We experinced a case of 4p+ syndrome in male infant. He had multiple anomalies such as flat occiput, hypertelorism, low set malformed ear, lower anterior hair line, depressed nose, broad nasal bridge, bilateral complete cleft lip and palate, short neck, unusual position of fingers, ventricular septal defect and umblical hernia. He menifested growth and developmental retardation. Karyotype with banding revealed an extra short arm of chromosome 4. The mother's karyotype was normal. His father and father's sister had a translocation between the short arm of chromosome 4 and the short arm of chromosome 9; their karyotypes were 46, XY, t(4;9) and 46, XX, t(4;9), respectively. In this case, trisomy 4p was the result of parental balanced translocatiom. As this is the first case in Korea, it is worthwhile to report with reviewing literature.
Arm ; Chromosomes, Human, Pair 4 ; Chromosomes, Human, Pair 9 ; Cleft Lip ; Ear ; Fathers ; Fingers ; Growth and Development ; Hair ; Heart Septal Defects, Ventricular ; Hernia ; Humans ; Hypertelorism ; Infant ; Karyotype ; Korea ; Male ; Neck ; Nose ; Palate ; Parents ; Siblings ; Trisomy

BACKGROUND: Prolotherapy is a therapeutic procedure used for chronic musculoskeletal and arthritic pain. It involves injecting an irritant solution to pain sites and causes patient discomfort, which can lead to treatment discontinuation. Remifentanil is an ultra short-acting micro-opiate receptor agonist that permits a rapid transition from intense analgesia to a minimal residual effect. Here, we evaluated the effect of remifentanil as a preparative medication for ambulatory prolotherapy. METHODS: Eighty patients taking prolotherapy were assigned into three groups for pre-therapeutic injections: remifentanil 0.1 microgram/kg/min alone (Group R), remifentanil 0.05 microgram/kg/min with midazolam 2 mg (Group M), and normal saline (Group C). Pain and sedation scores, blood pressure, pulse oxygen saturation, heart rate, satisfaction score, and time to discharge were measured. RESULTS: Pain scores in groups M and R were lower than group C during and after prolotherapy. The sedation score of group M was higher than groups R and C. Nine patients in group R experienced dizziness during prolotherapy. In group M, 8 patients experienced dizziness and 2 patients experienced nausea. There was no difference in time to discharge among all groups. Satisfaction scores in group M (7.3 +/- 0.8) and group R (7.0 +/- 0.8) were higher than that of group C (5.3 +/- 0.6). CONCLUSIONS: Remifentanil and remifentanil/midazolam effectively reduce the pain produced by prolotherapy.
Analgesia ; Anesthesia ; Blood Pressure ; Dizziness ; Heart Rate ; Humans ; Midazolam ; Nausea ; Outpatients ; Oxygen ; Piperidines

Radioembolization using ⁹⁰Y microspheres (glass or resin) has been introduced as an effective intraarterial therapy for unresectable primary and metastatic liver cancers. Although the basic therapeutic effect of chemoembolization results from ischemia, the therapeutic efficacy of radioembolization comes from radiation. Furthermore, compared with surgical resection and local ablation therapy, radioembolization is available with less limitation on the sites or number of liver cancers. The radioisotope ⁹⁰Y is a β-radiation emitter without γ-radiation, with the emission of secondary bremsstrahlung photons and small numbers of positrons. Administration of ⁹⁰Y microspheres into the hepatic artery can deliver a high dose of radiation selectively to the target tumor with limited radiation exposure to the surrounding normal parenchyma, and has low systemic toxicity. In general, radioembolization has been considered for patients with unresectable primary or metastatic liver-only or liver-dominant cancers with no ascites or other clinical signs of liver failure, life expectancy of > 12 weeks, and good performance status. Here, we review the current radioactive compounds, pretreatment assessment, and indications for radioembolization in patients with hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and liver metastases from colorectal cancer.
Ascites ; Carcinoma, Hepatocellular ; Cholangiocarcinoma ; Colorectal Neoplasms ; Electrons ; Hepatic Artery ; Humans ; Ischemia ; Life Expectancy ; Liver Failure ; Liver Neoplasms ; Liver ; Microspheres ; Neoplasm Metastasis ; Photons ; Radiation Exposure

Radioembolization using ⁹⁰Y microspheres (glass or resin) has been introduced as an effective intraarterial therapy for unresectable primary and metastatic liver cancers. Although the basic therapeutic effect of chemoembolization results from ischemia, the therapeutic efficacy of radioembolization comes from radiation. Furthermore, compared with surgical resection and local ablation therapy, radioembolization is available with less limitation on the sites or number of liver cancers. The radioisotope ⁹⁰Y is a β-radiation emitter without γ-radiation, with the emission of secondary bremsstrahlung photons and small numbers of positrons. Administration of ⁹⁰Y microspheres into the hepatic artery can deliver a high dose of radiation selectively to the target tumor with limited radiation exposure to the surrounding normal parenchyma, and has low systemic toxicity. In general, radioembolization has been considered for patients with unresectable primary or metastatic liver-only or liver-dominant cancers with no ascites or other clinical signs of liver failure, life expectancy of > 12 weeks, and good performance status. Here, we review the current radioactive compounds, pretreatment assessment, and indications for radioembolization in patients with hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and liver metastases from colorectal cancer.

