No abstract available.
Child ; Cleft Lip ; Humans ; Palate ; Parents ; Skull ; Statistics as Topic

BACKGROUND/AIMS: There is considerable variance in individual susceptibility to hepato-toxic effects of ethanol as evidenced by the finding that only about 10-20% of alcoholics develop alcoholic liver cirrhosis. The aims of this study were, 1) to get the data on the genetic polymorphisms of three major ethanol-metabolizing enzymes (ADH, CYP2E1, ALDH) in normal Korean adults, and to search for the specific genotypes influencing alcohol drinking behavior by the comparison of allele frequencies between healthy control group and heavy drinker group with or without liver disease, 2) to investigate the influence of the genetic polymorphisms of these enzymes on the susceptibility to alcoholic liver disease by the comparison of allele frequencies between heavy drinker group without liver disease and alcoholic liver cirrhosis group. METHODS: Healthy control group included 53 healthy males in military service without evidence of liver disease or alcoholism. Heavy drinker group without liver cirrhosis included 29 males who had been drinking 80g or more of alcohol daily for more than ten years but did not have any clinical evidence of liver disease. Alcoholic cirrhosis group included 43 male patients who had drunk 80g or more of alcohol daily for more than ten years and had clinical evidences of overt cirrhosis. Subjects with hepatitis B surface antigen or anti-hepatitis C antibody were excluded. Genotypes of the three enzymes were determined by PCR (polymerase chain reaction) and restriction fragment length polymorphism (RFLP) with genomic DNAs extracted from peripheral leukocytes. RESULTS: 1) In healthy Korean males, allele frequency of ADH22, ADH31, CYP2E1 c2 and ALDH22 was 81%, 94%, 30% and 14%, respectively. 2) The absence of ALDH22 or CYP2E1 c2 allele were significant risk factors for being a heavy drinker (odds ratio,' 0.09, 0.42, respectively). 3) Although it was not associated with the polymorphism of each ethanol-metabolizing enzymes, the susceptibility to alcoholic liver cirrhosis was significantly associated with combined genotypes of ADH2(22) & ADH3(1+1)& CYP2E1 B or C. COMCLUSION: Genetic polymorphisms of ethanol-metabolizing enzyrnes are significantly associated with the suseptability to alcoholic liver disease as well as alcohol drinking behavior.
Adult ; Alcohol Drinking ; Alcoholics* ; Alcoholism ; Alleles ; Cytochrome P-450 CYP2E1 ; DNA ; Drinking ; Ethanol ; Fibrosis ; Gene Frequency ; Genotype ; Hepatitis B Surface Antigens ; Humans ; Leukocytes ; Liver Cirrhosis ; Liver Cirrhosis, Alcoholic* ; Liver Diseases ; Liver Diseases, Alcoholic ; Male ; Military Personnel ; Polymerase Chain Reaction ; Polymorphism, Genetic* ; Polymorphism, Restriction Fragment Length ; Risk Factors

Objectives: . Cochlear implant (CI) users typically report impaired ability to understand speech in noise. Speech understanding in CI users decreases with noise due to reduced temporal processing ability, and speech perceptual errors involve stop consonants distinguished by voice onset time (VOT). The current study examined the effects of noise on various speech perception tests while at the same time used cortical auditory evoked potentials (CAEPs) to quantify the change of neural processing of speech sounds caused by noise. We hypothesized that the noise effects on VOT processing can be reflected in N1/P2 measures, the neural changes relate to behavioral speech perception performances. Methods: . Ten adult CI users and 15 normal-hearing (NH) people participated in this study. CAEPs were recorded from 64 scalp electrodes in both quiet and noise (signal-to-noise ratio +5 dB) and in passive and active (requiring consonant discrimination) listening. Speech stimulus was synthesized consonant-vowels with VOTs of 0 and 50 ms. N1-P2 amplitudes and latencies were analyzed as a function of listening condition. For the active condition, the P3b also was analyzed. Behavioral measures included a variety of speech perception tasks. Results: . For good performing CI users, performance in most speech test was lower in the presence of noise masking. N1 and P2 latencies became prolonged with noise masking. The P3b amplitudes were smaller in CI groups compared to NH. The degree of P2 latency change (0 vs. 50 ms VOT) was correlated with consonant perception in noise. Conclusion . The effects of noise masking on temporal processing can be reflected in cortical responses in CI users. N1/P2 latencies were more sensitive to noise masking than amplitude measures. Additionally, P2 responses appear to have a better relationship to speech perception in CI users compared to N1.

