• Journal of Audiology & Otology
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• v.23 no.2
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• pp.69-75
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• 2019

Background and Objectives: The antioxidant ebselen will be able to limit or prevent the ototoxicity arising from 2-hydroxypropyl-β-cyclodextrin (HPβCD). Niemann-Pick Type C (NPC) disease is a disorder of lysosomal storage manifested in sphingolipidosis. Recently, it was noted that experimental use of HPβCD could partially resolve the symptoms in both animals and human patients. Despite its desirable effect, HPβCD can induce hearing loss, which is the only major side effect noted to date. Understanding of the pathophysiology of hearing impairment after administration of HPβCD and further development of preventive methods are essential to reduce the ototoxic side effect. The mechanisms of HPβCD-induced ototoxicity remain unknown, but the resulting pathology bears some resemblance to other ototoxic agents, which involves oxidative stress pathways. To indirectly determine the involvement of oxidative stress in HPβCD-induced ototoxicity, we tested the efficacy of an antioxidant reagent, ebselen, on the extent of inner ear side effects caused by HPβCD. Materials and Methods: Ebselen was applied prior to administration of HPβCD in mice. Auditory brainstem response thresholds and otopathology were assessed one week later. Bilateral effects of the drug treatments also were examined. Results: HPβCD-alone resulted in bilateral, severe, and selective loss of outer hair cells from base to apex with an abrupt transition between lesions and intact areas. Ebselen co-treatment did not ameliorate HPβCD-induced hearing loss or alter the resulting histopathology. Conclusions: The results indirectly suggest that cochlear damage by HPβCD is unrelated to reactive oxygen species formation. However, further research into the mechanism(s) of HPβCD otopathology is necessary.

• Korean Journal of Audiology
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• v.23 no.2
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• pp.69-75
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• 2019

Background and Objectives: The antioxidant ebselen will be able to limit or prevent the ototoxicity arising from 2-hydroxypropyl-β-cyclodextrin (HPβCD). Niemann-Pick Type C (NPC) disease is a disorder of lysosomal storage manifested in sphingolipidosis. Recently, it was noted that experimental use of HPβCD could partially resolve the symptoms in both animals and human patients. Despite its desirable effect, HPβCD can induce hearing loss, which is the only major side effect noted to date. Understanding of the pathophysiology of hearing impairment after administration of HPβCD and further development of preventive methods are essential to reduce the ototoxic side effect. The mechanisms of HPβCD-induced ototoxicity remain unknown, but the resulting pathology bears some resemblance to other ototoxic agents, which involves oxidative stress pathways. To indirectly determine the involvement of oxidative stress in HPβCD-induced ototoxicity, we tested the efficacy of an antioxidant reagent, ebselen, on the extent of inner ear side effects caused by HPβCD. Materials and Methods: Ebselen was applied prior to administration of HPβCD in mice. Auditory brainstem response thresholds and otopathology were assessed one week later. Bilateral effects of the drug treatments also were examined. Results: HPβCD-alone resulted in bilateral, severe, and selective loss of outer hair cells from base to apex with an abrupt transition between lesions and intact areas. Ebselen co-treatment did not ameliorate HPβCD-induced hearing loss or alter the resulting histopathology. Conclusions: The results indirectly suggest that cochlear damage by HPβCD is unrelated to reactive oxygen species formation. However, further research into the mechanism(s) of HPβCD otopathology is necessary.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.144-154
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• 2007

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multimodality therapeutic approaches might be important for better outcome.

• Archives of Plastic Surgery
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• v.34 no.1
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• pp.123-127
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• 2007

Purpose: Rigid external distraction(RED) is a highly effective technique for correction of maxillary hypoplasia in patients with cleft or syndromic craniosynostosis. Despite many advantages of RED, it also has the problem of relapse as the conventional advancement surgery. Bicoronal approach, that is the common approach to gain access to the craniofacial skeleton, had some morbidity, such as hair loss, sensory loss, wide scar and temporal hollowing. We present our clinical experience of RED distraction with minimal invasive approach and early rigid fixation to overcome these disadvantages. Methods: A 27-year-old female patient with Crouzon's disease underwent Le Fort III osteotomy and RED device application through the minimal invasive direct skin incisions. After the latent period of 5 days, distraction was undertaken until proper convexity and advancement were obtained. During the rigid retention period, inflammation occurred on the right cheek, and proper conservative managements were done including continuous irrigation. To maintain the stability of distraction, early rigid fixation was undertaken on the osteotomy sites through another skin incisions. Preoperative and postoperative orthodontic treatments were performed. Serial photographs and cephalometric radiographs were obtained preoperatively, after distraction and 6 months after distraction. Results: The cephalometric analysis demonstrated postoperatively significant advancement of the maxilla and improvement of facial convexity. After 6-month follow-up period, the maxilla was stable in the sagittal plane and no relapse was found. Facial scars were not noticeable and other deformity and morbidity did not occur. Conclusion: This effective and stable technique will be a good alternative for the patients who need large amount of distraction and for adult patients with severe maxillary hypoplasia or syndromic craniosynostosis.

