A 74-year old woman presented with partial and secondarily generalized status epilepticus lasted for 11 days. Initially her seizures consisted of only unformed visual hallucination, which progressed to formed hallucinations, and then memory disturbance and GTCs. During the period of recurrent formed visual hallucinations, T2-weighted brain MRI revealed high signal intensities in the left occipital lobe. After intravenous phenytoin loading, she did not develop any further GTCs but visual hallucinations persisted. Follow-up MRI performed after complete recovery of seizures showed complete recovery of the previous focal lesions, however, 1H-MRS showed a significant decrease of NAA in the recovered area. These features suggested the neuronal loss in the area of seizure focus, despite the complete recovery of transient focal abnormalities in MRI. This case provides a supportive evidence of neuronal damage even in focal status epilepticus, which stress the importance of early treatment and EEG confirmation of the complete seizure control after the disappearance of clinically obvious seizures.
Aged ; Brain ; Electroencephalography ; Female ; Follow-Up Studies ; Hallucinations ; Humans ; Magnetic Resonance Imaging ; Memory ; Neurons* ; Occipital Lobe* ; Phenytoin ; Seizures ; Status Epilepticus*

The World Health Organization declared that a new strain of novel swine-origin influenza A (H1N1) virus was responsible for the pandemic infection in June 2009. We report a case of encephalitis diagnosed as the H1N1 virus infection. We describe a 17-year-old patient who had a seizure attack, diagnosed with a H1N1 virus infection via real time reverse-transcriptase polymerase chain reaction (RT-PCR). The H1N1 virus infection can be causative of the encephalitis, as with other influenza virus infections. Careful monitoring is essential for reducing complications.
Adolescent ; Animals ; Encephalitis, Viral/*diagnosis/*virology ; Humans ; Influenza A Virus, H1N1 Subtype/*pathogenicity ; Male ; Swine/*virology

Nitric oxide synthases (NOSs) that catalyzed the conversion of L-arginine to nitric oxide and L-citrulline play a role in ischemic-reperfusion injury. The purpose of this study was to observe the expression patterns of nNOS, iNOS and eNOS in the rat tibialis anterior and soleus muscles after multiple cyclic episodes of ischemic preconditioning (IP). Nine weeks old male SD rats were divided into control and IP groups. The IP group was further divided into 3 groups based on cycle of IP. For IP, left commom iliac artery was occluded 3, 6 and 10 times for 5 minutes ischemia followed by 5 minutes reperfusion using rodent vascular clamps. The animals were sacrificed at 0, 3, 6, 24 and 72 hours of reperfusion and the left tibialis anterior and soleus muscles were removed. The expression of nNOS, iNOS and eNOS were examined with immunohistochemical methods and Western blot analysis. IP increased the expression of nNOS, compared with the control. In the tibialis anterior muscle, the levels of nNOS in the 3IP and 6IP were higher than that in 10IP. IP increased the expression of iNOS, compared with the control, and the levels of iNOS in tibialis anterior muscle were higher than that in soleus muscle. The level of iNOS in the 10IP was higher than those in the 3IP and 6IP. IP increased the expression of eNOS, compared with the control, and the level of eNOS in soleus muscle were higher than that in tibialis anterior muscle. At 0 and 3 hours after reperfusion, the level of eNOS in 6IP and 10IP were higer than that in 3IP. In summary, these results suggest that the ischemic preconditioning increases the expression of nNOS, iNOS and eNOS, and 10 times of ischemic preconditioning may induce ischemic injury through upregulation of iNOS. And tibialis anterior muscle is more susceptabile to ischemic injury than soleus muscle.
Animals ; Arginine ; Blotting, Western ; Humans ; Iliac Artery ; Ischemia ; Ischemic Preconditioning* ; Male ; Muscle, Skeletal ; Muscles* ; Nitric Oxide Synthase* ; Nitric Oxide* ; Rats* ; Reperfusion ; Rodentia ; Up-Regulation

