A twenty four-year-old female patient had suffered progressive dyspnea for 6 years until death. She denied any symptoms suggestive of connective tissue disease, or deep vein thrombosis. She suffered an episode of pontine infarct in 1995. Four years after diagnosis of primary pulmonary hypertension, she died of sudden death during hospitalization. Gross features of pulmonary arteries at autopsy were as follows: left main pulmonary artery showed dilation of the lumen and thickening of the wall, and right main pulmonary artery was markedly dilated and contained fresh thrombus. Hematoxylin and eosin-stained sections of lung tissue showed plexiform lesions of pulmonary arteries, complete luminal obliteration of pulmonary arterioles and dilated lesion of pulmonary arterioles, and capillaries. This patient represents a typical case with a primary pulmonary arteriopathy with plexiform lesions with thrombotic lesion, demonstrating the importance of thrombosis in situ in the pathogenesis of primary pulmonary hypertension. To our knowledge, this is the first autopsy report on the primary pulmonary hypertension in Korea.
Arterioles ; Autopsy ; Capillaries ; Connective Tissue Diseases ; Death, Sudden ; Diagnosis ; Dyspnea ; Female ; Hematoxylin ; Hospitalization ; Humans ; Hypertension, Pulmonary* ; Korea ; Lung ; Phenobarbital ; Pulmonary Artery ; Thrombosis ; Venous Thrombosis

Congenital hypohidrotic ectodermal dysplasia is a rare genodermatosis. It is characterized by hypohidrosis hypotrichosis, dental hypoplasia and characterstic facial features, which reflect a wide constellation of developmental defec of tissue from the ectoderm. We have encountered three cases of congenital hypohidrotic ectodermal dysplasia in a 28-year-old female, her new-born baby, and a 10-month-old boy with a family history. All of the three patients had hypohidrosis, hypotrichosis, defective dentition, and characterstic facial features, which were characterstic features of this disorder. In addition, they showed dry skin, sparse and thin hairs. Histopathologic findings of previous cases revealed no eccrine gland structure in the dermis with routine and immunohistochemical stainning such cytokeratin and filaggrin. We report three typical cases of hypohidrotic ectodermal dysplasia with the review of literature.
Adult ; Dentition ; Dermis ; Eccrine Glands ; Ectoderm ; Ectodermal Dysplasia 1, Anhidrotic* ; Female ; Hair ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Keratins ; Male ; Skin

No abstract available.
Replantation

To dtermine the optimal timing for Aortic Valve Replacement in patients of aortic insufficiency is very important, because irreversible myocardial damage can result before symtoms such as dyspnea, syncope and chest pain, appear for the first time. Henry suggested that the echocardiographic index of left ventricular end systolic dimension 55mm could be useful as an indicator of whether to operate or not. But Fioretti, et al., insisted that there was no significant difference postoperatively between those below 55mm and those over 55mm. To determine whether a left ventricular end systolic dimension greater than 55mm is one of the risk factors for AVR in patients with aortic insufficiency, we analyzed the serial echocardiographic examinations of 23 patients who underwent AVR for isolated AI. Group I patients (n=17) had a preoperative left ventricular end systolic dimension less than or equal to 55mm and Group II patients (n=6) had a preoperative left ventricular end systolic dimension greater than than 55mm. 1) left ventricular end systolic dimension decreased in both groups significantly, and there was a statistically significant difference between Group I and Group II at 12 modths after operation. 2) Left ventricular end diastolic dimension also decreased in both groups significantly, and there was a statistically significant difference between Group I and Group II at 12 months after operation. 3) Ejection Fraction was decreased markedly just postoperatively, but it was recovered soon in both groups, and there was no statistically significant differences of the ejection fractions between the two groups postoperatively. 4) SV1+/-RV6 in EKG, as an indicator of LV mass, was also analzed and it revealed significant reduction in both groups postoperatively, but we could not observe statistically significant differences between the two groups from 6 months after the operation. We concluded that the preoperative left ventricular end systolic dimension 55mm could be useful as an index to predict postoperative prognosis and to determine the timing of aortic valve replacement.
Aortic Valve* ; Chest Pain ; Dyspnea ; Echocardiography* ; Electrocardiography ; Humans ; Prognosis ; Risk Factors ; Syncope