• Journal of Korean Medicine for Obesity Research
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• v.9 no.2
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• pp.33-45
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• 2009

Objectives This study was performed to evaluate the relation between hair tissue mineral elements(HTME) and Heart rate variability(HRV) of the metabolic syndrome(MS). Methods 89 persons(41-69 ages) who visited Oriental hospital for medical examination were divided MS group(n=22) and control group(n=67). HTME and HRV were compared, and were analyzed correlation with five contents of the MS. Results (1) In total subjects, waist circumference had a positive correlation with Pb. Blood pressure had a negative correlation with Mg. High density lipoprotein cholesterol(HDL-chol) had a negative correlation with Pb, Sb and K, while positive correlation with Ca/K. Triglyceride(TG) had a negative correlation with Mg and Na/K, while positive correlation with K and Ca/Mg. (2) In total subjects, TG and fasting blood sugar(FBS) had negative correlation with high frequency(HF), while positive correlation with low frequency/high frequency ratio(LF/HF). (3) In MS group, most of the level of toxic minerals were higher, and the level of major nutritional minerals were lower, but there were no statistical signficance. In two groups, there is no contrast between the correlations of the MS contents and HTME. (4) In MS group, HF was significantly lower and LF/HF was higher than normal group. LF/HF had a negative correlation with waist circumference in MS group, while positive correlation in normal group. (5) In total subjects, TP had a positive correlation with Mg. In two groups, there is no contrast between the correlations of the MS contents and HRV. However LF/HF had a positive correlation with Na in MS group, TP and LF had negative correlation with Ca, while RMSSD and HF had negative correlation with Cu in normal group. Conclusion These results may suggest that HTME and HRV are useful in diagnosing and preventing the metabolic syndrome.

• The Journal of Pediatrics of Korean Medicine
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• v.14 no.2
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• pp.149-168
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• 2000

The Purpose of this study was to investigate the new effective oriential medicine tretments in pediatric disease and its clinical applicability The study was composed of 1245 new patients who had been treated at the all unit in the Dong-Seo oriential Medicine Hospitial for 1 year, from 1 April 1999 to 31 March 2000, and aged between 0 and 18 years. The chief complaint was mainly categorized by oral examination on patients and their care-givers. Result 1. The numbers of children who involved in this study is total 1245: Male children are 668 and female children are 577 children. The sex rate between male and female is 1.15 to 1. 2. Most of the above patients came to the hospital at first time from diseases such as musculoskelectal disease, weakness, asthma, cough, anorexia, common cold, rhiorrhea, sweating, dyspepsia, dematitis, night terror, obesity, stomach, short stature, Besides, they also came to the hospital at first time from various diseases such as epistasxis, pyrexia. Bell's palsy, nocturia, contipation, cerebral palsy, disorder, CVA. diarrhea. stress disease, Allergic disease, Tic disorder. Visual disorder, Kawasaki disease, Pierre Robin's syndrom, hematuria, edema and so on. 3. Looking at the frequent diseases, Respiratory dis. children including asthma, cough, rhiorrhea, sweating, common cold, pyrexia covers 36%, in 399 numbers. digestive dis. children including anorexia, dyspepsia, stomach, diarrhea, constipation, indigestion covers 19%, in 211 numbers. cadiovasculary disease children including arrthymia, terror in frequency, night crying, sediation, Tic disorder covers 8%, in 85 numbers. Hepatobiliary disease children including short stature, dizziness, visual difficulty, sprain, disorder of nail covers 21%, in 238 numbers. renal disease. children including nocturia, hematuria, hemation, disorder of hair, menorrhea, cerebral palsy, edema in 44 numbers. Fatigue children covers 13%. in 143 numbers. 4. In case of respatory disease children. total number is 399 children. dematitis children are 108 numbers and asthma children are 96 numbers. These show that children seem to the best have dematitis and asththma. The age from 0 to 6 is 290 numbers, covering 73 percentage. The others are covering 27 percentage. These data demonstrate that the age from 0 to 6 age could easily get these kinds - of diseases. The 29 percent of children had these kinds of diseases in spring. The 28 percent of children had these kinds of diseases in autumn. These show that children seem to frequently have these kinds of diseases in both spring and autumn season.(inter-season) 5. In case of digestive disease, anorexia covers 39%, in 83 numbers, dyspepsia covers 28%, in 59 numbers, anorexia and dyspepsia were the lagest group in digestive disease and the age from 0 to 6 covers 59%, in 125 numbers and the other age covers 39%. According to these data, infant seems to be vulnerable to these kinds of diseases. The 24 percent of children had these kinds of diseases in spring and summer. The 33 percent of children had these kinds of diseases in winter, which means that children mostly had winter. but decreased significant in autumn. 6. In case of cadiovasculary diseases, it can be divided into two categories: night covers 73% and nervous characteristics covers 27%. The age from 0 to 6 occupies 75%, and the other age occupies 25%. These data also show that infants can easily infected with these kinds of diseases. Analyzing by the season, summer could be the most frequent season that children have these kinds of diseases. but decreased in autumn. 7. In case of hepatobiliary children, sprains covers 166 numbers, fatigue covers 32 numbers, epistasxia covers 24 members, the reasion which sprain occupyied most of % were sprain covered ages. The age from 0 to 14 covers 59% and from 15 to 18 covers 41%. In the conclusion the adolescent seems to be vulnerable to sprains. In spring 29%, insummer 31%, in autumn 23%, and in winter 28% of children got these kinds of diseases, which show that children seems to have this kinds of disease in summer season. and decreased in autumn. 8. In case of renal disease. nocturia and hematuria covers 52%(occupied overhalf). The age from 0 to 6 covers 52%(occupied overhalf). Analyzing by season, in spring increased in summer(59%), decreased in autumn(45%) Conclusion 1. The chief complant in pediatric diseases that needed an oriental medical tretment was mainly the disease that tends to take iong time and the weakness. and appeared frequency in respiratory disease : 2. The oriential medical tretment was still preferred as a way to improve the weakness by patients, rather than a way to overcome their disease. In paticular, the study shows that the oriental medical tretment should be emphasized in terms of preventing the disease 3, The new disease, which were developed with the change of human life and envir oment(just like seual disorder, short stature, obesity, dynamic disorder, examinee disease), should be in vestigated as a new field of oriental medical tretment.

