No abstract available.
Gaucher Disease*

Porencephaly is a rare congenital anomaly characterized by a cavitary hole extending from the cerebral mantle to the ventricle of the brain and covered by leptomeningeal membrane. It is now generally believed that the porencephaly represents a fetal or neonatal destructive process of the neural tissue. Authors experienced an autopsy case of bilateral porencephaly in a 35-day-old Korean male infant who died of sepsis. The brain weighed 150 gms and morphologically well formed except for two large symmetric defects at the fronto-parietal region. These defects were characterized by cleft-like shape, covering the entire cerebral mantle with resultant direct communication between ventricular cavity and leptomeninges. Thin leptomeningeal trabeculae were covering the cavity. There was a smooth transition between ependyma and roof of the defects. There are focal, microscopic cerebral infarct and abscess without connection with the cavitary lesion. The remaining central nervous system was unremarkable.
Infant ; Male ; Female ; Infant, Newborn ; Humans

In order to elucidate the biological effects of pulsating magnetic field in in vitro culture system we designed a pulsating magnetic apparatus using 120 Hertz, 24 Volt direct current. It can generate 63~225 Gauss in the experimental area of 90 mm petri dish, and has little thermal effect on the culture media in 37.5oC, 5% CO2. Human osteogenic sarcoma (HOS) cells were cultured in the pulsating magnetic field and the nuclear changes of cultured cells were observed routinely by hematoxylin staining, and apoptotic change was detected by ApopTag staining using both peroxidase and fluorescein labelings. Compared to the control group which formed well organized whorling pattern of HOS cell line in 3 days culture, the HOS cells cultured in the pulsating magnetic field for 12 hours or 24 hours grew irregularly and showed increased number of apoptotic cells. When the flow of pulsating magnetic field was interrupted by insertion of strong permanent magnetic bar (1000 Gauss, 5530 mm) beneath the petri dish during in vitro culture, the area of sparse pulsating magnetic field showed active proliferation and aggregation of HOS cells even in 24 hour exposure group. These data suggest that the pulsating magnetic field may play a role in inducing growth retardation and apoptosis of HOS cells. Furthermore, the hazardous effects of pulsating magnetic field can be lessened or nullified by the interruption of pulsating magnetic field with a strong permanent magnetic bar.
Apoptosis ; Cell Line* ; Cells, Cultured ; Culture Media ; Fluorescein ; Hematoxylin ; Humans* ; Magnetic Fields* ; Osteosarcoma* ; Peroxidase

No abstract available.
Cervix Uteri* ; Female ; Health Expenditures*

Tolosa-Hunt syndrome (THS) is a clinical syndrome of painful ophthalmoplegia associated with an idiopathic inflammation in the superior orbital fissure or cavernous sinus. We report a 61-year-old woman with recurrent THS. She had been treated with steroids for the painful ophthalmoplegia. After a symptom-free period, she developed similar symptoms. The postcontrast brain MRI showed increased signal in the cavernous sinus, which was also seen in the previous MRI.
Brain ; Cavernous Sinus ; Female ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Middle Aged ; Ophthalmoplegia ; Orbit ; Steroids ; Tolosa-Hunt Syndrome*

No abstract available.
Burkitt Lymphoma* ; Child* ; Humans

Primary cutaneous aspergillosis is rare. In this report we describe primary skin infection by Aspergillus in 9 children with leukemia. The skin lesion was characterized clinically by erythematous macule and papule associated with pain and itching, followed by a rapid progression to ulcer and central black eschars with raised erythematous border at the site of venipuncture, insertion of intravenous cannula, or where arm boards had been taped to extremities. Diagnosis was confirmed by skin biopsy and wound culture. Positive revealed in 6 patients, including A. glaucus in 2 cases, A. flavus in 1 case. Treatment consisted of temporary withdrawl of anticancer chemotherapy, intravenous amphotericin B, oral flucytosine, itraconazole and/or rifampin. One patient recovered completely without antifungal medication with resolution of leukopenia. Six of eight treated patients recovered. One patient discharged against medical advice, while cutaneous aspergillosis was improving. One patient died with persistent skin lesion and neutropenia. We conclude that primary cutaneous aspergillosis is increasingly recognized in association with intravenous cannula, intravenous puncture or prolonged contact with arm boards in immunocompromised patients, and that this serious disease can be treated successfully with appropriate management.
Amphotericin B ; Arm ; Aspergillosis* ; Aspergillus ; Biopsy ; Catheters ; Child* ; Diagnosis ; Drug Therapy ; Extremities ; Flucytosine ; Humans ; Immunocompromised Host ; Itraconazole ; Leukemia ; Leukopenia ; Neutropenia ; Phlebotomy ; Pruritus ; Punctures ; Rifampin ; Skin ; Ulcer ; Wounds and Injuries

