Beckwith-Wiedemann sydrome is a multisystemic pattern of congenital anomalies with overgrowth. This syndrome is first described independently by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We experienced a case of Beckwith-Wiedemann syndrome who developed left adrenal cortical neoplasm of indeterminate malignant potential.
Beckwith-Wiedemann Syndrome ; Hernia, Umbilical ; Hypoglycemia ; Macroglossia ; Wilms Tumor

No abstract available.
Anemia*

No abstract available.
Child* ; Humans

Acute arterial occlusion of the extremity may result from obstruction of an artery by embolism or by thrombosis in situ. This results in the sudden cessation of blood flow to an extremity. So immediate managements are required to prevent propagation of the clot and to restore blood flow to the ischemic extremity promptly. We report a case of a acute arterial occlusion which was developed during prolonged fasting. A 59-year-old male was transferred due to severe ischemic pain, coldness and loss of pulse in left lower extremity during fast. The arteriogram shows a complete obstruction of external iliac artery and non-visualization of femoral artery and popliteotibial artery in the left lower extremity. Selective intra-arterial urokinase thrombolytic therapy and percutaneous transluminal angioplasty resulted in recannulation of obstructed artery and relief of symptoms.
Angioplasty ; Arteries ; Embolism ; Extremities ; Fasting* ; Femoral Artery ; Humans ; Iliac Artery ; Lower Extremity* ; Male ; Middle Aged ; Thrombolytic Therapy ; Thrombosis ; Urokinase-Type Plasminogen Activator

No abstract available.
Diabetes Mellitus, Type 1* ; Echocardiography*

No abstract available.
Hypereosinophilic Syndrome*

PURPOSE: Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. METHODS: Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. RESULTS: Ten (67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. O2 saturation and pH were lower and cardiothoracic (CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia (P< 0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but O2 saturation was lower in functional atresia group (P< 0.05). 13 patients (87%) were managed with PGE1. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period (mean 37 months). 3 patients (20%) died and none of patients without pulmonary atresia died. CONCLUSION: We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.
Infant, Newborn ; Humans

Arrythmias are often present and may lead to unexpected death in acute myocarditis. Occasionally, conduction disturbances are seen with varying degree of heart block yet complete atriove ntricular block is rare. We experienced a case of transient complete heart block caused by coxsackie B5myocarditis in a previously healthy 12-year-old girl. During the use of a transvenous temporary pacemaker for 5 days, the abnormal cardiac rhythm changed to sinus rhythm and clinical status improved progressively.
Arrhythmias, Cardiac ; Atrioventricular Block* ; Child ; Female ; Heart Block ; Humans ; Myocarditis*

Cementifying fibroma is an odontogenic tumor of periodontal ligament origin, consistingof a proliferation of fibrous tissue that forms cementum. Cemento-ossifying fibroma, cemenrifying fibroma and ossifying firoma are difficult to distinguish on clinical and radiographic examination, and on histoapathology. However, when the calcified products is represented by ovoid or curvilinear deposites, the lesion is often referred to as cementifying fibroma. Cementifying fibroma of the jaws are well-circumscribed, generally slow-growing lesions. Although most lesions appear to be limited to the tooth-bearing areas, a few have extended into the angle-ramus area or encroached on the maxillary sinus. Radiographically, the cementifying fibroma could present as a radiolucent, radiopaque or mixed density lesion, depending on the degree of maturity. We have oberved two cases f cementifyingfibroma occurred in the left maxillary premolar-molar area of 38-year-old woman and 35-year old man. We obtained that two cases were shown the followed results ; 1. Clinically, main clinical sympton was facial swelling and pain at palpation. 2. Radiographically, well-circumscribed radiolucent lesion and radiolucent lesion with radiopaque foci were seen on the left maxillary premolar-molar areas. Loss of lamina dura and root resorption or divergency were also seen. 3. Histopathologically, many cementum-like calcifications were observed in the hypercellular connective tissue.
Adult ; Connective Tissue ; Dental Cementum ; Female ; Fibroma* ; Humans ; Jaw ; Maxillary Sinus ; Odontogenic Tumors ; Palpation ; Periodontal Ligament ; Root Resorption

The incidence of colorectal cancer has been increased gradually and it was about 6.9% of all malignancies in Korea. Early diagnosis was recognized as the most important factor influencing the prognosis of colorectal cancer and the incidence of early colorectal cancer was increasing. Thus great change was observed in the treatment of early colorectal cancer, endoscopic therapy as well as curative surgical resection is being accepted. We report a case of effective and safe endoscopic therapy for early rectal cancer in 5S-year-old male patient. He presented with rectal prolapse and bleeding of a year's duration. A large polypoid mass with fine nodular surface and thick, short stalk was seen in the rectum at 4cm above the anal verge during flexible sigmoidoscopy. After the histologic examination of specimen obtained by bite biopsy, CT scan of pelvic cavity and ultrasonogram of abdomen, the tumor was removed safe and completely by 2 sessions of piecemeal polypectomies and strip biopsy. He has been well without symptoms and signs of recurrence of rectal cancer for 20 months.
Abdomen ; Biopsy* ; Colorectal Neoplasms ; Early Diagnosis ; Hemorrhage ; Humans ; Incidence ; Korea ; Male ; Prognosis ; Rectal Neoplasms* ; Rectal Prolapse ; Rectum ; Recurrence ; Sigmoidoscopy ; Tomography, X-Ray Computed ; Ultrasonography