The Marfan syndrome, a rare disease causing a marked decrease in life expectancy by involving the skeletal, ocular, and cardiovascular systems, is known as a connective tissue disorder that is inherited autosomal dominant. The cardiovascular complications directly related to the cause of death are associated with more than 90% of the Marfan syndrome. Aortic aneurysm with rupture could occur because of dilatation of aorta due to defect of media. Aortic and mitral insufficiency, mitral valve prolapse, bacterial endocarditis, arrhythmia, and aneurysm of interatrial septum are also frequently observed. A few literatures were reported domestically pertaining to the Marfan syndrome thus far, and there are increased discoveries of cardiovascular complications of the syndrome with the application of echocardiogram. However, the Marfan syndrome with LA myxoma has never been reported both domestically and internationally(INDEX MEDICUS, 1966-1989). Therefore we hereby report a case of the Marfan syndrome with LA myxoma from the observation of a patient who was admitted to Eulji General Hospital at Taejeon i August of 1989.
Aneurysm ; Aorta ; Aortic Aneurysm ; Arrhythmias, Cardiac ; Cardiovascular System ; Cause of Death ; Connective Tissue ; Daejeon ; Dilatation ; Echocardiography* ; Endocarditis, Bacterial ; Hospitals, General ; Humans ; Life Expectancy ; Marfan Syndrome* ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Myxoma* ; Rare Diseases ; Rupture

Krukenberg tumor, which can account for 30-40% of metastatic cancers to the ovaries, arises in the ovarian stroma and is usually metastatic from the gastrointestinal tract, especially from the stomach. The pathognomonic feature is the presence of signet ring cells, which may be arranged in acini or appear as individual cells. We experienced a Krukenberg tumor which was bilateral and associated with left ovarian dermoid cyst. This patient was treated for a primary gastric carcinoma(Stage II) about 3 years ago. After bilateral salpingoophorectomy, she received adjuvant chemotherapy. But, she died about fow months after operation.
Chemotherapy, Adjuvant ; Dermoid Cyst* ; Female ; Gastrointestinal Tract ; Humans ; Krukenberg Tumor* ; Ovary ; Stomach

This study was designed to identify, describe and classify orientations of cancer patient's family members to death and to identify factors related to their attitudes on death. Death to the male is understood as a comprehensive system and believed to be highly subjective experience. Therefore attitude on death is affected by personalities. As an attempt to measure the subjective meaning toward death, the unstructured Q-methodology was used. Korean Death Orientation Questionnaire prepared by Kim was used. Item-reliability and Sorting reliability were tested. Forty five cancer patient's family members hospitalized in one university medical center in Seoul were sampled. Sorting the 65 Q-items according to the level of personal agreement; A forced normal distribution into the 11 levels, were carried out by the 45 P-samples. The demographic data and information related to death orientation of the P-sample was collected through face to face in depth interviews. Data was gathered from August 30 till September 22, 1995. The Z-scores of the Q-items were computed and principal component factor analysis was carried out by PC-QUANL Program. Three unique types of the death orientation were identified and labeled. Type I consists of twenty P-samples. Life and death was accepted as people's destiny. They firmly believed the existence of life after life. They kept aloof from death and their concern was facing the end of the life with dignity. They were in favor of organ donation. Type II consists of Nine P-samples. They considered that death was the end of everything and did not believed the life after life. They were very concerned about the present life. Type III consists of Sixteen P-samples. They regarded the death as a natural phenomena. And they considered that the man is just a traveller and is bound to head for the next life which is believed to be free of agony, pain or darkness. They neither feared death nor its process. Their concerns were on the activities to prepare themselves for the eternal-life after death. Thus, it was concluded that there were three distinctive type of attitudes on death among cancer patient family members, and their death attitudes were affected by demographic and socio-cultural factors such as sex, education, and religion.
Academic Medical Centers ; Classification* ; Darkness ; Education ; Head ; Humans ; Male ; Surveys and Questionnaires ; Seoul ; Tissue and Organ Procurement

The Marfan syndrome is classified as a heritable disorder of connective tissue with clinical and pathological alterations involving supporting elements. In its classic form, the Marfan syndrome is associated with abnormalities of the eye(ectopia lentis), aorta(aortic dilatation and aortic regurgitation) and skeleton(dolichostenomelia, arachnodactyly and pectus deformity). The authers have experienced a case of marfan syndrome recently. The patient was 30-years-old male complaining of anterior chest pain radiating to upper back. Physical examination showed tall status, increased length of the limbs as compared with the trunk and arachnodactyly. On auscultation, heart sound was normal without murmur. He wore glasses because of moderate myopia. Roentgenogram of the chest P-A revealed normal cardiac and aortic contours and lung fields. Left lateral view of chest X-ray revealed concave depression of sternum showing mild pectus excavatum, fusiform dilatation of aorta from aortic root to mid portion of thoracic aorta and resulting in obliteration of retrosternal clear space. Echocardiogram demonstrated moderate dilatation of aortic root A-P diameter(4.5cm) without aortic or mitral valvular abnormalities and normal size of left ventricular cavity(EDD:5.3cm). These data coupled with a distinct family history could permit the diagnosis of Marfan syndrome.
Aorta ; Aorta, Thoracic ; Arachnodactyly ; Chest Pain ; Connective Tissue ; Depression ; Diagnosis ; Dilatation ; Extremities ; Eyeglasses ; Funnel Chest ; Glass ; Heart Auscultation ; Humans ; Lung ; Male ; Marfan Syndrome* ; Myopia ; Physical Examination ; Sternum ; Thorax

No abstract available.
Cerebral Palsy* ; Early Diagnosis* ; Neurologic Examination*

