• 대한유전성대사질환학회지
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• 제16권1호
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• pp.1-9
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• 2016

국내 정부 지원 무료 검사로 진행 중인 6종 신생아 선별 검사에서 호모시스틴뇨증을 진단하기 위해 메티오닌의 증가가 마커로 사용되고 있다. 그러나 실제 고메티오닌혈증에서 감별해야 하는 질환들에는 간질환, tyrosinemia type I (MIM #276700), methionine adenosyltransferase (MAT) I/III 결핍, glycine N-methyltransferase (GNMT) 결핍, adenosylhomocy-steine hydrolase (SAHH) 결핍, adenosine kinase (ADK) 결핍, citrin deficiency (citrullinemia type I) 등이 있다. 고메티오닌혈증의 흔한 원인이자 양성 질환으로 알려졌던 MAT I/III 결핍 질환은 유전 방식에 따라 신경학적 증상 발현 및 치료의 필요성이 보고되고 있어 신생아 선별검사에서 고메티오닌혈증 양성으로 나올 경우 감별 진단 및 처치에 대한 가이드라인이 필요하겠다. 신생아 선별검사에서 고메티오닌혈증 양성으로 나올 경우, 간수치 및 혈장 아미노산 분석, 혈장 총 호모시스테인 수치를 통해 여러 질환 들을 감별할 수 있으며 혈장 총 호모시스테인의 증가가 40 umol/L 미만의 경우에서는 MAT I/III 결핍을 먼저 고려해 볼 수 있겠다. MAT I/III 결핍에서는 우성 유전형일 경우에는 치료가 필요 없지만 열성 유전형에서는 정기적인 발달 지표, 혈장 메티오닌과 총 호모시스테인 수치의 추적이 필요하겠으며 심한 고메티오인혈증(>800umol/L)에서는 저메티오닌식이를, 발달 지연, 뇌말이집 형성 장애가 동반한 경우에는 S-adenosylmethio-nine (SAM) 복용을 고려한다. 호모시스틴뇨증에서는 절반에서 피리독신 반응형을 보이고 피리독신 반응형은 조기에 메티오닌 증가가 없을 수 있기에 선별검사에서 놓칠 수 있다. 치료에는 저메티오닌 식이, 피리독신, 베타인, 엽산 등이 있으며 베타인 투약시 메티오닌 증가로 인한 뇌부종에 대한 주의가 필요하다. 그 외 GNMT, SAHH, ADK 결핍은 현재 환자 수와 예후가 제한적으로 조기 진단 및 치료에 대한 뚜렷한 이득이 명확하지 않은 상태이다. 미국, 유럽의 일부 기관들에서는 낮은 메티오닌 수치로 재메칠화 장애에 대한 선별검사도 시행하고 있어 국내에도 관련 질환에 대한 현황 및 선별검사 도입의 필요성에 대해 논의가 필요하겠다.

• Pediatric Infection and Vaccine
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• 제21권2호
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• pp.104-113
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• 2014

