A 4-week-old male piglet being maintained in a research facility was found dead without any previous clinical signs. The piglet had been born from a surrogate mother after somatic nuclear transfer as part of a xenotransplantation study. Ovaries for nuclear transfer were obtained from a private farm outside the research facility. Histopathologically, multifocal to coalescing granulomatous myocarditis was observed in the heart, characterized by infiltration of lymphocytes, macrophages and multinucleated giant cells, and by myocardial necrosis and fibrosis. Lymphoid tissues showed marked lymphoid depletion with infiltration by histiocytes or giant cells. Immunohistochemistry showed PCV-2 antigens in necrotic myocytes, macrophages and multinucleated giant cells in the heart, as well as in macrophages and giant cells in lymphoid depleted areas of lymphoid tissues. Reproductive failure associated with PCV-2 in aborted or stillborn piglets is frequently characterized by myocarditis, and similar lesions were observed in this 4-week-old piglet with PCV-2 infection. The PCV-2 infection in this piglet may have been due to contamination or infection of an ovary from the pig farm.
Cystic lesions or progressive cystic changes in adenocarcinoma of the lung have rarely been reported. We report a case of lung adenocarcinoma that progressed from ground-glass opacities (GGOs) and consolidations or nodules to extensive cystic lesions during 12 months in a young adult patient. A 29-year-old male was initially diagnosed with primary lung adenocarcinoma by transbronchial lung biopsy of the right lower lobe and lung to lung metastasis in both lungs according to imaging findings. The initial chest computed tomography (CT) scans showed multifocal GGOs, consolidations, and nodules in both lungs. Despite treatment with palliative chemotherapy, the patient's follow-up CT scans showed multiple, cystic changes in both lungs and that the lesions had progressed more extensively. He died of hypoxic respiratory failure one year after his diagnosis.
The Journal of the Korean bone and joint tumor society
/
v.5
no.3
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pp.169-177
/
1999
Osteosarcoma in patients older than 40 years are rare, however they have different clinical, radiological and pathological features from those of younger patients. Sometimes accurate histologic diagnosis is not easy, which is important in determining the correct surgical treatment and appropriate chemotherapy. Since January 1995, 11 patients with osteosarcoma occurring in patients older than 40 years have been diagnosed, treated and followed up for more than 6 months. In contrast to osteosarcoma in children and adolescents, only 4 cases(36.4%) were conventional types, while the others included 2 malignant fibrous histiocytoma-like types, 2 small cell types, 2 periosteal osteosarcomas and 1 giant cell-rich type. Seven cases showed purely osteolytic or predominantly osteolytic bony lesions and 8 were in Enneking stage IIB. Performed surgical treatments included 2 amputations, 6 wide resections and reconstructions, and one curettage and autogenous bone graft. In the remaining 2 cases, definitive surgical treatments included not carried out because of old age, multifocal involvement or poor medical tolerance. Neoadjuvant and adjuvant chemotherapies were performed in 9 of 11 patients. At last follow-up, there were 6 continuously disease-free survivals, 3 alive with diseases and 2 died of diseases. The overall cumulative 4-year survival rate calculated using Kaplan-Meier's productlimit method was 59.3%. For improved oncologic outcomes and survivals, early and accurate diagnosis, surgical treatment with adequate margin and neoadjuvant and adjuvant chemotherapy will be necessary.
