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.
Yang, Jung Kyung;Lee, Jung-Ho;Kwon, Mi-Hye;Jeong, Ji Hyun;Lee, Go Eun;Cho, Hyun Min;Kim, Young Jin;Jung, Sung Mee;Choi, Eu Gene;Son, Ji Woong;Na, Moon Jun
Tuberculosis and Respiratory Diseases
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v.63
no.3
/
pp.261-267
/
2007
Background: The causes of the pleural effusion are remained unclear in a the substantial number of patients with exudative effusions determined by an examination of the fluid obtained via thoracentesis. Among the various tools for diagnosing exudative pleural effusions, thoracoscopy has a high diagnostic yield for cancer and tuberculosis. Medical thoracoscopy can also be carried out under local anesthesia with mild sedation. The aim of this study was to determine diagnostic accuracy and safety of medical thoracoscopy. Methods: Twenty-five patients with exudative pleural effusions of an unknown cause underwent medical thoracoscopy between October 2005 and September 2006 in Konyang University Hospital. The clinical data such as age, gender, preoperative pulmonary function, amounts of pleural effusion on lateral decubitus radiography were collected. The vital signs were recorded, and arterial blood gas analyses were performed five times during medical thoracoscopy in order to evaluate the cardiopulmonary status and acid-base changes. Results: The mean age of the patients was 56.8 years (range 22-79). The mean depth of the effusion on lateral decubitus radiography (LDR) was 27.49 mm. The medical thoracoscopic pleural biopsy was diagnostic in 24 patients (96.0%), with a diagnosis of tuberculosis pleurisy in 9 patients (36%), malignant effusions in 8 patients (32%), and parapneumonic effusions in 7 patients (28%). Medical thoracoscopy failed to confirm the cause of the pleural effusion in one patient, who was diagnosed with tuberculosis by a pericardial biopsy. There were no significant changes in blood pressure, heart rate, acid-base and no major complications in all cases during medical thoracoscopy (p>0.05). Conclusions: Medical thoracoscopy is a safe method for patients with unknown pleural effusions with a relatively high diagnostic accuracy.
Park Han-Ki;Youn Young-Nam;Yang Hong-Seok;Yoo Byoung-Won;Choi Jae-Young;Park Young-Hwan
Journal of Chest Surgery
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v.39
no.4
s.261
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pp.281-288
/
2006
Background: Extracardiac pericardial-flap lateral tunnel Fontan operation has theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. Material and Method: Clinical data was reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was $2.8{\pm}1.5$ years and the body weight was $12.3{\pm}3.2$ kg. Extracardiac tunnel was constructed using the pedicled pericardium with the base undetached. By reviewing the follow-up cardiac angiograms, the diameter and the cross-sectional area of the lateral tunnel was compared to those of inferior vena cava. Result: There were four operative mortality cases (9.8%) and the causes of death were low cardiac output for all four cases. Postoperatively, five patients had prolonged pleural effusion longer than two weeks and one patient required a permanent pacemaker due to complete heart block. Follow-up was possible in 37 patients and the follow up duration was $3.8{\pm}2.2$ years. During that period, one patient died, of upper gastrointestional bleeding combined with heart failure and one patient died a sudden death of unknown cause. Two patients required reoperation due to subaortic stenosis and anastomosis site stenosis between inferior vena cava and lateral tunnel. In one patient, bradyarrhythmia was anew but there was no thromboembolic complication. The lateral tunnel showed growth in proportion to the size of the inferior vena cava. Conclusion: Extracardiac pericardial-flap lateral tunnel Fontan operation is relatively simple and safe. The mid-term result was favorable and the extracardiac tunnel showed potential for growth.
Recently, video-assisted thoracoscopic surgery for mediastinal lesions has been considered a new effective therapeutic method. From March, 1992 to April, 1997, 33 cases of video assisted thoracoscopic surgery for mediastinal lesions were performed. Gender distribution was 16 males and 17 females. Average age was 42 years old(ranged from 14 to 69). The locations of lesions were anterior mediastinum in 14 cases, middle mediastinum in 5 cases, posterior mediastinum in 11 cases, and superior mediastinum in 3 cases. These included 9 neurilemmomas, 5 benign cystic teratoma, 4 pericardial cysts, 2 ganglioneuroma, 2 thymus, 2 thymic cyst, 1 thymoma, 2 esophageal leiomyomas, 1 dermoid cyst, 1 lipoma, 1 malignant lymphoma, 1 bronchogenic cyst, 1 pericardial effusion, and 1 Boerhaave's disease with empyema. Working window was needed in 6 cases. We converted to open thoracotomy in 6 cases. Reasons of convertion to open thoracotomy were large sized mass(1), severe adhesion(3), and difficult location to approach(2). The average operation time was 116min($\pm$56 min). The average chest tube drainage time was 4.7days. The average hospital stay was 8.7 days. Operative complications were atelectasis(2), empyema with mediastinitis(1), recurrent laryngeal nerve palsy(1), and plenic nerve palsy(1). In conclusion, VATS for mediastinal lesions were performed with shorter operation time and hospital stay, and lesser complications and pain than those of conventional thoracotomy.