BACKGROUND: Charcot-Marie-Tooth (CMT) disease is the most common form of inherited motor and sensory neuropathy. Neurofilament light chain polypeptide (NEFL) is one of the most abundant cytoskeletal components of the neuron. The NEFL gene encoding the neurofilament light chain plays an important role in the axonal structure that includes an extensive fibrous network in the cytoplasm of the neuron. Mutations in the NEFL gene are also present in CMT2E, CMT type 1 and Dejerine-Sottas syndrome. However, there have been no reports to investigate the NEFL genes in Korean CMT patients. Therefore, we investigated to find the clinical characteristics in patients with the NEFL gene mutation. METHODS: We examined mutations of the NEFL gene in 125 Korean CMT families. Mutations were confirmed by the sequencing of both strands. Nerve conduction studies were carried out on CMT patients having each mutation. RESULTS: Three pathogenic mutations were found in 3 families, and 2 polymorphisms in 2 families. Two mutations (Leu334Pro, Pro22Arg) were determined too novel, and those were not detected in 105 healthy controls. A de novo missense mutation was found in a CMT family with the NEFL mutation. The frequency of the NEFL mutation was 2.4%, which was similar in Europeans, and lower than those found in Japanese. Pro22Arg and Glu397Lys mutations showed demyelinating neuropathy and Leu334pro mutation showed axonal neuropathy. CONCLUSIONS: We found NEFL mutations in patients with sporadic or dominantly inherited CMT. NEFL mutations should be considered in the evaluation of CMT or related neuropathies with various clinical features.
Asian Continental Ancestry Group ; Axons ; Charcot-Marie-Tooth Disease* ; Cytoplasm ; Hereditary Sensory and Motor Neuropathy ; Humans ; Mutation, Missense ; Neural Conduction ; Neurons

The hyaline membrane disease is not a common disease in Korea. Only a few reports of small scale are avaiable in the literature. We have experienced 5 cases of HMD during approximately 1 year period. The diagnosis was made either on characteristic clinical and roentgenological features or postmortem examination. The birth weights of these cases were in the range of 1,000-1,500gm in 2 cases and 2,000-2,500gm in 3 cases. And their gestational age was 28-34 weeks in most of the cases. Three cases were delivered by C-section. There was 1 case of placenta previa. Four of these 5 cases died after average 18 hours postnatum. Postmortem findings in two cases were characterized by typical hyaline membrane lining th respiratory bronchioles and alveolar ducts. Other prominent findings were atelectasis, interstitial edema and congestion and lymphatic dilatation. One case complicated with multifocal bronchopneumonia and perinatal telencephalic leukoencephalopathy. The other case showed acute subarachnoid hemorrhage probably from germinal matrix hemorrhage.
Autopsy* ; Birth Weight ; Bronchioles ; Bronchopneumonia ; Diagnosis ; Dilatation ; Edema ; Estrogens, Conjugated (USP) ; Gestational Age ; Hemorrhage ; Humans ; Hyalin* ; Hyaline Membrane Disease* ; Infant, Newborn ; Korea ; Leukoencephalopathies ; Membranes ; Placenta Previa ; Pulmonary Atelectasis ; Subarachnoid Hemorrhage