Previous studies have shown that the heterocycles including thiazoles are efficacious in inducing phase phase II metabolizing enzyme as well as certain cytochrome P450s and that the inductin of these matabolizing enzymes by the heterocyclic agents is highly associated with their hepatotoxicity. In the present study, the effects of benzylisothiazole (BIT), which has a isothiazole moiety, on the expression of microsomal epoxide hydrolase (mEH), major glutathione S-transerases and cytochrome P450s were studied in the rat liver in association with its hepatotoxicity. Treatment of rats with BIT(1.17 mmol/kg, 1~3d) resulted in substantial increases in the mEH. rGSTA2, rGSTA2, rGSTM1 and rGSTM2 mRNA levels, whereas rGSTA3 and rGSTA5 mRNA levels were increased to much lesser extents. A time-course study showed that the mRNA levels of mEH and rGSTs were greater at 24hr after treatment than those after 3 days of consecutive treatment. Relative changes in mEH and rGST mRNA levels were consistent with those in the proteins, as assessed by Western immunoblot analysis. Hepatic cytochrom P450 levels were monitored after BIT treatment under the assumption that metabolic activation of BIT may affect expression of the enzymes in conjunction with hepatotoxicity. Immunoblot analysis revealed that cytochrome P450 2B1/2 were 3-to 4-fold induced in rats teatd with BIT(1.17 mmol/kg/day.3days), whereas P450 1A2, 2C11 and 3A1/2 levels were decreased to 20~30% of those in unteatd rats. P450 2E1 was only slightly decreased by BIT. Thus, the levels of several cytochrome P450s were suppressed by BIT treatment. Rats treated with BIT at the dose of 1.17mmol/kg for 3 days exhibited extensive multifocal nodular necrosis with moderate to extensive diffuse liver cell degeneration. No notable toxicity was observed in the kidney. These results showed that BIT induces mEH and rGSTs in the liver with increases in the mRNA levels, whereas the agent significantly decreased major cytochrome P450s. The changes in the detoxifying enzymes might be associated with the necrotic liver after consecutive treatment.
An assessment of the subtalar joint in cerebral palsies can contribute to predict the function of ambulation in CP children. Ambulation is one of the most important function to guarantee the CP children independent life. This paper is to investigate some relationships between the function of standing and walling and the assessment of the subtalar joint in children with Cerebral palsy. And also to present the correlation between the ambulation and the Gross Motor Function Measures in children with cerebral palsy. Sixty-eight children with cerebral palsy were participated in this study. Evaluations of the subtalar pint parameters were performed by the goniometer and the angle finder, and the GMFM scores were measured by their teacher and researcher trained technically. A regression analysis was applied to figure out the relationship between the subtalar pint parameters(ROM and RCSP) and the function of standing and walking. A correlation analysis was employed to see how much the subtalar pint parameters could be predicted from GMFM scores in walling and standing. The results were as follows: 1) The significant differences were not observed between the total ROM, RCSP and the function of standing(F=8.065, p<.001) and walking(F=6.511, p<.001) in CP children. 2) The subtalar pint parameters(total ROM, RCSP) have the lower relevance to the function of standing and walling in CP children.(p>.05) 3) The total ROM and RCSP in both feet have the significant differences between the CP children and the normal children.(p<.001) 4) The GMFM scores were significantly correlated with the function of walling and standing in CP children.(r=247, p<.05) In this research, it is found that the significant relevance between the quantitative analysis of subtalar pint in children with cerebral palsy and the gross motor function of ambulation in standing and walling. However, it is difficult to predict the direct relationship of subtalar pint parameters and the function of ambulation, because subtalar pint scores and GMFM are only measured as quantities not qualities. Therefore, it is more reasonable to investigate the influence of subtalar pint parameters on ambulation in children with cerebral palsies, adding to the multifocal assessment of the children, rather than vice versa.
