Background : Congenital bronchoesophageal fistula(BEF) presented in adult life is a rare disorder and has characteristic clinical findings such as paroxysmal cough after water ingestion and recurrent respiratory infections. It usually manifested recurrent pneumonia and chronic cough with purulent phlegmon which was mis-or under-diagnosed as chronic bronchitis, bronchiectasis or lung abscess so forth. Methods : We reviewed retrospectively 13 cases of congenital BEF in adult of Paik Hospital, College of Medicine, Inje University including 22 cases of congenital BEF previously reported in literature of Korea from 1979 through 1995. Results : The mean age at diagnosis was $40.2{\pm}14.3$. There was no difference in sex ratio(Male : Female 18 : 17). The most common symptom was cough(91.4%), followed by chronic sputum(74.3), hemoptysis(25.7), and paroxysmal nocturnal cough at specific position(20%). Twenty one of 31 patients who were able to review have the most specific sign, Ono's sign presented as paroxysmal cough after liquid ingestion. By classification of Braimbridge-Keith, Fourteen(45.1%) of 31 patients were group I (associated with esophageal diverticulum), 15(48.4%) were group II (simple fistula), and group Ill and IV was one case in each. The opening of fistula confined to right lower lobe in 26(76.5%), left lower lobe in 6(17.6%), and left main bronchus in 2(5.9%) cases. Conclusion : Congenital bronchoesophageal fistula is uncommon disorder which has characteristic histories and specific symptoms such as chronic and recurrent lower respiratory infections, and paroxysmal cough after liquid ingestion. Medical attention and careful history should be done in patients who have localized recurrent lower respiratory infections in right lower lobe.
Cancer is a complex disease heterogeneously composed of various types of cells including cancer stem-like cells responsible for relapse and chemoresistance in the tumor microenvironment. The conventional two-dimensional cell culture-based platform has critical limitations for representing the heterogeneity of cancer cells in the three-dimensional tumor niche in vivo. To overcome this insufficiency, three-dimensional cell culture methods in a scaffold-dependent or -free physical environment have been developed. In this study, we improved and simplified the HCT-8 colon cancer cell-based spheroid culture protocol and evaluated the relationship between cancer stemness and responses of chemosensitivity to 5- Fluorouracil (5-FU), a representative anticancer agent against colon cancer. Supplementation with defined growth factors in the medium and the culture dish of the regular surface with low attachment were required for the formation of constant-sized spheroids containing $CD44^+$ and $CD133^+$ colon cancer stem cells. The chemo-sensitivities of $CD44^+$ cancer stem cells in the spheroids were much lower than those of $CD44^-$ non-stem-like cancer cells, indicating that the chemoresistance to 5-FU is due to the stemness of colon cancer cells. Taken together, the inflammation and oncogenic gut environment-sensitive HCT-8 cell-based colon cancer spheroid culture and comparative evaluation using the simplified model would be an efficient and applicable way to estimate colon cancer stemness and pharmaceutical response to anticancer drugs in the realistic tumor niche.
More than 6,000 rare disorders including genetic diseases have been reported. Of them, 1,500 diseases (1,211 for clinical diagnosis and 289 for research only) are technically possible for genetic testing. In Korea, since 2005, only 63 genetic diseases is permitted for prenatal genetic testing by the "Bioethics and Biosafety Law". The article 25 in the law prescribes 63 genetic diseases without clear indication for its selection and inclusion criteria. In EU, USA, and other foreign countries, however, there is no provision in the statute on prenatal genetic testing; it is not restricted by a law. Recently, a woman (Mrs. L, 38y) who is a carrier for Menkes disease made an appeal to a government for an amendment of the "Bioethics and Biosafety Law" prohibiting the prenatal diagnosis of her pregnancy at risk for Menkes disease. Menkes disease (MNK) is an X-linked recessive disorder characterized by neurodegeneration, connective tissue defects and hair abnormalities, and no effective treatment is available yet. The prevalence rate of MNK is one in about 250,000 live births. Menkes syndrome patients fail to absorb copper from the gastrointestinal tract in quantities adequate for meeting nutritional needs. These needs seem particularly acute during the initial 12 month of life, when the velocity of brain growth and motor neurodevelopment. Most of pts. die around 3yrs. of age. Mrs. L had a boy with Menkes disease who died at 2y.o. in 2001. Subsequent pregnancy in 2003, she was able to have prenatal genetic testing for mutation of the Menkes (ATP7A) gene and delivered a healthy baby boy. Now, She is pregnant again and wants to have prenatal diagnosis. however, this time, she was not allowed to have any more because Menkes disease is not included in 63 genetic diseases permitted by the law for prenatal genetic testing, in spite of the fact that she is a Menkes disease carrier and her pregnancy is at risk to have an affected baby. This case shows the practical problem of the legal restriction for prenatal genetic testing in Korea. In this study, we report a arguable case and discuss the controversial issues in the legal restriction for prenatal genetic testing in Korea.
