Emery-Dreifuss muscular dystrophy has become recognized as a distinct neuromuscular disorder with features including X-linked inheritance, insidious onset in childhood of a distinct pattern of muscle contractures and weakness, slow progression without loss of ambulation, and occurrence by mid-childhood of atrial conduction defects, which, if untreated, cause sudden death. We report a case of Emery-Dreifuss dystrophy with cardiac involvement of atrial standstill. The patient was 24 year-old man, who had suffered from dyspnea and bradycardia and was inserted by VVI type permanent pacemaker. Cardiac involvement usually becomes evident as muscle weakness progress and provided that the diagnosis is made sufficiently early, the insertion of a cardiac pacemaker can be life saving.
Bradycardia ; Contracture ; Death, Sudden ; Diagnosis ; Dyspnea ; Genes, X-Linked ; Humans ; Muscle Weakness ; Muscular Dystrophy, Emery-Dreifuss* ; Walking ; Young Adult

No abstract available.
Magnetic Resonance Imaging ; Malformations of Cortical Development*

A fifty-five year old male was admitted because of fever and chill for one month. He complained of upper abdominal pain, malaise, anorexia and weight loss. So diagnostic procedures were performed: stool exam., bload chemistry testing, ultrasonography, radionuclide scintigraphy. ERCP, abdominal CT, and sonoguided liver aspiration, which revealed intrahepatic cholangiocarcinoma associated with clonorchiasis.
Abdominal Pain ; Anorexia ; Chemistry ; Cholangiocarcinoma* ; Cholangiopancreatography, Endoscopic Retrograde ; Clonorchiasis* ; Fever ; Humans ; Liver ; Male ; Radionuclide Imaging ; Tomography, X-Ray Computed ; Ultrasonography ; Weight Loss ; Cholangiocarcinoma

PURPOSE: Total anomalous venous return(TAPVR) is associated in more than 60Yo of patients with right isomerism and can significantly complicate the management of single ventricle patients at any stage of management. We studied the results of management and sought to determine factors that may influence survival in patients with TAPVR in right atrial isomerism. METHODS: Between February 1991 and July 1999, 14 patients with TAPVR in right atrial isomerism underwent operations,' we reviewed our experience after performing single ventricle palliation RESULTS: Seven patients were of the obstructive type TAPVR and seven patients were of the non-obstructive type TAPVR. The mean age at operation was 17 months and mean body weight at operation was 7.3kg. Direct surgical repair for the pulmonary vein was performed in seven patients and in the others, TAPVR could be satisfactorily managed by the use of bilateral cavopulrnonary anastomosis(BCPS) to exclude the distal superior vena cava(SVC). At a mean follow-up of 27 months, there were five deaths, arid pulrnonary vein restenosis developed in four patients in the direct surgical repair group. In the other group, there was neither mortality nor morbidity. Also, the presence of pulmonary venous obstruction was associated with high mortality. CONCLUSION: According to our study, TAPVR can be satisfactorily managed by the use of BCPS to exclude the distal SVC in the non-obstructive type. But further evaluation of surgical methods about other types of TAPVR are warranted, because TAPVR not requiring intervention includes any low supracardiac and some mixed types.
Body Weight ; Follow-Up Studies* ; Heterotaxy Syndrome* ; Humans ; Isomerism ; Mortality ; Pulmonary Veins ; Scimitar Syndrome* ; Veins

Atrial flutter, a common rhythm disturbance, was first described over 80 years ago. Despite extensive investigations, several important issues remain unresolved concerning its exact mechanism and management. Present therapeutic strategies often appear effective to prevent and terminate atrial flutter. However, controlled trial and definitive studies comparing the various treatment options are surprisingly scarce. Here we report on a study of 9 episodes of spontaneous atrial flutter(AF)(flutter wave cycle length 224+/-39 msec) treatedd by transesophageal atrial pacing(TAP) in 9 patients(7 men and 2 women; mean age 56.9 yrs). TAP was effective in 5 patients : sinus rhythm resumption was immediate in 3 patients and followed a short period of atrial fibrillation in 2 patients. TAP was unsuccessful in 4 patients. All the patients tolerated the procedure well. These data strongly support the immediate first choice use of TAP in AF therapy.
Atrial Fibrillation ; Atrial Flutter* ; Female ; Humans ; Male