• The Journal of Korean Institute of Communications and Information Sciences
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• v.30 no.6C
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• pp.585-592
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• 2005

One of the most important things in the researches on diseases is to attach more importance to prevention of a disease and preservation of health than to treatment of a disease, also to foods rather than to medicines. In this context, the most significant concern in examining a patient is to find the presence of disease, and, if any, to diaguose the type of disease, after which a pharmacotherapy is followed. In this paper, various diagnosis methods of Oriental medicines are discussed. And ocular inspection, the most important method among the 4 disease diagnoses of Oriental medicines, is studied. Observing a person's shape and color has been the major method for ocular inspection, which usually has been dependent upon doctor's intuition as of these days. We are developing an automatic system which provides objective basic data for ocular inspection. As the first stage, we applied the signal processing techniques to automatic feature extraction of faces for ocular inspection. Firstly, facial regions are extracted from the point of frontal view, which was followed by extraction of their features. The experiment applied to 20 persons showed that frontal face regions are perfectly extracted, as well as their features, such as eyes, eyebrows, noses and mouths. Future work will seek to address the issues of morphological operation for a few unfinished extraction results, such as combined hair and eyebrows.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.492-498
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• 1999

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1141-1145
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• 2002

Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal cell carcinoma, intravesical ureterocele, and osteoporosis. The incidence is 1 in 400-1,000 of the population and urological abnormalities are not common. However a case of Klinefelter syndrome associated with multicystic dysplastic kidney has not been not reported up to date. Therefore, we describe a 1-day-year old baby boy who presented with Klinefelter syndrome with unilateral multicystic kidney dysplastic disease, plus with a brief review of the literature.

• Korean Journal of Veterinary Research
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• v.34 no.4
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• pp.787-799
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• 1994

To elucidate morphologic lesion of porcine exudative epidermitis which is occurred sporadically in Korea, Staphylococcus hyicus subsp. hyicus isolated from the naturally affected pigs was inoculated to suckling pigs. The infected piglets were observed grossly and histopathologically. Although affected piglets were taking acute, subacute, or chronic course, some piglets suffered from chronic disease showed poor prognosis and marked growth depression. Affected peglets had erythematous skin on the face, ear, and abdomen and these localized lesions appear as brownish spots of exudative epidermitis and fromed crust in the early stage. But, after this stage, the skin were covered by viscous greasy exudate and formed blackish brown crust and appeared fissures and hypertrophy. Grossly, there has been hemorrhage with the removal of crust-like materials of epidermis and edematous subcutis. The superficial lymph nodes were edematous and swollen or congested and hemorrhagic. Some piglets had swollen ureters, cysts in the renal cortex, or polyarthritis. A few cases had mild edematous swelling of kidney, intestinal catarrh and congestion of brain. Microscopically, skin lesions had detachment of keralinized layer and parakeratosis of epidermis, hydropic degeneration of epidermal cell, and retrogressive degeneration of hair root sheath. Dermis had edema, and infiltration of neutrophils and mononuclear cells. As the disease was proceeded, there was marked perivasculitis with lots of mononuclear inflammatory cells. More chronic lesions formed granuloma-like bodies(nodules) due to more mononuclear, perivascular inflammatory cell infiltration and proliferation of fibroblast. Lots of plasma cells and eosinophils were also present in dermis. Epidermis was hyperplastic by proliferation of basal cells stratum germinativum and epidermal pegs often extended into the dermis. In secondary infection, lots of neutrophils could be seen in epidermis and derms. Kidney had neutrophilic infiltration, necrotic and cystic glomeruli, and dilation of renal tubules and ureters. Purulent arthritis was sometimes observed in joints. Three days old mice administrated Staphylococcus hyicus subsp hyicus subcutaneously before had focal congestion and hemorrhage, necrosis, and subcutaneous edema of the skin. This observation was also seen in the study of mice administrated exfoliatin toxin of Staphylococcus which evoked human staphylococcal scalded skin syndrome.