OBJECTIVE: Moyamoya disease (MMD) is an uncommon cerebrovascular disorder, characterized by progressive occlusion at the terminal portion of the internal carotid artery. Incidence of the disease is high in East Asia and familial MMD accounts for about 15% of the disease. Although the pathogenesis is unknown, association of HLA class I or II alleles with MMD has been reported with conflicting results. We investigated whether there is a difference in HLA class II association between familial and non-familial forms of the disease. METHODS: A total of 70 Korean children with MMD, including 16 familial cases (10 probands), and 207 healthy controls were studied. Among familial cases, only 10 probands were used for the HLA frequency analysis. High resolution HLA-DRB1 and DQB1 genotyping was performed using polymerase chain reaction (PCR)-sequence specific oligonucleotide hybridization and PCR-single strand conformation polymorphism methods. RESULTS: The phenotype frequencies of HLA-DRB1*1302 (70.0%) and DQB1*0609 (40.0%) were significantly increased in familial MMD compared to both controls [vs. 15.5%, corrected p (pc) = 0.008, odds ratio (OR) = 12.76; vs. 4.3%, pc = 0.02, OR = 14.67] and non-familial MMD patients (vs. 14.8%, pc = 0.02, OR = 13.42; vs. 1.9%, pc = 0.02, OR = 35.33). The frequencies of DRB1 and DQB1 alleles in non-familial MMD patients were not significantly different from those in controls. CONCLUSION: Our findings suggest that the genetic polymorphism of HLA class II genes or other closely linked disease relevant gene(s) could be a genetic predisposing factor for familial MMD.
Alleles ; Carotid Artery, Internal ; Cerebrovascular Disorders ; Child ; Chimera ; Far East ; Genes, MHC Class II ; HLA-DQ Antigens ; HLA-DR Antigens ; HLA-DRB1 Chains ; Humans ; Incidence ; Moyamoya Disease ; Odds Ratio ; Phenotype ; Polymerase Chain Reaction ; Polymorphism, Genetic

No abstract available.
Epidemiology*

No abstract available.
Emergencies* ; Emergency Service, Hospital* ; Humans ; Statistics as Topic*

No abstract available.
Rheumatic Fever*

Torsion of a mucocele of the vermiform appendix is an extremely rare condition and also a rare cause of an acute abdomen with a clinical presentation that is indistinguishable from acute appendicitis, and thus, the condition is diagnosed during operation. Here, the authors describe the case of a 78-year-old female, who presented with intermittent abdominal pain. The appendix had a pelvic position and the torsion was counterclockwise. In addition, the torsion was associated with mucocele of the appendix, which was considered a secondary factor of torsion. Appendectomy and drainage were performed.
Abdomen, Acute ; Abdominal Pain ; Aged ; Appendectomy ; Appendicitis ; Appendix ; Drainage ; Female ; Humans ; Mucocele ; Torsion Abnormality

No abstract available.
Colorectal Neoplasms* ; Electrophoresis* ; Genes, ras* ; Humans* ; Point Mutation* ; Polymerase Chain Reaction*

OBJECTIVES: The aim of this study was to estimate the burden of disease through an analysis of Years of Life Lost due to premature deaths, one component of the Disability-Adjusted Life Years (DALY). In addition, the cause of death statistics were adjusted to improve validity, and the results were compared with those of the Global Burden of Disease (GBD). METHODS: In closely following the approach taken in the original GBD study, most of the explicit assumptions and the value judgments were not changed. However, the statistics for some problematic concerns such as deaths of infants or those due to senility, were adjusted. Deaths, standard expected years of life lost (SEYLL), and potential years of life lost (PYLL) were computed using vital registration data compiled by the National Statistical Office. RESULTS: The burden for males is 1.8 and 2.3 times higher than that for females, according to SEYLL and PYLL, respectively. The proportions of deaths due to Group I, II, and III causes are 5.4%, 80.4%, and 14.3%, respectively, for PYLL, but in a major shift from Group II to III they are 6.3%, 66.2%, and 27.5%, respectively, for SEYLL. The proportion of Group III causes in Korea, 27.5%, is extremely high when compared to 10.1% for the world, 7.6% for developed countries, and 10.7% for developing countries. CONCLUSIONS: Estimation results showed that the total burden due to premature deaths is smaller than that for the entire world but larger than that for developed countries. The disease structure of Korea has changed to resemble that of developed countries. Also, an overly large portion of the total burden in Korea stems from injuries arising from car accidents.
Cause of Death ; Cost of Illness ; Developed Countries ; Developing Countries ; Female ; Health Transition ; Humans ; Infant ; Judgment ; Korea* ; Life Expectancy ; Male ; Mortality, Premature*

