BACKGROUND: "Rebuild-up" phenomenon, induced by hyperventilation, is a characteristic finding on EEG in children with Moyamoya disease. Its mechanism, however, remains obscure. In this study, we examined the relationship between cerebral lesions on MRI, stenosis or occlusion of cerebral vessel on cerebral angiography, and EEG findings in children with Moyamoya disease. METHODS: We have reviewed medical records of 33 patients, who were confirmed as Moyamoya disease by cerebral angiography at Asan Medical Center. EEG and brain MRI were carried out in all subjects. RESULTS: 1) Epidemiologic data were : the male to female ratio was 1:1.1; highest rate(90.7%) of onset in age group below 10 years; mean age at clinical onset was 7.4 years; average diagnostic interval from clinical onset to diagnosis was 1.9 years. 2) The most common initial and recurrent or residual symptoms were motor deficit. 3) The most common site of occlusion or stenosis of cerebral vessel on cerebral angiography was anterior cerebral area(>97%) and the most common cerebral infarction area on brain MRI was anterior cerebral area, too. 4) The hyperventilation(for 3 minutes) on EEG were carried out in 25 patients and the prolonged build-up or rebuild-up phenomenon was observed in 13 patients(52%). 5) The prolonged build-up or rebuild-up phenomenon on EEG was observed in 6 of 15 patients who were occlusion of cerebral vessel, and in 7 of 10 patients who were stenosis of cerebral vessel on angiography. CONCLUSION: 1) The background slowings on EEG maybe suggestive of the infarction stage of Moyamoya disease in children. 2) The prolonged build-up or rebuild-up phenomenon might indicate the preinfarction stage of Moyamoya disease in children.
Angiography ; Brain ; Cerebral Angiography ; Cerebral Infarction ; Child ; Chungcheongnam-do ; Constriction, Pathologic ; Diagnosis ; Electroencephalography ; Female ; Humans ; Hyperventilation ; Infarction ; Magnetic Resonance Imaging ; Male ; Medical Records ; Moyamoya Disease*

No abstract available.
Biliary Tract* ; Lymphatic Diseases*

BACKGROUND AND OBJECTIVES: The goal of this study was to examine the safety and feasibility of a primary (direct) stenting in acute myocardial infarction (AMI). In the treatment of AMI, Percutaneous transluminal coronary angioplasty (PTCA) has documented superior reperfusion rate and improved clinical outcomes than thrombolytic therapy. However, there are several limitations of PTCA, such as recurrent ischemia in 10 to 15%, reinfarction in 3 to 5% and restenosis in 30 to 50% of patients. There are several reports that, compared with PTCA, the implantation of coronary stent has been shown to reduce angiographic restenosis and improve late clinical outcomes. But in general, stenting has been contraindicated in thrombus containing lesion due to the risk of subacute thrombosis. With advance in technique and the recognition of the importance of adequate platelet inhibition, the incidence of subacute thrombosis has fallen in patients with acute coronary syndrome and thrombus laden lesion. Methods and Results: In our study, primary stenting was performed in 42 patients of AMI. There are 6 cases (22.5%) target lesion restenosis during the follow up coronary angiography (150+/-86day) and no in-hospital death. Three cases (7.1%) of them require revascularization including two re-PCTA and a coronary artery bypass graft for the recurrent ischemic symptoms. There were no reinfarction and death after discharge. Six-months event free survival reate was 85.7%. CONCLUSION: Primary stenting is safe and feasible in the majority of patients with AMI and results in excellent mid-term outcomes compared with PTCA.
Acute Coronary Syndrome ; Angioplasty, Balloon, Coronary ; Blood Platelets ; Coronary Angiography ; Coronary Artery Bypass ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Incidence ; Ischemia ; Myocardial Infarction* ; Reperfusion ; Stents* ; Thrombolytic Therapy ; Thrombosis ; Transplants

Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

We report a rare cause of lung cavities, occurring in a patient with intrapulmonary cystic teratoma. Computed tomography (CT) provided us more detailed informations about the tumor characteristics containing fat and calcification, which could not be distinguished on the plain radiographs. In addition, CTscans clearly demonstrated the dilated anterior segmental bronchus of the left upper lobe entering the posterior aspect of the cavity.
Bronchi ; Humans ; Lung* ; Teratoma*

The shortage of available organ donors is a significant problem and various efforts have been made to avoid the loss of organ donors. Among these, extracorporeal membrane oxygenation (ECMO) has been introduced to help support and manage potential donors. Many traumatic brain injury patients have healthy organs that might be eligible for donation for transplantation. However, the condition of a donor with a fatal brain injury may rapidly deteriorate prior to brain death determination; this frequently results in the loss of eligible donors. Here, we report the use of venoarterial ECMO to support a potential donor with a fatal brain injury before brain death determination, and thereby preserve donor organs. The patient successfully donated his liver and kidneys after brain death determination.
Brain Death* ; Brain Injuries* ; Brain* ; Extracorporeal Membrane Oxygenation* ; Humans ; Kidney ; Liver ; Tissue and Organ Procurement ; Tissue Donors*

Crystalline retinopathy is a tapetoretinal degeneration characterized by glistening yellow intraretinal crystals in the posterior pole, chorioidal sclerosis, and corneal crystalline dystrophy. We have recently encountered a 36 year-old female patient with bilateral intraretinal crystalline depositions who presented with a complaint of visual disturbance in her right eye for the last six months. Typical fundus findings of crystalline deposition in the posterior pole, tapetoretinal dysfunctions in the electrophysiologic tests and choriocapillaris filling defects in the fluorescein angiogram suggest that this the disorder is a widespread choriocapillaris and tapetoretinal disorder. There are no significant abnormal findings in serologic and radiologic examinations.
Adult ; Crystallins* ; Female ; Fluorescein ; Humans ; Retinitis Pigmentosa ; Sclerosis

No abstract available.
Finger Injuries* ; Fingers*

BACKGROUND: It has been generally accepted that Cisapride (Prepulsid?or propulsid?), a widely used gastrointestinal prokinetic agent, is associated with Torsades de Points, a life-threatening arrhythmia. Recently, cisapride-induced APD (action potential duration)-prolongation was inhibited by glibenclamide, a KATP channel blocker. But the direct effect of cisapride on K(ATP) channels has not been studied until now. Therefore, we investigated cisapride's effects on KATP channels of isolated rat ventricular myocytes. METHODS: After the isolation of rat ventricular myocytes, we analysed the single channel current with patch pipettes. The method of analysis was the student t-test. RESULTS: 1) Cisapride (10(-6) M- 10(-4) M) inhibited KATP channel opening without changing channel conductance Ki was about 20micronM, and Hill coefficient was 0.75. 2) Cisapride inhibited pinacidil-induced KATP channel opening in the cell attached mode. CONCLUSIONS: These results suggest that cisapride-induced APD prolongation and arrythmic effects may be partly related to KATP channel inhibition.
Animals ; Arrhythmias, Cardiac ; Cisapride* ; Glyburide ; Humans ; KATP Channels ; Muscle Cells ; Rats

A 36-year-old woman was presented with extensive anterior wall myocardial infarction. We tried to perform direct coronary angiography for the purpose of primary stenting. However, coronary angiogram revealed normal coronary arteries without intracoronary thrombi. We continued further evaluations to find out the cause of normal coronary myocardial infarction. The findings of severe hypertensive retinopathy and concentric left ventricular hypertrophy suggested that she had secondary hypertension. The detailed history, laboratory and radiological findings revealed the pheochromocytoma. The tumor was successfully removed by operation.
Adult ; Anterior Wall Myocardial Infarction ; Coronary Angiography ; Coronary Vessels ; Female ; Humans ; Hypertension ; Hypertensive Retinopathy ; Hypertrophy, Left Ventricular ; Myocardial Infarction* ; Pheochromocytoma* ; Stents