Marfan's Syndrome was a collagenous hereditary disorder affected by the long bone, heart and eyes. This typical arachnodactyly patient was 8 year old boy who has had visual disturbance due to central opacitous lens subluxation of both eyes. We performed successfull intracapsular lens extraction by use of cryolens extractor for both eyes. X-ray chest suggested the evidence of aortic aneurysm. Electron microscopic study for the zonular fibers of the lens showed a relative loose aggregated zonular fibers with small amount of its bundles, but chromosomal study for this patient showed no abnormality.
Aortic Aneurysm ; Arachnodactyly ; Child ; Collagen ; Heart ; Humans ; Lens Subluxation ; Male ; Marfan Syndrome ; Thorax

The term "entropion" is defined as a turning inward of the eyelid. Depending on the mechanism of its causation, it may be classified into four types; mechanical, spastic, senile, and cicatricial. Cicatricial entropion follows scarring of the palpebral conjunctiva, which may be caused by trauma, chemical injuries, infections such as trachoma, benign ocular pemphigus, Stevens-Johnson syndrome, and eyelid surgery. Distichiasis is a rare congenital anomaly in which aberrant separate row of lashes usually composed of hairs smaller than normal, appears along the posterior lid margin. Numerous surgical procedures have been described for surgical correction of cicatricial entropion and distichiasis in accordance with the severity of the condition. We have treated cicatricial entropion in 6 eyes(8 lids) and distichiasis in 3 eyes (3 lids) with sliding tarsoconjunctival grafting, and the results were satisfactory.
Cicatrix ; Conjunctiva ; Entropion* ; Eyelids ; Hair ; Muscle Spasticity ; Pemphigus ; Stevens-Johnson Syndrome ; Trachoma ; Transplants

A new stereoisomeric monoterpene glycoside and five already-known compounds were isolated from the n-BuOH soluble fraction of Clematis heracleifolia leaves. On the basis of spectral data, the structures of the isolated compounds were identified as protocatechuic acid (1), ferulic acid (2), caffeic acid (3), aesculin (4), (6Z)-9-hydroxylinaloyl glucoside (5), and 9-hydroxylinaloyl glucoside (6) and these were isolated for the first time from this plant. Among these compounds, (6Z)-9-hydroxylinaloyl glucoside (5) is a newly isolated from plant source.
Clematis* ; Esculin ; Plants ; Stereoisomerism*

Behcet's Disease (BD) is a chronic inflammatory disorder characterized by vasculitis of unknown cause, involving multiple organs. In the past years, renal involvement has not been regarded as a feature of BD. However, renal lesions in BD, although uncommon, have increasingly been recognized in recent years. Five categories of renal lesions associated with BD have been identified, including glomerulonephritis, amyloidosis, renal vascular involvement, interstitial nephritis, and other problems, such as complications of drug therapy or abnormalities of the genitourinary tract. On the other hand, regional differences in the disease expression of BD are well appreciated. Renal lesions accompanied by BD have rarely been reported in Korea, although it have been described occasionally in the Middle Eastern countries. Hereby, we described a 46-year-old female patient who had BD with persistent hematuria and proteinuria, whose renal biopsy specimens were consistent with thin glomerular basement membrane disease. Interestingly, some portions of her glomerular capillary walls were filled with fuchsinophilic fibrin-like materials.
Amyloidosis ; Basement Membrane ; Biopsy ; Capillaries ; Drug Therapy ; Female ; Glomerular Basement Membrane* ; Glomerulonephritis ; Hand ; Hematuria ; Humans ; Korea ; Middle Aged ; Nephritis, Interstitial ; Proteinuria ; Vasculitis

A 55-year-old woman was referred to our hospital with a 6-month history of dyspnea (NYHA II-III). The physical examination revealed a grade 2/6 "tumor plop," i.e., a low-pitched sound heard during early or mid-diastole. The chest X-ray showed mild cardiomegaly with lung congestion in both lower lung fields. Two-dimensional echocardiogram showed a large myocardial mass, prolapsing into the left ventricle during diastole. Chest computed tomography showed a multi-lobulated mass (6.8x4.1 cm) attached to the interatrial septum during systole and prolapsing into the left ventricle during diastole. Coronary angiography demonstrated large tumor vessels arising from and surrounding the posterior lateral branch of the right coronary artery. The mass was excised and the patient recovered uneventfully.
Cardiomegaly ; Coronary Angiography ; Coronary Vessels ; Diastole ; Dyspnea ; Estrogens, Conjugated (USP) ; Female ; Glycosaminoglycans ; Heart Atria ; Heart Ventricles ; Humans ; Lung ; Middle Aged ; Myxoma ; Physical Examination ; Systole ; Thorax

