No abstract available.
Aorta, Thoracic*

OBJECTIVES: Hypertension provokes left ventricular diastolic dysfunction due to decreased elasticity of the ventricular myocardium at first. It has been proposed that the Doppler echocardiography might provide information concerning diastole because of its ability to measure the blood flow velocities across the mitral valve noninvasively. But Doppler mitral velocity may be "normalized" in hypertensive patients in different conditions. The purpose of this study is to evaluate diastolic function in hypertensive hearts using Doppler echocardiography of the pulmonary venous flow in conjunction with mitral flow velocity pattern. METHODS: We measured the mitral flow velocity curves and the pulmonary flow velocity curves in fifty hypertensive patients(mean age : 56.3+/-10.0, man : 19, woman : 31) nad forty healthy adults(mean age : 51.6+/-12.5, man : 14, woman : 26) from March 1995 to December 1995 by using the SONO 1000 of Hewlett Packerd. RESULTS: 1) The mitral flow velocity pattern in 50 hypertensive patients was characterized by the decrease in the peak early diastolic filling velocity(E : 0.59+/-0.12m/sec) and the ratio of E to peak filling velocity(A : 1.00+/-0.46m/sec). Isovolumetric relaxation time and deceleration time were 127.2+/-33.3msec, 258.9+/-40.9msec respectively. They were longer than normotensive patients(P < 0.05). 2) The pulmonary venous flow velocity pattern in hypertensive patients was characterized by the decreased peak diastolic forward velocity(D : 33.7+/-9.8cm/sec) and the ratio of peak systolic forward velocity(S) to D(S/D : 1.60+/-0.5)(P < 0.05). 3) There was no relation between the flow velocity integral parameters and the peak flow velocity parameters of pulmonary venous flow pattern in hypertensive patients. 4) The dofference in the pulmonary venous duration and the mitral A wave duration at atrial contraction (DA-DR) was 20.9+/-29.4 msec in hypertensive patients. DA-DR in normatensive patients was 24.3+/-32.9msec. DA-DR was not significant in hypertensive patients compared with that of the healthy subjects (P < 0.05) CONCLUSION: Analysis of pulmonary venous flow velocity pattern in conjunction with mitral flow velocity pattern in hypertensive patients provides more accurate assessment of left ventricular diastolic function.
Blood Flow Velocity ; Deceleration ; Diastole ; Echocardiography, Doppler ; Elasticity ; Female ; Heart* ; Humans ; Hypertension ; Mitral Valve ; Myocardium ; NAD ; Relaxation

PURPOSE: We conducted this study to evaluate the efficacy of Doppler study by examining obstruction at the site of anastomosis in patients with total anomalous pulmonary venous return(TAPVR). METHODS: Retrograde analysis of the postoperative echocardiography results was done in 14 patients with simple TAPVR, who were operated at Dong-A University Hospital from January 1993 to July 1998. The peak systolic velocities, peak diastolic velocities and flow patterns of the 14 patients were compared with those of 9 control cases. Among the 14 patients, 2 cases showed evidence of obstruction at the anastomosis site. RESULTS: Pulmonary venous flow patterns of normal infants were biphasic, varying with the cardiac cycle. The peak velocities during systole and diastole were 40 to 60cm/sec(mean 51+/-9cm/sec) and 45 to 78cm/sec(mean 59+/-9cm/sec), respectively. The flow patterns of patients without postoperative stenosis were also biphasic. The peak velocities during systole and diastole was 38 to 115cm/sec(mean 71+/-27cm/sec) and 55 to 140cm/sec(mean 111+/-28cm/sec), respectively. The diastole peak velocity was significantly higher than normal(P=0.0002). The flow patterns of patients with postoperative stenosis was continuous, non-phasic or increased peak velocity even though it was phasic. CONCLUSION: Postoperative Doppler echocardiographic evaluation of pulmonary venous return in patients with TAPVR is useful in examining obstruction at the site of anastomosis. But a study on the Doppler echocardiographic normal range of postoperative patients will be needed.
Constriction, Pathologic ; Diastole ; Echocardiography* ; Echocardiography, Doppler ; Humans ; Infant ; Reference Values ; Scimitar Syndrome* ; Systole

