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

No abstract available.
Aneurysm* ; Heart Septal Defects, Ventricular*

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.
Aged* ; Dementia* ; Gyeongsangbuk-do* ; Humans ; Prevalence*

No abstract available.
Aged* ; Dementia* ; Gyeongsangbuk-do* ; Humans ; Prevalence*

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

The infantile polycystic kidney disease is rare fetal urinary tract anomaly. It is inherited with an autosomal recessive pattern and recurrence rate is 25%. The gene locus is on chromosome 6p. The pathogenesis of infantile polycystic kidney is the primary defect of the collecting ducts. The ultrasonographic finding of infantile polycystic kidney is oligohydramnios, bilaterally symmetrical enlarged kidneys with maintenance of their reinform shape. The differential diagnosis with adult polycystic kidney disease is important. The massive enlargement of the kidneys is rarely seen in adult polycystic kidney disease and the examination of the parents and other members of the family is helpful to confirm the adult polycystic kidney disease. If there is severe renal involvements, stillbirth or neonatal death secondary to pulmonary hypoplasia would be developed. If it were diagnosed before viability, termination of pregnancy is recommended. In a fetus at risk, diagnosed after viability, pregnancy termination is also recommended since this condition is uniformly fatal. We present a case of infantile polycystic kidney.
Diagnosis, Differential ; Female ; Fetus ; Humans ; Kidney ; Oligohydramnios ; Parents ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant ; Pregnancy ; Recurrence ; Stillbirth ; Urinary Tract