Park, Sun-Haeng;Kim, Ji-Hyun;Bae, Sun-Sik;Hong, Ki-Whan;Choi, Byung-Tae;Shin, Hwa-Kyoung
Journal of Life Science
/
v.21
no.5
/
pp.647-655
/
2011
The neurotoxicity of aggregated amyloid ${\beta}$ ($A{\beta}$) has been implicated as a critical cause in the pathogenesis of Alzheimer's disease (AD). It can cause neurotoxicity in AD by evoking a cascade of apoptosis to neuron. Here, we investigated the neuroprotective effects of cilostazol, which acts as a phosphodiesterase III inhibitor, on $A{\beta}_{25-35}$-induced cytotoxicity in mouse neuronal cells and cognitive decline in the C57BL/6J AD mouse model via peroxisome proliferator-activated receptor (PPAR)-${\gamma}$ activation. $A{\beta}_{25-35}$ significantly reduced cell viability and increased the number of apoptotic-like cells. Cilostazol treatment recovered cells from $A{\beta}$-induced cell death as well as rosiglitazone, a PPAR-${\gamma}$ activator. These effects were suppressed by GW9662, an antagonist of PPAR-${\gamma}$ activity, indicative of a PPAR-${\gamma}$-mediated signaling. In addition, cilostazol and rosiglitazone also restored PPAR-${\gamma}$ activity levels that had been altered as a result of $A{\beta}_{25-35}$ treatment, which were antagonized by GW9662. Furthermore, cilostazol also markedly decreased the number of apoptotic-like cells and decreased the Bax/Bcl-2 ratio. Intracerebroventricular injection of $A{\beta}_{25-35}$ in C57BL/6J mice resulted in impaired cognitive function. Oral administration of cilostazol (20 mg/kg) for 2 weeks before $A{\beta}_{25-35}$ injection and once a day for 4 weeks post-surgery almost completely prevented the $A{\beta}_{25-35}$-induced cognitive deficits, as did rosiglitazone. Taken together, our findings suggest that cilostazol could attenuate $A{\beta}_{25-35}$-induced neuronal cell injury and apoptosis as well as promote the survival of neuronal cells, subsequently improving cognitive decline in AD, partly because of PPAR-${\gamma}$ activation. The phosphodiesterase III inhibitor cilostazol may be the basis of a novel strategy for the therapy of AD.
The Journal of the Korean bone and joint tumor society
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v.12
no.1
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pp.1-8
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2006
Purpose: To analyze the clinical outcome of stage IIB osteosarcomas arisen in the long bones. Materials and Methods: Since February 1993, 58 Enneking stage IIB osteosarcomas arisen in long bones were managed and followed at least one year at our four university hospitals. There were 48 wide resections, 2 marginal resections and 8 amputations. The mean follow up period was 4.1years (up to 12.2years). Local recurrence, distant metastasis, complication, survival rate, and oncologic and functional results were evaluated. Results: At the last follow up, 35 patients were CDF, 9 were NED, 5 were AWD, 7 were DOD and the remained 2 died with unrelated disease. Eight local recurrences (13.8%) and 18 distant metastases (31.0%) were occurred. Nine infections were developed after 48 reconstructions (18.8%). Overall functional outcome was 24.3 (81.%). The 5 year overall survival rate was 84.6% and the continuous disease-free survival rate was 68.7% at 5 years and 42.3% at 10 years. Conclusion: Forty-six of 58 stage IIB osteosarcomas arisen in long bones (79.3%) showed CDF or NED at an average 4.1 year follow up. Overall 5-year survival rate was 84.6% and overall functional outcome score was 24.3, which were comparable to those of other studies.
