Amyloid fibril formation has been implicated in the pathogenesis of neurodegenerative diseases. Fibrillation generates numerous conformers. Presumably, the conformers may possess specific biological properties, thus providing a biochemical framework for strains of prions. However, the precise relationship between various fibril conformers and their pathogenic functions has not been determined because of limited accessibility to adequate amounts of fibrils from tissue samples. α-Synuclein is one such protein, and it has been implicated in Parkinson disease. Using a technique known as protein misfolding cyclic amplification, originally developed for amplifying prions, we established a procedure through which the amplification of α-synuclein fibrils is possible. With a trace amount of seeds, we succeeded in amplifying α-synuclein fibrils. The replication of the seeds was faithful in terms of conformation even after multiple rounds of cyclic amplification. Moreover, two transgenic mouse strains each representing a distinct synucleinopathy were used to investigate different conformers by using this technique. The amplified α-synuclein fibrils derived from the tissue extracts of these two strains led to the production of two different fibril conformers with distinct proteinase K digestion profiles. Together, our results demonstrated that a trace amount of α-synuclein fibrils in tissue extracts could be amplified with their conformations conserved. This procedure should be useful in amplifying α-synuclein fibrils from the brains and body fluids of patients afflicted with synucleinopathies and may serve as a potential diagnostic tool for Parkinson disease and other synucleinopathies.
Amyloid ; Animals ; Body Fluids ; Brain ; Digestion ; Endopeptidase K ; Humans ; Mice ; Mice, Transgenic ; Neurodegenerative Diseases ; Parkinson Disease ; Prions ; Tissue Extracts

PURPOSE: The purpose of this study was to assess the value of adding digital breast tomosynthesis (DBT) to full-field digital mammography (FFDM) in the diagnostic workup of breast cancer and to determine which lesion variables affect cancer detectability in the combined modality. METHODS: Between March and May 2012, paired FFDM and DBT images were obtained from 203 women as part of a diagnostic workup for breast cancer. Images from FFDM alone, DBT alone, and DBT combined with FFDM were reviewed in separate sessions by six blinded readers. Jackknife alternative free-response receiver operating characteristic (JAFROC) figure of merit (FOM), sensitivity, and specificity were compared between the modalities. Lesion characteristics affecting the cancer detection rate when using the combined modality were also analyzed. RESULTS: Among the 203 women, 126 women had a total of 129 malignancies and 77 women had total of 77 benign lesions. The overall JAFROC FOM of the combined modality was higher than that of FFDM alone (0.827 vs. 0.775, p<0.001) and that of DBT alone was higher than that of FFDM alone (0.807 vs. 0.775, p=0.027). The overall sensitivity of the combined modality was higher than that of FFDM alone (80.0% vs. 73.2%, p<0.001) and that of DBT alone was higher than that of FFDM alone (78.3% vs. 73.2%, p=0.007). Compared to FFDM alone, the combined modality detected an additional 48 cancers. Using the combined modality, the presence of masses or microcalcifications was significantly associated with the cancer detection rate (p<0.001). CONCLUSION: The combination of DBT with FFDM results in a higher diagnostic yield than FFDM alone. Additionally, DBT alone performs better than FFDM alone. However, even when DBT is combined with FFDM, breast cancers with no discernible masses and those lacking calcifications are difficult to detect.
Breast Neoplasms ; Breast* ; Early Detection of Cancer ; Female ; Humans ; Imaging, Three-Dimensional ; Mammography* ; ROC Curve ; Sensitivity and Specificity

OBJECTIVE: To assess the feasibility and clinical efficacy of laparoscopic myomectomy (LM). METHODS: We analyzed retrospectively the data for 110 LM preformed at Kangbuk Samsung Hospital between July 2003 and July 2006. We reviewed their clinical charts and the operative and anesthetic records, and analyzed data on the patients' age, parity, previous operative history, preoperative indication, the diameter of the largest myoma, operating time, number of the removed myoma, hospital stay, change of the hemoglobin concentration from preoperative to postoperative day 1, concomitant procedures, histopathological reports, and complications. RESULTS: The mean age of the patients was 35.7+/-5.8 years, the mean parity was 0.8+/-0.9, and 26 (23%) patients had a previous operative history. The most common operative indication was a palpable abdominal mass (46 patients, 41.9%), followed by chronic pelvic pain (32 patients, 29.0%), abnormal uterine bleeding (24 patients, 21.8%), urinary frequency (7 patients, 6.4%), and infertility (1 patient, 0.9%). The mean operating time was 82.9+/-30.8 minutes, and the mean diameter of the largest myoma was 7.1+/-2.3 cm. The heaviest of the removed myoma weighed 795 gm. The mean change of the hemoglobin concentration was 2.2+/-1.1 g/dL, and the mean hospital stay was 3.5+/-1.4 days. Postoperatively, transfusions were done 10% (11 cases) of patients, and 2 cases of paralytic ileus and a case of subcutaneous emphysema were noted. Conversion rate to laparotomy was 0.9% (1 case). CONCLUSION: LM for various sized myomas can be performed successfully and effectively by decreasing laparoconversion, if the surgical team and the laparoscopic surgeon are experienced and enhanced equipment is available.
Female ; Humans ; Infertility ; Intestinal Pseudo-Obstruction ; Laparotomy ; Length of Stay ; Myoma* ; Parity ; Pelvic Pain ; Retrospective Studies ; Subcutaneous Emphysema ; Uterine Hemorrhage

