No abstract available.
Glycogen Storage Disease* ; Glycogen*

No abstract available.
Accidents, Traffic* ; Fistula*

Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

Priapism is usually classified into two types, high flow and low flow, by the intracavernosal blood gas analysis. These two types of priapism is categorized pathophysiologically and hemodynamically; therefore, different treatments were performed according to the type. We report a case with recurrent idiopathic priapism, which was diagnosed and managed as low flow priapism at first. But finally it changed to a high flow priapism confirmed by blood gas analysis and further duplex ultrasonic study. During treatment, it was very difficult to cure the ocmplicated infection because of the necrosis of the cavernosal tissue.
Blood Gas Analysis ; Necrosis ; Priapism* ; Ultrasonics ; Wound Infection

PURPOSE: Leigh syndrome is a kind of mitochondrial disease with a pathological production of lactate and variable clinical manifestations. Recently biochemical assay of the mitochondrial enzyme activity and MRS are becoming a powerful tool for the definitive antemorterm diagnosis of this disease. The aim of this study was designed to determine the usefulness of biochemical enzyme assay and MRS in Leigh syndrome. METHODS: A clinical study of 8 cases of pediatric patients were diagnosed as Leigh syndrome on the basis of clinical manifestations and MRI features at the department of pediatrics, Ulsan university hospital, from July, 1992 to June, 1997. Biochemical enzyme assay of the cultured skin fibroblasts was consulted to hospital for sick children, Toronto, Canada. Cerebral lactates were detected on MRS were analysed in relation to the MRI findings and lactate levels in the CSF. RESULTS: 1) Age at diagnosis ranged from 7 months to 8.9 years; seven were boys and one was girl. 2) Main clinical symptoms were as follows in the order of frequency; seizure(75%), ophthalmoplegia(50%), hemiplegia(50%), hypotonia(50%), respiratory difficulty(50%), developmental delay(38%), consciousness change(38%), mental retardation( 25%). 3) Anatomical locations of the focal lesions detected on the initial MRI were as follows in the order of frequency; putamen(75%), caudate nuclei(63%), medulla oblongata (18,50%), substantia nigra(38%), thalamus(38%). 4) Biochemical enzyme assay revealed deficiency of NADH cytochrome c reductase (complex I) and deficiency of cytochrome c oxidase(complex N) respectively in 2 patients who had markedly elevated serum and CSF lactate levels and lactate/pyruvate ratio. 5) Cerebral lactates were detected by MRS from 6 lesions of 1 week-4 months old in 6 patients who had normal or mildly elevated serum and CSF lactate levels and lactate/pyruvate ratio. CONCLUSION: MRS detected the brain parenchymal lactate non-invasively from the acute or subacute lesions. Therefore, combined evaluation by MRS and biochemical enzyme assay will provide an important information for the diagnosis and estimation of disease activity in pediatric patients who are suspected to have Leigh syndrome.
Brain ; Canada ; Child ; Consciousness ; Cytochromes c ; Diagnosis ; Enzyme Assays* ; Female ; Fibroblasts ; Humans ; Lactates ; Lactic Acid ; Leigh Disease* ; Magnetic Resonance Imaging ; Medulla Oblongata ; Mitochondrial Diseases ; NADH Dehydrogenase ; Pediatrics ; Skin ; Ulsan

PURPOSE: To study the effect of ceftezole Na on the matrix of femoral epiphyseal plates. MATERIALS AND METHODS: Rats were intraperitoneally injected with ceftezole Na (1500 mg/kg B.W) was every day for a week. The specimens obtained from the femoral head were stained with alcian blue (pH 2.5)-P.A.S. to detect the distribution of acid and neutral mucopolysaccharides, and ultrastructural studies for growth plates were also performed. RESULTS: We found that interterritorial and territorial matrices of treatment groups were weakly stained than that of control groups and dilatation and sacculation were observed in rough endoplasmic reticulums in the chondrocyte of the treated group. CONCLUSIONS: According to the histochemical and ultrastructural research, ceftezole Na induces a decrease in mucosubstances in the chondrocytes of growth plate.
Alcian Blue ; Animals ; Cephalosporins* ; Chondrocytes ; Dilatation ; Endoplasmic Reticulum, Rough ; Glycosaminoglycans ; Growth Plate* ; Head* ; Rats*

