• Childhood Kidney Diseases
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• 제25권1호
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• pp.29-34
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• 2021

C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80-150 mg/dL) and 22 mg/mL (17-40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.

• Journal of Genetic Medicine
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• 제20권1호
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• pp.6-14
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• 2023

Fabry disease (FD), a rare X-linked lysosomal storage disorder, is caused by mutations in the α-galactosidase A gene gene encoding α-galactosidase A (α-Gal A). The functional deficiency of α-Gal A results in progressive accumulation of neutral glycosphingolipids, causing multi-organ damages including cardiac, renal, cerebrovascular systems. The current treatment is comprised of enzyme replacement therapy (ERT), oral pharmacological chaperone therapy and adjunctive supportive therapy. ERT has been introduced 20 years ago, changing the outcome of FD patients with proven effectiveness. However, FD patients have many unmet needs. ERT needs a life-long intravenous therapy, inefficient bio-distribution, and generation of anti-drug antibodies. Migalastat, a pharmacological chaperone, augmenting α-Gal A enzyme activity only in patients with mutations amenable to the therapy, is now available for clinical practice. Furthermore, these therapies should be initiated before the organ damage becomes irreversible. Development of novel drugs aim at improving the clinical effectiveness and convenience of therapy. Clinical trial of next generation ERT is underway. Polyethylene glycolylated enzyme has a longer half-life and potentially reduced antigenicity, compared with standard preparations with longer dosing interval. Moss-derived enzyme has a higher affinity for mannose receptors, and seems to have more efficient access to podocytes of kidney which is relatively resistant to reach by conventional ERT. Substrate reduction therapy is currently under clinical trial. Gene therapy has now been started in several clinical trials using in vivo and ex vivo technologies. Early results are emerging. Other strategic approaches at preclinical research level are stem cell-based therapy with genome editing and systemic mRNA therapy.

• 한방안이비인후피부과학회지
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• 제29권4호
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• pp.1-13
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• 2016

Objectives : The purpose of this study is to compare hair and alopecia of Korean Medicine with those of western medicine. Methods : We studied relationships between hair and essense(精), qi(氣), blood(血), five vicera(五臟) and meridians(經絡) through literature review about hair and alopecia. We compared Korean medicine with western medicine on physiology of hair and treatment of alopecia based on the study. Results & Conclusion : 1. Congenital essense(先天之精) is related with genetic factor and acquired essence(後天之精) is connected with nutritional factor. Defending function of Defense qi(衛氣) is related with immune reaction and qi stagnation(氣鬱) is associated with stress reaction. Atrophy of vascular tissues observed in alopecia scalp means deep relationship between blood(血) and alopecia, further deficiency of blood can cause telogen effluvium. 2. Kidney qi(腎氣) is related with inhibiting combination of Androgen receptor and Dihydrotestosterone(DHT) or activating hair growth factior. Pi(脾) is connected with alopecia seborrheica caused by damp-heat(濕熱) and alopecia areata caused by excessive prudence(思慮過度). Heart(心) is associated with atrophy in vascular tissue of scalp and liver(肝) is connected with metabolism. 3. Armpit hair and pubic hair as secondary sex characteristics are realated with Yangming Meridian(陽明經) and beard and hair at crown part where Type II $5{\alpha}$-reductase is activated much are associated with Taiyang Meridian(太陽經). 4. Juglandis Semen pharmacopuncture and Ganoderma lucidum pharmacopucture have better effects on inhibit $5{\alpha}$-reductase than Finasteride. Minoxidil and PRP are similar with promoting blood flow and removing stasis(活血祛瘀). Seven-star needling(七星針) is similar with microneedling. 5. Alopecia can be caused by due to lack of circulation Views we need solution to improve circulation coincide in opinion with Korean Medicine and Western medicine.

• 한국가금학회지
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• 제42권3호
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• pp.239-245
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• 2015

Transgenic animals have been widely used for developmental biology studies, as disease models, and even in industry such as transgenic bioreactor animals. For transgenic birds, quail has the great advantages of small body size, short generation time, and frequent egg production. To date, retroviral or lentiviral transduction has been used to generate transgenic quail for various purposes. However, the efficiency of transgenic offspring production with these methods is relatively low and viral vector usage has safety issues. Unfortunately, non-viral transgenesis has not been established in quail due to a deficiency of stem cell and germ cell culture systems. In this study, we established a direct in ovo lipofection method that could be used to create transgenic quail without germline-competent cells or viruses. To optimize the injection stage during embryo development, the liposome complex (containing piggyBacCMV-GFP and transposase plasmids) was introduced into an embryonic blood vessel at 50 hr, 55 hr or 60 hr. GFP expression was detected in various tissues (heart, kidney, liver and stomach) on day 12 of incubation under a fluorescence microscope. Additionally, GFP-positive cells were detected in the recipient embryonic gonads. In conclusion, the direct in ovo lipofection method with the piggyBac transposon could be an efficient and useful tool for generating transgenic quail.