BACKGROUND/AIMS: Combined esophageal variceal ligation and sclerotherapy has been hypothesized to be more effective for the control of bleeding esophageal varices than ligation alone. The present study was to compare the combined therapy with ligation alone in terms of variceal eradication, rebleeding, complication and survival rates in patients with bleeding esophageal varices. METHODS: Thirty-eight patients with bleeding esophageal varices were randomly assigned to receive ligation alone in 20 patients or the combined therapy in 18 patients. The clinical and endoscopic characteristics of patients in the ligation group were similar to those of patients in the combination group. In the combination group, 1-3 mL of ethanolamine was injected proximal to each ligated site. Treatments were repeated every 2- to 3-month until varices were eradicated. RESULTS: No significant differences were found between the ligation and combination groups in variceal eradication rates (70% vs. 72%), numbers of endoscopic sessions required to achieve eradication (3.5+/-0.33 vs. 3.3+/-0.31), rebleeding rates (30% vs. 28%) or 2-yr cumulative survival rates (95% vs. 75%). There were significantly more complications in the combination group (25% vs. 89%, p=0.001). CONCLUSION: Ligation alone is recommended rather than the combined ligation and sclerotherapy because of its lower complication rates.
Esophageal and Gastric Varices* ; Ethanolamine ; Hemorrhage* ; Humans ; Ligation* ; Sclerotherapy* ; Survival Rate ; Varicose Veins

BACKGROUND/AIMS: Combined esophageal variceal ligation and sclerotherapy has been hypothesized to be more effective for the control of bleeding esophageal varices than ligation alone. The present study was to compare the combined therapy with ligation alone in terms of variceal eradication, rebleeding, complication and survival rates in patients with bleeding esophageal varices. METHODS: Thirty-eight patients with bleeding esophageal varices were randomly assigned to receive ligation alone in 20 patients or the combined therapy in 18 patients. The clinical and endoscopic characteristics of patients in the ligation group were similar to those of patients in the combination group. In the combination group, 1-3 mL of ethanolamine was injected proximal to each ligated site. Treatments were repeated every 2- to 3-month until varices were eradicated. RESULTS: No significant differences were found between the ligation and combination groups in variceal eradication rates (70% vs. 72%), numbers of endoscopic sessions required to achieve eradication (3.5+/-0.33 vs. 3.3+/-0.31), rebleeding rates (30% vs. 28%) or 2-yr cumulative survival rates (95% vs. 75%). There were significantly more complications in the combination group (25% vs. 89%, p=0.001). CONCLUSION: Ligation alone is recommended rather than the combined ligation and sclerotherapy because of its lower complication rates.
Esophageal and Gastric Varices* ; Ethanolamine ; Hemorrhage* ; Humans ; Ligation* ; Sclerotherapy* ; Survival Rate ; Varicose Veins

Isoflurane causes little myocardial depression, rapid onset and recovery during controlled hypotensive anesthesia. Nitroglycerin, vasodilating agent, has short plasma half-life and myocardial protective effect, is easy to cantrol, and has no direct toxic effect. Doxapram hydrochloride(doxapram Hcl), respiratory stimulant, has been found to be safe and significantly potent, but also has significant pressor effect when larger doses are administered. The purpose of this study was to evaluate the effects of doxapram on the hemodynamics after isoflurane and nitroglycerin-induced hypotensive anesthesia in dogs. Hemodynamic measurement including the value of left ventricular pressure, aortic pressure, pulmonary eapillary wedge pressure, pulmonary artery pressure, heart rate, cardiac output, maximal and minimal dP/dT were determined in 8 dogs before doxapram Hcl administration, Smin, 15min and 30min after doxapram Hcl administration. 1) Left ventricular pressure and aortic pressure increased at 5min and 15min after doxapram Hcl administration but did not change significantly at 30min compared to the preadministration values. 2) Pulmonary capillary wedge pressure and pulmonary artery pressure increased significantly at Smin and 15min, but did not change significantly 30min compared to the preadministration values. 3) Heart rate increased significantly at Smin, but did not change significantly at 15min and 30min compared to the preadministration value. 4) Cardiac output and body temperature did not change significantly at 5min, 15min compared to the preadministation values. 5) Maximal dP/dT increased signifieantly at Smin and 15min, but did not change at 30min compared to the preadministration value, minimal dP/dT increased significantly at 5min, but did not change at 15min and 30min compared to the preadministration value.
Anesthesia* ; Animals ; Arterial Pressure ; Arteries ; Body Temperature ; Cardiac Output ; Depression ; Dogs* ; Doxapram* ; Half-Life ; Heart Rate ; Hemodynamics* ; Isoflurane* ; Nitroglycerin* ; Plasma ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Ventricular Pressure