BACKGROUND: In recent years in Korea, human brucellosis (HB) and human leptospirosis (HL) have become the major zoonoses with a dramatic increased cases of incidence in man; we analyzed the current state and epidemiological aspects of related risk factors from 2001 to 2008. METHODS: Based on the surveillance data of HB and HL, most are confirmed cases in Korea from the Annual Reports of HB and HL in the Disease Web Statistics System, Korea Center for Disease Control and Prevention (KCDCP). RESULTS: The incidence of HB in Korea from 2001 to 2008 was 596 cases, and that of HL was 1,025 cases. When both prevalence rates were compared during the same period, the HB was lower than that of HL. The seasonal distribution of HB cases showed that the incidence from spring to summer were higher than that of HL (P<0.01), while the outbreaks of HL in autumn was much more than that of HB (P<0.01). Geographical distribution HB cases were western and central regions of the rural (60.4% of total) in the Korean peninsula, showing higher outbreaks than other areas, while HL occurred in easterly regions (72.7%). Significantly more males were infected in both HB (84.2%) and HL (58.9%) than those of females in both HB (13.1%) and HL (41.1%), respectively (P<0.01). The distribution by age groups were different between HB and HL, while the outbreaks over 62.8% of the cases of HB occurred in 40 to 59 year-old age group, and that of HL was clearly showing a high incidence in the elderly age over-60-year-old (60.8%) (P<0.01). In both diseases, elderly people especially in farmers showed a very high prevalence rate (62.8% of HB and 60.8% of HL), which is possibly due to increased outdoor activities and a decreased number of young people in those areas. The occupational distribution of HB cases were farmers, veterinarians, dairyman and others, and those of HL cases were broad. CONCLUSION: The difference in HB and HL risk factors reflects the different influences of hosts/vector, climate, and geographical and environmental characteristics in the epidemiological patterns.
Aged ; Brucellosis ; Centers for Disease Control and Prevention (U.S.) ; Climate ; Disease Outbreaks ; Female ; Humans ; Incidence ; Korea ; Leptospirosis ; Male ; Prevalence ; Public Health ; Risk Factors ; Seasons ; Veterinarians ; Zoonoses

The purpose of these studies was an establishment of human auricular chondrocyte cell line using retrovirus mediated v-myc transfer, characterizing the human auricular chondrocyte cell line by type II collagen mRNA expression and transplantation of human auricular cell line into immunological incompetent nude mice to establish neocartilage formation. Also, I evaluated the growth rate of chondrocyte cell line to measure the cellular proliferative potency. I have established the human auricular chondrocyte cell line integrated v-myc and confirmed by v-myc transduced Myc protein expression by immunohistochemistry and immunoblotting study. And, growth rate of established human auricular chondrocyte cell line increased 4 folds times faster than primarily cultured human auricular chondrocyte. The established human auricular chondrocyte had type II collagen mRNA upto 8 months in monolayer culture. And we observed formation of neocartilage on the back of nude mice using chondrocyte cell line/fibrin glue polymer at 12 weeks transplantation.
Adhesives ; Adult* ; Animals ; Cell Line* ; Chondrocytes ; Collagen Type II ; Humans* ; Immunoblotting ; Immunohistochemistry ; Mice ; Mice, Nude ; Polymers ; Retroviridae ; RNA, Messenger

