• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.9-17
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• 2015

Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.166-173
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• 2003

Purpose : The cause and pathogenesis of $Henoch-Sch\"{o}nlein$ purpura has been studied for many years but the results are disappointing. Recently the hypothesis that abnormalities involving the glycosylation of the hinge region of immunoglobulin Al(IgAl) may have an important role in the pathogenesis of $Henoch-Sch\"{o}nlein$ purpura is being approved. $Henoch-Sch\"{o}nlein$ purpura is the most common vasculitis Ihat affects children and the prognosis is good. But if kidney invovement occurs, the course may be chronic and troublesome. So we evaluated children with $Henoch-Sch\"{o}nlein$ purpura especially from the point of epidemiology and clinical manifestations. Methods : Investigation of 124 children who were diagnosed with $Henoch-Sch\"{o}nlein$ purpura at Inje University Ilsan Paik Hospital from December 1999 to July 2003 was performed retrospectively through chart review. Efforts were made to get informations about the profile, epidemiology, clinical manifestations, progress of the disease and recurrence rate of patients. Results : The patients were 69 boys and 55 girls, with a mean age of $6.1{\pm}2.7$ years at the time of data collection. The male to female ratio was 1.25 : 1. The occurrence rate was much higher in autumn(from September to November, 31.5%) and winter(from December to February, 28.2%) than in spring and summer, with a peak in November. Joint involvement was shown in 66.9% of patients mostly on the foot/ankle(75.9%), knee(39.8%). Seventy(56.5%) out of 124 patients had abdominal pain and 10 patients(8.1%) showed bloody stools. Renal involvement was observed in 24 patients(19.4%) after 21.1 days on the average. IgA was elevated in 10 of 21 patients(47.6%). $C_3$ and $C_4$ levels were normal in 40 of 49 patients (81.7%) and 47 of 48 patients(97.9%), respectively Antistreptolysin-O(ASO) titer was elevated over 250 Todd units in 29 of 62 Patients(46.8%). Mycoplasma antibody titer was elevated in 21 of 49 patients(42.9%) equal or greater than 1:80. Radiologic studies were peformed in 23 patients. Seven patients(30.4%) showed bowel wall thickening and one of them received intestinal resection and anastomosis operation due to terminal ileum necrosis. Eighty four patients took steroid 1.4 mg/kg/day in average. Recurrence rate was 2.5 in 37 patients(29.8%). Conclusion : $Henoch-Sch\"{o}nlein$ purpura in childhood appears most in about 6 years of age. The occurrence rate is much higher in autumn and winter relatively. Diagnosis can be made through the perspective history taking and the inspection of clinical manifestations, but the laboratory findings are not of great help. A small portion of the patients might show abdominal pain or arthritis before purpura develops, therfore various diagnosis can be made. Radiologic evaluation should be performed to avoid surgical complications in cases accompanying abdominal pain, and long term follow up should be needed especially in patients suffering from kidney involvement. In about 30% of the patients $Henoch-Sch\"{o}nlein$ purpura would recur. Steroid can be used safely without side effects.

• Pediatric Infection and Vaccine
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• v.4 no.2
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• pp.210-217
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• 1997

Purpose: To prevent residual physical disability and chronic infection, prompt diagnosis and adequate treatment are important in the skeletal infections in children. Although radioisotope scanning is knwon as the method of choice for early diagnosis of bone infection, we conducted a study on twenty nine children who had skeletal infections to reevaluate the most appropriate way in diagnosis and management. Methods: A retrospective study was conducted on twenty nine children, who were admitted to the departments of Pediatrics and Orthopedic Surgery and who had acute osteomyelitis or septic arthritis, through review of medical records, radiologic & radioisotope study results. Their diagnoses were confirmed by bacteriologic cultures on the aspirated specimens from suspected bony lesions. Results: 1) Among twenty nine patients, there were 6 infants including 5 newborn infants, and 23 children were aged between 1 and 15 years. Male to female ratio was 1.4 to 1. 2) Point tenderness was noted in all cases, and the common physical signs were swelling, limitation of motion, fever and local heat in the order of frequency. 3) Fifty two percents of the patients were diagnosed within a week after onset of symptoms and all cases were within 15 days. 4) Leukocytosis was noted in only 58.6% of cases but erythrocyte sedimentation rate was increased in all cases except only one case. Staphylococcus aureus was revealed as the most common etiologic agent. 5) Radioisotope scans showed hot uptake in five of six cases(83.3%) who had no abnormal finding on plain skeletal radiolograms. Conclusions: Although radioisotope scan and MRI are helpful in early diagnosis before radiologic finding was detected on plain X-ray film, the antimicrobial therapy can be started after bacteriologic study of the aspirated specimens from the suspected skeletal lesions if skeletal infection is highly suspected clinically.

