No abstract available.
Bronchiectasis*

No abstract available.
Humans ; Spondylolisthesis*

The vagina is an infrequent site of primary sarcomas, which are less than 2% of all malignant vaginal neoplasm. The most common malignant mesenchymal tumor of the vagina is rhabdomyosarcoma, but leiomyosarcoma is rare type tumor of the vagina. Microscopically, spinle cells with pleomorphic nuclei and mitotic figures were frequently noted, but cross-striations were not found in the tissue. Alpha-smooth muscle actin and desmin were positive, but sarcomeric actin was negative in the tumor cells. The authors report a case of primary leiomyosarcoma of the vagina with brief literature review.
Actins ; Desmin ; Leiomyosarcoma* ; Rhabdomyosarcoma ; Sarcoma ; Vagina* ; Vaginal Neoplasms

PURPOSE: This study analyzed and evaluated the demographic, clinical, and cytogenetic data [G-banded karyotyping and array-based comparative genomic hybridization (array CGH)] of patients with unexplained developmental delay or intellectual disability at a single Korean institution. MATERIALS AND METHODS: We collected clinical and cytogenetic data based on retrospective charts at Ajou University Medical Center, Suwon, Korea from April 2008 to March 2012. RESULTS: A total of 190 patients were identified. Mean age was 5.1+/-1.87 years. Array CGH yielded abnormal results in 26 of 190 patients (13.7%). Copy number losses were about two-fold more frequent than gains. A total of 61.5% of all patients had copy number losses. The most common deletion disorders included 22q11.2 deletion syndrome, 15q11.2q12 deletion and 18q deletion syndrome. Copy number gains were identified in 34.6% of patients, and common diseases among these included Potocki-Lupski syndrome, 15q11-13 duplication syndrome and duplication 22q. Abnormal karyotype with normal array CGH results was exhibited in 2.6% of patients; theses included balanced translocation (n=2), inversion (n=2) and low-level mosaicism (n=1). Facial abnormalities (p<0.001) and failure to thrive were (p<0.001) also more frequent in the group of patients with abnormal CGH findings. CONCLUSION: Array CGH is a useful diagnostic tool in clinical settings in patients with developmental delay or intellectual disability combined with facial abnormalities or failure to thrive.
Adolescent ; Adult ; Child ; Child, Preschool ; Comparative Genomic Hybridization/*methods ; Female ; Gene Dosage/genetics ; Humans ; Infant ; Intellectual Disability/*genetics ; Karyotype ; Male ; Republic of Korea ; Retrospective Studies ; Tertiary Healthcare/statistics & numerical data ; Young Adult

Deletion and duplication of the -3.7-Mb region in 17p11.2 result in two reciprocal syndrome, Smith-Magenis syndrome and Potocki-Lupski syndrome. Smith-Magenis syndrome is a well-known developmental disorder. Potocki-Lupski syndrome has recently been recognized as a microduplication syndrome that is a reciprocal disease of Smith-Magenis syndrome. In this paper, we report on the clinical and cytogenetic features of two Korean patients with Smith-Magenis syndrome and Potocki-Lupski syndrome. Patient 1 (Smith-Magenis syndrome) was a 2.9-yr-old boy who showed mild dysmorphic features, aggressive behavioral problems, and developmental delay. Patient 2 (Potocki-Lupski syndrome), a 17-yr-old boy, had only intellectual disabilities and language developmental delay. We used array comparative genomic hybridization (array CGH) and found a 2.6 Mb-sized deletion and a reciprocal 2.1 Mb-sized duplication involving the 17p11.2. These regions overlapped in a 2.1 Mb size containing 11 common genes, including RAI1 and SREBF.
Adolescent ; Asian Continental Ancestry Group/*genetics ; Child, Preschool ; *Chromosomes, Human, Pair 17 ; Comparative Genomic Hybridization ; Developmental Disabilities/etiology/genetics ; Gene Deletion ; Gene Duplication ; Humans ; Intellectual Disability/etiology/genetics ; Karyotyping ; Male ; Smith-Magenis Syndrome/diagnosis/*genetics ; Sterol Regulatory Element Binding Protein 1/genetics ; Transcription Factors/genetics