PURPOSE: We conducted this study to evaluate the efficacy of Doppler study by examining obstruction at the site of anastomosis in patients with total anomalous pulmonary venous return(TAPVR). METHODS: Retrograde analysis of the postoperative echocardiography results was done in 14 patients with simple TAPVR, who were operated at Dong-A University Hospital from January 1993 to July 1998. The peak systolic velocities, peak diastolic velocities and flow patterns of the 14 patients were compared with those of 9 control cases. Among the 14 patients, 2 cases showed evidence of obstruction at the anastomosis site. RESULTS: Pulmonary venous flow patterns of normal infants were biphasic, varying with the cardiac cycle. The peak velocities during systole and diastole were 40 to 60cm/sec(mean 51+/-9cm/sec) and 45 to 78cm/sec(mean 59+/-9cm/sec), respectively. The flow patterns of patients without postoperative stenosis were also biphasic. The peak velocities during systole and diastole was 38 to 115cm/sec(mean 71+/-27cm/sec) and 55 to 140cm/sec(mean 111+/-28cm/sec), respectively. The diastole peak velocity was significantly higher than normal(P=0.0002). The flow patterns of patients with postoperative stenosis was continuous, non-phasic or increased peak velocity even though it was phasic. CONCLUSION: Postoperative Doppler echocardiographic evaluation of pulmonary venous return in patients with TAPVR is useful in examining obstruction at the site of anastomosis. But a study on the Doppler echocardiographic normal range of postoperative patients will be needed.
Constriction, Pathologic ; Diastole ; Echocardiography* ; Echocardiography, Doppler ; Humans ; Infant ; Reference Values ; Scimitar Syndrome* ; Systole

BACKGROUND: The intramural coronary artery has been known as a risk factor for early death after an arterial switch operation (ASO). We reviewed the morphological characteristics and evaluated the early and mid-term results of ASO for patients with an intramural coronary artery. MATERIALS AND METHODS: From March 1994 to September 15th 2010, 158 patients underwent ASO at Dong-A and Pusan National University Hospitals for repair of transposition of the great arteries and double outlet right ventricle. Among these patients, 14 patients (8.9%) had an intramural coronary artery. Mean age at operation was 13.4+/-10.2 days (4 to 39 days) and mean body weight was 3.48+/-0.33 kg (2.88 to 3.88 kg). All patients except one were male. Eight patients had TGA/IVS and 4 patients had an aortic arch anomaly. Two patients (14.3%) had side-by-side great artery relation, of whom one had an intramural right coronary artery and the other had an intramural left anterior descending coronary artery. Twelve patients had anterior-posterior relation, all of whom had an intramural left coronary artery (LCA). The aortocoronary flap technique was used in coronary transfer in 8 patients, of whom one patient required a switch to the individual coronary button technique 2 days after operation because of myocardial ischemia. An individual coronary button implantation technique was adopted in 6, of whom 2 patients required left subclavian artery free graft to LCA during the same operation due to LCA injury during coronary button mobilization and LCA torsion. RESULTS: There was 1 operative death (7.1%), which occurred in the first patient in our series. This patient underwent an aortocoronary flap procedure for coronary transfer combining aortic arch repair. Overall operative mortality for 144 patients without an intramural coronary artery was 13.2% (19/144). There was no statistical difference in operative mortality between the patients with and without an intramural coronary artery (p>0.1). There was no late death. The mean follow-up duration was 52.1+/-43.0 months (0.5 to 132 months). One patient who had a subclavian artery free graft required LCA stenting 6.5 years after surgery for LCA anastomotic site stenosis. No other surviving patient needed any intervention for coronary problems. All patients had normal ventricular function at latest echocardiography and were in NYHA class 1. CONCLUSION: The arterial switch operation in Transposition of Great Arteries or Double Outlet Right Ventricle patients with intramural coronary can be performed with low mortality; however, there is a high incidence of intraoperative or postoperative coronary problems, which can be managed with conversion to the individual coronary button technique and a bypass procedure using a left subclavian free graft. Both aortocoronary flap and individual coronary button implantation techniques for coronary transfer have excellent mid-term results.
Aorta, Thoracic ; Arteries ; Body Weight ; Constriction, Pathologic ; Coronary Vessels ; Double Outlet Right Ventricle ; Echocardiography ; Follow-Up Studies ; Hospitals, University ; Humans ; Incidence ; Male ; Myocardial Ischemia ; Risk Factors ; Stents ; Subclavian Artery ; Transplants ; Transposition of Great Vessels ; Ventricular Function

