Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare neurodegenerative multisystem disorder inherited in an autosomal recessive manner and characterized clinically by gastrointestinal dysmotility, cachexia, ophthalmoparesis and/or ptosis, peripheral neuropathy and leukoencephalopathy. Heterogenous causative mutations in the thymidine phosphorylase (TP) gene located on chromosome 22q13 have been identifi ed. This is the fi rst reported case of a 25-year-old Malaysian patient, of indigenous Bajau ethnicity who presented with recurrent abdominal pain before developing other clinical features of classical MNGIE. Biochemical correlates include elevated plasma levels of thymidine, deoxyuridine and lactate. The brain MRI showed diffuse leucoencephalopathy while nerve conduction studies were consistent with demyelinating polyneuropathy. Direct DNA sequencing of the nine coding exons of the TP gene showed both a novel and a previously described mutation. The former is a point mutation in exon 5 (NG_011860.1:g.7387C>T) at amino acid position 179, resulting in a stop codon and premature truncation of thymidine phosphorylase(TP) protein while the latter mutation occurred at exon 10 (NG_011860.1:g.9279C>T) resulting in a missense homozygous mutation at amino acid position 471. Defi nite diagnosis was based on clinical features, plasma and urinary nucleosides and the identifi cation of mutations in the TP gene. This case report adds to the knowledge of genotype-phenotype relationship of TP mutations and its occurrence among ethnic groups worldwide.

In order to evaluate the hypotensive effect of the lisinopril, a long acting angiotensin converting enzyme inhibitor, 10 to 30mg of lisinopril were administered in 35 hypertensive Korean adults during six weeks after a week observation for washout with stepwise increments of the dose according to the response of the patients blood pressure in every two weeks. The results were ; 1) The supine blood pressures were decreased from 163.7+/-16.6/99.8+/-9.3mmHg to 140.7+/-15.5/87.4+/-9.9mmHg at the end of six weeks' drug therapy(p<0.001). The standing blood pressures were also decreased conferrably and to the some lower levels. 2) In 14 patients to whom the drug was administered longer period(12 to 28 weeks) the blood pressure lowering effects were maintained at the level of that of 6th week. 3) Hematologic examination and blood chemistry revealed no discernible abnormal findings before and after the treatment. 4) In those patients who showed no adequate blood pressure control with other classes of antihypertensive drugs the lisinopril was effective in lowering their blood pressures by adding small doses. 5) During the period of the study a few probably drug-related symptoms developed but not troublesome except dry cough and dry mouth shich forced to stop administering the drug after completion of six weeks' period in one patients. From above results we concluded that lisnopril is effective and safe for the treatment of hypertension in Korean adults.
Adult ; Antihypertensive Agents ; Blood Pressure ; Chemistry ; Cough ; Humans ; Hypertension ; Lisinopril* ; Mouth ; Peptidyl-Dipeptidase A

A retrospective analysis of 351 linear skull fractures, during the period of January 1, 1992 to December 31, 1993, reveals the following: 1) Motor-Vehicle-related accidents were responsible for most of the injuries(56%), in which pedestrians(47%) were the primary victims. 2) In 172 cases(49%), the Glasgow Coma Scale(GCS) ranged from 13 to 15. Patients wth parietal fractures usually rated on a lower scale than those with fractures on other sites. 3) Occipital fractures were most common(23%), which was followed by parietal(19%), temporal(15%), frontal(15%), and multiple fractures(8%). Occipital fractures were frequently due to a pedstrian T.A. or a fall from high elevation while temporal or parietal fractures were frequently due to bicycle and motorcycle accidents. 4) The incidence of associated intracranial lesions was 70%. Patients with occipital fractures had a lower percentage of associated intracranial lesions than those with parietal fractures. Coup injuries were found in 175 cases and contre coup injuries in 106 cases. In many cases, frontal, parietal and temporal fractures were found to be coup injuries whereas with occipital fractures, contre coup injuries. 5) Sixty-one percents of the patients were assessed in Glasgow Outcome Scale(GOS) grade I.
Coma ; Humans ; Incidence ; Motorcycles ; Rabeprazole ; Retrospective Studies ; Skull Fractures*