• 동의생리병리학회지
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• 제23권6호
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• pp.1292-1298
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• 2009

Sipjeon-Daebo-Tang (SDT) is indicated for deficiency syndrome of both gi and blood, marked by pale or sallow complexion, dizziness, lassitude, shortness of breath, dislike for talking, poor appetite, pale tongue with thin whitish fur, thready and weak pulse. Gami-Sipjeon-Daebo-Tang(GSDT) is composed of 10 herbs within SDT and Cervi Pantotrichum Cornu (CPC). CPC can noursh kidney-yang, promote the production of the essence and blood, strengthen tendons and bones. Recently SDT is known as anti-cancer drug. Especially CPC is reported to have anti-oxidative action. For these reasons, we investigated the protective effects on cell death induced by chemicals such as paraquat, hydrogen peroxide and anti-oxidative effects in C6 glioma cells. In our results, GSDT accelerated proliferation rates of C6 cells in vitro. In addition, protective effects on cell death induced by hydrogen peroxide and rotenone. In addition, SOD activities were increased by treatment with both SDT and GSDT. In conclusion, these results suggest the possibility of GSDT to protect brain cell or neuronal cell from damage induced by oxidative stress. And also suggest that related mechanisms are involved in SOD activities.

• Childhood Kidney Diseases
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• 제8권1호
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• pp.57-62
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• 2004

Schinzel-Giedion 증후군은 상염색체 열성 유전을 하는 것으로 추정되는 매우 드문 질환으로서 선천성 수신증, 골격계 이형성, 심한 발달 지연 등이 특징인 이형 증후군이다. 저자들은 유전질환의 병력이 없는 건강한 부모에서 태어난 후 진단된 Schinzel-Giedion 증후군으로서 신 수질의 석회화와 K. pneumoniae에 의한 요로감염이 추가로 발생한 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 동의신경정신과학회지
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• 제15권2호
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• pp.211-219
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• 2004

상기 환자는 $4{\sim}5$년 발한 공황장애로 로컬 정신과에서 1년간 po. med 하시다 별무 호전하시고, 입원 3일전 증세 심하여 본원의 한방치료를 받은 환자로, DSM-4에 의거하면 광장공포증을 동반한 공황장애 환자로 한방적으로는 신음양구허(腎陰陽俱虛)에 간울화화(肝鬱化火)를 겸하였다고 하겠다. 지원탕(志遠湯)을 투여하고, 변증에 따라 자침하며, 주관법(走罐法)을 시행하고, 더불어 인지행동치료를 병행하여 PDSS상 21점이 13점으로 감소하는 좋은 결과를 내어 이를 보고하는 바이다. 하지만 관찰 기간이 그리 길지 않았고, 한방치료와 인지행동치료를 함께 시행함으로써 이상의 치료들 중 어떤 치료가 어느 정도의 효과를 내었는지 그 정도를 측정할 수 없으며, 측정절차도 환자의 주관적인 증상의 호소에 의존하여 객관성이 다소 결여되었다. 이를 보완하기 위해 공황장애에 대한 한방치료, 인지행동치료, 그리고 두 가지를 병행한 치료를 각각 시행한 후 효과를 후위분석연구를 통해 검증한다면 한방치료와 인지행동치료가 각각 어느 부분에 어느 정도의 치료효과를 발생시켰는지 검증할 수 있을 것이다. 이를 위한 향후 객관적이고 지속적인 연구가 필요하리라 사료된다.

• Journal of Acupuncture Research
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• 제26권3호
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• pp.67-80
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• 2009

