• Title/Summary/Keyword: Progressive Pulmonary Fibrosis

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Tc-99m-MIBI Uptake in Active Pulmonary Tuberculosis : A Case Report (활동성 폐 결핵에서의 Tc-99m-MIBI 섭취 : 1예 보고)

  • Lee, Chang-Ho;Park, Chan-Hee;Hwang, Hee-Sung;Bae, Moon-Sun
    • The Korean Journal of Nuclear Medicine
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    • v.30 no.3
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    • pp.379-381
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    • 1996
  • Technetium-99m MIBI was developed as a myocardiac perfusion imagine agent and has been used effectively in the detection and post-therapeutic evaluation of various neoplasm such as thyroid, lung, bone and breast tumors. As an infrequent findings, Tc-99m MIBI agent has shown in non-neoplastic pulmonary conditions Including fibrosing alveolitis, pulmonary actinomycosis, active pulmonary sarcoidosis, pulmonary interstitial fibrosis in progressive systemic sclerosis and active osteomyelitis. In a recent report conducted by Cetin Oncel, Tc-99m MIBI imaging is an effective method in the detection and follow-up of pulmonary tuberculosis We have also experienced Tc-99m MIBI uptake in active pulmonary tuberculosis incidentally found in a patient with suspected proliferative villonodular synovitis of the left ankle.

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An Experience of Judicial Autopsy for a Death by Muscular Dystrophy: An Autopsy Case (근이영양증으로 인한 사망의 사법부검 사례 경험: 증례 보고)

  • Kim, Youn Shin;Park, Ji Hye
    • The Korean Journal of Legal Medicine
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    • v.42 no.4
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    • pp.159-163
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    • 2018
  • Progressive muscular dystrophy (PMD) is a primary muscle disease characterized by progressive muscle weakness and wasting, which is inherited by an X-linked recessive pattern and occurs mainly in males. There are several types of muscular dystrophies classified according to the distribution of predominant muscle weakness including Duchenne and Becker, Emery-Dreifuss, facioscapulohumeral, oculopharyngeal, and limb-girdle type. Clinical manifestations of PMD are clumsy, unsteady gait, pneumonia, heart failure, pulmonary edema, hydropericardium, hydrothorax, aspiration, syncopal attacks, and sudden cardiac death. The deceased was a 34-year-old man, and the onset of the first clinical symptom, gait disturbance, was in his late teens. His elder brother had the same disease and experienced brain death after a head trauma and died after mechanical ventilation was discontinued. After an autopsy, we found contracture of the joints, pseudohypertrophy of the calf, wasting and fat replacement of the thigh muscle, pericardial effusion (80 mL), fibrosis and fat replacement of the cardiac ventricular wall, pulmonary edema, and froth in the bronchus. The cause of death was heart failure and dyspnea due to muscular dystrophy. There was no sign or suspicion of foul play in his death.

A Case of the patient who was admitted to hospital the 8th day of Paraquat Poisoning (파라콰트 중독 8일 후 내원한 환자 1례)

  • Heo Geum-Jeong;Kim Dong-Jo;Park Byung-Wook;Koo Chang-Mo;Nam Chang-Gyu
    • The Journal of Internal Korean Medicine
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    • v.24 no.4_2
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    • pp.1087-1092
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    • 2003
  • Paraquat is a non-selective contact herbicide. When it is consumed, it may cause fatal disorders such as acute renal failure, hepatic dysfunction, and progressive respiratory failure. In spite of many efforts to cure patients poisoned with paraquat, the mortality rate still remain high. In this case, after using Gamdutanghaphwangryunhaedoktang-gamibang and Cheongsangboha-tang we got positive result in hepato-renal function, but progressive respiratory failure was unstoppable.