목적: 본 연구는 최근 10년 동안 전주 예수병원에 쯔쯔가무시병으로 입원한 환자를 조사하여 소아와 성인의 임상 양상을 비교하고자 하였다. 방법: 2003년 1월부터 2012년 12월까지, 쯔쯔가무시병의 진단 기준에 포함되는 768명의 환자를 후향적으로 검토하여, 소아 49명, 성인 719명의 임상적 특징과 검사소견 및 합병증 등을 분석 비교하였다. 결과: 연도별 환자의 증가 추세는 소아와 성인 모두에서 뚜렷하게 보이지 않았고, 10월과 11월에 환자가 가장 많이 발생하였다. 남녀비에서 소아는 남아가 많은 반면에, 성인은 여자가 많았고, 거주 지역은 소아가 성인에 비해 도시가 더 많았다. 증상은 소아에서 발진과 가피가 많았고, 근육통은 성인에서 더 많았다. 검사상 소아는 빈혈이 많은 반면에, 혈소판, 간기능, 신기능의 이상 소견이 적었다. 입원 기간과 합병증에서는 성인보다 양호한 결과를 보였다. 소아에서 독시사이클린과 마크로라이드계열 항생제의 치료 효과를 비교하였을 때, 두 약제 간에 유의한 차이는 보이지 않았다. 결론: 쯔쯔가무시병 환자에서 소아는 남아에서 많았고, 발진 및 가피의 발생이 더 많았다. 검사상 이상 소견은 성인에 비해 경하였으며, 입원 기간이 짧고, 합병증이 적으며, 마크로라이드계열 항생제에도 동일한 치료 효과를 보였다. 따라서 유행 시기에, 소아에서 쯔쯔가무시병이 의심될 때에는, 세밀한 이학적 진찰과 혈청학적 검사를 실시하여 빠른 진단을 내릴 수 있도록 노력해야 하며, 상대적으로 부작용이 적은 마크로라이드계 항생제로도 좋은 치료 결과를 기대해 볼 수 있다.

• 대한한의학방제학회지
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• 제19권2호
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• pp.179-189
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• 2011

Objectives : Aconiti Lateralis Preparata Radix(fuzi, 附子) and its class herbs(chaunwu, caowu etc.) are necessary for some clinical conditions, such as cold pain, chilling etc,. But, they has some poison component. And, they have been known to cause liver and kidney injury, and dangerous in the patients who has abnormal range of LFT and RFT. This study shows the consequences for aspartate aminotransferase(AST), alanine aminotransferase(ALT), blood urea nitrate(BUN), and Creatinine were analyzed using samples from patients who took the decoction containing fuzi. Methods : Blood samples for Experiment Group(E) were collected from 63 patients, who took the decoction containing fuzi, admitted into the 6th internal medicine department of Dong Eui Oriental Medical Hospital between from January 2007 to March 2011. In compared to those of experiment group, blood samples as Control Group(C) were collected from 64 patients, who took the decoction not containing fuzi, admitted into the same hospital from January 2009 to April 2011. Results and Conclusions : 1. AST No changed : E 4.17%, C 2.63%, Increased : E 12.5%, C 28.95%, Decreased : E 80.33%, C 68.42% 2. ALT No changed : E2.94%, C5.13%, Increased : E 8.82%, C 20.51%, Decreased : E 88.24%, C 74.36% 3. BUN No changed : E 0%, CG 7.14%, Increased : E 32.25%, CG 14.29% Decreased : EG 67.65%, CG 78.57% 4. Creatinine No changed : EG 5.00%, CG 0%, Increased : EG 35.00%, CG 54.55% Decreased : EG 60.00%, CG 45.45% 5. The results suggest that the decoction containing fuzi isn't harmful AST, ALT, BUN, Cr of the patients who has high range of them.

• Applied Microscopy
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• 제27권2호
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• pp.197-215
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• 1997

A morphologic study on the effect of butylated hydroxytoluene (BHT) on experimental hepatocarcinogenesis induced by 3'-methyl-4-dimethyl aminoazobenzene (3'-MeDAB) was investigated. A total of 110 Sprague-Dowley male rats weighting about 200 g each were used for the experiment, and divided into 4 groups; the 3'-MeDAB, BHT, 3'-MeDAB/BHT treated group, and the control group. Four to eight rats of each group were sacrified on the 4th, 8th, 14th and 16th experimental weeks, with continuous pelletized feeding containing 0.09% 3'-MeDAB and 0.5% BHT. The liver was examined by transmission and scanning electron microscopy. The results were as follows; Electron microscopically, the fine structure of the hepatocytes remained consistently abnormal up to 16 weeks after the 3'-MeDAB treatment. There was no significant difference in the groups observed earlier than in the ones observed later. Many subcellular changes were observed : nuclear change, decreased glycogen, mitochondrial abnormalities, disaggregated rough endoplasmic reticulum, marked proliferation of smooth endoplasmic reticulum, dilatation and distortion of bile canaliculi, increased lysosomes, apoptotic bodies, migration of bile ductule cell. In the BHT treated group, the ultrastructural changes of hepatocytes were not significant, except for the lipid droplets and proliferated smooth endoplasmic reticulum among hepatocytes depending on the experimental duration. The various subcellular changes of 3'-MeDAB/BHT treated groups were simillar to those of the 3'-MeDAB treated group, but the degree of changes in the 3'-MeDAB/ BHT treated group decreased compared with those of the 3'-MeDAB treated group. These results suggest that dietary butylated hydroxytoluene has a protective/inhibitory effect on the hepatocarcinogenesis induced by 3'-methyl-4-dimethyl -aminoazobenzene.