PURPOSE: Cancer of the uterine cervix remains the leading cause of cancer death in Korean women. Conventional examinations still have limitations with regards to sensitivity and specificity in diagnosis and to monitoring of the disease. Thus, an additional specific tumor marker is needed for early detection of recunence of uterine cervical carcinoma and for estimation of prognosis. MATERIALS AND METHODS: Monoclonal antibodies against human cervical carcinoma were generated using hybridoma technology. These tnurine monoclonal antibodies were produced by fusion of spleen cells obtained from mice immunized with CUMC-6, a human cell line of squamous cell carcinoma derived from uterine cervix, and P3-X63-Ag8 mouse myeloma cells. RESULTS: We obtained 415 hybridomas secreting specific monoclonal antibodies to cervical carcinoma antigen continuously. Among them, one hybridoma designated CCS that was highly reactive with cervical carcinoma was selected and examined on. the staining pattern and the reactivity with antigenic detenninants of cervical carcinoma. Immunohistochemical staining revealed that CCS monoclonal antibody reacted with all of the seven cervical carcinoma tissues, but also reacted with one of the ten (10%) normal cervical tissues. Westem blot analysis showed that CC5 monoclonal antibody detected single 19.5-kDa protein band in cervical cancer patient's sera. The detection rate was 88% (7/8). However, the antibody did not show any reactivity to 15 sera of normal healthy women tested. Sodium dodecyl sulfate polyacrylamide gel electrophoretic (SDS-PAGE) analysis of CCS monoclonal antibody immunoprecipitates of extracts of L-[S] methionine-labeled human cer vical carcinoma cells showed a major band in apparent molecular weight of 51,000 daltons. The isotype and subclass of CC5 monoclonal antibody was IgG2b in hemagglutination assay. CONCLUSIONS: We have developed a new monoclonal antibody, CC5, against squamous cell carcinoma of the human uterine cervix. Further investigation is needed to establish this monoclonal antibody as an immunodiagnostic devise for cervical cancer.
Animals ; Antibodies, Monoclonal ; Carcinoma, Squamous Cell ; Cell Line ; Cervix Uteri ; Diagnosis ; Female ; Hemagglutination ; Humans ; Hybridomas ; Immunoglobulin G ; Mice ; Molecular Weight ; Prognosis ; Sensitivity and Specificity ; Sodium Dodecyl Sulfate ; Spleen ; Uterine Cervical Neoplasms

Melanin is a pigment produced from tyrosine in melanocytes. Although melanin has a protective role against UVB radiation-induced damage, it is also associated with the development of melanoma and darker skin tone. Tyrosinase is a key enzyme in melanin synthesis, which regulates the rate-limiting step during conversion of tyrosine into DOPA and dopaquinone. To develop effective RNA interference therapeutics, we designed a melanin siRNA pool by applying multiple prediction programs to reduce human tyrosinase levels. First, 272 siRNAs passed the target accessibility evaluation using the RNAxs program. Then we selected 34 siRNA sequences with ΔG ≥−34.6 kcal/mol, i-Score value ≥65, and siRNA scales score ≤30. siRNAs were designed as 19-bp RNA duplexes with an asymmetric 3′ overhang at the 3′ end of the antisense strand. We tested if these siRNAs effectively reduced tyrosinase gene expression using qRT-PCR and found that 17 siRNA sequences were more effective than commercially available siRNA. Three siRNAs further tested showed an effective visual color change in MNT-1 human cells without cytotoxic effects, indicating these sequences are anti-melanogenic. Our study revealed that human tyrosinase siRNAs could be efficiently designed using multiple prediction algorithms.
Dihydroxyphenylalanine ; Gene Expression ; Humans ; Melanins ; Melanocytes ; Melanoma ; Monophenol Monooxygenase* ; RNA ; RNA Interference ; RNA, Small Interfering* ; Skin Pigmentation ; Tyrosine ; Weights and Measures

BACKGROUND: Cancer is the second cause of childhood death following accident, and leukemia is the most frequent childhood cancer in Korea. For the desirable control of childhood leukemia, of which the mortality is still high, the basic data for the incidence has a great importance. This is the second report from the data during 1991~1995 following the first one that analyzed the data from 328 cases of childhood leukemia during 1981~1990 in the same area, Pusan city, Korea. METHODS: The data were obtained from 138 new cases(84 males and 54 females from 0 to 15 years old) of childhood leukemia who had been living in the city of Pusan and were admitted to the 4 university hospitals and 11 general hospitals from 1991 to 1995. The cases were confirmed by CBC and bone marrow examination. RESULTS: The crude annual incidence rate per 100,000 population varied 1.50~5.30, 2.59~6.00 and 1.58~2.61 in the age group of 0~4 years, 5~9 years and 10~14 years, respectively. The standardized annual incidence rate per 100,000 population varied from 2.05 to 3.46(male 2.96~4.89, female 0.98~3.57). Sex ratio(male to female) was 1.58:1, 1.44:1, and 1.82:1 in total cases, ALL and AML, respectively, while incalculable in CML. By the major types of childhood leukemia, the cases were composed of 105 ALL (76.1%), 31 AML(22.5%), 2 CML(1.4%). CONCLUSION: It was concluded that the annual incidence rate of childhood leukemia per 100,000 population in Pusan city during 1991~1995 was similar to that of previous report during 1981~1990, while the proportion of ALL had tendency to increase up to that of United States, in contrast to the low proportions of ALL in the previous reports.
Bone Marrow Examination ; Busan* ; Female ; Hospitals, General ; Hospitals, University ; Humans ; Incidence* ; Korea* ; Leukemia* ; Male ; Mortality ; United States

