Severe myoclonic epilepsy of infancy(SMEI) is a condition beginning with recurrent, prolonged febrile convulsion in normal children, followed within months to 4 years by generalized tonic clonic seizures, partial seizures, atypical absences, myoclonic seizures and status epilepticus. The seizures are generally difficult to control. Carbamazepine which is appropriate for partial seizures, is not effective and may aggravate generalized seizures, but sodium valproate has been reported to be helpful. The evolution is always bad with persistent seizures and mental retardation. We experienced a severe myoclonic epilepsy of infancy in a 16-month-old male patient who had episodes of prolonged febrile convulsions followed by mixed type of seizures. We report a case of SMEI with a brief review of literatures.
Carbamazepine ; Child ; Epilepsies, Myoclonic* ; Humans ; Infant ; Intellectual Disability ; Male ; Seizures ; Seizures, Febrile ; Status Epilepticus ; Valproic Acid

No abstract available.

In case of submucosal tumor, adenoma and atypism, we can't frequently use the snare polypectomy for treatment of them. Adenoma and atypism are premalignant, so other therapeutic modalities are necessary for resection of them. We have perfortned endoscopic mueosectomy such as strip biopsy, and "O"ring ligation on various mucosal lesions of upper GI tract in 23 patients(11 men, l2 women) from June 1993 to December l994. Fourteen patients wbo had adenoma were followed up endoscopically for 6 months to 24 months(mean: 14 months). The results were as follows: 23 patients(27 lesions) were enrolled in this study. Mean age was 50.3(range 32-74 years). The method of mucosectomy was strip biopsy in 19 cases(21 lesions), and "O"ring ligation in 4 cases(6 lesions). The rate of complete resection was 88.9%. If adenomas were resected incompletely, we added a laser therapy. There was no recurrence except a case in which carcinoma was detected on the other site during follow-up. No serious compication occurred such as massive bleeding or perforation. Based on the above results, the endoscopic mucosectomy is considered to be a safe and effective modality in the treatment of the flat polyps of upper GI tract and could be used for prevention of gastric cancer.
Adenoma ; Biopsy ; Follow-Up Studies ; Hemorrhage ; Humans ; Laser Therapy ; Ligation ; Male ; Polyps ; Recurrence ; SNARE Proteins ; Stomach Neoplasms ; Upper Gastrointestinal Tract*

No abstract available.
Miller Fisher Syndrome

No abstract available.
Paragonimiasis*

No abstract available.
Electroencephalography* ; Neuronal Ceroid-Lipofuscinoses ; Neurons*

Immunoglobulin A (IgA) nephropathy in patients with the clinical findings of rapidly progressive glomerulonephritis usually demonstrate crescentic glomerulonephritis (CrGN). It is characterized by mesangial immune complex deposits containing IgA and is rarely associated with ANCA. The following case report illustrates a CrGN with mesangial & capillary wall IgA deposits by immunofluorescence (IF) and mesangial & endocapillary electron dense deposits by electron microscopy (EM) in patient with positive ANCA serology. A 54-year-old male visited due to the discomfort of right flank. Blood pressure was 150/90 mmHg. BUN and serum Creatinine was 40 mg/dL, 4.4 mg/dL respectively. Urinalysis revealed protein 2+, >30 RBC's/ HPF (dysmorphic), 24hr urine protein 1,612 mg/day and creatinine clearance 19 mL/min/1.73m2. Serological P-ANCA was positive by indirect immunofluorescence & ELISA. The histologic findings showed global scleorsis and fibrocelluar crescent without hypercellularity on LM, mesangial IgA deposition on IF, mesangial and subendothelial electrone dense material depositions on EM. After treatment (Methylprednisolon 500 mg/day for 3 days and then prednisolone 60 mg/day), Serum creatinin level, proteinuria and ANCA titer were decreased.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic* ; Antigen-Antibody Complex ; Blood Pressure ; Capillaries ; Creatinine ; Enzyme-Linked Immunosorbent Assay ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Male ; Microscopy, Electron ; Middle Aged ; Prednisolone ; Proteinuria ; Urinalysis