• Journal of muscle and joint health
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• v.3 no.1
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• pp.90-103
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• 1996

The purpose of this paper is to compare two approach to assessment of coping patterns. The sampling method was a purposive sampling technique. The study participants were out patients 113 of rheumatoid arthritis center of one University hospitals in Seoul. Datas were collected from Nov. 13 to Nov. 24, 1995. The instruments used for this study were Graphic Rating Scales of pain, The Beck Depression Inventory and Coping Patterns tool. The collected data were analyzed for frequency, means, SD, factor analysis., Pearsons' correlations and ANOVA. The results were summerized as follows ; 1. When raw scores were used : there were not correlation in all three coping patterns. 2. When relative scores were used : there were significantly correlated in all three coping patterns. 1) Active coping and Positive-cognitive coping (r=-0.352, p< 0.0001) 2) Positive-cognitive coping and Negative-cognitive coping (r=-0.594, p< 0.0001) 3) Active coping and Negative-cognitive coping(r=-0.544, p< 0.0001) The results of this research with relative scales provided more insight into the correlation in all three coping patterns. 3. Pearsons' Correlations were computed for each coping pattern, age, pain level, duration of pain and BDI. 1) Using raw score : (1) Active coping was significantly related to pain level(sensory score ; r=0.268, p<0.05, affective score ; r=0.266, p< 0.05) (2) Positive-cognitive coping was significantly related to age (r=-0.252, p< 0.05), pain level (sensory score ; r= -0.244, p< 0.05) (3) Negative-cognitive coping was significantly related to depression level (r=0.312 p< 0.0001). 2) Using relative score (1) Active coping was significantly related to pain level(sensory score ; r=0.299, p<0.05, affective score ; r=0.246, p< 0.05) (2) Positive-cognitive coping was significantly related to age (r= -0.187, P< 0.05), pain level (sensory score ; r=-0.317, p<0.0001, affective score : r=-0.305, p<0.0001) and depression level(-0.339, p<0.0001)) (3) Negative-cognitive coping was significantly related to depression. level(r=0.313, p<0.0001). 4. When raw and realtive coping scores were compared to those of age groups, religious groups and BDI level(high, middle, low) ; 1) Using raw score : (1) Active coping : there were not significantly difference (2) Positive-cognitive coping ; 20-39 age group and 50-59age group had significantly higher scores than over 60age group. BDI-low level group had significantly higher scores than other groups. (3) Negative-cognitive coping : 20-39age group and over 60age group had significantly higher scores than 40-49age group. Non-religious group had significantly higher scores than christian group. BDI-high level group had significantly higher scores than other groups. 2) Using relative score : (1) Active coping : over 60 age group had significantly higher scores than 20-39 age group and 40-49age group had significantly higher scores than 20-39 age group (2) Positive-cognitive coping ; 40-49age group, 20-39age group and 50-59age group had significantly higher scores than over 60age group. Christian group had significantly higher scores than non-religious group. BDI-low level group had significantly higher scores than other groups. (3) Negative-cognitive coping ; Non-religious group had significantly higher scores than christian group and buddhistic group. BDI-high level group had significantly higher scores than other groups. The current data suggest that relative scores may yield a different perspective on coping patters than raw scores. The use of relative scores reveals the relation clearly, without its being blurred statistically by the effect of other coping strategies or being relegated to a partial correlation. The use of relative scores holds promise for delineating the relations between ways of coping and health-related behavior.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.31 no.3
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• pp.50-59
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• 2018