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A Case of Antisynthetase Syndrome (항 Synthetase 증후군 1예)

  • Kim, Min-Jeong;Kim, Min Ah;Kim, Eung-Gyu;Kim, Chan-Hwan;Kim, Sang-Jin
    • Annals of Clinical Neurophysiology
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    • v.8 no.2
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    • pp.196-198
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    • 2006
  • It has been reported that antisynthetase syndrome belongs to the idiopathic myositis group which includes pulmonary interstitial disease, arthritis, Raynaud's phenomenon, and mechanic's hand, associated with the anti-Jo1 antibody. A 60- year-old man presented with one month history of lower limbs weakness, rapidly progressive exertional dyspnea, and arthralgia. A markedly increased titers of anti-Jo1 antibodies were found. Chest CT showed idiopathic pulmonary fibrosis. Muscle biopsies were consistent with polymyositis. A high dose corticosteroids and cyclosporine were not effective. We report a case of antisynthetase syndrome, in which immunosuppressive agents could not rescue the deteriorating disease course.

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Middle East Respiratory Syndrome-Coronavirus Infection into Established hDPP4-Transgenic Mice Accelerates Lung Damage Via Activation of the Pro-Inflammatory Response and Pulmonary Fibrosis

  • Kim, Ju;Yang, Ye Lin;Jeong, Yongsu;Jang, Yong-Suk
    • Journal of Microbiology and Biotechnology
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    • v.30 no.3
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    • pp.427-438
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    • 2020
  • Middle East respiratory syndrome coronavirus (MERS-CoV) infects the lower respiratory airway of humans, leading to severe acute respiratory failure. Unlike human dipeptidyl peptidase 4 (hDPP4), a receptor for MERS-CoV, mouse DPP4 (mDPP4) failed to support MERS-CoV infection. Consequently, diverse transgenic mouse models expressing hDPP4 have been developed using diverse methods, although some models show no mortality and/or only transient and mild-to-moderate clinical signs following MERS-CoV infection. Additionally, overexpressed hDPP4 is associated with neurological complications and breeding difficulties in some transgenic mice, resulting in impeding further studies. Here, we generated stable hDPP4-transgenic mice that were sufficiently susceptible to MERS-CoV infection. The transgenic mice showed weight loss, decreased pulmonary function, and increased mortality with minimal perturbation of overexpressed hDPP4 after MERS-CoV infection. In addition, we observed histopathological signs indicative of progressive pulmonary fibrosis, including thickened alveolar septa, infiltration of inflammatory monocytes, and macrophage polarization as well as elevated expression of profibrotic molecules and acute inflammatory response in the lung of MERS-CoV-infected hDPP4-transgenic mice. Collectively, we suggest that this hDPP4-transgenic mouse is useful in understanding the pathogenesis of MERS-CoV infection and for antiviral research and vaccine development against the virus.

Rigid Bronchoscopy for Post-tuberculosis Tracheobronchial Stenosis

  • Hojoong Kim
    • Tuberculosis and Respiratory Diseases
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    • v.86 no.4
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    • pp.245-250
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    • 2023
  • The healing process of tracheobronchial tuberculosis (TB) results in tracheobronchial fibrosis causing airway stenosis in 11% to 42% of patients. In Korea, where pulmonary TB is still prevalent, post-TB tracheobronchial stenosis (PTTS) is one of the main causes of benign airway stenosis causing progressive dyspnea, hypoxemia, and often life-threatening respiratory insufficiency. The development of rigid bronchoscopy replaced surgical management 30 years ago, and nowadays PTTS is mainly managed by bronchoscopic intervention in Korea. Similar to pulmonary TB, tracheobronchial TB is treated with combination of anti-TB medications. The indication of rigid bronchoscopy is more than American Thoracic Society (ATS) grade 3 dyspnea in PTTS patients. First, the narrowed airway is dilated by multiple techniques including ballooning, laser resection, and bougienation under general anesthesia. Then, most of the patients need silicone stenting to maintain the patency of dilated airway; 1.5 to 2 years after indwelling, the stent could be removed, this has shown a 70% success rate. Acute complications without mortality develop in less than 10% of patients. Subgroup analysis showed successful removal of the stent was significantly associated with male sex, young age, good baseline lung function and absence of complete one lobe collapse. In conclusion, rigid bronchoscopy could be applied to PTTS patients with acceptable efficacy and tolerable safety.

