• 대한세포병리학회지
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• 제13권1호
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• pp.38-41
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• 2002

Localized amyloidosis of the ureter is rare and clinically confused with neoplasm. We describe a case of localized amyloidosis of the ureter in which the presence of amyloid was detected in ureteral washing cytology. A 75-year-old female presented with gross hematuria. Abdominal CT and retrograde pyelography revealed hydronephrosis and hydroureter on the left side with abrupt narrowing of the distal ureter. Ureteral washing cytology yielded a hypocellular smear with many irregular clumps of amorphous, extracellar, waxy material. Biopsy sections demonstrated submucosal deposits of eosinophilic amorphous material which gave characteristic apple green birefringence with Congo-red stain under the polarized light. Familarity with the cytologic features of amyloid is helpful for preoperative diagnosis and proper treatment.

• Tuberculosis and Respiratory Diseases
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• 제42권2호
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• pp.250-255
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• 1995

미만성 기관기관지형 유전분증은 폐를 침범하는 유전분증 중에서 기침, 호흡곤란, 혈담 및 반복되는 폐렴 등의 증상을 일으키고 기관지경 검사에서 기관 및 여러 기관지에 종괴를 형성하는 질환이다. 저자들은 58세 여자 환자에서 발생한 미만성 기관기관지형 유전분증을 기관지경하 생검으로 진단하고 전기조작술을 시행하여 기관지협착을 성공적으로 치료한 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제65권6호
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• pp.532-536
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• 2008

저자들은 가래를 동반한 기침과 쉰 목소리로 내원한 68세 남자에게 흉부 전산화 단층촬영으로 우중엽 무기폐를 확인하고 기관지경 및 생검을 시행하여 후두와 기관기관지에 국한된 유전분증 1예를 경험하였기에 문헌고찰과 함께 보고한다.

• Tuberculosis and Respiratory Diseases
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• 제52권2호
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• pp.174-178
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• 2002

기침 및 호흡곤란 주소로 내원한 43세 남자 환자에서 흉부 전산화 단층촬영 및 기관지경하 생검으로 다른 장기의 침범 없이 폐에만 국한된 미만성 기관기관지형 유전분증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• 대한영상의학회지
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• 제82권2호
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• pp.429-434
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• 2021

아밀로이드증은 세포 외 공간에 비정상적인 단백질이 축적되는 것을 특징으로 하는 전신 질환이며, 심근을 포함한 다양한 기관을 침범한다. 저자들은 심장 아밀로이드증 환자에서 항암 화학 요법 및 줄기세포 이식 후 심장 자기공명영상의 추적검사에서 조영 전 T1 및 T2 수치와 세포 외 부피 분율의 감소를 보인 사례를 보고한다. 조영 전 T1 이완시간 및 세포 외 부피 분율은 아밀로이드증 환자에서 조직의 아밀로이드 축적 정도와 밀접한 관련이 있다. 이 사례를 통해 T1 지도화 심장 자기공명영상 기법이 심장 아밀로이드증의 치료 반응 모니터링에 비침습적이며 정량적인 도구로서 큰 역할을 할 수 있는 가능성을 확인할 수 있다.

• 한국자기공명학회논문지
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• 제24권3호
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• pp.91-95
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• 2020

Transthyretin (TTR) is an abundant protein in blood plasma and cerebrospinal fluid (CSF), working as a homo-tetrameric complex to transport thyroxine (T₄) and a holo-retinol binding protein. TTR is well-known for its amyloidogenic property; several types of systemic amyloidosis diseases are caused by aggregation of either wild-type TTR or its variants, for which more than 100 mutations were reported to increase the amyloidogenicity of TTR. The rate-limiting step of TTR aggregation is the dissociation of a monomeric subunit from a tetrameric complex. A wide range of biochemical and biophysical techniques have been employed to elucidate the TTR aggregation processes, among which nuclear magnetic resonance (NMR) spectroscopy contributed much to characterize the structural and functional features of TTR during its aggregation processes. The present review focuses on discussing the recent advances of our understanding to the amyloidosis mechanism of TTR and to the structural features of its monomeric aggregation-prone state in solution. We expect that the present review provides novel insights to appreciate the molecular basis of TTR amyloidosis and to develop novel therapeutic strategies to treat diverse TTR-related diseases.

• Tuberculosis and Respiratory Diseases
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• 제76권6호
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• pp.292-294
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• 2014

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.

• Tuberculosis and Respiratory Diseases
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• 제44권6호
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• pp.1426-1432
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• 1997

Sj$\ddot{o}$gren's syndrome(SS) is a chronic inflammatory disorder characterized by lymphocytic infiltration of lacrimal and sailvary glands, which results in dry eyes and dry mouth. SS may exist as a primary condition or as a secondary condition in association with connective tissue disease such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. We experienced a patient with primary SS who developed multiple bullae, nodular type of pulmonary amyloidosis and lymphocytic interstitial peumonitis. We believe this to be the first reported case of SS acompanied by these three types pulmonary manifestations at the same time.

• 대한상부위장관⦁헬리코박터학회지
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• 제18권3호
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• pp.209-212
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• 2018

We report a rare case of systemic amyloidosis with gastrointestinal and lymph node involvement. A 64-year-old woman was admitted to our hospital with dyspepsia and weight loss. Initial esophagogastroduodenoscopy (EGD) revealed nonspecific findings, and abdominal computed tomography showed necrotizing lymphadenopathy at the porta hepatis. Laparoscopic lymph node biopsy was performed under suspicion of tuberculous lymphadenopathy, but a definite diagnosis was not established. Follow-up EGD performed 6 months later revealed multiple telangiectasia-like lesions at the gastric body, and endoscopic biopsy revealed amyloid deposition. Through additional blood and urine protein electrophoresis, the patient was finally diagnosed with systemic amyloidosis associated with multiple myeloma. She was treated with dexamethasone, thalidomide, and bortezomib; however, she died 3 months after diagnosis because of pneumonia and multiple organ failure.

• 한국임상수의학회지
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• 제39권2호
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• pp.81-86
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• 2022

A female, 14-year-old, African penguin (Spheniscus demersus) weighing 2.5 kg with the anorexia for one day and 22 months history of bumblefoot was found dead in aquarium. Grossly, severe bumblefoot in the central surface of the right foot, severe enlarged liver and kidneys with orange color were observed. Histopathologically, pinkish amorphous materials were accumulated in liver, spleen, kidney, and stomach. The pinkish materials in the liver, kidney, spleen, and stomach were confirmed as amyloid with typical orange color reactions using Congo red stain under the light microscope and with bright red color expressions using Congo red stain under the fluorescence microscope. Based on the typical gross and histopathologic findings and special staining, this case was confirmed as systemic amyloidosis. A long period time of moderate bumblefoot might be closely associated with the occurrence of systemic amyloidosis in the captive penguin.