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

PURPOSE: We investigated the operative outcome after bypass surgery in patients selected using viability criteria on F-18 FDG PET. MATERALS AND METHODS: Rest-24hr delay redistribution imaging of Tl-201 SPECT and F-18 FDG PET were performed in 11 patients. Seven of these 11 patients (6 men, 1 woman) were evaluated to have viable myocardium by F-18 FDG PET. Changes in symptoms and left ventricular ejection fraction (LVEF) after operation were evaluated. RESULTS: In seven of 11 patients, a significant amount of viable myocardium was found on F-18 FDG PET and Tl-201 SPECT. Severity of both chest pain and dyspnea improved markedly in all patients. Mean LVEF improved from 22% to 32%. CONCLUSION: F-18 FDG PET could be used to select the patients who will benefit from coronary artery bypass surgery.
Cardiomyopathies* ; Chest Pain ; Coronary Artery Bypass ; Dyspnea ; Humans ; Male ; Myocardium ; Positron-Emission Tomography ; Stroke Volume ; Tomography, Emission-Computed, Single-Photon

The Iinvolvement of subaortic structures in the aortic valve endocarditis appears more commonly than previously recognized. These subaortic complications are most commonly located in the mitral-aortic intervalvular fibrosa and may be presented as abscess, or as pseudoaneurysm with or without perforation. Perforated pseudoaneurysm can lead to the development of communication between the left ventricular outflow tract and various cardiac chambers, most commonly the left atrium. These complications are related with poor prognosis. Early and precise recognition of these complications is important for optimal treatment. At present, transesophageal echocardiography (TEE) has been validated as the technique of choice. We describe a case of infectious pseudoaneurysm of mitral-aortic intervalvular fibrosa featuring the connection of the fistulous simultaneously to the left atrium and aorta. In our case, accurate interpretation of TEE imaging revealing the subaortic structures was not so easy due to interference of both aortic and mitral prosthetic valves. We expect the further development of (Ed-confirming that here you don't intend, "We expect to further develop") TEE and other imaging modalities to substantially improve the future diagnosis of these undesirable complications.
Abscess ; Aneurysm, False* ; Aorta ; Aortic Valve* ; Diagnosis ; Echocardiography, Transesophageal ; Endocarditis* ; Fistula* ; Heart Atria ; Prognosis

The case of a 33-day-old boy with Pierre Robin syndrome using a Cook(R) airway exchange catheter in laryngeal mask airway-guided fiberoptic intubation is presented. After induction with sevoflurane, classical reusable laryngeal mask airway (LMA) #1 was inserted and ultrathin fiberoptic bronchoscope (FOB) was passed through. A Cook(R) airway exchange catheter (1.6 mm ID, 2.7 mm OD) was passed through the LMA under the guidance of the FOB but failed to enter the trachea despite many trials. Then, an endotracheal tube (3.0 mm ID) was mounted on the FOB and railroaded over the FOB. After successful intubation, the Cook(R) airway exchange catheter was placed in the midtrachea through the lumen of the endotracheal tube. Even though the tracheal tube was accidentally displaced out of the trachea during LMA removal, the endotracheal tube could be easily railroaded over the airway exchange catheter.
Bronchoscopes ; Catheters ; Humans ; Infant, Newborn ; Intubation ; Laryngeal Masks ; Methyl Ethers ; Pierre Robin Syndrome ; Railroads ; Songbirds ; Trachea

PURPOSE: We studied the differences in the antibiotic susceptibilities of the microorganisms that causeing urinary tract infections (UTI) in children to obtain useful information on appropriate drug selection for childhood UTI. METHODS: We retrospectively analyzed the antibiotic susceptibilities of 429 microorganisms isolated from 900 patients diagnosed with UTI in the Department of Pediatrics, Chungbuk National University Hospital, from 2003 to 2008. RESULTS: The most common causative microorganisms for UTI were Escherichia coli (81.4%), Klebsiella pneumoniae (8.4%), Enterobacter spp. (1.7%), and Proteus spp. (0.4%). E. coli showed relatively high susceptibility as compared to imipenem (100%), amikacin (97.7%), aztreonam (97.9%), cefepime (97.7%), and ceftriaxone (97.1%), while it showed relatively low susceptibility to gentamicin (GM) (79.0%), trimethoprim/sulfamethoxazole (TMP/SMX) (68.7%), ampicillin/sulbactam (33.0%), and ampicillin (AMP) (28.6%). There were no significant differences in the image findings for causative microorganisms. CONCLUSION: Gram-negative organisms showed high susceptibility to amikacin and third-generation cephalosporins, and low susceptibility to AMP, GM, and TMP/SMX. Therefore, the use of AMP or TMP/SMX as the first choice in empirical and prophylactic treatment of childhood UTI in Korea should be reconsidered and investigated further.
Amikacin ; Ampicillin ; Aztreonam ; Ceftriaxone ; Cephalosporins ; Child ; Enterobacter ; Escherichia coli ; Gentamicins ; Humans ; Imipenem ; Klebsiella pneumoniae ; Korea ; Pediatrics ; Proteus ; Retrospective Studies ; Urinary Tract ; Urinary Tract Infections