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment (스테로이드 치료로 호전을 보인 폐동맥 고혈압을 유발한 특발성 섬유화성 종격동염 1예)

  • Jung, Bo Yong;Eo, Sung Jun;Park, Eun Seo;Kim, Young Tong;Cho, Jae Sung;Oh, Mi Hye;Seo, Gi Hyun;Na, Joo Ock;Lee, Sang Do;Uh, Soo Taek;Kim, Yong Hoon;Park, Choon Sik
    • Tuberculosis and Respiratory Diseases
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    • v.61 no.1
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    • pp.74-79
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    • 2006
  • Idiopathic fibrosing mediastinitis is, an uncommon cause of pulmonary hypertension this is characterized by excessive fibrosis of the mediastinum with an unknown etiology. Steroid therapy has been suggested for individuals with progressive symptoms, bu there is littlet data demonstrating the efficacy of such therapy are lacking. We present a case of pulmonary hypertension secondary to a compression of a main pulmonary artery by fibrosing mediastinitis which was confirmed by a biopsy with a thoracotomy. The chest CT scan and 2D echocardiography performed before and after a trial of steroid therapy demonstrated improvement after steroid therapy.

Surgical Management of Aortic Insufficiency in Behcet`s Syndrome - An Experience of 8 Cases - (Behcet 씨 증후군에 의한 대동맥판 폐쇄부전의 수술치험 -3례 보고-)

  • 원용순
    • Journal of Chest Surgery
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    • v.21 no.5
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    • pp.899-904
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    • 1988
  • In Behcet syndrome, cardiac involvements are rare and have been reported pericarditis, myocarditis, right heart endocardial fibrosis, right ventricle mural thrombus with pulmonary embolism, active endocarditis, granulomatous endocarditis, conduction disturbance, acute aortic insufficiency, mitral valve prolapse. Our three patients underwent AVR because of aortic insufficiency and ascending aorta enlargement combined with Behcet syndrome. Two patients had mitral regurgitation too. So one underwent MAP and the other underwent MVR concomitantly. One who underwent AVR have been well for 50 months. Another who underwent AVR+MAP and redo AVR due to aortic paravalvular leakage was died of congestive heart failure. The other who underwent AVR+MVR and repeated AVR three times because of aortic paravalvular leakage is in condition of aortic paravalvular leakage. Paravalvular leakage is considered to recur due to progressive dilatation and fragility of aortic root that is the result of pathologic change of Behcet syndrome in it. If Open heart surgery is needed in Behcet`s syndrome during inflammatory reaction is active, postoperative complications such as paravalvular leakage or suture line rupture may be prevented with pre- and postoperative anti-inflammatory management.

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Combined Bilateral Lung Transplantation and Off-Pump Coronary Artery Bypass

  • Do, Young Woo;Jung, Hee Suk;Lee, Chang Young;Lee, Jin Gu;Youn, Young-Nam;Paik, Hyo Chae
    • Journal of Chest Surgery
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    • v.49 no.6
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    • pp.461-464
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    • 2016
  • Coronary artery disease has historically been a contraindication to lung transplantation. We report a successful combined bilateral lung transplantation and off-pump coronary artery bypass in a 62-year-old man. The patient had a progressive decline in lung function due to idiopathic pulmonary fibrosis and a history of severe occlusive coronary artery disease.

Longterm Benefit and Unmet Needs in Enzyme Replacement Therapy of Gaucher (고셔병 환자에서 효소대체치료의 장기적 효과와 한계)

  • Kim, Yoo-Mi
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.14 no.1
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    • pp.42-47
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    • 2014
  • Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.