No abstract available.
Humans* ; Myelin Basic Protein* ; Myelin Sheath* ; Myelin-Associated Glycoprotein* ; Oligodendroglia*

No abstract available.
Diagnosis* ; Epilepsy*

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

Mitral valvular orifice area is important for the evaluation of prognosis and treatment of patients with mitral valvular stenosis. Until recently, Gorlin's formula using cardiac catheterization has been utilized in the measurement of mitral valve area, but it is invasive and impractical to examine repeatedly. Recently 2-Dimensional echocardiography appeared to be a practical and useful substitute in measurement of valve area and also it is economical and has no risk to patients. In 31 patients with mitral valvular stenosis examined at the echocardiography room of Busan national University Hospital from March 1982 to March 1983, we measured the mitral valve area with 2-Dimensional echocardiography and evaluated its relationship with the left ventricular functions measured on M-mode echocardiogram. Among many parameters on M-mode measurements EF slope, excursion amplitude and the ratio of left atrial dimension to aortic root dimension were rather helpful for the assessment of severity of mitral valvular stenosis than other parameters. And each relationship with the valve area showed correlation coefficient of 0.60, 0.05 and -0.58, respectively.
Busan ; Cardiac Catheterization ; Cardiac Catheters ; Constriction, Pathologic* ; Echocardiography* ; Humans ; Mitral Valve* ; Prognosis ; Ventricular Function, Left

PURPOSE: The ictal perfusion patterns of cerebellum and basal ganglia have not been systematically investigated in patients with temporal lobe epilepsy (TLE). Their ictal perfusion patterns were analyzed in relation with temporal lobe and frontal lobe hyperperfusion during TLE seizures using SPECT subtraction. MATERIALS AND METHODS: Thirty-three TLE patients had interictal and ictal SPECT, video-EEG monitoring, SPGR MRI, and SPECT subtraction with MRI co-registration. RESULTS: The vermian cerebellar hyperperfusion (CH) was observed in 26 patients (78.8%) and hemispheric CH in 25 (75.8%). Compared to the side of epileptogenic temporal lobe, there were seven ipsilateral hemispheric CH (28.0%), fifteen contralateral hemispheric CH (60.0%) and three bilateral hemispheric CH (12.0%). CH was more frequently observed in patients with additional frontal hyperperfusion (14/15, 93.3%) than in patients without frontal hyperperfusion (11/18, 61.1%). The basal ganglia hyperperfusion (BGH) was seen in 11 of the 15 patients with frontotemporal hyperperfusion (73.3%) and 11 of the 18 with temporal hyperperfusion only (61.1%). In 17 patients with unilateral BGH, contralateral CH to the BGH was observed in 14 (82.5%) and ipsilateral CH to BGH in 2 (11.8%) and bilateral CH in 1 (5.9%). CONCLUSION: The cerebellar hyperperfusion and basal ganglia hyperperfusion during seizures of TLE can be contralateral, ipsilateral or bilateral to the seizure focus. The presence of additional frontal or basal ganglia hyperperfusion was more frequently associated with contralateral hemispheric CH to their sides. However, temporal lobe hyperperfusion appears to be related with both ipsilateral and contralateral hemispheric CH.
Basal Ganglia* ; Cerebellum* ; Epilepsy, Temporal Lobe* ; Frontal Lobe ; Humans ; Magnetic Resonance Imaging ; Perfusion ; Seizures ; Temporal Lobe* ; Tomography, Emission-Computed, Single-Photon

