No abstract available.
Bowen's Disease* ; Carcinoma, Merkel Cell*

Truncus arteriosus is characterized by a single arterial vessel arising from the base of the heart and giving origin to the systemic, pulmonary and coronary circulation. To evaluate the incidence, types, and associated anomalies, 18cases of truncus arteriosus diagnosed by angiocardiography, were reviewed and were compared with other reports. Ten of them were confirmed by operation. The overall incidence of truncus arteriosus was 0.127%. Accordin to Collett and Edwards classification. Type I was encountered in 11cases(61%), type II in 4cases(22%), and type III in 3cases(17%), All the patients had subtruncal ventricular septal defects. Eight patients showed truncal valve regurgitation and valve stenosis was noted in one case. A right aortic arch was present in 9 cases. Associated anomalies were atrial septal defect(8cases), patent ductus arteriosus(3cases), coarctation of the aorta(2cases), isolation of the left subclavian artery(2 cases), and right aortic arch with an aberrant left subclavian artery(1case). Truncus arteriosus is an uncommon congenital cardio vascular malformation. In the group of cases which we encountered, type I was the most common anomaly. Frequently associated anomalies were right aortic arch, incompetent truncal valve and atrial septal defect.
Angiocardiography ; Aorta, Thoracic ; Classification ; Constriction, Pathologic ; Coronary Circulation ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence* ; Truncus Arteriosus* ; Vascular Malformations

We report a case of ciliated hepatic foregut cyst which was incidentally found in a 64 year-old man. The cyst, 6 cm in diameter, was unilocular, solitary and was located in the medial segment of left lobe, just below the Glisson's capsule. Microscopically, the cyst wall consisted of 4 layers; pseudostratified ciliated columnar epithelium, subepithelial loose connective tissue, smooth muscle bundles and an outermost fibrous capsule. Although cartilage or subepithelial sero-mucous glands were absent, the morphologic features of the cyst correspond with those of an incomplete form of brochogenic cyst.
Cysts

No abstract available.
Facial Asymmetry*

No abstract available.
Immunotherapy ; Melanoma*

Fibrotic lesions occurring in the corpus cavernosum are usually cases of Peyronie's disease that originate from the tunica albuginea, or they are the fibrotic result of inflammatory processes. The lesion involving the corpus cavernosum, but not tunica albuginea is rare. We present here a case of fibrotic nodule arising in the corpus cavernosum with the sonographic and magnetic resonance imaging features. A 38-year-old man complained a small nodular mass in the left corpus cavernosum at the level of penoscrotal junction without abnormal curvature of the organ. We performed ultrasonography and magnetic resonance imaging to determine exactly what the lesion was. The lesion was removed and it was pathologically found to be a localized fibrotic nodule of the corpus cavernosum with some narrow-channeled vascular structures.
Adult ; Fibrosis ; Humans ; Magnetic Resonance Imaging ; Male ; Penile Induration ; Penis ; Ultrasonography

BACKGROUND: In anesthesia for cesarean section, thiopental sodium is regarded as the standard induction agent. Propofol, 2,6 di-isopropyl phenol, is a relatively new intravenous anesthetic agent and has been used for induction and maintenance of general anesthesia or total intravenous anesthesia. Propofol has properties which suggest that it might be useful alternative to thiopental. METHODS: Forty patients (ASA physical status 1,2) scheduled for cesarean section were randomized to either propofol (n=20) or thiopental group (n=20). In thiopental group anesthesia was induced with thiopental 4-5 mg/kg intravenously and maintained by inhalation of enflurane and nitrous oxide. In propofol group anesthesia was induced with propofol 2 mg/kg intravenously and maintained by continuous infusion of propofol 6-10 mg/kg/hr and inhalation of nitrous oxide. RESULTS: Systolic and mean arterial pressure were increased significantly in both groups at 1 min after intubation, but degree of increase were less in propofol group. There was no significant difference in diastolic pressure in both groups. Heart rate was increased significantly in both groups at afterinduction, but degree of increase were less in propofol group. The Apgar scores of the neonates and blood gas analyses of umbilical vein were not significantly different in both groups. Maternal recovery from anesthesia was quicker in propofol group. CONCLUSIONS: A propofol infusion coupled with nitrous oxide was proved to be clinically satisfactory anesthesia for cesarean section with no adverse effect on both mother and fetus. Conclusively, propofol would be an excellant alternative to thiopental sodium and inhalation anesthetic in general anesthesia for cesarean section.
Anesthesia* ; Anesthesia, General ; Anesthesia, Intravenous ; Anesthetics ; Arterial Pressure ; Blood Gas Analysis ; Blood Pressure ; Cesarean Section* ; Enflurane ; Female ; Fetus ; Heart Rate ; Humans ; Infant, Newborn ; Inhalation ; Intubation ; Mothers ; Nitrous Oxide ; Phenol ; Pregnancy ; Propofol ; Thiopental* ; Umbilical Veins