Hwang, Kyu Sig;Roh, Young Wook;Song, Sung Heon;Kim, Sang Heon;Sohn, Jang Won;Yoon, Ho Joo;Shin, Dong Ho;Park, Sung Soo;Oh, Young-Ha;Kim, Tae-Hyung
Tuberculosis and Respiratory Diseases
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v.67
no.1
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pp.37-41
/
2009
Lymphocytic interstitial pneumonia (LIP) is a rare disorder characterized by a diffuse infiltration of the alveolar space, interstitium by lymphocytes, plasma cells, and reticuloendothelial cells. Although its etiology is unknown, LIP has been associated with autoimmune disorders and with viral infections. Because it's clinical and radiographic features are nonspecific, a confirmatory diagnosis is performed by open lung biopsy. A 59-year-old female presented dry cough, which had been present for 1 month. On initial findings of multifocal consolidation at the right middle lobe on both lower lobes in chest radiography, the first diagnosis of cryptogenic organizing pneumonia was suggested. On open lung biopsy, LIP was diagnosed. The patient had no autoimmune disease, viral infection or monoclonal gammopathy. After 3 months of corticosteroid treatment, the patient experienced improved symptoms, reduced abnormalities on chest radiography, and improved pulmonary function testing.
Lee, Sun Ho;Jeon, Mina;Lee, Hyun Joo;Park, Dae Young;Kim, Se Hoon;Lee, Young-Mock
Journal of The Korean Society of Inherited Metabolic disease
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v.15
no.2
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pp.72-77
/
2015
Purpose: Deficits of the respiratory chain are reported to be the major cause of Leigh syndrome is said to be the underlying causes. The need for biochemical diagnosis to draw more accurate diagnosis or prognosis to support treatments is rapidly increasing. This study tried to analyze the aspects of clinical characteristics and biochemical diagnosis of mitochondrial respiratory chain complex (MRC) defect in Leigh syndrome, using methods of biochemical enzyme assay. Methods: We included total number of 47 patients who satisfied the clinical criteria of Leigh syndrome and confirmed by biochemical diagnosis. All those patients went through muscle biopsy to perform biochemical enzyme assay to analyze MRC enzyme in order to find the underlying cause of Leigh syndrome. Results: MRC I defect was seen in 23 (48.9%) cases taking the first place and MRC IV defect in 15 (31.9%) following it. There were 9 (19.2%) cases of combined MRC defect. Combined cases of type I and IV were detected in 7 (14.9%) patients while type I and V in 2 (4.3%). The onset age of symptom was less than 1 year old in 28 (59.6%). The most common early symptom, observed in 23 (48.9%), was delayed development, but there were other various neurological symptoms observed as well. In regard with the disease progression, 35 (74.5%) patients showed slowly progressive course, the one that progressed continuously but slowly over 2 years of period. As for Maximum motor development, 22 (46.8%) were bed-ridden state, most of them suffering serious delayed development. Patients showed various symptoms with different organs involved, though neuromuscular involvement was most prominent. Delayed development was seen in all cases. Multifocal lesion in brain MRI study was seen in 36 (76.6 %) cases, taking a greater percentage than 11 (23.4%) cases with single lesion. In MR spectroscopy study, the characteristic lactate peak of mitochondrial disease was identified in 20 (42.6%) patients. Conclusions: Further analysis of clinical and biochemical diagnosis on more extended group of patients with Leigh syndrome will enable us to improve diagnostic precision and to understand the natural course of mitochondrial disease.
Bang, Do Seok;Jung, In Sung;Kang, Ki Man;Park, Bum Chul;Yoon, Young Gul;Kim, Jae Su;Park, Yol;Lee, Sung Hoon;Hong, Young Chul;Ko, Kyoung Tae;Park, Sang Min;Na, Dong Jib
Tuberculosis and Respiratory Diseases
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v.57
no.6
/
pp.604-608
/
2004
A 47-year-old-man was admitted to the emergency department with dyspnea, right pleuritic pain, and high fevers for 3 days. He had a nonproductive cough that exacerbated the chest pain. A clinical examination revealed distressed and slightly tachypneic patient, with blood pressure of 110/90 mmHg, temperature of $39^{\circ}C$, pulse of 90 beats/min, respiratory rate of 24 breaths/min. A chest examination showed significantly diminished breath sounds in the right lung with dullness to percussion. Laboratory investigation demonstrated leukocytosis and a raised C-reactive protein. The results of arterial blood gas analysis revealed moderate hypoxemia. A radiograph and a CT scan of the chest showed extensive consolidation with multifocal low densities, and pleural effusion in the right lung. A diagnostic thoracentesis revealed straw-colored fluid, which was found to be a neutrophil-predominant exudate. At 7 days after admission, the clinical symptoms had not improved and the temperature was still $39^{\circ}C$ despite the aggressive therapy of community-acquired pneumonia. After comprehensive history taking, we realized then that he accidentally aspirated kerosene while siphoning from fuel tank to put into the boiler 3 days ago. Bronchoscopy with bronchial washings could be successful in establishing the diagnosis of hydrocarbon pneumonitis by demonstration of a high lipid-laden macrophage index. Thereafter, the symptoms and radiographic opacities gradually improved, and he was discharged several days later.