BACKGROUND: We evaluated the performance and false positive rate of Mediace RPR test (Sekisui, Japan), a newly introduced nontreponemal test using a chemistry autoanalyzer. METHODS: The sensitivity of Mediace RPR test was analyzed using sera from 50 patients with syphilis in different stages (8 primary, 7 secondary, and 35 latent), 14 sera positive with fluorescent treponemal antibody absorption (FTA-ABS) IgM, and 74 sera positive with conventional rapid plasma regain (RPR) card test (Asan, Korea) and also positive with Treponema pallidum hemagglutination (TPHA) test or FTA-ABS IgG test. The specificity was analyzed on 108 healthy blood donors. We also performed RPR card test on 302 sera that had been tested positive with Mediace RPR test and also performed TPHA or FTA-ABS IgG test to analyze the false positive rate of Mediace RPR test. A cutoff value of 0.5 R.U. (RPR unit) was used for Mediace RPR test. RESULTS: Mediace RPR test on syphilitic sera of different stages (primary, secondary, and latent stages) and FTA-ABS IgM positive sera showed a sensitivity of 100%, 100%, 82.9% and 100%, respectively. Among the 74 sera positive with conventional RPR card test and TPHA or FTA-ABS IgG test, 55 were positive with Mediace test. The specificity of Mediace RPR test on blood donors was 97.2%. Among the 302 sera positive with Mediace RPR test, 137 sera (45.4%) were negative by RPR card and TPHA/FTA-ABS IgG tests. CONCLUSIONS: Although the sensitivities of Mediace RPR were good for primary and secondary syphilis, due to its high negative rate of Mediace RPR over the conventional RPR positive samples, further studies are necessary whether it can replace conventional nontreponemal test for screening purpose. Moreover, in view of the high false positive rate, positive results by Mediace RPR test should be confirmed with treponemal tests.
Autoanalysis/methods ; False Positive Reactions ; Humans ; ROC Curve ; Sensitivity and Specificity ; Syphilis/*diagnosis ; Syphilis Serodiagnosis/*methods

The hyaline membrane disease is not a common disease in Korea. Only a few reports of small scale are avaiable in the literature. We have experienced 5 cases of HMD during approximately 1 year period. The diagnosis was made either on characteristic clinical and roentgenological features or postmortem examination. The birth weights of these cases were in the range of 1,000-1,500gm in 2 cases and 2,000-2,500gm in 3 cases. And their gestational age was 28-34 weeks in most of the cases. Three cases were delivered by C-section. There was 1 case of placenta previa. Four of these 5 cases died after average 18 hours postnatum. Postmortem findings in two cases were characterized by typical hyaline membrane lining th respiratory bronchioles and alveolar ducts. Other prominent findings were atelectasis, interstitial edema and congestion and lymphatic dilatation. One case complicated with multifocal bronchopneumonia and perinatal telencephalic leukoencephalopathy. The other case showed acute subarachnoid hemorrhage probably from germinal matrix hemorrhage.
Autopsy* ; Birth Weight ; Bronchioles ; Bronchopneumonia ; Diagnosis ; Dilatation ; Edema ; Estrogens, Conjugated (USP) ; Gestational Age ; Hemorrhage ; Humans ; Hyalin* ; Hyaline Membrane Disease* ; Infant, Newborn ; Korea ; Leukoencephalopathies ; Membranes ; Placenta Previa ; Pulmonary Atelectasis ; Subarachnoid Hemorrhage

BACKGROUND: We compared the results of liver biopsy and the levels of serum type IV collagen of the hepatitis B carriers with normal liver function test (LFT) to evaluate the clinical usefulness of serum type IV collagen in predicting the progression of histopathological findings. METHODS : Thirty one chronic hepatitis B carriers with normal LFT and no significant clinical symptoms, who were Korean combat police, were classified into three groups according to their histologic results of the liver biopsies. The classification followed the standard proposed by Korean Society of Pathology. Blood samplings for serum type IVcollagen (reference : less than 5 ng/mL) were done in the morning of the same day of the liver biopsy. RESULTS: Of thirty one patients, thirteen patients showed normal histologic findings (41.9%, Group A), eleven patients revealed histologic abnormalities without fibrosis (35.5%, Group B) and seven patients were with fibrosis on liver biopsy (22.6%, Group C). Serum type IV collagen levels of Group A, B and C were 3.53 +/- .57 ng/mL, 3.56 +/- .17 ng/mL and 3.97 +/- .88 ng/mL, respectively. The average of serum type IV collagen levels of Group C was higher than of Group B and the average of Group B higher than that of Group A without any statistical significance (p > 0.05). The averages of serum type IV collagen of eighteen patients with histologic abnormalities (Group B and C) and twenty four patients without fibrosis (Group A and B) were 3.73 +/- 1.06 ng/mL and 3.55 +/- .88 ng/mL respectively. Upon comparison of these averages with the those of Group A and C, no statistical significance was established (p > 0.05). CONCLUSION : In chronic hepatitis B carriers with normal LFT findings, levels of serum type IV collagen were elevated along with histologic severities without statistical significance, therefore can not represent the changing degree of the histologic findings. Liver biopsy is considered to be one of the most accurate tool to assess the histologic status of the liver.
Biopsy* ; Classification ; Collagen Type IV* ; Collagen* ; Fibrosis ; Hepatitis B Surface Antigens ; Hepatitis B* ; Hepatitis B, Chronic ; Hepatitis* ; Humans ; Liver Function Tests* ; Liver* ; Needles ; Pathology ; Police

