Klippel-Feil syndrome(KFS) consists of short neck, low posterior hairline and restriction of motion of the neck due to fusion of cervical vertebrae. The typical disorder results from a failure of the normal segmentation of mesodermal somites during 3-8 weeks of gestation. In 1912, the first complete clinical description of this syndrome was given by Klippel and Feil. Feil reported additional cases in 1919 and distinguished between three morphologic groups. The incidence of KFS has been estimated to be approximately 1 : 40,000-42,000 births. A slight female predilection has been noted. Although the disorder is sporadic, there are examples of familial occurrence; how ever, no clear mechanism of inheritance has been accepted. Since the disturbance producing a short neck occurs early in embryogenesis, defects in other organ systems may occur at the same time. Common musculoskeletal anomalies that accompany KFS include scoliosis, as well as Sprengel's deformity in as many as one-third of cases. Neurologic, cardiovascular, and urinary tract anomalies are associated with KPS. We report a case of Klippel-Feil syndrome with associates anomalies include Sprengel's deformity.
Cervical Vertebrae ; Congenital Abnormalities ; Embryonic Development ; Female ; Humans ; Incidence ; Klippel-Feil Syndrome* ; Mesoderm ; Neck ; Parturition ; Pregnancy ; Scoliosis ; Somites ; Urinary Tract ; Wills

Human adenovirus type 5 (hAd5) vectors have been demonstrated to be useful vehicles for gene expressions in animals. However, it has not been reported whether hAd5 transduction might be hampered in the sera of livestock animals in Republic of Korea. We collected 205 samples of livestock animals, such as pig (n=84), cattle (n=84), and goat (n=37) in Korea. The neutralizing antibody (NAb) titers to hAd5 virus were less than 15 in most of samples. Only 8% of goat samples had a NAb titer of 15 or 30. Thus, we showed that hAd5 virus was not neutralized in sera from cattle, pig, and goat, and suggest that the hAd5 vector could be used for the effective delivery of vaccines or proteins in livestock animals in the field.
Adenoviruses, Human* ; Animals ; Antibodies, Neutralizing ; Cattle* ; Gene Expression ; Goats* ; Korea ; Livestock ; Republic of Korea* ; Vaccines

BACKGROUND: It has been anticipated that the amount and composition of mucin are changed in patients with chronic airway diseases. We evaluated whether RTO3 (mAb against rat tracheal mucins) could que ntify the amount of mucin from the airway in the patients with chronic airway diseases. METHODS AND RESULTS: 1) RTO3 was bound to high molecular weight of mucin based on Western blot in sputum and BALF from patients with chronic airway diseases. 2) The goblet cells and submucosal glands in main bronchus from human were observed by PAS stain. And immunohistochemical stain with RTO3 showed immunoreactivity on some goblet cells. 3) The amount of mucin was more increased in patients with chronic airway diseases compared to those in normal subjects. 4) In the exacerbation of asthmatics, mucin amounts were more increased than stable asthmatics. CONCLUSION: We suggested that secreted mucin in chronic airway diseases can be quantified by ELISA with RTO3.
Animals ; Blotting, Western ; Bronchi ; Enzyme-Linked Immunosorbent Assay ; Goblet Cells ; Humans ; Molecular Weight ; Mucins* ; Rats ; Sputum

BACKGROUND: The criteria set by the International Society on Thrombosis and Hemostasis (ISTH) with the criteria of the Korean Society on Thrombosis and Hemostasis (KSTH) for the diagnosis of DIC was compared to evaluate the agreement between two criteria and the characteristics of each criteria. METHODS: Two hundred ninety-six adult patients with sepsis (57 pneumonia, 75 hepatobiliary and gastrointestinal infection, 25 urinary tract infection, 51 infection associated with malignant diseases and 84 other causes) were studied. The rate of agreement in the diagnosis of DIC by the two diagnostic systems was analyzed. Characteristics of each criteria was also analyzed by the comparison of laboratory criteria. RESULTS: The kappa coefficient and concordance rate, agreement parameters in the diagnosis of DIC by the two diagnostic systems was 0.78 and 89.5%, respectively. The median platelet count was lower and FDP level was higher in the patients diagnosed by ISTH criteria than in patients diagnosed by KSTH criteria. CONCLUSION: The agreement between ISTH and KSTH criteria was significantly high.
Adult ; Dacarbazine ; Diagnosis ; Disseminated Intravascular Coagulation* ; Hemostasis* ; Humans ; Platelet Count ; Pneumonia ; Sepsis ; Thrombosis* ; Urinary Tract Infections

