• 대한후두음성언어의학회지
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• 제9권2호
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• pp.147-151
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• 1998

Primary localized laryngeal amyloidosis is an uncommon disorder of unknown cause that occurs in the absence of systemic amyloidosis or associated disease. There is a risk of either missing concomitant systemic amyloidosis or exhaustively investigating for this when it is not present through failure to appreciate the nature of the disease. We present 2 cases of primary localized laryngeal amyloidosis in supraglottic region. Biopsy of the mass of patients revealed findings consistent with amyloidosis, which were Congo red reaction with a apple green birefringence in polarized light fluorescence microscopy. An extensive workup for systemic amyloidosis was negative. All of two cases were treated by vaporization via $CO_2$ LASER using "Swiftlase Flshscan" for creating a wide, shallow char-free treatment site by precisely controlling ablation depth without causing residual thermal damage to tissue. The postoperative recovery of all cases was uneventful with good vocal quality and no aspiration. At the present time, the patients have no evidence of disease, recurrence and complication.

• Journal of Chest Surgery
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• 제32권11호
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• pp.1060-1063
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• 1999

Amyloidosis is a rare disease which is characterized by the deposition of a histochemically specific substance called amyloid in many tissue bodies, and causes various symptoms according to the organs involved. Amyloid is usually recognized by its staining reaction with Congo red stain. Primary pulmonary amyloidosis is very rare. Nodular pulmonary amyloidosis is an uncommon entity that usually manifests itself as an asymptomatic incidental finding on the chest roentgenogram and is misdiagnosed as lung cancer or pulmonary tuberculosis.

• Journal of Chest Surgery
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• 제48권6호
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• pp.422-425
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• 2015

Amyloid deposits in the heart are not exceptional in systemic amyloidosis. The clinical manifestations of cardiac amyloidosis may include restrictive cardiomyopathy, characterized by progressive diastolic and eventually systolic biventricular dysfunction; arrhythmia; and conduction defects. To the best of our knowledge, no previous cases of isolated tricuspid regurgitation as the initial manifestation of cardiac amyloidosis have been reported. We describe a rare case of cardiac amyloidosis that initially presented with severe tricuspid regurgitation in a 42-year-old woman who was successfully treated with tricuspid valve replacement. Unusual surgical findings prompted additional evaluation that established a diagnosis of plasma cell myeloma.

• 대한후두음성언어의학회지
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• 제21권1호
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• pp.54-56
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• 2010

Amyloidosis is a diverse group of disorders that share the deposition of amyloid protein in various extracellular tissues. Systemic amyloidosis may involve any body part and about 9-15% of it is localized. In the head and neck, the larynx is known as most common involved site. A histological diagnosis of amyloidosis is necessary to establish a definite diagnosis. Conservative surgery can be done for relieved airway obstruction or restored voice. We present three cases of patients with laryngeal amyloidosis that was treated with laryngeal microscopic surgery with laser.

• International Journal of Heart Failure
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• 제6권1호
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• pp.36-43
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• 2024

Background and Objectives: Atrial fibrillation is common in patients with cardiac amyloidosis. However, the optimal anticoagulation strategy to prevent thromboembolic events in patients with cardiac amyloidosis and atrial fibrillation is unknown. This systematic review and meta-analysis compares direct oral anticoagulants (DOACs) vs. vitamin K antagonists (VKAs) in patients with cardiac amyloidosis and atrial fibrillation. Methods: We performed a systematic literature review to identify clinical studies of anticoagulation therapies for patients with cardiac amyloidosis and atrial fibrillation. The primary outcomes of major bleeding and thrombotic events were reported using random effects risk ratios (RRs) with 95% confidence interval (CI). Results: Our search yielded 97 potential studies and evaluated 14 full-text articles based on title and abstract. We excluded 10 studies that were review articles or did not compare anticoagulation. We included 4 studies reporting on 1,579 patients. The pooled estimates are likely underpowered due to small sample sizes. There was no difference in bleeding events for patients with cardiac amyloidosis and atrial fibrillation treated with DOACs compared to VKAs with a RR of 0.64 (95% CI, 0.38-1.10; p=0.10). There were decreased thrombotic events for patients with cardiac amyloidosis and atrial fibrillation treated with DOACs compared to VKAs with a RR of 0.50 (95% CI, 0.32-0.79; p=0.003). Conclusions: This systematic review and meta-analysis suggests that DOACs are as safe and effective as VKAs in patients with cardiac amyloidosis and atrial fibrillation. However, more data are needed to investigate clinical differences in anticoagulation therapy in this patient population.

• 대한영상의학회지
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• 제82권5호
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• pp.1334-1340
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• 2021

아밀로이드증과 비호지킨림프종과의 연관관계는 이전의 연구들에서 보고된 바 있다. 림프종과 아밀로이드증과의 연관관계는 두 개의 구분되는 범주로 나뉘는데 림프종 연관 전신성 아밀로이드증과 종괴 주위 아밀로이드증이다. 저자들은 두경부 MALT 림프종 환자에서 발생한 국소적 종괴 주위 아밀로이드증의 드문 증례를 보고하고자 한다. 전산화단층촬영에서 병변은 연조직음영을 보이는 무정형석회화를 동반한 불규칙한 모양의 종괴였으며, 조영증강 시 불균일한 조영증강을 보였다. 자기공명영상에서는 종괴가 T1 강조영상과 T2 강조영상에서 모두 불균일한 낮은 신호 강도를 보였다. 종괴는 강한 조영증강을 보였으며, 내부에는 여러 개의 조영증강되지 않는 부분들을 포함하고 있었다. 림프종 환자에서 치료 전 영상검사에서 발견된 석회화들은 종괴 주위 아밀로이드증과 같은 동반된 다른 이차성 병변을 시사하는 소견이 될 수 있을 것이다.

• Annals of Clinical Neurophysiology
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• 제24권2호
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• pp.90-92
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• 2022

AGel amyloidosis is an autosomal dominantly inherited disease caused by a GSN mutation, and affected patients typically present with the clinical triad of corneal lattice dystrophy, progressive cranial neuropathy, and cutis laxa. We report a Korean family with AGel amyloidosis with predominant manifestations of facial and bulbar muscle weakness. Whole-exome sequencing revealed a common missense mutation (p.Asp214Tyr) in GSN. This case strongly suggests that AGel amyloidosis should be considered when a patient presents with progressive facial and bulbar palsies.

• Annals of Clinical Neurophysiology
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• 제17권1호
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• pp.24-27
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• 2015

Amyloidosis is a systemic disorder associated with clonal plasma cell dyscrasia. Nephrotic syndrome, congestive heart failure, autonomic and peripheral neuropathy is often associated features in amyloidosis. Early diagnosis is most important because of different prognosis by stage. The diagnosis can be delayed since symptoms of amyloidosis may vary or nonspecific. We describe a patient of myeloma-associated amyloidosis, who showed orthostatic intolerance as the first symptom of the disease.

• Childhood Kidney Diseases
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• 제22권2호
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• Tuberculosis and Respiratory Diseases
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• 제78권4호
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• pp.385-389
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• 2015

Amyloidosis is defined as the presence of extra-cellular deposits of an insoluble fibrillar protein, amyloid. The pulmonary involvement of amyloidosis is usually classified as tracheobronchial, parenchymal nodular, or diffuse alveolar septal. A single nodular lesion can mimic various conditions, including malignancy, pulmonary tuberculosis, and fungal infection. To date, only one case of nodular pulmonary amyloidosis has been reported in Korea, a case involving multiple nodular lesions. Here, we report and discuss the case of a patient having single nodular amyloidosis.