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선천성 Antithrombin III 결핍증에서 발생한 폐색전증 1 예
박형관,박창민,고경행,임명수,김유일,황준화,임성철,김영철,박경옥,Park, Hyeong-Kwan,Park, Chang-Min,Ko, Kyoung-Haeng,Rim, Myung-Soo,Kim, Yu-Il,Hwang, Jun-Hwa,Lim, Sung-Chul,Kim, Young-Chul,Park, Kyung-Ok 대한결핵및호흡기학회 1999 Tuberculosis and Respiratory Diseases Vol.47 No.3
저자들은 갑작스런 흉통을 주소로 내원한 젊은 남자에서 AT III 결핍증에 의한 폐색전증을 진단하고 세 자녀 모두에서도 AT III 결핍이 확인됨으로써 폐색전증을 동반한 선천성, 가족성 AT III 결핍증을 경험하였기에 문헌고찰과 함께 보고한다. We report a case of congenital and familial antithrombin III deficiency developing massive pulmonary thromboembolism. A 44-year-old man was admitted to our hospital because of sudden chest pain and severe dyspnea. Five years ago, he was operated due to a mesenteric vein thrombosis of unknown cause. On admission, radioisotopic venogram showed deep vein thrombosis and lung scintigram showed multiple segmental perfusion defects. His plasma antithrombin III level was 10.5 mg/dL which was less than 50% of normal and those of a son and two daughters were also decreased. After treatment with tissue plasminogen activator, heparin and coumadin, his symptom and lung scintigram were significantly improved. As far as we reviewed, there were very rare reports with congenital antithrombin III deficiency presenting as pulmonary thromboembolism in Korea.
선천성 Antithrombin 3 결핍증에서 발생한 폐색전증
박형관 ( Hyeong Kwan Park ),박창민 ( Chang Min Park ),고경행 ( Kyoung Haeng Ko ),임명수 ( Myung Soo Rim ),김유일 ( Yu Il Kim ),황준화 ( Jun Hwa Hwang ),임성철 ( Sung Chul Lim ),김영철 ( Young Chul Kim ),박경옥 ( Kyung Ok Park ) 대한결핵 및 호흡기학회 1999 Tuberculosis and Respiratory Diseases Vol.47 No.3
결핵성 및 비결핵성 흉막삼출액에서 TNF-$\alpha$ 농도의 진단적 의의
나현주,박석채,강광원,박형관,김영철,최인선,박경옥,Na, Hyun-Joo,Park, Seog-Chea,Kang, Kwang-Won,Park, Hyeong-Kwan,Kim, Young-Chul,Choi, In-Seon,Park, Kyung-Ok 대한결핵및호흡기학회 1997 Tuberculosis and Respiratory Diseases Vol.44 No.3
연구배경 : 결핵성 흉막염의 감별 진단 목적으로 널리 이용되고 있는 검사는 adenosine deaminase와 INF-$\gamma$를 세포성 면역에 또다른 중요한 매개체로 알려진 TNF-$\alpha$의 진단적 의의를 조사하고, 현재 임상에서 유용한 지표로 사용되고 있는 adenosine deaminase와의 감별력을 비교하고자 본 연구를 시행하였다. 방 법 : 삼출성 흉막염 80예(결핵성 : 39예, 암성 : 31예, 부폐렴흉막염 : 10예)를 대상으로 흉수의 기본적인 세포조성, 화학 검사와 더불어 ADA와 TNF-$\alpha$(Medgenix IRMA kit)를 측정하였다. 결 과 : 흉수내 ADA농도는 결핵성 흉막염에서 $48.7{\pm}32.7U/L$로 비결핵성 흉막염 $26.0{\pm}41.3U/L$에 비해 유의한 차이로 높았고(p < 0.05), TNF-$\alpha$치 또한 결핵성 흉막염에서 $184.1{\pm}214.2pg/mL$로 비결핵성 흉막삼출액 $44.1{\pm}114.2pg/mL$에 비해 유의하게 높았다(p < 0.01). ROC 곡선을 이용하여 ADA와 TNF-$\alpha$의 감별력을 가장 높일 수 있는 기준치를 정하였을 때, ADA는 30U/ml, TNF-$\alpha$는 15pg/ml로 측정되었고, 각각의 기준치를 이용하여 감수성과 특이도를 구하였을때, ADA는 감수성 66.7%, 특이도 85.0%, TNF-$\alpha$는 감수성 69.2% 특이도 87.1%를 보였다. 두 검사의 민감도와 특이도를 비교하기 위한 ROC 곡선에서, ROC 곡선아래의 면적(area under curve)은 ADA와 TNF-$\alpha$사이에 유의한 차이가 없었다(ADA 0.83, TNF-$\alpha$ 0.82). 다중회귀분석(multiple stepwise regression)에서 ADA가 가장 유용한 지표로 계산되었으나 TNF-$\alpha$의 추가가 분별력에 더 이상의 기여함은 없었다. 결 론 : 결핵성 흉막염과 비결핵성 흉막염을 감별 진단하는데 ADA와 함께 TNF-$\alpha$도 유용한 지표로 이용될 수 있으나 두가지 검사를 함께 시행함에 따른 진단에 있어서의 잇점은 없었다. Objectives : The differentiation of tuberculous effusion from the other causes of exudative pleural effusion remained difficult even with aids of biochemical analyses and pleural biopsy. As the pathophysiology of tuberculous pleural effusion is an enhanced cell mediated immunity, Adenosine deaminase(ADA) and various eytokines including Inteferon-$\gamma$, tumor necrosis factor alpha(TNF-$\alpha$) are considered as useful diagnostic tools in differentiating exudative pleural effusion. The author would like to demonstrate the diagnostic usefulness of TNF-$\alpha$ in the differentiation of exudative