Head injury in the youngest age group is distinct from that occurring in older children or adults because of differences in mechanisms, injury thresholds, and the frequency with which the question of child abuse is encountered. "Shaken baby syndrome" has results in intracranial and introcular hemorrhages with no evidence of external trauma. The cause of these injuries is vigorous shaking of an infant being held by the chest, shoulders, or extremities. Severe head injuries commonly diagnosed as shaking injuries require impact to occur and that shaking alone in an otherwise normal baby is unlikely to cause the shaken baby syndrome. "Shaken impact syndrome" has the advantage of being more inclusive of verifiable impact mechanisms and of reflecting the extreme forces that appear to be necessary to produce these often devastating injuries. All clinicians must recognize the wide spectrum of injuries in child abuse to ultimate protect the victim or other children in an at-risk situation. And physicians play an important role in diagnosis, management and prevention of child abuse and shaken baby syndrome.
Adult ; Child ; Child Abuse ; Craniocerebral Trauma ; Diagnosis ; Extremities ; Hemorrhage ; Humans ; Infant ; Shaken Baby Syndrome* ; Shoulder ; Thorax

The complement system is known to be involved in the pathogenesis of the skin lesions in pernphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita, and systemic lupus erythematosus. Authors examined the skin specimens of each disease cases, who did not show any evidence of complement deficiency, to determine the deposition of complement components(C4, C3, Chb-9) and their inhibitors(C4bp, Factor H, S-protein) by modified direct immunofluorescence. We also looked at the staining pattern and localization, for further insights of their pathobiologic contributions in each disease. The findings of deposits of complement components up to C9, as well as inhibitor proteins at the primary histopathologic sites, in the majority of those cases, may indicate that the complement system, to certain extent, involves the inflamrnatory reactions in these diseases. The co-localization of C5b-9 and S-protein could be regarded as the consequence of in situ formation of SC5b-9 complexs or as the result of non-lytic adsorbed complexes of fluid phase SC5b-9. The pathologic role of the complement seems to depend mostly on the complement-fixing biologic property and the amount of the tissue bound immune complexes, which are often heterogeneous to different diseases and among different patients.
Antigen-Antibody Complex ; Complement Factor H ; Complement Membrane Attack Complex ; Complement System Proteins* ; Dermatitis Herpetiformis ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Direct ; Humans ; Lupus Erythematosus, Systemic ; Pemphigoid, Bullous ; Skin ; Skin Diseases*

Pemphgus vulgaris (PV), Bullus pemphigoid (BP), and Epidermolysis bullous acqusita (EBA) are autoimmune bullous dermatoses, characterized by circulating IgG autoantibodies. These antibodies react with antigens located at the intercellular substance (ICS) of epidermis, basement membrane zone (BMZ), and subepidermal anchoring fibril zone (AFZ), respectively. The subclass distribution of IgG autoantibodies, and the properties and degrees of complement fixing activities of these autoantibodies in each of the above diseases have not been well understood. Indirect immunofluorescence and in vitro complement stainings were performed for the titration of subclasses of IgG antibodies and for the immunofluorescence staining reactivities of complement components C3, C4, C5b-9, H, C4bp, and S. Each serum specimen from five cases of PV, five cases of BP. and three cases of EBA was tested. The findings of multistep technique with monoclonal and polyclonal antibodies are as follows : All four subclasses of IgG antibodies were identified at the antigenic sites in these group, however there were some differences in the antibodies titers. In PV and BP the dominant subclass of highest antibody titer was IgG1 and/or IgG4. In EBA only IgG4 was dominant in all three cases. The results of complement component stainings, in most of the cases of PV, showed positive for C3 and C4 but were negative for the other components or inhibitor proteins at the ICS of epidermis. In BP most of the cases revealed positive staining reactivities at the BMZ for C3, C4, C5b-9, H, and C4bp-9 with no staining reactivities for the inhibitor proteins No significant relevancy was found between the titers of complement fixing IgG subclasses and the numbers of positive complement staining reactivities for complement components. The results suggest that the complement system may contribute more strongly to the formation of bullous lesions in BP and EBA than in PV.
Antibodies ; Autoantibodies ; Basement Membrane ; Blister ; Complement Membrane Attack Complex ; Complement System Proteins ; Epidermis ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Immunoglobulin G ; Pemphigoid, Bullous* ; Pemphigus* ; Skin Diseases, Vesiculobullous

Revision of the post-graduate medical education (PGME) system, which has been almost fixed since the introduction of the intern-resident training system in Korea more than 50 years ago, is particularly demanding because of changes in the contents of basic medical education and the patient-doctor relationship, division into sub-specialties, the introduction of fellowship training, changing patterns in doctors' work as information technology advanced, and the modifications to the PGME system in the advanced countries. The internship is not an effective course for PGME. The graduates of medical colleges or medical schools can practice by himself or herself without a PGME course. The durations of PGME for various subspecialties are almost the same: one year of internship and four years of residency. The working conditions of trainees are not adequate for proper education and patient safety. Current internships should be merged into residency by a straight internship. PGME should be mandatory for those who want to practice after graduation from medical colleges or medical school without resident training. The duration of PGME for each subspecialty should be newly adjusted. The working conditions of trainees must be improved. Even though this report suggests future directions for PGME on a broad scale, fine adjustment and design of further details should follow. This report has taken comments and opinions from the medical societies into consideration. Nonetheless, it may need further discussion among the stakeholders of PGME including trainees in order to minimize misunderstanding and 'trial and error' during the revision process.
Education, Medical ; Fellowships and Scholarships ; Internship and Residency ; Korea ; Patient Safety ; Propylene Glycols ; Schools, Medical ; Societies, Medical