We evaluated retrospectively the clinical characteristics of 16 consecutive exotropia patients over 10PD following surgery for esotropia, the patients were differentiated into nonoperative and operative group. The nonoperative group comprised of 11 patients with exodeviation 10PD through 20PD(mean 12.9PD) and the orthphoria through 15PD(mean 10.5PD) at final visit. The operative group of 5 patients with exodeviation of 10PD through 30PD(mean 21PD) at postoperatively 2 weeks and 20PD through 35PD(mean 24PD) before operation of consecutive exotropia. In the second operatio, 3 patients with adduction limitation were operated with advancement of medial rectus or recession of lateral rectus of same eye. Four patients showed orthotropia at postoperatively 2 weeks, and 2 patients revealed orthotropia at the final visit. The results suggest that the degree of deviation and adduction limitation will influence the choice of therapy and second operation method.
Esotropia ; Exotropia ; Humans ; Retrospective Studies

We evaluated retrospectively the clinical characteristics of 16 consecutive exotropia patients over 10PD following surgery for esotropia, the patients were differentiated into nonoperative and operative group. The nonoperative group comprised of 11 patients with exodeviation 10PD through 20PD(mean 12.9PD) and the orthphoria through 15PD(mean 10.5PD) at final visit. The operative group of 5 patients with exodeviation of 10PD through 30PD(mean 21PD) at postoperatively 2 weeks and 20PD through 35PD(mean 24PD) before operation of consecutive exotropia. In the second operatio, 3 patients with adduction limitation were operated with advancement of medial rectus or recession of lateral rectus of same eye. Four patients showed orthotropia at postoperatively 2 weeks, and 2 patients revealed orthotropia at the final visit. The results suggest that the degree of deviation and adduction limitation will influence the choice of therapy and second operation method.
Esotropia ; Exotropia ; Humans ; Retrospective Studies

BACKGROUND: Peripheral arterial disease is frequently accompanied with systemic arteriosclerosis and more than half of the cause of deaths is due to the development of coronary arterial disease. Moreover, it is known that the most frequent cause of death after a bypass surgery of chronic arterial obstruction is heart related complications. Especially in patients with atherosclerotic arterial obstruction in the lower extremities who had no history of heart disease or had no presenting symptoms of ischemic heart disease showed a high rate of postoperative mortality and for this reason we suggest preoperative evaluation in these patients to evaluate whether or not coronary arterial disease is accompanied. MATERIAL AND METHOD: Since Feb. 2001 to Oct. 2004, we analyzed 52 patients who were operated on for atherosclerotic arterial obstruction in the lower extremities, with the exception of patients with a past history of heart disease or symptoms of ischemic heart disease. They underwent on the same day a coronary and femoral angiography for evaluation of accompanying coronary arterial disease. Of among these patients, we compared those who received bypass surgery of the arteries of the peripheral extremities alone to those who underwent combined coronary artery bypass surgery. RESULT: 63% of the reported cases of atherosclerotic arterial obstruction in the lower extremities were accompanied with coronary arterial disease. Old age, hypertension, diabetes mellitus, smoking, and hypercholesterolemia are known risk factors for arteriosclerosis and of these, only old age and hypertension had statistically significance in patients with severe atherosclerotic arterial obstruction in the lower extremities accompanied with coronary arterial disease. Diabetes, smoking, and hypercholesterolemia showed no statistical significance in this group. With the increase in severity of the range and the degree of atherosclerotic arterial obstruction, coronary arterial disease is frequently accompanied and its severity also increased. Patients who received both peripheral artery and coronary artery bypass surgery showed no difference in the period of hospitalization and ICU stay period compared with patients who received bypass surgery of the arteries of the lower extremities alone. CONCLUSION: Patients with atherosclerotic arterial obstruction in the lower extremities without symptoms of ischemic heart disease should undergo a preoperative coronary angiography to evaluate coronary arterial disease for active treatment, especially in the patients with old age, hypertension and high AVD scores.
Angiography ; Arteries ; Arteriosclerosis ; Atherosclerosis ; Cause of Death ; Coronary Angiography* ; Coronary Artery Bypass ; Diabetes Mellitus ; Extremities ; Heart ; Heart Diseases ; Hospitalization ; Humans ; Hypercholesterolemia ; Hypertension ; Lower Extremity* ; Mortality ; Myocardial Ischemia ; Peripheral Arterial Disease ; Peripheral Vascular Diseases ; Risk Factors ; Smoke ; Smoking