Effects of melatonin on hepatic glutathione-peroxidase (GSH-Px) and glutathione-reductase (GSH-reductase) activities were studied in Sprague-Dawley (SD) rats administered i.p. (10 mg/kg body weight) with melatonin during 15 days. The activity of cytosolic GSH-reductase in the liver was not changed by melatonin. However, melatonin injection increased significantly the activity of liver cytosolic GSH-Px activity compared with those in saline-treated rats. At the same time, plasma GSH-Px was also increased significantly in melatonin-treated rats. Since GSH-Px, a major antioxidative enzyme, removes H-2O-2 and lipid peroxides which are formed during lipid peroxidation from cellular membrane, such elevation of heptatic GSH-Px activity may contribute to the improvement of antioxidative effects under oxidative damage in the liver.
Animals ; Cytosol ; Lipid Peroxidation ; Lipid Peroxides ; Liver ; Melatonin* ; Membranes ; Plasma ; Rats ; Rats, Sprague-Dawley*

BACKGROUND: Every practitioner, however skilled, will encounter patients who are unexpectedly difficult to intubation. The incidence of difficult laryngoscopy appears to be approximately 0.5% to 2%. So many methods are used to intubate the trachea, but endotraheal intubation by them is not always possible. The purpose of the this study is to evaluate the effectiveness of magnet on the endotracheal intubation. METHODS: Twenty patients(aged 30 to 59yr, ASA physical status 1) required general anesthesia with an endotracheal tube. Anesthesia was induced with thiopental sodium and succinylcholine, ventilation was controlled with 100% O2. The tip of the epiglottis was exposed wtih a No.3 MacIntosh laryngoscope. A catheter with ferrous stylet was placed behind epiglottis close to tracheal lumen and a magnet was placed over the cricoid cartilage allowing the stylet to be pulled. Endotraheal tube was guided into the trachea over the stylet. The time to intubation, the blood pressure and heart rate of pre- and postintubation, and the difficulty of intubation were recorded and the complications of the endotracheal intubation such as bronchial spasm, oral and tracheal mucosal trauma, and sore throat, were observed. RESULTS: The mean time to intubation was 31.2+/- 8(18 to 50)sec. There were statistically signigicant increase in blood pressure and heart rates following intubation. The incidence of excellent and good intubating condition were 14 and 6 respectively. Seventeen and three intubations were successful on the 1st and 2nd attempts respectively. There were no differences in incidence of complications of endotracheal intubation compared to other reports. CONCLUSIONS: Though magnet guided technique has some limitations to use in case of difficult laryngoscopy, it can be used with merits such as simplicity and cheapness when other methods are not available.
Anesthesia ; Anesthesia, General ; Blood Pressure ; Bronchial Spasm ; Catheters ; Cricoid Cartilage ; Epiglottis ; Heart Rate ; Humans ; Incidence ; Intubation* ; Intubation, Intratracheal ; Laryngoscopes ; Laryngoscopy ; Pharyngitis ; Succinylcholine ; Thiopental ; Trachea ; Ventilation

No abstract available.
Arteries* ; Atherectomy*

Pelvic actinomycosis is a chronic suppurative and granulomatous disease with multiple abscess and draining sinus tracts caused by Actinomyces, a Gram-positive, anaerobic, non-acid-fast bacterium. Pelvic actinomycosis is a rare disease, but it is more common with intrauterine device users than others. The symptoms and signs of pelvic actinomycosis are variable, from asymptomatic to mimicking the symptoms and signs of pelvic mass or acute peritonitis, and sometimes it is difficult to discriminate pelvic malignancy from actinomycosis. Identifying the typical "sulfur granule" with hematoxylin-eosin stain and detecting the actinomycotic colony in Gram stain may suggest the diagnosis. We have experienced a case of pelvic actinomycosis complicated by right tuboovarian abscess in a 39-year-old woman using intrauterine device and report it with brief review of literatures.
Abscess* ; Actinomyces ; Actinomycosis* ; Adult ; Diagnosis ; Female ; Humans ; Intrauterine Devices ; Peritonitis ; Rare Diseases