Objectives : The purpose of this survey is the development on the neck pain for acupuncture and moxibustion clinical guideline. Methods : The survey questionnaire was developed by the committee of experts who major in acupuncture & moxibustion or statistics for acupuncture clinical trial protocol development. The questionnaires were distributed via e-mail to 75 members of Korean Acupuncture & moxibustion society. 57 members completed answers, and the computerized data were analyzed by SPSS 17.0 statistical program. Results and Conclusions : 1. The first selected pattern identification on the neck pain This study shows that the meridian pattern identification was selected 35 times(61.4%), the pattern identification based on cause of disease was selected 8 times(14.0%), the visceral pattern identification was selected 7 times(12.3%), the other pattern identification was selected 4 times(7.0%), Qi blood yin yang pattern identification was selected 2 times(3.5%), according to symptoms was selected 1 time(2.4%). 2. Meridian pattern identification Small intestine meridian of hand Taeyang was used 39 times(18.1%), Large intestine meridian of hand Yangmyeong and Bladder meridian of foot Taeyang was used 34 times(15.7%), Gall-bladder meridian of foot Soyang was 32 times(14.8%), Tripple energizer meridian of hand Soyang was used 31 times(14.4%), Governor meridian was used 30 times(13.9%), Lung meridian of hand Taeeum was used 8 times(3.7%), Heart meridian of hand Soeum and Pericarduim meridian of hand Gworeum was used 4 times(1.9%). 3. Pattern identification based on cause of disease Wind-Cold-Dampness was used 31 times(17.5%), Accumulation of the collateral by Phelgm-Dampness was used 16 times(14.0%), affection by exopathogen Wind-Cold(stiff neck, sprain of cervical) was used 13 times (11.4%), Defecient-Cold was used 10 times(8.8%), affection by exopathogen Wind-Dampness was used 9 times(7.9%), Deep Invasion by Wind-Cold was used 8 times(7.0%), Wind-Cold was used 7 times (6.1%), Wind-Cold was used 6 times(5.3%), Accumulation in the Center by Phelgm-Dampness, Imparement of bou fluid by Pathogenic Heat, Wind-Heat with Dampness was used 5 times(4.4%), affection by exopathogen Wind-Dampness and Accumulation of the collateral by Wind-Cold was used 4 times(3.5%), Invasion of Dampness-Heat was used 2 times(1.8%). 4. Visceral pattern identification Rising of the Liver yang was used 16 times(41%), Yin deficiency of Liver and Kidney+pathogens was used 15 times(38.5%), Yin deficiency of Liver and Kidney was used 8 times(20.5%) on this survey.

• Journal of Nutrition and Health
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• 제18권2호
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• pp.126-138
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• 1985

한국산 두류중 대두, 팥, 녹두 및 강남콩의 영양학적 가치를 흰쥐를 이용한 두차례의 동물실험을 통하여 알아보았다. 첫번째 실험에서는 젖뗀 albino rat을 사용하여 단백질원을 모두 생두류 단백질로 하여 식이의 15 %가 되도록 하였으며 각 두류별 영양 저해 정도를 파악하고 이러한 저해가 두류의 methionine결핍인지 또는 methionine을 포함한 다른 아미노산들과의 복합적인 결핍인지를 알아보고자 각 두류별로 대조군, methionine첨가군, 단백질 첨가군으로 나누어 각각 methionine 0.18%와 북어 단백질 5%를 첨가해 준 후 4주간 사육하였다. 두번째 실험에서는 생두류 대신 익힌 두류를 사용하였으며 나머지는 첫번째 실험과 같은 조건으로 하여 영양저해 요인의 소실정도를 파악하고자 하였다. 각각의 실험에서 성장에 미치는 영향을 알아보기 위하여 식이섭취량, 체중, 식이와 단백질의 소화흡수율 및 식이의 효율 (FER), 단백질의 효율(PER)둥을 구하였고 췌장 무게를 측정하여 비대여부를 관찰하였다. 그 결과를 요약하면 다음과 같다. 1) 생두류 섭취군에서 성장저해가 나타났고 이 중 강남콩이 가장 심하여 100%의 치사율을 보였으며 팥도 대두와 녹두에 비해 심한 저해를 보였다. 이러한 성장저해는 methionine이나 북어 단백질 첨가에 의해 극복되지 않아 그 저해요인이 아미노산 결핍이라기 보다는 생두류중에 함유되어 있는 독성물질들로 말미암아 식이의 소화흡수율 및 단백질의 소화흡수율이 저하되었기 때문인 것으로 생각된다. 이 중 특히 생강남콩의 심한 성장저해 및 치사의 주원인은 hemagglutinin 으로 인한 흡수방해라고 생각된다. 2) 또한 생팥 섭취군에서 체중감소 및 치사등 성장저해효과가 생대두에서 보다 더 심하게 나타났는데 생팥의 성장저해의 일차 원인은 생강남콩과는 달리 tryp-sin저해제에 의한 단백질 소화방해라고 볼 수 있다. 또 생팥은 북어 단백질 첨가에 의해 이러한 소화흡수 저해현상이 뚜렷이 극복된 것으로 보아 methionine외의 여러 아미노산의 부족도 한 원인으로 생각할 수 있다. 3) 생두류 섭취군에서 췌장의 비대현상을 볼 수 있었으며 이와 같은 현상은 methionine 첨가에 의해서는 방지되었으나 북어 단백질 첨가로는 유의적인 차이가 없었다. 4) 익힌두류를 섭취한 군들은 생두류 섭취군에 비해 체중이 증가하였고 치사율도 훨씬 감소하였으며 췌장도 비대해 지지 않아 두류독성물질인 trypsin저해제와 hemagglutinin은 파괴된 것으로 간주할 수 있겠으나 팥을 제외하고는 식이나 단백질의 소화율 및 효율은 크게 향상되지 않았다. 또 익힌두류에 methionine 이나 북어 단백질을 첨가하였을 때 식이의 효율 및 체중의 증가가 뚜렷하였으나 강남콩군 외에는 단백질의 소화 흡수율 및 효율은 크게 향상되지 않아 단백질의 소화 흡수율을 떨어뜨리는 요인에 관한 연구가 집중적 으로 이루어져야 하리라고 생각된다.