We have experienced 2 cases of the hunshot wound sof the chest involving cardiac injuries at department of the thoracic surgery, Capital Armed Forces General Hospital during I year from April I 1979 to Jan. 1980. In one case of two patients , he was a 22 years old man who was transported to this emergency room 4 hour 10 minutes after having gunshot wound of the left chest by helicopter. Physical examination showed small inlet in left 3rd ICS and left parasternal border, large outlet in left 8th ICS and left scapular line, no breath sound on left side and distant heart sound. chest roentgenography demonstrated marked pleural effusion in left side and mediastinum shifted to right. As soon as chest X-ray was taken, the bleeding through penetrating wound became profuse and cardiac arrest ensued. Closed chest cardiac massage was started and vigorous transfusion continued, but no effective cardiac activity could not be obtained. The patient was pronounced dead due to exsanguinating hemorrhage from wuwpected cardiac wounds. In this critically injured patient with evidence of intrathoracic hemorrhage and suspected cardiac penetration, only emergency thoracic exploration and immediate surgical control of bleeding points might offer the maximum possibility of survival. The other case was a 23 years old man who was transferred to the emergency room 4 hours 50 minutes after having kmultiple communicated fractures of sternum and linear fracture of right mandible by a missile. Examination revealed about 30% skin loss of the anterior chest wall, weak pulse of 96 beats/min., distant heart sound and decreased breath sounds bilaterally. finding on the chest X-ray films showed multiple sternal fractures, marked pericardial effusion indicating hemopericardium. So, the patient was moved immediately to the operation room where, after endotracheal tube inserted, a median sternotomy was performced. A hemorrhagic congestion of the right upper lobe and marked bulging pericardium were disclosed. The pericardium was opened anterior to right phrenic nerve and exsanguinating hemorrhage ensued from the 0.5cm lacerated wound in the auricle of right atrium. The rupture site of right atrium was occluded with non-crushing vascular clamps and then was over sewn with interrupted sutures. It was thought to be highly possible that he was alive long enough to have cardiorrhaphy because of cardiac tamponade, which prevented exsanguinating hemorrhage. He was taken closed reduction for linear fracture of right mandible 2 weeks after repair of ruptured right auricle in dental clinic. This patient's post-operative course was not eventful.
Mutant form of the p53 gene product is abnormally accumulated in the nuclei of the tumor cells due to prolonged half life, and readily detected by immunohistochemical methods. To determine the positivity rate of p53 in body cavity fluid according the primary site and histological types of tumors and the utility of p53 immunostaining as an adjunct in the diagnosis of malignancy, we reviewed 69 effusions, including pleural effusion, ascitic fluid, and pericardial fluid, that were diagnosed as overt malignancy and 21 effusions of suspicious malignancy, immunohistochemistry was performed on paraffin-embedded cell blocks using a monoclonal antibody to p53 supressor gene product(Clone DO7) and a standard avidin-biotin complex technique with a citrate buffer antigen retrieval solution. The results were as follows; of the 46 pleural effusions with overt malignancy, 22 were immunopositive for p53 protein; of the 21 ascitic fluids with overt malignancy, 5 were positive for p53. Positivity rates according to the primary sites of tumors were 18 of 34(52.9%), 8 of 21(38.1%), 1 of 9(11.1%) cases of the tumors of the lung, GI tract, and ovary, respectively. According to the histologic types of lung cancer, 11 cases(61.6%) were positive out of 18 adenocarcinomas, 2 of 5 large cell undifferentiated carcinomas, and 1 of 2 small cell undifferentiated carcinomas. Of 21 cases of suspicious malignancy, 6 were positive for p53 and all of them(6/6) were confirmed as adenocarcinoma of the lung or GI tract. These findings indicate that p53 immunostaining using paraffin embedded cell block is useful diagnostic and prognostic marker in body fluid cytology although negative immunostaining does not exclude malignancy.