We examined the neurotoxic effects of 3 glutathione (GSH) depletors, buthionine sulfoximine (BSO), diethyl maleate (DEM) and phorone, under the presence of trolox, cycloheximide (CHX), pyrrolidine dithiocarbamate (PDTC) or MK-801 in primary mouse cortical cell cultures. All three depletors induced neuronal death in dose and exposure time dependent manner, and decreased total cellular GSH contents. The patterns of the neuronal death and the GSH decrements were dependent on the individual agents. DEM (200 micrometer) induced rapid and irreversible decrement of the GSH. BSO (1 mM) also decreased the GSH irreversibly but the rate of decrement was more progressive than that of DEM. Phorone (1 mM) reduced the GSH content to 40% by 4 hr exposure, that is comparable to the decrement of BSO, but the GSH recovered and reached over the control value by 36 hr exposure. BSO showed a minimal neurotoxicity (0-10%) at the end of 24 hr exposure, but marked neuronal cell death at the end of 48 hr exposure. The BSO (1 mM)-induced neurotoxicity was markedly inhibited by trolox or CHX and partially attenuated by MK-801. DEM induced dose-dependent cytotoxicity at the end of 24 hr exposure. Over the doses of 400 micrometer, glial toxicity also appeared. DEM (200 micrometer)-induced neurotoxicity was markedly inhibited by trolox or PDTC. Phorone (1 mM) induced moderate neurotoxicity (40%) at the end of 48 hr exposure. Only CHX showed significant inhibitory effect on the phorone-induced neurotoxicity. These results suggest that the GSH depletors induce neuronal injury via different mechanisms and that GSH depletors should be carefully employed in the researches of neuronal oxidative injuries.
Animals ; Buthionine Sulfoximine ; Cell Culture Techniques* ; Cell Death ; Cycloheximide ; Dizocilpine Maleate ; Glutathione* ; Mice* ; Neurons

To evaluate the depth of enflurane anesthesia, spectrum analysis of rat EEG was used. Bipolar EEG was recorded through one lead form rat scalp during inhalation with 1,2,3 and 4% of enflurane, and analysed to produce the spectrum from which the density of each band(delta 1-3.25Hz, theta 3.5-7.75Hz, alpha 8-12.75Hz, beta2 18-20.75Hz, and beta3 21-31.75Hz)and total density, and medican power frequency were calculated. Differences among the EEG patterns, which were represented by F values through discriminant analysis of those 8 variables, in each level of anesthesia were significant among all concentrations except between 1% and 2% enflurane administration. The results suggest that the discriminat anlysis for the EEG parameters derived from power spectrum analysis can apply to determine the level of enflurane anesthesia.
Anesthesia ; Anesthetics* ; Animals ; Electroencephalography* ; Enflurane* ; Inhalation ; Rats* ; Scalp ; Spectrum Analysis*

To evaluate the incidence and clinical course of AV conduction disturbances associated with acute myocardial infarction(MI) and coronary angiographic characteristics in acute inferior MI with AV blocks. We reviewed the medical records and serial ECG's in 89 patients with acute MI treated in CCU of Chonnam National University Hospital from january, 1987 through August, 1990. The subjects were 44 anterior MI's, 43 inferior MI's, and 2 anterior and inferior MI's. AV conduction disturbances were observed in 25.8% of all the patients with acute MI's, 48.8% of 43 inferior MI's and 4.5% of 44 anterior MI's. High degree AV block was observed in 20.0% of all the subjects, 39.3% of inferior MI patients, and none of anterior MI patients. The most severe AV blocks observed in each patients were 7(30.4%) first-degree, 5(21.7%) second-degree, and 11(47.8%) third-degree AV block. The initial AV conduction disturbances developed within 6 hours after onset of symptoms in 9(47.4%) and after 24-hours in 9(47.4%). Seven(30.4%) of 23 patients with AV block showed a transient progression in the degree of AV block, 5(29.4%) of 17 patients with first-or second-degree AV block progressed to third-degree AV block thereby constituting 45.5% of 11 third-degree AV blocks. Nine patients with early AV block less tended to progress in the degree of AV block than the patients with late AV block (1/9 vs 4.9). In early AV block the duration of high-degree AV block was shorter than late AV block(2.5 days vs 6.1 days). The duration of third-degree AV block was less than 2-hurs in 36.4~24 hours in 27.3%, and more than 24 hours in 36.4%. All third-degree AV blocks(90.9%) but one with the longest duration of 13 days returned to 1 : 1 AV conduction within 7 days. There was no significant difference in coronary angiographic findings including the incidence of stenotic lesion in proximal LAD and first septal perforator, number of involved vessel(s), and severity of RCA lesion between the patients with AV block and the patients without AV block in inferior MI.
Atrioventricular Block ; Humans ; Incidence ; Jeollanam-do ; Medical Records ; Myocardial Infarction*

