No abstract available.
Kasabach-Merritt Syndrome*

The hereditary sensory neuropathy is a very rare disease characterized by prominent sensory loss without corresponding motor involvement, but may be associated with autonomic features. Currently, the disease is divided into five main types and most frequent are Type I and Type II. The type II hereditary sensory neuropathy is characterized by autosomal recessive inheritance, onset in utero or in infancy, loss of touch-pressure sense more than paintemperature sense, and almost total absence of myelinated nerve fibers. In this case, we describe a 23 years old female patient with acroosteolysis and heel ulcer who was diagnosed as hereditary sensory neuropathy type II.
Acro-Osteolysis* ; Female ; Heel ; Hereditary Sensory and Autonomic Neuropathies* ; Humans ; Nerve Fibers, Myelinated ; Rare Diseases ; Ulcer ; Wills ; Young Adult

No abstract available.
Peripheral Nervous System Diseases* ; Scleroderma, Systemic*

Electronic health records (EHR) hold promise in improving the quality and efficiency of health care, yet the health care system remains years behind other industries with respect to the adoption of information technology (IT). Stakeholders in the medical community, including government and tertiary hospitals, have emphasized the urgent need to adopt IT systems. This paper reviews the current research and development efforts related to EHR in Korea, which have been supported by the government since December 2005, on the basis of the following core EHR components: EHR architectures that incorporate an external, implementation-independent view of a complete EHR; EHR functionality for defining tasks that an EHR system should perform; semantic ontology for developing standard vocabularies at the national level; EHR messaging standards for exchanging data; and clinical decision support systems for improving patient safety. Recent international work on EHR systems and the underlying trends are described, and suitable directions for research and development are suggested under relevant subtopics. Advances in the relevant areas will greatly facilitate our ability to achieve interoperability and promote patient safety. However, EHR systems will perform optimally only if we improve our understanding of the political, structural, and technical foundations for EHR, and reach consensus via collaborations between all the stakeholders in the health care system.
Consensus ; Cooperative Behavior ; Decision Support Systems, Clinical ; Delivery of Health Care ; Electronic Health Records* ; Foundations ; Health Level Seven ; Humans ; Korea ; Patient Safety* ; Semantics ; Tertiary Care Centers ; Vocabulary

We report our initial experience of a laparoscopic nephron sparing surgery through retroperitoneal and transperitoneal approaches. One patient with a renal tumor for each approach underwent operations. Cooling technique was not used in either cases and an occlusion of the renal artery with laparoscopic bulldog clamp was performed in the second case. Complete removal of the renal tumor was achieved in both cases. Bleeding control and repair of the collecting system with watertight sutures were successful. The operative time was 2 h and 40 min, 4 h and 5 min in each, warm ischmic time was 15 min in the second case. The hospital stay was for 4 and 8 days. The final pathologic evaluation was for lipoma and renal cell carcinoma (3.5x3.5cm in size) with a negative surgical margin. A laparoscopic nephron sparing surgery is quite feasible and might be offered as an advantage over open surgery with regards to the length of hospital stay, convalescence time and cosmetic results with more experience. But, further long term follow-up for laparascopic nephron sparing surgery might be necessary.
Carcinoma, Renal Cell ; Convalescence ; Follow-Up Studies ; Hemorrhage ; Humans ; Kidney ; Kidney Neoplasms ; Laparoscopy ; Length of Stay ; Lipoma ; Nephrectomy ; Nephrons* ; Operative Time ; Renal Artery ; Sutures

DCRV is a rare cause of right ventricular outflow obstruction by anomalous muscle bundle at the level of sinus portion. The symptoms due to DCRV itself were mild inspite of significant pressure gradient over 90mmHg between proximal and distal chamber, and became severe by associated complication of bacterial endocarditis or aortic regurgitation in ventricular septal defect. In 12 cases(40%), right ventricular hypertrophy configuration in electrocardiogram was characteristic. Although R wave in V1 was tall, S wave in V6 and R wave in aVR were indistinctive. Those were very useful findings for diagnosis of DCRV which was thought to be possible only invasively. With operation prior to overt right ventricular faliure, the prognosis was good with only minor complication.
Adolescent* ; Adult* ; Aortic Valve Insufficiency ; Diagnosis ; Electrocardiography ; Endocarditis, Bacterial ; Heart Septal Defects, Ventricular ; Humans ; Hypertrophy, Right Ventricular ; Prognosis ; Ventricular Outflow Obstruction