Objectives : The aim of this review is to investigate studies on skin adverse reactions and to demonstrate subjects related to the adverse effects in dermatology. Methods : Electric searches were performed with KISS(Korean studies Information Search System) and the key words were combination of 'skin' and 'adverse effect'. 87 literatures investigated in this review were issued from 1900 to 2016. Results : Among the 87 papers, dermatologic adverse reactions were reported in 83 papers in medicine, accounting for 95.4%. Of the adverse effects seen on the skin, 84 discoloration such as erythema, pigmentation and hemotelangiosis were the most common, accounting for 21%. Among the medical adverse reactions not seen on the skin, 21 infection were the most common, accounting for 25%. Among the subjective adverse reactions, of which 32 pruritus were the most common, accounting for 43%. Among the 87 papers, there were 3 cases with underlying diabetes and 3 cases with underlying hypertension, followed by 2 cases with chronic renal failure, HBV, atopic dermatitis and respectively 1 case with alcoholism, depression addiction, multiple myeloma, arthritis and psoriasis. The most frequent period until adverse reactions appeared was within 2 weeks, accounting for 13 papers. And 4 were the most frequent adverse reactions lasting less than 1 month, and 4 were more than 3 months and less than 6 months. There were 48 cases where adverse reactions were caused by nonmedical practioner's treatment. The adverse reactions by the pharmacist were the highest at 11 cases (23%). There were 17 cases of adverse reactions due to medical treatment, among which dermatologists and nondermatologists accounted for the majority of 5 cases, 29%. The most common cause of adverse reactions was the application of external medicine (41 cases), followed by 36 cases of foreign body implantation, eyebrow tattooing, ear piercing, etc. Conclusions : In this report, we demonstrated patterns of adverse reactions in the medical field of dermatology caused by non-medical personnel than medical personnel. We suggest that more effort should be followed by medical personnel to establish clear awareness of skin disease and by patients to be aware of the risks of the illegal medical treatment by non-medical personnel.

• Tuberculosis and Respiratory Diseases
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• v.56 no.2
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• pp.203-209
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• 2004

Background : Methotrexate (MTX) has been used to treat a wide range of malignant and benign diseases including osteosarcoma, advanced stage non-Hodgkin's lymphoma, psoriasis, severe rheumatoid arthritis, sarcoidosis, and Wegener's granulomatosis. MTX-induced lung injury occurs in up to 10% of treated patients. Although both acute and chronic presentations have been described, typical manifestation of MTX-induced lung injury is subacute with symptoms usually developing within several months after starting therapy. Nonspecific interstitial pneumonia (NSIP) is the most common histopathologic manifestation of MTX-induced lung disease, while bronchiolitis obliterans organizing pneumonia (BOOP) and diffuse alveolar damage (DAD) are less common. Granuloma formation is reported in 34.7%. In Korea, Two reports of MTX pneumonitis have been published. The one presented with NSIP and the other with DAD. We recently experienced a case of MTX pneumonitis with presentation of hypersensitivity pneumonitis.

• Journal of the Korean Arthroscopy Society
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• v.11 no.1
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• pp.39-44
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• 2007

Purpose: To evaluate the results of subtalar arthroscopy and to define the indications for the procedure. Materials and Methods: Fifty-four patients were followed up for more than 1 year after subtalar arthroscopy. The mean age was 40 years, and the mean follow-up period was 18 months. Preoperative diagnoses included sinus tarsi syndrome in 19 cases, degenerative arthritis in 13 cases, calcaneal fracture in 10 cases, arthrofibrosis in 5 cases, os trigonum syndrome in 3 cases, talar fracture in 3 cases, talocalcaneal coalition in 3 cases and calcaneal tumor in 1 case. Clinically AOFAS ankle-hindfoot scale and satisfaction of the patients were evaluated. Results: There were 23 synovectomies, 13 subtalar fusions, 11 diagnostic arthroscopies, 5 adhesiolyses, 4 loose body removals, 3 excisions of os trigonum and 1 arthroscopic reduction of fracture. Twenty-five ankle arthroscopies and 11 modified Brostrom's operations were performed for the accompaning 17 ankle impingment syndromes, 11 chronic ankle instabilities and 7 osteochondral lesions of talus. AOFAS ankle-hindfoot scale was increased from 33 points preoperatively to 77 points postoperatively in subtalar fusion group, and was increased from 69 points preoperatively to 89 points postoperatively in other-than-fusion group. Ninety one percent of patients were satisfied with the procedures. There were no serious complications related to the subtalar arthroscopy. Conclusion: Subtalar arthroscopy appears to be safe and highly accurate procedure for subtalar pathology, although it requires technical expertise.

• Journal of Haehwa Medicine
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• v.9 no.1
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• pp.547-594
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• 2000