The followings are the results for external quality assessment (EQA) in immunoserology for 2003: 1.Evaluation of EQA was done in 2 trials in May and November, about 99% of laboratories participating average 8.2 items. 2.In C-reactive protein (CRP), rheumatoid factor (RF) and anti-streptolysin O (ASO) tests, about 63%, 49% and 44% of the participating laboratories respectively have used quantitative assays. Because the laboratories using quanitiative assays were on the increase annually, commercial control, Liquicheck(TM) Immunology Contol from Bio-Rad Laboratories (Irvine, CA, USA) was used to assure the quality of quantitiavie results in 2003. A few laboratories reproted the outlier results, comparing with the reference ranges presented by the company. 3.Over 92% of participating laboratoreis have used imunoassays including enzyme immunoassay (EIA), microparticle EIA (MEIA), chemiluminescence immunoassay (CIA), immunochromatography assay (ICA) or radioimmunoassay (RIA) for detedting viral antigens or antibodies. Especially for anti-HCV, over 98% of participating laboratoreis have used various kind of imunoassays. Laboratories using ICA increased and about 24% of participating laboratoreis have used ICA for anti-HCV and anti-HIV. However, many laboratories using ICA for detecting anti-HCV reported false negative results, suggesting lower sensitivity of ICA than those of other immunoassays. 4.The criteria of interpretation were considered to be evaluated in Widal test and laboratories using ICA increased in serological tests for syphilis.
Allergy and Immunology ; Antibodies ; Antigens, Viral ; C-Reactive Protein ; Hepatitis B Surface Antigens ; Immunoassay ; Immunochromatography ; Immunoenzyme Techniques ; Korea* ; Luminescence ; Nephelometry and Turbidimetry ; Radioimmunoassay ; Reference Values ; Rheumatoid Factor ; Serologic Tests ; Syphilis

The followings are the results for external quality assessment (EQA) in immunoserology for 2002: 1. Evaluation of EQA was done in 2 trials in May and November, about 96% of laboratories participating average 8.3 items. 2. In C-reactive protein (CRP), rheumatoid factor (RF) and anti-streptolysin O (ASO) tests, about 40%, 53% and 52% of the participating laboratories respectively have used qualitative assays, mainly latex agglutination. And about 55%, 43% and 40% of the participating laboratories have used quantitative assays, turbidimetric immunoassay (TIA) or nephelometry in CRP, RF and ASO tests respectively. Laboratories using TIA increased and those using nephelometry decreased. The instruments which were the most frequently used in nephelometry were BN series (Dade Behring Inc., Germany). The instruments of Hitachi series (Hitachi Ltd., Japan), Cobas Integra and Mira series (Roche Diagnostics GmbH, Germany), Toshiba series (Toshiba Corporation, Japan) and Olympus AU series (Olympus Optical Co., Ltd., Japan) were frequently used in TIA. The quantitative results were quite variable according to the methods or reagents, especially in RF and ASO. 3. Over 90% of participating laboratoreis have used imunoassay including enzyme immunoassay (EIA), microparticle EIA (MEIA), chemiluminescence immunoassay (CIA), immunochromatography assay (ICA) or radioimmunoassay (RIA). Laboratories using CIA and ICA increased. Sensitivities of ICA were lower than those of other immunoassays in the results of HBsAg and anti-HCV. The sensitivity of SD HCV (Standard Diagnostics, Inc., Korea) was especially lower in anti-HCV results. Sensitivities of CIA and ICA were also lower than those of EIA including MEIA in the results of anti-HIV. 4. The criteria of interpretation were considered to be evaluated in Widal test and laboratories using ICA increased in serological tests for syphilis.
Agglutination ; C-Reactive Protein ; Hepatitis B Surface Antigens ; Immunoassay ; Immunochromatography ; Immunoenzyme Techniques ; Indicators and Reagents ; Korea* ; Latex ; Luminescence ; Nephelometry and Turbidimetry ; Radioimmunoassay ; Rheumatoid Factor ; Serologic Tests ; Syphilis

The followings are the results for external quality assessment (EQA) in immunoserology for 2004: 1. Evaluation of EQA was done in 2 trials in May and November, about 99% of laboratories participating average 8.4 items. EQA for anti-HBc test was newly started in 2004. 2. Commercial control, MASR Immunology Control from Medical Analysis Systems (Camarillo, CA, USA) was used to assure the quality of quantitative results of C-reactive protein (CRP), rheumatoid factor (RF) and anti-streptolysin O (ASO) tests in 2004. All the specimens for Immunoserology in EQA were delivered refrigerated for the first time, being received within 48 hours after sending. 3. EQA for detection of HBsAg mutants was tried for the first time, using the recombinant HBsAg mutant (Gly/Arg 145) kindly provided by Abbott Laboratories, USA. 4. The laboratories using immunochromatography assay (ICA) were increased, however, many laboratories using ICA reported falsely negative for the positive specimens. The sensitivity of ICA test kits as well as various factors influencing the ICA results should be evaluated. 5. Standardization of methods including calibrators for quantitative results should be required for the harmonization of results.
Allergy and Immunology ; C-Reactive Protein ; Hepatitis B Surface Antigens ; Immunochromatography ; Korea* ; Nephelometry and Turbidimetry ; Rheumatoid Factor