BACKGROUND: Carotid atherosclerosis has been known to be associated with systemic inflammatory status. The present study aimed to investigate the relationship between hepatitis viral infection or vaccination and carotid atherosclerosis in a relatively healthy population. METHODS: A cross-sectional study was performed in 281 subjects (mean age+/-SD, y; 43.8+/-7.2) in the Chonbuk national university hospital. All the participants were examined for the carotid intima-media thickness (IMT) in both common carotid, carotid bulb, and internal carotid arteries. Hepatitis B surface antigen (HBsAg) and IgG antibodies against hepatitis B and C virus (anti-HBs and anti-HCV) were determined by enzyme linked immunosorbent assays. RESULTS: Twelve subjects (4.3%) were HBsAg seropositive and 6 (2.1%) were anti-HCV positive but the positivity did not affect the mean carotid IMT. However, the hepatitis B-exposure group including both the HBsAg positive and anti-HBs positive without vaccination history showed a significantly higher carotid IMT (mean+/-SD, mm; 0.757+/-0.107 vs. 0.728+/-0.105, P=0.031), even after adjusting for the potential confounders. And, in the subgroup having anti-HBs, the carotid IMT was lower in the hepatitis B vaccinated subjects than in the others (0.725+/-0.103 vs. 0.760+/-0.111, P=0.019). CONCLUSIONS: Subjects exposed to the hepatitis B pathogen, even though they had anti-HBs, showed the higher carotid IMT, and the participants with a vaccination history demonstrated the lower IMT values. Subsequent study in a large representative population might be needed to further delineate the characteristic associations.
Antibodies ; Carotid Artery Diseases ; Carotid Artery, Internal ; Carotid Intima-Media Thickness* ; Cross-Sectional Studies ; Hepatitis B ; Hepatitis B Surface Antigens ; Hepatitis* ; Immunoglobulin G ; Jeollabuk-do ; Vaccination

BACKGROUND AND OBJECTIVES: This study was intended to test how the inflammation at the Bacille Calmette-Guerin (BCG) inoculation site (BCGitis) can be a useful a diagnostic feature of Kawasaki disease (KD). SUBJECTS AND METHODS: All subjects were infants at the time of admission, and had received BCG vaccination during their neonatal period. There were 54 patients with complete KD (group 1) and 29 patients with incomplete KD (group 2). All 83 patients had BCGitis during the acute phase of illness. Data regarding the coronary artery diameters in 31 age-matched controls were used for comparison. RESULTS: The 2 patient groups did not differ in clinical and laboratory variables. During the acute phase, the median z scores of the left anterior descending coronary artery (LAD) diameter were 0.20, 0.42, and -0.48 in groups 1, 2, and control respectively, and that of right coronary artery (RCA) diameters were -0.15, -0.16, and -1.17 respectively. The z scores in both patient groups were greater than those in controls (p=0.0014 in LAD and p<0.0001 in RCA between group 1 and controls; p=0.0023 in LAD and p<0.0001 in RCA between group 2 and controls). A similar pattern was observed during the subacute and convalescent phases. CONCLUSION: BCGitis is a useful feature in the diagnosis of incomplete KD in infants who received BCG vaccine during neonatal period.
BCG Vaccine ; Coronary Vessels ; Humans ; Infant ; Inflammation ; Mucocutaneous Lymph Node Syndrome ; Mycobacterium bovis ; Vaccination