• 혜화의학회지
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• 제7권2호
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• pp.609-620
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• 1999

Over the period between January 1997 and December 1998, herbal medicine was more than three times administered to the patients coming to Na dong gyu's Oriental Medical Clinic on account of the symptom of growth deficiency. According to radiological opinions about the patients providing cooperation for measuring their height and weight as well as their bone age every three months, it was found that the growth plates were not closed. A research was conducted for 200 children randomly selected of patients in prepuberty (they grew by less than 5cm a year before treatment at a age of 12years for female children and 14years for male children). As a result, the following conclusion was drawn: 1. The randomly selected subjects were made up of 116 male and 84 female children in terms of gender. The age direstribution was most 10 to 12 years in 86 children(34.00%), followed by 8-10 years(27.50%) and 12 to 14 years(19.50%). 2. Considering the distribution of sick children's parental height, the fathers of 141 children(70.50%) measured less than 170cm high, the subaverage height, while the mothers of 172 children(86%) measured less than 160cm high, the subaverage height. It was shown that sick childen's height was genetically influenced by their parents. 3. Children patients's weight at a time of birth was most 3.1-3.5kg for 85 children(44%) and less than 2.5kg which came under the range of growth dificiency for 19 children(9.5%). 4. The highest proportian of the children patients with growth dificiency(56.33%) had the symptom of digestive disorders, of which 77 children patients(18.78%) had anorexia, 16.59% of children patients had the high level of respiratory disorders. Both the digestive disorder and the respiratory disorder put together, they had the high rate of 72.92%. Therefore, this indicates that both the digestive disorder and the respiratory disorder have a great effect on children's growth dificiency. 10.74% of chilren patients were shown to have allergic disorders, which indicates that they also exert an effect on growth deficiency. Specifically, 7.07% of the children patients had the high level of obesity, which shows that an excessive uptake of nutrition may rather induce children to have growth dificiency though an appropriate amount of nutritional uptake is necessary. 5. Comparing their bone age and their chronological age, 58 children patients(29.00%) showed that they were the same at the highest percent. 79 children patients(39.50%) showed that thier bone age was lower than their chronological age. And 63 children patients(31.50%) showed that their bone age was higher than their chronological age. 6. As regards the prescription administered to children patients for treating their growth dificiency, Growth tang A related to the kidney, the congenital factor, of the causes for growth dificiency in traditional Oriental Medicine was administered to 108 children patients(54%), whereas the Growth tang B related to the spleen, the acquired factor, was administered to 92 children patients(46%). 7. 116 male children patients with growth dificiency had the average value of growth for one year before treatment, 4.39cm, while 84 female children patients had the average value of growth for one year before treatment, 4.24cm. A total of 200 children had the average value of growth for one year before treatment, 4.33cm. The annual average value of growth in growth curve surveyed by the Korea Pediatrics Society was 5.79cm. Compared to this value, the one year average value of growth for 108 male and female children patients taking Growth tang A was shown be 8.44cm, which indicates a greater growth by 4.12cm(95.37%) in comparison with the average value of growth before treatment, 4.32cm, and a greater growth by 2.65cm(45.77%) compared to 5.79cm, the average value of growth in growth curve surveyed by the Korea Pediatrics Society. Also, the average value of growth before treatment for 92 male and female children patients taking Growth tang B was shown to be 8.47cm, which indicates a greater increase by 4.15cm(96.06%) compared to 4.32cm, the average value of growth before treatment and a greater increase by 2.67cm(46.29%) in comparison with 5.79cm, the average value of growth in growth curve surveyed by the Korea Pediatrics Society. Considering the average value of growth for male and female children patients taking Growth tang A and B, it was shown to be 8.46cm, which indicates a greater increase by 4.14cm(95.81%) compared to 4.32cm, the average value of growth before treatment, and a greater increase by 2.67cm(46.11%) compared to 5.79cm, the average value of growth in growth curve surveyed by the Korea Pediatrics Society.