No abstract available.
Classification* ; Risk Factors* ; Trophoblastic Neoplasms* ; Trophoblasts*

Ventricular tachycardia is an important tachyarrhythmia which is encountered commonly in clinical field. The accompanying manifestations could be variable just from palpitation to sudden cardiac death. The classification of this arrhythmia has not completly settled yet, but in a broad way this arrhythmia is classified according to the prescence or abscence or underlying heart disease, especially coronary artery disease. Recently, therapeutic modalities for this arrhythmia have been changed a lot from classical antiarrhythmic drugs to radiofrequency ablation or ICD implantation although there are still some problems to overcome. We experienced a case of 34-year-old female having an incessant ventricular tacycardia which was abolished after endomyocardial biopsy performed to differentiate underlying myocardial pathology.
Adult ; Anti-Arrhythmia Agents ; Arrhythmias, Cardiac ; Biopsy* ; Catheter Ablation ; Classification ; Coronary Artery Disease ; Death, Sudden, Cardiac ; Female ; Heart Diseases ; Humans ; Pathology ; Tachycardia ; Tachycardia, Ventricular*

The purpose of this study was to evaluate the significance of telomerase activity in gestational trophoblastic disease and the association of telomerase activity in complete hydatidiform mole and subsequent development of persistent gestational trophoblastic tumor. By using the standard telomerase repeat assay, we examined telomerase activity in 2 normal placentas, 31 complete hydatidiform moles, 7 invasive moles, 5 choriocarcinoma tissues and choriocarcinoma cell line (JEG-3). Telomerase activity was detected in 13 of 15 (86.7%) complete hydatidiform mole patients who eventually had chemotherapy for the treatment of persistent gestational trophoblastic tumor. All of the 9 patients with metastatic disease (FIGO Stage III) had telomerase activity in their initial molar tissue. In contrast, telomerase activity was evident in only two of 16 (12.5%) complete hydatidiform mole patients with spontaneous remission. While telomerase activity was not detected in normal placentas, high level of telomerase activity was detected in all of 7 invasive moles, 5 choriocarcinoma tissues and choriocarcinoma cell line (JEG-3). The presence of telomerase activity in a complete hydatidiform mole is associated with the development of persistent gestational trophoblastic tumor, such as invasive mole and choriocarcinoma.
Cell Line ; Choriocarcinoma ; Drug Therapy ; Female ; Gestational Trophoblastic Disease* ; Humans ; Hydatidiform Mole ; Hydatidiform Mole, Invasive ; Molar ; Placenta ; Pregnancy ; Remission, Spontaneous ; Telomerase* ; Telomere ; Trophoblastic Neoplasms

Simultaneous occurrence of remote intracerebral hemorrhage (ICH) and intracranial aneurysmal subarachnoid hemorrhage is very rare. We report on a case of concurrent hypertensive ICH at the left thalamus and rupture of an intracranial aneurysm at the bifurcation of the single A2 segment of the anterior cerebral artery in a 64-year-old woman, which was clipped previously, with review of the literature. To our knowledge, this is the first case report demonstrating bleeding of previously clipped aneurysm with simultaneous hypertensive ICH. Hypertensive crisis following ICH seems to have provoked rupture of the residual aneurysm.
Aneurysm ; Anterior Cerebral Artery ; Cerebral Hemorrhage ; Female ; Hemorrhage ; Humans ; Intracranial Aneurysm ; Intracranial Hemorrhage, Hypertensive ; Middle Aged ; Rupture ; Subarachnoid Hemorrhage ; Thalamus

For evaluating the reproductive performances of GTD patients, we found 115 cases of GTD patients, 77 HM and 38 GTT, who became pregnant after the completion of treatments and follow-up period. The results of this study suggest subsequent pregnancies after the completion of treatments may promise normal reproductive outcomes regardless of the chemotherapy.
Drug Therapy ; Follow-Up Studies ; Gestational Trophoblastic Disease* ; Humans ; Pregnancy