Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by concurrent nevus flammeus (capillary malformation) and pigmentary nevus. According to current research, the major pathophysiologic mechanism in PPV is venous dysplasia with resultant compensatory collateral channels and venous hypertension. Arterial involvement is rare. We herein report our experience on renovascular hypertension, intermittent claudication, and severe rhabdomyolysis due to diffuse stenosis of multiple arteries in a patient with PPV type IIb associated with SWS.
Arteries ; Constriction, Pathologic ; Humans ; Hypertension ; Hypertension, Renovascular ; Intermittent Claudication ; Intracranial Aneurysm ; Neurocutaneous Syndromes* ; Nevus ; Port-Wine Stain ; Rhabdomyolysis* ; Sturge-Weber Syndrome* ; Vascular Diseases

STATEMENT OF PROBLEM: Little information is available about the buccal gingival level of multiple implant restorations. PURPOSE: This study was aimed to evaluate the relationship between width and height of buccal soft tissue around single and 2 adjacent implant restorations. MATERIAL AND METHODS: Four implant restoration groups (first and second molars, single second molars, posterior single restorations between teeth, and anterior single restorations between teeth) were randomly chosen from one dental institute. Each group comprised of 6 patients. After 6 months of function, silicone impressions were taken and stone models were fabricated for each restoration group. The stone models were cut in bucco-lingual direction at the most apical point of buccal gingival margin. The height and width of buccal supra-implant soft tissue were measured. One way ANOVA and Tukey HSD post hoc tests were performed to analyze the data obtained (P < .05). RESULTS: The most unfavorable width-height ratio was noted for the group, which was comprised of the second molar in the multiple adjacent (first and second molar) implant-supported restorations. The group also resulted in the shorter height of buccal supra-implant mucosa rather than that of anterior single implant restorations between natural teeth. CONCLUSION: To achieve a favorable level of buccal gingival margin, greater thickness of buccal supra-implant mucosa is required for the implant restorations without a neighboring natural tooth compared to the implant restorations next to a natural tooth.
Humans ; Molar ; Mucous Membrane ; Silicones ; Tooth

Although the confirmative diagnosis of typhoid fever is by culture of the causative organism, usually from blood, a serological test is still necessary to provide a more rapid method of diagnosis. The indirect fluorescent antibody test, using a Salmonella typhi Vi antigen and a FITC-conjugated rabbit anti-human polyvalent immunoglobulin, was evaluated for the diagnosis of typhoid fever. Serum specimens were collected from patients with febrile diseases on admission. Of the 32 patients with titers of 1:64 or more, 22 were confirmed to have typhoid fever by blood culture and 7 had fever of undetermined origin that was considered to be typhoid fever clinically. Three patients were diagnosed to have salmonellosis other than typhoid fever. Of the 121 patients with titers of 1:32 or less, 105 patients had non-typhoidal febrile disease, 15 patients had fever of undetermined origin, and one patient was confirmed to have typhoid fever by blood culture. When a Vi antibody titer of 1:64 or more was taken as serological evidence for the diagnosis of typhoid fever, the sensitivity and specificity were 95.7% and 97.2%, respectively. The incidence of positive test results following fever onset was 70.0% within 1 week of fever onset, 88.9% from 1 to 2 weeks, and 100% after 2 weeks. In conclusion, the Vi-indirect fluorescent antibody test(Vi-IFAT) can be employed as a useful serologic test in the diagnosis of typhoid fever.
Antigens, Bacterial/*analysis ; Fluorescent Antibody Technique/*standards ; Human ; Salmonella typhi/immunology ; Sensitivity and Specificity ; Typhoid Fever/*diagnosis