PURPOSE: Cytokines play important roles on the expression of various neuronal inflammatory disease and insults. The purpose of this study was to evaluate the levels of interleukine (IL)-6, IL-8, IL-10 in cerebrospinal fluid (CSF) in children with aseptic meningitis and compare them with those of the patients having other acute neurological symptoms. METHODS: We retrospectively reviewed the medical records of the children who admitted in the pediatric department of Hanyang University Guri Hospital for acute neurological symptoms and had CSF examinations from September 2012 to July 2013. We classified them into six groups as acute encephalopathy, epilepsy, febrile convulsion, headache, infantile fever, and meningitis. We analyzed the clinical and laboratory data from them. RESULTS: A total of 87 CSFs of the patients were available. The levels of CSF IL-6, IL-8, and IL-10 were significantly increased in the group with aseptic meningitis group as compared to the other groups (P<0.05). CSF IL-6 (r=0.576, P=0.000), IL-8 (r=0.329, P=0.003), and IL-10 (r=0.523, P=0.000) were all significantly correlated with CSF White bood cell (WBC) count. Among the patients with aseptic meningitis, CSF enterovirus positive patients (CSF entero+) showed significantly increased IL-6, IL-8, IL-10 levels than CSF enterovirus negative patients (CSF entero-) (P<0.05). In addition, the CSF entero+ and the increase of IL-10 were significantly correlated (x2=6.827, P=0.033). CONCLUSION: In patients with aseptic meningitis, the CSF IL-6, IL-8 and IL-10 were more expressed than in other neurological disease group. Among them, the enteroviral meningitis may be more related with IL-6, IL-8 and IL-10 expression than in other causes of aseptic meningitis.
Cerebrospinal Fluid* ; Child* ; Cytokines ; Enterovirus ; Epilepsy ; Fever ; Headache ; Humans ; Interleukin-10 ; Interleukin-6 ; Interleukin-8 ; Interleukins ; Medical Records ; Meningitis ; Meningitis, Aseptic* ; Neurons ; Retrospective Studies ; Seizures, Febrile

PURPOSE: Human acellular dermal matrix(ADM) is widely used in the treatment of congenital anomalies and soft tissue deficiencies. But it is rapidly degraded in the body and does not provide satisfactory results. There is a need to improve collagen fiber stability through various methods and ultimately regulate the speed of degradation. METHODS: The ADMs were added with various cross- linking agents called glutaraldehyde, dimethyl 3,3'-dithiobispropionimidate to produce cross-linked acellular dermal matrices. 1,4-butanediol diglycidyl ether solution was applied with a pH of 4.5 and 9.0, respectively. The stability of cross-linked dermal matrix was observed by measuring the shrinkage temperature and the degradation rates. The cross- and non-cross linked dermis were placed in the rat abdomen and obtained after 8, 12 and 16 weeks. RESULTS: The shrinkage temperature significantly increased and the degradation rate significantly decreased, compared to the control(p<0.05). All of cross- linked dermises were observed grossly in 16 weeks, but most of non-cross linked dermis were absorbed in 8 weeks. Histologically, the control group ADM was found to have been infiltrated with fibroblasts and most of dermal stroma were transformed into the host collagen fibers. However, infiltration of fibroblasts in the experiment was insignificant and the original collagen structure was intact. CONCLUSION: Collagen cross-linking increases the structural stability and decreases degradation of acellular dermis. Therefore, decrease in body absorption and increase in duration can be expected.
Abdomen ; Absorption ; Acellular Dermis ; Animals ; Butylene Glycols ; Collagen ; Dermis ; Ether, Ethyl ; Fibroblasts ; Glutaral ; Humans ; Hydrogen-Ion Concentration ; Imidoesters ; Rats

BACKGROUND: This study investigates the prevalance of HBV precore mutant in chronic B hepatitis patients and whether HBV precore mutants affect hepatic inflammation and response to interferon alfa. METHODS: HBV DNA in liver tissue from 48 chronic hepatitis patients was amplified by polymerase chain reaction. The HBV precore mutants were detected by direct sequencing of amplified PCR products. Thirty-three HBeAg-positive patients (Group 1: wild- type, Group 2: mixed) were received 3-6 MU INF three times a week for 4-6 months. We did follow-ups for at least six months(mean : Group 1-11.3, Group 2- 13.7 months). A complete responder was defined as persistent(>6 months) normalization of transaminase and loss of HBeAg and/or seroconversion. RESULTS: The HBV precore mutants were found in 15 cases(31.2%) among 48 patients: 7 cases(21.2%) in 33 HBeAg-positive patients and 8 cases(53.3%) in 15 HBeAg-negative patients. The HBV precore mutants were more frequently found in HBeAg-negative patients(p= 0.043). Differences in severity of hepatic pathology were not observed in the wild-type versus mutant-type chronic hepatitis B patients(p =1.00). Initial response rate was not significantly different between two Groups(p= 0.228), but complete response rate had a lower tendency in Group 2 (p=0.073). CONCLUSION: There is a tendency for HBV precore mutants to be less responsive to INF therapy than wild type. Therefore the patients with chronic hepatitis B should be treated as early as possible in natural history of their liver disease before the emergence of HBV precore mutants.
DNA ; Follow-Up Studies ; Hepatitis ; Hepatitis B e Antigens* ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Inflammation ; Interferon-alpha ; Interferons* ; Liver Diseases ; Liver* ; Natural History ; Pathology ; Polymerase Chain Reaction