Park, Chul;Yoo, Jong-Hyun;Kim, Dae-Young;Park, Hee-Myung
Journal of Veterinary Clinics
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v.25
no.3
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pp.187-191
/
2008
A 7-year-old, intact female Yorkshire terrier dog was presented for coughing, anorexia, chest pain and dyspnea. Right lateral thoracic radiograph demonstrated a large mass shape on the heart base with decreased cardiac silhouette and severe right deviation of the trachea with the heart shifted to the left thoracic wall was observed on the ventrodorsal thoracic projection. Echocardiographic examination revealed a large rounded mass compressing left atrium around the heart base without signs of pericardial effusion. On computed tomographic (CT) findings, sagittal CT images depicted the possibility of cranial vena caval invasion and heart base involvement of the mass associated with biatrial compression. Dorsal CT image revealed the right deviation of trachea due to the heart base mass and markedly shrunk lung space was detected on the transverse CT image. Because the dog suddenly had died during the recovery from anesthesia after finishing CT scan, necropsy was performed. On gross findings, a large and lobulated mass was located at the base of the heart. A poorly-demarcated, infiltrative, multilobulated tumor composed of polyhedral cells in solid cellular sheets was confirmed based on histopathologic examination. This dog was diagnosed as a chemodectoma. This case report describes the clinical findings, diagnostic consistency of thoracic radiography, echocardiography and CT, and histopathologic confirmation in a spontaneously occurring chemodectoma with a Yorkshire terrier dog.
Cardiac rupture after blunt chest trauma is a relatively uncommon diagnosis, and it is associated with a very high mortality rate. A 41-years-old man crashed his car into a guardrail and he was then transported to hospital. Although unstable vital signs are the most common symptoms of cardiac injury, this patient had stable vital signs. The chest CT scan showed pericardial effusion, and echocardiography did not clearly reveal cardiac tamponade, but the right atrium was slightly collapsed. Cardiac injury was suspected, and surgery was commenced for obtaining the diagnosis and treatment. A 2cm laceration at the junction of the left atrium and the lefl inferior pulmonary vein was discovered and this was repaired with $4{\sim}0$ Polypropylne monofilament sutures. We report here on the successful management of a patient with left atrial rupture following blunt chest trauma.
Park, Il-Hwan;Bong, Jung-Pyo;Seo, Jung-Ok;Kwon, Jang-Woo
Korean Journal of Bronchoesophagology
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v.15
no.2
/
pp.64-70
/
2009
Descending necrotizing mediastinitis(DNM) can occur as a complication of oropharyngeal and cervical infections that spread to the mediastinum via the cervical spaces. Delayed diagnosis and inadequate mediastinal drainage through a cervical or minor thoracic approach are the primary causes of a high mortality rate. Therefore, We emphasize that aggressive and emergent mediastinal drainage by surgical approach is most important method of DNM treatment. We studied 5cases diagnosed as DNM from 2005 through 2007. All patients underwent emergent surgical drainage of deep neck infection combined with mediastinal drainage through a thoracic approach. Primary oropharyngeal infection lead to DNM in four cases(80%) and odontogenic abscess in one case(20%). The outcomes were favorable 5patients. Overall mortality rate was 0%. The time interval from diagnosis based on manifestation of initial symptoms(oral or pharyngolaryngeal area) to surgical intervention was $7.4{\pm}4.2$days. One patient required reoperation due to remnant mediastinal abscess and pericardial effusion. Early diagnosis and emergent combined drainage with neck and chest incisions, together with broad spectrum intravenous antibiotics, should be considered standard care for this disease. And intensive postoperative care which it is continuous mediastinal irrigation and antibiotics use can significantly reduce the mortality rate.
Park, So-Ra;Lee, Soo-Min;Choi, Seong-Heon;Jung, Yee-Hong;Lee, Soo-Kyung
Journal of Sasang Constitutional Medicine
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v.26
no.4
/
pp.400-408
/
2014
Objectives The aim of this study was to report the improvement of delirium and performance status in the small-cell lung cancer patient who had multiple brain metastases and pericardial effusion after Sasang constitutional treatment. Methods We retrospectively reviewed the medical records, medical laboratory and image scans of 71-year-old male patient diagnosed as small-cell lung cancer. Results The small-cell lung cancer with multiple brain metastases patient sometimes talked deliriously even after the whole brain radiation therapy. During the hospitalization period, he showed delirium. We treated him with Gihwangbaekho-tang and Dojeokgangki-tang as a main therapy. After treatment, he didn't show delirium and performance status was improved. Conclusions A small-cell lung cancer with multiple brain metastases patient showed the improvement of symptoms (delirium, poor performance status, constipation and poor oral intake) with the treatment of Gihwangbaekho-tang, Yanggyuksanhwa-tang and Dojeokgangki-tang.
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