OBJECTIVE: Chronic hepatitis B infection (CHB) occurs in 6% to 10% of population in Korea. In ethinic communities where prevalence of chronic infection is high such as Korea, transmission of hepatitis B infection is either vertical (ie, by perinatal infection) or by close family contact (usually from mothers or siblings) during the first 5 years of life. The development of chronic hepatitis B infection is increasingly more common the earlier a person is exposed to the virus, particularly in fetal and neonatal life. And it progress to cirrhosis and hepatocellular carcinoma, especially in severe liver damage and perinatal infection. Histopathology of CHB is important when evaluating the final outcomes. A numerical scoring system which is a semiquantitatively assessed objective reproducible classification of chronic viral hepatitis, is a valuable tool for statistical analysis when predicting the outcome and evaluating antiviral and other therapies. In this study, a numerical scoring system (Ludwig system) was applied and compared with the conventional histological classification of De Groute. And the comparative analysis of cinical findings, family history, serology, and liver function test by histopathological findings in chronic hepatitis B of children was done. METHODS: Ninety nine patients [mean age=9 years (range=17 months to 16 years)] with clinical, biochemical, serological and histological patterns of chronic HBV infection included in this study. Five of these children had hepatocelluar carcinoma. They were 83 male and 16 female children. They all underwent liver biopsies and histologic evaluation was performed by one pathologist. The biopsy specimens were classified, according to the standard criteria of De Groute as follows: normal, chronic lobular hepatitis (CLH), chronic persistent hepatitis (CPH), mild to severe chronic active hepatitis (CAH), or active cirrhosis, inactive cirrhosis, hepatocellular carcinoma (HCC). And the biopsy specimens were also assessed and scored semiquantitatively by the numerical scoring Ludwig system. Serum HBsAg, anti-HBs, HBeAg, anti-HBe, anti-HBc (IgG, IgM), and HDV were measured by radioimunoassays. RESULTS: Male predominated in a proportion of 5.2 : 1 for all patients. Of 99 patients, 2 cases had normal, 2 cases had CLH, 22 cases had CPH, 40 cases had mild CAH, 19 cases had moderate CAH, 1 case had severe CAH, 7 cases had active cirrhosis, 1 case had inactive cirrhosis, and 5 cases had HCC. The mean age, sex distribution, symptoms, signs, and family history did not differ statistically among the different histologic groups. The numerical scoring system was correlated well with the conventional histological classification. The histological activity evaluated by both the conventional classification and the scoring system was more severe as the levels of serum aminotransferases were higher. In contrast, the levels of serum aminotransferases were not useful for predicting the degree of histologic activity because of its wide range overlapping. When the histological activity was more severe and especially the cirrhosis more progressing, the prothrombin time was more prolonged. The histological severity was inversely related with the duration of seroconversion of HBeAg. CONCLUSIONS: The histological activity could not be accurately predicted by clinical and biochemical findings, but by the proper histological classification of the numerical scoring system for the biopsy specimen. The numerical scoring system was correlated well with the conventional histological classification, and it seems to be a valuable tool for the statistical analysis when predicting the outcome and evaluating effects of antiviral and other therapies in chronic hepatitis B in children.
Biopsy ; Carcinoma, Hepatocellular ; Child* ; Classification* ; Female ; Fibrosis ; Hepatitis ; Hepatitis B ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Korea ; Liver ; Liver Function Tests ; Male ; Mothers ; Prevalence ; Prothrombin Time ; Sex Distribution ; Transaminases

No abstract available.
Humans ; Sarcoma, Kaposi*

Early defibrillation with an automated external defibrillator (AED) is crucial to survival success in sudden cardiac arrest. Dissemination of public-access AEDs have increased the frequency of early defibrillations and contributed to improved outcomes after out-of-hospital cardiac arrests (OHCAs). However, public-access AEDs are not yet widely-disseminated in Korea. We report the first survival case of an OHCA patient who received shocks from a public-access AED in Korea.
Death, Sudden, Cardiac ; Defibrillators ; Heart Arrest ; Humans ; Korea ; Out-of-Hospital Cardiac Arrest ; Shock

PURPOSE: The purpose of this study was to analyze the CT findings of hepatoblastoma before and afterchemotherapy, and to compare them with surgical and pathologic features. MATERIALS AND METHODS: Twelvehepatoblastoma patients underwent chemotherapy prior to surgery; in all cases, CT scanning was performed beforeand after chemotherapy. We reviewed the findings with special attention to changes in tumor volume, the extent andpattern of contrast enhancement, the extent of low-attenuation area in the tumor, the presence of a septum, andcalcification or ossification within the mass before and after chemotherapy. Post-chemotherapy CT findings werecompared with operative and pathologic findings. RESULTS: After chemotherapy, the volume of the tumor massdecreased in all patients, and the extent of involved segments decreased in nine(75%), the non-enhancing areawithin the mass, on the other hand, increased in nine (75%). On pre-chemotherapy CT, calcifications were detectedin seven patients(58%), and on post-chemotherapy CT, in nine (75%); the extent of calcification increased in sevenpatients. On the basis of CT findings, viable tumor and necrosis areas could not be distinguished. Massivecalcification or an osteoid mixed with loose connective tissue was noted in the mesenchymal component of thetumor; the whirling pattern of enhancement within the area of low density asen on CT scanning corresponded toosteoid mixed with loose connective tissue, which contained rich blood vessels. CONCLUSION: We describe the CTfindings of hepatoblastoma both before and after chemotherapy, highlighting the changes which occurred. Anunderstanding of these changes is helpful for the proper management of this condition.
Blood Vessels ; Connective Tissue ; Drug Therapy* ; Hand ; Hepatoblastoma* ; Humans ; Necrosis ; Tomography, X-Ray Computed ; Tumor Burden