BACKGROUND: The skin changes of X-linked recessive ichthyosis are cnused by the deficiency of the enzyme steroid sulfatase, which usually results from deletions of this gene in Caucasian populations. OBJECTIVE AND MEHTODS: To disgnose X-linked recessive ichthyosis and detect its carrier, we have investigated distinctive gene deletion and measured gene dosage of steroid sulfatase gene by southern blot hybridization in Korean patients with X-linked recessive ichthyosis. RESULTS: Patients from 8 of 9 unrelated families exhibited deletions, if the steroid sulfatase gene. Of 6 families showing a family history compatible with X-linked recessive inheritance, One family exhibited a normal pattern of hybridization. All but one family showed deletion of steroid sulfatase gene. All three patients lacking a fami1y history of the disease exhibited gene deletions. The ratio of the steroid sulfatsse specific band density to the Factor VIII specific band density was measured in 8 obligate carriers using a laser densitometer. The average ratio exhibited by the car riers was less than half that of normal women. Conclusian: These results suggest that the X-linked recessive ichth osis patient and its carrier can also be diagnosed and detected by Southern blot hybridization of steroid sulfatase gene in Korea.
Blotting, Southern* ; Diagnosis* ; Factor VIII ; Female ; Gene Deletion ; Gene Dosage ; Humans ; Ichthyosis* ; Korea ; Skin ; Steryl-Sulfatase ; Wills

BACKGROUND: A fully automated enzyme-immunoassay (EIA) analyzer, Enzymun System, ES-300 (Boehringer Mannheim, Germany) uses streptavidin technology and performs single test or panels of up to 12 tests per run. We evaluated the results of ES-300 for anti-HCV by comparing the results with microplate-EIA, radioimmunoassay (RIA), and confirmatory test. METHODS: Total 79 sera (51 positive, 24 negative, 4 indeterminate results confirmed by Lucky HCD Confirm) were analysed. ES-300 with Enzymun-Test(R) Anti-HCV (Boehringer Mannheim, Germany) and microplate-EIA (Green Cross Center Innotest HCV 3.0(R)) were used. Fifty one sera were examined additionally by 2nd-generation RIA method, NANBDINE 125C(General Biologicals Corp., R.O.C.). And all results were compared to the results of Lucky HCD Confirm. RESULTS: The overall concordance rate of ES-300 and Innotest(R) was 72/79 (91.1%). The results of Lucky HCD Confirm on seven discrepant samples were five negative and two indeterminate. The results of ES-300 and NANBDINE 125C showed concordance rate of 90.2%. The sensitivity and specificity of ES-300 with regard to Lucky HCD Confirm were 94.5%, and 87.5%, respectively, and that of Innotest(R) were 98.2% and 66.7%, respectively. Clear distinction of positive and negative results by signal/cut off ratio was available in both EIAs. The positive predictive values of ES-300 and Innotest(R) were 94.5%, and 87.1%, respectively. CONCLUSIONS: ES-300 showed relatively good results in sensitivity and positive predictive value with regard to confirmatory test. In EIA-positive persons, however, follow-up study would be necessary for reliable evaluation of HCV infection.
Humans ; Radioimmunoassay ; Sensitivity and Specificity ; Streptavidin

We report herein two cases of pachyonychia congenita in 19-month-old and 7-year-old girls. In addition to severely deformed nails, both had white plaques on the tongue and keratotic papules on the elbows and knees. In the 19-month-old girl, her father showed deformity of the nails and severe hyperkeratosis of the palms and soles, The family history was normal in other case.
Child ; Congenital Abnormalities ; Elbow ; Fathers ; Female ; Humans ; Infant ; Knee ; Nails, Malformed* ; Pachyonychia Congenita* ; Tongue

No abstract available.
Plasma Exchange* ; Plasma* ; Seoul*