Cortical dysplasia(CD) represents a spectrum of neuropathologic changes reflecting a derangement of the normal process of neocortical development. We have presented 32 patients who underwent cortical recectiom for intractable seizures and demonstrated the neuropathologic features, which could be explained by a disturbance in the process of neural development in the farm. It could be characterized by light microscopic features: cortical laminar disorganization, neurons in the molecular layer, subpial re=ants of granule calls, remnants of marginal glioneuronal heterotopia, neuronal heterotopia in the white matter, polymicrogyria, neuronal cytomegaly and balloon cell change. Even though cortical dyslamimtion was the consistent finding of all the cases, the neuronal cytomegaly and balloon cell change were diagnostic hallmarks in the study. The cytomegatic neurons were strongly reactive to silver impregration and to immunohistochemical marrkers of neurons, such as neurofilament protein (NF, 68 and 200 kDa) and neuron-specific enolase(NSE). They showed hypertrophic endoplmmic reticul= and increased number of mitochondria in their cytoplasm and incomplete synapses in electron microscopic study. The balloon cells were positively stained by glial fibrillary acidic protein, NSE and vimentin and were filled with intermediate filaments in their cytoplasm. These results indicated that both cytomegalic neurons and balloon cells are produced by faulty cell differentiation involving neuroblast in the former, and both neuronal and glial stem cell lines in the latter.
Cell Differentiation ; Cytoplasm ; Glial Fibrillary Acidic Protein ; Humans ; Intermediate Filaments ; Malformations of Cortical Development* ; Mitochondria ; Neurons ; Seizures ; Silver ; Stem Cells ; Synapses ; Vimentin

Deveolopmental coax vara represents coax vara not present at birth but rather developing in early childhood, showing a progressive deterioration in the proximal femoral neck-shaft angle during growth. In order to determine the factors that could affect the results of corrective osteotomy, we evaluated the results of 15 developmental coax vara in 12 patients who had been treated with the femoral osteotomy at the Department of Pediatric Orthopedic Surgery, Seoul National University Children's Hospital, from February 1983 to March 1993. Of the 12 patients, there were 6 boys and 6 girls. Three patients had bilateral operations, 5 patients on the right, and the remaining 4 patients on the left. Average at the onset of symptoms was 4 years plus 5 months(range; from 1 year to 7 years plus 2 months), and average age at the tome of operation was 6 years plus 3 months(range; from 2 years plus 4 months to 10 years). We could obtain the following results: 1. Average post-operative loss of neck-shaft angle was 5% in the cases in which post-operative neck-shaft angle was converted more than 130 degrees, and was 8% in the cases in which post-operative neck-shaft angle was converted less than 130 degrees. 2. Loss of neck-shaft angle was higher during the first post-operative period, and was higher in cases in which the triangular osseous defect was persistent post-operatively. There was no correlation between the post-operative neck-shaft angle and disappearance of triangular osseous defect. 3. The femoral anteversion was converted 8.7 degrees to 27.2 postoperatively. 4. The premature arrest of the capital femoral physis was higher in cases in which the neck-shaft angle was less than 130 degrees postoperatively. 5. Leg length discrepancy, which was 2.1 cm preoperatively, did not change in cases in which the neck-shaft angle was more than 130 degrees postoperatively. However, it was converted to 3.3 cm in cases in which the neck-shaft angle was less than 130 postoperatively. 6. we could observe the femoral head deformity in 6 out of 7 cases in which the operation was performed after 7 years of age. We could draw the following conclusion based on our results: 1. We must correct the neck-shaft angle more than 130 degrees. 2. We could not equalize the leg length discrepancy by corrective osteotomy alone. 3. It may be reasonable to perform the corrective osteotomy before 7 years of age.
Congenital Abnormalities ; Coxa Vara ; Female ; Head ; Humans ; Leg ; Orthopedics ; Osteotomy ; Parturition ; Seoul