BACKGROUND AND OBJECTIVES: Various surgical approach has been used for the removal of pituitary tumor. Recently, endoscopic transnasal transsphenoidal approach has been technically upgraded and the morbidity associated with surgical treatment of the pituitary tumor has been decreased. The objectives of our study was to describe surgical techniques and materials used in sellar repair after endoscopic transnasal transsphenoidal approach. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 18 patients who had received endoscopic transnasal transsphenoidal pituitary surgery between November 2002 and January 2004. We evaluated effectiveness of this technique by analyzing surgical techniques, symptom improvement and complications after surgery. RESULTS: In most of the case, tumor was found to be macroadenoma and 8 of the cases had suprasellar extension. Tumor was totally removed in 13 cases and partially removed in 5 cases. In all cases we used endoscopic unilateral transnasal transsphenoidal approach with anterior sphenoidotomy. CONCLUSION: We may consider that this approach is more safe and effective and a better treatment method for pituitary tumor surgery than the transcranial approach.
Endoscopy ; Humans ; Hypophysectomy* ; Medical Records ; Pituitary Neoplasms* ; Retrospective Studies

PURPOSE: Pigmented spindle cell tumor of the palpebral conjunctiva is very rare, and most of the tumors arise at extremities or trunk. To our best knowledge, no case has been repoted in Korea so far. We report one case of pigmented spindle cell nevus with literature review. METHODS: A 21 years-old male patient complaining of mass on the left upper palpebral conjunctiva underwent excisional biopsy with minimal touch technique. RESULTS: On histopathologic examination, the mass consisted of small, regular and spindle cells with pigmentation. And HMB-45 and CD-34 stains were negative, while S-100 stain was weakly positive. CONCLUSIONS: We report one case of pigmented spindle cell nevus as the first case in Korea.
Biopsy ; Coloring Agents ; Conjunctiva* ; Extremities ; Humans ; Korea ; Male ; Nevus, Spindle Cell* ; Pigmentation ; Young Adult

Five to fifteen percent of patients affected by human immunodeficiency virus(HIV) are children in developing countries. In Korea, most of HIV infections in children have been transfusion-related, and cases of vertical transmission have been very rare so far and are usually suspected due to a maternal positive history. We experienced a case of vertical transmission of HIV in a 19 month- old girl, incidentally diagnosed in the process of work-up for failure to thrive without suspicion from maternal HIV history. With the increasing number of adult HIV patients in Korea, HIV in fection should be included in the differential diagnosis of children with symptoms compatible with HIV infection even when parental HIV history is not suggestive.
Acquired Immunodeficiency Syndrome ; Adult ; Child ; Developing Countries ; Diagnosis, Differential ; Failure to Thrive* ; Female ; HIV Infections ; HIV* ; Humans ; Humans* ; Infant* ; Korea ; Parents

PURPOSES: To investigate the viral etiology of community-acquired pneumonia in Korean adults, we have detected respiratory viruses (Respiatory syncytial virus, adenovirus, influenza virus and parainfluenza virus) in the way of prospective, multi-center study. METHODS: From July 1997 to April 2000, nasal aspirates or sputum were obtained from adults patients with community pneumonia admitted to the participating hospitals and transferred immediately to the central laboratory in the Seoul National University Children's Hospital. The specimens were divided into three parts. One part was used for indirect immunofluorescent test for respiratory viruses, the other part for the culture of RSV and adenovirus in HEp-2 cell monolayer. Another part was used for the culture of influenza virus and parainfluenza virus in MDCK or LLC- MK2 cell monolayers. RESULTS: Of 317 samples, 32 (10.1%) specimens were positive for viral isolation by indirect IF staining or culture, including one dual-infected specimen (adenovirus and parainfluenza virus). Influenza virus was most commonly detected (16 specimens). Parainfluenza virus, adenovirus and RSV were detected in 10, 4 and 3 patients, respectively. All isolated influenza viruses were type A (H3N2 in 9 patients, H1N1 in 2 and unspecified in 5), and 8 out of 10 parainfluenza virus isolates were type 3. CONCLUSION: Similar to previous foreign reports, a significant portion of community-acquired pneumonia in Korean adult is caused by respiratory viruses. Our data empathized the need of referral system for viral diagnosis and of nationwide investigation on respiratory virus infections.
Adenoviridae ; Adult* ; Diagnosis ; Humans ; Orthomyxoviridae ; Paramyxoviridae Infections ; Pneumonia* ; Prospective Studies ; Referral and Consultation ; Seoul ; Sputum