• Journal of Preventive Medicine and Public Health
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• 제33권1호
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• pp.17-24
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• 2000

Objectives : To propose the referential data to evaluate the health impacts of Vietnam veterans' children whose father were exposed to herbicides in Vietnam War. Methods : Vietnam veterans who visited to Pusan Veteran Hospital for medical care were recruited from April to October, 1998. They were 71 and asked about their own combat history, symptoms and illness, and health status of their 182 children. The informations were collected by direct and phone interview. Exposure estimation was also performed as exposure score depending on year and unit of participation, and personal episodes related to exposure to herbicide in the war. It classified into three groups; lower(<18.0), moderate(18-53), high$(\geq53)$ exposure group. Results : The mean age and the period into the combat of the veterans were 52.8 years and 15.0 months. The mean exposure score was $18.1{\pm}9.9$, and mainly distributed in lower (46.5%) and moderate(52.1%) exposure group. Most(90.1%) of them were diagnosed as sequelae(21 cases) and suspected sequelae(43 cases) of the herbicides by Korean veteran's hospital diagnostic criteria. The major sequelae was peripheral neuropathy 13 cases, chloracne 5 cases, and the major suspected sequelae was hypertension 20 cases, diabetes mellitus 18 cases, liver disease 12 cases, central neuropathy 11 cases, etc. About birth, 42.2% and 16.9% experienced spontaneous abortion and stillbirth, respectively. The mean exposure score was higher in stillbirth experience group(p<0.05). About half of the children(90 cases, 49.5%) hold the abnormal health status: those were skin pigmentation 38 cases, rash 23 cases, congenital anomaly 15 cases, general weakness 12 cases, purpura 8 cases, visual disturbance 8 cases, etc. These health problems had no association with father's exposure level(p>0.05). Conclusions : These results were depend on their own answers, and expectation for compensation did not excluded, therefore, this study may have limitations: inaccuracy of informations due to recall bias and response bias. Nevertheless, through this study, we could image the fundamental aspect for health impacts of Vietnam veterans' children for preparing the national control program and policy. A large scale epidemiologic study with valid exposure assessment on the health impacts of Vietnam veterans' children is recommneded.

• Journal of Chest Surgery
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• 제27권3호
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• pp.177-186
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• 1994