Bacteroides fragilis is a Gram negative nonsporulating anaerobic rod bacterium that makes up about 1 to 2% of the norrnal human colonic microflora. In 1984, Myer et al. reported that some strains of B. fragilis produce enterotoxin and cause diarrheal disease in cattle and human. Since then it has been termed enterotoxigenic B. fragilis (ETBF). In this study, we tried to detect enterotoxin gene from 37 B. fragilis strains, isolated in Korean patients, to confirm the existence of ETBF and usefulness of PCR as a rapid diagnosis method. By this method, we identified 9 ETBF strains and confirmed their pathogenesis by cytotoxicity test. No significant cross- reactivity with other anaerobes or aerobes was observed. Thus, the PCR method may be considered useful for the sensitive and rapid detection of anaerobic infections. And the entire amplified PCR mixture was ligated into a pT7Blue T-vector and transformed into E. coli. When the nucleotide sequences of cloned PCR products were compared with reported enterotoxin gene, pBF529 inserted DNA sequence was nearly in good agreement with it but pBF570 inserted DNA sequence showed some difference at nucleotide 270-300. A search for nucleotide sequence homologies revealed that pBF529 exhibited 99%, but pBF570 indicated only 90% identity with reported enterotoxin gene. According to these results, it was suggested that ETBF toxin can be differentiated into at least 2 subtypes.
Animals ; Bacteroides fragilis* ; Bacteroides* ; Base Sequence ; Cattle ; Clone Cells ; Colon ; Diagnosis ; Enterotoxins* ; Humans ; Korea* ; Polymerase Chain Reaction*

The antiarrhythmic efficacy if low dose amiodarone treatment was studied in 30 cases of ventricular premature beats(VPBs). Amiodarone was administered 600mg daily in three divided doses for for initial 7-10 days as loadihg dosage,then 100-200mg once daily as maintenance. The results obtained were as follow : 1) The complete control of VPBs was achieved by amiodarone treatment in 90%, 27cases of 30 cases(all 11 cases with simple VPBs and 16 cases of the remainders with complex VPBs). 2) The QT interval and QTc were significantly prolonged, whereas heart rate was reduced significantly after amiodarone treatment. 3) In 27 cases of responder, the frequency of VPBs began to decrease overtly 2-3 days after amiodarone administration, then relatively stablized in 6 days, and complete cnotrol of VPBs was achieved in all cases about 10 days after treatment. 4) No significant side-reaction was observed except the decrease of serm T3 level after treatment.
Amiodarone* ; Arrhythmias, Cardiac* ; Heart Rate ; Selective Estrogen Receptor Modulators

We evaluated the clinical effects of Nicorandil in 27 patients (17 male and 10 female) with ischemic heart disease (17 patients of stable effort angina, 3 patients of unstable effort angina, 6 patients of spontaneous angina, 1 patient of variant angina) in terms of the effect on the anginal pain, electrocardiographic changes and side effects. The results obtained were as follows; 1. The pulse rate was not changed by the drug administration and blood pressure were decreased slightly by Nicorandil in a daily dose of 15 mg divided into 3 dose, but these decrease were not significant in statistical meaning. 2. Improvement in EKG changes was observed in 9 patients (69%) among the 13 patients who showed abnormal EKG initially. 3. Anti-anginal effect of nicorandil were excellent in 14 patients, good in 8 patients, fair in 3 patients and so the rate of global improvement was 82%. 4. Nicorandil had side effects in 7 patients, headache (4 patients), palpitation, ocular pain, edema, but these were transitory and tolerable except of one case who could not be continued because of severe headache.
Angina Pectoris* ; Blood Pressure ; Edema ; Electrocardiography ; Headache ; Heart Rate ; Humans ; Male ; Myocardial Ischemia ; Nicorandil*

Antihypertensive effect and side effect of Tripamide(Normonal(R)) was studied in 22 cases of essential hypertension using 15mg once daily regimen for average period of 9 weeks. 1) Antihypertensive effect Average reduction of 25mmHg in systolic and 19mmHg in systolic and 19 mmHg in diastolic pressure was observed and reduction rate was 15.7% and 17.6% respectively. The overall effect rate was 86%. The blood pressure lowered significantly in 1 week of treatment both in systolic and diastolic pressure and the extent of fall in systolic pressure approached near the level of maximum reduction in 2 weeks of administration and that of diastolic after 5 weeks of treatment. 2) Laboratory tests and side effect There was no significant changes in individual and mean value of Na, K, Cl, BUN, creatinine, uric acid, fasting blood sugar, cholesterol, GOT, GPT studied before and after treatment except one case showing elevation of uric acid. No significant side effect was observed during trial except of 2 cases of transient diuretic effect.
Blood Glucose ; Blood Pressure ; Cholesterol ; Creatinine ; Diuretics ; Fasting ; Hypertension ; Uric Acid