BACKGROUND: Excitotoxicity and epileptogenesis have often been associated with glutamate receptor activation. Some evidence indicates that selective down regulation of AMPA receptor may be the mechanism of delayed neuronal cell death in the hippocampus. METHODS: We used in situ hybridization to examine the hybridization density (HD) of NMDA and AMPA receptors on excitotoxicity and epileptogenesis in the hippocampus of the kainic acid (KA)-induced rat seizure model. Some Sprague-Dawley rats were injected with KA, and others with MK-801 prior to KA injection. The rats were killed at 8 hours or 4 weeks after KA or MK-801/KA injection. HD of [3H]MK-801 and [3H]AMPA bind-ing in subfields of the hippocampus was measured by an image analyzer. RESULTS: After 8 hours of KA injection, [3H]MK-801 binding was increased in CA1 and CA3, and decreased in dentate gyrus, and [3H]AMPA binding was decreased in all of CA1, CA3 and fascia dentata, and pretreatment of MK-801 did not affect [3H]AMPA binding in all of CA1, CA3 and dentate gyrus. After 4 weeks, both [3H]MK-801 and [3H]AMPA binding were prominently increased in inner molecular layer of dentate gyrus. CONCLUSIONS: Glutamate receptors, especially NMDA receptor, were associ-ated with excitotoxicity in the hippocampus but the selective down regulation of GluR2 subunit of AMPA receptor without NMDA receptor activation may not be sufficient to cause excitotoxic neuronal cell death in CA1 and CA3. In addition, the synaptic reorganization in inner molecular layer of dentate gyrus was proved to be chronically hyperex-citable in function and may contribute epileptogenesis.
Animals ; Autoradiography* ; Cell Death ; Dentate Gyrus ; Dizocilpine Maleate* ; Down-Regulation ; Glutamic Acid* ; Hippocampus ; In Situ Hybridization ; Kainic Acid ; N-Methylaspartate ; Neurons ; Rats ; Rats, Sprague-Dawley ; Receptors, AMPA ; Receptors, Glutamate* ; Receptors, Ionotropic Glutamate* ; Seizures*

Pulmonary actinomycosis is a chronic suppurative disease usually caused by Actinomyces israelii, characterized by suppuration, sinus tract formation, and purulent discharge containing yellowish "sulfur granules". It is usually caused by aspiration of contaminated material from mouth or oropharynx. The diagnosis of pulmonary actinomycosis is difficult and its initial clinical manifestations mimic tuberculosis or neoplasm. We experienced a case of thoracic actinomycosis in a 47-year old male patient who had complained of cough, chest pain and fever for 2 months. The diagnosis was confirmed pathologically by ultrasonography guided gun-biopsy of pleural mass and thoracoscopic wedge biopsy of parenchymal lung lesion.
Actinomyces ; Actinomycosis* ; Biopsy ; Chest Pain ; Cough ; Diagnosis ; Fever ; Humans ; Lung ; Male ; Middle Aged ; Mouth ; Oropharynx ; Suppuration ; Tuberculosis ; Ultrasonography

Color-dilution alopecia is a relatively uncommon hereditary skin disease seen in "Blue" and other color-diluted dogs. This syndrome is associated with a color-dilution gene. The initial clinical signs are the gradual onset of a dry, dull and poor hair coat quality. Hair shafts and hair regrowth are poor, and follicular papules may develop and progress to frank comedones. Hair loss and comedo formation are usually most severe on the trunk, especially color-diluted area on the skin. Six cases of color-dilution alopecia are reported in 3 months to 10 years old dogs. The breeds of dogs are blue Doberman Pinscher, Miniature Pinscher, Dachshund, and Schnauzer. Grossly, extensive partial hair loss was seen on the skin. Histopathologically, the epidermis is relatively normal but may be hyperplastic. Hair follicles are characterized by atrophy and distortion. Heavily clumped melanin is present in the epidermis, dermis and hair follicles.
Alopecia/genetics/*veterinary ; Animals ; Dog Diseases/*genetics ; Dogs ; Female ; Hair Color/*genetics ; Male ; Skin/pathology

OBJECTIVE: Deep vein thrombosis (DVT) is one of the most common complications of acute spinal cord injury (SCI). It is well known that the incidence of DVT in post-acute SCI patients decreases to a level similar to that in the general population. The aim of this study was to evaluate the blood coagulation status and the possibility of DVT occurrence in post-acute SCI patients. METHOD: Twenty-three SCI patients (SCI group) were included in this study. Ten patients having spinal fracture with no evidence of SCI were used as the control group. Coagulation status was examined using factor VIII antigen, factor VIII procoagulant, fibrinogen, D-dimer, protein C, and protein S in both groups. Duplex ultrasonography was performed for the diagnosis of DVT. RESULTS: Prevalence of the abnormally decreased protein S level was significantly high in the SCI group (87%) than in the control group (30%). Prevalence of the abnormally increased F VIII:Ag, F VIII:C, and fibrinogen levels in the SCI group were significantly high in the SCI group (p<0.05). CONCLUSION: We conclude that post-acute SCI patients may have hypercoagulability. Proper physical prevention and thromboprophylaxis should be considered in post-acute SCI patients because they have relatively higher risk of thromboembolic complication.
Blood Coagulation ; Diagnosis ; Factor VIII ; Fibrinogen ; Humans ; Incidence ; Prevalence ; Protein C ; Protein S ; Spinal Cord Injuries ; Spinal Cord* ; Spinal Fractures ; Thrombophilia ; Ultrasonography ; Venous Thrombosis