Pentastarch is a hydroxyethyl starch similar to hetastarch, but lower average molecular weight and fewer hydroxyethyl groups which result in enhanced enzymatic hydrolysis and faster renal elimination.This report was performed to compare the clinical efficacy and safety of 10 % pentastarch[Pentaspan , group I] for plasma volume expansion after open heart surgery with that of 5% albumin[Plasmanate, group II]. There were no statistically significant differences between the group I [n=18] and group II [n:19] in the preoperative parameters [age, sex, body weight] and operative parameters[bypass time, aorta cross clamping time]. During the first 24 hours after arrival of the patient in the surgical intensive care unit, colloid solution [500--1000 ml] was infused to maintain left atrial pressure of more than 8 mmHg, or cardiac index of 2.0 L/min/M2 of more. In results, there were 3 complications of hypotension immediately after infusion of 5 % albumin solution and 2 among the 3 patients were excluded for the study. However there was no complication after infusion of 10 % pentastarch solution. Hemodynamic responses to infusion was similar for both groups, although in group I a greater increase in both left atrial pressure[mean 1.8 versus 0.7 mmHg, p< 0.05] and right atrial pressure [mean 2.2 versus 1.7 mmHg, p < 0.05] was observed during infusion of the first 500 ml. There were no significant differences in any of the measured respiratory parameters[PaO2, intrapulmonary shunt, and effective lung compliance]. Homodilution with colloid significantly reduced hemoglobin [mean 1.2 versus 0.8 gm/dl], and serum protein and albumin level[total protein;4.8$\pm$ 0.5 versus 5.2 $\pm$0.5 gm/dl, p < 0.05: albumin: 3.2 $\pm$0.4 versus 3.6 $\pm$0.6 gm/dl, p < 0.05] by 6:00 AM on 1 day postoperatively, however there were no significant differences on 7 day postoperatively. The mean serum colloid osmotic pressure and osmolarity was similar in both group.There were no abnormal findings of liver function and kidney function in all the patients. There were no significant between-group differences in bleeding time, platelets, prothrombin time, activated partial thromboplastin time and amount of chest tube output measured on 1st and 7th postoperative day. These findings demonstrated that 10% pentastarch is more effective and safe for plasma volume expension than 5 % albumin solution with no adverse effects on coagulation. Also 10 % pentastarch is less expensive than 5 % albumin and it would appeare to be a reasonable first choice for plasma volume expansion.

• 동의생리병리학회지
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• 제19권5호
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• pp.1296-1302
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• 2005

Laminariae Thallus has been known to be effective for the treatment of obesity. Te show the effectiveness of Laminariae Thallus in a more scientific way, Laminariae Thallus extract was prepared and evaluated in high fat diet rats by measuring the changes of body weight and lipid metabolism as described briefly below. 200 g of crushed Laminariae Thallus was extracted with methyl alcohol. The extract was evaporated under reduced pressure to give 14.4g. For 10 weeks, control group rat were ingested high fat diet, while the test group rat were fed with a fat diet pius Laminariae Thallus extract. Normal group was fed with normal diet. 150 mg of Laminariae Thallus extract per 1 kg of body weight was added to the diet in a test group rats. Control group rats on a high fat diet gained weight significantly, whereas the test group rats on a high fat diet plus Laminariae Thallus extract gained weight less. Significant increase of liver weight caused in a high fat diet was also inhibited by the Laminariae Thallus extract treatment. Total lipid, triglyceride, phospholipid and total cholesterol levels of serum in the high fat diet rats were remarkably increased, whereas their levels on a high fat diet plus Laminariae Thallus extract were less increased. While serum HDL-cholesterol levels was remarkably decreased in a high fat diet, its level was less decreased in a high fat diet plus Laminariae Thallus extract. Furthermore, we observed that the activities of hepatic acetyl-CoA carboxylase and fatty acid synthetase increased in a high fat diet, while their activities in a high fat diet plus Laminariae Thallus extract were getting back nearly to the normal levels of normal diet rats. These result showed that the obesity caused by a high fat diet was effectively inhibited by a Laminariae Thallus extract. Our results also showed that the abnormal lipid metabolism caused by a high fat diet was effectively cured by adding Laminariae Thallus extract.

