Annuloaortic ectasia, cystic medial degeneration of the afflicted aortic wall leading to progressive dilatation, is often accompanied by Marfan's syndrome. Some portions of intimal flap is commonly demonstrated along the aorta in the noninvasive diagnosis of aortic dissection. We report the first case of circumscribed aortic dissection developed in a 28 year old obese non-Marfanoid patient. He was transferred after thrombolytic therapy at a community hospital because of severe chest pain and ST segment elevation. Transthoracic echocardiography showed markedly dilated aortic root, moderate amount of pericardial effusion, mild aortic regurgitation in spite of normal regional wall motion of left ventricle. Intimal flap, characteristic of aortic dissection, was not seen with computed tomography. Intimal tear was demonstrated just above aortic valve only by transesophageal echocardiography. Two parallel intimal tear and small circumscribed dissection was demonstrated by autopsy.
Adult ; Aorta ; Aortic Valve ; Aortic Valve Insufficiency ; Autopsy ; Chest Pain ; Diagnosis ; Dilatation ; Dilatation, Pathologic* ; Echocardiography ; Echocardiography, Transesophageal ; Heart Ventricles ; Hospitals, Community ; Humans ; Marfan Syndrome ; Pericardial Effusion ; Thrombolytic Therapy

Tuberous sclerosis is a rare disease, which occurs sporadically or hereditarily and is recognized by its neurological and dermatological manifestations and may be accompanied with renal anomalies. The classical triad is composed of seizure, mental retardation and adenoma sebaceum on face. We experienced two cases of tuberous sclerosis in sporadic forms by mutation without any familial history which suggests the diseases were occurred by mutation rather than by autosomal dominant inheritance. In the first case, a 24-year-female patient with hypertension and abnormal renal function tests which were noted on the routine prenatal check at 32 weeks of gestation delivered normally at 37 weeks. The daughter of patient had seizure when she was 6 years old and was diagnosed as polycystic kidney disease by abdominal computed tomography. This case developed sporadic form of disease without familial history but, the daughter of patient might inherited by autosomal dominant form. The patient's clinical feature was characterized by history of epilepsy, painless abdominal mass due to polycystic kidney disease, abnormal renal function, skin abnormalites including angiofibroma and shagreen patch. Abdominal computed tomography demonstrated numerous variable sized cysts throughout both kidney. In second case, the patient was a 32-year-female patient complaining of 5kg weight gain, abdominal distension due to palpable masses. Her clinical feature was characterized by bilateral huge renal angiomyolipoma with normal renal function and skin abnormality such as erythematous papule on the face. Abd CT and MRI revealed huge angiomyolipoma of about 15cm X 18.5cm X 30cm and 14.5cm X 18cm X 30cm respectively. We presented the two cases with brief review of the literatures.
Angiofibroma ; Angiomyolipoma ; Child ; Epilepsy ; Humans ; Hypertension ; Intellectual Disability ; Kidney ; Magnetic Resonance Imaging ; Nuclear Family ; Polycystic Kidney Diseases ; Pregnancy ; Rare Diseases ; Seizures ; Skin ; Skin Abnormalities ; Tuberous Sclerosis* ; Weight Gain ; Wills

BACKGROUND/AIMS: Midazolam is utilized as a premedication for uppoer gastrointestinal endoscopy. Midazolam has a more rapid onset of reaction than that of diazepam and its duration is shorter. But the Consciousness of premedicated patients has not been regained sooner. The Purpose of this study was to examine the effectiveness of flumazenil against midazolam as premedication for upper gastrointesinal endoscopy. METHODS: Sixty patients underwent upper gastrointestinal endoscopy. These patients were divided to three groups: Group I included twenty patients without premedication; Group II Included twenty patients with premedication of midazolam and then were not given an antisedative agent excluign of normal saline; and Group III included the others with midazolam and flumazenil as an antisedative agent. RESULTS: There was no change in vital signs after midazolam and flumazenil as an antisedative agent. RESULTS: There was no change in vital signs after midazolam injection, compared with presedation value. Modified Steward Coma Scale showed a significant increase after flumazenil injection as an antagonist of midazolam. The assessment of the endoscopist and the comfort of patients were satisfactory. When the 40 patients were asked about their willingness to undergo the same procedure in the future, thirty-four patients responded favorably. CONCLUSION: Midazolam was safe and effective for sedation for upper gastrointestinal endoscopy. There was rapid regaining of consciousness with flumazenil indection after midazolam, so the use of flumazenil against midazolam injection also appeared to be effective.
Coma ; Consciousness ; Diazepam ; Endoscopy ; Endoscopy, Gastrointestinal* ; Flumazenil* ; Humans ; Midazolam* ; Premedication* ; Vital Signs