Percutaneous cannulatien has become an accepted technique for monitoring central venous pressure and pulmonary wedge pressure via a Swan Gans catheter. The technique is not without hazard. Complications include thrombophlebitis, infection and hydrothorax. These are case reports demonstrating the complications of hydrothorax and broken catehters from central venous catheterization.
Catheterization, Central Venous* ; Catheters* ; Central Venous Catheters* ; Central Venous Pressure ; Hydrothorax* ; Pulmonary Wedge Pressure ; Thrombophlebitis

This is the clinical statistics concerning the admissions in the Pediatric Department of the Chosun University Hospital during the past 5 years from Jan. 1975 to Dec. 1979. All the patients were classified by y Classification of Disease by W.H.O. The Obtained results are as follows: 1. Total number of admission during 5 year period were 3249 cases, of which 2121 cases (65.28%) were male, 1128 cases (34.72%) were female and male to female ratio was 1.88:1. 2. The number of patients were increased every year gradually. 3. On age group, Infancy age group was the most frequent group, 714 cases (22%), followed by School age group, 6679 cases (20.9%) and Preschool age group, 679 cases (20. 9% ) in the order of frequency. 4. On monthly distribution, there was the more admission in the September and the Autumn on season, 5. Infectious and Parasitic diseases were the most frequent, 840 cases(25. 9%), followed by Neonatal disease, 573 cases (17.6%) and Respiratory disease, 476 cases (14.7%) 6. Ten major leadil1g causes of hospitalization were Diarrheal disease, 323 cases (9. 9%) ,prematurity, pneumonia, common cold, epidemic encephalitis, hyperbilirubinemia, epilepsy, glomerulonephritis, hepatitis and typhoid fever in the order frequency.
Classification ; Common Cold ; Encephalitis, Arbovirus ; Epilepsy ; Female ; Glomerulonephritis ; Hepatitis ; Hospitalization ; Humans ; Hyperbilirubinemia ; Inpatients* ; Male ; Parasitic Diseases ; Pneumonia ; Seasons ; Typhoid Fever

With the availability of the method of analysis of serum protein using minute amounts of material, it was felt desirable to understand the protein metabolism and physiologic function in the body. The present study was undertaken to clarify the serum albumin, globulin and total protein at term to demonstrate the normal concentration and correlation between the 30 mother and newborn infant pairs. Serum albumin, globulin and total protein were determined by the Biuret method with pooled human serum. The A/G ration was calculated by formula of A/G. The following result were obstained. 1) In comparing the newborn infants of nonanemic mothers a albumin and total protein concentrations were higher and globlin concentrations decreased in the anemic mothers. 2) In comparing the nonanemic mothers and anemic mothers the mean albumin concentrations were nearly equal but globulin and total protein were slightly increased in the nonanemic mothers. 3) The mean serum albumin(of maternal and umbilical cord blood) was 3. 8+/-0. 35 gm/100 ml and 3. 8+/-0. 49 gm/100 ml respectively. 4) The mean serum globulin of mate. nal and umbilical cord blood was 2. 7+/-0. 41 gm/100 ml and 2. 32+/-0. 47 gm/100 ml respectively. The correlation of the globulin status between mot-hers and their newborn infants was not significant(r=0. 32). 5) The m-an serum total protein of maternal and umbilical cord blood was 6. 59+/-0. 59 gm/100 ml and 6. 02+/-0.57gm/100ml respectively.
Biuret ; Fetal Blood ; Humans ; Infant, Newborn* ; Metabolism ; Mothers* ; Serum Albumin* ; Umbilical Cord