BACKGROUND/AIMS: Duodenal ulcers occur relatively frequently in adolescents, like in adults, and may relate to Helicobacter pylori infection and duodenal gastric metaplasia (DGM). This study investigated the association between H. pylori infection and DGM in healthy adults aged 20-29. METHODS: Between 1995 and 2005, endoscopic biopsies of the duodenum, antrum and body were taken from healthy, young volunteers, who were first-year medical students, faculty staff, residents, and research assistants of Gyeongsang National University in Jinju, Korea. Urease tests were performed and the extent of DGM and histopathological grades according to the Updated Sydney System were determined. RESULTS: In total, 662 subjects were enrolled (429 males and 233 females). The median age was 22.3 years. The overall incidence of DGM was 11.5% but DGM was more frequent in males (15.4%) than in females (4.3%) (p<0.0001). While H. pylori positivity rates changed significantly during the 1995-2005 period (p<0.01), the incidences of DGM did not. DGM was observed in 7.2% and 14.9% of subjects who were and were not colonized with H. pylori, respectively. DGM was also associated with less severe chronic gastritis and the absence of active gastritis in both the antrum and body, and the absence of follicles in the antrum (p<0.05). CONCLUSIONS: These findings suggested that DGM is not rare in healthy young adults and is unrelated to gastric H. pylori infection.
Adult ; Age Factors ; Duodenum/*pathology ; Female ; Gastric Mucosa/*pathology ; Helicobacter Infections/complications/*diagnosis/epidemiology ; *Helicobacter pylori ; Humans ; Male ; Metaplasia/complications/*diagnosis/epidemiology ; Pyloric Antrum/*pathology ; Severity of Illness Index ; Sex Factors ; Young Adult

Low-abundance cellular proteins normally invisible on the standard two-dimensional SDS-polyacrylamide gel electrophoresis (2-DE SDS-PAGE) map must be enriched appropriately in order to be visualized and identified in cells or tissues. We applied proteins of H. pylori strain 26695 to a immobilized heparin-affinity resin, which has an affinity for nucleic acid-binding proteins, protein biosynthesis factors, and growth factors. The whole cell extract of H. pylori strain 26695 was fractionated by the heparin-agarose chromatography, and was analyzed by 2-DE. The 2-DE SDS-PAGE displayed spots after silver staining, which were identified by matrix-assisted laser desorption ionization mass spectrometry (MALDI-MS). Among the ca. 150 spots that were processed, 79 proteins representing 57 genes were identified. Eleven proteins were determined to be nucleic acid-associated. Eighteen proteins were newly identified in this study, including DNA topoisomerase I. These results may provide guidance for enriching low abundance proteins of H. pylori and contribute to the construction of a master protein map of H. pylori.
Chromatography* ; DNA Topoisomerases, Type I ; Electrophoresis ; Electrophoresis, Polyacrylamide Gel ; Helicobacter pylori* ; Helicobacter* ; Heparin* ; Intercellular Signaling Peptides and Proteins ; Mass Spectrometry ; Protein Biosynthesis ; Proteome ; Silver Staining

Purpose: To derive the optimized intraocular lens (IOL) constants from automated and manifest refraction after cataract surgery in Korean patients, and to evaluate whether there is a difference in optimized IOL constants according to the refraction method. Methods: This retrospective multicenter cohort study enrolled 4,103 eyes of 4,103 patients who underwent phacoemulsification and in-the-bag IOL implantation at 18 institutes. Optimized IOL constants for the SRK/T, Holladay, Hoffer Q, and Haigis formulas were calculated via autorefraction or manifest refraction of samples using the same biometry and IOL. The IOL constants derived from autorefraction and manifest refraction were compared. Results: Of the 4,103 eyes, the majority (62.9%) were measured with an IOLMaster 500 followed by an IOLMaster 700 (15.2%). A total of 33 types of IOLs were used, and the Tecnis ZCB00 was the most frequently used (53.0%). There was no statistically significant difference in IOL constants derived from autorefraction and manifest refraction when IOL constants were optimized with a large number of study subjects. On the other hand, optimized IOL constants derived from autorefraction were significantly smaller than those from manifest refraction when the number of subjects was small. Conclusions It became possible to use the IOL constants optimized from Koreans to calculate the IOL power. However, if the IOL constant is optimized using autorefraction in a small sample group, the IOL constant tends to be small, which may lead to refractive error after surgery.