Schizencephaly is a congenital brain anomaly, a disorder of cell migration characterized by gray matter-lined clefts, which is caused by faulty migration of the subependymal neuroblasts. The cleft may be closed (Type I) or open by a cavity communicating with the lateral ventricle (Type II). We identified the characteristic features of schizencephaly on CTs in one patient and on MRIs in 15 patients. Age at detection ranged from 16 months to 37 years. Three patients had bilateral clefts and 13 patients had unilateral cleft. Twelve patients had closed lip cleft whlle four patients had open lip cleft. Small sized unilateral closed cleft, the most comrnon type, was found in ten patients. Three patients had multiple clefts. Clinically these patients presented with seizures in 15 patients, motor disturbance in six patients, speech impairment in five patients, developemental delay in four patients, and mental retardation in three patients. The presence and severity of these symptoms correlated with the size and location of the lesions. Associated cerebral anomalies included ventricular dilatation in seen patients, agenesis of septum pallucidum in seven patients, other neuronal migration disorder in four patients, agenesis or dysgenesis of corpus callosum in three patients, septo-optic dysplasia in two patients, abnormal extension of svlvian fissure in one patient, and arteriovenous malformation in one patient.
Arteriovenous Malformations ; Brain ; Cell Movement ; Corpus Callosum ; Dilatation ; Humans ; Intellectual Disability ; Lateral Ventricles ; Lip ; Magnetic Resonance Imaging ; Malformations of Cortical Development* ; Neuronal Migration Disorders ; Seizures ; Septo-Optic Dysplasia

Neuronal migration disorder is a rare group of congenital malfomations of the brain caused by insults to migrating neuroblasts during the six to fifteen gestational weeks. We identified 36 neuronal migration disorders on CTs in two patients and on MRIs in 34 patients and analyzed their characteristic radiologic, clinical, and EEG findings. These 36 patients with neuronal migration disorders consisted of 18 with schizencephaly, eight with pachygyria, five with heterotopias, three with lissencephaly, and two with polymicrogyria. Patient ranged in age from 6 months to 37 years old and mean age was 18.2 years old. Associated cerebral anomalies included ventricular dilatation in 13 patients, agenesis of septum pallucidum and hypoplasia of corpus callosum in nine patients. Lissencephaly was associated with other cerebral anomalies most frequently and all of them had ventricular dilatation and hypoplasia of corpus callosum. Only one patient with pachygyria had ventricular dilatation. Clinically, these patients presented with seizures in 91.7%, speech impairment in 33.3%, abnormal motor function in 30.5%, developmental delay in 27.8%, mental retardation in 25%. Patients with large or medium size of neuronal migration disorders had significantly more severe developmental delay(p=0.001), mental retardation (p=0.004) and speech impairment (p=0.01) than those with small size. Abnormal motor dysfunctions were not significantly associated with lesion size statistically. Seizures did not correlate with lesion size.
Adult ; Brain ; Corpus Callosum ; Dilatation ; Electroencephalography ; Humans ; Intellectual Disability ; Lissencephaly ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Neuronal Migration Disorders* ; Neurons* ; Seizures

BACKGROUND: Paraquat causes severe tissue toxicity when ingested, but has no effective treatment modality. We have shown that high dose vitamin C has effective antioxidant activities against the paraquat intoxication in a previous animal experiment. This study was designed to evaluate the effect of antioxidant therapy with high dose vitamin C and vitamin E in human cases of paraquat intoxication. METHODS: From August 1999 to August 2001, 19 paraquat intoxication patients who visited the emergency department of the Seoul National University Hospital and the Kyounghee University Hospital were enrolled to this study. They were devided into two groups, a control group(9 patients) and a study group(10 patients). The control group received only conservative managements including gastro-intestinal decontaminati-on. The study group received conservative managements plus the antioxidant therapy which was composed of vitamin C 24 gm/day intravenously and 20 gm/day orally, and vitamin E 1.6 gm/day orally. RESULTS: In the study group, 5 of 10 patients(50%) survived, but all patients of the control group died(p=0.003). There were no significant differences in age, sex, and usage of gastric lavage and activated charcoal between the two groups. Difference in ingested amount of paraquat between the two groups could not be analyzed due to the inexact and subjective measuring methods based on patients'histories. CONCLUSION: Antioxidant therapy with high dose vitamin C and vitamin E is effective in improving survival rate in paraquat intoxicated patients.
Animal Experimentation ; Ascorbic Acid ; Charcoal ; Emergency Service, Hospital ; Gastric Lavage ; Humans ; Paraquat* ; Seoul ; Survival Rate ; Vitamin E ; Vitamins

Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Adult* ; Biopsy ; Cardiomyopathies ; Diagnosis ; Echocardiography ; Electrocardiography ; Embryonic Development ; Female ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Male ; Myocardium* ; Pregnancy ; Syncope ; Tachycardia, Ventricular* ; Young Adult

Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Adult* ; Biopsy ; Cardiomyopathies ; Diagnosis ; Echocardiography ; Electrocardiography ; Embryonic Development ; Female ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Male ; Myocardium* ; Pregnancy ; Syncope ; Tachycardia, Ventricular* ; Young Adult

The National Evidence-based Collaborating Agency (NECA) holds the NECA Round-table Conference that not only disseminates objective and systematic information on topics of social concern in public health care but also organizes discussions on core issues under dispute in the literature through panels composed of multidisciplinary experts. Accordingly, the Round-table Conference was composed of multidisciplinary experts including medical specialists in the areas of psychiatry and preventive medicine, psychiatric and mental health nursing, psychologists, social welfare experts, consultation experts, religious leaders, and government officials from the Ministry of Education, Science and Technology, and Ministry of Health and Welfare. The Round-table Conference, tasked with analysis of the actual status and causes of, and search for solutions for suicide in adolescents, has reached consensus on the current status, trend, risk factors and prevention factors, problems and issues in prevention and coping strategies, effective prevention and coping strategies and areas of research needed for the future. The Round-table Conference commented on the actual status and gravity of suicides in adolescents, and came to the agreement that mental health issues including stress from interpersonal relationships and depression are the key risk factors of suicide. It was further agreed that problems in the measures being implemented for each of the areas include lack of manpower and funding, and inadequate organic association and cooperation among relevant institutions. They also agreed that development of a government-initiated suicide prevention program for adolescents, association among relevant experts, and development, and management of practical guidelines that are of broad and practical use are important. Furthermore, the panels were in agreement that the mass media must comply with the recommended level of coverage in reporting of suicide as adolescents are greatly influenced by provocative mass media reports due to their strong impulsive dispositions.
Adenosine-5'-(N-ethylcarboxamide) ; Adolescent ; Cause of Death ; Consensus ; Depression ; Dissent and Disputes ; Financial Management ; Gravitation ; Humans ; Mass Media ; Mental Health ; Occupational Groups ; Porphyrins ; Preventive Medicine ; Public Health ; Risk Factors ; Social Welfare ; Specialization ; Suicide

Primary inflammatory myofibroblastic tumors (IMTs) of the breast are uncommon and metastasis of IMTs is extremely rare. To date, the natural course of this disease is not fully understood. Although patients with IMTs should undergo regular follow-up after complete surgical resection of the tumor, the appropriate interval and method of follow-up are unclear. We report the case of a patient with an IMT of the breast that metastasized 2 years after complete surgical resection. This unusual case emphasizes the importance of preoperative examinations to determine whether the IMT has atypical features that should guide the interval and method of follow-up.
Breast Neoplasms ; Breast* ; Follow-Up Studies ; Granuloma ; Humans ; Myofibroblasts* ; Neoplasm Metastasis* ; Plasma Cells

BACKGROUND: Proficiency testing is part of a total quality management program that provides objective evidence of clinical laboratory testing competence for customers, accrediting bodies, and regulatory agencies. Performing alternative assessment procedures for clinical tests, without proficiency testing, is recommended by Clinical and Laboratory Standards Institute (CLSI) guideline. In our study, an alternative assessment procedure was performed for blood bank tests that do not have an external proficiency program. METHODS: The laboratory for development and an evaluation center, supervised the program. Proficiency testing by seven institutions was performed 3 times at 6 month intervals by evaluating isoagglutinin and anti-D titers, and Weak D, Rh C and E typing, using ID-Internal Quality Control (Bio-Rad Laboratories) kits. RESULTS: Isoagglutinin and anti-D titer results were within one fold dilution range for all seven participating institutions, and Weak D, Rh C and E typing results all demonstrated identical antigenic reference patterns. CONCLUSION: An alternative assessment procedure was successfully performed without a proficiency testing program. Commercially manufactured reference materials could be an alternative method to support commutable, external, proficiency testing program.
Blood Banks ; Isoantibodies ; Mental Competency ; Quality Control ; Total Quality Management