The amino acids formed by degradation of proteins ingested produce ammonia. The ammonia which is broken down and excreted as urea through a process known as the Klebs-Hensleit cycle or the urea cycle. 1) The urea cycle consists of five enzymes necessary for the synthesis of carbamyl phosphate, citrulline, argininosuccinate, arginine, and urea: carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinate synthetase (AS), argininosuccinate lyase (AL), and arginase (ARG). 2) Congenital deficiencies of the enzymes involved in the urea cycle are diseases that are almost fatal without treatment, showing symptoms like vomiting, lethargy, dyspnea, and coma due to hyperammonemia coming from the accumulation of ammonia and metabolic precursors resulting from the deficiency of one of these enzymes. 3) Among these, the disease manifested by the congenital deficiency of argininosuccinate synthetase (AS) which is associated with the formation of argininosuccinate in citrulline is called argininosuccinate synthetase deficiency or citrullinemia. There have been two reports on this so far in Korea; one in July 1987 by Kim et al. 4) and the other by Park et al. 5) in 1995. We are to report a case of successful treatment of a child with citrullinemia who was transferred to our hospital due to dyspnea, lethargy, feeding difficulties, convulsions and cyanosis together with some document studies related to this case.
Amino Acids ; Ammonia ; Arginase ; Arginine ; Argininosuccinate Lyase ; Argininosuccinate Synthase ; Carbamyl Phosphate ; Child* ; Citrulline ; Citrullinemia* ; Coma ; Cyanosis ; Dyspnea ; Humans ; Hyperammonemia ; Korea ; Lethargy ; Ligases ; Ornithine Carbamoyltransferase ; Seizures ; Urea ; Urea Cycle Disorders, Inborn ; Vomiting

Purpose: The pathogenesis of lower urinary tract symptoms (LUTS) without benign prostatic hyperplasia (BPH) in men is unclear. In this study, patients with LUTS without BPH were analyzed with a urodynamic study to search for effective treatments. MATERIALS AND METHODS: Ninety nine men with LUTS without BPH were assessed by their medical history, symptom score, uroflowmetry, filling cystometry and a pressure- flow study. The patients were divided into irritating and obstructive symptom groups according to their chief complaints. The urodynamic parameters between the two groups were compared. RESULTS: On urodynamics, 33 (33.3%) patients showed demonstrable evidence of detrusor instability (DI) of whom 10 had a concomitant bladder outlet obstruction (BOO), while 8 had concomitant detrusor underactivity (DU). BOO and DU was identified in 21 (21.2%) and 30 (30.3%) patients, respectively. Of the 61 patients presented with irritating symptoms, DI, BOO and an impaired contractility (IC) was found in 26.3, 13.1, and 13.1% of patients, respectively. Of the 38 patients with obstructive symptoms, DI, BOO and IC was found in 7.1, 8.1, and 17.2%, respectively. There was a significant positive correlation between the irritating symptoms and the presence of DI. CONCLUSIONS: Lower urinary tract symptoms in men are common and often misdiagnosed. This study demonstrated that 84 (84.8%) patients with lower urinary tract symptoms without a benign prostatic hyperplasia had urodynamic abnormalities such as DI, BOO and IC. A urodynamic study may to be useful in establishing a correct diagnosis and launching the appropriate therapy.
Diagnosis ; Humans ; Lower Urinary Tract Symptoms* ; Male ; Multiple Endocrine Neoplasia Type 1 ; Prostatic Hyperplasia* ; Urinary Bladder Neck Obstruction ; Urodynamics*