A doctor has to establish his or her own philosophy of medical practice for the management of congenitally disabled patients. To help the senior medical students to put their ideas on the management of such patients in shape, a question which asked them to describe their views on the medical, social, ethical aspects of the management was included in the examination for the 225 senior students of Seoul National University College of Medicine in 1992. The responses were summarized. The dismal prognosis of some malformations, considerable cases of abandonment by the misconception of parents and doctors, the conflict among the family members caused by the economic burden and social handicaps, the relative lack of support and the psychological and vacational discrimination by the society were among the prevalent problem. Of the 225 students, 131(58%) insisted that 'all' the congenitally disabled patients be provided with the best quality of treatment while 26(12%) argued against the idea. Fifteen students(7%) recommended to have a certain period of 'natural selection' and 14(6%) denied and warned against the doctor's role as a decision maker. Eleven students(5%) stated that the aggressive treatment should be confined to the patients who were selected by the medical criteria which supports the abandonment of poorly disabled patients. Three(1%) emphasized the 'prevention' as the ultimate goal. Twenty five(11%) showed reponses which lacked their own ideas or were inappropriate. Representative or unique statements are quoted. Though the ideas of students were not so contradictory to one another, the views were diverse as much as the variety of problem in the management of congenitally disabled patients. The authors believe it is worthwhile to ask medical students to think about the medically and ethically difficult situations before he or she becomes a doctor.
Discrimination (Psychology) ; Humans ; Parents ; Philosophy ; Prognosis ; Seoul ; Students, Medical*

To figure out the occurrence pattern of pediatric brain tumors in the aspects of age, sex, location and histopathological diagnosis, 365 cases of pediatric(age less than 16 year) brain tumor which were operated upon at the Department of Neurosurgery, Seoul National University Hospital from January 1959 to June 1993, were reviewed. Only the tumors, of which the pathological specimens were taken, were included. Tumors of bone origin or purely extradural mass, non-tumorous cystic lesions and vascular malformations were excluded. The mean age of 365 cases was 8.1 years and the sex ratio(M : F) was 1.3 : 1. Supratentorial tumors(56.2%) were more prevalent than infratentorial tumors(43.4%). Neuroepithelial tumors comprised of 64.4% of the 365 brain tumors. Forth three percent of the neuroepithelial tumors were embryonal origin such as medulloblastoma(MBL), primitive neuroectodermal tumor(PNET) and ependymoblastoma. Pathological common tumors were astrocytic tumor(22.7%), MBL(20.0%), craniopharyngioma(13.4%), germ cell tumor(GCT, 8.8%), PNET(6.6%), and ependymal tumor(6.3%) in the order of decreasing frequency. PNET and ependymal tumors were frequent in early childhood while pituitary adenoma and non-teratomatous GCT were common in older children. GCT, PNET and ependymoma were prevalent in male. The relative incidences of GCT, PNET, brain stem glioma, oligodendroglial tumor, pituitary adenoma and neuronal tumor increased in MRI era. In contrast, those of astrocytic tumor, MBL and ependymal tumor decreased.
Adenoma ; Brain Neoplasms* ; Brain Stem ; Brain* ; Child ; Diagnosis* ; Ependymoma ; Germ Cells ; Glioma ; Humans ; Incidence ; Magnetic Resonance Imaging ; Male ; Neoplasms, Neuroepithelial ; Neural Plate ; Neuroectodermal Tumors, Primitive ; Neurons ; Neurosurgery ; Pituitary Neoplasms ; Seoul ; Vascular Malformations

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.

No abstract available.

Ependymoma is the most common spinal intramedullary tumor in adults. The authors reviewed clinical data of 31 patients with spinal ependymoma who underwent operations between 1979 and 1996. The ages of the patients ranged from 15 to 62 years with a mean of 36.9. We analyzed clinical manifestations, radiologic findings, extents of surgical removal, histologic subtypes and follow-up results. Most patients were presented with sensory symptoms as initial symptoms which had lasted for 36.5 months on the average. The most frequent location was conus region(10 cases, 32%) followed by cervical, thoracic and cervico-thoracic spinal level. All cases were divided into two groups histologically, 12 myxopapillary subtypes and 19 non-myxopapillary subtypes. Operative results were dependent on the locations and the histologic subtypes of the tumor. Total removal was achieved in 4 cases out of 10 cases with masses around the conus and in 19 cases out of 21 cases with masses at other regions(p=0.003). Tumors were totally removed in 97% of 19 non-myxopapillary subtypes, but in 42% of 12 myxopapillary subtypes(p=0.001). From the follow-up data, we found that mean progression free interval was 83 months and 5 year progression free rate was 70%. Extent of removal was the only significant prognostic factor on multivariate analysis. Other factors such as tumor location, histologic subtype and radiation therapy were not significant. Disease progression was noted in 2 cases out of 23 cases of total removal group, but in 4 cases out of 8 cases of incomplete removal group (p=0.008). Postoperative radiation therapy was done in 4 cases in incomplete removal group and tumor regrowth was noted more frequently in non-radiation group than in radiation group without statistic significance. We concluded that disease progression can be determined by the extent of removal which is related to the tumor location and histologic subtypes.
Adult* ; Conus Snail ; Disease Progression ; Ependymoma* ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Prognosis