BACKGROUND: Peripheral arterial disease is frequently accompanied with systemic arteriosclerosis and more than half of the cause of deaths is due to the development of coronary arterial disease. Moreover, it is known that the most frequent cause of death after a bypass surgery of chronic arterial obstruction is heart related complications. Especially in patients with atherosclerotic arterial obstruction in the lower extremities who had no history of heart disease or had no presenting symptoms of ischemic heart disease showed a high rate of postoperative mortality and for this reason we suggest preoperative evaluation in these patients to evaluate whether or not coronary arterial disease is accompanied. MATERIAL AND METHOD: Since Feb. 2001 to Oct. 2004, we analyzed 52 patients who were operated on for atherosclerotic arterial obstruction in the lower extremities, with the exception of patients with a past history of heart disease or symptoms of ischemic heart disease. They underwent on the same day a coronary and femoral angiography for evaluation of accompanying coronary arterial disease. Of among these patients, we compared those who received bypass surgery of the arteries of the peripheral extremities alone to those who underwent combined coronary artery bypass surgery. RESULT: 63% of the reported cases of atherosclerotic arterial obstruction in the lower extremities were accompanied with coronary arterial disease. Old age, hypertension, diabetes mellitus, smoking, and hypercholesterolemia are known risk factors for arteriosclerosis and of these, only old age and hypertension had statistically significance in patients with severe atherosclerotic arterial obstruction in the lower extremities accompanied with coronary arterial disease. Diabetes, smoking, and hypercholesterolemia showed no statistical significance in this group. With the increase in severity of the range and the degree of atherosclerotic arterial obstruction, coronary arterial disease is frequently accompanied and its severity also increased. Patients who received both peripheral artery and coronary artery bypass surgery showed no difference in the period of hospitalization and ICU stay period compared with patients who received bypass surgery of the arteries of the lower extremities alone. CONCLUSION: Patients with atherosclerotic arterial obstruction in the lower extremities without symptoms of ischemic heart disease should undergo a preoperative coronary angiography to evaluate coronary arterial disease for active treatment, especially in the patients with old age, hypertension and high AVD scores.
Angiography ; Arteries ; Arteriosclerosis ; Atherosclerosis ; Cause of Death ; Coronary Angiography* ; Coronary Artery Bypass ; Diabetes Mellitus ; Extremities ; Heart ; Heart Diseases ; Hospitalization ; Humans ; Hypercholesterolemia ; Hypertension ; Lower Extremity* ; Mortality ; Myocardial Ischemia ; Peripheral Arterial Disease ; Peripheral Vascular Diseases ; Risk Factors ; Smoke ; Smoking

Angiomyxolipoma is a rare variant of lipoma, which is described by Mai, 1996, at first. The nine cases of which have been reported to date. Microscopically, the lesion consists of adipose tissue with the paucicellular myxoid areas and fat tissue with numerous thin, dilated, and congestive blood vessels. The reported cases mostly located to the superficial layer on the scalp, subungual, extremities in adults. We report one case of angiomyxolipoma located in the submuscular and parosteal area in the distal femur around knee joint in a child.
Adipose Tissue ; Adult ; Blood Vessels ; Child ; Estrogens, Conjugated (USP) ; Extremities ; Femur ; Humans ; Knee Joint ; Lipoma ; Scalp

BACKGROUND: Manual ventilation in pediatric anesthesia limits the anesthesiologist's ability to manage patients suffering from unexpected problems. Although modified Mapleson D systems connected to ventilators have been used for controled ventilation of anesthetized infants and children, the complexity of factors affecting rebreathing within the Mapleson D circuit has resulted in a variety of recommendations for fresh gas flow and minute ventilation. The aim of this study was to devise a formula for ventilator settings which would provide normal tidal volume, respiratory rate, and minute ventilation without rebreathing during mechanical ventilation in pediatric anesthesia and compare this method to manual ventilation. METHODS: 56 infants and small children anesthetized with enflurane and nitrous oxide were studied. We have constructed a formula for ventilator settings which would generate a predictable normocapnia. PetCO2, PaCO2, SpO2, and SaO2 were measured during manual ventilation (control) and during mechanical ventilation. RESULTS: Mean PaCO2 with the mechanical ventilation was within normal range. PaCO2 was significantly lower (P<0.05) with the manual ventilation than with the mechanical ventilation. CONCLUSIONS: We conclude that our formula for ventilator settings can be safely and competently applied to mechanical ventilation with Jackson-Rees system in pediatric anesthesia.
Anesthesia* ; Child ; Enflurane ; Humans ; Infant ; Nitrous Oxide ; Reference Values ; Respiration, Artificial ; Respiratory Rate ; Tidal Volume ; Ventilation* ; Ventilators, Mechanical