there are only two cases of amyloidosis in the Waldeyer's ring 6, 13, 14. We hereby describe a rare case of amyloidosis in areas not associated with the upper aero-digestive tract: tonsil pillar, rhinopharynx, supraglottis and glottis, without visible continuity of amyloid tissue. We will also discuss post-operative follow up with severe dysphagia.
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PMID:Multiple-sited amyloidosis in the upper aerodigestive tract: case report and literature review. 1866 Oct 24


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