Backround : Pneumoconiosis is the parenchymal lung disease that results from the inhalation and deposition of dust, usually mineral dust of occupational or environmental origin. Most of the pneumoconiosis can be categorized to coal workers' pneumoconiosis (CWP) in Korea. No effective treatement is currently available, and the therapy for symptomatic CWP is limited to treatment of complication. Therefore authors analyzed and reviewed clinical features and radiological findings of 95 patients with pneumoconiosis for assessing the prognostic factors in disease progression. Method: We reviewed medical records of 95 cases with pneumoconiosis including history, chest X-ray, pulmonary function test, electrocardiography, AFB stain and culture of sputum, and routine blood examination between June 1995 and June 1997 in Seonam University Namkwang Hospital. Results: All of cases are male(mean age, 57.4 years), 91 cases out of them are miners. The mean duration of exposure to dust is 18.8 years. Major clinical symptoms are dyspnea (100%), sputum (71.6%), chest pain (55.8%), cough (23.2%), and hemoptysis (6.3%). 82 % of cases are over Morgan-Seaton Grade 2 in the degree of dyspnea. Small opacity on chest x-ray is 82.1 % and large opacity is 17.9%. Small opacity has tit type (37.2%), q/q type (25.6%) and r/r type (11.5%). B type is 42.2% in large opacity. For the pulmonary function test, restrictive type is 40.3%, mixed type 19.5% and obstructive type 8.3%. The more increasing chest Xray density, the more decreasing $FEV_1$ (p<0.01). 38% of patients show tuberculosis in chest X-ray, 15.8% positive smear of acid fast bacilli in sputum. The prevalence of pulmonary tuberculosis is high in patients with poor clinical condition. The cases with the active pulmonary tuberculosis have severe dyspnea. Expired cases show 100% and 75% of positive pulmonary tuberculosis in chest X-ray and sputum examination, respectively. 75% of expired cases show the chronic cor pulmonale, who died of acute respiratory failure. Conclusion: These findings indicate that tuberculoois infection has a decisive influence on the progress and prognoois of pneumoconioois.
As a part of investigation on causes of drop in egg production in domestic chicken breeding farm, bacteriological contamination on air, feed, drinking water and artificial insemination instruments of randomly selected three farms was surveyed. Total bacterial population in the air was very high in all of the chicken houses tested and was not significantly different among these farms . However, total bacterial counts in the air of the problem house having egg drop problem and colibacillosis was higher than normal house within the problem farm. Bacterial population in the assorted feed was low before or after administration on the normal farm while it was much more increased after administration than before administration on the problem farm. Bacterial population of the drinking water in the source of water supply was very low and has no differences among farms tested. Also, bacterial population in the normal farm was not significantly different between source of water supply and after administration. However, population of total bacteria and coliform bacteria after administration was increased. Bacterial population was much higher in the artificial insemination instrument of problem farm than normal farm. However, this bacterial population in the problem farm was decreased to those of normal farm after these instruments were sanitized.