Sinus of Valsalva aneurysms are rare cardiac anomalies and are usually caused by the separation of the aortic wall media from the valve ring tissue. These aneurysms frequently rupture into the low-pressure areas like the right ventricle and right atrium, rarely do they rupture into the left atrium, left ventricle, pericardial sac, or pulmonary artery. Cerebral infarction has been reported as a rare complication of unruptured sinus of Valsalva aneurysm. We experienced very rare two cases of Valsalva aneurysms of right coronary sinus dissecting into the interventricular septum in patients with cerebral infarction. In two cases these aneurysms ruptured into the left ventricle. These aneurysms were excised and the defect was closed with autopericardium. At the end of the surgical repair, coaptation was found to be insufficient and aortic valve replacement was undertaken.
Aneurysm* ; Aortic Valve ; Cerebral Infarction* ; Coronary Sinus ; Heart Atria ; Heart Ventricles ; Humans ; Pulmonary Artery ; Rupture ; Sinus of Valsalva*

No abstract available.
Arteries* ; Atherectomy*

AIMTo evaluate the effects of 60 Hz extremely low frequency (ELF) elelctromagnetic field (EMF) exposure on germ cell apoptosis in the testis of mice.

METHODSAdult male BALB/c mice (7 weeks of age) were exposed to a 60 Hz EMF of 0.1 mT or 0.5 mT for 24 h/day. A sham-exposed group served as the control. After 8 weeks of exposure, the mice were sacrificed. Germ cell apoptosis in the testis was assessed by histopathological examination, the terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling assay (TUNEL) and flow cytometric examination of isolated spermatogenic cells stained with 7 aminoactinomycin D (7-AAD).

RESULTSEMF exposure did not significantly affect the body and testis weights, but significantly increased the incidence of germ cell death. The distinguishing morphological feature of EMF exposure was a decrement in the number of well organized seminiferous tubules. Quantitative analysis of TUNEL-positive germ cells showed a significantly higher apoptotic rate in the 0.5 mT exposed mice than that in the sham controls (P<0.05), while the difference between the two exposed groups was insignificant. The TUNEL-positive cells were mainly spermatogonia. In flow cytometry analysis, the percentage of live cells [forward scatter count (FSC)(high)7-AAD(-)] was lower in the exposed groups than that in the controls (Figure 5A), but the decrease in viability was not statistically significant.

CONCLUSIONContinuous exposure to ELF EMF may induce testicular germ cell apoptosis in mice.

Animals ; Apoptosis ; Body Weight ; Electromagnetic Fields ; adverse effects ; Flow Cytometry ; In Situ Nick-End Labeling ; Male ; Mice ; Mice, Inbred BALB C ; Organ Size ; Spermatozoa ; cytology ; Testis ; cytology

Coronary artery and valvular injuries after blunt chest trauma are an unusual condition. This diagnosis is very difficult to estabilish, but prompt diagnosis and proper management are important in life saving. We report one patient who develop left main coronary artery dissection, tricuspid insufficiency, mitral insufficiency and pericardial rupture following blunt chest trauma. One year ago, he had suffered a frontal impact in a traffic accident and recieved anti-tuberculosis medication for 10 months for chest discomfort. The correct diagnosis was confirmed noninvasively by transesophageal echocardiography and the patient was treated left main coronary artery dissection flap removal, mitral valve replacement, tricuspid valvuloplasty and repair of ruptured pericardium. The postoperative course was uneventful and the patient was fully recovered.
Accidents, Traffic ; Coronary Vessels* ; Diagnosis ; Echocardiography, Transesophageal ; Humans ; Mitral Valve ; Mitral Valve Insufficiency* ; Pericardium ; Rupture* ; Thorax*

To evaluate the results of cardiac anesthesia during open heart surgery using cardiopulmonary bypass, the cases of 100 patients from December 1986 to May 1989 were reviewed. The results were as follows: 1) There were 56 patients with congenital heart disease (male 32, female 24) and 44 patients with acquired heart disease (male 14, female 30). 2) The mean age of patients with congenital heart disease was 17.0+/-10.0 years and the mean age of patients with acquired heart disease was 41.2+/-14.6 years. 3) There were 54 cases of acyanotic heart disease and 2 cases of cyanotic heart disease among congenital heart disease, and 41 cases of valvular heart disease. 4) Glycopyroolate and hydroxyzine were mostly used as premedicants and morphine was used in some cases of acquired heart disease. 5) Anesthesia was induced by morphine and diazepam mostly while ketamine was used in some congenital heart disease. 6) The periods of cardiopulmonary bypass and aortic cross clamp in acquired heart disease were twice as long as in congenital heart disease. 7) The lowest body temperature during cardiopulmonry bypass was 26.7+/-2.5 degrees C in congenital heart disease and 24.1+/-3.6 degrees C in acquired heart disease while mean arterial pressure was maintained between 50-80 mmHg. 8) There were 33 cases of complications in 19 patients in which wound infection and arrythmia were most predominant. Four fatalities resulted from 3 cases of low cardiac output syndrome and 1 case of cerebrovascular disease.
Anesthesia* ; Arrhythmias, Cardiac ; Arterial Pressure ; Body Temperature ; Cardiac Output, Low ; Cardiopulmonary Bypass ; Diazepam ; Female ; Heart Defects, Congenital ; Heart Diseases ; Heart Valve Diseases ; Heart* ; Humans ; Hydroxyzine ; Ketamine ; Morphine ; Thoracic Surgery* ; Wound Infection