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: 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

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*

Primary cardiac lymphoma defined as involving only the heart and pericardium, is very rare and is diagnosed predominantly late in the course of illness or autopsy. This tumor is commonly fatal and until recently were rarely diagnosed antemortem. Recently, it was reported in patients with acquired immunodeficiency syndrome. We report a case of primary cardiac lymphoma in a 56 year old female who showed progressive exertional dyspnea. On echocardiogram and CT scan, large ill defined mass was demonstrated in right atrial and ventricular wall. It was diagnosed as B-cell type lymphoma on open cardiac biopsy.
Acquired Immunodeficiency Syndrome ; Autopsy ; B-Lymphocytes ; Biopsy ; Dyspnea ; Female ; Heart ; Heart Neoplasms ; Humans ; Lymphoma* ; Middle Aged ; Pericardium ; Tomography, X-Ray Computed

BACKGROUND: Systemic embolism especially, cerebral infarction is one of the most important complications in patients with mitral stenosis. The authors analyse the some that could predict the development of cerebral infarction in cases of mitral stenosis and propose preventive therapeutic measures. METHODS: Retrospective study of 127 patients with rheumatic mitral stenosis was performed by analysis their medical records for transthoracic(TTE) or transesophageal echocardiography(TEE) over a 12months period. The patients were divided into two groups according to the presence (Group I: n=26, age: 55+/-13 years) or absence (Group II: n=101, age: 48.5+/-13 years) of cerebral infarction. No significant difference was observed between the two groups with respect to sex and functional class. RESULTS: Patients of group I were older (55.0+/-13 vs 48.5+/-13;p<0.05), had more dilated left atrial size(5.10+/-0.48 vs 4.81+/-0.70;p<0.05) and smaller mitral surface area(1.01+/-0.39 vs 1.21+/-0.45;p<0.05). In Group I, the incidence of atrial fibrillation(22 out of 26 vs 57 out of 101;p<0.05) and spontaneous left intra-atrial contrast phenomenon(22 out of 26 vs 44 out of 101;p<0.05) was more frequently observed. On multivariate analysis, atrial fibrillation and anticoagulant therapy were the independent predictive factors. CONCLUSION: Age, left atrial dilatation, the severity of mitral stenosis, the presence of spontaneous contrast and especially the presence of atrial fibrillation are the main predictive factors of the development of cerebral infarction in mitral stenosis. Patients presenting one or several of these factors may benefit from prophylactic anticoagulant treatment.
Atrial Fibrillation ; Cerebral Infarction* ; Dilatation ; Embolism ; Humans ; Incidence ; Medical Records ; Mitral Valve Stenosis* ; Multivariate Analysis ; Retrospective Studies

BACKGROUND: We sought to examine the use and outcomes with early intraaortic balloon couterpulsation(IABP) combined early reperfusion therapy in patients presenting with cardiogenic shock complicating acute myocardial infarction. The use of IABP in patients with cardiogenic shock is widely accepted. however, there is a paucity of information on the use of this technique in patients with cardiogenic shock who are treated with reperfusion therapy in Korea. MATERIALS AND METHODS: Twenty-eight Patients presented with cardiogenic shock were classified into two groups: the early IABP group (insertion within 12 hours after AMI onset time) and late IABP group (insertion after 12 hours). We compared In-hospital mortality in two group (early IABP group vs late IABP group). RESULTS: Two groups show no significant difference at clinical feature and coronary angiographic results. Among total 28 patients, 7 patients were treated with thrombolytic therapy and 21 patients with PTCA. Insertion site bleeding, fever, thrombocytopenia were reported as some of the complications of IABP insertion. In-hospital mortality of early IABP group and late IABP group were 4 patients(25%) and 8 patients(66%), respectively(p<0.05). Early IABP insertion and early PTCA showed lower hospital mortality rates. There was significant difference in the time to PTCA after AMI onset between the to groups(p<0.05). CONCLUSION: IABP appears to be useful in patients presenting with cardiogenic shock unresponsive medical therapy. Early IABP insertion and early Reperfusion therapy may reduce In-hospital mortality rates in PostMI Cardiogenic shock patients.
Fever ; Hemorrhage ; Hospital Mortality ; Humans ; Korea ; Myocardial Infarction ; Reperfusion* ; Shock, Cardiogenic* ; Thrombocytopenia ; Thrombolytic Therapy