BACKGROUND: Pheochromocytoma may arise within the adrenal medulla, or in other locations where sympathetic ganglia or chromaffin tissues are known to exist. Approximately 0.1% of hypertensive patients have pheochromocytoma. Most of this hypertension can be cured by surgical removal of the tumor, but lethal complication can develop if proper treatment is not prepared. Therefore, accurate diagnosis and preoperative preparation is very important. The objective of this study was to evaluate the clinical characteristics of pheochromocytoma and the blood pressure change following tumor removal. METHOD: The medical records of 45 patients [29 cases of intra-adrenal, 16 cases of extra-adrenal (paraganglioma)] diagnosed with of pheochromocytoma at Korea University Medical Center between 1991 and 2001 were reviewed. RESULTS: Twenty of the cases were male and 25 were female. The mean age of these patients was 43 years old. Hypertension, headaches, palpitations and impaired glucose tolerance were observed more frequently in cases of intra-adrenal tumor than in those of extra-adrenal. In the extra-adrenal group, abdominal pain, hypertension and nausea were more frequently observed. A biochemical study showed that the sensitivity of the test for catecholamines and metabolites in 24 hours urine was over 80%. For the localization of tumors we used abdominal CT and MIBG. The sensitivities of the CT and MIBG for the tumor localization were 97 and 91%, respectively. Blood pressures during the operations were effectively controlled by preoperative treatment with phenoxybenzamine (non-competitive, non-selective -adrenoreceptor antagonists). CONCLUSION: Surgical treatment cured 23 of the 30 cases of hypertension with pheochromocytoma. We have to take careful approaches in the care of the patient who may have pheochromocytoma, due to the various clinical signs and symptoms.
3-Iodobenzylguanidine ; Abdominal Pain ; Academic Medical Centers ; Adrenal Medulla ; Adult ; Blood Pressure ; Catecholamines ; Diagnosis ; Female ; Ganglia, Sympathetic ; Glucose ; Headache ; Humans ; Hypertension ; Korea ; Male ; Medical Records ; Nausea ; Paraganglioma ; Phenoxybenzamine ; Pheochromocytoma* ; Tomography, X-Ray Computed

In this study, the paclitaxel eluting stent (PES) was prepared by coating a biliary stent with paclitaxel using various biopolymer such as poly (vinyl acetate) (PVAc), poly (lactic-co-glycolic acid) (PLGA), Silicone rubber for restenosis prevention in gastrointestinal disease by a dip-coating method. Drug contents of PES were increased as surface area of stent, concentration and molecular weight of coating polymer increase. In 1H-NMR specta, we know that drug did not change by confirming specific peaks of paclitaxel in PES. As shown in SEM image, PES prepared using various biopolymer is coated clearly and regularly except Silicone rubber coating polymer. In in vitro cell cytotoxicity test, bare stent showed low cytotoxic effect against CT-26 colon carcinoma cell line on 3 day. However, PES coated with PLGA 502H showed the highest cytotoxicity because PLGA 502H is biodegradable polymer and has less molecular weight than other coating polymer. These results suggest that PES coated various biopolymer can be prevented restenosis in gastrointestinal disease.
Biopolymers ; Cell Line ; Colon ; Gastrointestinal Diseases ; Lactic Acid ; Molecular Weight ; Paclitaxel ; Polyglycolic Acid ; Polymers ; Silicone Elastomers ; Stents