The effect of Atenolol on the blood pressure were studied in 31 cases of essential hypertension and on the effect of plasma renin activity in 8 cases. There were 8 cases of male with age 49.5(42-70) and 23 cases of female with age 49(35-71) years. 27 cases of 31 cases were untreated hypertensive patients and 4 cases were refractory to hypotensive drugs of diuretics and vasodilators. The Atenolol 50mg once-daily was given to all patients orally for 3 weeks. The blood pressure, heart rate, ECG and symptoms were checked in one week interval. In 8 cases, the plasma renin activity was measured by Dainabot Kit before and after one week medication. The results were as follows: 1. In 27 cases of untreated hypertension, the control blood pressure was 182.9/11.4mmHg in average. The blood pressure decreased in average by 18.3/11mmHg in one week, 23.7/15.9mmHg in two week and 21.5/10.5mmHg in three week. The heart rate also decreased by 7.4/min. in one week, 14.9/min. in two week and 7.8/min. in three week. These data showed considerable reduction of blood pressure and heart rate with 50mg Atenolol once-daily. 2. The basal plasma renin activity after one week medication was reduced by 46% in 8 cases and this data showed considerable reduction regardless control level of basal plasma renin activity. 3. There were considerable blood pressure reduction by adding Atenolol 50mg once-daily to diuretics and vasodilator to which patient's blood pressures were refractory. 4. There was no orthostatic hypotension or serious side effect by Atenolol medication.
Atenolol ; Blood Pressure* ; Diuretics ; Electrocardiography ; Female ; Heart Rate ; Humans ; Hypertension* ; Hypotension, Orthostatic ; Male ; Plasma* ; Renin* ; Vasodilator Agents

BACKGROUND: Primary stenting as a direct reperfusion procedure after acute myocardial infarction might reduce recurrent myocardial infarction and target vessel revascularization. However, result of long or multiple stenting in the long or tandem lesions were not known. METHOD: From Jan. 1996 to Dec. 1998, patients with acute myocardial infarction including cardiogenic shock were undergone primary stenting using long or multiple stent. The clinical end points were death, recurrent infarction, subsequent bypass surgery, or repeat angioplasty of the infarct-related vessel. The results were compared with age, sex, lesion, and risk matched standard stenting group. RESULT: Baseline characteristics were similar for the 20 patients who underwent standard length stenting and the 13 patients who underwent long or multiple stenting. Stent diameter was similar in two group (3.4+/-0.3 mm vs. 3.5+/-0.4 mm, p=0.65), but total stent length was longer in long or multiple stenting group (15.5+/-1.8 mm vs. 40.18.4 mm, p=0.01). Average numbers of stent using in multiple stenting were 1.5+/-0.7. Stenting in the infarct-related artery was successful in all patients randomized to stent treatment. At 6 months, the incidence of the primary end point was 25% (5/20) in the standard stent group and 31%(4/13) in the long or multiple stent group (p=0.5). CONCLUSION: Outcomes of long or multiple stenting including mortality, recurrent myocardial infarction and target vessel revascularization was similar to standard stenting. Long or multiple stenting after acute myocardial infarction may possible procedure in some selective cases having long or tandem lesion.
Angioplasty* ; Arteries ; Humans ; Incidence ; Infarction ; Mortality ; Myocardial Infarction ; Reperfusion ; Shock, Cardiogenic ; Stents*

No abstract available.
Heart Septal Defects, Ventricular* ; Oximetry*

Apexcardiograms were evaluated in 24 normal adults (14 males and 10 females) aged 23 to 56 (mean 39) years and 42 patients with ischemic heart disease (29 males and 13 females) aged 40 to 81 (mean 57) years. The ratio of the duration of slow and rapid filling periods (SFP/RFP) and the height of the a wave in realtion to the total apexcardiographic deflection (a/H) were measured in each case and the results obtained from the IHD group were compared with data from the control group. The results obtained were as follows. 1. The was significant difference (p<0.001) between mean SFP/RFP ratio in control group (2.1+/-0.6) and in IHD group (3.4+/-1.5). Setting the upper SEP/RFP ratio at 2.7(mean+1 S.D.), 57.15 of IHD group and 20.8% of control group were above, and at 3.3(mean+2 S.D.), 40.5% of IHD group and none of control group were above this value. 2. There was no difference between the mean a/H ratio in control group (0.08+/-0.07) and in IHD group (0.09+/-0.11). The a/H ratio exceeding 0.15 (15%) was noted in 11.9% of IHD group. 3. The significance of SFP/RFP ratio in the apexcardiogram in the diagnosis of IHD was discussed. The SFP/RFP ratio is a more useful noninvasive adjunctive measurement for detecting IHD than a/H ratio.
Adult ; Diagnosis ; Humans ; Male ; Myocardial Ischemia*

Diffuse neonatal hemangiomatosis is an uncommon disease that is characteristed by a diffuse nature of the lesions, and distinguished form a single or few, or superficial or deep, capillary, cavernous, or mixed hemangiomas occuring in early or adult life. We report an autopsy case of multiple hemangiomatosis, which is associated with massive osteolysis of right humerus and arteriovenous fistulae in surrounding soft tissues. The patient is a 23 day old female infant and had a 2.0x1.5 cm sized cystic destructive bony lesion which was located in the proximal shaft of right humerus. Right arm was hypertrophied, compared to the normal looking left. There were multiple hemangiomas in right humerus, lung, cutaneous skeletal muscles and nerves. This case shares clinical characteristics of Gorham's disease.
Infant ; Adult ; Male ; Female ; Infant, Newborn ; Humans ; Hemangioma