• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.9 no.2
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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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• v.32 no.11
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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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• v.48 no.6
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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.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.21 no.1
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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.

• Annals of Clinical Neurophysiology
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• v.24 no.2
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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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• v.17 no.1
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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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• v.22 no.2
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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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• v.78 no.4
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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.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.243-250
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• 2002

Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain at)d characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.

• Investigative Magnetic Resonance Imaging
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• v.24 no.2
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• pp.85-89
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• 2020

Amyloidosis is an all-inclusive disease of deposition of amyloid proteins in the extracellular spaces, which in localized or systemic form cause tissue damage and dysfunction. Herein, we report a case of small bowel involvement of systemic amyloidosis presenting with multiple polypoid wall thickening mimicking small bowel polyposis syndrome in an age 75 male. Interestingly, polypoid wall thickening and amyloidoma showed hypointensity on T2-weighted images. To our knowledge, there has been no literature describing MRI findings of poylpoid wall thickening and amyloidoma. Although the underlying mechanisms are unclear and need validation, hypointensity on T2-weighted images could be valuable in diagnosing small bowel involvement of amyloidosis in patients presenting with poylpoid wall thickening and amyloidoma.