The dose-related effects of halothane, enflurane, and isoflurane on the rat EEG were evaluated qusntitstively by spectral analysis of the EEG recorded from the rat skull. The anesthetics were inhaled by animal ventilator into 10L glass bottle, in which the preparated rats were placed, at various concentrations, and then bipolar EEG was recorded from the rat skull and its spectrum was calculated by power speetral analysis. The density of each spectral bands (delta 1-3.25, theta 3.5-7.75, alpha 8-12.25, and beta 13-31.75Hz), total density, delta ratio, spectral edge frequency, and medisn power frequency were derived from the spectra. With inspection of conventional EEG, 1.5 MAC of hslothane revealed spindles, but higher dose decreased the amplitude. 1.5 MAC of enflurane revealed a lot of spike waves but 2.0 MAC revealed several spike waves and decreased the amplitude, and 1.5 MAC isoflurane revealed isolated spike waves but 2.0 MAC revealed cerebro- electrical silence. In quantitative spectral analysis of EEG, significant EEG changes were identified during inhalation of all anestheties. In halothane dominent frequencies in EEG were delta waves at 1.5 MAC and 2.0 MAC. In enflurane dominent waves in EEG were theta waves at 1.5 MAC and 2.0 MAC and in isoflurane those were theta waves. Taken together, these findings suggest that analysis for EEG parameters derived from power spectral analysis could be applied to determine the depth of halothane, enflurane, and isoflurane anesthesia.
Anesthesia ; Anesthetics ; Animals ; Electroencephalography* ; Enflurane* ; Glass ; Halothane* ; Inhalation* ; Isoflurane* ; Rats* ; Skull ; Spectrum Analysis ; Ventilators, Mechanical

BACKGROUND: Nonspecific interstitial pneumonitis (NSIP) is most likely to be confused with usual interstitial pneumonitis (UIP). Unlike patients with UIP, the majority of patients with NSIP have a good prognosis, with most patients improving after treatment with corticosteroids. Therefore it is clinically important to differentiate NSIP from UIP. UP to now, the only means of differentiating these two diseases was by means of surgical lung biopsy. American Thoracic Society (ATS) proposed a clinical diagnostic criterial for UIP to provide assistance to clinicians in its diagnosis without surgical lung biopsy. This study is aimed to investigate whether there were clinical and radiological differences between NSIP and UIP, and the usefulness of ATS clinical diagnostic criteria for UIP in Korea. METHODS: we studied 60 patients with UIP and NSIP confirmed by surgical lung biopsy. Clinical manifestations, pulmonary function test, arterial blood gas analysis, bronchoalveolar lavage (BAL), and high resolution computed tomography (HRCT) were evaluated and analyzed by Chi-square test or t-test. The clinical criteria for UIP proposed by ATS were applied to all patients with idiopathic interstitial pneumonia. RESULTS: Forty-two patients with UIP and 18 with NSIP were pathologically identified. Among the 18 patients with NSIP (M : F = 1 : 17), the mean age was 55.2± 8.4 (44~73)yr. Among the 42 patients with UIP (M : F = 33 : 9), the mean age was 59.5±7.1 (45~74) yr (p=0.0460. Fever was more frequent in NSIP (39%) (p=0.034), but clubbing was frequently observed in UIP (33%) (p=0.023). BAL lymphocytosis was more frequent (23%) (p=0.0001) and CD4/CD8 ratio was lower in NSIP (p=0.045). On HRCT, UIP frequently showed honeycomb appearance (36 of 42 patients) through not in NSIP (p=0.0001). Six of 42 UIP patients (14.3%) met the ATS clinical criteria for IPF, and 3 of 16 NSIP patients (18.8%) met the diagnostic criteria. CONCLUSION: Being a relatively young female and having short duration of illness, fever, BAL lymphocytosis, low CD4/CD8 ratio with the absence of clubbing and honeycomb appearance in HRCT increase the likelihood of the illness being NSIP. The usefulness of ATS clinical diagnostic criteria for UIP may be low in Korea.
Adrenal Cortex Hormones ; Biopsy ; Blood Gas Analysis ; Bronchoalveolar Lavage ; Diagnosis ; Diagnosis, Differential* ; Female ; Fever ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis* ; Korea ; Lung ; Lung Diseases, Interstitial* ; Lymphocytosis ; Prognosis ; Pulmonary Fibrosis ; Respiratory Function Tests