OBJECTIVES: The treadmill exercise eletrocardiography(ECG) is the most commonly used non-invasive method in the evaluation of patients with chest pain. But the accuracy of treadmill exercise ECG in detecting the coronary artery disease(CAD) is still controversial. To improve the accuracy of the treadmill exercise test, exercise treadmill score(ETS) based on exercise duration, degree of ST deviation, and treadmill anginal index during treadmill exercise ECG has been used. METHODS: The authors calculated ETS by simple equation(total exercise duration-5 X maximal ST-segment deviation during or after exercise-4 X treadmill angina index) and analyzed coronary angiograms of 173 patients(mean age '55.5 +/- 8.7, male: female=2.7: 1) who underwent treadmill exercise ECG and coronary angiography in Chonnam University Hospital from January, 1990 through March, 1993. RESULTS: 1) The studied subjects were subdivided into 3groups according to ETS. Group A(high risk, ETSETS>or=11) 71cases(mean age 60.2 +/- 7.4, male: female=3.3:1), group C(low risk, ETS>5) 87cases(mean age 54.8 +/- 9.2, male-female =2.5:1). Clinical diagnoses of the studiedsubjects were 63stable angina, 61unstable angina, 3acute myocardial infarction, and 46 old myocardial infarction. On coronary angiographic findings, 61patients had single vessel disesase, 23patients had two vessel disease and 13patients had three vessel disease. 2) The sensitivity of the treadmill exercise ECG in diagnosing coronary artery disease was 88% and the specificity was 46%. 3) One hundred percent of group A patients had CAD and 54% of them had multivessel disease, 75% of group B had CAD and 27% of them had multivessel disease, and 33% of group C had CAD and 10% of them had multivessel disease. 4) There were no significant differences in the siite of stenotic lesion and degree of stenosis according to ETS in the patients with single vessel disease. 5) There were no significant differences in left ventricular ejection fraction and left ventricular end-diastolic pressure among three groups. CONCLUSION: Exercise treadmill score is useful in predicting the presence and severity of CAD and that low ETS less than -11 may be an indicator of multivessel coronary disease.
Chest Pain ; Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Disease* ; Coronary Disease ; Coronary Vessels* ; Diagnosis ; Electrocardiography ; Exercise Test ; Female ; Humans ; Jeollanam-do ; Male ; Myocardial Infarction ; Sensitivity and Specificity ; Stroke Volume

BACKGROUND: Catheter ablation of accessory pathways using radiofrequency(RF) energy was recently introduced to cure accessory pathway related tachyarrhythmias. The purpose of the present study was to evaluate the efficacy and safety of radiofrequency catheter ablation of accessory pathways and to determine factors influencing the outcome of catheter ablation. METHODS: Electrophysiology study was performed with standard technique and catheter ablation of accessory pathways using RF and conventional ablation technique. The outcome of RF catheter ablation were evaluated according to the location and the overtness of accessory pathways. Eighty patients(Mean +/-SD age, 36+/-15 years ; 50 male, 30 female)comprising 49(61%) with Wolff-Parkinson-White(WPW) syndrome and 31(39%) with atrioventricular reentry tachycardia(AVRT) using concealed bypass tract underwent RF catheter ablation for total of 85 accessory pathways in the Chonnam University Hospital. Five(6.3%) patients had multiple accessory pathways. RESULTS: Seventy-nine(92.9%) out of 85 pathways and all the pathways in 75(93.8%) out of 80 patients were ablated successfully. The success rate showed no significant difference between patients with overt accessory pathways and patients with concealed accessory pathways(92.0% vs. 93.9%). However, the success rate in right free wall location(72.7% of 11) was significantly lower than that in the other sites (95.9% of 74, p<0.05). More attempts were tried to ablate right-sided accessory pathway than left-sided pathways(6.4+/-24.1 vs. 3.8+/-6.8, p<0.05). Three(3.8%) pathways recurred within 30 minutes after the initial successful ablation. Four(5.1%) pathways recurred from 16 hours to 7 months after completion of the initial successful ablation session during the mean follow-up period of 43+/-24 weeks(range, 2-84 weeks). This late recurrence was more frequent, although statistically insignificant, in right-sided accessory pathways(11.1% vs. 3.3%, p=0.22). All 4 recurrent pathways(1 at the same session, 3 at the repeated sessions) reattempted for ablation were successfully ablated. As procedure-related complications, second degree AV block developed in a patients with mid septal and posteroseptal pahways and hemopericardium in a patients with a left anterolateral pathway. CONCLUSION: RF catheter ablation of atrioventricular accessory pathways is very effective and safe, with a success rate of 93.8% and a complication rate of 2.5%. Right-sided accessory pathways are more difficult to ablate than left-sided accessory pathways, requiring the development of a better technique for right free wall pathways.
Ablation Techniques ; Atrioventricular Block ; Catheter Ablation* ; Catheters* ; Electrophysiology ; Follow-Up Studies ; Humans ; Jeollanam-do ; Male ; Pericardial Effusion ; Recurrence ; Tachycardia