• Tuberculosis and Respiratory Diseases
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• 제53권2호
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• pp.196-201
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• 2002

악성 흑색종은 멜라닌세포에서 발생하는 종양으로 전체 악성 종양의 3%를 차지하고, 피부, 안구, 피부주위점막에서 호발한다. 이들은 주로 피부, 림프절, 폐, 간, 골 등으로 전이되고 폐의 전이는 약 5~15%에서 일어난다. 그러나 기관지 점막으로의 전이는 흔하지 않다. 특히 기관지 및 폐장으로 전이된 원발불명의 악성 흑색종은 아직 보고된 예가 없다. 이에 저자들은 기침과 검은색 객담을 주소로 내원하였던 34세 남자환자에서 굴곡성 기관지 내시경 및 조직검사를 시행하여 vimentin, S-100 protein, HMB-45 염색에 양성을 보이는 양측 폐장 및 기관지점막으로 전이된 악성 흑색종을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• 생명과학회지
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• 제22권11호
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• pp.1500-1506
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• 2012

Transforming growth factor-${\beta}$ (TGF-${\beta}$)에 의해 유도되는 세포사멸 과정은 간에서 손상 받은 조직이나 비정상적인 조직을 제거하는데 중요한 역할을 담당한다. 간세포에서 TGF-${\beta}$에 의해 유도되는 세포사멸 과정 동안 중요한 기능을 담당하는 유전자를 탐색하고자 쥐의 간세포인 AML12 세포를 이용하여 TGF-${\beta}$ 처리 전후에 발현이 변화되는 유전자를 microarray analysis를 통해 확인하였다. TGF-${\beta}$에 의해 여러 가지 유전자들의 발현이 변화됨을 확인하였는데, 그 가운데 여러 가지 세포사멸 인자들의 활성을 억제하여 세포사멸을 억제한다고 알려져 있는 SGK1의 발현 감소를 확인하였다. TGF-${\beta}$에 의해 SGK1의 mRNA와 단백질 level이 모두 감소함을 확인하였고, 항상 active한 형태의 SGK1 (CA-SGK1)을 발현시켰을 때 TGF-${\beta}$에 의한 세포사멸이 억제됨을 확인하였다. 이러한 결과들은 간세포에서 SGK1의 발현 감소가 TGF-${\beta}$에 의한 세포사멸을 유도하는데 중요한 기능을 담당할 가능성이 높음을 의미하는 것이다.

• 대한약리학회지
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• 제31권2호
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• pp.241-250
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• 1995

Protein B23 is one of the major nucleolar phosphoproteins associated with pre-ribosomal particles, and is localized in the granular region of the nucleolus. Recent studies suggest that protein B23 shuttles between nucleus and cytoplasm and also interacts with HIV Rev. These findings indicate that protein B23 is important in nucleocytoplasmic relationship and viral replication. However, the exact function of protein B23 is not clear yet. In acute nucleolar hypertrophy of rat liver, treated with thioacetamide, there was observed an increase of not only protein B23 but also B23-like protein p45 when anti-B23 monoclonal antibody (MAb) was used for identification. On the basis of the large B23 specific epitope structure composed of 68 amino acids, a hypothesis was formulated to examine that p45 is the pre-B23 resulting from excessive production of B23. In an attempt to investigate the precursor of B23, we analyzed the subcellular fractions and microsomal subfractions. Subsequently, we analyzed the finger printings of B23-like proteins using the tryptic peptide mapping. The results are summarized: 1) Using B23 MAb, we observed the presence of B23-like proteins in nucleolar fraction, nucleoplasmic fraction and microsomal fraction. 2) In the further microsomal subfractionation, we could partially purify B23-like protein in 2M layer of sucrose gradient. 3) When ion exchange chromatography was employed, there were protein species 80kDa(p80), 65kDa(p65) and 60kDa(p60). 4) Based on the tryptic map analysis of $^{125}I$ labeled proteins, the similarity between B23 and p80 was found only in 9 out of 14(B23) and 21(p80) peptides, and difference was found in the remaining peptides. p80 and p60 had 18 common peptides, and all the peptides of p60 were similar to those of p80. From these results, it is proposed that p45 is an abnormal metabolite resulting from carcinogenesis by thioacetamide, and it is not the precursor of B23. In addition, we suggest that p80 may be a precursor of p45.