BACKGROUND/AIMS: Gout is a common inf lammatory arthritis triggered by the crystallization of uric acid in the joints. Serum uric acid levels are highly heritable, suggesting a strong genetic component. Independent studies to confirm the genetic associations with gout in various ethnic populations are warranted. We investigated the association of polymorphisms in the ABCG2 and SLC2A9 genes with gout in Korean patients and healthy individuals. METHODS: We consecutively enrolled 109 patients with gout and 102 healthy controls. The diagnosis of gout was based on the preliminary criteria of the America College of Rheumatology. Genomic DNA was extracted from whole blood samples. We identified single nucleotide polymorphism (SNP) changes in the ABCG2 and SLC2A9 genes using a direct sequencing technique. rs2231142 in ABCG2 and rs6449213 and rs16890979 in SLC2A9 and nearby regions were amplified by polymerase chain reaction. RESULTS: Patients with gout had significantly higher A/A genotype (29.3% vs. 4.9%, respectively) and A allele (52.8% vs. 26.5%, respectively) frequencies of rs2231142 in ABCG2 than did controls (chi2 = 29.42, p < 0.001; odds ratio, 3.32; 95% confidence interval, 2.11 to 5.20). We found novel polymorphisms (c.881A>G and c.1002+78G>A) in the SLC2A9 gene. The univariate logistic regression analysis revealed that the c.881A>G and c.1002+78G>A SNPs were significantly higher in patients than in controls. CONCLUSIONS: We demonstrated a significant association between rs2231142 in the ABCG2 gene and gout and identified novel SNPs, c.881A>G and c.1002+78G>A, in the SLC2A9 gene that may be associated with gout in a Korean population.
ATP-Binding Cassette Transporters/*genetics ; Arthritis, Gouty/blood/diagnosis/ethnology/*genetics ; Asian Continental Ancestry Group/genetics ; Biomarkers/blood ; Case-Control Studies ; Chi-Square Distribution ; Gene Frequency ; Genetic Association Studies ; Genetic Predisposition to Disease ; Glucose Transport Proteins, Facilitative/*genetics ; Haplotypes ; Humans ; Logistic Models ; Neoplasm Proteins/*genetics ; Odds Ratio ; Phenotype ; *Polymorphism, Single Nucleotide ; Republic of Korea ; Risk Factors ; Uric Acid/blood

The incidence of systemic lupus erythematosus (SLE) is known to be affected by sex hormone. Patients with Klinefelter's syndrome were reported to have abnormal sex hormonal metabolism and their chronic estrogenic stimulation seems to affect the pathogenesis of SLE. Therefore, association of SLE and Klinefelter's syndrome has been considered as a clue of the effect of sex hormone on SLE. We report the first case of Klinefelter's syndrome in a patient with SLE in Korea and discuss the association of SLE with Klinefelter's syndrome.
Estrogens ; Humans ; Incidence ; Klinefelter Syndrome* ; Korea ; Lupus Erythematosus, Systemic* ; Metabolism

BACKGROUND: Longitudinal melanonychia is characterized by pigmented bands on the nail plate. It is not unusual to encounter pediatric melanonychia patients in the dermatologic clinic. OBJECTIVE: To assess the clinical manifestations of melanonychia in childhood in Korea, we reviewed the pediatric patient registry of a tertiary referral hospital in Korea. METHODS: Patients under fifteen years of age with longitudinal melanonychia referred between January, 2001, and March, 2015, were enrolled in the study. All patients had clinical as well as photo records. Clinical characteristics including age, sex, location, disease duration, width and color of pigmentation, morphological change over time, underlying disease, and dermoscopic images or biopsy findings were evaluated where available. RESULTS: A total of 92 patients had 158 pigmented nails, with more than one melanonychia identified in 14 patients. The patient sample included 43 male and 49 female patients with an average age of 6.1 years at diagnosis. A follow-up examination was performed for 132 nails in 80 patients with a mean period of 27.8 months and morphological changes were identified in 61 nails. Changes included remission or aggravation of pigmentation. In 48% and 17% of patients, nail plate dystrophy and Hutchinson's sign were observed, respectively. Dermoscopic image analysis was available for 79 nails. A nail biopsy was performed for 8 nails and none showed evidence of malignant melanoma. CONCLUSION: Pediatric longitudinal melanonychia generally presents a benign nature despite overt morphological changes, diffuse pigmentation, or Hutchinson's sign. Characteristically, melanonychia in childhood is frequently accompanied by nail dystrophy. Based on clinical reference of our study, aggressive nail biopsy may not be routinely necessary in pediatric melanonychia.
Biopsy ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Korea ; Male ; Melanoma ; Pigmentation ; Tertiary Care Centers