Recurrent respiratory tract infections and dysphagia after the first years of life are rarely caused by vascular compression of the esophagus. We experienced a case of dysphagia and frequent vomiting resulted from esophageal compression by bronchial artery hypertrophy, which might had been aggravated by recurrent aspiration pneumonia caused by underlying swallowing difficulty. The patient initially had significant motor delay and swallowing difficulty. Videofluoroscopic swallowing studies demonstrated deglutition abnormalities and aspiration. In addition, significant amount of swallowed food was regurgitated through esophagus. On barium esophagography and angiography, posterior indentation of the esophagus without proximal dilatation and bronchial artery hypertrophy were noted without congenital heart disease. Pulmonary trunk and its branches showed normal appearance. Therefore, we considered that bronchial artery hypertrophy attributed to pulmonary artery fibrosis due to recurrent aspiration pneumonia may cause esophageal compression, which in turn contribute to dysphagia, frequent vomiting and recurrent aspiration pneumonia.
Angiography ; Barium ; Bronchial Arteries ; Deglutition ; Deglutition Disorders ; Dilatation ; Esophagus ; Fibrosis ; Heart Diseases ; Humans ; Hypertrophy ; Pneumonia, Aspiration ; Pulmonary Artery ; Respiratory Tract Infections ; Vomiting

Several factors, including genetic and environmental insults, impede protein folding and secretion in the endoplasmic reticulum (ER). Accumulation of unfolded or mis-folded protein in the ER manifests as ER stress. To cope with this morbid condition of the ER, recent data has suggested that the intracellular event of an unfolded protein response plays a critical role in managing the secretory load and maintaining proteostasis in the ER. Tauroursodeoxycholic acid (TUDCA) is a chemical chaperone and hydrophilic bile acid that is known to inhibit apoptosis by attenuating ER stress. Numerous studies have revealed that TUDCA affects hepatic diseases, obesity, and inflammatory illnesses. Recently, molecular regulation of ER stress in tooth development, especially during the secretory stage, has been studied. Therefore, in this study, we examined the developmental role of ER stress regulation in tooth morphogenesis using in vitro organ cultivation methods with a chemical chaperone treatment, TUDCA. Altered cellular events including proliferation, apoptosis, and dentinogenesis were examined using immunostaining and terminal deoxynucleotidyl transferase dUTP nick end labeling assay. In addition, altered localization patterns of the formation of hard tissue matrices related to molecules, including amelogenin and nestin, were examined to assess their morphological changes. Based on our findings, modulating the role of the chemical chaperone TUDCA in tooth morphogenesis, especially through the modulation of cellular proliferation and apoptosis, could be applied as a supporting data for tooth regeneration for future studies.

Purpose: The clinical significance of margin status in pancreatic head cancer is still controversial due to the nonstandardized definition of R status and pathologic reporting. This study aims to evaluate the impact of the margin status including location and the role of radiation therapy in pancreatic head cancer. Methods: A total of 314 patients who underwent curative-intent surgery for pancreatic head cancer between 2010 and 2017 were analyzed. Demographics, survival, and local recurrences were compared according to 2 definitions: 0-mm R1 as direct involvement and 1-mm R1 as close resection margin less than 1 mm. The specific margins were divided into 4 groups according to the location around the pancreas: pancreas transection, anterior surface, posterior surface, and vessel (superior mesenteric artery/superior mesenteric vein) margin. Results: The 0-mm R1-rate was 15.6%, and increased to 36.3% in 1-mm R1. The median overall survival rate of 0-mm R0 vs. R1 was 26 months vs. 16 months (P = 0.052) and that of 1-mm R0 vs. R1 was 27 months vs. 18 months, respectively (P = 0.016). In individual margins, posterior, anterior surface, and pancreas transection margin involvement were associated with poor outcome, and the 1 mm posterior surface involvement was an independent risk factor for disease-free survival (hazard ratio, 1.63). Adjuvant radiation therapy had oncologic benefits, especially in R1 patients (P = 0.011) compared to R0 patients (P = 0.088). Conclusion Margin status, especially 1-mm R1 status is an important predictive factor, and involved posterior surface has a clinical impact. Patients with positive margins should be considered adjuvant radiation therapy.

Enamel knot (EK)—a signaling center—refers to a transient morphological structure comprising epithelial tissue. EK is believed to regulate tooth development in early organogenesis without its own cellular alterations, including proliferation and differentiation. EKs show a very simple but conserved structure and share functions with teeth of recently evolved vertebrates, suggesting conserved signaling in certain organs, such as functional teeth, through the course of evolution. In this study, we examined the expression patterns of key EK-specific genes including Dusp26 , Fat4, Meis2, Sln , and Zpld1 during mice embryogenesis. Expression patterns of these genes may reveal putative differentiation mechanisms underlying tooth morphogenesis.