BACKGROUND: The mechanism of the closure of umbilical vessels is known to be multifactorial. In order to verify that apoptosis is one of the possible closure mechanisms, we studied to identify apoptosis in umbilical vessels and evaluate its mechanism by studying apoptosis-related gene and the relationship between the pattern of apoptosis and gestational age (GA). METHODS: Twelve umbilical cords of GA of 37-42 weeks were obtained immediatly (less than 10 min. for minimal ongoing external influences) after birth. The presence of apoptotic cells was demonstrated by electron microscopy (EM) and terminal deoxynucleotidyl transferase- mediated dUTP nick end labeling (TUNEL). Immunohistochemical staining and Western blotting were used for the analysis of the proteins of apoptosis-related gene. RESULTS: Apoptosis of the smooth muscle cells of media and intima of umbilical vessels was identified at EM, regardless of GA from 37 to 42 weeks. The proportion of TUNEL (+) cells was 80% in intima, 40% in media, 80% in connective tissue of umbilical cord. The expressions of bax-alpha, bcl-Xs/L were strong in intima, in media and connective tissue, but those of bcl-2 were detected in only in connective tissue, regardless of GA in immunohistochemistry. The ratios of expressions of bax-alpha to bcl-2, bcl-Xs to bcl-XL, did not change with increasing GA from 37 to 42 weeks in Western blotting. CONCLUSION: Apoptosis was identified in umbilical vessels. The closure of umbilical vessels can be explained by apoptosis where the ratios of bax-alpha/bcl-2, bcl-Xs/ bcl-XL play an important role. The fact that there were no differences in the extent of apoptosis and the expressions of bax-alpha/bcl-2, bcl-Xs/bcl-XL according to GA, suggests that apoptosis of umbilical vessels is more dependent on the external stimuli during delivery than GA.
Apoptosis* ; Blotting, Western ; Connective Tissue ; Gestational Age ; Humans* ; Immunohistochemistry ; In Situ Nick-End Labeling ; Microscopy, Electron ; Myocytes, Smooth Muscle ; Parturition* ; Umbilical Cord

BACKGROUND AND OBJECTIVES: revious studies have shown a high restenosis rate after balloon angioplasty for diffuse in-stent restenosis. Debulking strategy has been expected to be helpful to reduce the restenosis rate. This study evaluated the safety and long-term clinical event rate after excimer laser coronary angioplasty (ELCA) and adjunctive balloon angioplasty for in-stent restenosis. MATERIALS AND METHOD: We included 29 in-stent restenotic lesions treated in 28 patients (18 men, 10 women, mean age 60+/-2 years) admitted to Samsung Medical Center between June 1997 and August 1998. Quantitative coronary angiography was performed and clinical characteristics, acute complications, 30-day and 8-month major cardiac adverse event rate was analyzed. RESULTS: Initial success rate was 97%. We stopped the ELCA procedure in one lesion located in the proximal left anterior descending artery due to bradycardia and hypotension. In the 28 lesions successfully treated with ELCA and adjunctive balloon angioplasty, the minimal luminal diameter increased from 0.7+/-0.1 mm before ELCA to 1.9+/-0.1 mm after ELCA and to 2.7+/-0.1 mm after adjunctive balloon angioplasty (p<.0001). The acute luminal gain after ELCA was 60%. The diameter stenosis decreased from 75+/-2% before ELCA to 36+/-2% after ELCA and to 15+/-2% after adjunctive balloon angioplasty (p<.0001). There was no in-hospital death, Q wave acute myocardial infarction (AMI), emergency coronary artery bypass graft (CABG), but non-Q AMI was noted in 1 case (3%). During the followed-up period of 8 months, there were 1 death (4%) due to congestive heart failure, 1 nonQ-AMI (4%) and 7 target lesion revascularization (26%) among the successfully treated 27 patients, but there was no CABG, Q-AMI. Combined event rate at the 8-month follow-up was 33% and target lesion revascularization rate at 8-month follow-up was 26%. CONCLUSION: The ELCA and adjunctive balloon angioplasty seems to be safe and effective for the treatment of in-stent restenosis. A prospective randomized trial comparing ELCA versus other ablative technique is required.
Angioplasty* ; Angioplasty, Balloon ; Arteries ; Bradycardia ; Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Bypass ; Emergencies ; Female ; Follow-Up Studies* ; Heart Failure ; Humans ; Hypotension ; Lasers, Excimer* ; Male ; Myocardial Infarction ; Phenobarbital ; Transplants

BACKGROUND: Induction of coagulation necrosis by using thermal energy sources such as radio-frequency (RF), microwaves and lasers has recently been as a new, minimally invasive technique for percutaneous tumor ablation. RF ablation is considerd effective and safe for the local control of small HCCs. The aim of this study is to evaluate the therapeutic efficacy and complications according to the diameter and location of the HCC. METHODS: Between May 1999 and December 2000, one hundred fifty patients underwent RF thermal ablation in Dong-A university hospital. Among them, forty nine patients were enrolled in this study who have no history of prior other treatment including hepatic resection, TAE, PEI, and chemotherapy, also who have follow-up CT performed at least six months after ablation. The patients devided into two groups, who have small HCC (3 cm in diameter), the mean follow-up duration was 10.8 months and local recurrence rate was 51.0%+/-12.4. In small HCC group ( Carcinoma, Hepatocellular* ; Drug Therapy ; Follow-Up Studies ; Hemorrhage ; Humans ; Liver ; Microwaves ; Necrosis ; Pleural Effusion ; Pneumothorax ; Recurrence ; Tomography, Spiral Computed