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

Atrial flutter is and infrequent, but potentially unstable tachyarrythmia that occurs in pediatric ages. Transesophageal atrial pacing was used for treatment of 10 episodes of atrial flutter in 7 patients. At the time of atrial flutter conversion, patients were 6 days to 14 years old. 6 patients had associated with congenital heart disease. The atrial cycle length of atrial flutter ranged from 140 to 280 msec with variable atrioventricular conduction. Transesophageal atrial pacing was performed using a bipolar 4 F transesophageal electrode catheter. Atrial flutter conversion was accomplished with stimulation bursts using about 5 seconds of stimuli, 10 msec in duration at 20 to 27 mA. Pacing cycle length was 45 to 110 msec less than the atrial cycle length of tachycardia in 6 episodes. But in a neonate, underdrive pacing converted atrial flutter to sinus rhythm. Conversion attempts were unsuccessful on 2 occasions. Transesophageal atrial pacing is a safe and effective, minimally invasive technique for treatment of atrial flutter in infants and children.
Adolescent ; Atrial Flutter* ; Catheters ; Child* ; Electrodes ; Heart Defects, Congenital ; Humans ; Infant* ; Infant, Newborn ; Tachycardia

Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.
Bacterial Infections ; Cardiovascular Diseases ; Developed Countries ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Opportunistic Infections ; Sepsis* ; Tumor Necrosis Factor-alpha ; Infliximab

BACKGROUND: Idiopathic left ventricular tachycardia with a QRS pattern of right bundle branch block and left axis deviation that is sensitive to verapamil is electrophysiologically distinct arrhythmia entity but descriptions in pediatric ages are infrequent. This study attempted to describe the clinical presentation and dignostic clues from the surface ECG in children with idiopathic left venteicular tachycardia. METHODS: We retrospectively reviewed the medical records, electrocardiograms, Holter recordings, echocardiographic reports of 10 children with idiopathic left ventricular tachycardia who have been managed at Sejong General Hospital and Asan Medical Center, Seoul, Korea from January 1991 to July 1996. Follow-up periods are 2 months to 5 years(mean=26 months). RESULTS: 1) Age of tachycardia onset was 8 months to 14 years(median=4.3 years). In 3 children tachycardia began from infancy. The main complaint in older children was palpitation and chest discomfort but young childen complained abdominal pain during tachycardia attack. Tachycardia was paroxysmal and sustained. Tachycardia responded to intravenous use of verapamil in 9 patients and was controlled by chronic verapamil medication in 6 patients. 2 patients were well without medication. Radiofrequency catheter ablation was done in an adolescent patient successfully. 2) In sinus rhythm, surface ECG showed normal QRS axis and normal QRS morphologies in all patients. During ventricular tachycardia, the heart rate was 183 to 270 beats/min. The taxhycardia of RBBB morphology was observed in all patients. The mean QRS duration was 109+-12ms and the QRS axis was -50* to -90*. Themean RS interval was 60+-12ms. AVdissociation was found on surface RCG in all patients and confirmed in 4 patients by transesophageal ECG. We tried rapid transesophageal atrial pacing in 3 patients and could induce and terminate the ventricular tachycardia. CONCLUSIONS: Idiopathic left ventricular tachycaedia is rare in childhood and is seen from infancy. Idiopathic left ventricular tachycardia is generally benign but occasionally causesmyocardial dysfunction. Idiopathic ventricular tachycardia with a QRS pattern of right bundle branch block amd left axis deviation that is sensitive to verapamil in children should not be confused with supraventricular tachycardia with aberrancy and most helpful diagnostic clue is AV dissociation in surface ECG.
Abdominal Pain ; Adolescent ; Arrhythmias, Cardiac ; Axis, Cervical Vertebra ; Bundle-Branch Block ; Catheter Ablation ; Child ; Chungcheongnam-do ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Rate ; Hospitals, General ; Humans ; Korea ; Medical Records ; Retrospective Studies ; Seoul ; Tachycardia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular* ; Thorax ; Verapamil

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

Historically, gastrointestinal stroma tumors (GIST) have been considered as smooth muscle tumors, but the controversy over this histogenesis is provoked due to various results with utilizing immunohistochemical methods. In andeffort to further clarify the histogenesis of GIST, we performed the immunohistochemical study, as well as histopathologic reexamination, of 24 cases, all diagnosed as smooth muscle tumors of gastrointestinal tract, from Seoul Paik Hospital and Ewha University Hospital between 1980 and 1989, and the main results were as follows; 1) In the histopathologic features by light microscopic study, 11 benign and 13 malignant lesions (including one high grade malignancy and 12 low-grade malignant lesions) were disclosed. 2) In the immunohistochemical study, all tumors showed Vimentin positivity (100%), but no tumor showed S-100 protein positivity (0%), and 7 cases (29.1%) showed Desmin positivity. Positive reaction for Desmin made it possible to suggest that the histogenesis of GIST be in smooth muscle, and neurogenic origin would be excluded by all negativity for S-100 protein. In summary, we would like to conclude that GIST would be smooth muscle tumors on account of their morphological characteristics and their intramural location, but most of them appear poorly differentiated by immunohistochemical method.