Churg-Strauss syndrome is a rare form of systemic necrotizing vasculitis that occurs exclusively in patients with asthma, and is associated with blood and tissue eosinophilia. The classic pathology findings in the lung include a combination of eosinophilic pneumonia, granulomatous inflammation and necrotizing vasculitis. However, there are few reports of tracheobronchial mucosal lesions in Churg-Strauss syndrome. We report a case of Churg-Strauss syndrome with multiple tracheobronchial mucosal lesions in a 33-year-old man with a history of bronchial asthma and allergic rhinitis. He had been diagnosed with community acquired pneumonia at another hospital and was treated with antibiotics. However, the chest radiographic findings were aggravated and showed multifocal consolidations in the whole lung fields. He was transferred to the Asan Medical Center. Fiberoptic bronchoscopy revealed multiple nodular mucosal lesions of the trachea and bronchi. The histopathology of the mucosal lesions revealed necrotizing bronchial inflammation with eosinophilic infiltration. Video Assisted Thoracic Surgery was performed. The wedge resected lung tissue revealed chronic eosinophilic pneumonia that was consistent with Churg-Strauss syndrome. Methylprednisolone (1 mg/kg q 8 hr) was prescribed and his symptoms resolved gradually. The chest radiographic findings improved significantly, and a follow-up fiberoptic bronchoscopy performed eight days later showed that the tracheobronchial mucosal lesions had resolved. The patient was prescribed oral prednisolone for 20 months after discharge. Currently, the patient is not taking steroids and is being followed up.
Kwon, Ah Reum;Park, Eun Jung;Kim, Ki Hwan;Kim, Dong Soo
Clinical and Experimental Pediatrics
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v.53
no.2
/
pp.262-266
/
2010
Tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) is a major proinflammatory cytokine involved in the pathophysiology of juvenile rheumatoid arthritis. Etanercept is an effective inhibitor of $TNF-{\alpha}$ and has shown a beneficial effect in patients with JRA. However, the most important cause of concern related to etanercept administration is infection. We report a case of encephalitis in a JRA patient receiving long-term treatment with etanercept. The patient was a 4-year-old boy with refractory JRA, and he received etanercept subcutaneously at a dose of $0.4\;mg\;kg^{-1}\;day^{-1}$ twice a week for 14 months, along with non-steroidal anti-inflammatory drugs, methotrexate, oral steroids, and sulfasalazine. The patient presented with sudden fever, headache, vomiting, a generalized tonic seizure, and changes in mental status. We suspected a central nervous system infection, and simultaneously administered antibiotics, an antiviral agent, and steroids. After 2 days of hospitalization, his mental function returned to normal, and he showed no further seizure-like movements. Brain magnetic resonance imaging scan of the patient showed a multifocal cortical lesion on both sides of the temporoparietooccipital lobe, which indicated encephalitis. Although we were unable to identify the causative organism of encephalitis, we think that the encephalitis may be attributed to infection, and the use of etanercept may have increased the risk of severe infection. Therefore, etanercept was discontinued and the patient recovered shortly after. To the best of our knowledge, this is the first case of encephalitis in a juvenile rheumatoid arthritis patient treated with etanercept.
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