BACKGROUND: Nonspecific interstitial pneumonia (NSIP) has been reported recently to show much better response to medical treatment and better prognosis compared with idiopathic UIP. However, clinical characteristics of idiopathic NSIP discriminating from UIP have not been defined clearly. METHOD: Among 120 patients with biopsy-proven diffuse interstitial lung diseases between July 1996 and March 2000 at Samsung Medical Center, 18 patients with idiopathic NSIP were included in this study. Retrospective chart review and radiographic analysis were performed. RESULTS: 1) At diagnosis, 17 patients were female and average age was 55.2 +/-8.4 years (44~73 years). The average duration from development of respiratory symptom to surgical lung biopsy was 9.9+/-17.1 months. Increase in bronchoalveolar lavage fluid lymphocytes (23.0 +/-13.1%) was noted. On HRCT, ground glass and irregular linear opacity were seen but honeycombing was absent in all patients. 2) Corticosteroids were initially given to 13 patients of whom medication was stopped in 3 patients due to severe side effects. Further medical therapy was impossible in 1 patient who experienced streroid-induced psychosis. Herpes zoster (n=3), tuberculosis (n=1), avascu lar necrosis of hip (n=1), cataract (n=2) and diabetes mellitus (n=1) developed during prolonged corticosteroid administration. Of 7 patients receiving oral cyclophosphamide therapy, hemorrhagic cystitis hindered one patient from continuous medication. 3) After medical treatment, 14 of 17 patients improved and 3 patients remained stable (mean w-up ; 24.1+/-11.2 months). FVC increased by 20.2 +/-11.2% of predicted value and the extent of ground glass opacity on HRCT decreased significantly (15.7+/-14.7%). 4) Of 14 patients who had stopped medication, 5 showed recurrence of NSIP and 2 aggravated during steroid tapering. All patients with recurrence showed deterioration within one year after completion of initial treatment. CONCLUSION: Since idiopathic NSIP has unique clinical profiles and shows a good prognosis, differential diagnosis from UIP and aggressive medical treatment are needed.
Adrenal Cortex Hormones ; Biopsy ; Bronchoalveolar Lavage Fluid ; Cataract ; Cyclophosphamide ; Cystitis ; Diabetes Mellitus ; Diagnosis ; Diagnosis, Differential ; Female ; Glass ; Herpes Zoster ; Hip ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases, Interstitial* ; Lymphocytes ; Necrosis ; Prognosis ; Psychotic Disorders ; Recurrence ; Retrospective Studies ; Tuberculosis

BACKGROUND/AIMS: Despite of increasing numbers of reports on intraductal papillary mucinous tumor (IPMT), there is still difficulty in its' diagnosis, treatment and prediction of prognosis. The purpose of this multicenter study was to evaluate the clinico-pathological features of IPMT in Korea and suggest the prediction criteria of malignancy in IPMT. METHODS: We retrospectively reviewed the clinico-pathological data of 208 patients who underwent operations with IPMT between 1993 and 2002 at 28 institutes in Korea. RESULTS: Of the 208 patients with a mean age of 60.5+/-9.7 years, 147 were men and 61 were women. 124 patients underwent pancreatoduodenectomy, 42 distal pancreatectomy, 17 total pancreatectomy, 25 limited pancreas resection. Benign cases were 128 (adenoma (n=62), borderline (n=66)) and malignant cases were 80 (non-invasive (n=29), invasive (n=51)). A significant difference in 5-year survival was observed between benign and malignant group (92.6% vs. 65.3%; p=0.006). Of the 6 factors (age, location, duct dilatation, tumor appearance, main duct type, and tumor size) that showed the statistical difference in univariate analysis between benign and malignant group, we found three significant factors (tumor appearance (p=0.009), tumor size (p=0.023), and dilated duct size (p=0.010)) by multivariate analysis. CONCLUSION: Although overall prognosis of IPMT is superior to ordinary pancreatic cancer, more curative surgery is recommended in malignant IPMT. Tumor appearance (papillary), tumor size (> or =30 mm) and dilated duct size (> or = 12 mm) can be used as preoperative indicators of malig-nancy in IPMT.
Academies and Institutes ; Diagnosis ; Dilatation ; Female ; Humans ; Korea* ; Male ; Mucins* ; Multivariate Analysis ; Pancreas* ; Pancreatectomy ; Pancreatic Neoplasms ; Pancreaticoduodenectomy ; Prognosis ; Retrospective Studies