PURPOSE: To compare immunogenicity and reactogenicity of a combined diphtheria-tetanus-acellular pertussis-inactivated poliovirus vaccine (DTPa-IPV, Infanrix(TM) IPV, GlaxoSmithKline Biologicals) with co-administration of commercially available DTPa and IPV vaccines at separate injection sites (DTPa+IPV). METHODS: A total of 458 infants aged 8-12 weeks were randomized to receive three-dose primary vaccination at 2, 4 and 6 months with DTPa-IPV or DTPa+IPV. Blood samples were collected pre and post vaccination for measurement of immune responses. Reactogenicity was assessed following each dose using diary cards. RESULTS: One month post-dose 3, seroprotection rates for anti-diphtheria, anti-tetanus and anti-poliovirus types 1, 2 and 3 were > or =99.5% and vaccine response rates to pertussis antigens were at least 98.6% in both DTPa-IPV and DTPa + IPV groups. Non-inferiority between the groups was demonstrated based on pre-defined statistical criteria. Incidences of both local and systemic symptoms were within the same range across both groups with grade 3 symptoms reported following no more than 4.3% of DTPa-IPV doses and 4.5% of DTPa + IPV doses. Two serious adverse events (both pyrexia) after DTPa-IPV administration were considered vaccine-related. Both infants recovered fully. CONCLUSION: Combined DTPa-IPV vaccine was immunogenic and well tolerated when used as a three-dose primary vaccination course in Korean infants. DTPa-IPV could be incorporated into the Korean vaccination schedule, reducing the number of injections required to complete primary immunization.
Aged ; Appointments and Schedules ; Humans ; Immunization ; Incidence ; Infant ; Pentetic Acid ; Poliovirus ; Vaccination ; Vaccines ; Whooping Cough

BACKGROUND: During the 2009 influenza pandemic in Korea, school-age children were mostly attacked by the novel influenza A virus (H1N1). Nevertheless, there is limited information on the cumulative incidence of the 2009 influenza pandemic among school populations. The aim of this study was to estimate the incidence of 2009 H1N1 influenza and influenza-like illness (ILI), and characterize the epidemiology among school-based populations in Korea. MATERIALS AND METHODS: This study analyzed the data collected by the daily school influenza reporting system for laboratory-confirmed influenza (H1N1 2009 pdm) and ILI in elementary, middle and high schools in Korea during the period, July 1 to December 20, 2009. RESULTS: Between July 1 and December 20, 2009, a total of 525,668 cases of H1N1 2009 pdm were reported from the schools analyzed. The cumulative incidence of H1N1 2009 pdm was highest in elementary school children between 8 and 13 years of age (11,058 per 100,000 population), followed by middle school students between 14 and 16 years of age (10,080 per 100,000), and high school students between 17 and 19 years of age (8,886 per 100,000). With the alignment of the epidemic curves by the three levels of school education, the age group-specific differences in the timing of the infection became apparent. The infections peaked 1 week earlier in both middle and high school students (November 3, 2009) than in elementary school children (November 10, 2009). The infections occurred an average of 7.3 [95% confidence interval(CI): 7.2-7.4] and 3.4 (95% CI: 3.3-3.5) days earlier in the middle and high school students, respectively, than elementary school children (P<0.001). The overall trend of the epidemic waves from the school reporting system data was similar to that of the national surveillance date from the Korea Centers for Disease Control and Prevention. CONCLUSIONS: This study suggests that during the 2009 pandemic, one student in every 10 was infected with H1N1 2009 pdm, which is double that estimated from the national surveillance data. In addition, middle and high school students were important drivers of H1N1 2009 pdm transmission in 2009. During the 2009 influenza pandemic, the daily school reporting system provided valuable information for estimating the incidence as well as the epidemiological characteristics in school-based populations.
Centers for Disease Control and Prevention (U.S.) ; Child ; Humans ; Incidence ; Influenza A virus ; Influenza, Human ; Korea ; Pandemics