I. Introduction The essence of Oriental medicine consists of ancient books, experienced doctors and succeeded skills of common society. Many famous doctors studied medical science by their fathers or teachers. So the history of medical science is long. $\ll$DangDaeMyeongIImJeungJeongHwa(當代名醫臨證精華)$\gg$ written by SaWoogWang(史宇廣) and DanSeoGeon(單書健) has many medical experience of famous doctors. So it has important historical value. Bi(痺) means blocking. BiJeung is one kind of symptoms making muscles, bones and jonts feel pain, numbness or edema. For example it can be gout or SLE etc. So I studied ${\ll}BiJeungJuJip{\gg}$. II. Final Decision Following decisions of Chinese doctors of 20th century are as follows ; 1. JuYangChun(朱良春) emphasized on IkSinJangDok(益腎壯督) to treat BiJeong. And he devided WanBi(頑痺) as PungHanSeup(風寒濕), DamEo(痰瘀), YeolDok(熱毒), SinHeo(腎虛). He used insects for medicine. 2. ChoSuDoek(焦樹德) introduced past prescription. He used ChiBiTang(治痺湯) to treat HaengBi(行痺), TongBi(痛痺), ChakBi(着痺). He insisted that Han(寒; coldness) and Seup(濕; dampness) be Eum(陰) and Pung(風; wind) can change his character to be Eum. After all BiJeung is usually EumJeung. So he used GaeJi(桂枝) and BuJa(附子). By the way he used ChungYeolSanBiTang(淸熱散痺湯) for YeolBi, BoSinGeoHanChiWangTang SaBok(王士福) emphasized on the importance of medicine. He introduced many treatments like CheongYeol(情熱) for YeolBi and YiO(二烏) for HanBi. And he divided BiJeung period for three steps. At 1st step, we must use GeoSa(祛邪), at 2nd step, we must use BuJeong(扶正) and at 3rd step, we must use BoHyeol(補血), he insisted. And he introduced many herbs to treat BiJeung. 4. JeongGwangJeok(丁光迪) said that GaeJi(桂枝), MaHwang(麻黃), OYak(烏蘖) and BuJa(附子) are very important for TongRak(通絡). And pain usually results from Han(寒), so he liked to use hot-character herbs. 5. MaGi(馬志) insisted that BiJeung usually result from ChilJeong(七情). And he liked to use insects for treatment of BiJeung. 6. WeolSeokMu(越錫武) introduced 8 kinds of treatments and divided BiJeung period. Also he divided BeJeung for PungBi(風痺), HanBi(寒痺) and SeupBi(濕痺). 7. SeoGeaHam(徐季含) observed many patients and concluded that 86.7% of BiJeung is HeuJeung(虛症). 8. YuJiMyeong(劉志明) said that YeolBi is important and CheongYeol is also important. So he emphasized on DangGyuiJeomTongTang(當歸拈痛湯) and SeonBiTang(宣痺湯). 9. WangLiChu(汪履秋) studied cause of WanBi. Internal cause is GiHyeolHeo(氣血虛) and GanSinHeo(肝腎虛) and external cause is SaGi(邪氣) he insisted. 10. WangSaSang(王士相) said that YeolBi can be SeupYeolBi or EumHeuYeolBi(陰虛熱痺) and HanSeupBi(寒濕痺) is rare. He use WooBangJaSan(牛蒡子散) and BangPungHwan(防風丸) for SeupYeolBi, DangGyuiSaYeokTang(當歸四逆湯) for HanSeupBi. 11. JinTaekGang(陳澤江) treated YeolBi with BaekHoGaGyeJiTang(自虎加桂枝湯) and SaMyoSan(四妙散). If they don't have effect, he tried to cure BiJeung step by step. And he used e term of GeunBi(筋痺) and BangGiMogwaEIInTang(防己木瓜薏苡仁湯) was good for GeunBi. 12. MaSeoJeong(麻瑞亭) said that PungSeupYeokJeul(風濕歷節) is BiJeung and it is related to GanBinSin(肝脾腎; liver, Spleen, Kindey). And he emphasized on balance WiGi(衛氣) and YoungHeul(營血). 13. SaJeJu(史濟桂) said that GeunGolBi(筋骨痺) is similar to arthritis and sometimes called ChakBi. And SinBi(腎痺) is terminal stage of ChakBi, he said. He also used insects for treatment. 14. JeongJeNam(丁濟南) tried to cure SLE and used GyeJi, CheonCho(川椒), SinGeunCho(伸筋草), SunRyeongBi(仙靈脾), HyconSam(玄蔘) and GamCho(甘草). 15. JinGYungHwa(陳景和) emphasized on diagnosis of tongue. If the color of tongue is blue, it usually has EoHyeol(瘀血), for example. And he also used insects. 16. JuSongI(朱松毅) tried to devide YeolBi with OnByeong(溫病), Wi(衛), Gi(氣) and Hyeol(血). 17. RuDaBong(蔞多峰) said that JyeongHeo(正虛), OiSa(外邪) and EoHyeol are closely related. And he explained BiJeung by deviding the body into the part, for example head, neck, shoulder, waist, upper limb and lower limb. 18. YuMuBo(劉茂甫) defined PungHanSyubBi as chronic stage and YeolBi as acute stage.