PURPOSE: We measured anti-H. pylori IgG in Korean elementary school children living in Shinchon area of Seoul, Korea to evaluate the influence of environmental living standards on H. pylori infection. METHODS: IgG antibodies to H. pylori were measured in plasma using a commercial ELISA kit (GAP IgG Helicobacter pylori, Bio-Rad Laboratories Inc., Hercules, CA, USA). Information on environmental status such as place of birth, parental income, type of housing, number of persons in the household, parents' occupation, family history of peptic ulcer disease and gastric cancer was obtained. Statistical analysis was done by Chi-square and logistic regression test using SPSS 7.0(TM) for Windows. RESULTS: Study subjects consisted of 571 children, and the age distribution ranged from 6.0 to 13.6 years with a mean of 9.6+/-1.8 years. Male-to-female ratio was 1.1:1. The seropositive rates of H. pylori infection ranged from 10.4% in children aged 6 years to 30.9% in 12 year-old group, overall 16.8%. The prevalence of H. pylori infection progressively increased with age, but there was no significant difference in seropositive rates among children in different age groups (p=0.06). Seropositive rates of anti-H. pylori IgG on the basis of gender, place of birth, parental income, type of housing, parents' occupation, family history of peptic ulcer disease and gastric cancer showed no statistically significant difference. Interestingly, however, seropositive rate of anti-H. pylori IgG showed statistical significance in relation to number of persons in the household (p=0.003; Odds ratio 1.50 by logistic regression test). CONCLUSION: These results suggest that number of persons in the household is the most important factor among environmental living standards, and that risk of H. pylori infection increases by increment of 1.5 times as the number of persons in the household increases by one.
Age Distribution ; Antibodies ; Child* ; Enzyme-Linked Immunosorbent Assay ; Family Characteristics ; Helicobacter pylori* ; Helicobacter* ; Housing ; Humans ; Immunoglobulin G ; Korea ; Logistic Models ; Occupations ; Odds Ratio ; Parents ; Peptic Ulcer ; Plasma ; Prevalence ; Residence Characteristics ; Seoul ; Socioeconomic Factors* ; Stomach Neoplasms

Steroid withdrawal followed by interferon therapy is an alternative approach for treating chronic hepatitis B virus infection when there has been no therapeutic response to interferon alone. The effectiveness of steroid withdrawal followed by interferon therapy and factors predictive of the response were evaluated in 35 children with biopsy-proven chronic hepatitis B. Patients had received a 1-month course of prednisolone, 1 mg/kg per day orally, followed by a 2-week rest, and then were treated with interferon alpha 3 MU three times per week for 4-6 months. The serum aminotransferase values normalized in 80%, and negative seroconversion rates of HBeAg and HBV-DNA were 69% and 66%. The good response rate was associated with a pretreatment HBV-DNA level lower than 100 pg/ml and a posttreatment ALT level more than 200 IU/L. Normalization of ALT values usually took 5 months, and the clearance of HBV-DNA and HBeAg took 7.8 and 6.7 months, respectively. These results suggest that steroid withdrawal followed by interferon therapy is useful in the treatment of chronic hepatitis B in children, and that a good response rate can be expected in children with lower pretreatment HBV-DNA levels (< 100 pg/ml).
Adolescence ; Alanine Transaminase/blood ; Child ; Child, Preschool ; DNA, Viral/analysis ; Female ; Hepatitis B, Chronic/virology ; Hepatitis B, Chronic/therapy* ; Hepatitis B, Chronic/pathology ; Human ; Infant ; Interferon Alfa-2a/therapeutic use* ; Male ; Prednisolone/therapeutic use*

PURPOSE: Prenatal diagnosis of congenital heart disease has been made by fetal echocardiography and its clinical impact on the outcome of CHD cases has been analysed. METHODS: A prospective study was performed for the fetal diagnosis of CHD for the standard risk pregnancy, confirmed postnatally or at second study and/or at autopsy and/or follow up at CHA hospital in 2001. Incidence of CHD has been calculated and compared to the incidence of CHD of the prescreening period(1992.3-1995.2). RESULTS: There were 66 cases of CHD prenatally during the study period. CHD consisted of 7 cases of ventricular septal defects(VSD), 7 cases of tetralogy of Fallot(TOF), 5 cases of heterotaxy, 5 cases of double outlet right ventricle(DORV), 5 cases of severe pulmonary stenosis(PS), 3 cases of hypoplastic left heart syndrome(HLHS), 3 cases of coarctation, 3 cases of critical aortic stenosis(AS), 3 cases of pulmonary atresia with intact ventricular septum(PA IVS). Among 66 fetal CHD cases, 30 cases of CHD has been terminated, 1 case died in utero and 23 cases has been delivered at cardiac center through planned delivery. The most common factors of termination were extracardiac and chromosomal anomaly. 17 cases of false negative diagnosis were small VSD and mild PS that couldn't be diagnosed in fetal stage. There was no false positive diagnosis. There were 30 cases of CHDs born in 2001 at CHA hospital. 28 cases were diagnosed postnatally. Among 30 cases of CHDs, there were 18 cases of VSD(17 small VSD), 6 cases of mild PS, 4 ASD, 1 PDA. Incidence rate of CHD in 2001 and period 1992.3-1995.2 were 5.95 and 10.15/1,000 livebirth respectively. CONCLUSION: The data suggested that all of the significant CHDs could be diagnosed prenatally accurately by fetal echocardiography. And the incidence rate of CHD has been decreased by 41.4%. And the most of the complex CHD has been transferred to the cardiac center for planned delivery or has been terminated. The rate of termination was 45.5%.
Autopsy ; Diagnosis* ; Echocardiography* ; Follow-Up Studies ; Heart ; Heart Defects, Congenital* ; Incidence ; Pregnancy ; Prenatal Diagnosis ; Prospective Studies ; Pulmonary Atresia