OBJECTIVE: Recently, microdissection of tissue sections has been used increasingly for the isolation of morphologically identified homogeneous cell populations, thus overcoming the obstacle of tissue complexity for the analysis cell-specific expression of macromolecules. The aim of the present study was to establish the minimal conditions required for the RNA extraction and amplification from the cells captured by the laser captured microdissection. METHODS: Mouse ovaries were fixed and cut into serial sections (7 micrometer thickness). Oocytes were captured by laser captured microdissection (LCM) method by using PixCell IITM system. The frozen sections were fixed in 70% ethanol and stained with hematoxylin and eosin, while the paraffin sections were stained with Multiple stain. Sections were dehydrated in graded alcohols followed by xylene and air-dried for 20 min prior to LCM. All reactions were performed in ribonuclease free solutions to prevent RNA degradation. After LCM, total RNA extraction from the captured oocytes was performed using the guanidinium isothiocyanate (GITC) solution, and subsequently evaluated by reverse transcriptase -polymerase chain reaction (RT-PCR) for glyceraldehyde-3-phosphate-dehydrogenase (GAPDH). RESULTS: With the frozen sections, detection of the GAPDH mRNA expression in the number of captured 25 oocytes were not repeatable, but the expression was always detectable from 50 oocytes. With 25 oocytes, at least 27 PCR cycles were required, whereas with 50 oocytes, 21 cycles were enough to detect GAPDH expression. Amount of the primary cDNA required for RT-PCR was reduced down to at least 0.25 microl with 50 oocytes, thus the resting 19.75 microl cDNA can be used for the testing other interested gene expression. Tissue-to-slide, tissue-to-tissue forces were very high in the paraffin sections, thus the greater number of cell procurement was required than the frozen sections. CONCLUSION: We have described a method for analyzing gene expression at the RNA level with the homogeneously microdissected cells from the small amount of tissues with complexity. We found that LCM coupled with RT-PCR could detect housekeeping gene expression in 50 oocytes captured. This technique can be easily applied for the study of gene expression with the small amount of tissues.
Alcohols ; Animals ; DNA, Complementary ; Eosine Yellowish-(YS) ; Ethanol ; Female ; Frozen Sections ; Gene Expression* ; Genes, Essential ; Guanidine ; Hematoxylin ; Mice ; Microdissection* ; Oocytes* ; Ovary ; Paraffin ; Polymerase Chain Reaction ; Ribonucleases ; RNA Stability ; RNA* ; RNA, Messenger ; RNA-Directed DNA Polymerase ; Xylenes

From January to December 1984, 191 patients with associated ventricular septal defect(VSD) were studied in the cardiac cathetherization laboratory. Among theses 191 cases, 58 cases(30.4%) of subpulmonic VSD were identified by angiocardiography. Among the 59 cases of subpulmonic VSD, 12 cases(20.6%) were complicated with aortic infficiency(AI). The relative frequency of subpulmonic VSD increased with the increase of age and the frequency of complication of AI with the subpulmonic VSD also increased with the increase of age. The amount of left to right shunt in the patients with subpulmonic VSD and AI was small and Qp/Qs ratio was less than 2.0 in all of the 12 cases. Among the 12 patients 4 cases had a pressure gradient greater than 20 mmHg across the infundibular region of the right ventricle.
Angiocardiography ; Heart Septal Defects, Ventricular* ; Heart Ventricles ; Humans

We reviewed the clinical and hemodynamic studies in 10 patients with the isolated congenital mitral insufficiency. The patients ranged in age from 6 months to 16 years at the time of diagnosis and the incidence was 0.26% among the total congenital heart disease proven by cardiac catheterization and angiography. In two cases anterior mitral cleft was demonstrated by 2-D echocardiography and/or operation. In two cases left coronary artery was originated from pulmonary artery. Eight cases out of ten presented symptoms indicating varying degrees of left ventricular failure. Apical systolic murmur was heard in all except one, who had only triple rhythm. The electrocardiograms revealed left ventricular hypertrophy in 8 cases and biventricular hypertrophy in 2 cases. Roentgenographically left atrial enlargement was observed in 4 cases. Left arterial and ventricular dimension were increased in 7 cases by echocardiography. In 5 cases of them, pulmonary artery wedge pressure or left atrial pressure was increased. One patient died in congestive heart failure at the age of 10 months and one patient died postoperatively. Other 4 patients have been managed with medication without difficulty.
Angiography ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Diagnosis ; Echocardiography ; Electrocardiography ; Heart Defects, Congenital ; Heart Failure ; Hemodynamics ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Incidence ; Mitral Valve Insufficiency* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Systolic Murmurs

No abstract available.
Cholangitis*