pleural effusion, and compared the discriminating ability of TNF-$\alpha$ with ADA. Methods : Pleural fluids obtained from 80 patients (tuberculous : 39, malignant : 31, parapneumonic : 10) with exudate pleural effusions were processed for cell counts and biochemical analysis including ADA and TNF-$\alpha$. Results : Tuberculous pleural fluid showed higher levels of ADA and TNF-$\alpha$, $48.7{\pm}32.7U/L$ and $184.1{\pm}214.2pg/mL$ than that of non-tuberculous effusion $26.0{\pm}41.3U/L$ and $44.1{\pm}114.2pg/mL$, respectively (ADA, TNF-$\alpha$, p < 0.05, p < 0.01). Receiver operating characteristics(ROC) curves were generated for ADA and TNF-$\alpha$ and the best cut-off value for adenosine deaminase and TNF-$\alpha$were considered as 30U/L and 15pg/ml, respectively. Comparing the area under the ROC curves, there was no significant difference between ADA and TNF-$\alpha$. Conclusion : For the differential diagnosis of tuberculous pleural effusion from the other causes of exudative pleural effusions, TNF-$\alpha$ as well as ADA was considered as useful diagnostic method. However adding TNF-$\alpha$ to ADA has no further diagnotic benefit than ADA alone.
고칼슘혈증을 보인 폐암환자에서 부갑상선호르몬(PTH)과 부갑상선호르몬관련단백질(PTHrP)이 동시에 증가된
김유일 ( Yu Il Kim ),김규식 ( Kyu Sik Kim ),유영권 ( Young Kwon Yu ),박창민 ( Chang Min Park ),임명수 ( Myung Soo Rim ),고경행 ( Kyung Haeng Ko ),황준화 ( Jun Hwa Hwang ),박형관 ( Hyeong Kwan Park ),임성철 ( Sung Chul Lim ),김영철 대한결핵 및 호흡기학회 1999 Tuberculosis and Respiratory Diseases Vol.47 No.4
김준우(Joon Woo Kim),강광원(Kwang Won Kang),황준화(Jun Hwa Hwang),장일권(Il Gweon Jang),박형관(Hyeong Kwan Park),박창환(Chang Whan Park),이정훈(Jeong Hoon Lee),김영철(Young Chul Kim),박종춘(Jong Chun Park),박경옥(Kyung Ok Park) 대한내과학회 1997 대한내과학회지 Vol.53 No.4
Hepatopulmonary syndrome consists of a triad of liver dysfunction, intrapulmonary vascular dilatation, and hypoxemia. This is one of the main causes of arterial hypoxemia in patients with chronic liver disease. The vascular abnormalities are precapillary dilatation, direct arterial-venous communication, and dilated pleural vessels. In this article, we report a case of hepatopulmonary syndrome in a 62-year-old woman who had complained progressively worsening dyspnea, platypnea, and orthodeoxia. She had huge splenomegaly, clubbing fingers and cyanosis of lip and fingers. Arterial blood gas analysis showed refractory arterial hypoxemia and orthodeoxia suggesting right-to-left shunting. Chest X-ray showed increased interstitial markings on the lower part of right lung, In 99mTc-labeled macroaggregated albumin (MAA) lung perfusion scan, there was no perfusion defect in the lung, but labeled radionuclide were taken up in the intraabdominal organs, kidney, liver and spleen. The amount of shunted radionuclide were about 58 percent. In contrast echocardiography, microbubbles which were injected via cephalic vein were visualized in the left atrium at 4 cardiac cycles after leaving the right ventricle indicating intrapulmonary right-to-left shunting rather than intracardiac shunt. Pulmonary angiographic finding revealed diffuse blotchy arterial dilatation on both lung fields, especially lower lobes of both lungs. Current modalities of treatment of hepatopulmonary syndrome are the therapeutic embolization of direct arterial-venous communication for focal vascular dilatations, and TIPSS (Transjugular intrahepatic porto-systemic shunt) or liver transplantation for diffuse intrapulmonary vascular dilatations. Despite our recommendation of TIPSS, she refused the procedure and is under home oxygen therapy.