PURPOSE: This study was aimed to evaluate the feasibility of echo-Doppler study on fetal lamb, analyse the distribution of blood flow before and after fetal cardiac bypass, and consequently assess the effect of fetal cardiac bypass on the fetal cardiovascular system. METHODS: Ten fetal lambs at 120 to 150 days of gestation which underwent cardiac bypass for 30 minutes were studied by echocardiography and Doppler study. Five fetuses survived after bypass. Blood flow volume was measured if possible in aorta, main pulmonary artery, ductus and branch pulmonary arteries before and after bypass. RESULTS: Echocardiographic evaluation was successful in 5 of 9 studied in utero, and all 3 exteriorized cases before bypass and all 5 post-bypass cases. Before bypass cardiac output and combined vascular resistance tended to increase according to body weight, but was not significant. After cardiac bypass, cardiac output decreased significantly(P<0.05). The ratios of main pulmonary artery to aorta blood flow volume were 1.40(1.16-1.58) at pre-bypass and 1.47(0.644- 2.34) at post-bypass(P>0.05). Combined vascular resistance was 188unit(92-340unit) at pre-bypass and 341 unit(128-533 unit) at post-bypass. There was no significant difference in combined vascular resistance in 3 cases of which both pre-bypass and post-bypass studies were completed. Doppler study demonstrated systolic forward flow and diastolic reverse flow in ductus and aorta after cardiac bypass. CONCLUSION: Echocardiography and Doppler study are feasible methods for the evaluation of blood flow after fetal cardiac bypass. This study suggests that cerebral and pulmonary vascular resistance may elevate less compared with that of placenta after cardiac bypass.
Aorta ; Body Weight ; Cardiac Output ; Cardiovascular System* ; Echocardiography ; Fetus ; Placenta ; Pregnancy ; Pulmonary Artery ; Vascular Resistance

A case of telangiectatic focal nodular hyperplasia (FNH) was detected at birth and was surgically removed. Grossly, the lesion was a solitary nodule and showed vague nodularity, appearing as an adenoma-like mass with fine fibrous septa, but having no macroscopic scar. On microscopic scale, the mass typically had neither fibrous central scar nor hyperplastic nodules different from the usual FNHs. The hepatic plates were separated by sinusoidal dilatation, sometimes alternating with areas of marked ectasia. Instead of large fibrous scar, thin fibrous septa were often found, and contained abnormal tortuous large arteries. These high-pressure vessels were connected directly into the adjacent sinusoids and made marked dilation of sinusoids. Bile ductular proliferation was also noted in the thin fibrous septa. To our knowledge, this is considered to be the first reported case of telangiectatic FNH detected at birth.
Focal Nodular Hyperplasia/*congenital/*diagnosis/radiography ; Human ; Hyperplasia ; Infant ; Liver/pathology ; Liver Neoplasms/congenital/*diagnosis/radiography ; Male ; Tomography, X-Ray Computed

PURPOSE: The purpose of our study was to review the perinatal clinical characteristics of extremely low birth weight(ELBW) infants and determine their risk factors of their deaths. METHODS: The medical records of 96 infants weighing less than 1,000g, who were born at Seoul National University Hospital and admitted to our neonatal intensive care unit(NICU) were analyzed retrospectively on the basis of clinical characteristics, obstetrical problems, postnatal complications and outcome. RESULTS: The annual birth rate of ELBW infants was 0.11% to 0.90% of total live births and the mean annual birth rate was 0.56% at Seoul National University Hospital. Preeclampsia was the most common obstetrical problem(34.4%), followed by incompetent internal os of cervix(IIOC) (13.5%), multiple pregnancy(13.5%) and in vitro fertilization(IVF)(13.5%). Respiratory distress syndrome(RDS) was the most common postnatal complication(78.1%), followed by sepsis(60.4%) and apnea(39.6%). Comparing the ELBW infants weighing less than 750g with those weighing more than 751g, sepsis was more frequent in the latter group(p<0.05), whereas high grade intraventricular hemorrhage(grade Ill) was more frequent in the former group(p<0.05). Otherwise there was no statistically significant difference concerning the frequency of perinatal complications between two groups. Comparing the ELBW infants born before the year 1991, when surfactant treatment started to be used routinely at our NICU, with those born after the year 1991, apnea and sepsis could be observed more frequent in the latter group(p<0.05). Otherwise there was no statistically significant difference in the frequency of perinatal complications between two groups, The survival rate of ELBW infants was 0.0% in 1986, 50.0% in 1991 and 40.0% in 1995. The risk factors of neonatal deaths of ELBW infants included birth weight, gestational period, mode of delivery, IIOC, RDS, apnea, pneumonia, pneumothorax, acute renal failure(ARF) and infections(except pneumonia), but analyzing these risk factors by multivariate logistic regression analysis, the resultant significant independent risk factors consisted only of birth weight, RDS and pneumonia(p<0.05). There was no statistically significant difference in survival rate between ELBW infants born before and after the year 1991, but there was a tendency toward increasing survival rates in the latter group. CONCLUSION: Recently, the survival rate of ELBW infants is improving steadily, but is still lower than that of western countries and postnatal morbidity remains high. Therefore, there is an urgent need to give more efforts to the neonatal intensive care of ELBW inFants in order to increase survival rates and reduce postnatal morbidity. But it is more important to reduce preterm birth by the treatment of preventable obstetrical risk factors.
Apnea ; Birth Rate ; Birth Weight ; Humans ; Infant* ; Infant, Extremely Low Birth Weight ; Infant, Low Birth Weight* ; Infant, Newborn ; Intensive Care, Neonatal ; Live Birth ; Logistic Models ; Medical Records ; Mortality ; Parturition ; Pneumonia ; Pneumothorax ; Pre-Eclampsia ; Premature Birth ; Retrospective Studies ; Risk Factors ; Seoul ; Sepsis ; Statistics as Topic* ; Survival Rate