We report on a case of ischemic dysfunction of the sinus node as a complication after percutaneous transluminal coronary angioplasty of the distal left circumflex artery. After local thrombolytic therapy in the sinus node artery, sinus node arterial flow was re-established and sinus node function normalized over the period of a week. Our experience suggests that immediate reperfusion of a totally occluded nodal artery can be re-established. Ischemic dysfunction of the sinus node, as a complication of angioplasty, is generally transient and requires a prolonged period for recovery. Therefore the decision to implant a permanent pacemaker should be delayed for at least one week after the ischemic insult.
Urinary Plasminogen Activator/administration & dosage/*therapeutic use ; Thrombolytic Therapy/*methods ; Sinoatrial Node/*physiopathology ; Myocardial Ischemia/*complications/radiography/therapy ; Middle Aged ; Male ; Infusions, Intravenous ; Humans ; Follow-Up Studies ; Fibrinolytic Agents/administration & dosage/*therapeutic use ; Electrocardiography ; Coronary Angiography ; Arrhythmia/diagnosis/*drug therapy/etiology ; Angioplasty, Transluminal, Percutaneous Coronary/adverse effects

BACKGROUND: The resting heart rate (HR) or HR recovery after exercise is one of the important predictors of cardiovascular disease mortality. However, few studies have addressed the ideal range of the HR. We sought to define the normal HR of healthy adults who have no evidence of cardiovascular or systemic illness, and none of the component of metabolic syndrome (MS). METHODS: We analyzed a total of 20,162 asymptomatic adults, who were referred for a general health evaluation. All participants underwent careful clinical evaluation, including a detailed history, physical examination and laboratory workup. The mean HR for 30 seconds in the morning after an overnight fast was obtained. There were 7,823 subjects who were free of any component of MS. There were 935 MS patients, and 10,492 patients had > or =1 component of MS. RESULTS: The HR was faster in women. The resting HR of healthy men was 59.9+/-8.2 bpm, and that of healthy women was 63.7+/-8.5 bpm. There was significant correlation between the HR and the age of healthy adults (r=-0.008, p<0.001). The mean resting HR was higher in the MS patients than that of their healthy counterparts (67.4+/-10.6 bpm vs 62.0+/-8.6 bpm, respectively, p=0.000). A significant gradual increase of HR was observed as the numbers of MS component increased (r=0.127, p<0.001). The systolic blood pressure, fasting blood sugar, HbA1c, triglyceride, gamma GTP, uric acid and CRP were significantly correlated with HR. CONCLUSIONS: We herein newly define the optimal HR in a healthy adult population. Follow-up study is needed to clarify the role of HR as a risk stratifier.
Adult* ; Blood Glucose ; Blood Pressure ; Cardiovascular Diseases ; Fasting ; Female ; Follow-Up Studies ; Guanosine Triphosphate ; Heart Rate* ; Heart* ; Humans ; Male ; Metabolic Syndrome X ; Mortality ; Physical Examination ; Triglycerides ; Uric Acid

BACKGROUND: Doppler echocardiography is widely used for the noninvasive evaluation of left ventricular diastolic function. However the mitral flow velocity pattern is affected by several physiologic factors. The mitral annular velocity profile by Doppler tissue imaging may provide more additional information about left ventricular diastolic function. Thus, this study designed to assess the relationship between cardiac catheterization, MUGA scan, mitral flow velocity, and mitral annular velocity data and to assess the clinical availavility of mitral annulus velocity in the evaluation of left ventricular diastolic function. METHODS: The study population consisted of 20 patients with dilated cardiomyopathy( 64+/-7years), 20 patients with normal left ventricular function (61+/-7years). Left ventricular catheterization was performed with fluid-filled catheter and left ventricular end diastolic pressure, -dP/dtmax were measured. The mitral flow velocity was recorded at mitral valve tip and the mitral annulus velocity during diastole was measured by Doppler tissue imaging(DTI). Simultaneously EF(ejection fraction), PER(Peak filling rate), PFR(Peak filling rate) were measured by MUGA blood pool scan. RESULTS: Mean peak E velocity, mean peak A velocity, E/A ratio, mean peak E' velocity, mean peak A' velocity, E'/A' ratio and -dP/dtmax significantly difference betweeen two group. -dP/dtmax by cardiac catheterization showed significant correlation with mean peak E' velocity (r=0.552, p=0.003), E'/A' ratio(r=0.507, p=0.003), DT of E'(r=-0.556, p=0.001), TVI of E'(r=0.689, p<0.001) and DT of E wave(r=-0.538, p=0.003). PFR by MUGA scan also showed significant correlation with -dP/dtmax(r=0.537, p=0.01). CONCLUSION: Among mitral annulus velocity index mean peak E' velocity, E'/A' ratio, DT of E',TVI of E' had significant correlation with -dP/dtmax. And DT by mitral flow velocity, PFR by MUGA scan also had significant correlation with -dP/dtmax. Mitral annulus velocity determined by DTI is relatively convenient, safe, and preload-independent variable in evaluating diastole function. Thus mitral annulus velocity by Doppler tissue imaging is may be useful diagnostic modality for evaluating left ventricular diastolic function.
Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Catheterization ; Catheters ; Diastole ; Echocardiography, Doppler ; Humans ; Mitral Valve ; Ventricular Function, Left