Sex and its tropic hormones influence the lacrimal system, corneal anatomy and disease, aqueous humor dynamics and glaucoma, crystalline lens and cataract, and retinal disease. Dry eye occurs especially frequently during pregnancy, oral contraceptive use, and after menopause, during which androgen levels decrease. Androgen control development, differentiation, and lipid production of sebaceous glands throughout the body, and androgen deficiency also leads to meibomian gland dysfunction and evaporative dry eye. On the other hand, estrogen causes a reduction in size, activity, and lipid production of sebaceous glands. Sex and its tropic hormones also influence the corneal anatomy and disease, and corneal thickening occurred on the second day of the menstrual cycle and around the time of ovulation and appeared to be related to estrogen levels. Fuchs' dystrophy is more commonly seen in postmenopausal women than men and may be linked to hormonal changes that occur with aging. In addition, overexpression of estrogen and progesterone receptors in the conjunctiva of vernal keratoconjunctivitis patients. Serum progesterone levels also may be associated with intraocular pressure especially in pregnant women, and for the women. For women with cataracts, hormone levels were typical of menopause, and there was a significant negative correlation between estradiol and follicular stimulating hormone levels. In addition, serum testosterone levels are associated with the development of diabetic retinopathy. Although the role of sex hormones on the eye is largely unknown, and the results should be interpreted with caution until replicated, the functions of sex hormones in ocular disease remains to be investigated, because they may be involved in structure and function of the ocular components, which are important in the pathogenesis of ocular disease.
Objectives : Cognitive complaints are reported frequently after breast cancer treatments. The causes of cognitive decline are multifactorial, a result of the effect of cancer itself, chemotherapy, and psychological factors such as depression and anxiety. However, cognitive decline does not always correlate with neuropsychological test performance. The purpose of this study was to examine the relationship of subjective cognitive decline with objective measurement and to explore associated factors of cognitive function in breast cancer survivors. Methods : We included 29 breast cancer survivors who complain cognitive decline at least 6 months after treatment and 20 age-matched healthy controls. Neuropsychological tests were performed in all participants. Multivariable regression analysis evaluated associations between neuropsychological test scores and psychological distress including depression and anxiety, also considering age, education, and comorbidity. Results : There were no statistically significant differences in neuropsychological test performances. However, the breast cancer survivors showed a significantly higher depression(p=0.002) and anxiety(p<0.001) than the healthy controls did. Among the cancer survivors, poorer executive function was strongly associated with higher depression(${\beta}=-0.336$, p=0.001) and anxiety(${\beta}=-0.273$, p=0.009), after controlling for age, education, and comorbidity. In addition, poorer attention was also significantly related with depression(${\beta}=-0.375$, p=0.023) and anxiety (${\beta}=-0.404$, p=0.013). Conclusions : The results of this study showed the discrepancies between subjective complaints and objective measures of cognitive function in breast cancer survivors. It suggests that subjective cognitive decline could be indicators of psychological distress such as depression and anxiety.
Purpose : Juvenile rheumatoid arthritis(JRA) is one of the most common rheumatic diseases of childhood and is an important cause of short- and long-term disability. The purpose of this study was to determine the disease course and outcome in childhood patients with JRA. Methods : Fifty nine patients with JRA who were diagnosed and treated in the Department of Pediatrics, Asan Medical Center from August 1990 to November 2004 were enrolled in this study. Sex, age, type, affected joints, extra-articular manifestations, laboratory and radiologic findings, treatments, and outcomes of JRA patients were reviewed retrospectively. Results : Among JRA patients, 32.2 percent had pauciarticular type, 30.5 percent had polyarticular type and 37.3 percent had systemic type. The ratio of boys to girls was 1.7 : 1 and the mean age at onset was $9.3{\pm}3.7$(1.3-15.9) years. The most commonly affected joints were knee, ankle and wrist. The extra-articular manifestations observed were fever, rash, myalgia and lymph node enlargement, etc. The main laboratory findings observed were leukocytosis, anemia, thrombocytosis, elevated ESR, and elevated CRP. Rheumatoid factor and antinuclear antibody(ANA) were positive in 5.3 percent and 18.0 percent. Nonsteroid anti-inflammatory drugs(NSAID) were used most frequently and methotrexate with or without steroids was added in 27.1 percent of patients unresponsive to NSAID. 88.1 percent of patients were cured without functional disability and only one patient was in functional status IV. One patient, who had pulmonary involvement, died. Conclusion : Our results showed an even distribution in type of onset, male predominance, older age of onset, low incidence of iridocyclitis, and low positivity of ANA in JRA patients; this differs from occidental data. This study may suggest regional differences and variability in disease groups of JRA among different racies, but further multi-center trials and large scale epidemiological studies are needed to confirm our conclusion.
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