PURPOSE: The management and clinical course in pediatric patients who had ingested foreign body were investigated retrospectively to evaluate the frequency and factor associated with successful removal of fishbone foreign body. METHODS: Based on the medical records of patients younger than 15 years old who visited emergency room because of foreign body ingestion from January 1999 to December 2012, the authors reviewed clinical characteristics including type of ingested foreign bodies, time to visits, managements and complications. RESULTS: Fishbone (50.1%) was the most common ingested foreign body in children. Among 416 patients with ingested fishbone, 245 (58.9%) were identified and removed using laryngoscope, rigid or flexible endoscope from pharynx or upper esophagus by otolaryngologists and pediatric gastroenterologists. The kind of ingested fish bone in children was diverse. The mean age of identified and removed fishbone group was 7.39 years old, and higher than that of unidentified fishbone group (5.81 years old, p<0.001). Identified and removed fishbone group had shorter time until hospital visit than the unidentified fishbone group (2.03 vs. 6.47 hours, p<0.001). No complication due to ingested fishbone or procedure occurred. CONCLUSION: Older age and shorter time from accident to hospital visit were the different factors between success and failure on removal of ingested fish bone in children.
Child* ; Eating* ; Emergency Service, Hospital ; Endoscopes ; Esophagus ; Foreign Bodies ; Humans ; Laryngoscopes ; Medical Records ; Pharynx ; Retrospective Studies

We tested correlations between anti-Helicobacter pylori IgG and IgA levels and the urease test, anti-CagA protein antibody, degree of gastritis, and age. In total, 509 children (0-15 years) were enrolled. Subjects were stratified as 0-4 years (n = 132), 5-9 years (n = 274), and 10-15 years (n = 103) and subjected to the urease test, histopathology, ELISA, and western blot using whole-cell lysates of H. pylori strain 51. The positivity rate in the urease test (P = 0.003), the degree of chronic gastritis (P = 0.021), and H. pylori infiltration (P < 0.001) increased with age. The median titer for anti-H. pylori IgG was 732.5 IU/mL at 0-4 years, 689.0 IU/mL at 5-9 years, and 966.0 IU/mL at 10-15 years (P < 0.001); the median titer for anti-H. pylori IgA was 61.0 IU/mL at 0-4 years, 63.5 IU/mL at 5-9 years, and 75.0 IU/mL at 10-15 years (P < 0.001). The CagA-positivity rate was 26.5% at 0-4 years, 36.5% at 5-9 years, and 46.6% at 10-15 years for IgG (P = 0.036), and 11.3% at 0-4 years, 18.6% at 5-9 years, and 23.3% at 10-15 years for IgA (P < 0.001). Anti-H. pylori IgG and IgA titers increased with the urease test grade, chronic gastritis degree, active gastritis, and H. pylori infiltration. Presence of CagA-positivity is well correlated with a high urease test grade and high anti-H. pylori IgG/IgA levels.
Adolescent ; Antibodies, Bacterial/*blood ; Antigens, Bacterial/*analysis/immunology ; Bacterial Proteins/*analysis/immunology/metabolism ; Blotting, Western ; Child ; Child, Preschool ; Chronic Disease ; Enzyme-Linked Immunosorbent Assay ; Female ; Gastritis/pathology ; Helicobacter Infections/blood/microbiology/*pathology ; Helicobacter pylori/isolation & purification/*metabolism ; Humans ; Immunoglobulin A/*blood ; Immunoglobulin G/*blood ; Infant ; Infant, Newborn ; Male ; Severity of Illness Index ; Urease/metabolism