Spinal muscular atrophy(SMA) is the second most common fatal disease of childhood with autosomal dominant mode of inheritance, and in its less severe form the third most common neuromuscular disease of childhood after Duchenne muscular dystrophy. The genetic defect was found to be on the long arm of chromosome 5(5q11.2-q13.3) where many genes and microsatellite markers were missing. One of the most important genes is the Survival Motor Neuron(SMN) gene which is homozygously missing in 90% of SMA patients. Another important gene, the Neuronal Apoptosis Inhibitory Protein(NAIP) gene was found to be defective in 67% of SMA type I patients. Studies so far suggest SMA occurs when the genes on the long arm of chromosome 5 are mutated or deleted. Recently our hospital encountered 2 SMA patients of type I and II respectively. These patients both had homozygously defective SMN genes but intact NAIP genes. We are reporting these cases with bibliographic review and discussion. Korean SMA patients presumably have defects in SMN genes similar to those found in European patients, although the siginificance of NAIP genes remains to be established. SMN gene defects can be easily diagnosed using PCR and restriction enzymes, and this method could be applied towards convenient prenatal diagnosis and towards screening for family members at risk.
Apoptosis ; Arm ; Chromosomes, Human, Pair 5 ; Diagnosis* ; Humans ; Mass Screening ; Microsatellite Repeats ; Muscular Atrophy, Spinal* ; Muscular Dystrophy, Duchenne ; Neuromuscular Diseases ; Neurons ; Polymerase Chain Reaction ; Prenatal Diagnosis ; Wills

PURPOSE: To assess the accuracy of intraocular lens (IOL) power prediction for cataract surgery with open angle glaucoma (OAG) and to identify preoperative angle parameters correlated with postoperative unpredicted refractive errors. MATERIALS AND METHODS: This study comprised 45 eyes from 45 OAG subjects and 63 eyes from 63 non-glaucomatous cataract subjects (controls). We investigated differences in preoperative predicted refractive errors and postoperative refractive errors for each group. Preoperative predicted refractive errors were obtained by biometry (IOL-master) and compared to postoperative refractive errors measured by auto-refractometer 2 months postoperatively. Anterior angle parameters were determined using swept source optical coherence tomography. We investigated correlations between preoperative angle parameters [angle open distance (AOD); trabecular iris surface area (TISA); angle recess area (ARA); trabecular iris angle (TIA)] and postoperative unpredicted refractive errors. RESULTS: In patients with OAG, significant differences were noted between preoperative predicted and postoperative real refractive errors, with more myopia than predicted. No significant differences were recorded in controls. Angle parameters (AOD, ARA, TISA, and TIA) at the superior and inferior quadrant were significantly correlated with differences between predicted and postoperative refractive errors in OAG patients (-0.321 to -0.408, p<0.05). Superior quadrant AOD 500 was significantly correlated with postoperative refractive differences in multivariate linear regression analysis (β=-2.925, R²=0.404). CONCLUSION: Clinically unpredicted refractive errors after cataract surgery were more common in OAG than in controls. Certain preoperative angle parameters, especially AOD 500 at the superior quadrant, were significantly correlated with these unpredicted errors.
Biometry ; Cataract* ; Glaucoma, Open-Angle* ; Humans ; Iris ; Lenses, Intraocular ; Linear Models ; Myopia ; Refractive Errors* ; Tomography, Optical Coherence

PURPOSE: The incidence of secondary malignancies in hematologic patients is known to be higher than it is in other patients. However, the characteristics of secondary malignancy and surveillance have not yet been established for colorectal cancer in leukemic patients. METHODS: From 1995 to 2007, 6,030 patients who were diagnosed with acute myeloid leukemia (AML), acute lymphoid leukemia (ALL), chronic myeloid leukemia (CML), chronic lymphoid leukemia (CLL), and multiple myeloma (MM) were enrolled in this study. Among them, 9 patients were diagnosed with colorectal cancer at St. Mary's Hospital and were analyzed retrospectively. RESULTS: Three of the 2,570 patients with AML, 1 of the 1,158 patients with CML, 2 of the 83 patients with CLL, 2 of the 422 patients with MM, and none of the 1,797 patients with ALL were found to have colorectal cancer. There were no operative mortalities, but 2 patients refused to have surgery. The ratio of observed to expected subsequent colorectal cancer in CLL was higher than it was in the other groups, indicating that the relative risk of colorectal cancer is higher in patients with CLL. CONCLUSION: Compared to the Surveillance, Epidemiology and End-Result (SEER) program at the National Cancer Institute (NCI) in the United State, we have the same high relatively risk in CLL patients. Careful attention should be paid to the possibility of colorectal cancer in CLL patients.
Colon ; Colonic Neoplasms ; Colorectal Neoplasms ; Hematologic Diseases ; Humans ; Incidence ; Leukemia ; Leukemia, Lymphoid ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Multiple Myeloma ; National Cancer Institute (U.S.) ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies