No abstract available.
Muscular Dystrophy, Duchenne* ; Polymorphism, Restriction Fragment Length*

Dermatomyositis is a disease of unknown etiology characterized by inflammation and degeneration of skeletal muscles and cutaneous abnormalities. Cardiac involvement in dermatomyositis-polymyositis is thought to be rare. In recent year, however, there has been an increasing number of reports on cardiac abnormalities in adult dermatomyositis and polymyositis due to development of noninvasive diagnostic techniques. Categorically, these abnormalities have included electrocardiographic changes, cardiac arrhythmias, congestive heart failure, coronary artery disease, and pericarditis. A 56-year-old woman was admittied to the Ewha womans University Hospital with dyspnea and palpitation. She was diagnosed as having dermatomyositis and followed up our department of Dermatology. Electrocardiogram showed a paroxysmal supraventricular tachycardia at as rate of 195/min. The patient was treated with 240mg/day verapamil p.o, 60mg/day prednisone p.o, there was marked improvement of symptoms. Systematic study of cardiac function utilizing echocardiography, Holter monitoring, thallium-201-scan, and gated blood pool studies were conducted in five newly diagnosed patients with dematomyositis-polymyositis. A significant elevation of serum CPK-MB is indicative of cardiac involvement. Cardiac involvement is a serious prognostic sign. We report a case with the review of the literature.
Adult ; Arrhythmias, Cardiac ; Coronary Artery Disease ; Dermatology ; Dermatomyositis* ; Dyspnea ; Echocardiography ; Electrocardiography ; Electrocardiography, Ambulatory ; Female ; Heart Failure ; Humans ; Inflammation ; Middle Aged ; Muscle, Skeletal ; Pericarditis ; Polymyositis ; Prednisone ; Prognosis ; Tachycardia, Supraventricular* ; Verapamil

No abstract available.
Animals ; Humans ; Infant, Newborn* ; Intestines* ; Rats*

3-methylcrotonyl-coenzyme A carboxylase (3MCC) deficiency is an autosomal recessive disorder in which leucine catabolism is hampered, leading to increased urinary excretion of 3-methylcrotonylglycine. In addition, 3-hydroxyisovalerylcarnitine levels increase in the blood, and the elevated levels form the basis of neonatal screening. 3MCC deficiency symptoms are variable, ranging from neonatal onset with severe neurological abnormality to a normal, asymptomatic phenotype. Although 3MCC deficiency was previously considered to be rare, it has been found to be one of the most common metabolic disorders in newborns after the neonatal screening test using tandem mass spectrometry was introduced. Additionally, asymptomatic 3MCC deficient mothers have been identified due to abnormal results of unaffected baby's neonatal screening test. Some of the 3MCC-deficient mothers show symptoms such as fatigue, myopathy, or metabolic crisis with febrile illnesses. In the current study, we identified an asymptomatic 3MCC deficient mother when she showed abnormal results during a neonatal screening test of a healthy infant.
Asymptomatic Diseases ; Fatigue ; Humans ; Infant ; Infant, Newborn ; Leucine ; Metabolism ; Mothers ; Muscular Diseases ; Neonatal Screening* ; Phenotype ; Tandem Mass Spectrometry

BACKGROUND: Rapid and accurate identification of methicillin-resistant Staphylococcus (MRSA) is very important for patients because they are one of the most common etiologic agents of hospital infection. Conventional identification methods for MRSA are influenced by various factors such as pH, concentration of salt, conditions of media. METHODS: 53 methicillin resistant staphylococcus strains identified by ATB plus system (Biomerieux, France) were preformed the polymerase chain reaction (PCR), Southern blot hybridization fort the detection of mec A gene, and subcultured in Meuller-Hinton media containing 4 microgram/mL oxacillin for the comparison. RESULTS: The correlation of detection rate of mec A gene PCR and ATB plus systems was 81.6%. The correlation of mec A gene PCR and MRSA on Mueller-Hinton media containing 4 microgram/mL oxacillin was 80%. We confirmed by Southern blot hybridization the amplified mer A gene originated from chromosome of MRSA. As the results of oxacillin sensitivity test, minimal inhibitory concentrations of MRSA were distributed between 40 microgram/mL and 320 microgram/mL. When compared with executing time, ATB plus system took 24 hours, but PCR took 5 hours for identification. CONCLUSION: We concluded that mec A gone PCR techniques were simple and rapid for detection of MRSA comparative to conventional methods.
Blotting, Southern ; Cross Infection ; Genes, vif ; Humans ; Hydrogen-Ion Concentration ; Methicillin Resistance* ; Methicillin-Resistant Staphylococcus aureus* ; Oxacillin ; Polymerase Chain Reaction* ; Staphylococcus

No abstract available.

A child, aged 4-year-old girl was admitted to our hospital because of impaired vision and right hemiparesis. Brain MRI showed a lesion in the left parietal white matter, and visual evoked potentials and brainstem evoked potentials gave abnormal findings showing lesions also of the optic nerves and brainstem. Her symptoms were resolved with steroid therapy. Afterwards, she had 3 episodes of spastic paraparesis, cerebellar syndrome, downbeat nystagmus, and clinical seizures for a year. With a history of remissions and exacerbations and brain MRls that revealed new scattered lesions each time, the diagnosis of multiple sclerosis was able to be made. This is the one of the youngest of known patients with MS, yet described, in Korea, whose first attack occurred at 4 years old of age.
Brain ; Brain Stem ; Cerebellar Diseases ; Child ; Child, Preschool* ; Diagnosis ; Evoked Potentials ; Evoked Potentials, Visual ; Female ; Humans ; Korea ; Magnetic Resonance Imaging ; Multiple Sclerosis* ; Optic Nerve ; Paraparesis, Spastic ; Paresis ; Rabeprazole ; Seizures

This report is concerned with our clinical experience of 150 cases of anesthesia for open heart surgery at department of anesthesiology, In chon Gil hospital from Februry, 1990 to April, 1993. The results were as follows ; 1) Among 150 cases, 68 cases (45.3%) were congenital heart disease and 82 cases (54.7%) were acquired heart disease. 62 cases (41.3%) were males and 88 cases (58.7%) were females. 2) Glycopyrrolate, diazepam, morphine, were used as premedicants. 3) Fentanyl, ketamine, diazepam, thiopental sodium were used as induction agents and injected singly or in combination. 4) In congenital cyanotic heart diseases, ketamine was used as main anesthetic agent. In other heart diseases, fentanyl, isoflurane, diazepam were used. 5) Vecuronium was used for intubation and maintenance of muscle relaxation. 6) Overall mortality rate was 3.3% (5 cases) and the causes of death were low cardiac output, respiratory insufficiency, mediastinal infection.
Anesthesia ; Anesthesiology ; Cardiac Output, Low ; Cause of Death ; Diazepam ; Female ; Fentanyl ; Glycopyrrolate ; Heart Defects, Congenital ; Heart Diseases ; Heart* ; Humans ; Incheon ; Intubation ; Isoflurane ; Ketamine ; Male ; Morphine ; Mortality ; Muscle Relaxation ; Respiratory Insufficiency ; Thiopental ; Thoracic Surgery* ; Vecuronium Bromide

BACKGROUND: Infants are very different from children and adults in the point of anatomy, physiology, pharmacology and psychology. The mortality rate of infant anesthesia is higher than that of child and adult. So that this study is to analyze the infant's operation and to recognize the tendency and problems of infant's anesthesia and also to improve the outcome. METHODS: A retrospective analysis was performed on 1436 infants who had operations from april 1987 to february 1995 according to age, sex, department, disease, operation time, postoperative complications, mortality rate and anesthetic method. RESULTS: Total number of operation tend to increase annually. The male to female ratio was 67.5% to 32.5%. The distribution of patients by department was 60.6% of general surgery, 21.0% of plastic surgery and small percentage of other department. The distribution of disease was the following : inguinal hernia - the most common; cleft lip and palate, intussusception and others. The postoperative complications developed in 98 of 1436. The most common complication was the respiratory problems, the others were sepsis, electrolyte unbalance, gastrointestinal and CNS problems. The total mortality rate was 3.6%. The emergency operation case was 17.9%. CONCLUSIONS: The most common distribution of age was neonate and the most commm department was general surgery and the most common disease was inguinal hernia. The most common postoperative complication was respiratory problems and the second was infection and sepsis. The mortality rate in preterm infant, neonate, emergency operation and long duration operation was higher than total motality rate.
Adult ; Anesthesia* ; Child ; Cleft Lip ; Emergencies ; Female ; Hernia ; Hernia, Inguinal ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intussusception ; Male ; Mortality ; Palate ; Pharmacology ; Physiology ; Postoperative Complications ; Psychology ; Retrospective Studies* ; Sepsis ; Surgery, Plastic

BACKGROUND: Infants are very different from children and adults in the point of anatomy, physiology, pharmacology and psychology. The mortality rate of infant anesthesia is higher than that of child and adult. So that this study is to analyze the infant's operation and to recognize the tendency and problems of infant's anesthesia and also to improve the outcome. METHODS: A retrospective analysis was performed on 1436 infants who had operations from april 1987 to february 1995 according to age, sex, department, disease, operation time, postoperative complications, mortality rate and anesthetic method. RESULTS: Total number of operation tend to increase annually. The male to female ratio was 67.5% to 32.5%. The distribution of patients by department was 60.6% of general surgery, 21.0% of plastic surgery and small percentage of other department. The distribution of disease was the following : inguinal hernia - the most common; cleft lip and palate, intussusception and others. The postoperative complications developed in 98 of 1436. The most common complication was the respiratory problems, the others were sepsis, electrolyte unbalance, gastrointestinal and CNS problems. The total mortality rate was 3.6%. The emergency operation case was 17.9%. CONCLUSIONS: The most common distribution of age was neonate and the most commm department was general surgery and the most common disease was inguinal hernia. The most common postoperative complication was respiratory problems and the second was infection and sepsis. The mortality rate in preterm infant, neonate, emergency operation and long duration operation was higher than total motality rate.
Adult ; Anesthesia* ; Child ; Cleft Lip ; Emergencies ; Female ; Hernia ; Hernia, Inguinal ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intussusception ; Male ; Mortality ; Palate ; Pharmacology ; Physiology ; Postoperative Complications ; Psychology ; Retrospective Studies* ; Sepsis ; Surgery, Plastic