PURPOSE: There has been many reports for the effect of attenuation correction on myocardial perfusion SPECT. We studied the effect of attenuation correction with CT (computed tomography) in patients with normal coronary angiography. MATERIALS AND METHODS: Fifteen patients with normal coronary artery on angiography and low likelihood of coronary artery disease were enrolled in this study (male: 6, female: 9, mean age: 58+/-8 year). Myocardial perfusion SPECT was done with Millennium VG with Hawkeye device (GE, SPECT/CT camera). A visual analysis and polar map quantification (Emory tool box) was performed. In quantitative analysis, percent uptake of each myocardial wall on polar map (percent of maximal uptake) was compared between non-corrected (NC) and corrected (AC) images. RESULTS: Visual analysis showed AC images led to an increase of uptake in the inferior wall, but decrease of uptake in the anterior wall, apex and septum. Liver activity is also increased in AC images. In quantitative analysis, the percent uptake is decreased in the anterior wall, apex and septum, but increased in the inferior wall. It is helpful to interpret the images in the inferior wall after AC, but difficult in the apex and anterior wall after AC. CONCLUSION: AC is helpful in the inferior wall. But in the apex or anterior wall, AC must be carefully applied to normal perfused myocardium.
Angiography ; Coronary Angiography ; Coronary Artery Disease ; Coronary Vessels ; Female ; Humans ; Liver ; Myocardium ; Perfusion* ; Tomography, Emission-Computed, Single-Photon*

BACKGROUND: The purpose of this study is to evaluate the outcomes of four patients receiving stock Biomet TMJ prosthesis for reconstruction of the TMJs. METHODS: TMJ reconstruction with stock Biomet TMJ prosthesis was performed in four patients who had joint damages by trauma, tumor, resorption, and ankylosis, which represent the indications of alloplastic prosthesis. RESULTS: Loss of condyle from trauma and resorption of joint are good indications for prosthesis, but the patients should be informed about limitation of jaw movement. In case of structural damage of TMJ by tumor, tumor recurrence should be considered before planning TMJ reconstruction. Considering heterotopic bone formation in case of ankylosis, periodic follow-up and special surgical technique are required. CONCLUSIONS: Given careful treatment planning and understanding the functional limitation of TMJ prosthesis, alloplastic prosthesis is a safe and effective management option for the reconstruction of TMJs.
Ankylosis ; Cimetidine ; Follow-Up Studies ; Humans ; Jaw ; Joints ; Osteogenesis ; Prostheses and Implants* ; Recurrence ; Temporomandibular Joint*

BACKGROUND: Percutaneous mitral balloon valvuloplasty (PMV) has been proposed as an alternative to surgery for selected patients with symptomatic mitral stenosis. The presence of mild mitral regurgitation (MR) in mitral KERN=5535>stenosis is usually not considered as a contraindication for the procedure. But, the results of PMV in the subgroup KERN=>of patients with concomitant mild MR are unknown. Accordingly, this study evaluates the clinical outcome of a consecutive series of patients with mitral stenosis and mild MR undergoing PMV. METHODS: Between August 1992 and December 1996 we attempted to dilate the mitral valves of 43 consecutive patients with symptomatic mitral stenosis. Mean age of the group was 45.9+/-10.6 years and 37 patients were women. They were divided into two groups according to the presence or absence of mild MR before the procedure. Clinical feature, inital result, complication were compared. RESULTS: 1) Age, gender, symptomatic status, and atrial fibrillation were similar in both groups. 2) Patients with MR had more thickened valve (2.50+/-0.33 vs 1.84+/-0.55, p=0.048) and subvalvular tissue (2.55+/-0.73 vs 1.88+/-0.73, p=0.015), higher echocardiographic score (8.44+/-1.01 vs 7.08+/-1.65, p=0.004). 3) Mitral valve area gain on the first day after PMV was smaller in patients with MR(p=0.008). 4) The success rate of PMV, the incidence of severe MR after PMV, and the restenosis rate on 6 month were similar in both groups. CONCLUSION: Although the PMV in patients with mild MR obtained smaller increments in mitral valve area compared to those without MR, they showed similar immediate and short-term results after PMV.
Atrial Fibrillation ; Balloon Valvuloplasty* ; Echocardiography ; Female ; Humans ; Incidence ; Mitral Valve ; Mitral Valve Insufficiency* ; Mitral Valve Stenosis*