Subacute necrotizing lymphadenitis, or Kikuchi's disease, primarily affects the cervical lyrnph nodes of lung adults and has a self-limited clinical course. Differential diagnoses are malignant lymphoma and systemic lupus erythematosus. Recurrence rate is low as 3.3%, and there have been a few reports describing the recurrence of the disease. The symptoms in almost all recurrent cases were similar to those of the early stages of the disease. The etiology of the disease remains unknown, but various viral infections and autoimmune processes have been postulated to be the cause. A 6-year-old boy presented with a fever of approximately 40C degrees, painless lumps on both sides of the neck, and intermittent abdominal pain for 2 weeks. Subacute necrotizing lymphadenitis was diagnosed 3 years before admission, which was reported by us in 1996. Diagnosis of recurred subacute necrotizing lymphadenitis was confirmed by histological examination of the lymph node. We report a recurred case of subacute necrotizing lymphadenitis during childhood.
Abdominal Pain ; Adult ; Child ; Diagnosis ; Diagnosis, Differential ; Fever ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lung ; Lupus Erythematosus, Systemic ; Lymph Nodes ; Lymphadenitis ; Lymphoma ; Male ; Neck ; Recurrence

Fifty-five strains of Haemophilus influenzae recovered at a children's hospital in Korea from 1992 through 1997, were analyzed for serotype and antibiotic resistance. Antimicrobial susceptibility was tested by broth dilution method. Among the 55 strains, 26 were from normally sterile body fluids, of which 17 were from the immunocompetent children. Spectrum in the immunocompetent included meningitis (47%), bacteremic pneumonia (18%), and bacteremia without focus (35%). Three (12%) of 26 invasive infections were caused by non-type b: one type d and two type f. Nine of 29 non-sterile body fluid isolates belonged to one of encapsulted serotypes: four a, two c, one of each of b, d and e. Thirty two (58%) strains were resistant to ampicillin, and all of which produced beta-lactamase. All of the strains were highly susceptible to amoxicillin/clavulanate, cefixime, cefuroxime, azithromycin and ciprofloxacin, while 1 (2%), 7 (13%), 4 (7%) and 4 (7%) strains were intermediate to cefprozil, cefaclor, loracarbef, and clarithromycin, respectively. The serotype distribution of H. influenzae in Korean children is similar to those in developed countries before the introduction of Hib conjugate vaccine, and ampicillin resistance rate is among the highest published to date.
Antibiotics/pharmacology* ; Child ; Haemophilus Infections/microbiology* ; Haemophilus Infections/drug therapy ; Haemophilus Vaccines ; Haemophilus influenzae/isolation & purification ; Haemophilus influenzae/drug effects* ; Haemophilus influenzae/classification ; Human ; Korea ; Microbial Sensitivity Tests ; Serotyping

PURPOSE: Infective endocarditis (IE) is a serious complication in children with structural heart disease. We reviewed 35 cases of IE to identify the recent changes in the pattern of preexisting heart diseases, the spectrum of causative organisms and prognosis. METHODS: The clinical records of children diagnosed as IE at the Seoul National University Children's Hospital from January 1987 through December 1997, were reviewed retrospectively. Duke criteria was used for diagnosis. Cases were categorized into primary group(PG) IE in an unoperated heart and post operative group(POG), and the latter further into early POG(within 2 months after operation) and late POG. RESULTS: There were 35 cases of IE developed in 34 patients; 18 cases in the PG, 6 cases in the early POG, and 11 cases in the late POG. Male to female ratio was 16 : 19. Mean age of POG, especially early POG was less than that of PG (early POG : late POG : PG=1.65 years : 6.5 years : 8.34 years, P=0.0267). Preexisting heart diseases were identified in 30 cases; rheumatic heart disease 1 case and congenital heart disease (CHD) 29 cases. Causative organisms were identified in 80%; viridans streptococci, 10 cases (33.3%); pneumococci, 2 cases; Group-D streptococci, 3 cases; staphylococci, 8 cases; Gram (-) organisms, 5 cases and Candida albicans, 2 cases. Vegetation was detected in 88.9% of PG and 64.7% of POG. The most common indication for surgery was uncontrolled infection, which were required in 9 cases. The overall mortality rate was 12.1%. Mortality in POG was higher than that of PG (23.5% versus 0%, P=0.033). CONCLUSION: The discrepancy of mean age among patient groups and the high proportion of patients belonging to POG, were consistent with the increase in the number of newly risky population that survived after cardiac surgery. A more aggressive consideration for operative management may improve the treatment results.
Candida albicans ; Child* ; Diagnosis ; Endocarditis* ; Female ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Male ; Mortality ; Prognosis ; Retrospective Studies ; Rheumatic Heart Disease ; Seoul ; Thoracic Surgery ; Viridans Streptococci